Adrenal adenoma

Case contributed by Ammar Ashraf
Diagnosis certain

Presentation

Known case of hyperprolactinemia and primary infertility (for 6 years) with incidental finding of mass lesion in the right hypochondrium on abdominal ultrasound.

Patient Data

Age: 25 years
Gender: Female
ultrasound

Mildly heterogeneous hypoechoic, hypervascular solid lesion measuring 5.3 x 7.5 cm, is seen in the right hypochondrium, between the liver and right kidney. 

ct

Well-defined heterogeneous lesion measuring approximately 5.5 x 5.9 cm seen between the liver and right kidney. The lesion shows high internal vascularity through a bunch of small vessels directly from the abdominal aorta with early venous filling, suggestive of underlying arterio-venous shunting. The prominent draining veins are running in a cephalad direction, connecting the lesion to the lateral limb of right adrenal gland and finally draining into the IVC. No fat or calcifications are seen in the lesion. Morphology of the left adrenal gland is within normal limits. Renal arteries, renal veins and IVC are patent and well-opacified.

Two small lesions (averaging 5 mm each) showing vivid enhancement in the arterial phase and not clearly appreciated in the rest of the phases, are seen in segment VII and high sub-capsular segment VIII of the liver; these lesions are likely flash filling hepatic hemangiomas. A non-enhancing hypodensity (likely a hepatic cyst) measuring 3 mm is noted in peripheral segment VII of the liver. Average size spleen with a few tiny peripheral hypodensities which are likely splenic cysts. Left renal partial duplex collecting system with two ureters traceable up to L4/L5 level.

mri

Re-demonstration of well-defined solid mass lesion with prominent vascular components in the right hypochondrium. The lesion is abutting the under-surface of the liver and is also inseparable from the tip of the lateral limb of the right adrenal gland and lateral wall of the second part of the duodenum. Re-demonstration of small enhancing lesion (flash filling hemangioma?) and tiny cyst in segment VII of the liver. Segment VIII lesion is not clearly appreciable. Re-demonstration of few small peripheral splenic cysts.

Biochemistry

  • Plasma metanephrine (LC-MS/MS) (ep) = <50 ng/l (<90)
  • Plasma Normetanephrine (LC-MS/MS) (ep) = 66 ng/l (<129)
  • Cortisol (urine) = 378+ ug/24hour (21-292)
  • Serum gastrin = 47.4 pg/ml (up to 115)
  • Serum Chromogranin A = 57ug/l (<102)
  • Urinary catecholamines (HPLC) (u):
    • Epinephrine/Creatinine =  value below limit of detection. Calculation of creatinine-related excretion is therefore not possible. 
    • Norepinephrine/Creatinine = 28.7 ug/gCrea (<114)
    • Dopamine/Creatinine (U) = 269 ug/gCrea (<422)
    • Medication and drugs-Creatinine (enzymatic) (u) = 453 mg/l (290-2260)

Case Discussion

Right hypochondrial mass lesion, likely arising from the right adrenal gland. Other possibility can be exophytic hepatic lesion or exophytic duodenal mass lesion like GIST.

The patient underwent surgery (laparotomy and right adrenalectomy).

Histopathology: Right adrenal adenoma (measuring 8 x 6 x 4 cm). The immunostain with the antibody anti-Cytokeratin AE1/AE3, Synaptophysin, Vimentin, Inhibin, CD56, Calretinin, CK (CAM5.2) is positive. The immunostain with the antibody anti-S100, NSE, BCL2, HMB45 and Chromogranin are negative. The immunostain with the antibody anti-Melan-A is diffusely positive in tumor cells.  Immunostain with the antibody anti-neurofilaments is negative. The proliferation index Ki67 is very low (<5%).

Comment: The application of the Weiss Criteria showed the following scoring:  Vein invasion score: 0. Capsular invasion score: 0. Nuclear grade score: 0. Mitosis (< 2 mitoses/50 HPF) score: 0.  Sinusoidal invasion score: 0.  Necrosis score: 0.  Atypical mitosis score: 0.  Diffuse architecture score:1.  Clear vacuolated cells (<25%) score: 1.  Total score: 2. According to the score, the tumor is classified as adenoma (benign) (Score <3).

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