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 melanoma of soft parts.[2]

## Contents

* 1 Signs and symptoms
* 2 Cause
* 3 Treatment
* 4 References
* 5 External links

## Signs and symptoms

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

## Cause

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

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

1. ↑ "Clear cell sarcoma of the kidney". Journal of Postgraduate Medicine 47 (3): 206–7. 2001. PMID 11832627.
2. ↑ "Soft tissue tumors: Clear cell sarcoma.". Atlas of Genetics and Cytogenetics in Oncology and Haematology 7 (1): 49–53. 2003. doi:10.4267/2042/37939. https://biblio.ugent.be/publication/4181590.
3. ↑ "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. January 2000. doi:10.1097/00000478-200001000-00002. PMID 10632483.
4. ↑ "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. November 2005. doi:10.1158/1078-0432.CCR-05-1354. PMID 16299227.
5. ↑ "Clear Cell Sarcoma of the Kidney". Medscape Reference. 11 February 2014. http://emedicine.medscape.com/article/993245-overview.
6. ↑ "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. February 2004. doi:10.1200/JCO.2004.06.058. PMID 14752069.

## External links

* 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 the U.S. National Cancer Institute document "Dictionary of Cancer Terms".

{{Navbox

| name = Soft tissue tumors and sarcomas
| title = Connective/[[Physics:Soft tsoft tissue tumors and sarcomas (ICD-O 8800–9059) (C45–C49/D17–D21, 171/214–215)
| state = autocollapse|

| listclass = hlist

| group1 = Not otherwise specified
| list1 =

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

| group2 = Connective tissue neoplasm

| list2 =

Fibromatous|

| Fibroma/fibrosarcoma:|

* Dermatofibrosarcoma protuberans
* Desmoplastic fibroma

|

Fibroma/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

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

| group3 = Lipomatous
| list3 =

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

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

| group4 = Myomatous

| list4 =

general:|

* Myoma/myosarcoma

|

smooth muscle:|

* Leiomyoma/leiomyosarcoma

skeletal muscle:|

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

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

| group5 = Complex mixed and stromal
| list5 =

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

| group6 = Mesothelial
| list6 =

* Mesothelioma
* Adenomatoid tumor

}} Template:Urologic neoplasia

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