Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1

Vasconcelos, Roberto André Torres de; Coscarelli, Pedro Guimarães; Alvarenga, Regina; Acioly, Marcus André

doi:10.1590/0004-282x20170052

Cited by 26 publications

(19 citation statements)

References 12 publications

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“…NF1 is an unfavorable prognostic factor of MPNST . Our knowledge on MPNST in children with NF1 comes mainly from case series studies, but an MPNST risk of 0.5% for NF1 patients under 20 years of age has been estimated in one population‐based cohort …”

Section: Introductionmentioning

confidence: 99%

Pediatric malignancies in neurofibromatosis type 1: A population‐based cohort study

Peltonen

Kallionpää

Rantanen

et al. 2019

Intl Journal of Cancer

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Neurofibromatosis type 1 (NF1) is a cancer predisposition syndrome with an incidence of 1:2,000. Patients with NF1 have an increased cancer risk and mortality, but there are no population‐based cohort studies specifically investigating the risk of childhood malignancies. We used the Finnish NF1 cohort to analyze the incidence, risk and prognosis of malignancies in NF1 patients <20 years of age. Persons born in 1987–2011 were included, and 524 persons were followed through the files of the Finnish Cancer Registry from birth up to age 20 years. This amounted to 8,376 person years. Fifty‐three patients had cancer <20 years of age, yielding a standardized incidence ratio (SIR) of 35.6. The most frequent location of pediatric cancers was the central nervous system (CNS); there were 45 cases and the SIR was 115.7. Exclusion of 22 optic pathway gliomas (OPGs) gave an SIR of 59.1 for the CNS and 21.6 for all cancers. There were nine malignant peripheral nerve sheath tumors (MPNSTs); their cumulative risk was 2.7% by age 20. No cases of leukemia were observed. NF1 patients showed considerable excess mortality with a standardized mortality ratio (SMR) of 73.1. The survival of NF1 patients with CNS tumors other than OPGs did not differ from that of non‐NF1 controls (HR 0.64, 95% CI 0.23 to 1.76). In conclusion, brain tumors in childhood and MPNSTs in adolescence are malignancies of major concern in patients with NF1. The risk for myeloid malignancies may not be as high as suggested in the literature.

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Section: Introductionmentioning

confidence: 99%

Pediatric malignancies in neurofibromatosis type 1: A population‐based cohort study

Peltonen

Kallionpää

Rantanen

et al. 2019

Intl Journal of Cancer

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“…The epidemiological, clinical, and therapeutic characteristics were previously published. 37 The histological diagnosis was confirmed by experienced institutional pathologists and reviewed for this study (TMV, WSN). Tumors were considered NF1-associated if the patient had the clinical diagnosis of NF1, based on two or more of the National Institutes of Health criteria.…”

Section: Patients and Tumorsmentioning

confidence: 99%

“…Malignant peripheral nerve sheath tumor (MPNST) is a rare malignant neoplasm, which corresponds to 2%-10% of all soft tissue sarcomas. [1][2][3] These tumors mainly affect adults with a median age of [35][36][37][38][39][40][41][42][43][44] years and rarely affect children. 2,[4][5][6] These are aggressive tumors with a significant susceptibility to metastasize early in their course.…”

Section: Introductionmentioning

confidence: 99%

Prognostic significance of mast cell and microvascular densities in malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1

et al. 2019

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Malignant peripheral nerve sheath tumors (MPNSTs) are rare and aggressive soft tissue sarcomas with a significant susceptibility to metastasize early in their course. Pathogenesis is yet to be fully elucidated. Recently, the essential role of mast cells in the tumor onset of neurofibromatosis type 1 (NF1)‐associated neurofibromas and MPNSTs was confirmed in both experimental and human studies. In this study, we investigate mast cell density (MCD), microvascular density (MVD), and proliferation index (Ki‐67) in MPNST. A secondary aim was to correlate histological staining to clinical data and survival in patients with and without NF1. In total, 34 formalin‐fixed paraffin‐embedded MPNST tissues from 29 patients were eligible. MCD, MVD, and Ki‐67 labeling index (LI) were analyzed in all stained tissues by a computer‐based quantitative algorithm (Aperio ImageScope). In addition, chart review was performed for clinical data and survival analysis. Overall, MCD, MVD, and Ki‐67 LI were evenly distributed throughout tumor tissue. There was a negative correlation of NF1 status (affected, P = 0.037), tumor size (>10 cm, P = 0.023), and MVD in the tumor periphery (higher tercile, P = 0.002) to survival. Multivariate analysis confirmed the association of MVD in the tumor periphery (higher tercile, P = 0.019) with a decreased overall survival. Diverse mast cell and microvascular distributions suggest that angiogenesis in MPNST occurs independently. The role of mast cells in tumor progression is unclear and lacks prognostic value. Higher MVD has prognostic significance with possible therapeutic implications in MPNST.

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“…Malignant peripheral nerve sheath tumor is associated with an overall poor prognosis. Poorly differentiated, large, NF1‐associated and/or recurrent disease are considered adverse prognostic features …”

Section: Malignant Peripheral Nerve Sheath Tumormentioning

confidence: 99%

“…Poorly differentiated, large, NF1-associated and/or recurrent disease are considered adverse prognostic features. 35,30 Points for the surgeon:…”

Section: Prognosismentioning

confidence: 99%

Pathology of retroperitoneal sarcomas: A brief review

Renne

Iwenofu

2017

Journal of Surgical Oncology

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Sarcomas represent a highly heterogeneous group of tumors as reflected in the significant overlap between their histologic phenotypes between the different types, posing diagnostic challenges for the pathologist. Definitive tumor classification is increasingly important because of prognostication and emergence of targeted therapies for some of the sarcoma types. In this review, we highlight pertinent pathologic and molecular aspects of sarcomas common in the retroperitoneum, relevant to the surgical oncologist.

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Malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1

Abstract: Tumor size and NF1 status were the most important predictors of overall survival in our population.

Cited by 26 publications

References 12 publications

Pediatric malignancies in neurofibromatosis type 1: A population‐based cohort study

Pediatric malignancies in neurofibromatosis type 1: A population‐based cohort study

Prognostic significance of mast cell and microvascular densities in malignant peripheral nerve sheath tumor with and without neurofibromatosis type 1

Pathology of retroperitoneal sarcomas: A brief review

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