Pediatric Malignant Peripheral Nerve Sheath Tumor: The Italian and German Soft Tissue Sarcoma Cooperative Group

Carli, Modesto; Ferrari, Andrea; Mattke, Adrian; Zanetti, Ilaria; Casanova, Michela; Bisogno, Gianni; Cecchetto, Giovanni; Alaggio, Rita; Sio, Luigi De; Koscielniak, E.; Sotti, Guido; Treuner, J.

doi:10.1200/jco.2005.01.4886

Cited by 312 publications

(381 citation statements)

References 19 publications

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“…The present subset of extremity cases confirms our main findings in NRSTS of all sites, reported elsewhere [9][10][11], i.e., quite a satisfactory overall outcome (5-year OS 84%), the prognostic role of IRS grouping, grade of malignancy, tumor size and local invasiveness, the poor prognosis for patients with the MPNST histotype [12] and with metastases at diagnosis. It is worth noting that treatment for extremity NRSTS proved more effective over the years, while this was not the case for RMS.…”

Section: Discussionsupporting

confidence: 91%

Soft-tissue sarcomas of the extremities in patients of pediatric age

Casanova

Meazza

Gronchi

et al. 2007

Journal of Children's Orthopaedics

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Purpose The extremity site is a peculiar location for softtissue sarcomas (STS) of children and adolescents. Here we report the experience of the Pediatric Oncology Unit of the Istituto Nazionale Tumori of Milan, Italy, concerning 204 patients with STS of the limbs treated between 1977 and 2006. Methods The study series included 52 patients with rhabdomyosarcoma (RMS)(65% of which were of the alveolar subtype), nine with extraosseous Ewing sarcoma and 143 with non-rhabdomyosarcoma soft-tissue sarcomas (NRSTS), 38% of which were synovial sarcoma. Patients were treated with a multimodality approach including surgery, chemotherapy, and radiotherapy. Results For the RMS patients, the 5-year event-free survival (EFS) rate was 37.1%, with distant metastases being the main cause of treatment failure. The outcome was particularly poor for patients with large invasive tumors, hand/foot involvement and/or nodal or distant metastases, and for patients who were not given radiotherapy. For the extraosseous Ewing sarcoma cases, 5-year EFS was 74%. For the NRSTS cases, the 5-year EFS was 72.6%: tumor size and local invasiveness, tumor grade, malignant peripheral nerve sheath tumor (MPNST) histology, and distant metastases were the main prognostic factors. Discussion While the limbs are the most common sites of NRSTS and are often characterized by a more favorable prognosis than for axial tumors, the clinical features of extremity RMS often differ from those of RMS of other sites, with a higher incidence of unfavorable prognostic factors (e.g., alveolar subtype) and consequently unsatisfactory treatment results. The treatment of these patients is complex and necessarily multidisciplinary, and it demands not only adequate experience of treating children and adolescents in clinical trials, but also particular skills in the field of orthopedic surgery.

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Section: Discussionsupporting

confidence: 91%

Soft-tissue sarcomas of the extremities in patients of pediatric age

Casanova

Meazza

Gronchi

et al. 2007

Journal of Children's Orthopaedics

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“…In vivo, doxorubicin also showed no effect on established tumors and no added benefit to RAD001 alone. This result is consistent with the generally poor response to chemotherapy shown by MPNST patients (1). In combination with RAD001, doxorubicin did not show significant added benefit when cell viability was assayed.…”

Section: Discussionsupporting

confidence: 79%

“…MPNSTs are treated by resection of the tumor followed by treatment with chemotherapeutic agents, including anthracyclines and alkylating agents. A retrospective study of patients treated with various chemotherapeutics found that the use of chemotherapy increased overall and event-free survival in MPNSTs (1). However, the 5-year survival for patients with unresectable tumors and metastatic MPNST was 30% and patients with NF1 had lower response rate than those with sporadic cases (17.6% versus 55%).…”

Section: Introductionmentioning

confidence: 99%

“…However, the 5-year survival for patients with unresectable tumors and metastatic MPNST was 30% and patients with NF1 had lower response rate than those with sporadic cases (17.6% versus 55%). Improved survival in sporadic cases of MPNST may result from earlier detection and/or distinct genetic alterations that underlie tumorigenesis (1). Preclinical models using human MPNST cells would be helpful to screen and compare targeted therapeutics and chemotherapeutics; however, comparisons among agents have not been carried out.…”

Section: Introductionmentioning

confidence: 99%

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Effective in vivo targeting of the mammalian target of rapamycin pathway in malignant peripheral nerve sheath tumors

Johansson¹,

Mahller

Collins³

et al. 2008

Molecular Cancer Therapeutics

117

121

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Malignant peripheral nerve sheath tumors (MPNST) are chemoresistant sarcomas with poor 5-year survival that arise in patients with neurofibromatosis type 1 (NF1) or sporadically. We tested three drugs for single and combinatorial effects on collected MPNST cell lines and in MPNST xenografts. The mammalian target of rapamycin complex 1 inhibitor RAD001 (Everolimus) decreased growth 19% to 60% after 4 days of treatment in NF1 and sporadic-derived MPNST cell lines. Treatment of subcutaneous sporadic MPNST cell xenografts with RAD001 significantly, but transiently, delayed tumor growth, and decreased vessel permeability within xenografts. RAD001 combined with the epidermal growth factor receptor tyrosine kinase inhibitor erlotinib caused additional inhibitory effects on growth and apoptosis in vitro, and a small but significant additional inhibitory effect on MPNST growth in vivo that were larger than the effects of RAD001 with doxorubicin. RAD001 plus erlotinib, in vitro and in vivo, reduced phosphorylation of AKT and total AKT levels, possibly accounting for their additive effect. The results support the consideration of RAD001 therapy in NF1 patient and sporadic MPNST. The preclinical tests described allow rapid screening strata for drugs that block MPNST growth, prior to tests in more complex models, and should be useful to identify drugs that synergize with

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“…The effectiveness of adjuvant chemotherapy for MPNST remains controversial [7]. However, it is worth noting that relatively high overall response rates (45%) have been found in paediatric patients with MPSNTs who received adjuvant chemotherapy [14]. Nevertheless, the efficacy of adjuvant chemotherapy in the treatment of MPNSTs warrants further investigation in a large-scale, multicenter, prospective trial.…”

Section: Discussionmentioning

confidence: 99%

Malignant peripheral nerve sheath tumours of the spine: clinical manifestations, classification, treatment, and prognostic factors

Zhu

Liu

et al. 2011

Eur Spine J

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Background and objectives To summarise our experience treating patients with spinal malignant peripheral nerve sheath tumours (MPNSTs). Methods We retrospectively reviewed the records of patients diagnosed with spinal MPNSTs who received surgical treatment from January 1998 to December 2009. Results Postoperative follow-up data were available for 14/16 patients with spinal MPNSTs (7 men, 7 women; median age = 44 years [range: 23-68 years]). Eight of 14 (57.1%) patients had primary and 6/14 (42.9%) recurrent MPNSTs. A total of 12/14 (85.7%) patients underwent total tumour resection, whereas 2/14 (14.3%) patients underwent subtotal tumour resection. Malignancies were graded low in 4 (28.6%) and high in 10 (71.1%) cases. A total of 12/14 (85.7%) patients experienced tumour recurrence and 10/14 (71.4%) patients died during the course of follow-up. The 0.5-1-, 3-, and 5-year survival rates were 64.3, 48.2, 32.1, and 21.4%, respectively. Overall survival was significantly associated with tumour malignant degree (P = 0.012).Conclusion Diagnosis of spinal MPNSTs should be made with reference to clinical, radiological, and pathological findings. Surgical resection is the best available option for treating spinal MPNST; however, postoperative prognosis is poor.

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Pediatric Malignant Peripheral Nerve Sheath Tumor: The Italian and German Soft Tissue Sarcoma Cooperative Group

Cited by 312 publications

References 19 publications

Soft-tissue sarcomas of the extremities in patients of pediatric age

Soft-tissue sarcomas of the extremities in patients of pediatric age

Effective in vivo targeting of the mammalian target of rapamycin pathway in malignant peripheral nerve sheath tumors

Malignant peripheral nerve sheath tumours of the spine: clinical manifestations, classification, treatment, and prognostic factors

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