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Clear cell sarcoma (malignant melanoma) of soft parts: a clinicopathologic study of 52 cases.

Hocar O, Le Cesne A, Berissi S, Terrier P, Bonvalot S, Vanel D, Auperin A, Le Pechoux C, Bui B, Coindre JM, Robert C - Dermatol Res Pract (2012)

Bottom Line: High mitotic rate (>10) was found in 25 cases (48.07%).The EWS/ATF-1 translocation was found in 28 (53.84%) of 31 patients studied, and mutation of BRAF or NRAS was found in only 2 of 22 patients analyzed cases (3.84%).Various CT regimens were used in 37 patients (71.15%) with no significant efficacy.

View Article: PubMed Central - PubMed

Affiliation: Melanoma Committee, Gustave Roussy Institute, 114 Rue Edouard Vaillant, 94805 Villejuif, Cedex, France.

ABSTRACT
Clear cell sarcomas are aggressive, rare soft tissue tumors and their classification among melanoma or sarcoma is still undetermined due to their clinical, pathologic, and molecular properties found in both types of tumors. This is a retrospective study of 52 patients with CCS seen between April 1979 and April 2005 in two institutions. The EWS-ATF-1 fusion transcript was studied in 31 patients and an activating mutation of the BRAF or NRAS gene was researched in 22 patients. 30 men and 22 women, with a mean age of 33 were studied. Forty-three tumors (82.69%) were located in the extremities, specially the foot (19 tumors). Median initial tumor size was 4.8 cm (1 to 15 cm). Necrosis involving more than 50% of the tumor cells was found in 14 cases (26.92%). High mitotic rate (>10) was found in 25 cases (48.07%). The EWS/ATF-1 translocation was found in 28 (53.84%) of 31 patients studied, and mutation of BRAF or NRAS was found in only 2 of 22 patients analyzed cases (3.84%). Among the tumor-associated parameters, only tumor size (>4 cm) emerged as a significant prognostic factor. Forty-nine patients had a localized disease at diagnosis (94.23%) and underwent surgical resection immediately (90%) or after neoadjuvant chemotherapy (CT) (10%). Various CT regimens were used in 37 patients (71.15%) with no significant efficacy. The 5- and 10-year OS rates were 59% and 41%, respectively. Tumor size was the only emerging prognosis factor in our series. Complete surgical resection remains the optimal treatment for this aggressive chemoresistant tumor.

No MeSH data available.


Related in: MedlinePlus

Overall survival of 52 patients with clear cell sarcoma.
© Copyright Policy - open-access
Related In: Results  -  Collection


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fig1: Overall survival of 52 patients with clear cell sarcoma.

Mentions: The overall survival rate was 59% at 5 years and 41% at 10 years (Figure 1). Median time of follow up was 120 months (11–348). On multivariate analysis, only tumor size (P : 0.01) emerged as a significant prognostic factor (Table 2 and Figure 2).


Clear cell sarcoma (malignant melanoma) of soft parts: a clinicopathologic study of 52 cases.

Hocar O, Le Cesne A, Berissi S, Terrier P, Bonvalot S, Vanel D, Auperin A, Le Pechoux C, Bui B, Coindre JM, Robert C - Dermatol Res Pract (2012)

Overall survival of 52 patients with clear cell sarcoma.
© Copyright Policy - open-access
Related In: Results  -  Collection

Show All Figures
getmorefigures.php?uid=PMC3369396&req=5

fig1: Overall survival of 52 patients with clear cell sarcoma.
Mentions: The overall survival rate was 59% at 5 years and 41% at 10 years (Figure 1). Median time of follow up was 120 months (11–348). On multivariate analysis, only tumor size (P : 0.01) emerged as a significant prognostic factor (Table 2 and Figure 2).

Bottom Line: High mitotic rate (>10) was found in 25 cases (48.07%).The EWS/ATF-1 translocation was found in 28 (53.84%) of 31 patients studied, and mutation of BRAF or NRAS was found in only 2 of 22 patients analyzed cases (3.84%).Various CT regimens were used in 37 patients (71.15%) with no significant efficacy.

View Article: PubMed Central - PubMed

Affiliation: Melanoma Committee, Gustave Roussy Institute, 114 Rue Edouard Vaillant, 94805 Villejuif, Cedex, France.

ABSTRACT
Clear cell sarcomas are aggressive, rare soft tissue tumors and their classification among melanoma or sarcoma is still undetermined due to their clinical, pathologic, and molecular properties found in both types of tumors. This is a retrospective study of 52 patients with CCS seen between April 1979 and April 2005 in two institutions. The EWS-ATF-1 fusion transcript was studied in 31 patients and an activating mutation of the BRAF or NRAS gene was researched in 22 patients. 30 men and 22 women, with a mean age of 33 were studied. Forty-three tumors (82.69%) were located in the extremities, specially the foot (19 tumors). Median initial tumor size was 4.8 cm (1 to 15 cm). Necrosis involving more than 50% of the tumor cells was found in 14 cases (26.92%). High mitotic rate (>10) was found in 25 cases (48.07%). The EWS/ATF-1 translocation was found in 28 (53.84%) of 31 patients studied, and mutation of BRAF or NRAS was found in only 2 of 22 patients analyzed cases (3.84%). Among the tumor-associated parameters, only tumor size (>4 cm) emerged as a significant prognostic factor. Forty-nine patients had a localized disease at diagnosis (94.23%) and underwent surgical resection immediately (90%) or after neoadjuvant chemotherapy (CT) (10%). Various CT regimens were used in 37 patients (71.15%) with no significant efficacy. The 5- and 10-year OS rates were 59% and 41%, respectively. Tumor size was the only emerging prognosis factor in our series. Complete surgical resection remains the optimal treatment for this aggressive chemoresistant tumor.

No MeSH data available.


Related in: MedlinePlus