Journal of Pathology and Translational Medicine

Original Article

Infantile Myofibromatosis(Congenital Generalized Fibromatosis): Associated with multiple congenital malformations and basaloid follicular hamartomas in the skin.: Eun Sook Nam, Yoo Hun Kim, Han Kyeom Kim, Insun Kim, Je Geun Chi; Korean J Pathol. 1995;29(6):776-782.

Abstract PDF: Infantile myofibromatosis with systemic involvement is a very rare disease and is characterized by numerous nodules composed of spindle cells of a myofibroblastic nature. There are often disseminated throughout the subcutis, muscle, skeleton and viscera. We report an autopsy case of infantile myofibromatosis in a stillborn female fetus of 32 weeks of gestation. The nodules, Imm to 2 cm, were found over the whole body and viscera. The involved viscera were the heart, tongue, esophagus, gastrointestinal tract, portal areas of the liver, spleen anc pancreas. There were also associated malformations, viz., frontal meningoencephalocele, flexion defer-mities, syndactyly, cleft palate, agenesis of corpus callosum, pachygyria, diaphragmatic hemia, renal hypoplasia, etc. Multiple basaloid follicular hamartomas of the skin were noted on the face and extremeties. There are no previous reports in the literature of infantile myofibromatosis in conjunction with the above skin lesion and congenital malformations.

Case Report

Infantile Hemangioendothelioma of the Liver: Brief case report.: Hyang Jeong Jo, Ki Jung Yun, Jae Kyu Lee, Ji Shin Lee, Hyung Bae Moon; Korean J Pathol. 1997;31(6):586-588.

Abstract PDF: Infantile hemangioendothelioma of the liver is a common vascular tumor in infancy. The tumor is usually multinodular or diffuse and classified into two types. We present a case of infantile hemangioendothelioma of the liver, which predominantly consists of type 2. A 4-month-old female was admitted for an evaulation of an abdominal distension. A CT scan of the liver showed a multinodular mass. The right lobectomy was done. Grossly, the mass consisted of round nodules ranging from 2cm to 5cm in diameter. Microscopically, the tumor revealed proliferation of small vascular channels lined by endothelial cells. Bizarre cells and mitotic cells were frequently noted. Vesicular nuclei and multilayering of the endothelial cells were also noted.

Original Article

Infantile Hemangioendothelioma of the Liver: A histological and immunohistochemicalstudy of 4 cases.: Mee Yon Cho, Sun Hee Sung, Soon Hee Jung, Chanil Park; Korean J Pathol. 1995;29(2):145-151.

Abstract PDF: A pathological study was performed on four cases of infantile hemangioendothelioma of the liver. All the patients were between the age of 1 -5 months and the tumors were typical hemangio-endotheliomas, type 1. The tumors were composed basically of two components; the endothelial cell proliferation and the myxoid matrix. The endothelial cells were cytologically innocuous and formed vascular channels of varying sizes and shapes from capillary to sinusoidal and cavernous vessels. Fibrosis of the matrix, albeit not a major component of the tumor, was found particularly near the center. Immunohistochemically, CD31 was expressed strongly in almost all endothelial cells, in contrast to the stain for von Willebrand factor which was only focally and weakly positive. Alpha-fetoprotein was expressed in hepatocytes within the tumor or in hepatocytes around the tumor. Intratumoral bile duct structures were located mainly at peripheral portion. The results indicated that the type I infantile hemangioendothelioma is a tumor of endothelial cells and myxoid stroma, and that the endothelial cells undergo gradual maturation to form sinusoidal and cavernous vessels in accordance with gradual fibrosis of the myxoid stroma.

Case Report

Infantile Fibrosarcoma: A case report.: Chan Pil Park, Geun Shin Lyu, Chan Kum Park, Jung Dal Lee; Korean J Pathol. 1994;28(3):313-315.

Abstract: Fibrosarcoma in newborns and infants, designated as congenital, infantile, or juvenile fibrosarcoma is an uncommon soft tissue neoplasm occurring most frequently during the first year of life. Infantile fibrosarcoma is associated with favorable clinical behavior that is markedly different from that of adult fibrosarcoma., We report a case of infantile fibrosarcoma occured in a 3-year-old boy presenting as a palpable mass in the left lower extremity since 3 months of life. Histologic findings of the tumor are similar to those of f ibrosarcomas in adult.

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