ABSTRACT
@EN The multiple endocrine neoplasia (MEN) syndromes were classified into two broad categories, MEN type Ⅰ (MEN Ⅰ) and MEN type Ⅱ (MEN Ⅱ). The MEN Ⅱ syndrome has been further subcategorized into two variants called MEN Ⅱa and MEN Ⅱb
(formerly MEN
Ⅲ). There are a number of rare hereditary MEN syndromes which do not fit the MEN Ⅰ and MEN Ⅱ categories, which is called as mixed type. This mixed type MEN occurs very rarely. In 1978, Tateishi et al. report a case of bilateral
pheochromocytoma
associated with pancreatic islet cell tumor for the first time. Thereafter, only several cases of such mixed type MEN have been reported all over the world. However there was no case report of a mixed type MEN in Korea.
Recently, we experienced a case of familial pheochromocytoma associated with pancreatic islet cell tumor. A pancreatic islet cell tumor occurred in a 25-year old woman who received a bilateral adrenalectomy for pheochromocytoma 6 years ago. She
had
family of mother and younger sister diagnosed as having pancreatic head tumor, and younger brother who had been operated for pheochromocytoma and confirmed histologically. (J Kor Endocrinol 8:100~105, 1993)
|