ECEESPE2025 ePoster Presentations Endocrine Related Cancer (100 abstracts)
1University of Sousse, Faculty of Medicine of Sousse, Farhat Hached University Hospital, Endocrinology Diabetology Department, 4000, Sousse, Tunisia, Sousse, Tunisia; 2University of Sousse, Faculty of Medicine of Sousse, Farhat Hached University Hospital, Biochemistry Department, 4000, Sousse, Tunisia, Sousse, Tunisia
JOINT3255
Introduction: Adrenocortical carcinoma (ACC) is an exceptionally rare malignancy. It may be classified into functioning and non-functioning types. Estrogen-secreting ACC is exceedingly rare, representing 1% to 2% of all ACCs. In men, estrogen excess manifests as gynecomastia, decreased libido, and hypogonadotropic hypogonadism. This report describes a rare case of estrogen-secreting ACC in which bilateral gynecomastia was the predominant clinical feature.
Case Presentation: A 45-year-old male presented to our department with bilateral gynecomastia, accompanied by a 6 kg weight loss, fatigue, anorexia, abdominal pain, erectile dysfunction, and decreased libido over three months. Physical examination revealed facial erythema, bilateral grade II gynecomastia, and a palpable 20 cm abdominal mass located in the left flank and extending to the epigastric and umbilical regions. Laboratory findings included testosterone at 2.97 ng/mL associated with elevated estradiol (169 pg/mL), suppressed FSH (0.3 mIU/mL), and elevated lactate dehydrogenase. TSH, FT4, prolactin and hCG levels were in the normal range. Abdominopelvic CT-scan identified a large (23×20×13 cm) left retroperitoneal mass with heterogeneous enhancement, necrosis, and calcifications, closely associated with the stomach, duodenum (D4), left kidney, and left renal vein. A low-dose dexamethasone suppression test confirmed ACTH-independent Cushings syndrome with non-suppressed cortisol at 103.68 ng/mL and low ACTH level at 0.87 pg/mL. The patient was referred for extensive adrenalectomy with lymph node dissection.
Discussion: Feminizing adrenal tumors are rare, and hyperestrogenemia typically arises from peripheral androgen conversion. However, in this case, it is suggested that estradiol is directly secreted by the tumor. Histopathology remains the gold standard for ACC diagnosis, and surgical resection is the primary curative treatment. Adjuvant therapies are tailored to tumor grade, stage, and patient-specific factors. This case underscores the importance of considering ACC in males presenting with gynecomastia and highlights the need for prompt diagnostic evaluation and multidisciplinary management.