Intra-Cranial Malignant Peripheral Nerve Sheath Tumor of Olfactory Nerve: A Case Report and Review of Literature

Abstract Malignant Peripheral Nerve Sheath Tumors (MPNSTs) are one of the very rare high grade malignancies usually affecting extremities or trunk. Incidence is 1/Lac. Intracranial MPNSTs are even rarer, schwannomatous and commonly affecting cranial nerves VIII &VII). Intra-cranial MPNSTs are usually sporadic, arising de novo. The second most common mode of origin is from malignant transformation from pre-existing schwannomas or neurofibroma. We present an extremely rare and probably the first case of intra-cranial malignant peripheral nerve sheath tumor of the olfactory nerve in a non neurofibrosis patient with no prior history of irradiation.

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

Journal of Neurosurgery: Case Lessons ◽

10.3171/case21255 ◽

2021 ◽

Vol 2 (6) ◽

Author(s):

Renato J. Galzio ◽

Mattia Del Maestro ◽

Diamantoula Pagkou ◽

Massimo Caulo ◽

Sofia Asioli ◽

...

Keyword(s):

Peripheral Nerve ◽

Cranial Nerves ◽

Illustrative Case ◽

Nerve Sheath Tumor ◽

Falx Cerebri ◽

Peripheral Nerve Sheath Tumor ◽

Documented Case ◽

Nerve Sheath ◽

First Case ◽

The Right

BACKGROUND The authors reported the first documented case of intracranial extraaxial nonneurofibromatosis type 1–related nontriton malignant peripheral nerve sheath tumor (MPNST) originating from the falx cerebri. OBSERVATIONS A 34-year-old man with headache, short-term memory deficit, postural instability, and blurred vision presented with a large heterogenous contrast-enhanced intraventricular cystic lesion originating from the free margin of the falx cerebri. The patient received surgery using the right posterior interhemispheric approach. Gross total resection was performed, and the inferior border of the falx cerebri was resected. The postoperative course was uneventful. Histological examination revealed hypercellular foci of neoplastic spindle cells with hyperchromatic and wavy nuclei. Hence, a diagnosis of MPNST was made based on concomitant immunochemistry findings, including mouse double minute 2 homolog focal positivity and geographic loss of H3K27me3. The patient received adjuvant radiotherapy, and recurrence was not observed. LESSONS Intracranial MPNSTs are extremely rare tumors, typically originating from the cranial nerves in the posterior cranial fossa. An even rarer variant of these tumors, referred to as malignant intracerebral nerve sheath tumors, may directly arise from the brain parenchyma. The authors reported the first case of an intracranial MPNST originating from the dura mater of the falx cerebri, acting as an extraaxial lesion with prevalent expansion in the right ventricle.

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Glioblastoma of the cerebellopontine angle and internal auditory canal mimicking a peripheral nerve sheath tumor: case report

Journal of Neurosurgery ◽

10.3171/2018.8.jns181702 ◽

2019 ◽

Vol 131 (6) ◽

pp. 1835-1839 ◽

Cited By ~ 1

Author(s):

Hirokazu Takami ◽

Christoph M. Prummer ◽

Christopher S. Graffeo ◽

Maria Peris-Celda ◽

Caterina Giannini ◽

...

Keyword(s):

Peripheral Nerve ◽

Cerebellopontine Angle ◽

Frozen Section ◽

Cranial Nerves ◽

Internal Auditory Canal ◽

Molecular Testing ◽

Nerve Sheath Tumor ◽

Peripheral Nerve Sheath Tumor ◽

Nerve Sheath ◽

First Case

Glioblastoma (GBM) of the internal auditory canal (IAC) is exceedingly rare, with only 3 prior cases reported in the literature. The authors present the fourth case of cerebellopontine angle (CPA) and IAC GBM, and the first in which the lesion mimicked a vestibular schwannoma (VS) early in its natural history. A 55-year-old man presented with tinnitus, hearing loss, and imbalance. MRI identified a left IAC/CPA lesion measuring 8 mm, most consistent with a benign VS. Over the subsequent 4 months he developed facial weakness. The tumor grew remarkably to 24 mm and surgery was recommended; the main preoperative diagnosis was malignant peripheral nerve sheath tumor (MPNST). Resection proceeded via a translabyrinthine approach with resection of cranial nerves VII and VIII, followed by facial-hypoglossal nerve anastomosis. Intraoperative frozen section suggested malignant spindle cell neoplasm, but final histopathological and molecular testing confirmed the lesion to be a GBM. The authors report the first case in which absence of any brainstem interface effectively excluded a primary parenchymal tumor, in particular GBM, from the differential diagnosis. Given the dramatic differences in treatment and prognoses between malignant glioma and MPNST, this case emphasizes the importance of surgical intervention on an aggressively growing lesion, which provides both the best probability of local control and the critical tissue diagnosis.

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Retroperitoneal Malignant Peripheral Nerve Sheath Tumor Replacing an Absent Kidney in a Child

Case Reports in Oncological Medicine ◽

10.1155/2013/627472 ◽

2013 ◽

Vol 2013 ◽

pp. 1-4 ◽

Cited By ~ 1

Author(s):

Samin Alavi ◽

M. T. Arzanian ◽

Yalda Nilipour

Keyword(s):

Peripheral Nerve ◽

Wilms Tumor ◽

Abdominal Mass ◽

Soft Tissue Sarcomas ◽

Nerve Sheath Tumor ◽

Peripheral Nerve Sheath Tumor ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath ◽

First Case ◽

Genitourinary Tract Anomalies

Malignant peripheral nerve sheath tumors (MPNSTs) are nonrhabdomyosarcoma soft tissue sarcomas with rare occurrence in children specially in the retroperitoneum. We describe a young child who presented with an abdominal mass. Both ultrasound and computed tomography revealed a large right-sided abdominal mass in the anatomic place of right kidney, while no kidney or ureter was observed at that side. He underwent surgical resection of the tumor with a primary impression of Wilms tumor. To the authors’ knowledge, this is the first case of retroperitoneal malignant peripheral nerve sheath tumor and absent kidney. This case suggests the very rare probability of association of MPNSTs in children with genitourinary tract anomalies such as renal agenesis.

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Microcystic/Reticular Schwannoma of the Frontal Lobe: An Unusual Occurrence

Case Reports in Pathology ◽

10.1155/2017/4728585 ◽

2017 ◽

Vol 2017 ◽

pp. 1-5 ◽

Cited By ~ 2

Author(s):

Lauren Pearson ◽

Erinc Akture ◽

Julien Wonderlick ◽

Gregory Fuller ◽

Maryam Zenali

Keyword(s):

Frontal Lobe ◽

Cranial Nerves ◽

Neck Region ◽

Nerve Sheath Tumor ◽

Head And Neck Region ◽

Peripheral Nerve Sheath Tumor ◽

Reticular Pattern ◽

Nerve Sheath ◽

First Case ◽

Unusual Occurrence

Schwannoma is a benign peripheral nerve sheath tumor that typically involves cranial nerves of the head and neck region. Intraparenchymal occurrence of this tumor is uncommon. Even rarer in this site is the microcystic/reticular pattern of schwannoma. This histologic variant, first described in 2008, has a predilection for visceral organs. Herein, we report the first case of microcystic/reticular schwannoma of the frontal lobe.

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FORAMINAL NERVE SHEATH TUMORS

Neurosurgery ◽

10.1227/01.neu.0000341632.39692.9e ◽

2009 ◽

Vol 64 (suppl_2) ◽

pp. A33-A43 ◽

Cited By ~ 13

Author(s):

Judith A. Murovic ◽

Iris C. Gibbs ◽

Steven D. Chang ◽

Bret C. Mobley ◽

Jon Park ◽

...

Keyword(s):

Peripheral Nerve ◽

Medical Center ◽

Nerve Sheath Tumor ◽

Peripheral Nerve Sheath Tumor ◽

Central Necrosis ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Neurological Findings ◽

Nerve Sheath ◽

Asymptomatic Case

Abstract OBJECTIVE To conduct a retrospective review of outcomes in 15 patients with 18 foraminal tumors, including 17 benign peripheral nerve sheath tumors and 1 malignant peripheral nerve sheath tumor, who underwent CyberKnife (Accuray, Inc., Sunnyvale, CA) radiosurgery at Stanford University Medical Center from 1999 to 2006. METHODS Symptoms and findings, neurofibromatosis (NF) association, previous radiation, imaging, dosimetry, tumor volume, central necrosis, and the relation of these factors to outcomes were evaluated. RESULTS Before treatment, 1 asymptomatic patient had radiculopathic findings, 3 patients experienced local pain with intact neurological examinations, and 7 patients had radiculopathic complaints with intact (1 patient), radiculopathic (4 patients), or radiculomyelopathic examinations (2 patients). Five patients had myelopathic complaints and findings. Three patients had NF1-associated neurofibromas, 1 patient with NF2 had a schwannoma, and 1 patient had a schwannomatosis-related lesion. Two likely radiation-induced lesions, a neurofibroma and a malignant peripheral nerve sheath tumor, were observed. Prescribed doses ranging from 16 to 24 Gy, delivered in 1 to 3 fractions of 6 to 20 Gy, resulted in maximum tumor doses ranging from 20.9 to 30 Gy. Target volumes ranged from 1.36 to 16.9 mL. After radiosurgery, the asymptomatic case remained asymptomatic, and neurological findings improved. Thirteen of 15 symptomatic patients with (12 patients) or without (3 patients) neurological findings improved (3 cases after resection) or remained stable, and 2 patients worsened. Symptoms and examinations remained stable or improved in 8 (80%) of 10 patients with schwannomas and 3 (60%) of 5 patients with neurofibromas. Tumor volumes decreased in 12 (67%) of 18 tumors and increased in 3 tumors. Tumor volumes decreased in 8 of 10 schwannomas and 3 of 7 neurofibromas. Central necrosis developed in 8 (44%) of 18 tumors. CONCLUSION CyberKnife radiosurgery resulted in pain relief and functional preservation in selected foraminal peripheral nerve sheath tumors and a malignant peripheral nerve sheath tumor. Symptomatic and neurological improvements were more noticeable with schwannomas. Myelopathic symptoms may necessitate surgical debulking before radiosurgery.

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Malignant Peripheral Nerve Sheath Tumor With Rhabdomyosarcomatous Differentiation (Malignant Triton Tumor)

Archives of Pathology & Laboratory Medicine ◽

10.5858/2006-130-1878-mpnstw ◽

2006 ◽

Vol 130 (12) ◽

pp. 1878-1881 ◽

Cited By ~ 5

Author(s):

Christopher J. Stasik ◽

Ossama Tawfik

Keyword(s):

Peripheral Nerve ◽

Correct Diagnosis ◽

Strong Association ◽

Clinical History ◽

Nerve Sheath Tumor ◽

Malignant Triton Tumor ◽

Peripheral Nerve Sheath Tumor ◽

Prognostic Features ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath

Abstract Malignant peripheral nerve sheath tumors arise from Schwann cells or within existing neurofibromas and have a strong association with type 1 neurofibromatosis. These tumors are histologically diverse and may contain malignant areas of divergent mesenchymal differentiation, the most common of which is skeletal muscle (rhabdomyosarcoma). Malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation is also known as malignant triton tumor. Malignant triton tumor has a worse prognosis than classic malignant peripheral nerve sheath tumor does, and the correct diagnosis requires attention to the clinical history and knowledge of the complexities regarding its differential diagnosis. In this review we discuss the clinical, histopathological, immunohistochemical, and prognostic features of this rare neoplasm.

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Somatic Mutations of lats2 Cause Peripheral Nerve Sheath Tumors in Zebrafish

Cells ◽

10.3390/cells8090972 ◽

2019 ◽

Vol 8 (9) ◽

pp. 972 ◽

Cited By ~ 1

Author(s):

Zachary J. Brandt ◽

Paula N. North ◽

Brian A. Link

Keyword(s):

Peripheral Nerve ◽

Somatic Mutations ◽

Tumor Formation ◽

Hippo Signaling ◽

Nerve Sheath Tumor ◽

Peripheral Nerve Sheath Tumor ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath ◽

Early Lethality

The cellular signaling pathways underlying peripheral nerve sheath tumor (PNST) formation are poorly understood. Hippo signaling has been recently implicated in the biology of various cancers, and is thought to function downstream of mutations in the known PNST driver, NF2. Utilizing CRISPR-Cas9 gene editing, we targeted the canonical Hippo signaling kinase Lats2. We show that, while germline deletion leads to early lethality, targeted somatic mutations of zebrafish lats2 leads to peripheral nerve sheath tumor formation. These peripheral nerve sheath tumors exhibit high levels of Hippo effectors Yap and Taz, suggesting that dysregulation of these transcriptional co-factors drives PNST formation in this model. These data indicate that somatic lats2 deletion in zebrafish can serve as a powerful experimental platform to probe the mechanisms of PNST formation and progression.

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Inaccurate summary of peripheral nerve sheath tumors in extradigital sites. Comment on “Hybrid peripheral nerve sheath tumor of the nasal cavity showing schwannomatous, neurofibromatus, and perineuriomatous areas. Med Mol Morphol (2010) 43:82-85”

Medical Molecular Morphology ◽

10.1007/s00795-010-0526-z ◽

2010 ◽

Vol 43 (4) ◽

pp. 253-253 ◽

Cited By ~ 1

Author(s):

Teresa Pusiol ◽

Maria Grazia Zorzi ◽

Doriana Morichetti ◽

Francesco Piscioli

Keyword(s):

Peripheral Nerve ◽

Nasal Cavity ◽

Nerve Sheath Tumor ◽

Peripheral Nerve Sheath Tumor ◽

Nerve Sheath Tumors ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath

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Malignant Peripheral Nerve Sheath Tumor of the Vestibulocochlear Nerve and Brainstem

Neurosurgery ◽

10.1227/neu.0b013e318223bc2a ◽

2011 ◽

Vol 69 (5) ◽

pp. E1152-E1165 ◽

Cited By ~ 6

Author(s):

Kristophe J. Karami ◽

Prashant S. Kelkar ◽

Michael P. Verdon ◽

Inga S. Grills ◽

Dennis I. Bojrab ◽

...

Keyword(s):

Radiation Therapy ◽

Peripheral Nerve ◽

Soft Tissues ◽

Gamma Knife Radiosurgery ◽

Surgical Approaches ◽

Nerve Sheath Tumor ◽

Vestibulocochlear Nerve ◽

Peripheral Nerve Sheath Tumor ◽

Peripheral Nerve Sheath Tumors ◽

Nerve Sheath

Abstract BACKGROUND AND IMPORTANCE Malignant peripheral nerve sheath tumors are the most common malignant mesenchymal tumors of soft tissues, but they are very rare when found to arise from a cranial nerve and when not in association with neurofibromatosis. These tumors are highly malignant and carry a poor prognosis with survival usually less than 6 months. CLINICAL PRESENTATION The authors report the case of a 23-year-old female with no history of phakomatoses, previous irradiation, or known genetic disorders, who presented with a malignant peripheral nerve sheath tumor of the vestibulocochlear nerve and brainstem. Multiple staged skull base approaches were carried out with maximal possible resection. Adjunctive therapies including standard radiation therapy, intensity-modulated radiation therapy, and stereotactic gamma knife radiosurgery were used with an ultimate patient survival of 27 months. CONCLUSION To our knowledge, this is the first report describing a patient with a malignant peripheral nerve sheath tumor of the vestibulocochlear nerve and brainstem treated with staged surgical approaches in conjunction with multiple forms of radiotherapy and having a significant survival of more than 2 years.

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