What is Infantile Myofibromatosis? RareDiseases Benign Tumor InfantileMyofibromatosis
Case reports Infantile myofibromatosis , a rare disorder, is one of the most common proliferative fibrous tumors of infancy. First described in 1954, it is characterized by the proliferation of fibroblasts and myofibroblasts. The tumors can develop in the skin, subcutaneous tissue, muscle, or bone. Infantile myofibromatosis is a benign tumor that normally develops during infancy and childhood, with 90% of instances occurring before the age of two.
It has been observed that infantile myofibromatosis can exhibit both autosomal-recessive and autosomal dominant inheritance patterns. Several pedigrees have been shown to have consanguinity, which has been linked to an AR-pattern of inheritance. A vast number of pedigrees with sick individuals found throughout generations support the theory that IM is an AD disease.
Soft tissue lesions are more common in children, although they can occur at any age and, more intriguingly, they can reverse spontaneously. However, because of cardiac or gastrointestinal problems, visceral lesions are associated with substantial morbidity and mortality despite chemotherapy and surgery, whereas the other kinds have a great prognosis with surgery alone. Intracranial involvement is uncommon, with most lesions occurring in the skull or dura, with varying intracranial extension.
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