Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C1261473 (sarcoma)
 25,952 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Alveolar soft part sarcoma is a very uncommon soft tissue malignancy which accounts for <1% of soft tissue sarcoma. It is a malignant and highly vascular tumor arising most commonly in the musculature of the lower extremities, with metastasis primarily to the lungs, bones, and brain. Cardiac metastasis is very rare and only 5 cases have been reported in the literature so far. We report a case of a young woman with a history of surgically resected alveolar soft part sarcoma of left thigh who presented with persistent dry cough and was found to have a cardiac mass, which on biopsy proved to be alveolar soft part sarcoma.
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PMID:Alveolar Soft Part Sarcoma with Unusual Cardiac Metastasis: A Case Report and Review of the Literature. 2884 14

Alveolar soft part sarcoma is a very rare, slow growing highly angiogenic tumor with poor prognosis. Most common site in children and infants is head and neck region and in adults it most commonly occurs in extremities especially thigh. In our case study, an 8 years old female patient presented with a gradually progressive left shoulder lump. FNAC from the lesion showed cellular smears with polyhedral and spindly cells showing abundant finely vacuolated cytoplasm, nuclear pleomorphism, intranuclear pseudoinclusions, and few bare nuclei. Perivascular arrangement of cells was peculiar in addition to the presence of intracytoplasmic metachromatic PAS positive diastase resistant granules. A presumptive diagnosis of alveolar soft part sarcoma with differentials of granular cell tumor and PEComa was considered and the lesion was excised. Although the histopathological features were not characteristic (ie, showing mainly solid pattern without classic alveolar pattern), immunohistochemistry were diagnostic (negative for S 100, Desmin, Cytokeratin, EMA, and moderate to strong nuclear positivity for TFE3). Thus, the diagnosis of alveolar soft part sarcoma was established. This case is being presented for its rarity and unique cytological and histopathological features.
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PMID:Alveolar soft part sarcoma: A case report with emphasis on some unusual cytological features. 2890 97

Alveolar soft part sarcoma (ASPS), a rare soft tissue sarcoma in children and adolescents, carries a poor prognosis. ASPS is an aggressive tumor of controversial histogenesis that, unlike other soft tissue sarcomas, tends to metastasize to the brain. A 9-year-old boy presented to our outpatient clinic in April 2009 with a chief complaint of a large painless mass in the left thigh whose size had increased significantly over the past 10 months. After staging the tumor, we performed open biopsy; the diagnosis was ASPS and he underwent wide local excision. In the course of 4-year follow-up by clinical and imaging studies, there was no evidence of early tumor recurrence or metastasis. Complete surgical resection is the treatment of choice in patients with ASPS.
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PMID:Alveolar Soft Part Sarcoma of the Extremity: Case Report and Literature Review. 2914 76

Alveolar soft part sarcoma (ASPS) is a rare soft tissue neoplasm. The incidence of orbital involvement is the highest in ASPS arising in the head and neck region. However, limited information is available regarding its clinical characteristics. The present study presents the clinical manifestations, imaging findings, pathological features, treatment strategies and prognosis records of 8 cases of orbital ASPS over the last 15 years in a single hospital, along with a review of the literature. Included were 3 male and 5 female patients, with the median age at presentation being 9.5 years. The mean average largest tumour diameter was 3.6 cm. A total of 5 patients underwent surgical excision of the tumour, with 2 undergoing orbital exenteration and 1 undergoing partial orbital exenteration. In total, 6 patients received postoperative radiotherapy and 2 received chemotherapy. Upon follow-up, 6 patients were doing well with no evidence of recurrence or metastasis. Local recurrence developed in 2 patients, of whom 1 succumbed following withdrawal from treatment. According to the present series and the cases mentioned in the literature, orbital alveolar soft part sarcoma has characteristics distinct from those of alveolar soft part sarcoma which arises in other locations. Orbital alveolar soft part sarcoma presents itself in a younger population with a shorter course of disease, smaller tumour size, improved prognosis, a marked association with the extraocular muscles and with the Ki-67 proliferation index possibly associated with prognosis of the disease.
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PMID:Orbital alveolar soft part sarcoma: A report of 8 cases and review of the literature. 2928 93

Alveolar soft part sarcoma (ASPS) is a very rare sarcoma, report <1% of all soft tissue tumor. Majority of cases were young adult and tumor occurred in lower extremities and trunk. Here, we present a case of 53-year-old Thai female with rapidly glowing mass over her right forearm. The magnetic resonance imaging scan demonstrated a hypervascular mass with multiple feeding vessels located in flexure tendon of right forearm. Ultrasound-guided biopsy revealed malignant poorly differentiated epithelioid neoplasm with clear cell feature and focal necrosis. Surgery may be considered the first-line treatment in localized ASPS and may potentially increase long-term survival. Complete surgical excision is the mainstay of treatment. TFE3 and Cathepsin K immunohistochemistry are useful in confirming a diagnosis of ASPS with a distinctive clinicopathologic features.
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PMID:Alveolar soft part sarcoma of flexure tendon. 2930 6

Alveolar soft part sarcoma (ASPS) is a rare form of soft-tissue sarcoma arising from connective tissue. It is most often seen in adolescents and young adults and has high propensity for recurrence and metastasis. Clinically, it mimics haemangioma or arteriovenous malformations. In our case report, an 18-year-old female presented with markedly vascular tumour in the left forearm, for which excision biopsy was done. Histopathological report revealed ASPS. The patient was screened for metastasis. Ultrasound abdomen, computed tomography (CT) chest, CT brain and whole-body skeletal survey was done. The patient was found to have bilateral pulmonary metastasis. The patient was given 6 cycles of chemotherapy with adriamycin, cyclophosphamide and vincristine. There was no locoregional and pulmonary recurrence for 11 months after being treated by excision of the tumour followed by chemotherapy.
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PMID:Alveolar soft part sarcoma of the forearm. 2961 68

Alveolar soft part sarcoma (ASPS) is a highly aggressive rare soft tissue sarcoma (STS) with poor prognosis especially in the metastatic form. ASPS is resistant to standard chemotherapy. Although, early diagnosis and surgical resection of operable tumor could lead to improved patient survival but novel treatment options are needed for advanced (metastatic) ASPS. This malignancy exhibits highly angiogenic behavior which reflects hyper-activation and over expression of angiogenic factors. Understanding the molecular events in this type of sarcoma is important in finding novel molecular based targeted therapies. We aim to review molecular aspects of ASPS growth and treatment.
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PMID:Molecular Landscape in Alveolar Soft Part Sarcoma: Implications for Molecular Targeted Therapy. 2971 May 5

Alveolar soft part sarcoma is a rare highly malignant neoplasm of the soft tissue and usually occurs in the lower extremities of children and young adults. We report two cases of alveolar soft part sarcoma: a 24-year-old Latino man with a 10-cm neck mass and a 56-year-old Latino woman with a recurring thigh mass. Fine-needle aspiration and a core biopsy were performed on both, which was followed by tumor resection on the man. The smears displayed numerous loosely cohesive or single large cells with abundant granular cytoplasm, round nuclei, vesicular chromatin, and occasional prominent nucleoli. Periodic and Schiff (PAS)-positive, diastase-resistant rhomboid, or needle-shaped crystals were present. Both tumors had diffuse and strong nuclear TFE3 expression and aberrant cytoplasmic CD68 expression. Fluorescence in situ hybridization analysis was performed in the first case, which detected a characteristic translocation t(X;17)(p11;q25). The diagnosis of alveolar soft part sarcoma was rendered in both cases. Herein, we present the cytology, histology, immunohistochemistry, and molecular findings and discuss the differential diagnosis.
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PMID:Fine-needle aspiration of alveolar soft part sarcoma: Histologic correlation and aberrant CD68 expression. 3045 6

Alveolar soft part sarcoma (ASPS) is an exceptionally rare non-rhabdomyosarcomatous soft tissue sarcoma (NRSTS), characterized by the translocation t(X;17) p(11.2;q25). This translocation results in the chimeric ASPSCR1-TFE3 transcription factor which drives tumorigenesis. Complete surgical resection is crucial in allowing a successful outcome in these cases. Here, we describe an 11-month-old female infant who presented with a well-circumscribed lesion of the tongue, with the clinical and radiologic appearances of an infantile hemangioma. This led to an initial plan for surveillance management. However, the mass continued to enlarge and the lesion was therefore biopsied when the infant was 17 months old. Histology showed plump epithelioid tumor cells, in many places lining pseudoalveolar spaces. Occasional Pas-D inclusions were present in the cytoplasm. Immunostaining showed nuclear positivity for TFE-3. Real-time quantitative polymerase chain reaction testing confirmed the presence of ASPSCR1-TFE3 fusion transcripts, characteristic of the translocation t(X;17) p(11.2;q25) observed in ASPS. This represents the youngest reported ASPS case with a confirmed molecular diagnosis. Complete surgical resection was undertaken and a surveillance imaging schedule implemented. This case highlights the need for regular review of the initial diagnosis and the importance of multidisciplinary discussion and early biopsy where the clinical course does not follow that expected for the putative (nonhistologically confirmed) diagnosis.
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PMID:Lingual Alveolar Soft Part Sarcoma in a 1-Year-Old Infant: Youngest Reported Case With Characteristic ASPSCR1-TFE3 Fusion. 3074 36

Alveolar soft part sarcoma is a rare malignant soft tissue tumor, mainly localized in the extremities and occurring principally in adolescents and young adults. Alveolar soft part sarcoma are uncommon in the female genital tract. We here report a case of alveolar soft part sarcoma in a 20-year-old nullipara, presenting with vaginal bleeding and profound anemia requiring blood transfusions. Ultrasonographic examination revealed a polyp in the lower uterine segment. Surgical resection of the polyp was performed, and pathological evaluation showed typical histological, immunohistochemical, and molecular features consistent with alveolar soft part sarcoma. Patient underwent for total hysterectomy. Currently, she follows up with her surgeon and has no new complains. Given the infrequency of alveolar soft part sarcoma, this case report raises the awareness of alveolar soft part sarcoma as one of the entities to consider when confronted with a uterine polyp in a young patient.
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PMID:Alveolar soft part sarcoma presenting as a uterine polyp: A case report. 3218 Sep 83


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