Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Clear cell sarcoma is a rare tumor with an unpredictable clinical course and usually poor prognosis. Almost all of the patients will have pulmonary metastases during the clinical course. Other more common metastatic sites are bones, lymph nodes, liver and spleen. Treatment of clear cell sarcoma is primarily surgical aiming to achieve uninvolved margins. Role of chemotherapy, radiotherapy and immunotherapy is controversial and remission of metastases has been reported rarely. In this study we report a case of clear cell sarcoma, with gastroduodenal and pulmonary metastases and remission of these metastases in response to chemotherapy.
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PMID:Gastroduodenal metastases in a clear cell sarcoma patient. 1272 35

Clear cell sarcoma is a rare malignant soft tissue neoplasm that usually arises adjacent to tendons or aponeuroses. The clinical course is rather slow, with repeated local recurrences followed by late metastases and eventual death. The principal sites of this neoplasm are the extremities, but tumors do occur in the trunk on rare occasions. We report a case of clear cell sarcoma arising from the chest wall. The patient, a 20-year-old woman, had noticed a chest wall mass and pain for 2 years. Biopsy of the mass showed abundant nests of round cells with clear cytoplasm. On immunohistochemical examination, tumor cells were strongly immunoreactive for S-100 and HMB-45. A diagnosis of clear cell sarcoma was confirmed. There was no other lesion found in the patient through routine imaging studies. She was treated with two courses of chemotherapy using ifosfamide, carboplatin, and etoposide. Subsequently, the tumor, including adjacent tissue, the chest wall, and sternum, was resected with a wide margin; and the defect of the chest wall was covered with Marlex mesh fabric, regin, and a musculocutaneous flap. She has shown no symptoms or signs of recurrence during 2 years of follow-up.
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PMID:Clear cell sarcoma arising from the chest wall: a case report. 1504 47

Clear cell sarcoma of the tendons and aponeuroses (CCSTA) is an aggressive, rare soft-tissue tumor representing approximately 1 per cent of all soft tissue sarcomas. Although it appears to be histogenetically related to melanoma, its clinical behavior resembles soft tissue sarcoma with a propensity for lymph node metastases. We report our experience at a tertiary referral center. Eight cases of CCSTA evaluated at the University of Illinois at Chicago's Medical Center between 1980 and 2002 were retrospectively reviewed. Patient data analyzed included age, gender, anatomic location, size of tumor, development of local, regional, and distant recurrence, and patient status at last follow-up. Local recurrence did not develop in any patient. Metastases developed in three patients with median time to presentation of metastases of 23 months (range, 8-36 months) from the time of initial diagnosis. Two of these three patients died of their disease with an average interval until death of 22 months from the time of initial diagnosis. The disease-free 2- and 5-year survivals were 68 per cent and 50 per cent, respectively. The overall 2- and 5-year survivals were 86 per cent and 68 per cent, respectively. CCSTA is an aggressive tumor of the soft tissues. These tumors present a high risk for development of distant disease and therefore warrant aggressive surgical management and treatment. Neither aggressive multiagent chemotherapy nor radiotherapy appeared to have an impact on outcome.
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PMID:Clear cell sarcoma: an institutional review. 1509 80

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.
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PMID:Clear cell sarcoma (malignant melanoma of soft parts) and sentinel lymph node biopsy. 1570 94

Malignant melanoma of soft tissue, also called clear cell sarcoma (CCS) of tendons and aponeuroses, presents as a soft tissue mass, usually in the lower extremity of young females. We report two cases of CCS of the upper extremity--in a Korean and a Saudi Arabian girl. Because of the presence of melanin, melanosomes, S-100 protein and the tendency for regional nodal metastases, it has been suggested that this entity be considered a melanoma rather than a soft tissue sarcoma. The evidence for a neuroectodermal origin of these neoplasms is compelling. Despite these features, CCS has been reported as showing a distinct chromosomal translocation pattern t(12;22)(q13;q12) in certain isolated cytogenetic or molecular diagnostic case reports; however, there is no information about the breakpoints at genomic level. Such genetic studies need to be performed on primary and metastatic cutaneous, mucosal or ocular melanomas prior to considering them specific for malignant melanoma of soft tissue/CCS. Microscopically, solid nests and fascicles of pale fusiform or cuboidal cells are divided by fine fibrous tissue septa. Characteristically, the cells possess a round to oval nucleus with a central prominent nucleolus. Pleomorphism may or may not be present; the cytoplasm is clear due to the presence of glycogen but, occasionally, the cytoplasm is more eosinophilic, resembling a melanoma. A tumour size (> or = 5 cm) and the presence of necrosis are statistically significant predictors of poor prognosis. A lesion on the proximal part of an extremity progresses slower than one that is more distal (i.e. acral). An early diagnosis and initial radical surgery are essential for a favourable clinical outcome. Once regional lymph node metastases or haematogenous dissemination has occurred, the prognosis becomes dismal.
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PMID:Lessons to be learned: a case study approach. Malignant melanoma of soft tissue. 1571 52

Clear cell sarcoma of the tendons and aponeuroses is an aggressive, rare soft tissue tumor with frequent metastases to regional lymph nodes. Sentinel lymph node biopsy, which has dramatically changed the management of melanoma, was used for clear cell sarcoma for an evaluation of popliteal and groin lymph node status. Although all isosulfan blue-stained groin lymph nodes were negative for malignancy, a popliteal lymph node was positive. Adjuvant 50 Gy of radiotherapy to the popliteal node might have been effective for local control for one year.
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PMID:Sentinel lymph node biopsy reveals a positive popliteal node in clear cell sarcoma. 1633 18

Clear cell sarcoma (CCS) of soft tissue is a rare sarcoma with morphologic similarities to malignant melanoma but a distinct genetic background including a chromosomal translocation, t(12;22)(q13;q12), or a resultant EWSR1-ATF1 fusion gene. In addition, the tumors occurring in the gastrointestinal tract may have a variant fusion gene EWSR1-CREB1. This study analyzed the clinicopathologic and molecular genetic features of 33 CCSs of soft tissue. The patients' ages ranged from 13 to 73 years (median, 30 y), and there was a male predominance (20 males, 13 females). The tumors were located in the deep soft tissues of the extremities (N=25) or in the trunk or limb girdles (N=8). The median tumor size was 4 cm (range, 1 to 15 cm). The tumor cells were either spindle or epithelioid, and they were arranged predominantly in a short fascicular (N=19) or a solid sheetlike growth pattern (N=14). Minor histologic variations included the existence of rhabdoid cells (N=8), bizarre pleomorphic cells (N=6), alveolar structures due to loss of cellular cohesion (N=3), and a seminomalike pattern (N=2). Tumor necrosis was evident in 14 tumors, and the mitotic activity ranged from 0 to 43 mitotic figures (MF)/10 high-power fields (HPF) (mean: 4 MF/10 HPF). Immunohistochemically, the tumors were consistently positive for S-100 protein (33/33) and variably or focally for HMB45 (32/33), microphthalmia transcription factor (26/32), Melan A (23/32), CD57 (25/33), bcl-2 (30/32), synaptophysin (14/32), CD56 (7/32), epithelial membrane antigen (12/33), cytokeratin (AE1/AE3) (1/32), CD34 (3/32), c-erbB-2 (10/32), c-kit (5/32), and c-met (5/32). alpha-Smooth muscle actin, desmin, and cytokeratin (CAM5.2) were negative. Reverse transcription-polymerase chain reaction using RNA extracted from formalin-fixed, paraffin-embedded tissues demonstrated transcripts of the EWSR1-ATF1 (31/33) or EWSR1-CREB1 fusion gene (2/33). In 26 cases with available clinical information, local recurrences and metastases developed in 2 and 15 patients, respectively. Ten patients were dead of the disease, and the overall survival rate was 63% at 5 years. However, no clinicopathologic or molecular variables associated with the patients' prognosis were identified. This study confirms that CCS is an aggressive soft tissue tumor with a melanocytic phenotype and wider morphologic variations than had been generally considered. In cases with unusual histologic findings, molecular detection of the EWSR1-ATF1/CREB1 fusion genes provides critical information regarding the diagnosis of the tumor.
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PMID:Clear cell sarcoma of soft tissue: a clinicopathologic, immunohistochemical, and molecular analysis of 33 cases. 1830 Aug 4

Clear cell sarcoma of soft tissue (CCSST) is a rare soft tissue neoplasm with marked variable tumor progression and prognosis. Although morphologically similar to malignant melanoma, CCSST can be distinguished by the presence of a t(12; 22)(q13; q12) and/or associated EWSR1-ATF1 chimeric gene. CCSST has an affinity for the extremities and is capable of metastasizing to a wide variety of sites including bone, lung, and lymph nodes and rarely to skin, liver, heart, muscle, and brain. Metastases have been known to occur as late as 29 years after initial presentation. We report a case of a 33-year-old woman who presented with bilateral ovarian cystic tumors, ascites, and pulmonary nodules. Her past medical history was significant for clear cell sarcoma of the left foot 2 years earlier. Bilateral salpingo-oophorectomy was performed and the light microscopic and immunohistochemical findings coupled with the detection of an EWSR1 rearrangement by fluorescence in situ hybridization were compatible with a diagnosis of CCSST metastases to the ovaries. To the best of our knowledge, this is the first reported case of CCSST metastatic to the ovaries.
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PMID:Clear cell sarcoma of soft tissue metastatic to the ovaries: a heretofore unreported occurrence. 1962 Sep 41

Clear cell sarcoma (CCS) of tendons and aponeuroses/malignant melanoma (MM) of soft parts is a rare tumor and in the majority of cases presents a characteristic reciprocal translocation t(12;22)(q13;q12) that results in fusion of the EWS and ATF1 genes. Although the melanocytic differentiation of CCS is indisputable, its precise lineage remains unclear. Typically, the slowly growing tumor affects the extremities of adolescents or young adults, especially around the ankle and foot. CCS is classically regarded as a deep soft tissue tumor associated with tendons or aponeuroses. This traditional view is put into perspective by the description of primary CCS of the gastrointestinal tract that may have a variant fusion gene EWSR1-CREB1. We describe 12 cases of cutaneous CCS and discuss the differential diagnoses. These 12 cases share an identical immunohistochemical profile with MM and thus can easily be confused with a dermal variant of spindle cell MM or metastasis of MM. The patients' ages ranged from 6 to 74 years (median: 25 y), and there was a female predominance (10 females, 2 males). Most tumors (n = 9) were located on the extremities, 2 tumors arose on the back, and 1 on the abdomen. The mean tumor size was 0.97 cm (range, 0.4 to 1.7 cm). Six cases showed invasion of the subcutis, the other 6 cases were entirely dermal. Tumor necrosis was evident in 2 cases, melanin pigment in 2 cases, and ulceration in 1 tumor. All cases showed uniform nests and fascicles of pale spindled or slightly epitheloid cells with finely granular eosinophilic or clear cytoplasm. There was fair pleomorphism with plump spindled nuclei and significantly prominent nucleoli. Multinucleated wreath-like tumor giant cells were observed in two-thirds of cases, but were usually present only focally. The dense cellular aggregates were encased by delicate fibrous septa. The stroma showed a sclerotic reticulated pattern. Partly, the nests of spindle cells bordered the epidermis, prima vista mimicking junctional nests of melanocytes. The specific translocation pattern was confirmed in all cases by fluorescence in situ hybridization. Local recurrences and metastases developed in 2 and 3 patients, respectively, and 1 patient died of the disease.
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PMID:Cutaneous clear cell sarcoma: a clinicopathologic, immunohistochemical, and molecular analysis of 12 cases emphasizing its distinction from dermal melanoma. 2008 59

Clear cell sarcoma or melanoma of soft tissues is a rare tumor in young adults, mainly located in the extremities. We report a new case observed in a 15-year-old teenager who had injured her left knee 1 month before hospitalization. The patient consulted for deformation of the left knee with pain and limitation in flexion. A CT scan of the knee showed a hypodense mass of the anterior compartment of the left leg. The histological and immunohistochemical study of the biopsy fragment concluded in clear cell sarcoma of the soft tissue. The histogenesis of this tumor continues to be debated and raises diagnostic challenges for the pathologist who must eliminate melanoma metastases, whose prognosis is different.
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PMID:[Clear cell sarcoma of soft tissues: a case report]. 2057 Apr 97


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