Introduction. Angiomyolipoma is a common benign renal tumor. It is associated with Tuberous Sclerosis Complex (TSC) in 20% of patients. Angiomyolipomas are classically multiple, bilateral, and growing; they may lead to complications such as Wunderlich syndrome or, in rare cases, to venous extension. Observation. a 74-year-old woman with TSC presented with an angiomyolipoma of the right kidney with inferior vena cava (IVC) fatty thrombus. She underwent partial nephrectomy and thrombectomy. After a 7-year follow-up there was no evidence of recurrence or metastasis and her renal function was preserved. Review of Literature. It is the 44th reported angiomyolipoma associated with IVC thrombus. The mean size of angiomyolipomas was 86.1？mm and 67.4% of patients were symptomatic. Pulmonary embolism was found in 6 patients. There were 2 cases of recurrence/metastatic outcome after radical nephrectomy and thrombectomy. They were associated with epithelioid form. The mean size of epithelioid tumors was significantly bigger than in classical angiomyolipomas (127.1？mm versus 82.6？mm, ). With a median follow-up of 12 months, 91.3% of patients were recurrence and metastasis free, with 3 cases of nephron sparing surgery. Conclusion. Nephron sparing surgery for angiomyolipoma with IVC fatty thrombus can be safely performed in TSC, even in sporadic angiomyolipoma. 1. Introduction Angiomyolipomas (AMLs) represent 0.3% of all renal tumors. The sex ratio is 4 men for 11 women. Most of the time, AMLs are sporadic but in 20% of patients AMLs are associated with Tuberous Sclerosis Complex. Thus they are classically multiple, bilateral, and growing [1]. They may become symptomatic and may require active management which has to be as conservative as possible. Many cases of inferior vena cava thrombus associated with AML have been reported [2–43]. We report a new clinical case of AML with inferior vena cava thrombus in a TSC patient that raises the question of the best surgical approach regarding the necessity of preserving renal function. 2. Materials and Methods 2.1. Observation A 74-year-old woman with tuberous sclerosis and multiple bilateral AML who had undergone partial polar superior nephrectomy for a renal cell carcinoma in 1990 came after 7 years of surveillance with the evidence of an inferior vena cava thrombus developed from the right renal vein (Figure 1). Figure 1: Sonography of the IVC thrombus originated from the right renal vein. Computerized tomography showed multiple renal tumors with spontaneous density inferior to—20 Hounsfield Units (HU) without contrast
References
[1]
J. N. Eble, “Angiomyolipoma of kidney,” Seminars in Diagnostic Pathology, vol. 15, no. 1, pp. 21–40, 1998.
[2]
R. Kutcher, R. Rosenblatt, and S. M. Mitsudo, “Renal angiomyolipoma with sonographic demonstration of extension into the inferior vena cava,” Radiology, vol. 143, no. 3, pp. 755–756, 1982.
[3]
R. E. Brantley, J. W. Mashni, and R. E. Bethards, “Computerized tomographic demonstration of inferior vena caval tumor thrombus from renal angiomyolipoma,” Journal of Urology, vol. 133, no. 5, pp. 836–837, 1985.
[4]
D. M. Rothenberg, T. D. Brandt, and I. D'Cruz, “Computed tomography of renal angiomyolipoma presenting as right atrial mass,” Journal of Computer Assisted Tomography, vol. 10, no. 6, pp. 1054–1056, 1986.
[5]
F. Camunez, J. Lafuente, R. Robledo et al., “CT demonstration of extension of renal angiomyolipoma into the inferior vena cava in a patient with tuberous sclerosis,” Urologic Radiology, vol. 9, no. 3, pp. 152–154, 1987.
[6]
A. M. Arenson, R. T. Graham, P. Shaw, J. Srigley, and S. Herschorn, “Angiomyolipoma of the kidney extending into the inferior vena cava: sonographic and CT findings,” American Journal of Roentgenology, vol. 151, no. 6, pp. 1159–1161, 1988.
[7]
T. Umeyama, Y. Saitoh, Y. Tomaru, and K. Kitaura, “Bilateral renal angiomyolipoma associated with bilateral renal vein and inferior vena caval thrombi,” Journal of Urology, vol. 148, no. 6, pp. 1885–1887, 1992.
[8]
D. B. Reiff and J. Dow, “Case report: invasive renal angiomyolipoma—sonographic and CT features,” Clinical Radiology, vol. 48, no. 4, pp. 283–285, 1993.
[9]
N. Honda, K. Matsuoka, K. Fujimoto et al., “Renal angiomyolipoma extending into inferior vena cava: MR demonstration,” Journal of Computer Assisted Tomography, vol. 17, no. 1, pp. 161–162, 1993.
[10]
S. B. Morris, S. J. Hampson, P. Jackson, T. Treasure, and R. J. Shearer, “Surgical management of an angiomyolipoma extending into the inferior vena cava,” British Journal of Urology, vol. 74, no. 3, pp. 383–385, 1994.
[11]
G. Moulin, J.-F. Berger, C. Chagnaud, P. Piquet, and J.-M. Bartoli, “Imaging of fat thrombus in the inferior vena cava originating from an angiomyolipoma,” CardioVascular and Interventional Radiology, vol. 17, no. 3, pp. 152–154, 1994.
[12]
R. A. Leder, “Genitourinary case of the day. Angiomyolipoma of the kidney with fat thrombus in the inferior vena cava,” American Journal of Roentgenology, vol. 165, no. 1, pp. 198–199, 1995.
[13]
H. Hibi, M. Takashi, Y. Yamada, M. Yamamoto, and T. Shimoji, “Angiomyolipoma of the kidney with extension into the inferior vena cava,” International Journal of Urology, vol. 2, no. 5, pp. 332–335, 1995.
[14]
J. Baert, B. Vandamme, R. Sciot, R. Oyen, H. van Poppel, and L. Baert, “Benign angiomyolipoma involving the renal vein and vena cava as a tumor thrombus: case report,” Journal of Urology, vol. 153, no. 4, pp. 1205–1207, 1995.
[15]
G. Cittadini Jr., F. P. Mucelli, F. M. Danza, L. E. Derchi, and R. S. P. Mucelli, “‘Aggressive’ renal angiomyolipoma,” Acta Radiologica, vol. 37, no. 6, pp. 927–932, 1996.
[16]
J. Rubio-Briones, J. P. Redorta, J. S. Bayarri et al., “Incidentally detected renal angiomyolipoma with tumour thrombus into the inferior vena cava,” Scandinavian Journal of Urology and Nephrology, vol. 31, no. 2, pp. 189–191, 1997.
[17]
M. R. Bernstein, S. B. Malkowicz, E. S. Siegelman, M. Acker, J. E. Tomaszewski, and A. J. Wein, “Progressive angiomyolipoma with inferior vena cava tumor thrombus,” Urology, vol. 50, no. 6, pp. 975–977, 1997.
[18]
A. Gotoh, K. Gohji, M. Fujisawa et al., “Renal angiomyolipoma associated with inferior vena caval tumour thrombus,” British Journal of Urology, vol. 81, no. 5, pp. 773–774, 1998.
[19]
A. P. Christiano, X. Yang, and G. S. Gerber, “Malignant transformation of renal angiomyolipoma,” Journal of Urology, vol. 161, no. 6, pp. 1900–1901, 1999.
[20]
H. Ito, S. Nakashima, H. Toma, and T. Misaki, “Renal angiomyolipoma associated with inferior vena caval and right atrial thrombus,” Journal of Urology, vol. 162, no. 4, pp. 1371–1372, 1999.
[21]
M. I. Davydov, V. B. Matveev, A. B. Lukianchenko, B. V. Kudashev, and N. N. Petrovichev, “Renal angiomyolipoma extending into the right atrium,” Urologia Internationalis, vol. 67, no. 2, pp. 168–169, 2001.
[22]
S. S. Wilson, P. E. Clark, and J. P. Stein, “Angiomyolipoma with vena caval extension,” Urology, vol. 60, no. 4, pp. 695–696, 2002.
[23]
L. Schips, M. Ratschek, G. Gallé et al., “Angiomyolipoma with a caval thrombus,” Urologia Internationalis, vol. 70, no. 4, pp. 332–334, 2003.
[24]
X. Gamé, M. Soulié, S. Moussouni et al., “Renal angiomyolipoma associated with rapid enlargment and inferior vena caval tumor thrombus,” Journal of Urology, vol. 170, no. 3, pp. 918–919, 2003.
[25]
A. H. M. M. Islam, T. Ehara, H. Kato, M. Hayama, T. Kashiwabara, and O. Nishizawa, “Angiomyolipoma of kidney involving the inferior vena cava,” International Journal of Urology, vol. 11, no. 10, pp. 897–902, 2004.
[26]
T. Haritharan, S. Sritharan, and S. Bhimji, “Renal angiomyolipoma with inferior vena caval involvement,” Medical Journal of Malaysia, vol. 61, no. 4, pp. 493–495, 2006.
[27]
Y. Akcali, O. I. Karahan, G. Kahriman, H. Ceyran, and S. Balkanli, “Angiomyolipoma with cavoatrial extension,” European Urology, vol. 50, no. 3, pp. 605–606, 2006.
[28]
H. K. Park, S. Zhang, M. K. K. Wong, and H. L. Kim, “Clinical presentation of epithelioid angiomyolipoma,” International Journal of Urology, vol. 14, no. 1, pp. 21–25, 2007.
[29]
G. R. Schade, O. N. Gofrit, and K. C. Zorn, “Renal angiomyolipoma with intravascular extension into the inferior vena cava: a case report and review of the literature,” The Canadian Journal of Urology, vol. 15, no. 2, pp. 4012–4015, 2008.
[30]
D. Ban, S. Yamamoto, H. Kuno et al., “A case of huge colon carcinoma and right renal angiomyolipoma accompanied by proximal deep venous thrombosis, pulmonary embolism and tumor thrombus in the renal vein,” Japanese Journal of Clinical Oncology, vol. 38, no. 10, pp. 710–714, 2008.
[31]
S. M. Moudouni, M. Tligui, M. Sibony et al., “Malignant epithelioid renal angiomyolipoma involving the inferior vena cava in a patient with tuberous sclerosis,” Urologia Internationalis, vol. 80, no. 1, pp. 102–104, 2008.
[32]
M. W. Christian and T. D. Moon, “Renal angiomyolipoma with inferior vena caval thrombus in a 32-year-old male,” Indian Journal of Urology, vol. 25, no. 4, pp. 529–530, 2009.
[33]
X. Durand, R. Renard-Penna, E. Comperat, M. O. Bitker, and F. Richard, “Renal angiomyolipoma associated with inferior vena cava thrombus,” Case Reports in Medicine, vol. 2009, Article ID 789078, 3 pages, 2009.
[34]
Y. S. Tan, K. H. Yip, P. H. Tan, and W. S. Cheng, “A right renal angiomyolipoma with IVC thrombus and pulmonary embolism,” International Urology and Nephrology, vol. 42, no. 2, pp. 305–308, 2010.
[35]
C. Govednik-Horny and M. Atkins, “Angiomyolipoma with vascular invasion during pregnancy,” Annals of Vascular Surgery, vol. 25, no. 8, pp. 1138.e9–1138.e13, 2011.
[36]
J. Lopater, O. Hartung, F. Bretelle, and C. Bastide, “Management of angiomyolipoma vena cava thrombus during pregnancy,” Obstetrics and Gynecology, vol. 117, no. 2, pp. 440–443, 2011.
[37]
V. Mittal, B. S. Aulakh, and G. Daga, “Benign renal angiomyolipoma with inferior vena cava thrombosis,” Urology, vol. 77, no. 6, pp. 1503–1506, 2011.
[38]
C. Grant, J. M. Lacy, and S. E. Strup, “A 22-year-old female with invasive epithelioid angiomyolipoma and tumor thrombus into the inferior vena cava: case report and literature review,” Case Reports in Urology, vol. 2013, Article ID 730369, 3 pages, 2013.
[39]
X. Li, Q. Li, Y. Miao et al., “A case of renal angiomyolipoma with intracardiac extension and asymptomatic pulmonary embolism,” International Journal of Clinical and Experimental Pathology, vol. 6, no. 6, pp. 1180–1186, 2013.
[40]
H.-M. Li, L.-R. Yeh, and K. Lu, “Renal angiomyolipoma with coexistent hemorrhagic aneurysm formation and fatty thrombus in inferior vena cava: a rare presentation,” Abdominal Imaging, vol. 38, no. 1, pp. 180–183, 2013.
[41]
S. Fernandez-Pello, I. G. Rodriguez, L. R. Villamil et al., “Laparoscopic management of right renal angiomyolipoma with involvement of the inferior vena cava: case report and review of the literature,” Scandinavian Journal of Urology, vol. 47, no. 4, pp. 340–344, 2013.
[42]
Y. Nouira, Y. Kallel, M. Gargouri et al., “Renal angiomyolipoma with Fatty thrombus extending to the right atrium: an exceptional presentation,” Case Reports in Urology, vol. 2013, Article ID 120383, 3 pages, 2013.
[43]
C. K. Sandstrom, J. Pugsley, and L. M. Mitsumori, “Renal angiomyolipoma with nontraumatic pulmonary fat embolus,” American Journal of Roentgenology, vol. 192, no. 6, pp. W275–W276, 2009.
[44]
H. P. H. Neumann, G. Schwarzkopf, and E. P. Henske, “Renal angiomyolipomas, cysts, and cancer in tuberous sclerosis complex,” Seminars in Pediatric Neurology, vol. 5, no. 4, pp. 269–275, 1998.
[45]
N. Nese, G. Martignoni, C. D. Fletcher et al., “Pure epithelioid PEComas (so-called epithelioid angiomyolipoma) of the kidney: a clinicopathologic study of 41 cases: detailed assessment of morphology and risk stratification,” American Journal of Surgical Pathology, vol. 35, no. 2, pp. 161–176, 2011.
[46]
J. N. Eble, M. B. Amin, and R. H. Young, “Epithelioid angiomyolipoma of the kidney: a report of five cases with a prominent and diagnostically confusing epithelioid smooth muscle component,” American Journal of Surgical Pathology, vol. 21, no. 10, pp. 1123–1130, 1997.
[47]
M. Pea, F. Bonetti, G. Martignoni et al., “Apparent renal cell carcinomas in tuberous sclerosis are heterogeneous: the identification of malignant epithelioid angiomyolipoma,” American Journal of Surgical Pathology, vol. 22, no. 2, pp. 180–187, 1998.
[48]
E. Wyluda, G. Baquero, N. Lamparella, C. Abendroth, and J. Drabick, “Fatal malignant metastastic epithelioid angiomyolipoma presenting in a young woman: case report and review of the literature,” Rare Tumors, vol. 5, no. 3, article e46, 2013.
[49]
O. Hélénon, S. Merran, F. Paraf et al., “Unusual fat-containing tumors of the kidney: a diagnostic dilemma,” Radiographics, vol. 17, no. 1, pp. 129–144, 1997.
[50]
M. Jinzaki, A. Tanimoto, Y. Narimatsu et al., “Angiomyolipoma: imaging findings in lesions with minimal fat,” Radiology, vol. 205, no. 2, pp. 497–502, 1997.
[51]
M. S. Steiner, S. M. Goldman, E. K. Fishman, and F. F. Marshall, “The natural history of renal angiomyolipoma,” Journal of Urology, vol. 150, no. 6, pp. 1782–1786, 1993.
[52]
C. P. Nelson and M. G. Sanda, “Contemporary diagnosis and management of renal angiomyolipoma,” Journal of Urology, vol. 168, no. 4, pp. 1315–1325, 2002.
[53]
S. Fazeli-Matin and A. C. Novick, “Nephron-sparing surgery for renal angiomyolipoma,” Urology, vol. 52, no. 4, pp. 577–583, 1998.
[54]
S. A. Boorjian, I. Frank, B. Inman et al., “The role of partial nephrectomy for the management of sporadic renal angiomyolipoma,” Urology, vol. 70, no. 6, pp. 1064–1068, 2007.
[55]
A. Minervini, G. Giubilei, L. Masieri, F. Lanzi, S. Serni, and M. Carini, “Simple enucleation for the treatment of renal angiomyolipoma,” BJU International, vol. 99, no. 4, pp. 887–891, 2007.
[56]
T. Harabayashi, N. Shinohara, H. Katano, K. Nonomura, T. Shimizu, and T. Koyanagi, “Management of renal angiomyolipomas associated with tuberous sclerosis complex,” Journal of Urology, vol. 171, no. 1, pp. 102–105, 2004.
[57]
N. Shinohara, M. Kotegawa, Y. Kiyohara et al., “An autopsy case of pulmonary embolism due to renal angiomyolipoma in an elderly woman,” Nippon Ronen Igakkai Zasshi, vol. 36, no. 6, pp. 420–424, 1999.
[58]
E. H. Kim, S. Jain, B. M. Benway, and R. S. Figenshau, “Partial nephrectomy in two patients with known T3a tumours involving the renal vein,” BJU International, vol. 109, no. 9, pp. 1345–1348, 2012.
[59]
S. B. Kolla, C. Ercole, P. E. Spiess, J. M. Pow-Sang, and W. J. Sexton, “Nephron-sparing surgery for pathological stage T3b renal cell carcinoma confined to the renal vein,” BJU International, vol. 106, no. 10, pp. 1494–1498, 2010.
[60]
S. L. Woldu, L. J. Barlow, T. Patel, G. W. Hruby, M. C. Benson, and J. M. McKiernan, “Single institutional experience with nephron-sparing surgery for pathologic stage T3bNxM0 renal cell carcinoma confined to the renal vein,” Urology, vol. 76, no. 3, pp. 639–642, 2010.
