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Spinal intradural extraosseous Ewing’s sarcoma  [cached]
Farrah Mateen,Aziza Nassar,Aditya Bardia,Aminah Jatoi
Rare Tumors , 2011, DOI: 10.4081/rt.2011.e7
Abstract: 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.
Ewings Sarcoma-Primitive Neuroectodermal Tumour-Rare Extraosseous Presentation  [PDF]
Arcot Rekha, Prabhu Purushothaman, Rufus Ranjitsingh Edwin, Anukiran Ravichandran
Case Reports in Clinical Medicine (CRCM) , 2016, DOI: 10.4236/crcm.2016.53017
Abstract: Introduction: Ewings sarcoma is uncommon in extra osseous locations. They belong to Ewings family of tumours and the primitive neuroectodermal tumour is often diagnosed based on immuno histochemistry and molecular genetic studies. Discussion: A 50-year-old gentleman presented with complaints of a swelling in the right thigh. With a diagnosis of soft tissue sarcoma, the patient underwent a wide local excision of the tumour. We review literature of the small round blue cell tumour. Conclusion: Extraosseous Ewings sarcoma presenting as a thigh swelling is uncommon. The sarcoma was vimentin positive and CD99 membrane positivity.
Extraosseous Intra-Articular Osteochondroma  [PDF]
Pragash Mohanen,Kumaresan Palania Pillai,Kanagasabai Rangasamy
Case Reports in Orthopedics , 2013, DOI: 10.1155/2013/181862
Abstract: Background. Conventional osteochondromas are common bone lesions developing in the metaphyseal region of growing skeleton. Marginal excision is the treatment of choice for such tumours. Extraosseous cartilaginous tumours are rare and their biological potential is poorly characterized. Case Presentation. A-52-year old woman presented with 3-year history of fullness and dull pain and inability to flex her left knee, sit cross-legged, or squat. Clinical and imaging studies revealed a nodular mineralised mass in the anterior portion of the knee displacing the patellar tendon laterally. Excision biopsy confirmed the diagnosis of extraosseous osteochondroma-like soft tissue mass. There is no recurrence at two-year followup. Conclusion. An integrated clinicopathological diagnosis helps to clarify the nature of extraosseous cartilaginous tumour that can arise at an unusual anatomic site. Complete surgical excision is the treatment of choice. 1. Background Conventional osteochondromas are common bone lesions developing in relation to the periosteum and occur around the growth plate of long bones, especially the knee [1]. They tend to grow away from the joint. These tumours usually stop growing with closure of the physeal plate [1]. Conventional treatment is by marginal excision. Synovial chondromatosis is a less-common proliferation of multiple nodules of hyaline cartilage within the synovium, in most cases requiring thorough synovectomy [2]. Para-articular or extraosseous osteochondromas are rare in older individuals [3] and their biological potential is poorly characterized. In joints with a large capsular space such as the knee joint, osteochondromas can remain intra-articular [4]. Usually arising in juxta-articular soft tissue without attachment to bone, these lesions may be very large and show histologic features suggestive of a malignant process, including hypercellularity of the cartilaginous component and atypia of individual chondrocytes. Furthermore, some of these tumours continue to grow after skeletal maturity. Because marginal excision is adequate management, it is important to distinguish the extraosseous osteochondroma from chondrosarcoma and synovial chondromatosis. The terminology used to describe these osteocartilaginous lesions has been historically inconsistent and confusing and so far only 21 cases have been reported in the literature [4–14] especially from the West. Here we report one more case of extraosseous osteochondroma-like soft tissue mass in the anterior portion of the knee joint. 2. Case Report A-52-year old woman presented with
Fine-Needle Aspiration Cytology of Ewing’s Sarcoma of Thoracic Spine with Extension into the Intradural Space  [PDF]
Sandhya Bordia,Sweta Meena,Bijendar Kumar Meena,Vijay Rajak
Case Reports in Oncological Medicine , 2014, DOI: 10.1155/2014/351386
Abstract: Ewing’s sarcoma/peripheral primitive neuroectodermal tumor is a small, round, and blue cell malignancy that occurs most often in bone and soft tissues of children and young adults. The intraspinal manifestation of the disease is rare, and when present, this is often misdiagnosed with other varieties of primary spinal tumors. Fine-needle aspiration cytology (FNAC) plays important role in the early diagnosis of these cases. We report such a case of Ewing’s sarcoma of thoracic spine with extension into the intradural space, which was initially suspected to be a case of metastatic lesion in an 18-year-old boy. 1. Introduction Ewing’s sarcoma family of tumors are a group of small round-cell neoplasms, which include Ewing’s sarcoma (EWS), primitive neuroectodermal tumor (PNET), Askins tumor, PNET of the bone, and extraosseous Ewing’s sarcoma (ESS) [1]. Ewing’s sarcoma/peripheral primitive neuroectodermal tumor is presumed to be in neuroectodermal children and young adults. The intraspinal extraosseous Ewing’s sarcoma (CNS-ESS) is extremely rare and often misdiagnosed. 2. Case Report An 18-year-old boy was admitted to Neurosurgery Ward of RNT Medical College, Udaipur Rajasthan, with chief complaints of progressively increasing dullaching pain in the lower back and both limbs and tenderness at D7, D8 vertebral region. There was a history of loss of appetite and malaise. The patient was thin built and on general examination there was a mild pallor. Systemic examination did not reveal any abnormalities except weakness (power 3/5) in both lower limbs and decrease in sensation of touch. Investigation revealed haemoglobin 9?gm.%, total leukocyte count 8200?cells/mm?cu with 70% polymorphs and 26% lymphocytes. Erythrocyte sedimentation rate was 60?mm at the end of 1 hour. The urine examination and bone marrow examination did not reveal any abnormality. Tuberculin test was negative. Chest radiograph was normal. CT scan (Figure 1) of the spine showed an expansile lytic lesion of posterior element of D7 vertebrae involving the spinous process, associated with heterogeneously enhancing soft tissue component, which was extending from D7 to D9 vertebrae. Figure 1 The soft tissue component was seen involving the thecal sac, lamina, and spinous process of D7 and causing compression and displacement of spinal cord anteriorly. MRI of the spine (Figure 2) reveals an expansile lesion of posterior element of D7 involving spinous process and neural arch, associated with fairly large, enhancing soft tissue component in posterior paraspinal region extending from D6-D7 to D8-D9
Ewing's Sarcoma of big Toe
Javaid A.Bhat,Naseer A.Mir,Sushail Maajid,S.D. Palijor,Suhail Bashir,Ishaq,Wahid Bhat
JK Science : Journal of Medical Education & Research , 2003,
Abstract: Extraosseous Ewing's sarcoma of the big toe with metastasis to lung in an eighteen year old malebecause of rarity is presented.
Cell Cycle Deregulation in Ewing's Sarcoma Pathogenesis  [PDF]
Ashley A. Kowalewski,R. Lor Randall,Stephen L. Lessnick
Sarcoma , 2011, DOI: 10.1155/2011/598704
Abstract: Ewing's sarcoma is a highly aggressive pediatric tumor of bone that usually contains the characteristic chromosomal translocation t(11;22)(q24;q12). This translocation encodes the oncogenic fusion protein EWS/FLI, which acts as an aberrant transcription factor to deregulate target genes necessary for oncogenesis. One key feature of oncogenic transformation is dysregulation of cell cycle control. It is therefore likely that EWS/FLI and other cooperating mutations in Ewing's sarcoma modulate the cell cycle to facilitate tumorigenesis. This paper will summarize current published data associated with deregulation of the cell cycle in Ewing's sarcoma and highlight important questions that remain to be answered. 1. Introduction First described by James Ewing in 1921, Ewing’s sarcoma is characterized as a highly aggressive, undifferentiated tumor of the bone [1]. Although it is the second most common primary bone tumor in children and adolescents, Ewing’s sarcoma can also develop in extraosseous sites as a soft-tissue malignancy [2, 3]. The etiology of Ewing’s sarcoma remains unknown, but there appears to be a predominance of cases within the Caucasian population, with males being slightly more susceptible than females [3, 4]. This disease is highly invasive with approximately one-fourth of all Ewing’s sarcoma patients presenting with metastases at the time of diagnosis [2, 5]. Current treatment methods include surgery, radiation, and systemic chemotherapy [6]. Despite such an aggressive regimen, the 5-year disease-free survival rate for patients with localized Ewing’s sarcoma is only 60–70% and that for individuals presenting with metastases drops to a mere 30% [5, 7]. Approximately 85% of Ewing’s sarcoma tumors harbor the reciprocal translocation t(11;22)(q24;q12), which fuses the portion of EWSR1 from chromosome 22 with the portion of FLI1 from chromosome 11 [8, 9]. EWSR1 encodes the EWS protein, which belongs to the TLS/EWS/TAF15 (TET) family of putative RNA-binding proteins [10, 11]. Understanding the physiologic roles of TET proteins has recently become of greater scientific interest as data continues to surface identifying these members as being intrinsic to the development of other sarcomas arising from similar chromosomal translocations. Currently, EWS has been hypothesized to perform a number of functions, including, but not limited to: RNA transcription and/or processing, neuronal cell differentiation, meiosis, B-lymphocyte development, and proneural cell survival in the developing zebrafish embryo [12]. Interestingly, it also appears that EWS may
Extraosseous Ewing’s tumor of lateral abdominal wall  [cached]
Nikhil Gupta,Tirlok Chand,Nidhi Yadav,Rajeev Kumar
Clinics and Practice , 2011, DOI: 10.4081/cp.2011.e135
Abstract: Extraosseous Ewings tumor (EES) is a rare entity. Few cases have been reported in literature. There are no specific guidelines for management of this disease. We are reporting a case of EES arising from left lateral abdominal wall. We did wide excision of tumor followed by chemoradiation. Patient is asymptomatic after 8 months of follow up.
Well-Differentiated Extraskeletal Osteosarcoma Arising from the Retroperitoneum That Recurred as Anaplastic Spindle Cell Sarcoma
Hiromasa Arai,Yasushi Rino,Teppei Nishii,Norio Yukawa,Nobuyuki Wada,Hisashi Oshiro,Tsuyoshi Ishida,Noboru Nakaigawa,Munetaka Masuda
Case Reports in Medicine , 2010, DOI: 10.1155/2010/327591
Abstract: Extraskeletal osteosarcoma is an uncommon high-grade malignant soft tissue sarcoma. Well-differentiated extraskeletal osteosarcoma is thought to have a better prognosis than classical extraskeletal osteosarcoma, but dedifferentiation after recurrence has also been reported. We present a case of a primary retroperitoneal extraskeletal osteosarcoma in a 62-year-old Japanese woman. Abdominal CT revealed a large mass with diffuse calcification in the right retroperitoneal space and tumor resection was performed. The histopathological diagnosis was well-differentiated retroperitoneal extraskeletal osteosarcoma. She was followed up by CT every 6 months without adjuvant radiotherapy and chemotherapy for 31 months until anaplastic high-grade spindle cell sarcoma recurred in the retroperitoneum. Our case is the seventh reported description of well-differentiated extraskeletal sarcoma, and the first to arise in the retroperitoneum and recur as an entirely dedifferentiated spindle cell sarcoma.
Large Mass Arising From the Tongue as an Initially and Sole Manifestation of Kaposi Sarcoma  [cached]
Amir Feily, Esmaeil Rafeie, Zahra Moosavi, Ahmad Khazanee, Nastaran Ranjbari, Kambiz Masoumi, Omid Ghasemzadeh, Mosleh Safarpoor
International Journal of Medical Sciences , 2011,
Abstract: We report a 30- year-old Iranian woman presenting with a red to yellowish, well demarcated, painless exophytic and lobulated mass originating from the right hand side of the tongue. An excisional biopsy was obtained and it was diagnosed histopathologically as Kaposi's sarcoma by detecting atypical spindle cells with rare mitoses delineating blood-filled vascular slits.
Primary synovial sarcoma of the parapharyngeal space: a clinicopathologic study of five cases  [cached]
Zhu Ming,Li Jun,Wang Ke-Jing,Shang Jin-Biao
World Journal of Surgical Oncology , 2012, DOI: 10.1186/1477-7819-10-158
Abstract: We report five cases of primary synovial sarcomas arising in the parapharyngeal space. The patients were all men with a median age of 35 years (range 22 to 41 years). The tumors were non-encapsulated solid masses ranging from 2.0 to 6.6 cm in size. Histologically, three cases were biphasic subtype, and the other two cases were monophasic subtype. Immunohistochemically, the tumor cells were strongly positive for bcl-2 and CD99, partly positive for CK and EMA, and negative for CD117, CD34, SMA and desmin in all five cases. S-100 protein was detected in one case. The presence of an SYT–SSX1 and/or SYT-SSX2 gene fusion resulting from t(X;18) was demonstrated from paraffin blocks by reverse transcriptase polymerase chain reaction in five cases. All five patients received tumor radical excision and postoperative radiotherapy, and two patients with pulmonary metastasis received additional chemotherapy. Follow-up data revealed that two patients with tumor size <5 cm were alive without disease for 54 and 57 months, one patient with tumor size <5 cm was alive with pulmonary metastasis for 78 months, and two patients with tumor size >5 cm died of disease 26 and 37 months after the diagnosis, respectively. Primary parapharyngeal synovial sarcoma is a rare variant that occurs more frequently in males than females. Accurate diagnosis depends on morphologic and immunohistochemical examination and proper molecular analysis. The prognosis is relatively good in those patients whose tumor size is less than 5 cm.
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