Category: Pituitary Disorders/Neuroendocrinology
MEN-1 syndrome is associated with a germline mutation and classically characterized by tumors of the parathyroid glands, endocrine pancreas, pituitary gland and associated with other neuroendocrine tumors. The mutated MEN1 tumor suppressor gene on chromosome 11 predisposes individuals to these tumors, however expression between families is widely variable with dozens of other malignancies having been identified. The management of these individuals generally consists of treatment of hormone excess and surgical resection. Numerous non-endocrine tumors have been associated with MEN-1 and prior to an aggressive approach we must consider the morbidity of treatment.
A 71 year-old man presented for surveillance of his multiple endocrinopathies. Patient had a history of primary hyperparathyroidism, metastatic gastrinoma, non-functional pituitary adenoma and bronchial carcinoid tumor. He had confirmed genetic testing for MEN1 syndrome with both his mother and son suffering from a similar disease process.
His treatment had required aggressive surgical therapy for over three decades. Hyperparathyroidism had required 3.5 gland parathyroidectomy, pituitary neoplasm required two trans-sphenoidal resections, with a residual 1.5 cm tumor in the cavernous sinus persistently threatening compromise of the optic nerve. Bronchial carcinoid tumor was treated with a thoracotomy and metastatic gastrinoma was treated with high dose PPI. Given his persistent vision symptoms, he underwent periodic imaging, which revealed an incidental 2.3 x 1.4 x 2.8 cm mass of a minor salivary gland.
Given the aggressive treatment for his other neoplasms and his lifespan of over 30 years from diagnosis of MEN1, the patient opted for surgical resection.
Surgical pathology revealed a 2.4 x 1.9 x 1.0 cm tumor consistent with a pleomorphic adenoma with no features of a high grade malignancy.
Discussion : The multiple neoplasms associated with MEN-1 syndrome vary significantly between individuals. Salivary neoplasms are not typically associated and in our case this was found to be a lower grade malignancy.
Given the treatment burden in these individuals, identifying low grade malignancies could favor surveillance strategies and significantly improve long term morbidity.
University of Florida
University of Florida