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Sample records for bone myeloid sarcoma

  1. Intraparenchymal Myeloid Sarcoma and Subsequent Spinal Myeloid Sarcoma for Acute Myeloblastic Leukemia

    OpenAIRE

    Eom, Ki Seong; Kim, Tae Young

    2011-01-01

    Myeloid sarcoma is a solid, extramedullary tumor composed of leukemic myeloblasts or immature myeloid cells. Intraparenchymal myeloid sarcoma without the involvement of the skull or meninges is extremely rare. Here, we present the case of a 49-year-old man who developed intraparenchymal myeloid sarcoma on the left cerebellum after allogeneic bone marrow transplantation (BMT). He received radiotherapy after complete removal of intraparenchymal myeloid sarcoma, but he was diagnosed spinal myelo...

  2. Recurrent isolated extramedullary relapses as granulocytic sarcomas following allogeneic bone marrow transplantation for acute myeloid leukemia

    OpenAIRE

    Au, WY; Chan, ACL; Lie, AKW; Chen, FE; Liang, R.; Kwong, YL

    1998-01-01

    Isolated extramedullary relapses as granulocytic sarcomas (GS) following allogeneic bone marrow transplantation (BMT) for acute myeloid leukemia (AML) are rare events. We describe three such patients who presented with a unique pattern of GS relapse post-BMT. The clinical features included repeated relapses in multiple sites, absence of marrow involvement, and prolonged survival. Fluorescence in situ hybridization (FISH) demonstrated persistence of donor hematopoiesis despite disseminated GS....

  3. Myeloid Sarcoma of the Skin

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    Ruksan Elal

    2013-06-01

    Full Text Available Myeloid sarcoma (MS (granulocytic sarcoma, extramedullary myeloid tumor, chloroma is a rare malignant extramedullary neoplasms of myeloid precursor cells. Skin is one of the most common localization of MS. The tumor may be isolated or associated with acute myeloid leukemia, chronic myeloid leukemia, primary myelofibrosis, hypereosinophilic syndrome and polycythemia vera. MS is a disease that is rare and difficult to diagnose. Perhaps the most important factor in the diagnosis is suggestion of MS. In this article, clinicopathological features of skin localized MS case are presented.

  4. [Cytogenetics of bone sarcomas].

    Science.gov (United States)

    Vagner-Capodano, A M; Poitout, D

    There has been much progress in the cytogenesis, and molecular biology of bone tumours such as Ewing sarcoma and osteosarcomas, greatly improving diagnostic possibilities and prognosis. Ewing's sarcoma is an indifferentiated sarcoma with round cells which usually occurs in children or adolescents. Ewing's sarcoma corresponds to 6% of all bone tumours. Histologically Ewing's sarcoma belongs to a group of small round cell tumours including neuroblastoma, embryon and alveolar rhabdomyosarcoma and non-Hodgkin's lymphoma. Differential diagnosis is difficult. Cytogenetic examinations can now differentiate Ewing's sarcoma from other small round cell tumours. There is a specific 11:12 translocation (q24; q12) which can be used as a marker. PMID:8785922

  5. Cutaneous myeloid sarcoma of the penile foreskin.

    Science.gov (United States)

    Afrose, Ruquiya; Nebhnani, Deepa; Wadhwa, Neelam

    2015-01-01

    Myeloid sarcoma, considered to herald the onset of a blast crisis in the setting of chronic myeloproliferative neoplasm/dysplasia, typically presents during the course of the disorder. Cutaneous involvement is uncommon and lesions on genital skin are seldom seen. We present a case of a well-differentiated myeloid sarcoma in the penile foreskin in an apparently healthy 29-year-old male presenting with phimosis. The unusual composition of the inflammatory cell infiltrate, and characteristic sparing of dermal blood vessels, nerves and smooth muscle fibres led to the correct diagnosis. Absence of commonly observed changes in the circumcision skin like those of balanitis xerotica was also helpful. Detailed hematological work up revealed a previously undiagnosed chronic myeloid leukemia in chronic phase. The patient also had simultaneous priapism, another rare presentation of chronic myeloid leukemia. One year hence, the patient is in hematological remission with no evidence of extramedullary disease. Although priapism has been described as a rare presenting symptom in chronic myeloid leukemia, the present case is unique as this is the first time a cutaneous myeloid sarcoma has been documented in the penile foreskin. PMID:24913300

  6. Recurrence of a t(8;21-Positive Acute Myeloid Leukemia in the Form of a Granulocytic Sarcoma Involving Cranial Bones: A Diagnostic and Therapeutic Challenge

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    Ambra Di Veroli

    2013-01-01

    Full Text Available Granulocytic sarcoma (GS is a rare extramedullary solid tumor defined as an accumulation of myeloblasts or immature myeloid cells. It can cooccur with or precede the acute myeloid leukemia (AML as well as following treated AML. The incidence of GS in AML patients is 3–8% but it significantly rises in M2 FAB subtype AML. This variety of AML harbors t(8;21 in up to 20–25% of cases (especially in children and black ones of African origin and, at a molecular level, it is characterized by the generation of a fusion gene known as RUNX1-RUNX1T1. Approximately 10% of M2 AML patients will develop GS, as a consequence, the t(8;21 and the relative transcript represent the most common cytogenetic and molecular abnormalities in GS. FLT3-ITD mutation was rarely described in AML patients presenting with GS. FLT3 ITD is generally strongly associated with poor prognosis in AML, and is rarely reported in patients with t(8;21. GS presentation is extremely variable depending on organs involved; in general, cranial bones and sinus are very rarely affected sites. We report a rare case of GS occurring as a recurrence of a previously treated t(8;21, FLT3-ITD positive AML, involving mastoid bones and paravertebral tissues.

  7. Myeloid sarcoma in a child with acute myeloblastic leukaemia

    International Nuclear Information System (INIS)

    We report a rare occurrence of myeloid sarcoma in a 7 years old child with acute myeloblastic leukaemia (AML - FAB type M2). He presented with fever, generalized weakness, bilateral proptosis and left parotid swelling. CT scan revealed a mass in paranasal sinuses extending into brain and retro-orbital region. Diagnosis of AML M2 was made on bone marrow aspiration and special stains. Induction therapy for AML was given according to standard protocol. The extramedullary lesion as well as the acute leukaemia went into complete remission. (author)

  8. Myeloid sarcoma of the rib: An atypical isolated chest finding

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    Antonio Raucci

    2015-03-01

    Systemic treatment was administered and currently neither systemic nor local relapse has been identified. Our experience suggests surgical resection could be a valid treatment in isolated myeloid sarcoma patients.

  9. Myeloid Sarcoma Presenting with Leukemoid Reaction in a Child Treated for Acute Lymphoblastic Leukemia

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    Aylin Canbolat Ayhan

    2014-01-01

    Full Text Available Background. Myeloid sarcoma is an extramedullary neoplasm of immature myeloid cells. Our study reports a presentation of myeloid sarcoma which presented with severe leukemoid reaction as a secondary malignancy in a patient who was treated for acute lymphoblastic leukemia previously. The case emphasizes the difficulties in diagnosis of patients who do not have concomitant leukemia. Case Presentation. A 6-year-old girl who was treated for acute lymphoblastic leukemia previously presented with fatigue, paleness, and hepatosplenomegaly. Peripheral blood smear and bone marrow aspirate examination did not demonstrate any blasts in spite of severe leukemoid reaction with a white cell count 158000/mm3. FDG/PET CT revealed slight uptake in cervical and supraclavicular lymph nodes. Excisional lymph node biopsy was performed from these lymph nodes and it showed myeloid sarcoma. Conclusion. Myeloid sarcoma can develop as a secondary malignancy in children who are treated for acute lymphoblastic leukemia. It can be associated with severe leukemoid reaction and diagnosis may be difficult if there is not concomitant leukemia. PET/CT is helpful in such cases.

  10. Myeloid Sarcoma: The Clinician's Point of View

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    M. Malagola

    2011-01-01

    Full Text Available Myeloid Sarcoma may occur in patients with an acute or chronic myeloproliferative disorder as well as de novo, with no apparent sign or symptom of concomitant haematological disease. The patients are preferentially young male and the site of disease localization may vary from central nervous system to pleura and thorax, with a common involvement of the reticuloendothelial system. The disease often shows chromosomal rearrangements, involving chromosomes 7, 8 and 3 and sometimes a complex karyotype (more than 3 abnormalities is detected at diagnosis. The prognosis of this disease is dismal and only high-dose chemotherapy with autologous or allogeneic stem cells transplantation (auto or allo-SCT may be potentially curative. In the absence of definitive elements that can define the prognosis of extra-medullary localization of “standard risk” AML, Clinicians should pursue the collection of data from different Centres and design of homogeneous treatment strategies, that could integrate standard chemotherapy with specific approaches, such as radiotherapy, transplant procedures or, in selected cases (such as those displaying molecular abnormalities involving protein tyrosine-kinases, molecularly targeted therapies.

  11. Clinicopathologic features and responses to radiotherapy of myeloid sarcoma

    International Nuclear Information System (INIS)

    To evaluate clinicopathological features, radiotherapeutic parameters, and their associations with responses to radiotherapy (RT) in patients with myeloid sarcoma (MS). We reviewed 20 patients receiving RT for MS lesions (in 43 RT courses) and analyzed the patients’ clinicopathologic features and radiotherapeutic parameters, and their associations with complete responses (CR) to RT using Fisher’s exact test and univariate logistic regression analysis. Generalized Estimating Equation was used to analyze all 43 irradiated lesions and account for the correlations in RT responses among lesions from the same patient. We found that the underlying hematological diseases of the evaluated patients were acute myeloid leukemia (AML) in 14 patients (70%), chronic myeloid leukemia in 4 patients (20%), myelodysplastic syndrome with AML transformation in one patient (5%), and de novo MS in one patient (5%). Most patients (55%) received RT for MS at the time of relapse following bone marrow transplantation (BMT). The most common cytogenetic abnormality was t(8;21)(q22;q22). The median RT dose of 20 Gy (range 6–35 Gy), administered in 1.5-3.5 Gy fractions, provided a 63% CR rate. RT dose, sex, cytogenetics, and bone marrow status at the time of RT had no significant effect on CR. Younger age (<50 y, P = 0.06), BMT prior to RT (P = 0.05), and underlying AML (P = 0.05) were marginally associated with higher CR to RT. Our results indicate that a modest RT dose (20-30 Gy) achieves good local control of MS. Age, previous BMT, and underlying hematologic disease can affect RT response

  12. Paget's sarcoma of bone

    International Nuclear Information System (INIS)

    The most sinister complication of Paget's disease is malignant degeneration. The true etiology of Paget's disease remains unproven and unknown. The relative incidence of sarcoma ranges from 0.9% to 20% depending upon the population source. The anatomical distribution of lesions is similar to that of uncomplicated Paget's disease except for the high incidence in the humerus and rare occurrence in the spine. Approximately 50% of the lesions histologically represent osteosarcomas, with fibrosarcoma, fibrous histiocytoma, chondrosarcoma and giant cell tumor rarely occurring. The radiographic presentation is most commonly lytic, but sclerotic and mixed patterns occur. Cortical destruction and soft tissue mass are the cardinal radiographic signs. Malignant periosteal response is seldom found associated with Paget's sarcoma. The most common sites are femur, humerus, pelvis, skull and tibia. The prognosis is fatal at a very rapid rate. There are but a few isolated cases of long-term survival reported. (orig.)

  13. Myeloid Sarcoma: An Unusual Presentation of Acute Promyelocytic Leukemia Causing Spinal Cord Compression

    OpenAIRE

    Tay Za Kyaw; Jayaranee A.s. Maniam; Ping Chong Bee; Edmund Fui Min Chin; Veera Sekaran Nadarajan; Hemalatha Shanmugam; Khairul Azmi Abd Kadir

    2012-01-01

    Acute promyelocytic leukemia with concurrent myeloid sarcoma is a rare clinical event. Herein we describe a patient that presented with back pain and bilateral leg weakness caused by spinal cord compression due to extramedullary deposition of leukemic cells. Acute promyelocytic leukemia was suspected based on immunophenotypic findings of malignant cells in bone marrow aspirate. The diagnosis was confirmed by the presence of PML-RARα fusion copies. MRI showed multiple hyperintense changes on t...

  14. Primary clear cell sarcoma of bone

    International Nuclear Information System (INIS)

    Clear cell sarcoma is a rare soft tissue sarcoma of young adults with melanocytic differentiation. It occurs predominantly in the soft tissue of extremities, typically involving tendons and aponeuroses. Primary clear cell sarcoma of bone is extremely rare. We report a case of primary clear cell sarcoma of the right first metatarsal in a 48-year-old woman and provide a literature review of the entity. (orig.)

  15. Myeloid sarcoma developing in pre-existing pyoderma gangrenosum

    DEFF Research Database (Denmark)

    Kristensen, Ida Bruun; Møller, Hanne; Kjaerskov, Mette Wanscher;

    2009-01-01

    We report here a case of pyoderma gangrenosum in a patient with myelodysplastic syndrome developing into myeloid sarcoma as a sign of transformation to acute leukaemia. The patient was treated successfully with intensive chemotherapy and achieved complete remission, and her otherwise expanding ul...

  16. Myeloid Sarcoma: An Unusual Case of Mediastinal Mass and Malignant Pleural Effusion with Review of Literature.

    Science.gov (United States)

    Sahu, Kamal Kant; Tyagi, Ruchita; Law, Arjun Datt; Khadwal, Alka; Prakash, Gaurav; Rajwanshi, Arvind; Varma, Subhash Chander; Malhotra, Pankaj

    2015-12-01

    Myeloid sarcoma is an extramedullary tumor seen most commonly in patients with acute myeloid leukemia and less frequently in chronic myeloid leukemia, myelodysplastic syndrome and rarely, in an isolated form without any other underlying malignancy. Malignant pleural effusion in hematological malignancies is rare when compared with solid tumors. We present an unusual case of myeloid sarcoma in which a mediastinal mass with pleural effusion was the initial presentation. A 27 year old gentleman presented with complaints of fever, chest pain and swelling in the anterior chest wall for 6 months. Examination revealed a lump measuring 5 × 5 cm on the left side of the chest wall. Hematological evaluation showed hemoglobin-14.2 g/dL, platelet count-233 × 10(9)/L, TLC-117 × 10(6)/L with normal differential counts. Contrast enhanced computerised tomography (CECT) confirmed the presence of a soft tissue mass in the superior mediastinum abutting against the chest wall. Core biopsy was suggestive of myeloid sarcoma and immunohistochemistry was positive for myeloperoxidase and negative for CD3, CD 20 and CD 23. Pleural fluid analysis showed the presence of malignant cells. Bone marrow examination did not show an excess of blasts. A final diagnosis of extramedullary myeloid sarcoma with malignant pleural effusion was made. The patient was given induction chemotherapy (3 + 7 regimen) with daunorubicin and cytosine arabinoside. Repeat CECT done on day 28 showed complete resolution of pleural effusion and significant reduction in the size of mediastinal mass. The patient has successfully completed three cycles of consolidation therapy following which there has been complete resolution of the mass. He remains asymptomatic on close follow up. PMID:26306072

  17. Hypercalcemia and diffuse osteolytic lesions in a 45-year-old patient with myeloid sarcoma with megakaryocytic differentiation

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    Aditya Goud

    2016-04-01

    Full Text Available Acute megakaryocytic leukemia is a rare form of acute myeloid leukemia that carries a poor prognosis. As most cases of osteolytic lesions are due to plasma cell and myeloid malignancies, maintaining a broad differential directly influences clinical course. We document a 45-year-old patient with progressive constitutional symptoms, osteolytic bone lesions in the setting of hypercalcemia, who developed acutely worsening pancytopenia. The diagnosis of myeloid sarcoma with megakaryocytic differentiation was made after obtaining tissue from osteolytic bone that stained strong for CD34. Immunohistochemical testing underscores the importance of how serologic and urine testing remains limited and can delay early diagnosis in this disease.

  18. Differential diagnosis of isolated myeloid sarcoma: a case report and review of the literature

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    Patrick A. Hagen

    2015-06-01

    Full Text Available Myeloid sarcoma (MS is a rare disease entity identified as a variety of manifestations defined by the occurrence of extramedullary myeloid cell masses with or without bone marrow involvement. This case describes an unusual presentation of isolated MS in a 60-year-old otherwise healthy male, who initially presented to his primary care physician with vague abdominal pain. After extensive workup including three omental biopsies, umbilical core biopsy, and inguinal lymph node biopsy, he was ultimately diagnosed with isolated MS with extensive extramedullary tumor burden. Despite advanced extramedullary disease, peripheral cell counts were normal and bilateral bone marrow biopsies unremarkable with normal cellular lineages, morphology, and cytogenetics. The patient underwent induction chemotherapy and is now greater than 100 days post myeloablative unrelated donor marrow transplantation with no evidence of disease recurrence and 100% donor status with full chimerism. This case demonstrates that making a prompt diagnosis with rapid initiation of treatment in myeloid sarcoma can be challenging due to its varied clinical presentation, cytomorphology, cytochemistry, and cytogenetic overlap with other lymphoid malignancies. Once a diagnosis of MS has been made, moving quickly to induction therapy is important. Several studies have shown that improved overall survival is attained when MS is treated as acute myeloid leukemia and increased survival is noted for patients undergoing bone marrow transplantation. Further prospective studies are needed to elucidate the many remaining questions in regards to the natural history, prognosis, and optimal treatment strategies for this deadly disease.

  19. Differential Diagnosis of Isolated Myeloid Sarcoma: A Case Report and Review of the Literature.

    Science.gov (United States)

    Hagen, Patrick A; Singh, Charanjeet; Hart, Melissa; Blaes, Anne H

    2015-06-01

    Myeloid sarcoma (MS) is a rare disease entity identified as a variety of manifestations defined by the occurrence of extramedullary myeloid cell masses with or without bone marrow involvement. This case describes an unusual presentation of isolated MS in a 60-year-old otherwise healthy male, who initially presented to his primary care physician with vague abdominal pain. After extensive workup including three omental biopsies, umbilical core biopsy, and inguinal lymph node biopsy, he was ultimately diagnosed with isolated MS with extensive extramedullary tumor burden. Despite advanced extramedullary disease, peripheral cell counts were normal and bilateral bone marrow biopsies unremarkable with normal cellular lineages, morphology, and cytogenetics. The patient underwent induction chemotherapy and is now greater than 100 days post myeloablative unrelated donor marrow transplantation with no evidence of disease recurrence and 100% donor status with full chimerism. This case demonstrates that making a prompt diagnosis with rapid initiation of treatment in myeloid sarcoma can be challenging due to its varied clinical presentation, cytomorphology, cytochemistry, and cytogenetic overlap with other lymphoid malignancies. Once a diagnosis of MS has been made, moving quickly to induction therapy is important. Several studies have shown that improved overall survival is attained when MS is treated as acute myeloid leukemia and increased survival is noted for patients undergoing bone marrow transplantation. Further prospective studies are needed to elucidate the many remaining questions in regards to the natural history, prognosis, and optimal treatment strategies for this deadly disease. PMID:26330997

  20. Diagnostic confusion resulting from CD56 expression by cutaneous myeloid sarcoma

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    Sheeja T. Pullarkat

    2009-12-01

    Full Text Available Myeloid sarcomas are tumor masses composed of aggregates of malignant myeloid precursors in extramedullary sites including the skin. We report a case of myeloid sarcoma in a patient who presented with an ear lobe mass and facial nerve paralysis. Expression of CD56 by the malignant cells led to an initial misdiagnosis as Merkel cell tumor. Comprehensive pathological evaluation confirmed the diagnosis of myeloid sarcoma with aberrant expression of CD56 and carrying the translocation t(8;21 (q22;q22. Aberrant antigen expression by cutaneous myeloid sarcomas can cause diagnostic confusion with other cutaneous neoplasms. This is especially relevant when myeloid sarcoma is the sole manifestation of acute myeloid leukemia.

  1. Brachial Plexopathy due to Myeloid Sarcoma in a Patient With Acute Myeloid Leukemia After Allogenic Peripheral Blood Stem Cell Transplantation

    OpenAIRE

    Ha, Yumi; Sung, Duk Hyun; Park, Yoonhong; Kim, Du Hwan

    2013-01-01

    Myeloid sarcoma is a solid, extramedullary tumor comprising of immature myeloid cells. It may occur in any organ; however, the invasion of peripheral nervous system is rare. Herein, we report the case of myeloid sarcoma on the brachial plexus. A 37-year-old woman with acute myelogenous leukemia achieved complete remission after chemotherapy. One year later, she presented right shoulder pain, progressive weakness in the right upper extremity and hypesthesia. Based on magnetic resonance images ...

  2. Myeloid sarcoma of the urinary bladder with cutaneous tumour seeding after percutaneous suprapubic catheterization.

    Science.gov (United States)

    Geok Chin, Tan; Masir, Noraidah; Noor Hussin, Hamidah; Mohd Sidik, Shiran; Boon Cheok, Lee; Yean, Thean

    2011-06-01

    Myeloid sarcoma (MS) is a rare extramedullary myeloid tumour. It has been reported in various sites, including lymph node, bone, skin, soft tissue, various organs and the CNS. It may precede or occur concurrently with acute myeloid leukemia. Urinary bladder involvement is extremely uncommon. We report a 70-year-old female who had MS of the urinary bladder, presented with frank and persistent hematuria associated with lower abdominal pain. She subsequently had tumour seeding in the abdominal skin via percutaneous suprapubic catheter. Tumours from both the urinary bladder and skin showed immature cells that were immunoreactive toward LCA (focal), MPO (strong), CD99 (weak) and CD117 (weak). Summary of cases in the literature is presented. The potential of its misdiagnosis and the useful markers for the diagnosis of MS are discussed. PMID:21874752

  3. Case Report: Myelodysplastic syndrome- associated myeloid sarcoma: an unusual clinical presentation of a rare disease.

    Science.gov (United States)

    Horvath, Emoke; Demian, Smaranda; Nagy, Elod

    2016-01-01

    Myeloid sarcoma results from the extramedullary homing and proliferation of immature myeloid precursors. We present the timeline, events and diagnostic pitfalls related to a 66 year-old male patient's case, admitted to the Hematology Clinic for pancytopenia, fever, weight loss and fatigue. The severe cytopenia and the few blasts observed in his blood smear indicated a bone marrow biopsy. The bone marrow showed hypercellularity and multilineage dysplasia with the presence of 15% myeloblasts. After the biopsy, he promptly developed paraplegia and nuclear magnetic resonance revealed an epidural tumour which was then resected.In the epidural tumour mass blast-like, round cells were observed with a complex immunophenotype, characterized by myeloperoxidase, CD117, CD15, CD99, leucocyte common antigen positivity and a high Ki-67 proliferation index. Considering the main differential diagnostic issues, the final diagnosis was stated as myelodysplastic syndrome-associated myeloid sarcoma. The prognosis was unfavourable, the bone marrow was quickly invaded by proliferating blast cells, and despite chemotherapy attempts, the patient died. PMID:27019694

  4. Rare myeloid sarcoma/acute myeloid leukemia with adrenal mass after allogeneic mobilization peripheral blood stem cell transplantation

    Institute of Scientific and Technical Information of China (English)

    Ya-Fei Wang; Qian Li; Wen-Gui Xu; Jian-Yu Xiao; Qing-Song Pang; Qing Yang; Yi-Zuo Zhang

    2013-01-01

    Myeloid sarcoma (MS) is a rare hematological neoplasm that develops either de novo or concurrently with acute myeloid leukemia (AML). This neoplasm can also be an initial manifestation of relapse in a previously treated AML that is in remission. A 44-year-old male patient was diagnosed with testis MS in a local hospital in August 2010. Atfer one month, bone marrow biopsy and aspiration conifrmed the diagnosis of AML. Allogeneic mobilization peripheral blood stem cell transplantation was performed, with the sister of the patient as donor, after complete remission (CR) was achieved by chemotherapy. Five months after treatment, an adrenal mass was detected by positron emission tomography-computed tomography (PET-CT). Radiotherapy was performed for the localized mass after a multidisciplinary team (MDT) discussion. hTe patient is still alive as of May 2013, with no evidence of recurrent MS or leukemia.

  5. Rare myeloid sarcoma/acute myeloid leukemia with adrenal mass after allogeneic mobilization peripheral blood stem cell transplantation

    International Nuclear Information System (INIS)

    Myeloid sarcoma (MS) is a rare hematological neoplasm that develops either de novo or concurrently with acute myeloid leukemia (AML). This neoplasm can also be an initial manifestation of relapse in a previously treated AML that is in remission. A 44-year-old male patient was diagnosed with testis MS in a local hospital in August 2010. After one month, bone marrow biopsy and aspiration confirmed the diagnosis of AML. Allogeneic mobilization peripheral blood stem cell transplantation was performed, with the sister of the patient as donor, after complete remission (CR) was achieved by chemotherapy. Five months after treatment, an adrenal mass was detected by positron emission tomography-computed tomography (PET-CT). Radiotherapy was performed for the localized mass after a multidisciplinary team (MDT) discussion. The patient is still alive as of May 2013, with no evidence of recurrent MS or leukemia

  6. Brachial Plexopathy due to Myeloid Sarcoma in a Patient With Acute Myeloid Leukemia After Allogenic Peripheral Blood Stem Cell Transplantation.

    Science.gov (United States)

    Ha, Yumi; Sung, Duk Hyun; Park, Yoonhong; Kim, Du Hwan

    2013-04-01

    Myeloid sarcoma is a solid, extramedullary tumor comprising of immature myeloid cells. It may occur in any organ; however, the invasion of peripheral nervous system is rare. Herein, we report the case of myeloid sarcoma on the brachial plexus. A 37-year-old woman with acute myelogenous leukemia achieved complete remission after chemotherapy. One year later, she presented right shoulder pain, progressive weakness in the right upper extremity and hypesthesia. Based on magnetic resonance images (MRI) and electrophysiologic study, a provisional diagnosis of brachial plexus neuritis was done and hence steroid pulse therapy was carried out. Three months later the patient presented epigastric pain. After upper gastrointestinal endoscopy, myeloid sarcoma of gastrointestinal tract was confirmed pathologically. Moreover, 18-fluoride fluorodeoxyglucose positron emission tomography showed a fusiform shaped mass lesion at the brachial plexus overlapping with previous high signal lesion on the MRI. Therefore, we concluded the final diagnosis as brachial plexopathy due to myeloid sarcoma. PMID:23705126

  7. A Case Report on the Progression of Myeloid Sarcoma to Form Multiple Metastatic Deposits without Developing Acute Myeloid Leukaemia

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    Sunita Kohli

    2015-01-01

    Full Text Available Introduction. Myeloid sarcomas (MS are rare tumours occurring at extramedullary sites. They are usually associated with other haematology disorders such as acute myeloid leukaemia, myelodysplastic syndrome, and chronic myeloproliferative neoplasms. They frequently occur with a diagnosis of acute myeloid leukaemia (AML or with relapse of preexisting disease. Patients with myeloid sarcomas without history or evidence of myeloid leukaemia typically progress to form AML. Case Presentation. A case report of a patient diagnosed with an isolated myeloid sarcoma that rarely did not transform to AML but instead spread to form multiple myeloid sarcomas throughout the body. Discussion. This case identifies the risk of metastatic spread of these tumours rather than the development of AML which is poorly documented in the literature, due to the rarity of cases, and may be significant in the investigation and management of isolated myeloid sarcomas. This case highlights the need for clinicians to consider repeat cross-sectional imaging to investigate unexplained clinical decline or symptoms, when there is no sign of AML progression and to consider radiotherapy treatment early.

  8. [Grading of soft tissue and bone sarcomas].

    Science.gov (United States)

    Petersen, I; Wardelmann, E

    2016-07-01

    Malignancy grading is an essential element in the classification of sarcomas. It correlates with the prognosis of the disease and the risk of metastasis. This article presents the grading schemes for soft tissue, bone and pediatric sarcomas. It summarizes the histological criteria of the Federation Nationale des Centres de Lutte Contre le Cancer (FNCLCC) system and the Pediatric Oncology Group as well as the grading of bone tumors by the College of American Pathologists (CAP). Furthermore, the potential relevance of gene expression signatures, the complexity index in sarcoma (CINSARC) and single genetic alterations (p53, MDM2, p16, SWI/SNF, EWSR1 fusions and PAX3/PAX7-FOXO1 fusions) for the prognosis of sarcomas are discussed. PMID:27384333

  9. Sarcomas of Soft Tissue and Bone.

    Science.gov (United States)

    Ferrari, Andrea; Dirksen, Uta; Bielack, Stefan

    2016-01-01

    The definition of soft tissue and bone sarcomas include a large group of several heterogeneous subtypes of mesenchymal origin that may occur at any age. Among the different sarcomas, rhabdomyosarcoma, synovial sarcoma, Ewing sarcoma and osteosarcoma are aggressive high-grade malignancies that often arise in adolescents and young adults. Managing these malignancies in patients in this age bracket poses various clinical problems, also because different therapeutic approaches are sometimes adopted by pediatric and adult oncologists, even though they are dealing with the same condition. Cooperation between pediatric oncologists and adult medical oncologists is a key step in order to assure the best treatment to these patients, preferably through their inclusion into international clinical trials. PMID:27595362

  10. Myeloid Sarcoma and Acute Myelomonocytic Leukemia in an Adolescent with Tetrasomy 8: Staging With {sup 18}F-FDG PET/CT

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    Makis, William [Brandon Regional Health Centre, Brandon (Canada); Rakheja, Rajan; Lavoie, Josee; Marc Hickeson [McGill Univ. Health Centre, Brandon (Canada)

    2012-06-15

    Tetrasomy 8 is a relatively rare chromosomal abnormality that has been reported in only 33 cases in hematologic disorders, It is known for its association with aggressive acute myeloid leukemia (AML) and myeloid sarcoma and is considered a very poor prognostic factor. Myeloid sarcoma is a rare hematologic malignancy characterized by tumor masses consisting of immature myeloid cells, presenting at an extramedullary site. We present a case of a 17-year-old boy referred for an {sup 18}F-FDG PET/CT for the evaluation of pleural masses and spinal bone lesions seen on CT, after presenting with a 4 month history of chest pain. The PET/CT revealed extensive FDG-avid extrame-dullary disease in the soft tissues of the chest, abdomen, and pelvis, which were biopsy-proven to be myeloid sarcoma, as well as extensive intramedullary disease biopsy proven to be AML. This is the first report of the use of {sup 18}F-FDG PET/CT to stage a subset of aggressive AML and myeloid sarcoma in a patient with an associated chromosomal abnormality (tatrasomy 8)

  11. Myeloid Sarcoma Developing in Prexisting Hydroxyurea-Induced Leg Ulcer in a Polycythemia Vera Patient

    Directory of Open Access Journals (Sweden)

    Hatim Nafil

    2013-01-01

    Full Text Available Myeloid sarcoma (MS is an extramedullary tumour consisting of myeloblasts or immature myeloid cells located in an extramedullary site. It may occur at presentation of AML, at relapse, or prior to the onset of frank leukemia. We report a rare case of MS developing in prexisting Hydroxyurea-induced leg Ulcer in a 70-year-old woman.

  12. Myeloid Sarcoma Developing in Prexisting Hydroxyurea-Induced Leg Ulcer in a Polycythemia Vera Patient

    OpenAIRE

    Hatim Nafil; Illias Tazi; Lahoucine Mahmal

    2013-01-01

    Myeloid sarcoma (MS) is an extramedullary tumour consisting of myeloblasts or immature myeloid cells located in an extramedullary site. It may occur at presentation of AML, at relapse, or prior to the onset of frank leukemia. We report a rare case of MS developing in prexisting Hydroxyurea-induced leg Ulcer in a 70-year-old woman.

  13. Case Report: Myelodysplastic syndrome- associated myeloid sarcoma: an unusual clinical presentation of a rare disease [version 1; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    Emoke Horvath

    2016-02-01

    Full Text Available Myeloid sarcoma results from the extramedullary homing and proliferation of immature myeloid precursors. We present the timeline, events and diagnostic pitfalls related to a 66 year-old male patient’s case, admitted to the Hematology Clinic for pancytopenia, fever, weight loss and fatigue. The severe cytopenia and the few blasts observed in his blood smear indicated a bone marrow biopsy. The bone marrow showed hypercellularity and multilineage dysplasia with the presence of 15% myeloblasts. After the biopsy, he promptly developed paraplegia and nuclear magnetic resonance revealed an epidural tumour which was then resected.In the epidural tumour mass blast-like, round cells were observed with a complex immunophenotype, characterized by myeloperoxidase, CD117, CD15, CD99, leucocyte common antigen positivity and a high Ki-67 proliferation index. Considering the main differential diagnostic issues, the final diagnosis was stated as myelodysplastic syndrome-associated myeloid sarcoma. The prognosis was unfavourable, the bone marrow was quickly invaded by proliferating blast cells, and despite chemotherapy attempts, the patient died.

  14. Gastric myeloid sarcoma - A report of two cases addressing diagnostic issues

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    Viijaya Gadage

    2011-01-01

    Full Text Available Presented herein are two cases of gastric myeloid sarcoma to highlight the diagnostic conundrum and pointers toward accurate diagnosis in such instances. The first case was a 35-year-old man with an ulceronodular mass in the body of stomach. Multiple biopsies were reported as inconclusive chiefly due to the fact that the lamina propria infiltrate was innocuous and failed to mark with CD20 or CD3. Subsequently the patient had extensive disseminated disease which was recognized as myeloid sarcoma but patient succumbed to the disease soon. The second case was a 25-year-old boy who presented with symptoms of gastric outlet obstruction since 6 months. An endoscopy revealed diffuse gastric wall thickening which on biopsy was recognized as myeloid sarcoma but patient developed intestinal obstruction and required ileal resection for symptomatic relief, postoperative patient never recovered and succumbed to the disease. Both patients had marrow involvement by acute myeloid leukemia (AML-M2 with a normal leukocyte count in peripheral blood. Thus gastric myeloid sarcomas are prone to a delayed diagnosis chiefly due to rarity. Pathologist should think of myeloid sarcoma in a hematolymphoid appearing tumor in stomach that is CD20, CD3 negative, has avid Ki67 and shows an infiltrate chiefly centered in lamina propria.

  15. Complex treatment of localized bone marrow sarcoma in children

    International Nuclear Information System (INIS)

    The retrospective analysis included the results of the treatment of 67 children suffering from localized sarcomas of bone (Ewing's sarcoma, lymphosarcoma). The advantage was demonstrated in patients, received combination of chemotherapy and radiotherapy on the involved bone. The resection of the primary tumor in combination with radio-chemotherapy improves the 10-year survival

  16. Isolated extramedullary relapse after allogeneic bone marrow transplantation for chronic myeloid leukemia

    OpenAIRE

    Au, WY; Chan, ACL; Lie, AKW; So, JCC; Liang, R.; Kwong, YL

    1998-01-01

    Relapse of chronic myeloid leukemia (CML) as extramedullary granulocytic sarcoma (GS) after allogeneic bone marrow transplantation (BMT) is a rare occurrence. We report two patients who developed spinal GS as the first indication of relapse after allogeneic BMT for CML. In both cases, the marrow was in morphologic and karyotypic remission. However, fluorescence in situ hybridization (FISH) successfully demonstrated the presence of a minor Ph-positive clone in the marrow, as well as an occult ...

  17. Donor-Derived Myeloid Sarcoma in Two Kidney Transplant Recipients from a Single Donor

    Directory of Open Access Journals (Sweden)

    Amudha Palanisamy

    2015-01-01

    Full Text Available We report the rare occurrence of donor-derived myeloid sarcoma in two kidney transplant patients who received organs from a single deceased donor. There was no evidence of preexisting hematologic malignancy in the donor at the time of organ recovery. Both recipients developed leukemic involvement that appeared to be limited to the transplanted organ. Fluorescence in situ hybridization (FISH and molecular genotyping analyses confirmed that the malignant cells were of donor origin in each patient. Allograft nephrectomy and immediate withdrawal of immunosuppression were performed in both cases; systemic chemotherapy was subsequently administered to one patient. Both recipients were in remission at least one year following the diagnosis of donor-derived myeloid sarcoma. These cases suggest that restoration of the immune system after withdrawal of immunosuppressive therapy and allograft nephrectomy may be sufficient to control HLA-mismatched donor-derived myeloid sarcoma without systemic involvement.

  18. [Refractory primary myeloid sarcoma of the breast with MLL-AF9 rearrangement].

    Science.gov (United States)

    Uchida, Emi; Watanabe, Ken; Oshikawa, Gaku; Sakashita, Chizuko; Kurosu, Tetsuya; Fukuda, Tetsuya; Arai, Ayako; Murakami, Naomi; Miura, Osamu; Yamamoto, Masahide

    2016-01-01

    A 28-year-old woman presented with a right breast mass and axillary lymphadenopathy. Biopsy of the breast mass revealed myeloid sarcoma (MS) staining positive for CD4, CD13, CD33, and CD68/KP-1. Bone marrow aspiration revealed leukemic cell infiltration (9%). Leukemic cells possessed cytogenetic abnormalities of +8 and t(9;11)(p22;q23) with +22 (lymph node only), and molecular analyses confirmed the MLL-AF9 fusion gene. After induction chemotherapy and 2(nd) consolidation therapy, complete remission was maintained. However, during consolidation radiotherapy for the breast mass, the disease progressed in both the breast and bone marrow. She received re-induction therapy and proceeded to allogeneic stem cell transplantation. However, the disease relapsed in the breast soon after transplantation, and she died from disease progression. Trisomy 8 and the MLL-AF9 fusion gene have been reported in cases with MS in the breast. Trisomy 22 found additionally and exclusively in the extramedullary lesion implies extramedullary progression of MS from the medullary site of origin and may have been associated with the distinctive therapy resistance of these lesions in our case. PMID:26861104

  19. UK guidelines for the management of bone sarcomas

    OpenAIRE

    Gerrand, Craig; Athanasou, Nick; Brennan, Bernadette; Grimer, Robert; Judson, Ian; Morland, Bruce; Peake, David; Seddon, Beatrice; Whelan, Jeremy; ,

    2016-01-01

    This document is an update of the British Sarcoma Group guidelines published in 2010. The aim is to provide a reference standard for the clinical care of patients in the UK with bone sarcomas. Recent recommendations by the European Society of Medical Oncology, The National Comprehensive Cancer Network and The National Institute for Health and Care Excellence have been incorporated, and the literature since 2010 reviewed. The standards represent a consensus amongst British Sarcoma Group member...

  20. A case of steroid-dependent myeloid granulocytic sarcoma masquerading as Crohn's disease

    Institute of Scientific and Technical Information of China (English)

    Lola Y Kwan; Stephan R Targan; David Q Shih

    2011-01-01

    Small bowel tumors and Crohn's disease are common causes of small bowel obstruction. Early stage neoplasms can easily be mistaken for Crohn's disease. Therefore, thorough work-ups including imaging studies and endoscopic evaluation with biopsies are critical for accurate diagnosis. Here we report a case of an otherwise healthy female with progressive onset of multiple, recurrent obstructive symptoms secondary to terminal ileal narrowing who was referred for management of steroid-dependent Crohn's disease. After thorough evaluation, the diagnosis was revised to myeloid granulocytic sarcoma involving the terminal ileum. In this case, a delay in diagnosis can be detrimental for prognosis, as myeloid granulocytic sarcoma is highly predictive of underlying acute myeloid leukemia and needs urgent referral for chemotherapy and/or resection.

  1. The risk of bone sarcoma from plutonium-239

    International Nuclear Information System (INIS)

    It is postulated that the risk of bone sarcoma induction by 239Pu in humans can be quantitated from a consideration of the well-documented induction of bone sarcomas by sup(226,228)Ra. The basic assumption is that the risk of this malignancy is a function of the energy deposited by alpha particles in the layer of cells adjacent to bone surfaces. From calculations of the energy delivered to this cellular layer by bone-deposited 239Pu and 226Ra the ratio of the systemic intakes required to produce equal bone-cell doses is obtained. A dose-response relationship of the form I = βD2esup(-γD) was found to provide the best fit to the observed bone-sarcoma incidence for female radium dial workers, where I was the number of bone sarcomas per person x year of risk and D was the quantity of radium that entered the systemic circulation. It is proposed that such an expression, modified for the energy effectiveness of plutonium compared with radium, provides the best estimate of the risk of induction of bone sarcomas by 239Pu. The derived risk estimate is tested by comparing its predictions of bone sarcomas in two groups of people carrying known body burdens of plutonium. The predictions are not contradicted by the lack of any observed bone sarcomas in either the 27 workers exposed at Los Alamos during World War II, or in the 18 seriously ill cases injected with plutonium in 1945-47. Its form permits an immediate evaluation of the risk at any level of intake. (author)

  2. [Langerhans cell sarcoma developing acute myeloid leukemia after achieving complete response by THP-COP].

    Science.gov (United States)

    Hamaguchi, Kota; Hashimoto, Akari; Fujimi, Akihito; Kanisawa, Yuji; Shibata, Takanori; Nakajima, Chisa; Hayasaka, Naotaka; Yamada, Shota; Okuda, Toshinori; Minami, Shinya; Kamihara, Yusuke; Ohshima, Koichi; Kato, Junji

    2015-12-01

    An 86-year-old man presented with enlarged left submandibular, left inguinal, and superficial femoral lymph nodes. He was diagnosed with Langerhans cell sarcoma (LCS) on the basis of the histopathological findings of the left inguinal lymph node biopsy. In addition, laboratory examinations revealed normocytic normochromic anemia, and bone marrow aspiration and biopsy led to a diagnosis of idiopathic cytopenia of undetermined significance (ICUS). Because of the patient's age, he was administered a regimen of cyclophosphamide, pirarubicin, vincristine, and prednisolone (THP-COP), and achieved a partial response after six courses. However, he developed acute myeloid leukemia (AML) 11 months after completion of the THP-COP therapy, and received only supportive care until his death. LCS is an extremely rare and aggressive dendritic cell neoplasm. To the best of our knowledge, only 67 cases have been reported in the literature. There are case reports describing the concurrence of hematological malignancies. Herein, we report the first documented development of LCS in a patient with ICUS who progressed to AML, and summarize the published data on the epidemiology of and therapeutic options for LCS. PMID:26725355

  3. Primary reticulum cell sarcoma of bone

    International Nuclear Information System (INIS)

    Seventeen cases of primary reticulum cell sarcoma of bone are presented. The disease may be seen at any age, usually occurs in the long or flat bones and, in spite of involving a given bone very extensively, usually leaves the patient in good general condition. Roentgenographically, the lesion is primarily destructive, often massive. In the early stages, diffuse medullary mottling may be the only sign. Later, the expansive tumor may give rise to fragmentation of the cortex and pathological fracture. Histologically, the tumor cells have round, oval, indented, or lobulated nuclei which are nearly twice the size of that of a lymphocyte. The chromatin is scattered and the cytoplasm is considerable in amount. In spite of its apparently malignant nature, the tumor is amenable to appropriate treatment, 13 of the 17 cases being alive from 6 months to 14 years from the initial symptom. Seven of these patients have been apparently free from disease 10 or more years. Five patients were treated by amputation alone. Of these, 2 are dead; 3 are alive from 3 to 11 years from onset. The authors have already referred to a possible explanation for the deaths in this group. Three patients were treated by radiation alone. Of these 1 is dead; 2 are alive from 1 to 3 years later, but in each there is still neoplastic disease. Of the 9 patients treated by amputation and radiation, 8 are alive from 6 months to 14 years from onset. From a consideration of the cases presented, the best procedure would appear to be early diagnosis by biopsy followed by immediate amputation and radiation

  4. Isolated myeloid sarcoma of the neck and chest: differential diagnosis and therapeutic approach

    Directory of Open Access Journals (Sweden)

    Djurdjevic Predrag

    2015-01-01

    Full Text Available In this article, we present the case of a 57-yearold man with cervical and mediastinal tumor mass, normal blood count as well as virusological status. Cervical tumor tissue biopsy revealed cells positive for CD34, CD13, LCA, CD33, and CD163 but negative for T-cell and B-cell markers, NK-cell markers, plasmacytic markers and anaplastic large cell lymphoma markers. These features were consistent with myeloid sarcoma of the neck with involvement of the mediastinum. We discussed differential diagnosis and therapy of isolated myeloid sarcoma and suggest that clinical presentation, cell morphology, complete immunophenotype, and specific genotypic lesions in some cases, must be evaluated.

  5. Donor-Derived Myeloid Sarcoma in Two Kidney Transplant Recipients from a Single Donor

    OpenAIRE

    Amudha Palanisamy; Paul Persad; Koty, Patrick P.; Douglas, Laurie L.; Stratta, Robert J.; Jeffrey Rogers; Reeves-Daniel, Amber M.; Giuseppe Orlando; Farney, Alan C; Beaty, Michael W.; Pettenati, Mark J.; Iskandar, Samy S.; Grier, David D; Scott A. Kaczmorski; Doares, William H.

    2015-01-01

    We report the rare occurrence of donor-derived myeloid sarcoma in two kidney transplant patients who received organs from a single deceased donor. There was no evidence of preexisting hematologic malignancy in the donor at the time of organ recovery. Both recipients developed leukemic involvement that appeared to be limited to the transplanted organ. Fluorescence in situ hybridization (FISH) and molecular genotyping analyses confirmed that the malignant cells were of donor origin in each pati...

  6. Myeloid sarcoma derived from the gastrointestinal tract: A case report and review of the literature

    Science.gov (United States)

    YU, TENG; XU, GENBO; XU, XIAOHUA; YANG, JING; DING, LUYIN

    2016-01-01

    Myeloid sarcoma is a type of malignant neoplasm composed of myeloblasts that locates extramedullary. The present study reports the case of a 31-year-old female who presented with upper abdominal pain, melena, vomiting and jaundice. The abdominal computed tomography revealed a mass in gastric antrum area and possible infiltration of the duodenum, gallbladder and head of the pancreas, with possible retroperitoneal lymph node metastasis. The tumor grew quickly and led to serious obstructive jaundice. New masses developed in the bilateral orbits and left breast within 2 months of admission. The pathological results of the gastroscopic biopsy and the fine-needle biopsy of the breast revealed myeloid sarcoma. Transhepatic cholangial drainage, radiotherapy and chemotherapy were administered, but the disease reoccurred and became resistant to chemotherapy, so salvage allogenetic peripheral blood stem cell transplantation was performed. The disease relapsed at 5 months post-transplantation, and chemotherapy and donor lymphocytes transfusions were then administered. The patient declined further treatment and succumbed to disease on May 19, 2015. The present study could improve the understanding of myeloid sarcoma and provide a reference for standardized and individualized treatments for this disease.

  7. BONE TUMOR ENVIRONMENT AS POTENTIAL THERAPEUTIC TARGET IN EWING SARCOMA

    Directory of Open Access Journals (Sweden)

    Françoise eREDINI

    2015-12-01

    Full Text Available Ewing sarcoma is the second most common pediatric bone tumor, with three cases per million worldwide. In clinical terms, ES is an aggressive, rapidly fatal malignancy that mainly develops in osseous sites (85%, but also in extraskeletal soft tissue. It spreads naturally to the lungs, bones and bone marrow with poor prognosis in the two latter cases. Bone lesions from primary or secondary (metastases tumors are characterized by extensive bone remodeling, more often due to osteolysis. Osteoclast activation and subsequent bone resorption is responsible for the clinical features of bone tumors including pain, vertebral collapse and spinal cord compression. Based on the vicious cycle concept of tumor cells and bone resorbing cells, drugs which target osteoclasts may be promising agents as adjuvant setting for treating bone tumors, including Ewing sarcoma. There is also increasing evidence that cellular and molecular protagonists present in the bone microenvironment play a part in establishing a favorable niche for tumor initiation and progression. The purpose of this review is to discuss the potential therapeutic value of drugs targeting the bone tumor microenvironment in Ewing Sarcoma. The first part of the review will focus on targeting the bone resorbing function of osteoclasts by means of bisphosphonates (BPs or drugs blocking the pro-resorbing cytokine Receptor Activator of NF-kappa B Ligand (RANKL. Second, the role of this peculiar hypoxic microenvironment will be discussed in the context of resistance to chemotherapy, escape from the immune system, or neo-angiogenesis. Therapeutic interventions based on these specificities could be then proposed in the context of Ewing sarcoma.

  8. Dose-response relationships for radium-induced bone sarcomas

    International Nuclear Information System (INIS)

    The incidence of bone sarcomas among 3055 female radium-dial workers who entered the dial industry before 1950 was used to determine dose-response relationships for the induction of bone sarcomas by radium. Two subpopulations were analyzed: all measured cases who survived at last five years after the start of employment and all cases who survived at least two years after first measurement. The first constituted a group based on year of entry; it contained 1468 women who experienced 42 bone sarcomas; the expected number was 0.4. The second comprised a group based on first measurement; it contained 1257 women who experienced 13 bone sarcomas; the expected number was 0.2. The dose-response function, I = (C + αD + #betta#D2)e/sup -#betta#D/, and simplifications of this general form, were fit to each data set. Two functions, I = (C + αD + #betta#D2)e/sup -#betta#D/ and I = (C + #betta#D2)e/sup -#betta#D/, fit the data for year of entry (p greater than or equal to 0.05); both these functions and I = (C + αD) fit the data for first measurement. The function I = (C + #betta#D2)e/sup -#betta#D/ was used to predict the number of bone sarcomas in all other pre-1950 radium cases (medical, laboratory, and other exposure); fewer were actually observed than the fit of this function to the female dial workers predicted

  9. A case of synovial sarcoma with bone metastasis identified by bone marrow scintigraphy

    Energy Technology Data Exchange (ETDEWEB)

    Otsuka, N.; Morita, R.; Yamamoto, T.; Muranaka, A.; Tomomitsu, T.; Yanagimoto, S.; Sone, T.; Fukunaga, M.

    1985-04-01

    In a patient with synovial sarcoma, routine bone survey showed no abnormality, while bone marrow scintigraphy with Tc-99m sulfur colloid revealed a defect in the fifth lumbar vertebra. At surgery, tumorous invasion was noted in the fifth lumbar vertebra and the surrounding tissues. It was suggested that the bone marrow scintigraphy was particularly useful in the detection of tumorous invasion into the bone marrow at the early stage before the destruction of skeletal tissue.

  10. A case of synovial sarcoma with bone metastasis identified by bone marrow scintigraphy

    International Nuclear Information System (INIS)

    In a patient with synovial sarcoma, routine bone survey showed no abnormality, while bone marrow scintigraphy with Tc-99m sulfur colloid revealed a defect in the fifth lumbar vertebra. At surgery, tumorous invasion was noted in the fifth lumbar vertebra and the surrounding tissues. It was suggested that the bone marrow scintigraphy was particularly useful in the detection of tumorous invasion into the bone marrow at the early stage before the destruction of skeletal tissue

  11. Bone sarcoma in humans induced by radium: A threshold response?

    International Nuclear Information System (INIS)

    The radium 226 and radium 228 have induced malignancies in the skeleton (primarily bone sarcomas) of humans. They have also induced carcinomas in the paranasal sinuses and mastoid air cells. There is no evidence that any leukemias or any other solid cancers have been induced by internally deposited radium. This paper discuses a study conducted on the dial painter population. This study made a concerted effort to verify, for each of the measured radium cases, the published values of the skeletal dose and the initial intake of radium. These were derived from body content measurements made some 40 years after the radium intake. Corrections to the assumed radium retention function resulted in a considerable number of dose changes. These changes have changed the shape of the dose response function. It now appears that the induction of bone sarcomas is a threshold process

  12. Immunohistochemistry and molecular analyses in myeloid sarcoma of the breast in a patient with relapse of NPM1-mutated and FLT3-mutated AML after allogeneic stem cell transplantation.

    Science.gov (United States)

    Choschzick, Matthias; Bacher, Ulrike; Ayuk, Francis; Lebeau, Annette

    2010-06-01

    Myeloid sarcoma of the breast is a rare manifestation of acute myeloid leukaemia (AML). This report describes a patient who was diagnosed with AML FAB M2. Molecular analysis showed evidence of an NPM1 mutation (subtype A) and internal tandem duplications of the FLT3 gene (FLT3-ITD). Eight months after allogeneic stem cell transplantation, the patient developed a palpable mass in the left breast initially suspected as breast carcinoma. Core needle biopsy of the lesion resulted in diagnosis of myeloid sarcoma. Molecular analysis of formalin-fixed specimens of the breast tumour confirmed the known FLT3 and NPM1 gene mutations. Immunohistochemically, an aberrant cytoplasmic staining pattern for NPM1 and overexpression of FLT3 were demonstrated. The myeloid sarcoma showed complete transient resolution following treatment with the kinase inhibitor sorafenib. However, the patient developed bone marrow relapse and died in fatal cerebral haemorrhage 1 year after initial diagnosis of AML. In summary, combined molecular and immunohistochemical examination of NPM1 and FLT3 is helpful in the diagnosis of extramedullary manifestations of AML in core needle biopsies. PMID:20360144

  13. Radiographic features of Ewing's sarcoma of the bones of the hands and feet

    International Nuclear Information System (INIS)

    The radiographic features of Ewing's sarcoma of the bones of the hands and feet are reviewed utilizing cases obtained from the Mayo Clinic patient files and the consultation files of Drs. D.C. Dahlin and K.K. Unni. This series consists of a total of 43 cases of pathologically proven Ewing's sarcoma involving the small bones of the hands and feet. The classic radiographic features of Ewing's sarcoma in the long bones, including lytic, permeative destruction, aggressive periosteal reaction, cortical violation, and a soft tissue mass, are also seen in the bones of the hands and feet, with similar frequency. These classic features are most commonly present in lesions affecting the short tubular bones. Lesions affecting the tarsal bones more often demonstrate atypical radiographic features. These atypical radiographic appearances may play a role in the reported delay in diagnosis of Ewing's sarcoma within the tarsal bones. (orig.)

  14. Bone sarcoma in humans induced by radium: a threshold response?

    International Nuclear Information System (INIS)

    The radium isotopes, 226Ra 228Ra, have induced in humans, at sufficiently high levels in the body, malignancies in the skeleton, primarily bone sarcomas. They have also induced, at approximately half the frequency, carcinomas arising in the paranasal sinuses and mastoid air cells. There is no evidence that any leukemias have been induced by internally deposited radium, nor any other solid cancers. However, some radium cohorts have shown elevated levels of breast cancer, while others have not. It has been suggested, at least for the dial painter population, that breast cancer may be the consequence of external radiation from the radium dial paint. Prior to the termination of the U.S. radium studies program in 1990, a concerted effort was made to verify, for each of the measured radium cases, the published values of the skeletal dose and the initial intake of radium. These were derived from body content measurements made, on the average, some 40 years after radium intake. Corrections to the assumed radium retention function resulted in a considerable number of dose changes. These changes, in turn, have changed the shape of the dose response function. It now appears that the induction of bone sarcomas is a threshold process; below the calculated threshold no malignancies have been, above the level the probability of the induction of a malignancy increases rapidly. (author)

  15. Myeloid Sarcoma of the Uterine Cervix as Presentation of Acute Myeloid Leukaemia after Treatment with Low-Dose Radioiodine for Thyroid Cancer: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Anne Sophie Weingertner

    2009-01-01

    Full Text Available The development of acute myeloid leukaemia after low-dose radioiodine therapy and its presentation as a myeloid sarcoma of the uterine cervix are both rare events. We report a case of acute myeloid leukaemia revealed by a myeloid sarcoma of the uterine cervix in a 48-year-old woman, 17 months after receiving a total dose of 100 mCi 131I for papillary thyroid cancer. A strict hematological follow-up of patients treated with any dose of 131I is recommended to accurately detect any hematological complications which might have been underestimated. Unusual presentations, such as chloroma of the uterine cervix, may reveal myeloid malignancy and should be kept in mind.

  16. Myeloid-derived suppressor cells function as novel osteoclast progenitors enhancing bone loss in breast cancer

    OpenAIRE

    Sawant, Anandi; Deshane, Jessy; Jules, Joel; Lee, Carnella M.; Harris, Brittney A.; Feng, Xu; Ponnazhagan, Selvarangan

    2012-01-01

    Enhanced bone destruction is a hallmark of various carcinomas such as breast cancer, where osteolytic bone metastasis is associated with increased morbidity and mortality. Immune cells contribute to osteolysis in cancer growth but the factors contributing to aggressive bone destruction are not well understood. In this study, we demonstrate the importance of myeloid-derived suppressor cells (MDSC) in this process at bone metastatic sites. Since MDSC originate from the same myeloid lineage as m...

  17. Quality of Life Following Amputation or Limb Preservation in Patients with Lower Extremity Bone Sarcoma

    OpenAIRE

    Gary E Mason; Lele eAung; Sarah eGall; Meyers, Paul A; Robert eButler; Sarah eKrug; Mimi eKim; Healey, John H.; Richard eGorlick

    2013-01-01

    PURPOSE: Although functional differences have been described between patients with lower extremity bone sarcoma with amputation and limb preservation surgery, differences have not clearly been shown between the two groups related to quality of life. The aim of the study was to determine if there is a difference in overall quality of life in lower extremity bone sarcoma survivors related to whether they had an amputation or a limb preservation procedure. PATIENTS AND METHODS: Eighty-two lon...

  18. Oxidative stress and antioxidant status in primary bone and soft tissue sarcoma

    International Nuclear Information System (INIS)

    Oxidative stress is characterised by an increased level of reactive oxygen species (ROS) that disrupts the intracellular reduction-oxidation (redox) balance and has been implicated in various diseases including cancer. Malignant tumors of connective tissue or sarcomas account for approximately 1% of all cancer diagnoses in adults and around 15% of paediatric malignancies per annum. There exists no information on the alterations of oxidant/antioxidant status of sarcoma patients in literature. This study was aimed to determine the levels of oxidative stress and antioxidant defence in patients with primary bone and soft tissue sarcoma and to investigate if there exists any significant differences in these levels between both the sarcomas. The study cohort consisted of 94 subjects; 20 soft tissue sarcoma, 27 primary bone sarcoma and 47 healthy controls. Malondialdehyde (MDA) and protein carbonyls were determined to assess their oxidative stress levels while antioxidant status was evaluated using catalase (CAT), superoxide dismutase (SOD), thiols and trolox equivalent antioxidant capacity (TEAC). Sarcoma patients showed significant increase in plasma and urinary MDA and serum protein carbonyl levels (p < 0.05) while significant decreases were noted in TEAC, thiols, CAT and SOD levels (p < 0.05). No significant difference in oxidative damage was noted between both the sarcomas (p > 0.05). In conclusion, an increase in oxidative stress and decrease in antioxidant status is observed in both primary bone and soft tissue sarcomas with a similar extent of damage. This study offers the basis for further work on whether the manipulation of redox balance in patients with sarcoma represents a useful approach in the design of future therapies for bone disease

  19. Myeloid sarcoma occurring in the gingiva:a case report%牙龈髓细胞肉瘤1例

    Institute of Scientific and Technical Information of China (English)

    乌玉红; 吕威; 王瑞梅

    2015-01-01

    Myeloid sarcoma (MS) is a localized extramedullary mass of immature granulocytic cells. MS may be found in any location, but intraoral occurrence is rare. This report presents a case of MS in the gingiva.%髓细胞肉瘤是一种由原粒细胞构成的髓外瘤性包块,可发生于任何部位,但发生于口腔者较罕见。本文报道1例发生于牙龈的髓细胞肉瘤。

  20. Long-term adverse outcomes in survivors of childhood bone sarcoma: the British Childhood Cancer Survivor Study

    OpenAIRE

    Fidler, M M; Frobisher, C; Guha, J; K. Wong; Kelly, J; Winter, D. L.; Sugden, E; Duncan, R.; Whelan, J; Reulen, R C; Hawkins, M. M.

    2015-01-01

    Background: With improved survival, more bone sarcoma survivors are approaching middle age making it crucial to investigate the late effects of their cancer and its treatment. We investigated the long-term risks of adverse outcomes among 5-year bone sarcoma survivors within the British Childhood Cancer Survivor Study. Methods: Cause-specific mortality and risk of subsequent primary neoplasms (SPNs) were investigated for 664 bone sarcoma survivors. Use of health services, health and marital st...

  1. Granulocytic Sarcoma of the Stomach Presenting as Dysphagia during Pregnancy

    Directory of Open Access Journals (Sweden)

    Anuradha Sekaran

    2011-01-01

    Full Text Available Granulocytic sarcoma also known as extramedullary myeloid sarcoma or chloroma is an uncommon manifestation of leukemia and presents as a deposit of leukemic cells outside the bone marrow. We report a case of a twenty-five-year-old pregnant woman who presented with progressive dysphagia and recurrent postprandial vomiting. Upper GI endoscopy had shown large flat laterally spread nodular lesions in the cardia and proximal body of stomach. Biopsies from the gastric lesion showed granulocytic sarcoma of the stomach. Concurrent peripheral and bone marrow picture was suggestive of acute myeloid leukemia (AML–M4. There is limited reported literature on granulocytic sarcoma of the stomach. Concurrent gastric granulocytic sarcoma involving cardia and AML in pregnancy has not been reported till date.

  2. Synovial sarcoma of primary bone origin: a rare case in a rare site with atypical features

    Energy Technology Data Exchange (ETDEWEB)

    Jung, Seung Chai [Seoul National University College of Medicine, Department of Radiology and Institute of Radiation Medicine, Seoul (Korea); Choi, Jung-Ah; Lee, Joon Woo; Kang, Heung Sik [Seoul National University College of Medicine, Department of Radiology and Institute of Radiation Medicine, Seoul (Korea); Seoul National University Bundang Hospital, Department of Radiology, Gyeongi-Do (Korea); Chung, Jin-Haeng [Seoul National University Bundang Hospital, Department of Pathology, Gyeongi-Do (Korea); Oh, Joo Han [Seoul National University Bundang Hospital, Department of Orthopedic Surgery, Gyeongi-Do (Korea)

    2007-01-15

    Synovial sarcoma of bone origin is extremely rare and difficult to diagnose. We present a case in which the lesion arose in the cortex of the distal tibia. It showed heterogeneous intermediate signal intensity on T1-weighted images and heterogeneous intermediate to low signal intensity on T2-weighted images with heterogeneous contrast enhancement on MRI. The lesion was confirmed as synovial sarcoma using a combination of histological and molecular genetic studies. (orig.)

  3. Synovial sarcoma of primary bone origin: a rare case in a rare site with atypical features

    International Nuclear Information System (INIS)

    Synovial sarcoma of bone origin is extremely rare and difficult to diagnose. We present a case in which the lesion arose in the cortex of the distal tibia. It showed heterogeneous intermediate signal intensity on T1-weighted images and heterogeneous intermediate to low signal intensity on T2-weighted images with heterogeneous contrast enhancement on MRI. The lesion was confirmed as synovial sarcoma using a combination of histological and molecular genetic studies. (orig.)

  4. MR criteria for differentiation of 'pseudo tumourous' lesions from bone sarcomas of the extremities

    International Nuclear Information System (INIS)

    The MRT scans of 57 patients with Ewing or osteosarcomas and 34 patients with haematogenous osteomyelitis or periostitis/stress fractures were examined in order to determine whether a distinction between benign or malignant lesions is possible. Four criteria were evaluated: The margin of the bone marrow component; intensity and homogeneity of the T1-weighted signal in the bone marrow; presence of an extraosseus structured soft tissue mass and/or soft tissue edema. It was found that central osteosarcomas and Ewing's sarcomas reduced signal intensity of the marrow to become muscle-isointense with a well defined margin. In acute haematogenous osteomyelitis and periostitis/stress fracture the marrow lesion was not sharply demarcated. In contrast to patients with bone sarcomas only one case of osteomyelitis showed an extrosseus structured soft tissue mass. On the basis of these findings we believe that acute haematogenous osteomyelitis can be distinguished with high degree of accuracy from Ewing's sarcoma and central osteosarcomas. (orig.)

  5. Quality of Life Following Amputation or Limb Preservation in Patients with Lower Extremity Bone Sarcoma

    Directory of Open Access Journals (Sweden)

    Gary E Mason

    2013-08-01

    Full Text Available PURPOSE: Although functional differences have been described between patients with lower extremity bone sarcoma with amputation and limb preservation surgery, differences have not clearly been shown between the two groups related to quality of life. The aim of the study was to determine if there is a difference in overall quality of life in lower extremity bone sarcoma survivors related to whether they had an amputation or a limb preservation procedure. PATIENTS AND METHODS: Eighty-two long-term survivors of lower extremity bone sarcoma were studied to make a comparison of the overall quality of life, pain assessment and psychological evaluations in limb preservation and amputation patients. Forty-eight patients with limb preservation and thirty-four patients with amputations were enrolled in the study. Validated psychometric measures including the Quality of Life Questionnaire, the Minnesota Multiphasic Personality Inventory and visual analog scales were utilized.RESULTS: The overall quality of life of patients with limb preservation was significantly higher than patients with amputation (p-value < 0.01. Significant differences were noted in the categories of material well being, job satisfiers and occupational relations. CONCLUSION: The overall quality of life of patients with limb preservation appears to be better than for those patients with amputation based on the quality of life questionnaire in patients surviving lower extremity bone sarcoma. Further analysis needs to verify the results and focus on the categories that significantly affect the overall quality of life.

  6. Myeloid-derived suppressor cells as a novel target for the control of osteolytic bone disease

    OpenAIRE

    Sawant, Anandi; Ponnazhagan, Selvarangan

    2013-01-01

    Myeloid-derived suppressor cells (MDSC) from mice bearing bone metastases differentiate into functional osteoclasts in vitro and in vivo, through a signaling pathway that relies on nitric oxide. In addition, MDSC-targeting drugs have been shown to robustly inhibit osteolysis. Thus, MDSC stand out as novel osteoclast progenitors and hence as candidate targets for the control of osteolytic bone disease.

  7. 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.

  8. A novel treatment for bone lesions of multifocal epithelioid sarcoma-like hemangioendothelioma.

    Science.gov (United States)

    Davis, Adrian T; Guo, A Mary; Phillips, Nancy J; Greenberg, David D

    2015-07-01

    Epithelioid sarcoma-like hemangioendothelioma (ES-H) is a rare, indolent vascular neoplasm with characteristics similar to epithelioid sarcoma. It typically affects young males who present with skin and subcutaneous lesions in the extremities. Bone lesions, occurring in approximately 20% of patients, are often multifocal, seen in conjunction with soft tissue lesions, and usually found in bones of the lower extremities. This report details the case of a 20-year-old male who presented with a 1-year history of painful skin lesions on his left lower extremity. Staging studies revealed bone lesions in the cuboid and calcaneus. Bone and soft tissue pathology was consistent with ES-H. The soft tissue lesions were treated with wide excision and the bony lesions with radiofrequency ablation (RFA). The patient had no evidence of recurrence at the 2-year follow-up. Treatment of ES-H typically consists of wide excision of all soft tissue lesions and possible adjuvant radiation therapy. Management of bony lesions has included marginal excision, wide excision, amputation, chemotherapy, observation, or a combination of these modalities. ES-H has the potential for distant metastases. There is no consensus regarding the appropriate treatment of multifocal epithelioid sarcoma-like hemangioendothelioma involving bone. A potential treatment strategy of wide excision of soft tissue lesions and RFA of bone lesions is proposed. PMID:25564226

  9. Four year experience of sarcoma of soft tissues and bones in a tertiary care hospital and review of literature

    Directory of Open Access Journals (Sweden)

    Ansari Tayyaba Z

    2011-05-01

    Full Text Available Abstract Background Sarcoma encompasses an uncommon group of cancer and the data is insufficient from Pakistan. We report our four years experience of Sarcoma of soft tissues and bones. Methods This cross sectional study was carried out at Aga Khan University Hospital from 2004 to 2008. The patients were divided into two groups from the outset i.e. initially diagnosed and relapsed group and separate sub group analysis was conducted. Results Out of 93 newly diagnosed patients, 58 belonged to bone sarcoma and 35 to soft tissue sarcoma group. While for relapsed patients, 5 had soft tissue sarcoma and 9 had bone sarcoma. Mean age was 32.5 years. At presentation, approximately two third patients had localised disease while remaining one third had metastatic disease. The Kaplan Meier estimate of median recurrence free survival was 25 months, 35 months, and 44 months for Osteogenic sarcoma, Ewing's sarcoma and Chondrosarcoma respectively. For Leiomyosarcoma and Synovial sarcoma, it was 20 and 19 months respectively. The grade of the tumour (p = 0.02 and surgical margin status (p = 0.001 were statistically significant for determination of relapse of disease. Conclusion The median recurrence free survival of patients in our study was comparable to the reported literature but with significant lost to follow rate. Further large-scale, multi centre studies are needed to have a more comprehensive understanding of this heterogeneous disease in our population.

  10. Splenic irradiation before bone marrow transplantation for chronic myeloid leukaemia

    International Nuclear Information System (INIS)

    A total of 229 patients with chronic myeloid leukaemia (CML) in chronic phase were randomized between 1986 and 1990 to receive or not receive additional splenic irradiation as part of their conditioning prior to bone marrow transplantation (BMT). Both groups, 115 patients with and 114 patients without splenic irradiation, were very similar regarding distribution of age, sex, donor/recipient sex combination, conditioning, graft-versus-host disease (GvHD) prevention method and blood counts at diagnosis or prior to transplant. 135 patients (59%) are alive as of October 1995 with a minimum follow-up of 5 years. 52 patients have relapsed (23%), 26 patients in the irradiated, 26 patients in the non-irradiated group (n.s.) with a relapse incident at 6 years of 28%. The main risk factor for relapse was T-cell depletion as the method for GvHD prevention, and an elevated basophil count in the peripheral blood prior to transplant. Relapse incidence between patients with or without splenic irradiation was no different in patients at high risk for relapse, e.g. patients transplanted with T-cell-depleted marrows (P = n.s.) and in patients with low risk for relapse, e.g. patients transplanted with non-T-cell-depleted transplants and basophil counts 3% basophils in peripheral blood). In this patient group, relapse incidence was 11% at 6 years with splenic irradiation but 32% in the non-irradiated group (P = 0.05). Transplant-related mortality was similar whether patients received splenic irradiation or not. This study suggests an advantage in splenic irradiation prior to transplantation for CML in this subgroup of patients and illustrates the need for tailored therapy. (Author)

  11. Summary of dosimetry, pathology, and dose response for bone sarcomas in beagles injected with radium-226

    International Nuclear Information System (INIS)

    In the completed 226Ra portion of a 30-year-long experiment to determine the relative radiotoxicity of injected 226Ra and 239Pu, 42 of 116 animals injected with 226Ra developed 63 bone sarcomas; none were observed in 44 controls. Average alpha plus beta dose to the skeleton to death was calculated on the basis of mathematical functions developed from sequential measurements of radium and radon retention in each dog. Bone sarcomas were identified radiographically or clinically, with subsequent histopathological confirmation and classification. Most primary bone tumors were classified as osteosarcomas if osteoid arose from a malignant stroma. The dose-response curve over the six lowest injected dose levels fits well to a linear, no-threshold, least squares fit, through a control incidence of 0.8%, and with a slope of 0.042% incidence per rad. 19 refs., 5 figs., 2 tabs

  12. Effects of Time of Initial Exposure to MSV Sarcoma on Bone Induction by Dentine Matrix Implants and on Orthotopic Femora

    Directory of Open Access Journals (Sweden)

    Aniela Brodzikowska

    2010-09-01

    Full Text Available HCl-demineralized murine lower incisors were implanted intramuscularly into syngeneic BALB/c mice to induce heterotopic osteogenesis. Implants were exposed at the early, preosteogenic stage (4, or at the later, osteogenic stage (12 to the Moloney sarcoma virus (MSV, which within 3–4 days results in a sarcoma. The yield of bone induction was determined by weight of dry bone mass following NaOH hydrolysis of soft tissues. To verify the effect of this sarcoma on orthotopic local femoral bone, the dry mass of the tumor-exposed femora was measured and compared with the weight of MSV-unexposed contralateral controls. MSV-sarcoma or cells involved with their spontaneous rejection have a stimulatory effect on the periosteal membrane of the tumor-adjacent femoral bones, increasing their dry mass on average by 18%. No stimulatory effect on heterotopic bone induction was observed when the MSV sarcoma grew during the early, preosteogenic stage (4 onward, but when the tooth matrix had been exposed to such tumor at the already bone-forming stage, (12 onward, the yield of bone induction was enhanced. Thus, it is postulated that lesions induced by MSV during the early, preosteogenic stage inhibit recruitment of osteoprogenitor cells or degrade Bone Morphogenetic Proteins (BMPs released by matrix resorbing inflammatory cells, whereas when acting on already existing bone they have a stimulatory effect.

  13. 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

  14. Bone Density Changes After Radiation for Extremity Sarcomas: Exploring the Etiology of Pathologic Fractures

    International Nuclear Information System (INIS)

    Purpose: The incidental irradiation (RT) of adjacent bone that takes place during treatment of soft tissue extremity sarcomas is generally presumed to 'weaken' the bone by decreasing its density, which subsequently increases the risk for pathologic fracture. This investigation intended to assess the relative effects on bone density of both RT and diminished mechanical loading secondary to tumor-induced and therapy-induced functional extremity impairment. Methods and Materials: 19 patients treated with surgical excision and RT for soft tissue extremity sarcomas had bone density measured using dual energy X-ray absorptiometry at four sites: the irradiated (A) and contralateral (B) bone, and an uninvolved bone (C) in the treated extremity and its contralateral counterpart (D). Analysis included (1) [A-B], (2) [C-D], (3) [(A-B), - (C-D)], and (4) [(A-B)/B - (C-D)/D]. Results: The mean bone density for all irradiated sites was increased 0.08 ± 0.22 g/cm2 (variance) compared to the contralateral unirradiated side when corrected for weight-bearing effects (3). An average increase in bone density of 9 ± 22% (p = 0.08) was also seen when the differences were divided by individual control densities to normalize variation in density of different anatomic sites (4). Conclusions: RT does not routinely decrease bone density when corrected for weight bearing or mechanical effects. The pathogenesis for the known increased risk of pathologic fracture in irradiated bones is likely multifactorial, including possible alterations in bone remodeling that can result in stable, or even increased, bone density. Further clinical and basic studies are needed to confirm our unexpected findings.

  15. Theory of the induction of bone sarcoma by bone-seeking alpha emitters and its application to risk assessment

    International Nuclear Information System (INIS)

    This work discusses the theory of bone sarcoma induction by bone seeking alpha emitters, which is based strictly on biological considerations relative to the mechanism of radiation-induced carcinogenesis, identification of cells at risk and their location in bone, bone tissue renewal processes and bone cell kinetics with or without radiation exposure. The model is consistent with the data on bone sarcoma incidence human with incorporated long-lived isotopes Ra-226 + Ra-228. Extrapolation of these data to a low intake region of the basis of the developed theoretical approach suggests that the linear ICRP-UNSCEAR model overestimates carciogenic risk at low doses, possibly by a factor of 2-4. The model suggests a linear response of target cells to the initiation effects of alpha irradiation. The non-linear (linear-quadratic) initial part of dose-response curve for osteosarcoma induction is explained quantitatively by a model based on a promoter effect of regenerative hyperplasia resulting from invitation effects of alpha radiation. The maximum overestimation inherent to the model of the low-level risk due to the the dose-dependent promotion factor is estimated using bo+1/bo, where bo is a model parameter which is proportional to the normal division rate of osteogenic cells in vivo and which can be estimated within the framework of the model. The model provides confirming evidence that, for radiation protection purposes, endosteal cells may be considered the only group of cells at risk of sarcoma induction by low doses of bone-seeking alpha emitters, whereas the role of marrow stromal (osteogenic) cells as target cells is much more significant with increasing intakes, and can become dominating if intake is high enough

  16. Theory of the induction of bone sarcoma by bone-seeking alpha emitters and its application to risk assessment

    Energy Technology Data Exchange (ETDEWEB)

    Petojan, I.M.

    1992-06-01

    This work discusses the theory of bone sarcoma induction by bone seeking alpha emitters, which is based strictly on biological considerations relative to the mechanism of radiation-induced carcinogenesis, identification of cells at risk and their location in bone, bone tissue renewal processes and bone cell kinetics with or without radiation exposure. The model is consistent with the data on bone sarcoma incidence human with incorporated long-lived isotopes Ra-226 + Ra-228. Extrapolation of these data to a low intake region of the basis of the developed theoretical approach suggests that the linear ICRP-UNSCEAR model overestimates carciogenic risk at low doses, possibly by a factor of 2-4. The model suggests a linear response of target cells to the initiation effects of alpha irradiation. The non-linear (linear-quadratic) initial part of dose-response curve for osteosarcoma induction is explained quantitatively by a model based on a promoter effect of regenerative hyperplasia resulting from invitation effects of alpha radiation. The maximum overestimation inherent to the model of the low-level risk due to the the dose-dependent promotion factor is estimated using bo{sup +1}/bo, where bo is a model parameter which is proportional to the normal division rate of osteogenic cells in vivo and which can be estimated within the framework of the model. The model provides confirming evidence that, for radiation protection purposes, endosteal cells may be considered the only group of cells at risk of sarcoma induction by low doses of bone-seeking alpha emitters, whereas the role of marrow stromal (osteogenic) cells as target cells is much more significant with increasing intakes, and can become dominating if intake is high enough.

  17. Myeloid-specific TGF-β signaling in bone promotes basic-FGF and breast cancer bone metastasis.

    Science.gov (United States)

    Meng, X; Vander Ark, A; Lee, P; Hostetter, G; Bhowmick, N A; Matrisian, L M; Williams, B O; Miranti, C K; Li, X

    2016-05-01

    Breast cancer (BCa) bone metastases cause osteolytic bone lesions, which result from the interactions of metastatic BCa cells with osteoclasts and osteoblasts. Osteoclasts differentiate from myeloid lineage cells. To understand the cell-specific role of transforming growth factor beta (TGF-β) in the myeloid lineage, in BCa bone metastases, MDA-MB-231 BCa cells were intra-tibially or intra-cardially injected into LysM(Cre)/Tgfbr2(floxE2/floxE2) knockout (LysM(Cre)/Tgfbr2 KO) or Tgfbr2(floxE2/floxE2) mice. Metastatic bone lesion development was compared by analysis of both lesion number and area. We found that LysM(Cre)/Tgfbr2 knockout significantly decreased MDA-MB-231 bone lesion development in both the cardiac and tibial injection models. LysM(Cre)/Tgfbr2 knockout inhibited the tumor cell proliferation, angiogenesis and osteoclastogenesis of the metastatic bones. Cytokine array analysis showed that basic fibroblast growth factor (bFGF) was downregulated in MDA-MB-231-injected tibiae from the LysM(Cre)/Tgfbr2 KO group, and intravenous injection of the recombinant bFGF to LysM(Cre)/Tgfbr2 KO mice rescued the inhibited metastatic bone lesion development. The mechanism by which bFGF rescued the bone lesion development was by promotion of tumor cell proliferation through the downstream mitogen-activated protein kinase (MAPK)-extracellular signal-regulated kinase (ERK)-cFos pathway after binding to the FGF receptor 1 (FGFR1). Consistent with animal studies, we found that in human BCa bone metastatic tissues, TGF-β type II receptor (TβRII) and p-Smad2 were expressed in osteoclasts and tumor cells, and were correlated with the expression of FGFR1. Our studies suggest that myeloid-specific TGF-β signaling-mediated bFGF in the bone promotes BCa bone metastasis. PMID:26279296

  18. Giant myxoinflammatory fibroblastic sarcoma with bone invasion:a very rare clinical entity and literature review

    Institute of Scientific and Technical Information of China (English)

    Guray Togral; Murat Arikan; Elif Aktas; Safak Gungor

    2014-01-01

    Myxoinflammatory fibroblastic sarcoma (MIFS) is a rare low-grade, malignant soft tissue tumor that is usually observed in the extremities of adult patients. Magnetic resonance imaging findings for this tumor type have rarely been reported. We report a case involving the distal left femur of a middle-aged man and tumoral invasion of the bone, which, to our knowledge, has been previously described only once. He was treated with distal femoral tumor resection and reconstruction with a modular prosthesis. Histopathologic diagnosis confirmed MIFS. We reviewed literature of the diagnostic imaging and bone invasion findings associated with this tumor type.

  19. Bone sarcomas linked to radiotherapy and chemotherapy in children

    International Nuclear Information System (INIS)

    We estimated the risk of subsequent bone cancer among 9170 patients who had survived two or more years after the diagnosis of a cancer in childhood. As compared with the general population, the patients had a relative risk of 133 (95 percent confidence interval, 98 to 176) and a mean (+/- SE) 20-year cumulative risk of 2.8 +/- 0.7 percent. Detailed data on treatment were obtained on 64 patients in whom bone cancer developed after childhood cancer. As compared with 209 matched controls who had survived cancer in childhood but who did not have bone cancer later, patients who had had radiation therapy had a 2.7-fold risk (95 percent confidence interval, 1.0 to 7.7) and a sharp dose-response gradient reaching a 40-fold risk after doses to the bone of more than 6000 rad. The relative dose-response effect among patients who had been treated for retinoblastoma resembled that among patients with all other types of initial tumors, although the cumulative risk of bone cancer in the retinoblastoma group was higher. Similar numbers of patients were treated with orthovoltage and megavoltage; the patterns of risk among categories of doses did not differ according to the type of voltage. After adjustment for radiation therapy, treatment with alkylating agents was also linked to bone cancer (relative risk, 4.7; 95 percent confidence interval, 1.0 to 22.3), with the risk increasing as cumulative drug exposure rose. We conclude that both radiotherapy and chemotherapy with alkylating agents for childhood cancer increase the subsequent risk of bone cancer

  20. Can Bone Tissue Engineering Contribute to Therapy Concepts after Resection of Musculoskeletal Sarcoma?

    Directory of Open Access Journals (Sweden)

    Boris Michael Holzapfel

    2013-01-01

    Full Text Available Resection of musculoskeletal sarcoma can result in large bone defects where regeneration is needed in a quantity far beyond the normal potential of self-healing. In many cases, these defects exhibit a limited intrinsic regenerative potential due to an adjuvant therapeutic regimen, seroma, or infection. Therefore, reconstruction of these defects is still one of the most demanding procedures in orthopaedic surgery. The constraints of common treatment strategies have triggered a need for new therapeutic concepts to design and engineer unparalleled structural and functioning bone grafts. To satisfy the need for long-term repair and good clinical outcome, a paradigm shift is needed from methods to replace tissues with inert medical devices to more biological approaches that focus on the repair and reconstruction of tissue structure and function. It is within this context that the field of bone tissue engineering can offer solutions to be implemented into surgical therapy concepts after resection of bone and soft tissue sarcoma. In this paper we will discuss the implementation of tissue engineering concepts into the clinical field of orthopaedic oncology.

  1. The reverse protraction factor in the induction of bone sarcomas in radium-224 patients

    International Nuclear Information System (INIS)

    More than 50 bone sarcomas have occurred among a collective of about 800 patients who had been injected in Germany after World War II with large activities of radium-224 for the intended treatment of bone tuberculosis and ankylosing spondylitis. In an earlier analysis it was concluded that, at equal mean absorbed doses in the skeleton, patients with longer exposure time had a higher incidence of bone sarcomas. The previous analysis was based on approximations; in particular, it did not account for the varying times at risk of the individual patients. In view of the implications of a reverse protraction factor for basic considerations in radiation protection, the need was therefore felt to reevaluate the data from the continued follow-up by more rigorous statistical methods. A first step of the analysis demonstrates the existence of the reverse dose-rate effect in terms of a suitably constructed rank-order test. In a second step of the analysis it is concluded that the data are consistent with a linear no-threshold dose dependence under the condition of constant exposure time, while there is a steeper than linear dependence on dose when the exposure times increase proportionally to dose. A maximum likelihood fit of the data is then performed in terms of a proportional hazards model that includes the individual parameters, dose, treatment duration, and age at treatment. The fit indicates proportionality of the tumor rates to mean skeletal dose with an added factor (1 + 0.18.tau), where tau is the treatment time in months. This indicates that a protraction of the injections over 15 months instead of 5 months doubles the risk of bone sarcoma

  2. Significance of bone marrow histology in the diagnosis of acute myeloid leukemia

    International Nuclear Information System (INIS)

    Acute Myeloid Leukemia (AML) is a heterogeneous disease. The precise diagnosis requires a careful morphological examination of a well pre-pared bone marrow aspirate along with flow cytometry and genetic analysis wherever required. Traditionally, bone marrow biopsy has not been considered an essential diagnostic modality for AML. The aim of this study was to assess the diagnostic as well as prognostic significance of bone marrow histology in patient with acute myeloid leukemia. Forty (40) patients of AML underwent a bone marrow examination including an aspirate and a trephine biopsy. Air dried films of peripheral blood and aspirates were fixed in methanol and stained with Giemsa. The following cytochemical stains were also applied: PAS, Myeloperoxidase, Non specific esterase, Chloracetate Esterase and Acid Phosphatase, and SBB. Bone marrow biopsy specimens were obtained from post superior iliac crest with a manual trephine and were processed in plastic after decalcification. Results: In all the cases there were better diagnostic clues through histological examination of bone marrow particularly in assessing the cellularity, degree of fibrosis, extent of blast infiltration, percentage of inflammatory cells, dysplastic changes and residual haematopoiesis. All these features were better noted in histological examination of core biopsy. The histological examination provided information additional to that provided by aspirate smears about the bone marrow changes in AML and suggested that some of the features may also have pro-gnostic significance in addition to diagnostic importance. (author)

  3. Lysophosphatidic acid mediates myeloid differentiation within the human bone marrow microenvironment.

    Directory of Open Access Journals (Sweden)

    Denis Evseenko

    Full Text Available Lysophosphatidic acid (LPA is a pleiotropic phospholipid present in the blood and certain tissues at high concentrations; its diverse effects are mediated through differential, tissue specific expression of LPA receptors. Our goal was to determine if LPA exerts lineage-specific effects during normal human hematopoiesis. In vitro stimulation of CD34+ human hematopoietic progenitors by LPA induced myeloid differentiation but had no effect on lymphoid differentiation. LPA receptors were expressed at significantly higher levels on Common Myeloid Progenitors (CMP than either multipotent Hematopoietic Stem/Progenitor Cells (HSPC or Common Lymphoid Progenitors (CLP suggesting that LPA acts on committed myeloid progenitors. Functional studies demonstrated that LPA enhanced migration, induced cell proliferation and reduced apoptosis of isolated CMP, but had no effect on either HSPC or CLP. Analysis of adult and fetal human bone marrow sections showed that PPAP2A, (the enzyme which degrades LPA was highly expressed in the osteoblastic niche but not in the perivascular regions, whereas Autotaxin (the enzyme that synthesizes LPA was expressed in perivascular regions of the marrow. We propose that a gradient of LPA with the highest levels in peri-sinusoidal regions and lowest near the endosteal zone, regulates the localization, proliferation and differentiation of myeloid progenitors within the bone marrow marrow.

  4. Bone sarcoma as a second malignant neoplasm in children: influence of radiation and genetic predisposition

    International Nuclear Information System (INIS)

    Osteosarcoma or chondrosarcoma developed as a second malignant neoplasm (SMN) in 40 of 188 individuals with SMN whose first neoplasm occurred in childhood. A genetic susceptibility to cancer was found in 23; the SMN developed in an irradiated field in 32; both factors were present in 16; neither in one. When a genetic predisposition was present, radiation shortened the interval to SMN. The intervals between tumors and the age at which the bone sarcomas developed in relation to genetic disease and therapy were analyzed by a two-mutation hypothesis

  5. Management of high-grade bone sarcomas over two decades: The Norwegian Radium Hospital experience

    Energy Technology Data Exchange (ETDEWEB)

    Hege Aksnes, Liv; Sundby Hall, Kirsten; Folleraas, Gunnar; Stenwig, Anna Elisabeth; Bjerkehagen, Bodil; Taksdal, Ingeborg; Winderen, Mette; Bruland, Oeyvind Sverre; Saeter, Gunnar [The Norwegian Radium Hospital, Montebello, Oslo (Norway)

    2006-02-15

    All cases of high-grade osteosarcoma (OS) (n=196) and Ewing's sarcoma of bone (ES) (n=56) treated at the Norwegian Radium Hospital in the period 1980-1999 were analyzed retrospectively. They were allocated to consecutive ten-year periods by their time of diagnosis. Patient and tumour characteristics have been relatively stable. Eighty percent of all patients received surgical treatment and the amputation rate decreased from 64% to 23%. The percentage of patients receiving chemotherapy has remained around 80%. The use of radiotherapy in primary treatment decreased gradually from 33% to 18%. Sarcoma specific survival (SSS) at five years for all patients increased significantly from 39% to 53%. Similar trends for improvement were seen for both OS and ES. In multivariate analysis, independent prognostic factors for improved SSS were non-metastatic disease at diagnosis, age under 40, extremity tumours, small tumours and treatment from 1995 onwards. No major new treatment options have emerged over these 20 years. The improved outcome appears partly to be due to refinements in the use of existing modalities and improved quality and integration of multidisciplinary approaches. Improved formalized organisation of the sarcoma group and annual audited reports of its patient and research activity may also have contributed to improved focus and performance.

  6. CD13-positive bone marrow-derived myeloid cells promote angiogenesis, tumor growth, and metastasis

    OpenAIRE

    Dondossola, Eleonora; Rangel, Roberto; Guzman-Rojas, Liliana; Barbu, Elena M.; Hosoya, Hitomi; St. John, Lisa S.; Molldrem, Jeffrey J.; Corti, Angelo; Sidman, Richard L.; Arap, Wadih; Pasqualini, Renata

    2013-01-01

    The progression of many solid tumors is associated with increased vascularization. We previously recognized involvement in tumor development and angiogenesis of tumor stromal cells expressing the CD13 protease aminopeptidase. The basic biological concept of participation of nontumor cells in the cancer stroma microenvironment is strengthened in the present study by our finding that a CD11b+CD13+ myeloid subset of bone marrow-derived cells affects pericyte biology and angiogenesis and thereby ...

  7. 5-Azacytidine as Salvage Treatment in Relapsed Myeloid Tumors after Allogeneic Bone Marrow Transplantation

    OpenAIRE

    Bolaños-Meade, Javier; Smith, B. Douglas; Gore, Steven D.; McDevitt, Michael A.; Luznik, Leo; Fuchs, Ephraim J.; Jones, Richard J.

    2010-01-01

    Relapse after allogeneic blood or marrow transplantation carries a very poor prognosis. Current strategies for management that include donor lymphocyte infusions (DLIs) and salvage chemotherapies are usually toxic and ineffective. Here we report the outcome of 10 patients with myeloid malignancies that received 5-azacytidine after a failed allogeneic bone marrow transplant. Of the 10 patients, 6 achieved a complete remission, 1 had stable disease, and 3 progressed after a median of 6 cycles a...

  8. Changing bone marrow micro-environment during development of acute myeloid leukaemia in rats

    DEFF Research Database (Denmark)

    Mortensen, B T; Jensen, P O; Helledie, N;

    1998-01-01

    bromodeoxyuridine (BrdUrd) to identify DNA replicating cells. The leukaemia progressed slowly until day 27 after which a rapid deterioration could be observed leading to severe changes over the following 5 d. In whole blood there was evidence of progressing metabolic acidosis. In bone marrow the fraction of......The Brown Norwegian rat transplanted with promyelocytic leukaemic cells (BNML) has been used as a model for human acute myeloid leukaemia. We have previously shown that both the blood supply to the bone marrow and the metabolic rate decrease in relation to the leukaemic development in these rats...

  9. 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

  10. Bone sarcoma induction by 224Ra in Beagles: An interim report

    International Nuclear Information System (INIS)

    During 1963-1968, 20 young adult Beagle dogs were given a single intravenous injection of 224Ra that was unintentionally contaminated with 210Pb and 228Th. All of these dogs have died, eight with bone sarcomas. In addition, 128 Beagle dogs were given purified 224Ra in one, ten or fifty weekly injections during 1977-1979. The acute lethal dosage (LD50) for a single injection was about 400 kBq 224Ra/kg (11 μCi 224Ra/kg). As of September 14, 1987, at the highest skeletal dose of 3 Gy from purified 224Ra, bone sarcomas occurred in 5 of 6 dogs at an average ± SD of 2069 ± 302 days after the start of injections (no survivors), in 4 of 6 dogs at 2485 ± 1,110 days after the start of 10 injections (healthy survivor) and in 1 of 8 dogs at 3066 days after a single injection (3 healthy survivors). At 3 Gy the toxicity of 224Ra relative to 239Pu, based on the ratio of skeletal doses at equal tumor appearance times, was 1.0 for 50 injections of 224Ra, 0.7 for 10 injections, and 0.4 for one injection. In contrast, the effectiveness per Gy of a 224Ra-equivalent dose from a single injection of 224Ra contaminated with 228Th and 210Pb was equal to that from 239Pu, assuming that the 224Ra-equivalent dose equaled the 224Ra dose plus the 228Th dose pus 0.1 of the 210Pb dose. A possible explanation is that protracted irradiation from the 1.91-yr half-life 228Th and the 22.3-yr half-life 210Pb may have 'activated' residual damage from the short-lived, 3.62 day half-life 224Ra, possibly by stimulating the division of damaged stem cells to replace the bone cells killed by the protracted alpha particle irradiation. An important spin off from the study is experimental support for the assumption that the bone sarcoma effectiveness for 210Pb is similar to that from long-lived, 1600 yr half-life 226Ra. (author)

  11. Bone marrow derived myeloid cells orchestrate antiangiogenic resistance in glioblastoma through coordinated molecular networks.

    Science.gov (United States)

    Achyut, B R; Shankar, Adarsh; Iskander, A S M; Ara, Roxan; Angara, Kartik; Zeng, Peng; Knight, Robert A; Scicli, Alfonso G; Arbab, Ali S

    2015-12-28

    Glioblastoma (GBM) is a hypervascular and malignant form of brain tumors. Anti-angiogenic therapies (AAT) were used as an adjuvant against VEGF-VEGFR pathway to normalize blood vessels in clinical and preclinical studies, which resulted into marked hypoxia and recruited bone marrow derived cells (BMDCs) to the tumor microenvironment (TME). In vivo animal models to track BMDCs and investigate molecular mechanisms in AAT resistance are rare. We exploited recently established chimeric mouse to develop orthotopic U251 tumor, which uses as low as 5 × 10(6) GFP+ BM cells in athymic nude mice and engrafted >70% GFP+ cells within 14 days. Our unpublished data and published studies have indicated the involvement of immunosuppressive myeloid cells in therapeutic resistance in glioma. Similarly, in the present study, vatalanib significantly increased CD68+ myeloid cells, and CD133+, CD34+ and Tie2+ endothelial cell signatures. Therefore, we tested inhibition of CSF1R+ myeloid cells using GW2580 that reduced tumor growth by decreasing myeloid (Gr1+ CD11b+ and F4/80+) and angiogenic (CD202b+ and VEGFR2+) cell signatures in TME. CSF1R blockade significantly decreased inflammatory, proangiogenic and immunosuppressive molecular signatures compared to vehicle, vatalanib or combination. TCK1 or CXCL7, a potent chemoattractant and activator of neutrophils, was observed as most significantly decreased cytokine in CSF1R blockade. ERK MAPK pathway was involved in cytokine network regulation. In conclusion, present study confirmed the contribution of myeloid cells in GBM development and therapeutic resistance using chimeric mouse model. We identified novel molecular networks including CXCL7 chemokine as a promising target for future studies. Nonetheless, survival studies are required to assess the beneficial effect of CSF1R blockade. PMID:26404753

  12. Bone marrow cells participate in tumor vessel formation that supports the growth of Ewing’s sarcoma in the lung

    OpenAIRE

    Zhou, Zhichao; Stewart, Keri Schadler; Yu, Ling; Kleinerman, Eugenie S.

    2010-01-01

    An MHC-mismatch bone marrow (BM) transplant Ewing’s sarcoma mouse model was used to investigate whether BM cells participate in the vessel formation that support Ewing’s sarcoma lung metastasis. BM cells from H-2Kb/d donor mice were transplanted into sublethally irradiated H-2Kd recipient mice. Donor BM cells were identified using the H-2Kb marker. Engraftment was confirmed by identifying the H-2Kb IL-1β-type specific polymorphism. After engraftment highly lung metastatic TC71-PM4 cells were ...

  13. Myeloid derived suppressor cells regulate growth of multiple myeloma by inhibiting T cells in bone marrow

    OpenAIRE

    Ramachandran, Indu; Martner, Anna; Pisklakova, Alexandra; Condamine, Thomas; Chase, Tess; Vogl, Thomas; Roth, Johannes; Gabrilovich, Dmitry; Nefedova, Yulia

    2013-01-01

    Myeloid-derived suppressor cells (MDSC) are one of the major factors limiting immune response in cancer. However, their role in bone marrow (BM), the site of primary localization of multiple myeloma (MM), is poorly understood. In this study we found a significant accumulation of CD11b+CD14−CD33+ immune suppressive MDSC in BM of patients with newly diagnosed MM. To assess the possible role of MDSC in MM, we used immune competent mouse models. Immune suppressive MDSC accumulated in BM of mice a...

  14. [A CASE OF GRANULOCYTIC SARCOMA IN THE BILATERAL URETER].

    Science.gov (United States)

    Miyagawa, Tomoaki; Tanaka, Ken; Ikeda, Atsushi; Komine, Manabu; Tsutsumi, Masakazu; Shinagawa, Atsushi

    2015-07-01

    A 61-year-old female presented with the complaints of fever and left back pain. She had previously undergone bone marrow transplantation for acute myeloid leukemia and achieved remission. Abdominal computed tomography (CT) revealed left hydronephrosis and suspected tumor lesions in the right upper ureter and left lower ureter. Ureteroscopy was performed for a diagnosis. A yellowish tumor was detected in the left lower ureter, and tissue biopsy was performed. Two weeks after the examination, abdominal CT revealed ascites and retroperitoneal dissemination. The results of the ascites cytology showed infiltration by leukemia cells, and the patient was diagnosed as having recurrent leukemia. A week later, she died. The ureteral tumor was diagnosed as a granulocytic sarcoma. Granulocytic sarcoma, a condition characterized by mass formation outside the bone marrow by granulocytic cells, is classified as a myeloid sarcoma, occurring in an estimated 2-8% of patients with myeloid leukemia. The possibility of granulocytic sarcoma should be considered when treating patients with a history of leukemia. PMID:26419078

  15. Surgical Guides (Patient-Specific Instruments for Pediatric Tibial Bone Sarcoma Resection and Allograft Reconstruction

    Directory of Open Access Journals (Sweden)

    Laura Bellanova

    2013-01-01

    Full Text Available To achieve local control of malignant pediatric bone tumors and to provide satisfactory oncological results, adequate resection margins are mandatory. The local recurrence rate is directly related to inappropriate excision margins. The present study describes a method for decreasing the resection margin width and ensuring that the margins are adequate. This method was developed in the tibia, which is a common site for the most frequent primary bone sarcomas in children. Magnetic resonance imaging (MRI and computerized tomography (CT were used for preoperative planning to define the cutting planes for the tumors: each tumor was segmented on MRI, and the volume of the tumor was coregistered with CT. After preoperative planning, a surgical guide (patient-specific instrument that was fitted to a unique position on the tibia was manufactured by rapid prototyping. A second instrument was manufactured to adjust the bone allograft to fit the resection gap accurately. Pathologic evaluation of the resected specimens showed tumor-free resection margins in all four cases. The technologies described in this paper may improve the surgical accuracy and patient safety in surgical oncology. In addition, these techniques may decrease operating time and allow for reconstruction with a well-matched allograft to obtain stable osteosynthesis.

  16. Granulocytic sarcoma masquerading as Ewing′s sarcoma: A diagnostic dilemma

    Directory of Open Access Journals (Sweden)

    Haresh Kunhi

    2008-01-01

    Full Text Available An eleven-year-old boy presented with a swelling in his left elbow. Radiologically the features were that of an Ewing′s sarcoma involving the ulna. Histopathology showed small round cell tumor strongly positive for Monoclonal Imperial Cancer research fund 2 (MIC2 antigen. Similar cells in the bone marrow were involved with MIC2 positivity. The patient developed skin lesions, which on biopsy were found to be chloromas. The initial biopsies were reevaluated with special stains revealing granulocytic sarcomas in acute myeloid leukemia masquerading as Ewing′s due to its MIC2 positivity. The possibility of myeloid neoplasms should be considered routinely with known MIC2 positive round cell tumors.

  17. Kaposi's Sarcoma-Associated Herpesvirus OX2 Glycoprotein Activates Myeloid-Lineage Cells To Induce Inflammatory Cytokine Production

    OpenAIRE

    Chung, Young-Hwa; Means, Robert E.; Choi, Joong-Kook; Lee, Bok-Soo; Jae U. Jung

    2002-01-01

    Kaposi's sarcoma is an inflammatory cytokine-mediated angioproliferative disease which is triggered by infection by Kaposi's sarcoma-associated herpesvirus (KSHV). KSHV contains an open reading frame, K14, that has significant homology with cellular OX2, designated viral OX2 (vOX2). In this report, we demonstrate that vOX2 encodes a glycosylated cell surface protein with an apparent molecular mass of 55 kDa. Purified glycosylated vOX2 protein dramatically stimulated primary monocytes, macroph...

  18. 5-Azacytidine as Salvage Treatment in Relapsed Myeloid Tumors after Allogeneic Bone Marrow Transplantation

    Science.gov (United States)

    Bolaños-Meade, Javier; Smith, B. Douglas; Gore, Steven D.; McDevitt, Michael A.; Luznik, Leo; Fuchs, Ephraim J.; Jones, Richard J.

    2011-01-01

    Relapse after allogeneic blood or marrow transplantation carries a very poor prognosis. Current strategies for management that include donor lymphocyte infusions (DLIs) and salvage chemotherapies are usually toxic and ineffective. Here we report the outcome of 10 patients with myeloid malignancies that received 5-azacytidine after a failed allogeneic bone marrow transplant. Of the 10 patients, 6 achieved a complete remission, 1 had stable disease, and 3 progressed after a median of 6 cycles administered. Only 1 patient has died (of disease progression), and no flares of graft-versus-host disease (GVHD) were observed with 5-azacytidine. As of latest follow-up, the median overall survival (OS) for the group was 422.5 days (127–1411). These results further suggest that 5-azacytidine is an active agent after failing an allogeneic bone marrow transplant, and prospective studies are warranted. PMID:20951817

  19. Omega 3 fatty acids reduce myeloid progenitor cell frequency in the bone marrow of mice and promote progenitor cell differentiation

    Directory of Open Access Journals (Sweden)

    Sollars Vincent E

    2009-03-01

    Full Text Available Abstract Background Omega 3 fatty acids have been found to inhibit proliferation, induce apoptosis, and promote differentiation in various cell types. The processes of cell survival, expansion, and differentiation are of key importance in the regulation of hematopoiesis. We investigated the role of omega 3 fatty acids in controlling the frequency of various myeloid progenitor cells in the bone marrow of mice. Increased progenitor cell frequency and blocked differentiation are characteristics of hematopoietic disorders of the myeloid lineage, such as myeloproliferative diseases and myeloid leukemias. Results We found that increasing the proportion of omega 3 fatty acids relative to the proportion of omega 6 fatty acids in the diet caused increased differentiation and reduced the frequency of myeloid progenitor cells in the bone marrow of mice. Furthermore, this had no adverse effect on peripheral white blood cell counts. Conclusion Our results indicate that omega 3 fatty acids impact hematopoietic differentiation by reducing myeloid progenitor cell frequency in the bone marrow and promoting progenitor cell differentiation. Further exploration of this discovery could lead to the use of omega 3 fatty acids as a therapeutic option for patients that have various disorders of hematopoiesis.

  20. Vascularized fibula grafts for reconstruction of bone defects after resection of bone sarcomas

    DEFF Research Database (Denmark)

    Petersen, Michael Mørk; Hovgaard, Dorrit; Elberg, Jens Jørgen; Rechnitzer, Catherine; Daugaard, Søren; Muhic, Aida

    2010-01-01

    /3/1) operated on form 2000 to 2006. The bone defects reconstructed were proximal femoral diaphysis and epiphysis (n = 2), humeral diaphysis (n = 2), humeral proximal diaphysis and epiphysis (n = 1), femoral diaphysis (n = 1), ulnar diaphysis (n = 1), and tibial diaphysis (n = 1). One patient with Ewing...

  1. Collecting and Storing Tissue, Blood, and Bone Marrow Samples From Patients With Rhabdomyosarcoma or Other Soft Tissue Sarcoma

    Science.gov (United States)

    2016-03-18

    Adult Rhabdomyosarcoma; Childhood Desmoplastic Small Round Cell Tumor; Chordoma; Desmoid Tumor; Metastatic Childhood Soft Tissue Sarcoma; Nonmetastatic Childhood Soft Tissue Sarcoma; Previously Treated Childhood Rhabdomyosarcoma; Previously Untreated Childhood Rhabdomyosarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Childhood Rhabdomyosarcoma; Recurrent Childhood Soft Tissue Sarcoma; Stage I Adult Soft Tissue Sarcoma; Stage II Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

  2. Prognostic value of IGF-1R expression in bone and soft tissue sarcomas: a meta-analysis

    Directory of Open Access Journals (Sweden)

    Liang JB

    2015-07-01

    Full Text Available Junbo Liang,1,2 Binghao Li,3 Li Yuan,4 Zhaoming Ye3 1School of Clinical Medicine, Wenzhou Medical University, Wenzhou, 2Department of Orthopedics, Taizhou Hospital, Taizhou, 3Department of Orthopaedics, Institute of Orthopaedic Research, Second Affiliated Hospital of Zhejiang University School of Medicine, Hangzhou, 4School of Public Health, Fudan University, Shanghai, People’s Republic of China Abstract: Accumulated evidence has indicated a correlation between IGF-1R and bone and soft tissue sarcoma (BSTS progression. However, research on the prognostic role of IGF-1R in sarcomas has revealed very different or even totally opposite results. This meta-analysis aimed to unveil the controversial role IGF-1R plays in predicting the outcome of BSTS patients. We systematically reviewed the evidence for the effect of IGF-1R expression in multiple types of BSTSs, including osteosarcoma, Ewing’s sarcoma, synovial sarcoma, liposarcoma, and rhabdomyosarcoma, to elucidate this issue. The prognostic value of IGF-1R expression in BSTS patients was evaluated regarding overall survival, measured by pooled hazard ratios (HRs with 95% confidence intervals (CIs. Seven studies including 627 patients were enrolled in this meta-analysis. Our results demonstrated that IGF-1R expression was associated with poor outcome in terms of overall survival in BSTS patients (pooled HR =2.15, 95% CI: 1.06–4.38; P=0.03. In subtypes of BSTSs, elevated IGF-1R expression was revealed to be significantly correlated with worse prognosis in osteosarcoma (pooled HR =2.20, 95% CI: 1.59–0.03; P<0.001, while no statistical significance was discovered in Ewing’s sarcoma (pooled HR =1.01, 95% CI: 0.45–2.27; P=0.99. Expression of IGF-1R could be a negative prognostic biomarker for patients suffering from BSTSs. Keywords: overall survival, prognostic significance osteosarcoma, meta-analysis, IGF-1R

  3. HSP10 selective preference for myeloid and megakaryocytic precursors in normal human bone marrow

    Directory of Open Access Journals (Sweden)

    F Cappello

    2009-06-01

    Full Text Available Heat shock proteins (HSPs constitute a heterogeneous family of proteins involved in cell homeostasis. During cell life they are involved in harmful insults, as well as in immune and inflammatory reactions. It is known that they regulate gene expression, and cell proliferation, differentiation and death. HSP60 is a mitochondrial chaperonin, highly preserved during evolution, responsible of protein folding. Its function is strictly dependent on HSP10 in both prokaryotic and eukaryotic elements. We investigated the presence and the expression of HSP60 and HSP10 in a series of 20 normal human bone marrow specimens (NHBM by the means of immunohistochemistry. NHBM showed no expression of HSP60, probably due to its being below the detectable threshold, as already demonstrated in other normal human tissues. By contrast, HSP10 showed a selective positivity for myeloid and megakaryocytic lineages. The positivity was restricted to precursor cells, while mature elements were constantly negative.We postulate that HSP10 plays a role in bone marrow cell differentiation other than being a mitochondrial co-chaperonin. The present data emphasize the role of HSP10 during cellular homeostasis and encourage further investigations in this field.

  4. Ewing sarcoma of the bone in children under 6 years of age.

    Directory of Open Access Journals (Sweden)

    Maria Antonietta De Ioris

    Full Text Available BACKGROUND: Ewing Sarcoma Family Tumours (ESFT are rare in early childhood. The aim of this study was to report the clinical characteristics and outcome of children under 6 years of age affected by ESFT of the bone in Italy. METHODS: The records of all the children diagnosed with osseous ESFT in centres members of the Associazione Italiana di Ematologia ed Oncologia Pediatrica (AIEOP from 1990 to 2008 were reviewed. The Kaplan-Meier method was used for estimating overall and progression-free survival (OS, PFS curves; multivariate analyses were performed using Cox proportional hazards regression model. RESULTS: This study includes 62 patients. An axial primary localization was present in 66% of patients, with the primary site in the chest wall in 34%. Fourteen (23% patients presented metastatic disease. The 5-year OS and PFS were 73% (95% confidence interval, CI, 58-83% and 72% (95% CI 57-83% for patients with localized disease and 38% (95% CI 17-60% and 21% (95% CI 5-45% for patients with metastatic disease. Metastatic spread, skull/pelvis/spine primary localization, progression during treatment and no surgery predicted worse survival (P<0.01, while patients treated in the last decade had better survival (P = 0.002. In fact, the 5-year OS and PFS for patients diagnosed in the period 2000-2008 were 89% (95% CI 71-96% and 86% (95% CI 66-94%, respectively. CONCLUSION: The axial localization is the most common site of ESFT in pre-scholar children. Patients treated in the most recent period have an excellent outcome.

  5. Primary Ewing′s sarcoma of the squamous part of temporal bone in a young girl treated with adjuvant volumetric arc therapy

    OpenAIRE

    Moujhuri Nandi; Jibak Bhattacharya; Suchanda Goswami; Chanchal Goswami

    2015-01-01

    Ewing′s sarcoma (ES)/peripheral primitive neuroectodermal tumors usually arise in the long bones of children and young adults. Primary ES of the cranium is unusual. Treatment involves multi-modality therapy incorporating surgery, radiotherapy and chemotherapy; outcomes are similar to those arising from long bones. We report a case of Primary ES of the squamous part of temporal bone with intracranial extension in a 9-year-old girl who was treated with surgery, chemotherapy followed by adjuvant...

  6. Protective mitochondrial transfer from bone marrow stromal cells to acute myeloid leukemic cells during chemotherapy.

    Science.gov (United States)

    Moschoi, Ruxanda; Imbert, Véronique; Nebout, Marielle; Chiche, Johanna; Mary, Didier; Prebet, Thomas; Saland, Estelle; Castellano, Rémy; Pouyet, Laurent; Collette, Yves; Vey, Norbert; Chabannon, Christian; Recher, Christian; Sarry, Jean-Emmanuel; Alcor, Damien; Peyron, Jean-François; Griessinger, Emmanuel

    2016-07-14

    Here we demonstrate that in a niche-like coculture system, cells from both primary and cultured acute myeloid leukemia (AML) sources take up functional mitochondria from murine or human bone marrow stromal cells. Using different molecular and imaging approaches, we show that AML cells can increase their mitochondrial mass up to 14%. After coculture, recipient AML cells showed a 1.5-fold increase in mitochondrial adenosine triphosphate production and were less prone to mitochondrial depolarization after chemotherapy, displaying a higher survival. This unidirectional transfer enhanced by some chemotherapeutic agents required cell-cell contacts and proceeded through an endocytic pathway. Transfer was greater in AML blasts compared with normal cord blood CD34(+) cells. Finally, we demonstrate that mitochondrial transfer was observed in vivo in an NSG immunodeficient mouse xenograft model and also occurred in human leukemia initiating cells and progenitors. As mitochondrial transfer provides a clear survival advantage following chemotherapy and a higher leukemic long-term culture initiating cell potential, targeting mitochondrial transfer could represent a future therapeutic target for AML treatment. PMID:27257182

  7. Total lymphoid irradiation preceding bone marrow transplantation for chronic myeloid leukaemia

    International Nuclear Information System (INIS)

    Between August 1985 and October 1987 35 patients with chronic myeloid leukaemia (CML) were treated by high dose chemotherapy, total body irradiation (TBI) (1000 or 1200 cGy, n=31) and total lymphoid irradiation (TLI) (800 or 600 cGy, n=35) preceding allogeneic bone marrow transplantation (BMT). Both TBI and TLI were given at 200 cGy/fraction. Twenty-three patients had HLA-identical sibling donors, nine patients had HLA-matched but unrelated donors, and three partially HLA-mismatched donors. Twenty-two patients received T-cell depleted marrow. TLI did not add greatly to the toxicity. Four patients had recurrent leukaemia before engraftment was evaluable. The other 31 patients engrafted and no graft failed. Twenty-two patients survive at a median time from transplant of 305 days (range 81-586 days). Fourteen have no evidence of disease; eight have or had only cytogenetic evidence of leukaemia. It is concluded that addition of TLI to pretransplant immunosuppression increases the probability of reliable engraftment in patients receiving T-cell depleted marrow. This is not associated with significantly increased toxicity. (author)

  8. Notch signalling drives bone marrow stromal cell-mediated chemoresistance in acute myeloid leukemia.

    Science.gov (United States)

    Takam Kamga, Paul; Bassi, Giulio; Cassaro, Adriana; Midolo, Martina; Di Trapani, Mariano; Gatti, Alessandro; Carusone, Roberta; Resci, Federica; Perbellini, Omar; Gottardi, Michele; Bonifacio, Massimiliano; Nwabo Kamdje, Armel Hervé; Ambrosetti, Achille; Krampera, Mauro

    2016-04-19

    Both preclinical and clinical investigations suggest that Notch signalling is critical for the development of many cancers and for their response to chemotherapy. We previously showed that Notch inhibition abrogates stromal-induced chemoresistance in lymphoid neoplasms. However, the role of Notch in acute myeloid leukemia (AML) and its contribution to the crosstalk between leukemia cells and bone marrow stromal cells remain controversial. Thus, we evaluated the role of the Notch pathway in the proliferation, survival and chemoresistance of AML cells in co-culture with bone marrow mesenchymal stromal cells expanded from both healthy donors (hBM-MSCs) and AML patients (hBM-MSCs*). As compared to hBM-MSCs, hBM-MSCs* showed higher level of Notch1, Jagged1 as well as the main Notch target gene HES1. Notably, hBM-MSCs* induced expression and activation of Notch signalling in AML cells, supporting AML proliferation and being more efficientin inducing AML chemoresistance than hBM-MSCs*. Pharmacological inhibition of Notch using combinations of Notch receptor-blocking antibodies or gamma-secretase inhibitors (GSIs), in presence of chemotherapeutic agents, significant lowered the supportive effect of hBM-MSCs and hBM-MSCs* towards AML cells, by activating apoptotic cascade and reducing protein level of STAT3, AKT and NF-κB.These results suggest that Notch signalling inhibition, by overcoming the stromal-mediated promotion of chemoresistance,may represent a potential therapeutic targetnot only for lymphoid neoplasms, but also for AML. PMID:26967055

  9. Simultaneous malignancies consisting of cecal cancer, sigmoid colon cancer and pleomorphic sarcoma around the left iliac bone

    International Nuclear Information System (INIS)

    A 71-year-old woman, who had surgery and subsequent irradiation for uterine canner 29 years prior, developed pain around her left hip and leg. Computed tomography showed a soft tissue density mass around the left iliac bone measuring 13 cm in diameter. An incisional biopsy revealed a pleomorphic sarcoma. On positron emission tomography and colonoscopy, a cecal cancer a sigmoid colon cancer were detected. These two colonic cancers were surgically removed to prevent bowel obstruction. Both of the tumors presented as bulky masses with well-defined margins invading the proper muscle layers and the surrounding colonic wall; they exhibited dysplasia, foamy cells, and thickened arterial walls that showed hyalinization, indicating radiation-induced colitis. Namely, all these tumors including the pleomorphic sarcoma around the iliac bone were considered to be radiation-induced tumors. Radiation-induced cancer is a late complication of irradiation and when diagnosed is often advanced. Both patients and clinicians should be cognizant of the potential consequences of irradiation; appropriate follow-up should be instituted. (author)

  10. Quality of Life and Utility in Patients with Metastatic Soft Tissue and Bone Sarcoma: The Sarcoma Treatment and Burden of Illness in North America and Europe (SABINE Study

    Directory of Open Access Journals (Sweden)

    Peter Reichardt

    2012-01-01

    Full Text Available The aim of the study was to assess health-related quality of life (HRQoL among metastatic soft tissue (mSTS or bone sarcoma (mBS patients who had attained a favourable response to chemotherapy. We employed the EORTC QLQ-C30, the 3-item Cancer-Related Symptoms Questionnaire, and the EQ-5D instrument. HRQoL was evaluated overall and by health state in 120 mSTS/mBS patients enrolled in the SABINE study across nine countries in Europe and North America. Utility was estimated from responses to the EQ-5D instrument using UK population-based weights. The mean EQ-5D utility score was 0.69 for the pooled patient sample with little variation across health states. However, patients with progressive disease reported a clinically significant lower utility (0.56. Among disease symptoms, pain and respiratory symptoms are common. This study showed that mSTS/mBS is associated with reduced HRQoL and utility among patients with metastatic disease.

  11. Quality of Life and Utility in Patients with Metastatic Soft Tissue and Bone Sarcoma: The Sarcoma Treatment and Burden of Illness in North America and Europe (SABINE) Study

    Science.gov (United States)

    Reichardt, Peter; Leahy, Michael; Garcia del Muro, Xavier; Ferrari, Stefano; Martin, Javier; Gelderblom, Hans; Wang, Jingshu; Krishna, Arun; Eriksson, Jennifer; Staddon, Arthur; Blay, Jean-Yves

    2012-01-01

    The aim of the study was to assess health-related quality of life (HRQoL) among metastatic soft tissue (mSTS) or bone sarcoma (mBS) patients who had attained a favourable response to chemotherapy. We employed the EORTC QLQ-C30, the 3-item Cancer-Related Symptoms Questionnaire, and the EQ-5D instrument. HRQoL was evaluated overall and by health state in 120 mSTS/mBS patients enrolled in the SABINE study across nine countries in Europe and North America. Utility was estimated from responses to the EQ-5D instrument using UK population-based weights. The mean EQ-5D utility score was 0.69 for the pooled patient sample with little variation across health states. However, patients with progressive disease reported a clinically significant lower utility (0.56). Among disease symptoms, pain and respiratory symptoms are common. This study showed that mSTS/mBS is associated with reduced HRQoL and utility among patients with metastatic disease. PMID:22550425

  12. A reappraisal of hemangiopericytoma of bone; analysis of cases reclassified as synovial sarcoma and solitary fibrous tumor of bone

    DEFF Research Database (Denmark)

    Verbeke, Sofie L J; Fletcher, Christopher D M; Alberghini, Marco; Daugaard, Søren; Flanagan, Adrienne M; Parratt, Tim; Kroon, Herman M; Hogendoorn, Pancras C W; Bovée, Judith V M G

    2010-01-01

    Hemangiopericytoma (HPC) was first described as a neoplasm with distinct morphologic features, presumably composed of pericytes. In soft tissue, it is accepted that most such lesions are solitary fibrous tumors (SFTs), monophasic synovial sarcomas (SSs), or myofibromatoses. It is unclear whether...

  13. Tetraspanin CD82 regulates bone marrow homing of acute myeloid leukemia by modulating the molecular organization of N-cadherin

    Science.gov (United States)

    Marjon, Kristopher D.; Termini, Christina M.; Karlen, Karin L.; Saito-Reis, Chelsea; Soria, Cesar E.; Lidke, Keith A.; Gillette, Jennifer M.

    2016-01-01

    Communication between acute myeloid leukemia (AML) and the bone marrow microenvironment is known to control disease progression. Therefore, regulation of AML cell trafficking and adhesion to the bone marrow is of significant interest. In this study, we demonstrate that differential expression of the membrane scaffold CD82 modulates the bone marrow homing of AML cells. By combining mutational analysis and super-resolution imaging, we identify membrane protein clustering by CD82 as a regulator of AML cell adhesion and bone marrow homing. Cluster analysis of super-resolution data indicates that N-linked glycosylation and palmitoylation of CD82 are both critical modifications that control the microdomain organization of CD82 as well as the nanoscale clustering of associated adhesion protein, N-cadherin. We demonstrate that inhibition of CD82 glycosylation increases the molecular packing of N-cadherin and promotes the bone marrow homing of AML cells. In contrast, we find that inhibition of CD82 palmitoylation disrupts the formation and organization of N-cadherin clusters and significantly diminishes bone marrow trafficking of AML. Taken together, these data establish a mechanism where the membrane organization of CD82, through specific post-translational modifications, regulates N-cadherin clustering and membrane density, which impacts the in vivo trafficking of AML cells. As such, these observations provide an alternative model for targeting AML where modulation of protein organization within the membrane may be an effective treatment therapy to disrupt the bone marrow homing potential of AML cells. PMID:26592446

  14. 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

  15. Bone Fractures Following External Beam Radiotherapy and Limb-Preservation Surgery for Lower Extremity Soft Tissue Sarcoma: Relationship to Irradiated Bone Length, Volume, Tumor Location and Dose

    International Nuclear Information System (INIS)

    Purpose: To examine the relationship between tumor location, bone dose, and irradiated bone length on the development of radiation-induced fractures for lower extremity soft tissue sarcoma (LE-STS) patients treated with limb-sparing surgery and radiotherapy (RT). Methods and Materials: Of 691 LE-STS patients treated from 1989 to 2005, 31 patients developed radiation-induced fractures. Analysis was limited to 21 fracture patients (24 fractures) who were matched based on tumor size and location, age, beam arrangement, and mean total cumulative RT dose to a random sample of 53 nonfracture patients and compared for fracture risk factors. Mean dose to bone, RT field size (FS), maximum dose to a 2-cc volume of bone, and volume of bone irradiated to ≥40 Gy (V40) were compared. Fracture site dose was determined by comparing radiographic images and surgical reports to fracture location on the dose distribution. Results: For fracture patients, mean dose to bone was 45 ± 8 Gy (mean dose at fracture site 59 ± 7 Gy), mean FS was 37 ± 8 cm, maximum dose was 64 ± 7 Gy, and V40 was 76 ± 17%, compared with 37 ± 11 Gy, 32 ± 9 cm, 59 ± 8 Gy, and 64 ± 22% for nonfracture patients. Differences in mean, maximum dose, and V40 were statistically significant (p = 0.01, p = 0.02, p = 0.01). Leg fractures were more common above the knee joint. Conclusions: The risk of radiation-induced fracture appears to be reduced if V40 <64%. Fracture incidence was lower when the mean dose to bone was <37 Gy or maximum dose anywhere along the length of bone was <59 Gy. There was a trend toward lower mean FS for nonfracture patients.

  16. Mesenchymal stromal cells derived from acute myeloid leukemia bone marrow exhibit aberrant cytogenetics and cytokine elaboration

    International Nuclear Information System (INIS)

    Bone marrow-derived mesenchymal stromal cells (BM-MSCs) play a fundamental role in the BM microenvironment (BME) and abnormalities of these cells may contribute to acute myeloid leukemia (AML) pathogenesis. The aim of the study was to characterize the cytokine and gene expression profile, immunophenotype and cytogenetics of BM-MSCs from AML patients compared to normal BM-MSCs from healthy donors. AML BM-MSCs showed decreased monocyte chemoattractant protein-1 levels compared to normal BM-MSCs. AML BM-MSCs expressed similar β1 integrin, CD44, CD73, CD90 and E-cadherin compared to normal BM-MSCs. Cytogenetic analysis revealed chromosomal aberrations in AML BM-MSCs, some overlapping with and others distinct from their corresponding AML blasts. No significant difference in gene expression was detected between AML BM-MSCs compared to normal BM-MSCs; however, comparing the differences between AML and MSCs from AML patients with the differences between normal hematopoietic cells and normal MSCs by Ingenuity pathway analysis showed key distinctions of the AML setting: (1) upstream gene regulation by transforming growth factor beta 1, tumor necrosis factor, tissue transglutaminase 2, CCAAT/enhancer binding protein alpha and SWItch/Sucrose NonFermentable related, matrix associated, actin dependent regulator of chromatin, subfamily a, member 4; (2) integrin and interleukin 8 signaling as overrepresented canonical pathways; and (3) upregulation of transcription factors FBJ murine osteosarcoma viral oncogene homolog and v-myb avian myeloblastosis viral oncogene homolog. Thus, phenotypic abnormalities of AML BM-MSCs highlight a dysfunctional BME that may impact AML survival and proliferation

  17. Mesenchymal stromal cells derived from acute myeloid leukemia bone marrow exhibit aberrant cytogenetics and cytokine elaboration.

    Science.gov (United States)

    Huang, J C; Basu, S K; Zhao, X; Chien, S; Fang, M; Oehler, V G; Appelbaum, F R; Becker, P S

    2015-01-01

    Bone marrow-derived mesenchymal stromal cells (BM-MSCs) play a fundamental role in the BM microenvironment (BME) and abnormalities of these cells may contribute to acute myeloid leukemia (AML) pathogenesis. The aim of the study was to characterize the cytokine and gene expression profile, immunophenotype and cytogenetics of BM-MSCs from AML patients compared to normal BM-MSCs from healthy donors. AML BM-MSCs showed decreased monocyte chemoattractant protein-1 levels compared to normal BM-MSCs. AML BM-MSCs expressed similar β1 integrin, CD44, CD73, CD90 and E-cadherin compared to normal BM-MSCs. Cytogenetic analysis revealed chromosomal aberrations in AML BM-MSCs, some overlapping with and others distinct from their corresponding AML blasts. No significant difference in gene expression was detected between AML BM-MSCs compared to normal BM-MSCs; however, comparing the differences between AML and MSCs from AML patients with the differences between normal hematopoietic cells and normal MSCs by Ingenuity pathway analysis showed key distinctions of the AML setting: (1) upstream gene regulation by transforming growth factor beta 1, tumor necrosis factor, tissue transglutaminase 2, CCAAT/enhancer binding protein alpha and SWItch/Sucrose NonFermentable related, matrix associated, actin dependent regulator of chromatin, subfamily a, member 4; (2) integrin and interleukin 8 signaling as overrepresented canonical pathways; and (3) upregulation of transcription factors FBJ murine osteosarcoma viral oncogene homolog and v-myb avian myeloblastosis viral oncogene homolog. Thus, phenotypic abnormalities of AML BM-MSCs highlight a dysfunctional BME that may impact AML survival and proliferation. PMID:25860293

  18. Ewing sarcoma

    International Nuclear Information System (INIS)

    Ewing sarcoma is the second most frequent primary bone cancer affecting children or young adults. Advances in molecular biology have revealed common chromosomal translocations such as EWS-FLI1 among Ewing sarcoma and related diseases such as primitive neuroectodermal tumor (PNET), so these are considered as Ewing sarcoma family tumor (ESFT). Although fewer than 10% of patients with ESFT survived before establishment of modern multiagent chemotherapy, the multimodal therapeutic regimens including combination chemotherapy, radiotherapy, and surgery can cure 60% of patients with localized disease, due to the collaborative research in European-American or the international trials. The standard chemotherapy for localized ESFT now comprises vincristine, actinomycin D, cyclophosphamide and doxorubicin (VACD) in Europe or vincristine, doxorubicin, cyclophosphamide, ifosfamide and etoposide (VDC-IE) in North America. Meanwhile, those with metastatic disease have a much worse outcome with an approximately 10-30% 5-year event-free survival rate. New American-European collaborative trials such as EURO-E.W.I.N.G. 99 are in progress for further improvement of the cure rate in localized and metastatic ESFT. In Japan, Japan Ewing Sarcoma Study Group (JESS) phase II clinical trial for localized ESFT, and some clinical trials including new drugs are ongoing and waiting for results. (author)

  19. Volume-Based F-18 FDG PET/CT Imaging Markers Provide Supplemental Prognostic Information to Histologic Grading in 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

    The aim of the study is to assess the prognostic value of different volume-based calculations of tumor metabolic activity in the initial assessment of patients with high-grade bone sarcomas (BS) and soft tissue sarcomas (STS) using F-18 FDG PET/CT.A single-site, retrospective study from 2002 to...

  20. Expression of Neuropilin-1 Gene in Bone Marrow Stromal Cells from Patients with Myeloid Leukemia and Normal Individuals

    Institute of Scientific and Technical Information of China (English)

    SUYing; WANGZhen; WUXiuli; HUANGMeijuan; CHENShaohua; YANGLijian; LIYangqiu

    2005-01-01

    Objective: To investigate the expression of neuropilin-1 (NP-1) gene in bone marrow stromal cells (BMSCs) from myeloid leukemia (AML and CML) and normal individuals. Methods: Mononuclear cells were isolated from bone marrow (BM) of CML (14 cases), AML (12 cases) and normal individuals (20 cases). Adherent cells (i.e. BMSCs) were collected after long-term culture in vitro. The expression of NP-1 gene in three groups was detected respectively by reverse-transcription polymerase chain reaction (RT-PCR). Results: The long-term culture of BMSCs was successfully established. The expression level of NP-1 gene was significantly lower in BMSCs from AML (47.1%) and CML (50%) than in normal individuals (85%). Conclusion: NP-1 gene is expressed in BMSCs from some AML or CML patients and most normal individuals. The low-expression of NP-1 gene in BMSCs from AML or CML patients might be related with abnormality of regulation in hematopoiesis.

  1. Short-term myeloid growth factor mediated expansion of bone marrow haemopoiesis studied by localized magnetic resonance proton spectroscopy

    DEFF Research Database (Denmark)

    Jensen, K E; Hansen, P B; Larsen, V A;

    1994-01-01

    spectroscopy (MRS). Six patients were treated with daily subcutaneous injections of recombinant human granulocyte colony-stimulating factor (rhG-CSF, n = 2) or granulocyte-macrophage colony-stimulating factor (rhGM-CSF, n = 4) for 5d before marrow harvest. MRS investigations were performed prior to treatment....../76 x 10(3) (range 28.4-1180.6/23.2-2850.0). MRS detected a significant increase in bone marrow 'relative water content' day 12, 1 week after myeloid growth factor treatment was stopped, from median 30.5% (range 16-45) to 79% (range 56-93). In parallel, haemopoiesis was detected in new areas of femur....... In conclusion, the non-invasive MRS method may be a useful and reliable in vivo examination for expansion of haemopoiesis and a correspondent reduction of fat tissue in bone marrow after priming with recombinant human haemopoietic growth factors....

  2. Down-regulated E-cadherin expression is associated with poor five-year overall survival in bone and soft tissue sarcoma: results of a meta-analysis.

    Directory of Open Access Journals (Sweden)

    Ning Wang

    Full Text Available To conduct a meta-analysis to evaluate the prognostic role of E-cadherin expression in bone and soft tissue sarcomas.The PubMed, EMBASE, and Web of Science databases were searched using terms related to E-cadherin, sarcoma, and prognosis for all articles published in English before March 2014. Pooled effect was calculated from the available data to evaluate the association between negative E-cadherin expression and 5-year overall survival and tumor clinicopathological features in sarcoma patients. Pooled odds ratios (OR and risk ratios (RR with 95% confidence intervals (CI were calculated using a fixed-effects model.Eight studies met the selection criteria and reported on 812 subjects. A total of 496 subjects showed positive E-cadherin expression (59.9%. Negative E-cadherin expression in bone and soft tissue sarcomas was correlated with lower 5-year overall survival (OR = 3.831; 95% CI: 2.246-6.534, and was associated with higher clinical stage (RR = 1.446; 95% CI: 1.030-2.028 and with male sex (RR = 0.678; 95% CI: 0.493-0.933.In the E-cadherin negative group, 5-year overall survival was significantly worse than in the E-cadherin positive group. However, further studies are required to confirm these results.

  3. Association between the methylation status of the MGMT promoter in bone marrow specimens and chemotherapy outcomes of patients with acute myeloid leukemia

    OpenAIRE

    Hong, Qingxiao; Chen, Xiaoying; Ye, Huadan; ZHOU, ANNAN; GAO, YUTING; Jiang, Danjie; Wu, Xiaodong; TIAN, BINGRU; CHEN, YOUFEN; Wang, Ming; Xie, JiPing; XIA, YONGMING; Duan, Shiwei

    2016-01-01

    The O(6)-methylguanine-DNA methyltransferase (MGMT) gene is a tumor suppressor gene that is associated with the risk of developing acute myeloid leukemia (AML). However, the association between the methylation status of the MGMT promoter and the chemotherapeutic outcomes of patients with AML remains unknown. In the present study, 30 bone marrow samples derived from patients with AML were collected prior and subsequent to chemotherapy. The methylation status of the MGMT promoter in the bone ma...

  4. Exatecan in pretreated adult patients with advanced soft tissue sarcoma: results of a phase II--study of the EORTC Soft Tissue and Bone Sarcoma Group

    DEFF Research Database (Denmark)

    Reichardt, P; Nielsen, Ole Steen; Bauer, S;

    2007-01-01

    No standard treatment is established for patients with advanced soft tissue sarcoma after previous chemotherapy with anthracyclines and ifosfamide, given either in combination or sequentially. Exatecan (DX-8951f) is a totally synthetic analogue of the topoisomerase I-inhibitor camptothecin, which...... was synthesised to impart increased aqueous solubility, greater tumour efficacy, and less toxicity than camptothecin itself, topotecan or irinotecan. Since some activity against soft tissue sarcomas, especially leiomyosarcomas, has been reported for topoisomerase I-inhibitors, a study with a new and...... more potent agent seemed justified. We report on a prospective multicentre phase II study of Exatecan in adult soft tissue sarcomas failing 1 or 2 lines of chemotherapy in advanced phase, performed within the STBSG of EORTC. Thirty-nine patients (16 leiomyosarcomas and 23 other histologies) were...

  5. Epirubicin is not Superior to Doxorubicin in the Treatment of Advanced Soft Tissue Sarcomas.The Experience of the EORTC Soft Tissue and Bone Sarcoma Group

    DEFF Research Database (Denmark)

    Nielsen, Ole Steen; Dombernowsky, Per; Mouridsen, Henning T;

    2000-01-01

    studies the EORTC STBSG tested whether epirubicin (epi) is an alternative to standard dose dox in the treatment of chemonaive patients with advanced soft tissue sarcoma. The present report gives the final results of these studies.Patients/Methods. In the first study 210 patients were randomized to receive......Purpose. Doxorubicin (dox) still appears to be one of the most active drugs in the treatment of soft tissue sarcomas. However, treatment duration is limited due to cumulative cardiotoxicity. A number of small studies from single institutions have suggested activity of other analogues. In two......, epi is not superior to dox in the treatment of patients with advanced soft tissue sarcomas. In addition, the results illustrate that the data from small studies of single institutions should always be confirmed by large multi-institutional studies before being taken for granted....

  6. Vismodegib and Gamma-Secretase/Notch Signalling Pathway Inhibitor RO4929097 in Treating Patients With Advanced or Metastatic Sarcoma

    Science.gov (United States)

    2015-05-22

    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

  7. Genetic variants of human granzyme B predict transplant outcomes after HLA matched unrelated bone marrow transplantation for myeloid malignancies.

    Directory of Open Access Journals (Sweden)

    Luis J Espinoza

    Full Text Available Serine protease granzyme B plays important roles in infections, autoimmunity, transplant rejection, and antitumor immunity. A triple-mutated granzyme B variant that encodes three amino substitutions (Q48R, P88A, and Y245H has been reported to have altered biological functions. In the polymorphism rs8192917 (2364A>G, the A and G alleles represent wild type QPY and RAH mutant variants, respectively. In this study, we analyzed the impact of granzyme B polymorphisms on transplant outcomes in recipients undergoing unrelated HLA-fully matched T-cell-replete bone marrow transplantation (BMT through the Japan Donor Marrow Program. The granzyme B genotypes were retrospectively analyzed in a cohort of 613 pairs of recipients with hematological malignancies and their unrelated donors. In patients with myeloid malignancies consisting of acute myeloid leukemia and myelodysplastic syndrome, the donor G/G or A/G genotype was associated with improved overall survival (OS; adjusted hazard ratio [HR], 0.60; 95% confidence interval [CI], 0.41-0.89; P = 0.01 as well as transplant related mortality (TRM; adjusted HR, 0.48; 95% CI, 0.27-0.86, P = 0.01. The recipient G/G or A/G genotype was associated with a better OS (adjusted HR, 0.68; 95% CI, 0.47-0.99; P = 0.05 and a trend toward a reduced TRM (adjusted HR, 0.61; 95% CI, 0.35-1.06; P = 0.08. Granzyme B polymorphism did not have any effect on the transplant outcomes in patients with lymphoid malignancies consisting of acute lymphoid leukemia and malignant lymphoma. These data suggest that there is an association between the granzyme B genotype and better clinical outcomes in patients with myeloid malignancies after unrelated BMT.

  8. MR imaging of the bone marrow in myeloid leukemia and myelodysplastic syndrome. Comparison of the lumbar spine and femur

    International Nuclear Information System (INIS)

    MR imaging of the lumber spine and the femur was performed with T1-weighted SE sequence and comparison of the MRI findings of the lumber vertebral body and the femoral marrow was made in 15 patients with acute myeloid leukemia (AML), 5 chronic myelogenous leukemia (CML), and 9 myelodysplastic syndrome (MDS). The MRI appearance of the bone marrow was classified into four patterns: 1) fatty marrow; 2) faint signal; 3) heterogeneous infiltration; and 4) diffuse infiltration. The MRI of the lumber vertebral body showed a diffuse marrow infiltration pattern in over the half of the cases of AML and MDS. On the MRI of the femoral marrow, the signal intensity alteration, a low signal on T1-weighted SE image, began in the proximal femurs almost symmetrically. The abnormal low signal intensity area tended to gradually extend towards the distal portion of the femoral marrow with progression of the disease in the patients with AML and MDS. M2 type of AML tended to be demonstrated as a faint signal pattern, which was significantly different from the other types of AML. In all the cases of CML, a diffuse cellular infiltration pattern was noted with total replacement of the fatty marrow on both lumbar spinal and femoral MRI, and the femoral marrow involvement was more downwardly extended than AML. We concluded that MRI of the femoral marrow was more efficient than that of the lumbar spine in the assessment of myeloid leukemia and MDS. (author)

  9. Utility of opposed-phase magnetic resonance imaging in differentiating sarcoma from benign bone lesions

    Directory of Open Access Journals (Sweden)

    Barry E. Kenneally

    2015-12-01

    Conclusion: Opposed-phase imaging is helpful in differentiating benign from malignant lesions in bone. Confidence in diagnosis rose for both the attending and the resident as result of the inclusion of OP sequences.

  10. A reappraisal of hemangiopericytoma of bone; analysis of cases reclassified as synovial sarcoma and solitary fibrous tumor of bone

    DEFF Research Database (Denmark)

    Verbeke, Sofie L J; Fletcher, Christopher D M; Alberghini, Marco; Daugaard, Søren; Flanagan, Adrienne M; Parratt, Tim; Kroon, Herman M; Hogendoorn, Pancras C W; Bovée, Judith V M G

    2010-01-01

    HPC of bone exists. We reviewed 9 primary "HPC" of bone from 4 institutions diagnosed between 1952 and 2002. Immunohistochemistry was performed for CD31, CD34, von Willebrand factor, smooth muscle actin, keratin AE1/AE3, and epithelial membrane antigen. There were 4 male and 5 female patients between...... and varying cellularity, consistent with SFT; 3 of 5 cases examined were CD34-positive. Three tumors showed more densely packed sheets and fascicles of poorly differentiated cells, resembling SS, of which 2 showed focal staining for keratin AE1/AE3 or epithelial membrane antigen. Fluorescent in...

  11. Prognosis value of Hypoxia-inducible factor-1α expression in patients with bone and soft tissue sarcoma: a meta-analysis.

    Science.gov (United States)

    Li, Yongjiang; Zhang, Wenbiao; Li, Shuangjiang; Tu, Chongqi

    2016-01-01

    The prognostic significance of Hypoxia-inducible factor-1α (HIF-1α) in patients with bone and soft tissue sarcoma remains controversial. To investigate the impact of its expression on survival outcomes, we performed a meta-analysis. Comprehensive literature searches were conducted in PubMed, Web of Science, Embase and Cochrane Library. A total of 16 studies published from 2006 to 2015 were included. We found that expression of HIF-1α was significantly associated with higher rate of metastasis (RR 3.21, 95 % CI 2.12-4.84, P < 0.001), poorer overall survival (HR 2.05, 95 % CI 1.51-2.77, P < 0.001) and poorer disease-free survival (HR 2.05, 95 % CI 1.55-2.70, P < 0.001). In addition, when subgroup analysis was conducted according to histology type, the significant correlations to poor overall survival and disease-free survival were also observed in patients with osteosarcoma, chondrosarcoma and soft tissue sarcoma. Publication bias was not found and sensitivity analysis showed the results were stable. In conclusion, HIF-1α expression might be an effective predicative factor of poor prognosis for bone and soft tissue sarcoma. PMID:27606158

  12. Myeloid regeneration after whole body irradiation, autologous bone marrow transplantation, and treatment with an anabolic steroid.

    Science.gov (United States)

    Ambrus, C M; Ambrus, J L

    1975-01-01

    Stumptail monkeys (Macaca speciosa) received lethal whole body radiation. Autologous bone marrow injection resulted in survival of the majority of the animals. Treatment with Deca-Durabolin, an anabolic steroid, caused more rapid recovery of colony-forming cell numbers in the bone marrow than in control animals. Both the Deca-Durabolin-treated and control groups were given autologous bone marrow transplantation. Anabolic steroid effect on transplanted bone marrow colonyforming cells may explain the increased rate of leukopoietic regeneration in anabolic steroid-treated animals as compared to controls. PMID:124758

  13. Myeloid regeneration after whole body irradiation, autologous bone marrow transplantation, and treatment with an anabolic steroid

    International Nuclear Information System (INIS)

    Stumptail monkeys (Macaca speciosa) received lethal whole-body radiation. Autologous bone marrow injection resulted in survival of the majority of the animals. Treatment with Deca-Durabolin, an anabolic steroid, caused more rapid recovery of colony-forming cell numbers in the bone marrow than in control animals. Both the Deca-Durabolin-treated and control groups were given autologous bone marrow transplantation. Anabolic steroid effect on transplanted bone marrow colony-forming cells may explain the increased rate of leukopoietic regeneration in anabolic steroid-treated animals as compared to controls

  14. Extensive Bone Marrow Necrosis and Osteolytic Lesions in a Case of Acute Myeloid Leukemia Transformed from Polycythemia Vera.

    Science.gov (United States)

    Chambers, Isaac; Truong, Phu; Kallail, K James; Palko, William

    2016-01-01

    Acute myeloid leukemia (AML) is the most common leukemia in adults. In rare cases, bone marrow necrosis (BMN) and osteolytic lesions are presenting features of AML. The following case describes a patient with known polycythemia vera (PV) that presented with signs of multiple myeloma, including hypercalcemia, anemia, and lytic lesions of the thoracic spine and skull. Laboratory workup was not indicative of myeloma. A bone marrow biopsy was performed, which revealed extensive BMN and initial pathology was consistent with metastatic carcinoma. However, no immunohistochemical stains could be performed due to the extent of BMN; a repeat biopsy was therefore performed. Flow cytometry and CD45 staining were consistent with PV that had transformed to AML. Due to the patient's comorbidities, she was a poor candidate for stem cell transplant and did not wish to pursue chemotherapy. Ultimately, she pursued hospice care. Based on our literature review, both BMN and osteolytic lesions are rare manifestations of AML and have not been reported to occur simultaneously. These findings can lead to a diagnostic dilemma and suspicion of other malignancies. This case demonstrates that AML should remain in the differential diagnosis in those patients who present with BMN and osteolytic lesions. PMID:27433418

  15. Isolated granulocytic sarcoma of the nasopharynx: a case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Vishnu P

    2013-12-01

    Full Text Available Prakash Vishnu,1 Ravindra Reddy Chuda,2 Dick G Hwang,3 David M Aboulafia1,4 1Floyd and Delores Jones Cancer Institute at Virginia Mason Medical Center, Seattle, WA, USA; 2Department of Internal Medicine, University of Kansas Medical Center, Kansas City, KS, USA; 3Department of Pathology, Virginia Mason Medical Center, 4Division of Hematology, University of Washington, Seattle, WA, USA Abstract: Granulocytic sarcoma (GS is a rare extramedullary manifestation of acute myeloid leukemia (AML. It may also represent blastic transformation of myelodysplastic syndromes or myeloproliferative neoplasms. Although usually seen in the context of advanced and poorly controlled disease, it may also present as the first manifestation of illness, without concurrent bone marrow or blood involvement. In the medical literature, chloroma and GS are terms that have been used interchangeably with myeloid sarcoma. GS usually manifests as soft tissue or bony masses in several extracranial sites, such as bone, periosteum, and lymph nodes; involvement of the head and neck region is uncommon. We report a case of a woman with insidious onset of progressive nasal congestion and diminished hearing who was diagnosed with an isolated GS of the nasopharynx. With involved field radiotherapy, she achieved a complete remission of 12-months duration before being diagnosed with overt AML. She has remained disease-free for greater than 18 months following induction and consolidation chemotherapy. Through a MEDLINE®/PubMed® search we identified an additional 13 cases of nasopharyngeal GS. The median age was 37 years (range 1 to 81 years. The cases were equally distributed among the sexes. The most common presenting symptoms were conductive hearing loss and sinonasal congestion. Isolated GS was identified in six cases, and the median time from diagnosis of GS to AML was 12 months (range 3 to 48 months. The treatment varied, but responses were seen in all the patients who received

  16. Evaluation of the Therapeutic Potential of Bone Marrow-Derived Myeloid Suppressor Cell (MDSC) Adoptive Transfer in Mouse Models of Autoimmunity and Allograft Rejection

    OpenAIRE

    Drujont, Lucile; Carretero-Iglesia, Laura; Bouchet-Delbos, Laurence; Beriou, Gaelle; Merieau, Emmanuel; Hill, Marcelo; Delneste, Yves; Cuturi, Maria Cristina; Louvet, Cedric

    2014-01-01

    Therapeutic use of immunoregulatory cells represents a promising approach for the treatment of uncontrolled immunity. During the last decade, myeloid-derived suppressor cells (MDSC) have emerged as novel key regulatory players in the context of tumor growth, inflammation, transplantation or autoimmunity. Recently, MDSC have been successfully generated in vitro from naive mouse bone marrow cells or healthy human PBMCs using minimal cytokine combinations. In this study, we aimed to evaluate the...

  17. High-Dose Chemotherapy and Autologous Stem Cell Transplantation in Children with High-Risk or Recurrent Bone and Soft Tissue Sarcomas

    Science.gov (United States)

    2016-01-01

    Despite increasing evidence that high-dose chemotherapy and autologous stem cell transplantation (HDCT/auto-SCT) might improve the survival of patients with high-risk or recurrent solid tumors, therapy effectiveness for bone and soft tissue sarcoma treatment remains unclear. This study retrospectively investigated the feasibility and effectiveness of HDCT/auto-SCT for high-risk or recurrent bone and soft tissue sarcoma. A total of 28 patients (18 high-risk and 10 recurrent) underwent single or tandem HDCT/auto-SCT between October 2004 and September 2014. During follow-up of a median 15.3 months, 18 patients exhibited disease progression and 2 died of treatment-related toxicities (1 veno-occlusive disease and 1 sepsis). Overall, 8 patients remained alive and progression-free. The 3-year overall survival (OS) and event-free survival (EFS) rates for all 28 patients were 28.7% and 26.3%, respectively. In the subgroup analysis, OS and EFS rates were higher in patients with complete or partial remission prior to HDCT/auto-SCT than in those with worse responses (OS, 39.1% vs. 0.0%, P = 0.002; EFS, 36.8% vs. 0.0%, P < 0.001). Therefore, careful selection of patients who can benefit from HDCT/auto-SCT and maximal effort to reduce tumor burden prior to treatment will be important to achieve favorable outcomes in patients with high-risk or recurrent bone and soft tissue sarcomas. PMID:27366002

  18. Systemic expression of Kaposi sarcoma herpesvirus (KSHV) Vflip in endothelial cells leads to a profound proinflammatory phenotype and myeloid lineage remodeling in vivo.

    OpenAIRE

    Gianna Ballon; Gunkut Akar; Ethel Cesarman

    2015-01-01

    Author Summary Kaposi’s sarcoma (KS) is the most common cancer in men infected with HIV, and also among the most frequent malignancies in Sub-Equatorial Africa. KS is a tumor of endothelial cell origin that is caused by infection with a gamma-herpesvirus, called KS herpesvirus (KSHV) or human herpesvirus 8 (HHV-8). KSHV vFLIP is a viral oncoprotein expressed during latent infection. We report here the generation and characterization of mice expressing KSHV vFLIP in an inducible manner in endo...

  19. Kaposi sarcoma

    Science.gov (United States)

    ... HIV, a weakened immune system, and the human herpesvirus-8 (HHV-8). Kaposi sarcoma has been linked ... on foot References Kaye KM. Kaposi's sarcoma-associated herpesvirus (human herpesvirus type 8). In: Mandell GL, Bennett ...

  20. Changing bone marrow micro-environment during development of acute myeloid leukaemia in rats

    DEFF Research Database (Denmark)

    Mortensen, B T; Jensen, P O; Helledie, N;

    1998-01-01

    cells (from about 45% to 25%), evidently as a result of the severely changed microenvironment. In this study we have demonstrated in vivo the development of an acidic and hypoxic bone marrow hampering normal haemopoiesis during leukaemic growth. Our data support the notion of BNML as a valuable tool for...

  1. Bone marrow niche-mediated survival of leukemia stem cells in acute myeloid leukemia: Yin and Yang

    Science.gov (United States)

    Zhou, Hong-Sheng; Carter, Bing Z.; Andreeff, Michael

    2016-01-01

    Acute myeloid leukemia (AML) is characterized by the accumulation of circulating immature blasts that exhibit uncontrolled growth, lack the ability to undergo normal differentiation, and have decreased sensitivity to apoptosis. Accumulating evidence shows the bone marrow (BM) niche is critical to the maintenance and retention of hematopoietic stem cells (HSC), including leukemia stem cells (LSC), and an increasing number of studies have demonstrated that crosstalk between LSC and the stromal cells associated with this niche greatly influences leukemia initiation, progression, and response to therapy. Undeniably, stromal cells in the BM niche provide a sanctuary in which LSC can acquire a drug-resistant phenotype and thereby evade chemotherapy-induced death. Yin and Yang, the ancient Chinese philosophical concept, vividly portrays the intricate and dynamic interactions between LSC and the BM niche. In fact, LSC-induced microenvironmental reprogramming contributes significantly to leukemogenesis. Thus, identifying the critical signaling pathways involved in these interactions will contribute to target optimization and combinatorial drug treatment strategies to overcome acquired drug resistance and prevent relapse following therapy. In this review, we describe some of the critical signaling pathways mediating BM niche-LSC interaction, including SDF1/CXCL12, Wnt/β-catenin, VCAM/VLA-4/NF-κB, CD44, and hypoxia as a newly-recognized physical determinant of resistance, and outline therapeutic strategies for overcoming these resistance factors.

  2. Patient-derived acute myeloid leukemia (AML) bone marrow cells display distinct intracellular kinase phosphorylation patterns

    International Nuclear Information System (INIS)

    Multiparametric analyses of phospho-protein activation in patients with acute myeloid leukemia (AML) offers a quantitative measure to monitor the activity of novel intracellular kinase (IK) inhibitors. As recent clinical investigation with FMS-like tyrosine-3 inhibitors demonstrated, targeting IK with selective inhibitors can have a modest clinical benefit. Because multiple IKs are active in patients with AML, multikinase inhibitors may provide the necessary inhibition profile to achieve a more sustained clinical benefit. We here describe a method of assessing the activation of several IKs by flow cytometry. In 40 different samples of patients with AML we observed hyper-activated phospho-proteins at baseline, which is modestly increased by adding stem cell factor to AML cells. Finally, AML cells had a significantly different phospho-protein profile compared with cells of the lymphocyte gate. In conclusion, our method offers a way to determine the activation status of multiple kinases in AML and hence is a reliable assay to evaluate the pharmacodynamic activity of novel multikinase inhibitors

  3. Bone scintigraphy in acute myeloid leukemia patient with fungal vertebral osteomyelitis

    Directory of Open Access Journals (Sweden)

    Zekiye Hasbek

    2013-09-01

    Full Text Available The result of the magnetic resonance imaging (MRI study was reported as “metastasis of primary disease on L2-L3 vertebrae” in a 63-year-old male patient, who developed a back pain after receiving four courses of treatment for AML. The patient, who did not respond to pain medication, was sent to nuclear medicine department for a bone scintigraphy. Diffuse increased osteoblastic activity was reported on L2-L3 vertebrae with a suspicion about infection or fracture, together with a focal osteoblastic activity involvement in the right sacroiliac joint in the bone scintigraphy which was made with Tc99m-MDP. In the mean time, the patient complained about progressive loss of strength on bilateral lower extremities and numbness in legs. Repeated MRI was reported as “irregularities in L2-L3 vertebral disc region concordant with infection, prominent thecal pressure, loss of height in L2-L3 vertebrae associated with osteomyelitis and a mass concordant with paravertebral abscess and granulation tissue”. The patient was operated and necrotic tissue was removed by curettage, relieving the compression on L2-L3 and on the disc distance. In culture examination of the sample “candida albicans” was isolated. Antifungal treatment with Amphotericin B was started. Patient's pain was reduced and MRI findings showed some regression in abscess following the treatment. There was improvement in neurological examination. However, relapse in AML was observed in bone marrow aspiration, performed during follow-up and chemotherapy was started again. On the second day of chemotherapy high fever started and cellulitis developed on the right leg. The patient received hemodialysis treatment due to increase in BUN and creatinine levels. Pulmonary edema and associated respiratory insufficiency was developed and the patient died. Fungal infections are one of the most important clinical problems in leukemia patients. However, vertebral osteomyelitis secondary to fungal

  4. Suppression of proteoglycan-induced autoimmune arthritis by myeloid-derived suppressor cells generated in vitro from murine bone marrow.

    Directory of Open Access Journals (Sweden)

    Júlia Kurkó

    Full Text Available Myeloid-derived suppressor cells (MDSCs are innate immune cells capable of suppressing T-cell responses. We previously reported the presence of MDSCs with a granulocytic phenotype in the synovial fluid (SF of mice with proteoglycan (PG-induced arthritis (PGIA, a T cell-dependent autoimmune model of rheumatoid arthritis (RA. However, the limited amount of SF-MDSCs precluded investigations into their therapeutic potential. The goals of this study were to develop an in vitro method for generating MDSCs similar to those found in SF and to reveal the therapeutic effect of such cells in PGIA.Murine bone marrow (BM cells were cultured for 3 days in the presence of granulocyte macrophage colony-stimulating factor (GM-CSF, interleukin-6 (IL-6, and granulocyte colony-stimulating factor (G-CSF. The phenotype of cultured cells was analyzed using flow cytometry, microscopy, and biochemical methods. The suppressor activity of BM-MDSCs was tested upon co-culture with activated T cells. To investigate the therapeutic potential of BM-MDSCs, the cells were injected into SCID mice at the early stage of adoptively transferred PGIA, and their effects on the clinical course of arthritis and PG-specific immune responses were determined.BM cells cultured in the presence of GM-CSF, IL-6, and G-CSF became enriched in MDSC-like cells that showed greater phenotypic heterogeneity than MDSCs present in SF. BM-MDSCs profoundly inhibited both antigen-specific and polyclonal T-cell proliferation primarily via production of nitric oxide. Injection of BM-MDSCs into mice with PGIA ameliorated arthritis and reduced PG-specific T-cell responses and serum antibody levels.Our in vitro enrichment strategy provides a SF-like, but controlled microenvironment for converting BM myeloid precursors into MDSCs that potently suppress both T-cell responses and the progression of arthritis in a mouse model of RA. Our results also suggest that enrichment of BM in MDSCs could improve the

  5. Transformation of bone marrow stem-cells and radiation-induced myeloid leukemia in mice

    International Nuclear Information System (INIS)

    After a single whole-body X-irradiation of 300R to male RFM/MsNrs strain mice, the occurrence of myeloid leukemia initiated since four months and ceased at eleven months after irradiation. The cumulative incidence reached 24.5%. A time course study on the kinetics of pluripotential stem-cells (CFU-S) and granuloid committed stem-cells (CFU-C) in the marrow after 300R was also performed. The repopulation of CFU-S was accomplished within one month whereas that of CFU-C needed 210 days after irradiation. The incidence of leukemia was very rare after the complete repopulation of CFU-C. Simultaneously, collected spleen cells from the irradiated mice without overt leukemia were transplanted into 300-600R irradiated recipients of another sex. Three months thereafter, recipients were sacrificed to detect leukemic changes and the origin of leukemic cells by chromosome analysis. The results revealed that leukemic cell transformation of donor cells began 18 days after irradiation and on an average, 37.1% of the irradiated mice carried potentially leukemic cells for seven months after exposure, whereas none of the unirradiated mice carried leukemic cells at 7 months after irradiation. To investigate host factor(s) contributing to the proliferation of leukemic cells, the suppression of cellular immunity after 300R was measured by GVH mortality assay. However, the recovery of cellular immunity was observed until three months after irradiation and the role of cellular immunity to proliferation of leukemic cells after three months was negligible. (author)

  6. Reconstitution of the myeloid and lymphoid compartments after the transplantation of autologous and genetically modified CD34(+) bone marrow cells, following gamma irradiation in cynomolgus macaques

    International Nuclear Information System (INIS)

    Prolonged, altered hematopoietic reconstitution is commonly observed in patients undergoing myelo-ablative conditioning and bone marrow and/or mobilized peripheral blood-derived stem cell transplantation. We studied the reconstitution of myeloid and lymphoid compartments after the transplantation of autologous CD34+ bone marrow cells following gamma irradiation in cynomolgus macaques. The bone marrow cells were first transduced ex vivo with a lentiviral vector encoding eGFP, with a mean efficiency of 72% ± 4%. The vector used was derived from the simian immunodeficiency lentivirus SIVmac251, VSV-g pseudo-typed and encoded eGFP under the control of the phosphoglycerate kinase promoter. After myeloid differentiation, GFP was detected in colony-forming cells (37% ± 10%). A previous study showed that transduction rates did not differ significantly between colony-forming cells and immature cells capable of initiating long-term cultures, indicating that progenitor cells and highly immature hematopoietic cells were transduced with similar efficiency. Blood cells producing eGFP were detected as early as three days after transplantation,and eGFP-producing granulocyte and mononuclear cells persisted for more than one year in the periphery. Conclusion: The transplantation of CD34+ bone marrow cells had beneficial effects for the ex vivo proliferation and differentiation of hematopoietic progenitors, favoring reconstitution of the T-and B-lymphocyte, thrombocyte and red blood cell compartments. (authors)

  7. t(4;11 (q21;q23 in acute myeloid leukemia-M0 following treatment [EW92 Protocol] for Ewing's sarcoma Leucemia mielóide aguda-M0 com t(4;11 (q21;q23 após tratamento para sarcoma de Ewing com o protocolo EW92

    Directory of Open Access Journals (Sweden)

    Terezinha J. Marques Salles

    2004-01-01

    Full Text Available We report on a 7-year-old girl with Ewing's Sarcoma (ES who developed a poorly differentiated acute myeloid leukemia (AML-M0 20 months after beginning the EW92 protocol for the treatment of the primary tumor. She received a total dose of 1500 mg of etoposide, a tumor cumulative radiation dose of 35Gy and granulocyte colony-stimulating factor (G-CSF was as predicted in the protocol regimen. At onset of secondary malignancy her laboratorial analysis revealed immature blast cells characterized by CD34+/CD33-/a-MPO+ and a t(4;11(q21;q23 abnormality. This serious complication of ES treatment, which associates etoposide, irradiation and G-CSF schedule, should be weighed against its therapeutic benefits.Nós descrevemos o caso clínico de uma criança do sexo feminino, com 7 anos de idade, portadora de sarcoma de Ewing, que evoluiu com leucemia aguda mielóide pouco diferenciada (LMA-M0 após vinte meses de tratamento utilizando o protocolo EW92. Ela recebeu uma dose total de 1.500 mg de etoposídio, irradiação tumoral na dose total de 35G, e fator de estimulação de colônia granulocítica (G-CSF conforme programação do protocolo terapêutico. Os exames laboratoriais, por ocasião do diagnóstico da segunda malignidade, mostraram células blásticas imaturas caracterizadas pela expressão de CD34+/CD33-/aMPO+ e a translocação t(4;11 (q 21;q23. A exclusão do G-CSF nos esquemas terapêuticos que associam etoposídio e irradiação tumoral se justifica devido a esta séria complicação no tratamento do sarcoma de Ewing.

  8. Decitabine and Total-Body Irradiation Followed By Donor Bone Marrow Transplant and Cyclophosphamide in Treating Patients With Relapsed or Refractory Acute Myeloid Leukemia

    Science.gov (United States)

    2016-07-08

    Acute Myeloid Leukemia With Multilineage Dysplasia Following Myelodysplastic Syndrome; Adult Acute Myeloid Leukemia in Remission; Adult Acute Myeloid Leukemia With 11q23 (MLL) Abnormalities; Adult Acute Myeloid Leukemia With Del(5q); Adult Acute Myeloid Leukemia With Inv(16)(p13;q22); Adult Acute Myeloid Leukemia With t(15;17)(q22;q12); Adult Acute Myeloid Leukemia With t(16;16)(p13;q22); Adult Acute Myeloid Leukemia With t(8;21)(q22;q22); de Novo Myelodysplastic Syndromes; Previously Treated Myelodysplastic Syndromes; Recurrent Adult Acute Myeloid Leukemia; Secondary Acute Myeloid Leukemia

  9. 18F-FLT Positron Emission Tomography and Diffusion-Weighted Magnetic Resonance Imaging in Planning Surgery and Radiation Therapy and Measuring Response in Patients With Newly Diagnosed Ewing Sarcoma

    Science.gov (United States)

    2016-03-15

    Adult Supratentorial Primitive Neuroectodermal Tumor (PNET); Ewing Sarcoma of Bone; Extraosseous Ewing Sarcoma; Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Untreated Childhood Supratentorial Primitive Neuroectodermal Tumor

  10. Granulocytic sarcoma of the femur in a patient with acute megakaryoblastic leukaemia

    Directory of Open Access Journals (Sweden)

    Čolović Milica

    2011-01-01

    Full Text Available Introduction. Granulocytic sarcoma, chloroma or myeloblastoma are observed in 3% to7% of acute myeloid leukaemia and represents localized tumour composed of collection of immature leukaemic cells. It appears most frequently in patients with M2, M4 and M5 subtypes of acute myeloid leukaemia Case Outline. A 58-year-old female presented with pain and oedema of the right upper limb in November 2009. After two months the patinet had fracture dislocation and numerous osteolytic lesions of the right femur. Immunohistochemistry of tumour biopsy showed megakaryoblastic granulocytic sarcoma which was CD31++, F-XIII++, CD34-, FVIII+++, S100-, aktin-, EMA++, Bcl2++, CD43++, with positive proliferative marker measured with Ki-67 positivity in more of 50% of cells. Aspirate of bone marrow and immunophenotyping with flowcytometry revealed diagnosis of acute megakaryoblastic leukaemia. The course of the disease was rapid and the patient died before commencing chemotherapy, five months after first complaints. Conclusion. Granulocytic sarcoma is extramedullary localization of collection of leukaemia cells which can proceed, to arise concomitantly with leukaemia, or may be the only manifestation of the disease. The diagnosis can be established only with immunohystochemistry.

  11. 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

  12. MRI of perineural extramedullary granulocytic sarcoma

    International Nuclear Information System (INIS)

    Granulocytic sarcoma is an extramedullary solid tumour consisting of myelogenous leukaemic blast cells, usually seen in acute myeloid leukaemia and less commonly in patients with chronic myeloid leukaemia or myeloproliferative disorders. Blast cells have a predilection for periosteal and perineural regions and rarely precede evidence of systemic disease. We present two patients, aleukaemic on peripheral blood counts, both at presentation and during subsequent treatment. We present the MRI features of this rare but important condition. (orig.)

  13. MRI of perineural extramedullary granulocytic sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Graham, A. [Rehabilitation Medicine, Hunters Moor Neurological Rehabilitation Centre, Newcastle-Upon-Tyne (United Kingdom); Hodgson, T. [Neuroradiology Dept., Royal Hallamshire Hospital, Sheffield (United Kingdom); Jacubowski, J. [Neurosurgical Dept., Royal Hallamshire Hospital, Sheffield (United Kingdom); Norfolk, D. [Haematology Department, Leeds General Infirmary, Leeds LS1 3EX (United Kingdom); Smith, C. [Pathology Dept., Royal Hallamshire Hospital, Sheffield (United Kingdom)

    2001-06-01

    Granulocytic sarcoma is an extramedullary solid tumour consisting of myelogenous leukaemic blast cells, usually seen in acute myeloid leukaemia and less commonly in patients with chronic myeloid leukaemia or myeloproliferative disorders. Blast cells have a predilection for periosteal and perineural regions and rarely precede evidence of systemic disease. We present two patients, aleukaemic on peripheral blood counts, both at presentation and during subsequent treatment. We present the MRI features of this rare but important condition. (orig.)

  14. Carbon Ion Radiation Therapy Improves the Prognosis of Unresectable Adult Bone and Soft-Tissue Sarcoma of the Head and Neck

    Energy Technology Data Exchange (ETDEWEB)

    Jingu, Keiichi [Research Center for Charged Particle Therapy, National Institute of Radiological Sciences (NIRS), Chiba (Japan); Department of Radiation Oncology, Tohoku University School of Medicine, Sendai (Japan); Tsujii, Hirohiko, E-mail: tsujii@nirs.go.jp [Research Center for Charged Particle Therapy, National Institute of Radiological Sciences (NIRS), Chiba (Japan); Mizoe, Jun-Etsu; Hasegawa, Azusa; Bessho, Hiroki; Takagi, Ryo; Morikawa, Takamichi [Research Center for Charged Particle Therapy, National Institute of Radiological Sciences (NIRS), Chiba (Japan); Tonogi, Morio [Department of Oral Medicine, Tokyo Dental College, Ichihara (Japan); Tsuji, Hiroshi; Kamada, Tadashi [Research Center for Charged Particle Therapy, National Institute of Radiological Sciences (NIRS), Chiba (Japan); Yamada, Shogo [Department of Radiation Oncology, Tohoku University School of Medicine, Sendai (Japan)

    2012-04-01

    Purpose: To evaluate the safety and efficacy of carbon ion radiotherapy (C-ion RT) with 70.4 GyE for unresectable bone and soft-tissue sarcoma of the adult head and neck. Methods and Materials: Twenty-seven patients (mean age, 46.2 years) were enrolled in this prospective study on C-ion RT with 70.4 GyE/16 fractions (fr) between April 2001 and February 2008. The primary end points were acute and late reactions of normal tissues, local control rate, and overall survival rate. The secondary end point was efficacy of the treatment in comparison to historical results with 57.6 or 64.0 GyE/16 fr. Results: The 3-year local control rate and overall survival rate for all patients were 91.8% (95% confidence interval [CI] = 81.0-100%) and 74.1% (95% CI = 57.5-90.6%), respectively. Acute reaction of Grade 3 or more was observed in only 1 patient. With regard to late reactions, visual loss was observed in 1 patient and a Grade 3 reaction of the maxillary bone was observed in 4 patients. A comparison with historical results revealed that the local control rate with 70.4 GyE was significantly higher than that with 57.6 or 64.0 GyE (3-year, 91.8% vs. 23.6%, p < 0.0001). Furthermore, the overall survival with 70.4 GyE tended to be higher than that with 57.6 or 64.0 GyE (3-year, 74.1% vs. 42.9%, p = 0.09). Conclusion: C-ion RT with 70.4 GyE/16 fr for bone and soft-tissue sarcoma of the adult head and neck appears to be effective with acceptable toxicities in comparison to conventional RT and C-ion RT with lower doses.

  15. 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 (

  16. Acute myeloid leukemia masquerading as hepatocellular carcinoma.

    Science.gov (United States)

    Abu-Zeinah, Ghaith F; Weisman, Paul; Ganesh, Karuna; Katz, Seth S; Dogan, Ahmet; Abou-Alfa, Ghassan K; Stein, Eytan M; Jarnagin, William; Mauro, Michael J; Harding, James J

    2016-06-01

    Hepatocellular carcinoma (HCC) is often diagnosed on the basis of high quality imaging without a biopsy in the cirrhotic liver. This is a case of a 64-year-old Caucasian man with no history of liver disease or cirrhosis that presented with fatigue, weight loss, and abdominal distension and was found to have a large, isolated liver mass with arterial enhancement and portal venous washout on triple-phase computed tomography (CT) suspicious for HCC. The patient was initially referred for a surgical evaluation. Meanwhile, he developed fevers, pancytopenia, and worsening back pain, and a subsequent spinal MRI revealed a heterogeneous bone marrow signal suspicious for metastatic disease. A bone marrow biopsy that followed was diffusely necrotic. A core biopsy of the patient's liver mass was then performed and was diagnostic of acute monocytic-monoblastic leukemia. Findings from peripheral flow cytometry and a repeat bone marrow biopsy were also consistent with this diagnosis, and induction chemotherapy with cytarabine and idarubicin was initiated. This case describes a rare presentation of myeloid sarcoma (MS) as an isolated, hypervascular liver mass that mimics HCC in its radiographic appearance. Due to the broad differential for a liver mass, a confirmatory biopsy should routinely be considered prior to surgical intervention. PMID:27284485

  17. Acute myeloid leukemia masquerading as hepatocellular carcinoma

    Science.gov (United States)

    Abu-Zeinah, Ghaith F.; Weisman, Paul; Ganesh, Karuna; Katz, Seth S.; Dogan, Ahmet; Abou-Alfa, Ghassan K.; Stein, Eytan M.; Jarnagin, William; Mauro, Michael J.

    2016-01-01

    Hepatocellular carcinoma (HCC) is often diagnosed on the basis of high quality imaging without a biopsy in the cirrhotic liver. This is a case of a 64-year-old Caucasian man with no history of liver disease or cirrhosis that presented with fatigue, weight loss, and abdominal distension and was found to have a large, isolated liver mass with arterial enhancement and portal venous washout on triple-phase computed tomography (CT) suspicious for HCC. The patient was initially referred for a surgical evaluation. Meanwhile, he developed fevers, pancytopenia, and worsening back pain, and a subsequent spinal MRI revealed a heterogeneous bone marrow signal suspicious for metastatic disease. A bone marrow biopsy that followed was diffusely necrotic. A core biopsy of the patient’s liver mass was then performed and was diagnostic of acute monocytic-monoblastic leukemia. Findings from peripheral flow cytometry and a repeat bone marrow biopsy were also consistent with this diagnosis, and induction chemotherapy with cytarabine and idarubicin was initiated. This case describes a rare presentation of myeloid sarcoma (MS) as an isolated, hypervascular liver mass that mimics HCC in its radiographic appearance. Due to the broad differential for a liver mass, a confirmatory biopsy should routinely be considered prior to surgical intervention. PMID:27284485

  18. What Is Chronic Myeloid Leukemia?

    Science.gov (United States)

    ... leukemia? Next Topic Normal bone marrow and blood What is chronic myeloid leukemia? Cancer starts when cells ... their treatment is the same as for adults. What is leukemia? Leukemia is a cancer that starts ...

  19. Therapeutic opportunities in Ewing sarcoma: EWS-FLI inhibition via LSD1 targeting.

    Science.gov (United States)

    Theisen, Emily R; Pishas, Kathleen I; Saund, Ranajeet S; Lessnick, Stephen L

    2016-04-01

    Ewing sarcoma is an aggressive primary pediatric bone tumor, often diagnosed in adolescents and young adults. A pathognomonic reciprocal chromosomal translocation results in a fusion gene coding for a protein which derives its N-terminus from a FUS/EWS/TAF15 (FET) protein family member, commonly EWS, and C-terminus containing the DNA-binding domain of an ETS transcription factor, commonly FLI1. Nearly 85% of cases express the EWS-FLI protein which functions as a transcription factor and drives oncogenesis. As the primary genomic lesion and a protein which is not expressed in normal cells, disrupting EWS-FLI function is an attractive therapeutic strategy for Ewing sarcoma. However, transcription factors are notoriously difficult targets for the development of small molecules. Improved understanding of the oncogenic mechanisms employed by EWS-FLI to hijack normal cellular programming has uncovered potential novel approaches to pharmacologically block EWS-FLI function. In this review we examine targeting the chromatin regulatory enzymes recruited to conspire in oncogenesis with a focus on the histone lysine specific demethylase 1 (LSD1). LSD1 inhibitors are being aggressively investigated in acute myeloid leukemia and the results of early clinical trials will help inform the future use of LSD1 inhibitors in sarcoma. High LSD1 expression is observed in Ewing sarcoma patient samples and mechanistic and preclinical data suggest LSD1 inhibition globally disrupts the function of EWS-ETS proteins. PMID:26848860

  20. Targeting BCL2 Family in Human Myeloid Dendritic Cells: A Challenge to Cure Diseases with Chronic Inflammations Associated with Bone Loss

    Directory of Open Access Journals (Sweden)

    Selma Olsson Åkefeldt

    2013-01-01

    Full Text Available Rheumatoid arthritis (RA and Langerhans cell histiocytosis (LCH are common and rare diseases, respectively. They associate myeloid cell recruitment and survival in inflammatory conditions with tissue destruction and bone resorption. Manipulating dendritic cell (DC, and, especially, regulating their half-life and fusion, is a challenge. Indeed, these myeloid cells display pathogenic roles in both diseases and may be an important source of precursors for differentiation of osteoclasts, the bone-resorbing multinucleated giant cells. We have recently documented that the proinflammatory cytokine IL-17A regulates long-term survival of DC by inducing BCL2A1 expression, in addition to the constitutive MCL1 expression. We summarize bibliography of the BCL2 family members and their therapeutic targeting, with a special emphasis on MCL1 and BCL2A1, discussing their potential impact on RA and LCH. Our recent knowledge in the survival pathway, which is activated to perform DC fusion in the presence of IL-17A, suggests that targeting MCL1 and BCL2A1 in infiltrating DC may affect the clinical outcomes in RA and LCH. The development of new therapies, interfering with MCL1 and BCL2A1 expression, to target long-term surviving inflammatory DC should be translated into preclinical studies with the aim to increase the well-being of patients with RA and LCH.

  1. 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.

  2. Imaging characteristics of primary cranial Ewing sarcoma

    International Nuclear Information System (INIS)

    Ewing sarcoma accounts for 10-15% of all childhood malignant bone tumours and is second in prevalence to osteosarcoma. The skull bones are an unusual site of origin of primary Ewing sarcoma in children. Previous reports concentrate on the neurosurgical aspects and relatively good outcome compared to other bone tumours of the calvarium. Reported cases mainly describe the imaging characteristics on CT. To describe the MRI and CT features of primary cranial Ewing sarcoma. The neuroimaging of three cases of primary cranial Ewing sarcoma are reviewed. Our three cases show an extra-axial mass that is high attenuation on CT and low signal on T2-weighted MRI. Haemorrhagic components, dural extension and contrast enhancement are also characteristic features. CT attenuation and magnetic resonance signal characteristics reflect sheets of densely packed cells seen in Ewing sarcoma. (orig.)

  3. 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

  4. Immunotherapy of Childhood Sarcomas

    OpenAIRE

    Roberts, Stephen S.; Chou, Alexander J.; Cheung, Nai-Kong V.

    2015-01-01

    Pediatric sarcomas are a heterogeneous group of malignant tumors of bone and soft tissue origin. Although more than 100 different histologic subtypes have been described, the majority of pediatric cases belong to the Ewing’s family of tumors, rhabdomyosarcoma and osteosarcoma. Most patients that present with localized stage are curable with surgery and/or chemotherapy; however, those with metastatic disease at diagnosis or those who experience a relapse continue to have a very poor prognosis....

  5. Carbon Ion Radiation Therapy Improves the Prognosis of Unresectable Adult Bone and Soft-Tissue Sarcoma of the Head and Neck

    International Nuclear Information System (INIS)

    Purpose: To evaluate the safety and efficacy of carbon ion radiotherapy (C-ion RT) with 70.4 GyE for unresectable bone and soft-tissue sarcoma of the adult head and neck. Methods and Materials: Twenty-seven patients (mean age, 46.2 years) were enrolled in this prospective study on C-ion RT with 70.4 GyE/16 fractions (fr) between April 2001 and February 2008. The primary end points were acute and late reactions of normal tissues, local control rate, and overall survival rate. The secondary end point was efficacy of the treatment in comparison to historical results with 57.6 or 64.0 GyE/16 fr. Results: The 3-year local control rate and overall survival rate for all patients were 91.8% (95% confidence interval [CI] = 81.0–100%) and 74.1% (95% CI = 57.5–90.6%), respectively. Acute reaction of Grade 3 or more was observed in only 1 patient. With regard to late reactions, visual loss was observed in 1 patient and a Grade 3 reaction of the maxillary bone was observed in 4 patients. A comparison with historical results revealed that the local control rate with 70.4 GyE was significantly higher than that with 57.6 or 64.0 GyE (3-year, 91.8% vs. 23.6%, p < 0.0001). Furthermore, the overall survival with 70.4 GyE tended to be higher than that with 57.6 or 64.0 GyE (3-year, 74.1% vs. 42.9%, p = 0.09). Conclusion: C-ion RT with 70.4 GyE/16 fr for bone and soft-tissue sarcoma of the adult head and neck appears to be effective with acceptable toxicities in comparison to conventional RT and C-ion RT with lower doses.

  6. MR criteria for differentiation of 'pseudo tumourous' lesions from bone sarcomas of the extremities. MRT-Kriterien zur Differenzierung 'pseudotumoroeser' Laesionen von Knochensarkomen der Extremitaeten

    Energy Technology Data Exchange (ETDEWEB)

    Lehner, K. (Technische Univ. Muenchen, Klinikum rechts der Isar, Inst. fuer Roentgendiagnostik (Germany)); Rechl, H. (Technische Univ. Muenchen, Klinikum rechts der Isar, Orthopaedische Klinik (Germany)); Daschner, H. (Technische Univ. Muenchen, Klinikum rechts der Isar, Inst. fuer Roentgendiagnostik (Germany)); Kutschker, C. (Technische Univ. Muenchen, Klinikum rechts der Isar, Inst. fuer Roentgendiagnostik (Germany))

    1993-05-01

    The MRT scans of 57 patients with Ewing or osteosarcomas and 34 patients with haematogenous osteomyelitis or periostitis/stress fractures were examined in order to determine whether a distinction between benign or malignant lesions is possible. Four criteria were evaluated: The margin of the bone marrow component; intensity and homogeneity of the T[sub 1]-weighted signal in the bone marrow; presence of an extraosseus structured soft tissue mass and/or soft tissue edema. It was found that central osteosarcomas and Ewing's sarcomas reduced signal intensity of the marrow to become muscle-isointense with a well defined margin. In acute haematogenous osteomyelitis and periostitis/stress fracture the marrow lesion was not sharply demarcated. In contrast to patients with bone sarcomas only one case of osteomyelitis showed an extrosseus structured soft tissue mass. On the basis of these findings we believe that acute haematogenous osteomyelitis can be distinguished with high degree of accuracy from Ewing's sarcoma and central osteosarcomas. (orig.)

  7. Peripheral blood minimal residual disease may replace bone marrow minimal residual disease as an immunophenotypic biomarker for impending relapse in acute myeloid leukemia.

    Science.gov (United States)

    Zeijlemaker, W; Kelder, A; Oussoren-Brockhoff, Y J M; Scholten, W J; Snel, A N; Veldhuizen, D; Cloos, J; Ossenkoppele, G J; Schuurhuis, G J

    2016-03-01

    As relapses are common in acute myeloid leukemia (AML), early relapse prediction is of high importance. Although conventional minimal residual disease (MRD) measurement is carried out in bone marrow (BM), peripheral blood (PB) would be an advantageous alternative source. This study aims to investigate the specificity of leukemia-associated immunophenotypes used for MRD detection in blood samples. Consistency of PB MRD as compared with BM MRD was determined in flow cytometric data of 205 paired BM and PB samples of 114 AML patients. A significant correlation was found between PB and BM MRD (r=0.67, Pconsolidation therapy. As PB MRD appeared to be an independent predictor for response duration, the highly specific PB MRD assay may have a prominent role in future MRD assessment in AML. PMID:26373238

  8. Properties of immature myeloid progenitors with nitric-oxide-dependent immunosuppressive activity isolated from bone marrow of tumor-free mice.

    Directory of Open Access Journals (Sweden)

    Parvin Forghani

    Full Text Available Myeloid derived suppressor cells (MDSCs from tumor-bearing mice are important negative regulators of anti-cancer immune responses, but the role for immature myeloid cells (IMCs in non-tumor-bearing mice in the regulation of immune responses are poorly described. We studied the immune-suppressive activity of IMCs from the bone marrow (BM of C57Bl/6 mice and the mechanism(s by which they inhibit T-cell activation and proliferation. IMCs, isolated from BM by high-speed FACS, inhibited mitogen-induced proliferation of CD4(+ and CD8(+ T-cells in vitro. Cell-to-cell contact of T-cells with viable IMCs was required for suppression. Neither neutralizing antibodies to TGFβ1, nor genetic disruption of indolamine 2,3-dioxygenase, abrogated IMC-mediated suppressive activity. In contrast, suppression of T-cell proliferation was absent in cultures containing IMCs from interferon-γ (IFN-γ receptor KO mice or T-cells from IFN-γ KO mice (on the C57Bl/6 background. The addition of NO inhibitors to co-cultures of T-cells and IMC significantly reduced the suppressive activity of IMCs. IFN-γ signaling between T-cells and IMCs induced paracrine Nitric Oxide (NO release in culture, and the degree of inhibition of T-cell proliferation was proportional to NO levels. The suppressive activity of IMCs from the bone marrow of tumor-free mice was comparable with MDSCs from BALB/c bearing mice 4T1 mammary tumors. These results indicate that IMCs have a role in regulating T-cell activation and proliferation in the BM microenvironment.

  9. Immunotherapy of childhood Sarcomas

    Directory of Open Access Journals (Sweden)

    Stephen S Roberts

    2015-08-01

    Full Text Available Pediatric sarcomas are a heterogeneous group of malignant tumors of bone and soft tissue origin. Although more than 100 different histologic subtypes have been described, the majority of pediatric cases belong to the Ewing’s family of tumors, rhabdomyosarcoma and osteosarcoma. Most patients that present with localized stage are curable with surgery and/or chemotherapy; however, those with metastatic disease at diagnosis or those who experience a relapse continue to have a very poor prognosis. New therapies for these patients are urgently needed. Immunotherapy is an established treatment modality for both liquid and solid tumors, and in pediatrics, most notably for neuroblastoma and osteosarcoma. In the past, immunomodulatory agents such as interferon, interleukin-2, and Liposomal-muramyl  tripeptide phosphatidyl-ethanolamine (L-MTP have been tried, with some activity seen in subsets of patients; additionally, various cancer vaccines have been studied with possible benefit. Monoclonal antibody therapies against tumor antigens such as disialoganglioside GD2 or immune checkpoint targets such as CTLA4 and PD-1 are being actively explored in pediatric sarcomas. Building on the success of adoptive T cell therapy for EBV-related lymphoma, strategies to redirect T cells using chimeric antigen receptors and bispecific antibodies are rapidly evolving with potential for the treatment of sarcomas. This review will focus on recent preclinical and clinical developments in targeted agents for pediatric sarcomas with emphasis on the immunobiology of immune checkpoints, immunoediting, tumor microenvironment, antibody engineering, cell engineering, and tumor vaccines. The future integration of antibody based and cell based therapies into an overall treatment strategy of sarcoma will be discussed.

  10. Immunotherapy of Childhood Sarcomas.

    Science.gov (United States)

    Roberts, Stephen S; Chou, Alexander J; Cheung, Nai-Kong V

    2015-01-01

    Pediatric sarcomas are a heterogeneous group of malignant tumors of bone and soft tissue origin. Although more than 100 different histologic subtypes have been described, the majority of pediatric cases belong to the Ewing's family of tumors, rhabdomyosarcoma and osteosarcoma. Most patients that present with localized stage are curable with surgery and/or chemotherapy; however, those with metastatic disease at diagnosis or those who experience a relapse continue to have a very poor prognosis. New therapies for these patients are urgently needed. Immunotherapy is an established treatment modality for both liquid and solid tumors, and in pediatrics, most notably for neuroblastoma and osteosarcoma. In the past, immunomodulatory agents such as interferon, interleukin-2, and liposomal-muramyl tripeptide phosphatidyl-ethanolamine have been tried, with some activity seen in subsets of patients; additionally, various cancer vaccines have been studied with possible benefit. Monoclonal antibody therapies against tumor antigens such as disialoganglioside GD2 or immune checkpoint targets such as CTLA-4 and PD-1 are being actively explored in pediatric sarcomas. Building on the success of adoptive T cell therapy for EBV-related lymphoma, strategies to redirect T cells using chimeric antigen receptors and bispecific antibodies are rapidly evolving with potential for the treatment of sarcomas. This review will focus on recent preclinical and clinical developments in targeted agents for pediatric sarcomas with emphasis on the immunobiology of immune checkpoints, immunoediting, tumor microenvironment, antibody engineering, cell engineering, and tumor vaccines. The future integration of antibody-based and cell-based therapies into an overall treatment strategy of sarcoma will be discussed. PMID:26301204

  11. Ovarian granulocytic sarcoma: a case report and magnetic resonance imaging findings; Sarcoma granulocitico no ovario: relato de caso e achados na ressonancia magnetica

    Energy Technology Data Exchange (ETDEWEB)

    Pereira, Licia Pacheco [Hospital Geral de Fortaleza (HGF), CE (Brazil). Servico de Diagnostico por Imagem], e-mail: licia_p@hotmail.com; Silveira, Claudio Regis Sampaio [Hospital Geral de Fortaleza (HGF), CE (Brazil). Servico de Radiologia; Costa, Fabricio da Silva [Universidade Estadual do Ceara (UECE), CE (Brazil); Monte, Hipolito [Hospital Monte Klinikum, Fortaleza, CE (Brazil)

    2008-12-15

    Granulocytic sarcoma (chloroma) is a tumor consisting of myeloid precursors in an extramedullary site. It is complication of both acute and chronic myelogenous leukemias. Although the lesion can occur at any site, ovarian involvement is rare. We report a case of ovary tumor associated with acute myeloid leukaemia and its imaging appearance on magnetic resonance. (author)

  12. Administration of a tropomyosin receptor kinase inhibitor attenuates sarcoma-induced nerve sprouting, neuroma formation and bone cancer pain

    Directory of Open Access Journals (Sweden)

    Bloom Aaron P

    2010-12-01

    Full Text Available Abstract Pain often accompanies cancer and most current therapies for treating cancer pain have significant unwanted side effects. Targeting nerve growth factor (NGF or its cognate receptor tropomyosin receptor kinase A (TrkA has become an attractive target for attenuating chronic pain. In the present report, we use a mouse model of bone cancer pain and examine whether oral administration of a selective small molecule Trk inhibitor (ARRY-470, which blocks TrkA, TrkB and TrkC kinase activity at low nm concentrations has a significant effect on cancer-induced pain behaviors, tumor-induced remodeling of sensory nerve fibers, tumor growth and tumor-induced bone remodeling. Early/sustained (initiated day 6 post cancer cell injection, but not late/acute (initiated day 18 post cancer cell injection administration of ARRY-470 markedly attenuated bone cancer pain and significantly blocked the ectopic sprouting of sensory nerve fibers and the formation of neuroma-like structures in the tumor bearing bone, but did not have a significant effect on tumor growth or bone remodeling. These data suggest that, like therapies that target the cancer itself, the earlier that the blockade of TrkA occurs, the more effective the control of cancer pain and the tumor-induced remodeling of sensory nerve fibers. Developing targeted therapies that relieve cancer pain without the side effects of current analgesics has the potential to significantly improve the quality of life and functional status of cancer patients.

  13. Refinements in Sarcoma Classification in the Current 2013 World Health Organization Classification of Tumours of Soft Tissue and Bone.

    Science.gov (United States)

    Jo, Vickie Y; Doyle, Leona A

    2016-10-01

    The fourth edition of the World Health Organization (WHO) Classification of Tumours of Soft Tissue and Bone was published in February 2013. The 2013 WHO volume provides an updated classification scheme and reproducible diagnostic criteria, which are based on recent clinicopathologic studies and genetic and molecular data that facilitated refined definition of established tumor types, recognition of novel entities, and the development of novel diagnostic markers. This article reviews updates and changes in the classification of bone and soft tissue tumors from the 2002 volume. PMID:27591490

  14. Pim2 cooperates with PML-RARalpha to induce acute myeloid leukemia in a bone marrow transplantation model

    DEFF Research Database (Denmark)

    Agrawal-Singh, Shuchi; Koschmieder, Steffen; Gelsing, Sandra;

    2010-01-01

    role of Pim2 with promyelocytic leukemia/retinoic acid receptor alpha (PML/RARalpha), we used a well-established PML-RARalpha (PRalpha) mouse model. Pim2 coexpression in PRalpha-positive hematopoietic progenitor cells (HPCs) induces leukemia in recipient mice after a short latency. Pim2-PRalpha cells...... immature myeloid cells, compared with the previously described APL disease induced by PRalpha. However, it also clearly resembled an APL-like phenotype and showed signs of differentiation upon all-trans retinoic acid (ATRA) treatment in vitro. These results support the hypothesis that Pim2, which is also a...

  15. Molecular biological characteristics of the recruitment of hematopoietic stem cells from bone marrow niche in chronic myeloid leukemia

    OpenAIRE

    Zhu, Biao; Zhang, Jianbo; Chen, Jiao; Li, Chenglong; Wang, Xiaodong

    2015-01-01

    Chronic myeloid leukemia (CML) can be contextualized as a disease of unregulated self-renewal of stem cells which exist in a quiescent state and are instructed to differentiate and mobilize to circulation under pathologic circumstances leading to tumor invasion and metastasis. Here we found that matrix metalloproteinase-9 (MMP-9), induced by TGF-β1, upregulated s-KitL and s-ICAM-1, permitting the transfer of c-kit+ hematopoietic stem cells (HSCs) from the quiescent to proliferative niche in C...

  16. Association between the methylation status of the MGMT promoter in bone marrow specimens and chemotherapy outcomes of patients with acute myeloid leukemia

    Science.gov (United States)

    HONG, QINGXIAO; CHEN, XIAOYING; YE, HUADAN; ZHOU, ANNAN; GAO, YUTING; JIANG, DANJIE; WU, XIAODONG; TIAN, BINGRU; CHEN, YOUFEN; WANG, MING; XIE, JIPING; XIA, YONGMING; DUAN, SHIWEI

    2016-01-01

    The O(6)-methylguanine-DNA methyltransferase (MGMT) gene is a tumor suppressor gene that is associated with the risk of developing acute myeloid leukemia (AML). However, the association between the methylation status of the MGMT promoter and the chemotherapeutic outcomes of patients with AML remains unknown. In the present study, 30 bone marrow samples derived from patients with AML were collected prior and subsequent to chemotherapy. The methylation status of the MGMT promoter in the bone marrow specimens was determined by methylation-specific polymerase chain reaction. The results indicated that the methylation status of the MGMT promoter was influenced by different chemotherapeutic regimens. The MGMT methylation status of M4 patients (3 out of 6) were more chemosensitive, compared with that of patients with other AML subtypes (M1, 1 out of 3; M2, 0 out of 8; M3, 3 out of 7; M5, 0 out of 3; and M6, 1 out of 3). Age-based analysis revealed that the group aged ≤60 years (7 out of 24 patients) exhibited more methylation changes than patients aged >60 years (1 out of 6). Male patients (4 out of 13) were more susceptible to chemotherapy-induced methylation changes than female patients (4 out of 17). Thus, the methylation status of the MGMT promoter may serve as a potential biomarker to predict the therapeutic outcomes in male AML patients. However, further studies in larger sample sets are required to confirm the present findings. PMID:27073563

  17. Synchronous granulocytic sarcoma of the breast and spine: a case report and review of the literature

    Institute of Scientific and Technical Information of China (English)

    CUI Yan; ZHOU Jin-lian; WU Ji-hua; ZHANG Jian-zhong

    2008-01-01

    @@ Granulocytic sarcoma or chloroma is a rare tumour derived from myeloid cell precursors. It is generally seen before or after or together with the onset of myelocytic leukaemia. Immunohistochemical staining of myeloperoxidase is necessary for a definite diagnosis of granulocytic sarcoma. Recognition of this entity ensures an early aggressive chemotherapy that causes regression of the tumour.

  18. Involvement of primary mesenchymal precursors and hematopoietic bone marrow cells from chronic myeloid leukemia patients by BCR-ABL1 fusion gene.

    Science.gov (United States)

    Chandia, Mauricio; Sayagués, José-María; Gutiérrez, María-Laura; Chillón, María-Laura; Aristizábal, José-Alejandro; Corrales, Alejandro; Castellanos, Marta; Melón, Alberto; Sánchez, María-Luz; Bárcena, Paloma; Matarraz, Sergio; González-González, María; Barrena, Susana; López, Antonio; Cañizo, María-Consuelo; Sánchez-Guijo, Fermín; Orfao, Alberto

    2014-03-01

    For decades now, it is well established that chronic myeloid leukemia (CML) is a hematopoietic stem cell(HPC) disorder. However, it remains to be determined whether BCR-ABL1 gene rearrangement occurs in a HPC or at an earlier stem cell and whether the degree of involvement of hematopoiesis by the BCR-ABL1 fusion gene relates to the response to therapy. Here, we have investigated by interphase fluorescence in situ hybridization (iFISH) the distribution of BCR-ABL1 fusion gene in FACS-sorted bone marrow (BM) populations of mesenchymal precursor cells (MPC) and other hematopoietic cell populations from 18 newly diagnosed CML patients. Overall, our results showed systematic involvement at relatively high percentages of BM maturing neutrophils (97%615%), basophils (95%612%), eosinophils (90%68%), CD341 precursors cells (90%67%),monocytes (84%630%), nucleated red blood cells (87%624%), and mast cells (77%633%). By contrast, MPC(30%634%), B-cells (15%627%), T-lymphocytes (50%626%), and NK-cells (35%634%) were involved at lower percentages. In 8/18 CML patients, 2 tumor BCR-ABL11 subclones were detected by iFISH. Of note, all tumor cell subclones were systematically detected in CD341 cells, whereas MPC were only involved by the ancestral tumor cell subclone. In summary, here we confirm the presence at diagnosis of the BCR-ABL1 fusion gene inMPC, CD341 precursors, and other different BM hematopoietic myeloid cell lineages from CML patients,including also in a significant fraction of cases, a smaller percentage of T, B, and NK lymphocytes.Interestingly, involvement of MPC was restricted to the ancestral BCR-ABL11 subclone. PMID:24779036

  19. Stages of Ewing Sarcoma

    Science.gov (United States)

    ... such as size, shape, and the presence of tumor markers on the cell surface. The cells are stained ... cells. The disease is metastatic Ewing sarcoma, not lung cancer. Recurrent Ewing Sarcoma Recurrent Ewing sarcoma is cancer ...

  20. Sarcoma Foundation of America

    Science.gov (United States)

    ... Mission The mission of the Sarcoma Foundation of America (SFA) is to advocate for sarcoma patients by ... behalf of everyone at the Sarcoma Foundation of America (SFA),THANK YOU! The Celebration of Life drew ...

  1. Role of radiation therapy in the multidisciplinary management of Ewings Sarcoma of bone in pediatric patients: An effective treatment for local control

    International Nuclear Information System (INIS)

    Background: Radiotherapy (RT) plays an important role in the multidisciplinary management of Ewings Sarcoma (ES), especially in unresectable cases. Aim: Assessment of efficacy of RT in terms of local control in pediatric patients with primary ES of bone. Materials and methods: Thirty-six patients younger than 17 years old with ES treated with combined RT and chemotherapy with (N= 14) or without (N= 22) prior surgery from 1981 to 2008 were retrospectively reviewed. Since 1995, they were all treated according to the Spanish Society of Pediatric Oncology protocol (55.5% cases). Those patients received vincristine, ifosfamide, doxorubicin and etoposide. The TNM classification was as follows: 17 T1, 18 T2 and 1 T3; 36 N0; 29 M0, 5 M1a and 2 M1b. Analysis was stratified by treatment: definitive RT or pre/postoperative RT. Results: The 36 patients (21 male; 15 female) had a median age of 10 years (range 2 - 17 years). Median follow-up of living patients was 105 months. The 2-year local control (LC) rate for all patients was 88%. Five-year LC rates for patients treated with definitive and pre/postoperative RT were 91% and 86%, respectively. Two-year overall survival and disease-free survival rates for all patients were 68% and 66%, respectively. Low phosphatase alkaline levels and local and distant recurrences were significantly predictive of worse prognosis (P = 0.021, P = 0.011, P = 0.007, respectively). Conclusion: Radiotherapy with and without surgery is a highly effective local treatment option in the multidisciplinary management of ES in pediatric patients. (authors)

  2. Cixutumumab and Doxorubicin Hydrochloride in Treating Patients With Unresectable, Locally Advanced, or Metastatic Soft Tissue Sarcoma

    Science.gov (United States)

    2016-05-16

    Adult Angiosarcoma; Adult Desmoplastic Small Round Cell Tumor; 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 Undifferentiated High Grade Pleomorphic Sarcoma of Bone; Childhood Angiosarcoma; Childhood Desmoplastic Small Round Cell Tumor; Childhood Epithelioid Sarcoma; Childhood Fibrosarcoma; Childhood Leiomyosarcoma; Childhood Liposarcoma; Childhood Malignant Mesenchymoma; Childhood Malignant Peripheral Nerve Sheath Tumor; Childhood Pleomorphic Rhabdomyosarcoma; Childhood Rhabdomyosarcoma With Mixed Embryonal and Alveolar Features; Childhood Synovial Sarcoma; Dermatofibrosarcoma Protuberans; Malignant Adult Hemangiopericytoma; Malignant Childhood Hemangiopericytoma; Metastatic Childhood Soft Tissue Sarcoma; Previously Treated Childhood Rhabdomyosarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Childhood Rhabdomyosarcoma; Recurrent Childhood Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma; Untreated Childhood Rhabdomyosarcoma

  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. Sarcoma Immunotherapy

    International Nuclear Information System (INIS)

    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

  5. Cell Cycle Deregulation in Ewing's Sarcoma Pathogenesis

    Directory of Open Access Journals (Sweden)

    Ashley A. Kowalewski

    2011-01-01

    Full Text Available Ewing's sarcoma is a highly aggressive pediatric tumor of bone that usually contains the characteristic chromosomal translocation t(11;22(q24;q12. This translocation encodes the oncogenic fusion protein EWS/FLI, which acts as an aberrant transcription factor to deregulate target genes necessary for oncogenesis. One key feature of oncogenic transformation is dysregulation of cell cycle control. It is therefore likely that EWS/FLI and other cooperating mutations in Ewing's sarcoma modulate the cell cycle to facilitate tumorigenesis. This paper will summarize current published data associated with deregulation of the cell cycle in Ewing's sarcoma and highlight important questions that remain to be answered.

  6. Extramedullary blast crisis as initial presentation in chronic myeloid leukemia with the e1a2 BCR-ABL1 transcript: A case report

    Science.gov (United States)

    AI, DI; LIU, WEI; LU, GARY; PATEL, KEYUR PRAVINCHANDRA; CHEN, ZI

    2015-01-01

    A 23-year-old woman presented with enlarged right inguinal lymph nodes. The pathological examination of the nodes revealed infiltration by myeloid sarcoma. A bone marrow smear and biopsy revealed cytogenetic abnormalities, with 46,XX,t(9;22) and chronic myeloid leukemia (CML) was diagnosed. The e1a2 BCR-ABL1 fusion transcript was detected. The patient received imatinib-based combined chemotherapy, allogeneic hematopoietic stem cell transplantation, donor lymphocyte infusions and dasatinib treatment. The patient achieved complete response and has remained leukemia-free for >48 months. To the best of our knowledge, this is the first case report of CML with the e1a2 BCR-ABL1 transcript, with extramedullary blast crisis as the initial presentation. The aim of the present study was to discuss this special case with reference to the literature. PMID:26807241

  7. Mac-1low early myeloid cells in the bone marrow-derived SP fraction migrate into injured skeletal muscle and participate in muscle regeneration

    International Nuclear Information System (INIS)

    Recent studies have shown that bone marrow (BM) cells, including the BM side population (BM-SP) cells that enrich hematopoietic stem cells (HSCs), are incorporated into skeletal muscle during regeneration, but it is not clear how and what kinds of BM cells contribute to muscle fiber regeneration. We found that a large number of SP cells migrated from BM to muscles following injury in BM-transplanted mice. These BM-derived SP cells in regenerating muscles expressed different surface markers from those of HSCs and could not reconstitute the mouse blood system. BM-derived SP/Mac-1low cells increased in number in regenerating muscles following injury. Importantly, our co-culture studies with activated satellite cells revealed that this fraction carried significant potential for myogenic differentiation. By contrast, mature inflammatory (Mac-1high) cells showed negligible myogenic activities. Further, these BM-derived SP/Mac-1low cells gave rise to mononucleate myocytes, indicating that their myogenesis was not caused by stochastic fusion with host myogenic cells, although they required cell-to-cell contact with myogenic cells for muscle differentiation. Taken together, our data suggest that neither HSCs nor mature inflammatory cells, but Mac-1low early myeloid cells in the BM-derived SP fraction, play an important role in regenerating skeletal muscles

  8. In vitro expanded bone marrow-derived murine (C57Bl/KaLwRij) mesenchymal stem cells can acquire CD34 expression and induce sarcoma formation in vivo

    Energy Technology Data Exchange (ETDEWEB)

    Xu, Song [Department of Lung Cancer Surgery, Lung Cancer Institute, Tianjin Medical University General Hospital, 300052 Tianjin (China); Stem Cell Laboratory-Division Clinical Hematology, Universitair Ziekenhuis Brussel (UZ Brussel), Laarbeeklaan 101, 1090 Brussels (Belgium); Department of Hematology and Immunology, Vrije Universiteit Brussel (VUB)-Myeloma Center, Laarbeeklaan 103, 1090 Brussels (Belgium); De Becker, Ann [Stem Cell Laboratory-Division Clinical Hematology, Universitair Ziekenhuis Brussel (UZ Brussel), Laarbeeklaan 101, 1090 Brussels (Belgium); De Raeve, Hendrik [Department of Anatomopathology, Universitair Ziekenhuis Brussel (UZ Brussel), Laarbeeklaan 101, 1090 Brussels (Belgium); Van Camp, Ben; Vanderkerken, Karin [Department of Hematology and Immunology, Vrije Universiteit Brussel (VUB)-Myeloma Center, Laarbeeklaan 103, 1090 Brussels (Belgium); Van Riet, Ivan, E-mail: ivan.vanriet@uzbrussel.be [Stem Cell Laboratory-Division Clinical Hematology, Universitair Ziekenhuis Brussel (UZ Brussel), Laarbeeklaan 101, 1090 Brussels (Belgium); Department of Hematology and Immunology, Vrije Universiteit Brussel (VUB)-Myeloma Center, Laarbeeklaan 103, 1090 Brussels (Belgium)

    2012-08-03

    Highlights: Black-Right-Pointing-Pointer Murine MSCs can undergo spontaneously malignant transformation and form sarcoma. Black-Right-Pointing-Pointer Acquisition of CD34 is a transformation type for MSCs into sarcoma. Black-Right-Pointing-Pointer Notch/Hh/Wnt pathways are related to the malignant phenotype of transformed MSCs. -- Abstract: Mesenchymal stem cells (MSCs) have currently generated numerous interests in pre-clinical and clinical applications due to their multiple lineages differentiation potential and immunomodulary effects. However, accumulating evidence indicates that MSCs, especially murine MSCs (mMSCs), can undergo spontaneous transformation after long-term in vitro culturing, which might reduce the therapeutic application possibilities of these stem cells. In the present study, we observed that in vitro expanded bone marrow (BM) derived mMSCs from the C57Bl/KaLwRij mouse strain can lose their specific stem cells markers (CD90 and CD105) and acquire CD34 expression, accompanied with an altered morphology and an impaired tri-lineages differentiation capacity. Compared to normal mMSCs, these transformed mMSCs exhibited an increased proliferation rate, an enhanced colony formation and migration ability as well as a higher sensitivity to anti-tumor drugs. Transformed mMSCs were highly tumorigenic in vivo, resulting in aggressive sarcoma formation when transplanted in non-immunocompromised mice. Furthermore, we found that Notch signaling downstream genes (hey1, hey2 and heyL) were significantly upregulated in transformed mMSCs, while Hedgehog signaling downstream genes Gli1 and Ptch1 and the Wnt signaling downstream gene beta-catenin were all decreased. Taken together, we observed that murine in vitro expanded BM-MSCs can transform into CD34 expressing cells that induce sarcoma formation in vivo. We assume that dysregulation of the Notch(+)/Hh(-)/Wnt(-) signaling pathway is associated with the malignant phenotype of the transformed mMSCs.

  9. In vitro expanded bone marrow-derived murine (C57Bl/KaLwRij) mesenchymal stem cells can acquire CD34 expression and induce sarcoma formation in vivo

    International Nuclear Information System (INIS)

    Highlights: ► Murine MSCs can undergo spontaneously malignant transformation and form sarcoma. ► Acquisition of CD34 is a transformation type for MSCs into sarcoma. ► Notch/Hh/Wnt pathways are related to the malignant phenotype of transformed MSCs. -- Abstract: Mesenchymal stem cells (MSCs) have currently generated numerous interests in pre-clinical and clinical applications due to their multiple lineages differentiation potential and immunomodulary effects. However, accumulating evidence indicates that MSCs, especially murine MSCs (mMSCs), can undergo spontaneous transformation after long-term in vitro culturing, which might reduce the therapeutic application possibilities of these stem cells. In the present study, we observed that in vitro expanded bone marrow (BM) derived mMSCs from the C57Bl/KaLwRij mouse strain can lose their specific stem cells markers (CD90 and CD105) and acquire CD34 expression, accompanied with an altered morphology and an impaired tri-lineages differentiation capacity. Compared to normal mMSCs, these transformed mMSCs exhibited an increased proliferation rate, an enhanced colony formation and migration ability as well as a higher sensitivity to anti-tumor drugs. Transformed mMSCs were highly tumorigenic in vivo, resulting in aggressive sarcoma formation when transplanted in non-immunocompromised mice. Furthermore, we found that Notch signaling downstream genes (hey1, hey2 and heyL) were significantly upregulated in transformed mMSCs, while Hedgehog signaling downstream genes Gli1 and Ptch1 and the Wnt signaling downstream gene beta-catenin were all decreased. Taken together, we observed that murine in vitro expanded BM-MSCs can transform into CD34 expressing cells that induce sarcoma formation in vivo. We assume that dysregulation of the Notch(+)/Hh(−)/Wnt(−) signaling pathway is associated with the malignant phenotype of the transformed mMSCs.

  10. Myeloid cell-derived reactive oxygen species externally regulate the proliferation of myeloid progenitors in emergency granulopoiesis

    OpenAIRE

    Kwak, Hyun-Jeong; Liu, Peng; Bajrami, Besnik; Xu, Yuanfu; Park, Shin-Young; Nombela-Arrieta, Cesar; Mondal, Subhanjan; Sun, Yan; Zhu, Haiyan; Chai, Li; Silberstein, Leslie E.; Cheng, Tao; Luo, Hongbo R.

    2015-01-01

    The cellular mechanisms controlling infection-induced emergency granulopoiesis are poorly defined. Here we found that reactive oxygen species (ROS) concentrations in the bone marrow (BM) were elevated during acute infection in a phagocytic NADPH oxidase-dependent manner in myeloid cells. Gr1+ myeloid cells were uniformly distributed in the BM, and all c-Kit+ progenitor cells were adjacent to Gr1+ myeloid cells. Inflammation-induced ROS production in the BM played a critical role in myeloid pr...

  11. Intra articular synovial sarcoma

    Directory of Open Access Journals (Sweden)

    Sistla Radha

    2010-01-01

    Full Text Available Synovial sarcoma is a soft tissue neoplasm with a characteristic biphasic pattern. Incidence in soft tissues is 5-10%. Intra articularly synovial sarcoma is extremely rare. Fewer than 5% of all synovial sarcomas arise within the joint space. We report a case of intra articular synovial sarcoma in a young male who presented as internal derangement of the knee.

  12. Intra articular synovial sarcoma

    OpenAIRE

    Sistla Radha; Tameem Afroz; Vidyasagar JVS

    2010-01-01

    Synovial sarcoma is a soft tissue neoplasm with a characteristic biphasic pattern. Incidence in soft tissues is 5-10%. Intra articularly synovial sarcoma is extremely rare. Fewer than 5% of all synovial sarcomas arise within the joint space. We report a case of intra articular synovial sarcoma in a young male who presented as internal derangement of the knee.

  13. Postradiation sarcomas: importance of surgery

    International Nuclear Information System (INIS)

    Purpose: To evaluate the role of surgery in the treatment of Post-radiation sarcomas Materials. Post-radiation sarcomas is a rare entity and large series have rarely been reported. In order to improve knowledge about this entity the Radiotherapist group of the French Cancer Centres (FNCLCC) decided to collect retrospectively the cases treated in their institutions. In order to be sure of the histology, all the cases were reviewed by a panel of pathologists of the FNCLCC Pathologist group. A total of 129 cases of sarcomas, and 108 were reviewed; analysis of 8 is in progress, and no material was obtained in the other 11 cases. The diagnosis of sarcomas was confirmed in 80 cases. All patients (60 F, 20 M) have received radiation therapy (median dose 50 Gy; 9-110 Gy) for the treatment of the primary tumor. At this time the age was 44 years (6-83 y). Diagnoses included: breast C. 42%, Lymphomas 11.5%, gynaecological C. 10% benign lesions 5% miscellaneous. Sarcomas developed after a mean interval of 12 years (3-64 y), in bone in 30% of the cases and in soft tissue in 70%. The majority of lesions (90%) developed in the irradiated field (dose received was between 50 Gy and 60 Gy). Histologically there were 29% Malignant HistiocytofibroSarcomas, 19% OsteoSarcomas, 15% FibroSarcomas, 9% LipoSarcomas, 6% LeiomyoSarcomas, miscellaneous sarcomas 22%. Treatment included: Surgery 28 cases, Surgery+Chemotherapy 17 cases, Chemotherapy only 16 cases, Radiation therapy only 1 case, surgery + Radiation therapy 5 cases, Radiation therapy +chemotherapy 6 cases, Surgery + Radiation therapy + Chemotherapy 7 cases, no treatment 5 cases. Results. The outcome is known for all but 3 patients. 51 patients have died (44 of their sarcoma, 4 of the primary tumour, 2 of other cause and 1 iatrogenic). Median survival is 23 months (95% confidence interval 16-29 mo) but 9 patients survived 5 yr or more. Median survival was 43 mo for patients treated by surgery (28p), 6 mo for chemotherapy group (16 p

  14. Radiation Therapy With or Without Combination Chemotherapy or Pazopanib Hydrochloride Before Surgery in Treating Patients With Newly Diagnosed Non-Rhabdomyosarcoma Soft Tissue Sarcomas That Can Be Removed by Surgery

    Science.gov (United States)

    2016-08-03

    Adult Fibrosarcoma; Alveolar Soft Part Sarcoma; Angiomatoid Fibrous Histiocytoma; Atypical Fibroxanthoma; Clear Cell Sarcoma of Soft Tissue; Epithelioid Malignant Peripheral Nerve Sheath Tumor; Epithelioid Sarcoma; Extraskeletal Myxoid Chondrosarcoma; Extraskeletal Osteosarcoma; Fibrohistiocytic Neoplasm; Glomus Tumor of the Skin; Inflammatory Myofibroblastic Tumor; Intimal Sarcoma; Leiomyosarcoma; Liposarcoma; Low Grade Fibromyxoid Sarcoma; Low Grade Myofibroblastic Sarcoma; Malignant Cutaneous Granular Cell Tumor; Malignant Peripheral Nerve Sheath Tumor; Malignant Triton Tumor; Mesenchymal Chondrosarcoma; Myxofibrosarcoma; Myxoid Chondrosarcoma; Myxoinflammatory Fibroblastic Sarcoma; Nerve Sheath Neoplasm; PEComa; Pericytic Neoplasm; Plexiform Fibrohistiocytic Tumor; Sclerosing Epithelioid Fibrosarcoma; Stage IB Soft Tissue Sarcoma; Stage IIB Soft Tissue Sarcoma; Stage III Soft Tissue Sarcoma; Stage IV Soft Tissue Sarcoma; Synovial Sarcoma; Undifferentiated (Embryonal) Sarcoma; Undifferentiated High Grade Pleomorphic Sarcoma of Bone

  15. Whole-Body Radiation Therapy, Systemic Chemotherapy, and High-Dose Chemotherapy Followed By Stem Cell Rescue in Treating Patients With Poor-Risk Ewing Sarcoma

    Science.gov (United States)

    2015-01-07

    Adult Supratentorial Primitive Neuroectodermal Tumor (PNET); Ewing Sarcoma of Bone; Extraosseous Ewing Sarcoma; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Childhood Supratentorial Primitive Neuroectodermal Tumor; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Untreated Childhood Supratentorial Primitive Neuroectodermal Tumor

  16. Expression of CDKN1C in the bone marrow of patients with myelodysplastic syndrome and secondary acute myeloid leukemia is associated with poor survival after conventional chemotherapy.

    Science.gov (United States)

    Radujkovic, Aleksandar; Dietrich, Sascha; Andrulis, Mindaugas; Benner, Axel; Longerich, Thomas; Pellagatti, Andrea; Nanda, Kriti; Giese, Thomas; Germing, Ulrich; Baldus, Stefan; Boultwood, Jacqueline; Ho, Anthony D; Dreger, Peter; Luft, Thomas

    2016-09-15

    We tested the hypothesis that proliferative activity of hematopoietic stem cells has impact on survival in newly diagnosed patients with myelodysplastic syndrome (MDS) and secondary acute myeloid leukemia (AML). RNA expression profiles of CD34(+) cells were analyzed in 125 MDS patients and compared to healthy controls. Prognostic impact on overall survival (OS) of mRNA proliferation signatures established for solid tumor cells was analyzed retrospectively. For validation on the protein level, immunofluorescence and immunohistochemistry analyses in bone marrow (BM) biopsies were performed, and an independent cohort of 223 MDS and secondary AML patients was investigated. Lower proliferative activity correlated with the expression of cyclin-dependent kinase inhibitor 1C (CDKN1C) and with shorter OS (p < 0.001). In multivariable analysis, higher CDKN1C expression was associated with worse OS (p = 0.02). On the BM level, a total of 84 (38%) patients showed CDKN1C protein expression before start of treatment. Patient, disease and treatment characteristics did not differ between CDKN1C-positive and -negative patients. Positive CDKN1C BM status was associated with shorter OS in multivariable analysis (HR 1.54, p = 0.04). There was an interaction between CDKN1C BM status and subsequent treatment with negative impact on OS being most pronounced in patients receiving conventional cytotoxic chemotherapy (n = 83, 2-year OS 30% versus 58%, p = 0.002). In conclusion, low-proliferative phenotype and CDKN1C expression were associated with shorter OS. CDKN1C protein expression in the BM of newly diagnosed, treatment-naïve MDS and secondary AML patients was identified as a prognostic factor for poor survival in patients treated with antiproliferative chemotherapy. PMID:27170453

  17. Effective Concentration of a Multikinase Inhibitor within Bone Marrow Correlates with In Vitro Cell Killing in Therapy-Resistant Chronic Myeloid Leukemia.

    Science.gov (United States)

    Mu, Chaofeng; Wu, Xiaoyan; Ma, Helen; Tao, Wenjing; Zhang, Guodong; Xia, Xiaojun; Shen, Jianliang; Mai, Junhua; Sun, Tong; Sun, Xiaoping; Arlinghaus, Ralph B; Shen, Haifa

    2016-05-01

    Leukemia cells escape BCR-ABL-targeted therapy by developing mutations, such as T315I, in the p210(BCR-ABL) fusion protein in Philadelphia chromosome-positive chronic myeloid leukemia (CML). Although most effort has been focused on development of new tyrosine kinase inhibitors, enrichment of these small-molecule inhibitors in the tumor tissue can also have a profound impact on treatment outcomes. Here, we report that a 2-hour exposure of the T315I-mutant CML cells to 10 μmol/L of the multikinase inhibitor TG101209 suppressed BCR-ABL-independent signaling and caused cell-cycle arrest at G2-M. Further increase in drug concentration to 17.5 μmol/L blocked phosphorylation of the mutant BCR-ABL kinase and its downstream JAK2 and STAT5. The effective dosage to overcome therapy resistance identified in an in vitro setting serves as a guidance to develop the proper drug formulation for in vivo efficacy. A targeted formulation was developed to achieve sustained bone marrow TG101209 concentration at or above 17.5 μmol/L for effective killing of CML cells in vivo Potent inhibition of leukemia cell growth and extended survival were observed in two murine models of CML treated with 40 mg/kg intravenously administered targeted TG101209, but not with the untargeted drug at the same dosage. Our finding provides a unique approach to develop treatments for therapy-resistant CML. Mol Cancer Ther; 15(5); 899-910. ©2016 AACR. PMID:26846820

  18. Ewing's Sarcoma of the Stomach; Rare Case of Ewing's Sarcoma and Suggestion of New Treatment Strategy

    OpenAIRE

    Kim, Hyo-Sin; Kim, Sungsoo; Min, Young-Don; KEE, KEUN-HONG; HONG, RAN

    2012-01-01

    Ewing's sarcoma is a neoplasm of the undifferenciated small round cells, which generally affects the bone and deep soft tissues of children and adolescents. We present a case of gastric Ewing's sarcoma; a 35-year-old female who had no symptoms. While she was at a routine medical checkup, a protruding mass in her gastric antrum was incidentally found on esophagogastroduodenoscopy. Endoscopic ultrasonogram showed a submucosal mass on the same lesion and a laparosopic wedge resection was done. P...

  19. 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.

  20. Histology and imaging of soft tissue sarcomas

    International Nuclear Information System (INIS)

    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. Synovial sarcoma in childhood

    International Nuclear Information System (INIS)

    The clinical and radiologic findings in seven children with synovial sarcoma are described. The five boys and two girls had a mean age at presentation of 4.4 years. All seven had the lesion situated in an extremity. Plain radiographs in four revealed the presence of a soft-tissue mass with no calcification or bone and joint involvement. In two patients studied with computed tomography (CT), the primary lesions had peripheral irregular areas of enhancement with central areas of poor enhancement, reflecting the necrotic, cystic, and hemorrhagic changes found in the centers of these tumors. Although the exact margins of these lesions were difficult to define accurately even with intravenous contrast enhancement, CT is still recommended as the best imaging method for assessing the local extent of the primary tumor and is a useful tool in the planning of appropriate therapy as well as the gauging of the tumor response to ongoing treatment

  2. Synovial sarcoma in childhood

    Energy Technology Data Exchange (ETDEWEB)

    Israels, S.J.; Chan, H.S.L.; Daneman, A.; Weitzman, S.S.

    1984-04-01

    The clinical and radiologic findings in seven children with synovial sarcoma are described. The five boys and two girls had a mean age at presentation of 4.4 years. All seven had the lesion situated in an extremity. Plain radiographs in four revealed the presence of a soft-tissue mass with no calcification or bone and joint involvement. In two patients studied with computed tomography (CT), the primary lesions had peripheral irregular areas of enhancement with central areas of poor enhancement, reflecting the necrotic, cystic, and hemorrhagic changes found in the centers of these tumors. Although the exact margins of these lesions were difficult to define accurately even with intravenous contrast enhancement, CT is still recommended as the best imaging method for assessing the local extent of the primary tumor and is a useful tool in the planning of appropriate therapy as well as the gauging of the tumor response to ongoing treatment.

  3. Bone marrow myeloid-derived suppressor cells (MDSCs) inhibit graft-versus-host disease (GVHD) via an arginase-1–dependent mechanism that is up-regulated by interleukin-13

    OpenAIRE

    Highfill, Steven L.; Rodriguez, Paulo C.; Zhou, Qing; Goetz, Christine A.; Koehn, Brent H; Veenstra, Rachelle; Taylor, Patricia A.; Panoskaltsis-Mortari, Angela; Serody, Jonathan S.; Munn, David H.; Tolar, Jakub; Ochoa, Augusto C.; Blazar, Bruce R.

    2010-01-01

    Myeloid-derived suppressor cells (MDSCs) are a well-defined population of cells that accumulate in the tissue of tumor-bearing animals and are known to inhibit immune responses. Within 4 days, bone marrow cells cultured in granulocyte colony-stimulating factor and granulocyte-macrophage colony-stimulating factor resulted in the generation of CD11b+Ly6GloLy6C+ MDSCs, the majority of which are interleukin-4Rα (IL-4Rα+) and F4/80+. Such MDSCs potently inhibited in vitro allogeneic T-cell respons...

  4. Aberrant Gene Expression in Acute Myeloid Leukaemia

    DEFF Research Database (Denmark)

    Bagger, Frederik Otzen

    Summary Acute Myeloid Leukaemia (AML) is an aggressive cancer of the bone marrow, affecting formation of blood cells during haematopoiesis. This thesis presents investigation of AML using mRNA gene expression profiles (GEP) of samples extracted from the bone marrow of healthy and diseased subjects...

  5. Complex three-way translocation involving MLL, ELL, RREB1, and CMAHP genes in an infant with acute myeloid leukemia and t(6;19;11)(p22.2;p13.1;q23.3)

    DEFF Research Database (Denmark)

    Tuborgh, A; Meyer, C; Marschalek, R;

    2013-01-01

    Rearrangements affecting the MLL gene in hematological malignancies are associated with poor prognosis. Most often they are reciprocal translocations and more rarely complex forms involving at least 3 chromosomes. We describe an unusual case with cutaneous leukemic infiltrates that waxed and waned...... until progression to acute myeloid leukemia, AML-M5. The leukemic cells harbored a novel apparent 3-way translocation t(6;19;11)(p22.2;p13.1;q23.3). We utilized advanced molecular cytogenetic methods including 24-color karyotyping, high-resolution array comparative genomic hybridization (aCGH) and DNA...... initial stages of disease before clear morphological signs of bone marrow involvement. The patient responded well to therapy and remains in remission>6 years from diagnosis. This apparent 3-way translocation is remarkable because of its rarity and presentation with myeloid sarcoma, and may, as more cases...

  6. Adult soft tissue sarcoma

    Science.gov (United States)

    ... page: //medlineplus.gov/ency/article/007654.htm Adult soft tissue sarcoma To use the sharing features on this page, please enable JavaScript. Soft tissue sarcoma is cancer that forms in the soft ...

  7. What Is Kaposi Sarcoma?

    Science.gov (United States)

    ... with a certain virus (the Kaposi sarcoma associated herpesvirus or KSHV ) are more likely to develop KS. ... who already have a KSHV (Kaposi sarcoma associated herpesvirus) infection are more likely to develop KS. Endemic ( ...

  8. Primary Renal Synovial Sarcoma

    OpenAIRE

    Halil Ciftci; Adem Altunkol; Ismail Ozdemir; Dilek Mil; Ilyas Ozardali; Murat Savas; Ercan Yeni; Mehmet Gulum

    2011-01-01

    Synovial sarcomas are generally deep-seated tumors that most often occur in the proximity of large joints of adolescents and young adults. We describe two cases of primary renal synovial sarcoma that were treated successfully by radical nephrectomy. Synovial sarcoma originating from the kidney is extremely rare and the histogenesis is uncertain. Surgical resection and ifosfamide based chemotherapy are the mainstay for the management of renal synovial sarcoma. Fewer than 40 patients have been ...

  9. Proton Radiotherapy for Pediatric Sarcoma

    International Nuclear Information System (INIS)

    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

  10. Irradiation for conjunctival granulocytic sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Fleckenstein, K.; Geinitz, H.; Grosu, A.; Molls, M. [Dept. of Radiation Oncology, Technical Univ. of Munich (Germany); Goetze, K. [Dept. of Hematology and Oncology, Technical Univ. of Munich (Germany); Werner, M. [Dept. of Pathology, Technical Univ. of Munich (Germany)

    2003-03-01

    Case History and Findings: A 73-year-old woman with a history of myeloproliferative syndrome (MPS) presented with bilateral chemosis, redness and burning of the eyes. The ocular motility was severely impaired. Ophthalmological examination revealed markedly distended conjunctivas on both sides. Biopsy disclosed conjunctival granulocytic sarcoma as an initial symptom of acute myelogenous leukemia (AML). Diagnosis was confirmed by peripheral blood smear and bone marrow aspiration. Treatment and Outcome: The orbital tumor disappeared completely after local external beam irradiation with a total dose of 30 Gy and no further orbital recurrence occurred. With chemotherapy following irradiation transient hematological remission was achieved. 5 months after diagnosis the patient died of respiratory failure following atypical pneumonia as a consequence of her underlying disorder. Conclusion: Detection of orbital granulocytic sarcoma, even in the absence of typical leukemic symptoms is of practical importance, because treatment with irradiation can lead to stabilization or improvement in the patient's vision. (orig.)

  11. Irradiation for conjunctival granulocytic sarcoma

    International Nuclear Information System (INIS)

    Case History and Findings: A 73-year-old woman with a history of myeloproliferative syndrome (MPS) presented with bilateral chemosis, redness and burning of the eyes. The ocular motility was severely impaired. Ophthalmological examination revealed markedly distended conjunctivas on both sides. Biopsy disclosed conjunctival granulocytic sarcoma as an initial symptom of acute myelogenous leukemia (AML). Diagnosis was confirmed by peripheral blood smear and bone marrow aspiration. Treatment and Outcome: The orbital tumor disappeared completely after local external beam irradiation with a total dose of 30 Gy and no further orbital recurrence occurred. With chemotherapy following irradiation transient hematological remission was achieved. 5 months after diagnosis the patient died of respiratory failure following atypical pneumonia as a consequence of her underlying disorder. Conclusion: Detection of orbital granulocytic sarcoma, even in the absence of typical leukemic symptoms is of practical importance, because treatment with irradiation can lead to stabilization or improvement in the patient's vision. (orig.)

  12. 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.

  13. Synovial sarcoma with radiological appearances of primitive neuroectodermal tumour/Ewing sarcoma: differentiation by molecular genetic studies

    International Nuclear Information System (INIS)

    Synovial sarcoma (SS) arises in soft tissues but may invade adjacent bone. We describe a case of SS presenting as aggressive lysis of the proximal ulna, the imaging of which suggested a primary bone lesion. Needle biopsy showed a 'small round blue cell tumour', and a primitive neuroectodermal tumour (PNET)/Ewing sarcoma was suggested on the basis of the imaging appearances. The definitive diagnosis of synovial sarcoma was made following molecular genetic studies, which demonstrated a fusion product incorporating the genes SYT and SSX1. The importance of correct diagnosis to guide appropriate management, and, therefore, the necessity for molecular genetic studies, is discussed. (orig.)

  14. Synovial sarcoma with radiological appearances of primitive neuroectodermal tumour/Ewing sarcoma: differentiation by molecular genetic studies

    Energy Technology Data Exchange (ETDEWEB)

    O' Donnell, P. [Royal National Orthopaedic Hospital NHS Trust, Department of Radiology, Stanmore, Middlesex (United Kingdom); Diss, T.C. [University College Hospital, Department of Histopathology, London (United Kingdom); Whelan, J. [University College Hospital, Meyerstein Institute of Oncology, London (United Kingdom); Flanagan, A.M. [University College Hospital, Department of Histopathology, London (United Kingdom); Royal National Orthopaedic Hospital, Department of Histopathology, Stanmore, Middlesex (United Kingdom); University College, Institute of Orthopaedics, London (United Kingdom)

    2006-04-15

    Synovial sarcoma (SS) arises in soft tissues but may invade adjacent bone. We describe a case of SS presenting as aggressive lysis of the proximal ulna, the imaging of which suggested a primary bone lesion. Needle biopsy showed a 'small round blue cell tumour', and a primitive neuroectodermal tumour (PNET)/Ewing sarcoma was suggested on the basis of the imaging appearances. The definitive diagnosis of synovial sarcoma was made following molecular genetic studies, which demonstrated a fusion product incorporating the genes SYT and SSX1. The importance of correct diagnosis to guide appropriate management, and, therefore, the necessity for molecular genetic studies, is discussed. (orig.)

  15. SYNOVIAL CELL SARCOMA

    OpenAIRE

    Farzan, M

    1997-01-01

    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.

  16. 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.

  17. Synovial sarcoma

    International Nuclear Information System (INIS)

    Authors present the material of 12 patients with synovial sarcoma treated in Surgical Oncology Dept. Medical University of Gdansk between 1973-1993. In all cases the main symptom was a growing tumor; tumor accompanied by pain in 3 of them. Average time for symptoms was about 9 months. Tumors were classified according to GTNM and Enneking Classification. 9 cases were classified as G2T1NOMO and 3 as G2T2NOMO. According to Enneking Classification 1 case only was recognized as intracompartmental and 11 as extracompartmental. In 9 tumors cytogenetic analysis was done. In 8 of them specific chromosome aberration was discovered - translocation between X and 18 chromosomes: t (18;X)(p11;q11). The following procedures were performed: enucleation of tumor (non-radical) in 2 cases, wide excision in 8 cases, extremity amputation in 1 case. Local recurrence was observed in 9 cases. 30 months without recurrence was noticed as an average. The wide local excision was the method of treatment of local recurrence in 4 cases (followed by RTG-therapy in one case). In another 5 cases extremity amputation was performed (followed by CyVADic chemotherapy in one case). In our material 5-year survival was observed in 36% of cases. The longest notice follow-up was 30 years. (author)

  18. Myeloid Toxicity of Cancer Treatment

    OpenAIRE

    Kurtin, Sandra

    2012-01-01

    Myelotoxicity is one of the most common treatment-related adverse events for patients receiving systemic antineoplastic therapy or radiotherapy to bone marrow–producing regions. Myeloid cytopenias, including neutropenia, thrombocytopenia, and anemia, are the most common manifestations of treatment-related myelotoxicity and one of the most common reasons for dose modifications, dose delays, or discontinuation of therapy, potentially limiting therapeutic benefit. Risk factors for myelotoxicity ...

  19. Correlation of the findings of thallium-201 chloride scans with those of other imaging modalities and histology following therapy in patients with bone and soft tissue sarcomas

    Energy Technology Data Exchange (ETDEWEB)

    Kostakoglu, L. [Nuclear Medicine Service, Memorial Sloan-Kettering Cancer Center, New York, NY (United States); Panicek, D.M. [Department of Radiology, Memorial Sloan-Kettering Cancer Center, New York, NY (United States); Divgi, C.R. [Nuclear Medicine Service, Memorial Sloan-Kettering Cancer Center, New York, NY (United States); Botet, J. [Orthopedics Service, Memorial Sloan-Kettering Cancer Center, New York, NY (United States); Healy, J. [Orthopedics Service, Memorial Sloan-Kettering Cancer Center, New York, NY (United States); Larson, S.M. [Nuclear Medicine Service, Memorial Sloan-Kettering Cancer Center, New York, NY (United States); Abdel-Dayem, H.M. [Nuclear Medicine Service, Memorial Sloan-Kettering Cancer Center, New York, NY (United States)

    1995-11-01

    We performed a prospective study to evaluate the imaging potential of thallium-201 as compared with other imaging modalities in differentiating residual/recurrent tumors from post-therapy changes in patients with musculoskeletal sarcomas. {sup 201}Tl scans, magnetic resonance imaging (17), X-ray computed tomography (6) or contrast angiography (6) studies in 29 patients previously treated for musculoskeletal sarcomas were correlated with either histopathologic findings (26 patients) or 2-year clinical follow-up (three patients). All imaging studies were acquired within 2 weeks. Ratios of {sup 201}Tl tumor uptake to the contralateral (28 patients) or adjacent region of interest were calculated. When qualitative interpretation was in doubt, only those cases with a ratio of 1.5 or more were considered suggestive of recurrent or residual viable tumor tissue. Residual or recurrent tumor tissue was verified in 21 patients by biopsy. All had true-positive {sup 201}Tl scans while the other imaging modalities were true-positive in 20 and equivocal in one. In eight patients, there was no evidence of viable tumor tissue as proven by biopsy in five and long-term clinical follow-up in three. {sup 201}Tl scan was false-positive (ratio 1.5) in one patient and true-negative in seven while the other imaging modalities had four false-positives. The average {sup 201}Tl ratios were 3.8 {+-} 1.1 in the true-positive cases and 1.3 {+-} 0.3 in the true-negative cases. The percentage sensitivities, specificities, and accuracy for {sup 201}Tl were 100%, 87.5%, and 96.5% versus 95%, 50%, and 82.7% respectively for other imaging modalities. These results indicate that {sup 201}Tl scintigraphy is more accurate than other imaging modalities in differentiating residual/recurrent musculoskeletal sarcomas from post-therapy changes. (orig.)

  20. Treatment Option Overview (Ewing Sarcoma)

    Science.gov (United States)

    ... such as size, shape, and the presence of tumor markers on the cell surface. The cells are stained ... cells. The disease is metastatic Ewing sarcoma, not lung cancer. Recurrent Ewing Sarcoma Recurrent Ewing sarcoma is cancer ...

  1. Chemotherapy for Soft Tissue Sarcomas

    Science.gov (United States)

    ... Next Topic Targeted therapy for soft tissue sarcoma 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 ...

  2. The diagnostic and prognostic value of {sup 18}F-FDG PET/CT in the initial assessment of high-grade bone and soft tissue sarcoma. A retrospective study of 89 patients

    Energy Technology Data Exchange (ETDEWEB)

    Fugloe, Hanna Maria; Hovgaard, Dorrit; Petersen, Michael M. [Copenhagen University Hospital, Department of Orthopaedic Surgery, Rigshospitalet, Copenhagen Oe (Denmark); Joergensen, Simon Moeller; Loft, Annika [Copenhagen University Hospital, Department of Clinical Physiology, Nuclear Medicine and PET, Rigshospitalet, Copenhagen (Denmark)

    2012-09-15

    To evaluate the feasibility of {sup 18}F-FDG PET/CT for initial assessment in high-grade bone sarcomas (BS) and soft tissue sarcomas (STS). During the years 2001-2010, 89 patients (30 BS, 59 STS) referred for further evaluation and surgical treatment of a high-grade BS or STS also had a PET/CT scan performed for staging preoperatively (n = 68) or within 1 month of surgery (n = 21). Metastatic lesions suggested on the PET/CT scan were confirmed or rejected by histological evaluation, by additional imaging or by follow-up. In 68 patients (28 BS, 40 STS) the relationship between the maximal standardized uptake value (SUVmax) of the primary tumour and survival was examined. The PET/CT scan suggested the presence of 13 metastatic lesions in BS patients (5 lymph node, 8 distant) and 21 metastatic lesions (6 lymph node, 15 distant) in STS patients. The calculated sensitivity (SE) and specificity (SP) were 95 % and 96 % for detection of distant metastases, and the predictive value (PV) of a positive or a negative test was 87 % and 98 %, respectively. SE and SP were 100 % and 90 % for detection of lymph node metastases, and the PV of a positive or a negative test was 27 % and 100 %, respectively. The 5-year survival was 81 % among patients with SUVmax below the median value ({<=}10), but was 33 % among those with SUVmax >10. FDG PET/CT for the initial assessment of patients with high-grade BS or STS was feasible with high SE and SP, but in those with lymph node metastases the PV of a positive test was low. The SUVmax of the primary tumour was a strong prognostic factor for survival. (orig.)

  3. The diagnostic and prognostic value of 18F-FDG PET/CT in the initial assessment of high-grade bone and soft tissue sarcoma. A retrospective study of 89 patients

    International Nuclear Information System (INIS)

    To evaluate the feasibility of 18F-FDG PET/CT for initial assessment in high-grade bone sarcomas (BS) and soft tissue sarcomas (STS). During the years 2001-2010, 89 patients (30 BS, 59 STS) referred for further evaluation and surgical treatment of a high-grade BS or STS also had a PET/CT scan performed for staging preoperatively (n = 68) or within 1 month of surgery (n = 21). Metastatic lesions suggested on the PET/CT scan were confirmed or rejected by histological evaluation, by additional imaging or by follow-up. In 68 patients (28 BS, 40 STS) the relationship between the maximal standardized uptake value (SUVmax) of the primary tumour and survival was examined. The PET/CT scan suggested the presence of 13 metastatic lesions in BS patients (5 lymph node, 8 distant) and 21 metastatic lesions (6 lymph node, 15 distant) in STS patients. The calculated sensitivity (SE) and specificity (SP) were 95 % and 96 % for detection of distant metastases, and the predictive value (PV) of a positive or a negative test was 87 % and 98 %, respectively. SE and SP were 100 % and 90 % for detection of lymph node metastases, and the PV of a positive or a negative test was 27 % and 100 %, respectively. The 5-year survival was 81 % among patients with SUVmax below the median value (≤10), but was 33 % among those with SUVmax >10. FDG PET/CT for the initial assessment of patients with high-grade BS or STS was feasible with high SE and SP, but in those with lymph node metastases the PV of a positive test was low. The SUVmax of the primary tumour was a strong prognostic factor for survival. (orig.)

  4. Adult Intramedullary Ewing Sarcoma of the Proximal Hip

    Directory of Open Access Journals (Sweden)

    Preetam Gongidi

    2014-01-01

    Full Text Available Ewing sarcoma of bone is classically a permeative lesion in the diaphysis of long bones in children. While they occur primarily in children and adolescents, they can be seen in young adults in their 20s, but these are typically seen in flat bones. The permeative nature of the lesion can elicit new bone formation creating a partially sclerotic appearance, cortical expansion presenting as a “Codman triangle,” or have an “onion-skin” type of aggressive periosteal reaction/periostitis. Ewing sarcoma is rarely seen without an associated soft-tissue mass and is even rarer to just have benign-appearing periostitis (e.g., thick, uniform, or wavy cortex. We present such a case of Ewing sarcoma in a young adult confined to just the medullary metadiaphysis without cortical erosion or soft-tissue mass. To the best of our knowledge, this is the first case to be reported in the radiology literature.

  5. Adult intramedullary ewing sarcoma of the proximal hip.

    Science.gov (United States)

    Gongidi, Preetam; Jasti, Siva; Rafferty, William; Barshay, Veniamin; Lackman, Richard

    2014-01-01

    Ewing sarcoma of bone is classically a permeative lesion in the diaphysis of long bones in children. While they occur primarily in children and adolescents, they can be seen in young adults in their 20s, but these are typically seen in flat bones. The permeative nature of the lesion can elicit new bone formation creating a partially sclerotic appearance, cortical expansion presenting as a "Codman triangle," or have an "onion-skin" type of aggressive periosteal reaction/periostitis. Ewing sarcoma is rarely seen without an associated soft-tissue mass and is even rarer to just have benign-appearing periostitis (e.g., thick, uniform, or wavy cortex). We present such a case of Ewing sarcoma in a young adult confined to just the medullary metadiaphysis without cortical erosion or soft-tissue mass. To the best of our knowledge, this is the first case to be reported in the radiology literature. PMID:25024862

  6. Diagnosis and treatment of sarcomas and related tumors

    Energy Technology Data Exchange (ETDEWEB)

    1978-01-01

    The Cancergram focuses on clinical aspects of sarcomas involving soft tissue and bone, and also includes abstracts on related malignant and benign tumors. Soft tissues are considered as all non-epithelial extra-skeletal tissues of the body, with the exception of the reticuloendothelial system, the neuroglia, and visceral and parenchymal organs. Included, therefore, are sarcomas of the vascular system, fatty tissue, muscle tissues, connective tissues, and synovial tissues. Bone tumors included are osteosarcoma, chondrosarcoma, adamantinoma, chrondroblastoma, Ewing's sarcoma, and other benign and malignant disorders of the bone. This Cancergram excludes disorders of the bone marrow, which are the subjects of separate Cancergrams (see series CT03 for leukemias, and series CT12 for multiple myeloma). The scope includes diagnosis and staging, supportive care, evaluation, and therapy. Selected abstracts concerning epidemiology, etiology and other pre-clinical studies will also be included where they have direct clinical relevance.

  7. Pediatric Soft Tissue Sarcomas

    OpenAIRE

    Loeb, David M.; Thornton, Katherine; Shokek, Ori

    2008-01-01

    Soft tissue sarcomas in children are relatively rare. Approximately 850 to 900 children and adolescents are diagnosed each year with rhabdomyosarcoma (RMS) or one of the non-RMS soft tissue sarcomas (NRSTS). Of these, 350 are cases of RMS. RMS is the most common soft tissue sarcoma in children 14 years old and younger, and NRSTS is more common in adolescents and young adults. Infants also get NRSTS, but their tumors constitute a distinctive set of histologies, including infantile fibrosarcoma...

  8. Epidural spinal cord compression as initial clinical presentation of an acute myeloid leukaemia: case report and literature review

    Institute of Scientific and Technical Information of China (English)

    Dominique N'Dri Oka; Alpha Boubacar Bah; André Valentin Tokpa; Louis Derou

    2016-01-01

    Epidural localization of myeloid leukaemia is rarely reported.Spinal cord compression as an initial presentation of acute myeloid leukaemia is extremely rare.This is a report of a 17-year-old black boy who presented to emergency department with neurological symptoms of spinal cord compression.Imaging modalities showed multiple soft tissue masses in the epidural space.After surgical treatment,histopathological examination of the epidural mass showed myeloid leukaemia cells infiltration.Literature review on Medline and "scholar Google" database was done.The characteristics and management of extra-medullary leukaemia are discussed.Granulocytic sarcoma,myeloid sarcoma or chloroma with acute myeloid leukaemia should be considered as part of epidural spinal cord compression.Therefore surgery is indicated on an emergent basis.

  9. Bone tumors: Nursing care

    International Nuclear Information System (INIS)

    Bone tumors represent approximately 5% of childhood malignancies. osteosarcoma is the primary malignant bone tumor, accounting for 60% of cancer with peak incidence in the 2nd decade of life. Ewing's sarcoma is the second most common bone cancer with peak at a slightly younger age. This presentation discusses similarities and differences in the diagnosis and treatment of these two malignancies. Diagnostic procedures include plain radiographs, CT and MRI of the primary site, plain x-ray and CT of the chest, bone scan, and biopsy of the primary tumor. For patients diagnosed with Ewing's sarcoma, a bone marrow aspirate and biopsy will also be required. Our current approach to the treatment of bone tumors includes preoperative combination chemotherapy and en bloc surgical removal of the tumor followed by postoperative chemotherapy. In the case of Ewing's sarcoma, radiation therapy may be employed in addition to surgery, if margins are questionable of instead of surgery, if the tumor is not resectable

  10. Unilateral eyelid swelling, proptosis and diplopia as initial manifestation of acute myeloid leukemia

    OpenAIRE

    Chaudhry, Imtiaz A.; Alaraj, Ahmad M.; Hind M Alkatan

    2012-01-01

    Myeloid sarcoma is a tumor of immature myeloid cells occurring in many extramedullary sites, orbit being one of them where the tumor may occur prior to or after the diagnosis of underlying disease. We report a case of a 17-year-old male who presented with upper eyelid swelling, proptosis and diplopia after presumed blunt trauma without any other clinical signs and symptoms. Initial imaging suggested possibility of subperiosteal hematoma. Magnetic resonance imaging studies demonstrated a solid...

  11. Pathologic and Protective Roles for Microglial Subsets and Bone Marrow- and Blood-Derived Myeloid Cells in Central Nervous System Inflammation

    DEFF Research Database (Denmark)

    Wlodarczyk, Agnieszka; Cédile, Oriane; Jensen, Kirstine Nolling;

    2015-01-01

    Inflammation is a series of processes designed for eventual clearance of pathogens and repair of damaged tissue. In the context of autoimmune recognition, inflammatory processes are usually considered to be pathological. This is also true for inflammatory responses in the central nervous system...... (CNS). However, as in other tissues, neuroinflammation can have beneficial as well as pathological outcomes. The complex role of encephalitogenic T cells in multiple sclerosis and its animal model experimental autoimmune encephalomyelitis (EAE) may derive from heterogeneity of the myeloid cells with...... three populations of myeloid cells: CD11c(+) microglia, CD11c(-) microglia, and CD11c(+) blood-derived cells in terms of their pathological versus protective functions in the CNS of mice with EAE. Our data show that CNS-resident microglia include functionally distinct subsets that can be distinguished...

  12. Postirradiation sarcoma (malignant fibrous histiocytoma) following cervix cancer

    International Nuclear Information System (INIS)

    A case of postirradiation sarcoma is described. The tumor, a malignant fibrous histiocytoma, occurred in the radiation field 11 years following postoperative external beam radiation therapy (7,000 rad) for carcinoma of the cervix. Reports of postirradiation malignant fibrous histiocytoma are rare, and the occurrence of this neoplasm following treatment for cervix cancer has not previously been described. The literature concerning postirradiation bone and soft tissue sarcomas is briefly reviewed, with special attention to malignant fibrous histiocytomas. (author)

  13. Osteogenic sarcoma with skeletal muscle metastases

    Energy Technology Data Exchange (ETDEWEB)

    Peh, W.C.G. [Department of Diagnostic Radiology, The University of Hong Kong, Queen Mary Hospital (Hong Kong); Shek, T.W.H. [Department of Pathology, The University of Hong Kong, Queen Mary Hospital (Hong Kong); Wang Shihchang [Department of Diagnostic Imaging, National University of Singapore, National University Hospital (Singapore); Wong, J.W.K.; Chien, E.P. [Department of Orthopaedic Surgery, The University of Hong Kong, Queen Mary Hospital (Hong Kong)

    1999-05-01

    Two cases of osteogenic sarcoma with skeletal muscle metastases are described. A 40-year-old woman presented with progressive swelling of both calves and a soft tissue back lump. She had been diagnosed with mandibular chondroblastic osteogenic sarcoma 6 years earlier. Radiographs showed calcified masses. MRI scans and bone scintigraphy revealed multiple soft tissue masses in both calves. Bone scintigraphy also showed uptake in the back lump, right thigh and left lung base. Biopsy confirmed metastatic chondroblastic osteogenic sarcoma, which initially responded well to chemotherapy. However, the metastatic disease subsequently progressed rapidly and she died 21 months after presentation. The second case concerns a 20-year-old man who presented with a pathologic fracture of the humerus, which was found to be due to osteoblastic osteogenic sarcoma. He developed cerebral metastases 17 months later, followed by metastases at other sites. Calcified masses were subsequently seen on radiographs of the abdomen and chest. CT scans confirmed the presence of densely calcified muscle metastases in the abdominal wall, erector spinae and gluteal muscles. The patient`s disease progressed rapidly and he died 30 months after presentation. (orig.) With 6 figs., 29 refs.

  14. [Primary pulmonary sarcomas].

    Science.gov (United States)

    Jakubcová, T; Jakubec, P

    2009-01-01

    Primary pulmonary sarcomas are rare diseases unlike lung carcinomas. The occurence of these sarcomas is between 0.013-0.40% of all malignant lung tumours. There are malignant mesenchymal tumours. They are flowing from the soft tissue of lung. The pulmonary sarcomas are heterogenic group with various biological behaviour. Their morfologic structure does not digger from the sarcomas of soft tissue. The primary pulmonary sarcomas occur more often in childhood and in young people unlike lung carcinomas. Radiation and some toxic substances are noted risk factors. Some gene mutations, infectious pathoghens and contraception have a possible impact on the origin of some types of the sarcomas. The current hypothesis is, that most of the sarcomas, if not all sarcomas, stem from primitive multipotent mesenchymal cell by malignant transformation in one or more lines. The diagnostic standard is biopsy from tumour with histologic and immunohistochemistry examination of a sample. The basic diagnostic problem is exclusion of a secondary origin of sarcomatic cells in the lung, because pulmonary metastasis of extrapulmonary sarcomas are more often than the primary pulmonary involvement.The optimal treatment is a resection of the tumour.The other therapeutic modalities are radiotherapy and chemotherapy, but results of these modalities are unsatisfactory. There are various chemotherapeutic regimes, monotherapy or combination regimes. The basic cytostatics are doxorubicine, iphosphamide, dacarbazine. Problems of the chemotherapy are high toxicity and relatively low curative effect about 20%.The first studies with biological treatment of the sarcomas of soft tissue have been published recently.This types of drugs could be a part of the complex management of these primary pulmonary tumours in the future. The primary pulmonary sarcomas have mostly aggresive course and often recur. Their prognosis is usually not very good. The survival median is 48 months and 5-years survival ranges

  15. Current state of pediatric sarcoma biology and opportunities for future discovery: A report from the sarcoma translational research workshop.

    Science.gov (United States)

    Hingorani, Pooja; Janeway, Katherine; Crompton, Brian D; Kadoch, Cigall; Mackall, Crystal L; Khan, Javed; Shern, Jack F; Schiffman, Joshua; Mirabello, Lisa; Savage, Sharon A; Ladanyi, Marc; Meltzer, Paul; Bult, Carol J; Adamson, Peter C; Lupo, Philip J; Mody, Rajen; DuBois, Steven G; Parsons, D Williams; Khanna, Chand; Lau, Ching; Hawkins, Douglas S; Randall, R Lor; Smith, Malcolm; Sorensen, Poul H; Plon, Sharon E; Skapek, Stephen X; Lessnick, Stephen; Gorlick, Richard; Reed, Damon R

    2016-05-01

    Sarcomas are a rare subgroup of pediatric cancers comprised of a variety of bone and soft-tissue tumors. While significant advances have been made in improving outcomes of patients with localized pediatric sarcomas since the addition of systemic chemotherapy to local control many decades ago, outcomes for patients with metastatic and relapsed sarcoma remain poor with few novel therapeutics identified to date. With the advent of new technologies to study cancer genomes, transcriptomes and epigenomes, our understanding of sarcoma biology has improved tremendously in a relatively short period of time. However, much remains to be accomplished in this arena especially with regard to translating all of this new knowledge to the bedside. To this end, a meeting was convened in Philadelphia, PA, on April 18, 2015 sponsored by the QuadW foundation, Children's Oncology Group and CureSearch for Children's Cancer that brought together sarcoma clinicians and scientists from North America to review the current state of pediatric sarcoma biology and ongoing/planned genomics based clinical trials in an effort to identify and bridge knowledge gaps that continue to exist at present. At the conclusion of the workshop, three key objectives that would significantly further our understanding of sarcoma were identified and a proposal was put forward to develop an all-encompassing pediatric sarcoma biology protocol that would address these specific needs. This review summarizes the proceedings of the workshop. PMID:27132463

  16. Granulocytic Sarcoma as the First Sign of Acute Leukemia in Childhood

    Directory of Open Access Journals (Sweden)

    Aalia R Sufi

    2012-01-01

    Full Text Available Acute myeloid leukemia (AML may rarely involve the orbit as a solid tumor termed granulocytic sarcoma. This report describes the case of a child who presented with rapidly progressive unilateral proptosis and was diagnosed as rhabdomyosarcoma. However subsequent examination of the peripheral blood film revealed AML. Thus proptosis may present as the initial manifestation of AML.

  17. Ewing sarcoma versus osteomyelitis: differential diagnosis with magnetic resonance imaging

    International Nuclear Information System (INIS)

    To find and evaluate characteristic magnetic resonance imaging (MRI) patterns for the differentiation between Ewing sarcoma and osteomyelitis. We identified 28 consecutive patients referred to our department for MRI (1.5 T) of an unclear bone lesion with clinical symptoms suggestive of Ewing sarcoma or osteomyelitis. MRI scans were re-evaluated by two experienced radiologists, typical MR imaging features were documented and a diagnostic decision between Ewing sarcoma and osteomyelitis was made. Statistical significance of the association between MRI features and the biopsy-based diagnosis was assessed using Fisher's exact test. The most clear-cut pattern for determining the correct diagnosis was the presence of a sharp and defined margin of the bone lesion, which was found in all patients with Ewing sarcoma, but in none of the patients with osteomyelitis (P < 0.0001). Contrast enhancing soft tissue was present in all cases with Ewing sarcoma and absent in 4 patients with osteomyelitis (P = 0.0103). Cortical destruction was found in all patients with Ewing sarcoma, 4 patients with osteomyelitis did not present any cortical reaction (P = 0.0103). Cystic or necrotic areas were identified in 13 patients with Ewing sarcoma and in 1 patient with osteomyelitis (P = 0.004). Interobserver reliability was very good (kappa = 1) in Ewing sarcoma and moderate (kappa = 0.6) in patients with osteomyelitis. A sharp and defined margin, optimally visualized on T1-weighted images in comparison to short tau inversion recovery (STIR) images, is the most significant feature of Ewing sarcoma in differentiating from osteomyelitis. (orig.)

  18. Ewing sarcoma versus osteomyelitis: differential diagnosis with magnetic resonance imaging

    Energy Technology Data Exchange (ETDEWEB)

    Henninger, B.; Glodny, B.; Rudisch, A.; Trieb, T.; Loizides, A.; Judmaier, W.; Schocke, M.F. [Innsbruck Medical University, Department of Radiology, Innsbruck (Austria); Putzer, D. [Innsbruck Medical University, Department of Nuclear Medicine, Innsbruck (Austria)

    2013-08-15

    To find and evaluate characteristic magnetic resonance imaging (MRI) patterns for the differentiation between Ewing sarcoma and osteomyelitis. We identified 28 consecutive patients referred to our department for MRI (1.5 T) of an unclear bone lesion with clinical symptoms suggestive of Ewing sarcoma or osteomyelitis. MRI scans were re-evaluated by two experienced radiologists, typical MR imaging features were documented and a diagnostic decision between Ewing sarcoma and osteomyelitis was made. Statistical significance of the association between MRI features and the biopsy-based diagnosis was assessed using Fisher's exact test. The most clear-cut pattern for determining the correct diagnosis was the presence of a sharp and defined margin of the bone lesion, which was found in all patients with Ewing sarcoma, but in none of the patients with osteomyelitis (P < 0.0001). Contrast enhancing soft tissue was present in all cases with Ewing sarcoma and absent in 4 patients with osteomyelitis (P = 0.0103). Cortical destruction was found in all patients with Ewing sarcoma, 4 patients with osteomyelitis did not present any cortical reaction (P = 0.0103). Cystic or necrotic areas were identified in 13 patients with Ewing sarcoma and in 1 patient with osteomyelitis (P = 0.004). Interobserver reliability was very good (kappa = 1) in Ewing sarcoma and moderate (kappa = 0.6) in patients with osteomyelitis. A sharp and defined margin, optimally visualized on T1-weighted images in comparison to short tau inversion recovery (STIR) images, is the most significant feature of Ewing sarcoma in differentiating from osteomyelitis. (orig.)

  19. Local recurrence, rate and sites of metastases, and time to relapse as a function of treatment regimen, size of primary and surgical history in 62 patients presenting with non-metastatic Ewing's sarcoma of the pelvic bones

    International Nuclear Information System (INIS)

    This report reviews the experience of 62 patients who presented between 1972 and 1978 with non-metastatic Ewing's sarcoma of the pelvis and were entered on IESS I. Seventeen patients (27%) developed a local recurrence, 38 patients (61%) demonstrated metastases and 21 (34%) neither. In the dose range 4000 rad to 6000 rad no dose response could be detected for local control of tumor. Forty-six patients (74%) had a biopsy or exploratory surgery only, 5 patients (8%) had an incomplete resection and 11 patients (18%) has a complete resection of their tumor. In the 46 patients having a biopsy only, 13 developed a local recurrence (28%) as compared to 2 of 11 patients undergoing a complete resection (18%). The most common sites for metastases were lung in 19 patients (31%) and bone in 23 patients (37%). No significant difference was noted in the frequency of overall metastases or metastases to any site between those patients receiving one of the three treatment regimens used in IESS I: VAC and Adriamycin (regime I), VAC alone (regimen II) and VAC plus bilateral pulmonary irradiation (regimen III). At a median follow-up of 135 weeks no significant difference in median survival could be detected in patients with pelvic primaries between regimens I, II and III. The possible reasons for the poor prognosis of pelvic primary patients are discussed together with treatment policies that might improve the survival of this group of patients

  20. Identifying Targetable Liabilities in Ewing Sarcoma

    OpenAIRE

    Vallurupalli, Mounica

    2014-01-01

    Background: Despite multi-modality therapy, the majority of patients with metastatic or recurrent Ewing sarcoma (ES), the second most common pediatric bone malignancy, will die of their disease. ES tumors express aberrantly activated ETS transcription factors through translocations that fuse the EWS gene to ETS family genes FLI1 or ERG. The aberrant activation of ETS transcription factors promotes malignant transformation and proliferation. While, FLI1 or ERG cannot be readily targeted, ther...

  1. Isolated pancreatic granulocytic sarcoma: A case report and review of the literature

    Institute of Scientific and Technical Information of China (English)

    Xin-Ping Li; Wen-Fang Liu; Shu-Rong Ji; She-Hua Wu; Jian-Jun Sun; Yue-Zu Fan

    2011-01-01

    Granulocytic sarcoma (GS) is an extramedullary tumor mass consisting of immature myeloid cells. Isolated pancreatic granulocyte sarcoma is extremely rare. We report a very unusual pancreatic granulocytic sarcoma in a patient without acute myeloid leukemia. The patient presented with acute epigastric pain because of splenic infarction due to a mass consisting of myeloblasts in the pancreatic tail. The patients underwent splenectomy and distal pancreatectomy. Pathology and immunohistochemistry suggested a GS. Despite local surgery, an isolated tumor recurred 2 mo after operation and the patient died 3 mo after removal of the tumor. Only 7 reported cases of pancreatic GS were identified in the literature and the mass was located in the pancreatic head. This is the first report of GS in the pancreatic tail with splenic infarction.

  2. Isolated Biliary Granulocytic Sarcoma Followed by Acute Myelogeneous Leukemia with Multilineage Dysplasia: A Case Report and Literature Review

    OpenAIRE

    Sung, Chang Ohk; Ko,Young Hyeh; Park, Cheol Keun; Jang, Kee Taek; Heo, Jin Seok

    2006-01-01

    Granulocytic sarcoma is a rare extramedullary tumor composed of myeloid progenitor cells. Primary involvement of the biliary tract without evidence of leukemia is exceedingly rare. Here, we report an isolated biliary granulocytic sarcoma in a 30-yr-old man who presented with jaundice, fever, and chill without any evidence of leukemia. However, five months after the diagnosis, he developed acute myelogenous leukemia with multilineage dysplasia and chromosomal abnormality. A rare possibility of...

  3. Bone

    International Nuclear Information System (INIS)

    Bone scanning provides information on the extent of primary bone tumors, on possible metastatic disease, on the presence of osteomyelitis prior to observation of roentgenographic changes so that earlier therapy is possible, on the presence of collagen diseases, on the presence of fractures not disclosed by x-ray films, and on the evaluation of aseptic necrosis. However, the total effect and contribution of bone scanning to the diagnosis, treatment, and ultimate prognosis of pediatric skeletal diseases is, as yet, unknown. (auth)

  4. Elusive identities and overlapping phenotypes of proangiogenic myeloid cells in tumors.

    Science.gov (United States)

    Coffelt, Seth B; Lewis, Claire E; Naldini, Luigi; Brown, J Martin; Ferrara, Napoleone; De Palma, Michele

    2010-04-01

    It is now established that bone marrow-derived myeloid cells regulate tumor angiogenesis. This was originally inferred from studies of human tumor biopsies in which a positive correlation was seen between the number of tumor-infiltrating myeloid cells, such as macrophages and neutrophils, and tumor microvessel density. However, unequivocal evidence was only provided once mouse models were used to examine the effects on tumor angiogenesis by genetically or pharmacologically targeting myeloid cells. Since then, identifying the exact myeloid cell types involved in this process has proved challenging because of myeloid cell heterogeneity and the expression of overlapping phenotypic markers in tumors. As a result, investigators often simply refer to them now as "bone marrow-derived myeloid cells." Here we review the findings of various attempts to phenotype the myeloid cells involved and discuss the therapeutic implications of correctly identifying-and thus being able to target-this proangiogenic force in tumors. PMID:20167863

  5. Ulnar joint synovial sarcoma

    International Nuclear Information System (INIS)

    Statement of the diagnosis of synovial sarcoma in early period on the example of one patient is analyzed. It is too difficult to diagnose synovial sarcoma. Comprehensive X-ray ultrasonic examination is the most informative in detection of tumor. Surgical removal of revealed formation has been conducted to the patient. The formation is dense, joined to muscular fibers, is not connected with the joint. Histological investigation of the formation determines dissemination of malignant tumor, presented by round or oval cells. Tumor tissue is devided by thick fibrous layers, surrounded by fibrous capsula. The following conclusion is given: synovial sarcoma

  6. Primary renal synovial sarcoma

    OpenAIRE

    Bakhshi, Girish D.; Arshad S. Khan; Shaikh, Aftab S; Khan, Mohammad Ashraf A.; Mohammad Adil A. Khan; Jamadar, Nilofar M.

    2012-01-01

    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 u...

  7. Spinal granulocytic sarcoma (chloroma presenting as acute cord compression in a nonleukemic patient

    Directory of Open Access Journals (Sweden)

    Kalayci Murat

    2005-01-01

    Full Text Available The case of a previously healthy 24-year-old man diagnosed with extradural thoracic granulocytic sarcoma with no evidence of bone marrow or other hematological involvement is described. The tumor was removed totally by microsurgery. The histopathological examination was consistent with granulocytic sarcoma. Granulocytic sarcomas are most commonly found in the context of an acute myelogenous leukemia or in chronic myelogenous leukemia. They rarely have been reported in otherwise healthy patients without any evidence of systemic disease. A review of the literature revealed only 14 more nonleukaemic cases with granulocytic sarcoma causing thoracic spinal cord compression.

  8. Recent advances in targeted therapy for Ewing sarcoma [version 1; referees: 2 approved

    Directory of Open Access Journals (Sweden)

    Kathleen I. Pishas

    2016-08-01

    Full Text Available Ewing sarcoma is an aggressive, poorly differentiated neoplasm of solid bone that disproportionally afflicts the young. Despite intensive multi-modal therapy and valiant efforts, 70% of patients with relapsed and metastatic Ewing sarcoma will succumb to their disease. The persistent failure to improve overall survival for this subset of patients highlights the urgent need for rapid translation of novel therapeutic strategies. As Ewing sarcoma is associated with a paucity of mutations in readily targetable signal transduction pathways, targeting the key genetic aberration and master regulator of Ewing sarcoma, the EWS/ETS fusion, remains an important goal.

  9. Classic Kaposi Sarcoma

    Science.gov (United States)

    ... in the United States have been diagnosed in homosexual or bisexual men infected with HIV. Signs of ... type of nonepidemic Kaposi sarcoma that develops in homosexual men who have no signs or symptoms of ...

  10. Epidemic Kaposi Sarcoma

    Science.gov (United States)

    ... in the United States have been diagnosed in homosexual or bisexual men infected with HIV. Signs of ... type of nonepidemic Kaposi sarcoma that develops in homosexual men who have no signs or symptoms of ...

  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. Feline injection site sarcomas

    OpenAIRE

    Nóbrega, C.; Mesquita, Jr.; Cruz, R; C. Coelho; Esteves, F.; Mega, A. C.; Santos, C.; Vala, Helena

    2016-01-01

    Feline injection site sarcoma (FISS), formerly known as Vaccine Associated Sarcoma (VAS) is a rare, but life-threatening disease. The incidence estimates have varied from 1 case of FISS per 1,000-10,000 cats vaccinated in North America and between 1 per 5,000-12,500 vaccination visits in United Kingdom. It has been primarily associated with vaccine administration, but several other injectable materials/substances have been implicated as aetiologic agents, namely lufenuron, penicilin, meti...

  13. Chronic osteomyelitis mimicking sarcoma

    OpenAIRE

    Gulmann, C; Young, O.; Tolan, M.; O’Riordan, D.; Leader, M

    2003-01-01

    This report describes a rare case of chronic osteomyelitis in a 60 year old man mimicking a soft tissue sarcoma. Chronic osteomyelitis is an infrequent cause of a soft tissue mass and is usually diagnosed clinically by a combination of radiology and microbiology. Rarely, COM can mimic a primary bony neoplasm, but this is the first reported case where it mimicked a soft tissue sarcoma. The clinical, radiological, and histological appearances of this case will be discussed.

  14. Positron emission tomography with fluorine-deoxyglucose in sarcomas and non-sarcoma non-epithelial tumors

    International Nuclear Information System (INIS)

    Background: The usefulness of positron emission tomography (PET) with fluorine-deoxyglucose (FDG) in sarcomas and non-sarcoma non-epithelial (NSNE) tumors is not clearly defined. Aim: To report a Chilean experience with NSNE tumors evaluated using PET with FDG. Material and Methods: Retrospective review of the database of a PET laboratory. Demographic data, indications and metabolic findings were compared with conventional imaging in 88 adults and children with diverse bone and soft tissue sarcomas as well as 24 gastrointestinal stromal tumors (GIST), 6 pleural malignant mesotheliomas in adults, and 9 medulloblastomas in children. Results: FDG showed good concordance with conventional imaging in NSNE tumors. It was helpful for staging, restaging, follow-up after treatment and for the detection of new not previously suspected lesions. Conclusions: PET with FDG could have a prognostic role and help in patient management, mainly in musculoskeletal and high grade or less differentiated sarcomas. In GIST, it was a good tool for immunotherapy control

  15. Carbon ion radiotherapy for sarcomas

    International Nuclear Information System (INIS)

    Principles of heavy ion therapy, its application to bone and soft tissue sarcomas and outline of its general state are described. The heavy ion therapy has advantages of its high dose distribution to the target and strong biological effect due to the Bragg peak formation and high linear energy transfer, respectively. The authors use carbon ion generated by Heavy Ion Medical Accelerator in Chiba (HIMAC) for the therapy of performance state 0-2 patients with the sarcomas unresectable, diagnosed pathologically, and of 60 y, 45% and teens, 8%) have been treated, whose tumor site has been the pelvis in 73%, volume >600 mL in 63%, tissue type of bone tumor in 70% (where cordoma has amounted to>200 cases). Five-year local control rate is found 71% and survival, 59%. In 175 therapeutically fresh cases with sacral cordoma of median age 67 y, with median clinical target volume 9 cm, treated with median dose 70.4 GyE/16 irradiations, the 8-y local control rate is found to be 69% and survival, 74%, within the median follow-up 54 months; with severe skin ulcer in 2 cases and deterioration of nervous dysfunction in 15 cases; suggesting the therapy is as effective and useful as surgical resection. At present, the therapy is not applicable to Japan health insurance. In the author's hospital, the heavy ion therapy has been conducted to total of >6,000 patients, which amounting to the largest number in the world. Now, 3 Japanese facilities can do the therapy as well and 3 countries in the world.(T.T.)

  16. Histogenesis of mouse sarcomas induced by implantation of polyvinyl chloride film in radiation chimeras

    International Nuclear Information System (INIS)

    Sarcomas were induced in CBA/CBA-T6T6 mouse radiation chimeras by implantation of polyvinyl chloride film subcutaneously 13 months after irradiation and injection of donor's bone marrow. Of the 12 tumors studied 11 had the recipient's karyotype and one the donor's. The formation of connective-tissue cells from bone-marrow precursors thus, evidently does not play an essential role in the histogenesis of sarcomas induced by plastics

  17. The Relationship between Clinical Feature, Complex Immunophenotype, Chromosome Karyotype, and Outcome of Patients with Acute Myeloid Leukemia in China

    OpenAIRE

    Bingjie Ding; Lanlan Zhou; Xuejie Jiang; Xiaodong Li; Qingxiu Zhong; Zhixiang Wang; Zhengshan Yi; Zhongxin Zheng; Changxin Yin; Rui Cao; Libin Liao; Fanyi Meng

    2015-01-01

    Mixed phenotype acute leukemia (MPAL) is a complex entity expressing both lymphoid and myeloid immunophenotyping. In the present study, 47 MPAL, 60 lymphoid antigen-positive acute myeloid leukemia (Ly+AML), and 90 acute myeloid leukemia with common myeloid immunophenotype (Ly−AML) patients were investigated. We found that, in MPAL patients, there were high proportions of blast cells in bone marrow and incidence of hepatosplenomegaly, lymphadenopathy, and Philadelphia chromosome. The overall s...

  18. Clinical and prognostic significance of PD-1 and PD-L1 expression in sarcomas.

    Science.gov (United States)

    Paydas, Semra; Bagir, Emine Kilic; Deveci, Mehmet Ali; Gonlusen, Gulfiliz

    2016-08-01

    Programmed death-1 (PD-1) and programmed death-ligand 1 (PD-L1) are new targets in cancer immunotherapy in recent years. The aim of this study is to evaluate the PD-1/PD-L1 expressions in sarcomas and to determine association between PD-1/PD-L1 expressions and clinical/pathological properties in some sarcoma subtypes. Formalin-fixed, paraffin-embedded tissue samples from 65 cases with sarcomas were analyzed. Immunohistochemical staining was performed to detect the PD-1 and PD-L1 expressions in tumor tissue and microenvironment, separately. PD-1 expression in tumor tissue and microenvironment was detected in 11 (17 %) and 8 (12 %) cases, respectively. PD-L1 expression in tumor tissue and microenvironment was detected in 19 (29 %) and 20 cases (30 %), respectively. None of the 5 Ewing sarcomas involving bone showed PD-1/PD-L1 expression, while 2 of 3 cases with Ewing sarcomas involving soft tissue showed PD-1 and PD-L1 expression. Among 5 cases with Kaposi sarcoma, four showed PD-1 and/or PD-L1 expression in tumor or microenvironment. PD-1/PD-L1 expressions were detected 3 of 6 cases with pleomorphic sarcoma, 2 of 4 cases with peripheral nerve sheath tumors and 1 of 4 cases with synovial sarcoma. Interestingly, strongest PD-1/PD-L1 expressions in our study group were detected in 2 sarcoma cases with the history of giant cell tumor. PD-1 and PD-L1 expressions are up to 30 % of the cases with sarcomas. It may be rational to target programmed death pathway in Kaposi sarcoma, pleomorphic sarcoma and peripheral nerve sheath tumors. Strong expression of PD-1/PD-L1 in cases with previous giant cell bone tumor has been found to be interesting and must be studied in giant cell tumor samples. PMID:27421997

  19. A high proportion of bone marrow T cells with regulatory phenotype (CD4+CD25hiFoxP3+) in Ewing sarcoma patients is associated with metastatic disease.

    Science.gov (United States)

    Brinkrolf, Peter; Landmeier, Silke; Altvater, Bianca; Chen, Christiane; Pscherer, Sibylle; Rosemann, Annegret; Ranft, Andreas; Dirksen, Uta; Juergens, Heribert; Rossig, Claudia

    2009-08-15

    Immunosuppressive CD4+CD25(hi)FoxP3+ T cells (T(reg) cells) have been found at increased densities within the tumor microenvironment in many malignancies and interfere with protective antitumor immune responses. Osseous Ewing sarcomas (ESs) are thought to derive from a bone marrow (BM) mesenchymal cell of origin, and microscopic marrow involvement defines a subpopulation of patients at a high risk of relapse. We hypothesized that BM-resident T cells may contribute to a permissive milieu for immune escape of ESs. Using 6-color-flow cytometry, we investigated the pattern of immune cell subset distribution including NK cells, gammadelta T cells, central and effector memory CD8+ and CD4+ T cells as well as T cells with regulatory phenotype (T(reg) cells) in BM obtained at diagnosis from 45 primary or relapsed ES patients treated within standardized protocols. Although patients at relapse had an inverted CD4:CD8 T-cell ratio, neither CD8+ effector/memory T-cell subsets nor T(reg) cells significantly differed from patients at diagnosis. No significant associations of innate and effector/memory T-cell subpopulations with known risk factors were found, including age, gender, tumor site, primary metastases and histological tumor response. By contrast, T(reg) cells were found at significantly higher frequencies in patients with primary metastatic disease compared with localized ESs (5.0 vs. 3.3%, p = 0.01). Thus, increased BM T(reg) cells in patients with metastasized ES may reflect an immune escape mechanism that contributes to the development of metastatic disease. Immunotherapeutic strategies will have to adequately consider the regulatory milieu within areas of Ewing tumor-immune interactions. PMID:19480009

  20. Myeloid leukemia after hematotoxins

    Energy Technology Data Exchange (ETDEWEB)

    Larson, R.A.; LeBeau, M.M.; Vardiman, J.W.; Rowley, J.D. [Univ. of Chicago, IL (United States)

    1996-12-01

    One of the most serious consequences of cancer therapy is the development of a second cancer, especially leukemia. Several distinct subsets of therapy-related leukemia can now be distinguished. Classic therapy-related myeloid leukemia typically occurs 5 to 7 years after exposure to alkylating agents and/or irradiation, has a myelodysplastic phase with trilineage involvement, and is characterized by abnormalities of the long arms of chromosomes 5 and/or 7. Response to treatment is poor, and allogeneic bone marrow transplantation is recommended. Leukemia following treatment with agents that inhibit topoisomerase 11, however, has a shorter latency, no preleukemic phase, a monoblastic, myelomonocytic, or myeloblastic phenotype, and balanced translocations, most commonly involving chromosome bands 11 q23 or 21 q22. The MLL gene at 11 q23 or the AML1 gene at 21 q22 are almost uniformly rearranged. MLL is involved with many fusion gene partners. Therapy-related acute lymphoblastic leukemia also occurs with 1 1 q23 rearrangements. Therapy-related leukemias with 11 q23 or 21 q22 rearrangements, inv(16) or t(15;17), have a more favorable response to treatment and a clinical course similar to their de novo counterparts. 32 refs., 4 tabs.

  1. Aspergillosis in immunocompromised children acute myeloid leukemia and bone marrow aplasia.: Report of two cases Aspergilose em crianças imunocomprometidas com leucemia mielóide aguda e aplasta de medula óssea: Registro de 2 casos

    Directory of Open Access Journals (Sweden)

    Maria Zilda de Aquino

    1994-10-01

    Full Text Available Two cases of Aspergillosis in immunocompromised children are reported. Both were caused by Aspergillns flavus. Early diagnosis and treatment led to the remission of the process. One patient had acute myeloid leukemia; the fungus was isolated from the blood. The other patient with bone marrow aplasia, presented an invasive aspergillosis of the paranasal sinuses with dissemination of fungal infection; the diagnosis was obtained by histology and culture of biopsied tissue from a palatal ulceration.No presente trabalho são registrados dois casos de aspergilose em crianças imunocomprometidas. O estudo micológico completo identificou Aspergillus flavus como agente dos dois processos. A presença cada vez mais frequente da aspergilose invasiva deve-se ao número crescente de pacientes imunocomprometidos, muitos com hemopatias graves submetidos à quimioterapia. O diagnóstico precoce em um dos casos possibilitou remissão do processo. Tratava-se de paciente com leucemia mielóide aguda, tendo sido isolado o fungo do sangue circulante. O segundo caso evoluiu para óbito, com infecção fúngica generalizada.

  2. Induction of myeloid-derived suppressor cells by tumor exosomes

    OpenAIRE

    Xiang, Xiaoyu; Poliakov, Anton; Liu, Cunren; Liu, Yuelong; Deng, Zhong-Bin; wang, Jianhua; Cheng, Ziqiang; Shah, Spandan V.; Wang, Gui-Jun; Zhang, Liming; Grizzle, William E.; Mobley, Jim; Zhang, Huang-Ge

    2009-01-01

    Myeloid-derived suppressor cells (MDSCs) promote tumor progression. The mechanisms of MDSC development during tumor growth remain unknown. Tumor exosomes (T-exosomes) have been implicated to play a role in immune regulation, however the role of exosomes in the induction of MDSCs is unclear. Our previous work demonstrated that exosomes isolated from tumor cells are taken up by bone marrow myeloid cells. Here, we extend those findings showing that exosomes isolated from T-exosomes switch the di...

  3. Myeloid-Derived Suppressor Cells: Linking Inflammation and Cancer1

    OpenAIRE

    Ostrand-Rosenberg, Suzanne; Sinha, Pratima

    2009-01-01

    Many cancer immunotherapies developed in experimental animals have been tested in clinical trials. Although some have shown modest clinical effects, most have not been effective. Recent studies have identified myeloid-origin cells that are potent suppressors of tumor immunity and therefore a significant impediment to cancer immunotherapy. “Myeloid-derived suppressor cells” (MDSC) accumulate in the blood, lymph nodes, and bone marrow and at tumor sites in most patients and experimental animals...

  4. Leucemia Mielóide Crônica: transplante de medula óssea Cronic Myeloid Leukemia: bone marrow transplantation

    Directory of Open Access Journals (Sweden)

    Francisco J. P. Aranha

    2008-04-01

    Full Text Available A única terapia curativa para Leucemia Mielóide Crônica ainda é o transplante de células hematopoéticas progenitoras (TCHP; os atuais resultados com uso do imatinibe são suficientes para indicarmos o TCHP como um tratamento de segunda ou terceira linha. A decisão de realizar TCHP existe em uma variedade de momentos: falha em se conseguir remissão hemtológica, citogenética ou molecular, ou quando se perde a melhor resposta conseguida ou por progressão da doença para uma fase avançada. Há decisão também de como transplantar. Nesta revisão apontamos algumas destas decisões e as atuais controvérsias.Although the only curative therapy for chronic myeloid leukemia remains allogeneic stem cell transplantation (allo-SCT, the results of imatinib in newly diagnosed patients are sufficiently impressive to have displaced allo-SCT to second or third-line treatment. Patients now arrive at a decision for transplantation in a variety of disease situations: failing to achieve certain hematological, cytogenetic and molecular remission by some pre-determined timepoint, having lost a previous best response or due to progression to an advanced phase. The decision is also how to transplant. In this review article, the evidence supporting some of these decisions and current controversies are discussed.

  5. Influence of different chromosomal abnormalities in Ph-positive bone marrow cells on the chronic myeloid leukemia course during tyrosine kinase inhibitors therapy

    Directory of Open Access Journals (Sweden)

    O. Yu. Vinogradova

    2012-01-01

    Full Text Available The additional molecular and chromosomal abnormalities (ACA in Phositive cells usually considered as a genetic marker of chronic myeloid leukemia (CML progression. 457 patients in different CML phases received tyrosine kinase inhibitors (1st and 2nd generation were studied. During therapy 50 cases with additional chromosomal abnormalities in Ph+ clone (22 of them in chronic CML phase were revealed (median follow-up from CML diagnosis – 117 months, median imatinib therapy – 62 months. 86 % of patients in chronic phase with Ph+- cell abnormalities were cytogenetic resistance, and their 5-years overall survival was 80 % which was significantly lower than in patients without ACA (p < 0.005. The treatment results depend on chromosomal abnormalities detected. In patients with additional chromosome 8 imatinib therapy is effective, although complete cytogenetic response (CCR is achieved only in the later therapy stages. In patients with additional translocations CCR also achieved with imatinib or 2nd generation TKI. Only a third of patients with additional Ph-chromosome or BCR/ABL amplification achieved complete suppression of Ph+ clone using 2nd generation TKI. The presence of additional chromosome 7 abnormalities and complex karyotype disorders involving isochromosome i(17(q10 are poor prognostic factors of TKI treatment failures.

  6. Influence of different chromosomal abnormalities in Ph-positive bone marrow cells on the chronic myeloid leukemia course during tyrosine kinase inhibitors therapy

    Directory of Open Access Journals (Sweden)

    O. Yu. Vinogradova

    2014-07-01

    Full Text Available The additional molecular and chromosomal abnormalities (ACA in Phositive cells usually considered as a genetic marker of chronic myeloid leukemia (CML progression. 457 patients in different CML phases received tyrosine kinase inhibitors (1st and 2nd generation were studied. During therapy 50 cases with additional chromosomal abnormalities in Ph+ clone (22 of them in chronic CML phase were revealed (median follow-up from CML diagnosis – 117 months, median imatinib therapy – 62 months. 86 % of patients in chronic phase with Ph+- cell abnormalities were cytogenetic resistance, and their 5-years overall survival was 80 % which was significantly lower than in patients without ACA (p < 0.005. The treatment results depend on chromosomal abnormalities detected. In patients with additional chromosome 8 imatinib therapy is effective, although complete cytogenetic response (CCR is achieved only in the later therapy stages. In patients with additional translocations CCR also achieved with imatinib or 2nd generation TKI. Only a third of patients with additional Ph-chromosome or BCR/ABL amplification achieved complete suppression of Ph+ clone using 2nd generation TKI. The presence of additional chromosome 7 abnormalities and complex karyotype disorders involving isochromosome i(17(q10 are poor prognostic factors of TKI treatment failures.

  7. Primary Intracranial Synovial Sarcoma

    Science.gov (United States)

    Li, Luyuan; Sinson, Grant; Mueller, Wade

    2016-01-01

    Background. Synovial sarcoma is an aggressive soft tissue sarcoma with uncertain histological origin. The pathology frequently presents as a localized disease, especially near large joints around the knee and thigh. Intracranial disease, which is rare, has been reported as metastasis from synovial sarcoma. We report a case with no obvious primary extracranial pathology, suggesting primary intracranial disease; this has not been reported in the literature. Case Description. A 21-year-old male, with a prior right skull lesion resection for atypical spindle cell neoplasm, presented with headaches, gait instability, left arm weakness, and left homonymous hemianopsia. CT of head demonstrated a right parietal hemorrhagic lesion with mass effect, requiring surgical decompression. Histopathology revealed synovial sarcoma. FISH analysis noted the existence of the t(X;18)(p11.2;q11.2) chromosomal translocation. PET scan did not show other metastatic disease. He underwent stereotactic radiotherapy and adjuvant chemotherapy. At 2-year follow-up, he remained nonfocal without recurrence. Conclusion. We report the first known case of primary intracranial synovial sarcoma. Moreover, we stress that intracranial lesions may have a tendency for hemorrhage, requiring urgent lifesaving decompression.

  8. Primary Intracranial Synovial Sarcoma.

    Science.gov (United States)

    Patel, Mohit; Li, Luyuan; Nguyen, Ha Son; Doan, Ninh; Sinson, Grant; Mueller, Wade

    2016-01-01

    Background. Synovial sarcoma is an aggressive soft tissue sarcoma with uncertain histological origin. The pathology frequently presents as a localized disease, especially near large joints around the knee and thigh. Intracranial disease, which is rare, has been reported as metastasis from synovial sarcoma. We report a case with no obvious primary extracranial pathology, suggesting primary intracranial disease; this has not been reported in the literature. Case Description. A 21-year-old male, with a prior right skull lesion resection for atypical spindle cell neoplasm, presented with headaches, gait instability, left arm weakness, and left homonymous hemianopsia. CT of head demonstrated a right parietal hemorrhagic lesion with mass effect, requiring surgical decompression. Histopathology revealed synovial sarcoma. FISH analysis noted the existence of the t(X;18)(p11.2;q11.2) chromosomal translocation. PET scan did not show other metastatic disease. He underwent stereotactic radiotherapy and adjuvant chemotherapy. At 2-year follow-up, he remained nonfocal without recurrence. Conclusion. We report the first known case of primary intracranial synovial sarcoma. Moreover, we stress that intracranial lesions may have a tendency for hemorrhage, requiring urgent lifesaving decompression. PMID:27247811

  9. Primary Intracranial Synovial Sarcoma

    Directory of Open Access Journals (Sweden)

    Mohit Patel

    2016-01-01

    Full Text Available Background. Synovial sarcoma is an aggressive soft tissue sarcoma with uncertain histological origin. The pathology frequently presents as a localized disease, especially near large joints around the knee and thigh. Intracranial disease, which is rare, has been reported as metastasis from synovial sarcoma. We report a case with no obvious primary extracranial pathology, suggesting primary intracranial disease; this has not been reported in the literature. Case Description. A 21-year-old male, with a prior right skull lesion resection for atypical spindle cell neoplasm, presented with headaches, gait instability, left arm weakness, and left homonymous hemianopsia. CT of head demonstrated a right parietal hemorrhagic lesion with mass effect, requiring surgical decompression. Histopathology revealed synovial sarcoma. FISH analysis noted the existence of the t(X;18(p11.2;q11.2 chromosomal translocation. PET scan did not show other metastatic disease. He underwent stereotactic radiotherapy and adjuvant chemotherapy. At 2-year follow-up, he remained nonfocal without recurrence. Conclusion. We report the first known case of primary intracranial synovial sarcoma. Moreover, we stress that intracranial lesions may have a tendency for hemorrhage, requiring urgent lifesaving decompression.

  10. Adult Intramedullary Ewing Sarcoma of the Proximal Hip

    OpenAIRE

    Preetam Gongidi; Siva Jasti; William Rafferty; Veniamin Barshay; Richard Lackman

    2014-01-01

    Ewing sarcoma of bone is classically a permeative lesion in the diaphysis of long bones in children. While they occur primarily in children and adolescents, they can be seen in young adults in their 20s, but these are typically seen in flat bones. The permeative nature of the lesion can elicit new bone formation creating a partially sclerotic appearance, cortical expansion presenting as a “Codman triangle,” or have an “onion-skin” type of aggressive periosteal reaction/periostitis. Ewing sarc...

  11. Mobilized peripheral blood stem cells compared with bone marrow from HLA-identical siblings for reduced-intensity conditioning transplantation in acute myeloid leukemia in complete remission

    DEFF Research Database (Denmark)

    Nagler, Arnon; Labopin, Myriam; Shimoni, Avichai;

    2012-01-01

    Reduced-intensity conditioning (RIC)-alloSCT is increasingly used for acute myelogenous leukemia. Limited data are available for the comparison of peripheral blood stem cells with bone marrow for RIC-alloSCT. We used the European Group for Blood and Marrow Transplantation (EBMT) ALWP data to...... compare the outcome of mobilized peripheral blood stem cells (PBSC) (n = 1430) vs. bone marrow (BM) (n = 107) for acute myelogenous leukemia (AML) patients with complete remission that underwent RIC-alloSCT from compatible sibling donors. The leukemia features, the disease status, and the time from......-IV) and chronic GVHD did not differ between the groups. leukemia-free survival (LFS), relapse, and non-relapsed mortality (NRM) were 51 ± 2%, 32 ± 1%, and 17 ± 1% vs. 50 ± 6%, 38 ± 6%, and 12 ± 3% for the PBSC and BM groups, respectively. Our results indicate faster engraftment, but no difference in GVHD...

  12. 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.

  13. Myeloid cell-derived reactive oxygen species externally regulate the proliferation of myeloid progenitors in emergency granulopoiesis

    Science.gov (United States)

    Kwak, Hyun-Jeong; Liu, Peng; Bajrami, Besnik; Xu, Yuanfu; Park, Shin-Young; Nombela-Arrieta, Cesar; Mondal, Subhanjan; Sun, Yan; Zhu, Haiyan; Chai, Li; Silberstein, Leslie E.; Cheng, Tao; Luo, Hongbo R.

    2015-01-01

    Summary The cellular mechanisms controlling infection-induced emergency granulopoiesis are poorly defined. Here we found that reactive oxygen species (ROS) concentrations in the bone marrow (BM) were elevated during acute infection in a phagocytic NADPH oxidase-dependent manner in myeloid cells. Gr1+ myeloid cells were uniformly distributed in the BM, and all c-Kit+ progenitor cells were adjacent to Gr1+ myeloid cells. Inflammation-induced ROS production in the BM played a critical role in myeloid progenitor expansion during emergency granulopoiesis. ROS elicited oxidation and deactivation of phosphatase and tensin homolog (PTEN), resulting in up-regulation of PtdIns(3,4,5)P3 signaling in BM myeloid progenitors. We further revealed that BM myeloid cell-produced ROS stimulated proliferation of myeloid progenitors via a paracrine mechanism. Taken together, our results establish that phagocytic NADPH oxidase-mediated ROS production by BM myeloid cells plays a critical role in mediating emergency granulopoiesis during acute infection. PMID:25579427

  14. Elusive Identities and Overlapping Phenotypes of Proangiogenic Myeloid Cells in Tumors

    OpenAIRE

    Coffelt, Seth B.; Lewis, Claire E.; Naldini, Luigi; Brown, J. Martin; Ferrara, Napoleone; De Palma, Michele

    2010-01-01

    It is now established that bone marrow-derived myeloid cells regulate tumor angiogenesis. This was originally inferred from studies of human tumor biopsies in which a positive correlation was seen between the number of tumor-infiltrating myeloid cells, such as macrophages and neutrophils, and tumor microvessel density. However, unequivocal evidence was only provided once mouse models were used to examine the effects on tumor angiogenesis by genetically or pharmacologically targeting myeloid c...

  15. Intravascular synovial sarcoma.

    OpenAIRE

    Robertson, N. J.; Halawa, M H; Smith, M E

    1998-01-01

    A case of intravascular biphasic synovial sarcoma arising from the wall of the left femoral vein in a 34 year old woman is described. This is the third case of an intravascular synovial sarcoma known to be reported in the medical literature. The two previous cases arose from the left femoral vein and inferior vena cava in women of 34 and 31 years old, respectively. A characteristic clinical pattern appears to be emerging--that is, location in large veins of the lower extremity and trunk in yo...

  16. Iodine I 131 Monoclonal Antibody BC8, Fludarabine Phosphate, Cyclophosphamide, Total-Body Irradiation and Donor Bone Marrow Transplant in Treating Patients With Advanced Acute Myeloid Leukemia, Acute Lymphoblastic Leukemia, or High-Risk Myelodysplastic Syndrome

    Science.gov (United States)

    2016-07-18

    Acute Myeloid Leukemia Arising From Previous Myelodysplastic Syndrome; Adult Acute Lymphoblastic Leukemia in Remission; Adult Acute Myeloid Leukemia in Remission; Chronic Myelomonocytic Leukemia; Previously Treated Myelodysplastic Syndrome; Refractory Anemia With Excess Blasts; Refractory Anemia With Ring Sideroblasts; Refractory Cytopenia With Multilineage Dysplasia; Refractory Cytopenia With Multilineage Dysplasia and Ring Sideroblasts

  17. Prognostic and predictive factors for outcome to first-line ifosfamide-containing chemotherapy for adult patients with advanced soft tissue sarcomas An exploratory, retrospective analysis on large series from the European Organization for Research and Treatment of Cancer-Soft Tissue and Bone, Sarcoma Group (EORTC-STBSG)

    DEFF Research Database (Denmark)

    Sleiffer, S.; Ouali, M.; van Glabbeke, M.; Krarup-Hansen, A.; Rodenhuis, S.; Le Cesne, A.; Hogendoorn, P.C.W.; Verweij, J.; Blay, J.Y.

    2010-01-01

    and ifosfamide, locally advanced disease, and tumour entity with a lower risk to progress for synovial sarcoma patients compared to leiomyosarcoma. For response, independent favourable prognostic factors were doxorubicin combined with ifosfamide, higher histological grade, and histology with synovial......Background: Adult patients with advanced soft tissue sarcomas (STS) are generally treated similarly, regardless of great differences between STS subtypes, disease presentation and patients' characteristics. As ifosfamide is frequently applied in first line systemic therapy, we aimed to establish...... sarcoma patients having the highest chance to respond. Predictive factor analysis showed that compared to doxorubicin monotherapy, patients who benefited less from ifosfamide-based therapies were leiomyosarcoma patients in terms of OS, and patients with liposarcoma for response. No predictive factors were...

  18. Ewing's sarcoma in mandibular similar to dental abscess

    OpenAIRE

    Forouz Keshani; Gholamreza Jahanshahi; Bijan Movahedian Attar; Mahsa Kalantari; Seyed Mohammad Razavi; Zahra Hashemzade; Payam Tavakoli

    2014-01-01

    Ewing′s sarcoma is a rare malignant neoplasm that comprises approximately 4-6% of primary bone tumors. In most cases, femur and pelvis are affected, and less commonly the head and neck areas (in the jaws, usually the mandible). These tumors have been reported more frequently in males, mostly aged 5-20 years old. Systemic symptoms and signs such as fever, weight loss, anemia, leukocytosis, and elevated erythrocyte sedimentation rate (ESR) may be the first signs in oral Ewing′s sarcoma. Such...

  19. Synovial sarcoma: current perspectives

    Directory of Open Access Journals (Sweden)

    Brennan B

    2016-04-01

    Full Text Available Bernadette Brennan Department of Paediatric Oncology, Royal Manchester Children's Hospital, Manchester, UK Abstract: Synovial sarcoma (SS is a distinct soft tissue sarcoma, occurring across all ages, from young children to the elderly, but the incidence of SS peaks in young adults. Recently, its biology, specifically the biomarker genomic index, may prove to be the most important prognostic marker, explaining findings such as the positive effect of younger age on outcome. While believed to be a chemosensitive soft tissue sarcoma, surgery remains the most important modality of treatment for many people, especially in localized disease, plus or minus radiotherapy to improve local control. Moving forward, the demonstration that SS has multiple therapeutic targets such as vascular endothelial growth factor, and new emerging targets, allows us to start to consider different systemic therapeutic interventions other than just chemotherapy. This is particularly important for advanced/metastatic SS which, with conventional chemotherapy only, continues to have a very poor outcome. The way forward, therefore, is an all-age trial combining perhaps anti-angiogenesis agents with chemotherapy, focusing on those SS cases whose outcome, as determined by biology, site, or metastatic status, is poor with just conventional sarcoma chemotherapy. This also allows prospective evaluation of the role of genomic index and other biomarkers. Keywords: children/adolescents, genomic index, targeted therapy, MAMS design

  20. 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....

  1. Targeting interleukin-2 to the bone marrow stroma for therapy of acute myeloid leukemia relapsing after allogeneic hematopoietic stem cell transplantation.

    Science.gov (United States)

    Schliemann, Christoph; Gutbrodt, Katrin L; Kerkhoff, Andrea; Pohlen, Michele; Wiebe, Stefanie; Silling, Gerda; Angenendt, Linus; Kessler, Torsten; Mesters, Rolf M; Giovannoni, Leonardo; Schäfers, Michael; Altvater, Bianca; Rossig, Claudia; Grünewald, Inga; Wardelmann, Eva; Köhler, Gabriele; Neri, Dario; Stelljes, Matthias; Berdel, Wolfgang E

    2015-05-01

    The antibody-based delivery of IL2 to extracellular targets expressed in the easily accessible tumor-associated vasculature has shown potent antileukemic activity in xenograft and immunocompetent murine models of acute myelogenous leukemia (AML), especially in combination with cytarabine. Here, we report our experience with 4 patients with relapsed AML after allogeneic hematopoietic stem cell transplantation (allo-HSCT), who were treated with the immunocytokine F16-IL2, in combination with low-dose cytarabine. One patient with disseminated extramedullary AML lesions achieved a complete metabolic response identified by PET/CT, which lasted 3 months. Two of 3 patients with bone marrow relapse achieved a blast reduction with transient molecular negativity. One of the 2 patients enjoyed a short complete remission before AML relapse occurred 2 months after the first infusion of F16-IL2. In line with a site-directed delivery of the cytokine, F16-IL2 led to an extensive infiltration of immune effector cells in the bone marrow. Grade 2 fevers were the only nonhematologic side effects in 2 patients. Grade 3 cytokine-release syndrome developed in the other 2 patients but was manageable in both cases with glucocorticoids. The concept of specifically targeting IL2 to the leukemia-associated stroma deserves further evaluation in clinical trials, especially in patients who relapse after allo-HSCT. PMID:25672398

  2. Immature myeloid Gr-1+ CD11b+ cells from lipopolysaccharide-immunosuppressed mice acquire inhibitory activity in the bone marrow and migrate to lymph nodes to exert their suppressive function.

    Science.gov (United States)

    Landoni, Veronica I; Martire-Greco, Daiana; Rodriguez-Rodrigues, Nahuel; Chiarella, Paula; Schierloh, Pablo; Isturiz, Martin A; Fernández, Gabriela C

    2016-02-01

    Secondary infections due to post-sepsis immunosuppression are a major cause of death in patients with sepsis. Repetitive inoculation of increasing doses of lipopolysaccharide (LPS) into mice mimics the immunosuppression associated with sepsis. Myeloid-derived suppressor cells (MDSCs, Gr-1(+) CD11b(+)) are considered a major component of the immunosuppressive network, interfering with T-cell responses in many pathological conditions. We used LPS-immunosuppressed (IS) mice to address whether MDSCs acquired their suppressive ability in the bone marrow (BM) and whether they could migrate to lymph nodes (LNs) to exert their suppressive function. Our results showed that Gr-1(+) CD11b(+) cells of IS mice already had the potential to inhibit T-cell proliferation in the BM. Moreover, soluble factors present in the BM from IS mice were responsible for inducing this inhibitory ability in control BM cells. In addition, migration of Gr-1(+) CD11b(+) to LNs in vivo was maximal when cells obtained from the BM of IS mice were inoculated into an IS context. In this regard, we found chemoattractant activity in cell-free LN extracts (LNEs) from IS mice and an increased expression of the LN-homing chemokine receptor C-C chemokine receptor type 7 (CCR7) in IS BM Gr-1(+) CD11b(+) cells. These results indicate that Gr-1(+) CD11b(+) cells found in BM from IS mice acquire their suppressive activity in the same niche where they are generated, and migrate to LNs to exert their inhibitory role. A better understanding of MDSC generation and/or regulation of factors able to induce their inhibitory function may provide new and more effective tools for the treatment of sepsis-associated immunosuppression. PMID:26582821

  3. Comparative evaluation of bone marrow cells morpho-functional activity in chronic myeloid leukemia patients treated with tyrosine kinase inhibitors of the first and second generation

    Directory of Open Access Journals (Sweden)

    I. O. Zhaleyko

    2014-07-01

    Full Text Available The efficiency of using the culture techniques of research for monitoring the patient’s response to the treatment by tyrosine kinase inhibitors of the first and second generation is shown. Thus, the functional activity of bone marrow cells in patients having the optimal treatment response to inhibitors of tyrosine kinases was significantly lower compared with patients with the acquired resistance to the drug, and patients who had CML diagnosed for first time. Furthermore, for patients with the optimal response to the nilotinib therapy, numbers of colonies in semi-solid agar in vitro was lower, than in patients with the optimal response to imatinib. When the leukaemic cell clone becomes resistant to tyrosine kinase inhibitors, the prevalence of early cells of granulocyte-macrophage hematopoietic stem cells is observed in CFU culture which can be an important prognostic factor for choosing the appropriate treatment strategy.

  4. Immunological and inflammatory features of Kaposi's sarcoma and other Kaposi's sarcoma-associated herpesvirus/human herpesvirus 8-associated neoplasias.

    Science.gov (United States)

    Riva, Giovanni; Barozzi, Patrizia; Torelli, Giuseppe; Luppi, Mario

    2010-01-01

    During the last 15 years, virologic and immunologic studies have provided a series of valuable clues on the modalities of gamma-herpesvirus-induced oncogenesis, which do not only consist of the direct subversion of intracellular signaling pathways, leading to a frank neoplastic molecular network in the infected cell, but also rely on viral manipulations of the cellular and cytokine microenvironment, especially in conditions of immunodeficiency in the host. At the virus-host interface, something iniquitous, strikingly favoring the aggressive expansion of human herpesvirus 8-infected lympho-endothelial clones, known as Kaposi's sarcoma, often occurs in different types of immunocompromised patients, able to establish a deleterious "pro-Kaposi's sarcoma" neo-angiogenic inflammatory network. However, these patients may control - or even resolve - the neoplastic burden as soon as an immunologic reassessment restores functional anti-Kaposi's sarcoma immune responses and reconstitutes a proper inflammatory environment. Indeed, the occurrence of iatrogenic Kaposi's sarcoma remissions, after the reduction or switch of immunosuppressive regimens, strongly suggests that the reset of immunologic constraints characterizing the Kaposi's sarcoma onco-pathogenic system may be sufficient to inhibit human herpesvirus 8-positive lympho-endothelial proliferations. Accordingly, immunologic reports all underline the pivotal protective role of anti-human herpesvirus 8 memory T-cells (harmonically, both CD8+ and CD4+ subsets), thus definitely implying a general requirement for an effective, antiviral immuno-inflammatory environment, based on correct and productive interactions between different compartments of dendritic, myeloid, and specific T-cells, in order to achieve and maintain optimal control on human herpesvirus 8-associated antigenic stimulations and Kaposi's sarcoma disease. In this review, we recapitulate some remarkable features about the outstanding immunologic issue raised by

  5. Bone marrow chimeras and c-fms conditional ablation (Mafia) mice reveal an essential role for resident myeloid cells in lipopolysaccharide/TLR4-induced corneal inflammation.

    Science.gov (United States)

    Chinnery, Holly R; Carlson, Eric C; Sun, Yan; Lin, Michelle; Burnett, Sandra H; Perez, Victor L; McMenamin, Paul G; Pearlman, Eric

    2009-03-01

    The mammalian cornea contains an extensive network of resident macrophages and dendritic cells. To determine the role of these cells in LPS-induced corneal inflammation, TLR4(-/-) mice were sublethally irradiated and reconstituted with bone marrow cells from either enhanced GFP (eGFP)(+)/C57BL/6 or eGFP(+)/TLR4(-/-) mice. The corneal epithelium was abraded, LPS was added topically, and cellular infiltration to the corneal stroma and development of corneal haze were examined after 24 h. TLR4(-/-) mice reconstituted with C57BL/6, but not TLR4(-/-) bone marrow cells donor cells were found to cause infiltration of eGFP(+) cells to the cornea, including neutrophils, and also increased corneal haze compared with saline-treated corneas. In a second experimental approach, corneas of transgenic macrophage Fas induced apoptosis (Mafia) mice were stimulated with LPS. These mice express eGFP and a suicide gene under control of the c-fms promoter, and systemic treatment with the FK506 dimerizer (AP20187) causes Fas-mediated apoptosis of monocytic cells. AP20187-treated mice had significantly fewer eGFP(+) cells in the cornea than untreated mice. After stimulation with LPS neutrophil recruitment and development of corneal haze were impaired in AP20187-treated mice compared with untreated controls. Furthermore, LPS induced CXCL1/KC and IL-1alpha production within 4 h in corneas of untreated Mafia mice, which is before cellular infiltration; however, cytokine production was impaired after AP20187 treatment. Together, results from both experimental approaches demonstrate an essential role for resident corneal monocytic lineage cells (macrophages and dendritic cells) in development of corneal inflammation. PMID:19234168

  6. Bone Marrow Chimeras and c-fms Conditional Ablation (Mafia) Mice Reveal an Essential Role for Resident Myeloid Cells in Lipopolysaccharide/TLR4-Induced Corneal Inflammation1

    Science.gov (United States)

    Chinnery, Holly R.; Carlson, Eric C.; Sun, Yan; Lin, Michelle; Burnett, Sandra H.; Perez, Victor L.; McMenamin, Paul G.; Pearlman, Eric

    2012-01-01

    The mammalian cornea contains an extensive network of resident macrophages and dendritic cells. To determine the role of these cells in LPS-induced corneal inflammation, TLR4−/− mice were sublethally irradiated and reconstituted with bone marrow cells from either enhanced GFP (eGFP)+/C57BL/6 or eGFP+/TLR4−/− mice. The corneal epithelium was abraded, LPS was added topically, and cellular infiltration to the corneal stroma and development of corneal haze were examined after 24 h. TLR4−/− mice reconstituted with C57BL/6, but not TLR4−/− bone marrow cells donor cells were found to cause infiltration of eGFP+ cells to the cornea, including neutrophils, and also increased corneal haze compared with saline-treated corneas. In a second experimental approach, corneas of transgenic macrophage Fas induced apoptosis (Mafia) mice were stimulated with LPS. These mice express eGFP and a suicide gene under control of the c-fms promoter, and systemic treatment with the FK506 dimerizer (AP20187) causes Fas-mediated apoptosis of monocytic cells. AP20187-treated mice had significantly fewer eGFP+ cells in the cornea than untreated mice. After stimulation with LPS neutrophil recruitment and development of corneal haze were impaired in AP20187-treated mice compared with untreated controls. Furthermore, LPS induced CXCL1/KC and IL-1α production within 4 h in corneas of untreated Mafia mice, which is before cellular infiltration; however, cytokine production was impaired after AP20187 treatment. Together, results from both experimental approaches demonstrate an essential role for resident corneal monocytic lineage cells (macrophages and dendritic cells) in development of corneal inflammation. PMID:19234168

  7. Thyroid Synovial Sarcoma: A Case Report

    OpenAIRE

    Ali Ghafouri; Taha Anbara; Ali Mir; Marzieh Lashkari; Mohammad Nazari

    2013-01-01

    Sarcomas are rare tumors account for about 1% of cancers in adult. Soft tissue sarcomas are the most common one. Synovial sarcoma's incident is about 10% of all sarcomas and most commonly rise from para-articular regions in young adults. Based on our knowledge there have been only two reports of thyroid synovial sarcoma in medical literature. We report a 44-year old woman presented with a rapid growing neck mass. The pathology report revealed sarcoma and the immunohistochemistry (IHC) was com...

  8. Doxorubicin With Upfront Dexrazoxane for the Treatment of Advanced or Metastatic Soft Tissue Sarcoma

    Science.gov (United States)

    2016-03-07

    Sarcoma, Soft Tissue; Soft Tissue Sarcoma; Undifferentiated Pleomorphic Sarcoma; Leiomyosarcoma; Liposarcoma; Synovial Sarcoma; Myxofibrosarcoma; Angiosarcoma; Fibrosarcoma; Malignant Peripheral Nerve Sheath Tumor; Epithelioid Sarcoma

  9. Translocation (8;21) acute myeloid leukemia presenting as severe aplastic anemia

    OpenAIRE

    Enkhtsetseg Purev; Bogdan Dumitriu; Hourigan, Christopher S.; Young, Neal S.; Townsley, Danielle M.

    2014-01-01

    We report a case of t(8;21) acute myeloid leukemia presenting as severe aplastic anemia. While initial bone marrow biopsy lacked any cytogenetic abnormalities in 20 analyzed metaphases, repeat bone marrow biopsy eight days later demonstrated this translocation. Initial cytogenetic analysis of 20 metaphases was therefore insufficient to make the diagnosis of hypocellular acute myeloid leukemia. We discuss that further complementary molecular tests, such as CGH, would likely provide a more robu...

  10. Clinical impact of leukemic blast heterogeneity at diagnosis in cytogenetic intermediate-risk acute myeloid leukemia

    DEFF Research Database (Denmark)

    Hoffmann, Marianne Hutchings; Klausen, Tobias Wirenfeldt; Boegsted, Martin;

    2012-01-01

    Individual cellular heterogeneity within the acute myeloid leukemia (AML) bone marrow samples can be observed by multi parametric flow cytometry analysis (MFC) indicating that immunophenotypic screening for leukemic blast subsets may have prognostic impact.......Individual cellular heterogeneity within the acute myeloid leukemia (AML) bone marrow samples can be observed by multi parametric flow cytometry analysis (MFC) indicating that immunophenotypic screening for leukemic blast subsets may have prognostic impact....

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

    International Nuclear Information System (INIS)

    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.)

  12. 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.)

  13. Granulocytic Sarcoma in a Nonleukemic Patient: Place of Radiotherapy and Systemic Therapies

    Directory of Open Access Journals (Sweden)

    C. Chargari

    2011-01-01

    Full Text Available Granulocytic sarcoma is a rare extramedullary tumour, which most often occurs in the course of an acute or chronic leukaemia or myeloproliferative disorders. Rarely it is found before peripheral blood or bone marrow evidence of leukemia is present. We report an unusual case of acute paraplegia at first presentation of a spinal epidural granulocytic sarcoma without any haematological disorder. Therapeutic strategies are discussed in the light of the literature.

  14. Primary extraosseous intracranial Ewing′s sarcoma: Case report and literature review

    Directory of Open Access Journals (Sweden)

    Krishnangshu Bhanja Choudhury

    2011-01-01

    Full Text Available Ewing′s sarcoma / peripheral primitive neuroectodermal tumors occur most often in bone and soft tissues of children and young adults. The intracranial manifestation of the disease is rare, and when present, this is often misdiagnosed with other varieties of primary brain tumors. We report such a case of extraosseous Ewing′s sarcoma, which was initially suspected to be a case of meningioma in an 11-year-old girl.

  15. Primitive Neuroectodermal Tumor/Ewing Sarcoma Presenting with Pulmonary Nodular Lesions

    Directory of Open Access Journals (Sweden)

    Selvi Asker

    2015-01-01

    Full Text Available Primitive neuroectodermal tumors (PNETs and Ewing sarcoma (EWS belong to the same family of malignant, small, round cell neoplasms of soft tissue or bone origin. EWS-PNETs that arise in the lung parenchyma involvement are extremely rare in adults. A case of a 32-year-old male presenting with chest pain and diffuse pulmonary nodules on chest X-ray and diagnosed with Ewing sarcoma-PNETs will be presented here.

  16. MLN0128, an ATP-Competitive mTOR Kinase Inhibitor, with Potent In vitro and In vivo Antitumor Activity as Potential Therapy for Bone and Soft-Tissue Sarcoma

    Science.gov (United States)

    Slotkin, Emily K.; Patwardhan, Parag P.; Vasudeva, Shyamprasad Deraje; de Stanchina, Elisa; Tap, William D.; Schwartz, Gary K.

    2014-01-01

    The mammalian target of rapamycin (mTOR) is a serine/threonine protein kinase that exists in two complexes (mTORC1 and mTORC2) and integrates extracellular and intracellular signals to act as a master regulator of cell growth, survival, and metabolism. The PI3K/AKT/mTOR pro-survival pathway is often dysregulated in multiple sarcoma subtypes. First-generation allosteric inhibitors of mTORC1 (rapalogues) have been extensively tested with great pre-clinical promise, but have had limited clinical utility. Here we report that MLN0128, a second-generation, ATP-competitive, pan-mTOR kinase inhibitor, acts on both mTORC1 and mTORC2, and has potent in vitro and in vivo anti-tumor activity in multiple sarcoma subtypes. In vitro, MLN0128 inhibits mTORC1/2 targets in a concentration dependent fashion, and shows striking anti-proliferative effect in rhabdomyosarcoma (RMS), Ewing sarcoma (ES), malignant peripheral nerve sheath tumor, synovial sarcoma, osteosarcoma, and liposarcoma. Unlike rapamycin, MLN0128 inhibits phosphorylation of 4EBP1 and NDRG1 as well as prevents the reactivation of pAKT that occurs via negative feedback release with mTORC1 inhibition alone. In xenograft models, MLN0128 treatment results in suppression of tumor growth with two dosing schedules (1 mg/kg daily and 3 mg/kg BID TIW). At the 3 mg/kg dosing schedule, MLN0128 treatment results in significantly better tumor growth suppression than rapamycin in RMS and ES models. Additionally, MLN0128 induces apoptosis in models of RMS both in vitro and in vivo. Results from our study strongly suggest that MLN0128 treatment should be explored further as potential therapy for sarcoma. PMID:25519700

  17. Extramedullary Myeloid Cell Tumour Presenting As Leukaemia Cutis

    Directory of Open Access Journals (Sweden)

    Thappa Devinder Mohan

    2002-01-01

    Full Text Available We herewith report a case of extramedullary myeloid cell tumour presenting as leukaemia cutis for its rarity. It occurred in a 50 year old male patient who presented to us with a 40 days history of painless raised solid skin swellings over the trunk. Histopathological examination of the skin biopsy and bone marrow biopsy showed features suggestive of non-Hodgkin’s lymphoma. Immunophenotyping on skin biopsy specimens and bone marrow biopsy found tumour cells expressing CD43 and Tdt but were negative for CD3 and CD20. These features were consistent with extramedullary myeloid cell tumour involving skin and subcutis (cutaneous manifestation of acute myeloid leukaemia.

  18. Prognostic and predictive factors for outcome to first-line ifosfamide-containing chemotherapy for adult patients with advanced soft tissue sarcomas An exploratory, retrospective analysis on large series from the European Organization for Research and Treatment of Cancer-Soft Tissue and Bone Sarcoma Group (EORTC-STBSG)

    DEFF Research Database (Denmark)

    Sleiffer, S.; Ouali, M.; van Glabbeke, M.; Krarup-Hansen, A.; Rodenhuis, S.; Le Cesne, A.; Hogendoorn, P.C.W.; Verweij, J.; Blay, J.Y.

    2010-01-01

    monotherapy served as comparators. Results: Independent favourable prognostic factors for overall survival (OS) were good performance status, female gender, low histological grade, extremity primary tumour site and locally advanced disease; for progression-free survival (PFS), the combination of doxorubicin...... and ifosfamide, locally advanced disease, and tumour entity with a lower risk to progress for synovial sarcoma patients compared to leiomyosarcoma. For response, independent favourable prognostic factors were doxorubicin combined with ifosfamide, higher histological grade, and histology with synovial...

  19. Vascular endothelial growth factor signaling in acute myeloid leukemia

    NARCIS (Netherlands)

    Kampen, Kim R.; ter Elst, Arja; de Bont, Eveline S. J. M.

    2013-01-01

    This review is designed to provide an overview of the current literature concerning vascular endothelial growth factor signaling (VEGF) in acute myeloid leukemia (AML). Aberrant VEGF signaling operates in the bone marrow of AML patients and is related to a poor prognosis. The altered signaling pathw

  20. Autografting as first line treatment for chronic myeloid leukaemia.

    OpenAIRE

    Singer, I O; Franklin, I M

    1998-01-01

    Interest in autografting for chronic myeloid leukaemia and its clinical relevance has revived in recent years. This followed observations that with various chemotherapeutic regimens it was possible to achieve, temporarily at least, peripheral blood and bone marrow that were Philadelphia negative. Bone marrow or peripheral blood progenitor cells could then be harvested and reinfused following a high dose procedure, hopefully eliminating any residual disease, and resulting in prolonged disease ...

  1. Retroperitoneal synovial sarcoma presenting as paraneoplastic hypoglycaemia

    Directory of Open Access Journals (Sweden)

    Reddy VV

    2015-01-01

    Full Text Available Synovial sarcoma is a well-recognized malignant mesenchymal neoplasm. Primary retroperitoneal synovial sarcoma is extremely rare and has poor prognosis. There are sparse reports in the literature on the secretory synovial sarcomas. In this report, we present the case of a patient with retroperitoneal synovial sarcoma who presented with recurrent attacks of hypoglycaemia.

  2. Retroperitoneal synovial sarcoma presenting as paraneoplastic hypoglycaemia

    OpenAIRE

    Reddy VV; Sarala S; Mathai V; Madhu; Sreedhar Babu KV

    2015-01-01

    Synovial sarcoma is a well-recognized malignant mesenchymal neoplasm. Primary retroperitoneal synovial sarcoma is extremely rare and has poor prognosis. There are sparse reports in the literature on the secretory synovial sarcomas. In this report, we present the case of a patient with retroperitoneal synovial sarcoma who presented with recurrent attacks of hypoglycaemia.

  3. Characterisation of the Leukemic Stem Cell in a Murine Model of CALM/AF10 Positive Myeloid Leukemia

    OpenAIRE

    Deshpande, Aniruddha

    2006-01-01

    We have demonstrated, that an acute leukemia with a predominantly myeloid phenotype can be propagated by a progenitor with lymphoid characteristics in a mouse model of the t(10;11) (p13;q14) translocation. Mice transplanted with bone marrow retrovirally engineered to express the leukemia specific CALM/AF10 fusion gene consistently developed an acute leukemia with a short latency. The leukemia showed characteristic myeloid features such as the presence of myeloid marker positive cells infilt...

  4. Efficacy of triplet regimen antiemetic therapy for chemotherapy-induced nausea and vomiting (CINV) in bone and soft tissue sarcoma patients receiving highly emetogenic chemotherapy, and an efficacy comparison of single-shot palonosetron and consecutive-day granisetron for CINV in a randomized, single-blinded crossover study

    International Nuclear Information System (INIS)

    The first aim of this study was to evaluate combination antiemetic therapy consisting of 5-HT3 receptor antagonists, neurokinin-1 receptor antagonists (NK-1RAs), and dexamethasone for multiple high emetogenic risk (HER) anticancer agents in bone and soft tissue sarcoma. The second aim was to compare the effectiveness of single-shot palonosetron and consecutive-day granisetron in a randomized, single-blinded crossover study. A single randomization method was used to assign eligible patients to the palonosetron or granisetron arm. Patients in the palonosetron arm received a palonosetron regimen during the first and third chemotherapy courses and a granisetron regimen during the second and fourth courses. All patients received NK-1RA and dexamethasone. Patients receiving the palonosetron regimen were administered 0.75 mg palonosetron on day 1, and patients receiving the granisetron regimen were administered 3 mg granisetron twice daily on days 1 through 5. All 24 patients in this study received at least 4 chemotherapy courses. A total of 96 courses of antiemetic therapy were evaluated. Overall, the complete response CR rate (no emetic episodes and no rescue medication use) was 34%, while the total control rate (a CR plus no nausea) was 7%. No significant differences were observed between single-shot palonosetron and consecutive-day granisetron. Antiemetic therapy with a 3-drug combination was not sufficient to control chemotherapy-induced nausea and vomiting (CINV) during chemotherapy with multiple HER agents for bone and soft tissue sarcoma. This study also demonstrated that consecutive-day granisetron was not inferior to single-shot palonosetron for treating CINV

  5. Synovial sarcoma of the parapharyngeal space: CT and MRI

    International Nuclear Information System (INIS)

    We present the CT and MRI findings of a histologically proven synovial sarcoma arising in the left parapharyngeal space of a 21-year-old man. CT was useful for confirming the presence of calcification within the tumour, which may be a favourable prognostic sign, and in excluding involvement of cortical bone. The CT and MRI findings were, however, nonspecific. MRI was superior to CT for assessing the topographical relationships of the tumour to the vessels and the invasion of neighbouring structures. (orig.)

  6. Variable expression of PIK3R3 and PTEN in Ewing Sarcoma impacts oncogenic phenotypes.

    Directory of Open Access Journals (Sweden)

    Brian F Niemeyer

    Full Text Available Ewing Sarcoma is an aggressive malignancy of bone and soft tissue affecting children and young adults. Ewing Sarcoma is driven by EWS/Ets fusion oncoproteins, which cause widespread alterations in gene expression in the cell. Dysregulation of receptor tyrosine kinase signaling, particularly involving IGF-1R, also plays an important role in Ewing Sarcoma pathogenesis. However, the basis of this dysregulation, including the relative contribution of EWS/Ets-dependent and independent mechanisms, is not well understood. In the present study, we identify variable expression of two modifiers of PI3K signaling activity, PIK3R3 and PTEN, in Ewing Sarcoma, and examine the consequences of this on PI3K pathway regulation and oncogenic phenotypes. Our findings indicate that PIK3R3 plays a growth-promotional role in Ewing Sarcoma, but suggest that this role is not strictly dependent on regulation of PI3K pathway activity. We further show that expression of PTEN, a well-established, potent tumor suppressor, is lost in a subset of Ewing Sarcomas, and that this loss strongly correlates with high baseline PI3K pathway activity in cell lines. In support of functional importance of PTEN loss in Ewing Sarcoma, we show that re-introduction of PTEN into two different PTEN-negative Ewing Sarcoma cell lines results in downregulation of PI3K pathway activity, and sensitization to the IGF-1R small molecule inhibitor OSI-906. Our findings also suggest that PTEN levels may contribute to sensitivity of Ewing Sarcoma cells to the microtubule inhibitor vincristine, a relevant chemotherapeutic agent in this cancer. Our studies thus identify PIK3R3 and PTEN as modifiers of oncogenic phenotypes in Ewing Sarcoma, with potential clinical implications.

  7. [Synovial sarcoma. Case report].

    Science.gov (United States)

    Deme, Dániel; Abdulfatah, Bishr; Telekes, András

    2016-02-01

    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

  8. 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.

  9. Calcific bursitis mimicking a parosteal osteogenic sarcoma

    International Nuclear Information System (INIS)

    A 43-year-old woman with no history of trauma or major medical illness, presented with a ten day history of right hip and thigh pain. The pain was described as constant, dull, and aching. It was nonradiating and was not relieved by analgesics. Physical examination revealed diffuse tenderness over the right hip and right lateral thigh region; no mass was palpable. The CBC, serum electrolytes, calcium, phosphorus, and alkaline phosphatase determinations were all normal. Radiographs of the right hip demonstrated amorphous soft tissue calcification adjacent to the lateral aspect of the right femur as well as periosteal reaction and apparent destruction in the adjacent bone. Because of these suspicious X-rays findings, the initial working diagnosis was parosteal osteogenic sarcoma. A bone scan was performed two hours after the intravenous administration of 15 millicuries of Tc-99m-MDP. It showed focal uptake overlying the upper femur, approximately where the X-ray had shown periosteal reaction and apparent bony destruction. In addition, the bone images also demonstrated a linear band of activity extending through the soft tissues from the greater trochanter to the lower lateral thigh. Because of the unexpected and quite extensive soft tissue uptake seen on the scan, the possibility that a benign process was involved was then considered seriously for the first time. An open biopsy was then performed. It revealed acute calcific trochanteric bursitis; there was no evidence of bone involvement. The patient was treated conservatively and symptoms gradually resolved. (orig.)

  10. Lumbar Myeloid Cell Trafficking into Locomotor Networks after Thoracic Spinal Cord Injury.

    Science.gov (United States)

    Hansen, Christopher N; Norden, Diana M; Faw, Timothy D; Deibert, Rochelle; Wohleb, Eric S; Sheridan, John F; Godbout, Jonathan P; Basso, D Michele

    2016-08-01

    Spinal cord injury (SCI) promotes inflammation along the neuroaxis that jeopardizes plasticity, intrinsic repair and recovery. While inflammation at the injury site is well-established, less is known within remote spinal networks. The presence of bone marrow-derived immune (myeloid) cells in these areas may further impede functional recovery. Previously, high levels of the gelatinase, matrix metalloproteinase-9 (MMP-9) occurred within the lumbar enlargement after thoracic SCI and impeded activity-dependent recovery. Since SCI-induced MMP-9 potentially increases vascular permeability, myeloid cell infiltration may drive inflammatory toxicity in locomotor networks. Therefore, we examined neurovascular reactivity and myeloid cell infiltration in the lumbar cord after thoracic SCI. We show evidence of region-specific recruitment of myeloid cells into the lumbar but not cervical region. Myeloid infiltration occurred with concomitant increases in chemoattractants (CCL2) and cell adhesion molecules (ICAM-1) around lumbar vasculature 24h and 7days post injury. Bone marrow GFP chimeric mice established robust infiltration of bone marrow-derived myeloid cells into the lumbar gray matter 24h after SCI. This cell infiltration occurred when the blood-spinal cord barrier was intact, suggesting active recruitment across the endothelium. Myeloid cells persisted as ramified macrophages at 7days post injury in parallel with increased inhibitory GAD67 labeling. Importantly, macrophage infiltration required MMP-9. PMID:27191729

  11. Synovial Sarcoma: Laryngopharynx a Challenge

    OpenAIRE

    Verma, Ravinder; Verma, Ravneet Ravinder; Verma, Rohan Ravinder; Sardana, N. K.

    2013-01-01

    Synovial sarcoma is a rare malignant tumor. It derives from a mesenchymal precursor stem cell that is unrelated to mature synovial tissue. Synovial sarcoma classically affects lower limbs between the ages of 15 and 40 years and the proportion of male-to-female patients is 3:2. It is very rare in the head and neck region especially in laryngopharynx. Till date, only six cases of synovial sarcoma involving laryngopharynx have been reported in the English literature. Painless mass, hoarseness, u...

  12. The Role of Chemokine Receptor CXCR4 in the Biologic Behavior of Human Soft Tissue Sarcoma

    Directory of Open Access Journals (Sweden)

    Roger H. Kim

    2011-01-01

    Full Text Available The molecular basis of sarcoma remains poorly understood. However, recent studies have begun to uncover some of the molecular pathways involved in sarcomagenesis. The chemokine receptor CXCR4 has been implicated in sarcoma development and has been found to be a prognostic marker for poor clinical outcome. There is growing evidence that overexpression of CXCR4 plays a significant role in development of metastatic disease, especially in directing tumor cells towards the preferential sites of metastases in sarcoma, lung and bone. Although further investigation is necessary to validate these pathways, there is potential for clinical application, particularly in the use of pharmacologic inhibitors of CXCR4 as means of preventing sarcoma metastasis.

  13. Synovial Sarcoma Associated With Indwelling Intramedullary Pin in a Peach-Faced Lovebird (Agapornis roseicollis).

    Science.gov (United States)

    Nakano, Yumiko; Une, Yumi

    2016-03-01

    Sarcoma developing in association with a metallic orthopedic procedure is an uncommon but well-recognized complication in mammals. We report on a synovial sarcoma that developed at the site of an intramedullary pin after surgery to treat a bone fracture. A 17-year-old female peach-faced lovebird (Agapornis roseicollis) developed a spherical mass on the distal right dorsal wing at a site that was previously fractured and surgically repaired with an indwelling intramedullary pin. The right wing was amputated at the scapulohumeral joint. One year later, the bird died. Postmortem examination revealed metastases in the right lung, left thoracic wall, and proventricular serosa. Histologically, the tumor had a characteristic biphasic pattern. The tumor was immunohistologically and ultrastructurally identified as a synovial sarcoma. This is the first report of a suspected fracture-associated sarcoma in a bird. PMID:27088741

  14. Extraskeletal Ewing’s Sarcoma Arising from the Sciatic Nerve: A Diagnostic Challenge

    Directory of Open Access Journals (Sweden)

    Aadhar Sharma

    2015-01-01

    Full Text Available Ewing’s sarcoma is a common bone tumour of childhood but is a rare occurrence in individuals over 20 years of age. Few cases are reported as originating from peripheral nerves. We present an unusual case of extraosseous Ewing’s sarcoma originating from the sciatic nerve in a 66-year-old patient which had the clinical hallmarks of a benign nerve sheath tumour. Following discussion at a multidisciplinary meeting, excision biopsy of the suspected benign nerve sheath tumour was planned. At operation, the mass had malignant features. Histology confirmed the presence of Ewing’s sarcoma. Due to the morbidity of nerve resection, radiotherapy and chemotherapy were commenced. Ewing’s sarcoma is known to mimic benign pathologies. In this case there were subtle signs of a malignant process in the form of unremitting pain. It is vital to keep in mind the less common tumours that can affect the peripheral nervous system in such cases.

  15. Copy Number Alterations and Methylation in Ewing's Sarcoma

    Directory of Open Access Journals (Sweden)

    Mona S. Jahromi

    2011-01-01

    Full Text Available Ewing's sarcoma is the second most common bone malignancy affecting children and young adults. The prognosis is especially poor in metastatic or relapsed disease. The cell of origin remains elusive, but the EWS-FLI1 fusion oncoprotein is present in the majority of cases. The understanding of the molecular basis of Ewing's sarcoma continues to progress slowly. EWS-FLI1 affects gene expression, but other factors must also be at work such as mutations, gene copy number alterations, and promoter methylation. This paper explores in depth two molecular aspects of Ewing's sarcoma: copy number alterations (CNAs and methylation. While CNAs consistently have been reported in Ewing's sarcoma, their clinical significance has been variable, most likely due to small sample size and tumor heterogeneity. Methylation is thought to be important in oncogenesis and balanced karyotype cancers such as Ewing's, yet it has received only minimal attention in prior studies. Future CNA and methylation studies will help to understand the molecular basis of this disease.

  16. MRI findings of central nervous system granulocytic sarcoma (chloroma)

    International Nuclear Information System (INIS)

    To characterize MRI findings of central nervous system (CNS) granulocytic sarcoma (chloroma) and to analyse the points which differentiate it from other CNS tumors. We evaluated MRI in six patients with CNS granulocytic sarcoma proven by surgery or bone marrow biopsy (intracranical, one case and spine five cases). A 0.5T superconductive MR machine was used for diagnosis and, axial, coronal and sagittal T1- and T2-weighted spin echo images and Gd-DTPA enhanced T1-weighted images were obtained. We retrospectively analized the location, signal intensity, margin, contrast enhancement and homogeneity, and bony change around the tumor. MRI findings of CNS granulocytic sarcomas were as follows : one tumor was seen to be an extra-axial mass in the posterior fossa of the brain, four were epidural, and one was an epidural and presacral masses in the spine;tumor magins were lobulated and three were smooth. On T1-weighted images, all tumors were of isoignal intensity;on T2-weighted images, four were of isosignal intersity and two were of high signal intensity. Contrast enhancement was inhomogeneous in five of six cases. Bony change around the tumor was seen in two cases. On T1-weighted images, CNS granulocytic sarcomas (chloromas) were of isosignal intensity, relative to brain parenchyma or spinal cord;on T2-weighted images, they were of iso or high signal intensity, with relative contrast enhancement. These points could be useful in differentiating them from other CNS tumors

  17. The diagnosis and differential diagnosis of granulocytic sarcoma%探讨粒细胞肉瘤的诊断与鉴别诊断

    Institute of Scientific and Technical Information of China (English)

    Xiaoli Feng; Jianming Ying; C. Cameron Yin; Ling Li; Susheng Shi; Hongtu Zhang; Yuntian Sun

    2008-01-01

    Objective: To discuss the diagnosis and differential diagnosis of granulocytic sarcoma (GS). Methods: Six cases were reported in this paper. They were assessed by pathologists. Immunohistochemistry (IHC) stain and routine hematoxylin and eosin (H&E) stain were applied. Results:All patients involved in different anatomic sites respectively including skin, lymph node, soft tissue, breast, cervix and penis. All cases were previously error diagnoses. Three of them were initially diagnosed as non-Hodgkin lymphoma (NHL). One case of cervical lymph node lesion was first considered as metastasized carcinoma by clinician. One biopsied skin sample was initially reported as Karposi's sarcoma. And one breast case was suspicious of the Iobular carcinoma with the frozen samples without antecedent clinical history information. GS was accompanied with acute myeloid leukemia (AML) in one case and with acute lymphocytic leukemia (ALL) in one case. Histopathologically, blastic, immature and differentiated variants were found in four, one and one, respectively. Immunohistochemistry (IHC) showed that myeloperoxidase (MPO) and lysozyme were both found to be positive in all cases, CD43 was found in 5 of 6 cases. Three of six cases were CD68, CD15 and LCA positive. CD34 and CD117 were positive in 1/5 and 1/6 cases, respectively. However, CD20 and CD3 were negative in all cases. Conclusion: GS was uncommon and it may be misdiagnosed easily in routine practice. Each area had its own character, but they had the common features too. It can be correctly diagnosed by combination of H&E stain, IHC stain, peripheral blood and bone marrow. MPO and Lysozyme were necessary for the nature of granulocytes. In addition, CD43, CD68 and CD15 were very helpful.

  18. Treatment Options for Kaposi Sarcoma

    Science.gov (United States)

    ... in the United States have been diagnosed in homosexual or bisexual men infected with HIV. Signs of ... type of nonepidemic Kaposi sarcoma that develops in homosexual men who have no signs or symptoms of ...

  19. General Information about Kaposi Sarcoma

    Science.gov (United States)

    ... in the United States have been diagnosed in homosexual or bisexual men infected with HIV. Signs of ... type of nonepidemic Kaposi sarcoma that develops in homosexual men who have no signs or symptoms of ...

  20. Treatment Option Overview (Kaposi Sarcoma)

    Science.gov (United States)

    ... in the United States have been diagnosed in homosexual or bisexual men infected with HIV. Signs of ... type of nonepidemic Kaposi sarcoma that develops in homosexual men who have no signs or symptoms of ...

  1. Post irradiation maxillary sinus sarcoma

    International Nuclear Information System (INIS)

    Radiation-induced cancer of the head and neck is well known, but radiation-induced sarcoma is rare. Only nine cases have been reported in the Japanese literature. We treated a patient with post irradiation sarcoma arising in the maxillary sinus. She is now 60 years old and had received radiation therapy for left maxillary carcinoma in 1952. In 1972, left maxillary sarcoma was found and she received postoperative radiation therapy again. Then right nasal carcinoma was found in 1980, and she again received postoperative radiation therapy. Then left maxillary sarcoma was found in 1992. There have been no previous reports of three consecutive radiation-induced malignancies developing in a patient. (author)

  2. 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.

  3. Can Kaposi Sarcoma Be Prevented?

    Science.gov (United States)

    ... KS) is caused by the Kaposi sarcoma associated herpesvirus (KSHV). There are no vaccines at this time ... HIV-infected people who take drugs to treat herpesvirus infections (such as ganciclovir or foscarnet) are less ...

  4. Trabectedin in Soft Tissue Sarcomas

    OpenAIRE

    Petek, Bradley J.; Loggers, Elizabeth T.; Pollack, Seth M.; Jones, Robin L.

    2015-01-01

    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 ag...

  5. Synovial sarcoma--a misnomer.

    OpenAIRE

    Miettinen, M.; Virtanen, I

    1984-01-01

    For an evaluation of the putative histogenetic relationship of synovia and synovial sarcomas, normal synovia, villonodular synovitis, and synovial sarcomas were compared for their patterns of expression of intermediate filaments of keratin and vimentin type and epithelial membrane antigen and for lectin binding sites. The lining cells in both normal synovia and villonodular synovitis reacted with anti-vimentin antibodies, but not with antibodies to different types of keratins or epithelial me...

  6. Primary renal synovial sarcoma

    Directory of Open Access Journals (Sweden)

    Vandana U Grampurohit

    2011-01-01

    Full Text Available Primary synovial sarcoma (SS of kidney is very rare and difficult to diagnose. Here, we present a case of a 21-year-old female clinically diagnosed as renal cell carcinoma. Right nephrectomy specimen showed a cystic tumor in the upper pole of kidney with areas of hemorrhage and solid growth. Histologically, it showed poorly differentiated cells with hemangiopericytoma-like vascular pattern. Morphologic and immunohistochemical features were compatible with the diagnosis of poorly differentiated SS of kidney. Primary renal SS is a recently described entity. To the best of our knowledge, approximately 34 cases have been reported till date and this is the eighth documented case of poorly differentiated variant. Most of the time, poorly differentiated SS of kidney exhibits hemangiopericytoma like histology. Reverse transcriptase-polymerase chain reaction analysis to demonstrate SYT-SSX fusion gene transcript helps to confirm the diagnosis.

  7. Clear cell sarcoma

    Directory of Open Access Journals (Sweden)

    Pinar Ozuguz

    2014-01-01

    Full Text Available Malignant melanoma (MM of soft tissue, also called clear cell sarcoma (CCS of tendons and aponeuroses, derives from the neural crest. CCS is similar morphologically to MM but has no precursor skin lesion, and instead, has a characteristic chromosomal translocation. Prognosis is related to the tumor size. Early recognition and initial radical surgery is the key to a favorable outcome. The tumor has to be differentiated from other benign and malignant lesions of the soft tissues, such as fibrosarcoma. The demonstration of melanin and a positive immunohistochemical reaction for S-100 protein and HMB-45 can assist in the differential diagnosis. We report the case of a 58-year-old woman with CCS arising from the soft tissue of her little finger.

  8. Radiosensitivity of soft tissue sarcomas

    Energy Technology Data Exchange (ETDEWEB)

    Hirano, Toru; Iwasaki, Katsuro; Suzuki, Ryohei; Monzen, Yoshio; Hombo, Zenichiro

    1989-05-01

    The correlation between the effectiveness of radiation therapy and the histology of soft tissue sarcomas was investigated. Of 31 cases with a soft tissue sarcoma of an extremity treated by conservative surgery and postoperative radiation of 3,000-6,000 cGy, local recurrence occurred in 12; 5 out of 7 synovial sarcomas, 4 of 9 MFH, one of 8 liposarcomas, none of 4 rhabdomyosarcomas and 2 of 3 others. As for the histological subtyping, the 31 soft tissue sarcomas were divided into spindle cell, pleomorphic cell, myxoid and round cell type, and recurrence rates were 75%, 33.3%, 16.7% and 0%, respectively. From the remarkable difference in recurrent rate, it was suggested that round cell and myxoid type of soft tissue sarcomas showed a high radiosensitivity compared to the spindle cell type with low sensitivity. Clarifying the degree of radiosensitivity is helpful in deciding on the management of limb salvage in soft tissue sarcomas of an extremity. (author).

  9. Chronic Myeloid Leukemia

    Science.gov (United States)

    Leukemia is cancer of the white blood cells. White blood cells help your body fight infection. Your blood cells form in your bone marrow. In leukemia, the bone marrow produces abnormal white blood cells. ...

  10. Acute Myeloid Leukemia

    Science.gov (United States)

    Leukemia is cancer of the white blood cells. White blood cells help your body fight infection. Your blood cells form in your bone marrow. In leukemia, however, the bone marrow produces abnormal white blood ...

  11. Tissue characteristics of high- and low-incidence plutonium-induced osteogenic sarcoma sites in life-span beagles

    International Nuclear Information System (INIS)

    On the basis of information gathered from the 239Pu life-span study in beagles at the University of Utah, the tissue features were found to be characteristic of high-incidence bone-tumor sites compared to low-incidence sites included more hematopoietic tissues in the bone marrow; greater trabecular bone mass; greater bone remodeling rates; greater mineral apposition rates; greater density and activity of bone surface cells; greater density of putative bone-cell precursors; greater initial uptake of plutonium on bone surfaces; and greater marrow vascular volumes and a venous sinusoidal bed. Although most of these studies are not yet complete, the information being collected should contribute to our understanding of the mechanisms of radiation-induced osteogenic sarcomas. This should aid in predicting the types and characteristics of osseous tissues where radiation-induced osteogenic sarcomas may arise in humans. 25 refs., 4 figs., 3 tabs

  12. Osteogenic sarcoma of the breast arising in a cystosarcoma phyllodes: a case report and review of the literature

    Directory of Open Access Journals (Sweden)

    Chintamani

    2011-07-01

    Full Text Available Abstract Introduction Primary tumors of the breast containing bone and cartilage are extremely rare, and an osteogenic sarcoma arising from a cystosarcoma phyllodes is exceptional. Case presentation A 40-year-old Indian woman presented with a breast mass which was diagnosed as osteosarcoma of the breast on biopsy. Our patient was treated with a simple mastectomy after excluding the presence of skeletal primary and extra-mammary metastases. Final pathology showed a cystosarcoma phyllodes with signs of osteogenic sarcoma. Conclusion Although osteogenic sarcomas of the breast are rare, they need to be distinguished from carcinosarcomas and metaplastic carcinomas as the management of the two differ.

  13. Sorafenib in Treating Patients With Metastatic, Locally Advanced, or Recurrent Sarcoma

    Science.gov (United States)

    2014-05-07

    Adult Angiosarcoma; Adult Epithelioid Sarcoma; Adult Leiomyosarcoma; Adult Malignant Fibrous Histiocytoma; Adult Neurofibrosarcoma; Adult Synovial Sarcoma; Ovarian Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Uterine Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage III Uterine Sarcoma; Stage IV Adult Soft Tissue Sarcoma; Stage IV Uterine Sarcoma; Uterine Carcinosarcoma; Uterine Leiomyosarcoma

  14. BCL11A expression in acute phase chronic myeloid leukemia.

    Science.gov (United States)

    Yin, Jiawei; Zhang, Fan; Tao, Huiquan; Ma, Xiao; Su, Guangsong; Xie, Xiaoli; Xu, Zhongjuan; Zheng, Yanwen; Liu, Hong; He, Chao; Mao, Zhengwei Jenny; Wang, Zhiwei; Chang, Weirong; Gale, Robert Peter; Wu, Depei; Yin, Bin

    2016-08-01

    Chronic myeloid leukemia (CML) has chronic and acute phases. In chronic phase myeloid differentiation is preserved whereas in acute phase myeloid differentiation is blocked. Acute phase CML resembles acute myeloid leukemia (AML). Chronic phase CML is caused by BCR-ABL1. What additional mutation(s) cause transition to acute phase is unknown and may differ in different persons with CML. BCL11A encodes a transcription factor and is aberrantly-expressed in several haematological and solid neoplasms. We analyzed BCL11A mRNA levels in subjects with chronic and acute phase CML. BCL11A transcript levels were increased in subjects with CML in acute phase compared with those in normals and in subjects in chronic phase including some subjects studied in both phases. BCL11A mRNA levels were correlated with percent bone marrow blasts and significantly higher in lymphoid versus myeloid blast crisis. Differentiation of K562 with butyric acid, a CML cell line, decreased BCL11A mRNA levels. Cytology and flow cytometry analyses showed that ectopic expression of BCL11A in K562 cells blocked differentiation. These data suggest BCL11A may operate in transformation of CML from chronic to acute phase in some persons. PMID:27285855

  15. The Role of Myeloid-Derived Suppressor Cells in Immune Ontogeny

    OpenAIRE

    Gantt, Soren; Gervassi, Ana; Jaspan, Heather; Horton, Helen

    2014-01-01

    Myeloid-derived suppressor cells (MDSC) are a heterogeneous population of granulocytic or monocytic cells that suppress innate as well as adaptive immune responses. In healthy adults, immature myeloid cells differentiate into macrophages, dendritic cells, and granulocytes in the bone marrow and MDSC are rarely detected in peripheral blood. However, in certain pathologies, in particular malignancies and chronic infection, differentiation of these cells is altered resulting in accumulation of c...

  16. Management of pulmonary Kaposi's sarcoma: new perspectives

    International Nuclear Information System (INIS)

    Pulmonary Kaposi's sarcoma (KS) is a frequent complication of human immunodeficiency virus infection. This article focuses on the current approach to diagnosis and treatment of Kaposi's sarcoma involving the respiratory system. (author). 27 refs., 3 figs., 5 tabs

  17. Pericardial Synovial Sarcoma: A Rare Clinical Entity.

    Science.gov (United States)

    Goldblatt, Joshua; Saxena, Pankaj; McGiffin, David C; Zimmet, Adam

    2015-11-01

    Synovial sarcoma is an extremely rare form of primary malignancy of the pericardium. We present a case of primary synovial sarcoma of the pericardium followed by a review of the literature. PMID:26347295

  18. 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 ...

  19. Soft Tissue Sarcomas and Agent Orange

    Science.gov (United States)

    ... ZIP code here Soft Tissue Sarcomas and Agent Orange VA presumes some soft tissue sarcomas in Veterans are related to their exposure to Agent Orange or other herbicides during military service. The soft ...

  20. The molecular biology of radiation-induced carcinogenesis: thymic lymphoma, myeloid leukaemia and osteosarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Janowski, M. (Centre d' Etude de l' Energie Nucleaire, Mol (Belgium)); Cox, R. (Medical Research Council, Harwell (UK). Radiobiological Research Unit); Strauss, P.G. (GSF, Neuherberg (Germany, F.R.). Abt. fuer Molekulare Zellpathologie)

    1990-04-01

    In mice, external X- or {gamma}-irradiation may induce thymic lymphomas or myeloid leukaemias, while bone-seeking {alpha}-emitters may induce osteosarcomas, and to a lesser extent acute myeloid leukaemia. The paper reviews briefly some experimental data in respect to molecular mechanisms underlying these radio-carcinogenic processes. Thymic lymphomagenesis proceeds by an indirect mechanism in which recombinant proviruses could be involved. Myeloid leukaemogenesis is characterized by a very early putative initiating event, consisting of non-random rearrangements and/or deletions of chromosome 2. Osteosarcomagenesis in mice is often associated with the expression of proviruses, and the tumors often contain somatically acquired proviruses. (UK).

  1. Computed tomography in malignant primary bone tumours

    International Nuclear Information System (INIS)

    The importance of computed tomography is examined in malignant primary bone tumours using a strongly defined examination group of 13 Patients (six Ewing's-sarcomas, five osteosarcomas, one chondrosarcoma and one spindle-shaped cell sarcoma). Computed tomography is judged superior compared to plain radiographs in recognition of bone marrow infiltration and presentation of parosteal tumour parts as well as in analysis of tissue components of tumours, CT is especially suitable for therapy planning and evaluating response to therapy. CT does not provide sufficient diagnostic information to determine dignity and exact diagnosis of bone tumours. (orig.)

  2. Soft tissue sarcomas of the kidney

    OpenAIRE

    Olivia Köhle; Dominik Abt; Christian Rothermundt; Christian Öhlschlegel; Christiane Brugnolaro; Hans-Peter Schmid

    2015-01-01

    Soft tissue sarcomas are rare mesenchymal tumors. Amongst others, primitive neuroectodermal tumors (PNET) of the kidney and synovial sarcoma of the kidney belong to the group of soft tissue sarcomas. Synovial sarcomas can occur almost anywhere in the body, most frequently, however, in the lower (62%) or upper extremities (21%). Metastases occur in 50-70% of cases, and thus the prognosis is poor. PNETs are rare, highly aggressive neoplastic lesions which mainly occur in the torso or axial skel...

  3. Synovial sarcoma of the sellar region

    OpenAIRE

    Scheithauer, BW; Silva, AI; Kattner, K; Seibly, J; Oliveira, AM; Kovacs, K.

    2007-01-01

    Primary sarcomas of the sellar region are uncommon, although a wide variety have been reported. To date, no cases of primary synovial sarcoma have been described as occurring at this site. We report an immunohistochemically and molecular genetically confirmed primary synovial sarcoma involving the sellar/parasellar region and cavernous sinus in an adult male. Subtotal resection and radiosurgery proved to be efficacious. The spectrum of primary sellar region sarcomas is summarized.

  4. Mesenchymal stem cells: From stem cells to sarcomas.

    Science.gov (United States)

    Lye, Kwan Liang; Nordin, Norshariza; Vidyadaran, Sharmili; Thilakavathy, Karuppiah

    2016-06-01

    Mesenchymal stem cells (MSCs) have garnered vast interests in clinical settings, especially in regenerative medicine due to their unique properties-they are reliably isolated and expanded from various tissue sources; they are able to differentiate into mesodermal tissues such as bones, cartilages, adipose tissues, and muscles; and they have unique immunosuppressive properties. However, there are some concerns pertaining to the role of MSCs in the human body. On one hand, they are crucial component in the regeneration and repair of the human body. On the contrary, they are shown to transform into sarcomas. Although the exact mechanisms are still unknown, many new leads have pointed to the belief that MSCs do play a role in sarcomagenesis. This review focuses on the current updates and findings of the role of MSCs in their transformation process into sarcomas. PMID:26992453

  5. Synovial sarcoma of the abdominal wall

    International Nuclear Information System (INIS)

    A case report of synovial sarcoma arising in the abdominal wall is presented. A brief review of the clinical and radiological features of synovial sarcoma is made. Pre-operative diagnosis of an abdominal wall synovial sarcoma is virtually impossible, but should be considered when a soft tissue swelling is found to show amorphous stippled calcification X-ray. (author)

  6. Synovial sarcoma; Maziowczak zlosliwy

    Energy Technology Data Exchange (ETDEWEB)

    Kopacz, A.; Jastrzebski, T.; Jaskiewicz, J. and others [Klinika Chirurgii Onkologicznej, Akademia Medyczna, Gdansk (Poland)

    1995-12-31

    Authors present the material of 12 patients with synovial sarcoma treated in Surgical Oncology Dept. Medical University of Gdansk between 1973-1993. In all cases the main symptom was a growing tumor; tumor accompanied by pain in 3 of them. Average time for symptoms was about 9 months. Tumors were classified according to GTNM and Enneking Classification. 9 cases were classified as G2T1NOMO and 3 as G2T2NOMO. According to Enneking Classification 1 case only was recognized as intracompartmental and 11 as extracompartmental. In 9 tumors cytogenetic analysis was done. In 8 of them specific chromosome aberration was discovered - translocation between X and 18 chromosomes: t (18;X)(p11;q11). The following procedures were performed: enucleation of tumor (non-radical) in 2 cases, wide excision in 8 cases, extremity amputation in 1 case. Local recurrence was observed in 9 cases. 30 months without recurrence was noticed as an average. The wide local excision was the method of treatment of local recurrence in 4 cases (followed by RTG-therapy in one case). In another 5 cases extremity amputation was performed (followed by CyVADic chemotherapy in one case). In our material 5-year survival was observed in 36% of cases. The longest notice follow-up was 30 years. (author) 20 refs, 3 tabs, 1 fig

  7. Cerebellar, Pancreatic, and Paraspinal Metastases in Soft Tissue Sarcomas: Unusual Sites or Changing Patterns?

    Directory of Open Access Journals (Sweden)

    Girish Bedre

    2007-07-01

    Full Text Available Context Soft tissue sarcomas generally first metastasize to the lungs followed by the involvement of other sites such as lymph nodes and bones as part of the disseminated disease. Cerebellar and pancreatic metastases from tumors of mesenchymal origin such as soft tissue sarcomas are exceptional, more so in the absence of pulmonary metastases. Case report A previously treated case of chest wall sarcoma presented with the sudden onset of neurological symptoms. An MRI brain scan was suggestive of a solitary cerebellar metastasis. A CT scan of the thorax and abdomen showed no evidence of disease. A metastasectomy of the solitary brain lesion confirmed a deposit from a previously treated sarcoma. Within two months he presented with central abdominal pain and low backache radiating down both lower limbs. FDG-PET and CT scans revealed a large pancreatic and left paraspinal mass with intense tracer uptake suggestive of metastatic involvement. There was no evidence of pulmonary metastases. A CT-guided biopsy was suggestive of high-grade sarcoma. He was treated with palliative radiotherapy with good symptomatic relief. Conclusion Cerebellar, pancreatic, and paraspinal metastases from soft tissue sarcomas are rare, especially in the absence of pulmonary metastases. A high index of suspicion is necessary, and appropriate imaging should be considered for symptomatic patients.

  8. Radiation Therapy for Chloroma (Granulocytic Sarcoma)

    Energy Technology Data Exchange (ETDEWEB)

    Bakst, Richard; Wolden, Suzanne [Department of Radiation Oncology, Memorial Sloan-Kettering Cancer Center, New York, NY (United States); Yahalom, Joachim, E-mail: yahalomj@mskcc.org [Department of Radiation Oncology, Memorial Sloan-Kettering Cancer Center, New York, NY (United States)

    2012-04-01

    Objectives: Chloroma (granulocytic sarcoma) is a rare, extramedullary tumor of immature myeloid cells related to acute nonlymphocytic leukemia or myelodysplastic syndrome. Radiation therapy (RT) is often used in the treatment of chloromas; however, modern studies of RT are lacking. We reviewed our experience to analyze treatment response, disease control, and toxicity associated with RT to develop treatment algorithm recommendations for patients with chloroma. Patients and Methods: Thirty-eight patients who underwent treatment for chloromas at our institution between February 1990 and June 2010 were identified and their medical records were reviewed and analyzed. Results: The majority of patients that presented with chloroma at the time of initial leukemia diagnosis (78%) have not received RT because it regressed after initial chemotherapy. Yet most patients that relapsed or remained with chloroma after chemotherapy are in the RT cohort (90%). Thirty-three courses of RT were administered to 22 patients. Radiation subsite breakdown was: 39% head and neck, 24% extremity, 9% spine, 9% brain, 6% genitourinary, 6% breast, 3% pelvis, and 3% genitourinary. Median dose was 20 (6-36) Gy. Kaplan-Meier estimates of progression-free survival and overall survival in the RT cohort were 39% and 43%, respectively, at 5 years. At a median follow-up of 11 months since RT, only 1 patient developed progressive disease at the irradiated site and 4 patients developed chloromas at other sites. RT was well tolerated without significant acute or late effects and provided symptom relief in 95% of cases. Conclusions: The majority of patients with chloromas were referred for RT when there was extramedullary progression, marrow relapse, or rapid symptom relief required. RT resulted in excellent local disease control and palliation of symptoms without significant toxicity. We recommend irradiating chloromas to at least 20 Gy, and propose 24 Gy in 12 fractions as an appropriate regimen.

  9. Granulocytic Sarcoma Presenting as Atypical Mastoiditis with Facial Paralysis: Description of a Case

    Directory of Open Access Journals (Sweden)

    M. Crovetto

    2011-01-01

    Full Text Available We describe a case of temporal granulocytic sarcoma in a 26-year-old patient after apparent molecular remission of an acute myeloid leukaemia. He complained of otodynia with hearing loss and facial paralysis on the right side. He was treated with chemotherapy and self-transplant haematopoietic stem cells. He was cured clinically, molecular remission of the haematological processes was achieved, and he remained asymptomatic for three years. Facial paralysis and hearing loss associated with temporal GS should be treated with chemotherapy. Aggressive surgery may complicate the clinical course of the disease and it should be avoided.

  10. Nonleukemic granulocytic sarcoma of kidney with mixed phenotype blasts: A diagnostic dilemma

    Directory of Open Access Journals (Sweden)

    Vinita Agrawal

    2011-01-01

    Full Text Available Granulocytic sarcoma (GS usually presents concomitantly with or after the onset of acute myeloid leukemia, blastic phase of chronic myeloid leukemia (CML, or myelodysplastic syndromes. Rarely, it may present even before the onset of overt leukemia and when so, it is often misdiagnosed. We are reporting a case of GS of kidney presenting as an isolated renal mass with normal laboratory investigations including a normal peripheral blood smear. It was initially misdiagnosed as lymphoma as the blasts, in addition to the morphological similarity with lymphoma cells, also showed positive immunohistochemistry for B cell markers. Based on further investigations including immunophenotyping and cytogenetic studies, a final diagnosis of CML-blast crisis (mixed phenotype presenting initially as GS was made. To the best of our knowledge, this is the first antemortem report of nonleukemic GS presenting as kidney mass that later on progressed to CML-blast crisis with mixed phenotype blasts.

  11. Primary Ewing's sarcoma of the vertebral column

    International Nuclear Information System (INIS)

    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 0.53. Disease

  12. Magnetic resonance imaging features of extremity sarcomas of uncertain differentiation

    International Nuclear Information System (INIS)

    The purpose of this review is to illustrate the pertinent clinical and imaging features of extremity sarcomas of uncertain differentiation, including synovial sarcoma, epithelioid sarcoma, clear-cell sarcoma, and alveolar soft part sarcoma. These tumours should be considered in the differential diagnosis when a soft-tissue mass is encountered in the extremity of an adolescent or young adult

  13. Magnetic resonance imaging features of extremity sarcomas of uncertain differentiation

    Energy Technology Data Exchange (ETDEWEB)

    Stacy, G.S. [Department of Radiology, University of Chicago Hospitals, Chicago, Illinois (United States)], E-mail: sstacy@radiology.bsd.uchicago.edu; Nair, L. [Department of Radiology, University of Chicago Hospitals, Chicago, Illinois (United States)

    2007-10-15

    The purpose of this review is to illustrate the pertinent clinical and imaging features of extremity sarcomas of uncertain differentiation, including synovial sarcoma, epithelioid sarcoma, clear-cell sarcoma, and alveolar soft part sarcoma. These tumours should be considered in the differential diagnosis when a soft-tissue mass is encountered in the extremity of an adolescent or young adult.

  14. Transcriptional Heterogeneity and Lineage Commitment in Myeloid Progenitors

    DEFF Research Database (Denmark)

    Paul, Franziska; Arkin, Ya'ara; Giladi, Amir;

    2015-01-01

    Within the bone marrow, stem cells differentiate and give rise to diverse blood cell types and functions. Currently, hematopoietic progenitors are defined using surface markers combined with functional assays that are not directly linked with in vivo differentiation potential or gene regulatory...... mechanisms. Here, we comprehensively map myeloid progenitor subpopulations by transcriptional sorting of single cells from the bone marrow. We describe multiple progenitor subgroups, showing unexpected transcriptional priming toward seven differentiation fates but no progenitors with a mixed state...... that in vivo priming may still allow for plasticity given strong perturbations. These data establish a reference model and general framework for studying hematopoiesis at single-cell resolution....

  15. Para-oesophageal synovial sarcoma

    International Nuclear Information System (INIS)

    An unusual case of mediastinal synovial sarcoma with secondary invasion of the oesophagus simulating an intra-oesophageal mass is reported. The location and radiological appearance of this tumour are exceptional, and, to the authors' knowledge, have not been reported previously. (orig.)

  16. Synovial sarcoma of the larynx

    OpenAIRE

    Bhandari, Anita; Sharma, Man Prakash; Bapna, A. S.

    1998-01-01

    A 23 years old male presented with a soft cystic mass in the left paralaryngeal space since 3 months. Indirect laryngoscopy revealed a bulging of the left lateral pharyngeal wall. Histopathologic al examination of the biopsy proved it to be a synovial sarcoma. The case is the seventh case reported in literature.

  17. Para-oesophageal synovial sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Pulpeiro, J.R.; Cruz, R.; Arenas, A.; Perez-Espejo, G.

    1988-05-01

    An unusual case of mediastinal synovial sarcoma with secondary invasion of the oesophagus simulating an intra-oesophageal mass is reported. The location and radiological appearance of this tumour are exceptional, and, to the authors' knowledge, have not been reported previously.

  18. Immunotherapy in Sarcoma: Future Horizons.

    Science.gov (United States)

    Burgess, Melissa; Gorantla, Vikram; Weiss, Kurt; Tawbi, Hussein

    2015-11-01

    Immunologic approaches to cancer are over a century old. Over the years, the strategy has been fine-tuned from inciting infections in subjects to inhibiting negative regulatory signals from the innate immune system. Sarcomas are among the first tumors to be considered for immune interventions. From Coley's toxin to cytokine-based therapies to adoptive cell therapy, there have been numerous immunotherapeutic investigations in this patient population. A promising strategy includes adoptive T cell therapy which has been studied in small cohorts of synovial sarcoma, a subtype that is known to widely express the cancer testis antigen, NY-ESO-1. Additionally, recent data in metastatic melanoma and renal cell carcinoma demonstrate the utility and tremendous efficacy of immune checkpoint blockade with increased rates of durable responses compared to standard therapies. Responses in traditionally "non-immunogenic" tumors, such as lung and bladder cancers, provide ample rationale for the study of immune checkpoint inhibitors in sarcoma. While immunotherapy has induced some responses in sarcomas, further research will help clarify optimal patient selection for future clinical trials and new combinatorial immunotherapeutic strategies. PMID:26423769

  19. Synovial sarcoma of the parapharyngeal space: CT and MRI

    Energy Technology Data Exchange (ETDEWEB)

    Tillich, M.; Ranner, G.; Humer-Fuchs, U.; Lang-Loidolt, D. [Department of Radiology, Karl-Franzens University, Hospital and Medical School Graz, Auenbruggerplatz 9, A-8036 Graz (Austria)

    1998-04-01

    We present the CT and MRI findings of a histologically proven synovial sarcoma arising in the left parapharyngeal space of a 21-year-old man. CT was useful for confirming the presence of calcification within the tumour, which may be a favourable prognostic sign, and in excluding involvement of cortical bone. The CT and MRI findings were, however, nonspecific. MRI was superior to CT for assessing the topographical relationships of the tumour to the vessels and the invasion of neighbouring structures. (orig.) With 3 figs., 13 refs.

  20. Case report 501: Alveolar soft parts sarcoma with pulmonary metastases

    International Nuclear Information System (INIS)

    Alveolar soft part sarcoma (ASPS) is a rare, soft tissue malignancy, usually presenting in young adults. We report a patient with ASPS in the thigh, evaluated with plain films, computed tomography, and a radionuclide scan. The findings in this patient and those in which CT findings have been reported in the literature, show a similar appearance which we believe is highly suggestive of this unusual tumor. The combination of a soft tissue lesion, that on CT appears highly vascular, with features suggestive of an arteriovenous malformation, in association with multiple pulmonary nodules and a bone scan with features suggestive of infection should lead to a consideration of this rare entity. (orig./GDG)

  1. Magnetic resonance imaging of Ewing sarcoma: morphology and tumour delineation

    International Nuclear Information System (INIS)

    Magnetic resonance images (spin-echo techniques; T1-w pre/post gadolinium, T2-w) of 59 patients as part of a multicenter study were evaluated retrospectively. Qualitative image analysis was performed: signal intensity (point of reference extraosseous: muscle, intraosseous: bone marrow), enhancement patterns, lesion delineation and differentiation between tumor and oedema. Morphology of Ewing sarcoma in magnetic resonance imaging is rather uniform. The lesion is intra- and extraosseous sharply delineated, though tumour and oedema can be rarely differentiated. (orig./MG)

  2. Local Ablative Therapies to Metastatic Soft Tissue Sarcoma.

    Science.gov (United States)

    Gronchi, Alessandro; Guadagnolo, B Ashleigh; Erinjeri, Joseph Patrick

    2016-01-01

    The approach to metastatic soft tissue sarcoma is complex and depends upon several factors, such as the extent of the disease, the histologic subtype of the primary tumor, the disease-free interval, patient status and comorbidities, and previous treatments. The effect of systemic chemotherapy is suboptimal, therefore local ablative therapies are often considered when the disease is limited, especially if confined to a single site/organ. Historically, surgery has been considered the treatment of choice for isolated lung metastases. This approach also has been extended to metastases in the liver, although a formal demonstration of its benefit has never been provided. Radiation therapy instead has been mainly used to obtain pain control and to reduce the risk of bone fracture and cord compression. Advances in techniques, such as the development of more precise conformational modalities and the employment of particles, may change the role of this modality in the strategic approach to metastatic soft tissue sarcoma. Recently, the use of interventional radiology in this scenario has expanded. Ablative approaches, such as radiofrequency ablation and cryoablation, have shown durable eradication of tumors. Catheter-directed therapies, such as hepatic artery embolization, are potential techniques for treating the patient who has multiple unresectable liver metastases. Understanding the timing and role of these three different modalities in the multidisciplinary approach to metastatic soft tissue sarcoma is critical to provide better care and to personalize the approach to the single patient. PMID:27249769

  3. Juxtacortical osteogenic sarcoma of chondroblastic type on the maxilla

    International Nuclear Information System (INIS)

    39-year-old female had been treated for the exophytic mass on buccal aspect of the left maxillary posterior area 2 years and 8 months ago. Tentative diagnosis was obtained as fibrous dysplasia on clinical and radiographic examinations and histopathologic findings revealed as osteochondroma after bone trimming at that time. She revisited for the treatment of recurred lesions. We reviewed this case with clinical, radiologic and histopathologic standpoints retrospectively, and came to a conclusion that the tumor primarily occurred was juxtacortical osteogenic sarcoma and recurred due to inadequate treatment and then expanded over intramedullary. This case shows that the diagnosis of osteosarcoma should take account of the patient history, clinical, radiographic and histopathologic findings and it requires attentive follow up check. Retrospectively reviewed results were as follows ; At first visit, oral examination revealed a bony hard swelling on the buccal aspect of the left maxillary posterior area. Radiographically, a dense radiopaque mass was noted on the site. The lesion showed hot uptake of 99mTc-MDP. Histopathologic diagnosis was done as osteochondroma, but it was considered as osteogenic sarcoma when compared with the recurrent lesion. When she revisited for the treatment of multiple bony swelling on the left maxilla, radiograms showed typical features of malignancy such as widening of periodontal ligament space and sun-ray appearance, and coincided with benign characters as follows ; relatively well circumscribed lesion and expansion and displacement of the adjacent structures. Finally, histopathologic findings of the lesion was well differentiated chondroblastic osteogenic sarcoma.

  4. Kaposi's sarcoma in South Africa.

    Science.gov (United States)

    Sitas, F; Newton, R

    2001-01-01

    Kaposi's sarcoma was endemic in South Africa even before the advent of the human immunodeficiency virus (HIV). Between 1988 and 1996, the incidence of Kaposi's sarcoma in South Africa has risen at least threefold and continues to increase as the HIV epidemic grows. Research from South Africa has shown that infection with human herpesvirus 8 (HHV8) is associated with Kaposi's sarcoma but not with any other major cancer site or type. In addition, the risk of Kaposi's sarcoma increases with increasing antibody titer to HHV8, but, for a given titer, the risk is greater in HIV-seropositive compared with HIV-seronegative individuals. The age- and sex-standardized seroprevalence of HHV8 in black South African hospital patients was found to be slightly more than 30%; the seroprevalence of HHV8 increased with age and was similar in men and in women. The modes of transmission of HHV8 are yet to be fully elucidated. Limited evidence exists for sexual transmission in black South African adults, but mother-to-child and person-to-person transmission in childhood is also likely. Furthermore, the seroprevalence of HHV8 decreases with increasing levels of education and is lower in whites than in blacks, suggesting that factors associated with poverty may be important determinants of transmission. Future research should focus on risk factors for Kaposi's sarcoma in HHV8-infected individuals, on determinants and mode of transmission of HHV8, and on the elucidation of the effect of primary HHV8 infection in adults and in children. PMID:11158199

  5. Sapanisertib or Pazopanib Hydrochloride in Treating Patients With Locally Advanced or Metastatic Sarcoma

    Science.gov (United States)

    2016-06-01

    High Grade Sarcoma; Metastatic Leiomyosarcoma; Metastatic Malignant Peripheral Nerve Sheath Tumor; Metastatic Synovial Sarcoma; Metastatic Undifferentiated Pleomorphic Sarcoma; Myxofibrosarcoma; Recurrent Leiomyosarcoma; Recurrent Malignant Peripheral Nerve Sheath Tumor; Recurrent Synovial Sarcoma; Recurrent Undifferentiated Pleomorphic Sarcoma; Uterine Corpus Leiomyosarcoma

  6. Primary retroperitoneal synovial sarcoma in CT and MRI

    Directory of Open Access Journals (Sweden)

    Alhazzani Abdulmajeed

    2010-01-01

    Full Text Available Synovial sarcomas are most commonly localized in the extermities, especially the lower thigh and knee areas. Retroperitoneal synovial sarcoma is very rare. We decribe the radiological findings of an adult retroperitoneal synovial sarcoma.

  7. Primary retroperitoneal synovial sarcoma in CT and MRI

    OpenAIRE

    Alhazzani Abdulmajeed; El-sharkawy Mohamed; Hassan Hamdy

    2010-01-01

    Synovial sarcomas are most commonly localized in the extermities, especially the lower thigh and knee areas. Retroperitoneal synovial sarcoma is very rare. We decribe the radiological findings of an adult retroperitoneal synovial sarcoma.

  8. Dissecting the Role of Bone Marrow Stromal Cells on Bone Metastases

    OpenAIRE

    Denise Buenrostro; Serk In Park; Julie A. Sterling

    2014-01-01

    Tumor-induced bone disease is a dynamic process that involves interactions with many cell types. Once metastatic cancer cells reach the bone, they are in contact with many different cell types that are present in the cell-rich bone marrow. These cells include the immune cells, myeloid cells, fibroblasts, osteoblasts, osteoclasts, and mesenchymal stem cells. Each of these cell populations can influence the behavior or gene expression of both the tumor cells and the bone microenvironment. Addit...

  9. Patterns of care for patients with advanced soft tissue sarcoma: experience from Australian sarcoma services

    OpenAIRE

    Bae, Susie; Crowe, Philip; Gowda, Raghu; Joubert, Warren; Carey-Smith, Richard; Stalley, Paul; Desai, Jayesh

    2016-01-01

    Background There is a paucity of data on the current management of patients with advanced soft tissue sarcoma (STS) in the Australian health care setting. This study utilised the Australian sarcoma database to evaluate the patterns of care delivered to patients with advanced STS at Australian sarcoma services. Methods Prospectively collected data from six sarcoma centres in Australia were sourced to identify patients diagnosed with advanced STS between 1 January 2010 and 31 December 2012. Des...

  10. Postirradiation malignant fibrous histiocytoma expressing cytokeratin. Implications for the immunodiagnosis of sarcomas

    Energy Technology Data Exchange (ETDEWEB)

    Weiss, S.W.; Bratthauer, G.L.; Morris, P.A.

    1988-07-01

    A malignant fibrous histiocytoma of the sacrum complicating the course of radiation therapy for endometrial carcinoma is presented. Although the tumor fulfilled the clinical, radiologic, and histologic criteria for a postirradiation malignant fibrous histiocytoma of bone, it also expressed cytokeratin. That this immunoreactivity reflected keratin synthesis by the tumor and not an unusual pattern of cross-reactivity with another intermediate filament such as vimentin is strongly suggested by the reproducibility of the immunoreactivity utilizing both polyclonal and monoclonal antibodies and extinction of the immunoreactivity following absorption of the primary antiserum with keratin proteins. This is the first reported instance of keratin expression by a malignant fibrous histiocytoma; it indicates that sarcomas apart from synovial sarcoma and epithelioid sarcoma may sometimes express this protein.

  11. Postirradiation malignant fibrous histiocytoma expressing cytokeratin. Implications for the immunodiagnosis of sarcomas

    International Nuclear Information System (INIS)

    A malignant fibrous histiocytoma of the sacrum complicating the course of radiation therapy for endometrial carcinoma is presented. Although the tumor fulfilled the clinical, radiologic, and histologic criteria for a postirradiation malignant fibrous histiocytoma of bone, it also expressed cytokeratin. That this immunoreactivity reflected keratin synthesis by the tumor and not an unusual pattern of cross-reactivity with another intermediate filament such as vimentin is strongly suggested by the reproducibility of the immunoreactivity utilizing both polyclonal and monoclonal antibodies and extinction of the immunoreactivity following absorption of the primary antiserum with keratin proteins. This is the first reported instance of keratin expression by a malignant fibrous histiocytoma; it indicates that sarcomas apart from synovial sarcoma and epithelioid sarcoma may sometimes express this protein

  12. Immune characterization of the Lewis rats inoculated with K2 sarcoma cell line and newly derived R5-28 malignant cells

    Czech Academy of Sciences Publication Activity Database

    Morávková, Alena; Málek, Ondřej; Pokorná, Eva; Strnádel, Ján; Hradecký, Jan; Horák, Vratislav

    2005-01-01

    Roč. 51, - (2005), s. 159-165. ISSN 0015-5500 R&D Projects: GA ČR GA524/04/0102 Grant ostatní: Ústav živočišné fyziologie a genetiky AV ČR(CZ) IAPG/05/16 Institutional research plan: CEZ:AV0Z50450515 Keywords : rat sarcoma * malignancy * myeloid cells Subject RIV: EC - Immunology Impact factor: 0.719, year: 2005

  13. Neuronal Differentiation of Synovial Sarcoma and Its Therapeutic Application

    OpenAIRE

    Ishibe, Tatsuya; Nakayama, Tomitaka; Aoyama, Tomoki; Nakamura, Takashi; Toguchida, Junya

    2008-01-01

    Synovial sarcoma is a rare sarcoma of unknown histologic origin. We previously reported the gene expression profile of synovial sarcoma was closely related to that of malignant peripheral nerve sheath tumors, and the fibroblast growth factor (FGF) signal was one of the main growth signals in synovial sarcoma. Here we further demonstrate the neural origin of synovial sarcoma using primary tumors and cell lines. The expression of neural tissue-related genes was confirmed in synovial sarcoma tum...

  14. Can Fluorine-18-Fluorodeoxyglucose Positron Emission Tomography Be Used As a Useful Method to Evaluate the Treatment Response to Neoadjuvant Therapy Combined With Sorafenib and Anti-VEGF in Children Diagnosed With Metastatical Bone Sarcoma?

    Science.gov (United States)

    Tacyildiz, Nurdan; Tanyildiz, Hikmet Gulsah; Dincaslan, Handan Ugur; Yavuz, Gulsan; Unal, Emel; Ozkan, Elgin; Soydal, Cigdem; Kucuk, Ozlem; Yildiz, Yusuf

    2016-01-01

    Background The prognosis is still poor for patients with a metastatic bone tumor and new treatment approaches (anti-VEGF and tyrosine kinase inhibitors vs) are therefore needed. Objectives The aim of our study was to evaluate how the primary and metastatic lesions of our patients with a bone tumor were affected by these treatments and to determine the importance of the 18F-FDG PET method. Patients and Methods Twenty metastatic bone tumor cases were included. Sorafenib and anti-VEGF were added to the standard treatment in cases with widespread metastatic disease at diagnosis or after neoadjuvant chemotherapy showing less than 90% tumor necrosis in the surgical sample. Positron emission tomography (PET) imaging was performed at diagnosis, the preoperative period following neoadjuvant chemotherapy, during postoperative follow-up, and when treatment was discontinued. Results The primary treatment region median SUVmax level decreased from 7.35 to 2.5 in the living patients (n = 16) while there was no significant decrease in the patients who succumbed to the disease (P < 0.001). Comparison of the pre- and post-treatment metastasis region median SUVmax levels in patients with metastatic involvement showed a decrease from 2.1 to 0 in the surviving patients but only from 4.8 to 3.2 in the deceased patients (P < 0.01). Survival results indicated that 28.6% of the patients receiving classical treatment only died while all the patients receiving additional sorafenib and anti-VEGF survived. Conclusions 18F-PET may be a useful technique before and during the follow-up of neoadjuvant treatment in pediatric metastatic bone tumor patients. The addition of sorafenib and anti-VEGF to classical treatment has a favorable contribution to the response and therefore the survival duration. PMID:27307968

  15. Primary pleuropulmonary synovial sarcoma: a case report

    OpenAIRE

    Yuan, Lianfang; Guan, Zhiyu; Dai, Xuan; Jie XU

    2015-01-01

    Pleuropulmonary synovial sarcoma (PPSS) is an extremely rare malignant tumor, which is increasingly recognized as a subtype of sarcoma with a distinctive chromosomal translocation specific to synovial sarcoma. It is often presents like any thoracic tumor with symptoms such as chest pain or cough. Here we report a case of PPSS in a 49-year-old woman presenting with cough, shortness of breath and chest pain. And who were found upon histologic examination of the resection specimen to have cystic...

  16. Primary synovial sarcoma of the maxilla

    OpenAIRE

    Manikkath Aparna; Jayalakshmi Natarajan; Chakravarty Arumugam; Raghu Radhakrishnan

    2014-01-01

    An innocuously appearing gingival mass in the maxilla revealed extensive osteolysis on radiographic examination. A rare clinical presentation of synovial sarcoma, appropriate diagnostic strategies and suitable treatment protocol in a 21-year-old male is reported herewith. It is only the third case of primary monophasic synovial sarcoma of the maxilla to be reported to the best of our knowledge and the first to have occurred in a male patient. The importance of considering synovial sarcoma in ...

  17. Synovial Sarcoma of the Thyroid Gland

    OpenAIRE

    Ryu, Chang Hwan; Cho, Kyung-Ja; Choi, Seung-Ho

    2011-01-01

    Primary synovial sarcoma of the thyroid is an extremely rare condition which has only been reported twice in the literature. We herein report a case of highly aggressive and rapidly lethal primary synovial sarcoma of the thyroid. A 72-year-old woman presented with extensive local invasion, rapid progression, and early distant metastasis secondary to primary thyroid synovial sarcoma. The tumor exhibited an atypical histologic and immunohistochemical staining pattern. Detection of SYT/SSX fusio...

  18. Intraneural synovial sarcoma of the median nerve

    OpenAIRE

    Rahul Kasukurthi; Pruzansky, Mark E; Mackinnon, Susan E.; Lipira, Angelo B; Ray, Wilson Z.

    2010-01-01

    Synovial sarcomas are soft-tissue malignancies with a poor prognosis and propensity for distant metastases. Although originally believed to arise from the synovium, these tumors have been found to occur anywhere in the body. We report a rare case of synovial sarcoma arising from the median nerve. To our knowledge, this is the twelfth reported case of intraneural synovial sarcoma, and only the fourth arising from the median nerve. Because the diagnosis may not be apparent until after pathologi...

  19. A Primary Synovial Sarcoma of Lung

    OpenAIRE

    Roy, P. P.; Das, A.; A. Sarkar; A K Dwari; Datta, S.

    2012-01-01

    Primary pulmonary synovial sarcoma is an extremely rare tumor. The diagnosis is established only after sarcoma like primary lung malignancies and metastatic sarcoma have been excluded. It has four subtypes: monophasic fibrous, monophasic epithelial, biphasic, and poorly differentiated subtypes, We report a case of a 55-year-old man, who complained of left-sided chest pain and shortness of breath, had a large heterogeneous mass, occupying most of left hemithorax, associated with ipsilateral pl...

  20. Cytomorphology of epithelioid sarcoma: A diagnostic enigma

    Directory of Open Access Journals (Sweden)

    Yogesh K Yadav

    2012-01-01

    Full Text Available Epithelioid sarcoma is an uncommon soft tissue malignancy with a known propensity for recurrence as well as metastasis. We report epithelioid sarcoma in 50 year old male with a recurrent ulcerative nodule over the right ankle since five months, initially misdiagnosed as malignant fibrous histiocytoma. The article aims at assessing the key cytological features of epithelioid sarcoma and their reliability in the diagnosis of the lesion. Also described are its histopathological and immunohistochemical features.

  1. Intraneural synovial sarcoma of the median nerve

    Directory of Open Access Journals (Sweden)

    Rahul Kasukurthi

    2010-06-01

    Full Text Available Synovial sarcomas are soft-tissue malignancies with a poor prognosis and propensity for distant metastases. Although originally believed to arise from the synovium, these tumors have been found to occur anywhere in the body. We report a rare case of synovial sarcoma arising from the median nerve. To our knowledge, this is the twelfth reported case of intraneural synovial sarcoma, and only the fourth arising from the median nerve. Because the diagnosis may not be apparent until after pathological examination of the surgical speci­men, synovial sarcoma should be kept in mind when dealing with what may seem like a benign nerve tumor.

  2. Fine-needle aspiration cytology of postirradiation sarcomas, including angiosarcoma, with immunocytochemical confirmation

    Energy Technology Data Exchange (ETDEWEB)

    Silverman, J.F.; Lannin, D.L.; Larkin, E.W.; Feldman, P.; Frable, W.J. (East Carolina Univ. School of Medicine, Greenville, NC (USA))

    1989-01-01

    Postirradiation sarcomas are an unusual but well-recognized late effect of cancer therapy. In this article, a fine-needle aspiration (FNA) series of four cases is presented. There were three female patients and one male patient, with an age range of 28-55 yr (mean, 41). Two of the patients were irradiated for uterine cervical carcinoma while the other two received irradiation for malignant lymphoma. The time interval to the development of the postirradiation sarcoma ranged from 10 to greater than 20 yr. There were a postirradiation synovial sarcoma of the buttock region, malignant fibrous histiocytoma of the bone (femur), and rhabdomyosarcoma and angiosarcoma of the retroperitoneum. A spectrum of cytologic findings was encountered, reflecting the specific types of sarcomas. Immunocytochemical studies performed on the aspirated material from the angiosarcoma demonstrated the utility of immunoperoxidase stains for ULEX europaeus agglutinin-1 (UEA-1) and, to a lesser degree, factor VIII-related antigen antibody, confirming the vascular nature of this malignancy. The FNA findings from all four cases demonstrated cytologic features that allowed recognition of this unusual complication of irradiation treatment. This article confirms the utility of FNA cytology in following patients with previous malignancies and differentiating a postirradiation sarcoma from recurrent carcinoma.

  3. Fine-needle aspiration cytology of postirradiation sarcomas, including angiosarcoma, with immunocytochemical confirmation

    International Nuclear Information System (INIS)

    Postirradiation sarcomas are an unusual but well-recognized late effect of cancer therapy. In this article, a fine-needle aspiration (FNA) series of four cases is presented. There were three female patients and one male patient, with an age range of 28-55 yr (mean, 41). Two of the patients were irradiated for uterine cervical carcinoma while the other two received irradiation for malignant lymphoma. The time interval to the development of the postirradiation sarcoma ranged from 10 to greater than 20 yr. There were a postirradiation synovial sarcoma of the buttock region, malignant fibrous histiocytoma of the bone (femur), and rhabdomyosarcoma and angiosarcoma of the retroperitoneum. A spectrum of cytologic findings was encountered, reflecting the specific types of sarcomas. Immunocytochemical studies performed on the aspirated material from the angiosarcoma demonstrated the utility of immunoperoxidase stains for ULEX europaeus agglutinin-1 (UEA-1) and, to a lesser degree, factor VIII-related antigen antibody, confirming the vascular nature of this malignancy. The FNA findings from all four cases demonstrated cytologic features that allowed recognition of this unusual complication of irradiation treatment. This article confirms the utility of FNA cytology in following patients with previous malignancies and differentiating a postirradiation sarcoma from recurrent carcinoma

  4. [Sarcomas of the small intestine].

    Science.gov (United States)

    Beyrouti, M L; Abid, M; Beyrouti, R; Ben Amar, M; Gargouri, F; Frikha, F; Affes, N; Boujelbene, S; Ghorbel, A

    2005-03-12

    Sarcomas of the small intestine are rare, clearly differentiated, malignant, mesenchymatous tumours that can be of smooth muscle, Schwann cell or fibroblastic origin. From a clinical point of view, the pain and abdominal mass are the 2 types of symptoms that frequently reveal the disease. In rare cases, sarcomas of the small intestine are manifested by an acute complication. No imaging method can clearly confirm the diagnosis. Before immunohistochemistry, differential diagnosis was made on undifferentiated mesenchymatous "stromal" tumours, which are also rare. Exeresis must be complete and without perforation of the tumour because of the risk of locoregional relapse. The benefits provided by chemotherapy and radiotherapy are limited because of the low mitotic activity of the tumour cells and its weak vascularisation. Long-term survival is limited by poor prognosis criteria: high grade malignancy, size greater than 5 cm, tumour extension, perforation of the tumour, quality of surgical resection and histological type. PMID:15859576

  5. Epithelioid sarcoma: A diagnostic challenge

    Directory of Open Access Journals (Sweden)

    Pai Kanthilatha

    2006-01-01

    Full Text Available Epithelioid sarcoma is an uncommon slow-growing soft tissue malignancy, associated with a high incidence of local recurrence and metastasis. We report a 26-year-old male with epithelioid sarcoma on the right palm with a long history of over seven years, which was initially misdiagnosed as cutaneous tuberculosis and epithelioid hemangioendothelioma, as a result of which the treatment was delayed. No metastasis was found in our patient. The patient was referred to the oncology centre where he underwent wide excision of the lesion followed by radiotherapy. The review of the literature including clinical and histological differential diagnosis is presented as it mimics inflammatory, benign tumors as well as other malignant conditions.

  6. Reticular schwannoma mimicking myxoid sarcoma

    OpenAIRE

    Chaurasia, Jai Kumar; Afroz, Nishat; Sahoo, Biswajit; Naim, Mohammed

    2014-01-01

    Reticular/microcystic schwannoma is a recently described rare distinctive variant of schwannoma with a predilection for gastrointestinal tract. Its occurrence in soft tissue is extremely rare. We report a case of reticular/microcystic variant of schwannoma in a 55-year-old Indian woman who presented with a painless slow-growing swelling in the right forearm for the past 6 months. MRI findings suggested myxoid sarcoma. However, histological and immunohistochemical findings in the excised speci...

  7. MR findings of synovial sarcoma

    International Nuclear Information System (INIS)

    MR images of seven patients with histologically documented synovial sarcoma were reviewed to find the suggestive or pathognomonic findings of the disease. MR appearance of the tumor was inhomogeneous, multisepatated mass with various degree of internal septation and infiltrative tumor margin. The signal intensity of the mass was slightly hyperintense relative to muscle on T1-weighted image and hyperintense relative to subcutaneous fatty tissue on T2-weighted image

  8. MR findings of synovial sarcoma

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Jin Joo; Byun, Hong Sik; Kim, Kie Hwan; Yoon, Il Yoon; Chin, Soo Yil [Korea Cancer Center Hospital, Seoul (Korea, Republic of)

    1993-09-15

    MR images of seven patients with histologically documented synovial sarcoma were reviewed to find the suggestive or pathognomonic findings of the disease. MR appearance of the tumor was inhomogeneous, multisepatated mass with various degree of internal septation and infiltrative tumor margin. The signal intensity of the mass was slightly hyperintense relative to muscle on T1-weighted image and hyperintense relative to subcutaneous fatty tissue on T2-weighted image.

  9. Synovial sarcoma of the foot

    International Nuclear Information System (INIS)

    The case of a 29 year-old female patient who had experienced pain in the right midfoot for 5 years which was diagnosed as a degenerative or rheumatic change and treated by physiotherapy and medication. By means of magnetic resonance imaging we identified a soft-tissue tumor of the midfoot. Histology provided the findings of a monophasic fibrous synovial sarcoma. The case history is reported together with a presentation of the disease and its radiological diagnosis. (orig.)

  10. Epithelioid sarcoma of the orbit

    OpenAIRE

    Alkatan, Hind M.; Chaudhry, Imtiaz; Al-Qahtani, Abdullah

    2011-01-01

    Epithelioid sarcoma is an aggressive and rare malignancy first recognized by Enzinger in 1970. It is known most commonly to affect the distal upper extremities in young adults. The classical “distal” form has a male predominance and can also involve other less frequent sites including lower extremities, proximal upper extremities, and the trunk. The “proximal” variant of this tumor is deep seated, tends to occur in older patients and predominantly develops in the pelvis, perineum, and genital...

  11. Synovial sarcoma of the foot.

    Science.gov (United States)

    Bekarev, Mikhail; Elsinger, Elisabeth C; Villanueva-Siles, Esperanza; Borzykowski, Ross M; Geller, David S

    2013-01-01

    We report the case of a 75-year-old male who underwent lung lobectomy for presumed lung cancer. Thereafter, he presented with a painful mass between the third and fourth metatarsal heads in the foot that was assumed to be Morton's neuroma. After extensive oncologic evaluation, the foot mass was diagnosed as a synovial sarcoma. In retrospect, his lung lesion was understood to be metastatic disease. PMID:23632071

  12. Fibromyxoid sarcoma of the pancreas

    Directory of Open Access Journals (Sweden)

    Čolović Radoje

    2008-01-01

    Full Text Available Introduction Fibromyxoid sarcoma is a rare mesenchymal neoplasm, usually appearing in the soft tissue of the extremities, less frequently in the groin, trunk, neck, and upper extremities. Within the abdomen, the tumour is usually localised within the retroperitoneum. Case OutlineWe present a 56-year-old woman in whom, during the routinely performed investigation for atacks of choking with lots of bronchial secretion, and arterial hypertension, an ultrasonographer found a tumour within the head of the pancreas 6×6 cm in diameter. At operation, a dark pink, lobulated soft tumour, surrounded by a tiny capsule, clearly different from the completely normal pancreatic tissue of the posterior side of the head of the pancreas, was easily and ideally excised.The postoperative recovery was stormy. She developed postoperative pancreatitis, temporary biliary and duodenal fistula, which all settled by conservative treatment. The histology of the 80 g weighing tumour showed a circumscribed fibromyxoid sarcoma of low malignancy. Immunohistochemistry showed diffuse vimentin and CD34 strong positivity, as well as focal anti-SMA and anti-EMA immunopositivity. Six months after surgery, she died with signs of cerebrovascular insult, asthmatic status, and recurrent suppurative abdominal fistula, probably related to the previous pancreatitis. Ultrasonography showed a possible liver secondary. The exact cause of death was not confirmed as the autopsy was refused by the family. Conclusion Primary sarcomas of the pancreas are very rare, but should be considered in differential diagnosis of pancreatic neoplasms. To the best of our knowledge, there has been no previously described fibromyxoid sarcoma of the pancreas. .

  13. TREM2-transduced myeloid precursors mediate nervous tissue debris clearance and facilitate recovery in an animal model of multiple sclerosis.

    Directory of Open Access Journals (Sweden)

    Kazuya Takahashi

    2007-04-01

    Full Text Available BACKGROUND: In multiple sclerosis, inflammation can successfully be prevented, while promoting repair is still a major challenge. Microglial cells, the resident phagocytes of the central nervous system (CNS, are hematopoietic-derived myeloid cells and express the triggering receptor expressed on myeloid cells 2 (TREM2, an innate immune receptor. Myeloid cells are an accessible source for ex vivo gene therapy. We investigated whether myeloid precursor cells genetically modified to express TREM2 affect the disease course of experimental autoimmune encephalomyelitis (EAE, an animal model of multiple sclerosis. METHODS AND FINDINGS: EAE was induced in mice by immunization with a myelin autoantigen. Intravenous application of TREM2-transduced bone marrow-derived myeloid precursor cells at the EAE peak led to an amelioration of clinical symptoms, reduction in axonal damage, and prevention of further demyelination. TREM2-transduced myeloid cells applied intravenously migrated into the inflammatory spinal cord lesions of EAE-diseased mice, showed increased lysosomal and phagocytic activity, cleared degenerated myelin, and created an anti-inflammatory cytokine milieu within the CNS. CONCLUSIONS: Intravenously applied bone marrow-derived and TREM2-tranduced myeloid precursor cells limit tissue destruction and facilitate repair within the murine CNS by clearance of cellular debris during EAE. TREM2 is a new attractive target for promotion of repair and resolution of inflammation in multiple sclerosis and other neuroinflammatory diseases.

  14. Langerhan’s cell sarcoma: two case reports

    Directory of Open Access Journals (Sweden)

    Tasneem A. Kaleem

    2016-04-01

    Full Text Available Langerhan’s cell sarcoma (LCS is a rare neoplasm with a poor prognosis. To our knowledge, only sixty-six cases have been published. We discuss two patients who presented very differently with LCS, as well as a recently published review of all sixty-six cases. Our first case had a complicated history of metastatic, high-grade myxofibrosarcomas and presented with a single skin lesion of LCS which was treated with resection to a positive margin and adjuvant radiotherapy. The LCS recurred locoregionally and was again resected. The patient is alive two years after initial diagnosis. The second case presented with bone marrow and splenic involvement, leukocytosis, and thrombocytopenia. This patient had an excellent response to etoposide, prednisone, oncovorin, cyclophosphamide, and adriamycin, with normalization of the complete blood count, negative bone marrow biopsy at follow up, and splenectomy without viable neoplasm. This patient is alive without signs of disease at 16 months after initial diagnosis.

  15. Primary Ewing sarcoma of the coronoid process of mandible.

    Science.gov (United States)

    Sabir, Husain; Kumbhare, S; Pande, S; Sachdeva, S; Gajbhiye, N

    2016-01-01

    Ewing sarcoma (ES) is a rare, primary malignancy of the bone that occurs mainly in childhood and early adolescence. ES usually occurs in long bones of the axial skeleton. Although uncommon in the jaws, ES at this site is most likely to occur in the posterior mandible. The outcome for patients with localised disease has improved over the decades, due to better combination chemotherapies and better methods of local control. We present the clinicopathologic features and management of a case of ES that developed in the left coronoid process of the mandible of a 31-year-old male. Chemotherapy and, later, a segmental mandibulectomy were used to achieve local control. A fibula-free flap repair was performed with good aesthetic results. This case elucidates the importance of the interdisciplinary approach required for the evaluation and treatment of this aggressive neoplasm. PMID:27514181

  16. General Information about Adult Acute Myeloid Leukemia

    Science.gov (United States)

    ... Treatment Childhood AML Treatment Research Adult Acute Myeloid Leukemia Treatment (PDQ®)–Patient Version General Information About Adult Acute Myeloid Leukemia Go to Health Professional Version Key Points Adult ...

  17. Treatment Option Overview (Adult Acute Myeloid Leukemia)

    Science.gov (United States)

    ... Treatment Childhood AML Treatment Research Adult Acute Myeloid Leukemia Treatment (PDQ®)–Patient Version General Information About Adult Acute Myeloid Leukemia Go to Health Professional Version Key Points Adult ...

  18. Stages of Adult Acute Myeloid Leukemia

    Science.gov (United States)

    ... Treatment Childhood AML Treatment Research Adult Acute Myeloid Leukemia Treatment (PDQ®)–Patient Version General Information About Adult Acute Myeloid Leukemia Go to Health Professional Version Key Points Adult ...

  19. Treatment Options for Adult Acute Myeloid Leukemia

    Science.gov (United States)

    ... Treatment Childhood AML Treatment Research Adult Acute Myeloid Leukemia Treatment (PDQ®)–Patient Version General Information About Adult Acute Myeloid Leukemia Go to Health Professional Version Key Points Adult ...

  20. Cytotoxic capacity of IL-15-stimulated cytokine-induced killer cells against human acute myeloid leukemia and rhabdomyosarcoma in humanized preclinical mouse models

    OpenAIRE

    Eva eRettinger; Vida eMeyer; Hermann eKreyenberg; Andreas eVolk; Selim eKuci; Andre eWillasch; Ewa eKoscielniak; Simone eFulda; Winfried eWels; Halvard eBoenig; Thomas eKlingebiel; Peter eBader

    2012-01-01

    Allogeneic stem cell transplantation (allo-SCT) has become an important treatment modality for patients with high-risk acute myeloid leukemia (AML) and is also under investigation for soft tissue sarcomas. The therapeutic success is still limited by minimal residual disease (MRD) status ultimately leading to patients’ relapse. Adoptive donor lymphocyte infusions based on MRD status using IL-15-expanded cytokine-induced killer (CIK) cells may prevent relapse without causing graft-versus-host-d...

  1. Bone cysts: unicameral and aneurysmal bone cyst.

    Science.gov (United States)

    Mascard, E; Gomez-Brouchet, A; Lambot, K

    2015-02-01

    Simple and aneurysmal bone cysts are benign lytic bone lesions, usually encountered in children and adolescents. Simple bone cyst is a cystic, fluid-filled lesion, which may be unicameral (UBC) or partially separated. UBC can involve all bones, but usually the long bone metaphysis and otherwise primarily the proximal humerus and proximal femur. The classic aneurysmal bone cyst (ABC) is an expansive and hemorrhagic tumor, usually showing characteristic translocation. About 30% of ABCs are secondary, without translocation; they occur in reaction to another, usually benign, bone lesion. ABCs are metaphyseal, excentric, bulging, fluid-filled and multicameral, and may develop in all bones of the skeleton. On MRI, the fluid level is evocative. It is mandatory to distinguish ABC from UBC, as prognosis and treatment are different. UBCs resolve spontaneously between adolescence and adulthood; the main concern is the risk of pathologic fracture. Treatment in non-threatening forms consists in intracystic injection of methylprednisolone. When there is a risk of fracture, especially of the femoral neck, surgery with curettage, filling with bone substitute or graft and osteosynthesis may be required. ABCs are potentially more aggressive, with a risk of bone destruction. Diagnosis must systematically be confirmed by biopsy, identifying soft-tissue parts, as telangiectatic sarcoma can mimic ABC. Intra-lesional sclerotherapy with alcohol is an effective treatment. In spinal ABC and in aggressive lesions with a risk of fracture, surgical treatment should be preferred, possibly after preoperative embolization. The risk of malignant transformation is very low, except in case of radiation therapy. PMID:25579825

  2. Systemic therapy for selected skull base sarcomas: Chondrosarcoma, chordoma, giant cell tumour and solitary fibrous tumour/hemangiopericytoma.

    Science.gov (United States)

    Colia, Vittoria; Provenzano, Salvatore; Hindi, Nadia; Casali, Paolo G; Stacchiotti, Silvia

    2016-01-01

    This review highlights the data currently available on the activity of systemic therapy in chondrosarcoma, chordoma, giant cell tumour of the bone (GCTB) and solitary fibrous tumour, i.e., four rare sarcomas amongst mesenchymal malignancy arising from the skull base. PMID:27330421

  3. CD34 expression on bone marrow blasts is a novel predictor of poor prognosis independent of FlT3-ITD in acute myeloid leukemia with the NPM1-mutation.

    Science.gov (United States)

    Zhu, Hong-Hu; Liu, Yan-Rong; Jiang, Hao; Lu, Jin; Qin, Ya-Zhen; Jiang, Qian; Bao, Li; Ruan, Guo-Rui; Jiang, Bin; Huang, Xiaojun

    2013-06-01

    To explore the prognostic value of CD34 expression in NPM1-mutated acute myeloid leukemia (NPM1m+AML), seventy-one NPM1m+AML patients were retrospectively analyzed. The patients with >7% CD34 expression (according to the ROC analysis) had a lower complete remission (CR) rate after 1 course of induction, disease-free survival (DFS), and overall survival (OS) compared to those with ≤7% CD34 expression (p=0.0038; p=0.001; p<0.0001). A multivariate analysis revealed that CD34 expression is a prognostic factor that is independent of FlT3-ITD for relapse, DFS and OS. We established a novel prognostic model based on the CD34 and FLT3 status at diagnosis, which could facilitate the segregation of patients into three prognostically different subgroups. We demonstrate that CD34 expression on blasts is a novel, poor predictor independent of FlT3-ITD in NPM1-mutated patients and established a new prognostic model based on the CD34 and FLT3 status at diagnosis, which may facilitate immediate clinical utility for prognostic stratification and risk-adapted therapeutic choices. PMID:23473998

  4. Nasopharyngeal Synovial Sarcoma - An Oncological Surprise

    OpenAIRE

    Ram Abhinav Kannan; Kirthi Koushik; Nalini Kilara; Aparna Gangoli

    2014-01-01

    Synovial sarcoma is a rare and aggressive high-grade neoplasm with poor prognosis. It is usually encountered in the lower extremities of young adults; only a few cases have been reported in the head and neck region. We report the fourth case of synovial sarcoma of the nasopharynx, treated with neoadjuvant chemotherapy, complete surgical excision and adjuvant radiation with extensive review of literature.

  5. Delays in the management of retroperitoneal sarcomas

    DEFF Research Database (Denmark)

    Seinen, Jojanneke; Almquist, Martin; Styring, Emelie;

    2010-01-01

    Retroperitoneal sarcomas are rare and treatment should optimally be centralized. Despite successful centralization with 90% of the patients referred prior to surgery, delays occur, which led us to assess lead times in a population-based series. Method. Patients diagnosed with retroperitoneal sarc...... indicate that development of coordinated diagnostic packages could shorten delays at the sarcoma centre....

  6. Functional imaging techniques for evaluation of sarcomas

    OpenAIRE

    Hicks, Rodney J.

    2005-01-01

    Sarcomas are often characterised by significant histopathologic heterogeneity, both between and within tumours. This heterogeneity reflects physiologic, biochemical and genetic processes that are amenable to characterisation by functional imaging. Although anatomically based imaging modalities such as plain radiography, X-ray computed tomography (CT) and magnetic resonance imaging (MRI) remain the primary diagnostic modalities for staging sarcomas, nuclear medicine approaches including gamma ...

  7. Primary stromal sarcoma of the breast

    Directory of Open Access Journals (Sweden)

    Radovanović Aleksandra

    2012-01-01

    Full Text Available Primary sarcomas of the breast are rare and there are only a few hundred cases reported in the literature; hence, their significance is primarily educational. We report a case of a 57-year old patient who developed breast sarcoma without previous history on any breast diseases.

  8. [Cytogenetic monitoring acute myeloid leucosis in children].

    Science.gov (United States)

    Fleĭshman, E V; Sokova, O I; Popa, A V; Konstantinova, L N; Metel'kova, N F; Serebriakova, I N; Peregudov, D A

    2009-01-01

    Preliminary results of cytogenetic monitoring acute myeloid leukosis (AML) in children are presented. Repeated chromosomal analyses were accomplished in 23 patients that presented with cell clones showing various karyotype abnormalities prior to the onset of therapy. All the patients were treated following identical protocols. Complete hematological remission was achieved in 20 cases. The majority of patients did not have cells with chromosomal abnormalities changes after a 2-4 month follow up. Anomalous metaphases persisted in 6 patients although their occurrence decreased. Five of them poorly responded to therapy whereas simultaneous achievement of morphological and cytogenetic remission ensured more beneficial outcome of the treatment. Results of the study agree with recent reports of delayed reversion to normal karyotype under effect of AML therapy that as a rule predicts an unfavourable prognosis Repeated analysis during stable hematological remission did not reveal cells with karyotype abnormalities in bone marrow with the exception of a single patient who had marrow cells with chromosomal translocation (16:16) up to the 8th month of complete hematological remission. This patient remains under observation (duration of remission is now 15 months). It was shown that the relative amount of cells with abnormal karyotype in bone marrow frequently exceeds that of blast cells (usually before the onset of therapy and sometimes in the beginning of morphological remission). During stable remission, such an excess is an antecedent of relapse. It is concluded that cytogenetic analysis for monitoring AML extends the possibility of detecting leukemia cells. PMID:19827640

  9. Bone-metastasizing primary renal tumors in children

    International Nuclear Information System (INIS)

    Seven cases of childhood renal tumor with extensive bone involvement are reported. These neoplasms had been classified originally as wills tumors with atypical clinical and pathologic features. Subsequent to a retrospective histologic analysis, the lesions were reclassified as follows: three cases as bone-metastasizing renal tumors of childhood, one as rhabdomyosarcoma, two as indifferentiated Sarcomas and one case as indifferentiated malignant neoplasm. (Author)

  10. 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.

  11. Angiography of histopathologic variants of synovial sarcoma

    International Nuclear Information System (INIS)

    Synovial sarcomas are rare soft tissue tumors which histopathologically can be divided into monophasic, biphasic and mixed variants. As part of a protocol for intra-arterial chemotherapy 12 patients with biopsy proven synovial sarcoma underwent angiography. The angiograms on these patients were reviewed to determine whether synovial sarcomas and their variants demonstrated a characteristic angiographic appearance. Synovial sarcomas appeared angiographically as soft tissue masses which showed a fine network of tumor vessels with an inhomogeneous capillary blush. Their degree of vascularity varied according to their histopathology. Monophasic synovial sarcomas demonstrated in general a higher degree of neovascularity than the biphasic form. This finding was also suggested by histopathologic analysis of the vessels in the tumor. Although angiography did not show a distinctive vascular pattern it may be useful to evaluate tumor size and vascularity. (orig.)

  12. Penile epithelioid sarcoma: MR imaging findings

    International Nuclear Information System (INIS)

    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.)

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

    International Nuclear Information System (INIS)

    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.

  14. 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.

  15. Analysis of eosinophils and myeloid progenitor responses to modified forms of MPIF-2.

    Science.gov (United States)

    Grzegorzewski, K J; Yao, X T; Kreider, B; Olsen, H S; Morris, T S; Zhang, L; Sanyal, I; Nardelli, B; Zukauskas, D; Brewer, L; Bong, G W; Kim, Y; Garotta, G; Salcedo, T W

    2001-02-21

    Myeloid progenitor inhibitory factor (MPIF)-2 is a beta-chemokine with select and potent activities on eosinophils and myeloid progenitors. In the beta-chemokine family, biological activity is modulated by differential processing of the amino-terminus. Here, for MPIF-2, we describe the biological activities of NH(2)-terminal deletion mutants and compare regions necessary for eosinophil and myeloid progenitor activities. Five MPIF-2 proteins with deletions at the amino-terminus were produced in Escherichia coli and assayed for calcium mobilization, chemotaxis and receptor binding activities on eosinophils, and for their ability to inhibit colony formation of human myeloid bone marrow progenitors. For eosinophils, deletion of the first two amino acids did not markedly alter activity, while subsequent truncations result in a complete loss of activity. One of the MPIF-2 mutants, MPIF-2 (P30-R99) was converted from an agonist to an antagonist of eotaxin, MPIF-2 and MCP-4 functional responses in eosinophil calcium flux and chemotaxis assays. Surprisingly, while displaying a complete loss of agonist activity toward eosinophils, MPIF-2 (P30-R99) retains ability to inhibit human bone marrow myeloid progenitor cell colony formation. In addition, processing at the amino terminus of MPIF-2 in vivo, may result in a chemokine with altered biological activities. PMID:11237428

  16. Expression of fibroblast growth factor 23 by canine soft tissue sarcomas.

    Science.gov (United States)

    Hardcastle, M R; Dittmer, K E

    2016-09-01

    Tumour-induced osteomalacia (TIO) is a rare paraneoplastic syndrome of humans. Some mesenchymal tumours (often resembling haemangiopericytomas) express molecules that normally regulate phosphorus metabolism; most frequently, fibroblast growth factor 23. Patients develop renal phosphate wasting and inappropriately low serum concentrations of 1, 25 (OH)2 vitamin D3 , leading to osteomalacia. Surgical removal of the tumour is curative. The authors examined expression of canine fibroblast growth factor 23 in 49 soft tissue sarcomas, and control tissues from normal adult dogs. RNA extracted from bone or formalin-fixed, paraffin-embedded tissues was analysed by end point and quantitative reverse transcriptase-polymerase chain reaction. Fibroblast growth factor 23 expression was detected in bone, lung, kidney, lymph node and thymus. Fifteen of 49 sarcomas (31%) expressed fibroblast growth factor 23, three of these had high relative expression and some features resembling phosphatonin-expressing mesenchymal tumours of humans. Further work is required to determine whether TIO may occur in dogs. PMID:24923416

  17. Wee1 inhibition by MK-1775 leads to tumor inhibition and enhances efficacy of gemcitabine in human sarcomas.

    Science.gov (United States)

    Kreahling, Jenny M; Foroutan, Parastou; Reed, Damon; Martinez, Gary; Razabdouski, Tiffany; Bui, Marilyn M; Raghavan, Meera; Letson, Douglas; Gillies, Robert J; Altiok, Soner

    2013-01-01

    Sarcomas are rare and heterogeneous mesenchymal tumors affecting both pediatric and adult populations with more than 70 recognized histologies. Doxorubicin and ifosfamide have been the main course of therapy for treatment of sarcomas; however, the response rate to these therapies is about 10-20% in metastatic setting. Toxicity with the drug combination is high, response rates remain low, and improvement in overall survival, especially in the metastatic disease, remains negligible and new agents are needed. Wee1 is a critical component of the G2/M cell cycle checkpoint control and mediates cell cycle arrest by regulating the phosphorylation of CDC2. Inhibition of Wee1 by MK1775 has been reported to enhance the cytotoxic effect of DNA damaging agents in different types of carcinomas. In this study we investigated the therapeutic efficacy of MK1775 in various sarcoma cell lines, patient-derived tumor explants ex vivo and in vivo both alone and in combination with gemcitabine, which is frequently used in the treatment of sarcomas. Our data demonstrate that MK1775 treatment as a single agent at clinically relevant concentrations leads to unscheduled entry into mitosis and initiation of apoptotic cell death in all sarcomas tested. Additionally, MK1775 significantly enhances the cytotoxic effect of gemcitabine in sarcoma cells lines with different p53 mutational status. In patient-derived bone and soft tissue sarcoma samples we showed that MK1775 alone and in combination with gemcitabine causes significant apoptotic cell death. Magnetic resonance imaging (MRI) and histopathologic studies showed that MK1775 induces significant cell death and terminal differentiation in a patient-derived xenograft mouse model of osteosarcoma in vivo. Our results together with the high safety profile of MK1775 strongly suggest that this drug can be used as a potential therapeutic agent in the treatment of both adult as well as pediatric sarcoma patients. PMID:23520471

  18. Microarrays in Molecular Profiling of Ewing Sarcoma Family of Tumors and CDKN2A Aberrations

    OpenAIRE

    Savola, Suvi

    2009-01-01

    Background: The Ewing sarcoma family of tumors (ESFT) are rare but highly malignant neoplasms that occur mainly in bone or but also in soft tissue. ESFT affects patients typically in their second decade of life, whereby children and adolescents bear the heaviest incidence burden. Despite recent advances in the clinical management of ESFT patients, their prognosis and survival are still disappointingly poor, especially in cases with metastasis. No targeted therapy for ESFT patients is currentl...

  19. Radiological diagnostic in cat stratch disease and bone lesions - a case report

    International Nuclear Information System (INIS)

    Cat scratch disease is not a common disease in immunocompetent patients. Its association with bone lesions is rare. A patient with bone complain and radiologic alterations of bone lesion must be investigated for this disease. A simple story can make the differential diagnosis with more complex disease like Ewing sarcoma or eosinophilic granuloma. (author)

  20. Primary bone tumours of the hand

    International Nuclear Information System (INIS)

    Twenty-one primary bone tumours of the hand in children from 8 paediatric hospitals are reported. Osteochondromas and enchondromas were not included. Our material consisted of 16 patients with common tumours (3 Ewing's sarcoma, 5 aneurysmal bone cyst, 6 osteoid osteoma and 2 epithelioma) and 5 patients with uncommon tumours (osteoma, simple bone cyst, haemangiopericytoma, capillary angiomatous tumour and benign ossifying fibroma or osteoblastoma). The X-ray diagnosis of the common tumours should have high concordance with histology, whereas that of uncommon tumours in much more difficult and uncertain. The characteristic features of Ewing's sarcoma are stressed as all our children with this tumour had a delayed diagnosis and a fatal outcome. Differential diagnosis with other short tubular bone lesions of the hand - specifically osteomyelitis - is discussed and the posibilities of microscopic diagnosis are stressed. (orig.)

  1. Establishment of a Ewing's sarcoma mouse model: JAK/STAT signalling in Ewing's sarcoma

    International Nuclear Information System (INIS)

    The Ewing's sarcoma family of tumours (ESFT) comprises paediatric cancers of bone and soft tissue which presumably originate from mesenchymal progenitor cells(MPC). One hallmark of ESFT is the presence of a chromosomal translocation. In 90% of the cases chromosome 11 fuses with chromosome 22. This translocation generates the EWS/FLI-1 fusion which acts as an aberrant transcription factor deregulating many genes involved in tumour development. Surgery and/or radiotherapy combined with chemotherapy are the usual forms of treatment for ESFT. But since there is only little progress in the field of chemotherapy the need for an animal model for pre-clinical drug testing is evident. Thus, the main focus of this thesis was to establish a mouse model that develops sarcomas resembling the phenotype of ESFT. We used a conditional EWS/FLI-1 mouse model, which upon Cre activity (controlled by a tissue specific promotor) expressed EWS/FLI-1 in the targeted cells. Since ESFT arises in bone and surrounding soft tissue we decided to direct expression of EWS/FLI-1 to the mesenchymal lineage by using different Cre lines. Only when using the Prx1Cre, double transgenic mice tolerated EWS/FLI-1 expression. We observed developmental abnormalities with severe skeletal deformations. Bone formation was impaired due to the absence of mature chondrocytes and osteoblasts and hence a lack of calcified bone. The lack of mature bone cells in EWS/FLI-1 expressing Prx1Cre mice supports in vitro data showing that EWS/FLI-1 impedes differentiation of murine mesenchymal progenitor cells. Currently, the project is extended to analysis of an inducible Prx1Cre system which circumvents the early lethality of Prx1Cre EF mice. This should provide the basis for tumour formation in these mice and hence the development of an appropriate mouse model for pre-clinical research. In the second project of my PhD thesis, the role of the Janus Kinase/Signal Transducer and Activator of Transcription (JAK

  2. Transformation of myelodysplastic syndromes into acute myeloid leukemias

    Institute of Scientific and Technical Information of China (English)

    施均; 邵宗鸿; 刘鸿; 白洁; 曹燕然; 何广胜; 凃梅峰; 王秀丽; 郝玉书; 杨天楹; 杨崇礼

    2004-01-01

    Background Myelodysplastic syndromes (MDSs), also called preleukemias, are a group of myeloid hematopoietic malignant disorders. We studied the transformation of MDS into acute myeloid leukemia (AML).Methods Leukemic transformation in 151 patients with MDS was dynamically followed up. The clinical manifestation, peripheral blood and bone marrow condition, karyotypes, immunophenotypes, response to treatment, and prognosis of AML evolution from MDS (MDS-AML) were also observed.Results During the course of this study, over the past eight years and seven months, 21 (13.91%) of 151 MDS patients progressed to overt leukemia, with a median interval of 5 (1-23) months. There were no significant differences between rates of leukemic transformation in comparison with the refractory anemia (RA), RA with excess of blasts (RAEB), and RAEB in transformation (RAEB-t) patient groups. Transformation occurred either gradually or rapidly. There were five parameters positively correlated to leukemic transformation: under 40 years of age, pancytopenia of 3 lineages, more than 15% blasts in the bone marrow, at least two abnormal karyotypes, and treatment with combined chemotherapy. All of the 21 patients with leukemia suffered from MDS-AML, and most of them were M2, M4, or M5. Two (9.52%) MDS-AML patients developed extramedullary infiltration. Leukopenia was found in 47.62% of these patients. Two thirds of these patients, whose bone marrows were generally hypercellular, suffered from neutropenia. After developing AML, 8 (47.06%) patients developed abnormal karyotypes. High expression of immature myeloid antigens, including CD33 [(49.83±24.50)%], CD13 [(36.38±33.84)%], monocytic antigen CD14 [(38.50±24.60)%], and stem cell marker CD34 [(34.67±30.59)%], were found on bone marrow mononuclear cells from MDS-AML patients after leukemic transformation. In some cases, lymphoid antigens, such as CD5, CD7, CD9, and CD19, coexisted with myeloid antigens. A low complete remission rate (31

  3. LEUKEMIC PLEURAL EFF USION AS INITIAL MAN IFESTATION OF ACUTE MYELOID LEUKEMIA: A RARE CASE REPORT

    Directory of Open Access Journals (Sweden)

    Rajeev Kumar

    2015-06-01

    Full Text Available Leukemic effusion is an uncommon presentation of Acute Myeloid leukemia with only isolated reports in literature. We report a case of 45 years old female who presented with unilateral pleural effusion and was diagnosed with haematological malignancy on pleural fluid cytology which revealed presence of myeloblasts. Subsequent, peripheral blood smear and bone marrow examination confirmed the diagnosis of Acute Myeloid Leukemia (AML M1. This case report highlights the uncommon presentation of AML as well as utility of meticulous examination of effusion fluids for diagnosing unsuspected malignancies.

  4. Myeloid and lymphoid contribution to non-haematopoietic lineages through irradiation-induced heterotypic cell fusion

    DEFF Research Database (Denmark)

    Nygren, J.M.; Liuba, K.; Breitbach, M.; Stott, S.; Thorén, Lina Anna Maria; Roell, W.; Geisen, C.; Sasse, P.; Kirik, D.; Bjorklund, A.; Nerlov, C.; Fleischmann, B.K.; Jovinge, S.; Jacobsen, S.E.

    2008-01-01

    Recent studies have suggested that regeneration of non-haematopoietic cell lineages can occur through heterotypic cell fusion with haematopoietic cells of the myeloid lineage. Here we show that lymphocytes also form heterotypic-fusion hybrids with cardiomyocytes, skeletal muscle, hepatocytes and...... is induced by organ-specific injuries or whole-body irradiation, which has been used in previous studies to condition recipients of bone marrow transplants. Our findings demonstrate that blood cells of the lymphoid and myeloid lineages contribute to various non-haematopoietic tissues by forming rare...

  5. The role of myeloid cells in the promotion of tumour angiogenesis.

    Science.gov (United States)

    Murdoch, Craig; Muthana, Munitta; Coffelt, Seth B; Lewis, Claire E

    2008-08-01

    The use of various transgenic mouse models and analysis of human tumour biopsies has shown that bone marrow-derived myeloid cells, such as macrophages, neutrophils, eosinophils, mast cells and dendritic cells, have an important role in regulating the formation and maintenance of blood vessels in tumours. In this Review the evidence for each of these cell types driving tumour angiogenesis is outlined, along with the mechanisms regulating their recruitment and activation by the tumour microenvironment. We also discuss the therapeutic implications of recent findings that specific myeloid cell populations modulate the responses of tumours to agents such as chemotherapy and some anti-angiogenic therapies. PMID:18633355

  6. Synovial sarcoma: MR evaluation in 23 patients

    International Nuclear Information System (INIS)

    The synovial sarcoma is one of the most common soft tissue sarcomas. MR is the technique of choice to determine to local extension of malignant soft tissue tumors. To assess the clinical and MR imaging parameters associated with synovial sarcomas that aid in establishing their diagnosis. We review the clinical findings and images of 23 histologically confirmed synovial sarcomas that were studied by MR. Synovial sarcomas usually develop in young adults as soft tissue tumors, preferentially in the deep tissues of an extremity in close proximity to a joint. They are characterized as having a lobulated contour and septa, frequently infiltrating neighboring tissues at some point, and are heterogeneous. The presence of hemorrhage, as well as infiltration of the fascia in subcutaneous tumors, suggests the diagnosis of synovial sarcoma. The development of perilesional edema is not uncommon. Although, logically, the clinical and radiological features of synovial sarcomas can overlap with those of other soft tissue tumors, the findings described here are fairly characteristic of these lesions: thus, when present, they should serve to orient the diagnostic process. (Author) 16 refs

  7. Hypereosinophilia associated with genital sarcomas

    International Nuclear Information System (INIS)

    Eosinophils are phagocytic leukocytes, regulators reactions Mast cell mediated hypersensitivity. toxicity and primarily responsible antiparasitic. Predominate in epithelial tissues near the interface surface (skin, digestive tract) .The cytotoxic reaction exerted by deposit cell surface substances from the granules themselves: peroxidases, neurotoxins, and other cationic proteins. Hypereosinophilia is defined as the increase in eosinophils above 1500 / m m3. The most common causes are parasitic infections and reactions allergic. About 60% of tumors may be associated with an elevation eosinophil discrete but marked eosinophilia in these patients is little frequent. Tumors are most associated lung cancer and tumors hematology. There are few reports of this entity associated with uterine sarcomas. Although the pathophysiologic mechanism is unclear, it is assumed that the base is the increased secretion of cytokines eosinofilopoiétics: interleukins (Il) I L-3, Il-5 and G M-CSFR among other possible. Self-morbidity is primarily maintained hypereosinophilic heart, and is derived from the cytotoxic action, with endomyocardial fibrosis and thrombosis. Treatment should be directed at the control of the underlying disease, as good Tumor response was associated with the account and normalizaciónd eosinófiles. Los corticosteroids prednisone, 60 mg / day po) may be effective because antagonize The stimulatory effect of cytokines. In the vast majority of cases, the disease is associated with hypereosinophilia disseminated and poor overall prognosis We present a case of vaginal sarcoma with pulmonary metastases and hypereosinophilia seniors who responded to treatment with chemotherapy

  8. Primary cerebellar extramedullary myeloid cell tumor mimicking oligodendroglioma.

    Science.gov (United States)

    Ho, D M; Wong, T T; Guo, W Y; Chang, K P; Yen, S H

    1997-10-01

    Extramedullary myeloid cell tumors (EMCTs) are tumors consisting of immature cells of the myeloid series that occur outside the bone marrow. Most of them are associated with acute myelogenous leukemia or other myeloproliferative disorders, and a small number occur as primary lesions, i.e., are not associated with hematological disorders. Occurrence inside the cranium is rare, and there has been only one case of primary EMCT involving the cerebellum reported in the literature. The case we report here is a blastic EMCT occurring in the cerebellum of a 3-year-old boy who had no signs of leukemia or any hematological disorder throughout the entire course. The cerebellar tumor was at first misdiagnosed as an "oligodendroglioma" because of the uniformity and "fried egg" artifact of the tumor cells. The tumor disappeared during chemotherapy consisting of 12 treatments. However, it recurred and metastasized to the cerebrospinal fluid (CSF) shortly after the therapy was completed. A diagnosis of EMCT was suspected because of the presence of immature myeloid cells in the CSF, and was confirmed by anti-myeloperoxidase and anti-lysozyme immunoreactivity of the cerebellar tumor. The patient succumbed 1 year and 3 months after the first presentation of the disease. PMID:9341943

  9. Soft tissue sarcomas or intramuscular haematomas?

    Energy Technology Data Exchange (ETDEWEB)

    Taieb, Sophie [Department of Radiology, Centre Oscar Lambret, 03, rue Frederic Combemale, BP 307, 59020 Lille Cedex (France)], E-mail: s-taieb@o-lambret.fr; Penel, Nicolas [Department of Oncology, Centre Oscar Lambret, 03, rue Frederic Combemale, BP 307, 59020 Lille Cedex (France); Vanseymortier, Luc [Department of Surgery, Centre Oscar Lambret, 03, rue Frederic Combemale, BP 307, 59020 Lille Cedex (France); Ceugnart, Luc [Department of Radiology, Centre Oscar Lambret, 03, rue Frederic Combemale, BP 307, 59020 Lille Cedex (France)

    2009-10-15

    Haematomas are common and sarcomas are rare. However the absence of trauma or a light trauma should alert the clinician to the possibility that the abnormality may represent haemorrhage into a tumor and not just haematoma, even in a haemophilic patient. Clinical findings, sonography with Doppler assessment and magnetic resonance images with contrast administration will help in the differential diagnosis. The diagnosis of a high grade sarcoma must be considered in these patients and any doubt should be resolved with a biopsy to avoid tragic consequences of missed sarcoma.

  10. Intraosseous Synovial Sarcoma of the Proximal Tibia

    OpenAIRE

    Beck, Sarah E.; G. Petur Nielsen; Raskin, Kevin A.; Schwab, Joseph H.

    2011-01-01

    Synovial Sarcoma is a malignant mesenchymal tumor that comprises 5–10% of all soft tissue sarcomas. The mean age of onset is thirty years old. Intraosseous presentation is very rare and has only been documented a few times. We report herein a case of a 53-year-old man with synovial sarcoma arising in the left proximal tibia. The patient underwent a wide surgical resection and reconstruction, followed by adjuvant chemotherapy. Three years later, the patient developed a local recurrence that re...

  11. Pediatric rhabdomyosarcomas and nonrhabdomyosarcoma soft tissue sarcoma

    Directory of Open Access Journals (Sweden)

    Agarwala Sandeep

    2006-01-01

    Full Text Available Tumors arising from the soft tissues are uncommon in children, accounting for about 6% of all childhood malignancies. More than half (53% of these originate from the striated muscles and are called rhabdomyosarcomas (RMS the remaining are nonrhabdomyosarcoma soft tissue sarcomas (NRSTS. Almost two-thirds of RMS cases are diagnosed in children < 6 years of age. They can arise at varied locations like the head and neck region, genitourinary tract, extremities, trunk and retroperitoneum. Pathologically RMS is now classified as superior, intermediate and poor outcome histologies. For stratification of treatment and also comparison of results the RMS are now staged both by the clinical grouping and the TNM staging systems. The ultimate outcome depends on the site, extent of disease and histology. Currently, approximately 70% of the patients survive for 5 years or more and are probably cured. This is credited to the use of multi-modal, risk-adapted therapy, refinements in tumor grouping and better supportive care which has emerged out of cooperative studies like Intergroup Rhabdomyosarcoma Study (IRS and the International Society of Pediatric Oncology studies (SIOP. The treatment involves chemotherapy, radiotherapy and organ/function preserving surgery. The gold standard chemotherapy is still vincristine, actinomycin D and cyclophosphamide (VAC regime with high doses of intensity bone marrow rescue with colony stimulating factors. The NRSTS are rare and of heterogenous histologies and so it has been difficult to arrive at a treatment strategy for these. What is definitely understood is that these are usually immature and poorly differentiated tumors that respond poorly to chemotherapy and so surgical resection forms the mainstay of treatment with adjuvant radiotherapy and chemotherapy to prevent local recurrences. In all likelihood, the molecular analysis of RMS will further refine current classification schemes and knowledge of genetic features of

  12. Comprehensive analysis of published phase I/II clinical trials between 1990-2010 in osteosarcoma and Ewing sarcoma confirms limited outcomes and need for translational investment

    Directory of Open Access Journals (Sweden)

    van Maldegem Annemiek M

    2012-01-01

    Full Text Available Abstract Background High grade primary bone sarcomas are rare cancers that affect mostly children and young adults. Osteosarcoma and Ewing sarcoma are the most common histological subtypes in this age group, with current multimodality treatment strategies achieving 55-70% overall survival. As there remains an urgent need to develop new therapeutic interventions, we have reviewed published phase I/II trials that have been reported for osteosarcoma and Ewing sarcoma in the last twenty years. Results We conducted a literature search for clinical trials between 1990 and 2010, either for trials enrolling bone sarcoma patients as part of a general sarcoma indication or trials specifically in osteosarcoma and Ewing sarcoma. We identified 42 clinical trials that fulfilled our search criteria for general sarcoma that enrolled these patient groups, and eight and twenty specific trials for Ewing and osteosarcoma patients, respectively. For the phase I trials which enrolled different tumour types our results were incomplete, because the sarcoma patients were not mentioned in the PubMed abstract. A total of 3,736 sarcoma patients were included in these trials over this period, 1,114 for osteosarcoma and 1,263 for Ewing sarcoma. As a proportion of the worldwide disease burden over this period, these numbers reflect a very small percentage of the potential patient recruitment, approximately 0.6% for Ewing sarcoma and 0.2% for osteosarcoma. However, these data show an increase in recent activity overall and suggest there is still much room for improvement in the current trial development structures. Conclusion Lack of resources and commercial investment will inevitably limit opportunity to develop sufficiently rapid improvements in clinical outcomes. International collaboration exists in many well founded co-operative groups for phase III trials, but progress may be more effective if there were also more investment of molecular and translational research into

  13. The histopathology of a human mesenchymal stem cell experimental tumor model: support for an hMSC origin for Ewing's sarcoma?

    DEFF Research Database (Denmark)

    Burns, J S; Abdallah, B M; Schrøder, Henrik Daa;

    2008-01-01

    Sarcomas display varied degrees of karyotypic abnormality, vascularity and mesenchymal differentiation. We have reported that a strain of telomerized adult human bone marrow mesenchymal stem cells (hMSC-TERT20) spontaneously evolved a tumorigenic phenotype after long-term continuous culture. We...... asked to what extent our hMSC-TERT20 derived tumors reflected events found in human sarcomas using routine histopathological procedures. Early versus late passage hMSC-TERT20 cultures persistently expressed mesenchymal lineage proteins e.g. CD105, CD44, CD99 and vimentin. However, late passage cultures...... hypothesis that sarcomas may arise from hMSC, providing a unique diploid model for exploring human sarcoma biology....

  14. Pediatric Malignant Bone Tumors: A Review and Update on Current Challenges, and Emerging Drug Targets.

    Science.gov (United States)

    Jackson, Twana M; Bittman, Mark; Granowetter, Linda

    2016-07-01

    Osteosarcoma (OS) and the Ewing sarcoma family of tumors (ESFT) are the most common malignant bone tumors in children and adolescents. While significant improvements in survival have been seen in other pediatric malignancies the treatment and prognosis for pediatric bone tumors has remained unchanged for the past 3 decades. This review and update of pediatric malignant bone tumors will provide a general overview of osteosarcoma and the Ewing sarcoma family of tumors, discuss bone tumor genomics, current challenges, and emerging drug targets. PMID:27265835

  15. Depsipeptide (Romidepsin) in Treating Patients With Metastatic or Unresectable Soft Tissue Sarcoma

    Science.gov (United States)

    2014-08-26

    Adult Alveolar Soft-part Sarcoma; Adult Angiosarcoma; Adult Epithelioid Sarcoma; Adult Extraskeletal Chondrosarcoma; Adult Extraskeletal Osteosarcoma; Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Fibrous Histiocytoma; Adult Malignant Hemangiopericytoma; Adult Malignant Mesenchymoma; Adult Neurofibrosarcoma; Adult Rhabdomyosarcoma; Adult Synovial Sarcoma; Gastrointestinal Stromal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Adult Soft Tissue Sarcoma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

  16. Biphasic synovial sarcoma in mandibular region

    Directory of Open Access Journals (Sweden)

    Vijay Wadhwan

    2011-01-01

    Full Text Available The term synovioma was coined by Smith in 1927, and later in 1936 Knox suggested the name synovial sarcoma. It occurs primarily in the paraarticular regions, usually in close association with tendon sheaths, bursae, and joint capsules. On rare occasions it may be seen in areas without any apparent relationship to synovial structures as in parapharyngeal region or the abdominal cavity. The first description of synovial sarcoma in the head and neck region was by Pack and Ariel in 1950. The majority of these tumors seem to take origin from paravertebral connective tissue spaces and manifest as solitary retropharyngeal or parapharyngeal masses near the carotid bifurcation. Synovial sarcoma has been reported in soft palate, tongue, maxillofacial region, angle of mandible, sternoclavicular region, scapular region, and the esophagus. We report a case of 28-year-old male patient with synovial sarcoma in mandibular region with biphasic pattern.

  17. General Information about Adult Soft Tissue Sarcoma

    Science.gov (United States)

    ... sarcoma include the following: Past treatment with radiation therapy for certain cancers. Being exposed to certain chemicals , such as Thorotrast (thorium dioxide), vinyl chloride , or arsenic . Having swelling ( lymphedema ) in the arms or legs for a long ...

  18. Stages of Adult Soft Tissue Sarcoma

    Science.gov (United States)

    ... sarcoma include the following: Past treatment with radiation therapy for certain cancers. Being exposed to certain chemicals , such as Thorotrast (thorium dioxide), vinyl chloride , or arsenic . Having swelling ( lymphedema ) in the arms or legs for a long ...

  19. Treatment Option Overview (Adult Soft Tissue Sarcoma)

    Science.gov (United States)

    ... sarcoma include the following: Past treatment with radiation therapy for certain cancers. Being exposed to certain chemicals , such as Thorotrast (thorium dioxide), vinyl chloride , or arsenic . Having swelling ( lymphedema ) in the arms or legs for a long ...

  20. Treatment Options for Adult Soft Tissue Sarcoma

    Science.gov (United States)

    ... sarcoma include the following: Past treatment with radiation therapy for certain cancers. Being exposed to certain chemicals , such as Thorotrast (thorium dioxide), vinyl chloride , or arsenic . Having swelling ( lymphedema ) in the arms or legs for a long ...

  1. Immunosuppressive Therapy-Related Kaposi Sarcoma

    Science.gov (United States)

    ... in the United States have been diagnosed in homosexual or bisexual men infected with HIV. Signs of ... type of nonepidemic Kaposi sarcoma that develops in homosexual men who have no signs or symptoms of ...

  2. Childhood Soft Tissue Sarcoma: Treatment Information

    Science.gov (United States)

    ... Kidney/Wilms Tumor Liver Cancer Lymphoma (Non-Hodgkin) Lymphoma (Hodgkin) Neuroblastoma Osteosarcoma Retinoblastoma Rhabdomyosarcoma Skin Cancer Soft Tissue Sarcoma Thyroid Cancer Understanding Children's Cancer Anxiety Around Procedures Childhood Cancer Statistics Late ...

  3. Myeloid-derived suppressor cells in Chronic myeloid leukemia

    Directory of Open Access Journals (Sweden)

    Cesarina eGiallongo

    2015-05-01

    Full Text Available The suppression of the immune system create a permissive environment for development and progression of cancer. One population of immunosuppressive cells that have become the focus of intense study is myeloid derived suppressor cells (MDSCs, immature myeloid cells able to induce immune-escape, angiogenesis and tumor progression. Two different subpopulations have been identified and studied: granulocytic and monocytic MDSCs, with a different immunophenotype and immunosuppressive properties. Recently, an accumulation of both Gr-MDSCs and Mo-MDSCs cells has been found in the peripheral blood of chronic myeloid leukemia (CML patients. They are part of the tumor clone showing BCR/ABL expression. Imatinib therapy decreases both MDSCs and arginase 1 levels to normal ones. This review will focus on actual knowledge for human MDSCs and their immunosuppressive activity in CML patients with a critical attention to comparison of Gr-MDSCs and polymorphonuclear cells (PMNs. We will then suggest the monitoring of MDSCs in patients who have discontinued tyrosine kinase inhibitors (TKIs therapy to evaluate if their increase could correlate with disease relapse.

  4. Pseudoaneurysms Masquerading as Malignant Bone Tumours

    Science.gov (United States)

    Byregowda, Suman; Puri, Ajay; Prabhu, Aruna; Janu, Amit

    2016-01-01

    Swelling around the knees can be due to both neoplastic as well as non neoplastic aetiology. One of the rare cause for the same is pseudoaneurysm. Pseudoaneurysms can mimic a bone tumor or a soft tissue sarcoma. Pseudoanuerysms result because of the gradual growth occurring due to constant arterial pressure and development of a reactive fibrous capsule. Inappropriate diagnosis and management of these lesions may result in major morbidity and rarely mortality. We present two cases of pseudoaneurysms around the knees which were misdiagnosed as osteogenic sarcomas. These cases also stress on the importance of multidisciplinary care, in preventing avoidable morbidity, mortality and providing safe and optimal care to the patients. PMID:27437336

  5. Pulmonary metastasectomy from soft tissue sarcomas

    OpenAIRE

    Rodrigo Afonso da Silva Sardenberg; Luiz Poli de Figueiredo; Fábio José Haddad; Jefferson Luiz Gross; Riad Naim Younes

    2010-01-01

    INTRODUCTION: Isolated pulmonary metastases from soft tissue sarcomas occur in 20-50% of these (the issue is about metastases, not lung cancer) patients, and 70% of these patients will present disease limited only to the lungs. Surgical resection is well accepted as a standard approach to treat metastases from soft tissue sarcomas isolated in the lungs, and many studies investigating this technique have reported an overall 5-year survival ranging from 30-40%. The most consistent predictor of ...

  6. Primary biphasic synovial sarcoma of the orbit.

    OpenAIRE

    Ratnatunga, N.; Goodlad, J. R.; Sankarakumaran, N.; Seimon, R.; Nagendran, S.; Fletcher, C D

    1992-01-01

    Synovial sarcoma is one of the most common soft tissue malignancies of adolescents and young adults. Despite its name, it is no longer thought to be histogenetically derived from the synovium. What seems to be the first case of synovial sarcoma to arise in the orbit presented in a 21 year old woman as a slowly enlarging subconjunctival mass. Although this tumour was typically biphasic, the monophasic spindle cell variant arising at this site could easily be confused with less aggressive orbit...

  7. Biphasic synovial sarcoma in mandibular region

    OpenAIRE

    Vijay Wadhwan; Sangeeta Malik; Nitin Bhola; Minal Chaudhary

    2011-01-01

    The term synovioma was coined by Smith in 1927, and later in 1936 Knox suggested the name synovial sarcoma. It occurs primarily in the paraarticular regions, usually in close association with tendon sheaths, bursae, and joint capsules. On rare occasions it may be seen in areas without any apparent relationship to synovial structures as in parapharyngeal region or the abdominal cavity. The first description of synovial sarcoma in the head and neck region was by Pack and Ariel in 1950. The majo...

  8. MicroRNAs in Ewing Sarcoma

    OpenAIRE

    PaulJedlicka; LayneDylla

    2013-01-01

    MicroRNAs have emerged recently as important regulators of gene expression in the cell. Frequently dysregulated in cancer, microRNAs have shed new light on molecular mechanisms of oncogenesis, and have generated substantial interest as biomarkers, and novel therapeutic agents and targets. Recently, a number of studies have examined microRNA biology in Ewing Sarcoma. Findings indicate that alterations in microRNA expression in Ewing Sarcoma are widespread, involve both EWS/Ets oncogenic fusion...

  9. Bronchopulmonary Kaposi's sarcoma in patients with AIDS.

    OpenAIRE

    Mitchell, D. M.; McCarty, M.; Fleming, J.; Moss, F. M.

    1992-01-01

    BACKGROUND: Kaposi's sarcoma is the most common secondary neoplasm to complicate HIV infection and may cause pulmonary disease. METHODS: A prospective study was carried out in 140 consecutive patients who were HIV seropositive and required bronchoscopy for new respiratory symptoms of at least two weeks' duration, with either a chest radiographic abnormality or abnormality of pulmonary function. The patients were classified into those with single local endobronchial lesions of Kaposi's sarcoma...

  10. Surgical management of soft tissue sarcomas

    International Nuclear Information System (INIS)

    This volume reflects the latest thinking in surgical and adjuvant forms of therapy that can be offered to the sarcoma patient. Based on their analysis of sarcoma patients, the authors stress management based on site of origin, and discuss tumors on and about the shoulder girdle, hip joint, extremity, retroperitoneum, etc. Coverage includes methods for limb preservation; techniques for regional node resection; indications and methods for arterial perfusion, cryosurgery and isotope implantation; pre- and post-operative immunotherapy chemotherapy and radiation therapy

  11. Local Recurrence of Extremity Soft Tissue Sarcoma.

    Science.gov (United States)

    Guerrero, Whitney M; Deneve, Jeremiah L

    2016-10-01

    The management of recurrent soft tissue sarcoma is a challenging problem for clinicians and has a significant physical, mental, emotional, and oncologic impact for the patient. Despite excellent limb-preservation therapies, approximately one-quarter of patients may eventually develop recurrence of disease. How to most appropriately manage these patients is a matter of debate. Several treatment options exist, including surgical resection, irradiation, systemic chemotherapy, amputation, and regional therapies. This article highlights the management of recurrent extremity soft tissue sarcoma. PMID:27542648

  12. Studying Genes in Tissue Samples From Younger and Adolescent Patients With Soft Tissue Sarcomas

    Science.gov (United States)

    2016-05-13

    Childhood Alveolar Soft-part Sarcoma; Childhood Angiosarcoma; Childhood Desmoplastic Small Round Cell Tumor; Childhood Epithelioid Sarcoma; Childhood Fibrosarcoma; Childhood Leiomyosarcoma; Childhood Liposarcoma; Childhood Malignant Mesenchymoma; Childhood Neurofibrosarcoma; Childhood Synovial Sarcoma; Chordoma; Desmoid Tumor; Metastatic Childhood Soft Tissue Sarcoma; Nonmetastatic Childhood Soft Tissue Sarcoma; Recurrent Childhood Soft Tissue Sarcoma

  13. Undifferentiated embryonal sarcoma of liver

    Directory of Open Access Journals (Sweden)

    Avyakta Kallam

    2015-12-01

    Full Text Available Undifferentiated embryonal sarcoma of the liver (UESL is a rare malignant hepatic tumor. A 47 year old male presented with symptoms of sour taste in his mouth, occasional nausea, indigestion and 15-pound weight loss over two months. He had an unremarkable upper gastrointestinal endoscopy. Imaging showed a large liver mass in the left hepatic lobe that was resected and then reported as UESL. He went on to develop lung metastases and was initially treated with doxorubicin and ifosfamide followed by switching of therapy to gemcitabine and docetaxel due to progression of disease. He had a good response after two cycles and went on to receive four more cycles, achieving stable disease. We can therefore conclude that the combination of gemcitabine and docetaxel is a potential therapeutic option for patients with UESL.

  14. Undifferentiated Embryonal Sarcoma of Liver.

    Science.gov (United States)

    Kallam, Avyakta; Krishnamurthy, Jairam; Kozel, Jessica; Shonka, Nicole

    2015-12-29

    Undifferentiated embryonal sarcoma of the liver (UESL) is a rare malignant hepatic tumor. A 47 year old male presented with symptoms of sour taste in his mouth, occasional nausea, indigestion and 15-pound weight loss over two months. He had an unremarkable upper gastrointestinal endoscopy. Imaging showed a large liver mass in the left hepatic lobe that was resected and then reported as UESL. He went on to develop lung metastases and was initially treated with doxorubicin and ifosfamide followed by switching of therapy to gemcitabine and docetaxel due to progression of disease. He had a good response after two cycles and went on to receive four more cycles, achieving stable disease. We can therefore conclude that the combination of gemcitabine and docetaxel is a potential therapeutic option for patients with UESL. PMID:26788276

  15. 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.

  16. Synovial sarcoma of the mandible

    Directory of Open Access Journals (Sweden)

    Maryam Khalili

    2012-01-01

    Full Text Available Synovial sarcoma (SS is a relatively common soft tissue tumor but only 6%-7% of cases are diagnosed in the head and neck region. It typically occurs in young adults and is slightly more common in males. The most common sites in the head and neck region are hypopharynx and parapharyngeal spaces. However, SS can also occur in tonsils, tongue, and orofacial soft tissues. It is not difficult to diagnose SS microscopically with its classic biphasic appearance, but the diagnosis of monophasic forms is more challenging especially in unusual locations. In this article, we report a rare case of monophasic SS of the mandible. The clinical, histopathological, and immunohistochemical features are discussed and compared with previously reported cases in the literature. To our knowledge, only six primary involvements have been reported in the jaws. Therefore, our case represents the seventh reported case of SS in the area.

  17. Synovial sarcoma of the heart

    International Nuclear Information System (INIS)

    A young male presented with dyspnoea and was found to have a diastolic murmur at the left lower sternal edge. Trans-thoracic and trans oesophageal echocardiograms found a large mass attached to the tricuspid valve, which was projecting into the right atrium and the right ventricle. The mass was causing significant obstruction of the right ventricular inflow. Emergency surgery had to be performed because the patient developed severe vomiting (of unknown cause) leading to haemodynamic compromise. This condition was not responding to fluid resuscitation as there was obstruction to right ventricular inflow. Intraoperatively the mass was removed and the tricuspid valve was repaired. The histological and immunohistochemical examination of the excised tissue confirmed the rare diagnosis of synovial sarcoma of the heart. Post surgical imaging showed no metastases. The patient received postoperative radio therapy and chemotherapy. The tumour recurred after 6 months and the patient succumbed to his illness and expired. (author)

  18. Transcriptional Heterogeneity and Lineage Commitment in Myeloid Progenitors.

    Science.gov (United States)

    Paul, Franziska; Arkin, Ya'ara; Giladi, Amir; Jaitin, Diego Adhemar; Kenigsberg, Ephraim; Keren-Shaul, Hadas; Winter, Deborah; Lara-Astiaso, David; Gury, Meital; Weiner, Assaf; David, Eyal; Cohen, Nadav; Lauridsen, Felicia Kathrine Bratt; Haas, Simon; Schlitzer, Andreas; Mildner, Alexander; Ginhoux, Florent; Jung, Steffen; Trumpp, Andreas; Porse, Bo Torben; Tanay, Amos; Amit, Ido

    2015-12-17

    Within the bone marrow, stem cells differentiate and give rise to diverse blood cell types and functions. Currently, hematopoietic progenitors are defined using surface markers combined with functional assays that are not directly linked with in vivo differentiation potential or gene regulatory mechanisms. Here, we comprehensively map myeloid progenitor subpopulations by transcriptional sorting of single cells from the bone marrow. We describe multiple progenitor subgroups, showing unexpected transcriptional priming toward seven differentiation fates but no progenitors with a mixed state. Transcriptional differentiation is correlated with combinations of known and previously undefined transcription factors, suggesting that the process is tightly regulated. Histone maps and knockout assays are consistent with early transcriptional priming, while traditional transplantation experiments suggest that in vivo priming may still allow for plasticity given strong perturbations. These data establish a reference model and general framework for studying hematopoiesis at single-cell resolution. PMID:26627738

  19. Primary Synovial Cell Sarcoma of the Heart: A Rare Case

    OpenAIRE

    Anil Sharma; Sunil Dixit; Mohit Sharma; Neeraj Sharma; Omeshwar Sharma

    2015-01-01

    A synovial sarcoma (also known as malignant synovioma) is a rare form of cancer that usually occurs near the joints of the arm, neck, or leg. It is one of the soft tissue sarcomas. Primary cardiac neoplasms are rare. Most common site for synovial sarcoma is lower limb. Synovial sarcoma of the heart is extremely rare. Occurrence of synovial sarcoma at extra synovial site is very uncommon. It is two times common in right side heart than left side so left side synovial sarcoma is rarest of rare,...

  20. Imaging sarcomas of the great vessels and heart.

    Science.gov (United States)

    Bendel, Emily C; Maleszewski, Joseph J; Araoz, Philip A

    2011-10-01

    Primary sarcomas of the aorta, pulmonary artery, superior vena cava, inferior vena cava, and the heart are rare neoplasms. Aortic sarcomas are broadly categorized as either primarily luminal or primarily mural, with luminal sarcomas more likely to be misdiagnosed as thrombus. Pulmonary artery sarcomas are often mistaken for pulmonary embolism both clinically and at imaging. Vena caval sarcomas appear as intraluminal or extraluminal masses connecting to or filling the veins. The most common are leiomyosarcomas of the inferior vena cava. Primary sarcomas of the heart are rare and usually appear as heterogeneous aggressive masses. PMID:21963161

  1. 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.

  2. Bone tumor

    Science.gov (United States)

    Tumor - bone; Bone cancer; Primary bone tumor; Secondary bone tumor ... The cause of bone tumors is unknown. They often occur in areas of the bone that grow rapidly. Possible causes include: Genetic defects ...

  3. Bone Grafts

    Science.gov (United States)

    A bone graft transplants bone tissue. Surgeons use bone grafts to repair and rebuild diseased bones in your hips, knees, spine, and sometimes other bones and joints. Grafts can also repair bone loss caused by some ...

  4. Acute Myeloid Leukemia Presenting as Acute Appendicitis

    Directory of Open Access Journals (Sweden)

    Sherri Rauenzahn

    2013-01-01

    Full Text Available Appendicitis in leukemic patients is uncommon but associated with increased mortality. Additionally, leukemic cell infiltration of the appendix is extremely rare. While appendectomy is the treatment of choice for these patients, diagnosis and management of leukemia have a greater impact on remission and survival. A 59-year-old Caucasian female was admitted to the surgical service with acute right lower quadrant pain, nausea, and anorexia. She was noted to have leukocytosis, anemia, and thrombocytopenia. Abdominal imaging demonstrated appendicitis with retroperitoneal and mesenteric lymphadenopathy for which she underwent laparoscopic appendectomy. Peripheral smear, bone marrow biopsy, and surgical pathology of the appendix demonstrated acute myeloid leukemia (AML with nonsuppurative appendicitis. In the setting of AML, prior cases described the development of appendicitis with active chemotherapy. Of these cases, less than ten patients had leukemic infiltration of the appendix, leading to leukostasis and nonsuppurative appendicitis. Acute appendicitis with leukemic infiltration as the initial manifestation of AML has only been described in two other cases in the literature with an average associated morbidity of 32.6 days. The prompt management in this case of appendicitis and AML resulted in an overall survival of 185 days.

  5. ERYTHEMA NODOSUM REVEALING ACUTE MYELOID LEUKEMIA

    Directory of Open Access Journals (Sweden)

    Chebbi Wafa

    2013-07-01

    Full Text Available Introduction: Erythema nodosum (EN is the most common type of panniculitis. It may be idiopathic or secondary to various etiologies. However, the occurrence of erythema nodosum in malignant hemopathy had rarely been reported. Case report: A 42 year-old woman presented with a four week history of recurrent multiple painful erythematous nodules developed on the lower limbs associated with arthralgia of the ankles and fever. The clinical features of skin lesions with contusiform color evolution allowed establishing the diagnosis of EN. No underlying cause was found. The skin lesions were improved with non-steroidal anti-inflammatory drugs and colchicine. Three months later, the patient consulted for recurrence of EN associated with fever, inflammatory polyarthralgia and hepatosplenomegaly. The peripheral blood count revealed pancytopenia. A bone marrow examination confirmed the diagnosis of acute myeloid leukemia type 2. Initiation of chemotherapy was followed by the complete disappearance of skin lesions of EN. Conclusion: Paraneoplastic erythema nodosum is a rare entity. In the literature, a few cases of association with leukemia have been reported. Exploration for solid neoplasms or hemopathy in case of recurrent EN or resistance to conventional treatment should be systematic

  6. Consolidation of massive bone allografts in limb-preserving operations for bone tumours

    OpenAIRE

    San-Julian, M.; Leyes, M.; Mora, G. (Gonzalo); Cañadell, J.M. (J. M.)

    1995-01-01

    This study analysed the influence of several factors affecting the consolidation time of 83 massive bone allografts in 79 patients with malignant bone tumours: osteosarcoma 57; Ewing's sarcoma 8; malignant fibrous histiocytoma 3; chondrosarcoma 4; fibrosarcoma 5; and giant cell tumours 2. The mean age of the patients was 19 years and the mean length of the allografts was 18 cm. The minimum follow up was for 12 months. The mean consolidation time for metaphyseal and diaphyseal osteotomies was ...

  7. Hypoxia and hypoglycaemia in Ewing's sarcoma and osteosarcoma: regulation and phenotypic effects of Hypoxia-Inducible Factor

    International Nuclear Information System (INIS)

    Hypoxia regulates gene expression via the transcription factor HIF (Hypoxia-Inducible Factor). Little is known regarding HIF expression and function in primary bone sarcomas. We describe HIF expression and phenotypic effects of hypoxia, hypoglycaemia and HIF in Ewing's sarcoma and osteosarcoma. HIF-1α and HIF-2α immunohistochemistry was performed on a Ewing's tumour tissue array. Ewing's sarcoma and osteosarcoma cell lines were assessed for HIF pathway induction by Western blot, luciferase assay and ELISA. Effects of hypoxia, hypoglycaemia and isoform-specific HIF siRNA were assessed on proliferation, apoptosis and migration. 17/56 Ewing's tumours were HIF-1α-positive, 15 HIF-2α-positive and 10 positive for HIF-1α and HIF-2α. Expression of HIF-1α and cleaved caspase 3 localised to necrotic areas. Hypoxia induced HIF-1α and HIF-2α in Ewing's and osteosarcoma cell lines while hypoglycaemia specifically induced HIF-2α in Ewing's. Downstream transcription was HIF-1α-dependent in Ewing's sarcoma, but regulated by both isoforms in osteosarcoma. In both cell types hypoglycaemia reduced cellular proliferation by ≥ 45%, hypoxia increased apoptosis and HIF siRNA modulated hypoxic proliferation and migration. Co-localisation of HIF-1α and necrosis in Ewing's sarcoma suggests a role for hypoxia and/or hypoglycaemia in in vivo induction of HIF. In vitro data implicates hypoxia as the primary HIF stimulus in both Ewing's and osteosarcoma, driving effects on proliferation and apoptosis. These results provide a foundation from which to advance understanding of HIF function in the pathobiology of primary bone sarcomas

  8. Piperlongumine induces apoptotic and autophagic death of the primary myeloid leukemia cells from patients via activation of ROS-p38/JNK pathways

    OpenAIRE

    Xiong, Xin-xin; Liu, Ju-mei; Qiu, Xin-yao; Pan, Feng; Yu, Shang-bin; Xiao-qian CHEN

    2015-01-01

    Aim: To investigate the effects of piperlongumine (PL), an anticancer alkaloid from long pepper plants, on the primary myeloid leukemia cells from patients and the mechanisms of action. Methods: Human BM samples were obtained from 9 patients with acute or chronic myeloid leukemias and 2 patients with myelodysplastic syndrome (MDS). Bone marrow mononuclear cells (BMMNCs) were isolated and cultured. Cell viability was determined using MTT assay, and apoptosis was examined with PI staining or fl...

  9. High ALDH activity identifies chemotherapy-resistant Ewing's sarcoma stem cells that retain sensitivity to EWS-FLI1 inhibition.

    Directory of Open Access Journals (Sweden)

    Ola Awad

    Full Text Available BACKGROUND: Cancer stem cells are a chemotherapy-resistant population capable of self-renewal and of regenerating the bulk tumor, thereby causing relapse and patient death. Ewing's sarcoma, the second most common form of bone tumor in adolescents and young adults, follows a clinical pattern consistent with the Cancer Stem Cell model - remission is easily achieved, even for patients with metastatic disease, but relapse remains frequent and is usually fatal. METHODOLOGY/PRINCIPAL FINDINGS: We have isolated a subpopulation of Ewing's sarcoma cells, from both human cell lines and human xenografts grown in immune deficient mice, which express high aldehyde dehydrogenase (ALDH(high activity and are enriched for clonogenicity, sphere-formation, and tumor initiation. The ALDH(high cells are resistant to chemotherapy in vitro, but this can be overcome by the ATP binding cassette transport protein inhibitor, verapamil. Importantly, these cells are not resistant to YK-4-279, a small molecule inhibitor of EWS-FLI1 that is selectively toxic to Ewing's sarcoma cells both in vitro and in vivo. CONCLUSIONS/SIGNIFICANCE: Ewing's sarcoma contains an ALDH(high stem-like population of chemotherapy-resistant cells that retain sensitivity to EWS-FLI1 inhibition. Inhibiting the EWS-FLI1 oncoprotein may prove to be an effective means of improving patient outcomes by targeting Ewing's sarcoma stem cells that survive standard chemotherapy.

  10. Acute myeloid leukemia with t( 11; 22 )( q23; q11.2) : two cases report and literature review

    Institute of Scientific and Technical Information of China (English)

    王彤

    2014-01-01

    Objective To report two de novo acute myeloid leukemia(AML)patients with t(11;22)(q23;q11.2)and summarize the clinical and biological characteristics.Methods Bone marrow cells morphology,immunophenotype,chromosome karyotype,fluorescence in situ hybridization(FISH),PCR and gene sequencing were performed.Clinical manifestation and routine laboratory tests

  11. Azathioprine-associated acute myeloid leukemia in a patient with Crohn's disease and thiopurine S-methyltransferase deficiency

    DEFF Research Database (Denmark)

    Yenson, P.R.; Forrest, D.; Schmiegelow, K.;

    2008-01-01

    risk of hematologic toxicity and leukemogenesis. We present such a patient who was a slow metabolizer for azathioprine, and developed a rapidly lethal form acute myeloid leukemia after relatively low dose exposure to the drug. There was prominent hemophagocytic activity in the bone marrow, and...

  12. Loss of C/EBP alpha cell cycle control increases myeloid progenitor proliferation and transforms the neutrophil granulocyte lineage

    DEFF Research Database (Denmark)

    Porse, Bo T; Bryder, David; Theilgaard-Mönch, Kim; Hasemann, Marie S; Anderson, Kristina; Damgaard, Inge; Jacobsen, Sten Eirik W; Nerlov, Claus

    2005-01-01

    dissociate the ability of C/EBP alpha to block cell cycle progression through E2F inhibition from its function as a transcriptional activator impair the in vivo development of the neutrophil granulocyte and adipose lineages. We now show that such mutations increase the capacity of bone marrow (BM) myeloid...

  13. Do We Know What Causes Soft Tissue Sarcomas?

    Science.gov (United States)

    ... Topic Can soft tissue sarcomas be prevented? Do we know what causes soft tissue sarcomas? Scientists still ... the instructions for nearly everything our cells do. We usually look like our parents because they are ...

  14. Primary synovial cell sarcoma of the heart: A rare case

    Directory of Open Access Journals (Sweden)

    Anil Sharma

    2015-01-01

    Full Text Available A synovial sarcoma (also known as malignant synovioma is a rare form of cancer that usually occurs near the joints of the arm, neck, or leg. It is one of the soft tissue sarcomas. Primary cardiac neoplasms are rare. Most common site for synovial sarcoma is lower limb. Synovial sarcoma of the heart is extremely rare. Occurrence of synovial sarcoma at extra synovial site is very uncommon. It is two times common in right side heart than left side so left side synovial sarcoma is rarest of rare, only very limited cases are reported. Here, we report a case of primary synovial cell sarcoma of the left side of the heart in a 26-year-old female. She presented with dyspnea and weakness only. She was operated for left-sided mass covering mitral valve; further histo-pathology revealed the mass as synovial cell sarcoma. In Indian scenario, this type of rare case needs documentation.

  15. What Should You Ask Your Doctor about Uterine Sarcoma?

    Science.gov (United States)

    ... after treatment for uterine sarcoma? What should you ask your doctor about uterine sarcoma? It is important ... your work schedule. You may also want to ask about second opinions or about clinical trials for ...

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

    Science.gov (United States)

    ... after treatment for Kaposi sarcoma? What should you ask your doctor about Kaposi sarcoma? As you cope ... honest, open discussions with your doctor. You should ask any question on your mind no matter how ...

  17. Soft tissue sarcoma - diagnosis and treatment

    International Nuclear Information System (INIS)

    Significant progress in the treatment of soft tissue sarcoma (STS), both primary tumor and local recurrences/metastatic disease, has been achieved in recent years. Surgery is essential modality, but the use of combined treatment (standard combination of surgery with adjuvant radiotherapy, chemotherapy in selected cases and perioperative rehabilitation) in highly-experienced centers increased possibility of cure and limitations of extent of local surgery. Current combined therapy together with the use of reconstructive methods allows for limb-sparing surgery in majority of soft tissue sarcoma patients (amputation in 10% of cases as compared to approximately 50% in the 1960 - 70s). The slow, but constant, increase of rate of soft tissue sarcoma patients with long-term survival has been observed. Contemporary 5-year overall survival rate in patients with extremity soft tissue sarcomas is 55 -78%. In case of diagnosis of metastatic disease the prognosis is still poor (survival of approximately 1 year). Good results of local therapy may be expected only after planned (e.g., after preoperative biopsy - tru - cut or incisional) radical surgical excision of primary tumor with pathologically negative margins (R0 resection). Following appropriate diagnostic check-up, adjuvant radiotherapy is necessary in the majority of patients treated with radical surgery need, as well as long-term rehabilitation and follow-up examinations in treating center are needed for at least 5 years. The progress is due to the introduction of targeted therapy acting on molecular or genetic cellular disturbances detected during studies on etiopathogenetic mechanisms of sarcoma subtypes. In view of rarity of sarcomas and necessity of multidisciplinary therapy, the crucial issue is that management of these tumors should be hold in experienced oncological sarcoma centers. (authors)

  18. Dosimetric comparison between VMAT with different dose calculation algorithms and protons for soft-tissue sarcoma radiotherapy

    Energy Technology Data Exchange (ETDEWEB)

    Fogliata, Antonella [Oncology Inst. of Southern Switzerland, Medical Physics Unit, Bellinzona (Switzerland)], e-mail: Antonella.Fogliata-Cozzi@eoc.ch; Scorsetti, Marta; Navarria, Piera [IRCCS Instituto Clinico Humanitas, Radiation Oncology, Rozzano, Milan (Italy)] [and others

    2013-04-15

    Background: To appraise the potential of volumetric modulated arc therapy (VMAT, RapidArc) and proton beams to simultaneously achieve target coverage and enhanced sparing of bone tissue in the treatment of soft-tissue sarcoma with adequate target coverage. Material and methods: Ten patients presenting with soft-tissue sarcoma of the leg were collected for the study. Dose was prescribed to 66.5 Gy in 25 fractions to the planning target volume (PTV) while significant maximum dose to the bone was constrained to 50 Gy. Plans were optimised according to the RapidArc technique with 6 MV photon beams or for intensity modulated protons. RapidArc photon plans were computed with: 1) AAA; 2) Acuros XB as dose to medium; and 3) Acuros XB as dose to water. Results: All plans acceptably met the criteria of target coverage (V{sub 95%} >90-95%) and bone sparing (D{sub 1cm}{sup 3} <50 Gy). Significantly higher PTV dose homogeneity was found for proton plans. Near-to-maximum dose to bone was similar for RapidArc and protons, while volume receiving medium/low dose levels was minimised with protons. Similar results were obtained for the remaining normal tissue. Dose distributions calculated with the dose to water option resulted 5% higher than corresponding ones computed as dose to medium. Conclusion: High plan quality was demonstrated for both VMAT and proton techniques when applied to soft-tissue sarcoma.

  19. Dosimetric comparison between VMAT with different dose calculation algorithms and protons for soft-tissue sarcoma radiotherapy

    International Nuclear Information System (INIS)

    Background: To appraise the potential of volumetric modulated arc therapy (VMAT, RapidArc) and proton beams to simultaneously achieve target coverage and enhanced sparing of bone tissue in the treatment of soft-tissue sarcoma with adequate target coverage. Material and methods: Ten patients presenting with soft-tissue sarcoma of the leg were collected for the study. Dose was prescribed to 66.5 Gy in 25 fractions to the planning target volume (PTV) while significant maximum dose to the bone was constrained to 50 Gy. Plans were optimised according to the RapidArc technique with 6 MV photon beams or for intensity modulated protons. RapidArc photon plans were computed with: 1) AAA; 2) Acuros XB as dose to medium; and 3) Acuros XB as dose to water. Results: All plans acceptably met the criteria of target coverage (V95% >90-95%) and bone sparing (D1cm3 <50 Gy). Significantly higher PTV dose homogeneity was found for proton plans. Near-to-maximum dose to bone was similar for RapidArc and protons, while volume receiving medium/low dose levels was minimised with protons. Similar results were obtained for the remaining normal tissue. Dose distributions calculated with the dose to water option resulted 5% higher than corresponding ones computed as dose to medium. Conclusion: High plan quality was demonstrated for both VMAT and proton techniques when applied to soft-tissue sarcoma

  20. Tc-99m-tetrofosmin scintigraphy in patients with metastatic soft tissue sarcoma

    International Nuclear Information System (INIS)

    Full text: The lipophilic cationic radiopharmaceutical compound Tc-99m-tetrofosmin, which is approved for clinical use as myocardial perfusion imaging agent, has been demonstrated to be a valuable tool for the detection of a variety of tumors. Recently, Tc-99m-tetrofosmin uptake by sarcomas in vitro as well as in vivo has been reported. Data about the visualization of metastatic soft tissue sarcomas are missing so far. Upon diagnosis of metastatic disease 9 consecutive patients with histopathologically verified soft tissue sarcoma were included in the present study. Three of them had metastatic disease at the time of primary diagnosis thus being not eligible for surgery with curative intent. The remaining 6 patients had developed metastatic disease after initial removal of the primary tumor. Five patients had previously received cytotoxic treatment, the other four patients were chemonaive. All patients underwent whole body planar examination 10 minutes after administration of 500-550 MBq Tc-99m-tetrofosmin and in case of lung metastases SPECT images were carried out. Non-physiological accumulation of the tracer was considered as a positive result. Visualization of distant metastases was achieved in 4 patients (sensitivity 44 %) all of which were chemonaive. The remaining five patients who had had received chemotherapy previously showed false negative results (progressive disease was confirmed by follow up in all of three patients). No difference between planar and SPECT images was found in regard to tumor detection, however, SPECT acquisition supplied additional information for the localization of the metastases. In one patient with diffuse bone marrow infiltration (inflammatory myofibroblastic sarcoma) Tc-99m-tetrofosmin scintigraphy was positive, while CT showed a negative result. According to our results, detection of metastatic soft tissue sarcomas by Tc-99m-tetrofosmin scintigraphy was strongly dependent on history of previous treatment of the patient. A

  1. Synovial sarcoma of kidney in a child: A rare presentation

    OpenAIRE

    Venkatraman Radhakrishnan; Manikandan Dhanushkodi; Kathiresan Narayanswamy; Anand Raja; Shirley Sundersingh; Tenali Sagar

    2016-01-01

    There are no reported cases in the literature of primary renal synovial sarcoma in pediatric patients. The management of renal synovial sarcoma has been extrapolated from the management of soft tissue sarcomas at other sites. We present a 4-year-old female who was suspected to have Wilms’ tumor. The patient underwent guided biopsy as she did not respond to neoadjuvant chemotherapy for Wilms’ tumor. The biopsy was consistent with primary renal synovial sarcoma. The child was treated with chang...

  2. Primary Synovial Sarcoma of the Parietal Pleura: A Case Report

    OpenAIRE

    Kang, Min-Kyun; Cho, Kwang-Hyun; Lee, Yang-Haeng; Han, Il-Yong; Yoon, Young Chul; Park, Kyung-Taek; Kang, Do Kyun; Kim, Bo-Mi

    2013-01-01

    Synovial sarcoma is a malignant soft tissue tumor that most commonly occurs in the extremities of young and middle-aged adults, in the vicinity of large joints. Although synovial sarcoma is frequently associated with joints, it may arise in unexpected sites, such as the mediastinum, heart, lung, pleura, or chest wall. Primary synovial sarcoma of the pleura is rare. To date, nearly 36 cases of primary synovial sarcoma of the pleura have been reported since Gaertner et al. published the first c...

  3. Expression of Smooth Muscle Calponin in Synovial Sarcoma

    OpenAIRE

    Katsuhito Takahashi; Hiroshi Sonobe; Masaharu Tatsuta; Nobuhito Araki; Nobuyuki Hashimoto; Yumiko Koyanagi; Masayuki Manou; Shingo Ishiguro; Hiroko Funai; Ikuo Kudawara; Takafumi Ueda; Hisako Yamamura; Hidefumi Ono; Hideki Yoshikawa

    1999-01-01

    Purpose. Histogenesis of synovial sarcoma remains controversial and reliable molecular markers for diagnosis are necessary. Expression of basic calponin, a smooth muscle differentiation-specific actin-binding protein, was studied in synovial sarcoma. Subjects and Methods. The basic calponin gene and the gene product were analyzed by reverse transcription PCR analysis (RT-PCR) and immunohistochemistry in 14 synovial sarcomas and a human synovial sarcoma cell line (HS-SY-II). Results and Discus...

  4. Midfoot reconstruction with serratus anterior-rib osteomuscular free flap following oncological resection of synovial sarcoma.

    Science.gov (United States)

    Battiston, Bruno; Artiaco, Stefano; Piana, Raimondo; Boux, Elena; Tos, Pierluigi

    2015-12-01

    During recent decades, the concept of surgical treatment of malignant bone and soft tissue sarcomas has evolved, with the aim of preserving limb function. In this paper we report a case of metatarsal reconstruction by means of serratus and rib free flap after excision of a synovial sarcoma located in the dorsal aspect of the midfoot. Five years after the operation, the patient was free from recurrence and recovered full foot function. Amputation has been widely used in the past and this procedure still remains a valuable option when limb salvage is not possible. Nevertheless, in selected cases, reconstruction by means of composite free flaps may be successfully used for limb preservation in the treatment of malignant foot tumors after surgical excision. PMID:25838161

  5. Correlation between radiologic evaluation modalities and histologic tumor response in chemotherapy-treated Ewing sarcoma

    International Nuclear Information System (INIS)

    In Ewing sarcoma, the addition of preoperative and postoperative chemotherapy has dramatically raised the 5-year survival rate. Radiologic evaluation of chemotherapy response becomes important so that the treatment plan can be altered in cases of poor response. The authors evaluated sequential examinations, including plain radiographs, Tc-99m skeletal scintigrams, and CT scans in 48 patients with Ewing sarcoma of bone. In 31 patients, biopsy material was obtained for histologic grading of treatment response. Good tumor response (grades 3 and 4) led over the ensuing 1-3 months to disappearance of the soft-tissue tumor component, solid transformation of the previously lamellated or spiculated periosteal reaction, and filling in of the lytic regions. Insufficient tumor response (grades 1 and 2) demonstrated persistence of soft-tissue tumor component and lamellated or spiculated periosteal reaction as well as absence, filling in, or even enlargement of lytic regions

  6. Presentation of Ewing’s sarcoma in unlikely age group at an unusual location

    Directory of Open Access Journals (Sweden)

    Prasad DV, Sanjay Mulay, Krishna Badgire , Abhinav S.Jadhav, Deepak Datrange, Arun alex

    2014-11-01

    Full Text Available Ewing’s sarcoma is a highly malignant, round cell neoplasm of uncertain origin. It is the sixth most common malignant tumour of bone. It must be distinguished from chronic osteomyelitis and other malignant round cell tumours like lymphoma, metastatic neuroblastoma and small cell osteosarcoma. Most patients are between 10 to 25years old; rarely, patients are younger than age 5 years and older than age 40 years. We report a 55 years female who presented with swelling over right shoulder with pain and inability to move right shoulder later diagnosed as Ewing’s sarcoma of proximal part of humerus right side. The earlier diagnosis at this age may help in better management of the condition and prevent further complications and have a better prognosis.

  7. THE RESPONSE OF DISSEMINATED RETICULUM CELL SARCOMA TO THE INTRAVENOUS INJECTION OF COLLOIDAL RADIOACTIVE GOLD

    Energy Technology Data Exchange (ETDEWEB)

    Rubin, Philip; Levitt, Seymour H.

    1963-06-15

    Case histories of two patients treated with colloidal radiogold for diffuse reticulum cell sarcoma are presented. Further analysis of the method is suggested by the unusually long survival time of one of the patients. It was concluded that, although external radiotherapy remains the treatment of choice in localized reticulum cell sarcoma, intravenous colloidal radiogold may be a useful agent in lymphosarcomas with diffuse minute neoplastic liver and spleen involvements. Intravenous colloidal radiogold can produce bone marrow depression and thrombocytopenia which can lead to death. This factor tends to argue against therapeutic use of the agent. It is suggested that no more than 50 mC Au/sup 198/ intravenously should be used for treatment of this disease. (R.M.G.)

  8. 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, i

  9. Synovial sarcoma arising in association with a popliteal cyst

    International Nuclear Information System (INIS)

    Synovial sarcoma is a relatively common soft tissue sarcoma particularly in the adolescent and young adult. We report an unusual case of a synovial sarcoma arising within a popliteal cyst in a 13-year-old female presenting with bilateral popliteal cysts. MR imaging demonstrated the cyst with evidence of subacute haemorrhage and a discrete nodule of tumour. (orig.)

  10. Primary Synovial Sarcoma of Lung: A Rare Tumor.

    Science.gov (United States)

    Raj, Prince; Kumar, Parveen; Sarin, Yogesh Kumar

    2016-01-01

    Synovial sarcoma of lung is a rare tumor with few case reports in literature. Though named synovial sarcoma due to its resemblance to synovium on light microscopy, it arises from mesenchymal tissue. Here, we present a case of synovial sarcoma of lung in a 7-year old boy, with main emphasis on difficulty faced in the management. PMID:27170917

  11. Synovial sarcoma arising in association with a popliteal cyst

    Energy Technology Data Exchange (ETDEWEB)

    Ayoub, K.S.; Grimer, R.J. [Royal Orthopaedic Hospital, Birmingham (United Kingdom). Dept. of Orthopaedic Oncology; Davies, A.M. [MRI Centre, Royal Orthopaedic Hospital, Birmingham (United Kingdom); Mangham, D.C. [Dept. of Pathology, Royal Orthopaedic Hospital, Birmingham (United Kingdom); Twiston Davies, C.W. [Jersey General Hospital (United Kingdom). Dept. of Orthopaedic Surgery

    2000-12-01

    Synovial sarcoma is a relatively common soft tissue sarcoma particularly in the adolescent and young adult. We report an unusual case of a synovial sarcoma arising within a popliteal cyst in a 13-year-old female presenting with bilateral popliteal cysts. MR imaging demonstrated the cyst with evidence of subacute haemorrhage and a discrete nodule of tumour. (orig.)

  12. Monophasic synovial sarcoma of the pharynx: a case report

    OpenAIRE

    Betal, Dibendu; Babu, Ramesh; Mehmet, Veysi

    2009-01-01

    Synovial sarcomas are a rare form of soft tissue sarcomas. We present a case of a 62 year-old male presenting with a left thyroid lump initially though to be a thyroid adenoma but subsequently diagnosed as a monophasic synovial sarcoma of the pharynx. We discuss the diagnosis and treatment of this case.

  13. A case of epithelioid sarcoma arising in the vulva

    OpenAIRE

    Kim, Ju Hyun; Choi, Youn Seok; Lee, Tae Sung

    2008-01-01

    Epithelioid sarcoma of vulva is extremely rare. Only 20 cases have been reported in the literature so far. Early diagnosis is difficult because of its benign appearance as a painless subcutaneous nodule. Therefore treatment can be delayed, although epithelioid sarcoma requires prompt surgery. We report a case of epithelioid sarcoma that occurred in the left vulva with a brief review of literature.

  14. Sarcomas mandibulares: experiencia quirúrgica en los últimos 10 años Mandibular sarcomas: surgical experience over the past 10 years

    Directory of Open Access Journals (Sweden)

    Javier Gutiérrez Santamaría

    2012-09-01

    Full Text Available Introducción: Los sarcomas mandibulares representan una entidad de difícil estudio por su escasa incidencia e histopatología. Pacientes y métodos: Presentamos la experiencia del servicio de Cirugía Oral y Maxilofacial del Hospital Vall d'Hebron de Barcelona en los últimos 10 años (2001-2010 en el manejo de los sarcomas mandibulares, realizando una revisión retrospectiva de 12 casos de pacientes afectos por este tipo de tumor. Resultados: La técnica más utilizada para la reconstrucción fue el colgajo microvascularizado (hueso peroné: 8/12, recibiendo tratamiento adyuvante (quimioterapia y/o radioterapia el 82% de los pacientes. Cinco pacientes fallecieron (42%, 2 se encuentran con progresión de la enfermedad (16% y 5 sobreviven libres de enfermedad (42% hasta la finalización del seguimiento. Conclusiones: Los casos descritos representan una serie singular debido a la localización mandibular, no antes publicadas en la literatura. Aún así, los resultados obtenidos en términos de supervivencia y factores pronóstico son similares a los descritos para los sarcomas de cabeza y cuello. La consecución de márgenes libres con la cirugía es la clave del tratamiento, siendo necesario el tratamiento complementario para mejorar el pronóstico.Introduction: Sarcomas located in the mandible are difficult to study due to their relatively rare appearance and histology. Patients and Methods: We present the experience of the Oral and Maxillofacial Surgery Department of the Vall d'Hebron Hospital in Barcelona over the last 10 years (2001-2010 in the management of jaw sarcomas, performing a retrospective review of 12 cases of patients affected by this type of tumour. Results: The technique mostly used for the reconstruction was the microvascularised bone graft (fibula: 8/12, with 82% of the patients receiving adjuvant therapy (chemotherapy and radiotherapy. Five of the patients died (42%, two were found with disease progression (16%, and 5

  15. Synovial sarcoma: Clinicopathologic evaluation of 82 cases

    Directory of Open Access Journals (Sweden)

    Taner AKALIN

    2006-09-01

    Full Text Available The current retrospective study examined a relatively large group of synovial sarcoma patients of all ages who were evaluated at a single institution over a 21 year period.There were 42 females and 40 males, whose age ranged from 13 to 75 (median 34 years. The neoplasms measured from 1,5 to 24 cm in greatest diameter (median 8,5 cm. Tumor locations were as follows; lower extremities (53 cases, upper extremities (16 cases, trunk (7 cases and head-neck (3 cases.One hundred thirty six material belonging to 82 cases were evaluated histologically. Of these cases, 58 were monophasic fibrous type, 18 were biphasic type and 6 were poorly differentiated type synovial sarcoma. Immunohistochemically, monophasic fibrous type and poorly differentiated type synovial sarcoma cases (64 cases were positive for EMA or cytokeratin. Of these cases, 14 were EMA positive but cytokeratin negative, however six were cytokeratin positive but EMA negative.Follow up was available for 43 patients with a duration of median 47 months. Of the 43 patients, 12 died of disease, nine had metastatic disease, nine had local reccurence and 13 (30% had no evidence of disease.In conclusion, synovial sarcoma is an aggressive neoplasm and is affected from radiochemotherapy in varying proportions. Most of the synovial sarcoma cases can be diagnosed by the help of morphology and immunohistochemistry (by applying both EMA and cytokeratin.

  16. 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.

  17. MPLW515L is a novel somatic activating mutation in myelofibrosis with myeloid metaplasia.

    OpenAIRE

    Yana Pikman; Lee, Benjamin H; Thomas Mercher; Elizabeth McDowell; Ebert, Benjamin L.; Maricel Gozo; Adam Cuker; Gerlinde Wernig; Sandra Moore; Ilene Galinsky; DeAngelo, Daniel J.; Clark, Jennifer J.; Lee, Stephanie J.; Golub, Todd R.; Martha Wadleigh

    2006-01-01

    Editors' Summary Background. Myelofibrosis with myeloid metaplasia (MF) is one of a group of chronic blood disorders, known as chronic myeloproliferative disorders. These disorders sometimes turn into acute leukemia. The main abnormality in myelofibrosis is for the bone marrow to become filled with fibrous (scar) tissue (hence the name myelofibrosis), which stops it from producing normal blood cells efficiently. In addition, the white blood cells that remain are abnormal (that is, metaplastic...

  18. Aspirin inhibits in vitro maturation and in vivo immunostimulatory function of murine myeloid dendritic cells

    OpenAIRE

    Hackstein, H; Morelli, AE; Larregina, AT; Ganster, RW; Papworth, GD; Logar, AJ; Watkins, SC; Falo, LD; Thomson, AW

    2001-01-01

    Aspirin is the most commonly used analgesic and antiinflammatory agent. In this study, at physiological concentrations, it profoundly inhibited CD40, CD80, CD86, and MHC class II expression on murine, GM-CSF + IL-4 stimulated, bone marrow-derived myeloid dendritic cells (DC). CD11c and MHC class I expression were unaffected. The inhibitory action was dose dependent and was evident at concentrations higher than those necessary to inhibit PG synthesis. Experiments with indomethacin revealed tha...

  19. Resistance to chemotherapy is associated with altered glucose metabolism in acute myeloid leukemia

    OpenAIRE

    SONG, KUI; Li, Min; Xu, Xiaojun; Xuan, Li; HUANG, GUINIAN; Liu, Qifa

    2016-01-01

    Altered glucose metabolism has been described as a cause of chemoresistance in multiple tumor types. The present study aimed to identify the expression profile of glucose metabolism in drug-resistant acute myeloid leukemia (AML) cells and provide potential strategies for the treatment of drug-resistant AML. Bone marrow and serum samples were obtained from patients with AML that were newly diagnosed or had relapsed. The messenger RNA expression of hypoxia inducible factor (HIF)-1α, glucose tra...

  20. Priapism - A Rare Presentation in Chronic Myeloid Leukemia: Case Report.

    Science.gov (United States)

    Nerli, Rajendra B; Magdum, Prasad V; Hiremath, Siddayya C; Patil, Amey Y; Pai, Suvarna V; Handigund, Rajeshwari S; Hiremath, M B

    2016-01-01

    Priapism is a complication rarely seen in leukemia. We report a 19-year-old man presented with persistent painful erection of penis for over 24 hours at home. The patient had underwent immediate irrigation and decompression of priapism by urologist at emergency services of the hospital. This approach resulted in a flaccid penis later. During hospitalization, peripheral blood smear and bone marrow aspiration confirmed the diagnosis of chronic myeloid leukemia. PMID:26793565