What Is Myxofibrosarcoma?

Myxofibrosarcoma (MFS) is categorized as a type of soft tissue sarcoma cancer that originates in the body's connective tissues. These tissues encompass various structures, such as bones, cartilage, and fat, which provide essential structural support and safeguard organs. Typically, MFS is found to develop in the arms or legs, often presenting as a small lump.

The growth of myxofibrosarcoma can occur in the connective tissues situated just beneath the skin or in the deeper layers surrounding muscles. This cancerous tumour exhibits an aggressive nature, characterized by its rapid spread (metastasis) to other areas of the body. Furthermore, MFS tends to have a higher likelihood of recurrence after treatment compared to certain other types of cancers.

Given its aggressive behaviour, timely diagnosis, treatment, and ongoing monitoring are vital in effectively managing myxofibrosarcoma.¹

Symptoms and causes

The precise origins of most soft tissue sarcomas remain unclear.

The initiation of soft tissue sarcoma occurs when there are modifications in the DNA of connective tissue cells. DNA carries the instructions guiding cellular activities. These changes convert the connective tissue cells into cancerous cells. This alteration instructs the cancer cells to proliferate and generate more cells. Unlike healthy cells that undergo programmed cell death, cancer cells persistently multiply due to the absence of cessation instructions.

Consequently, cancer cells amass and develop into a mass known as a tumour. In particular forms of soft tissue sarcoma, the cancer cells remain confined to a specific location, persistently multiplying and enlarging the tumour. Conversely, certain soft tissue sarcomas witness cancer cells detaching and migrating to other body parts.

The nature of the cell undergoing DNA changes dictates the subtype of soft tissue sarcoma. For instance, angiosarcoma originates from blood vessel lining cells, while liposarcoma emerges from fat cells.

A selection of soft tissue sarcoma types comprises:

• Angiosarcoma
• Dermatofibrosarcoma protuberans
• Epithelioid sarcoma
• Gastrointestinal stromal tumour (GIST)
• Kaposi's sarcoma
• Leiomyosarcoma
• Liposarcoma
• Malignant peripheral nerve sheath tumour
• Myxofibrosarcoma
• Rhabdomyosarcoma
• Solitary fibrous tumour
• Synovial sarcoma
• Undifferentiated pleomorphic sarcoma²

Initially, a soft tissue sarcoma might not display any noticeable symptoms. As the cancer progresses, the following manifestations might emerge:

1. Visible Lump or Swelling: The presence of a palpable lump or swelling becomes apparent.
2. Pain: Pain can arise when the growing mass exerts pressure on nerves or muscles.²

Risk factors

Certain factors are associated with an elevated risk of developing sarcoma, including:

1. Inherited Syndromes: There is a familial predisposition to soft tissue sarcoma. Genetic conditions that heighten the risk encompass hereditary retinoblastoma, Li-Fraumeni syndrome, familial adenomatous polyposis, neurofibromatosis, tuberous sclerosis, and Werner syndrome.
2. Chemical Exposure: Exposure to specific chemicals can increase susceptibility to soft tissue sarcomas. Notable among these chemicals are herbicides, arsenic, and dioxin.
3. Radiation Exposure: Individuals who have undergone radiation therapy for different types of cancers have a higher likelihood of developing soft tissue sarcomas as a consequence of the radiation treatment.²

Diagnosis and tests

The process of diagnosing soft tissue sarcoma involves various tests and procedures, including imaging tests and tissue sampling.

Imaging tests

Imaging procedures generate visual representations of the body's internal structures, aiding in the assessment of the size and location of the soft tissue sarcoma. Common examples of imaging tests are:

• X-rays
• CT scans
• MRI scans
• PET scans

Tissue sampling

Tissue sampling, known as a biopsy, is essential for testing and diagnosis. To ensure that the biopsy doesn't hinder future surgical options, it's advisable to seek care from a medical 

centre experienced in treating this type of cancer. Skilled healthcare teams will determine the most suitable biopsy approach.

Types of biopsy procedures for soft tissue sarcoma comprise:

• Core needle biopsy: This method employs a needle to extract tissue samples from cancer, usually from multiple areas of the tumour.
• Surgical biopsy: In some cases, surgery might be recommended to acquire a larger tissue sample.

Biopsy samples are sent to a laboratory for analysis. Pathologists, specialists in examining blood and tissue samples, will examine the cells to ascertain their cancerous nature. Additional lab tests provide further insights into the cancer cells, including their specific cell type.²

Management and treatment

Treatment for myxofibrosarcoma involves several considerations, including tumour grade, stage, size, and depth.

Surgery and additional treatments

In many cases, surgical removal of the tumour along with a margin of surrounding tissue is the primary treatment. Depending on the tumour's characteristics, additional treatments might be recommended:

• Radiation Therapy: This treatment may be administered before or after surgery. It aims to shrink the tumour or reduce the risk of recurrence. It's commonly used for higher-grade myxofibrosarcomas or cases where the tumour is larger or deeper.
• Chemotherapy: While chemotherapy is less frequently used for myxofibrosarcoma compared to other cancers, it might still be considered in certain situations.
• Factors Influencing Treatment

Several factors influence the treatment approach:

• Grade: Low-grade myxofibrosarcoma may often be treated with surgery alone, while high-grade cases may involve a combination of surgery and other treatments like radiation.
• Stage: Staging helps determine the extent of cancer spread. Higher-stage myxofibrosarcoma may necessitate a more comprehensive treatment plan.
• Size: Smaller tumours might only require surgical removal, whereas larger tumours could necessitate additional therapies like radiation.
• Depth: Tumors extending into deeper tissues may require a combination of surgery and radiation to ensure comprehensive treatment.

The treatment plan will be tailored to your situation, aiming to effectively address the cancer while minimising potential side effects. Consulting with a healthcare provider experienced in treating myxofibrosarcoma is crucial for making informed decisions about your treatment journey.¹

FAQs

What is high-grade myxofibrosarcoma?

Grading is an essential aspect of understanding the nature and behaviour of tumours. It helps healthcare providers assess the aggressiveness of the tumour and plan appropriate treatment strategies.

Grading scale and implications:

• Low-Grade Tumors: These tumours are composed of well-differentiated cells that closely resemble normal cells. They tend to grow slowly and are less likely to spread aggressively. Treatment may involve surgical removal, with less emphasis on aggressive therapies like chemotherapy.
• High-Grade Tumors: High-grade tumours consist of less-differentiated cells that often show rapid growth and potential for aggressive spread to nearby tissues or even distant parts of the body. Treatment for high-grade tumours typically involves a more comprehensive approach, which may include surgery, radiation therapy, and chemotherapy.

The grading system helps guide healthcare providers in making informed decisions about the best treatment options for each case. Patients need to have a clear understanding of their tumour's grade and how it influences their treatment plan. This information empowers patients to collaborate with their healthcare team in making the best decisions for their health.¹

Who might get myxofibrosarcoma?

Myxofibrosarcoma is prevalent among individuals aged over 50 and is slightly more frequent in those designated male at birth (DMAB) compared to those designated female at birth (DFAB).

Additional factors contributing to an augmented risk of malignant soft tissue tumours, including myxofibrosarcoma, encompass:

1. Genetics: Specific genetic conditions can heighten the susceptibility to developing soft tissue sarcomas.
2. Environmental factors: Exposure to certain chemicals, such as arsenic and herbicides, is associated with an increased risk.
3. Radiation therapy: A history of undergoing radiation therapy can amplify the likelihood of developing particular cancers, including myxofibrosarcoma.¹

How common is myxofibrosarcoma?

Myxofibrosarcoma is considered a rare condition despite being among the more prevalent forms of soft tissue sarcomas. However, it's important to note that soft tissue sarcomas constitute merely around 2% of the entire spectrum of cancer diagnoses.¹

How can I reduce my risk of myxofibrosarcoma?

Preventing myxofibrosarcoma entirely is not guaranteed. However, adopting a healthy lifestyle can potentially reduce the risk, as is the case with various forms of cancer. It's advisable to minimize exposure to risk factors like radiation and chemicals whenever feasible.¹

What is the prognosis for myxofibrosarcoma?

Myxofibrosarcoma displays a higher tendency to recur post-treatment compared to other variants of soft tissue sarcomas. It's estimated that up to 50% of individuals with myxofibrosarcoma experience a recurrence within five years of treatment.

In cases of low-grade myxofibrosarcoma, the likelihood of recurrence is lower than that of higher-grade tumours. Following myxofibrosarcoma treatment, routine imaging assessments will be conducted by your healthcare provider. Consistent follow-up appointments with your healthcare provider enhance the prospects of detecting and managing myxofibrosarcoma at an early stage in the event of a recurrence.¹

Summary

Myxofibrosarcoma falls under the category of soft tissue sarcomas. Typically, it originates as a small, painless lump. As this lump expands, it might lead to discomfort or swelling. Surgical removal is a common approach employed by healthcare providers to eliminate the growth. However, myxofibrosarcoma has a higher likelihood of recurrence after treatment compared to other soft tissue sarcoma types. Regular consultations with your healthcare provider heighten the possibility of timely detection and intervention if myxofibrosarcoma reoccurs.¹