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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 ...
IMF tumors are usually painless, well-encapsulated, rubbery to hard, and freely movable-to-fixed masses. [8] They may be evident at birth in up to 60% of cases [4] but generally go undetected until they [9] are diagnosed in the first year of life, [8] uncommonly in older infants and young (<10 years/old) children, [4] or rarely in older children and adults (one individual was diagnosed with ...
Treatment is mainly surgical; radiotherapy or chemotherapy is usually an indication of relapse. [clarification needed] Head and neck desmoid fibromatosis is a serious condition due to local aggression, specific anatomical patterns and the high rate of relapse. For children surgery is particularly difficult, given the potential for growth ...
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]
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]
Desmoid tumors may occur in the head and neck, more commonly among children, and tend to be more aggressive than in other extra-abdominal locations. These tumors constitute up to 23% of extra-abdominal cases. [16] Treatment is typically more aggressive due to the increased dangers of a tumor in the area. [29] [34]
It is diagnosed in children who are usually less than 2 years old or, in up to 20% of cases, develops in utero and is diagnosed in an infant at birth. [ 1 ] The cells involved in FHI include bland fibroblasts / myofibroblasts , mature fat cells , and primitive-appearing spindle-shaped and/or star-shaped cells. [ 2 ]
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).