Musculoskeletal Fibromatoses: Radiologic-Pathologic Correlation

Abstract

Musculoskeletal fibromatoses represent a wide spectrum of fibroblastic and myofibroblastic neoplasms with similar pathologic appearances and variable clinical behavior. These lesions can be categorized by location (superficial or deep) or by the age group predominantly affected. Superficial fibromatoses in adults (palmar and plantar) and children (calcifying aponeurotic fibroma, lipofibromatosis, and inclusion body fibromatosis) are often small slow-growing lesions; their diagnosis is suggested by location. Deep fibromatoses in adults (desmoid type and abdominal wall) and children (fibromatosis colli and myofibroma and myofibromatosis) are frequently large and more rapidly enlarging; location of these lesions may be nonspecific. Radiographic findings typically are nonspecific. Cross-sectional imaging (ultrasonography, computed tomography, or magnetic resonance [MR] imaging) reveals lesion location, extent, and involvement of adjacent structures for staging and evaluation of local recurrence. MR imaging findings of predominantly low to intermediate signal intensity, nonenhancing bands of low signal intensity on long repetition time MR images that represent collagenized regions, and extension along fascial planes ("fascial tail" sign) add specificity for diagnosis. Additional features that aid in diagnostic specificity include an abdominal wall location related to pregnancy (abdominal wall fibromatosis), a lower neck location in a young child (fibromatosis colli), an adipose component (lipofibromatosis), or multiple lesions in young children (myofibromatosis). Treatment may be conservative or surgical resection, depending on the specific diagnosis. Local recurrence is common after surgical resection owing to the infiltrative growth of these lesions. Recognition that the appearances of the various types of musculoskeletal fibromatoses reflect their pathologic characteristics improves radiologic assessment and helps optimize patient management.

Whole-body magnetic resonance imaging in the diagnosis and follow-up of multicentric infantile myofibromatosis: A case report

“…Myofibromatosis clinically presents as single mass or diffuse multiple nodules that may develop anywhere, from the skin to subcutaneous tissues or viscera, characterized by spindle-shaped cells, SMA and vimentin positivity, related to myofibroblast proliferation; the lesions are often associated with richly vascular central areas, hemangiopericytomalike, frequently displaced by sheets of hyalinization or necrosis and calcification due to extensive apoptosis (3)(4)(5). According to several authors, hemangiopericytoma and infantile myofibromatosis are a continuous spectrum of a single myofibroblast disease (8).…”

“…Solitary or multicentric disease without visceral involvement is usually associated with a very good prognosis with spontaneous regression, whereas the multi- centric form with visceral spread requires aggressive surgery and/or radiotherapy and chemotherapy; however, the prognosis is often poor, particularly when the lungs are involved (1,4,5). A solitary visceral form has rarely been reported, and its prognosis and treatment depend on the involved organ and associated symptoms (7).…”

“…The lesions of myofibromatosis appear on CT scan as masses that exhibit soft tissue attenuation, or mildly higher, although lower attenuation is also possible due to necrosis, and sparse hyperdense loci of calcification may be present as well (3,5).…”

“…CT should be limited to determining eventual lung or bone involvement; the latter is usually seen as lytic lesions, with or without cortical sclerosis, which is more commonly observed when the lesions start to heal. Pulmonary involvement is usually correlated with a worse prognosis (3)(4)(5).…”

“…Conservative management and observation are preferred for the solitary or multicentric forms without visceral involvement, as they usually undergo spontaneous regression; occasionally, surgery is suggested to reduce eventual mass effect symptoms. Aggressive treatment with radiotherapy, chemotherapy and radical surgery is used for disseminated forms with visceral involvement (1,4,5).…”