Abstract
Introduction: We report a rare case of cortisol secreting
extracranial primary neuroectodermal tumor (PNET) originating from
adrenal gland in adulthood. PNETs are typically not functional, making
this case extremely rare. cortisol secreting PNETS are as of yet,
unheard of.
Patient profile : The patient was admitted to the outpatient
clinic with complaints of loss of libido, nausea, vomiting, pain in the
abdomen and gynecomastia. the patient underwent hormonal examination,
showing a marked increase in serum cortisol and estradiol. An abdominal
CT scan was performed due to abdominal pain that found a space occupying
lesion in retroperitoneal space displacing the IVC. Therefore, then
patient underwent laparoscopic adrenalectomy. After an
Immunohistological examination done on the excised tumor, the it was
diagnosed definitively PNET of the adrenal gland. The patient had no
complications and the hormonal levels returned to normal shortly after.
We referred the patient to oncology department and chemotherapy was
started for him.
Conclusion: This case has shown that an atypical manifestation
of PNET could be as a functional tumor, secreting hormones or other
bioactive material, in this case cortisol. PNET could also originate
from less likely organs and should be considered in fringe cases.