title: Neuroblastic tumors associated with opsoclonus-myoclonus syndrome: histological, immunohistochemical and molecular features of 15 Italian cases.
abstract: The aim of this study was to investigate the histological, immunohistochemical and molecular features of a series of children with neuroblastic tumors (NTs) and opsoclonus-myoclonus syndrome (OMS). Of 1187 children (age 0-15 years) with previously untreated NTs registered between 1979 and 1995, 15 (1.3%) had OMS at presentation. The majority of patients showed favorable biological characteristics, such as lack of amplification of the neuroblastoma-associated avian myelocytomatosis homolog MYCN oncogene and aneuploid nuclear DNA content. Tumor histology was reviewed according to the International Neuroblastoma Pathology Classification. Histology of the 15 cases of NTs with OMS was ganglioneuroblastoma, intermixed, in 10 patients; ganglioneuroma, maturing, in 1; and neuroblastoma in 4. Of 15 tumors, 12 (10 ganglioneuroblastomas, 2 neuroblastomas) showed abundant interstitial or perivascular lymphoid infiltrates, the latter often organized in secondary lymphoid follicles. The three remaining cases had only minimal infiltrates. A review of 91 cases of age- and stage-matched neuroblastic tumors not associated with OMS tested as controls showed that the degree of lymphoid infiltration was significantly lower than that detected in OMS-related tumors. Furthermore, lymphoid follicles were always present in the latter tumors, whereas they were detected only in a few ganglioneuroma, intermixed tumors from the control group. In conclusion, ganglioneuroblastoma, intermixed subtype, lack of MYCN amplification, aneuploid DNA content and presence of lymphoid infiltrates may contribute to favorable prognosis in NTs associated with OMS.
authors.affiliation: Service of Pathology, Giannina Gaslini Children's Hospital, Largo Gerolamo Gaslini 5, 16148, Genova, Italy. claudiogambini@ospedale-gaslini.ge.it
authors.forename: Claudio
authors.initials: C
authors.lastname: Gambini
authors.forename: Massimo
authors.initials: M
authors.lastname: Conte
authors.forename: Gabriella
authors.initials: G
authors.lastname: Bernini
authors.forename: Paola
authors.initials: P
authors.lastname: Angelini
authors.forename: Andrea
authors.initials: A
authors.lastname: Pession
authors.forename: Paolo
authors.initials: P
authors.lastname: Paolucci
authors.forename: Alberto
authors.initials: A
authors.lastname: Donfrancesco
authors.forename: Edvige
authors.initials: E
authors.lastname: Veneselli
authors.forename: Katia
authors.initials: K
authors.lastname: Mazzocco
authors.forename: Gian Paolo
authors.initials: GP
authors.lastname: Tonini
authors.forename: Lizzia
authors.initials: L
authors.lastname: Raffaghello
authors.forename: Carlo
authors.initials: C
authors.lastname: Dominici
authors.forename: Adriana
authors.initials: A
authors.lastname: Morando
authors.forename: Francesca
authors.initials: F
authors.lastname: Negri
authors.forename: Anna
authors.initials: A
authors.lastname: Favre
authors.forename: Bruno
authors.initials: B
authors.lastname: De Bernardi
authors.forename: Vito
authors.initials: V
authors.lastname: Pistoia
mesh_terms: D000293:Adolescent; D000782:Aneuploidy; D002648:Child; D002675:Child, Preschool; D004273:DNA, Neoplasm / Q000032:analysis; D005260:Female; D005434:Flow Cytometry; D005784:Gene Amplification; D006801:Humans; D007124:Immunoenzyme Techniques; D007223:Infant; D007231:Infant, Newborn; D007558:Italy; D016246:Lymphocytes, Tumor-Infiltrating / Q000473:pathology; D008297:Male; D009447:Neuroblastoma / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D020361:Paraneoplastic Syndromes, Nervous System / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D016271:Proto-Oncogene Proteins c-myc / Q000032:analysis / Q000235:genetics; D012042:Registries
chemical_list: D004273:DNA, Neoplasm; D016271:Proto-Oncogene Proteins c-myc