Undifferentiated Pleomorphic Sarcoma or Malignant Fibrous Histiocytoma

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Norton Children’s Cancer Institute

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Undifferentiated pleomorphic sarcoma is a rare cancer that usually forms in soft tissue in the legs, arms or abdomen, but it can form in any soft tissue or bone anywhere in the body.

Also called malignant fibrous histiocytoma or undifferentiated sarcoma, this aggressive cancer can grow fast and often spreads to other parts of the body, including the lungs.

The survival rate for undifferentiated pleomorphic sarcoma is 60% at five years and 48% at 10 years, according to a 2019 study. As with all cancers, early detection improves the survival rate. By their nature, long-term survival rates don’t reflect the latest advancements in treatment and may underestimate current survival chances.

The board-certified and fellowship-trained oncologists at Norton Children’s Cancer Institute, affiliated with the UofL School of Medicine, come together at regular conferences to share viewpoints from various perspectives that help determine the best course of treatment. It’s like getting second, third and fourth opinions all at once.

Our physicians are active researchers, conducting clinical trials and publishing their discoveries to advance medical knowledge.

By staying at the forefront of research, our physicians are experienced with newly approved therapies and can provide access to trials of experimental treatments.

Diagnosis and Treatment of Undifferentiated Pleomorphic Sarcoma

Undifferentiated pleomorphic sarcoma occurs at any age over 2 years and has no known cause or risk factors.

One of the most common symptoms is a growing lump in the leg that can become painful. The cancer also may be found in the arms and abdomen, or anywhere in the body. Fever and weight loss also can be symptoms. Like many childhood and adolescent cancers, undifferentiated pleomorphic sarcoma can have vague symptoms that can be easily overlooked or attributed to other causes. An accurate diagnosis requires oncologists with skill, experience and knowledge of the latest discoveries. Consulting with a pediatric cancer specialist offers the best opportunity for early diagnosis and successful treatment.

Undifferentiated pleomorphic sarcoma typically is diagnosed after more common cancers have been ruled out. In addition to a physical exam, the physician may recommend imaging scans, such as CT scan or MRI, to get a detailed look at the mass, its structure and whether it is connected to other tissues.

A definitive diagnosis is made with a biopsy. A sample of the tumor is removed either with a needle or surgically and sent to a lab for evaluation.

Experience with soft tissue sarcomas is important in getting a correct diagnosis. Biopsies need to be performed in such a way that won’t complicate future surgery to remove the tumor. Undifferentiated pleomorphic sarcoma has the best chance of being cured when it’s treated by pediatric cancer specialists. Young adults may benefit from treatment at a children’s hospital instead of an adult-service hospital because of the care team’s expertise specifically in childhood diseases.

The Norton Children’s Difference

Norton Children’s Hospital’s cancer care program is one of the oldest oncology programs in the U.S. that has been continuously accredited by the American College of Surgeons’ Commission on Cancer. Backed by nearly 60 years of pediatric expertise, we have a proven cancer care team of more than 200 leading cancer specialists, including oncologists, surgeons, nurses, social workers, chaplains, behaviorists, therapists and pharmacists. This skilled, multidisciplinary team is entirely focused on the needs of your child and family.

In addition, we are home to:

  • Kentucky’s leading multidisciplinary pediatric brain tumor program
  • Kentucky’s only pediatric apheresis and pediatric photopheresis programs
  • An immunotherapy program
  • One of the country’s largest sickle cell anemia treatment programs
  • Kentucky’s leading Adolescent and Young Adult Program and Transition Clinic
  • Kentucky’s only CAR-T cell therapy treatment for pediatric patients
  • Life after cancer survivorship program
  • Pediatric bleeding and clotting program (hemostasis and thrombosis)

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