Management and prognosis of malignant peripheral nerve sheath tumors: The experience of the French Sarcoma Group (GSF-GETO)

Valentin, Thibaud; Cesne, Axel Le; Ray‐Coquard, Isabelle; Italiano, Antoîne; Decanter, Gauthier; Bompas, Emmanuelle; Isambert, Nicolás; Thariat, Juliette; Linassier, Claude; Bertucci, François; Bay, J.-O.; Bellesœur, Audrey; Penel, Nicolas; Guellec, Sophie Le; Filleron, Thomas; Chevreau, Christine

doi:10.1016/j.ejca.2015.12.015

Cited by 120 publications

(183 citation statements)

References 18 publications

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“…For benign tumours resection with minimisation of residual neurological deficit is the aim, and in many cases can result in improvement in peripheral nerve function [159]. Malignant peripheral nerve sheath tumours (MPNST) are aggressive tumours with a relatively poor prognosis [160]. In general management is as for malignant soft-tissue sarcomas as described earlier in this guideline.…”

Section: Borderline Tumoursmentioning

confidence: 99%

UK guidelines for the management of soft tissue sarcomas

Dangoor¹,

et al. 2016

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Soft tissue sarcomas (STS) are rare tumours arising in mesenchymal tissues, and can occur almost anywhere in the body. Their rarity, and the heterogeneity of subtype and location means that developing evidence-based guidelines is complicated by the limitations of the data available. However, this makes it more important that STS are managed by teams, expert in such cases, to ensure consistent and optimal treatment, as well as recruitment to clinical trials, and the ongoing accumulation of further data and knowledge. The development of appropriate guidance, by an experienced panel referring to the evidence available, is therefore a useful foundation on which to build progress in the field. These guidelines are an update of the previous version published in 2010 (Grimer et al. in Sarcoma 2010:506182, 2010). The original guidelines were drawn up following a consensus meeting of UK sarcoma specialists convened under the auspices of the British Sarcoma Group (BSG) and were intended to provide a framework for the multidisciplinary care of patients with soft tissue sarcomas. This current version has been updated and amended with reference to other European and US guidance. There are specific recommendations for the management of selected subtypes of disease including retroperitoneal and uterine sarcomas, as well as aggressive fibromatosis (desmoid tumours) and other borderline tumours commonly managed by sarcoma services. An important aim in sarcoma management is early diagnosis and prompt referral. In the UK, any patient with a suspected soft tissue sarcoma should be referred to one of the specialist regional soft tissues sarcoma services, to be managed by a specialist sarcoma multidisciplinary team. Once the diagnosis has been confirmed using appropriate imaging, plus a biopsy, the main modality of management is usually surgical excision performed by a specialist surgeon. In tumours at higher risk of recurrence or metastasis pre- or post-operative radiotherapy should be considered. Systemic anti-cancer therapy (SACT) may be utilized in some cases where the histological subtype is considered more sensitive to systemic treatment. Regular follow-up is recommended to assess local control, development of metastatic disease, and any late-effects of treatment. For local recurrence, and more rarely in selected cases of metastatic disease, surgical resection would be considered. Treatment for metastases may include radiotherapy, or systemic therapy guided by the sarcoma subtype. In some cases, symptom control and palliative care support alone will be appropriate.

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Section: Borderline Tumoursmentioning

confidence: 99%

UK guidelines for the management of soft tissue sarcomas

Dangoor¹,

et al. 2016

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“…High-grade MPNST has the highest risk of sarcoma-specific death [7]. The 5-year overall survival is approximately 20% to 50% [8–10], and outcome is especially dismal in those with unresectable or metastatic disease [11]. Most clinicopathologic series of MPNSTs report NF1-associated MPNSTs to be approximately equally common as sporadic cases [8,11–15].…”

mentioning

confidence: 99%

Histopathologic evaluation of atypical neurofibromatous tumors and their transformation into malignant peripheral nerve sheath tumor in patients with neurofibromatosis 1—a consensus overview

et al. 2017

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Neurofibromatosis 1 (NF1) patients develop multiple neurofibromas, with 8–15% of patients experiencing malignant peripheral nerve sheath tumor (MPNST) during their lifetime. Prediction of transformation, typically from plexiform neurofibroma, is clinically and histologically challenging. In this overview, following a consensus meeting in October 2016, we outline the histopathologic features and molecular mechanisms involved in the malignant transformation of neurofibromas. Nuclear atypia alone is generally insignificant. However, with atypia, loss of neurofibroma architecture, high cellularity, and/or mitotic activity >1/50 but <3/10 high power fields, the findings are worrisome for malignancy. We propose the term “atypical neurofibromatous neoplasms of uncertain biologic potential (ANNUBP)” for lesions displaying at least two of these features. This diagnosis should prompt additional sampling, clinical correlation, and possibly, expert pathology consultation. Currently such tumors are diagnosed inconsistently as atypical neurofibroma or low-grade MPNST. Most MPNSTs arising from neurofibromas are high-grade sarcomas and pose little diagnostic difficulty, although rare non-necrotic tumors with 3–9 mitoses/10 HPFs can be recognized as low-grade variants. While neurofibromas contain numerous S100 protein/SOX10-positive Schwann cells and CD34-positive fibroblasts, both components are reduced or absent in MPNST. Loss of p16/CDKN2A expression, elevated Ki67 labeling, and extensive nuclear p53 positivity are also features of MPNST that can to some degree already occur in ANNUBP. Complete loss of trimethylated histone 3 lysine 27 (H3K27me3) expression is potentially more reliable, being immunohistochemically detectable in about half of MPNSTs. Correlated clinicopathologic, radiologic, and genetic studies should increase our understanding of malignant transformation in neurofibromas, hopefully improving diagnosis and treatment soon.

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“…For example, the efficacy of eribulin, an inhibitor of microtubule dynamics, was studied in a randomized phase 3 trial in previously treated patients with liposarcoma or leiomyosarcoma. 26 Earlier studies have reported worse prognosis in patients with NF1-related MPNST. This was expected because we had included patients up to age 80 years.…”

Section: Discussionmentioning

confidence: 98%

“…19 In general, OS was worse in OA. 27 In the study by Valentin et al, 26 a worse prognosis was demonstrated for patients with NF1-related MPNST, but this was concluded as being due to different clinical features at the time of diagnosis rather than NF1 status. However, we also found that the older age group was more likely to experience a disease recurrence.…”

Section: Discussionmentioning

confidence: 99%

Soft‐tissue sarcoma in adolescents and young adults compared with older adults: A report among 5000 patients from the Scandinavian Sarcoma Group Central Register

Papworth

Arroyo

Styring

et al. 2019

Cancer

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BACKGROUND: In recent years, there has been growing awareness of the distinct characteristics of adolescents and young adults (AYA) diagnosed with cancer. Soft-tissue sarcoma (STS) accounts for approximately 1% of all cancers diagnosed in adults and 8% of cancers diagnosed in AYA. To the best of our knowledge, only a few sarcoma registers include data regarding histological subtype, age at diagnosis, and detailed clinical information. Therefore, little is known regarding clinical presentation and outcomes in AYA diagnosed with STS. METHODS: Using the Scandinavian Sarcoma Group Central Register, data were obtained regarding 4977 patients who were diagnosed with STS for the period between 1986 and 2011. AYA (those aged 18-39 years) were compared with older adults (OA; those aged 40-80 years) with respect to clinical presentation, treatment, and outcome. RESULTS: There were 868 AYA and 4109 OA. Overall and by STS subtype, there were significant differences noted between AYA and OA with regard to presentation, treatment, and survival. The distribution of STS subtypes was different between OA and AYA (eg, OA were more likely to be diagnosed with malignant fibrous histiocytoma compared with AYA [34% vs 16%; P < .001]). OA also were more likely to have tumors measuring ≥5 cm (68% vs 56%; P < .001) and a higher malignancy grade (75% vs 67%; P < .001). In the majority of STS subtypes AYA had significantly better overall survival and less disease recurrence compared with OA, but this finding was not true for those with malignant peripheral nerve sheath tumors. CONCLUSIONS: There are several differences between AYA and OA with STS with regard to presentation, treatment, and survival, and such differences must be taken into consideration when designing clinical trials. Additional work also is needed to characterize the potential biological mechanisms underlying these differences. Cancer 2019;125:3595-3602.

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Management and prognosis of malignant peripheral nerve sheath tumors: The experience of the French Sarcoma Group (GSF-GETO)

Cited by 120 publications

References 18 publications

UK guidelines for the management of soft tissue sarcomas

UK guidelines for the management of soft tissue sarcomas

Histopathologic evaluation of atypical neurofibromatous tumors and their transformation into malignant peripheral nerve sheath tumor in patients with neurofibromatosis 1—a consensus overview

Soft‐tissue sarcoma in adolescents and young adults compared with older adults: A report among 5000 patients from the Scandinavian Sarcoma Group Central Register

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