Fibrosarcomatous changes and expression of CD34+ and apolipoprotein-D in dermatofibrosarcoma protuberans
| Autor: | Stefano Ferrari, Stefano Bonarelli, Eric L. Staals, Gabriela Sieberova, Pietro Ruggieri, Licciana Zanella, Marilena Cesari, Alessandra Longhi, Piero Picci, Marco Gambarotti, Marco Alberghini, Emanuela Palmerini |
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| Přispěvatelé: | Palmerini E, Gambarotti M, Staals EL, Zanella L, Sieberova G, Longhi A, Cesari M, Bonarelli S, Picci P, Ruggieri P, Alberghini M, Ferrari S. |
| Rok vydání: | 2012 |
| Předmět: |
medicine.medical_specialty
Chemotherapy business.industry Research dermatofibrosarcoma protuberans medicine.medical_treatment Soft tissue sarcoma CD34 Apolipoprotein-D Histology lcsh:Neoplasms. Tumors. Oncology. Including cancer and carcinogens medicine.disease lcsh:RC254-282 Trunk Gastroenterology Surgery Radiation therapy Oncology Surgical oncology soft tissue sarcoma Internal medicine medicine Dermatofibrosarcoma protuberans business |
| Zdroj: | Clinical Sarcoma Research, Vol 2, Iss 1, p 4 (2012) Clinical Sarcoma Research |
| ISSN: | 2045-3329 |
| DOI: | 10.1186/2045-3329-2-4 |
| Popis: | Background Dermatofibrosarcoma protuberans (DFSP) is a relatively common soft-tissue tumor. A more aggressive appearing fibrosarcoma may arise in DFSP, changing its biological behavior. CD34 and apolipoprotein-D are highly expressed in DFSP, but their prognostic significance is uncertain. Methods DFSP and fibrosarcomatous-DFSP (FS-DFSP) patients referred to our institute between 1982 and 2009 were identified. Fibrosarcomatous changes, expression of CD34 and apolipoprotein-D were evaluated. Results 40 patients, (median age 43 years, 55% males) were identified. Tumor was located in the limbs in 60%, in the trunk in 40%. Thirty-seven patients had localized and 3 had metastatic disease. Thirteen (32%) patients were FS-DFSP. All but one underwent surgery with adequate surgical margins in 72%. 7 FS-DFSP received also radiotherapy (RT). Chemotherapy was administered to 3 patients with FS-DFSP. With a median follow-up of 49 months, the 5-OS was 90%. Local recurrence rate was 23%: 42% FS-DFSP, 15% DFSP. Metastases developed in three FS-DFSP patients. The 5-year EFS was 70% in localized patients. Histology (DFSP 75% vs. FS-DFSP 52%, p = 0.002), surgical margins (adequate 74% vs. inadequate 55%, p = 0.02), site (limb 47% vs. trunk 100%), CD34 expression (CD34 positive: 70% vs. CD34 negative: 33%, p = 0.05), and apolipoprotein-D expression (Apo-D positive: 73% vs. Apo-D negative: 33%, p = 0.02) influenced the 5-year EFS, whereas sex, use of RT or number of previous surgical treatments did not. Conclusions Patients with DFSP have a high survival probability. Site, adequate surgical margins, presence of the fibrosarcomatous component, lack of CD34 expression and apolipoprotein-D influence outcome. |
| Databáze: | OpenAIRE |
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