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Pleomorphic Liposarcoma

Article
Diseases & Conditions
Cancer & Benign Tumors
Contributed byMaulik P. Purohit MD MPHDec 23, 2018

What are the other Names for this Condition? (Also known as/Synonyms)

  • Pleomorphic LS
  • PLS (Pleomorphic Liposarcoma)

What is Pleomorphic Liposarcoma? (Definition/Background Information)

  • Pleomorphic Liposarcoma is a very rare and aggressive type of sarcoma that is typically present on the lower limbs. Unlike other liposarcomas, Pleomorphic Liposarcomas are not slow-growing tumors
  • The cause of development of these tumors is unknown, but some genetic involvement has been observed
  • The treatment course includes complete surgical excision and removal of the tumor. The prognosis of Pleomorphic Liposarcoma is typically excellent with early detection and complete removal of the tumor, when the tumor occurs in the subcutaneous tissue
  • A liposarcoma (LPS or LS) is a rare and malignant tumor of the fat cells. They are generally asymptomatic and can occur anywhere in the body
  • There are 4 main types of liposarcomas and these include:
    • Well-Differentiated Liposarcoma (WDLS): Approximately 45% of all types of liposarcomas are well-differentiated
    • Dedifferentiated Liposarcoma: It is a high-grade morphology tumor
    • Myxoid Liposarcoma: This type constitute 30% of all liposarcomas
    • Pleomorphic Liposarcoma (PLS): It is the rarest type and constitute about 5% of all liposarcomas

Who gets Pleomorphic Liposarcoma? (Age and Sex Distribution)

  • Pleomorphic Liposarcoma is observed with a slightly higher incidence in men, who are around 50-60 years old; though women may also be affected
  • There is no known ethnic or racial preference; it is observed worldwide

What are the Risk Factors for Pleomorphic Liposarcoma? (Predisposing Factors)

  • No clear risk factors have been identified for Pleomorphic Liposarcoma occurrence

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Pleomorphic Liposarcoma? (Etiology)

  • The exact cause and mechanism of formation of Pleomorphic Liposarcoma is unknown
  • It is thought to occur spontaneously due to certain genetic mutations (chromosomal abnormalities)
  • Contrary to some beliefs, trauma does not contribute to the formation of liposarcomas. Current studies also do not support that liposarcomas arise from lipomas (very common, benign fatty tumors)

What are the Signs and Symptoms of Pleomorphic Liposarcoma?

The signs and symptoms of Pleomorphic Liposarcomas are mostly dependent upon the location of the tumors. The indications may include:

  • The tumors are aggressive and painless
  • Pleomorphic Liposarcoma is frequently found on the lower limbs (legs) and rarely under the skin (in the subcutaneous tissue)
  • If the tumor occurs on the thigh, a painless lump may be observed. Thigh tumors may also cause leg pain and restricted movement (walking difficulties)

How is Pleomorphic Liposarcoma Diagnosed?

Pleomorphic Liposarcoma may be diagnosed by using the following tools and procedures:

  • A complete physical examination and thorough evaluation of the individual’s medical history
  • Radiological studies, such as x-ray, whole body CT scan, MRI, or ultrasound scan of the affected region
  • Needle biopsy of tumor
  • Open biopsy of tumor: A tissue biopsy is performed and sent to a laboratory for a pathological examination, who examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

Note: A needle biopsy may not be helpful, because one may not be able to visualize the different morphological areas of the tumor. Hence, a needle biopsy as a diagnostic tool has certain limitations, and an open surgical biopsy is preferred.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications for Pleomorphic Liposarcoma?

Complications from Pleomorphic Liposarcomas may include the following:

  • Tumors in the leg may result in the formation of varicose veins
  • Metastasis of the tumor to other body regions
  • Damage to the muscles, vital nerves, and blood vessels, during surgery to remove the tumor
  • Pleomorphic Liposarcomas have the tendency to recur after treatment (incomplete surgical removal of the tumor)

How is Pleomorphic Liposarcoma Treated?

Treatment measures for Pleomorphic Liposarcoma include the following:

  • Complete excision and removal of the tumor with clear margins
  • Radiation therapy and chemotherapy are employed depending upon the specific case
  • In some rare cases, limb amputation may have to be undertaken, due to limb tumors
  • Post-operative care is important: One must maintain minimum activity levels, until the surgical wound heals
  • Follow-up care with regular screening and check-ups are important, since the tumor may recur in many cases

How can Pleomorphic Liposarcoma be Prevented?

Current medical research has not established a way of preventing Pleomorphic Liposarcomas.

What is the Prognosis of Pleomorphic Liposarcoma? (Outcomes/Resolutions)

  • The prognosis of Pleomorphic Liposarcoma is excellent with early detection and complete surgical excision and removal, if these tumors are at a subcutaneous location
  • Liposarcomas that are in a location where complete excision is easy to perform, have a better prognosis than the tumors that are difficult to operate upon, such as in the retroperitoneal region

Additional and Relevant Useful Information for Pleomorphic Liposarcoma:

  • In general, liposarcomas form approximately 5% of all sarcomas
  • Among all the different types of liposarcomas, Pleomorphic Liposarcomas are the most aggressive type
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On the Article

Maulik P. Purohit MD MPH picture
Approved by

Maulik P. Purohit MD MPH

Assistant Medical Director, Medical Editorial Board, DoveMed Team

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