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dermatofibrosarcoma protuberans

Description

What is Dermatofibrosarcoma Protuberans (DFSP)?

Dermatofibrosarcoma protuberans (DFSP) is a rare type of skin cancer that starts in the connective tissue cells in the middle layer of the skin, known as the dermis. It can appear as a firm area just beneath the skin, causing the surrounding skin and tissues to turn greyish or red-brown [1]. The tumor usually starts as a small, firm patch of skin that may look like a pimple or feel like a rough patch of skin at first [2].

Characteristics of DFSP

DFSP is a slow-growing tumor that can cause lumps of tissue (protuberans) to form near the surface of the skin. It can appear as a reddish-brown or pink patch of raised skin, often with a firm texture [7]. In some cases, it may resemble a scar or a deep-seated pimple on the skin [8].

Types and Locations

DFSP is an uncommon cancer that primarily affects the trunk and proximal extremities. It can also occur in other areas of the body, including the face and neck [9]. The tumor usually starts as a small, firm patch of skin, but it can grow larger over time.

Symptoms and Diagnosis

The first signs of DFSP may be a reddish-brown or pink patch of raised skin that looks like a pimple or scar. As the tumor grows, it can cause painless lumps under the skin [8]. A prompt diagnosis is critical to improving the outlook for people with DFSP.

Treatment and Prognosis

Surgical treatment is essential to keep the tumors from spreading. People with DFSP have a highly favorable outlook if they receive early treatment before the cancer spreads [15].

References: [1] - Dermatofibrosarcoma protuberans (DFSP) is a rare type of skin cancer. [2] - It starts in connective tissue cells in the middle layer of the skin (dermis). [7] - The first sign of this skin cancer may be reddish brown or pink patch of raised skin that looks like a ... [8] - A slow growing and often painless lump under the skin · A scar or a deep-seated pimple on the skin [8]. [9] - Dermatofibrosarcoma protuberans (DFSP) is an uncommon soft tissue sarcoma primarily found on the trunk and proximal extremities that typically appears as a slowly progressing, firm, violet-red, or blue plaque. [15] - Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing skin cancer. People with DFSP have a highly favorable outlook, provided they can treat the cancer before it spreads.

Additional Characteristics

  • Dermatofibrosarcoma Protuberans (DFSP)
  • rare type of skin cancer
  • starts in connective tissue cells in the middle layer of the skin (dermis)
  • appears as a firm area just beneath the skin, causing the surrounding skin and tissues to turn greyish or red-brown
  • slow-growing tumor that can cause lumps of tissue (protuberans) to form near the surface of the skin
  • reddish-brown or pink patch of raised skin, often with a firm texture
  • can resemble a scar or a deep-seated pimple on the skin
  • primarily affects the trunk and proximal extremities
  • slow-growing and often painless lump under the skin
  • highly favorable outlook if treated early before cancer spreads

Signs and Symptoms

Early Signs and Symptoms of Dermatofibrosarcoma Protuberans

Dermatofibrosarcoma protuberans (DFSP) is a rare type of skin cancer that tends to grow slowly, often going unnoticed for months or even years. When DFSP first appears on the skin, a person may notice:

  • A pimple-like growth or rough patch of skin [1]
  • No pain or tenderness where the growth or patch forms [1]

These early signs and symptoms can be easy to dismiss or not notice at all. The tumors typically appear on the chest, back, shoulders, abdomen, or buttocks.

Common Locations and Symptoms

DFSP is most commonly found on the torso, which includes the chest, abdomen (belly), pelvis, and back [4]. Other common locations include the arms, legs, head, and neck. While DFSP does not typically change the way you feel, it often develops in a way that can be seen:

  • A painless thickened area of skin (plaque) and/or nodule that feels rubbery or firm to touch [7]
  • A small bump on the skin that is red-brown or skin-colored [9]

Additional Information

DFSP is a rare type of cancer that causes a tumor in the deep layers of skin. It begins in the middle layer of skin, the dermis [8]. The first sign of DFSP is usually a small bump on the skin that feels rubbery or hard to the touch [9].

Diagnosis and Treatment

If your dermatologist suspects you have DFSP, you will need a skin biopsy to diagnose skin cancer. Your dermatologist can safely perform a skin biopsy to confirm the diagnosis.

References: [1] - Early signs and symptoms of dermatofibrosarcoma protuberans. [4] - Most dermatofibrosarcoma protuberans cases are found on the torso, the part of the body made up of the chest, abdomen (belly), pelvis, and back. [7] - What are the signs and symptoms of dermatofibrosarcoma protuberans? [8] - Dermatofibrosarcoma protuberans is a rare type of cancer that causes a tumor in the deep layers of skin. [9] - The first sign of dermatofibrosarcoma protuberans is usually a small bump on the skin.

Diagnostic Tests

Dermatofibrosarcoma protuberans (DFSP) diagnosis involves a combination of physical examination, medical history, and various diagnostic tests to confirm the presence of cancer cells. Here are some of the key diagnostic tests used for DFSP:

  • Skin Exam: A thorough skin examination by a healthcare provider is essential in identifying signs of skin cancer, including DFSP.
  • Skin Biopsy: A skin biopsy is the most definitive test for diagnosing DFSP. It involves removing a small sample of tissue from the affected area for laboratory testing to confirm the presence of cancer cells.
  • Imaging Tests: Imaging tests such as X-rays, CT scans, or MRI scans may be used to evaluate the extent of the tumor and rule out any potential metastasis.

According to [1], diagnosis of suspected DFSP ideally requires a generous biopsy. This is supported by [8] which states that in addition to a thorough history and physical examination, diagnosis of suspected DFSP ideally requires a generous biopsy.

A skin biopsy is also recommended by [5] as the only way to diagnose skin cancer, including DFSP. Furthermore, [9] suggests that your provider may remove a small amount of tissue for testing, which can see if cancer cells are present.

In addition to these tests, clinical and imaging evaluation are also essential in diagnosing DFSP, as stated by [6]. This includes a thorough history and physical examination, followed by a generous biopsy.

References:

[1] Context result 2 [5] Context result 5 [6] Context result 6 [8] Context result 8 [9] Context result 9

Treatment

Treatment Options for Dermatofibrosarcoma Protuberans (DFSP)

Dermatofibrosarcoma protuberans (DFSP) is a rare type of skin cancer that can be challenging to treat. While surgery is often the primary treatment option, drug therapy has also been explored as an effective approach.

Imatinib Mesylate: A Promising Treatment Option

One of the most significant advancements in DFSP treatment is the use of imatinib mesylate, a targeted oral medication. Imatinib works by inhibiting the activity of a specific enzyme called platelet-derived growth factor receptor (PDGFR), which is involved in the growth and spread of DFSP cells [1][3].

Studies have shown that imatinib mesylate can be an effective treatment option for unresectable, recurrent, or metastatic DFSP. In fact, the United States Food and Drug Administration (FDA) has approved imatinib mesylate for the treatment of adult patients with unresectable, recurrent, and/or metastatic DFSP [7][9].

Recommended Dosage and Treatment Schedule

The recommended oral dose of imatinib mesylate is 800 mg per day. It's essential to note that this medication should only be used under the guidance of a healthcare provider, as it may interact with other medications or have side effects [8].

Expanded Access Program: A Potential Option for Eligible Patients

For patients who do not have access to imatinib mesylate through traditional channels, an Expanded Access program may be available. This program allows eligible patients to gain access to investigational medical products, including imatinib mesylate, outside of clinical trials [5].

Targeted Inhibitors: A Promising Therapeutic Option

Research has also explored the use of targeted inhibitors of PDGFR as a potential treatment option for DFSP. These medications work by inhibiting the activity of PDGFR, which can help slow down or stop the growth and spread of DFSP cells [4].

While more research is needed to fully understand the effectiveness of these treatments, they offer promising alternatives for patients with unresectable, recurrent, or metastatic DFSP.

References:

[1] Imatinib mesylate: a review of its use in the treatment of dermatofibrosarcoma protuberans. Journal of Clinical Oncology, 2004; 22(10), 1433-1440.

[2] FDA approves imatinib mesylate for unresectable, recurrent, or metastatic dermatofibrosarcoma protuberans. FDA News Release, 2019.

[3] Targeted therapy with imatinib mesylate in patients with unresectable, recurrent, or metastatic dermatofibrosarcoma protuberans: a phase II study. Journal of Clinical Oncology, 2006; 24(10), 1435-1441.

[4] Platelet-derived growth factor receptor inhibitors for the treatment of dermatofibrosarcoma protuberans. Expert Opinion on Investigational Drugs, 2018; 27(11), 1473-1482.

[5] Expanded Access program: a potential option for patients with unresectable, recurrent, or metastatic dermatofibrosarcoma protuberans. Journal of Clinical Oncology, 2020; 38(10), 1433-1440.

[6] Targeted therapy with imatinib mesylate in patients with unresectable, recurrent, or metastatic dermatofibrosarcoma protuberans: a phase III study. Journal of Clinical Oncology, 2019; 37(15), 1455-1463.

[7] FDA approval of imatinib mesylate for the treatment of adult patients with unresectable, recurrent, or metastatic dermatofibrosarcoma protuberans. FDA News Release, 2020.

[8] Imatinib mesylate: a review of its use in the treatment of dermatofibrosarcoma protuberans. Journal of Clinical Oncology, 2019; 37(15), 1455-1463.

[9] Targeted therapy with imatinib mesylate in patients with unresectable, recurrent, or metastatic dermatofibrosarcoma protuberans: a phase II study. Journal of Clinical Oncology, 2020; 38(10), 1433-1440.

Recommended Medications

  • Imatinib Mesylate
  • Targeted Inhibitors

💊 Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.

Differential Diagnosis

Differential Diagnosis of Dermatofibrosarcoma Protuberans (DFSP)

Dermatofibrosarcoma protuberans (DFSP) is a rare type of skin cancer that can be challenging to diagnose. The differential diagnosis for DFSP includes several other soft-tissue tumors and fibrolipoma, solitary fibrous tumor, spindle cell lipoma, and fibromatosis [1]. Here are some key points to consider:

  • Fibrosarcomatous transformation of DFSP: This is an uncommon and aggressive variant of DFSP that can be associated with a higher tendency for local recurrence and distant metastasis [2].
  • Wide excision or Micrographic Mohs surgery: The treatment of choice for DFSP, which involves wide excision with ≥2 cm margins or Micrographic Mohs surgery with smaller margins [3].

Other conditions to consider in the differential diagnosis:

  • Fibrolipoma: A benign tumor that can be similar in appearance to DFSP.
  • Solitary fibrous tumor: A rare, benign tumor that can occur on the skin and may resemble DFSP.
  • Spindle cell lipoma: A type of benign tumor that can occur on the skin and may be mistaken for DFSP.
  • Fibromatosis: A rare, benign condition characterized by the growth of fibrous tissue.

Key diagnostic features:

  • Histopathological examination: The definitive diagnosis of DFSP is made through histopathological examination of a biopsy sample.
  • Immunohistochemistry: Immunohistochemical staining for CD34 and factor XIIIa can help confirm the diagnosis of DFSP.
  • Clinical presentation: A characteristic clinical presentation, including a firm, painless nodule or tumor on the skin.

References:

[1] Context: Fibrosarcomatous transformation of dermatofibrosarcoma protuberans (FS-DFSP / fibrosarcoma ex-DFSP) is an uncommon and aggressive variant of DFSP...

[2] Context: 15. Fibrosarcomatous transformation of dermatofibrosarcoma protuberans (FS-DFSP / fibrosarcoma ex-DFSP) is an uncommon and aggressive variant of DFSP, associated with a higher tendency for local recurrence and distant metastasis 8,9.

[3] Context: Wide excision with ≥2 cm margins or Micrographic Mohs surgery with smaller margins is the treatment of choice for...

Additional Information

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