Primär renales Angiosarkom

Rüb, J.; Bauer, Sebastian; Pastor, J.; Noldus, Joachim; Palisaar, Rein-Jüri

doi:10.1007/s00120-015-3809-0

Cited by 3 publications

(1 citation statement)

References 8 publications

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“…Because of the absence of standard chemotherapy agents intended for PRA, a large variety of chemical substances have been administered. Paclitaxel-based chemotherapy, doxorubicin and the combination of ifosfamide with epirubicin, doxorubicin or anthracycline have been reported (2,5,(52)(53)(54)(55). One study reported the use of oxaliplatin with paclitaxel followed by bevacizumab, but unfortunately patients did not respond (34).…”

Section: Therapeutic Managementmentioning

confidence: 99%

Primary Angiosarcoma of the Kidney: Literature Review of a Rare Nosologic Entity

et al. 2020

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Primary angiosarcoma of the kidney is a rare tumor, hence little is known concerning its diagnostic features and therapeutic management. We conducted this survey to present a complete literature review with emphasis on clinicopathological features, diagnosis and therapy. A thorough search was conducted in MEDLINE/PubMed. All relevant studies concerning primary renal angiosarcomas in adults were thoroughly reviewed. Primary renal angiosarcoma is characterized by an overall poor prognosis, is of unknown etiology and occurs most commonly in males between 60 and 70 years old. Presence of distant metastasis at the time of diagnosis is prevalent. Histopathological examination and immunohistochemical studies are the most important diagnostic tools. Treatment options include surgery, chemotherapy, radiotherapy and immunotherapy. Conclusion: Primary renal angiosarcoma is a rare but aggressive malignancy with low response to available therapeutic regimens and dismal survival rates.Angiosarcoma is an extremely rare and aggressive malignant tumor occurring in fewer than 2% of all soft-tissue sarcomas (1). Primary angiosarcoma arises most commonly from the skin, superficial soft tissue, liver, spleen, bone, and breast (2, 3). An exceedingly infrequent location is the kidney, which accounts for 1% of the total of angiosarcomas diagnosed (4). Primary renal angiosarcoma (PRA) has an overall dismal prognosis, with an estimated average life expectancy of 6 months. It is also referred to as renal hemangiosarcoma and was first described in 1942, with few reported cases since then. Its etiology is still unknown and its predisposing factors remain ill-defined (5, 6). However, case reports show development of PRA in patients with pre-existing renal angiomyolipoma or multicystic kidney disease (7,8). White males between 60-70 years old are most commonly affected (9). Presenting symptoms in the majority of patients include flank pain, hematuria, anemia and palpable mass (3).Initial diagnostic approach involves abdominal computed tomography (CT) and magnetic resonance imaging which may reveal a solitary renal mass. Further total body scan, which is indicated for TNM staging, may unveil distant metastatic lesions in the lungs, liver, bone, spleen, abdominal lymph nodes, peritoneum, or soft tissues (3). Histopathologically, it is defined by an angiomatous structure composed of atypical proliferation of endothelial cells, with epithelioid, spindle or histiocytoid characteristics, and anastomotic vascular channels (10). Biopsy and cytological characteristics combined with a positive immunohistochemical staining for CD31, CD34, factor VIII, vimentin, and friend leukemia integration 1 (FLI1) are the gold standard methods for the definitive diagnosis of PRA (6, 10).

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Section: Therapeutic Managementmentioning

confidence: 99%

Primary Angiosarcoma of the Kidney: Literature Review of a Rare Nosologic Entity

et al. 2020

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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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Primary vascular tumours of the kidney

Omiyale¹

2021

WJCO

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Primary vascular tumours of the kidney are rare and may pose diagnostic difficulties because of their similar clinical, morphological, and immunohistochemical features. This article summarizes the clinical and pathological features of primary renal angiosarcoma and anastomosing haemangioma of the kidney including epidemiology, genetics, and prognosis. Renal anastomosing haemangiomas are benign neoplasms characterized by anastomosing capillary-sized vascular channels. These tumours are rare, with about 75 cases reported in the literature. Most anastomosing haemangiomas are found incidentally on ultrasound, computed tomography, or magnetic resonance imaging. Common symptoms include abdominal pain, haematuria, and abdominal mass. Renal anastomosing haemangiomas are characterized by recurrent mutations in GNAQ and GNA14 genes. The prognosis of anastomosing haemangioma is excellent. Primary renal angiosarcomas are malignant tumours showing endothelial differentiation. To date, 76 cases have been described in the literature. Primary renal angiosarcomas are frequently symptomatic. The clinical features of renal angiosarcomas are similar to those of renal anastomosing haemangiomas, including abdominal pain, haematuria, and abdominal mass. Angiogenesis-related genes and vascular-specific receptor tyrosine kinases such as KDR, TIE1, SNRK, TEK , and FLT1 are upregulated in angiosarcomas. Primary renal angiosarcomas are highly aggressive neoplasms with a poor prognosis despite surgical treatment, chemotherapy, radiotherapy, or targeted therapy.

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Primär renales Angiosarkom

Cited by 3 publications

References 8 publications

Primary Angiosarcoma of the Kidney: Literature Review of a Rare Nosologic Entity

Primary Angiosarcoma of the Kidney: Literature Review of a Rare Nosologic Entity

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

Primary vascular tumours of the kidney

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