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.

Keywords: Angiosarcoma; Eye; Eyelid; Hemangioendothelioma; Hobnail hemangioendothelioma; Retiform hemangioendothelioma; Tumor.

Fig. 1

Clinical presentation. a External photograph demonstrates diffuse edema and mild erythema involving the right upper eyelid and superior orbit, associated with eyelid ptosis. b Axial post-contrast computed tomography scan shows a diffuse, poorly circumscribed preseptal and periorbital swelling, without a distinct mass lesion.

Fig. 2

Pathologic findings, initial excision. a Infiltrative neoplasm, composed of well-formed channels with features of vascular and lymphatic endothelium, involves the eyelid dermis. b The elongated channels have branching and anastomosing arrangement, reminiscent of rete testis, and are surrounded by hyalinized collagen and prominent inflammatory infiltrate. c The channels are lined by the uniform ovoid cells that protrude into the lumen in a hobnail fashion. d Large papillary tufts with central hyalinized cores extend into the lumen of the channel, which contains few lymphocytes and material reminiscent of lymph. e Cellular aggregates of cells with slightly larger and more pleomorphic nuclei, prominent nucleoli, and mitotic activity (arrow) permeate the collagen bundles. f Perineural invasion is identified. g The neoplastic cells immunoreact with vascular and lymphatic endothelial marker ERG. h There is diffuse immunoreactivity of the tumor for lymphatic endothelial marker D2–40. Stains: H&E (a-f), ERG (g), and D2–40 (h); original magnification ×10 (a), ×20 (b, h), ×50 (f, g), ×100 (c–e).

Fig. 3

Pathologic findings, re-excision. a Prominent multilayering of hyperchromatic cells, which dissect collagen bundles without forming well-defined vascular channels, is present. b Frozen section margin demonstrates inconspicuous foci of malignant cells (arrows), morphologically similar to the adjacent lymphocytic infiltrate. Stain: H&E; original magnification ×100.