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Cytogenetically, congenital infantile fibrosarcoma is characterized by the majority of cases having a translocation between chromosomes 12 and 15 (notated as t(12;15)(p13;q25)) that results in formation of the fusion gene, ETV6-NTRK3, plus individual cases exhibiting trisomy for chromosomes 8, 11, 17, or 20. [3]
Infantile fibrosarcoma, also termed congenital infantile fibrosarcoma and fibrosarcoma, infantile type. [36] Malignant FMTs. Solitary fibrous tumor, malignant type, a malignant form of the solitary fibrous tumors [37]
Mesoblastic nephroma and congenital infantile sarcoma appear to be the same diseases with mesoblastic lymphoma originating in the kidney and congenital infantile sarcoma originating in non-renal tissues. [4] [6] [9] [10] Rhabdoid tumor, which accounts for 5-10% of childhood kidney neoplasms, occurs predominantly in children from 1 to 2 years of ...
M8810/3 Fibrosarcoma, NOS M8811/0 Fibromyxoma Myxoid fibroma; Myxofibroma, nos; M8811/3 Fibromyxosarcoma M8812/0 Periosteal fibroma (C40._, C41._) Periosteal sarcoma, NOS; M8813/0 Fascial fibroma M8813/3 Fascial fibrosarcoma M8814/3 Infantile fibrosarcoma Congenital fibrosarcoma; M8815/0 Solitary fibrous tumor Localized fibrous tumor
ETV6-NTRK3 gene fusion is the translocation of genetic material between the ETV6 gene located on the short arm (designated p) of chromosome 12 at position p13.2 (i.e. 12p13.2) and the NTRK3 gene located on the long arm (designated q) of chromosome 15 at position q25.3 (i.e. 15q25.3) to create the (12;15)(p13;q25) fusion gene, ETV6-NTRK3.
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 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; Aggressive infantile fibromatosis
Embryonal rhabdomyosarcoma (ERMS) is the most common histological variant, comprising about 60–70% of childhood cases. It is most common in children birth to four years old, with a maximum reported incidence of four cases per million children.