Dermatofibrosarcoma Protuberans (DFSP)

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Dermatofibrosarcoma protuberans (DFSP) is a rare tumor that typically appears in young adults. DFSP grows in the skin and the layer of fat just below the skin.

DFSP is a malignant cancer, but it rarely spreads (metastasizes) to other parts of the body. It can come back after treatment. Recurrence can be followed by spread, typically to the lungs, much later in life — though this is rare. More than 99% of patients survive at least 10 years after diagnosis.

Typically, DFSP appears as a firm, elevated, slow-growing lesion on the trunk, extremities, head or neck. Initially, it may resemble a scar. As it develops nodules, these can grow and become open, painful sores.

DFSP 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 physicians at Norton Children’s Cancer Institute, affiliated with the UofL School of Medicine, actively conduct clinical trials and publish their discoveries about new ways to identify and treat sarcomas and other pediatric cancers.

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

Our board-certified and fellowship-trained oncologists 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.

Diagnosis and Treatment of DFSP

A skin biopsy is the most common diagnostic test. A small sample of the lesion is collected and examined by a pathologist under a microscope. Sometimes an MRI scan may be ordered to determine whether any of the lesion’s root-like tentacles have reached deeper than the skin.

Mohs micrographic surgery is the most common treatment for DFSP. In this procedure, the surgeon will remove one layer of tissue at a time. Each layer is checked for the presence of cancer, and the process is repeated until all affected tissue is removed.

Traditional surgery to remove the tumor is recommended in some cases. Chemotherapy typically is used only in adults with inoperable, recurring or metastatic DFSP.

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