Abstract
Clear cell sarcoma of the kidney (CCSK) is a rare renal neoplasm of paediatrics, making up about 3% of all renal tumours in paediatrics, with a high tendency for developing bone metastasis. A seven year-old boy was referred to our clinic with two firm, large masses over the manubrium of the sternum and right frontal area, which pathologically were confirmed as a metastatic CCSK. The patient had a history of a renal mass three years earlier, for which radical nephrectomy had been performed, and histopathologic diagnosis was compatible with mesoblastic nephroma. Thus, no further investigation and therapy had been applied for the patient. CCSK is a rare but malignant and aggressive paediatric renal tumour, with a high tendency for developing distant bone metastases, leading to its poor prognosis. CCSK could be misdiagnosed as several other renal tumours such as mesoblastic nephroma, and thus CCSK should be taken carefully into consideration in the diagnosis of renal tumours.
Highlights
Clear cell sarcoma of the kidney (CCSK) is a rare renal neoplasm of paediatrics, comprising about 3% of all paediatric renal tumours [1, 2]
CCSK was initially recognised because of its tendency to metastasise to the bone, leading to its poor prognosis, despite currently available chemotherapy regimens and radical surgery [1, 4]
We report a rare case of CCSK presenting at an older age with two metastatic masses in the sternum and frontal bones; the patient had previously undergone nephrectomy following a diagnosis of mesoblastic nephroma
Summary
Clear cell sarcoma of the kidney (CCSK) is a rare renal neoplasm of paediatrics, comprising about 3% of all paediatric renal tumours [1, 2]. The skeleton and skull are the most common sites of bone metastases and are associated with poor outcome and high mortality [6] As these tumours are radiologically indistinguishable from Wilms’ tumour [7] and the prognosis is very poor [1, 4, 6], the clinical entity should be kept in mind, so that the diagnosis and treatment are not postponed. We report a rare case of CCSK presenting at an older age with two metastatic masses in the sternum and frontal bones; the patient had previously undergone nephrectomy following a diagnosis of mesoblastic nephroma. Chemotherapy with Ifosfamide, Carboplatin, and Etoposide (ICE protocol) was started, and due to the lack of favourable response after three courses, doxotubicin and cyclophosphamide were added Six months later, he had additional active bone lesions in the left humerus and ischium. He was scheduled to receive radiotherapy over his involved bones and considered as a candidate for autologous stem cell transplantation
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