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[Fulminant hypercorticism due to ACTG producing pheochromocytoma].
Z T Useinova1, E A Pigarova1, D G Bel'tsevich1
1Endocrinology Research Centre.
Problemy Endokrinologii
|July 29, 2024
Summary
This case study highlights pheochromocytoma as a rare cause of ACTH-ectopic syndrome, leading to Cushing's syndrome. Early diagnosis and surgical removal of the adrenal tumor are crucial for managing hypercortisolemia.
Area of Science:
- Endocrinology
- Oncology
- Pathology
Background:
- Endogenous hypercorticism (EH) results from hypercortisolemia, with ACTH-dependent and independent forms.
- ACTH-ectopic syndrome (ACTH-ES), a rare cause of ACTH-dependent EH, involves ACTH hyperproduction by non-pituitary neuroendocrine tumors.
- Pheochromocytoma (PCC) is an uncommon source of ACTH-ES.
Purpose of the Study:
- To present a clinical case of ACTH-ectopic syndrome caused by pheochromocytoma.
- To illustrate the diagnostic challenges and management of this rare condition.
Main Methods:
- Clinical case presentation.
- Diagnostic workup including biochemical tests (urinary catecholamines) and imaging (MSCT).
- Morphological and immunohistochemical analysis (Chromogranin A, ACTH) post-adrenalectomy.
Main Results:
- A patient presented with clinical signs of hypercorticism and confirmed Cushing's syndrome.
- Multispiral CT revealed a left adrenal gland mass; elevated urinary catecholamines indicated pheochromocytoma.
- Histopathology confirmed pheochromocytoma with strong ACTH and Chromogranin A expression.
Conclusions:
- Pheochromocytoma can manifest as a paraneoplastic cause of ACTH-ectopic syndrome and Cushing's syndrome.
- Comprehensive diagnostic evaluation is essential for identifying rare causes of hypercortisolemia.
- Surgical resection of the pheochromocytoma led to successful management.
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