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RESUMO

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.

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RESUMO

Renal angiomyolipoma is a benign tumor, composed of adipocytes, smooth muscle cells and blood vessels. The association with pregnancy is rare and related with an increased risk of complications, including rupture with massive retroperitoneal hemorrhage. The follow-up is controversial because of the lack of known cases, but the priorities are: timely diagnosis in urgent cases and a conservative treatment when possible. The mode of delivery is not consensual and should be individualized to each case. We report a case of a pregnant woman with 18 weeks of gestation admitted in the emergency room with an acute right low back pain with no other symptoms. The diagnosis of rupture of renal angiomyolipoma was established by ultrasound and, due to hemodynamically stability, conservative treatment with imaging and clinical monitoring was chosen. At 35 weeks of gestation, it was performed elective cesarean section without complications for both mother and fetus.

Assuntos

RESUMO

Renal angiomyolipoma is a benign tumor, composed of adipocytes, smooth muscle cells and blood vessels. The association with pregnancy is rare and related with an increased risk of complications, including rupture with massive retroperitoneal hemorrhage. The follow-up is controversial because of the lack of known cases, but the priorities are: timely diagnosis in urgent cases and a conservative treatment when possible. The mode of delivery is not consensual and should be individualized to each case. We report a case of a pregnant woman with 18 weeks of gestation admitted in the emergency room with an acute right low back pain with no other symptoms. The diagnosis of rupture of renal angiomyolipoma was established by ultrasound and, due to hemodinamically stability, conservative treatment with imaging and clinical monitoring was chosen. At 35 weeks of gestation, it was performed elective cesarean section without complications for both mother and fetus.

O angiomiolipoma é um tumor benigno, constituído por adipócitos, células de músculo liso e vasos sanguíneos. Sua associação com a gravidez é rara e está relacionada com um aumento de complicações, nomeadamente rotura com hemorragia retroperitoneal maciça. O follow-up é controverso em razão do escasso número de casos descritos, no entanto as prioridades são: diagnóstico atempado nas situações urgentes e, sempre que possível, tratamento conservador. O tipo de parto não é consensual e deve ser individualizado caso a caso. Relatamos um caso de uma grávida com 18 semanas de gestação que recorreu ao serviço de urgência por lombalgia direita aguda, sem outros sintomas relevantes. Diagnosticou-se ecograficamente rotura de angiomiolipoma renal e, em decorrência da estabilidade hemodinâmica do quadro, procedeu-se ao tratamento conservador com monitorização imagiológica e clínica. Às 35 semanas de gestação, realizou-se uma cesariana eletiva que decorreu sem complicações maternas ou fetais.

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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.

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Renal angiomyolipomas are uncommon tumours during pregnancy, but it's important to consider them in the differential diagnosis, for the increased risk of complications such as bleeding, specially during pregnancy. Their characteristics are different depending on if the tumour is associated or not with facomatosis. To show both possibilities, we describe two cases of pregnant women with angiomyolipomas: the first one presented with bleeding of the tumour (Wünderlich's syndrome), but surveillance without treatment was possible until 37th week of gestation, and embolization of the tumour was performed afterwards. The second patient had multiple well known angiomyolipomas, associated to tuberous sclerosis; even though she had required two previous embolizations, she had an uneventful pregnancy, without any episodes of bleeding. Both pregnancies ended successfully by means of a cesarean section. Since there are few papers available in the literature, there is not an agreement about therapeutic management when pregnant women present with bleeding angiomyolipomas (Wünderlich's syndrome). Treatment options include partial or total nephrectomy (with cesarean section simultaneously in patients at 28 weeks of gestation or later), transcateter selective arterial embolization (which can also be preventive), and sometimes, conservative management. If this last option is possible, there's still discussion about the risk of vaginal delivery compared with that of cesarean section, in terms of bleeding of the tumour.

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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.

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RESUMO

Angiomyolipoma (AML) is a benign clonal neoplasm that consists of varying amounts of mature adipose tissue, smooth muscle, and thick-walled vessels. Approximately 20% of AMLs are found in patients with tuberous sclerosis syndrome (TS), an autosomal-dominant disorder characterized by mental retardation, epilepsy, and adenoma sebaceous, a distinctive skin lesion. Massive retroperitoneal hemorrhage from AML, also known as Wunderlich's syndrome, has been found in up to 10% of patients and represents the most significant and feared complication. The presence of even a small amount of fat within a renal lesion on CT scan (confirmed by Hounsfield unit's < or = 10) is considered diagnostic of AML. Intervention should be considered for larger tumors, particularly if the patient is symptomatic, taking into account patient age, comorbidities, and other related factors. A nephron-sparing approach, via either partial nephrectomy or selective embolization, is clearly preferred. We present the case of a fifty-nine-year old patient with an angiomyolipoma and massive retroperitoneal hemorrhage with Lenk syndrome.

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Renal angiomyolipoma is a benign tumor formed by smooth muscle, adipose tissue and blood vessels. It is commonly found incidentally and its clinical manifestations are pain and abdominal mass or spontaneous tumor rupture with retroperitoneal bleeding. The clinical presentation of a hemorrhagic shock secondary to a retroperitoneal hematoma is uncommon. We report a 40 year-old male who presented to the emergency room with lumbar pain and deterioration of hemodynamic parameters. The CT scan showed a left renal injury associated to an expansive retroperitoneal process. The abdominal exploration, vascular control of the renal pedicle and nephrectomy allowed a successful outcome.

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