Orbital alveolar soft part sarcoma: Histopathologic report of two cases

Abstract

Alveolar soft part sarcoma is considered as a distinct histopathological entity with rare cases reported from the orbit area. Two cases of alveolar soft part sarcomas occurring in the orbit of two patients along with their histopathologic findings are reported herewith. In both cases, the patients presented with eyelid swelling and proptosis. The diagnosis was made by incisional biopsies and histopathology. The literature is reviewed regarding occurrence of this tumor, its diagnosis and management.

Alveolar soft part sarcoma of the orbit, a case report of a rare tumor

“…Orbital cases manifest as a slowly growing mass resulting in proptosis (Kim et al, 2013), conjunctival injection, globe displacement, ptosis, dilated episcleral vessels and decreased vision. It may also present with epibulbar mass or eyelid swelling (Morris et al, 2005;Alkatan et al, 2010).…”

“…Macroscopically the lesions are usually well circumscribed, encapsulated (Wang et al, 2015), red and vascularized (Alkatan et al, 2010); mostly located in the deep tissues, causing no specific symptoms for an extended period of time. Its late discovery is thought to be caused by its indolent growth (Wang et al, 2015).…”

“…Histologically the tumor is characterized by polygonal to round cells with abundant eosinophyllic cytoplasm (Kashyap et al, 2004), showing crystalline structures which are PASpositive diastase-resistant approximately in two thirds of the cases (Alkatan et al, 2010). In the remaining cases, only PAS-positive granules, which are likely precursors of the crystals, are present (Kashyap et al, 2004).…”

“…Alveolar soft part sarcoma (ASPS) is a clinically and morphologically distinct softtissue sarcoma, first defined and named by Christopherson et al (1952) (Kashyap et al, 2004;Alkatan et al 2010). It is a rare and histologically distinct soft tissue malignancy of uncertain histogenesis (Kanhere et al, 2005;Wang et al, 2015).…”

“…The tumor received its name due to its pseudo-alveolar histological appearance (Kayton et al, 2006). It accounts for approximately 0.5% -1% of all soft tissue sarcomas (Alkatan et al, 2010;Kim et al, 2013;Wang et al,2015;Jour et al, 2015). Most case, occur predominantly in the lower extremities, particularly the anterior portion of the thighs in adults, and in head and neck regions, especially the orbit and the tongue, in infants (Kashyap et al, 2004;Jour et al, 2015Wang et al, 2015.…”