SUN-388 Immune Checkpoint Inhibitor Nivolumab in Advanced or Recurrent Adrenocortical Carcinoma

Abstract

Introduction: Adrenocortical carcinoma (ACC) is a rare and highly aggressive malignancy. Due to the rarity of this malignancy, controlled trials investigating efficacy of chemotherapy or immunotherapy are uncommon. Newer therapy with immune check point inhibitor Nivolumab, a monoclonal antibody of the programmed cell death 1 receptor ( PD 1) is investigational for the treatment of advanced or refractory ACC. Here we present a case of advanced stage IV ACC which was treated with nivolumab, and the disease showed response with shrinkage of the visceral metastatic lesions. Case: A 22-year-old female presented with vague abdominal pain and was found on US scan of the abdomen to have a mass in the left adrenal area. CT scan, PET scan followed by biopsy, confirmed ACC with bilateral lower lobe pulmonary nodules. Adrenal hormone evaluation was unremarkable. She underwent left adrenalectomy of a 20 cm tumor with radical nephrectomy and also bilateral wedge resections of lower lobe pulmonary nodules, all of which was positive for metastatic ACC. Surgical resection was followed by adjuvant chemotherapy (Adriamycin, Cis-platinum, Etoposide along with mitotane) and local radiation therapy to the primary tumor bed. Over the following months, she started to have worsening low back pain, and subsequently found to have a metastatic spinal lesion at the T12 vertebral body, which was treated with radiosurgery. She was also found to have liver metastases which were resected surgically. Despite chemotherapy, her disease continued to progress with recurrence of metastases in the lungs, liver and bone. Sunitinib was initiated without any response. Eventually she was started on off-label investigational single-agent nivolumab 3 years from the time of initial presentation. Follow-up CT scan 3 months post treatment with nivolumab showed radiological evidence of interval decrease in size of known metastatic lesions likely representing a response to therapy with nivolumab. The use of nivolumab was complicated by thyroiditis. Conclusion: Nivolumab, an immune checkpoint PD-1 inhibitor not currently approved for the treatment of ACC, may slow progression of ACC. Nivolumab has may many immune-related endocrine adverse effects, including hypophysitis, thyroiditis, autoimmune diabetes, and adrenal insufficiency. Our patient developed painless thyroiditis followed by hypothyroidism. In our patient, treatment with nivolumab showed a stabilization of visceral disease for > 6 months. Nivolumab may be a treatment option for patients with advanced or recurrent ACC