A Case of Multiple Endocrine Neoplasia Type I Presenting with a Watery Diarrhea.
- Author:
Won Hyeok CHOE
1
;
Yu Jeong PARK
;
Il Chol HONG
;
Se Hoon PARK
;
Sung Chul CHOI
;
Hyo Rak LEE
;
In Kyung JEONG
;
Jae Hoon CHUNG
;
yong Ki MIN
;
Myung Shik LEE
;
Moon Kyu LEE
;
Kwang Won KIM
;
Won Seog KIM
Author Information
1. Division of Endocrinology and Metabolism, Division of Hemato-Oncology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
- Publication Type:Original Article
- Keywords:
MEN type I;
VIPoma;
Parathyroid hyperplasia
- MeSH:
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
- From:Journal of Korean Society of Endocrinology
2001;16(2):231-237
- CountryRepublic of Korea
- Language:Korean
-
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.
