Adrenocorticotropin independent macronodular adrenocortical hyperplasia (AIMAH)

Case contributed by Dr Vitalijs Lobarevs

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

Age: 45 years
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% (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
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Case information

rID: 44014
Published: 6th Apr 2016
Last edited: 14th Aug 2019
System: Urogenital
Inclusion in quiz mode: Included

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