Alveolar soft part sarcoma in childhood: A report of two cases and review of literature

Abstract

Background: Alveolar soft part sarcoma (ASPS) is a rare, distinctive soft tissue sarcoma, typically occurring in young people. In children, a substantial percentage of cases occur in the head and neck, often in the orbit or tongue. Although it displays a relatively indolent clinical course, the ultimate prognosis is poor with late metastases. Aims and Objectives: Review of literature to give an insight into the current knowledge about this enigmatic tumor, in terms of genetic and diagnostic aspects. Materials and Methods: Clinical, radiological, and morphological features of two cases, an 8-year-old female with tongue lesion and a 14-year-old female with orbital lesion are described. Immunohistochemistry (IHC) with a panel of antibodies was performed. Results: Clinical presentation and microscopic features were remarkable in two young females, one in the tongue and another in the orbit and similar to other case reports. The use of TFE3 for IHC established the diagnosis. Conclusion: Alveolar soft-part sarcoma remains an enigma clinically. ASPS should be considered in the differential diagnosis of pediatric head and neck masses with a solid morphology.

Clinico-pathological spectrum of alveolar soft part sarcoma: case series from a tertiary care cancer referral centre in india with a focus on unusual clinical and histological features

“…The dominant nuclear feature (>50% of tumor nuclei) were bland nuclei with marked nuclear folding leading to concave, apple bite, and crenated nuclei without any nucleoli in nearly 68.2% of the cases and these nuclei were focal in the rest of the patients. These features were first observed by Fanburg-Smith et al and Chatura et al in lingual ASPS but it was an universal finding in the present series, independent of site ( 12 , 16 ). We also observed focal nuclear grooves in 3 cases which are not documented in the literature.…”