Introduction

Myxofibrosarcoma is a malignant cancer that affects soft tissue and can have a high local recurrence rate, meaning that it returns in a specific area frequently.¹ This makes it a tricky disease to treat and can be devastating to live with. However, understanding the nature of recurrent myxofibrosarcoma and management options can empower patients and families with knowledge, providing them with guidance through this difficult period. That is what we aim to do in this article.

Understanding Myxofibrosarcoma

What is Myxofibrosarcoma?

Myxofibrosarcoma is a cancer of the connective tissue that commonly originates in the arms and legs, forming a painless lump under the skin that grows slowly.¹ On rare occasions, myxofibrosarcoma can also originate from the trunk, head or neck.

What does recurrence mean?

Recurrence, in this context, is when the cancer returns after a certain remission period. This usually happens because the surgery/treatment previously performed didn’t remove all the cancer cells. This means a few tumour cells remained that then developed into cancer again.

Myxofibrosarcoma is a type of cancer with a high recurrence rate. The most common areas of spread include the lymph nodes and lungs.

Recognising recurrence signs

New lumps near the site of surgery are usually the first sign of recurrence and must be seen by a professional immediately. Apart from this, abdominal issues such as bloating, passing urine more frequently, and constipation are also signs to watch out for.¹ 

Regular monitoring is also extremely important so keeping up to date with scheduled check-ups and having regular imaging tests (e.g. MRI, CT scans, etc.) as suggested by your doctor is crucial. This helps track the progression of the cancer and allows any malignancies to be detected at its early stages, helping you stay two steps ahead of the cancer.

Diagnostic process for recurrence

The initial steps for diagnosing recurred cancer include physical examinations and a thorough review of the patient’s medical history by the healthcare team.¹ Following this, there would be diagnostic tests like imaging studies and biopsies taken as well as some pathological analysis. This should provide the patient and healthcare team with a better understanding of the current situation and the doctor may then guide the patient and their loved ones if there is indeed a recurrence.

Treatment options

There are a number of treatment options available for myxofibrosarcoma and they are dependent on the stage, grade, size and depth of the cancer. It is important to discuss which one is best for you with your healthcare team. 

Surgery

If the cancer is localised and has not spread to any other sites on the body yet, then surgical resection coupled with neoadjuvant radiotherapy and chemotherapy is the primary method of treatment.

Neoadjuvant therapy is when treatment (radiotherapy or chemotherapy) is first given to shrink the tumour so that surgery can then be performed to remove the reduced tumour.² A larger tumour is also more likely to lead to recurrence because there is a higher chance that some tumour cells are left behind following resection surgery.

Chemotherapy

If the cancer has metastasised (spread) to other parts of the body, chemotherapy tends to be the answer. 

Usually, the first line of chemotherapeutic drugs given are anthracycline (also known as doxorubicin) and ifosfamide, either separately or together, and sometimes along with dacarbazine.³ As a second line of treatment, the drugs often prescribed include gemcitabine, either with or without docetaxel. 

It is important to understand the heavy burden of side effects that often accompany chemotherapy, such as deep tiredness, infection, hair loss and nausea. The patient and their loved ones should ensure there are sufficient supportive care systems in place to help them go through this difficult time together in as much comfort as possible.

Radiation therapy

Another treatment option for myxofibrosarcoma is radiation therapy.³ 

Most patients who undergo radiotherapy tend to have tumours that are bigger than 5cm in diameter. The primary function of radiotherapy in this context is likely to be as part of neoadjuvant radiotherapy. It may, however, also be given as adjuvant therapy, which is when radiotherapy is given following surgery, to decrease the chance of recurrence.³ 

Side effects of radiation therapy include intense tiredness, nausea and infection, much like chemotherapy. Radiation therapy does, however, tend to have fewer side effects than chemotherapy because its nature is more localised to one area, instead of systemic, like in chemotherapy.³

Targeted therapy and clinical trials

The final option we’re going to discuss involves targeted therapy and clinical trials.⁴ 

Targeted therapy is a type of treatment where we specifically target proteins that contribute to the growth and spread of the cancer. Currently, the most common targeted therapy used for myxofibrosarcoma involves inhibiting the progression of tumour cells through the cell cycle. A more effective use of targeted therapy is likely to focus on a variety of different pathways and proteins, considering most sarcomas, like myxofibrosarcoma, affect many different signalling pathways.⁴

Clinical trials are another option, though these require careful deliberation and evaluation so that the right clinical trial can be chosen for the patient. 

Several clinical trials are specific to targeted therapy, but also ones that are focused on other emerging treatments. It is important to ensure the clinical trial you find is through a reputable source such as clinicaltrials.gov. This is part of the National Library of Medicine and is therefore a trustworthy place to start looking. 

Once you have chosen a trial, it’s now time to assess it. Pay close attention to the study description as this will detail exactly what is going to happen as part of the study. It is highly recommended that you read through this with your doctor so that a full understanding of the trial is gained before you enter into it. Contacting the study organisers is also a good idea if there is any additional information you would like to know that hasn’t been covered in the study description.⁴

Managing treatment side effects

Managing the side effects of treatments for myxofibrosarcoma can be incredibly challenging and often debilitating. 

The physical side effects can be made more manageable by appropriate pain management and by helping the patient conserve as much energy as possible to limit the fatigue that often accompanies treatments. However, cancer also has a huge emotional and psychological toll on patients and their loved ones. Hope is one of the most powerful tools in your armoury and one that you are more important now than ever. Support networks can be a valuable source of comfort and can guide you through this difficult period. Stay positive; stay strong.

It is crucial to remain strong through these trying times by having coping strategies to work through stress and anxiety. Joining support groups or counselling services can also help people manage their new lives and the challenges that accompany them.

Apart from this, nutritional and lifestyle considerations are also essential, and the healthcare team must be consulted about diet and exercise recommendations. Maintaining your overall health can be incredibly beneficial, not only to preserve your mental well-being but also in terms of fighting the cancer itself.

In short: 

• Have appropriate pain management in place
• Try to conserve your energy where possible
• Have a support system in place with family, friends and loved ones
• Have coping strategies for stress and anxiety
• Join support groups (Sarcoma UK and Sarcoma Alliance)
• Join counselling services 
• Consult your healthcare team about diet and lifestyle
• Eat highly nutrient meals 
• Partake in manageable physical activity

Future outlook and research

There are many advances in treatment for myxofibrosarcoma, with current research into immunotherapies and targeted therapies showing promising results.⁵ Staying up to date on new treatment options available can be extremely important and may lead to a brighter future for patients and their loved ones. Putting in place strategies for ongoing care and monitoring is also an integral part of long-term management as you plan for the future.

Summary

• There are a variety of different treatment and management choices available for myxofibrosarcoma depending on the stage, grade, size and depth of the cancer
• From surgery to clinical trials and targeted therapy, it is important to discuss all possible treatment plans with the healthcare team before committing to one
• Consulting with your doctor as well as doing your own research can help you stay up-to-date and informed about advances in treatments
• Support is incredibly important during such hard times so joining support groups and counselling services specifically for sarcomas like myxofibrosarcoma