Adrenocorticotropin independent macronodular adrenocortical hyperplasia (AIMAH)

Case contributed by Dr Vitalijs Lobarevs


Patient admitted to the hospital after neck abscess drainage with fatigue, dyspnea and back pain. On physical exam patient had hypertension (TA - 180/110 mmHg), tachycardia (110 bpm) and typical signs of Cushing syndrome: moon face, striae, hirsutism and central obesity (BMI: 37, waist circumference: 120 cm). Patient also had hyperkyphosis, and a five year history of type 2 diabetes mellitus, that was treated with 1000 mg of metformin, and chronic hepatitis C.

Patient Data

Age: 40-45Y
Gender: Male

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%.

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 is 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 was performed
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Case information

rID: 44014
Published: 6th Apr 2016
Last edited: 6th Apr 2016
System: Urogenital
Inclusion in quiz mode: Included

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