Malignant peripheral nerve sheath tumor: A rarity

There is no author summary for this article yet. Authors can add summaries to their articles on ScienceOpen to make them more accessible to a non-specialist audience.

Abstract

Malignant peripheral nerve sheath tumor (MPNST) of the mandible is an uncommon tumor that develops either from a preexisting neurofibroma or de novo. MPNSTs are sarcomas that originate from peripheral nerves or from cells associated with the nerve sheath, such as Schwann cells, perineural cells or from fibroblasts. Because MPNSTs can arise from multiple cell types, the overall appearance can vary greatly from one case to the next. A case of MPNST of the right side of the mandible in a 23-year-old female is reported.

Related collections

Most cited references 17

Record: found
Abstract: found
Article: not found

International consensus statement on malignant peripheral nerve sheath tumors in neurofibromatosis.

R Ferner, David Gutmann (2002)

Neurofibromatosis 1 (NF1) is an autosomal dominant tumor predisposition syndrome in which affected individuals have a greatly increased risk of developing malignant peripheral nerve sheath tumors (MPNSTs). These cancers are difficult to detect and have a poor prognosis. Because patients may present to specialists from widely differing disciplines, the association with NF1 is often not appreciated, and there is no cohesive pattern of care. A multidisciplinary group of 33 clinicians and scientists with specialist knowledge in MPNST and NF1 reviewed the current published and unpublished data in this field, and distilled their collective experience to produce a consensus summary on MPNST in NF1. The known clinical, pathological, and genetic information on MPNST in NF1was collated, and a database was established to record information in a uniform manner. Subgroups with a higher risk of developing MPNSTwere identified within the NF1 population. The consortium formulated proposals and guidelines for clinical and pathological diagnosis, surgical management, and medical treatment of MPNST in individuals with NF1.A multidisciplinary team approach to the management of this complex disorder is advocated. Progress can be made by adopting the guidelines proposed by this consortium and by widespread dissemination of standardized information. Collaborative research should be promoted with the aim of harnessing advances in molecular genetics to develop targeted therapies for MPNST in people with NF1.

0 comments Cited 115 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: not found

First-line chemotherapy for malignant peripheral nerve sheath tumor (MPNST) versus other histological soft tissue sarcoma subtypes and as a prognostic factor for MPNST: an EORTC soft tissue and bone sarcoma group study.

J.R. Kroep, M Ouali, H Gelderblom … (2011)

the role of chemotherapy in advanced malignant peripheral nerve sheath tumor (MPNST) is unclear. chemotherapy-naive soft tissue sarcomas (STS) patients treated on 12 pooled nonrandomized and randomized European Organization for Research and Treatment of Cancer Soft Tissue and Bone Sarcoma Group trials were retrospectively analyzed. Clinical outcomes, overall survival, progression-free survival (PFS) and response were determined for MPNST and other STS histotypes and compared. Additionally, prognostic factors within the MPNST population were defined. Studied cofactors were demographics, sarcoma history, disease extent and chemotherapy regimen. after a median follow-up of 4.1 years, 175 MPNST out of 2675 eligible STS patients were analyzed. Outcome was similar for MPNST versus other STS histotypes, with a response rate, median PFS and overall survival of 21% versus 22%, 17 versus 16 weeks and 48 versus 51 weeks, respectively. Performance status was an independent prognostic factor for overall survival. Chemotherapy regimen was an independent prognostic factor for response (P < 0.0001) and PFS (P = 0.009). Compared with standard first-line doxorubicin, the doxorubicin-ifosfamide regimen had the best response, whereas ifosfamide had the worst prognosis. this series indicates the role of chemotherapy in treatment of advanced MPNST. This first comparison showed similar outcomes for MPNST and other STS histotypes. The apparent superiority of the doxorubicin-ifosfamide regimen justifies further investigations of this combination in randomized trials.

0 comments Cited 66 times – based on 0 reviews      Review now

Bookmark

Record: found
Abstract: found
Article: not found

Malignant peripheral nerve sheath tumors of the head and neck: management of 10 cases and literature review.

Amir Minovi, Oliver Basten, Ben Hunter … (2007)

This study analyzes the management and outcomes of a series of 10 malignant peripheral nerve sheath tumors (MPNST) of the head and neck. From 1984 to 2004, 10 patients underwent surgical treatment of a MPNST. We retrospectively reviewed presenting symptoms, radiological findings, surgical management, and follow-up status and performed a literature review. Eight tumors were located at the lateral skull base; 2 involved the vagus nerve in isolation. Two lesions were growing within the sinonasal tract. The most common presenting symptom was a rapidly enlarging cervical mass. Seventy percent of the tumors could be resected completely. Long-term follow-up showed a 2-year disease-specific survival rate of 50% and 5-year survival rate of 20%. Negative prognostic indicators were advanced tumor stage, early recurrence, and presumably also the presence of von Recklinghausen's disease. Postoperative adjuvant radiotherapy was found to make no difference in outcome. Although rare, MPNST is one of the most aggressive tumors in the head and neck area. Complete tumor removal is the mainstay of treatment and most important prognostic factor of MPNST. Adjuvant radiotherapy should be used to assist surgical excision in local control. The role of adjuvant chemotherapy remains controversial. (c) 2006 Wiley Periodicals, Inc.

0 comments Cited 24 times – based on 0 reviews      Review now

Bookmark

All references

Author and article information

Journal

Journal ID (nlm-ta): Indian J Dent

Journal ID (iso-abbrev): Indian J Dent

Journal ID (publisher-id): IJDENT

Title: Indian Journal of Dentistry

Publisher: Medknow Publications & Media Pvt Ltd (India )

ISSN (Print): 0975-962X

ISSN (Electronic): 2213-3666

Publication date (Print): Jan-Mar 2015

Volume: 6

Issue: 1

Pages: 53-56

Affiliations

[1] Department of Oral Medicine and Radiology, MNR Dental College and Hospital, Sangareddy, Telangana, India

Author notes

Address for correspondence: Dr. Rashmi Shivakumar, Department of Oral Medicine and Radiology, MNR Dental College and Hospital, Sangareddy, Telangana, India. E-mail: rashmi.doshetty@ 123456gmail.com

Article

Publisher ID: IJDENT-6-53

DOI: 10.4103/0975-962X.151712

PMC ID: 4357080

PubMed ID: 25767362

SO-VID: 6efb542f-5a09-4601-89e3-b38d4f474e23

License:

This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Malignant peripheral nerve sheath tumor: A rarity

Read this article at

Abstract

Related collections

Gamma Delta T cells

Most cited references 17

International consensus statement on malignant peripheral nerve sheath tumors in neurofibromatosis.

First-line chemotherapy for malignant peripheral nerve sheath tumor (MPNST) versus other histological soft tissue sarcoma subtypes and as a prognostic factor for MPNST: an EORTC soft tissue and bone sarcoma group study.

Malignant peripheral nerve sheath tumors of the head and neck: management of 10 cases and literature review.

Author and article information

Journal

Affiliations

Author notes

Article

History

Categories

Comments

Comment on this article

Similar content 639

Cited by 4

Most referenced authors 79