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Clear-cell sarcoma of the kidney (CCSK) is a rare malignant tumor of childhood, known for its aggressiveness, its tendency to recurrence and to metastasis to bone. We report an observation of a child of 48 months carrying a large abdominal mass. The diagnosis of the SCCR was made on biopsy, since imaging remained uncertain as to the renal origin of the mass. Indeed, our observation underlines the difficulty of its diagnosis. Excepting the morphological aspect, there is no criterion for its recognition. Its prognosis has been improved by the new treatments., (Copyright 2009 Elsevier Masson SAS. All rights reserved.)

Unlabelled: Clear cell sarcoma of the kidney (CCSK) also called a "bone-metastasizing renal tumor of childhood" is the second common pediatric renal neoplasm. This tumor is associated with a higher rate of relapse and a wider distribution of metastases than Wilms' tumor., Patients and Methods: We have reviewed records of 13 cases of CCSK among 277 renal tumors (5%) diagnosed at the children's hospital of Rabat between 1990 and 2002., Results: The median age at diagnosis was 14 months (5 months-9 years). The male to female ratio was 5.5:1.00. Abdominal mass, usually the first physical finding, was associated with hematuria in four cases. No congenital malformation syndrome or familial Wilms' tumor were observed. Imaging studies found out seven right and six left intrarenal processes. Preoperative chemotherapy was given according to the SIOP9, SIOP93-01 and GFAOP 98 protocols. Twelve of 13 children underwent nephrectomy. Tumor measurements varied through 450-3450 g and 7-26 cm. The classic morphologic pattern was seen in nine cases (69%). The distribution local stage was I: three cases; II: three cases; III: six cases; IV: one case. Postoperative chemotherapy and radiotherapy (21 600-30 600 cGy) was done in 10 cases. With a median follow up of 44 months, four patients showed bone metastases (31%), four are alive in CR, four are lost for follow up and five died., Conclusion: CCSK remains the pediatric renal tumor most frequently misdiagnosed. Its aggressiveness and its ability to give bone metastases need to recognize early this diagnosis for an adapted treatment.

Clear cell sarcoma (malignant melanoma of soft parts) is a rare malignancy that is found in the young adult, and is generally located in the extremities of the limbs. In this study, a new case has been reported in a 24-year old male with no previous history of cutaneous melanoma. The tumor consisted of fusiform or round cells with clear or granulocytic cytoplasm and vesicular nuclei. The patient was treated by surgical resection of the tumor and postoperative radiotherapy. Eight years later, metastatic nodes were detected in the inguinal region. The histogenesis of this tumor has not yet been determined, and it poses a diagnostic problem for pathologists as it can be mistaken for a malignant metastatic cutaneous melanoma.

A case of malignant melanoma of the quadriceps tendon is reported. This is an uncommon soft tissue sarcoma of melanocytic origin. The appearance on MRI depends on its melanin content. The microscopic appearance is distinctive and prognosis is poor. This tumor should be kept in mind when a nodular lesion is detected in specific tendon or aponeurosis.

Purpose of the Study: Clear cell chondrosarcoma is a variant of bone chondrosarcoma. These unusual tumors are peculiar by their epiphyseal location in long bones, by their cytology and by their cytology and by their protracted course. Clinical, radiographical and histological features are described before therapeutic approach., Material and Methods: We report a series of thirteen cases of clear cell chondrosarcoma identified and treated between 1965 and 1995 in Cochin Hospital., Results: Clear cell chondrosarcoma represented 5.4 per cent of the whole chondrosarcomas registered in the same period. There was a male prevalence (8 cases). Most patients were in their third decade (8 cases). Clinical course was usually of long duration ranging from three months to nine years. Roentgenographically, the lesion was often indistinguishable from chondroblastoma; this tumor involved the proximal part of the femur in 8 cases, located in the epiphysis. Histologically, cells with clear cytoplasm were always present, and areas of conventional chondrosarcoma were seen in half of cases. All patients underwent surgical treatment: "en bloc" resection was performed in 12 cases, after a curettage with bone grafting in 2 cases; a simple curettage with bone grafting was performed in one case. Two recurrences occurred: a local recurrence after a bone grafting treated by resection with good result, and another recurrence with pulmonary metastasis leading to death within 8 years., Discussion: Clinical, radiographical and histological features are the same in our series and in the series published by Unni et al. in 1976 and by Bjornsson et al. in 1984. Treatment consists in "en bloc" resection because the lesion may recur after curettage. Rarely the tumor may metastasize. Prognosis is good with a five year survival rate similar to grade 1 chondrosarcoma (O'Neal and Ackerman classification)., Conclusion: These rare bone tumors are characterized by their good prognosis when the treatment is an "en bloc" resection.