Clinical and pathological features of pediatric dermatofibrosarcoma protuberans
Dermatofibrosarcoma protuberans (DFSP) is an uncommon malignant mesenchymal tumor characterized by local invasion and recurrence. Fewer than 50 cases have been reported in the pediatric population. We reviewed our experience in the treatment of children with DFSP to define clinical and pathological...
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| Veröffentlicht in: | Annals of plastic surgery 1995-12, Vol.35 (6), p.633-637 |
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| container_title | Annals of plastic surgery |
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| creator | KESHEN, T. H CEDERNA, P. S SAVELL, V. H PLATZ, C. E CHANG, P RICCIARDELLI, E. J |
| description | Dermatofibrosarcoma protuberans (DFSP) is an uncommon malignant mesenchymal tumor characterized by local invasion and recurrence. Fewer than 50 cases have been reported in the pediatric population. We reviewed our experience in the treatment of children with DFSP to define clinical and pathological characteristics. Seven pediatric patients were included in the study (mean age, 11.7 yr). Clinically, the tumors were described as firm nodules fixed to the skin but mobile over the deep fascia, with slow, progressive growth. Diagnosis was made by excisional biopsy in 6 patients and punch biopsy in 1 patient. Six of 7 patients had positive margins after the diagnostic procedure. Pathologically, diagnosis was based on histology, with confirmation by CD34 staining. Definitive surgical therapy consisted of wide local excision (1-3 cm margins) in 5 patients and Moh's micrographic resection in 2 patients. There have been no local recurrences or distant metastases, with a mean follow-up of 15.1 months. Pathological and clinical diagnostic criteria for the pediatric population are reviewed, and treatment options are discussed. |
| doi_str_mv | 10.1097/00000637-199512000-00013 |
| format | Article |
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H ; CEDERNA, P. S ; SAVELL, V. H ; PLATZ, C. E ; CHANG, P ; RICCIARDELLI, E. J</creator><creatorcontrib>KESHEN, T. H ; CEDERNA, P. S ; SAVELL, V. H ; PLATZ, C. E ; CHANG, P ; RICCIARDELLI, E. J</creatorcontrib><description>Dermatofibrosarcoma protuberans (DFSP) is an uncommon malignant mesenchymal tumor characterized by local invasion and recurrence. Fewer than 50 cases have been reported in the pediatric population. We reviewed our experience in the treatment of children with DFSP to define clinical and pathological characteristics. Seven pediatric patients were included in the study (mean age, 11.7 yr). Clinically, the tumors were described as firm nodules fixed to the skin but mobile over the deep fascia, with slow, progressive growth. Diagnosis was made by excisional biopsy in 6 patients and punch biopsy in 1 patient. Six of 7 patients had positive margins after the diagnostic procedure. Pathologically, diagnosis was based on histology, with confirmation by CD34 staining. Definitive surgical therapy consisted of wide local excision (1-3 cm margins) in 5 patients and Moh's micrographic resection in 2 patients. There have been no local recurrences or distant metastases, with a mean follow-up of 15.1 months. Pathological and clinical diagnostic criteria for the pediatric population are reviewed, and treatment options are discussed.</description><identifier>ISSN: 0148-7043</identifier><identifier>EISSN: 1536-3708</identifier><identifier>DOI: 10.1097/00000637-199512000-00013</identifier><identifier>PMID: 8748347</identifier><identifier>CODEN: APCSD4</identifier><language>eng</language><publisher>Hagerstown, MD: Lippincott Williams & Wilkins</publisher><subject>Adolescent ; Antigens, CD34 - analysis ; Biological and medical sciences ; Biomarkers, Tumor - analysis ; Biopsy ; Child ; Dermatofibrosarcoma - pathology ; Dermatofibrosarcoma - surgery ; Dermatologic Surgical Procedures ; Dermatology ; Diagnosis, Differential ; Fascia - pathology ; Fasciotomy ; Female ; Humans ; Male ; Medical sciences ; Skin - pathology ; Skin Neoplasms - pathology ; Skin Neoplasms - surgery ; Soft Tissue Neoplasms - pathology ; Soft Tissue Neoplasms - surgery ; Tumors of the skin and soft tissue. 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E</creatorcontrib><creatorcontrib>CHANG, P</creatorcontrib><creatorcontrib>RICCIARDELLI, E. J</creatorcontrib><title>Clinical and pathological features of pediatric dermatofibrosarcoma protuberans</title><title>Annals of plastic surgery</title><addtitle>Ann Plast Surg</addtitle><description>Dermatofibrosarcoma protuberans (DFSP) is an uncommon malignant mesenchymal tumor characterized by local invasion and recurrence. Fewer than 50 cases have been reported in the pediatric population. We reviewed our experience in the treatment of children with DFSP to define clinical and pathological characteristics. Seven pediatric patients were included in the study (mean age, 11.7 yr). Clinically, the tumors were described as firm nodules fixed to the skin but mobile over the deep fascia, with slow, progressive growth. Diagnosis was made by excisional biopsy in 6 patients and punch biopsy in 1 patient. Six of 7 patients had positive margins after the diagnostic procedure. Pathologically, diagnosis was based on histology, with confirmation by CD34 staining. Definitive surgical therapy consisted of wide local excision (1-3 cm margins) in 5 patients and Moh's micrographic resection in 2 patients. There have been no local recurrences or distant metastases, with a mean follow-up of 15.1 months. Pathological and clinical diagnostic criteria for the pediatric population are reviewed, and treatment options are discussed.</description><subject>Adolescent</subject><subject>Antigens, CD34 - analysis</subject><subject>Biological and medical sciences</subject><subject>Biomarkers, Tumor - analysis</subject><subject>Biopsy</subject><subject>Child</subject><subject>Dermatofibrosarcoma - pathology</subject><subject>Dermatofibrosarcoma - surgery</subject><subject>Dermatologic Surgical Procedures</subject><subject>Dermatology</subject><subject>Diagnosis, Differential</subject><subject>Fascia - pathology</subject><subject>Fasciotomy</subject><subject>Female</subject><subject>Humans</subject><subject>Male</subject><subject>Medical sciences</subject><subject>Skin - pathology</subject><subject>Skin Neoplasms - pathology</subject><subject>Skin Neoplasms - surgery</subject><subject>Soft Tissue Neoplasms - pathology</subject><subject>Soft Tissue Neoplasms - surgery</subject><subject>Tumors of the skin and soft tissue. 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Premalignant lesions</topic><toplevel>peer_reviewed</toplevel><toplevel>online_resources</toplevel><creatorcontrib>KESHEN, T. H</creatorcontrib><creatorcontrib>CEDERNA, P. S</creatorcontrib><creatorcontrib>SAVELL, V. H</creatorcontrib><creatorcontrib>PLATZ, C. E</creatorcontrib><creatorcontrib>CHANG, P</creatorcontrib><creatorcontrib>RICCIARDELLI, E. J</creatorcontrib><collection>Pascal-Francis</collection><collection>Medline</collection><collection>MEDLINE</collection><collection>MEDLINE (Ovid)</collection><collection>MEDLINE</collection><collection>MEDLINE</collection><collection>PubMed</collection><collection>CrossRef</collection><collection>MEDLINE - Academic</collection><jtitle>Annals of plastic surgery</jtitle></facets><delivery><delcategory>Remote Search Resource</delcategory><fulltext>fulltext</fulltext></delivery><addata><au>KESHEN, T. H</au><au>CEDERNA, P. S</au><au>SAVELL, V. H</au><au>PLATZ, C. E</au><au>CHANG, P</au><au>RICCIARDELLI, E. 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Diagnosis was made by excisional biopsy in 6 patients and punch biopsy in 1 patient. Six of 7 patients had positive margins after the diagnostic procedure. Pathologically, diagnosis was based on histology, with confirmation by CD34 staining. Definitive surgical therapy consisted of wide local excision (1-3 cm margins) in 5 patients and Moh's micrographic resection in 2 patients. There have been no local recurrences or distant metastases, with a mean follow-up of 15.1 months. Pathological and clinical diagnostic criteria for the pediatric population are reviewed, and treatment options are discussed.</abstract><cop>Hagerstown, MD</cop><pub>Lippincott Williams & Wilkins</pub><pmid>8748347</pmid><doi>10.1097/00000637-199512000-00013</doi><tpages>5</tpages></addata></record> |
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| ispartof | Annals of plastic surgery, 1995-12, Vol.35 (6), p.633-637 |
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| subjects | Adolescent Antigens, CD34 - analysis Biological and medical sciences Biomarkers, Tumor - analysis Biopsy Child Dermatofibrosarcoma - pathology Dermatofibrosarcoma - surgery Dermatologic Surgical Procedures Dermatology Diagnosis, Differential Fascia - pathology Fasciotomy Female Humans Male Medical sciences Skin - pathology Skin Neoplasms - pathology Skin Neoplasms - surgery Soft Tissue Neoplasms - pathology Soft Tissue Neoplasms - surgery Tumors of the skin and soft tissue. Premalignant lesions |
| title | Clinical and pathological features of pediatric dermatofibrosarcoma protuberans |
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