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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 ...
This form of IMF, particularly in cases with tumors involving the gastrointestinal tract, heart, and/or lung, [6] has a far higher morbidity and mortality than the other forms [8] with death occurring in infants during the first weeks to 4 months of life [4] in 30–70% of cases [9] Death is typically due to a tumor's compression of vital ...
Other names include musculoaponeurotic fibromatosis, referring to the tendency of these tumors to be adjacent to and infiltrating deep skeletal muscle, aggressive fibromatosis and desmoid tumor. A clear difference should be made between intra-abdominal and extra-abdominal localizations. Fibromatosis is a different entity from neurofibromatosis.
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]
Fibromatosis colli (FMC), also termed sternocleidomastoid tumor of infancy, pseudotumor of infancy, [1] and infancy sternocleidomastoid pseudotumor, [2] is an uncommon (incidence: 0.4%–1.3% of live births), congenital tumor in one of the two sternocleidomastoid neck muscles although rare cases have presented with a FMC tumor in both sternocleidomastoid muscles. [3]
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]
Gardner syndrome is also associated with familial adenomatous polyposis and may manifest as aggressive fibromatosis (desmoid tumors) of the retroperitoneum. [11] Desmoid tumors arise most frequently from the aponeurosis of the rectus abdominal muscle of multiparous women. The extra-abdominal form is rare and desmoids of the breast may arise in ...
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).