Stavroussi, Panayiota; Andreou, Georgia; Karagiannopoulou, Dimitra
The purpose of this study was to examine verbal fluency and verbal short-term memory in 12 adults with Down syndrome (DS) and 12 adults with Intellectual Disability (ID) of unspecified origin, matched for receptive vocabulary and chronological age. Participants' performance was assessed on two conditions of a verbal fluency test, namely, semantic…
... Vascular Tumors Treatment Research Adult Soft Tissue Sarcoma Treatment (PDQ®)–Patient Version General Information About Adult Soft ... dye reacts to the light. Certain factors affect treatment options and prognosis (chance of recovery). The treatment ...
... Vascular Tumors Treatment Research Adult Soft Tissue Sarcoma Treatment (PDQ®)–Patient Version General Information About Adult Soft ... dye reacts to the light. Certain factors affect treatment options and prognosis (chance of recovery). The treatment ...
van der Graaf, Winette T A; Orbach, Daniel; Judson, Ian R; Ferrari, Andrea
Survival outcomes for adolescent and young adult patients with soft tissue sarcomas lag behind those of children diagnosed with histologically similar tumours. To help understand these differences in outcomes, we discuss the following issues with regard to the management of these patients with soft tissue sarcomas: delays in diagnosis, trial availability and participation, aspects of the organisation of care (with an emphasis on age-specific needs), national centralisation of sarcoma care, international consortia, and factors related to tumour biology. Improved understanding of the causes of the survival gap between adolescents and young adults with sarcomas will help drive new initiatives to improve final health outcomes in these populations. In this Review, we specifically focus on embryonal and alveolar rhabdomyosarcoma, synovial sarcoma, and adult soft tissue sarcomas diagnosed in adolescents and young adults, and discuss the age-specific needs of these patients. Copyright © 2017 Elsevier Ltd. All rights reserved.
Full Text Available Lara E Davis,1,2 Suman Malempati2 1Department of Medicine, 2Department of Pediatrics, Oregon Health and Science University, Portland, OR, USA Abstract: Ewing sarcoma (ES is a rare tumor that most often occurs in adolescents and young adults. This review discusses the diagnosis, prognosis, and treatment of localized and metastatic ES, with an emphasis on the care of adolescent and young adult patients. The pathobiology is reviewed. Particular attention is given to recent and current clinical trials, and an introduction to future directions for therapy of ES is provided. Keywords: Ewing, sarcoma, primitive neuroectodermal tumor, PNET
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.
Andritsch, Elisabeth; Beishon, Marc; Bielack, Stefan; Bonvalot, Sylvie; Casali, Paolo; Crul, Mirjam; Bolton, Roberto Delgado-; Donati, Davide Maria; Douis, Hassan; Haas, Rick; Hogendoorn, Pancras; Kozhaeva, Olga; Lavender, Verna; Lovey, Jozsef; Negrouk, Anastassia; Pereira, Philippe; Roca, Pierre; de Lempdes, Godelieve Rochette; Saarto, Tiina; van Berck, Bert; Vassal, Gilles; Wartenberg, Markus; Yared, Wendy; Costa, Alberto; Naredi, Peter
ECCO essential requirements for quality cancer care (ERQCC) are checklists and explanations of organisation and actions that are necessary to give high-quality care to patients who have a specific tumour type. They are written by European experts representing all disciplines involved in cancer care. ERQCC papers give oncology teams, patients, policymakers and managers an overview of the elements needed in any healthcare system to provide high quality of care throughout the patient journey. References are made to clinical guidelines and other resources where appropriate, and the focus is on care in Europe. Sarcoma: essential requirements for quality care • Sarcomas - which can be classified into soft tissue and bone sarcomas - are rare, but all rare cancers make up more than 20% of cancers in Europe, and there are substantial inequalities in access to high-quality care. Sarcomas, of which there are many subtypes, comprise a particularly complex and demanding challenge for healthcare systems and providers. This paper presents essential requirements for quality cancer care of soft tissue sarcomas in adults and bone sarcomas. • High-quality care must only be carried out in specialised sarcoma centres (including paediatric cancer centres) which have both a core multidisciplinary team and an extended team of allied professionals, and which are subject to quality and audit procedures. Access to such units is far from universal in all European countries. • It is essential that, to meet European aspirations for high-quality comprehensive cancer control, healthcare organisations implement the requirements in this paper, paying particular attention to multidisciplinarity and patient-centred pathways from diagnosis and follow-up, to treatment, to improve survival and quality of life for patients. Taken together, the information presented in this paper provides a comprehensive description of the essential requirements for establishing a high-quality service for soft
Hartmann, Jörg T
For the subgroup of patients with inoperable gastrointestinal stromal tumors, progress has been made by the rapid development and approval of the targeted therapy imatinib mesylate. Small round cell sarcoma, such as Ewing/PNET, desmoplastic small round cell sarcoma and rhabdomyosarcoma, are chemotherapy-sensitive and potentially curable malignancies, which are treated with multimodality, dose-intensitive and neoadjuvant protocols regardless of size or overt metastatic disease. A limited number of effective agents available for the treatment of patients with metastatic adult soft-tissue sarcoma exists, which have failed anthracyline and ifosfamide-based chemotherapy. Most other high-grade (grading >I) so-called adult-type soft-tissue sarcomas such as fibro, lipo, pleomorphic and synovial sarcoma are treated with a anthracycline-based regimen with or without ifosfamide as front-line therapy. In this review, the therapeutic activities of drugs currently available as second-line treatment in patients with metastatic soft tissue sarcoma are summarized, providing an overview of contentious or emerging treatment issues. In relapsed 'adult-type' soft-tissue sarcomas trofosfamide, gemcitabine and ecteinascidin (ET-743) appear to be drugs associated with moderate activity and an acceptable toxicity profile. An interesting finding to be noted is that the different drugs have particular effects in distinct subtypes of soft-tissue sarcoma; however, it has to be taken into account that the number of patients included in those phase II trials are limited. The role of the newer agents (e.g. patupilone derivates, brostallicin) is currently not definable. The so-called selective therapy targeting vascular endothelial growth factor (receptor), epidermal growth factor receptor, c-kit, Raf kinase or platelet-derived growth factor receptor and bcl-2 antisensing, proteasome, protein kinase C/B, and mammalian target of rabamycin inhibition will continue to be tested in gastrointestinal
Burningham Zachary; Hashibe Mia; Spector Logan; Schiffman Joshua D
Abstract Sarcomas account for over 20% of all pediatric solid malignant cancers and less than 1% of all adult solid malignant cancers. The vast majority of diagnosed sarcomas will be soft tissue sarcomas, while malignant bone tumors make up just over 10% of sarcomas. The risks for sarcoma are not well-understood. We evaluated the existing literature on the epidemiology and etiology of sarcoma. Risks for sarcoma development can be divided into environmental exposures, genetic susceptibility, a...
Melissa H. Tang
Full Text Available Background. Extremity sarcoma represents a heterogeneous group of rare cancers that carries a relatively high morbidity with regards to physical function. Quality of Life (QoL as an outcome is an important consideration in this cohort. We aimed to identify the correlates of QoL in extremity sarcoma cohorts. Methods. A systematic review of the literature on extremity sarcoma in adults from five databases over the last ten years was undertaken. Results. Twelve articles were chosen and assessed for quality. Physical and social function of extremity sarcoma survivors is below that of the general population. Overall QoL scores of these patients are comparable to those of the general population. Studies that used more recently treated cohorts found that patients who had limb sparing surgery displayed superior functional outcomes over those that underwent amputations. Pain and perceiving that the cancer negatively influenced opportunities was associated with poor outcomes. Conclusion. The available literature regarding QoL in extremity sarcoma patients is heterogeneous in terms of aims and assessment tools. Results need to be interpreted in light of the improved management of extremity sarcoma in more recent patient cohorts.
Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Osteosarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Osteosarcoma; Stage I Adult Soft Tissue Sarcoma; Stage II Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma
Iacoangeli, Maurizio; Dobran, Mauro; Di Rienzo, Alessandro; di Somma, Lucia Giovanna Maria; Alvaro, Lorenzo; Moriconi, Elisa; Nocchi, Niccolò; Gladi, Maurizio; Scerrati, Massimo
Although the spine is frequently involved in metastatic Ewing's sarcoma, primary involvement of the spine, beside sacrum, is much less frequent, especially in adult patients. Because of the low incidence of these tumors, there are currently no clinical guidelines outlining their management and a multitude of therapeutic strategies have been employed with varying success. The definitive management of Ewing's sarcoma of the spine, as in other locations, could include the combination of three main modalities: aggressive surgery, radiotherapy, and combined chemotherapy. Whenever possible, en bloc spondylectomy or extralesional resection is preferable, providing a better oncological result with a longer survival and a better preservation of the spine biomechanics. This is the lesson we learned about the case, we present here, of nonmetastatic lumbar localization by Ewing's sarcoma in as adult patient. PMID:23133768
Full Text Available Although the spine is frequently involved in metastatic Ewing's sarcoma, primary involvement of the spine, beside sacrum, is much less frequent, especially in adult patients. Because of the low incidence of these tumors, there are currently no clinical guidelines outlining their management and a multitude of therapeutic strategies have been employed with varying success. The definitive management of Ewing's sarcoma of the spine, as in other locations, could include the combination of three main modalities: aggressive surgery, radiotherapy, and combined chemotherapy. Whenever possible, en bloc spondylectomy or extralesional resection is preferable, providing a better oncological result with a longer survival and a better preservation of the spine biomechanics. This is the lesson we learned about the case, we present here, of nonmetastatic lumbar localization by Ewing’s sarcoma in as adult patient.
Sucari S.C. Vlok
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.
Full Text Available The Ewing sarcoma family of tumors comprises a rare class of cancers of mesenchymal origin. Cases of Ewing’s sarcoma in the central nervous system – specifically, intracranial Ewing’s – are extremely rare. Almost all reported cases have occurred in children. However, this rare presentation can also occur in the adult population. It is important to distinguish these tumors from primitive neuroectodermal tumors at the time of diagnosis. Testing for EWSR1(22q12 gene rearrangement using fluorescence in situ hybridization is a useful tool for making the distinction between these 2 similar but distinct entities. We present here the case of a middle-aged male patient with intracranial Ewing’s sarcoma, and discuss diagnostic challenges and potential new treatment approaches for this rare disease.
AIM: To evaluate the undifferentiated embryonal sarcoma of liver (UESL) in adults in order to improve its diagnosis and treatment. METHODS: Four primary and one recurrent cases of UESL were clinicopathologically evaluated and immunohistochemically investigated with a panel of antibodies using the EnVision+ system. Relevant literature about UESL in adults was reviewed. RESULTS: Three males and one female were enrolled in this study. Their chief complaints were abdominal pain, weight loss, or fever. Laborator...
Xunjie Cheng; Yue Wu; Jie Yao; Schwebel, David C; Guoqing Hu
Introduction: Recent changes in unspecified unintentional injury mortality for the elderly by U.S. state remain unreported. This study aims to examine U.S. state variations in mortality from unspecified unintentional injury among Americans aged 65+, 1999–2013; Methods: Using mortality rates from the U.S. CDC’s Web-based Injury Statistics Query and Reporting System (WISQARS™), we examined unspecified unintentional injury mortality for older adults aged 65+ from 1999 to 2013 by state. Specifica...
Tamada, Tsutomu; Sone, Teruki; Miyaji, Yoshiyuki; Kozuka, Yuji; Ito, Katsuyoshi [Kawasaki Medical School, Okayama (Japan)
Prostatic stromal sarcoma (PSS) is quite rare. Herein, we describe magnetic resonance imaging (MRI) features of a PSS identified in a 26-year-old man with dysuria and hematuria. MRI clearly depicted the extent and multinodular appearance of the tumor, which was mainly located in the central zone of the prostate. The tumor appeared as a heterogeneously signal-hyperintense mass with a pseudocapsule on T2-weighted imaging. Contrast-enhanced T1-weighted MRI showed necrotic portions in the gradually enhanced solid mass, and diffusion-weighted imaging permitted the accurate assessment of the local extent of the tumor. Thus, the appearance on MRI was quite different from that of adenocarcinoma of the prostate.
Van Damme, J.P.; Schmitz, S.; Machiels, J.P.; Galant, C.; Grégoire, V.; Lengelé, B.; Hamoir, M.
Abstract Objectives The primary objectives of this study were to analyse the outcome of patients diagnosed with head and neck soft tissue sarcomas (HNSTS) and to identify relevant prognostic factors. As well as this, we compared the prognostic value of two staging systems proposed by the American Joint Committee on Cancer (AJCC) and the Memorial Sloan-Kettering Cancer Center (MSKCC). Methods From 07/1988 to 01/2008, the charts of 42 adult patients w...
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
Wagner, Michael J; Gopalakrishnan, Vancheswaran; Ravi, Vinod; Livingston, J Andrew; Conley, Anthony P; Araujo, Dejka; Somaiah, Neeta; Zarzour, Maria A; Ratan, Ravin; Wang, Wei-Lien; Patel, Shreyaskumar R; Lazar, Alexander; Ludwig, Joseph A; Benjamin, Robert S
There are no clinical trials specifically addressing chemotherapy for adults with Ewing sarcoma (ES). Five-year event-free survival (EFS) of adults on pediatric studies of ES (44%-47%) is worse than that of children treated with the same therapy (69%). The object of this study was to review the results of therapy with vincristine, ifosfamide, and doxorubicin (VID) in the multidisciplinary treatment of adults with ES at our institution. Charts for adults treated for ES from 1995 to 2011 were retrospectively reviewed. Clinician-reported radiographic tumor response, type of local therapy, pathologic response, and survival data were collected. Seventy-one patients were identified who received VID as initial therapy. The median age was 25 (range: 16-64). Forty-two patients (59%) presented with a localized disease and 29 patients (41%) presented with a distant metastasis. Of all patients treated with VID, 83.6% showed a radiological response. Patients who presented with a localized disease had a 5-year overall survival (OS) of 68% (median not reached), compared with 10.3% (median: 1.9 years) in those who presented with distant metastases. Five-year EFS was 67%. The nine patients with a pelvic primary tumor had inferior 5-year OS (42%) to the 33 with primary tumors at other sites (75%). The 5-year OS of those who had greater than or equal to 95% necrosis after neoadjuvant VID (n = 20; 5-year OS: 84%) was superior to those who had less than 95% necrosis (n = 13; 5-year OS: 53%). In adults with primary ES, VID combined with an adjuvant strategy based on post-treatment percent necrosis has favorable outcomes compared with historical adult controls. Ewing sarcoma (ES) is a rare tumor in adults, and there are no dedicated clinical trials in the adult population. Most therapy is modeled after the published pediatric studies, although the small numbers of adult patients included on those studies did significantly worse than the children. We modeled our treatment on other
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
Full Text Available Introduction: Recent changes in unspecified unintentional injury mortality for the elderly by U.S. state remain unreported. This study aims to examine U.S. state variations in mortality from unspecified unintentional injury among Americans aged 65+, 1999–2013; Methods: Using mortality rates from the U.S. CDC’s Web-based Injury Statistics Query and Reporting System (WISQARS™, we examined unspecified unintentional injury mortality for older adults aged 65+ from 1999 to 2013 by state. Specifically, the proportion of unintentional injury deaths with unspecified external cause in the data was considered. Linear regression examined the statistical significance of changes in proportion of unspecified unintentional injury from 1999 to 2013; Results: Of the 36 U.S. states with stable mortality rates, over 8-fold differences were observed for both the mortality rates and the proportions of unspecified unintentional injury for Americans aged 65+ during 1999–2013. Twenty-nine of the 36 states showed reductions in the proportion of unspecified unintentional injury cause, with Oklahoma (−89%, Massachusetts (−86% and Oregon (−81% displaying the largest changes. As unspecified unintentional injury mortality decreased, mortality from falls in 28 states and poisoning in 3 states increased significantly. Mortality from suffocation in 15 states, motor vehicle traffic crashes in 12 states, and fire/burn in 8 states also decreased; Conclusions: The proportion of unintentional injuries among older adults with unspecified cause decreased significantly for many states in the United States from 1999 to 2013. The reduced proportion of unspecified injury has implications for research and practice. It should be considered in state-level trend analysis during 1999–2013. It also suggests comparisons between states for specific injury mortality should be conducted with caution, as large differences in unspecified injury mortality across states and over time
Full Text Available Abstract Undifferentiated embryonal sarcoma of the liver (USEL is a rare malignant hepatic tumor with a poor prognosis that is usually observed in children (aged 6 to10 years and rarely seen in adults. We present a case of USEL in a 27-year-old woman with no previous history of the disease. Laboratory tests performed on admission showed that the patient had mildly elevated levels of aspartate aminotransferase, alanine transaminase, alkaline phosphatase, lactate dehydrogenase, and γ-glutamyl transpeptidase. The levels of viral hepatitis and tumor serum markers were all within normal limits. Computed tomography showed a large mass involving the right lobe and the medial segment of the liver. Right trisectionectomy was performed. Microscopically, the tumor was composed of pleomorphic and polynuclear dyskaryotic cells in a myxoid stroma with focal eosinophilic globules and no clear differentiation to muscle. Histological diagnosis showed undifferentiated embryonal sarcoma. Adjuvant therapy with cisplatin, vincristine, doxorubicin, cyclophosphamide, and actinomycin D was initiated. We administered a high dose of etoposide to extract the patient’s peripheral blood stem cells and performed radiation therapy and peripheral blood stem cell transplantation. At 5-year follow-up, the patient was alive without any evidence of recurrence. Here, we describe the clinical and histopathological features of USEL as well as the therapeutic options for USEL in adults with this disease.
Full Text Available Overview Alternate Names: PharmGKB Accession Id: PA165108452 External Vocabularies MeSH: Cholecystitis, Acut...e (D041881) SnoMedCT: Acute cholecystitis unspecified (197409009) UMLS: C0149520 (C0149520) MedDRA: Acute... cholecystitis (10000691) NDFRT: Cholecystitis, Acute [Disease/Finding] (N0000010952) ...Common Searches Search Medline Plus Search CTD Pharm GKB: Acute cholecystitis unspecified ...
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
Casey, Dana L.; Alektiar, Kaled M.; Gerber, Naamit K.; Wolden, Suzanne L., E-mail: email@example.com
Purpose: To evaluate feasibility and patterns of failure in adult patients with Ewing sarcoma (ES) treated with whole lung irradiation (WLI) for pulmonary metastases. Methods and Materials: Retrospective review of all ES patients treated at age 18 or older with 12-15 Gy WLI for pulmonary metastases at a single institution between 1990 and 2014. Twenty-six patients met the study criteria. Results: The median age at WLI was 23 years (range, 18-40). The median follow-up time of the surviving patients was 3.8 years (range, 1.0-9.6). The 3-year cumulative incidence of pulmonary relapse (PR) was 55%, with a 3-year cumulative incidence of PR as the site of first relapse of 42%. The 3-year event-free survival (EFS) and overall survival (OS) were 38 and 45%, respectively. Patients with exclusively pulmonary metastases had better outcomes than did those with extrapulmonary metastases: the 3-year PR was 45% in those with exclusively lung metastases versus 76% in those with extrapulmonary metastases (P=.01); the 3-year EFS was 49% versus 14% (P=.003); and the 3-year OS was 61% versus 13% (P=.009). Smoking status was a significant prognostic factor for EFS: the 3-year EFS was 61% in nonsmokers versus 11% in smokers (P=.04). Two patients experienced herpes zoster in the radiation field 6 and 12 weeks after radiation. No patients experienced pneumonitis or cardiac toxicity, and no significant acute or late sequelae were observed among the survivors. Conclusion: WLI in adult patients with ES and lung metastases is well tolerated and is associated with freedom from PR of 45% at 3 years. Given its acceptable toxicity and potential therapeutic effect, WLI for pulmonary metastases in ES should be considered for adults, as it is in pediatric patients. All patients should be advised to quit smoking before receiving WLI.
K.M. Glorie (Kristiaan); M. de Klerk; J.J. van de Klundert (Joris); W.C. Zuidema (Wilij); F.H.J. Claas (Frans); W. Weimar (Willem)
textabstractThis paper studies participation of unspecified donors in kidney exchange through simultaneous domino paired donation (DPD) and non-simultaneous extended altruistic donor (NEAD) chains. It extends existing research by investigating the termination of chains, the possibility of
Lee, Ji Ae; Kim, Tae Wan; Min, Jae Hoon; Byon, Sun Jung; Jang, Seung Hoon; Choi, Seung Yeon; Kim, Hong Joo
Undifferentiated sarcoma is an uncommon primary malignant tumor of the liver typically occurring in older children. It is also referred to as malignant mesenchymoma, fibromyxosarcoma, or mesenchymal sarcoma. We experienced a case of undifferentiated sarcoma in 72-year-old male. Contrast enhanced liver CT scan revealed a 3.4 cm heterogeneously enhancing, ill-defined, and low attenuated mass in the left liver and subtle intrahepatic duct dilatation. And, in tubogram, there were segmental stenosis and occlusion from the hilum to the proximal common bile duct. We did ultrasonography guided liver biopsy. The pathologic finding revealed infiltrative growth of atypical cells with rhabdoid features. Some atypical cells showed clear cytoplasm, but no organoid pattern was identified. The stroma around atypical cells was filled with eosinophilic hyaline material. These tumor cells were positive for vimentin only, and the tumor was consistent with undifferentiated sarcoma of the liver.
Merletti, Franco; Richiardi, Lorenzo; Bertoni, Franco; Ahrens, Wolfgang; Buemi, Antoine; Costa-Santos, Cristina; Eriksson, Mikael; Guénel, Pascal; Kaerlev, Linda; Jöckel, Karl-Heinz; Llopis-Gonzalez, Agustin; Merler, Enzo; Miranda, Ana; Morales-Suárez-Varela, Maria M; Olsson, Håkan; Fletcher, Tony; Olsen, Jorn
We investigated the association between occupational factors and risk of bone sarcoma, a rare tumor with a largely unknown aetiology. A multicentric case-control study was conducted in 7 European countries in 1995-97. Ninety-six cases aged 35-69 years with a centrally reviewed diagnosis of bone sarcoma (68 chondrosarcomas and 28 osteosarcomas) were compared to 2,632 population (68%) or colon cancer (32%) controls. Subjects were interviewed to obtain information on occupational, medical and reproductive history, smoking and alcohol consumption and selected exposures including use of pesticides. Response proportions were 90% among cases and 66% among controls. Odds ratios (OR) and 95% confidence intervals (CI) were estimated for selected categories of job titles and branches of industry and for use of pesticides. We found an increased OR for bone sarcoma among blacksmiths, toolmakers, machine-tool operators (OR = 2.14, 95% CI 1.08-4.26), woodworkers (OR = 2.68, 95% CI 1.36-5.29) and construction workers (OR = 1.62, 95% CI 0.92-2.87). Ever users of pesticide had an OR of 2.33 (95% CI 1.31-4.13), with similar risks for exposure to insecticides and exposure to herbicides. Neither duration of employment in any of the analyzed occupational categories nor duration of use of pesticides showed an increasing trend in the risk of bone sarcoma. ORs of bone sarcoma were 1.03 (95% CI 0.23-4.57), 3.13 (95% CI 1.26-7.76) and 1.44 (95% CI 0.43-4.85) for the first, second and third tertile of days of use of pesticides. Our study suggests that novel and previously reported (woodworking) occupational factors play a role in the aetiology of bone sarcomas.
... forms in soft tissues of the body, including muscle, tendons, fat, blood vessels, lymph vessels, nerves, and tissue around joints. Soft tissue sarcoma occurs in children and adults. Soft ... disorders can increase the risk of childhood soft tissue sarcoma. Anything ...
... forms in soft tissues of the body, including muscle, tendons, fat, blood vessels, lymph vessels, nerves, and tissue around joints. Soft tissue sarcoma occurs in children and adults. Soft ... disorders can increase the risk of childhood soft tissue sarcoma. Anything ...
... forms in soft tissues of the body, including muscle, tendons, fat, blood vessels, lymph vessels, nerves, and tissue around joints. Soft tissue sarcoma occurs in children and adults. Soft ... disorders can increase the risk of childhood soft tissue sarcoma. Anything ...
Bone cancer - Ewing sarcoma; Ewing family of tumors; Primitive neuroectodermal tumors (PNET); Bone neoplasm - Ewing sarcoma ... this tissue to help determine how aggressive the cancer is and what treatment may be best.
Fernebro, Josefin; Carneiro, Ana; Rydholm, Anders
Purpose. Patients with soft tissue sarcomas (STS) are at increased risk of second primary malignancies, including a second STS, but distinction between metastases and a second primary STS is difficult. Patients and Methods. Array-based comparative genomic hybridization (aCGH) was applied to 30...... multiple STS of the extremities and the trunk wall from 13 patients. Different histotypes were present with malignant fibrous histiocytomas/undifferentiated pleomorphic sarcomas being the predominant subtype. Results. aCGH profiling revealed genetic complexity with multiple gains and losses in all tumors....... In an unsupervised hierarchical cluster analysis, similar genomic profiles and close clustering between the first and subsequent STS were identified in 5 cases, suggesting metastatic disease, whereas the tumors from the remaining 8 patients did not cluster and showed only weak pairwise correlation, suggesting...
Full Text Available Purpose. Patients with soft tissue sarcomas (STS are at increased risk of second primary malignancies, including a second STS, but distinction between metastases and a second primary STS is difficult. Patients and Methods. Array-based comparative genomic hybridization (aCGH was applied to 30 multiple STS of the extremities and the trunk wall from 13 patients. Different histotypes were present with malignant fibrous histiocytomas/undifferentiated pleomorphic sarcomas being the predominant subtype. Results. aCGH profiling revealed genetic complexity with multiple gains and losses in all tumors. In an unsupervised hierarchical cluster analysis, similar genomic profiles and close clustering between the first and subsequent STS were identified in 5 cases, suggesting metastatic disease, whereas the tumors from the remaining 8 patients did not cluster and showed only weak pairwise correlation, suggesting development of second primary STS. Discussion. The similarities and dissimilarities identified in the first and second STS suggest that genetic profiles can be used to distinguish soft tissue metastases from second primary STS. The demonstration of genetically different soft tissue sarcomas in the same patient suggests independent tumor origin and serves as a reminder to consider development of second primary STS, which has prognostic and therapeutic implications.
Mohagheghi Mohammad Ali
Full Text Available To define efficacy of pre-operative chemotherapy in down staging of advanced non-round cell soft tissue sarcomas. From Sep 2002 to Dec 2005, 70 patients were treated by Ifosfamid, MESNA, cisplatin, adriamycin, mitomycin and subsequent surgery. Postoperatively, patients received radiotherapy in cases of microscopically incomplete resection or local recurrence. The median age of the patients was 34 years and the median tumor size was 14 cm. According to AJCC classification 46 patients had stage 3 and 24 had stage 4 diseases. The most common subtypes were MFH and leiomyosarcoma. The most common sites of tumors were lower extremity and trunk. Toxicity grades three or higher consisted of nausea, Leucopenia and infection. About 50% of the patients received G-CSF. Response to chemotherapy was assessable in 63 patients; 9 patients achieved complete response and 16 showed partial response. Disease progressed in 8 and did not change in 37. The best response was seen with MFH, fibrosarcoma and synovial sarcoma. After chemotherapy seventy percent of patients underwent complete surgery. Disease relapsed in 41 patients and twenty two patients died of metastasis. Median survival of patients was 30 months. IMAP plus G-CSF is safe and effective as preoperative chemotherapy in some subtypes of sarcomas, although the metastasis problem has not been eliminated
Girish D. Bakhshi
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.
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)
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.
Maher, Ossama M; Khatua, Soumen; Mukherjee, Devashis; Olar, Adriana; Lazar, Alexander; Luthra, Raja; Liu, Diane; Wu, Jimin; Ketonen, Leena; Zaky, Wafik
There is a paucity of literature reporting the outcome of intracranial sarcomas (IS) in children, adolescents, and young adults (CAYA). A multimodal therapeutic approach is commonly used, with no well-established treatment consensus. We conducted a retrospective review of CAYA with IS, treated at our institution, to determine their clinical findings, treatments, and outcomes. Immunohistochemistry (PDGFRA and EGFR) and DNA sequencing were performed on 5 tumor samples. A literature review of IS was also conducted. We reviewed 13 patients (median age, 7 years) with a primary diagnosis of IS between 1990 and 2015. Diagnoses included unclassified sarcoma (n = 9), chondrosarcoma (n = 2), and rhabdomyosarcoma (n = 2). Five patients underwent upfront gross total resection (GTR) of the tumor. The 5-drug regimen (vincristine, doxorubicin, cyclophosphamide, etoposide, and ifosfamide) was the most common treatment used. Nine patients died due to progression or recurrence (n = 8) or secondary malignancy (n = 1). The median follow-up period of the 4 surviving patients was 1.69 years (range 1.44-5.17 years). The 5-year progression-free survival and overall survival rates were 21 and 44 %, respectively. BRAF, TP53, KRAS, KIT, ERBB2, MET, RET, ATM, and EGFR mutations were detected in 4 of the 5 tissue samples. All 5 samples were immunopositive for PDGFRA, and only 2 were positive for EGFR. IS remain a therapeutic challenge due to high progression and recurrence rates. Collaborative multi-institutional studies are warranted to delineate a treatment consensus and investigate tumor biology to improve the disease outcome.
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
Full Text Available Peripheral primitive neuroectodermal tumor/Ewing′s sarcoma (PNET/EWS is primarily a tumor of soft tissues and bones. Primary localization of PNET/EWS in genitourinary organs is rare. No data on this localization of PNET/EWS are available in literature from Pakistan. We searched our adult uro-oncology records from 1994 till date and identified all cases of adult genitourinary and adrenal PNET/EWS diagnosed on histology and immunohistochemistry. Their case records were reviewed to obtain data on demographics, presentation, pathologic features, management and outcome. Six cases were found; all were young and had aggressive disease at presentation. Four had renal PNET/EWS. One case each of prostate and adrenal PNET/EWS was seen. Surgery and chemotherapy formed the mainstay of management. Three patients (50% died during treatment, two were lost to follow-up and one case with renal PNET/EWS showed good initial response to chemotherapy but was later on lost to follow-up. In conclusion, PNET/EWS should be considered in the differential diagnosis of genitourinary malignant tumors in young patients. These tumors are aggressive with poor outcome.
Porpiglia, Andrea S; Reddy, Sanjay S; Farma, Jeffrey M
Retroperitoneal sarcomas are rare tumors, representing only 15% of all sarcomas. The mainstay of therapy is surgical resection with negative margins. However, this is challenging because of the late presentation of many of these tumors and involvement with adjacent structures. Decisions on radiation therapy and chemotherapy should be made in a multidisciplinary setting at a tertiary referral center.
Reichardt, P; Nielsen, Ole Steen; Bauer, S;
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...... modification. The main toxicity was haematotoxicity with grade 3/4 neutropenia in 49%, grade 3/4 thrombocytopenia in 23%, and grade 3/4 anaemia in 15% of patients, respectively. Non-haematological toxicity consisted mainly of grade 2/3 dyspnoea in 36% of patients and grade 2/3 fatigue in 28%. One treatment...
Amankwah EK; Conley AP; Reed DR
Ernest K Amankwah,1 Anthony P Conley,2 Damon R Reed2 1Department of Cancer Epidemiology, H Lee Moffitt Cancer Center and Research Institute, Tampa, FL, USA; 2Sarcoma Department, H Lee Moffitt Cancer Center and Research Institute, Tampa, FL, USA Abstract: Sarcomas are cancers arising from the mesenchymal layer that affect children, adolescents, young adults, and adults. Although most sarcomas are localized, many display a remarkable predilection for metastasis to the lungs, liver, bones, subc...
Waks, Zeev; Goldbraich, Esther; Farkash, Ariel; Torresani, Michele; Bertulli, Rossella; Restifo, Nicola; Locatelli, Paolo; Casali, Paolo; Carmeli, Boaz
Clinical decision support systems (CDSSs) are gaining popularity as tools that assist physicians in optimizing medical care. These systems typically comply with evidence-based medicine and are designed with input from domain experts. Nonetheless, deviations from CDSS recommendations are abundant across a broad spectrum of disorders, raising the question as to why this phenomenon exists. Here, we analyze this gap in adherence to a clinical guidelines-based CDSS by examining the physician treatment decisions for 1329 adult soft tissue sarcoma patients in northern Italy using patient-specific parameters. Dubbing this analysis "CareGap", we find that deviations correlate strongly with certain disease features such as local versus metastatic clinical presentation. We also notice that deviations from the guideline-based CDSS suggestions occur more frequently for patients with shorter survival time. Such observations can direct physicians' attention to distinct patient cohorts that are prone to higher deviation levels from clinical practice guidelines. This illustrates the value of CareGap analysis in assessing quality of care for subsets of patients within a larger pathology.
... Treatment? Kaposi Sarcoma About Kaposi Sarcoma What Is Kaposi Sarcoma? Cancer starts when cells in the body begin ... the lungs may cause trouble breathing. Types of Kaposi sarcoma The different types of KS are defined by ...
Maskew, Mhairi; MacPhail, A Patrick; Whitby, Denise; Egger, Matthias; Fox, Matthew P.
Background The possible impact of co-infection with Kaposi’s sarcoma associated herpes virus on the response to antiretroviral therapy (ART) is unknown. Prospective studies are rare, particularly in Africa. Methods We enrolled a prospective cohort of HIV-infected adults initiating ART in Johannesburg, South Africa. Subjects were defined as seropositive to KSHV if reactive to either KSHV lytic K8.1 or latent Orf73 antigen or both. Subjects were followed from ART initiation until 18-months on treatment. HIV viral load and CD4 counts were tested 6 monthly. Linear generalized estimating and log-binomial regression models were used to estimate the effect of KSHV infection on immunologic recovery and response as well as HIV viral load suppression within 18-months after ART initiation. Results 385 subjects initiating ART from November 2008-March 2009 were eligible including 184 (48%) KSHV+. The KSHV+ group was similar to the KSHV− in terms of age, gender, initiating CD4 count, body mass index, tuberculosis and haemoglobin levels. The KSHV+ group gained a similar number of cells at 6- (difference of 10 cells/mm3, 95% CI: −11–31), 12- (3 cells/mm3, 95% CI: −19–25) and 18-months (24 cells/mm3, 95% CI: −13–61) compared to the KSHV− group. Adjusted relative risk of failure to suppress viral load to <400 copies/mL (1.03; 95% CI: 0.90–1.17) were similar for KSHV+ and KSHV− by 6-months on treatment. Conclusions In a population with a high KSHV prevalence, HIV-positive adults co-infected with KSHV achieved similar immunologic and virologic responses to ART early after treatment initiation compared to those KSHV−. PMID:23614996
Duczkowska, Agnieszka; Duczkowski, Marek; Bragoszewska, Hanna; Romaniuk-Doroszewska, Anna; Iwanowska, Beata; Szkudlinska-Pawlak, Sylwia; Madzik, Jaroslaw; Bilska, Katarzyna; Raciborska, Anna
Purpose. To check whether primary involvement of brain/spinal cord by bone/soft tissue sarcomas' metastases in children is as rare as described and to present various morphological forms of bone/soft tissue sarcomas' CNS metastases. Methods. Patients with first diagnosis in 1999–2014 treated at single center were included with whole course of disease evaluation. Brain/spinal canal magnetic resonance imaging (MRI)/computed tomography were performed in cases suspicious for CNS metastases. Extension from skull/vertebral column metastases was excluded. Results. 550 patients were included. MRI revealed CNS metastases in 19 patients (incidence 3.45%), 14 boys, aged 5–22 years. There were 12/250 osteosarcoma cases, 2/200 Ewing's sarcoma, 1/50 chondrosarcoma, 3/49 rhabdomyosarcoma (RMS), and 1/1 malignant mesenchymoma. There were 10 single metastases and 7 cases of multiple ones; in 2 RMS cases only leptomeningeal spread in brain and spinal cord was found. Calcified metastases were found in 3 patients and hemorrhagic in 4. In one RMS patient there were numerous solid, cystic, hemorrhagic lesions and leptomeningeal spread. Conclusions. CNS metastases are rare and late in children with bone/soft tissue sarcomas, although in our material more frequent (3.45%) than in other reports (0.7%). Hematogenous spread to brain and hemorrhagic and calcified lesions dominated in osteosarcoma. Ewing sarcoma tended to metastasize to skull bones. Soft tissue sarcomas presented various morphological forms.
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
Aljabab, A. S.; Nason, R. W.; Kazi, R; Pathak, K. A.
Sarcomas are malignant neoplasms originating from mesodermal tissues and constitute less than 1% of body’s tumors, including those of the head and neck region. 5–15% of adult sarcomas are in the head and neck region (20% from bones and cartilages and 80% in soft tissues). Commonly encountered sarcomas in the head and neck region are - osteosarcoma, rhabdomyosarcoma, malignant fibrous histiocytoma, fibrosarcoma and angiosarcoma. This article reviews the available literature on head and neck sa...
Clear cell sarcoma of kidney(CCSK) is a rare and malignant sarcoma, whose the origin of organization is not clear,and most reports indicated the patients is children,but not adults.The sarcoma has hard prognosis and poor sur-vival rate.CCSK is similar to other renal malignant tumors very much in clinical and histological aspects,therefore,the differential diagnosis with other renal malignant tumor is very important.Now we report a case of an adult female pa-tients who was already diagnosed with CCSK of the kidney,and state the discrimination with CCSK of kidney and Wilms’tumor.%肾透明细胞肉瘤（CCSK）是一种组织来源尚未十分清楚的罕见软组织恶性肿瘤，该病多见于儿童，成人患者罕见。成人CCSK预后极差，生存期短。 CCSK临床表现和组织形态学与多种肾恶性肿瘤相似，误诊率较高，在患者预后和治疗方面，CCSK与其他肾恶性肿瘤差别较大，因此，CCSK与其他肾恶性肿瘤的鉴别诊断对于CCSK的确诊至关重要。本文报道我院1例已确诊为CCSK的成年女性患者，简要阐述该疾病与肾透明细胞癌、肾母细胞瘤的鉴别。
Ho Hyun Kim; Jung Chul Kim; Eun Kyu Park; Young Hoe Hur; Yang Seok Koh; Chol Kyoon Cho; Hyung Seok Kim; Hyun Jong Kim
BACKGROUND: Undifferentiated embryonal sarcoma (UES) of the liver is a rare, highly malignant neoplasm with a poor prognosis occurring almost exclusively in late childhood (6-10 years of age). Only a few cases have been reported in adults, accounting for less than 1% of all primary liver neoplasms. METHODS: A 47-year-old woman presented with a palpable mass in the left upper abdomen. Magnetic resonance imaging revealed a 12×10 cm cystic mass with hemorrhage in the left lateral segment of the liver. The initial impression was a hemorrhagic cystic tumor of the liver. The patient underwent a left lateral sectionectomy of the liver. Histopathology and immunohistochemistry helped make a diagnosis of UES. RESULTS: The patient recovered uneventfully and received systemic chemotherapy. Radiologic examination for follow-up revealed a metastatic lesion in the lumbar spine (L5). She was subjected to radiotherapy at the lumbar spine. She survived 48 months. CONCLUSION: Although hepatic cyst as UES of the liver is difficult to diagnose because of its rarity in adults and lack of specific findings, it should be considered in a differential diagnosis.
Eduardo Silva Machado
Full Text Available A 59-year-old white woman, SC, after being treated for pneumonia, presented with an increase in the size of lymph nodes. The immunohistochemical examination diagnosed histiocytic sarcoma. Relapse occurred 12 months after starting chemotherapy. The patient evolved with febrile neutropenia, septic shock and death.
Full Text Available Purpose. To check whether primary involvement of brain/spinal cord by bone/soft tissue sarcomas’ metastases in children is as rare as described and to present various morphological forms of bone/soft tissue sarcomas’ CNS metastases. Methods. Patients with first diagnosis in 1999–2014 treated at single center were included with whole course of disease evaluation. Brain/spinal canal magnetic resonance imaging (MRI/computed tomography were performed in cases suspicious for CNS metastases. Extension from skull/vertebral column metastases was excluded. Results. 550 patients were included. MRI revealed CNS metastases in 19 patients (incidence 3.45%, 14 boys, aged 5–22 years. There were 12/250 osteosarcoma cases, 2/200 Ewing’s sarcoma, 1/50 chondrosarcoma, 3/49 rhabdomyosarcoma (RMS, and 1/1 malignant mesenchymoma. There were 10 single metastases and 7 cases of multiple ones; in 2 RMS cases only leptomeningeal spread in brain and spinal cord was found. Calcified metastases were found in 3 patients and hemorrhagic in 4. In one RMS patient there were numerous solid, cystic, hemorrhagic lesions and leptomeningeal spread. Conclusions. CNS metastases are rare and late in children with bone/soft tissue sarcomas, although in our material more frequent (3.45% than in other reports (0.7%. Hematogenous spread to brain and hemorrhagic and calcified lesions dominated in osteosarcoma. Ewing sarcoma tended to metastasize to skull bones. Soft tissue sarcomas presented various morphological forms.
Adult Supratentorial Primitive Neuroectodermal Tumor (PNET); Childhood Supratentorial Primitive Neuroectodermal Tumor; Ewing Sarcoma of Bone; Extraosseous Ewing Sarcoma; Extraosseous Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Peripheral Primitive Neuroectodermal Tumor of the Kidney; Untreated Childhood Supratentorial Primitive Neuroectodermal Tumor
Unspecified kidney donation is an emerging resource to bridge the gap between supply and demand of kidneys for transplantation. However, uncertainty remains among both the transplantation community and lay public with regard to the intention, motivation, and legitimacy of such donors. Even within programs that use unspecified kidney donors, there is a lack of consensus regarding how to optimize the potential of the gifted kidney (and indirectly potentiate the altruistic benefit for the donor). Despite emerging guidance on how to work up unspecified donors, centers have adopted individualized unspecified donor pathways with regards to assessment, evaluation, and use. There are a variety of models for unspecified kidney donation, ranging from donation directly to deceased-donor waiting lists to benefit one recipient or chain transplantations occurring simultaneously (domino-paired donation) or nonsimultaneously (extended altruistic donor chains) to benefit many. After a brief exploration on the basis of altruism, this review will discuss the assessment, evaluation, and reported outcomes associated with unspecified kidney donation. It will also critique current utilization models and highlight some unresolved controversies. The aim is to highlight the principles, practice, and potential of unspecified kidney donation to bridge the current disparate international practice.
Huh, Jimi; Park, Seong Joon; Kim, Hyoung Jung; Lee, Jong Seok; Ha, Hyun Kwon; Tirumani, Sree Harsha; Ramaiya, Nikhil H.
Objective To comprehensively analyze the spectrum of imaging features of the primary tumors and metastatic patterns of the Extraskeletal Ewing sarcoma family of tumors (EES) in adults. Materials and Methods We performed a computerized search of our hospital's data-warehouse from 1996 to 2013 using codes for Ewing sarcoma and primitive neuroectodermal tumors as well as the demographic code for ≥ 18 years of age. We selected subjects who were histologically confirmed to have Ewing sarcoma of extraskeletal origin. Imaging features of the primary tumor and metastatic disease were evaluated for lesion location, size, enhancement pattern, necrosis, margin, and invasion of adjacent organs. Results Among the 70 patients (mean age, 35.8 ± 15.6 years; range, 18-67 years) included in our study, primary tumors of EES occurred in the soft tissue and extremities (n = 20), abdomen and pelvis (n = 18), thorax (n = 14), paravertebral space (n = 8), head and neck (n = 6), and an unknown primary site (n = 4). Most primary tumors manifested as large and bulky soft-tissue masses (mean size, 9.0 cm; range, 1.3-23.0 cm), frequently invading adjacent organs (45.6%) and showed heterogeneous enhancement (73.7%), a well-defined (66.7%) margin, and partial necrosis/cystic degeneration (81.9%). Notably, 29 patients had metastatic disease detected at their initial diagnosis. The most frequent site of metastasis was lymph nodes (75.9%), followed by bone (31.0%), lung (20.7%), abdominal solid organs (13.8%), peritoneum (13.8%), pleura (6.9%), and brain (3.4%). Conclusion Primary tumors of EES can occur anywhere and mostly manifest as large and bulky, soft-tissue masses. Lymph nodes are the most frequent metastasis sites. PMID:26175577
Malignant Rhabdoid Tumors (MRT); Rhabdoid Tumors of the Kidney (RTK); Atypical Teratoid Rhabdoid Tumors (ATRT); Selected Tumors With Rhabdoid Features; Synovial Sarcoma; INI1-negative Tumors; Malignant Rhabdoid Tumor of Ovary; Renal Medullary Carcinoma; Epithelioid Sarcoma
Gouw, Launce G., E-mail: firstname.lastname@example.org [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)
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.
Chung, Jin; Shim, Hyo Sub; Ham, Seok Jin; Kim, Tae Hoon; Kim, Sang Jin [Gangnam Severance Hospital, Seoul (Korea, Republic of)
Peripheral T-cell lymphoma is a rare type of lymphoma that's derived from postthymic lymphoid T cells. Pulmonary involvement of peripheral T-cell lymphoma of the unspecified type is very rare and the imaging findings of this illness have rarely been reported. We present here a case of peripheral T-cell lymphoma of the unspecified type with a cavitary lesion in the lung parenchyma, and we pathologically confirmed this illness by performing video-assisted thoracoscopic surgery.
Full Text Available Synovial sarcoma has been defined by the World Health Organization (WHO in 2002 as a type of mesenchymal tissue cell tumor that exhibits epithelial differentiation and represents the third most common soft-tissue sarcoma in adults, accounting for approximately 10% of soft-tissue sarcomas. To date, only few reports have focused on mediastinal synovial sarcoma imaging findings. Herein, we report a case of a 13 cm primary mediastinal giant synovial sarcoma, diagnosed in a 56-year-old patient admitted in our Department of Radiology with a six-month history of dyspnea and back pain.
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: email@example.com [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)
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.
Trout, Andrew T.; Sharp, Susan E.; Gelfand, Michael J. [Cincinnati Children' s Hospital Medical Center, Department of Radiology, Cincinnati, OH (United States); Turpin, Brian K. [Cincinnati Children' s Hospital Medical Center, Cancer and Blood Diseases Institute, Division of Oncology, Cincinnati, OH (United States); Zhang, Bin [Cincinnati Children' s Hospital Medical Center, Division of Biostatistics and Epidemiology, Cincinnati, OH (United States)
Granulocyte colony-stimulating factors (G-CSF) speed recovery from chemotherapy-induced myelosuppression but the marrow stimulation they cause can interfere with interpretation of F-18 fluorodeoxyglucose positron emission tomography (F-18 FDG PET) exams. To assess the frequency of interfering G-CSF-induced bone marrow activity on FDG PET imaging in children and young adults with Ewing sarcoma and rhabdomyosarcoma and to define an interval between G-CSF administration and FDG PET imaging that limits marrow interference. Blinded, retrospective review of FDG PET exams performed in patients treated with long-acting G-CSF as part of their chemotherapeutic regimen. Exams were subjectively scored by two reviewers (R1 and R2) who assessed the level of marrow uptake of FDG and measured standardized uptake values in the marrow, liver, spleen and blood pool. FDG PET findings were correlated with time since G-CSF administration and with blood cell counts. Thirty-eight FDG PET exams performed in 17 patients were reviewed with 47.4% (18/38) of exams having marrow uptake of FDG sufficient to interfere with image interpretation. Primary predictors of marrow uptake of FDG were patient age (P = 0.0037) and time since G-CSF exposure (P = 0.0028 for subjective marrow uptake of FDG, P = 0.008 [R1] and P = 0.004 [R2] for measured maximum standardized uptake value (SUVmax)). The median interval between G-CSF administration and PET imaging in cases with marrow activity considered normal or not likely to interfere was 19.5 days (range: 7-55 days). In pediatric and young adult patients with Ewing sarcoma and rhabdomyosarcoma, an interval of 20 days between administration of the long-acting form of G-CSF and FDG PET imaging should limit interference by stimulated marrow. (orig.)
Bradley J. Petek
Full Text Available Soft tissue sarcomas are a group of rare tumors derived from mesenchymal tissue, accounting for about 1% of adult cancers. There are over 60 different histological subtypes, each with their own unique biological behavior and response to systemic therapy. The outcome for patients with metastatic soft tissue sarcoma is poor with few available systemic treatment options. For decades, the mainstay of management has consisted of doxorubicin with or without ifosfamide. Trabectedin is a synthetic agent derived from the Caribbean tunicate, Ecteinascidia turbinata. This drug has a number of potential mechanisms of action, including binding the DNA minor groove, interfering with DNA repair pathways and the cell cycle, as well as interacting with transcription factors. Several phase II trials have shown that trabectedin has activity in anthracycline and alkylating agent-resistant soft tissue sarcoma and suggest use in the second- and third-line setting. More recently, trabectedin has shown similar progression-free survival to doxorubicin in the first-line setting and significant activity in liposarcoma and leiomyosarcoma subtypes. Trabectedin has shown a favorable toxicity profile and has been approved in over 70 countries for the treatment of metastatic soft tissue sarcoma. This manuscript will review the development of trabectedin in soft tissue sarcomas.
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)
Sleiffer, S.; Ouali, M.; van Glabbeke, M.;
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...... prognostic and predictive factors for outcome to ifosfamide-based therapy. Methods: A retrospective, exploratory analysis was performed on data from 1337 advanced STS patients who received first-time ifosfamide-containing chemotherapy. For predictive factor analysis, 660 patients treated with doxorubicin...
... Stage Soft Tissue Sarcoma Treating Soft Tissue Sarcomas Chemotherapy for Soft Tissue Sarcomas Chemotherapy (chemo) is the use of drugs given into ... Depending on the type and stage of sarcoma, chemotherapy may be given as the main treatment or ...
... and Prevention Do We Know What Causes Kaposi Sarcoma? Kaposi sarcoma (KS) is caused by infection with a ... Sarcoma? Can Kaposi Sarcoma Be Prevented? More In Kaposi Sarcoma About Kaposi Sarcoma Causes, Risk Factors, and Prevention ...
Full Text Available We present the case of a 54-year-old gentleman who presented with abdominal distension and a CT scan of his abdomen revealed a large (25 cm left hepatic lobe tumor. He received chemotherapy for over 1.5 years. The CT scans at the completion of this therapy revealed that the tumor had actually slightly grown in size. He underwent orthotopic liver transplantation without any major complications. The explant histopathology revealed an undifferentiated embryonal cell sarcoma (UECS composed of relatively bland spindled cells arranged in short fascicles. It is now 10 years and 3 months since his last transplant and the patient remains well with no tumor recurrence.
Full Text Available Abstract Background Kaposi sarcoma (KS is the most common AIDS-defining tumour in HIV-infected individuals in Africa. Kaposi sarcoma herpes virus (KSHV infection precedes development of KS. KSHV co-infection may be associated with worse outcomes in HIV disease and elevated KSHV viral load may be an early marker for advanced HIV disease among untreated patients. We examined the prevalence of KSHV among adults initiating antiretroviral therapy (ART and compared immunological, demographic and clinical factors between patients seropositive and seronegative for KSHV. Results We analyzed cross-sectional data collected from 404 HIV-infected treatment-naïve adults initiating ART at the Themba Lethu Clinic, Johannesburg, South Africa between November 2008 and March 2009. Subjects were screened at ART initiation for antibodies to KSHV lytic K8.1 and latent Orf73 antigens. Seropositivity to KSHV was defined as positive to either lytic KSHV K8.1 or latent KSHV Orf73 antibodies. KSHV viremia was determined by quantitative PCR and CD3, 4 and 8 lymphocyte counts were determined with flow cytometry. Of the 404 participants, 193 (48% tested positive for KSHV at ART initiation; with 76 (39% reactive to lytic K8.1, 35 (18% to latent Orf73 and 82 (42% to both. One individual presented with clinical KS at ART initiation. The KSHV infected group was similar to those without KSHV in terms of age, race, gender, ethnicity, smoking and alcohol use. KSHV infected individuals presented with slightly higher median CD3 (817 vs. 726 cells/mm3 and CD4 (90 vs. 80 cells/mm3 counts than KSHV negative subjects. We found no associations between KSHV seropositivity and body mass index, tuberculosis status, WHO stage, HIV RNA levels, full blood count or liver function tests at initiation. Those with detectable KSHV viremia (n = 19, however, appeared to present with signs of more advanced HIV disease including anemia and WHO stage 3 or 4 defining conditions compared to those in whom
Komdeur, R; Plaat, BE; Graaf, van der WT; Hoekstra, H.J.; Hollema, H; Berg, van den E; Zwart, N; Scheper, R.J.; Molenaar, W.M.
0.0001). P-gp expression was most pronounced in malignant fibrous histiocytoma (MFH), but was low in leiomyosarcomas. MRP1 was expressed in most malignant peripheral nerve sheath tumours (MPNST). LRP was strongly expressed in MFH and unspecified sarcomas, but was low in liposarcomas. MRP1 and LRP ex
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.
Full Text Available Ewing′s sarcoma constitutes three per cent of all pediatric malignancies. Ewing′s sarcoma has generally been more responsive to chemotherapy than adult-type sarcomas, and chemotherapy is now recommended for all patients with this disease. It is essential to integrate local control measures in the form of surgery and/or radiotherapy at the appropriate time, along with chemotherapy to eradicate the disease. This approach has improved the survival substantially to the tune of 70% in localized disease, although outcome for metastatic disease remains dismal. Newer therapeutic approaches are required to improve outcome for metastatic and recurrent or refractory Ewing′s sarcoma in organized co-operative group trials.
Reubi, Jean Claude; Koefoed, Pernille; Hansen, Thomas von O
PURPOSE: Ewing sarcoma is a rapidly growing mesenchymal tumor in young adults. Although it was shown previously to express the cholecystokinin (CCK) gene, it is unknown whether CCK gene expression is detectable at protein level in Ewing sarcoma tumor cell lines, in tumor tissue, and in plasma fro...... in human cancer; Ewing sarcomas synthesize and secrete proCCK that can be identified in plasma as circulating tumor marker....... Ewing sarcoma patients, and, if so, whether CCK peptides might play a role as tumor markers. EXPERIMENTAL DESIGN: CCK gene expression was evaluated with in situ hybridization or reverse transcription-PCR in tumor tissue. CCK precursors and bioactive CCK were measured with specific RIAs in tumor tissue...
Mário Henrique Leite de Alencar
Full Text Available Synovial sarcomas are uncommon malignant mesenchymal tumors occurring mainly near the joints of the extremities of young adults. Synovial sarcomas are exceedingly rare neoplasms of the digestive tract. We report the first diagnosed case of esophageal synovial sarcoma, highlighting its diagnostic features surgical management and follow-up.
Alsaif H Saif
Full Text Available Synovial sarcoma is a malignant mesenchymal neoplasm which commonly occurs in the extremities of adults, in close association with joint capsules, tendon sheaths, bursae and fascial structures. Only a few cases of synovial sarcoma occurring in the abdominal wall have been reported. A case of a primary synovial sarcoma arising from the anterior abdominal wall fascial aponeurosis is presented.
Michael E Herce
Full Text Available Introduction: HIV-associated Kaposi sarcoma (HIV-KS is the most common cancer in Malawi. In 2008, the non-governmental organization, Partners In Health, and the Ministry of Health established the Neno Kaposi Sarcoma Clinic (NKSC to treat HIV-KS in rural Neno district. We aimed to evaluate 12-month clinical outcomes and retention in care for HIV-KS patients in the NKSC, and to describe our implementation model, which featured protocol-guided chemotherapy, integrated antiretroviral therapy (ART and psychosocial support delivered by community health workers. Methods: We conducted a retrospective cohort study using routine clinical data from 114 adult HIV-KS patients who received ART and ≥1 chemotherapy cycle in the NKSC between March 2008 and February 2012. Results: At enrolment 97% of patients (n/N=103/106 had advanced HIV-KS (stage T1. Most patients were male (n/N=85/114, 75% with median age 36 years (interquartile range, IQR: 29–42. Patients started ART a median of 77 days prior to chemotherapy (IQR: 36–252, with 97% (n/N=105/108 receiving nevirapine/lamivudine/stavudine. Following standardized protocols, we treated 20 patients (18% with first-line paclitaxel and 94 patients (82% with bleomycin plus vincristine (BV. Of the 94 BV patients, 24 (26% failed to respond to BV requiring change to second-line paclitaxel. A Division of AIDS grade 3/4 adverse event occurred in 29% of patients (n/N=30/102. Neutropenia was the most common grade 3/4 event (n/N=17/102, 17%. Twelve months after chemotherapy initiation, 83% of patients (95% CI: 74–89% were alive, including 88 (77% retained in care. Overall survival (OS at 12 months did not differ by initial chemotherapy regimen (p=0.6. Among patients with T1 disease, low body mass index (BMI (adjusted hazard ratio, aHR=4.10, 95% CI: 1.06–15.89 and 1 g/dL decrease in baseline haemoglobin (aHR=1.52, 95% CI: 1.03–2.25 were associated with increased death or loss to follow-up at 12 months. Conclusions
Ryan, A J A
Primary cutaneous peripheral T-cell lymphomas (PTL), unspecified, are rare lymphomas, with a poor prognosis. They grow and disseminate rapidly, leading to widespread disease. We report a case of PTL, unspecified occurring on the nose. Despite its aggressive histology, this tumour behaved indolently. It is remarkably similar, clinically and histologically, to four recently described cases that occurred on the ear.
Baby, Anto; Somanathan, Thara; Konoth, Sreedevi
Sarcomas account for less than 1% of malignant neoplasms arising in the head and neck in adults. Laryngeal synovial sarcoma is an extremely rare form of laryngeal malignancy with less than 20 cases reported in the literature. We report the case of a 48-year-old man with synovial sarcoma of the larynx. He underwent excision of the tumor followed by radiation. He is alive in remission at 36 months. The literature on synovial sarcoma of the larynx is reviewed. PMID:28280643
Lemuel; Leon; Dent; Cesar; Yamil; Cardona; Michael; Clause; Buchholz; Roosevelt; Peebles; Julie; Denise; Scott; Derrick; Jerome; Beech; Billy; Ray; Ballard
Soft tissue sarcomas are unusual malignancies comprising 1% of cancer diagnoses in the United States. Undifferentiated pleomorphic sarcoma accounts for approximately 5% of sarcomas occurring in adults. The most common site of metastasis is the lung, with other sites being bone, the brain, and the liver. Metastasis to the gastrointestinal tract has rarely been documented. We present an unusual case of high-grade pleomorphic sarcoma with metastasis to the stomach, complicated by upper gastrointestinal bleedin...
Marina, Neyssa M; Liu, Qi; Donaldson, Sarah S; Sklar, Charles A; Armstrong, Gregory T; Oeffinger, Kevin C; Leisenring, Wendy M; Ginsberg, Jill P; Henderson, Tara O; Neglia, Joseph P; Stovall, Marilyn A; Yasui, Yutaka; Randall, R Lor; Geller, David S; Robison, Leslie L; Ness, Kirsten K
Ewing sarcoma survivors (ESSs) are at increased risk for treatment-related complications. The incidence of treatment-related morbidity and late mortality with aging is unknown. This study reports survival probabilities, estimated with the Kaplan-Meier method, and the cumulative incidence of cause-specific mortality and chronic conditions among ESSs in the Childhood Cancer Survivor Study who were treated between 1970 and 1986. Piecewise exponential models were used to estimate relative rates (RRs) and 95% confidence intervals (CIs) for these outcomes. Chronic conditions were graded with the Common Terminology Criteria for Adverse Events (version 4.03). Among 404 5-year ESSs (median age at last follow-up, 34.8 years; range, 9.1-54.8 years), the 35-year survival rate was 70% (95% CI, 66%-74%). Late recurrence (cumulative incidence at 35 years, 15.1%) was the most common cause of death, and it was followed by treatment-related causes (11.2%). There were 53 patients with subsequent neoplasms (SNs; cumulative incidence at 35 years, 24.0%), and 38 were malignant (14.3% at 35 years). The standardized incidence ratios were 377.1 (95% CI, 172.1-715.9) for osteosarcoma, 28.9 (95% CI, 3.2-104.2) for acute myeloid leukemia, 14.9 (95% CI, 7.9-25.5) for breast cancer, and 13.1 (95% CI, 4.8-28.5) for thyroid cancer. Rates of chronic conditions were highest for musculoskeletal (RR, 18.1; 95% CI, 12.8-25.7) and cardiac complications (RR, 1.8; 95% CI, 1.4-2.3). Thirty-five years after the diagnosis, the cumulative incidences of any chronic conditions and 2 or more chronic conditions were 84.6% (95% CI, 80.4%-88.8%) and 73.8% (95% CI, 67.8%-79.9%), respectively. With extended follow-up, ESSs' risk for late mortality and SNs does not plateau. Treatment-related chronic conditions develop years after therapy, and this supports the need for lifelong follow-up. Cancer 2017;123:2551-60. © 2017 American Cancer Society. © 2017 American Cancer Society.
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
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
Sushma G Gurwale
Full Text Available Epithelioid sarcoma (ES is a rare mesenchymal tumor of unknown histogenesis which displays multidirectional differentiation, predominantly epithelial. They have no normal cellular counterpart and differ from both synovial sarcoma and other carcinomas. It mainly affects young adults. It has two variants, classic type and proximal type. The more common classic type presents as a slowly growing painless nodule or plaque on the distal extremities. It is rare in children and older individuals. There is male predominance. The size varies from few millimeters to several centimeters. Central deeply seated lesions in pelvis and genital tract are termed as proximal ES. It has a multinodular growth pattern and usually occurs in older patients. These are comparatively more aggressive and metastasize early. On histopathological examination, these lesions need to be distinguished from other tumors showing epithelioid morphology. Primary ES of scalp is an exceedingly rare tumor. We present a case of nodular tumor on the scalp with cervical lymph node metastasis.
Jørgensen, Peter Holmberg; Lausten, Gunnar Schwarz; Pedersen, Alma B
AIM: The aim of the database is to gather information about sarcomas treated in Denmark in order to continuously monitor and improve the quality of sarcoma treatment in a local, a national, and an international perspective. STUDY POPULATION: Patients in Denmark diagnosed with a sarcoma, both...... skeletal and ekstraskeletal, are to be registered since 2009. MAIN VARIABLES: The database contains information about appearance of symptoms; date of receiving referral to a sarcoma center; date of first visit; whether surgery has been performed elsewhere before referral, diagnosis, and treatment; tumor...... of Diseases - tenth edition codes and TNM Classification of Malignant Tumours, and date of death (after yearly coupling to the Danish Civil Registration System). Data quality and completeness are currently secured. CONCLUSION: The Danish Sarcoma Database is population based and includes sarcomas occurring...
Xu, Siyan; Hua, Steven Y; Menton, Ronald; Barker, Kerry; Menon, Sandeep; D'Agostino, Ralph B
Two drugs are bioequivalent if the ratio of a pharmacokinetic (PK) parameter of two products falls within equivalence margins. The distribution of PK parameters is often assumed to be log-normal, therefore bioequivalence (BE) is usually assessed on the difference of logarithmically transformed PK parameters (δ). In the presence of unspecified variances, test procedures such as two one-sided tests (TOST) use sample estimates for those variances; Bayesian models integrate them out in the posterior distribution. These methods limit our knowledge on the extent that inference about BE is affected by the variability of PK parameters. In this paper, we propose a likelihood approach that retains the unspecified variances in the model and partitions the entire likelihood function into two components: F-statistic function for variances and t-statistic function for δ. Demonstrated with published real-life data, the proposed method not only produces results that are same as TOST and comparable with Bayesian method but also helps identify ranges of variances, which could make the determination of BE more achievable. Our findings manifest the advantages of the proposed method in making inference about the extent that BE is affected by the unspecified variances, which cannot be accomplished either by TOST or Bayesian method.
Cirurgia conservadora, radioterapia externa e reforço de dose com braquiterapia de alta taxa de dose: uma nova perspectiva no tratamento de sarcomas de partes moles do adulto Limb-sparing surgery, external beam radiotherapy and boost with high-dose rate brachytherapy: a new perspective for the treatment of soft tissue sarcomas in adults
Antonio Cássio Assis Pellizzon
Full Text Available OBJETIVO: Avaliar a influência no controle local de pacientes adultos e portadores de sarcoma de partes moles em extremidades e submetidos a cirurgia conservadora do membro, com braquiterapia de alta taxa de dose (BATD como reforço para a radioterapia externa (RT. MATERIAL E MÉTODOS: Foram avaliados 16 pacientes tratados, de 1993 até 1999. A RT foi utilizada com finalidade pré ou pós-operatória (30--55 Gy e BATD com dose de 18--36 Gy (fx 3--6 Gy BID. Com base no modelo linear quadrático calculou-se a dose efetiva biológica ("biological effective dose" - BED para o tumor e comparou-se seu valor a dados da literatura internacional, que utiliza tratamentos com RT e braquiterapia de baixa taxa de dose (BBTD. RESULTADOS: Os valores médios e medianos da BED para os sarcomas de partes moles foram de 78,5 Gy7 e 80 Gy7. A análise univariada mostrou que a BED para o tumor, quando utilizada BATD, era semelhante ao valor de 83 Gy7 quando utilizada BBTD (p = 0,008. As taxas de controle local, sobrevida livre de doença e sobrevida global atuarial em cinco anos foram de 83,2%, 75% e 93,7%, respectivamente. CONCLUSÕES: A BATD, quando utilizada como método complementar no reforço de dose da RT no tratamento conservador dos sarcomas de partes moles, apresenta taxas de controle local equiparáveis às da literatura internacional; no entanto, estudos com número maior de pacientes e período maior de seguimento são ainda necessários para determinar o verdadeiro potencial da BATD em substituir a BBTD.PURPOSE: To evaluate the influence on local control in adult patients with soft tissue sarcomas of the limbs that underwent limb-sparing surgery and high-dose rate brachytherapy (HDRB in association with teletherapy. MATERIAL AND METHODS: Sixteen patients treated from 1993 to 1999 were reviewed. Teletherapy was used pre- or postoperatively (30--55 Gy in association with HDRB in a dose range of 18--36 Gy (fx 3--6 Gy BID. The linear quadratic model was
In this clinical trial, adult patients with any form of advanced Kaposi sarcoma will be treated with liposomal doxorubicin and bevacizumab every 3 weeks for a maximum of six treatments. Patients who respond to this therapy or have stable disease will rec
... tumor that forms from a certain kind of cell in bone or soft tissue . Ewing sarcoma may be found in the bones of the legs, arms, feet, hands, chest , pelvis , spine , or skull . Ewing sarcoma also may be found in the soft tissue of the trunk, arms, legs, head and neck, abdominal cavity , or ...
D. A. Ryabchikov
Full Text Available The article presents an overview of the literature about breast sarcomas (nonepithelial malignances. Primary sarcomas are extremely rare, with less than 1 % of all malignant tumors of the breast. Breast carcinomas cause an increased interest of the scientists due to their unique clinical and pathological features and unpredictable prognosis.
Full Text Available Extraosseous osteogenic sarcoma is a very rare malignant neoplasm. Out of the more than 400 cases of soft tissue sarcomas on file in our hospital, only 2 were extraosseous osteogenic sarcomas. Both were situated in the thigh. The first case was initially diagnosed as a hematoma and treated by marginal excision. The diagnosis of high-grade osteosarcoma primarily arising in soft tissue was made from histopathologic examination. Radiotherapy of 60 Gy in 30 fractions was given postoperatively. The second patient, primarily diagnosed as having a soft tissue sarcoma, was treated by wide excision. The final pathologic report was high-grade extraosseous osteogenic sarcoma. Adjuvant chemotherapy was given postoperatively. Both patients are alive without local recurrence and distant metastasis at postoperative 90-month and 107-month follow-up, respectively.
Rhabdomyosarcoma; Malignant Peripheral Nerve Sheath Tumors; Chondrosarcoma; Sarcoma, Ewing's; Sarcoma, Alveolar Soft Part; Chordoma; Epithelioid Sarcoma; Giant Cell Tumor of Bone; Hemangiopericytoma; Gastrointestinal Stromal Tumor (GIST)
Joshua D. Schiffman
Full Text Available Ewing's sarcoma (ES is a rare tumor that is most common in children and young adults. Late effects of ES therapy include second cancers, a tragic outcome for survivors of such a young age. This paper will explore the frequencies and types of malignancies that occur after ES. Additionally, it will review how second malignancies have changed with the shift in treatment from high-dose radiation to chemotherapy regimens including alkylators and epipodophyllotoxins. The risk of additional cancers in ES survivors will also be compared to survivors of other childhood cancers. Finally, the possible genetic contribution to ES and second malignancies will be discussed.
Kang, Sung Wook; Jang, Beom Seon [Seoul National University, Seoul (Korea, Republic of)
A three-dimensional numerical model was constructed to analyze the heat transfer of friction stir welding using Fluent and ANSYS multi-physics. The analysis result was used to calculate welding deformation and residual stress. Before the numerical simulation, several simplifying assumptions were applied to the model. Three different methods of heat transfer analysis were employed, and several assumptions were applied to each heat source model. In this work, several parametric studies were performed for certain unspecified variables. The calculated temperature data were compared with experimental data from relevant studies. Additionally, the advantages and disadvantages of the three heat transfer analysis methods were compared.
Korah, Mariam P., E-mail: firstname.lastname@example.org [Department of Radiation Oncology, Emory University, Atlanta, GA (United States); Deyrup, Andrea T. [Department of Pathology, Emory University, Atlanta, GA (United States); Monson, David K.; Oskouei, Shervin V. [Department of Orthopedics, Emory University, Atlanta, GA (United States); Weiss, Sharon W. [Department of Pathology, Emory University, Atlanta, GA (United States); Landry, Jerome; Godette, Karen D. [Department of Radiation Oncology, Emory University, Atlanta, GA (United States)
Purpose: To examine the influence of anatomic location in the upper extremity (UE) vs. lower extremity (LE) on the presentation and outcomes of adult soft tissue sarcomas (STS). Methods and Materials: From 2001 to 2008, 118 patients underwent limb-sparing surgery (LSS) and external beam radiotherapy (RT) with curative intent for nonrecurrent extremity STS. RT was delivered preoperatively in 96 and postoperatively in 22 patients. Lesions arose in the UE in 28 and in the LE in 90 patients. Patients with UE lesions had smaller tumors (4.5 vs. 9.0 cm, p < 0.01), were more likely to undergo a prior excision (43 vs. 22%, p = 0.03), to have close or positive margins after resection (71 vs. 49%, p = 0.04), and to undergo postoperative RT (32 vs. 14%, p = 0.04). Results: Five-year actuarial local recurrence-free and distant metastasis-free survival rates for the entire group were 85 and 74%, with no difference observed between the UE and LE cohorts. Five-year actuarial probability of wound reoperation rates were 4 vs. 29% (p < 0.01) in the UE and LE respectively. Thigh lesions accounted for 84% of the required wound reoperations. The distribution of tumors within the anterior, medial, and posterior thigh compartments was 51%, 26%, and 23%. Subset analysis by compartment showed no difference in the probability of wound reoperation between the anterior and medial/posterior compartments (29 vs. 30%, p = 0.68). Neurolysis was performed during resection in (15%, 5%, and 67%, p < 0.01) of tumors in the anterior, medial, and posterior compartments. Conclusions: Tumors in the UE and LE differ significantly with respect to size and management details. The anatomy of the UE poses technical impediments to an R0 resection. Thigh tumors are associated with higher wound reoperation rates. Tumor resection in the posterior thigh compartment is more likely to result in nerve injury. A better understanding of the inherent differences between tumors in various extremity sites will assist in
R. L. Randall
Full Text Available Ewing's sarcoma is a highly malignant tumor of children and young adults. The molecular mechanisms that underlie Ewing's Sarcoma development are beginning to be understood. For example, most cases of this disease harbor somatic chromosomal translocations that fuse the EWSR1 gene on chromosome 22 with members of the ETS family. While some cooperative genetic events have been identified, such as mutations in TP53 or deletions of the CDKN2A locus, these appear to be absent in the vast majority of cases. It is therefore uncertain whether EWS/ETS translocations are the only consistently present alteration in this tumor, or whether there are other recurrent abnormalities yet to be discovered. One method to discover such mutations is to identify familial cases of Ewing's sarcoma and to then map the susceptibility locus using traditional genetic mapping techniques. Although cases of sibling pairs with Ewing's sarcoma exist, familial cases of Ewing's sarcoma have not been reported. While Ewing's sarcoma has been reported as a 2nd malignancy after retinoblastoma, significant associations of Ewing's sarcoma with classic tumor susceptibility syndromes have not been identified. We will review the current evidence, or lack thereof, regarding the potential of a heritable condition predisposing to Ewing's sarcoma.
Full Text Available Peter Holmberg Jørgensen,1 Gunnar Schwarz Lausten,2 Alma B Pedersen3 1Tumor Section, Department of Orthopedic Surgery, Aarhus University Hospital, Aarhus, 2Tumor Section, Department of Orthopedic Surgery, Rigshospitalet, Copenhagen, 3Department of Clinical Epidemiology, Aarhus University Hospital, Aarhus, Denmark Aim: The aim of the database is to gather information about sarcomas treated in Denmark in order to continuously monitor and improve the quality of sarcoma treatment in a local, a national, and an international perspective. Study population: Patients in Denmark diagnosed with a sarcoma, both skeletal and ekstraskeletal, are to be registered since 2009. Main variables: The database contains information about appearance of symptoms; date of receiving referral to a sarcoma center; date of first visit; whether surgery has been performed elsewhere before referral, diagnosis, and treatment; tumor characteristics such as location, size, malignancy grade, and growth pattern; details on treatment (kind of surgery, amount of radiation therapy, type and duration of chemotherapy; complications of treatment; local recurrence and metastases; and comorbidity. In addition, several quality indicators are registered in order to measure the quality of care provided by the hospitals and make comparisons between hospitals and with international standards. Descriptive data: Demographic patient-specific data such as age, sex, region of living, comorbidity, World Health Organization's International Classification of Diseases – tenth edition codes and TNM Classification of Malignant Tumours, and date of death (after yearly coupling to the Danish Civil Registration System. Data quality and completeness are currently secured. Conclusion: The Danish Sarcoma Database is population based and includes sarcomas occurring in Denmark since 2009. It is a valuable tool for monitoring sarcoma incidence and quality of treatment and its improvement, postoperative
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
Sleiffer, S.; Ouali, M.; van Glabbeke, M.;
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 ...... contribute to further treatment individualisation of advanced STS patients. (C) 2009 Elsevier Ltd. All rights reserved...
... Kaposi Sarcoma What Are the Key Statistics About Kaposi Sarcoma? Before the AIDS epidemic, Kaposi sarcoma (KS) was ... in Kaposi Sarcoma Research and Treatment? More In Kaposi Sarcoma About Kaposi Sarcoma Causes, Risk Factors, and Prevention ...
... Kaposi Sarcoma About Kaposi Sarcoma What’s New in Kaposi Sarcoma Research and Treatment? A great deal of research ... in Kaposi Sarcoma Research and Treatment? More In Kaposi Sarcoma About Kaposi Sarcoma Causes, Risk Factors, and Prevention ...
Tolar, Jakub; Nauta, Alma J; Osborn, Mark J; Panoskaltsis Mortari, Angela; McElmurry, Ron T; Bell, Scott; Xia, Lily; Zhou, Ning; Riddle, Megan; Schroeder, Tania M; Westendorf, Jennifer J; McIvor, R Scott; Hogendoorn, Pancras C W; Szuhai, Karoly; Oseth, Leann; Hirsch, Betsy; Yant, Stephen R; Kay, Mark A; Peister, Alexandra; Prockop, Darwin J; Fibbe, Willem E; Blazar, Bruce R
To study the biodistribution of MSCs, we labeled adult murine C57BL/6 MSCs with firefly luciferase and DsRed2 fluorescent protein using nonviral Sleeping Beauty transposons and coinfused labeled MSCs with bone marrow into irradiated allogeneic recipients. Using in vivo whole-body imaging, luciferase signals were shown to be increased between weeks 3 and 12. Unexpectedly, some mice with the highest luciferase signals died and all surviving mice developed foci of sarcoma in their lungs. Two mice also developed sarcomas in their extremities. Common cytogenetic abnormalities were identified in tumor cells isolated from different animals. Original MSC cultures not labeled with transposons, as well as independently isolated cultured MSCs, were found to be cytogenetically abnormal. Moreover, primary MSCs derived from the bone marrow of both BALB/c and C57BL/6 mice showed cytogenetic aberrations after several passages in vitro, showing that transformation was not a strain-specific nor rare event. Clonal evolution was observed in vivo, suggesting that the critical transformation event(s) occurred before infusion. Mapping of the transposition insertion sites did not identify an obvious transposon-related genetic abnormality, and p53 was not overexpressed. Infusion of MSC-derived sarcoma cells resulted in malignant lesions in secondary recipients. This new sarcoma cell line, S1, is unique in having a cytogenetic profile similar to human sarcoma and contains bioluminescent and fluorescent genes, making it useful for investigations of cellular biodistribution and tumor response to therapy in vivo. More importantly, our study indicates that sarcoma can evolve from MSC cultures.
Wang, Yuanjia; Chen, Huaihou
We examine a generalized F-test of a nonparametric function through penalized splines and a linear mixed effects model representation. With a mixed effects model representation of penalized splines, we imbed the test of an unspecified function into a test of some fixed effects and a variance component in a linear mixed effects model with nuisance variance components under the null. The procedure can be used to test a nonparametric function or varying-coefficient with clustered data, compare two spline functions, test the significance of an unspecified function in an additive model with multiple components, and test a row or a column effect in a two-way analysis of variance model. Through a spectral decomposition of the residual sum of squares, we provide a fast algorithm for computing the null distribution of the test, which significantly improves the computational efficiency over bootstrap. The spectral representation reveals a connection between the likelihood ratio test (LRT) in a multiple variance components model and a single component model. We examine our methods through simulations, where we show that the power of the generalized F-test may be higher than the LRT, depending on the hypothesis of interest and the true model under the alternative. We apply these methods to compute the genome-wide critical value and p-value of a genetic association test in a genome-wide association study (GWAS), where the usual bootstrap is computationally intensive (up to 10(8) simulations) and asymptotic approximation may be unreliable and conservative. © 2012, The International Biometric Society.
... Treatment Childhood Vascular Tumors Treatment Research Kaposi Sarcoma Treatment (PDQ®)–Patient Version General Information About Kaposi Sarcoma ... Certain factors affect prognosis (chance of recovery) and treatment options. The prognosis (chance of recovery ) and treatment ...
Paolo Pedrazzoli, Simona Secondino, Vittorio Perfetti, Patrizia Comoli, Daniela Montagna
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.
Haberthür, F; Almendral, A C; Feichter, G; Torhorst, J K
Sarcoma of the breast represents only 0.2-1% of all mammary malignancies. This study reports 5 such cases, including 2 osteosarcomas, 1 fibro-, 1 lipo-, and 1 malignant fibrous sarcoma. The treatment used was mastectomy in 3 cases with excision of axillary lymph nodes. The remaining 2 patients were treated by simple mastectomy whereby 1 of these received a immediate reconstruction with a prosthesis. 1 patient demonstrated local recurrence and died. The remaining 4 patients did not develop neither metastases nor local recurrence and are still alive after an observing period between 12 months up to 17 years. Today, first-line treatment is wide local excision or simple mastectomy. Excision of the axillary lymphatics, adjuvant radiotherapy, and chemotherapy have been disappointing in the treatment of breast sarcoma.
van Kessel, Anne; Quint, Koen D
Nowadays, Kaposi sarcoma is a multidisciplinary condition, not only observed by dermatologists. Since the HIV epidemic in the 80s and 90s of the last century, more insight into the aetiology of Kaposi sarcoma has been acquired. However, this sarcoma had already been described in 1872 by a Hungarian dermatologist named Moritz Kaposi (1832-1902). Kaposi described the entity as 'idiopathic multiple pigmented sarcoma of the skin'. This entity was an extraordinary diagnosis at that time, mostly observed in Jewish or Mediterranean men. In 1912, 10 years after the death of Moritz Kaposi, the entity name was changed to Kaposi sarcoma.
Kim, Chul-Hwan; Jang, Jong-Won; Kim, Moon-Young; Kim, Yong-Hwan; Kim, Hang-Gul; Kim, Joo-Hwan
Undifferentiated pleomorphic sarcoma (UPS), previously known as malignant fibrous histiocytoma, occurs commonly in the soft tissues in adult, but is rare in the maxillofacial region. It consists of undifferentiated mesenchymal tumor cells resembling histiocytes and fibroblasts. The purpose of this article is to report a case of UPS in the mandible. A 44-year-old patient presented with a painful growing mass in the mandible of two months' duration. Computed tomography and positron emission tomography-computed tomography revealed an ill-defined heterogenous, hypermetabolic mass about 4 cm in size in the left mandible invading adjacent soft tissues. A left mandiblulectomy and reconstruction with a fibular free flap were performed. Immunohistochemical study gave a diagnosis of UPS. The patient was referred for adjuvant chemotherapy after surgical removal of the tumor.
Park, Eun Hae; Lee, Young Han; Hahn, Seok; Kim, Sang kyum; Suh, Jin Suck [Yonsei University College of Medicine, Seoul (Korea, Republic of)
Epithelioid sarcoma is an uncommon, slow-growing soft tissue tumor that usually arises in the distal part of an upper extremity, predominantly occurring in young adults. Classic-type epithelioid sarcomas appear to be less aggressive and have a better prognosis. In magnetic resonance imaging, epithelioid sarcoma usually appears as a subcutaneous mass or ulcer with cutaneous erosion. Here, we report a classic-type epithelioid sarcoma in a 68-year-old male without an apparent mass, presenting with only numerous small disseminated droplet-like ring-enhancing nodules mimicking abscesses, and multiple metastases.
Hoekstra, Harald Joan
For more than 50 years, the technique of extremity perfusion has been explored in the limb salvage treatment of local, recurrent, and multifocal sarcomas. The "discovery" of tumor necrosis factor-or. in combination with melphalan was a real breakthrough in the treatment of primarily irresectable ext
... and its treatment, see the AIDSinfo website . Nonepidemic Gay-related Kaposi Sarcoma There is a type of ... better than another. Trials are based on past studies and what has been learned ... by their creator. In such cases, it is necessary to contact the writer, artist, ...
... and its treatment, see the AIDSinfo website . Nonepidemic Gay-related Kaposi Sarcoma There is a type of ... better than another. Trials are based on past studies and what has been learned ... by their creator. In such cases, it is necessary to contact the writer, artist, ...
Hoekstra, Harald Joan
For more than 50 years, the technique of extremity perfusion has been explored in the limb salvage treatment of local, recurrent, and multifocal sarcomas. The "discovery" of tumor necrosis factor-or. in combination with melphalan was a real breakthrough in the treatment of primarily irresectable
Svejstrup, Jesper Q
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....
Full Text Available Sarcomas are rare malignant tumors affecting all age groups. They are typically classified according to their resemblance to corresponding normal tissue. Their heterogeneous features, for example in terms of disease-driving genetic aberrations and body location, complicate both disease classification and development of novel treatment regimens. Many years of failure of improved patient outcome in clinical trials has lead to the conclusion that novel targeted therapies are likely needed in combination with current multimodality regimens. Sarcomas have not, in contrast to the common carcinomas, been the subject for larger systematic studies on how tumor behavior relates to characteristics of the tumor microenvironment. There is consequently an urgent need for identifying suitable molecular targets, not only in tumor cells, but also in the tumor microenvironment. This review discusses preclinical and clinical data about potential molecular targets in sarcomas. Studies on targeted therapies involving the tumor microenvironment are prioritized. A greater understanding of the biological context is expected to facilitate more successful design of future clinical trials in sarcoma.
The leukosis/sarcoma (L/S) group of diseases designates a variety of transmissible benign and malignant neoplasms of chickens caused by members that belong to the family Retroviridae. Because the expansion of the literature on this disease, it is no longer feasible to cite all relevant publications ...
Gutowski, Christina J; Basu-Mallick, Atrayee; Abraham, John A
Treatment of bone sarcoma requires careful planning and involvement of an experienced multidisciplinary team. Significant advancements in systemic therapy, radiation, and surgery in recent years have contributed to improved functional and survival outcomes for patients with these difficult tumors, and emerging technologies hold promise for further advancement. Copyright © 2016 Elsevier Inc. All rights reserved.
Full Text Available Objective To investigate clinical manifestation of unspecified functional bowel disorder (UFBD, the features of coexistence with functional gastrointestinal disorder (FGID and its relationship with psychological factors and sleep disturbance in the Chinese Army servicemen. Methods cFGIDs were diagnosed based on the Rome Ⅲ Modular Questionnaire. The subjects were 189 servicemen with UFBD (UFBD group and 372 without FGID (control group. All subjects completed symptom checklist 90 (SCL-90 and Pittsburgh Sleep Quality Index (PSQI questionnaire. Results 'Have to rush to the toilet when having a desire to defecate' was the most frequent symptom of UFBD (93.7%. More than one half of UFBD patients had the symptom 'a feeling of incomplete emptying as bowel movements' or 'straining during bowel movements'. Twenty-eight percent of UFBD subjects had combined FGID (namely cFGID. Among them, the most frequent was proctalgia fugax (7.9%, followed by cyclic vomiting syndrome (6.3%, functional fecal incontinence (6.3%, functional dyspepsia (4.8% and belching (4.8%. The UFBD group scored significantly higher than the control group in the global severity index (GSI and in all SCL-90 subscales (P0.05. Conclusion Pathogenesis of UFBD may be closely correlated with psychiatric and psychological factors and sleep disturbance. cFGID are associated with an increased severity of psychopathological features. DOI: 10.11855/j.issn.0577-7402.2017.01.15
... Stage Soft Tissue Sarcoma Treating Soft Tissue Sarcomas Radiation Therapy for Soft Tissue Sarcomas Radiation therapy uses ... spread. This is called palliative treatment . Types of radiation therapy External beam radiation therapy: For this treatment, ...
Yu, Ri-Sheng; Chen, Ying; Jiang, Biao; Wang, Liu-Hong [Zhejiang University School of Medicine, Department of Radiology, Hangzhou (China); Xu, Xiu-Fang [Zhejiang Medical College, Teaching and Research Group of Radiology, Hangzhou (China)
Primary hepatic sarcomas are rare tumors that are difficult to diagnose clinically. Different primary hepatic sarcomas may have different clinical, morphologic, and radiological features. In this pictorial review, we summarized computed tomography (CT) findings of some relatively common types of hepatic sarcomas, including angiosarcoma, epithelioid hemangioendothelioma (EHE), liposarcoma, undifferentiated embryonal sarcoma (UES), leiomyosarcoma, malignant fibrous histiocytoma (MFH), and carcinosarcoma (including cystadenocarcinosarcoma). To our knowledge, hepatic cystadenocarcinosarcoma has not been described in the English literature. The CT findings in our case are similar to that of cystadenocarcinoma, a huge, multilocular cystic mass with a large mural nodule and solid portion. The advent of CT has allowed earlier detection of primary hepatic sarcomas as well as more accurate diagnosis and characterization. In addition, we briefly discuss the MRI findings and diagnostic value of primary hepatic sarcomas. (orig.)
Gebhardt, M C
There has been a virtual explosion of information relating to the biology of sarcomas with which we as orthopaedists deal. Much more is yet to be learned. These findings will teach us more about the etiology of these tumors. More important, the findings will alter the way in which these tumors are treated. It is unlikely that we will continue to treat osteosarcoma or Ewing's sarcoma patients with currently available drug regimens and surgery or make treatment decisions based on the histologic classification of tumors we know today. If we are to remain active in the management of these patients we must be aware of the findings as they occur. That will ensure both that we remain the primary caretakers of these patients, and that we will continue to be stimulated intellectually by these intriguing scientific investigations.
Casanova, Michela; Basso, Eleonora; Magni, Chiara; Bergamaschi, Luca; Chiaravalli, Stefano; Carta, Roberto; Tirtei, Elisa; Massimino, Maura; Fagioli, Franca; Ferrari, Andrea
Pazopanib is an oral multikinase inhibitor that has proved effective in adults treated for relapsing soft tissue sarcoma and synovial sarcoma in particular. Two cases are reported here of pediatric patients with pretreated relapsing synovial sarcoma whose tumors showed a prolonged response to pazopanib given on compassionate grounds. These results suggest that new agents found effective in adult patients might achieve similar results in adolescents with the same disease. Facilitating the availability of new drugs for children and adolescents is a major challenge for pediatric oncologists.
... tumor that forms from a certain kind of cell in bone or soft tissue . Ewing sarcoma may be found in the bones of the legs, arms, feet, hands, chest , pelvis , spine , or skull . Ewing sarcoma also may be found in the soft tissue of the trunk, arms, legs, head and neck, abdominal cavity , or ...
... tumor that forms from a certain kind of cell in bone or soft tissue . Ewing sarcoma may be found in the bones of the legs, arms, feet, hands, chest , pelvis , spine , or skull . Ewing sarcoma also may be found in the soft tissue of the trunk, arms, legs, head and neck, abdominal cavity , or ...
Berrington de Gonzalez Amy
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 (
Askin Tumor; Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
Full Text Available Synovial sarcoma (SS is a soft tissue, generally deep seated neoplasms that occurs generally in the proximity of large joints. We report of a case of a 33-year-old man who was diagnosed with primary SS of the kidney which is an extremely rare tumor that accounts for less than 2% of malignant renal tumors. Contemporary management of renal synovial sarcoma includes surgical resection and ifosfamide-based chemotherapy and they remain the mainstay of therapy of synovial sarcoma, which is often applied, combined as part of an aggressive treatment approach. Fewer than 50 patients have been described in the English literature. Physicians should be aware of the possibility of malignancy in cystic renal masses and raise the suspicion of synovial sarcoma, especially when patients with renal masses are young adults. Along with the case report a literature review on primary synovial sarcomas of the kidney is provided with focus on the renal tumors’ differential diagnosis.
Jordan M. Blum
Full Text Available Rhabdomyosarcoma (RMS is the most common soft tissue sarcoma in children, whereas undifferentiated pleomorphic sarcoma (UPS is one of the most common soft tissue sarcomas diagnosed in adults. To investigate the myogenic cell(s of origin of these sarcomas, we used Pax7-CreER and MyoD-CreER mice to transform Pax7+ and MyoD+ myogenic progenitors by expressing oncogenic KrasG12D and deleting Trp53 in vivo. Pax7-CreER mice developed RMS and UPS, whereas MyoD-CreER mice developed UPS. Using gene set enrichment analysis, RMS and UPS each clustered specifically within their human counterparts. These results suggest that RMS and UPS have distinct and overlapping cells of origin within the muscle lineage. Taking them together, we have established mouse models of soft tissue sarcoma from muscle stem and progenitor cells.
Deme, Dániel; Abdulfatah, Bishr; Telekes, András
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
Full Text Available Epithelioid sarcoma is a soft tissue sarcoma of children and young adults for which the preferred treatment for localised disease is wide surgical resection. Medical management is to a great extent undefined, and therefore for patients with regional and distal metastases, the development of targeted therapies is greatly desired. In this review we will summarize clinically-relevant biomarkers (e.g., SMARCB1, CA125, dysadherin and others with respect to targeted therapeutic opportunities. We will also examine the role of EGFR, mTOR and polykinase inhibitors (e.g., sunitinib in the management of local and disseminated disease. Towards building a consortium of pharmaceutical, academic and non-profit collaborators, we will discuss the state of resources for investigating epithelioid sarcoma with respect to cell line resources, tissue banks, and registries so that a roadmap can be developed towards effective biology-driven therapies.
Ohshima, Koichi; Karube, Kennosuke; Kawano, Riko; Tsuchiya, Takeshi; Suefuji, Hiroaki; Yamaguchi, Takahiro; Suzumiya, Junji; Kikuchii, Masahiro
WHO classification for malignant lymphoma was recently proposed. However, PTCL is heterogeneous. Chemokines and its receptors are closely associated with the T-cell subtypes. To clarify the T-cell subtype in PTCL, we conducted DNA chips of chemokine, its receptor (R) and cytokines. Angioimmunoblastic T-cell lymphoma (AILD, n=4), anaplastic large cell lymphoma (ALCL, n=4), adult T-cell leukemia lymphoma (ATLL, n=7), NK-cell lymphoma (NKL, n=2) and PTCL, unspecified (PTCL-U, n=6) were analyzed using DNA chips. In addition, immunological stainings were performed in 280 cases. In DNA chip, AILD, ALCL, NKL and ATLL showed a tendency for respective clusters, otherwise, PTCL-U clustered with AILD, ALCL and ATLL. From the gene expression profiling, CCR4, CCR3, MIG, CXCR3 and BLC were selected for immunohistochemistry. ATLL (n=48) expressed CCR4. ALCL (n=26) expressed CCR3, NKL (n=20) expressed MIG, and AILD (n=29) expressed CXCR3 and/or BLC. From the expression patterns, PTCL-U (n=134) were classified into three groups; CCR4 type (CCR4(+), n=42), CCR3 type (CCR3(+), n=31) and CXCR3 type (CXCR3(+) BLC(+/-), n=54). The prognosis was poor for ATLL, intermediate for AILD and favorable for ALCL (P=0.0014). Among PTCL-U, CCR4 type, CXCR3 type and CCR3 type had prognoses equivalent to ATLL, AILD and ALCL, respectively (P<0.0001).
Thomas, M Kate; Murray, Regan; Flockhart, Logan; Pintar, Katarina; Fazil, Aamir; Nesbitt, Andrea; Marshall, Barbara; Tataryn, Joanne; Pollari, Frank
Foodborne illness estimates help to set food safety priorities and create public health policies. The Public Health Agency of Canada estimates that 4 million episodes of foodborne illness occur each year in Canada due to 30 known pathogens and unspecified agents. The main objective of this study was to estimate the number of domestically acquired foodborne illness-related hospitalizations and deaths. Using the estimates of foodborne illness for Canada along with data from the Canadian Hospitalization Morbidity Database (for years 2000-2010) and relevant international literature, the number of hospitalizations and deaths for 30 pathogens and unspecified agents were calculated. Analysis accounted for under-reporting and underdiagnosis. Estimates of the proportion foodborne and the proportion travel-related were incorporated for each pathogen. Monte Carlo simulations were performed to account for uncertainty generating mean estimates and 90% probability intervals. It is estimated that each year there are 4000 hospitalizations (range 3200-4800) and 105 (range 75-139) deaths associated with domestically acquired foodborne illness related to 30 known pathogens and 7600 (range 5900-9650) hospitalizations and 133 (range 77-192) deaths associated with unspecified agents, for a total estimate of 11,600 (range 9250-14,150) hospitalizations and 238 (range 155-323) deaths associated with domestically acquired foodborne illness in Canada. Key pathogens associated with these hospitalizations or deaths include norovirus, nontyphoidal Salmonella spp., Campylobacter spp., VTEC O157 and Listeria monocytogenes. This is the first time Canada has established pathogen-specific estimates of domestically acquired foodborne illness-related hospitalizations and deaths. This information illustrates the substantial burden of foodborne illness in Canada.
Park, Eung Chun; Kim, Young Il; Choi, Won Jae; Kim, Young Jin [Dept. of Dentomaxillofacial Radiology, College of Dentistry, Chosun University, Kwangju (Korea, Republic of)
The author observed a case of osteogenic sarcoma in a 11-year-old female with complaint of painful swelling on face in right side. The observed results were as follows: 1. Large hematoma was observed, and patient complained painfull swelling on c/c site. 2. Predisposing factor of osteogenic sarcoma was not clear, but patient had history of extraction before patient visiting infirmary of our dental collage. 3. Serologic findings were not specific, and serum aldaline level was normal. 4. Radiographic findings were as follows: a. Diffuse faint radiopacit in the lesion. b. Bony destruction and increased radiopacity in right antrum. c. Displacement of multiple teeth on involved area (i. e no 12, 15, 55, 16, 17, 18) d. Increased periodontal space in single tooth (no 13) e. Destruction of bony crypt on involved teeth (no 13, 14, 15, 17, 18) f. Loss of lamina dura of three teeth in involved area (no 11, 12, 16) 5. Computed tomographic findings were as follows: a. Large calcific and heterogenous component mass in the Rt. maxillary sinus, and this mass extending to Rt. maxilla, alveolar bone, ethmoid sinus. b. Soft tissue bulging in to Rt. side nasal cavity and oral cavity. c. Bone destruction of maxillary sinus wall and Rt. alveolar bone.
... What Should You Ask Your Doctor About Kaposi Sarcoma? Kaposi Sarcoma Early Detection, Diagnosis, and Staging What Should You Ask Your Doctor About Kaposi Sarcoma? As you cope with Kaposi sarcoma (KS) and ...
Theron, Salomine [University of Stellenbosch, Department of Radiology, Tygerberg Academic Hospital, Cape Town (South Africa); University of Stellenbosch, Department of Radiology, Tygerberg Hospital, Cape Town (South Africa); Andronikou, Savvas; Plessis, Jaco du; George, Reena; Mapukata, Ayanda; Grobbelaar, Marie; Hayes, Murray [University of Stellenbosch, Department of Radiology, Tygerberg Academic Hospital, Cape Town (South Africa); Goussard, Pierre [University of Stellenbosch, Department of Child Health, Tygerberg Academic Hospital, Cape Town (South Africa); Wieselthaler, Nicky [University of Cape Town, Department of Radiology, Red Cross Children' s Hospital, Cape Town (South Africa); Davidson, Alan [University of Cape Town, Department of Oncology, Red Cross Children' s Hospital, Cape Town (South Africa)
Pulmonary involvement in Kaposi sarcoma is rare in children and can be difficult to distinguish from other pathology. To describe the radiological findings in paediatric pulmonary Kaposi sarcoma. Sequential chest radiographs of six children and CT scans of four of these children were evaluated retrospectively. Their ages ranged from 18 months to 10 years; four were male and two were female. All six children were HIV-positive. The observers were two radiologists. Chest radiographs revealed air-space (100%) and reticular (83%) opacification in the mid- and lower lung zones; pleural effusions were present in 83% of the children. All the children showed progressive air-space opacification on follow-up radiography. CT demonstrated bilateral air-space opacification in a perihilar distribution in all the children; reticular opacification was seen in 75%. All the children had mediastinal and axillary lymphadenopathy; 75% had bilateral hilar lymphadenopathy. In both adults and children, chest radiography demonstrates perihilar and lower zone involvement. Pleural effusions are more common on radiographs in children. Air-space disease and lymphadenopathy are much more common on CT in children than adults. (orig.)
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.
Thomas, M Kate; Murray, Regan; Flockhart, Logan; Pintar, Katarina; Pollari, Frank; Fazil, Aamir; Nesbitt, Andrea; Marshall, Barbara
Estimates of foodborne illness are important for setting food safety priorities and making public health policies. The objective of this analysis is to estimate domestically acquired, foodborne illness in Canada, while identifying data gaps and areas for further research. Estimates of illness due to 30 pathogens and unspecified agents were based on data from the 2000-2010 time period from Canadian surveillance systems, relevant international literature, and the Canadian census population for 2006. The modeling approach required accounting for under-reporting and underdiagnosis and to estimate the proportion of illness domestically acquired and through foodborne transmission. To account for uncertainty, Monte Carlo simulations were performed to generate a mean estimate and 90% credible interval. It is estimated that each year there are 1.6 million (1.2-2.0 million) and 2.4 million (1.8-3.0 million) episodes of domestically acquired foodborne illness related to 30 known pathogens and unspecified agents, respectively, for a total estimate of 4.0 million (3.1-5.0 million) episodes of domestically acquired foodborne illness in Canada. Norovirus, Clostridium perfringens, Campylobacter spp., and nontyphoidal Salmonella spp. are the leading pathogens and account for approximately 90% of the pathogen-specific total. Approximately one in eight Canadians experience an episode of domestically acquired foodborne illness each year in Canada. These estimates cannot be compared with prior crude estimates in Canada to assess illness trends as different methodologies were used.
Qian, Xiaoxiao; Gigantelli, James W; Abromowitch, Minnie; Morgan, Linda A; Suh, Donny W
The authors describe a case of myeloid sarcoma of the orbit in a pediatric patient. An 8-month-old male infant presented to the ophthalmology clinic with a left orbital mass, which had been increasing in size over the previous 2 months. The mass was initially diagnosed at another clinic as an infantile hemangioma, and had been treated with a topical formulation of timolol. In the ophthalmology clinic, orbital magnetic resonance imaging showed a solid enhancing mass. A biopsy was performed, and histopathology revealed myeloid sarcoma. The disease responded well to a standard chemotherapy regimen. Myeloid sarcoma is a rare, extra-medullary presentation that can occur as an isolated tumor, concurrently with or at relapse of acute myeloid leukemia. Because few cases of myeloid sarcoma in the orbit have been reported, this case report aids in the management of myeloid sarcoma in pediatric patients. The report describes an 8-month-old male infant, the youngest patient to develop myeloid sarcoma without preexisting acute myeloid leukemia. [J Pediatr Ophthalmol Strabismus. 2016;53:e64-e68.].
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.
... 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 ...
... type of cancer or a benign disease. Several types of biopsies are used to diagnose sarcomas. Doctors experienced with ... But if FNA results suggest a sarcoma, another type of biopsy will usually be done to remove enough tissue ...
Lukas M. Nystrom
Full Text Available Soft tissue sarcoma is a rare malignancy, with approximately 11,000 cases per year encountered in the United States. It is primarily encountered in adults but can affect patients of any age. There are many histologic subtypes and the malignancy can be low or high grade. Appropriate staging work up includes a physical exam, advanced imaging, and a carefully planned biopsy. This information is then used to guide the discussion of definitive treatment of the tumor which typically involves surgical resection with a negative margin in addition to neoadjuvant or adjuvant external beam radiation. Advances in imaging and radiation therapy have made limb salvage surgery the standard of care, with local control rates greater than 90% in most modern series. Currently, the role of chemotherapy is not well defined and this treatment is typically reserved for patients with metastatic or recurrent disease and for certain histologic subtypes. The goal of this paper is to review the current state of the art in multidisciplinary management of soft tissue sarcoma.
Mona S. Jahromi
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.
Carrascosa, R; Alfageme, F; Roustán, G; Suarez, M D
The use of ultrasound imaging has recently been increasing in numerous dermatologic diseases. This noninvasive technique provides additional details on the structure and vascularization of skin lesions. Kaposi sarcoma is a vascular tumor that typically arises in the skin and mucosas. It can spread to lymph nodes and internal organs. We performed B-mode and color Doppler ultrasound studies in 3 patients with a clinical diagnosis of Kaposi sarcoma confirmed by histological examination. We found differences in the ultrasound pattern between nodular and plaque lesions, in both B-mode and color Doppler. We believe that skin ultrasound imaging could be a useful technique for studying cutaneous Kaposi sarcoma, providing additional information on the structural and vascular characteristics of the lesion.
Thomas, David M; Wilhelm, Miriam; Cleton-Jansen, Anne-Marie; Dirksen, Uta; Entz-Werlé, Natacha; Gelderblom, Hans; Hassan, Bass; Jürgens, Heribert; Koster, Jan; Kovar, Heinrich; Lankester, Arjan C; Lewis, Ian J; Myklebost, Ola; Nathrath, Michaela H M; Picci, Piero; Whelan, Jeremy S; Hogendoorn, Pancras C W; Bielack, Stefan S
A key workshop was held in The Netherlands in June 2011, hosted by several European bone sarcoma networks and with a broad range of stakeholders from Europe and Australia. The purpose of the meeting was to identify the strengths and weaknesses in current clinical trials for bone sarcomas and to make recommendations as to how to accelerate progress in this field. Two areas of particular interest were discussed. First, all participants agreed upon the importance of tumor biology to understanding clinical responses for all types of bone sarcoma. Various barriers to biobanking tumor and germline specimens were canvassed and are outlined in this paper. Second, there was consideration of the particular challenges of dealing with adolescent and young adult cancers, exemplified by bone sarcomas. Participants recommended greater engagement of both pediatric and adult sarcoma trial organizations to address this issue. Specific opportunities were identified to develop biological sub-studies within osteosarcoma, focused on understanding germ line risk and pharmacogenomics defining toxicity and biological responses. In Ewing sarcoma, it was harder to define opportunities for biological insights. There was agreement that the results for insulin-like growth factor pathway inhibition in Ewing family tumors were disappointing, but represented a clear indication of the need for companion biologic studies to develop predictive biomarkers. The meeting ended with broad commitment to working together to make progress in this rare but important subgroup of cancers.
Wedde, T B; Lobmaier, I V K; Brennhovd, B; Lohne, F; Hall, K S
Objective. Ewing's sarcoma of the kidney is rare and is usually found in young adults. We present here a single case study of Ewing's sarcoma found in an elderly man. Material and methods. A 73-year-old man underwent routine surgery for hydrocoele of the testis. He developed urinary obstruction symptoms, and radiological examinations revealed a tumour in the right kidney. Results. Microscopical, immunohistochemical, and molecular pathological analysis of the tumour was consistent with Ewing's sarcoma. FISH showed rearrangement of chromosomes 22q12 (EWSR1). The patient subsequently underwent nephrectomy followed by 6 adjuvant chemotherapy cycles. Follow-up after 7 months shows no recurrence. Conclusion. This case report presents not only the rare finding of Ewing's sarcoma in the kidney, but also the occurrence of this tumour entity in an elderly patient. Treatment options for the different types of renal tumours are vastly different and the need for a correct diagnosis is, therefore, vital.
T. B. Wedde
Full Text Available Objective. Ewing’s sarcoma of the kidney is rare and is usually found in young adults. We present here a single case study of Ewing's sarcoma found in an elderly man. Material and methods. A 73-year-old man underwent routine surgery for hydrocoele of the testis. He developed urinary obstruction symptoms, and radiological examinations revealed a tumour in the right kidney. Results. Microscopical, immunohistochemical, and molecular pathological analysis of the tumour was consistent with Ewing's sarcoma. FISH showed rearrangement of chromosomes 22q12 (EWSR1. The patient subsequently underwent nephrectomy followed by 6 adjuvant chemotherapy cycles. Follow-up after 7 months shows no recurrence. Conclusion. This case report presents not only the rare finding of Ewing's sarcoma in the kidney, but also the occurrence of this tumour entity in an elderly patient. Treatment options for the different types of renal tumours are vastly different and the need for a correct diagnosis is, therefore, vital.
Weiler, Catherine R; Butterfield, Joseph
Mast cell sarcoma is a disorder that results in abnormal mast cells as identified by morphology, special stains, and in some publications, c-kit mutation analysis. It affects animal species such as canines more commonly than humans. In humans it is a very rare condition, with variable clinical presentation. There is no standard therapy for the disorder. It can affect any age group. It is occasionally associated with systemic mastocytosis and/or urticaria pigmentosa. The prognosis of mast cell sarcoma in published literature is very poor in humans.
Sampson, Valerie B; Kamara, Davida F; Kolb, E Anders
Introduction There are > 75 histological types of solid tumors that are classified into two major groups: bone and soft-tissue sarcomas. These diseases are more prevalent in children, and pediatric sarcomas tend to be highly aggressive and rapidly progressive. Sarcomas in adults may follow a more indolent course, but aggressive tumors are also common. Sarcomas that are metastatic at diagnosis, or recurrent following therapy, remain refractory to current treatment options with dismal overall survival rates. A major focus of clinical trials, for patients with sarcoma, is to identify novel and more effective therapeutic strategies targeted to genomic or proteomic aberrations specific to the malignant cells. Critical to the understanding of the potential for targeted therapies are models of disease that are representative of clinical disease and predictive of relevant clinical responses. Areas covered In this article, the authors discuss the use of mouse xenograft models and genetically engineered mice in cancer drug discovery. The authors provide a special focus on models for the two most common bone sarcomas: osteosarcoma (OS) and Ewing's sarcoma (ES). Expert opinion Predicting whether a new anticancer agent will have a positive therapeutic index in patients with OS and ES remains a challenge. The use of mouse sarcoma models for understanding the mechanisms involved in the response of tumors to new treatments is an important step in the process of drug discovery and the development of clinically relevant therapeutic strategies for these diseases. PMID:23844615
... and its treatment, see the AIDSinfo website . Nonepidemic Gay-related Kaposi Sarcoma There is a type of ... better than another. Trials are based on past studies and what has been learned ... by their creator. In such cases, it is necessary to contact the writer, artist, ...
Ekmekci, T R; Ayabakan, O; Sakiz, D; Koslu, A
Lipoedema is a form of lipodistrophy, which consists of abnormal accumulation of fat in subcutaneous tissue of the lower limbs. It does not cause any disease and it has not been reported association with malignity. We describe a 63-year-old woman occurring of Kaposi sarcoma on the lipoedema base.
Wilson, Sarah E; Deeks, Shelley L; Rosella, Laura C
In Ontario, Canada, we conducted an evaluation of rotavirus (RV) vaccine on hospitalizations and Emergency Department (ED) visitations for acute gastroenteritis (AGE). In our original analysis, any one of the International Classification of Disease, Version 10 (ICD-10) codes was used for outcome ascertainment: RV-specific- (A08.0), viral- (A08.3, A08. 4, A08.5), and unspecified infectious- gastroenteritis (A09). Annual age-specific rates per 10,000 population were calculated. The average monthly rate of AGE hospitalization for children under age two increased from 0.82 per 10,000 from January 2003 to March 2009, to 2.35 over the period of April 2009 to March 31, 2013. Similar trends were found for ED consultations and in other age groups. A rise in events corresponding to the A09 code was found when the outcome definition was disaggregated by ICD-10 code. Documentation obtained from the World Health Organization confirmed that a change in directive for the classification of unspecified gastroenteritis occurred with the release of ICD-10 in April 2009. AGE events previously classified under the code K52.9, are now classified under code A09.9. Based on change in the classification of unspecified gastroenteritis we modified our outcome definition to also include unspecified non-infectious-gastroenteritis (K52.9). We recommend other investigators consider using both A09.9 and K52.9 ICD-10 codes for outcome ascertainment in future rotavirus vaccine impact studies to ensure that all unspecified cases of AGE are captured, especially if the study period spans 2009.
The heat-balance integral method of Goodman has been thoroughly analyzed in the case of a parabolic profile with unspecified exponent depending on the boundary condition imposed. That the classical Good man's boundary conditions defining the time-dependent coefficients of the prescribed temperature profile do not work efficiently at the front of the thermal layers if the specific parabolic profile at issue is employed. Additional constraints based on physical assumption enhance the heat-balance integral method and form a robust algorithm defining the parabola exponent . The method has been compared by results provided by the Veinik's method that is by far different from the Good man's idea but also assume forma tion of thermal layer penetrating the heat body. The method has been demonstrated through detailed solutions of 4 1-D heat-conduction problems in Cartesian co-ordinates including a spherical problem (through change of vari ables) and over-specified boundary condition at the face of the thermal layer.
... changes in the chromosomes . FISH (fluorescence in situ hybridization) : A laboratory test used to look at genes ... a picture of areas inside the body. Blood chemistry studies : A procedure in which a blood sample ...
... changes in the chromosomes . FISH (fluorescence in situ hybridization) : A laboratory test used to look at genes ... a picture of areas inside the body. Blood chemistry studies : A procedure in which a blood sample ...
Kollar, A.; Jones, R.L.; Stacchiotti, S.; Gelderblom, H.; Guida, M.; Grignani, G.; Steeghs, N.; Safwat, A.; Katz, D.; Duffaud, F.; Sleijfer, S.; Graaf, W.T. van der; Touati, N.; Litiere, S.; Marreaud, S.; Gronchi, A.; Kasper, B.
BACKGROUND: Pazopanib is a multitargeted tyrosine kinase inhibitor approved for the treatment of patients with selective subtypes of advanced soft tissue sarcoma (STS) who have previously received standard chemotherapy including anthracyclines. Data on the efficacy in vascular sarcomas are limited.
Cidre-Aranaz, Florencia; Alonso, Javier
Ewing sarcoma is an aggressive bone malignancy that affect children and young adults. Ewing sarcoma is the second most common primary bone malignancy in pediatric patients. Although significant progress has been made in the treatment of Ewing sarcoma since it was first described in the 1920s, in the last decade survival rates have remained unacceptably invariable, thus pointing to the need for new approaches centered in the molecular basis of the disease. Ewing sarcoma driving mutation, EWS–FLI1, which results from a chromosomal translocation, encodes an aberrant transcription factor. Since its first characterization in 1990s, many molecular targets have been described to be regulated by this chimeric transcription factor. Their contribution to orchestrate Ewing sarcoma phenotype has been reported over the last decades. In this work, we will focus on the description of a selection of EWS/FLI1 targets, their functional role, and their potential clinical relevance. We will also discuss their role in other types of cancer as well as the need for further studies to be performed in order to achieve a broader understanding of their particular contribution to Ewing sarcoma development. PMID:26258070
Metastatic Angiosarcoma; Metastatic Epithelioid Sarcoma; Metastatic Fibrosarcoma; Metastatic Leiomyosarcoma; Metastatic Liposarcoma; Metastatic Malignant Peripheral Nerve Sheath Tumor; Metastatic Synovial Sarcoma; Metastatic Undifferentiated Pleomorphic Sarcoma; Myxofibrosarcoma; Pleomorphic Rhabdomyosarcoma; Stage III Soft Tissue Sarcoma; Stage IV Soft Tissue Sarcoma; Undifferentiated (Embryonal) Sarcoma
Uterine sarcomas are rare tumors accounting for 3,4% of all uterine cancers. Even after radical hysterectomy, most patients relapse or present with distant metastases. The very limited clinical benefit of adjuvant cytotoxic treatments is reflected by high mortality rates, emphasizing the need for new treatment strategies. This review summarizes rising potential targets in four distinct subtypes of uterine sarcomas: leiomyosarcoma, low-grade and high-grade endometrial stromal sarcoma, and undi...
Purpose. Relatively few results are available in the literature about the radiation response of unresectable sarcomas in relation to their histology. Therefore, an attempt was made to summarize the present situation. Materials and methods. This report is based on a review of the literature and the author's own experience. Adult-type soft tissue sarcomas, chondrosarcomas, and chordomas were analyzed. Radioresponse was mainly associated with the degree of tumor shrinkage, that is, objective res...
Despite recent advances in our understanding of pathogenic mechanisms involved, the true nature of Kaposi sarcoma remains an enigma. Four clinical variants have been described for the disease, differing in natural history, site of predilection, and prognosis. All forms of Kaposi sarcoma may manifest in the oral cavity and Kaposi sarcoma-associated virus appears essential to development of all clinical variants. The spectrum of therapeutic strategies is broad and selection of appropriate intervention mandates a thorough understanding of disease spread and the patient's symptomatology, as well as risks and benefits of therapy. This article provides an overview of epidemiology, subtypes, clinical course, pathogenesis, and management strategies for Kaposi sarcoma.
Ladra, Matthew M.; Yock, Torunn I., E-mail: email@example.com [Department of Radiation Oncology, Massachusetts General Hospital, Boston, MA 02114 (United States)
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.
Teyssonneau, Diego; Italiano, Antoine
Soft tissue sarcomas (STS) are rare malignant tumors. Unfortunately, the first-line doxorubicin-based treatment has not been improved since the 1970s. Platelet-derived growth factor (PDGF) receptor alpha (PDGFR-α) and its ligands are co-expressed in many types of cancer, including sarcomas. They are involved in stimulating growth and regulating stromal-derived fibroblasts and angiogenesis. PDGFR-α and its ligand may play an important role in tumorigenesis and be a potential target in the treatment of sarcomas. Olaratumab is a fully human IgG1-type anti-PDGFR-α monoclonal antibody with a high affinity and a low 50% inhibitory concentration (IC50). Areas covered: The authors review the role of olaratumab in the treatment of STS by focusing on the recent, randomized Phase II JDGD trial that challenged patients with unresectable or metastatic STS with doxorubicin in the presence or absence of olaratumab. This trial showed a great improvement in overall survival (OS), with an increase in survival from 14.7 months to 26.5 months for patients in the experimental arm and showed acceptable toxicity. Expert opinion: Results seem promising. However, it must be qualified, as the study includes several uncertainties. These uncertainties should be addressed by the ongoing Phase 3 JGDJ confirmatory trial, for which the final efficacy analysis is expected by 2019.
Lombardi, F; Gasparini, M; Gianni, C; Petrillo, R; Tesoro-Tess, J D; Volterrani, F; Musumeci, R
All the pertinent radiographs of 83 patients with histologically proven Ewing's sarcoma were reviewed. Forty-nine patients were in the pediatric age group, and 34 were adults. The mean age, the symptoms and time from symptoms to diagnosis were evaluated in the 2 groups. The site of primary involvement was in 54% the long bones, 35% the flat bones, 8% the small bones and 3% extraosseous. For the primary site we considered the diagnostic results of the standard radiographic investigations and in some cases the usefulness of angiography, xeroradiography and telethermography. At presentation we also evaluated the possible diffusion of the disease with standard radiographic surveys (chest and skeletal, including limbs) and with foot lymphography in selected cases. In this way, 57 patients (69%) were considered to have localized disease. In this group, we also considered the value of the periodic radiographic follow-up, which enabled us to disclose the appearance of metastases (chest 64%, bone 54%, lymph nodes 11%) in 28 cases (49%). Finally, we made a comparison of the different radiologic and epidemiologic findings between children and adults.
Kuiper, DR; Hoekstra, HJ; Veth, RPH; Wobbes, T
Clear cell sarcoma is a rare soft tissue tumour, constituting approximately 1% of all soft tissue sarcomas. Prognosis is reported to be poor due to the great propensity to metastasise regionally and distantly. In this paper, we report the surgical experience of two university hospitals. Both disease
Conclusions: Low-grade endometrial stromal sarcoma typically has an indolent clinical course and favorable prognosis. Surgical resection is the primary therapeutic approach, and adjuvant therapy with radiotherapy, chemotherapy, or progesterone therapy should be considered for the management of residual or recurrent low-grade endometrial stromal sarcomas.
Objective: We enrolled retrospective data to determine the efficacy of combined chemotherapy and surgery for local tumour control and survival in patients with high-risk soft-tissue sarcomas. Methods: We collected data from 25 patients with high-risk soft-tissue sarcomas treated with four cycles of etoposide, ifosfamide and doxorubicin (EIA) followed by definitive surgery with or without postoperative radiotherapy and adjuvant chemotherapy. 21 patients received chemotherapy in a neoadjuvant/adjuvant clinical setting;eighteen of them completed adjuvant chemotherapy. Four patients received chemotherapy in an adjuvant setting only. Results: The objective response rate of neoadjuvant chemotherapy assessable in 21 patients was 43%. Including NED (n=7) and partial remissions (n=3), the radiographic response rate was 47.6% with additional 42.9% stable diseases (n=9). Surgery was performed in two patients before completing four neoadjuvant chemotherapy cycles because of disease progression. Median overall survival for all patients was 21.6+ months. After completion of chemotherapy, in 62% of patients R0-resection could be performed. Conclusion: High proportion of R0-resections supports the idea of tumour down-staging after neoadjuvant treatment. Response to neoadjuvant chemotherapy is predictive for improved local tumour control resulting in long-term survival benefit.
Egwuonwu, Steve; Gatto-Weis, Cara; Miranda, Roberto; Casas, Luis De Las
Kaposi sarcoma is a vascular tumor manifesting as nodular lesions on skin, mucous membranes, or internal organs. This is a case of a 42-year-old human immunodeficiency virus- (HIV) positive bisexual male, not on highly active antiretroviral therapy (HAART) since diagnosis four years ago. He presented with a three-day history of abdominal pains, fever, vomiting, and a one-week history of melena stools. Endoscopy revealed Kaposi sarcoma in the stomach and duodenum. Postendoscopy, he developed acute abdomen. Exploratory laparotomy revealed extensive Kaposi sarcoma of the gastrointestinal tract with appendiceal involvement. The patient underwent appendectomy and had an uneventful recovery. A review of the literature discusses appendiceal Kaposi sarcoma with appendicitis, a rare but critical manifestation of gastrointestinal Kaposi sarcoma.
Seinen, Jojanneke; Almquist, Martin; Styring, Emelie
at the general practitioner, 36 days at local hospitals, and 55 days at the sarcoma centre. Conclusion. Centralization per se is not sufficient for optimized and efficient management. Our findings suggest that delays can be minimized by direct referral of patients from primary health care to sarcoma centers...... sarcoma in the southern Sweden health care region 2003-2009 were eligible for the study. Data on referrals and diagnostic investigations were collected from clinical files from primary health care, local hospitals, and from the sarcoma centre. Lead times were divided into patient delays and health care...... delays caused by primary health care, local hospitals, or procedures at the sarcoma centre. Results. Complete data were available from 33 patients and demonstrated a median patient delay of 23 days (0-17 months) and median health care delay of 94 days (1-40 months) with delays of median 15 days...
Seinen, Jojanneke; Almquist, Martin; Styring, Emelie
at the general practitioner, 36¿days at local hospitals, and 55¿days at the sarcoma centre. Conclusion. Centralization per se is not sufficient for optimized and efficient management. Our findings suggest that delays can be minimized by direct referral of patients from primary health care to sarcoma centers...... sarcoma in the southern Sweden health care region 2003-2009 were eligible for the study. Data on referrals and diagnostic investigations were collected from clinical files from primary health care, local hospitals, and from the sarcoma centre. Lead times were divided into patient delays and health care...... delays caused by primary health care, local hospitals, or procedures at the sarcoma centre. Results. Complete data were available from 33 patients and demonstrated a median patient delay of 23¿days (0-17¿months) and median health care delay of 94¿days (1-40¿months) with delays of median 15¿days...
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)
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.
Desmoplastic Small Round Cell Tumor; Ewing Sarcoma of Bone or Soft Tissue; Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor
Sareen, Pooja; Chhabra, Lovely; Trivedi, Nitin
Sellar tumours in adults are most commonly pituitary adenomas. Primary spindle cell sarcoma of the sella turcica without a prior history of cranial radiation is extremely rare. We report a case of a large sellar mass with suprasellar and cavernous sinus extension in a geriatric male patient who presented with complete left oculomotor nerve palsy and panhypopituitarism. The patient underwent partial resection of the sellar mass through transcranial route. The pathology of the mass revealed a poorly differentiated spindle cell neoplasm most consistent with a sarcoma. Postoperatively, the size of the residual sellar mass decreased significantly following six cycles of external beam radiation in conjunction with temozolomide.
A. P. Polyakov
Full Text Available Sarcomas in the head and neck are rare, and account for approximately 5-15% of all sarcomas in adults, is less than 1% of all neoplasms of the head and neck. Due to anatomical and physiological features of the structure of the nasopharynx, surgical treatment of tumors of this localization is associated with a number of technical difficulties, the high risk of non-radical removal of the tumor, it was therefore necessary for adequate surgical access. The article describes video-assisted access for removal of tumors of the nasopharynx with good visualization of the tumor process simultaneously after laryngectomy with the formation of pharyngotomy, providing the opportunity to monitor the postoperative area of the nasopharynx and early detection of continued tumor growth in patients with soft tissue sarcoma of the nasopharynx and combined lesions of the larynx.
Celli, Romulo; Cai, Guoping
Ewing sarcoma/primitive neuroectodermal tumor represents a spectrum of undifferentiated tumors with similar biology that together represent the second most common sarcoma in the pediatric-young adult age range. Very rarely, this tumor presents as a primary neoplasm of the kidney. The clinical presentation of this tumor is not specific, and other renal tumors may present with a similar histologic appearance. Establishing the correct diagnosis is critical because renal Ewing sarcoma/primitive neuroectodermal tumor carries a strikingly dismal prognosis and thus dictates a specific treatment strategy. A low threshold for the use of ancillary molecular tests is recommended, particularly in diagnostically problematic cases. Important considerations with regards to morphology, immunohistochemistry, and molecular alterations will be reviewed here and should be taken into account before rendering this rare and lethal diagnosis.
Sharma, R R; Netalkar, A; Lad, S D
Primary Ewing's sarcoma is an uncommon lethal tumour of the long bones and pelvic girdle mainly affecting children and young adults. An origin in the cranial bones is extremely rare. We report a unique case of primary involvement of the greater wing of sphenoid bone in a 16-year-old patient. Aggressive management using microsurgical resection, radiotherapy and chemotherapy was curative. Localized, primary Ewing's sarcoma of the cranial bones should be considered as a distinct clinicopathological entity with an extremely low rate of dural penetration and metastases, and with a relatively better prognosis as compared with those of long bones and pelvic girdle. In neurosurgical practice, primary Ewing's sarcoma of the cranial bones requires early aggressive management to achieve adequate long-term prognosis and cure.
Akang Effiong E
Full Text Available Abstract Background Primary extra-osseous osteogenic sarcomas have been reported in many tissues of the body but their occurrence in the breast is extremely rare. It can arise as a result of osseous metaplasia in a pre-existing benign or malignant neoplasm of the breast or as non-phylloides sarcoma from the soft tissue of a previously normal breast. Case presentation A 40 year-old Nigerian woman was clinically diagnosed to have carcinoma of the left breast. The histology report of core-needle biopsy of the mass showed a malignant neoplasm comprising islands of chondroblastic and osteoblastic stromal cells. This report changed the diagnosis from carcinoma to osteogenic sarcoma of the breast. She had a left modified radical mastectomy, however there was significant post surgery skin deficit. A latissimus dorsi musculocutaneous flap was used to cover the anterior chest wall defect. Sections from the mastectomy specimen confirmed the diagnosis of osteogenic sarcoma. She died six months after mastectomy. Conclusion A diagnosis of osteogenic sarcoma of the breast was made based on histology report and after excluding an osteogenic sarcoma arising from underlying ribs and sternum. This is the second documented case of primary osteogenic sarcoma of the breast coming from Nigeria
VanDervort, Darcy R; López, Dina L; Orantes, Carlos M; Rodríguez, David S
Chronic kidney disease of unknown etiology is occurring in various geographic areas worldwide. Cases lack typical risk factors associated with chronic kidney disease, such as diabetes and hypertension. It is epidemic in El Salvador, Central America, where it is diagnosed with increasing frequency in young, otherwise-healthy male farmworkers. Suspected causes include agrochemical use (especially in sugarcane fields), physical heat stress, and heavy metal exposure. To evaluate the geographic relationship between unspecified chronic kidney disease (unCKD) and nondiabetic chronic renal failure (ndESRD) hospital admissions in El Salvador with the proximity to cultivated crops and ambient temperatures. Data on unCKD and ndESRD were compared with environmental variables, crop area cultivated (indicator of agrochemical use) and high ambient temperatures. Using geographically weighted regression analysis, two model sets were created using reported municipal hospital admission rates are per thousand population for unCKD 2006-2010 and rates of ndESRD 2005-2010 [corrected]. These were assessed against local percent of land cultivated by crop (sugarcane, coffee, corn, cotton, sorghum, and beans) and mean maximum ambient temperature, with Moran's indices determining data clustering. Two-dimensional geographic models illustrated parameter spatial distribution. Bivariate geographically weighted regressions showed statistically significant correlations between percent area of sugarcane, corn, cotton, coffee, and bean cultivation, as well as mean maximum ambient temperature with both unCKD and ndESRD hospital admission rates. Percent area of sugarcane cultivation had greatest statistical weight (p ≤ 0.001; Rp2 = 0.77 for unCKD). The most statistically significant multivariate geographically weighted regression model for unCKD included percent area of sugarcane, cotton and corn cultivation (p ≤ 0.001; Rp2 = 0.80), while, for ndESRD, it included the percent area of sugarcane, corn
Full Text Available Kaposi′s sarcoma is described as cutaneous and extracutaneous neoplasm predominantly affecting older individuals. Though earlier uncommon and endemic to certain African areas, its incidence is on a rise due to infections with human immunodeficiency virus and also due to transplant-associated immunosuppression. Further, certain benign conditions like Pseudo Kaposi′s sarcoma, certain infective conditions like bacillary angiomatosis of acquired immunodeficiency syndrome can mimic Kaposi′s sarcoma both clinically and histologically leading to a diagnostic dilemma. We report such a case here.
Tamarit Conejeros, José Manuel; Estrems Navas, Paloma; Estellés Ferriol, Enrique; Dalmau Galofre, José
Synovial sarcoma is the fourth most common type of sarcoma. It is usually found in the knee or ankle joints, and is exceptional in the head and neck. Most cases are diagnosed in men between 20 and 40 years of age. Diagnosis is often casual due to the infrequent nature of this tumour and its non-specific clinical and radiological characteristics. Confirmation is therefore based on immunohistochemistry and electron microscopy techniques. We report a case of biphasic sinovial sarcoma located in the infratemporal fossa treated at our hospital and we make a review of the literature. Copyright © 2009 Elsevier España, S.L. All rights reserved.
Hernán Augusto Centurión Sobral; Enzo Martins Taglietti; Elisângela Plazaz Monteiro; Marília Resende Von Sonnleithner Gama; Sérgio Henrique Couto Horta; Galdino José Sitonio Formiga
Os sarcomas são neoplasias que se originam das células mesenquimais primitivas, sendo raros na região anorretal. O objetivo é relatar um caso de sarcoma sinovial anorretal, neoplasia extremamente rara nesta localização. É descrito o caso de uma paciente de 77 anos que apresentava nodulação anal dolorosa e sangrante às evacuações, associada a puxo, tenesmo e perda ponderal. A lesão foi biopsiada e o estudo imunohistoquímico evidenciou sarcoma sinovial anorretal. A paciente foi submetida a ampu...
Torriani, Martin; Ouellette, Hugue [Massachusetts General Hospital and Harvard Medical School, Boston, MA (United States). Dept. of Radiology. Div. of Musculoskeletal Radiology; Etchebehere, Mauricio [Universidade Estadual de Campinas, SP (Brazil). Faculdade de Ciencias Medicas. Dept. de Ortopedia; Amstalden, Eliane M.I. [Universidade Estadual de Campinas, SP (Brazil). Faculdade de Ciencias Medicas. Dept. de Patologia
Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft-tissue tumor with a deceptively benign histologic appearance affecting predominantly young adults during the fourth decade of life. (MR) imaging features of a surgically confirmed case of LGFMS, affecting the shoulder is presented. A 30-year-old man presented with a 20-year history of a painless slow-growing mass in the right shoulder. Magnetic resonance images were obtained on a 2.0T scanner (Elscint, Haifa, Israel), demonstrating a well-defined soft tissue mass measuring 12.0 x 7.0 x 9.0 cm located between the deltoid muscle, rotator-cuff muscles, and proximal humerus. The differential diagnosis of LGFMS includes several benign and malignant neoplasms containing variable amounts of myxoid and fibrous tissue. Histologically, the most important differential diagnosis is with myxofibrosarcoma. Low-grade fibromyxoid sarcoma is a rare soft tissue tumor with slow growth and deceptively benign histologic appearance. The possibility of LGFMS must be considered when elaborating differential diagnostic possibilities for young adults with a large soft tissue mass exhibiting MR imaging characteristics of intermixed fibrous and myxoid tissue.
Kirova, Y.M.; Belembaogo, E.; Frikha, H.; Yu, S.J.; Le Bourgeois, J.P. [Hopital Henri-Mondor, 94 - Creteil (France)
Moriz Kaposi was the first who, in 1872, described five patients presenting with `sarcoma idiopathicum multiple hemorrhagicum`. In 1912 Sternberg termed this disease Kaposi`s sarcoma. Since then various forms of this rare disease have been observed. In 1914 Hallenberg described the first cases of African or endemic Kaposi`s sarcoma. In the 1960`s the first reports discussing Kaposi`s sarcoma following organ transplantation and immunosuppressive therapy were published. After 1981, the epidemic form associated with the acquired immunodeficiency syndrome (AIDS) was described. All these forms, their history, treatment methods and the role of radiation therapy in the management of this rare malignancy are discussed, and the literature is reviewed. (authors)
Joseph P. Kriz
Full Text Available Pulmonary artery intimal sarcomas are rare and lethal malignant tumors that typically affect larger vessels: the aorta, inferior vena cava, and pulmonary arteries. Since symptoms and imaging of pulmonary arterial intimal sarcomas mimic pulmonary thromboembolism, the differential diagnosis of a patient presenting with chest pain, dyspnea, and filling defect within the pulmonary arteries should include intimal sarcoma. Often right ventricular failure is observed due to pulmonary hypertension caused by the obstructive effect of the tumor and concomitant chronic thromboembolism. We report the case of a 72-year-old African-American male with arterial intimal sarcoma of the left and right pulmonary artery with extension through the right artery into the bronchus and right lung.
... Non-Hodgkin) Lymphoma (Hodgkin) Neuroblastoma Osteosarcoma Retinoblastoma Rhabdomyosarcoma Skin Cancer Soft Tissue Sarcoma Thyroid Cancer Cancer Resources Childhood Cancer Statistics Coping With Cancer CureSearch CancerCare App Late Effects ...
Full Text Available INTRODUCTION Sarcomas are quite rare with only 15,000 new cases per year in the United States. Sarcomas therefore represent about one percent of the 1.5 million new cancer diagnoses in that country each year. Sarcoma can be defined as cancer whose cells originate from the cells of mesenchymal origin. The bones, cartilages, muscles are a few examples to be mentioned. This is in contrast to a malignant tumour originating from epithelial cells, which are termed carcinoma. AIMS AND OBJECTIVES 1. To clinically analyze the sarcomas. 2. To analyze the surgical outcome of this disease. The survival of the patient depends on the extent of metastasis and the primary identification. The study forms a base for further studies. So atleast it could be diagnosed earlier and treated to the full extent.
Martin, G; Kleinsasser, O
Based on two observations and a review of the literature, the pathological and clinical findings in sarcomas of the neck in patients with neurofibromatosis are described. Histologically these neurogenic tumours show a manifold picture; in addition to spindle-cell sarcomas pleomorphic structures are to be found, which can be similar to rhabdomyo-, lipo-, chondro-, angio-, or osteogenic sarcomas so that a histological diagnosis of a neurogenic sarcoma cannot always be made without clinical details. Up to the present surgical treatment is preferred; the value of cytostatic therapy and irradiation is controversial. The results of treating these tumours are unsatisfactory. Of 29 cases reported in the literature, only two could be found in which the patient survived without a recurrence for more than five years.
Kind, Michele [Departement d' Imagerie Medicale, Institut Bergonie, 229 cours de l' Argonne, 33076 Bordeaux Cedex (France)], E-mail: firstname.lastname@example.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)
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.
Full Text Available Objective: To report a rare case of a myeloid sarcoma of submandibular salivary gland. Methods: A 65-year-old woman with a history of successfully treated myelodysplastic syndrome, presenting with periodic painful swelling of her right submandibular area. Results: Physical evaluation, ultrasound and CT scan revealed the presence of a 3-cm mass contiguous to the submandibular salivary gland. A core needle biopsy confirmed the diagnosis of myeloid sarcoma. Bone marrow biopsy was still showing complete remission and the submandibular gland was the only extramedullary site involved. The patient was submitted to chemotherapy. Conclusion: Myeloid sarcoma is a rare extramedullary neoplasm. It can virtually involve any anatomic site, but it usually involves lymph nodes, paranasal sinuses, skin, soft tissue and periostium. Myeloid sarcomas of salivary glands are very rare and ENTs should be aware of this disease in order to include it in the differential diagnosis of a solitary neck mass.
... 2015 2014 2013 2012 Media Resources Media Contacts Multicultural Media ... This page lists cancer drugs approved by the Food and Drug Administration (FDA) for soft tissue sarcoma. The list includes ...
Aisner, Seena C.; Mirani, Neena; Hameed, Meera [University Hospital, Department of Pathology, Newark, NJ (United States); New Jersey Medical School, Newark (United States); Beebe, Kathleen [University Hospital, Department of Orthopedic Surgery, Newark, NJ (United States); New Jersey Medical School, Newark (United States); Blacksin, Marcia [University Hospital, Department of Radiology, Newark, NJ (United States); New Jersey Medical School, Newark (United States)
Alveolar soft part sarcoma is a rare soft tissue tumor typically affecting young adults. These tumors are most often seen in the deep soft tissues of the extremities and patients generally present with advanced disease. Primary bone involvement is extremely rare and has only been reported in seven cases. This is the first case of alveolar soft part sarcoma in bone documenting the ASPL-TFE3 gene product. Herein, we report a rare presentation of alveolar soft part sarcoma presenting as a primary bone neoplasm involving the proximal fibula in a 41-year-old woman. (orig.)
Full Text Available Ewing sarcoma is the second most common primary bone tumor seen in children and adolescents, typically presenting between 10 and 20 years of age. Extraosseous sarcomas of the Ewing family in adults are rare. We report a manifestation of this tumor entity in the periprostatic tissue of a 33-year-old male and discuss our treatment approach. Transrectal biopsy is a feasible and simple diagnostic tool for unclear pelvic masses. Multi-modal therapy and central registries are needed to gain knowledge of rare pelvic tumors like Ewing sarcoma.
Sreejith, G; Gangadharan, V P; Elizabath, K A; Preetha, S; Chithrathara, K
Granulocytic sarcomas are rare extramedullary tumors of malignant myeloid precursor cells. Exceedingly rare in childhood, it commonly involves skin, lymph nodes, bone, and the spine. Ovarian involvement is rare. It can arise de novo, precede the development of acute nonlymphocytic leukemia, or be the sole manifestation of relapse. We describe a 26-year-old woman with granulocytic sarcoma of the ovary without any hematologic disorder.
Mathias, Melissa; Chou, Alexander J; Meyers, Paul; Shukla, Neerav; Hameed, Meera; Agaram, Narasimhan; Wang, Lulu; Berger, Michael F.; Walsh, Michael; Kentsis, Alex
Poorly differentiated round cell sarcomas present diagnostic challenges due to their variable morphology and lack of specific immunophenotypic markers. We present a case of a 15-year-old female with a tibial tumor that exhibited features of Ewing-like sarcoma, including apparent rearrangement of the EWSR1 gene. Hybridization capture-based next-generation DNA sequencing showed evidence of complex genomic rearrangements, absence of known pathogenic Ewing-like chromosome translocations, and dele...
Chen, Quanchi; Zhou, Zifei; Shan, Liancheng; Zeng, Hui; Hua, Yingqi; Cai, Zhengdong
Src is a tyrosine kinase that is of significance in tumor biology. The present review focuses on Src, its molecular structure, and role in cancer, in addition to its expression and function in sarcoma. In addition, the feasibility of Src as a potential drug target for the treatment of sarcoma is also discussed. Previous studies have suggested that Src has essential functions in cell proliferation, apoptosis, invasion, metastasis and the tumor microenvironment. Thus, it may be a potential targ...
Satyendra Kumar Singh
Full Text Available Acroangiodermatitis or Pseudo-Kaposi sarcoma is a rare angioproliferative entity, related to chronic venous insufficiency or certain other vascular anomalies. It is often associated with chronic venous insufficiency, arteriovenous malformation of the legs, chronic renal failure treated with dialysis, paralyzed legs and amputation stumps. We hereby describe a case of 45 year old female presenting with pitting pedal edema, multiple ulcers over bilateral lower limbs with irregular margins with erythema and hyperpigmentation of the surrounding skin. Color Doppler study of bilateral lower limbs was normal. Histopathological examination from one of the lesions showed hyperplastic epidermis, proliferation of capillaries in dermis, hemosiderin deposits and lymphocytic infiltrate. These features thus confirmed the diagnosis of Acroangiodermatitis.
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.
Full Text Available Uterine sarcomas are rare tumors accounting for 3,4% of all uterine cancers. Even after radical hysterectomy, most patients relapse or present with distant metastases. The very limited clinical benefit of adjuvant cytotoxic treatments is reflected by high mortality rates, emphasizing the need for new treatment strategies. This review summarizes rising potential targets in four distinct subtypes of uterine sarcomas: leiomyosarcoma, low-grade and high-grade endometrial stromal sarcoma, and undifferentiated uterine sarcoma. Based on clinical reports, promising approaches for uterine leiomyosarcoma patients include inhibition of VEGF and mTOR signaling, preferably in combination with other targeted or cytotoxic compounds. Currently, the only targeted therapy approved in leiomyosarcoma patients is pazopanib, a multitargeted inhibitor blocking VEGFR, PDGFR, FGFR, and c-KIT. Additionally, preclinical evidence suggests effect of the inhibition of histone deacetylases, tyrosine kinase receptors, and the mitotic checkpoint protein aurora kinase A. In low-grade endometrial stromal sarcomas, antihormonal therapies including aromatase inhibitors and progestins have proven activity. Other potential targets are PDGFR, VEGFR, and histone deacetylases. In high-grade ESS that carry the YWHAE/FAM22A/B fusion gene, the generated 14-3-3 oncoprotein is a putative target, next to c-KIT and the Wnt pathway. The observation of heterogeneity within uterine sarcoma subtypes warrants a personalized treatment approach.
Hernán Augusto Centurión Sobral
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.
Cuppens, Tine; Tuyaerts, Sandra; Amant, Frédéric
Uterine sarcomas are rare tumors accounting for 3,4% of all uterine cancers. Even after radical hysterectomy, most patients relapse or present with distant metastases. The very limited clinical benefit of adjuvant cytotoxic treatments is reflected by high mortality rates, emphasizing the need for new treatment strategies. This review summarizes rising potential targets in four distinct subtypes of uterine sarcomas: leiomyosarcoma, low-grade and high-grade endometrial stromal sarcoma, and undifferentiated uterine sarcoma. Based on clinical reports, promising approaches for uterine leiomyosarcoma patients include inhibition of VEGF and mTOR signaling, preferably in combination with other targeted or cytotoxic compounds. Currently, the only targeted therapy approved in leiomyosarcoma patients is pazopanib, a multitargeted inhibitor blocking VEGFR, PDGFR, FGFR, and c-KIT. Additionally, preclinical evidence suggests effect of the inhibition of histone deacetylases, tyrosine kinase receptors, and the mitotic checkpoint protein aurora kinase A. In low-grade endometrial stromal sarcomas, antihormonal therapies including aromatase inhibitors and progestins have proven activity. Other potential targets are PDGFR, VEGFR, and histone deacetylases. In high-grade ESS that carry the YWHAE/FAM22A/B fusion gene, the generated 14-3-3 oncoprotein is a putative target, next to c-KIT and the Wnt pathway. The observation of heterogeneity within uterine sarcoma subtypes warrants a personalized treatment approach. PMID:26576131
Steiner, Michael A.; Giles, Henry W. [University of Mississippi, Department of Diagnostic Radiology, Jackson, MS (United States); Daley, William P. [University of Mississippi, Department of Pathology, Jackson, MS (United States)
Low-grade fibromyxoid sarcoma (LGFMS) is a rare soft-tissue sarcoma that usually presents in young adults as a painless, slow-growing mass. Evans first described LGFMS in 1987 as a spindle-cell sarcoma with bland histological features and paradoxically aggressive behavior. Although young adults are most frequently affected, recent reports describe pediatric cases being increasingly more common. Males and females are affected approximately equally and common locations include the deep soft tissue of the lower extremity, particularly the thigh and trunk. Primary occurrence within the chest cavity is exceedingly rare. Local recurrence and metastasis are not uncommon and present the clinician and radiologist with challenges regarding follow-up recommendations. Review of the literature reveals many cases of slowly progressive symptoms related to a mass effect. We present a healthy 12-year-old African-American girl who interestingly developed acute symptoms of shortness of breath and chest pain while playing with her brother. (orig.)
Full Text Available Ewing′s sarcoma (ES is a highly malignant tumor of children and young adults. Modern therapy for Ewing′s sarcoma combines high-dose chemotherapy for systemic control of disease, with advanced surgical and/or radiation therapeutic approaches for local control. Despite optimal management, the cure rate for localized disease is only approximately 70%, whereas the cure rate for metastatic disease at presentation is less than 30%. Patients who experience long-term disease-free survival are at risk for significant side-effects of therapy, including infertility, limb dysfunction and an increased risk for second malignancies. The identification of new targets for innovative therapeutic approaches is, therefore, strongly needed for its treatment. Many new pharmaceutical agents have been tested in early phases of clinical trials in ES patients who have recurrent disease. While some agents led to partial response or stable disease, the percentages of drugs eliciting responses or causing an overall effect have been minimal. Furthermore, of the new pharmaceuticals being introduced to clinical practice, the most effective agents also have dose-limiting toxicities. Novel approaches are needed to minimize non-specific toxicity, both for patients with recurrence and at diagnosis. This report presents an overview of the potential molecular targets in ES and highlights the possibility that they may serve as therapeutic targets for the disease. Although additional investigations are required before most of these approaches can be assessed in the clinic, they provide a great deal of hope for patients with Ewing′s sarcoma.
... not been proven to cause soft tissue sarcomas. Arsenic has also been linked to a type of ... Tissue Sarcoma Causes, Risk Factors, and Prevention Early Detection, Diagnosis, and Staging Treatment After Treatment Back To ...
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.
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,
Full Text Available Ewing’s sarcoma is typically an aggressive, poorly differentiated tumor affecting children and young adults, it accounts for 4–6% of all primary bone tumors and facial primary localizations occur in only 1–4% of all cases, mostly in the mandible and calvaria. Paranasal sinus involvement is rare. A 22-year-old female was reviewed in Oral & Cranio Maxillofacial Surgery Department. She complained of swelling of the right paranasal area, of one-month duration, progressively increasing in size and associated with pain. The medical history was unremarkable, Contrast Enhanced Computed Tomography scan showed a destructive lesion of the anterior wall of the right maxillary sinus reaching up to the medial wall of the maxillary sinus, other paranasal sinus appearance was normal. Incisional biopsy proved it to be Ewing’s Sarcoma. She was treated by chemotherapy using Vincristine, Adriamycin, and Cyclophosphamide alternating with Etoposide & Ifosfamide and Radiotherapy, and this resulted in complete regression of the tumor. Repeated PET scans every 6 months did not suggest any recurrence of the right maxillary sinus tumor. We concluded that treatment by induction chemotherapy followed by radiation therapy leads to a favorable outcome in the above described case, avoiding the morbidity that can result from surgical options.
Full Text Available Although multimodal therapies including surgery, chemotherapy, and radiotherapy have improved clinical outcomes of patients with bone and soft tissue sarcomas, the prognosis of patients has plateaued over these 20 years. Immunotherapies have shown the effectiveness for several types of advanced tumors. Immunotherapies, such as cytokine therapies, vaccinations, and adoptive cell transfers, have also been investigated for bone and soft tissue sarcomas. Cytokine therapies with interleukin-2 or interferons have limited efficacy because of their cytotoxicities. Liposomal muramyl tripeptide phosphatidylethanolamine (L-MTP-PE, an activator of the innate immune system, has been approved as adjuvant therapeutics in combination with conventional chemotherapy in Europe, which has improved the 5-year overall survival of patients. Vaccinations and transfer of T cells transduced to express chimeric antigen receptors have shown some efficacy for sarcomas. Ipilimumab and nivolumab are monoclonal antibodies designed to inhibit immune checkpoint mechanisms. These antibodies have recently been shown to be effective for patients with melanoma and also investigated for patients with sarcomas. In this review, we provide an overview of various trials of immunotherapies for bone and soft tissue sarcomas, and discuss their potential as adjuvant therapies in combination with conventional therapies.
Full Text Available Endometrial stromal tumors are rare uterine tumors (<1%. Four main categories include endometrial stromal nodule, low-grade endometrial stromal sarcoma (LG-ESS, high-grade endometrial stromal sarcoma (HG-ESS, and uterine undifferentiated sarcoma (UUS. This review is a series of articles discussing the uterine sarcomas. LG-ESS, a hormone-dependent tumor harboring chromosomal rearrangement, is an indolent tumor with a favorable prognosis, but characterized by late recurrences even in patients with Stage I disease, suggesting the requirement of a long-term follow-up. Patients with HG-ESS, based on the identification of YWHAE-NUTM2A/B (YWHAE-FAM22A/B gene fusion, typically present with advanced stage diseases and frequently have recurrences, usually within a few years after initial surgery. UUS is, a high-grade sarcoma, extremely rare, lacking a specific line of differentiation, which is a diagnosis of exclusion (the wastebasket category, which fails to fulfill the morphological and immunohistochemical criteria of translocation-positive ESS. Surgery is the main strategy in the management of uterine sarcoma. Due to rarity, complex biological characteristics, and unknown etiology and risk factors of uterine sarcomas, the role of adjuvant therapy is not clear. Only LG-ESS might respond to progestins or aromatase inhibitors.
Montero Pérez, Iria; Rodríguez-Pazos, Laura; Álvarez-Pérez, Adriana; Ferreirós, M Mercedes Pereiro; Aliste, Carlos; Suarez-Peñaranda, Jose Manuel; Toribio, Jaime
Classical Kaposi sarcoma (KS) usually appears on lower extremities accompanied or preceded by local lymphedema. However, the development in areas of chronic lymphedema of the arms following mastectomy, mimicking a Stewart-Treves syndrome, has rarely been described. We report an 81-year-old woman who developed multiple, erythematous to purple tumors, located on areas of post mastectomy lymphedema. Histopathological examination evidenced several dermal nodules formed by spindle-shaped cells that delimitated slit-like vascular spaces with some red cell extravasation. Immunohistochemically, the human herpesvirus type 8 (HHV-8) latent nuclear antigen-1 was detected in the nuclei of most tumoral cells confirming the diagnosis of KS. Lymphedema could promote the development of certain tumors by altering immunocompetence. Although angiosarcoma (AS) is the most frequent neoplasia arising in the setting of chronic lymphedema, other tumors such as benign lymphangiomatous papules (BLAP) or KS can also develop in lymphedematous limbs. It is important to establish the difference between AS and KS because their prognosis and treatment are very different. Identification by immunohistochemistry of HHV-8 is useful for the distinction between KS and AS or BLAP.
Luiz Fernando Fróes Fleury Jr
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.
Picciotto, Franco; Zaccagna, Alessandro; Derosa, Giovanni; Pisacane, Alberto; Puiatti, Paolo; Colombo, Enrico; Dardano, Fabrizio; Ottinetti, Antonio
Clear cell sarcoma of the tendons and aponeuroses is an aggressive, rare soft tissue tumour that occurs predominantly in the extremities of young adults. Although it appears to be histogenetically related to melanoma, its clinical behaviour resembles soft tissue sarcoma. Prognosis is reported to be poor due to the great propensity of regional and distant metastases. The risk of metastases to regional lymph nodes is very high. We describe a case of clear cell sarcoma of the hand and evaluate the feasibility of the sentinel lymph node biopsy (SLNB) technique in this kind of tumour. Up to now, there are no reports that describe the use of SLNB in clear cell sarcoma. The SLNB technique was carried out with success and the sentinel lymphnode was easily identified. Histological examination of the lymphnode allowed identification of metastatic cells and thus provide for radical lymphadenectomy. We maintain that SLNB can be successfully used for this type of tumour although this result should be evaluated and confirmed by larger case studies.
Ben Hassouna, J.; Slimane, M.; Damak, T.; Chargui, R.; Ben Bachouche, W.; Oukad, M.; Bouzaine, H.; Bouzid, T.; Mtallah, M.; Khomsi, F.; Gamoudi, A.; Hechiche, M.; Dhiab, T.; Rahal, K. [Institut Salah Azaiz, Service de Chirurgie Carcinologique, Tunis (Tunisia)
Purpose: To analyse the management and clinical outcome of patients treated for retroperitoneal soft tissue sarcoma and to identify prognosis factors. Patients and method: This is a retrospective study of 20 adults; 11 women and nine men treated between 1980 and 2000 in our institution. Results: There were seven lipo sarcomas and 13 leiomyo-sarcomas. The mean tumor size was 26 cm. Three patients underwent a complete resection (R0), 14 patients an incomplete resection (R1/R2) with microscopic or macroscopic tumour remained after surgery. Only three patients had a surgical biopsy. Median follow-up was 18 months. Overall survival rates was 45%, 10% at two and five years, respectively. Among the following factors, sex, age, symptom duration, tumor size, histologic type, histologic grade, resection type, mitotic index, necrosis extension, histologic differentiation, and A.J.C.C. stage, only mitotic index factor (scale 1,2 versus scale 3) significantly influenced patient survival (p = 0.02). Conclusion: This study re-emphasizes the poor outcome of patients with retroperitoneal sarcoma. Survival rates appeared low when compared with other studies. These results incite for a more aggressive approach. (authors)
Full Text Available "nBackground: Epithelioid sarcoma is a malignant soft tissue tumor of uncertain histogenesis, categorized as a morphologically distinct neoplasm that characteristically affects the distal parts of the extremities in young adults. In fact, epithelioid sarcoma is the most common soft tissue sarcoma in the hand and wrist. "nCase report: This 32 year-old male presented with an inguinal swelling, for which he underwent surgery with the initial intent of inguinal hernia repair. With this uncommon manifestation and site, among the differential diagnosis were abscess and GI malignancies. However, after a supplementary evaluation that included biopsy of the ulcer margin, the diagnosis focused on undifferentiated high-grade epithelial tumor, highly suggestive of epithelioid sarcoma. Immunohistochemical studies revealed CD34 and cytokeratin positivity, which confirmed this diagnosis. "nConclusion: Due to the malignant nature, invasive behavior and high recurrence rate of this tumor, as well as its unknown response to chemotherapy and radiation, extensive resection and hepatectomy are recommended for treatment.
Puneet Kumar Bagri
Full Text Available Epithelioid sarcoma is a rare variety of soft tissue sarcoma that affects young adults mostly involves forearm and hand. It mainly presents as a painless slow growing mass, but can present as multifocal lesions. It has a high rate of recurrence and metastasis. A 35-year-old male presented to our department in November 2013 with a painful ulcer with bloody discharge in the medial side of left wrist. The patient gave a history of swelling with serous discharge from 11 years. Local excision was done 6 times from 2003 to 2011. The patient also gave a history of falling on an outstretched hand and swelling at same site in July 2013, this time again wide local excision was done. The histopathology report was suggestive of high-grade osteogenic sarcoma. Immunohistochemistry (IHC showed expression of cytokeratin, epithelial membrane antigen, CD34 and CA-125; tumor cells had lost INI-1 expression. IHC confirmed the diagnosis of epithelioid sarcoma of left wrist. Contrast-enhanced computed tomography thorax was suggestive of lung metastasis. Thus, doxorubicin based chemotherapy was planned.
Matsuo, Koji; Eno, Michele L; Im, Dwight D; Rosenshein, Neil B
We conducted a literature review to determine the clinical characteristics of genital sarcoma during pregnancy. The systematic literature search was conducted using the search engines PubMed and MEDLINE with keywords "sarcoma" and "pregnancy" and was limited to female genital organs such as ovary, uterus, cervix, vagina, vulva, and retroperitoneal sarcoma. Kaposi's sarcoma, metastatic sarcoma, history of sarcoma, bone sarcoma located in pelvis, and fetal sarcoma were excluded in this study. There were 40 cases of genital sarcoma during pregnancy between 1955 and 2007. The majority of the cases were uterine sarcoma (37.5%), followed by retroperitoneal sarcoma (27.5%), vulvar sarcoma (22.5%), and vaginal sarcoma (12.5%). Mean age of the patient was 27.8 +/- 7.0. The distribution in the onset of symptoms had two peaks: first trimester (27.5%) and third trimester (50.0%). Growing mass (42.5%), abdominal pain (30.0%), and vaginal bleeding (22.5%) were the three most common symptoms. Incidental diagnosis was made in 22.5% and included during cesarean section (12.5%) and routine pelvic exam (7.5%). The cases initially not suspicious for malignancy were 42.5%. Thirty-three (82.5%) cases had live-born infants with term delivery in 55.2%. Mean birth weight was 2843 +/- 791 g, and male infants were more common (66.7%). Intrauterine growth retardation was seen in 12.5% of cases. Preterm labor was a common complication. Median survival period was 2.5 years (95% confidence, 1.9 to 3.1). The 2-, 3-, and 5-year cumulative survival rates were 60%, 38%, and 17%, respectively. Genital sarcomas in pregnancy are rare. There is a delay in diagnosis due to low index of suspicion. A majority had live births, and the 5-year survival is similar to that of advanced-stage sarcoma in nonpregnant women.
Fritz, Jan; Claussen, Claus D.; Pereira, Philippe L.; Horger, Marius S. [Eberhard-Karls-University, Department of Diagnostic Radiology, Tuebingen (Germany); Vogel, Wichard [Eberhard-Karls-University, Department of Internal Medicine-Oncology, Tuebingen (Germany); Wehrmann, Martin [Eberhard-Karls-University, Department of Pathology, Tuebingen (Germany)
We report a case of granulocytic sarcoma exclusively manifesting as diffuse intramuscular infiltration of the proximal upper and lower limb girdle and the torso muscles in a patient with previous history of acute myelogenous leukemia 5a. Whole-body CT showed widespread distribution of ill-defined intramuscular, homogeneously enhancing lesions. On whole-body MRI, lesions were homogeneously hyperintense on fat saturated T2-weighted images, isointense on T1-weighted images and strongly enhancing after intravenous gadolinium contrast administration. Histopathology revealed muscular infiltration of blast cells with identical immunochemistry to the initial manifestation of leukemia, diagnostic for an extramedullary relapse manifesting as granulocytic sarcoma. CT and MRI characteristics of this previously undocumented manifestation of granulocytic sarcoma should assist in the identification of such cases. (orig.)
OBJECTIVE To investigate the clinical diagnosis and differential diagnosis of synovial sarcoma (SS).METHODS A total of 41 paraffin-embedded synovial sarcoma samples were examined by H&E staining, immunohistochemistry staining and the reverse transcriptase polymerase chain reaction (RT-PCR), in order to provide a scientific bases for diagnosis and differential diagnosis.RESULTS Twelve cases were a biphasic type, 22 cases were a monophasic fibrous type, and 7 cases were a poorly differentiated type. Thirty-six cases were both CK (and/or EMA) and Vim positive. Five cases were only Vim positive. A SYT-SSX fusion gene was detected in 18 cases by RT-PCR.CONCLUSION By observation of the histomorphology, immunohistochemistry markers and detection of a SYT-SSX fusion gene, we can make a clinical pathological diagnosis of synovial sarcoma.
Lidia Torres Ajá
Full Text Available Primary breast sarcoma is the least frequent non-epithelial malignant tumour, representing less than 1 % of all breast cancers. It has a dismal prognosis with the presence of early metastases, mainly in the lungs and bones. Survival is very poor at 5 years. A case of a 79 year-old female patient with a large ulcerated sarcoma in the left breast is presented. The patient was examined in an interdisciplinary consultation, and the presence of a stromal sarcoma of the breast without apparent visceral or bone metastases was confirmed by an aspiration biopsy with thick needle, excisional biopsy by paraffin and by immunohistochemical studies. The publication of this report has clinical interest for health professionals due to the rarity of the disease.
Ergül, N; Aydın, M
The role of FDG PET/CT in management of soft tissue and bone sarcomas has been described in many studies up-to-date. However, contribution of PET/CT to diagnosis and treatment in some types of sarcomas that are seen with low incidence has not been identified properly yet. Clear cell sarcoma, synovial sarcoma of chest and myxoid lyposarcoma are rare types of sarcomas. We aimed to describe the FDG uptake patterns of these rare tumors and find out the role of FDG PET/CT in management of disease. Copyright © 2012 Elsevier España, S.L. and SEMNIM. All rights reserved.
Deschênes, Isabelle; Dion, Louise; Beauchesne, Claude; de Brum-Fernandes, Artur
The association between Kaposi's sarcoma and infection with human herpesvirus 8 is now well recognized. Immunologic impairment is associated with 2 forms of Kaposi's sarcoma, epidemic [associated with human immunodeficiency virus (HIV) infection] and iatrogenic (associated with immunosuppressive treatment); both forms have become more common during the last decade. We describe an HIV negative 54-year-old man who developed Kaposi's sarcoma 2 months after the beginning of immuno-suppressive therapy for Wegener's granulomatosis (WG). With tapering of medication, complete remission of Kaposi's sarcoma was achieved in one year. To our knowledge, this is the second reported case of iatrogenic Kaposi's sarcoma in a patient with WG.
Evola, Francesco R.; Costarella, Luciano; Pavone, Vito; Caff, Giuseppe; Cannavò, Luca; Sessa, Andrea; Avondo, Sergio; Sessa, Giuseppe
Osteosarcoma is the most frequent malignant bone neoplasm, followed by chondrosarcoma and Ewing sarcoma. The diagnosis of bone neoplasms is generally made through histological evaluation of a biopsy. Clinical and radiological features are also important in aiding diagnosis and to complete the staging of bone cancer. In addition to these, there are several non-specific serological or specific molecular markers for bone neoplasms. In bone tumors, molecular markers increase the accuracy of the diagnosis and assist in subtyping bone tumors. Here, we review these markers and discuss their role in the diagnosis and prognosis of the three most frequent malignant bone neoplasms, namely osteosarcoma, chondrosarcoma, and Ewing sarcoma. PMID:28439237
Teshima, Takahiro; Hata, Takashi; Nezu, Yoko; Michishita, Masaki; Matsumoto, Hirotaka; Mizutani, Hisashi; Takahashi, Kimimasa; Koyama, Hidekazu
A 9-year-old spayed female domestic shorthair cat presented with a skin lesion of the left tarsus. The lesion was biopsied and, based on the microscopic appearance and immunohistochemical characteristics, histiocytic sarcoma was diagnosed. Amputation was performed with improved demeanor seen postoperatively. However, between 44 and 60 days following the surgery, relapse of skin lesions appeared in multiple locations, including at the previous amputation site, and euthanasia was elected. This is the first report of a histiocytic sarcoma treated with amputation in a cat.
H. Krishna Moorthy
Full Text Available Primary renal synovial sarcoma is a rare tumor having a specific chromosomal translocation t(X; 18 (p11.2; q11.2. The clinical features of this tumor and radiologic appearances are quite similar to those of renal cell carcinoma. Confirmatory diagnosis requires fluorescent in situ hybridization or reverse transcriptase polymerase chain reaction validation for differentiating the tumors from sarcomatoid renal cell carcinoma. We present a case of primary renal synovial sarcoma that was diagnosed in a middle-aged man.
Choi, J; Means, R E; Damania, B; Jung, J U
Kaposi's Sarcoma associated Herpesvirus (KSHV) is the most recently discovered human tumor virus and is associated with the pathogenesis of Kaposi's sarcoma, primary effusion lymphoma, and Multicentric Casttleman's disease. KSHV contains numerous open reading frames with striking homology to cellular genes. These viral gene products play a variety of roles in KSHV-associated pathogenesis by disrupting cellular signal transduction pathways, which include interferon-mediated anti-viral responses, cytokine-regulated cell growth, apoptosis, and cell cycle control. In this review, we will attempt to cover our understanding of how viral proteins deregulate cellular signaling pathways, which ultimately contribute to the conversion of normal cells to cancerous cells.
Cooper, T M
A retrospective review of 33 cases of soft tissue sarcoma of the extremity presenting over a 10 year period was undertaken. The history, patterns of referral, diagnostic investigations, procedures undertaken and outcomes were studied. We found there was a frequent delay in diagnosis and sometimes misinterpretation of biopsy specimens. Patients were seen by a variety of specialists from disciplines such as general surgery, plastic surgery, orthopaedic surgery and rheumatology. Considerable progress has been made in the treatment of soft tissue sarcomas, often allowing local control of the tumour without amputation. We believe there should be early referral of patients having these tumours to a centre where a combined multidisciplinary approach can be undertaken.
Panda, Kitela Ghislain; Hale, Martin J; Kruger, Deirdre; Luvhengo, Thifhelimbilu Emmanuel
Tumour size, grade and subtype are the main prognostic factors in adult patients presenting with soft-tissue sarcoma. Planning for appropriate management, including the need for additional staging investigations and neoadjuvant therapy, is dependent on reliable preoperative histopathological results. To determine whether there is agreement between preoperative and post-excision histological findings in patients presenting with soft-tissue sarcoma, and whether the agreement is influenced by the subtypes of sarcomas. Records of adult patients who had soft-tissue sarcomas excised were reviewed. Kaposi's sarcoma and gastrointestinal stromal tumours were excluded. Data were retrieved from the Department of Anatomical Pathology of the National Health Laboratory Service and theatre records at Chris Hani Baragwanath Academic Hospital, and included patient demography, tumour sites and size, HIV status, biopsy types and post-excision histological findings. Records of 153 patients were found (median age 44 years). The majority of the sarcomas were >5 cm in diameter, deep seated and localised in extremities. The commonest subtype, irrespective of HIV status, was dermatofibrosarcoma protuberans. Fine-needle aspiration biopsy (FNAB) results were inaccurate in determining the malignant nature, grade and subtype of sarcoma. Rates of accurate tumour subtype classification following core needle and incision biopsies when compared with post-excision histological findings were 73.1% and 78.3%, respectively. FNAB should not be used in the primary evaluation of soft-tissue tumours. A report of spindle cells on the FNAB smear should be followed by core needle or incision biopsy. Incision biopsy is superior to core needle biopsy in the classification of sarcomas by subtype.
Stewart, Elizabeth; Goshorn, Ross; Bradley, Cori; Griffiths, Lyra M; Benavente, Claudia; Twarog, Nathaniel R; Miller, Gregory M; Caufield, William; Freeman, Burgess B; Bahrami, Armita; Pappo, Alberto; Wu, Jianrong; Loh, Amos; Karlström, Åsa; Calabrese, Chris; Gordon, Brittney; Tsurkan, Lyudmila; Hatfield, M Jason; Potter, Philip M; Snyder, Scott E; Thiagarajan, Suresh; Shirinifard, Abbas; Sablauer, Andras; Shelat, Anang A; Dyer, Michael A
Ewing sarcoma (EWS) is a tumor of the bone and soft tissue that primarily affects adolescents and young adults. With current therapies, 70% of patients with localized disease survive, but patients with metastatic or recurrent disease have a poor outcome. We found that EWS cell lines are defective in DNA break repair and are sensitive to PARP inhibitors (PARPis). PARPi-induced cytotoxicity in EWS cells was 10- to 1,000-fold higher after administration of the DNA-damaging agents irinotecan or temozolomide. We developed an orthotopic EWS mouse model and performed pharmacokinetic and pharmacodynamic studies using three different PARPis that are in clinical development for pediatric cancer. Irinotecan administered on a low-dose, protracted schedule previously optimized for pediatric patients was an effective DNA-damaging agent when combined with PARPis; it was also better tolerated than combinations with temozolomide. Combining PARPis with irinotecan and temozolomide gave complete and durable responses in more than 80% of the mice.
Hasan, Roumina; Kumar, Sandeep; Rao, Lakshmi
Pelvic localization of synovial sarcoma is a rare phenomenon and to the best of our knowledge its presentation as a large "dumb-bell"-shaped abdomino-pelvic mass showing extension to the thigh has never been reported in the literature. We report a case of a young adult presenting with retention of urine and was found to have a large abdomino-pelvic mass causing bony destruction and compression of pelvic viscera. A biopsy revealed a cellular tumor composed of spindle to oval cells arranged in a hemangiopericytomatous pattern. Histopathology was suggestive of poorly differentiated synovial sarcoma. Immunohistochemistry (IHC) was positive for vimentin, CD 99, Bcl2, Mic2 and focally for EMA and negative for CD 34, CK, desmin, synaptophysin, and WT1. Due to equivocal IHC findings molecular analysis was done which confirmed the diagnosis as synovial sarcoma.
Full Text Available Pelvic localization of synovial sarcoma is a rare phenomenon and to the best of our knowledge its presentation as a large "dumb-bell"-shaped abdomino-pelvic mass showing extension to the thigh has never been reported in the literature. We report a case of a young adult presenting with retention of urine and was found to have a large abdomino-pelvic mass causing bony destruction and compression of pelvic viscera. A biopsy revealed a cellular tumor composed of spindle to oval cells arranged in a hemangiopericytomatous pattern. Histopathology was suggestive of poorly differentiated synovial sarcoma. Immunohistochemistry (IHC was positive for vimentin, CD 99, Bcl2, Mic2 and focally for EMA and negative for CD 34, CK, desmin, synaptophysin, and WT1. Due to equivocal IHC findings molecular analysis was done which confirmed the diagnosis as synovial sarcoma.
Ryland, Katherine E; Hawkins, Allegra G.; Weisenberger, Daniel J.; Punj, Vasu; Borinstein, Scott C.; Laird, Peter W.; Martens, Jeffrey R.; Lawlor, Elizabeth R.
Polycomb proteins are essential regulators of gene expression in stem cells and development. They function to reversibly repress gene transcription via post-translational modification of histones and chromatin compaction. In many human cancers, genes that are repressed by polycomb in stem cells are subject to more stable silencing via DNA methylation of promoter CpG islands. Ewing sarcoma is an aggressive bone and soft tissue tumor that is characterized by over-expression of polycomb proteins. This study investigates the DNA methylation status of polycomb target gene promoters in Ewing sarcoma tumors and cell lines and observes that the promoters of differentiation genes are frequent targets of CpG-island DNA methylation. In addition, the promoters of ion channel genes are highly differentially methylated in Ewing sarcoma compared to non-malignant adult tissues. Ion channels regulate a variety of biological processes, including proliferation, and dysfunction of these channels contributes to tumor pathogenesis. In particular, reduced expression of the voltage-gated Kv1.5 channel has been implicated in tumor progression. These data show that DNA methylation of the KCNA5 promoter contributes to stable epigenetic silencing of Kv1.5 channel. This epigenetic repression is reversed by exposure to the DNA methylation inhibitor decitabine, which inhibits Ewing sarcoma cell proliferation through mechanisms that include restoration of Kv1.5 channel function. Implications This study demonstrates that promoters of ion channels are aberrantly methylated in Ewing sarcoma and that epigenetic silencing of KCNA5 contributes to tumor cell proliferation, thus providing further evidence of the importance of ion channel dyregulation to tumorigenesis. PMID:26573141
... and young adults. Affected individuals usually feel stiffness, pain, swelling, or tenderness of the bone ... of genetic change, called a somatic mutation, is not inherited. The protein produced from ...
van Maldegem Annemiek M
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
Brambilla, L; Labianca, R; Ferrucci, SM; Taglioni, M; Boneschi, [No Value
Background: Several drugs are active in aggressive classical Kaposi's sarcoma (CKS); chemotherapeutic agents with fewer side-effects, more rapid response and able to overcome resistance to previous treatment are advisable when treating patients in a second line. Gemcitabine, an analogue of deoxycyti
Fabrizio Albarello, MD
Full Text Available A rapidly enlarging right sternoclavicular mass in a young male was labeled as a nonspecific mass. MRI played a crucial role in characterizing the lesion, helping to define the possible mesenchymal origin and the relative involvement of the surrounding structures. We also discuss the differential diagnosis of an extraosseus Ewing sarcoma (ES, with its imaging findings.
Aldenhoven, M.; Barlo, N. P.; Sanders, C. J. G.
Kaposi's sarcoma (KS) remains the most commonly diagnosed malignancy in HIV-infected patients, and is one of the AIDS-defining diagnoses. Several different therapeutic options are available, but the optimal therapy is still unclear. The incidence of KS has sharply declined since highly active antire
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)
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.
Mani, S.; Naik, L.; Shet, S.; Vora, I.M.; Rananavare, R. [BYL Nail Hospital, Bombay (India). Departments of Radiology and Pathology
A 35-year-old woman presented with abdominal distension and a palpable liver mass. Ultrasonography and computed tomography revealed a large well-delineated liver mass with bilobar involvement. Based on autopsy and immunohistochemical findings, a final diagnosis of primary pleomorphic liver sarcoma with myogenic differentiation W established. Copyright (1998) Blackwell Science Pty Ltd 8 refs., 5 figs.
Shiba, K.; Fukuma, H.; Beppu, Y.; Hirota, T. (National Cancer Center, Tokyo (Japan). Hospital); Shinohara, N.
Criteria for the diagnosis of radiation-induced sarcoma have been previously described. All cases must have a history of irradiation and the second neoplasm must have arisen in the area of the radiation field. A latent period of several years must have elapsed after irradiation before clinical evidence of a second malignant neoplasm. Most important thing is that, all suspected cases must have been proved histologically. We have experienced 3 cases of radiation-induced sarcoma, they were 42-years-old man who developed an osteosarcoma of the lumbar spine at the field of postoperative irradiation for seminoma 7 years previously, 69-years-old woman who developed a malignant fibrous histiocytoma of the buttock at the field of radical radiation for uterine carcinoma 7 years previously and 59-years-old woman who developed an extraskeletal osteosarcoma of the abdominal wall at the field of postoperative irradiation for uterine sarcoma 7 years previously. The last case is very rare and only 8 cases of radiation-induced extraskeletal osteosarcoma have been reported. Since there has been a definite trend in the treatment of cancer toward employing radiation for more favorable cases, in addition to technical improvements in the administration of radiotherapy and more modern equipment, survival data may have been altered considerably in many malignant tumors. Accordingly, more radiation-induced tumors may be encountered in the future. The clinical presentation and histopathology of these radiation-induced sarcomas are presented with a review of the literature.
Wilcock, Brian; Wilcock, Anne; Bottoms, Katherine
Comparison of the annual prevalence of feline postvaccinal sarcomas among 11 609 feline skin mass submissions from 1992 to 2010 revealed no decrease in disease prevalence or increase in the age of affected cats in response to changes in vaccine formulation or recommended changes in feline vaccination protocols.
Wilcock, Brian; Wilcock, Anne; Bottoms, Katherine
Comparison of the annual prevalence of feline postvaccinal sarcomas among 11 609 feline skin mass submissions from 1992 to 2010 revealed no decrease in disease prevalence or increase in the age of affected cats in response to changes in vaccine formulation or recommended changes in feline vaccination protocols.
Gholami, Sepideh; Jacobs, Charlotte D.; Kapp, Daniel S.; Parast, Layla M.; Norton, Jeffrey A.
Introduction. Retroperitoneal sarcomas are uncommon large malignant tumors. Methods. Forty-one consecutive patients with localized retroperitoneal sarcoma were retrospectively studied. Results. Median age was 58 years (range 20–91 years). Median tumor size was 17.5 cm (range 4–41 cm). Only 2 tumors were <5 cm. Most were liposarcoma (44%) and high-grade (59%). 59% were stage 3 and the rest was stage 1. Median followup was 10 months (range 1–106 months). Thirty-eight patients had an initial complete resection; 15 (37%) developed recurrent sarcoma and 12 (80%) had a second complete resection. Patients with an initial complete resection had a 5-year survival of 46%. For all patients, tumor grade affected overall survival (P = .006). Complete surgical resection improved overall survival for high-grade tumors (P = .03). Conclusions. Tumor grade/stage and complete surgical resection for high-grade tumors are important prognostic variables. Radiation therapy or chemotherapy had no significant impact on overall or recurrence-free survival. Complete surgical resection is the treatment of choice for patients with initial and locally recurrent retroperitoneal sarcoma. PMID:19826633
Soft tissue sarcomas are rare malignancies originating from mesenchymal origin. They may occur at any age, but the incidence increases with age: about 50% of the patients are over 60 years of age. A distinct peak incidence is made up by embryonal rhabdomyosarcomas that mostly afflict children at age
Full Text Available Introduction. Retroperitoneal sarcomas are uncommon large malignant tumors. Methods. Forty-one consecutive patients with localized retroperitoneal sarcoma were retrospectively studied. Results. Median age was 58 years (range 20–91 years. Median tumor size was 17.5 cm (range 4–41 cm. Only 2 tumors were <5 cm. Most were liposarcoma (44% and high-grade (59%. 59% were stage 3 and the rest was stage 1. Median followup was 10 months (range 1–106 months. Thirty-eight patients had an initial complete resection; 15 (37% developed recurrent sarcoma and 12 (80% had a second complete resection. Patients with an initial complete resection had a 5-year survival of 46%. For all patients, tumor grade affected overall survival (=.006. Complete surgical resection improved overall survival for high-grade tumors (=.03. Conclusions. Tumor grade/stage and complete surgical resection for high-grade tumors are important prognostic variables. Radiation therapy or chemotherapy had no significant impact on overall or recurrence-free survival. Complete surgical resection is the treatment of choice for patients with initial and locally recurrent retroperitoneal sarcoma.
Farshadpour, F; Schaapveld, M; Suurmeijer, AJH; Wymenga, ANM; Otter, R; Hoekstra, HJ
Background : Soft tissue sarcomas (STS) are uncommon malignancies and elderly STS patients have been reported to receive less definitive treatment compared to young STS patients. The present study was performed to investigate whether withholding treatment was based on disease specific aspects, patie
Full Text Available Objective: In this study, the role of GLUT1 expression in synovial sarcomas and its association with disease pathogenesis were examined.Materials and Methods: Twenty two cases of synovial sarcoma were included in this study. The clinicopathological features of the cases, such as age, sex, localization of tumor, information of primary or metastatic tumor, histopathological type were recorded. The tissue microarray paraffin block containing tumor tissues was built by using tissue microarrayer. GLUT1 expression was analyzed on tissue sections by immunohistochemistry.Results: A total of 22 cases (mean age 36 years; range 14-54 years were analyzed. All cases except one were primary tumors. The tumors showed monophasic histological type in 13 cases and biphasic type in 9 cases. GLUT1 expression was found in 3 cases with biphasic type (14%. The cytoplasmic and incomplete membranous GLUT1 expression was seen in the tumor cells showing epithelial-glandular differentiation, whereas spindled cells were negative.Conclusion: Although GLUT1 expression is a diagnostic marker for juvenile capillary hemangioma and perineural tumors, both of which included in the group of mesenchymal tumors, it can be seen in a subset of synovial sarcomas. In our series, the observation of GLUT1 expression especially in the epithelial component of biphasic synovial sarcomas suggests that; i GLUT1 may be relatively used by tumoral cells composing epithelial component of the tumor, and ii the spindle cell component of the tumor would have been positive for other glucose transporters. The finding of uncommon GLUT1 expression in synovial sarcomas is indirectly consistent with the reported results of decreased standardized uptake value by Positron emission tomography with 2-[18F]fluoro-2-deoxy-D-glucose method in the literature.
Tellez-Gabriel, Marta; Brown, Hannah K.; Young, Robin; Heymann, Marie-Françoise; Heymann, Dominique
Sarcomas are a heterogeneous group of malignant neoplasms of mesenchymal origin, many of which have a propensity to develop distant metastases. Cancer cells that have escaped from the primary tumor are able to invade into surrounding tissues, to intravasate into the bloodstream to become circulating tumor cells (CTCs), and are responsible for the generation of distant metastases. Due to the rarity of these tumors and the absence of specific markers expressed by sarcoma tumor cells, the characterization of sarcoma CTCs has to date been relatively limited. Current techniques for isolating sarcoma CTCs are based on size criteria, the identification of circulating cells that express either common mesenchymal markers, sarcoma-specific markers, such as CD99, CD81, or PAX3, and chromosomal translocations found in certain sarcoma subtypes, such as EWS-FLI1 in Ewing’s sarcoma, detection of osteoblast-related genes, or measurement of the activity of specific metabolic enzymes. Further studies are needed to improve the isolation and characterization of sarcoma CTCs, to demonstrate their clinical significance as predictive and/or prognostic biomarkers, and to utilize CTCs as a tool for investigating the metastatic process in sarcoma and to identify novel therapeutic targets. The present review provides a short overview of the most recent literature on CTCs in sarcoma. PMID:27656422
Solid Tumor; Adult Central Nervous System Germ Cell Tumor; Adult Rhabdomyosarcoma; Childhood Central Nervous System Germ Cell Tumor; Childhood Soft Tissue Sarcoma; Ewing Sarcoma; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Ovarian Mixed Germ Cell Tumor; Previously Untreated Childhood Rhabdomyosarcoma; Recurrent Adult Brain Tumor; Recurrent Adult Soft Tissue Sarcoma; Recurrent Childhood Brain Stem Glioma; Recurrent Childhood Cerebellar Astrocytoma; Recurrent Childhood Cerebral Astrocytoma; Recurrent Childhood Ependymoma; Recurrent Childhood Malignant Germ Cell Tumor; Recurrent Childhood Medulloblastoma; Recurrent Childhood Pineoblastoma; Recurrent Childhood Supratentorial Primitive Neuroectodermal Tumor; Recurrent Childhood Visual Pathway and Hypothalamic Glioma; Recurrent Childhood Visual Pathway Glioma; Recurrent Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Recurrent Extragonadal Germ Cell Tumor; Recurrent Extragonadal Non-seminomatous Germ Cell Tumor; Recurrent Malignant Testicular Germ Cell Tumor; Recurrent Neuroblastoma; Recurrent Ovarian Germ Cell Tumor; Recurrent Wilms Tumor and Other Childhood Kidney Tumors; Unspecified Adult Solid Tumor, Protocol Specific; Unspecified Childhood Solid Tumor, Protocol Specific
Metastatic Ewing Sarcoma; Metastatic Osteosarcoma; Recurrent Ewing Sarcoma; Recurrent Osteosarcoma; Stage III Osteosarcoma AJCC v7; Stage IV Osteosarcoma AJCC v7; Stage IVA Osteosarcoma AJCC v7; Stage IVB Osteosarcoma AJCC v7; Unresectable Ewing Sarcoma; Unresectable Osteosarcoma
Full Text Available An undifferentiated embryonal sarcoma of the liver is a rare malignant tumour. We highlight the diagnostic dilemma and differential diagnosis of a case involving a large cystic liver lesion in a young adult. A 20-year-old man presented with a large liver lesion to the Universiti Kebangsaan Malaysia Medical Center, Kuala Lumpur, Malaysia, in February 2012. The initial clinico-radiological presentations were suggestive of a liver abscess. A total tumour resection was performed and the final histopathological results of the resected specimen indicated an undifferentiated embryonal sarcoma of the liver. The ultrasound and computed tomographic images of the tumour were reviewed and found to be contradictory in appearance, as the tumour seemed predominantly solid in the ultrasound image and predominantly cystic in the computed tomographic image. Familiarisation with the imaging appearance of this tumour and a high index of suspicion is therefore crucial in making a successful diagnosis.
Aggerholm-Pedersen, Ninna; Maretty-Kongstad, Katja; Keller, Johnny
sarcoma were included. Of these patients, 63 were diagnosed with chondrosarcoma and 109 patients with Ewing/osteosarcoma. The median age was 55 years for chondrosarcoma and 19 years for Ewing/osteosarcoma patients. The overall 5-year mortality was 31% [95% confidence interval (CI): 21-44] and 41% (95% CI......OBJECTIVE: Certain biomarkers such as the C-reactive protein, serum albumin, and the neutrophils to lymphocyte ratio are of prognostic significance regarding survival in different types of cancers. Data from sarcoma patients are sparse and mainly derived from soft tissue sarcoma and/or metastatic...... with localized bone sarcomas and to adjust for potential confounders. MATERIAL AND METHODS: All patients diagnosed with localized intermediate and high-grade bone sarcoma during 1994 to 2008 were extracted from the Aarhus Sarcoma Registry. The serum levels of albumin, C-reactive protein, hemoglobin, neutrophils...
Scully, J.M.; Uno, J.M.; McIntyre, M.; Mosely, S. (Bay Area Hospital, Coos Bay, OR (USA))
A 64-year-old man had a prostatic sarcoma 8 years after transurethral prostatectomy and radical bilateral pelvic lymph node dissection with insertion of 125-iodine implants for stage B1N carcinoma of the prostate. Therapy for the sarcoma consisted of isolated pelvic perfusion and then pelvic exenteration with creation of an ileal conduit and colostomy. The pathology report showed well encapsulated grade 2 spindle cell sarcoma of the prostate. Multiple small metallic particles were embedded in the tumor specimen.
Thanos, Loukas; Mylona, Sofia; Kalioras, Vasilios; Pomoni, Maria; Batakis, Nikolaos [Radiology Department, ' ' Korgialeneio-Benakeio' ' , Red Cross Hospital of Athens, 1 Athanasaki Street, 11526, Athens (Greece)
A case of osseous Kaposi sarcoma in a 35-year-old man is described. The patient (HIV-positive for 8 years) suffered from cutaneous Kaposi sarcoma and presented with right-sided chest pain. He underwent a chest CT scan that revealed three osteolytic lesions involving rib and vertebra with large soft tissue masses, without cutaneous lesions at these sites. CT-guided core needle biopsy led to a histological diagnosis of Kaposi sarcoma. (orig.)
Llombart-Cussac, A.; Pivot, X; Contesso, G; Rhor-Alvarado, A.; Delord, J P; Spielmann, M.; Türsz, T.; Le Cesne, A.
The effect of additional treatments after surgery in patients with primary cardiac sarcoma (PCS) remains unknown. The present study aims to evaluate the benefit of chemotherapy in patients with non-metastatic cardiac sarcomas after optimal resection. Between October 1979 and December 1995, 15 patients with a median age of 45 (range 16-66) and a resected primary cardiac sarcoma [angiosarcoma (six), malignant fibrous histiocytoma (three), leiomyosarcoma (two), rhabdomyosarcoma (two), liposarcom...
Michelle S. Ginsberg
Full Text Available Background. Subcentimeter pulmonary nodules are being detected with increasing frequency in patients with sarcoma due to the greater use of chest CT, the advent of helical (spiral CT scanning and multidetector scanners, and the attendant decrease in image section thickness.Assessing the clinical significance of these pulmonary nodules is of particular importance in sarcoma patients, due to the frequent occurrence of pulmonary metastasis from sarcomas.
Hu, S C-S; Ke, C-L K; Lee, C-H; Wu, C-S; Chen, G-S; Cheng, S-T
Kaposi's sarcoma is a vascular tumour characterized by a proliferation of spindle cells and endothelial cells to form closely arranged slit-like vascular spaces. Currently, the definitive diagnosis of Kaposi's sarcoma relies on histology. The dermoscopic features of Kaposi's sarcoma are not clearly defined in the scientific literature. We seek to evaluate the dermoscopic features of Kaposi's sarcoma and compare them with other vascular tumours. One hundred forty-one lesions from seven patients with histologically proven Kaposi's sarcoma were evaluated using polarized light dermoscopy for the presence of various dermoscopic features. Twenty patients with other vascular tumours were also examined. Dermoscopic examination revealed bluish-reddish coloration (84% of lesions), multicoloured areas showing various colours of the rainbow spectrum (36%), scaly surface (29%), and small brown globules (15%). The 'rainbow pattern' was found in six out of seven patients with Kaposi's sarcoma and was not observed in other vascular tumours. In addition, there was an absence of dermoscopic features specific for other vascular and non-vascular skin tumours, such as well-defined lacunae or structured vascular pattern, in most of the Kaposi's sarcoma lesions. The most frequent dermoscopic patterns in Kaposi's sarcoma were found to be bluish-reddish coloration, the 'rainbow pattern', and scaly surface. The rainbow pattern is a dermoscopic feature which has not been previously described. We propose that dermoscopy, as an adjunct to clinical examination, may enhance accuracy in the preoperative diagnosis of Kaposi's sarcoma.
Amrit Pal Kaur
Full Text Available Endometrial stromal sarcomas (ESS are rare endometrial tumours arising from stroma of endometrium i.e. connective tissue of endometrium rather than glands. Usually a pre-operative diagnosis is difficult. Total abdominal hysterectomy with bilateral salpingo-oophorectomy is main line of treatment. Adjuvant hormone therapy in the form of progesterones, GnRH analogues, aromatase inhibitors are effective for prevention of recurrences as these tumours are invariably positive for oestrogen & progesterone receptors. Surgical excision, radiotherapy, hormone therapy are recommended for recurrences. We report a 52 yrs widow with undifferentiated endometrial stromal sarcoma weighing 3.75 kg with a short history of 3 months diagnosed only after histopathology. [Int J Reprod Contracept Obstet Gynecol 2014; 3(1.000: 276-278
Surov, Alexey; Gottschling, Sebastian; Wienke, Andreas; Meyer, Hans Jonas; Spielmann, Rolf Peter; Dralle, Henning
Different types of malignant tumors can occur within the thyroid. Primary cancer is the most common type of thyroid malignancy. Non-epithelial malignancies can also arise within the thyroid. The aim of the present study was to analyze clinical and radiological characteristics of reported primary thyroid sarcomas (PTS), based on a large sample of cases. The PubMed database was screened for articles from between 1990 and 2014. Overall, 86 articles with 142 patients were identified. Ultrasound evaluation was reported for 36 patients. Data regarding computed tomography of the neck were available for 29 cases. Magnetic resonance imaging was performed for eight patients. The following data were retrieved for the identified sarcomas: localization, size, homogeneity, internal texture, and margin characteristics. In most cases, PTS occurred in patients over 40 years of age, with a peak incidence for the group aged 60-79 years. Angiosarcoma was diagnosed in 29 cases (20.4%), followed by malignant hemangioendothelioma (n=23, 16.3%), malignant fibrous histiocytoma (n=20, 14.1%), leiomyosarcoma (n=16, 11.3%), and fibrosarcoma (n=13, 9.2%). In most patients (n=113, 79.6%), PTS manifested clinically as a painless goiter. On ultrasound, PTS were predominantly mixed hypo-to-hyperechoic in comparison to the normal thyroid tissue. On non-contrast computed tomography, most sarcomas were inhomogeneous hypo-to-hyperdense. On post-contrast magnetic resonance images, most sarcomas showed marked non-homogenous enhancement. In 26.8%, infiltration of the adjacent organs was seen. The trachea or esophagus was affected more frequently in patients with malignant histiocytoma and liposarcoma. Different strategies were used in the treatment of PTS. Our analysis provides clinical and radiological characteristics of PTS. The described features should be taken into consideration in the differential diagnosis of thyroid tumors.
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)
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.)
Kim, Jeong-Ah; Lee, Myung Sook; Choi, Jong-Sun
Objective The study was performed to present the sonographic findings of uterine endometrial stromal sarcoma (ESS). Materials and Methods We conducted a retrospective review of sonographic findings of 10 cases that were diagnosed as uterine ESS. The patients' ages ranged from 25 to 51 years (mean age: 36.1 years). The reviews focused on the location, margin, size, number and echotexture of the lesions. Hysterectomy (n = 9) and myomectomy (n = 1) were performed and a pathologic diagnosis was o...
Evola, Francesco R.; Costarella, Luciano; Pavone, Vito; Caff, Giuseppe; Cannavò, Luca; Sessa, Andrea; Avondo, Sergio; Sessa, Giuseppe
Osteosarcoma is the most frequent malignant bone neoplasm, followed by chondrosarcoma and Ewing sarcoma. The diagnosis of bone neoplasms is generally made through histological evaluation of a biopsy. Clinical and radiological features are also important in aiding diagnosis and to complete the staging of bone cancer. In addition to these, there are several non-specific serological or specific molecular markers for bone neoplasms. In bone tumors, molecular markers increase the accuracy of the d...
Paryani, Nitesh N.; Zlotecki, Robert A.; Swanson, Erika L.; Morris, Christopher G. [Department of Radiation Oncology, University of Florida College of Medicine, Gainesville, FL (United States); Grobmyer, Stephen R.; Hochwald, Steven N. [Department of General Surgery, University of Florida College of Medicine, Gainesville, FL (United States); Marcus, Robert B. [Department of Radiation Oncology, University of Florida College of Medicine, Gainesville, FL (United States); University of Florida Proton Therapy Institute, Jacksonville, FL (United States); Indelicato, Daniel J., E-mail: email@example.com [Department of Radiation Oncology, University of Florida College of Medicine, Gainesville, FL (United States); University of Florida Proton Therapy Institute, Jacksonville, FL (United States)
Purpose: Soft-tissue sarcomas of the retroperitoneum are rare tumors comprising less than 1% of all malignancies. Although surgery continues as the mainstay of treatment, the large size of these tumors coupled with their proximity to critical structures make resection with wide margins difficult to achieve. The role and timing of radiotherapy are controversial. This study updates our institutional experience using multimodality local therapy for resectable retroperitoneal sarcoma and identifies prognostic factors impacting disease control and survival. Methods and Materials: Between 1974 and 2007, 58 patients with nonmetastatic retroperitoneal sarcoma were treated with surgery and radiation at University of Florida. The median age at radiotherapy was 57 years old (range, 18-80 years). Forty-two patients received preoperative radiotherapy and 16 received postoperative radiotherapy. Nineteen patients received 1.8 Gy once daily and 39 patients received 1.2 Gy twice daily. Variables analyzed for prognostic value included age, grade, kidney involvement, histology, de novo versus recurrent presentation, tumor diameter, margin status, radiotherapy sequencing (preoperative vs. postoperative), total radiation dose, fractionation scheme, and treatment era. Results: The 5-year overall survival, cause-specific survival, and local control rates were 49%, 58%, and 62%, respectively. Nearly two-thirds of disease failures involved a component of local progression. On multivariate analysis, only margin status was significantly associated with improved 5-year local control (85%, negative margins; 63%, microscopic positive margins; 0%, gross positive margins; p < 0.0001) and 5-year overall survival (64%, negative margins; 56%, microscopic positive margins; 13%, gross positive margins; p = 0.0012). Thirty-one Grade 3 or greater toxicities were observed in 22 patients, including two treatment-related deaths (3%). Conclusion: For retroperitoneal sarcoma, local control remains a
Full Text Available Kaposi's sarcoma is a malignant disease that originates in the lymphatic endothelium. It has a broad spectrum of clinical manifestations. Its four distinct clinical forms are: classic, endemic, iatrogenic and epidemic Kaposi's sarcoma. In non-HIV-associated Kaposi's sarcoma, the disease is typically limited to the lower extremities, but in immunodeficient patients, it is a multifocal systemic disease. The clinical course of the disease differs among patients, ranging from a single or a few indolent lesions to an aggressive diffuse disease. Advanced Kaposi's sarcoma lesions, typically those on the lower extremities, are often associated with lymphedema. In this paper, we report a case of a patient with a rare form of AIDS-associated Kaposi sarcoma called lymphangiectatic Kaposis's sarcoma.O sarcoma de Kaposi é uma neoplasia originária do endotélio linfatico, que apresenta um amplo espectro de manifestações, com quatro formas clínicas: sarcoma de Kaposi clássico, endêmico, iatrogêncio e epidêmico ou associado ao HIV. Em pacientes imunocompetentes, a doença é tipicamente limitada às extremidades. Porém em pacientes imunideprimidos, o sarcoma de Kaposi é uma doença sistêmica multifocal. Apresenta cursos clínicos diferentes, desde simples lesões cutâneas isoladas até lesões agressivas e difusas, com ou sem envolvimento sistêmico. Lesões avançadas de sarcoma de Kaposi, principalmente as localizadas nas extremidades, podem apresentar linfedema. Neste trabalho, reportamos caso de paciente com forma rara de Sarcoma de Kaposi associado a Aids, chamada de sarcoma de Kaposi linfangiectásico.
Posada García, Celia; García-Cruz, Aranzazu; García-Doval, Ignacio; De La Torre, Carlos; Cruces, Manuel José
Kaposi sarcoma (KS) is a multifocal vascular disease with uncertain histogenesis. It is characterized by clinical and histologic polymorphism. The "lymphangioma-like" variant is very uncommon, accounting for less than 5% of all cases. We report the case of a 76-year-old woman, HIV negative, with a 4-year history of classic Kaposi sarcoma treated with cryotherapy who developed new bullous lesions on her lower extremities. Biopsy revealed histologic findings of lymphangioma-like KS (LLKS), together with areas of classic KS; HHV-8 staining was positive. Diagnosis of LLKS was made and the patient was proposed for radiotherapy. The lymphangioma-like Kaposi sarcoma is a rare morphologic expression of KS characterized by dilated and bizarrely shaped vascular channels lined by flattened endothelium permeating the dermis. "Bulla-like" lesions have been considered as a clinical hallmark of this variant. Its histologic appearance suggests a lymphatic origin of KS and it may resemble other vascular tumors. Findings of areas of typical KS and positive staining for HHV-8 may help to make a definitive diagnosis.
S. P. D'Angelo
Full Text Available Sarcomas are heterogeneous malignant tumors of mesenchymal origin characterized by more than 100 distinct subtypes. Unfortunately, 25–50% of patients treated with initial curative intent will develop metastatic disease. In the metastatic setting, chemotherapy rarely leads to complete and durable responses; therefore, there is a dire need for more effective therapies. Exploring immunotherapeutic strategies may be warranted. In the past, agents that stimulate the immune system such as interferon and interleukin-2 have been explored and there has been evidence of some clinical activity in selected patients. In addition, many cancer vaccines have been explored with suggestion of benefit in some patients. Building on the advancements made in other solid tumors as well as a better understanding of cancer immunology provides hope for the development of new and exciting therapies in the treatment of sarcoma. There remains promise with immunologic checkpoint blockade antibodies. Further, building on the success of autologous cell transfer in hematologic malignancies, designing chimeric antigen receptors that target antigens that are over-expressed in sarcoma provides a great deal of optimism. Exploring these avenues has the potential to make immunotherapy a real therapeutic option in this orphan disease.
Gramolelli, Silvia; Schulz, Thomas F
Kaposi sarcoma (KS) is an unusual vascular tumour caused by an oncogenic-herpesvirus, Kaposi sarcoma-associated herpesvirus (KSHV), also known as human herpesvirus 8 (HHV 8). KS lesions are characterized by an abundant inflammatory infiltrate, the presence of KSHV-infected endothelial cells that show signs of aberrant differentiation, as well as faulty angiogenesis/ vascularization. Here we discuss the molecular mechanisms that lead to the development of these histological features of KS, with an emphasis on the viral proteins that are responsible for their development.
Full Text Available Estudo histopatológico da implantação intracerebral de sarcomas, realizado em ratos (fibrosarcoma e em camundongos (sarcoma 180. A implantação intracerebral de fibrosarcoma desenvolveu, em cerca de 45 dias, neoplasia relativamente bem delimitada, com pequena infiltração do parênquima nervoso adjacente, nunca se propagando a maiores distãncias devido a forte coesão entre as suas células, com grande diferenciação de fibrilas reticulares e de colágeno. Ao contrário, a inoculação do sarcoma 180, principalmente sob a forma ascítica, levou rapidamente a um quadro de forte hipertensão intracraniana, com disseminação das células neoplásticas pelos espaços subaracnoideanos e intraventriculares. Ambas as neoplasias propagavam-se pelos espaços subaracnoideanos e intraventriculares. Ambas as neoplasias propagavam-se pelos espaços perivasculares, porém o faziam de maneira diversa; o sarcoma 180 tinha uma disseminação muito intensa e rápida, que se fazia a longas distâncias, enquanto que o fibrossarcoma somente se disseminava nos vasos próximos à neoplasia em desenvolvimento. Tomando por base observações próprias e outras colhidas na bibliografia especializada, conclui-se que os agentes etiológicos destes tumores exercem sua ação sobre tipos celulares diferentes.Histopathological study of intracerebral implantation of sarcomas, performed in rats (fibrosarcomas and mice (sarcoma 180. The intracerebral implantation of fibrosarcoma has developed in about 45 days a well limited tumor, with little infiltration of the adjacent nervous parenchyma, never streading to longer distances because the strong cohesion between its cells, with great differentiation of reticular fibers and collagen. On the contrary, the innoculation of sarcoma 180, chiefly of the ascitical form, has rapidly lead to a strong intracranial hipertension, with dissemination of neoplastic cells through the subarachnoid and intraventricular spaces. Both
Full Text Available Tumours defined as Ewing sarcoma (ES constitute a group of highly malignant neoplasms that most often affect children and young adults in the first 2 decades of life. The EWS/Fli-1 fusion gene, a product of the translocation t(11;22 (q24; 12, is detected in 95% of ES patients. Recently, it was validated that cells emit a heterogeneous mixture of vesicular, organelle-like structures (microvesicles, MVs into their surroundings including blood and body fluids, and that these MVs contain a selected set of tumor-related proteins and high levels of mRNAs and miRNAs. In this present study, we detected the Ewing sarcoma-specific EWS/Fli-1 mRNA in MVs from the culture medium of ES cell lines carrying t(11;22 (q24; 12. Also, we detected this fusion gene in approximately 40% of the blood samples from mice inoculated with xenografts of TC135 or A673 cells. These findings indicate the EWS/Fli-1 mRNA in MVs might be a new non-invasive diagnostic marker for specific cases of Ewing sarcoma.
Full Text Available Ewing sarcoma is a bone tumour that commonly appears between ages five and 10 in the diaphysis of the long bones and predominantly presents with pain and swelling. The case of an 18-year-old girl who presented with back pain, cough, dyspnea, weakness and fever is described. Chest radiograph showed a homogenous density in the middle and inferior zones of the right hemithorax. Thoracic computed tomography revealed a diffuse pleural effusion and a 6.99 cmx4.45 cm solid mass composed of lobulated, small cystic lesions and calcifications in the right hemithorax. Biochemical analysis of pleural fluid showed hemorrhagic effusion and exudate. A pleural needle biopsy demonstrated solid uniform tumour cells with narrowed cytoplasm, round nuclei and uncertain nucleoli. All of the tumour cell cytoplasms stained with CD99. The pathological examination supported Ewing sarcoma. Three-phase Tc-99m methylene diphosphonate scintigraphy of the whole body showed pathological tracer uptake in a broad area of the eighth costal bone and in smaller areas of the ninth and 10th costal bones. This case is reported because Ewing sarcoma is a rare cause of pleural effusion in clinical practice among younger adults.
The effect of a methylcholanthrene (MCA) sarcoma on the protein status of adult, male, Fischer-344 rats was investigated. Three groups of ten rats were used. One group (TB) was inoculated with MCA sarcoma tissue, control (C) and pair-fed (PF) groups received saline only. Rats were fed a purified (20% casein, 20% fat, 45% sucrose) diet from day 7 post-transplant, when tumors became palpable. Food intake of TB was depressed relative to C at day 15 PT. Animals were killed at day 22 PT. In comparison to C, tumor growth was associated with a decrease in gastrocnemius muscle protein content and an increase in spleen weight and protein content. There was no effect of tumor growth on the weight or protein content of liver, heart, or kidneys. Data from PF animals suggested that decreased gastrocnemius muscle protein content was partly attributable to decreased food intake and partly to tumor growth; the increased spleen protein was due to tumor growth per se. Thus, growth of the MCA sarcoma affects host protein status. Some of these effects are caused partly by hypophagia and partly by tumor growth and others are due to tumor growth per se.
Full Text Available Synovial sarcomas (SS are classified as subgroup of soft tissue sarcomas affecting mainly extremities of young adults. Primary SS of kidney are very rare tumours with poor prognosis. Though they have characteristic histology and immunohistochemistry (IHC due to rarity of incidence it is difficult to diagnose them. Sometimes chromosomal rearrangement studies are required to confirm the diagnosis. We are presenting a case of 41-year-old male who was referred to our cancer centre for evaluation of left renal mass. CT scan of abdomen revealed a large left renal mass encasing the aorta. Biopsy of renal mass revealed poorly differentiated sarcoma and IHC was positive for vimentin, CD99, and BCL2 and negative for AE1, epithelial membrane antigen, and leukocyte common antigen. The patient was clinically inoperable as renal mass was encasing the aorta. So he was subsequently offered palliative chemotherapy in form of ifosfamide and adriamycin. CT abdomen shows partial response after 3 cycles of chemotherapy according to RECIST criteria.
Puls, Florian; Niblett, Angela; Marland, Gillian; Gaston, Czar Louie L; Douis, Hassan; Mangham, D Chas; Sumathi, Vaiyapuri P; Kindblom, Lars-Gunnar
BCOR-CCNB3 fusion transcripts resulting from an X-chromosomal paracentric inversion were recently identified in a series of unclassifiable soft tissue and bone sarcomas with Ewing sarcoma-like morphology. The morphologic and clinical features of these sarcomas are, as yet, not well characterized. Here we describe the clinicopathologic features of 10 cases of BCOR-CCNB3 sarcoma and compare their clinical course with typical Ewing sarcoma. Nine of 10 patients were male, and all were 11 to 18 years of age. Seven tumors were located in the bone and 3 in the deep soft tissues. The histomorphologic spectrum was quite wide, with 7 tumors predominately showing small primitive cell morphology with angulated nuclei simulating so-called atypical Ewing sarcoma and 3 predominately showing spindle cell morphology. Recurrent and metastatic lesions showed increased cellularity and marked pleomorphism. Immunohistochemistry showed expression of CCNB3 (100%), bcl2 (90%), CD99 (60%), and CD117 (60%). Reverse transcription polymerase chain reaction for BCOR-CCNB3 fusion transcripts was positive in all 9 cases, which yielded sufficient extracted RNA. Five- and 10-year survival rates were 75% and 56%, respectively. BCOR-CCNB3 sarcomas located in axial skeleton and soft tissues showed a significantly shorter survival. The Ewing sarcoma overall survival was not statistically different, although there was a trend for longer survival of patients with BCOR-CCNB3 sarcomas in the extremities. In conclusion, this study provides a detailed description of the histologic spectrum, immunohistochemical features, and clinical characteristic of BCOR-CCNB3 sarcoma justifying distinction from Ewing sarcoma with its typical EWS/FUS-ETS translocations. Ideally immunohistochemistry is used in combination with reverse transcription polymerase chain reaction for definitive diagnosis.
Borinstein, Scott C; Beeler, Natalie; Block, John J; Gorlick, Richard; Grohar, Patrick; Jedlicka, Paul; Krailo, Mark; Morris, Carol; Phillips, Sharon; Siegal, Gene P; Lawlor, Elizabeth R; Lessnick, Stephen L
Outcomes for patients with metastatic and recurrent Ewing sarcoma remain poor and a better understanding of the biology of this malignancy is critical to the development of prognostic biomarkers and novel therapies. Therefore, the Children's Oncology Group (COG) has created tissue banking protocols designed to collect high quality, clinically annotated, tumor specimens that can be distributed to researchers to perform basic science and correlative investigation. Data from the COG Ewing sarcoma tissue banking protocols AEWS02B1 and its successor study AEWS07B1 were reviewed in this study. Six-hundred and thirty five patients were enrolled on AEWS02B1 and 396 patients have had tissue submitted to AEWS07B1. The average age of participation was 13.2 years. About 86% were less than 19 years old and only 6% were greater than 21 years of age at diagnosis. When compared to SEER data, approximately 18% of all cases and only 8% of all patients >20 years old diagnosed with Ewing sarcoma annually in the United States have had tumor banked. The majority of participants submitted formalin fixed, paraffin embedded, primary tumor and blood samples. In total, fresh frozen tissue was submitted for only 29% of cases. Only seven metastatic tumor samples have been collected. Although the COG has been successful in collecting tumor samples from patients newly diagnosed with Ewing sarcoma, fresh frozen tumor specimens from primary and metastatic disease are critically needed, especially from young adult patients, in order to conduct high quality basic science and translational research investigation with a goal of developing better treatments.
Borinstein, Scott C.; Beeler, Natalie; Block, John J.; Gorlick, Richard; Grohar, Patrick; Jedlicka, Paul; Krailo, Mark; Morris, Carol; Phillips, Sharon; Siegal, Gene P.; Lawlor, Elizabeth R.; Lessnick, Stephen L.
Outcomes for patients with metastatic and recurrent Ewing sarcoma remain poor and a better understanding of the biology of this malignancy is critical to the development of prognostic biomarkers and novel therapies. Therefore, the Children’s Oncology Group (COG) has created tissue banking protocols designed to collect high quality, clinically annotated, tumor specimens that can be distributed to researchers to perform basic science and correlative investigation. Data from the COG Ewing sarcoma tissue banking protocols AEWS02B1 and its successor study AEWS07B1 were reviewed in this study. Six-hundred and thirty five patients were enrolled on AEWS02B1 and 396 patients have had tissue submitted to AEWS07B1. The average age of participation was 13.2 years. About 86% were less than 19 years old and only 6% were greater than 21 years of age at diagnosis. When compared to SEER data, approximately 18% of all cases and only 8% of all patients >20 years old diagnosed with Ewing sarcoma annually in the United States have had tumor banked. The majority of participants submitted formalin fixed, paraffin embedded, primary tumor and blood samples. In total, fresh frozen tissue was submitted for only 29% of cases. Only seven metastatic tumor samples have been collected. Although the COG has been successful in collecting tumor samples from patients newly diagnosed with Ewing sarcoma, fresh frozen tumor specimens from primary and metastatic disease are critically needed, especially from young adult patients, in order to conduct high quality basic science and translational research investigation with a goal of developing better treatments. PMID:23519678
Manara, Maria Cristina; Garofalo, Cecilia; Ferrari, Stefano; Belfiore, Antonino; Scotlandi, Katia
Drug "repurposing" is the process of finding new therapeutic indications for existing drugs, and can be considered as a more efficient and realistic strategy for the design of therapies against rare diseases than the current efforts to develop targeted-drugs. In this review, we explore the difficulties related to the identification and development of tailored therapies for individual patients with sarcomas, which are relatively rare diseases characterized by an extreme genetic and histologic variability. Overall, sarcomas comprise about 1% of all adult tumors and 10% of pediatric cancers. They are conventionally divided in bone and soft-tissue sarcomas, considering their site of origin. However, each group is highly heterogeneous and recent global characterization of their genetic alterations has clearly identified the existence of peculiarities that render these group of tumors even more "orphan" for pharmaceutical companies to develop and market specific- targeted drugs. The present review highlights key examples of molecular targets identification in bone sarcomas, reexamining the history of insulin-like growth factor receptor (IGF-IR) and its role in physiology and in cancer as well as developments regarding phase I to II clinical trials of agents directed against this receptor. The IGF system is quite complex, with many players in the field. Insulin receptor function in cancer cells has certainly been underestimated, but also little attention was paid to the type of ligands that are mainly involved in each tumor type. Strategies considering the system in its complex are encouraged and, in this context, drugs aimed at reducing circulating insulin levels, such as metformin, should receive attention as potential anti-cancer agents.
Issels, Rolf D; Lindner, Lars H; Verweij, Jaap;
The optimum treatment for high-risk soft-tissue sarcoma (STS) in adults is unclear. Regional hyperthermia concentrates the action of chemotherapy within the heated tumour region. Phase 2 studies have shown that chemotherapy with regional hyperthermia improves local control compared with chemother...
Mesiha, Mena; Bauer, Thomas; Andrish, Jack
Iliotibial band friction syndrome is a common entity that is often quickly diagnosed in orthopedic clinics. However, synovial sarcoma is an elusive clinical entity that appears around many joints with variable presentations. This case report is an example of a patient with a classic presentation of iliotibial band friction syndrome that was diagnosed as a synovial sarcoma on further investigation.
Fukuda, Wakako; Morohashi, Satoko; Fukuda, Ikuo
Pulmonary artery intimal sarcoma is a rare tumour and the diagnosis is often delayed. We report the case of a woman with a primary pulmonary artery intimal sarcoma who presented with massive pulmonary embolism. The definitive diagnosis was elucidated after the patient's death by autopsy specimen. We discuss the diagnosis and lessons learned from this case.
Weatherby, R.P.; Dahlin, D.C.; Ivins, J.C.
Postradiation sarcoma of bone is an uncommon but serious sequela of radiation therapy. Seventy-eight Mayo Clinic patients have been treated for sarcomas arising in irradiated bones. They received their initial radiotherapy for a wide variety of nonneoplastic and neoplastic conditions, both benign and malignant. Thirty-five sarcomas arose in bone that was normal at the time of radiotherapy, and 43 arose in irradiated preexisting osseous lesions. The latent period between radiotherapy and diagnosis of sarcoma averaged 14.3 years. Ninety percent of the postradiation sarcomas were either osteosarcomas or fibrosarcomas; chondrosarcoma, malignant (fibrous) histiocytoma, malignant lymphoma, Ewing's tumor, and metastasizing chondroblastoma also occurred. Prompt radical surgery, when feasible, is usually the treatment of choice for the sarcoma. About 30% of patients with sarcomas of the extremities or craniofacial bones survived 5 years without recurrence; there were no disease-free survivors among patients with tumors of the vertebral column, pelvis, or shoulder girdle. The low risk of sarcoma following radiotherapy for the treatment of cancer should not be a contraindication to its use in these patients; however, radiation therapy for benign bone tumors should be reserved for lesions that are not amenable to surgical treatment. An unusual case is also reported herein in which a fibrosarcoma was discovered in the humerus of a patient who had received radiotherapy 55 years previously for a verified osteosarcoma in the same site.
Conclusions and importance: The authors postulate emboli from the left atrial sarcoma entered systemic circulation and subsequently caused brief episodes of transient occlusion to retinal, ophthalmic and/or ciliary arteries leading to momentary retinal hypoxia. We believe this is a novel finding, previously unreported in the literature, of transient embolic occlusion without permanent visual sequelae due to a malignant primary cardiac pleomorphic sarcoma.
Kenneth R. Gundle
Full Text Available In addition to patient reported outcome measures, accelerometers may provide useful information on the outcome of sarcoma patients treated with limb salvage. The StepWatch (SW Activity Monitor (SAM is a two-dimensional accelerometer worn on the ankle that records an objective measure of walking performance. The purpose of this study was to validate the SW in a cross-sectional population of adult patients with lower extremity sarcoma treated with limb salvage. The main outcome was correlation of total steps with the Toronto Extremity Salvage Score (TESS. In a sample of 29 patients, a mean of 12 days of SW data was collected per patient (range 6–16, with 2767 average total steps (S.D. 1867; range 406–7437. There was a moderate positive correlation between total steps and TESS (r=0.56, P=0.002. Patients with osseous tumors walked significantly less than those with soft tissue sarcoma (1882 versus 3715, P<0.01. This study supports the validity of the SAM as an activity monitor for the objective assessment of real world physical function in sarcoma patients.
Sato, Yukiko; Tanaka, Hiroko; Sasaki, Toru; Kawabata, Kazuyoshi; Mitani, Hiroki; Yonekawa, Hiroyuki; Fukushima, Hirofumi; Shimbashi, Wataru
Ectopic hamartomatous thymoma (EHT) is an extremely rare benign tumor. EHTs are difficult to differentiate from sarcomas, especially synovial sarcomas. We encountered two cases of EHT that were referred from other hospitals because sarcoma was suspected. In these cases, fusion gene detection via polymerase chain reaction or fluorescence in situ hybridization was useful for differentiating EHT from synovial sarcoma. EHT requires accurate diagnosis before surgery to avoid excessive treatment. Both tumor location and the presence of fat inside the tumor are important imaging findings for EHT, and confirmation of spindle cells, epithelial cells, and mature adipose cells in the tumor is an important pathological finding. It is important to exclude synovial sarcoma from the differential diagnosis via fusion gene analysis. PMID:28168073
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.
Min, Li; Shen, Jacson; Tu, Chongqi; Hornicek, Francis; Duan, Zhenfeng
Better diagnostic biomarkers and therapeutic options are still necessary for patients with sarcomas due to the current limitations of diagnosis and treatment. Exosomes are small extracellular membrane vesicles that are released by various cells and are found in most body fluids. Tumor-derived exosomes have been proven to mediate tumorigenesis, intercellular communication, microenvironment modulation, and metastasis in different cancers, including in sarcomas. Recently, exosomes have been considered as potential biomarkers for sarcoma diagnosis and prognosis, and as possible targets for sarcoma therapy. Moreover, due to their specific cell tropism and bioavailability, exosomes can also be engineered as vehicles for drug delivery. In this review, we discuss recent advances in the roles of tumor-derived exosomes in sarcoma and their potential clinical applications.
Chu Kathryn M
Full Text Available Abstract Background AIDS-associated Kaposi's sarcoma is an important, life-threatening opportunistic infection among people living with HIV/AIDS in resource-limited settings. In western countries, the introduction of combination antiretroviral therapy (cART and new chemotherapeutic agents has resulted in decreased incidence and improved prognosis of AIDS-associated Kaposi's sarcoma. In African cohorts, however, mortality remains high. In this study, we describe disease characteristics and risk factors for mortality in a public sector HIV programme in South Africa. Methods We analysed data from an observational cohort study of HIV-infected adults with AIDS-associated Kaposi's sarcoma, enrolled between May 2001 and January 2007 in three primary care clinics. Paper records from primary care and tertiary hospital oncology clinics were reviewed to determine the site of Kaposi's sarcoma lesions, immune reconstitution inflammatory syndrome stage, and treatment. Baseline characteristics, cART use and survival outcomes were extracted from an electronic database maintained for routine monitoring and evaluation. Cox regression was used to model associations with mortality. Results Of 6292 patients, 215 (3.4% had AIDS-associated Kaposi's sarcoma. Lesions were most commonly oral (65% and on the lower extremities (56%. One quarter of patients did not receive cART. The mortality and lost-to-follow-up rates were, respectively, 25 (95% CI 19-32 and eight (95% CI 5-13 per 100 person years for patients who received cART, and 70 (95% CI 42-117 and 119 (80-176 per 100 person years for patients who did not receive cART. Advanced T stage (adjusted HR, AHR = 5.3, p Patients with AIDS-associated Kaposi's sarcoma presented with advanced disease and high rates of mortality and loss to follow up. Risk factors for mortality included advanced Kaposi's sarcoma disease and lack of chemotherapy use. Contributing factors to the high mortality for patients with AIDS
Baldini, Elizabeth H., E-mail: firstname.lastname@example.org [Department of Radiation Oncology, Dana-Farber Cancer Institute, Brigham and Women' s Hospital, Boston, Massachusetts (United States); Abrams, Ross A. [Department of Radiation Oncology, Rush University Medical Center, Chicago, Illinois (United States); Bosch, Walter [Department of Radiation Oncology, Washington University, St. Louis, Missouri (United States); Roberge, David [Department of Radiation Oncology, Centre Hospitalier de l' Universite de Montreal, Montreal, Quebec (Canada); Haas, Rick L.M. [Department of Radiotherapy, Netherlands Cancer Institute, Amsterdam (Netherlands); Catton, Charles N. [Department of Radiation Oncology, Princess Margaret Cancer Centre, Toronto, Ontario (Canada); Indelicato, Daniel J. [Department of Radiation Oncology, University of Florida Medical Center, Jacksonville, Florida (United States); Olsen, Jeffrey R. [Department of Radiation Oncology, Washington University, St. Louis, Missouri (United States); Deville, Curtiland [Department of Radiation Oncology, University of Pennsylvania, Philadelphia, Pennsylvania (United States); Chen, Yen-Lin [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); Finkelstein, Steven E. [Translational Research Consortium, 21st Century Oncology, Scottsdale, Arizona (United States); DeLaney, Thomas F. [Department of Radiation Oncology, Massachusetts General Hospital, Boston, Massachusetts (United States); Wang, Dian [Department of Radiation Oncology, Rush University Medical Center, Chicago, Illinois (United States)
Purpose: The purpose of this study was to evaluate the variability in target volume and organ at risk (OAR) contour delineation for retroperitoneal sarcoma (RPS) among 12 sarcoma radiation oncologists. Methods and Materials: Radiation planning computed tomography (CT) scans for 2 cases of RPS were distributed among 12 sarcoma radiation oncologists with instructions for contouring gross tumor volume (GTV), clinical target volume (CTV), high-risk CTV (HR CTV: area judged to be at high risk of resulting in positive margins after resection), and OARs: bowel bag, small bowel, colon, stomach, and duodenum. Analysis of contour agreement was performed using the simultaneous truth and performance level estimation (STAPLE) algorithm and kappa statistics. Results: Ten radiation oncologists contoured both RPS cases, 1 contoured only RPS1, and 1 contoured only RPS2 such that each case was contoured by 11 radiation oncologists. The first case (RPS 1) was a patient with a de-differentiated (DD) liposarcoma (LPS) with a predominant well-differentiated (WD) component, and the second case (RPS 2) was a patient with DD LPS made up almost entirely of a DD component. Contouring agreement for GTV and CTV contours was high. However, the agreement for HR CTVs was only moderate. For OARs, agreement for stomach, bowel bag, small bowel, and colon was high, but agreement for duodenum (distorted by tumor in one of these cases) was fair to moderate. Conclusions: For preoperative treatment of RPS, sarcoma radiation oncologists contoured GTV, CTV, and most OARs with a high level of agreement. HR CTV contours were more variable. Further clarification of this volume with the help of sarcoma surgical oncologists is necessary to reach consensus. More attention to delineation of the duodenum is also needed.
Kollár, A; Jones, R L; Stacchiotti, S; Gelderblom, H; Guida, M; Grignani, G; Steeghs, N; Safwat, A; Katz, D; Duffaud, F; Sleijfer, S; van der Graaf, W T; Touati, N; Litière, S; Marreaud, S; Gronchi, A; Kasper, B
Pazopanib is a multitargeted tyrosine kinase inhibitor approved for the treatment of patients with selective subtypes of advanced soft tissue sarcoma (STS) who have previously received standard chemotherapy including anthracyclines. Data on the efficacy in vascular sarcomas are limited. The main objective of this study was to investigate the activity of pazopanib in vascular sarcomas. A retrospective study of patients with advanced vascular sarcomas, including angiosarcoma (AS), epithelioid hemangioendothelioma (HE) and intimal sarcoma (IS) treated with pazopanib in real life practice at EORTC centers as well as patients treated within the EORTC phase II and III clinical trials (62043/62072) was performed. Patient and tumor characteristics were collected. Response was assessed according to RECIST 1.1. and survival analysis was performed. Fifty-two patients were identified, 40 (76.9%), 10 (19.2%) and two (3.8%) with AS, HE and IS, respectively. The response rate was eight (20%), two (20%) and two (100%) in the AS, HE and IS subtypes, respectively. There was no significant difference in response rate between cutaneous and non-cutaneous AS and similarly between radiation-associated and non-radiation-associated AS. Median progression-free survival (PFS) and median overall survival (OS; from commencing pazopanib) were three months (95% CI 2.1-4.4) and 9.9 months (95% CI 6.5-11.3) in AS, respectively. The activity of pazopanib in AS is comparable to its reported activity in other STS subtypes. In this study, the activity of pazopanib was similar in cutaneous/non-cutaneous and in radiation/non-radiation-associated AS. In addition, pazopanib showed promising activity in HE and IS, worthy of further evaluation.
Guray Togral; Murat Arikan; Elif Aktas; Safak Gungor
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.
Baheti, Akahay D. [Dept. of Radiology, Tata Memorial Centre, Mumbai (India); Tirumani, Harika [Dept. of Radiology, University of Arkansas for Medical Sciences, Little Rock (United States); O' Neill, Alibhe; Jagannathan, Jyothi P. [Dept. of Imaging, Dana-Farber Cancer Institute, Boston (United States)
Non-gastrointestinal stromal tumor (GIST) soft tissue sarcomas (STSs) are a heterogeneous group of neoplasms whose classification and management continues to evolve with better understanding of their biologic behavior. The 2013 World Health Organization (WHO) has revised their classification based on new immunohistochemical and cytogenetic data. In this article, we will provide a brief overview of the revised WHO classification of soft tissue tumors, discuss in detail the radiology and management of the two most common adult non-GIST STS, namely liposarcoma and leiomyosarcoma, and review some of the emerging histology-driven targeted therapies in non-GIST STS, focusing on the role of the radiologist.
Full Text Available Abstract This review provides a comprehensive overview of the broad clinicopathologic spectrum of cutaneous Kaposi sarcoma (KS lesions. Variants discussed include: usual KS lesions associated with disease progression (i.e. patch, plaque and nodular stage; morphologic subtypes alluded to in the older literature such as anaplastic and telangiectatic KS, as well as several lymphedematous variants; and numerous recently described variants including hyperkeratotic, keloidal, micronodular, pyogenic granuloma-like, ecchymotic, and intravascular KS. Involuting lesions as a result of treatment related regression are also presented.
Malagón Gutiérrez, Sandra Ximena; Chimenos Küstner, Eduardo
La neoplasia más común asociada al VIH es el sarcoma de Kaposi (SK). Esta puede ser superficialmente mucocutánea o internamente visceral, apareciendo frecuentemente en la cavidad oral. Este tumor se ha relacionado con una proliferación atípica de estructuras vasculares, la cual haya sido inducida por un agente angiogénico estimulado a su vez por la infección retroviral. Este artículo es una revisión bibliográfica sobre esta lesión, la cual representa una patología importante para la profesión...
Azulay Rubem David
Full Text Available Acroangiodermatite é enfermidade rara, caracterizada por lesões eritêmato-violáceas bem delimitadas que acometem pernas e pés com aspecto semelhante ao do sarcoma de Kaposi. É relatado o caso de paciente do sexo feminino, de 57 anos, com início súbito de lesões eritêmato-violáceas nas pernas sem outras alterações. O caso acrescenta aprendizado por sua dificuldade diagnóstica e reafirma a importância da imuno-histoquímica. Trata-se da publicação do primeiro caso brasileiro.
Male construction worker in his early twenties with a middle school education has been hospitaliaed and refuses to be discharged due to feeling he will die without help. Patient presents with panic,worry,anxiety,feelings of impending doom,fear of death,insecurity,and tactile sensations of someone repeatedly cutting his face. His prior mental health history includes anti - anxiety medications which had no effect and electroconvulsive therapy,with only short - term results. For several years,he was a substance abuser but has maintained sobriety for the past year. Previously,while stressed or under the influence of drugs,he experienced more severe hallucinations. Patient’s diagnosis is Unspecified Psychotic Disorder with substance abuse in remission,not severe enough to meet the criteria for schiaophrenia. Antipsychotic medication and continued drug abstinence is indicated. Patient is further advised of the long - term side effects of prolonged drug use and recommended to use caution while operating heavy machinery while taking anti - dopamine medication,which affects cognitive and motor skills.%一位男性建筑工人，20岁出头，中学学历，目前正在住院治疗，因为害怕在得不到救助的时候死掉，所以拒绝出院。患者反复表现出惊恐症状、担心自己在劫难逃、害怕死亡并且脸上有刀割感。他先前使用抗焦虑药物治疗无效，而使用电抽搐治疗获得短期疗效。数年前，他是毒品滥用者，在使用毒品时出现过严重的幻觉，现已戒毒5年。医生的诊断是“未特定的精神病性障碍，伴物质滥用缓解期”，患者的严重程度还没有达到精神分裂症的诊断标准。在干预措施上，给患者处方抗精神病药并预防再次接触毒品，并叮嘱患者在工作中操作重型机械时谨防事故，因为服用阻断多巴胺受体的药物会影响其认知和运动技能。
Meireles, Paula; Albuquerque, Gabriela; Vieira, Mariana; Foia, Severiano; Ferro, Josefo; Carrilho, Carla; Lunet, Nuno
Kaposi sarcoma is expressed in four clinical variants, all associated with human herpes virus type 8 infection, namely, classic, endemic, immunosuppression-related and AIDS-related. The latter currently accounts for most of the burden of Kaposi sarcoma in sub-Saharan Africa, reflecting the frequency of HIV infection and its management. We aimed to estimate the incidence of Kaposi sarcoma in Mozambique and in its provinces. We estimated the number of incident cases of Kaposi sarcoma by adding up the expected number of endemic and AIDS-related cases. The former were estimated from the rates observed in Kyandondo, Uganda (1960-1971). The latter were computed from the number of AIDS-related deaths in each region, assuming that the ratio between the AIDS-related Kaposi sarcoma incident cases and the number of AIDS-related deaths observed in the city of Beira applies to all regions. A total of 3862 Kaposi sarcoma cases were estimated to have occurred in Mozambique in 2007, mostly AIDS-related, in the age group 25-49 years, and in provinces from South/Centre. The age-standardized incidence rates were 36.1/100 000 in men and 11.5/100 000 in women, with a more than three-fold variation across provinces. We estimated a high incidence of Kaposi sarcoma in Mozambique, along with large regional differences. These results can be used to improve disease management and to sustain political decisions on health policies.
Karlisch, C; Harati, K; Chromik, A M; Bulut, D; Klein-Hitpass, L; Goertz, O; Hirsch, T; Lehnhardt, M; Uhl, W; Daigeler, A
Soft tissue sarcomas (STS) are a heterogeneous group of malignant tumours representing 1% of all malignancies in adults. Therapy for STS should be individualised and multimodal, but complete surgical resection with clear margins remains the mainstay of therapy. Disseminated soft tissue sarcoma still represents a therapeutic dilemma. Commonly used chemotherapeutic agents such as doxorubicin and ifosfamide have proven to be effective in fewer than 30% in these cases. Therefore, we tested the apoptotic and anti-proliferative in vitro effects of TNF-related apoptosis-inducing ligand (TRAIL) and taurolidine (TRD) on rhabdomyosarcoma (A-204), leiomyosarcoma (SK-LMS-1) and epithelioid cell sarcoma (VA-ES-BJ) cell lines. Viability, apoptosis and necrosis were quantified by FACS analysis (propidium iodide/Annexin V staining). Gene expression was analysed by DNA microarrays and the results validated for selected genes by rtPCR. Protein level changes were documented by western blot analysis. Cell proliferation was analysed by BrdU ELISA assay. The single substances TRAIL and TRD significantly induced apoptotic cell death and decreased proliferation in rhabdomyosarcoma and epithelioid cell sarcoma cells. The combined use of TRAIL and TRD resulted in a synergistic apoptotic effect in all three cell lines, especially in rhabdomyosarcoma cells leaving 18% viable cells after 48 h of incubation (p<0.05). Analysis of the differentially regulated genes revealed that TRD and TRAIL influence apoptotic pathways, including the TNF-receptor associated and the mitochondrial pathway. Microarray analysis revealed remarkable expression changes in a variety of genes, which are involved in different apoptotic pathways and cross talk to other pathways at multiple levels. This in vitro study demonstrates that TRAIL and TRD synergise in inducing apoptosis and inhibiting proliferation in different human STS cell lines. Effects on gene expression differ relevantly in the sarcoma entities. These
Stefanie W. Leacock
Ewing’s sarcoma, a malignant bone tumor of children and young adults, is a member of the small-round-blue-cell tumor family. Ewing’s sarcoma family tumors (ESFTs, which include peripheral primitive neuroectodermal tumors (PNETs, are characterized by chromosomal translocations that generate fusions between the EWS gene and ETS-family transcription factors, most commonly FLI1. The EWS-FLI1 fusion oncoprotein represents an attractive therapeutic target for treatment of Ewing’s sarcoma. The cell of origin of ESFT and the molecular mechanisms by which EWS-FLI1 mediates tumorigenesis remain unknown, and few animal models of Ewing’s sarcoma exist. Here, we report the use of zebrafish as a vertebrate model of EWS-FLI1 function and tumorigenesis. Mosaic expression of the human EWS-FLI1 fusion protein in zebrafish caused the development of tumors with histology strongly resembling that of human Ewing’s sarcoma. The incidence of tumors increased in a p53 mutant background, suggesting that the p53 pathway suppresses EWS-FLI1-driven tumorigenesis. Gene expression profiling of the zebrafish tumors defined a set of genes that might be regulated by EWS-FLI1, including the zebrafish ortholog of a crucial EWS-FLI1 target gene in humans. Stable zebrafish transgenic lines expressing EWS-FLI1 under the control of the heat-shock promoter exhibit altered embryonic development and defective convergence and extension, suggesting that EWS-FLI1 interacts with conserved developmental pathways. These results indicate that functional targets of EWS-FLI1 that mediate tumorigenesis are conserved from zebrafish to human and provide a novel context in which to study the function of this fusion oncogene.
Full Text Available Purpose. Relatively few results are available in the literature about the radiation response of unresectable sarcomas in relation to their histology. Therefore, an attempt was made to summarize the present situation. Materials and methods. This report is based on a review of the literature and the author's own experience. Adult-type soft tissue sarcomas, chondrosarcomas, and chordomas were analyzed. Radioresponse was mainly associated with the degree of tumor shrinkage, that is, objective responses. Histopathologic responses, that is, the degree of necrosis, are only discussed in relation to radiation treatment reports of soft tissue sarcomas as a group. Results. Radiation therapy alone leads to major responses in about 50% of lipo-, fibro-, leiomyo-, or chondrosarcomas. The response rate is less than 50% in malignant fibrous histiocytomas, synovial, neurogenic, and other rare soft tissue sarcomas. The response rates may increase up to 75% through the addition of radiosensitizers such as halogenated pyrimidines or razoxane, or by the use of high-LET irradiation. Angiosarcomas become clearly more responsive if biologicals, angiomodulating, and/or tubulin affinic substances are given together with radiation therapy. Razoxane is able to increase the duration and quality of responses even in difficult-to-treat tumors like chondrosarcomas or chordomas. Conclusions. The available data demonstrate that the radioresponsiveness of sarcomas is very variable and dependent on histology, kind of radiation, and various concomitantly given drugs. The rate of complete sustained remissions by radiation therapy alone or in combination with drugs is still far from satisfactory although progress has been made through the use of sensitizing agents.
Purpose. Relatively few results are available in the literature about the radiation response of unresectable sarcomas in relation to their histology. Therefore, an attempt was made to summarize the present situation. Materials and methods. This report is based on a review of the literature and the author's own experience. Adult-type soft tissue sarcomas, chondrosarcomas, and chordomas were analyzed. Radioresponse was mainly associated with the degree of tumor shrinkage, that is, objective responses. Histopathologic responses, that is, the degree of necrosis, are only discussed in relation to radiation treatment reports of soft tissue sarcomas as a group. Results. Radiation therapy alone leads to major responses in about 50% of lipo-, fibro-, leiomyo-, or chondrosarcomas. The response rate is less than 50% in malignant fibrous histiocytomas, synovial, neurogenic, and other rare soft tissue sarcomas. The response rates may increase up to 75% through the addition of radiosensitizers such as halogenated pyrimidines or razoxane, or by the use of high-LET irradiation. Angiosarcomas become clearly more responsive if biologicals, angiomodulating, and/or tubulin affinic substances are given together with radiation therapy. Razoxane is able to increase the duration and quality of responses even in difficult-to-treat tumors like chondrosarcomas or chordomas. Conclusions. The available data demonstrate that the radioresponsiveness of sarcomas is very variable and dependent on histology, kind of radiation, and various concomitantly given drugs. The rate of complete sustained remissions by radiation therapy alone or in combination with drugs is still far from satisfactory although progress has been made through the use of sensitizing agents.
Lucas K. Vitzthum
Full Text Available Head and neck soft tissue sarcomas (HNSTSs are rare and heterogeneous cancers in which radiation therapy (RT has an important role in local tumor control (LC. The purpose of this study was to evaluate outcomes and patterns of treatment failure in patients with HNSTS treated with RT. A retrospective review was performed of adult patients with HNSTS treated with RT from January 1, 1998, to December 31, 2012. LC, locoregional control (LRC, disease-free survival (DFS, overall survival (OS, and predictors thereof were assessed. Forty-eight patients with HNSTS were evaluated. Five-year Kaplan-Meier estimates of LC, LRC, DFS, and OS were 87, 73, 63, and 83%, respectively. Angiosarcomas were found to be associated with worse LC, LRC, DFS, and OS. Patients over the age of 60 had lower rates of DFS. HNSTSs comprise a diverse group of tumors that can be managed with various treatment regimens involving RT. Angiosarcomas have higher recurrence and mortality rates.
Full Text Available Ewing sarcoma (EWS is a tumor of the bone and soft tissue that primarily affects adolescents and young adults. With current therapies, 70% of patients with localized disease survive, but patients with metastatic or recurrent disease have a poor outcome. We found that EWS cell lines are defective in DNA break repair and are sensitive to PARP inhibitors (PARPis. PARPi-induced cytotoxicity in EWS cells was 10- to 1,000-fold higher after administration of the DNA-damaging agents irinotecan or temozolomide. We developed an orthotopic EWS mouse model and performed pharmacokinetic and pharmacodynamic studies using three different PARPis that are in clinical development for pediatric cancer. Irinotecan administered on a low-dose, protracted schedule previously optimized for pediatric patients was an effective DNA-damaging agent when combined with PARPis; it was also better tolerated than combinations with temozolomide. Combining PARPis with irinotecan and temozolomide gave complete and durable responses in more than 80% of the mice.
Soft-tissue sarcomas (STS) account for 1% of adult and 7% of pediatric malignancies. Histopathology and classification of these rare tumors requires further refinements. The aim of this paper is to describe the current incidence and survival of STS from 1994 to 2012 in Ireland and compare these with comparably coded international published reports. This is a retrospective, population study based on the data from the National Cancer Registry of Ireland (NCRI). Incidence and relative survival rates for STS in Ireland were generated. Incidence of STS based on gender, age and anatomical location was examined. Annual mean incidence rate (European Age Standardized) in Ireland between 1994 and 2012 was 4.48 ± 0.15 per 100,000 person-years. The overall relative 5-year survival rate of STS for the period 1994-2011 in Ireland was 56%, which was similar to that reported in the U.K. but lower than in most of Europe and U.S.A. Survival rate fluctuated over the period examined, declining slightly in females but showing an increase in males. STS incidence trends in Ireland were comparable to international reports. Survival trends of STS were significantly different between Ireland and other European countries, requiring further study to understand causation.
Boisseau-Garsaud, A M; Vergier, B; Beylot-Barry, M; Nastasel-Menini, F; Dubus, P; de Mascarel, A; Eghbali, H; Beylot, C
A 28-year-old man presented with a histiocytic sarcoma of a very uncommon origin, as it had developed for several years like a benign cutaneous histiocytosis resembling generalized eruptive histiocytoma before becoming acute, with nodal and massive pulmonary involvement. Despite various chemotherapies, the patient died within 8 months. Skin biopsies showed histiocytic proliferation in the dermis and node biopsies showed histiocytic proliferation with a sinusoidal pattern. Immunohistochemical analysis, performed on paraffin-embedded sections, demonstrated strong labeling of tumoral cells for CD68 and moderate labeling for CD3 and CD4. CD30 labeling was negative. S-100 protein was positive on a Langerhans' cell reactive subpopulation. Electron microscopy confirmed the histiocytic nature of malignant cells and showed cytoplasmic inclusions such as regularly laminated bodies, dense bodies and pleomorphic inclusions. No Birbeck granules were seen. A gene rearrangement study of T-cell receptor gamma and immunoglobulin heavy chain genes showed a germline configuration. Histiocytic sarcoma is an extremely rare true histiocytic malignancy, the existence of which has been recently debated since it has often been mistaken in the past for large cell lymphomas. Such a deceptive onset as benign cutaneous histiocytosis has not been described in the literature to our knowledge.
Shobha S. Pillai
Full Text Available Endometrial Stromal Sarcoma (ESS is a rare slow growing tumour of mesodermal origin arising from the stroma of the endometrium and accounting for less than 1% of all uterine cancers. It is characterized by late recurrences and distant metastases. This report presents a case of ESS in a 40 year old nulliparous woman who had a myomectomy for a clinically suspected Leiomyoma uterus in a local hospital. The histopathological examination of the specimen revealed ESS and the patient was referred to our tertiary institute. Here after investigations including a CT scan which also revealed pulmonary metastases, patient underwent Modified Radical Hysterectomy with Bilateral Salpingo-oophorectomy with pelvic lymph node sampling. Histopathological Examination of the uterine specimen confirmed the diagnosis. The patient was given the option of referral to a thoracic surgeon for resection of the isolated lung metastasis, but she refused this and opted instead for hormone therapy which she is presently undergoing. ESS is a very rare tumour often presenting with clinical and examination findings suggestive of leiomyoma of the uterus and hence misdiagnosed. In cases of rapidly growing tumours and suspicious radiological features, suspect sarcoma and initiate timely diagnosis and proper treatment. Recommended long-term follow up in view of late recurrences. [Int J Reprod Contracept Obstet Gynecol 2014; 3(3.000: 812-815
Full Text Available Many years ago the treatment of sarcoma was radiotherapy up to 2000-4000 rad. This treatment was very complicated, due to producing neoplasm after radiotherapy. By this method of treatment of osteosarcoma, the rate of survival became about 20% (two years. The second method of treatment was chemotherapy for a period of 2-5 weeks that amputation was performed afterwards. By chemotherapy, the rate of being alive reached up to 25-27% (five years. Right now, the best treatment for sarcoma is limb salvage. In our report, the chance of being alive in chondrosarcoma was about four years. This was nearly the same as that of the other institutes in the world especially in America, Europe, and Japan. The rate of recurrence was also more than that from different parts of the world. The survival rate in osteosarcomatic patients was about two years less for males the females, and it was more in tall people than short ones. The survival rate of the patients with giant cell tumor was more than osteosarcoma up to five years, and it has no recurrence or metastasis
Kim, Jeong Ah; Lee, Myung Sook; Choi, Jong Sun [Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)
The study was performed to present the sonographic findings of uterine endometrial stromal sarcoma (ESS). We conducted a retrospective review of sonographic findings of 10 cases that were diagnosed as uterine ESS. The patients ages ranged from 25 to 51 years (mean age: 36.1 years). The reviews focused on the location, margin, size, number and echotexture of the lesions. Hysterectomy (n = 9) and myomectomy (n = 1) were performed and a pathologic diagnosis was obtained in all cases. The masses were located in the uterine wall (n = 6), or they presented as a polypoid mass protruding into the endometrial cavity from the myometrium (n = 3) or as a central cavity mass (n = 1). The lesion margins were smooth (n = 5), ill defined (n = 2), or smooth with partially nodular extensions (n = 3). The maximal mass length was 38 mm to 160 mm with a mean mass length of 83.5 mm. There were single lesions in eight cases and multiple lesions in two cases. The lesion echotextures were hypoechoic solid (n = 3), heterogeneously intermediate echoic (n = 5), diffuse myometrial thickening with heterogeneous echogenicity (n = 1) and septated cystic (n = 1). Endometrial stromal sarcoma presents with four patterns of its sonographic appearance; a polypoid mass with nodular myometrial extension, an intramural mass with an ill defined margin and heterogeneous echogenicity, an ill defined large central cavity mass or, diffuse myometrial thickening.
Tuncer, Osman Nuri; Erbasan, Ozan; Golbasi, Ilhan
Synovial sarcoma (SS), a mesenchymal spindle cell tumor, displays variable epithelial differentiation, including glandular formation, and features a specific chromosomal translocation, t(X;18)(p11;q11). SS accounts for 5% to 10% of soft-tissue sarcomas. These tumors occur mostly in the joints, especially near the knee, but they also occur in other locations. Primary intravascular SS (IVSS) are extremely rare; only 6 well-documented cases have been reported in the English literature. We describe a new case of primary IVSS of the superior vena cava (SVC) in a 16-year-old boy. A transthoracic echocardiogram confirmed a large (4.8 × 4.6 cm) circumscribed mass filling the right atrium, as well as a moderate pericardial effusion. The mass extended from the SVC to the tricuspid valve but did not prevent valve coaptation. Surgery via a transatrial approach revealed a huge mass (8 to 12 cm) attached to the SVC via a 5-mm pedicle. The tumor was excised, and the patient experienced an uneventful postoperative course. Fluorescence in situ hybridization analysis revealed the presence of the SS-specific translocation.
Full Text Available The case was a 40-year-old female. She visited a local doctor with a chief complaint of right side abdominal pain. A right kidney tumor measuring 10 cm in diameter was observed in an abdominal Computed Tomography (CT scan. Based on the CT image, the possibility of angiomiolipoma (AML could not be ruled out, but a high maximum standardized uptake value (SUVmax of 7.8 was observed in a Positron Emission Tomography CT (PET-CT scan and there was a possibility of malignancy. We therefore performed a transperitoneal right radial nephrectomy. Although adhesion of the tumor to the duodenum and the inferior vena cava was observed, it was possible to perform an excision. The tumor accounted for a large proportion of the excised kidney; the surrounding areas had taken on a cyst-like structure, and the interior comprised grayish brittle tissue exhibiting solid growth. Histologically, gland-like and cyst-like structures composed of cylindrical cuboidal cells and mainly characterized by the solid growth of short fusiform-shaped and oval-shaped basophilic cells were observed, and we believed it was a synovial sarcoma. There were no malignant findings in the adrenal gland. There have been approximately 30 reported cases around the world of synovial sarcoma that developed in the kidney, and we herein report this case with bibliographic considerations.
Kabashi, Serbeze; Hoxha, Naim; Gashi, Shkelzen; Ahmegjekaj, Ilir; Bejta, Ilir; Sadiku, Muharrem; Ymeri, Halit; Kabashi, Antigona; Bicaj, Xhavit; Mucaj, Sefedin
Primary malignant heart tumors represent rare entities where fibro sarcoma represents about 3% of all. Introducing the patient: A 15 years old patient with cardiac insufficiency (heart failure) symptoms, such as weakness, cyanosis, palpitations and breathing difficulties; enlargement of upper mediastinum and pleural effusion. Through echocardiography a pericardial effusion and intracavitary thrombus in atrium was diagnosed. With computed tomography is diagnosed a tumoral mass in right atrium which is also spread in the right ventricle of the heart. Tumor is completely removed; pat histology result showed primary fibro sarcoma of the heart. At that time no metastasis was found. Conclusion. Primary malignant heart tumors may manifest like cardiac insufficiency or like systemic diseases. Fibrosarcomas are rare and have bad prognosis. On average patients can live around six months after initial symptoms appeared and diagnosis of the tumor was done. In the case of cardiac insufficiency with differential diagnosis we should also think of heart tumors, which could certainly be proved for or eliminated by echocardiography.
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)
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.)
Çelenk, Fatih; Yilmaz, Metin; Asal, Korhan; Ekinci, Özgür; Tokgöz, Nil
Primary nasopharyngeal Kaposi sarcoma is extremely rare, as only 1 case has been previously reported in the literature. We report a new case, which occurred in a 37-year-old man with a known history of acquired immune deficiency syndrome (AIDS). The patient presented with complaints of recurrent epistaxis and postnasal hemorrhage. Endoscopic examination detected a bluish, smooth, firm, nonpulsatile mass in the nasopharyngeal wall. Histopathologic findings on biopsy were consistent with Kaposi sarcoma. The tumor was successfully treated with radiotherapy. Kaposi sarcoma should be considered in the differential diagnosis of any AIDS patient who presents with recurrent unilateral nasal bleeding.
Kutlubay, Zekayi; Yardimci, Gürkan; Engin, Burhan; Demirkesen, Cuyan; Aydin, Övgü; Khatib, Rashid; Tuzun, Yalçın
Pseudo-Kaposi sarcoma is a benign reactive vascular proliferative disorder, which can be seen at any age. It occurs when the chronic venous pressure changes result in vascular proliferation in the upper and mid dermis. This disease is divided into two subtypes: the most frequent subtype is the Mali type and seen in early ages. The Mali type is seen in chronic venous insufficiency and in those patients with arteriovenous shunts. The rare subtype is the Stewart-Bluefarb type. This disease must be distinguished from Kaposi sarcoma because of their clinical resemblance. Herein, we present a patient with pseudo-Kaposi sarcoma, which developed after bullous erysipelas.
Andreas M. Stark
Full Text Available Medulloblastoma, astrocytoma, and ependymoma represent the most common infratentorial tumors in childhood, while Ewing sarcomas in that localization are extremely rare. A large left infratentorial space-occupying lesion was diagnosed in a 12-year-old girl with signs of increased intracranial pressure. Following total tumor resection, histological and molecular examination revealed Ewing sarcoma with rearranged EWSR-1 gene. The patient achieved complete remission following adjuvant chemotherapy and radiotherapy according to Euro-EWING 2008 treatment protocol. Intracranial Ewing sarcoma, although rare, should be an important differential diagnosis of intracranial tumors in childhood which requires aggressive multimodal treatment.
GAC E,PATRICIO; CABANÉ T,PATRICIO; Gallegos M,Iván; ABUSLEME P,EUGENIA; ORTÚZAR E,WALDO; Amat V,José; MARAMBIO R,ANDRÉS; MARTÍNEZ G,FELIPE; MIRANDA V,CAROLINA
El sarcoma sinovial es un tumor maligno de partes blandas, bien diferenciado y que representa entre 5.6% a 10% de todos los sarcomas. Su localización en cabeza y cuello no es común, y representa cerca de un 9%, con menos de 100 casos reportados en la literatura. La localización intraoral es muy peculiar, existiendo 32 casos previamente descritos en el mundo, de los cuales 3 corresponden al tipo monofásico. Se presenta un caso de un paciente varón de 16 años con un caso de sarcoma sinovial int...
Hong, Sun Hwa; Suh, Sang Il; Seol, Hae Young; Cho, Jea Gu; Shin, Bong Kyung [Guro Hospital, Korea University College of Medicine, Seoul (Korea, Republic of)
Myeloid sarcoma manifesting as multiple lymphadenopathy is quite rare. We present here a case of myeloid sarcoma that first presented with palpable bilateral neck masses. A 53-year-old woman complained about repetitive swelling in the right infraauricular and submental areas for 3 years. The results of computed tomography showed multiple lymphadenopathy in both areas of the neck as well as other parts of the body. So, the presumptive diagnosis was lymphoma, but the result of the excisional biopsy of the neck mass confirmed it to be a myeloid sarcoma.
Hartwig, M; Matthes, E; Arnold, W
The superhelical properties of chromosomal DNA from cells of a mouse sarcoma were investigated in neutral sucrose gradients containing ethidium bromide. Removal of negative supercoiling from the DNA of the sarcoma cells required a substantially higher dye concentration than was necessary in the case of DNA from cultured mouse fibroblasts. The calculated value of the mean superhelical density in malignant cells (sigma = -0.14) appears abnormally high compared with the value (sigma = -0.09) obtained for DNA of mouse fibroblasts. Chromosomal DNA from mouse sarcoma cells is therefore concluded to be highly deficient in helical turns.
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.
Sakahara, H.; Endo, K.; Nakashima, T.; Koizumi, M.; Ohta, H.; Kunimatsu, M.; Torizuka, K.; Nakamura, T.; Tanaka, H.; Kotoura, Y.
The authors have developed several monoclonal antibodies against human osteogenic sarcoma, one of which; OST7 (IgGl) selectively localized in osteogenic sarcoma xenografts in nude mice. In the present study, F(ab')/sub 2/ fragment was compared with whole IgG and those labeled with In-111 as well as I-131 were used as a radiotracer for the scintigraphic imaging of tumors. IgC and F(ab')/sub 2/ were labeled with I-131 using chloramine-T method and injected into nude mice bearing human osteogenic sarcoma. Scintigrams at day 2 clearly delineated the site of tumors with almost no radioactivity in other organs with F(ab')/sub 2/, which yielded much better images than whole IgG. Tumor-to-blood ratio of 6.09-27.87 was obtained at day 2 using F(ab')/sub 2/, whereas it was 0.76-1.12 at day 2 and 2.05-3.27 at day 7 with IgG. I-131 labeled nonspecific F(ab')/sub 2/ or IgG resulted in no or very low tumor uptake with tumor-to-blood ratio of 0.94-1.18 at day 2 for F(ab')/sub 2/ and 0.67-0.76 at day 7 for IgG, respectively. In-111 labeled F(ab')/sub 2/ fragment of OST7, which was prepared using DTPA as a bifunctional chelate, also showed a high tumor accumulation with tumor-to-blood ratio of 11.67-17.54 at day 2, but higher background activity in the liver and kidney was observed than I-131 labeled one. These results indicate that F(ab')/sub 2/ fragment of OST7 labeled with either I-131 or In-111, has a great potential for the radioimmunoimaging of osteogenic sarcoma.
Full Text Available Adenocarcinoma and squamous cell carcinoma account for the vast majority of oesophageal malignancies. Other malignancies known to occur in the oesophagus include melanoma, sarcoma, and lymphoma. Among the sarcomas, carcinosarcoma is the commonest with both carcinomatous and sarcomatous elements followed by leiomyosarcoma of mesenchymal origin. Other sarcomas reported in the literature are liposarcoma, synovial sarcoma, myxofibrosarcoma, Ewing's sarcoma, granulocytic sarcoma, histiocytic sarcoma, schwannoma rhabdomyosarcoma, and epithelioid sarcoma. We report a case of malignant spindle cell tumour of oesophagus. Sarcomas of esophagus present as a polypoid exophytic soft tissue mass. Our patient presented with a stricture which is a rare presentation. Locally aggressive treatment with surgery is beneficial, and local palliative treatment including radiotherapy is worthwhile.
Oesophageal cancer and Kaposi's Sarcoma in Malawi: a comparative analysis. ... Given that oesophageal cancer (OC) is common in Malawi and its outcome is so dismal, would it be pragmatic to promptly mitigate the effects of ... Article Metrics.
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.
Conclusion: Surgery represents the only potentially curative therapy for breast sarcoma. Tumor size and adequate resection margin are the most important prognostic factors. Approximately 80% of recurrences appear in the first two years.
Treatment for Kaposi's sarcoma include radiotherapy, chemotherapy and/or immunotherapy whereby ... Physical examination revealed an elderly man of ... Pulse rate was ... ultrasound was normal. .... effective treatment for pulmonary Kaposi's.
Jun 11, 2015 ... Primary synovial sarcoma is a very rare tumor of the mediastinum, which is unreported in the entire ... and cytogenetics are required to therapeutic management ... off the left upper lung zone and resected from the anterior.
Anjali R. Dhote
Full Text Available Kaposi's sarcomas the most common malignancy associated with Human Herpesvirus-8 (HHV8 infection. Though name is sarcoma but it is low grade vascular neoplasm. It is the tumour which arises from endothelial lining of vessels as well as lymphatic channels. So it involved all sites such as skin, Gastro intestine, lungs along with lymph nodes. We are presenting one such case of 65 year immunocompromised Indian male presented with multiple non blanching reddish bluish nodules on all extremities, chest, back with submandibular and cervical lymphadenopathy. Fine needle aspiration cytology (FNAC was performed and diagnosis was given low grade spindle cell neoplasm consistent with Kaposi's sarcoma which was confirmed on histopathology as Kaposi's sarcoma. [Int J Res Med Sci 2014; 2(2.000: 789-791
Aggerholm-Pedersen, Ninna; Demuth, Christina; Safwat, Akmal;
Background. One of the major challenges affecting sarcoma treatment outcome, particularly that of metastatic disease, is resistance to chemotherapy. Cancer-initiating cells are considered a major contributor to this resistance. Methods. An immortalised nontransformed human stromal (mesenchymal) s...
Lim, Sue Zann; Ong, Kong Wee; Tan, Benita Kiat Tee; Selvarajan, Sathiyamoorthy; Tan, Puay Hoon
Breast sarcoma is a rare condition. It consists of a heterogeneous group of non-epithelial tumours arising from the mesenchymal tissue of the breast. It has a distinctly different natural history, treatment response and prognosis as compared with carcinoma of the breast. A different diagnostic approach and treatment strategy have to be defined for this group of tumours. Due to its rarity, the current understanding on breast sarcoma is limited and is mostly based on small retrospective case series or case reports. Hence, the management generally follows the algorithms derived from randomised control trials of soft tissue sarcomas in the extremities and chest wall. Through this review, we discuss the results of major retrospective studies on breast sarcomas including data on epidemiology, aetiology, diagnostic approach, treatment strategies and outcomes of this challenging and potentially aggressive condition.
Full Text Available Abstract Introduction Kaposi's sarcomas have been associated with different conditions of immunosuppression and are also known to be a typical complication of solid organ transplantations. Case presentation We report the case of a 65-year-old Turkish man with a history of heart transplantation 10 months ago who presented for clarification of his dyspnea. The patient had a known history of chronic obstructive pulmonary disease and a smoking history of 40 pack years. Radiologically, three progressively growing intra-pulmonary nodules were detected. The histology was diagnostic for a Kaposi's sarcoma. Visceral and especially primary intra-pulmonary Kaposi's sarcomas are very rare and have been described to have a rather unfavorable prognosis. Conclusions Even with a history suggestive for conventional lung cancer, Kaposi's sarcomas should be considered in patients after transplantation of solid organs. It should be noted that in a minority of cases this tumor exists in the absence of the typical cutaneous lesions.
Full Text Available Ionizing radiation has been known to induce malignant transformation in human beings. Radiation-induced sarcomas are a late sequel of radiation therapy. Most sarcomas have been reported to occur after exposure to a radiation dose of 55 Gray (Gy and above, with a dose ranging from 16 to 112 Gys. Spindle cell sarcomas, arising after radiotherapy given to treat the carcinoma of head and neck region is a very uncommon sequel. This is a rare case report of spindle cell sarcoma of left maxilla, in a 24-year-old male, occurring as a late complication of radiotherapy with Cobalt-60 given for the treatment of retinoblastoma of the left eye 21 years back.
Full Text Available This article describes the medical, psychological, and social challenges encountered during home-based, family-centred palliative care of a 3-year-old female with secondary histiocytic sarcoma diagnosed during treatment for T-cell acute lymphoblastic leukaemia. Histiocytic sarcoma is an exceedingly rare cancer in adults, but even less frequent and highly aggressive when presenting as a secondary cancer in children. Comprehensive, multidisciplinary paediatric hospice care services are not widely available across Slovakia,thus limiting the number of patients and families offered such highly specialized end-of-life care. This case study illustrates the primary benefits for the child and family of such a program as well as the impact on the medical and nursing professionals working in the fi eld of paediatric haematology-oncology.
Maryem Seco Meza
Full Text Available Los sarcomas de tejidos blandos aparecen en cualquier localización y abarcan más de 50 tipos histológicos; se originan del tejido mesodérmico embrionario y del ectodermo o neuroectodermo. Son pocos frecuentes y representan el 1 % de tumores sólidos abdominales de alta agresividad en adultos. Los más agresivos y con mayor capacidad metastásica son los de alto grado, mientras que los de bajo grado son agresivos pero de forma local. Al respecto, se presenta el caso de una paciente femenina de 52 años, del área rural, con 3 meses de evolución del cuadro clínico, quien presentó aumento progresivo de volumen abdominal, sensación de peso y masa palpable, concomitantemente emesis, adinamia, pérdida de peso y edema en miembros inferiores. Se realizó ultrasonografía abdominal que evidenció tumoración ecomixta de 16 x 20 cm en línea media. La Tomografía Axial Computarizada abdominal reportó lesión ocupante de espacio heterogénea, ovalada, de bordes irregulares de 15 x 10 cm que desplaza asas delgadas. La laparotomía exploradora media mostró ausencia de implantes peritoneales y carcinomatosis, tumor muy vascularizado, encapsulado de contextura lisa, consistencia firme, con bordes definidos de 15 x 20 cm. Después de la resección, no se observaron adenopatías ni metástasis locales y a distancia. El reporte histopatológico identificó un sarcoma de alto grado. A dos años de la operación no se encontró metástasis. Contrario a lo que la literatura reporta, se mantuvo un comportamiento “benigno” a pesar de su histología, lo cual es inusual considerando la historia natural de la enfermedad. Unusual Behavior of High-grade sarcoma malignancy in abdomen. A case report Abstract Soft tissue sarcomas appear in any location and include more than 50 histological types; they originate from mesodermal embryonic tissue, ectoderm or neuroectoderm. They are infrequent and represent 1% of highly aggressive abdominal solid tumors in adults
Full Text Available Introducción: Los sarcomas son tumores malignos poco frecuentes, siendo raros en cabeza y cuello. En su etiología se involucran factores como agentes químicos, radiación, inmunosupresión y síndromes y anomalías genéticas. Caso clínico: Varón de 64 años, que presenta lesión en piel de mejilla derecha de un año de evolución, localización en la que presentó hace veinte años un carcinoma basocelular tratado con radioterapia. Tras descartar existencia de metástasis, se realizó exéresis de la lesión con márgenes de seguridad y reconstrucción con colgajo de Mustardé. Se complementó el tratamiento con radioterapia. Discusión: El diagnóstico es anatomopatológico, siendo fundamental descartar afectación metastásica. Para mejorar la supervivencia y disminuir su elevada tasa de recidiva, deberían tratarse de forma multidisciplinar (cirugía, radioterapia y/o quimioterapia. Conclusión: A pesar de su baja frecuencia, los sarcomas deben estar presentes en el diagnóstico diferencial de toda lesión que aparezca en zonas radiadas previamente, especialmente en la piel facial.Introduction: Sarcomas are malignant tumors that are infrequent, being rare in the head and neck. Factors such as chemical agents, radiation, immunosuppression, and genetic syndromes and abnormalities are involved in their etiology. Case report: A 64-year-old man developed a skin lesion on the right cheek one year earlier at the site where he had presented a basal cell carcinoma 20 years earlier that was treated with radiation therapy. After ruling out the existence of metastasis, the lesion was treated by surgical resection with safety margins and reconstruction with the Mustardé flap. Treatment was supplemented with radiation therapy. Discussion: The diagnosis of sarcomas is histopathologic and it is essential to rule out metastasis. To improve survival and reduce the high rate of recurrence, a multidisciplinary approach to treatment should be used (surgery
张永贞; 杨国庆; 王新正; 郭雪蓉; 陈万青; 张思维; 郑荣寿; 苏芳
[Purpose] To investigate the incidence of kidney cancer and unspecified urinary organs cancer in China. [Methods] The incidence data from 32 cancer registries in China from 2003 to 2007 were enrolled in this analysis. Specified incidences by region, age and comparison of cancer incidences between China and other 184 countries in the same period were analyzed. [Results] The incidence of kidney cancer and unspecified urinary organs cancer in China was 536/105 (6.58/105 in male, 4.10/105 in female; 6.58/105 in urban, 1.18/105 in rural ),which was ranked the 12th in all cancer sites incidence. The incidence of kidney cancer and unspecified urinary organs cancer had been increasing from 4.35/105 in 2003 to 6.21/105 in 2007. [Conclusion] The incidence of kidney cancer and unspecified urinary organs cancer shows an increasing trend. Comparing to other countries and areas, the incidence in China is lower than that with the average level of the world and the developed countries.%[目的]分析中国肾及泌尿系统其他癌的发病情况.[方法]采用2003～2007年全国32个肿瘤登记地区的数据资料,分析不同地区、不同年龄段肾及泌尿系统其他癌的发病率,同时与世界184个国家和地区的发病情况进行比较.[结果]全国肾及泌尿系统其他癌在全部恶性肿瘤发病构成中排列第12位,发病率为5.36/10万,其中男、女性发病率分别为6.58/10万、4.10/10万,城市、农村地区发病率分别为6.58/10万、1.18/10万.全国肾及泌尿系统其他癌的发病率从2003年的4.35/10万上升到2007年的6.21/10万.[结论]中国肾及泌尿系统其他癌发病率呈上升趋势;与世界其他国家和地区相比低于全球和发达国家平均水平.
Neilsen, Paul M.; Pishas, Kathleen I.; Callen, David F; Thomas, David M.
The p53 tumour suppressor plays a pivotal role in the prevention of oncogenic transformation. Cancers frequently evade the potent antitumour surveillance mechanisms of p53 through mutation of the TP53 gene, with approximately 50% of all human malignancies expressing dysfunctional, mutated p53 proteins. Interestingly, genetic lesions in the TP53 gene are only observed in 10% of Ewing Sarcomas, with the majority of these sarcomas expressing a functional wild-type p53. In addition, the p53 downs...
Full Text Available Myeloid sarcomas are extramedullary accumulations of immature myeloid cells that may present with or without evidence of pathologic involvement of the bone marrow or peripheral blood, and often coincide with or precede a diagnosis of acute myeloid leukemia (AML. A dearth of experimental models has hampered the study of myeloid sarcomas and led us to establish a new system in which tumor induction can be evaluated in an easily accessible non-hematopoietic tissue compartment. Using ex-vivo transduction of oncogenic Kras(G12V into p16/p19(-/- bone marrow cells, we generated transplantable leukemia-initiating cells that rapidly induced tumor formation in the skeletal muscle of immunocompromised NOD.SCID mice. In this model, murine histiocytic sarcomas, equivalent to human myeloid sarcomas, emerged at the injection site 30-50 days after cell implantation and consisted of tightly packed monotypic cells that were CD48+, CD47+ and Mac1+, with low or absent expression of other hematopoietic lineage markers. Tumor cells also infiltrated the bone marrow, spleen and other non-hematopoietic organs of tumor-bearing animals, leading to systemic illness (leukemia within two weeks of tumor detection. P16/p19(-/-; Kras(G12V myeloid sarcomas were multi-clonal, with dominant clones selected during secondary transplantation. The systemic leukemic phenotypes exhibited by histiocytic sarcoma-bearing mice were nearly identical to those of animals in which leukemia was introduced by intravenous transplantation of the same donor cells. Moreover, murine histiocytic sarcoma could be similarly induced by intramuscular injection of MLL-AF9 leukemia cells. This study establishes a novel, transplantable model of murine histiocytic/myeloid sarcoma that recapitulates the natural progression of these malignancies to systemic disease and indicates a cell autonomous leukemogenic mechanism.
Full Text Available Myxoinflammatory fibroblastic sarcoma is a low grade sarcoma that is composed of a mixed inflammatory infiltrate along with spindled, epithelioid and bizarre appearing cells in a background of hyaline and myxoid zones. Seen affecting the distal extremities commonly, with an equal sex predilection, these tumors are rare and require an extensive immunohistochemical work up for proper diagnosis. They have a tendency to recur.
Kubo, Takatosh; Ohtomo, Kuni [Graduate School of Medicine, University of Tokyo, Tokyo (Japan); Kiryu, Shigeru; Akai, Hiroyuki; Ora, Yasunori; Tojo, Arinobu; Yoshida, Hideo; Kato, Naoya; Nakano, Yoshiyasu [Institute of Medical Science, University of Tokyo, Tokyo (Japan)
Histiocytic sarcoma in the liver is an extremely rare hematological malignancy. Herein, we reported the case of a 68-year-old woman who presented with characteristic wedge-shaped abnormality bounded by hepatic veins on computed tomography and magnetic resonance imaging of the liver. In the wedge-shaped area, decreased portal flow and the deposition of iron were observed. These imaging findings are consistent with intrasinusoidal tumor cell infiltration. A liver biopsy was performed, and histiocytic sarcoma was confirmed histopathologically.
Kubo, Takatoshi [Department of Radiology, Graduate School of Medicine, University of Tokyo, Tokyo 113-8654 (Japan); Kiryu, Shigeru; Akai, Hiroyuki [Department of Radiology, Institute of Medical Science, University of Tokyo, Tokyo 108-8639 (Japan); Ota, Yasunori [Department of Pathology, Institute of Medical Science, University of Tokyo, Tokyo 108-8639 (Japan); Tojo, Arinobu [Department of Hematology and Oncology, Institute of Medical Science, University of Tokyo, Tokyo 108-8639 (Japan); Yoshida, Hideo [Department of Gastroenterology, Japanese Red Cross Medical Center, Tokyo 150-8935 (Japan); Kato, Naoya [Advanced Medical Science, Institute of Medical Science, University of Tokyo, Tokyo 108-8639 (Japan); Nakano, Yoshiyasu [Department of Radiology, Institute of Medical Science, University of Tokyo, Tokyo 108-8639 (Japan); Ohtomo, Kuni [Department of Radiology, Graduate School of Medicine, University of Tokyo, Tokyo 113-8654 (Japan)
Histiocytic sarcoma in the liver is an extremely rare hematological malignancy. Herein, we reported the case of a 68-year-old woman who presented with characteristic wedge-shaped abnormality bounded by hepatic veins on computed tomography and magnetic resonance imaging of the liver. In the wedge-shaped area, decreased portal flow and the deposition of iron were observed. These imaging findings are consistent with intrasinusoidal tumor cell infiltration. A liver biopsy was performed, and histiocytic sarcoma was confirmed histopathologically.
Full Text Available Retroperitoneal sarcomas (RPS are rare tumours that typically present late and carry a poor prognosis even following grossly complete resection. In an attempt to improve the outlook for patients with RPS, sarcoma specialists have employed various adjuvant therapies, including extermal beam radiation, intraoperative radiation, brachyradiation and systemic chemotherapy. This article reviews the presentation and prognosis of RPS, and focuses on the results of new treatment strategies compared with conventional management.
Dezube Bruce J
Full Text Available Abstract Background Involvement of the subcutis by Kaposi sarcoma (KS occurs primarily when cutaneous KS lesions evolve into deep penetrating nodular tumors. Primary KS of the subcutaneous tissue is an exceptional manifestation of this low-grade vascular neoplasm. Case presentation We present a unique case of acquired immune deficiency syndrome (AIDS-associated KS manifesting primarily in the subcutaneous tissue of the anterior thigh in a 43-year-old male, which occurred without overlying visible skin changes or concomitant KS disease elsewhere. Radiological imaging and tissue biopsy confirmed the diagnosis of KS. Conclusion This is the first documented case of primary subcutaneous KS occurring in the setting of AIDS. The differential diagnosis of an isolated subcutaneous lesion in an human immunodeficiency virus (HIV-infected individual is broad, and requires both imaging and a histopathological diagnosis to guide appropriate therapy.
Alio, Jorge L; Sales-Sanz, Marco; Vaz, Maria A; Barrancos, Constanza; Reguero, Maria E; Diamantopoulus, Jorge; Poveda, Pedro
A 40-year-old man presented with painless, progressive vision loss and mild proptosis of the OD. CT revealed a right intraconal mass with slight penetration of the optic canal not contiguous with any bony structure. Incisional biopsy through a transfrontal orbitotomy revealed a diffuse growth of homogeneous, small, round cells. Immunohistochemical stains were positive for vimentin and MIC2 (CD99), and the translocation at EWS gene (22q12) was detected. Metastatic workup and a full-body bone scan were negative, confirming primary orbital extraosseous Ewing sarcoma. The patient received neoadjuvant chemotherapy and an orbital exenteration with preservation of eyelids and conjunctiva. He also received adjuvant chemotherapy and local radiotherapy, and he has remained disease-free for almost 3 years.
Yamato, M.; Nishimura, G. [Department of Radiology, Dokkyo University School of Medicine, Tochigi (Japan); Yamaguchi, Takehiko [Department of Pathology, Dokkyo University School of Medicine, Tochigi (Japan); Tamai, Kazuya; Saotome, Koichi [Department of Orthopaedic Surgery, Dokkyo University School of Medicine, Tochigi (Japan)
The case of a patient with epithelioid sarcoma in the right arm is reported. The diagnosis was delayed because of misinterpretation arising from complexity in the MR findings, including a honeycomb pattern in the subcutaneous fat simulating lymphedema, and an intramuscular diffuse high signal intensity on T2-weighted images without a discrete mass lesion. The histological findings revealed that the diffuse muscular abnormality mainly resulted from denervation of the muscles due to perineural invasion by the tumor, and subcutaneous edema from lymphedema secondary to lymphatic tumor spread concurrent with lymphatic fibrosis. Multiple foci of cortical erosions in the humerus, a rare manifestation of this tumor, were detected 6 months later. (orig.) 11 refs.
Gómez Martín, Cristina; Ortega, María I; Aramburu, José A; Fernández-Cañamaque, José L
Myxoinflammatory fibroblastic sarcoma (MIFS) is a rare low-grade tumor of modified fibroblasts, with tendency to local recurrence. This unusual entity typically presents as a slow-growing painless mass in the distal extremities of middle-aged subjects. A 48-year-old woman presented to our clinic with a painless subcutaneous mass in the right temporal region. Excisional biopsy made the rare diagnosis of MIFS. Histologic examination showed the unique features that characterize this lesion: a myxoid component with a superimposed inflammatory infiltrate and the presence of distinctive, large, and bizarre Reed-Stemberg-like cells. A second wide tumor bed resection was performed, achieving clear margins. No adjuvant therapy was administered, and the patient is free of disease at 18 months postoperatively. To the best of our knowledge, this is the first reported case of MIFS presenting in the face. This adds another possibility for differential diagnoses of soft tissue tumors of the face.
Jiménez Soto, Daniela; Soto Fallas, Javier; Garro Ortiz, Mario; Vega Ulate, Gustavo
El grupo de tumores conocido como sarcoma de Ewing consiste en neoplasias de células redondas, morfológicamente similares y por la presencia de una translocación cromosómica común. Aunque poco frecuentes, estos tumores constituyen el tercer grupo de tumores primarios de hueso, después del osteosarcoma y el condrosarcoma. Afecta con mayor frecuencia a niños y adolescentes. Algunos casos presentan metástasis, con afección sistémica. El tratamiento se da con múltiples fármacos, así como el contr...
Teng Jin; Ping Zhang Co-first author; Xiaoming Li
Objective The aim of this study was to analyze the imaging features of alveolar soft part sarcoma (ASPS). Methods The imaging features of 11 cases with ASPS were retrospectively analyzed. Results ASPS mainly exhibited an isointense or slightly high signal intensity on T1-weighted imaging (T1WI), and a mixed high signal on T2-weighted imaging (T2WI). ASPS was partial, with rich tortuous flow voids, or “line-like” low signal septa. The essence of the mass was heterogeneous enhancement. The 1H-MRS showed a slight choline peak at 3.2 ppm. Conclusion The wel-circumscribed mass and blood voids, combined with “line-like” low signals play a significant role in diagnosis. The choline peak and the other signs may be auxiliary diagnoses.
Alonso-Dominguez, J. M.; Calbacho, M.; Talavera, M.; Villalon, C.; Abalo, L.; Garcia-Gutierrez, J. V.; Lozano, S.; Tenorio, M.; Villarrubia, J.; Lopez-Jimenez, J.; Ferro, M. T.
Histiocytic sarcoma (HS) is a neoplasm derived from histiocytes. Its diagnosis was not clear until its immunohistochemistry profile was correctly established. Not much is known about its genetic properties. We report a case of a 48-year-old male patient whose bone marrow was almost completely occupied by monomorphic medium size neoplastic cellularity. Its immunohistochemical profile was CD68+, CD4+, CD45+ with negativity of other dendritic cells, and other lineage markers. Cytogenetic study showed 4 related clones: one with trisomy 8 and extra material on the short arms of chromosome 4; a second line with tetrasomy of chromosome 8, add(4)(p16); the third clone had the same alterations as the previous and deletion of chromosome 3 at q11; the fourth line had tetrasomy 8 and translocation t(3;5)(q25;q35). To our knowledge this is the first HS case showing chromosome 8 trisomy and tetrasomy and the other described alterations. PMID:22937328
J. M. Alonso-Dominguez
Full Text Available Histiocytic sarcoma (HS is a neoplasm derived from histiocytes. Its diagnosis was not clear until its immunohistochemistry profile was correctly established. Not much is known about its genetic properties. We report a case of a 48-year-old male patient whose bone marrow was almost completely occupied by monomorphic medium size neoplastic cellularity. Its immunohistochemical profile was CD68+, CD4+, CD45+ with negativity of other dendritic cells, and other lineage markers. Cytogenetic study showed 4 related clones: one with trisomy 8 and extra material on the short arms of chromosome 4; a second line with tetrasomy of chromosome 8, add(4(p16; the third clone had the same alterations as the previous and deletion of chromosome 3 at q11; the fourth line had tetrasomy 8 and translocation t(3;5(q25;q35. To our knowledge this is the first HS case showing chromosome 8 trisomy and tetrasomy and the other described alterations.
Osasan, Stephen; Zhang, Mingyong; Shen, Fan; Paul, Paulose J; Persad, Sujata; Sergi, Consolato
Compared to other bone tumors, bone osteogenic sarcoma (BOS) continues to confer a much grimmer prognosis as the survival benefit of traditional chemotherapy treatment regimens is still unsatisfactory. Chemotherapy was demonstrated to be effective in eradicating both primary tumor and pulmonary metastases in the last century, with effective agents used in various combination regimens having changed the survival rate from less than 10% to 75%. The most common primary bone cancer, BOS is conventionally a primary intramedullary high-grade malignant tumor characterized by malignant cells forming immature bone or osteoid. BOS is a disease with diverse morphological presentations. The treatment of all morphological variants seem to have been the same for over 30 years. The introduction of antiproliferative agents such as insulin growth factor-binding protein 3 hold promise of a potentially veritable therapeutic target. In this review, we highlight recent data on osteosarcoma to consolidate a platform able to connect bench and bedside.
Olimpiadi, Yuliya; Song, Suisui; Hu, James S; Matcuk, George R; Chopra, Shefali; Eisenberg, Burton L; Sener, Stephen F; Tseng, William W
Management of retroperitoneal soft tissue sarcomas (RP STS) can be very challenging. In contrast to the more common extremity STS, the two predominant histologic subtypes encountered in the retroperitoneum are well-differentiated/dedifferentiated liposarcoma and leiomyosarcoma. Surgery remains the mainstay of treatment for RP STS. Preoperative planning and anticipation of the need for resection of adjacent organs/structures are critical. The extent of surgery, including the role of compartmental resection, is still controversial. Radiation therapy may be an important adjunct to surgery to provide locoregional disease control; this is currently being evaluated in the preoperative setting in the EORTC STRASS trial. Systemic therapy, tailored to the specific histologic subtype, may also be of benefit for the management of RP STS. Further investigation of novel therapies (e.g., targeted therapies, immunotherapy) is needed. Overall, multi-institutional collaboration is important moving forward, to continue to better understand and optimize management of this disease.
Full Text Available Kaposi’s sarcoma-associated herpesvirus (KSHV has an etiologic role in Kaposi’s sarcoma, primary effusion lymphoma and multicentric Castleman’s disease. These diseases are most common in immunocompromised individuals, especially those with AIDS. Similar to all herpesviruses, KSHV infection is lifelong. KSHV infection in tumor cells is primarily latent, with only a small subset of cells undergoing lytic infection. During latency, the KSHV genome persists as a multiple copy, extrachromosomal episome in the nucleus. In order to persist in proliferating tumor cells, the viral genome replicates once per cell cycle and then segregates to daughter cell nuclei. KSHV only expresses several genes during latent infection. Prominent among these genes, is the latency-associated nuclear antigen (LANA. LANA is responsible for KSHV genome persistence and also exerts transcriptional regulatory effects. LANA mediates KSHV DNA replication and in addition, is responsible for segregation of replicated genomes to daughter nuclei. LANA serves as a molecular tether, bridging the viral genome to mitotic chromosomes to ensure that KSHV DNA reaches progeny nuclei. N-terminal LANA attaches to mitotic chromosomes by binding histones H2A/H2B at the surface of the nucleosome. C-terminal LANA binds specific KSHV DNA sequence and also has a role in chromosome attachment. In addition to the essential roles of N- and C-terminal LANA in genome persistence, internal LANA sequence is also critical for efficient episome maintenance. LANA’s role as an essential mediator of virus persistence makes it an attractive target for inhibition in order to prevent or treat KSHV infection and disease.
Full Text Available Abstract Sarcomas constitute less than 1% of all cervical malignancies. With over 150 reported cases, rhabdomyosarcomas represent the most commonly reported sarcoma at this location. In this report, a select group of the more uncommon sarcomas of the uterine cervix are reviewed, including all previously reported examples of leiomyosarcoma, liposarcoma, alveolar soft part sarcoma, Ewing sarcoma/primitive neuroectodermal tumor, undifferentiated endocervical sarcoma, and malignant peripheral nerve sheath tumor (MPNST. Emphasis is placed on any distinctive clinicopathologic features of these entities at this unusual location.
Eitan R. Friedman
Full Text Available Kaposi’s sarcoma (KS is a multicentric vascular neoplasm associated with the Kaposi’s sarcoma-associated herpes virus (KSHV. KS can occur in immunocompromised patients as well as certain populations in Africa or in the Mediterranean. Less than 5% of KS cases can present with lymphangioma-like kaposi sarcoma (LLKS, which can occur in all KS variants. KS presents with characteristic skin lesions that appear as brown, red, blue, or purple plaques and nodules. The lesions are initially flat and if untreated will become raised. LLKS presents similarly to KS but is associated with severe lymphedema and soft tissue swelling as well as bulla-like vascular lesions. We present the case of an 85-year-old Lebanese, HIV negative, man who presented with a swollen and painful right lower extremity accompanied by necrotic lesions. Wound cultures were positive, and we began the work-up for secondarily infected gangrene. However, skin biopsy results revealed that he in fact had lymphangioma-like Kaposi sarcoma, which allowed us to shift our management. Advanced Kaposi’s sarcoma can present similar to gangrene. It is important to recognize the typical skin lesions of KS and not to overlook Kaposi’s sarcoma or LLKS within the differential.
MENG Guo-zhao; ZHANG Hong-ying; BU Hong; ZHANG Xian-liang; PANG Zong-guo; KE Qi; LIU Xi; YANG Guo
Background Myofibroblastic sarcoma was used to be a controversial neoplasm. This study investigated the clinicopathological features of 20 cases of myofibroblastic sarcoma arising in different locations.Methods The paraffin-embedded tissue samples from 20 cases of patients with myofibroblastic sarcoma were stained immunohistochemically, and 5 cases examined by electron microscopy. Student's t test was used to analyze the difference of Ki-67 labeling index between grade 1 and grade 2 myofibroblastic sarcomas.Results Histologically, the tumors were composed of slender spindle cells with eosinophilic cytoplasm, and fusiform,tapering, wavy, or plump ovoid; vesicular nuclei and a small central eosinophilic nucleoli. Immunohistochemically, the tumor cells expressed smooth muscle actin (18/20), muscle specific actin (16/20), fibronectin (20/20) and desmin (2/20).Ultrastructurally, the tumor cells revealed abundant rough endoplasmic reticulum and longitudinally arranged fine filaments with focal densities in the cytoplasm. A clinical follow-up of 19 patients showed that 2 cases experienced local recurrence and distant metastasis 6 months to 4 years after the initial operation. Nine cases recurred locally 17 to 46 months after the initial excision, and 9 cases were alive with no evidence of disease.Conclusions Myofibroblastic sarcomas, which exhibit diverse histological appearance, can easily be misdiagnosed as benign tumors. Myofibroblastic sarcomas are local destructive lesions with frequent recurrence, and may metastase distantly.
Brecht, I B; Treuner, J
The very heterogeneous group of paediatric soft tissue sarcomas account for approximately 7 % of all malignant childhood tumours. More than one half of all cases are rhabdomyosarcomas, some of the over 20 entities are very rare. The prognosis and biology of soft tissue sarcomas in children and adolescents vary greatly depending on histological subtype, the age of the patient, the primary site, the tumour size, tumour invasiveness and the extent of disease at diagnosis. Since 1981, 2918 children and adolescents with soft tissue sarcomas were treated prospectively according to the common treatment protocols of the Cooperative Soft Tissue Sarcoma Study Group (CWS-81 - 96). The known prognostic factors were used to develop a more and more detailed risk stratification. The multimodal treatment includes the use of surgery, chemotherapy and radiotherapy and should be planned by a multidisciplinary team. That way, an overall survival of nearly 70 % over all risk groups could be achieved.
Background Hormonal manipulation is sometimes recommended in the treatment of metastatic endometrial stromal sarcoma, but there are few data assessing the efficacy of endocrine therapies in this subtype of uterine sarcomas. Methods We performed a retrospective electronic medical record review of patients with metastatic ESS treated with a hormonal agent at Royal Marsden Hospital between 1999 and 2011. We assessed progression-free survival (PFS), objective response and toxicity profile among p...
Benedetti, Marta; Fazzo, Lucia; Buzzoni, Carlotta; Comba, Pietro; Magnani, Corrado; Fusco, Mario
The aim of the present study was to investigate the possible association between occurrence of soft tissue sarcomas (STS) and residence in an Italian area affected by illegal practices of dumping and setting fire to both hazardous and solid urban wastes. Standardized incidence ratios (SIRs) were computed separately for STS and some specific STS subtypes. The analysis was performed for the total population and for specific age groups, namely, children, adolescents, and adults. In adults, no significant increase in STS was found other than for gastrointestinal stromal tumors in males. A nonsignificant increase in incidence of STS was observed for male children and female adolescents. The results of the present study do not allow conclusions for a causal association. In the absence of previous epidemiological studies on this issue, further investigations are needed.
Zur, Oz; Ronen, Ayelet; Melzer, Itshak; Carmeli, Eli
The vestibulo-ocular response (VOR) may not be fully developed in children with an intellectual and developmental disability (IDD). This study aimed to identify the presence of VOR deficit in children and young adults with unspecified mild-to-moderate intellectual and developmental disability and its effect on balance control. Twenty-one children…
Andrew S Brohl
Full Text Available The Ewing sarcoma family of tumors (EFT is a group of highly malignant small round blue cell tumors occurring in children and young adults. We report here the largest genomic survey to date of 101 EFT (65 tumors and 36 cell lines. Using a combination of whole genome sequencing and targeted sequencing approaches, we discover that EFT has a very low mutational burden (0.15 mutations/Mb but frequent deleterious mutations in the cohesin complex subunit STAG2 (21.5% tumors, 44.4% cell lines, homozygous deletion of CDKN2A (13.8% and 50% and mutations of TP53 (6.2% and 71.9%. We additionally note an increased prevalence of the BRCA2 K3326X polymorphism in EFT patient samples (7.3% compared to population data (OR 7.1, p = 0.006. Using whole transcriptome sequencing, we find that 11% of tumors pathologically diagnosed as EFT lack a typical EWSR1 fusion oncogene and that these tumors do not have a characteristic Ewing sarcoma gene expression signature. We identify samples harboring novel fusion genes including FUS-NCATc2 and CIC-FOXO4 that may represent distinct small round blue cell tumor variants. In an independent EFT tissue microarray cohort, we show that STAG2 loss as detected by immunohistochemistry may be associated with more advanced disease (p = 0.15 and a modest decrease in overall survival (p = 0.10. These results significantly advance our understanding of the genomic and molecular underpinnings of Ewing sarcoma and provide a foundation towards further efforts to improve diagnosis, prognosis, and precision therapeutics testing.
Silveira, Sara Martoreli; Villacis, Rolando Andre Rios; Marchi, Fabio Albuquerque;
Undifferentiated high-grade pleomorphic sarcomas (UPSs) display aggressive clinical behavior and frequently develop local recurrence and distant metastasis. Because these sarcomas often share similar morphological patterns with other tumors, particularly leiomyosarcomas (LMSs), classification...
Hu, Jessie; Kent, Paul; Lennon, Joshua M; Logan, Latania K
Acute necrotising ulcerative gingivitis is an acute onset disease characterised by ulceration, necrosis, pain and bleeding in gingival surfaces. It is predominantly seen in severely malnourished children and young adults with advanced HIV infection. We present a unique presentation in a young adult with high-grade osteogenic sarcoma.
Julia Maria Matera
Full Text Available The feline injection-site sarcoma (FIS is a challenge for the veterinarian and the affected cat’s owner. The injectable applications (vaccines, medications seems to be the reason for that neoplasia, more specifically, the inflammation caused by injury of given drugs or antigens to the health tissue. Generally the FIS presents a more aggressive behavior when compared to sarcoma not associated to application. The most effective treatment has not been established yet, but it is believed that a multimodality of therapies, surgery, radiotherapy, and chemotherapy would be the most indicated option. The knowledge of the illness in all of its aspects will supply to professionals colleges subsidies in relation to the best way to approach its diagnosis and treatment.O sarcoma de aplicação felino (SAF é atualmente um grande desafio para o médico veterinário e também para o proprietário do felino acometido. Aplicações injetáveis por via subcutânea ou intramuscular, como vacinas e medicações, aparecem como iniciadoras do processo de neogênese dessa neoplasia, mais precisamente a inflamação persistente, causada pela lesão ao tecido sadio decorrente do fármaco ou antígeno administrado. Geralmente o SAF apresenta comportamento mais agressivo quando comparado ao sarcoma não associado à aplicação. O tratamento mais eficaz ainda não está estabelecido, mas acredita-se que a multimodalidade de terapias, cirurgia, radioterapia e quimioterapia seja a opção mais indicada. O conhecimento da afecção em todos os seus aspectos irá fornecer aos colegas profissionais subsídios em relação a melhor maneira de abordá-la em termos de diagnóstico, tratamento e prevenção.
Charbonneau, Bridget; Vogel, Rachel Isaksson; Manivel, J Carlos; Rizzardi, Anthony; Schmechel, Stephen C; Ognjanovic, Simona; Subramanian, Subbaya; Largaespada, David; Weigel, Brenda
Although rare, synovial sarcoma (SS) is one of the most common soft tissue sarcomas affecting young adults. To investigate potential tumor markers related to synovial sarcoma prognosis, we carried out a single-institution retrospective analysis of 103 patients diagnosed with SS between 1980 and 2009. Clinical outcome data were obtained from medical records, and archived tissue samples were used to evaluate the relationship between progression-free survival (PFS) and several prognostic factors, including tumor expression of FGFR3 and FGFR4. No associations were found between PFS and gender, body mass index, tumor site, SS18-SSX translocation, or FGFR4 expression. As seen in previous studies, age at diagnosis (5 cm, 22% 10-year PFS; P < .0001) were associated with PFS in SS patients. In addition, in a subset of patients with available archived tumor samples taken prior to chemotherapy or radiation (n = 34), higher FGFR3 expression was associated with improved PFS (P = .030). To the best of our knowledge, this is the largest study of SS to date to suggest a potential clinical role for FGFR3. While small numbers make this investigation somewhat exploratory, the findings merit future investigation on a larger scale.
Gasparetto, Taisa Davaus; Marchiori, Edson; Lourenço, Sílvia; Zanetti, Gláucia; Vianna, Alberto Domingues; Santos, Alair A S M D; Nobre, Luiz Felipe
Kaposi sarcoma is a low-grade mesenchymal tumor involving blood and lymphatic vessels. There are four variants of this disease, each presenting a different clinical manifestation: classic or sporadic, African or endemic, organ transplant-related or iatrogenic, and AIDS-related or epidemic. Kaposi sarcoma is the most common tumor among patients with HIV infection, occurring predominantly in homosexual or bisexual men. The pulmonary involvement in Kaposi sarcoma occurs commonly in critically immunosupressed patients who commonly have had preceding mucocutaneous or digestive involvement.The etiology of Kaposi sarcoma is not precisely established; genetic, hormonal, and immune factors, as well as infectious agents, have all been implicated. There is evidence from epidemiologic, serologic, and molecular studies that Kaposi sarcoma is associated with human herpes virus type 8 infection. The disease starts as a reactive polyclonal angioproliferative response towards this virus, in which polyclonal cells change to form oligoclonal cell populations that expand and undergo malignant transformation.The diagnosis of pulmonary involvement in Kaposi sarcoma usually can be made by a combination of clinical, radiographic, and laboratory findings, together with the results of bronchoscopy and transbronchial biopsy. Chest high-resolution computed tomography scans commonly reveal peribronchovascular and interlobular septal thickening, bilateral and symmetric ill-defined nodules in a peribronchovascular distribution, fissural nodularity, mediastinal adenopathies, and pleural effusions. Correlation between the high-resolution computed tomography findings and the pathology revealed by histopathological analysis demonstrate that the areas of central peribronchovascular infiltration represent tumor growth involving the bronchovascular bundles, with nodules corresponding to proliferations of neoplastic cells into the pulmonary parenchyma. The interlobular septal thickening may represent
Full Text Available Abstract Kaposi sarcoma is a low-grade mesenchymal tumor involving blood and lymphatic vessels. There are four variants of this disease, each presenting a different clinical manifestation: classic or sporadic, African or endemic, organ transplant-related or iatrogenic, and AIDS-related or epidemic. Kaposi sarcoma is the most common tumor among patients with HIV infection, occurring predominantly in homosexual or bisexual men. The pulmonary involvement in Kaposi sarcoma occurs commonly in critically immunosupressed patients who commonly have had preceding mucocutaneous or digestive involvement. The etiology of Kaposi sarcoma is not precisely established; genetic, hormonal, and immune factors, as well as infectious agents, have all been implicated. There is evidence from epidemiologic, serologic, and molecular studies that Kaposi sarcoma is associated with human herpes virus type 8 infection. The disease starts as a reactive polyclonal angioproliferative response towards this virus, in which polyclonal cells change to form oligoclonal cell populations that expand and undergo malignant transformation. The diagnosis of pulmonary involvement in Kaposi sarcoma usually can be made by a combination of clinical, radiographic, and laboratory findings, together with the results of bronchoscopy and transbronchial biopsy. Chest high-resolution computed tomography scans commonly reveal peribronchovascular and interlobular septal thickening, bilateral and symmetric ill-defined nodules in a peribronchovascular distribution, fissural nodularity, mediastinal adenopathies, and pleural effusions. Correlation between the high-resolution computed tomography findings and the pathology revealed by histopathological analysis demonstrate that the areas of central peribronchovascular infiltration represent tumor growth involving the bronchovascular bundles, with nodules corresponding to proliferations of neoplastic cells into the pulmonary parenchyma. The interlobular
Full Text Available Abstract Background Undifferentiated embryonal sarcoma (UES of liver is a rare malignant neoplasm, which affects mostly the pediatric population accounting for 13% of pediatric hepatic malignancies, a few cases has been reported in adults. Case presentation We report a case of undifferentiated embryonal sarcoma of the liver in a 20-year-old Caucasian male. The patient was referred to us for further investigation after a laparotomy in a district hospital for spontaneous abdominal hemorrhage, which was due to a liver mass. After a through evaluation with computed tomography scan and magnetic resonance imaging of the liver and taking into consideration the previous history of the patient, it was decided to surgically explore the patient. Resection of I–IV and VIII hepatic lobe. Patient developed disseminated intravascular coagulation one day after the surgery and died the next day. Conclusion It is a rare, highly malignant hepatic neoplasm, affecting almost exclusively the pediatric population. The prognosis is poor but recent evidence has shown that long-term survival is possible after complete surgical resection with or without postoperative chemotherapy.
Lin, JoAnn M; Heath, Jonathon E; Twaddell, William S; Castellani, Rudy J
Undifferentiated embryonal sarcoma of the liver (UESL) is an uncommon hepatic tumor usually found in children, with rare cases reported in adults. We present a case of a 53-year-old woman with an undifferentiated sarcoma of the liver (USL), which resembles UESL, who initially presented with a markedly elevated hematocrit (61.2%). Cytogenetic studies for polycythemia vera were negative, but the patient's erythropoietin (EPO) was elevated. A computed tomography scan and subsequent partial hepatectomy revealed a well-circumscribed, partially cystic mass in the right lobe of the liver measuring 34 cm. Following surgery, the patient's EPO level and hematocrit dropped to within normal range and remained so for 1 year, at which point it rose again. A subsequent magnetic resonance imaging scan showed a liver mass at the previous resection margin, consistent with a recurrence. In this case study, we describe the first reported USL resembling an UESL that secretes EPO, which was a useful marker of tumor recurrence. © The Author(s) 2013.
Morel, Melanie; Taieb, Sophie; Ceugnart, Luc [Centre Oscar Lambret, Department of Radiology, Lille (France); Penel, Nicolas [Centre Oscar Lambret, Department of Oncology, Lille (France); Mortier, Laurent [Centre Hospitalier Universitaire de Lille, Department of Dermatology, Hopital Claude Huriez, Lille (France); Vanseymortier, Luc [Centre Oscar Lambret, Department of Surgery, Lille (France); Robin, Y.M. [Centre Oscar Lambret, Departement of Pathology, Lille (France); Gosset, Pierre [Groupement Hospitalier de l' Institut Catholique-Faculte Libre de Medecine de Lille, Department of Pathology, Hopital Saint-Philibert, Lomme (France); Cotten, Anne [Centre Hospitalier Universitaire de Lille, Department of Musculoskeletal Radiology, Centre Hopital Roger Salengro, Lille (France)
Superficial soft-tissue sarcomas are malignant mesenchymal tumors located within the cutaneous and/or subcutaneous layers. Most superficial soft-tissue sarcomas are low-grade tumors; yet, the risk of local recurrence is high, and initial wide surgery is the main prognostic factor. Some of these superficial sarcomas may grow, following an infiltrative pattern, and their real extent may be underestimated clinically. Imaging techniques are useful to determine precisely the real margins of the tumor, especially in cases of clinically doubtful or recurrent or large superficial lesions. Imaging tools enable one to determine the relationship with the superficial fascia separating the subcutaneous layer from the underlying muscle. In our institution ultrasonographic examination is followed by magnetic resonance (MR) imaging when the size of the lesion exceeds 3-5 cm. Imaging assessment is performed prior to biopsy, enabling optimal surgical management. Imaging features of the main superficial sarcomas are detailed in the following article, according to their major locations: those arising in the epidermis and/or dermis, which are most often diagnosed by dermatologists, and the subcutaneous sarcomas. (orig.)
Paul M. Neilsen
Full Text Available The p53 tumour suppressor plays a pivotal role in the prevention of oncogenic transformation. Cancers frequently evade the potent antitumour surveillance mechanisms of p53 through mutation of the TP53 gene, with approximately 50% of all human malignancies expressing dysfunctional, mutated p53 proteins. Interestingly, genetic lesions in the TP53 gene are only observed in 10% of Ewing Sarcomas, with the majority of these sarcomas expressing a functional wild-type p53. In addition, the p53 downstream signaling pathways and DNA-damage cell cycle checkpoints remain functionally intact in these sarcomas. This paper summarizes recent insights into the functional capabilities and regulation of p53 in Ewing Sarcoma, with a particular focus on the cross-talk between p53 and the EWS-FLI1 gene rearrangement frequently associated with this disease. The development of several activators of p53 is discussed, with recent evidence demonstrating the potential of small molecule p53 activators as a promising systemic therapeutic approach for the treatment of Ewing Sarcomas with wild-type p53.
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)
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.)
Full Text Available Abstract Background Epithelioid sarcoma is an uncommon high-grade sarcoma, mostly involving the extremities. Case presentation A 33-year-old man was referred to our institute with a diagnosis of Volkmann's contracture with the symptom of flexion contracture of the fingers associated with swelling in his left forearm. Magnetic resonance imaging (MRI showed abnormal signal intensity, comprising iso-signal intensity on T1- and high-signal intensity on T2-weighted images surrounding the flexor tendons in the forearm. Diagnosis of epithelioid sarcoma was made by open biopsy, and amputation at the upper arm was then undertaken. [18F]-2-fluoro-2-deoxy-D-glucose-positron emission tomography (FDG-PET detected multiple lesions with an increased uptake in the right neck, the bilateral upper arms and the right thigh, as well as in the left axillary lymph nodes, with maximum standardized uptake value (SUVmax ranging from 2.0 to 5.5 g/ml. Magnetic resonance imaging confirmed that there was a lesion within the right thigh muscle which was suggestive of metastasis, even though the lesion was occult clinically. Conclusion Increased uptake on FDG-PET might be representative of epithelioid sarcoma, and for this reason FDG-PET may be useful for detecting metastasis. Muscle metastasis is not well documented in epithelioid sarcoma. Accordingly, the frequency of muscle metastasis, including occult metastasis, needs to be further analyzed.
Lieber, M.R.; Winans, C.S.; Griem, M.L.; Moossa, R.; Elner, V.M.; Franklin, W.A.
Therapeutic gastric irradiation has been used to reduce peptic juice secretion in patients with peptic ulcer disease. Between 1937 and 1968 a total of 2049 patients received such therapy at the University of Chicago. Three of these patients are known to have developed sarcomas in the field of radiation. Two gastric leiomyosarcomas of the stomach were diagnosed 26 and 14 years after treatment and a malignant fibrous histiocytoma of the anterior chest wall was removed six years after gastric irradiation. Of 743 peptic ulcer patients treated without irradiation and constituted as a control group for the study of therapeutic gastric radiation, none is known to have developed sarcoma. As the incidence of sarcoma in these patient groups is known only from the tumor registry of the University of Chicago, other cases of sarcoma may exist in the groups. While an increased incidence of sarcoma has not been proven to occur in patients who received therapeutic gastric irradiation for peptic ulcer disease, the possibility of such a risk should be borne in mind by physicians caring for such patients.
Seddon, Beatrice M., E-mail: email@example.com [London Sarcoma Service, Department of Oncology, University College Hospital, 1st Floor Central, 250 Euston Road, London, NW1 2PG (United Kingdom); Davda, Reena [London Sarcoma Service, Department of Oncology, University College Hospital, 1st Floor Central, 250 Euston Road, London, NW1 2PG (United Kingdom)
Uterine sarcomas are a group of rare tumours that provide considerable challenges in their treatment. Radiological diagnosis prior to hysterectomy is difficult, with the diagnosis frequently made post-operatively. Current staging systems have been unsatisfactory, although a new FIGO staging system specifically for uterine sarcomas has now been introduced, and may allow better grouping of patients according to expected prognosis. While the mainstay of treatment of early disease is a total abdominal hysterectomy, it is less clear whether routine oophorectomy or lymphadenectomy is necessary. Adjuvant pelvic radiotherapy may improve local tumour control in high risk patients, but is not associated with an overall survival benefit. Similarly there is no good evidence for the routine use of adjuvant chemotherapy. For advanced leiomyosarcoma, newer chemotherapy agents including gemcitabine and docetaxel, and trabectedin, offer some promise, while hormonal therapies appear to be more useful in endometrial stromal sarcoma. Novel targeted agents are now being introduced for sarcomas, and uterine sarcomas, and show some indications of activity. Non-pharmacological treatments, including surgical metastatectomy, radiofrequency ablation, and CyberKnife radiotherapy, are important additions to systemic therapy for advanced metastatic disease.
Sitanggang Firman P
Full Text Available Synovial sarcoma is the fourth most common type of sarcoma. It represents between 5% -10% of all soft tissue sarcomas and most prevalent in aged between 15 - 40 years. Synovial sarcoma is the most commonly misdiagnosed soft tissue malignancy, initially as an inflammation process,often because it may be slow-growing, have a benign appearance on imaging studies, may vary in size, and may have pain similar to that associated with trauma. A rare case is presented of 17 years old women with a synovial sarcoma biphasic. The primary tumor originated in the left elbow since 2004. Since then, the patient has had repeated passive or active left elbow pain and tenderness. No history of trauma. She has not developed metastases of the lung. The patient refused to have surgery and others medical procedures that already planned and explained to her. The conclusion of this case report point that radiology is important to diagnosis and planning for further management
Full Text Available Myxoid soft-tissue sarcomas represent a heterogeneous group of mesenchymal tumors characterized by a predominantly myxoid matrix, including myxoid liposarcoma (MLS, low-grade fibromyxoid sarcoma (LGFMS, extraskeletal myxoid chondrosarcoma (EMC, myxofibrosarcoma, myxoinflammatory fibroblastic sarcoma (MIFS, and myxoid dermatofibrosarcoma protuberans (DFSP. Cytogenetic and molecular genetic analyses have shown that many of these sarcomas are characterized by recurrent chromosomal translocations resulting in highly specific fusion genes (e.g., FUS-DDIT3 in MLS, FUS-CREB3L2 in LGFMS, EWSR1-NR4A3 in EMC, and COL1A1-PDGFB in myxoid DFSP. Moreover, recent molecular analysis has demonstrated a translocation t(1; 10(p22; q24 resulting in transcriptional upregulation of FGF8 and NPM3 in MIFS. Most recently, the presence of TGFBR3 and MGEA5 rearrangements has been identified in a subset of MIFS. These genetic alterations can be utilized as an adjunct in diagnostically challenging cases. In contrast, most myxofibrosarcomas have complex karyotypes lacking specific genetic alterations. This paper focuses on the cytogenetic and molecular genetic findings of myxoid soft-tissue sarcomas as well as their clinicopathological characteristics.
The entity and nosology of pleomorphic malignant fibrous histiocytoma (MFH) is still ambiguous. The actual WHO-Classification uses pleomorphic malignant fibrous histiocytoma (MFH) and pleomorphic sarcoma NOS (not otherwise specified) synonymously. On the other hand text and illustrations convey the impression, that these tumors also could be pleomorphic lipo-, leio- or rhabdomyosarcomas etc. It would have been more informative to emphasize, that with the above mentioned specific sarcoma types MFH-like appearance may occur. Furthermore it would have been more up to date to consider pleomorphic sarcomas NOS as pleomorphic fibrosarcomas and include them in the chapter of fibroblastic and myofibroblastic tumors. This concept already has been carried out for the former myxoid variant of MFH, nowadays preferentially called myxofibrosarcoma. There is controversial discussion about the clinical significance of exact typing of pleomorphic sarcomas. Problems may also occur due to the lack of standards, which degree of desmin expression signifies leiomyosarcoma or just indicates myofibroblasts in MFH. The requirement of exclusion of other tumor-types before diagnosing pleomorphic fibrosarcoma still remains obligatory. After verification of the diagnosis pleomorphic sarcoma NOS or pleomorphic fibrosarcoma, grading e.g. according to criteria of the FFCCS can be carried out. Most cases of pleomorphic fibrosarcoma will qualify as high grade malignant.
Full Text Available Ewing’s sarcoma is a primary bone cancer that mainly affects the long bones. This malignancy is particularly common in pediatric patients. Primary cranial involvement accounts for 1% of cases, with occipital involvement considered extremely rare. In this case study, primary occipital Ewing’s sarcoma with a posterior fossa mass and subsequent relapse resulting in spinal seeding is reported. A 3-year-old patient presented with a 1-year history of left-sided headaches, localized over the occipital bone with progressive torticollis. Computed tomography (CT imaging showed a mass in the left posterior fossa compressing the brainstem. The patient then underwent surgical excision followed by adjuvant chemoradiation therapy. Two years later, the patient presented with severe lower back pain and urinary incontinence. Whole-spine magnetic resonance imaging (MRI showed cerebrospinal fluid (CSF seeding from the L5 to the S4 vertebrae. Primary cranial Ewing’s sarcoma is considered in the differential diagnosis of children with extra-axial posterior fossa mass associated with destructive permeative bone lesions. Although primary cranial Ewing’s sarcoma typically has good prognosis, our patient developed metastasis in the lower spine. Therefore, with CNS Ewing’s sarcoma, screening of the entire neural axis should be taken into consideration for early detection of CSF seeding metastasis in order to decrease the associated morbidity and mortality.
Kyle J. Sanniec
Full Text Available Background. Sarcoma is a rare malignancy, and more recent management algorithms emphasize a multidisciplinary approach and limb salvage, which has resulted in an increase in overall survival and limb preservation. However, limb salvage has resulted in a higher rate of wound complications. Objective. To compare the complications between immediate and delayed (>three weeks reconstruction in the multidisciplinary limb salvage sarcoma patient population. Methods. A ten-year retrospective review of patients who underwent sarcoma resection was performed. The outcome of interest was wound complication in the postoperative period based on timing of reconstruction. We defined infection as any infection requiring intravenous antibiotics, partial flap failure as any flap requiring a debridement or revision, hematoma/seroma as any hematoma/seroma requiring drainage, and wound dehiscence as a wound that was not completely intact by three weeks postoperatively. Results. 70 (17 delayed, 53 immediate patients who underwent sarcoma resection and reconstruction met the inclusion criteria. Delayed reconstruction significantly increased the incidence of postoperative wound infection and wound dehiscence. There was no difference in partial or total flap loss, hematoma, or seroma between the two groups. Discussion and Conclusion. Immediate reconstruction results in decreased wound complications may reduce the morbidity associated with multidisciplinary treatment in the limb salvage sarcoma patient.
Full Text Available Abstract Background Tyrosine kinase inhibitors (TKIs have gained much attention in recent years as targeted agents for the treatment of a wide range of human cancers. We have investigated the effect of the TKIs gefitinib and vandetanib on tumor cell lines derived from Ewing sarcoma, a highly malignant tumor affecting bone and soft tissue in children and young adults. Gefitinib is an inhibitor of epidermal growth factor receptor tyrosine kinase activity (EGFR and vandetanib selectively targets vascular endothelial growth factor receptor-2 (VEGFR-2 with additional activity against VEGFR-3, EGFR and RET kinase receptors. Results Two Ewing sarcoma cell lines investigated showed high levels of nuclear EGFR expression as well as moderate expression in plasma membrane and cytoplasm. When treated with concentrations of 5 μM and more of either gefitinib or vandetanib, we observed a significant decrease in cell proliferation. However, there were no detectable changes in p44/42 MAPK and Akt-1 phosphorylation, or in the expression of cyclin D1 or c-Myc following gefitinib or vandetanib treatment. Conclusion We conclude that Ewing sarcoma tumor cell proliferation is not highly sensitive to inhibition of EGFR signaling alone or the simultaneous inhibition of VEGFR receptors, EGFR and RET kinase. Decreased tumor cell proliferation could be achieved with gefitinib and vandetanib, but only at higher doses where non-specific effects of the compounds may be overriding. As Ewing tumor cells do not seem to depend on EGFR and VEGFR pathways for survival, other key factors in the cellular signaling of Ewing sarcoma should be targeted in order to obtain a potent therapeutic response.
Joual, A; Faik, H; Rabii, R; Hafiani, M; Bennani, S; el Mrini, M; Benjelloun, S
Retroperitoneal soft tissue sarcomas (RPS) are uncommon tumors. The diagnosis is frequently made later in the evolution of the disease due to the absence of specific symptomatology. Surgery with total resection of the tumor is the treatment of choice, but is only possible in 38 to 75% of cases. Six cases of RPS have been retrospectively reviewed; the mean time to diagnosis was 5 months; diagnosis was established via CT scan, which determined the retroperitoneal tumor location and its relationship to the neighboring organs. The most common symptoms were the presence of an abdominal mass and accompanying abdominal pain; signs of urinary and vascular compression were not found until later. Surgery with total resection was performed in 4 cases, and with partial resection in 2 cases. The histological findings were as follows: 3 liposarcomas, 2 rhabdomyosarcomas, and 1 fibrosarcoma. Tumor recurrence developed in 2 cases (liposarcomas), necessitating further surgery and complete resection. Two patients were lost to follow-up. RPS are characterized by locoregional relapse and metastases. Disease outcome depends on the histological type, tumor grade, and on the possibility of carrying out complete resection.
Ileana Agramonte Centelles
Full Text Available Paciente masculino de 29 años de edad, raza blanca, soltero, profesor universitario, con antecedentes de padecer crisis de epilepsia tratado con fenitoína y actualmente controlado, menciona que desde hace aproximadamente 4 semanas comenzó con ojo rojo y molestias oculares del ojo derecho, por lo cual acudió a su área de salud donde fue tratado como cuadro de conjuntivitis. No mostró mejoría alguna, sino empeoramiento del cuadro clínico, y observó un enrojecimiento ocular intenso en el ángulo interno de dicho ojo que se fue extendiendo, acompañado de ligera fotofobia. Por la tórpida evolución del cuadro decidió acudir a nuestra institución por lo cual fue remitido a la Consulta de Oculoplastia. También refirió que desde hacía dos meses había presentado anorexia, dificultad al comer, así como pérdida de peso, por lo cual se decidió comenzar estudio y tratamiento. Se decidió realizar la resección de la masa tumoral en conjuntiva bulbar y se envió para estudio anatomopatológico. El resultado fue compatible con un sarcoma de Kaposi.
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.
Full Text Available Extraosseous Ewing’s sarcoma (EES involving the central nervous system is rare, but can be diagnosed and distinguished from other primitive neuroectodermal tumors (PNET by identification of the chromosomal translocation (11;22(q24;q12. We report EES arising from the spinal intradural extramedullary space, based on imaging, histopathological, and molecular data in two men, ages 50 and 60 years old and a review of the literature using PubMed (1970-2009. Reverse transcriptase polymerase chain reaction (RT-PCR identified the fusion product FL1-EWS. Multimodal therapy, including radiation and alternating chemotherapy including vincristine, cyclophosphamide, doxorubicin and ifosfamide and etoposide led to local tumor control and an initial, favorable therapeutic response. No systemic involvement was seen from the time of diagnosis to the time of last follow-up (26 months or death (4 years. This report confirms that EES is not confined to the earliest decades of life, and like its rare occurrence as an extra-axial meningeal based mass intracranially, can occasionally present as an intradural mass in the spinal canal without evidence of systemic tumor. Gross total resection followed by multimodal therapy may provide for extended progression free and overall survival.
Jesús Ramón León Polanco
Full Text Available Se presenta el caso de un paciente masculino de 33 años de edad, con antecedentes de VIH-SIDA desde hace 10 años, que se mantiene en tratamiento con antirretrovirales. Durante todo este tiempo ha presentado varios episodios de infecciones respiratorias, incluyendo tuberculosis pulmonar 5 años atrás. Acude a consulta refiriendo edemas en miembros inferiores acompañado de lesiones en piel de color violáceo de un año de evolución, previamente interpretado como linfangitis rebelde al tratamiento y que se extendió a la cara interna de los muslos y a los miembros inferiores. Con pérdida de peso, no prurito en las lesiones, fiebre, lesiones en la mucosa oral. Se determinó hemoglobina 89 g/L, leucocitos 4,5 x 109 /L, se estudiaron las funciones hepática y renales resultando normales. Radiografías de tórax y ultrasonido abdominal normales. Se realizó estudio anatomopatológico de piel que informó Sarcoma de Kaposi. Se impuso tratamiento con quimioterapia
Radoje B Colovic; Nikica M Grubor; Marjan T Micev; Slavko V Matic; Henry Dushan Edward Atkinson; Stojan M Latincic
Ewing's sarcoma (ES) is a neoplasm of undifferentiated small round cells, which occurs in the bones and deep soft tissues of children and adolescents. We present a rare case of a 44-year-old woman with gastric ES presenting with epigastric pain and weight loss. Ultrasound and computed tomography scans indicated a solid/cystic mass in the pancreatic tail. At laparotomy, the tumor was found attached to the posterior surface of the stomach, completely free from the pancreas, with no lymphadenopathy or local metastases. The polynodal, partly pseudocystic, dark-red soft tumor was excised. Histopathology revealed an anaplastic small-round-cell tumor with strong membranous CD99 immunoexpression. Additionally, there was patchy immunostaining for S-100 protein, vimentin, protein gene product (PGP) 9.5 and neuron-specific enolase, and weak focal CD117 cytoplasmic immunoreactivity. The patient had no adjuvant chemotherapy; her postoperative recovery was uneventful, and she remains symptom-free, and without any sign of recurrence at 20 mo. To the best of our knowledge, this is only the third ever case of gastric ES.
Luksch, Roberto; Grignani, Giovanni; D'Angelo, Paolo; Prete, Arcangelo; Puma, Nadia; Podda, Marta; Casanova, Michela; Ferrari, Andrea; Morosi, Carlo; Fagioli, Franca; Aglietta, Massimo; Ferrari, Stefano; Picci, Piero; Massimino, Maura
The aim was to assess the activity of cisplatin (CDDP) in Ewing sarcoma (ES). The study consisted of front-line window therapy with CDDP 120 mg/sqm every 3 weeks for two courses in children and young adults with primary disseminated ES. Response was assessed using the Response Evaluation Criteria in Solid Tumours criteria, and Simon's two-stage design was applied. Twelve consecutive patients were enrolled in stage 1. Only one objective response was observed. Since the target response rate was not achieved, accrual was stopped and CDDP as a single agent in ES was judged unworthy of further assessment. © 2017 Wiley Periodicals, Inc.
Xu Deyong; Zhan Alai; Luan Hongmei; Feng Weihua; Sun Xihe; Yang Zuwen
Objective: To study the clinical characteristics and radiological features of synovial sarcoma in childhood and its relation to the diagnosis and treatment. Methods:The clinical radiological features of 15 children with synovial sarcoma proved surgically and pathologically were analyzed. Results: In children, the tumor boundaries are poorly defined due to paucity of fat, and metastasis usually occurs early. Eight patients in this series had bone involvement, including: direct erosion by tumor causing cortical destruction, indirect pressure defect with sharp margin and reactive bone sclerosis and bone destruction of the primary intraosseous synovial sarcoma.Conclusion: The tumor is often misdiagnosed, the final confirmed diagnosis must be made by histological examination with imaging findings. It is emphasized that the patients should be treated with radiotherapy and chemotherapy preoperatively and postoperatively.
Goodman, Laura F.; Bateni, Cyrus P.; Bishop, John W.; Canter, Robert J.
Complications from lost gallstones after cholecystectomy are rare but varied from simple perihepatic abscess to empyema and expectoration of gallstones. Gallstone complications have been reported in nearly every organ system, although reports of malignant masquerade of retained gallstones are few. We present the case of an 87-year-old woman with a flank soft tissue tumor 4 years after laparoscopic cholecystectomy. The initial clinical, radiographic and biopsy findings were consistent with soft tissue sarcoma (STS), but careful review of her case in multidisciplinary conference raised the suspicion for retained gallstones rather than STS. The patient was treated with incisional biopsy/drainage of the mass, and gallstones were retrieved. The patient recovered completely without an extensive resectional procedure, emphasizing the importance of multidisciplinary sarcoma care to optimize outcomes for potential sarcoma patients. PMID:27333918
Full Text Available Primary lung sarcoma is an extremely rare tumor, accounting for less than 0.5% of all lung tumors. Histological subtypes are differentiated on the basis of immunohistochemical markers, such as vimentin, desmin, actin, CD99, and epithelial membrane antigen. A 50-year-old male presented with progressively increasing shortness of breath with cough for 2 months. On Contrast Enhanced Computed Tomography (CECT of thorax a large heterogeneous mass with multiple areas of necrosis, occupying almost whole of left hemithorax was seen. CT-guided Fine Needle Aspiration Cytology (FNAC revealed spindle cell neoplasm. Histopathological examination revealed a spindle cell sarcoma. On immunohistochemistry the tumor cells expressed both epithelial membrane antigen and vimentin. Hence, final impression from immunohistochemistry was primary monophasic synovial sarcoma of lung. [Int J Res Med Sci 2014; 2(4.000: 1729-1731
Winkler, B; Grapow, M; Seeberger, M; Matt, P; Aulitzky, W; Eckstein, F
Synovial sarcoma metastasis affecting the heart and infiltrating the mitral valve is a very rare pathology. We report the case of a 44-year-old male treated with chemotherapy for atypical synovial sarcoma of the oral mucosa who presented to our clinic after cardiac decompensation with a presumptive diagnosis of myxoma of the left atrium. A large necrotic tumour positive for CK 22, EMA, CD 99 and BCL-2 but negative for translocation in COBRA-FISH analysis by break-apart probe could be excised and revealed a very rare subtype of synovial sarcoma metastasis arising from the endocard of the left atrium. The tumour was resected and the mitral valve reconstructed through ring annuloplasty. Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.
Gilberto Serrano Ocaña
Full Text Available Before the AIDS epidemic, Kaposi's sarcoma was found mainly in elderly men of Mediterranean coast, eastern European background and Jewish ancestry (rarely in older women and is a slow growing skin tumor. In AIDS patients, the KS tends to develop more rapidly compromising the skin, lungs, gastrointestinal tract and other organs. In people with AIDS, Kaposi's sarcoma is caused by an interaction between HIV, a weakened immune system and human herpes virus 8. It affects approximately 20% of people with HIV that don’t take antiretroviral drugs. It is more common in homosexual’s patients, but may appear in any HIV positive individual, in Africa where heterosexual HIV transmission route is the most important can also be found in children and women. We are presenting a case of Kaposi sarcoma in a young female admitted at the Internal Medicine Department of Dora Nginza Hospital.
Demetri, George D; Blay, Jean-Yves; Casali, Paolo G
Intensive clinical research in the sarcoma field has provided insight into the histopathological diversity of soft tissue sarcomas (STS) and led to the introduction of many new agents that promise to play an important role in the management of patients with STS. While an increasing body of scientific data has advanced our knowledge of this complex family of mesenchymal diseases, several controversies remain to be resolved: Is doxorubicin-based therapy still the definitive standard first-line treatment for all patients with unresectable and/or metastatic STS of all subtypes? Is histology-driven therapy beyond gastrointestinal stromal tumors a reality or are we pursuing an unachievable objective? Are we making practical headway in the establishment of sarcoma reference centers? Is it clearly established which is the best parameter to evaluate the efficacy of a new agent in STS?
Barberis, M; Brenna Betti, N; Lauritano, D; Sangiani, L; Spadari, F; Villa, S
The epidemic form of Kaposi's sarcoma is the most frequent tumor in sieropositive patients. Every part of the body including oral cavity is affected by these lesions. According to modern acknowledgement in treating oropharynge carcinoma, radiotherapy is used for management of oral Kaposi's sarcoma. This paper reports a study of 10 patients suffering from Kaposi's sarcoma correlated to AIDS (EKS) treated with radiotherapy and chemiotherapy, achieving good results, at the Istituto Nazionale per lo Studio e la Cura dei Tumori of Milan (Divisone di Radioterapia C) from 1988 to 1992. Treatment has been performed using linear accelerator (6 Mev) or Co 60 unity in order to reach the deepest layer of mucosa lesions. Radiotherapy schedule consisted of 150-200 cGy daily fractions given 5 times/week (w) for 4-5 w in split-course.
Jordanov, Martin I.; Block, John J. [Vanderbilt University Medical Center, Department of Radiology and Radiological Sciences, Nashville, TN (United States); Gonzalez, Adriana L. [Vanderbilt University Medical Center, Department of Pathology, Nashville, TN (United States); Green, Neil E. [Vanderbilt Children' s Hospital, Department of Pediatric Orthopaedics, Nashville, TN (United States)
Transarticular spread of tumor is rare; it has only been reported in the sacroiliac joint, intervertebral disk spaces, and facet joints. The anatomic and kinetic characteristics of the sacroiliac joint, as well as the changes the joint undergoes during a lifetime, make it particularly vulnerable to transarticular tumor invasion. Although extremely rare, Ewing sarcoma can extend through the sacroiliac joint and be virtually indistinguishable radiologically from septic arthritis. Furthermore, the clinical presentation of a child with Ewing sarcoma can be similar to that of a child with osteomyelitis. Laboratory values are quite nonspecific and are not always helpful in differentiating between the entities. Therefore, the possibility of sacroiliac joint transarticular Ewing sarcoma should be considered in a child presenting with hip pain, despite clinical, radiological and laboratory findings suggesting an infectious process. (orig.)
Kim, Yong Hee; Kim, Myung Joon; Lee, Mi Jung [Dept. of Radiology and Research Institute of Radiological Science, Severance Children' s Hospital, Yonsei University College of Medicine, Seoul (Korea, Republic of); Kim, Se Hwa [Dept. of Pathology, Severance Hospital, Yonsei University College of Medicine, Seoul (Korea, Republic of)
Undifferentiated sarcomas are rare tumors not classified into any sarcoma subtype. Due to their rarity, imaging findings of undifferentiated sarcomas are poorly characterized. The purpose of this report was to present imaging findings of a pathologically confirmed undifferentiated sarcoma originated from the left kidney of a 12-year-old boy. The mass was infiltrative involving the renal pelvis. It mimicked massive hilar lymphadenopathy with a preserved renal contour visible by both ultrasonography and CT. Renal vein thrombosis was also observed. Although undifferentiated sarcomas are rare, they should be considered in differential diagnosis of infiltrative renal masses with renal pelvis invasion in children.
Yoo, Jisook; Jo, Mingyul; Kim, Min-Soo; Choi, Kwang-Hyun; Park, Hyang-Joon
Kaposi's sarcoma is a multifocal proliferative vascular tumor involving the skin and other organ and psoriasis is a chronic cutaneous disease with papules and plaques with white scale. Development of Kaposi's sarcoma in psoriasis patients has been reported rarely. A 71-year-old man presented with multiple brownish to violaceous plaques on both feet and arms which were found 4 months ago. The biopsy confirmed Kaposi's sarcoma. The patient was diagnosed with psoriasis vulgaris 10 years ago and Kaposi's sarcoma lesions developed between psoriatic plaques. We herein report a rare case of simultaneous occurrence of Kaposi's sarcoma and psoriasis vulgaris which need quite different treatment. PMID:27904275
Full Text Available Ewing′s sarcomas and peripheral primitive neuroectodermal tumors (ES/PNETs are high grade malignant neoplasms. These malignancies are characterized by a chromosome 22 rearrangement, arise from bone or soft tissue, predominantly affect children and young adults, and are grouped in the Ewing family of tumors. Multimodality treatment programs are the treatment of choice. Primary localization of ES/PNET in the mediastinum is extremely rare. We describe a case of ES/PNET presenting as a mediastinal mass with tracheal compression and initial signs of superior vena cava in a 66-year-old woman.
Luciana Wernersbach Pinto
Full Text Available Both multicentric Castleman disease and Kaposi sarcoma are more frequently observed in HIV infected patients. The coexistence of these Human herpesvirus 8 related lesions, in the same tissue, has been observed, but literature reports are scant. On the other hand, the expression of HHV-8-LANA-1 is easily demonstrable by immunohistochemistry. This has been shown to be a powerful tool for the diagnosis of these entities. The aim of this report is to communicate our experience with a case of multicentric Castleman disease occurring in the setting of HIV infection, which demonstrated microscopic Kaposi sarcoma in the same lymph node during the pathological work-up
Taylor, Amanda; Eichelberger, Bunita; Hodo, Carolyn; Cooper, Jocelyn; Porter, Brian
A 12-year-old mixed breed dog was presented for evaluation of progressive paraparesis and ataxia. Magnetic resonance (MR) imaging was performed and identified multifocal intradural spinal cord mass lesions. The lesions were hyperintense in T2-weighted sequences, isointense to mildly hyperintense in T1-weighted sequences with strong contrast enhancement of the intradural lesions and spinal cord meninges. Spinal cord neoplasia was suspected. A diagnosis of intramedullary spinal cord histiocytic sarcoma, confined to the central nervous system, was confirmed histopathologically. Spinal cord histiocytic sarcoma is a rare neoplasm, but should be included in the differential diagnosis for dogs with clinical signs of myelopathy.
Los sarcomas de hueso y tejidos blandos son un grupo poco frecuente de tumores. Su prevalencia es inferior a 1 caso por 10000 habitantes, lo que los convierte en una enfermedad rara. Algunos de estos tumores, como el sarcoma sinovial, el tumor de Ewing o el osteosarcoma, son más habituales en los adolescentes o en los adultos jóvenes, mientras que existen neoplasias como el leiomiosarcoma o el liposarcoma, más frecuentes en pacientes de edad superior a los 55 años. Desde el punto de vista his...
Full Text Available A 15-yr old boy presented with low backache for 4 months associated with weakness of left lower limb. MRI of lumbosacral spine showed a sacral lesion with intraspinal and presacral soft tissue extension with neural compression. A diagnosis of tuberculosis was considered in the view of high prevalence in this part of the world, however biopsy revealed Ewing's sarcoma. Ewing's tumor of sacrum is rare, but should be suspected in low backache in children. Differential diagnosis for a sacral lesion includes tuberculosis, pyogenic osteomyelitis, lymphoma, chordoma, osteosarcoma and Ewing's sarcoma. MRI is sensitive in detecting these lesions but is nonspecific requiring histopathological examination for confirmation.
Ekrem Cengiz Seyhan
Full Text Available Synovial sarcoma (SS is a rare tumor originating from mesenchymal tissue and accounting for approximately 5–10% of all soft tissue sarcomas. A rare case of primary pulmonary SS in an asymptomatic 18-year-old man admitted to our hospital for investigation of a 6 × 6.5 cm, oval-shaped, well-delineated pleural based peripheral mass in the left lower lobe in his thorax CT is presented. Left lower lobectomy was done. Immunohistochemically, tumor cells were positive for cytokeratin, epithelial membrane antigen (EMA, and vimentin so that the histopathological diagnosis was compatible with biphasic spindle cell type SS in the lung.
Radic-Sarikas, Branka; Tsafou, Kalliopi P; Emdal, Kristina B.;
including approved drugs. We were able to retrieve highly synergistic drug combinations specific for Ewing sarcoma and identified signaling processes important for Ewing sarcoma cell proliferation determined by EWS-FLI1 We generated a molecular target profile of PKC412, a multikinase inhibitor with strong...... and IGF1R inhibitors. The mechanism of the drug synergy between these inhibitors is different from the sum of the mechanisms of the single agents. The combination effectively inhibited pathway crosstalk and averted feedback loop repression, in EWS-FLI1-dependent manner. Mol Cancer Ther; 16(1); 88...
SARC is a non-for-profit organization whose mission and vision is to advocate for the collaboration on the design of clinical trials on sarcoma, to further the knowledge regarding the diagnosis and treatment of sarcoma and provide accurate and up to date information to physicians, patients and families. The objectives are to assist in the development of the infrastructure for the continued growth and spectrum of clinical research, to facilitate biannual meeting of investigators, and to develop a preclinical research base that would design and conduct research that would improve the process of drug treatments selected for clinical research trials.
Kang, Tae Wook; Lee, Soon Jin; Song, Hye Jong [Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul (Korea, Republic of)
Follicular dendritic cell sarcoma is a rare neoplasm that originates from follicular dendritic cells in lymphoid follicles. This disease usually involves the lymph nodes, and especially the head and neck area. Rarely, extranodal sites may be affected, including tonsil, the oral cavity, liver, spleen and the gastrointestinal tract. We report here on the imaging findings of follicular dendritic cell sarcoma of the abdomen that involved the retroperitoneal lymph nodes and colon. It shows as a well-defined, enhancing homogenous mass with internal necrosis and regional lymphadenopathy.
Full Text Available Clear cell sarcoma (CCS is a recently described variant of sarcoma characterized by prominent clear cells showing features similar to clear cell melanoma. This neoplasm was first described by Dr. Franz M. Erzinger. Primary CCS usually arises in deeper soft tissues, in association with fascia, tendons, or aponeuroses. Characteristic translocation t(12;22 (q13;q12 has been considered pathognomonic for CCS. Prognosis is related to tumor size. An early recognition and initial radical surgery is the key to a favourable outcome. We present a patient with an unusual neoplasm that resembled malignant melanoma.
Chernichenko, V A; Tolstopiatov, B A; Monich, A Iu; Konovalenko, V F; Galakhin, K A; Palivets, A Iu; Vorona, A M
Results of treatment of 101 cases of soft tissue sarcoma are presented in the paper. Preoperative irradiation technique and radical program of treatment are described. Combined radiation and surgical treatment was given to 45 patients whereas conservative--to 56. Sixty-three cases received adjuvant combination chemotherapy. Response and three-year survival rates were compared to those in control group treated by photons. The results observed in patients of combined and conservative treatment groups who had been irradiated with fast neutrons proved significantly better than in controls. These data suggest vistas in application of fast neutron irradiation for the treatment of soft tissue sarcomas.
Kaposi's sarcoma-associated herpesvirus (KSHV) is the primary etiological agent of Kaposi's sarcoma, primary effusion lymphoma and muticentric Castleman's disease. In common with the other herpesviruses, KSHV exhibits both latent and lytic life cycles, both of which are characterized by distinct gene expression profiles and programs. KSHV encodes proteins which play essential roles in the inhibition of host adaptive and innate immunity, the inhibition of apoptosis, and the regulation of the cell cycle. KSHV also encodes several proteins which have transforming and intrcellular signalling activity.
Bakst, Richard; Wolden, Suzanne [Department of Radiation Oncology, Memorial Sloan-Kettering Cancer Center, New York, NY (United States); Yahalom, Joachim, E-mail: firstname.lastname@example.org [Department of Radiation Oncology, Memorial Sloan-Kettering Cancer Center, New York, NY (United States)
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.
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
Full Text Available Bone sarcomas are rare tumors, approximating 0.2% of all cancers, with osteosarcoma (OGS, chondrosarcoma, and Ewing sarcoma being the most common cancers in this subset. The formation of disease management groups/clinics focused on sarcomas has resulted in better understanding and management of these uncommon tumors. Multiple large-scale retrospective data from Tata Memorial Hospital (TMH and All India Institute of Medical Sciences have reported outcomes comparable to Western data in the field of OGS and Ewing sarcoma, with interesting prognostic factors identified for further evaluation. Soft tissue sarcomas are a rare heterogeneous group of tumors, more than 50 different tumor entities. The common subtypes identified in India include Ewing sarcoma and synovial sarcoma. Valuable work regarding brachytherapy has been done by radiation oncologists from the TMH, especially in pediatric patients.
Full Text Available Sarcoma includes some of the most heterogeneous tumors, which make the diagnosis, prognosis and treatment of these rare yet diverse neoplasms especially challenging. Long noncoding RNAs (lncRNAs are important regulators of cancer initiation and progression, which implies their potential as neoteric prognostic and diagnostic markers in cancer, including sarcoma. A relationship between lncRNAs and sarcoma pathogenesis and progression is emerging. Recent studies demonstrate that lncRNAs influence sarcoma cell proliferation, metastasis, and drug resistance. Additionally, lncRNA expression profiles are predictive of sarcoma prognosis. In this review, we summarize contemporary advances in the research of lncRNA biogenesis and functions in sarcoma. We also highlight the potential for lncRNAs to become innovative diagnostic and prognostic biomarkers as well as therapeutic targets in sarcoma.
Milton Marcio Machota Junior
Full Text Available INTRODUÇÃO: O sarcoma de sistema nervoso central (SNC é uma neoplasia rara, com incidência de 0,1% a 4,3% dos tumores intracranianos. São tumores agressivos com prognóstico reservado e a maioria é tratada com ressecção radical. RELATO: Homem, 29 anos, com episódios de crises convulsivas e diagnóstico de hemorragia intraparenquimatosa. Durante a cirurgia, foi identificada lesão bem delimitada. A histologia demonstrou neoplasia fusocelular com atipias e numerosas mitoses. Os únicos marcadores imuno-histoquímicos positivos foram vimentina e S-100. O diagnóstico foi de sarcoma indiferenciado de alto grau. CONCLUSÃO: No diagnóstico diferencial de sarcomas de SNC, devem-se excluir lesões metastáticas e gliossarcoma.INTRODUCTION: The central nervous system (CNS sarcoma is a rare neoplasm with an incidence of 0.1% to 4.3% in intracranial tumors. They are aggressive with poor prognosis, and mostly treated with radical resection. REPORT: 29 year-old male patient with episodes of seizures and diagnosed with intraparenchymal hemorrhage. During the surgery a well-defined lesion was identified. Histology showed a spindle cell neoplasm with atypia and numerous mitoses. The immunohistochemical markers were positive only for vimentin and S-100. The diagnosis was high-grade undifferentiated sarcoma. CONCLUSION: Metastatic lesions and gliosarcoma should be excluded in the differential diagnosis of CNS sarcomas.
Holly Lynn Spraker
Full Text Available Ewing sarcoma (ES is the second most common bone tumor affecting primarily adolescents and young adults. Despite recent advances in biological understanding, intensification of chemotherapeutic treatments, and progress in local control with surgery and/or radiation therapy, patients with metastatic or recurrent ES continue to have a dismal prognosis with less than 20% overall survival. All ES likely is metastatic at diagnosis although our methods of detection and classification may not account for this. Progressive disease may arise via a combination of: 1 selection of chemotherapy-resistant clones, 2 signaling from bone or lung microenvironments that may attract tumor cells to distant locations, and/or 3 genetic changes within the ES cells themselves due to a combination of therapy-related selection and DNA-damaging chemotherapeutic agents. These possibilities and the evidence base to support them are explored.
Spraker, Holly L; Price, Shawn L; Chaturvedi, Aashi; Schiffman, Joshua D; Jones, Kevin B; Lessnick, Stephen L; Beckerle, Mary; Randall, R Lor
Ewing sarcoma (ES) is the second most common bone tumor affecting primarily adolescents and young adults. Despite recent advances in biological understanding, intensification of chemotherapeutic treatments, and progress in local control with surgery and/or radiation therapy, patients with metastatic or recurrent ES continue to have a dismal prognosis with less than 20% overall survival. All ES is likely metastatic at diagnosis although our methods of detection and classification may not account for this. Progressive disease may arise via a combination of: (1) selection of chemotherapy-resistant clones in primary tumor, (2) signaling from bone or lung microenvironments that may attract tumor cells to distant locations, and/or (3) genetic changes within the ES cells themselves due to DNA-damaging chemotherapeutic agents or other "hits." These possibilities and the evidence base to support them are explored.
Spraker, Holly L.; Price, Shawn L.; Chaturvedi, Aashi; Schiffman, Joshua D.; Jones, Kevin B.; Lessnick, Stephen L.; Beckerle, Mary; Randall, R. Lor
Ewing sarcoma (ES) is the second most common bone tumor affecting primarily adolescents and young adults. Despite recent advances in biological understanding, intensification of chemotherapeutic treatments, and progress in local control with surgery and/or radiation therapy, patients with metastatic or recurrent ES continue to have a dismal prognosis with less than 20% overall survival. All ES is likely metastatic at diagnosis although our methods of detection and classification may not account for this. Progressive disease may arise via a combination of: (1) selection of chemotherapy-resistant clones in primary tumor, (2) signaling from bone or lung microenvironments that may attract tumor cells to distant locations, and/or (3) genetic changes within the ES cells themselves due to DNA-damaging chemotherapeutic agents or other “hits.” These possibilities and the evidence base to support them are explored. PMID:22649772
Hugo Meister; Thaís C. Miranda; Manuel G. G. Nóbrega
O sarcoma sinovial primário da cabeça e pescoço é um tumor raro. Em torno de 90 casos foram descritos na literatura. Relatamos um caso adicional ocorrido em um paciente jovem do sexo masculino comprometendo a orofaringe. O tumor apresentou típico crescimento bifásico, fibrossarcomatoso e epitelial, com estruturas pseudo-glandulares. O diagnóstico final foi obtido pelo exame da peça cirúrgica com preparações histológicas de rotina e análise imunohistoquímica.Primary synovial sarcoma of the hea...
Wilky, B A; Kim, C; McCarty, G; Montgomery, E A; Kammers, K; DeVine, L R; Cole, R N; Raman, V; Loeb, D M
RNA helicase DDX3 has oncogenic activity in breast and lung cancers and is required for translation of complex mRNA transcripts, including those encoding key cell-cycle regulatory proteins. We sought to determine the expression and function of DDX3 in sarcoma cells, and to investigate the antitumor activity of a novel small molecule DDX3 inhibitor, RK-33. Utilizing various sarcoma cell lines, xenografts and human tissue microarrays, we measured DDX3 expression at the mRNA and protein levels, and evaluated cytotoxicity of RK-33 in sarcoma cell lines. To study the role of DDX3 in Ewing sarcoma, we generated stable DDX3-knockdown Ewing sarcoma cell lines using DDX3-specific small hairpin RNA (shRNA), and assessed oncogenic activity. DDX3-knockdown and RK-33-treated Ewing sarcoma cells were compared with wild-type cells using an isobaric mass-tag quantitative proteomics approach to identify target proteins impacted by DDX3 inhibition. Overall, we found high expression of DDX3 in numerous human sarcoma subtypes compared with non-malignant mesenchymal cells, and knockdown of DDX3 by RNA interference inhibited oncogenic activity in Ewing sarcoma cells. Treatment with RK-33 was preferentially cytotoxic to sarcoma cells, including chemotherapy-resistant Ewing sarcoma stem cells, while sparing non-malignant cells. Sensitivity to RK-33 correlated with DDX3 protein expression. Growth of human Ewing sarcoma xenografts expressing high DDX3 was inhibited by RK-33 treatment in mice, without overt toxicity. DDX3 inhibition altered the Ewing sarcoma cellular proteome, especially proteins involved in DNA replication, mRNA translation and proteasome function. These data support further investigation of the role of DDX3 in sarcomas, advancement of RK-33 to Ewing sarcoma clinical trials and development of RNA helicase inhibition as a novel anti-neoplastic strategy.
Wang, Xiaodong; Yang, Lei; Robertson, Erle S.; Lan, Ke
Kaposi's sarcoma-associated herpesvirus (KSHV) is etiologically related to Kaposi's sarcoma (KS), primary effusion lymphoma (PEL) and multicentric Castleman's disease (MCD). It typically displays two different phases in its life cycle, the default latency and occasional lytic replication. The epigenetic modifications are thought to determine the fate of KSHV infection. Previous studies elegantly depicted epigenetic landscape of latent viral genome in in vitro cell culture systems. However, the physiologically relevant scenario in clinical KS tissue samples is unclear. In the present study, we established a protocol of ChIP-Seq for clinical KS tissue samples and mapped out the epigenetic landscape of KSHV genome in classic KS tissues. We examined AcH3 and H3K27me3 histone modifications on KSHV genome, as well as the genome-wide binding sites of latency associated nuclear antigen (LANA). Our results demonstrated that the enriched AcH3 was mainly restricted at latent locus while H3K27me3 was widespread on KSHV genome in classic KS tissues. The epigenetic landscape at the region of vIRF3 gene confirmed its silenced state in KS tissues. Meanwhile, the abundant enrichment of LANA at the terminal repeat (TR) region was also validated in the classic KS tissues, however, different LANA binding sites were observed on the host genome. Furthermore, we verified the histone modifications by ChIP-qPCR and found the dominant repressive H3K27me3 at the promoter region of replication and transcription activator (RTA) in classic KS tissues. Intriguingly, we found that the TR region in classic KS tissues was lacking in AcH3 histone modifications. These data now established the epigenetic landscape of KSHV genome in classic KS tissues, which provides new insights for understanding KSHV epigenetics and pathogenesis. PMID:28118409
Eddy Millán Escalona
Full Text Available El sarcoma de Ewing es un tumor óseo maligno de células redondas pequeñas. Es el segundo tumor óseo maligno más frecuente en la infancia, y se puede presentar en cualquier momento durante la niñez y comienzos de la edad adulta. El tumor puede originarse en cualquier parte del cuerpo, generalmente en los huesos largos de las extremidades, la pelvis o el tórax, al igual que en el cráneo o en los huesos planos del tronco. Se trata de un paciente masculino, mestizo, de 38 años de edad, que acude a consulta externa en el hospital de Nickerie, Suriname, por presentar aumento de volumen en la región lateral derecha del abdomen. Refiere que ha presentado pérdida de peso, decaimiento marcado y anorexia. Estos síntomas se iniciaron 3 meses antes, y se han incrementado paulatina y progresivamente. Tiene también dificultad para deambular y dolor en la pierna derecha.The Ewing's sarcoma is a malignant bone tumor of round cells. The second malignant bone tumor more frequent in childhood and may to appear in any moment during this life stage and at onset of adulthood. Tumor may to originate in any place of body, generally in limbs long bones, pelvis or thorax as well as in skull or in trunk flat bones. This case is a black male patient aged 38 seen in external consultation in the hospital of Nickerie, Surinam presenting with a volume increase in the right lateral region of abdomen. He refers a weight loss, a marked weakness and anorexia. These symptoms appeared three months before increasing in a gradual and progressively way. He has difficulty to walk and pain in the right leg.
Full Text Available Kaposi's sarcoma (KS became a critical health issue with the emergence of acquired immunodeficiency syndrome (AIDS in the 1980s. Four clinical-epidemiological forms of KS have been described: classical KS, endemic KS, iatrogenic KS, and AIDS-associated KS. In 1994, Kaposi's sarcoma-associated herpesvirus (KSHV or human herpesvirus type 8 was identified by Chang and colleagues, and has been detected worldwide at frequencies ranging from 80 to 100%. The aim of the present study was to evaluate the frequency of KSHV infection in KS lesions from HIV-positive and HIV-negative patients in Brazil, as well as to review the current knowledge about KS transmission and detection. For these purposes, DNA from 51 cases of KS was assessed by PCR: 20 (39.2% cases of classical KS, 29 (56.9% of AIDS-associated KS and 2 (3.9% of iatrogenic KS. Most patients were males (7.5:1, M/F, and mean age was 47.9 years (SD = ± 18.7 years. As expected, HIV-positive KS patients were younger than patients with classical KS. On the other hand, patients with AIDS-associated KS have early lesions (patch and plaque compared to classical KS patients (predominantly nodular lesions. This is assumed to be the result of the early diagnose of KS in the HIV-positive setting. KSHV infection was detected by PCR in almost all cases (48/51; 94.1%, irrespectively of the clinical-epidemiological form of KS. These results show that KSHV is associated with all forms of KS in Brazilian patients, a fact that supports the role of this virus in KS pathogenesis.
Reyners, A.K.L.; Sprenger, H.G; Suurmeijer, A.J.H.; van der Graaf, W.T.A.
A 42-year-old heterosexual man presented with bluish-purple spots on his skin and in his mouth cavity that had been present for a few months; a 48-year-old homosexual man had painful lymphadenopathy in the groins and left axilla. Both men appeared to have a Kaposi's sarcoma and to be HIV-positive. D
Fossati, S; Boneschi, [No Value; Ferrucci, S; Brambilla, L
BACKGROUND. The concomitant occurrence of more than one primary neoplasm in the same individual has led researchers to seek possible common etiopathogenetic factors. Kaposi sarcoma (KS) is a multicentric neoplasm of vascular origin and perhaps viral etiology. Four forms of KS are known: classic or M
Stephen L. Lessnick
Full Text Available Numerous molecular abnormalities contribute to the genetic derangements involved in tumorigenesis. Chromosomal translocations are a frequent source of these derangements, producing unique fusion proteins with novel oncogenic properties. EWS/ETS fusions in Ewing sarcoma are a prime example of this, resulting in potent chimeric oncoproteins with novel biological properties and a unique transcriptional signature essential for oncogenesis. Recent evidence demonstrates that EWS/FLI, the most common EWS/ETS fusion in Ewing sarcoma, upregulates gene expression using a GGAA microsatellite response element dispersed throughout the human genome. These GGAA microsatellites function as enhancer elements, are sites of epigenetic regulation and are necessary for EWS/FLI DNA binding and upregulation of principal oncogenic targets. An increasing number of GGAA motifs appear to substantially enhance EWS/FLI-mediated gene expression, which has compelling biological implications as these GGAA microsatellites are highly polymorphic within and between ethnically distinct populations. Historically regarded as junk DNA, this emerging evidence clearly demonstrates that microsatellite DNA plays an instrumental role in EWS/FLI-mediated transcriptional regulation and oncogenesis in Ewing sarcoma. This unprecedented role of GGAA microsatellite DNA in Ewing sarcoma provides a unique opportunity to expand our mechanistic understanding of how EWS/ETS fusions influence cancer susceptibility, prognosis and transcriptional regulation.
Nieto-Soler, Maria; Morgado-Palacin, Isabel; Lafarga, Vanesa
Ewing sarcomas (ES) are pediatric bone tumors that arise from a driver translocation, most frequently EWS/FLI1. Current ES treatment involves DNA damaging agents, yet the basis for the sensitivity to these therapies remains unknown. Oncogene-induced replication stress (RS) is a known source of en...
C. Parker Gibbs
Full Text Available Bone sarcomas are a clinically and molecularly heterogeneous group of malignancies characterized by varying degrees of mesenchymal differentiation. Despite advances in medical and surgical management, survival rates for high-grade tumors have remained static at 50% to 70%. Tumor stem cells have been recently implicated in the pathogenesis of other heterogeneous, highly malignant tumors. We demonstrate here the existence of a small subpopulation of self-renewing bone sarcoma cells that are capable of forming suspended spherical, clonal colonies, also called “sarcospheres,” in anchorage-independent, serum-starved conditions. These bone sarcoma cells as well as tissue specimens express activated STAT3 and the marker genes of pluripotent embryonic stem (ES cells, Oct 3/4 and Nanog. Expression levels of Oct 3/4 and Nanog are greater in sarcospheres than in adherent cultures. A subset of bone sarcoma cells displays several surface markers of mesenchymal stem cells (Stro-1, CD105, and CD44 as well as attributes of mesodermal, ectodermal, and endodermal differentiation. Although previously documented in brain and breast tumors, our results support the extension of the cancer stem cell hypothesis to include tumors of mesenchymal lineage. Furthermore, they suggest the participation of ES cell homeobox proteins in non-germ cell tumorigenesis.
de Bruijn, Diederik R. H.; Nap, Jan-Peter; van Kessel, Ad Geurts
Human synovial sarcomas are aggressive soft tissue tumors with relatively high rates of recurrences and metastases. They display a variable response to common treatment protocols such as radiation and chemotherapy. For the development of novel diagnostic, prognostic, and therapeutic approaches, deta
Bruijn, D.R.H. de; Nap, J.P.; Geurts van Kessel, A.H.M.
Human synovial sarcomas are aggressive soft tissue tumors with relatively high rates of recurrences and metastases. They display a variable response to common treatment protocols such as radiation and chemotherapy. For the development of novel diagnostic, prognostic, and therapeutic approaches, deta
França,S.A.; Serakides,R.; Silva,A.E.; Rachid,M.A.; J.R.C. Moraes; Lavalle,G.E.; Ocarino,N.M.
One four-year-old, female, Collie, dog presented subcutaneous enlarged mass at the lateral aspect of the left hindlimb, close to the knee joint. Based on the anatomopathological and immunohistochemical findings it was confirmed the diagnosis of extra-articular bifasic synovial sarcoma - grade II.
Komdeur, R; Hoekstra, HJ; van den Berg, E; Molenaar, WM; Pras, E; de Vries, EGE; van der Graaf, WTA
Soft tissue sarcomas (STSs) are rare tumors, notorious for early hematogenous metastasizing. Metastatic disease is seldom amenable to curative treatment; therefore new treatment modalities are required. Treatment-related and tumor-related prognostic factors can be assessed to estimate the risk for s
Balebail Gopalakrishna Dharmanand
Full Text Available Presence of sacroiliitis (SI is one of the hallmarks for diagnosing seronegative spondyloarthropathy, especially ankylosing spondylitis. In certain cases, the occurrence of erosions around sacroiliac joint due to other causes can mislead the diagnosis. We are discussing here a rare case of Ewing’s sarcoma, which clinically presented as SI.
Michelle C. Cora
Full Text Available A 1.5 year old, female, spayed, Labrador retriever with a history of three abdominal surgeries within the previous two months presented to the North Carolina State University Veterinary Teaching Hospital for evaluation of a pelvic inlet mass causing fecal tenesmus, obstipation, and dysuria. Abdominal ultrasound revealed a caudal abdominal mass extending into the pelvic cavity. Cytologic evaluation of the mass showed a pleomorphic round to fusiform cell population with histiocytic and suppurative inflammation. The primary differential was neoplasia, but inflammation with cellular pleomorphism could not be excluded. Via histopathology and immunohistochemistry, a diagnosis of poorly differentiated sarcoma originating from the uterus or cervix with widespread intra-abdominal dissemination and metastasis was made. Sarcomas of any type are rare in young dogs with only sporadic cases of poorly or undifferentiated sarcomas reported. This case is a unique presentation of an aggressive, poorly differentiated sarcoma arising from the cervix or uterus in a young dog and illustrates the importance of histologic evaluation of surgically resected tissues that are abnormal in appearance.
This case report deals with a 16-year-old patient wrongly diagnosed with odontogenic infection and abscess, and hospitalized. As the symptoms did not remit, biopsy was carried out and the patient was operated on with Ewing′s sarcoma diagnosis.
Full Text Available Extraskeletal Ewing's sarcoma (EES is a rare soft tissue tumor that is morphologically indistinguishable from skeletal ES. We report a case of a 25-year-old female with recurrent EES/primitive neuroectodermal tumor of right breast with imaging findings on mammogram, ultrasound, magnetic resonance imaging breast, and positron emission tomography–computed tomography.
Jørgensen, L J; Lyon, H; Myhre-Jensen, O;
Twenty-five synovial sarcomas were studied with a battery of antibodies directed against keratin and epithelial membrane antigen (EMA). The keratin antibody MNF 116 showed reactivity in 24 tumors. In addition, 22 tumors showed reactivity with the antibody Keratin Wide Spectrum, 20 with the antibody...
... Finding a Cure for Sarcoma Home About Us History People Public Filings News & Media SFA in the News SFA Videos SFA Press ... or - Get your current location Home About Us History People Public Filings News & Media SFA in the News SFA Videos SFA Press ...
Pommier, P; Sunyach, M-P; Hu, Yi; Amsalem, E; Claude, L; Moncort-Boulch, D; Toutenu, P; Balosso, J
The radiobiological properties of the hadrons (neutrons, protons, carbon ions) led to their therapeutic use in sarcomas, as a referent therapy or as an alternative to photon therapy. An extensive review of the literature has been conducted to assess the present indications and the perspectives for hadrontherapy. Compared to photons, neutrons are characterized by a higher biological efficiency that is on particular importance for these tumours usually considered as radio-resistant. Neutrons have been considered as a standard therapy for sarcoma' patients, contra-indicated for surgery or with a definitive R2 resection, but their indications and use have been restricted due to the occurrence of late severe toxicities related to their poor ballistic' properties. Thanks to their physical properties (Bragg Peak), protons are characterized by a higher conformity index compared to photons (and neutrons) with optimal organs at risk preservation that permits a dose escalation. Protontherapy is to date the standard of care for base of skull, spinal and paraspinal sarcomas. Carbon ions combined both advantages from protons and neutrons. Literature data permits to consider this radiation modality as a referent therapy for unresectable sarcomas. The ongoing diffusions of protons and carbon ions radiotherapy facilities will permit to offer these therapies to more patients and to conduct studies that are warranted to determine their indications and their results.
Full Text Available Soft-tissue reconstruction following preoperative radiotherapy and wide resection of soft tissue sarcoma remains a challenge. Pedicled and free tissue transfers are an essential part of limb sparing surgery. We report 22 cases of sarcoma treated with radiotherapy and wide excision followed by one-stage innervated free or pedicled musculocutaneous flap transfers. The resection involved the upper limb in 3 cases, the lower limb in 17, and the abdominal wall in 2. The flaps used for the reconstruction were mainly latissimus dorsi and gracilis. The range of motion was restored fully in 14 patients. The muscle strength of the compartment reconstructed was of grades 4 and 5 in all patients except one. The overall function was excellent in all the cases with functional scores of 71.2% in the upper limb and 84% in the lower limb. The only 2 major complications were flap necrosis, both revised with another flap, one of which was innervated with restoration of function. Innervated flaps are valuable alternatives for reconstruction after sarcoma resection in the extremity and in the abdominal wall. The excellent functional results are encouraging, and we believe that innervated muscle reconstruction should be encouraged in the treatment of sarcoma after radiotherapy and wide resection.
Wong, FL; Boice, JD; Abramson, DH; Tarone, RE; Kleinerman, RA; Stovall, M; Goldman, MB; Seddon, JM; Tarbell, N; Fraumeni, JF; Li, FP
Context.-There is a substantial risk of a second cancer for persons with hereditary retinoblastoma, which is enhanced by radiotherapy. Objective.-To examine long-term risk of new primary cancers in survivors of childhood retinoblastoma and quantify the role of radiotherapy in sarcoma development. De
Ent, Wietske van der
Ewing sarcoma (EWS) is a disease with a high need for novel therapeutic strategies. To aid in investigating such compounds in an in vivo setting, we have developed several zebrafish model systems for EWS, which are presented in this thesis. The first is a manual xenograft model in 2-day-old
Full Text Available Abstract Background Sarcomas are rare tumors (1-2% of all cancers of mesenchymal origin that may develop in soft tissues and viscera. Since the International Classification of Disease (ICD attributes visceral sarcomas (VS to the organ of origin, the incidence of sarcoma is grossly underestimated. The rarity of the disease and the variety of histological types (more than 70 or locations account for the difficulty in acquiring sufficient personal experience. In view of the above the European Commission funded the project called Connective Tissues Cancers Network (CONTICANET, to improve the prognosis of sarcoma patients by increasing the level of standardization of diagnostic and therapeutic procedures through a multicentre collaboration. Methods/Design Two protocols of epidemiological researches are here presented. The first investigation aims to build the population-based incidence of sarcoma in a two-year period, using the new 2002 WHO classification and the "second opinion" given by an expert regional pathologist on the initial diagnosis by a local pathologist. A three to five year survival rate will also be determined. Pathology reports and clinical records will be the sources of information. The second study aims to compare the effects on survival or relapse-free period - allowing for histological subtypes, clinical stage, primary site, age and gender - when the disease was treated or not according to the clinical practice guidelines (CPGs. Discussion Within CONTICANET, each group was asked to design a particular study on a specific objective, the partners of the network being free to accept or not the proposed protocol. The first protocol was accepted by the other researchers, therefore the incidence of sarcoma will be assessed in three European regions, Rhone-Alpes and Aquitaine (France and Veneto (Italy, where the geographic distribution of sarcoma will be compared after taking into account age and gender. The conformity of the clinical
Bagán, José; Poveda-Roda, Rafael
Objectives A review is made of the clinical, diagnostic, therapeutic and survival characteristics of Ewing sarcoma (ES) of the oral cavity. Material and Methods A systematic literature search was carried out, with restrictions referred to time (1960-2014), language (English and Spanish) and type of study (case reports, letters, datasets, reviews). The following MeSH terms and boolean operators were used: Ewing AND Sarcoma AND [tongue, jaw, maxilla, cheek, condyle OR temporomandibular, floor AND mouth, gum OR gingiva, palate OR palatal, lip, uvula, head AND neck]. Results Seventy-one cases of ES of the oral cavity were documented from 53 articles. The main differences versus ES of other locations were a younger age at manifestation, a shorter time from symptoms onset to diagnosis, and swelling as the most frequent clinical manifestation versus swelling and pain in the rest of disease locations. The way in which ES manifests in the oral cavity is varied and comprises dental displacement (19.7%), dental mobility (7%), root reabsorption (5.6%), destruction of the dental follicle (4.2%), premature exfoliation (4.2%) and paresthesia of the chin (2.8%). Metastatic neck adenopathies appear in 11.3% of the cases. Significant differences in survival are observed between patients with a complete diagnosis of ES (hematoxylin-eosin staining, PAS positivity, CD99 positivity) and those with an incomplete diagnosis. Conclusions Ewing sarcoma of the oral cavity presents a series of specific features that distinguish it from ES of other locations. Key words:Primitive neuroectodermal tumor, PNET, Ewing sarcoma, Ewing tumor, sarcoma, oral cavity. PMID:28210452
Full Text Available One four-year-old, female, Collie, dog presented subcutaneous enlarged mass at the lateral aspect of the left hindlimb, close to the knee joint. Based on the anatomopathological and immunohistochemical findings it was confirmed the diagnosis of extra-articular bifasic synovial sarcoma - grade II.
Quevedo Moreno, P.; Hernandez Moreno, L.; Perez Diaz, M.; Lafuente Martinez, J.L.; Arozamena Laso, M.
Extraosseous Ewing's sarcoma (EES) is an uncommon lesion included in the group of soft tissue tumors. We present a case in a 19-year-old woman in which the diagnosis was not initially suspected because of the absence of clinical and radiological evidence. (Author)
He, Rui; Patel, Rajiv M; Alkan, Serhan; Hammadeh, Rasheed; Weiss, Sharon W; Goldblum, John R; Venkataraman, Girish; Baila, Horea
Synovial sarcoma in its classic biphasic form can be distinguished readily from other soft tissue lesions; however, monophasic and poorly differentiated forms are diagnostically more problematic. For this reason, we assessed the efficacy of immunostaining for SYT and SSX1 proteins, the gene products resulting from unique synovial sarcoma translocation, to distinguish synovial sarcoma from other soft tissue lesions. A total number of 146 cases were analyzed, including 47 synovial sarcoma cases (all of which were verified by FISH to have t(X; 18) translocation and SYT-SSX fusion gene) and 99 soft tissue tumors of various types. A polyclonal IgG antibody against SYT was used to stain formalin-fixed paraffin embedded tissues. Forty-one out of 47 (87%) synovial sarcoma displayed strong positive nuclear staining (ranging from 80 to 90% of the tumor cells) for SYT antibody. Nineteen of 99 (19%) non-synovial sarcoma cases showed variable nuclear and cytoplasmic staining with SYT, which ranged from 20 to 60% of tumor nuclei, and included malignant peripheral nerve sheath tumor (5/25), solitary fibrous tumor (2/14), Ewing sarcoma (2/6), low grade fibromyxoid tumor (2/4), extraskeletal mesenchymal chondrosarcoma (2/6), gastrointestinal tumor (4/17), epithelioid sarcoma (2/2). The remaining non-synovial sarcomas were negative. This is the first study demonstrating SYT protein expression in tissue sections of synovial sarcoma. This method could provide an easy, rapid and widely applicable means of assisting in the diagnosis of synovial sarcoma, particularly when material and/or resources are unavailable for PCR or FISH-based testing. However, as variable weak staining for SYT may be encountered in a small percentage of non-synovial sarcoma sarcomas, a positive interpretation should be made only when the staining is strong, nuclear and present in the majority of cells.
Molenaar, W.M.; Muntinghe, F.L.H.
In a previous study, it was shown that rhabdomyosarcomas widely express "neural" markers, such as neural cell adhesion molecules (N-CAM) and neurofilament protein isoforms, In the current study, a series of Ewing's sarcomas of bone and soft tissue sarcomas other than rhabdomyosarcoma was probed for
Galinier, P; Scheiner, C; Bardot, J; Vasse, D; Rimareix, F; Faissal, T; Magalon, G
We describe two cases of giant-cell fibroblastoma (GCF) with dermato fibro sarcoma protuberans (DFSP) component, occurring in two children in a chest wall localization. One case recurred 1 year later. The two patients were tumor-free 12 and 8 years later. GCF is a rare mesenchymal cutaneous and subcutaneous tumor reported mostly in the first two decades of life. Dermato fibro sarcoma protuberans, occurring preferentially in adults, is a rare skin tumor with a pronounced tendency to local recurrence. Some cases of association of recurrence of GFC under the form of DFSP have been reported, raising the question of a continuum between the two tumors. The treatment of choice of the two tumors is a wide local excision.
Morena, Deborah; Maestro, Nicola; Bersani, Francesca; Forni, Paolo Emanuele; Lingua, Marcello Francesco; Foglizzo, Valentina; Šćepanović, Petar; Miretti, Silvia; Morotti, Alessandro; Shern, Jack F; Khan, Javed; Ala, Ugo; Provero, Paolo; Sala, Valentina; Crepaldi, Tiziana; Gasparini, Patrizia; Casanova, Michela; Ferrari, Andrea; Sozzi, Gabriella; Chiarle, Roberto; Ponzetto, Carola; Taulli, Riccardo
Embryonal Rhabdomyosarcoma (ERMS) and Undifferentiated Pleomorphic Sarcoma (UPS) are distinct sarcoma subtypes. Here we investigate the relevance of the satellite cell (SC) niche in sarcoma development by using Hepatocyte Growth Factor (HGF) to perturb the niche microenvironment. In a Pax7 wild type background, HGF stimulation mainly causes ERMS that originate from satellite cells following a process of multistep progression. Conversely, in a Pax7 null genotype ERMS incidence drops, while UPS becomes the most frequent subtype. Murine EfRMS display genetic heterogeneity similar to their human counterpart. Altogether, our data demonstrate that selective perturbation of the SC niche results in distinct sarcoma subtypes in a Pax7 lineage-dependent manner, and define a critical role for the Met axis in sarcoma initiation. Finally, our results provide a rationale for the use of combination therapy, tailored on specific amplifications and activated signaling pathways, to minimize resistance emerging from sarcomas heterogeneity.
Bariani, Maria Carolina Prado Fleury; Fleury Júnior, Luiz Fernando Fróes; Ribeiro, Ana Maria Quinteiro; Carneiro, Siderley de Souza; Pereira, Tiago Arantes
The association of mycosis fungoides and kaposi’s sarcoma in HIV-negative patients is a rare phenomenon. The presence of human herpesvirus 8 (HHV-8) – associated with all forms of Kaposi’s sarcoma – has also been recently identified in mycosis fungoides lesions. However, a causal association between HHV-8 and the onset of mycosis fungoides has not been established yet. The present case reports a patient who developed Kaposi’s sarcoma lesions after a two-year UVB phototherapy to treat a mycosis fungoides. Negative immunohistochemistry staining for Kaposi’s sarcoma-associated herpesvirus in the initial mycosis fungoides lesions strengthens the absence of a link between Kaposi’s sarcoma-associated herpesvirus and mycosis fungoides. Immunosuppression caused by the lymphoma and prolonged phototherapy were probably the contribut ing factors for the onset of Kaposi’s sarcoma.
Aïm, F; Rosier, L; Dumontier, C
A 63-year-old woman with long-standing AIDS and previous Kaposi sarcomas of the lower limb presented to our consultation complaining of a painful left ring finger with pulp enlargement. X-rays revealed an osteolytic lesion of the distal phalanx. We suspected an isolated osseous Kaposi sarcoma and at surgery we found a hemorrhagic lesion with bone extension into the phalanx. Bone involvement is rare in Kaposi sarcoma and even rarer in patients without a cutaneous location.
Jamshidi, Khodamorad; Yahyazadeh, Hooman; Bagherifard, Abolfazl
Periarticular cyst and cystic soft tissue lesion around the knee are common. Synovial sarcoma is a rare and malignant soft tissue tumor accounting for approximately 5% of soft tissue sarcoma. A case is presented where a lesion adjacent to the joint line of the knee was diagnosed clinically and on imaging as a meniscal cyst. MRI signal was homogenous and no concomitant meniscal tears were seen. The tissue diagnosis was monophasic synovial sarcoma.
Full Text Available Periarticular cyst and cystic soft tissue lesion around the knee are common. Synovial sarcoma is a rare and malignant soft tissue tumor accounting for approximately 5% of soft tissue sarcoma. A case is presented where a lesion adjacent to the joint line of the knee was diagnosed clinically and on imaging as a meniscal cyst. MRI signal was homogenous and no concomitant meniscal tears were seen. The tissue diagnosis was monophasic synovial sarcoma.
Full Text Available Sarcoma-like mural nodule is a very rare occurrence in a mucinous tumour of the ovary. Two such nodules having morphologically benign features with osteoclastic giant cells, in the wall of mucinous cystadenoma are described. In addition, these nodules exhibited reactive vascular proliferations. The sarcoma-like nodules are associated with a favourable outcome and must be distinguished from other malignant nodules composed of sarcoma or anaplastic carcinoma that may also occur in the wall of mucinous ovarian tumours.
Ryan Alexander, DO; Magda Rizer, DO; William Burke, MD; Lawrence Ciment, MD
Kaposi sarcoma (KS) is a low-grade mesenchymal tumor involving blood and lymphatic vessels. There are four types, based on clinical presentation: classic, endemic (Africana), iatrogenic (typically, involving renal allograft recipients), and AIDS-associated (epidemic). Kaposi's sarcoma-associated herpes virus infection has been linked along with other factors to the development of KS. The Kaposi's sarcoma-associated herpes virus interacts and encodes for numerous molecular proteins that play a...
Al-Brahim, Nabeel; Zaki, Ashraf H; El-Merhi, Khaled; Ahmad, Mahmoud S
Kaposi sarcoma is a malignant vascular neoplasm uncommonly seen in immunosuppressed patients. Herein we report an unusual case of tonsillar Kaposi sarcoma in a patient with membranous glomerulonephritis treated with prednisolone and cyclosporine. The patient presented after 10 months of starting the treatment with a tonsillar mass. Histological examination was typical of monomorphic spindle cell proliferation with slit-like vascular channels. The tumor cells expressed CD34, D2-40 and positive nuclear stain for HHV-8. Kaposi sarcoma is associated with immunosuppression and rarely occurs in the tonsil. Clinicians should be aware of this rare presentation of Kaposi sarcoma.
Full Text Available Geoffrey W Siegel,1 J Sybil Biermann,1 Rashmi Chugh,2 Jon A Jacobson,3 David Lucas,4 Mary Feng,5 Andrew C Chang,6 Sean R Smith,7 Sandra Wong,6 Jill L Hasen1 1Department of Orthopedics, 2Department of Medical Oncology, 3Department of Radiology, 4Department of Pathology, 5Department of Radiation, 6Department of Surgery, 7Department of Physical Medicine and Rehabilitation, University of Michigan, Ann Arbor, MI, USA Abstract: The rarity of bone and soft tissue sarcoma, the difficulty in interpretation of imaging and histology, the plethora of treatment modalities, and the complexity and intensity of the treatment contribute to the need for systematic multidisciplinary team management of patients with these diseases. An integrated multidisciplinary clinic and team with a structured sarcoma tumor board facilitate team coordination and communication. This paper reviews the rationale for multidisciplinary management of sarcoma and details the operational structure of the Multidisciplinary Sarcoma Clinic and Sarcoma Tumor Board. The structured Multidisciplinary Sarcoma Tumor Board provides opportunity for improvement in logistics, teaching, quality, and enrollment in clinical trials. Keywords: sarcoma, sarcoma care, sarcoma tumor board, collaborative approach
Erban, S B; Sokas, R K
The association of Kaposi's sarcoma with malignant lymphoreticular diseases and immunosuppressive therapy is well documented. This report describes an elderly man who presented with fulminant Wegener's granulomatosis that responded to treatment with cyclophosphamide and corticosteroids. Rapidly progressing cutaneous Kaposi's sarcoma developed ten weeks after the start of immunosuppressive therapy yet regressed on discontinuation of the corticosteroid therapy, despite continuation of cyclophosphamide therapy. To our knowledge, this is the first reported case of Kaposi's sarcoma occurring in association with Wegener's granulomatosis. The literature on Kaposi's sarcoma in immunosuppressed patients is reviewed.
Full Text Available Synovial sarcoma comprises approximately 10% of all soft tissue sarcoma diagnoses; a primary synovial sarcoma of the myocardium is exceedingly rare. There have been very few cases reported in the literature thus far. With the identification of the characteristic and diagnostic chromosomal abnormality t(X;18, this may become an increasingly recognized entity. Our report adds to the limited published cases of primary cardiac synovial sarcoma with the characteristic t(X;18. Further elucidation of the effects of this translocation on the cell cycle may lead to directed therapies in the future.
Kathleen I. Pishas
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.
Tao KANG; Feng-chun Ye; Shou-jiang gao; Lin-ding WANG
Tumor angiogenesis is the uncontrolled growth of blood vessels in tumors,serving to supply nutrients and oxygen,and remove metabolic wastes.Kaposi's sarcoma (KS),a multifocal angioproliferative disorder characterized by spindle cell proliferation,neo-angiogenesis,inflammation,and edema,is associated with infection by Kaposi's sarcoma-associated herpesvirus (KSHV).Recent studies indicate that KSHV infection directly promotes angiogenesis and inflammation through an autocrine and paracrine mechanism by inducing pro-angiogenic and pro-inflammatory cytokines.Many of these cytokines are also expressed in KS lesions,implicating a direct role of KSI-IV in the pathogenesis of this malignancy.Several KSHV genes are involved in KSHV-induced angiogenesis.These studies have provided insights into the pathogenesis of KS,and identified potential therapeutic targets for this malignancy.
Schulz, Thomas F; Cesarman, Ethel
Kaposi Sarcoma-associated Herpesvirus (KSHV, HHV8) causes three human malignancies, Kaposi Sarcoma (KS), an endothelial tumor, as well as Primary Effusion Lymphoma (PEL) and the plasma cell variant of Multicentric Castleman's Disease (MCD), two B-cell lymphoproliferative diseases. All three cancers occur primarily in the context of immune deficiency and/or HIV infection, but their pathogenesis differs. KS most likely results from the combined effects of an endotheliotropic virus with angiogenic properties and inflammatory stimuli and thus represents an interesting example of a cancer that arises in an inflammatory context. Viral and cellular angiogenic and inflammatory factors also play an important role in the pathogenesis of MCD. In contrast, PEL represents an autonomously growing malignancy that is, however, still dependent on the continuous presence of KSHV and the action of several KSHV proteins.
Jørgensen, L J; Lyon, H; Myhre-Jensen, O
Twenty-five synovial sarcomas were studied with a battery of antibodies directed against keratin and epithelial membrane antigen (EMA). The keratin antibody MNF 116 showed reactivity in 24 tumors. In addition, 22 tumors showed reactivity with the antibody Keratin Wide Spectrum, 20 with the antibody...... Keratin 56, 64, and 19 with CAM 5.2. Seventeen tumors showed reactivity with EMA. The keratin and EMA reactivity was present in cells lining obvious cleft-like structures in biphasic tumors. In the spindle cell areas of both biphasic and monophasic fibrous tumors, we found clusters of a few reacting cells...... apparently located around small clefts. In the synovioblastic tumors, clusters of plump tumor cells reactive for both the keratins and EMA were present. In conclusion, we found that proper identification of epithelial differentiation in synovial sarcomas is facilitated by an immunohistochemical application...
Full Text Available The life cycle of Kaposi's sarcoma-associated herpesvirus (KSHV consists of latent and lytic replication phases. During latent infection, only a limited number of KSHV genes are expressed. However, this phase of replication is essential for persistent infection, evasion of host immune response, and induction of KSHV-related malignancies. KSHV reactivation from latency produces a wide range of viral products and infectious virions. The resulting de novo infection and viral lytic products modulate diverse cellular pathways and stromal microenvironment, which promote the development of Kaposi's sarcoma (KS. The mechanisms controlling KSHV latency and reactivation are complex, involving both viral and host factors, and are modulated by diverse environmental factors. Here, we review the cellular and molecular basis of KSHV latency and reactivation with a focus on the most recent advancements in the field.
Kim, Bum-Soo; Joo, Sun-Hyung; Kim, Hyun-Cheol
Laparoscopic cholecystectomy has become a standard treatment of symptomatic gallstone disease. Although spilled gallstones are considered harmless, unretrieved gallstones can result in intra-abdominal abscess. We report a case of abscess formation due to spilled gallstones after laparoscopic cholecystectomy mimicking a retroperitoneal sarcoma on radiologic imaging. A 59-year-old male with a surgical history of a laparoscopic cholecystectomy complicated by gallstones spillage presented with a 1 mo history of constant right-sided abdominal pain and tenderness. Computed tomography and magnetic resonance imaging demonstrated a retroperitoneal sarcoma at the sub-hepatic space. On open exploration a 5 cm × 5 cm retroperitoneal mass was excised. The mass contained purulent material and gallstones. Final pathology revealed abscess formation and foreign body granuloma. Vigilance concerning the possibility of lost gallstones during laparoscopic cholecystectomy is important. If possible, every spilled gallstone during surgery should be retrieved to prevent this rare complication.
Full Text Available Background: Myeloid sarcoma (MS or granulocytic sarcoma is a rare tumor consisting of myeloid blasts with or without maturation occurring at anatomic sites other than the bone marrow. MS can involve any organ system but shows a predilection for skin, bone, and soft tissues of head and neck region.Case report: We report four cases of MS occurring at unusual sites, out of which three were de novo and one was associated with acute myeloid leukemia (AML.Conclusion: Although MS is associated with AML, it can rarely present without any existent hematologic disease. Differential diagnosis of a soft tissue mass should include MS even in the absence of leukemia. Establishment of the correct diagnosis depends on morphologic, histochemical, and immunohistochemical examination.
P. Y. Kuo
Full Text Available The prevalence of pain in patients with sarcoma is not well documented. We investigated this in outpatients at a tertiary cancer referral centre, assessing the adequacy of pain control and for risk factors leading to higher prevalence and severity of pain. 149 patients were surveyed. Patients with pain within the previous 7 days completed pain assessment tools (BPI, S-LANSS, PMI. 53% of patients had pain within the previous 7 days, and 25% had significant pain. Of those with pain, 63% was inadequately controlled and neuropathic pain was identified in 36%. Age, gender, tumour type, and the type of cancer treatment were not significant predictors of the prevalence or severity of the pain. Based on our results, patients with sarcoma should be actively screened for pain and have regular reviews of their analgesic requirements.
Trevisan, Flávia; Xavier, Celia Antonia; Pinto, Clovis Antonio Lopes; Cattete, Fernanda Gomes; Stock, Fabíola Schauffler; Martins, Marcella Ledo
Histiocytic sarcoma is a rare hematologic malignant neoplasia originating from histiocytic or dendritic cell clones. The lesions may be in nodal or extranodal sites, most commonly in the gastrointestinal tract. A small number of cases presents as unique cutaneous lesions. The definitive diagnosis is made by positivity for the immunohistochemical markers CD163, CD68, CD4 and lysozyme. The treatment is controversial, often with combined systemic chemotherapy. This is a case of cutaneous histiocytic sarcoma in an 82-year-old patient presenting two nodular lesions in the breast and right arm which were treated with simple excision and multidisciplinary follow-up, avoiding aggressive management and exhaustive investigations. Although most studies report aggressive evolution, the patient had good and stable clinical status during the twelve-month follow-up period.
Trevisan, Flávia; Xavier, Celia Antonia; Pinto, Clovis Antonio Lopes; Cattete, Fernanda Gomes; Stock, Fabíola Schauffler; Martins, Marcella Ledo
Histiocytic sarcoma is a rare hematologic malignant neoplasia originating from histiocytic or dendritic cell clones. The lesions may be in nodal or extranodal sites, most commonly in the gastrointestinal tract. A small number of cases presents as unique cutaneous lesions. The definitive diagnosis is made by positivity for the immunohistochemical markers CD163, CD68, CD4 and lysozyme. The treatment is controversial, often with combined systemic chemotherapy. This is a case of cutaneous histiocytic sarcoma in an 82-year-old patient presenting two nodular lesions in the breast and right arm which were treated with simple excision and multidisciplinary follow-up, avoiding aggressive management and exhaustive investigations. Although most studies report aggressive evolution, the patient had good and stable clinical status during the twelve-month follow-up period. PMID:24173190
Grossniklaus, Hans E; Shehata, Bahig; Sorensen, Poul; Bergstrom, Chris; Hubbard, G Baker
An 11-year-old boy underwent enucleation of his left eye for an intraocular tumor. Examination showed a small, round blue cell tumor arising in the peripheral retina near the ciliary body. Immunohistochemical stain results were positive for neuron-specific enolase, synaptophysin, cluster of differentiation 99 (CD99), Friend leukemia integration 1, and CD56. Ultrastructural findings included occasional intracytoplasmic dense core granules. Polymerase chain reaction of the tumor showed a Ewing sarcoma/Friend leukemia integration gene fusion product. The tumor was classified as a primitive neuroectodermal tumor/Ewing sarcoma of the retina and should be distinguished from retinoblastoma. To our knowledge, this is the first case of primary primitive neuroectodermal tumor of the retina.
Full Text Available Clear cell sarcoma of the kidney is a rare malignant neoplasm of childhood, known for its aggressiveness, its tendency for recurrence, and to metastasize to bone. We report the observation of 8-month-old child presenting with a large abdominal mass. Clinically, it was diagnosed as Wilm′s tumor, and left nephrectomy was done. Grossly, 10 cm × 8 cm × 3.5 cm globular, white, encapsulated, smooth mass uniformly involving the whole kidney was noted. Histologically, the tumor was diagnosed as clear cell sarcoma with renal vein showing presence of tumor embolus in lumen. The tumor was given stage-II (NWTS-5 protocol. Immunohistochemistry showed vimentin positive and cytokeratin negative tumor cells. The child is currently undergoing chemotherapy and has not yet shown any sign of bony metastasis.
Schack, Line H; Mouritsen, Lene S; Elowsson, Charlotte;
centers in Denmark. Known prognostic factors such as age, gender and performance status, the histopathology as well as other new factors such as response to previous chemotherapy and hyponatremia were tested. RESULTS: Median age was 59 years. Lipo- and leiomysosarcomas (L-sarcomas) represented 43......% of the cases and 18% had hyponatremia before the start of trabectedin. The median number of previous lines of chemotherapy was two (range 0-6) and the median number of chemotherapy cycles given before trabectedin was nine (range 0-85). The median number of trabectedin cycles was three (range 1-17). The median...... OS for the whole cohort was seven months. Poor performance status, non-L-sarcomas, and hyponatremia were statistically significant adverse prognostic factors with median survival of: 4, 5, and 2 months compared to 9, 12 and 13 months, respectively. Moreover, having achieved clinical benefit [complete...
Mitchell, B D; Fox, B D; Viswanathan, A; Mitchell, A H; Powell, S Z; Cech, D A
We describe the first patient with an extradural, extramedullary Ewing's sarcoma tumor mimicking a nerve sheath tumor with no overt evidence of metastasis. A 28-year-old woman with no past medical history presented with a progressive 3-year history of low back pain and right-sided lower extremity radiculopathy after having failed conservative therapies. MRI of the lumbar spine revealed a right-sided enhancing, dumbbell-shaped lesion at the right neural foramen appearing to originate from the L4 nerve root, suspicious for a peripheral nerve sheath tumor or schwannoma. The patient and findings are discussed in the context of the literature, including an update on the relatively recent diagnostic redesignation of the Ewing's sarcoma family tumors.
So, Hoonsub; Kim, Sun A; Yoon, Dok Hyun; Khang, Shin Kwang; Hwang, Jihye; Suh, Chong Hyun; Suh, Cheolwon
Histiocytic sarcoma is a type of lymphoma that rarely involves the central nervous system (CNS). Its rarity can easily lead to a misdiagnosis. We describe a patient with primary CNS histocytic sarcoma involving the cerebral hemisphere and spinal cord, who had been initially misdiagnosed as demyelinating disease. Two biopsies were necessary before a correct diagnosis was made. A histologic examination showed bizarre shaped histiocytes with larger nuclei and nuclear atypia. The cells were positive for CD68, CD163, and S-100 protein. As a resection was not feasible due to multifocality, he was treated with highdose methotrexate, but showed no response. As a result, he was switched to high dose cytarabine; but again, showed no response. The patient died 2 months from the start of chemotherapy and 8 months from the onset of symptoms. Since few patients with this condition have been described and histopathology is difficult to diagnose, suspicion of the disease is essential. PMID:25345462
Full Text Available Alveolar soft part sarcoma (ASPS is a rare soft-tissue sarcoma, commonly occurring in children and adolescents. The tumor mostly involves the lower extremities. The prognosis of the patient depends on whether there is metastasis. We present a 19-year-old female with ASPS in her right lower leg. Grayscale and color Doppler ultrasound showed a well-defined hypoechoic lesion with hypervascularity and very low resistive index (RI. Magnetic resonance imaging revealed iso signal intensity to muscle on T1-weighted images, high signal intensity to muscle on T2-weighted images with signal voids, and good enhancement after gadolinium administration. In a mass with hypervascularity and very low RI on sonography and hypervascularity with flow voids on magnetic resonance imaging, ASPS should be considered.
Lin, Guoshu; Wang, Hongyan; Fan, Xing; Li, Hui; Wang, Zaixing; Lin, Da; Yang, Sen; Zhang, Xuejun
Kaposi sarcoma (KS) is a neoplasm of the endothelial cells. It often manifests with multiple vascular nodules on the skin and other organs. It is a systemic, malignant and multifactor disease and has a variable course. We describe an elderly Chinese man who had a rapidly growing maroon nodule on his right foot, both arms and cheekbones. KS in HIV-negative patients has only been reported sporadically.
李明仁; 王惠丰; 陈萍
Full Text Available Patients with HIV are prone to a number of unusual infectious and malignant conditions, typically resulting from declining immune function. However, there are also a few of reports of HIV associated conditions potentially created by viral release of interleukin-6 (IL-6. Herein, we present a case of HIV IL-6-related systemic inflammatory syndrome, a Kaposi sarcoma (KS-associated syndrome in the absence of multicentric Castleman disease (MCD.
Tellez-Gabriel, M.; Brown, H K; Young, R.; Heymann, M. F.; Heymann, D
International audience; Sarcomas are a heterogenous group of malignant neoplasms of mesenchymal origin, many of which have a propensity to develop distant metastases. Cancer cells that have escaped from the primary tumour are able to invade into surrounding tissues, to intravasate into the bloodstream to become Circulating Tumour Cells (CTCs), and are responsible for the generation of distant metastases. Due to the rarity of these tumours and the absence of specific markers expressed by sarco...
Full Text Available A 20-year-old female presented to the Pulmonary Medicine Department with complaints of fever, left sided chest pain and progressive dyspnoea of four months duration. Radiological examination revealed a mass lesion with massive pleural effusion and rib erosion. Histopathology showed neoplastic cells with scanty cytoplasm, hyperchromatic nuclei and rosette formation suggestive of Ewing sarcoma. The rarity of this tumor and its unusual presentation prompted this report.
Travaglini, Grazia; Biagetti, Simona; Alfonsi, Simona; Bearzi, Italo; Marmorale, Cristina
Il caso clinico che vi presentiamo riguarda la straordinaria localizzazione intra-addominale di un sinovial-sarcoma. Questo tumore dei tessuti molli generalmente interessa le piccole e grandi articolazioni. Sono state descritte altre localizzazioni a livello intra-addominale e retroperitoneale. La nostra paziente si ricovera per una tumefazione dolente dell’addome, alla TC addome si evidenzia una voluminosa massa intra-addominale che comprime e disloca i parenchimi e visceri circostanti e che affiora ai tessuti più superficiali della parete addominale. La biopsia eco-guidata della lesione, insieme allo studio immunoistochimico, permette di porre la diagnosi di sinovial-sarcoma. La paziente viene candidata ad un trattamento chemioterapico a scopo neoadiuvante. Per il sopraggiungere di anemizzazione da sanguinamento della neoplasia, valutata anche la risposta parziale al trattamento sistemico, si pone indicazione all’intervento chirurgico. La massa con peduncolo vascolare maggiore costituito dai vasi gastroepiploici di destra, viene completamente asportata. L’esame definitivo conferma la diagnosi di Sinovial-Sarcoma bifasico. L’indagine genetica condotta sul campione conservato in formalina non ha permesso, a causa della difficoltà di estrarre ed amplificare un quantitativo adeguato di RNA, di identificare la traslocazione (X; 18) (p 11.2; q 11.2) specifica del Sinovial-Sarcoma. La paziente è stata dimessa dopo un decorso clinico regolare. Il follow-up ad un anno non ha mostrato ripresa di malattia ma dopo 18 mesi alla TC torace-addome di rivalutazione si evidenzia la ripresa di malattia a livello intra-addominale e la presenza di metastasi polmonari bilaterali.
Almefty, Rami O; Tyree, Tammy L; Fusco, David J; Coons, Stephen W; Nakaji, Peter
Histiocytic sarcoma is a rare malignancy with only 10 reports confirmed primarily involving the CNS. The diagnosis is dependent on the finding of malignant cells with histiocytic morphology and immunophenotype. The authors report a case of pathologically proven HS of the CNS. A 16-year-old boy presented with headaches, emesis, and altered sensorium. Noncontrast head CT scanning demonstrated a left parietal mass consistent with a tumor. Surgery was undertaken. Intraoperative findings revealed green-yellow exudates consistent with an abscess. Cultures were obtained and broad-spectrum antibiotics were started. The patient subsequently underwent multiple surgical procedures, including drainage and debulking of abscesses and hemicraniectomy. Two months after initial presentation, the patient's diagnosis of histiocytic sarcoma was confirmed. Pathological examination demonstrated necrotizing inflammation with preponderant neutrophil infiltration, variably atypical mononuclear and multinucleate histiocytes, and numerous mitoses. Additional immunohistochemistry studies confirmed immunoreactivity for CD68, CD45, CD45RO, and CD15 and were negative for CD3, CD20, melanoma cocktail, CD30, CD1a, CD34, HMB-45, and melan-A. Once the diagnosis of histiocytic sarcoma was confirmed, antibiotics were stopped and radiation therapy was undertaken. Despite treatment, the patient's neurological status continued to decline and the patient died 126 days after initial presentation. This case represents a rare confirmed example of CNS histiocytic sarcoma. A profound inflammatory infiltrate seen on pathology and green exudates seen intraoperatively make the condition difficult to distinguish from an abscess. Immunohistochemistry showing a histiocytic origin and negative for myeloid, dendritic, or other lymphoid markers is essential for the diagnosis. Further research is needed to establish consensus on treatment.
Gandolfo, N. [Ist. di Radiologia, Univ. di Genova (Italy); Gandolfo, N.G.; Serafini, G. [Servizio di Radiologia, Pietra Ligure (Italy); Martinoli, C. [Servizio di Radiologia, Azienda Ospedale S. Martino e Cliniche Universitarie Convenzionate, Genova (Italy)
We describe the MRI and US features of two patients with endometrial stromal sarcoma of the uterus. Both lesions appeared as voluminous polypoid masses within an expanded endometrial cavity on both US and MRI. They had mixed echotexture and heterogenous signal intensity on both T1- and T2-weighted sequences. T2-weighted images were most helpful in detecting the endometrial nature of the disease and its relationships with surrounding myometrium. (orig.)
Belletti, B; Nicoloso, M S; Schiappacassi, M; Berton, S; Lovat, F.; Wolf, K.; Canzonieri, V; D'Andrea, S.; Zucchetto, A; Friedl, P.H.A.; Colombatti, A; Baldassarre, G.
The balanced activity of microtubule-stabilizing and -destabilizing proteins determines the extent of microtubule dynamics, which is implicated in many cellular processes, including adhesion, migration, and morphology. Among the destabilizing proteins, stathmin is overexpressed in different human malignancies and has been recently linked to the regulation of cell motility. The observation that stathmin was overexpressed in human recurrent and metastatic sarcomas prompted us to investigate sta...
Full Text Available A forty year old male patient presented with swollen, deformed right hand with multiple irregular ulcers. The axillary lymph nodes were enlarged, firm and tender. The biopsy of nodules present on the hand revealed areas of haemorrhage and necrosis. The histopathological examination confirmed the clinical diagnosis of epithelioid sarcoma. Following below-elbow amputation, patient failed to regain consciousness and expired. On autopsy, distant metastases were found in the regional lymph nodes, pleura, kidney and cerebrum.
Full Text Available Alexandre Christinat, Serge LeyvrazCentre Pluridisciplinaire d’Oncologie, University Hospital, Lausanne, SwitzerlandAbstract: Interest in marine natural products has allowed the discovery of new drugs and trabectedin (ET-743, Yondelis, derived from the marine tunicate Ecteinascidia turbinata, was approved for clinical use in 2007. It binds to the DNA minor groove leading to interferences with the intracellular transcription pathways and DNA-repair proteins. In vitro antitumor activity was demonstrated against various cancer cell lines and soft tissue sarcoma cell lines. In phase I studies tumor responses were observed also in osteosarcomas and different soft tissue sarcoma subtypes. The most common toxicities were myelosuppression and transient elevation of liver function tests, which could be reduced by dexamethasone premedication. The efficacy of trabectedin was established in three phase II studies where it was administered at 1.5 mg/m2 as a 24 h intravenous infusion repeated every three weeks, in previously treated patients. The objective response rate was 3.7%–8.3% and the tumor control rate (which included complete response, partial response and stable disease was obtained in half of patients for a median overall survival reaching 12 months. In nonpretreated patients the overall response rate was 17%. Twenty-four percent of patients were without progression at six months. The median overall survival was almost 16 months with 72% surviving at one year. Predictive factors of response are being explored to identify patients who are most likely to respond to trabectedin. Combination with other agents are currently studied with promising results. In summary trabectedin is an active new chemotherapeutic agents that has demonstrated its role in the armamentarium of treatments for patients with sarcomas.Keywords: soft tissue sarcoma, trabectedin, chemotherapy, DNA-minor groove binder
Mahadeva, R; Stewart, S.; Wallwork, J
A 39 year old female presented with bilateral pneumothoraces and interstitial shadowing on chest x ray. A diagnosis of lymphangioleiomyomatosis was made following an open lung biopsy. Over the next eight years she developed respiratory failure leading to single lung transplantation but she died in the immediate postoperative period. Necropsy examination and review of the previous open lung biopsy revealed multiple pulmonary metastases from a low grade endometrial stromal sarcoma of the uterus...
Kondo, Tadashi; Kawai, Akira
Sarcomas are rare and clinically diverse malignancies, and treatment optimization requires the development of suitable biomarkers. In earlier research employing proteomics analysis, we identified peroxiredoxin 2 as a biomarker of osteosarcoma (OS) that can predict response to neoadjuvant chemotherapy and verified its functional significance in the resistance of OS cells to chemotherapeutic drugs. In addition, in gastrointestinal stromal tumor (GIST), we identified pfetin as a prognostic bioma...
Full Text Available Objective: Myeloid sarcoma is a tumoral mass of mature or immature myeloid blasts in extramedullary anatomic locations. It can be seen de novo or in association with acute myeloid leukemia, myeloproliferative neoplasias, or myelodysplastic syndrome. Isolated myeloid sarcoma can be seen as a relapse in cases with allogenic bone marrow transplantation. Although it may involve any tissue in the body, the most common locations are skin, soft tissues, lymph nodes, and the gastrointestinal tract. Immunohistochemically, most cases show myelomonocytic or pure monoblastic differentiation. We reviewed the clinicopathological features of 20 cases of myeloid sarcoma diagnosed in our institute in view of the literature. Materials and Methods: The cases diagnosed between 2005 and 2012 at the Ankara University Faculty of Medicine, Department of Pathology, were selected. Clinicopathological findings including the age and sex of the patients; symptoms; anatomic location; accompanying hematological disease; and the morphological, immunohistochemical, and cytogenetic features of the cases were noted. Results: Sixteen of the patients were male and 4 were female. The median age at diagnosis was 47 years. The most commonly involved locations were the lymph nodes and skin. Immunohistochemically, eleven cases were of the myelomonocytic and 7 cases were of the myeloid phenotype, whereas 2 cases showed pure monoblastic differentiation. The median follow-up period for the 18 cases with known clinical data was 33 weeks. Five patients died of the disease in an average of 36 weeks. Conclusion: Myeloid sarcoma is a rare presentation of leukemias, myeloproliferative neoplasias, or myelodysplastic syndrome, composed of immature myelomonocytic cells in extramedullary tissues. It may present with variable morphological and phenotypic features, always creating a challenge in pathological diagnosis.
Full Text Available Objective To study the clinical manifestations, pathologic features, diagnosis, treatment and prognosis of primary synovial sarcoma in the anterior mediastinum. Methods A case of primary synovial sarcoma in the anterior mediastinum was reported. Clinical features, imaging manifestations, pathology features and therapeutic effect were analysed and the relevant literature was reviewed. Results A 48-year-male patient was admitted with complaint of right chest pain for 4 days. Chest computerized tomography revealed a large mass located at the right anterior mediastinum, and it was primarily diagnosed as invasive thymoma. Pathological examination by CT-guided percutaneous needle biopsy manifested that, under microscope, the tumor cells were short and spindle in shape forming a nest structure, suggested it was a thymoma. The patient then underwent resection of thymoma with removal of fat and connective tissue in the anterior mediastinum. During the operation the size of the tumor was 15cm×15cm×10cm, being located at the anterior mediastinum, and it tended to bleed. The diagnosis of primary monophasic synovial sarcoma in the mediastinum was confirmed by postoperative/pathology examination. Immunohistochemistry staining showed that the tumor cells were positive for the markers Bcl-2 and EMA, but negative for the markers CK (pan and S100. The patient suffered from local recurrence with metastases to lung 4 months after surgery. The patient received 2 chemotherapeutic courses with ifosfamide, epirubicin and cisplatin. He died 6 months after surgery. Conclusion Primary synovial sarcoma in the anterior mediastinum is an extremely rare and highly malignant tumor with poor prognosis. The diagnosis depends on the pathological features, immunohistochemistry and RT-PCR. Radical resection combined with comprehensive treatment may improve the survival rate. DOI: 10.11855/j.issn.0577-7402.2014.12.12
Full Text Available Abstract Background In this study, the clinical and morphological features of vesiculobullous lesions observed in Kaposi sarcoma are analyzed, and the features of bullous Kaposi sarcoma cases are emphasized. Methods A total of 178 biopsy materials of 75 cases diagnosed as classic-type cutaneous Kaposi sarcoma were reviewed. Twenty-five cases showing vesiculobullous features were included in the study. Tumor, epidermis, dermis, and clinical data regarding these cases was evaluated. Results Vesicular changes were observed in 21 (12% out of 178 lesions of the 75 cases, while bullous changes were present in only 4 (2%. In all cases where vesicular and bullous changes were detected, tumor, epidermis, and dermis changes were similar. All cases were nodular stage KS lesions, whereas hyperkeratosis and serum exudation in the epidermis, marked edema in the dermis, and enlarged lymphatic vessels and chronic inflammatory response were observed. Conclusions Our findings suggest that changes in vascular resistance occurring during tumor progression are the most important factors comprising vesiculobullous morphology. Virtual slides The virtual slide(s for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1646397188748474
Perut, Francesca, E-mail: email@example.com [Laboratory for Orthopaedic Pathophysiology and Regenerative Medicine, Istituto Ortopedico Rizzoli, Bologna (Italy); Avnet, Sofia; Fotia, Caterina; Baglìo, Serena Rubina; Salerno, Manuela [Laboratory for Orthopaedic Pathophysiology and Regenerative Medicine, Istituto Ortopedico Rizzoli, Bologna (Italy); Hosogi, Shigekuni [Laboratory for Orthopaedic Pathophysiology and Regenerative Medicine, Istituto Ortopedico Rizzoli, Bologna (Italy); Department of Molecular Cell Physiology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto (Japan); Kusuzaki, Katsuyuki [Department of Molecular Cell Physiology, Graduate School of Medical Science, Kyoto Prefectural University of Medicine, Kyoto (Japan); Baldini, Nicola [Laboratory for Orthopaedic Pathophysiology and Regenerative Medicine, Istituto Ortopedico Rizzoli, Bologna (Italy); Department of Biomedical and Neuromotor Sciences, University of Bologna, Bologna (Italy)
Malignant tumors show intense glycolysis and, as a consequence, high lactate production and proton efflux activity. We investigated proton dynamics in osteosarcoma, rhabdomyosarcoma, and chondrosarcoma, and evaluated the effects of esomeprazole as a therapeutic agent interfering with tumor acidic microenvironment. All sarcomas were able to survive in an acidic microenvironment (up to 5.9–6.0 pH) and abundant acidic lysosomes were found in all sarcoma subtypes. V-ATPase, a proton pump that acidifies intracellular compartments and transports protons across the plasma membrane, was detected in all cell types with a histotype-specific expression pattern. Esomeprazole administration interfered with proton compartmentalization in acidic organelles and induced a significant dose-dependent toxicity. Among the different histotypes, rhabdomyosarcoma, expressing the highest levels of V-ATPase and whose lysosomes are most acidic, was mostly susceptible to ESOM treatment. - Highlights: • Osteosarcoma, rhabdomyosarcoma, and chondrosarcoma survive in acidic microenvironment. • At acidic extracellular pH, sarcoma survival is dependent on V-ATPase expression. • Esomeprazole administration induce a significant dose-dependent toxicity.
Lindner, L H; Angele, M; Dürr, H R; Rauch, J; Bruns, C
Patients with high-risk soft tissue sarcomas (FNCLCC grades 2-3, > 5 cm and deep lying) are at a high risk of local recurrence or distant metastases despite optimal surgical tumor resection. Therefore, multimodal treatment should be considered for this difficult to treat patient group. Besides surgery, radiation therapy and chemotherapy, hyperthermia has become a valid, complementary treatment option within multimodal treatment concepts. Hyperthermia in this context means the selective heating of the tumor region to temperatures of 40-43 °C for 60 min by microwave radiation in addition to simultaneous chemotherapy or radiation therapy. A randomized phase III study demonstrated that the addition of hyperthermia to neoadjuvant chemotherapy improved tumor response and was associated with a minimal risk of early disease progression as compared to chemotherapy alone. The addition of hyperthermia to a multimodal treatment regimen for high-risk soft tissue sarcoma consisting of surgery, radiation therapy and chemotherapy, either in the neoadjuvant or adjuvant setting after incomplete or marginal tumor resection, significantly improved local progression-free and disease-free survival. Based on these results and due to the generally good tolerability of hyperthermia, this treatment method in combination with chemotherapy should be considered as a standard treatment option within multimodal treatment approaches for locally advanced high-risk soft tissue sarcoma.
Introduction The complications of HIV/AIDS can produce grossly abnormal pathology. In low-resourced settings, women can present late with huge lesions. Massive vulval pathology copresenting in pregnancy produces difficulties in managing the patients and may lead to poor maternal or fetal outcomes. Case report A 27-year-old P1 G2 (second pregnancy one live birth) patient presented at 30 weeks gestation with a massive vulval lesion. She was HIV seropositive and taking anti-retroviral therapy. She was anemic with a hemoglobin level of 5.9 and was transfused 4.0 of packed cells. She underwent examination under anesthesia and vulval biopsy. She went into preterm labor and was delivered by cesarean section. Unfortunately, the baby had died while receiving corticosteroid therapy. The histopathological report confirmed a Kaposi’s sarcoma, and she was referred to oncologists for chemotherapy. Conclusion Kaposi’s sarcoma can occur in pregnancy in both seropositive and seronegative patients. Kaposi’s sarcoma causes significant fetal and maternal health complications. PMID:27536163
Full Text Available Sarcomas constitute less than 2% of all cancers, and are a highly diverse group of neoplasms. Primary sarcomas of the central nervous system (CNS are even less common, and our experience is limited by lack of studies with sufficient size that can address the challenges in predicting behavior or management. It is critical to recognize the characteristics of these uncommon neoplasms and to develop better predictors for prognosis and behavior.We have conducted a search of the UCSF Department of Pathology and UCSF Cancer Center Registry databases for all primary CNS sarcomas that were diagnosed and treated between 1985 and 2005. Hemangiopericytomas were included, so were the solitary fibrous tumors due to their close association with the former. We excluded all cases of metastatic sarcomas, chordomas, sarcomatoid variants of all neuroepithelial neoplasms, Ewing’s sarcomas and other embryonal tumors. In addition, we have identified all soft tissue sarcomas diagnosed and treated during the same period. There were 43 primary CNS neoplasms that fulfilled the inclusion criteria. At the same time, we identified 1706 sarcomas primary to the soft tissue. Primary CNS sarcomas included 16 hemangiopericytomas, 15 chondrosarcomas, 3 solitary fibrous tumors, 3 osteosarcomas, 2 leiomyosarcomas, 2 undifferentiated sarcomas, 1 histiocytic sarcoma, and 1 fibrosarcoma. There was a distinctly higher frequency of hemangiopericytoma in the CNS compared to soft tissue. In addition, a group of low grade, parasagittal chondrosarcomas were noted for their highly indolent biological behavior. Unlike some previous series, our cohort was devoid of angiosarcoma and malignant fibrous histiocytoma. This study underscores the limitations of single institutional series, and highlights the value of multi-institutional studies to understand and better treat primary CNS sarcomas.
Wilmar José Manoel
Full Text Available OBJETIVO: Relatar as características clínicas dos sarcomas de partes moles de alto grau e apresentar a experiência do Hospital Araújo Jorge no tratamento destes sarcomas. MÉTODO: Análise retrospectiva dos casos de sarcoma de alto grau em adultos admitidos no Hospital Araújo Jorge (HAJ entre 1996 e 2000. Idade, sexo, características anátomo-patológicas (tamanho e tipo histológico, localização, tratamentos oncológicos realizados (cirurgias de preservação de órgãos e membros, margens, quimioterapia, radioterapia, recorrência local, recorrência distante e sobrevida foram estudados. Análise descritiva, curvas de Kaplan-Meier, log-rank test e teste ÷² foram usados quando pertinentes. RESULTADOS: Foram registrados 235 pacientes com sarcomas de partes moles entre 1996 - 2000, sendo que 131 eram de alto grau. A média de idade foi de 47,2 anos. O tipo histológico não foi determinado em 23,7% dos casos. O tipo mais freqüente foi o leiomiossarcoma (13,7%, seguido do sarcoma sinovial (10,7% e rabdomiossarcoma (9,2%. O tamanho mediano foi de 10 cm (2-48 cm. A distribuição nos estádios II,III e IV foi de 15%, 55% e 30%, respectivamente. Nos pacientes com estádios II e III, a margem cirúrgica adequada foi obtida em 51,9% dos pacientes. Radioterapia e quimioterapia adjuvantes foram indicadas em 33,7% e 26,1% dos casos, respectivamente. As recorrências locais e distantes ocorreram em 31,5% e 34,8% dos pacientes, respectivamente. A sobrevida global em 5 anos foi 61,8%. CONCLUSÃO: A maioria dos pacientes atendidos no HAJ é portadora de lesões localmente avançadas, volumosas ou com metástase ao diagnóstico. Os pacientes apresentaram evolução adversa, com altas taxas de recorrência local e distante.
Isabela Alves Pacheco
Full Text Available Sarcomas are rare tumors, mainly stemming from the embryonic mesoderm, with a high grade of morbidity and mortality. OBJECTIVE: To carry out a retrospective study of head and neck sarcoma cases between 1999 and 2008 in three specialized centers in the city of Fortaleza. MATERIALS AND METHODS: Data collection was based on the charts of the patients in the study. For statistical analysis purposes we used the chi-square associations and the z test for proportions. RESULTS: We found records of 36 patients, and the most affected ones were adult brown males, in the age range between 20 and 59 years - mean age of 39.7. The man/woman ratio was 1.76:1. The most prevalent histological type was the rhabdomyosarcoma and their most common locations were the face and the neck. Most of the sample was made up of live patients without evidence of the disease in the last visit - 41.6% of the cases. The most common treatment modes were the combination of surgery + radiotherapy + chemotherapy and surgery + radiotherapy, with 27.8% of the cases each. CONCLUSION: Sarcomas have a great histological variability and may have numerous locations. Since these are rare and not well-known lesions, new epidemiological studies must be carried out in order to enhance our understanding of the disease.Sarcomas são tumores raros originados predominantemente do mesoderma embrionário e que apresentam elevado grau de morbidade e mortalidade. OBJETIVO: Realizar estudo retrospectivo dos casos de sarcomas em cabeça e pescoço no período de 1999 a 2008 em três centros especializados. MATERIAL E MÉTODO: A coleta de dados teve base nos prontuários dos pacientes estudados. Para análise estatística, foram utilizados os testes de associações qui-quadrado e o teste z para proporções. RESULTADO: Foram encontrados registros de 36 pacientes, sendo os indivíduos mais acometidos homens adultos, da raça parda, na faixa etária de 20 a 59 anos, com idade média de 39,7. A rela
Full Text Available O sarcoma sinovial primário da cabeça e pescoço é um tumor raro. Em torno de 90 casos foram descritos na literatura. Relatamos um caso adicional ocorrido em um paciente jovem do sexo masculino comprometendo a orofaringe. O tumor apresentou típico crescimento bifásico, fibrossarcomatoso e epitelial, com estruturas pseudo-glandulares. O diagnóstico final foi obtido pelo exame da peça cirúrgica com preparações histológicas de rotina e análise imunohistoquímica.Primary synovial sarcoma of the head and neck is a rare tumor. Fewer than 90 cases having been reported in the literature. This report presents an addicional case of synovial sarcoma involving the oropharinx in a young man. Microscopically the tumor showed a biphasic pattern of pseudo-ephitelial cells and a spindle cell element with fibrosarcomatous and epithelial appearance. A proper diagnosis was done after routine pathological and immunohistochemical studies of the ressected tumors.
Muhic, A.; Hovgaard, D.; Mork, Petersen M.;
PURPOSE: The purpose of this study was to evaluate local control, survival and complication rate after treatment of soft tissue sarcoma (STS) with limb-sparing surgery combined with pulsed-dose rate (PDR) interstitial brachytherapy (BRT) and external beam radiotherapy (EBRT). PATIENTS AND METHODS...... surgery, combined with PDR BRT and EBRT can result in good local control in patients with soft tissue sarcomas. BRT is an effective modality with good cosmetic results and acceptable toxicity Udgivelsesdato: 2008/9......PURPOSE: The purpose of this study was to evaluate local control, survival and complication rate after treatment of soft tissue sarcoma (STS) with limb-sparing surgery combined with pulsed-dose rate (PDR) interstitial brachytherapy (BRT) and external beam radiotherapy (EBRT). PATIENTS AND METHODS......: A retrospective review of 39 adult patients (female/male=25/14, mean age 51(range 21-78) years) with STS who underwent primary limb-sparing surgery combined with PDR BRT (20Gy) and additional post-operative EBRT (50Gy) during the years 1995-2004. RESULTS: Five patients developed local recurrence after a mean...
Hélias-Rodzewicz, Zofia; Pérot, Gaëlle; Chibon, Frédéric; Ferreira, Céline; Lagarde, Pauline; Terrier, Philippe; Coindre, Jean-Michel; Aurias, Alain
In a series of 404 adult soft tissue sarcomas, analyzed by array-CGH, we have observed in approximately 10% of them a genomic amplification of either chromosome bands 11q22 or 3p12. These two amplicons likely target the YAP1 and VGLL3 genes, respectively. Both genes encode proteins that are cofactors of the TEAD family of transcription factors. Very good correlations between amplification and expression levels were observed. Welch test analyses of transcriptome data demonstrate that tumors with amplicons share a large set of upregulated and downregulated genes. Inhibition of YAP1 and VGLL3 in cell lines with these amplifications/overexpressions leads to similar phenotypes: decrease of proliferation rate, and to a lesser extent decrease of migration properties. These data, and the fact that these amplicons are observed either in de-differentiated liposarcomas or in undifferentiated pleomorphic sarcomas, suggest that these genetics events could be involved in oncogenesis and progression of soft tissue sarcomas. © 2010 Wiley-Liss, Inc.
Issels, R D; Bosse, D; Abdel-Rahman, S; Starck, M; Panzer, M; Jauch, K W; Stiegler, H; Berger, H; Sauer, H; Peter, K
From November 1990 to September 1991, 23 adults with high-risk, nonmetastatic sarcomas (20 soft-tissue sarcomas and 3 chondrosarcomas) were entered in a pilot protocol (RHT-91) involving regional hyperthermia combined with systemic chemotherapy followed by surgery. Of these patients, 12 had undergone previous surgery and/or radiation, 5 had received previous multidrug chemotherapy, and 6 were previously untreated. A tumor size of > 8 cm and/or an extracompartmental tumor location (11 patients) or local recurrence (12 patients) were defined as high-risk factors in addition to tumor grading (21 patients had grade 2 or 3 sarcomas). Regional hyperthermia was produced by an electromagnetic deep-regional-heating device. For systemic chemotherapy, all patients received etoposide/ifosfamide/doxorubicin (EIA) and mesna, with regional hyperthermia being given only on days 1 and 4 in repeated EIA/regional hyperthermia cycles every 3 weeks. Tumor temperatures (range, 40 degrees-44 degrees C) were measured by invasive thermometry in all patients during each regional hyperthermia treatment. A total of 181 regional hyperthermia treatments were applied within the pelvic region (11 patients) or extremities (12 patients) bearing relatively large tumors (mean volume, 848 cm3). By the cutoff date for this analysis (October 15, 1991), 13 patients had undergone surgery after receiving 2-6 (mean, 3.8) cycles of EIA chemotherapy combined with regional hyperthermia; all tumors except one were resected without disfiguration. In 22 evaluable patients (minimum, 2 EIA plus regional hyperthermia cycles), the clinical response rate was 27%, with 6 patients showing partial responses (PRs). In addition, a pathologic response to preoperative thermochemotherapy was evaluable in 13 patients, with 4 responders (31%) having > 50% histologic necrosis. In all, 3 of the responders (1 PR and 2 patients with > 50% histologic necrosis) relapsed within 3 months of surgical resection. The other 7 responding