scholarly journals Complete Response to Pembrolizumab in a Patient with Malignant Peripheral Nerve Sheath Tumor: The First Case Reported

2017 ◽  
Keyword(s):  
Peripheral Nerve ◽  
Complete Response ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
First Case

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

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.

scholarly journals Advanced Epithelioid Malignant Peripheral Nerve Sheath Tumor Showing Complete Response to Combined Surgery and Chemotherapy: A Case Report

2011 ◽  
Vol 2011 ◽  
pp. 1-5 ◽  
Author(s):  
Tomohiro Minagawa ◽  
Ryuta Shioya ◽  
Chigusa Sato ◽  
Ryuji Shichinohe ◽  
Go Yasui ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Lung Metastases ◽  
English Literature ◽  
Complete Response ◽  
Nerve Sheath Tumor ◽  
Thoracoscopic Resection ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
Therapeutic Procedures ◽  
Epithelioid Variant

Malignant peripheral nerve sheath tumor (MPNST) is a rare high-grade soft tissue sarcoma. The epithelioid variant accounts for 5% or less of MPNSTs; the clinical behavior of this variant is unclear. Reports of approximately 40 cases are available in the English literature; however, most reports addressed clinicopathological features rather than therapeutic procedures or clinical courses. We describe a case of a 62-year-old male with an epithelioid MPNST of the left foot. Multiple lung metastases developed after radical surgery on the primary lesion. The response to adjuvant chemotherapy including doxorubicin and ifosfamide was favorable, and thoracoscopic resection was subsequently performed on the remaining three metastases. No evidence of recurrence or metastasis was observed at the 12-month followup after the first operation. Further followup and chemotherapy may be required.

Pembrolizumab Achieves a Complete Response in an NF-1 Mutated, PD-L1 Positive Malignant Peripheral Nerve Sheath Tumor

2022 ◽  
Vol Publish Ahead of Print ◽  
Author(s):  
Kristian Larson ◽  
Atlantis Russ ◽  
Hina Arif-Tiwari ◽  
Daruka Mahadevan ◽  
Alex Elliott ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Complete Response ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath

Low-grade malignant peripheral nerve sheath tumor: a report of the first case in the breast and literature review

Apmis ◽  
2016 ◽  
Vol 124 (5) ◽  
pp. 428-430 ◽  
Author(s):  
Jasmina Redzepagic ◽  
Faruk Skenderi ◽  
Jasmina Bajrovic ◽  
Vanesa Beslagic ◽  
Nermina Ibisevic ◽  
...  
Keyword(s):  
Literature Review ◽  
Peripheral Nerve ◽  
Low Grade ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
First Case

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

2019 ◽  
Vol 131 (6) ◽  
pp. 1835-1839 ◽  
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.

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

2018 ◽  
Vol 32 (3) ◽  
pp. 469-472
Author(s):  
Varun Aggarwal ◽  
Amit Narang ◽  
Chandni Maheshwari ◽  
Divya Kavita
Keyword(s):  
Peripheral Nerve ◽  
De Novo ◽  
Cranial Nerves ◽  
Olfactory Nerve ◽  
Prior History ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath ◽  
First Case

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.

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

2013 ◽  
Vol 2013 ◽  
pp. 1-4 ◽  
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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