Document Detail


Congenital peripheral primitive neuroectodermal tumor refractory to treatment.
MedLine Citation:
PMID:  15543017     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
The authors report an unusual case of an infant presenting with a congenital peripheral primitive neuroectodermal tumor (PPNET) of the left hand refractory to treatment. A newborn girl was born with a large bluish-red mass of 4.5 cm diameter protruding into the palm and the dorsum of the left hand. Tumor biopsy confirmed the diagnosis of PPNET. The initial metastatic workup for the detection of metastases was negative. Four cycles of chemotherapy according to CCSG 7881/POG 8850 regimen B were given. Despite this aggressive chemotherapy the tumor grew to involve the entire left hand. The left hand was amputated, and then two cycles of topotecan/cyclophosphamide were given. Five months later extensive metastases developed, involving the brain, lungs, liver, and skeleton, and the child died at the age of 2.5 years. PPNET presenting at birth is uncommon; presentation in the hand is unusual, and the fact that it did not respond to treatment is still more uncommon.
Authors:
Mohammed El Hayek; Omar Trad; Sherief Islam
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Journal of pediatric hematology/oncology     Volume:  26     ISSN:  1077-4114     ISO Abbreviation:  J. Pediatr. Hematol. Oncol.     Publication Date:  2004 Nov 
Date Detail:
Created Date:  2004-11-15     Completed Date:  2004-12-21     Revised Date:  2011-10-06    
Medline Journal Info:
Nlm Unique ID:  9505928     Medline TA:  J Pediatr Hematol Oncol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  770-2     Citation Subset:  IM    
Affiliation:
Department of Pediatric Hematology/Oncology, Tawam University Hospital and Faculty of Medicine and Health Sciences, Al Ain, United Arab Emirates. mohhayek@emerates.net.ae
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MeSH Terms
Descriptor/Qualifier:
Amputation
Antineoplastic Combined Chemotherapy Protocols / therapeutic use*
Fatal Outcome
Female
Hand
Humans
Infant
Neoplasm Metastasis
Neuroectodermal Tumors, Primitive, Peripheral / congenital,  diagnosis*,  therapy*
Treatment Failure

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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