ABSTRACT
@#A 40-year-old female complains of right flank plain associated with progressive abdominal enlargement. She had stable vital signs and normal renal function. CT urogram revealed bilateral flank masses suggestive of bilateral giant angiomyolipomas. She was counseled on the various treatment options and opted to undergo open surgical excision. She underwent an open clamp-less partial nephrectomy with no intraoperative events. Operative time was 120 minutes and estimated blood loss was 250cc. She was discharged in good clinical condition on postoperative day 4. Final histopathological analysis revealed angiomyolipoma. Genetic testing was positive for mosaic variant of tuberous sclerosis. After a year of follow up, she remains stable and is maintained on everolimus. Open ischemia-free partial nephrectomy may be done safely for giant renal angiomyolipomas. Radical nephrectomy should be reserved for the last option because the presence of contralateral disease may also require surgical excision in the future.
ABSTRACT
Objective To explore the CT and MRI characteristics of hepatic epithelioid angiomyolipomas (H EA)in order to improve recognition and diagnostic accuracy of the disease.Methods The CT and MR imaging data of 8 patients with HEA proved by pathology were analyzed retrospectively.Among these patients,4 cases underwent plain and enhanced CT scans,2 plain and enhanced MRI scans and 2 both CT and MRI scans.Results In the 8 cases,7 were female.All lesions were solitary and well-defined,with maximum diameters ranging from 42 mm to 68 mm.6 leions on plain CT displayed slightly low density,of which 1 showed fat density and other 2 showed patchy cystic areas.MRI showed 4 masses with slight hypointensity on T1 WI,slight hyperintensity or hyperintensity on T2 WI,of which one demonstrated decreased signal on out-phase of T1 WI.On enhanced MR images,7 masses were significantly inhomogeneous in arterial phase,5 showed "fast wash-in and slow wash-out",2 "fast wash-in and wash-out"and 1 "slow wash-in and wash-out".6 showed"central vascular sign"in arterial phase and 2 displayed incomplete pseudocapsule in the portal venous and delayed phases.Conclusion Inhomogeneous enhancement in arterial phase,central vascular sign,fast wash-in and slow wash-out enhancement and no pseudocapsule are imaging features of HEA,which can help to improve the diagnostic accuracy.Its diagnosis still depends on histopathology and immunohistochemistry.
ABSTRACT
Objective To explore the CT and MRI characteristics of hepatic epithelioid angiomyolipomas (H EA)in order to improve recognition and diagnostic accuracy of the disease.Methods The CT and MR imaging data of 8 patients with HEA proved by pathology were analyzed retrospectively.Among these patients,4 cases underwent plain and enhanced CT scans,2 plain and enhanced MRI scans and 2 both CT and MRI scans.Results In the 8 cases,7 were female.All lesions were solitary and well-defined,with maximum diameters ranging from 42 mm to 68 mm.6 leions on plain CT displayed slightly low density,of which 1 showed fat density and other 2 showed patchy cystic areas.MRI showed 4 masses with slight hypointensity on T1 WI,slight hyperintensity or hyperintensity on T2 WI,of which one demonstrated decreased signal on out-phase of T1 WI.On enhanced MR images,7 masses were significantly inhomogeneous in arterial phase,5 showed "fast wash-in and slow wash-out",2 "fast wash-in and wash-out"and 1 "slow wash-in and wash-out".6 showed"central vascular sign"in arterial phase and 2 displayed incomplete pseudocapsule in the portal venous and delayed phases.Conclusion Inhomogeneous enhancement in arterial phase,central vascular sign,fast wash-in and slow wash-out enhancement and no pseudocapsule are imaging features of HEA,which can help to improve the diagnostic accuracy.Its diagnosis still depends on histopathology and immunohistochemistry.
ABSTRACT
Objective To study the initial experience and outcomes of laparoscopic enucleation with preoperative selective arterial embolization ( PSAE) for the treatment of renal angiomyolipomas. Methods A total of 43 patients with sporadic renal angiomyolipomas from July 2013 to November 2014 underwent laparoscopic nephron-sparing surgery ( NSS) .The patients were divided into either PSAE group ( n =19 ) or non-PSAE group ( n =24 ) .The data of patient demographics, success rate of surgery, perioperative complications rate, operating time ( OT ) , warm ischemia time ( WIT ) , estimated blood loss ( EBL ) , length of hospitalization, kidney estimated glomerular filtration rate (eGFR) and serum creatinine (Scr) level over 3 months, and ipsilateral recurrence were collected and compared between the two groups. Results Enucleation was successful in 100%patients (19/19) of the PSAE group and in 62.5%patients (15/24) of the non-PSAE group,with no significant difference ( Fisher' s test, P=0.105) The EBL was (46.4 ±20.6) ml in the PSAE group, which was significantly less than the non-PSAE group [(89.5 ±30.4) ml, t=-5.287, P=0.000].The OT and WIT were significantly shorter in the PSAE group than those in the non-PSAE group [(90.3 ± 21.1) min vs.(131.7 ±18.6) min, t=-6.831,P=0.000;(9.5 ±5.7) min vs.(24.2 ±4.8) min, t=-9.181, P=0.000]. The length of hospitalization was significantly longer in the PSAE group than that in the non-PSAE group [(7.7 ±1.1) d vs.(6.3 ± 1.3) d, t=3.748, P=0.000].No severe complications occurred in the PSAE group, whereas the non-PSAE group had 1 case of urinary leakage and 1 case of hematoma.The Scr level over 3 months after surgery was more ideal in the PSAE group [(70.1 ±13.7)μmol/L vs.(84.2 ±9.1) μmol/L, t=-4.045, P=0.000].No statistically significant difference was found in the eGFR between the PSAE group and the non-PSAE group [(72.6 ±12.8) ml· min-1· 1.73 m-2 vs.(68.0 ±10.7) ml· min-1· 1.73 m-2, t=1.284, P=0.206].No evidence of recurrence was found during follow-up period in both groups. Conclusions Laparoscopic enucleation with PSAE is a safe and effective minimally invasive procedure for the treatment of renal angiomyolipomas.As compared to traditional laparoscopic partial nephrectomy, it has advantages of less WIT, less EBL, and better protection of renal functions.It can be recommended in well-selected patients.
ABSTRACT
Objective To assess the value of selective renal arterial embolization in treating mononephrous renal angiomyolipoma. Methods 1 6 patients with mononephrous renal angiomyolipoma were retrospectively analyzed.To observe and analyze the changes in renal function,lesions reduction and its complications.Results Symptoms have been improved significantly after treatment,no se-rious complications were observed during operation and postoperation.The creatinine level in postoperation was lower than the pre-operative level,followed-up after 1year.Conclusion Selective renal artery embolization is a safe and effective method for the treat-ment of mononephrous renal angiomyolipoma.
ABSTRACT
Tuberous Sclerosis Complex (TSC) is an autosomal dominant genetic disorder characterized by hamartoma formation in multiple organs, particularly the skin, Brain, Eye, Kidney and heart. TSC is caused by mutation of two genes TSC1, and TSC2 which encodes for Hemartin and Tuberin. The case of 19 year old female with TSC is reported here since it is associated with Angiomyolipomas of kidneys, Dentigerous cyst, and polycystic ovarian disease, calcified subependymal nodules in the lateral ventricles of brain and multiple radial lens opacities in the eyes. Methodical systemic examination with appropriate investigations is mandatory to diagnose a case of Tuberous Sclerosis Complex.
ABSTRACT
Se presenta un caso de una mujer de 27 años, quien acude al Cuerpo de Guardia con dolor abdominal moderado de reciente comienzo. Al examen físico, se constata una masa abdominal que ocupaba ambos flancos. Luego de los estudios clínicos e imagenológicos, se comprobó la presencia de angiomiolipomas renales bilaterales, nódulos subependimarios y lesiones en piel por lo que se diagnosticó esclerosis tuberosa. La esclerosis tuberosa es una enfermedad neurocutánea caracterizada por cambios hamartomatosos en los pulmones, cerebro, riñones, piel, corazón y otros órganos. Para el diagnóstico se aplican criterios basados en el hallazgo de manifestaciones mayores y menores. En esto, la Imagenología tiene un importante papel(AU)
A 27 year old woman was given to the emergency department with mild, acute onset of right side abdominal pain. Clinical examination revealed firm masses bilaterally occupying almost the entire abdomen. Because the presence of bilateral angiomyolipomas, subependymal tuberous and adenoma sebaceum of the skin the patient was diagnosed as having a case of tuberous sclerosis . TS is a neurocutaneous disease characterized by hamartomatous changes in the lungs, brain, kidneys, skin, heart and others organs. The diagnostic criteria consisted of a set of major and minor diagnostic features. The imagenology plays a very important role(AU)
Subject(s)
Humans , Female , Adult , Tuberous Sclerosis/epidemiology , Tuberous Sclerosis/diagnostic imagingABSTRACT
Objective To observe the perfusion pattern of hepatic angiomyolipomas using contrastenhanced ultrasound(CEUS) and compare diagnostic efficacy of contrast-enhanced ultrasound with contrastenhanced helical CT(CECT).Methods Nineteen patients with 21 resected and pathologically proven hepatic angiomyolipomas were included in this study.Low mechanical index (mechanical index less than 0.2) realtime CEUS was performed in nineteen patients (5 patients with pulse inversion harmonic, 14 patients with contrast pulse sequencing, CPS) after 2.4ml bolus injection of contrast agent SonoVue.CECT was performed using 16-slice helical CT and contrast agent Ultravist.The diagnostic performance was calculated by considering histologic results as the reference standards.Results Fifteen tumors were correctly diagnosed as hepatic angiomyolipomas,4 tumors were misdiagnosed (including 1 hepatocellular carcinoma,2hepatic adenomas, 1 hemangioma) and 2 tumors were characterized as benign lesions by CEUS.Eight tumors were correctly diagnosed as hepatic angiomyolipomas, 13 tumors were misdiagnosed (including 7hepatocellular carcinoma, 1 liposarcoma, 2 lipomas, 1 hepatic adenomas, 2 hemangiomas) by CECT.The preoperative diagnostic accuracy was 71.4% for CEUS and 38.1 % for CECT(P＜0.05 ).Conclusions CEUS can demonstrate typical imaging characteristics of most hepatic angiomyolipomas, and has higher diagnostic performance than CECT in characterization of hepatic angiomyotipomas.
ABSTRACT
Objective To analyze the variable appearances of hepatic angiomyolipomas on CT and MRI, and to improve the diagnostic accuracy with CT and MRI. Methods All 13 cases were proved by surgical pathology. Helical CT scanning of pre- and post-contrast arterial phase, portal venous phase were performed in 12 cases, delayed phase scanning was performed in 5 of 12 cases. Magnetic resonance imaging with SE T 1WI, FSE T 2WI, and FMPSPGR axial dynamic multi-phase contrast scanning were performed in 7 cases. Results On pre-contrast CT scans, 11 of 12 lesions appeared as hypodensity, the other one appeared as slight hyperdensity. On the arterial phase scans, all lesions were markedly enhanced, the central vascular structure could be seen in 8 lesions. On the portal venous phase, 8 lesions remained enhanced and the central vascular structure could also be seen in 6 lesions. 5 of 7 lesions showed inhomogeneous hypointensity on SE T 1WI, and all of 7 lesions showed hyperintensity (from slight hyperintensity to strong hyperintensity) on FSE T 2WI. 6 lesions showed enhancement on the MR arterial phase scans, the other one showed no marked enhancement because the most parts of the lesion were fat. 4 lesions showed prolonged enhancement on the portal venous phase, and the other 3 lesions showed hypointensity. The central vascular structure could also be seen in 4 of 7 lesions on MR contrast dynamic scanning. Conclusion Both CT and MR could demonstrate the characterization of hepatic angiomyolipomas, especially the central vascular structure in the lesions strongly hint the diagnosis of angiomyolipomas. MR SE sequence with fat suppression is more sensitive than CT in the demonstration of the fat. CT and MR dynamic multi-phase contrast scanning can fully reflect the characterization of hepatic angiomyolipomas and can be helpful to improve the diagnostic accuracy.