Multiple Endocrine Neoplasia Type Ila : A Case Report
TOSSAPORN SEEHERUNVONG, M.D.*, SUNCHAI CHURESIGAEW, M.D.*, VICHITRA HEMSRICHART, M.D.**
Affiliation : * Division of Endocrinology and Metabolism, Department of Medical Services, Children's Hospital, Bangkok 10400, ** Institute of Pathology, Department of Medical Services, Bangkok 10400, Thailand.
Abstract The authors reported a twelve year and four-month old girl who had prolonged fever for 2 weeks. Physical examination revealed a painless enlarged thyroid gland with firm consistency. Hyperparathyroidism was suspected because of hyp·ercalcemia, hypophosphatemia, high level of serum alkaline phosphatase, and decreased density of long bones. Thyroid scan showed a cold nodule of the left upper lobe which subsequently proved to be a medullary thyroid carcinoma by high serum thyrocalcitonin level and pathological examination. Her 24-hour urinary vanillyl mandelic acid was in the normal range, and abdominal ultrasonography demonstrated normal adrenal glands. Multiple endocrine neoplasia type Ua (MEN Ila) was diagnosed by medullary thyroid carcinoma and hyperparathyroidism. However, the fully developed syndrome is cha- racterized by the combined occurrence of medullary thyroid carcinoma, primary hyperpara- thyroidism, and pheochromocytomas. This syndrome is a rare, complex, and potentially lethal disease so early recognition and family screening are very important.
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