Updates on the diagnosis and treatment of intracranial nerve malignant peripheral nerve sheath tumors

Abstract

Background:Malignant peripheral nerve sheath tumors (MPNSTs) are rare entities and MPNSTs of intracranial nerves are even more sporadic. MPNSTs present diagnosis and treatment challenges since there are no defined diagnosis criteria and no established therapeutic strategies.Methods:We reviewed literature for MPNST-related articles. We found 45 relevant studies in which 60 cases were described.Results:We identified 60 cases of intracranial nerve MPNSTs. The age ranged from 3 to 75 years old. Male to female ratio was 1.5:1. The most involved cranial nerves (CNs) were CN VIII (60%), CN V (27%), and CN VII (10%). Most of the MPNSTs reported (47%) arose sporadically, 40% arose from a schwannoma, 8% arose from a neurofibroma, and 6% arose from an unspecified nerve tumor. Twenty patients had a history of radiation exposure, four patients had neurofibromatosis type 1 (NF1), four patients had neurofibromatosis type 2 (NF2), and NF2 was suspected in two other patients. Twenty-two patients were treated with radiotherapy and presented a higher survival rate. Seventy-two percent of patients died of their disease while 28% of patients survived. One-year survival rate was 33%. Forty-five percent of tumors recurred and 19% of patients had metastases.Conclusion:MPNSTs involving CNs are very rare. Diagnosis is made in regards to the histological and pathological findings. Imaging may help orient the diagnosis. A preexisting knowledge of the clinical situation is more likely to lead to a correct diagnosis. The mainstay of treatment is radical surgical resection with adjuvant radiotherapy. Since these tumors are associated with a poor prognosis, a close follow-up is mandatory.

Malignant Peripheral Nerve Sheath Tumors of the Head and Neck: A Case Series and Literature Review

“…However, given the relatively small space within the head and neck, the proximity to vital structures, and the potential for microscopic local spread, obtaining negative margins can be difficult. These factors have led to high rates of local recurrence (22% to 52%) and distant metastasis (18% to 33%) and thus a multimodality treatment approach is most favorable [ 2 , 22 – 24 ].…”

“…In addition to surgery, many studies have cited the importance of radiotherapy as adjunctive treatment, especially for improving local control [ 2 , 15 , 18 , 20 , 24 – 27 ]. The radiation dose commonly administered in studies is 50–60 Gy.…”

“…The oncology consensus group, as part of a uniform treatment policy for MPNSTs, recommends adjuvant radiotherapy for all “intermediate- to high-grade lesions and for low-grade tumors after a marginal excision” [ 18 ]. Though the consensus group states that radiotherapy only provides local control and has little effect on long-term survival, some more recent studies have also reported survival benefit [ 2 , 15 , 24 , 28 ]. Our series continues to highlight the importance of adjunctive radiotherapy.…”

A malignant peripheral nerve sheath tumor developed from the auditory nerve: a case report and a literature review

“…Злокачественная опухоль оболочек периферических нервов -malignant peripheral nerve sheath tumor (MPNST) относится к редкой и гетерогенной группе мезенхимальных злокачественных новообразований, рассматриваемых в большинстве публикаций как саркоматозный процесс. Эта группа опухолей составляет 5-10% всех сарком мягких тканей, с примерной частотой встречаемости 1 человек на 1 млн населения в год [1][2][3][4].…”

“…Черепные нервы являются источником роста опухоли в менее чем 5% случаев [3,6,7]. Из интракраниальных MPNST чаще поражаются лицевой и слуховой нервы [2,4]. На 1041 вестибулярную шванному приходится 1 случай MPNST слухового нерва [1].…”