A Rare Malignancy of the Eyelid: Report A Case of Primary Periocular Histiocytoid Carcinoma

Abstract

Primary periocular histiocytoid carcinoma is a very rare malignant tumor. Until now, less than 50 cases have been reported in the English literature. It is characterized by resistant epiphora, limitation in extraocular motility, and ptosis. The definitive diagnosis of this lesion is made based on detecting histological histiocytoid features along with tracing positivity of specific biomarkers using immunohistochemistry. However, pathologists may be faced with two major obstacles in the diagnosis of this tumor including distinguishing it from metastatic histiocytoid lesions and also from benign mimics such as reactive inflammatory lesions. Here, we describe a case of primary periocular histiocytoid carcinoma located on the eyelid as well as review the literature to clarify the histopathological and diagnostic features of this tumor.

Keywords: Eyelid malignancy; Histiocytoid carcinoma; Orbital malignancy; PPHC; Periorbital malignancy.

Fig. 1

A, B. H&E-stained slide showing infiltrating isolated and small aggregates of cells with mild nuclear atypia and foamy vacuolated cytoplasm (original magnification *40)

Fig. 2

Immunohistochemical studies reveal: A. Positive staining for AE1/AE3. B. Positive staining for CK7. C. Negative staining for CK20. D. Positive staining for GATA3 and E. Positive staining for GCDEP15