Primary Central Nervous System Tumors in Adolescents: A Population-Based Study on Epidemiology and Clinical Pathways in a Challenging Age Group
Abstract
:1. Introduction
2. Materials and Methods
2.1. Data Collection
2.2. Statistical Analysis
3. Results
3.1. Incidence: Newly Diagnosed Primary CNS Tumors in Adolescents, 2008–2020
3.2. Survival: From Diagnosis to 5 Years in Adolescents, 2008–2017
3.3. Distributions of Tumors by Behavior, Site and Histology
- The most common tumor site was the pituitary gland and craniopharyngeal duct, representing 22.4% of all tumors.
- Frontal (4.6%), temporal (7.3%), and parietal (4.6%) lobes combined represented 16.5% of all tumors.
- The cerebellum accounted for 12.3% of all tumors.
- The most frequently reported histology overall was non-malignant pituitary tumors (13.7%), followed by pilocytic astrocytoma (11%) and ependymal tumors (7.3%).
- For malignant tumors, parietal (9.5%), frontal (8.1%), temporal (4.1%) lobes accounted for 21.7% of cases (Figure 4).
- The most common malignant CNS tumors were germ cell tumors (20.3%), followed by embryonal tumors (16.2%). Among embryonal tumors, medulloblastoma and atypical teratoid rhabdoid tumor (ATRT) accounted for 91.7% and 8.3%, respectively.
- For non-malignant tumors, 33.1% were located in the pituitary gland and craniopharyngeal duct (CP).
- The most common histology among non-malignant tumors was pituitary adenoma (20.7%), followed by pilocytic astrocytoma (16.6%).
3.4. Pathway of Care
4. Discussion
5. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Acknowledgments
Conflicts of Interest
Abbreviations
CCRC | Childhood Cancer Registry of Campania |
CNS | Central nervous system |
AYAs | Adolescents and young adults |
AIEOP | Associazione Italiana Ematologia Oncologia Pediatrica |
ICD-O | Classification of Diseases for Oncology |
ICCC | Classification of Childhood Cancer |
WHO | World Health Organization |
OS | Observed survival |
IR | Incident rate |
CPP | Choroid plexus papilloma |
ESMO | European Society for Medical Oncology |
SIOPE | European Society for Pediatric Oncology |
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n = 55 (25.1%) | Neuroimaging (n, %) | Specific Tumor Markers (n, %) |
---|---|---|
Non-malignant neoplasm (80,000, 80,001) | 33 (100%) | 0 |
Malignant neoplasm (80,003) | 3 (100%) | 0 |
Pituitary adenoma (82,710, 82,720) | 5 (33.3%) | 10 (66.7%) |
Malignant glioma (93,803) | 3 (100%) | 0 |
Subependymal giant cell astrocytoma (93,841) | 1 (100%) | 0 |
Intracranial and Intraspinal Tumors International Classification of Childhood Cancer—3rd ed | ICD-O-3a Histology Code | Sex (M/F) | N° of Newly Diagnosed Tumors | % | WHO Grade | Histologically Confirmed (%) | Incidence Rate ** | |
---|---|---|---|---|---|---|---|---|
IIIa Ependymoma and choroid plexus tumor | 13/6 | 19 | 10.1 | 100 | 4.2 | |||
Myxopapillary ependymoma * | 9394/1 | 5/3 | 8 | 4.3 | I | 100 | 1.8 | |
Choroid plexus papilloma * | 9390/0 | 2/1 | 3 | 1.6 | I | 100 | 0.7 | |
Ependymoma, other and NOS | 9391/3, 9392/3 | 6/2 | 8 | 4.3 | II | 100 | 1.8 | |
IIIb Astrocytomas | 27/19 | 46 | 24.5 | 100 | 10.3 | |||
Pilocytic astrocytoma * | 9421/1 | 14/10 | 24 | 12.8 | I | 100 | 5.4 | |
Pilomyxoid astrocytoma | 9425/3 | 1/1 | 2 | 1.1 | I | 100 | 0.4 | |
Fibrillary astrocytoma | 9420/3 | 1/0 | 1 | 0.5 | II | 100 | 0.2 | |
Anaplastic astrocytoma | 9401/3 | 1/0 | 1 | 0.5 | III | 100 | 0.2 | |
Subependymal astrocytoma * | 9384/1 | 0/1 | 1 | 0.5 | 0 | 0.2 | ||
Pleomorphic xanthoastrocytoma | 9424/3 | 0/1 | 1 | 0.5 | 100 | 0.2 | ||
Glioblastoma and variants | 9440/39,441/3 | 6/2 | 8 | 4.3 | IV | 100 | 1.8 | |
Astrocytomas, NOS | 9400/3 | 4/4 | 8 | 4.3 | 100 | 1.8 | ||
IIIc Intracranial and intraspinal embryonal tumor | 8/5 | 12 | 6.4 | 100 | 2.7 | |||
Medulloblastoma, variants | 9470/3, 9474/3 | 6/2 | 8 | 4.3 | IV | 100 | 1.8 | |
Desmoplastic/nodular medulloblastoma | 1/2 | 3 | 1.6 | IV | 100 | 0.7 | ||
Atypical teratoid/rhabdoid tumor | 9508/3 | 1/0 | 1 | 0.5 | IV | 100 | 0.2 | |
IIId Other gliomas | 2/9 | 11 | 5.9 | 73 | 2.5 | |||
Oligodendroglioma | 9450/3 | 0/1 | 1 | 0.5 | II | 100 | 0.2 | |
Astroblastoma | 9430/3 | 0/1 | 1 | 0.5 | 100 | 0.2 | ||
Glioma NOS (excl.optic nerve) | 9380/3, 9385/3 | 2/7 | 9 | 4.8 | 56 | 2.0 | ||
IIIe Other specified CNS tumors | 23/41 | 64 | 34.0 | 77 | 14.3 | |||
Dysembryoplastic neuroepithelial tumor * | 9413/0 | 2/3 | 5 | 2.7 | I | 100 | 1.1 | |
Gangliocytomas, ganglioglioma * | 9505/1 | 4/5 | 9 | 4.8 | I | 100 | 2.0 | |
Meningioma, non-malignant * | 9531/0, 9532/0, 9533/0, 9537/0, 9538/1, 9539/1 | 7/1 | 8 | 4.3 | I | 100 | 1.8 | |
Craniopharyngioma * | 9351/1 | 2/0 | 2 | 1.1 | I | 100 | 0.4 | |
Central neurocytoma * | 9506/1 | 1/6 | 7 | 3.7 | II | 100 | 1.6 | |
Papillary glioneuronal tumor * | 9509/1 | 0/3 | 3 | 1.6 | I | 100 | 0.7 | |
Pituitary tumors * | 8270/0, 8271/0 | 7/23 | 30 | 16.0 | - | 50 | 6.7 | |
IIIf Other specified CNS tumors | 14/22 | 36 | 19.1 | 0 | 8.0 | |||
Malignant | 8000/3 | 1/2 | 3 | 1.6 | - | 0 | 0.7 | |
Non-malignant * | 8000/0, 1 | 13/20 | 33 | 17.6 | - | 0 | 7.4 | |
Total malignant CNS tumors | 29/26 | 55 | 29.3 | 89 | 12.3 | |||
Total malignant & non-malignant CNS tumors | 87/101 | 188 | 71 | 42.0 | ||||
Other intracranial and intraspinal tumors | ||||||||
II Lymphomas and others reticuloendothelial cell tumors | 3/0 | 3 | 9.7 | 100 | 0.7 | |||
Malignant lymphoma, large B-cell, diffuse, NOS | 9680/3 | |||||||
Malignant lymphoma, large B-cell, diffuse, immunoblastic, NOS | 9683/3 | |||||||
Langerhans cell histiocytosis * | 9751/1 | |||||||
IXb Fibrosarcomas, peripheral nerve sheath tumors | 1/0 | 1 | 3.2 | 100 | 0.2 | |||
Nerve sheath tumors | 9540/3 | 1/0 | 1 | 3.2 | 100 | 0.2 | ||
Xa Intracranial and intraspinal germ cell tumors | 15/0 | 15 | 48.4 | 100 | 3.3 | |||
Dysgerminoma | 9060/3 | 1/0 | 1 | 3.2 | 100 | 0.2 | ||
Germinoma | 9064/3 | 12/0 | 12 | 38.7 | 100 | 2.7 | ||
Mixed germ cell tumors | 9085/3 | 2/0 | 2 | 6.5 | 100 | 0.4 | ||
XId Melanomas | 1/0 | 1 | 3.2 | 100 | 0.2 | |||
Meningeal melanomatosis | 8720/3 | 1/0 | 3.2 | 100 | 0.2 | |||
No ICCC coded | 5/7 | 11 | 35.5 | 100 | 2.5 | |||
Schwannomas * | 9560/0 | 4/7 | 11 | 35.5 | 100 | 2.5 | ||
Total other intracranial and intraspinal tumors | 24/7 | 31 | 100 | 6.9 |
5-Year Survival | Equality of Survivor Functions | ||||
---|---|---|---|---|---|
OS% | 95% CI | Log-Rank Test (p Value) | |||
Sex | |||||
Males | 76.7 | 63.8 | 85.5 | ||
Females | 90.3 | 80.7 | 95.2 | <0.05 | |
Behavior | |||||
Malignant | 59.5 | 42.0 | 73.2 | ||
Non-malignant | 93.7 | 86.5 | 97.1 | <0.001 | |
Period | |||||
2008–2012 | 78.2 | 64.8 | 87.0 | ||
2013–2017 | 88.3 | 78.7 | 93.7 | 0.11 | |
ICCC-3 | |||||
IIIa Ependymoma and choroid plexus tumor | 85.7 | 53.9 | 96.2 | ||
IIIb Astrocytomas | 70.0 | 50.3 | 83.1 | ||
IIIc Intracranial and intraspinal embryonal tumor | 75.0 | 31.5 | 93.1 | ||
IIId Other gliomas | 50.0 | 11.1 | 80.4 | ||
IIIe Other specified CNS tumors | 93.9 | 82.2 | 98.0 | ||
IIIf Other unspecified CNS tumors | 92.0 | 71.6 | 97.9 | ||
Xa Intracranial and intraspinal germ cell tumors | 90.9 | 50.8 | 98.7 |
N (%) | Histologically Confirmed | |
---|---|---|
Adult neurosurgery
| 143 (65.3%) 79 (55.2%) 64 (44.8%) | 133 (93%) 71 (89.9%) 58 (90.6%) |
Pediatric neurosurgery
| 32 (14.6%) 17 (53.1%) 15 (46.9%) | 30 (93.7%) 14 (82.3%) 15 (100%) |
Other medical unit
| 44 (20.1%) 29 (65.9%) 15 (34.1%) | 0 (0%) |
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Share and Cite
De Martino, L.; Piga, P.; Sessa, M.; Calì, C.; Russo, C.; Picariello, S.; Onorini, N.; Spennato, P.; Quaglietta, L.; Donofrio, M.V.; et al. Primary Central Nervous System Tumors in Adolescents: A Population-Based Study on Epidemiology and Clinical Pathways in a Challenging Age Group. Curr. Oncol. 2025, 32, 222. https://doi.org/10.3390/curroncol32040222
De Martino L, Piga P, Sessa M, Calì C, Russo C, Picariello S, Onorini N, Spennato P, Quaglietta L, Donofrio MV, et al. Primary Central Nervous System Tumors in Adolescents: A Population-Based Study on Epidemiology and Clinical Pathways in a Challenging Age Group. Current Oncology. 2025; 32(4):222. https://doi.org/10.3390/curroncol32040222
Chicago/Turabian StyleDe Martino, Lucia, Patrizia Piga, Marcella Sessa, Camilla Calì, Camilla Russo, Stefania Picariello, Nicola Onorini, Pietro Spennato, Lucia Quaglietta, Maria Vittoria Donofrio, and et al. 2025. "Primary Central Nervous System Tumors in Adolescents: A Population-Based Study on Epidemiology and Clinical Pathways in a Challenging Age Group" Current Oncology 32, no. 4: 222. https://doi.org/10.3390/curroncol32040222
APA StyleDe Martino, L., Piga, P., Sessa, M., Calì, C., Russo, C., Picariello, S., Onorini, N., Spennato, P., Quaglietta, L., Donofrio, M. V., Cinalli, G., Vetrano, F., & Savoia, F. (2025). Primary Central Nervous System Tumors in Adolescents: A Population-Based Study on Epidemiology and Clinical Pathways in a Challenging Age Group. Current Oncology, 32(4), 222. https://doi.org/10.3390/curroncol32040222