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.