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EANO–EURACAN clinical practice guideline for diagnosis, treatment, and follow-up of post-pubertal and adult patients with medulloblastoma

Diagnosis

  • Expert Center

  • MultiDisciplinary Team

Clinical and radiological diagnosis

  • Diagnosis of primary central nervous system (CNS) tumor is suspected on CNS MRI performed for investigation of neurological symptoms :
    • headaches
    • seizures
    • cognitive impairment
    • other neurological deficits
  • Patients suspected of primary CNS tumor should be referred to an expert center hosting a multidisciplinary team including neuroradiologists, neurosurgeons, neuropathologists, molecular biologists, neuro-oncologists and radiation oncologists.

Pathological and molecular diagnosis

  • The first step of diagnostic procedure is getting a piece of the tumor for pathological analysis (i.e. examination under the microscope)
  • The neurosurgeon gets a piece of tumor during a biopsy if the tumor is not resectable or during surgical resection. If safe, surgical resection is also the first therapeutic intervention.
  • The pathological diagnosis should be performed according to The 2016 World Health Organization Classification of Tumors of the Central Nervous System, which incorporates classical pathology with molecular biology (integrated diagnosis)
  • Close 150 subtypes are individualized by the WHO, with profound differences in clinical behavior, treatment options, outcomes.

drawing about Diagnosis
picture - Staging and risk assessment

Staging and risk assessment

  • In some tumor types and faced to specific neurological symptoms additional investigation can be proposed
  • Whole CNS MRI if initial MRI was restricted to the brain or the spinal cord in specific diseases (i.e. ependymal tumors, medulloblastoma)
  • Lumbar puncture for cerebrospinal fluid is necessary in specific cases
  • Treatment decision making in neuro-oncology is also based on clinical conditions and age of the patients mainly for safety reason and ability to tolerate intensive therapies.
picture - Treatment

Treatment

  • MultiDisciplinary Team

  • Expert Center

  • Surgery

  • Radiation Therapy

  • Chemotherapy

  • Standard Treatment

  • After pathological and molecular diagnosis in one hand and staging/risk assessment in the other hand a treatment plan is established by the multidisciplinary tumor board
  • After surgery, additional treatments including radiotherapy and/or chemotherapy are needed

Follow-up, long-term implications and survivorship

  • Long term follow-up is required for primary CNS tumors patients
  • Follow up includes clinical, biological and radiological examination on a regular basis
  • Follow up is design according the tumor type to detect side effects, social impact and tumor recurrence to guide further interventions

Credits

Citation

Franceschi E, Hofer S, Brandes AA, et al. EANO-EURACAN clinical practice guideline for diagnosis, treatment, and follow-up of post-pubertal and adult patients with medulloblastoma. Lancet Oncol. 2019;20(12):e715-e728. doi.org/10.1016/S1470-2045(19)30669-2

PMID 31797797

Authors

Enrico Franceschi*, Silvia Hofer*, Alba A Brandes, Didier Frappaz, Rolf-Dieter Kortmann, Jacoline Bromberg, Volodia Dangouloff-Ros,
Nathalie Boddaert, Elke Hattingen, Benedikt Wiestler, Steven C Clifford, Dominique Figarella-Branger, Felice Giangaspero, Christine Haberler,
Torsten Pietsch, Kristian W Pajtler, Stefan M Pfister, Raphael Guzman, Walter Stummer, Stephanie E Combs, Clemens Seidel, Dagmar Beier,
Martin G McCabe, Michael Grotzer, Florence Laigle-Donadey, Ana S Guerreiro Stücklin, Ahmed Idbaih, Matthias Preusser, Martin van den Bent,
Michael Weller, Peter Hau

*Contributed equally

 

Patient’s typology

Child
Teenager
Adult

Schemas

Schema : Brain MRI showing medulloblastoma located on the right hemisphere of the cerebellum
Figure 1. Brain MRI showing medulloblastoma located on the right hemisphere of the cerebellum
Click to enlarge the picture : Brain MRI showing medulloblastoma located on the right hemisphere of the cerebellum
Schema : Pathological examination showing a desmoplastic/nodular medulloblastoma characterized by nodules and internodular zones. (Credit : Dr Franck Bielle). 
Figure 2. Pathological examination showing a desmoplastic/nodular medulloblastoma characterized by nodules and internodular zones. (Credit : Dr Franck Bielle). 
Click to enlarge the picture : Pathological examination showing a desmoplastic/nodular medulloblastoma characterized by nodules and internodular zones. (Credit : Dr Franck Bielle).