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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

Histology. Bland appearing spindle cells arranged in monotonous storiform pattern, infiltrating between lobules of fat in an honeycomb pattern in DFSP (H&E; panel a). CD34 (panel b) and Apolipoprotein-D (panel c) expression in DFSP. Malignat fibrous histicitoma-like areas in DFSP (DFSP-FS) (panel d). Loss of expression of CD 34 (panel e) and Apolopoprotein-D (panel f).
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Figure 1: Histology. Bland appearing spindle cells arranged in monotonous storiform pattern, infiltrating between lobules of fat in an honeycomb pattern in DFSP (H&E; panel a). CD34 (panel b) and Apolipoprotein-D (panel c) expression in DFSP. Malignat fibrous histicitoma-like areas in DFSP (DFSP-FS) (panel d). Loss of expression of CD 34 (panel e) and Apolopoprotein-D (panel f).

Mentions: A total of 51 consecutive patients with histologic diagnosis of DFSP or FS-DFSP made between 1982 and 2009 were identified. Eleven patient were consultation cases, therefore 40 patients were included in the study (Table 1). Reason for admission at our institution was: a new diagnosis in 4 patients (10%), local recurrence in 19 (47.5%), scar re-excision 14 (35%) and distant (lymph node) metastases plus local recurrence in 3 patients (7.5%). In 13 (32.5%) patients, sarcomatous changes were documented. In 26 of the 40 evaluable patients tissue samples were available for immunohistochemical assessment of the expression of CD34+ and Apo-D (Figure 1, Table 2).


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)

Histology. Bland appearing spindle cells arranged in monotonous storiform pattern, infiltrating between lobules of fat in an honeycomb pattern in DFSP (H&E; panel a). CD34 (panel b) and Apolipoprotein-D (panel c) expression in DFSP. Malignat fibrous histicitoma-like areas in DFSP (DFSP-FS) (panel d). Loss of expression of CD 34 (panel e) and Apolopoprotein-D (panel f).
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 1: Histology. Bland appearing spindle cells arranged in monotonous storiform pattern, infiltrating between lobules of fat in an honeycomb pattern in DFSP (H&E; panel a). CD34 (panel b) and Apolipoprotein-D (panel c) expression in DFSP. Malignat fibrous histicitoma-like areas in DFSP (DFSP-FS) (panel d). Loss of expression of CD 34 (panel e) and Apolopoprotein-D (panel f).
Mentions: A total of 51 consecutive patients with histologic diagnosis of DFSP or FS-DFSP made between 1982 and 2009 were identified. Eleven patient were consultation cases, therefore 40 patients were included in the study (Table 1). Reason for admission at our institution was: a new diagnosis in 4 patients (10%), local recurrence in 19 (47.5%), scar re-excision 14 (35%) and distant (lymph node) metastases plus local recurrence in 3 patients (7.5%). In 13 (32.5%) patients, sarcomatous changes were documented. In 26 of the 40 evaluable patients tissue samples were available for immunohistochemical assessment of the expression of CD34+ and Apo-D (Figure 1, Table 2).

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