Central nervous system tumors

Back to all scientific topics
Header illustration for the page "Central nervous system tumors"

Central nervous system (CNS) tumors are relatively common in children and young adults, showing the second highest incidence after hematological cancers in childhood (Siegel et al, 2022). 

Upon CNS diagnosis, high dose chemotherapy and cranial radiation represent the elective oncological treatment, posing an increased risk for patients’ reproductive function. Radiation to the hypothalamic-pituitary-axis can negatively impact pubertal development and increase the risk of further infertility due to gonadotropin dysregulation (Vern-Gross et al, 2015). 

Young patients diagnosed with CNS tumors are eligible for fertility preservation, since they have an increased risk of premature ovarian insufficiency due to gonadotoxic treatments. Although both oocyte cryopreservation and ovarian tissue cryopreservation can be performed, the latter appears to be the most commonly offered to these patients, due to their prepubertal status in the majority of cases, and making up around 11% of patients having their tissue cryopreserved nowadays (Poirot et al, 2019). 

CNS tumors histotype at young age include astrocytoma, medulloblastoma, glioblastoma and ependymoma in more than 50% of cases (Steliarova-Foucher et al, 2017). These tumor types have a limited capacity of extraneural metastases, ranging from 0.5 to 18 % of cases (Rickert et al, 2003). The risk of metastases may be related in some cases to shunt placement, which is more likely to be needed in the presence of pediatric tumors for their location to the posterior fossa and obstructing the ventricular system (Xu et al, 2018). 

Ovarian metastases from CNS tumors are rarely reported in the literature. However, different histological tumor types may exert different behavior, varying the risk of minimal residual disease dissemination upon ovarian tissue transplantation. Recently, oncological risk of ovarian tissue transplantation has been assessed as low for a number of histological types including astrocytoma, ependymoma, glioblastoma (Nguyen et al, 2021), in which no evidence of disease reimplantation was observed. This has been partially assessed also in a case series of 3 patients diagnosed with CNS-primitive neuroectodermal tumor (PNET) (Nguyen et al, 2020). 

PNET are a group of embryonal tumors consisting of poorly differentiated neuroepithelial cells and represent the 2-3 % of all childhood brain tumors (Louis et al, 2021). Although they are characterized by an aggressive behavior, extracranial metastases are infrequent (Varan et al, 2006). 

Regarding medulloblastoma, two cases of cancer metastases have been reported in the literature, the first one involving a 4-year-old patient with disseminated disease to several pelvic organs (Paterson, 1961) and a 33-year-old patient with peritoneal dissemination of the disease (Lamovec and Pogaènik, 2001). However, a larger case series investigating ovarian tissue testing for minimal residual diseases found no evidence of CNS tumor cell reimplantation within ovarian tissue (Nguyen et al, 2021). 

References:

  1. Siegel RL, Miller KD, Fuchs HE, Jemal A. Cancer statistics, 2022. CA Cancer J Clin. 2022 Jan;72(1):7-33.
  2. Vern-Gross TZ, Bradley JA, Rotondo RL, Indelicato DJ. Fertility in childhood cancer survivors following cranial irradiation for primary central nervous system and skull base tumors. Radiother Oncol. 2015 Nov;117(2):195-205.
  3. Poirot C, Brugieres L, Yakouben K, Prades-Borio M, Marzouk F, de Lambert G, Pacquement H, Bernaudin F, Neven B, Paye-Jaouen A, et al. Ovarian tissue cryopreservation for fertility preservation in 418 girls and adolescents up to 15 years of age facing highly gonadotoxic treatment. Twenty years of experience at a single center. Acta Obstet Gynecol Scand. 2019 May;98(5):630-637.
  4. Steliarova-Foucher E, Colombet M, Ries LAG, Moreno F, Dolya A, Bray F, Hesseling P, Shin HY, Stiller CA; IICC-3 contributors. International incidence of childhood cancer, 2001-10: a population-based registry study. Lancet Oncol. 2017 Jun;18(6):719-731.
  5. Rickert CH. Extraneural metastases of paediatric brain tumours. Acta Neuropathol. 2003 Apr;105(4):309-27.
  6. Xu K, Khine KT, Ooi YC, Quinsey CS. A systematic review of shunt-related extraneural metastases of primary central nervous system tumors. Clin Neurol Neurosurg. 2018 Nov;174:239-243.
  7. Nguyen TYT, Cacciottola L, Camboni A, Ravau J, De Vos M, Demeestere I, Donnez J, Dolmans MM. Ovarian tissue cryopreservation and transplantation in patients with central nervous system tumours. Hum Reprod. 2021 Apr 20;36(5):1296-1309.
  8. Nguyen TYT, Camboni A, Masciangelo R, Donnez J, Dolmans MM. Is Ovarian Tissue Transplantation Safe in Patients with Central Nervous System Primitive Neuroectodermal Tumors? J Clin Med. 2020 Dec 18;9(12):4101.
  9. Louis DN, Perry A, Wesseling P, Brat DJ, Cree IA, Figarella-Branger D, Hawkins C, Ng HK, Pfister SM, Reifenberger G, Soffietti R, von Deimling A, Ellison DW. The 2021 WHO Classification of Tumors of the Central Nervous System: a summary. Neuro Oncol. 2021 Aug 2;23(8):1231-1251.
  10. Varan A, Sari N, Akalan N, Söylemezoğlu F, Akyüz C, Kutluk T, Büyükpamukçu M. Extraneural metastasis in intracranial tumors in children: the experience of a single center. J Neurooncol. 2006 Sep;79(2):187-90.
  11. PATERSON E. Distant metastases from medulloblastoma of the cerebellum. Brain. 1961 Jun;84:301-9. doi: 10.1093/brain/84.2.301.
  12. Lamovec J, Pogaènik A. Metastatic medulloblastoma to the breast. Virchows Arch. 2001 Aug;439(2):201-5.

Share this content