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Dermatofibrosarcoma protuberans association with nuchal-type fibroma
A Hafeez Diwan1, Marcelo G Horenstein
1Department of Pathology, University of Texas M. D. Anderson Cancer Center, Houston, TX, USA.
Journal of Cutaneous Pathology
|December 17, 2003
Summary
This study reports the first association between dermatofibrosarcoma protuberans (DFSP) and nuchal-type fibroma (NTF). Findings suggest a potential continuum between these CD34-positive tumors, which infiltrate fat and recur.
Area of Science:
- Dermatopathology
- Oncology
- Surgical Pathology
Background:
- Dermatofibrosarcoma protuberans (DFSP) is a CD34-positive, low-grade sarcoma often found on the trunk and extremities.
- Nuchal-type fibroma (NTF) is a CD34-positive fibrous tumor that can occur in various locations, sometimes associated with Gardner syndrome.
Observation:
- A 43-year-old male presented with a recurrent DFSP on his back and a concurrent NTF on his neck.
- Histopathology revealed a composite lesion with central DFSP transitioning to a peripheral NTF-like area.
- Previous DFSP excisions also showed similar composite features with surrounding NTF-like areas.
Findings:
- This is the first reported association between DFSP and NTF.
- Both DFSP and NTF components expressed CD34 and CD99, and lacked elastin.
- Morphological evidence suggests a potential continuum between DFSP and NTF.
Implications:
- The findings suggest a possible relationship or shared pathway between DFSP and NTF.
- These CD34-positive lesions share a tendency for adipose tissue infiltration and recurrence.
- Further research may elucidate the underlying biology connecting these distinct but potentially related soft tissue tumors.