Ayo O Omiyale1, James Carton2. 1. Department of Cellular Pathology, Imperial College Healthcare NHS Trust, London, UK. ayodeji.omiyale@nhs.net. 2. Department of Cellular Pathology, Imperial College Healthcare NHS Trust, London, UK.
Abstract
PURPOSE OF REVIEW: Primary angiosarcoma of the kidney is extremely rare; hence, relatively little is known regarding its clinicopathologic features and prognosis. Herein, we review the literature on primary renal angiosarcoma with emphasis on the clinical and pathologic features. RECENT FINDINGS: Approximately 64 cases have been reported in the literature, and most cases occur in the 6th-7th decade with a strong male predominance. The aetiology is unknown. Patients present with flank pain, haematuria, abdominal mass and weight loss. A considerable number of patients develop metastatic disease at diagnosis or shortly afterwards. Grossly, the tumour comprises ill-defined haemorrhagic spongy masses often with necrosis. Microscopically, the tumour is composed of anastomosing capillary-sized vessels which are lined by malignant endothelial cells. The mainstay of treatment is surgery followed by radiation therapy with or without chemotherapy. Renal angiosarcomas are highly aggressive tumours with dismal outcome, and they must be distinguished from morphologically similar lesions of the kidney.
PURPOSE OF REVIEW: Primary angiosarcoma of the kidney is extremely rare; hence, relatively little is known regarding its clinicopathologic features and prognosis. Herein, we review the literature on primary renal angiosarcoma with emphasis on the clinical and pathologic features. RECENT FINDINGS: Approximately 64 cases have been reported in the literature, and most cases occur in the 6th-7th decade with a strong male predominance. The aetiology is unknown. Patients present with flank pain, haematuria, abdominal mass and weight loss. A considerable number of patients develop metastatic disease at diagnosis or shortly afterwards. Grossly, the tumour comprises ill-defined haemorrhagic spongy masses often with necrosis. Microscopically, the tumour is composed of anastomosing capillary-sized vessels which are lined by malignant endothelial cells. The mainstay of treatment is surgery followed by radiation therapy with or without chemotherapy. Renal angiosarcomas are highly aggressive tumours with dismal outcome, and they must be distinguished from morphologically similar lesions of the kidney.
Authors: Cristina R Antonescu; Akihiko Yoshida; Tianhuo Guo; Ning-En Chang; Lei Zhang; Narasimhan P Agaram; Li-Xuan Qin; Murray F Brennan; Samuel Singer; Robert G Maki Journal: Cancer Res Date: 2009-09-01 Impact factor: 12.701
Authors: Sam Behjati; Patrick S Tarpey; Helen Sheldon; Inigo Martincorena; Peter Van Loo; Gunes Gundem; David C Wedge; Manasa Ramakrishna; Susanna L Cooke; Nischalan Pillay; Hans Kristian M Vollan; Elli Papaemmanuil; Hans Koss; Tom D Bunney; Claire Hardy; Olivia R Joseph; Sancha Martin; Laura Mudie; Adam Butler; Jon W Teague; Meena Patil; Graham Steers; Yu Cao; Curtis Gumbs; Davis Ingram; Alexander J Lazar; Latasha Little; Harshad Mahadeshwar; Alexei Protopopov; Ghadah A Al Sannaa; Sahil Seth; Xingzhi Song; Jiabin Tang; Jianhua Zhang; Vinod Ravi; Keila E Torres; Bhavisha Khatri; Dina Halai; Ioannis Roxanis; Daniel Baumhoer; Roberto Tirabosco; M Fernanda Amary; Chris Boshoff; Ultan McDermott; Matilda Katan; Michael R Stratton; P Andrew Futreal; Adrienne M Flanagan; Adrian Harris; Peter J Campbell Journal: Nat Genet Date: 2014-03-16 Impact factor: 38.330