Primary Cutaneous Angiosarcoma of the Eyelid: A Diagnostic and Therapeutic Challenge

Abstract

Primary cutaneous angiosarcoma is a rare vasoformative malignant neoplasm that can present a diagnostic and therapeutic challenge. We describe a 76-year-old Caucasian man with right upper eyelid swelling and nodularity, initially suspected clinically to represent either ocular adnexal lymphoma or basal cell carcinoma. Incisional biopsy and wide resection of the mass with frozen section control of margins were interpreted as compatible with hobnail (Dabska-retiform) hemangioendothelioma. Foci of atypia were noted in the tumor, raising speculation of evolution into a more aggressive neoplasm, such as conventional angiosarcoma. The patient subsequently underwent two additional wide resections with frozen section control of margins in an attempt to obtain complete excision of residual tumor, which demonstrated histopathologic features favoring angiosarcoma. The histologic material from the original and subsequent resections was sent in consultation to several soft tissue pathology experts and the final diagnosis of low-grade cutaneous angiosarcoma was established. Despite repeated surgical interventions, there was continued persistence of the tumor in the deep orbital tissues. Various management options, including adjuvant radiotherapy/chemotherapy with and without orbital exenteration, were discussed. The patient decided against further surgical intervention and is currently undergoing adjuvant radiotherapy/chemotherapy. This case illustrates the diagnostic and management difficulties of ocular adnexal angiosarcoma.

Recurrence of an Angiosarcoma in the Eyelid: A Case Report of a Rare Entity

“…In the management of angiosarcoma involving the periocular area, a variety of surgical approaches with either associated neoadjuvant radiotherapy, adjuvant chemotherapy, adjuvant radiation, or therapy with biologic agents are described [4,5].…”

“…The presentation of cutaneous angiosarcoma can vary widely, mimicking diverse benign and malignant tumors, inflammatory lesions, edema, and infections [4,5]. The differential diagnosis of angiosarcoma includes capillary hemangioma, epithelioid hemangioma, Kaposiʼs sarcoma, epithelioid hemangioblastoma, and hemangiopericytoma [5].…”

Experience with Orbital Tumors from a Tertiary Cancer Centre of North East India: A Pathology Perspective

“…Primary eyelid involvement is rare, with less than 30 cases reported in the literature. 27 The rarity of ocular adnexal angiosarcoma further contributes to the clinical diagnostic challenge, with about one-quarter of the lesions initially misdiagnosed as basal cell carcinoma or pyogenic granuloma. 28 The histomorphology can be variable, ranging from highly differentiated tumors resembling benign or intermediate grade vascular lesions such as hemangioma or hemangioendothelioma, to anaplastic ones difficult to distinguish from a PDC or other high grade sarcomas.…”

Angiosarcoma presenting as a diffuse facial edematous induration: a rare and atypical clinical feature

“…In terms of treatment, there is no standard and most data are based on retrospective studies or case reports [15]. As for cases listed in Table 1, chemotherapy and radiotherapy were usually chosen because of the extent of the lesions, although resection was chosen in two cases in which cutaneous manifestations were limited to part of the face [10,13]. In our case, a specimen from the relapsed erythematous plaque showed irregularly proliferating endothelial cells without formation of any vasculature.…”