Pathologic Complete Response of a Malignant Peripheral Nerve Sheath Tumor in the Lung Treated With Neoadjuvant Ifosfamide and Radiation Therapy

Abstract BACKGROUND Malignant peripheral nerve sheath tumor (MPNST) is an aggressive neoplasm associated with neurofibromatosis type 1 (NF1). Despite multimodal therapy, clinical outcomes remain poor. To elucidate markers of MPNST treatment response, we retrospectively reviewed the medical records of MPNST patients at a single institution and performed histopathological and immunohistochemical (IHC) analysis for predictive and prognostic features. METHODS We identified 54 consecutive patients treated at University of California San Francisco between 1990 and 2018 that met diagnostic criteria for MPNST on pathologic review with sufficient tissue available for histology and immunohistochemistry (IHC) assays. IHC was performed for Ki-67, EGFR, p53, H3K27me3, neurofibromin, S100, p75NTR, SOX10, p16, and SOX2. Overall survival (OS), metastasis free survival (MFS), and locoregional failure free rate (LFFR), were estimated using the Kaplan-Meier method. Log-rank test, Cox Proportional Hazards regression, and hierarchical clustering were performed in R. RESULTS With a median follow up of 19.2 months, the 5-year OS, MFS, and LFFR were 58%, 68%, and 66%, respectively, with no significant differences between NF1 associated (n=32) and sporadic tumors (n=22). Radiation therapy significantly improved 5-year LFFR (80% versus 49%, p=0.05), but not OS or MFS. Tumor grade was associated with worse OS by Fédération Nationale des Centres de Lutte Contre Le Cancer (FNCLCC) grading (p=0.02). Furthermore, elevated Ki-67 index was associated with worse 5-year OS (39% versus 73% for Ki-67 index ³ 60 and Ki-67 index < 60, p=0.01). Finally, hierarchical clustering of IHC data identified a predictive signature defined by elevated Ki-67 and EGFR expression associated with improved responses to radiation therapy (5-year OS 86% versus 10%, p=0.004). CONCLUSIONS Our data provide insights into the diagnosis and treatment of MPNST. Additional investigation is needed to understand the biologic mechanisms and generalizability of the signatures uncovered in our analysis.

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

Case Reports in Oncological Medicine ◽

10.1155/2011/705345 ◽

2011 ◽

Vol 2011 ◽

pp. 1-5 ◽

Cited By ~ 1

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.

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

Journal of Immunotherapy ◽

10.1097/cji.0000000000000410 ◽

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

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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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