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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...
The Retinoblastoma Gene01:20

The Retinoblastoma Gene

Tumor suppressor genes are normal genes that can slow down cell division, repair DNA mistakes, or program the cells for apoptosis in case of irreparable damage. Hence, they play an essential role in preventing the proliferation of damaged cells.
The first-ever tumor suppressor gene called Rb was identified in retinoblastoma - a rare eye tumor in children. In inherited forms of the disease, a child inherits one defective copy of the Rb gene, which predisposes them to retinoblastoma. However,...
Rous Sarcoma Virus (RSV) and Cancer01:03

Rous Sarcoma Virus (RSV) and Cancer

Rous Sarcoma virus or RSV was discovered by F. Peyton Rous in the year 1911 as a filterable transmissible agent that could cause tumors in chickens. He won a Nobel Prize for this discovery in 1966. His experiments clearly demonstrated that some cancers could be caused by infectious agents and led to the discovery of many more cancer-causing viruses in animals as well as humans.
RSV is a retrovirus that contains two copies of a plus-strand  RNA genome. Its genome consists of four main open...

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Anterior High-Resolution Optical Coherence Tomography in the Diagnosis and Therapeutic Monitoring of Ocular Surface Squamous Neoplasia
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Anterior High-Resolution Optical Coherence Tomography in the Diagnosis and Therapeutic Monitoring of Ocular Surface Squamous Neoplasia

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[Dermatofibrosarcoma protuberans in children].

I Zaraa1, M Ben abdallah, M Driss

  • 1Service de dermatologie, hôpital La Rabta, 1007 Jabbari, Tunis, Tunisia. inesrania@myway.com

Archives De Pediatrie : Organe Officiel De La Societe Francaise De Pediatrie
|October 19, 2010
PubMed
Summary
This summary is machine-generated.

Dermatofibrosarcoma protuberans (DFSP) is a rare pediatric skin cancer. Surgical excision with wide margins is the recommended treatment for these children, showing no recurrence in observed cases.

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Area of Science:

  • Oncology
  • Dermatology
  • Pediatric Pathology

Background:

  • Dermatofibrosarcoma protuberans (DFSP) is a rare, slow-growing skin neoplasm.
  • While typically affecting adults, pediatric DFSP is exceptionally infrequent.
  • Understanding pediatric DFSP is crucial due to its unique presentation and management considerations.

Observation:

  • Three pediatric cases of DFSP are presented, involving boys aged 8, 9, and 15 years.
  • Lesions were firm, nodular skin tumors on the trunk, ranging from 1 to 5 cm.
  • No preceding trauma was reported, and diagnosis was confirmed via immunohistology.

Findings:

  • DFSP is an intermediate-grade infiltrative tumor with low metastatic potential but high local recurrence rates (≥50%).
  • Pediatric DFSP may present with a deceptively benign appearance, potentially delaying diagnosis.
  • Unlike typical presentations on extremities possibly linked to injury, observed trunkal lesions mirrored adult presentations.

Implications:

  • Accurate diagnosis relies on characteristic histopathological features, despite uncertain pathogenesis.
  • Surgical excision with wide margins is the standard and most effective treatment for pediatric DFSP.
  • Prompt and adequate surgical intervention significantly influences the prognosis for children with DFSP.