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

Schuch, Lauren Frenzel; Kirschnick, Laura Borges; Arruda, José Alcides Almeida de; Klein, Isadora Peres; Silveira, Felipe Martins; Vasconcelos, Ana Carolina Uchôa; Santos‐Silva, Alan Roger; Lopes, Márcio Ajudarte; Carrard, Vinícius Coelho; Vargas, Pablo Agustı́n; Martins, Marco Antônio Trevizani; Wagner, Vivian Petersen; Martins, Manoela Domingues

doi:10.1111/odi.13982

Cited by 3 publications

(5 citation statements)

References 101 publications

(114 reference statements)

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“…The exact etiology is not known and it may be associated with syndromes like neurofibromatosis type 1. 4 Benign Schwannoma of oral cavity is an uncommon presentation. Less then 1% arises from the oral cavity even when these areas are highly innervated.…”

Section: Discussionmentioning

confidence: 99%

“…They commonly presents as rapidly growing tumor with pain, tenderness, numbness, paraesthesia and burning sensation. 4 The diagnosis is based on the histopathological examination after biopsy or excision. Histologically, they are of seven subtypes: classical (verocay), plexiform, cellular, cranial nerve, melanotic, degenerated (ancient), and granular cell schwannomas.…”

Section: Discussionmentioning

confidence: 99%

“…Cases of recurrence or malignant transformation in lower lip schwannoma has been reported. 4 Malignant peripheral nerve sheath tumors are rare tumors which can arise from a pre-existing benign nerve sheath tumor like neurofibromas or neurilemmomas. It is an aggressive tumor which are highly metastatic with high recurrence rate and poor outcomes.…”

Section: Introductionmentioning

confidence: 99%

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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%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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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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“…2,3,5,7 In our study, similar risk factors were identified. Large lesions (>10 cm), 5,17 metastasis at initial diagnosis, 15,19 and recurrence 17 were significantly poor prognostic factors of 5-year overall survival (Table 5).…”

Section: Discussionmentioning

confidence: 99%

Survival analysis of malignant peripheral nerve sheath tumor: Experience of a tertiary center in Taiwan

Chang

Liao

2023

Journal of the Chinese Medical Association

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Background: This study aimed to analyze the demographic characteristics and prognostic factors of malignant peripheral nerve sheath tumor (MPNST) in a Taiwanese population. Single-center treatment outcomes were also presented. Methods: This retrospective cohort study analyzed the medical records of 54 patients with pathological diagnoses of MPNSTs from 2005 to 2021 at a single institution. The primary endpoint was the 5-year overall survival rate of MPNST, and the secondary endpoint was recurrence-free 5-year survival. Variables including patient characteristics, metastasis status at initial diagnosis, and surgical outcomes were analyzed with competing risk analysis. Results: Among all 41 eligible patients diagnosed with MPNST, female predominance was noted, and the median age at diagnosis was 44 years. The most common site of lesion was found at the trunk (46.34%), and eight patients were diagnosed with notable metastasis. Twelve patients were diagnosed with type 1 neurofibromatosis (NF1). The 5-year overall survival rate was 36.84% and the 5-year recurrence-free survival was 28.95%. Metastasis diagnosed at presentation, large lesion sizes, and recurrence were identified as significant poor prognostic factors of survival. Metastasis diagnosed at presentation was identified as the only significant risk factor of recurrence. Conclusion: In our series, metastasis diagnosed at presentation, large lesion sizes, and recurrence were identified as significant poor prognostic factors of survival. Metastasis was also identified as the only significant risk factor of recurrence. NF1-associated MPNSTs presented with significantly larger tumor sizes and additional treatment postoperatively did not significantly improve survival. The limitations of this study include its retrospective nature and sample size.

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“…Malignant schwannoma has a high risk of local recurrence and distant metastasis. Survival rate is generally low ( 8 , 15 ).…”

Section: Related Literature Learningmentioning

confidence: 99%

Case Report: Adrenal glands degenerated schwannoma: Report of three cases and literature review

et al. 2023

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BackgroundSchwannoma is a benign tumor, of which degenerated schwannoma is a subtype. Retroperitoneal schwannomas are extremely rare, as they account for only 3% of retroperitoneal tumors.Degenerated schwannoma is a schwannoma subtype. However,degenerated schwannoma occurring in the adrenal glands is extremely rare.Case summaryCase 1: A 42-year-old man was referred to our hospital for further examination of a left adrenal mass that was incidentally discovered during a routine physical check-up.No significant abnormalities were found in laboratory tests results. Robotic-assisted laparoscopic excision of the left adrenal gland was performed under general anesthesia. Case 2: A 47-year-old man was admitted to the hospital because of a left adrenal mass found on a routine physical examination.The patient was previously in good health, and there was no family history of a similar disorder. Left-sided laparoscopic adrenalectomy was performed under general anaesthesia. Case 3: A 62-year-old woman with hypertension and diabetes mellitus was referred to our hospital after an incidentally found left adrenal mass.There was no family history of a similar disorder. Left-sided laparoscopic adrenalectomy was performed under general anaesthesia. None of the patients had a recurrence in our study during the postoperative follow-up.ConclusionDegenerated schwannoma of the adrenal glands is very rare. The clinical presentations of degenerated schwannoma are nonspecific; a small number of patients do not have any symptoms, and the mass is only found incidentally during physical examination for any number of reasons. The preoperative diagnosis of adrenal degenerated schwannoma is difficult because the diagnosis must rely on pathological examination and immunohistochemistry assays. The management is surgical excision and regular follow-up.

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

Cited by 3 publications

References 101 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

Survival analysis of malignant peripheral nerve sheath tumor: Experience of a tertiary center in Taiwan

Case Report: Adrenal glands degenerated schwannoma: Report of three cases and literature review

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