Mucoepidermoid Carcinoma of Parotid Gland in Multiple Endocrine Neoplasia Type 1

Presentation Number: LBS-062
Date of Presentation: March 7th, 2015

Sira Korpaisarn*, Hugo Akabane and Vineetha Jose
MetroWest Medical Center, FRAMINGHAM, MA

Abstract

Introduction
           MEN type 1 is an autosomal dominant syndrome, which generally involves parathyroid glands, anterior pituitary gland and entero-pancreatic endocrine cells. Particular nonendocrine tumors are common manifestations including angiofibromas, collaginomas and lipomas. We hereby report a case of mucoepidermoid carcinoma of parotid gland, a nonendocrine tumor, in MEN type 1 patient who is eight-year postradiation therapy for pituitary macroadenoma.

Clinical case
           A 39-year-old male, with history remarkable for multiple endocrine neoplasia type 1 diagnosed at age of 17, was accidentally found with mass in left parotid gland from MRI head. The patient was completely asymptomatic. There was neither palpable parotid mass nor facial nerve defect. His MEN type 1 was continually active during the past couple years. He has history of pituitary macroadenoma, parathyroid adenomas, gastrinoma, mestastatic carcinoid tumor to liver, bilateral adrenocortical adenomas and meningioma. He underwent multiple operations including pituitary adenoma resection followed by radiation therapy eight years ago, parathyroidectomy and partial gastrectomy for gastrinoma. MRI of head was done due to a complaint of headache and it revealed a 2.6 x 2.3 x 2.9 cm lobulated mass in deep segment of the left parotid gland. Both parotid glands are otherwise unremarkable. No pathologically enlarged lymph nodes were seen. The tumor was finally resected followed by radiation therapy. The pathological study showed intermediate grade mucoepidermoid carcinoma of parotid gland, which is nonendocrine tumor.

Discussion
           Mucoepidermoid carcinoma is malignant epithelial tumor of salivary glands. Thus far, there is no published report regarding relationship between salivary gland tumor and MEN type 1 yet. Although the interval is too short, the only risk factor in this patient is a radiation of pituitary macroadenoma, which was done eight years ago. This clinical vignette raises the awareness of possible sequelae of head and neck radiation, which should be attended along with the main disease.

 

Nothing to Disclose: SK, HA, VJ