Liposarcoma is the most common subtype of soft tissue sarcoma, accounting for at least 20% of all sarcomas in adults. This is a group of rare tumors with more than 150 different histological subtypes. Liposarcomas arise from adipocyte progenitor cells, the precursors of fat cells, and develop in adipose tissue throughout the body, including intractable sites such as the deep layers of subcutaneous tissue and visceral fat within the abdominal cavity.
While all liposarcoma share some features that resemble fat cells, there are several forms of liposarcoma that differ based on their clinical presentation, severity, genetic abnormalities, prognosis, and treatment options.
According to the 2020 World Health Organization classification, liposarcoma can be divided into five major subtypes, including: 1) atypical fatty tumor/benign liposarcoma; 2) dedifferentiated liposarcoma; 3) myxoid liposarcoma ; 4) pleomorphic liposarcoma; 5) myxoid pleomorphic liposarcoma. These subtypes have different clinical features and prognosis, with atypical fatty tumors/benign liposarcoma being considered relatively non-aggressive and benign.
All five forms of liposarcoma can locally infiltrate adjacent tissues and organs and recur after surgical removal, potentially progressing to life-threatening disease.
While surgical resection is often the initial treatment for these tumors, existing chemotherapy and radiation therapy options often have limited effectiveness for these tumors, so more research is needed to evaluate more effective treatment options.
Liposarcomas are generally considered large tumors, usually larger than 10 centimeters in diameter, but can be any size. They mainly occur in middle-aged and elderly people, with only 0.7% of cases occurring in people under the age of 16. These tumors must be distinguished from other soft tissue tumors based on their location and clinical manifestations, including histologically atypical lipomas, atypical fibromas, etc.
The combination of atypical fatty tumors and benign liposarcoma accounts for 40% to 45% of all liposarcoma. Although these tumors rarely metastasize, they are locally aggressive and have the potential to transform into the more aggressive dedifferentiated liposarcoma.
Tumors diagnosed as atypical fatty tumors often occur in the arms or legs, while benign liposarcoma is often found in deep and difficult to remove surgically. This term carries a prognostic connotation.
Atypical fat tumors and benign liposarcoma usually present as slowly enlarging masses that are virtually painless. If it is located on the surface, it is easy to detect; if it is located in the deep layer, it may cause dysfunction of the corresponding organs.
The Secret of GeneticsAtypical fatty tumors/benign liposarcoma cells often contain extra chromosomes that carry genes involved in tumor development, including MDM2 and CDK4. Overexpression of these genes is thought to promote tumor development and progression.
For the treatment of atypical fatty tumors and benign liposarcoma, removal of all tumor tissue is the surgical procedure of choice. But even so, these tumors still have a 30% to 50% recurrence rate. Especially in areas where surgical sampling is difficult, such as the retroperitoneum, their recurrence rate is higher and may even be life-threatening.
Therefore, further research is needed to determine the effectiveness of more biological therapies and the development of new therapies for the treatment of liposarcoma. This also makes us think about how to achieve effective treatment in the future medical development. Treating these rare tumors?According to more randomized controlled trials, tumor recurrence is becoming more and more serious, suggesting the need to find effective adjuvant treatments.