Nephropathology Since 2006
   
Case 162
Diagnosis
 
     
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Diagnosis: Primary Renal Angiosarcoma

Angiosarcomas are rare comprising about 2%-3% of adult soft tissue sarcomas. They can occur anywhere in the body but the most common locations are skin, soft tissues, liver, and bones. Kidneys are involved as part of the widespread metastatic disease. Primary angiosarcoma of the kidney is very rarely reported. Most occur in patients over 50 years old (Zhang HM, et al. Primary angiosarcoma of the kidney: case analysis and literature review. Int J Clin Exp Pathol. 2014 Jun 15;7:3555-62. [PubMed link]).

Primary angiosarcoma of the kidney has a poor prognosis. Local recurrence and distant metastasis is common. Primary angiosarcoma of the kidney shares similar clinical presentation with other renal tumors and imaging does not allow for tumor specific diagnosis (Omiyale AO. Clinicopathological features of primary angiosarcoma of the kidney: a review of 62 cases. Transl Androl Urol. 2015;4(4):464-73. [PubMed link]).

Angiosarcomas have a microscopic spectrum ranging from morphologically low grade tumors demonstrating well formed vessels with mild cytologic atypia, to morphologically high grade tumors showing severe pleomorphism, mitotic activity and solid growth pattern resembling an undifferentiated pleomorphic sarcoma y distinguishing feature from benign vascular lesions is an infiltrative growth pattern. (Reisenbichler ES. Breast Angiosarcoma. In:PathologyOutlines.com [Link]).

Angiosarcomas may show epithelioid morphology, simulating carcinomas, as can be seen in some of the images in this case. Immunohistochemistry in our case was essential to demonstrate endothelial differentiation, with the absence of epithelial markers. Aggressive clinical behavior and invasion of the lesion, evidenced by imaging studies or pathology, are key to demonstrate malignancy in cases of low-grade histological lesions, in which the differential diagnosis may be benign vascular lesions and hemangioendothelioma.

See the chapter: Renal Neoplasms of our Tutorial (this chapter has only Spanish version).

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References

  • Boni A, Cochetti G, Sidoni A, Bellezza G, Lepri E, Giglio A, Turco M, Vermandois JAR, Zingaro MD, Cirocchi R, Mearini E. Primary Angiosarcoma of the Kidney: Case Report and Comprehensive Literature Review. Open Med (Wars). 2019;14:443-455. [PubMed link]
  • Darlington D, Anitha FS. Primary Renal Angiosarcoma Mimicking Renal Cell Carcinoma: A Case Report. Cureus. 2019 Jan 7;11(1):e3841. [PubMed link]
  • Heo SH, Shin SS, Kang TW, Kim GE. Primary renal angiosarcoma with extensive hemorrhage: CT and MRI findings. Int Braz J Urol. 2019;45(2):402-405. [PubMed link]
  • Samaan S, Quddus MR, Matoso A. "Man in Istanbul" Lesions of the Urinary Tract (Known Entities in an Unusual Context): Melanoma, Carcinoid Tumors, Epithelioid Angiosarcoma. Surg Pathol Clin. 2018;11(4):825-836. [PubMed link]
  • Lodhi HT, Inayat F, Munir A, Ilyas G. Primary renal angiosarcoma: a diagnostic and therapeutic challenge. BMJ Case Rep. 2018;2018. pii: bcr-2018-225484. [PubMed link]
  • Waqas M, Rahim W, Shohab D, Khawaja MA, Ali Z, Mamoon N. Primary Renal Epithelioid Angiosarcoma. J Coll Physicians Surg Pak. 2018;28(3):S66-S68. [PubMed link]
  • Omiyale AO, Carton J. Clinical and Pathologic Features of Primary Angiosarcoma of the Kidney. Curr Urol Rep. 2018;19(2):4. [PubMed link]
  • Omiyale AO. Clinicopathological features of primary angiosarcoma of the kidney: a review of 62 cases. Transl Androl Urol. 2015;4(4):464-73. [PubMed link]
  • Zhang HM, Yan Y, Luo M, Xu YF, Peng B, Zheng JH. Primary angiosarcoma of the kidney: case analysis and literature review. Int J Clin Exp Pathol. 2014 Jun 15;7:3555-62. [PubMed link]

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