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The case study highlights the possibility of pharmacological therapy with everolimus for patients with extensive kidney angiomyolipomatosis, which poses a serious risk of bleeding to the patients. [ABSTRACT FROM AUTHOR]

We present a case report of gigantic recurrent angiomyolipoma of the horseshoe kidney in a young patient with suspected tuberous sclerosis syndrome. The condition was solved by repeated cytoreductive surgery in combination of systemic treatment with mTOR inhibitors. [ABSTRACT FROM AUTHOR]

Objective: Retrospective evaluation of a cohort of patients who suffered from Wunderlich´s syndrome Assessment of the etiology, clinical manifestation, standard diagnostic tools and treatment options in patients with Wunderlich´s syndrome Methods: We performed a retrospective evaluation of a cohort of 8 patients who suffered from spontaneous renal hemorrhage / retroperitoneal bleeding during the 15 years period The group of patients was divided according to the etiology, clinical manifestation, diagnostic tools and treatment procedures performed in individual patients Post treatment clinical evaluation was performed as well Results: There were 4 cases of renal hemorrhage / retroperitoneal bleeding due to spontaneous rupture of angiolipoma 2 patients suffered from bleeding due to renal carcinoma rupture There was 1 patient with tuberous sclerosis and 1 patient with spontaneous rupture of the extra adrenal pheochromocytoma (an extremely rare condition) 5 patients underwent open surgical treatment, while in the remaining 3 patients endovascular procedure was performed (with the aim of preserving the affected organ/kidney) There were no serious adverse events related to the procedure performed / with relationship to the performed procedure One patient died shortly after the procedure due to tumor progression The remaining patients are alive Conclusion: Spontaneous, nontraumatic renal hemorrhage / retroperitoneal bleeding presented as Wunderlich´s syndrome is a very serious and in some cases life threatening condition Therapeutic approach is determined by the assessment of the patient´s health status as well as by the possible etiology of bleeding. [ABSTRACT FROM AUTHOR]

Introduction: A robotic system is another major step in the development of minimally invasive surgery in the pelvic as well as upper urinary tract regions. In terms of resection procedures of the kidney, it stretches the possibilities of mini‑invasiveness to even where laparoscopy, due to tumour size or position, is no longer feasible or is too risky. What we consider the greatest benefit of a robotic system is the possibility of a safe treatment of the resection bed. This fact gives the courage to relatively safely attempt to perform a minimally invasive procedure in a setting where classic open approach was indicated previously. Method: A 55-year‑old male patient with a finding of a large angiomyolipoma of the left kidney was referred to our centre for consultation. The size of the tumour was 68 x 55 x 78 mm with a centrally extending portion. The patient had been followed up at his catchment area facility for two years. Due to an increasing size of the tumour over time and an increased risk of rupture and major bleeding, a decision was made to use an active approach. The EAU guidelines on the treatment of AML are rather unequivocal. The criterion of 4 cm AML that has been used for years as an indication criterion for surgical management is no longer present in the 2018 guidelines. The patient had successive consultations at several practices with a proposal to remove the whole affected kidney. One of the options considered was multi‑stage vasographic obliteration of the tumour, however, with an uncertain outcome. At our centre, the patient was offered robotic‑assisted resection, bearing in mind a high risk of nephrectomy. The procedure was performed in the right flank position with the daVinci® Xi robotic surgical system using three robotic arms (a camera, bipolar instrument, and monopolar scissors or needle driver) and one assistant port. Normally, we do not use the fourth robotic arm (prograsp) during resection procedures. The reason for this is only minimal benefit from its use for the surgeon as well as an effort to reduce the costs. If necessary, its role can be taken over by the assistant port. After releasing adhesions in the abdominal cavity, incision of the posterior peritoneum, and colon mobilization, the tumour and the adjacent portion of the kidney were exposed. The hilum was dissected and, during the course of resection and the placement of the first layer of suturing, the renal artery was clamped. To place stitches around the resection area, including the open hollow system, V‑LocTM sutures were used in three successive steps, while being anchored with non‑absorbable Hem‑o-lok® clips. Given the price and excellent experience, we exclusively use non‑absorbable clips at our centre. The suturing of the resection area was thus performed in two layers, with the third suture used to close the Gerota fascia. We have very good experience with this absorbable, self‑tightening suture from laparoscopic procedures. It enables rapid and safe treatment of the resection area without a need for knotting. The specimen was extracted from the body using an endobag through an extended port site at the umbilicus. A drain was passed through the lateral port. The surgery was uneventful. Following surgery, a 48-hour resting period was ordered, with the drain having been removed after 24 hours. The postoperative course was completely uneventful. As of now, the patient is one year since surgery, with no evidence of local recurrence on ultrasound. Follow‑up appointments are scheduled at yearly intervals; prospectively, he will be discharged from surveillance. Results: The duration of surgery was 126 mins (skin to skin), out of which the procedure itself took 80 mins, docking 10 mins, and undocking, specimen extraction and wound suturing 26 mins. Blood loss was 50 ml. The renal artery was clamped for 12 mins. Angiomyolipoma was confirmed by histology. The duration of hospital stay was 5 days. Conclusion: Robotic‑assisted resection of renal tumours is another advancement in minimally invasive upper urinary tract surgery. There has been a major increase in the numbers of patients who can be offered minimally invasive surgery regarding the size of the tumour, its position, or safe treatment of the resection bed. [ABSTRACT FROM AUTHOR]

Bocková J, Kuldan R. Spontaneous rupture of renal angiomyolipoma We present a case report of spontaneous rupture of an angiomyolipoma with bleeding into the retroperitoneum. A hirty three year‑old woman with spontaneous rupture of an angiomyolipoma presented with progressive anemia. Bleeding into the retroperitoneum due to sponaneus rupture of an angiomyolipoma was diagnosed. This situation required surgical revision and left‑sided nephrectomy. [ABSTRACT FROM AUTHOR]

Renal angiomyolipoma (AML) is a benign mesenchymal tumor, which occurs sporadically or is associated with tuberous sclerosis complex (TSC). The overall prevalence of AML is 0.44 %, its incidence in women is 4 times higher than in men. Prevalence in the female population is thus 0.6 %, but male rates 0.28 %. Diagnosis is based on the presence of fat in the tumor. In most cases AML can be identified preoperatively. AML morphology is highly variable and in cases where fat is missing, preoperative diagnostic based on imaging methods is not possible. Active monitoring of patients with AML is crucial, because AML grows slowly and with minimal morbidity. Patient can present with haematuria, pain or retroperitoneal bleeding by tumors rupture. The main risk factors for bleeding are tumors volume, percentage of the vascular component in the tumor and the presence of TSC. Selective arterial embolization resolves hemorrhagic complications and reduces the volume of the tumor. The long-term outcomes of embolisation are poor due to tumor revascularization and growth. Nephron sparring surgery is associated with better long term outcomes. Radiofrequency ablation is another option, but strongly restricted by the volume tumor. An additional option is cryoablation, which has better results in cases where tumor is large, but this requires special equipment. Systematic treatment using mTOR inhibitors (everolimus, sirolimus), has recently been introduced, which are essential in patients with TSC, where the disease is often complicated by multiple and recurrent lesion. This therapy can help reduce the total volume of AML and allow eventual resection. [ABSTRACT FROM AUTHOR]

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We present a case report of a rare clinical manifestation of spontaneous rupture of an angiomyolipoma. Fourty eight year-old woman on antigoalution treatment with Warfarin* presented with haemoperitoneum. Bleeding into the retroperitoneum and peritoneal cavity due to spontaneous rupture of an angiomyolipoma was diagnosed. This situation led to the development of hemorhagic shock with a need for emergency surgical revision and right-sided nephrectomy We describe the diagnostic algorhytm, course of the disease and treatment outcomes. [ABSTRACT FROM AUTHOR]