Alveolar Soft Part Sarcoma: A Rare Diagnosis
A 32-years-old female from a remote village presented with multiple painless soft globular swellings all over the body including face, tongue, neck, chest, abdomen, back, genitalia, and all the limbs for a duration of 3 months [Figures and ]. Most of the lesions were skin-colored, but few of the lesions over forehead, neck, back, and genitalia showed reddish to bluish discoloration while few showed crusting over the surface. The patient gave history of similar type of lesion in right thigh 3 years back, for which she underwent surgical excision, but no histopathological examination, was done at that time. There was associated history of anorexia, weight loss, and fatigue. On examination, apart from the aforesaid lesions, a nodule was palpable in the posterior fornix of vagina and another in the anterior rectal wall 6 cm from anal verge. Other systems were within normal limit. After thorough investigations, all the blood parameters were found to be normal, except moderate degree of anemia. Fortunately, her chest X-ray, USG of whole abdomen, CT scan of brain revealed no abnormality. The most symptomatic swelling which were present over right thigh (at the site of previous excision) and back were excised followed by biopsy. Histopathology showed large polygonal cells with eccentrically-placed prominent nucleus and abundant eosinophilic cytoplasm arranged in an alveolar pattern separated by fibrous septae and occasional mitotic figures []. Cytopathology revealed the tumors cells expressing vimentin. Following surgery, the patient was referred to radiotherapy department and was put on chemotherapy regimen comprising of vincristine (2 mg), adriamycin (60 mg), and cyclophosphamide (90 mg), given at an interval of 3 weeks. She received 6 such cycles and is under close follow-up. There is mild reduction of the lesions with moderate symptomatic improvement and without any features of metastasis.