scholarly journals Toward Understanding the Mechanisms of Malignant Peripheral Nerve Sheath Tumor Development

2021 ◽  
Vol 22 (16) ◽  
pp. 8620
Author(s):  
Teddy Mohamad ◽  
Camille Plante ◽  
Jean-Philippe Brosseau
Keyword(s):  
Peripheral Nerve ◽  
Tumor Development ◽  
Neurofibromatosis Type ◽  
Type I ◽  
Nerve Sheath Tumor ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Research Perspectives ◽  
Nerve Sheath ◽  
History Of

Malignant peripheral nerve sheath tumors (MPNSTs) originate from the neural crest lineage and are associated with the neurofibromatosis type I syndrome. MPNST is an unmet clinical need. In this review article, we summarize the knowledge and discuss research perspectives related to (1) the natural history of MPNST development; (2) the mouse models recapitulating the progression from precursor lesions to MPNST; (3) the role of the tumor microenvironment in MPNST development, and (4) the signaling pathways linked to MPNST development.

Primary malignant peripheral nerve sheath tumor of the spine with acute hydrocephalus: a rare clinical entity

2014 ◽  
Vol 21 (3) ◽  
pp. 367-371 ◽  
Author(s):  
Yaxiong Li ◽  
Fengshi Fan ◽  
Jianguo Xu ◽  
Jie An ◽  
Weining Zhang
Keyword(s):  
Peripheral Nerve ◽  
English Language ◽  
Standard Of Care ◽  
Nerve Sheath Tumor ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
History Of ◽  
Brain Ct Scan ◽  
The Right

Primary malignant peripheral nerve sheath tumors (MPNSTs) are extremely rare in patients without a history of neurofibromatosis; only 18 cases have been reported in the English-language literature to this point. The authors report their experience with 1 new case of a primary MPNST. A 33-year-old woman presented with low-back pain radiating to the right calf that progressed over 1 year. Magnetic resonance imaging of the spine revealed an intradural extramedullary lesion at the T12–L1 level. The patient was diagnosed with primary MPNST, underwent two surgical excisions and radiation therapy, and developed leptomeningeal metastases as well as brain metastases. The patient revisited the emergency room with sudden loss of consciousness. A brain CT scan displayed bilateral lateral ventricle enlargement, for which a ventriculoperitoneal shunt was inserted. These symptoms have not been described in any previous report. Primary spinal MPNST is an exceedingly rare entity, and the overall prognosis is very poor. To the authors' knowledge, no standard of care for primary spinal MPNSTs has yet been established. All 19 cases of primary spinal MPNSTs are reviewed, and the authors discuss their clinical, radiological, and therapeutic features and outcomes.

Radiation-Induced Glandular Malignant Peripheral Nerve Sheath Tumor

2017 ◽  
Vol 25 (7) ◽  
pp. 635-639 ◽  
Author(s):  
Ivy John ◽  
David L. Bartlett ◽  
Uma N. M. Rao
Keyword(s):  
Peripheral Nerve ◽  
Femoral Nerve ◽  
Neurofibromatosis Type ◽  
Soft Tissue Tumors ◽  
Nerve Sheath Tumor ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
Divergent Differentiation ◽  
Radiation Induced

Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive soft-tissue tumors. They can occur in patients with neurofibromatosis type-1 (NF-1) or as sporadic tumors. Only 10% of MPNSTs are radiation induced. Divergent differentiation in MPNSTs can occur in 15% of cases and may include cartilage, bone, skeletal muscle, blood vessels, and very rarely well-formed glands, the latter typically described in NF-1–associated MPNSTs. We report an exceedingly rare case of radiation induced glandular MPNST arising in a neurofibroma of the femoral nerve in a patient previously irradiated for endometrial carcinoma.

scholarly journals The Role of ER Stress-Related Phenomena in the Biology of Malignant Peripheral Nerve Sheath Tumors

2021 ◽  
Vol 22 (17) ◽  
pp. 9405
Author(s):  
Anna Walczak ◽  
Maciej Radek ◽  
Ireneusz Majsterek
Keyword(s):  
Peripheral Nerve ◽  
Early Stage ◽  
Neurofibromatosis Type ◽  
Negative Regulator ◽  
Stress Factors ◽  
Type I ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
The Impact

Malignant peripheral nerve sheath tumors (MPNST) are rare but one of the most aggressive types of cancer. Currently, there are no effective chemotherapy strategies for these malignancies. The inactivation of the neurofibromatosis type I (NF1) gene, followed by loss of TP53, is an early stage in MPNST carcinogenesis. NF1 is a negative regulator of the Ras proteins family, which are key factors in regulating cell growth, homeostasis and survival. Cell cycle dysregulation induces a stress phenotype, such as proteotoxic stress, metabolic stress, and oxidative stress, which should result in cell death. However, in the case of neoplastic cells, we observe not only the avoidance of apoptosis, but also the impact of stress factors on the treatment effectiveness. This review focuses on the pathomechanisms underlying MPNST cells physiology, and discusses the possible ways to develop a successful treatment based on the molecular background of the disease.

Malignant Peripheral Nerve Sheath Tumor With Divergent Glandular Differentiation

2017 ◽  
Vol 25 (4) ◽  
pp. 310-313 ◽  
Author(s):  
Yurina Miki ◽  
Khin Thway
Keyword(s):  
Differential Diagnosis ◽  
Peripheral Nerve ◽  
Neurofibromatosis Type ◽  
Nerve Sheath Tumor ◽  
Histologic Diagnosis ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Immunohistochemical Markers ◽  
Nerve Sheath ◽  
Divergent Differentiation

Malignant peripheral nerve sheath tumors (MPNST) are soft tissue neoplasms with evidence of nerve sheath differentiation. They usually arise from peripheral nerves or from preexisting benign nerve sheath neoplasms, often in patients with neurofibromatosis type 1 (NF1). The histologic diagnosis of MPNST is challenging as their morphology is highly variable, and there has been a lack of routine diagnostic immunohistochemical markers and specific genetic aberrations. Although divergent differentiation is well documented in MPNST, it is most frequently toward mesenchymal elements. Differentiation toward epithelial elements is very rare, and we illustrate a case of MPNST with glandular differentiation, comprising prominent well-formed glands, with a brief discussion of biphasic (spindle and glandular) neoplasms in the differential diagnosis. An index of suspicion for MPNST is necessary, due to the differing management from tumors in its differential diagnosis, and because of the potential for therapies toward molecular targets in future.

scholarly journals Neurofibromatosis type I with malignant peripheral nerve sheath tumors in the upper arm

Medicine ◽  
2019 ◽  
Vol 98 (13) ◽  
pp. e15017 ◽  
Author(s):  
Lili Zhang ◽  
Fangfang Sun ◽  
Hongyu Li ◽  
Jie Du ◽  
Lirong Zhao
Keyword(s):  
Peripheral Nerve ◽  
Neurofibromatosis Type ◽  
Type I ◽  
Neurofibromatosis Type I ◽  
Upper Arm ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

scholarly journals Case Report: An incidental finding of an adrenal metastases noted in a “collision tumor” from a large malignant nerve sheath tumor of the thigh

F1000Research ◽  
2017 ◽  
Vol 6 ◽  
pp. 1964
Author(s):  
Matthew D. Shaines ◽  
Shitij Arora
Keyword(s):  
Peripheral Nerve ◽  
Incidental Finding ◽  
Neurofibromatosis Type ◽  
Collision Tumor ◽  
Adrenal Metastasis ◽  
Nerve Sheath Tumor ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

In cases of peripheral nerve sheath tumors, current guidelines do not recommend routine abdominal imaging to stage the disease, as extra-pulmonary metastasis is considered rare. We report a case of large peripheral nerve sheath tumor in a 40 year-old-female with neurofibromatosis type 1 who had isolated adrenal metastasis. She underwent primary and adrenal metastasis resection.

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