scholarly journals Histopathologic findings in malignant peripheral nerve sheath tumor predict response to radiotherapy and overall survival

2020 ◽  
Vol 2 (1) ◽  
Author(s):  
Calixto-Hope G Lucas ◽  
Harish N Vasudevan ◽  
William C Chen ◽  
Stephen T Magill ◽  
Steve E Braunstein ◽  
...  
Keyword(s):  
Overall Survival ◽  
Peripheral Nerve ◽  
San Francisco ◽  
Strong Predictor ◽  
Malignant Neoplasm ◽  
Nerve Sheath Tumor ◽  
Ki 67 ◽  
Peripheral Nerve Sheath Tumor ◽  
Locoregional Failure ◽  
Nerve Sheath

Abstract Background Malignant peripheral nerve sheath tumor (MPNST) is an aggressive and poorly understood malignant neoplasm. Even in the setting of multimodal therapy, the clinical course of MPNST is frequently marked by metastatic conversion and poor overall prognosis, with optimal treatment paradigms for this rare tumor unknown. Methods We reviewed the medical records and histopathology of 54 consecutive patients who were treated at University of California San Francisco between 1990 and 2018. Results Our cohort consisted of 24 male and 30 female patients (median age 38 years). Fédération Nationale des Centres de Lutte Contre Le Cancer (FNCLCC) sarcoma grading criteria segregated patients into groups with differences in overall survival (OS) (P = .02). Increasing Ki-67 labeling index was associated with poor OS (hazard ratio [HR] 1.36 per 10%, P = .0002). Unsupervised hierarchical clustering-based immunohistochemical staining patterns identified 2 subgroups of tumors with differences in H3K27me3, Neurofibromin, S100, SOX10, p16, and EGFR immunoreactivity. In our cohort, cluster status was associated with improved locoregional failure-free rate (P = .004) in response to radiation. Conclusions Our results lend support to the FNCLCC sarcoma grading criteria as a prognostic scheme for MPNST, although few cases of grade 1 were included. Further, we identify increased Ki-67 labeling as a strong predictor of poor OS from MPNST. Finally, we identify a subset of MPNSTs with a predictive immunohistochemical profile that has improved local control with adjuvant radiotherapy. These data provide insights into the grading and therapy for patients with MPNST, although further studies are needed for independent validation.

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.

scholarly journals A Rare Malignant Peripheral Nerve Sheath Tumor of the Maxilla Mimicking a Periapical Lesion

2016 ◽  
Vol 2016 ◽  
pp. 1-5
Author(s):  
José Alcides Arruda ◽  
Pamella Álvares ◽  
Luciano Silva ◽  
Alexandrino Pereira dos Santos Neto ◽  
Cleomar Donizeth Rodrigues ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Peripheral Nerve ◽  
Soft Tissue Sarcomas ◽  
Malignant Neoplasm ◽  
Surgical Approaches ◽  
Type I ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath ◽  
Malignant Soft Tissue Tumor

Malignant peripheral nerve sheath tumor is a malignant neoplasm that is rarely found in the oral cavity. About 50% of this tumor occurs in patients with neurofibromatosis type I and comprises approximately 10% of all soft tissue sarcomas of head and neck region. Intraosseous malignant peripheral nerve sheath tumor of the maxilla is rare. This article is the first to address malignant peripheral nerve sheath tumor of the maxilla presenting as a periapical radiolucency on nonvital endodontically treated teeth in the English medical literature. Surgical approaches to malignant soft tissue tumor vary based on the extent of the disease, age of the patient, and pathological findings. A rare case of intraosseous malignant peripheral nerve sheath tumor is reported in a 16-year-old woman. The patient presented clinically with a pain involving the upper left incisors region and with defined unilocular periapical radiolucency lesion involved between the upper left incisors. An incisional biopsy was made. Histological and immunohistochemical examination were positive for S-100 protein and glial fibrillary acidic protein showed that the lesion was an intraosseous malignant peripheral nerve sheath tumor of the maxilla. Nine years after the surgery, no regional recurrence was observed.

scholarly journals Malignant peripheral nerve sheath tumor - a case report and review

2020 ◽  
Vol 1 (3) ◽  
pp. 16-27
Author(s):  
Danielle Rabelo Gonzalez Veldman ◽  
Fernando Henrique Batista da Mota ◽  
Laís Ribeiro Vieira ◽  
Igor Diego Carrijo dos Santo ◽  
Anna Carolina Araújo Marques ◽  
...  
Keyword(s):  
Case Report ◽  
Peripheral Nerve ◽  
Malignant Tumor ◽  
Myelin Sheath ◽  
Malignant Neoplasm ◽  
Immunohistochemical Analysis ◽  
Distant Metastases ◽  
Nerve Sheath Tumor ◽  
Peripheral Nerve Sheath Tumor ◽  
Nerve Sheath

This case report aims to contribute to the understanding and to present a new look on the treatment of a rare, aggressive and poorly understood pathology, known as malignant tumor of the peripheral nerve myelin sheath. The objective of this research is to help building a better understanding of this pathology and to evaluate what is new in diagnosis and treatment. We used PubMed’s articles with the descriptors: malignant peripheral nerve sheath tumor, Ki67, and malignant tumor immunohistochemistry of the peripheral nerve sheath. 17 articles were selected. We also used the descriptor “sarcoma staging” for the visualization of a book chapter and a journal. Malignant neoplasm of the peripheral nerve myelin sheath is a disease that mainly affects neurofibromatosis-1 or patients with prior radiotherapy, but may occur randomly, as with the patient in question. Due to illness multiple incidence locations, the symptoms may be late and staging is difficult. The staging is performed taking into account the size of the primary tumor, its location, lymph node involvement, presence of distant metastases and degree of cell differentiation. The diagnosis is made after resection of the piece with anatomopathological and immunohistochemical analysis, which may delay the treatment. Therefore, it has been seen that the most common treatment continues to be complete surgery with free margins, but there are promising studies in the genetics field for the treatment and better understanding of this pathology

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

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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