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PubMed1998

Successful double autografts for patients with relapsed clear cell sarcoma of the kidney.

Although the prognosis of clear cell sarcoma of the kidney (CCSK) has improved, when metastases occur the probability of cure is very low. We have treated two pediatric patients with relapsed CCSK, one with multiple bone metastases and another with brain metastases. After one or two courses of re-induction chemotherapy and radiation therapy to the sites of metastasis, they received double high-dose chemotherapy with autologous bone marrow rescue. Conditioning regimens were ifosphamide plus melphalan for the first autograft and busulfan plus thiotepa for the second. Hematological recovery was prompt, and no severe complications were observed. They are doing well without evidence of recurrence at 19 and 49 months after the second autograft, respectively.

Bone Marrow Transplantation↗

PubMed2002

[Clear cell sarcoma of the kidney: 3 case reports].

UNLABELLED: Clear cell carcoma of the kidney is a distinct, highly malignant pediatric neoplasm. Its occurrence in adults is extremely rare and the subject of isolated case reports. MATERIAL AND METHODS: We report 3 cases of clear cell sarcoma of the kidney in one men and two women between 23 and 65 years old (mean age is 40 years). A radical nephrectomy with hilar lympgh node dissection was accomplished. A combination chemotherapy regiment (cisplatin and doxorubicin) was performed on 6 cycles in 1 case. The other 2 case was not underwent chemotherapy or radiation. RESULTS: In the patient underwent the combination chemotherapy there was not evidence of tumor in the abdomen and thorax on CT Scan 4 years later. In one of the two patient not underwent chemotherapy or radiation, the CT scan revealed a left psoas reccurrence three month later; therapy consisted for surgery without chemotherapy or radiation. Four month later, tfe CT scan revealed a reccurrence in the left retroperitoneal region and liver and speen metastasis. The patient was dead two month later. The other patient not underwent chemotherapy or radiation was dead seven month after nephrectomy. CONCLUSION: Optimal treatment is unknown, and surgery; radiotherapy and chemotherapy are used alone but mostly in combination.

Adult↗

PubMed2006

Oncogenic MITF dysregulation in clear cell sarcoma: defining the MiT family of human cancers.

Clear cell sarcoma (CCS) harbors a pathognomonic chromosomal translocation fusing the Ewing's sarcoma gene (EWS) to the CREB family transcription factor ATF1 and exhibits melanocytic features. We show that EWS-ATF1 occupies the MITF promoter, mimicking melanocyte-stimulating hormone (MSH) signaling to induce expression of MITF, the melanocytic master transcription factor and an amplified oncogene in melanoma. Knockdown/rescue studies revealed that MITF mediates the requirement of EWS-ATF1 for CCS survival in vitro and in vivo as well as for melanocytic differentiation. Moreover, MITF and TFE3 reciprocally rescue one another in lines derived from CCS or pediatric renal carcinoma. Seemingly unrelated tumors thus employ distinct strategies to oncogenically dysregulate the MiT family, collectively broadening the definition of MiT-associated human cancers.

Activating Transcription Factor 1↗

PubMed2001

Clear cell sarcoma: the Roswell Park experience.

BACKGROUND AND OBJECTIVES: Clear cell sarcoma of the tendons and aponeuroses (CCSTA) is an aggressive, rare soft-tissue tumor with approximately 300 reported cases. Although it appears to be histogenetically related to melanoma, its clinical behavior resembles soft tissue sarcoma with a propensity for lymph node metastases. We report our experience at a tertiary cancer center. METHODS: Eight cases of CCSTA evaluated at Roswell Park Cancer Institute between 1970 and 1998 were reviewed retrospectively. Patient data analyzed included patient age, gender, anatomic location, size of tumor, development of local, regional and distant recurrence, and patient status at last follow up. RESULTS: Six of eight patients were alive at 2 years, while three of seven patients were alive at 5 years. Of the patients alive with no evidence of recurrence, two had tumors of less than 2 cm, and the remaining patient had incomplete information regarding tumor size. Five patients recurred within 2 years of definitive surgical management. Four had tumors > 5 cm. All five patients progressed to metastatic disease at a median follow up of 20 months (range 1-108 months) following definitive surgical management and all eventually died of their disease at a median of 3 months (range 0-24 months) from presentation with metastatic disease. Four of five patients with lesions > 5 cm received adjuvant chemotherapy with intent to cure, but all eventually died of disease at 4, 22, 34, and 41 months from initial presentation. CONCLUSIONS: CCSTA is an aggressive tumor of the soft tissues. Early recognition and management are associated with an excellent long-term prognosis. Tumors greater than 5 cm warrant aggressive surgical management and treatment, and are at high risk of the development of distant disease. Aggressive multiagent chemotherapy appeared to have no impact on outcome. Other adjuvant therapeutic options including immunotherapy should be investigated.

Adolescent↗

PubMed2008

Treatment of recurrent clear cell sarcoma of the kidney with brain metastasis.

BACKGROUND: Clear cell sarcoma of the kidney (CCSK) is known for its propensity to metastasize to bone, but it also spreads to other sites including the brain. This study was undertaken to describe the treatment and outcomes of patients with recurrent CCSK involving the brain. METHODS: A retrospective records review was conducted on eight patients with CCSK who developed brain metastases after complete responses to initial therapy. RESULTS: The recurrences occurred at a median of 24.5 months after initial diagnosis (range, 12-53 months). At the time of recurrence, patients were treated with multimodal therapy including biopsy or resection, radiation therapy, and chemotherapy. All patients received a variable number of courses of ifosfamide, carboplatin, and etoposide (ICE), with or without other agents. Four patients received high-dose chemotherapy with autologous stem cell rescue. One patient died from complications of bacteremia 8 weeks after starting chemotherapy. The other seven patients achieved a complete response after either surgery or ICE chemotherapy. Of these, six patients were alive without disease with a median follow-up of 30 months from the time of recurrence (range, 24 to 71 months). All six survivors received radiation therapy and four had gross total resections. Three survivors received high-dose chemotherapy with stem cell rescue. CONCLUSION: Patients with recurrent CCSK involving the brain can have durable survival after recurrence. ICE chemotherapy, together with radiation therapy and surgery, provides a reasonable salvage regimen for recurrent CCSK. It is unclear whether high-dose chemotherapy confers a benefit compared to conventional-dose chemotherapy.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed1999

Clear cell sarcoma of the abdominal wall with peritoneal sarcomatosis: CT features.

Clear cell sarcoma, also called malignant melanoma of soft parts, is an uncommon neoplasm that involves tendons or aponeuroses of the lower extremity. The CT features of a clear cell sarcoma arising from the abdominal wall with later peritoneal dissemination are described. Peritoneal sarcomatosis from soft tissue sarcomas is a very rare condition previously unreported in the radiologic literature. Metastases to peritoneal surfaces must therefore be considered a possible site for systemic dissemination of soft tissue sarcomas.

Abdominal Muscles↗

PubMedDate not supplied

Clear cell sarcoma kidney: clinical features and outcome.

Clear cell sarcoma of kidney (CCSK) is a rare, highly malignant tumor. The clinical features and treatment outcome of 12 patients with CCSK are reported. From 1982 through December 1996, 12 cases of CCSK were seen at the Regional Cancer Centre, Trivandrum, India. Patients were staged according to NWTS III recommendation. They were treated with chemotherapy containing vincristine, actinomycin, and Adriamycin and radiotherapy. The survival curve was calculated by the Kaplan-Meier method. Mass and pain in the abdomen were the presenting symptoms. Male/female ratio was 3:1. Six had stage I, 4 had stage II, and 2 had stage III disease. Of the 12, 10 were evaluable, 6 are alive, and 3 recurred in 9 evaluable. Six patients are alive free of disease 10 to 108 months after diagnosis. The overall survival and disease-free survival of the 10 patients are 64 and 56%. It would appear that combined modality treatment can cure two thirds of children with CCSK. Effective treatment needs to be developed for children who fail after first line treatment.

Child↗

PubMedDate not supplied

[Clear cell sarcoma of the kidney in children].

Clear cell sarcoma of the kidney (CCSK) is a distinct, highly malignant renal tumour of childhood which was diagnosed in 5 (3.9%) of 133 primary renal tumours in children under 15 years of age in the period from 1976 to 1990. The clinical and pathological features of these patients are presented. The most common clinical features were abdominal mass and haematuria. The male to female ratio was 1: 1.5 and there was predilection for the left side (4:1). The age of patients varied from 5 months to 4 years (average age 23 months). In four children the tumour was in Stage I, and in one child in Stage II. Three tumours showed classical microscopic pattern of CCSK. The treatment for all patients consisted of preoperative chemotherapy, radical surgery and additional aggressive chemotherapy. Four patients developed metastases: three of them to bones and one to the liver. Three patients died of the tumour, one is alive but with the tumour (3 years follow-up), and one child is alive and without any evidence of disease (3-years follow-up). Although relatively uncommon, these tumours are responsible for 50% of all children's deaths from renal tumours and, so, their precise classification is essential for proper treatment.

Adolescent↗

PubMed2000

Clear cell sarcoma arising in the transverse colon.

A case of clear cell sarcoma (CCS) arising in the transverse colon is presented. The tumor consisted of sheets or small nests of epithelioid malignant cells possessing pleomorphic nuclei with one or more prominent nucleoli and ample clear or slightly eosinophilic cytoplasm. Some of the tumor cells contained various amounts of melanin pigments that were confirmed by histochemical and ultrastructural examinations. Immunohistochemical examination showed a positive immunoreactivity for HMB45 and S-100 protein. A metastatic nodule, which was found 9 months after surgery, showed similar histological findings to those of the primary one but lacked melanin pigments. Reverse transcriptase- polymerase chain reaction using total ribonucleic acid obtained from metastatic nodule demonstrated the presence of EWS-ATF-1 fusion gene. Based on these findings, the present case tumor is a CCS of the colon.

Aged↗

PubMed2005

Clear cell sarcoma of the small bowel: a potential pitfall. Case report.

Clear cell sarcoma (soft-part melanoma) is a very rare entity with a distinctive histopathologic and molecular profile. Herein, we present the sixth reported case of a primary gastrointestinal clear cell sarcoma discovered in a 21-year-old woman. The patient underwent numerous tests prior to the diagnosis of her small bowel pathology, including the use of capsule endoscopy, which allowed for visualization and final localization of the tumour. Additionally, we discuss this rare type of sarcoma that affects young adults and has a poor prognosis characterized by the balanced chromosomal translocation t(12;22)(q13;q12) with special emphasis on the necessity for pathologists to be able to distinguish it from melanoma -- potentially a major pitfall in diagnosis.

Adult↗

PubMedDate not supplied

Clear cell sarcoma of soft tissues in children and young adults: the St. Jude Children's Research Hospital experience.

Clear cell sarcoma is a rare soft tissue neoplasm whose clinical behavior and outcome has not been previously characterized. This study reviewed the clinical characteristics and outcome of all children with clear cell sarcoma of the soft tissues who were treated at St. Jude Children's Research Hospital from March 1962 through August 1998. Of 225 children with nonrhabdomyosarcomatous soft tissue sarcomas, 5 (2.2%) were diagnosed with clear cell sarcoma. Median age at diagnosis was 15 years 3 months. Primary sites included the extremities (n = 3), chest wall (n = 1), and abdomen (n = 1). At diagnosis 3 patients had localized disease. Following surgical resection (n = 3), radiotherapy (n = 2), and chemotherapy (n = 1) all three survive disease-free 10, 11, and 90 months after diagnosis, respectively. The remaining two patients with metastatic disease at diagnosis died 21 days and 9 months after diagnosis. Clear cell sarcoma of the soft tissues is rare in pediatrics. Complete surgical resection with negative margins is the most effective treatment for this disease. Patients with metastatic disease are candidates for multiinstitutional chemotherapy trials.

Adolescent↗

PubMed2001

[Clear cell sarcoma (malignant melanoma of soft tissues) of the calf ].

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.

Adult↗

PubMed2002

Establishment and characterisation of a human clear cell sarcoma model in nude mice.

We have established a new experimental model of human clear cell sarcoma, UM-CCS1, using serial subcutaneous transplantation of intact tumour tissue in nude mice. The heterotransplanted nude mouse tumours retained characteristic morphological features of the primary clear cell sarcoma. Immunohistochemical analysis showed the retained expression patterns of S-100 protein, melanoma-associated antigen HMB-45 and vimentin in the xenografts as compared to the primary tumour. DNA index showed low variations both between the xenografts in the same passage and between the serial passages. Cytogenetic analysis of the primary tumour and the xenografts showed the unbalanced translocation der(6)t(6;12)(p23;q13). Based on the combined genetic data a reasonable interpretation of our findings is that there was a complex chromosomal rearrangement resulting in a cytogenetically cryptic EWS-ATF1 fusion gene. Analysis of cell kinetics using in vivo incorporation of iododeoxyuridine and flow cytometry showed generally short potential doubling time (T(pot)) of the xenografts. Volume doubling time showed low variations without correlation with T(pot). The retained phenotypic and genotypic characteristics of the primary tumour and the morphological and structural stability over time makes the model suitable for studies on the tumour biology and treatment of clear cell sarcoma.

Activating Transcription Factor 1↗

PubMedDate not supplied

Clear cell sarcoma: a dilemma on pathological staging and clinical management.

Clear cell sarcoma of the kidney (CCSK) has been classified as high risk tumour in the previous UK and international Wilms tumor studies. The current Society of Paediatric Oncology (SIOP) trial, UK version, advocates chemotherapy including doxorubicin prior to nephrectomy. Pathological staging and histology of the resected tumor are then applied to determine the extent and intensity of the postoperative therapy. We describe an unusual case with a trilobed tumor and debate the appropriate postoperative management.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed1995

Clear-cell sarcoma of tendons and aponeuroses studied by immunoscintigraphy.

Clear-cell sarcoma is a rare tumor that arises in association with tendons and aponeuroses. Although it shares with malignant melanoma several histologic and ultrastructural features, it has a clinical course different from that of conventional melanomas. A case of clear-cell sarcoma studied by immunoscintigraphy with 99mTc-labeled F(ab')2 fragments of the monoclonal antibody 225.28 S is reported.

Adenocarcinoma, Clear Cell↗

PubMed2005

[Clear-cell sarcoma of tendons and aponeuroses: three case reports].

Clear-cell sarcoma (CCS) of tendons and aponeuroses is a rare malignant tumor representing about 1% of soft tissue tumors. Preferentially observed in young adults in the second or third decade, the tumor generally develops in the limbs. Only 2% of SCC of tendons and aponeuroses have been reported in children less than 10 years of age. This slowly progressive tumor usually forms a painless mass. The tumor increases in size followed by metastatic dissemination to lymph nodes and the lungs. The prognosis is related to tumor size. At the present time, the recognized limit is greater than 5 cm. Early diagnosis must be achieved to enable effective treatment by carcinological surgical resection. We report the three cases of CCS of tendons and aponeuroses observed at the Strasbourg University hospital over a 35-year period. Each case had a special clinical presentation. The first patient, treated in 1967, presented tumor bone lysis on the plain x-ray, an observation rarely reported in the literature. In the second patient, treated in 2002, the tumor was discovered after trauma. This patient developed skin ulceration associated with paraplegia secondary to metastatic thoracic cord compression. The third case occurred in a 12-year-old girl, treated in 2002.

Adult↗

PubMed1993

Translocation t(12;22)(q13;q12.2-12.3) in a clear cell sarcoma of tendons and aponeuroses.

Cytogenetic analysis of a short-term culture from a clear cell sarcoma revealed a complex karyotype with the mainline of 49,XY,t(7;18)(p11.2;q21.3), +der(7)t(7;18)(p11.2;q21.3), +8, +der (8;17)(q10;q10),t(12;22)(q13;q12.2-12.3),add(13)(p13). An apparently identical translocation t(12;22) has been described recently in four clear cell sarcomas, indicating that this constitutes a primary cytogenetic change specific for this type of tumor. In our case, the breakpoint on chromosome 22 could be assigned to band 22q12.2 or 22q12.3. Together with the present case, trisomy or tetrasomy 8 has been found in six of nine clear cell sarcomas, suggesting that, as in Ewing's sarcoma and myxoid liposarcoma, trisomy/tetrasomy 8 represents a nonrandom secondary aberration. We conclude that the finding of the specific translocation t(12;22) may prove to be an important marker in the differential diagnosis of clear cell sarcoma from some other soft tissue sarcomas and malignant melanoma.

Adult↗

PubMed2002

Clear cell sarcoma of the kidney in an adult. A case report.

DEFINITION: Clear cell sarcoma of the kidney was initially thought to be a variant of Wilms tumour with an unfavourable prognosis. METHODS: A 23 years-old woman presented with a right abdominal mass. CT scan revealed a solid 23 cm tumour of the right kidney. A right radical nephrectomy with retroperitoneal lymph node dissection was accomplished. The histological diagnosis was clear cell sarcoma. A combination chemotherapy regimen (cisplatin and doxorubicin) was performed on six cycles. RESULTS: After two years, there was no evidence of tumour in the abdomen and thorax on CT Scan. The patient is being well. CONCLUSION: Optimal treatment is unknown, and surgery, radiotherapy and chemotherapy are used alone but mostly in combination.

Adult↗