Renal Neoplasia in Polycystic Kidney Disease: An Assessment of Tuberous Sclerosis Complex-associated Renal Neoplasia and PKD1/TSC2 Contiguous Gene Deletion Syndrome.

To determine the incidence of renal neoplasia among patients undergoing nephrectomy for polycystic kidney disease (PKD), we queried our institutional nephrectomy registry (years 2000-2020). Approximately 4% (231 of 5757) of patients who underwent nephrectomy had PKD, and 26 of these 231 patients (11.3%) had renal neoplasia. Tumors from an additional two patients with PKD were also evaluated. Patients with PKD who had tuberous sclerosis complex (TSC)-associated renal neoplasia were screened for PKD1/TSC2 contiguous gene deletion syndrome (CGS) using single nucleotide polymorphism arrays. The median age of patients with PKD and renal neoplasia at nephrectomy was 54 yr. The median tumor size was 2.0 cm and the tumors were predominantly of low grade and stage. The tumors consisted of 23 renal cell carcinomas (RCCs), one epithelioid angiomyolipoma, and four angiomyolipomas. The median follow-up was 59.5 mo (n = 26) and only one patient with clear cell RCC developed metastases. Two patients with angiomyolipomas had PKD1/TSC2 CGS. Our results support screening of patients with PKD and TSC-associated renal neoplasia as well as TSC patients with cystic renal disease for CGS, as identification of patients with CGS can better define the manifestation and prognosis of CGS and guide counseling regarding patterns of inheritance. PATIENT SUMMARY: We identified patients with abnormal kidney cell growth (called renal neoplasia) among those undergoing removal of kidney tissue for polycystic kidney disease (PKD) and patients with a syndrome involving deletions in two genes, called PKD1/TSC2 contiguous gene deletion syndrome (CGS) at our institution. Of 231 PKD patients with removal of kidney tissue, 11.3% had renal neoplasia, and two patients with angiomyolipoma tumors had PKD1/TSC2 CGS. Detection of renal neoplasia associated with a condition called tuberous sclerosis complex in PKD may increase the identification of patients with PKD1/TSC2 CGS and guide patient counseling regarding outcomes and patterns of inheritance.

A Comprehensive Computer-Assisted Diagnosis System for Early Assessment of Renal Cancer Tumors.

Renal cell carcinoma (RCC) is the most common and a highly aggressive type of malignant renal tumor. In this manuscript, we aim to identify and integrate the optimal discriminating morphological, textural, and functional features that best describe the malignancy status of a given renal tumor. The integrated discriminating features may lead to the development of a novel comprehensive renal cancer computer-assisted diagnosis (RC-CAD) system with the ability to discriminate between benign and malignant renal tumors and specify the malignancy subtypes for optimal medical management. Informed consent was obtained from a total of 140 biopsy-proven patients to participate in the study (male = 72 and female = 68, age range = 15 to 87 years). There were 70 patients who had RCC (40 clear cell RCC (ccRCC), 30 nonclear cell RCC (nccRCC)), while the other 70 had benign angiomyolipoma tumors. Contrast-enhanced computed tomography (CE-CT) images were acquired, and renal tumors were segmented for all patients to allow the extraction of discriminating imaging features. The RC-CAD system incorporates the following major steps: (i) applying a new parametric spherical harmonic technique to estimate the morphological features, (ii) modeling a novel angular invariant gray-level co-occurrence matrix to estimate the textural features, and (iii) constructing wash-in/wash-out slopes to estimate the functional features by quantifying enhancement variations across different CE-CT phases. These features were subsequently combined and processed using a two-stage multilayer perceptron artificial neural network (MLP-ANN) classifier to classify the renal tumor as benign or malignant and identify the malignancy subtype as well. Using the combined features and a leave-one-subject-out cross-validation approach, the developed RC-CAD system achieved a sensitivity of 95.3%±2.0%, a specificity of 99.9%±0.4%, and Dice similarity coefficient of 0.98±0.01 in differentiating malignant from benign tumors, as well as an overall accuracy of 89.6%±5.0% in discriminating ccRCC from nccRCC. The diagnostic abilities of the developed RC-CAD system were further validated using a randomly stratified 10-fold cross-validation approach. The obtained results using the proposed MLP-ANN classification model outperformed other machine learning classifiers (e.g., support vector machine, random forests, relational functional gradient boosting, etc.). Hence, integrating morphological, textural, and functional features enhances the diagnostic performance, making the proposal a reliable noninvasive diagnostic tool for renal tumors.

Kidney Imaging Surveillance in Commercially Insured Patients With Tuberous Sclerosis Complex.

BACKGROUND: Kidney disease has historically been the primary source of early mortality in adults with tuberous sclerosis complex (TSC). Kidney imaging surveillance promotes early detection of lesions requiring intervention. We describe kidney imaging frequency in relationship to patient-level characteristics for commercially insured patients with TSC in the United States. METHODS: This retrospective observational study used 2003 to 2016 enrollment and claims data from a de-identified fully insured commercial health insurer. Patients with TSC less than 65 years were included. The patient-level kidney imaging rate was calculated as the number of kidney imaging procedures divided by length of continuous enrollment. A multiple linear regression model was used to determine the relationship between imaging rate and progression of TSC-associated kidney disease, number of specialists seen, and nephrologist care. RESULTS: At least half of the 70 patients with TSC included in the study were aged 16 years or younger. Over a follow-up period of up to 14 years, the median kidney imaging rate was 0.13 procedures per year with 43% (N = 30) of patients lacking evidence of kidney imaging during the observation period. Imaging frequency increased with progression of TSC-associated kidney disease, more specialists, and nephrologist care (P < 0.05 for all three in regression model). CONCLUSIONS: A substantial percentage of patients with TSC in the United States are at risk for delayed detection of kidney manifestations due to infrequent kidney imaging surveillance. Multispecialty care, including neurologists, may positively affect kidney surveillance rates.

Everolimus compliance and persistence among tuberous sclerosis complex patients with renal angiomyolipoma or subependymal giant cell astrocytoma.

OBJECTIVE: Everolimus is the only FDA approved drug to treat renal angiomyolipoma or subependymal giant-cell astrocytoma (SEGA) in tuberous sclerosis complex (TSC). Potential differences exist between patients with commercial and Medicaid insurance on everolimus use; however, there is limited information from the real world. This study compared compliance and persistence of everolimus between commercial and Medicaid patients using US claims data. METHODS: Patients with ≥1 claim of TSC with renal angiomyolipoma or SEGA were selected from the MarketScan commercial (1 January 2009-31 August 2016) and Medicaid (1 January 2009-30 June 2015) databases. Patients were followed from index date (the earliest date of TSC, renal angiomyolipoma or SEGA diagnosis) to death or end of data. Non-persistence, defined as ≥60 day gap without everolimus, and medication possession ratio (MPR) were assessed among the subset of patients with ≥1 year of follow-up from the first everolimus claim. RESULTS: A total of 1497 TSC patients met the study criteria (896 renal angiomyolipoma only, 411 SEGA only and 190 both). Compared to Medicaid patients (N = 513), commercial patients (N = 984) had the same ages (22 years) but a shorter length of follow-up (38 vs. 48 months, p < .001). Medicaid and commercial patients had similar rates of being treated with everolimus (14.4% vs. 13.6%, p = .668), but it took Medicaid patients a longer time to start everolimus (871 vs. 704 days, p < .001). Although the non-persistence rate was not significantly different between commercial and Medicaid patients (42.5% vs. 35.1%, p = .561), the number of days from everolimus initiation to non-persistence was significantly lower for commercial patients (945 vs. 1132, p < .001). During the 1 year post everolimus initiation, commercial patients had a significantly higher MPR (0.81 vs. 0.74, p < .001) and higher percentage of patients with MPR ≥0.80 (67.8% vs. 58.1%, p < .001). CONCLUSIONS: Among TSC patients with renal angiomyolipoma or SEGA and treated with everolimus, everolimus MPR was between 0.74 and 0.81. Medicaid patients had lower MPR than commercial patients but better persistence.

Are We Overtreating Renal Angiomyolipoma: A Review of the Literature and Assessment of Contemporary Management and Follow-Up Strategies.

Renal angiomyolipoma (AML) are benign tumours composed of fat, muscle, and disorganised blood vessels. Historic treatment algorithms for sporadic AML based on size fail to consider additional risk factors such as tumour vascularity and pseudoaneurysm formation. As AML is now predominantly incidental, rupture is rare and its mortality low. The recent publication of the largest longitudinal series to date also suggest that growth is uncommon, challenging existing surveillance paradigms. The evidence assessing treatment strategies in AML are reviewed, with particular emphasis on incidental sporadic AML. The relative merits of various AML treatments are discussed, and areas of clinical uncertainty highlighted.

Healthcare utilization and costs in patients with tuberous sclerosiscomplex-related renal angiomyolipoma.

OBJECTIVE: To quantify healthcare utilization and costs in patients with tuberous sclerosis complex (TSC) and renal angiomyolipoma (AML) in a matched cohort of patients without TSC or AML. METHODS: Administrative data from the MarketScan Research Databases were used to select patients with TSC and renal AML during January 1, 2000-March 31, 2013 from the Commercial database and January 1, 2000-June 30, 2012 from the Medicaid database. Patients were required to have at least 30 days of follow-up from initiation into the study, and were followed until inpatient death, end of insurance coverage, or the end of study. Age, calendar year, and payer-matched controls that had no TSC and no AML were selected. All-cause annualized healthcare utilization and costs were calculated by service category. RESULTS: A total of 218 patients under 18 years and 377 patients 18 years and older with TSC-renal AML were selected from the Commercial database, and matched to 654 and 1,131 controls, respectively. Thirty-eight patients under 18 years and 110 patients 18 years or older with TSC-renal AML were selected from the Medicaid database, and matched to 54 and 212 controls, respectively. Within the Commercial cohort, and across both age groups, TSC-renal AML patients utilized more healthcare services than their matched controls. Within the Medicaid cohort, in both age groups, utilization was higher in TSC-renal AML patients vs control patients for inpatient admissions, emergency room visits, physician office visits, and hospital-based outpatient visits. Across age groups and in both the Commercial and Medicaid cohorts, the annual average total costs were significantly higher in TSC-renal AML patients compared to control patients (p < 0.05 for all). Healthcare costs ranged from $29,240-$48,499 for TSC-renal AML patients and from $2,082-$10,864 for control patients. CONCLUSIONS: Compared to controls, TSC-renal AML patients incurred substantially higher annual healthcare utilization and costs.

Kidney involvement in tuberous sclerosis complex: the impact on healthcare resource use and costs.

OBJECTIVE: Tuberous sclerosis complex (TSC) is associated with non-malignant kidney lesions-angiomyolipomata-that may be associated with chronic kidney disease (CKD). This study investigated the relationship between renal angiomyolipomata and CKD in TSC, including the impact on healthcare resource utilization (HCRU) and costs. METHODS: This was a retrospective, longitudinal cohort study based on medical record data spanning January 1990-April 2012 for 369 TSC patients treated at a specialty center in the Netherlands. Cohorts were established based on CKD stage and angiomyolipoma size. Rates of HCRU (physician visits, monitoring, and interventions) were compared across cohorts using rate ratios. Healthcare costs were compared across cohorts using cost differences. Regression models were used to identify predictive factors for HCRU and healthcare costs. RESULTS: Sixteen per cent of patients reached CKD stage 3 or higher during follow-up. Patients at more advanced stages of CKD more frequently had either large or multiple small angiomyolipomata and higher HCRU rates and healthcare costs. In the multivariate analyses, male gender, CKD stage >1, angiomyolipoma size ≥3.5 cm, embolization, and the presence of moderate or severe lymphangioleiomyomatosis (LAM) were associated with greater HCRU (p ≤ 0.002 for all comparisons). Definite (vs suspected) TSC diagnosis, CKD stage 5 (vs CKD stage 1), angiomyolipoma size ≥3.5 cm, and moderate or severe LAM were associated with higher costs (p = 0.050 for TSC diagnosis, p ≤ 0.002 for other comparisons). Costs in CKD stage 5 were driven primarily by dialysis. CONCLUSIONS: A substantial proportion of patients with TSC developed moderate-to-severe CKD, which was associated with renal angiomyolipomata and increased HCRU and costs.

Rates of interventional procedures in patients with tuberous sclerosis complex-related renal angiomyolipoma.

OBJECTIVE: To describe rates of renal artery embolization, partial nephrectomy, and complete nephrectomy in patients with tuberous sclerosis complex (TSC) and renal angiomyolipoma. METHODS: Data from the MarketScan® Research Databases were used to select patients with TSC and renal angiomyolipoma during January 1, 2000-March 31,2013 (Commercial database) and January 1, 2000-June 30, 2012 (Medicaid database). Patients had at least 30 days of follow-up and were followed until the earliest of inpatient death, end of enrollment, or end of study. Rates of embolization and nephrectomy were calculated. RESULTS: In total, 218 patients <18 years (mean = 9.7 years) and 378 patients ≥18 years (mean 36.9 years) were selected from the Commercial database. Fifty-nine patients <18 years (mean = 7.2 years) and 117 patients ≥18 years (mean = 37.2 years) were selected from the Medicaid database. Follow-up in the Medicaid cohorts was approximately twice that of the Commercial cohorts. Among patients in the study, 24.2% had at least one interventional procedure: 15.2% had embolization, 5.2% had partial nephrectomy, and 7.6% had complete nephrectomy. Within the Commercial cohort ≥18 years, 18.5% had embolization, 7.7% had partial nephrectomy, and 11.4% had complete nephrectomy. Corresponding percentages in the Medicaid adult cohort were 17.1%, 5.1%, and 4.3%. Repeat embolization procedures occurred in up to 7.7% of Commercial patients and in up to 6.8% of Medicaid patients. Repeat partial nephrectomy occurred in up to 4.5% and 1.7% of Commercial and Medicaid patients, respectively. CONCLUSIONS: Approximately 25% of patients with TSC-renal angiomyolipoma experienced embolization or nephrectomy, with some patients undergoing repeat procedures. Study limitations included small sample sizes, the majority of the study period occurred prior to the approval of mammalian target of rapamycin inhibitors for the treatment of TSC-renal AML, and results may not be generalizable to patients with insurance other than commercial or Medicaid.

Correlation between radiological assessment and histopathological diagnosis in retroperitoneal tumors: analysis of 291 consecutive patients at a tertiary reference sarcoma center.

OBJECTIVES: Aim of study was to assess the correlation between computed tomography scan (CT) findings and histopathology. MATERIAL AND METHODS: Data were collected on consecutive patients with suspected retroperitoneal sarcoma (RPS) referred to a tertiary sarcoma center. Patients underwent contrast enhanced multi-detector CT scans. Radiological features of lesions were classified according to the presence of a fatty (Group A) mass, or non-fatty (Group B) mass, both subdivided according to homogeneity and intralesional high-contrasted appearance. Radiological classification was compared with histopathological diagnosis. Sensitivity, specificity, positive/negative predictive value (PPV, NPV) were analyzed. RESULTS: Of 291 patients, 103/291 (35.4%) masses were classified in Group A and 188/291 (64.6%) in Group B. Diagnosis of mesenchymal tumor was obtained in 231/291 cases (79%) and non-mesenchymal tumor in 60/291 (21%). Sensitivity and specificity of Group A for liposarcoma were 76.7% and 92.0%; PPV and NPV were 86.4% and 85.6%. Sensitivity of Group B for a mesenchymal tumor was 55.4% and specificity was 0%; PPV and NPV were 68.1% and 0%. CONCLUSIONS: None of radiological criteria were sufficient to anticipate a specific diagnosis, with the only exception of well differentiated liposarcoma and angiomyolipoma. In a series of suspected RPS, 21% of the lesions were finally non-mesenchymal tumors.

Assessment of renal lesions with blood oxygenation level-dependent MRI at 3 T: preliminary experience.

OBJECTIVE: The purpose of this article is to prospectively determine the feasibility of using blood oxygenation level-dependent (BOLD) MRI at 3 T in differentiating various renal lesions. SUBJECTS AND METHODS: Seventy-one patients (mean age, 53.8 years; range, 22-80 years) underwent kidney MRI, including BOLD MRI at 3 T. BOLD MRI was performed using a multiple fast-field echo (FFE) sequence to acquire 20 T2(*)-weighted images within a single breath-hold. The rate of spin dephasing (R2(*)) values of solid and benign cystic renal lesions were compared, and the results of four subgroups--simple cysts, renal cell carcinomas (RCCs), hemorrhagic cysts, and angiomyolipomas (AMLs)--were analyzed. The degree of overall imaging quality in BOLD MRI was also assessed. RESULTS: One hundred three renal lesions were detected in the 71 patients. The mean (± SD) R2(*) values of 45 solid lesions (30.6 ± 19.4 1/s) were significantly higher than those of 58 benign cystic lesions (4.5 ± 4.8 1/s; p < 0.001). The mean R2(*) values of simple cysts, RCCs, hemorrhagic cysts, and AMLs were 1.7 ± 1.7, 22.6 ± 12.6, 10.7 ± 3.7, and 48.0 ± 25.5 1/s, respectively (p < 0.001). The degree of overall imaging quality in all patients was satisfactory or better. CONCLUSION: Renal BOLD MRI at 3 T is a feasible technique and may be useful for differentiating various renal lesions.

Pure epithelioid PEComas (so-called epithelioid angiomyolipoma) of the kidney: A clinicopathologic study of 41 cases: detailed assessment of morphology and risk stratification.

Epithelioid angiomyolipomas (perivascular epithelioid cell tumors) of the kidney are defined as potentially malignant mesenchymal lesions that are closely related to classic angiomyolipoma. Although approximately 120 cases are published, mostly as case reports with variably used diagnostic criteria, the pathologic prognostic predictors of outcome are unknown. We analyzed the clinicopathologic parameters in a large series of 41 cases of pure epithelioid angiomyolipomas of the kidney, which we designate as pure (monotypic) epithelioid PEComas to contrast them from classic angiomyolipomas that are regarded by some as PEComas. We use the terminology "pure" to separate these cases from those that may have variable epithelioid components. The mean age of the patients was 40.7 years (range, 14 to 68 y). The male-to-female ratio was 1:1. Seventy-nine percent of patients were symptomatic at presentation with metastatic disease at onset in 12 cases. Follow-up and/or disease progression information were available for 33 of 41 cases (mean, 44.5 mo and median, 24.5 mo; range, 4 to 240); 9 patients had a history of associated tuberous sclerosis. Recurrence and metastasis were seen in 17% and 49% of patients; 33% of patients died of disease. Lymph node involvement was seen in 24% of patients; the liver (63%), lung (25%), and mesentery (18.8%) were the most common metastatic sites. Clinicopathologic parameters associated with disease progression (recurrence, metastasis, or death due to disease) in univariate analysis included associated tuberous sclerosis complex or concurrent angiomyolipoma (any metastasis, P=0.046), necrosis (metastasis at diagnosis, P=0.012), tumor size >7 cm (progression, P=0.021), extrarenal extension and/or renal vein involvement (progression, P=0.023), and carcinoma-like growth pattern (progression, P=0.040) (the 5 adverse prognostic parameters for pure epithelioid PEComas). Tumors with <2 adverse prognostic parameters (13 cases) were considered to be low risk for progression tumor, with 15% having disease progression. Tumors with 2 to 3 adverse prognostic parameters (14 cases) were considered to be "intermediate risk," with 64% having disease progression. Tumors with more than 4 or more adverse prognostic parameters (6 cases) were considered to be high risk, with all patients having disease progression. Of tumors with 3 or more adverse prognostic parameters, 80% had disease progression. An exact logistic regression analytic model showed that only carcinoma-like growth pattern and extrarenal extension and/or renal vein involvement were significant predictors of outcome (P=0.009 and 0.033, respectively). Our data of a large series with uniform definitional criteria confirm the malignant potential for pure epithelioid PEComas and provide adverse prognostic parameters for risk stratification in these patients.

Ultrasound and CT imaging assessment of renal angiomyolipoma.

The authors report a case of a 41-year-old woman who was admitted to the emergency department of our hospital because of acute right flank pain. Laboratory investigations and cultures were negative. A transabdominal ultrasonography revealed a large mass of the upper pole of the right kidney as an incidental finding.

Assessment of triple-phase CT findings for the differentiation of fat-deficient hepatic angiomyolipoma from hepatocellular carcinoma in non-cirrhotic liver.

BACKGROUND: To evaluate the triple-phase CT findings for the differentiation of fat-deficient angiomyolipoma from hepatocellular carcinoma in non-cirrhotic liver. METHODS: We retrospectively reviewed contrast-enhanced triple-phase CT images of 10 patients with fat-deficient hepatic angiomyolipoma and 28 patients with 29 hepatocellular carcinomas in non-cirrhotic liver proved on histologic examination. The CT findings for the two types of tumors were compared using Fisher's exact test. RESULTS: Early draining vein depicted on arterial or portal phases was seen in eight (80%) angiomyolipomas and two hepatocellular carcinomas (7%) (p<0.001), in which the early draining vein was connected with tumoral vessels. The tumoral vessels in the angiomyolipoma were more prominent and ectatic, were distributed both centrally and peripherally, and were seen in smaller tumors than in the hepatocellular carcinoma. Tumor capsule enhancement was absent in all angiomyolipomas as compared with two (7%) hepatocellular carcinomas with no tumor capsule (p<0.001). The other CT findings were not significantly different for the two different types of tumors. CONCLUSIONS: The presence of early draining vein connecting with prominent tumoral vessels and absent tumor capsule were useful CT findings for the differentiation of fat-deficient angiomyolipoma from hepatocellular carcinoma in non-cirrhotic liver.

[Renal angiomyolipoma and fever: assessment with isotopic renogram and 67Ga scintigraphy]. / Angiomiolipoma renal y fiebre: valoración mediante renograma isotópico y gammagrafía con 67Ga.

A 26 year-old woman with tuberous sclerosis who came to the Emergency Department with high fever, bilious vomit, right hemiabdomen pain and syncope during 2 weeks. Laboratory analyses show hemoglobin 6.7 g/dl, creatinine 1.5 mg/dl and leukocytes 30,000. Abdominal CT is performed because of suspicion of active bleeding in right hemiabdomen, following rupture of right angiomyolipoma, treated by selective arterial embolization. She was referred to the Nuclear Medicine Department to perform a 67Gallium scintigraphy for the detection of infection, and static and dynamic renal scintigraphy for evaluation of the renal morphology and function.

Utility of frozen section analysis of resection margins during partial nephrectomy.

OBJECTIVES: To evaluate the utility of routine intraoperative frozen-section histologic analysis during partial nephrectomy to ensure negative surgical margins. Partial nephrectomy has gained acceptance for surgical treatment of small renal cancers. Many surgeons send specimens for intraoperative frozen section histologic analysis to ensure negative margins. METHODS: We reviewed the records of 78 patients who underwent partial nephrectomy for presumed malignancy. Patient demographics, intraoperative findings, and pathologic and clinical outcomes were analyzed. RESULTS: Seventy-nine partial nephrectomies were performed in 78 patients. Frozen sections were obtained intraoperatively in 76 cases. In 1 case (1.3%), a single margin was interpreted as positive for carcinoma, prompting deeper resection. The final histopathologic finding was interpreted as angiomyolipoma rather than carcinoma. The final pathologic examination revealed renal cell carcinoma in 52 (66%) of 79 cases. The mean oncologic follow-up was 16.2 months. One local recurrence was noted (1.9%). It arose in the resection bed 19 months after removal of a 4.5-cm tumor (pathologic Stage T3a). Both intraoperative frozen section margins and final pathologic margins were negative in this case. One patient developed pulmonary metastases and represented the only metastatic recurrence, as well as the only cancer-related death in our cohort (1.9%). CONCLUSIONS: Our data suggest that when partial nephrectomy is performed with attention to excising a perimeter of grossly normal-appearing parenchyma, sending specimens for intraoperative frozen section analyses may provide an unnecessary expense without providing meaningful, reliable information. Additional studies, including larger cohorts and extended follow-up, are needed to support these results.

Renal function after tumor enucleation: assessment by quantitative SPECT of 99mTc-dimercaptosuccinic acid uptake by the kidneys.

UNLABELLED: The purpose of this study was to evaluate the amount of functioning renal mass removed and the amount of remaining individual renal function after tumor enucleation. METHODS: Renal functional volume, percentage injected dose (%ID) per cubic centimeter of renal tissue and individual renal uptake of 24 operated and 24 contralateral kidneys were studied by two sequential SPECT quantitations of 99mTc-dimercaptosuccinic acid (DMSA) uptake by the kidneys (QDMSA). The first study was before surgery and the second study was 1 to 6 mo (mean 3.5 mo) after surgery. Mean tumor size was 3.4 +/- 0.99 cm and all tumors were confined to the renal parenchyma (stages pT1 and pT2). RESULTS: In the operated kidneys, there was a statistically significant decrease in renal cortical volume (170 +/- 46 mL after surgery versus 207 +/- 45 mL before surgery, t = 6.2, P < 0.001) and individual renal uptake (10.3% +/- 3.0% after surgery versus 13.0% +/- 2.9% before surgery, t = 5.4, P < 0.001). There was no statistically significant change after surgery compared with before surgery in the %ID per cubic centimeter of renal tissue of the operated kidneys, and in the volume, %ID per cubic centimeter and uptake of the contralateral normal kidneys. CONCLUSION: The results suggest that QDMSA is a noninvasive method able to assess changes in separate renal function. The limited functioning parenchymal loss after tumor enucleation had no effect on the opposite kidneys.

Lung transplantation for lymphangioleiomyomatosis: role of imaging in the assessment of complications related to the underlying disease.

PURPOSE: To identify the complications and imaging findings related to lymphangioleiomyomatosis (LAM) after lung transplantation. MATERIALS AND METHODS: The authors retrospectively reviewed the clinical histories and imaging studies of 13 patients from five major medical centers who underwent unilateral (n = 8) or bilateral (n = 5) lung transplantation for LAM between 1991 and 1997. Complications related to LAM, both before and after transplantation, were recorded. RESULTS: The following LAM-related complications were found during and after transplantation: excessive pleural adhesions (n = 4), native lung pneumothorax (n = 3), chylous effusion (n = 1), chylous ascites (n = 3), complications from renal angiomyolipomas (n = 4), and recurrent LAM (n = 1). Diagnosis could be made or suggested with computed tomography (CT) in all cases. Four patients (31%) died; one patient died of complications of LAM. CONCLUSIONS: Patients who have undergone lung transplantation for LAM have increased morbidity and mortality due to complications related to their underlying disease. These LAM-related complications can be diagnosed or suggested with CT.