Thoracic neuroblastoma: A pediatric oncology group study

Adams, Gregg A.; Shochat, Stephen J.; Smith, E. Ide; Shuster, Jonathan J.; Joshi, Vijay V.; Altshuler, Geoffrey; Hayes, F. Ann; Nitschke, Rupert; McWilliams, Nancy B.; Castleberry, Robert P.

doi:10.1016/0022-3468(93)90234-c

Cited by 76 publications

(48 citation statements)

References 21 publications

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“…8,9 These previous studies indicate that primary tumor site may account for some of the heterogeneity in clinical features, tumor biology, and clinical outcomes in NB. Given the small size and limited scope of these previous studies, a clear understanding of the impact of primary tumor site has not been possible.…”

Section: Journal Of Clinical Oncology O R I G I N a L R E P O R T V Omentioning

confidence: 94%

“…For example, the superior unadjusted EFS and OS rates of thoracic primary tumors found in this study (80.0% and 87.6%, respectively) are comparable with those seen in single-center or cooperative group studies with overall survival rates ranging from 71.2% to 100%. 8,15,16,21 The biologically favorable profile of thoracic tumors may explain the better prognosis in these tumors. In one study, MYCN-amplified tumors with a thoracic primary were shown to have a better outcome compared with all nonthoracic NB tumors in a previous univariable survival analysis; however, a multivariable Cox analysis was not conducted in that study.…”

Section: Differences In Outcomes In Neuroblastoma By Primary Tumor Sitementioning

confidence: 99%

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Clinical, Biologic, and Prognostic Differences on the Basis of Primary Tumor Site in Neuroblastoma: A Report From the International Neuroblastoma Risk Group Project

Matthay

Neuhaus

et al. 2014

JCO

166

127

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Purpose Neuroblastoma (NB) is a heterogeneous tumor arising from sympathetic tissues. The impact of primary tumor site in influencing the heterogeneity of NB remains unclear. Patients and Methods Children younger than age 21 years diagnosed with NB or ganglioneuroblastoma between 1990 and 2002 and with known primary site were identified from the International Neuroblastoma Risk Group database. Data were compared between sites with respect to clinical and biologic features, as well as event-free survival (EFS) and overall survival (OS). Results Among 8,369 children, 47% had adrenal tumors. All evaluated clinical and biologic variables differed statistically between primary sites. The features that were > 10% discrepant between sites were stage 4 disease, MYCN amplification, elevated ferritin, elevated lactate dehydrogenase, and segmental chromosomal aberrations, all of which were more frequent in adrenal versus nonadrenal tumors (P < .001). Adrenal tumors were more likely than nonadrenal tumors (adjusted odds ratio, 2.09; 95% CI, 1.67 to 2.63; P < .001) and thoracic tumors were less likely than nonthoracic tumors (adjusted odds ratio, 0.20; 95% CI, 0.11 to 0.39; P < .001) to have MYCN amplification after controlling for age, stage, and histologic grade. EFS and OS differed significantly according to the primary site (P < .001 for both comparisons). After controlling for age, MYCN status, and stage, patients with adrenal tumors had higher risk for events (hazard ratio, 1.13 compared with nonadrenal tumors; 95% CI, 1.03 to 1.23; P = .008), and patients with thoracic tumors had lower risk for events (HR, 0.79 compared with nonthoracic; 95% CI, 0.67 to 0.92; P = .003). Conclusion Clinical and biologic features show important differences by NB primary site, with adrenal and thoracic sites associated with inferior and superior survival, respectively. Future studies will need to investigate the biologic origin of these differences.

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Section: Journal Of Clinical Oncology O R I G I N a L R E P O R T V Omentioning

confidence: 94%

Section: Differences In Outcomes In Neuroblastoma By Primary Tumor Sitementioning

confidence: 99%

Clinical, Biologic, and Prognostic Differences on the Basis of Primary Tumor Site in Neuroblastoma: A Report From the International Neuroblastoma Risk Group Project

Matthay

Neuhaus

et al. 2014

JCO

166

127

View full text Add to dashboard Cite

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“…Schiling y cols, suponen que esta hipótesis puede encontrarse en NB torácicos incompletamente resecados, el sólo encontró recurrencia local en un paciente de 13 casos operados 8 . Adams y cols, en 96 pacientes con NB torácico, 49% no tenían manifestaciones clínicas, estaban totalmente asintomáticos, mientras que el 19% tenía paraplejia por compresión medular causada por el tumor y un 3% síndrome de Horner al debut 10 . En nuestro caso se plantea que el crecimiento lento, paulatino de éste tumor hizo que se detectara fortuitamente debido a una hipertermia, sin otras manifestaciones.…”

Section: Discussionunclassified

“…La quimioterapia adyuvante antes de la cirugía es empleada para reducir los grandes volúmenes tumorales en casos avanzados como parte de la terapia multimodal, continuando con la misma asociada o no a la radioterapia sobre todo si es imposible la resección completa del tumor o ya existe extensión a otras estructuras, con lo cual se ha logrado elevar la sobrevida [9][10][11] . En nuestro país se utilizan iguales esquemas internacionales de grupos cooperativos y nacionales para el tratamiento de éstos tumores, el Dr.…”

Section: Discussionunclassified

“…Alert y cols 13 , han publicado trabajos del empleo de la radioterapia junto a la quimioterapia adyuvante para el tratamiento de diversos tumores pediátricos como es el NB con muy buenos resultados en etapas clínicas avanzadas de la enfermedad. El más efectivo protocolo para el tratamiento del NB no ha sido bien establecido aún, porque a pesar de los esfuerzos que se realizan por los equipos multidisciplinarios y esquemas multidrogas la sobrevida actual global es de un 50% en el NB avanzado [8][9][10][11][12][13] . El NB torácico estadio avanzado (etapas III y IV) después de una quimioterapia intensa y radioterapia, aunque no se haya podido resecar completamente el tumor, muestran maduración espontánea a ganglioneuroblastoma y ganglioneuroma detectados en la segunda cirugía 2,[13][14][15] .…”

Section: Discussionunclassified

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