DEFYING THE PROGNOSIS

LONG-TERM SURVIVAL IN ADVANCED ADRENOCORTICAL CARCINOMA WITH MULTIMODAL THERAPY

Abstract

INTRODUCTION/BACKGROUND
Adrenocortical carcinoma (ACC) is a rare and aggressive malignancy with poor prognosis. Long-term survival is challenging due to high recurrence rates and limited treatment options. A multimodal treatment strategy includes surgical resection, systemic therapy, and radiotherapy. Stereotactic body radiotherapy (SBRT), a highly precise form of radiation therapy, targets tumours effectively with minimal surrounding damage.

CASE
A 33-year-old Chinese female presented with a one-month history of low back pain and constitutional symptoms. Abdominal CT scan revealed a 15 × 15 cm left adrenal mass with inferior vena cava (IVC) thrombosis and lung metastases. She underwent extensive surgery including left adrenalectomy, IVC thrombectomy, splenectomy, and lung metastasectomy. Histopathology confirmed metastatic ACC with a Ki-67 proliferation index of 40%.

One month post-operatively, mitotane was initiated and titrated up to a maximum tolerable dose of 3 g daily. Sorafenib was trialed but discontinued after four months due to adverse effects. Seven months post-op, PET scan revealed FDG-avid activity in a right upper lobe lung nodule and active IVC thrombus. The thrombus was deemed inoperable, and she underwent 10 cycles of SBRT.

A follow-up FDG-PET scan eight months later showed resolution of both the lung nodule and IVC thrombus. At 4- and 6-years post-surgery, PET-DOTATATE scans revealed DOTATATE-avid lesions in the upper lobes, but biopsy confirmed benign findings. Later FDG-PET/CT scans showed hypermetabolic activity consistent with bronchiectasis and atelectasis, with no signs of local recurrence.

CONCLUSION
Despite the typically poor prognosis of advanced ACC, this patient achieved long-term survival beyond eight years through a comprehensive, individualised treatment strategy involving surgery, systemic therapy, targeted SBRT, and multidisciplinary follow-up. This case highlights the potential role of SBRT in ACC and the importance of coordinated, patient-specific oncologic care.