A male infant case of lipofibromatosis in the submental region exhibited the expression of the connective tissue growth factor.
OBJECTIVE
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.
Kabasawa
Yuhji
Y
Graduate School of Tokyo Medical and Dental University, Tokyo, Japan. kabasawa.osur@tmd.ac.jp
Katsube
Ken-ichi
K
Harada
Hiroyuki
H
Nagumo
Kiyoko
K
Terasaki
Haruka
H
Perbal
Bernard
B
Okada
Norihiko
N
Omura
Ken
K
D000071443:12E7 Antigen; D000273:Adipose Tissue / Q000737:chemistry / Q000473:pathology; D015703:Antigens, CD / Q000032:analysis; D018952:Antigens, CD34 / Q000032:analysis; D015815:Cell Adhesion Molecules / Q000032:analysis; D055513:Connective Tissue Growth Factor; D005350:Fibroma / Q000737:chemistry / Q000378:metabolism / Q000473:pathology*; D006258:Head and Neck Neoplasms / Q000737:chemistry / Q000378:metabolism* / Q000473:pathology*; D006801:Humans; D017874:Immediate-Early Proteins / Q000096:biosynthesis; D007150:Immunohistochemistry; D007223:Infant; D018969:Insulin-Like Growth Factor Binding Proteins / Q000096:biosynthesis; D036341:Intercellular Signaling Peptides and Proteins / Q000096:biosynthesis; D019394:Ki-67 Antigen / Q000032:analysis; D008067:Lipoma / Q000737:chemistry / Q000378:metabolism / Q000473:pathology*; D008297:Male; D009363:Neoplasm Proteins / Q000096:biosynthesis; D055514:Nephroblastoma Overexpressed Protein; D051881:Receptor, Notch1 / Q000096:biosynthesis; D009418:S100 Proteins / Q000032:analysis
D000071443:12E7 Antigen; D015703:Antigens, CD; D018952:Antigens, CD34; C526317:CCN2 protein, human; C526319:CCN3 protein, human; C061828:CD99 protein, human; D015815:Cell Adhesion Molecules; D017874:Immediate-Early Proteins; D018969:Insulin-Like Growth Factor Binding Proteins; D036341:Intercellular Signaling Peptides and Proteins; D019394:Ki-67 Antigen; C497761:NOTCH1 protein, human; D009363:Neoplasm Proteins; D055514:Nephroblastoma Overexpressed Protein; D051881:Receptor, Notch1; D009418:S100 Proteins; D055513:Connective Tissue Growth Factor