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Background/objective: To present the long-term result and efficacy of robotic partial nephrectomy (RPN) for renal angiomyolipomas (AMLs) with perioperative outcome and renal function preservation., Methods: From September 2006 to October 2014, the database of a single medical center was reviewed and patients who underwent RPN for AMLs were enrolled. The patient demographics, perioperative complications, and postoperative outcomes were analyzed., Results: We identified 23 patients who were treated with RPN for renal AMLs. The average age was 52.7 (± 9.9) years, and 20 (87%) patients were female. The median size of the resected AML was 5.2 [interquartile range (IQR)=3.1-6.8] cm. The median estimated blood loss was 100 (IQR=50-225) mL, and three (13%) patients required blood transfusion. Perioperative complications occurred in six (26%) patients and none of them are higher than Clavien Grade II. The median estimated glomerular filtration rate at 3-month and the latest follow-ups were 103 (IQR=85.5-112) mL/min/1.73m 2 and 104 (IQR=90-112) mL/min/1.73m 2 , respectively, with a median of 89.6% (IQR=84.2-100) and 86.9% (IQR=81.3-97.8) preservation, respectively. The median follow-up period was 40 (IQR=30.5-61.5) months. None of the patients developed complications requiring a second intervention or local recurrence of AML., Conclusion: A long-term follow-up of RPN for renal AMLs revealed good preservation of renal function with a low complication rate. It may be considered as a reliable method to manage renal AMLs., (Copyright © 2016. Published by Elsevier Taiwan.)

Background: Liver angiomyolipomas (AML) are mesenchymal neoplasms with an uncertain clinical behavior. The spectrum of presentation, imaging and histologic features, and outcomes were analyzed in all patients treated at Memorial Sloan-Kettering Cancer Center., Methods: Demographics, disease, pathologic, treatment, and outcome-related variables for consecutive patients were reviewed retrospectively. All imaging studies obtained at presentation were reexamined, categorized, and compared using Fisher and Wilcoxon tests., Results: Between 1989 and 2010, 238 patients with AML were seen and 22 (9.3%) had liver involvement (exclusive = 17; combined with kidney = 5). The median age was 53 years; 18 were females, and 15 had symptoms. AML was not suspected at initial presentation in any patient. Fat-containing neoplasms on imaging were larger (P = .03), with more heterogeneous enhancement compared with fat-poor neoplasms (P = .001), but none of the imaging/histologic features correlated with outcome. Thirteen patients underwent resection (R0 = 9), 4 were observed, 2 received chemotherapy, 2 embolization, and 1 embolization plus intra-arterial chemotherapy. Two patients treated with R0-resection recurred and 2 treated with chemotherapy progressed, but no patient died of AML-related causes. At a median follow-up of 36 months, 7 patients were free of disease, 13 were alive with disease, 1 died of an unrelated cause, and another was lost to follow-up., Conclusion: AMLs are rare neoplasms with an indolent course in most patients. Subtypes based on fat content are recognized, but clinical behavior does not seem to be different. Recurrence after resection is not associated with disease-related mortality. Resection may be unnecessary in selected asymptomatic patients if the diagnosis can be established definitively., (Copyright © 2011 Mosby, Inc. All rights reserved.)

Background: Renal angiomyolipomas are recognized as clonal neoplasms with clonal chromosomal aberrations and a common progenitor cell, the perivascular epithelial cell (PEC). The epithelioid variant is a recently identified entity, characterized by predominance of PEC and a unique morphologic and immunohistochemical profile. There is accumulating evidence suggesting that renal epithelioid angiomyolipoma (REA) is a malignant disease., Methods: We searched the literature for descriptions of clinical behavior of REA. A Pubmed search was performed using the following key words: angiomyolipoma, epithelioid, perivascular epithelial cell or/and renal tumors. We reviewed a case of fatal REA at our institution. A pathologist reviewed slides to confirm the diagnosis., Results: Upon review of 140 articles, 37 eligible articles were found including 10 articles describing the clinical course of REA. Almost all of the patients described, for whom there was a follow-up available, died of neoplastic progression of the disease, with liver, lung and bone metastases. Four cases were reclassified after retrospective pathology review, and they were fatal. Three of these had been misdiagnosed as renal cell carcinoma (RCC), while 1 was diagnosed as classic angiomyolipoma., Conclusion: Unlike commonly benign classic angiomyolipoma, REA behaves aggressively. It is crucial for the clinician to be aware of and identify this epithelioid variant as a malignant disease. It should be carefully differentiated from RCC. Resection alone may not be curative, and adjuvant therapy should be considered. A multimodality treatment approach needs to be explored for this newly recognized malignant variant renal angiomyolipoma.

Background: The treatment for and long-term outcome of renal angiomyolipoma (AML) at high-risk for bleeding has not been determined., Objective: To evaluate the complication rates and the long-term outcomes among patients treated by selective arterial embolization (SAE) for a large or symptomatic renal AML., Design, Setting, and Participants: Forty-one patients with 48 kidneys containing AML were treated by SAE at a single tertiary academic center., Intervention: All patients were treated by SAE and followed in a single center., Measurements: SAE was performed with a mixture of 96% ethanol and polyvinyl alcohol particles. The variables used for the analysis included age, gender, presence of tuberous sclerosis (TS), and maximal tumor size prior to SAE. The study end points were recurrence of symptoms or bleeding, the need for re-embolization or surgery, and disease-specific survival. The mean follow-up period for the entire group was 4.8 yr., Results and Limitations: Mean patient age was 51 yr (range: 24-82), and the mean initial tumor size was 10.3 cm. Successful SAE was achieved in 40 patients (91%) with a minor complication rate of 11%. Avoidance of surgery was achieved in 96% of the kidneys. No retroperitoneal hemorrhage was noted during follow-up, and 98% of the kidneys were preserved during the follow-up period. No significant changes in creatinine levels were noted following SAE (P=0.27). The freedom from surgical treatment at 5 yr following SAE was 94% (95% CI, 89-99%). Disease-specific survival of the entire cohort was 100%. The study is a retrospective, and treatment was not given according to prospective protocol, and therefore sample bias may be present., Conclusions: SAE of renal AML has long-term efficacy in preventing hemorrhagic complications of renal AML, and preservation of the involved kidneys is amenable in both TS and sporadic cases.

An analysis in 53 patients with kidney cancer has been conducted, a group on which kidney sparing operations have been performed. 25 women and 28 men have been examined at the age of 53.7 in the postoperative period of 7 divided by 130 months. The average observation time without any recurrences or metastases was 48.8 months. 5.7% local and 1.9% remote decease related recurrences have been found. The stage of clinical progression and the degree of histological malignancy are factors determining the five-year survival being 98.0%, while without any recurrences or metastases being 88.4%.