Clear Cell Sarcoma of the Kidney

Aldera, Alessandro Pietro; Pillay, Komala

doi:10.5858/arpa.2018-0353-rs

Cited by 35 publications

(34 citation statements)

References 23 publications

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“…Of the 17 adult CCSK patients, 11 had immunohistochemistry data. Ten cases stained positive for vimentin, which is consistent with most literature ( 12 , 13 ). In fact, vimentin positivity is a strong pathological characteristic of CCSK.…”

Section: Discussionsupporting

confidence: 91%

Clear cell sarcoma of the kidney in an adult: a case report and literature review

Cao¹,

Zhang²,

Ma³

et al. 2022

Transl Cancer Res TCR

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Clear cell sarcoma of the kidney (CCSK) in adults is extremely rare. In fact, only 16 adult CCSK cases have been reported from 1989 to 2020 in the English language literature. The pathologic diagnosis of the disease is difficult, and the optimal treatment is still unknown. Currently, no literature review has been done on adult CCSK patients. Herein, we report the case of a 24-year-old man who presented with right flank pain for one month. The patient underwent a series of diagnostic tests, and imaging examinations revealed a large mass in his right kidney. The patient underwent retroperitoneal laparoscopic nephrectomy and regional lymphadenectomy. Pathological examination of the tumor revealed nests and cords of fairly uniform oval cells with clear cytoplasm. Immunohistochemistry showed that the tumor cells were positive for vimentin, CyclinD1, and Bcl-2 and that the sample was uniformly negative for Wilms' tumor 1 (WT1), CD34, desmin, and cytokeratin staining. Based on these histopathological and immunohistochemical results, the patient was diagnosed with CCSK. The patient subsequently refused chemotherapy and radiotherapy.During the 2-year follow-up, no recurrence or metastasis was observed. We reviewed the English language literature on adult CCSK published in the PubMed database. A pooled analysis was performed, and the results suggested that an accurate pathological diagnosis of CCSK could be achieved based on microscopy and immunohistochemistry. Nephrectomy and regional lymphadenectomy are the main treatments for adult patients with CCSK. While the value of adjuvant radiotherapy and chemotherapy remains controversial, multimodal oncologic treatment, including surgery and chemotherapy with or without radiation, may be efficacious in preventing local recurrence and distant metastases.

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Section: Discussionsupporting

confidence: 91%

Clear cell sarcoma of the kidney in an adult: a case report and literature review

Cao¹,

Zhang²,

Ma³

et al. 2022

Transl Cancer Res TCR

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“…It has been reported that BCOR family tumours show substantial morphological overlap, 1,15 and delicate capillary networks were observed not only in BCS, but also in other BCOR mutant tumours such as clear cell sarcoma of the kidney. [15][16][17][18] Tumour cell clusters with thin and delicate vascular cores and tiny vascular fragments of BCS may reflect the cytopathological features of BCOR mutant tumours.…”

Section: Discussionmentioning

confidence: 99%

Cytological features of BCOR‐CCNB3 sarcoma: Comparison with Ewing sarcoma and synovial sarcoma

et al. 2021

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Introduction BCOR‐CCNB3 sarcoma (BCS) is one of the histological types classified as an undifferentiated small round cell sarcoma of bone and soft tissue. This sarcoma frequently develops in males under 20 years of age. Histologically, a delicate capillary network has been reported as a conspicuous finding. In this study, the cytological findings of BCS were observed in two cases of primary lesions and one case of a lung metastatic lesion. The cytological findings of BCS were compared with its histological mimics, and the characteristic findings of BCS were examined. Methods Three cases of BCS were studied, and a cytological comparison was performed with 8 cases of Ewing sarcoma (ES) and 10 cases of synovial sarcoma (SS; monophasic type: 7 cases, biphasic type: 2 cases, poorly differentiated: 1 case). Results In all BCS cases, small clusters with thin and delicate vascular cores and tiny vascular fragments were conspicuous. In ES and SS cases, although small clusters with vascular cores were observed, the vascular cores were thicker than in BCS, and no tiny vascular fragments appeared in most cases. Cytomorphological differences of tumour cells were also observed among BCS, ES, and SS. Predominantly rounded nuclei with fine chromatin and inconspicuous nucleoli can be cytological clues for BCS. Conclusions BCS shows characteristic cytological findings that make the diagnosis of BCS more likely than that of ES and SS. Cytological evaluation is a useful tool for appropriate differential diagnosis that leads to a more accurate final diagnosis and rapid treatment.

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“…Our cases were older, in the fifth decade, and both presented with synchronous pulmonary metastases at diagnosis. As CCSK are known to evolve with very long‐term pulmonary and bone metastasis [ 39 ], it was tempting to document these bone/pulmonary tumours as metastases from an occult CCSK. We found no argument in the medical records arguing in favour of this hypothesis.…”

Section: Discussionmentioning

confidence: 99%

Intra‐ and extra‐cranial BCOR‐ITD tumours are separate entities within the BCOR‐rearranged family

Bouchoucha

Tauziède‐Espariat

Gauthier

et al. 2022

The Journal of Pathology CR

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BCOR‐ITD tumours form an emerging family of aggressive entities with an internal tandem duplication (ITD) in the last exon of the BCOR gene. The family includes cerebral tumours, termed central nervous system BCOR‐ITD (CNS BCOR‐ITD), and sarcomatous types described in the kidney as clear cell sarcoma of the kidney (CCSK), in the endometrium as high‐grade endometrial stromal sarcoma, and in the bone and soft tissue as undifferentiated round cell sarcoma or primitive myxoid mesenchymal tumour of infancy. Based on a series of 33 retrospective cases, including 10 CNS BCOR‐ITD and 23 BCOR‐ITD sarcomas, we interrogated the homogeneity of the entity regarding clinical, radiological, and histopathological findings, and molecular signatures. Whole‐transcriptomic sequencing and DNA methylation profiling were used for unsupervised clustering. BCOR‐ITD tumours mostly affected young children with a median age at diagnosis of 2.1 years (range 0–62.4). Median overall survival was 3.9 years and progression‐free survival was 1.4 years. This dismal prognosis is shared among tumours in all locations except CCSK. Histopathological review revealed marked differences between CNS BCOR‐ITD and BCOR‐ITD sarcomas. These two groups were consistently segregated by unsupervised clustering of expression (n = 22) and DNA methylation (n = 21) data. Proximity between the two groups may result from common somatic changes within key pathways directly related to the novel activity of the ITD itself. Conversely, comparison of gene signatures with single‐cell RNA‐Seq atlases suggests that the distinction between BCOR‐ITD sarcomas and CNS BCOR‐ITD may result from differences in cells of origin.

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Clear Cell Sarcoma of the Kidney

Cited by 35 publications

References 23 publications

Clear cell sarcoma of the kidney in an adult: a case report and literature review

Clear cell sarcoma of the kidney in an adult: a case report and literature review

Cytological features of BCOR‐CCNB3 sarcoma: Comparison with Ewing sarcoma and synovial sarcoma

Intra‐ and extra‐cranial BCOR‐ITD tumours are separate entities within the BCOR‐rearranged family

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