Endocrine Abstracts

The first case is a 19-year-old male who presented to his GP with lumbar pain, scrotal bruising, and weight loss. Abdominal CT showed a left 6.4 cm x 12 cm suprarenal mass; the right adrenal gland was atrophic. Plasma metanephrines and MIBG were normal. Urinary steroid profile (USP) showed raised 11-deoxycortisol, consistent with adrenocortical carcinoma (ACC). He underwent a left nephrectomy and adrenalectomy. Histology confirmed ACC. The lesion was encapsulated with negative margins; Modified-Weiss score 7, Ki67 43.2%; ENSAT staging 2 and pT2N0. Post-operative CT showed non-specific nodules in the surgical bed and normal ACC markers. He proceeded with Carboplatin-Etoposide chemotherapy after sperm banking and Mitotane. He was reviewed in the clinic, remained active, and referred for genetics. The second case is a 20-year-old female with one month of abdominal pain. She had an ultrasound which showed a left suprarenal mass. CT adrenal showed a 10.5 cm soft tissue within the left adrenal, with no evidence of invasion and normal right adrenal. The examination was unremarkable. There was no weight loss; periods were regular. There was no family history. Plasma metanephrines were normal. USP showed increased metabolites of steroid-precursors consistent with ACC. She had left adrenalectomy. The histology confirmed ACC, Modified-Weiss score 3, Ki67 2.6%, pT2 N0, ENSAT stage 2. Post-operative imaging showed no recurrence or metastases. USP showed no evidence of recurrence. She had TP53 gene testing. Both cases demonstrate rare cancer presenting in a younger age group with different trajectories. The presentation can include non-specific symptoms. There was no evidence of functionality in both patients; the size of the tumour was similar; however, Ki67 differed significantly. In ACC, the 5-year survival is 20-35%. Recurrence is 23-85% after complete surgical removal. A Ki67 of ≤10% is a good prognostic factor. The ADIUVO-2 Trial will provide data on low-risk patients, comparing observation against Mitotane treatment. It has shown 5-year recurrence-free survival is approximately 75%. A careful follow-up period should be established in this group. As in our second patient, where there is no clear pathway for post-operative management, subjecting them to Mitotane treatment could be more harmful to health. An MDT approach is required for meticulous management of the patient. Further studies looking into the recurrence-free patient groups are necessary. As endocrinologists, we must emphasise the uncertainty of this condition and provide holistic management, including addressing fertility and genetic screening. Management of ACC should be addressed with a personalised approach and tailored care.