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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,...
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
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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...
Skin Diseases and Disorders01:23

Skin Diseases and Disorders

Skin is the first line of defense and encounters a variety of microbes. Some pathogenic strains are often the cause of a broad range of infections of the skin and other body systems. These conditions can affect people of all ages and may have different causes, including genetic factors, infections, autoimmune reactions, environmental factors, and lifestyle choices.
Gram-positive Staphylococcus spp. and Streptococcus spp. are responsible for many of the most common skin infections. However, many...

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Updated: May 14, 2026

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

Published on: August 9, 2024

Congenital dermatofibrosarcoma protuberans.

Orlando Oliveira de Morais1, Lisley Calixto de Araújo, Ciro Martins Gomes

  • 1Hospital Universitário de Brasilia, Universidade de Brasilia, Brasil.

Cutis
|February 16, 2013
PubMed
Summary
This summary is machine-generated.

Congenital dermatofibrosarcoma protuberans (DFSP), a rare pediatric cancer, presents at birth and requires prompt diagnosis. Early detection and surgical intervention, including Mohs micrographic surgery, are crucial for managing this locally invasive neoplasm.

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Last Updated: May 14, 2026

Anterior High-Resolution Optical Coherence Tomography in the Diagnosis and Therapeutic Monitoring of Ocular Surface Squamous Neoplasia
06:15

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Combining Reflectance Confocal Microscopy with Optical Coherence Tomography for Noninvasive Diagnosis of Skin Cancers via Image Acquisition
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Published on: August 18, 2022

Area of Science:

  • Dermatology
  • Pediatric Oncology
  • Surgical Pathology

Background:

  • Congenital dermatofibrosarcoma protuberans (DFSP) is an exceptionally rare low-grade malignant neoplasm.
  • It typically exhibits localized invasive growth with a low metastatic potential.
  • Occurrence in newborns and children is exceedingly uncommon.

Observation:

  • A case report details a patient born with a congenital DFSP initially misdiagnosed as localized scleroderma.
  • The lesion evolved from a macule to atrophic plaques and nodules.
  • Diagnosis at age five was confirmed via clinical and immunohistochemical findings (CD34 positivity, spindle cell proliferation).

Findings:

  • The study highlights diagnostic challenges in congenital DFSP due to its rarity and initial misdiagnosis.
  • Immunohistochemistry, particularly CD34 staining, is vital for accurate diagnosis.
  • Despite diagnostic delays, a multi-step surgical approach, including Mohs micrographic surgery (MMS), was recommended.

Implications:

  • This case underscores the importance of considering rare pediatric tumors in differential diagnoses.
  • Timely and accurate diagnosis of congenital DFSP is critical for effective management.
  • Mohs micrographic surgery offers a precise method for complete tumor removal in complex cases.