Atypical Fibroxanthoma of the Bulbar Conjunctiva: A Unique Case Describing the Pathology, Clinical Presentation, and Management

Abstract

Background/aims: Atypical fibroxanthoma is an uncommon tumor that usually occurs in the skin of the head and neck of the elderly with significant sun exposure. We describe a unique case featuring a rare ocular surface conjunctival tumor (atypical fibroxanthoma) and provide insight on its characteristic clinical features, surgical management, and histology.

Methods: A 71-year-old male fisherman with no pertinent ocular history presented to an academic center with a rapidly enlarging bulbar conjunctival mass in the right perilimbal region for the past several months. The patient underwent surgical excisional biopsy with cryotherapy, adjuvant alcohol, and amniotic membrane transplantation.

Results: Pathology specimen illustrated an atypical spindle cell tumor with inflammatory cells, increased mitotic activity, cytologic atypia, and positive diffuse staining with CD163 and CD10 consistent with an atypical fibroxanthoma.

Conclusion: Atypical fibroxanthoma is an extremely rare ocular surface tumor that may simulate conjunctival or ocular surface squamous neoplasia. While this lesion typically pursues a benign clinical course, it may recur or rarely metastasize. Thus it should be treated aggressively with excisional biopsy, cryotherapy, absolute alcohol, and/or amniotic membrane transplantation.

Keywords: Biopsy; Conjunctiva; Fibroxanthoma; Immunohistochemistry; Pathology; Tumor.

Fig. 1

a Preoperative photo demonstrating raised vascular lesion extending from the bulbar conjunctiva over the limbus. b Postoperative photo following excisional biopsy of the lesion.

Fig. 2

a Low-power photomicrograph showing a “pyogenic granuloma”-like architecture with elevated well-circumscribed mass showing an epithelial collar and a partially ulcerated surface. Hematoxylin and eosin, 40× magnification. b High-power photomicrograph demonstrating a spindle cell population of atypical cells, some with very bizarre nuclei with multinucleation. Mitoses are present. Hematoxylin and eosin, 400× magnification.

Fig. 3

Immunohistochemical profile demonstrating diffusely positive staining for CD163 (a) and CD10 (b); faint or focal positivity for calponin (c), CD99 (d), and SMA (e); and negative staining for SOX10 (f) and AE1/3 (g).