Primary renal angiosarcoma

Sabharwal, Sagar; John, Nirmal Thampi; Kumar, Rajesh; Kekre, Nitin S

doi:10.4103/0970-1591.114040

Cited by 10 publications

(10 citation statements)

References 5 publications

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“…The endothelial nature of the neoplastic cells in our case was verified by diffuse positive immune-histochemical staining for the highly sensitive vascular marker CD-34 in 100% of the tumour cells [9, 35]. The raising proliferative nature of the tumour is suggested by the Ki-67 index reported to be 30% [36], 40% (9) [37], and >80% [38] respectively. In our specimen, the Ki-67 index was about 50%, and we now confirm it to indicate a high aggressiveness of this tumour.…”

Section: Discussionsupporting

confidence: 55%

Primary angiosarcoma of the kidney: case report and comprehensive literature review

et al. 2019

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AbstractPurposeAngiosarcoma (AS) is an aggressive malignant tumour of endothelial origin, most frequently compelling the skin. The kidney is a rare site of primary occurrence of AS with a quite challenging diagnosis and poor prognosis, mainly due to its raw metastasising power. We described our first case of AS treatment. A comprehensive literature review in this field is also carried out.Patients and methodsWe report the case of a 56-years-old man with radiological appearance of an 11 cm poor vascularised left renal mass. The pre-operative imaging ruled out distant metastases and inferior vena cava thrombosis. Thus, after radioembolization, we performed a transperitoneal open radical nephrectomy with regional lymphadenectomy with the removal of the middle portion of the ileo-psoas muscle, followed by two lines of adjuvant chemotherapy. We analysed all the papers concerning primary kidney AS until December 2018.ResultsEstimated intraoperative blood loss was 200 mL with an operative time of 100 minutes. No postoperative complications were recorded, with the patient discharge in the 8th postoperative day. The pathological assessment showed a pT3a N0, M0 tumour compatible with AS of the kidney. An immune-histochemical study showed a vascular positive for CD31, CD34, Fli-1 with a Ki-67/Mib 1 of 50%. One month after surgery the patient began adjuvant chemotherapy with gemcitabine. After 45 days from surgery, a Computed Tomography scan showed lung and liver metastases with disease relapse in the left kidney lodge. The response to the first-line treatment was excellent, unlike the second-line, encountering a chemo-refractory disease. The cancer-specific survival was of 15 months. We included 66 cases in our review.ConclusionThis is the first case of treatment of kidney AS performed at our Institute. Our findings added new information about the unclear biology and progression of this subset of tumours, demonstrating an abysmal prognosis.

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Section: Discussionsupporting

confidence: 55%

Primary angiosarcoma of the kidney: case report and comprehensive literature review

et al. 2019

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“…Diagnosis is only possible after immunohistochemical staining. The most commonly used markers are CD-31, CD-34 which are endothelial adhesion molecule and a human hematopoietic progenitor cell antigen [ 11 , 12 ].…”

Section: Discussionmentioning

confidence: 99%

Primary perirenal angiosarcoma: A rare presentation of a perirenal mass

Samadi

Arellano

2019

Radiology Case Reports

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“…Tumors measuring <5 cm in diameter confer an improved prognosis compared with those >5 cm (11,12). The five-year survival rates for tumors >5 and <5 cm are 13 and 32%, respectively (9).…”

Section: Discussionmentioning

confidence: 99%

Primary renal angiosarcoma with progressive clinical course despite surgical and adjuvant treatment: A case report

et al. 2015

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Abstract. Angiosarcoma is an extremely rare, high-grade malignancy, which accounts for <2% of all soft-tissue sarcomas. Cases of primary renal angiosarcoma represent 1% of these. Angiosarcomas involving the kidney usually originate from metastatic skin lesions or primary visceral lesions and most often occur in the sixth and seventh decades of life. The present study describes a case of primary renal angiosarcoma that presented as a large right-sided renal mass with symptoms of flank pain. Despite surgical removal of the tumor, recurrent disease with associated lung metastases was identified at the surgical site following adjuvant chemotherapy. The patient succumbed to the disease 13 months after the diagnosis.

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Primary renal angiosarcoma

Abstract: Primary angiosarcoma of the kidney is a rare tumor with only a few case reports in the literature. Management is not standardized and the prognosis is poor. However, clinicians need to be aware of this uncommon entity.

Cited by 10 publications

References 5 publications

Primary angiosarcoma of the kidney: case report and comprehensive literature review

Primary angiosarcoma of the kidney: case report and comprehensive literature review

Primary perirenal angiosarcoma: A rare presentation of a perirenal mass

Primary renal angiosarcoma with progressive clinical course despite surgical and adjuvant treatment: A case report

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