Clear-cell sarcoma of the kidney

Medical condition

Clear-cell sarcoma of the kidney

Specialty| Oncology, nephrology

Clear cell sarcoma of the kidney (CCSK) is an extremely rare type of kidney cancer comprising 3% of all pediatric renal tumours.[1] Clear cell sarcoma of the kidney can spread from the kidney to other organs, most commonly the bone, but also including the lungs, brain, and soft tissues of the body.

Despite the similarities in names, clear cell sarcoma of the kidney is unrelated to clear cell sarcoma of soft tissue, also known as malignant melanoma of soft parts.[2]

## Signs and symptoms

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Typical gross features include large size (mean diameter 11.3 cm), a mucoid texture, foci of necrosis, and prominent cyst formation.[3]

## Cause

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Research in 2005 showed that CCSK can arise within a renal mesenchymal cell that shows a wide variety of neural markers.[4] Typical presentation is between 1 and 4 years of age, and a 2:1 male-to-female ratio is observed.[5]

## Treatment

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2004 research showed that CCSK patients exhibit an improved relapse-free survival from a longer course of therapy when using vincristine, doxorubicin, and dactinomycin, but their long-term survival is unchanged compared with patients receiving 6 months of therapy.[6]

## References

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1. ^ Sharma SC, Menon PA (2001). "Clear cell sarcoma of the kidney". Journal of Postgraduate Medicine. 47 (3): 206–7. PMID 11832627.
2. ^ Poppe B, Forsyth R, Dhaene K, Speleman F (2003). "Soft tissue tumors: Clear cell sarcoma". Atlas of Genetics and Cytogenetics in Oncology and Haematology. 7 (1): 49–53. doi:10.4267/2042/37939. hdl:1854/LU-4181590.
3. ^ Argani P, Perlman EJ, Breslow NE, Browning NG, Green DM, D'Angio GJ, Beckwith JB (January 2000). "Clear cell sarcoma of the kidney: a review of 351 cases from the National Wilms Tumor Study Group Pathology Center". The American Journal of Surgical Pathology. 24 (1): 4–18. doi:10.1097/00000478-200001000-00002. PMID 10632483.
4. ^ Cutcliffe C, Kersey D, Huang CC, Zeng Y, Walterhouse D, Perlman EJ (November 2005). "Clear cell sarcoma of the kidney: up-regulation of neural markers with activation of the sonic hedgehog and Akt pathways". Clinical Cancer Research. 11 (22): 7986–94. doi:10.1158/1078-0432.CCR-05-1354. PMID 16299227. S2CID 9130030.
5. ^ Seibel N (11 February 2014). "Clear Cell Sarcoma of the Kidney". Medscape Reference. Retrieved 18 May 2014.
6. ^ Seibel NL, Li S, Breslow NE, Beckwith JB, Green DM, Haase GM, Ritchey ML, Thomas PR, Grundy PE, Finklestein JZ, Kim T, Shochat SJ, Kelalis PP, D'Angio GJ (February 2004). "Effect of duration of treatment on treatment outcome for patients with clear-cell sarcoma of the kidney: a report from the National Wilms' Tumor Study Group". Journal of Clinical Oncology. 22 (3): 468–73. doi:10.1200/JCO.2004.06.058. PMID 14752069.

## External links

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* Clear cell sarcoma of the kidney entry in the public domain NCI Dictionary of Cancer Terms

Classification|

* ICD-10: C64
* ICD-O: 8964/3

|

External resources|

* Orphanet: 457246

This article incorporates public domain material from Dictionary of Cancer Terms. U.S. National Cancer Institute.

* v
* t
* e

Connective/soft tissue tumors and sarcomas

Not otherwise specified|

* Soft-tissue sarcoma
* Desmoplastic small-round-cell tumor
* Skin sarcoma

Connective tissue neoplasm|

| Fibromatous|

| Fibroma/fibrosarcoma|

* Dermatofibrosarcoma protuberans
* Desmoplastic fibroma

|

Fibroma/fibromatosis|

* Aggressive fibromatosis
* Aggressive infantile fibromatosis
* Aponeurotic fibroma
* Collagenous fibroma
* Diffuse infantile fibromatosis
* Familial myxovascular fibromas
* Fibroma of tendon sheath
* Fibromatosis colli
* Infantile digital fibromatosis
* Juvenile hyaline fibromatosis
* Plantar fibromatosis
* Pleomorphic fibroma
* Oral submucous fibrosis
* Pachydermodactyly

Histiocytoma/histiocytic sarcoma|

* Benign fibrous histiocytoma
* Malignant fibrous histiocytoma
* Atypical fibroxanthoma
* Solitary fibrous tumor

Myxomatous|

* Myxoma/myxosarcoma
* Cutaneous myxoma
* Superficial acral fibromyxoma
* Angiomyxoma
* Ossifying fibromyxoid tumour

Fibroepithelial|

* Brenner tumour
* Fibroadenoma
* Phyllodes tumor

Synovial-like|

* Synovial sarcoma
* Clear-cell sarcoma

Lipomatous|

* Lipoma/liposarcoma
* Myelolipoma
* Myxoid liposarcoma
* PEComa
* Angiomyolipoma

* Chondroid lipoma
* Intradermal spindle cell lipoma
* Pleomorphic lipoma
* Lipoblastomatosis
* Spindle cell lipoma
* Hibernoma

Myomatous|

| General|

* Myoma/myosarcoma

|

Smooth muscle|

* Leiomyoma/leiomyosarcoma

Skeletal muscle|

* Rhabdomyoma/rhabdomyosarcoma: Embryonal rhabdomyosarcoma
* Sarcoma botryoides
* Alveolar rhabdomyosarcoma

* Leiomyoma
* Cutaneous
* Angioleiomyoma
* Angiolipoleiomyoma
* Genital leiomyoma
* Leiomyosarcoma
* Multiple cutaneous and uterine leiomyomatosis syndrome
* Multiple cutaneous leiomyoma
* Neural fibrolipoma
* Solitary cutaneous leiomyoma
* STUMP

Complex mixed and stromal|

* Adenomyoma
* Pleomorphic adenoma
* Mixed Müllerian tumor
* Mesoblastic nephroma
* Wilms' tumor
* Malignant rhabdoid tumour
* Clear-cell sarcoma of the kidney
* Hepatoblastoma
* Pancreatoblastoma
* Carcinosarcoma

Mesothelial|

* Mesothelioma
* Adenomatoid tumor

* v
* t
* e

Tumors of the urinary and genital systems

Kidney|

| Glandular and epithelial neoplasm|

* Renal cell carcinoma
* Renal oncocytoma

|

Mixed tumor|

* Wilms' tumor
* Mesoblastic nephroma
* Clear-cell sarcoma of the kidney
* Angiomyolipoma
* Cystic nephroma
* Metanephric adenoma

by location|

* Renal medullary carcinoma
* Juxtaglomerular cell tumor
* Renal medullary fibroma

Ureter|

* Ureteral neoplasm

Bladder|

* Transitional cell carcinoma
* Squamous-cell carcinoma
* Inverted papilloma

Urethra|

* Transitional cell carcinoma
* Squamous-cell carcinoma
* Adenocarcinoma
* Melanoma

Other|

* Malignant fibrous histiocytoma

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