title: Favorable histology, MYCN-amplified 4S neonatal neuroblastoma.
abstract: We report a neonate with 4S neuroblastoma and MYCN amplification, but favorable Shimada histology, successfully treated with chemotherapy and 13-cis-retinoic acid without stem cell transplantation. MYCN amplification in neuroblastoma is usually associated with unfavorable Shimada histology; the presence of these features in infants with 4S disease confers a poor prognosis. A small number of infants with 4S neuroblastoma and MYCN amplification have favorable Shimada histology. In this subgroup of infants, histopathology may be equally important in predicting outcome.
authors.affiliation: Division of Pediatric Hematology/Oncology, State University of New York at Stony Brook, Stony Brook, New York 11776, USA. elchan@notes.cc.sunysb.edu
authors.forename: Edward L
authors.initials: EL
authors.lastname: Chan
authors.forename: Richard E
authors.initials: RE
authors.lastname: Harris
authors.forename: Kathleen H
authors.initials: KH
authors.lastname: Emery
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Gelfand
authors.forename: Margaret H
authors.initials: MH
authors.lastname: Collins
authors.forename: Ralph A
authors.initials: RA
authors.lastname: Gruppo
mesh_terms: D000310:Adrenal Gland Neoplasms / Q000151:congenital / Q000188:drug therapy / Q000235:genetics* / Q000378:metabolism / Q000473:pathology / Q000601:surgery; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000097:blood / Q000652:urine; D016190:Carboplatin / Q000008:administration & dosage; D003520:Cyclophosphamide / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D005047:Etoposide / Q000008:administration & dosage; D005260:Female; D005784:Gene Amplification; D052138:Genes, Neoplasm; D016259:Genes, myc; D006529:Hepatomegaly / Q000209:etiology; D006801:Humans; D007231:Infant, Newborn; D015474:Isotretinoin / Q000008:administration & dosage; D008113:Liver Neoplasms / Q000097:blood / Q000151:congenital / Q000188:drug therapy / Q000556:secondary; D008207:Lymphatic Metastasis; D008279:Magnetic Resonance Imaging; D009447:Neuroblastoma / Q000151:congenital / Q000188:drug therapy / Q000235:genetics* / Q000378:metabolism / Q000473:pathology / Q000556:secondary / Q000601:surgery; D011379:Prognosis; D012074:Remission Induction; D012307:Risk Factors
chemical_list: D014408:Biomarkers, Tumor; D005047:Etoposide; D004317:Doxorubicin; D003520:Cyclophosphamide; D016190:Carboplatin; D015474:Isotretinoin