Liposarcoma – Causes, Symptoms, Diagnosis, Treatment

Liposarcoma is a malignant tumor of mesenchymal origin a significant tissue diversity tumor of lip blasts, rare mesenchymal neoplasm and involves deep soft tissues including retroperitoneum and popliteal fossa [rx]. It is cancer that arises in the fat cells in soft tissue, such as that inside the thigh or in the retroperitoneum portion of the body.^[rx] The relative frequency of liposarcoma in various body sites and it is much dependent on the tumor subtypes. For example, dedifferentiated liposarcoma is much the most common in retroperitoneal locations, while myxoid liposarcoma occurs in the lower extremities maximum times[rx]. Liposarcoma is exceedingly rare in the esophagus [rx][rx]. Esophageal liposarcoma typically behaves as a slow-growing tumor and involves the upper part of the throat.

Liposarcoma is a tumor that arises from fat tissue in the human body. This tumor most often occurs in the thigh, legs, behind the knee, or in the abdomen, but it can be found in other parts of the body frequently, in the retroperitoneum; and, less often, in the head and neck area. Their primary occurrence in the skin is rarer. Because liposarcoma may grow into surrounding tissues or organs in the body, it is considered a malignant tumor frequently.^[rx]

Although surgical removal of the tumor is the curative treatment, some patients may benefit from chemotherapy and radiation.^[rx]

Types of Liposarcoma

The World Health Organization classification of soft tissue tumors recognizes 5 types of liposarcomas. There are five types of liposarcoma, each with its own unique characteristics and behaviors.

• Well-differentiated liposarcoma – It’s the most common type, and it spread to grow slowly. It has another characteristic that usually doesn’t spread to other parts of your body. Low-grade tumor cells look much like normal fat cells under the microscope.
• Myxoid liposarcoma – It is an intermediate to high-grade tumor. Its cells look less normal under the microscope and may have a high-grade component. It can grow faster than well-differentiated tumors, and it’s more likely to spread to other parts of your body. Its cells can form a unique shape or pattern.
• Pleomorphic liposarcoma – It is the rarest or uncommon type and is a high-grade tumor with cells that look very different from normal cells. It is a less-common form of cancer and often spreads very quickly.
• Dedifferentiated liposarcoma – It gradually occurs when a low-grade tumor changes and the newer cells in the tumor are high-grade. This type of tumor is a slow-growing tumor that starts to change to a faster-growing, more aggressive type.
• Round cell – It often develops in the thigh and can involve changes in the chromosomes (proteins that carry genetic information) in cells. This type can also grow faster than well-differentiated tumors and is often found in the arms or legs.

Stages

The risk of recurrence and metastasis with liposarcoma increases with a higher grade.  The following are the different stages:

• Stage 1A – the tumor is 5 cm or less in size, grade 1, and cancer has not spread to lymph nodes or distant sites
• Stage 1B – the tumor is larger than 5 cm, grade 1, and cancer has not spread to lymph nodes or distant sites
• Stage 2A – the tumor is 5 cm or less, grade 2 or 3, and cancer has not spread to lymph nodes or distant sites
• Stage 2B – the tumor is larger than 5 cm, grade 2, and cancer has not spread to lymph nodes or distant sites
• Stage 3A – the tumor is larger than 5 cm, grade 3, and cancer has not spread to lymph nodes or distant sites OR the tumor is any size and cancer has spread to nearby lymph nodes but not other sites
• Stage 4 – the tumor is any size and any grade, and has spread to lymph nodes and/or to other sites

Causes of Liposarcoma

The specific cause of liposarcoma is still unknown to us. But clinically, it can be first noticed particularly in the extremity in an area of recent trauma, injured soft tissue where the patient may find a large mass, however, the cause and effect are quite likely purely coincidental. Liposarcoma generally contributes to a change in some of the genes that are normally present in fat cells. A series of abnormalities in these genes (mutations or DNA alterations) can lead to malignant cancerous changes characterized by uncontrollable growth.

Some inherited or acquired DNA mutations are found, or defects can make you more prone to developing a soft tissue sarcoma

• Basal cell nevus syndrome increases the risk of basal cell skin cancer, rhabdomyosarcoma, and fibrosarcoma.
• Inherited retinoblastoma in the eyes causes a kind of childhood eye cancer, but it can also increase the risk of other soft tissue sarcomas.
• The Li-Fraumeni syndrome increases the risk of many kinds of cancer, often from radiation exposure.
• Gardner’s syndrome leads to cancers in the stomach or bowel and gastrointestinal area.
• Neurofibromatosis can cause nerve sheath tumors.
• Tuberous sclerosis can cause rhabdomyosarcoma.
• Werner’s syndrome can cause many health problems, including an increased risk of all soft tissue sarcomas.
• Exposure to certain toxins, such as dioxin, vinyl chloride, arsenic, and herbicides that contain phenoxy acetic acid at high doses may increase your risk of developing soft tissue sarcomas.
• Radiation exposure, especially from radiation therapy, can be a risk factor. Radiation therapy often treats more common cancers such as breast cancer, prostate cancer, or lymphomas. However, this effective therapy can increase your risk of developing certain other forms of cancer, such as a soft tissue sarcoma.

Symptoms of Liposarcoma

Most patients with liposarcoma have no symptoms until the tumor is large and invades the neighboring organs or tissues, causing tenderness, pain, or functional problems.

Treatment of Liposarcoma

Treatments for liposarcoma include

• Surgery – The goal of surgery is to remove all of the cancer cells. Whenever possible, surgeons work to remove the entire liposarcoma. If a liposarcoma grows to involve nearby organs, removal of the entire liposarcoma may not be possible. In those situations, your doctor may recommend other treatments to shrink the liposarcoma to make it easier to remove during an operation.
• Radiation therapy – Radiation therapy uses powerful energy beams, such as X-rays and protons, to kill cancer cells. Radiation may be used after surgery to kill any cancer cells that remain. Radiation may also be used before surgery to shrink a tumor in order to make it more likely that surgeons can remove the entire tumor.
• Chemotherapy – Chemotherapy uses drugs to kill cancer cells. Not all types of liposarcoma are sensitive to chemotherapy drugs. Careful analysis of your cancer cells by an expert pathologist can determine whether chemotherapy is likely to help you. Chemotherapy may be used after surgery to kill any cancer cells that remain or before surgery to shrink a tumor. Chemotherapy is sometimes combined with radiation therapy.

Newer drugs for liposarcoma

Halaven® (eribulin) and Yondelis® (trabedectin) are approved for people who have not responded to earlier treatment, have widespread liposarcoma, or have cancers that cannot be removed via surgery.

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