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Infantile myofibromatosis (IMF) is a rare tumor found in 1 in 150,000 to 1 in 400,000 live births. It is nonetheless the most common tumor derived from fibrous connective tissue that occurs primarily in infants and young children.
Infantile digital fibromatosis (IDF), also termed inclusion body fibromatosis or Reye's tumor, usually occurs as a single, small, asymptomatic, nodule in the dermis on a finger or toe [1] of infants and young children. [2] IMF is a rare disorder with approximately 200 cases reported in the medical literature as of 2021. [3]
Subtypes of juvenile fibromatosis include: [3] Infantile myofibromatosis: solitary tumors commonly occurring in the head and neck regions; multiple tumors occurring in the skin, subcutaneous tissue, muscles, and/or less commonly bones; or, rarely, tumors occurring in an internal organ(s). [4] Aponeurotic fibroma; Infantile digital fibromatosis
Diffuse infantile fibromatosis is a rare condition affecting infants during the first three years of life. This condition is a multicentric infiltration of muscle fibers with fibroblasts resembling those seen in aponeurotic fibromas, presenting as lesions and tumors confined usually to the muscles of the arms, neck, and shoulder area [1]: 607 Diffuse infantile fibromatosis is characterized by ...
Juvenile hyaline fibromatosis, also termed fibromatosis hyalinica multiplex juvenilis and the Murray–Puretic–Drescher syndrome, an autosomal recessive inherited genetic disease. [9] Infantile digital fibromatosis, also termed inclusion body fibromatosis [10] or Reye tumor [11] Fibroma of tendon sheath [12]
On gross pathological examination, FHI tumors are soft, poorly demarcated, fibro-fatty masses located in subcutaneous tissues. [10] These tumor masses tend to blend with the surrounding fat tissues, may be lobulated, and in rare cases, especially when large, extend into nearby muscles, nerves, and/or fibrous/connective tissue structures. [10]
Aponeurotic fibroma, also known as calcifying aponeurotic fibroma, and juvenile aponeurotic fibroma is characterized by a lesion that usually presents as a painless, solitary, deep fibrous nodule, often adherent to tendon, fascia, or periosteum, on the hands and feet. [1]
Juvenile hyaline fibromatosis (also known as fibromatosis hyalinica multiplex juvenilis [2] and Murray–Puretic–Drescher syndrome [2]) is a very rare, autosomal recessive disease due to mutations in capillary morphogenesis protein-2 (CMG-2 gene).