Inflammatory Myofibroblastic Tumor - An Overview

Introduction:

Inflammatory myofibroblastic tumor (IMT) is a very rare tumor with unknown etiology. It includes different degrees of inflammatory infiltration as well as myofibroblastic proliferation. The lesion has been referred to by several terms, such as lymphoid hamartoma, benign myofibroblastoma, fibrous xanthoma, inflammatory pseudotumor, plasma cell granuloma, myxoid hamartoma, pseudosarcoma, inflammatory myo fibrohistiocytic proliferation, and most recently, inflammatory myofibroblastic tumor.

What Is Inflammatory Myofibroblastic Tumor?

Inflammatory myofibroblastic tumors, or IMTs, are rare tumors in the body's mucosal and mesenteric surfaces. The mouth, lungs, digestive system, eyes, genital tract, nose, and urinary tract all have mucosal surfaces. The mesentery connects the abdominal organs. The most common site of tumor occurrence is the mucosal and mesenteric surfaces. IMTs usually appear first in the lungs, although they can appear in other places such as the bladder, uterus, larynx, stomach, liver, or intestine. IMT, also called inflammatory fibrosarcoma or IMFT, is named after two cell types commonly seen in tumors. It is mostly derived from myofibroblasts involved in wound healing and organ structural maintenance. IMTs also have a lot of immune cells in them, which makes them look like an infection or inflammation.

Inflammatory myofibroblastic tumors are benign, meaning the tumor cells typically do not spread to other body areas. IMT can, however, infiltrate surrounding tissue and result in numerous issues. Important organs like the stomach and lungs may get obstructed by IMT growth. IMT can very rarely spread to distant organs. IMTs can happen at any age, but they are most common in children and young people.

IMT is extremely rare in the maxillofacial area, and it is frequently misdiagnosed as cancer. The diagnosis is still difficult based on the histologic study of the lesions. IMTs have been described in the mandible, gingiva, tongue, hard palate, buccal mucosa, and submandibular salivary gland, among other areas of the oral cavity.

How Is Inflammatory Myofibroblastic Tumor Diagnosed?

While there are certain symptoms that IMT might generate, some individuals with IMT have no symptoms. The tumor's location, along with its size, affects the symptoms of IMT. Clinically, IMTs manifest as firm, painless lumps or swellings. IMTs do not have an exact age inclination, but they tend to affect kids and teens more commonly. Other features could also include fever, sweats at night, weight loss, and general malaise (body ache). The diagnostic procedures include:

• Imaging: The doctor will utilize imaging scans, such as ultrasound, CT (computed tomography), or MRI (magnetic resonance imaging) if they have symptoms of IMT to locate and measure the tumor. When IMTs are found in the head and neck region, CT and MRI scans may not identify the exact lesion as they frequently point to granulomatous illness, aggressive malignant lesion, or infiltrative growth.

• Biopsy: The doctor will use a needle to remove a tiny sample of the tumor to determine whether the tumor is IMT. Under a microscope, a pathologist will examine the sample's cells to determine the type of tumor.

• Molecular Diagnostics: These methods involve examining molecules in a tissue or fluid, such as proteins, DNA (deoxyribonucleic acid), and RNA (ribonucleic acid), to determine the presence of a disease. They are employed because IMTs can be difficult to diagnose and may resemble other tumors or diseases. The physician will test the biopsy sample for indicators that indicate the presence of IMT. This aids them in selecting the optimal course of treatment for the patient.

How Is Inflammatory Myofibroblastic Tumor Managed?

Treatment strategies for inflammatory myofibroblastic tumors (IMTs) vary depending on the tumor's location, protein expression, and existence of metastases. Given the difficulties in achieving complete elimination through surgery and the potential for recurrence, a multidisciplinary approach that combines targeted therapy, chemotherapy, and surgery may be used. The specifics of each case will determine the best course of action. To choose the best course of treatment for IMTs, patients and healthcare professionals must work together to make decisions.

• Surgery - Though IMTs are completely removed, surgery is still the first choice when it comes to treatment. Following surgery, routine monitoring is essential for the early diagnosis of recurrence.

• Chemotherapy - Chemotherapy may be used to treat aggressive or recurring cases to control IMTs that grow more quickly or that return after surgery.

• Targeted Therapy - A potentially effective treatment option is targeted therapy, particularly for IMTs that express particular proteins that are sensitive to novel medications. The consideration of targeted therapy may depend on the outcome of the surgery. These medications may provide more individualized and efficient care to target particular molecular markers within the tumor.

IMTs of the head and neck are treated with aggressive excision, steroids, irradiation, and chemotherapy. A newly developed form of treatment is CO2 (carbon dioxide) laser therapy.

Is Inflammatory Myofibroblastic Tumors (IMTs) Hereditary?

IMTs are not considered hereditary in the context of families. Researchers continue to look into how tumors form, even though it might be difficult to establish a causal relationship. On the other hand, chromosomal abnormalities in which the structures that house genes within cells break and reassemble incorrectly have been observed in some cases of IMT. These modifications may seriously impair regular cell activities. ALK (anaplastic lymphoma kinase) is a key gene linked to IMT that can combine with other genes to change how cells behave. Doctors frequently use tests to detect these chromosomal mutations, especially those affecting the ALK gene, to confirm an IMT diagnosis. This analysis is an essential first step to verify the existence of an IMT and provide direction for treatment choices.

How Is the Prognosis of Inflammatory Myofibroblastic Tumor?

A disease's prognosis, which indicates its long-term effects, depends on several factors:

• Tumor Location: The tumor's location within the body has a major impact on the prognosis.

• Metastasis: The prognosis is frequently worse when it affects other body parts.

• Surgical Excision: The prognosis is greatly influenced by the degree of tumor extraction accomplished during surgery.

Individual traits and these elements interact to determine each patient's prognosis after receiving a diagnosis. Understanding these aspects enables medical professionals to customize treatment strategies and offer more precise prognosis evaluations for individuals suffering from tumors such as IMTs.

Conclusion:

IMT survival rates are often calculated using past data from patient populations. Due to the rarity of IMT cases, these data can be inaccurate and overlook therapeutic advances. Still, most patients with fully excised IMTs have good prognosis, with many living for more than ten years. Despite what historical data suggest, more recent treatments, such as targeted medications and improved surgical methods, can improve prognosis. While lifespan estimates have limits, total surgical excision is still a critical component linked to remarkably high long-term survival rates, indicating a good prognosis for many IMT patients.