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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 ...
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 ...
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]
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 ]
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