Treatment of fat-poor renal angiomyolipoma with ectopic blood supply by fluorescent laparoscopy:A case report and review of literature

BACKGROUND Renal angiomyolipoma and renal cell carcinoma are the most common benign and malignant tumors of the kidney respectively,and the preoperative differential diagnosis is crucial due to the wide difference in treatment methods.Fat-poor renal angiomyolipoma is a relatively rare type of in renal angiomyolipoma.Its fat imaging features are not obvious,and it is easily misdiagnosed as renal cell carcinoma.CASE SUMMARY We report the case of a 41-year-old man who complained of osphyalgia.Subsequent abdominal computed tomography scans revealed that a heterogeneous mass was seen in the lower pole of the right kidney,with the size of about 53 mm×47 mm.And showed two right renal arteries,with the mass supplied by an ectopic vessel from the abdominal aorta.Fluorescent laparoscopic blockade of the right renal heterotopic artery and partial nephrectomy was performed.Based on histological and immunohistochemical findings,the tumor was diagnosed as fatpoor renal angiomyolipoma.CONCLUSION The use of fluorescent laparoscopy can effectively help intraoperative management,and the fluorescence pattern provided by intravenous indocyanine green can help suggest the final diagnosis,effectively guide the surgical decisionmaking,and avoid preoperative imaging diagnosis leading to nephrectomy for benign renal tumors,through fluorescent navigation of tumor supply vessel precise block,minimize the loss of renal function.

Embolization of symptomatic renal angiomyolipoma with a mixture of lipiodol and PVA, a mid-term result

Objective： To evaluate the clinical effect of renal artery embolization with a mixture of lipiodol and polyvinyl alcohol （PVA） particles on symptomatic renal angiomyolipoma and to present the mid-term results from our single-center site. Methods： We performed a retrospective review of the seven patients who underwent embolization with a mixture of lipiodol and PVA particles to treat symptomatic renal angiomyolipoma in our center between February 2011 and December 2013. Medical records and follow-up results were reviewed and analyzed. Results： Seven patients underwent eight episodes of embolization using a mixture of lipiodol and PVA particles to treat symptomatic renal angiomyolipoma. One patient required a subsequent embolization of the right kidney 9 months after the initial embolization of the left kidney. No nephrectomy was required in any of these cases during follow-up. None had recurrence of tumor bleeding or rupture during follow-up. Decreased tumor size was achieved in six patients （85.7%） during the mid-term follow-up. Conclusions： Embolization with a mixture of lipiodol and PVA particles is an effective and safe treatment for symptomatic renal angiomyolipoma. Based on the durable mid-term results of the present study, a mixture of lipiodol and PVA particles is recommended for embolization.

Anesthetics management of a renal angiomyolipoma using pulse pressure variation and non-invasive cardiac output monitoring: A case report

BACKGROUND Hypovolemic shock can lead to life-threatening organ dysfunction,and adequate fluid administration is a fundamental therapy.Traditionally,parameters such as vital signs,central venous pressure,and urine output have been used to estimate intravascular volume.Recently,pulse pressure variation(PPV)and non-invasive cardiac monitoring devices have been introduced.In this case report,we introduce a patient with massive active bleeding from giant renal angiomyolipoma(AML).During emergent nephrectomy,we used non-invasive cardiac monitoring with CSN-1901(Nihon Kohden,Tokyo,Japan)and PPV to evaluate the patient's intravascular volume status to achieve optimal fluid management.CASE SUMMARY A 30-year-old male patient with giant AML with active bleeding was referred to the emergency room complaining of severe abdominal pain and spontaneous abdominal distension.AML was diagnosed by computed tomography,and emergent nephrectomy was scheduled.Massive bleeding was expected so we decided to use non-invasive cardiac monitoring and PPV to assist fluid therapy because they are relatively easy and fast compared to invasive cardiac monitoring.During the surgery,6000 mL of estimated blood loss occurred.Along with the patient's vital signs and laboratory results,we monitored cardiac output,cardiac output,stroke volume,stroke volume index with a non-invasive cardiac monitoring device,and PPV using an intra-arterial catheter to evaluate intravascular volume status of the patient to compensate for massive bleeding.CONCLUSION In addition to traditional parameters,non-invasive cardiac monitoring and PPV are useful methods to evaluate patient's intravascular volume status and provideguidance for intraoperative management of hypovolemic shock patients.

Clinical Efficacy of Super-Selective Renal Artery Embolization for Rupture of Renal Angiomyolipoma

OBJECTIVE To evaluate the efficacy of selective transcatheter arterial embolization （TAE） in renal angiomyolipoma （AML） spontaneous ruptures with hemorrhage. METHODS A retrospective evaluation was carried out in 21 renal AML cases with acute bleeding confirmed by imaging. Selective renal arterial embolization was used to control bleeding. All the cases were detected by renal arteriography had abnormal vascular hyperplasia and enlarged blood vessels. RESULTS Initial renal arteriography for all the patients showed that tortuous, hypervascular, and aneurysm-forming angiogenic components with aneurysm formation occurred in 13 cases （61.9%） and extravasation of the contrast agent was found in 8 cases （38.1%）. Immediate complete obliteration was technically successful in 19 （90.5%） of the 21 patients. To prevent uncontrollable complications, 3 patients received nephron-sparing surgery after hemodynamic status was stabilized with TAE a week later. Two days and 3 days after the embolizations, 2 patients presented with incomplete embolizations and then underwent nephrectomy when they were in a stable condition. There were no significant differences in the plasma creatinine levels before and after the treatment. All the patients were followed up for 6 months to 6 years （median, 45 months）. The largest tumor diameter was reduced from （11.57-±4.28） cm to （9.57±2.28） cm. The tumor had no blood supply and no relapses have occurred. CONCLUSION TAE is a technically feasible and minimally invasive procedure for ruptured renal angiomyolipoma. The aneurysms were a predictor of renal AML spontaneous rupture and detection of such aneurysms by CT may help to determine the timing of embolization. In patients who still need surgical treatment, TAE can make tumor resection easier to perform and reduce blood loss during the operation.

Fat Poor Renal Angiomyolipoma Showing Collapsed Shape: A Case Report

Fat-poor renal angiomyolipoma (fpAML) and renal cell carcinoma (RCC) are difficult to differentiate and misdiagnosis can lead to unnecessary nephrectomy. We experienced a case showing a “collapsed shape” which reflected a fpAML tissue type. A renal tumor was incidentally discovered in a 42-year-old female during an abdominal ultrasound. RCC was suspected according to CT and MRI imaging results, and a partial nephrectomy was performed. However, the pathologic diagnosis was fpAML. Upon reevaluation of preoperative images, morphological change to the tumor due to contact with surrounding tissues: the collapsed shape was observed and could be identified by CT, which is the gold standard test for differentiating renal tumors. In cases where the collapsed shape is observed in a renal tumor, fpAML should be considered.

Differentiation of CT scan diagnosis between minimal fat renal angiomyolipoma with sufficient blood supply and clear cell renal carcinoma

Objective： The aim of our study was to investigate the feature of minimal fat renal angiomyolipoma with sufficient blood supply using CT scans and improve the diagnosis accuracy required to differentiate it from clear cell renal carcinoma. Methods： Retrospective analysis of 24 cases of post-surgery confirmed angiomyolipoma with sufficient blood supply （total of 25 tumors） in our hospital that were used for a pathological comparison study. Results： Among the 24 patients diagnosed with angiomyolipoma, nobody had bloody urine, Of the 96 patients diagnosed with clear cell renal cancer, 14 had bloody urine （14.6%）. In our studied group, the size of angiomyolipomas with sufficient blood supply was between 1.5 cm× 2.0 cm to 8.0 cm× 10.0 cm. During CT scan analysis, twenty tumors had similar density, and five of them had higher density. Only one tumor had a few dots of calcification （4%）. Adipose tissue was not visible in 9 tumors, while 16 tumors had visible dots of adipose tissue, as visualized by CT scan. Intensive scanning indicated that all of the tumors showed a strong enhancement in the renal corticomedullary phase. Twenty tumors had significant heterogeneous enhancement in the early phase, while another set of five cases had homogenous prolonged enhancement. Nineteen patients had surgery to remove the angiomyolipomas, while six patients had single side kidney removal due to misdiagnosis for renal cancer in cases where the tumor severely compromised the renal parenchyma and sinus. All 25 cases were classified as renal angiomyolipoma by pathological analysis. Within the 96 cases of clear cell renal cancer, 64 tumors had relatively low density, 29 tumors had equal density, and 3 cases had relatively higher density. Fourteen of the tumors had calcification （14.6%）, and none of them had visualized adipose tissue. Enhanced CT scans indicated that 69 cases of renal cancer showed significant enhancement in the renal corticomedu^ary phase, which had the abnormal pattern of ＂fast-in-and-fast-out＂. Additionally, 27 cases had slow and prolonged enhancement. Conclusion： Similar to clear cell renal carcinomas, angiomyolipomas with sufficient blood supply also appear to exhibit abnormal enhancement with a pattern of＂fast-in-and-fast-out＂ during the early phase, which is easily misdiagnosed as renal cancer. It is difficult to differentiate them merely through CT scans; the key to differentiating them is to identify the adipose tissue within the tumor. Therefore, it is helpful to use thin-layer CT scans to locate the adipose tissue.

Embolisation of Bleeding Renal Angiomyolipoma in Pregnancy

Bleeding from renal angiomyolipoma in pregnancy can be catastrophic to both mother and fetus. Selective arterial embolisation is considered more superior than conventional surgery in these patients. Our case report exemplifies how a bleeding angiomyolipoma was halted with embolisation in our pregnant patient.

Renal Angiomyolipoma Presenting as Fever of Unknown Origin

A 50 yr man presented with fatigue and fever. He was found to have a 7.2 cm left renal mass. Radical nephrectomy was done. The mass was HMB-45 negative renal angiomyolipoma. This patient did not have tuberous sclerosis. His fever resolved after the tumor excision. This case is unique because of the unusual morphology and histology of the tumor. It reiterates that getting rid of inflammatory cytokines being produced by the tumor will cause resolution of fever.

Resection of a giant renal angiomyolipoma in a solitary kidney with preoperative arterial embolization

Renal angiomyolipoma is a type of benign tumor that occurs sporadically in addition to being associated with tuberous sclerosis. Preoperative embolization of large tumors is important to avoid excessive blood loss during surgery. We reported a patient with a 5505-g giant renal angiomyolipoma in a solitary kidney. The patient was treated with preoperative embolization and radical nephrectomy without complications. This type of treatment for an enormous angiomyolipoma can reduce the risk of uncontrolled hemorrhage caused by rupture of the tumor during the operation and should be considered for the treatment of similar tumors.

Giant renal angiomyolipoma with tuberous sclerosis complex

Though rare,angiomyolipomas(AMLs)are the most common mesenchymal tumors of kidney.In general,AMLs can always be associated with two conditions affecting other organ systems:tuberous sclero-sis complex(TSC)and sporadic lymphangioleiomyoma-tosis.This article presents a case of renal AML occurring in a 14-year-old girl with a definite diagnosis of TSC.She had been diagnosed with TSC at the age of three,and a schedule for close observation was disobeyed.At this time,she underwent a series of examinations:physical examina-tion,ultrasonography,angiography,computed tomography(CT),and magnetic resonance imaging(MRI)scans.The physical examination showed adenoma sebaceum in a butterfly paranasal distribution,and a mass was palpated in the left upper quadrant.There were no neurological deficits.Imaging studies(including ultrasonography,angiography,CT,and MRI)of the abdomen showed a large heterogeneous mass arising from the left kidney.Partial nephrectomy was performed.The pathological diagnosis was hemorrhagic renal AML.No recurrence was found in the three-year follow-up.We concluded that schedule of close observation on patients with TSC should be strictly abided by for the high morbidity of AMLs.The specific risks of renal AMLs are spontaneous hemorrhage and rupture.Treatment options for AMLs include conservative and interventional(total/partial nephrectomy,cryoptherapy,and embolization)treatments.

Renal Angiomyolipoma：Diagnosis with B－ultrasonography，CT Scanning，DSA and Its Interventional Treatment

From 1989.15 cases of renal angiomyolipoma (AML) have been diagnosed by ultrasonography, CT scanning and digital subtraction angiography (DSA) at our hospital. In 8 patients with uneven hyperechoes on B-mode ultrasonography (B-US)(8/15) and 7 with low density of fat on CT scanning (7/12)accurate diagnosis was established preoperatively. DSA revealed the 'berry-like'pseudoaneurysms in the arterial phase (14 cases), the defined lucent area in the nephrogram phase (10 cases) and the 'onion-peel appearances' during venous phases (8 cases),correct diagnosis was achieved in all patients. 8 cases were surgically treated and 7 treated by subselective embolization of renal artery. Effects in all cases were good. The diagnostic value of B-US. CT scanning, DSA and interventional treatment of AML was discussed. It was believed that the diagnosis with DSA was a technique with high specificity, and embolization therapy was simple and effective for AML.

Successful robot-assisted partial nephrectomy for giant renal hilum angiomyolipoma through the retroperitoneal approach: A case report

BACKGROUND Giant renal angiomyolipomas(AMLs)may lead to complications including flank pain,hematuria,hypertension,retroperitoneal hemorrhage and even death.Giant AMLs which grow around renal hilar vessels and the ureter are rare.Most previous reports on the treatment of giant renal AMLs have focused on open surgery or a transperitoneal approach,with few studies on the retroperitoneal approach for large AMLs.We here report a case of giant renal hilum AML successfully treated with robot-assisted laparoscopic nephron sparing surgery the retroperitoneal approach,with a one-year follow-up.CASE SUMMARY A 34-year-old female patient was diagnosed with renal AML 11 years ago and showed no discomfort.The tumor gradually increased in size to a giant AML over the years,which measured 63 mm×47 mm×90 mm and was wrapped around the right hilum.Therefore,a robotic laparoscopic partial nephrectomy(LPN)via the retroperitoneal approach was performed.The patient had no serious postoperative complications and was discharged soon after the operation.At the one-year follow-up,the patient's right kidney had recovered well.CONCLUSION Despite insufficient operating space via the retroperitoneal approach,LPN for giant central renal AMLs can be completed using a well-designed procedure with the assistance of a robotic system.

Occult Renal Cell Carcinoma of Eosinophilic Morphology Detected within Renal Angiomyolipoma Mass in a Patient with Tuberous Sclerosis Complex

The kidney is affected in about 80-85% of tuberous sclerosis complex （TSC） patients. Renal manifestations in TSC patients include an increased incidence of epithelial cysts and tumors, such as multiple renal angiomyolipomas （AMLs）, renal cell carcinoma （RCC）, and oncocytoma. The coexistence of RCC and renal AML within same tumor masses, namely collision tumor, is very rare, and about six cases have been reported. Here, we present a case of a young male with TSC and multiple AMLs, containing RCC with eosinophilic morphology.

Value of Apparent Diffusion Coefficient(ADC) of Diffusion eighted Magnetic Resonance Imaging in Common Renal Disease Diagnosis

Objective：To find the value of the apparent diffusion coefficient （ADC） of diffusion weighted magnetic resonance imaging of common renal diseases. Methods： There were 30 healthy subjects and 81 patients with renal lesions （56 cases of renal carcinoma, 18 lesions of 12 cases of renal angiomyolipomal and 21 lesions of 13 cases of renal cysts）. Conventional magnetic resonance imaging and diffusion weighted magnetic resonance imaging were carried out. We measured the average ADC value of the renal lesions and normal kidneys. ADC maps from different b values were generated by a statistical package. Results： The ADC values of normal kidneys with three different motion-probing gradients（b=500, 800, 1000 sec/mm^2） were 2.78 ± 0.14 × 10^-3mm^2s^-1, 2.45 ± 0.13 × 10^3mm^2s^-1, 2.13 ± 0.14 × 10^-3mm^2s^-1, respectively. The ADC values of renal cell carcinoma with three different motion-probing gradients（b=500, 800, 1000 sec/mm^2） were 1.63 ± 0.14 × 10^3mm^2s^-1, 1.31 ± 0.18 × 10^3mm^2s^-, 1.07 ± 0.15 × 10^-3mm^2s^-1, respectively. Among the renal cell carcinoma, the ADC value of clear cell type were 1.67 ± 0.09 × 10^3mm^2s^-1, 1.36 ± 0.13 × 10^3mm^2s^-1, 1.15 ± 0.14 × 10^3mm^2s^-1,respectively; the ADC values of granular cell type were 1.59±0.19 × 10^3mm^2s^-1, 1.25 ± 0.22 × 10^3mm^2s^-1, 0.97 ± 0.12 × 10^3mm^2s^-1, respectively. The ADC values of renal angiomyolipoma with three different motion-probing gradients（b=500, 800,1000 sec/mm^2） were 0.88 ± 0.08 × 10^3mm^2s^-1, 0.63 ± 0.07 × 10^3mm^2s^-1, 0.43 ± 0.04 × 10^3mm^2s^-1, respectively. The ADC values of renal cystic lesions with three different motionprobing gradients（b=500, 800, 1000 sec/mm^2） were 3.73 ± 0.18 × 10^3mm^2s^-1, 3.44 ± 0.13 × 10^3mm^2s^-1, 3.09± 0.21 × 10^3mm^2s^-1, respectively. Statistically significant differences exists between the ADC values of normal kidney, renal carcinomas, renal angiomyolipomas and renal cysts when the b value is the same. Among the different cell types of renal carcinomas, the ADC value of granular cell carcinoma is lower than that of clear cell carcinomas. Conclusion： It is of benefit in diagnosing and distinguishing between benign and malignant renal tumors to know the ADC values in diffusion weighted magnetic resonance imaging. Furthermore, these values help to know the internal structure of the tumor and the tumor typel, which is helpful to the treatment and in predicting the patient＇ s prognosis.

Contrast enhanced ultrasound of renal masses

Contrast enhanced ultrasound(CEUS) has gained clinical importance over the last years for the characterization of hepatic masses.Its role in extrahepatic indications has been investigated repeatedly but has been less comprehensively studied.Currently more than 50% of renal masses are incidentally diagnosed,mostly by B-mode ultrasound.The method of choice for characterization of renal lesions is contrast enhanced computed tomography(CECT).In the case of cystic lesions CECT refers to the Bosniak classification for cystic lesions to assess the risk of malignant behavior.The majority of masses are renal cell carcinoma,but the exact proportion is controversial.Disadvantages of CECT are a significant risk for patients with impaired renal function,allergic reactions and hyperthyroidism due to iodinated contrast agents.Several studies concerning CEUS for the characterization of both solid and cystic renal lesions have been published,but prospective multicenter studies are missing,the presented data being mainly descriptive.The aim of the this manuscript is to review the current literature for CEUS in renal masses,to summarize the available data and focus on possible concepts for studies in the future.

Nephrectomy Avoided in a Patient with Lipid Poor Angiomylipoma with Radiological Features Suspicious for Renal Cell Carcinoma: A Case Report

Angiomyolipoma (AML) is a benign neoplasm that is easily mistaken for renal cell carcinoma (RCC) upon radiological investigation. The management of the two pathologies is significantly different, and so accurate diagnosis is vital. We report a case in which ultrasound guided biopsy saved the patient from an unnecessary nephrectomy when an AML had radiological appearances consistent with RCC. This case report also discusses the literature surrounding radiological features and novel imaging techniques of minimal-fat AML and RCC.

MSCT对乏脂肾血管平滑肌脂肪瘤与非透明细胞肾癌的鉴别诊断

目的分析乏脂肾血管平滑肌脂肪瘤(乏脂AML)与非透明细胞肾癌(肾乳头状细胞癌、嫌色细胞癌)的CT特征,提高病变的鉴别诊断效能。方法回顾性分析经手术病理证实乏脂AML19例、肾乳头状细胞癌(PRCC)16例及肾嫌色细胞癌(ChRCC)22例。测量肿瘤四期(平扫期、皮髓质期、实质期及排泄期)相同ROI的CT值及健侧肾皮质CT值,计算肿瘤强化百分比、多期相净增值、相对强化比并进行统计学分析。结果乏脂AML在平扫期及皮髓质期CT值高于PRCC与ChRCC的CT值,差异均有统计学意义(均P<0.05),在实质期及排泄期,乏脂AML与两者比较差异无统计学意义(均P>0.05)。乏脂AML在皮髓质期、实质期和排泄期的强化百分比高于PRCC、ChRCC,差异均有统计学意义(均P<0.05)。乏脂AML与PRCC、ChRCC在皮髓质期及实质期多期相净增值、相对强化比的差异均有统计学意义(均P<0.05),在排泄期差异无统计学意义(P>0.05)。结论乏脂AML与非透明细胞癌平扫及增强扫描有其特征性CT表现,强化百分比、多期相净增值、相对强化比可进一步提高AML与非透明细胞肾癌的诊断及鉴别诊断能力。

186例结节性硬化症相关肾血管平滑肌脂肪瘤临床特点分析

目的 结节性硬化症(TSC)是一种罕见的常染色体显性遗传病,可累及多个器官及系统。肾血管平滑肌脂肪瘤(RAML)是引起成人TSC患者死亡的主要原因。本研究旨在分析TSC-RAML的临床表现,使临床医师更好地认识该病在中国患者中的特点。方法 回顾性分析2014年1月至2023年1月北京协和医院TSC-RAML患者的病历资料。收集患者的年龄、性别、影像学检查、RAML分级及合并症等信息。结果 共纳入186例TSC-RAML患者,其中男65例,女121例,男女比例为1∶1.86。患者中位年龄为31岁。117例(62.9%)RAML为6级。22例(11.8%)有肿瘤破裂出血史,在肿瘤破裂出血方面,高分级与低分级间存在统计学差异(P=0.0475)。合并临床表现以血管纤维瘤/纤维斑块(155/186,83.3%)、室管膜下结节(103/146,70.5%)、肺淋巴管平滑肌瘤病(102/157,65.0%)、色素脱失斑(114/186,61.3%)、鲨革斑(83/186,44.6%)最为常见。其中肺淋巴管平滑肌瘤病患者均为女性(P<0.0001)。结论 在TSC-RAML患者中女性患者数量相对占优。大多数TSC-RAML为6级,RAML分级越高,存在肿瘤破裂出血的比例越多。皮肤病变和神经系统病变为最常见的合并症。TSC相关肺淋巴管平滑肌瘤病患者均为女性。

中国结节性硬化症相关肾血管平滑肌脂肪瘤诊疗的发展和现状

结节性硬化症(TSC)是一种由TSC1和TSC2基因突变引起的罕见常染色体显性遗传病,可引发多器官病变,肾脏病变主要是肾血管平滑肌脂肪瘤(RAML)。近年来,随着国内对TSC-RAML的认识不断加深,在诊疗上逐渐规范并完善,同时进行发病机制和治疗方面的探索。本文对中国TSC-RAML的诊疗研究发展进行回顾并展望。

肾脏最大径大于4 cm的大体积上皮样血管平滑肌脂肪瘤临床特征分析

目的:分析大体积(最大径>4 cm)肾脏上皮样血管平滑肌脂肪瘤(EAML)的临床特征。方法:纳入2010至2021年在我院诊断为肾脏EAML,且符合纳入标准的30例患者,分析其临床、病理和治疗特点。结果:30例受试者中,男性12例,女性18例,年龄26~74岁。所有患者均行CT扫描。大多数EAML患者(23/30)在平扫CT图像上表现为相对于肾实质的高密度及以高密度为主的混杂密度,7例表现为低密度。完全性EAML的患者相对于部分EAML的患者,其肿块在平扫CT上表现为更大面积的高密度影。肿瘤最大径(8.7±6.6)(4.0~27.7)cm。所有患者均接受手术治疗并获得随访,随访时间24~150个月,中位随访时间59.5个月。随访患者中,1例死于肿瘤恶液质并伴有肝转移,3例出现同侧复发,1例患者出现肝转移,均为完全性EAML。结论:上皮样血管平滑肌脂肪瘤(EAML)是一种相对罕见的乏脂肪肾肿瘤。相对于小体积的EAML存在典型的CT表现,大体积的EAML在平扫CT上高密度特征已不再典型。只有少数EAML具有恶性潜能。手术治疗效果较好,但术后可能会出现复发和转移,尤其是老年患者,以及完全性EAML患者,建议密切随访。