CASE REPORT

A 47-year-old male presented to emergency room complaining of hypersensitivity, intractable nausea and vomiting. He exhibited poor general conditions, suffering from dehydration due to having nausea for the last 3 days. The patient had been experiencing anxiety and decreased libido for 6 months before being admitted at the amin hospital in Isfahan. He had been self-medicating with benzodiazepines (chlordiazepoxide 5mg/day) for the aforementioned anxiety and loss of libido for the last 6 months. The patient had no history of hypertension and reported a negative family history of such symptoms.
Findings included pain and tenderness in the right upper quadrant and the perigastric area. The pain worsened when the patient bent forward. Severe dehydration with no discernable weight loss and stage 4 tanner gynecomastia were also observed in the patient. The rest of the examinations were unremarkable and did not reveal any new finding.
As the first step to stabilize the patient, hydration therapy with an infusion of 500cc of normal saline IV was used. Because of the location and the pattern of the pain, a biliary ultrasound was performed that revealed a normal gallbladder with no signs of gallstones or biliary sludge. With a clear biliary ultrasound and no answers, an abdominal ultrasound was performed that revealed a mass in the region of the right adrenal gland alongside aortic lymphadenopathy. needing further evaluation for the aforementioned mass, MDCT with IV and PO contrast was performed. CT scan reported a large right sided retroperitoneal mass, displacing inferior vena cava (IVC) anteriorly and the right kidney inferiorly (fig 1).
The mass implied an adrenal origin. The absolute washout was 60% and an Hounsfield unit (HU) of 100-120 was recorded. The mass measured at an 175x120 mm. with the diagnoses of a large retroperitoneal mass of apparent adrenal origin, the need for a full hormone function test of the tumor became apparent. Tests for serum cortisol, ACTH, dehydroepiandrosterone sulfate (DHEAS) and 17-hydroxyprogesterone were performed to assess the functionality of the tumor. Patient’s serum ACTH and cortisol levels reported a rise in Cortisol ( 33.4 µg/dl (normal value 5-23 5-23g/dl)) and a reduction in ACTH levels (5.6 pg/ml (normal range 10-60 pg/mL)), and serum DHEAS and 17-hydroxyprogesterone also reported as 127 µg/dl (normal value 124-482µg/dl) and 9.8 ng/ml (normal value 0.2-3.1 ng/ml) respectively. With urinary Free epinephrine and norepinephrine of the patient being under 100 mcg and total metanephrine being lower than 1.3 mg, pheochromocytoma was ruled out.
As the patient presented with gynecomastia, LH, FSH, β-HCG, testosterone and estradiol were all tested. Lab results showed serum total testosterone level of the patient to be reduced to 0.6 ng/dl (normal values 1.75-9.5 ng/dl), and an elevated estradiol level of 112 pg/ml (normal value 4-94 pg/ml for males). serum LH was measured at 3.4 IU/L(normal value 1.2-8.6 IU/L), and β-HCG was 0.3 µg/dl (normal value <10 µg/dl) (Table 1). the results show the tumor to be also secreting estradiol and likely causing the aforementioned gynecomastia and loss of libido, and also suppressing testosterone production.
[Table 1. full hormone panel of the patient]
The patient also underwent a low dose dexamethasone suppression test with 1mg of dexamethasone. After the test serum cortisol of the patient was 2.12 µg/dl (cutoff of 1.8 µg/dl), affirming the Cushing’s syndrome diagnosis. There were no signs of buffalo hump, Cushing’s striae, proximal myopathy or easy bruising leading us to an ACTH independent subclinical Cushing’s syndrome. With the Cushing’s syndrome all but confirmed, the patient underwent an adrenalectomy with hydrocortisone coverage.
After the general anesthesia, right Kocher incision was done. The mass had almost enveloped the IVC and had to be dissected. With the complicated geography of the area and the invasive nature of the mass, the surgery was a challenge. And during the surgery IVC was damaged and had to be repaired immediately. 4 units of packed red blood cells and 4 units of FFP were transfused, alongside of about two liters of normal saline infusion.
Samples taken during the surgery were sent to pathology. preliminary reports indicated small round proliferation of epithelial cells forming nests and islets, Containing hyperchromic and granular nuclei with moderate amounts of cytoplasm. In the immunohistochemical report, the specimen was CD99+ and synaptophysin positive. CK20, CK7, chromogranin, synaptophysin, LCA, CD20 and CK markers were all reported negative alongside a K67 index of 30%. Samples were confirmed by two pathologists to be a case of definitive PNET.
Post-surgical hormonal panel showed a serum testosterone of 4ng/ml (normal value 1.72-9.5), serum ACTH of 56.8 pg/ml (normal values up to 46 pg/ml) which could be easily explained by the suppressed adrenal glands due to ectopic cortisol secretion of the mass and an 8 am. serum cortisol level reduced to 2.67mcgr/dl. In regards to the high risk of suppression in the left adrenal gland and the apparent risk of acute adrenal crisis in the patient, we decided to start the patient with a regiment of hydrocortisone and fluid therapy.
The patient was tapered off from corticosteroids during the next 6 months. he was referred to oncology department and started undergoing chemotherapy. As of now, 13 months has passed since the surgery and the patient has appeared to have made a full recovery, with the chemotherapy being successful and no signs of metastases or residual mass is seen.