Clinical analysis of 12 rhabdomyosarcoma children with intracranial extension

Objective\r\nTo explore the clinical characteristics, treatment and prognosis of rhabdomyosarcoma (RMS) with intracranial extension in children.\r\n\r\n\r\nMethods\r\nThe clinical data of 12 cases of children(5 males and 7 females) with RMS and intracranial involvement admitted into the Department of Pediatrics, Beijing Tongren Hospital Affiliated to Capital Medical University from December 2012 to December 2017 were analyzed retrospectively.There were 5 males and 7 females in 12 cases.The onset age ranged from 1.2 to 10.2 years old, with a median age of 3.4 years old.The clinical features, treatment methods and prognosis were summarized.\r\n\r\n\r\nResults\r\nThe primary sites of 9 cases were in the head and neck (75%), 2 cases were in the chest and back (17%), and 1 case was in the perineum (8%). Nine patients had obvious central nervous system involvement symptoms, and cranial imaging findings were studied in all of the 12 patients.The clinical pathological stage and risk classification were: 6 cases of stage Ⅳ (1 case of central aggression); 6 cases of stage Ⅲ (4 cases of central aggression); 10 cases were in the high-risk group (4 cases of stage Ⅲ with central violations), and 2 cases (stage Ⅲ) in the intermediate risk group.All 12 cases received systemic chemotherapy, 8 cases received local or total cranial/spine radiotherapy, 9 cases underwent primary resection, and 4 cases underwent craniotomy to remove intracranial lesions.Up to January 2018, 4 cases of 12 children survived (including 2 disease-free patients) and 8 children died.The overall survival rate was 33% (4/12 cases) and the disease-free survival rate was 17% (2/12 cases).\r\n\r\n\r\nConclusions\r\nThe prognosis of RMS with intracranial infiltration is very poor, and the treatment effect is not good.Complete resection of early lesions is the key to cure RMS.Radiotherapy, individualized chemotherapy and surgical removal of intracranial lesions may have the value in controlling disease and prolonging survival time.\r\n\r\n\r\nKey words: \r\nRhabdomyosarcoma; Intracranial extension; Chemotherapy; Prognosis