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.