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Fibrosarcomatous changes and expression of CD34+ and apolipoprotein-D in dermatofibrosarcoma protuberans.

Palmerini E, Gambarotti M, Staals EL, Zanella L, Sieberova G, Longhi A, Cesari M, Bonarelli S, Picci P, Ruggieri P, Alberghini M, Ferrari S - Clin Sarcoma Res (2012)

Bottom Line: 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.Site, adequate surgical margins, presence of the fibrosarcomatous component, lack of CD34 expression and apolipoprotein-D influence outcome.

View Article: PubMed Central - HTML - PubMed

Affiliation: Chemotherapy, Musculoskeletal Oncology Department, Istituto Ortopedico Rizzoli, via Pupilli 1, Bologna, 40136, Italy. emanuela.palmerini@ior.it.

ABSTRACT

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.

No MeSH data available.


Related in: MedlinePlus

Overall Survival. Five-year overall survival (OS) in 40 DFSP patients with localized disease and metastatic disease at presentation (left). Five-year overall survival (OS) according to histology (right).
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Figure 2: Overall Survival. Five-year overall survival (OS) in 40 DFSP patients with localized disease and metastatic disease at presentation (left). Five-year overall survival (OS) according to histology (right).

Mentions: Two patients were lost to follow-up. With a median follow-up of 49 months (9-184), the 5-year overall survival (OS) was 90% (95%CI 80-100) (Figure 2). The 5-year event-free survival (EFS) was 69% (95%CI 51-87) in localized patients. Histology (DFSP 75% (95%CI 53-97) vs. FS-DFSP 52%,(95%CI 21-83), p = 0.002), surgical margins (adequate 75% (95%CI 55-95) vs. inadequate 55% (95%CI 16-95), p = 0.02), site (extremity 47% (95%CI 22-73) vs. trunk 100%, p = 0.005), influenced the 5-year EFS, whereas sex, use of RT or number of previous surgical treatments did not (Figure 3). Multivariate analysis (via Cox regression) was performed. Site (extremity RR 36.9, 95%CI 2.1 to 621.7, p = 0.01), sex (female RR 0.08, 95%CI 0.009 to 0.7, p = 0.02) and histology (FS-DFSP RR 0.1, 95%CI 0.01 to 0.8, p = 0.03) independently influenced EFS.


Fibrosarcomatous changes and expression of CD34+ and apolipoprotein-D in dermatofibrosarcoma protuberans.

Palmerini E, Gambarotti M, Staals EL, Zanella L, Sieberova G, Longhi A, Cesari M, Bonarelli S, Picci P, Ruggieri P, Alberghini M, Ferrari S - Clin Sarcoma Res (2012)

Overall Survival. Five-year overall survival (OS) in 40 DFSP patients with localized disease and metastatic disease at presentation (left). Five-year overall survival (OS) according to histology (right).
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC3351741&req=5

Figure 2: Overall Survival. Five-year overall survival (OS) in 40 DFSP patients with localized disease and metastatic disease at presentation (left). Five-year overall survival (OS) according to histology (right).
Mentions: Two patients were lost to follow-up. With a median follow-up of 49 months (9-184), the 5-year overall survival (OS) was 90% (95%CI 80-100) (Figure 2). The 5-year event-free survival (EFS) was 69% (95%CI 51-87) in localized patients. Histology (DFSP 75% (95%CI 53-97) vs. FS-DFSP 52%,(95%CI 21-83), p = 0.002), surgical margins (adequate 75% (95%CI 55-95) vs. inadequate 55% (95%CI 16-95), p = 0.02), site (extremity 47% (95%CI 22-73) vs. trunk 100%, p = 0.005), influenced the 5-year EFS, whereas sex, use of RT or number of previous surgical treatments did not (Figure 3). Multivariate analysis (via Cox regression) was performed. Site (extremity RR 36.9, 95%CI 2.1 to 621.7, p = 0.01), sex (female RR 0.08, 95%CI 0.009 to 0.7, p = 0.02) and histology (FS-DFSP RR 0.1, 95%CI 0.01 to 0.8, p = 0.03) independently influenced EFS.

Bottom Line: 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.Site, adequate surgical margins, presence of the fibrosarcomatous component, lack of CD34 expression and apolipoprotein-D influence outcome.

View Article: PubMed Central - HTML - PubMed

Affiliation: Chemotherapy, Musculoskeletal Oncology Department, Istituto Ortopedico Rizzoli, via Pupilli 1, Bologna, 40136, Italy. emanuela.palmerini@ior.it.

ABSTRACT

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.

No MeSH data available.


Related in: MedlinePlus