CURATIVE TREATMENT OF MEDULLARY THYROID CARCINOMA IN A PATIENT WITH de novo MULTIPLE ENDOCRINE NEOPLASIA TYPE 2B

• Published in: Nihon Rinsho Geka Gakkai Zasshi (Journal of Japan Surgical Association) Vol. 67; no. 6; pp. 1213 - 1217
• Main Authors: SUZUKI, Rumi, KAWAMATA, Akiko, OMI, Yoko, IIHARA, Masatoshi, OBARA, Takao
• Format: Journal Article
• Language: English
• Published: Japan Surgical Association 25.06.2006
• ISSN: 1345-2843; 1882-5133
• DOI: 10.3919/jjsa.67.1213

We report a patient with multiple endocrine neoplasia type 2B (MEN 2B) who was treated successfully for medullary thyroid carcinoma (MTC). The patient, a 15-year-old boy, was referred to us for treatment of MTC. He had been suspected of having MEN 2B at an early age because of his characteristic body configuration (Marfanoid habitus) and multiple mucosal neuromas of the lips and tongue. He had no family history of MEN 2B, but the genetic testing revealed a point mutation at codon 918 of the RET protooncogene. Initial examination revealed tumors in both lobes of the thyroid gland and elevated serum levels of calcitonin (530pg/ml) and CEA (9.6ng/ml). Laboratory data and CT scan were negative for pheochromocytoma. The patient underwent total thyroidectomy and bilateral modified neck dissection (D3a) under a diagnosis of MTC (T2mN0M0, Stage II) without any complications. Histological examination revealed multiple MTC and no lymph node metastasis. Currently, he remains well without evidence of tumor recurrence 2 years after surgery. Although MEN 2B is quite a rare disease, it is important to recognize the syndrome because MTC in MEN 2B is well known for its clinical aggressiveness, and only early intervention provides a chance of cure.