scholarly journals Impact of Multimodality Imaging to Improve Radiation Therapy (RT) Target Volume Definition for Malignant Peripheral Nerve Sheath Tumor (MPNST)

2021 ◽  
Vol 34 (3) ◽  
Author(s):  
Ferrat Dincoglan
Keyword(s):  
Radiation Therapy ◽  
Peripheral Nerve ◽  
Multimodality Imaging ◽  
Target Volume ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Target Volume Definition ◽  
Nerve Sheath

scholarly journals RADT-20. HISTOPATHOLOGIC FINDINGS IN MALIGNANT PERIPHERAL NERVE SHEATH TUMOR ARE BOTH PROGNOSTIC FOR OVERALL SURVIVAL AND PREDICTIVE FOR RESPONSE TO RADIATION THERAPY

2020 ◽  
Vol 22 (Supplement_2) ◽  
pp. ii185-ii186
Author(s):  
Harish N Vasudevan ◽  
Calixto-Hope G Lucas ◽  
William Chen ◽  
Stephen Magill ◽  
Steve Braunstein ◽  
...  
Keyword(s):  
Overall Survival ◽  
Radiation Therapy ◽  
Peripheral Nerve ◽  
Hierarchical Clustering ◽  
Nerve Sheath Tumor ◽  
Ki 67 ◽  
Peripheral Nerve Sheath Tumor ◽  
Prognostic Features ◽  
Nerve Sheath ◽  
Egfr Expression

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.

Malignant Peripheral Nerve Sheath Tumor of the Vestibulocochlear Nerve and Brainstem

Neurosurgery ◽  
2011 ◽  
Vol 69 (5) ◽  
pp. E1152-E1165 ◽  
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.

Malignant Peripheral Nerve Sheath Tumor Arising from a Solitary Neurofibroma in a Patient without Neurofibromatosis Type 1

2016 ◽  
Vol 78 (5) ◽  
pp. 516-521
Author(s):  
Fumihisa SAWADA ◽  
Eiichi MAKINO ◽  
Takenobu YAMAMOTO ◽  
Ryo TANAKA ◽  
Yutaka FUJIWARA ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Neurofibromatosis Type 1 ◽  
Neurofibromatosis Type ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
Solitary Neurofibroma

Malignant peripheral nerve sheath tumor with multilineage divergent differentiation including a neuroblastic component

2021 ◽  
Author(s):  
Lucas F. Abrahao‐Machado ◽  
Carlos E. Bacchi ◽  
Cristielle P. Freitas ◽  
Jason L. Hornick
Keyword(s):  
Peripheral Nerve ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
Divergent Differentiation

scholarly journals RARE-20. MALIGNANT PERIPHERAL NERVE SHEATH TUMOR OF A CRANIAL NERVE IN AN INFANT WITH NEUROCUTANEOUS MELANOSIS

2020 ◽  
Vol 22 (Supplement_3) ◽  
pp. iii446-iii446
Author(s):  
Lacey Carter ◽  
Naina Gross ◽  
Rene McNall-Knapp ◽  
,and Jo Elle Peterson
Keyword(s):  
Peripheral Nerve ◽  
Cranial Nerve ◽  
Leptomeningeal Disease ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Neurocutaneous Melanosis ◽  
Spindle Cell Neoplasm ◽  
Lower Back ◽  
Nerve Sheath ◽  
S 100

Abstract At one month of age, a female presented with a giant congenital nevus along lower back and thighs and hydrocephalus. A ventriculoperitoneal shunt was placed. An MRI was done at six months, initially reported as normal. At eleven months of age, five months after original MRI, patient presented with dysconjugate gaze and lethargy. MRI showed new 3.8 x 3.7 x 3.4 cm right cerebellopontine angle mass extending into Meckel’s cave and foramen ovale along with leptomeningeal disease extending from the mass along the entire length of the spinal cord. Retrospective review of prior MRI revealed subtle leptomeningeal enhancement concerning for neurocutaneous melanosis (NCM). Given the leptomeningeal disease, family elected for open biopsy and debulking of lesion instead of aggressive resection. Histologically, the mass showed hypercellular spindle cell neoplasm with mitotic activity and necrosis mixed with remnants of normal cranial nerve. GFAP was negative, excluding a glioma. HMB-45, MITF, panmelanoma, and Melan-A were negative, excluding melanoma. A negative myogenin stain ruled out ectomesenchymoma. S-100 protein and SOX-10 positivity with variable loss of staining for trimethylation of histone H3 K27 were indicative of malignant peripheral nerve sheath tumor (MPNST). Given the course of the mass, trigeminal nerve MPNST was presumed. Given the poor prognosis of intracranial MPNST and NCM, family elected to forgo treatment and was discharged with hospice. She died 25 days after surgery. Cranial nerve MPNST is rare. MPNST in patients with NCM has not previously been reported to our knowledge.

Chromosomal translocations inactivating CDKN2A support a single path for malignant peripheral nerve sheath tumor initiation

2021 ◽  
Author(s):  
Miriam Magallón-Lorenz ◽  
Juana Fernández-Rodríguez ◽  
Ernest Terribas ◽  
Edgar Creus-Batchiller ◽  
Cleofe Romagosa ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Chromosomal Translocations ◽  
Nerve Sheath Tumor ◽  
Tumor Initiation ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
Single Path
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