Primary Primitive Neuroectodermal Tumor of the Spine With t(11;22): Report of 3 Cases and Review of Literature.
J Pediatr Hematol Oncol
; 43(7): e983-e986, 2021 Oct 01.
Article
in En
| MEDLINE
| ID: mdl-33480656
ABSTRACT
Intradural extramedullary peripheral primitive neuroectodermal tumor (pPNET) with t(11;22) is a rare clinical finding in the pediatric population with few published cases in the literature. The authors report 3 cases of intradural primary pPNET and discuss the clinical presentation, treatment, and survival of the patients. Clinicians should be vigilant in considering pPNET in the differential diagnosis of extradural masses. The authors also compare the clinical course and outcome of therapy with primary PNET of the central nervous system and Ewing sarcoma family of tumors. In addition, this report highlights the risk for leptomeningeal dissemination at recurrence and discusses the importance of central nervous system-targeted therapy for durable disease control.
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Sarcoma, Ewing
/
Spinal Neoplasms
/
Translocation, Genetic
/
Bone Neoplasms
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Chromosomes, Human, Pair 11
/
Chromosomes, Human, Pair 22
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Neuroectodermal Tumors, Primitive
Type of study:
Diagnostic_studies
/
Prognostic_studies
/
Systematic_reviews
Limits:
Adolescent
/
Adult
/
Female
/
Humans
/
Male
Language:
En
Journal:
J Pediatr Hematol Oncol
Journal subject:
HEMATOLOGIA
/
NEOPLASIAS
/
PEDIATRIA
Year:
2021
Type:
Article