A Case of Multiple Endocrine Neoplasia Associated with VIPoma.

Si Won Lee; Young Sik Choi; Yo Han Park; Kyung Seung Oh; Jung Woo Shin; Il Jin Kim; Shinya Uchino

doi:10.3803/jkes.2005.20.1.64

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Case Report A Case of Multiple Endocrine Neoplasia Associated with VIPoma.: Si Won Lee, Young Sik Choi, Yo Han Park, Kyung Seung Oh, Jung Woo Shin, Il Jin Kim, Shinya Uchino; Endocrinology and Metabolism 2005;20(1):64-70
DOI: https://doi.org/10.3803/jkes.2005.20.1.64
Published online: February 1, 2005

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¹Department of Internal Medicine, Kosin University College of Medicine, Pusan, Korea, Korean Hereditary Tumor Registry, Korea.
²Department of Radiology, Kosin University College of Medicine, Pusan, Korea, Korean Hereditary Tumor Registry, Korea.
³Department of Nuclear Medicine, Kosin University College of Medicine, Pusan, Korea, Korean Hereditary Tumor Registry, Korea.
⁴Cancer Research Institute, Seoul National University, Seoul, Korea.
⁵Noguchi Thyroid Clinic & Hospital Foundation, Beppu, Japan.

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Abstract

Multiple endocrine neoplasia I(MEN I) is a genetic disorder that consists of neoplasia of neuroendocrine type in the parathyroid glands, in the islets of Langerhans in the pancreas, and in the anterior pituitary gland. Primary hyperparathyroidism is the most common feature and occurs in approximately 95% of MEN I patients. Pancreatic islet cell tumors occur in 40% of MEN I patients. Most of these tumors produce excessive amounts of hormones, such as gastrin, insulin, glucagon and vasoactive intestinal polypeptide(VIP). VIP-producing pancreatic tumors(VIPoma) associated with MEN I are rare and so far only one has been reported in Korea. Recently, we came across a case of MEN I, associated VIPoma presented persistent hypercalcemia after a parathyroidectomy. A 70 year old man had suffered from large amount of watery diarrhea, severe general weakness and paralysis of lower limbs for 3 months which suggests symptoms of hypercalcemia. Before the patient visited our hospital, he underwent subtotal parathyroidectomy due to hyperparathyroidism. Even though he was operation, there was no subsidization of the symptoms and abnormal findings of blood chemistry such as hypercalcemia, hypocalemia were remained unchanged. However, the parathyroid hormone level was still within normal limits. Abdominal computerized tomography scan demonstrated a mass of 2.5cm diameter in tail of the pancreas. As serum level of VIP hormone was also elevated, distal pancreatectomy was carried out to performed. There was improvement in the symptoms towards the normal condition and the level of biochemical parameters such as serum potassium, calcium and VIP, were also within the normal limits. In a direct sequence analysis, GAC-->CAT(Asp-->His) point mutation, at codon 383 of exon 9 of the MEN I gene was identified in both the patient and his son. The authors report a rare case of VIPoma associated with MEN I with review of literature on MEN I.

Keywords: VIPoma;Multiple endocrine neoplasia I;Hypercalcemia;Germline mutation;

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