SAT322 Case report: Impact Of Combined Treatment To Prolong Overall Survival In Advanced Adrenocortical Pediatric Cancer Associated With Li-Fraumeni Syndrome

Abstract

Abstract Disclosure: I.P. de Magalhães: None. N.C. de Sousa: None. H.L. Charchar: None. A.W. Kuhn: None. M.Y. Nishi: None. F.L. Ledesma: None. R.I. Lopes: None. F.T. Dénes: None. P. Herman: None. M.R. Menezes: None. A. Latronico: None. B.B. Mendonca: None. M.Q. Almeida: None. M.B. Fragoso: None. Background: Adrenocortical cancer (ACC) in pediatric patients (<15 years of age) are even rarer compared to the general population it is a classic presentation of Li-Fraumeni syndrome (LFS). ACC incidence in this subgroup is limited to 0.3 cases per million per year with a high incidence in Sothwest of Brazil. Furthermore, the overall five-year survival of pediatric ACC cases is better than in adult with a rate of approximately 50%, considering a complete resection required for cure. However, prognosis of childhood ACC is highly variable and difficult to predict in clinical practice. Clinical Case: We present the case of a 3-year-old girl who was diagnosed with metastatic adrenocortical carcinoma with classical signs of virilization and Cushing's syndrome. The patient underwent transperitoneal right adrenalectomy in May 2011. During surgery, there was rupture of the tumor capsule and the inferior vena cava. She was referred to our service after 8 months due to the presence of locoregional recurrence and maintained hormonal secretion. Histological data confirmed ACC and the tumor was classified as Weiss score 5, Wieneke: 4, Ki67 40%. Genetic analysis identified germline confirmed p-R337H on TP53, XAF1-E134. Imaging exams documented suspected liver metastasis and peritoneal implants. She underwent palliative treatment with mitotane (M) and systemic chemotherapy EDP+M with partial response. A new surgical approach to the residual lesions was proposed. The patient underwent right partial hepatectomy and peritoneal implant removed in September 2012. After metastasectomy, the patient presented persistence of the disease. The patient was exposed to a new cycle of EDP+M, but the disease progressed. She underwent radiofrequency ablation of abdominal lesions in July 2013 without success. A new surgical approach was indicated in July 2013. The histological analysis confirmed metastases of ACC. She underwent a new chemotherapy regimen with gemcitabine, docetaxel and fluorouracil + M. She presented a complete response confirmed by radiological exams and hormonal data. Mitotane was discontinued in 2016 due to bladder bleeding. She presented an excellent response with combined treatment, without recurrence up to now with 10 years of recurrence free survival and 12 years of overall survival. She developed normal puberty and reached the target height and maintains primary adrenal insufficiency with glico and mineralocorticoid replacement. Although the tumor spillage, histological and molecular data predicted a dismal outcome this patient surprisingly is presenting an excellent evolution under continuous surveillance. Clinical Lesson: This report described a challenging pediatric advanced ACC which the combined treatment strategy provided successful outcome Presentation: Saturday, June 17, 2023