Malignant nerve sheath tumor involving glossopharyngeal, vagus and spinal nerve with intracranial-extracranial extension and systemic metastases in a patient with type 1 neurofibromatosis: A case report

Abstract

HighlightsIntracranial malignant peripheral nerve sheath tumors are an extremely rare pathology with a high morbidity and mortality.The most frequently affected cranial nerves in decreasing order of frequency are the trigeminal, vestibulocochlear and facial, being glossopharyngeal, vagus and spinal nerves extremely rare affected.This manuscript is the first in English-written literature to expose the case of an adult female patient with NF1 who presented a MPNST involving the glossopharyngeal, vagus and spinal left nerves with intracranial-extracranial extension and systemic metastases.Treatment strategies for this particular pathology are still unclear due to the low frequency of the disease and a lack of number of cases, making them a challenge for the neurosurgeon.

First documented case of intracranial falcine malignant peripheral nerve sheath tumor: illustrative case

“…21 Intracranial MPNSTs are extraaxial neoplasms affecting the cranial nerves and are associated with the nerve root, particularly affecting the vestibular, trigeminal, and lower cranial nerves. 7,[9][10][11][12]22 L'heureux-Lebeau and Saliba published a systematic review based on 60 cases of intracranial MPNSTs. 23 A subtype of intraparenchymal MPNST, which is rarer, was described and referred to as MINST.…”

“…Intracranial cases are rare and often classified in two groups based on their relationships with the neurovascular structures primarily involved: (1) extraaxial MPNSTs originating from the cranial nerves in the posterior cranial fossa, more frequently from the vestibular, trigeminal, and lower cranial nerves, and (2) intraparenchymal type, referred to as malignant intracerebral nerve sheath tumor (MINST). [7][8][9][10][11][12] The histological, immunohistochemical, and ultrastructural characteristics of MINST are similar to those of MPNST. However, the former originates directly from brain parenchymal tissue, and it has no relationship with the cranial nerves.…”

Glossopharyngeal schwannoma a rare case report: Diagnostic and surgical approach

“…Schwannomas can grow on both peripheral nerves and nerve roots; with a notable example being vestibular schwannomas and jugular foramen schwannomas. These originate from the ninth, tenth, and eleventh cranial nerves [ 3 , 4 ]. Among the posterior fossa tumors, the extremely rare glossopharyngeal schwannomas often present with an increased likelihood towards males and usually occur during the third to fifth decades of life.…”

“…Among the posterior fossa tumors, the extremely rare glossopharyngeal schwannomas often present with an increased likelihood towards males and usually occur during the third to fifth decades of life. Symptoms of patients with schwannomas may not manifest until the tumor attains a fairly large size, and these symptoms may vary from subtle to severe [ 1 , 4 ]. The diagnosis of glossopharyngeal schwannomas is usually made once the tumor's attachment to the ninth nerve is seen at surgery [ 1 ].…”