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Dermatofibrosarcoma protuberans (DFSP)

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Overview

Dermatofibrosarcoma protuberans (DFSP) is a rare type of skin cancer that originates in the middle layer of the skin, known as the dermis. Initially, it may appear as a pimple-like growth or a rough patch of skin. As it progresses, lumps of tissue (protuberans) may form near the skin’s surface. DFSP often develops on the arms, legs, and trunk. Fortunately, it grows slowly and rarely spreads beyond the skin.

Types of Dermatofibrosarcoma protuberans (DFSP)

  1. Classic DFSP: The most common type, characterized by slow growth and irregular shape.
  2. Pigmented DFSP (Bednar Tumors): Contains melanin-producing cells, resulting in a mix of colors (red, brown, blue, and purple).
  3. Giant Cell Fibroblastoma (Juvenile DFSP): Mostly found in children or adolescents.
  4. Myxoid DFSP: Composed of abnormal connective tissue called myxoid stroma.
  5. Fibrosarcomatous DFSP (DFSP-FS): Aggressive subtype with a higher likelihood of spreading and recurrence.

Causes of Dermatofibrosarcoma protuberans (DFSP)

The exact cause of DFSP remains unclear. However, it often develops in areas with skin damage from burns, scars, or tattoos. Genetic mutations may also play a role.

Risk Factors of Dermatofibrosarcoma protuberans (DFSP)

  • Skin Damage: Burns, scars, and tattoos increase the risk.
  • Genetic Factors: Some cases involve specific genetic changes.
  • Race: DFSP appears more frequently in Black individuals.

Symptoms of Dermatofibrosarcoma protuberans (DFSP)

1. Initial Signs: Pimple-like Growth: DFSP may start as a small, painless bump resembling a pimple or a rough patch of skin. Rough Texture: The affected area might feel rough to the touch.
2. Progression: Lump Formation: As DFSP advances, a lump (protuberan) may develop. This lump can vary in size and consistency (hard or rubbery). Color Changes: The lump may appear reddish, brownish, or bluish due to pigmentation.
3. Characteristics: Slow Growth: DFSP typically grows slowly over months or years. Lack of Pain: Unlike some other skin conditions, DFSP is usually painless. Stable Appearance: The lump may remain relatively unchanged for a while.

Preventions of Dermatofibrosarcoma protuberans (DFSP)

  • Protect your skin from burns, scars, and excessive sun exposure.
  • Regular skin checks to detect any changes.
  • Seek medical evaluation for suspicious skin growths.

Diagnosis of Dermatofibrosarcoma protuberans (DFSP)

  • Skin biopsy to confirm the diagnosis.
  • Pathologists examine tissue under a microscope.
  • Imaging tests (MRI) to assess the extent of the cancer.

Treatment for Dermatofibrosarcoma protuberans (DFSP)

  1. Surgical Removal: Mohs surgery is commonly used to remove DFSP tumors.
  2. Radiation Therapy: Used if complete removal is not possible.
  3. Targeted Therapy: Imatinib (Gleevec) for specific cases.
  4. Clinical Trials: Investigational treatments may be an option.

When to seek medical attention ?

Seek medical attention if you notice any unusual skin growths, especially if they persist or change over time.

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