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

• Main Authors: Landon J. Kunzelman (Author), Shweta Agarwal (Author), Nathan Boyd (Author), Cory J. Broehm (Author)
• Format: Book
• Published: Hindawi Limited, 2022-01-01T00:00:00Z.
• Subjects: article
• Online Access: Connect to this object online.
• Notes: 2090-679X; 10.1155/2022/5687190
• Keywords: Pathology; RB1-214
• Source: Case Reports in Pathology, Vol 2022 (2022)

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.