Understanding dedifferentiated liposarcoma: an ultra-rare cancer
Learn more about this ultra-rare form of cancer including its symptoms and treatment options.
What is dedifferentiated liposarcoma (DDLPS)?
Dedifferentiated liposarcoma (DDLPS) is an aggressive subtype of a category of cancers called soft tissue sarcoma (STS).
DDLPS is an aggressive form of liposarcoma with a poor prognosis:
There is not one defined cause that might lead to someone developing DDLPS, but it is thought that contributing factors might involve age, genes and exposure to chemicals or radiotherapy.3
Symptoms and diagnosis of DDLPS
Soft tissue sarcomas (STS) often have no obvious symptoms in the early stages, but the following symptoms can emerge as the condition progresses:3-5
- A new visible lump that develops under the skin (not usually painful at first)
- Painful swelling or numbness in the area around the lump
- Constipation
- Abdominal pain, cramping, and/or swelling
- Blood in the stool and/or vomit
- Cough or breathlessness
Due to the rarity of STS, patients are often misdiagnosed.6 In many cases, even when sarcoma is suspected, people are referred to the wrong center for treatment.6
Typically, DDLPS presents as painless masses that grow gradually in size, resulting in delayed diagnosis.7,8 Common locations include the abdomen, extremities, head or neck, pelvis and torso.9,10
Higher levels of a protein called mouse double minute 2 (MDM2) are seen in over 90% of patients with DDLPS and is therefore considered a hallmark of the disease.10,11 Specific diagnostic tests can detect markers of MDM2 and are recommended for an accurate and early diagnosis.10,12
Treatment and management of DDLPS
Treatment options for DDLPS are limited, and there have been no new first-line treatment options in nearly 50 years.11,13
Currently, the primary treatment for early-stage DDLPS is surgery.11 In some cases (e.g. if the cancer comes back in the same place), a secondary surgery might take place,14 but benefits are limited, and it is often accompanied by complications.14
Past surgery, there are limited systemic therapies for patients with advanced or metastatic DDLPS. The current standard of care is chemotherapy, but less than 10% of patients respond to it.11,13
Management of DDLPS should be handled by a multidisciplinary group of experienced specialists from the time of diagnosis,15 as treatment at specialized centers can provide better outcomes in patients.2
References
1. Schöffski P. Oncol Res Treat. 2022;45(9):525-543.
2. Gootee JM, Curtin CE, Aurit SJ, Randhawa SE, Kang BY, Silberstein PT. Federal Pract. 2019;36(Suppl 5):S34-S41.
3. NHS. Soft tissue sarcomas. https://www.nhs.uk/conditions/soft-tissue-sarcoma. Accessed April 2024.
4. Johns Hopkins Medicine. Sarcoma. https://www.hopkinsmedicine.org/health/conditions-and-diseases/sarcoma. Accessed April 2024.
5. Johns Hopkins Medicine. Liposarcoma. https://www.hopkinsmedicine.org/health/conditions-and-diseases/sarcoma/liposarcoma. Accessed April 2024.
6. Sarcoma UK. The loneliest cancer. https://sarcoma.org.uk/wp-content/uploads/2022/03/The-loneliest-cancer_Sarcoma-UK-Dec-2019.pdf. Accessed April 2024.
7. Gahvari Z, Parkes A. Curr Treat Options Oncol. 2020;21(2):15. doi:10.1007/s11864-020-0705-7.
8. Chen J, Hang Y, Gao Q, Huang X. Front Surg. 2021;8:672669. doi:10.3389/fsurg.2021.672669.
9. Nguyen K, Gootee J, Aurit S, Albagoush S, Curtin C, Silberstein P. Surg Case Rep. 2021;4(2):7-7. doi:10.31487/j.SCR.2021.02.02.
10. Sciot R. Diagnostics. 2021;11(3):496. doi:10.3390/diagnostics11030496.
11. McGovern Y, Zhou CD, Jones RL. Front Oncol. 2017;7:292. doi:10.3389/fonc.2017.00292.
12. Coindre JM, Pédeutour F, Aurias A. Virchows Arch. 2010;456(2):167-179.
13. Italiano A. Cancer Discov. 2023;13(8):1765-1767.
14. Gronchi A, Miceli R, Allard MA, et al. Ann Surg Oncol. 2015;22(5):1447-1454.
15. Gamboa AC, Gronchi A, Cardona K. CA Cancer J Clin. 2020;70(3):200-229.
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