title: Development of mediastinal lymphoma after radiotherapy for concurrent medulloblastoma and PNET in a patient with Gorlin syndrome.
abstract: BACKGROUND
  Very young children with Gorlin syndrome are at risk for developing medulloblastoma. Patients with Gorlin syndrome may have multiple system abnormalities, including basal cell carcinomas, jaw cysts, desmoplastic medulloblastoma, palmar/plantar pits, rib abnormalities, and intracranial falx calcification. The early diagnosis of Gorlin syndrome in desmoplastic medulloblastoma patients is very important because these patients should receive chemotherapy as a first-line treatment and should avoid radiotherapy as much as possible.
  
  
  METHODS
  In the present study, a 5-year-old male patient had a concurrent cerebellar desmoplastic medulloblastoma and temporal primitive neuroectodermal tumor. Examinations of this patient revealed multiple café-au-lait spots, a jaw cyst, and a bifid rib. A molecular classification analysis revealed that the patient's cerebellar tumor was of the sonic hedgehog subtype. Twenty-seven months after tumor resection and cerebrospinal irradiation were performed, mediastinal lymphoma was found in the patient. The patient ultimately died of lymphoma. To the best of our knowledge, this is the first report of a concurrent medulloblastoma and primitive neuroectodermal tumor and the fourth report of multiple café-au-lait spots in a patient with Gorlin syndrome. This report is also the first account of the development of mediastinal lymphoma after spinal irradiation in a patient with Gorlin syndrome.
  
  
  CONCLUSIONS
  Chemotherapy should be the first-line treatment for medulloblastoma patients with Gorlin syndrome. Young patients with medulloblastoma of the desmoplastic subtype and multiple café-au-lait spots should be thoroughly examined for Gorlin syndrome.
authors: Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, 6 Tiantan xili, Chonwen District, Beijing, 100050, China.|Beijing Neurosurgical Institute, Capital Medical University, Beijing, 100050, China.
authors: Tao
authors: T
authors: Jiang
authors: Beijing Neurosurgical Institute, Capital Medical University, Beijing, 100050, China.
authors: Junmei
authors: J
authors: Wang
authors: Beijing Chao-Yang Hospital, Capital Medical University, Beijing, 100020, China. littletony2004@163.com.
authors: Ying
authors: Y
authors: Wang
authors: Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University, 6 Tiantan xili, Chonwen District, Beijing, 100050, China. lichundelicd@163.com.
authors: Chunde
authors: C
authors: Li
mesh_terms: D001478:Basal Cell Nevus Syndrome / Q000532:radiotherapy*; D001932:Brain Neoplasms / Q000532:radiotherapy*; D002528:Cerebellar Neoplasms / Q000532:radiotherapy; D002675:Child, Preschool; D017809:Fatal Outcome; D006801:Humans; D016399:Lymphoma, T-Cell / Q000175:diagnosis / Q000209:etiology*; D008297:Male; D008479:Mediastinal Neoplasms / Q000175:diagnosis / Q000209:etiology*; D008527:Medulloblastoma / Q000532:radiotherapy; D009378:Neoplasms, Multiple Primary / Q000532:radiotherapy*; D009381:Neoplasms, Radiation-Induced / Q000175:diagnosis / Q000209:etiology*; D018242:Neuroectodermal Tumors, Primitive / Q000532:radiotherapy*
keywords: Café-au-lait spots; Chemotherapy; Gorlin syndrome; Medulloblastoma; SHH subtype