J.Jpn. Surg. Soc.
ISSN 1880-1129

[Abstract] [Full Text PDF] （in Japanese / 4454KB） [Members Only And Two Factor Auth.]

J.Jpn. Surg. Soc.. 88(8): 1017-1023, 1987

Case report

AN OPERATED CASE OF MULTIPLE ENDOCRINE NEOPLASIA TYPE IIb AND REVIEW OF THE JAPANESE LITERATURES

1) First Department of Surgery, Teikyo University School of Medicine, Tokyo, Japan
2) Ito Hospital, Tokyo, Japan
3) Departments of Pathology, School of Medicine, Keio University, Tokyo, Japan
4) Departments of Surgery, School of Medicine, Keio University, Tokyo, Japan

Hiroshi Takami1), Osamu Ozaki2), Kunihiko Ito2), Jun-ichi Shikata1), Yasuhiro Hosoda3), Takashi Mimura4), Osahiko Abe4)

Multiple endocrine neoplasia, type IIb (MEN IIb) is a rare syndrome characterized by the occurrence of medullary thyroid carcinoma (MTC), pheochromocytoma and mucosal neuroma. A 35-year-old male patient with MEM IIb having megacolon, marfanoid habitus and no family history of the disease underwent surgery. Because MTC was present in both lobes, total thyroidectomy and modified neck dissection were performed. Pheochromocytoma was found bilaterally and bilateral adrenalectomy with adrenal autotransplantation in the rectus abdominis muscle was carried out. Postoperative course was satisfactory except for transient hypocalcernia and mild ileus. After the slow corticosteroid weaning process, his adrenocortical function was at the lower level within a normal range. In August 1986 (24 postoperative months), he was maintained by the administration of 10mg of hydrocortisone every three days, and calcitonin and CEA levels in sera were normal. We collected 15 cases reported in Japanese literatures. MTC and mucosal neuroma were found in all cases, whereas pheochromocytoma was present in g cases. Bilateral and multicentric occurrences were usual, and total thyroidectomy and bilateral adrenalectomy were, warranted. We believe that autotransplantation following bilateral adrenalectomy is a worthy alternative.

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