CNS Tumors: Embryonal
Treatment Summary
- Consists of surgery, post-operative RT, and chemotherapy.
- Requirement of craniospinal irradiation, regardless of extent of disease, is the element that distinguishes the treatment of these tumors.
Atypical teratoid/ rhabdoid tumor (ATRT)
Pathology
- Monosomy 22, deletion of 22q11 and alteration in the 1NI1 gene are characteristic of ATRT
- NF2-22q11 (Characteristic gene mutations)
- Imi1-22q11.2 (Characteristic gene mutations)
Prognosis of Atypical teratoid/ rhabdoid tumor (ATRT)
- Carries worse prognosis compared to PNET, survival beyond 1 to 2 years is anecdotal.
Supratentorial PNET (S-PNET)
- < 5% of all CNS tumor
- Median age 3 years
- Worse prognosis than medulloblastoma: OS-5 of 20%-35%. (medulloblastoma OS-5 of 80%)
- Standard therapy for patients with sPNET includes :
- craniospinal irradiation to 36 Gy
- boost of 54 Gy to the initial tumor.
- adjuvant chemotherapy (8 in 1 or CCNU, prednisone, and vincristine)
- four-institution trial
- aggressive surgery
- craniospinal irradiation to 23.4 for M0 disease
- dose-intensive chemotherapy for 4 cylcles (Ref. Strother D)
- In the CCG 921 study in which neoadjuvant and adjuvant 8-in-1 chemotherapy was compared to adjuvant CCNU, prednisone, and vincristine, each given with radiation therapy, 3-year PFS and survival were 45% and 57%, respectively, for all patients with sPNET and did not differ significantly between the two study arms. (Ref. Cohen BH)
Medulloblastoma
Pineoblastoma
- Pineoblastoma is most often confused with medulloblastoma histologically and by contiguous anatomic location.
Others
- Primitive polar spongioblastoma is a rare cerebral tumor thought to be derived rom migrating glial precursor cells.
- Ependymoblastoma is a pooly differentiated embryonal tumor including ependymal differentiation signified by multilayered rosettes.
- Cerebral neuroblastoma ranges hitologically from an undifferentiated tumor similar to the extra-CNS childhood neuroblastoma often including unilayered Homer Wright rosettes, to lesions demonstrating considerable ganglionic differentiation.
- Medulloepithelioma is the most primitive embryonal tumor, histologically showing features of primitive medullary epithelium and primitive tubular structures.
page revision: 3, last edited: 16 Jun 2007 06:02