BACKGROUND: Alveolar soft part sarcoma is a rare soft tissue tumor of uncertain origin that is generally slow growing but unmistakably malignant due to its propensity for metastasis to lung, bone and brain early in the course of disease. Fine needle aspiration biopsy (FNAB) of these tumors and recognition of the characteristic cytologic features precludes more invasive diagnostic measures and facilitates appropriate treatment. CASE: A 54-year-old African-American man presented to our institution with a 2-week history of left leg pain. Imaging studies revealed a left leg soft tissue mass just below the popliteal fossa and multiple bilateral lung lesions suggestive of metastatic neoplasm. FNAB of the left lower extremity mass yielded uniform clusters of cells and sigle cells with large nuclei and single prominent nucleoli. Histologically, the biopsy showed nests of large polygonal cells with abundant eosinophilic cytoplasm, round regular nuclei and prominent nucleoli. A periodic acid-Schiff (PAS) stain highlighted intracytoplasmic rhomboidal crystals, a feature diagnostic of alveolar soft part sarcoma. CONCLUSION: Alveolar soft part sarcoma may be diagnosed by its unique morphologic characteristics and should be considered in the differential diagnosis of all cytologically sampled soft tissue lesions.
BACKGROUND:Alveolar soft part sarcoma is a rare soft tissue tumor of uncertain origin that is generally slow growing but unmistakably malignant due to its propensity for metastasis to lung, bone and brain early in the course of disease. Fine needle aspiration biopsy (FNAB) of these tumors and recognition of the characteristic cytologic features precludes more invasive diagnostic measures and facilitates appropriate treatment. CASE: A 54-year-old African-American man presented to our institution with a 2-week history of left leg pain. Imaging studies revealed a left leg soft tissue mass just below the popliteal fossa and multiple bilateral lung lesions suggestive of metastatic neoplasm. FNAB of the left lower extremity mass yielded uniform clusters of cells and sigle cells with large nuclei and single prominent nucleoli. Histologically, the biopsy showed nests of large polygonal cells with abundant eosinophilic cytoplasm, round regular nuclei and prominent nucleoli. A periodic acid-Schiff (PAS) stain highlighted intracytoplasmic rhomboidal crystals, a feature diagnostic of alveolar soft part sarcoma. CONCLUSION:Alveolar soft part sarcoma may be diagnosed by its unique morphologic characteristics and should be considered in the differential diagnosis of all cytologically sampled soft tissue lesions.