Ossifying Fibromyxoid Tumor - Causes, Symptoms, and Treatment

Introduction

Ossifying fibromyxiod tumor (OFMT) is a rare soft tissue cancer. There are only 300 cases diagnosed worldwide. OFMT can spread and affect different body organs. A tumor cell is a rapidly growing cell mass in the body. Tumor cells can be benign or malignant. Benign tumors are non-cancerous, whereas malignant tumors are cancerous and metastasizing. Metastasis is the process by which tumors invade other body organs and damage them. The exact cause of OSMT still needs to be clarified. It affects the extremities of the body, having the potential to be malignant.

OFMT is asymptomatic and a very rare but fatal condition. OFMT was found as aggressive in a small percentage. Most tumors of OFMT behave benignly and rarely metastasize to distant places. Therefore, accurate diagnosis is clinically crucial to ensure proper therapy and prognosis of the disease. The tumor resection is only the treatment of the choice, along with follow-up. After complete surgical excision, 17 percent to 27 percent of patients report local recurrence, while metastases are infrequent, with the typical OFMT's metastatic risk likely less than five percent . The introduction of new diagnosing techniques aids in reaching an accurate diagnosis for these potentially malignant (cancerous) tumors. In addition, these new techniques prevent the overdiagnosis of diseases such as osteosarcoma (cancer of the bone).

What Is Ossifying Fibromyxoid Tumor?

Ossifying fibromyxiod tumor (OFMT) is a rare malignant tumor of musculoskeletal. It occurs in adults of all ages (14 years to 83 years) but is most common in middle-aged individuals (50 years). It is most commonly seen in males. This tumor occurs in soft tissues or skeletal muscles of the limbs. In addition, OFMT is seen in the neck, head, mouth, chest, and breast. Tumors are small-sized, ranging from one to 14 centimeters (average four to five cm). Tumors are situated deep inside the skin of the patients. The tumors of OFMT have a lobulated appearance surrounded by fibrous capsules of bone. Most tumors (81 percent ) have the incomplete shell of mature, metaplastic woven, or lamellar bone in the capsular region, which is laid down by reactive osteoblasts (bone-forming cells). The tumors of OFMT are painless. The tumors of OFMT rarely metastases to distant places such as the lungs and soft tissues, including the opposite extremity (thigh), the hip region, and the neck region.

What Are the Causes of Ossifying Fibromyxoid Tumor?

Ossifying Fibromyxoid Tumor (OFMT) is a rare, benign tumor that most commonly affects middle-aged adults. The exact cause of OFMT is not yet fully understood, but several factors may contribute to its development.

The following are the causes of ossifying fibromyxoid tumor:

• Genetic Factors: Genetic mutations or abnormalities may be involved in the development of OFMT. Studies have found that certain genetic alterations, such as the fusion of the PHF1 and TFE3 genes, are commonly present in OFMTs.

• Trauma: Some studies suggest that OFMTs may develop in response to trauma or injury. It is thought that the trauma may trigger the abnormal growth of cells that can lead to the formation of a tumor.

• Hormones: OFMTs have been found to occur more frequently in women than in men. This suggests that hormones may play a role in the development of these tumors.

• Environmental Factors: Exposure to certain environmental toxins or chemicals may also contribute to the development of OFMTs. However, there is currently no strong evidence to support this theory.

• Immune System Dysfunction: OFMTs may develop due to abnormalities in the immune system. It is thought that a malfunctioning immune system may fail to recognize and destroy abnormal cells, leading to the growth of tumors.

What Are the Symptoms of Ossifying Fibromyxoid Tumor?

OFMT is aggressive and rapidly growing in nature. It is usually asymptomatic. The tumor is painless, and patients usually do not experience pain.

The following are the symptoms of ossifying fibromyxiod tumor:

• The discomfort felt by patients.

• Swelling in the limbs depends on the location of the tumor.

• Paresthesia (a condition in which the patient feels a tingling sensation in the leg, arm, and skin).

• Difficulty in breathing in case of nasal congestion.

• Fever.

• Weight loss.

• Lightheadedness and confusion.

• Tachycardia (high heart rate).

• Syncope (loss of consciousness).

• Fatigue.

How Is Ossifying Fibromyxoid Tumor Diagnosed?

Ossifying fibromyxoid tumors can be diagnosed by the histological features of the tumors.

The following are the histological features of the tumor of OFMT:

• The tumor of OFMT has nodular extension in the cells.

• It is covered by a fibrous capsule of bone.

• It is round, small, or oval in shape.

• It is situated deep in the skin.

• It has high cellularity (more or a dense number of cells).

• High nuclearity (more number of nuclei in the tumor cells, which promotes the growth of the tumor).

• Large giant cells are present around the tumor cells.

• Metastases tumor shows invasion in neighboring cells.

OFMT can be diagnosed by further examination and investigation.

The following are the ways to diagnose ossifying fibromyxoid tumors:

• Physical Examination - The tumors of OFMT can be palpated during the physical examination by the doctors.

• Biopsy - Biopsy is a medical test in which suspected tissue is taken from the patient and examined under a microscope.

• Computed Tomography (CT Scan) - Computed tomography helps show the lack of adjacent osseous (bone) reaction to the capsular or intralesional (inside the bone) metaplastic bone.

• Scintigraphy - Scintigraphy shows heterogeneous uptake within ossified areas, consistent with osteoblast activity

• Magnetic Resonance Imaging (MRI) - On MRI, the signal intensity is low to intermediate in the case of OFMT. MRI shows metastases evidence, if any.

• Radiographic Examination - On X-ray, patients with OFMT can be diagnosed through the images of the tumors. In addition, the tumor location, origin, extent, and depth can be seen in radiographic images.

• Histological Examination - The evidence of histological changes in the tumor cell confirms OFMT.

How Is Ossifying Fibromyxoid Tumor Treated?

1. Surgical excision remains the mainstay of the treatment of OFMT.

2. Because this tumor is prone to local recurrences and distant metastases, surgical removal, and vigilant monitoring are the preferred treatments, particularly in atypical and malignant kinds.

3. Only the malignant form of OFMT has been known to have metastases, whereas 10 percent of individuals with metastatic disease died due to their illness.

4. Therefore, surgical management is the initial treatment of choice for head and neck OFMT.

5. Local excision is the primary therapy, which is curative in most cases. In recurrent cases, wide and more radical excisions with secure margins would be the appropriate treatment.

Conclusion

An uncommon neoplasm with intermediate biologic potential, ossifying fibromyxoid tumor often develops in the trunk or extremities. However, it shares a histologic spectrum and morphologic traits with many other soft tissue neoplasms, including homogenous polygonal cells inside fibromyxoid stroma and traditionally a peripheral rim of lamellar bone. Even though most OFMTs behave benignly, getting the right diagnosis is crucial because there is a small chance of metastasis, death, and local recurrence. Greater knowledge of OFMT in the head and neck reasoning is likely to prevent misdiagnosis and help identify the best treatment source, which looks to be a total surgical excision and is resilient with recurrent surveillance.