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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.


Related in: MedlinePlus

Macroscopic appearance. The specimen has an irregular shape, measuring 7.3 × 1.3 × 7 cm. It is solid, heterogenous, yellowish brown with areas of necrosis and hemorrhage and rubbery consistency (A). Small specimens with homogeneous solid appearance, whitish, yellow and soft rubbery consistencies were observed (B and C).
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fig0015: Macroscopic appearance. The specimen has an irregular shape, measuring 7.3 × 1.3 × 7 cm. It is solid, heterogenous, yellowish brown with areas of necrosis and hemorrhage and rubbery consistency (A). Small specimens with homogeneous solid appearance, whitish, yellow and soft rubbery consistencies were observed (B and C).

Mentions: Glossopharyngeal, vagus and spinal nerves were surrounded entirely by the tumor and dissection was deemed impossible; the exact tumor origin could not be established. Tumor and the inner nerves were sectioned at its rostral and caudal extremes (Fig. 3). There were no incidents during surgery.


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
Macroscopic appearance. The specimen has an irregular shape, measuring 7.3 × 1.3 × 7 cm. It is solid, heterogenous, yellowish brown with areas of necrosis and hemorrhage and rubbery consistency (A). Small specimens with homogeneous solid appearance, whitish, yellow and soft rubbery consistencies were observed (B and C).
© Copyright Policy - CC BY-NC-ND
Related In: Results  -  Collection

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

fig0015: Macroscopic appearance. The specimen has an irregular shape, measuring 7.3 × 1.3 × 7 cm. It is solid, heterogenous, yellowish brown with areas of necrosis and hemorrhage and rubbery consistency (A). Small specimens with homogeneous solid appearance, whitish, yellow and soft rubbery consistencies were observed (B and C).
Mentions: Glossopharyngeal, vagus and spinal nerves were surrounded entirely by the tumor and dissection was deemed impossible; the exact tumor origin could not be established. Tumor and the inner nerves were sectioned at its rostral and caudal extremes (Fig. 3). There were no incidents during surgery.

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.


Related in: MedlinePlus