Salvage rates and prognostic factors after relapse in children and adolescents with malignant peripheral nerve sheath tumors

Bergamaschi, Luca; Bisogno, Gianni; Manzitti, Carla; D’Angelo, Paolo; Milano, Giuseppe Maria; Scagnellato, Angela; Cappelletti, Mirko; Chiaravalli, Stefano; Dall’Igna, Patrizia; Alaggio, Rita; Ruggiero, Antonio; Martino, Martina Di; Affinita, Maria Carmen; Pierobon, Marta; Garaventa, Alberto; Casanova, Michela; Ferrari, Andrea

doi:10.1002/pbc.26816

Cited by 19 publications

(19 citation statements)

References 42 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Actually, 5-year OS rate was more than 50% in studies [15,[24][25][26] in which the participants were more than 300. For an Italian study focusing on pediatric patients (n=73) with relapsing MPNST, Bergamaschi, L. et al [28] reported that the survival rate was 15.8% at 5 years which was the lowest rate in our included studies. For non-NF 1 MPNST children (n=44) in the Netherlands, Martin, E. et al [42] showed the highest 5-year survival rate (75.8%) in these cohorts.…”

Section: -Year Os Rate 5-year Efs and Lr Ratementioning

confidence: 59%

“…Among sarcomas, MPNST has the highest recurrence rate [44]. Bergamaschi, L. et al [28] reported the highest LR rate of 86.3% in 73 patients, with 64.4% only local recurrence and 21.9% local and metastatic recurrence of MPNST. Keizman, D. et al [37] investigated 46 patients with MPNST, indicating 13% of patients occurred local recurrence with follow-up ranging from 3 to 120 months.…”

Section: -Year Os Rate 5-year Efs and Lr Ratementioning

confidence: 99%

See 1 more Smart Citation

Prognosis and Risk Factors for Malignant Peripheral Nerve Sheath Tumor: A Systematic Review and Meta-analysis

Cai

Tang

Liang

et al. 2020

Preprint

View full text Add to dashboard Cite

Background: No available meta-analysis was printed to systematically introduce the MPNST clinic outcome and risk factors based on largely pooled data. This systematic review and meta-analysis aimed to investigate 5-year OS rate, 5-year EFS rate and LR rate for MPNST, and to assess potential risk factors for prognosis.Methods: Electronic articles published between January 1, 1966 and February 29, 2020, were searched and critically evaluated. The authors independently reviewed the abstracts and extracted data for 5-year OS rate, 5-year EFS rate, LR rate, and potential risk factors for prognosis.Results: Twenty-eight literatures were finally included for meta-analysis. The pooled 5-year OS rate, 5-year EFS rate, and LR rate were 49%, 37%, and 37%, respectively. The significant prognostic factors for survival were NF-1 status, tumor size, depth, location, malignant grade, margin status, chemotherapy, and radiotherapy. Age and sex were not associated with survival.Conclusion: Survival and local recurrence of MPNST are poor. Worse prognosis is mainly associated with NF 1 mutation, large size, deep to fascia, high grade, metastases and location (trunk and head & neck). Complete resection with adequate surgical margins is the mainstay protective factor of MPNST patients, following necessary adjuvant therapies.

show abstract

Section: -Year Os Rate 5-year Efs and Lr Ratementioning

confidence: 59%

Section: -Year Os Rate 5-year Efs and Lr Ratementioning

confidence: 99%

Prognosis and Risk Factors for Malignant Peripheral Nerve Sheath Tumor: A Systematic Review and Meta-analysis

Cai

Tang

Liang

et al. 2020

Preprint

View full text Add to dashboard Cite

show abstract

“…One possible reason why the survival rates of AYA patients with MPNST were poorer than those of the other age groups is that a larger part of AYA patients with MPNST may have neurofibromatosis type 1 (NF1). MPNST patients with NF1 have been reported to be significantly younger at the time of MPNST diagnosis than those with sporadic tumors (median age, 26 years vs. 53 years) and have poor outcomes [16,17]. Thus, the mean age of MPNST patients with NF1 is within the AYA age group.…”

Section: Discussionmentioning

confidence: 99%

Descriptive Epidemiology and Outcomes of Soft Tissue Sarcomas in Adolescent and Young Adult Patients in Japan

Fukushima

Akiyama²,

Takeshita

et al. 2020

Preprint

View full text Add to dashboard Cite

Background: Compared to young children or older adults, the prognoses of adolescent and young adult (AYA) patients with cancer, i.e., those aged from 15 to 39 years, have not improved. In this study, we focused on soft tissue sarcoma (STS) in AYA patients and aimed to determine whether there is a correlation between the AYA age group and overall poor cancer survival in STS. We further aimed to determine which histologic subtypes are more common in AYA patients and investigate the cause of poor outcomes in this group.Methods: The medical records of 5853 Japanese patients diagnosed with STS between 2006 and 2013 were accessed from the Bone and Soft Tissue Tumor registry (BSTT). We analyzed and compared the epidemiological features of AYA patients with those of other age groups. The cancer survival rates were calculated using the Kaplan-Meier method. Cox proportional hazards models were used to analyze the prognostic factors for cancer survival. The primary endpoint for prognosis was the occurrence of tumor-related death.Results: On multivariate analysis, age was not a prognostic factor for poor cancer survival among these patients. Compared to the same categories in other age groups, the proportions of myxoid/round cell liposarcomas, synovial sarcomas, malignant peripheral nerve sheath tumors (MPNST), primitive neuroectodermal tumor, and rhabdomyosarcoma in AYA patients were the highest, but none of the categories were significantly more prevalent in AYA patients. The cancer survival rates of AYA patients with MPNST were poorer than those of the other age groups; however, AYA age was not a prognostic factor on multivariate analysis in MPNST patients.Conclusions: Our study is the first to investigate STS in AYA patients using the nationwide BSTT registry. Our findings demonstrate that AYA age is not a prognostic factor for poor cancer survival among those with STS in Japan.

show abstract

“…The picture, therefore, remains bleak for MPNST, and while Bergamaschi and colleagues demonstrate the ability to rescue some patients who relapse, this is limited to tiny numbers of patients . The message from these tumors is therefore “get it right first time around.” This is possible, but only if the patient presents early with a small tumor or, indeed, if current and future programs in screening NF1 patients with DNLs lead to the removal of these early premalignant lesions.…”

mentioning

confidence: 99%

“…[1][2][3][4][5] They conclude that this is possible, albeit in small numbers, in particular if a radical second operation is performed leading to an R0 resection. 1 Perhaps, however, this paper deflects attention from a more important message that pediatric MPNSTs continue to have an extremely poor outcome unless they are small, localized, and resectable. 4,6 Indeed, the median overall survival in the pediatric age group is 30 months, which decreases in the older adolescent group to 23 months.…”

mentioning

confidence: 99%

Malignant peripheral nerve sheath tumor: The need to get it right first time around

Brennan

2017

Pediatric Blood & Cancer

View full text Add to dashboard Cite

Salvage rates and prognostic factors after relapse in children and adolescents with malignant peripheral nerve sheath tumors

Cited by 19 publications

References 42 publications

Prognosis and Risk Factors for Malignant Peripheral Nerve Sheath Tumor: A Systematic Review and Meta-analysis

Prognosis and Risk Factors for Malignant Peripheral Nerve Sheath Tumor: A Systematic Review and Meta-analysis

Descriptive Epidemiology and Outcomes of Soft Tissue Sarcomas in Adolescent and Young Adult Patients in Japan

Malignant peripheral nerve sheath tumor: The need to get it right first time around

Contact Info

Product

Resources

About