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

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

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.

Tumores que invaden la vena cava inferior: revisión pictográfica de los principales hallazgos imagenológicos en tomografía computarizada y resonancia magnética / Tumours that invade the inferior vena cava: an illustrative review of the main imaging features on computed tomography and magnetic resonance

Abstract. The objective of this article is to describe the multiple entities that can affect and invade the inferior vena cava. These can be either benign entities, but with an aggressive behaviour, or malignancies that originate in the organs adjacent to this vascular structure, or in itself as a leiomyosarcoma. The present review shows different examples in Computed Tomography and Magnetic Resonance, with cases of angiomyolipoma, phaeochromocytoma, adrenal cortical carcinoma, renal cell carcinoma, hepatocellular carcinoma, a retroperitoneal sarcoma and a leiomyosarcoma originating in the inferior vena cava. Besides these, there are situations that may lead to diagnostic errors, such as the flow artefacts and pseudolipoma.

El objetivo de este artículo es describir las múltiples entidades que pueden afectar e invadir la vena cava inferior. Entre ellas podemos enumerar tanto entidades benignas, pero con un comportamiento agresivo, como procesos malignos que se originan en órganos adyacentes a esta estructura vascular o en ella misma como el leiomiosarcoma. En la presente revisión se exponen diferentes ejemplos en tomografía computarizada y resonancia magnética con casos de angiomiolipoma, feocromocitoma, carcinoma suprarrenal, carcinoma de células renales, hepatocarcinoma, sarcoma retroperitoneal y leiomiosarcoma originado en la vena cava inferior. Además se exponen situaciones que pueden inducir a errores diagnósticos como los artefactos de flujo y el pseudolipoma.

Esclerosis tuberosa, diagnóstico fetal y materno / Tuberous sclerosis, fetal and maternal diagnosis

La esclerosis tuberosa (ET) es una enfermedad genética, autosómica dominante que tiene expresividad variable y que se caracteriza por la presencia de hamartomas en múltiples órganos de diferentes sistemas (piel, cerebro y corazón). Es causada por mutaciones en los genes TSC1 locus 9q34 y TSC2 locus 16p13. Tiene una prevalencia de 1 en cada 5000 a 10000 recién nacidos vivos. Se reporta el caso de una gestante y su feto con diagnóstico de ET. Se observaron masas en corazón y cerebro en el feto. El examen físico exhaustivo de la embarazada mostró criterios mayores de la enfermedad. Los hallazgos del diagnóstico prenatal fueron confirmados en el recién nacido. Se contribuye a la construcción de datos epidemiológicos latinoamericanos, y se aporta un plan de manejo a fetos, embarazadas y recién nacidos con ET.

Tuberous Sclerosis (TS) is a genetic disorder, exhibits an autosomal dominant inheritance pattern with variable expression, characterized by the presence of hamartomas in multiple organs of various systems (skin, brain and hearth). It is caused by mutations in genes TSC1 loci 9q34 and TSC2 loci 16p13. It has a prevalence of 1 in every 5000 to 10000 live births. We report a case of a pregnant and her fetus with diagnosis of TS. Masses in heart and brain in the fetus were observed, the thorough physical examination of pregnant also showed major criteria of the disease. Prenatal diagnosis findings were confirmed in the newborn. The objective is contribute to the construction of Latin American epidemiological data, a management plan for fetuses, pregnant women and infants with TS is provided.

Diagnostic Efficacy of Contrast-Enhanced Ultrasound for Small Renal Masses

PURPOSE: Ultrasound (US) is highly sensitive in the detection of renal masses. However, it may not be able to differentiate benign and malignant lesions in smaller masses. The purpose of this study was to determine the diagnostic efficacy of contrast-enhanced ultrasound (CEUS) for small renal masses. MATERIALS AND METHODS: From January 2011 to December 2013, a total of 85 patients underwent CEUS for evaluation of renal masses. Of these patients, CEUS findings were retrospectively analyzed for small renal cell carcinoma (RCC) cases (n=38) and angiomyolipoma (AML) cases (n=11). The tumor echogenicity and enhancement patterns and degrees were evaluated. The diagnostic efficacy of CEUS in differentiating the two diseases was compared. RESULTS: On CEUS, the findings of diffuse heterogeneous enhancement (observed in 78.9% of RCCs and 27.3% of AMLs, p=0.003), washout from hyperenhancement or iso-enhancement to hypoenhancement in late phase (73.7% of RCCs and 18.2% of AMLs, p=0.001), and perilesional rim-like enhancement (57.9% of RCCs and 9.1% of AMLs, p=0.006) were significantly different between AML and RCC cases. The corresponding sensitivity, specificity, positive predictive value, negative predictive value, and accuracy were 86.8% (33/38), 63.6% (7/11), 89.2% (33/37), 58.3% (7/12), and 81.6% (40/49), respectively. CONCLUSIONS: Our results suggest that the characteristic CEUS features could have diagnostic value in the evaluation of small renal mass. CEUS showed a higher diagnostic efficacy than conventional US for differentiating RCC and AML.

Diagnostic Efficacy of Contrast-Enhanced Ultrasound for Small Renal Masses

PURPOSE: Ultrasound (US) is highly sensitive in the detection of renal masses. However, it may not be able to differentiate benign and malignant lesions in smaller masses. The purpose of this study was to determine the diagnostic efficacy of contrast-enhanced ultrasound (CEUS) for small renal masses. MATERIALS AND METHODS: From January 2011 to December 2013, a total of 85 patients underwent CEUS for evaluation of renal masses. Of these patients, CEUS findings were retrospectively analyzed for small renal cell carcinoma (RCC) cases (n=38) and angiomyolipoma (AML) cases (n=11). The tumor echogenicity and enhancement patterns and degrees were evaluated. The diagnostic efficacy of CEUS in differentiating the two diseases was compared. RESULTS: On CEUS, the findings of diffuse heterogeneous enhancement (observed in 78.9% of RCCs and 27.3% of AMLs, p=0.003), washout from hyperenhancement or iso-enhancement to hypoenhancement in late phase (73.7% of RCCs and 18.2% of AMLs, p=0.001), and perilesional rim-like enhancement (57.9% of RCCs and 9.1% of AMLs, p=0.006) were significantly different between AML and RCC cases. The corresponding sensitivity, specificity, positive predictive value, negative predictive value, and accuracy were 86.8% (33/38), 63.6% (7/11), 89.2% (33/37), 58.3% (7/12), and 81.6% (40/49), respectively. CONCLUSIONS: Our results suggest that the characteristic CEUS features could have diagnostic value in the evaluation of small renal mass. CEUS showed a higher diagnostic efficacy than conventional US for differentiating RCC and AML.

FDG PET or PET/CT in Evaluation of Renal Angiomyolipoma

OBJECTIVE: Angiomyolipoma is the most common benign kidney tumor. However, literature describing FDG PET findings on renal angiomyolipoma (AML) is limited. This study reports the FDG PET and PET/CT findings of 21 cases of renal AML. MATERIALS AND METHODS: The study reviews FDG PET and PET/CT images of 21 patients diagnosed with renal AML. The diagnosis is based on the classical appearance of an AML on CT scan with active surveillance for 6 months. The study is focused on the observation of clinical and radiographic features. RESULTS: Six men and 15 women were included in our study. The mean age of the patients was 57.14 +/- 9.67 years old. The mean diameter of 21 renal AML on CT scans was 1.76 +/- 1.00 cm (Min: 0.6 cm; Max: 4.4 cm). CT scans illustrated renal masses typical of AMLs, and the corresponding FDG PET scans showed minimal FDG activities in the area of the tumors. None of the 21 AMLs showed a maximum standardized uptake value (SUVmax) greater than 1.98. No statistically significant correlation was present between SUVmax and tumor size. CONCLUSION: Renal AMLs demonstrate very low to low uptake on FDG PET and PET/CT imaging in this study. When a fat-containing tumor in the kidney is found on a CT scan, it is critical to differentiate an AML from a malignant tumor including an RCC, liposarcoma, and Wilms tumor. This study suggests that FDG PET or PET/CT imaging is useful for differentiating a renal AML from a fat-containing malignant tumor.

Angiomyolipoma : a report of two cases

Objective: We report two cases of unusual renal tumours in two Nigerian adults. The first case was in a 45 year old female; who presented with an insidious onset of right upper abdominal pain with right abdominal swelling. The second case was a 33 year old pregnant lady; presenting with three week history of right lumbar pain and an accompanying mass. The various modes of radiological investigations and their significance in making a prompt diagnosis are highlighted. Method: Abdominal ultrasound scan; intravenous urography and CT scan confirmed the presence of renal masses in both cases and the suspicion of renal malignancies were raised clinically. They both had nephrectomy. Results : Histology report of the masses revealed angiomyolipoma (AML). Conclusions: In a period of three years; out of twenty four solid renal tumours operated upon in the Lagos State University Teaching Hospital (LASUTH); two were unusual. All the others were renal cell carcinoma. All diagnosis were confirmed histologically