Retrospective study of childhood ganglioneuroma.
OBJECTIVE
To review a historical cohort of childhood ganglioneuroma (GN), the benign representative of the peripheral neuroblastic tumor (PNT) family.


METHODS
Of 2,286 PNTs enrolled between 1979 and 2005, 146 (6.4%) were registered as GN. Histological revision was carried out on 76 tumors. Diagnosis was confirmed in 45, while 27 were reclassified as ganglioneuroblastoma intermixed (GNBI) and four were reclassified as other PNT subtypes.


RESULTS
GNs differed from other PNTs for sex, age, tumor site, stage, tumor markers, and scintigraphic results. Characteristics of 76 reviewed and 70 nonreviewed patients were comparable. Reviewed GN and GNBI patients were comparable except for homovanillic acid excretion, metaiodobenzylguanidine scintigraphy, and DNA content. Seven patients were only biopsied and 139 underwent surgery. Twenty-two patients suffered surgery-related complications, of which two were fatal and seven were severe. Radical tumor resection and surgery-related complication rates were comparable for GN, GNBI, and nonreviewed instances. Six patients developed tumor progression but survived. Two patients developed a late malignancy but survived. None of the 146 patients received chemotherapy. Of 146 patients, two died of surgery-related complications and 144 survived.


CONCLUSIONS
Diagnosis was changed to GNBI for approximately one third of 76 reviewed tumors. Patients with confirmed GN, reclassified as GNBI, and nonreviewed histology presented with comparable clinical, biochemical, and biologic features. Surgical results, complication rate, number of progressions, and outcome were similar for the three groups. Surgery was associated with significant risk of complications. Survival was not influenced by extent of tumor resection. Aggressive surgical approach should not be recommended for childhood GN and GNBI.
De Bernardi
Bruno
B
Department of Pediatric Hematology-Oncology, Giannina Gaslini Children Hospital, Largo Gerolamo Gaslini 5, 16148 Genova, Italy. brunodebernardi@ospedale-gaslini.ge.it
Gambini
Claudio
C
Haupt
Riccardo
R
Granata
Claudio
C
Rizzo
Antonino
A
Conte
Massimo
M
Tonini
Gian Paolo
GP
Bianchi
Maurizio
M
Giuliano
Maria
M
Luksch
Roberto
R
Prete
Arcangelo
A
Viscardi
Elisabetta
E
Garaventa
Alberto
A
Sementa
Angela Rita
AR
Bruzzi
Paolo
P
Angelini
Paola
P
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D005260:Female; D005729:Ganglioneuroma / Q000145:classification / Q000473:pathology* / Q000601:surgery; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D011379:Prognosis; D012189:Retrospective Studies