Malignant peripheral nerve sheath tumor: A rare malignancy

Sharma, Sheetal; Shah, Jigna S; Bali, Harleen

doi:10.4103/jomfp.jomfp_9_20

Cited by 11 publications

(5 citation statements)

References 10 publications

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“…6 Malignant peripheral nerve sheath tumor (MPNST) are rare and only 10-12% are reported in oral and maxillofacial area. 7 It may arise sporadically in the setting of previous radiation, from pre-existing neoplasms, including neurofibromas and neurilemmomas, or as part of neurofibromatosis type 1 (NF1). MPNST is consider if one of the following criteria can be established: (I) origin of the tumour from a peripheral nerve; (II) origin from a preexisting benign lesion of the nerve sheath; and (III) microscopically, the tumour reveals differentiation of Schwann cells.…”

Section: Discussionmentioning

confidence: 99%

An unexpected case of lower lip benign Schwannoma: diagnosis, management and review of literature

Mona¹,

Anand²,

Sharma³

2023

Int Surg J

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Schwannoma also known as neurinomas of verocay is a benign, slow growing, nerve sheath tumor arising from the Schwann cells of the nerve. Around 25-40% of the schwannoma arises from the soft tissues of head and neck areas. Out of which, only <1% arises from the oral cavity. The most common intra-oral site is the tongue and the least common site being the lips. Moreover, Schwannoma in the lower lip are excessively rare during childhood and adolescence. Due to its rarity in lower lip, it’s diagnosis is usually overlooked during the primary examination. Complete surgical excision of the tumor is the treatment of choice. Recurrence cases and malignant degeneration are extremely rare. Here, we reported a case of a 17 year old female with peripheral Schwannoma of the lower lip, presented to the Otorhinolaryngology department, Indira Gandhi Hospital, Dwarka, Delhi with complaints of painless swelling in the lower lip causing cosmetic deformity for the patient. She underwent complete enucleation of the mass and is being follow-up in the outpatient department.

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Section: Discussionmentioning

confidence: 99%

An unexpected case of lower lip benign Schwannoma: diagnosis, management and review of literature

Mona¹,

Anand²,

Sharma³

2023

Int Surg J

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“…Despite fibrosarcoma and synovial sarcoma have a uniform fascicular pattern, they clearly lack features of neural differentiation (Patel et al, 2015). Leiomyosarcoma is also cited as a histopathological hypothesis, but its cells have deeply eosinophilic cytoplasm, centrally placed blunt‐ended nuclei and juxtanuclear vacuoles, showing immunoreactivity to most of the smooth muscle markers—opposition characteristics of MPNST (Sharma et al, 2020). Desmoplastic melanoma, solitary fibrous tumour and nodular fasciitis could be also included as differential diagnosis.…”

Section: Discussionmentioning

confidence: 99%

“…Virtually any location can be affected by MPNST, and a predilection is observed for the extremities and pelvis (Farid et al, 2014). In the oral and maxillofacial region, this neoplasm is particularly rare, representing only 10% to 12% of all lesions occurring in the head and neck region (Sharma et al, 2020). For instance, in a large study conducted in Japan, only one of 1,643 individuals examined had an MPNST (Sumida et al, 2015).…”

Section: Introductionmentioning

confidence: 99%

Malignant peripheral nerve sheath tumour of the oral and maxillofacial region—A systematic review

et al. 2021

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To integrate the available data published on malignant peripheral nerve sheath tumours (MPNST) of the oral and maxillofacial region. Searches in Embase, PubMed, Web of Science and Scopus were conducted for the identification of case reports/case series in English language. The risk of bias was assessed using the Joanna Briggs Institute tool. Outcomes were evaluated by Cox regression and Kaplan–Meier methods. A total of 306 articles were retrieved, 50 of which reporting 57 MPNST were included. The lesion showed a predilection for the mandible (n = 18/31.57%) of middle‐aged adults (~40.5 years) with a male/female ratio of 1.1:1. The individuals were mostly symptomatic with a mean evolution time of 9.6 months. Surgical removal plus adjuvant therapy (especially radiotherapy) was the main approach (51.86%). Recurrence was reported in 39.62% of cases. Nodal and distant metastases were identified in 28.26% and 26.66% of cases, respectively. The 2‐year cumulative survival rate was 55%. Independent predictors of poor survival were the presence of neurofibromatosis type 1 (p = 0.04) and distant metastases (p = 0.004). The diagnosis of MPNST is challenging due to the variety of its clinical and histopathological presentations. Local aggressiveness and the potential for metastases are common outcomes of this neoplasm.

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“…The use of monoclonal antibodies to disrupt the interaction between PD-1 and PD-L1 is a widely accepted ICB therapy, however, having a T cell-enriched TME is critical for ICB therapy success (17). Unfortunately, MPNSTs are non-T cellinflamed or "cold" tumors and are therefore not likely to elicit an antitumor immune response to checkpoint inhibition (18)(19)(20). It has been shown that some MPNSTs have more prevalent PD-L1 expression than do normal nerves, benign neurofibromas, or schwannomas (19), whereas another study reported similar lev-Neurofibromatosis type 1 (NF1) is caused by mutations in the NF1 gene that encodes neurofibromin, a RAS GTPase-activating protein.…”

Section: Introductionmentioning

confidence: 99%

STING activation reprograms the microenvironment to sensitize NF1-related malignant peripheral nerve sheath tumors for immunotherapy

Somatilaka,

Madana,

Sadek

et al. 2024

Journal of Clinical Investigation

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Neurofibromatosis type 1 (NF1) is caused by mutations in the NF1 gene that encodes neurofibromin, a RAS GTPase–activating protein. Inactivating NF1 mutations cause hyperactivation of RAS-mediated signaling, resulting in the development of multiple neoplasms, including malignant peripheral nerve sheath tumors (MPNSTs). MPNSTs are an aggressive tumor and the main cause of mortality in patients with NF1. MPNSTs are difficult to resect and refractory to chemo- and radiotherapy, and no molecular therapies currently exist. Immune checkpoint blockade (ICB) is an approach to treat inoperable, undruggable cancers like MPNST, but successful outcomes require an immune cell–rich tumor microenvironment. While MPNSTs are noninflamed “cold” tumors, here, we converted MPNSTs into T cell–inflamed “hot” tumors by activating stimulator of IFN genes (STING) signaling. Mouse genetic and human xenograft MPNST models treated with a STING agonist plus ICB exhibited growth delay via increased apoptotic cell death. This strategy offers a potential treatment regimen for MPNSTs.

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Malignant peripheral nerve sheath tumor: A rare malignancy

Cited by 11 publications

References 10 publications

An unexpected case of lower lip benign Schwannoma: diagnosis, management and review of literature

An unexpected case of lower lip benign Schwannoma: diagnosis, management and review of literature

Malignant peripheral nerve sheath tumour of the oral and maxillofacial region—A systematic review

STING activation reprograms the microenvironment to sensitize NF1-related malignant peripheral nerve sheath tumors for immunotherapy

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