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Synovial sarcoma

Xiaoyang Li, Nicole A Seebacher, Cassandra Garbutt, Hangzhan Ma, Peng Gao, Tao Xiao, Francis J Hornicek, Zhenfeng Duan
Synovial sarcoma is a highly aggressive but rare form of soft tissue malignancy that primarily affects the extremities of the arms or legs, for which current chemotherapeutic agents have not been proven to be very effective. The cyclin-dependent kinase 4/6-retinoblastoma protein (CDK4/6-Rb) pathway of cell cycle control is known to be aberrant in a large proportion of cancers. Recently, CDK4 inhibitors have successfully been used pre-clinically for the treatment of many human cancers, and in 2015, following the success of clinical trials, the FDA approved the first selective CDK4/6 inhibitor, palbociclib, for the treatment of endocrine therapy resistant breast cancers...
April 18, 2018: Cell Death & Disease
Rima Gammoudi, Amina Aounallah, Colandane Belajouza, Rafiaa Nouira
Maffucci syndrome is a rare nonhereditary disorder comprising of lymphovascular malformations and multiple enchondromas, which may be associated with several internal malignancies. This report describes a new association of Maffucci syndrome with pedal synovial sarcoma. Our case is also remarkable as lymphangioma circumscriptum is the sole lymphovascular component, which has been rarely reported. The aim of this report is to generate awareness about this rare condition and also highlight the importance of screening for malignancies in this disorder...
April 17, 2018: Indian Journal of Dermatology, Venereology and Leprology
Isidro Machado, Akihiko Yoshida, María Gema Nieto Morales, Lucas Faria Abrahão-Machado, Samuel Navarro, Julia Cruz, Javier Lavernia, Antonina Parafioriti, Piero Picci, Antonio Llombart-Bosch
BACKGROUND: Despite extensive immunohistochemical (IHC) and molecular studies combined with morphologic findings, a group of round/ovoid cell tumors histologically similar to Ewing sarcomas (ES) but lacking EWSR1-rearrangements may remain unclassifiable. DESIGN: We retrospectively analyzed 41 Ewing-like tumors (formalin-fixed, paraffin-embedded) previously determined as negative or non-informative for EWSR1-rearrangements by FISH and/or RT-PCR. A new histopathology revision and additional IHC and molecular analyses were carried out in order to investigate whether additional IHC and/or molecular testing in combination with the morphological findings may help in reaching a definitive diagnosis...
November 29, 2017: Annals of Diagnostic Pathology
Hisaki Aiba, Satoshi Yamada, Jun Mizutani, Norio Yamamoto, Hideki Okamoto, Katsuhiro Hayashi, Hiroaki Kimura, Akihiko Takeuchi, Shinji Miwa, Takashi Higuchi, Kensaku Abe, Yuta Taniguchi, Yoshihiro Araki, Hiroyuki Tsuchiya, Takanobu Otsuka
PURPOSE: Radio-hyperthermo-chemo (RHC) therapy, which combines radiotherapy, hyperthermia, and chemotherapy, for malignant soft tissue tumors has been introduced with the aim of decreasing the possibility of local recurrence after surgery. To avoid unnecessary neoadjuvant therapy and to plan the appropriate surgical treatment, surveillance of RHC therapeutic efficacy during treatment is necessary. In this study, we determined the optimal response criteria to evaluate the efficacy of RHC by comparing preoperative images before and after RHC with pathological evaluation of necrosis in the resected tumor...
2018: PloS One
Sevastián S Medina-Ornelas, Herlinda Vera-Hermosillo, Rafael Delgado-Espín, Francisco O García-Pérez
Background: Imaging studies, particularly simple and contrast-enhanced tomography, constitute the first diagnostic approach to detect recurrence of musculoskeletal tumors. The aim of the present retrospective study was to demonstrate the usefulness of scintigraphy plus SPECT/CT (single photon emission computed tomography) with thallium-201 (201 Tl) in the evaluation of malignant musculoskeletal tumors with suspicion of recurrence or metastatic disease. Methods: Eight weeks after the last therapy, 72 scintigraphy and SPECT/CT studies were performed to assess regional recurrence and metastatic disease in 42 patients with different types of malignant musculoskeletal tumors, such as osteosarcoma, Ewing's sarcoma, rhabdomyosarcoma, retinoblastoma, synovial sarcoma, and Wilms tumor at the Hospital Infantil de México Federico Gómez...
2018: Boletín Médico del Hospital Infantil de México
Rieko Oyama, Fusako Kito, Marimu Sakumoto, Kumiko Shiozawa, Shunichi Toki, Makoto Endo, Akihiko Yoshida, Akira Kawai, Tadashi Kondo
Synovial sarcoma is an aggressive mesenchymal tumor, characterized by the presence of unique transfusion gene, SS18-SSX. Cell lines enable researchers to investigate the molecular backgrounds of disease and the significance of SS18-SSX in relevant cellular contexts. We report the establishment and proteomic characterization of a novel synovial sarcoma cell line. Primary tissue culture was performed using tumor tissue of synovial sarcoma. The established cell line was authenticated by assessing its DNA microsatellite short tandem repeat analysis and characterized by in vitro assay...
April 6, 2018: In Vitro Cellular & Developmental Biology. Animal
Wael Braham, Angelo Pisani, Bastien Poitier, Aurélie Sannier
Primary cardiac synovial sarcoma is an extremely rare disease. Its prognosis is poor with a median survival time of 24 months, even when adequate and timely treatment is given. This case reports a 61-year-old woman presenting with primary cardiac synovial sarcoma with an 8-year survival time following surgical and adjuvant chemotherapy.
March 30, 2018: Interactive Cardiovascular and Thoracic Surgery
Z Su, J Zhang, P Gao, J Shi, M Qi, L Chen, X Wang
This report outlines the work-up and management of a 35-year-old man with a synovial sarcoma of the lateral border of the tongue. Synovial sarcoma of the tongue is an extremely rare tumour with variable morphological microscopic types and characters of immunohistochemical profile. There have only been 15 cases previously reported. This report aims to update the literature on this subject and explore the best treatment options.
April 1, 2018: Annals of the Royal College of Surgeons of England
Yongjin Felix Lee, Robert K Cleary
We report a third case of a primary intramural synovial sarcoma of the lower gastrointestinal tract. A 50-year-old woman presented with hematochezia, dizziness and shortness of breath. CT imaging revealed a transverse colo-colonic intussusception with a colonic mass serving as a lead point. A subtotal colectomy was performed with oncologic resection of a sausage-like mass in the mid-transverse colon. Pathological assessment showed a biphasic synovial sarcoma. The postoperative hospital course was without complications and the patient was discharged home with plans for postoperative surveillance...
March 30, 2018: BMJ Case Reports
Clara Alfaro-Cervelló, Octavio Burgués
Primary synovial sarcoma of the breast is very rare. We report a case of a 33-year-old woman, who had previously undergone a radical mastectomy, having been diagnosed with fusocellular breast carcinoma. Histopathology revealed a hypercellular lesion formed by spindle cells with storiform and herringbone patterns. Immunohistochemistry showed strong expression of vimentin and CD99, and focal bcl2, EMA, CK AE1-AE3, actin and desmin, with negativity for S100, CD34, CK7, CK14, CK19, hormone receptors, caldesmon and myosin...
April 2018: Revista Española de Patología
Amna Asif, Sajid Mushtaq, Usman Hassan, Noreen Akhtar, Mudassar Hussain, Muhammad Azam, Romena Qazi
Introduction:Soft tissue sarcomas are rare tumors comprising 1 percent of solid malignancies. The latest edition of WHO soft tissue pathology lists 94 benign and malignant soft tissue tumors. Many of these show a large degree of morphological overlap. Immunohistochemistry has been shown to be reliable in many cases for differential diagnosis of lesions, although cytogenetic tests are considered the gold standard for many entities.Fluorescence in-situ hybridization (FISH) is a cytogenetic technique that uses fluorescent probes that bind to only those parts of the chromosome which have a high degree of sequence complementarity...
March 27, 2018: Asian Pacific Journal of Cancer Prevention: APJCP
Yong Huang, Dawei Liu, Junhang Luo, Wei Chen
Synovial sarcomas (SSs) are very rare, poorly studied tumors that generally occurs around joint and muscle tendons. Primary SSs of the kidney are even rarer, accounting for <2% of all malignant renal tumors. We report the case of a 44-year-old man who was diagnosed with primary renal SS on the basis of imaging, histopathological, and immunohistochemical examination. We also present a comprehensive review of the literature, with a focus on the differential diagnosis and treatment of renal tumors.
2018: Journal of Cancer Research and Therapeutics
Cheng Liu, Dingfeng Li, Jun Guo, Qiu Cui, Linlin Zhang, Yanjun Zeng
To probe into the reasons for misdiagnoses of pelvic tumor as deep venous thromboses as well as the diagnostic methods and effective treatments on pelvic tumor. Four case reports on misdiagnosing pelvic tumor as deep venous thromboses and further analysis on the causes of misdiagnosis, diagnosis, and treatment with the literature study. The four cases were misdiagnosed as pelvic tumor, which actually were fibroneuroma, myxo.fluidity liposarcoma, moderately differentiated squamous cell carcinoma, and synovial sarcoma, respectively...
2018: Journal of Cancer Research and Therapeutics
Ning Wang, Zi-Han Liu, Hong Zou, Li-Juan Pang, Wen-Yi Gu, Jian-Ming Hu, Dong-Mei Li, Jin Zhao, Jun Zhang, Chun-Xia Liu, Wen-Jie Zhang, Yan Qi, Feng Li
Synovial sarcoma (SS) is a mesenchymal malignant neoplasm showing characteristics of epithelial-mesenchymal biphasic differentiation. SS is of uncertain cellular origin; however, studies have suggested that SS originates from a somatic stem cell population.In this study, we aimed to determine whether differential morphological features of the epithelial-mesenchymal transition (EMT) contributed to the tumorigenesis of SS invasion and metastasis. Twelve paraffin-embedded formalin-fixed tissue (FFPE) SS tissue specimens were obtained, and laser capture microdissection (LCM) with the ArcturusXT system and small chip method (SCM) were used to isolate and purify spindle and epithelial cells from SS specimens...
March 25, 2018: Clinical and Experimental Pharmacology & Physiology
Christian Koelsche, Wolfgang Hartmann, Daniel Schrimpf, Damian Stichel, Susanne Jabar, Andreas Ranft, David E Reuss, Felix Sahm, David T W Jones, Melanie Bewerunge-Hudler, Marcel Trautmann, Thomas Klingebiel, Christian Vokuhl, Manfred Gessler, Eva Wardelmann, Iver Petersen, Daniel Baumhoer, Uta Flucke, Cristina Antonescu, Manel Esteller, Stefan Fröhling, Marcel Kool, Stefan M Pfister, Gunhild Mechtersheimer, Uta Dirksen, Andreas von Deimling
Undifferentiated solid tumors with small blue round cell histology and expression of CD99 mostly resemble Ewing sarcoma. However, they also may include other tumors such as mesenchymal chondrosarcoma, synovial sarcoma, or small cell osteosarcoma. Definitive classification usually requires detection of entity-specific mutations. While this approach identifies the majority of Ewing sarcomas, a subset of lesions remains unclassified and, therefore, has been termed "Ewing-like sarcomas" or small blue round cell tumors not otherwise specified...
March 23, 2018: Modern Pathology: An Official Journal of the United States and Canadian Academy of Pathology, Inc
Sarina Gouravan, Leonardo A Meza-Zepeda, Ola Myklebost, Eva W Stratford, Else Munthe
The BRAFV600E mutation, which in melanoma is targetable with vemurafenib, is also found in sarcomas and we here evaluate the therapeutic potential in sarcoma cell lines. METHODS: Four sarcoma cell lines harboring the BRAFV600E mutation, representing liposarcomas (SA-4 and SW872), Ewing sarcoma (A673) and atypical synovial sarcoma (SW982), were treated with vemurafenib and the effects on cell growth, apoptosis, cell cycle progression and cell signaling were determined. RESULTS: Vemurafenib induced a strong cytostatic effect in SA-4 cells, mainly due to cell cycle arrest, whereas only moderate levels of apoptosis were observed...
March 23, 2018: International Journal of Molecular Sciences
Ketul S Puj, Shashank J Pandya, Vikas Warikoo, Tapansingh Chauhan, Satarupa T Samanta
Primary renal synovial sarcoma(PRSS) is a rare entity. It should be considered as one of the differential diagnoses of spindle cell tumors of the kidney. Immunohistochemistry and genetic translocation studies should be used to confirm the diagnosis. Due to lack of consistent literature data regarding the treatment options, it remains a therapeutic challenge. In view of chemosensitive nature of the tumor, we propose multimodality treatment in form of surgery and chemotherapy in these patients. Here we report a rare case of PRSS in a 17 year old female...
March 19, 2018: Urology
Aanchal Kakkar, Madhu Rajeshwari, Pirabu Sakthivel, Mehar C Sharma, Suresh C Sharma
INTRODUCTION: Biphenotypic sinonasal sarcoma (BSNS) is a recently described mesenchymal tumor exclusive to the sinonasal region. It is a low grade sarcoma, displaying evidence of myogenic and neural differentiation. Role of β-catenin immunohistochemistry in distinguishing it from its morphological mimics is not well-established. We conducted this study to identify cases of BSNS from our archives, and to examine immunopositivity for β-catenin in them as well as in its close differential diagnosis...
April 2018: Annals of Diagnostic Pathology
Ashutosh Gupta, Gunjan Agrawal, Vivek Chaudhary, Heena Mazhar, Santanu Tiwari
Synovial sarcoma is the uncommon malignant tumor of children and adolescents. It usually involves the soft tissues of the upper and lower joints and most commonly affects adults in their fourth decade of life. We report a rare case of synovial sarcoma of the head and neck region in a five-year-old child who has presented with right side cervical lymphadenopathy associated with dysphagia and hoarseness of voice. Patient was managed by modified radical neck dissection followed by chemotherapy.
March 2018: Indian Journal of Surgical Oncology
Danny Mangual, Luis A Bisbal-Matos, Ricardo Jiménez-Lee, Román Vélez, Miguel Noy
The case of a 27-year-old Hispanic female who presented with an occipito-parietal tumor after suffering trauma to the area. A physical examination revealed no tenderness to palpation and with evidence of healing ulcerations. The biopsy was consistent with a synovial sarcoma. A wide excision of the mass (15cm x 14cm x 6cm) followed by a pericranial flap was performed. A follow-up CT showed recurrence involving the parietal sagittal sinus. After a second biopsy the mass was determined to be a small-cell sarcoma, consistent with Ewing's sarcoma...
March 2018: Puerto Rico Health Sciences Journal
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