What Is Myxoid Liposarcoma?

  • Ellie Kerrod, Neuroscience BSc, The University of Manchester

Overview

Myxoid liposarcoma is a sub-type of soft tissue sarcoma that forms in adipose tissue. Adipose is a tissue in our body that normally stores fat. Currently, the overall survival with myxoid liposarcoma is very low, and it has a high recurrence rate. Besides, the treatment options are limited. Therefore, there is a need for serious cooperation among the oncologists, operators, nurses, counsellors, and other healthcare workers to work together and decide the best strategy. Additionally, the treatment requires close monitoring of the situation to determine the retardation as early as possible. Gladly, there are ongoing research efforts that give hope for better tackling myxoid liposarcoma.

What is myxoid liposarcoma

Sarcomas are known as mesenchymal tissue cancers such as bone, cartilage, muscles, and connective tissues.1 Soft tissue sarcomas cover 80% of sarcomas, whereas the rest %15 and 5% are shared between bone sarcomas and gastrointestinal stromal tumours, respectively. Liposarcomas accounts for the most common soft tissue, sarcomas, and it occurs in the adipose tissue, which is responsible for storing the fat in our body.2,3 Meanwhile, myxoid liposarcoma is the second most common subtype of liposarcoma that forms 20-30% of the liposarcoma cases.3,4 Therefore, myxoid liposarcoma is a type of soft tissue sarcoma within the group of liposarcomas. Myxoid Liposarcoma is identified by its round shape, which is different from other liposarcomas.5 Moreover, we can further subdivide myxoid liposarcoma into higher grade and lower grade types, where the higher grade one indicates a more advanced myxoid liposarcoma.6

What causes myxoid liposarcoma? 

The causes of myxoid liposarcoma remain medically unknown, and more research is required to find answers. However, literature so far suggests that genetic mutations cause the formation of myxoid liposarcoma.4 Those genetic mutations result in abnormally functioning genes that initiate the development of cancer cells and the formation of tumours by altering the normal functioning of the systems. To find more definite answers that will lead to better treatment options, a research gap must be covered.

Who is at risk for myxoid liposarcoma?

Several risk factors can increase your likelihood of having poor prognostic onset of myxoid liposarcoma.3,6

  • Your age at diagnosis
  • Tumor size
  • Tumor grade and depth of tumor
  • Molecular features of your cancer cells (Differentiation, necrosis, mitotic rate, proliferation index (MIB-1, Ki-67 immunostain)). These are the behaviours and markers of cancer cells that either represent a challenging, stable or less advanced situation.
  • Hyperactivity of a gene known as P53 
  • Gender

Higher age is linked with a poorer mechanism of repairing errors in your DNA. Therefore, the unrepaired mutations are believed to result in a worse prognosis in myxoid liposarcoma.6 People over 45 have been shown to have significantly poor prognosis relative to people younger than 45.

Generally, larger tumour size is associated with a worse prognosis in myxoid liposarcoma scientific research. In particular, tumour size over 5 cm results in significantly poorer outcomes compared to tumours less than 5 cm. 

Furthermore, Higher-grade myxoid liposarcoma has more chance of developing metastasis (movement of cancer to other tissues or organs) compared to lower-grade myxoid liposarcoma. Therefore, metastasis makes the disease outcome more complex. 

On the gender basis, Males have shown lower survival outcomes compared to female patients.6 However, the sex-based differences in response to myxoid liposarcoma treatment are still debatable among health sector workers and scientists.

What are the signs and symptoms of myxoid liposarcoma?

As indicated previously, due to a lack of enough research in the field, there is no definite list of signs and symptoms of myxoid liposarcoma. However, some literature data put forward the following signs and symptoms of myxoid liposarcoma, but the symptoms are not limited to this list:7

  • A visible lump under the skin
  • Pain
  • Tiredness
  • Nausea
  • Unintentional weight loss

Self-diagnosis is very important. Accordingly, being aware of the symptoms and regular controls for abnormalities related to our body are also very important and will provide the route to self-diagnosis. 

What are the methods of diagnosis for myxoid liposarcoma?

Diagnostic methods for myxoid liposarcoma can involve many methodologies, including:

  • Barium swallow
  • Computed tomography (CT) scan
  • Magnetic resonance imaging (MRI)
  • Esophagogastroduodenoscopy

These methods can be used individually; however, doctors prefer to use a few of them in combination to increase the specificity of the diagnosis involving all the histological features based on specific cases. However, the most accurate diagnosis is achieved by tissue examination, which is known as biopsy.5

As mentioned above, histologic markers are very important for the prognostic indicators of the disease; it is highly important to make a detailed diagnosis of the type of myxoid liposarcoma tumour.

Is myxoid liposarcoma curable?

Yes, myxoid liposarcoma is curable as long as the disease is low-grade. Overall survival in low-grade myxoid liposarcoma is 78.6%.8 Treatment options for myxoid liposarcoma depend on the individual diagnosis as in every disease. Your doctor or health specialists will determine what will be the best option for your case. However, we can have a look at the common treatment options in general.  

Removing the tumour by surgery is the main point of treatment. It also involves deep cleaning of the surrounding tissues to prevent reoccurrence of the cancer.  Radiotherapy, which involves delivering high-intensity radio waves to kill cancer cells, is applied after the surgery.5

Chemotherapy may also be applied after a minor operation where the small size of the tumour is removed if the tumour is below 10 cm.9 However, both radiotherapy and chemotherapy have side effects, such as being toxic and reducing the quality of life. 

Can myxoid liposarcoma come back after treatment?

Liposarcoma has the chance of coming back after the treatment by 80% (5). Moreover, survival can be as poor as 4 months. 

How can patients cope with the emotional impact of a myxoid liposarcoma treatment?

Mentally being affected is a very common situation in cancer patients and they can experience a variety of psychological issues, for example:

  • Depression
  • Anxiety
  • Post-traumatic stress
  • Post-traumatic growth 

We can divide the methods of coping with this stress into two main categories and evaluate them individually. One of them is problem-focused, and the second one is emotion-focused. 

Problem-focused:

  • Choosing the treatment team (this can help develop trust and be more relaxed in following the treatment route)
  • Getting as much information as possible for all aspects of the situation
  • Getting along with the treatment 
  • Self-motivation and self-help (to continue and to make it easier)
  • Adjusting the lifestyle accordingly to the disease and treatment
  • Organizing the priorities in the life (changing plans accordingly)
  • Using supportive medicine (painkillers, emotion helpers, anxiety reducers)
  • Strong and healthy communication with the treatment team
  • Support seeking from family, friends, and pets

Emotion Focused:

  • Getting plenty of emotional support 
  • If you are a believer, praying and meditation can help you to feel better  
  • Accepting reality can help you to feel more fulfilled in your life 
  • Avoiding thoughts about the future and focusing on present-Moments 
  • Making humour of the situation 
  • Faith (believing in better days will come by the God) 
  • Expressing all the emotions honestly 
  • Artistic hobbies or creating some stuff  
  • You can deliver self-talk with yourself in a positive manner 
  • Carrying out activities for muscle relaxation can help with emotional stress 

Those emotion-focused and problem-based solutions are healthy options and can be applied together to find the best way for yourself. However, there are unhealthy options such as oversleeping, using alcohol, and smoking.10 You should strictly avoid those options because they will reduce your life expectancy, prevent you from getting the full efficiency from your treatment, and lead to horrifying mental health. 

Even terminally ill patients deserve to live the best days at the moment. Therefore, seeking professional help is very important to have a good quality of life during the disease course.

Are there any ongoing research efforts for myxoid liposarcoma?

Promisingly, a recent phase II clinical trial, known as the DOREMY trial, has shown the effectiveness of a new method of radiation therapy in myxoid liposarcoma patients.11,12 This study utilized deintensified radiation therapy, and 91% of the patients showed improvement in their disease.11

Another study also confirmed that deintensified radiotherapy does not affect the recurrence of the myxoid liposarcoma.12 The type of radiotherapy method used by the DOREMY trial is highly influential and has the potential to reduce the toxic effects of radiotherapy, which will improve cancer patients' life conditions.

Additionally, there are ongoing clinical trials based on anthracycline chemotherapy for advanced-stage myxoid liposarcoma patients and trabectedin-based therapies as well.11

Those active clinical trials are highly affirming, taking into consideration that the high-grade advanced-stage myxoid liposarcoma patients still require a more definite solution to improve their life expectancy.

Summary

In summary, myxoid liposarcoma is a curable disease. However, it is highly life-threatening to diagnose it in its early stages. Life expectancy decreases at high-grade tumours, whereas the challenges with the treatment increase. Being aware of the symptoms and regular checks for tumourous formation around the body is also very beneficial for early diagnosis. Apart from these, active communication with multidisciplinary teams during the treatment course is highly important due to the lack of certainties in treatment, especially at the advanced stage of the disease. Meanwhile, there is hope with the ongoing clinical trials, which have already shown improved disease outcomes for the patients. Last but not least, getting emotional support for myxoid liposarcoma patients is highly important. There should be more public awareness about this, and even the hospital's actions should be taken to make emotional care part of the treatment.

References

  1. Damerell V, Pepper MS, Prince S. Molecular mechanisms underpinning sarcomas and implications for current and future therapy. Signal Transduction and Targeted Therapy [Internet]. 2021 Jun 30;6(1):1–19. Available from: https://www.nature.com/articles/s41392-021-00647-8
  2. Kamehama F, Kinjo T, Miyagi Y, Furugen T, Teruya T, Tamaki T, et al. Laparoscopic resection of a metastatic myxoid liposarcoma in the mesentery of the small intestine: a case report. Surgical Case Reports [Internet]. 2023 Jul 21 [cited 2023 Aug 18];9(1):133. Available from: https://rdcu.be/djOmM
  3. Muratori F, Bettini L, Frenos F, Mondanelli N, Greto D, Livi L, et al. Myxoid Liposarcoma: Prognostic Factors and Metastatic Pattern in a Series of 148 Patients Treated at a Single Institution. International Journal of Surgical Oncology. 2018 May 16;2018:1–9.
  4. Chamberlain F, Benson C, Thway K, Huang P, Jones RL, Gennatas S. Pharmacotherapy for liposarcoma: current and emerging synthetic treatments. Future Oncology. 2021 Jul;17(20):2659–70.
  5. Zafar R, Wheeler Y. Liposarcoma [Internet]. PubMed. Treasure Island (FL): StatPearls Publishing; 2020. Available from: https://www.ncbi.nlm.nih.gov/books/NBK538265/
  6. Fiore M, Grosso F, Lo Vullo S, Pennacchioli E, Stacchiotti S, Ferrari A, et al. Myxoid/round cell and pleomorphic liposarcomas. Cancer. 2007 Jun 15;109(12):2522–31.
  7. Myxoid/Round Cell Liposarcoma - NCI [Internet]. www.cancer.gov. 2020. Available from: https://www.cancer.gov/pediatric-adult-rare-tumor/rare-tumors/rare-soft-tissue-tumors/myxoid-round-cell-liposarcoma 
  8. Chowdhry V, Goldberg S, DeLaney TF, Cote GM, Chebib I, Kim J, et al. Myxoid Liposarcoma: Treatment Outcomes from Chemotherapy and Radiation Therapy. Sarcoma. 2018 Nov 1;2018:1–6.
  9. Yehia Tfayli, Baydoun A, Ahmad Salaheddine Naja, Said Saghieh. Management of myxoid liposarcoma of the extremity (Review). Oncology Letters [Internet]. 2021 Jun 9 [cited 2023 Aug 18];22(2). Available from: https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8228380/ 
  10. Adaptation, Coping, and Distress in Sarcoma Patients [Internet]. Liddy Shriver Sarcoma Initiative. [cited 2023 Aug 18]. Available from: http://sarcomahelp.org/coping.html 
  11. Haddox CL, Riedel RF. Recent advances in the understanding and management of liposarcoma. Faculty Reviews. 2021 Jan 4;10.12. Lam SW, Silva TM, Traast-Kooistra J, Bruijn IB, Van Den Akker B, Bakker PAC, et al. Histological response to radiotherapy is an early event in myxoid liposarcoma. Virchows Archiv [Internet]. 2023 Aug 12 [cited 2023 Aug 18]; Available from: https://rdcu.be/djOpN
This content is purely informational and isn’t medical guidance. It shouldn’t replace professional medical counsel. Always consult your physician regarding treatment risks and benefits. See our editorial standards for more details.

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Selun Ilseven

Masters of Cancer Research and Precision Oncology- MSc, University of Glasgow, Scotland.

Selun, with a robust foundation in genetics, cancer research, and precision oncology, she combines her extensive scientific knowledge with years of expertise in science writing, communication, and managing scientific societies.

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