Epidemiology
An emerging molecular entity that can appear in children or adults, in the skin, subcutaneous tissue, or deep soft tissue. Identifying the NTRK fusion is clinically useful because targeted therapies exist for unresectable or advanced disease.
Basic features
It forms a morphological spectrum: at one end, low-grade infiltrative lesions (such as lipofibromatosis-like neural tumor); at the other, high-grade tumors. It usually presents as a soft-tissue mass, most often in the limbs.
Usual treatment
Resection is used for localized disease; if it is unresectable, recurrent, or metastatic, targeted therapy with TRK inhibitors (larotrectinib or entrectinib) is considered.
Outcomes and prognosis
Its behavior is variable: the spectrum ranges from low-grade lesions, with a mainly local risk, to high-grade sarcomas that can metastasize; so morphology, grade, and genetics are combined to plan treatment and follow-up.
