The 2020 WHO classification of tumors of soft tissue: selected changes and new entities

JH Choi, JY Ro - Advances in Anatomic Pathology, 2021 - journals.lww.com
Soft tissue tumors are a relatively rare and diagnostically challenging group of neoplasms
that can have varying lines of differentiation. Accurate diagnosis is important for appropriate …

The 2020 WHO classification: what's new in soft tissue tumor pathology?

ME Kallen, JL Hornick - The American journal of surgical …, 2021 - journals.lww.com
The fifth edition of the World Health Organization Classification of Tumors of Soft Tissue and
Bone was published in early 2020. The revisions reflect a consensus among an …

Malignant peripheral nerve sheath tumor: models, biology, and translation

BN Somatilaka, A Sadek, RM McKay, LQ Le - Oncogene, 2022 - nature.com
Malignant peripheral nerve sheath tumors (MPNSTs) are aggressive, invasive cancer that
comprise around 10% of all soft tissue sarcomas and develop in about 8–13% of patients …

ERN GENTURIS tumour surveillance guidelines for individuals with neurofibromatosis type 1

C Carton, DG Evans, I Blanco, RE Friedrich… - …, 2023 - thelancet.com
Summary Background Neurofibromatosis type 1 (NF1) is a multisystem genetic disorder,
predisposing development of benign and malignant tumours. Given the oncogenic potential …

A novel group of spindle cell tumors defined by S100 and CD34 co‐expression shows recurrent fusions involving RAF1, BRAF, and NTRK1/2 genes

AJH Suurmeijer, BC Dickson… - Genes …, 2018 - Wiley Online Library
Tumors characterized by co‐expression of S100 and CD34, in the absence of SOX10,
remain difficult to classify. Triggered by a few index cases with monomorphic …

MEK inhibitors for neurofibromatosis type 1 manifestations: Clinical evidence and consensus

PMK De Blank, AM Gross, S Akshintala… - Neuro …, 2022 - academic.oup.com
The wide variety of clinical manifestations of the genetic syndrome neurofibromatosis type 1
(NF1) are driven by overactivation of the RAS pathway. Mitogen-activated protein kinase …

Diagnostic pathology of tumors of peripheral nerve

SM Belakhoua, FJ Rodriguez - Neurosurgery, 2021 - journals.lww.com
Neoplasms of the peripheral nervous system represent a heterogenous group with a wide
spectrum of morphological features and biological potential. They range from benign and …

Biology and management of undifferentiated pleomorphic sarcoma, myxofibrosarcoma, and malignant peripheral nerve sheath tumors: state of the art and …

BC Widemann, A Italiano - Journal of clinical oncology, 2018 - ascopubs.org
Undifferentiated pleomorphic sarcomas, myxofibrosarcomas, and malignant peripheral
nerve sheath tumors are characterized by complex genomic characteristics and aggressive …

[HTML][HTML] Multiple Nf1 Schwann cell populations reprogram the plexiform neurofibroma tumor microenvironment

LJ Kershner, K Choi, J Wu, X Zhang, M Perrino… - JCI insight, 2022 - ncbi.nlm.nih.gov
To define alterations early in tumor formation, we studied nerve tumors in neurofibromatosis
1 (NF1), a tumor predisposition syndrome. Affected individuals develop neurofibromas …

Genomic patterns of malignant peripheral nerve sheath tumor (MPNST) evolution correlate with clinical outcome and are detectable in cell-free DNA

I Cortes-Ciriano, CD Steele, K Piculell, A Al-Ibraheemi… - Cancer discovery, 2023 - AACR
Malignant peripheral nerve sheath tumor (MPNST), an aggressive soft-tissue sarcoma,
occurs in people with neurofibromatosis type 1 (NF1) and sporadically. Whole-genome and …