OBJECTIVE: Epithelioid angiomyolipomas (AML) of the kidney are malignant tumors with aggressive clinical behavior. Methods The Authors report on some cases epithelioid AM recently diagnosed at their Institute to highlight the spectrum of clinical presentations. RESULTS: In all cases, renal lesions observed at computed tomography were suspicious for renal cell carcinoma (RCC). Histologically, these tumors can resemble RCC. The diagnosis of epithelioid AML was established by positive staining for melanoma and smooth muscle cell markers, and presence of perivascular epithelioid cells.Four patient presented with a renal tumor extending into the inferior vena cava to the level of the hepatic veins. Two patients developed recurrent, metastatic disease following nephrectomy. CONCLUSIONS: These tumors are distinguished from RCC by positive immunostaining for melanoma markers and smooth muscle cell markers.They resemble conventional RCC on imaging. Epithelioid AML, may be locally aggressive and metastasized.KEY WORDS: Angiomyolipoma, Epithelioid angiomyolipoma, Kidney, Renal cell carcinoma
BIETTIVO: Gli angiomyolipomi del rene sono tumori maligni con comportamento clinico aggressivo. METODI: Viene riportata una rassegna si riporta una recente di casi con AML diagnosticati nella nostra nostra istituzione al fine d ievidenziare lo spettro di presenta- zioni cliniche.RISULTATI: In tutti i casi trattati, le lesioni renali osser- vate con la tomografia computerizzata erano orientate per carcinoma renale. Istologicamente questi tumori possono assomigliare a RCC. La diagnosi di angiomiolipoma è stata stabilita da una chiazzatura positiva per melanoma e presenza di cel- lule epitelioidi . Quattro pazienti presentavano un tumo- re renale che si estendeva alla vena cava inferiore al livel- lo delle vene epatiche. Due pazienti svilupparono ricorente malattia metastatica successiva a nefrectomia. CONCLUSIONI: Questi tumori sono distinti da RCC da immunostaining positivo per marcatori di melanoma. La loro somiglianza a RCC convenzionale su imaging TC viene differenziata dalla clinica in quanto l’AML epite- lioide può essere localmente arente malattia metastatica successiva a nefrectomia. CONCLUSIONI: Questi tumori sono distinti da RCC da immunostaining positivo per marcatori di melanoma. La loro somiglianza a RCC convenzionale su imaging TC viene differenziata dalla clinica in quanto l’AML epite- lioide può essere localmente aggressivo e metastatizzante.
Clinical evalutation of epithelioid angiomyolipoma
A. Graziano;UMANA, DANILO SAVIO DOMENICO
2008-01-01
Abstract
OBJECTIVE: Epithelioid angiomyolipomas (AML) of the kidney are malignant tumors with aggressive clinical behavior. Methods The Authors report on some cases epithelioid AM recently diagnosed at their Institute to highlight the spectrum of clinical presentations. RESULTS: In all cases, renal lesions observed at computed tomography were suspicious for renal cell carcinoma (RCC). Histologically, these tumors can resemble RCC. The diagnosis of epithelioid AML was established by positive staining for melanoma and smooth muscle cell markers, and presence of perivascular epithelioid cells.Four patient presented with a renal tumor extending into the inferior vena cava to the level of the hepatic veins. Two patients developed recurrent, metastatic disease following nephrectomy. CONCLUSIONS: These tumors are distinguished from RCC by positive immunostaining for melanoma markers and smooth muscle cell markers.They resemble conventional RCC on imaging. Epithelioid AML, may be locally aggressive and metastasized.KEY WORDS: Angiomyolipoma, Epithelioid angiomyolipoma, Kidney, Renal cell carcinomaI documenti in IRIS sono protetti da copyright e tutti i diritti sono riservati, salvo diversa indicazione.