ABSTRACT
PURPOSE: We encountered a case of lipofibromatosis in a 21-month-old male patient and examined its histopathologic properties. METHODS: We examined morphological aspects of the tumor and immunohistochemical patterns. RESULTS: Tumor proliferation was infiltrative, which did not show apparent encapsulation. Positive immunoreactivity was found for CD-34, CD-99, Ki-67, and connective tissue growth factor/CCN2 in the fibrous region, S-100 in the adipose region, and Notch1 stain was observed in the eccrine sweat gland cells juxtaposed to the tumor adipose tissue, but no reactivity for Bcl-2, alphaSMA, Notch 2-4, CCN1, and CCN3. CONCLUSIONS: The clinical process and immunohistochemical pattern of this case was consistent with the criteria of reported lipofibromatosis. Specific expression of CCN2 might be significant for the development of the tumor.