Rare Case Report of Alveolar Soft Part Sarcoma of the Orbit

Abstract

Alveolar soft part sarcoma (ASPS) is a rare but distinct soft tissue tumor with unique histopathological and electron microscopic features. Orbital involvement is rare with only few reports published in the literature. ASPS have an indolent clinical course, but it is known to metastasize. Primary modality of treatment is surgery followed by adjuvant treatment. This case is a unique presentation with orbital mass with on and off bleeding. This is the largest orbital ASPS for which orbital exenteration was performed.

Role of pre-operative percutaneous embolization in orbital alveolar soft part sarcoma – An experience from a tertiary eye-care center

“…There were 39 males and 45 females. [ 1 2 5 7 8 9 ] Our series showed a male: female ratio of 4:1, contrary to that reported in a majority of the studies. The most common signs were reduced visual acuity, an abaxial proptosis, and restricted extraocular movements.…”

“…Alveolar soft-part sarcoma (ASPS) is a rare tumor that accounts for approximately 0.5–1% of all soft-tissue sarcomas. [ 1 2 ] It was first described in 1952 by Christopherson et al . [ 3 ] in a series of 12 cases that shared a common histological pattern.…”

“…A recent trend toward conservative globe salvage surgery has been reported in 47 (63.5%) out of 74 cases in the literature. [ 1 2 5 7 8 9 ]…”

Alveolar soft part sarcoma of the retroperitoneum: A case report

“…In 2002 WHO soft tissue tumor classification, Alveolar soft part sarcoma (ASPS) was included in the category of "Tumor of uncertain differentiation" [1]. ASPS is a rare but discrete soft tissue tumour accounting for 0.5-1% of softtissue sarcomas [2].…”

“…The tumor typically has intermediate signal intensity-mildly higher than that of muscle-on T1-weighted images and heterogeneous high signal intensity on T2-weighted images, with multiple flow voids within and around it. The intermediate or mildly high T1 signal intensity is perhaps due to its rich vascularity and intratumoral bleeding, in addition to the slow flowing blood within the tumor [1]. There is a strong and uniform enhancement with IV contrast medium.…”