Natural History and Biology of Stage A Neuroblastoma: A Pediatric Oncology Group Study

Alvarado, Caupolicán; Wb, London; At, Look; Gm, Brodeur; Dh, Altmiller; Ps, Thorner; Vv, Joshi; St, Rowe; Mb, Nash; Ei, Smith; Rp, Castleberry; Cohn, Susan L.

doi:10.1097/00043426-200005000-00003

Cited by 94 publications

(48 citation statements)

References 37 publications

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“…Overall, 411 out of 905 enrolled patients were classified as either INSS stage 1 or stage 2 without MYCNA. The 288 stage 1 patients in this series did very well and had an OS of 98.9%; this is in line with previous literature reports ( De Bernardi et al, 1995;Evans et al, 1996;Kushner et al, 1996;Alvarado et al, 2000;Perez et al, 2000), and confirms that neuroblastomas that are resected macroscopically and have normal MYCN status do not need adjuvant therapy and are commonly salvageable in the event of relapse. Indeed, only one of three deaths was due to disease progression, whereas the other two were related to treatment.…”

Section: Discussionsupporting

confidence: 91%

“…The remaining children present with localised disease and have an overall better prognosis, mainly depending on the degree of tumour resection (Cecchetto et al, 1983;von Schweinitz et al, 2000). Indeed, completely resected tumours (INSS stage 1) (Brodeur et al, 1993) rarely relapse and do not usually undergo postoperative chemotherapy (Kushner et al, 1996;Alvarado et al, 2000). By contrast, localised but unresectable neuroblastomas (INSS stage 3) require primary chemotherapy before surgical excision is attempted, and in any case have a greater likelihood of relapse and a lower cure rate (Garaventa et al, 2002).…”

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confidence: 99%

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Treatment of localised resectable neuroblastoma. Results of the LNESG1 study by the SIOP Europe Neuroblastoma Group

et al. 2008

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Main objective of this study was to confirm that surgery alone is an effective and safe treatment for localised resectable neuroblastoma except stage 2 with amplified MYCN gene (MYCNA). Of 427 eligible stages 1 -2 patients, 411 had normal MYCN and 16 had MYCNA. Of the 288 stage 1 patients with normal MYCN, 1 died of complications and 16 relapsed, 2 of whom died; 5-year relapse-free survival (RFS) and overall survival (OS) rates were 94.3% (95% confidence interval (CI): 91.6 -97) and 98.9% (95% CI: 97.7 -100), respectively. Of the 123 stage 2 patients with normal MYCN, 1 died of sepsis and 22 relapsed, 8 of whom died (RFS 82.8%, 95% CI: 76.2 -89.5; OS 93.2%, 95% CI: 88.7 -97.8). In stage 2, OS and RFS were worse for patients with elevated LDH and unfavourable histopathology. Of 16 children with MYCNA, 7 were stage 1 (5 relapses and 4 deaths) and 9 were stage 2 (3 relapses and 2 deaths) patients. In conclusion, surgery alone yielded excellent OS for both stage 1 and 2 neuroblastoma without MYCNA, although stage 2 patients with unfavourable histopathology and elevated LDH suffered a high number of relapses. Both stage 1 and 2 patients with MYCNA were at greater risk of relapse.

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Section: Discussionsupporting

confidence: 91%

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confidence: 99%

Treatment of localised resectable neuroblastoma. Results of the LNESG1 study by the SIOP Europe Neuroblastoma Group

et al. 2008

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“…The INRG classification system was developed based on analysis of 13 clinical and biological variables for effects on event-free survival rates, including patient age at diagnosis, INRGSS tumor stage, serum lactate dehydrogenase and ferritin levels, tumor histological category, grade of differentiation, tumor [51,52] (Figure 3). For those patients with localized, resectable disease, surgical resection alone is generally curative, and chemotherapy is effective salvage for those with relapses after resection [53][54][55]. Low-stage tumors with favorable biological features often do not metastasize even after incomplete resection, and therefore, for these patients, chemotherapy is reserved for those with life or organ-threatening symptoms, such as spinal cord compression or respiratory compromise.…”

Section: Prognosis and Risk Stratificationmentioning

confidence: 99%

Overview and recent advances in the treatment of neuroblastoma

Whittle

Smith

Doherty

et al. 2017

Expert Review of Anticancer Therapy

311

258

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Introduction: Children with neuroblastoma have widely divergent outcomes, ranging from cure in >90% of patients with low risk disease to <50% for those with high risk disease. Recent research has shed light on the biology of neuroblastoma, allowing for more accurate risk stratification and treatment reduction in many cases, although newer treatment strategies for children with high-risk and relapsed neuroblastoma are needed to improve outcomes. Areas covered: Neuroblastoma epidemiology, diagnosis, risk stratification, and recent advances in treatment of both newly diagnosed and relapsed neuroblastoma. Expert commentary: The identification of newer tumor targets and of novel cell-mediated immunotherapy agents may lead to novel therapeutic approaches, and clinical trials for regimens designed to target individual genetic aberrations in tumors are underway. A combination of therapeutic modalities will likely be required to improve survival and cure rates for patients with high-risk neuroblastoma.ARTICLE HISTORY

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“…Numbers of patients with stage 1 or 2 MYCN-amplified neuroblastoma enrolled on previous cooperative group treatment studies have been small, [6][7][8][9] thus it has been difficult to develop an evidence-based therapeutic strategy for this group. To establish the basis for a rational therapeutic approach to low-stage neuroblastoma with unfavorable biologic features, information regarding outcomes for these patients was obtained from the International Neuroblastoma Risk Group (INRG) database.…”

Section: Introductionmentioning

confidence: 99%

Significance of MYCN Amplification in International Neuroblastoma Staging System Stage 1 and 2 Neuroblastoma: A Report From the International Neuroblastoma Risk Group Database

Bagatell

Beck‐Popovic

London

et al. 2009

JCO

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122

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A B S T R A C T PurposeTreatment of patients with localized neuroblastoma with unfavorable biologic features is controversial. To evaluate the outcome of children with low-stage MYCN-amplified neuroblastoma and develop a rational treatment strategy, data from the International Neuroblastoma Risk Group (INRG) database were analyzed. Patients and MethodsThe database is comprised of 8,800 patients. Of these, 2,660 patients (30%) had low-stage (International Neuroblastoma Staging System stages 1 and 2) neuroblastoma, known MYCN status, and available follow-up data. Eighty-seven of these patients (3%) had MYCN amplified tumors. Results Patients with MYCN-amplified, low-stage tumors had less favorable event-free survival (EFS)and overall survival (OS) than did patients with nonamplified tumors (53% Ϯ 8% and 72% Ϯ 7% v 90% Ϯ 1% and 98% Ϯ 1%, respectively). EFS and OS were statistically significantly higher for patients whose tumors were hyperdiploid rather than diploid (EFS, 82% Ϯ 20% v 37% Ϯ 21%; P ϭ .0069; OS, 94% Ϯ 11% v 54% Ϯ 15%; P ϭ .0056, respectively). No other variable had prognostic significance. Initial treatment consisted of surgery alone for 29 (33%) of 87 patients. Details of additional therapy were unknown for 14 patients. Twenty-two patients (25%) underwent surgery and moderateintensity chemotherapy; another 22 underwent surgery, intensive chemotherapy, and radiation therapy. Nine of the latter 22 underwent stem cell transplantation. Survival in patients who received transplantation did not differ from survival in those who did not receive transplantation. ConclusionAmong patients with low-stage, MYCN-amplified neuroblastoma, outcomes of patients with hyperdiploid tumors were statistically, significantly better than those with diploid tumors. The data suggest that tumor cell ploidy could potentially be used to identify candidates for reductions in therapy. Further study of MYCN-amplified, low-stage neuroblastoma is warranted.

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Natural History and Biology of Stage A Neuroblastoma: A Pediatric Oncology Group Study

Cited by 94 publications

References 37 publications

Treatment of localised resectable neuroblastoma. Results of the LNESG1 study by the SIOP Europe Neuroblastoma Group

Treatment of localised resectable neuroblastoma. Results of the LNESG1 study by the SIOP Europe Neuroblastoma Group

Overview and recent advances in the treatment of neuroblastoma

Significance of MYCN Amplification in International Neuroblastoma Staging System Stage 1 and 2 Neuroblastoma: A Report From the International Neuroblastoma Risk Group Database

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