SEOM clinical guideline for management of adult medulloblastoma (2020)

Clin Transl Oncol. 2021 May;23(5):940-947. doi: 10.1007/s12094-021-02581-1. Epub 2021 Apr 1.

Abstract

Recent advances in molecular profiling, have reclassified medulloblastoma, an undifferentiated tumor of the posterior fossa, in at least four diseases, each one with differences in prognosis, epidemiology and sensibility to different treatments. The recommended management of a lesion with radiological characteristics suggestive of MB includes maximum safe resection followed by a post-surgical MR < 48 h, LCR cytology and MR of the neuroaxis. Prognostic factors, such as presence of a residual tumor volume > 1.5 cm2, presence of micro- or macroscopic dissemination, and age > 3 years as well as pathological (presence of anaplastic or large cell features) and molecular findings (group, 4, 3 or p53 SHH mutated subgroup) determine the risk of relapse and should guide adjuvant management. Although there is evidence that both high-risk patients and to a lesser degree, standard-risk patients benefit from adjuvant craneoespinal radiation followed by consolidation chemotherapy, tolerability is a concern in adult patients, leading invariably to dose reductions. Treatment after relapse is to be considered palliative and inclusion on clinical trials, focusing on the molecular alterations that define each subgroup, should be encouraged. Selected patients can benefit from surgical rescue or targeted radiation or high-dose chemotherapy followed by autologous self-transplant. Even in patients that are cured by chemorradiation presence of significant sequelae is common and patients must undergo lifelong follow-up.

Keywords: Adults; Medulloblastoma; Treatment.

Publication types

  • Practice Guideline

MeSH terms

  • Adult
  • Antineoplastic Agents / adverse effects
  • Antineoplastic Agents / therapeutic use
  • Cerebellar Neoplasms / diagnosis*
  • Cerebellar Neoplasms / genetics
  • Cerebellar Neoplasms / pathology
  • Cerebellar Neoplasms / therapy*
  • Cisplatin / adverse effects
  • Combined Modality Therapy / methods
  • Evidence-Based Medicine
  • Humans
  • Medical Oncology
  • Medulloblastoma / diagnosis*
  • Medulloblastoma / genetics
  • Medulloblastoma / pathology
  • Medulloblastoma / therapy*
  • Molecular Targeted Therapy / methods
  • Neoplasm Recurrence, Local / therapy
  • Palliative Care
  • Postoperative Complications / etiology
  • Prognosis
  • Radiotherapy / adverse effects
  • Retreatment / methods
  • Societies, Medical
  • Spain
  • Vincristine / adverse effects

Substances

  • Antineoplastic Agents
  • Vincristine
  • Cisplatin