A male infant case of lipofibromatosis in the submental region exhibited the expression of the connective tissue growth factor

PurposeWe encountered a case of lipofibromatosis in a 21-month-old male patient and examined its histopathologic properties.MethodsWe examined morphological aspects of the tumor and immunohistochemical patterns.ResultsTumor 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, αSMA, Notch 2-4, CCN1, and CCN3.ConclusionsThe 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.