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Clear cell sarcoma: a case mimicking primary cutaneous malignant melanoma.

Rodríguez-Martín M, Sáez-Rodríguez M, Esquivel B, Gonzáalez RS, Cabrera AN, Herrera AM - Indian J Dermatol (2009)

Bottom Line: Characteristic translocation t(12;22) (q13;q12) has been considered pathognomonic for CCS.Prognosis is related to tumor size.An early recognition and initial radical surgery is the key to a favourable outcome.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Hospital Universitario de Canarias, University of La Laguna, 38320 - La Laguna, Tenerife, Spain. marinarm@gmail.com

ABSTRACT
Clear cell sarcoma (CCS) is a recently described variant of sarcoma characterized by prominent clear cells showing features similar to clear cell melanoma. This neoplasm was first described by Dr. Franz M. Erzinger. Primary CCS usually arises in deeper soft tissues, in association with fascia, tendons, or aponeuroses. Characteristic translocation t(12;22) (q13;q12) has been considered pathognomonic for CCS. Prognosis is related to tumor size. An early recognition and initial radical surgery is the key to a favourable outcome. We present a patient with an unusual neoplasm that resembled malignant melanoma.

No MeSH data available.


Related in: MedlinePlus

(a) In a low power picture, oval to polygonal in shape cells with a clear cytoplasm and irregular nuclear contours and complex nucleoli can be observed. Cells were disposed in nests separated by fibrous connective tissue. (b) Intraepidermal involvement can be observed. (H&E, ×40); (c) In dermis, cells were disposed in nests separated by fibrous connective tissue, showing an alveolar pattern (H&E, ×40); (d) Neoplastic cells displayed positivity for HMB 45; and (e) Positivity for S-100 protein.
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Figure 0002: (a) In a low power picture, oval to polygonal in shape cells with a clear cytoplasm and irregular nuclear contours and complex nucleoli can be observed. Cells were disposed in nests separated by fibrous connective tissue. (b) Intraepidermal involvement can be observed. (H&E, ×40); (c) In dermis, cells were disposed in nests separated by fibrous connective tissue, showing an alveolar pattern (H&E, ×40); (d) Neoplastic cells displayed positivity for HMB 45; and (e) Positivity for S-100 protein.

Mentions: Skin biopsy was performed. Histologically, the cutaneous specimen demonstrated epidermal involvement similar to MM. Predominantly, the tumor was composed of oval to polygonal cells with clear cytoplasms and enlarged, irregular, hyperchromatic nuclei with nucleoli. Tumoral cells were arranged in sheets or small nests [Figure 2a]. Junctional activity could be observed in the tumor, with nests of proliferating melanocytic cells showing cytologic atypia in the basal layer. At the lateral margins, atypical melanocytes nested in dermoepidermal junction [Figure 2b], showing features of a remnant nevus in the specimen. In dermis, cells were disposed in nests separated by fibrous connective tissue, showing an alveolar pattern [Figure 2c]. The depth of invasion was 11mm. Melanin granules were not detected. Mononuclear inflammatory infiltrate, necrotic, and hemorragic areas were also found. Superficial ulceration was observed. Neoplastic cells displayed positivity for HMB-45 and S-100 protein. Immunohistochemistry also showed intraepidermal positivity [Figure 2d and e]. Stains for CEA and cytokeratin were negative. Translocation t(12; 22)(q13; q12) was found in cytogenetic analysis by fluorescence in situ hybridization (FISH). FISH technique was performed using the LSI CHOP (12q13) Dual Color, Break Apart Rearrangement Probe (Abbott Laboratories). Our results showed 20–30% of rearrangement in Chop gene over the analyzed tissue [Figure 3a and b].


Clear cell sarcoma: a case mimicking primary cutaneous malignant melanoma.

Rodríguez-Martín M, Sáez-Rodríguez M, Esquivel B, Gonzáalez RS, Cabrera AN, Herrera AM - Indian J Dermatol (2009)

(a) In a low power picture, oval to polygonal in shape cells with a clear cytoplasm and irregular nuclear contours and complex nucleoli can be observed. Cells were disposed in nests separated by fibrous connective tissue. (b) Intraepidermal involvement can be observed. (H&E, ×40); (c) In dermis, cells were disposed in nests separated by fibrous connective tissue, showing an alveolar pattern (H&E, ×40); (d) Neoplastic cells displayed positivity for HMB 45; and (e) Positivity for S-100 protein.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2807157&req=5

Figure 0002: (a) In a low power picture, oval to polygonal in shape cells with a clear cytoplasm and irregular nuclear contours and complex nucleoli can be observed. Cells were disposed in nests separated by fibrous connective tissue. (b) Intraepidermal involvement can be observed. (H&E, ×40); (c) In dermis, cells were disposed in nests separated by fibrous connective tissue, showing an alveolar pattern (H&E, ×40); (d) Neoplastic cells displayed positivity for HMB 45; and (e) Positivity for S-100 protein.
Mentions: Skin biopsy was performed. Histologically, the cutaneous specimen demonstrated epidermal involvement similar to MM. Predominantly, the tumor was composed of oval to polygonal cells with clear cytoplasms and enlarged, irregular, hyperchromatic nuclei with nucleoli. Tumoral cells were arranged in sheets or small nests [Figure 2a]. Junctional activity could be observed in the tumor, with nests of proliferating melanocytic cells showing cytologic atypia in the basal layer. At the lateral margins, atypical melanocytes nested in dermoepidermal junction [Figure 2b], showing features of a remnant nevus in the specimen. In dermis, cells were disposed in nests separated by fibrous connective tissue, showing an alveolar pattern [Figure 2c]. The depth of invasion was 11mm. Melanin granules were not detected. Mononuclear inflammatory infiltrate, necrotic, and hemorragic areas were also found. Superficial ulceration was observed. Neoplastic cells displayed positivity for HMB-45 and S-100 protein. Immunohistochemistry also showed intraepidermal positivity [Figure 2d and e]. Stains for CEA and cytokeratin were negative. Translocation t(12; 22)(q13; q12) was found in cytogenetic analysis by fluorescence in situ hybridization (FISH). FISH technique was performed using the LSI CHOP (12q13) Dual Color, Break Apart Rearrangement Probe (Abbott Laboratories). Our results showed 20–30% of rearrangement in Chop gene over the analyzed tissue [Figure 3a and b].

Bottom Line: Characteristic translocation t(12;22) (q13;q12) has been considered pathognomonic for CCS.Prognosis is related to tumor size.An early recognition and initial radical surgery is the key to a favourable outcome.

View Article: PubMed Central - PubMed

Affiliation: Department of Dermatology, Hospital Universitario de Canarias, University of La Laguna, 38320 - La Laguna, Tenerife, Spain. marinarm@gmail.com

ABSTRACT
Clear cell sarcoma (CCS) is a recently described variant of sarcoma characterized by prominent clear cells showing features similar to clear cell melanoma. This neoplasm was first described by Dr. Franz M. Erzinger. Primary CCS usually arises in deeper soft tissues, in association with fascia, tendons, or aponeuroses. Characteristic translocation t(12;22) (q13;q12) has been considered pathognomonic for CCS. Prognosis is related to tumor size. An early recognition and initial radical surgery is the key to a favourable outcome. We present a patient with an unusual neoplasm that resembled malignant melanoma.

No MeSH data available.


Related in: MedlinePlus