Alveolar soft part sarcoma

Abstract

SUMMARYAlveolar soft part sarcoma (ASPS) is a rare form of soft tissue sarcoma and is most often seen in adolescents and young adults. Despite its unique histology and wellcharacterised genetic translocation, many questions remain regarding the pathogenesis and treatment of this tumour type. Surgical excision of the primary tumour and pulmonary metastases has resulted in prolonged survival of some patients while the benefit of adjuvant chemotherapy and/or radiotherapy has been disputed. A 35-year-old woman presented with a tumour in the right leg and right side of the mandible along with active metastasis to lungs and multiple skeletal sites. BACKGROUND

Alveolar soft-part sarcoma of the oral cavity: A review of literature

“…While in our analysis, we have found out an age range of 1.5-64 years with a mean of 18.25 ± 15.243 years. There were 31 (41.9%) males 9,10,15,16,23,25,[27][28][29][31][32][33][34][35][36] and 43 (58.1%) females 2,3,5,8,[11][12][13][14][16][17][18][19][20][21]24,26,27,[36][37][38][39][40][41][42][43][44][45][46] in the review and male-tofemale ratio of 1:1.4 with female predilection. Gender predilection in our review is in accordance with Wang et al 30 and male-to-female ratio is in contrast with that of Fanburg-Smith et al 16 According to Kim et al, 36 most of the patients were from the second decade with slender female predilection.…”

Primary alveolar soft part sarcoma of the chest wall: A case report

“…12 As in our patient, these metastases are found in approximately 20%-25% of patients at the time of diagnosis. 6 Except for the bone metastases, our patients had several lung and cerebral metastases. Our patient did not consult a doctor despite the evident swelling on his chest wall, and he presented with symptoms of cerebral and pulmonary metastases, a characteristic that is more common in individuals with tumors in the extremities rather than the chest wall.…”

“… 12 As in our patient, these metastases are found in approximately 20%–25% of patients at the time of diagnosis. 6 …”

“…The majority of investigations have reported a female preponderance in adult patients, while no such preference has been observed in adolescents. 6 Lieberman and colleagues studied the histopathological and clinical characteristics of 102 ASPS patients and found that the median age of patients at the time of diagnosis was 22 years, with a 1.5 to 1 female preponderance. 8 Although the precise etiology of ASPS is unknown, investigations suggest that genetic alterations probably play a significant role.…”

“…Other treatments, including as radiation therapy, chemotherapy, and biologic therapy, are less well recognized, and the evidence is still unclear. 6 Given the disease's rarity, comprehensive and detailed knowledge of its clinical course, pathophysiology, and effective therapy remains challenging. In this case report, we describe the accurate diagnosis and treatment of primary ASPS of the chest wall in a young man who presented with headache, nausea, and vomiting.…”