Endocrine Abstracts

JOINT2559

Background: Adrenocortical carcinoma (ACC) is a rare endocrine malignancy often with an unfavourable prognosis. The coexistence of acromegaly and ACC is quite rare. Here we describe a case of metastatic ACC in a patient who had Acromegaly treated with surgery, radiotherapy, and medical management.

Case: A male in his late 30s was referred to the endocrine department in 2018 to investigate for change in facial appearance. He was biochemically confirmed to have acromegaly, a 33 mm pituitary macroadenoma with bilateral cavernous invasion. He underwent transsphenoidal adenectomy and histology revealed somatotroph adenoma of partially granulated type with Ki-67 index of 3-5 %. Post-operatively, he had a biochemical recurrence of acromegaly along with panhypopituitarism and residual tumour in the left cavernous sinus. Hence, he was commenced on lanreotide 120 mg monthly injections. His IGF-1 failed to control on lanreotide and was then offered 25 sessions of pituitary radiotherapy in 2019. Subsequently, he was started on pegvisomant injections in 2021 due to suboptimal IGF-1 control following which IGF-1 levels were controlled. In 2023, while being continued on pegvisomant, he was noted to have progressive lower limb swelling, weight gain and deranged liver function tests. Therefore pegvisomant, lanreotide and hydrocortisone were discontinued. Detailed evaluation revealed non-suppressed plasma cortisol (1593 nmol/L), significantly raised urine cortisol (>5000 nmol/l)and suppressed ACTH. Abdominal imaging revealed a right adrenocortical mass with metastasis, inferior vena cava thrombus, bilateral pleural effusion and ascites. He was initially planned for surgical excision followed by aggressive chemotherapy. However, a repeat CT scan of the adrenal showed rapid tumour progression, and his general condition deteriorated. He was initiated on Mitotane and Metyrapone with hydrocortisone. A multi-disciplinary team concluded that he was not for surgery and palliative care was the best option. He was initiated on enoxaparin for IVC thrombus, unfortunately, the tumour bled resulting in further deterioration and death. Extensive evaluation for genetic studies revealed negative results for AIP, MEN1, CDC73, CDKN1B, PRKAR1A, RET, VHL genes.

Conclusion: Acromegaly is associated with greater morbidity and a high incidence of tumours, possibly due to the permissive role of elevated GH and IGF-I levels. Adrenal lesions seem more frequent in acromegaly than in the general population, however, no single factor (GH/IGF-I levels or disease duration) predicts them. Certain genetically inherited conditions are associated with aggressive pituitary tumours and ACC. Negative results of frequently mutated gene profiles open a path for more research on novel genetic associations.