[Parathyroid glands involvement in multiple endocrine neoplasia].Rev Med Chir Soc Med Nat Iasi. 2009 Apr-Jun; 113(2):482-96.RM
Multiple endocrine neoplasias are syndromes characterized by the involvement of at least two endocrine glands. Parathyroid gland involvement is usually noted in Multiple Endocrine Neoplasia (MEN) type I and type II. Parathyroid glands tumor associated with endocrine pancreatic tumor, as well as pituitary tumors is the typical pattern of MEN I. The parathyroid gland is the most frequent abnormality in MEN I.
We presented five cases with MEN I and parathyroid glands involvement. In three cases with young ages (28-33 years old) and familial setting, the MEN I syndrome was "complete" (parathyroid adenoma, gastrinoma or insulinoma and pituitary adenoma--prolactinoma or GH-secreting tumors), and, in the other two cases, with 57 and 68 years old respectively, the MEN I syndrome was "incomplete" with parathyroid glands and pituitary gland involvement. The cases with gastrinomas were operated in emergency for complication of peptic ulcer (perforation associated with peritonitis and gastro-intestinal bleeding); then the pancreatic tumors were diagnosed and left pancreatectomy with spleen preservation and respectively, tumor resection have been performed. Hyperparathyrodism was then diagnosed and subtotal parathyroidectomy has been performed in both cases. Unfortunately one patients died due to severe endocrine disorder. During the necropsy, the pituitary adenoma has been diagnosed. The third case with "complete" MEN was a women of 33 years old, admitted for severe hypoglycemia. The imagistic and laboratory test diagnosed a tumor situated into the pancreatic body, and an parathyroid adenoma. The resection of pancreatic tumor associated with resection of the parathyroid adenoma, in the same time, were performed. The other two cases with "incomplete" MEN were older then the first patients, and were diagnosed with hyperparathyrodism and pituitary gland tumor. The resection of parathyroid gland adenoma has been performed in both cases, with uneventful postoperative course. The literature data was also discussed.
The parathyroid glands involvement in MEN is common. There are two kinds of MEN associated with parathyroid gland involvement: the "complete" form, especially in young patients, with diffuse involvement of the parathyroid glands, and the subtotal parathyroidy is the best choice, and the "incomplete" form, especially in elderly, with the involvement of a single parathyroid gland; in this way, the resection of the adenoma associated with biopsy from the other parathyroid gland is the best approach. The presence of gastrinomas, complicate the disease prognosis and the surgical approach. The treatment of these patients is challenging and has to be done in multidisciplinary team.