MIBG scans in patients with stage 4 neuroblastoma reveal two metastatic patterns, one is associated with MYCN amplification and in MYCN-amplified tumours correlates with a better prognosis

Self Cite

Background Prior studies suggest that neuroblastomas that do not accumulate metaiodobenzyl-guanidine (MIBG) on diagnostic imaging (MIBG non-avid) may have more favorable features compared with MIBG avid tumors. We compared clinical features, biologic features, and clinical outcomes between patients with MIBG nonavid and MIBG avid neuroblastoma. Procedure Patients had metastatic high- or intermediate-risk neuroblastoma and were treated on Children’s Oncology Group protocols A3973 or A3961. Comparisons of clinical and biologic features according to MIBG avidity were made with chi-squared or Fisher exact tests. Event-free (EFS) and overall (OS) survival compared using log–rank tests and modeled using Cox models. Results Thirty of 343 patients (8.7%) had MIBG nonavid disease. Patients with nonavid tumors were less likely to have adrenal primary tumors (34.5 vs. 57.2%; P = 0.019), bone metastases (36.7 vs. 61.7%; P = 0.008), or positive urine catecholamines (66.7 vs. 91.0%; P < 0.001) compared with patients with MIBG avid tumors. Nonavid tumors were more likely to be MYCN amplified (53.8 vs. 32.6%; P =0.030) and had lower norepinephrine transporter expression. Patients with MIBG non-avid disease had a 5-year EFS of 50.0% compared with 38.7% for patients with MIBG avid disease (P = 0.028). On multivariate testing in high-risk patients, MIBG avidity was the sole adverse prognostic factor for EFS identified (hazard ratio 1.77; 95% confidence interval 1.04–2.99; P = 0.034). Conclusions Patients with MIBG nonavid neuroblastoma have lower rates of adrenal primary tumors, bone metastasis, and catecholamine secretion. Despite being more likely to have MYCN-amplified tumors, these patients have superior outcomes compared with patients with MIBG avid disease.

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

MIBG avidity correlates with clinical features, tumor biology, and outcomes in neuroblastoma: A report from the Children's Oncology Group

DuBois

Mody

Naranjo

et al. 2017

Self Cite

“…Though several potential biologic markers for substratification of risk in stage M neuroblastoma have been identified, their precise relationship to disease progression remains unclear . Patterns of metastasis may provide insight into biologic mechanisms of disease progression and provide a basis for imaging‐based risk stratification in high‐risk, stage M neuroblastoma …”

Section: Discussionmentioning

confidence: 99%

“…[4,18,19] Patterns of metastasis may provide insight into biologic mechanisms of disease progression and provide a basis for imaging-based risk stratification in high-risk, stage M neuroblastoma. [10,20,21] Bone metastasis is associated with poor prognosis in neuroblastoma and is present in most children with stage M disease. [2,3,10] In this study, we found that while extraorbital bone metastasis conferred no additional risk in children ࣙ18 months of age with stage M (high-risk) disease, the presence of orbital bone metastasis was associated with decreased overall and 5YS postdiagnosis of neuroblastoma.…”

Section: Discussionmentioning

confidence: 99%

Orbital Metastasis Is Associated With Decreased Survival in Stage M Neuroblastoma

Harreld

Bratton²,

Federico

et al. 2015

Background Approximately 30% of patients with metastatic (stage M) neuroblastoma present with periorbital ecchymosis from orbital osseous disease. Though locoregional disease is staged by imaging, the prognostic significance of metastatic site in stage M disease is unknown. We hypothesize that, compared to non-orbital metastasis, orbital metastasis is associated with decreased survival in patients with stage M neuroblastoma, and that periorbital ecchymosis reflects location and extent of orbital disease. Procedure Medical records and imaging from 222 patients with stage M neuroblastoma seen at St. Jude Children’s Research Hospital between January 1995 and May 2009 were reviewed. Thirty-seven patients were <18 months of age at diagnosis and 185 were ≥18 months. Overall (OS) and 5-year survival (5YS) were compared for patients with and without orbital, calvarial and non-orbital osseous metastasis, and with and without periorbital ecchymosis (log-rank test). Associations of periorbital ecchymosis with orbital metastasis location/extent were explored (fisher’s exact test, t-test). Results In patients ≥18 months of age, only orbital metastasis was associated with decreased 5YS (p=0.0323) and OS (p=0.0288). In patients <18 months of age, neither orbital, calvarial or non-orbital bone metastasis was associated with OS or 5YS. Periorbital ecchymosis was associated with higher number of involved orbital bones (p=0.0135), but not location or survival. Conclusions In patients ≥ 18 months of age with stage M neuroblastoma, orbital metastatic disease is associated with decreased 5YS and OS. In future clinical trials, orbital disease may be useful as an imaging-based risk factor for sub-stratification of stage M neuroblastoma.

“…1 The pattern and extent of metastatic disease (number of systems/compartments involved) is also prognostic in patients with high-risk metastatic disease. [3][4][5] The presence of tumour segmental chromosomal abnormalities (SCA) 6 and pattern of metaiodobenzylguanidine (mIBG) uptake (particularly in patients with MNA tumours) 7 also have prognostic impact. The extent of mIBG-avid disease (assessed using semi-quantitative scoring) at diagnosis and after induction chemotherapy correlates with outcome.…”

Section: Introductionmentioning

confidence: 99%

Risk stratification of high‐risk metastatic neuroblastoma: A report from the HR‐NBL‐1/SIOPEN study

Morgenstern

Pötschger

Moreno

et al. 2018

Self Cite