Malignant Nerve Sheath Tumor of the Spinal Accessory Nerve: A Unique Presentation of a Rare Tumor

Sheikh, Omair A.; Reaves, Ann; Kralick, Francis; Brooks, Ari D.; Musial, Rachel; Gasperino, James

doi:10.3988/jcn.2012.8.1.75

Cited by 12 publications

(12 citation statements)

References 10 publications

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“…7 Malignant peripheral nerve sheath tumors tend to recur locally at the original sites and metastasize to the lungs, liver, lymph nodes, and bones. 10 In this report, we present a case of a primary MPNST at the T-12 and L-1 vertebrae with metastasis to the brain leading to acute hydrocephalus, and provide a detailed clinical and pathological analysis in conjunction with a brief literature review. Ethics approval for this study was provided by the Ethics Committee of Hebei University, and consent to publish the details of this case was obtained from the patient's relatives.…”

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confidence: 99%

Primary malignant peripheral nerve sheath tumor of the spine with acute hydrocephalus: a rare clinical entity

Li¹,

Fan²,

et al. 2014

SPI

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Primary malignant peripheral nerve sheath tumors (MPNSTs) are extremely rare in patients without a history of neurofibromatosis; only 18 cases have been reported in the English-language literature to this point. The authors report their experience with 1 new case of a primary MPNST. A 33-year-old woman presented with low-back pain radiating to the right calf that progressed over 1 year. Magnetic resonance imaging of the spine revealed an intradural extramedullary lesion at the T12–L1 level. The patient was diagnosed with primary MPNST, underwent two surgical excisions and radiation therapy, and developed leptomeningeal metastases as well as brain metastases. The patient revisited the emergency room with sudden loss of consciousness. A brain CT scan displayed bilateral lateral ventricle enlargement, for which a ventriculoperitoneal shunt was inserted. These symptoms have not been described in any previous report. Primary spinal MPNST is an exceedingly rare entity, and the overall prognosis is very poor. To the authors' knowledge, no standard of care for primary spinal MPNSTs has yet been established. All 19 cases of primary spinal MPNSTs are reviewed, and the authors discuss their clinical, radiological, and therapeutic features and outcomes.

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confidence: 99%

Primary malignant peripheral nerve sheath tumor of the spine with acute hydrocephalus: a rare clinical entity

Li¹,

Fan²,

et al. 2014

SPI

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“…MPNSTs may develop within an associated neurofibroma 2,9 . The malignant transformation of a neurofibroma has an extremely poor prognosis with prevalent recurrences and distant metastasis 9,10 .…”

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confidence: 99%

A Case of Recurrence of MPNST in Nf 1 With Intracranial and Hepatic Metastases

Mukherjee¹,

Naveen²,

Manohar³

2013

jemds

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Neurofibromatoses Type 1(NF 1) is an autosomal dominant disease characterized by disordered growth of ectodermal tissues, and is part of a group of disorders called Phakomatoses (neurocutaneous syndrome). NF 1 (aka Von Recklinghausen's disease) patients develop both benign and malignant tumors at an increased frequency. Malignant peripheral nerve sheath tumours (MPNST) is a very rare tumor, with an incidence of 1 per 1,00,000 population. MPNSTs occur in about 2% to 5% of neurofibromatosis patients and constitutes between 3 to 10% of all soft tissue sarcomas. Most MPNSTs are considered as high-grade sarcomas originating from tissues of mesenchymal origin and are biologically aggressive tumours with notoriety for recurrence and metastases.In this paper, we present a case of a 64 yr old male, who was diagnosed with NF 1 at the age of 25 yrs. He presented with a local recurrence of MPNST in left thigh, He was previously operated for MPNST (Stage 1) at the same site with a tumor free margin, 3 yrs back. Thereafter patient was disease free but two and half yrs later developed a swelling at the same site. Diagnostic core needle biopsy demonstrated a high grade MPNST. Metastatic work up yielded intracranial and hepatic metastases.

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“…The differential diagnosis of a cerebellomedullary cistern mass should include meningioma, schwannoma, malignant peripheral nerve sheath tumors, ependymoma, andalthough rare-AOVMs. 25,35,50,54 For CMs located within the cerebellomedullary cistern as well as for any AOVM located within any cistern or suspected to be involving a cranial nerve, we recommend including immunohistochemistry with primary antibody to neurofilament in the histopathology workup. …”

Section: Spinal Accessory Nerve and Aovmsmentioning

confidence: 99%

Angiographically occult vascular malformation of the intracranial accessory nerve: case report

Terterov¹,

McLaughlin²,

Vinters

et al. 2016

JNS

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A ngiogrAphicAlly occult cerebral vascular malformation (AOVM) is a clinicoradiographic term that describes a heterogeneous group of entities, including cavernous malformations (CMs), thrombosed arteriovenous malformations, venous malformations, capillary telangiectasias, and mixed vascular malformations. 48 These account for 5%-15% of all cerebral vascular lesions, with a general population prevalence estimated at 0.3%-0.5%. Approximately half of AOVMs occur sporadically, while the other half are inherited in an autosomaldominant pattern. Given that histologically most AOVMs are CMs, the current literature concerns patient populations with CMs. Indeed, only a minority of AOVMs are venous malformations, arteriovenous malformations, or mixed vascular malformations. 52,56 CMs are found within the brain parenchyma in approximately 95% of cases, with spinal CMs accounting for 5%. Of the intracranial CMs, 80% are supratentorial and 20% are infratentorial.33 CMs usually have a characteristic appearance on MRI with a mixed T2 signal intensity reticulated core surrounded by a rim of decreased signal intensity. However, smaller lesions can appear as small areas of decreased signal intensity. 24,57 Uncommonly, CMs (as the most common form of AOVMs) can be found within the cavernous sinus, basal cisterns, cauda equina, and cranial nerves. 10,41,43,44 Although CMs have been previously reported to be associated with almost all cranial nerves, their involvement of the lower cranial nerves is extremely rare. We report a case of an angiographically occult vascular malformation (AOVM) arising from the intracranial portion of the spinal accessory nerve, with pathological confirmation of nerve fibers traversing the lesion. case report History and PresentationA 46-year-old woman presented with a history of episodic frontal headaches and episodes of nausea and dizabbreviatioNs AVOM = angiographically occult cerebral vascular malformation; CM = cavernous malformation; SMA = smooth muscle actin. Angiographically occult cerebral vascular malformations (AOVMs) are usually found in the supratentorial brain parenchyma. Uncommonly, AOVMs can be found within the cavernous sinus or basal cisterns and can be associated with cranial nerves. AOVMs involving the intracranial segment of the spinal accessory nerve have not been described. A 46-year-old female patient presented with a history of episodic frontal headaches and episodes of nausea and dizziness, as well as gait instability progressing over 6 months prior to evaluation. Imaging revealed a well-circumscribed 3-cm extraaxial T1-weighted isointense and T2-weighted hyperintense contrast-enhancing mass centered in the region of the right lateral cerebellomedullary cistern. The patient underwent resection of the lesion. Although the intraoperative appearance was suggestive of a cavernous malformation, some histological findings were atypical, leading to the final diagnosis of vascular malformation, not otherwise specified. The patient's postoperative course was uneventful with complete resol...

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Malignant Nerve Sheath Tumor of the Spinal Accessory Nerve: A Unique Presentation of a Rare Tumor

Cited by 12 publications

References 10 publications

Primary malignant peripheral nerve sheath tumor of the spine with acute hydrocephalus: a rare clinical entity

Primary malignant peripheral nerve sheath tumor of the spine with acute hydrocephalus: a rare clinical entity

A Case of Recurrence of MPNST in Nf 1 With Intracranial and Hepatic Metastases

Angiographically occult vascular malformation of the intracranial accessory nerve: case report

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