Renal angiomyolipoma with epithelial cyst

Angiomyolipoma with epithelial cysts (AMLEC) is a recently described entity and is an uncommon subtype of kidney angiomyolipomas. AMLEC is a benign entity but usually masquerades a renal cell carcinoma on imaging examination. AMLEC has a distinct histological and immunohistochemical staining pattern, which helps in the pathological diagnosis. We present a rare case of AMLEC in a 26-year-old female, which was provisionally diagnosed as renal cell carcinoma on radiology. We also summarize the differential diagnosis of this rare variant, its characteristic features, and a review of the literature.

Angiomyolipoma of the broad ligament

Angiomyolipoma is a benign mesenchymal neoplasm of the renal parenchyma, accounting for 1% of all renal parenchymal tumors. However, this entity may rarely occur in extrarenal sites. Extrarenal angiomyolipoma has been documented in various sites of the body, but angiomyolipoma of the broad ligament was reported in only two cases. We report the reputed third case of angiomyolipoma of the broad ligament in a 33-year-old female, who presented clinically with abdominal distension. With a working diagnosis of low-grade neoplasm, an en-bloc excision of the left broad ligament mass was performed. Based on histopathology and immunohistochemistry, a diagnosis of the classical variant of angiomyolipoma of the left broad ligament was made. The post-operative period was uneventful with no recurrence after 6 months of follow-up.

Epithelioid angiomyolipoma with tumor thrombus in IVC and right atrium

Epithelioid angiomyolipoma is an uncommon subtype of renal angiomyolipoma associated with potentially malignant behavior and is considered a distinct entity by the World Health Organization classification of renal tumors. We present a case of an epithelioid variant of angiomyolipoma with extension into the renal vein, inferior vena cava reaching up to the right atrium. Pre-operatively, a diagnosis of renal cell carcinoma was considered based on imaging findings. Intra-operatively due to extensive adhesions, surgical resection was not performed and only tissue sampling was performed for histopathology. Microscopic examination revealed short fascicles of spindle cells and perivascular epithelioid cells. A differential diagnosis of renal cell carcinoma with sarcomatoid differentiation was considered. The immunohistochemical profile showed tumor cells that express Melan-A and smooth muscle actin, while they were negative for pan-cytokeratin, PAX8, CK7, CD117 and CD34. Therefore a diagnosis of epithelioid angiomyolipoma was rendered. The presence of intravascular thrombi on radiological investigation and carcinoma-like growth pattern on light microscopy may compound an erroneous diagnosis of renal cell carcinoma. Hence, it is prudent for the urologist to consider differential diagnosis other than renal cell carcinoma when confronted with a renal neoplasm presenting with intravascular thrombi. In these cases, a core biopsy should be planned pre-operatively and diagnosis should be made with aid of appropriate immunohistochemical markers.

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.

Minimal fat renalangiomyolipoma with multiple lymph nodes enlargement and postoperative refractory lymphatic fistula: a case report and literature review / 北京大学学报(医学版)

Renal angiomyolipoma (AML) is a common benign tumor in the urinary system, mainly composed of adipose tissue, blood vessels and muscle tissue. Renal AML is sporadic in most of patients, while a few are associated with tuberous sclerosis. Classical renal AML occurs predominantly in middle-aged females. Most cases are found incidentally during imaging examinations. The fat content makes AML have unique imaging characteristics and is easy to be identified with other renal tumors. However, the amount of fat varies in each tumor. AML that contains only microscopically detectable fat and whose amount of intratumoral fat may be too small to be identified on unenhanced computed tomography (CT) images is termed minimal fat or fat-poor renal AML, which appears as a high density shadow in the renal parenchyma on unenhanced CT images. Thus, it can be difficult to distinguish it from renal cell carcinoma (RCC) on imaging. Since the imaging findings are atypical, the diagnosis depends on pathological results. In addition, a few of AML can mimic malignant neoplasms. Recent studies suggested that AML might involve to peri-renal or renal sinus fat, regional lymphatics and other visceral organs, as well as inferior vena cava, which further makes the diagnosis more difficult. However, there is currently no reports about involvement of regional limphatics in minimal fat renal AML. In this article, we report a 27-year-old female patient without family history of tuberous sclerosis, who came to visit the hematologist because a high density shadow near the left kidney was found during CT scan which was accompanied by neck, armpits, groin, abdominal cavity and retroperitoneal lymph nodes enlargement. She was suspected of lymphoma in the beginning and transferred to Department of Urology to perform laparoscopic left renal mass and retroperitoneal lymph node excision and pathological examination for a definitive diagnosis. Finally, pathologic results revealed AML. Postoperative continuous lymphatic fistula developed and the retroperitoneal drainage of chylous fluid was 100-200 mL per day, lasting for 12 weeks. The fistula was successfully closed after conservative treatment including fasting and rehydration. This article summaries and discusses the diagnosis and treatment of renal AML with lymph nodes enlargement and the management of postoperative refractory lymphatic fistula by reviewing the related cases and literature.

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.

Hematuria macroscópica en paciente con angiomiolipoma renal y síndrome de cascanueces asintomático / Macroscopic hematuria in a patient with renal angiolipoma and asymptomatic nutcracker syndrome

El angiomiolipoma renal es un tumor benigno poco frecuente y constituye menos del 2% de todos los tumores renales, teniendo una incidencia de 0.3-3% aproximadamente a nivel mundial. En Venezuela solo existe un reporte de angiomiolipoma, publicado en el 2015. Aunque estos tumores se asocian en un 20% con el complejo de esclerosis tuberosa y la linfangioleiomiomatosis esporádica, pueden presentarse aisladamente con una relación mujer-hombre de 2:1 y más en la 4ª y 5a década de la vida. Presentamos el caso de una mujer de 32 años de edad, quien consultó en 2006 por hematuria macroscópica, dolor en hipogastrio, sin relación con la menstruación; concomitante polaquiuria, astenia y palpitaciones; tuvo episodios recurrentes 3-4 veces/año con transfusión de hemoderivados por anemia severa. En el año 2015 la tomografía mostró "síndrome de cascanueces". La uretrocistoscopia mostró hemorragia activa por uréter derecho, y se realizó nefrectomía parcial derecha cuya biopsia reportó angiomiolipoma renal, con buena evolución y egreso a las 72 horas(AU)

Renal angiomyolipoma is a benign, rare tumor present in less than 2% of all renal tumors; it´s world incidence is 0.3-3%. In Venezuela only one case was reported in 2015. Although these tumors are associated with 20% of the Tuberous Sclerosis, they can present with a gender proportion of 2:1, more often in the 4th and 5th decade. We present here the case of a 32 year- old woman who in 2006 had consulted for hematuria, pelvic pain, weakness and palpitations not related with her menses. These episodes recurred several times per year and she received red blood cell transfusions. In the year 2015 a computerized tomography showed Nutcracker Syndrome and the urethrocystoscopy showed an active bleeding originating in the right urether. A right partial nephrectomy was done and the biopsy was reported as Renal angiomyolipoma. She was discharged 72 hours after her operation(AU)

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.

Benign nodules mimicking hepatocellular carcinoma on gadoxetic acid-enhanced liver MRI

No abstract available.

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. .

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.

Hepatic angiomyolipoma with minimal fat, mimicking hepatocellular carcinoma

No abstract available.

Trends of Presentation and Clinical Outcome of Treated Renal Angiomyolipoma

PURPOSE: The purpose of this study is to set guidelines for the management of renal angiomyolipoma (AML), clinical prognosis according to tumor size, in association with tuberous sclerosis complex (TSC), multiplicity, radiographic finding, and treatment modality. MATERIALS AND METHODS: Between March 1998 and October 2008, 129 out of 254 patients with AML who underwent surgical intervention or angioembolization were enrolled. Diagnosis of AML was determined by the presence of a low attenuated component on CT imaging or by pathological confirmation. Indications of treatment were intractable pain, hematuria, suspicion of malignancy, large tumor size, spontaneous rupture, and radiographically equivocal tumors in which a differential diagnosis was needed to rule out malignancy. Parameters including age, sex, tumor size, multiplicity, radiographic characteristics, association with TSC, and treatment modality were reviewed. RESULTS: Age at presentation was 50.6 years and mean tumor size was 3.5 cm. Presentation symptoms were flank pain, hematuria, spontaneous rupture, and fatigue. 97 (75.2%) patients were incidentally discovered. 100 (77.5%) were females. 68 (52.7%) underwent nephron-sparing surgery (NSS), 35 (27.1%) radical nephrectomy, and 26 (20.2%) angioembolization. TSC was accompanied in 12 (9.3%) patients. No patient developed renal function impairment during the mean follow-up period of 64.8 months. Patients with TSC presented at a younger age, along with larger, bilateral, and multiple lesions. CONCLUSION: Significant differences in clinical manifestations and treatment outcomes were noted in respect to tumor characteristics, association with TSC, and treatment modality. Considering the benign nature of AML, these parameters ought to be considered when deciding upon active surveillance or prophylactic intervention.

Angiomyolipoma of the kidney: the experience at the University Hospital of the West Indies / El angiomiolipoma del riñón: la experiencia del Hospital Universitario de West Indies

OBJECTIVE: Angiomyolipoma (AML) of the kidney is an uncommon tumour that, until recently, was often misdiagnosed preoperatively as renal cell carcinoma (RCC). Newer radiological techniques have allowed more accurate preoperative diagnosis which can facilitate preoperative counselling and planning for conservative therapy. This study reviews the experience with these uncommon tumours at the University Hospital of the West Indies. METHODS: All cases of AML diagnosed during the period 1980 to 2007 were retrospectively identified from the files of the Department of Pathology. From these records, selected data were retrieved and analysed. These included patient demographics, clinical history, clinical diagnosis and pathologic characteristics of the specimen submitted. The total number of primary renal tumours diagnosed in adults during the same period was also determined for comparison. RESULTS: Eleven cases of AML were identified among 149 primary renal tumours in adults. Ten of these cases occurred in women. Amongst these, a single case of tuberous sclerosis was confirmed in a patient with bilateral lesions. Excluding this patient, who was 24 years old, ages ranged from 24 to 86 years with a mean of 44 years (median 40.5 years) and an equal number of lesions was present on each side. Abdominal or flank pain were the most common clinical symptoms, present in six cases but in three cases, the tumours were discovered incidentally. The correct clinical diagnosis was made pre-operatively in a single case. By contrast, a diagnosis of RCC or other malignant tumour was proffered in eight cases. Pathologically, the maximum dimension of the seven excised tumours, in whom such information was recorded, ranged from 3.5 cm to 12 cm with a median of 7 cm. Spontaneous haemorrhage in the tumour was noted in three cases, all greater than 4.5 cm in maximum dimension. CONCLUSIONS: These data confirm that AML is uncommon at the University Hospital of the West Indies. There was an overwhelming female preponderance and patients presented, most commonly, in the 3rd to 4th decades. Tuberous sclerosis was identified in a solitary case. In this series, symptomatic lesions were > 4.5 cm in maximum dimension and haemorrhage complicated three cases. Most cases were incorrectly diagnosed preoperatively.

OBJETIVO: El angiomiolipoma (AML) del riñón es un tumor poco común que hasta hace poco era a menudo mal diagnosticado preoperatoriamente como carcinoma celular renal (RCC). Técnicas radiológicas más nuevas han permitido realizar un diagnóstico preoperatorio más exacto, que puede facilitar aconsejamiento preoperatorio y planificar una terapia conservadora. El presente estudio examina la experiencia con estos tumores poco comunes en el Hospital Universitario de West Indies. MÉTODOS: Todos los casos de AML diagnosticados durante el periodo de 1980 a 2007 fueron identificados retrospectivamente a partir de los archivos del Departamento de Patologías. De estos archivos, se seleccionaron datos que fueron recuperados y analizados. Estos abarcaron: la demografía de los pacientes, las historias clínicas, el diagnóstico clínico y las características patológicas del espécimen presentado. El número total de tumores renales primarios diagnosticados en adultos durante el mismo período, se determinó también por comparación. RESULTADOS: Se identificaron once casos de AML entre 149 tumores renales primarios en adultos. Diez de estos casos ocurrieron en mujeres. Entre éstas, se confirmó sólo un caso de esclerosis tuberosa, en un paciente con lesiones bilaterales. Excluyendo a este paciente, que tenía 24 años de edad, las edades fluctuaron de 24 a 86 años para una media de 44 años (mediana 40.5) y un número igual de lesiones estuvieron presentes en cada lado. El dolor abdominal o el lateral, constituyeron los síntomas clínicos más comunes, presentes en seis casos, pero en tres casos, los tumores fueron descubiertos incidentalmente. El diagnóstico clínico correcto se realizó preoperatoriamente en un solo caso. En contraste con ello, un diagnóstico de RCC u otro tumor maligno se presentó en ocho casos. Patológicamente, la máxima dimensión de los siete tumores extirpados, en los cuales se registró tal información fue registrada, fluctuó de 3.5 a 12 cm con una mediana de 7 cm. La hemorragia espontánea en el tumor fue observada en tres casos, todos mayores de 4.5 cm. de tamaño máximo. CONCLUSIONES: Estos datos confiman que la AML es poco común en el Hospital Universitario de West Indies. Hubo una abrumadora preponderancia de casos femeninos, y los pacientes se presentaron con mayor frecuencia en las décadas 3era y 4ta. La esclerosis tuberosa se identificó sólo en un caso. En esta serie, las lesiones sintomáticas tuvieron > 4.5 cm. de dimensión máxima, y la hemorragia complicó tres casos. La mayoría de los casos fueron diagnosticados incorrectamente en la etapa preoperatorio.

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.

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.

A case of retroperitoneal lipoleiomyoma

We report a case of lipoleiomyoma which arose in retroperitoneum and presented with progressively distended abdomen in a 56-yr-old woman. The tumor was well encapsulated and consisted of two components, benign smooth muscle cells and mature adipose tissue without any atypia. It is likely to be mistaken as extrarenal angiomyolipoma, well-differentiated liposarcoma and leiomyoma with fatty change. We review the histologic characteristics of previously reported myolipoma and describe essential points of differential diagnosis.

A Case of Mediastinal Angiomyolipoma

Angiomyolipoma is a common tumor of the kidney but has rarely been found in the mediastinum. We report a case of angiomyolipoma of the posterior mediastinum in a 62-year-old woman. She experienced exertional dyspnea and intermittent cough at admission. Computed tomography indicated a tumor located at the left paravertebral and upper posterior mediastinum and MRI imaging demonstrated a mass with low signal intensity in T1-weighted image at T4-5 level. Thoracotomy was done for surgical removal of the tumor and histologic examination revealed a mesenchymal tumor composed of mature fat, capillaries and smooth muscle fibers. The tumor was immunohistochemically positive for CD34 and factor-VIII (for vascular component) smooth muscle actin (for smooth muscle component) and S-100 protein (for fat component). There have been four case reports about mediastinal angiomyolipoma, namely three Japanese cases and one French case. It is suggested that angiomyolipoma could be considered for the differential diagnosis of mediastinal tumors.

Hamartomas renales en el adulto / Adult renal hamartomas

Los angiomiolipomas renales, los hamartomas renales más frecuentes, son muy conocidos por los radiólogos a pesar de ser poco frecuentes y de importancia clínica limitada, ya que representan una de las pocas lesiones en las que en la mayoría de los casos se logra el diagnóstico específico basado en los hallazgos radiológicos. Debido a la cantidad tan variable de los diferentes componentes celulares y a que ocasionalmente se asocian a hemorragia, las características radiológicas de los angiomiolipomas son muy variadas. En el ultrasonido, los angiomiolipomas se observan como masas ecogénicas con sombra acústica. En la tomografía computarizada (TC) estas lesiones típicamente aparecen como masas corticales bien delimitadas, pequeñas (tamaño < 5 cm), con predominio de tejido adiposos y gran cantidad de tejido blando heterogéneo disperso en toda la masa. Algunos angiomiolipomas son más grandes y mal delimitados debido a la hemorragia. los angiomiolipomas clásicos se componen principalmente de grasa; infrecuentemente algunos tumores en los cuales no se demuestra la grasa, no pueden diferenciarse radiológicamente del carcinoma de células renales. El leiomioma renal, una lesión que patológicamente se solapa con el agniomiolipoma, tiene una imagen bastante diferente (p.e.), homogénea, sin grasa que no puede diferenciarse de las lesiones renales malignas