Abstract
Definition: Sarcoma with melanocytic differentiation of tendons and aponeuroses.
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Definition: Sarcoma with melanocytic differentiation of tendons and aponeuroses.
Epidemiology: Very rare, females, average age of 25 years.
Location: Foot, ankle, knee, and upper limb.
Clinical: Slowly growing, moderate size, painless, globose mass.
Diagnosis: On MRI, elliptical, smoothly outlined mass, slightly or markedly hyperintense on T1, white lesion with multiple low-intensity septations on T2, homogeneous enhancement after gadolinium.
Histopathology: Adherent to a tendon or to an aponeurosis, but not to the subcutaneous or to the skin. Firm, globose, well-defined boundaries, capsulated appearance. Round, oval, spindle cells with clear cytoplasm. Nuclei are round, ovoid or elongated, vesicular, with a large nucleolus. Mitotic figures are rare. Cells are arranged in nests surrounded by a fine reticular stroma or rough collagen bands that may give an almost acinar or glandular impression at first glance. Immunohistochemistry shows consistent positivity for S-100, HMB45, MART-1, and MiTF. Molecular findings are characterized by a specific translocation: t(12;22)(q13;q12), with an EWS-ATF1 fusion transcript.
Course and Staging: Local recurrences are frequent when treatment is inadequate due to the not very aggressive clinical presentation. Metastases are late (>6 years) but very frequent in lymph nodes and lungs. Usually, stage II B.
Treatment: Very wide or radical excision with regional lymph nodes.
FormalPara Immunohistochemical PanelS100 | + |
HMB45 | + |
MART-1 | + |
Mitf | + |
t(12;22) (q13;q12) | EWSR1-ATF1 (type 1, type2) | >90 % |
Spindle cell neoplasm with optically clear cytoplasms
Selected Bibliography
Dim DC, Cooley LD, Miranda RN (2007) Clear cell sarcoma of tendons and aponeuroses: a review. Arch Pathol Lab Med 131(1):152–156. Review
Kosemehmetoglu K, Folpe AL (2010) Clear cell sarcoma of tendons and aponeuroses, and osteoclast-rich tumour of the gastrointestinal tract with features resembling clear cell sarcoma of soft parts: a review and update. J Clin Pathol 63(5):416–423. Review
Malchau SS, Hayden J, Hornicek F, Mankin HJ (2007) Clear cell sarcoma of soft tissues. J Surg Oncol 95(6):519–522. Review
Meis-Kindblom JM (2006) Clear cell sarcoma of tendons and aponeuroses: a historical perspective and tribute to the man behind the entity. Adv Anat Pathol 13(6):286–292. Review
Sandberg AA, Bridge JA (2001) Updates on the cytogenetics and molecular genetics of bone and soft tissue tumors: clear cell sarcoma (malignant melanoma of soft parts). Cancer Genet Cytogenet 130(1):1–7. Review
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Gambarotti, M. (2014). Clear Cell Sarcoma. In: Picci, P., Manfrini, M., Fabbri, N., Gambarotti, M., Vanel, D. (eds) Atlas of Musculoskeletal Tumors and Tumorlike Lesions. Springer, Cham. https://doi.org/10.1007/978-3-319-01748-8_79
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DOI: https://doi.org/10.1007/978-3-319-01748-8_79
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