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Related Concept Videos

Skin Cancer01:30

Skin Cancer

Skin cancer is a type of cancer that occurs when there is an abnormal growth of skin cells, usually triggered by damage to the DNA within the skin cells. It is primarily caused by exposure to ultraviolet (UV) radiation from the sun or artificial sources like tanning beds. Skin cancer is the most common type of cancer worldwide, and its incidence continues to rise.
Basal Cell Carcinoma (BCC): BCC is the most common type of skin cancer, accounting for about 80% of cases. It typically develops in...
Papillary Dermis01:11

Papillary Dermis

Dermis
The dermis might be considered the "core" of the integumentary system, as distinct from the epidermis and hypodermis. It contains blood and lymph vessels, nerves, and other structures, such as hair follicles and sweat glands. The dermis is made of two layers of connective tissue that comprise an interconnected mesh of elastin and collagenous fibers, produced by fibroblasts.
Papillary Layer
The papillary layer is made of loose, areolar connective tissue, which means the collagen and...
Reticular Dermis01:15

Reticular Dermis

The papillary and reticular dermis are the two layers of the dermis. They are made of connective tissue with fibers of collagen extending from one to the other, making the border between the two somewhat indistinct. The dermal papillae extending into the epidermis belong to the papillary layer, whereas the dense collagen fiber bundles below belong to the reticular layer.
Reticular Layer
Underlying the papillary layer is the much thicker reticular layer, composed of dense, irregular connective...

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Is Neoadjuvancy with Imatinib Useful Before Mohs Surgery in Locally Advanced Dermatofibrosarcoma Protuberans? Experience of a Dermato-Oncology Referral Center.

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Updated: May 25, 2026

Identifying, Diagnosing, and Grading Malignant Peripheral Nerve Sheath Tumors in Genetically Engineered Mouse Models
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Identifying, Diagnosing, and Grading Malignant Peripheral Nerve Sheath Tumors in Genetically Engineered Mouse Models

Published on: May 17, 2024

Dermatofibrosarcoma protuberans.

C Serra-Guillén1, B Llombart, O Sanmartín

  • 1Servicio de Dermatología, Instituto Valenciano de Oncología, Valencia, España. cserraguillen@gmail.com

Actas Dermo-Sifiliograficas
|January 31, 2012
PubMed
Summary
This summary is machine-generated.

Dermatofibrosarcoma protuberans, a rare skin sarcoma, is best treated with Mohs surgery to minimize recurrence. Imatinib is an option for advanced cases.

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Area of Science:

  • Oncology
  • Dermatology
  • Surgical Pathology

Background:

  • Dermatofibrosarcoma protuberans (DFSP) is the most common cutaneous sarcoma, characterized by slow growth and dermal origin, with potential for deep invasion.
  • DFSP presents as an indurated plaque with nodular development, arising in the dermis and capable of invading subcutaneous tissue, fascia, muscle, and bone.
  • The COL1A1-PDGFB translocation is a specific molecular marker aiding in DFSP diagnosis.

Purpose of the Study:

  • To review the literature on dermatofibrosarcoma protuberans diagnosis and treatment.
  • To compare surgical outcomes for DFSP, focusing on recurrence rates.
  • To identify alternative treatments for advanced or unresectable DFSP.

Main Methods:

  • Literature review of studies on dermatofibrosarcoma protuberans.
  • Analysis of recurrence rates following different surgical modalities.
  • Evaluation of non-surgical treatment options for metastatic or mutilating DFSP.

Main Results:

  • Mohs micrographic surgery demonstrates significantly lower recurrence rates for DFSP compared to conventional wide local excision.
  • The COL1A1-PDGFB fusion is a diagnostic hallmark for dermatofibrosarcoma protuberans.
  • Imatinib, a tyrosine kinase inhibitor, is an approved treatment for metastatic or surgically challenging DFSP cases.

Conclusions:

  • Mohs micrographic surgery is the preferred treatment for dermatofibrosarcoma protuberans due to its efficacy in reducing recurrence.
  • Accurate diagnosis, supported by molecular markers like COL1A1-PDGFB, is crucial for effective management of DFSP.
  • Imatinib offers a valuable therapeutic option for advanced dermatofibrosarcoma protuberans, particularly when surgery is not feasible or would be disfiguring.