SUN-060 - Parotid Gland Tumor as A Non-Endocrine Manifestation of Multiple Endocrine Neoplasia Type 1 (MEN1) Syndrome: A Case Report

Background: MEN1 is a disease that results from an inherited mutation in the MEN1 gene that results in tumor formation in the pituitary gland, parathyroid glands, and pancreas. Development of non-endocrine tumors such as cutaneous, smooth muscle, and central nervous system tumors have been reported; however, only one prior case of a salivary gland tumor associated with MEN1 has been reported (1). We describe a second case.

Clinical

Case: A 65 year old woman with hypertension and nephrolithiasis presented with polydipsia and nephrostomy tube leakage. Upon presentation, labs showed elevated calcium (12.9 mg/dL, n < 10.2 mg/dL) and ionized calcium of 1.5 mmol/L (n < 1.40 mmol/L). Imaging incidentally revealed a 2.1 cm hyperenhancing lesion in the pancreatic body suggestive of a neuroendocrine tumor (NET). She was admitted for further work-up of hypercalcemia and received fluids, calcitonin, and zoledronic acid. Labs revealed elevated PTH levels (349.6 pg/mL, n < 65 pg/mL) and low parathyroid hormone-related protein ( < 2.0 pmol/L, n < 2.0 pmol/L) indicating primary hyperparathyroidism. Neck ultrasound showed multiple thyroid nodules and a left parathyroid adenoma. A 4D CT parathyroid confirmed two parathyroid adenomas, a multinodular goiter, and a 2 cm enhancing mass in the left parotid gland, suggestive of a primary salivary tumor. The patient underwent subtotal parathyroidectomy with autotransplantation. Postoperatively, she was started on calcium and vitamin D supplementation. Given concerns for MEN1, a gallium 68 DOTATATE scan was performed which showed a hypermetabolic nodule in the pancreatic mass, uptake in the left inferior parotid gland, and asymmetric enlargement of the left thyroid gland. Endoscopic ultrasound with biopsy confirmed the presence of a pancreatic NET. The patient was discharged with follow-up for thyroid ultrasound, pituitary MRI, otolaryngology for biopsy of the parotid mass, and hepatobiliary surgery for the pancreatic NET. She later underwent distal pancreatectomy and splenectomy, revealing two Grade 1 NETs. Biopsy of the parotid gland mass is pending at the time of reporting.

Conclusion: Although salivary gland tumors are not classically associated with MEN1, this case, together with a previously published case of pleomorphic adenoma, suggests a broader tumor spectrum. Further studies are needed to evaluate the incidence of salivary gland tumors in patients with MEN1, as well as their malignant potential, to guide urgency of management while not overburdening a population that already must undergo multiple surgical interventions.

Reference: (1) Srihari A, M.D., Danan D, M.D. SALIVARY GLAND NEOPLASM ASSOCIATED WITH MEN-1 SYNDROME. Endocrine Practice, suppl.SUPPLEMENT 1. 2019;25:255-256.

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