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BMC Nephrology Mar 2024Women are counseled preconceptionally about the potential risks of rAML progression and chance of complications during and due to pregnancy. However, a systematic search...
BACKGROUND
Women are counseled preconceptionally about the potential risks of rAML progression and chance of complications during and due to pregnancy. However, a systematic search investigating the evidence on which this advice is based does not exist. The aim of this systematic review is to determine the effect of pregnancy on renal angiomyolipoma (rAML) size and risk of haemorrhage in patients with tuberous sclerosis complex (TSC).
METHODS
We searched PubMed, EMBASE, Medline and ClinicalTrials.gov using terms for "renal angiomyolipoma" and "pregnancy". English-language articles published between January 1st 2000, and December 31st 2020 of which full-text was available were included. The initial search resulted in 176 articles. After the screening process we included 45 case reports and 1 retrospective study. For the retrospective study we assessed the risk of bias using the Newcastle-Ottawa Scale. We included articles about renal AML and pregnancy with and without an established diagnosis of TSC. From these articles we recorded the rAML sizes and rAML complications.
RESULTS
Seven case reports, from a total of 45 case reports, provided follow-up data on renal AML size (these were all cases of renal AML without a known diagnosis of TSC). Of these cases, renal AML size decreased in one patient, was stable in one patient, increased in three patients and fluctuated in two others. Renal AML size of women who suffered a haemorrhage were significantly larger (12.1 ± 4.6 cm) than rAMLs of women who did not suffer a haemorrhage (8.3 ± 3.2 cm). Data from the retrospective study showed no difference in renal complications between the women with and without a history of pregnancy. Haemorrhage occurred in 30% of the women with a history of pregnancy (n = 20) and in 11% in the patients without a history of pregnancy (n = 2), however this retrospective study had methodological limitations.
CONCLUSION
The effect of pregnancy on renal AML size and complications in patients with TSC is unclear. More research is needed to determine the risk of pregnancy on TSC-associated kidney disease in TSC patient.
Topics: Humans; Female; Pregnancy; Angiomyolipoma; Kidney Neoplasms; Tuberous Sclerosis; Retrospective Studies; Hemorrhage; Leukemia, Myeloid, Acute
PubMed: 38519911
DOI: 10.1186/s12882-024-03483-4 -
PloS One 2023Differentiation of fat-poor angiomyolipoma (fp-AMLs) from renal cell carcinoma (RCC) is often not possible from just visual interpretation of conventional... (Meta-Analysis)
Meta-Analysis
PURPOSE
Differentiation of fat-poor angiomyolipoma (fp-AMLs) from renal cell carcinoma (RCC) is often not possible from just visual interpretation of conventional cross-sectional imaging, typically requiring biopsy or surgery for diagnostic confirmation. However, radiomics has the potential to characterize renal masses without the need for invasive procedures. Here, we conducted a systematic review on the accuracy of CT radiomics in distinguishing fp-AMLs from RCCs.
METHODS
We conducted a search using PubMed/MEDLINE, Google Scholar, Cochrane Library, Embase, and Web of Science for studies published from January 2011-2022 that utilized CT radiomics to discriminate between fp-AMLs and RCCs. A random-effects model was applied for the meta-analysis according to the heterogeneity level. Furthermore, subgroup analyses (group 1: RCCs vs. fp-AML, and group 2: ccRCC vs. fp-AML), and quality assessment were also conducted to explore the possible effect of interstudy differences. To evaluate CT radiomics performance, the pooled sensitivity, specificity, and diagnostic odds ratio (DOR) were assessed. This study is registered with PROSPERO (CRD42022311034).
RESULTS
Our literature search identified 10 studies with 1456 lesions in 1437 patients. Pooled sensitivity was 0.779 [95% CI: 0.562-0.907] and 0.817 [95% CI: 0.663-0.910] for groups 1 and 2, respectively. Pooled specificity was 0.933 [95% CI: 0.814-0.978]and 0.926 [95% CI: 0.854-0.964] for groups 1 and 2, respectively. Also, our findings showed higher sensitivity and specificity of 0.858 [95% CI: 0.742-0.927] and 0.886 [95% CI: 0.819-0.930] for detecting ccRCC from fp-AML in the unenhanced phase of CT scan as compared to the corticomedullary and nephrogenic phases of CT scan.
CONCLUSION
This study suggested that radiomic features derived from CT has high sensitivity and specificity in differentiating RCCs vs. fp-AML, particularly in detecting ccRCCs vs. fp-AML. Also, an unenhanced CT scan showed the highest specificity and sensitivity as compared to contrast CT scan phases. Differentiating between fp-AML and RCC often is not possible without biopsy or surgery; radiomics has the potential to obviate these invasive procedures due to its high diagnostic accuracy.
Topics: Humans; Carcinoma, Renal Cell; Angiomyolipoma; Retrospective Studies; Diagnosis, Differential; Kidney Neoplasms; Tomography, X-Ray Computed; Sensitivity and Specificity; Leukemia, Myeloid, Acute
PubMed: 37498830
DOI: 10.1371/journal.pone.0287299 -
The Cochrane Database of Systematic... Jul 2023Potential benefits of rapamycin or rapalogs for treating people with tuberous sclerosis complex (TSC) have been shown. Currently everolimus (a rapalog) is only approved... (Review)
Review
BACKGROUND
Potential benefits of rapamycin or rapalogs for treating people with tuberous sclerosis complex (TSC) have been shown. Currently everolimus (a rapalog) is only approved for TSC-associated renal angiomyolipoma and subependymal giant cell astrocytoma (SEGA), but not other manifestations of TSC. A systematic review needs to establish evidence for rapamycin or rapalogs for various manifestations in TSC. This is an updated review.
OBJECTIVES
To determine the effectiveness of rapamycin or rapalogs in people with TSC for decreasing tumour size and other manifestations and to assess the safety of rapamycin or rapalogs in relation to their adverse effects.
SEARCH METHODS
We identified relevant studies from the Cochrane-Central-Register-of-Controlled-Trials (CENTRAL), Ovid MEDLINE and ongoing trials registries with no language restrictions. We searched conference proceedings and abstract books of conferences. Date of the last searches: 15 July 2022.
SELECTION CRITERIA
Randomised controlled trials (RCTs) or quasi-RCTs of rapamycin or rapalogs in people with TSC.
DATA COLLECTION AND ANALYSIS
Two review authors independently extracted data and assessed the risk of bias of each study; a third review author verified the extracted data and risk of bias decisions. We assessed the certainty of the evidence using GRADE.
MAIN RESULTS
The current update added seven RCTs, bringing the total number to 10 RCTs (with 1008 participants aged 3 months to 65 years; 484 males). All TSC diagnoses were by consensus criteria as a minimum. In parallel studies, 645 participants received active interventions and 340 placebo. Evidence is low-to-high certainty and study quality is mixed; mostly a low risk of bias across domains, but one study had a high risk of performance bias (lack of blinding) and three studies had a high risk of attrition bias. Manufacturers of the investigational products supported eight studies. Systemic administration Six studies (703 participants) administered everolimus (rapalog) orally. More participants in the intervention arm reduced renal angiomyolipoma size by 50% (risk ratio (RR) 24.69, 95% confidence interval (CI) 3.51 to 173.41; P = 0.001; 2 studies, 162 participants, high-certainty evidence). In the intervention arm, more participants in the intervention arm reduced SEGA tumour size by 50% (RR 27.85, 95% CI 1.74 to 444.82; P = 0.02; 1 study; 117 participants; moderate-certainty evidence) ,and reported more skin responses (RR 5.78, 95% CI 2.30 to 14.52; P = 0.0002; 2 studies; 224 participants; high-certainty evidence). In one 18-week study (366 participants), the intervention led to 25% fewer seizures (RR 1.63, 95% CI 1.27 to 2.09; P = 0.0001) or 50% fewer seizures (RR 2.28, 95% CI 1.44 to 3.60; P = 0.0004); but there was no difference in numbers being seizure-free (RR 5.30, 95% CI 0.69 to 40.57; P = 0.11) (moderate-certainty evidence). One study (42 participants) showed no difference in neurocognitive, neuropsychiatry, behavioural, sensory and motor development (low-certainty evidence). Total adverse events (AEs) did not differ between groups (RR 1.09, 95% CI 0.97 to 1.22; P = 0.16; 5 studies; 680 participants; high-certainty evidence). However, the intervention group experienced more AEs resulting in withdrawal, interruption of treatment, or reduced dose (RR 2.61, 95% CI 1.58 to 4.33; P = 0.0002; 4 studies; 633 participants; high-certainty evidence and also reported more severe AEs (RR 2.35, 95% CI 0.99 to 5.58; P = 0.05; 2 studies; 413 participants; high-certainty evidence). Topical (skin) administration Four studies (305 participants) administered rapamycin topically. More participants in the intervention arm showed a response to skin lesions (RR 2.72, 95% CI 1.76 to 4.18; P < 0.00001; 2 studies; 187 participants; high-certainty evidence) and more participants in the placebo arm reported a deterioration of skin lesions (RR 0.27, 95% CI 0.15 to 0.49; 1 study; 164 participants; high-certainty evidence). More participants in the intervention arm responded to facial angiofibroma at one to three months (RR 28.74, 95% CI 1.78 to 463.19; P = 0.02) and three to six months (RR 39.39, 95% CI 2.48 to 626.00; P = 0.009; low-certainty evidence). Similar results were noted for cephalic plaques at one to three months (RR 10.93, 95% CI 0.64 to 186.08; P = 0.10) and three to six months (RR 7.38, 95% CI 1.01 to 53.83; P = 0.05; low-certainty evidence). More participants on placebo showed a deterioration of skin lesions (RR 0.27, 95% CI 0.15 to 0.49; P < 0.0001; 1 study; 164 participants; moderate-certainty evidence). The intervention arm reported a higher general improvement score (MD -1.01, 95% CI -1.68 to -0.34; P < 0.0001), but no difference specifically in the adult subgroup (MD -0.75, 95% CI -1.58 to 0.08; P = 0.08; 1 study; 36 participants; moderate-certainty evidence). Participants in the intervention arm reported higher satisfaction than with placebo (MD -0.92, 95% CI -1.79 to -0.05; P = 0.04; 1 study; 36 participants; low-certainty evidence), although again with no difference among adults (MD -0.25, 95% CI -1.52 to 1.02; P = 0.70; 1 study; 18 participants; low-certainty evidence). Groups did not differ in change in quality of life at six months (MD 0.30, 95% CI -1.01 to 1.61; P = 0.65; 1 study; 62 participants; low-certainty evidence). Treatment led to a higher risk of any AE compared to placebo (RR 1.72, 95% CI 1.10, 2.67; P = 0.02; 3 studies; 277 participants; moderate-certainty evidence); but no difference between groups in severe AEs (RR 0.78, 95% CI 0.19 to 3.15; P = 0.73; 1 study; 179 participants; moderate-certainty evidence).
AUTHORS' CONCLUSIONS
Oral everolimus reduces the size of SEGA and renal angiomyolipoma by 50%, reduces seizure frequency by 25% and 50% and implements beneficial effects on skin lesions with no difference in the total number of AEs compared to placebo; however, more participants in the treatment group required a dose reduction, interruption or withdrawal and marginally more experienced serious AEs compared to placebo. Topical rapamycin increases the response to skin lesions and facial angiofibroma, an improvement score, satisfaction and the risk of any AE, but not severe adverse events. With caution regarding the risk of severe AEs, this review supports oral everolimus for renal angiomyolipoma, SEGA, seizure, and skin lesions, and topical rapamycin for facial angiofibroma.
Topics: Adult; Male; Humans; MTOR Inhibitors; Sirolimus; Everolimus; Angiofibroma; Angiomyolipoma; Tuberous Sclerosis; Astrocytoma; Kidney Neoplasms
PubMed: 37432030
DOI: 10.1002/14651858.CD011272.pub3 -
Clinical Radiology Jun 2023To determine the feasibility of spectral computed tomography (CT) in the differentiation of focal liver lesions from hepatocellular carcinoma (HCC) using a network... (Meta-Analysis)
Meta-Analysis
AIM
To determine the feasibility of spectral computed tomography (CT) in the differentiation of focal liver lesions from hepatocellular carcinoma (HCC) using a network meta-analysis (NMA).
MATERIALS AND METHODS
The review was completed in accordance with PRISMA guidelines. Searches of three medical databases were performed. A total of nine articles were found for the qualitative synthesis. The meta-analysis was performed on five studies for the normalised iodine concentration (NIC; which is the iodine concentration in the lesion divided by the iodine concentration in the aorta) and the lesion-normal parenchyma iodine ratio (LNR; which is the iodine concentration in the lesion divided by the iodine concentration in the non-tumour hepatic parenchyma) on portal venous and arterial phase images due to sufficient data.
RESULTS
Spectral CT can be used to differentiate HCC from hepatic haemangioma (HH), focal nodular hyperplasia (FNH), regenerative nodules, neuroendocrine tumours (NETs), abscesses, and angiomyolipoma (AML). Hepatic metastases versus abscess and FNH versus HH could also be differentiated. The NMA demonstrated that HCC, NETs, and regenerative nodules could be differentiated due to lower quantitative iodine values. FNH, AML, and HH all had higher values.
CONCLUSION
Spectral CT shows promise in differentiating focal liver lesions. Studies with larger sample sizes are warranted. Future studies should be performed comparing benign lesions using quantitative markers.
Topics: Humans; Carcinoma, Hepatocellular; Liver Neoplasms; Contrast Media; Diagnosis, Differential; Tomography, X-Ray Computed; Liver; Iodine; Focal Nodular Hyperplasia; Angiomyolipoma; Hemangioma; Leukemia, Myeloid, Acute
PubMed: 37019736
DOI: 10.1016/j.crad.2023.02.017 -
Medicine Dec 2022Lymphangioleiomyomatosis (LAM) is a rare disease involving multiple systems, which is divided into sporadic LAM (S-LAM) and tuberous sclerosis complex-LAM, mostly...
BACKGROUND
Lymphangioleiomyomatosis (LAM) is a rare disease involving multiple systems, which is divided into sporadic LAM (S-LAM) and tuberous sclerosis complex-LAM, mostly affecting women who are in childbearing age stage. Data on male patients are limited and scattered. Therefore, it is necessary to conduct a systematic review to investigate the clinical features, diagnosis, treatment, and outcomes of LAM in male.
METHODS
We performed a literature review by searching for all the published reported cases of LAM in male during the past 35 years (April 1986-October 2021).
RESULTS
36 male patients described in 26 references were included in this article. The median age of onset was 34 years (interquartile range: 1-79). The most common initial manifestations were cough, dyspnea, respite, and hemoptysis, with pulmonary complications such as pneumothorax and chylothorax. Five patients (13.9%) were asymptomatic at admission. Nearly half of the 36 male patients had thin-walled air-filled cysts that were visible throughout both lungs. Considering the abovementioned atypical clinical features, misdiagnosis was committed in 8 patients (22.2%). In addition, patients with tuberous sclerosis complex lymphangioleiomyomatosis often have no pulmonary manifestations at onset but present multiple extrapulmonary manifestations and have higher rates of renal angiomyolipomas than patients with S-LAM (P < 0.01). Eventually, 4 patients with S-LAM eventually died.
CONCLUSION
Physicians should increase the awareness of LAM in male. Early monitoring of various systems should be recommended to ensure early management and active follow-up. Tuberous sclerosis complex patients should immediately be tracked for the onset of LAM disease to improve prognosis.
Topics: Humans; Male; Female; Adult; Lymphangioleiomyomatosis; Tuberous Sclerosis; Prognosis; Angiomyolipoma; Kidney Neoplasms; Lung Neoplasms
PubMed: 36596036
DOI: 10.1097/MD.0000000000032492 -
Current Urology Mar 2022Misdiagnosis of benign renal neoplasms can lead to unnecessary surgical resections, which increases the risk of other morbidities and mortality. Therefore, it is crucial... (Review)
Review
Misdiagnosis of benign renal neoplasms can lead to unnecessary surgical resections, which increases the risk of other morbidities and mortality. Therefore, it is crucial to find a diagnostic modality for differentiation between benign and malignant renal masses. In the current study, we summarized published pieces of evidence concerning the use of technetium-99m (Tc)-sestamibi single-photon emission computed tomography/computed tomography (SPECT/CT) as a promising diagnostic nuclear imaging modality for the differentiation of renal neoplasms. The study was conducted according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement for Systematic Reviews and Meta-Analyses. We conducted a systematic electronic database search for suitable studies from inception till February 20, 2020 in 9 databases. The risk of bias was assessed for the included studies using the Quality Assessment of Diagnostic Accuracy Studies-2 tool. We identified 9373 records after exclusion of 8978 duplicates using EndNote software. Title and abstract screening resulted in 761 records for further full-text screening. Finally, four studies were included with total sample size of 80 patients. The overall risk of bias was low to moderate. The results of all the included studies supported using Tc-sestamibi SPECT/CT for the differentiation between benign and malignant renal neoplasms. The use of Tc-sestamibi SPECT/CT could be a rapid, less invasive, promising diagnostic modality for histological diagnosis and staging of renal neoplasm, as well as monitoring post-therapy tumor's response. However, more studies with large sample sizes are essential to confirm the reliability and accuracy of this modality for usage.
PubMed: 35633856
DOI: 10.1097/CU9.0000000000000089 -
Cureus Feb 2022The aim of this review is to evaluate the current evidence regarding the best management in terms of active surveillance of angiomyolipoma (AML) cases less than 4 cm,... (Review)
Review
The aim of this review is to evaluate the current evidence regarding the best management in terms of active surveillance of angiomyolipoma (AML) cases less than 4 cm, particularly the optimal timing of active surveillance. In addition, we aimed to describe their initial size, clinical presentation, and growth rates. The present systematic review included prospective and retrospective studies that evaluated and followed up patients with AML through active surveillance. Studies were retrieved through an online bibliographic search of the Medline database via PubMed, SCOPUS, Web of Science, and Cochrane Library from their inception to January 2022. Seven studies were included in the present systematic review. Concerning the active surveillance protocol, only four studies describe the frequency of active surveillance and the utilized imaging modality. Some studies followed up lesions by ultrasound annually for two to five years, while other studies followed-up patients twice for the first year, then annually for a median follow-up period of 49 (9-89) months. The used modalities were ultrasound, CT, and magnetic resonance imaging (MRI). Notably, the incidence of spontaneous bleeding was consistent across the included studies (ranging from 2.3 - 3.1%), except for one study which showed an incidence rate of 15.3%. In terms of the need for active treatment, the rate of active treatment was slightly higher in some studies than the others. However, this variation could not be considered clinically relevant to favor one surveillance strategy over the other. We concluded that active surveillance is the first line of management in all small asymptomatic ALMs. ALMs less than 2 cm do not require active surveillance. The current published literature suggested that active surveillance for two years may provide the same benefits as a five-year surveillance strategy, with fewer radiation hazards and less socioeconomic burden.
PubMed: 35371642
DOI: 10.7759/cureus.22678 -
Urology Annals 2021Renal angiomyolipoma (AML) is the most frequent mesenchymal tumor of the kidney. Although there is a rare possibility of malignant transformation of AML, this risk has...
BACKGROUND
Renal angiomyolipoma (AML) is the most frequent mesenchymal tumor of the kidney. Although there is a rare possibility of malignant transformation of AML, this risk has not been studied in immunosuppressed patients. The safety of donors with AML and their kidney transplant recipients has not been well established.
METHODS
A literature search was conducted utilizing MEDLINE, EMBASE, and Cochrane databases from inception through May 15, 2018 (updated on October 2019). We included studies that reported the outcomes of kidney donors with AML or recipients of donor with AML. The protocol for this meta-analysis is registered with PROSPERO (International Prospective Register of Systematic Reviews; no. CRD42018095157).
RESULTS
Fourteen studies with a total of 16 donors with AML were identified. None of the donors had a diagnosis of tuberous sclerosis complex (TSC), pulmonary lymphangioleiomyomatosis (LAM), or epithelioid variant of AML. Donor age ranged from 35 to 77 years, and recipient age ranged from 27 to 62 years. Ninety-two percent of the donors were female. Only 8% were deceased donor renal transplant. The majority underwent resection (65%) before transplantation, followed by no resection (18%), and the remaining had resection. Tumor size varied from 0.4 cm to 7 cm, and the majority (87%) were localized in the right kidney. Follow-up time ranged from 1 to 107 months. Donor creatinine prenephrectomy ranged 0.89-1.1 mg/dL and postnephrectomy creatinine 1.0-1.17 mg/dL. In those who did not have resection of the AML, tumor size remained stable. None of the donors with AML had end-stage renal disease or died at last follow-up. None of the recipients had malignant transformation of AML.
CONCLUSION
These findings are reassuring for the safety of donors with AML (without TSC or LAM) as well as their recipients without evidence of malignant transformation of AML. As such, this can also positively impact the donor pool by increasing the number of available kidneys.
PubMed: 33897168
DOI: 10.4103/UA.UA_14_20 -
Cancers Mar 2021Radiomics may increase the diagnostic accuracy of medical imaging for localized and metastatic RCC (mRCC). A systematic review and meta-analysis was performed. Doing so,... (Review)
Review
Radiomics may increase the diagnostic accuracy of medical imaging for localized and metastatic RCC (mRCC). A systematic review and meta-analysis was performed. Doing so, we comprehensively searched literature databases until May 2020. Studies investigating the diagnostic value of radiomics in differentiation of localized renal tumors and assessment of treatment response to ST in mRCC were included and assessed with respect to their quality using the radiomics quality score (RQS). A total of 113 out of 1098 identified studies met the criteria and were included in qualitative synthesis. Median RQS of all studies was 13.9% (5.0 points, IQR 0.25-7.0 points), and RQS increased over time. Thirty studies were included into the quantitative synthesis: For distinguishing angiomyolipoma, oncocytoma or unspecified benign tumors from RCC, the random effects model showed a log odds ratio (OR) of 2.89 (95%-CI 2.40-3.39, < 0.001), 3.08 (95%-CI 2.09-4.06, < 0.001) and 3.57 (95%-CI 2.69-4.45, < 0.001), respectively. For the general discrimination of benign tumors from RCC log OR was 3.17 (95%-CI 2.73-3.62, < 0.001). Inhomogeneity of the available studies assessing treatment response in mRCC prevented any meaningful meta-analysis. The application of radiomics seems promising for discrimination of renal tumor dignity. Shared data and open science may assist in improving reproducibility of future studies.
PubMed: 33802699
DOI: 10.3390/cancers13061348 -
International Journal of Surgery... Dec 2020To investigate the overall prevalence of benign pathology after partial nephrectomy (PN) and identify predictive factors for benign pathology after PN. (Meta-Analysis)
Meta-Analysis
OBJECTIVE
To investigate the overall prevalence of benign pathology after partial nephrectomy (PN) and identify predictive factors for benign pathology after PN.
METHODS
A systematic review was performed following the PRISMA guidelines. PubMed/Medline, Embase, and the Cochrane Library were searched up to January 2019PRISMA guidelines. The data for the meta-analysis and network meta-analysis were pooled using a random-effects model.
RESULTS
There were 144 studies included in the final analysis, which was comprised of 79 observational studies (n = 37,300) and 65 comparative studies (n = 18,552). The overall prevalence rate of benign pathology after PN was 0.19 (95% CI: 0.18-0.21). According to the procedure types, the prevalence rate of benign pathology was 0.17 (95% CI: 0.15-0.19), 0.24 (95% CI: 0.22-0.27), and 0.16 (95% CI: 0.15-0.18) in open partial nephrectomy, laparoscopic partial nephrectomy, and robot-assisted laparoscopic partial nephrectomy, respectively. The significant moderating factors were gender, publication year, the origin of the study, and procedure types. The three most common benign pathology types were oncocytomas, angiomyolipomas, and renal cysts (44.50%, 30.20%, and 10.99%, respectively).
CONCLUSIONS
The overall prevalence of benign pathology after PN was not low and it was affected by female gender, studies published before 2010, studies originating from Western areas, and laparoscopic procedure types.
Topics: Angiomyolipoma; Humans; Kidney Neoplasms; Laparoscopy; Nephrectomy; Prevalence; Robotic Surgical Procedures
PubMed: 33220454
DOI: 10.1016/j.ijsu.2020.11.009