J Korean Soc Endocrinol.  2001 Apr;16(2):231-237.

A Case of Multiple Endocrine Neoplasia Type I Presenting with a Watery Diarrhea

• Choe WH
• Park YJ
• Hong IC
• Park SH
• Choi SC
• Lee HR
• Jeong IK
• chung JH
• Min YK
• Lee MS
• Lee MK
• Kim KW
• Kim WS

Abstract

MEN1 is characterized by the combined occurrence of tumors of the parathyroids, pancreatic islet cells and the anterior pituitary. Pancreatic islet cell tumors occur in 40% of MEN1 patients. Pancreatic polypeptidomas occur most commonly but they are asymptomatic. Gastrinomas are the second most common type. VIPomas are rare and there has been no report of a case in Korea so far. We encountered a case of pancreatic VIPoma in MEN Type I. A 49 year old man was referred from his local hospital presenting with a sudden onset of an explosive watery diarrhea of 3 months duration. Abnormal findings in his blood chemistry were hypercalcemia and hypokalemia. The 99mTc-sestamibi sintigraphy showed an increased uptake in right lower parathyroid gland. Abdominal CT demonstrated a mass of 6x4 cm in tail of the pancreas and multiple lesions in both hepatic lobes. Serum levels of VIP hormones were elevated. Subtotal parathyroidectomy and subtotal pancreatectomy were done. Postoperatively his symptoms were improved transiently, however the patient showed repetitive attacks of watery diarrhea. So in order to palliate his symptoms, an RF ablation of the metastatic liver masses was performed. After that therapy his clinical symptoms were reduced dramatically. Unfortunately, the patients condition worsened again. Despite of continuous octreotide therapy, interferon and two courses of combination chemotherapy, the hepatic metastases failed to regress and the patient died 10 months after the diagnosis of a metastatic VIPoma. This is the first report of pancreatic VIPoma in MEN type I in Korea.

MeSH Terms

Chemistry
Diagnosis
Diarrhea*
Drug Therapy, Combination
Gastrinoma
Humans
Hypercalcemia
Hypokalemia
Interferons
Islets of Langerhans
Korea
Liver
Male
Middle Aged
Multiple Endocrine Neoplasia Type 1*
Multiple Endocrine Neoplasia*
Neoplasm Metastasis
Octreotide
Pancreas
Pancreatectomy
Parathyroid Glands
Parathyroidectomy
Technetium Tc 99m Sestamibi
Tomography, X-Ray Computed
Vipoma
Interferons
Octreotide
Technetium Tc 99m Sestamibi