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CCS Case Studies
Clear cell sarcoma of the esophagus: A rare location
Publication Date:
December 22, 2019
Abstract
Clear cell sarcoma of the esophagus is very rare. The etiology of this neoplasm remains unknown. Confirmed diagnosis requires histopathology with immunochemistry and molecular study. CCS typically shows highly aggressive behavior with a high rate of local recurrence, metastases, and death from disease.
Author(s)
Yosra Yahyaoui 1, Yosr Zenzri 1, Khalil Behi 1, Nadia Boujelbene 2, Amina Mokrani 1, Imen Abbas 2, Karima Mrad 2, Amel Mezlini 1
A case of clear cell sarcoma-A rare malignancy
Publication Date:
June 1, 2017
Abstract
Introduction: Clear cell sarcoma (CCS) is a rare tumour of the soft tissue often misdiagnosed, as it shares characteristics with malignant melanoma (MM). Previously, CCS has been characterised, as malignant melanoma of the soft tissue, contemporary immunohistochemical techniques, however, have made this designation obsolete. The true incidence remains unknown, but CCS is believed to represent less than one percent of all sarcomas.
Presentation of case: A 22-year-old patient presented with a mass sized 2.6×2.7×2.7cm of the left gluteal region, pain, and malaise. Initially, the symptoms were interpreted as an infection. Subsequent, pathological diagnosis after surgical removal was tentatively MM albeit definitive pathological diagnosis was CCS.
Discussion: The patient of this case underwent definitive surgical treatment with 2cm margin. In spite of time delay, because of prolonged time for definitive diagnosis, PET-CT and sentinel lymph node biopsy did not show any metastasis. One-year postoperatively, multidisciplinary follow-up is without suspicion of relapse.
Conclusion: Accurate and timely diagnosis of CCS are imperative, as initial misdiagnosis, may cause delay and further tumour growth, which is correlated to the prognosis.
Author(s)
Jacob Juel 1, Rami Mossad Ibrahim 2
Clear cell sarcoma of tendons and aponeuroses in pediatric patients
Publication Date:
June 15, 2002
Abstract
Backround:
Clear cell sarcoma (CCS) of tendons and aponeuroses is extremely rare in childhood and little information is available on its clinical management. Originally believed to be a type of melanoma of soft tissue origin, CCS is now considered a distinct clinicopathologic entity that behaves like a high-grade soft tissue sarcoma. We report on a series of 28 pediatric patients treated from 1980 to 2000 by the Soft Tissue Sarcoma Italian Cooperative Group and the German Cooperative Group.
Methods:
Patients were treated with a multimodality therapeutic approach. Surgical resection was complete in 17 patients (mutilating in 3), radiotherapy was administered to 8 patients, and 20 patients received chemotherapy.
Results:
After a median follow-up of 102 months (range, 19–238 months), the 5-year and event-free survival rates were 66.4% and 63.3%, respectively. Seventeen patients were alive in first remission, two were alive in second remission, and nine had died of disease. The response to chemotherapy in the 7 evaluable patients included one partial remission, one minor response, and five no responses. Radiotherapy contributed to achieving local control in four of six Intergroup Rhabdomyosarcoma Study (IRS) Group II patients. Statistically significant differences in outcome were evident according to IRS group, tumor size, and site.
Conclusions:
Our study confirms the aggressive behavior of CCS. Complete surgical resection represents the mainstay of treatment, and even the only treatment for patients with small tumors. Radiotherapy may control microscopic residual disease after surgery. Chemotherapy is ineffective and the prognosis is unfavorable for patients with unresectable and large tumors. Cancer 2002;94:3269–76. © 2002 American Cancer Society.
Author(s)
Andrea Ferrari M.D., Michela Casanova M.D., Gianni Bisogno M.D., Adrian Mattke M.D., Cristina Meazza M.D., Lorenza Gandola M.D., Guido Sotti M.D., Giovanni Cecchetto M.D., Dieter Harms M.D., Eva Koscielniak M.D., Jorn Treuner M.D., Modesto Carli M.D.
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