What is myofibroblastoma

Myofibroblastoma is a rare tumor disease. The patient's symptoms are not obvious in the early stages and can occur anywhere in the body. It is more common in children, and the tumors often occur in the lungs and greater omentum of children. In the early stages of the disease, children may experience varying degrees of fever that lasts for a relatively long time, unexplained weight loss, local pain, and anemia is often found during examination. There is currently no clear answer to the specific cause of this disease in clinical practice. The treatment method is mainly surgical operation, with early detection and early resection. Let's take a closer look at what myofibroblastoma is.

It is common in children and adolescents, with an average age of 10 years old. It can also occur in adults, and is slightly more common in women. It occurs in soft tissues and internal organs and can be located anywhere in the body, with the most common sites being the lungs, greater omentum, and mesentery. The greater omentum is the most common site outside the lung, accounting for 43% of cases outside the lung. Other sites include soft tissue, mediastinum, gastrointestinal tract, pancreas, genitals, oral cavity, breast, nerves, bones and central nervous system.

1. Clinical manifestations of inflammatory myofibroblastic tumor

Depending on the site of onset, the onset is often insidious. Clinical symptoms are mostly caused by the tumor itself and its compression of surrounding organs. There may also be fever, weight loss, pain, anemia, thrombocytosis, accelerated erythrocyte sedimentation rate, etc. The clinical symptoms are similar to those of malignant tumors, but they lack specificity. Symptoms and signs often disappear after tumor resection.

2. Imaging manifestations of inflammatory myofibroblastic tumor

The manifestations in different parts are diverse and lack specific signs.

1. IMT occurring in the lungs is a single lesion, mostly located under the peripheral pleura of the lung.

2. The boundary of central lesions is clear. It may be accompanied by atelectasis and various forms of calcification can be seen inside. It is more common in children.

3. Peripheral lesions appear as irregular tumors. The tumor-lung interface is blurred. Thick and long spicules or thorn-like protrusions can be seen, which are different from the short spicules of lung cancer. At a certain level of the lesion, one side of the edge can be seen to be straight and knife-cut-like. This is the "straight sign." It may be caused by traction of fibrosis at the edge of the lesion, or it may be related to the formation of the lesion along the edge of the lobe or segment; the edge of the lesion may also change into a sharp angle, which may be caused by pleural adhesion and fibrous tissue proliferation around the lesion; these signs can be used as a basis for distinguishing benign and malignant lung tumors.

Extrapulmonary IMT often presents as a single soft tissue tumor. Enhanced scanning shows uniform or uneven moderate to significant enhancement, with no obvious specific manifestations.