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Related Concept Videos

Skin Cancer01:30

Skin Cancer

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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.
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Dermatofibrosarcoma Protuberans in Children.

Aseel Sleiwah1, Thomas C Wright2, Thomas Chapman2

  • 1Department of Plastic and Reconstructive Surgery, Southmead Hospital, Southmead Road, Bristol, BS10 5NB, UK. aseelnajeeb@yahoo.com.

Current Treatment Options in Oncology
|April 8, 2022
PubMed
Summary
This summary is machine-generated.

Paediatric dermatofibrosarcoma protuberans (DFSP) is a rare, locally aggressive skin cancer often misdiagnosed. Early suspicion and specialized surgical techniques are crucial for effective treatment and optimal outcomes in children.

Keywords:
Cutaneous surgeryDermatofibrosarcomaPaediatric dermatofibrosarcomaSkin tumoursSoft tissue sarcoma

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Area of Science:

  • Pediatric Oncology
  • Dermatology
  • Surgical Oncology

Background:

  • Paediatric dermatofibrosarcoma protuberans (DFSP) is a rare malignant soft tissue tumor with aggressive local behavior and low metastatic potential.
  • Delayed diagnosis is common due to its frequent misdiagnosis as other skin conditions, especially in early stages.
  • DFSP typically presents as an indolent 'rubbery lump' that enlarges and changes over time, sometimes appearing ulcerated or depressed.

Purpose of the Study:

  • To highlight the diagnostic challenges and emphasize the importance of early detection of paediatric DFSP.
  • To review optimal surgical management strategies for DFSP in children, considering cosmetic and functional outcomes.
  • To discuss the role of multidisciplinary care and pathologist collaboration in managing this rare pediatric tumor.

Main Methods:

  • Review of current literature and clinical guidelines for paediatric DFSP management.
  • Emphasis on diagnostic criteria and differential diagnoses for early-stage lesions.
  • Discussion of surgical techniques including Mohs surgery, wide local excision, and margin assessment.

Main Results:

  • DFSP arises from the dermis with finger-like projections, necessitating margin-controlled excision.
  • Slow Mohs technique with en bloc excision is a viable option; standard Mohs may be challenging in children.
  • Excision margins in children require tailored planning based on imaging, tumor site, age, and patient build.

Conclusions:

  • A high index of suspicion and prompt referral to specialized units are critical for early diagnosis.
  • Surgical resection with complete margin assessment is the gold standard; reconstruction should follow oncologic clearance.
  • Adjuvant radiotherapy should be avoided in children due to risks of secondary malignancies and growth disruption; a multidisciplinary approach is essential.