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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

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ABSTRACT

Intracranial 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.

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.

No MeSH data available.


Preoperative brain MRI showing a hyperintense neoplastic lesion on T2 sequence at left cerebellopontine cistern (A) with extension to C4-C5 level through jugular foramen (B and C), it enhances at gadolinium administration (D–F) and shows a low signal intensity in the center (E and F).
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fig0005: Preoperative brain MRI showing a hyperintense neoplastic lesion on T2 sequence at left cerebellopontine cistern (A) with extension to C4-C5 level through jugular foramen (B and C), it enhances at gadolinium administration (D–F) and shows a low signal intensity in the center (E and F).

Mentions: In MRI a neoplastic aspect lesion was observed, which extended from the left cerebellopontine cistern to C4-C5 levels through the jugular foramen (Fig. 1). Positron emission tomography combined with computed tomography (PET-CT) with 18-fludeoxyglucose revealed increased uptake at paravertebral levels, pelvic cavity and left thigh (Fig. 2). In addition to the known diagnoses of type 1 neurofibromatosis and malnutrition, Villaret́s syndrome was diagnosed secondary to neoplastic lesion at left jugular foramen, compatible with MPNST.


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
Preoperative brain MRI showing a hyperintense neoplastic lesion on T2 sequence at left cerebellopontine cistern (A) with extension to C4-C5 level through jugular foramen (B and C), it enhances at gadolinium administration (D–F) and shows a low signal intensity in the center (E and F).
© Copyright Policy - CC BY-NC-ND
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC5121163&req=5

fig0005: Preoperative brain MRI showing a hyperintense neoplastic lesion on T2 sequence at left cerebellopontine cistern (A) with extension to C4-C5 level through jugular foramen (B and C), it enhances at gadolinium administration (D–F) and shows a low signal intensity in the center (E and F).
Mentions: In MRI a neoplastic aspect lesion was observed, which extended from the left cerebellopontine cistern to C4-C5 levels through the jugular foramen (Fig. 1). Positron emission tomography combined with computed tomography (PET-CT) with 18-fludeoxyglucose revealed increased uptake at paravertebral levels, pelvic cavity and left thigh (Fig. 2). In addition to the known diagnoses of type 1 neurofibromatosis and malnutrition, Villaret́s syndrome was diagnosed secondary to neoplastic lesion at left jugular foramen, compatible with MPNST.

View Article: PubMed Central - PubMed

ABSTRACT

Intracranial 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.

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.

No MeSH data available.