Alveolar Soft-Part Sarcoma: A Case Report: Mexico Hospital,San Jose, Costa Rica
Journal Title: Biomedical Journal of Scientific & Technical Research (BJSTR) - Year 2017, Vol 1, Issue 3
Abstract
Alveolar soft-part sarcoma is a rare malignant entity with poor prognosis. Chemotherapy has limited activity in this type of sarcoma, we describe here the outcomes of the first case documented in our Institution with metastatic disease, treated exclusively with sunitinib.Soft-tissue sarcomas (STS) constitute an uncommon and heterogeneous group of tumors of mesenchymal origin, they share several clinical and pathological features and are usually considered as a group for diagnostic and therapeutic purposes, with the exception of specific particularities of some subtypes [1]. Alveolar soft‐part sarcoma (ASPS) is a rare, distinctive sarcoma, typically occurring in young patients. Although it displays a relatively indolent clinical course, the ultimate prognosis is poor and is often characterized by late metastases [2]. In advanced cases, with multiple metastatic involvement and recurrent cases, only palliative treatment is preferred due to its bad prognosis [3].We describe our experience treating a patients with ASPS with metastatic disease using tyrosine kinase inhibitor (sunitinib) with an outstanding survival and quality of lifeThis 23-year-old man with unremarkable past medical history was seen in our clinic with a history of 2 years of increasing rectal pain associated with swelling in his right leg, and subsequently rectal bleeding with mild diarrhea. On physical examination he was found to have a very important skin in duration besides the anus, and at the digital rectal examination a 5cm mass was found at the anal verge.A Thorax-Abdomen and Pelvis CT Scan was performed, and showed: Many pulmonary nodules distributed diffusely with diameters up to 25 mm, compatible with metastatic disease. And also was reported a solid, heterogeneous pelvic mass with well defined borders, approximately 75 mm in maximum diameter, with gas present inside suggesting a fistulous process, with infiltration of the anal canal, as well as the anus elevator muscle in its left portion. Colonoscopy showed an indurated lesion in the perianal region. A biopsy of the lesion was taken in the operating room. The biopsy showed a malignant neoplasm with well defined nests of cells separated by fibrous stroma, composed of large polygonal cells with prominent nucleoli, with alveolar patter tumor cells. Overall features were suggestive of ASPS. Many of the cells were periodic acid Schiff (PAS)–positive. The cells were negative for pancytokeratin, chromogranin, synaptophysin, PLAP, EMA, myoglobin, myogenin, caldesmon, S-100, melan A, CD34, Factor VIII y LCA. The cells were strongly positive for desmin and actin (focally) (Figures 1 & 2).
Authors and Affiliations
Rojas-Vigott Raquel, Landaverde Denis, Rivera-Delgado Vanessa
Keywords
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- EP ID EP569601
- DOI 10.26717/BJSTR.2017.01.000257
- Views 188
- Downloads 0
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