Infantile Myofibromatosis(Congenital Generalized Fibromatosis): Associated with multiple congenital malformations and basaloid follicular hamartomas in the skin.
- Author:
Eun Sook NAM
;
Yoo Hun KIM
;
Han Kyeom KIM
;
Insun KIM
;
Je Geun CHI
- Publication Type:Original Article
- Keywords:
Infantile myofibromatosis;
Congenital generalized fibromatosis;
Congenital mal formation;
Basaloid follicular hamartoma
- MeSH:
Infant;
Male;
Female;
Humans;
Hamartoma
- From:Korean Journal of Pathology
1995;29(6):776-782
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
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.
