Renal anaplastic sarcoma

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Medical condition

Renal anaplastic sarcoma

Specialty| Urology, oncology

Renal anaplastic sarcoma is a rare tumour of the kidney.[1][2] By 2017 about 25 cases have been reported.[3] This tumour occurs in children and young adults and is more common in females than males.

Because of its rarity its natural history is not well understood.

## Genetics

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An association with mutations in the DICER-1 gene has been reported.[4][5][6]

## Diagnosiss

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Aspiration cytology may be of use in making the diagnosis. CT scans of the abdomen and the rest of the body are normally done to assist in surgical planning.

The age at diagnosis of this condition varies between 10 months to 41 years.[7] The male:female ratio is 2:3. The most common presentation is an asymptomatic abdominal mass. The tumour is more common on the right and it may metastise to lung, liver and bone.

### Histology

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On histology the tumours have a marked spindle cell component. Other cells may be bizarre in shape. Cartilage or bone tissue may be found. A cystic component may be present.

The differential diagnosis includes

* anaplastic Wilms' tumor
* renal synovial sarcomas
* malignant mesenchymomas
* ectomesenchymomas

## Treatment

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Because of the rarity of this tumour, optimal treatment is as yet unknown. It is usually treated by excision. Radiation and chemotherapy have also been used in addition to surgery.

## History

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This tumour was first described in 2007.[7] This lesion was recognised during a review of a series of 15,000 renal tumours.

## References

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1. ^ Watanabe N, Omagari D, Yamada T, Nemoto N, Furuya T, Sugito K, Koshinaga T, Yagasaki H, Sugitani M (2013) Anaplastic sarcoma of the kidney: case report and literature review. Pediatr Int 55(5):e129-132
2. ^ Labanaris A, Zugor V, Smiszek R, Nützel R, Kühn R (2009) Anaplastic sarcoma of the kidney. ScientificWorldJournal 9:97-101
3. ^ Arabi H, Al-Maghraby H, Yamani A, Yousef Y, Huwait H (2016) Anaplastic sarcoma of the kidney: A rare unique renal neoplasm. Int J Surg Pathol 24(6):556-561
4. ^ Wu MK, Vujanic GM, Fahiminiya S, Watanabe N, Thorner PS, O'Sullivan MJ, Fabian MR, Foulkes WD (2017) Anaplastic sarcomas of the kidney are characterized by DICER1 mutations. Mod Pathol
5. ^ Yoshida M, Hamanoue S, Seki M, Tanaka M, Yoshida K, Goto H, Ogawa S, Takita J, Tanaka Y (2016) Metachronous anaplastic sarcoma of the kidney and thyroid follicular carcinoma as manifestations of DICER1 abnormalities. Hum Pathol 61:205-209
6. ^ Wu MK, Cotter MB, Pears J, McDermott MB, Fabian MR, Foulkes WD, O'Sullivan MJ (2016) Tumor progression in DICER1-mutated cystic nephroma-witnessing the genesis of anaplastic sarcoma of the kidney. Hum Pathol 53:114-120
7. ^ a b Vujanić GM, Kelsey A, Perlman EJ, Sandstedt B, Beckwith JB (2007) Anaplastic sarcoma of the kidney: a clinicopathologic study of 20 cases of a new entity with polyphenotypic features. Am J Surg Pathol 31(10):1459-1468