Pleomorphic dermal sarcoma

Pleomorphic dermal sarcoma (PDS) is a rare subtype of a soft tissue cancer (sarcoma) cancer that occurs in the skin. Although these tumors can develop anywhere in the body, they are typically found in sun-exposed areas of the arms, legs, and scalp.

It is more common in men than women. The risk increases with age and these tumors most often affect older adults. Occasionally, they occur in areas that have been treated with radiation therapy in the past.

What are the symptoms of pleomorphic dermal sarcoma?

PDS tumors typically begin as a painless, unremarkable bump or nodule on the skin or just under the skin. Symptoms include:

  • Rapidly growing lump, usually larger than 2 centimeters
  • Lump may turn red, and begin to bleed
  • May crust over

PDS tumors can be difficult to distinguish from another rare skin cancer called atypical fibroxanthoma (AFX). However, PDS is considered an aggressive cancer and likely to metastasize and recur. Early detection and aggressive treatment improve outcomes. Roswell Park’s team of dermatopathologists – pathologists who specialize in diagnosing diseases of the skin – have unique experience with identifying rare cancer subtypes.

How are PDS tumors diagnosed?

A physician will perform a visual examination and ask about symptoms. They may also order tests, such as:

  • Dermoscopy, in which the physician uses a handheld visual aid to examine lesions on the skin more closely.
  • Shave or punch biopsy to take a sample of the tissue for laboratory analysis.
  • Imaging, such as CT scan to check whether the cancer has spread.

How is PDS treated?

PDS tumors are usually removed surgically first and ensuring clear (no cancer) in the margins is very important. This may be followed by immunotherapy or chemotherapy for metastatic disease. In some cases, radiation therapy or chemotherapy may come first, to shrink the tumor before surgery.

Why Roswell Park?

Accurate diagnosis and treatment require an expert team with key experience in managing these rare and aggressive cancers. In addition, surgical treatment may leave large deficits that necessitate plastic and reconstructive surgery to repair.

At Roswell Park, these teams work together to plan both cancer treatment and reconstruction simultaneously. For example, with PDS of the scalp, our plastic & reconstructive team may place tissue expanders under the skin in the weeks before cancer surgery so that when the tumor is removed, the patient has additional scalp tissue – with hair – to replace what was removed with the tumor.

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