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 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.
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PMID:Primary alveolar soft part sarcoma of fibula demonstrating ASPL-TFE3 fusion: a case report and review of the literature. 1859 8

Sarcoma metastatic to the brain is uncommon and rarely occurs as the initial manifestation of tumor. Alveolar soft part sarcoma (ASPS) is a rare but well-studied subtype of sarcoma. A 39-year-old man presented with seizures due to a left temporal meningeal-enhancing lesion with striking brain edema on MRI. The patient underwent neurosurgical resection for suspected meningioma. Histology showed large tumor cells clustering and forming small nests, in places with pseudoalveolar pattern. Diastase-resistant periodic acid-Schiff revealed very rare granular and rod-like cytoplasmic inclusions. Immunohistochemistry showed convincing positivity only with vimentin and smooth muscle actin. The histological features were strongly suggestive of ASPS. At the molecular level RT-PCR and sequencing analysis demonstrated ASPCR1-TFE3 fusion confirming the histological diagnosis of ASPS. There was no evidence of primary extracranial tumor by physical examination and on chest and abdominal CT scan 11 months after presentation. ASPS typically arise from the soft tissues of the extremities and develop multiple metastatic deposits usually with a long clinical course. This case may represent primary meningeal ASPS although metastatic deposit from an undiscovered primary site cannot be entirely excluded.
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PMID:Meningeal alveolar soft part sarcoma confirmed by characteristic ASPCR1-TFE3 fusion. 1871 11

Alveolar soft part sarcoma is a rare, aggressive malignancy of uncertain histological origin with a propensity for vascular invasion and distant metastasis. The case presented involves a 31-year-old woman with alveolar soft part sarcoma in the tongue root. The clinical features, pathogenesis, diagnosis and treatment were discussed.
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PMID:[Alveolar soft part sarcoma of the tongue root: a case report]. 1878 May 14

Alveolar soft part sarcoma (ASPS) is a rare soft tissue sarcoma which is characterized by the presence of a specific chromosomal translocation encoding the chimeric transcription factor (ASPL-TFE3) that activates expression of MET. We reviewed the clinical features and treatment outcome of 12 ASPS patients. The presence of ASPL-TFE3 fusion transcripts was assessed by reverse transcriptase polymerase chain reaction. In addition, we performed immunohistochemical studies for MET, TFE3, Ki-67, and EGFR expression. Lower extremity was the most commonly affected primary site (2 thigh, 3 lower leg, and 1 foot). Of four patients who received primary cytotoxic chemotherapy, no patient demonstrated treatment response. With follow-up duration of 94.4 months, median overall survival was 53.2 (95% C.I. 40.9-65.5) months. The immunohistochemical staining demonstrated 100% TFE3 positivity (8 of 8), 75% MET positivity (6 of 8) with a strong association between TFE3 expression and MET positivity with correlation coefficient of 0.808 (P = 0.02). The high expression of MET in ASPL-TFE3 (+) ASPS may further support the potential role of targeted agents against MET in this rare, chemoresistant tumor.
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PMID:Expression of MET in alveolar soft part sarcoma. 1947 90

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.
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PMID:Ultrasonographic and MR findings of alveolar soft part sarcoma. 1954 71

Alveolar soft part sarcoma is a rare type of sarcoma that usually affects young adult women. It occurs mostly in the lower extremities, and nearly one quarter of the cases are found in the head and neck region. The most common site of origin in the head and neck region is the tongue followed by the orbit. Herein, we present an unusual case of alveolar soft part sarcoma of the tongue in an 18-year-old woman. The clinical, diagnostic, and therapeutic features of this quite rare entity were discussed.
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PMID:Alveolar soft part sarcoma of the tongue. 1988 39

Alveolar soft part sarcoma (ASPS) is a rare subtype of soft tissue sarcoma that occurs predominantly in young patients. Despite its relatively indolent course, it generally has a poor prognosis with widespread metastases. The common metastatic sites from an ASPS include the lung, brain and bone. However, metastasis of an ASPS to the gastrointestinal tract is extremely rare. Here, we report a rare case of upper gastrointestinal bleeding and jejunal intussusception due to gastrointestinal metastases from an ASPS.
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PMID:Unusual gastrointestinal metastases from an alveolar soft part sarcoma. 2044 9

Alveolar Soft Part Sarcoma (ASPS), also called Alveolar Soft-Tissue Sarcoma, is a rare type of soft-tissue neoplasm with a poor long term prognosis. Such tumors originating in the retro peritoneal space are extremely rare. In this article we discuss a 34-year-old woman who was referred to our hospital with an increasing mass in her left lower abdomen. Ultrasonography and conventional Computed Tomography revealed a large hard mass occupying the left retroperitoneal space with a clear border. The pathological diagnosis was ASPS.
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PMID:Alveolar soft part sarcoma of the retro peritoneum. 2047 65

Alveolar soft part sarcoma (ASPS) is a very rare, but distinctive type of soft tissue sarcoma, whose name is derived from the pseudoalveolar appearance of its histology. In this report, the FDG PET/CT findings of ASPS are described in a 17-year-old asthmatic female who presented with worsening respiratory symptoms and a pelvic mass. The staging PET showed heterogeneous intense incorporation of FDG within the mass and variable FDG incorporation within the multiple lung nodules. In concordance with other soft tissue sarcomas, PET/CT helped to confirm the anatomic origin of the ASPS, to direct its biopsy, and to assess the distribution of disease.
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PMID:FDG PET of alveolar soft part sarcoma. 2083 1

Alveolar soft part sarcoma is a rare soft-tissue neoplasm that accounts for <1% of all soft-tissue sarcomas. Most cases arise in the extremities and head and neck region of young adults. The common sites of metastasis are lung, brain, and bone. Cardiac metastasis is extremely rare, in particular. Here we present the diagnostic evaluation and successful surgical resection of such a cardiac tumor in a patient aged 50 years with a histology-confirmed diagnosis of metastatic alveolar soft part sarcoma.
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PMID:Giant metastatic alveolar soft part sarcoma in the left ventricle: appearance in echocardiography, magnetic resonance imaging, and histopathology. 2126 29


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