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A Novel Method: Super-selective Adrenal Venous Sampling
Published on: September 15, 2017
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Adrenal Cushing syndrome with detectable ACTH from an unexpected source.
Thinzar M Lwin1, Nehal Galal2, Shweta Gera3
1Department of Surgery, Mount Sinai Beth Israel, New York, New York, USA.
BMJ Case Reports
|December 2, 2016
Summary
Rare mixed corticomedullary adrenal tumours (MCMT) can present with both Cushing syndrome and pheochromocytoma. This case underscores the need for comprehensive biochemical testing in patients with adrenal masses.
Area of Science:
- Endocrinology
- Oncology
Background:
- Mixed corticomedullary adrenal tumours (MCMT) are exceptionally rare endocrine neoplasms.
- Adrenal masses require thorough biochemical evaluation due to potential for hormone overproduction.
Observation:
- A 48-year-old male presented with hypertension and Cushing syndrome, initially suggesting adrenal or ectopic ACTH-dependent Cushing syndrome.
- Initial biochemical tests showed elevated cortisol and ACTH, with normal plasma metanephrines.
- During attempted laparoscopic adrenalectomy, the patient experienced resistant hypertension, prompting repeat testing that revealed elevated plasma metanephrines.
Findings:
- Pathological examination confirmed a mixed corticomedullary adrenal tumour (MCMT).
- The patient's clinical course indicated coexisting Cushing syndrome and pheochromocytoma.
- Successful management involved α-blockade prior to adrenalectomy.
Implications:
- This case highlights the diagnostic challenge posed by MCMT, which can secrete multiple hormones.
- Emphasizes the critical importance of comprehensive biochemical screening for adrenal masses, including metanephrines, even with initial normal results.
- Suggests a potential for MCMT to present with concurrent hormonal syndromes, necessitating careful pre-operative assessment and management.
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