Primary thymic carcinoid with Cushing's syndrome.
In a 52-year-old Caucasian man osteopoikilosis had been misdiagnosed roentgenologically 2 years before his death. Gradually he developed Cushing's syndrome and ultimately superior vena caval obstruction. At autopsy a primary thymic carcinoid with extensive osteoblastic bone metastasis was found. Immunohistochemically the tumor was shown to be positive for adrenocorticotropic hormone (ACTH), cytokeratin (KL1), neuron-specific enolase, synaptophysin, chromogranin and glucagon. Remarkably the tumour was negative for serotonin despite high urinary hydroxyindolacetic acid levels. Bilateral hyperplasia of the adrenal cortex was found. The adenohypophysis showed a considerable reduction of ACTH-producing cells and numerous Crooke's cells with a characteristic immunohistochemical pattern.
Department of Pathology, University of Freiburg, Federal Republic of Germany.
G
G
Wollensak
E W
EW
Herbst
A
A
Beck
H E
HE
Schaefer
D000302:Adrenal Cortex / Q000473:pathology; D000324:Adrenocorticotropic Hormone / Q000032:analysis; D001859:Bone Neoplasms / Q000000981:diagnostic imaging / Q000556:secondary*; D002276:Carcinoid Tumor / Q000737:chemistry / Q000150:complications* / Q000648:ultrastructure; D003480:Cushing Syndrome / Q000150:complications*; D003951:Diagnostic Errors; D006801:Humans; D007633:Keratins / Q000032:analysis; D008297:Male; D008875:Middle Aged; D010023:Osteopoikilosis / Q000000981:diagnostic imaging; D010751:Phosphopyruvate Hydratase / Q000032:analysis; D011859:Radiography; D013953:Thymus Neoplasms / Q000737:chemistry / Q000150:complications* / Q000648:ultrastructure; D014683:Vena Cava, Superior / Q000473:pathology
D007633:Keratins; D000324:Adrenocorticotropic Hormone; D010751:Phosphopyruvate Hydratase