Visceral solitary IMF tumors that cause significant tissue injury, are located in vital areas, and/or are life-threatening have been treated by surgical excision or, if surgery is deemed inappropriate, are treated with drugs and/or radiation therapy as indicated in the following section on multiple tumors (with visceral involvement). Surgical excision has also be used if the diagnosis of the tumor is in doubt. In rare cases, aggressive IMF tumors have recurred after regressing and required further observation and/or treatment. Overall, the prognosis in almost all cases of solitary IMF is good to excellent. Multiple tumors (no viscera involvement)
Multiple tumors (no viscera involvement) also may regress spontaneously and carry a good to excellent prognosis even when they invade local tissues. However, neonates presenting with multiple IMF tumors should undergo a through examination including medical imaging tests to determine the extent of their disease, particularly with respect to determining if a visceral tumor(s) is present and therefore that the proper diagnosis is multiple tumors (with viscera involvement). The treatment of multiple tumors (no viscera involvement) is similar to that used for solitary IMF tumors. Multiple tumors (with viscera involvement)
Individuals with multiple tumors (with viscera involvement) have numerous tumors one or more of which are located in and often injure a vital internal organ and may be life-threatening. Cases in which one of the tumors is in a vital organ and seriously symptomatic have been successfully treated with surgical resection.
cells that blend a variable set of features seen in fibroblasts, the most common cell type in connective tissue, with a variable set of features seen in smooth muscle cells.Treatment of IMF tumors depends upon the tumor numbers, locations, and genetic abnormalities found in each individual and often include more than one therapeutic regimen. Individuals with solitary tumors are usually treated by observation with the expectation that many of these tumors will regress spontaneiously. Single tumors located in vital areas (e.g. intracranial tumors) and tumors that do not regress over suitable observation periods are often treated by surgical removal. Multiple tumors (with viscera involvement) and surgically inaccessible life-threatening IMF tumors have been treated with one or a combination of chemotherapy drugs, radiation therapy or, in tumors with certain PDGFRB gene mutations, drugs directed specifically against this mutated genes protein product. Presentation
IMF tumors are usually painless, well-encapsulated, rubbery to hard, and freely movable-to-fixed masses. They may be evident at birth in up to 60% of cases but generally go undetected until they are diagnosed in the first year of life, uncommonly in older infants and young (<10 years/old) children, or rarely in older children and adults (one individual was diagnosed with IMF at 85 years of age). The tumors may be flesh-colored papules or nodules; plaques (i.e. flat-topped papules that are equal to or greater than 10 mm in largest diameter); pedunculated, calcified masses; or ulcerated, necrotic masses. Most of these tumors are <1 cm in diameter but can be much larger, e.g. 5.2 cm in diameter.