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central_nervous_system_tumor

Central nervous system tumor

Classification

Epidemiology

Tumours of central nervous system constitute 1%-2% of tumours in adults. The incidence of brain tumours has been reported to be around 3.9 and 3.0/one lac/year in males and females respectively.

Glioma and meningioma are the two most common primary central nervous system tumors, representing 70% and 20% of brain tumors, respectively 1) 2).

A total of 16 116 institutional records of CNS tumors were analyzed. The frequency and distribution of CNS tumors were evaluated by tumor type, patient age and patient gender. The annual relationship between CNS tumors and surgical discharges (SD) over the last 20 years was estimated.

The frequencies of most CNS tumors were consistent with those found worldwide, and the most common tumors were neuroepithelial tumors (33%), particularly astrocytic tumors (67%); meningeal tumors (26%); and pituitary tumors (20%). The incidence of pituitary tumors in these data was twice as high as that reported in other regions of the world, and the relationship between CNS tumors and SD was consistent over time (0.22-0.39).

This study summarizes the largest sample of CNS tumor cases analyzed in Mexico and provides an important reference of the frequency of this tumor type in the country. This work will serve as a basis for conducting studies evaluating factors associated with the presence of CNS tumors and for identifying adequate public health interventions 3).

see Central Brain Tumor Registry of the United States.

Diagnosis

Accurate diagnosis is essential for optimal clinical management. The increasing use of molecular diagnostics has opened up novel possibilities for more precise classification of CNS tumors.

Bächli et al., report a single-institutional collection of pediatric brain tumor cases that underwent a refinement or a change of diagnosis after completion of molecular analysis that affected clinical decision-making including the application of molecularly informed targeted therapies. 13 pediatric CNS tumors were analyzed by conventional histology, immunohistochemistry, and molecular diagnostics including DNA methylation profiling in 12 cases, DNA sequencing in 8 cases and RNA sequencing in 3 cases. 3 tumors had a refinement of diagnosis upon molecular testing, and 6 tumors underwent a change of diagnosis. Targeted therapy was initiated in 5 cases. An underlying cancer predisposition syndrome was detected in 5 cases. Although this case series, retrospective and not population based, has its limitations, insight can be gained regarding precision of diagnosis and clinical management of the patients in selected cases. Accuracy of diagnosis was improved in the cases presented here by the addition of molecular diagnostics, impacting clinical management of affected patients, both in the first-line as well as in the follow-up setting. This additional information may support the clinical decision making in the treatment of challenging pediatric CNS tumors. Prospective testing of the clinical value of molecular diagnostics is currently underway 4).

Outcome

Central nervous system tumors account for the highest mortality among pediatric malignancies.

1)
Ohgaki H and Kleihues P (2005) Epidemiology and etiology of gliomas. Acta Neuropathol 109: 93–108.
2)
Longstreth WT Jr., Dennis LK, McGuire VM, Drangsholt MT and Koepsell TD (1993) Epidemiology of intracranial meningioma. Cancer 72: 639–648.
3)
Aguirre-Cruz L, Rangel-López E, Cruz-Aguilera DL, Rodríguez-Pérez CE, Ruano L, Velásquez-Pérez L, Martínez-Moreno M, Garduño-Espinosa J, Sotelo J. Historical distribution of central nervous system tumors in the Mexican National Institute of Neurology and Neurosurgery. Salud Publica Mex. 2016 Apr;58(2):171-178. PubMed PMID: 27557375.
4)
Bächli H, Ecker J, van Tilburg C, Sturm D, Selt F, Sahm F, Koelsche C, Grund K, Sutter C, Pietsch T, Witt H, Herold-Mende C, von Deimling A, Jones D, Pfister S, Witt O, Milde T. Molecular Diagnostics in Pediatric Brain Tumors: Impact on Diagnosis and Clinical Decision-Making - A Selected Case Series. Klin Padiatr. 2018 Jul 11. doi: 10.1055/a-0637-9653. [Epub ahead of print] PubMed PMID: 29996150.
central_nervous_system_tumor.txt · Last modified: 2018/07/12 11:31 by administrador