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Review
. 2018 Sep 6;15(4):963-966.
doi: 10.1016/j.jor.2018.08.039. eCollection 2018 Dec.

Clear cell sarcoma-A review

Rami Mossad Ibrahim  1 , Signe Steenstrup Jensen  2 , Jacob Juel  3
Affiliations
Review

Clear cell sarcoma-A review

Rami Mossad Ibrahim et al. J Orthop. .

Erratum in

Abstract

Clear cell sarcoma (CCS) previously known as malignant melanoma (MM) of the soft tissue, although, similar in morphology to MM, contemporary histopathologic and cytogenetic techniques have made this diagnosis obsolete, as it is now possible to distinguish between CCS and MM. CCS is often diagnosed in young adults with median age of 25 years. Overall mortality is generally poor, and the 5-year survival is between 40 and 60%. Hence, early diagnosis and radical surgery are key in the treatment of this extremely rare malignancy of the soft tissue comprising only about 1% of all sarcomas. This article present an overview of this rare malignancy.

Keywords: Clear cell sarcoma; Malignant melanoma of the soft tissue; Sarcoma; Treatment of clear cell sarcoma.

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Figures

Figure 1
Figure 1
MRI scan:
An 80-year-old woman with subcutaneous soft tissue tumor in the lower extremities with relation to both tendons, skin and vital blood vessels. Primary differential diagnosis was sarcoma. A biopsy was performed. Histopathological examinations raised doubt regarding the diagnosis; was it malignant melanoma or clear cell sarcoma. Subsequently verified as a metastasis of malignant melanoma.
Figure 2
Figure 2
CCS, hematoxylin-eosin stain:
There are irregular nests of cells separated by fibrous septa. Cells are fusiform and not pleomorphic. They have regular vesicular nuclei and prominent nucleoli with moderate to abundant eosinophilic or clear cytoplasm.
Figure 3
Figure 3
FNCLCC grading.
See this image and copyright information in PMC

References

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