Consolidation Intensity-Modulated Whole Abdominal Radiotherapyas Part of the Complex Treatment for Metastatic AdrenocorticalCarcinoma in Adolescence

Abstract

Adrenocortical carcinoma (ACC) is an extremely rare solid malignant tumor. We present rare local advanced ACC with lung metastases in adolescence. After visible radical surgery for a 100mm/130mm/50mm tumor, the initial histological diagnosis was anaplastic pleomorphic neuroblastoma originating from the left adrenal gland. Despite the eight courses chemotherapy, the disease progression was reported, which requires a pathohistological revision with a broad immunohistochemical (IHC) analysis. After surgical excision of peritoneal metastases, a system Ch alteration of Vincristine/ Cyclophospamide/ Farmarubicin and Vepesid/ Cisplatin - on 4 blocks each, in combination with the constant intake of Mitotane (Lesydren) was performed. Due to the disease progression, a consolidation intensity modulated radiotherapy (IMRT) of the right lung metastasis measuring 23 mm/ 16 mm with PET/ CT hypermetabolic activity/ SUV max 9,67 and of a vertebral L2 metastasis was performed. In December 2021, a right-sided thoracotomy with resection of three pulmonary
metastases, followed by pulmonary segmentectomy of the irradiated right-sided lung metastasis was performed. The peritoneal metastatic dessimination necessitated a consolidation whole abdominal IMRT with boost in the tumor bed/ upper pole of the left kidney. 

With this rare clinical case, we emphasize the role of complex treatment, especially with respect to local oncological therapy (surgery and IMRT) in metastatic adolescence ACC. Despite metastatic disease, complex treatment achieves a 3 -year survival rate.