Medulloblastomas in Pediatric and Adults

Adv Exp Med Biol. 2023:1405:117-152. doi: 10.1007/978-3-031-23705-8_5.

Abstract

Medulloblastoma is the primary malignant embryonic tumor of the cerebellum and the most common malignant tumor of childhood, accounting up to 25% of all CNS tumors in children, but is extremely rare in adults. Despite the fact that medulloblastomas are one of the most malignant human tumors, it is worthy to note that a great breakthrough has been achieved in our understanding of oncogenesis and the development of real methods of treatment. The main objective of surgical treatment is a maximum resection of tumor with minimal impairment of neurological functions, in order to reduce the volume, remove tumor tissue, get the biopsy, and restore the cerebrospinal fluid flow. The progress of surgical techniques (using a microscope, ultrasound suction), anesthesiology, and intensive care has significantly decreased surgical mortality and increased radicality of tumor removal. Postoperative mortality is less than one percent in most studies, while neurological complications have been reported between 5-10%. Radiotherapy is the main method of treatment in patients older than 3 years, which dramatically improved the recurrence-free survival. Nevertheless, the radiation therapy without systemic chemotherapy leads to a high risk of systemic metastases. After the role of chemotherapy was statistically proven, investigations of the optimal combination of different chemotherapy regimens continued around the world. Currently, 80% of patients can already be cured, however, the quality of life of patients in the long-term period remains quite low, which depends on many factors including endocrinological, cognitive, neurological, and otoneurologic aspects. Thus, the main strategic goal of the development of neuro-oncology is to reduce the doses of radiation therapy to the CNS and the main task of international research is to optimize existing protocols and develop fundamentally new ones based on molecular genetic research in order to improve the quality of life.

Keywords: Chemotherapy; Long-term follow-up; Medulloblastoma; Molecular biology; Neuroendocrinology; Neuroimaging; Radiotherapy; Surgery.

MeSH terms

  • Adult
  • Antineoplastic Combined Chemotherapy Protocols
  • Cerebellar Neoplasms* / drug therapy
  • Cerebellar Neoplasms* / radiotherapy
  • Child
  • Combined Modality Therapy
  • Humans
  • Medulloblastoma* / therapy
  • Quality of Life