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Primary thymic carcinoid with Cushing's syndrome
G Wollensak1, E W Herbst, A Beck
1Department of Pathology, University of Freiburg, Federal Republic of Germany.
Virchows Archiv. A, Pathological Anatomy and Histopathology
|January 1, 1992
Summary
A thymic carcinoid tumor caused Cushing's syndrome and bone metastases in a patient initially misdiagnosed with osteopoikilosis. The tumor produced adrenocorticotropic hormone (ACTH) but lacked serotonin, despite elevated hydroxyindolacetic acid levels.
Area of Science:
- Endocrinology
- Oncology
- Pathology
Background:
- A 52-year-old man with misdiagnosed osteopoikilosis developed Cushing's syndrome and superior vena cava obstruction.
- Autopsy revealed a primary thymic carcinoid tumor with widespread osteoblastic bone metastases.
Observation:
- Immunohistochemistry confirmed the tumor expressed adrenocorticotropic hormone (ACTH), cytokeratin, neuron-specific enolase, synaptophysin, chromogranin, and glucagon.
- Tumor tissue was negative for serotonin, contrasting with high urinary hydroxyindolacetic acid levels.
- Bilateral adrenal cortex hyperplasia and adenohypophysis changes, including reduced ACTH cells and Crooke's cells, were observed.
Findings:
- The thymic carcinoid was the likely cause of Cushing's syndrome through ectopic ACTH production.
- Extensive osteoblastic bone metastases were a significant feature of the carcinoid.
- Discrepancy between tumor serotonin and urinary hydroxyindolacetic acid suggests complex hormonal regulation or metabolism.
Implications:
- This case highlights a rare presentation of thymic carcinoid with paraneoplastic Cushing's syndrome and extensive bone metastases.
- Understanding the hormonal profile of neuroendocrine tumors is crucial for accurate diagnosis and treatment.
- The findings underscore the importance of comprehensive diagnostic workup in complex endocrine and oncologic cases.