Dual unifocal primary Epithelioid Angiosarcoma: A case report and review of the literature unveiling a rare genre of neoplasia

Abstract

Introduction and importance: Angiosarcomas are an exceedingly rare and malignant form of soft tissue sarcoma that are derived from endothelial cells. Overall, they comprise <1 % of the total number of soft tissue sarcomas. Due to nonspecific and misleading symptoms, the subsequent clinical presentations can easily result in misdiagnosis. This leads to life-threatening complications for patients. Contemplating this tumor as a differential diagnosis during the preoperative phase allows for essential time-sensitive therapeutic interventions to be accomplished.

Case presentation: Herein, we present the seldom precedented case of a 66-year-old Middle Eastern male who came to our surgical clinic chiefly complaining of an exacerbation of chronic left hypochondriac pain accompanied by gradual inexplicable abdominal distention. Our diagnostic radiological evaluation demonstrated two isolated abdominal mass formations.

Clinical discussion: Sheer excision of the neoplastic masses with safety margins was successfully executed via open surgery. The stemming histopathological examination through Hematoxylin and Eosin and immunohistochemical staining established the definitive diagnosis of an Epithelioid Angiosarcoma.

Conclusion: Epithelioid Angiosarcomas belong to the category of profoundly rare tumors. The available published literature conveys this rarity through the scarcity of epidemiological parameters and studies. It necessitates being borne in mind when facing similar clinical scenarios so that apt therapeutic interventions can be achieved. Structured diagnostic methods, timely surgical interventions and proper techniques, and comprehensive follow-up patient surveillance protocols are, therefore, merited. After thorough review of the published literature, we reckon herewith that ours is the first documented case from our country of an Epithelioid Angiosarcoma.

Keywords: Abdominal Surgery; Angiosarcoma; Case Report; Epithelioid Angiosarcoma; Surgical Oncology; Vascular Neoplasia.