WorldWideScience

Sample records for sarcoma primary effusion

  1. High-grade primary myxoid lung sarcoma presenting as recurrent hemorrhagic pleural effusions in a young woman.

    Science.gov (United States)

    Tahir, Hassan; Coleman, Cinthia; Sagi, Jahnavi; Wani, Adil; Daruwalla, Vistasp

    2015-01-01

    Primary lung sarcomas are rare but aggressive tumors accounting for less than 0.5% of all lung tumors. The diagnosis of primary lung sarcoma should only be considered after exclusion of other sites. A 32-year-old female presented with recurrent hemorrhagic pleural effusions, shortness of breath and persistent cough. Pleural effusion was drained twice, and each time its analysis was normal. Patient developed atelectasis of left lung with hemothorax for which she underwent video-assisted thoracoscopic surgery. A large mass was found compressing the entire lower lobe of left lung with extension into mediastinum, the biopsy of which showed myxoid sarcoma. The tumor was inoperable and options of chemotherapy or radiotherapy were discussed with the patient. Primary lung sarcoma can rarely present with recurrent hemorrhagic pleural effusion. A high degree of suspicion is required for early diagnosis as large hemothorax on computed tomography or chest X-ray may obscure lung mass and make its diagnosis difficult.

  2. Primary effusion lymphoma: a distinct clinicopathologic entity associated with the Kaposi's sarcoma-associated herpes virus.

    Science.gov (United States)

    Nador, R G; Cesarman, E; Chadburn, A; Dawson, D B; Ansari, M Q; Sald, J; Knowles, D M

    1996-07-15

    We recently discovered the Kaposi's sarcoma-associated herpes virus (KSHV/HHV-8) in an uncommon and unusual subset of AIDS-related lymphomas that grow mainly in the body cavities as lymphomatous effusions without an identifiable contiguous tumor mass. The consistent presence of KSHV and certain other distinctive features of these body cavity-based lymphomas suggest that they represent a distinct entity. We tested this hypothesis by investigating 19 malignant lymphomatous effusions occurring in the absence of a contiguous tumor mass for their clinical, morphologic, immunophenotypic, viral, and molecular characteristics, KSHV was present in 15 of 19 lymphomas. All four KSHV-negative lymphomatous effusions exhibited Burkitt or Burkitt-like morphology and c-myc gene rearrangements and, therefore, appeared to be Burkitt-type lymphomas occurring in the body cavities. In contrast, all 15 KSHV-positive lymphomatous effusions exhibited a distinctive morphology bridging large-cell immunoblastic lymphoma and anaplastic large-cell lymphoma, and all 12 cases studied lacked c-myc gene rearrangements. In addition, these lymphomas occurred in men (15/15), frequently but not exclusively in association with HIV infection (13/15), in which homosexuality was a risk factor (13/13), presented initially as a lymphomatous effusion (14/15), remained localized to the body cavity of origin (13/15), expressed CD45 (15/15) and one or more activation-associated antigens (9/10) in the frequent absence of B-cell-associated antigens (11/15), exhibited clonal immunoglobulin gene rearrangements (13/13), contained Epstein-Barr virus (14/15), and lacked bcl-2, bcl-6, ras and p53 gene alterations (13/15). These findings strongly suggest that the KSHV-positive malignant lymphomatous effusions represent a distinct clinicopathologic and biologic entity and should be distinguished from other malignant lymphomas occurring in the body cavities. Therefore, we recommend that these malignant lymphomas be

  3. High-grade primary myxoid lung sarcoma presenting as recurrent hemorrhagic pleural effusions in a young woman

    Directory of Open Access Journals (Sweden)

    Hassan Tahir

    2015-12-01

    Full Text Available Primary lung sarcomas are rare but aggressive tumors accounting for less than 0.5% of all lung tumors. The diagnosis of primary lung sarcoma should only be considered after exclusion of other sites. A 32-year-old female presented with recurrent hemorrhagic pleural effusions, shortness of breath and persistent cough. Pleural effusion was drained twice, and each time its analysis was normal. Patient developed atelectasis of left lung with hemothorax for which she underwent video-assisted thoracoscopic surgery. A large mass was found compressing the entire lower lobe of left lung with extension into mediastinum, the biopsy of which showed myxoid sarcoma. The tumor was inoperable and options of chemotherapy or radiotherapy were discussed with the patient. Primary lung sarcoma can rarely present with recurrent hemorrhagic pleural effusion. A high degree of suspicion is required for early diagnosis as large hemothorax on computed tomography or chest X-ray may obscure lung mass and make its diagnosis difficult.

  4. Primary Right Atrial Sarcoma Presenting with Cardiac Tamponade and Massive Pleural Effusion

    Directory of Open Access Journals (Sweden)

    Shahram Momtahen

    2016-07-01

    Full Text Available Primary cardiac sarcomas are very rare and there is no consensus on management. Clinical presentation is usually late. Despite newer diagnostic technology, prognosis remains dismal. We report a case of right atrial sarcoma in a 28-year-old man who presented with acute cardiac tamponade. Emergency subxiphoid pericardial drainage stabilized the patient's critical condition. The lesion was advanced. Therefore, we only performed a suboptimal surgical resection. Despite planning for radiation, the patient's status deteriorated. Only palliative measures continued during the next four months before his death due to disseminated metastasis and progressive cardiopulmonary failure.

  5. Expression of Kaposi's sarcoma-associated herpesvirus G protein-coupled receptor monocistronic and bicistronic transcripts in primary effusion lymphomas.

    Science.gov (United States)

    Nador, R G; Milligan, L L; Flore, O; Wang, X; Arvanitakis, L; Knowles, D M; Cesarman, E

    2001-08-15

    Kaposi's sarcoma-associated herpesvirus (KSHV/HHV-8) encodes a G protein-coupled receptor (vGPCR) in open reading frame (ORF) 74, which is homologous to human chemokine receptors. KSHV vGPCR is constitutively active and induces VEGF-mediated angiogenesis. Previous studies have shown that ORF 74 is transcribed as part of a bicistronic message containing ORF K14 upstream of ORF 74, with an early lytic pattern of expression. We have now extended these studies by analyzing three different KSHV-positive primary effusion lymphoma (PEL) cell lines and three PEL clinical samples. In addition, we have identified another less abundant monocistronic transcript containing only ORF 74. Both transcripts were identified at low but similar levels in two PEL clinical samples. We evaluated the degree of sequence and functional conservation of ORF74 in three additional PELs and two KS clinical specimens, demonstrating complete identity at the amino acid level among all isolates. While it is expressed as an early lytic transcript in PEL cell lines, in primary clinical PEL samples transcription of KSHV vGPCR can be readily detected.

  6. A role for MALT1 activity in Kaposi's sarcoma-associated herpes virus latency and growth of primary effusion lymphoma.

    Science.gov (United States)

    Bonsignore, L; Passelli, K; Pelzer, C; Perroud, M; Konrad, A; Thurau, M; Stürzl, M; Dai, L; Trillo-Tinoco, J; Del Valle, L; Qin, Z; Thome, M

    2017-03-01

    Primary effusion lymphoma (PEL) is an incurable malignancy that develops in immunodeficient patients as a consequence of latent infection of B-cells with Kaposi's sarcoma-associated herpes virus (KSHV). Malignant growth of KSHV-infected B cells requires the activity of the transcription factor nuclear factor (NF)-κB, which controls maintenance of viral latency and suppression of the viral lytic program. Here we show that the KSHV proteins K13 and K15 promote NF-κB activation via the protease mucosa-associated lymphoid tissue lymphoma translocation protein-1 (MALT1), a key driver of NF-κB activation in lymphocytes. Inhibition of the MALT1 protease activity induced a switch from the latent to the lytic stage of viral infection, and led to reduced growth and survival of PEL cell lines in vitro and in a xenograft model. These results demonstrate a key role for the proteolytic activity of MALT1 in PEL, and provide a rationale for the pharmacological targeting of MALT1 in PEL therapy.

  7. Ago HITS-CLIP expands understanding of Kaposi's sarcoma-associated herpesvirus miRNA function in primary effusion lymphomas.

    Directory of Open Access Journals (Sweden)

    Irina Haecker

    Full Text Available KSHV is the etiological agent of Kaposi's sarcoma (KS, primary effusion lymphoma (PEL, and a subset of multicentricCastleman's disease (MCD. The fact that KSHV-encoded miRNAs are readily detectable in all KSHV-associated tumors suggests a potential role in viral pathogenesis and tumorigenesis. MiRNA-mediated regulation of gene expression is a complex network with each miRNA having many potential targets, and to date only few KSHV miRNA targets have been experimentally determined. A detailed understanding of KSHV miRNA functions requires high-through putribonomics to globally analyze putative miRNA targets in a cell type-specific manner. We performed Ago HITS-CLIP to identify viral and cellular miRNAs and their cognate targets in two latently KSHV-infected PEL cell lines. Ago HITS-CLIP recovered 1170 and 950 cellular KSHV miRNA targets from BCBL-1 and BC-3, respectively. Importantly, enriched clusters contained KSHV miRNA seed matches in the 3'UTRs of numerous well characterized targets, among them THBS1, BACH1, and C/EBPβ. KSHV miRNA targets were strongly enriched for genes involved in multiple pathways central for KSHV biology, such as apoptosis, cell cycle regulation, lymphocyte proliferation, and immune evasion, thus further supporting a role in KSHV pathogenesis and potentially tumorigenesis. A limited number of viral transcripts were also enriched by HITS-CLIP including vIL-6 expressed only in a subset of PEL cells during latency. Interestingly, Ago HITS-CLIP revealed extremely high levels of Ago-associated KSHV miRNAs especially in BC-3 cells where more than 70% of all miRNAs are of viral origin. This suggests that in addition to seed match-specific targeting of cellular genes, KSHV miRNAs may also function by hijacking RISCs, thereby contributing to a global de-repression of cellular gene expression due to the loss of regulation by human miRNAs. In summary, we provide an extensive list of cellular and viral miRNA targets representing an

  8. Kaposi's Sarcoma-Associated Herpesvirus K-bZIP Protein Is Necessary for Lytic Viral Gene Expression, DNA Replication, and Virion Production in Primary Effusion Lymphoma Cell Lines▿ †

    OpenAIRE

    Lefort, Sylvain; Flamand, Louis

    2009-01-01

    Kaposi's sarcoma-associated herpesvirus (KSHV) is the causative agent of three human proliferative disorders, namely, Kaposi's sarcoma, primary effusion lymphomas (PEL), and multicentric Castleman's disease. Lytic DNA replication of KSHV, which is essential for viral propagation, requires the binding of at least two KSHV proteins, replication and transactivation activator (RTA) and K-bZIP, on the lytic origin of replication. Moreover, K-bZIP physically interacts with RTA and represses its tra...

  9. Azidothymidine Sensitizes Primary Effusion Lymphoma Cells to Kaposi Sarcoma-Associated Herpesvirus-Specific CD4+ T Cell Control and Inhibits vIRF3 Function

    Science.gov (United States)

    Stürzl, Michael; Sabbah, Shereen

    2016-01-01

    Kaposi sarcoma-associated herpesvirus (KSHV) is linked with the development of Kaposi sarcoma and the B lymphocyte disorders primary effusion lymphoma (PEL) and multi-centric Castleman disease. T cell immunity limits KSHV infection and disease, however the virus employs multiple mechanisms to inhibit efficient control by these effectors. Thus KSHV-specific CD4+ T cells poorly recognize most PEL cells and even where they can, they are unable to kill them. To make KSHV-infected cells more sensitive to T cell control we treated PEL cells with the thymidine analogue azidothymidine (AZT), which sensitizes PEL lines to Fas-ligand and TRAIL challenge; effector mechanisms which T cells use. PELs co-cultured with KSHV-specific CD4+ T cells in the absence of AZT showed no control of PEL outgrowth. However in the presence of AZT PEL outgrowth was controlled in an MHC-restricted manner. To investigate how AZT sensitizes PELs to immune control we first examined BJAB cells transduced with individual KSHV-latent genes for their ability to resist apoptosis mediated by stimuli delivered through Fas and TRAIL receptors. This showed that in addition to the previously described vFLIP protein, expression of vIRF3 also inhibited apoptosis delivered by these stimuli. Importantly vIRF3 mediated protection from these apoptotic stimuli was inhibited in the presence of AZT as was a second vIRF3 associated phenotype, the downregulation of surface MHC class II. Although both vFLIP and vIRF3 are expressed in PELs, we propose that inhibiting vIRF3 function with AZT may be sufficient to restore T cell control of these tumor cells. PMID:27893813

  10. Kaposi's sarcoma-associated herpesvirus-positive primary effusion lymphoma tumor formation in NOD/SCID mice is inhibited by neomycin and neamine blocking angiogenin's nuclear translocation.

    Science.gov (United States)

    Bottero, Virginie; Sadagopan, Sathish; Johnson, Karen E; Dutta, Sujoy; Veettil, Mohanan Valiya; Chandran, Bala

    2013-11-01

    Angiogenin (ANG) is a 14-kDa multifunctional proangiogenic secreted protein whose expression level correlates with the aggressiveness of several tumors. We observed increased ANG expression and secretion in endothelial cells during de novo infection with Kaposi's sarcoma-associated herpesvirus (KSHV), in cells expressing only latency-associated nuclear antigen 1 (LANA-1) protein, and in KSHV latently infected primary effusion lymphoma (PEL) BCBL-1 and BC-3 cells. Inhibition of phospholipase Cγ (PLCγ) mediated ANG's nuclear translocation by neomycin, an aminoglycoside antibiotic (not G418-neomicin), resulted in reduced KSHV latent gene expression, increased lytic gene expression, and increased cell death of KSHV(+) PEL and endothelial cells. ANG detection in significant levels in KS and PEL lesions highlights its importance in KSHV pathogenesis. To assess the in vivo antitumor activity of neomycin and neamine (a nontoxic derivative of neomycin), BCBL-1 cells were injected intraperitoneally into NOD/SCID mice. We observed significant extended survival of mice treated with neomycin or neamine. Markers of lymphoma establishment, such as increases in animal body weight, spleen size, tumor cell spleen infiltration, and ascites volume, were observed in nontreated animals and were significantly diminished by neomycin or neamine treatments. A significant decrease in LANA-1 expression, an increase in lytic gene expression, and an increase in cleaved caspase-3 were also observed in neomycin- or neamine-treated animal ascitic cells. These studies demonstrated that ANG played an essential role in KSHV latency maintenance and BCBL-1 cell survival in vivo, and targeting ANG function by neomycin/neamine to induce the apoptosis of cells latently infected with KSHV is an attractive therapeutic strategy against KSHV-associated malignancies.

  11. Primary renal synovial sarcoma

    Directory of Open Access Journals (Sweden)

    Girish D. Bakhshi

    2012-03-01

    Full Text Available Primary Renal Sarcoma is rare tumor comprising only 1% of all renal tumours. Synovial sarcomas are generally deep-seated tumors arising in the proximity of large joints of adolescents and young adults and account for 5-10% of all soft tissue tumours. Primary synovial sarcoma of kidney is rare and has poor prognosis. It can only be diagnosed by immunohistochemistry. It should be considered as a differential in sarcomatoid and spindle cell tumours. We present a case of 33-year-old female, who underwent left sided radical nephrectomy for renal tumour. Histopathology and genetic analysis diagnosed it to be primary renal synovial sarcoma. Patient underwent radiation therapy and 2 years follow up is uneventful. A brief case report with review of literature is presented.

  12. Primary synovial sarcoma of lung

    Directory of Open Access Journals (Sweden)

    Devleena

    2014-01-01

    Full Text Available A synovial sarcoma (SS is a rare form of cancer which usually occurs near the joints of the arm, neck, or leg, but has been documented in most human tissues and organs, including the brain, prostate, and heart. Primary pulmonary SS is an extremely rare tumor. We report a case of primary SS of lung who presented with severe chest pain and a large right lung mass with right-sided pleural effusion in computed tomography (CT scan of thorax. The diagnosis was made on the basis of CT-guided core biopsy and immunohistochemistry. On immunohistochemistry, tumor cell expressed epithelial membrane antigen, bcl 2, Vimentin and smooth muscle actin and were immunonegative for S100 and cytokeratin. So, the final diagnosis was primary SS.

  13. Ewing’s Sarcoma Presenting as Pleural Effusion

    Directory of Open Access Journals (Sweden)

    Nuzhat Husain

    2011-09-01

    Full Text Available  A 20-year-old female presented to the Pulmonary Medicine Department with complaints of fever, left sided chest pain and progressive dyspnoea of four months duration. Radiological examination revealed a mass lesion with massive pleural effusion and rib erosion. Histopathology showed neoplastic cells with scanty cytoplasm, hyperchromatic nuclei and rosette formation suggestive of Ewing sarcoma. The rarity of this tumor and its unusual presentation prompted this report.

  14. Primary hepatic sarcomas: CT findings

    Energy Technology Data Exchange (ETDEWEB)

    Yu, Ri-Sheng; Chen, Ying; Jiang, Biao; Wang, Liu-Hong [Zhejiang University School of Medicine, Department of Radiology, Hangzhou (China); Xu, Xiu-Fang [Zhejiang Medical College, Teaching and Research Group of Radiology, Hangzhou (China)

    2008-10-15

    Primary hepatic sarcomas are rare tumors that are difficult to diagnose clinically. Different primary hepatic sarcomas may have different clinical, morphologic, and radiological features. In this pictorial review, we summarized computed tomography (CT) findings of some relatively common types of hepatic sarcomas, including angiosarcoma, epithelioid hemangioendothelioma (EHE), liposarcoma, undifferentiated embryonal sarcoma (UES), leiomyosarcoma, malignant fibrous histiocytoma (MFH), and carcinosarcoma (including cystadenocarcinosarcoma). To our knowledge, hepatic cystadenocarcinosarcoma has not been described in the English literature. The CT findings in our case are similar to that of cystadenocarcinoma, a huge, multilocular cystic mass with a large mural nodule and solid portion. The advent of CT has allowed earlier detection of primary hepatic sarcomas as well as more accurate diagnosis and characterization. In addition, we briefly discuss the MRI findings and diagnostic value of primary hepatic sarcomas. (orig.)

  15. Human herpesvirus 8 open reading frame 26 and open reading frame 65 sequences from multiple myeloma patients: a shared pattern not found in Kaposi's sarcoma or primary effusion lymphoma.

    Science.gov (United States)

    Ma, H J; Sjak-Shie, N N; Vescio, R A; Kaminsky, M; Mikail, A; Pold, M; Parker, K; Beksac, M; Belson, D; Moss, T J; Wu, C H; Zhou, J; Zhang, L; Chen, G; Said, J W; Berenson, J R

    2000-11-01

    Human herpesvirus 8 (HHV-8), also known as Kaposi's sarcoma-associated herpesvirus, has been implicated in the pathogenesis of Kaposi's sarcoma (KS), primary effusion lymphoma (PEL), multicentric Castleman's disease, and recently multiple myeloma (MM). DNA sequence analyses of HHV-8 suggest that multiple HHV-8 strains exist. We extracted DNA from 24 patients with MM and 3 patients with monoclonal gammopathy of undetermined significance and compared HHV-8 open reading frames (ORFs) 26 and 65 sequences with those derived from patients with KS, PEL, and two HHV-8-positive PEL cell lines KS-1 and BC-1. ORF26 sequence data suggest that MM patients are consistently carriers of HHV-8 strain subtype C3. All MM patients also consistently revealed either a single bp deletion or substitution at position 112197 in ORF65. This unique alteration is not present in patients with KS or PEL or in PEL cell lines. It occurs in the portion of ORF65 that is known to be responsible for a serological response to HHV-8.

  16. Primary fibro sarcoma of the heart.

    Science.gov (United States)

    Kabashi, Serbeze; Hoxha, Naim; Gashi, Shkelzen; Ahmegjekaj, Ilir; Bejta, Ilir; Sadiku, Muharrem; Ymeri, Halit; Kabashi, Antigona; Bicaj, Xhavit; Mucaj, Sefedin

    2013-01-01

    Primary malignant heart tumors represent rare entities where fibro sarcoma represents about 3% of all. Introducing the patient: A 15 years old patient with cardiac insufficiency (heart failure) symptoms, such as weakness, cyanosis, palpitations and breathing difficulties; enlargement of upper mediastinum and pleural effusion. Through echocardiography a pericardial effusion and intracavitary thrombus in atrium was diagnosed. With computed tomography is diagnosed a tumoral mass in right atrium which is also spread in the right ventricle of the heart. Tumor is completely removed; pat histology result showed primary fibro sarcoma of the heart. At that time no metastasis was found. Conclusion. Primary malignant heart tumors may manifest like cardiac insufficiency or like systemic diseases. Fibrosarcomas are rare and have bad prognosis. On average patients can live around six months after initial symptoms appeared and diagnosis of the tumor was done. In the case of cardiac insufficiency with differential diagnosis we should also think of heart tumors, which could certainly be proved for or eliminated by echocardiography.

  17. Primary intravascular synovial sarcoma: case report.

    Science.gov (United States)

    Tuncer, Osman Nuri; Erbasan, Ozan; Golbasi, Ilhan

    2012-10-01

    Synovial sarcoma (SS), a mesenchymal spindle cell tumor, displays variable epithelial differentiation, including glandular formation, and features a specific chromosomal translocation, t(X;18)(p11;q11). SS accounts for 5% to 10% of soft-tissue sarcomas. These tumors occur mostly in the joints, especially near the knee, but they also occur in other locations. Primary intravascular SS (IVSS) are extremely rare; only 6 well-documented cases have been reported in the English literature. We describe a new case of primary IVSS of the superior vena cava (SVC) in a 16-year-old boy. A transthoracic echocardiogram confirmed a large (4.8 × 4.6 cm) circumscribed mass filling the right atrium, as well as a moderate pericardial effusion. The mass extended from the SVC to the tricuspid valve but did not prevent valve coaptation. Surgery via a transatrial approach revealed a huge mass (8 to 12 cm) attached to the SVC via a 5-mm pedicle. The tumor was excised, and the patient experienced an uneventful postoperative course. Fluorescence in situ hybridization analysis revealed the presence of the SS-specific translocation.

  18. Primary epithelioid sarcoma of scalp

    Directory of Open Access Journals (Sweden)

    Sushma G Gurwale

    2017-01-01

    Full Text Available Epithelioid sarcoma (ES is a rare mesenchymal tumor of unknown histogenesis which displays multidirectional differentiation, predominantly epithelial. They have no normal cellular counterpart and differ from both synovial sarcoma and other carcinomas. It mainly affects young adults. It has two variants, classic type and proximal type. The more common classic type presents as a slowly growing painless nodule or plaque on the distal extremities. It is rare in children and older individuals. There is male predominance. The size varies from few millimeters to several centimeters. Central deeply seated lesions in pelvis and genital tract are termed as proximal ES. It has a multinodular growth pattern and usually occurs in older patients. These are comparatively more aggressive and metastasize early. On histopathological examination, these lesions need to be distinguished from other tumors showing epithelioid morphology. Primary ES of scalp is an exceedingly rare tumor. We present a case of nodular tumor on the scalp with cervical lymph node metastasis.

  19. Primary effusion lymphomas in AIDS: CT findings in two cases

    Energy Technology Data Exchange (ETDEWEB)

    Ferrozzi, F.; Tognini, G.; Mulonzia, N.W.; Pavone, P. [Ist. di Scienze Radiologiche, Univ. di Parma (Italy); Bova, D.

    2001-04-01

    Primary effusion lymphomas represent an unusual subset of AIDS-related non-Hodgkin's lymphomas. They are associated with herpes virus 8 and Epstein-Barr virus and characterized by predominant involvement of the serous body cavities (pleura, pericardium, peritoneum) as lymphomatous effusion without any identifiable tumour mass. We report herein CT findings in two patients with primary effusion lymphoma emphasizing the possible neoplastic nature of a pleural effusion in a patient with AIDS. (orig.) (orig.)

  20. Massive Pleural Effusion in an 18-Year-Old Girl with Ewing Sarcoma

    Directory of Open Access Journals (Sweden)

    Cengiz Özge

    2004-01-01

    Full Text Available Ewing sarcoma is a bone tumour that commonly appears between ages five and 10 in the diaphysis of the long bones and predominantly presents with pain and swelling. The case of an 18-year-old girl who presented with back pain, cough, dyspnea, weakness and fever is described. Chest radiograph showed a homogenous density in the middle and inferior zones of the right hemithorax. Thoracic computed tomography revealed a diffuse pleural effusion and a 6.99 cmx4.45 cm solid mass composed of lobulated, small cystic lesions and calcifications in the right hemithorax. Biochemical analysis of pleural fluid showed hemorrhagic effusion and exudate. A pleural needle biopsy demonstrated solid uniform tumour cells with narrowed cytoplasm, round nuclei and uncertain nucleoli. All of the tumour cell cytoplasms stained with CD99. The pathological examination supported Ewing sarcoma. Three-phase Tc-99m methylene diphosphonate scintigraphy of the whole body showed pathological tracer uptake in a broad area of the eighth costal bone and in smaller areas of the ninth and 10th costal bones. This case is reported because Ewing sarcoma is a rare cause of pleural effusion in clinical practice among younger adults.

  1. Primary osteogenic sarcoma of the breast

    Directory of Open Access Journals (Sweden)

    Akang Effiong E

    2006-12-01

    Full Text Available Abstract Background Primary extra-osseous osteogenic sarcomas have been reported in many tissues of the body but their occurrence in the breast is extremely rare. It can arise as a result of osseous metaplasia in a pre-existing benign or malignant neoplasm of the breast or as non-phylloides sarcoma from the soft tissue of a previously normal breast. Case presentation A 40 year-old Nigerian woman was clinically diagnosed to have carcinoma of the left breast. The histology report of core-needle biopsy of the mass showed a malignant neoplasm comprising islands of chondroblastic and osteoblastic stromal cells. This report changed the diagnosis from carcinoma to osteogenic sarcoma of the breast. She had a left modified radical mastectomy, however there was significant post surgery skin deficit. A latissimus dorsi musculocutaneous flap was used to cover the anterior chest wall defect. Sections from the mastectomy specimen confirmed the diagnosis of osteogenic sarcoma. She died six months after mastectomy. Conclusion A diagnosis of osteogenic sarcoma of the breast was made based on histology report and after excluding an osteogenic sarcoma arising from underlying ribs and sternum. This is the second documented case of primary osteogenic sarcoma of the breast coming from Nigeria

  2. Primary Renal Synovial Sarcoma: An Oncologic Surprise

    Directory of Open Access Journals (Sweden)

    H. Krishna Moorthy

    2014-09-01

    Full Text Available Primary renal synovial sarcoma is a rare tumor having a specific chromosomal translocation t(X; 18 (p11.2; q11.2. The clinical features of this tumor and radiologic appearances are quite similar to those of renal cell carcinoma. Confirmatory diagnosis requires fluorescent in situ hybridization or reverse transcriptase polymerase chain reaction validation for differentiating the tumors from sarcomatoid renal cell carcinoma. We present a case of primary renal synovial sarcoma that was diagnosed in a middle-aged man.

  3. Primary pleomorphic sarcoma of the liver

    Energy Technology Data Exchange (ETDEWEB)

    Mani, S.; Naik, L.; Shet, S.; Vora, I.M.; Rananavare, R. [BYL Nail Hospital, Bombay (India). Departments of Radiology and Pathology

    1998-02-01

    A 35-year-old woman presented with abdominal distension and a palpable liver mass. Ultrasonography and computed tomography revealed a large well-delineated liver mass with bilobar involvement. Based on autopsy and immunohistochemical findings, a final diagnosis of primary pleomorphic liver sarcoma with myogenic differentiation W established. Copyright (1998) Blackwell Science Pty Ltd 8 refs., 5 figs.

  4. Primary Breast Sarcoma. A Case Report

    Directory of Open Access Journals (Sweden)

    Lidia Torres Ajá

    2013-06-01

    Full Text Available Primary breast sarcoma is the least frequent non-epithelial malignant tumour, representing less than 1 % of all breast cancers. It has a dismal prognosis with the presence of early metastases, mainly in the lungs and bones. Survival is very poor at 5 years. A case of a 79 year-old female patient with a large ulcerated sarcoma in the left breast is presented. The patient was examined in an interdisciplinary consultation, and the presence of a stromal sarcoma of the breast without apparent visceral or bone metastases was confirmed by an aspiration biopsy with thick needle, excisional biopsy by paraffin and by immunohistochemical studies. The publication of this report has clinical interest for health professionals due to the rarity of the disease.

  5. Systematic analysis of a xenograft mice model for KSHV+ primary effusion lymphoma (PEL.

    Directory of Open Access Journals (Sweden)

    Lu Dai

    Full Text Available Kaposi's sarcoma-associated herpesvirus is the causative agent of primary effusion lymphoma (PEL, which arises preferentially in the setting of infection with human immunodeficiency virus (HIV. Even with standard cytotoxic chemotherapy, PEL continues to cause high mortality rates, requiring the development of novel therapeutic strategies. PEL xenograft models employing immunodeficient mice have been used to study the in vivo effects of a variety of therapeutic approaches. However, it remains unclear whether these xenograft models entirely reflect clinical presentations of KSHV(+ PEL, especially given the recent description of extracavitary solid tumor variants arising in patients. In addition, effusion and solid tumor cells propagated in vivo exhibit unique biology, differing from one another or from their parental cell lines propagated through in vitro culture. Therefore, we used a KSHV(+ PEL/BCBL-1 xenograft model involving non-obese diabetic/severe-combined immunodeficient (NOD/SCID mice, and compared characteristics of effusion and solid tumors with their parent cell culture-derived counterparts. Our results indicate that although this xenograft model can be used for study of effusion and solid lymphoma observed in patients, tumor cells in vivo display unique features to those passed in vitro, including viral lytic gene expression profile, rate of solid tumor development, the host proteins and the complex of tumor microenvironment. These items should be carefully considered when the xenograft model is used for testing novel therapeutic strategies against KSHV-related lymphoma.

  6. Primary Thyroid Sarcoma: A Systematic Review.

    Science.gov (United States)

    Surov, Alexey; Gottschling, Sebastian; Wienke, Andreas; Meyer, Hans Jonas; Spielmann, Rolf Peter; Dralle, Henning

    2015-10-01

    Different types of malignant tumors can occur within the thyroid. Primary cancer is the most common type of thyroid malignancy. Non-epithelial malignancies can also arise within the thyroid. The aim of the present study was to analyze clinical and radiological characteristics of reported primary thyroid sarcomas (PTS), based on a large sample of cases. The PubMed database was screened for articles from between 1990 and 2014. Overall, 86 articles with 142 patients were identified. Ultrasound evaluation was reported for 36 patients. Data regarding computed tomography of the neck were available for 29 cases. Magnetic resonance imaging was performed for eight patients. The following data were retrieved for the identified sarcomas: localization, size, homogeneity, internal texture, and margin characteristics. In most cases, PTS occurred in patients over 40 years of age, with a peak incidence for the group aged 60-79 years. Angiosarcoma was diagnosed in 29 cases (20.4%), followed by malignant hemangioendothelioma (n=23, 16.3%), malignant fibrous histiocytoma (n=20, 14.1%), leiomyosarcoma (n=16, 11.3%), and fibrosarcoma (n=13, 9.2%). In most patients (n=113, 79.6%), PTS manifested clinically as a painless goiter. On ultrasound, PTS were predominantly mixed hypo-to-hyperechoic in comparison to the normal thyroid tissue. On non-contrast computed tomography, most sarcomas were inhomogeneous hypo-to-hyperdense. On post-contrast magnetic resonance images, most sarcomas showed marked non-homogenous enhancement. In 26.8%, infiltration of the adjacent organs was seen. The trachea or esophagus was affected more frequently in patients with malignant histiocytoma and liposarcoma. Different strategies were used in the treatment of PTS. Our analysis provides clinical and radiological characteristics of PTS. The described features should be taken into consideration in the differential diagnosis of thyroid tumors.

  7. Primary mediastinal giant synovial sarcoma: A rare case report

    Directory of Open Access Journals (Sweden)

    Gaetano Rea

    2015-03-01

    Full Text Available Synovial sarcoma has been defined by the World Health Organization (WHO in 2002 as a type of mesenchymal tissue cell tumor that exhibits epithelial differentiation and represents the third most common soft-tissue sarcoma in adults, accounting for approximately 10% of soft-tissue sarcomas. To date, only few reports have focused on mediastinal synovial sarcoma imaging findings. Herein, we report a case of a 13 cm primary mediastinal giant synovial sarcoma, diagnosed in a 56-year-old patient admitted in our Department of Radiology with a six-month history of dyspnea and back pain.

  8. Adjuvant chemotherapy for primary cardiac sarcomas: the IGR experience.

    OpenAIRE

    Llombart-Cussac, A.; Pivot, X; Contesso, G; Rhor-Alvarado, A.; Delord, J P; Spielmann, M.; Türsz, T.; Le Cesne, A.

    1998-01-01

    The effect of additional treatments after surgery in patients with primary cardiac sarcoma (PCS) remains unknown. The present study aims to evaluate the benefit of chemotherapy in patients with non-metastatic cardiac sarcomas after optimal resection. Between October 1979 and December 1995, 15 patients with a median age of 45 (range 16-66) and a resected primary cardiac sarcoma [angiosarcoma (six), malignant fibrous histiocytoma (three), leiomyosarcoma (two), rhabdomyosarcoma (two), liposarcom...

  9. Primary Kaposi sarcoma of the subcutaneous tissue

    Directory of Open Access Journals (Sweden)

    Dezube Bruce J

    2008-09-01

    Full Text Available Abstract Background Involvement of the subcutis by Kaposi sarcoma (KS occurs primarily when cutaneous KS lesions evolve into deep penetrating nodular tumors. Primary KS of the subcutaneous tissue is an exceptional manifestation of this low-grade vascular neoplasm. Case presentation We present a unique case of acquired immune deficiency syndrome (AIDS-associated KS manifesting primarily in the subcutaneous tissue of the anterior thigh in a 43-year-old male, which occurred without overlying visible skin changes or concomitant KS disease elsewhere. Radiological imaging and tissue biopsy confirmed the diagnosis of KS. Conclusion This is the first documented case of primary subcutaneous KS occurring in the setting of AIDS. The differential diagnosis of an isolated subcutaneous lesion in an human immunodeficiency virus (HIV-infected individual is broad, and requires both imaging and a histopathological diagnosis to guide appropriate therapy.

  10. Primary extraosseous Ewing sarcoma of the orbit.

    Science.gov (United States)

    Alio, Jorge L; Sales-Sanz, Marco; Vaz, Maria A; Barrancos, Constanza; Reguero, Maria E; Diamantopoulus, Jorge; Poveda, Pedro

    2013-01-01

    A 40-year-old man presented with painless, progressive vision loss and mild proptosis of the OD. CT revealed a right intraconal mass with slight penetration of the optic canal not contiguous with any bony structure. Incisional biopsy through a transfrontal orbitotomy revealed a diffuse growth of homogeneous, small, round cells. Immunohistochemical stains were positive for vimentin and MIC2 (CD99), and the translocation at EWS gene (22q12) was detected. Metastatic workup and a full-body bone scan were negative, confirming primary orbital extraosseous Ewing sarcoma. The patient received neoadjuvant chemotherapy and an orbital exenteration with preservation of eyelids and conjunctiva. He also received adjuvant chemotherapy and local radiotherapy, and he has remained disease-free for almost 3 years.

  11. The surprising outcome of a giant primary mediastinal synovial sarcoma treated with neoadjuvant chemotherapy.

    Science.gov (United States)

    Balieiro, Marcos Alexandre; Lopes, Agnaldo José; Costa, Bruno Pinheiro; Veras, Gustavo Perissé Moreira; Perelson, Paulo Sergio; Acatauassú Nunes, Rodolfo; Saito, Eduardo Haruo

    2013-02-01

    There are only a few cases of primary mediastinal synovial sarcoma in the literature. Normally, they do not respond well to chemotherapy. In our case, a 30-year-old patient was admitted due to thoracic pain, dyspnea, orthopnea, cough, hoarseness and weight loss over a 3-month period as well as a dramatic worsening a week before the admission. A chest radiography showed a completely white left hemithorax and contralateral mediastinal shift; in addition, a chest tomography revealed a giant heterogeneous mediastinal mass, lung atelectasia and a small pleural effusion. The patient was submitted to Chamberlain procedure (biopsy) under local anesthesia and the diagnosis of a synovial sarcoma was obtained after immunohistochemical analysis. Due to his poor general condition, he received chemotherapy first, with a dramatic response, after what, the mass that had been reduced was removed surgically. After a 5-year- follow-up period there are no signs of disease recurrence.

  12. Giant primary synovial sarcoma of the anterior mediastinum: A case ...

    African Journals Online (AJOL)

    2015-06-11

    Jun 11, 2015 ... Primary synovial sarcoma is a very rare tumor of the mediastinum, which is unreported in the entire ... and cytogenetics are required to therapeutic management ... off the left upper lung zone and resected from the anterior.

  13. Sarcomas cutâneos primários Primary cutaneous sarcomas

    Directory of Open Access Journals (Sweden)

    Luiz Fernando Fróes Fleury Jr

    2006-06-01

    Full Text Available Os sarcomas com apresentação cutânea primária são tumores raros e de grande heterogeneidade histológica. Com a evolução da oncologia cutânea e da cirurgia dermatológica, os dermatologistas têm sido cada vez mais requisitados para o diagnóstico e orientação terapêutica de tumores menos freqüentes. Este artigo de revisão analisa os sarcomas cutâneos primários observando suas características clínicas, etiopatogênicas e histológicas, bem como aspectos do tratamento e evolução. Enfatiza os sarcomas de maior relevância para o dermatologista, como angiossarcoma, dermatofibrossarcoma protuberans, fibroxantoma atípico, leiomiossarcoma, lipossarcoma, tumor maligno de bainha de nervo periférico e sarcoma epitelióide. O sarcoma de Kaposi não é abordado devido a suas características individuais específicas.Soft tissue tumors represent a heterogeneous group of mesenchymal and neural lesions. The cutaneous presentation of these tumours is rare. With the evolution of dermatologic surgery and cutaneous oncology, dermatologists have emerged as specialists for skin cancer management. This article reviews primary cutaneous sarcomas with particular emphasis on the epidemiologic, clinical, and histological features of diagnosis, as well as treatment modalities and prognosis. The most frequent cutaneous sarcomas were reviewed, including angiosarcoma, dermatofibrosarcoma protuberans, atypical fibroxanthoma, leiomyosarcoma, liposarcoma, malignant nerve sheath tumor, and epithelioid sarcoma. Kaposi's sarcoma, due to specific characteristics, was omitted from this review.

  14. Primary granulocytic sarcoma of the ovary.

    Science.gov (United States)

    Sreejith, G; Gangadharan, V P; Elizabath, K A; Preetha, S; Chithrathara, K

    2000-06-01

    Granulocytic sarcomas are rare extramedullary tumors of malignant myeloid precursor cells. Exceedingly rare in childhood, it commonly involves skin, lymph nodes, bone, and the spine. Ovarian involvement is rare. It can arise de novo, precede the development of acute nonlymphocytic leukemia, or be the sole manifestation of relapse. We describe a 26-year-old woman with granulocytic sarcoma of the ovary without any hematologic disorder.

  15. AIDS-related primary Kaposi sarcoma of the nasopharynx.

    Science.gov (United States)

    Çelenk, Fatih; Yilmaz, Metin; Asal, Korhan; Ekinci, Özgür; Tokgöz, Nil

    2011-06-01

    Primary nasopharyngeal Kaposi sarcoma is extremely rare, as only 1 case has been previously reported in the literature. We report a new case, which occurred in a 37-year-old man with a known history of acquired immune deficiency syndrome (AIDS). The patient presented with complaints of recurrent epistaxis and postnasal hemorrhage. Endoscopic examination detected a bluish, smooth, firm, nonpulsatile mass in the nasopharyngeal wall. Histopathologic findings on biopsy were consistent with Kaposi sarcoma. The tumor was successfully treated with radiotherapy. Kaposi sarcoma should be considered in the differential diagnosis of any AIDS patient who presents with recurrent unilateral nasal bleeding.

  16. Primary Pericardial Sarcoma with Right Atrial Invasion and Multiple Bilateral Pulmonary Metastases in a Patient with Hereditary Nonpolyposis Colorectal Cancer

    Directory of Open Access Journals (Sweden)

    Eugene Wong

    2016-01-01

    Full Text Available Primary tumours originating from the pericardium are extremely rare. Previous studies have reported that these tumours account for only 6.7–12.8% of all mediastinal tumours with an overall prevalence of 0.001% to 0.007%. The majority of these tumours are benign lipomas or pericardial cysts. The most common pericardial malignancy is mesothelioma. Sarcomas are soft-tissue mesenchymal malignancies originating from various parts of the body but are extremely rare in this area. We report a case of a 52-year-old female who was diagnosed with a primary sarcoma with rhabdoid differentiation originating from the pericardium. The patient presented to her GP with a four-week history of progressive dyspnea and chest pain on exertion. Chest X-Ray demonstrated a prominent pericardial effusion and suspicious chest and pericardial lesions. Biopsies of the effusion and primary tumour identified on FDG/PET scans revealed the diagnosis of primary undifferentiated sarcoma. On thoracotomy, it was noted that the tumour had invaded the right atrium; therefore, pericardial window was aborted and a drain inserted instead. The patient was then started on chemotherapy; however, progression soon occurred and the patient died within 4 months, suggesting there is urgent need for efficacious treatments for sarcomatous lesions.

  17. Primary Occipital Ewing’s Sarcoma with Subsequent Spinal Seeding

    Directory of Open Access Journals (Sweden)

    Ali Alqahtani

    2017-01-01

    Full Text Available Ewing’s sarcoma is a primary bone cancer that mainly affects the long bones. This malignancy is particularly common in pediatric patients. Primary cranial involvement accounts for 1% of cases, with occipital involvement considered extremely rare. In this case study, primary occipital Ewing’s sarcoma with a posterior fossa mass and subsequent relapse resulting in spinal seeding is reported. A 3-year-old patient presented with a 1-year history of left-sided headaches, localized over the occipital bone with progressive torticollis. Computed tomography (CT imaging showed a mass in the left posterior fossa compressing the brainstem. The patient then underwent surgical excision followed by adjuvant chemoradiation therapy. Two years later, the patient presented with severe lower back pain and urinary incontinence. Whole-spine magnetic resonance imaging (MRI showed cerebrospinal fluid (CSF seeding from the L5 to the S4 vertebrae. Primary cranial Ewing’s sarcoma is considered in the differential diagnosis of children with extra-axial posterior fossa mass associated with destructive permeative bone lesions. Although primary cranial Ewing’s sarcoma typically has good prognosis, our patient developed metastasis in the lower spine. Therefore, with CNS Ewing’s sarcoma, screening of the entire neural axis should be taken into consideration for early detection of CSF seeding metastasis in order to decrease the associated morbidity and mortality.

  18. Primary pulmonary synovial sarcoma: a rare case report

    Directory of Open Access Journals (Sweden)

    Sumalatha Kasturi

    2014-08-01

    Full Text Available Primary lung sarcoma is an extremely rare tumor, accounting for less than 0.5% of all lung tumors. Histological subtypes are differentiated on the basis of immunohistochemical markers, such as vimentin, desmin, actin, CD99, and epithelial membrane antigen. A 50-year-old male presented with progressively increasing shortness of breath with cough for 2 months. On Contrast Enhanced Computed Tomography (CECT of thorax a large heterogeneous mass with multiple areas of necrosis, occupying almost whole of left hemithorax was seen. CT-guided Fine Needle Aspiration Cytology (FNAC revealed spindle cell neoplasm. Histopathological examination revealed a spindle cell sarcoma. On immunohistochemistry the tumor cells expressed both epithelial membrane antigen and vimentin. Hence, final impression from immunohistochemistry was primary monophasic synovial sarcoma of lung. [Int J Res Med Sci 2014; 2(4.000: 1729-1731

  19. Genomic analysis of xCT-regulatory network in KSHV+ primary effusion lymphomas

    Directory of Open Access Journals (Sweden)

    Zhiqiang Qin

    2016-06-01

    Full Text Available Kaposi's sarcoma-associated herpesvirus (KSHV is the etiological agent of primary effusion lymphoma (PEL, a rapidly progressing malignancy mostly arising in HIV-infected patients Chen et al. (2007 [1]. Even under conventional chemotherapy, PEL continues to portend nearly 100% mortality within several months, which urgently requires novel therapeutic strategies. We have previously demonstrated that targeting xCT, an amino acid transporter for cystine/glutamate exchange, induces significant PEL cell apoptosis through regulation of multiple host and viral factors [2]. More importantly, one of xCT selective inhibitors, Sulfasalazine (SASP, effectively prevents PEL tumor progression in an immune-deficient xenograft model [2]. In the current study, we use Illumina microarray to explore the profile of genes altered by SASP treatment within 3 KSHV+ PEL cell-lines, and discover that many genes involved in oxidative stress/antioxidant defense system, apoptosis/anti-apoptosis/cell death, and cellular response to unfolded proteins/topologically incorrect proteins are potentially regulated by xCT Dai et al. (2015 [3]. The microarray original data have been submitted to Gene Expression Omnibus (GEO database (Accession number: GSE65418.

  20. Primary Pulmonary Synovial Sarcoma: A Very Rare Presentation

    Directory of Open Access Journals (Sweden)

    Ekrem Cengiz Seyhan

    2014-01-01

    Full Text Available Synovial sarcoma (SS is a rare tumor originating from mesenchymal tissue and accounting for approximately 5–10% of all soft tissue sarcomas. A rare case of primary pulmonary SS in an asymptomatic 18-year-old man admitted to our hospital for investigation of a 6 × 6.5 cm, oval-shaped, well-delineated pleural based peripheral mass in the left lower lobe in his thorax CT is presented. Left lower lobectomy was done. Immunohistochemically, tumor cells were positive for cytokeratin, epithelial membrane antigen (EMA, and vimentin so that the histopathological diagnosis was compatible with biphasic spindle cell type SS in the lung.

  1. Clear cell sarcoma: A case mimicking primary cutaneous malignant melanoma

    Directory of Open Access Journals (Sweden)

    Rodriguez-Martin M

    2009-01-01

    Full Text Available Clear cell sarcoma (CCS is a recently described variant of sarcoma characterized by prominent clear cells showing features similar to clear cell melanoma. This neoplasm was first described by Dr. Franz M. Erzinger. Primary CCS usually arises in deeper soft tissues, in association with fascia, tendons, or aponeuroses. Characteristic translocation t(12;22 (q13;q12 has been considered pathognomonic for CCS. Prognosis is related to tumor size. An early recognition and initial radical surgery is the key to a favourable outcome. We present a patient with an unusual neoplasm that resembled malignant melanoma.

  2. Primary Synovial Sarcoma of the Kidney

    Directory of Open Access Journals (Sweden)

    Takashi Kawahara

    2009-10-01

    Full Text Available The case was a 40-year-old female. She visited a local doctor with a chief complaint of right side abdominal pain. A right kidney tumor measuring 10 cm in diameter was observed in an abdominal Computed Tomography (CT scan. Based on the CT image, the possibility of angiomiolipoma (AML could not be ruled out, but a high maximum standardized uptake value (SUVmax of 7.8 was observed in a Positron Emission Tomography CT (PET-CT scan and there was a possibility of malignancy. We therefore performed a transperitoneal right radial nephrectomy. Although adhesion of the tumor to the duodenum and the inferior vena cava was observed, it was possible to perform an excision. The tumor accounted for a large proportion of the excised kidney; the surrounding areas had taken on a cyst-like structure, and the interior comprised grayish brittle tissue exhibiting solid growth. Histologically, gland-like and cyst-like structures composed of cylindrical cuboidal cells and mainly characterized by the solid growth of short fusiform-shaped and oval-shaped basophilic cells were observed, and we believed it was a synovial sarcoma. There were no malignant findings in the adrenal gland. There have been approximately 30 reported cases around the world of synovial sarcoma that developed in the kidney, and we herein report this case with bibliographic considerations.

  3. Targeting HGF/c-MET induces cell cycle arrest, DNA damage, and apoptosis for primary effusion lymphoma.

    Science.gov (United States)

    Dai, Lu; Trillo-Tinoco, Jimena; Cao, Yueyu; Bonstaff, Karlie; Doyle, Lisa; Del Valle, Luis; Whitby, Denise; Parsons, Chris; Reiss, Krzysztof; Zabaleta, Jovanny; Qin, Zhiqiang

    2015-12-24

    Kaposi sarcoma-associated herpesvirus (KSHV) is a principal causative agent of primary effusion lymphoma (PEL) with a poor prognosis in immunocompromised patients. However, it still lacks effective treatment which urgently requires the identification of novel therapeutic targets for PEL. Here, we report that the hepatocyte growth factor (HGF)/c-MET pathway is highly activated by KSHV in vitro and in vivo. The selective c-MET inhibitor, PF-2341066, can induce PEL apoptosis through cell cycle arrest and DNA damage, and suppress tumor progression in a xenograft murine model. By using microarray analysis, we identify many novel genes that are potentially controlled by HGF/c-MET within PEL cells. One of the downstream candidates, ribonucleoside-diphosphate reductase subunit M2 (RRM2), also displays the promising therapeutic value for PEL treatment. Our findings provide the framework for development of HGF/c-MET-focused therapy and implementation of clinical trials for PEL patients.

  4. One Case about Primary Mediastinal Primary Mediastinal Tumor 
with Mediastinal Sarcoma and Literature Review

    Directory of Open Access Journals (Sweden)

    Jinchen DU

    2016-09-01

    Full Text Available Primary mediastinal seminoma is a kind of germ cell malignancy outside the gonads, and it’s rarer with sarcoma component. This disease which has no special clinical manifestations and imaging characteristics is difficult to identify with other mediastinal tumors and mediastinal type lung cancer. This paper reported a case of primary mediastinal seminoma with mediastinal sarcoma. Through the analysis of the diagnosis and treatment process in this patient, we will make a comprehensive review of the disease.

  5. Primary synovial sarcoma of the abdominal wall: A case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Alsaif H Saif

    2008-01-01

    Full Text Available Synovial sarcoma is a malignant mesenchymal neoplasm which commonly occurs in the extremities of adults, in close association with joint capsules, tendon sheaths, bursae and fascial structures. Only a few cases of synovial sarcoma occurring in the abdominal wall have been reported. A case of a primary synovial sarcoma arising from the anterior abdominal wall fascial aponeurosis is presented.

  6. Cytology of Pericardial Effusion due to Malignancy

    Directory of Open Access Journals (Sweden)

    Kalogeraki Alexandra

    2016-09-01

    Full Text Available Background. Malignant pericardial effusion occurs in one tenth of all cancers. It is a very serious disorder that is mainly a secondary process due to metastasis because primary neoplasms of the pericardium such as mesotheliomas, sarcomas being exceedingly rare.

  7. An unusual case of posttransplant peritoneal primary effusion lymphoma with T-cell phenotype in a HIV-negative female, not associated with HHV-8.

    Science.gov (United States)

    Venizelos, Ioannis; Tamiolakis, Demetrio; Lambropoulou, Maria; Nikolaidou, Sylva; Bolioti, Sophia; Papadopoulos, Hlias; Papadopoulos, Nikolas

    2005-01-01

    Primary effusion lymphoma (PEL) is a recently individualized form of non-Hodgkin's lymphoma (WHO classification) that mainly develops in HIV infected males, more frequently in homosexuals and advanced stages of the disease (total CD4+ lymphocyte count below 100-200/microL). Occasionally, it appears in other immunodepressive states (such as solid organs transplant period) and even, although very rarely, in immunocompetent patients. From a pathogenetic point of view, PEL has been related to Kaposi's sarcoma associated herpes virus (also named human herpesvirus 8, HHV-8), an etiological factor of Kaposi's sarcoma. The relative infrequency of this disease, the absence of wide casuistics allowing a better characterization, and its unfavorable outcome support the need of a deeper knowledge. We present here the clinical-biological findings of a patient, HIV seronegative, who was diagnosed with peritoneal PEL of T-cell origin, and not HHV-8-associated, five years after renal transplantation.

  8. Sangivamycin induces apoptosis by suppressing Erk signaling in primary effusion lymphoma cells

    Energy Technology Data Exchange (ETDEWEB)

    Wakao, Kazufumi [Department of Biotechnology, Interdisciplinary Graduate School of Medicine and Engineering, University of Yamanashi, Kofu-shi 400-8511 (Japan); Watanabe, Tadashi [Department of Cell Biology, Kyoto Pharmaceutical University, Misasagi-Shichonocho 1, Yamashinaku, Kyoto 607-8412 (Japan); Takadama, Tadatoshi; Ui, Sadaharu [Department of Biotechnology, Interdisciplinary Graduate School of Medicine and Engineering, University of Yamanashi, Kofu-shi 400-8511 (Japan); Shigemi, Zenpei; Kagawa, Hiroki [Department of Cell Biology, Kyoto Pharmaceutical University, Misasagi-Shichonocho 1, Yamashinaku, Kyoto 607-8412 (Japan); Higashi, Chizuka; Ohga, Rie; Taira, Takahiro [Department of Molecular Cell Biology, Faculty of Medicine, University of Yamanashi, Chuoh-shi 409-3898 (Japan); Fujimuro, Masahiro, E-mail: fuji2@mb.kyoto-phu.ac.jp [Department of Cell Biology, Kyoto Pharmaceutical University, Misasagi-Shichonocho 1, Yamashinaku, Kyoto 607-8412 (Japan)

    2014-02-07

    Highlights: • Sangivamycin induces the apoptosis of B cell lymphoma PEL cells. • Sangivamycin suppresses Erk signaling by inhibiting Erk phosphorylation in PEL cells. • The activation of Erk signaling is essential for PEL cell survival. • Sangivamycin induces the apoptosis of PEL cells without production of progeny virus. • Sangivamycin may serve as a novel drug for the treatment of PEL. - Abstract: Sangivamycin, a structural analog of adenosine and antibiotic exhibiting antitumor and antivirus activities, inhibits protein kinase C and the synthesis of both DNA and RNA. Primary effusion lymphoma (PEL) is an aggressive neoplasm caused by Kaposi’s sarcoma-associated herpesvirus (KSHV) in immunosuppressed patients and HIV-infected homosexual males. PEL cells are derived from post-germinal center B cells, and are infected with KSHV. Herein, we asked if sangivamycin might be useful to treat PEL. We found that sangivamycin killed PEL cells, and we explored the underlying mechanism. Sangivamycin treatment drastically decreased the viability of PEL cell lines compared to KSHV-uninfected B lymphoma cell lines. Sangivamycin induced the apoptosis of PEL cells by activating caspase-7 and -9. Further, sangivamycin suppressed the phosphorylation of Erk1/2 and Akt, thus inhibiting activation of the proteins. Inhibitors of Akt and MEK suppressed the proliferation of PEL cells compared to KSHV-uninfected cells. It is known that activation of Erk and Akt signaling inhibits apoptosis and promotes proliferation in PEL cells. Our data therefore suggest that sangivamycin induces apoptosis by inhibiting Erk and Akt signaling in such cells. We next investigated whether sangivamycin, in combination with an HSP90 inhibitor geldanamycin (GA) or valproate (valproic acid), potentiated the cytotoxic effects of the latter drugs on PEL cells. Compared to treatment with GA or valproate alone, the addition of sangivamycin enhanced cytotoxic activity. Our data thus indicate that

  9. Primary Histiocytic Sarcoma of the Central Nervous System

    Science.gov (United States)

    So, Hoonsub; Kim, Sun A; Yoon, Dok Hyun; Khang, Shin Kwang; Hwang, Jihye; Suh, Chong Hyun; Suh, Cheolwon

    2015-01-01

    Histiocytic sarcoma is a type of lymphoma that rarely involves the central nervous system (CNS). Its rarity can easily lead to a misdiagnosis. We describe a patient with primary CNS histocytic sarcoma involving the cerebral hemisphere and spinal cord, who had been initially misdiagnosed as demyelinating disease. Two biopsies were necessary before a correct diagnosis was made. A histologic examination showed bizarre shaped histiocytes with larger nuclei and nuclear atypia. The cells were positive for CD68, CD163, and S-100 protein. As a resection was not feasible due to multifocality, he was treated with highdose methotrexate, but showed no response. As a result, he was switched to high dose cytarabine; but again, showed no response. The patient died 2 months from the start of chemotherapy and 8 months from the onset of symptoms. Since few patients with this condition have been described and histopathology is difficult to diagnose, suspicion of the disease is essential. PMID:25345462

  10. Breast Carcinoma Cells in Primary Tumors and Effusions Have Different Gene Array Profiles

    Directory of Open Access Journals (Sweden)

    Sophya Konstantinovsky

    2010-01-01

    Full Text Available The detection of breast carcinoma cells in effusions is associated with rapidly fatal outcome, but these cells are poorly characterized at the molecular level. This study compared the gene array signatures of breast carcinoma cells in primary carcinomas and effusions. The genetic signature of 10 primary tumors and 10 effusions was analyzed using the Array-Ready Oligo set for the Human Genome platform. Results for selected genes were validated using PCR, Western blotting, and immunohistochemistry. Array analysis identified 255 significantly downregulated and 96 upregulated genes in the effusion samples. The majority of differentially expressed genes were part of pathways involved in focal adhesion, extracellular matrix-cell interaction, and the regulation of the actin cytoskeleton. Genes that were upregulated in effusions included KRT8, BCAR1, CLDN4, VIL2, while DCN, CLDN19, ITGA7, and ITGA5 were downregulated at this anatomic site. PCR, Western blotting, and immunohistochemistry confirmed the array findings for BCAR1, CLDN4, VIL2, and DCN. Our data show that breast carcinoma cells in primary carcinomas and effusions have different gene expression signatures, and differentially express a large number of molecules related to adhesion, motility, and metastasis. These differences may have a critical role in designing therapy and in prognostication for patients with metastatic disease localized to the serosal cavities.

  11. Epithelioid sarcoma mimicking a primary osseous multifocal scapula lesion

    Energy Technology Data Exchange (ETDEWEB)

    Nakashima, Hiroatsu; Sugiura, Hideshi; Nishida, Yoshihiro; Yamada, Yoshihisa [Department of Orthopaedic Surgery, Nagoya University School of Medicine (Japan); Katagiri, Hirohisa [Department of Orthopaedic Surgery, Nagoya Memorial Hospital, Nagoya (Japan); Yonekawa, Masahiro [Department of Orthopaedic Surgery, Aichi Prefectural Hospital, Nagoya (Japan)

    2002-07-01

    This report describes a case of bone involvement by epithelioid sarcoma, which on imaging had the appearance of a primary intraosseous lesion of the scapula. The tumor initially presented as a subcutaneous nodule which was mistakenly diagnosed as ''fibrosis'' following initial resection. The lesion recurred locally and after several resections presented with several ulcerated subcutaneous nodules, at which time all imaging studies were performed. The patient was treated with en bloc upper humeral interscapulothoracic resection and shoulder reconstruction. Two years after the operation the patient is alive without local recurrence or metastasis. (orig.)

  12. Primary pulmonary/pleural melanoma in a 13 year-old presenting as pleural effusion.

    Science.gov (United States)

    Baniak, Nick; Podberezin, Mark; Kanthan, Selliah C; Kanthan, Rani

    2017-02-01

    Melanoma in children, adolescents, and young adults is uncommon and reported almost exclusively as cutaneous melanoma. Melanoma presenting as a pleural effusion is very rare in adults and not reported in the pediatric population. Additionally, primary pulmonary melanoma is overall very rare and undocumented in pediatric patients. Furthermore, the distinction between a primary pulmonary/pleural melanoma versus a regressed cutaneous melanoma with pulmonary/pleural metastases remains extremely challenging. We discuss a case of a previously healthy 13-year-old girl that presented with a left-sided pleural effusion. Investigations revealed a large mediastinal mass, left-sided pleural and pulmonary nodules, a sacral mass, and bone marrow infiltration. The neoplasm was subsequently diagnosed by morphology and immunocytochemistry with histological correlation as malignant melanoma. As no mucosal, eye, or cutaneous lesions were identified, we deliberate the likelihood of a regressed cutaneous melanoma with metastases versus primary pulmonary/pleural melanoma with pleural effusion and discuss its diagnostic approach.

  13. Primary Cardiac Synovial Sarcoma: A Case Report and Brief Review of the Literature

    Directory of Open Access Journals (Sweden)

    Brian Boulmay

    2007-01-01

    Full Text Available Synovial sarcoma comprises approximately 10% of all soft tissue sarcoma diagnoses; a primary synovial sarcoma of the myocardium is exceedingly rare. There have been very few cases reported in the literature thus far. With the identification of the characteristic and diagnostic chromosomal abnormality t(X;18, this may become an increasingly recognized entity. Our report adds to the limited published cases of primary cardiac synovial sarcoma with the characteristic t(X;18. Further elucidation of the effects of this translocation on the cell cycle may lead to directed therapies in the future.

  14. Rapidly progressive course of primary renal synovial sarcoma: Case report

    Directory of Open Access Journals (Sweden)

    Marković-Lipkovski Jasmina

    2013-01-01

    Full Text Available Introduction. Primary kidney sarcoma, especially synovial sarcoma (SS, is a very rare neoplasm. Pre-operative signs and symptoms are very similar to renal cell carcinoma, therefore, the proper diagnosis is very difficult and usually made after nephrectomy. This is a case report of primary renal SS. Case Outline. A 38-year-old man presented with a history of fever and hematuria, and right flank pain 3 weeks ago. Abdominal computerized tomography revealed a heterogeneous well-marginated soft tissue mass arising in the lower part of the right kidney. Right nephrectomy was performed. A cystic tumor of 120x85 mm in size with soft solid growth, and with the extensive areas of hemorrhage and necrosis was seen on gross examination. Histopathology revealed a neoplasm composed of solid monomorphic sheets of spindle cells. Immunohistochemistry showed tumor cells strongly positive for BCL2, CD99, CD56 and vimentin, and focally positive for epithelial membrane antigen (EMA. The histological diagnosis of primary renal SS was based on morphology and immunohistochemistry. FISH analysis and RT-PCR was carried out on formalin-fixed paraffin-embedded tissue sections. The molecular analysis demonstrated translocation of SYT gene on chromosome 18 and SSX2 gene on chromosome X. The findings were consistent with diagnosis of SS. Conclusion. Our case shows that histopathological diagnosis of primary kidney SS, although difficult, is possible to be made on the basis of morphological and immunohistochemical analysis. However, this diagnosis should be corroborated by molecular techniques confirming SYT-SSX translocation on chromosome 18 and chromosome X. Here we present visceral monophasic SS with aggressive clinical course and poor outcome. [Projekat Ministarstva nauke Republike Srbije, br. OI 175047

  15. Primary synovial sarcomas of the mediastinum: a clinicopathologic, immunohistochemical, and ultrastructural study of 15 cases.

    Science.gov (United States)

    Suster, Saul; Moran, Cesar A

    2005-05-01

    A series of 15 cases of primary mediastinal neoplasms displaying histopathologic, immunohistochemical, and ultrastructural features of synovial sarcoma is presented. The patients' ages ranged from 3 to 83 years, with a male-to-female ratio of 2:1. Nine cases presented as anterior mediastinal masses with chest pain, shortness of breath, and pleural effusion, and 6 cases were in paravertebral location in the posterior mediastinum and presented with neck or back pain and pleural effusion. The tumors measured from 5 to 20 cm in greatest diameter and showed a tan white, soft to rubbery cut surface with areas of hemorrhage and necrosis and foci of gelatinous material. Four cases showed areas of cystic degeneration. In 7 cases, the tumors were well circumscribed; in 6 cases, the tumors grossly invaded the pleura, pericardium, heart, great vessels, chest wall, rib, and vertebra. Histologically, 5 cases displayed a biphasic growth pattern, with well-formed glandular elements admixed with a monotonous spindle cell population. Ten cases were exclusively composed of a monotonous atypical spindle cell proliferation. Immunohistochemical studies showed focal positivity of the tumor cells for cytokeratin and/or epithelial membrane antigen, and strong positivity for vimentin and bcl-2 in the spindle cells in all cases studied (10 of 10). Eight cases also showed focal positivity for CD99. Electron microscopic examination in 5 cases showed oval to spindle tumor cells with closely apposed cell membranes, abundant cytoplasmic intermediate filaments and rough endoplasmic reticulum, and immature desmosome-type cell junctions. Ten patients were treated by complete surgical excision and two by partial excision followed by radiation therapy. In 4 patients, the tumors were inoperable and treated with radiation therapy only. Clinical follow-up was available in 5 patients and showed local recurrence with metastases to lung, lymph nodes, and epidural space from 1 to 3 years in 4 cases and liver

  16. Pericardial Effusion with Cardiac Tamponade as a Form of Presentation of Primary Hypothyroidism

    Directory of Open Access Journals (Sweden)

    Acir Rachid

    2002-06-01

    Full Text Available The authors describe a case of pericardial effusion accompanied by cardiac tamponade caused by primary hypothyroidism. Diagnosis was made by exclusion, because other causes of cardiac tamponade are more frequent. Emergency treatment of cardiac tamponade is pericardiocentesis (with possible pericardial window, and, after stabilization, performance of hormonal reposition therapy with L-thyroxin.

  17. Variation in gene expression patterns in effusions and primary tumors from serous ovarian cancer patients

    Directory of Open Access Journals (Sweden)

    Tropè Claes G

    2005-07-01

    Full Text Available Abstract Background While numerous studies have characterized primary ovarian tumors, little information is available regarding expression patterns of metastatic sites of this cancer. To define sets of genes that distinguish primary and metastatic ovarian tumors, we used cDNA microarrays to characterize global gene expression patterns in 38 effusions (28 peritoneal, 10 pleural and 8 corresponding primary ovarian tumors, and searched for associations between expression patterns and clinical parameters. Results We observed multidimensional variation in expression patterns among the cancers. Coordinate variation in expression of genes from two chromosomal regions, 8q and 19q, was seen in subsets of the cancers indicating possible amplifications in these regions. A set of 112 unique genes of known function was differentially expressed between primary tumors and effusions using supervised analysis. Relatively few differences were seen between effusions isolated from the pleural and peritoneal cavities or between effusions from patients diagnosed with stage III and stage IV cancers. A set of 84 unique genes was identified that distinguished high from lower grade ovarian cancers. The results were corroborated using immunocytochemistry, mRNA in situ hybridization, and immunoblotting. Conclusion The extensive variation in expression patterns observed underscores the molecular heterogeneity of ovarian cancer, but suggests a similar molecular profile for ovarian carcinoma cells in serosal cavities.

  18. Proteasome inhibitors induce apoptosis and reduce viral replication in primary effusion lymphoma cells

    Energy Technology Data Exchange (ETDEWEB)

    Saji, Chiaki [Faculty of Pharmaceutical Sciences, Hokkaido University, Kita-ku, Sapporo 060-0812 (Japan); Higashi, Chizuka; Niinaka, Yasufumi [Faculty of Medicine, University of Yamanashi, Chuoh-shi 409-3898 (Japan); Yamada, Koji [Faculty of Pharmaceutical Sciences, Hokkaido University, Kita-ku, Sapporo 060-0812 (Japan); Noguchi, Kohji [Faculty of Pharmacy, Keio University, 1-5-30 Shiba-koen, Minato-ku, Tokyo 105-8512 (Japan); Fujimuro, Masahiro, E-mail: fuji2@mb.kyoto-phu.ac.jp [Department of Cell Biology, Kyoto Pharmaceutical University, Misasagi-Shichonocho 1, Yamashinaku, Kyoto 607-8412 (Japan)

    2011-12-02

    Highlights: Black-Right-Pointing-Pointer Constitutive NF-{kappa}B signaling is essential for the survival and growth of PEL cells. Black-Right-Pointing-Pointer NF-{kappa}B signaling is upregulated by the proteasome-dependent degradation of I{kappa}B{alpha}. Black-Right-Pointing-Pointer Proteasome inhibitors suppress NF-{kappa}B signaling and induce apoptosis in PEL cells through stabilization of I{kappa}B{alpha}. Black-Right-Pointing-Pointer Proteasome inhibitors suppress viral replication in PEL cells during lytic KSHV infection. -- Abstract: Primary effusion lymphoma (PEL) is an aggressive neoplasm caused by Kaposi's sarcoma-associated herpesvirus (KSHV). This study provides evidence that proteasomal activity is required for both survival of PEL cells stably harboring the KSHV genome and viral replication of KSHV. We evaluated the cytotoxic effects of proteasome inhibitors on PEL cells. The proteasome inhibitors MG132, lactacystin, and proteasome inhibitor I dramatically inhibited cell proliferation and induced apoptosis of PEL cells through the accumulation of p21 and p27. Furthermore, proteasome inhibitors induced the stabilization of NF-{kappa}B inhibitory molecule (I{kappa}B{alpha}) and suppressed the transcriptional activity of NF-{kappa}B in PEL cells. The NF-{kappa}B specific inhibitor BAY11-7082 also induced apoptosis in PEL cells. The constitutive activation of NF-{kappa}B signaling is essential for the survival and growth of B cell lymphoma cells, including PEL cells. NF-{kappa}B signaling is upregulated by proteasome-dependent degradation of I{kappa}B{alpha}. The suppression of NF-{kappa}B signaling by proteasome inhibitors may contribute to the induction of apoptosis in PEL cells. In addition, proteasome activity is required for KSHV replication in KSHV latently infected PEL cells. MG132 reduced the production of progeny virus from PEL cells at low concentrations, which do not affect PEL cell growth. These findings suggest that proteasome

  19. Primary sarcomas of the central nervous system: UCSF experience (1985-2005

    Directory of Open Access Journals (Sweden)

    Tarık TİHAN

    2007-01-01

    Full Text Available Sarcomas constitute less than 2% of all cancers, and are a highly diverse group of neoplasms. Primary sarcomas of the central nervous system (CNS are even less common, and our experience is limited by lack of studies with sufficient size that can address the challenges in predicting behavior or management. It is critical to recognize the characteristics of these uncommon neoplasms and to develop better predictors for prognosis and behavior.We have conducted a search of the UCSF Department of Pathology and UCSF Cancer Center Registry databases for all primary CNS sarcomas that were diagnosed and treated between 1985 and 2005. Hemangiopericytomas were included, so were the solitary fibrous tumors due to their close association with the former. We excluded all cases of metastatic sarcomas, chordomas, sarcomatoid variants of all neuroepithelial neoplasms, Ewing’s sarcomas and other embryonal tumors. In addition, we have identified all soft tissue sarcomas diagnosed and treated during the same period. There were 43 primary CNS neoplasms that fulfilled the inclusion criteria. At the same time, we identified 1706 sarcomas primary to the soft tissue. Primary CNS sarcomas included 16 hemangiopericytomas, 15 chondrosarcomas, 3 solitary fibrous tumors, 3 osteosarcomas, 2 leiomyosarcomas, 2 undifferentiated sarcomas, 1 histiocytic sarcoma, and 1 fibrosarcoma. There was a distinctly higher frequency of hemangiopericytoma in the CNS compared to soft tissue. In addition, a group of low grade, parasagittal chondrosarcomas were noted for their highly indolent biological behavior. Unlike some previous series, our cohort was devoid of angiosarcoma and malignant fibrous histiocytoma. This study underscores the limitations of single institutional series, and highlights the value of multi-institutional studies to understand and better treat primary CNS sarcomas.

  20. Extracavitary manifestation of primary effusion lymphoma as a right atrial mass.

    Science.gov (United States)

    Marak, Creticus P; Ponea, Ana M; Shim, Chang; Shaheen, Shagufta; Guddati, Achuta K

    2013-01-01

    Primary effusion lymphoma (PEL) is a subset of large B cell lymphomas and has been mostly associated with human immunodeficiency virus infection. Rare cases have been reported in organ transplant recipients and chronic hepatitis C patients. It typically presents as an effusion in the pleural and pericardial spaces but rarely disseminates. However, involvement of the gastrointestinal tract, lymph nodes and bone marrow has been reported. Diagnosis is based on characteristic clinical, histopathological and immunohistochemical features. We present a case with a right atrial mass which tested positive for human herpes virus 8 (HHV-8), CD20, CD30 and lambda light chains and negative for CD138, kappa light chain, PAX5, Epstein-Barr virus, latent membrane protein 1, CD2, CD3, CD8 and CD56. Bilateral pleural effusions and pericardial effusions were noted which tested positive for HHV-8, CD30 and CD45. The patient responded well to the R-EPOCH regimen with complete resolution of the effusions and a significant decrease in the size of the right atrial mass. This case report illustrates the atypical manifestation of PEL as a right atrial mass.

  1. Extracavitary Manifestation of Primary Effusion Lymphoma as a Right Atrial Mass

    Directory of Open Access Journals (Sweden)

    Creticus P. Marak

    2013-02-01

    Full Text Available Primary effusion lymphoma (PEL is a subset of large B cell lymphomas and has been mostly associated with human immunodeficiency virus infection. Rare cases have been reported in organ transplant recipients and chronic hepatitis C patients. It typically presents as an effusion in the pleural and pericardial spaces but rarely disseminates. However, involvement of the gastrointestinal tract, lymph nodes and bone marrow has been reported. Diagnosis is based on characteristic clinical, histopathological and immunohistochemical features. We present a case with a right atrial mass which tested positive for human herpes virus 8 (HHV-8, CD20, CD30 and lambda light chains and negative for CD138, kappa light chain, PAX5, Epstein-Barr virus, latent membrane protein 1, CD2, CD3, CD8 and CD56. Bilateral pleural effusions and pericardial effusions were noted which tested positive for HHV-8, CD30 and CD45. The patient responded well to the R-EPOCH regimen with complete resolution of the effusions and a significant decrease in the size of the right atrial mass. This case report illustrates the atypical manifestation of PEL as a right atrial mass.

  2. Bilateral Pleural Effusions as an Initial Presentation in Primary Sjögren's Syndrome

    Science.gov (United States)

    Makimoto, Go; Asano, Michiko; Fujimoto, Nobukazu; Fuchimoto, Yasuko; Ono, Katsuichiro; Ozaki, Shinji; Taguchi, Koji; Kishimoto, Takumi

    2012-01-01

    Sjögren's syndrome (SS) is a systemic autoimmune disease characterized by sicca symptoms. Interstitial pulmonary fibrosis and tracheobronchial sicca are the most common symptoms of pulmonary involvement in primary SjS, and they are rarely accompanied by serositis such as pleuritis or pericarditis. We report a case of SS presenting initially with bilateral pleural effusions. A 63-year old man was admitted to our hospital with a one-month history of cough, dyspnea, and right chest pain. Chest-computed tomography revealed bilateral pleural effusions. Serum anti-SS-A antibody titer was 1 : 256. Ophthalmological examination revealed a positive Schirmer test. Lip biopsy showed atrophy and plasmacytic infiltration of the salivary gland. Corticosteroid treatment was initiated. Pleural effusions were almost completely resolved by day 30. The patient has not experienced any recurrence. PMID:23198246

  3. Bilateral Pleural Effusions as an Initial Presentation in Primary Sjögren’s Syndrome

    Directory of Open Access Journals (Sweden)

    Go Makimoto

    2012-01-01

    Full Text Available Sjögren’s syndrome (SS is a systemic autoimmune disease characterized by sicca symptoms. Interstitial pulmonary fibrosis and tracheobronchial sicca are the most common symptoms of pulmonary involvement in primary SjS, and they are rarely accompanied by serositis such as pleuritis or pericarditis. We report a case of SS presenting initially with bilateral pleural effusions. A 63-year old man was admitted to our hospital with a one-month history of cough, dyspnea, and right chest pain. Chest-computed tomography revealed bilateral pleural effusions. Serum anti-SS-A antibody titer was 1 : 256. Ophthalmological examination revealed a positive Schirmer test. Lip biopsy showed atrophy and plasmacytic infiltration of the salivary gland. Corticosteroid treatment was initiated. Pleural effusions were almost completely resolved by day 30. The patient has not experienced any recurrence.

  4. Primary Ewing's sarcoma of the vertebral column

    Energy Technology Data Exchange (ETDEWEB)

    Ilaslan, Hakan; Sundaram, Murali [Department of Radiology, Mayo Clinic, Ch2-290 200 First Street, SW, Rochester, 55905, MN (United States); Unni, K.Krishnan [Department of Laboratory Medicine and Pathology, Mayo Clinic, 200 First Street, SW, 55905, Rochester, MN (United States); Dekutoski, Mark B. [Department of Orthopedic Surgery, Mayo Clinic, 200 First Street, SW, 55905, Rochester, MN (United States)

    2004-09-01

    To determine the demographics, imaging findings, clinical symptoms, and prognosis of primary vertebral Ewing's sarcoma (PVES). A retrospective review of medical records and radiological studies of patients diagnosed with PVES from 1936 through 2001 in our institution and Department of Pathology consultation files was undertaken. Metastatic and soft tissue Ewing's sarcoma cases were excluded. From a total of 1,277 cases of Ewing's sarcoma, 125 (9.8%) had a primary vertebral origin. There were 48 females and 76 males. Patient ages ranged from 4 to 54 (mean 19.3, standard deviation 10.7, median 16) years. Vertebral column distribution was four cervical (3.2%), 13 thoracic (10.5%), 31 lumbar (25%), and 67 sacrum (53.2%). More than one vertebral segment was involved in ten cases (8%). Satisfactory imaging studies were available in 51 patients: 49 radiographs, 27 computerized tomography (CT), and 23 magnetic resonance imaging (MRI) studies. The majority of tumors were lytic (93%). Three cases were mixed lytic and sclerotic (6%) and one sclerotic. In the nonsacral spine, the majority of lesions (12/20) involved the posterior elements with extension into the vertebral body. Five cases were centered in the vertebral body with extension into the posterior elements. Two cases were limited to the posterior elements, and one case solely involved the vertebral body. Ala was the most frequently affected site in the sacrum (18/26). Spinal canal invasion was frequent (91%). Detailed clinical information was available in 53 patients. Duration of symptoms ranged from 1 to 30 (mean 7) months. Local pain was the first symptom and seen in all cases. Neurological deficits were present in 21 (40%) cases. All patients received radiation in various dosages; 70% additionally received chemotherapy. Twenty-five patients had surgery, and two patients received bone marrow transplantation. Forty-five patients had follow-up; the five-year disease-free survival probability is 0

  5. Sarcoma sinovial primário da orofaringe: relato de caso Primary synovial sarcoma of the oropharinx: a case report

    OpenAIRE

    Hugo Meister; Thaís C. Miranda; Manuel G. G. Nóbrega

    2003-01-01

    O sarcoma sinovial primário da cabeça e pescoço é um tumor raro. Em torno de 90 casos foram descritos na literatura. Relatamos um caso adicional ocorrido em um paciente jovem do sexo masculino comprometendo a orofaringe. O tumor apresentou típico crescimento bifásico, fibrossarcomatoso e epitelial, com estruturas pseudo-glandulares. O diagnóstico final foi obtido pelo exame da peça cirúrgica com preparações histológicas de rotina e análise imunohistoquímica.Primary synovial sarcoma of the hea...

  6. Primary sarcoma of the lung: A very rare diagnosis and poor prognosis

    Directory of Open Access Journals (Sweden)

    Rhizlane Belbaraka

    2014-01-01

    Full Text Available Primary lung sarcoma is a very rare malignant tumor, accounting for less than 0.5% of all lung tumors. We describe the case of a 50-year-old man who presented with primary pulmonary sarcoma. X-ray chest and thoracic computed tomography (CT revealed a 40 mm Χ 60 mm mass in the right upper lobe. Biopsy was reported as fusiform sarcoma. Extensive clinical examination, followed by full body CT scan was performed to exclude primary synovial sarcoma located peripherally and distant metastases. Hence, surgical excision was planned after three cycles of chemotherapy, but the patient died after the second cycle. Because a sarcoma of the lung is rare, data regarding its natural history and published cases are limited. Further investigation and data collection are required to optimize the treatment of this group of rare and aggressive tumors.

  7. [Primary pulmonary artery sarcoma in 36-year-old women: 3-years follow-up after partial resection and radiotherapy].

    Science.gov (United States)

    Drożdż, Jarosław; Warchoł, Ewa; Fijuth, Jacek; Filipiak, Krzysztof; Spych, Michał; Maciejewski, Marek; Piestrzeniewicz, Katarzyna; Ludomir, Stafańczyk; Janaszek-Sitkowska, Hanna; Januszewicz, Andrzej; Zembala, Marian

    2013-01-01

    Intimal sarcoma of the heart and pulmonary artery is a very rare, malignant, primary tumour. The prognosis in patients with primary sarcoma of the pulmonary artery, including intimal sarcoma, is poor. We present the case and 3-years follow-up of 36-year-old woman who was successfully treated with surgical, partial resection of the tumour followed by radiotherapy.

  8. Pericardial Effusion due to Primary Malignant Pericardial Mesothelioma: A Common Finding but an Uncommon Cause

    Directory of Open Access Journals (Sweden)

    Valery Istomin

    2016-01-01

    Full Text Available This case report describes a 37-year-old female who was admitted to our Emergency Department because of shortness of breath. On physical examination, she had dyspnea and tachycardia and blood pressure was 80/50 mmHg with a pulsus paradoxus of 22 mmHg. Neck veins were distended, heart sounds were distant, and dullness was found on both lung bases. Her chest X-ray revealed bilateral pleural effusion and cardiomegaly. On both computed tomography and echocardiography the heart was of normal size and a large pericardial effusion was noted. The echocardiogram showed signs of impending tamponade, so the patient underwent an emergent pericardiocentesis. No infectious etiology was found and she was assumed to have viral pericarditis and was treated accordingly. However, when the pericardial effusion recurred and empirical therapy for tuberculosis failed, a pericardial window was performed. A typical staining pattern for mesothelioma was found on her pericardial biopsy specimen. Since no other mesodermal tissue was affected, a diagnosis of primary malignant pericardial mesothelioma was made. Chemotherapy was not effective and she passed away a year after the diagnosis was made. This case highlights the difficulties in diagnosing this uncommon disease in patients that present with the common finding of pericardial effusion.

  9. Primary lung sarcomas: long survivors obtained with iterative complete surgery.

    Science.gov (United States)

    Magné, N; Porsin, B; Pivot, X; Tchiknavorian, X; Marcy, P Y; Foa, C; Otto, J; Schneider, M; Thyss, A

    2001-01-01

    Primary lung sarcomas are uncommon histologic types of primary lung cancer and presents a wide spectrum of clinical behaviour. Nine patients treated at Antoine Lacassagne Cancer Center between 1982 and 1995 were studied. The median age was 63 years (range, 35-73 years) and the most common histologic types were malignant fibrous histiocytoma (four) and leiomyosarcoma (three). All of them underwent surgery, six patients had a complete surgery and three patients incomplete resections. The median overall survival for all patients was 36 months. In the subgroup of patients with initial complete resection, the median survival was significantly longer (47 months) than in the subgroup of patients with incomplete resection (6 months) (Prank test). Moreover, two patients had a second complete resection for ipsilateral lung relapse and were long survivors (overall survival of 58 and 83 months, respectively). The ability to achieve a complete second surgery stress the possible benefit of an early detection of local recurrence. Because no specific symptom was linked with the local relapse, a systematic CT scan every 2 or 3 months could be required.

  10. Extensive primary Ewings' sarcoma in the greater wing of the sphenoid bone.

    Science.gov (United States)

    Apostolopoulos, Kostas; Ferekidis, Eleftherios

    2003-01-01

    We describe a rare case of an extensive primary cranial Ewing's sarcoma located in the greater wing of the sphenoid bone with extension to the orbit, the endocranium, the parapharyngeal and infratemporal space. The patient presented with diplopia, anosmia and prolapse of the left eye. He was given chemo- and radiotherapy and was free of symptoms on re-examination 1.5 years later. The prognosis of Ewing's sarcoma in the absence of surgery is uncertain, but prompt treatment appears to have a satisfactory therapeutic outcome. In the future, more cases should be studied in order to investigate the biological behaviour of a primary cranial Ewing's sarcoma.

  11. Primary undifferentiated high-grade pleomorphic sarcoma in the perihepatic space: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Ki Beom; Kim, Song Hoon [Dept. of Radiology, Daegu Fatima Hospital, Daegu (Korea, Republic of)

    2015-07-15

    Undifferentiated high-grade pleomorphic sarcoma (UPS) is a pleomorphic soft tissue sarcoma derived from histiocytes capable of fibroblastic transformation. UPS affects soft tissues of the body, especially the extremities and retroperitoneum, and primary UPS in the perihepatic space is extremely rare. We report a case of primary perihepatic UPS in a 44-year-old male, who presented with fever and right upper abdominal discomfort.

  12. MR findings of primary Ewing's sarcoma of greater wing of sphenoid.

    Science.gov (United States)

    Singh, Paramjeet; Jain, Manoj; Singh, D P; Kalra, Naveen; Khandelwal, N; Suri, S

    2002-12-01

    Primary Ewing's sarcoma of the skull is a very rare entity. We report MRI findings in a case of Ewing's sarcoma of the greater wing of sphenoid in a 4-year-old patient. Magnetic resonance imaging showed markedly heterogenous signal intensity with areas of haemorrhage and necrosis. It also demonstrated the exact extent of tumour due to its multiplanar capabilities and was, therefore, helpful in planning surgery.

  13. SIRT1 and AMPK pathways are essential for the proliferation and survival of primary effusion lymphoma cells.

    Science.gov (United States)

    He, Meilan; Tan, Brandon; Vasan, Karthik; Yuan, Hongfeng; Cheng, Fan; Ramos da Silva, Suzane; Lu, Chun; Gao, Shou-Jiang

    2017-07-01

    Primary effusion lymphoma (PEL) is a rare and aggressive B-cell lymphoma with a dismal prognosis caused by infection of Kaposi's sarcoma-associated herpesvirus. Despite the findings that numerous viral genes and cellular pathways are essential for the proliferation and survival of PEL cells, there is currently no effective therapeutic treatment for PEL. Here, we report that the metabolic sensor SIRT1 is functionally required for sustaining the proliferation and survival of PEL cells. Knockdown of SIRT1 with specific shRNAs or inhibition of SIRT1 with an inhibitor (tenovin-6) induced cell cycle arrest and apoptosis in PEL cells. We detected high levels of AMPK activation in PEL cells, reflected in AMPKα1 phosphorylation at T174. Knockdown or inhibition of SIRT1 reduced AMPK activation, indicating that SIRT1 was required for AMPK activation. Interestingly, knockdown of AMPK with specific shRNAs or inhibition of AMPK with the inhibitor compound C recapitulated the phenotype of SIRT1, and induced cell cycle arrest and apoptosis, whereas overexpression of a constitutively active AMPK construct rescued the cytotoxic effect of SIRT1 knockdown. Remarkably, treatment with tenovin-6 effectively inhibited the initiation and progression of PEL, and significantly extended the survival of mice in a murine PEL model. Taken together, these results illustrate that the SIRT1-AMPK axis is essential for maintaining the proliferation and survival of PEL and identify SIRT1 and AMPK as potential therapeutic targets, and tenovin-6 as a candidate therapeutic agent for PEL patients. Copyright © 2017 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd. Copyright © 2017 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.

  14. Sarcoma sinovial primário da orofaringe: relato de caso Primary synovial sarcoma of the oropharinx: a case report

    Directory of Open Access Journals (Sweden)

    Hugo Meister

    2003-06-01

    Full Text Available O sarcoma sinovial primário da cabeça e pescoço é um tumor raro. Em torno de 90 casos foram descritos na literatura. Relatamos um caso adicional ocorrido em um paciente jovem do sexo masculino comprometendo a orofaringe. O tumor apresentou típico crescimento bifásico, fibrossarcomatoso e epitelial, com estruturas pseudo-glandulares. O diagnóstico final foi obtido pelo exame da peça cirúrgica com preparações histológicas de rotina e análise imunohistoquímica.Primary synovial sarcoma of the head and neck is a rare tumor. Fewer than 90 cases having been reported in the literature. This report presents an addicional case of synovial sarcoma involving the oropharinx in a young man. Microscopically the tumor showed a biphasic pattern of pseudo-ephitelial cells and a spindle cell element with fibrosarcomatous and epithelial appearance. A proper diagnosis was done after routine pathological and immunohistochemical studies of the ressected tumors.

  15. Bilateral Pleural Effusions as an Initial Presentation in Primary Sjögren's Syndrome

    OpenAIRE

    Go Makimoto; Michiko Asano; Nobukazu Fujimoto; Yasuko Fuchimoto; Katsuichiro Ono; Shinji Ozaki; Koji Taguchi; Takumi Kishimoto

    2012-01-01

    Sjögren’s syndrome (SS) is a systemic autoimmune disease characterized by sicca symptoms. Interstitial pulmonary fibrosis and tracheobronchial sicca are the most common symptoms of pulmonary involvement in primary SjS, and they are rarely accompanied by serositis such as pleuritis or pericarditis. We report a case of SS presenting initially with bilateral pleural effusions. A 63-year old man was admitted to our hospital with a one-month history of cough, dyspnea, and right chest pain. Chest-c...

  16. Prevalence of Otitis Media with Effusion Among Primary School Age-Children and Etiopathogenic Examination

    OpenAIRE

    Sanli, Arif; Tasdemir, Omer; Eken, Mehmet; Celebi, Ozlem; Yilmaz, Suleyman Hilmi

    2011-01-01

    A prevalence study was carried out in order to look into the etiopathogenic relation of otitis media with effusion (OME) in primary school-age children. The study included ENT examination and a questionnaire that was carried out in 4 randomly selected schools on 1,165 children. 143 of the cases (12.2%) had OME. A significant relation between OME and adenoid vegetation was found (P0.05). As a result, OME is a common disease that can lead to other hard-to-cure health problems and be costly if e...

  17. Primary sarcoma of the ovary: clinicopathological characteristics, prognostic factors and evaluation of therapy

    Institute of Scientific and Technical Information of China (English)

    DAI Yi; SHEN Keng; LANG Jing-he; HUANG Hui-fang; PAN Ling-ya; WU Ming; YANG Jia-xin; ZHONG Ding-rong

    2011-01-01

    Background The primary ovarian sarcoma is a very rare malignancy. The objective of this study was to further investigate the clinicopathologic features and outcome in patients with primary sarcoma of the ovary.Methods Between 1988 and 2007, 24 patients with primary ovarian sarcoma who underwent treatment at Peking Union Medical Hospital were reviewed retrospectively. Response to treatment, progression and overall survival were analyzed.Results Patients with ovarian sarcoma had a mean age of (54.3±10.3) years, and 16 of them were postmenopausal.The most common symptom was abdominal pain, present in 14 patients. Of the 24 patients, 16 patients were pathologically diagnosed as carcinosarcoma (known as malignant mixed mesodermal tumor (MMMT)), 2 as ovarian leiomyosarcoma (LS) and 6 patients as ovarian endometrial stromal sarcoma (ESS). The patients in optimal debulking group had a median survival period of 28 months and 1-year survival rate of 71%. The patients in suboptimal debulking group had a significantly lower median survival of 6 months (P=-0.02) and 1-year survival rate of 29%. Among the patients,23 patients received chemotherapy and most of regimens were based on platinum, 3 patients received chemoradiation.The mean number of courses of combined chemotherapy was 6.6±5.0, and the response was unsatisfactory. The median survival for the entire group was 18.7 months. The one-year survival rate was 58%, and two-year survival rate only 29%.Conclusions Ovarian primary sarcoma has a poor overall prognosis. Optimal debulking surgery appears to be of prognostic significance. There is a clear need for further study to explore the role and the regimen of platinum-based chemotherapy in primary ovarian sarcoma.

  18. Primary Ewing's sarcoma of the greater wing of the sphenoid bone.

    Science.gov (United States)

    Sharma, R R; Netalkar, A; Lad, S D

    2000-02-01

    Primary Ewing's sarcoma is an uncommon lethal tumour of the long bones and pelvic girdle mainly affecting children and young adults. An origin in the cranial bones is extremely rare. We report a unique case of primary involvement of the greater wing of sphenoid bone in a 16-year-old patient. Aggressive management using microsurgical resection, radiotherapy and chemotherapy was curative. Localized, primary Ewing's sarcoma of the cranial bones should be considered as a distinct clinicopathological entity with an extremely low rate of dural penetration and metastases, and with a relatively better prognosis as compared with those of long bones and pelvic girdle. In neurosurgical practice, primary Ewing's sarcoma of the cranial bones requires early aggressive management to achieve adequate long-term prognosis and cure.

  19. Primary extraskeletal Ewing's sarcoma/primitive neuroectodermal tumor of breast

    Directory of Open Access Journals (Sweden)

    Smita Srivastava

    2016-01-01

    Full Text Available Extraskeletal Ewing's sarcoma (EES is a rare soft tissue tumor that is morphologically indistinguishable from skeletal ES. We report a case of a 25-year-old female with recurrent EES/primitive neuroectodermal tumor of right breast with imaging findings on mammogram, ultrasound, magnetic resonance imaging breast, and positron emission tomography–computed tomography.

  20. Isolated granulocytic sarcoma of the pancreas: A tricky diagnostic for primary pancreatic extramedullary acute myeloid leukemia

    Directory of Open Access Journals (Sweden)

    Messager Mathieu

    2012-01-01

    Full Text Available Abstract We report two clinical cases of primary granulocytic sarcoma of the pancreas that were diagnosed on the surgical specimen. Atypical clinical and morphological presentations may have lead to pretherapeutic biopsies of the pancreatic mass in order to indicate primary chemotherapy. Literature review of this rare clinical presentation may help physicians to anticipate diagnostic and therapeutic strategies.

  1. Primary intra-abdominal synovial sarcoma: a case report.

    Science.gov (United States)

    Travaglini, Grazia; Biagetti, Simona; Alfonsi, Simona; Bearzi, Italo; Marmorale, Cristina

    2013-09-03

    Il caso clinico che vi presentiamo riguarda la straordinaria localizzazione intra-addominale di un sinovial-sarcoma. Questo tumore dei tessuti molli generalmente interessa le piccole e grandi articolazioni. Sono state descritte altre localizzazioni a livello intra-addominale e retroperitoneale. La nostra paziente si ricovera per una tumefazione dolente dell’addome, alla TC addome si evidenzia una voluminosa massa intra-addominale che comprime e disloca i parenchimi e visceri circostanti e che affiora ai tessuti più superficiali della parete addominale. La biopsia eco-guidata della lesione, insieme allo studio immunoistochimico, permette di porre la diagnosi di sinovial-sarcoma. La paziente viene candidata ad un trattamento chemioterapico a scopo neoadiuvante. Per il sopraggiungere di anemizzazione da sanguinamento della neoplasia, valutata anche la risposta parziale al trattamento sistemico, si pone indicazione all’intervento chirurgico. La massa con peduncolo vascolare maggiore costituito dai vasi gastroepiploici di destra, viene completamente asportata. L’esame definitivo conferma la diagnosi di Sinovial-Sarcoma bifasico. L’indagine genetica condotta sul campione conservato in formalina non ha permesso, a causa della difficoltà di estrarre ed amplificare un quantitativo adeguato di RNA, di identificare la traslocazione (X; 18) (p 11.2; q 11.2) specifica del Sinovial-Sarcoma. La paziente è stata dimessa dopo un decorso clinico regolare. Il follow-up ad un anno non ha mostrato ripresa di malattia ma dopo 18 mesi alla TC torace-addome di rivalutazione si evidenzia la ripresa di malattia a livello intra-addominale e la presenza di metastasi polmonari bilaterali.

  2. Primary histiocytic sarcoma of the brain mimicking cerebral abscess.

    Science.gov (United States)

    Almefty, Rami O; Tyree, Tammy L; Fusco, David J; Coons, Stephen W; Nakaji, Peter

    2013-09-01

    Histiocytic sarcoma is a rare malignancy with only 10 reports confirmed primarily involving the CNS. The diagnosis is dependent on the finding of malignant cells with histiocytic morphology and immunophenotype. The authors report a case of pathologically proven HS of the CNS. A 16-year-old boy presented with headaches, emesis, and altered sensorium. Noncontrast head CT scanning demonstrated a left parietal mass consistent with a tumor. Surgery was undertaken. Intraoperative findings revealed green-yellow exudates consistent with an abscess. Cultures were obtained and broad-spectrum antibiotics were started. The patient subsequently underwent multiple surgical procedures, including drainage and debulking of abscesses and hemicraniectomy. Two months after initial presentation, the patient's diagnosis of histiocytic sarcoma was confirmed. Pathological examination demonstrated necrotizing inflammation with preponderant neutrophil infiltration, variably atypical mononuclear and multinucleate histiocytes, and numerous mitoses. Additional immunohistochemistry studies confirmed immunoreactivity for CD68, CD45, CD45RO, and CD15 and were negative for CD3, CD20, melanoma cocktail, CD30, CD1a, CD34, HMB-45, and melan-A. Once the diagnosis of histiocytic sarcoma was confirmed, antibiotics were stopped and radiation therapy was undertaken. Despite treatment, the patient's neurological status continued to decline and the patient died 126 days after initial presentation. This case represents a rare confirmed example of CNS histiocytic sarcoma. A profound inflammatory infiltrate seen on pathology and green exudates seen intraoperatively make the condition difficult to distinguish from an abscess. Immunohistochemistry showing a histiocytic origin and negative for myeloid, dendritic, or other lymphoid markers is essential for the diagnosis. Further research is needed to establish consensus on treatment.

  3. Primary Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) of the penis: a report of an extraordinarily unusual site and a literature review of extraskeletal Ewing's sarcoma.

    Science.gov (United States)

    Ma, Zheng; Brimo, Fadi; Zeizafoun, Nebras

    2013-02-01

    Ewing's sarcomas/primitive neuroectodermal tumors (ES/PNETs) arise from a multipotent progenitor cell and are considered to be of neuroectodermal derivation. Most tumors commonly arise in the skeletal system, which are the classic ES/PNET and occasionally occur in the soft tissue of extraskeletal sites, which are named extraskeletal Ewing's sarcomas (EES/PNET). This study reports a case of a 28-year-old man with primary EES/PNET of the penis.

  4. Linfoma primário de cavidade pleural em paciente imunocompetente Primary effusion lymphoma in an immunocompetent patient

    Directory of Open Access Journals (Sweden)

    Leila Antonangelo

    2005-12-01

    Full Text Available O linfoma primário de cavidade é um tipo raro de linfoma não-Hodgkin que acomete principalmente pacientes imunocomprometidos e, mais raramente, pacientes imunocompetentes. Neste relato de caso são apresentados os achados clínicos e laboratoriais de um paciente imunocompetente com derrame pleural diagnosticado como linfoma primário de cavidade pleural.Primary effusion lymphoma is an unusual non-Hodgkin's lymphoma rarely seen in immunocompetent patients. Herein, we present clinical and biochemical data obtained from an immunocompetent patient diagnosed with primary effusion lymphoma.

  5. HIV-associated primary effusion lymphoma presenting as a paracardial mass

    Science.gov (United States)

    Katz, Heather; Rose, Cielo; Rivera, Nina Thakker; Bray, Natasha

    2015-01-01

    Primary effusion lymphoma (PEL), a rare type of non-Hodgkin's lymphoma, is an AIDS-defining illness and always associated with human herpesvirus 8 (HHV-8). Classic presentations involve the pleural, pericardial or peritoneal cavities. Infrequently, extracavitary solid tumours develop. Treatment of PEL requires chemotherapy and highly active antiretroviral therapy (HAART). We report a case of a 46-year-old man, who presented with right-sided chest pain, dyspnoea and night sweats. Evaluation revealed decreased breath sounds and dullness to percussion on the right side of the chest. Imaging demonstrated a 6.1 cm×6.3 cm right paracardial mass and right-sided pleural effusion. Pleural fluid was HHV-8 positive. The patient was diagnosed with PEL with extracavitary involvement and treated with chemotherapy and concurrent HAART. This case is the first reported case of extracavitary paracardial involvement and adds new insight to the accepted treatment for PEL with extracavitary lesions. PMID:25725030

  6. FDG PET/CT imaging in primary osseous and soft tissue sarcomas: a retrospective review of 212 cases

    Energy Technology Data Exchange (ETDEWEB)

    Charest, Mathieu [Lakeshore General Hospital, Service of Nuclear Medicine, Pointe-Claire (Canada); Hickeson, Marc; Lisbona, Robert; Novales-Diaz, Javier A.; Derbekyan, Vilma [McGill University Health Center, Department of Nuclear Medicine, Montreal (Canada); Turcotte, Robert E. [McGill University Health Center, Department of Orthopedic Surgery, Montreal (Canada)

    2009-12-15

    The aims of this study are to evaluate the sensitivity of FDG PET/CT for detection of soft tissue and osseous sarcomas on the basis of FDG avidity. We retrospectively evaluated 212 consecutive patients with known soft tissue or osseous sarcoma who had undergone a FDG PET/CT study for the initial staging or assessment of recurrence of disease. The maximum standardized uptake value (SUV{sub max}) of each primary and/or most intense metastatic lesion was measured and compared with the histological data provided in the final pathological reports. An SUV{sub max} of 2.5 or greater was considered positive for our analysis. Sufficient histopathological data were available for 160 soft tissue sarcomas and 52 osseous sarcomas. FDG PET/CT detected 93.9% of all sarcomas with a sensitivity of 93.7% for soft tissue sarcomas and 94.6% for osseous sarcomas. The sensitivities of the most common sarcoma histologies were 100% for leiomyosarcomas, 94.7% for osteosarcomas, 100% for Ewing's sarcomas, 88.9% for liposarcomas, 80.0% for synovial sarcomas, 100% for gastrointestinal stromal tumors, 87.5% for malignant peripheral nerve sheath tumors, 100% for fibroblastic and myoblastic sarcomas, and 100% for malignant fibrohistiocytic tumors. The receiver-operating characteristic curve revealed an area under the curve of 94% for the discrimination of low-grade and high-grade sarcomas imaged for initial staging by FDG PET/CT. The combined metabolic and morphological information of FDG PET/CT imaging allows high sensitivity for the detection of various sarcomas and accurate discrimination between newly diagnosed low-grade and high-grade sarcomas. (orig.)

  7. Primary breast sarcoma: A retrospective study over 35 years from a single institution

    DEFF Research Database (Denmark)

    Holm, Maibritt Provstgaard; Aggerholm-Pedersen, Ninna; Mele, Marco

    2016-01-01

    AIM: The aim of this study was to contribute to the collected knowledge of prognostic factors in primary breast sarcomas (PBS) to the benefit of possible future prospective studies and therapeutic guidelines. METHOD: All patients with pathologically verified PBS in the period of 1979-2014 were...

  8. Primary paraesophageal Ewing’s sarcoma: an uncommon case report and literature review

    Science.gov (United States)

    Tarazona, Noelia; Navarro, Lara; Cejalvo, Juan Miguel; Gambardella, Valentina; Pérez-Fidalgo, J Alejandro; Sempere, Alejo; Navarro, Samuel; Cervantes, Andrés

    2015-01-01

    Ewing’s sarcoma is a rare and highly aggressive cancer most frequently arising in people under 20 years of age. We report an uncommon case of primary paraesophageal Ewing’s sarcoma in a 25-year-old male harboring the infrequent EWSR1/ERG fusion transcript with multiple splice variants coexisting in the same tumor. The patient was totally refractory to chemotherapy and died 17 months after diagnosis. We underscore the need for better understanding of the molecular pathogenesis of the disease and improved systemic therapy options. PMID:25999740

  9. CT findings of primary undifferentiated pleomorphic sarcoma in the small bowel: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Youe Ree; Lee, Young Hwan; Yoon, Kwon Ha; Yun, Ki Jung [Wonkwang University School of Medicine and Hospital, Institute of Wonkwang Medical Science, Iksan (Korea, Republic of)

    2015-11-15

    Undifferentiated pleomorphic sarcoma (UPS), previously known as malignant fibrous histiocytoma, is a soft tissue sarcoma arising from mesenchymal tissue of the body. UPS of the gastrointestinal tract is known to be rare and only a few cases have been reported in the literature. Based on our case and review of the other relevant literature, the CT findings of primary UPS of the small bowel included nodular bowel wall thickening with homogeneous enhancement. It presents as a rapidly growing tumor without bowel obstruction, and it may be accompanied by distant metastasis.

  10. Primary undifferentiated spindle-cell sarcoma of sella turcica: successful treatment with adjuvant temozolomide.

    Science.gov (United States)

    Sareen, Pooja; Chhabra, Lovely; Trivedi, Nitin

    2013-05-27

    Sellar tumours in adults are most commonly pituitary adenomas. Primary spindle cell sarcoma of the sella turcica without a prior history of cranial radiation is extremely rare. We report a case of a large sellar mass with suprasellar and cavernous sinus extension in a geriatric male patient who presented with complete left oculomotor nerve palsy and panhypopituitarism. The patient underwent partial resection of the sellar mass through transcranial route. The pathology of the mass revealed a poorly differentiated spindle cell neoplasm most consistent with a sarcoma. Postoperatively, the size of the residual sellar mass decreased significantly following six cycles of external beam radiation in conjunction with temozolomide.

  11. Molecular piracy of Kaposi's sarcoma associated herpesvirus.

    Science.gov (United States)

    Choi, J; Means, R E; Damania, B; Jung, J U

    2001-01-01

    Kaposi's Sarcoma associated Herpesvirus (KSHV) is the most recently discovered human tumor virus and is associated with the pathogenesis of Kaposi's sarcoma, primary effusion lymphoma, and Multicentric Casttleman's disease. KSHV contains numerous open reading frames with striking homology to cellular genes. These viral gene products play a variety of roles in KSHV-associated pathogenesis by disrupting cellular signal transduction pathways, which include interferon-mediated anti-viral responses, cytokine-regulated cell growth, apoptosis, and cell cycle control. In this review, we will attempt to cover our understanding of how viral proteins deregulate cellular signaling pathways, which ultimately contribute to the conversion of normal cells to cancerous cells.

  12. Pyrrolidinium fullerene induces apoptosis by activation of procaspase-9 via suppression of Akt in primary effusion lymphoma

    Energy Technology Data Exchange (ETDEWEB)

    Watanabe, Tadashi [Department of Cell Biology, Kyoto Pharmaceutical University, Misasagi-Shichonocho 1, Yamashinaku, Kyoto 607-8412 (Japan); Nakamura, Shigeo [Department of Chemistry, Nippon Medical School, 1-7-1 Kyonan-cho, Musashino, Tokyo 180-0023 (Japan); Ono, Toshiya; Ui, Sadaharu [Department of Biotechnology, Interdisciplinary Graduate School of Medicine and Engineering, University of Yamanashi, Kofu 400-8511 (Japan); Yagi, Syota; Kagawa, Hiroki [Department of Cell Biology, Kyoto Pharmaceutical University, Misasagi-Shichonocho 1, Yamashinaku, Kyoto 607-8412 (Japan); Watanabe, Hisami [Center of Molecular Biosciences, Tropical Biosphere Research Center, University of the Ryukyus, 1 Senbaru, Nishihara-cho, Okinawa 903-0213 (Japan); Ohe, Tomoyuki; Mashino, Tadahiko [Department of Pharmaceutical Sciences, Faculty of Pharmacy, Keio University, 1-5-30 Shibakoen, Minato-ku, Tokyo 105-8512 (Japan); Fujimuro, Masahiro, E-mail: fuji2@mb.kyoto-phu.ac.jp [Department of Cell Biology, Kyoto Pharmaceutical University, Misasagi-Shichonocho 1, Yamashinaku, Kyoto 607-8412 (Japan)

    2014-08-15

    Highlights: • Seven fullerenes were evaluated in terms of their cytotoxic effects on B-lymphomas. • Pyrrolidinium fullerene induced apoptosis of KSHV-infected B-lymphoma PEL cells. • The activation of Akt is essential for PEL cell survival. • Pyrrolidinium fullerene activated caspase-9 by inactivating Akt in PEL cells. • Pyrrolidinium fullerene have potential as novel drugs for the treatment of PEL. - Abstract: Primary effusion lymphoma (PEL) is a subtype of non-Hodgkin’s B-cell lymphoma and is an aggressive neoplasm caused by Kaposi’s sarcoma-associated herpesvirus (KSHV) in immunosuppressed patients. In general, PEL cells are derived from post-germinal center B-cells and are infected with KSHV. To evaluate potential novel anti-tumor compounds against KSHV-associated PEL, seven water-soluble fullerene derivatives were evaluated as potential drug candidates for the treatment of PEL. Herein, we discovered a pyrrolidinium fullerene derivative, 1,1,1′,1′-tetramethyl [60]fullerenodipyrrolidinium diiodide, which induced apoptosis of PEL cells via a novel mechanism, the caspase-9 activation by suppressing the caspase-9 phosphorylation, causing caspase-9 inactivation. Pyrrolidinium fullerene treatment reduced significantly the viability of PEL cells compared with KSHV-uninfected lymphoma cells, and induced the apoptosis of PEL cells by activating caspase-9 via procaspase-9 cleavage. Pyrrolidinium fullerene additionally reduced the Ser473 phosphorylation of Akt and Ser196 of procaspase-9. Ser473-phosphorylated Akt (i.e., activated Akt) phosphorylates Ser196 in procaspase-9, causing inactivation of procaspase-9. We also demonstrated that Akt inhibitors suppressed the proliferation of PEL cells compared with KSHV-uninfected cells. Our data therefore suggest that Akt activation is essential for cell survival in PEL and a pyrrolidinium fullerene derivative induced apoptosis by activating caspase-9 via suppression of Akt in PEL cells. In addition, we evaluated

  13. A Scary Onset of a Rare and Aggressive Type of Primary Breast Sarcoma: A Case Report

    Directory of Open Access Journals (Sweden)

    Inês Ramalho

    2016-11-01

    Full Text Available Primary breast sarcoma, arising from connective tissue within the breast, is extremely rare, accounting for less than 1% of all primary breast malignancies and no more than 5% of all sarcomas. The rarity of this pathology limits most studies to case reports and small retrospective studies, which has led to a lack of consensus on the clinical management. We report a clinical case of a 52-year-old woman, perimenopausal, previously healthy, with regular breast surveillance, who presented with a large (>20 cm and rapidly expanding hypervascularized tumor of the left breast developed over 10 days, with a very thin preulcerative skin over the last 4 days. There was no systemic dissemination. The patient was submitted to total mastectomy and excision of axillary adenopathy. The tumor was diagnosed histologically as malignant phyllodes tumor associated with areas of high-grade sarcoma. Due to rapid growth and aggressive histological characteristics, adjuvant chemotherapy and radiotherapy were performed. There is a lot of evidence that tumors larger than 5 cm are associated with a poor prognosis. Despite the poor prognosis associated with this aggressive entity, the patient had no recurrence during 5 years of follow-up. We review the relevant literature about primary breast sarcomas.

  14. A Scary Onset of a Rare and Aggressive Type of Primary Breast Sarcoma: A Case Report

    Science.gov (United States)

    Ramalho, Inês; Campos, Sara; Rebelo, Teresa; Figueiredo Dias, Margarida

    2016-01-01

    Primary breast sarcoma, arising from connective tissue within the breast, is extremely rare, accounting for less than 1% of all primary breast malignancies and no more than 5% of all sarcomas. The rarity of this pathology limits most studies to case reports and small retrospective studies, which has led to a lack of consensus on the clinical management. We report a clinical case of a 52-year-old woman, perimenopausal, previously healthy, with regular breast surveillance, who presented with a large (>20 cm) and rapidly expanding hypervascularized tumor of the left breast developed over 10 days, with a very thin preulcerative skin over the last 4 days. There was no systemic dissemination. The patient was submitted to total mastectomy and excision of axillary adenopathy. The tumor was diagnosed histologically as malignant phyllodes tumor associated with areas of high-grade sarcoma. Due to rapid growth and aggressive histological characteristics, adjuvant chemotherapy and radiotherapy were performed. There is a lot of evidence that tumors larger than 5 cm are associated with a poor prognosis. Despite the poor prognosis associated with this aggressive entity, the patient had no recurrence during 5 years of follow-up. We review the relevant literature about primary breast sarcomas. PMID:28101028

  15. A computational profiling of changes in gene expression and transcription factors induced by vFLIP K13 in primary effusion lymphoma.

    Directory of Open Access Journals (Sweden)

    Vasu Punj

    Full Text Available Infection with Kaposi's sarcoma associated herpesvirus (KSHV has been linked to the development of primary effusion lymphoma (PEL, a rare lymphoproliferative disorder that is characterized by loss of expression of most B cell markers and effusions in the body cavities. This unique clinical presentation of PEL has been attributed to their distinctive plasmablastic gene expression profile that shows overexpression of genes involved in inflammation, adhesion and invasion. KSHV-encoded latent protein vFLIP K13 has been previously shown to promote the survival and proliferation of PEL cells. In this study, we employed gene array analysis to characterize the effect of K13 on global gene expression in PEL-derived BCBL1 cells, which express negligible K13 endogenously. We demonstrate that K13 upregulates the expression of a number of NF-κB responsive genes involved in cytokine signaling, cell death, adhesion, inflammation and immune response, including two NF-κB subunits involved in the alternate NF-κB pathway, RELB and NFKB2. In contrast, CD19, a B cell marker, was one of the genes downregulated by K13. A comparison with K13-induced genes in human vascular endothelial cells revealed that although there was a considerable overlap among the genes induced by K13 in the two cell types, chemokines genes were preferentially induced in HUVEC with few exceptions, such as RANTES/CCL5, which was induced in both cell types. Functional studies confirmed that K13 activated the RANTES/CCL5 promoter through the NF-κB pathway. Taken collectively, our results suggest that K13 may contribute to the unique gene expression profile, immunophenotype and clinical presentation that are characteristics of KSHV-associated PEL.

  16. Imaging Primary Mouse Sarcomas After Radiation Therapy Using Cathepsin-Activatable Fluorescent Imaging Agents

    Energy Technology Data Exchange (ETDEWEB)

    Cuneo, Kyle C. [Department of Radiation Oncology, Duke University School of Medicine, Durham, North Carolina (United States); Mito, Jeffrey K.; Javid, Melodi P. [Department of Pharmacology and Cancer Biology, Duke University School of Medicine, Durham, North Carolina (United States); Ferrer, Jorge M. [Department of Chemistry, Massachusetts Institute of Technology, Cambridge, Massachusetts (United States); Kim, Yongbaek [Department of Clinical Pathology, College of Veterinary Medicine, Seoul National University, Seoul (Korea, Republic of); Lee, W. David [The David H. Koch Institute for Integrative Cancer Research, Massachusetts Institute of Technology, Cambridge, Massachusetts (United States); Bawendi, Moungi G. [Department of Chemistry, Massachusetts Institute of Technology, Cambridge, Massachusetts (United States); Brigman, Brian E. [Department of Orthopedic Surgery, Duke University School of Medicine, Durham, North Carolina (United States); Kirsch, David G., E-mail: david.kirsch@duke.edu [Department of Radiation Oncology, Duke University School of Medicine, Durham, North Carolina (United States); Department of Pharmacology and Cancer Biology, Duke University School of Medicine, Durham, North Carolina (United States)

    2013-05-01

    Purpose: Cathepsin-activated fluorescent probes can detect tumors in mice and in canine patients. We previously showed that these probes can detect microscopic residual sarcoma in the tumor bed of mice during gross total resection. Many patients with soft tissue sarcoma (STS) and other tumors undergo radiation therapy (RT) before surgery. This study assesses the effect of RT on the ability of cathepsin-activated probes to differentiate between normal and cancerous tissue. Methods and Materials: A genetically engineered mouse model of STS was used to generate primary hind limb sarcomas that were treated with hypofractionated RT. Mice were injected intravenously with cathepsin-activated fluorescent probes, and various tissues, including the tumor, were imaged using a hand-held imaging device. Resected tumor and normal muscle samples were harvested to assess cathepsin expression by Western blot. Uptake of activated probe was analyzed by flow cytometry and confocal microscopy. Parallel in vitro studies using mouse sarcoma cells were performed. Results: RT of primary STS in mice and mouse sarcoma cell lines caused no change in probe activation or cathepsin protease expression. Increasing radiation dose resulted in an upward trend in probe activation. Flow cytometry and immunofluorescence showed that a substantial proportion of probe-labeled cells were CD11b-positive tumor-associated immune cells. Conclusions: In this primary murine model of STS, RT did not affect the ability of cathepsin-activated probes to differentiate between tumor and normal muscle. Cathepsin-activated probes labeled tumor cells and tumor-associated macrophages. Our results suggest that it would be feasible to include patients who have received preoperative RT in clinical studies evaluating cathepsin-activated imaging probes.

  17. Genetic Profiling Differentiates Second Primary Tumors from Metastases in Adult Metachronous Soft Tissue Sarcoma

    Directory of Open Access Journals (Sweden)

    Josefin Fernebro

    2008-01-01

    Full Text Available Purpose. Patients with soft tissue sarcomas (STS are at increased risk of second primary malignancies, including a second STS, but distinction between metastases and a second primary STS is difficult. Patients and Methods. Array-based comparative genomic hybridization (aCGH was applied to 30 multiple STS of the extremities and the trunk wall from 13 patients. Different histotypes were present with malignant fibrous histiocytomas/undifferentiated pleomorphic sarcomas being the predominant subtype. Results. aCGH profiling revealed genetic complexity with multiple gains and losses in all tumors. In an unsupervised hierarchical cluster analysis, similar genomic profiles and close clustering between the first and subsequent STS were identified in 5 cases, suggesting metastatic disease, whereas the tumors from the remaining 8 patients did not cluster and showed only weak pairwise correlation, suggesting development of second primary STS. Discussion. The similarities and dissimilarities identified in the first and second STS suggest that genetic profiles can be used to distinguish soft tissue metastases from second primary STS. The demonstration of genetically different soft tissue sarcomas in the same patient suggests independent tumor origin and serves as a reminder to consider development of second primary STS, which has prognostic and therapeutic implications.

  18. Sarcoma de Kaposi primário do pênis Primary Kaposi's sarcoma of the penis

    Directory of Open Access Journals (Sweden)

    Maira Mitsue Mukai

    2009-10-01

    Full Text Available Sarcoma de Kaposi é um tumor vascular que afeta a parede dos vasos linfáticos. Possui quatro formas: clássica, endêmica, iatrogênica e associada ao HIV. É uma doença sistêmica, maligna, multifatorial e de curso variável. A apresentação inicial no pênis é rara, e mais observada em pacientes HIV positivos. Em pacientes HIV negativos, os casos que ocorrem nesta região, apresentam-se com pápulas, nódulos, placas e lesões verruciformes, assintomáticas. Para o tratamento da forma clássica, dispõem-se de excisão cirúrgica, crioterapia, eletrocirurgia, laser e radioterapia. Neste trabalho, é relatado um caso raro de um paciente com a forma clássica, em região peniana tratado com sucesso com radioterapia.Kaposi's sarcoma is a vascular tumor involving the wall of lymphatic vessels. There are four types: classic, endemic, iatrogenic and HIV-associated. It is a systemic, malignant and multifactorial disease and has a variable course. The primary presentation on the penis is uncommon and is mainly observed in HIV-positive patients. In HIV-negative individuals, asymptomatic papules, nodules, plaques and verrucous lesions are found. The treatment for the classic form involves surgery, cryotherapy, electrosurgery, laser and radiation therapy. The authors present a rare case of a patient with the classic form on the penis, who was successfully treated by radiation therapy.

  19. Radiation-induced Sarcomas Occurring in Desmoid-type Fibromatosis Are Not Always Derived From the Primary Tumor.

    Science.gov (United States)

    Verschoor, Arie J; Cleton-Jansen, Anne-Marie; Wijers-Koster, Pauline; Coffin, Cheryl M; Lazar, Alexander J; Nout, Remi A; Rubin, Brian P; Gelderblom, Hans; Bovée, Judith V M G

    2015-12-01

    Desmoid-type fibromatosis is a rare, highly infiltrative, locally destructive neoplasm that does not metastasize, but recurs often after primary surgery. Activation of the Wnt/β-catenin pathway is the pathogenic mechanism, caused by an activating mutation in exon 3 of CTNNB1 (85% of the sporadic patients). Radiotherapy is a frequent treatment modality with a local control rate of approximately 80%. In very rare cases, this may result in the development of radiation-induced sarcoma. It is unclear whether these sarcomas develop from the primary tumor or arise de novo in normal tissue. In 4 tertiary referral centers for sarcoma, 6 cases of desmoid-type fibromatosis that subsequently developed sarcoma after radiotherapy were collected. The DNA sequence of CTNNB1 exon 3 in the desmoid-type fibromatosis and the subsequent postradiation sarcoma was determined. Sarcomas developed 5 to 21 years after the diagnosis of desmoid-type fibromatosis and included 2 osteosarcomas, 2 high-grade undifferentiated pleomorphic sarcomas, 1 fibrosarcoma, and 1 undifferentiated spindle cell sarcoma. Three patients showed a CTNNB1 hotspot mutation (T41A, S45F, or S45N) in both the desmoid-type fibromatosis and the radiation-induced sarcoma. The other 3 patients showed a CTNNB1 mutation in the original desmoid-type fibromatosis (2 with a T41A and 1 with an S45F mutation), which was absent in the sarcoma. In conclusion, postradiation sarcomas that occur in the treatment area of desmoid-type fibromatosis are extremely rare and can arise through malignant transformation of CTNNB1-mutated desmoid fibromatosis cells, but may also originate from CTNNB1 wild-type normal cells lying in the radiation field.

  20. Primary Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Stomach

    Directory of Open Access Journals (Sweden)

    Safi Khuri

    2016-11-01

    Full Text Available Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET is a tumor of small round cells arising in skeletal tissues. These tumors rarely arise in the stomach. We present a 31-year-old healthy female patient who was admitted to our surgical ward due to upper gastrointestinal hemorrhage. Upper endoscopy revealed a large ulcerated bleeding mass originating from the lesser curvature. Biopsy revealed tumor cell immunoreactivity positive for CD99, vimentin, and Ki67 (an index of proliferation. These findings were compatible with gastric ES/PNET. The fluorescence in situ hybridization analysis result for the EWSR1 gene rearrangement (11: 22 translocation was positive. The patient refused neoadjuvant treatment and thus underwent an operation during which a mass at the lesser curvature of the stomach was found. The mass was adhering to the pancreatic tail and to the mesentery of the transverse and descending colon. Total gastrectomy, distal pancreatectomy, splenectomy, and left adrenalectomy were done. The patient refused adjuvant treatment. She is free of disease 3 years after surgery.

  1. Primary Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Stomach

    Science.gov (United States)

    Khuri, Safi; Gilshtein, Hayim; Sayidaa, Sa'd; Bishara, Bishara; Kluger, Yoram

    2016-01-01

    Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) is a tumor of small round cells arising in skeletal tissues. These tumors rarely arise in the stomach. We present a 31-year-old healthy female patient who was admitted to our surgical ward due to upper gastrointestinal hemorrhage. Upper endoscopy revealed a large ulcerated bleeding mass originating from the lesser curvature. Biopsy revealed tumor cell immunoreactivity positive for CD99, vimentin, and Ki67 (an index of proliferation). These findings were compatible with gastric ES/PNET. The fluorescence in situ hybridization analysis result for the EWSR1 gene rearrangement (11: 22 translocation) was positive. The patient refused neoadjuvant treatment and thus underwent an operation during which a mass at the lesser curvature of the stomach was found. The mass was adhering to the pancreatic tail and to the mesentery of the transverse and descending colon. Total gastrectomy, distal pancreatectomy, splenectomy, and left adrenalectomy were done. The patient refused adjuvant treatment. She is free of disease 3 years after surgery. PMID:27920700

  2. Progressive growth of primary synovial sarcoma of the lung.

    Science.gov (United States)

    Nakano, Jun; Yokomise, Hiroyasu; Huang, Cheng-Long; Misaki, Noriyuki; Chang, Sung-Soo; Okuda, Masaya; Kushida, Yoshio; Haba, Reiji

    2010-06-01

    An 80-year-old male was admitted because of a giant mass in the left lower lobe of the lung on a routine chest X-ray. Chest computed tomography verified this to be a well-defined heterogeneous mass as described with no associated lymphadenopathy. FDG-PET depicted moderately marginal FDG uptake. The patient underwent a left lower lobectomy and lymphadenectomy. Grossly, the tumor measured 60 × 50 mm and was uniformly filled with a pure white, pudding-like friable substance. No lymph node metastasis was observed microscopically. Histologically, the tumor showed a dense proliferation of rounded or spindled malignant cells with a frequent mitotic activity and an increased nuclear-to-cytoplasmic ratio. The immunohistochemical staining was positive for vimentin, negative for cytokeratin, keratin-wide, EMA, CD34. A SYT-SSX2 fusion gene transcript was detected as a result of RT-PCR analysis. Because of these results, the tumor was diagnosed as a monophasic synovial sarcoma.

  3. Quercetin induces apoptosis and autophagy in primary effusion lymphoma cells by inhibiting PI3K/AKT/mTOR and STAT3 signaling pathways.

    Science.gov (United States)

    Granato, Marisa; Rizzello, Celeste; Gilardini Montani, Maria Saveria; Cuomo, Laura; Vitillo, Marina; Santarelli, Roberta; Gonnella, Roberta; D'Orazi, Gabriella; Faggioni, Alberto; Cirone, Mara

    2017-03-01

    Quercetin, a bioflavonoid contained in several vegetables daily consumed, has been studied for long time for its antiinflammatory and anticancer properties. Quercetin interacts with multiple cancer-related pathways such as PI3K/AKT, Wnt/β-catenin and STAT3. These pathways are hyperactivated in primary effusion lymphoma (PEL), an aggressive B cell lymphoma whose pathogenesis is strictly linked to the oncogenic virus Kaposis' Sarcoma-associated Herpesvirus (KSHV). In this study, we found that quercetin inhibited PI3K/AKT/mTOR and STAT3 pathways in PEL cells, and as a consequence, it down-regulated the expression of the prosurvival cellular proteins such as c-FLIP, cyclin D1 and cMyc. It also reduced the release of IL-6 and IL-10 cytokines, leading to PEL cell death. Moreover, quercetin induced a prosurvival autophagy in these cells and increased the cytotoxic effect of bortezomib, a proteasomal inhibitor, against them. Interestingly, quercetin decreased also the expression of latent and lytic KSHV proteins involved in PEL tumorigenesis and up-regulated the surface expression of HLA-DR and calreticulin, rendering the dying cells more likely detectable by the immune system. The results obtained in this study indicate that quercetin, which does not exert any cytotoxicity against normal B cells, may represent a good candidate for the treatment of this aggressive B cell lymphoma, especially in combination with autophagy inhibitors or with bortezomib. Copyright © 2016 Elsevier Inc. All rights reserved.

  4. Primary monophasic synovial sarcoma lung with brain metastasis diagnosed on transthoracic FNAC: Report of a case with literature review

    Directory of Open Access Journals (Sweden)

    Paras Nuwal

    2012-01-01

    Full Text Available Synovial sarcoma is highly malignant tumor of soft tissues, occurring chiefly in the extremities and limb girdle with a propensity for local recurrence and sometimes metastases to the lungs. Primary synovial sarcoma arising in the lungs is rare and brain metastasis as presentation is further uncommon. We report a case of primary monophasic synovial sarcoma lung presenting with brain metastasis in a 35-year-old male patient. The diagnosis was made on percutaneous transthoracic needle aspiration from left-sided pulmonary mass and later confirmed by immunohistochemistry. The utility of preoperative diagnosis by percutaneous aspiration cytology is also stressed.

  5. Primary Ewing’s sarcoma of the spine: case report Sarcoma de Ewing primário da coluna vertebral: relato de caso

    Directory of Open Access Journals (Sweden)

    Leandro I. Dini

    2006-09-01

    Full Text Available Primary malignant sarcomas of the spine are extremely rare. Because of biological heterogeneity, these tumors have variable sensitivity to radiation and chemotherapy. Adequate local control through complete tumor removal is an important therapeutic goal. However, aggressive resection of tumors in the spinal column must be coupled with restoration of spinal column stability and minimization of neural deficits. The balance of these factors makes treatment of primary sarcomas of the spine challenging, and dictates an individual approach to treatment. We report on a 18 years old man with primary Ewing's sarcoma of the nonsacral spine. The clinical picture and imaging characteristics were analyzed as well as the management modalities and outcome.Sarcomas malignos primários da coluna são extremamente raros. Devido sua heterogeneidade biológica, estes tumores apresentam sensibilidade variada à radioterapia e à quimioterapia.O controle local adequado através da ressecção tumoral completa é um objetivo terapêutico importante. Contudo, a ressecção completa dos tumores da coluna vertebral deve ser realizada com a restauração da estabilidade espinhal e minimização do déficit neurológico. O equilíbrio entre estes fatores torna o tratamento dos sarcomas da coluna desafiador, exigindo-se uma abordagem individualizada para o tratamento de cada paciente. Relatamos um caso de sarcoma de Ewing da coluna não-sacral em um homem de 18 anos. O quadro clínico e características de imagem foram analisados, bem como a modalidades de manejo e o prognóstico.

  6. Primary intraoral granulocytic sarcoma: A rare case presenting as generalized gingival enlargement

    Directory of Open Access Journals (Sweden)

    Thayalan Dineshkumar

    2016-01-01

    Full Text Available Granulocytic sarcoma (GS is an extremely rare condition involving infiltration of myeloblasts or immature myeloid cells in an extramedullary site. It is also known as chloroma, myeloid sarcoma or extramedullary myeloid tumor. It usually occurs concomitantly with acute myelogenous leukemia or with the onset of blastic phase of chronic myelogenous leukemia. On rare occasions, it evolves even before the onset of leukemias, and when it precedes leukemias without any overt signs, it is referred to as the primary type. Although GSs can involve any body part, localization in the oral cavity is extremely rare. The recognition of this rare primary entity is important because early aggressive chemotherapy can cause regression of the tumor and improve survival. Here, we report a rare case of GS in a nonleukemic 62-year-old female who presented with generalized gingival enlargement involving both maxilla and mandible.

  7. Genetic profiling differentiates second primary tumors from metastases in adult metachronous soft tissue sarcoma

    DEFF Research Database (Denmark)

    Fernebro, Josefin; Carneiro, Ana; Rydholm, Anders

    2008-01-01

    Purpose. Patients with soft tissue sarcomas (STS) are at increased risk of second primary malignancies, including a second STS, but distinction between metastases and a second primary STS is difficult. Patients and Methods. Array-based comparative genomic hybridization (aCGH) was applied to 30...... multiple STS of the extremities and the trunk wall from 13 patients. Different histotypes were present with malignant fibrous histiocytomas/undifferentiated pleomorphic sarcomas being the predominant subtype. Results. aCGH profiling revealed genetic complexity with multiple gains and losses in all tumors....... In an unsupervised hierarchical cluster analysis, similar genomic profiles and close clustering between the first and subsequent STS were identified in 5 cases, suggesting metastatic disease, whereas the tumors from the remaining 8 patients did not cluster and showed only weak pairwise correlation, suggesting...

  8. Pericardial effusion associated with metastatic disease from an unknown primary tumor in a dog.

    Science.gov (United States)

    Kirsch, J A; Dhupa, S; Cornell, K K

    2000-01-01

    A 6.5-year-old, spayed female Siberian husky presented with signs of cardiac tamponade and weakness. Pleural, pericardial, and abdominal effusion were identified with radiographs and ultrasound. Pericardiocentesis relieved signs of tamponade, and the dog was clinically improved. Pericardial effusion recurred, and pericardiectomy was performed. Histopathological examination of excised tissues failed to reveal evidence of infectious or neoplastic disease. After pericardiectomy, clinically apparent thoracic effusion persisted. The dog was euthanized, and postmortem histopathological examination revealed emboli of metastatic carcinoma cells in the epicardium. The location of intrathoracic disease in this dog made antemortem diagnosis difficult, if not impossible.

  9. Otitis media with effusion (OME in primary care: follow-up protocol

    Directory of Open Access Journals (Sweden)

    José Ignacio BENITO-OREJAS

    2016-07-01

    Full Text Available Introduction and objective: With the intention of improving the quality of care of children with otitis media with effusion (OME, the pediatricians of primary care (PAP have been provided procedures diagnostic and training media, establishing a rule of consensus action. In this paper we present the Protocol developed for the monitoring of OME in children. Method: A team of pediatricians and otolaryngologists from the same Healthcare Area, who have worked to achieve this consensus. Through the published guides and review of the literature on OME, we find his best adaptation to our environment. Results: We expose the necessary steps for the diagnosis, follow-up, referral and documentation of OME in children. Discussion: A correct therapeutic intervention for OME in children involves documenting patient characteristics and clinical findings of the pneumatic otoscopy and tympanometry, perform periodic and vigilant monitoring of potential disruptions, know and apply the reasons for referral to specialized care and maintaining good communication between pediatricians and ENT. Conclusions: The agreement between pediatricians and ENT has helped establish a joint guide of action in children for OME, which together with the improvement of diagnosis, will result in a greater degree of efficiency in the childhood care of OME.

  10. Pathogenesis and Associated Diseases of Kaposi's Sarcoma-associated Herpesvirus

    Institute of Scientific and Technical Information of China (English)

    Lin-ding WANG

    2007-01-01

    Kaposi's sarcoma-associated herpesvirus (KSHV) is the primary etiological agent of Kaposi's sarcoma, primary effusion lymphoma and muticentric Castleman's disease. In common with the other herpesviruses, KSHV exhibits both latent and lytic life cycles, both of which are characterized by distinct gene expression profiles and programs. KSHV encodes proteins which play essential roles in the inhibition of host adaptive and innate immunity, the inhibition of apoptosis, and the regulation of the cell cycle. KSHV also encodes several proteins which have transforming and intrcellular signalling activity.

  11. Primary Sarcoma of the Specialised Prostatic Stroma: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Rosanna Zamparese

    2011-01-01

    Full Text Available Primary sarcoma tumours of the prostate are rare and are classified, according to their histology, as stromal tumours of uncertain malignant potential (STUMP and stromal prostatic sarcoma (PS; low and high grade. We describe a case of a 71-year-old man that developed progressive urinary obstruction symptoms and was subjected to a transurethral prostatic resection (TURP. Histologically, there is a diffuse proliferation of epithelioid and spindle cells that showed rare atypical mitotic figures. Immunohistochemically, the neoplastic cells express diffusely CD34 and focally progesterone whereas no immunoreactivity was seen for cytocheratin, desmin, S-100, Bcl-2, chromogranin, CD117, and actin smooth muscle. A final diagnosis of low-grade prostatic stromal sarcoma (LG-PS was made. This is a really rare neoplasm; in the literature, in fact, to our knowledge, only 6 cases are described and all of these were alive and free of disease at followup. Our patient too is free of disease at 15 months from the diagnosis.

  12. Primary paraesophageal Ewing’s sarcoma: an uncommon case report and literature review

    Directory of Open Access Journals (Sweden)

    Tarazona N

    2015-05-01

    Full Text Available Noelia Tarazona,1,* Lara Navarro,2,* Juan Miguel Cejalvo,3,* Valentina Gambardella,3,* J Alejandro Pérez-Fidalgo,3 Alejo Sempere,2 Samuel Navarro,2 Andrés Cervantes31Department of Medicine, GI and Lymphoma Unit, The Royal Marsden NHS Foundation Trust, London and Surrey, UK; 2Department of Pathology, 3Department of Hematology and Medical Oncology, Biomedical Research Institute, INCLIVA, University of Valencia, Valencia, Spain*These authors contributed equally to this workAbstract: Ewing’s sarcoma is a rare and highly aggressive cancer most frequently arising in people under 20 years of age. We report an uncommon case of primary paraesophageal Ewing’s sarcoma in a 25-year-old male harboring the infrequent EWSR1/ERG fusion transcript with multiple splice variants coexisting in the same tumor. The patient was totally refractory to chemotherapy and died 17 months after diagnosis. We underscore the need for better understanding of the molecular pathogenesis of the disease and improved systemic therapy options.Keywords: Ewing’s sarcoma, recurrence, immunohistochemistry, fusion genes

  13. Primary Synovial Sarcoma of Kidney: A Rare Differential Diagnosis of Renomegaly

    Directory of Open Access Journals (Sweden)

    Gaurang Modi

    2014-01-01

    Full Text Available Synovial sarcomas (SS are classified as subgroup of soft tissue sarcomas affecting mainly extremities of young adults. Primary SS of kidney are very rare tumours with poor prognosis. Though they have characteristic histology and immunohistochemistry (IHC due to rarity of incidence it is difficult to diagnose them. Sometimes chromosomal rearrangement studies are required to confirm the diagnosis. We are presenting a case of 41-year-old male who was referred to our cancer centre for evaluation of left renal mass. CT scan of abdomen revealed a large left renal mass encasing the aorta. Biopsy of renal mass revealed poorly differentiated sarcoma and IHC was positive for vimentin, CD99, and BCL2 and negative for AE1, epithelial membrane antigen, and leukocyte common antigen. The patient was clinically inoperable as renal mass was encasing the aorta. So he was subsequently offered palliative chemotherapy in form of ifosfamide and adriamycin. CT abdomen shows partial response after 3 cycles of chemotherapy according to RECIST criteria.

  14. Primary Vaginal Myeloid Sarcoma: A Rare Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Gaurang Modi

    2015-01-01

    Full Text Available Myeloid sarcoma (chloroma, granulocytic sarcoma, or extramedullary myeloid tumour is an extramedullary mass forming neoplasm composed of myeloid precursor cells. It is usually associated with myeloproliferative disorders but very rarely may precede the onset of leukemia. Here, we are presenting a rare case of primary vaginal myeloid sarcoma in a geriatric female patient without initial presentation of acute myeloid leukemia (AML. A 68-year-old female patient with ECOG Performance Score of 1 presented with pervaginal bleeding for 20 days. On colposcopic examination, she was found to have mass in the anterior fornix of vagina. A punch biopsy specimen revealed chloromatous infiltration of the vagina. LCA (leukocyte common antigen, MPO (myeloperoxidase, and c-kit were strongly positive on IHC (immunohistochemistry. The patient’s routine blood investigations were normal including peripheral smear, lactose dehydrogenase, uric acid, 2D echocardiography, conventional cytogenetics, bone marrow aspiration, and biopsy. The patient was given 4 cycles of decitabine (Decitex, manufactured by Sun Pharmaceutical Industries Limited, India, 20 mg/m2 for 5 days at an interval of 28 days. There was a partial response to decitabine according to RECIST criteria. As decitabine therapy was well tolerated, we are continuing in the same way until disease progression without any complications. The patient is undergoing regular follow-up at our centre.

  15. Sarcoma indiferenciado primário no sistema nervoso central Primary undifferentiated sarcoma of the central nervous system

    Directory of Open Access Journals (Sweden)

    Milton Marcio Machota Junior

    2012-04-01

    Full Text Available INTRODUÇÃO: O sarcoma de sistema nervoso central (SNC é uma neoplasia rara, com incidência de 0,1% a 4,3% dos tumores intracranianos. São tumores agressivos com prognóstico reservado e a maioria é tratada com ressecção radical. RELATO: Homem, 29 anos, com episódios de crises convulsivas e diagnóstico de hemorragia intraparenquimatosa. Durante a cirurgia, foi identificada lesão bem delimitada. A histologia demonstrou neoplasia fusocelular com atipias e numerosas mitoses. Os únicos marcadores imuno-histoquímicos positivos foram vimentina e S-100. O diagnóstico foi de sarcoma indiferenciado de alto grau. CONCLUSÃO: No diagnóstico diferencial de sarcomas de SNC, devem-se excluir lesões metastáticas e gliossarcoma.INTRODUCTION: The central nervous system (CNS sarcoma is a rare neoplasm with an incidence of 0.1% to 4.3% in intracranial tumors. They are aggressive with poor prognosis, and mostly treated with radical resection. REPORT: 29 year-old male patient with episodes of seizures and diagnosed with intraparenchymal hemorrhage. During the surgery a well-defined lesion was identified. Histology showed a spindle cell neoplasm with atypia and numerous mitoses. The immunohistochemical markers were positive only for vimentin and S-100. The diagnosis was high-grade undifferentiated sarcoma. CONCLUSION: Metastatic lesions and gliosarcoma should be excluded in the differential diagnosis of CNS sarcomas.

  16. The Neurological Compromised Spine Due to Ewing Sarcoma. What First: Surgery or Chemotherapy? Therapy, Survival, and Neurological Outcome of 15 Cases With Primary Ewing Sarcoma of the Vertebral Column

    NARCIS (Netherlands)

    Mirzaei, L.; Kaal, S.E.J.; Schreuder, H.W.B.; Bartels, R.H.M.A.

    2015-01-01

    BACKGROUND: The vertebral column is an infrequent site of primary involvement in Ewing sarcoma. Yet when Ewing sarcoma is found in the spine, the urge for decompression is high because of the often symptomatic compression of neural structures. It is unclear in alleviating a neurological deficit whet

  17. A RARE CASE OF ATYPICAL PRIMARY EWING’S SARCOMA OF OCCIPITAL BONE

    Directory of Open Access Journals (Sweden)

    K. Srihari

    2016-07-01

    Full Text Available BACKGROUND Ewing’s Sarcoma is an aggressive malignant neoplasm most frequently manifesting in the second decade of life and accounting for 4% of childhood and adolescent malignancies. These tumours were first described by James Ewing in 1921 as tumours that arise from bone. These osseous lesions have since become infamous for their highly aggressive course with 20% to 30% of patients having evidence of metastasis at the time of diagnosis and an estimated 10-year survival rate of 50%. Metastases to the CNS have most recently been estimated to occur in less than 5% of cases and are usually due to direct extension of an osseous lesion into the extradural space or more rarely through haematogenous spread. CASE REPORT In this article, we report a case of 17-year-old boy who presented to the radiology department with complaints of recurrent episodes of headache and vomiting for the past 3 days. On radiological investigation, there was a large well-defined, lobulated, extra-axial mass lesion measuring 3.6 X 5.7 X 5.9 cm noted in the supratentorial left occipital region which was fairly enhancing after contrast administration. The mass was causing permeative type of destruction of the left occipital bone and extending into extracranial soft tissue. Final diagnosis was done by biopsy and histopathology which showed “Atypical Ewing’s Sarcoma” of the left occipital bone. Considering its unusual site and soft tissue extension, we report this case of Primary Atypical Ewing’s sarcoma of occipital bone. CONCLUSION Primary cranial Ewing's sarcoma is to be considered in the differential diagnosis in children with a tumour involving the skull with destruction of the bone and presence of extra-axial soft tissue swelling. CT is the excellent modality for demonstration of bone destruction while MRI depicts soft tissue extension and metastasis if any.

  18. Primary Ewing's Sarcoma of the Spine in a Two-Year-Old Boy

    Science.gov (United States)

    Electricwala, Jaffer T.

    2016-01-01

    Ewing's Sarcoma (ES) is a highly malignant bone tumour. It may involve any part of the skeleton but the most frequent parts are the ilium and diaphysis of femur and tibia (Alfeeli et al., 2005; Zhu et al., 2012). Primary ES of the spine is extremely rare (Yan et al., 2011). It accounts for only 3.5 to 14.9 percent of all primary bone sarcomas. The age of presentation ranges from 12 to 24 years (median 21 years) (Ferguson, 1999; Sharafuddin et al., 1992; Klimo Jr. et al., 2009). We report an unusual case of primary ES of the spine in a two-year-old boy, who presented to us with paraparesis and features of cauda equina syndrome. MRI scan showed a tumour mass arising from the pedicle of L4 vertebra invading the spinal canal. Tc-99 bone scan showed increased tracer uptake in L4 vertebra and normal tracer uptake elsewhere in the skeleton. After reaching the diagnosis of a space occupying lesion invading the lumber spinal canal, we performed a decompressive laminectomy and a biopsy was sent which confirmed the diagnosis of ES. Immunohistochemistry showed tumour cells staining positive for CD-99 (specific stain for ES). Gene testing showed an EWS-FLI 1 chimera. Surgery was followed by good improvement in motor signs. The child was then referred to a specialized oncotherapy centre for further treatment, radiation, and chemotherapy. To the best of our knowledge, we are the first to report primary ES of the spine at the age of two years. PMID:27895949

  19. Primary Ewing’s Sarcoma of the Spine in a Two-Year-Old Boy

    Directory of Open Access Journals (Sweden)

    Ali J. Electricwala

    2016-01-01

    Full Text Available Ewing’s Sarcoma (ES is a highly malignant bone tumour. It may involve any part of the skeleton but the most frequent parts are the ilium and diaphysis of femur and tibia (Alfeeli et al., 2005; Zhu et al., 2012. Primary ES of the spine is extremely rare (Yan et al., 2011. It accounts for only 3.5 to 14.9 percent of all primary bone sarcomas. The age of presentation ranges from 12 to 24 years (median 21 years (Ferguson, 1999; Sharafuddin et al., 1992; Klimo Jr. et al., 2009. We report an unusual case of primary ES of the spine in a two-year-old boy, who presented to us with paraparesis and features of cauda equina syndrome. MRI scan showed a tumour mass arising from the pedicle of L4 vertebra invading the spinal canal. Tc-99 bone scan showed increased tracer uptake in L4 vertebra and normal tracer uptake elsewhere in the skeleton. After reaching the diagnosis of a space occupying lesion invading the lumber spinal canal, we performed a decompressive laminectomy and a biopsy was sent which confirmed the diagnosis of ES. Immunohistochemistry showed tumour cells staining positive for CD-99 (specific stain for ES. Gene testing showed an EWS-FLI 1 chimera. Surgery was followed by good improvement in motor signs. The child was then referred to a specialized oncotherapy centre for further treatment, radiation, and chemotherapy. To the best of our knowledge, we are the first to report primary ES of the spine at the age of two years.

  20. Primary vaginal Ewing's sarcoma or primitive neuroectodermal tumor in a 17-year-old woman: a case report

    Directory of Open Access Journals (Sweden)

    Kurkure Purna

    2010-03-01

    Full Text Available Abstract Introduction Primary Ewing's sarcoma or primitive neuroectodermal tumor of the genital tract of women is uncommon. Rarer still is its occurrence in the vagina, with only five cases described so far. Out of these, only one case was confirmed using molecular analysis. Case presentation We present an extremely rare case of Ewing's sarcoma or primitive neuroectodermal tumor in a 17-year-old Indian girl. She presented with a vaginal mass that was initially diagnosed as a malignant round cell tumor. Immunohistochemistry showed diffuse positivity for vimentin, membranous positivity for MIC2, and positivity for BCL2 and FLI-1. On the other hand, she was negative for cytokeratin, epithelial membrane antigen, desmin, Myo D-1, myogenin and smooth muscle actin. A diagnosis of primitive neuroectodermal tumor was thus offered. Furthermore, a molecular analysis of our patient using reverse transcription-polymerase chain reaction technique showed positivity for t(11; 22 (q24; q12 (EWSR1-FLI1, thus confirming the diagnosis of a Ewing's sarcoma/primitive neuroectodermal tumor. Our patient was offered chemotherapy on Institutional protocol EFT 2001. Conclusion This is a rare case of primary vaginal Ewing's sarcoma or primitive neuroectodermal tumor, which was confirmed with molecular analysis, in the youngest patient known so far. This study reinforces the value of integrating morphological features with membranous MIC2 positivity, along with application of molecular techniques in objective identification of an Ewing's sarcoma or primitive neuroectodermal tumor at uncommon sites.

  1. Primary Intimal (Spindle Cell Sarcoma of the Heart: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    A. Ibrahim

    2013-01-01

    Full Text Available Intimal (spindle cell sarcomas of the left atrium are extremely rare primary cardiac tumours with three cases reported (Li et al. (2013, Cho et al. (2006, and Modi et al. (2009. We present a 69-year-old man who first came to medical attention after experiencing abdominal discomfort. He had a 30 lb weight loss apparently due to dieting. He denied any other constitutional symptoms. His symptoms persisted despite a course of antibiotics for presumed diverticulitis. Laboratory values were within normal limits, though the haemoglobin was 131 g/L (normal: 140–180. Subsequent abdominal computed tomography (CT scan revealed an abdominal wall mass and intracardiac lesion; the cardiac mass was further characterized by transesophageal echo (TEE, magnetic resonance imaging (MRI, and dedicated cardiac CT. TEE revealed a mass attached to the posterolateral wall of the left atrium above the mitral annulus, and the cardiac CT and MRI confirmed the TEE findings. The patient underwent extensive surgical resection and repair of the left side of the heart. Postoperatively, he developed acute renal failure requiring dialysis and reintubation for volume overload. He became acutely hypotensive, developed multiorgan failure, and succumbed to his illness. Histopathologic examination of the left atrial mass showed an intimal sarcoma.

  2. Primary renal undifferentiated sarcoma as an infiltrative mass in a 12 year old boy

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Yong Hee; Kim, Myung Joon; Lee, Mi Jung [Dept. of Radiology and Research Institute of Radiological Science, Severance Children' s Hospital, Yonsei University College of Medicine, Seoul (Korea, Republic of); Kim, Se Hwa [Dept. of Pathology, Severance Hospital, Yonsei University College of Medicine, Seoul (Korea, Republic of)

    2015-09-15

    Undifferentiated sarcomas are rare tumors not classified into any sarcoma subtype. Due to their rarity, imaging findings of undifferentiated sarcomas are poorly characterized. The purpose of this report was to present imaging findings of a pathologically confirmed undifferentiated sarcoma originated from the left kidney of a 12-year-old boy. The mass was infiltrative involving the renal pelvis. It mimicked massive hilar lymphadenopathy with a preserved renal contour visible by both ultrasonography and CT. Renal vein thrombosis was also observed. Although undifferentiated sarcomas are rare, they should be considered in differential diagnosis of infiltrative renal masses with renal pelvis invasion in children.

  3. Primary Mediastinal Synovial Sarcoma Presenting as Superior Vena Cava Syndrome: A Rare Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Irappa Madabhavi

    2015-01-01

    Full Text Available Primary mediastinal sarcomas are aggressive tumors with a very rare incidence. This report describes the case of a 35-year-old male patient who presented with acute symptoms of dyspnoea, facial puffiness, voice-hoarseness, and engorged neck veins. With the clinical picture consistent with the superior vena cava (SVC syndrome, the patient was investigated with computed tomography of the chest. This revealed a large soft tissue density mass lesion compressing the SVC along with other critical superior mediastinal structures. Histopathological evaluation of the mass revealed features consistent with a soft tissue sarcoma and positive staining was observed for vimentin and S-100. Cytogenetic analysis by fluorescent in situ hybridisation (FISH demonstrated the t(X:18 translocation. Thus diagnosis was established as primary mediastinal synovial sarcoma. Patient was treated with three cycles of neoadjuvant chemotherapy, to which there was a partial response as per the RECIST criteria. Surgical excision of the mediastinal mass was performed, and further postoperative treatment with adjuvant chemoradiotherapy was provided. Patient currently is free of disease. This is to the best of our knowledge the first report in the world literature of a successfully treated case of “primary mediastinal sarcomas presenting as SVC syndrome.” Patient is under regular surveillance at our clinic and remains free of recurrence one year after treatment completion.

  4. Primary effusion lymphoma associated with Human Herpes Virus-8 and Epstein Barr virus in an HIV-infected woman from Kampala, Uganda: a case report

    Directory of Open Access Journals (Sweden)

    Osuwat Lawrence O

    2011-02-01

    Full Text Available Abstract Introduction Primary effusion lymphoma is a recently recognized entity of AIDS related non-Hodgkin lymphomas. Despite Africa being greatly affected by the HIV/AIDS pandemic, an extensive MEDLINE/PubMed search failed to find any report of primary effusion lymphoma in sub-Saharan Africa. To our knowledge this is the first report of primary effusion lymphoma in sub-Saharan Africa. We report the clinical, cytomorphologic and immunohistochemical findings of a patient with primary effusion lymphoma. Case presentation A 70-year-old newly diagnosed HIV-positive Ugandan African woman presented with a three-month history of cough, fever, weight loss and drenching night sweats. Three weeks prior to admission she developed right sided chest pain and difficulty in breathing. On examination she had bilateral pleural effusions. Haematoxylin and eosin stained cytologic sections of the formalin-fixed paraffin-embedded cell block made from the pleural fluid were processed in the Department of Pathology, Makerere University, College of Health Sciences, Kampala, Uganda. Immunohistochemistry was done at the Institute of Haematology and Oncology "L and A Seragnoli", Bologna University School of Medicine, Bologna, Italy, using alkaline phosphatase anti-alkaline phosphatase method. In situ hybridization was used for detection of Epstein-Barr virus. The tumor cells were CD45+, CD30+, CD38+, HHV-8 LANA-1+; but were negative for CD3-, CD20-, CD19-, and CD79a- and EBV RNA+ on in situ hybridization. CD138 and Ki-67 were not evaluable. Our patient tested HIV positive and her CD4 cell count was 127/μL. Conclusions A definitive diagnosis of primary effusion lymphoma rests on finding a proliferation of large immunoblastic, plasmacytoid and anaplastic cells; HHV-8 in the tumor cells, an immunophenotype that is CD45+, pan B-cell marker negative and lymphocyte activated marker positive. It is essential for clinicians and pathologists to have a high index of suspicion of

  5. Alpha Interferon Inhibits Human Herpesvirus 8 (HHV-8) Reactivation in Primary Effusion Lymphoma Cells and Reduces HHV-8 Load in Cultured Peripheral Blood Mononuclear Cells

    Science.gov (United States)

    Monini, Paolo; Carlini, Francesca; Stürzl, Michael; Rimessi, Paola; Superti, Fabiana; Franco, Marina; Melucci-Vigo, Gianna; Cafaro, Aurelio; Goletti, Delia; Sgadari, Cecilia; Butto’, Stefano; Leone, Patrizia; Leone, Pasqualina; Chiozzini, Chiara; Barresi, Caterina; Tinari, Antonella; Bonaccorsi, Angela; Capobianchi, Maria R.; Giuliani, Massimo; di Carlo, Aldo; Andreoni, Massimo; Rezza, Giovanni; Ensoli, Barbara

    1999-01-01

    Infection by human herpesvirus 8 (HHV-8) is associated with the development of Kaposi’s sarcoma (KS). Since regression of KS can be achieved by treatment of the patients with alpha interferon (IFN-α), we analyzed the effects of IFN-α or anti-IFN-α antibodies (Ab) on HHV-8 latently infected primary effusion lymphoma-derived cell lines (BCBL-1 and BC-1) and on peripheral blood mononuclear cells (PBMC) from patients with all forms of KS and from at-risk subjects. IFN-α inhibited in a dose-dependent manner the amplification of HHV-8 DNA in BCBL-1 cells induced to lytic infection with tetradecanoyl phorbol acetate (TPA). This effect was associated with the inhibition of the expression of HHV-8 nut-1 and kaposin genes that are induced early and several hours, respectively, after TPA treatment. In addition, IFN-α inhibited virus production and/or release from BCBL-1 cells. Inhibition of nut-1 and kaposin genes by IFN-α was also observed in BC-1 cells induced with n-butyrate. Conversely, the addition of anti-IFN-α Ab to TPA-induced BCBL-1 cells resulted in a larger number of mature enveloped particles and in a more extensive cytopathic effect due to the neutralization of the endogenous IFN produced by these cells. IFN was also produced by cultured PBMC from HHV-8-infected individuals, and this was associated with a loss of viral DNA during culture. However, the addition of anti-IFN-α Ab or anti-type I IFN receptor Ab promoted the maintenance of HHV-8 DNA in these cells that was associated with the detection of the latency-associated kaposin RNA. Finally, the addition of IFN-α reduced the HHV-8 load in PBMC. Thus, IFN-α appears to have inhibitory effects on HHV-8 persistent infection of PBMC. These results suggest that, in addition to inhibiting the expression of angiogenic factors that are key to KS development, IFN-α may induce KS regression by reducing the HHV-8 load and/or inhibiting virus reactivation. PMID:10196299

  6. Kaposi's sarcoma-associated herpesvirus contains G protein-coupled receptor and cyclin D homologs which are expressed in Kaposi's sarcoma and malignant lymphoma.

    OpenAIRE

    1996-01-01

    A new human herpesvirus was recently identified in all forms of Kaposi's sarcoma (Kaposi's sarcoma-associated herpesvirus [KSHV] or human herpesvirus 8), as well as in primary effusion (body cavity-based) lymphomas (PELs). A 12.3-kb-long KSHV clone was obtained from a PEL genomic library. Sequencing of this clone revealed extensive homology and colinearity with the right end of the herpesvirus saimiri (HVS) genome and more limited homology to the left end of the Epstein-Barr virus genome. Fou...

  7. Primary alveolar soft part sarcoma of fibula demonstrating ASPL-TFE3 fusion: a case report and review of the literature

    Energy Technology Data Exchange (ETDEWEB)

    Aisner, Seena C.; Mirani, Neena; Hameed, Meera [University Hospital, Department of Pathology, Newark, NJ (United States); New Jersey Medical School, Newark (United States); Beebe, Kathleen [University Hospital, Department of Orthopedic Surgery, Newark, NJ (United States); New Jersey Medical School, Newark (United States); Blacksin, Marcia [University Hospital, Department of Radiology, Newark, NJ (United States); New Jersey Medical School, Newark (United States)

    2008-11-15

    Alveolar soft part sarcoma is a rare soft tissue tumor typically affecting young adults. These tumors are most often seen in the deep soft tissues of the extremities and patients generally present with advanced disease. Primary bone involvement is extremely rare and has only been reported in seven cases. This is the first case of alveolar soft part sarcoma in bone documenting the ASPL-TFE3 gene product. Herein, we report a rare presentation of alveolar soft part sarcoma presenting as a primary bone neoplasm involving the proximal fibula in a 41-year-old woman. (orig.)

  8. Primary Pulmonary Poorly Differentiated Synovial Sarcoma: Transducer-Like Enhancer of Split 1 Immunohistochemistry as A Valuable Diagnostic Aid

    Science.gov (United States)

    Kaur, Amulyajit; Tazelaar, Henry Dale; Sahai, Kavita

    2013-01-01

    Poorly differentiated primary pulmonary synovial sarcomas are rare and challenging for a surgical pathologist to diagnose. Although the demonstration of the tumor specific translocation, t (x; 18)(p11.2;q11.2) or the resultant fusion gene (SYT-SSX) is the gold standard for diagnosis, this test is not always accessible. We report the use of immunohistochemistry, including transducer-like enhancer of split-1 in the diagnosis of one such tumor in a young individual. PMID:24014971

  9. Concomitant Kaposi sarcoma and multicentric Castleman's disease in a heart transplant recipient.

    Science.gov (United States)

    Patel, Ami; Bishburg, Eliahu; Zucker, Mark; Tsang, Patricia; Nagarakanti, Sandhya; Sabnani, Indu

    2014-01-01

    Post-transplant human herpes virus -8 (HHV-8)/Kaposi sarcoma herpes virus (KSHV) infection is associated with neoplastic and non-neoplastic diseases. Kaposi sarcoma (KS), multicentric Castleman's disease (MCD), and primary effusion lymphomas (PEL) are the most common HHV-8-associated neoplastic complications described in solid organ transplant (SOT) patients. Concurrent KS and MCD have been previously described after transplantation only twice - once after liver transplantation and once after renal transplantation. We describe a unique heart transplant patient who also developed concurrent KS and MCD. To our knowledge this is the first documented case of a heart transplant recipient presenting with these two HHV-8-mediated complications at the same time.

  10. Treatment of Kaposi sarcoma-associated herpesvirus (KSHV-associated cancers

    Directory of Open Access Journals (Sweden)

    Dirk P Dittmer

    2012-04-01

    Full Text Available Kaposi sarcoma (KS is the most frequent AIDS-defining cancer worldwide. Kaposi sarcoma associated herpesvirus (KSHV is the etiological agent of KS, and the virus is also associated with two lymphoproliferative diseases. Both KS and KSHV-associated lymphomas, are cancers of unique molecular composition. They represent a challenge for cancer treatment and an opportunity to identify new mechanisms of transformation. Here, we review the current clinical insights into KSHV-associated cancers and discuss scientific insights into the pathobiology of KS, primary effusion lymphoma and multicentric Castleman’s disease.

  11. Primary Ewing's Sarcoma of the Kidney in a 73-Year-Old Man.

    Science.gov (United States)

    Wedde, T B; Lobmaier, I V K; Brennhovd, B; Lohne, F; Hall, K S

    2011-01-01

    Objective. Ewing's sarcoma of the kidney is rare and is usually found in young adults. We present here a single case study of Ewing's sarcoma found in an elderly man. Material and methods. A 73-year-old man underwent routine surgery for hydrocoele of the testis. He developed urinary obstruction symptoms, and radiological examinations revealed a tumour in the right kidney. Results. Microscopical, immunohistochemical, and molecular pathological analysis of the tumour was consistent with Ewing's sarcoma. FISH showed rearrangement of chromosomes 22q12 (EWSR1). The patient subsequently underwent nephrectomy followed by 6 adjuvant chemotherapy cycles. Follow-up after 7 months shows no recurrence. Conclusion. This case report presents not only the rare finding of Ewing's sarcoma in the kidney, but also the occurrence of this tumour entity in an elderly patient. Treatment options for the different types of renal tumours are vastly different and the need for a correct diagnosis is, therefore, vital.

  12. Primary Ewing’s Sarcoma of the Kidney in a 73-Year-Old Man

    Directory of Open Access Journals (Sweden)

    T. B. Wedde

    2011-01-01

    Full Text Available Objective. Ewing’s sarcoma of the kidney is rare and is usually found in young adults. We present here a single case study of Ewing's sarcoma found in an elderly man. Material and methods. A 73-year-old man underwent routine surgery for hydrocoele of the testis. He developed urinary obstruction symptoms, and radiological examinations revealed a tumour in the right kidney. Results. Microscopical, immunohistochemical, and molecular pathological analysis of the tumour was consistent with Ewing's sarcoma. FISH showed rearrangement of chromosomes 22q12 (EWSR1. The patient subsequently underwent nephrectomy followed by 6 adjuvant chemotherapy cycles. Follow-up after 7 months shows no recurrence. Conclusion. This case report presents not only the rare finding of Ewing's sarcoma in the kidney, but also the occurrence of this tumour entity in an elderly patient. Treatment options for the different types of renal tumours are vastly different and the need for a correct diagnosis is, therefore, vital.

  13. Management of malignant pleural effusions.

    LENUS (Irish Health Repository)

    Uzbeck, Mateen H

    2010-06-01

    Malignant pleural effusions are a common clinical problem in patients with primary thoracic malignancy and metastatic malignancy to the thorax. Symptoms can be debilitating and can impair tolerance of anticancer therapy. This article presents a comprehensive review of pharmaceutical and nonpharmaceutical approaches to the management of malignant pleural effusion, and a novel algorithm for management based on patients\\' performance status.

  14. Recurrent Primary Synovial Sarcoma of Median Nerve: A Case Report and Literature Review.

    Science.gov (United States)

    Bhat, A K; Jayakrishnan, K N; Acharya, A M

    2016-10-01

    We report a case of intraneural synovial sarcoma of the median nerve in a 39 year old lady with multiple local recurrences over thirteen years with no distant metastasis. The diagnosis of biphasic type of synovial sarcoma was confirmed by histopathology and immunohistochemistry. At the time of the fourth recurrence below elbow amputation was performed. This case is being reported for its rarity and propensity for local recurrence without distant metastasis.

  15. Effusion cytomorphology of small round cell tumors

    Directory of Open Access Journals (Sweden)

    Katsuhide Ikeda

    2016-01-01

    Conclusion: We believe that it might be possible to diagnose DLBL and SCLC from cytologic analysis of effusion samples but it is very difficult to use this method to distinguish EWS, synovial sarcoma, and rhabdomyosarcoma. Statistical and mathematical analyses indicated that nuclear density and dispersion of nuclear and cytoplasmic sizes are useful adjuncts to conventional cytologic diagnostic criteria, which are acquired from experience.

  16. Primary Ewing's sarcoma/primitive neuroectodermal tumor of the urogenital tract in children

    Institute of Scientific and Technical Information of China (English)

    SONG Hong-cheng; SUN Ning; ZHANG Wei-ping; HUANG Cheng-ru

    2012-01-01

    Background Primary Ewing's sarcoma/primitive neuroectodermal tumor (ES/PNET) of urogenital tract is a rare condition with non-specific clinical presentations,which can make it difficult to diagnose.In this study,we summarize the clinical presentation,pathological features,therapeutic strategies,and prognosis of ES/PNET.Methods Clinical information on two cases of ES/PNET in the penis and ureter was analyzed,and relevant literature was reviewed.Results ES/PNET was confirmed pathologically,immunohistochemically and via molecular biology techniques in the penis (n=1) and ureter (n=1).In one case,a tumor was found at the base of penis,which had invaded the corpus cavernosum,and resulted in a massive enlargement of the penis.This tumor was initially diagnosed as an endocrine disorder.However,a confirmed diagnosis was made 11 months later when massive metastases in both lungs were noted.A tumor biopsy was performed to confirm the diagnosis,and chemotherapy with a CAV (cyclophosphamide+doxorubicin+vincristine) + IE (ifosfamide+ etoposide) regimen for 9 months was prescribed.In the second case,a child was admitted due to abdominal pain and a hydroureter in the right kidney,as determined by ultrasonography.A tumor was found in the right ureter at the level of iiiac vessels.Removal of the tumor and ureteral anastomosis were performed,and chemotherapy with CAV+IE for 8 months were prescribed.Both patients are currently being followed-up closely.Conclusions ES/PNET is a highly malignant tumor and has poor prognosis.Pre-operative diagnosis of ES/PNET of urogenital tract is difficult and largely depends on pathology,immunohistochemistry,and,if applicable,molecular biology.Comprehensive therapy may include surgery,chemotherapy and radiotherapy.

  17. Immediate chest wall reconstruction during pregnancy: surgical management after extended surgical resection due to primary sarcoma of the breast.

    Science.gov (United States)

    Arruda, Eduardo Gustavo; Munhoz, Alexandre Mendonça; Montag, Eduardo; Filassi, José Roberto; Gemperli, Rolf

    2014-01-01

    Breast sarcoma during pregnancy is an extremely rare event and represents a complex problem because of a more advanced stage at presentation. This report presents the first case of a 24-year-old woman with a gestational age of 20 weeks with a fast growing tumour in her left breast (29 × 19 × 15 cm) and infiltrating the skin/pectoralis muscles. Radical mastectomy was performed with a gestational age of 22 weeks and a different design was planned for the latissimus dorsi musculocutaneous flap (LDMF) with primary closure in the V-Y pattern. Satisfactory chest wall coverage and contour were achieved. Final histopathological findings allowed a diagnosis of undifferentiated sarcoma. With a gestational age of 37 weeks, a healthy infant was delivered by means of a caesarean section. The patient is currently in the second postoperative year and no recurrence has been observed. Management of a large breast sarcoma in a pregnant patient presents unique challenges in consideration of the potential risks to the foetus and the possible maternal benefit. The results of this study demonstrate that the VY-LDMF is a reliable technique and should be considered in cases of immediate large thoracic wound reconstruction. Copyright © 2013 British Association of Plastic, Reconstructive and Aesthetic Surgeons. Published by Elsevier Ltd. All rights reserved.

  18. [Primary Synovial Sarcoma in the Anterior Mediastinum;Report of a Case].

    Science.gov (United States)

    Yanagawa, Naoki; Shiono, Satoshi; Katahira, Masato; Osakabe, Mitsumasa; Abiko, Masami; Ogata, Shinya

    2016-06-01

    We report a rare case of synovial sarcoma in the anterior mediastinum. A 43-year-old man consulted our hospital with a complaint of dyspnea and chest discomfort. Chest computed tomography revealed an anterior mediastinal mass. Small open biopsy was performed, and the pathological examination revealed spindle-shaped cells with severe atypia. Tumor resection was performed. On pathology, fascicular and storiform patterns of spindle-shaped cells with severe atypia were noted. The tumor cells were positive for cytokeratin 7, vimentin, Bcl -2 and CD99, and the amplification of SYT-SSX fusion gene was also found. Therefore it was diagnosed as a synovial sarcoma.

  19. Targeted therapy for sarcomas

    Directory of Open Access Journals (Sweden)

    Forscher C

    2014-03-01

    Full Text Available Charles Forscher,1 Monica Mita,2 Robert Figlin3 1Sarcoma Program, Samuel Oschin Comprehensive Cancer Institute, Cedars-Sinai Medical Center, Los Angeles, CA, USA; 2Experimental Therapeutics Program, Samuel Oschin Comprehensive Cancer Institute, Cedars-Sinai Medical Center, Los Angeles, CA, USA; 3Academic Development Program, Samuel Oschin Comprehensive Cancer Institute, and Division of Hematology/Oncology, Cedars-Sinai Medical Center, Los Angeles, CA, USA Abstract: Sarcomas are tumors of mesenchymal origin that make up approximately 1% of human cancers. They may arise as primary tumors in either bone or soft tissue, with approximately 11,280 soft tissue tumors and 2,650 bone tumors diagnosed each year in the United States. There are at least 50 different subtypes of soft tissue sarcoma, with new ones described with ever-increasing frequency. One way to look at sarcomas is to divide them into categories on the basis of their genetic make-up. One group of sarcomas has an identifiable, relatively simple genetic signature, such as the X:18 translocation seen in synovial sarcoma or the 11:22 translocation seen in Ewing's sarcoma. These specific abnormalities often lead to the presence of fusion proteins, such as EWS-FLI1 in Ewing's sarcoma, which are helpful as diagnostic tools and may become therapeutic targets in the future. Another group of sarcomas is characterized by complex genetic abnormalities as seen in leiomyosarcoma, osteosarcoma, and undifferentiated sarcoma. It is important to keep these distinctions in mind when contemplating the development of targeted agents for sarcomas. Different abnormalities in sarcoma could be divided by tumor subtype or by the molecular or pathway abnormality. However, some existing drugs or drugs in development may interfere with or alter more than one of the presented pathways. Keywords: sarcoma, targeted agents, tyrosine kinase inhibitors, mTor inhibition

  20. A case of primary mediastinal Ewing′s sarcoma /primitive neuroectodermal tumor presenting with initial compression of superior vena cava

    Directory of Open Access Journals (Sweden)

    Alessia Reali

    2013-01-01

    Full Text Available Ewing′s sarcomas and peripheral primitive neuroectodermal tumors (ES/PNETs are high grade malignant neoplasms. These malignancies are characterized by a chromosome 22 rearrangement, arise from bone or soft tissue, predominantly affect children and young adults, and are grouped in the Ewing family of tumors. Multimodality treatment programs are the treatment of choice. Primary localization of ES/PNET in the mediastinum is extremely rare. We describe a case of ES/PNET presenting as a mediastinal mass with tracheal compression and initial signs of superior vena cava in a 66-year-old woman.

  1. Breast sarcomas. Literature review

    Directory of Open Access Journals (Sweden)

    D. A. Ryabchikov

    2014-01-01

    Full Text Available The article presents an overview of the literature about breast sarcomas (nonepithelial malignances. Primary sarcomas are extremely rare, with less than 1 % of all malignant tumors of the breast. Breast carcinomas cause an increased interest of the scientists due to their unique clinical and pathological features and unpredictable prognosis.

  2. Primary peripheral neurolymphomatosis mimicking synovial sarcoma: FDG PETCT to the rescue

    Directory of Open Access Journals (Sweden)

    S Padma

    2014-01-01

    Full Text Available Our understanding of the association between synovial sarcoma and peripheral neurolymphomatosis is limited to a few case reports in literature. Delay in diagnosis or misdiagnosis is possible due to its insidious onset and varied presentation compounded by non-specific imaging findings. Needle biopsy also may not be confirmatory especially, in cases of biphasic sarcoma as in our case, and it may be necessary to proceed to open biopsy. Here, is a case of a non-tender right calf muscle mass, which was reported as biphasic synovial sarcoma by FNAC. Positron emission tomography computed tomography - computed tomography (PETCT showed right sciatic nerve involvement and multiple infra diaphragmatic lymph nodal lesions. Intensity of 18 F FDG ( 18 Flourine labeled fluro de oxy glucose uptake and the infra diaphragmatic lymph nodal lesions distribution, was more in favour of a lymphoma diagnosis rather than a sarcoma, (which are usually low metabolically active tumors. Thus, this case highlights the usefulness of FDG PETCT in arriving at a diagnosis in the background of indeterminate clinicopathological and radiologic findings.

  3. Primary Kaposi's sarcoma in lymph nodes concurrent with chronic lymphatic leukemia.

    Science.gov (United States)

    Weshler, Z; Leviatan, A; Krasnokuki, D; Kopolovitch, J

    1979-02-01

    Both Kaposi's sarcoma and chronic lymphatic leukemia affect the lymph nodes, and not infrequently, the same patient. The authors describe the occurrence of both diseases in the same lymph node. The rarity of this finding suggests different histopathogenic origins of the two diseases.

  4. Routine bone scintigraphy in primary staging of soft tissue sarcoma - Is it worthwhile?

    NARCIS (Netherlands)

    Jager, PL; Hoekstra, HJ; Leeuw, JA; van der Graaf, WTA; de Vries, EGE; Piers, DA

    2000-01-01

    BACKGROUND. The incidence of bone metastases in soft tissue sarcoma (STS) patients seems to be low but has not been studied separately. In this study, the authors aimed to determine the value of routine radionuclide bone scanning in preoperative staging of STS patients. METHODS. Preoperative bone

  5. Routine bone scintigraphy in primary staging of soft tissue sarcoma - Is it worthwhile?

    NARCIS (Netherlands)

    Jager, PL; Hoekstra, HJ; Leeuw, JA; van der Graaf, WTA; de Vries, EGE; Piers, DA

    2000-01-01

    BACKGROUND. The incidence of bone metastases in soft tissue sarcoma (STS) patients seems to be low but has not been studied separately. In this study, the authors aimed to determine the value of routine radionuclide bone scanning in preoperative staging of STS patients. METHODS. Preoperative bone sc

  6. Primary B Lymphocytes Infected with Kaposi's Sarcoma-Associated Herpesvirus Can Be Expanded In Vitro and Are Recognized by LANA-Specific CD4+ T Cells

    Science.gov (United States)

    Nicol, Samantha M.; Sabbah, Shereen; Brulois, Kevin F.; Jung, Jae U.; Bell, Andrew I.

    2016-01-01

    ABSTRACT Kaposi's sarcoma-associated herpesvirus (KSHV) has tropism for B lymphocytes, in which it establishes latency, and can also cause lymphoproliferative disorders of these cells manifesting as primary effusion lymphoma (PEL) and multicentric Castleman disease (MCD). T cell immunity is vital for the control of KSHV infection and disease; however, few models of B lymphocyte infection exist to study immune recognition of such cells. Here, we developed a model of B lymphocyte infection with KSHV in which infected tonsillar B lymphocytes were expanded by providing mitogenic stimuli and then challenged with KSHV-specific CD4+ T cells. The infected cells expressed viral proteins found in PELs, namely, LANA and viral IRF3 (vIRF3), albeit at lower levels, with similar patterns of gene expression for the major latency, viral interleukin 6 (vIL-6), and vIRF3 transcripts. Despite low-level expression of open reading frame 50 (ORF50), transcripts for the immune evasion genes K3 and K5 were detected, with some downregulation of cell surface-expressed CD86 and ICAM. The vast majority of infected lymphocytes expressed IgM heavy chains with Igλ light chains, recapitulating the features seen in infected cells in MCD. We assessed the ability of the infected lymphocytes to be targeted by a panel of major histocompatibility complex (MHC) class II-matched CD4+ T cells and found that LANA-specific T cells restricted to different epitopes recognized these infected cells. Given that at least some KSHV latent antigens are thought to be poor targets for CD8+ T cells, we suggest that CD4+ T cells are potentially important effectors for the in vivo control of KSHV-infected B lymphocytes. IMPORTANCE KSHV establishes a latent reservoir within B lymphocytes, but few models exist to study KSHV-infected B cells other than the transformed PEL cell lines, which have likely accrued mutations during the transformation process. We developed a model of KSHV-infected primary B lymphocytes that

  7. Kaposi Sarcoma-associated Herpesvirus: mechanisms of oncogenesis.

    Science.gov (United States)

    Schulz, Thomas F; Cesarman, Ethel

    2015-10-01

    Kaposi Sarcoma-associated Herpesvirus (KSHV, HHV8) causes three human malignancies, Kaposi Sarcoma (KS), an endothelial tumor, as well as Primary Effusion Lymphoma (PEL) and the plasma cell variant of Multicentric Castleman's Disease (MCD), two B-cell lymphoproliferative diseases. All three cancers occur primarily in the context of immune deficiency and/or HIV infection, but their pathogenesis differs. KS most likely results from the combined effects of an endotheliotropic virus with angiogenic properties and inflammatory stimuli and thus represents an interesting example of a cancer that arises in an inflammatory context. Viral and cellular angiogenic and inflammatory factors also play an important role in the pathogenesis of MCD. In contrast, PEL represents an autonomously growing malignancy that is, however, still dependent on the continuous presence of KSHV and the action of several KSHV proteins.

  8. AIDS-associated Kaposi's sarcoma is linked to advanced disease and high mortality in a primary care HIV programme in South Africa

    Directory of Open Access Journals (Sweden)

    Chu Kathryn M

    2010-07-01

    Full Text Available Abstract Background AIDS-associated Kaposi's sarcoma is an important, life-threatening opportunistic infection among people living with HIV/AIDS in resource-limited settings. In western countries, the introduction of combination antiretroviral therapy (cART and new chemotherapeutic agents has resulted in decreased incidence and improved prognosis of AIDS-associated Kaposi's sarcoma. In African cohorts, however, mortality remains high. In this study, we describe disease characteristics and risk factors for mortality in a public sector HIV programme in South Africa. Methods We analysed data from an observational cohort study of HIV-infected adults with AIDS-associated Kaposi's sarcoma, enrolled between May 2001 and January 2007 in three primary care clinics. Paper records from primary care and tertiary hospital oncology clinics were reviewed to determine the site of Kaposi's sarcoma lesions, immune reconstitution inflammatory syndrome stage, and treatment. Baseline characteristics, cART use and survival outcomes were extracted from an electronic database maintained for routine monitoring and evaluation. Cox regression was used to model associations with mortality. Results Of 6292 patients, 215 (3.4% had AIDS-associated Kaposi's sarcoma. Lesions were most commonly oral (65% and on the lower extremities (56%. One quarter of patients did not receive cART. The mortality and lost-to-follow-up rates were, respectively, 25 (95% CI 19-32 and eight (95% CI 5-13 per 100 person years for patients who received cART, and 70 (95% CI 42-117 and 119 (80-176 per 100 person years for patients who did not receive cART. Advanced T stage (adjusted HR, AHR = 5.3, p Patients with AIDS-associated Kaposi's sarcoma presented with advanced disease and high rates of mortality and loss to follow up. Risk factors for mortality included advanced Kaposi's sarcoma disease and lack of chemotherapy use. Contributing factors to the high mortality for patients with AIDS

  9. Chylothorax in a patient with metastatic Kaposi sarcoma: Differential diagnostic considerations

    Directory of Open Access Journals (Sweden)

    Ryan Alexander, DO

    2015-01-01

    Full Text Available Kaposi sarcoma (KS is a low-grade mesenchymal tumor involving blood and lymphatic vessels. There are four types, based on clinical presentation: classic, endemic (Africana, iatrogenic (typically, involving renal allograft recipients, and AIDS-associated (epidemic. Kaposi's sarcoma-associated herpes virus infection has been linked along with other factors to the development of KS. The Kaposi's sarcoma-associated herpes virus interacts and encodes for numerous molecular proteins that play a role in the pathogenesis of KS, including latency-associated nuclear antigen, viral G protein-coupled receptor, viral FLICE inhibitory protein, and viral IL-6. KS primarily affects the skin and causes disseminated disease in a variety of organs. Involvement of visceral organs other than the lining of the alimentary tract is extremely rare. While the chylous pleural effusions of KS may resemble other pulmonary diseases (including lymphangioma, lymphangectasis, and lymphangioleiomyomatosis with chylous effusions at thoracic CT, differentiating features may allow for more prompt diagnosis and treatment. The presumptive diagnosis of AIDS-related pulmonary KS is often clinical. A tissue diagnosis is not required to establish the diagnosis of pulmonary KS. There are a variety of causes of chylothorax. The primary finding is a near-water-attenuating pleural effusion. The secondary findings of chylothorax can help differentiate the etiology.

  10. Primary synovial sarcoma of pericardium:A case report%心包原发性滑膜肉瘤1例报道

    Institute of Scientific and Technical Information of China (English)

    Rangreze Imran; Banday Manzoor Ahmad; Sheikh Aejaz Aziz; Bhat Salma; Mohammad Latif Charoo

    2011-01-01

    Primary pericardial sarcomas are extremely rare. We report a case of 19 year old male who presented with cough,dyspnoea, and orthopnea. Investigations and exploratory thoracotomy revealed a large pericardial mass. Surgical debulking of the tumor was performed and the histopathological examination was compatible with synovial sarcoma. The tumor was unresectable due to its invasion and adhesion to mediastinal structures. Hence patient was started on palliative chemotherapy (adriamycin and ifosfamide based). Patient showed an initial symptomatic response but later on there was a clinical progression and died within six months of his diagnosis.

  11. Preoperative, intraoperative, and postoperative radiation in the treatment of primary soft tissue sarcoma.

    Science.gov (United States)

    Suit, H D; Mankin, H J; Wood, W C; Proppe, K H

    1985-06-01

    The rationale for combining radiation with conservative surgery in the treatment of sarcoma of soft tissue is discussed, as well as the advantages for performing the radiation preoperatively on the one hand and postoperatively on the other. The results of treatment of soft tissue sarcoma by radical resectional surgery or amputation in 464 patients at four centers and by conservative surgery, and postoperative radiation in 416 patients at three centers, have been reviewed. The local failure rates were 18.1% and 18.3%, respectively. The results obtained by radiation administered postoperatively (110 patients) or preoperatively (60 patients) at the Massachusetts General Hospital during the period September 1971 to August 1982 are analyzed and discussed with reference to 5-year actuarial local control and survival results as well as causes of failure with respect to AJC stage, histologic type, anatomic site, and size of tumor. The results which have been obtained by the preoperative approach are judged to be superior, particularly for the larger lesions and higher grades that predominated in that group. Of a total of 170 patients, there were 19 local failures; 13 of these were diagnosed at the time when metastatic disease was not evident. Of those 13, 12 have been subjected to salvage surgery and 7 remain with no evidence of disease at 1 to 3.5 years after the salvage procedure. A major problem in the management of these patients remains the occult metastatic disease.

  12. Primary retroperitoneal mucinous cystadenoma with a sarcoma-like mural nodule: an immunohistochemical study with histogenetic considerations and literature review.

    Science.gov (United States)

    Demirel, Dilaver; Gun, Ismet; Kucukodaci, Zafer; Balta, Ahmet Ziya; Ramzy, Ibrahim

    2013-01-01

    Primary retroperitoneal mucinous cystadenomas (PRMCs) are extremely rare tumors and their association with sarcoma-like mural nodules (SLMNs) has not been described thoroughly. The aim of this study is to characterize the gross and microscopic features and the immunohistochemical profile of the first case of PRMC with SLMN and to discuss the differential diagnosis of SLMNs. The literature related to primary retroperitoneal mucinous tumors is reviewed in an attempt to clarify the histogenesis of the epithelial and sarcomatoid components of the associated mural nodules. A 34-yr-old woman presented with a 14-cm retroperitoneal cystic lesion with a 6-cm mural nodule. An immunohistochemical study with a panel of 19 antibodies and a histochemical study for mucin stains were performed. The epithelial component of the PRMC showed positive staining for cytokeratin (CK) 7, CK AE1/3, epithelial membrane antigen, carcinoembryonic antigen, and calretinin. The neoplasm was not immunoreactive for CK 20, CK 5/6, and the other antibodies used in this study. In addition, it stained positively for mucin by mucicarmine, periodic acid-Schiff, and Alcian blue. The stromal cells of the cyst showed estrogen receptor positivity. SLMN cells were negative for all CKs and other epithelial markers used in the study, but they showed diffuse positive staining for vimentin and CD68, and positive staining for Ki-67 was demonstrated in 25% of these cells. The immunohistochemical and histochemical profiles of PRMC were similar to those of ovarian mucinous neoplasms and the mesothelium. The formation of SLMNs seems to be related to subepithelial hemorrhage and some reactive epithelial changes near the mural nodules. The specific immunohistochemical and morphologic features of SLMNs are helpful in differentiating them from malignant mural nodules, including true sarcomas, osteoclast-rich undifferentiated carcinomas, and carcinosarcomas. Such a differentiation is critical in view of its significant

  13. Primary alveolar soft part sarcoma of vertebra: a case report and literature review

    Energy Technology Data Exchange (ETDEWEB)

    Zhu, Fei-Peng [Nanjing University, Department of Medical Imaging, Jinling Hospital, Clinical School of Medical College, Nanjing, Jiangsu Province (China); Xuzhou Medical College, Xuzhou, Jiangsu Province (China); Lu, Guang-Ming; Zhang, Long-Jiang [Nanjing University, Department of Medical Imaging, Jinling Hospital, Clinical School of Medical College, Nanjing, Jiangsu Province (China); Wang, Jian-Dong [Nanjing University, Department of Pathology, Jinling Hospital, Clinical School of Medical College, Nanjing, Jiangsu Province (China); An, Xiao-Jing [Nanjing University, Department of Pathology, Jinling Hospital, Clinical School of Medical College, Nanjing, Jiangsu Province (China); Nanjing University, Medical College, Nanjing, Jiangsu Province (China); Dong, Ying-Chun [Nanjing Stomatology Hospital/The Affiliated Stomatology Hospital of Medical School of Nanjing University, Department of Anesthesiology, Nanjing, Jiangsu Province (China)

    2009-08-15

    Alveolar soft part sarcoma (ASPS) is a rare malignant soft tissue tumor, which rarely occurs in bone. We present a case of ASPS in a 23-year-old man with a 2-month history of back pain. Computed tomography scanning and magnetic resonance images demonstrated a destructive process in the 12th thoracic vertebra associated with a unilateral soft tissue mass. The tumor showed evidence of hypervascularity on MRI; it obviously was enhanced on T1-weighted images after injection of Gd-GDPA, and signal voids were shown on all pulse sequences which may help to differentiate ASPS from other tumors of the vertebra. We believe that this is the first case of ASPS arising in a vertebra. (orig.)

  14. Limb salvage with microvascular free fibula following primary bone sarcoma resection

    Directory of Open Access Journals (Sweden)

    Sahasrabudhe Parag

    2016-01-01

    Full Text Available Background: Extremity sarcomas are challenging to manage. Total eradication of tumour has to be balanced with restoration of limb function to prevent mortality and morbidity. Disease-free survival with maximum limb function is the ultimate goal in these patients. Materials and Methods: We present a series of ten cases of extremity malignancies, where limb salvage was attempted with microvascular free fibula for limb reconstruction from the period of 2008 to 2015. Results: Of the ten cases in the study, there were two females and eight males. There were nine patients with lower limb malignancies and one patient with upper limb malignancy. There were four patients with Ewing's sarcoma of femur, five patients with osteosarcoma of femur and one patient with chondrosarcoma of the humerus. The follow-up period ranged from 1.2 to 6.2 years with mean follow-up of 3.1 years. There were two deaths during follow-up, both were due to distant metastasis. The assessment of the function was done on the basis of Musculoskeletal Tumour Society functional score. Maximum score was 30 and minimum score was 24, the average score being 26. Of the eight surviving patients, three patients had full weightbearing, four patients had partial weightbearing at end of 2 years and one patient of upper limb reconstruction had complete upper limb function. None of the patients had to undergo limb amputation. Conclusion: Limb salvage with vascularised fibula graft offers good functional outcome along with good disease-free survival rates.

  15. Ewing sarcoma

    Science.gov (United States)

    Bone cancer - Ewing sarcoma; Ewing family of tumors; Primitive neuroectodermal tumors (PNET); Bone neoplasm - Ewing sarcoma ... this tissue to help determine how aggressive the cancer is and what treatment may be best.

  16. Synovial Sarcoma in the Rectovesical Space: A Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Kil, Min Chul; Cho, Bum Sang; Han, Gi Seok; Park, Kil Sun; Kim, Sung Jin; Cha, Sang Hoon; Lee, Seung Young; Kang, MIn Ho [Dept. of Radiology, Chungbuk National University Hospital, Chunju (Korea, Republic of); Lee, Ok Jun [Dept. of Pathology, Chungbuk National University Hospital, Chunju (Korea, Republic of)

    2011-08-15

    Synovial sarcoma is an uncommon soft tissue malignancy usually arising in the extremities of young adults. Synovial sarcomas at unusual anatomic locations have been reported; however, to the best of our knowledge, there are no reports on primary synovial sarcoma in the rectovesical space. Here, we describe the radiologic findings of primary synovial sarcoma in the rectovesical space and review relevant literature.

  17. Primary intracranial soft tissue sarcomas in children, adolescents, and young adults: single institution experience and review of the literature.

    Science.gov (United States)

    Maher, Ossama M; Khatua, Soumen; Mukherjee, Devashis; Olar, Adriana; Lazar, Alexander; Luthra, Raja; Liu, Diane; Wu, Jimin; Ketonen, Leena; Zaky, Wafik

    2016-03-01

    There is a paucity of literature reporting the outcome of intracranial sarcomas (IS) in children, adolescents, and young adults (CAYA). A multimodal therapeutic approach is commonly used, with no well-established treatment consensus. We conducted a retrospective review of CAYA with IS, treated at our institution, to determine their clinical findings, treatments, and outcomes. Immunohistochemistry (PDGFRA and EGFR) and DNA sequencing were performed on 5 tumor samples. A literature review of IS was also conducted. We reviewed 13 patients (median age, 7 years) with a primary diagnosis of IS between 1990 and 2015. Diagnoses included unclassified sarcoma (n = 9), chondrosarcoma (n = 2), and rhabdomyosarcoma (n = 2). Five patients underwent upfront gross total resection (GTR) of the tumor. The 5-drug regimen (vincristine, doxorubicin, cyclophosphamide, etoposide, and ifosfamide) was the most common treatment used. Nine patients died due to progression or recurrence (n = 8) or secondary malignancy (n = 1). The median follow-up period of the 4 surviving patients was 1.69 years (range 1.44-5.17 years). The 5-year progression-free survival and overall survival rates were 21 and 44 %, respectively. BRAF, TP53, KRAS, KIT, ERBB2, MET, RET, ATM, and EGFR mutations were detected in 4 of the 5 tissue samples. All 5 samples were immunopositive for PDGFRA, and only 2 were positive for EGFR. IS remain a therapeutic challenge due to high progression and recurrence rates. Collaborative multi-institutional studies are warranted to delineate a treatment consensus and investigate tumor biology to improve the disease outcome.

  18. Primary Ewing’s Sarcoma of the Sinonasal Tract: A Case Report

    Directory of Open Access Journals (Sweden)

    Tomoharu Suzuki

    2017-01-01

    Full Text Available A 23-year-old male presented with a 3-month history of left purulent rhinorrhea, progressive nasal obstruction, and intermittent epistaxis. A fiberoptic examination revealed a large vascular polypoid mass completely filling the left nasal cavity. CT and MRI scans showed a large hypervascular mass involving the left nasal airway, maxillary antrum, and the anterior ethmoid cells. There was no bony erosion or contiguous spread, and the remaining sinuses, orbit, and cranial fossa were uninvolved. The patient underwent complete removal of the mass via an external lateral rhinotomy approach. The soft mass was large and vascular. A microscopic analysis revealed an undifferentiated tumor consisting of a solid sheet of small, round blue cells. Mitotic figures were also present. Immunohistochemically, the tumor cells were strongly positive for CD99. Molecular studies using a PCR confirmed the chromosomal translocation of FLI1 (exon 6. These findings were considered diagnostic for Ewing’s sarcoma. Postoperatively, the patient was treated with combined chemotherapy and radiotherapy. Adjuvant chemotherapy consisting of vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide (total: 7 cycles was commenced. He also received radiation therapy for local control (total dose: 50.4 Gy. The patient is currently alive without any evidence of recurrence or metastasis.

  19. Primary histiocytic sarcoma arising in the head and neck with predominant spindle cell component

    Directory of Open Access Journals (Sweden)

    Zhao XF

    2007-02-01

    Full Text Available Abstract This is the first case report of Histiocytic Sarcoma (HS with predominant spindle cell component occurring in the head and neck region of a 41-year-old man. The tumor was composed of sheets of large round to oval cells with pleomorphic vesicular nuclei, prominent nucleoli and abundant eosinophilic cytoplasm. Multinucleated forms, numerous mitoses, and tumor necrosis were also noted. Sheets, fascicles, and whorls of spindle cells with spindled to ovoid vesicular nuclei, small to medium-sized distinct nucleoli, and eosinophilic cytoplasm were frequently observed. Immunohistochemical staining in the tumor cells was positive for CD163, CD68, lysozyme, CD45, and NSE. Focal expression of CD4 and S-100 was also noted. Electron microscopy demonstrated an abundance of lysosomes in the cytoplasm of tumor cells. Chromosome study revealed a 57–80 hyperdiploid [7]/46, XY [13] karyotype, including 3 to 4 copies of various chromosomes. The immunohistochemical and ultrastructural findings confirmed the diagnosis of HS.

  20. DNA Methylation and Gene Expression Profiling of Ewing Sarcoma Primary Tumors Reveal Genes That Are Potential Targets of Epigenetic Inactivation

    Directory of Open Access Journals (Sweden)

    Nikul Patel

    2012-01-01

    Full Text Available The role of aberrant DNA methylation in Ewing sarcoma is not completely understood. The methylation status of 503 genes in 52 formalin-fixed paraffin-embedded EWS tumors and 3 EWS cell lines was compared to human mesenchymal stem cell primary cultures (hMSCs using bead chip methylation analysis. Relative expression of methylated genes was assessed in 5-Aza-2-deoxycytidine-(5-AZA-treated EWS cell lines and in a cohort of primary EWS samples and hMSCs by gene expression and quantitative RT-PCR. 129 genes demonstrated statistically significant hypermethylation in EWS tumors compared to hMSCs. Thirty-six genes were profoundly methylated in EWS and unmethylated in hMSCs. 5-AZA treatment of EWS cell lines resulted in upregulation of expression of hundreds of genes including 162 that were increased by at least 2-fold. The expression of 19 of 36 candidate hypermethylated genes was increased following 5-AZA. Analysis of gene expression from an independent cohort of tumors confirmed decreased expression of six of nineteen hypermethylated genes (AXL, COL1A1, CYP1B1, LYN, SERPINE1, and VCAN. Comparing gene expression and DNA methylation analyses proved to be an effective way to identify genes epigenetically regulated in EWS. Further investigation is ongoing to elucidate the role of these epigenetic alterations in EWS pathogenesis.

  1. A Unique Case of Primary Ewing’s Sarcoma of the Cervical Spine in a 53-Year-Old Male: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Marshall T. Holland

    2015-01-01

    Full Text Available Extraskeletal Ewing’s sarcoma (EES is a rare presentation, representing only 15% of all primary Ewing’s sarcoma cases. Even more uncommon is EES presenting as a primary focus in the spinal canal. These rapidly growing tumors often present with focal neurological symptoms of myelopathy or radiculopathy. There are no classic characteristic imaging findings and thus the physician must keep a high index of clinical suspicion. Diagnosis can only be definitively made by histopathological studies. In this report, we discuss a primary cervical spine EES in a 53-year-old man who presented with a two-month history of left upper extremity pain and acute onset of weakness. Imaging revealed a cervical spinal canal mass. After undergoing cervical decompression, histopathological examination confirmed a diagnosis of Ewing’s sarcoma. A literature search revealed fewer than 25 reported cases of primary cervical spine EES published in the past 15 years and only one report demonstrating this pathology in a patient older than 30 years of age age=38. Given the low incidence of this pathology presenting in this age group and the lack of treatment guidelines, each patient’s plan should be considered on a case-by-case basis until further studies are performed to determine optimal evidence based treatment.

  2. Front-line window therapy with cisplatin in patients with primary disseminated Ewing sarcoma: A study by the Associazione Italiana di Ematologia ed Oncologia Pediatrica and Italian Sarcoma Group.

    Science.gov (United States)

    Luksch, Roberto; Grignani, Giovanni; D'Angelo, Paolo; Prete, Arcangelo; Puma, Nadia; Podda, Marta; Casanova, Michela; Ferrari, Andrea; Morosi, Carlo; Fagioli, Franca; Aglietta, Massimo; Ferrari, Stefano; Picci, Piero; Massimino, Maura

    2017-06-14

    The aim was to assess the activity of cisplatin (CDDP) in Ewing sarcoma (ES). The study consisted of front-line window therapy with CDDP 120 mg/sqm every 3 weeks for two courses in children and young adults with primary disseminated ES. Response was assessed using the Response Evaluation Criteria in Solid Tumours criteria, and Simon's two-stage design was applied. Twelve consecutive patients were enrolled in stage 1. Only one objective response was observed. Since the target response rate was not achieved, accrual was stopped and CDDP as a single agent in ES was judged unworthy of further assessment. © 2017 Wiley Periodicals, Inc.

  3. Reactivation of Latent Infection of Human Herpesvirus 8 in BC-3 Cells from Primary Effusion Lymphoma by Recombinant CytokinesSimilar to that Produced by HIV-1-infected T Cells

    Institute of Scientific and Technical Information of China (English)

    卢春; 曾怡; 黄丽

    2003-01-01

    Objective:To study and confirm that recombinant cytokines similar to those produced by HIV-1 infected T cells induced lytic cycle replication of human pervirus 8(HHV-8) in BC-3 cells,another cell line from primary effusion lymphoma(PEL).Methods:The persistent stimulation of BC-3 was conducted by several cytokines known to be produced by HIV-1-infected T cells and important in grouth and proliferation of Kaposi's sarcoma(KS) cells in vitro,such as the inter feron-γ(IFN-γ),the hepatocyte grouth factor /scatter factor (HGF/SF),the Oncostain M(OSM),and the tumor necrosis factor-α(TNF-α)which is not produced by HIV-1-infeted T cells.Treated and antrented BC-3 cells were collected at the 3rd and 7th day of persistent stimulation,respeclively.Immunohistochemical(IHC) staining. Northern blot,quantitative PCR(real-time PCR) and electron microscopy(EM) were carried out to detect the expression of immumogenic protein ORF59,messenger RNA(mRNA) of minor capsid protein ORF26,and the presence of viral particles of HHV-8 from treated and untreated BC-3 cells.Results:It showed that IFN-γ,HGF/SF/OSM,and TNF-α were found to induce an increase in mRNA expression of ORF26 when added individually to BC-3 cells.Particularly,ORF26 expression stimulated with IFN-γ and TNF-α respectively,increased 6.1 and 2.5-fold(from,real-time PCR results) at the 7th day when compared with untreated BC-3 cells.Meanwhile ,about 20% of IFN-γ stimulated BC-3 cells expressed ORF59 at the 7th day as compared with 1.5% of untreated BC-3 cells when IHC staining was employed.In addition,viral particles of HHV-8 were readily idelified in BC-3 cells stimulated with IFN-γ at the 7th day with EM analysis.Conclusion:TNF-α and recombinant cytokines being similar to those produced by HIV-1 infected T Cells could really induce HHV-8 lytic cycle replication in BC-3 cells,another cell line of PEL.

  4. Dual-Energy Micro-Computed Tomography Imaging of Radiation-Induced Vascular Changes in Primary Mouse Sarcomas

    Energy Technology Data Exchange (ETDEWEB)

    Moding, Everett J. [Department of Pharmacology and Cancer Biology, Duke University Medical Center, Durham, North Carolina (United States); Clark, Darin P.; Qi, Yi [Center for In Vivo Microscopy, Department of Radiology, Duke University Medical Center, Durham, North Carolina (United States); Li, Yifan; Ma, Yan [Department of Radiation Oncology, Duke University Medical Center, Durham, North Carolina (United States); Ghaghada, Ketan [The Edward B. Singleton Department of Pediatric Radiology, Texas Children' s Hospital, Houston, Texas (United States); Johnson, G. Allan [Center for In Vivo Microscopy, Department of Radiology, Duke University Medical Center, Durham, North Carolina (United States); Kirsch, David G. [Department of Pharmacology and Cancer Biology, Duke University Medical Center, Durham, North Carolina (United States); Department of Radiation Oncology, Duke University Medical Center, Durham, North Carolina (United States); Badea, Cristian T., E-mail: cristian.badea@duke.edu [Center for In Vivo Microscopy, Department of Radiology, Duke University Medical Center, Durham, North Carolina (United States)

    2013-04-01

    Purpose: To evaluate the effects of radiation therapy on primary tumor vasculature using dual-energy (DE) micro-computed tomography (micro-CT). Methods and Materials: Primary sarcomas were generated with mutant Kras and p53. Unirradiated tumors were compared with tumors irradiated with 20 Gy. A liposomal-iodinated contrast agent was administered 1 day after treatment, and mice were imaged immediately after injection (day 1) and 3 days later (day 4) with DE micro-CT. CT-derived tumor sizes were used to assess tumor growth. After DE decomposition, iodine maps were used to assess tumor fractional blood volume (FBV) at day 1 and tumor vascular permeability at day 4. For comparison, tumor vascularity and vascular permeability were also evaluated histologically by use of CD31 immunofluorescence and fluorescently-labeled dextrans. Results: Radiation treatment significantly decreased tumor growth from day 1 to day 4 (P<.05). There was a positive correlation between CT measurement of tumor FBV on day 1 and extravasated iodine on day 4 with microvascular density (MVD) on day 4 (R{sup 2}=0.53) and dextran accumulation (R{sup 2}=0.63) on day 4, respectively. Despite no change in MVD measured by histology, tumor FBV significantly increased after irradiation as measured by DE micro-CT (0.070 vs 0.091, P<.05). Both dextran and liposomal-iodine accumulation in tumors increased significantly after irradiation, with dextran fractional area increasing 5.2-fold and liposomal-iodine concentration increasing 4.0-fold. Conclusions: DE micro-CT is an effective tool for noninvasive assessment of vascular changes in primary tumors. Tumor blood volume and vascular permeability increased after a single therapeutic dose of radiation treatment.

  5. Upregulation of NKX2.2, a target of EWSR1/FLI1 fusion transcript, in primary renal Ewing sarcoma

    Directory of Open Access Journals (Sweden)

    Yoshinari Yamamoto

    2015-01-01

    Full Text Available Renal Ewing sarcoma (ES is a rare malignant tumor characterized by fusion of the EWSR1 gene with a member of the ETS family of oncogenes, arising at a specific chromosomal translocation. Diagnosis of ES can be problematic, especially from cytological or small bioptical specimens because the differential diagnoses comprising a diverse group of small round blue cell tumors (SRBCTs. We report a case of primary renal ES in a young male, which had a t(11;22 (q24;q12 chromosome translocation encoding a type2 EWSR1/FLI1 fusion transcript. The tumor cells showed diffuse cytoplasmic immunoreactivity for CD99 and diffuse nuclear immunoreactivity for NKX2.2, an important oncogenic transcriptional target of EWSR1/FLI1, not only in the histological, but also in the cytological specimens. From the results of this case, we speculate that NKX2.2, in combination with CD99, may be a useful immunocytochemical marker to distinguish renal ES from other SRBCTs of kidney.

  6. Tuberculous Pleural Effusion

    Directory of Open Access Journals (Sweden)

    Shira A. Schlesinger

    2012-09-01

    Full Text Available Pleural effusions are a common finding in emergency departments, with cytologic analysis traditionally required for definitive diagnosis. This article describes a classic sonographic appearance of tuberculous pleural effusion.

  7. Primary high grade sarcoma of the specialised prostatic stroma: a case report with clinico-pathological considerations.

    Science.gov (United States)

    Fraggetta, F; Pepe, P; Giunta, M L; Aragona, F

    2008-12-01

    Malignant tumours of the prostate other than carcinomas are rare. One such malignant tumours arising from the specialised stromal tissue of the prostate is stromal prostatic sarcoma (namely low-grade and high-grade). Herein, we report the clinico-pathological features of a high grade stromal sarcoma of the prostate occurring in a 65-year-old man who presented for urinary obstructive symptoms. The clinical picture suggested a benign prostatic hyperplasia, and surgery consisting in a transcapsular adenomectomy was performed. Following a pathological diagnosis of high grade prostatic stromal sarcoma, a radical cystoprostatectomy and bilateral pelvic node dissection was performed showing residual high grade stromal sarcoma of the prostate and incidental in situ urothelial carcinoma of the bladder. No further medical treatments were planned. One year after surgery the patient is well with no evidence of local disease or distant metastases.

  8. Kinetics of Kaposi’s Sarcoma-Associated Herpesvirus Gene Expression

    OpenAIRE

    Sun, Ren; Lin, Su-Fang; Staskus, Katherine; Gradoville, Lyndle; Grogan, Elizabeth; Haase, Ashley; Miller, George

    1999-01-01

    Herpesvirus gene expression can be classified into four distinct kinetic stages: latent, immediate early, early, and late. Here we characterize the kinetic class of a group of 16 Kaposi’s sarcoma-associated herpesvirus (KSHV)/human herpesvirus 8 genes in a cultured primary effusion cell line and examine the expression of a subset of these genes in KS biopsies. Expression of two latent genes, LANA and vFLIP, was constitutive and was not induced by chemicals that induce the lytic cycle in prima...

  9. Distinct p53, p53:LANA, and LANA Complexes in Kaposi's Sarcoma-Associated Herpesvirus Lymphomas▿

    OpenAIRE

    Chen, Wuguo; Hilton, Isaac B.; Staudt, Michelle R; Burd, Christin E.; Dittmer, Dirk P

    2010-01-01

    The role of p53 in primary effusion lymphoma (PEL) is complicated. The latency-associated nuclear antigen (LANA) of Kaposi's sarcoma-associated herpesvirus (KSHV) binds p53. Despite this interaction, we had found that p53 was functional in PEL, i.e., able to induce apoptosis in response to DNA damage (C. E. Petre, S. H. Sin, and D. P. Dittmer, J. Virol. 81:1912-1922, 2007), and that hdm2 was overexpressed. To further elucidate the relationship between LANA, p53, and hdm2, we purified LANA com...

  10. Persistent benign pleural effusion.

    Science.gov (United States)

    Porcel, J M

    In this narrative review we describe the main aetiologies, clinical characteristics and treatment for patients with benign pleural effusion that characteristically persists over time: chylothorax and cholesterol effusions, nonexpansible lung, rheumatoid pleural effusion, tuberculous empyema, benign asbestos pleural effusion and yellow nail syndrome. Copyright © 2017 Elsevier España, S.L.U. and Sociedad Española de Medicina Interna (SEMI). All rights reserved.

  11. Imaging Features of Primary Tumors and Metastatic Patterns of the Extraskeletal Ewing Sarcoma Family of Tumors in Adults: A 17-Year Experience at a Single Institution

    Science.gov (United States)

    Huh, Jimi; Park, Seong Joon; Kim, Hyoung Jung; Lee, Jong Seok; Ha, Hyun Kwon; Tirumani, Sree Harsha; Ramaiya, Nikhil H.

    2015-01-01

    Objective To comprehensively analyze the spectrum of imaging features of the primary tumors and metastatic patterns of the Extraskeletal Ewing sarcoma family of tumors (EES) in adults. Materials and Methods We performed a computerized search of our hospital's data-warehouse from 1996 to 2013 using codes for Ewing sarcoma and primitive neuroectodermal tumors as well as the demographic code for ≥ 18 years of age. We selected subjects who were histologically confirmed to have Ewing sarcoma of extraskeletal origin. Imaging features of the primary tumor and metastatic disease were evaluated for lesion location, size, enhancement pattern, necrosis, margin, and invasion of adjacent organs. Results Among the 70 patients (mean age, 35.8 ± 15.6 years; range, 18-67 years) included in our study, primary tumors of EES occurred in the soft tissue and extremities (n = 20), abdomen and pelvis (n = 18), thorax (n = 14), paravertebral space (n = 8), head and neck (n = 6), and an unknown primary site (n = 4). Most primary tumors manifested as large and bulky soft-tissue masses (mean size, 9.0 cm; range, 1.3-23.0 cm), frequently invading adjacent organs (45.6%) and showed heterogeneous enhancement (73.7%), a well-defined (66.7%) margin, and partial necrosis/cystic degeneration (81.9%). Notably, 29 patients had metastatic disease detected at their initial diagnosis. The most frequent site of metastasis was lymph nodes (75.9%), followed by bone (31.0%), lung (20.7%), abdominal solid organs (13.8%), peritoneum (13.8%), pleura (6.9%), and brain (3.4%). Conclusion Primary tumors of EES can occur anywhere and mostly manifest as large and bulky, soft-tissue masses. Lymph nodes are the most frequent metastasis sites. PMID:26175577

  12. Retroperitoneal Sarcomas.

    Science.gov (United States)

    Porpiglia, Andrea S; Reddy, Sanjay S; Farma, Jeffrey M

    2016-10-01

    Retroperitoneal sarcomas are rare tumors, representing only 15% of all sarcomas. The mainstay of therapy is surgical resection with negative margins. However, this is challenging because of the late presentation of many of these tumors and involvement with adjacent structures. Decisions on radiation therapy and chemotherapy should be made in a multidisciplinary setting at a tertiary referral center.

  13. Synovial sarcoma

    Directory of Open Access Journals (Sweden)

    Sucari S.C. Vlok

    2014-12-01

    Full Text Available Synovial sarcoma is a malignant, predominantly juxta-articular, soft-tissue tumour representing approximately 10% of all soft-tissue sarcomas. Frequently initially incorrectly diagnosed as a benign lesion, it should be considered as a diagnosis when a young adult patient presents with a calcified juxta-articular soft-tissue mass of insidious onset.

  14. Molecular and immunohistochemical detection of Kaposi sarcoma herpesvirus/human herpesvirus-8.

    Science.gov (United States)

    Chadburn, Amy; Wilson, Janet; Wang, Y Lynn

    2013-01-01

    Kaposi sarcoma herpesvirus/human herpesvirus-8 (KSHV/HHV-8) is etiologically related to the development of several human diseases, including Kaposi sarcoma, primary effusion lymphoma (PEL)/extra-cavitary (EC) PEL, multicentric Castleman disease (MCD), and large B-cell lymphoma arising in KSHV/HHV-8-associated multicentric Castleman disease. Although serologic studies can identify persons infected with this virus, molecular genetics, specifically PCR (polymerase chain reaction) and immunohistochemical techniques, are rapid, sensitive, and specific, and are able to more closely link KSHV/HHV-8 to a given disease process. As these KSHV/HHV-8-related diseases cause significant morbidity and mortality in affected individuals, the identification of the virus within lesional tissue will allow for more targeted therapy.

  15. The Biology of Kaposi's Sarcoma-Associated Herpesvirus and the Infection of Human Immunodeficiency Virus

    Institute of Scientific and Technical Information of China (English)

    Di QIN; Chun LU

    2008-01-01

    Kaposi sarcoma-associated herpesvirus (KSHV),also known as human herpesvirus 8 (HHV-8),is discovered in 1994 from Kaposi's sarcoma (KS) lesion of an acquired immunodeficiency syndrome (AIDS)patient.In addition to its association with KS,KSHV has also been implicated as the causative agent of two other AIDS-associated malignancies:primary effusion lymphoma (PEL) and multicentric Castleman's disease (MCD).KSHV is a complex DNA virus that not only has the ability to promote cellular growth and survival for tumor development,but also can provoke deregulated angiogenesis,inflammation,and modulate the patient's immune system in favor of tumor growth.As KSHV is a necessary but not sufficient etiological factor for KS,human immunodeficiency virus (HIV) is a very important cofactor.Here we review the basic information about the biology of KSHV,development of pathogenesis and interaction between KSHV and HIV.

  16. Kaposi's Sarcoma-Associated Herpesvirus-Related Solid Lymphoma Involving the Heart and Brain

    Directory of Open Access Journals (Sweden)

    Jason R. Andrews

    2011-01-01

    Full Text Available Since its discovery in 1994, Kaposi's sarcoma-associated herpesvirus (KSHV has been associated with lymphoproliferative disorders, particularly in patients infected with human immunodeficiency virus (HIV. The disorders most strongly linked to KSHV are multicentric Castleman's Disease (MCD, primary effusion lymphoma, and diffuse large B-cell lymphomas. We report an unusual case of KSHV-associated lymphoma in an HIV-infected patient manifesting with myocardial and central nervous system involvement. We discuss this case in the context of increasing array of KSHV-associated lymphomas. In the HIV-infected patient with a mass lesion, a history of cutaneous Kaposi's sarcoma and prolonged immunosuppression should alert clinicians as to the possibility of KSHV-associated lymphoproliferative disorders, in order to establish a timely diagnosis.

  17. Effusion under the microscope.

    LENUS (Irish Health Repository)

    Chong, S G

    2012-03-01

    Pseudochylothorax is a very rare form of pleural effusion. It is also called chyliform or cholesterol pleural effusion. It is usually a unilateral process and approximately one-third of patients are asymptomatic at presentation. We report a case of a 60 year old man with a background of rheumatoid arthritis who presented with progressive dyspnea. Chest X-ray revealed a new left pleural effusion and a small persistent right pleural effusion. He presented 5 years prior due to recurrent pleural effusion and no diagnosis was made. Repeat thoracentesis yielded 350 milliliters of thick, milky, tan-colored fluid.

  18. Ewing Sarcoma of the Bone With EWS/FLI1 Translocation After Successful Treatment of Primary Osteosarcoma.

    Science.gov (United States)

    Yodoya, Noriko; Iwamoto, Shotaro; Matsumine, Akihiko; Azuma, Eiichi; Toyoda, Hidemi; Miura, Yoshihiro; Nakatani, Kaname; Imai, Hiroshi; Hirayama, Masahiro; Komada, Yoshihiro

    2017-01-01

    Although prognosis in patients with localized osteosarcoma has been dramatically improved by the introduction of multiple chemotherapy agents known as combination chemotherapy, there is growing concern about the development of secondary malignant neoplasms. We report the case of a 13-year-old girl in whom the diagnosis of Ewing sarcoma of bone localized on the shaft of left femur was made 2 years after successful treatment without radiotherapy for osteosarcoma of right proximal femur. EWS-FLI1 fusion gene was detected by reverse transcriptase-polymerase chain reaction. To our knowledge, this is the first case with Ewing sarcoma of the bone as a secondary malignant neoplasm developed in osteosarcoma survivor. We collected 15 cases, included this case, with secondary Ewing sarcoma family of tumor by utilizing the PubMed search and might consider the causes of this secondary cancer.

  19. Recurrent Uveal Effusion after Laser Iridotomy

    Science.gov (United States)

    Sakai, Hiroshi; Yonahara, Michiko; Sakai, Miyako

    2017-01-01

    A 59-year-old woman was seen by an ophthalmologist for blurred vision, ocular pain, headache, and nausea. She was diagnosed with acute primary angle closure (APAC) and successfully treated with medications. Using ultrasound biomicroscopy (UBM), engorged episcleral vein was observed and small uveal effusion was diagnosed after laser peripheral iridotomy (LPI). The uveal effusion disappeared and was again diagnosed by UBM together with anterior segment inflammation with ocular pain. Iritis caused by LPI after APAC might be a cause of uveal effusion in this specific case. PMID:28203193

  20. Recurrent Uveal Effusion after Laser Iridotomy

    Directory of Open Access Journals (Sweden)

    Hiroshi Sakai

    2017-01-01

    Full Text Available A 59-year-old woman was seen by an ophthalmologist for blurred vision, ocular pain, headache, and nausea. She was diagnosed with acute primary angle closure (APAC and successfully treated with medications. Using ultrasound biomicroscopy (UBM, engorged episcleral vein was observed and small uveal effusion was diagnosed after laser peripheral iridotomy (LPI. The uveal effusion disappeared and was again diagnosed by UBM together with anterior segment inflammation with ocular pain. Iritis caused by LPI after APAC might be a cause of uveal effusion in this specific case.

  1. What Is Kaposi Sarcoma?

    Science.gov (United States)

    ... Treatment? Kaposi Sarcoma About Kaposi Sarcoma What Is Kaposi Sarcoma? Cancer starts when cells in the body begin ... the lungs may cause trouble breathing. Types of Kaposi sarcoma The different types of KS are defined by ...

  2. Primary cutaneous Ewing's sarcoma/primitive neuroectodermal tumor manifesting numerous small and huge ulcerated masses: its complete remission by chemotherapy and magnetic resonance imaging findings

    Energy Technology Data Exchange (ETDEWEB)

    Bahk, Won-Jong [The Catholic University of Korea, Department of Orthopedic Surgery, Uijeongbu St. Mary' s Hospital, Uijeongbu (Korea); Chang, Eun-Deok; Lee, An-Hee [The Catholic University of Korea, Department of Pathology, Uijeongbu St. Mary' s Hospital, Uijeongbu (Korea); Bae, Jung Min; Kim, Jin Woo [The Catholic University of Korea, Department of Dermatology, Uijeongbu St. Mary' s Hospital, Uijeongbu (Korea); Chun, Kyung-Ah [The Catholic University of Korea, Department of Radiology, Uijeongbu St. Mary' s Hospital, Uijeongbu (Korea); Rho, Sang-Young [The Catholic University of Korea, Department of Hemato-oncology, Uijeongbu St. Mary' s Hospital, Uijeongbu (Korea)

    2010-06-15

    Extraskeletal Ewing's sarcoma (ES) and primitive neuroectodermal tumor (PNET) are widely regarded as clinically and histologically identical tumors which consist of small blue round cells. Extraskeletal ESs/PNETs usually occur in the deep soft tissues of the paraspinal region, chest wall, or lower extremities. However, superficially located cases, so-called cutaneous ESs/PNETs, are exceedingly rare, and the vast majority of the reported cases present as a single small mass. We present magnetic resonance imaging (MRI) findings and clinical course of a unique case of primary cutaneous ES/PNET presenting as numerous huge masses with severe ulceration on them. (orig.)

  3. Primary spindle cell sarcoma of the prostate and 18 F-fluorodeoxyglucose-positron-emission tomography/computed tomography findings

    Directory of Open Access Journals (Sweden)

    Hakan Öztürk

    2015-01-01

    Conclusion: Spindle sarcomas of the prostate have quite aggressive nature and they have high potential to metastase. Average life expectancy is <1 year and the prognosis is poor. CTx and radiation therapy can′t yield curative effects due to poor differentiation.

  4. Apigenin induces apoptosis via downregulation of S-phase kinase-associated protein 2-mediated induction of p27Kip1 in primary effusion lymphoma cells.

    Science.gov (United States)

    Hussain, A R; Khan, A S; Ahmed, S O; Ahmed, M; Platanias, L C; Al-Kuraya, K S; Uddin, S

    2010-04-01

    The mechanisms that regulate mitogenic and antiapoptotic signals in primary effusion lymphoma (PEL) are not well known. In efforts to identify novel approaches to block the proliferation of PEL cells, we assessed the effect of apigenin (4',5,7-trihydroxyflavone), a flavonoid on a panel of PEL cell lines. We studied the effect of apigenin on four PEL cell lines. Apoptosis was measured by annexin V/PI dual staining and DNA laddering. Protein expression was measured by immunoblotting. Apigenin induced apoptosis in PEL cell lines in a dose dependent manner. Such effects of apigenin appeared to result from suppression of constitutively active kinase AKT resulting in down-regulation of SKP2, hypo-phosphorylation of Rb and accumulation of p27Kip1. Apigenin treatment of PEL cells caused dephosphorylation of p-Bad protein leading to down regulation of the anti-apoptotic protein, Bcl-2 and an increase in Bax/Bcl2 ratio. Apigenin treatment also triggered Bax conformational change and subsequently translocation from cytosole to mitochondria causing loss of mitochondrial membrane potential with subsequent release of cytochrome c. Released cytochrome c onto the cytosole activated caspase-9 and caspase-3, followed by polyadenosin-5'-diphosphate-ribose polymerase (PARP) cleavage. Finally, treatment of PEL cells with apigenin down-regulated the expression of inhibitor of apoptosis protein (IAPs). Altogether, these data suggest a novel function for apigenin, acting as a suppressor of AKT/PKB pathway in PEL cells, and raise the possibility that this agent may have a future therapeutic role in PEL and possibly other malignancies with constitutive activation of the AKT/PKB pathway.

  5. Triptolide inhibits transcription of hTERT through down-regulation of transcription factor specificity protein 1 in primary effusion lymphoma cells

    Energy Technology Data Exchange (ETDEWEB)

    Long, Cong; Wang, Jingchao [Department of Pathogen Biology, School of Basic Medical Sciences, Wuhan University, Wuhan, 430071 (China); Guo, Wei [Department of Pathology and Physiology, School of Basic Medical Sciences, Wuhan University, Wuhan, 430071 (China); Wang, Huan; Wang, Chao; Liu, Yu [Department of Pathogen Biology, School of Basic Medical Sciences, Wuhan University, Wuhan, 430071 (China); Sun, Xiaoping, E-mail: xsun6@whu.edu.cn [Department of Pathogen Biology, School of Basic Medical Sciences, Wuhan University, Wuhan, 430071 (China); State Key Laboratory of Virology, Wuhan University, Wuhan, 430072 (China)

    2016-01-01

    Primary effusion lymphoma (PEL) is a rare and aggressive non-Hodgkin's lymphoma. Human telomerase reverse transcriptase (hTERT), a key component responsible for the regulation of telomerase activity, plays important roles in cellular immortalization and cancer development. Triptolide purified from Tripterygium extracts displays a broad-spectrum bioactivity profile, including immunosuppressive, anti-inflammatory, and anti-tumor. In this study, it is investigated whether triptolide reduces hTERT expression and suppresses its activity in PEL cells. The mRNA and protein levels of hTERT were examined by real time-PCR and Western blotting, respectively. The activity of hTERT promoter was determined by Dual luciferase reporter assay. Our results demonstrated that triptolide decreased expression of hTERT at both mRNA and protein levels. Further gene sequence analysis indicated that the activity of hTERT promoter was suppressed by triptolide. Triptolide also reduced the half-time of hTERT. Additionally, triptolide inhibited the expression of transcription factor specificity protein 1(Sp1) in PEL cells. Furthermore, knock-down of Sp1 by using specific shRNAs resulted in down-regulation of hTERT transcription and protein expression levels. Inhibition of Sp1 by specific shRNAs enhanced triptolide-induced cell growth inhibition and apoptosis. Collectively, our results demonstrate that the inhibitory effect of triptolide on hTERT transcription is possibly mediated by inhibition of transcription factor Sp1 in PEL cells. - Highlights: • Triptolide reduces expression of hTERT by decreasing its transcription level. • Triptolide reduces promoter activity and stability of hTERT. • Triptolide down-regulates expression of Sp1. • Special Sp1 shRNAs inhibit transcription and protein expression of hTERT. • Triptolide and Sp1 shRNA2 induce cell proliferation inhibition and apoptosis.

  6. Screening of the Human Kinome Identifies MSK1/2-CREB1 as an Essential Pathway Mediating Kaposi's Sarcoma-Associated Herpesvirus Lytic Replication during Primary Infection

    Science.gov (United States)

    Cheng, Fan; Sawant, Tanvee Vinod; Lan, Ke; Lu, Chun; Jung, Jae U.

    2015-01-01

    ABSTRACT Viruses often hijack cellular pathways to facilitate infection and replication. Kaposi's sarcoma-associated herpesvirus (KSHV) is an oncogenic gammaherpesvirus etiologically associated with Kaposi's sarcoma, a vascular tumor of endothelial cells. Despite intensive studies, cellular pathways mediating KSHV infection and replication are still not well defined. Using an antibody array approach, we examined cellular proteins phosphorylated during primary KSHV infection of primary human umbilical vein endothelial cells. Enrichment analysis identified integrin/mitogen-activated protein kinase (integrin/MAPK), insulin/epidermal growth factor receptor (insulin/EGFR), and JAK/STAT as the activated networks during primary KSHV infection. The transcriptional factor CREB1 (cyclic AMP [cAMP]-responsive element-binding protein 1) had the strongest increase in phosphorylation. While knockdown of CREB1 had no effect on KSHV entry and trafficking, it drastically reduced the expression of lytic transcripts and proteins and the production of infectious virions. Chemical activation of CREB1 significantly enhanced viral lytic replication. In contrast, CREB1 neither influenced the expression of the latent gene LANA nor affected KSHV infectivity. Mechanistically, CREB1 was not activated through the classic cAMP/protein kinase A (cAMP/PKA) pathway or via the AKT, MK2, and RSK pathways. Rather, CREB1 was activated by the mitogen- and stress-activated protein kinases 1 and 2 (MSK1/2). Consequently, chemical inhibition or knockdown of MSKs significantly inhibited the KSHV lytic replication program; however, it had a minimal effect on LANA expression and KSHV infectivity. Together, these results identify the MSK1/2-CREB1 proteins as novel essential effectors of KSHV lytic replication during primary infection. The differential effect of the MSK1/2-CREB1 pathway on the expression of viral latent and lytic genes might control the robustness of viral lytic replication, and therefore the

  7. Alveolar Soft Part Sarcoma of Urinary Bladder Occurring as a Second Primary Malignancy: A Case Report and Literature Review

    Directory of Open Access Journals (Sweden)

    Manint Usawachintachit

    2016-01-01

    Full Text Available We report a case of bladder alveolar soft part sarcoma in an 18-year-old Thai male patient who had been treated with testicular radiation and systemic chemotherapy for acute lymphoblastic leukemia with testicular relapse. He presented with recurrent dysuria and gross hematuria. Cystoscopy revealed a 2-centimeter irregular sessile mass at the bladder base adjacent to left ureteral orifice. Transurethral resection of the tumor was performed. The histopathological diagnosis was alveolar soft part sarcoma. Chest and abdominal computed tomography showed no evidence of metastasis. He was treated with partial cystectomy and left ureteral reimplantation with negative surgical margin. No evidence of recurrence was found during a 28-month follow-up period with surveillance cystoscopy and computed tomography of the chest and abdomen.

  8. Evaluation of Risk Factors for Vertebral Compression Fracture after Carbon-Ion Radiotherapy for Primary Spinal and Paraspinal Sarcoma

    Directory of Open Access Journals (Sweden)

    Yoshihiro Matsumoto

    2017-01-01

    Full Text Available Background and Purpose. Carbon-ion radiotherapy (C-ion RT was effective therapy for inoperable spinal and paraspinal sarcomas. However, a significant adverse event following radiotherapies is vertebral compression fractures (VCFs. In this study, we investigated the incidence of and risk factors for post-C-ion RT VCFs in patients with spinal or paraspinal sarcomas. Material and Methods. Thirty consecutive patients with spinal or paraspinal sarcomas treated with C-ion RT were retrospectively reviewed. Various clinical parameters and the Spinal Instability Neoplastic Score (SINS were used to evaluate the risk factors for post-C-ion RT VCFs. Results. The overall incidence of VCFs was 23% (median time: 7 months. Patients with VCFs showed a markedly higher SINS score (median value, 9 points than those without VCF (5 points. The area under the receiver operating characteristic curve for the SINS score was 0.88, and the optimum SINS cut-off score was 8 points. The cumulative incidence of VCFs at 1 year was 9% for patients with a SINS score under 8 points, versus 80% for those with a SINS score of 8 points or higher (p<0.0001. Conclusions. In patients with a SINS score of 8 points or higher, referral to a spine surgeon for stabilization and multidisciplinary discussion is appropriate.

  9. Kaposi’s Sarcoma Herpesvirus Genome Persistence

    Directory of Open Access Journals (Sweden)

    Franceline Juillard

    2016-08-01

    Full Text Available Kaposi’s sarcoma-associated herpesvirus (KSHV has an etiologic role in Kaposi’s sarcoma, primary effusion lymphoma and multicentric Castleman’s disease. These diseases are most common in immunocompromised individuals, especially those with AIDS. Similar to all herpesviruses, KSHV infection is lifelong. KSHV infection in tumor cells is primarily latent, with only a small subset of cells undergoing lytic infection. During latency, the KSHV genome persists as a multiple copy, extrachromosomal episome in the nucleus. In order to persist in proliferating tumor cells, the viral genome replicates once per cell cycle and then segregates to daughter cell nuclei. KSHV only expresses several genes during latent infection. Prominent among these genes, is the latency-associated nuclear antigen (LANA. LANA is responsible for KSHV genome persistence and also exerts transcriptional regulatory effects. LANA mediates KSHV DNA replication and in addition, is responsible for segregation of replicated genomes to daughter nuclei. LANA serves as a molecular tether, bridging the viral genome to mitotic chromosomes to ensure that KSHV DNA reaches progeny nuclei. N-terminal LANA attaches to mitotic chromosomes by binding histones H2A/H2B at the surface of the nucleosome. C-terminal LANA binds specific KSHV DNA sequence and also has a role in chromosome attachment. In addition to the essential roles of N- and C-terminal LANA in genome persistence, internal LANA sequence is also critical for efficient episome maintenance. LANA’s role as an essential mediator of virus persistence makes it an attractive target for inhibition in order to prevent or treat KSHV infection and disease.

  10. The Epidemiology of Sarcoma

    OpenAIRE

    Burningham Zachary; Hashibe Mia; Spector Logan; Schiffman Joshua D

    2012-01-01

    Abstract Sarcomas account for over 20% of all pediatric solid malignant cancers and less than 1% of all adult solid malignant cancers. The vast majority of diagnosed sarcomas will be soft tissue sarcomas, while malignant bone tumors make up just over 10% of sarcomas. The risks for sarcoma are not well-understood. We evaluated the existing literature on the epidemiology and etiology of sarcoma. Risks for sarcoma development can be divided into environmental exposures, genetic susceptibility, a...

  11. A Unique Case of Malignant Pleuropericardial Effusion: HHV-8-Unrelated PEL-Like Lymphoma-A Case Report and Review of the Literature.

    Science.gov (United States)

    Mohammad, Farhan; Siddique, Muhammad Neaman; Siddiqui, Faraz; Popalzai, M; Asgari, Masoud; Odaimi, Marcel

    2014-01-01

    Primary effusion lymphoma (PEL) or body cavity lymphoma is a rare type of extra nodal lymphoma of B-cell origin that presents as lymphomatous effusion(s) without any nodal enlargement or tumor masses. It belongs to the group of AIDS related non-Hodgkin's lymphomas. First described in 1996 in HIV infected individuals who were coinfected with Kaposi's sarcoma-associated herpesvirus (KSHV) or HHV-8 virus, it was included as a separate entity in WHO classification of tumors of hematopoietic and lymphoid tissue in the year 2001. The definition included association with HHV-8 virus as a mandatory diagnostic criterion. However, cases were later reported where PEL-like disease process was diagnosed in HHV-8 negative patients. This was eventually recognized as a rare but distinct entity termed as "HHV-8-unrelated PEL-like lymphoma". Herein, we are reporting a case of an elderly patient who presented with a large pleuropericardial effusion and was eventually diagnosed with this entity. Till date, only around 50 cases of HHV-8-unrelated PEL-like lymphoma have been reported and our case being EBV, HIV, and Hepatitis C negative makes it very unique and rare occurrence. We are also presenting a review of relevant literature focused mainly on comparing outcomes in patients treated with and without chemotherapy.

  12. A Unique Case of Malignant Pleuropericardial Effusion: HHV-8-Unrelated PEL-Like Lymphoma—A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Farhan Mohammad

    2014-01-01

    Full Text Available Primary effusion lymphoma (PEL or body cavity lymphoma is a rare type of extra nodal lymphoma of B-cell origin that presents as lymphomatous effusion(s without any nodal enlargement or tumor masses. It belongs to the group of AIDS related non-Hodgkin’s lymphomas. First described in 1996 in HIV infected individuals who were coinfected with Kaposi’s sarcoma-associated herpesvirus (KSHV or HHV-8 virus, it was included as a separate entity in WHO classification of tumors of hematopoietic and lymphoid tissue in the year 2001. The definition included association with HHV-8 virus as a mandatory diagnostic criterion. However, cases were later reported where PEL-like disease process was diagnosed in HHV-8 negative patients. This was eventually recognized as a rare but distinct entity termed as “HHV-8-unrelated PEL-like lymphoma”. Herein, we are reporting a case of an elderly patient who presented with a large pleuropericardial effusion and was eventually diagnosed with this entity. Till date, only around 50 cases of HHV-8-unrelated PEL-like lymphoma have been reported and our case being EBV, HIV, and Hepatitis C negative makes it very unique and rare occurrence. We are also presenting a review of relevant literature focused mainly on comparing outcomes in patients treated with and without chemotherapy.

  13. Pathology of Kaposi's sarcoma-associated herpesvirus infection

    Directory of Open Access Journals (Sweden)

    Hitomi eFukumoto

    2011-08-01

    Full Text Available Kaposi’s sarcoma-associated herpesvirus (KSHV, human herpesvirus 8, HHV-8 is a human herpesvirus, classified as a gamma-herpesvirus. KSHV is detected in Kaposi’s sarcoma (KS, primary effusion lymphoma (PEL, and some cases of multicentric Castleman’s disease (MCD. Similar to other herpes viruses, there are two phases of infection, latent and lytic. In KSHV-associated malignancies such as KS and PEL, KSHV latently infects almost all tumor cells. Quantitative PCR analysis revealed that each tumor cell contains one copy of KSHV in KS lesions. The oncogenesis by KSHV has remained unclear. Latency-associated nuclear antigen (LANA-1 plays an important role in the pathogenesis of KSHV-associated malignancies through inhibition of apoptosis and maintenance of latency. Because all KSHV-infected cells express LANA-1, LANA-1 immunohistochemistry is a useful tool for diagnosis of KSHV infection. KSHV encodes some homologs of cellular proteins including cell-cycle regulators, cytokines and chemokines, such as cyclin D, G-protein coupled protein, interleukin-6, and macrophage inflammatory protein-1 and -2. These viral proteins mimic or disrupt host cytokine signals, resulting in microenvironments amenable to tumor growth. Lytic infection is frequently seen in MCD tissues, suggesting a different pathogenesis from KS and lymphoma.

  14. Histiocytic sarcoma

    Directory of Open Access Journals (Sweden)

    Eduardo Silva Machado

    2011-01-01

    Full Text Available A 59-year-old white woman, SC, after being treated for pneumonia, presented with an increase in the size of lymph nodes. The immunohistochemical examination diagnosed histiocytic sarcoma. Relapse occurred 12 months after starting chemotherapy. The patient evolved with febrile neutropenia, septic shock and death.

  15. Semi-Quantitative Calculations of Primary Tumor Metabolic Activity Using F-18 FDG PET/CT as a Predictor of Survival in 92 Patients With High-Grade Bone or Soft Tissue Sarcoma

    DEFF Research Database (Denmark)

    Andersen, Kim Francis; Fuglo, Hanna Maria; Rasmussen, Sine Hvid;

    2015-01-01

    To assess the prognostic value of primary tumor metabolic activity in patients with high-grade bone sarcomas (BS) or soft tissue sarcomas (STS) using F-18 FDG PET/CT. A single-site, retrospective study including 92 patients with high-grade BS or STS. Pretreatment F-18 FDG PET/CT scan was performed...... metabolic activity with pretherapeutic SUVmax using F-18 FDG PET/CT demonstrates independent properties beyond histologic grading for prediction of survival in patients with high-grade STS, but not with high-grade BS....

  16. Delays in the management of retroperitoneal sarcomas

    DEFF Research Database (Denmark)

    Seinen, Jojanneke; Almquist, Martin; Styring, Emelie

    2010-01-01

    at the general practitioner, 36 days at local hospitals, and 55 days at the sarcoma centre. Conclusion. Centralization per se is not sufficient for optimized and efficient management. Our findings suggest that delays can be minimized by direct referral of patients from primary health care to sarcoma centers...... sarcoma in the southern Sweden health care region 2003-2009 were eligible for the study. Data on referrals and diagnostic investigations were collected from clinical files from primary health care, local hospitals, and from the sarcoma centre. Lead times were divided into patient delays and health care...... delays caused by primary health care, local hospitals, or procedures at the sarcoma centre. Results. Complete data were available from 33 patients and demonstrated a median patient delay of 23 days (0-17 months) and median health care delay of 94 days (1-40 months) with delays of median 15 days...

  17. Delays in the management of retroperitoneal sarcomas

    DEFF Research Database (Denmark)

    Seinen, Jojanneke; Almquist, Martin; Styring, Emelie

    2010-01-01

    at the general practitioner, 36¿days at local hospitals, and 55¿days at the sarcoma centre. Conclusion. Centralization per se is not sufficient for optimized and efficient management. Our findings suggest that delays can be minimized by direct referral of patients from primary health care to sarcoma centers...... sarcoma in the southern Sweden health care region 2003-2009 were eligible for the study. Data on referrals and diagnostic investigations were collected from clinical files from primary health care, local hospitals, and from the sarcoma centre. Lead times were divided into patient delays and health care...... delays caused by primary health care, local hospitals, or procedures at the sarcoma centre. Results. Complete data were available from 33 patients and demonstrated a median patient delay of 23¿days (0-17¿months) and median health care delay of 94¿days (1-40¿months) with delays of median 15¿days...

  18. Extrauterine Low-Grade Endometrial Stromal Sarcoma

    Directory of Open Access Journals (Sweden)

    Yu-Ju Chen

    2005-12-01

    Conclusions: Low-grade endometrial stromal sarcoma typically has an indolent clinical course and favorable prognosis. Surgical resection is the primary therapeutic approach, and adjuvant therapy with radiotherapy, chemotherapy, or progesterone therapy should be considered for the management of residual or recurrent low-grade endometrial stromal sarcomas.

  19. Intra-Articular Synovial Sarcomas: Incidence and Differentiating Features from Localized Pigmented Villonodular Synovitis.

    Science.gov (United States)

    Nordemar, D; Öberg, J; Brosjö, O; Skorpil, M

    2015-01-01

    Purpose. To determine the incidence of intra-articular synovial sarcomas and investigate if any radiological variables can differentiate them from localized (unifocal) pigmented villonodular synovitis (PVNS) and if multivariate data analysis could be used as a complementary clinical tool. Methods. Magnetic resonance images and radiographs of 7 cases of intra-articular synovial sarcomas and 14 cases of localized PVNS were blindedly reviewed. Variables analyzed were size, extra-articular growth, tumor border, blooming, calcification, contrast media enhancement, effusion, bowl of grapes sign, triple signal intensity sign, synovial low signal intensity, synovitis, age, and gender. Univariate and multivariate data analysis, the method of partial least squares-discriminant analysis (PLS-DA), were used. Register data on all synovial sarcomas were extracted for comparison. Results. The incidence of intra-articular synovial sarcomas was 3%. PLS-DA showed that age, effusion, size, and gender were the most important factors for discrimination between sarcomas and localized PVNS. No sarcomas were misclassified as PVNS with PLS-DA, while some PVNS were misclassified as sarcomas. Conclusions. The most important variables in differentiating intra-articular sarcomas from localized PVNS were age, effusion, size, and gender. Multivariate data analysis can be helpful as additive information to avoid a biopsy, if the tumor is classified as most likely being PVNS.

  20. Intra-Articular Synovial Sarcomas: Incidence and Differentiating Features from Localized Pigmented Villonodular Synovitis

    Directory of Open Access Journals (Sweden)

    D. Nordemar

    2015-01-01

    Full Text Available Purpose. To determine the incidence of intra-articular synovial sarcomas and investigate if any radiological variables can differentiate them from localized (unifocal pigmented villonodular synovitis (PVNS and if multivariate data analysis could be used as a complementary clinical tool. Methods. Magnetic resonance images and radiographs of 7 cases of intra-articular synovial sarcomas and 14 cases of localized PVNS were blindedly reviewed. Variables analyzed were size, extra-articular growth, tumor border, blooming, calcification, contrast media enhancement, effusion, bowl of grapes sign, triple signal intensity sign, synovial low signal intensity, synovitis, age, and gender. Univariate and multivariate data analysis, the method of partial least squares-discriminant analysis (PLS-DA, were used. Register data on all synovial sarcomas were extracted for comparison. Results. The incidence of intra-articular synovial sarcomas was 3%. PLS-DA showed that age, effusion, size, and gender were the most important factors for discrimination between sarcomas and localized PVNS. No sarcomas were misclassified as PVNS with PLS-DA, while some PVNS were misclassified as sarcomas. Conclusions. The most important variables in differentiating intra-articular sarcomas from localized PVNS were age, effusion, size, and gender. Multivariate data analysis can be helpful as additive information to avoid a biopsy, if the tumor is classified as most likely being PVNS.

  1. Epigenetic Landscape of Kaposi's Sarcoma-Associated Herpesvirus Genome in Classic Kaposi's Sarcoma Tissues

    Science.gov (United States)

    Wang, Xiaodong; Yang, Lei; Robertson, Erle S.; Lan, Ke

    2017-01-01

    Kaposi's sarcoma-associated herpesvirus (KSHV) is etiologically related to Kaposi's sarcoma (KS), primary effusion lymphoma (PEL) and multicentric Castleman's disease (MCD). It typically displays two different phases in its life cycle, the default latency and occasional lytic replication. The epigenetic modifications are thought to determine the fate of KSHV infection. Previous studies elegantly depicted epigenetic landscape of latent viral genome in in vitro cell culture systems. However, the physiologically relevant scenario in clinical KS tissue samples is unclear. In the present study, we established a protocol of ChIP-Seq for clinical KS tissue samples and mapped out the epigenetic landscape of KSHV genome in classic KS tissues. We examined AcH3 and H3K27me3 histone modifications on KSHV genome, as well as the genome-wide binding sites of latency associated nuclear antigen (LANA). Our results demonstrated that the enriched AcH3 was mainly restricted at latent locus while H3K27me3 was widespread on KSHV genome in classic KS tissues. The epigenetic landscape at the region of vIRF3 gene confirmed its silenced state in KS tissues. Meanwhile, the abundant enrichment of LANA at the terminal repeat (TR) region was also validated in the classic KS tissues, however, different LANA binding sites were observed on the host genome. Furthermore, we verified the histone modifications by ChIP-qPCR and found the dominant repressive H3K27me3 at the promoter region of replication and transcription activator (RTA) in classic KS tissues. Intriguingly, we found that the TR region in classic KS tissues was lacking in AcH3 histone modifications. These data now established the epigenetic landscape of KSHV genome in classic KS tissues, which provides new insights for understanding KSHV epigenetics and pathogenesis. PMID:28118409

  2. Molecular biology of sarcomas.

    Science.gov (United States)

    Gebhardt, M C

    1996-07-01

    There has been a virtual explosion of information relating to the biology of sarcomas with which we as orthopaedists deal. Much more is yet to be learned. These findings will teach us more about the etiology of these tumors. More important, the findings will alter the way in which these tumors are treated. It is unlikely that we will continue to treat osteosarcoma or Ewing's sarcoma patients with currently available drug regimens and surgery or make treatment decisions based on the histologic classification of tumors we know today. If we are to remain active in the management of these patients we must be aware of the findings as they occur. That will ensure both that we remain the primary caretakers of these patients, and that we will continue to be stimulated intellectually by these intriguing scientific investigations.

  3. Sarcoma Immunotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Gouw, Launce G., E-mail: launce.gouw@hsc.utah.edu [Departments of Oncology, Huntsman Cancer Institute at the University of Utah, 2000 Circle of Hope, Salt Lake City, UT 84112 (United States); Jones, Kevin B. [Departments of Orthopaedic Surgery, Huntsman Cancer Institute at the University of Utah, 2000 Circle of Hope, Salt Lake City, UT 84112 (United States); Sharma, Sunil [Departments of Oncology, Huntsman Cancer Institute at the University of Utah, 2000 Circle of Hope, Salt Lake City, UT 84112 (United States); Randall, R. Lor [Departments of Orthopaedic Surgery, Huntsman Cancer Institute at the University of Utah, 2000 Circle of Hope, Salt Lake City, UT 84112 (United States)

    2011-11-10

    Much of our knowledge regarding cancer immunotherapy has been derived from sarcoma models. However, translation of preclinical findings to bedside success has been limited in this disease, though several intriguing clinical studies hint at the potential efficacy of this treatment modality. The rarity and heterogeneity of tumors of mesenchymal origin continues to be a challenge from a therapeutic standpoint. Nonetheless, sarcomas remain attractive targets for immunotherapy, as they can be characterized by specific epitopes, either from their mesenchymal origins or specific alterations in gene products. To date, standard vaccine trials have proven disappointing, likely due to mechanisms by which tumors equilibrate with and ultimately escape immune surveillance. More sophisticated approaches will likely require multimodal techniques, both by enhancing immunity, but also geared towards overcoming innate mechanisms of immunosuppression that favor tumorigenesis.

  4. Synovial sarcoma presenting with huge mediastinal mass: a case report and review of literature

    Science.gov (United States)

    2013-01-01

    Background Synovial sarcoma presenting in the mediastinum is exceedingly rare. Furthermore, data addressing optimal therapy is limited. Herein we present a case where an attempt to downsize the tumor to a resectable state with chemotherapy was employed. Case presentation A 32 year female presented with massive pericardial effusion and unresectable huge mediastinal mass. Computed axial tomography scan - guided biopsy with adjunctive immunostains and molecular studies confirmed a diagnosis of synovial sarcoma. Following three cycles of combination Ifosfamide and doxorubicin chemotherapy, no response was demonstrated. The patient refused further therapy and had progression of her disease 4 months following the last cycle. Conclusion Synovial sarcoma presenting with unresectable mediastinal mass carry a poor prognosis. Up to the best of our knowledge there are only four previous reports where primary chemotherapy was employed, unfortunately; none of these cases had subsequent complete surgical resection. Identification of the best treatment strategy for patients with unresectable disease is warranted. Our case can be of benefit to medical oncologists and thoracic surgeons who might be faced with this unique and exceedingly rare clinical scenario. PMID:23800262

  5. Primary intracranial soft tissue sarcoma in children and adolescents: a cooperative analysis of the European CWS and HIT study groups.

    Science.gov (United States)

    Benesch, Martin; von Bueren, André O; Dantonello, Tobias; von Hoff, Katja; Pietsch, Torsten; Leuschner, Ivo; Claviez, Alexander; Bierbach, Uta; Kropshofer, Gabriele; Korinthenberg, Rudolf; Graf, Norbert; Suttorp, Meinolf; Kortmann, Rolf Dieter; Friedrich, Carsten; von der Weid, Nicolas; Kaatsch, Peter; Klingebiel, Thomas; Koscielniak, Ewa; Rutkowski, Stefan

    2013-02-01

    Purely intracranial soft tissue sarcomas (ISTS) are very rare among children. A retrospective database analysis of the Cooperative Weichteilsarkom Studiengruppe (CWS) and brain tumor (HIT) registries was conducted to describe treatment and long-term outcome of children and adolescents with ISTS. Nineteen patients from Germany, Austria and Switzerland were reported between 1988 and 2009. Median age at diagnosis was 9.7 years (range, 0.5-17.8). Central pathological review was performed in 17 patients. Eleven patients underwent a total and five a subtotal tumor resection. A biopsy was done in one patient. In two patients no data concerning extent of initial resection was available. Radiotherapy was performed in 15 patients (first-line, n = 11; following progression, n = 4). All but one patient received chemotherapy (first-line, n = 7, following progression, n = 5; first-line and following progression, n = 6). With a median follow-up of 5.8 years (range, 0.6-19.8) ten patients were alive in either first or second complete remission. Seven patients died due to relapse or progression and two were alive with progressive disease. Estimated progression-free and overall survival at 5 years were 47 % (±12 %) and 74 % (±10 %), respectively. About 50 % of patients with ISTS remain relapse-free after 5 years. Multimodality treatment including complete tumor resection and radio-/chemotherapy is required to achieve sustained tumor control in patients with ISTS. Early initiation of postoperative non-surgical treatment seems to be important to prevent recurrence. Due to the intracranial localization local therapy should follow the recommendations used in brain tumors rather than in soft tissue sarcomas, whereas chemotherapy should be guided by histological subtype.

  6. Large pericardial effusion :a clinical dilemma!

    Science.gov (United States)

    Ramalingam, Rangaraj; Kadako, Nitinkumar S; Pati, Shivanand; Manjunath, C N

    2013-07-01

    A 55yr old gentleman known diabetic and hypertensive presented with breathlessness and tingling sensation of both upper and lower limbs with strong family history of similar neurological problems. On extensive evaluation he was found to have amyloidic peripheral neuropathy with large pericardial effusion. Tubercular etiology was confirmed by pericardial fluid PCR and culture. Here the diagnostic dilemma was whether Amloidosis is primary, secondary to Tubercular pericardial effusion or Hereditary Amyloidosis. In the end, how we have arrived at the diagnosis of Hereditary Amyloidosis based upon the strong family history and nerve biopsy is interestingly presented in the following case report.

  7. Antibodies against lytic and latent Kaposi's sarcoma-associated herpes virus antigens and lymphoma in the European EpiLymph case–control study

    Science.gov (United States)

    Benavente, Y; Mbisa, G; Labo, N; Casabonne, D; Becker, N; Maynadie, M; Foretova, L; Cocco, P L; Nieters, A; Staines, A; Bofetta, P; Brennan, P; Whitby, D; de Sanjosé, S

    2011-01-01

    Background: Kaposi's sarcoma-associated herpes virus is associated with primary effusion lymphoma and multicentric Castleman's disease. Methods: Seropositivity to lytic and latent Kaposi's sarcoma herpes virus (KSHV) antigens were examined in 2083 lymphomas and 2013 controls from six European countries. Results: Antibodies against KSHV latent and lytic antigens were detectable in 4.5% and 3.4% of controls, respectively, and 3.6% of cases (P>0.05). The KSHV seropositivity was associated with splenic marginal zone lymphoma (SMZL) (odds ratio (OR)=4.11, 95% confidence interval (CI)=1.57–10.83) and multiple myeloma (OR=0.31, 95% CI=0.11–0.85). Conclusion: The KSHV is unlikely to contribute importantly to lymphomagenesis among immunocompetent subjects. However, the observed association with SMZL may underline a chronic antigen mechanism in its aetiology. PMID:21952625

  8. 原发性髓细胞肉瘤6例并文献分析%Primary myeloid sarcoma: a report of six cases and literature review

    Institute of Scientific and Technical Information of China (English)

    曾惠; 胡俊斌; 郭晓珺

    2012-01-01

    Objective:To study the clinico-pathological characteristics, diagnose and treatment of the primary myeloid sarcoma (MS). Method; The diagnostic and treatment data for six cases of primary MS were retrospectively analyzed in combination with relevant literature. Result- Lymphonode, skin, reproductive organs and digestive organs were the most common extramedullary sites of involvement by primary MS. Imminohistochemistry features of six cases were positive of MPO. Three patients progressed to acute myeloid leukemii( AMD , and another three cases did not develop to leukemia. At present two patients survived, one patient recurned, and the remaining three cases were dead. Conclusion: Immunohistochemistry is essential for the diagnosis of NS. Intensive chemotherapy similar with that/used to treat AML would improve long-term survival.%目的:提高对原发性髓细胞肉瘤(myeloid sarcoma,MS)的临床病理特点、诊断、治疗的认识.方法:回顾性分析6例MS患者的诊治资料,结合相关文献分析MS的临床表现、免疫组织化学特点、治疗方法与预后. 结果:淋巴结、皮肤、泌尿生殖系统和消化系统是MS最常见的发生部位.免疫组织化学示6例病例的MPO均阳性. 3例患者进展为急性髓系白血病,另外3例患者骨髓未累及.目前2例存活,1例复发,其余3例均已死亡.结论:免疫组织化学检查对明确诊断很重要.强有力的抗白血病样化疗可提高其长期生存率.

  9. Tuberculous pleural effusion.

    Science.gov (United States)

    Ferreiro, Lucía; San José, Esther; Valdés, Luis

    2014-10-01

    Tuberculous pleural effusion (TBPE) is the most common form of extrapulmonary tuberculosis (TB) in Spain, and is one of the most frequent causes of pleural effusion. Although the incidence has steadily declined (4.8 cases/100,000population in 2009), the percentage of TBPE remains steady with respect to the total number of TB cases (14.3%-19.3%). Almost two thirds are men, more than 60% are aged between 15-44years, and it is more common in patients with human immunodeficiency virus. The pathogenesis is usually a delayed hypersensitivity reaction. Symptoms vary depending on the population (more acute in young people and more prolonged in the elderly). The effusion is almost invariably a unilateral exudate (according to Light's criteria), more often on the right side, and the tuberculin test is negative in one third of cases. There are limitations in making a definitive diagnosis, so various pleural fluid biomarkers have been used for this. The combination of adenosine deaminase and lymphocyte percentage may be useful in this respect. Treatment is the same as for any TB. The addition of corticosteroids is not advisable, and chest drainage could help to improve symptoms more rapidly in large effusions. Copyright © 2013 SEPAR. Published by Elsevier Espana. All rights reserved.

  10. Symptomatic Pericardial Effusion After Chemoradiation Therapy in Esophageal Cancer Patients

    Energy Technology Data Exchange (ETDEWEB)

    Fukada, Junichi, E-mail: fukada@rad.med.keio.ac.jp [Department of Radiology, School of Medicine, Keio University, Tokyo (Japan); Shigematsu, Naoyuki [Department of Radiology, School of Medicine, Keio University, Tokyo (Japan); Takeuchi, Hiroya [Department of Surgery, School of Medicine, Keio University, Tokyo (Japan); Ohashi, Toshio [Department of Radiology, School of Medicine, Keio University, Tokyo (Japan); Saikawa, Yoshiro [Department of Surgery, School of Medicine, Keio University, Tokyo (Japan); Takaishi, Hiromasa [Division of Gastroenterology and Hepatology, Department of Internal Medicine, School of Medicine, Keio University, Tokyo (Japan); Hanada, Takashi; Shiraishi, Yutaka [Department of Radiology, School of Medicine, Keio University, Tokyo (Japan); Kitagawa, Yuko [Department of Surgery, School of Medicine, Keio University, Tokyo (Japan); Fukuda, Keiichi [Department of Cardiology, School of Medicine, Keio University, Tokyo (Japan)

    2013-11-01

    Purpose: We investigated clinical and treatment-related factors as predictors of symptomatic pericardial effusion in esophageal cancer patients after concurrent chemoradiation therapy. Methods and Materials: We reviewed 214 consecutive primary esophageal cancer patients treated with concurrent chemoradiation therapy between 2001 and 2010 in our institute. Pericardial effusion was detected on follow-up computed tomography. Symptomatic effusion was defined as effusion ≥grade 3 according to Common Terminology Criteria for Adverse Events v4.0 criteria. Percent volume irradiated with 5 to 65 Gy (V5-V65) and mean dose to the pericardium were evaluated employing dose-volume histograms. To evaluate dosimetry for patients treated with two-dimensional planning in the earlier period (2001-2005), computed tomography data at diagnosis were transferred to a treatment planning system to reconstruct three-dimensional plans without modification. Optimal dosimetric thresholds for symptomatic pericardial effusion were calculated by receiver operating characteristic curves. Associating clinical and treatment-related risk factors for symptomatic pericardial effusion were detected by univariate and multivariate analyses. Results: The median follow-up was 29 (range, 6-121) months for eligible 167 patients. Symptomatic pericardial effusion was observed in 14 (8.4%) patients. Dosimetric analyses revealed average values of V30 to V45 for the pericardium and mean pericardial doses were significantly higher in patients with symptomatic pericardial effusion than in those with asymptomatic pericardial effusion (P<.05). Pericardial V5 to V55 and mean pericardial doses were significantly higher in patients with symptomatic pericardial effusion than in those without pericardial effusion (P<.001). Mean pericardial doses of 36.5 Gy and V45 of 58% were selected as optimal cutoff values for predicting symptomatic pericardial effusion. Multivariate analysis identified mean pericardial dose as the

  11. Chemotherapy for Soft Tissue Sarcomas

    Science.gov (United States)

    ... Stage Soft Tissue Sarcoma Treating Soft Tissue Sarcomas Chemotherapy for Soft Tissue Sarcomas Chemotherapy (chemo) is the use of drugs given into ... Depending on the type and stage of sarcoma, chemotherapy may be given as the main treatment or ...

  12. The Epidemiology of Sarcoma

    Directory of Open Access Journals (Sweden)

    Burningham Zachary

    2012-10-01

    Full Text Available Abstract Sarcomas account for over 20% of all pediatric solid malignant cancers and less than 1% of all adult solid malignant cancers. The vast majority of diagnosed sarcomas will be soft tissue sarcomas, while malignant bone tumors make up just over 10% of sarcomas. The risks for sarcoma are not well-understood. We evaluated the existing literature on the epidemiology and etiology of sarcoma. Risks for sarcoma development can be divided into environmental exposures, genetic susceptibility, and an interaction between the two. HIV-positive individuals are at an increased risk for Kaposi’s sarcoma, even though HHV8 is the causative virus. Radiation exposure from radiotherapy has been strongly associated with secondary sarcoma development in certain cancer patients. In fact, the risk of malignant bone tumors increases as the cumulative dose of radiation to the bone increases (p for trend

  13. Do We Know What Causes Kaposi Sarcoma?

    Science.gov (United States)

    ... and Prevention Do We Know What Causes Kaposi Sarcoma? Kaposi sarcoma (KS) is caused by infection with a ... Sarcoma? Can Kaposi Sarcoma Be Prevented? More In Kaposi Sarcoma About Kaposi Sarcoma Causes, Risk Factors, and Prevention ...

  14. Acute subdural effusion in vasculitis

    Directory of Open Access Journals (Sweden)

    Raghavendra Seetharam

    2008-01-01

    Full Text Available We report a 29-year-old man with a unique presentation of vasculitis as acute unilateral subdural effusion and meningoencephalitis. Magnetic resonance imaging showed a brainstem lesion that spread to the thalamus over time. There were no systemic features of vasculitis other than a positive pathergy test. Histopathological examination from the pathergy site showed neutrophilic infiltrate and leucocytoclastic vasculitis. The condition was steroid responsive and he remained in remission at two years′ follow-up. The anatomy of the brainstem lesion, absence of other inflammatory and infective conditions on evaluation suggests a vasculitic pathology either as primary central nervous system angiitis or as neurological presentation of systemic vasculitis like Behηet′s disease although the international diagnostic criteria for Behηet′s were not fulfilled.

  15. Palliative Treatment of Malignant Pleural Effusion

    Directory of Open Access Journals (Sweden)

    Chenyang Liu

    2015-01-01

    Full Text Available Malignant pleural effusion (MPE is a common clinical problem caused by cancers. Pleural effusion can be the first sign of cancer in more than 25% of patients. Lung cancer and breast cancer are the most common cancers that metastasize to the pleura in men and women, respectively. Other cancers, including, but not limited to, lymphomas, ovarian cancer, stomach cancer, and several unknown primary cancers can also lead to MPE. Dyspnea and chest pain are the most common symptoms of MPE along with other symptoms such as a cough, weight loss, anorexia, fatigue, and weakness. Aggravation of these symptoms is closely related to the rate of accumulation of pleural effusion. Treatment options to MPE are determined by the type and extent of the underlying malignancy. The major goals of the treatment are to relieve symptoms, restore functions, improve the quality of life, and minimize the duration of hospital stay and costs. Although some patients can be treated with systemic therapies, most of these treatments are temporary, and MPE would recur soon. Hence, further palliative treatments to effectively control pleural effusions and relieve symptoms are necessary. This review addresses the pathophysiology of MPE and the treatment options for patients with MPE.

  16. Severe iritis and choroidal effusion following selective laser trabeculoplasty.

    Science.gov (United States)

    Kim, Danny Y; Singh, Anna

    2008-01-01

    A patient with uncontrolled primary open-angle glaucoma underwent selective laser trabeculoplasty and developed a significant anterior chamber reaction, shallow anterior chamber, and choroidal effusion. Common complications associated with selective laser trabeculoplasty include conjunctival injection, mild anterior chamber reaction, and post-treatment intraocular pressure elevation. The authors believe this is the first reported case of severe iritis and choroidal effusion following selective laser trabeculoplasty.

  17. SYNOVIAL CELL SARCOMA

    Directory of Open Access Journals (Sweden)

    M. Farzan

    1997-06-01

    Full Text Available Ten cases of synovial cell sarcoma are reported. The youngest patient was a 2'A years old boy with synovial cell sarcoma of the knee and the oldest one was a man with synovial cell sarcoma of the elbow.

  18. 骶骨原发尤文肉瘤15例分析%Clinical analysis of primary Ewing’s sarcoma of the sacrum:15 cases report

    Institute of Scientific and Technical Information of China (English)

    李晓; 郭卫; 杨荣利; 杨毅; 唐顺

    2014-01-01

    Objective To investigate the clinical features, treatment strategies and prognoses of primary Ewing’s sarcoma of the sacrum. Methods The data of 15 patients with primary Ewing’s sarcoma of the sacrum who were adopted from June 1998 to June 2011 were retrospectively analyzed. There were 9 males and 6 females, whose average age was 18 years old ( range;11-45 years ). All the patients had sacrococcygeal pain, and 11 patients had perineal numbness and pain in the lower extremities. Three patients had palpable masses. The single segment was invaded in 2 cases, and double or more segments were invaded in 13 cases. The preoperative X-ray iflms and CT scans indicated that there was much irregular osteolytic bone destruction in the sacral vertebra. The CT and MRI examination often showed huge soft tissue masses. The systemic chemotherapy and surgical treatment were performed on all the patients, and the radiotherapy on 10 patients postoperatively. Nine patients underwent block resection of sacral tumors and reconstruction, and 6 patients underwent en bloc resection, among whom 4 patients received one-stage total sacrectomy by a combined anterior-posterior approach and rod-screw reconstruction. Results All patients were followed up for an average period of 5.2 years ( range, 2-10 years ). The local pain in all the patients got relieved. Five patients had perioperative complications and 4 of them had wound healing problems. Eleven patients had bladder and bowel dysfunction in varying degrees. During the follow-up, 10 cases of local recurrence and metastasis were found. The local recurrence rate was 60%( 9/15 ) and the lung metastasis rate was 33.3%( 5/15 ), including 4 cases of both local recurrence and lung metastasis. Among the 6 patients treated by en bloc resection, 1 patient had local recurrence in the sacrum and 1 patient had both local recurrence and lung metastasis. The duration of metastasis and local recurrence was 7-60 months. The 10 patients who had local

  19. Ewing’s Sarcoma: An Analysis of miRNA Expression Profiles and Target Genes in Paraffin-Embedded Primary Tumor Tissue

    Directory of Open Access Journals (Sweden)

    Antonina Parafioriti

    2016-04-01

    Full Text Available The molecular mechanism responsible for Ewing’s Sarcoma (ES remains largely unknown. MicroRNAs (miRNAs, a class of small non-coding RNAs able to regulate gene expression, are deregulated in tumors and may serve as a tool for diagnosis and prediction. However, the status of miRNAs in ES has not yet been thoroughly investigated. This study compared global miRNAs expression in paraffin-embedded tumor tissue samples from 20 ES patients, affected by primary untreated tumors, with miRNAs expressed in normal human mesenchymal stromal cells (MSCs by microarray analysis. A miRTarBase database was used to identify the predicted target genes for differentially expressed miRNAs. The miRNAs microarray analysis revealed distinct patterns of miRNAs expression between ES samples and normal MSCs. 58 of the 954 analyzed miRNAs were significantly differentially expressed in ES samples compared to MSCs. Moreover, the qRT-PCR analysis carried out on three selected miRNAs showed that miR-181b, miR-1915 and miR-1275 were significantly aberrantly regulated, confirming the microarray results. Bio-database analysis identified BCL-2 as a bona fide target gene of the miR-21, miR-181a, miR-181b, miR-29a, miR-29b, miR-497, miR-195, miR-let-7a, miR-34a and miR-1915. Using paraffin-embedded tissues from ES patients, this study has identified several potential target miRNAs and one gene that might be considered a novel critical biomarker for ES pathogenesis.

  20. Ewing’s Sarcoma: An Analysis of miRNA Expression Profiles and Target Genes in Paraffin-Embedded Primary Tumor Tissue

    Science.gov (United States)

    Parafioriti, Antonina; Bason, Caterina; Armiraglio, Elisabetta; Calciano, Lucia; Daolio, Primo Andrea; Berardocco, Martina; Di Bernardo, Andrea; Colosimo, Alessia; Luksch, Roberto; Berardi, Anna C.

    2016-01-01

    The molecular mechanism responsible for Ewing’s Sarcoma (ES) remains largely unknown. MicroRNAs (miRNAs), a class of small non-coding RNAs able to regulate gene expression, are deregulated in tumors and may serve as a tool for diagnosis and prediction. However, the status of miRNAs in ES has not yet been thoroughly investigated. This study compared global miRNAs expression in paraffin-embedded tumor tissue samples from 20 ES patients, affected by primary untreated tumors, with miRNAs expressed in normal human mesenchymal stromal cells (MSCs) by microarray analysis. A miRTarBase database was used to identify the predicted target genes for differentially expressed miRNAs. The miRNAs microarray analysis revealed distinct patterns of miRNAs expression between ES samples and normal MSCs. 58 of the 954 analyzed miRNAs were significantly differentially expressed in ES samples compared to MSCs. Moreover, the qRT-PCR analysis carried out on three selected miRNAs showed that miR-181b, miR-1915 and miR-1275 were significantly aberrantly regulated, confirming the microarray results. Bio-database analysis identified BCL-2 as a bona fide target gene of the miR-21, miR-181a, miR-181b, miR-29a, miR-29b, miR-497, miR-195, miR-let-7a, miR-34a and miR-1915. Using paraffin-embedded tissues from ES patients, this study has identified several potential target miRNAs and one gene that might be considered a novel critical biomarker for ES pathogenesis. PMID:27144561

  1. High incidence of Kaposi sarcoma-associated herpesvirus infection in HIV-related solid immunoblastic/plasmablastic diffuse large B-cell lymphoma.

    Science.gov (United States)

    Deloose, S T P; Smit, L A; Pals, F T; Kersten, M-J; van Noesel, C J M; Pals, S T

    2005-05-01

    Kaposi sarcoma-associated herpesvirus (KSHV) is known to be associated with two distinct lymphoproliferative disorders: primary effusion lymphoma (PEL) and multicentric Castleman disease (MCD)/MCD-associated plasmablastic lymphoma. We here report a high incidence of KSHV infection in solid HIV-associated immunoblastic/plasmablastic non-Hodgkin's lymphomas (NHLs), in patients lacking effusions and without evidence of (prior) MCD. Within a cohort of 99 HIV-related NHLs, 10 cases were found to be KSHV positive on the basis of immunostaining for KSHV LNA-1 as well as KSHV-specific polymerase chain reaction. All but one of the tumors coexpressed Epstein-Barr virus. Interestingly, all KSHV-positive cases belonged to a distinctive subgroup of 26 diffuse large B-cell lymphomas characterized by the expression of CD138 (syndecan-1) and plasmablastic/immunoblastic morphology. These KSHV-positive lymphomas were preceded by Kaposi sarcoma in 60% of the patients and involved the gastrointestinal tract in 80%. Our results indicate that KSHV infection is not restricted to PEL and MCD; it is also common (38%) in HIV-related solid immunoblastic/plasmablastic lymphomas.

  2. Pulmonary Kaposi sarcoma in six children

    Energy Technology Data Exchange (ETDEWEB)

    Theron, Salomine [University of Stellenbosch, Department of Radiology, Tygerberg Academic Hospital, Cape Town (South Africa); University of Stellenbosch, Department of Radiology, Tygerberg Hospital, Cape Town (South Africa); Andronikou, Savvas; Plessis, Jaco du; George, Reena; Mapukata, Ayanda; Grobbelaar, Marie; Hayes, Murray [University of Stellenbosch, Department of Radiology, Tygerberg Academic Hospital, Cape Town (South Africa); Goussard, Pierre [University of Stellenbosch, Department of Child Health, Tygerberg Academic Hospital, Cape Town (South Africa); Wieselthaler, Nicky [University of Cape Town, Department of Radiology, Red Cross Children' s Hospital, Cape Town (South Africa); Davidson, Alan [University of Cape Town, Department of Oncology, Red Cross Children' s Hospital, Cape Town (South Africa)

    2007-12-15

    Pulmonary involvement in Kaposi sarcoma is rare in children and can be difficult to distinguish from other pathology. To describe the radiological findings in paediatric pulmonary Kaposi sarcoma. Sequential chest radiographs of six children and CT scans of four of these children were evaluated retrospectively. Their ages ranged from 18 months to 10 years; four were male and two were female. All six children were HIV-positive. The observers were two radiologists. Chest radiographs revealed air-space (100%) and reticular (83%) opacification in the mid- and lower lung zones; pleural effusions were present in 83% of the children. All the children showed progressive air-space opacification on follow-up radiography. CT demonstrated bilateral air-space opacification in a perihilar distribution in all the children; reticular opacification was seen in 75%. All the children had mediastinal and axillary lymphadenopathy; 75% had bilateral hilar lymphadenopathy. In both adults and children, chest radiography demonstrates perihilar and lower zone involvement. Pleural effusions are more common on radiographs in children. Air-space disease and lymphadenopathy are much more common on CT in children than adults. (orig.)

  3. Etiologies of bilateral pleural effusions

    Science.gov (United States)

    Puchalski, Jonathan T.; Argento, A. Christine; Murphy, Terrence E.; Araujo, Katy L.B.; Oliva, Isabel B.; Rubinowitz, Ami N.; Pisani, Margaret A.

    2017-01-01

    Summary Background To evaluate the safety, etiology and outcomes of patients undergoing bilateral thoracentesis. Methods This is a prospective cohort study of 100 consecutive patients who underwent bilateral thoracenteses in an academic medical center from July 2009 through November 2010. Pleural fluid characteristics and etiologies of the effusions were assessed. Mean differences in levels of fluid characteristics between right and left lungs were tested. Associations between fluid characteristics and occurrence of bilateral malignant effusions were evaluated. The rate of pneumothorax and other complications subsequent to bilateral thoracentesis was determined. Results Exudates were more common than transudates, and most effusions had multiple etiologies, with 83% having two or more etiologies. Bilateral malignant effusions occurred in 19 patients, were the most common single etiology of exudative effusions, and were associated with higher levels of protein and LDH in the pleural fluid. Among 200 thoracenteses performed with a bilateral procedure, seven resulted in pneumothoraces, three of which required chest tube drainage and four were ex vacuo. Conclusions More often than not, there are multiple etiologies that contribute to pleural fluid formation, and of the combinations of etiologies observed congestive heart failure was the most frequent contributor. Exudative effusions are more common than transudates when bilateral effusions are present. Malignancy is a common etiology of exudative effusions. This study suggests that the overall complication rate following bilateral thoracentesis is low and the rate of pneumothorax subsequent to bilateral thoracentesis is comparable to unilateral thoracentesis. PMID:23219348

  4. CLINICAL ANALYSIS AND SURGICAL RESULTS IN SARCOMA

    Directory of Open Access Journals (Sweden)

    Basavaraju

    2016-02-01

    Full Text Available INTRODUCTION Sarcomas are quite rare with only 15,000 new cases per year in the United States. Sarcomas therefore represent about one percent of the 1.5 million new cancer diagnoses in that country each year. Sarcoma can be defined as cancer whose cells originate from the cells of mesenchymal origin. The bones, cartilages, muscles are a few examples to be mentioned. This is in contrast to a malignant tumour originating from epithelial cells, which are termed carcinoma. AIMS AND OBJECTIVES 1. To clinically analyze the sarcomas. 2. To analyze the surgical outcome of this disease. The survival of the patient depends on the extent of metastasis and the primary identification. The study forms a base for further studies. So atleast it could be diagnosed earlier and treated to the full extent.

  5. Acute Pulmonary Artery Obstruction as the Primary Manifestation of a Rapidly Growing Intimal Sarcoma in a 54-Year-Old Patient.

    Science.gov (United States)

    Westhofen, Sumi; Kugler, Christian; Reichenspurner, Hermann; Deuse, Tobias

    2016-12-01

    Pulmonary artery sarcoma is a rare malignant neoplasm that is often misdiagnosed and most often only recognized postmortem during the autopsy. We present the case of a male patient with a rapidly progressive pulmonary tumor who underwent urgent pneumonectomy for increasing symptoms of chest pain and septic clinical picture. Histological analysis revealed the diagnosis of a pulmonary artery sarcoma. Despite an R1-resection and adjuvant chemotherapy, the patient is in good clinical health and free of tumor relapse 1 year after the surgery.

  6. Regional prevalence and transmission route of Kaposi's sarcoma-associated herpes virus in Zhejiang, China

    Institute of Scientific and Technical Information of China (English)

    JU Hong-zhen; ZHU Biao; WANG Ying-jie; SHENG Zi-ke; SHENG Ji-fang

    2012-01-01

    Background The infection of Kaposi's sarcoma-associated herpes virus (KSHV) is most likely the cause of clinical Kaposi's sarcoma,primary effusion lymphoma,and multi-center Castleman's disease.KSHV infection has very limited epidemiological survey data in China,and its definite mode of transmission remains controversial.This study aimed to determine the infection status and the main transmission route of KSHV in Chinese population.Methods An enzyme-linked immunosorbent assay (ELISA) utilizing KSHV ORF65 recombinant protein was employed to analyze the antibody response to KSHV ORF65 in sera from 122 healthy physical examination people,107intravenous drug users,135 non-intravenous drug users,211 hepatitis B (HBV) patients infected via blood transmission,107 kidney transplant recipients,and 72 female sex workers in Zhejiang Province in Southeast China.Results KSHV infection occurred relatively common (13.1%) in healthy population in Zhejiang,China.Infection rate was 16.7% in female sex workers,but significantly elevated in intravenous drug addicts (58.9%),blood-transmitted HBV patients (28.0%) and kidney transplant patients (41.1%).Conclusion Blood borne transmission of KSHV is probably the main route of infection in Zhejiang Province.

  7. Characterization of Kaposi's Sarcoma-Associated Herpesvirus-Related Lymphomas by DNA Microarray Analysis

    Directory of Open Access Journals (Sweden)

    Keiji Ueda

    2011-01-01

    Full Text Available Among herpesviruses, γ-herpesviruses are supposed to have typical oncogenic activities. Two human γ-herpesviruses, Epstein-Barr virus (EBV and Kaposi's sarcoma-associated herpesvirus (KSHV, are putative etiologic agents for Burkitt lymphoma, nasopharyngeal carcinoma, and some cases of gastric cancers, and Kaposi's sarcoma, multicentric Castleman's disease, and primary effusion lymphoma (PEL especially in AIDS setting for the latter case, respectively. Since such two viruses mentioned above are highly species specific, it has been quite difficult to prove their oncogenic activities in animal models. Nevertheless, the viral oncogenesis is epidemiologically and/or in vitro experimentally evident. This time, we investigated gene expression profiles of KSHV-oriented lymphoma cell lines, EBV-oriented lymphoma cell lines, and T-cell leukemia cell lines. Both KSHV and EBV cause a B-cell-originated lymphoma, but the gene expression profiles were typically classified. Furthermore, KSHV could govern gene expression profiles, although PELs are usually coinfected with KSHV and EBV.

  8. The HIV protease inhibitor nelfinavir inhibits Kaposi's sarcoma-associated herpesvirus replication in vitro.

    Science.gov (United States)

    Gantt, Soren; Carlsson, Jacquelyn; Ikoma, Minako; Gachelet, Eliora; Gray, Matthew; Geballe, Adam P; Corey, Lawrence; Casper, Corey; Lagunoff, Michael; Vieira, Jeffrey

    2011-06-01

    Kaposi's sarcoma (KS) is the most common HIV-associated cancer worldwide and is associated with high levels of morbidity and mortality in some regions. Antiretroviral (ARV) combination regimens have had mixed results for KS progression and resolution. Anecdotal case reports suggest that protease inhibitors (PIs) may have effects against KS that are independent of their effect on HIV infection. As such, we evaluated whether PIs or other ARVs directly inhibit replication of Kaposi's sarcoma-associated herpesvirus (KSHV), the gammaherpesvirus that causes KS. Among a broad panel of ARVs tested, only the PI nelfinavir consistently displayed potent inhibitory activity against KSHV in vitro as demonstrated by an efficient quantitative assay for infectious KSHV using a recombinant virus, rKSHV.294, which expresses the secreted alkaline phosphatase. This inhibitory activity of nelfinavir against KSHV replication was confirmed using virus derived from a second primary effusion lymphoma cell line. Nelfinavir was similarly found to inhibit in vitro replication of an alphaherpesvirus (herpes simplex virus) and a betaherpesvirus (human cytomegalovirus). No activity was observed with nelfinavir against vaccinia virus or adenovirus. Nelfinavir may provide unique benefits for the prevention or treatment of HIV-associated KS and potentially other human herpesviruses by direct inhibition of replication.

  9. The Challenges of Detecting Circulating Tumor Cells in Sarcoma

    Science.gov (United States)

    Tellez-Gabriel, Marta; Brown, Hannah K.; Young, Robin; Heymann, Marie-Françoise; Heymann, Dominique

    2016-01-01

    Sarcomas are a heterogeneous group of malignant neoplasms of mesenchymal origin, many of which have a propensity to develop distant metastases. Cancer cells that have escaped from the primary tumor are able to invade into surrounding tissues, to intravasate into the bloodstream to become circulating tumor cells (CTCs), and are responsible for the generation of distant metastases. Due to the rarity of these tumors and the absence of specific markers expressed by sarcoma tumor cells, the characterization of sarcoma CTCs has to date been relatively limited. Current techniques for isolating sarcoma CTCs are based on size criteria, the identification of circulating cells that express either common mesenchymal markers, sarcoma-specific markers, such as CD99, CD81, or PAX3, and chromosomal translocations found in certain sarcoma subtypes, such as EWS-FLI1 in Ewing’s sarcoma, detection of osteoblast-related genes, or measurement of the activity of specific metabolic enzymes. Further studies are needed to improve the isolation and characterization of sarcoma CTCs, to demonstrate their clinical significance as predictive and/or prognostic biomarkers, and to utilize CTCs as a tool for investigating the metastatic process in sarcoma and to identify novel therapeutic targets. The present review provides a short overview of the most recent literature on CTCs in sarcoma. PMID:27656422

  10. [Sarcoid pleural effusion].

    Science.gov (United States)

    Rodríguez-Núñez, Nuria; Rábade, Carlos; Valdés, Luis

    2014-12-09

    Pleural effusion (PE) is a very uncommon manifestation of sarcoidosis. It is equally observed in men and women, can appear at any age and in all radiologic stages, though it is more common in stages i and ii. Effusions have usually a mild or medium size and mainly involve the right side. Various mechanisms can be implicated. PE will be a serous exudate if there is an increase in the capillary permeability due to direct involvement of the pleural membrane, a chylothorax if mediastinum lymph nodes compress the thoracic duct and/or the lymphatic drainage from the pleural cavity, an hemothorax if granuloma compress or invade pleural small vessels or capillaries, and even a transudate if there is compression of the inferior vena cava, atelectasis due to complete bronchial obstruction or when the resolution of the PE is incomplete with chronic thickening of visceral pleura (trapped lung). It manifests biochemically as a pauci-cellular exudate with a predominance of lymphocytes, though there can be a preponderance of eosinophils or neutrophils. Protein concentrations are usually proportionately higher than lactate dehidrogenase, adenosine deaminase is normally low and it is possible to find increased levels of CA-125 in women. The tuberculin test is negative and pleural or lung biopsies yield the diagnosis by confirming the presence of non-caseating granulomata. These PE can have a favorable self-limited outcome, even though in most cases treatment with corticosteroids is needed, while surgery is required in a few cases. Copyright © 2013 Elsevier España, S.L.U. All rights reserved.

  11. Intimal sarcoma of the pulmonary artery--diagnostic challenge.

    Science.gov (United States)

    Fukuda, Wakako; Morohashi, Satoko; Fukuda, Ikuo

    2011-08-01

    Pulmonary artery intimal sarcoma is a rare tumour and the diagnosis is often delayed. We report the case of a woman with a primary pulmonary artery intimal sarcoma who presented with massive pulmonary embolism. The definitive diagnosis was elucidated after the patient's death by autopsy specimen. We discuss the diagnosis and lessons learned from this case.

  12. Amaurosis fugax due to pleomorphic sarcoma in the left atrium

    Directory of Open Access Journals (Sweden)

    Sheila Pabon

    2016-12-01

    Conclusions and importance: The authors postulate emboli from the left atrial sarcoma entered systemic circulation and subsequently caused brief episodes of transient occlusion to retinal, ophthalmic and/or ciliary arteries leading to momentary retinal hypoxia. We believe this is a novel finding, previously unreported in the literature, of transient embolic occlusion without permanent visual sequelae due to a malignant primary cardiac pleomorphic sarcoma.

  13. The Danish Sarcoma Database

    DEFF Research Database (Denmark)

    Jørgensen, Peter Holmberg; Lausten, Gunnar Schwarz; Pedersen, Alma B

    2016-01-01

    AIM: The aim of the database is to gather information about sarcomas treated in Denmark in order to continuously monitor and improve the quality of sarcoma treatment in a local, a national, and an international perspective. STUDY POPULATION: Patients in Denmark diagnosed with a sarcoma, both...... skeletal and ekstraskeletal, are to be registered since 2009. MAIN VARIABLES: The database contains information about appearance of symptoms; date of receiving referral to a sarcoma center; date of first visit; whether surgery has been performed elsewhere before referral, diagnosis, and treatment; tumor...... of Diseases - tenth edition codes and TNM Classification of Malignant Tumours, and date of death (after yearly coupling to the Danish Civil Registration System). Data quality and completeness are currently secured. CONCLUSION: The Danish Sarcoma Database is population based and includes sarcomas occurring...

  14. Thoracoscopy in undiagnosed pleural effusions

    African Journals Online (AJOL)

    diagnostic thoracoscopy for pleural effusions of unknown origin. Design. Retrospective review of consecutive patients referred for diagnostic thoracoscopy ... symptoms such as fever and sweats is highly associated with a final diagnosis of ...

  15. Pericardial effusion in celiac disease

    Directory of Open Access Journals (Sweden)

    Farzaneh Ashrafi

    2014-01-01

    Full Text Available Celiac disease is an autoimmune disorder that affected 1% of all population in United State. Classic manifestations of disease consist of early childhood diarrhea, malabsorption, steatorrhea and growth retardation but disease can affects adult at any age. In adult anemia is a more frequent finding. This patient was a 40-year-old lady with progressive fatigue and lower extremities pitting edema. Iron deficiency anemia and celiac disease were diagnosed on the basis of low serum ferritin, elevated serum level of IgA endomysial and tissue transglutaminase anti-bodies and histologic findings in small bowel biopsies. Pericardial effusion in her evaluation was detected incidentally. Asymptomatic pericardial effusion in this patient was only detectable with imaging. After starting of gluten free diet and iron supplement fatigue, peripheral edema and pericardial effusion on echocardiography decreased. It should be noted that asymptomatic pericardial effusion may be seen in adults with celiac disease.

  16. Minoxidil-associated exudative pleural effusion.

    Science.gov (United States)

    Siddiqui, Atif; Ansari, Mohammed; Shakil, Jawairia; Chemitiganti, Rama

    2010-05-01

    Recurrent pleural effusions are associated with significant morbidity and mortality. Drug-related reactions causing pleural effusions are not common, but their identification can potentially improve patient outcome. Minoxidil has been implicated in pleuropericardial effusions in patients with chronic kidney disease. The exact mechanism by which pleural effusion occurs is still unclear. We report a case of isolated exudative pleural effusion associated with minoxidil in a patient without underlying kidney disease that almost completely resolved after the drug was discontinued.

  17. Uma nova abordagem para as endopróteses parciais de joelho em sarcomas primários ósseos A new approach to partial knee endoprosthesis in primary bone sarcomas

    Directory of Open Access Journals (Sweden)

    Valter Penna

    2009-02-01

    Full Text Available OBJETIVO: As endopróteses parciais de joelho para as ressecções em sarcomas ósseos demonstram serem boa solução para o tratamento de pacientes com imaturidade esquelética. O objetivo deste estudo é avaliar o escore funcional, as vantagens, as desvantagens e indicações para esta técnica cirúrgica em quatorze pacientes em um protocolo brasileiro de osteossarcoma e sarcoma de Ewing. MÉTODOS: Análise retrospectiva realizada para identificar a evolução funcional e as possíveis complicações do procedimento. 14 pacientes com idade entre 10 e 22 anos avaliados funcionalmente pelos critérios de Enneking/ISOLS (International Society of Limb Salvage, sendo todos operados na mesma Instituição e pelo mesmo cirurgião. Foram utilizadas endopróteses parciais das extremidades distal do fêmur e proximal da tíbia com reconstrução ligamentar. ReSULTADOS: A análise do escore funcional de Enneking/ISOLS demonstrou 78,6 % de excelentes resultados e 21,4% de bons. Dos 14 pacientes, todos portadores de tumores primitivos ósseos em protocolo de quimioterapia, nove não apresentaram nenhum tipo de complicação e cinco indivíduos evoluíram com complicações relacionadas ao procedimento, sendo que houve relação estatística positiva entre os maus resultados e a presença de complicações (p=0,027. CONCLUSÃO: As endopróteses parciais de joelhos são menos prejudiciais ao estoque ósseo de pacientes com esqueleto imaturo. As críticas sobre os maus resultados funcionais estão sendo suplantadas pelas novas técnicas de reconstrução, corretos protocolos de reabilitação, qualidade e tecnologia dos implantes, e o aumento da curva de aprendizado. Essa opção de tratamento per-mite a preservação do estoque ósseo e a possibilidade de revisão da artroplastia não convencional de modo menos agressivo.OBJECTIVE: Partial knee endoprosthesis to bone sarcomas resections seems to be a good solution to treat this immature skeletal patients

  18. Primary synovial sarcoma of the heart: a cytogenetic and molecular genetic analysis combining RT-PCR and COBRA-FISH of a case with a complex karyotype.

    Science.gov (United States)

    Hazelbag, Hans Martin; Szuhai, Károly; Tanke, Hans J; Rosenberg, Carla; Hogendoorn, Pancras C W

    2004-11-01

    Synovial sarcomas usually occur in the soft tissues of the extremities of adolescents and middle-aged patients, in the vicinity of large joints. We present a patient with a synovial sarcoma of the left atrium and ventricle, which is an extremely rare location. Diagnosis was confirmed by reverse transcriptase polymerase chain reaction (RT-PCR), showing the t(X;18) fusion transcript. With a multicolor COmbined Binary RAtio labeling Fluorescence In Situ Hybridization (COBRA-FISH) technique, a complex karyotype evolved with identification of derivative chromosomes with multiplex rearrangements. This underscores the importance of molecular analysis of spindle cell tumors in unusual locations. Moreover, it shows that the presumed diagnostic translocation t(X;18) can be embedded in a sequence of other chromosomal rearrangements of which the function is as yet unknown.

  19. Stages of Ewing Sarcoma

    Science.gov (United States)

    ... tumor that forms from a certain kind of cell in bone or soft tissue . Ewing sarcoma may be found in the bones of the legs, arms, feet, hands, chest , pelvis , spine , or skull . Ewing sarcoma also may be found in the soft tissue of the trunk, arms, legs, head and neck, abdominal cavity , or ...

  20. Extraosseous Osteogenic Sarcoma

    Directory of Open Access Journals (Sweden)

    Shao-Ying Lin

    2005-11-01

    Full Text Available Extraosseous osteogenic sarcoma is a very rare malignant neoplasm. Out of the more than 400 cases of soft tissue sarcomas on file in our hospital, only 2 were extraosseous osteogenic sarcomas. Both were situated in the thigh. The first case was initially diagnosed as a hematoma and treated by marginal excision. The diagnosis of high-grade osteosarcoma primarily arising in soft tissue was made from histopathologic examination. Radiotherapy of 60 Gy in 30 fractions was given postoperatively. The second patient, primarily diagnosed as having a soft tissue sarcoma, was treated by wide excision. The final pathologic report was high-grade extraosseous osteogenic sarcoma. Adjuvant chemotherapy was given postoperatively. Both patients are alive without local recurrence and distant metastasis at postoperative 90-month and 107-month follow-up, respectively.

  1. Trial of Dasatinib in Advanced Sarcomas

    Science.gov (United States)

    2016-10-12

    Rhabdomyosarcoma; Malignant Peripheral Nerve Sheath Tumors; Chondrosarcoma; Sarcoma, Ewing's; Sarcoma, Alveolar Soft Part; Chordoma; Epithelioid Sarcoma; Giant Cell Tumor of Bone; Hemangiopericytoma; Gastrointestinal Stromal Tumor (GIST)

  2. Kinetics of Kaposi's sarcoma-associated herpesvirus gene expression.

    Science.gov (United States)

    Sun, R; Lin, S F; Staskus, K; Gradoville, L; Grogan, E; Haase, A; Miller, G

    1999-03-01

    Herpesvirus gene expression can be classified into four distinct kinetic stages: latent, immediate early, early, and late. Here we characterize the kinetic class of a group of 16 Kaposi's sarcoma-associated herpesvirus (KSHV)/human herpesvirus 8 genes in a cultured primary effusion cell line and examine the expression of a subset of these genes in KS biopsies. Expression of two latent genes, LANA and vFLIP, was constitutive and was not induced by chemicals that induce the lytic cycle in primary effusion lymphoma (PEL) cell lines. An immediate-early gene, Rta (open reading frame 50 [ORF50]), was induced within 4 h of the addition of n-butyrate, and its 3.6-kb mRNA was resistant to inhibition by cycloheximide. Early genes, including K3 and K5 that are homologues of the "immediate-early" gene of bovine herpesvirus 4, K8 that is a positional homologue of Epstein-Barr virus BZLF1, vMIP II, vIL-6, and polyadenylated nuclear (PAN) RNA, appeared 8 to 13 h after chemical induction. A second group of early genes that were slightly delayed in their appearance included viral DHFR, thymidylate synthase, vMIP I, G protein-coupled receptor, K12, vBcl2, and a lytic transcript that overlapped LANA. The transcript of sVCA (ORF65), a late gene whose expression was abolished by Phosphonoacetic acid, an inhibitor of KSHV DNA replication, did not appear until 30 h after induction. Single-cell assays indicated that the induction of lytic cycle transcripts resulted from the recruitment of additional cells into the lytic cycle. In situ hybridization of KS biopsies showed that about 3% of spindle-shaped tumor cells expressed Rta, ORF K8, vIL-6, vMIP I, vBcl-2, PAN RNA, and sVCA. Our study shows that several KSHV-encoded homologues of cellular cytokines, chemokines, and antiapoptotic factors are expressed during the viral lytic cycle in PEL cell lines and in KS biopsies. The lytic cycle of KSHV, probably under the initial control of the KSHV/Rta gene, may directly contribute to tumor

  3. Diagnostic yield of cytopathology in evaluating pericardial effusions: Clinicopathologic analysis of 419 specimens.

    Science.gov (United States)

    Saab, Jad; Hoda, Rana S; Narula, Navneet; Hoda, Syed A; Geraghty, Brian E; Nasar, Abu; Alperstein, Susan A; Port, Jeffrey L; Giorgadze, Tamar

    2017-02-01

    Pericardial effusions can cause considerable morbidity and potentially may lead to mortality. Malignant pericardial effusions are uncommon, and data on malignancies encountered in pericardial effusion cytology specimens are limited. Relevant records of all pericardial effusions from January 2008 to September 2014 were examined and compared with pericardial biopsy results when performed. Discrepant cases were reviewed to determine the cause of the disagreement. In total, 419 pericardial effusion specimens obtained from 364 patients were examined. Cytologic diagnostic categories included: negative for malignancy (332 specimens; 79%), equivocal (25 specimens; 6%), and positive (62 specimens from 51 patients; 15%). Forty-seven patients who had positive effusions were known to have malignancy. The most common primary malignancies were breast (39.3%) and lung (39.3%) cancers in women and lung cancer (47.4%) in men. A concurrent pericardial biopsy was performed in 46% of patients. Excluding equivocal cytologic diagnoses, cytology and biopsy were concordant in 153 of 173 paired samples (88.4%). The sensitivity of cytology in diagnosing malignancy was 92.1% compared with 55.3% for pericardial biopsy. Cytologic examination has significant diagnostic utility in the evaluation of pericardial effusions and exhibits a lower false-negative rate compared with pericardial biopsy. Submission of pericardial biopsy alongside effusion cytology is associated with increased sensitivity for detecting malignancy and may be especially useful in the setting of low-volume pericardial effusion. Cancer Cytopathol 2017;125:128-137. © 2016 American Cancer Society. © 2016 American Cancer Society.

  4. The role of liquid-based cytology and ancillary techniques in pleural and pericardic effusions: an institutional experience.

    Science.gov (United States)

    Rossi, Esther Diana; Bizzarro, Tommaso; Schmitt, Fernando; Longatto-Filho, Adhemar

    2015-04-01

    Fine-needle aspiration cytology (FNAC) of serous membrane effusions may fulfil a challenging role in the diagnostic analysis of both primary and metastatic disease. From this perspective, liquid-based cytology (LBC) represents a feasible and reliable method for empowering the performance of ancillary techniques (ie, immunocytochemistry and molecular testing) with high diagnostic accuracy. In total, 3171 LBC pleural and pericardic effusions were appraised between January 2000 and December 2013. They were classified as negative for malignancy (NM), suspicious for malignancy (SM), or positive for malignancy (PM). The cytologic diagnoses included 2721 NM effusions (2505 pleural and 216 pericardic), 104 SM effusions (93 pleural and 11 pericardic), and 346 PM effusions (321 pleural and 25 pericardic). The malignant pleural series included 76 unknown malignancies (36 SM and 40 PM effusions), 174 metastatic lesions (85 SM and 89 PM effusions), 14 lymphomas (3 SM and 11 PM effusions), 16 mesotheliomas (5 SM and 11 SM effusions), and 3 myelomas (all SM effusions). The malignant pericardic category included 20 unknown malignancies (5 SM and 15 PM effusions), 15 metastatic lesions (1 SM and 14 PM effusions), and 1 lymphoma (1 PM effusion). There were 411 conclusive immunocytochemical analyses and 47 molecular analyses, and the authors documented 88% sensitivity, 100% specificity, 98% diagnostic accuracy, 98% negative predictive value, and 100% positive predictive value for FNAC. FNAC represents a primary diagnostic tool for effusions and a reliable approach with which to determine the correct follow-up. Furthermore, LBC is useful for ancillary techniques, such as immunocytochemistry and molecular analysis, with feasible diagnostic and predictive utility. © 2015 American Cancer Society.

  5. Nuclear Localization and Cleavage of STAT6 Is Induced by Kaposi’s Sarcoma-Associated Herpesvirus for Viral Latency

    Science.gov (United States)

    Zhang, Liming; Li, Yuhong; Feng, Yanling; Xu, Jianqing; Wang, Bin; Yuan, Zhenghong; Robertson, Erle S.; Cai, Qiliang

    2017-01-01

    Emerging evidence implies that STAT6 plays an important role in both the adaptive and innate immune responses to virus infection. Kaposi’s sarcoma-associated herpesvirus (KSHV) is an oncogenic γ-herpesvirus agent associated with several human malignancies, including Kaposi’s sarcoma (KS) and primary effusion lymphomas (PELs). Previously, we demonstrated that KSHV blocks IL-4-induced STAT6 phosphorylation and retains a basal IL-13/STAT6 constitutive activation for cell survival and proliferation. However, the mechanism by which KSHV regulates STAT6 remains largely unknown. Here, we found that KSHV-encoded LANA interacts with STAT6 and promotes nuclear localization of STAT6 independent of the tyrosine 641-phosphorylation state. Moreover, nuclear localization of STAT6 is also dramatically increased in KS tissue. The latent antigen LANA induces serine protease-mediated cleavage of STAT6 in the nucleus, where the cleaved STAT6 lacking transactivation domain functions as a dominant-negative regulator to repress transcription of Replication and Transcription Activator (RTA) and in turn shut off viral lytic replication. Blockade of STAT6 by small interference RNA dramatically enhances expression of RTA, and in turn reduces KSHV-infected endothelial cell growth and colony formation. Taken together, these results suggest that nuclear localization and cleavage of STAT6 is important for modulating the viral latency and pathogenesis of KSHV. PMID:28099521

  6. Kaposi's sarcoma-associated herpesvirus-encoded LANA associates with glucocorticoid receptor and enhances its transcriptional activities

    Energy Technology Data Exchange (ETDEWEB)

    Togi, Sumihito; Nakasuji, Misa; Muromoto, Ryuta; Ikeda, Osamu; Okabe, Kanako; Kitai, Yuichi; Kon, Shigeyuki [Department of Immunology, Graduate School of Pharmaceutical Sciences Hokkaido University, Sapporo 060-0812 (Japan); Oritani, Kenji [Department of Hematology and Oncology, Graduate School of Medicine, Osaka University, 2-2 Yamada-oka, Suita, Osaka 565-0871 (Japan); Matsuda, Tadashi, E-mail: tmatsuda@pharm.hokudai.ac.jp [Department of Immunology, Graduate School of Pharmaceutical Sciences Hokkaido University, Sapporo 060-0812 (Japan)

    2015-07-31

    Kaposi's sarcoma-associated herpesvirus (KSHV)-encoded latency-associated nuclear antigen (LANA), which interacts with cellular proteins, plays a central role in modification of viral and/or cellular gene expression. Here, we show that LANA associates with glucocorticoid receptor (GR), and that LANA enhances the transcriptional activity of GR. Co-immunoprecipitation revealed a physical interaction between LANA and GR in transiently transfected 293T and HeLa cells. In human B-lymphoma cells, LANA overexpression enhanced GR activity and cell growth suppression following glucocorticoid stimulation. Furthermore, confocal microscopy showed that activated GR was bound to LANA and accumulated in the nucleus, leading to an increase in binding of activated GR to the glucocorticoid response element of target genes. Taken together, KSHV-derived LANA acts as a transcriptional co-activator of GR. Our results might suggest a careful use of glucocorticoids in the treatment of patients with KSHV-related malignancies such as Kaposi's sarcoma, primary effusion lymphoma, and multicentric Castleman disease. - Highlights: • KSHV-LANA enhances the transcriptional activity of GR in 293T and HeLa cells. • KSHV-LANA physically associates with GR. • KSHV-LANA enhances GR activation and cell growth suppression in human B-lymphocytes. • KSHV-LANA influences the nuclear retention and DNA binding activity of GR.

  7. Kaposi΄s sarcoma-associated herpesvirus ORF36 protein induces chromosome condensation and phosphorylation of histone H3.

    Science.gov (United States)

    Kim, Sunmi; Cha, Seho; Jang, Jun Hyeong; Kim, Yejin; Seo, Taegun

    2013-01-01

    Kaposi΄s sarcoma-associated herpesvirus (KSHV) has been known as an agent causing Kaposi΄s sarcoma, primary effusion lymphoma, and multicentric Castleman΄s disease. In the lytic phase of the virus cycle, various viral genes are expressed, which causes host cell dysregulation. Among the lytic genes, viral protein kinase (vPK) encoded by ORF36 is a member of serine/threonine protein kinase (CHPK) family, which is involved in viral gene expression, viral DNA replication and encapsidation, and nuclear egress of virions. Recent studies have shown that the BGLF4 protein of Epstein-Barr virus (EBV), a member of the CHPK family, alters the host cell chromatin structure through phosphorylation of its key regulators. The role of KSHV ORF36 in cellular mitotic events, however, is not yet understood. In the current study, we showed that KSHV ORF36 induced chromosome condensation and phosphorylation of histone H3 on Ser 10, which are known as cellular mitosis markers. These processes have occurred in a kinase activity-dependent manner.

  8. Modulation of immune system by Kaposi’s sarcoma-associated herpesvirus:Lessons from viral evasion strategies

    Directory of Open Access Journals (Sweden)

    Hye-Ra eLee

    2012-03-01

    Full Text Available Kaposi’s sarcoma-associated herpesvirus (KSHV, a member of the herpesvirus family, has evolved to establish a long-term, latent infection of cells such that while they carry the viral genome gene expression is highly restricted. Latency is a state of cryptic viral infection associated with genomic persistence in their host and this hallmark of KSHV infection leads to several clinical-epidemiological diseases such as Kaposi’s sarcoma (KS, a plasmablastic variant of multicentric Castelman’s disease (MCD, and primary effusion lymphoma (PEL upon immune suppression of infected hosts. In order to sustain an efficient life-long persistency as well as their life cycle, KSHV dedicates a large portion of its genome to encode immunomodulatory proteins that antagonize its host’s immune system. In this review, we will describe our current knowledge of the immune evasion strategies employed by KSHV at distinct stages of its viral life cycle to control the host’s immune system.

  9. The Danish Sarcoma Database

    Directory of Open Access Journals (Sweden)

    Jorgensen PH

    2016-10-01

    Full Text Available Peter Holmberg Jørgensen,1 Gunnar Schwarz Lausten,2 Alma B Pedersen3 1Tumor Section, Department of Orthopedic Surgery, Aarhus University Hospital, Aarhus, 2Tumor Section, Department of Orthopedic Surgery, Rigshospitalet, Copenhagen, 3Department of Clinical Epidemiology, Aarhus University Hospital, Aarhus, Denmark Aim: The aim of the database is to gather information about sarcomas treated in Denmark in order to continuously monitor and improve the quality of sarcoma treatment in a local, a national, and an international perspective. Study population: Patients in Denmark diagnosed with a sarcoma, both skeletal and ekstraskeletal, are to be registered since 2009. Main variables: The database contains information about appearance of symptoms; date of receiving referral to a sarcoma center; date of first visit; whether surgery has been performed elsewhere before referral, diagnosis, and treatment; tumor characteristics such as location, size, malignancy grade, and growth pattern; details on treatment (kind of surgery, amount of radiation therapy, type and duration of chemotherapy; complications of treatment; local recurrence and metastases; and comorbidity. In addition, several quality indicators are registered in order to measure the quality of care provided by the hospitals and make comparisons between hospitals and with international standards. Descriptive data: Demographic patient-specific data such as age, sex, region of living, comorbidity, World Health Organization's International Classification of Diseases – tenth edition codes and TNM Classification of Malignant Tumours, and date of death (after yearly coupling to the Danish Civil Registration System. Data quality and completeness are currently secured. Conclusion: The Danish Sarcoma Database is population based and includes sarcomas occurring in Denmark since 2009. It is a valuable tool for monitoring sarcoma incidence and quality of treatment and its improvement, postoperative

  10. Thoracoscopic evaluation of 129 cases having undiagnosed exudative pleural effusions

    Directory of Open Access Journals (Sweden)

    Chetan Basavaraj Patil

    2016-01-01

    Full Text Available Background: Medical thoracoscopy is a minimally invasive procedure used in diagnostic and therapeutic applications for pleural diseases. In this study, we describe our experience in the outcome and analysis of thoracoscopy in undiagnosed pleural effusion presenting to our center. Materials and Methods: This is a prospective study conducted over last 2 years. We performed thoracoscopy in 129 cases of undiagnosed exudative pleural effusions using rigid thoracoscope. Clinical, radiological, cyto and histopathological data of the patients were collected prospectively and analyzed. Results: The overall diagnostic yield of thoracoscopic pleural biopsy was 110/129 (85.2% in patients with undiagnosed pleural effusion, and 19/129 (14.8% patients remained unexplained. Histopathological diagnosis confirmed malignancy in 66.4% patients (both primary and metastatic pleural carcinoma, tuberculosis in 28.2%, others including parapneumonic effusion in 4 cases followed by multiple myeloma, lupus pleuritis, and pulmonary langerhans cell histiocytosis in one case each. Procedure-related mortality was nil. Minor complications related to the procedure include hemorrhage, subcutaneous emphysema, etc. Conclusion: Thoracoscopy is relatively a safe and well-tolerated procedure with high diagnostic accuracy in undiagnosed pleural effusions, decreasing the need of formal diagnostic thoracotomy. Every chest physician must, therefore, consider this procedure to decrease the time lag in achieving the final diagnosis and to initiate the treatment as early as possible.

  11. Nonchylous idiopathic pleural effusion in the newborn

    Directory of Open Access Journals (Sweden)

    Geeta Gathwala

    2011-01-01

    Full Text Available Congenital isolated pleural effusion is a rare cause of respiratory distress in neonates. It is usually chylous. Herein, we report a rare case of nonchylous congenital idiopathic pleural effusion.

  12. Pleuropericardial effusion associated with minoxidil administration

    Science.gov (United States)

    Webb, D. B.; Whale, R. J.

    1982-01-01

    A patient on minoxidil developed pericardial and pleural effusions with a high protein content. This finding is not compatible with the view that such effusions in patients taking minoxidil are transudates. PMID:7111124

  13. What Are the Key Statistics about Kaposi Sarcoma?

    Science.gov (United States)

    ... Kaposi Sarcoma What Are the Key Statistics About Kaposi Sarcoma? Before the AIDS epidemic, Kaposi sarcoma (KS) was ... in Kaposi Sarcoma Research and Treatment? More In Kaposi Sarcoma About Kaposi Sarcoma Causes, Risk Factors, and Prevention ...

  14. What's New in Kaposi Sarcoma Research and Treatment?

    Science.gov (United States)

    ... Kaposi Sarcoma About Kaposi Sarcoma What’s New in Kaposi Sarcoma Research and Treatment? A great deal of research ... in Kaposi Sarcoma Research and Treatment? More In Kaposi Sarcoma About Kaposi Sarcoma Causes, Risk Factors, and Prevention ...

  15. Pleural effusion following ovarian hyperstimulation.

    Science.gov (United States)

    Junqueira, Jader Joel Machado; Bammann, Ricardo Helbert; Terra, Ricardo Mingarini; Castro, Ana Cristina P; Ishy, Augusto; Fernandez, Angelo

    2012-01-01

    Ovarian hyperstimulation syndrome (OHSS) is an iatrogenic complication that occurs in the luteal phase of an induced hormonal cycle. In most cases, the symptoms are self-limited and spontaneous regression occurs. However, severe cases are typically accompanied by acute respiratory distress. The objective of the present study was to describe the clinical presentation, treatment, and outcome of pleural effusion associated with OHSS in three patients undergoing in vitro fertilization. The patients ranged in age from 27 to 33 years. The onset of symptomatic pleural effusion (bilateral in all cases) occurred, on average, 43 days (range, 27-60 days) after initiation of hormone therapy for ovulation induction. All three patients required hospitalization for massive fluid resuscitation, and two required noninvasive mechanical ventilation. Although all three patients initially underwent thoracentesis, early recurrence of symptoms and pleural effusion prompted the use of drainage with a pigtail catheter. Despite the high output from the pleural drain (mean, 1,000 mL/day in the first week) and prolonged drainage (for 9-22 days), the outcomes were excellent: all three patients were discharged from hospital. Although pleural effusion secondary to OHSS is probably underdiagnosed, the associated morbidity should not be underestimated, especially because it affects potentially pregnant patients. In this study, early diagnosis and appropriate supportive measures yielded favorable results, limiting the surgical approach to adequate pleural drainage.

  16. Lymphoproliferative disorder in pleural effusion in a subject with past asbestos exposure

    Directory of Open Access Journals (Sweden)

    Naofumi Hara

    2015-01-01

    Full Text Available Primary effusion lymphoma (PEL is a subtype of non-Hodgkin lymphoma that presents as serous effusions without detectable masses or organomegaly. Here we report a case of PEL-like lymphoma in a patient with past asbestos exposure. A 65-year-old man was referred to our hospital due to dyspnea upon exertion. He had been exposed to asbestos for three years in the construction industry. Chest X-ray and CT images demonstrated left pleural effusion. Cytological analysis of the pleural effusion revealed large atypical lymphocytes with distinct nuclear bodies and high nucleus-to-cytoplasm ratio. Immunohistochemical analyses showed that the cells were CD20+, CD3−, CD5−, and CD10−. These findings led to a diagnosis of diffuse large B-cell lymphoma. PEL or PEL-like lymphoma should be considered a potential cause of pleural effusion in subjects with past asbestos exposure.

  17. Pulmonary involvement in Kaposi sarcoma: correlation between imaging and pathology.

    Science.gov (United States)

    Gasparetto, Taisa Davaus; Marchiori, Edson; Lourenço, Sílvia; Zanetti, Gláucia; Vianna, Alberto Domingues; Santos, Alair A S M D; Nobre, Luiz Felipe

    2009-07-14

    Kaposi sarcoma is a low-grade mesenchymal tumor involving blood and lymphatic vessels. There are four variants of this disease, each presenting a different clinical manifestation: classic or sporadic, African or endemic, organ transplant-related or iatrogenic, and AIDS-related or epidemic. Kaposi sarcoma is the most common tumor among patients with HIV infection, occurring predominantly in homosexual or bisexual men. The pulmonary involvement in Kaposi sarcoma occurs commonly in critically immunosupressed patients who commonly have had preceding mucocutaneous or digestive involvement.The etiology of Kaposi sarcoma is not precisely established; genetic, hormonal, and immune factors, as well as infectious agents, have all been implicated. There is evidence from epidemiologic, serologic, and molecular studies that Kaposi sarcoma is associated with human herpes virus type 8 infection. The disease starts as a reactive polyclonal angioproliferative response towards this virus, in which polyclonal cells change to form oligoclonal cell populations that expand and undergo malignant transformation.The diagnosis of pulmonary involvement in Kaposi sarcoma usually can be made by a combination of clinical, radiographic, and laboratory findings, together with the results of bronchoscopy and transbronchial biopsy. Chest high-resolution computed tomography scans commonly reveal peribronchovascular and interlobular septal thickening, bilateral and symmetric ill-defined nodules in a peribronchovascular distribution, fissural nodularity, mediastinal adenopathies, and pleural effusions. Correlation between the high-resolution computed tomography findings and the pathology revealed by histopathological analysis demonstrate that the areas of central peribronchovascular infiltration represent tumor growth involving the bronchovascular bundles, with nodules corresponding to proliferations of neoplastic cells into the pulmonary parenchyma. The interlobular septal thickening may represent

  18. Pulmonary involvement in Kaposi sarcoma: correlation between imaging and pathology

    Directory of Open Access Journals (Sweden)

    Vianna Alberto

    2009-07-01

    Full Text Available Abstract Kaposi sarcoma is a low-grade mesenchymal tumor involving blood and lymphatic vessels. There are four variants of this disease, each presenting a different clinical manifestation: classic or sporadic, African or endemic, organ transplant-related or iatrogenic, and AIDS-related or epidemic. Kaposi sarcoma is the most common tumor among patients with HIV infection, occurring predominantly in homosexual or bisexual men. The pulmonary involvement in Kaposi sarcoma occurs commonly in critically immunosupressed patients who commonly have had preceding mucocutaneous or digestive involvement. The etiology of Kaposi sarcoma is not precisely established; genetic, hormonal, and immune factors, as well as infectious agents, have all been implicated. There is evidence from epidemiologic, serologic, and molecular studies that Kaposi sarcoma is associated with human herpes virus type 8 infection. The disease starts as a reactive polyclonal angioproliferative response towards this virus, in which polyclonal cells change to form oligoclonal cell populations that expand and undergo malignant transformation. The diagnosis of pulmonary involvement in Kaposi sarcoma usually can be made by a combination of clinical, radiographic, and laboratory findings, together with the results of bronchoscopy and transbronchial biopsy. Chest high-resolution computed tomography scans commonly reveal peribronchovascular and interlobular septal thickening, bilateral and symmetric ill-defined nodules in a peribronchovascular distribution, fissural nodularity, mediastinal adenopathies, and pleural effusions. Correlation between the high-resolution computed tomography findings and the pathology revealed by histopathological analysis demonstrate that the areas of central peribronchovascular infiltration represent tumor growth involving the bronchovascular bundles, with nodules corresponding to proliferations of neoplastic cells into the pulmonary parenchyma. The interlobular

  19. Sarcoma derived from cultured mesenchymal stem cells.

    Science.gov (United States)

    Tolar, Jakub; Nauta, Alma J; Osborn, Mark J; Panoskaltsis Mortari, Angela; McElmurry, Ron T; Bell, Scott; Xia, Lily; Zhou, Ning; Riddle, Megan; Schroeder, Tania M; Westendorf, Jennifer J; McIvor, R Scott; Hogendoorn, Pancras C W; Szuhai, Karoly; Oseth, Leann; Hirsch, Betsy; Yant, Stephen R; Kay, Mark A; Peister, Alexandra; Prockop, Darwin J; Fibbe, Willem E; Blazar, Bruce R

    2007-02-01

    To study the biodistribution of MSCs, we labeled adult murine C57BL/6 MSCs with firefly luciferase and DsRed2 fluorescent protein using nonviral Sleeping Beauty transposons and coinfused labeled MSCs with bone marrow into irradiated allogeneic recipients. Using in vivo whole-body imaging, luciferase signals were shown to be increased between weeks 3 and 12. Unexpectedly, some mice with the highest luciferase signals died and all surviving mice developed foci of sarcoma in their lungs. Two mice also developed sarcomas in their extremities. Common cytogenetic abnormalities were identified in tumor cells isolated from different animals. Original MSC cultures not labeled with transposons, as well as independently isolated cultured MSCs, were found to be cytogenetically abnormal. Moreover, primary MSCs derived from the bone marrow of both BALB/c and C57BL/6 mice showed cytogenetic aberrations after several passages in vitro, showing that transformation was not a strain-specific nor rare event. Clonal evolution was observed in vivo, suggesting that the critical transformation event(s) occurred before infusion. Mapping of the transposition insertion sites did not identify an obvious transposon-related genetic abnormality, and p53 was not overexpressed. Infusion of MSC-derived sarcoma cells resulted in malignant lesions in secondary recipients. This new sarcoma cell line, S1, is unique in having a cytogenetic profile similar to human sarcoma and contains bioluminescent and fluorescent genes, making it useful for investigations of cellular biodistribution and tumor response to therapy in vivo. More importantly, our study indicates that sarcoma can evolve from MSC cultures.

  20. Treatment Option Overview (Kaposi Sarcoma)

    Science.gov (United States)

    ... Treatment Childhood Vascular Tumors Treatment Research Kaposi Sarcoma Treatment (PDQ®)–Patient Version General Information About Kaposi Sarcoma ... Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery ) and treatment ...

  1. Factors related to pleural effusion following hepatectomy for primar y liver cancer

    Institute of Scientific and Technical Information of China (English)

    Kai-Jian Chu; Xiao-Ping Yao; Xiao-Hui Fu

    2007-01-01

    BACKGROUND:Pleural effusion frequently complicates hepatectomy and multiple factors contribute to its development following hepatectomy for primary liver cancer. The purpose of this study was to evaluate these factors. METHODS:From March 2003 to May 2005, 228 consecutive patients with primary liver cancer underwent hepatectomy in our department were evaluated retrospec-tively to identify factors related to postoperative pleural effusion. RESULTS: Among the 228 patients, postoperative pleural effusions arose in 58 (25.4%). Univariate analysis showed signiifcant differences in postoperative ascites, subphrenic collection, Pringle manoeuvre length, drainage amount on postoperative day 1, albumin level on postoperative day 7, alanine aminotransferase (ALT) level on postoperative days 1 and 3, prealbumin level on postoperative days 3 and 7, and tumor size (P CONCLUSION: Subphrenic collection and operative injury to the liver appeared to be signiifcantly related to pleural effusion after hepatectomy for primary liver cancer.

  2. The epigenetic landscape of latent Kaposi sarcoma-associated herpesvirus genomes.

    Directory of Open Access Journals (Sweden)

    Thomas Günther

    Full Text Available Herpesvirus latency is generally thought to be governed by epigenetic modifications, but the dynamics of viral chromatin at early timepoints of latent infection are poorly understood. Here, we report a comprehensive spatial and temporal analysis of DNA methylation and histone modifications during latent infection with Kaposi Sarcoma-associated herpesvirus (KSHV, the etiologic agent of Kaposi Sarcoma and primary effusion lymphoma (PEL. By use of high resolution tiling microarrays in conjunction with immunoprecipitation of methylated DNA (MeDIP or modified histones (chromatin IP, ChIP, our study revealed highly distinct landscapes of epigenetic modifications associated with latent KSHV infection in several tumor-derived cell lines as well as de novo infected endothelial cells. We find that KSHV genomes are subject to profound methylation at CpG dinucleotides, leading to the establishment of characteristic global DNA methylation patterns. However, such patterns evolve slowly and thus are unlikely to control early latency. In contrast, we observed that latency-specific histone modification patterns were rapidly established upon a de novo infection. Our analysis furthermore demonstrates that such patterns are not characterized by the absence of activating histone modifications, as H3K9/K14-ac and H3K4-me3 marks were prominently detected at several loci, including the promoter of the lytic cycle transactivator Rta. While these regions were furthermore largely devoid of the constitutive heterochromatin marker H3K9-me3, we observed rapid and widespread deposition of H3K27-me3 across latent KSHV genomes, a bivalent modification which is able to repress transcription in spite of the simultaneous presence of activating marks. Our findings suggest that the modification patterns identified here induce a poised state of repression during viral latency, which can be rapidly reversed once the lytic cycle is induced.

  3. Etiological diagnosis of pericardial effusion.

    Science.gov (United States)

    Levy, Pierre-Yves; Habib, Gilbert; Collart, Fréderic; Lepidi, Hubert; Raoult, Didier

    2006-08-01

    Detection and treatment of pericarditis remains a challenging problem and the etiology is unknown in 40-85% of cases. As a result, a large proportion of cases are labeled idiopathic pericarditis. The advent of echocardiography, an accurate noninvasive method for the detection of effusion, has clarified the definition from pericarditis to pericardial effusion, which is a standardized and clear entity. A systematic approach to diagnostic testing based on standardized practice guidelines has been proposed. This strategy has led to a decrease in the number of cases classified as idiopathic and to the identification of treatable conditions. Percutaneous pericardiocentesis, guided by fluoroscopy or echocardiography, can now be carried out safely and rapidly and has also allowed the intrapericardial instillation of drugs, representing a new treatment strategy. The inclusion of flexible pericardioscopy, immunohistochemistry and contemporary molecular biology tools has improved the diagnostic value of the biopsy.

  4. Pleural effusion in liver disease.

    Science.gov (United States)

    Alonso, José Castellote

    2010-12-01

    Hepatic hydrothorax is the paradigmatic pleural effusion in liver cirrhosis. It is defined as a pleural effusion in a patient with portal hypertension and no cardiopulmonary disease. The estimated prevalence of this complication in patients with liver cirrhosis is 5 to 6%. Its pathophysiology involves movement of ascitic fluid from the peritoneal cavity into the pleural space through diaphragmatic defects. Thoracentesis and pleural fluid analysis are necessary for diagnosis. Initial management consists of sodium restriction, diuretics, and therapeutic thoracentesis. A transjugular intrahepatic portosystemic shunt may provide a bridge prior to liver transplantation. Spontaneous bacterial empyema is the infection of a preexisting hydrothorax. The more frequent bacteria involved are ENTEROBACTERIACEAE and gram-positive cocci. Antibiotic therapy is the cornerstone of therapy. This article reviews etiology, clinical manifestations, and therapy of these two complications of liver cirrhosis and portal hypertension.

  5. Uveal effusion syndrome (clinical case

    Directory of Open Access Journals (Sweden)

    Yu. A. Belyy

    2015-01-01

    Full Text Available The purpose — to evaluate the effectiveness of the posterior sclerectomy with the corneal trepan in a uveal effusion syndrome.Patients and methods. Patient 1. The man, 61 years, complained about a blindness of the right eye and the progressing decrease in vision of the left eye. According to data of examinations the diagnosis was: uveal effusion syndrome, detachment of the choroid, exudative retinal detachment, the complicated cataract of both eyes. Patient 2. The man, 62 years, with complaints to low vision of the right eye and a blindness of the left-hand eye. Diagnosis: uveal effusion syndrome, detachment of the choroid, exudative retinal detachment, the initial complicated cataract of the right eye. Subatrophy operated retinal detachment, complicated cataract, silicone into the vitreal cavity of the left eye. Both patients underwent trepanation posterior sclera. Results. The patient 1noted significant improvement of vision in both eyes at the last examination. Мisual acuity with correction was OD — 0,2, OS — 0.3. Intraocular pressure was normal, improvement of electrical sensitivity and lability was diagnosed. On ultrasound examination of the retina belonged, moderate swelling of the choroid remained. Patient 2. Visual acuity was 0,1 + 3,0 D at the time of the last inspection. According to the ultrasonic B-scan of the right eye retinal detachment decreased to 3,8 mm, moderate swelling of the choroid remained. The OСT has showed detachment of the neuroepithelium in the macula to 60 μm. In ultrasonic biomicroscopy circular, ciliochoroidal detachment to 0,15 mm was diagnised.Conclusion. Described clinical cases confirmed the almost complete recovery of patients with the uveal effusion syndrome. Sclerectomy with the use of corneal trepan is a technically simple procedure and helps to define the path of the outflow suprachoroidal fluid subtenon space.

  6. Management of malignant pleural effusion

    OpenAIRE

    Boshuizen, R.C.

    2017-01-01

    The first part of this thesis focuses on IPCs (indwelling pleural catheters) in malignant pleural effusion (MPE) management. In an invited review, the (dis)advantages and prejudices of IPCs are described (Chapter1.1). Since costs and reimbursement issues are the main reasons in the Netherlands to withhold patients from IPCs, we performed a retrospective analysis of a prospectively collected database. In this database, we registered patient characteristics (gender, tumor type), survival data a...

  7. Immunotherapeutic Intervention against Sarcomas

    Directory of Open Access Journals (Sweden)

    Paolo Pedrazzoli, Simona Secondino, Vittorio Perfetti, Patrizia Comoli, Daniela Montagna

    2011-01-01

    Full Text Available Advances in systemic therapy for sarcoma have produced, over the last two decades, relatively short-term benefits for the majority of patient. Among the novel biologic therapeutics that will likely increase our ability to cure human cancer in the years to come, immunotherapy is one of the most promising approaches. While past attempts to use immunotherapy have failed to dramatically shift the paradigm of care for the treatment of patients with sarcoma, major advances in basic and translational research have resulted, in more recent years, in clinical trial activity that is now beginning to generate promising results. However, to move from “proof of principle” to large scale clinical applicability, we need well-designed, multi-institutional clinical trials, along with continuous laboratory research to explore further the immunological characteristics of individual sarcoma subtypes and the consequent tailoring of therapy.

  8. [Sarcoma of the breast].

    Science.gov (United States)

    Haberthür, F; Almendral, A C; Feichter, G; Torhorst, J K

    1992-05-01

    Sarcoma of the breast represents only 0.2-1% of all mammary malignancies. This study reports 5 such cases, including 2 osteosarcomas, 1 fibro-, 1 lipo-, and 1 malignant fibrous sarcoma. The treatment used was mastectomy in 3 cases with excision of axillary lymph nodes. The remaining 2 patients were treated by simple mastectomy whereby 1 of these received a immediate reconstruction with a prosthesis. 1 patient demonstrated local recurrence and died. The remaining 4 patients did not develop neither metastases nor local recurrence and are still alive after an observing period between 12 months up to 17 years. Today, first-line treatment is wide local excision or simple mastectomy. Excision of the axillary lymphatics, adjuvant radiotherapy, and chemotherapy have been disappointing in the treatment of breast sarcoma.

  9. GLUT1 Expression in Synovial Sarcomas

    Directory of Open Access Journals (Sweden)

    Gülşah KAYGUSUZ

    2009-09-01

    Full Text Available Objective: In this study, the role of GLUT1 expression in synovial sarcomas and its association with disease pathogenesis were examined.Materials and Methods: Twenty two cases of synovial sarcoma were included in this study. The clinicopathological features of the cases, such as age, sex, localization of tumor, information of primary or metastatic tumor, histopathological type were recorded. The tissue microarray paraffin block containing tumor tissues was built by using tissue microarrayer. GLUT1 expression was analyzed on tissue sections by immunohistochemistry.Results: A total of 22 cases (mean age 36 years; range 14-54 years were analyzed. All cases except one were primary tumors. The tumors showed monophasic histological type in 13 cases and biphasic type in 9 cases. GLUT1 expression was found in 3 cases with biphasic type (14%. The cytoplasmic and incomplete membranous GLUT1 expression was seen in the tumor cells showing epithelial-glandular differentiation, whereas spindled cells were negative.Conclusion: Although GLUT1 expression is a diagnostic marker for juvenile capillary hemangioma and perineural tumors, both of which included in the group of mesenchymal tumors, it can be seen in a subset of synovial sarcomas. In our series, the observation of GLUT1 expression especially in the epithelial component of biphasic synovial sarcomas suggests that; i GLUT1 may be relatively used by tumoral cells composing epithelial component of the tumor, and ii the spindle cell component of the tumor would have been positive for other glucose transporters. The finding of uncommon GLUT1 expression in synovial sarcomas is indirectly consistent with the reported results of decreased standardized uptake value by Positron emission tomography with 2-[18F]fluoro-2-deoxy-D-glucose method in the literature.

  10. [Moritz Kaposi and his sarcoma].

    Science.gov (United States)

    van Kessel, Anne; Quint, Koen D

    2011-01-01

    Nowadays, Kaposi sarcoma is a multidisciplinary condition, not only observed by dermatologists. Since the HIV epidemic in the 80s and 90s of the last century, more insight into the aetiology of Kaposi sarcoma has been acquired. However, this sarcoma had already been described in 1872 by a Hungarian dermatologist named Moritz Kaposi (1832-1902). Kaposi described the entity as 'idiopathic multiple pigmented sarcoma of the skin'. This entity was an extraordinary diagnosis at that time, mostly observed in Jewish or Mediterranean men. In 1912, 10 years after the death of Moritz Kaposi, the entity name was changed to Kaposi sarcoma.

  11. T-cell prolymphocytic leukemia presenting with leukemic serous effusion in a prostate cancer patient

    Directory of Open Access Journals (Sweden)

    Ozan Salim

    2015-01-01

    Full Text Available T-cell prolymphocytic leukemia (T-PLL is highly aggressive mature postthymic lymphoproliferative disorder, which is characterized by several clinical features. Leukemic prolymphocytes are found in the peripheral blood, bone marrow, lymph nodes, spleen, liver, and sometimes skin. T-PLL and solid tumor coincidence was reported by only four previous cases. Solid tumor components included breast cancer, classic Kaposi sarcoma, gastric cancer, and lung cancer in those cases. We report the first case of T-PLL, an extremely rare disease, presented with serous effusion in an elderly prostate cancer patient in literature.

  12. Advance of Therapeutic Methods for Malignant Pleural Effusion

    Institute of Scientific and Technical Information of China (English)

    XU Tao-tao

    2016-01-01

    Malignant pleural effusion (MPE) is a condition caused by primary malignant tumors in the pleura or other malignant tumors metastasis to the pleura. It is also one of common serious complications of middle-late malignant tumor, which has severe impact on the quality of life, even threatening the life of the patients. The selection of treatments for MPE depends on many factors, including the symptoms, performance status, primary tumor types, response to systemic therapy, and degree of lung recruitment maneuvers (LRM) after drainage of pleural effusion. Generally, the treatment methods include thoracentesis, indwelling pleural catheter, pleurodesis, intrapleural injection of drugs, chemotherapy, radiotherapy, anti-angiogenesis therapy, surgery, and thermotherapy. With the in-depth study on pathogenesis of MPE, the treatments of MPE have continuous improvements. This study mainly reviewed the treatment methods for MPE so as to provide the basis for clinical practice in the future.

  13. Advance of Therapeutic Methods for Malignant Pleural Effusion

    Directory of Open Access Journals (Sweden)

    Tao-tao XU

    2016-06-01

    Full Text Available Malignant pleural effusion (MPE is a condition caused by primary malignant tumors in the pleura or other malignant tumors metastasis to the pleura. It is also one of common serious complications of middle-late malignant tumor, which has severe impact on the quality of life, even threatening the life of the patients. The selection of treatments for MPE depends on many factors, including the symptoms, performance status, primary tumor types, response to systemic therapy, and degree of lung recruitment maneuvers (LRM after drainage of pleural effusion. Generally, the treatment methods include thoracentesis, indwelling pleural catheter, pleurodesis, intrapleural injection of drugs, chemotherapy, radiotherapy, anti-angiogenesis therapy, surgery, and thermotherapy. With the in-depth study on pathogenesis of MPE, the treatments of MPE have continuous improvements. This study mainly reviewed the treatment methods for MPE so as to provide the basis for clinical practice in the future.

  14. Rapidly Regressive Unilateral Fetal Pleural Effusion

    Directory of Open Access Journals (Sweden)

    Tuncay Yuce

    2015-03-01

    Full Text Available Intrauterine pleural effusion of fetal lungs rarely regresses without intervention. In our case we treated a women at 32th weeks of gestation. Her pregnancy was complicated with fetal pleural effusion and polyhydramniosis. A therapeutic thoracocentesis was planned and she received two courses of betamethasone prior to procedure. On the day of planned procedure, a substantial regression of pleural effusion was observed and procedure was postponed. During her antenatal follow-up a complete regression of pleural effusion was observed. After delivery pleural effusion did not relapse. These findings hint there may be a role of antenatal steroids in treatment of fetal pleural effusion, which is known to be resistant to treatment modalities both during antenatal and postnatal period. [Cukurova Med J 2015; 40(Suppl 1: 25-28

  15. Pulmonary Kaposi's sarcoma after heart transplantation: a case report

    Directory of Open Access Journals (Sweden)

    Kristiansen Glen

    2010-07-01

    Full Text Available Abstract Introduction Kaposi's sarcomas have been associated with different conditions of immunosuppression and are also known to be a typical complication of solid organ transplantations. Case presentation We report the case of a 65-year-old Turkish man with a history of heart transplantation 10 months ago who presented for clarification of his dyspnea. The patient had a known history of chronic obstructive pulmonary disease and a smoking history of 40 pack years. Radiologically, three progressively growing intra-pulmonary nodules were detected. The histology was diagnostic for a Kaposi's sarcoma. Visceral and especially primary intra-pulmonary Kaposi's sarcomas are very rare and have been described to have a rather unfavorable prognosis. Conclusions Even with a history suggestive for conventional lung cancer, Kaposi's sarcomas should be considered in patients after transplantation of solid organs. It should be noted that in a minority of cases this tumor exists in the absence of the typical cutaneous lesions.

  16. Analysis of The Xx Century Effusive Activity At Mount Etna To Assess The Potential Effusive Hazard.

    Science.gov (United States)

    Andronico, D.; Lodato, L.; Neri, M.

    We examined older documents and publications together with more recent and de- tailed reports related to the 20th century effusive activity (terminal and flank eruptions) at Mt. Etna, with the primary objective of creating a single data-base of volcanologi- cal data, at present lacking in the literature. The restitution in digital form of the maps of the lava fields allowed to measure, with great precision compared with previous estimations, the area and length of every lava flow, allowing improved estimates of volumes and effusive rates. The quantity and the quality of the collected bibliographic information of the studied eruptions improve progressively from the beginning of the century up to the present, and can be considered complete for the flank activity. The data of each single eruption have been inserted into a relational database, that pro- vides both ease of use and the potential of upgrading with information from new or more ancient eruptions. By examining the collected data (e.g. maximum length of lava flows, altitude reached, eruption duration) we obtained some useful relationships, that allowed us to characterise some eruption typologies at Etna. The improvement of the volcanic data related to the effusive activity of the last century represents an impor- tant contribution in terms of volcanic hazard, suggesting for example the areas most at risk by opening of eruptive fractures and lava covering. Statistical observations can be a useful contribution to the planning required by civil defense in a strongly urban- ized area, such as the etnean one; assessment hazard, in particular, should be focused particularly on the southeastern foot of the volcanic belt, in which the volcanic risk in- creases due to the presence of several populated centers that amount to approximately 500.000 people.

  17. A Study on Significance of Serum Effusion Albumin Gradient in The Differential Diagnosisof Pleural Effusion

    OpenAIRE

    Arijit Kumar Das; Krishna Baruah

    2009-01-01

    To evaluate serum pleural effusion albumin gradient (SEAG) as method of differentiating pleural transudatesfrom exudates.Cases admitted in AMCH with diagnosed pleural effusion were divided into 2 groups basedon etiology. Group I (transudates): Comprising 14 patients of congestive heart failure (n=6) and nephroticsyndrome (n=3), Cirrhosis (n=4), pericardial effusion (n=1). Group II (exudates): comprising 26 cases oftuberculous (n=15), malignant (n=8) and parapneumonic effusion (n=2), rheumatoi...

  18. Extremity perfusion for sarcoma

    NARCIS (Netherlands)

    Hoekstra, Harald Joan

    2008-01-01

    For more than 50 years, the technique of extremity perfusion has been explored in the limb salvage treatment of local, recurrent, and multifocal sarcomas. The "discovery" of tumor necrosis factor-or. in combination with melphalan was a real breakthrough in the treatment of primarily irresectable ext

  19. Sarcoma Foundation of America

    Science.gov (United States)

    ... Google+ Twitter LinkedIn YouTube © 2017 Sarcoma Foundation of America | All Rights Reserved. | Terms of Use | Privacy Policy Website Design & Hosting by 270net Technologies, Inc. X - Enter Your Location - - or - Get your current location Home About Us History People Public Filings News & Media SFA in the ...

  20. Epidemic Kaposi Sarcoma

    Science.gov (United States)

    ... and its treatment, see the AIDSinfo website . Nonepidemic Gay-related Kaposi Sarcoma There is a type of ... better than another. Trials are based on past studies and what has been learned ... by their creator. In such cases, it is necessary to contact the writer, artist, ...

  1. Classic Kaposi Sarcoma

    Science.gov (United States)

    ... and its treatment, see the AIDSinfo website . Nonepidemic Gay-related Kaposi Sarcoma There is a type of ... better than another. Trials are based on past studies and what has been learned ... by their creator. In such cases, it is necessary to contact the writer, artist, ...

  2. Extremity perfusion for sarcoma

    NARCIS (Netherlands)

    Hoekstra, Harald Joan

    2008-01-01

    For more than 50 years, the technique of extremity perfusion has been explored in the limb salvage treatment of local, recurrent, and multifocal sarcomas. The "discovery" of tumor necrosis factor-or. in combination with melphalan was a real breakthrough in the treatment of primarily irresectable

  3. Synovial sarcoma mechanisms

    DEFF Research Database (Denmark)

    Svejstrup, Jesper Q

    2013-01-01

    Human synovial sarcoma is caused by a chromosome translocation, which fuses DNA encoding SSX to that encoding the SS18 protein. Kadoch and Crabtree now show that the resulting cellular transformation stems from disruption of the normal architecture and function of the human SWI/SNF (BAF) complex....

  4. Microenvironmental targets in sarcoma

    Directory of Open Access Journals (Sweden)

    Monika eEhnman

    2015-11-01

    Full Text Available Sarcomas are rare malignant tumors affecting all age groups. They are typically classified according to their resemblance to corresponding normal tissue. Their heterogeneous features, for example in terms of disease-driving genetic aberrations and body location, complicate both disease classification and development of novel treatment regimens. Many years of failure of improved patient outcome in clinical trials has lead to the conclusion that novel targeted therapies are likely needed in combination with current multimodality regimens. Sarcomas have not, in contrast to the common carcinomas, been the subject for larger systematic studies on how tumor behavior relates to characteristics of the tumor microenvironment. There is consequently an urgent need for identifying suitable molecular targets, not only in tumor cells, but also in the tumor microenvironment. This review discusses preclinical and clinical data about potential molecular targets in sarcomas. Studies on targeted therapies involving the tumor microenvironment are prioritized. A greater understanding of the biological context is expected to facilitate more successful design of future clinical trials in sarcoma.

  5. Leukosis/Sarcoma Group

    Science.gov (United States)

    The leukosis/sarcoma (L/S) group of diseases designates a variety of transmissible benign and malignant neoplasms of chickens caused by members that belong to the family Retroviridae. Because the expansion of the literature on this disease, it is no longer feasible to cite all relevant publications ...

  6. Management of Bone Sarcoma.

    Science.gov (United States)

    Gutowski, Christina J; Basu-Mallick, Atrayee; Abraham, John A

    2016-10-01

    Treatment of bone sarcoma requires careful planning and involvement of an experienced multidisciplinary team. Significant advancements in systemic therapy, radiation, and surgery in recent years have contributed to improved functional and survival outcomes for patients with these difficult tumors, and emerging technologies hold promise for further advancement. Copyright © 2016 Elsevier Inc. All rights reserved.

  7. Systematic identification of cellular signals reactivating Kaposi sarcoma-associated herpesvirus.

    Directory of Open Access Journals (Sweden)

    Fuqu Yu

    2007-03-01

    Full Text Available The herpesvirus life cycle has two distinct phases: latency and lytic replication. The balance between these two phases is critical for viral pathogenesis. It is believed that cellular signals regulate the switch from latency to lytic replication. To systematically evaluate the cellular signals regulating this reactivation process in Kaposi sarcoma-associated herpesvirus, the effects of 26,000 full-length cDNA expression constructs on viral reactivation were individually assessed in primary effusion lymphoma-derived cells that harbor the latent virus. A group of diverse cellular signaling proteins were identified and validated in their effect of inducing viral lytic gene expression from the latent viral genome. The results suggest that multiple cellular signaling pathways can reactivate the virus in a genetically homogeneous cell population. Further analysis revealed that the Raf/MEK/ERK/Ets-1 pathway mediates Ras-induced reactivation. The same pathway also mediates spontaneous reactivation, which sets the first example to our knowledge of a specific cellular pathway being studied in the spontaneous reactivation process. Our study provides a functional genomic approach to systematically identify the cellular signals regulating the herpesvirus life cycle, thus facilitating better understanding of a fundamental issue in virology and identifying novel therapeutic targets.

  8. Preparation and characterization of polyclonal antibody against Kaposi's sarcoma-associated herpesvirus lytic gene encoding RTA.

    Science.gov (United States)

    Fan, Weifei; Tang, Qiao; Shen, Chenyou; Qin, Di; Lu, Chun; Yan, Qin

    2015-11-01

    Replication and transcription activator (RTA) is a critical lytic protein encoded by Kaposi's sarcoma-associated herpesvirus (KSHV). To prepare rabbit polyclonal antibody against RTA, three antigenic polypeptides of KSHV RTA were initially synthesized. The fragment of RTA was cloned into p3FlagBsd to construct the recombinant plasmid, pRTA-Flag. 293 T and EA.hy926 cells were transfected with pRTA-Flag to obtain RTA-Flag fusion protein, which was detected using anti-Flag antibody. Next, New Zealand white rabbits were immunized with keyhole limpet hemocyanin-conjugated peptides to generate polyclonal antibodies against RTA. Enzyme-linked immunosorbent assays were performed to characterize the polyclonal antibodies, and the titers of the polyclonal antibodies against RTA were greater than 1:11,000. Western blotting and immunofluorescence assay revealed that the prepared antibody reacted specifically with the RTA-Flag fusion protein as well as the native viral protein in KSHV-infected primary effusion lymphoma cells. Collectively, our work successfully constructed the recombinant expression vector, pRTA-Flag, and prepared the polyclonal antibody against RTA, which was valuable for investigating the biochemical and biological functions of the critical KSHV lytic gene.

  9. [Soft tissue sarcoma in children and adolescents: experiences of the cooperative Soft Tissue Sarcoma Group Studies (CWS-81 - 96)].

    Science.gov (United States)

    Brecht, I B; Treuner, J

    2004-10-01

    The very heterogeneous group of paediatric soft tissue sarcomas account for approximately 7 % of all malignant childhood tumours. More than one half of all cases are rhabdomyosarcomas, some of the over 20 entities are very rare. The prognosis and biology of soft tissue sarcomas in children and adolescents vary greatly depending on histological subtype, the age of the patient, the primary site, the tumour size, tumour invasiveness and the extent of disease at diagnosis. Since 1981, 2918 children and adolescents with soft tissue sarcomas were treated prospectively according to the common treatment protocols of the Cooperative Soft Tissue Sarcoma Study Group (CWS-81 - 96). The known prognostic factors were used to develop a more and more detailed risk stratification. The multimodal treatment includes the use of surgery, chemotherapy and radiotherapy and should be planned by a multidisciplinary team. That way, an overall survival of nearly 70 % over all risk groups could be achieved.

  10. Radiation Therapy for Soft Tissue Sarcomas

    Science.gov (United States)

    ... Stage Soft Tissue Sarcoma Treating Soft Tissue Sarcomas Radiation Therapy for Soft Tissue Sarcomas Radiation therapy uses ... spread. This is called palliative treatment . Types of radiation therapy External beam radiation therapy: For this treatment, ...

  11. Postoperative pleural effusion following upper abdominal surgery

    DEFF Research Database (Denmark)

    Nielsen, P H; Jepsen, S B; Olsen, A D

    1989-01-01

    Of 128 patients who underwent upper abdominal surgery, examined by standard preoperative and postoperative chest roentgenograms for the formation of postoperative pleural effusions, 89 had postoperative pleural effusions. Their presence was not related to the type of operation, infection, serum a...

  12. Postoperative pleural effusion following upper abdominal surgery

    DEFF Research Database (Denmark)

    Nielsen, P H; Jepsen, S B; Olsen, A D

    1989-01-01

    Of 128 patients who underwent upper abdominal surgery, examined by standard preoperative and postoperative chest roentgenograms for the formation of postoperative pleural effusions, 89 had postoperative pleural effusions. Their presence was not related to the type of operation, infection, serum a...

  13. Diagnosis exjuvantibus of a persistent pleural effusion

    Directory of Open Access Journals (Sweden)

    José M. Porcel

    2013-12-01

    Full Text Available The diagnosis of uremia-associated effusion is one of exclusion. A patient with an unexplained chronic pleural exudate, which cleared with dialysis, is reported. The differential diagnosis of pleural effusions in patients with chronic kidney disease and the management of uremic pleuritis is briefly discussed.

  14. Vascular endothelial growth factor in diagnosis of pleural effusion

    Directory of Open Access Journals (Sweden)

    Nasr H. Khalil

    2017-01-01

    Conclusion: VEGF pleural fluid level could differentiate between malignant and non malignant effusion, while could not differentiate between tuberculous and nontuberculous, or between parapneumonic and nonparapneumonic exudative effusions.

  15. Intrapleural instillation of radioactive chromic phosphate in malignant pleural effusion

    Energy Technology Data Exchange (ETDEWEB)

    Nori, D.; Moorthy, C.R.; Hilaris, B.S.; Batata, M.A.; Chu, F.; Martini, N. (Memorial Sloan-Kettering Cancer Center, New York (USA))

    1981-12-01

    Eighty-five patients with pleural effusion were analyzed to determine the degree of response to intrapleural instillation of /sup 32/P colloidal suspension. Worthwhile palliation of either complete fluid suppression or definite diminution in the rate of accumulation was achieved in slightly more than one-half of the patients (51/85). It is not evident whether the primary site and histologic nature of the neoplasm are important or not in predicting the results of treatment. Patients with pleural adhesions and those with sanguinous effusions, are less likely to be benefited by the /sup 32/P instillation. The beneficial effect appears quite promptly in some patients, while in others, it may be delayed for several weeks.

  16. Pleural effusion: diagnosis, treatment, and management

    Directory of Open Access Journals (Sweden)

    Karkhanis VS

    2012-06-01

    Full Text Available Vinaya S Karkhanis, Jyotsna M JoshiDepartment of Respiratory Medicine, TN Medical College and BYL Nair Hospital, Mumbai, IndiaAbstract: A pleural effusion is an excessive accumulation of fluid in the pleural space. It can pose a diagnostic dilemma to the treating physician because it may be related to disorders of the lung or pleura, or to a systemic disorder. Patients most commonly present with dyspnea, initially on exertion, predominantly dry cough, and pleuritic chest pain. To treat pleural effusion appropriately, it is important to determine its etiology. However, the etiology of pleural effusion remains unclear in nearly 20% of cases. Thoracocentesis should be performed for new and unexplained pleural effusions. Laboratory testing helps to distinguish pleural fluid transudate from an exudate. The diagnostic evaluation of pleural effusion includes chemical and microbiological studies, as well as cytological analysis, which can provide further information about the etiology of the disease process. Immunohistochemistry provides increased diagnostic accuracy. Transudative effusions are usually managed by treating the underlying medical disorder. However, a large, refractory pleural effusion, whether a transudate or exudate, must be drained to provide symptomatic relief. Management of exudative effusion depends on the underlying etiology of the effusion. Malignant effusions are usually drained to palliate symptoms and may require pleurodesis to prevent recurrence. Pleural biopsy is recommended for evaluation and exclusion of various etiologies, such as tuberculosis or malignant disease. Percutaneous closed pleural biopsy is easiest to perform, the least expensive, with minimal complications, and should be used routinely. Empyemas need to be treated with appropriate antibiotics and intercostal drainage. Surgery may be needed in selected cases where drainage procedure fails to produce improvement or to restore lung function and for closure of

  17. Genetic profiling of putative breast cancer stem cells from malignant pleural effusions.

    Science.gov (United States)

    Tiran, Verena; Stanzer, Stefanie; Heitzer, Ellen; Meilinger, Michael; Rossmann, Christopher; Lax, Sigurd; Tsybrovskyy, Oleksiy; Dandachi, Nadia; Balic, Marija

    2017-01-01

    A common symptom during late stage breast cancer disease is pleural effusion, which is related to poor prognosis. Malignant cells can be detected in pleural effusions indicating metastatic spread from the primary tumor site. Pleural effusions have been shown to be a useful source for studying metastasis and for isolating cells with putative cancer stem cell (CSC) properties. For the present study, pleural effusion aspirates from 17 metastatic breast cancer patients were processed to propagate CSCs in vitro. Patient-derived aspirates were cultured under sphere forming conditions and isolated primary cultures were further sorted for cancer stem cell subpopulations ALDH1+ and CD44+CD24-/low. Additionally, sphere forming efficiency of CSC and non-CSC subpopulations was determined. In order to genetically characterize the different tumor subpopulations, DNA was isolated from pleural effusions before and after cell sorting, and compared with corresponding DNA copy number profiles from primary tumors or bone metastasis using low-coverage whole genome sequencing (SCNA-seq). In general, unsorted cells had a higher potential to form spheres when compared to CSC subpopulations. In most cases, cell sorting did not yield sufficient cells for copy number analysis. A total of five from nine analyzed unsorted pleura samples (55%) showed aberrant copy number profiles similar to the respective primary tumor. However, most sorted subpopulations showed a balanced profile indicating an insufficient amount of tumor cells and low sensitivity of the sequencing method. Finally, we were able to establish a long term cell culture from one pleural effusion sample, which was characterized in detail. In conclusion, we confirm that pleural effusions are a suitable source for enrichment of putative CSC. However, sequencing based molecular characterization is impeded due to insufficient sensitivity along with a high number of normal contaminating cells, which are masking genetic alterations of

  18. Synovial sarcoma of the kidney in a young patient with a review of the literature

    Directory of Open Access Journals (Sweden)

    Mahmoud Abbas

    2014-06-01

    Full Text Available Synovial sarcoma (SS is a soft tissue, generally deep seated neoplasms that occurs generally in the proximity of large joints. We report of a case of a 33-year-old man who was diagnosed with primary SS of the kidney which is an extremely rare tumor that accounts for less than 2% of malignant renal tumors. Contemporary management of renal synovial sarcoma includes surgical resection and ifosfamide-based chemotherapy and they remain the mainstay of therapy of synovial sarcoma, which is often applied, combined as part of an aggressive treatment approach. Fewer than 50 patients have been described in the English literature. Physicians should be aware of the possibility of malignancy in cystic renal masses and raise the suspicion of synovial sarcoma, especially when patients with renal masses are young adults. Along with the case report a literature review on primary synovial sarcomas of the kidney is provided with focus on the renal tumors’ differential diagnosis.

  19. Prevalence, hemodynamics, and cytokine profile of effusive-constrictive pericarditis in patients with tuberculous pericardial effusion.

    Directory of Open Access Journals (Sweden)

    Mpiko Ntsekhe

    Full Text Available BACKGROUND: Effusive constrictive pericarditis (ECP is visceral constriction in conjunction with compressive pericardial effusion. The prevalence of proven tuberculous ECP is unknown. Whilst ECP is distinguished from effusive disease on hemodynamic grounds, it is unknown whether effusive-constrictive physiology has a distinct cytokine profile. We conducted a prospective study of prevalence and cytokine profile of effusive-constrictive disease in patients with tuberculous pericardial effusion. METHODS: From July 2006 through July 2009, the prevalence of ECP and serum and pericardial levels of inflammatory cytokines were determined in adults with tuberculous pericardial effusion. The diagnosis of ECP was made by combined pericardiocentesis and cardiac catheterization. RESULTS: Of 91 patients evaluated, 68 had tuberculous pericarditis. The 36/68 patients (52.9%; 95% confidence interval [CI]: 41.2-65.4 with ECP were younger (29 versus 37 years, P=0.02, had a higher pre-pericardiocentesis right atrial pressure (17.0 versus 10.0 mmHg, P 15 mmHg (odds ratio [OR] = 48, 95%CI: 8.7-265; P 200 pg/ml (OR=10, 95%CI: 1.1, 93; P=0.04 were independently associated with ECP. CONCLUSION: Effusive-constrictive disease occurs in half of cases of tuberculous pericardial effusion, and is characterized by greater elevation in the pre-pericardiocentesis right atrial pressure and pericardial and serum IL-10 levels compared to patients with effusive non-constrictive tuberculous pericarditis.

  20. Mast cells mediate malignant pleural effusion formation

    Science.gov (United States)

    Giannou, Anastasios D.; Marazioti, Antonia; Spella, Magda; Kanellakis, Nikolaos I.; Apostolopoulou, Hara; Psallidas, Ioannis; Prijovich, Zeljko M.; Vreka, Malamati; Zazara, Dimitra E.; Lilis, Ioannis; Papaleonidopoulos, Vassilios; Kairi, Chrysoula A.; Patmanidi, Alexandra L.; Giopanou, Ioanna; Spiropoulou, Nikolitsa; Harokopos, Vaggelis; Aidinis, Vassilis; Spyratos, Dionisios; Teliousi, Stamatia; Papadaki, Helen; Taraviras, Stavros; Snyder, Linda A.; Eickelberg, Oliver; Kardamakis, Dimitrios; Iwakura, Yoichiro; Feyerabend, Thorsten B.; Rodewald, Hans-Reimer; Kalomenidis, Ioannis; Blackwell, Timothy S.; Agalioti, Theodora; Stathopoulos, Georgios T.

    2015-01-01

    Mast cells (MCs) have been identified in various tumors; however, the role of these cells in tumorigenesis remains controversial. Here, we quantified MCs in human and murine malignant pleural effusions (MPEs) and evaluated the fate and function of these cells in MPE development. Evaluation of murine MPE-competent lung and colon adenocarcinomas revealed that these tumors actively attract and subsequently degranulate MCs in the pleural space by elaborating CCL2 and osteopontin. MCs were required for effusion development, as MPEs did not form in mice lacking MCs, and pleural infusion of MCs with MPE-incompetent cells promoted MPE formation. Once homed to the pleural space, MCs released tryptase AB1 and IL-1β, which in turn induced pleural vasculature leakiness and triggered NF-κB activation in pleural tumor cells, thereby fostering pleural fluid accumulation and tumor growth. Evaluation of human effusions revealed that MCs are elevated in MPEs compared with benign effusions. Moreover, MC abundance correlated with MPE formation in a human cancer cell–induced effusion model. Treatment of mice with the c-KIT inhibitor imatinib mesylate limited effusion precipitation by mouse and human adenocarcinoma cells. Together, the results of this study indicate that MCs are required for MPE formation and suggest that MC-dependent effusion formation is therapeutically addressable. PMID:25915587

  1. The microbiome of otitis media with effusion.

    Science.gov (United States)

    Chan, Chun Ling; Wabnitz, David; Bardy, Jake Jervis; Bassiouni, Ahmed; Wormald, Peter-John; Vreugde, Sarah; Psaltis, Alkis James

    2016-12-01

    The adenoid pad has been considered a reservoir for bacteria in the pathogenesis of otitis media with effusion. This study aimed to characterize the middle ear microbiota in children with otitis media with effusion and establish whether a correlation exists between the middle ear and adenoid microbiota. Prospective, controlled study. Middle ear aspirates adenoid pad swabs were collected from 23 children undergoing ventilation tube insertion. Adenoid swabs from patients without ear disease were controls. Samples were analyzed using 16S rRNA sequencing on the Illumina MiSeq platform. Thirty-five middle ear samples were collected. The middle ear effusion microbiota was dominated by Alloiococcus otitidis (23% mean relative abundance), Haemophilus (22%), Moraxella (5%), and Streptococcus (5%). Alloiococcus shared an inverse correlation with Haemophilus (P = .049) and was found in greater relative abundance in unilateral effusion (P = .004). The microbiota of bilateral effusions from the same patient were similar (P otitis media with effusion microbiota were found to be dissimilar to that of the adenoid (P = .01), whereas the adenoid microbiota of otitis media with effusion and control patients were similar (P > .05) (permutational multivariate analysis of the variance). Dissimilarities between the local microbiota of the adenoid and the middle ear question the theory that the adenoid pad is a significant reservoir to the middle ear in children with otitis media with effusion. A otitidis had the greatest cumulative relative abundance, particularly in unilateral effusions, and shares an inverse correlation with the relative abundance of Haemophilus. NA Laryngoscope, 126:2844-2851, 2016. © 2016 The American Laryngological, Rhinological and Otological Society, Inc.

  2. General Information about Ewing Sarcoma

    Science.gov (United States)

    ... tumor that forms from a certain kind of cell in bone or soft tissue . Ewing sarcoma may be found in the bones of the legs, arms, feet, hands, chest , pelvis , spine , or skull . Ewing sarcoma also may be found in the soft tissue of the trunk, arms, legs, head and neck, abdominal cavity , or ...

  3. Treatment Option Overview (Ewing Sarcoma)

    Science.gov (United States)

    ... tumor that forms from a certain kind of cell in bone or soft tissue . Ewing sarcoma may be found in the bones of the legs, arms, feet, hands, chest , pelvis , spine , or skull . Ewing sarcoma also may be found in the soft tissue of the trunk, arms, legs, head and neck, abdominal cavity , or ...

  4. Effusion plate using additive manufacturing methods

    Energy Technology Data Exchange (ETDEWEB)

    Johnson, Thomas Edward; Keener, Christopher Paul; Ostebee, Heath Michael; Wegerif, Daniel Gerritt

    2016-04-12

    Additive manufacturing techniques may be utilized to construct effusion plates. Such additive manufacturing techniques may include defining a configuration for an effusion plate having one or more internal cooling channels. The manufacturing techniques may further include depositing a powder into a chamber, applying an energy source to the deposited powder, and consolidating the powder into a cross-sectional shape corresponding to the defined configuration. Such methods may be implemented to construct an effusion plate having one or more channels with a curved cross-sectional geometry.

  5. Effusion plate using additive manufacturing methods

    Science.gov (United States)

    Johnson, Thomas Edward; Keener, Christopher Paul; Ostebee, Heath Michael; Wegerif, Daniel Gerritt

    2016-04-12

    Additive manufacturing techniques may be utilized to construct effusion plates. Such additive manufacturing techniques may include defining a configuration for an effusion plate having one or more internal cooling channels. The manufacturing techniques may further include depositing a powder into a chamber, applying an energy source to the deposited powder, and consolidating the powder into a cross-sectional shape corresponding to the defined configuration. Such methods may be implemented to construct an effusion plate having one or more channels with a curved cross-sectional geometry.

  6. Sarcoma risk after radiation exposure

    Directory of Open Access Journals (Sweden)

    Berrington de Gonzalez Amy

    2012-10-01

    Full Text Available Abstract Sarcomas were one of the first solid cancers to be linked to ionizing radiation exposure. We reviewed the current evidence on this relationship, focusing particularly on the studies that had individual estimates of radiation doses. There is clear evidence of an increased risk of both bone and soft tissue sarcomas after high-dose fractionated radiation exposure (10 + Gy in childhood, and the risk increases approximately linearly in dose, at least up to 40 Gy. There are few studies available of sarcoma after radiotherapy in adulthood for cancer, but data from cancer registries and studies of treatment for benign conditions confirm that the risk of sarcoma is also increased in this age-group after fractionated high-dose exposure. New findings from the long-term follow-up of the Japanese atomic bomb survivors suggest, for the first time, that sarcomas can be induced by acute lower-doses of radiation (

  7. Sorafenib in Treating Patients With Soft Tissue Sarcomas (Extremity Sarcoma Closed to Entry as of 5/30/07)

    Science.gov (United States)

    2014-04-01

    Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Osteosarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Osteosarcoma; Stage I Adult Soft Tissue Sarcoma; Stage II Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

  8. Video-assisted thoracoscopic pericardial window for diagnosis and management of pericardial effusions.

    Science.gov (United States)

    Georghiou, Georgios P; Stamler, Alon; Sharoni, Erez; Fichman-Horn, Suzana; Berman, Marius; Vidne, Bernardo A; Saute, Milton

    2005-08-01

    Video-assisted thoracoscopy with the creation of a pericardial window is a noninvasive method of pericardial drainage. It also allows an excellent view of both the pleural cavity and pericardium and the precise selection of biopsy sites. We review our 3-year experience with this technique. Between January 2001 and February 2004, 18 patients (10 men, 8 women; mean age 57 years) with echocardiographically diagnosed pericardial effusion underwent video-assisted thoracoscopy at our center. Pericardial windows were created under general anesthesia and single-lung ventilation using 2 to 3 trocars. Mean operating time was 46 minutes. A right thoracic approach was used in 16 patients and a left thoracic approach in 2. Microbiology and virology cultures of the pericardial fluid were negative. Histologic findings were compatible with tuberculosis in 2 cases and granulocytic sarcoma, infiltrating breast carcinoma, and infiltrating nonsmall cell carcinoma in 1 case each. In the remaining patients, the histologic diagnosis was chronic or subacute nonspecific pericarditis. Talc pleurodesis was performed in 3 patients for concomitant malignant pleural effusion. In 4 patients, the pericardial effusion occurred secondary to cardiac surgery; 3 were receiving anticoagulants after valve replacement, and 1 had a heart transplant. There were no complications of the thoracoscopy technique. Video-assisted thoracoscopic fenestration is an effective technique for pericardial drainage and biopsy. Apart from its diagnostic value, it allows the physician to fashion a pleuropericardial window for effective drainage while avoiding the complications of classic surgical procedures. Concomitant pleural and pulmonary disorders may be managed simultaneously.

  9. TRANSFORMATION OF EFFUSION AND FORMATION OF GRANULATION TISSUE IN PATHOLOGIC PROCESS OF OTITIS MEDIA WITH EFFUSION

    Institute of Scientific and Technical Information of China (English)

    韦俊荣; 张青; 张全安

    2003-01-01

    Objective To explore the diversity of stagnant effusions and the pathologic processes leading to granulation tissue formation in otitis media with effusion(OME). Methods Temporal bone slides from 306 ears with OME were studied histopathologically under the light microscope. Results Results of this study revealed a pathologic process in witch the type and the condition of stagnant effusion in the middle ear cleft was a variable in the dynamics of OME progression from an early stage to an advanced stage. The location of granulation tissue and retentive effusion were found to be closely related. Conclusion Early stage granulation tissue formation exhibited a pathologic process in which granulation tissue formation occurred only in areas where effusion had stagnated or was absorbed. The incidence of the retentive effusion and formation of granulation tissue was much higher and the pathologic changes most extensive in the area around the ossicular chain.

  10. Collecting and Storing Biological Samples From Patients With Ewing Sarcoma

    Science.gov (United States)

    2016-11-21

    Askin Tumor; Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor

  11. Diagnostic Study of Tumor Characteristics in Patients With Ewing's Sarcoma

    Science.gov (United States)

    2013-06-20

    Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor

  12. Lava effusion rate definition and measurement--A review

    Science.gov (United States)

    Calvari, Sonia; Dehn, Jonathan; Harris, A.

    2007-01-01

    Measurement of effusion rate is a primary objective for studies that model lava flow and magma system dynamics, as well as for monitoring efforts during on-going eruptions. However, its exact definition remains a source of confusion, and problems occur when comparing volume flux values that are averaged over different time periods or spatial scales, or measured using different approaches. Thus our aims are to: (1) define effusion rate terminology; and (2) assess the various measurement methods and their results. We first distinguish between instantaneous effusion rate, and time-averaged discharge rate. Eruption rate is next defined as the total volume of lava emplaced since the beginning of the eruption divided by the time since the eruption began. The ultimate extension of this is mean output rate, this being the final volume of erupted lava divided by total eruption duration. Whether these values are total values, i.e. the flux feeding all flow units across the entire flow field, or local, i.e. the flux feeding a single active unit within a flow field across which many units are active, also needs to be specified. No approach is without its problems, and all can have large error (up to ∼50%). However, good agreement between diverse approaches shows that reliable estimates can be made if each approach is applied carefully and takes into account the caveats we detail here. There are three important factors to consider and state when measuring, giving or using an effusion rate. First, the time-period over which the value was averaged; second, whether the measurement applies to the entire active flow field, or a single lava flow within that field; and third, the measurement technique and its accompanying assumptions.

  13. Parapneumonic pleural effusion: early versus late thoracoscopy.

    Science.gov (United States)

    Pereira, Rodrigo Romualdo; Alvim, Cristina Gonçalves; Andrade, Cláudia Ribeiro de; Ibiapina, Cássio da Cunha

    2017-07-31

    To evaluate the best time to perform thoracoscopy for the treatment of complicated parapneumonic pleural effusion in the fibrinopurulent phase in patients ≤ 14 years of age, regarding the postoperative evolution and occurrence of complications. This was a retrospective comparative study involving patients with parapneumonic pleural effusion presenting with septations or loculations on chest ultrasound who underwent thoracoscopy between January of 2000 and January of 2013. The patients were divided into two groups: early thoracoscopy (ET), performed by day 5 of hospitalization; and late thoracoscopy (LT), performed after day 5 of hospitalization. We included 60 patients, 30 in each group. The mean age was 3.4 years; 28 patients (46.7%) were male; and 47 (78.3%) underwent primary thoracoscopy (no previous simple drainage). The two groups were similar regarding gender, age, weight, and type of thoracoscopy (p > 0.05 for all). There was a significant difference between the ET and the LT groups regarding the length of the hospital stay (14.5 days vs. 21.7 days; p sexo masculino, e 47 (78,3%) foram submetidos à toracoscopia primária, sem realização de drenagem simples prévia. Os grupos TP e TT foram semelhantes quanto ao sexo, idade, peso e tipo de toracoscopia (p > 0,05 para todos). Observou-se uma diferença significativa quanto à média de duração da internação nos grupos TP e TT (14,5 dias vs. 21,7 dias; p < 0,001). Houve também diferenças significativas entre os grupos quanto ao total de dias com febre, total de dias entre internação e início da drenagem e total de dias com dreno. Oito pacientes (13,6%) apresentaram alguma complicação após a toracoscopia, sem diferença entre os grupos. Não houve óbitos. A TP, realizada até o 5º dia da admissão hospitalar, associou-se a menor duração da internação, menor tempo de drenagem e menor duração da febre, sem estar associada a maior frequência de complicações, necessidade de CTI ou

  14. [Synovial sarcoma. Case report].

    Science.gov (United States)

    Deme, Dániel; Abdulfatah, Bishr; Telekes, András

    2016-02-07

    In 2013 there were 94,770 new cancer patients reported in Hungary. Synovial sarcoma accounts for 0.05-0.1% of all cancers and, therefore its incidence is predicted to be 47-94 patients/year in Hungary. The authors report the history of a 18-year-old man who was operated on a right upper abdominal wall tumor with R1 resection. During the next 5 months the tumor grew up to 8 cm in largest diameter. Histology revealed monophasic synovial sarcoma. Immunohistochemistry showed bcl2, focal CD99 and high molecular weight cytokeratin positivity, while smooth muscle actin, S100 and CD34 immunostainings were negative. Becose of this reoperation was not possible, curative six cycles of doxorubicine and ifosfamide with granulocyte colony stimulating factor support and 60 Gy radiotherapy was given to the tumor bed. After these treatments computed tomography scan was negative and the patient attended regular imaging every 3 months. At the age of 20 years the patient developed two neoplastic lesions in the surgical scar measuring 10 mm and 45 × 10 mm in size. R0 resection, partial rib resection and abdominal wall reconstruction were performed. Histology confirmed residual monophasic synovial sarcoma. Radiotherapy was not given because of a risk of intestinal wall perforation. Staging positron emission tomography-computed tomography proved to be negative. At the age of 22 years magnetic resonance imaging scans indicated no tumor recurrence, but after one month a rapidly growing tumorous lesion was found on ultrasound in the surgical scar measuring 20 × 20 × 12 mm in size. Cytology confirmed local recurrence and fluorescence in situ hibridization indicated t(x;18). R0 exstirpation and partial mesh resection were performed and histology showed the same monophasic synovial sarcoma. Because of the presence of vascular invasion and a close resection margin (1 mm) the patient underwent 3 cycles of adjuvant chemotherapy (doxorubicine and ifosfamide) with granulocyte colony stimulating

  15. Proclus and Mulla Sadra on First Effusion

    Directory of Open Access Journals (Sweden)

    Zahra Eskandari

    2013-12-01

    Full Text Available Mulla Sadra Mulla Sadra's philosophical system is built upon the belief in unique origin of world to which all diversities can be reduced. Then First Effusion debate can be taken on in this system. According to the principle which reads "from the One nothing is effused but One", contingent beings could not have been emanated from the Necessary Being in a horizontal fashion due to their diversity. Rather they have been effused from the Necessary in a vertical fashion and through a hierarchy of causes and effects. The first stage of the hierarchy is being represented by the First Effusion. Pre-Sadraeian theosophers regarded the First Effusion the first intellect in vertical chain of intellects. Mulla Sadra have two theories of the First Effusion. In some of his books he introduces the first intellect as the first entity effused from the Necessary keeping his pace with his own predecessors. But in some other works like Asfar he describes the Ever-unfolding existence as the First Effusion and takes it to be his final position. Having replaced existential gradation with causation and also individual unity of existence with existential gradation, Mulla Sadra asserts that the First Effusion could only be the Ever-unfolding Existence which is a manifestation of Divine Essence not an independent existence. It is indeed nothing but the Divine Essence although in the form of its first manifestation. Thus the Ever-unfolding Existence has three distinguished attributes which make it qualified to stand in direct relationship with Divine Essence, Existential (and not conceptual universality and comprehensiveness which enables this existence to include the other manifestions. Lack of particular limitation and determination; this is why it can reveal itself to every being in its own peculiar existential terms. Having unity while being imbued in diversity and being diversed while having unity (due to its true but shadowy unity (Rahimian, 1383: 187. First

  16. [The enhancement phenomenon of serous cavity effusions].

    Science.gov (United States)

    Gaeta, M; Volta, S; Stroscio, S; Carella, I; Mantineo, G; Bartiromo, G; Pandolfo, I

    1990-01-01

    The enhancement of ascites following a high dose of contrast medium has been recently described. The CT behavior of 26 peritoneal, pleural or pericardial effusions has been studied in 23 patients after the administration of a high dose of urographic contrast medium. The effusions were enhanced in all patients but one. No difference between malignant and non-malignant effusions could be observed with ionic and non-ionic contrast medium. Low molecular weight of the urographic contrast medium (600-800 daltons) can explain the free passage of the solute through peritoneum, pleura, and pericardium. The knowledge of such a phenomenon is mandatory to avoid misdiagnosing hematic effusion or urinary-peritoneal fistula.

  17. Proclus and Mulla Sadra on First Effusion

    Directory of Open Access Journals (Sweden)

    Saeed Rahimiyan

    2014-01-01

    Full Text Available Mulla Sadra Mulla Sadra's philosophical system is built upon the belief in unique origin of world to which all diversities can be reduced. Then First Effusion debate can be taken on in this system. According to the principle which reads "from the One nothing is effused but One", contingent beings could not have been emanated from the Necessary Being in a horizontal fashion due to their diversity. Rather they have been effused from the Necessary in a vertical fashion and through a hierarchy of causes and effects. The first stage of the hierarchy is being represented by the First Effusion. Pre-Sadraeian theosophers regarded the First Effusion the first intellect in vertical chain of intellects. Mulla Sadra have two theories of the First Effusion. In some of his books he introduces the first intellect as the first entity effused from the Necessary keeping his pace with his own predecessors. But in some other works like Asfar he describes the Ever-unfolding existence as the First Effusion and takes it to be his final position. Having replaced existential gradation with causation and also individual unity of existence with existential gradation, Mulla Sadra asserts that the First Effusion could only be the Ever-unfolding Existence which is a manifestation of Divine Essence not an independent existence. It is indeed nothing but the Divine Essence although in the form of its first manifestation. Thus the Ever-unfolding Existence has three distinguished attributes which make it qualified to stand in direct relationship with Divine Essence, Existential (and not conceptual universality and comprehensiveness which enables this existence to include the other manifestions. Lack of particular limitation and determination; this is why it can reveal itself to every being in its own peculiar existential terms. Having unity while being imbued in diversity and being diversed while having unity (due to its true but shadowy unity (Rahimian, 1383: 187. First

  18. Kinetics of Kaposi’s Sarcoma-Associated Herpesvirus Gene Expression

    Science.gov (United States)

    Sun, Ren; Lin, Su-Fang; Staskus, Katherine; Gradoville, Lyndle; Grogan, Elizabeth; Haase, Ashley; Miller, George

    1999-01-01

    Herpesvirus gene expression can be classified into four distinct kinetic stages: latent, immediate early, early, and late. Here we characterize the kinetic class of a group of 16 Kaposi’s sarcoma-associated herpesvirus (KSHV)/human herpesvirus 8 genes in a cultured primary effusion cell line and examine the expression of a subset of these genes in KS biopsies. Expression of two latent genes, LANA and vFLIP, was constitutive and was not induced by chemicals that induce the lytic cycle in primary effusion lymphoma (PEL) cell lines. An immediate-early gene, Rta (open reading frame 50 [ORF50]), was induced within 4 h of the addition of n-butyrate, and its 3.6-kb mRNA was resistant to inhibition by cycloheximide. Early genes, including K3 and K5 that are homologues of the “immediate-early” gene of bovine herpesvirus 4, K8 that is a positional homologue of Epstein-Barr virus BZLF1, vMIP II, vIL-6, and polyadenylated nuclear (PAN) RNA, appeared 8 to 13 h after chemical induction. A second group of early genes that were slightly delayed in their appearance included viral DHFR, thymidylate synthase, vMIP I, G protein-coupled receptor, K12, vBcl2, and a lytic transcript that overlapped LANA. The transcript of sVCA (ORF65), a late gene whose expression was abolished by Phosphonoacetic acid, an inhibitor of KSHV DNA replication, did not appear until 30 h after induction. Single-cell assays indicated that the induction of lytic cycle transcripts resulted from the recruitment of additional cells into the lytic cycle. In situ hybridization of KS biopsies showed that about 3% of spindle-shaped tumor cells expressed Rta, ORF K8, vIL-6, vMIP I, vBcl-2, PAN RNA, and sVCA. Our study shows that several KSHV-encoded homologues of cellular cytokines, chemokines, and antiapoptotic factors are expressed during the viral lytic cycle in PEL cell lines and in KS biopsies. The lytic cycle of KSHV, probably under the initial control of the KSHV/Rta gene, may directly contribute to tumor

  19. Cardiac tamponade due to low-volume effusive constrictive pericarditis in a patient with uncontrolled type II autoimmune polyglandular syndrome.

    Science.gov (United States)

    Palmer, William C; Kurklinsky, Andrew; Lane, Gary; Ussavarungsi, Kamonpun; Blackshear, Joseph L

    2014-03-01

    Type II autoimmune polyglandular syndrome (APS), a relatively common endocrine disorder, includes primary adrenal insufficiency coupled with type 1 diabetes mellitus and/or autoimmune primary hypothyroidism. Autoimmune serositis, an associated disease, may present as symptomatic pericardial effusion. We present a case of a 54-year old male with APS who developed pericarditis leading to cardiac tamponade with a subacute loculated effusion. After urgent pericardiocentesis intrapericardial pressure dropped to 0, while central venous pressures remain elevated, consistent with acute effusive constrictive pericarditis. Contrast computerized tomography confirmed increased pericardial contrast enhancement. The patient recovered after prolonged inotropic support and glucocorticoid administration. He re-accumulated the effusion 16 days later, requiring repeat pericardiocentesis. Effusive-constrictive pericarditis, an uncommon pericardial syndrome, is characterized by simultaneous pericardial inflammation and tamponade. Prior cases of APS associated with cardiac tamponade despite low volumes of effusion have been reported, albeit without good demonstration of hemodynamic findings. We report a case of APS with recurrent pericardial effusion due to pericarditis and marked hypotension with comprehensive clinical and hemodynamic assessment. These patients may require aggressive support with pericardiocentesis, inotropes, and hormone replacement therapy. They should be followed closely for recurrent tamponade.

  20. Human immunodeficiency virus negative Kaposi sarcoma and lymphoproliferative disorders

    NARCIS (Netherlands)

    Fossati, S; Boneschi, [No Value; Ferrucci, S; Brambilla, L

    1999-01-01

    BACKGROUND. The concomitant occurrence of more than one primary neoplasm in the same individual has led researchers to seek possible common etiopathogenetic factors. Kaposi sarcoma (KS) is a multicentric neoplasm of vascular origin and perhaps viral etiology. Four forms of KS are known: classic or M

  1. Cancer incidence after retinoblastoma - Radiation dose and sarcoma risk

    NARCIS (Netherlands)

    Wong, FL; Boice, JD; Abramson, DH; Tarone, RE; Kleinerman, RA; Stovall, M; Goldman, MB; Seddon, JM; Tarbell, N; Fraumeni, JF; Li, FP

    1997-01-01

    Context.-There is a substantial risk of a second cancer for persons with hereditary retinoblastoma, which is enhanced by radiotherapy. Objective.-To examine long-term risk of new primary cancers in survivors of childhood retinoblastoma and quantify the role of radiotherapy in sarcoma development. De

  2. Acute Liver Failure Due to Budd-Chiari Syndrome in the Setting of Cardiac Synovial Sarcoma

    OpenAIRE

    Stine, Jonathan G.; Newton, Kelly; Vinayak, Ajeet G

    2015-01-01

    Primary malignant tumors of the heart, specifically cardiac sarcomas, are rare and mainly diagnosed at autopsy. Acute Budd-Chiari syndrome is a recognized cause of acute liver failure and has been associated with several rare cardiac tumors: atrial myxoma, caval rhabdomyosarcoma, and primary cardiac adenocarcinoma. We present the first case of a fatal, highly differentiated cardiac synovial sarcoma that presented as acute liver failure from Budd-Chiari syndrome.

  3. Study on osteogenic sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Park, Eung Chun; Kim, Young Il; Choi, Won Jae; Kim, Young Jin [Dept. of Dentomaxillofacial Radiology, College of Dentistry, Chosun University, Kwangju (Korea, Republic of)

    1993-02-15

    The author observed a case of osteogenic sarcoma in a 11-year-old female with complaint of painful swelling on face in right side. The observed results were as follows: 1. Large hematoma was observed, and patient complained painfull swelling on c/c site. 2. Predisposing factor of osteogenic sarcoma was not clear, but patient had history of extraction before patient visiting infirmary of our dental collage. 3. Serologic findings were not specific, and serum aldaline level was normal. 4. Radiographic findings were as follows: a. Diffuse faint radiopacit in the lesion. b. Bony destruction and increased radiopacity in right antrum. c. Displacement of multiple teeth on involved area (i. e no 12, 15, 55, 16, 17, 18) d. Increased periodontal space in single tooth (no 13) e. Destruction of bony crypt on involved teeth (no 13, 14, 15, 17, 18) f. Loss of lamina dura of three teeth in involved area (no 11, 12, 16) 5. Computed tomographic findings were as follows: a. Large calcific and heterogenous component mass in the Rt. maxillary sinus, and this mass extending to Rt. maxilla, alveolar bone, ethmoid sinus. b. Soft tissue bulging in to Rt. side nasal cavity and oral cavity. c. Bone destruction of maxillary sinus wall and Rt. alveolar bone.

  4. Kaposi Sarcoma Herpesvirus (KSHV Latency-Associated Nuclear Antigen (LANA recruits components of the MRN (Mre11-Rad50-NBS1 repair complex to modulate an innate immune signaling pathway and viral latency.

    Directory of Open Access Journals (Sweden)

    Giuseppe Mariggiò

    2017-04-01

    Full Text Available Kaposi Sarcoma Herpesvirus (KSHV, a γ2-herpesvirus and class 1 carcinogen, is responsible for at least three human malignancies: Kaposi Sarcoma (KS, Primary Effusion Lymphoma (PEL and Multicentric Castleman's Disease (MCD. Its major nuclear latency protein, LANA, is indispensable for the maintenance and replication of latent viral DNA in infected cells. Although LANA is mainly a nuclear protein, cytoplasmic isoforms of LANA exist and can act as antagonists of the cytoplasmic DNA sensor, cGAS. Here, we show that cytosolic LANA also recruits members of the MRN (Mre11-Rad50-NBS1 repair complex in the cytosol and thereby inhibits their recently reported role in the sensing of cytoplasmic DNA and activation of the NF-κB pathway. Inhibition of NF-κB activation by cytoplasmic LANA is accompanied by increased lytic replication in KSHV-infected cells, suggesting that MRN-dependent NF-κB activation contributes to KSHV latency. Cytoplasmic LANA may therefore support the activation of KSHV lytic replication in part by counteracting the activation of NF-κB in response to cytoplasmic DNA. This would complement the recently described role of cytoplasmic LANA in blocking an interferon response triggered by cGAS and thereby promoting lytic reactivation. Our findings highlight a second point at which cytoplasmic LANA interferes with the innate immune response, as well as the importance of the recently discovered role of cytoplasmic MRN complex members as innate sensors of cytoplasmic DNA for the control of KSHV replication.

  5. Clear Cell Sarcoma of Gluteal Region Malignant Melanoma of Soft Parts

    Directory of Open Access Journals (Sweden)

    Haren V. Oza

    2013-04-01

    Full Text Available Clear cell sarcoma (CCS is described as variant of sarcoma characterized by prominent clear cells showing features similar to malignant melanoma of soft parts. This neoplasm was first described by Dr. Franz m. Enzinger. Primary CCS usually arises in deeper soft tissues, in association with fascia, tendons, or aponeuroses. Clear cell sarcoma (CCS is a rare malignant tumor with a propensity for slow progressive invasion. It is a tumor derived from Melanoblast like cell. They occur most commonly in the extremities, with a predilection for young females. Clear cell sarcoma of tendons and aponeuroses (malignant melanoma of soft parts and conventional malignant melanoma may demonstrate significant morphologic overlap at the light microscopic and ultra structural level. The tumor is very rare and can pose clinical challenges in early diagnosis. This case report demonstrates an unusual site of occurrence for clear cell sarcoma. [Natl J Med Res 2013; 3(2.000: 193-195

  6. Cystic synovial sarcomas: imaging features with clinical and histopathologic correlation

    Energy Technology Data Exchange (ETDEWEB)

    Nakanishi, Hirofumi; Araki, Nobuhito [Department of Orthopedic Surgery, Osaka Medical Center for Cancer and Cardiovascular Diseases, 1-3-3, Nakamichi, Higashinari-Ku, 537-8511, Osaka (Japan); Sawai, Yuka [Department of Radiology, Osaka Medical Center for Cancer and Cardiovascular Diseases, Osaka (Japan); Kudawara, Ikuo [Department of Orthopedic Surgery, Osaka National Hospital, Osaka (Japan); Mano, Masayuki; Ishiguro, Shingo [Department of Pathology, Osaka Medical Center for Cancer and Cardiovascular Diseases, Osaka (Japan); Ueda, Takafumi; Yoshikawa, Hideki [Department of Orthopedic Surgery, Osaka University Graduate School of Medicine, Suita, Osaka (Japan)

    2003-12-01

    To characterize the radiological and clinicopathologic features of cystic synovial sarcoma. Seven patients with primary cystic synovial sarcoma were evaluated. Computed tomography (CT) and magnetic resonance (MR) imaging were undertaken at the first presentation. The diagnosis of synovial sarcoma was made on the basis of histological examinations followed by molecular analysis. Radiological and clinicopathologic findings were reviewed. CT showed well-defined soft tissue mass without cortical bone erosion and invasion. Calcification was seen at the periphery of the mass in three cases. T2-weighted MR images showed multilocular inhomogeneous intensity mass in all cases, five of which showed fluid-fluid levels. On gross appearance, old and/or fresh hematomas were detected in six cases. In the one remaining case, microscopic hemorrhage in the cystic lumen was proven. Four cases had poorly differentiated areas. In five cases prominent hemangiopericytomatous vasculature was observed. Histologic grade was intermediate in one tumor and high in six. One case had a history of misdiagnosis for tarsal tunnel syndrome, one for lymphadenopathy, two for sciatica and two for hematoma. All cystic synovial sarcomas demonstrated multilocularity with well-circumscribed walls and internal septae. Synovial sarcoma should be taken into consideration in patients with deeply situated multicystic mass with triple signal intensity on T2-weighted MR imaging. (orig.)

  7. RADIOGRAPHIC IMAGING OF BIPHASIC SYNOVIAL SARCOMA ON LEFT ELBOW

    Directory of Open Access Journals (Sweden)

    Sitanggang Firman P

    2013-04-01

    Full Text Available Synovial sarcoma is the fourth most common type of sarcoma. It represents between 5% -10% of all soft tissue sarcomas and most prevalent in aged between 15 - 40 years. Synovial sarcoma is the most commonly misdiagnosed soft tissue malignancy, initially as an inflammation process,often because it may be slow-growing, have a benign appearance on imaging studies, may vary in size, and may have pain similar to that associated with trauma.    A rare case is presented of 17 years old women with a synovial sarcoma biphasic. The primary tumor originated in the left elbow since 2004. Since then, the patient has had repeated passive or active left elbow pain and tenderness. No history of trauma. She has not developed metastases of the lung. The patient refused to have surgery and others medical procedures that already planned and explained to her. The conclusion of this case report point that radiology is important to diagnosis and planning for further management

  8. SYNOVIAL SARCOMA IN CHILDHOOD: CLINICAL AND RADIOLOGICAL FINDINGS

    Institute of Scientific and Technical Information of China (English)

    Xu Deyong; Zhan Alai; Luan Hongmei; Feng Weihua; Sun Xihe; Yang Zuwen

    1998-01-01

    Objective: To study the clinical characteristics and radiological features of synovial sarcoma in childhood and its relation to the diagnosis and treatment. Methods:The clinical radiological features of 15 children with synovial sarcoma proved surgically and pathologically were analyzed. Results: In children, the tumor boundaries are poorly defined due to paucity of fat, and metastasis usually occurs early. Eight patients in this series had bone involvement, including: direct erosion by tumor causing cortical destruction, indirect pressure defect with sharp margin and reactive bone sclerosis and bone destruction of the primary intraosseous synovial sarcoma.Conclusion: The tumor is often misdiagnosed, the final confirmed diagnosis must be made by histological examination with imaging findings. It is emphasized that the patients should be treated with radiotherapy and chemotherapy preoperatively and postoperatively.

  9. What Should You Ask Your Doctor about Kaposi Sarcoma?

    Science.gov (United States)

    ... What Should You Ask Your Doctor About Kaposi Sarcoma? Kaposi Sarcoma Early Detection, Diagnosis, and Staging What Should You Ask Your Doctor About Kaposi Sarcoma? As you cope with Kaposi sarcoma (KS) and ...

  10. Drugs Approved for Kaposi Sarcoma

    Science.gov (United States)

    This page lists cancer drugs approved by the Food and Drug Administration (FDA) for Kaposi sarcoma. The list includes generic names and brand names. The drug names link to NCI's Cancer Drug Information summaries.

  11. Osteogenic Sarcoma: A 21st Century Review.

    Science.gov (United States)

    Osasan, Stephen; Zhang, Mingyong; Shen, Fan; Paul, Paulose J; Persad, Sujata; Sergi, Consolato

    2016-09-01

    Compared to other bone tumors, bone osteogenic sarcoma (BOS) continues to confer a much grimmer prognosis as the survival benefit of traditional chemotherapy treatment regimens is still unsatisfactory. Chemotherapy was demonstrated to be effective in eradicating both primary tumor and pulmonary metastases in the last century, with effective agents used in various combination regimens having changed the survival rate from less than 10% to 75%. The most common primary bone cancer, BOS is conventionally a primary intramedullary high-grade malignant tumor characterized by malignant cells forming immature bone or osteoid. BOS is a disease with diverse morphological presentations. The treatment of all morphological variants seem to have been the same for over 30 years. The introduction of antiproliferative agents such as insulin growth factor-binding protein 3 hold promise of a potentially veritable therapeutic target. In this review, we highlight recent data on osteosarcoma to consolidate a platform able to connect bench and bedside.

  12. Myeloid Sarcoma in the Orbit.

    Science.gov (United States)

    Qian, Xiaoxiao; Gigantelli, James W; Abromowitch, Minnie; Morgan, Linda A; Suh, Donny W

    2016-12-08

    The authors describe a case of myeloid sarcoma of the orbit in a pediatric patient. An 8-month-old male infant presented to the ophthalmology clinic with a left orbital mass, which had been increasing in size over the previous 2 months. The mass was initially diagnosed at another clinic as an infantile hemangioma, and had been treated with a topical formulation of timolol. In the ophthalmology clinic, orbital magnetic resonance imaging showed a solid enhancing mass. A biopsy was performed, and histopathology revealed myeloid sarcoma. The disease responded well to a standard chemotherapy regimen. Myeloid sarcoma is a rare, extra-medullary presentation that can occur as an isolated tumor, concurrently with or at relapse of acute myeloid leukemia. Because few cases of myeloid sarcoma in the orbit have been reported, this case report aids in the management of myeloid sarcoma in pediatric patients. The report describes an 8-month-old male infant, the youngest patient to develop myeloid sarcoma without preexisting acute myeloid leukemia. [J Pediatr Ophthalmol Strabismus. 2016;53:e64-e68.].

  13. Reversible tricuspid valve stenosis due to a metastatic dissemination of a noncardiac sarcoma.

    Science.gov (United States)

    Uribe-Etxebarria, Naia; Voces, Roberto; Rodriguez, Miguel Angel; Llorente, Alberto; Perez, Pedro; Aramendi, Jose I

    2005-07-01

    Malignant disease is present in the pericardium of 1.5% to 20.6% of patients dying of malignant diseases as was examined postmortem. We present a case of a 57-year-old man with a history of Hodgkin's disease and a sarcoma of gluteus who presented with tachypnea, generalized weakness, and anasarca for 7 days. The echocardiogram revealed the presence of a significant pericardial thickening and localized pericardial effusion resulting from a tricuspid stenosis. A right anterior thoracotomy was performed, and a pericardiectomy (4 x 4 cm) was done. The histologic examination of the pericardium revealed the presence of a metastatic dissemination from a sarcoma. The cause for the clinical presentation and the treatment of malignant pericardial disease are discussed.

  14. Pleural effusion and sarcoidosis: an unusual combination.

    Science.gov (United States)

    Ferreiro, Lucía; San José, Esther; González-Barcala, Francisco Javier; Suárez-Antelo, Juan; Toubes, M Elena; Valdés, Luis

    2014-12-01

    Pleural involvement in sarcoidosis is uncommon and appears in several forms. To document the incidence and characteristics of pleural effusion in sarcoidosis patients, a review of the cases diagnosed in our centre between January 2001 and December 2012 was carried out. One hundred and ninety-five patients with sarcoidosis were identified; three (two men and one woman) presented with unilateral pleural effusion (1.5%): one in the right side and two in the left. Two were in stageii and one was in stageiv. The pleural fluid of the two patients who underwent thoracocentesis was predominantly lymphocytic. One of these patients presented chylothorax and the other had high CA-125levels. In general, these effusions are lymphocyte-rich, paucicellular, serous exudates (sometimes chylothorax) and contain proportionally higher levels of protein than LDH. Most cases are treated with corticosteroids, although it may resolve spontaneously. Copyright © 2013 SEPAR. Published by Elsevier Espana. All rights reserved.

  15. Massive pleural effusion in a young woman

    Directory of Open Access Journals (Sweden)

    Paolo Ghiringhelli

    2012-12-01

    Full Text Available Pleural effusion is a clinical manifestation shared by several underlying pathologies. The differential diagnosis is based on the clinical history, the physical examination, the analysis of the pleural fluid, and the laboratory data (mainly blood tests. There are cases, such as the patient described, where TC is not enough, and unusual imaging techniques are required for the study of pleural effusion, i.e. magnetic resonance cholangiography, cholangiopancreatography (MRCP and endoscopic retrograde cholangiopancreatography (ERCP.This case analyses a 42-year-old female patient who arrived with progressive dyspnoea, chest pain, cough, a history of alcohol abuse, and a recent episode of acute pancreatitis. The physical examination revealed signs of right-sided pleural effusion. These features, together with laboratory data, made it possible to pose the diagnosis of pancreaticopleural fistula, to treat it, and to obtain a complete healing in a two-month period.

  16. Factors influencing pleural drainage in parapneumonic effusions.

    Science.gov (United States)

    Porcel, J M; Valencia, H; Bielsa, S

    2016-10-01

    The identification of parapneumonic effusions (PPE) requiring pleural drainage is challenging. We aimed to determine the diagnostic accuracy of radiological and pleural fluid findings in discriminating between PPE that need drainage (complicated PPE (CPPE)) and those that could be resolved with antibiotics only (uncomplicated PPE (UPPE)). A retrospective review of 641 consecutive PPE, of which 393 were categorized as CPPE and 248 as UPPE. Demographics, radiological (size and laterality on a chest radiograph) and pleural fluid parameters (pus, bacterial cultures, biochemistries) were compared among groups. Logistic regression was performed to determine variables useful for predicting chest drainage, and receiver-operating characteristic curves assisted in the selection of the best cutoff values. According to the likelihood ratios (LR), findings increasing the probability of chest tube usage the most were: effusions occupying ≥1/2 of the hemithorax (LR 13.5), pleural fluid pH ≤7.15 (LR 6.2), pleural fluid glucose ≤40mg/dL (LR 5.6), pus (LR 4.8), positive pleural fluid cultures (LR 3.6), and pleural fluid lactate dehydrogenase >2000U/L (LR 3.4). In the logistic regression analysis only the first two were selected as significant predictors of CPPE. In non-purulent effusions, the effusion's size and pleural fluid pH retained their discriminatory properties, in addition to a pleural fluid C-reactive protein (CRP) level >100mg/L. Large radiological effusions and a pleural fluid pH ≤7.15 were the best predictors for chest drainage in patients with PPE. In the subgroup of patients with non-purulent effusions, pleural fluid CRP also contributed to CPPE identification. Copyright © 2016 Elsevier España, S.L.U. and Sociedad Española de Medicina Interna (SEMI). All rights reserved.

  17. Primary Renal Synovial Sarcoma With Recurrence and Metastasis%原发性肾滑膜肉瘤及术后复发、转移的影像学表现

    Institute of Scientific and Technical Information of China (English)

    李炯佾; 曹文娟; 杨呈伟; 崔久法; 王林; 彭国辉; 李秉诚; 孙伟; 张维明; 张浩

    2012-01-01

    Objective To discuss the imaging features of primary renal synovial sarcoma(PRSS)with recurrence and tastasis and improve its diagnostic accuracy. Materials and Methods The clinical, imaging and pathology findings, im-munohistochemistry (IHC) of 1 patient with PRSS were retrospectively analyzed, and the literature was reviewed to evaluate the relationships between the imaging signs, clinical and pathologic findings. Results CT scan: A mass lesion with indistinguishable borders to the kidney, rooting from the right kidney and extending to the inferior and medial aspect anterior to the psoas muscle, about 10.7 cm × 12.3 cm × 15. 5 cm, demonstrating a tabular contour and including cystic areas was detected on CT. No additional tumor, lung metastases.paracaval or paraaortic lymph nodes were detected on CT. A hypoecho-ic solid mass lesion extending from the lower pole of the right kidney to the inferior and medial aspect, about 13.5 cm × 11.1 cm × 10.5 cm, demonstrating a tabular contour including cystic areas and star point like flow signals was seen on the ultrasonic examination. The diagnosis of PRSS in the patient was confirmed by postoperative pathology. Under microscope, tumor was typically mitotically active,monomorphic spindle cells growing in intersecting fascicles or in solid sheets with epithelial differentiation. In some areas a haemangiopericytoma like pattern was found. IHC showed that the tumor cells were positive for the markers Vimentin, CD 99 and Bel 2, but CK was negative. The patient died of local recurrence and multi-metastasis in 8 months after surgery. Conclusion PRSS is extremely rare and malignant tumor with poor prognosis. There are some relatively specific imaging findings, however its diagnosis is depend on pathology, IHC and SYT-SSX gene detection. It has been demonstrated that the use of CT in the diagnosis and the evaluation of the staging of the PRSS is useful by defining lymph node involvement, distant metastases, and local

  18. Toxocariasis: An unusual cause of pleural effusion.

    Science.gov (United States)

    Vallentin, Blandine; Carsin, Ania; Dubus, Jean-Christophe

    2015-10-01

    Toxocara canis, one of the most frequent parasites worldwide, rarely triggers respiratory symptoms. We report the case of a 5-year-old girl hospitalized for a unilateral eosinophilic pleural effusion due to Toxocara canis. Besides the fact that she was living in a squat, no other medical condition was reported. There was no other site of infection caused by the parasite and she was successfully treated with albendazole. This case report is obviously unique as very few cases of pleural effusion due to Toxocara canis are reported in literature, all in adult patients.

  19. Pleural effusion: An unusual cause and association

    Directory of Open Access Journals (Sweden)

    Alam K Navaz

    2013-01-01

    Full Text Available Filaria has a wide spectrum of presentation. We hereby present a case of Filarial pleural effusion that is a rarity in itself. Filarial lung involvement is usually in the form of tropical pulmonary eosinophilia with pulmonary infiltrates and peripheral eosinophilia, unlike our case where isolated pleural effusion of Filarial etiology was detected. Microfilaria has been isolated from Pleural fluid in very few cases, and ours was one such. Of late, there have been many incidental detections of Filarial parasites from varied anatomical sites in association with malignancy. Even in our case, we had one such unusual association.

  20. Coexistent transient pulmonary edema and pericardial effusion

    Energy Technology Data Exchange (ETDEWEB)

    Newman, B.; Oh, K.S.; Park, S.C.

    1988-09-01

    Eight (23%) of 35 children with acute pericardial effusions due to infection or juvenile rheumatoid arthritis (JRA) had associated transient pulmonary edema demonstrated on plain chest radiographs. The presence or absence of radiographic pulmonary edema correlated well with clinical and hemodynamic parameters in patients with JRA but not in those with infectious pericarditis. There was no definite relationship between radiographic edema and amount of pericardial fluid as estimated echocardiographically or removed at pericardiocentesis. Rapidity of pericardial fluid accumulation could not be assessed in this study. Children of young age with underlying JRA were the most likely subjects to have radiographic pulmonary edema in conjunction with an acute pericardial effusion.

  1. Treatment Options by Stage (Uterine Sarcoma)

    Science.gov (United States)

    ... Cancer Prevention Endometrial Cancer Screening Research Uterine Sarcoma Treatment (PDQ®)–Patient Version General Information About Uterine Sarcoma ... Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery ) and treatment ...

  2. How Are Soft Tissue Sarcomas Diagnosed?

    Science.gov (United States)

    ... type of cancer or a benign disease. Several types of biopsies are used to diagnose sarcomas. Doctors experienced with ... But if FNA results suggest a sarcoma, another type of biopsy will usually be done to remove enough tissue ...

  3. General Information about Childhood Soft Tissue Sarcoma

    Science.gov (United States)

    ... forms in soft tissues of the body, including muscle, tendons, fat, blood vessels, lymph vessels, nerves, and tissue around joints. Soft tissue sarcoma occurs in children and adults. Soft ... disorders can increase the risk of childhood soft tissue sarcoma. Anything ...

  4. Stages of Childhood Soft Tissue Sarcoma

    Science.gov (United States)

    ... forms in soft tissues of the body, including muscle, tendons, fat, blood vessels, lymph vessels, nerves, and tissue around joints. Soft tissue sarcoma occurs in children and adults. Soft ... disorders can increase the risk of childhood soft tissue sarcoma. Anything ...

  5. Treatment Options for Childhood Soft Tissue Sarcoma

    Science.gov (United States)

    ... forms in soft tissues of the body, including muscle, tendons, fat, blood vessels, lymph vessels, nerves, and tissue around joints. Soft tissue sarcoma occurs in children and adults. Soft ... disorders can increase the risk of childhood soft tissue sarcoma. Anything ...

  6. Treatment Option Overview (Childhood Soft Tissue Sarcoma)

    Science.gov (United States)

    ... forms in soft tissues of the body, including muscle, tendons, fat, blood vessels, lymph vessels, nerves, and tissue around joints. Soft tissue sarcoma occurs in children and adults. Soft ... disorders can increase the risk of childhood soft tissue sarcoma. Anything ...

  7. Allergic rhinitis is associated with otitis media with effusion

    DEFF Research Database (Denmark)

    Kreiner-Møller, E; Chawes, B L K; Thomasen, Per Caye

    2012-01-01

    Childhood otitis media with effusion is a common disease and a link to allergic diseases has been suggested.......Childhood otitis media with effusion is a common disease and a link to allergic diseases has been suggested....

  8. Isolated posterior uveal effusion: expanding the spectrum of the uveal effusion syndrome

    Directory of Open Access Journals (Sweden)

    Pautler SE

    2014-12-01

    Full Text Available Scott E Pautler,1 David J Browning2 1Department of Ophthalmology, University of South Florida, Morsani College of Medicine, Tampa, FL, USA; 2Charlotte Ear Eye Nose and Throat Associates, Charlotte, NC, USA Abstract: Uveal effusion syndrome usually causes peripheral chorioretinal detachment, but posterior effusion may present as isolated macular edema with serous macular detachment in the setting of hyperopia and a thickened posterior choroid. Carbonic anhydrase inhibitors may be effective to treat this condition. Keywords: uveal effusion, serous, macular detachment, macular edema

  9. Metachronous cardiac and cerebral sarcomas: case report with focus on molecular findings and review of the literature.

    Science.gov (United States)

    Guerriero, Angela; Giovenali, Paolo; La Starza, Roberta; Mecucci, Cristina; Montesi, Giampaolo; Pasquino, Stefano; Pierini, Tiziana; Ragni, Temistocle; Sidoni, Angelo

    2015-03-01

    Although multiple primary malignancies are relatively rare, they have increased in frequency over the last decades, partly because of advances in diagnosis and therapy. This report describes for the first time the case of a patient with past occupational exposure to asbestos and no family history of cancer who developed 2 rare primary malignancies: a cardiac sarcoma and a gliosarcoma 11 months later. Molecular-cytogenetic studies did not identify common lesions to these 2 rare metachronous sarcomas. The gliosarcoma was associated with monosomy 10 and underlying PTEN monoallelic loss, which has been recurrently observed. In the cardiac sarcoma, MDM2 amplification and CDKN2AB/9p21 biallelic deletion suggested intimal sarcoma. No causal relationship was found between cardiac sarcoma and asbestos exposure, although MDM2 abnormalities were linked to malignant mesothelioma.

  10. Trabectedin in Soft Tissue Sarcomas

    Directory of Open Access Journals (Sweden)

    Bradley J. Petek

    2015-02-01

    Full Text Available Soft tissue sarcomas are a group of rare tumors derived from mesenchymal tissue, accounting for about 1% of adult cancers. There are over 60 different histological subtypes, each with their own unique biological behavior and response to systemic therapy. The outcome for patients with metastatic soft tissue sarcoma is poor with few available systemic treatment options. For decades, the mainstay of management has consisted of doxorubicin with or without ifosfamide. Trabectedin is a synthetic agent derived from the Caribbean tunicate, Ecteinascidia turbinata. This drug has a number of potential mechanisms of action, including binding the DNA minor groove, interfering with DNA repair pathways and the cell cycle, as well as interacting with transcription factors. Several phase II trials have shown that trabectedin has activity in anthracycline and alkylating agent-resistant soft tissue sarcoma and suggest use in the second- and third-line setting. More recently, trabectedin has shown similar progression-free survival to doxorubicin in the first-line setting and significant activity in liposarcoma and leiomyosarcoma subtypes. Trabectedin has shown a favorable toxicity profile and has been approved in over 70 countries for the treatment of metastatic soft tissue sarcoma. This manuscript will review the development of trabectedin in soft tissue sarcomas.

  11. Cerebral cysticercus granuloma associated with a subdural effusion.

    Directory of Open Access Journals (Sweden)

    Rajshekhar V

    2001-04-01

    Full Text Available The association of a solitary cerebral cysticercus granuloma with a subdural effusion is being reported. The granuloma and the effusion resolved following albendazole therapy. We speculate that the spread of the inflammatory changes around the granuloma to the subdural space could have led to the development of the subdural effusion.

  12. Recurrent pleural effusion as a clinical manifestation of multiple myeloma

    Directory of Open Access Journals (Sweden)

    Marcelo Torquato Toneline

    2013-12-01

    Multiple myeloma is a hematologic malignant tumor of plasma cells, sometimes associated with pleural effusion. This, in most cases, is associated to infectious complications. Pleural effusion as the onset or progression of the disease itself is rare. This case reports a young male, who presented recurrent pleural effusions, diagnosed with multiple myeloma at diagnosis.

  13. Evaluation of CT findings for diagnosis of pleural effusions

    Energy Technology Data Exchange (ETDEWEB)

    Arenas-Jimenez, J.; Alonso-Charterina, S.; Fernandez-Latorre, F.; Gil-Sanchez, S. [Hospital General Universitario de Alicante (Spain). Dept. of Radiology; Sanchez-Paya, J. [Hospital General Universitario de Alicante (Spain). Dept. of Preventive Medicine; Lloret-Llorens, M. [Hospital Universitario La Fe, Valencia (Spain). Dept. of Radiology

    2000-04-01

    Computed tomography studies are usually used to assess patients with pleural effusions, and radiologists should be aware of the significance of different CT findings for the diagnosis of the effusion. The purpose of this study was to evaluate CT findings for etiological diagnosis of pleural effusions. Contrast-enhanced CT of the chest of 211 patients with pleural effusion of definite diagnosis were evaluated. The CT images were evaluated for the presence and extent of pleural effusion, thickening or nodules, extrapleural fat and other changes in the mediastinum or lung. The CT scans were read by two independent observers and correlation between them was evaluated. Comparison of CT findings between benign and malignant effusions, between exudates and transudates, and between empyemas and the other parapneumonic effusions were carried out. Kappa values for most CT findings were >0.85. Loculation, pleural thickening, pleural nodules, and extrapleural fat of increased density were only present in exudative effusions. Multiple pleural nodules and nodular pleural thickening were the only pleural findings limited to malignant pleural effusions. The signs were also more frequently seen in empyemas than in other parapneumonic effusions. Computed tomography findings can help to distinguish between transudates and exudates. Although there is some overlap between benign and malignant pleural effusions, pleural nodules and nodular pleural thickening were present almost exclusively in the latter. Although differences between CT findings of empyemas and the other parapneumonic effusions exist, there is no finding which can definitely differentiate between them. (orig.)

  14. A Comprehensive Single Institutional Review of 2 Years in a Designated Fast-Track Sarcoma Diagnostic Clinic Linked with a Sarcoma Specialist Advisory Group: Meeting the Target but Failing the Task?

    Directory of Open Access Journals (Sweden)

    Zoltan Szucs

    2016-01-01

    Full Text Available Background. National guidelines prompted the implementation of a designated two-week wait referral pathway to facilitate the early diagnosis of sarcomas, to improve treatment outcomes. Methods. Patients referred to the Cambridge Sarcoma Diagnostic Clinic between January 2013 and December 2014 were identified through the electronic appointments system. Information was retrospectively retrieved about patient characteristics and details of the diagnostic pathway. Results. 17.3% of patients referred (69/397 were diagnosed with a malignancy. Of these, 59.3% (41/69 had primary sarcomas, 17.4% (12/69 had metastatic cancer, and 23.2% (16/69 had a different primary malignancy. 15% of the 41 sarcomas were 10 cm. Sarcomas diagnosed through this clinic represented 13% (41/315 of sarcomas managed at the centre during the same 2 years. Conclusion. While we achieved the target of 10% (41/397 sarcoma diagnosis rate in the rapid access clinic, only 15% of these were <5 cm better prognosis lesions. This calls into question the “real world” impact of such diagnostic clinics on early diagnosis of sarcomas. In order to enhance generic cancer diagnostic skills, training in these diagnostic clinics could be usefully integrated into national training curricula for both surgical and nonsurgical oncologists.

  15. Diaphragmatic hernia masquerading as pleural effusion

    Directory of Open Access Journals (Sweden)

    Z Nalladaru

    2012-01-01

    Full Text Available Rupture of the diaphragm is almost always due to major trauma. We present here an unusual and rare case of late presentation of diaphragmatic hernia after an innocuous injury. The patient was initially misdiagnosed as a left pleural effusion on the basis of chest X-ray and ultrasound findings. Finally, the diagnosis was confirmed on computerized scanning.

  16. Pericardial effusion in a mixed breed dog.

    OpenAIRE

    2001-01-01

    A 7-year-old, spayed female mixed breed dog was evaluated for labored breathing, lethargy, and a distended abdomen. Pericardial effusion was diagnosed after radiographic and echocardiographic interpretation. Treatment consisted of thoracocentesis and a single pericardiocentesis. Follow-up examinations indicate that the dog's condition has remained stable.

  17. Pleural fluid MDA and serum-effusion albumin gradient in pleural effusion

    OpenAIRE

    Mangaraj, Manaswini; Kumari, S.; Nanda, R; Pattnaik, M. R.; Mohapatra, P. C.

    2008-01-01

    Pleural fluid malondialdehyde (PMDA) and serum effusion albumin gradient(SEAG) were estimated in 60 patients of pleural effusion of diverse etiologies. The results were compared with Light’s criteria to distinguish between transudates and exudates. The mean PMDA level was 0.68±0.24nmol/ml and 1.17±0.25nmol/ml in transudates and exudates respectively showing a statistically significant (p

  18. Postirradiation Sarcoma: Clinicopathologic Features and Role of Chemotherapy in the Treatment Strategy

    Directory of Open Access Journals (Sweden)

    Gaetan des Guetz

    2009-01-01

    Full Text Available Purpose. An analysis of the clinicopathologic features and treatment of patients was performed to guide evaluation and management of postirradiation sarcoma. Patients and Methods. Between 1994 and 2001, 25 patients with postirradiation sarcoma were treated in one center with different chemotherapy, mainly in neoadjuvant setting (19. Tumors for which these patients received radiotherapy initially were mainly breast carcinoma (for 15 patients. The postirradiation sarcomas were of different histopathologic forms, most frequently osteosarcoma, leiomyosarcoma, and angiosarcoma. Results. Of the 25 patients, 19 were initially treated with chemotherapy. Nine of 19 pretreated patients achieved clinical partial response (RP = 47%. Leiomyosarcomas were good responders (3/4 and undifferentiated sarcoma (3/5. Responders were more often treated with MAID (6/8. Eight of the 9 responders underwent surgery. Two patients achieved complete histological response. Seven of the 9 good responders are alive with a median follow up of 24 months. For all treated patients, median follow up 24 months (6–84 months, overall survival and disease free survival were, respectively, 17/25 (68%, and 14/25 (56%. Conclusion. From our data, postirradiation sarcoma should not be managed differently from primary sarcoma. Chemotherapy has to be included in the treatment plan of postirradiation sarcoma, in future studies.

  19. Myofibroblastic sarcoma of the base of tongue. Case report and review of the literature

    Energy Technology Data Exchange (ETDEWEB)

    Takacsi-Nagy, Zoltan; Fodor, Janos [National Institute of Oncology, Budapest (Hungary). Dept. of Radiotherapy; Murakoezy, Gyoergyi [Szent Imre Hospital, Budapest (Hungary). Dept. of Otorhinolaryngology; Pogany, Peter [Szent Imre Hospital, Budapest (Hungary). Dept. of Pathology; Orosz, Zsolt [National Institute of Oncology, Budapest (Hungary). Dept. of Pathology

    2009-03-15

    Background: Mesenchymal malignancies with myofibroblastic differentiation exhibit a spectrum from low-grade myofibroblastic sarcoma mimicking fibromatosis to pleomorphic high-grade sarcoma. Low-grade myofibroblastic sarcoma shows a wide anatomic distribution with a predilection for the head-and-neck region; however, intermediate- and high-grade myofibroblastic sarcomas in this localization are exceptional. Case Report: A 56-year-old woman with intermediate-grade myofibroblastic sarcoma of the base of tongue is presented. She was treated with surgical excision, but computed tomography proved local residual tumor. Reexcision and chemotherapy were refused by the patient. Irradiation was given to a total dose of 66 Gy. Result: 50 months after completion of radiotherapy, the patient is in good health without any evidence of disease. According to the review of the literature, base of tongue as the primary site of myofibroblastic sarcoma has not been published so far. Conclusion: Similarly to the low-grade form, intermediate- and high-grade myofibroblastic sarcomas may also occur in the head-and-neck region. In case of incomplete excision, radiotherapy may be an effective treatment. (orig.)

  20. Epidemiology and therapies for metastatic sarcoma

    Directory of Open Access Journals (Sweden)

    Amankwah EK

    2013-05-01

    Full Text Available Ernest K Amankwah,1 Anthony P Conley,2 Damon R Reed2 1Department of Cancer Epidemiology, H Lee Moffitt Cancer Center and Research Institute, Tampa, FL, USA; 2Sarcoma Department, H Lee Moffitt Cancer Center and Research Institute, Tampa, FL, USA Abstract: Sarcomas are cancers arising from the mesenchymal layer that affect children, adolescents, young adults, and adults. Although most sarcomas are localized, many display a remarkable predilection for metastasis to the lungs, liver, bones, subcutaneous tissue, and lymph nodes. Additionally, many sarcoma patients presenting initially with localized disease may relapse at metastatic sites. While localized sarcomas can often be cured through surgery and often radiation, controversies exist over optimal management of patients with metastatic sarcoma. Combinations of chemotherapy are the most effective in many settings, and many promising new agents are under active investigation or are being explored in preclinical models. Metastatic sarcomas are excellent candidates for novel approaches with additional agents as they have demonstrated chemosensitivity and affect a portion of the population that is motivated toward curative therapy. In this paper, we provide an overview on the common sarcomas of childhood (rhabdomyosarcoma, adolescence, and young adults (osteosarcoma, Ewing sarcoma, synovial sarcoma, and malignant peripheral nerve sheath tumor and older adults (leiomyosarcoma, liposarcoma, and undifferentiated high grade sarcoma in terms of the epidemiology, current therapy, promising therapeutic directions and outcome with a focus on metastatic disease. Potential advances in terms of promising therapy and biologic insights may lead to more effective and safer therapies; however, more clinical trials and research are needed for patients with metastatic sarcoma. Keywords: chemotherapy, pediatric sarcoma, rhabdomyosarcoma, osteosarcoma, Ewing sarcoma, synovial sarcoma

  1. Skin Ultrasound in Kaposi Sarcoma.

    Science.gov (United States)

    Carrascosa, R; Alfageme, F; Roustán, G; Suarez, M D

    2016-05-01

    The use of ultrasound imaging has recently been increasing in numerous dermatologic diseases. This noninvasive technique provides additional details on the structure and vascularization of skin lesions. Kaposi sarcoma is a vascular tumor that typically arises in the skin and mucosas. It can spread to lymph nodes and internal organs. We performed B-mode and color Doppler ultrasound studies in 3 patients with a clinical diagnosis of Kaposi sarcoma confirmed by histological examination. We found differences in the ultrasound pattern between nodular and plaque lesions, in both B-mode and color Doppler. We believe that skin ultrasound imaging could be a useful technique for studying cutaneous Kaposi sarcoma, providing additional information on the structural and vascular characteristics of the lesion.

  2. Expression of soluble Toll-like receptors in pleural effusions

    Institute of Scientific and Technical Information of China (English)

    YANG Hai-bo; XIE Kai-qing; DENG Jing-min; QIN Shou-ming

    2010-01-01

    Background The Toll-like receptors (TLRs) represent a group of single-pass transmembrane receptors expressed on sentinel cells that are central to innate immune responses.The aim of this study was to investigate the presence of soluble TLRs in pleural effusions, and the diagnostic values of TLRs for pleural effusion with various etiologies.Methods Pleural effusion and serum samples were collected from 102 patients (36 with malignant pleural effusion, 36with tuberculous pleural effusion, 18 with bacterial pleural effusion, and 12 with transudative pleural effusion).The concentrations of TLR1 to TLR10 were determined in effusion and serum samples by enzyme linked immunosorbent assay.Four classical parameters (protein, lactate dehydrogenase, glucose and C-reactive protein (CRP)) in the pleural fluid were also assessed.Receiver-operating characteristic curves were used to assess the sensitivity and specificity of pleural fluid TLRs and biochemical parameters for differentiating bacterial pleural effusion.Results The concentrations of TLR1, TLR3, TLR4, TLR7 and TLR9 in bacterial pleural effusion were significantly higher than those in malignant, tuberculous, and transudative groups, respectively.Analysis of receiver operating characteristic curves revealed that the area under the curves of TLR1, TLR3, TLR4, TLR7 and TLR9 were 0.831, 0.843,0.842, 0.883 and 0.786, respectively, suggesting that these TLRs play a role in the diagnosis of bacterial pleural effusion.Also, the diagnostic value of TLRs for bacterial pleural effusions was much better than that of biochemical parameters (protein, lactate dehydrogenase, glucose and CRP).Conclusions The concentrations of TLR1, TLR3, TLR4, TLR7 and TLR9 appeared to be increased in bacterial pleural effusion compared to non-bacterial pleural effusions.Determination of these pleural TLRs may improve the ability of clinicians to differentiate pleural effusion patients of bacterial origin from those with other etiologies.

  3. Derrame pleural neoplásico Malignant pleural effusion

    Directory of Open Access Journals (Sweden)

    Lisete Ribeiro Teixeira

    2006-08-01

    Full Text Available O derrame pleural neoplásico é uma complicação freqüente nos pacientes portadores de tumores avançados. A presença de células malignas no líquido pleural ou na biópsia da pleura é indicativa de disseminação da doença primária, com conseqüente redução da expectativa de vida. O diagnóstico e tratamento precoce do derrame pleural maligno são fundamentais para promover uma melhor qualidade de vida aos pacientes portadores de câncer avançado.The malignant pleural effusion is a frequent complication in patients with of advanced tumors. The presence of malignant cells in the pleural fluid or in the pleural biopsy is indicative of dissemination of the primary disease, with consequent reduction of life expectancy. The early diagnosis and treatment of the malignant effusion is pivotal in promoting a better quality of life to patients with advanced cancer.

  4. Evaluation of pleural and pericardial effusions by magnetic resonance imaging

    Energy Technology Data Exchange (ETDEWEB)

    Tscholakoff, D.; Sechtem, U.; De Geer, G.; Schmidt, H.; Higgins, C.B.

    1987-08-01

    MR examinations of 36 patients with pleural and/or pericardial effusions were retrospectively evaluated. The purpose of this study was to determine of MR imaging is capable of differentiating between pleural and pericardial effusions of different compositions using standard electrocardiogram (ECG)-gated and nongated spin echo pulse sequences. Additional data was obtained from experimental pleural effusions in 10 dogs. The results of this study indicate that old haemorhages into the pleural or pericardial space can be differentiated from other pleural or pericardial effusions. However, further differentiation between transudates, exudates and sanguinous effusions is not possible on MR images acquired with standard spin echo pulse sequences. (orig./MG)

  5. Mast cell sarcoma: clinical management.

    Science.gov (United States)

    Weiler, Catherine R; Butterfield, Joseph

    2014-05-01

    Mast cell sarcoma is a disorder that results in abnormal mast cells as identified by morphology, special stains, and in some publications, c-kit mutation analysis. It affects animal species such as canines more commonly than humans. In humans it is a very rare condition, with variable clinical presentation. There is no standard therapy for the disorder. It can affect any age group. It is occasionally associated with systemic mastocytosis and/or urticaria pigmentosa. The prognosis of mast cell sarcoma in published literature is very poor in humans.

  6. Limb-sparing management with surgical resection, external-beam and intraoperative electron-beam radiation therapy boost for patients with primary soft tissue sarcoma of the extremity. A multicentric pooled analysis of long-term outcomes

    Energy Technology Data Exchange (ETDEWEB)

    Calvo, Felipe A. [Hospital General Universitario Gregorio Maranon, Department of Oncology, Madrid (Spain); Complutense University, School of Medicine, Madrid (Spain); Sole, Claudio V. [Hospital General Universitario Gregorio Maranon, Department of Oncology, Madrid (Spain); Complutense University, School of Medicine, Madrid (Spain); Instituto de Radiomedicina, Service of Radiation Oncology, Santiago (Chile); Polo, Alfredo; Montero, Angel [Hospital Universitario Ramon y Cajal, Service of Radiation Oncology, Madrid (Spain); Cambeiro, Mauricio; Martinez-Monge, Rafael [Clinica Universidad de Navarra, Service of Radiation Oncology, Pamplona (Spain); Alvarez, Ana [Hospital General Universitario Gregorio Maranon, Service of Radiation Oncology, Madrid (Spain); Cuervo, Miguel [Hospital General Universitario Gregorio Maranon, Service of Orthopedics and Traumatology, Madrid (Spain); Julian, Mikel San [Clinica Universidad de Navarra, Service of Orthopedics and Traumatology, Pamplona (Spain)

    2014-10-15

    A joint analysis of data from three contributing centres within the intraoperative electron-beam radiation therapy (IOERT) Spanish program was performed to investigate the main contributions of IORT to the multidisciplinary treatment of high-risk extremity soft tissue sarcoma (STS). Patients with an histologic diagnosis of primary extremity STS, with absence of distant metastases, undergoing limb-sparing surgery with radical intent, external beam radiotherapy (median dose 45 Gy) and IOERT (median dose 12.5 Gy) were considered eligible for participation in this study. From 1986-2012, a total of 159 patients were analysed in the study from three Spanish institutions. With a median follow-up time of 53 months (range 4-316 years), 5-year local control (LC) was 82 %. The 5-year IOERT in-field control, disease-free survival (DFS) and overall survival (OS) were 86, 62 and 72 %, respectively. On multivariate analysis, only microscopically involved margin (R1) resection status retained significance in relation to LC (HR 5.20, p < 0.001). With regard to IOERT in-field control, incomplete resection (HR 4.88, p = 0.001) and higher IOERT dose (≥ 12.5 Gy; HR 0.32, p = 0.02) retained a significant association in multivariate analysis. From this joint analysis emerges the fact that an IOERT dose ≥ 12.5 Gy increases the rate of IOERT in-field control, but DFS remains modest, given the high risk of distant metastases. Intensified local treatment needs to be tested in the context of more efficient concurrent, neo- and adjuvant systemic therapy. (orig.) [German] Um den therapeutischen Beitrag einer intraoperativen Bestrahlung mit Elektronen (IOERT) als Teil eines multidisziplinaeren Behandlungskonzepts von Weichteilsarkomen (STS) im Extremitaetenbereich mit hohem Risikoprofil evaluieren zu koennen, wurde anhand des spanischen IOERT-Programms eine gepoolte Datenanalyse von drei teilnehmenden Zentren vorgenommen. Eingeschlossen in diese Studie wurden Patienten mit histologisch

  7. Poorly Differentiated Uterine or Cervical Sarcoma in a Young Dog

    Directory of Open Access Journals (Sweden)

    Michelle C. Cora

    2011-01-01

    Full Text Available A 1.5 year old, female, spayed, Labrador retriever with a history of three abdominal surgeries within the previous two months presented to the North Carolina State University Veterinary Teaching Hospital for evaluation of a pelvic inlet mass causing fecal tenesmus, obstipation, and dysuria. Abdominal ultrasound revealed a caudal abdominal mass extending into the pelvic cavity. Cytologic evaluation of the mass showed a pleomorphic round to fusiform cell population with histiocytic and suppurative inflammation. The primary differential was neoplasia, but inflammation with cellular pleomorphism could not be excluded. Via histopathology and immunohistochemistry, a diagnosis of poorly differentiated sarcoma originating from the uterus or cervix with widespread intra-abdominal dissemination and metastasis was made. Sarcomas of any type are rare in young dogs with only sporadic cases of poorly or undifferentiated sarcomas reported. This case is a unique presentation of an aggressive, poorly differentiated sarcoma arising from the cervix or uterus in a young dog and illustrates the importance of histologic evaluation of surgically resected tissues that are abnormal in appearance.

  8. Utility of semi-rigid thoracoscopy in undiagnosed exudative pleural effusion

    Directory of Open Access Journals (Sweden)

    Loganathan Nattusamy

    2015-01-01

    Full Text Available Background: Semi-rigid thoracoscopy is a safe and efficacious procedure in patients with undiagnosed pleural effusion. Literature on its utility from developing countries is limited. We herein describe our initial experience on the utility of semi-rigid thoracoscopy from a tertiary care teaching and referral center in north India. We also perform a systematic review of studies reporting the utility of semi-rigid thoracoscopy from India. Patients and Methods: The primary objective was to evaluate the diagnostic utility of semi-rigid thoracoscopy in patients with undiagnosed exudative pleural effusion. Semi-rigid thoracoscopy was performed under local anesthesia and conscious sedation in the bronchoscopy suite. Results: A total of 48 patients underwent semi-rigid thoracoscopy between August 2012 and December 2013 for undiagnosed pleural effusion. Mean age was 50.9 ± 14.1 years (range: 17-78 years. Pre-procedure clinico-radiological diagnoses were malignant pleural effusion [36 patients (75%], tuberculosis (TB [10 (20.83% patients], and empyema [2 patients (4.17%]. Patients with empyema underwent the procedure for pleural biopsy, optimal placement of intercostal tube and adhesiolysis. Thoracoscopic pleural biopsy diagnosed pleural malignancy in 30 (62.5% patients and TB in 2 (4.17% patients. Fourteen (29.17% patients were diagnosed with non-specific pleuritis and normal pleura was diagnosed on a pleural biopsy in 2 (4.17% patients. Overall, a definitive diagnosis of either pleural malignancy or TB was obtained in 32 (66.7% patients. Combined overall sensitivity, specificity, positive predictive value and negative predictive value of thoracoscopic pleural biopsy for malignant pleural effusion were 96.77%, 100%, 100% and 66.67%, respectively. There was no procedure-related mortality. On performing a systematic review of literature, four studies on semi-rigid thoracoscopy from India were identified. Conclusion: Semi-rigid thoracoscopy is a safe and

  9. [A case of undifferentiated (embryonal) liver sarcoma mimicking klatskin tumor in an adult].

    Science.gov (United States)

    Lee, Ji Ae; Kim, Tae Wan; Min, Jae Hoon; Byon, Sun Jung; Jang, Seung Hoon; Choi, Seung Yeon; Kim, Hong Joo

    2010-02-01

    Undifferentiated sarcoma is an uncommon primary malignant tumor of the liver typically occurring in older children. It is also referred to as malignant mesenchymoma, fibromyxosarcoma, or mesenchymal sarcoma. We experienced a case of undifferentiated sarcoma in 72-year-old male. Contrast enhanced liver CT scan revealed a 3.4 cm heterogeneously enhancing, ill-defined, and low attenuated mass in the left liver and subtle intrahepatic duct dilatation. And, in tubogram, there were segmental stenosis and occlusion from the hilum to the proximal common bile duct. We did ultrasonography guided liver biopsy. The pathologic finding revealed infiltrative growth of atypical cells with rhabdoid features. Some atypical cells showed clear cytoplasm, but no organoid pattern was identified. The stroma around atypical cells was filled with eosinophilic hyaline material. These tumor cells were positive for vimentin only, and the tumor was consistent with undifferentiated sarcoma of the liver.

  10. Undifferentiated pleomorphic sarcoma with osteoclast-like giant cells of the female breast

    Directory of Open Access Journals (Sweden)

    Balbi Giancarlo

    2013-01-01

    Full Text Available Abstract The authors describe a case of undifferentiated pleomorphic sarcoma of the breast occurring in a 50-year-old woman who presented with a palpable mass in her right breast. She first noticed the mass one month previously. Core needle biopsy showed connective tissue including epithelioid and spindle cells. The patient underwent total mastectomy without axillary lymph node dissection. Based on examination of the excised tumor, the initial pathologic diagnosis was atypical spindle-shaped and ovoid cells with uncertain malignant potential. Histological findings with immunomarkers led to the final diagnosis of undifferentiated pleomorphic sarcoma. This case highlights a rare and interesting variant of primary breast sarcoma and the important role of immunohistochemistry in defining histological type and differential diagnosis. Hence, undifferentiated pleomorphic sarcoma has been a diagnosis of exclusion performed through sampling and critical use of ancillary diagnostic techniques.

  11. The challenges of detecting circulating tumour cells in sarcoma

    OpenAIRE

    Tellez-Gabriel, M.; Brown, H K; Young, R.; Heymann, M. F.; Heymann, D

    2016-01-01

    International audience; Sarcomas are a heterogenous group of malignant neoplasms of mesenchymal origin, many of which have a propensity to develop distant metastases. Cancer cells that have escaped from the primary tumour are able to invade into surrounding tissues, to intravasate into the bloodstream to become Circulating Tumour Cells (CTCs), and are responsible for the generation of distant metastases. Due to the rarity of these tumours and the absence of specific markers expressed by sarco...

  12. Anterior mediastinal synovial sarcoma: A case report and literature review

    Directory of Open Access Journals (Sweden)

    Wen-xiang YUE

    2015-01-01

    Full Text Available Objective To study the clinical manifestations, pathologic features, diagnosis, treatment and prognosis of primary synovial sarcoma in the anterior mediastinum. Methods A case of primary synovial sarcoma in the anterior mediastinum was reported. Clinical features, imaging manifestations, pathology features and therapeutic effect were analysed and the relevant literature was reviewed. Results A 48-year-male patient was admitted with complaint of right chest pain for 4 days. Chest computerized tomography revealed a large mass located at the right anterior mediastinum, and it was primarily diagnosed as invasive thymoma. Pathological examination by CT-guided percutaneous needle biopsy manifested that, under microscope, the tumor cells were short and spindle in shape forming a nest structure, suggested it was a thymoma. The patient then underwent resection of thymoma with removal of fat and connective tissue in the anterior mediastinum. During the operation the size of the tumor was 15cm×15cm×10cm, being located at the anterior mediastinum, and it tended to bleed. The diagnosis of primary monophasic synovial sarcoma in the mediastinum was confirmed by postoperative/pathology examination. Immunohistochemistry staining showed that the tumor cells were positive for the markers Bcl-2 and EMA, but negative for the markers CK (pan and S100. The patient suffered from local recurrence with metastases to lung 4 months after surgery. The patient received 2 chemotherapeutic courses with ifosfamide, epirubicin and cisplatin. He died 6 months after surgery. Conclusion Primary synovial sarcoma in the anterior mediastinum is an extremely rare and highly malignant tumor with poor prognosis. The diagnosis depends on the pathological features, immunohistochemistry and RT-PCR. Radical resection combined with comprehensive treatment may improve the survival rate. DOI: 10.11855/j.issn.0577-7402.2014.12.12

  13. Treatment Options for Kaposi Sarcoma

    Science.gov (United States)

    ... and its treatment, see the AIDSinfo website . Nonepidemic Gay-related Kaposi Sarcoma There is a type of ... better than another. Trials are based on past studies and what has been learned ... by their creator. In such cases, it is necessary to contact the writer, artist, ...

  14. General Information about Kaposi Sarcoma

    Science.gov (United States)

    ... and its treatment, see the AIDSinfo website . Nonepidemic Gay-related Kaposi Sarcoma There is a type of ... better than another. Trials are based on past studies and what has been learned ... by their creator. In such cases, it is necessary to contact the writer, artist, ...

  15. Kaposi sarcoma associated with lipoedema.

    Science.gov (United States)

    Ekmekci, T R; Ayabakan, O; Sakiz, D; Koslu, A

    2005-05-01

    Lipoedema is a form of lipodistrophy, which consists of abnormal accumulation of fat in subcutaneous tissue of the lower limbs. It does not cause any disease and it has not been reported association with malignity. We describe a 63-year-old woman occurring of Kaposi sarcoma on the lipoedema base.

  16. Selective peptide inhibitors of antiapoptotic cellular and viral Bcl-2 proteins lead to cytochrome c release during latent Kaposi's sarcoma-associated herpesvirus infection.

    Science.gov (United States)

    Burrer, Christine M; Foight, Glenna W; Keating, Amy E; Chan, Gary C

    2016-01-04

    Kaposi's sarcoma-associated herpesvirus (KSHV) is associated with B-cell lymphomas including primary effusion lymphoma and multicentric Castleman's disease. KSHV establishes latency within B cells by modulating or mimicking the antiapoptotic Bcl-2 family of proteins to promote cell survival. Our previous BH3 profiling analysis, a functional assay that assesses the contribution of Bcl-2 proteins towards cellular survival, identified two Bcl-2 proteins, cellular Mcl-1 and viral KsBcl-2, as potential regulators of mitochondria polarization within a latently infected B-cell line, Bcbl-1. In this study, we used two novel peptide inhibitors identified in a peptide library screen that selectively bind KsBcl-2 (KL6-7_Y4eK) or KsBcl-2 and Mcl-1 (MS1) in order to decipher the relative contribution of Mcl-1 and KsBcl-2 in maintaining mitochondrial membrane potential. We found treatment with KL6-7_Y4eK and MS1 stimulated a similar amount of cytochrome c release from mitochondria isolated from Bcbl-1 cells, indicating that inhibition of KsBcl-2 alone is sufficient for mitochondrial outer membrane permiabilzation (MOMP) and thus apoptosis during a latent B cell infection. In turn, this study also identified and provides a proof-of-concept for the further development of novel KsBcl-2 inhibitors for the treatment of KSHV-associated B-cell lymphomas via the targeting of latently infected B cells.

  17. A European project on incidence, treatment, and outcome of sarcoma

    Directory of Open Access Journals (Sweden)

    Frasson Alvise

    2010-04-01

    Full Text Available Abstract Background Sarcomas are rare tumors (1-2% of all cancers of mesenchymal origin that may develop in soft tissues and viscera. Since the International Classification of Disease (ICD attributes visceral sarcomas (VS to the organ of origin, the incidence of sarcoma is grossly underestimated. The rarity of the disease and the variety of histological types (more than 70 or locations account for the difficulty in acquiring sufficient personal experience. In view of the above the European Commission funded the project called Connective Tissues Cancers Network (CONTICANET, to improve the prognosis of sarcoma patients by increasing the level of standardization of diagnostic and therapeutic procedures through a multicentre collaboration. Methods/Design Two protocols of epidemiological researches are here presented. The first investigation aims to build the population-based incidence of sarcoma in a two-year period, using the new 2002 WHO classification and the "second opinion" given by an expert regional pathologist on the initial diagnosis by a local pathologist. A three to five year survival rate will also be determined. Pathology reports and clinical records will be the sources of information. The second study aims to compare the effects on survival or relapse-free period - allowing for histological subtypes, clinical stage, primary site, age and gender - when the disease was treated or not according to the clinical practice guidelines (CPGs. Discussion Within CONTICANET, each group was asked to design a particular study on a specific objective, the partners of the network being free to accept or not the proposed protocol. The first protocol was accepted by the other researchers, therefore the incidence of sarcoma will be assessed in three European regions, Rhone-Alpes and Aquitaine (France and Veneto (Italy, where the geographic distribution of sarcoma will be compared after taking into account age and gender. The conformity of the clinical

  18. A case of tuberculous pericardial effusion.

    Science.gov (United States)

    Suman, Appukuttan; Sarin, Jawahar L; Grant, Simon C; Bazaraa, Talal A

    2003-07-01

    We report a case of an 80-year-old caucasian female in the UK who presented with weight loss and was found to have a pericardial effusion. There was neither previous exposure to tuberculosis nor any suggestion of immunosuppression. Repeated analysis of pericardial fluid established a tuberculous origin. Search of medical literature did not reveal any similar cases in the elderly in the UK.

  19. Effusion in magnetic resonance imaging of the temporomandibular joint

    Energy Technology Data Exchange (ETDEWEB)

    Nah, Kyung Soo [Pusan National University College of Medicine, Busan (Korea, Republic of)

    2003-03-15

    The purpose of this study was to investigate the distribution and frequency of temporomandibular joint (TMJ) effusion in magnetic resonance (MR) images of patients with disc displacements. On T2 weighted MR images of 148 TMJs taken from 74 patients presenting with TMJ pain and dysfunction, we assessed the cases showing TMJ effusion, defined as an amount of fluid that exceeded the maximum amount seen in a control group of asymptomatic volunteers. The amount of TMJ fluid was graded as: I (none or minimal), II (moderate), III (marked), and IV (extensive), according to a standard set by a reference. Disc displacement categories were also recorded. Of the 148 TMJs examined in this study, 52 joints (35.1%) presented with joint effusion, 24 (16.2%) showing bilateral joint effusion. 38 joints showed upper joint space effusion, and 3 showed lower joint space effusion, and 11 showed both upper and lower joint space effusion. 96 joints (64.9%) had grade I joint fluid, 27 (18.2%) grade II, 15 (10.1%) grade III, and 10 (6.8%) grade IV. 80.0% of the joints presenting with grade IV effusion showed disc displacement without reduction. Joint effusion was found not only in upper, but also in lower joint spaces. The higher the effusion grade, the greater the frequency of disc displacement without reduction.

  20. MR diagnosis of temporomandibular joint. A study of joint effusion

    Energy Technology Data Exchange (ETDEWEB)

    Kaneda, Takashi; Yamashiro, Mitsuaki; Ozawa, Kaoru; Suzuki, Hiromi; Okada, Hiroyuki; Yamamoto, Hirotsugu [Nihon Univ., Matsudo, Chiba (Japan). School of Dentistry

    1998-03-01

    The purposes of this study were to evaluate the relationship between correlation of MR joint effusion of the temporomandibular joint and disk position, to evaluate the relationship between joint effusion and aging, and to assess the frequency of MR joint effusion of bilateral temporomandibular joints. The temporomandibular joints of 192 patients with clinical symptoms of temporomandibular joint disorders were imaged bilaterally using high field, surface-coil MR imaging. Oblique sagittal and coronal proton density-weighted and T2-weighted images were obtained. Imaging findings of joint effusion were correlated with disk position, aging, and bilateral temporomandibular joints. MR showed effusion in 4% of the joints with normal superior disk position, 36% of the joints with disk displacement with reduction, and 45% of the joints with disk displacement without reduction. There were significant differences in the incidence of joint effusion between normal disk position and anterior disk displacement with or without reduction. Younger patients less than 40 years were significant higher the incidence of joint effusion than those of older patients. A significant association was seen between joint effusion and aging. MR showed effusion in 17% of the unilateral temporomandibular joint, 24% of the bilateral temporomandibular joints. There was no significant difference between unilateral and bilateral case. These results indicated that joint effusion using MR imaging was associated with varied temporomandibular joint pathologic states. (author)

  1. Bicavitary effusion secondary to liver lobe torsion in a dog

    Directory of Open Access Journals (Sweden)

    Khan Z

    2016-04-01

    Full Text Available Zaheda Khan,1 Kathryn Gates,2 Stephen A Simpson,31Emergency and Critical Care, Animal Specialty and Emergency Center, Los Angeles, CA, 2Emergency and Critical Care, Advanced Critical Care, Emergency and Specialty Services, Culver City, CA 3Emergency and Critical Care, Southern California Veterinary Specialty Hospital, Irvine, CA, USA Abstract: We described the diagnosis and successful treatment of pleural and peritoneal effusion secondary to liver lobe torsion in a dog. A 12-year-old female spayed Borzoi dog was referred for heart failure. Emergency room thoracic and abdominal ultrasound showed a large volume of pleural effusion with mild peritoneal effusion and an abdominal mass. Pleural fluid analysis classified the effusion as exudative. A complete ultrasound revealed mild peritoneal effusion and decreased blood flow to the right liver lobe. Other causes of bicavitary effusion were ruled out based on blood work, ultrasound, echocardiogram, and computed tomography. The patient was taken to surgery and diagnosed with caudate liver lobe torsion and had a liver lobectomy. At the 2-week postoperative recheck, the patient was doing well and there was complete resolution of the pleural effusion. Liver lobe torsion is a rare occurrence in dogs and can be difficult to diagnose. Clinical signs are nonspecific for liver lobe torsion and patients may present in respiratory distress with significant pleural fluid accumulation. When assessing patients with pleural and peritoneal effusion, liver lobe torsion should be considered as a differential diagnosis.Keywords: pleural effusion, peritoneal effusion, hepatic torsion

  2. MASSIVE PLEURAL EFFUSION: A CASE REPORT

    Directory of Open Access Journals (Sweden)

    Putu Bayu Dian Tresna Dewi

    2013-03-01

    Full Text Available Pleural effusion is abnormal fluid accumulation within pleural cavity between the parietal pleura and visceralis pleura, either transudation or exudates. A 47 year-old female presented with dyspneu, cough, and decreased of appetite. She had history of right lung tumor. Physical examination revealed asymmetric chest movement where right part of lung was lagged during breathing, vocal fremitus on the right chest was decreased, dullness at the right chest, decreased vesicular sound in the right chest, enlargement of supraclavicular and colli dextra lymph nodes, and hepatomegali. Complete blood count showed leukocytosis. Clinical chemistry analysis showed hipoalbumin and decreased liver function. Blood gas analysis showed hypoxemia. Pleural fluid analysis showed an exudates, murky red liquid color filled with erythrocytes, number of cells. Cytological examination showed existence of a non-small cell carcinoma tends adeno type. From chest X-ray showed massive right pleural effusion. Based on history, physical examination and investigations, she was diagnosed with massive pleural effusion et causa suspected malignancy. She had underwent pleural fluid evacuation and treated with analgesics and antibiotics.

  3. Clinical Investigation of Benign Asbestos Pleural Effusion.

    Science.gov (United States)

    Fujimoto, Nobukazu; Gemba, Kenichi; Aoe, Keisuke; Kato, Katsuya; Yokoyama, Takako; Usami, Ikuji; Onishi, Kazuo; Mizuhashi, Keiichi; Yusa, Toshikazu; Kishimoto, Takumi

    2015-01-01

    There is no detailed information about benign asbestos pleural effusion (BAPE). The aim of the study was to clarify the clinical features of BAPE. The criteria of enrolled patients were as follows: (1) history of asbestos exposure; (2) presence of pleural effusion determined by chest X-ray, CT, and thoracentesis; and (3) the absence of other causes of effusion. Clinical information was retrospectively analysed and the radiological images were reviewed. There were 110 BAPE patients between 1991 and 2012. All were males and the median age at diagnosis was 74 years. The median duration of asbestos exposure and period of latency for disease onset of BAPE were 31 and 48 years, respectively. Mean values of hyaluronic acid, adenosine deaminase, and carcinoembryonic antigen in the pleural fluid were 39,840 ng/mL, 23.9 IU/L, and 1.8 ng/mL, respectively. Pleural plaques were detected in 98 cases (89.1%). Asbestosis was present in 6 (5.5%) cases, rounded atelectasis was detected in 41 (37.3%) cases, and diffuse pleural thickening (DPT) was detected in 30 (27.3%) cases. One case developed lung cancer (LC) before and after BAPE. None of the cases developed malignant pleural mesothelioma (MPM) during the follow-up.

  4. Clinical Investigation of Benign Asbestos Pleural Effusion

    Directory of Open Access Journals (Sweden)

    Nobukazu Fujimoto

    2015-01-01

    Full Text Available There is no detailed information about benign asbestos pleural effusion (BAPE. The aim of the study was to clarify the clinical features of BAPE. The criteria of enrolled patients were as follows: (1 history of asbestos exposure; (2 presence of pleural effusion determined by chest X-ray, CT, and thoracentesis; and (3 the absence of other causes of effusion. Clinical information was retrospectively analysed and the radiological images were reviewed. There were 110 BAPE patients between 1991 and 2012. All were males and the median age at diagnosis was 74 years. The median duration of asbestos exposure and period of latency for disease onset of BAPE were 31 and 48 years, respectively. Mean values of hyaluronic acid, adenosine deaminase, and carcinoembryonic antigen in the pleural fluid were 39,840 ng/mL, 23.9 IU/L, and 1.8 ng/mL, respectively. Pleural plaques were detected in 98 cases (89.1%. Asbestosis was present in 6 (5.5% cases, rounded atelectasis was detected in 41 (37.3% cases, and diffuse pleural thickening (DPT was detected in 30 (27.3% cases. One case developed lung cancer (LC before and after BAPE. None of the cases developed malignant pleural mesothelioma (MPM during the follow-up.

  5. Management of Parapneumonic Pleural Effusion in Adults.

    Science.gov (United States)

    Ferreiro, Lucía; San José, María Esther; Valdés, Luis

    2015-12-01

    Pleural infections have high morbidity and mortality, and their incidence in all age groups is growing worldwide. Not all infectious effusions are parapneumonic and, in such cases, the organisms found in the pleural space are not the same as those observed in lung parenchyma infections. The diagnostic difficulty lies in knowing whether an infectious effusion will evolve into a complicated effusion/empyema, as the diagnostic methods used for this purpose provide poor results. The mainstays of treatment are to establish an early diagnosis and to commence an antibiotic regimen and chest drain as soon as possible. This should preferably be carried out with fine tubes, due to certain morphological, bacteriological and biochemical characteristics of the pleural fluid. Fluid analysis, particularly pH, is the most reliable method for assessing evolution. In a subgroup of patients, fibrinolytics may help to improve recovery, and their combination with DNase has been found to obtain better results. If medical treatment fails and surgery is required, video-assisted thoracoscopic surgery (VATS) is, at least, comparable to decortication by thoracotomy, so should only undertaken if previous techniques have failed. Further clinical trials are needed to analyze factors that could affect the results obtained, in order to define new evidence-based diagnostic and therapeutic strategies that provide more effective, standardized management of this disease. Copyright © 2014 SEPAR. Published by Elsevier Espana. All rights reserved.

  6. Pazopanib in advanced vascular sarcomas: an EORTC Soft Tissue and Bone Sarcoma Group (STBSG) retrospective analysis

    NARCIS (Netherlands)

    Kollar, A.; Jones, R.L.; Stacchiotti, S.; Gelderblom, H.; Guida, M.; Grignani, G.; Steeghs, N.; Safwat, A.; Katz, D.; Duffaud, F.; Sleijfer, S.; Graaf, W.T. van der; Touati, N.; Litiere, S.; Marreaud, S.; Gronchi, A.; Kasper, B.

    2017-01-01

    BACKGROUND: Pazopanib is a multitargeted tyrosine kinase inhibitor approved for the treatment of patients with selective subtypes of advanced soft tissue sarcoma (STS) who have previously received standard chemotherapy including anthracyclines. Data on the efficacy in vascular sarcomas are limited.

  7. Primary osteosarcoma of the breast: case report

    OpenAIRE

    Saber, Boutayeb; Nawal, Ahbeddou; Mohamed, Fetohi; Hassan, Errihani

    2008-01-01

    Mammary sarcomas are very uncommon and make up less than 1% of all primary breast malignancies. Primary osteosarcoma of the breast is extremely rare and represents 12.5% of mammary sarcomas. A secondary lesion from a primary osteosarcoma of the bone should be considered in the differential diagnosis. In addition, the absence of a direct connection between the tumour and the underlying skeleton is mandatory for the diagnosis. We report a case of primary osteosarcoma of the breast occurring in ...

  8. Pleural Fluid Pentraxin-3 for the Differential Diagnosis of Pleural Effusions

    Science.gov (United States)

    Yeo, Chang Dong; Kim, Jin Woo; Cho, Mi Ran; Kang, Ji Young; Kim, Young Kyoon; Lee, Sang Haak; Park, Chan Kwon; Kim, Sang Ho; Park, Mi Sun; Yim, Hyeon Woo; Park, Jong Y.

    2013-01-01

    Background Conventional biomarkers cannot always establish the cause of pleural effusions; thus, alternative tests permitting rapid and accurate diagnosis are required. The primary aim of this study is to assess the ability of pentraxin-3 (PTX3) in order to diagnose the cause of pleural effusion and compare its efficacy to that of other previously identified biomarkers. Methods We studied 118 patients with pleural effusion, classified as transudates and exudates including malignant, tuberculous, and parapneumonic effusions (MPE, TPE, and PPE). The levels of PTX3, C-reactive protein (CRP), procalcitonin (PCT) and lactate in the pleural fluid were assessed. Results The levels of pleural fluid PTX3 were significantly higher in patients with PPE than in those with MPE or TPE. PTX3 yielded the most favorable discriminating ability to predict PPE from MPE or TPE by providing the following: area under the curve, 0.74 (95% confidence interval, 0.63-0.84), sensitivity, 62.07%; and specificity, 81.08% with a cut-off point of 25.00 ng/mL. Conclusion Our data suggests that PTX3 may allow improved differentiation of PPE from MPE or TPE compared to the previously identified biomarkers CRP and PCT. PMID:24416055

  9. Establishment of a human herpes virus-8-negative malignant effusion lymphoma cell line (STR-428) carrying concurrent translocations of BCL2 and c-MYC genes.

    Science.gov (United States)

    Taira, Tamiko; Nagasaki, Akitoshi; Tomoyose, Takeaki; Miyagi, Jun-ichi; Kakazu, Naoki; Makino, Shigeyoshi; Shinjyo, Tetsuharu; Taira, Naoya; Masuda, Masato; Takasu, Nobuyuki

    2007-09-01

    A new cell line, STR-428 was established from ascites tumor cells of a malignant effusion lymphoma patient without human herpes virus-8 (HHV-8) infection. STR-428 cells showed an immunophenotype of mature B-cells and produced few cytokines related to lymphomatous effusion. Karyotypic and genetic analysis revealed complex translocations including t(14;18)(q32;q21) effecting IgH/BCL2 and der(8)t(3;8)(q27;q24) involving c-MYC. STR-428 represents a unique, B-cell lymphoma cell line carrying concurrent rearrangement of BCL2 and c-MYC genes with features distinct from those of HHV-8-related primary effusion lymphoma. This cell line may be a valuable tool, other than HHV-8, to investigate the pathogenesis of primary lymphomatous effusion.

  10. Ribociclib and Doxorubicin in Treating Patients With Metastatic or Advanced Soft Tissue Sarcomas That Cannot Be Removed by Surgery

    Science.gov (United States)

    2017-04-10

    Metastatic Angiosarcoma; Metastatic Epithelioid Sarcoma; Metastatic Fibrosarcoma; Metastatic Leiomyosarcoma; Metastatic Liposarcoma; Metastatic Malignant Peripheral Nerve Sheath Tumor; Metastatic Synovial Sarcoma; Metastatic Undifferentiated Pleomorphic Sarcoma; Myxofibrosarcoma; Pleomorphic Rhabdomyosarcoma; Stage III Soft Tissue Sarcoma; Stage IV Soft Tissue Sarcoma; Undifferentiated (Embryonal) Sarcoma

  11. Potential Therapeutic Targets in Uterine Sarcomas

    OpenAIRE

    2015-01-01

    Uterine sarcomas are rare tumors accounting for 3,4% of all uterine cancers. Even after radical hysterectomy, most patients relapse or present with distant metastases. The very limited clinical benefit of adjuvant cytotoxic treatments is reflected by high mortality rates, emphasizing the need for new treatment strategies. This review summarizes rising potential targets in four distinct subtypes of uterine sarcomas: leiomyosarcoma, low-grade and high-grade endometrial stromal sarcoma, and undi...

  12. Head and Neck Soft Tissue Sarcoma

    OpenAIRE

    Aljabab, A. S.; Nason, R. W.; Kazi, R; Pathak, K. A.

    2011-01-01

    Sarcomas are malignant neoplasms originating from mesodermal tissues and constitute less than 1% of body’s tumors, including those of the head and neck region. 5–15% of adult sarcomas are in the head and neck region (20% from bones and cartilages and 80% in soft tissues). Commonly encountered sarcomas in the head and neck region are - osteosarcoma, rhabdomyosarcoma, malignant fibrous histiocytoma, fibrosarcoma and angiosarcoma. This article reviews the available literature on head and neck sa...

  13. Epidemiology and therapies for metastatic sarcoma

    OpenAIRE

    Amankwah EK; Conley AP; Reed DR

    2013-01-01

    Ernest K Amankwah,1 Anthony P Conley,2 Damon R Reed2 1Department of Cancer Epidemiology, H Lee Moffitt Cancer Center and Research Institute, Tampa, FL, USA; 2Sarcoma Department, H Lee Moffitt Cancer Center and Research Institute, Tampa, FL, USA Abstract: Sarcomas are cancers arising from the mesenchymal layer that affect children, adolescents, young adults, and adults. Although most sarcomas are localized, many display a remarkable predilection for metastasis to the lungs, liver, bones, subc...

  14. EWS/FLI1 Target Genes and Therapeutic Opportunities in Ewing Sarcoma

    Science.gov (United States)

    Cidre-Aranaz, Florencia; Alonso, Javier

    2015-01-01

    Ewing sarcoma is an aggressive bone malignancy that affect children and young adults. Ewing sarcoma is the second most common primary bone malignancy in pediatric patients. Although significant progress has been made in the treatment of Ewing sarcoma since it was first described in the 1920s, in the last decade survival rates have remained unacceptably invariable, thus pointing to the need for new approaches centered in the molecular basis of the disease. Ewing sarcoma driving mutation, EWS–FLI1, which results from a chromosomal translocation, encodes an aberrant transcription factor. Since its first characterization in 1990s, many molecular targets have been described to be regulated by this chimeric transcription factor. Their contribution to orchestrate Ewing sarcoma phenotype has been reported over the last decades. In this work, we will focus on the description of a selection of EWS/FLI1 targets, their functional role, and their potential clinical relevance. We will also discuss their role in other types of cancer as well as the need for further studies to be performed in order to achieve a broader understanding of their particular contribution to Ewing sarcoma development. PMID:26258070

  15. Soft tissue reconstruction after lower extremity limb-sparing pediatric sarcoma resection

    Institute of Scientific and Technical Information of China (English)

    Kevin Shultz; Nicholas Webster; Miguel A Soto-Miranda; Anas Eid; Jon P Ver Halen

    2016-01-01

    Aim:Limb salvage is the treatment of choice for lower extremity bone sarcomas in children. To date, peers have not described algorithms for soft tissue reconstruction of these defects. This paper is to report a large single center series of lower extremity salvage after sarcoma treatment, with algorithm. Methods:The authors performed a retrospective review of patients undergoing resection of lower extremity bone sarcomas at a single center over 12 years. Results:In total, 65 children (29 girls, 36 boys) with a mean age of 13 years (range 2.9-23.3 years) underwent resection of a lower leg sarcoma with limb-salvage. Tumors types included 50 osteosarcomas, and 15 Ewing sarcoma family of tumors. The types of reconstruction utilized included:34 primary closures, 22 gastrocnemius and soleus lfaps, 3 bipedicled lfaps, 2 sural artery lfaps, 1 pedicled anterolateral thigh lfap, 3 pedicled posterior thigh lfaps for subsequent above-knee amputations. No free lfap based reconstructions were performed. An algorithm for reconstruction of leg defects in the setting of limb-salvage surgery is presented. Successful limb salvage rate was 95.4%. Limb salvage failed in 3 patients and they required amputation. Finally, 56 patients were able to ambulate without assistance at last follow-up. Conclusion:The authors present an algorithm for the reconstruction soft tissue after resection of lower extremity bone sarcomas. The use of these techniques helps to decrease complications and maximize function in children with these tumors.

  16. Hypoxia-Inducible Factors: Mediators of Cancer Progression; Prognostic and Therapeutic Targets in Soft Tissue Sarcomas

    Energy Technology Data Exchange (ETDEWEB)

    Sadri, Navid; Zhang, Paul J., E-mail: pjz@mail.med.upenn.edu [Anatomic Pathology, Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania, 3400 Spruce Street, 6th Floor Founders Building, Philadelphia, PA 19104 (United States)

    2013-04-02

    Soft-tissue sarcomas remain aggressive tumors that result in death in greater than a third of patients due to either loco-regional recurrence or distant metastasis. Surgical resection remains the main choice of treatment for soft tissue sarcomas with pre- and/or post-operational radiation and neoadjuvant chemotherapy employed in more advanced stage disease. However, in recent decades, there has been little progress in the average five-year survival for the majority of patients with high-grade soft tissue sarcomas, highlighting the need for improved targeted therapeutic agents. Clinical and preclinical studies demonstrate that tumor hypoxia and up-regulation of hypoxia-inducible factors (HIFs) is associated with decreased survival, increased metastasis, and resistance to therapy in soft tissue sarcomas. HIF-mediated gene expression regulates many critical aspects of tumor biology, including cell survival, metabolic programming, angiogenesis, metastasis, and therapy resistance. In this review, we discuss HIFs and HIF-mediated genes as potential prognostic markers and therapeutic targets in sarcomas. Many pharmacological agents targeting hypoxia-related pathways are in development that may hold therapeutic potential for treating both primary and metastatic sarcomas that demonstrate increased HIF expression.

  17. Kaposi sarcoma: review and medical management update.

    Science.gov (United States)

    Fatahzadeh, Mahnaz

    2012-01-01

    Despite recent advances in our understanding of pathogenic mechanisms involved, the true nature of Kaposi sarcoma remains an enigma. Four clinical variants have been described for the disease, differing in natural history, site of predilection, and prognosis. All forms of Kaposi sarcoma may manifest in the oral cavity and Kaposi sarcoma-associated virus appears essential to development of all clinical variants. The spectrum of therapeutic strategies is broad and selection of appropriate intervention mandates a thorough understanding of disease spread and the patient's symptomatology, as well as risks and benefits of therapy. This article provides an overview of epidemiology, subtypes, clinical course, pathogenesis, and management strategies for Kaposi sarcoma.

  18. Primitive neuroectodermal tumor/Ewing sarcoma of the retina.

    Science.gov (United States)

    Grossniklaus, Hans E; Shehata, Bahig; Sorensen, Poul; Bergstrom, Chris; Hubbard, G Baker

    2012-07-01

    An 11-year-old boy underwent enucleation of his left eye for an intraocular tumor. Examination showed a small, round blue cell tumor arising in the peripheral retina near the ciliary body. Immunohistochemical stain results were positive for neuron-specific enolase, synaptophysin, cluster of differentiation 99 (CD99), Friend leukemia integration 1, and CD56. Ultrastructural findings included occasional intracytoplasmic dense core granules. Polymerase chain reaction of the tumor showed a Ewing sarcoma/Friend leukemia integration gene fusion product. The tumor was classified as a primitive neuroectodermal tumor/Ewing sarcoma of the retina and should be distinguished from retinoblastoma. To our knowledge, this is the first case of primary primitive neuroectodermal tumor of the retina.

  19. Proton Radiotherapy for Pediatric Sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Ladra, Matthew M.; Yock, Torunn I., E-mail: tyock@partners.org [Department of Radiation Oncology, Massachusetts General Hospital, Boston, MA 02114 (United States)

    2014-01-14

    Pediatric sarcomas represent a distinct group of pathologies, with approximately 900 new cases per year in the United States alone. Radiotherapy plays an integral role in the local control of these tumors, which often arise adjacent to critical structures and growing organs. The physical properties of proton beam radiotherapy provide a distinct advantage over standard photon radiation by eliminating excess dose deposited beyond the target volume, thereby reducing both the dose of radiation delivered to non-target structures as well as the total radiation dose delivered to a patient. Dosimetric studies comparing proton plans to IMRT and 3D conformal radiation have demonstrated the superiority of protons in numerous pediatric malignancies and data on long-term clinical outcomes and toxicity is emerging. In this article, we review the existing clinical and dosimetric data regarding the use of proton beam radiation in malignant bone and soft tissue sarcomas.

  20. VEGF Correlates with Inflammation and Fibrosis in Tuberculous Pleural Effusion

    Directory of Open Access Journals (Sweden)

    Mauo-Ying Bien

    2015-01-01

    Full Text Available Objective. To investigate the relationship among angiogenic cytokines, inflammatory markers, and fibrinolytic activity in tuberculous pleural effusion (TBPE and their clinical importance. Methods. Forty-two patients diagnosed with TBPE were studied. Based on chest ultrasonography, there were 26 loculated and 16 nonloculated TBPE patients. The effusion size radiological scores and effusion vascular endothelial growth factor (VEGF, interleukin- (IL- 8, plasminogen activator inhibitor type-1 (PAI-1, and tissue type plasminogen activator (tPA were measured. Treatment outcome and pleural fibrosis, defined as radiological residual pleural thickening (RPT, were assessed at 6-month follow-up. Results. The effusion size and effusion lactate dehydrogenase (LDH, VEGF, IL-8, PAI-1, and PAI-1/tPA ratio were significantly higher, while effusion glucose, pH value, and tPA were significantly lower, in loculated than in nonloculated TBPE. VEGF and IL-8 correlated positively with LDH and PAI-1/tPA ratio and negatively with tPA in both loculated and nonloculated TBPE. Patients with higher VEGF or greater effusion size were prone to develop RPT (n=14; VEGF, odds ratio 1.28, P=0.01; effusion size, odds ratio 1.01, P=0.02, and VEGF was an independent predictor of RPT in TBPE (receiver operating characteristic curve AUC=0.985, P<0.001. Conclusions. Effusion VEGF correlates with pleural inflammation and fibrosis and may be targeted for adjunct therapy for TBPE.

  1. Presumed hydrochlorothiazide-associated immunologic-hypersensitivity-induced pericardial effusion

    Directory of Open Access Journals (Sweden)

    Michael J Chaskes

    2013-08-01

    Full Text Available A 50-year-old Caucasian female presented for a second opinion regarding a newly diagnosed pericardial effusion. Seven months previously, hydrochlorothiazide was introduced into her pharmacologic regimen to aid in the management of her hypertension. A routine echocardiogram indicated a large pericardial effusion with signs of early cardiac tamponade. The patient subsequently underwent successful pericardiocentesis with complete drainage of the pericardial effusion. The effusion was empirically attributed to a viral etiology. Repeat echocardiograms showed recurrence of the pericardial effusion. Prior to undergoing a second pericardiocentesis with pericardial biopsy, as her physicians recommended, the patient sought a second opinion. While obtaining the patient’s history, an allergy to sulfa was elicited. The possibility that the pericardial effusion may be secondary to an immunologic-hypersensitivity reaction was considered. It was recommended the patient discontinue the use of hydrochlorothiazide. Nine days following discontinuation of hydrochlorothiazide and without any other intervention, an echocardiogram was reported to show the size of the pericardial effusion had subsided substantially. Nine weeks following discontinuation, almost complete resolution of the pericardial effusion was reported. It is hypothesized that when treated with hydrochlorothiazide, the patient had an immune response leading to the pericardial effusion.

  2. Olaratumab for soft tissue sarcoma.

    Science.gov (United States)

    Teyssonneau, Diego; Italiano, Antoine

    2017-08-01

    Soft tissue sarcomas (STS) are rare malignant tumors. Unfortunately, the first-line doxorubicin-based treatment has not been improved since the 1970s. Platelet-derived growth factor (PDGF) receptor alpha (PDGFR-α) and its ligands are co-expressed in many types of cancer, including sarcomas. They are involved in stimulating growth and regulating stromal-derived fibroblasts and angiogenesis. PDGFR-α and its ligand may play an important role in tumorigenesis and be a potential target in the treatment of sarcomas. Olaratumab is a fully human IgG1-type anti-PDGFR-α monoclonal antibody with a high affinity and a low 50% inhibitory concentration (IC50). Areas covered: The authors review the role of olaratumab in the treatment of STS by focusing on the recent, randomized Phase II JDGD trial that challenged patients with unresectable or metastatic STS with doxorubicin in the presence or absence of olaratumab. This trial showed a great improvement in overall survival (OS), with an increase in survival from 14.7 months to 26.5 months for patients in the experimental arm and showed acceptable toxicity. Expert opinion: Results seem promising. However, it must be qualified, as the study includes several uncertainties. These uncertainties should be addressed by the ongoing Phase 3 JGDJ confirmatory trial, for which the final efficacy analysis is expected by 2019.

  3. Diagnostic value of Light's criteria and albumin gradient in classifying the pathophysiology of pleural effusion formation in cats.

    Science.gov (United States)

    Zoia, Andrea; Drigo, Michele

    2016-08-01

    The primary aim of this study was to assess whether human Light's criteria with the cut-off values previously published for cats are useful and superior to the traditional veterinary classification in diagnosing pathophysiology of fluid formation in cats with pleural effusion. The secondary aim was to assess if the albumin gradient (ALBg) is a reliable criterion for differentiating exudates from transudates in patients with pleural effusion thought to be transudative by clinical criteria but identified as exudative by Light's criteria. Nineteen client-owned cats with pleural effusion were studied. The aetiology of the pleural effusion was used to establish the pathophysiology of its formation. Parameters measured or calculated undergoing statistical analysis included Light's criteria, total protein and total nucleated cell count in the pleural effusions, and the ALBg. Based on the pathophysiology of fluid formation there were seven transudates caused by increased hydrostatic pressure and 12 exudates. There was a significant difference in the accuracy of the Light's criteria in correctly classifying origin of the pleural fluid formation compared with the traditional veterinary classification (84% vs 53%). ALBg values were significantly different between transudates and exudates. One of the three transudates misclassified as exudates by Light's criteria was correctly identified as a transudate by the ALBg. In conclusion, pleural effusion should be classified as either a transudate or an exudate using Light's criteria. In cats with pleural effusion thought to be transudative by clinical criteria, but identified as exudative by Light's criteria, the ALBg may further help in correctly differentiating exudates from transudates. © The Author(s) 2015.

  4. Ionizing radiation exposure and the development of soft-tissue sarcomas in atomic-bomb survivors.

    Science.gov (United States)

    Samartzis, Dino; Nishi, Nobuo; Cologne, John; Funamoto, Sachiyo; Hayashi, Mikiko; Kodama, Kazunori; Miles, Edward F; Suyama, Akihiko; Soda, Midori; Kasagi, Fumiyoshi

    2013-02-06

    Very high levels of ionizing radiation exposure have been associated with the development of soft-tissue sarcoma. The effects of lower levels of ionizing radiation on sarcoma development are unknown. This study addressed the role of low to moderately high levels of ionizing radiation exposure in the development of soft-tissue sarcoma. Based on the Life Span Study cohort of Japanese atomic-bomb survivors, 80,180 individuals were prospectively assessed for the development of primary soft-tissue sarcoma. Colon dose in gray (Gy), the excess relative risk, and the excess absolute rate per Gy absorbed ionizing radiation dose were assessed. Subject demographic, age-specific, and survival parameters were evaluated. One hundred and four soft-tissue sarcomas were identified (mean colon dose = 0.18 Gy), associated with a 39% five-year survival rate. Mean ages at the time of the bombings and sarcoma diagnosis were 26.8 and 63.6 years, respectively. A linear dose-response model with an excess relative risk of 1.01 per Gy (95% confidence interval [CI]: 0.13 to 2.46; p = 0.019) and an excess absolute risk per Gy of 4.3 per 100,000 persons per year (95% CI: 1.1 to 8.9; p = 0.001) were noted in the development of soft-tissue sarcoma. This is one of the largest and longest studies (fifty-six years from the time of exposure to the time of follow-up) to assess ionizing radiation effects on the development of soft-tissue sarcoma. This is the first study to suggest that lower levels of ionizing radiation may be associated with the development of soft-tissue sarcoma, with exposure of 1 Gy doubling the risk of soft-tissue sarcoma development (linear dose-response). The five-year survival rate of patients with soft-tissue sarcoma in this population was much lower than that reported elsewhere.

  5. Pericardial synovial sarcoma: challenges in diagnosis and management [v2; ref status: indexed, http://f1000r.es/329

    Directory of Open Access Journals (Sweden)

    Prajakta Phatak

    2014-03-01

    Full Text Available Introduction: Pericardial synovial sarcoma is an extremely rare tumor with poor prognosis. Timely diagnosis and aggressive multimodal management improves patient outcome. We present our experience of diagnosis and management of a young patient with monophasic synovial sarcoma arising from pericardium. Case: A 27-year-old man presented with dyspnea and cough of three weeks duration. Examination revealed sinus tachycardia, distant heart sounds and elevated jugular venous pressure. Chest X-ray showed widened mediastinum. Transthoracic echocardiogram (TTE noted large pericardial effusion with tamponade physiology. Therapeutic pericardiocentesis yielded hemorrhagic fluid. Computed tomography (CT of the chest showed persistent pericardial effusion and a left anterior mediastinal mass. Left anterior thoracotomy, pericardial window and left anterior mediastinotomy were done, revealing a well-encapsulated gelatinous tumor originating from the pericardium. Histology and immunohistochemical profile showed the tumor to be a monophasic synovial sarcoma. Fluorescent in-situ hybridization (FISH was positive for SS18 (SYT gene rearrangement on chromosome 18q11, substantiating the diagnosis. Work-up for metastases was negative. Neo-adjuvant chemotherapy with high dose ifosfamide led to substantial reduction in the size of the tumor. The patient underwent surgical resection and external beam radiation therapy (EBRT post surgery. He had symptom-free survival for 8 months prior to local recurrence. This was managed with left lung upper lobectomy and follow-up chemotherapy with docetaxel. The patient is currently stable with an acceptable functional status. Conclusion: In patients with pericardial effusions of unknown etiology, multiple modalities of cardiac imaging must be employed if there is suspicion of a pericardial mass. CT and magnetic resonance imaging (MRI are useful to evaluate for pericardial thickening or masses in addition to TTE. Treatment of synovial

  6. All that Curves is Not Effusion

    Directory of Open Access Journals (Sweden)

    Suhas H. S

    2017-07-01

    Full Text Available Endobronchial tuberculosis is a peculiar variant of tuberculosis hallmarked by involvement of the tracheobronchial tree and contributes to a sizable proportion to the overall morbidity due to the disease. Bronchostenotic variety is the predominant pattern with clinical spectrum ranging from asymptomatic affection to significant life threatening luminal compromise. The radiological features could be deceptive leading to a diagnostic delay. Awareness of the spectrum of the clinical manifestations and heterogeneity in radiological patterns is therefore vital for an accurate diagnosis. We hereby narrate a case of bronchostenotic variant of endobronchial tuberculosis overtly seeming like a pleural effusion on the basis of a chest radiograph.

  7. Management of Otitis Media with Effusion

    Institute of Scientific and Technical Information of China (English)

    LIU Li-min; DONG Min-ming

    2008-01-01

    Otitis media with effusion is one of the most common diseases in children. Its treatment remains controversial. Clinical practice guidelines of OME allow watchful waiting for 3 months before treatment if the child with OME is not at risk for speech/language/or learning problems. Tympanostomy tube insertion is the preferred initial procedure when a child becomes a surgical candidate. Complementary or alternative medicine is not recommended as a treatment for OME. This paper provides a systematic review of management of OME, which we hope will be helpful for clinicians.

  8. Large pericardial effusion induced by minoxidil.

    Science.gov (United States)

    Çilingiroğlu, Mehmet; Akkuş, Nuri; Sethi, Salil; Modi, Kalgi A

    2012-04-01

    A 53-year-old male admitted with increased shortness of breath. In the physical examination, he had dyspnea, tachycardia and tachypnea. An echocardiogram showed large pericardial effusion (PE) as well as significant pulmonary hypertension. He had been started recently on minoxidil for blood pressure control. PE was reported to occur with minoxidil treatment both in patients undergoing dialysis and those with normal renal function. Pulmonary hypertension has been reported to affect the cardiac tamponade physiology. Because of significant pulmonary hypertension in our patient, a right heart catheterization was also done, which prevented cardiac tamponade. He was treated conservatively without any intervention, and PE resolved spontaneously after discontinuation of minoxidil.

  9. Risk Factors Associated With Secondary Sarcomas in Childhood Cancer Survivors: A Report From the Childhood Cancer Survivor Study

    Energy Technology Data Exchange (ETDEWEB)

    Henderson, Tara O., E-mail: thenderson@peds.bsd.uchicago.edu [University of Chicago, Chicago, IL (United States); Rajaraman, Preetha [National Cancer Institute, Bethesda, MD (United States); Stovall, Marilyn [M.D. Anderson Cancer Center, University of Texas, Houston, TX (United States); Constine, Louis S. [University of Rochester, Rochester, NY (United States); Olive, Aliza [Drexel University, Philadelphia, PA (United States); Smith, Susan A. [M.D. Anderson Cancer Center, University of Texas, Houston, TX (United States); Mertens, Ann [Emory University, Atlanta, GA (United States); Meadows, Anna [Children' s Hospital of Philadelphia, Philadelphia, PA (United States); Neglia, Joseph P. [University of Minnesota, Minneapolis, MN (United States); Hammond, Sue [Nationwide Children' s Hospital, Columbus, OH (United States); Whitton, John [Fred Hutchinson Cancer Research Center, Seattle, WA (United States); Inskip, Peter D. [National Cancer Institute, Bethesda, MD (United States); Robison, Leslie L. [St. Jude Children' s Research Hospital, Memphis, TN (United States); Diller, Lisa [Dana-Farber Cancer Institute/Children' s Hospital Cancer Center, Boston, MA (United States)

    2012-09-01

    Purpose: Childhood cancer survivors have an increased risk of secondary sarcomas. To better identify those at risk, the relationship between therapeutic dose of chemotherapy and radiation and secondary sarcoma should be quantified. Methods and Materials: We conducted a nested case-control study of secondary sarcomas (105 cases, 422 matched controls) in a cohort of 14,372 childhood cancer survivors. Radiation dose at the second malignant neoplasm (SMN) site and use of chemotherapy were estimated from detailed review of medical records. Odds ratios (ORs) and 95% confidence intervals were estimated by conditional logistic regression. Excess odds ratio (EOR) was modeled as a function of radiation dose, chemotherapy, and host factors. Results: Sarcomas occurred a median of 11.8 years (range, 5.3-31.3 years) from original diagnosis. Any exposure to radiation was associated with increased risk of secondary sarcoma (OR = 4.1, 95% CI = 1.8-9.5). A dose-response relation was observed, with elevated risks at doses between 10 and 29.9 Gy (OR = 15.6, 95% CI = 4.5-53.9), 30-49.9 Gy (OR = 16.0, 95% CI 3.8-67.8) and >50 Gy (OR = 114.1, 95% CI 13.5-964.8). Anthracycline exposure was associated with sarcoma risk (OR = 3.5, 95% CI = 1.6-7.7) adjusting for radiation dose, other chemotherapy, and primary cancer. Adjusting for treatment, survivors with a first diagnosis of Hodgkin lymphoma (OR = 10.7, 95% CI = 3.1-37.4) or primary sarcoma (OR = 8.4, 95% CI = 3.2-22.3) were more likely to develop a sarcoma. Conclusions: Of the risk factors evaluated, radiation exposure was the most important for secondary sarcoma development in childhood cancer survivors; anthracycline chemotherapy exposure was also associated with increased risk.

  10. Viral oncogene-induced DNA damage response is activated in Kaposi sarcoma tumorigenesis.

    Directory of Open Access Journals (Sweden)

    Sonja Koopal

    2007-09-01

    Full Text Available Kaposi sarcoma is a tumor consisting of Kaposi sarcoma herpesvirus (KSHV-infected tumor cells that express endothelial cell (EC markers and viral genes like v-cyclin, vFLIP, and LANA. Despite a strong link between KSHV infection and certain neoplasms, de novo virus infection of human primary cells does not readily lead to cellular transformation. We have studied the consequences of expression of v-cyclin in primary and immortalized human dermal microvascular ECs. We show that v-cyclin, which is a homolog of cellular D-type cyclins, induces replicative stress in ECs, which leads to senescence and activation of the DNA damage response. We find that antiproliferative checkpoints are activated upon KSHV infection of ECs, and in early-stage but not late-stage lesions of clinical Kaposi sarcoma specimens. These are some of the first results suggesting that DNA damage checkpoint response also functions as an anticancer barrier in virally induced cancers.

  11. Subdural Effusions with Hydrocephalus after Severe Head Injury: Successful Treatment with Ventriculoperitoneal Shunt Placement: Report of 3 Adult Cases

    Directory of Open Access Journals (Sweden)

    N. Tzerakis

    2010-01-01

    Full Text Available Background. Subdural collections of cerebrospinal fluid (CSF with associated hydrocephalus have been described by several different and sometimes inaccurate terms. It has been proposed that a subdural effusion with hydrocephalus (SDEH can be treated effectively with a ventriculoperitoneal shunt (V-P shunt. In this study, we present our experience treating patients with SDEH without directly treating the subdural collection. Methods. We treated three patients with subdural effusions and hydrocephalus as a result of a head injury. All the patients were treated with a V-P shunt despite the fact that there was an extra-axial CSF collection with midline shift. Results. In all of the patients, the subdural effusions subsided and the ventricular dilatation improved in the postoperative period. The final clinical outcome remains difficult to predict and depends not only on the successful CSF diversion but also on the primary and secondary brain insult. Conclusion. Subdural effusions with hydrocephalus can be safely and effectively treated with V-P shunting, without directly treating the subdural effusion which subsides along with the treatment of hydrocephalus. However, it is extremely important to make an accurate diagnosis of an SDEH and differentiate this condition from other subdural collections which require different management.

  12. Activation of PI3K/AKT and ERK MAPK signal pathways is required for the induction of lytic cycle replication of Kaposi's Sarcoma-associated herpesvirus by herpes simplex virus type 1

    Directory of Open Access Journals (Sweden)

    Lv Zhigang

    2011-10-01

    Full Text Available Abstract Background Kaposi's sarcoma-associated herpesvirus (KSHV is causally linked to several acquired immunodeficiency syndrome-related malignancies, including Kaposi's sarcoma (KS, primary effusion lymphoma (PEL and a subset of multicentric Castleman's disease. Regulation of viral lytic replication is critical to the initiation and progression of KS. Recently, we reported that herpes simplex virus type 1 (HSV-1 was an important cofactor that activated lytic cycle replication of KSHV. Here, we further investigated the possible signal pathways involved in HSV-1-induced reactivation of KSHV. Results By transfecting a series of dominant negative mutants and protein expressing constructs and using pharmacologic inhibitors, we found that either Janus kinase 1 (JAK1/signal transducer and activator of transcription 3 (STAT3 or JAK1/STAT6 signaling failed to regulate HSV-1-induced KSHV replication. However, HSV-1 infection of BCBL-1 cells activated phosphatidylinositol 3-kinase (PI3K/protein kinase B (PKB, also called AKT pathway and inactivated phosphatase and tensin homologue deleted on chromosome ten (PTEN and glycogen synthase kinase-3β (GSK-3β. PTEN/PI3K/AKT/GSK-3β pathway was found to be involved in HSV-1-induced KSHV reactivation. Additionally, extracellular signal-regulated protein kinase (ERK mitogen-activated protein kinase (MAPK pathway also partially contributed to HSV-1-induced KSHV replication. Conclusions HSV-1 infection stimulated PI3K/AKT and ERK MAPK signaling pathways that in turn contributed to KSHV reactivation, which provided further insights into the molecular mechanism controlling KSHV lytic replication, particularly in the context of HSV-1 and KSHV co-infection.

  13. The management of clear cell sarcoma

    NARCIS (Netherlands)

    Kuiper, DR; Hoekstra, HJ; Veth, RPH; Wobbes, T

    2003-01-01

    Clear cell sarcoma is a rare soft tissue tumour, constituting approximately 1% of all soft tissue sarcomas. Prognosis is reported to be poor due to the great propensity to metastasise regionally and distantly. In this paper, we report the surgical experience of two university hospitals. Both disease

  14. Choroidal findings in idiopathic uveal effusion syndrome

    Directory of Open Access Journals (Sweden)

    Harada T

    2011-11-01

    Full Text Available Tomomi Harada, Shigeki Machida, Takamistu Fujiwara, Yasunori Nishida, Dajiro KurosakaDepartment of Ophthalmology, Iwate Medical University School of Medicine, Iwate, JapanPurpose: We report choroidal findings by means of enhanced depth imaging spectral-domain optical coherence tomography (EDI-OCT in a patient with idiopathic uveal effusion syndrome (IUES.Case report: A 41-year-old man was referred to us with ciliochoroidal and non-rhegmatogenous retinal detachments. Sclerectomies and sclerostomies were performed at the equator in the lower quadrants, resulting in resolution of the ciliochoroidal and retinal detachments. EDI-OCT demonstrated low-reflective areas in the outer choroid. The subfoveal choroidal thickness measured vertically from the outer border of the RPE to the inner border of the sclera was 787 µm which was significantly thicker than the normal value (272 ± 90 µm, n = 131 obtained from age-matched normal controls.Conclusions: The findings made by EDI-OCT have provided additional evidence that choroidal alterations play a role in the pathological process in IUES.Keywords: uveal effusion syndrome, spectral-domain optical coherence tomography, EDI-OCT, OCT, choroid

  15. Hydrogen effusion from tritiated amorphous silicon

    Science.gov (United States)

    Kherani, N. P.; Liu, B.; Virk, K.; Kosteski, T.; Gaspari, F.; Shmayda, W. T.; Zukotynski, S.; Chen, K. P.

    2008-01-01

    Results for the effusion and outgassing of tritium from tritiated hydrogenated amorphous silicon (a-Si:H:T) films are presented. The samples were grown by dc-saddle field glow discharge at various substrate temperatures between 150 and 300°C. The tracer property of radioactive tritium is used to detect tritium release. Tritium effusion measurements are performed in a nonvacuum ion chamber and are found to yield similar results as reported for standard high vacuum technique. The results suggest for decreasing substrate temperature the growth of material with an increasing concentration of voids. These data are corroborated by analysis of infrared absorption data in terms of microstructure parameters. For material of low substrate temperature (and high void concentration) tritium outgassing in air at room temperature was studied, and it was found that after 600h about 0.2% of the total hydrogen (hydrogen+tritium) content is released. Two rate limiting processes are identified. The first process, fast tritium outgassing with a time constant of 15h, seems to be related to surface desorption of tritiated water (HTO) with a free energy of desorption of 1.04eV. The second process, slow tritium outgassing with a time constant of 200-300h, appears to be limited by oxygen diffusivity in a growing oxide layer. This material of lowest H stability would lose half of the hydrogen after 60years.

  16. [Benign pleural effusion caused by asbestos exposure].

    Science.gov (United States)

    Vieira, J R; Alfarroba, E; Viegas, J; Freitas e Costa, M

    1992-05-01

    The Authors present the first case described among us of benign pleural effusion of an asbestotic origin. They stress the importance of thoracoscopy (pleuroscopy) in the diagnosis of this situation. Attention is drawn to the fact that asbestotic lesions and asbestotic bodies have been found in the lung and, in particular, in the parietal pleura as well. They emphasize the fact that exposure to asbestos was not realized by the patient, which made the clarification of the situation more difficult. It was a CT scan that showed the signs suggestive of exposure to asbestos which raised the diagnostic suspicion. They conclude that every patient with a pleural effusion must be thoroughly questioned about exposure to asbestos. Even if the exposure is accepted, they consider that one should proceed to a pleuro-pulmonar biopsy by thoracoscopy. This biopsy allows demonstration of the characteristic histopathological lesions and rule out other etiologies, namely malignancy and tuberculosis. They suggest that these patients must be highly motivated to stop any smoking and kept under periodic surveillance.

  17. External radiotherapy in the management of malignant pericardial effusion

    Energy Technology Data Exchange (ETDEWEB)

    Fairlamb, D.J. (The Royal Hospital, Wolverhampton (UK))

    1989-05-01

    Malignant pericardial effusions that are not causing tamponade can be effectively treated by external beam irradiation - a readily available non-invasive treatment. In a consecutive series six out of eight patients achieved good palliation of their effusions as a result of this treatment. (author).

  18. Pericardial effusion and cardiac tamponade in a dog with hypothyroidism

    Directory of Open Access Journals (Sweden)

    J Ojeda

    2015-01-01

    Full Text Available The relationship between hypothyroidism and cardiovascular diseases in dogs is frequently associated with clinical signs like sinusal bradicardia, myocardial alterations and ECG findings such as low voltage QRS and inverted T wave. Echocardiographic studies in hypothyroid dogs have not identified pericardial effusion. Hypothyroidism in human patients is a well-known cause of pericardial effusion, but cardiac tamponade is not a frequent clinical sign. A Golden Retriever was presented with a clinical history of progressive lethargy, exercise intolerance, cold intolerance and increased respiratory effort for 3 weeks. At the clinical examination the dog presented dyspnea, muffled heart sound and hypertension. Echocardiography showed fluid in the pericardial sac and cardiac tamponade and pericardiocentesis was performed. Analyses of the fluid showed a non-neoplastic etiology. The information given by the owners, the alopecia zones detected in tail and abdomen and the clinical response, led to analise the thyroid hormones, and hypothyroidism was confirmed. Following two pericardiocentesis and levothyroxine supplementation, the effusion resolved. Although the etiology of pericardial effusion was not clear, it is possible that the pericardial effusion had been caused by hypothyroidism or the dog had idiophatic pericardial effusion and coincidental hypothyroidism. However, according to the clinical signs and the evolution of the dog, an evaluation of thyroid hormones in dogs with pericardial effusion must be considered even though pericardial effusion is a non-described sign in canine hypothyroidism.

  19. Gastrointestinal Kaposi sarcoma with appendiceal involvement.

    Science.gov (United States)

    Egwuonwu, Steve; Gatto-Weis, Cara; Miranda, Roberto; Casas, Luis De Las

    2011-04-01

    Kaposi sarcoma is a vascular tumor manifesting as nodular lesions on skin, mucous membranes, or internal organs. This is a case of a 42-year-old human immunodeficiency virus- (HIV) positive bisexual male, not on highly active antiretroviral therapy (HAART) since diagnosis four years ago. He presented with a three-day history of abdominal pains, fever, vomiting, and a one-week history of melena stools. Endoscopy revealed Kaposi sarcoma in the stomach and duodenum. Postendoscopy, he developed acute abdomen. Exploratory laparotomy revealed extensive Kaposi sarcoma of the gastrointestinal tract with appendiceal involvement. The patient underwent appendectomy and had an uneventful recovery. A review of the literature discusses appendiceal Kaposi sarcoma with appendicitis, a rare but critical manifestation of gastrointestinal Kaposi sarcoma.

  20. Penile epithelioid sarcoma: MR imaging findings

    Energy Technology Data Exchange (ETDEWEB)

    Sirikci, A.; Bayram, M.; Demirci, M. [Department of Radiology, Faculty of Medicine, Gaziantep University, Kolejtepe, Gaziantep (Turkey); Bakir, K. [Department of Pathology, Faculty of Medicine, Gaziantep University, Kolejtepe, Gaziantep (Turkey); Sarica, K. [Department of Urology, Faculty of Medicine, Gaziantep University, Kolejtepe, Gaziantep (Turkey)

    1999-10-01

    Magnetic resonance imaging findings of a 38-year-old man with epithelioid sarcoma of the penis is presented. It started as a firm, painless and slowly growing nodule at the base of his penis 6 months previously which caused pain radiating to the testis during coitus. It has been well known that sarcomas may well mimic reactive processes. Initial presentation of epithelioid sarcoma may provoke considerable diagnostic difficulty, and its differentiation from benign lesions, such as Peyronie`s disease and chronic inflammation, may be a clinical problem. In our present report the MR findings are compared with those of the epithelioid sarcomas of various locations reported in the literature and differential diagnosis of the entity is discussed. To our knowledge, this is the first report regarding the MR findings of the epithelioid sarcoma of penis. (orig.) With 3 figs., 16 refs.

  1. Ewing's sarcoma: an approach to radiological diagnosis.

    Science.gov (United States)

    Lombardi, F; Gasparini, M; Gianni, C; Petrillo, R; Tesoro-Tess, J D; Volterrani, F; Musumeci, R

    1979-06-30

    All the pertinent radiographs of 83 patients with histologically proven Ewing's sarcoma were reviewed. Forty-nine patients were in the pediatric age group, and 34 were adults. The mean age, the symptoms and time from symptoms to diagnosis were evaluated in the 2 groups. The site of primary involvement was in 54% the long bones, 35% the flat bones, 8% the small bones and 3% extraosseous. For the primary site we considered the diagnostic results of the standard radiographic investigations and in some cases the usefulness of angiography, xeroradiography and telethermography. At presentation we also evaluated the possible diffusion of the disease with standard radiographic surveys (chest and skeletal, including limbs) and with foot lymphography in selected cases. In this way, 57 patients (69%) were considered to have localized disease. In this group, we also considered the value of the periodic radiographic follow-up, which enabled us to disclose the appearance of metastases (chest 64%, bone 54%, lymph nodes 11%) in 28 cases (49%). Finally, we made a comparison of the different radiologic and epidemiologic findings between children and adults.

  2. [Diagnostic value of tumor markers in pleural effusions].

    Science.gov (United States)

    Botte, G; Laferrere, L; Etchepare, S; Dalurzo, D; Duhart, J E; Adaro, F V

    1990-01-01

    In order to discriminate between benign and malignant effusions, the value of alpha-fetoprotein (AFP), carcinoembryonic antigen (CEA) and beta subunit of human chorionic gonadotropin (GNCH Sub-beta) has been estimated in pleural exudates. A sample of 65 patients, 33 with a malignant (histologically and/or cytologically established) and 32 with a benign effusion was analysed. Only mean CEA in malignant effusions was significantly higher than in benign effusions (p less than 0.01). In the detection of malignant effusion CEA showed a sensitivity of 57% and a specificity of 97%; AFP a sensitivity of 9% and a specificity of 97% and GNCH Sub beta a sensitivity of 9% and a specificity of 90%.

  3. Rheologic studies on middle ear effusions and their mucus glycoproteins.

    Science.gov (United States)

    FitzGerald, J E; Green, G G; Birchall, J P; Pearson, J P

    1989-04-01

    The properties of pooled thick and thin middle ear effusions, from children with otitis media with effusion, were studied by viscometry. Mucus glycoproteins were responsible for effusion viscosity. Their percentage by weight in thick and thin effusions was 25% and 8.2%, respectively. N-acetylcysteine and 0.2 mol/L of mercaptoethanol caused a 39% viscosity drop in a 5-mg/mL glycoprotein solution, whereas S-carboxymethylcysteine had no effect. Treatment of thick effusions with 0.2 mol/L of mercaptoethanol initially caused a viscosity decrease followed by a gradual increase. Higher reducing agent concentrations (0.5 mol/L) caused a more rapid decrease followed by a rapid increase, presumably by causing nonspecific aggregation of reduced protein molecules. These results suggest that the concentration of and the time that a mucolytic is in the middle ear would be of prime importance in achieving the desired decrease in viscosity.

  4. Therapeutic Trial for Patients With Ewing Sarcoma Family of Tumor and Desmoplastic Small Round Cell Tumors

    Science.gov (United States)

    2016-08-25

    Desmoplastic Small Round Cell Tumor; Ewing Sarcoma of Bone or Soft Tissue; Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor

  5. The local cytokine and chemokine milieu within malignant effusions.

    Science.gov (United States)

    Atanackovic, Djordje; Cao, Yanran; Kim, Ji-Won; Brandl, Stephan; Thom, Ina; Faltz, Christiane; Hildebrandt, York; Bartels, Katrin; de Weerth, Andreas; Hegewisch-Becker, Susanna; Hossfeld, Dieter Kurt; Bokemeyer, Carsten

    2008-01-01

    Malignant effusions offer a unique opportunity for the study of interactions between the human immune system and cancer. We have recently demonstrated that malignant effusions are characterized by an accumulation of T cells expressing chemokine receptors such as CCR4, which is commonly found on Th2 cells. In contrast, effector T cells expressing chemokine receptors typical for Th1 cells, such as CCR5, showed a diminished homing into malignant effusions. We analyzed concentrations of 12 different cytokines and 9 chemokines within malignant and nonmalignant effusions and investigated cytokine expression by effusion-infiltrating leukocytes. We observed that concentrations of the immunoregulatory cytokine TGF-beta(1) and of angiogenic factors VEGF and IL-8 were markedly increased within effusions caused by malignancies. However, we did not observe signs of a typical Th1 or Th2 milieu. Analyzing concentrations of 9 different chemokines, we found elevated concentrations of the chemokines MDC, eotaxin, I-TAC, and MCP-1 in malignant effusions. Interestingly, tumor-infiltrating leukocytes themselves seemed to contribute strongly to the creation of a distinct cytokine/chemokine pattern within cancer-related effusions. Additional analyses suggested that this cytokine/chemokine milieu might support an enrichment of immunosuppressive leukocytes. The local cytokine and chemokine milieu within malignant effusions seems to promote angiogenesis and to block an efficient immune-mediated antitumor response. An elimination of such tumor-promoting influences will be necessary in order to transform local immunotolerance into clinically relevant immune recognition of tumors causing malignant effusions. (c) 2008 S. Karger AG, Basel

  6. Vascular endothelial growth factor-D is a key molecule that enhances lymphatic metastasis of soft tissue sarcomas

    Energy Technology Data Exchange (ETDEWEB)

    Yanagawa, Takashi, E-mail: tyanagaw@med.gunma-u.ac.jp [Department of Orthopaedic Surgery, Gunma University Graduate School of Medicine, 3-39-22, Showa, Maebashi, Gunma, 371-8511 (Japan); Shinozaki, Tetsuya [Department of Orthopaedic Surgery, Gunma University Graduate School of Medicine, 3-39-22, Showa, Maebashi, Gunma, 371-8511 (Japan); Watanabe, Hideomi [Department of Physical Therapy, Gunma University School of Health Science, 3-39-22, Showa, Maebashi, Gunma, 371-8511 (Japan); Saito, Kenichi [Department of Orthopaedic Surgery, Gunma University Graduate School of Medicine, 3-39-22, Showa, Maebashi, Gunma, 371-8511 (Japan); Raz, Avraham [Tumor Progression and Metastasis Program, Karmanos Cancer Institute, Wayne State University, 110 E. Warren Ave., Detroit, MI (United States); Takagishi, Kenji [Department of Orthopaedic Surgery, Gunma University Graduate School of Medicine, 3-39-22, Showa, Maebashi, Gunma, 371-8511 (Japan)

    2012-04-15

    Studies on lymph node metastasis of soft tissue sarcomas are insufficient because of its rarity. In this study, we examined the expressions of vascular endothelial growth factor (VEGF)-C and VEGF-D in soft tissue sarcomas metastasized to lymph nodes. In addition, the effects of the two molecules on the barrier function of a lymphatic endothelial cell monolayer against sarcoma cells were analyzed. We examined 7 patients who had soft tissue sarcomas with lymph node metastases and who had undergone neither chemotherapy nor radiotherapy before lymphadenectomy. Immunohistochemistry revealed that 2 of 7 sarcomas that metastasized to lymph nodes expressed VEGF-C both in primary and metastatic lesions. On the other hand, VEGF-D expression was detected in 4 of 7 primary and 7 of 7 metastatic lesions, respectively. Interestingly, 3 cases that showed no VEGF-D expression at primary sites expressed VEGF-D in metastatic lesions. Recombinant VEGF-C at 10{sup -8} and VEGF-D at 10{sup -7}and 10{sup -8} g/ml significantly increased the random motility of lymphatic endothelial cells compared with controls. VEGF-D significantly increased the migration of sarcoma cells through lymphatic endothelial monolayers. The fact that VEGF-D induced the migration of fibrosarcomas through the lymphatic endothelial monolayer is the probable reason for the strong relationship between VEGF-D expression and lymph node metastasis in soft tissue sarcomas. The important propensities of this molecule for the increase of lymph node metastases are not only lymphangiogenesis but also down-regulation of the barrier function of lymphatic endothelial monolayers, which facilitates sarcoma cells entering the lymphatic circulation.

  7. Ewing's Sarcoma and Second Malignancies

    Directory of Open Access Journals (Sweden)

    Joshua D. Schiffman

    2011-01-01

    Full Text Available Ewing's sarcoma (ES is a rare tumor that is most common in children and young adults. Late effects of ES therapy include second cancers, a tragic outcome for survivors of such a young age. This paper will explore the frequencies and types of malignancies that occur after ES. Additionally, it will review how second malignancies have changed with the shift in treatment from high-dose radiation to chemotherapy regimens including alkylators and epipodophyllotoxins. The risk of additional cancers in ES survivors will also be compared to survivors of other childhood cancers. Finally, the possible genetic contribution to ES and second malignancies will be discussed.

  8. Isolated limb perfusion of soft tissue sarcomas : A comprehensive review of literature

    NARCIS (Netherlands)

    Seinen, Jojanneke M.; Hoekstra, Harald J.

    2013-01-01

    Patients with primary irresectable, locally advanced soft tissue sarcomas of the limbs form a challenging group for the treating physician. Multimodality treatment is necessary to guarantee optimal limb salvage and survival rates. Since the introduction of isolated limb perfusion in the late fifties

  9. CA-125 IN SERUM AND TUMOR FROM PATIENTS WITH UTERINE SARCOMA

    NARCIS (Netherlands)

    DUK, JM; BOUMA, J; BURGER, GTN; DEBRUIJN, HWA

    1994-01-01

    Serial serum samples of 33 patients with primary sarcoma of the uterus were analyzed for CA 125 and frozen tissue sections of tumor from 23 patients were tested for this antigen. Before treatment, 12 of 30 evaluable patients showed serum CA 125 levels >16 Uml-1 (40%). There was no relationship betwe

  10. Sarcoma sinovial primário da orofaringe: relato de caso

    OpenAIRE

    Meister Hugo; Miranda Thaís C.; Nóbrega Manuel G. G.

    2003-01-01

    O sarcoma sinovial primário da cabeça e pescoço é um tumor raro. Em torno de 90 casos foram descritos na literatura. Relatamos um caso adicional ocorrido em um paciente jovem do sexo masculino comprometendo a orofaringe. O tumor apresentou típico crescimento bifásico, fibrossarcomatoso e epitelial, com estruturas pseudo-glandulares. O diagnóstico final foi obtido pelo exame da peça cirúrgica com preparações histológicas de rotina e análise imunohistoquímica.Primary synovial sarcoma of the hea...

  11. Successful treatment of spindle cell sarcoma of the sella turcica. Case report.

    Science.gov (United States)

    Alpert, Tracy E; Hahn, Seung S; Chung, Chung T; Bogart, Jeffrey A; Hodge, Charles J; Montgomery, Craig

    2002-12-01

    A primary spindle cell sarcoma of the sella turcica in a patient without a history of radiation treatment is a very rare occurrence. Only one other case has been reported to date, with local recurrence 7 months after the patient underwent subtotal resection and stereotactic radiosurgery of the tumor. The authors present a case of spindle cell sarcoma of the sella turcica successfully treated by surgery, external-beam radiotherapy, and gamma knife radiosurgery. After 24 months of follow up, the patient continues to show no evidence of disease.

  12. Anticardiac Antibodies in Patients with Chronic Pericardial Effusion

    Directory of Open Access Journals (Sweden)

    Konstantinos Karatolios

    2016-01-01

    Full Text Available Objectives. Chronic pericardial effusion may be challenging in terms of diagnosis and treatment. Specific laboratory parameters predicting the frequency and severity of recurrences after initial drainage of pericardial effusion are lacking. Materials and Methods. Pericardial fluid (PF and serum (SE samples from 30 patients with chronic pericardial effusion (PE who underwent pericardiocentesis and pericardioscopically guided pericardial biopsy were compared with SE and PF samples from 26 control patients. The levels of antimyolemmal (AMLA and antifibrillary antibodies (AFA in PE and SE from patients with pericardial effusion as well as PF and SE from controls were determined and compared. Results. AMLAs and AFAs in PF and SE were significantly higher in patients with chronic pericardial effusion than in the control group (AMLAs: p = 0,01 for PF and p = 0,004 for serum; AFAs: p < 0,001 for PF and p = 0,003 for serum. Patients with recurrence of PE within 3 months after pericardiocentesis had significantly higher levels of AMLAs in SE (p = 0,029 than patients without recurrence of PE. Conclusions. The identification of elevated anticardiac antibodies in PE and SE indicates increased immunological reactivity in chronic pericardial effusion. High titer serum levels of AMLAs also correlate with recurrence of pericardial effusion.

  13. Allergic bronchopulmonary aspergillosis: a rare cause of pleural effusion.

    LENUS (Irish Health Repository)

    O'Connor, T M

    2012-02-03

    Aspergillus fumigatus is one of the most ubiquitous of the airborne saprophytic fungi. Allergic bronchopulmonary aspergillosis (ABPA) is a syndrome seen in patients with asthma and cystic fibrosis, and is characterized by hypersensitivity to chronic colonization of the airways with A. fumigatus. We report the case of a patient with ABPA presenting with pleural effusion. A 27-year-old male was referred with recurrent right pleural effusion. Past medical history was remarkable for asthma, allergic sinusitis, and recurrent pleurisy. Investigations revealed peripheral eosinophilia with elevated serum immunoglobulin E and bilateral pleural effusions with bilateral upper lobe proximal bronchiectasis. Precipitating serum antibodies to A. fumigatus were positive and the A. fumigatus immediate skin test yielded a positive reaction. A diagnosis of ABPA associated with bilateral pleural effusions was made and the patient was commenced on prednisolone. At review, the patient\\'s symptoms had considerably improved and his pleural effusions had resolved. ABPA may present with diverse atypical syndromes, including paratracheal and hilar adenopathy, obstructive lung collapse, pneumothorax and bronchopleural fistula, and allergic sinusitis. Allergic bronchopulmonary aspergillosis is a rare cause of pleural effusion and must be considered in the differential diagnosis of patients presenting with a pleural effusion, in particular those with a history of asthma.

  14. Long-term Outcome of Patients With Undiagnosed Pleural Effusion.

    Science.gov (United States)

    Gunluoglu, Gulsah; Olcmen, Aysun; Gunluoglu, Mehmet Zeki; Dincer, Ibrahim; Sayar, Adnan; Camsari, Gungor; Yilmaz, Veysel; Altin, Sedat

    2015-12-01

    The cause of exudative pleural effusion cannot be determined in some patients. The longterm outcomes of patients with undiagnosed pleural effusion were analyzed. Patients with exudative pleural effusion whose diagnostic procedures included pleural biopsy using video-assisted thoracoscopic surgery carried out between 2008 and 2012 were evaluated retrospectively. Patients diagnosed with non-specific pleuritis were included. Fifty-three patients with available follow-up data were included in the study. Forty men and 13 women (mean age 53.9±13.9 years) were included. Median follow-up time was 24 months. No diagnosis was given in 27 patients (51%), and a clinical diagnosis was given in 26 patients (49%) during the follow-up period. Malignant disease (malignant mesothelioma) was diagnosed in 2 (3.7%) patients. Other diseases were parapneumonic effusion in 12, congestive heart failure in 8, and miscellaneous in 4 patients. Volume of effusion at the time of initial examination and re-accumulation of fluid after video-assisted thoracoscopic surgery were associated with malignant disease (P=.004 and .0001, respectively). Although the probability is low, some patients with exudative pleural effusion undiagnosed after pleural biopsy via video-assisted thoracoscopic surgery may have malignant disease. Patients with an initially large volume of effusion that re-accumulates after examination should be closely monitored. Copyright © 2014 SEPAR. Published by Elsevier Espana. All rights reserved.

  15. Skull-base Ewing sarcoma with multifocal extracranial metastases

    Directory of Open Access Journals (Sweden)

    Sumit Thakar

    2012-01-01

    Full Text Available Intracranial occurrence of Ewing sarcoma (ES is unusual, with a skull-base location being anecdotal. We report a 29-year-old man who presented with rapidly progressive ophthalmoplegia, and was found to be harboring an infiltrative lesion involving the sphenoid sinus, sella, and clivus. He underwent trans-sphenoidal decompression of the lesion which was histologically suggestive of ES. He developed paraparesis 2 weeks after commencing adjuvant therapy. Imaging revealed two thoracic extradural lesions and florid vertebral and pulmonary metastases. This is the first report in indexed literature of a primary intracranial ES on the skull-base with disseminated extracranial disease.

  16. Diagnostic dilemma in Kaposi′s sarcoma

    Directory of Open Access Journals (Sweden)

    Rao Satish

    2006-01-01

    Full Text Available Kaposi′s sarcoma is described as cutaneous and extracutaneous neoplasm predominantly affecting older individuals. Though earlier uncommon and endemic to certain African areas, its incidence is on a rise due to infections with human immunodeficiency virus and also due to transplant-associated immunosuppression. Further, certain benign conditions like Pseudo Kaposi′s sarcoma, certain infective conditions like bacillary angiomatosis of acquired immunodeficiency syndrome can mimic Kaposi′s sarcoma both clinically and histologically leading to a diagnostic dilemma. We report such a case here.

  17. [Synovial sarcoma of the infratemporal fossa].

    Science.gov (United States)

    Tamarit Conejeros, José Manuel; Estrems Navas, Paloma; Estellés Ferriol, Enrique; Dalmau Galofre, José

    2010-01-01

    Synovial sarcoma is the fourth most common type of sarcoma. It is usually found in the knee or ankle joints, and is exceptional in the head and neck. Most cases are diagnosed in men between 20 and 40 years of age. Diagnosis is often casual due to the infrequent nature of this tumour and its non-specific clinical and radiological characteristics. Confirmation is therefore based on immunohistochemistry and electron microscopy techniques. We report a case of biphasic sinovial sarcoma located in the infratemporal fossa treated at our hospital and we make a review of the literature. Copyright © 2009 Elsevier España, S.L. All rights reserved.

  18. Sarcoma sinovial anorretal: relato de caso

    OpenAIRE

    Hernán Augusto Centurión Sobral; Enzo Martins Taglietti; Elisângela Plazaz Monteiro; Marília Resende Von Sonnleithner Gama; Sérgio Henrique Couto Horta; Galdino José Sitonio Formiga

    2006-01-01

    Os sarcomas são neoplasias que se originam das células mesenquimais primitivas, sendo raros na região anorretal. O objetivo é relatar um caso de sarcoma sinovial anorretal, neoplasia extremamente rara nesta localização. É descrito o caso de uma paciente de 77 anos que apresentava nodulação anal dolorosa e sangrante às evacuações, associada a puxo, tenesmo e perda ponderal. A lesão foi biopsiada e o estudo imunohistoquímico evidenciou sarcoma sinovial anorretal. A paciente foi submetida a ampu...

  19. Intracranial Dural Metastasis of Ewing's Sarcoma: a Case Report

    Energy Technology Data Exchange (ETDEWEB)

    Kim, Eung Yeop; Lee, Seung Koo; Kim, Dong Joon; Kim, Jin Na; Lee, Kyu Sung; Jung, Woo Hee; Kim, Dong Ik [Yonsei University College of Medicine, Seoul (Korea, Republic of)

    2008-02-15

    Ewing's sarcoma is a malignant bone tumor that can occur anywhere in the body, but it is most commonly observed in the long bones of the arms and legs, the pelvis and in the chest. The predominant sites of metastasis include the lung (38%), bone (including the spine; 31%), and the bone marrow (11%). Metastasis of Ewing's sarcoma to the central nervous system (CNS) is relatively rare, and most of the previous reports have demonstrated involvement of the bony calvarium or brain parenchyma. We describe here the imaging findings of dural metastasis of Ewing's sarcoma, and these imaging findings have not been previously reported on in the medical literature. In conclusion, dural metastasis of Ewing's sarcoma is very rare and its imaging characteristics are similar to those of a primary tumor, which mimic the findings of a schwannoma or meningioma. Despite its rarity, secondary Ewing's sarcoma may be included in the differential diagnosis of extra-axial dural masses.

  20. Ewing's sarcoma with metachronous pulmonary metastasis after successful treatment of osteosarcoma in a child.

    Science.gov (United States)

    Stebnicky, Milan; Belak, Jozef; Bohus, Peter; Gmitter, Frantisek; Jenco, Igor; Tkacova, Ruzena

    2009-01-01

    A rare case of duplicate tumor, osteosarcoma and Ewing's sarcoma, complicated by metachronous pulmonary metastasis in a child is reported. A nine-year-old girl's osteosarcoma was treated by neoadjuvant chemotherapy followed by surgery and adjuvant chemotherapy. Four years later, resection of the chest wall to remove an Ewing's sarcoma had to be performed, followed by chemotherapy and radiotherapy. At the age of 17, the girl underwent a metastasectomy of Ewing's sarcoma metastasis to the lung. Five years later, the patient is free from any recognizable malignant disease. We conclude that after the complete surgical removal of two primary tumors, metastasectomy is an optimal treatment procedure in case of a solitary pulmonary metastasis.

  1. Ewing Sarcoma/Primitive Neuroectodermal Tumor of the Kidney: A Rare and Lethal Entity.

    Science.gov (United States)

    Celli, Romulo; Cai, Guoping

    2016-03-01

    Ewing sarcoma/primitive neuroectodermal tumor represents a spectrum of undifferentiated tumors with similar biology that together represent the second most common sarcoma in the pediatric-young adult age range. Very rarely, this tumor presents as a primary neoplasm of the kidney. The clinical presentation of this tumor is not specific, and other renal tumors may present with a similar histologic appearance. Establishing the correct diagnosis is critical because renal Ewing sarcoma/primitive neuroectodermal tumor carries a strikingly dismal prognosis and thus dictates a specific treatment strategy. A low threshold for the use of ancillary molecular tests is recommended, particularly in diagnostically problematic cases. Important considerations with regards to morphology, immunohistochemistry, and molecular alterations will be reviewed here and should be taken into account before rendering this rare and lethal diagnosis.

  2. Nonmetastatic Ewing's Sarcoma of the Lumbar Spine in an Adult Patient

    Science.gov (United States)

    Iacoangeli, Maurizio; Dobran, Mauro; Di Rienzo, Alessandro; di Somma, Lucia Giovanna Maria; Alvaro, Lorenzo; Moriconi, Elisa; Nocchi, Niccolò; Gladi, Maurizio; Scerrati, Massimo

    2012-01-01

    Although the spine is frequently involved in metastatic Ewing's sarcoma, primary involvement of the spine, beside sacrum, is much less frequent, especially in adult patients. Because of the low incidence of these tumors, there are currently no clinical guidelines outlining their management and a multitude of therapeutic strategies have been employed with varying success. The definitive management of Ewing's sarcoma of the spine, as in other locations, could include the combination of three main modalities: aggressive surgery, radiotherapy, and combined chemotherapy. Whenever possible, en bloc spondylectomy or extralesional resection is preferable, providing a better oncological result with a longer survival and a better preservation of the spine biomechanics. This is the lesson we learned about the case, we present here, of nonmetastatic lumbar localization by Ewing's sarcoma in as adult patient. PMID:23133768

  3. Nonmetastatic Ewing’s Sarcoma of the Lumbar Spine in an Adult Patient

    Directory of Open Access Journals (Sweden)

    Maurizio Iacoangeli

    2012-01-01

    Full Text Available Although the spine is frequently involved in metastatic Ewing's sarcoma, primary involvement of the spine, beside sacrum, is much less frequent, especially in adult patients. Because of the low incidence of these tumors, there are currently no clinical guidelines outlining their management and a multitude of therapeutic strategies have been employed with varying success. The definitive management of Ewing's sarcoma of the spine, as in other locations, could include the combination of three main modalities: aggressive surgery, radiotherapy, and combined chemotherapy. Whenever possible, en bloc spondylectomy or extralesional resection is preferable, providing a better oncological result with a longer survival and a better preservation of the spine biomechanics. This is the lesson we learned about the case, we present here, of nonmetastatic lumbar localization by Ewing’s sarcoma in as adult patient.

  4. Reliability of Margin Assessment after Surgery for Extremity Soft Tissue Sarcoma: The SSG Experience

    Directory of Open Access Journals (Sweden)

    Clement S. Trovik

    2012-01-01

    Full Text Available Surgery remains the mainstay of soft tissue sarcoma (STS treatment and has been the primary treatment for the majority of patients in Scandinavia during the last 30 years although the use of adjuvant radiotherapy has increased. Patient and treatment characteristics have been recorded in the Scandinavian Sarcoma Group (SSG Register since 1987. When the effect of new radiotherapy guidelines from 1998 was evaluated, the reliability of surgical margin assessments among different Scandinavian institutions was investigated. Margins were reevaluated by a panel of sarcoma surgeons, studying pathology and surgical reports from 117 patients, randomly selected among 470 recorded patients treated between 1998–2003. In 80% of cases, the panel agreed with the original classification. Disagreement was most frequent when addressing the distinction between marginal and wide margins. Considered the element of judgment inherent in all margin assessment, we find this reliability acceptable for using the Register for studies of local control of STS.

  5. A Case of Haemorrhagic Constrictive Pericarditis with Bilateral Pleural Effusions

    Science.gov (United States)

    Islam, Julie; Talebi, Soheila; Cativo, Eder; Mushiyev, Savi; Pekler, Gerald; Visco, Ferdinand

    2016-01-01

    Presentation of pericardial disease is diverse, with the viral aetiology being the most common cause; however, when haemorrhagic pericardial effusion is present, these causes are narrowed to few aetiologies. We present a case of a young female of African descent who presented with diffuse abdominal pain and vomiting. Initial work-up showed pericardial effusion with impending echocardiographic findings of cardiac tamponade and bilateral pleural effusions. Procedures included a left video-assisted thoracoscopic surgery (VATS) with pericardial window. We consider that it is important for all physicians to be aware of not only typical presentation but also atypical and unusual clinical picture of pericardial disease.

  6. Delayed internal pancreatic fistula with pancreatic pleural effusion postsplenectomy

    Institute of Scientific and Technical Information of China (English)

    2010-01-01

    The occurrence of pancreatic pleural effusion,secondary to an internal pancreatic fistula,is a rare clinical syndrome and diagnosis is often missed.The key to the diagnosis is a dramatically elevated pleural fluid amylase.This pancreatic pleural effusion is also called a pancreatic pleural fistula.It is characterized by profuse pleural fluid and has a tendency to recur.Here we report a case of delayed internal pancreatic fistula with pancreatic pleural effusion emerging after splenectomy.From the treatment ...

  7. Otitis media with effusion:diagnosis and treatment

    Directory of Open Access Journals (Sweden)

    Ozgur Surmelioglu

    2013-04-01

    Full Text Available Otitis media with effusion (serous otitis media is characterised by an accumulation of fluid in the middle ear behind an intact tympanic membrane, without the symptoms or signs of acute infection. Serous otitis media is the most common cause of hearing loss in children in the developed world. Hearing loss may be affected speech, cognitive, and psychological development of the childeren. In addition, otitis media with effusion is not only seen in children, this sign may present with signs of nasopharyngeal diseases. For this reason, especially in childhood otitis media with effusion should be treated by early diagnosis. [Archives Medical Review Journal 2013; 22(2.000: 194-208

  8. A Case of Haemorrhagic Constrictive Pericarditis with Bilateral Pleural Effusions

    Directory of Open Access Journals (Sweden)

    Hans A. Reyes

    2016-01-01

    Full Text Available Presentation of pericardial disease is diverse, with the viral aetiology being the most common cause; however, when haemorrhagic pericardial effusion is present, these causes are narrowed to few aetiologies. We present a case of a young female of African descent who presented with diffuse abdominal pain and vomiting. Initial work-up showed pericardial effusion with impending echocardiographic findings of cardiac tamponade and bilateral pleural effusions. Procedures included a left video-assisted thoracoscopic surgery (VATS with pericardial window. We consider that it is important for all physicians to be aware of not only typical presentation but also atypical and unusual clinical picture of pericardial disease.

  9. Phase 3 Trials of Thermosensitive Ciprofloxacin Gel for Middle Ear Effusion in Children with Tubes.

    Science.gov (United States)

    Park, Albert H; White, David R; Moss, Jonathan R; Bear, Moraye; LeBel, Carl

    2016-08-01

    To investigate the efficacy, safety, and microbiology of a thermosensitive otic suspension of ciprofloxacin (OTO-201) in children with bilateral middle ear effusion undergoing tympanostomy tube placement. Two randomized, double-blind, sham-controlled phase 3 trials. Patients were randomized to intratympanic OTO-201 or sham. Children with bilateral middle ear effusion undergoing tympanostomy tube placement. Studies evaluated 532 patients (6 months to 17 years old) in a combined analysis of efficacy (treatment failure: presence of otorrhea, otic or systemic antibiotic use, lost to follow-up, missed visits), safety (audiometry, otoscopy, tympanometry), and microbiology. There was a lower cumulative proportion of treatment failures in patients receiving OTO-201 vs tympanostomy tubes alone (1) on days 4, 8, 15, and 29; (2) on day 15, primary end point (23.0% vs 45.1%; age-adjusted odds ratio, 0.341; P response) supported these findings. There were no drug-related serious adverse events; the most frequent treatment-emergent adverse events in both groups were pyrexia, postoperative pain, nasopharyngitis, cough, and upper respiratory tract infection. OTO-201 administration had no evidence of increased tube occlusion and no negative effect on audiometry, tympanometry, or otoscopy. Combined analysis of 2 phase 3 trials demonstrated a lower cumulative proportion of treatment failures through day 15 compared with TT alone when OTO-201 was administered intratympanically for otitis media with bilateral middle ear effusion at time of tympanostomy tube placement. © American Academy of Otolaryngology—Head and Neck Surgery Foundation 2016.

  10. Povidone-Iodine and Bleomycin in the Management of Malignant Pleural Effusion

    Directory of Open Access Journals (Sweden)

    Ali Asghar Alavi

    2011-09-01

    Full Text Available "nMalignant pleural effusion is a common complication in certain malignancies. Pleurodesis is the best option most of the time. The purpose of this study was to compare the choice of belomycin with povidone-iodine, which is not only determined by the efficacy of the agent but also by its cost, accessibility, safety, ease of administration and the number of administrations to achieve a complete response. We performed a randomized clinical trial on 39 patients presenting with symptomatic malignant pleural effusion. Patients were selected and randomly assigned to undergo chemical pleurodesis with either bleomycin or povidone-iodine. Primary characteristics of patients were assessed and graded before and after treatment concerning pain, dyspnea, and chest radiographs. A complete response was obtained in 79% of belomycin group and 75% of povidone-iodine group which was not statistically significant. Patients on belomycin treatment had a significantly lower score for dyspnea in one month follow up. This was significant after controlling for age, pain score and dyspnea score after drainage, using general linear model. Due to similar effect and significant cost advantage between bleomycin and povidone-iodine, we conclude that povidone- iodine is the agent of choice when utilizing pleurodesis for control of symptomatic malignant pleural effusions.

  11. Usefulness of pleural effusion antinuclear antibodies in the diagnosis of lupus pleuritis.

    Science.gov (United States)

    Toworakul, C; Kasitanon, N; Sukitawut, W; Wichinun, R; Louthrenoo, W

    2011-10-01

    We performed this study to determine sensitivity and specificity of pleural effusion antinuclear antibodies (ANA) at a titer of ≥1 : 160, and the ratio of pleural effusion to serum ANA of ≥1, to distinguish between pleural fluid from lupus pleuritis and other causes. A prospective study of 54 patients with pleural effusion (12 lupus pleuritis, seven parapneumonic effusion, 26 malignancy-associated pleural effusions, nine transudative effusions) was performed. ANA at a titer of ≥1 : 160 were found in 11 of 12 lupus pleuritis samples, and in four of 42 pleural effusions from non-systemic lupus erythematosus (SLE) patients. The pleural effusion ANA at a titer of ≥1 : 160 gave a sensitivity of 91.67% for lupus pleuritis, with a specificity of 83.33% when compared with all other pleural effusions, 90.91% when compared with exudative effusion (parapneumonic effusion and malignancy-associated effusion) and 55.56% when compared with the transudative pleural effusion group. Using the ratio of pleural effusion to serum ANA of ≥1, the sensitivity and the specificity decreased to 75.00% and 78.57%, respectively. This study provides further evidence that the pleural effusion ANA at a titer of ≥1 : 160 is a sensitive and specific diagnostic biomarker for lupus pleuritis in patients with lupus. However, pleural effusion ANA can occasionally be found in other conditions.

  12. A Study on Tuberculous Pleural Effusion

    Directory of Open Access Journals (Sweden)

    Zay Soe

    2010-03-01

    Full Text Available Background: Nearly one third of the global population i.e. two billion people are infected with mycobacteria tuberculosis and are at risk of developing the disease. Pleural effusion is one of the common complications of pulmonary tuberculosis. In this study, the clinical features, the positivity rate of microbiological procedures and blind pleural biopsies, radiological manifestations, biochemical and hematological profiles of serum and pleural fluid were analyzed. Objectives: To report our experience of 108 patients with tuberculous pleural effusion and discuss the clinical features, radiological findings, biochemical, cytological and microbiological analysis of pleural fluid, hematological and biochemical profiles of serum and positivity rate of microbiological procedures and blind pleural biopsies in these patients. Methods: This study was a hospital based descriptive cross sectional study performed at Chest Medical Ward, Yangon General Hospital, Myanmar, of study period from January 2004 through January 2005. A total of 108 patients were included. Thorough history taking and physical examinations, radiological findings, hematological and serum biochemical profiles were recorded. Pleural aspiration and biopsy were also performed. At least two pieces of pleural tissue were taken and one piece of each sample of pleural tissue was cultured for mycobacteria and the rest was sent for histological examination. Macroscopic findings, cytological, microbiological and biochemical analysis of pleural fluid were analyzed. Results: A total of 108 patients, 74 males and 34 females were included. Their mean age was 42.60 ± 16.34 (range 12-81 years. Common presentations were breathlessness (82.4%, cough (81.5%, fever (80.6%, and night sweat (78.7%, loss of appetite (74.1%, significant weight loss (72.2% and chest pain (67.6%. Only 39.3% of TB patients produced sputum in their history. Haemoptysis was present in only 7.4% of the patients. Regarding the

  13. Kaposi`s sarcoma; Sarcome de Kaposi

    Energy Technology Data Exchange (ETDEWEB)

    Kirova, Y.M.; Belembaogo, E.; Frikha, H.; Yu, S.J.; Le Bourgeois, J.P. [Hopital Henri-Mondor, 94 - Creteil (France)

    1997-09-01

    Moriz Kaposi was the first who, in 1872, described five patients presenting with `sarcoma idiopathicum multiple hemorrhagicum`. In 1912 Sternberg termed this disease Kaposi`s sarcoma. Since then various forms of this rare disease have been observed. In 1914 Hallenberg described the first cases of African or endemic Kaposi`s sarcoma. In the 1960`s the first reports discussing Kaposi`s sarcoma following organ transplantation and immunosuppressive therapy were published. After 1981, the epidemic form associated with the acquired immunodeficiency syndrome (AIDS) was described. All these forms, their history, treatment methods and the role of radiation therapy in the management of this rare malignancy are discussed, and the literature is reviewed. (authors)

  14. Pulmonary Artery Intimal Sarcoma: A Case Report

    Directory of Open Access Journals (Sweden)

    Joseph P. Kriz

    2016-04-01

    Full Text Available Pulmonary artery intimal sarcomas are rare and lethal malignant tumors that typically affect larger vessels: the aorta, inferior vena cava, and pulmonary arteries. Since symptoms and imaging of pulmonary arterial intimal sarcomas mimic pulmonary thromboembolism, the differential diagnosis of a patient presenting with chest pain, dyspnea, and filling defect within the pulmonary arteries should include intimal sarcoma. Often right ventricular failure is observed due to pulmonary hypertension caused by the obstructive effect of the tumor and concomitant chronic thromboembolism. We report the case of a 72-year-old African-American male with arterial intimal sarcoma of the left and right pulmonary artery with extension through the right artery into the bronchus and right lung.

  15. Treatment Option Overview (Adult Soft Tissue Sarcoma)

    Science.gov (United States)

    ... Vascular Tumors Treatment Research Adult Soft Tissue Sarcoma Treatment (PDQ®)–Patient Version General Information About Adult Soft ... dye reacts to the light. Certain factors affect treatment options and prognosis (chance of recovery). The treatment ...

  16. Treatment Options for Adult Soft Tissue Sarcoma

    Science.gov (United States)

    ... Vascular Tumors Treatment Research Adult Soft Tissue Sarcoma Treatment (PDQ®)–Patient Version General Information About Adult Soft ... dye reacts to the light. Certain factors affect treatment options and prognosis (chance of recovery). The treatment ...

  17. Childhood Soft Tissue Sarcoma: Treatment Information

    Science.gov (United States)

    ... Non-Hodgkin) Lymphoma (Hodgkin) Neuroblastoma Osteosarcoma Retinoblastoma Rhabdomyosarcoma Skin Cancer Soft Tissue Sarcoma Thyroid Cancer Cancer Resources Childhood Cancer Statistics Coping With Cancer CureSearch CancerCare App Late Effects ...

  18. Chemotherapy in Ewing′s sarcoma

    Directory of Open Access Journals (Sweden)

    Jain Sandeep

    2010-01-01

    Full Text Available Ewing′s sarcoma constitutes three per cent of all pediatric malignancies. Ewing′s sarcoma has generally been more responsive to chemotherapy than adult-type sarcomas, and chemotherapy is now recommended for all patients with this disease. It is essential to integrate local control measures in the form of surgery and/or radiotherapy at the appropriate time, along with chemotherapy to eradicate the disease. This approach has improved the survival substantially to the tune of 70% in localized disease, although outcome for metastatic disease remains dismal. Newer therapeutic approaches are required to improve outcome for metastatic and recurrent or refractory Ewing′s sarcoma in organized co-operative group trials.

  19. Neurogenic sarcomas of the neck in neurofibromatosis.

    Science.gov (United States)

    Martin, G; Kleinsasser, O

    1981-01-01

    Based on two observations and a review of the literature, the pathological and clinical findings in sarcomas of the neck in patients with neurofibromatosis are described. Histologically these neurogenic tumours show a manifold picture; in addition to spindle-cell sarcomas pleomorphic structures are to be found, which can be similar to rhabdomyo-, lipo-, chondro-, angio-, or osteogenic sarcomas so that a histological diagnosis of a neurogenic sarcoma cannot always be made without clinical details. Up to the present surgical treatment is preferred; the value of cytostatic therapy and irradiation is controversial. The results of treating these tumours are unsatisfactory. Of 29 cases reported in the literature, only two could be found in which the patient survived without a recurrence for more than five years.

  20. Histology and imaging of soft tissue sarcomas

    Energy Technology Data Exchange (ETDEWEB)

    Kind, Michele [Departement d' Imagerie Medicale, Institut Bergonie, 229 cours de l' Argonne, 33076 Bordeaux Cedex (France)], E-mail: kind@bergonie.org; Stock, Nathalie; Coindre, Jean Michel [Departement de Pathologie, Institut Bergonie, 229 cours de l' Argonne, 33076 Bordeaux Cedex (France); Universite Victor Segalen Bordeaux 2, 146 rue Leo Saignat, 33076 Bordeaux Cedex (France)

    2009-10-15

    Imaging and histology are two complementary morphological techniques which play a fundamental role in the diagnosis and management of soft tissue sarcomas. Imaging allows to identify some pseudosarcomatous benign lesions such as myositis ossificans, intramuscular hemangioma, angiomyolipoma, intramuscular lipoma, giant cell tumour of tendon sheath, desmoid tumour and elastofibroma. There is no formal criterion for diagnosing a sarcoma on magnetic resonance imaging (MRI) but malignancy is strongly suspected with the presence of necrosis and vascular, bone or joint invasion. Imaging may also suggest some histological types of sarcoma such as well-differentiated liposarcoma, dedifferentiated liposarcoma, synovial sarcoma or extraskeletal osteosarcoma. Imaging is also extremely helpful in determining the appropriate kind of sampling to carry out and in guiding the performance of a microbiopsy. The appearance observed on imaging should always be taken into consideration for the interpretation of the microbiopsy by the pathologist.

  1. Myeloid sarcoma of submandibular salivary gland

    Directory of Open Access Journals (Sweden)

    Federico Dagna

    2016-01-01

    Full Text Available Objective: To report a rare case of a myeloid sarcoma of submandibular salivary gland. Methods: A 65-year-old woman with a history of successfully treated myelodysplastic syndrome, presenting with periodic painful swelling of her right submandibular area. Results: Physical evaluation, ultrasound and CT scan revealed the presence of a 3-cm mass contiguous to the submandibular salivary gland. A core needle biopsy confirmed the diagnosis of myeloid sarcoma. Bone marrow biopsy was still showing complete remission and the submandibular gland was the only extramedullary site involved. The patient was submitted to chemotherapy. Conclusion: Myeloid sarcoma is a rare extramedullary neoplasm. It can virtually involve any anatomic site, but it usually involves lymph nodes, paranasal sinuses, skin, soft tissue and periostium. Myeloid sarcomas of salivary glands are very rare and ENTs should be aware of this disease in order to include it in the differential diagnosis of a solitary neck mass.

  2. Procholecystokinin as marker of human Ewing sarcomas

    DEFF Research Database (Denmark)

    Reubi, Jean Claude; Koefoed, Pernille; Hansen, Thomas von O

    2004-01-01

    PURPOSE: Ewing sarcoma is a rapidly growing mesenchymal tumor in young adults. Although it was shown previously to express the cholecystokinin (CCK) gene, it is unknown whether CCK gene expression is detectable at protein level in Ewing sarcoma tumor cell lines, in tumor tissue, and in plasma fro...... in human cancer; Ewing sarcomas synthesize and secrete proCCK that can be identified in plasma as circulating tumor marker....... Ewing sarcoma patients, and, if so, whether CCK peptides might play a role as tumor markers. EXPERIMENTAL DESIGN: CCK gene expression was evaluated with in situ hybridization or reverse transcription-PCR in tumor tissue. CCK precursors and bioactive CCK were measured with specific RIAs in tumor tissue...

  3. Drugs Approved for Soft Tissue Sarcoma

    Science.gov (United States)

    ... 2015 2014 2013 2012 Media Resources Media Contacts Multicultural Media ... This page lists cancer drugs approved by the Food and Drug Administration (FDA) for soft tissue sarcoma. The list includes ...

  4. Osteosarcoma with apparent Ewing sarcoma gene rearrangement

    OpenAIRE

    Mathias, Melissa; Chou, Alexander J; Meyers, Paul; Shukla, Neerav; Hameed, Meera; Agaram, Narasimhan; Wang, Lulu; Berger, Michael F.; Walsh, Michael; Kentsis, Alex

    2016-01-01

    Poorly differentiated round cell sarcomas present diagnostic challenges due to their variable morphology and lack of specific immunophenotypic markers. We present a case of a 15-year-old female with a tibial tumor that exhibited features of Ewing-like sarcoma, including apparent rearrangement of the EWSR1 gene. Hybridization capture-based next-generation DNA sequencing showed evidence of complex genomic rearrangements, absence of known pathogenic Ewing-like chromosome translocations, and dele...

  5. The importance of Src signaling in sarcoma

    OpenAIRE

    Chen, Quanchi; Zhou, Zifei; Shan, Liancheng; Zeng, Hui; Hua, Yingqi; Cai, Zhengdong

    2015-01-01

    Src is a tyrosine kinase that is of significance in tumor biology. The present review focuses on Src, its molecular structure, and role in cancer, in addition to its expression and function in sarcoma. In addition, the feasibility of Src as a potential drug target for the treatment of sarcoma is also discussed. Previous studies have suggested that Src has essential functions in cell proliferation, apoptosis, invasion, metastasis and the tumor microenvironment. Thus, it may be a potential targ...

  6. Eosinophilic Pleural Effusion: A Rare Manifestation of Hypereosinophilic Syndrome

    Directory of Open Access Journals (Sweden)

    Ndubuisi C. Okafor

    2009-01-01

    Full Text Available Several causes of eosinophilic pleural effusions have been described with malignancy being the commonest cause. Hypereosinophilic syndrome (HES is a rare disease and very few cases have been reported of HES presenting as eosinophilic pleural effusion (EPE. We report a case of a 26-year-old male who presented with shortness of breath. He had bilateral pleural effusions, generalized lymphadenopathy, splenomegaly, and leukocytosis with marked peripheral blood eosinophilia. The pleural fluid was exudative, with 25%–30% eosinophilis, and absence of neoplastic cells. Hypereosinophilic syndrome was diagnosed after other causes of eosinophilia were excluded. He continued to be dyspneic with persistent accumulation of eosinophilic pleural fluid, even after his peripheral eosinophil count had normalized in response to treatment. This patient represents a very unusual presentation of HES with dyspnea and pleural effusions and demonstrates that treatment based on response of peripheral eosinophil counts, as is currently recommended, may not always be clinically adequate.

  7. INVESTIGATION ON MODEL OF INFLAMMATORY PLEURAL EFFUSION IN GUINEA PIGS

    Institute of Scientific and Technical Information of China (English)

    冯源; 殷凯生; 王祥

    2002-01-01

    Objective To establish an animal model of inflammatory pleural effusion.Methods Forty guinea pigs were divided into two groups: experimental group with 7 subgroups and control group. In the experimental group the right chest cavity of each guinea pig was injected with 0.8~1.0 ml of 1% carrageenan, and guinea pigs of each subgroup were killed and observed respectively on day 1, 2, 3, 5, 7, 10 and day 14 after injection.Results Occurring on day 1(within 24 hours), pleural effusion reached the maximum on day 2~3 after injection, so did the neutrophil count in pleural effusion and inflammation of both pleura and lungs and then gradually decreased. The fibrosis and adhesion of pleura appeared on day 7 and were obvious on day 10. The encysted pleurisy was formed on day 14.Conclusion The carrageenan is an ideal pleural inflammatory inducer. This animal model is useful for studying pleural effusion.

  8. Acroangiodermatitis (Pseudo-Kaposi sarcoma

    Directory of Open Access Journals (Sweden)

    Satyendra Kumar Singh

    2014-01-01

    Full Text Available Acroangiodermatitis or Pseudo-Kaposi sarcoma is a rare angioproliferative entity, related to chronic venous insufficiency or certain other vascular anomalies. It is often associated with chronic venous insufficiency, arteriovenous malformation of the legs, chronic renal failure treated with dialysis, paralyzed legs and amputation stumps. We hereby describe a case of 45 year old female presenting with pitting pedal edema, multiple ulcers over bilateral lower limbs with irregular margins with erythema and hyperpigmentation of the surrounding skin. Color Doppler study of bilateral lower limbs was normal. Histopathological examination from one of the lesions showed hyperplastic epidermis, proliferation of capillaries in dermis, hemosiderin deposits and lymphocytic infiltrate. These features thus confirmed the diagnosis of Acroangiodermatitis.

  9. Undifferentiated Pleomorphic Sarcoma in Mandible.

    Science.gov (United States)

    Kim, Chul-Hwan; Jang, Jong-Won; Kim, Moon-Young; Kim, Yong-Hwan; Kim, Hang-Gul; Kim, Joo-Hwan

    2014-11-01

    Undifferentiated pleomorphic sarcoma (UPS), previously known as malignant fibrous histiocytoma, occurs commonly in the soft tissues in adult, but is rare in the maxillofacial region. It consists of undifferentiated mesenchymal tumor cells resembling histiocytes and fibroblasts. The purpose of this article is to report a case of UPS in the mandible. A 44-year-old patient presented with a painful growing mass in the mandible of two months' duration. Computed tomography and positron emission tomography-computed tomography revealed an ill-defined heterogenous, hypermetabolic mass about 4 cm in size in the left mandible invading adjacent soft tissues. A left mandiblulectomy and reconstruction with a fibular free flap were performed. Immunohistochemical study gave a diagnosis of UPS. The patient was referred for adjuvant chemotherapy after surgical removal of the tumor.

  10. Potential Therapeutic Targets in Uterine Sarcomas

    Directory of Open Access Journals (Sweden)

    Tine Cuppens

    2015-01-01

    Full Text Available Uterine sarcomas are rare tumors accounting for 3,4% of all uterine cancers. Even after radical hysterectomy, most patients relapse or present with distant metastases. The very limited clinical benefit of adjuvant cytotoxic treatments is reflected by high mortality rates, emphasizing the need for new treatment strategies. This review summarizes rising potential targets in four distinct subtypes of uterine sarcomas: leiomyosarcoma, low-grade and high-grade endometrial stromal sarcoma, and undifferentiated uterine sarcoma. Based on clinical reports, promising approaches for uterine leiomyosarcoma patients include inhibition of VEGF and mTOR signaling, preferably in combination with other targeted or cytotoxic compounds. Currently, the only targeted therapy approved in leiomyosarcoma patients is pazopanib, a multitargeted inhibitor blocking VEGFR, PDGFR, FGFR, and c-KIT. Additionally, preclinical evidence suggests effect of the inhibition of histone deacetylases, tyrosine kinase receptors, and the mitotic checkpoint protein aurora kinase A. In low-grade endometrial stromal sarcomas, antihormonal therapies including aromatase inhibitors and progestins have proven activity. Other potential targets are PDGFR, VEGFR, and histone deacetylases. In high-grade ESS that carry the YWHAE/FAM22A/B fusion gene, the generated 14-3-3 oncoprotein is a putative target, next to c-KIT and the Wnt pathway. The observation of heterogeneity within uterine sarcoma subtypes warrants a personalized treatment approach.

  11. Sarcoma sinovial anorretal: relato de caso

    Directory of Open Access Journals (Sweden)

    Hernán Augusto Centurión Sobral

    2006-03-01

    Full Text Available Os sarcomas são neoplasias que se originam das células mesenquimais primitivas, sendo raros na região anorretal. O objetivo é relatar um caso de sarcoma sinovial anorretal, neoplasia extremamente rara nesta localização. É descrito o caso de uma paciente de 77 anos que apresentava nodulação anal dolorosa e sangrante às evacuações, associada a puxo, tenesmo e perda ponderal. A lesão foi biopsiada e o estudo imunohistoquímico evidenciou sarcoma sinovial anorretal. A paciente foi submetida a amputação abdomino-perineal do reto, encontra-se assintomática, sem sinais de recidiva e em seguimento ambulatorial.Originated from mesenchymal cells, the sarcoma is rare in the anorectal area. The authors report a case of anorectal sinovial sarcoma, extremely rare in this location with no previous reports on literature. It's described a case of a 77 years old patient presenting an anal tumor, associated to pain, bleeding, tenesmus and weight loss. A synovial sarcoma was diagnosed after biopsy and imunohistochemical study. The pacient was submitted to a Miles procedure being assymptomatic and without any signs of disease in the last seven months.

  12. Potential Therapeutic Targets in Uterine Sarcomas

    Science.gov (United States)

    Cuppens, Tine; Tuyaerts, Sandra; Amant, Frédéric

    2015-01-01

    Uterine sarcomas are rare tumors accounting for 3,4% of all uterine cancers. Even after radical hysterectomy, most patients relapse or present with distant metastases. The very limited clinical benefit of adjuvant cytotoxic treatments is reflected by high mortality rates, emphasizing the need for new treatment strategies. This review summarizes rising potential targets in four distinct subtypes of uterine sarcomas: leiomyosarcoma, low-grade and high-grade endometrial stromal sarcoma, and undifferentiated uterine sarcoma. Based on clinical reports, promising approaches for uterine leiomyosarcoma patients include inhibition of VEGF and mTOR signaling, preferably in combination with other targeted or cytotoxic compounds. Currently, the only targeted therapy approved in leiomyosarcoma patients is pazopanib, a multitargeted inhibitor blocking VEGFR, PDGFR, FGFR, and c-KIT. Additionally, preclinical evidence suggests effect of the inhibition of histone deacetylases, tyrosine kinase receptors, and the mitotic checkpoint protein aurora kinase A. In low-grade endometrial stromal sarcomas, antihormonal therapies including aromatase inhibitors and progestins have proven activity. Other potential targets are PDGFR, VEGFR, and histone deacetylases. In high-grade ESS that carry the YWHAE/FAM22A/B fusion gene, the generated 14-3-3 oncoprotein is a putative target, next to c-KIT and the Wnt pathway. The observation of heterogeneity within uterine sarcoma subtypes warrants a personalized treatment approach. PMID:26576131

  13. Pericardiocentesis with cisplatin for malignant pericardial effusion and tamponade

    Institute of Scientific and Technical Information of China (English)

    Takatsugu; Oida; Kenji; Mimatsu; Hiso; Kano; Atsushi; Kawasaki; Youichi; Kuboi; Nobutada; Fukino; Sadao; Amano

    2010-01-01

    AIM:To evaluate the role and outcome of pericardiocentesis with intrapericardial cisplatin instillation for malignant pericardial effusion resulting from esophageal cancer. METHODS:We retrospectively studied 7 patients who underwent pericardiocentesis with intrapericardial cisplatin instillation for malignant pericardial effusion resulting from esophageal cancer.After pericardiocentesis,we performed catheterization of the pericardial space under ultrasonogram guidance.Malignant etiology of the pericardial f...

  14. Eosinophilic Endomyocarditis Combined With Pericardial and Pleural Effusion

    OpenAIRE

    You, Sung-Hye; Hong, Soon Jun; Ahn, Chul Min; Lim, Do-Sun

    2009-01-01

    Eosinophilic endomyocarditis is a manifestation of hypereosinophilic syndrome, characterized by prolonged (>6 months), unexplained peripheral blood eosinophilia (>1,500 cells/mm3) with end-organ damage in unknown causes. We report a case of a 42-year-old patient who developed eosinophilic endomyocarditis following upper respiratory tract symptoms for 2 months. Additionally, endomyocarditis was combined with massive pleural effusion and pericardial effusion, which have not been reported in Korea.

  15. Pleural Fluid Cholesterol in Differentiating Exudative and Transudative Pleural Effusion

    OpenAIRE

    Hamal, A. B.; Yogi, K. N.; N. Bam; Das, S. K.; Karn, R.

    2013-01-01

    Objectives. To study the diagnostic value of pleural fluid cholesterol in differentiating transudative and exudative pleural effusion. To compare pleural fluid cholesterol level for exudates with Light's criteria. Design. Cross sectional descriptive study. Settings. Medical wards of Tribhuvan University Teaching Hospital. Methods. Sixty two cases of pleural effusion with definite clinical diagnosis admitted in TUTH were taken and classified as transudates (19) and exudates (43). The parameter...

  16. Cyclooxygenase-2-prostaglandin E2-eicosanoid receptor inflammatory axis: a key player in Kaposi's sarcoma-associated herpes virus associated malignancies.

    Science.gov (United States)

    Paul, Arun George; Chandran, Bala; Sharma-Walia, Neelam

    2013-08-01

    The role of cyclooxygenase-2 (COX-2), its lipid metabolite prostaglandin E2 (PGE2), and Eicosanoid (EP) receptors (EP; 1-4) underlying the proinflammatory mechanistic aspects of Burkitt's lymphoma, nasopharyngeal carcinoma, cervical cancer, prostate cancer, colon cancer, and Kaposi's sarcoma (KS) is an active area of investigation. The tumorigenic potential of COX-2 and PGE2 through EP receptors forms the mechanistic context underlying the chemotherapeutic potential of nonsteroidal anti-inflammatory drugs (NSAIDs). Although role of the COX-2 is described in several viral associated malignancies, the biological significance of the COX-2/PGE2/EP receptor inflammatory axis is extensively studied only in Kaposi's sarcoma-associated herpes virus (KSHV/HHV-8) associated malignancies such as KS, a multifocal endothelial cell tumor and primary effusion lymphoma (PEL), a B cell-proliferative disorder. The purpose of this review is to summarize the salient findings delineating the molecular mechanisms downstream of COX-2 involving PGE2 secretion and its autocrine and paracrine interactions with EP receptors (EP1-4), COX-2/PGE2/EP receptor signaling regulating KSHV pathogenesis and latency. KSHV infection induces COX-2, PGE2 secretion, and EP receptor activation. The resulting signal cascades modulate the expression of KSHV latency genes (latency associated nuclear antigen-1 [LANA-1] and viral-Fas (TNFRSF6)-associated via death domain like interferon converting enzyme-like- inhibitory protein [vFLIP]). vFLIP was also shown to be crucial for the maintenance of COX-2 activation. The mutually interdependent interactions between viral proteins (LANA-1/vFLIP) and COX-2/PGE2/EP receptors was shown to play key roles in the biological mechanisms involved in KS and PEL pathogenesis such as blockage of apoptosis, cell cycle regulation, transformation, proliferation, angiogenesis, adhesion, invasion, and immune-suppression. Understanding the COX-2/PGE2/EP axis is very important to

  17. HITS-CLIP analysis uncovers a link between the Kaposi's sarcoma-associated herpesvirus ORF57 protein and host pre-mRNA metabolism.

    Directory of Open Access Journals (Sweden)

    Emi Sei

    2015-02-01

    Full Text Available The Kaposi's sarcoma associated herpesvirus (KSHV is an oncogenic virus that causes Kaposi's sarcoma, primary effusion lymphoma (PEL, and some forms of multicentric Castleman's disease. The KSHV ORF57 protein is a conserved posttranscriptional regulator of gene expression that is essential for virus replication. ORF57 is multifunctional, but most of its activities are directly linked to its ability to bind RNA. We globally identified virus and host RNAs bound by ORF57 during lytic reactivation in PEL cells using high-throughput sequencing of RNA isolated by cross-linking immunoprecipitation (HITS-CLIP. As expected, ORF57-bound RNA fragments mapped throughout the KSHV genome, including the known ORF57 ligand PAN RNA. In agreement with previously published ChIP results, we observed that ORF57 bound RNAs near the oriLyt regions of the genome. Examination of the host RNA fragments revealed that a subset of the ORF57-bound RNAs was derived from transcript 5' ends. The position of these 5'-bound fragments correlated closely with the 5'-most exon-intron junction of the pre-mRNA. We selected four candidates (BTG1, EGR1, ZFP36, and TNFSF9 and analyzed their pre-mRNA and mRNA levels during lytic phase. Analysis of both steady-state and newly made RNAs revealed that these candidate ORF57-bound pre-mRNAs persisted for longer periods of time throughout infection than control RNAs, consistent with a role for ORF57 in pre-mRNA metabolism. In addition, exogenous expression of ORF57 was sufficient to increase the pre-mRNA levels and, in one case, the mRNA levels of the putative ORF57 targets. These results demonstrate that ORF57 interacts with specific host pre-mRNAs during lytic reactivation and alters their processing, likely by stabilizing pre-mRNAs. These data suggest that ORF57 is involved in modulating host gene expression in addition to KSHV gene expression during lytic reactivation.

  18. Black Pleural Effusion: A Unique Presentation of Metastatic Melanoma

    Directory of Open Access Journals (Sweden)

    Akansha Chhabra

    2015-05-01

    Full Text Available Metastatic melanoma is a rare form of skin cancer, but one that comes with a high mortality rate. Pulmonary involvement is frequently seen in metastatic melanoma with only 2% of malignant melanoma patients with thorax metastasis presenting with pleural effusions. Herein, we report an extremely rare case of black pleural effusion from thoracic metastasis of cutaneous malignant melanoma. A 74-year-old man with known metastatic melanoma presented with a 1-month history of worsening lower back and hip pain and was found to have extensive osseous metastatic disease and multiple compression fractures. The patient underwent an uneventful kyphoplasty; however, the following day, he became acutely hypoxic and tachypneic with increased oxygen requirements. Radiographic evaluation revealed new bilateral pleural effusions. Bedside thoracentesis revealed a densely exudative, lymphocyte-predominant black effusion. Cytological examination showed numerous neoplastic cells with melanin deposition. A diagnosis of thoracic metastasis of malignant melanoma was established based on the gross and microscopic appearance of the pleural fluid. To the best of our knowledge, this is the first reported case of black pleural effusions secondary to metastatic melanoma in the United States. Despite the rarity of this presentation, it is important to determine the etiology of the black pleural effusion and to keep metastatic melanoma as a differential diagnosis.

  19. MicroRNA-16 suppresses metastasis in an orthotopic, but not autochthonous, mouse model of soft tissue sarcoma

    Directory of Open Access Journals (Sweden)

    Mohit Sachdeva

    2015-08-01

    Full Text Available MicroRNAs (miRNAs can regulate tumor cell invasion and metastasis in a tumor-specific manner. We recently demonstrated that global downregulation of miRNAs after deleting dicer can promote development of distant metastases in a mouse model of primary soft tissue sarcoma (STS. In this study, we identified miRNAs that are differentially downregulated in metastatic STS in both human and mouse, and investigated the role of these miRNAs in metastasis. miRNA- TaqMan PCR arrays showed a global downregulation of miRNAs in metastatic human sarcomas. Similar analysis in mouse metastatic sarcomas revealed overlap for several downregulated miRNAs including miR-16, miR-103, miR-146a, miR-223, miR-342 and miR-511. Restoration of these downregulated miRNAs in mouse primary sarcoma cell lines showed that miR-16, but not other downregulated miRNAs, was able to significantly suppress both migration and invasion in vitro, without altering cell proliferation. In addition, orthotopic transplantation of a sarcoma cell line stably expressing miR-16 into the muscle of immunocompromised mice revealed that restoration of miR-16 can significantly decrease lung metastasis in vivo. However, no change in the rate of lung metastasis was observed when miR-16 was deleted in mouse primary sarcomas at sarcoma initiation. Taken together, these results indicate that miR-16 can have metastasis-suppressing properties both in vitro and in vivo. However, the loss-of-function experiments in autochthonous tumors indicate that loss of miR-16 is not sufficient to promote metastasis in vivo.

  20. CNS Metastases from Bone and Soft Tissue Sarcomas in Children, Adolescents, and Young Adults: Are They Really So Rare?

    Science.gov (United States)

    Duczkowska, Agnieszka; Duczkowski, Marek; Bragoszewska, Hanna; Romaniuk-Doroszewska, Anna; Iwanowska, Beata; Szkudlinska-Pawlak, Sylwia; Madzik, Jaroslaw; Bilska, Katarzyna; Raciborska, Anna

    2017-01-01

    Purpose. To check whether primary involvement of brain/spinal cord by bone/soft tissue sarcomas' metastases in children is as rare as described and to present various morphological forms of bone/soft tissue sarcomas' CNS metastases. Methods. Patients with first diagnosis in 1999–2014 treated at single center were included with whole course of disease evaluation. Brain/spinal canal magnetic resonance imaging (MRI)/computed tomography were performed in cases suspicious for CNS metastases. Extension from skull/vertebral column metastases was excluded. Results. 550 patients were included. MRI revealed CNS metastases in 19 patients (incidence 3.45%), 14 boys, aged 5–22 years. There were 12/250 osteosarcoma cases, 2/200 Ewing's sarcoma, 1/50 chondrosarcoma, 3/49 rhabdomyosarcoma (RMS), and 1/1 malignant mesenchymoma. There were 10 single metastases and 7 cases of multiple ones; in 2 RMS cases only leptomeningeal spread in brain and spinal cord was found. Calcified metastases were found in 3 patients and hemorrhagic in 4. In one RMS patient there were numerous solid, cystic, hemorrhagic lesions and leptomeningeal spread. Conclusions. CNS metastases are rare and late in children with bone/soft tissue sarcomas, although in our material more frequent (3.45%) than in other reports (0.7%). Hematogenous spread to brain and hemorrhagic and calcified lesions dominated in osteosarcoma. Ewing sarcoma tended to metastasize to skull bones. Soft tissue sarcomas presented various morphological forms.

  1. Ewing’s sarcoma of the maxillary sinus

    Directory of Open Access Journals (Sweden)

    Firas Nasser

    2015-07-01

    Full Text Available Ewing’s sarcoma is typically an aggressive, poorly differentiated tumor affecting children and young adults, it accounts for 4–6% of all primary bone tumors and facial primary localizations occur in only 1–4% of all cases, mostly in the mandible and calvaria. Paranasal sinus involvement is rare. A 22-year-old female was reviewed in Oral & Cranio Maxillofacial Surgery Department. She complained of swelling of the right paranasal area, of one-month duration, progressively increasing in size and associated with pain. The medical history was unremarkable, Contrast Enhanced Computed Tomography scan showed a destructive lesion of the anterior wall of the right maxillary sinus reaching up to the medial wall of the maxillary sinus, other paranasal sinus appearance was normal. Incisional biopsy proved it to be Ewing’s Sarcoma. She was treated by chemotherapy using Vincristine, Adriamycin, and Cyclophosphamide alternating with Etoposide & Ifosfamide and Radiotherapy, and this resulted in complete regression of the tumor. Repeated PET scans every 6 months did not suggest any recurrence of the right maxillary sinus tumor. We concluded that treatment by induction chemotherapy followed by radiation therapy leads to a favorable outcome in the above described case, avoiding the morbidity that can result from surgical options.

  2. Surgical Management of Massive Pericardial Effusion and Predictors for Development of Constrictive Pericarditis in a Resource Limited Setting

    Directory of Open Access Journals (Sweden)

    Emeka B. Kesieme

    2016-01-01

    Full Text Available Background. The diagnosis and treatment of massive pericardial effusion and cardiac tamponade have evolved over the years with a tendency towards a more comprehensive diagnostic workup and less traumatic intervention. Method. We reviewed and analysed the data of 32 consecutive patients who underwent surgery on account of massive pericardial effusion and cardiac tamponade in a semiurban university hospital in Nigeria from February 2010 to February 2016. Results. The majority of patients (34.4% were between 31 and 40 years. Fourteen patients (43.8% presented with clinical and echocardiographic feature of cardiac tamponade. The majority of patients (59.4% presented with haemorrhagic pericardial effusion and the average volume of fluid drained intraoperatively was 846 mL  ± 67 mL. Pericardium was thickened in 50% of cases. Subxiphoid pericardiostomy was performed under local anaesthesia in 28 cases. No postoperative recurrence was observed; however 5 patients developed features of constrictive pericarditis. The relationship between pericardial thickness and development of pericardial constriction was statistically significant (p=0.004. Conclusion. Subxiphoid pericardiostomy is a very effective way of treating massive pericardial effusion. Removing tube after adequate drainage (50 mL/day and treatment of primary pathology are key to preventing recurrence. There is also a need to follow up patients to detect pericardial constriction especially those with thickened pericardium.

  3. Surgical Management of Massive Pericardial Effusion and Predictors for Development of Constrictive Pericarditis in a Resource Limited Setting.

    Science.gov (United States)

    Kesieme, Emeka B; Okokhere, Peter O; Iruolagbe, Christopher Ojemiega; Odike, Angela; Owobu, Clifford; Akhigbe, Theophilus

    2016-01-01

    Background. The diagnosis and treatment of massive pericardial effusion and cardiac tamponade have evolved over the years with a tendency towards a more comprehensive diagnostic workup and less traumatic intervention. Method. We reviewed and analysed the data of 32 consecutive patients who underwent surgery on account of massive pericardial effusion and cardiac tamponade in a semiurban university hospital in Nigeria from February 2010 to February 2016. Results. The majority of patients (34.4%) were between 31 and 40 years. Fourteen patients (43.8%) presented with clinical and echocardiographic feature of cardiac tamponade. The majority of patients (59.4%) presented with haemorrhagic pericardial effusion and the average volume of fluid drained intraoperatively was 846 mL  ± 67 mL. Pericardium was thickened in 50% of cases. Subxiphoid pericardiostomy was performed under local anaesthesia in 28 cases. No postoperative recurrence was observed; however 5 patients developed features of constrictive pericarditis. The relationship between pericardial thickness and development of pericardial constriction was statistically significant (p = 0.004). Conclusion. Subxiphoid pericardiostomy is a very effective way of treating massive pericardial effusion. Removing tube after adequate drainage (50 mL/day) and treatment of primary pathology are key to preventing recurrence. There is also a need to follow up patients to detect pericardial constriction especially those with thickened pericardium.

  4. Prostatic sarcoma of the Ewing family in a 33-year-old male – A case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Lukas Esch

    2016-04-01

    Full Text Available Ewing sarcoma is the second most common primary bone tumor seen in children and adolescents, typically presenting between 10 and 20 years of age. Extraosseous sarcomas of the Ewing family in adults are rare. We report a manifestation of this tumor entity in the periprostatic tissue of a 33-year-old male and discuss our treatment approach. Transrectal biopsy is a feasible and simple diagnostic tool for unclear pelvic masses. Multi-modal therapy and central registries are needed to gain knowledge of rare pelvic tumors like Ewing sarcoma.

  5. Kaposi's sarcoma-associated herpesvirus microRNA single-nucleotide polymorphisms identified in clinical samples can affect microRNA processing, level of expression, and silencing activity.

    Science.gov (United States)

    Han, Soo-Jin; Marshall, Vickie; Barsov, Eugene; Quiñones, Octavio; Ray, Alex; Labo, Nazzarena; Trivett, Matthew; Ott, David; Renne, Rolf; Whitby, Denise

    2013-11-01

    Kaposi's sarcoma-associated herpesvirus (KSHV) encodes 12 pre-microRNAs that can produce 25 KSHV mature microRNAs. We previously reported single-nucleotide polymorphisms (SNPs) in KSHV-encoded pre-microRNA and mature microRNA sequences from clinical samples (V. Marshall et al., J. Infect. Dis., 195:645-659, 2007). To determine whether microRNA SNPs affect pre-microRNA processing and, ultimately, mature microRNA expression levels, we performed a detailed comparative analysis of (i) mature microRNA expression levels, (ii) in vitro Drosha/Dicer processing, and (iii) RNA-induced silencing complex-dependent targeting of wild-type (wt) and variant microRNA genes. Expression of pairs of wt and variant pre-microRNAs from retroviral vectors and measurement of KSHV mature microRNA expression by real-time reverse transcription-PCR (RT-PCR) revealed differential expression levels that correlated with the presence of specific sequence polymorphisms. Measurement of KSHV mature microRNA expression in a panel of primary effusion lymphoma cell lines by real-time RT-PCR recapitulated some observed expression differences but suggested a more complex relationship between sequence differences and expression of mature microRNA. Furthermore, in vitro maturation assays demonstrated significant SNP-associated changes in Drosha/DGCR8 and/or Dicer processing. These data demonstrate that SNPs within KSHV-encoded pre-microRNAs are associated with differential microRNA expression levels. Given the multiple reports on the involvement of microRNAs in cancer, the biological significance of these phenotypic and genotypic variants merits further studies in patients with KSHV-associated malignancies.

  6. DNA damage, somatic aneuploidy, and malignant sarcoma susceptibility in muscular dystrophies.

    Directory of Open Access Journals (Sweden)

    Wolfgang M Schmidt

    2011-04-01

    Full Text Available Albeit genetically highly heterogeneous, muscular dystrophies (MDs share a convergent pathology leading to muscle wasting accompanied by proliferation of fibrous and fatty tissue, suggesting a common MD-pathomechanism. Here we show that mutations in muscular dystrophy genes (Dmd, Dysf, Capn3, Large lead to the spontaneous formation of skeletal muscle-derived malignant tumors in mice, presenting as mixed rhabdomyo-, fibro-, and liposarcomas. Primary MD-gene defects and strain background strongly influence sarcoma incidence, latency, localization, and gender prevalence. Combined loss of dystrophin and dysferlin, as well as dystrophin and calpain-3, leads to accelerated tumor formation. Irrespective of the primary gene defects, all MD sarcomas share non-random genomic alterations including frequent losses of tumor suppressors (Cdkn2a, Nf1, amplification of oncogenes (Met, Jun, recurrent duplications of whole chromosomes 8 and 15, and DNA damage. Remarkably, these sarcoma-specific genetic lesions are already regularly present in skeletal muscles in aged MD mice even prior to sarcoma development. Accordingly, we show also that skeletal muscle from human muscular dystrophy patients is affected by gross genomic instability, represented by DNA double-strand breaks and age-related accumulation of aneusomies. These novel aspects of molecular pathologies common to muscular dystrophies and tumor biology will potentially influence the strategies to combat these diseases.

  7. Pancreatic Sarcoma Mimicking Pseudocyst After Pancreatitis: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Cheng-Chi Lee

    2015-09-01

    Full Text Available A 45-year-old female presented with abdominal pain for 3 days without trauma history. The pain was located over the epigastric area with radiation to the back. A pancreatic pseudocyst was impressed initially according to clinical symptoms, laboratory data, and image studies. However, rapid progression of the lesions was noted later after discharge. Following image studies showed circumscribed tumors in the head, body, and tail regions of the pancreas. Surgery with the Whipple operation was performed during the second admission. The post operation course was uneventful. Pathologic results showed sarcoma of the pancreas. Primary pancreatic sarcomas are extremely rare lesions accounting for < 0.1% of all pancreatic malignancies. Pancreatic sarcomas tend to be aggressive and have a poor prognosis. In addition, < 5% of pancreatic tumors are cystic. The rarest cystic neoplasm is also the primary pancreatic sarcoma, with only a few cases having been documented. However, it should be considered in the differential diagnosis of pancreatic cystic lesions.

  8. DNA damage, somatic aneuploidy, and malignant sarcoma susceptibility in muscular dystrophies.

    Science.gov (United States)

    Schmidt, Wolfgang M; Uddin, Mohammed H; Dysek, Sandra; Moser-Thier, Karin; Pirker, Christine; Höger, Harald; Ambros, Inge M; Ambros, Peter F; Berger, Walter; Bittner, Reginald E

    2011-04-01

    Albeit genetically highly heterogeneous, muscular dystrophies (MDs) share a convergent pathology leading to muscle wasting accompanied by proliferation of fibrous and fatty tissue, suggesting a common MD-pathomechanism. Here we show that mutations in muscular dystrophy genes (Dmd, Dysf, Capn3, Large) lead to the spontaneous formation of skeletal muscle-derived malignant tumors in mice, presenting as mixed rhabdomyo-, fibro-, and liposarcomas. Primary MD-gene defects and strain background strongly influence sarcoma incidence, latency, localization, and gender prevalence. Combined loss of dystrophin and dysferlin, as well as dystrophin and calpain-3, leads to accelerated tumor formation. Irrespective of the primary gene defects, all MD sarcomas share non-random genomic alterations including frequent losses of tumor suppressors (Cdkn2a, Nf1), amplification of oncogenes (Met, Jun), recurrent duplications of whole chromosomes 8 and 15, and DNA damage. Remarkably, these sarcoma-specific genetic lesions are already regularly present in skeletal muscles in aged MD mice even prior to sarcoma development. Accordingly, we show also that skeletal muscle from human muscular dystrophy patients is affected by gross genomic instability, represented by DNA double-strand breaks and age-related accumulation of aneusomies. These novel aspects of molecular pathologies common to muscular dystrophies and tumor biology will potentially influence the strategies to combat these diseases.

  9. Identification of second malignancies on effusions and fine-needle aspirates using a panel of monoclonal antibodies.

    Science.gov (United States)

    Mottolese, M.; Venturo, I.; Rinaldi, M.; Lopez, M.; Bigotti, G.; Benevolo, M.; Natali, P. G.

    1997-01-01

    The longer survival of neoplastic patients achieved through improvements of therapeutic regimens has increased the relative risk of developing a second primary tumour (SPT). In this context, conventional cytopathology can define tumour histotype only in a small fraction of cases. In this study, we have evaluated whether selected combinations of monoclonal antibodies (MAbs) to tumour-associated antigens (TAAs) can increase the accuracy of conventional morphology in detecting second primary tumours (SPTs) in two particularly difficult areas of cytodiagnosis, namely that of effusions and pulmonary fine-needle aspirates (FNAs). The immunocytochemical (ICC) analysis of 334 cytological specimens demonstrated that the use of our selected panel of MAbs could allow a more efficient identification of SPTs in comparison with conventional morphology. This diagnostic improvement was statistically significant (P < 0.0001). The present findings show that the immunophenotyping of effusions and FNAs, providing a more accurate and objective identification of SPTs, may have significant therapeutic and epidemiological relevance. PMID:9052413

  10. What Are the Risk Factors for Soft Tissue Sarcoma?

    Science.gov (United States)

    ... not been proven to cause soft tissue sarcomas. Arsenic has also been linked to a type of ... Tissue Sarcoma Causes, Risk Factors, and Prevention Early Detection, Diagnosis, and Staging Treatment After Treatment Back To ...

  11. Effect of the Japanese herbal medicine, Boiogito, on the osteoarthritis of the knee with joint effusion

    Directory of Open Access Journals (Sweden)

    Majima Tokifumi

    2012-01-01

    Full Text Available Abstract Background Boiogito (Japanese herbal medicine, Tsumura Co. Tokyo, Japan contains sinomenin which inhibits inflammatory reactions. Since sinomenine is a principle component of the Boiogito, there is a possibility of it being effective on osteoarthritis (OA of the knee with joint effusion. However, there is no report concerning the effectiveness of Boiogito on knee OA. The objective of the present study is to investigate the therapeutic effect of Boiogito on OA of the knee associated with joint effusion in a comparative study among randomly assigned groups. Methods Study was performed using 50 patients who were diagnosed with primary osteoarthritis of the knee with joint effusion. The patients were randomly assigned to two groups: one group (25 patients using both loxoprofen (2-{4-[(2-oxocyclopentyl methyl]} propanoic acid and Boiogito and the other group (25 patients using loxoprofen, and were evaluated during a 12 week observation period. The assessment parameters including knee scores in the Knee Society Rating System including Knee score and Functional scores, amount of joint effusion by joint puncture in clinically detected cases, the 36-items short form of the Medical Outcome Study Questionnaire (SF-36 as a measurement of health related quality of life were used. Results The knee scores based on the Knee Society Rating System were improved in both groups. The staircase climbing up and down ability in the Knee society rating system functional score was significantly improved in the group using Boiogito and loxoprofen compared to the loxoprofen group. In the evaluation using SF-36, significant improvements were found in the scores in both groups in physical functioning after 12 weeks. The amount of joint fluid was significantly decreased at 4, 8 and 12 weeks compared to pre-administration baseline in the group using Boiogito and loxoprofen. A side effect of Boiogito, dry mouth, was found in one case. The symptom was mild and improved

  12. Limitations of Single Slice Dynamic Contrast Enhanced MR in Pharmacokinetic Modeling of Bone Sarcomas

    Energy Technology Data Exchange (ETDEWEB)

    Toms, Andoni P. (Dept. of Radiology, The Norfolk and Norwich Univ. Hospital, Norwich, Norfolk (United Kingdom)); White, Lawrence M.; Bleakney, Robert R. (Dept. of Medical Imaging, Mount Sinai Hospital, Toronto, ON (Canada)); Kandel, Rita (Dept. of Pathology and Laboratory Medicine, Mount Sinai Hospital, Toronto, ON (Canada)); Noseworthy, Michael (Health Sciences Centre, Faculty of Health Sciences, McMaster Univ., Hamilton, ON (Canada)); Lee, Shepstone (Institute of Health, Univ. of East Anglia, Norwich, Norfolk (United Kingdom)); Blackstein, Martin E. (Dept. of Oncology, Mount Sinai Hospital, Toronto, ON (Canada)); Wunder, Jay (Musculoskeletal Oncology Unit, Mount Sinai Hospital, Toronto, ON (Canada))

    2009-06-15

    Background: Single slice dynamic contrast-enhanced magnetic resonance imaging (DCE-MRI) appears to provide perfusion data about sarcomas in vivo that correlate with tumor necrosis on equivalent pathological sections. However, sarcomas are heterogeneous and therefore single slice DCE-MRI may not correlate with total tumor necrosis. Purpose: To determine whether changes in pharmacokinetic modeling of DCE-MRI, during chemotherapy for primary bone sarcomas correlated with histological measures of total tumor necrosis. Material and Methods: Twelve patients with appendicular primary bone sarcomas were included in the study. Each patient had DCE-MRI before, and after completion, of pre-operative chemotherapy. The mean arterial slope (A), endothelial permeability coefficient (Ktrans), and extravascular extracellular volume (Ve) were derived from each data set using a modified two compartment pharmacokinetic model. Total tumor necrosis rates were compared with changes in A, Ktrans, and Ve. Results: Six patients had total tumor necrosis of =90% and six had a measure of <90%. The median percentage changes in A, Ktrans, and Ve for the =90% necrosis group were -52.5% (-83 to 6), -66% (-82 to 26), and 23.5% (-26 to 40), respectively. For the <90% necrosis group, A = - 35% (-75 to 132), Ktrans= - 53 (-66 to 149) and Ve= - 14.5% (-42 to 40). One patient with >90% necrosis had increases in all three measures. Comparison of the two groups generated P-values of 0.699 for A, 0.18 for Ktrans, and 0.31 for Ve. Conclusion: There was no statistically significant correlation between changes in pharmacokinetic perfusion parameters and total tumor necrosis. When using single slice DCE-MRI heterogeneous histology of primary bone sarcomas and repair mediated angiogenesis might both be confounding factors

  13. Evaluation of minimal disseminated disease in cryopreserved ovarian tissue from bone and soft tissue sarcoma patients.

    Science.gov (United States)

    Dolmans, M M; Iwahara, Y; Donnez, J; Soares, M; Vaerman, J L; Amorim, C A; Poirel, H

    2016-10-01

    What is the risk of finding malignant cells in cryopreserved ovarian tissue from sarcoma patients? Minimal disseminated disease (MDD) was not detected in frozen-thawed ovarian tissue from 26 patients by any of the sensitive methods applied. In case of leukemia, the risk of malignant cell transmission through the graft is well known and widely documented. However, for bone cancer, like Ewing sarcoma or osteosarcoma, only a small number of case reports, have been published. These cancers often affect prepubertal girls, in whom ovarian tissue cryopreservation and transplantation is the only option to preserve fertility. The presence of malignant cells in cryopreserved ovarian tissue from patients with bone/soft tissue sarcoma was investigated with disease-specific markers for each patient, using immunohistochemistry (IHC), FISH and real-time quantitative RT-PCR (qPCR), with the original tumor serving as a positive control. Forty-eight sarcoma patients were enrolled in the study, 12 of whom subsequently died. In each case, tissue from the primary tumor was investigated in order to identify markers (immunohistochemical and/or molecular) to analyze the ovarian tissue case by case. Ovarian tissue from osteosarcoma (n = 15), liposarcoma (n = 1) and undifferentiated sarcoma (n = 5) patients could not be evaluated, as no specific markers were detected by FISH or sensitive IHC in any of their primary tumoral tissue. One patient with Li-Fraumeni syndrome was also excluded from the study. IHC analyses were therefore performed on ovarian tissue from 26 patients and qPCR on 19. The primary tumors involved were Ewing sarcoma family of tumors (n = 14), rhabdomyosarcoma (n = 7), synovial sarcoma (n = 2), clear cell sarcoma (n = 2) and a malignant peripheral nerve sheath tumor (n = 1). MDD was not detected in any of the 26 analyzed samples using sensitive techniques in this largest reported series, even from patients who subsequently died and/or those who presented

  14. Diagnostic importance of zinc in the clarification of pleural effusions etiology

    Directory of Open Access Journals (Sweden)

    Ranković Boško

    2002-01-01

    Full Text Available Concentration of zinc in blood serum and effusion was determined in 104 patients with the pleural effusion of different etiology. The importance of zinc concentration in serum and effusion was analyzed, as well as their relation regarding the differential diagnosis of pleural effusion. It was established that the isolated zinc concentrations in serum and pleural effusion could not be used separately either in differing transudates from exudates or in the diagnosis of the pleural diseases. The average value of zinc in the pleural effusion in relation to the serum value in patients with tuberculosis effusion was 1.37, higher than 1 in all patients and was significantly different from the average value of the ratio 0,74 in patients with nonspecific and malignant pleural effusions. The relation of zinc concentration in the effusion and serum higher than 1.0 reliably indicated the presence of tuberculous pleurisy.

  15. [Otitis media with effusion in children younger than 1 year].

    Science.gov (United States)

    Di Francesco, Renata Cantisani; Barros, Vivian Boschesi; Ramos, Rafael

    2016-06-01

    To determine the prevalence of otitis media with effusion in children younger than 1 year and its association with the season of the year, artificial feeding, environmental and perinatal factors. Retrospective study of 184 randomly included medical records from a total of 982 healthy infants evaluated for hearing screening tests. Diagnosis of otitis media with effusion was based on otoscopy (amber-gold color, fluid level, handle of malleus position), type B tympanometric curves and absence of otoacoustic emissions. Incomplete medical records or those describing acute otitis media, upper respiratory tract infections on the assessment day or in the last 3 months, neuropathies and craniofacial anomalies were excluded. Data such as gestational age, birth weight, Apgar score, type of feeding and day care attendance were compared between children with and without otitis media with effusion through likelihood tests and multivariate analysis. 25.3% of 184 infants had otitis media with bilateral effusion; 9.2% had unilateral. In infants with otitis media, the following were observed: chronological age of 9.6±1.7 months; gestational age >38 weeks in 43.4% and birth weight >2,500g in 48.4%. Otitis media with effusion was associated with winter/fall, artificial feeding, Apgar score otitis media with effusion. Otitis media with effusion was found in about one third of children younger than 1 year and was mainly associated with artificial feeding. Copyright © 2015 Sociedade de Pediatria de São Paulo. Publicado por Elsevier Editora Ltda. All rights reserved.

  16. Otitis media with effusion in children younger than 1 year

    Directory of Open Access Journals (Sweden)

    Renata Cantisani Di Francesco

    2016-06-01

    Full Text Available Abstract Objective: To determine the prevalence of otitis media with effusion in children younger than 1 year and its association with the season of the year, artificial feeding, environmental and perinatal factors. Methods: Retrospective study of 184 randomly included medical records from a total of 982 healthy infants evaluated for hearing screening tests. Diagnosis of otitis media with effusion was based on otoscopy (amber-gold color, fluid level, handle of malleus position, type B tympanometric curves and absence of otoacoustic emissions. Incomplete medical records or those describing acute otitis media, upper respiratory tract infections on the assessment day or in the last 3 months, neuropathies and craniofacial anomalies were excluded. Data such as gestational age, birth weight, Apgar score, type of feeding and day care attendance were compared between children with and without otitis media with effusion through likelihood tests and multivariate analysis. Results: 25.3% of 184 infants had otitis media with bilateral effusion; 9.2% had unilateral. In infants with otitis media, the following were observed: chronological age of 9.6±1.7 months; gestational age >38 weeks in 43.4% and birth weight >2500g in 48.4%. Otitis media with effusion was associated with winter/fall, artificial feeding, Apgar score <7 and day care attendance. The multivariate analysis showed that artificial feeding is the factor most often associated to otitis media with effusion. Conclusions: Otitis media with effusion was found in about one third of children younger than 1 year and was mainly associated with artificial feeding.

  17. Otitis media with effusion in children younger than 1 year

    Science.gov (United States)

    Di Francesco, Renata Cantisani; Barros, Vivian Boschesi; Ramos, Rafael

    2016-01-01

    Abstract Objective: To determine the prevalence of otitis media with effusion in children younger than 1 year and its association with the season of the year, artificial feeding, environmental and perinatal factors. Methods: Retrospective study of 184 randomly included medical records from a total of 982 healthy infants evaluated for hearing screening tests. Diagnosis of otitis media with effusion was based on otoscopy (amber-gold color, fluid level, handle of malleus position), type B tympanometric curves and absence of otoacoustic emissions. Incomplete medical records or those describing acute otitis media, upper respiratory tract infections on the assessment day or in the last 3 months, neuropathies and craniofacial anomalies were excluded. Data such as gestational age, birth weight, Apgar score, type of feeding and day care attendance were compared between children with and without otitis media with effusion through likelihood tests and multivariate analysis. Results: 25.3% of 184 infants had otitis media with bilateral effusion; 9.2% had unilateral. In infants with otitis media, the following were observed: chronological age of 9.6±1.7 months; gestational age >38 weeks in 43.4% and birth weight >2500g in 48.4%. Otitis media with effusion was associated with winter/fall, artificial feeding, Apgar score <7 and day care attendance. The multivariate analysis showed that artificial feeding is the factor most often associated to otitis media with effusion. Conclusions: Otitis media with effusion was found in about one third of children younger than 1 year and was mainly associated with artificial feeding. PMID:26559603

  18. Vismodegib and Gamma-Secretase/Notch Signalling Pathway Inhibitor RO4929097 in Treating Patients With Advanced or Metastatic Sarcoma

    Science.gov (United States)

    2016-06-09

    Adult Alveolar Soft Part Sarcoma; Adult Angiosarcoma; Adult Desmoplastic Small Round Cell Tumor; Adult Epithelioid Hemangioendothelioma; Adult Epithelioid Sarcoma; Adult Extraskeletal Myxoid Chondrosarcoma; Adult Extraskeletal Osteosarcoma; Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Mesenchymoma; Adult Malignant Peripheral Nerve Sheath Tumor; Adult Rhabdomyosarcoma; Adult Synovial Sarcoma; Adult Unclassified Pleomorphic Sarcoma; Chondrosarcoma; Clear Cell Sarcoma of the Kidney; Conjunctival Kaposi Sarcoma; Dermatofibrosarcoma Protuberans; Gastrointestinal Stromal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Osteosarcoma; Ovarian Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Adult Unclassified Pleomorphic Sarcoma of Bone; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Kaposi Sarcoma; Recurrent Osteosarcoma; Recurrent Uterine Corpus Sarcoma; Small Intestine Leiomyosarcoma; Stage III Adult Soft Tissue Sarcoma; Stage III Uterine Sarcoma; Stage IV Adult Soft Tissue Sarcoma; Stage IV Uterine Sarcoma; Unclassified Pleomorphic Sarcoma of Bone

  19. Epithelioid sarcoma : Still an only surgically curable disease

    NARCIS (Netherlands)

    de Visscher, Sebastiaan A. H. J.; van Ginkel, Robbert J.; Wobbes, Theo; Veth, Rene P. H.; ten Heuvel, Suzanne E.; Suurmeijer, Albert J. H.; Hoekstra, Harad J.

    2006-01-01

    BACKGROUND. Epithelioid sarcoma is a rare soft tissue sarcoma with a known high propensity for locoregional recurrence and distant metastases. The clinical behavior and prognostic factors that influence the survival of patients with epithelioid sarcoma were studied. METHODS. Twenty-three patients,

  20. Immunotherapy for Bone and Soft Tissue Sarcomas

    Directory of Open Access Journals (Sweden)

    Takenori Uehara

    2015-01-01

    Full Text Available Although multimodal therapies including surgery, chemotherapy, and radiotherapy have improved clinical outcomes of patients with bone and soft tissue sarcomas, the prognosis of patients has plateaued over these 20 years. Immunotherapies have shown the effectiveness for several types of advanced tumors. Immunotherapies, such as cytokine therapies, vaccinations, and adoptive cell transfers, have also been investigated for bone and soft tissue sarcomas. Cytokine therapies with interleukin-2 or interferons have limited efficacy because of their cytotoxicities. Liposomal muramyl tripeptide phosphatidylethanolamine (L-MTP-PE, an activator of the innate immune system, has been approved as adjuvant therapeutics in combination with conventional chemotherapy in Europe, which has improved the 5-year overall survival of patients. Vaccinations and transfer of T cells transduced to express chimeric antigen receptors have shown some efficacy for sarcomas. Ipilimumab and nivolumab are monoclonal antibodies designed to inhibit immune checkpoint mechanisms. These antibodies have recently been shown to be effective for patients with melanoma and also investigated for patients with sarcomas. In this review, we provide an overview of various trials of immunotherapies for bone and soft tissue sarcomas, and discuss their potential as adjuvant therapies in combination with conventional therapies.