RESUMEN

BACKGROUND: Intravascular fasciitis (IVF) is a rare nodular fasciitis that often involves the layers and lumens of blood vessels; therefore, it is easily misdiagnosed as a malignant tumor with invasion into blood vessels. CASE SUMMARY: A 13-year-old boy was admitted due to a mass on the left side of his neck. Duplex ultrasonography revealed a circular solid hypoechoic mass in the external jugular vein, and magnetic resonance imaging revealed an enhanced longitudinal mass-like lesion in the left supraclavicular fossa. Surgical treatment was arranged and completed, histopathological analysis showed a large amount of spindle cell proliferation, and immunohistochemistry showed that the spindle cells were positive for the expression of vimentin, caldesmon, and smooth muscle actin and negative for the expression of S-100 protein, desmin, CD34, and c-kit; Ki-67 staining revealed a low proliferative index (5%-10%), which confirmed the differentiation characteristics of myofibroblasts. Fluorescence in situ hybridization detected the rearrangement of USP6. IVF was subsequently diagnosed. CONCLUSION: IVF is characterized by intraluminal, intramural and extramural involvement of small to large arteries or veins. Unless the doctor has a deep understanding of the disease or suspects that there is an initial indicator, IVF may be confused with other intravascular malignancies, leading to unnecessary radical surgery. Imaging examination combined with histopathological examination can improve the diagnostic accuracy of this disease.