J.Jpn. Surg. Soc.. 88(9): 1149-1152, 1987

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Report on the annual meeting

TREATMENT OF MULTIPLE ENDOCRINE NEOPLASIA TYPE 2

In the treatment of patients with multiple endocrine neoplasia type 2 (MEN 2), we should take into account the following two risks. Firstly, the risk of recurrence of the tumor if we leave some apparently "normal" tissue unresected because the bilateral multicentric occurrence of both medullary thyroid carcinoma (MTC) and pheochromocytoma (PH) is one of the characteristics of MEN 2. Secondly, the risk of failure in postoperative hormone replacement therapy if total extirpation of the involved endocrine organ is performed.
Considering the 2 risks mentioned above, total thyroidectomy is recommended for MTC in MEN 2 patient.
For the PH in MEN 2 patient, we recommend more conservative treatment policy because the replacement therapy after bilateral total adrenalectomy is more troublesome and PH in MEN 2 is usually benign. Namely, the time and the extent of adrenal surgery for MEN 2 patients should be decided individually by considering the patient's age, sex and the size and endocrine activity of PH.
There are still some debates on the time of initiation of screening test for the children in high-risk kindreds, and also the time of operation for those children found by the screening to have very early MTC. We believe that it is early enough to start the screening at 12 years of age or so.

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