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ObjectiveTuberous sclerosis complex (TSC) is a rare autosomal dominant genetic disease involving multiple organ system. Renal angiomyolipoma (RAML) is a leading cause of death for adult TSC patients. Our study aims at investigating the clinical manifestations of TSC-RAML to enable clinicians to have a better understanding of the disease in Chinese patients.MethodsWe reviewed retrospectively the data collected from patients with TSC-RAML in Peking Union Medical College Hospital from January 2014 to January 2023. We also collected information about the age, gender, imageological examination, RAML stage, and comorbidities.ResultsA total of 186 TSC-RAML patients were registered, 65 of whom were males and 121 were females(male-to-female ratio 1∶1.86). The median age of all patients was 31 years old. Totally, 117 cases (62.9%)of RAML were rated stage 6. Twenty-two cases (11.8%) had a history of tumor rupture and bleeding. Research shows statistical differences between high and low grade on RAML rupture bleeding(P=0.0475). Angiofibromas/fibrous cephalic plaque (155/186, 83.3%), subependymal nodules(103/146, 70.5%), lymphangioleiomyomatosis (102/157, 65.0%), hypomelanotic macules (114/186, 61.3%), and shagreen patch (83/186, 44.6%)were the most common clinical manifestations. All patients with lymphangioleiomyomatosis(LAM) were female(P < 0.0001).ConclusionsFemale patients predominated TSC-RAML patients. Most RAML were in stage 6. About 11.8% cases had a history of tumor rupture and hemorrhage. The higher grade meant the higher proportion of tumor rupture and hemorrhage. Dermatological and nervous lesions were the most common comorbidities. All patients with LAM in this study were female.
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Tuberous sclerosis complex(TSC) is a rare autosomal dominant genetic disorder caused by mutations in the TSC1 or TSC2 genes. The disease can cause multiple organ lesions. The most common renal lesions are renal angiomyolipoma(RAML). In recent years, China has made progresss in the understanding of TSC-RAML, so that the standardization of the diagnosis and management of TSC-RAML have improved. Efforts have been taken in the studies of the pathogenesis and treatment of TSC-RAML. This article reviews the diagnosis, therapy, and research development of TSC-RAML in China.
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Tuberous sclerosis complex (TSC) is an autosomal dominant hereditary disease that affects multiple organs and systems throughout the body. TSC-associated kidney disease is the leading cause of death in adult TSC patients. This article retrospectively analyzed the characteristics of one TSC-related renal giant angiomyolipoma(RAML)treated with surgery. The patient, 25 years old, was diagnosed with tuberous sclerosis complex in 2000 due to multiple maculopapular rashes on both cheeks. At a regular follow-up in July 2019, imaging examinations revealed a tumor in the left lower quadrant with a maximum cross-sectional area of 16 cm×7 cm. Genetic testing showed a loss of heterozygosity in the EX18_ 41 of TSC2. After the diagnosis was confirmed, open left partial nephrectomy was performed, during which multiple tumors were found on the kidney surface and the largest one was located on the ventral side with a diameter of approximately 20 cm. After the renal artery was occluded, kidney tumors were completely enucleate. Postoperative pathological confirmed the diagnosis of angiomyolipoma. This case provides a reference for the treatment of TSC-related renal giant hamartoma.
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Renal angiomyolipomas (AMLs), formerly known as PEComas (tumors showing perivascular epithelioid cell differentiation) are common benign renal masses composed of a varying ratio of fat, blood vessels, and smooth muscles. They are largely asymptomatic and diagnosed incidentally on imaging. The adipose tissue content is the factor that gives AMLs their characteristic appearance on imaging and makes them easily identifiable. However, the fat-poor or fat-invisible varieties, which are difficult to differentiate radiologically from renal cell carcinomas (RCCs), present a diagnostic challenge. It is thus essential to establish the diagnosis and identify the atypical and hereditary cases as they require more intense surveillance and management due to their potential for malignant transformation. Multiple management options are available, ranging from conservative approach to embolization and to the more radical option of nephrectomy. While the indications for intervention are relatively clear and aimed at a rather small cohort, the protocol for follow-up of the remainder of the cohort forming the majority of cases is not well established. The surveillance and discharge policies therefore vary between institutions and even between individual practitioners. We have reviewed the literature to establish an optimum management pathway focusing on the typical AMLs.
Los angiomiolipomas renales (AML), antes conocidos como PEComas (tumores que muestran epitelioides perivasculares) son masas renales benignas frecuentes compuestas por una proporción variable de grasa, vasos sanguíneos y músculos lisos. Suelen ser asintomáticos y se diagnostican de forma incidental en las pruebas de imagen. El contenido de tejido adiposo es el factor que confiere a los AML su aspecto característico en las imágenes y los hace fácilmente identificables. Sin embargo, las variedades pobres en grasa o invisibles, que son difíciles de diferenciar radiológicamente de los carcinomas de células renales (CCR), suponen un reto diagnóstico. Por lo tanto, es esencial establecer el diagnóstico e identificar los casos atípicos y hereditarios, ya que requieren una vigilancia y un tratamiento más intensos debido a su potencial de malignización. debido a su potencial de transformación maligna. Existen múltiples opciones de tratamiento, que van desde el enfoque conservador hasta la embolización y la opción más radical de la nefrectomía. Si bien las indicaciones para la intervención son relativamente claras y están dirigidas a una cohorte bastante pequeña, el protocolo para el seguimiento del resto de la cohorte que forma la mayoría de los casos no está bien establecido. Por lo tanto, las políticas de vigilancia y alta varían entre instituciones e incluso entre profesionales individuales. Hemos revisado la literatura para establecer una ruta de manejo óptima centrada en los AML típicos.
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Humans , Carcinoma, Renal Cell , Clinical Protocols , Angiomyolipoma , Perivascular Epithelioid Cell Neoplasms , Therapeutics , Epithelioid Cells , NephrectomyABSTRACT
Objective:To comprehensively analyze the expression profile of circular RNA (circRNA) and construct competing endogenous RNA (ceRNA) regulatory networks in tuberous sclerosis complex related renal angiomyolipoma (TSC-RAML).Methods:According to the diagnostic criteria of TSC determined by the international consensus group on tuberous sclerosis in 2012, tumor tissues and paired normal renal tissues of 3 patients with TSC-RAML who were diagnosed in our hospital from January 2017 to January 2019 were collected. The circRNA, miRNA and mRNA of 3 paired samples were detected by circRNA, miRNA chip technology and next generation sequencing respectively, and the differential molecules were determined. Gene Ontology(GO) functional enrichment analysis and Kyoto Encyclopedia of genes and genomes(KEGG) pathway enrichment analysis were performed based on differential mRNA molecules and host genes of circRNA. Based on differential circRNA, miRNA and mRNA, up-regulated and down-regulated ceRNA regulatory networks were established.Results:A total of 330 up-regulated and 336 down-regulated differential circRNA, 8 up-regulated and 7 down-regulated miRNA, 800 up-regulated and 1130 down-regulated mRNA were screened. Through GO and KEGG enrichment analysis, many pathways including lipid metabolism, focal adhesion and mineral absorption were abnormally altered. Finally, the up-regualted ceRNA network led by hsa_circ_0092022, hsa_circ_0076859 and hsa_circ_0033388 and down-regulated network led by hsa_circ_0000374, hsa_circ_0000141, hsa_circ_0072665, hsa_circ_0009503 and hsa_circ_0000009 were constructed.Conclusions:There were many differentially expressed circRNA between TSC-RAML and paired normal renal tissues. ceRNA regulatory networks may be involved in the occurrence and development of TSC-RAML.
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Objective@#To explore the clinical characteristics of renal angiomyolipoma (AML) with inferior vena cava (IVC) tumor thrombus and to improve the diagnosis and treatment of the disease.@*Methods@#The clinical data of 3 patients with renal AML and inferior vena cava tumor thrombus was retrospectively reviewed. The patients were all female, aged 19 to 70 years. Among them, 2 patients presented with lumbago on the right side, and the other one was diagnosed by physical examination. The body mass index ranged from 18.4 to 24.6 kg/m2, with a median value of 20.4 kg/m2. According to the American Society of Anesthesiologists (ASA), they were classified as grade Ⅱ. Color doppler ultrasound examination of the kidney and IVC was performed in all the 3 patients, all of which showed hyperechoic solid mass in the right kidney. Color doppler ultrasound of IVC showed hyperechoic band in the IVC, indicating blood flow signals and the tumor thrombus. All the 3 cases showed irregular fat density or mixed density in the right kidney and multiple irregular fat density were observed in the right renal vein and inferior vena cava on CT. Two of them received MRI examination of IVC, which showed irregular lesions in the right kidney, short T1 and long T2 signals, low lipids, and no definite limited diffusion on DWI. Irregular fat signal were seen in the right renal vein and inferior vena cava. All 3 patients were diagnosed with right renal mass with IVC tumor thrombus, with 1 patient of Mayo grade Ⅲ tumor thrombus and the other 2 of Mayo gradeⅡtumor thrombus. One underwent laparoscopic radical nephrectomy and inferior vena cava tumor thrombectomy, another one underwent open right partial nephrectomy and tumor thrombectomy, and the third one suffered preoperative AML rupture, undergoing open radical nephrectomy and tumor thrombectomy.@*Results@#The operation time was 168 to 659 min, with median of 220 min. Intraoperative blood loss ranged from 50 to 300 ml, with the median of 50 ml. Postoperative indwelling time of drainage tube was 5 to 11 days, with the median of 6 days. Postoperative hospital stay ranged from 7 to 14 days, with a median of 8 days. Postoperative follow-up ranged from 12 to 16 months, with a median follow-up of 13 months. All the three patients underwent operation without postoperative complications. Postoperative pathology proved to be right renal angiomyolipoma. After 3 months of follow-up, the patients showed no tumor recurrence or metastasis.@*Conclusions@#Renal AML is a benign lesion, which is rarely concurrent with inferior vena cava cancer thrombus. Enhanced CT examination is the main diagnostic method, surgical resection of the lesion is the preferred treatment, partial nephrectomy combined with thrombectomy can be performed in patients with AML, if permitted, and postoperative prognosis turns out to be propitious.
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Objective To explore the clinical efficacy of laparoscopic tumor resection combined with aspiration for central renal angiomyolipoma(RAML).Methods Retrospective analysis of clinical data of 11 patients with central RAML between November 2015 and August 2018,including 8 females and 3 males,aged 35-58 years,mean (45.3 ± 4.8) years;4 cases on the left side,7 cases on the right side,including 4 endophytic tumors,7 exophytic tumors;body mass index (BMI) 22-27 kg/m2,mean (24.9 ±1.8) kg/m2.The maximum diameter of these tumors were 4.2-8.9 cm,with an average of (6.7 ± 2.4)cm.The preoperative serum creatinine was 53-85 μmol/L,with an average of (64.6 ± 16.4) μmol/L,and the glomerular filtration rate (GFR) of the affected side was 42-55 ml/min,with an average of (45.5 ±3.6) ml/min.Lateral kidney function is normal.8 patients underwent retroperitoneal laparoscopic surgery and 3 cases underwent laparoscopic surgery.Based on the preoperative imaging,tumor tissue was aspirated as much as possible by suction device if the tumor got maximal fat component and had not abundant blood supply.Clamp the renal artery in case of bleeding affected the surgery vision.On the contrary,if the tumor had abundant blood supply,the renal artery was clamped first,then removed the section of tumor with scissor and the remaining basal tumor was aspirated totally by suction device.The operation time,warm ischemia time,intraoperative blood loss,postoperative hospital stay,postoperative complications and surgical results were analyzed.Results All the 11 cases were successfully completed without conversion to open surgery,only one case got injury of renal collection system.The operation time was 95-126 min,mean (110.5 ± 12.8) min,and the blood loss was 120-200 ml,mean (155.6 ± 38.4) ml.The warm ischemia time was 22-31 min,mean (27.6 ± 3.3) min,and the postoperative hospital stay was 5-7 days.RAMLs were diagnosed by the postoperative pathological examination.The renal function was normal and no recurrence occurred in these patients at a mean follow-up of 13.9 months.All the 11 cases were successfully completed.Postoperative pathological diagnosis was RAML.There was no complication in the 11 cases.Preoperative serum creatinine and serum creatinine in the first month after surgery [(64.6 ± 16.4)μmol/L vs.(66.7 ± 15.5)tμmol/L,P =0.38] and preoperative renal GFR and GFR in the first month after surgery [(45.5 ± 3.6)ml/min vs.(43.2 ± 3.3) ml/min,P =0.067],the difference was not statistically significant.No recurrence was observed in the 11 cases with an average follow-up of 13.9 months.Conclusions Our initial experience suggests that laparoscopic tumor resection combined with aspiration is a safe,effective and minimally invasive method for the treatment of central RAML.
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Objective To explore the clinical characteristics of renal angiomyolipoma (AML) with inferior vena cava (IVC) tumor thrombus and to improve the diagnosis and treatment of the disease.Methods The clinical data of 3 patients with renal AML and inferior vena cava tumor thrombus was retrospectively reviewed.The patients were all female,aged 19 to 70 years.Among them,2 patients presented with lumbago on the right side,and the other one was diagnosed by physical examination.The body mass index ranged from 18.4 to 24.6 kg/m2,with a median value of 20.4 kg/m2.According to the American Society of Anesthesiologists (ASA),they were classified as grade Ⅱ.Color doppler ultrasound examination of the kidney and IVC was performed in all the 3 patients,all of which showed hyperechoic solid mass in the right kidney.Color doppler ultrasound of IVC showed hyperechoic band in the IVC,indicating blood flow signals and the tumor thrombus.All the 3 cases showed irregular fat density or mixed density in the right kidney and multiple irregular fat density were observed in the right renal vein and inferior vena cava on CT.Two of them received MRI examination of IVC,which showed irregular lesions in the right kidney,short T1 and long T2 signals,low lipids,and no definite limited diffusion on DWI.Irregular fat signal were seen in the right renal vein and inferior vena cava.All 3 patients were diagnosed with right renal mass with IVC tumor thrombus,with 1 patient of Mayo grade Ⅲ tumor thrombus and the other 2 of Mayo grade Ⅱ tumor thrombus.One underwent laparoscopic radical nephrectomy and inferior vena cava tumor thrombectomy,another one underwent open right partial nephrectomy and tumor thrombectomy,and the third one suffered preoperative AML rupture,undergoing open radical nephrectomy and tumor thrombectomy.Results The operation time was 168 to 659 min,with median of 220 min.Intraoperative blood loss ranged from 50 to 300 ml,with the median of 50 ml.Postoperative indwelling time of drainage tube was 5 to 11 days,with the median of 6 days.Postoperative hospital stay ranged from 7 to 14 days,with a median of 8 days.Postoperative follow-up ranged from 12 to 16 months,with a median follow-up of 13 months.All the three patients underwent operation without postoperative complications.Postoperative pathology proved to be right renal angiomyolipoma.After 3 months of follow-up,the patients showed no tumor recurrence or metastasis.Conclusions Renal AML is a benign lesion,which is rarely concurrent with inferior vena cava cancer thrombus.Enhanced CT examination is the main diagnostic method,surgical resection of the lesion is the preferred treatment,partial nephrectomy combined with thrombectomy can be performed in patients with AML,if permitted,and postoperative prognosis turns out to be propitious.
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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)
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Humans , Female , Adult , Angiomyolipoma/pathology , Kidney Neoplasms/pathology , Nephrectomy , Urologic Diseases , Internal MedicineABSTRACT
Objective To differentiate the renal angiomyolipoma with minimal fat (mf-RAML) from clear cell renal cell carcinoma (ccRCC) by Logistic regression analysis and CT quantitative analysis.Methods 25 patients with mf-RAML and 62 patients with ccRCC were collected and performed with enhanced CT scan.The CT signs,CT attenuation value and CT relative attenuation value were calculated and recorded.Results Pseudocapsule and enhancement pattern (OR=0.061,7.445,P=0.005,0.034) were the independent variables of Logistic regression equation.There were statistical difference between the unenhanced muscle％,CT relative attenuation value in renal cortical phase,CT attenuation value and CT relative attenuation value in renal medulla phase and CT relative attenuation value of the mf-RAML and those of the ccRCC (P =0.000-0.040).In addition,the two diseases had low positive correlation with the unenhanced muscle％ and CT attenuation value and CT relative attenuation value in renal medulla phase (r =0.221-0.343,P =0.001-0.040),and had low to middle negative correlation with CT relative attenuation value in renal cortical phase (r =-0.512,-0.225,P =0.000,0.036).The areas under the ROC curve of unenhanced muscle％ and CT relative attenuation value in renal medulla phase were relatively high (0.665-0.753).Conclusion Pseudocapsule and enhancement pattern were independent factors in differentiating mf-RAML from ccRCC.The CT attenuation value of the mf-RAML was similar to the erector spinae muscle.The enhanced degree of mf-RAML in renal cortical phase was lower than that of the ccRCC,while the reduced degree of the ccRCC was higher than that of the mf-RAML in renal medulla phase.The unenhanced muscle％ and the CT relative attenuation value in renal medulla phase are of great significance in diagnosing mf-RAML and ccRCC,especially the unenhanced muscle％.
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Objective To compare the CT findings of exophytic renal angiomyolipoma and perirenal liposarcoma.Methods CT data of 20 patients with exophytic renal angiomyolipoma and 20 pateints with perirenal liposarcoma were reviewed by two radiologists blinded to the clinical and pathological findings.These findings were compared between exophytic renal angiomyolipoma and perirenal liposarcoma using the Student t-test and Fisher 's exact test.Results Exophytic renal angiomyolipomas were smaller (P <0.05)and more commonly showed the following findings(P <0.05):renal parenchymal defect in 20 patietns,aneurysmal dilatation of intratumoral vessels in 18 patients,renal parenchymal vascular pedicle in 20 patients,supply of renal artery branches in 20 patients,hemorrhage in 6 patietns and combination with other intrarenal fatty lesions in 5 patients.Whereas,the findings associated with perirenal liposarcoma were non-fat soft tissue node in 20 patients (P <0.05)and kidney displacement in 19 patients (P <0.05).No significant difference was seen in calcification and sexuality(P >0.05).Conclusion Several CT findings were significantly different between exophytic renal angiomyolipoma and perirenal liposarcoma,which can be helpful for differentiating the two tumors,and provides the accurate imaging basis for the next therapy.
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Objective To compare the CT findings of exophytic renal angiomyolipoma and perirenal liposarcoma.Methods CT data of 20 patients with exophytic renal angiomyolipoma and 20 pateints with perirenal liposarcoma were reviewed by two radiologists blinded to the clinical and pathological findings.These findings were compared between exophytic renal angiomyolipoma and perirenal liposarcoma using the Student t-test and Fisher 's exact test.Results Exophytic renal angiomyolipomas were smaller (P <0.05)and more commonly showed the following findings(P <0.05):renal parenchymal defect in 20 patietns,aneurysmal dilatation of intratumoral vessels in 18 patients,renal parenchymal vascular pedicle in 20 patients,supply of renal artery branches in 20 patients,hemorrhage in 6 patietns and combination with other intrarenal fatty lesions in 5 patients.Whereas,the findings associated with perirenal liposarcoma were non-fat soft tissue node in 20 patients (P <0.05)and kidney displacement in 19 patients (P <0.05).No significant difference was seen in calcification and sexuality(P >0.05).Conclusion Several CT findings were significantly different between exophytic renal angiomyolipoma and perirenal liposarcoma,which can be helpful for differentiating the two tumors,and provides the accurate imaging basis for the next therapy.
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Objective To investigate the value of contrast-enhanced ultrasonography (CEUS) in the diagnosis of small renal cell carcinoma (SRCC) and renal angiomyolipoma (AML) with minimal fat.Methods The images and data of conventional ultrasound (US) and CEUS were retrospectively reviewed in 47 cases of small renal cell carcinoma and 8 cases of AML with minimal fat (d ＜ 30mm),which were confirmed by operation and pathologicall study,including 39 males and 16 females.The mean age of the patients was (54.8 ± 9.8) years old,ranged from 31 to 73 years old.The size,echo,boundary and color flow signals of renal lesions were observed by conventional US.Then the modality and phases of enhancement were observed,including the arrival time,the peak time,the washout time and the appearance of internal structures.Results On contrast-enhanced sonography,fast wash-in and wash-off were observed in most of SRCC,while slow wash-off were observed in most of RAML with minimal fat.The statistically significant differences were found between SRCC and RAML with minimal fat in the values of TFP (time a to peak) and PI (peak-intensity).The occurrence of round pseudo-capsule and contrast-enhancement characteristics in SRCC was far more often than RAML with minimal fat.Conclusions Contrast-enhanced sonography combined with time-intensity analysis provides more useful information for the diagnosis of SRCC and AML with minimal fat.
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Aims: The authors presents a case of pure Renal epithelioid angiomyolipoma (EAML) and discuss the clinicopathological characteristics and the diagnosis problems of this entity. Case Presentation: A 51 year-old man presented with a persistant pain in the left lumbar region. Imaging revealed a left kidney tumor that was hemorrhagic and necrotic on gross examination. At histology, proliferation of 99% of atypical epithelioid cells, staining for HMB-45 and Melan A was observed. Our patient had lung and right adrenal metastasis six months after surgery and succumbed one year later. Conclusion: In summary, the pure form of EAML is extremely rare. When diagnosed, the risk of tumor progression is high, especially if other histological predictive parameters of progression are observed. In that case, active treatment should be undergone and patients should be closely monitored.
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Renal angiomyolipoma is a rare disease seen during pregnancy. Rupture of renal angiomyolipoma could be catastrophic and might result in maternal and even perinatal mortality. Management includes conservative vs surgical approach. A 29-year-old woman Para 2 with history of bleeding renal angiomyolipoma in her first pregnancy at 11 weeks treated with selective arterial embolisation. The pregnancy was terminated. Even though having small residual tumour, her subsequent pregnancy progressed well with conservative management. Intervention is advisable in the presence of large or symptomatic renal angiomyolipoma prior to pregnancy in order to minimise potential lifethreatening haemorrhage during pregnancy. For those with small tumour of less than 4cm, perhaps conservative approaches i.e. frequent follow-up and close monitoring would assist in early identification of any rupture or bleeding.
Subject(s)
NephrectomyABSTRACT
Objective To investigate the characteristics of minimal fat renal angiomyolipoma (MFAML)and clear cell renal cell carcinoma(CCRCC)in high resolution multi-slice spiral CT(MSCT)and to improve the diagnosis accuracy for the renal tumors.Methods A retrospective analysis was performed on 24 MFAML patients(16 females,8 males)with mean age of 43(19-74)years and 24 CCRCC patients(16 females,8 males)with mean age of 44(21-76)years.All patients had undergone MSCT and proved histopathologically after surgery.The characteristics included tumor location,tumor attenuation on unenhanced CT,enhancement characteristics(degree of tumor enhancement in the early corticomedullary phase,homogeneity of enhancement,amount of enhancement,enhancement pattern over time),tumor margin,intratumoral calcification,and perinephric changes.The predictive value of each CT characteristic was determined by using multivariate logistic regression analysis.Results The tumor location in the kidney (upper pole:MFAML,6 cases,CCRCC,6 cases;middle:MFAML,7 cases,CCRCC,9 cases;lower pole:MFAML,11 cases,CCRCC,9 cases)and smooth tumor margin(MFAML,n=21;CCRCC,n=19)were not significantly different between MFAML patients and those with CCRCC,P＞0.05.Twenty-one cases of both MFAMLs and CCRCCs had the significant enhancement in the early corticomedullary phase,which were hypovascular tumors,whereas the mean amount of tumor enhancement was greater in CCRCC than in MFAML in both the early corticomedullary and the corticomedullary phases(CCRCC:175 HU,196 HU;MFAML:125 HU,145 HU;P＜0.05.MFAML usually showed homogeneous enhancement(n=15)rather than heterogeneous enhancement(n =9),whereas most CCRCC showed heterogeneous enhancement(n =17)rather than homogeneous enhancement(n =7),P＜0.05).Enhancement pattern was not a significant predictor.Within the 13 MFAML cases,8 cases had sufficient blood supply(6 cases showed obvious wash-in-and-wash-out,2 cases were with prolonged enhancement),5 cases with hypovascular showed a pattern of prolonged or gradual enhancement,while 21 CCRCC cases had sufficient blood supply and 71％ of them showed obvious wash-in-and-wash-out.High tumor attenuation on unenhanced scans(MFAML:17 patients (75％);CCRCC:2 patients(8％),P=0.002,OR=0.010)and threshold enhancement values of 129.5 HU in the corticomedullary phase(MFAML:5 patients(20％);CCRCC:20 patients(83％),P =0.004,OR =0.057)were valuable predictors for differentiating MFAML from CCRCC at multivariate logistic regression analysis.Conclusions MSCT is useful in differentiating MFAML from CCRCC,with high tumor attenuation on unenhanced scans and threshold enhancement values of 129.5 HU in the corticomedullary phase being the most valuable CT findings.75％ of MFAMLs with sufficient blood supply also show a pattern of wash-in-and-wash-out,which can easily misdiagnosed as a renal cancer.
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Renal angiomyolipomas are rare benign tumors known to occur sporadically and in association with genetic syndromes, tuberous sclerosis and lymphangioliomyomatosis. Surgical removal or radiographic embolisation of angiomyolipomas larger than 4 cm is usually indicated because of an increased risk of spontaneous hemorrhage. We describe successful nephron-sparing surgery for a giant angiomyolipoma and discuss relevant management issues in a patient with angiomyolipoma.
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Tuberous sclerosis is an autosomal dominant disorder characterized by facial skin lesions, epilepsy, and mental retardation. Pulmonary involvement in tuberous sclerosis is rare and shows characteristic reticulonodular infiltration and cystic changes. Lymphangioleiomyomatosis is characterized by the progressive proliferation of smooth muscle cells and occurs in 0.1-1% of patients with tuberous sclerosis. We encountered a case of pulmonary lymphangioleiomyomatosis associated with tuberous sclerosis and bilateral renal angiomyolipoma in a 31-year-old female patient. This case is reported here along with a brief review of the literature.
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Adult , Female , Humans , Angiomyolipoma , Epilepsy , Intellectual Disability , Lymphangioleiomyomatosis , Myocytes, Smooth Muscle , Skin , Tuberous SclerosisABSTRACT
Tuberous sclerosis is an autosomal dominant disorder characterized by facial skin lesions, epilepsy, and mental retardation. Pulmonary involvement in tuberous sclerosis is rare and shows characteristic reticulonodular infiltration and cystic changes. Lymphangioleiomyomatosis is characterized by the progressive proliferation of smooth muscle cells and occurs in 0.1-1% of patients with tuberous sclerosis. We encountered a case of pulmonary lymphangioleiomyomatosis associated with tuberous sclerosis and bilateral renal angiomyolipoma in a 31-year-old female patient. This case is reported here along with a brief review of the literature.