Management of Nontraumatic Spontaneous Renal Hemorrhage (Wünderlich Syndrome) through Robotic-Assisted Laparoscopic Nephrectomy: A Case Series.

BACKGROUND Wünderlich syndrome (WS) is a rare diagnosis of nontraumatic spontaneous renal hemorrhage into the subcapsular, perirenal, or pararenal spaces. Prompt and effective intervention is necessary for an accurate pathological diagnosis and preservation of life. In the current literature, open surgery is the primary option when conservative treatment fails, but there can be serious trauma and corresponding consequences. Herein, we present 3 cases of Wünderlich syndrome managed by robot-assisted laparoscopic nephrectomy via a retroperitoneal approach. CASE REPORT Patient 1 was a 44-year-old woman with right flank pain for 6 h. Patient 2 was a 53-year-old woman with a history of diabetes who had pain in her right flank pain and nausea for 1 day. Patient 3 was a 45-year-old man with left flank pain for 1 day. All cases of WS were confirmed by CT. All 3 patients were treated with retroperitoneal robot-assisted nephrectomy after conservative treatment failed. Pathological examination confirmed that patient 1 had angiomyolipoma, and patients 2 and 3 had renal clear cell carcinoma. At the 9-month follow-up, renal function was good and no evidence of recurrence or metastasis has been detected. CONCLUSIONS These cases have highlighted the importance of the clinical history and imaging findings in the diagnosis of Wünderlich syndrome, and show that rapid management can be achieved using robot-assisted laparoscopic nephrectomy. However, it is crucial to have a skilled surgical team and adequate preoperative preparation.

[Discussion on the surgical timing of rupture and hemorrhage of renal angiomyolipoma].

OBJECTIVE: To investigate the effect of different surgical timing on the surgical treatment of renal angiomyolipoma (RAML) with rupture and hemorrhage. METHODS: The demographic data and perioperative data of 31 patients with rupture and hemorrhage of RAML admitted to our medical center from June 2013 to February 2023 were collected. The surgery within 7 days after hemorrhage was defined as a short-term surgery group, the surgery between 7 days and 6 months after hemorrhage was defined as a medium-term surgery group, and the surgery beyond 6 months after hemorrhage was defined as a long-term surgery group. The perioperative related indicators among the three groups were compared. RESULTS: This study collected 31 patients who underwent surgical treatment for RAML rupture and hemorrhage, of whom 13 were males and 18 were females, with an average age of (46.2±11.3) years. The short-term surgery group included 7 patients, the medium-term surgery group included 12 patients and the long-term surgery group included 12 patients. In terms of tumor diameter, the patients in the long-term surgery group were significantly lower than those in the recent surgery group [(6.6±2.4) cm vs. (10.0±3.0) cm, P=0.039]. In terms of operation time, the long-term surgery group was significantly shorter than the mid-term surgery group [(157.5±56.8) min vs. (254.8±80.1) min, P=0.006], and there was no significant difference between other groups. In terms of estimated blood loss during surgery, the long-term surgery group was significantly lower than the mid-term surgery group [35 (10, 100) mL vs. 650 (300, 1 200) mL, P < 0.001], and there was no significant difference between other groups. In terms of intraoperative blood transfusion, the long-term surgery group was significantly lower than the mid-term surgery group [0 (0, 0) mL vs. 200 (0, 700) mL, P=0.014], and there was no significant difference between other groups. In terms of postoperative hospitalization days, the long-term surgery group was significantly lower than the mid-term surgery group [5 (4, 7) d vs. 7 (6, 10) d, P=0.011], and there was no significant difference between other groups. CONCLUSION: We believe that for patients with RAML rupture and hemorrhage, reoperation for more than 6 months is a relatively safe time range, with minimal intraoperative bleeding. Therefore, it is more recommended to undergo surgical treatment after the hematoma is systematized through conservative treatment.

Qualitative and quantitative characteristics of CEUS for renal cell carcinoma and angiomyolipoma: a narrative review.

Incidental findings of renal masses are increasing. However, a substantial portion of surgically treated renal masses turn out to be benign on histopathological examination. Thus, there is a clear need for improved pre-surgical assessment to minimize unnecessary invasive procedures. The challenge intensifies when distinguishing between renal cell carcinoma (RCC) and angiomyolipoma (AML) in renal lesions smaller than 4 cm with minimal adipose tissue. In such cases, contrast-enhanced ultrasound (CEUS) has emerged as a valuable diagnostic tool, by utilizing both qualitative and quantitative parameters. Quantitative measures offer objectivity, reliability, and reproducibility compared to qualitative parameters, enabling the characterization of RCC subtypes and differentiation from AML. Qualitative features as enhancement pattern, degree, and peak were less helpful in distinguishing triphasic minimal fat AML (TAML) from epithelioid AML (EAML), with the pseudocapsule sign potentially being the only distinguishing qualitative feature. The pseudocapsule sign was more frequently observed in ccRCCs (38.0%) than in AMLs (15.6%). Moreover, it was detected in 40.0% of EAMLs and 34.5% of ccRCCs but not in TAMLs due to similar growth patterns between EAMLs and low-grade ccRCCs. Quantitative measures such as the time-to-peak (TTP) ratio can further enhance diagnostic accuracy and also TOC ratio should be considered, as it was higher in clear cell RCCs (ccRCCs) and in EAMLs compared to TAMLs, indicating behavior similar to ccRCCs. However, CEUS remains an operator-dependent exam.

The safety and effectiveness of laparoscopic off-clamp tumor evacuation versus traditional nephron-sparing surgery for large (>4 cm) sporadic renal angiomyolipomas.

BACKGROUND: Off-clamp nephron-sparing surgery (NSS) have been selectively performed in renal tumors in which the majority are T1a (<4 cm) renal caners. Less is known, however, whether off-clamp is a safe and effective option for treatment of Sporadic Renal Angiomyolipomas (RAML), especially in those >4 cm. The objective of our study was to compare the perioperative and renal function outcomes of a novel off-clamp tumor evacuation technique versus conventional laparoscopic NSS for the treatment of large sporadic RAMLs (>4 cm). METHODS: From January 2021 to June 2022, 42 patients diagnosed with RAML were prospectively randomized to receive laparoscopic standard NSS (Group 1) and off-clamp tumor evacuation (Group 2). The surgical and postoperative outcomes of both groups were compared. RESULTS: Baseline characteristics demonstrated no discernible variation between Group 1 and Group 2. Compared to Group 1, Group 2 was associated shorter operative time (92.5 vs 82.3 min, p < 0.001), elimination of warm ischemic time (22.9 vs 0 min, p < 0.001), more blood loss (92.6 vs 161.9 ml, p = 0.02), and lower short-term renal function reduction of the operated kidney (17.2% vs 9%; p < 0.001). Neither major complication nor recurrence occurred. CONCLUSION: It seems that transperitoneal laparoscopic off-clamp tumor evacuation is a feasible and safe option for the treatment of RAML, with the added benefit of preserving renal function to a greater extent than the traditional methods.

Utilization of Re-VASC, the Novel Retroperitoneal Neovascularity Scoring System, for Characterization of T1a Small Renal Masses.

Purpose: Early characterization of small (T1a, <4 cm) renal masses is imperative for patient care and treatment planning. Renal biopsy is a sensitive and specific procedure that can accurately differentiate small renal masses as malignant or benign. However, it is an invasive procedure with a nonnegligible complication rate and is not performed routinely at most institutions. In this study, we sought to apply the Retroperitoneal Vascularity Assessment and Scoring in Carcinoma (Re-VASC) scoring system to T1a renal masses and analyzed whether it could differentiate these masses as benign or malignant. Methods: We obtained Institutional Review Board approval to retrospectively examine the records of all patients who presented to our single, urban academic referral center for surgical treatment of renal cell carcinoma (RCC). For the malignant group, patients with a diagnosis of T1a RCC from pathologic evaluation were included. Additionally, patients with a histopathological diagnosis of a T1a nonmalignant renal mass (fat poor-angiomyolipoma or oncocytoma) were included in our benign group. Results: This study includes 57 benign and 69 malignant T1a renal tumors. Average size for benign and malignant masses were 2.47 and 2.63, respectively (p = 0.267). Analysis demonstrated no significant difference between both groups in terms of sex, laterality, or size. The average Re-VASC score of benign and malignant masses was 0.175 and malignant masses was 0.784, respectively (p < 0.001). Additionally, the Re-VASC score was independently associated with malignancy with an odds ratio of 2.223 (p = 0.0109). Conclusion: The Re-VASC scoring system exhibits significantly greater values for malignant T1a renal masses when compared to benign masses. As a result, it shows promise as an adjunctive tool to renal biopsy for clinical decision-making. Further assessment of Re-VASC's true efficacy as a diagnostic marker will include prospective evaluation of a larger multicenter population.

A case report of anterior mediastinal angiomyolipoma that invaded the left thoracic cavity.

RATIONALE: Angiomyolipoma is a mesenchymal tumor composed of blood vessels, smooth muscle, and mature adipose tissue. It is most commonly found in the kidney, and is rare outside the kidney, especially in the mediastinum. Only about 12 cases have been reported worldwide so far. PATIENT CONCERNS: We report a young female patient who had been found with a left thoracic mass for 19 years. In the past 19 years, the patient had no chest pain, dyspnea and other symptoms, but this time she visited the doctor because of cough, and there were no other clinical signs. DIAGNOSES: The patient underwent computed tomography plain scan and enhanced scan after admission with imaging manifestations of a mixed density mass in the left chest cavity, calcification and fat density in the inside, and tortuous blood vessels after enhancement. Combined with imaging, the diagnosis was teratoma, not excluding hamartoma. INTERVENTIONS: The patient underwent a central open thoracic giant mass resection. OUTCOMES: The postoperative pathology confirmed that it was angiomyolipoma originating from anterior mediastinum invasion of the left chest cavity, and no clear recurrence was seen after 1 year of postoperative follow-up. LESSONS: Angiomyolipomas in the mediastinum are rare, especially those that invade the thorax. This article describes the clinical, imaging and pathological features of the patient in detail, which improves the understanding of the disease of clinical and imaging doctors, and provides a basis for the differential diagnosis of mediastinal lesions.

Comparisons of the Safety and Effectiveness of Robot-Assisted vs Laparoscopic Partial Nephrectomy for Central Renal Angiomyolipomas: A Propensity Score-Matched Analysis Study.

Objective: To compare the safety and effectiveness of robot-assisted partial nephrectomy (RAPN) vs laparoscopic partial nephrectomy (LPN) in the treatment of central renal angiomyolipomas (AMLs). Methods: We retrospectively analyzed the clinical data of 103 patients who were treated with either RAPN or LPN for central AMLs between January 2017 and June 2022. Propensity scores were matched according to sex, age, laterality, body mass index, symptoms, diameter of tumor, location of tumor distribution, R.E.N.A.L score, preoperative hemoglobin, preoperative serum creatinine, preoperative estimated glomerular filtration rate, chronic disease, previous abdominal surgery, preoperative selective arterial embolization, American Society of Anesthesiologists scale, and duration of follow-up, and after matching, perioperative and prognostic data of the two groups were compared. Results: A total of 57 patients underwent RAPN, and 46 patients underwent LPN. Before matching, there were more complex AMLs in the RAPN group, and R.E.N.A.L scores differed between the two groups (10 vs 9, p < 0.001). After matching, the median warm ischemic time in the RAPN group was significantly shorter than that in the LPN group (21.5 minutes vs 28 minutes, p = 0.034), as well as the median time of postoperative mobilization (1 day vs 2 days, p < 0.001). The other indicators were not significantly different between the groups. Conclusions: For central AMLs, both RAPN and LPN were safe and feasible surgical treatments, but RAPN might be associated with shorter warm ischemia time and earlier postoperative mobilization.

Epitheloid Angiomyolipomas of the Kidney: Rare Renal Tumors Associated With Poor Prognoses.

OBJECTIVE: To demonstrate the clinical spectrum and challenges associated with clinical management of epitheloid angiomyolipomas (eAML). METHODS: We retrospectively reviewed the surgical database of a high-volume tertiary kidney cancer center from 2015 to 2020 to identify cases with a final histological diagnosis of eAML. Descriptive analysis of all cases was conducted. RESULTS: Five surgical cases of eAMLs were identified. Two of which have had no tumor recurrence since surgery, and three patients passed away due to disease progression. CONCLUSION: eAML are rare renal tumors which the World Health Organisation (5th Edition, 2022) and International Classification of Diseases for Oncology classify as having unspecified, borderline, or uncertain behavior. Here, we report that can also demonstrate aggressive behavior with fatal consequences. Post-operative follow-up should be recommended for all, with shorter intervals for patients with poor prognostic factors.

A case report of hepatic angiomyolipoma, inflammatory subtype. Clinico-pathological characterization: a diagnostic challenge.

BACKGROUND: Angiomyolipoma is a benign mesenchymal neoplasm of a wide histological heterogeneity belonging to the PEComa "family." The liver, after the kidney, is their second most frequent location. However, inflammatory hepatic AMLs constitute a rare entity, with only fourteen documented cases until 2020. These neoplasms can overlap morphological features of IgG4-related diseases, being of great diagnostic relevance to demonstrating myomelanocytic-lineage differentiation of the neoplastic cells. CASE PRESENTATION: we present a new case of an inflammatory hepatic AML resembling an IgG4-related disease in a 35-year-old woman with a subcapsular 5 cm mass confined to segment VII of the right hepatic lobe. Although having reduced its size along the tumor's natural evolution, complete tumor resection was decided due to its hypermetabolic behavior (max. SUV = 12,6) assessed by PET-CT scan. Finally, the patient underwent a right hepatectomy due to spontaneous rupture and bleeding of the lesion during the intervention. All the diagnostic and therapeutic procedures occurred in the last months of the COVID-19 pandemic. CONCLUSIONS: This review aims to describe inflammatory hepatic AML histological and immunohistochemical features. We further sought to establish a clinicopathological contextualization of this tumoral subtype.

Renal Lipoma-Like Angiomyolipoma of Tumour Thrombus Extending to the Confluence of Inferior Vena Cava with Right Atrium: A Rare Case Report and Literature Review.

Angiomyolipoma (AML) complicated with tumour thrombus extending to the confluence of inferior vena cava (IVC) with right atrium is rarely observed. We report a female AML patient admitted to our centre on January 21, 2020, with complication of tumour thrombus extending to the confluence of IVC with right atrium and had no sign of difficult breathing. She underwent whole-abdominal enhanced CT for abdominal pain and was diagnosed with a possible renal AML with tumour thrombus. Open radical nephrectomy and thrombectomy of vena cava were performed. Intraoperative transoesophageal echocardiography indicated that the tumour thrombus has reached the confluence of IVC with right atrium. The operation took 255 min with an intraoperative haemorrhage of 800mL. The patient was discharged 7 days after surgery. Pathology revealed lipoma-like AML. Immunohistochemistry showed vimentin (+), EMA (-), HMB45 (+), S-100 (-), SMA (+), TFE-3 (-), melan A (+). After 2 years of follow-up, we found that the patient showed full recovery and had no recurrence. Therefore, lipoma-like AML should also be followed closely for recurrence and metastasis. When AML involves IVC tumour thrombus, open thrombectomy and radical nephrectomy are safe and effective methods.

Reprint of: lessons from histopathologic examination of nephrectomy specimens in patients with tuberous sclerosis complex: cysts, angiomyolipomas & renal cell carcinoma.

Renal manifestations in patients with tuberous sclerosis complex (TSC) include cysts, angiomyolipoma, and renal cell carcinoma. Unlike many hereditary predisposition syndromes, the spectrum of renal tumors in TSC patients (including both angiomyolipoma and renal cell carcinoma) is broad, with significant morphologic heterogeneity. An improved understanding of histopathologic findings in TSC patients and associated clinicopathologic correlates has significant implications not just in establishing a diagnosis of TSC, but also in the recognition of sporadic tumors occurring secondary to somatic alterations of TSC1/TSC2/MTOR pathway genes and accurate prognostication. In this review, we have discussed issues relevant to clinical management based on histopathologic findings in nephrectomy specimens from patients with TSC. This includes discussions related to screening for TSC, diagnosis of PKD1/TSC2 contiguous gene deletion syndrome, the morphologic spectrum of angiomyolipoma and renal epithelium-derived neoplasia, including the risk of disease progression.

Renal Angiomyolipoma: A Diagnostic and Therapeutic Challenge

Background: Angiomyolipomas (AML) represent less than 10% of renal tumours. They are most often detected incidentally during imaging tests, but there are several histological variants that pose difficulties in the radiological differential diagnosis. Their identification should allow the loss of renal parenchyma due to embolization or radical surgery to be prevented. Methods: Retrospective study of patients undergoing kidney surgery with post-surgical pathological diagnosis of AML at the Álvaro Cunqueiro Hospital (2016–2021). Patients with a radiological diagnosis of AML who underwent surgery based on clinical criteria were excluded. Results: 18 patients were registered, allowing for the assessment of 18 renal tumours. All of the cases were diagnosed incidentally. Preoperative radiological diagnosis was: 9 lesions suggestive of renal cell carcinoma (RCC) (50%), 7 cases suggestive of RCC vs. AML (38.9%) and 2 lesions suggestive of AML vs. retroperitoneal liposarcoma (11.1%). Histological variants of AML were found in 61.1% of cases (n = 11). The most widely used surgical technique was partial nephrectomy, in 66.67% of cases. Conclusions: The radiological differential diagnosis of AML, and particularly its variants, with malignant lesions have important limitations either due to the predominance or scarcity of any of the AML components. Some cases can also pose difficulties at the histological level. This fact highlights the importance of the specialization of uroradiologists and uropathologists and the performance of kidney-sparing therapeutic techniques (AU)

Hepatic epithelioid angiomyolipoma is scattered and unsuitable for surgery: a case report.

Hepatic epithelioid angiomyolipoma (HEAML) is a rare tumour of mesenchymal tissue with a malignant tendency. Occurring most frequently in women, the relative incidence in men and women, according to incomplete statistics, is approximately 1:5. In rare cases, disease occurrence and development is hidden. Lesions are generally discovered as chance findings by patients; abdominal pain is the first symptom, and imaging has no specificity in diagnosing the disease. Therefore, great difficulties exist in the diagnosis and treatment of HEAML. Here, the case of a 51-year-old female patient with a history of hepatitis B, and abdominal pain over 8 months as the initial symptom, is described. The patient was found to have multiple intrahepatic angiomyolipoma. Due to the small and scattered foci, complete resection was impossible, and because of her history of hepatitis B, conservative treatment was undertaken, with the patient undergoing regular follow-up. When hepatic cell carcinoma could not be excluded, the patient was treated with transcatheter arterial chemoembolization. No tumour neogenesis or metastasis was detected at the 1-year follow-up.

p53 aberration and TFE3 gene amplification may be predictors of adverse prognosis in epithelioid angiomyolipoma of the kidney.

BACKGROUND: Although epithelioid angiomyolipoma of the kidney has been studied by several groups, the reported prevalence of malignant behavior remains uncertain and there are not yet definitive predictive biomarkers. We evaluated the behavior of renal epithelioid angiomyolipoma in a consecutive series in a single institution and investigated the prognostic value of aberrant p53 expression and TFE3 gene abnormality. METHODS: We retrospectively reviewed 14 epithelioid angiomyolipomas, most with pure or close to pure epithelioid components, comprising 12 consecutive cases who had attended our institution and two consultation cases. Fluorescence in situ hybridization with TFE3 break-apart probe was performed on 14 cases. The 14 cases were also labeled for p53 and TFE3 by immunohistochemistry. All cases were followed up. RESULTS: Three of the epithelioid angiomyolipomas were strongly positive for TFE3 and two had a mutant expression of p53. Although no TFE3 gene rearrangement was found, the two tumors with strong TFE3 expression showed TFE3 gene amplification. Follow-up details were available for seven of the 12 consecutive cases: two of them had developed metastases and died (29%), their mean overall survival was 41 months, and both had mutant p53 expression. The two consultation cases with TFE3 gene amplification developed recurrence/metastasis within 1 year after surgery. CONCLUSIONS: Our series study from a single institution presented the prevalence of malignant behavior in pure epithelioid angiomyolipomas, although the small number of cases with follow-up data greatly reduced the accuracy. p53 may be a prognostic marker for epithelioid angiomyolipoma. Cases with TFE3 gene amplification had poor prognoses.

Laparoscopic Resection of a Hepatic Epithelioid Angiomyolipoma Revealed by Indocyanine Green Fluorescence Imaging.

Indocyanine green fluorescence imaging (ICG-FI)-a sensitive tool for detecting tumor localization in laparoscopic surgery-produces false positive results for benign liver tumors. This report is the first case of hepatic angiomyolipoma (HAML) treated laparoscopically with ICG-FI. We present the case of a 31-year-old woman with a liver tumor that was a 13-mm mass in the anterior superior segment. Though a benign tumor was suspected, malignant potential could not be ruled out. Therefore, minimally invasive laparoscopic resection using ICG-FI was planned. ICG, intravenously injected preoperatively, revealed the tumor's existence. Pure laparoscopic hepatectomy with ICG-FI was performed for excisional biopsy, during which the tumor was resected with adequate surgical margins, followed by histological confirmation of HAML. In conclusion, it is suggested that laparoscopic resection with ICG-FI is an effective minimal invasive surgery for tumors that are difficult to detect, such as HAML, leading to a safe surgical margin.