Cushing's Disease Due to ACTH Producing Pituitary Carcinoma / 대한내분비학회지

ABSTRACT

Primary carcinoma of the pituitary is only rarely reported. About half of the reported cases are nonfunctional. It is generally agreed that the presence of distant metastasis is required to clearly establish the diagnosis of pituitary carcinoma. We have experienced a case of ACTH-producing pituitary carcinoma causing Cushing's syndrome which could be diagnosed by histologic features only without evidence of distant metastasis. A 35-year-old female with Cushingoid appearance was diagnosed as Cushing's disease after biochemical and neuroradiological evaluation. Near total removal of the pituitary tumor was performed. By histopathologic examination, the tumor revealed evidences of histological malignancy such as prominent nuclear pleomorphism, frequent mitoses and extensive tumor necrosis. Pseudosarcomatous components were also noted. By immunohistochemical studies, the tumor cells expressed ACTH. However, there was no evidence of distant metastasis at the initial operation. She was diagnosed as having ACTH producing pituitary carcinoma by histologic feature only. One month later, she complained progressive nausea and vomiting, and follow-up brain MRI revealed regrowing pituitary mass. She was reoperated and near total tumor bulk was removed again followed by radiotherapy with 5580 rads. Four month after the second operation, she developed generalized tonic clonic seizure. Brain CT showed multiple enhancing nodules on left temporal and frontal lobes, and around falx cerebri. She refused further treatment and was managed only with anticonvalsants. About month after discharge she died at home.