"Immunohistochemistry stains are usually positive for S100 and CD34 whereas reactivity for SOX10 is absent 1,2. Most of them seem to retain HZ3K27me3 expression in contrast to many malignant nerve sheath tumours 4. Apart from that they usually show pan-TRK and TRK-A immunoreactivity 1,2."
"The prognosis seem to be related to the histological grade 1. The benign lipofibromatosis-like neural tumours seem to have a predisposition for recurrence. Higher-grade neoplasms might behave more aggressively and metastasise. However, they are therapeutically targetable via oncogenic tropomyosin receptor kinase such as TRK-A, TRK-B and TRK-C potentially improving prognosis 1,2."
"NTRK-rearranged soft tissue neoplasms (emerging), lipofibromatosis-like neural tumours or NTRK-positive tumour-resembling peripheral nerve sheath tumours are a group of rare molecularly defined spindle cell neoplasms excluding infantile fibrosarcoma 1.2. The tumours form a provisional category of uncertain differentiation, including lipofibromatosis-like neural tumours and tumours that are similar to peripheral nerve sheath tumours."
"NTRK-rearranged soft tissue neoplasms (emerging), lipofibromatosis-like neural tumours or NTRK-positive tumour-resembling peripheral nerve sheath tumours are a group of rare molecularly defined spindle cell neoplasms excluding infantile fibrosarcoma 1.2. The tumours form a provisional category of uncertain differentiation, including lipofibromatosis-like neural tumours and tumours that are similar to peripheral nerve sheath tumours."
"Many NTRK-rearranged soft tissue neoplasms occur in children and adolescents in the first two decades of life especially lipofibromatosis-like neural tumours and more than half of the cases of NTRK-positive tumour-resembling peripheral nerve sheath tumours occur in the paediatric group 1,2."
"Many NTRK-rearranged soft tissue neoplasms occur in children and adolescents in the first two decades of life especially lipofibromatosis-like neural tumours and more than half of the cases of NTRK-positive tumour-resembling peripheral nerve sheath tumours occur in the paediatric group 1,2."
"The diagnosis of NTRK-rearranged soft tissue neoplasms is established with histological, immunohistochemical and molecular pathological criteria."
"detection of NTRK fusions"
"Most NTRK-rearranged soft tissue tumours present as painless palpable soft tissue mass 1."
"NTRK-rearranged soft tissue neoplasms show a wide range of histological morphologies often displaying a monomorphic spindle cell morphology, infiltrative growth and immunoreactivity to S100 and CD34 1,2."
"The aetiology of NTRK-rearranged soft tissue neoplasms is unknown 1."
"NTRK-rearranged soft tissue neoplasms are usually found in the superficial and deep soft tissues of the trunk and the extremities 1."
"There are no specific definitions of the macroscopic appearance of NTRK-rearranged soft tissue neoplasms 1."
"Histologically NTRK-rearranged soft tissue neoplasms form a morphological spectrum and are characterised by different features 1-3."
"Immunohistochemistry stains are usually positive for S100 and CD34 whereas reactivity for SOX10 is absent 1,2. Most of them seem to retain HZ3K27me3 expression in contrast to many malignant nerve sheath tumours 4. Apart from that they usually show pan-TRK and TRK-A immunoreactivity 1,2."
"NTRK-rearranged soft tissue neoplasms often harbour fusions of the NTRK1 gene with different partner genes and less often NTRK2 or NTRK3 aberrations 1-3."
"On PET-CT NTRK-rearranged soft tissue neoplasms seem to show FDG avidity 6."
"The prognosis seem to be related to the histological grade 1. The benign lipofibromatosis-like neural tumours seem to have a predisposition for recurrence. Higher-grade neoplasms might behave more aggressively and metastasise. However, they are therapeutically targetable via oncogenic tropomyosin receptor kinase such as TRK-A, TRK-B and TRK-C potentially improving prognosis 1,2."
"The prognosis seem to be related to the histological grade 1. The benign lipofibromatosis-like neural tumours seem to have a predisposition for recurrence. Higher-grade neoplasms might behave more aggressively and metastasise. However, they are therapeutically targetable via oncogenic tropomyosin receptor kinase such as TRK-A, TRK-B and TRK-C potentially improving prognosis 1,2."
"NTRK-rearranged soft tissue neoplasms have been added as an emerging entity of uncertain differentition into the WHO classification of soft tissue tumours in 2020 1."
"The diagnosis of NTRK-rearranged soft tissue neoplasms is established with histological, immunohistochemical and molecular pathological criteria."
"NTRK-rearranged soft tissue neoplasms often harbour fusions of the NTRK1 gene with different partner genes and less often NTRK2 or NTRK3 aberrations 1-3."
Expected headings
"Immunophaenotype"
"The diagnosis of NTRK-rearranged soft tissue neoplasms is established with histological, immunohistochemical and molecular pathological criteria."
"form, location and size"
"There are no specific definitions of the macroscopic appearance of NTRK-rearranged soft tissue neoplasms 1."