Malignant Peripheral Nerve Sheath Tumor

James, Aaron W.; Shurell, Elizabeth; Singh, Arun S.; Dry, Sarah M.; Eilber, Fritz C.

doi:10.1016/j.soc.2016.05.009

Cited by 134 publications

(181 citation statements)

References 56 publications

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“…The quantitative FDG-PET imaging combined with CT or MRI may be the best way to distinguish MPNST from benign PNST. However, radiographic imaging of MPNST has not supplanted histopathologic examination as the gold standard for the diagnosis of MPNST [16]. In our case, the CECT of the abdomen revealed an intussusception in the ascending colon with a smooth enhancing 3.5 cm mass suspicious for neoplasm, but could not confirm its definite lesion.…”

Section: Discussionmentioning

confidence: 68%

“…It is important to recognize that there is still a lack of widely accepted diagnostic criteria for MPNST [16]. These tumors have well-described morphological heterogeneity, and staining reveals highly cellular spindle cell tumor in fascicles [10].…”

Section: Discussionmentioning

confidence: 99%

“…Forty to 65% of MPNST patients experience local recurrence, and 30 to 60% develop metastases within 12 months of initial surgery [16]. Factors that predict recurrence include anatomic site, tumor size (≥10 cm), and adequacy of margins.…”

Section: Discussionmentioning

confidence: 99%

“…Factors that predict recurrence include anatomic site, tumor size (≥10 cm), and adequacy of margins. Factors that predict metastases include tumor size (≥10 cm) or tumors that are American Joint Committee on Cancer stage III [16]. Over two thirds of metastases develop in the lung, whereas the other sites include the liver, brain, bone, and adrenal gland [16].…”

Section: Discussionmentioning

confidence: 99%

“…Factors that predict metastases include tumor size (≥10 cm) or tumors that are American Joint Committee on Cancer stage III [16]. Over two thirds of metastases develop in the lung, whereas the other sites include the liver, brain, bone, and adrenal gland [16]. To date, there is little knowledge on MPNST of the small bowel, which is thought to have a far worse prognosis than other soft tissue sarcomas.…”

Section: Discussionmentioning

confidence: 99%

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A distal ileum malignant peripheral nerve sheath tumor causing intussusception in a patient in China: a case report

Zhu

Xiao

et al. 2017

World J Surg Onc

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BackgroundMalignant peripheral nerve sheath tumors (MPNSTs) arise from a peripheral nerve or display nerve sheath differentiation. Most MPNSTs typically originate on the trunk, extremities, head, neck, and paravertebral regions. Gastrointestinal MPNSTs are rare entities with only 10 cases reported worldwide in the literatures.Case presentationHere, we report the first Chinese case of a malignant peripheral nerve sheath tumor of the distal ileum presenting as intussusception. A 53-year-old female patient without pathological antecedent for neurofibromatosis was admitted with pain in the right lower abdomen and multiple episodes of vomiting for 1 week. Preoperative diagnosis was intussusception with a contrast-enhanced computed tomography scan (CECT) of the abdomen showing characteristic target sign. Due to difficulty reducing the ileum-colon intussusception, right hemicolectomy with ileocolostomy was performed. Histopathology was suggestive of low-grade MPNST. The patient received postoperative care and was followed up for 9 months. There is no sign of tumor recurrence and metastatic disease.ConclusionsThis case is unique in terms of a rare tumor presenting with unusual complication.

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Section: Discussionmentioning

confidence: 68%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

See 3 more Smart Citations

A distal ileum malignant peripheral nerve sheath tumor causing intussusception in a patient in China: a case report

Zhu

Xiao

et al. 2017

World J Surg Onc

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Expanding a precision medicine platform for malignant peripheral nerve sheath tumors: New patient‐derived orthotopic xenografts, cell lines and tumor entities

Creus‐Bachiller,

Fernández‐Rodríguez,

Magallón‐Lorenz

et al. 2023

Molecular Oncology

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Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive soft tissue sarcomas with a poor survival rate, presenting either sporadically or in the context of neurofibromatosis type 1 (NF1). The histological diagnosis of MPNSTs can be challenging, with different tumors exhibiting great histological and marker expression overlap. This heterogeneity could be partly responsible for the observed disparity in treatment response due to the inherent diversity of the preclinical models used. For several years, our group has been generating a large patient‐derived orthotopic xenograft (PDOX) MPNST platform for identifying new precision medicine treatments. Herein, we describe the expansion of this platform using six primary tumors clinically diagnosed as MPNSTs, from which we obtained six additional PDOX mouse models and three cell lines, thus generating three pairs of in vitro–in vivo models. We extensively characterized these tumors and derived preclinical models, including genomic, epigenomic and histological analyses. Tumors were reclassified after these analyses: three remained as MPNSTs (two being classic MPNSTs), one was a melanoma, another an Neurotrophic tyrosine receptor kinase (NTRK)‐rearranged spindle cell neoplasm, and, finally, an unclassifiable tumor bearing Neurofibromin‐2 (NF2) inactivation, an Neuroblastoma RAS Viral Oncogene Homolog (NRAS) oncogenic mutation and a SWI/SNF‐related Matrix associated Actin‐dependent Regulator of Chromatin (SMARCA4) heterozygous truncated variant. New cell lines and PDOXs faithfully recapitulated histology, marker expression and genomic characteristics of the primary tumors. The diversity in tumor identity and their specific associated genomic alterations impacted on treatment responses obtained when we used the new cell lines for testing compounds against known altered pathways in MPNSTs. In summary, we present here an extension of our MPNST precision medicine platform, with new PDOXs and cell lines, including tumor entities confounded as MPNSTs in a real clinical scenario. This platform may constitute a useful tool for obtaining correct preclinical information to guide MPNST clinical trials.

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Targeting of AKT/ERK/CTNNB1 by DAW22 as a potential therapeutic compound for malignant peripheral nerve sheath tumor

Zhang

Chiu

et al. 2018

Cancer Medicine

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Malignant peripheral nerve sheath tumors (MPNSTs) are an aggressive form of soft tissue neoplasm with extremely poor prognosis and no effective medical options currently available. MPNSTs can occur either sporadically or in association with the neurofibromatosis type 1 (NF1) syndrome. Importantly, activation of RAS/RAF/MEK/ERK, PI3K/AKT/mTOR, and WNT/CTNNB1 signaling pathways has been reported in both NF1‐related and late‐stage sporadic MPNSTs. In this study, we found that DAW22, a natural sesquiterpene coumarin compound isolated from Ferula ferulaeoides (Steud.) Korov., could inhibit cell proliferation and colony formation in five established human MPNST cancer cell lines. Further molecular mechanism exploration indicated that DAW22 could target the main components in the MPNST tumorigenic pathways: namely suppress phosphorylation of AKT and ERK, and reduce levels of non‐phospho (active) CTNNB1. Using the xenograft mouse model transplanted with human MPNST cancer cell line, daily treatment with DAW22 for 25 days was effective in reducing tumor growth. These results support DAW22 as an alternative therapeutic compound for MPNST treatment by affecting multiple signaling transduction pathways in its disease progression.

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Malignant Peripheral Nerve Sheath Tumor

Cited by 134 publications

References 56 publications

A distal ileum malignant peripheral nerve sheath tumor causing intussusception in a patient in China: a case report

A distal ileum malignant peripheral nerve sheath tumor causing intussusception in a patient in China: a case report

Expanding a precision medicine platform for malignant peripheral nerve sheath tumors: New patient‐derived orthotopic xenografts, cell lines and tumor entities

Targeting of AKT/ERK/CTNNB1 by DAW22 as a potential therapeutic compound for malignant peripheral nerve sheath tumor

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