Endovascular treatment of intrarenal aneurysms bleeding and angiomyolipomas in a patient with tuberous sclerosis and polycystic kidney disease / Tratamento endovascular de sangramentos e angiomiolipomas de aneurismas intrarrenais em um paciente com esclerose tuberosa e doença renal policística

Abstract Tuberous sclerosis complex (TSC) and autosomal dominant polycystic kidney disease (ADPKD) are conditions related to renal failure that can rarely occur in association as a contiguous gene syndrome. Angiomyolipomas (AMLs) are renal tumors strongly related to TSC that may rupture and cause life-threatening bleedings. We present a patient with TSC, ADPKD, and renal AMLs with persistent hematuria requiring blood transfusion. The persistent hematuria was successfully treated through endovascular embolization, a minimally invasive nephron sparing technique.

Resumo O complexo de esclerose tuberosa (CET) e a doença renal policística autossômica dominante (DRPAD) são condições relacionadas à insuficiência renal que raramente podem ocorrer em associação como uma síndrome do gene contíguo. Angiomiolipomas (AMLs) são tumores renais fortemente relacionados ao CET que podem romper-se e causar hemorragias com risco de vida. Apresentamos um paciente com CET, DRPAD e AMLs renais com hematúria persistente que requer transfusão sanguínea. A hematúria persistente foi tratada com sucesso por meio de embolização endovascular, uma técnica de preservação do néfron minimamente invasiva.

Endovascular management of bilateral renal angiomyolipoma in a perimenopausal woman / Manejo endovascular de angiomiolipoma renal bilateral em uma mulher na perimenopausa

Abstract Renal angiomyolipoma is a benign and progressive tumor consisting of smooth muscle, fat, and vascular elements and is commonly associated with the tuberous sclerosis complex. Bilateral occurrence is rare and recent evidence suggest strong tumor positivity to female hormones with enlargement during pregnancy and oral contraceptive therapy. Treatment varies from a minimally invasive approach with selective embolization of the renal artery to partial nephrectomy or sometimes even radical nephrectomy. Presented here is a case of bilateral renal angiomyolipoma in 50-year-old lady who was successfully treated with super-selective embolization.

Resumo O angiomiolipoma renal é um tumor benigno e progressivo composto por músculo liso, gordura e elementos vasculares, comumente associado ao complexo da esclerose tuberosa. Sua ocorrência bilateral é rara, e evidências recentes sugerem forte positividade do tumor para hormônios femininos, com aumento do tamanho durante a gravidez e uso de contraceptivos orais. O tratamento varia desde uma abordagem minimamente invasiva de embolização seletiva da artéria renal até nefrectomia parcial ou, às vezes, nefrectomia radical. Apresentamos um caso de angiomiolipoma renal bilateral em uma mulher de 50 anos que passou por tratamento bem-sucedido com embolização superseletiva.

Angiomiolipoma nasal: reporte de dos casos y revisión de la literatura

Resumen El angiomiolipoma es una lesión hamartomatosa, habitualmente de localización renal asociado a esclerosis tuberosa. La ubicación nasosinusal es extremadamente infrecuente y se puede presentar con obstrucción nasal y epistaxis. Se presentan dos casos de angiomiolipoma de cavidad nasal diagnosticados en el Hospital Carlos Van Buren de Valparaíso. Ambos casos se presentaron como masa nasal unilateral, se estudiaron con tomografía computada y fueron tratados con biopsia escisional, sin presentar recidivas durante su seguimiento. El anigiomiolipoma está compuesto por vasos sanguíneos, músculo liso y adipocitos. Existen diferencias entre el angiomiolipoma del riñón versus el de piel, cavidad oral y nasal (angiomiolipomas mucocutáneos); estos últimos son extremadamente infrecuentes, de menor tamaño, con presencia de agregados linfoides, negativos para antígeno específico de melanoma HMB-45 y sin asociación a esclerosis tuberosa. Existen pocos casos reportados en la literatura de esta patología, por lo que existe poca evidencia. Clínicamente, se presentan con obstrucción nasal, epistaxis recurrente, sensación de masa nasal. El estudio preoperatorio depende de su extensión y puede incluir una tomografía computada y/o angiografía para identificar su irrigación. El diagnóstico definitivo es anatomopatológico. Si se logra la escisión completa, no se han reportado recidivas, por lo que presenta un excelente pronóstico.

Abstract Angiomyolipoma is a hamartomatous lesion, usually of a renal location associated with tuberous sclerosis. Nasosinusal location is extremely rare and can present with nasal obstruction and epistaxis. Two cases of angiomyolipoma of the nasal cavity diagnosed at the Carlos Van Buren Hospital in Valparaíso are presented. Both cases presented as a unilateral nasal mass, and were studied with computed tomography, and treated with an excisional biopsy, with no recurrences during follow-up. Anigiomyolipoma is composed of blood vessels, smooth muscle and adipocytes. There are differences between angiomyolipoma of the kidney versus skin, oral and nasal cavity (mucocutaneous angiomyolipomas). The latter are extremely infrequent, smaller in size, with the presence of lymphoid aggregates, negative for the HMB-45 specific melanoma antigen and without association with tuberous sclerosis. There are few cases reported in the literature of this pathology, so there is little evidence. Clinically, they present with nasal obstruction, recurrent epistaxis, sensation of nasal mass. The preoperative study depends on its extension and may include a computed tomography and/or angiography to identify its irrigation. The definitive diagnosis is pathological. If complete excision is achieved, no recurrences have been reported, so it has an excellent prognosis.

Ressecção laparoscópica de tumores benignos do fígado: posição atual / Laparoscopic liver resection for benign tumors: the current position

RESUMO - RACIONAL: As principais indicações das hepatectomias video-laparoscópicas (HVL), inicialmente, eram nas lesões hepáticas benignas. À medida que a HVL se tornou mais popular, as indicações de doenças malignas superaram as de doenças benignas. Este estudo teve como objetivo discutir as indicações e resultados da HVL para o tratamento de tumores hepáticos benignos. MÉTODOS: De 445 HVL realizadas em um único centro, 100 (22,4%) foram para tumores benignos. Os autores discutem as indicações para ressecção e apresentam seus resultados perioperatórios. RESULTADOS: No total, 100 pacientes com tumores benignos foram avaliados, a saber: 66 casos de adenomas hepatocelulares; 14 de neoplasia mucinosa biliar; 13 de hiperplasia nodular focal; 4 de angiomiolipomas; e 3 de hemangiomas. O tamanho médio das lesões foi de 7,6 cm (3,1 a 19,6 cm). A taxa de morbidade total foi de 19%, sendo 9% classificados como Clavien-Dindo 3 ou 4 e não foi observada mortalidade. CONCLUSÃO: A HVL para tumores hepáticos benignos é segura e apresenta excelentes resultados. No entanto, as indicações para cirurgia são cada vez mais restritas, não sendo recomendável indicar a ressecção somente por se tratar de procedimento minimamente invasivo.

ABSTRACT - BACKGROUND: The main indications of the use of laparoscopic liver surgery (LLS), in the early days, were benign liver lesions. As LLS became more popular, indications for malignant diseases outnumbered those for benign ones. This study aims to rule out the indications and results of LLS for the treatment of benign liver tumors. METHODS: Out of 445 LLS performed in a single center, 100 (22.4%) were for benign tumors. The authors discuss the indications for resection and present their perioperative results. RESULTS: In total, 100 patients with benign tumors were evaluated. Specifically, these were as follows: 66 cases of hepatocellular adenomas; 14 cases of biliary mucinous neoplasm; 13 cases of focal nodular hyperplasia; 4 cases of angiomyolipomas; and 3 cases of hemangiomas with a mean size of 7.6 cm (ranging from 3.1 to 19.6 cm). The total morbidity rate was 19%, with 9% classified as Clavien-Dindo grades 3 or 4. No mortality was observed. CONCLUSION: LLS for benign liver tumors is safe and presents excellent results. However, indications for resection are increasingly restricted and should not be performed just because it is a minimally invasive procedure.

Angiomiolipoma renal con invasión de vena renal y vena cava inferior. Presentación de un caso y revisión de la literatura / Renal Angiomyolipoma with Extension to Renal and Cava Veins. A Case Report and Revision of the Literature

Introducción El angiomiolipoma es un tumor benigno cuya presentación con invasión del seno renal y formación de trombo venoso tumoral es muy infrecuente, habiéndose descrito aproximadamente 50 casos desde que en 1982 Kutcher describiera el primero. Reporte de caso Presentamos un caso angiomiolipoma renal con afectación venosa diagnosticado mediante TAC a partir del hallazgo de trombo venoso en ecografía y resonancia en una paciente de 43 años embarazada y asintomática que presentó elevación de transminasas en una analítica. Ante la posibilidad de que se tratara de una variante epitelioide y/o clásica con potencial de malignización, se decidió tratamiento mediante nefrectomía y trombectomía abierta, sin incidencias intra ni postoperatorias. La paciente se encuentra asintomática tras 6 meses de seguimiento. La histología fue compatible con angiomiolipoma clásico con trombo venoso. Conclusión La invasión de vena renal por un angiomiolipoma es extremadamente rara. Aunque infrecuente, habría que considerar la posibilidad de malignización para no demorar su tratamiento

Introduction Angiomyolipoma is a benign kidney tumour that rarely affects the renal sinus or the renal or caval vein. Kutcher made the first case report in 1982, and since then there have been described approximately 50 cases. Case Report We present a case of angiomyolipoma with venous thrombus in a 43 years-old asymptomatic pregnant woman who presented elevation of transaminases in a blood test, with an ultrasonography that showed a venous thrombus without any renal mass, being diagnosed after giving birth by scanner. Having in account the possibility of malignancy of the mass, we performed surgical treatment by open radical nephrectomy and thrombectomy, without intraoperative nor postoperative incidences. The patient remains asymptomatic 6 months after surgery. The histology was compatible with classic angiomyolipoma with venous thrombus. Conclussion Angiomyolipoma causing venous invasion is extremely rare, but we should take into consideration its possibility of malignancy in order to treat it as soon as possible

Manejo conservador de choque hemorrágico por ruptura de angiomiolipoma renal: relato de caso / Nonoperative management of hemorrhagic shock due to renal angiomyolipoma rupture: a case report

O angiomiolipoma renal é um tumor benigno e incomum, que cursa com complicações variadas de modo agudo e crônico. Uma de suas manifestações mais dramáticas é a ruptura tumoral, que pode cursar com choque hemorrágico grave. Este relato de caso descreve um episódio de sangramento de grande monta de angiomiolipoma renal, previamente desconhecido, devido à um trauma contuso de baixa energia, que foi manejado com sucesso de modo conservador, utilizando protocolo análogo ao do tratamento não cirúrgico de lesões traumáticas de orgãos intra-abdominais, em um hospital de trauma de referência no Brasil

The renal angiomyolipoma is a benign and rare tumor, that may evolve with different types of acute and chronic complications. One of its most dramatic manifestations is the tumoral rupture, that may cause grave hemorrhagic shock. This case report describes an episode of large amount bleeding of an previously unknown renal angiomyolipoma, due to a low energy blunt trauma, that was successfully managed in a conservative way, using a protocol analogous to the one of non operative management of traumatic lesions of intra abdominal organs, in a reference trauma center in Brasil

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.

Embolization of ruptured renal angiomyolipoma in puerpera / Embolização de angiomiolipoma renal roto em puérpera

Abstract Angiomyolipomas (AMLs) are rare benign tumors derived from mesenchymal tissue and composed of varying degrees of adipose tissue, muscle and blood vessels. Renal AMLs (RAMLs) are the result of a sporadic event, and, in most of cases, the diagnosis is usually incidental, but hemorrhage and shock may be present. During pregnancy, the size of AMLs may increase and they may rupture, probably due to the high expression of hormone receptors, and the increase in maternal circulation and abdominal pressure. The authors present a case of a woman with ruptured RAML submitted to urgent endovascular treatment four days after giving birth by cesarean section.

Resumo Angiomiolipomas (AMLs) são tumores benignos raros derivados do tecido mesenquimal, compostos em graus variados de tecido adiposo, muscular e de vasos sanguíneos. Os AMLs renais (AMLRs) resultam de um evento esporádico e, na maioria dos casos, o diagnóstico costuma ser fortuito, mas quadros de hemorragia e choque podem estar presentes. Durante a gestação, os AMLs podem aumentar de tamanho e romper, provavelmente pela altaexpressãodereceptoreshormonais,epeloaumentodacirculaçãomaternaedapressão abdominal. Os autores apresentam um caso de uma paciente com AMLR roto submetida a tratamento endovascular de urgência no quarto dia pós-operatório de uma cesariana.

Tumor renal benigno versus carcinoma renal: reporte de caso y revisión de la literatura / Benign Kidney Tumor versus Renal Carcinoma: Case Report and Literature Review

Introducción El cáncer renal representa 2,4% de los casos diagnosticados de cáncer en la población general, es más común en hombres que en mujeres, y se presenta con más frecuencia entre la 6ta y la 8ta décadas de vida. Se estima que el 16% de los pacientes se diagnostican como enfermedad metastásica. Objetivo Se presenta el caso de un paciente cuyo diagnóstico de carcinoma renal se confundió inicialmente con un tumor benigno. Métodos A un hombre de 56 años de edad se le realizó hace 3 años ese diagnóstico en un estadio avanzado de la enfermedad, a pesar del hallazgo incidental de una masa, que se consideró benigna durante 5 años. Resultados Al momento del diagnóstico de carcinoma de células claras, el tumor era Estadio IV, con metástasis a pulmón. Recibió primera línea de tratamiento con sunitinib, pero fue suspendido por toxicidad; segunda línea con pazopanib durante 1 año, después presentó progresión de la enfermedad, por lo cual se cambió a tratamiento con axitinib con respuesta parcial, sin embargo, se suspendió por toxicidad cardiaca, entre otras. Al momento el paciente ha recibido 5 ciclos de bevacizumab con adecuada tolerancia. Conclusiones Es necesario resaltar la indicación de diagnóstico adecuado y manejo quirúrgico en masas renales sospechosas.

Introduction Kidney cancer represents 2.4% of diagnosed cases of cancer in the general population; it is more common in men than in women, and occurs more frequently between the 6th and 8th decades of life. It is estimated that 16% of patients are diagnosed as metastatic disease. Objective To report the case of a male patient whose diagnosis of renal carcinoma was initially misdiagnosed as a benign tumor. Methods We present a 56-year-old male diagnosed three years back with malignancy at an advanced stage of the disease, despite the incidental finding of a tumor that for 5 years was considered benign. Results At the time of diagnosis of clear cell carcinoma, the tumor was Stage IV, with lung metastasis. He received first line treatment with sunitinib, which was discontinued due to toxicity. Subsequently, a second line with pazotinib for 1 year, then presented progression of the disease, so treatment was changed to axitinib with partial response., It was discontinued, however, due to cardiac toxicity, among others. At the time of writing, the patient has received 5 cycles of bevacizumab with adequate tolerance. Conclusions It is necessary to highlight the need for adequate diagnosis and surgical management in suspicious renal masses.

Complejo linfangioleiomiomatosis-esclerosis tuberosa, ¿contraindicación para el trasplante pulmonar? Presentación de un caso / Lymphangioleiomyomatosis-Tuberous Sclerosis Complex. Contraindication For Lung Transplantation? A Case Report

El complejo linfangioleiomiomatosis (LAM)-esclerosis tuberosa (ET) es una enfermedad rara, con compromiso multisistémico que afecta principalmente pulmón y cerebro. Se presenta el caso de una paciente de 25 años con LAM pulmonar que ingresa a la institución para protocolo de trasplante pulmonar. Durante los estudios de extensión se documenta compromiso extrapulmonar sistémico del complejo LAM-ET. A partir de este caso, se realizó una revisión de la literatura de las manifestaciones radiológicas sistémicas de la enfermedad y las recomendaciones de diagnóstico y manejo de estos pacientes; en especial, las indicaciones y contraindicaciones del trasplante pulmonar.

The lymphangioleiomyomatosis (LAM) - Tuberous Sclerosis (TS) Complex is a rare disease with multisystem involvement affecting mainly lung and brain. We present the case of a 25-year-old female patient with pulmonary LAM. During the work-up studies for the lung transplantation protocol, the systemic extrapulmonary involvement of the LAM-TS complex is documented. From this case, a literature review of the systemic radiological manifestations of the disease and of the diagnosis and management recommendations of these patients was made. Special focus was made on the indications and contraindications of lung transplantation.

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.

Angiomiolipoma de cavidad nasal: reporte de un caso y revisión de la literatura / Angiomyolipoma of the nasal cavity: case report and literature review

El angiomiolipoma de cavidad nasal es un tumor hamartomatoso extremadamente infrecuente compuesto por adipocitos maduros, espacios vasculares con escaso tejido elástico y la presencia de haces de células musculares lisas maduras. Se manifiesta principalmente por obstrucción nasal unilateral y epistaxis recurrente. Se presenta el caso de una paciente de 73 años con historia crónica de obstrucción nasal y epistaxis recurrente izquierda asociada a rinorrea y descarga posterior intermitente. La tomografía computarizada (TC) y resonancia nuclear magnética (RNM) demuestran una lesión vascular circunscrita en fosa nasal izquierda. La angiografía demostró irrigación exclusiva de la arteria esfenopalatina izquierda. Se realizó extirpación de la lesión por abordaje endonasal endoscópico previa embolización arterial. La revisión de la literatura mundial muestra que es el duodécimo caso de angiomiolipoma de cavidad nasal reportado.

Angiomyolipoma of nasal cavity is an extremely rare hamartoma tumor composed of mature adipocytes, vascular spaces with lack of elastic tissue, and the presence of bundles of mature smooth muscle cells. It manifests itself mainly by unilateral nasal obstruction and recurrent epistaxis. We present the case of a 73-years-old woman with chronic history of left-sided nasal obstruction and recurrent epistaxis associated with rhinorrhea and intermittent post nasal drip. Computed tomography and magnetic resonance imaging demonstrate a vascular lesion inside the left nasal cavity. Angiography showed irrigation exclusively by the left sphenopalatine artery Surgical excision was performed by endoscopic endonasal approach after arterial embolization. World literature review showed that this is the twelfth reported case of angiomyolipoma of the nasal cavity.

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.

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)

Angiomiolipoma renal atípico versus carcinoma de células renales: dilema diagnóstico. Hallazgos útiles por tomografía computada para la discriminación de estos tumores / Atypical renal angiomyolipoma versus carcinoma renal cell: diagnosis dilemma. Findings by computed tomography for the discrimination of these tumors

Objetivo: Diferenciar el angiomiolipoma atípico del carcinoma de células renales del mismo tamaño mediante hallazgos tomográficos. Materiales y métodos: Se realizó un estudio retrospectivo con 68 pacientes (17 con angio-miolipomas atípicos y 51 con carcinoma de células renales) nefrectomizados por diagnóstico presuntivo preoperatorio, mediante tomografía computada trifásica (fases sin contraste, corticomedular y excretora temprana), de carcinoma de células renales menor de 45mm. Dos radiólogos que desconocían el diagnóstico definitivo evaluaron retrospectivamente las características generales del tumor, su atenuación en fase sin contraste y las características de su realce. Se realizó un análisis estadístico con software R, aplicándose el modelo logit para diferenciar el angiomiolipoma atípico del carcinoma de células renales a partir de los hallazgos tomográficos de masa renal, incluidos en el modelo final el contorno, la atenuación en fase sin contraste y el patrón de realce con el tiempo. Resultados: Los hallazgos de angiomiolipoma atípico que resultaron significativos fueron atenuación tumoral en fase sin contraste hiperdensa y patrón de realce con tiempo prolongado con chance de ocurrencia de 10,49 (p=0,0381) y 36,71 (p=0,0009), respectivamente. En los pacientes, según el valor de cada hallazgo incluido en el modelo, se calculó probabilidad, sensibilidad (0,2941) y especificidad (0,9804). La curva característica operativa del receptor (ROC) determinó un punto de corte óptimo (0,9694) para discriminar el angiomiolipoma atípico, por lo que se confirmó su presencia. Conclusión: La tomografía computada helicoidal trifásica es útil para diferenciar el angiomiolipoma atípico del carcinoma de células renales, siendo la atenuación hiperdensa sin contraste del tumor y su patrón de realce prolongado los hallazgos tomográficos más valiosos.

Objective: To compare various computed tomographic features of atypical angiomyolipoma with those of size-matched renal cell carcinoma. Materials and methods: Sixty-eight patients (17 with atypical angiomyolipomas and 51 with carcinoma renal cells) who had undergone nephrectomy by presumptive diagnosis of carcinoma renal cell<45mm on diameter by preoperative triphasic computed tomography (CT) (with unenhanced, corticomedullary, and early excretory phase scanning) were evaluated in a retrospective study. Two reviewers who were unaware of the diagnosis retrospectively recorded tumor attenuation on unenhanced scans, enhancement characteristics and general characteristics of the tumor. Statistical analysis was performed with R software, applying logit model to differentiate atypical angiomyolipoma from renal cell carcinoma from CT findings of renal mass, included in the final model the tumor margin, its attenuation on unenhanced scans and the enhancement pattern over time. Results: For atypical angiomyolipomas the most valuable CT findings were hyperdense attenuation tumour in unenhaced phase scan and prolonged enhancement pattern, with chance of occurrence of 10.49 (p=0.0381) and 36.71 (p=0.0009), respectively. In the patients, as each finding value in the model, probability, sensitivity (0.2941) and specificity (0.9804) was calculated ROC curve was constructed that determined optimal cutoff (0.9694) to discriminate atypical angiomyolipoma, confirming their presence. Conclusion: Triphasic helical CT may be useful in differentiating atypical angiomyolipoma from renal cell carcinoma, with hyperdense tumor attenuation on unenhanced scans and prolonged enhancement pattern being the most valuable CT findings.

Angiolipoleiomioma uterino: presentación de un caso y revisión de la literatura / Uterine angiolipoleiomyoma: case presentation and review of the literature

Objetivo: presentar el caso de una paciente con angiolipoleiomioma uterino confirmado histopatológicamente y diagnosticado prequirúrgicamente como un teratoma anexial, y realizar una revisión de la literatura respecto al diagnóstico clínico, imagenológico e histopatológico de la entidad. Materiales y métodos: se presenta el caso de una mujer de 61 años, a quien se le practicó laparotomía por sospecha de un teratoma anexial. En la cirugía se hace diagnóstico de miomatosis uterina y se practica histerectomía abdominal total y salpingooforectomía bilateral. En el estudio histopatológico se encuentran hallazgos compatibles con angiolipoleiomioma uterino. Se realizó una búsqueda en las bases de datos Medline vía PubMed, OVID y en Lilacs, con los términos "angiolipoleiomyoma", "angiolipoleiomyoma uterus", "uterine angiolipoleiomyoma","uterine angiolipoleiomyomas" y "angiomyolipoma uterus", se buscaron artículos de revisión, reportes y series de casos en inglés y español publicados desde 1966 hasta mayo de 2016. Resultados: se encontraron siete artículos, que corresponden a reportes de casos y revisión de la literatura, los cuales describen las principales características en cuanto a presentación clínica, diagnóstico clínico y paraclínicos basándose en pruebas de imagenología y estudios de histopatología. Conclusión: el angiolipoleiomioma uterino es una entidad benigna muy rara, cuyo diagnóstico diferencial clínico es complejo, ya que simula otras patologías ginecológicas. Es de suma importancia la sospecha clínica prequirúrgica de esta entidad y la posterior confirmación histopatológica.

Objectives: To present the case of a patient with a uterine angiolipoleiomyoma confirmed by histopathology, diagnosed as adnexal teratoma before surger y, and to conduct a review of the literature regarding clinical, imaging and histopathological diagnosis of this condition. Materials and methods: A 61-year old patient taken to laparotomy due a suspected adnexal teratoma. During surgery, uterine myomatosis was diagnosed and total abdominal hysterectomy and bilateral oophorectomy are performed. Findings on histopatholog y were consistent with uterine angiolipoleiomyoma. A search was conducted in the Medline databases via PubMed, Ovid and in LILACS using the terms "angiolipoleiomyoma", "angiolipoleiomyoma uterus", "uterine angiolipoleiomyoma", "uterine angiolipoleiomyomas" and "angiomyolipoma uterus". The search included review articles and case series and reports published in English and Spanish from 1966 to May 2016. Results: Seven study articles were found, including case reports and literature reviews describing the main characteristics in terms of clinical presentation, clinical diagnosis and paraclinical tests, based on imaging studies and histopathology. Conclusion: Uterine angiolipoleiomyoma is an extremely rare benign condition that mimics other gynaecological pathologies, making it a difficult clinical differential diagnosis. Preoperative clinical suspicion and postoperative histopathological confirmation are of the utmost importance.

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.

Multicentric angiomyolipoma in kidney, liver, and lymph node: case report/review of the literature / Angiomiolipoma multicêntrico em rim, fígado e linfonodo: relato de caso/revisão da literatura

ABSTRACT Introduction: The angiomyolipoma (AML) is constituted by adipose tissue, blood vessels and smooth muscle fiber; follows a benign clinical course, with slow growth and absence of metastasis, occurring in tuberous sclerosis or sporadically. Objective: We intend to describe the clinical, radiological and histological aspects of this tumor. Case report: A-64-year-old woman presented with abdominal pain and dyspepsia. Tomography showed hepatic, renal and mesenteric nodules. Histological evaluation of mesenteric lymph node revealed perivascular epithelioid differentiation neoplasia, compatible with AML/perivascular epithelioid cell tumor (PEComa), positive for anti-S100, anti-human melanoma black-45 (HMB-45) and anti-smooth muscle actin. Conclusion: We described a rare AML with renal, hepatic and lymph node involvement, representing a multicenter version instead of metastasis. .

RESUMO Introdução: O angiomiolipoma (AML) constitui-se de tecido adiposo, vasos sanguíneos e fibras musculares lisas; tem curso clínico benigno, crescimento lento e ausência de metástases, ocorrendo no complexo esclerose tuberosa ou esporadicamente. Objetivos: Objetivamos descrever aspectos clinicorradiológicos e histológicos desse tumor Relato do caso: Paciente do sexo feminino, 64 anos, com dor abdominal e dispepsia. Tomografia mostrou nódulos hepáticos, renais e mesentéricos. Exame histopatológico de linfonodo mesentérico evidenciou neoplasia com diferenciação epitelioide perivascular condizente com AML/neoplasias de células epitelioides perivasculares (PEComa), positiva para anti-S100, anti-human melanoma black-45 (HMB-45) e antiactina de músculo liso. Conclusão: Descrevemos raro envolvimento simultâneo renal, hepático e linfonodal por AML, representando versão multicêntrica, e não doença metastática. .

Neoplasias hepáticas benignas: variantes histológicas, problemas diagnósticos y diagnóstico diferencial / Diagnosis and Differential Diagnosis Problems with Histological Variants of Benign Liver Neoplasms

Un principio básico de la patología es que las neoplasias se diferencian según sus células de origen y en el hígado semejan sus constituyentes, sean las células hepáticas, del epitelio biliar, endoteliales, mesenquimales o una combinación de estas. Es importante recordar aquí que son las metástasis el tumor maligno más frecuente del hígado, con una relación de 30:1 en pacientes sin enfermedad hepática crónica o cirrosis subyacente; es rara la presencia de las mismas en hígados cirróticos. Las neoplasias gastrointestinales del colon, páncreas, vía biliar extrahepática, estómago, tumores neuroendocrinos y GIST, o extraintestinales del pulmón, mama, melanoma o tumores de cabeza y cuello, son las más frecuentes (1). En este artículo solo revisaremos las más frecuentes. Iniciaremos con las neoplasias benignas y las lesiones pseudotumorales haciendo especial énfasis en aquellas con dificultades diagnósticas, en la utilidad de estudios especiales de inmunohistoquímica o moleculares para su adecuada clasificación y diagnóstico diferencial.

A basic principle of pathology is that neoplasms differ according to their cells of origin. Neoplasms of the liver resemble its constituent liver, biliary, epithelial, endothelial, mesenchymal cells or some combination of these different types of cells. It is important to remember here that metastases are the most frequent malignant liver tumor occurring at ratio of 30: 1 in patients without underlying chronic liver disease or cirrhosis. Metastases are rare in cirrhotic livers. The most common primary sites are the colon, pancreas, common bile duct, stomach, neuroendocrine tumors and GIST, or extraintestinal tumors from the lung, breast, head, neck and skin (1). In this article we review only the most frequent benign neoplasms and tumor-like lesions with particular emphasis on diagnostic difficulties, special studies, and the usefulness of immunohistochemical or molecular studies for proper classification and differential diagnosis.