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Chirurgia 2000 February;13(1):11-8

language: Italian

Controversies in surgery of MEN 2A syndrome

Zurleni F., Ballabio A., Rossetti O., Palmieri B., Libroia A., Verga U., Gelli D., Forti D.


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Background. Recently the surgical treatment for medullary thyroid carcinoma, hyperparathyroidism and pheochromocytoma in multiple endocrine neoplasia type 2A (MEN 2A) has been discussed. Personal experience in 14 patients affected by this syndrome is reported, emphasizing the major problems related.
Methods. All patients were affected by thyroid lesions, treated by total thyroidectomy: in the first group of 7 patients a selective lymphadenectomy was associated. Postoperative plasma calcitonin resulted normal in 1 patient. In the second group of 7 patients (included 2 hyperplasia) a systematic lymphadenectomy was performed and postoperative plasma calcitonin levels were normal in 5 of them. Out of 14 patients, 6 affected by hyperparathyroidism were treated by total parathyroidectomy with autograft or subtotal procedure: 5 patients recovered (1 case of postoperative hypoparathyroidism), 1 patient remains persistent. Concerning the 8 patients not affected by hyperparathyroidism, 6 underwent biopsy of one parathyroid gland, while 2 underwent total parathyroidectomy with autograft and cryopreservation during total thyroidectomy with systematic lymphadenectomy. Out of 14 patients, 6 were affected by pheochromocytoma and required 3 bilateral adrenalectomy and 3 monolateral procedure, recently performed by laparoscopy.
Results. The follow-up refers to 14 surgical patients for an average of 9.3 years: all patients are still alive and long-term metastases have not been observed.
Conclusions. On the basis of personal experience and literature review, some difficult aspects in the treatment for men 2A, requiring and adaptable approach are underlined: surgical treatment of hyperparathyroidism and ³prophylactic² therapy of thyroid and parathyroid lesions, when genetic diagnosis is made. Finally the effectiveness of laparoscopic adrenalectomy for pheochromocytoma is confirmed.

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