PMID- 14601094
OWN - NLM
STAT- MEDLINE
DCOM- 20031125
LR  - 20220317
IS  - 0008-543X (Print)
IS  - 0008-543X (Linking)
VI  - 98
IP  - 10
DP  - 2003 Nov 15
TI  - Clear cell ependymoma: a clinicopathologic and radiographic analysis of 10 
      patients.
PG  - 2232-44
AB  - BACKGROUND: Clear cell ependymoma (CCE) is an uncommon central nervous system 
      tumor with a predilection for the supratentorial region in children. 
      Histologically, it may mimic oligodendroglioma, central neurocytoma, 
      hemangioblastoma, and renal cell carcinoma. METHODS: The authors reviewed the 
      clinical, radiographic, and pathologic features, therapy, and outcome in 10 
      children with CCE who were treated at St. Jude Children's Research Hospital 
      (1984-2003). Fluorescence in situ hybridization (FISH) was performed using 1p/1q, 
      19p/19q, CEP18/DAL1, and bcr/NF2 probe pairs. RESULTS: The median patient age at 
      diagnosis was 7.5 years (range, 1-19 years). Tumors occurred supratentorially in 
      9 of 10 patients. All tumors had rounded nuclei with surrounding, clear halos and 
      at least focal perivascular pseudorosettes. Seven tumors had anaplastic features. 
      No deletions involving 1p, 19q, or NF2 were detected. The tumors from 5 of 7 
      patients, all with anaplasia, had losses of both CEP18 and DAL-1. 
      Radiographically, all tumors were enhanced, and 9 tumors had associated cysts 
      with enhancing walls. Seven patients underwent gross total resection, which was 
      near total in one patient and subtotal in two patients. Five patients received 
      immediate postoperative local radiotherapy. Three patients were diagnosed 
      initially with pilocytic astrocytoma (one patient) and oligodendroglioma (two 
      patients) and were observed. The progression-free survival and overall survival 
      rates at 5 years were 34% +/- 20% and 75% +/- 19%, respectively. The median 
      follow-up was 37 months (range, 5-239 months). Five patients developed local 
      recurrence within a median of 9 months after diagnosis. Two patients developed 
      extracranial soft tissue and lymph node metastases. CONCLUSIONS: CCEs were found 
      to have a predilection for extraneural metastases and early recurrence and 
      demonstrate characteristic radiographic features, anaplastic histologic features, 
      and chromosome 18 losses. The authors recommend resection followed by local 
      radiotherapy as the treatment of choice in children.
CI  - Copyright 2003 American Cancer Society.
FAU - Fouladi, Maryam
AU  - Fouladi M
AD  - Department of Hematology-Oncology, St. Jude Children's Research Hospital, 
      Memphis, Tennessee 38105, USA. maryam.fouladi@stjude.org
FAU - Helton, Kathleen
AU  - Helton K
FAU - Dalton, James
AU  - Dalton J
FAU - Gilger, Elizabeth
AU  - Gilger E
FAU - Gajjar, Amar
AU  - Gajjar A
FAU - Merchant, Thomas
AU  - Merchant T
FAU - Kun, Larry
AU  - Kun L
FAU - Newsham, Irene
AU  - Newsham I
FAU - Burger, Peter
AU  - Burger P
FAU - Fuller, Christine
AU  - Fuller C
LA  - eng
GR  - PA30CA 21765/CA/NCI NIH HHS/United States
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PT  - Research Support, U.S. Gov't, P.H.S.
PL  - United States
TA  - Cancer
JT  - Cancer
JID - 0374236
SB  - IM
MH  - Adolescent
MH  - Adult
MH  - Brain Neoplasms/genetics/*pathology/radiotherapy/surgery
MH  - Child
MH  - Child, Preschool
MH  - *Chromosomes, Human, Pair 18
MH  - Combined Modality Therapy
MH  - Disease-Free Survival
MH  - Ependymoma/genetics/*pathology/radiotherapy/surgery
MH  - Female
MH  - Humans
MH  - Infant
MH  - Male
MH  - *Neoplasm Metastasis
MH  - *Neoplasm Recurrence, Local
MH  - Prognosis
MH  - Retrospective Studies
MH  - Treatment Outcome
EDAT- 2003/11/06 05:00
MHDA- 2003/12/03 05:00
CRDT- 2003/11/06 05:00
PHST- 2003/11/06 05:00 [pubmed]
PHST- 2003/12/03 05:00 [medline]
PHST- 2003/11/06 05:00 [entrez]
AID - 10.1002/cncr.11783 [doi]
PST - ppublish
SO  - Cancer. 2003 Nov 15;98(10):2232-44. doi: 10.1002/cncr.11783.