Epithelioid Hemangioendothelioma with WWTR1-CAMTA1 Fusion in the Parotid Gland Presenting as Bell's Palsy

Landon J Kunzelman; Shweta Agarwal; Nathan Boyd; Cory J Broehm

doi:10.1155/2022/5687190

Epithelioid Hemangioendothelioma with WWTR1-CAMTA1 Fusion in the Parotid Gland Presenting as Bell's Palsy

Case Rep Pathol. 2022 Jun 24:2022:5687190. doi: 10.1155/2022/5687190. eCollection 2022.

Authors

Landon J Kunzelman¹, Shweta Agarwal¹, Nathan Boyd², Cory J Broehm¹

Affiliations

¹ University of New Mexico, Department of Pathology, MSC08 4640, Reginald Heber Fitz Hall, Room 335, 1 University of New Mexico, Albuquerque, NM 87131, USA.
² University of New Mexico, Department of Surgery-Division of Otolaryngology-Head and Neck Surgery, MSC10 5610, 1 University of New Mexico, Albuquerque, NM 87131, USA.

Abstract

Epithelioid hemangioendothelioma is a rare tumor of endothelial differentiation most commonly arising in soft tissue, liver, and lung, following a variable clinical course. Most cases are characterized by a t(1;3)(p36;q23-25) resulting in WWTR1-CAMTA1 fusion. Only five epithelioid hemangioendothelioma have been previously reported arising in the salivary glands. None have presented as Bell's palsy. In the current case, a 37-year-old female presented with a longstanding complaint of pain and fullness in the right preauricular region and progressive episodes of Bell's palsy and facial nerve weakness. Surgical resection showed a tumor comprised of atypical cells with occasional intracytoplasmic vacuoles in a fibromyxoid stroma. Immunohistochemical stains demonstrated the neoplastic cells expressed ERG, CD31, and CD34, confirming vascular differentiation. Fluorescence in situ hybridization revealed a t(1;3)(p36;q25), confirming a diagnosis of epithelioid hemangioendothelioma. At 12-month follow-up, the patient has no evidence of disease.

Publication types

Case Reports