Adrenocorticotropin independent macronodular adrenocortical hyperplasia (AIMAH)
Presentation
Patient admitted to the hospital after a neck abscess drainage with fatigue, dyspnea and back pain. On physical examination, he was hypertensive (TA - 180/110 mmHg) and tachycardic (110 bpm) with typical signs of Cushing syndrome: moon face, striae, hirsutism and central obesity (BMI: 37, waist circumference: 120 cm). He also had hyperkyphosis of his spine and had a five-year history of type 2 diabetes mellitus on metformin and chronic hepatitis C.
Patient Data
Bilateral multinodular adrenal enlargement, largest nodule on the right side 4.5 cm, on the left side 5.5 cm. Absolute washout was 75-77% (pre 6 to 15 HU, portal venous 66 to 67 HU, delay 20 to 22 HU).
Additional findings: gallstone and sigmoid diverticula.
Thoracic hyperkyphosis, osteoporotic Th7-Th10 wedge compression injuries.
Surgical specimen: 13 x 11 cm, enlarged left adrenal gland with multiple yellow-orange nodules.
Specimen was partially cut during laparoscopic evacuation.
Case Discussion
Laboratory data:
- serum glucose (on admission): 15.90 mmol/L
- HbA1c: 7.40 %
- C-peptide: 3.09 ng/mL
- cortisol (8.00): 14.8 µg/dl
- cortisol (22.00): 14.2 µg/dl
- 24h Cortisol in urine: 777 µg
- AKTH: <1.00 pg/ml
Brain MRI showed no evidence of pituitary microadenoma.
Patient underwent laparoscopic left side adrenalectomy.
Pathologist conclusion was:
- Macroscopically: received tissue material is cut in small pieces, excision line and fatty capsule are difficult to visualize
- tissue size: 13 x 11 cm
- bright yellow in color.
- Microscopically: Adrenal cortical clear cell nodules, with solid, trabecular composition, lacunar hemorrhages and some amount of fibrosis in stroma
- Capsule intact
- ICD-O code: M8370/0
- No special immunohistological tests were performed