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PubMedDate not supplied

[Clear cell sarcoma and sentinel lymph node biopsy. Case report and literature review].

The procedure of sentinel node biopsy has been used previously in clear cell sarcoma. There are few studies reported. Due to the similar biological features with melanoma, this procedure can be effective. Sentinel biopsy is an option to detect nodal subclinical metastases. A 19-year-old man presented with ulcerated lesion in the fifth finger of the left hand. The biopsy reported clear cell sarcoma. Immunohistochemistry was positive for Vimentin and S-100. There was no evidence of regional disease. The sentinel lymph node biopsy, using patent blue and Tc-99 rhenium, was positive for metastases. Axillar dissection was carried out. The final report confirmed three metastasic nodes and the patient received adjuvant chemotherapy.

Adult↗
PubMed2000

MR imaging of clear cell sarcoma (malignant melanoma of the soft parts): a multicenter correlative MRI-pathology study of 21 cases and literature review.

OBJECTIVE: To evaluate MR imaging and pathology findings in order to define the characteristic features of clear cell sarcoma of the soft tissues (malignant melanoma of the soft parts). DESIGN AND PATIENTS: MR examinations of 21 patients with histologically proven clear cell sarcoma of the musculoskeletal system were retrospectively reviewed and assessed for shape, homogeneity, delineation, signal intensities on T1- and T2-weighted images, contrast enhancement, relationship with adjacent fascia or tendon, secondary bone involvement, and intratumoral necrosis. In 19 cases the pathology findings were available for review and for a comparative MR-pathology study. RESULTS: On T1-weighted images, lesions were isointense (n=3), hypointense (n=7) or slightly hyperintense to muscle (n=11). Immunohistochemical examination was performed in 17 patients. All 17 specimens showed positivity for HMB-45 antibody. In nine of 11 lesions with slightly increased signal intensity on T1-weighted images, a correlative MR imaging-pathology study was possible. All nine were positive to HMB-45 antibody. CONCLUSIONS: Clear cell sarcoma of the musculoskeletal system often has a benign-looking appearance on MR images. In up to 52% of patients, this lesion with melanocytic differentiation has slightly increased signal intensity on T1-weighted images compared with muscle. As the presence of this relative higher signal intensity on T1-weighted images is rather specific for tumors displaying melanocytic differentiation, radiologists should familiarize themselves with this rare entity and include it in their differential diagnosis when confronted with a well-defined, homogeneous, strongly enhancing mass with slightly higher signal intensity compared with muscle on native T1-weighted images.

Adolescent↗
PubMedDate not supplied

Clear cell sarcoma.

Explore the source record for details and available documents.

Adult↗
PubMed1991

Clear cell sarcoma of the kidney: CT, sonographic, and pathologic correlation.

The sonographic and computed tomographic findings in 12 children aged 1-6 years with clear cell sarcoma of the kidney were reviewed retrospectively. Tumor size, calcification, and internal architecture were characterized and correlated with the gross pathologic findings. All tumors were unilateral and large (8.5-16 cm in diameter). Except for one, all masses were predominantly solid, and all contained some well-defined portions of low attenuation or hypoechogenicity that represented tumor necrosis. In addition, seven tumors contained uncomplicated fluid-filled cysts with diameters ranging from a few millimeters to 5 cm. Extension into the inferior vena cava was not noted. The radiologic features of clear cell sarcoma of the kidney are common to all malignant renal neoplasms. Some cases, however, may have features that simulate those of benign conditions, such as multilocular cystic nephroma or segmental cystic dysplasia. No pattern was discerned that would permit discrimination between clear cell sarcoma of the kidney and the most common renal neoplasm of childhood, Wilms tumor.

Child↗
PubMed1994

A patient with rhabdomyosarcoma and clear cell sarcoma of the skin.

We describe a patient with rhabdomyosarcoma of the posterior cervical region and clear cell sarcoma on the occipital scalp. These two tumors later metastasized to distant skin. We differentiated these tumors by histopathologic, histochemical, immunohistochemical, and ultrastructural findings. Cells of the posterior cervical tumor showed differentiation toward striated muscle, whereas those of the occipital tumor showed findings compatible with melanocytic differentiation.

Aged↗
PubMed1998

Clear cell sarcoma of kidney with tumor extension into the right atrium.

A 28-year-old man with a Wilms tumor extending into his right atrium was successfully treated using cardiopulmonary bypass. Histologic examination revealed clear cell sarcoma with invasion of the inferior vena cava. This case represents the oldest patient with clear cell sarcoma of the kidney ever published in the English literature.

Adult↗
PubMed1987

[Orbital localization of clear-cell sarcoma of tendons and aponeuroses. Apropos of a case report].

Clear cell sarcoma of tendons and aponeuroses actually is a well identified soft tissue tumor. A primary orbital involvement is presented, with an optical and electron microscopic study. The orbital topography of such a tumor seems to be an exceptional opportunity. The histological study showed the typical pattern of this clear cell tumor. Ultrastructurally, there are not absolute specific features to establish the diagnosis, except the finding of intra-cellular melanosomes. Histogenesis has been debated for a long time between a melanotic or a synovial origin. According to present knowledge, this tumor may be attributed to a neural crest origin.

Adult↗
PubMed1991

Clear cell sarcomas and metastatic soft tissue melanomas. A flow cytometric comparison and prognostic implications.

Eleven soft tissue clear cell sarcomas (CCS) were examined flow cytometrically for their DNA content with a correlation of the DNA with the patients' survival. Diploidy was expressed in six sarcomas and it conferred a longer survival (mean, 68.6 months) than aneuploid sarcomas (mean, 8.2 months). For comparison, 13 metastases to soft tissues from cutaneous melanomas were assessed by flow cytometric study. The aneuploid neoplasms of both entities were categorized into low and high degree based on extent of the DNA index (DI). Low degree was defined by a DI of less than or equal to 1.5 and high degree by a DI of greater than 1.5. Significant differences in DNA content between CCS and metastatic melanomas were observed. Melanomas were preponderantly aneuploid (11/13) of high degree (mean DI, 1.7) whereas CCS manifested more diploidy (six of 11) and their aneuploidy was of a low degree (mean DI, 1.2; P = 0.001). Clear cell sarcomas and melanomas were also examined for their immunoreactivity to S-100 protein and HMB-45 antigen. All CCS reacted with S-100 protein and HMB-45. In CCS the reactions were diffuse for both in six tumors, diffuse with HMB-45 and moderate to S-100 protein in three tumors and diffuse with S-100 and moderate with HMB-45 in two tumors. All melanomas reacted diffusely to S-100 protein except for one heavily pigmented tumor which reacted only focally. The reaction to HMB-45 was diffuse in nine and focal in three melanomas. These data suggest that measurements of DNA content in CCS may be valuable in predicting clinical outcome and that there are quantitative differences in DNA content between CCS and metastatic melanoma in soft tissues.

Adolescent↗
PubMed1983

Clear-cell sarcoma of tendons and aponeuroses. An immunohistochemical and electron microscopic analysis indicating neural crest origin.

A clinico-pathological, light microscopic and immunohistochemical study of 15 clear-cell sarcomas, with an ultrastructural analysis of 6 of the tumors, is presented. The tumors showed a strong predilection for tendons and aponeuroses of the extremities in predominantly young and middle-aged people. The clinical setting, course and light microscopic appearance agree well with the original description by Enzinger (1965). Nine of the 15 patients developed metastases, most of them including lymph nodes, and 8 of the patients had died at the time of follow-up (median follow-up time 4.8 years). Reducing pigment was demonstrated within the cells of 2 tumors. Ultrastructurally the 6 tumors studied had a uniform appearance with characteristically rounded or oval tumor cells with a single nucleus containing one or two very prominent nucleoli, a light-staining cytoplasm with a moderate amount of organelles and a variable content of glycogen. Polymorphic melanosomes were seen in the cells of one of the tumors. External laminas enclosed groups of tumor cells and invested parts of individual tumor cells. With immunoperoxidase analysis for S-100 protein positive staining was observed in the vast majority of the tumor cells of all 15 clear-cell sarcomas. Metastases appearing in 9 of the 15 cases showed positive staining for S-100 protein. There was a strong staining of the cytoplasm and generally a weak and varying staining of nuclei. The immunohistochemical and electron microscopic findings indicate that clear-cell sarcoma is a homogenous entity among soft tissue sarcomas, of probable neural crest derivation.

Adolescent↗
PubMed1993

Clear cell sarcoma of the kidney in a 4-month-old infant: a case report.

A case of clear cell sarcoma of the kidney is described in a 4-month-old male infant. The tumor had a typical histological pattern, consisting of sheets of tumor cells possessing "empty" nuclei set within a delicate capillary network. There was extensive necrosis and tumor cell cytoplasm contained vimentin. This tumor occurs only rarely in infants less than six months of age. The different renal neoplasms of childhood have diverse prognoses and demand specific therapy so that accurate diagnosis is mandatory. The present case illustrates that clear cell sarcoma, a highly malignant neoplasm, must be considered in the differential diagnosis of a renal mass occurring in this age group.

Child, Preschool↗
PubMed1982

Clear cell sarcoma and selective IgM deficiency: a case report.

A case of clear cell sarcoma of tendons and aponeuroses and a co-existent IgM deficiency is reported. The tumor arose in the Achilles tendon with metastases to the skin, bone, and lymph nodes. The tumor, examined by light and electron microscopy, consisted of glycogen-containing clear cells with melanotic and amelanotic features. There was no detectable serum IgM. The IgA levels were normal and IgG levels were elevated. Peripheral blood lymphocytes contained a normal amount (4.5%) of IgM-bearing cells. Cultured mononuclear cells from the patient suppressed production of IgM by normal lymphocytes, suggesting a role of suppressor cells in the IgM deficiency. The co-existence of soft tissue sarcomas and immunoglobulin deficiency states is reviewed.

Achilles Tendon↗
PubMed1978

Clear cell sarcoma with melanin: report of two cases.

Two cases of clear cell sarcoma, one with many melanin pigmented cells and the other scanty, are described. Both are associated with tendons of the lower extremities of young Chinese adults. Their clinical behaviour differed vastly; one patient was dead seven months after local excision and block dissection of regional lymph nodes, while the other had a history of the growth of eight years prior to local excision and is well four years later.

Adolescent↗
PubMed1986

Clear cell sarcoma of the kidney. An immunohistochemical study.

Three cases of clear cell sarcoma of the kidney (CCSK) and 5 cases of Wilms' tumor were investigated immunohistochemically to examine the expression of tissue-specific intermediate filaments (cytokeratin, vimentin, and desmin) and myoglobin. In CCSK, tumor cells were negative for cytokeratin, except for occasional tubular structures, and vimentin was demonstrated in only one case. In Wilms' tumor, epithelial components were positive for cytokeratin and stromal cells were positive for vimentin, while no staining was found in blastemal cells for either. Both desmin and myoglobin were negative in all tumor cells except for skeletal muscle cells in Wilms' tumor. In the current study, some neoplastic cells in CCSK were revealed to be of mesenchymal nature, but blastemal cells in Wilms' tumor were not.

Antibodies, Monoclonal↗
PubMed1990

S-100 protein in clear cell sarcomas of the tendon sheath.

Fourteen histologically proved specimens of clear cell sarcomas (CCS) of tendon sheaths were studied for the presence of S-100 protein. Eleven cases were strongly positive, and 2 cases were weakly positive. There was only one case which was absolutely negative for this protein. This technique was useful to diagnose tumours which were not only easily diagnosed as CCS or soft tissue melanomas, but also in the atypical and spindle cell variants of CCS which have to be weeded out of the large family of other morphologically allied soft tissue tumours.

Humans↗
PubMedDate not supplied

Clear cell sarcoma of the kidney: an unusual presentation and review of the literature.

PURPOSE: To describe a child with clear cell sarcoma of the kidney (CCSK) with an unusual presentation, including a primary tumor of the left kidney with metastases to the right kidney and soft tissues of the lower extremities, and to review the literature. PATIENT AND METHODS: An 8-month-old infant presented with hypertension, an abdominal mass, and soft tissue masses in the left thigh and right foot. Imaging studies revealed a large left-sided renal tumor, left paravertebral soft tissue masses, and left thigh mass. At laparotomy, a lesion was noted in the lower pole of the contralateral kidney. CCSK with metastases to the contralateral kidney and to the soft tissues of left thigh, right foot, and left paravertebral region was diagnosed on histopathologic examination. RESULTS: Multimodal oncologic treatment included surgery, chemotherapy, and radiotherapy. Three months after completion of therapy, a soft tissue lesion in the left arm and, later, soft tissue lesions involving multiple parts of the body developed. The patient died 18 months after diagnosis without clinical or radiographic evidence of bone involvement. CONCLUSIONS: In a review of the literature, CCSK is most commonly associated with bone and lung metastases. Soft tissue involvement is uncommon. Metastasis to the contralateral kidney at initial diagnosis has not previously been reported. This case represents an unusual metastatic pattern of CCSK.

Combined Modality Therapy↗