Alveolar soft part sarcoma of tongue base — A rare presentation of a rare tumor

Abstract

Alveolar soft part sarcoma is a rare, aggressive malignancy of uncertain histologic origin and enigmatic clinical behaviour. It has a characteristic histopathological picture, with a propensity for vascular invasion and distant metastasis. We report a case of alveolar soft part sarcoma involving the tongue base in an adolescent female. She underwent laser assisted excision of the tongue base tumour followed by post-operative radiotherapy. The clinical presentation, histopathological picture, immunohistochemical & cytogenetic studies, radio-imaging, management protocols and prognosis of this tumor have been discussed.

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.…”

“…2,[20][21][22]25,27 SMA was found to be positive in 18 cases (85.71%) 9,14,20,23,26,27 and negative in 3 cases (14.28%). 12,13,25 Desmin (d-33) expression was positive in 18 cases (85.71%), 2,9,11,12,14,16,17,19,22,26,28 while negative in 3 (14.28%). 13,18,20 From the seven cases, five (71.42%) cases showed positivity (+) for MYOD1, 14,22,23,25,28,29 one (14.28%) case demonstrated strong positivity (++), while the remaining one (14.28%) showed negative expression.…”

Alveolar Soft Part Sarcoma of the Tongue: A Rare Tumor at an Unusual Location

“…Pre-operative staging proves vital due to the frequency of metastases at presentation and the relatively poor prognosis of ASPS. Long-term post-operative follow-up is mandatory for all patients with this deceptively indolent and highly malignant neoplasm [ 20 ]. As noted earlier, if the team decides on surgery with curative intent, the case should be extensively worked up for metastatic disease.…”

An atypical presentation of alveolar soft part sarcoma of tongue

“…[ 4 ] Twenty-five percent cases demonstrate NSE or S-100 positivity. [ 5 ] CK, epithelial membrane antigen, and neurofilament have been consistently negative. ASPS is usually slow growing and deceptively appears histologically benign with no atypical mitosis or nuclear pleomorphism.…”