Produção Nacional
Revisado por pares
Renal Angiomyolipoma With Cardiac Extension in Patient With Tuberous Sclerosis Complex
2011; Lippincott Williams & Wilkins; Volume: 124; Issue: 23 Linguagem: Inglês
10.1161/circulationaha.110.009779
ISSN1524-4539
AutoresVictor Martins Isac, Mario Renato Silva, Adenalva Lima de Souza Beck, Pedro Rincon Cintra da Cruz, Eduardo Ribeiro Carvalho, Ana Emília Borges de Azevedo, Joalbo M. Andrade,
Tópico(s)Tuberous Sclerosis Complex Research
ResumoHomeCirculationVol. 124, No. 23Renal Angiomyolipoma With Cardiac Extension in Patient With Tuberous Sclerosis Complex Free AccessBrief ReportPDF/EPUBAboutView PDFView EPUBSections ToolsAdd to favoritesDownload citationsTrack citationsPermissionsDownload Articles + Supplements ShareShare onFacebookTwitterLinked InMendeleyReddit Jump toSupplemental MaterialFree AccessBrief ReportPDF/EPUBRenal Angiomyolipoma With Cardiac Extension in Patient With Tuberous Sclerosis Complex Victor Martins Isac, MD, Mario Renato Silva, MD, Adenalva Lima de Souza Beck, MD, PhD, Pedro Rincon Cintra da Cruz, MD, Eduardo Ribeiro Carvalho, MD, Ana Emília Borges de Azevedo, MD and Joalbo Matos Andrade, MD, PhD Victor Martins IsacVictor Martins Isac From the Departments of Radiology (V.M.I., M.R.S., J.M.A.), Urology (P.R.C.C., E.R.C.), and Pathology (A.E.B.A.), Hospital of the University of Brasilia, Brasilia/DF, Brazil; and Institute of Cardiology of the Distrito Federal, Brasilia/DF, Brazil (A.L.S.B.). , Mario Renato SilvaMario Renato Silva From the Departments of Radiology (V.M.I., M.R.S., J.M.A.), Urology (P.R.C.C., E.R.C.), and Pathology (A.E.B.A.), Hospital of the University of Brasilia, Brasilia/DF, Brazil; and Institute of Cardiology of the Distrito Federal, Brasilia/DF, Brazil (A.L.S.B.). , Adenalva Lima de Souza BeckAdenalva Lima de Souza Beck From the Departments of Radiology (V.M.I., M.R.S., J.M.A.), Urology (P.R.C.C., E.R.C.), and Pathology (A.E.B.A.), Hospital of the University of Brasilia, Brasilia/DF, Brazil; and Institute of Cardiology of the Distrito Federal, Brasilia/DF, Brazil (A.L.S.B.). , Pedro Rincon Cintra da CruzPedro Rincon Cintra da Cruz From the Departments of Radiology (V.M.I., M.R.S., J.M.A.), Urology (P.R.C.C., E.R.C.), and Pathology (A.E.B.A.), Hospital of the University of Brasilia, Brasilia/DF, Brazil; and Institute of Cardiology of the Distrito Federal, Brasilia/DF, Brazil (A.L.S.B.). , Eduardo Ribeiro CarvalhoEduardo Ribeiro Carvalho From the Departments of Radiology (V.M.I., M.R.S., J.M.A.), Urology (P.R.C.C., E.R.C.), and Pathology (A.E.B.A.), Hospital of the University of Brasilia, Brasilia/DF, Brazil; and Institute of Cardiology of the Distrito Federal, Brasilia/DF, Brazil (A.L.S.B.). , Ana Emília Borges de AzevedoAna Emília Borges de Azevedo From the Departments of Radiology (V.M.I., M.R.S., J.M.A.), Urology (P.R.C.C., E.R.C.), and Pathology (A.E.B.A.), Hospital of the University of Brasilia, Brasilia/DF, Brazil; and Institute of Cardiology of the Distrito Federal, Brasilia/DF, Brazil (A.L.S.B.). and Joalbo Matos AndradeJoalbo Matos Andrade From the Departments of Radiology (V.M.I., M.R.S., J.M.A.), Urology (P.R.C.C., E.R.C.), and Pathology (A.E.B.A.), Hospital of the University of Brasilia, Brasilia/DF, Brazil; and Institute of Cardiology of the Distrito Federal, Brasilia/DF, Brazil (A.L.S.B.). Originally published6 Dec 2011https://doi.org/10.1161/CIRCULATIONAHA.110.009779Circulation. 2011;124:e736–e738A 26-year-old woman with a clinical diagnosis of tuberous sclerosis complex, presenting the classical triad of seizures, mental retardation, and facial angiofibromas and reporting tachycardia, dyspnea during moderate physical activity, and 3 episodes of syncope in the past 30 days, was admitted to our hospital.Echocardiography revealed right cardiac chambers with increased dimensions related to a large echogenic tumor with an inferior component within the inferior vena cava (Figure 1). Computed tomography of the chest and abdomen without iodinated venous contrast media depicted a large tumor arising from the right kidney with adipose tissue, extending superiorly via inferior vena cava with right cardiac chambers involvement (Figure 2). MRI of the abdomen and heart showed the same large right kidney tumor with high signal intensity in T1- and T2-weighted images, signal intensity loss in T2-weighted images with fat saturation, and enhancement after the use of venous paramagnetic contrast media (Figure 3 and Movies I, II, and III in the online-only Data Supplement).Download figureDownload PowerPointFigure 1. Echocardiography imaging. A, Transverse subcostal view showing an echogenic mass (star) in the right atrium. B, Apical 4-chamber view depicting the mass within the right atrium with extension to the right ventricle through the tricuspid valve. Transverse (C) and long-axis (D) parasternal views depicting the mass in the right ventricle (star) and in the right ventricle outflow tract, respectively.Download figureDownload PowerPointFigure 2. Computed tomography imaging without venous contrast media, axial images at the level of the right kidney (A) and at the level of the heart (B) demonstrating a fat-containing renal mass with extension to the right cardiac chambers involving the inferior vena cava showed in coronal (C) and sagittal (D) multiplanar reconstruction.Download figureDownload PowerPointFigure 3. MRI, axial T1 GRE (A), axial T2 turbo spin echo with fat saturation (B), and axial T1 GRE with gadolinium showing the renal origin of the angiomyolipoma (C). Axial double-inversion recovery images at the level of the intrahepatic inferior vena cava (D), right cardiac chambers (E), and right ventricle outflow tract (F) showing a large tumor with high signal intensity within those structures. The same sequence of images in coronal (G) and sagittal planes (H) showing the inferior and superior extension of the tumor. Coronal triple-inversion recovery (fat saturation) image (I) demonstrating loss of signal intensity of the tumor.The patient underwent surgical treatment with right nephrectomy and tumor resection. Pathology showed a angiomyolipoma with a cylindrical shape measuring 16×4×4 cm, and there were no atypical or epithelioid features seen on histological examination (Figure 4).Download figureDownload PowerPointFigure 4. Angiomyolipoma. A, Macroscopy. B, Hematoxylin and eosin 40× stain shows tumor composed of smooth muscle (box), fat (star), and thick-walled blood vessels (arrow head). Immunohistochemistry shows positivity for Desmin (C) and Melan-A (D).Renal angiomyolipoma (AML) is a benign neoplasm of the group of perivascular epithelioid cell tumors, consisting of 3 elements: smooth muscle, adipose tissue, and blood vessels. It is predominantly seen in women, and its incidence is highest in the fifth and sixth decades of life. It is well associated with tuberous sclerosis, seen in 80% of the patients. Typically, AML is confined to the kidney, but, in rare circumstances, the tumor can extend beyond the kidney.1Although renal AML is a benign lesion, it has the potential for significant sequelae. Retroperitoneal hemorrhage from the AML can lead to shock in up to 20% of the patients. In the tuberous sclerosis complex population, renal failure can occur because the patients often require multiple procedures to manage their AMLs. Finally, the extension of a renal AML into the renal vein, inferior vena cava, or right cardiac chambers is a significant point of morbidity in this disease.2 There have only been a few case reports of AML with tumor extension to the cardiac chambers.Renal AMLs occur as isolated, sporadic entities in 80% of cases, most commonly manifesting in middle-aged women. The other 20% of angiomyolipomas develop in association with tuberous sclerosis complex. Although the histological appearance of AMLs in these 2 entities is identical, the disease progression may not be. Angiomyolipomas that occur in association with tuberous sclerosis complex manifest at a younger age (31.5 versus 53.6 years, P<0.05), are likely to be larger and bilateral, and are prone to grow and need surgical treatment.3DisclosuresNone.FootnotesThe online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161/CIRCULATIONAHA.110.009779/-/DC1.Correspondence to Victor Martins Isac, MD, QMSW 5, Lote 4, Bloco D, Apartamento 123, Setor Sudoeste, Brasilia/DF, Brazil 70680-500. E-mail [email protected]com.brReferences1. Nelson CP, Sanda MG. Contemporary diagnosis and management of renal angiomyolipoma. J Urol. 2002; 168:1315–1325.CrossrefMedlineGoogle Scholar2. Bissler JJ, Kingswood JC. Renal angiomyolipoma. Kidney Int. 2004; 66:924–934.CrossrefMedlineGoogle Scholar3. Steiner MS, Goldman SM, Fishman EK, Marshall FF. The natural history of renal angiomyolipoma. J Urol. 1993; 150:1782–1786.CrossrefMedlineGoogle Scholar Previous Back to top Next FiguresReferencesRelatedDetailsCited By Navale P, Asgari M and Chen S (2015) Pigmented Perivascular Epithelioid Cell Tumor of the Skin, The American Journal of Dermatopathology, 10.1097/DAD.0000000000000320, 37:11, (866-869), Online publication date: 1-Nov-2015. Le Huu Nho R, Renard S, Maurin C, Souteyrand P and Le Treut Y (2014) Angiomyolipome rénal géant compliqué d'insuffisance cardiaque droite, Progrès en Urologie, 10.1016/j.purol.2014.02.001, 24:7, (479-481), Online publication date: 1-Jun-2014. December 6, 2011Vol 124, Issue 23 Advertisement Article InformationMetrics © 2011 American Heart Association, Inc.https://doi.org/10.1161/CIRCULATIONAHA.110.009779PMID: 22144635 Originally publishedDecember 6, 2011 PDF download Advertisement SubjectsCardiovascular SurgeryComputerized Tomography (CT)GeneticsImaging
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