Angiomiolipoma hepático epitelioide: reporte de un caso / Hepatic epithelioid angiomyolipoma: Case Report

Angiomyolipomas (AML) are mesenchymal tumors belonging to the group of perivascular epithelioid cell tumors, which, rarely, can display a malignant behavior. They are composed of adipose tissue, vessels, and muscle tissue in different proportions, and constitute a differential diagnosis for other focal liver lesions. We report a 34-year-old woman in whom a focal hepatic lesion was discovered incidentally. The pathology report of an ultrasound guided biopsy informed an epithelioid angiomyolipoma, a rare variant of these lesions. During ten years of imaging follow, the size and features of the lesion has not changed. The patient rejected a surgical excision.

Renal angiomyolipoma with inferior vena cava and right atrial embolism: A case report and literature review / 中南大学学报(医学版)

Renal angiomyolipoma (AML) with renal vein, inferior vena cava (IVC), and right atrial embolism is a rare solid tumor, whose etiology and pathogenesis are still unclear. Moreover, it is often misdiagnosed. One patient with renal AML complicated with renal vein, IVC, and right atrial embolism was admitted to the Second Xiangya Hospital of Central South University, who was a 35-year-old female, without any previous medical history, presented with right low back pain for more than 3 years. Computed tomography (CT) scan showed irregular lobulated fatty density mass in the right kidney, renal vein, IVC, and right atrium. The contrast-enhanced scan showed no enhancement of fat components at each phase and mild enhancement of solid components. Radical resection of the right kidney and removal of tumor thrombus were performed, and there was no recurrence 1 year after the operation. It is rare for renal AML to grow along the renal vein, IVC, and extend to the right atrium. Imaging examination is extremely important, and the CT findings of this case are characteristic, but the diagnosis eventually depends on pathological and immunohistochemical examinations.

Renal angiomyolipoma

SUMMARY INTRODUCTION Angiomyolipoma is one of the most common benign solid renal tumors. We investigated the characteristics of renal angiomyolipomas and the clinical outcomes of patients in the last thirteen years. METHODS The medical records of the patients who underwent nephrectomy were reviewed retrospectively from July 2005 to May 2018. The laboratory data, radiology, and pathology reports were recorded. Patients diagnosed with angiomyolipoma were included in the study. RESULTS A total of 28 patients were included in the study, eight of them male. The mean age of the patients was 55.89+14.49 years. The patients were treated with open and laparoscopic techniques. Partial nephrectomy was performed in 12 patients(42.85%). After pathological examination, 23 patients were diagnosed as fat rich, four patients as fat poor, and one as epithelioid angiomyolipoma. There were no recurrences in the follow-up 91.21+48.31 months. CONCLUSION Angiomyolipoma is a rare renal tumor in daily urology practice. Clinicians must be aware of its complications and manage patients well.

RESUMO OBJETIVO O angiomiolipoma é um dos tumores renais benignos sólidos mais comuns. Investigamos as características dos angiomiolipomas renais e os desfechos clínicos dos pacientes nos últimos treze anos. MÉTODOS Os prontuários dos pacientes, para os quais a nefrectomia foi realizada, foram revisados retrospectivamente de 2008 a 2018. Os dados laboratoriais, relatórios de radiologia e patologia foram registrados. Os pacientes diagnosticados como angiomiolipoma foram incluídos no estudo. RESULTADOS Vinte e oito pacientes foram incluídos no estudo, oito deles do sexo masculino. A média de idade dos pacientes foi de 55,89 + 14,49 anos. Os pacientes foram tratados com técnicas abertas e laparoscópicas. Nefrectomia parcial foi realizada em 12 pacientes (42,85%). Depois de exame patológico, 23 pacientes foram diagnosticados como ricos em gordura, quatro pacientes como gordurosos e um paciente como angiomiolipoma epitelioide. Nenhum paciente teve recorrências no seguimento. CONCLUSÕES O angiomiolipoma é um tumor renal raro na prática urológica diária. Os médicos devem estar cientes das complicações e gerenciar bem os pacientes.

Masas renales pequeñas: predictores de malignidad en una serie de 10 años / Small renal masses: analysis of 152 cases

Background: Small renal masses (SRM) are defined as complex organ-confined solid or cystic lesions < 4 cm. Up to 20% of these can be benign. A conservative management with active surveillance can be done in some patients. However, it is difficult to identify patients with a higher risk of malignancy. Aim: To characterize the clinical, radiological and histopathological aspects of patients with SRM, analyzing predictive factors for tumor aggressiveness. Material and Methods: Retrospective analysis of a cohort of patients undergoing partial or total nephrectomy for renal tumors between 2006 and 2016. All tumors of 4 cm or less were included. Four histological groups were defined: benign, favorable, intermediate and unfavorable. Two categories of risk were also defined: low and high. Preoperative clinical and radiological variables of these patients were analyzed. Results: Data of 152 patients were analyzed. Six percent had a benign histology, and the majority was of intermediate risk (74%). According to histological type, clear cell carcinoma was the most common type (74%). Three percent were benign angiomyolipomas. No malignancy predictive variable was identified. Conclusions: In these patients, the percentage of benign SRM was low. No variable that could predict the presence of a benign or malignant lesion in the definitive biopsy was identified.

Comparison of safety and effectiveness between retroperitoneal laparoscopic tumor aspiration and laparoscopic partial nephrectomy in the treatment of renal angiomyolipoma / 北京大学学报(医学版)

OBJECTIVE@#To compare the safety and treatment effectiveness of retroperitoneal laparoscopic tumor aspiration and laparoscopic partial nephrectomy (LPN) in the treatment of renal angiomyolipoma (RAML).@*METHODS@#We retrospectively reviewed the clinical data of patients with pathologically confirmed RAML who received operation between August 2010 and August 2016 in the Department of Urology, Peking University First Hospital. Among them, a series of 121 patients were included in this trial according to the inclusion criteria, of which 74 cases could be collected and followed-up effectively. Based on the detailed surgical route, the 74 patients were divided into groups A and B: group A, which underwent retroperitoneal laparoscopic tumor aspiration, included 43 cases; group B, which received retroperitoneal LPN, included 31 cases. Patient demographics, intraoperative variables and postoperative outcomes were reported and compared between the groups.@*RESULTS@#No statistical difference was detected in both groups before the treatment. Intraoperatively, the mean estimated blood loss was 48.7 mL in group A and 102.9 mL in group B, and the mean operative time was 70.1 min (21.2 min of warm ischemia time included) in group A and 103.6 min (28.5 min of warm ischemia time included) in group B, which were both statistically different. In group A, no complications occurred and yet 2 complications of transfusion and 1 complication of urine leakage were discovered in group B, although all finally recovered only with conservative treatment. A statistical difference was observed in the complication rates. Post-operatively, the mean serum creatinine level was 1.13 mg/dL in group A, and the level was 1.08 mg/dL in group B, in which no evident difference was detected. In a mean 52.6-months' follow-up, a recurrence of 3 cases in group A (7.0%) and a recurrence of 2 cases in group B (6.5%) were reported. No evident difference was also detected between the groups in the tumor recurrence rates.@*CONCLUSION@#Due to the improvements in the intraoperative blood loss and operative time, retroperitoneal laparoscopic tumor aspiration may be provided with more potential advantages in the safety, also with equal efficacy of lower tumor recurrence rates when compared with the traditional retroperitoneal LPN in the treatment of RAML.

PEComa (neoplasia de células epiteliodes perivasculares) asociado con cáncer papilar de tiroides bilateral sincrónico / PEComa (Perivascular Epithelioid Cell Neoplasms) associated with synchronous bilateral papillary thyroid cancer

Resumen Introducción: Los tumores de células epitelioides perivasculares (PEComas) son neoplasias poco frecuentes de origen mesenquimal. Estos incluyen el angiomiolipoma epitelioide (EAML), una neoplasia con potencial maligno y cuya presentación simultánea con cáncer de tiroides es bastante rara. Presentación del caso: Hombre de 40 años con masa de crecimiento rápido en relación con polo inferior derecho de tiroides e infiltración de tráquea. La TAC abdominopélvica contrastada muestra múltiples masas renales con masa dominante en polo superior izquierdo. El estudio histopatológico de tiroides evidenció una lesión en lóbulo derecho correspondiente a cáncer papilar de tiroides (PTC) de variante de células altas y una segunda neoplasia en el lóbulo contralateral correspondiente a PTC moderadamente diferenciado de variante clásica. En riñón se encontró una lesión correspondiente a EAML, con células de aspecto epitelioide equivalentes al 40%. Discusión: El presente artículo corresponde al primero en la literatura en describir la presentación simultánea de EAML con PTC bilateral sincrónico (SBiPTC). Se describe una vía molecular común, que corresponde a la vía TSC/mTOR, la prevalencia de SBiPTC y los marcadores inmunohistoquímicos para diagnóstico de EAML.

Abstract Introduction: Perivascular Epithelioid Cell Neoplasms (PEComas) are uncommon tumors of mesenchymal origin. These ones include the epithelioid angiomyolipoma (EAML), a tumor with malignant potential and whose simultaneous presentation with thyroid cancer is quite rare. Presentation of case: Forty years old man with fast-growing mass in relation to lower right thyroid pole and tracheal infiltration. Abdominopelvic contrast-enhanced CT shows multiple renal masses with dominant mass in the left upper pole. Thyroid histopathology showed a lesion in right lobe corresponding to tall cell variant of papillary thyroid cancer (PTC) and a second neoplasm in the contralateral lobe corresponding to classical variant of moderately differentiated PTC. In the kidney was found a tumor corresponding to EAML with epithelioid aspect cells equivalent to 40%. Discussion: This article corresponds to the first in the literature to describe the simultaneous presentation of EAML with synchronous bilateral PTC (SBiPTC) and its possible association. A common molecular pathway corresponding to the TSC/mTOR pathway is described, as well as SBiPTC prevalence and immunohistochemical markers for EAML diagnosis.

Tratamiento quirúrgico conservador en paciente monorrenal con esclerosis tuberosa y angiomiolipoma complicado: presentación de caso y revisión bibliográfica / Conservative surgical treatment in monorrenal patient with sclerosis and complicated tuberous angiomyolipom: case report and literature review

Los angiomiolipomas renales son formaciones renales que se presentan de forma aislada o asociadas con otras patologías como esclerosis tuberosa o enfermedad de Von Hippel Lindau. Los angiomiolipomas renales se pueden presentar clínicamente con un shock hipovolémico por lesión de uno de sus vasos o con dolor abdominal por efecto masa debido a su tamaño. La resolución de los angiomiolipomas puede ser de manera programada o de urgencia, siendo las vías elegidas la nefrectomía parcial o la embolización arterial selectiva, dependiendo siempre de los recursos que se tengan y la experiencia del equipo quirúrgico.

Renal angiomyolipomas are kidney formations presented in isolation or associated with other diseases such as tuberous sclerosis or Von Hippel Lindau disease. Renal angiomyolipoma may present clinically with hypovolemic shock due to injury of one of its vessels or with abdominal pain due to mass effect because of its size. Angiomyolipomas can be resolved on scheduled basis or emergency, where the chosen ways are partial nephrectomy or selective arterial embolization, always depending on the resources you count on and the experience of the surgical team.

Nefrectomía radical laparoscópica en angiomiolipoma renal con invasión de vena renal / Laparoscopic radical nephrectomy of angiomyolipoma with renal vein involvement

Background: Angiomyolipomas associated with tuberous sclerosis may invade the renal vein and generate intramural thrombi. Case report: We report a 36-years-old woman, consulting for left flank pain. CT scan showed a large tumor in the left kidney consistent with the diagnosis of infiltrating renal angiomyolipoma with tumor invasion of the vein. Laparoscopic nephrectomy was performed, with removal of tumor thrombus. The operative time was 127 minutes and estimated bleeding 20 ml. There were no intraoperative or postoperative complications. The patient is currently asymptomatic after 12 months of follow up. The pathological study of the surgical piece showed a renal angiomyolipoma with invasion of the kidney and a solid tumor in the lumen of the renal vein.

Objetivo: Se presenta el caso clínico de un Angiomiolipoma renal con extensión a vena renal, patología de muy baja frecuencia. Caso clínico: Mujer de 36 años, quien consulta por dolor en flanco izquierdo. En una tomografía computada se encuentra una lesión tumoral extensa del riñón izquierdo compatible con un Angiomiolipoma renal infiltrante e invasión tumoral de la vena renal. Se realiza nefrectomía laparoscópica, con extirpación de trombo tumoral. El tiempo operatorio fue de 127 min y el sangrado estimado de 20 ml. No hubo complicaciones intra ni postoperatorias. La paciente se encuentra actualmente asintomática luego de 12 meses de seguimiento. La histología mostró un Angiomiolipoma renal con invasión del riñón y un tumor sólido en el lumen de la vena renal. Conclusión: La invasión de vena renal por un Angiomiolipoma es extremadamente raro. Su resolución laparoscópica es posible, con sólo una comunicación previa en la literatura.

Active surveillance of renal masses: an analysis of growth kinetics and clinical outcomes stratified by radiological characteristics at diagnosis

AIMS To determine the growth rate of renal masses (RMs) under active surveillance (AS), and to describe the clinical outcome of AS patients. Materials and Methods We conducted a retrospective review of an AS database to obtain demographics, radiological and pathologic characteristics and RM size of patients. RMs were followed at 6-12 month intervals for ≥1 year with computed tomography (CT), magnetic resonance imaging (MRI), or renal ultrasound. Kaplan-Meier analysis determined the annual likelihood of intervention. RMs were divided into 3 radiographic subcategories (solid, cystic, and angiomyolipoma). A linear regression model determined RM growth rates. Results 131 RMs in 114 patients were included. Median age, Charlson Comorbidity Index score and mean follow-up were 69.1 years, 4.0 and 4.2±2.6 years, respectively. Maximal tumor diameter (MTD) at diagnosis was 2.1±1.3 cm. 49 RMs exhibited negative or zero net growth. Mean MTD growth rate for all RMs was 0.72±3.2 (95% CI: 0.16-1.28) mm/year. When stratified by MTD at diagnosis, mean RM growth rates were 0.84, 0.84, 0.44, 0.74 and 0.71 mm/year for RMs <1 cm, 1-<2cm, 2-<3cm, 3-<4cm and ≥4cm, respectively (p<0.01). The 5 and 10-year freedom from intervention rates were 93.1% and 88.5%, respectively. There was a single case of suspected metastases, but no deaths related to kidney cancer. Conclusions RMs under AS grew slowly, and had a low incidence of requiring surgical intervention and progression. Solid enhancing masses grew slowly, and were more likely to trigger intervention. AS should be considered for selected patients with small RMs. .

Robotic-assisted radical nephrectomy for renal angiomyolipoma with inferior vena cava thrombus extension

Renal angiomyolipoma with inferior vena caval venous extension is rare with only 40 cases reported in the literature. We report a case of a 35-year-old lady with angiomyolipoma with inferior vena caval thrombus that was managed surgically with robotic-assisted radical nephrectomy

Angiomiolipoma renal: ¿es siempre un tumor benigno? / Renal angiomyolipoma: Is it always a benign tumor?

El angiomiolipoma (AML) renal es un tumor sólido compuesto por células de músculo liso, vasos sanguíneos dismórfi cos y tejido adiposo. Esta lesión ha sido considerada siempre como una neoplasia benigna. Reportamos a una paciente de 44 años, asintomática, con una lesión sugerente de AML mayor a 4 cm en el TAC que fue sometida a nefrectomía parcial abierta. La biopsia definitiva informó un angiomiolipoma con componente epiteloídeo focal (AMLE). Controles de imágenes posteriores de esta paciente no han evidenciado recidivia. El angiomiolipoma epiteloídeo (AMLE) es una variante descrita en los últimos años y que sugiere un cambio en el paradigma clásico de “benignidad” asociada al AML Las guías para el manejo de los AML no toman en cuenta la posibilidad de que se trate de un AMLE en sus recomendaciones. Existe muy poca información respecto al manejo de este tipo de lesiones, sólo hay series de casos publicadas. Faltan estudios prospectivos que otorguen herramientas para la toma de decisiones terapéuticas adecuadas en estos pacientes.

Renal angiomyolipoma (AML) is a solid tumor formed by smooth muscle cells, dimorphic blood vessels and adipose tissue. This lesion has been always considered as a benign neoplasm. We report an asymptomatic 44 year-old female patient, with a tumor suggesting an AML in a CT scan greater than 4 cms, who had an open partial nephrectomy. The biopsy report showed an AML with a focal epithelioid component. Follow-up imaging in this case has not showed any recurrence. Epithelioid angiomyolipoma (EAML) is a variant with malignant potential that must be considered when a patient with a renal AML is been evaluated. Guidelines for AML management do not take AMLE as a differential diagnosis. Few studies have been published regarding the management of this kind of lesion, only consisting of case series. There is lack of prospective studies that could give tools for the decision-making process in the treatment of these patients.

Secure reconstruction technique after partial nephrectomy irrespective of tumor size and location

Introduction: Nephron-sparing surgery for large renal masses is not considered a safe procedure because of high complication rate. We present our experience using expanded polytetrafluoroethylene (Gore-Tex®) and Hem-O-Lok® (Weck® Clip) as hemostatic agents during open partial nephrectomy (OPN) to perform nephron-sparing surgery for large renal masses. Materials and Methods: Sixty patients underwent OPN for suspicious renal cell carcinomas. Thirty-four patients with tumors < 2.5 cm in size underwent OPN with Gore-Tex® alone (group 1). Clinical data from a computerized database were reviewed and compared to a contemporary group of 26 patients with tumors ≥ 2.5 cm in size who underwent OPN with Gore-Tex® and Hem-O-Lok® (group 2). Results: The mean patient age was 53 years (range, 35-85 years), and the mean duration of follow-up was 41.8 months (range, 6-56 months). The mean cold ischemic times were 24.0 minutes (range, 12-37 minutes) and 35.3 minutes (range, 18-65 minutes) respectively in group 1 and 2. The tumor sizes in groups 1 and 2 were 1.7 ± 0.4 cm and 4.74 ± 2.75 cm, respectively. No major complications, such as urine leakage or delayed bleeding, were noted in either group. Conclusions: Nephron-sparing surgery using Gore-Tex® alone or a Gore-Tex® and Hem-O-Lok® combination was safe without high-priced hemostatic agents because the tensile strength was sufficient to maintain firmness in the repaired parenchyma. In addition, the procedure is easy to perform and takes less time to complete. Furthermore, major complications, recurrence, and impaired renal function did not occur with this procedure.

Nefrectomía parcial laparoscópica en angiomiolipoma gigante / Laparoscopic partial nephrectomy in giant renal angiomyolipoma

Introducción: El angiomiolipoma es un tumor renal benigno constituido fundamentalmente por vasos sanguíneos, músculo liso y tejido adiposo. El tratamiento depende del tamaño, sintomatología y condición general del paciente. El manejo quirúrgico se debe enfocar en la preservación de la mayor cantidad de parénquima renal posible, siendo la nefrectomía parcial laparoscópica una alternativa a considerar. Reporte del caso: Paciente de 31 años de edad, sexo femenino, consulta en agosto del 2002 por dolor lumbar inespecífico y sintomatología de infección urinaria. La ecografía renal mostró una tumoración sólida de 4 cm en el polo superior del riñón derecho. La tomografía axial computada confirmó el diagnostico imagenológico de angiomiolipoma renal, por lo que se decide mantener una conducta expectante. En abril del 2006 se le diagnostica un embarazo molar que requiere manejo con quimioterapia. En el estudio de diseminación se demostró un aumento de tamaño del angiomiolipoma renal de 4 cm a 9 cm de diámetro, por lo que se planifica una nefrectomía parcial laparoscópica. En enero del 2006 se realiza la nefrectomía parcial laparoscópica sin incidentes. El tiempo de isquemia fue de 47 minutos con un sangrado aproximado de 250 ce y con un tiempo operatorio de 150 minutos. No se registraron complicaciones. La biopsia de la pieza operatoria informó una lesión compatible con un angiomiolipoma. Discusión: El angiomiolipoma es un tumor renal benigno más frecuente en mujeres en edad media. Se puede presentar con dolor en flanco, masa palpable y hematuria, sin embargo, sobre un 50 por ciento se pesquisan de manera incidental. La tomografía axial computada es el examen que entrega mayor información respecto a la naturaleza de la lesión. El tratamiento depende del tamaño tumoral, velocidad de crecimiento, condiciones del paciente y de la sintomatología. En relación al manejo quirúrgico, la evolución de la laparoscopia ha permitido que la nefrectomía parcial laparoscópica sea una...

Angiomyolipoma is a benign renal tumor. Its treatment depends on the size, symptoms and clinical condition of the patient. During its surgical excisión it is imperative to preserve the highest amount possible of normal kidney tissue. We report a 31 years old female consulting for pain in the lumbar area. A kidney ultrasound showed a solid tumor measuring 4 cm in the upper segment of the right kidney. A CAT sean confirmed the diagnosis of a renal angiolmyolipoma. In April 2006 she had a molar pregnancy and required chemotherapy. The imaging study showed that the size of the renal tumor increased from 4 to 9 cm. The patient was subjected to a partial laparoscopic nephrectomy. The pathology report of the surgical piece confirmed the diagnosis of angiomyolipoma.

Ruptura de angiomiolipoma renal gigante: relato de caso / Break giant renal angiomyolipoma: case report

Introdução: Angiomiolipoma renal é uma neoplasia beigna formada por vasos sanguíneos, adipócitos e fibras de músculos lisos. Pode se associar à esclerose tuberosa ou ser esporádico. Geralmente é assintomático e diagnosticado incidentalmente. Ruptura espontânea é rara, porém pode se constituir numa complicação severa e potencialmente fatal. Relatamos um caso de ruptura de angiomiolipoma gigante tratado cirurgicamente. Relato de caso: Uma paciente de 51 anos, branca, foi admitida com dor de início súbito em flanco e região lombar. Tomografia computadorizada mostrou um tumor adiposo renal com hematoma retroperitoneal. Tratada cirurgicamente por drenagem do hematoma e tumorectomia, sendo feito o diagnóstico de angiomiolipoma, apresentando excelente evolução. Conclusão: Descrevemos o caso de uma ruptura de angiomiolipoma gigante esporádico que foi tratado por cirurgia e teve uma boa recuperação.

Introduction: Renal Angiomyolipoma is a beign tumor formed by blood vessels, adipocytes and smooth muscle fibers. May be associated with tuberous sclerosis or be sporadic. It is usually asymptomatic and diagnosed incidentally. Spontaneous rupture is rare but can constitute a severe and potentially fatal complication. A case of ruptured giant angiomyolipoma treated surgically. Case report: A patient of 51 years, White was admitted with sudden pain in the flank and lumbar region. A CT scan showed a fatty tumor renal retroperitoneal hematoma. Treated surgically by lumpectomy and drainage of the hematoma, and the diagnosis of angiomyolipoma, showing excellent progress. Conclusion: We describe a case of a ruptured giant sporadic angiomyolipoma that was treated by surgery and had a good recovery.

Angiolipoma renal con extensión tumoral a vena cava inferior y aurícula derecha / Left kidney angiomyolipoma, spreading to the renal vein, inferior vena cava and involving the heart: report of one case

El angiomiolipoma renal es un tumor benigno mesenquimático. Constituyen sólo del 2 al 6 por ciento de los tumores renales. Existen alrededor de 12 casos reportados con invasión a las venas renales y la cava inferior. En menos de 5, hay extensión de trombo tumoral hasta aurícula derecha. Objetivo: Presentar el caso clínico, manejo y evolución de una paciente con un angiomiolipoma renal con extensión tumoral a vena renal y cava inferior y que compromete la cavidad auricular derecha casi en su totalidad. Paciente y método: 50 años, sexo femenino con hallazgo del tumor por un ultrasonografía abdominal motivada por el estudio de síntomas digestivos de origen probablemente funcional. Se confirma el diagnóstico con TAC y ecocardiograma doppler. Se realiza una cirugía combinada, abdominal y esternotómica, con la ayuda de un by pass aortopulmonar. Resultados: Nefrectomia izquierda, liberación intravascular del tumor que se empuja por cava inferior y se extrae en block por la aurícula derecha. Evoluciona inicialmente en forma satisfactoria, pero desarrolla distress respiratorio con angio TAC de tórax negativo para TEP a las 48 h de la cirugía. Se inicia anticoagulación empírica con HBPM y encontrándose extubada y en buenas condiciones hace un hemoperitoneo el día 14, encontrándose hemorragia en napa en los sitios de disección previa. De alta a los 21 días. El seguimiento alejado a los 2 años revela una hernia incisional, reparada sin incidentes, y sin otras complicaciones ni signos de recidiva de patología original. Conclusión: El manejo de equipo multidisciplinario nos permitió ayudar exitosamente a esta paciente con patología rara y compleja.

Renal angiomyolipoma is an uncommon benign tumor of mesenchymal origin. In less than five of 12 cases reported with renal vein and inferior vena cava involvement the thrombus extends to the right atrium. We report a 50 years old female with a left kidney angiomyolipoma, spreading to the renal vein, inferior vena cava and involving the heart, invading the right atrium almost completely. The tumor was found during a study for abdominal pain. She underwent combined abdominal and cardiac surgery with pulmonary bypass. A left nephrectomy, cavotomy and intravascular dissection of the tumor were performed. The intravascular mass was pushed from abdomen and pulled out through the right atrium. Forty eight hours after surgery, she developed respiratory distress. A chest angio CT scan negative for pulmonary thromboembolism. However, anticoagulation with low molecular weight heparin was started due to the high risk for thromboembolism. She recovered, but 14 days after the original surgery, presented a massive hemoperitoneum. She was operated again, finding a diffuse oozing from the sites of previous dissection. The dose of anticoagulation was lowered, with a good postoperative evolution, being discharged 21 days later. After two years of follow up, she developed an incisional hernia that is repaired.

Rotura de angiomiolipoma: relato de caso / Renal angiomyolipoma rupture: case report

Os angiomiolipomas são lesões geralmente benignas. Caracterizamsepela presença de tecido adiposo maduro, músculo liso e vasos sangüíneos com parede espessada. Os angiomiolipomas são lesões assintomáticas, porém em 10% dos casos, geralmente em tumoresmaiores, pode ocorrer sangramento intenso, acompanhado de choquehipovolêmico. Relata-se o caso de uma mulher de 33 anos, com rotura espontânea de um angiomiolipoma, tratada inicialmente por embolização da lesão, que estabilizou o quadro clínico da paciente, sendo posteriormente realizada nefrectomia parcial.

Bilateral giant renal angiomyolipoma associated with hepatic lipoma in a patient with tuberous sclerosis

OBJECTIVE: To report a case of bilateral giant renal angiomyolipoma associated with tuberous sclerosis, with successful treatment, and to review the literature concerning angiomyolipoma treatment. CASE REPORT: Patient with tuberous sclerosis and angiomyolipoma diagnosed by ultrasonography during her pregnancy. At that time, the angiomyolipoma on the right side was 9 cm in diameter. Conservative management was selected during her pregnancy. The patient returned 7 years later, with a 24.7 x 19.2 x 10.7 cm tumor on the right side and another of 13 x 11.5 x 6.5 cm on the left side, in addition to multiple small angiomyolipomas. A nephron-sparing surgery with tumoral enucleation was performed on the right side, and after 3 months, the tumor on the left side was removed. Renal function in the post-operative period was preserved, and contrast medium progression was uniform and adequate in both kidneys. CONCLUSION: We conclude that an angiomyolipoma larger than 4 cm should be removed surgically, since they have a greater growth rate and pose a risk of hemorrhage. Resection of smaller tumors is safe and has decreased morbidity. Tumoral enucleation is an effective treatment method that preserves kidney function