Alveolar soft part sarcoma (ASPS) is a rare malignant neoplasm originating from soft tissue. It was initially described as a distinctive clinical entity by Christopherson et al. in 1952. The peak age of incidence is between 15 and 35 years with slight sex predominance among women. The most common sites involved include extremities and trunk and in young adults and head and neck in children. ASPS is a slow-growing tumor with unusual patterns of metastasis which runs a poor prognosis. The aim of this article was to present a case of ASPS of retroperitoneal origin along with its diagnostic and therapeutic workups.
Case Report: A 31-year-old patients with a chief complain of an abdominal mass in right lower quadrant, underwent surgical resection as a neuroendocrine tumor according to pre-operative imaging studies. Pathology reported the lesion as ASPS. The patient refused adjuvant chemotherapy and unfortunately he did not show up to continue his treatment and further follow-ups. A careful investigation would be required including clinical findings, clinicopathological correlation, with appropriate radiological studies, before definitive treatment of ASPS.
The main problem to gain an extended insight into clinical features and optimal treatment is the rarity of the disease. Given the ineffectiveness of current treatments in advanced ASPS, further future investigation to find new therapeutic options would be required.