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Dermatofibrosarcoma Protuberans (DFSP)

Last updated May 21, 2018

Approved by: Krish Tangella MD, MBA, FCAP

LWozniak & KWZielinski

Microscopic pathology image showing Dermatofibrosarcoma protuberans (DFSP).

What are the other Names for this Condition? (Also known as/Synonyms)

  • Darier-Ferrand Tumor
  • Darier-Hoffmann Tumor
  • DFSP (Dermatofibrosarcoma Protuberans)

What is Dermatofibrosarcoma Protuberans? (Definition/Background Information)

  • Dermatofibrosarcoma Protuberans is a very rare tumor of the subcutaneous tissue of skin. These tumors occur as masses (nodules) on the skin
  • They grow slowly and are present for years, before they are noticed. They are usually painless
  • Dermatofibrosarcoma Protuberans is considered to be a ‘low-grade tumor’, which means that the tumors may recur after excision. Hence, a close medical follow-up of these tumors is essential and advised
  • Wide surgical excision is the standard treatment option, and generally, the prognosis is excellent with suitable treatment

Who gets Dermatofibrosarcoma Protuberans? (Age and Sex Distribution)

  • Dermatofibrosarcoma Protuberans is commonly found in middle-aged adults
  • Both males and females are affected, though the condition is more common in males
  • Individuals of all racial and ethnic background can be affected. Worldwide, no geographical localization of Dermatofibrosarcoma Protuberans has been reported

What are the Risk Factors for Dermatofibrosarcoma Protuberans? (Predisposing Factors)

Currently, no definitive risk factors have been reported for the development of Dermatofibrosarcoma Protuberans. However, the tumor has been linked to the following:

  • Site of trauma
  • Region of extensive burns
  • Surgical incision sites
  • Vaccinations sites (BCG vaccination)
  • Arsenic poisoning

The above mentioned risk factors are considered to be associations. Further studies to understand the risk factors are currently being performed.

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Dermatofibrosarcoma Protuberans? (Etiology)

  • The exact cause of development of Dermatofibroma Protuberans is unknown
  • Occasionally, certain genetic mutations have been reported; but, these are still being researched upon

What are the Signs and Symptoms of Dermatofibrosarcoma Protuberans?

The clinical signs and symptoms of Dermatofibroma Protuberans include:

  • The presence of a nodular, painless mass, underneath the skin that is usually slow-growing
  • The skin over the mass is usually reddish-brown; no ulceration of the overlying skin is noted
  • As the tumors grow and develop, the skin over the tumor may ulcerate, resulting in an infection
  • The mass usually occurs on the head and neck region, chest, abdominal wall, and in the back. Rarely, the tumor is seen to occur on the arms and the legs
  • Occasionally, case studies have reported the presence of the tumor in the salivary gland (parotid gland) and in the genital region

How is Dermatofibrosarcoma Protuberans Diagnosed?

A diagnosis of Dermatofibrosarcoma Protuberans may involve the following:

  • A thorough medical history and physical examination
  • Dermoscopy: Dermoscopy is a diagnostic tool, where a dermatologist examines the skin using a special magnified lens
  • A tissue biopsy of the tumor: A tissue biopsy is performed and sent to a laboratory for a pathological examination. Examination of the biopsy, under a microscope by a pathologist is considered to be gold standard in arriving at a conclusive diagnosis

After putting together the clinical findings, special studies on tissues (if needed), and with microscope findings, the pathologist arrives at a definitive diagnosis. Sometimes, the pathologist may perform special studies, which may include immunohistochemical stains, histochemical stains, molecular testing, and very rarely, electron microscopic studies. A pathological examination is the key to a definitive diagnosis of Dermatofibrosarcoma Protuberans.

  • The pathologist may have to distinguish this tumor from other similar tumors (such as dermatofibroma). Differential diagnosis, to eliminate other tumor types are often considered, before arriving at a definitive diagnosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Dermatofibrosarcoma Protuberans?

The following complications may be observed with Dermatofibrosarcoma Protuberans:

  • The tumor may spread to local tissues, if it is not treated promptly
  • Recurrence of the tumor after surgical excision

How is Dermatofibrosarcoma Protuberans Treated?

  • The preferred treatment of Dermatofibrosarcoma Protuberans is surgical excision
  • A complete wide excision helps in achieving a cure. An incomplete removal of the tumor may cause a tumor recurrence

How can Dermatofibrosarcoma Protuberans be Prevented?

Currently, there are no methods to prevent the onset of Dermatofibrosarcoma Protuberans, which is a very rare low-grade tumor.

What is the Prognosis of Dermatofibrosarcoma Protuberans? (Outcomes/Resolutions)

  • Dermatofibrosarcoma Protuberans is a low-grade sarcoma, which means that a complete excision of the tumor through surgery is necessitated. With complete surgical excision of the tumor, the prognosis is usually excellent
  • An incomplete removal of the tumor will result in its recurrence, which usually occurs, within 5 years of the initial surgery
  • Careful follow-up is necessary through periodic medical examinations

Additional and Relevant Useful Information for Dermatofibrosarcoma Protuberans:

  • A variety of immunostains may be used to arrive at a definitive diagnosis. Dermatofibrosarcoma Protuberans is usually positive for CD34 and vimentin
  • A common tumor called dermatofibroma may resemble Dermatofibrosarcoma Protuberans. Hence, a careful examination of the tumor is necessary. Dermatofibrosarcoma Protuberans is negative for S-100, epithelial membrane antigen, smooth muscle actin, and desmin. This immunostaining pattern will help in distinguishing it from a dermatofibroma

What are some Useful Resources for Additional Information?

References and Information Sources used for the Article:

Helpful Peer-Reviewed Medical Articles:

Reviewed and Approved by a member of the DoveMed Editorial Board
First uploaded: Aug. 25, 2014
Last updated: May 21, 2018