Where is undifferentiated pleomorphic sarcoma?

Last Update: April 20, 2022

This is a question our experts keep getting from time to time. Now, we have got the complete detailed explanation and answer for everyone, who is interested!

Asked by: Mr. Franz Mohr Sr.
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Undifferentiated pleomorphic sarcoma (UPS) is a rare type of cancer that begins mostly in the soft tissues of the body. Soft tissues connect, support and surround other body structures. UPS usually occurs in the arms or legs. Less often it can happen in the area behind the abdominal organs (retroperitoneum).

How rare is undifferentiated pleomorphic sarcoma?

Undifferentiated pleomorphic sarcoma (UPS), formerly called malignant fibrous histiocytoma and declassified by the World Health Organization in 2002, is a rare and malignant subtype [1]. These tumors are the fourth most common soft tissue sarcoma and have an incidence of about 0.08–1 per 100,000 [2].

Where are sarcoma tumors located?

They can be found in any part of the body. Most of them start in the arms or legs. They can also be found in the trunk, head and neck area, internal organs, and the area in back of the abdominal (belly) cavity (known as the retroperitoneum). Sarcomas are not common tumors.

How aggressive is pleomorphic sarcoma?

Treating UPS/MFH of the Bone

Undifferentiated pleomorphic sarcoma is usually aggressive with a generally high risk of localized recurrence. Although metastasis of this type of cancer can be rare, the most common distant site of metastasis is the lungs.

How is undifferentiated pleomorphic sarcoma treated?

Treatment for undifferentiated pleomorphic sarcoma usually involves surgery to remove the cancer cells. Other options include radiation therapy and drug treatments (systemic therapies), such as chemotherapy, targeted therapy and immunotherapy.

Undifferentiated Pleomorphic Sarcoma 101 (UPS) (formerly malignant fibrous histiocytoma/MFH)

36 related questions found

What is the survival rate for undifferentiated pleomorphic sarcoma?

A 5-year overall survival rate of 48% has been reported for patients with head and neck tumors versus 77% for patients with tumors arising on the trunk and extremities. The childhood variant appears have better prognosis.

Can pleomorphic sarcoma be cured?

Summary. MFH is a curable disease. The term "Malignant Fibrous Histiocytoma" has been changed by the WHO to Undifferentiated Pleomorphic Sarcoma Not Otherwise Specified. The mainstays of treatment for MFH are complete surgical excision most often supplemented with adjuvant radiation therapy.

What type of sarcoma is pleomorphic?

The undifferentiated pleomorphic sarcoma (UPS), formerly known as malignant fibrous histiocytoma (MFH), is a high-grade soft-tissue sarcoma (STS). The historical literature previously encompassed various STSs as MFH.

What does pleomorphic mean?

Listen to pronunciation. (PLEE-oh-MOR-fik) Occurring in various distinct forms. In terms of cells, having variation in the size and shape of cells or their nuclei.

What causes pleomorphic liposarcoma?

Certain risk factors have been shown to predispose individuals to developing soft tissue sarcomas, such as liposarcoma, including: prior radiation, familial cancer syndromes, damage to the lymph system, and long term exposure to certain toxic chemicals such as vinyl chloride, a chemical used to make plastic.

What is the most aggressive sarcoma?

Epithelioid sarcoma: These tumors are more common in young adults. The classic form of the disease grows slowly and occurs in the feet, arms, legs, or forearms of younger men. Epithelioid tumors can also begin in the groin, and these tumors tend to be more aggressive.

What is the difference between sarcoma and carcinoma?

A carcinoma forms in the skin or tissue cells that line the body's internal organs, such as the kidneys and liver. A sarcoma grows in the body's connective tissue cells, which include fat, blood vessels, nerves, bones, muscles, deep skin tissues and cartilage.

How serious is sarcoma?

A sarcoma is considered stage IV when it has spread to distant parts of the body. Stage IV sarcomas are rarely curable. But some patients may be cured if the main (primary) tumor and all of the areas of cancer spread (metastases) can be removed by surgery. The best success rate is when it has spread only to the lungs.

Is undifferentiated pleomorphic sarcoma hereditary?

They are: Genetic: This doesn't mean that undifferentiated pleomorphic sarcoma is inherited, rather that genetic abnormalities could be causing the cancer. Radiation: By far the most established of the risk factors is radiation therapy when used to address soft tissue sarcomas.

What does undifferentiated sarcoma mean?

A group of rare cancers that do not look like other types of sarcomas under a microscope and may be hard to diagnose. They usually form in the muscles that are attached to bones and that help the body move. Undifferentiated sarcomas tend to grow quickly and spread to other parts of the body.

How do you stage sarcoma?

Stage I: The tumor is small and low grade (GX or G1). Stage II: The tumor is small and higher grade (G2 or G3). Stage III: The tumor is larger and higher grade (G2 or G3). Stage IV: The cancer has spread to other parts of the body.

Are pleomorphic calcifications cancerous?

The morphology of microcalcifications can be pleomorphic (varying shape, size, and density), rounded, punctuate, or amorphous. Pleomorphic calcifications are potentially alarming as they can be cancerous in nature.

What is pleomorphic nature?

Pleomorphism, the existence of irregular and variant forms in the same species or strain of microorganisms, a condition analogous to polymorphism in higher organisms.

What cell is known as pleomorphic?

Pleomorphic is a word pathologists use to describe a group of cells that are very different from each other in either size, shape, or colour. For example, the cells in a tissue sample would be described as pleomorphic if some of the cells in a tissue sample were small while other were very large.

How is undifferentiated pleomorphic sarcoma diagnosed?

Signs and symptoms may include:
  1. Growing lump or area of swelling.
  2. If it grows very large, there may be pain, tingling and numbness.
  3. If it occurs in an arm or leg, there may be swelling in the hand or foot of an affected limb.
  4. If it occurs in the abdomen, there may be pain, loss of appetite and constipation.
  5. Fever.

What do sarcomas look like?

Soft-tissue sarcoma usually looks like a rounded mass beneath the skin surface. The skin is usually unaffected. The mass may be soft or firm. If the mass is deep, the arm or leg may appear larger or fuller than the other side.

What is pleomorphic liposarcoma?

A rare, fast-growing type of cancer that begins in fat cells. It usually forms in the deep soft tissues of the arms or legs, but it may also form in the abdomen or chest. Pleomorphic liposarcoma often recurs (comes back) after treatment and spreads to other parts of the body, including the lungs.

What is the most common sarcoma?

Soft tissue sarcomas are by far the most common. Osteosarcomas (sarcomas of the bone) are the second most common, while sarcomas that develop in the internal organs, such as the ovaries or lungs, are diagnosed least frequently.

How long can you live with liposarcoma?

Well-differentiated liposarcoma has a 100% 5-year survival rate, and most myxoid types have 88% 5-year survival rates. Round-cell and dedifferentiated liposarcomas have a 5-year survival rate of about 50%. Liposarcoma is a rare type of cancer that develops in connective tissues that resemble fat cells.