Cushing증후군을 수반한 흉선의 비정형성 유암종 -면역조직화학적 및 전자현미경적 방법을 통한 ACTH의 증명-
Atypical Thymic Carcinoid Associated with Ectopic ACTH Syndrome -Demonstration of ACTH secretion with ultrastructural and immunohistochemical studies-
서은희, 김성연, 심영수, 김용일,
소속 상세정보
서은희 ( )
서울대학교 의과대학 병리하교실
김성연 ( )
서울대학교 의과대학 내과학교실
심영수 ( )
서울대학교 의과대학 내과학교실
김용일 ( )
서울대학교 의과대학 병리하교실
KMID : 0357919860200010091
Abstract
We reported a case of atypical thymic carcinoid tumor with ectopic ACTH syndrome
which was proved by the presence of intracytoplasmic ACTH in the tumor cells using
PAP method and electron microscopy.
The patient was a 43-year-old housewife who was referred with Cushing's syndrome
of unknown origin, and subsequantly followed by many disabling symptoms including
infection, diabetes mellitus and electrolyte imbalance for which bilateral adrenalectomy
was carried out. Thereafter, rapid rise in serum ACTH level and hyperpigmentation
were followed. Pituitary irradiation was done under the impression of Nelson's syndrome
despite of lacking evidence of pituitary tumor, but high serum ACTH persisted. Seven
months after pituitary irradiation, two anterior mediastinal massed were first noticed and
removed. Immunohistochemical and ultrastructural studies confirmed this neoplasm as a
ACTH producing spindle cell variant of atypical thymic carcinoid tumor.
When dealing with a patient who developed Nelson's syndrome-like phenomenon
without apparent pituitary tumor, the possibility of ectopic ACTH syndrome caused by
thymic carcinoid should be considered.
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