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To evaluate clinicopathological features and survival outcomes of unilocular cystic renal cell carcinoma (ucRCC) compared with purely solid renal cell carcinoma (sRCC), and to evaluate the oncologic aggressiveness of ucRCC. The relevant data of 957 patients with sporadic unilateral renal cell carcinoma (RCC) underwent surgical treatment in 2 institutions from Jan 2014 to Oct 2018 were obtained. We excluded multilocular cystic renal neoplasm of low malignant potential (MCRNLMP), RCC with multilocular cysts and necrotic RCC. 74 ucRCCs were identified by pathology reports. We performed propensity score matching (PSM) and eventually selected 144 sRCCs. The clinicopathological features and survival outcomes were compared properly. After PSM, age, BMI, Charlson Comorbidity Index, and postoperative Chronic Kidney Disease grade were not significantly different. Both overall survival and progression-free survival of ucRCC were significantly better than sRCC by the log-rank test. Twenty-five cases of sRCCs were in the pT3 or pT4 stage, while no pT3 or pT4 tumors were found in ucRCCs. Fuhrman grade and lymphatic metastasis were found to be significant prognostic factors for the overall survival of ucRCC. Unilocular cystic RCC has a lower Fuhrman grade and pathological stage and a better prognosis compared with solid RCC. Patients with ucRCC still probably have lymphatic metastasis at surgery and may have postoperative metastasis, which is different from MCRNLMP. We recommend that the diagnosis of ucRCC should be reflected in pathology report. Different subtype of cystic RCC should be taken into consideration in counseling and management., (© 2022. The Author(s).)

Background: Gamma Knife radiosurgery (GKRS) has been proven to be a successful primary treatment for metastatic brain tumors (BM). BM can come in cystic lesions and are often too large for GKRS. An alternative approach to treat cystic BM is stereotactic cyst aspiration (SCA) for volume reduction, making it suitable for GKRS afterwards., Objective: Our objective is evaluation of volumetric reduction after SCA, tumor control, and complications after SCA directly followed by GKRS., Methods: We performed a retrospective analysis of all patients who underwent SCA directly followed by GKRS at the Gamma Knife Center of the Elisabeth-Tweesteden Hospital in Tilburg between 2002 and 2015. In total, 54 patients had undergone this combined approach. Two patients were excluded because of prior intracranial treatment. The other 52 patients were included for analysis., Results: SCA resulted in a mean volumetric reduction of 56.5% (range 5.50-87.00%). In 83.6% of the tumors (46 tumors), SCA led to sufficient volumetric reduction making GKRS possible. The overall local tumor control (OLTC) of the aspirated lesions post-GKRS was 60.9% (28 out of 46 tumors). Median progression-free survival (PFS) and overall survival (OS) for all patients were 3 (range 5 days-14 months) and 12 months (range 5 days-58 months), respectively. Leptomeningeal disease was reported in 5 (9.6%) cases., Conclusion: SCA directly followed by GKRS is an effective and time-efficient treatment for large cystic BM in selected patients in which surgery is contraindicated and those with deeply located lesions.

Objectives: Cholangitis and bile lakes are incompletely understood complications after portoenterostomy (PE). We investigated relationships between recurrent cholangitis, bile lakes, and clinical outcomes as well as surgical management of bile lakes., Methods: In this retrospective observational single institution study medical records and imaging studies of all patients who had undergone PE for biliary atresia during 1987 to 2016 (N = 61) were reviewed. We related occurrence of cholangitis episodes with the presence of intrahepatic bile lakes, patient characteristics, and PE outcomes. Risk factors for recurrent cholangitis and bile lakes, and management of bile lakes were analyzed., Results: Despite routine antibiotic prophylaxis median of 3.0 cholangitis episodes (0.75 episodes/year) occurred in 48 (79%) patients. Intrahepatic bile lakes were discovered in 8 (13%) patients by 16 months after PE. Overall, 54% had survived with their native liver at median age of 7.3 years and 28 (46%) patients had ≥1 cholangitis episodes/year. Number and frequency of cholangitis episodes were >5 times higher among patients with bile lakes (P < 0.001). Six patients underwent Roux-en-Y bile lake-jejunostomy, resulting in regression/disappearance of bile lakes and normalization of serum bilirubin in 5 with reduction of median yearly cholangitis rate from 8.8 to 1.1 (P = 0.028) and native liver survival of 6.3 (range, 1.3-17) years after the operation., Conclusions: Bile lakes are a significant risk factor for recurrent cholangitis after PE and efficiently treated by operative intestinal drainage providing prolonged jaundice-free native liver survival. Bile lakes should be actively screened among patients presenting with recurrent cholangitis after PE.

Background: Cyst infection is a frequent and serious complication of autosomal dominant polycystic kidney disease (ADPKD) that is often difficult to treat. However, the factors influencing cyst infection are still unknown. Therefore, we investigated clinical factors related to the duration of hospitalization and death due to cyst infection., Methods: All ADPKD patients undergoing cyst drainage or nephrectomy or partial hepatectomy at Toranomon Hospital from January 2004 to March 2016, in whom cyst fluid analysis showed bacteria or neutrophils indicating definite cyst infection, were reviewed retrospectively. Factors influencing the duration of hospitalization and death due to cyst infection were analyzed., Results: A total of 243 patients with cyst infection were enrolled, including 104 men and 139 women (mean age: 62.6 ± 10.0 years). Seventeen patients died of cyst infection. Multivariate logistic regression analysis revealed that hepatomegaly, positive cyst contents culture, and the maximum white blood cell (WBC) count were significantly associated with death from cyst infection. Death was significantly more likely if patients had massive hepatomegaly (≥5,113.2 mL) compared with mild hepatomegaly (< 1,898.7 mL; odds ratio 38.1; 95% CI 2.9-492.6, p = 0.0004). Multivariate Cox proportional hazards analysis revealed that older age, dialysis duration, hepatic transcatheter arterial embolization, maximum WBC count, and hepatomegaly were significantly associated with longer hospital stay for cyst infection, while female sex, nephrectomy, serum albumin, and serum cholinesterase were significantly associated with shorter hospital stay. Discharge alive from hospital was significantly associated with massive hepatomegaly versus mild hepatomegaly (hazard ratio 0.0072; 95% CI 0.0008-0.0659, p < 0.0001)., Conclusions: Hepatomegaly was strongly associated with both death and longer hospital stay due to cyst infection., (© 2018 S. Karger AG, Basel.)

Background: A non-thrombotic pulmonary embolism is defined as embolization to the pulmonary circulation. It may be caused by microorganisms, foreign bodies, different cell types or gas in the pulmonary circulation. Pulmonary hydatid cyst-induced embolization is a rare complication of heart or liver hydatid cysts., Case Report: We describe the fatal case of a 15-year-old boy without any known prior illness who was admitted to the hospital after feeling unwell and dropping to the ground while playing ball. During the autopsy, a lesional mass, with dimensions of 13x6 cm, was observed in the left lobe of the liver. The histomorphological examination of pulmonary sections showed scolices observed in pulmonary vessel lumina, thus a non-thrombosis hydatid embolism was diagnosed. Based on the findings, the cause of death was recorded as a non-thrombotic hydatid embolism., Conclusion: The present case is interesting because a non-thrombotic pulmonary embolism rarely results in sudden death, and a definitive diagnosis was possible only by a histopathological examination.

Background: Partial hepatectomy and cyst fenestration (PHCF) selectively provides clinical benefit in highly symptomatic patients with polycystic liver disease (PLD). This study aims to ascertain whether the reduction in liver volume (LV) achieved by PHCF is sustained long term., Study Design: Clinical data were retrieved from the electronic records of all patients with PLD who underwent PHCF between 1985 and 2014. Preoperative LVs (LV1), postoperative LVs (LV2), and late follow-up LVs (LV3) were measured from magnetic resonance or CT images., Results: Among 186 patients who underwent PHCF, 91% were Caucasian women with autosomal dominant polycystic kidney disease with a mean age of 49 years. Major perioperative complications (Clavien III/IV) occurred in 21% of the patients. Operative mortality (<90 days) was 2.7%. Eleven patients had liver failure develop, received liver transplants, or had liver-related deaths. Overall survival was 95.7%, 93.3%, 85.6%, and 77.7% at 1, 5, 10, and 15 years respectively. Imaging records for volumetry were unavailable in 32 patients. Of the remaining 154 patients, 34 had imaging for 1 LV, 64 for 2 LVs, and 55 for all 3 LVs. Median LV was 6,781 mL (interquartile range 4,903 to 8,341 mL) preoperatively and 2,502 mL (interquartile range 2,089 to 3,136 mL) after PHCF, leading to a median postoperative LV reduction of 61%. At follow-up (mean 8 years), median LV was 2,519 mL (interquartile range 2,083 to 3,752 mL). Interestingly, 33 of 62 patients with available LV2 and LV3 showed additional regression in LV at follow-up (median -14.1%), and the rest showed mild growth of 9.9%. Overall volumetric comparison of preoperative with follow-up liver imaging showed sustained LV reduction (median 61%)., Conclusions: Sustained long-term reductions in LV after PHCF can be achieved in selected patients with severe, highly symptomatic PLD. In our experience, liver-related death and subsequent liver transplantation are infrequent after PHCF., (Copyright © 2016 American College of Surgeons. Published by Elsevier Inc. All rights reserved.)

Objective: To evaluate bronchopulmonary dysplasia (BPD), serious brain injury, and severe retinopathy of prematurity (ROP) as predictors of poor long-term outcome in very low birth weight infants., Study Design: We examined the associations between counts of the 3 morbidities and long-term outcomes in 1514 of 1791 (85%) infants with birth weights of 500-1250 g who were enrolled in the Caffeine for Apnea of Prematurity trial from October 1999, to October 2004, had complete morbidity data, and were alive at 36 weeks postmenstrual age (PMA). BPD was defined as use of supplemental oxygen at 36 weeks PMA. Serious brain injury on cranial ultrasound included grade 3 and 4 hemorrhage, cystic periventricular leucomalacia, porencephalic cysts, or ventriculomegaly of any cause. Poor long-term outcome was death after 36 weeks PMA or survival to 5 years with 1 or more of the following disabilities: motor impairment, cognitive impairment, behavior problems, poor general health, deafness, and blindness., Results: BPD, serious brain injury, and severe ROP occurred in 43%, 13%, and 6% of the infants, respectively. Each of the 3 morbidities was similarly and independently correlated with poor 5-year outcome. Rates of death or disability (95% CI) in children with none, any 1, any 2, and all 3 morbidities were 11.2% (9.0%-13.7%), 22.9% (19.6%-26.5%), 43.9% (35.5%-52.6%), and 61.5% (40.6%-79.8%), respectively., Conclusions: In very low birth weight infants who survive to 36 weeks PMA, a count of BPD, serious brain injury, and severe ROP predicts the risk of a late death or survival with disability at 5 years., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Background: Transcatheter arterial embolization (TAE) has become a therapeutic option for symptomatic polycystic kidney disease (PKD) and polycystic liver disease (PLD). However, factors affecting survival with renal TAE remain unknown., Methods: All symptomatic patients with severe PKD and/or PLD who received renal and/or hepatic TAE at our center from October 1996 through March 2013 (n = 1,028) were followed until death. Their survival was compared with that of the general PKD population on dialysis in Japan. Factors affecting survival were analyzed using the Cox hazard model., Results: After renal TAE, 5- and 10-year survival was, respectively, 0.78 (95% confidence interval, 0.74-0.82) and 0.56 (0.49-0.63); with hepatic TAE, 0.69 (0.58-0.77) and 0.41 (0.22-0.60); and with dual TAE (renal and hepatic), 0.82 (0.72-0.88) and 0.45 (0.31-0.59). Survival after dialysis initiation was better among patients with renal TAE than among general PKD patients. Factors affecting survival after renal TAE were age [hazard ratio (HR) 3.02 (1.44-6.33) for every 10 years] and albumin [HR 0.70 (0.55-0.89) per 0.1 g/dl]. Kidney volume was not associated with patient death after TAE. The main causes of death among patients after renal TAE were similar to those of the general PKD population on dialysis whereas, after hepatic TAE, the main cause was cyst infection with liver failure (12.5% with PLD and 5.9% with PKD, p < 0.01)., Conclusion: Survival after renal TAE with severe PKD was better than for the general PKD population on dialysis, suggesting that renal TAE could overcome the disadvantage due to huge organ size.

Background: Polycystic liver disease (PLD) may lead to massive hepatomegaly, abdominal distension, pain, and various degrees of dyspnea. The surgical treatment of this entity remains controversial., Methods: We report our experience from a retrospective analysis of 23 patients suffering from PLD who were treated with liver transplantation (LT) in our institution., Results: Liver transplantation for PLD patients with extensive hepatic involvement offers excellent symptoms relief. The actuarial 1-, 3-, and 5-year survival rate after transplantation was 86%., Conclusions: Our experience demonstrates that PLD patients with extensive hepatic involvement and who are treated with LT have good long-term prognosis and excellent symptoms relief. LT might be considered in severe PLD cases where conventional surgery is not a curative option, and it must be balanced against the risks of LT and lifelong commitment to immunosuppression.

Objectives: To characterize pathological and cancer-specific outcomes of surgically resected cystic renal tumors and to identify clinical or radiographic features associated with these outcomes., Methods and Materials: All patients at our institution who underwent radical or partial nephrectomy for complex renal cystic masses between 2004 and 2011 with available computed tomographic imaging were included. The Bosniak score was determined, as were 10 specific radiographic characteristics of renal cysts in patients with preoperative imaging available for review. These characteristics were correlated with cystic mass histopathology. Recurrence-free survival after surgery was determined., Results: Overall, 133 patients underwent renal surgery for complex cystic lesions, 89 (67%) of whom had malignant lesions. Malignancy risk increased with Bosniak score (P≤0.01) and presence of mural nodules (P = 0.01). Most (63%) malignancies demonstrated clear cell histology. The papillary renal cell carcinomas (25%) exhibited lower enhancement levels (P = 0.04) and were less often septated (P<0.01). Of the malignancies, 79% were low stage (pT1), and 73% were Fuhrman grade 1 or 2. Large cyst size was associated with advanced tumor stage (P = 0.05). Neither Bosniak score nor any other radiographic parameter was associated with Fuhrman grade. In 70 patients with a median follow-up of 43 months, only 1 (1.4%) developed disease recurrence., Conclusions: Most cystic renal malignancies are low-stage, low-grade lesions. Papillary renal cell carcinomas account for nearly a quarter of cystic renal malignancies and have unique radiographic characteristics. Disease recurrence after surgical resection is rare. These findings suggest an indolent behavior for cystic renal tumors, and these lesions may be amenable to active surveillance., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Background: Although it is the only curative treatment for polycystic liver disease (PLD), orthotopic liver transplantation (OLT) has been reserved for severely symptomatic, malnourished, or refractory patients who are not candidates for palliative disease-directed interventions (DDI). Data on the effect of previous DDIs on post-transplant morbidity and mortality are scarce. We analyzed the outcomes after OLT for PLD recipients, and determined the effects of previous palliative surgical intervention on post-transplantation morbidity and mortality., Study Design: We performed a retrospective analysis of factors affecting perioperative outcomes after OLT for PLD between 1992 and 2013, including comparisons of recipients with previous major open DDIs (Open DDI, n = 12) with recipients with minimally invasive or no previous DDIs (minimal DDI, n = 16)., Results: Over the 20-year period, 28 recipients underwent OLT for PLD, with overall 30-day, 1-, and 5-year graft and patient survivals of 96%, 89%, 75%, and 96%, 93%, 79%, respectively. Compared with the minimal DDI group, open DDI recipients accounted for all 5 deaths, had inferior 90-day and 1- and 5-year survivals (83%, 83%, and 48% vs 100%, 100%, 100%; p = 0.009), and greater intraoperative (42% vs 0%; p = 0.003), total (58% vs 19%; p = 0.031), and Clavien grade IV or greater (50% vs 6%; p = 0.007) postoperative complications, more unplanned reoperations (50% vs 13%; p = 0.003), and longer total hospital (27 days vs 17 days; p = 0.035) and ICU (10 days vs 4 days; p = 0.045) stays., Conclusions: In one of the largest single-institution experiences of OLT for PLD, we report excellent long-term graft and patient survival. Previous open DDIs are associated with increased risks of perioperative morbidity and mortality. Improved identification of PLD patients bound for OLT may mitigate perioperative complications and potentially improve post-transplantation outcomes., (Copyright © 2014 American College of Surgeons. Published by Elsevier Inc. All rights reserved.)

Background: Hepatic transcatheter arterial embolization (TAE) has become an accepted treatment option for patients with symptomatic autosomal dominant polycystic kidney disease (ADPKD) who also have polycystic liver disease and who are not good candidates for surgery. However, indications for TAE and long-term outcome with it are still unclear., Study Design: Retrospective cohort study., Setting & Participants: Symptomatic patients with ADPKD with polycystic liver disease who underwent hepatic TAE, June 2001 to December 2012, at Toranomon Hospital and whose liver volume data were available were studied (N=244; 56% on dialysis therapy, none with kidney transplants). Mean age was 55 ± 9 (SD) years, and mean liver volumes were 8,353 ± 2,807 and 6,626 ± 2,485 cm(3) in men and women, respectively. Target arteries were embolized from the periphery using platinum microcoils., Predictors: Sex-specific quartiles (6,433, 8,142, and 9,574 cm(3) in men and 4,638, 6,078, and 8,181 cm(3) in women) of total liver volume pretreatment., Outcomes: All causes of mortality were obtained from medical records, followed up until July 31, 2013., Measurements: Laboratory values were measured before TAE and 1, 3, 6, and 12 months after. Organ volumes were measured pretreatment, then 6 and 12 months after, by summing the products of the organ areas traced in each computed tomographic image., Results: Liver/cyst volume decreased to 94.7% (95% CI, 93.5%-95.8%) at 6 months and 90.8% (95% CI, 88.7%-92.9%) at 12 months of pretreatment volumes. Serum protein and hematocrit values improved significantly without liver damage. Survival was significantly better for patients with liver volume ≤ 9,574 cm(3) (men) and ≤ 8,181 cm(3) (women) than for those with larger livers (5-year survival, 69% and 48%; P=0.02). Infection and liver failure caused most deaths, especially in patients with larger livers., Limitations: Referral bias and lack of control group., Conclusions: Hepatic TAE appears to be a safe and less invasive option for patients with symptomatic polycystic liver, especially those contraindicated for surgical treatment (eg, with malnutrition or on dialysis therapy), improving both hepatic volume and nutrition., (Copyright © 2014 National Kidney Foundation, Inc. Published by Elsevier Inc. All rights reserved.)

Background: The purpose of this study was to determine peri-operative mortality and long-term outcomes in patients undergoing liver transplantation in the US using the United Network for Organ Sharing (UNOS) database., Methods: This study is a retrospective review of liver transplantations (LT) recorded in the UNOS database performed between 1988 and 2010. In total, 107 411 LT were performed in the US, 357 (0.3%) were for adult polycystic liver disease (PLD). A random group of 9416 adult patients transplanted for other diagnoses was created for comparison (10% of the adult non-PLD database)., Results: Two hundred and seventy-one patients in the adult PLD group were females (75.9%), the mean age was 52.3 ± 8.2 [standard deviation (SD)] years. The median length of transplantation hospital stay was 11 days (interquartile range 8-21). Patients from the PLD group versus the comparison group (9416 patients) consisted of more females, lower Model for End-Stage Liver Disease (MELD) scores (17 versus 21 points), more multi-organ transplants (41% versus 4 %), chronic renal failure (creatinine 2.7 versus 1.5) and fewer patients with chronic hepatitis C (1.4% versus 32%). Peri-operative mortality (≤30 days) was 9% in the PLD versus 6% in the comparison group; however, at 1 year PLD survival was similar (85% versus 85%) to other diagnoses and better at 3 (81% versus 77%) and 5 years (77% versus 71%, overall Log Rank P = 0.006). A similar PLD survival advantage was observed in isolated initial transplants (P = 0.019)., Conclusion: In spite of early technical challenges and mortality, transplantation should be considered an option for selected patients with PLD as excellent long-term outcomes can be achieved., (© 2012 International Hepato-Pancreato-Biliary Association.)

Purpose: To assess the role of Gamma Knife radiosurgery (GKRS) in the treatment of nonsurgical cystic brain metastasis, and to determine predictors of response to GKRS., Methods: We reviewed a prospectively maintained database of brain metastases patients treated at our institution between 2006 and 2010. All lesions with a cystic component were identified, and volumetric analysis was done to measure percentage of cystic volume on day of treatment and consecutive follow-up MRI scans. Clinical, radiologic, and dosimetry parameters were reviewed to establish the overall response of cystic metastases to GKRS as well as identify potential predictive factors of response., Results: A total of 111 lesions in 73 patients were analyzed; 57% of lesions received prior whole-brain radiation therapy (WBRT). Lung carcinoma was the primary cancer in 51% of patients, 10% breast, 10% colorectal, 4% melanoma, and 26% other. Fifty-seven percent of the patients were recursive partitioning analysis class 1, the remainder class 2. Mean target volume was 3.3 mL (range, 0.1-23 mL). Median prescription dose was 21 Gy (range, 15-24 Gy). Local control rates were 91%, 63%, and 37% at 6, 12, and 18 months, respectively. Local control was improved in lung primary and worse in patients with prior WBRT (univariate). Only lung primary predicted local control in multivariate analysis, whereas age and tumor volume did not. Lesions with a large cystic component did not show a poorer response compared with those with a small cystic component., Conclusions: This study supports the use of GKRS in the management of nonsurgical cystic metastases, despite a traditionally perceived poorer response. Our local control rates are comparable to a matched cohort of noncystic brain metastases, and therefore the presence of a large cystic component should not deter the use of GKRS. Predictors of response included tumor subtype. Prior WBRT decreased effectiveness of SRS for local control rates., (Published by Elsevier Inc.)

Purpose: To retrospectively evaluate the effectiveness of fractionated stereotactic radiotherapy (FSRT) for craniopharyngioma., Methods and Materials: Between 1999 and 2005, 16 patients with craniopharyngioma were referred to Tokyo Medical University Hospital. They received FSRT alone after histologic confirmation by needle biopsy and underwent cyst drainage via endoscopy. The median prescription dose fraction was 30 Gy in six fractions. All patients except 1 were followed up until December 2009 or death., Results: The median follow-up period was 52 months (range, 4-117 months). Of the 17 patients, 3 experienced recurrence 4 to 71 months after FSRT. The 3-year local control rate was 82.4%. One patient died of thyroid cancer, and the 3-year survival rate was 94.1%. Eight patients had improved visual fields at a median of 2.5 months after FSRT, but hormonal functions did not improve in any patient., Conclusions: FSRT after cyst drainage seems to be safe and effective for patients with craniopharyngiomas, and it may be a safe alternative to surgery., (Copyright © 2012 Elsevier Inc. All rights reserved.)

Object: The presence of cystic features in glioblastoma (GBM) has been described as a favorable prognostic factor. The aim of this study was to determine the survival outcome in patients undergoing surgery for newly diagnosed primary GBM with a large cystic component as compared with a large cohort of patients with noncystic GBM, while controlling for well-characterized prognostic factors., Methods: A retrospective review of 354 consecutive patients treated with resection of primary GBM was performed using medical records and imaging information obtained at the University of California, San Francisco from 2005 to 2009. Within this cohort, 37 patients with large cysts (≥ 50% of tumor) were identified. Clinical presentations and surgical outcomes were statistically compared between the cystic and noncystic patients., Results: There were no statistically significant differences in clinical presentation between groups, including differences in age, sex, presenting symptoms, tumor location, or preoperative functional status, with the exception of tumor size, which was marginally larger in the cystic group. Surgical outcomes, including extent of resection and postoperative functional status, were equivalent. The median actuarial survival for the patients with cystic GBM was 17.0 months (95% CI 12.6-21.3 months), and the median survival for patients with noncystic GBM was 15.9 months (95% CI 14.6-17.2 months). There was no significant between-groups difference in survival (p = 0.99, log-rank test). A Cox multivariate regression model was constructed, which identified only age and extent of resection as independent predictors of survival. The presence of a cyst was not a statistically significant prognostic factor., Conclusions: This study, comprising the largest series of cases of primary cystic GBM reported in the literature to date, demonstrates that the presence of a large cyst in patients with GBM does not significantly affect overall survival as compared with survival in patients without a cyst. Preoperative discussions with patients with GBM should focus on validated prognostic factors. The presence of cystic features does not confer a survival advantage.

Background and Aim: Isolated polycystic liver disease (PCLD) is characterized by the presence of multiple cysts in the liver in the absence of polycystic kidneys. The clinical profile of PCLD is poorly defined and we set up a study for the clinical characteristics of PCLD., Methods: We collected clinical data on 188 PCLD patients (defined as >10 liver cysts) from five tertiary referral centres, and 137 patients were selected for the purpose of this study. We performed molecular analysis of the PCLD associated genes PRKCSH and SEC63 in 91 patients., Results: A total of 118 (86%) patients were female. The majority of patients (88%) had >20 cysts. The median age at diagnosis was 47 years (range 23-84). 37 (41%) patients carried a mutation. Clinical symptoms at presentation were present in 111 (84%) patients. γ-glutamyl transferase was elevated to 1.4 times upper limit of normal (interquartile range 1.0-2.7). The presence of a mutation and female gender predicted a more severe course: female patients were 9 years younger at the time of diagnosis (47 years; range 23-84) and 91% had symptoms (P<0.01); likewise, mutation carriers were younger at presentation (39 years; range 35-48) and 95% of this cohort had symptoms (P<0.01). During follow-up [median 8.2 years (range 0-35)], 10% of untreated and 51% of treated patients developed complications. Mortality in this cohort was 8%, but only 2% died of PCLD-related causes. 58% of patients were treated a median of 2 years (range 0-25) after diagnosis., Conclusion: Symptomatic PCLD patients are mainly females. Females and mutation carriers were younger at diagnosis and had a more severe course of disease., (© 2010 John Wiley & Sons A/S.)

Polycystic liver disease (PLD) is a celiopathy characterized by progressive growth of multiple hepatic cysts. In a minority of patients, severe symptomatic hepatomegaly necessitates liver transplantation (LT). The purpose of this study is to describe the postoperative and long-term outcomes of all patients transplanted for PLD at our center. All patients who underwent LT for PLD were identified through our database. Using patient charts, data were extracted on patient demographics and medical history, postoperative surgical and medical complications, length of hospitalization, prevalence of chronic kidney failure, and patient and graft survival. Subjects were contacted in April 2010 to verify their survival and confirm their need, if any, for hemodialysis and/or kidney transplantation. Descriptive statistics for patient and graft survival were performed. From 1993 to 2010, 14 subjects underwent LT and 1 subject underwent combined kidney and LT; all subjects were female and the mean age was 49.0 years. 10 (66.7%) subjects had polycystic kidney disease. Patients experienced a high rate of vascular complications, including hepatic artery thrombosis (HAT) or stenosis in 3 (20%) and 2 (13.3%) subjects, respectively. One subject had early graft loss due to HAT and underwent re-transplantation. The mean length of hospitalization was 18.8 days. After a mean of 66.8 months of follow-up (3-200), 13 (86.7%) subjects are alive with satisfactory graft function, and no patients had renal failure. In conclusion, patients who underwent LT for PLD had a high rate of postoperative vascular complications. However, long-term patient and graft survival, and kidney function, is excellent.

Purpose: Ciliated Hepatic Foregut Cyst (CHFC) is a rare congenital lesion arising from the embryonic foregut. Since squamous cell carcinomas arising from CHFC have been reported in adults, complete resection should be considered. We report our experience with CHFC., Methods: We reviewed the charts of 2 patients who had surgery after prenatal detection of a CHFC and 2 patients with postnatal diagnosis., Results: Two patients had antenatally detected liver cyst. Postnatal ultrasonography showed a cyst in segment IV, with wall calcifications and sediments. Bile ducts were encased in the wall of the cyst. They underwent central hepatectomy with double biliary diversion and uneventful post operative course. The two other patients underwent non anatomical resection of a cyst on the left lobe and in segment IV, found prior or during liver surgery. Pathology examination showed cysts filled with mucinous fluid, surrounded by an epithelium composed of ciliated cells. One case had a squamous metaplasia., Conclusion: In infants, CHFC are found antenatally or incidentally. A solitary uni or mutilocular cyst with wall calcifications, sediments, located in the central liver segments should raise the diagnosis. Resection of large cysts in the central segments of the liver is challenging and biliary diversion should be considered.

Introduction: The differential diagnosis of an abdominal cyst can be challenging, and an accurate diagnosis is crucial for optimal antenatal management. The aim of this study was to compare the ante- and postnatal diagnoses of cases with abdominal cyst and to determine the diagnostic accuracy of ultrasonography., Material and Methods: A database review was performed regarding the diagnosis of fetal abdominal cyst covering the period 2002-2009. Structural characteristics and localizations of the cysts in the abdomen were recorded. Ante- and postnatal diagnoses were classified into systems according to the origin of the cyst and were compared. Perinatal outcomes were obtained for all cases., Results: 71 cases with an abdominal cyst were identified. The mean gestational age at the time of diagnosis was 25 ± 5.1 weeks. In 9 cases, there were extra-fetal structural abnormalities, and in 5 cases a chromosomal abnormality was determined. Seven pregnancies were terminated. Overall spontaneous mortality was 11/64 (17%). In 12/64 cases (18%), the cyst resolved at birth. After birth, nearly half of the cases required surgical correction and of these, 20% died. Sensitivity, specificity and positive predictive value of ultrasonography in identifying the system of origin were 88.1, 95.7 and 92.0%, respectively, with a 4.1% false-positive rate., Conclusion: Incorporation of different disciplines in the counseling, management and postpartum follow-up is crucial. Postnatal physical examination of fetuses with an abdominal cyst will help to prevent unnecessary surgery., (Copyright © 2010 S. Karger AG, Basel.)

Introduction: The study of the clinical, histological and immunohistochemical aspects of three kidney tumors corresponding to synovial sarcomas operated on in our center over three years., Patients and Method: Three patients aged between 27 and 33 had an enlarged nephrectomy for kidney tumors corresponding to a histological examination of a synovial sarcoma. The tumors were symptomatic in 100% of cases with back pain and spontaneous rupture. Size varied from 5 to 13cm. The radiological aspect was a cystic tumor (BosniakIV) in two cases and in the other a spontaneous perirenal hematoma. Two were in the right kidney and one in the left kidney. The parts were analyzed after fixation. Immunohistochemical coloration and an analysis in molecular biology by RT-PCR of fusion transcripts were carried out., Results: One patient died because of local development and metastasis 24 months after an enlarged nephrectomy associated with radio chemotherapy (Maidprotocol). Two patients were in total remission after an average of 25 months following the same treatment. The histology found a mesenchymal fusocellular monotone proliferation corresponding to a sarcoma. In all three cases, it was a biphasic form with plaques of fusiform cells and epithelial cells. The immunohistochemical study shows a positivity of the contingent epithelial and fusiform. The muscular markers were negative. A study in molecular biology of the fusion transcript allowed for the finding of a translocation (X;18) in all three cases., Conclusion: Primitive synovial sarcoma of the kidney occured in young patients. The translocation (X;18) is pathognomonic of the diagnosis. The prognosis is bleak despite complete excision, radiotherapy and chemotherapy.

In the current Model for End-Stage Liver Disease system, patients with polycystic liver disease (PCLD) who have a poor quality of life secondary to their massive hepatomegaly are no longer competitive for a deceased donor liver transplant if their liver function is well preserved. Traditionally, a caval resection has been advocated in these patients because of the difficulty of the hepatectomy with hepatomegaly, which makes living donation impossible. This series looks at 3 patients who underwent a caval sparing hepatectomy and subsequent living donor liver transplantation (LDLT) for PCLD. Graft and patient survival was 100%, and there were few complications in either donors or recipients. LDLT is an ideal option for patients with PCLD and preserved liver function but poor quality of life.

Objective: Polycystic liver disease (PLD) is a rare, hereditary, benign disorder. Hepatic failure is uncommon and symptoms are caused by mass effects leading to abdominal distension and pain. Liver transplantation (LTX) offers fully curative treatment, but there is still some controversy about whether it is a relevant modality considering the absence of liver failure, relative organ shortage, perioperative risks and lifelong immunosuppression. The purpose of this study was to review our experience of LTX for PLD and to compare the survival with the overall survival of patients who underwent LTX from 1992 to 2005., Material and Methods: A retrospective study of the journals of 440 patients, who underwent 506 LTXs between 1992 and 2005, showed that 14 patients underwent LTX for PLD. All patients had normal liver function. Three were receiving haemodialysis and thus underwent combined liver/kidney transplantation. One patient had undergone kidney transplantation 10 years earlier., Results: Median follow-up was 55 months. One patient who underwent combined transplantation died after 5.4 months because of multiorgan failure after re-LTX, and one patient, with well-functioning grafts, died of lymphoma after 7 months. At present 12 patients are alive, relieved of symptoms and with good graft function., Conclusions: We conclude that patients treated for PLD by LTX have a good long-term prognosis and excellent relief of symptoms and that LTX might be considered in severe cases of PLD, where conventional surgery is not an option.

Introduction: Cystic disease of biliary tract (CDBT) characterizes by the presence of sacular expansions of the biliary tree. It is an uncommon disease associated with high morbidity and malignant transformation. More than 60% of patients are women and can be diagnosed in the adult life., Objective: To evaluate the results obtained during last three decades in the management of CDBT in the adult patient., Patients and Methods: All the patients with CDBT treated from 1970 to 2002 were included. Demographic data, clinical picture, boarding diagnosis, classification, treatment, evolution and survival were analyzed., Results: 34 patients. Twenty eight (82%) women and 6 (18%) men with a mean age of 33 years (range 13-84). The most frequent symptoms were abdominal pain, nausea-vomit and jaundice. Cholangiography was made in all cases. All the types described by Todani were documented. Twenty-seven patients (80%) were surgically treated. The mean follow-up was 84 months (range 1-408 months). Fifteen patients (44.1%) were readmitted and 9 (26.4%) had a reoperation. Three (9%) died with malignant transformation. The global survival was 91.1% to 12 months., Conclusions: In the adult patient, diagnosis of CDBT requires a high level of suspicion and its confirmation depends on the image studies. The CDBT diagnosis considers an indication of surgical treatment. Complete resection of the biliary tract with Roux en-Y hepato-jejunal anastomosis have less rate of mechanical complications, hospitalary readmissions and surgical reintervention.

Aim: To evaluate the immediate and long-term results in a series of patients with highly symptomatic polycystic liver disease (PLD) treated by combined hepatic resection with cystic fenestration., Methods: We reviewed our recent experience with a combined hepatic resection-fenestration procedure in seven highly symptomatic patients with PLD. Clinical data, liver manifestation of computed tomography (CT), and morbidity were recorded pre- and post-operation. Follow-up was made by clinical and CT examinations in all patients., Results: Symptomatic relief and reduction in abdominal girth were obtained in all patients during an average follow-up period of 20.4 mo. CT scans confirmed post-resection hypertrophy of the spared liver and lack of significant cyst progression. All patients had mild to severe ascites. Two patients were complicated with pleural effusion., Conclusion: Some highly symptomatic patients with massive PLD may benefit from combined hepatic resection and fenestration at acceptable risk. To stitch the dissected hepatic ligaments could prevent the instable remnant liver from kinking and collapsing., (Copyright 2004 The WJG Press ISSN)

Objective: The efficacy of stereotactic intracavitary irradiation with phosphorus-32 ((32)P) for patients with cystic craniopharyngiomas was assessed on the basis of patient survival, tumor control, and visual and endocrinological function before and after treatment. Limited data are available regarding long-term outcomes., Methods: Forty-nine patients were treated with stereotactic (32)P intracavitary irradiation. Of these, 25 had had no prior treatment as the primary treatment, and 24 were treated for residual or recurrent tumor cysts. At the time of (32)P intracavitary irradiation, 34 of the patients were adults, and 15 were children younger than 16 years of age. The mean cyst volume was 13 ml. The radiation dose varied from 189 to 250 Gy to the cyst wall during five half-lives of the isotope (mean, 224 Gy). The mean follow-up periods were 7 years after diagnosis and 4 years after (32)P treatment., Results: The actuarial survival rates were 90% at 5 years after the diagnosis and 80% at 10 years. The actuarial tumor cyst control rates were 76% at 5 years and 70% at 10 years after the diagnosis. After treatment, 9 (23%) of 40 patients who underwent preoperative and postoperative visual testing were found to have delayed worsening in visual function, 6 as a result of tumor progression and 3 attributed to irradiation. Nineteen patients (48%) had improved visual function. Of 17 patients who had normal preoperative pituitary function or stalk effect, 12 (71%) had preserved and 5 (29%) had worsened visual function. No complications other than visual or endocrinological deterioration occurred in these patients., Conclusion: For patients with cystic craniopharyngiomas, (32)P intracavitary irradiation proved effective, with a low risk of complications, for the control of tumor cysts but not of solid tumor components.

Purpose: To evaluate thin-section computed tomographic (CT) abnormalities in patients in the intensive care unit during the late stage of adult respiratory distress syndrome (ARDS) caused by severe acute respiratory syndrome (SARS)., Materials and Methods: Eight patients in the late stage of ARDS (ie, more than 2 weeks after onset) were imaged with thin-section CT. Images were evaluated for ground-glass opacification, consolidation, interstitial thickening, evidence of fibrosis, and any other abnormalities. Patient records were reviewed, and relevant respiratory and ventilatory parameters, total steroid dose, and outcome were recorded., Results: All patients received high-dose pulse methylprednisolone (minimum, 2.5 g total), and all patients who received ventilation received low-pressure, low-volume ventilation. Five patients received prolonged mechanical ventilation (for more than 14 days), one received ventilation for 72 hours, and two patients did not receive ventilation. Three patients died, four were discharged from the hospital, and one continued to require ventilation. Ground-glass opacification and interstitial thickening were present at CT in all eight patients. Consolidation was present in six patients. Three patients had evidence of fibrosis. Patients who received long-term ventilation, those who received short-term ventilation, and those who did not receive ventilation had similar pulmonary changes at CT. Pulmonary cysts, most of which were small (<1 cm), were present in five patients. Cysts were present in one patient who received only short-term low-pressure and low-volume ventilation and in one patient who received no mechanical ventilation., Conclusion: The CT features of late-stage ARDS caused by SARS are similar to those seen in late-stage ARDS of other causes, with no apparent differences between patients who do and patients who do not receive prolonged mechanical ventilation. The presence of cysts in one patient who received short-term and one patient who received no mechanical ventilation suggests that severe SARS-induced ARDS may independently result in cyst formation., (Copyright RSNA, 2004)

Background: Congenital high airway obstruction syndrome (CHAOS) is a life-threatening condition with a poorly understood natural history., Methods: A retrospective review of five patients with CHAOS between 1997 and 2002 was performed., Results: All fetuses had large echogenic lungs, dilated airways, inverted diaphragms, and massive ascites. One fetus with a laryngeal cyst was terminated at 22 weeks. A twin fetus with findings suggestive of a tracheal web had progressive hydrops, which led to fetal demise. The remaining 3 patients delivered via the ex utero intrapartum treatment (EXIT) procedure survived. The first patient tolerated progressive hydrops for 12 weeks in utero. He had tracheal atresia but underwent laryngotracheoplasty successfully. He is the first long-term CHAOS survivor and is speaking at 5 years of age. The 2 patients with relatively stable lung volumes prenatally have laryngeal atresia with a pinpoint posterior laryngeal fistula. Their postnatal clinical courses were much more benign than the first survivor., Conclusions: The prenatal natural history and postnatal course of CHAOS depends on whether the airway obstruction is complete. The EXIT procedure offers the potential for salvage of this otherwise lethal condition. Hydrops may be well tolerated prenatally for weeks with potential resolution if airway fistulization is present.

Autosomal dominant polycystic kidney disease (ADPKD) accounts for 8% to 10% of patients with end-stage renal disease (ESRD) in the United States and Europe. Progressive expansion of multiple bilateral renal cysts leads to massive enlargement of the kidneys and progressive renal failure. Extrarenal manifestations of ADPKD, such as liver cysts, intracranial aneurysms, cardiac valvular disease, and perhaps diverticulosis, have been documented extensively in cross-sectional studies, but little is known about their natural history. It is thought that extrarenal aspects of ADPKD contribute to increased mortality, yet survival on dialysis of the ADPKD population surpasses that of the general dialysis population. To address this issue, we analyzed the relative risk and causes of death after ESRD in ADPKD versus nondiabetic controls using data from the United States Renal Data System. Relative risk of death from any cause, including the major extrarenal manifestations of ADPKD, was determined as a function of ESRD treatment modality (dialysis or transplantation). We found a lower total mortality rate in ADPKD ESRD patients compared with nondiabetic control ESRD patients (relative risk of death in ADPKD = 0.57; P < 0.001). Mortality rates of extrarenal complications except for polycystic liver disease were similar or lower in ADPKD patients than in nondiabetic controls. Mortality secondary to extrarenal complications was substantially lower than that secondary to cardiovascular or cerebrovascular disease.

This paper is a retrospective review of all patients treated for a colloid cyst of the third ventricle at Royal Prince Alfred Hospital over an 18-year consecutive period. Our experience is fairly typical compared with other published series. Colloid cys ts made up 1.6% of tumours treated by the neurosurgical unit. Patients presented with non-specific neurological symptoms and signs, commonly suggestive of raised intracranial pressure. Microsurgical excision was carried out via the transcallosal route in 34 cases and the transfrontal approach in four cases. There was no apparent difference in results from the different surgical approaches. In 86% of patients reviewed after more than 6 months good long term outcome was achieved. These benign tumours can be safely cured if the diagnosis is made early and the cyst removed by careful microsurgical techniques., (Copyright 2001 Harcourt Publishers Ltd.)

To clarify the relationship between clinical features in utero and postnatal prognosis, 20 fetuses who underwent ultrasonic (US) evaluation for meconium peritonitis (MP) over a 17-year period were reviewed. According to final US findings in utero, patients were classified into three types. Type I (massive meconium ascites) was noted is 5 cases, type II (giant pseudocyst) in 4, and the other 11 were classified as type III (calcification and/or small pseudocyst). Abdominal calcifications were identified in only 5 cases (2 type I, 1 type II, 2 type III). Seven fetuses who had associated polyhydramnios (1 Type I, 1 Type II, 2 Type III) and fetal hydrops (3 Type II) were delivered before 36 weeks' gestation. Cardiopulmonary resuscitation at birth was required in 9 cases (5 type I, 4 type II) who underwent abdominal drainage before delivery and/or immediately after birth. Although dilatation of the intestine was identified in 10 fetuses (2 type II, 8 Type III), 18 had intestinal atresia and 2 had fecal obstruction of the distal ileum. Four infants (2 type I, 1 type II, 1 type III) died of respiratory failure and postoperative complications. These results indicated that careful fetal US may be useful for perinatal management of MP.

In patients suffering from polycystic liver disease sclerosing therapy, decompressive hepatic resection and liver transplantation represent the main therapeutic options. Since 1987, 10 females with highly symptomatic polycystic liver disease underwent hepatic resection for decompression (five left lateral bisegmentectomies, one left hemihepatectomy, one right central and three bilateral atypical resections). Despite of a 0% lethality 3 patients developed complications, in two cases an abscess had to be drained and in one case a postoperatively increasing hepatic insufficiency required urgent liver transplantation. Patient's symptoms were remarkably improved in 8 cases, but only 6 patients had a long lasting benefit over years. One patient was resected a second time three years after the first operation and is free of symptoms for another five years. 6/96 the "European Liver Transplant Registry" has listed 81 patients after liver transplantation because of polycystic liver disease. 5-year survival is 78.2%. So liver transplantation offers a therapeutic alternative for patients severely suffering from symptoms or increasing hepatic insufficiency.

Patients with tuberous sclerosis complex (TSC) are at increased risk of renal disease, predominantly angiomyolipomas and renal cysts. We retrospectively reviewed clinical data of 71 patients diagnosed with TSC. Progression of renal lesions was noted. TSC patients with renal lesions were compared with TSC patients without renal disease. Fifteen of 38 patients had renal abnormalities by imaging at presentation. Six of 9 with initially normal kidneys subsequently developed new lesions. Although not of statistical significance, there was a trend toward increased retinal hamartomas, cardiac rhabdomyomas, and skin lesions in those patients who also had renal abnormalities. Renal disease should be considered and sought in all patients with TSC, both at initial presentation and subsequently, since renal disease is a very significant cause of morbidity and mortality.

Polycystic liver disease (PLD) is an autosomal-dominant hereditary disease which usually presents together with polycystic kidney degeneration (ADPKD). The renal problems determine the course of this disease. Due to the development of dialysis an increasing number of patients present with symptoms from their liver cysts: These range from compression caused by hepatomegaly, which can severely limit the quality of life, to chronic liver failure. Ten patients with advanced symptoms of PLD underwent orthotopic liver transplantation, five of them with combined renal transplantation. Postoperative complications occurred in three patients. One patient died postoperatively from multiorgan failure after experiencing coagulopathy of unknown origin. After follow-up of 6-60 months, all patients had better quality of life after transplantation. There was a complete relief of symptoms; liver or renal failure did not occur. Liver transplantation should be considered in patients with highly symptomatic PLD. In the case of severe hepatomegaly or liver and renal failure the combined liver and renal transplantation are able to cure the PLD and ADPKD without rising the disadvantage of immunosuppression incurred with single organ transplantation.

Background: Occasionally patients with adult polycystic liver disease (APLD) have symptoms. For these patients surgery may represent a valuable therapeutic option to relieve symptoms., Methods: From September 1977 to August 1993 at our institution, 10 women with APLD were examined and surgically treated. They underwent a partial hepatic resection together with cyst fenestration. The surgical outcome and long-term follow-up were retrospectively analyzed., Results: Postoperative morbidity consisted of one case of pneumonia, and one case of acute pancreatitis with deep vein leg thrombosis. One patient died after acute Budd-Chiari syndrome developed as a result of liver collapse after fenestration of a posterior cyst. In the long term six of nine patients were symptom free. Late surgical complications included acute cholecystitis (one patient), small bowel obstruction (one), and incisional hernia (two)., Conclusions: A combined surgical approach of hepatic resection and cyst fenestration has proved feasible for patients with highly symptomatic APLD. Extensive fenestration of posterior cysts should be avoided; transverse hepatic resection (frontal hepatectomy) up to the costal margin is proposed. This therapy provides good results at long-term follow-up.

Symptomatic nonparasitic cysts of the liver require surgical intervention. Seventy-one patients were treated between 1977 and 1993 at the Department of General Surgery, University of Vienna. Different surgical treatments were compared with regard to complications and recurrence. Interventional puncture led to recurrences in nearly all cases and represents only a palliative procedure. Surgical treatment consisted of either laparoscopic (n = 7) or conventional (n = 44) fenestration or excision. The rates of recurrence did not differ significantly (14% vs 9%). The laparoscopic procedure is successful not only in polycystic disease but also in solitary cysts. Wide deroofing and excision were equally effective. Laparoscopic therapy should be tried in all cysts initially, because it causes less stress than celiotomy. Because of the small number of laparoscopically treated patients in the literature and in the authors' own experience, the significance of the difference in outcome between the two methods could not be established. In more patients, further investigations should be carried out to ascertain whether the laparoscopic method is superior regarding surgical stress and recurrence. Cystojejunostomy (n = 3) and hepatic resection (n = 2) are reserved for special indications. One homologous liver transplantation was successfully carried out 6 months after cystojejunostomy because of a cholangiocellular carcinoma. Frequent postoperative ascites represented a persistent problem in only one patient. Two of three cases of postoperative infection with intraabdominal abscesses led to death. Altogether, 16 patients died, including seven because of malignancy and three because of septic complications of a Potter III syndrome.

Objective: This study was designed to investigate the surgical outcome of translabyrinthine surgery of cystic acoustic neuromas., Design: Prospective registration of audiovestibular, otoneurologic, and neuroradiologic data of all patients operated on for cystic neuromas in the period 1976 to 1992. The diagnosis was proposed by neuroradiologic investigation, confirmed at surgery, and verified histologically. Clinical follow-up was performed 1 year after surgery., Setting: The study included all patients with cystic acoustic neuromas from the entire country, referred to the centralized otoneurosurgical team in the Department of Otorhinolaryngology-Head and Neck Surgery, Gentofte University Hospital, and Department of Neurosurgery, Rigshospitalet, University of Copenhagen, Denmark., Patients: The study included 23 patients (11 female and 12 male). Ages ranged between 23 and 77 years with a median of 51.2 years., Main Outcome: Our hypothesis suggested that surgical outcome of cystic acoustic neuromas was poorer compared with noncystic acoustic neuromas of matching size. The results of this study seem to confirm the hypothesis., Results: The results are rather poor compared with results achieved in surgery of noncystic tumors of matching size, particularly the poor postoperative facial nerve function., Conclusions: Even though the operation for a cystic acoustic neuroma may appear to be easier and faster than the operation for a solid tumor, there is a high risk for accidental lesion of the facial nerve, in spite of using facial nerve monitoring. Rapid symptomatic worsening may occur due to sudden expansion of cystic elements and, therefore, a wait-and-see policy should not be applied to patients with cystic tumors.

Objective: The purpose of this study was to report the authors' experience with hepatic resection for cystic lesions of the liver., Summary Background Data: Past experience with aspiration, sclerosing therapy, internal drainage, fenestration, and marsupialization are of limited value. Hepatic resection has evolved into a safe operation over the last two decades., Methods: A retrospective study of 44 patients with various cystic lesions of the liver (polycystic disease, 2; solitary or multiple congenital cysts, 19; biliary cystadenoma, 6; cystadenocarcinoma, 3; squamous cell carcinoma, 3; Caroli's disease, 5; and hydatid cyst, 6) was performed., Results: After 7 trisegmentectomies, 24 lobectomies, 6 left lateral segmentectomies, and 7 nonanatomical hepatic resections, only 1 operative death occurred in a Jehovah's Witness. Symptomatic relief was complete and permanent in all of the patients with benign congenital or parasitic hepatic cysts, except for the two patients with polycystic disease of the liver. One of the 3 patients with adenocarcinoma and 3 patients with squamous cell carcinoma of the cyst wall died of tumor recurrence between 3 and 14 months after hepatic resection., Conclusions: Hepatic resection is safe and effective for cystic lesions of the liver. Symptomatic relief is complete and permanent after hepatic resection, except in cases of diffuse polycystic disease of the liver. Liver transplantation should be considered for diffuse polycystic disease of the liver when the symptoms are extremely severe.

With the recent advances in imaging techniques, increased numbers of hepatic lesions are found today, and surgeons are asked frequently for the best course of management. Benign hepatic tumors sometimes cause life-threatening complications and more often trigger disabling or annoying symptoms in otherwise healthy individuals. Although various imaging techniques are quite accurate in identifying cysts and hemangiomas, other benign hepatic lesions, such as adenomas, focal nodular hyperplasia and other benign solid tumors, cannot be differentiated from malignant lesions with a high degree of confidence.

Tumor and tumor-like lesions of the thymus, excluding Hodgkin and nonHodgkin lymphomas, are among the least frequent mediastinal tumors in childhood. For this reason the diagnosis is often overlooked. The clinical and pathologic features of four thymus related masses, three of which were thymomas and one a thymic cyst, are reported in children between the ages of five and 15 years. The thymic cyst occurred in a child with a five year history of aplastic anemia. A rapid clinical course to death occurred in all four cases. The behavior of these lesions is compared with that described in the literature. The impression exists that thymomas in the pediatric age group are much more aggressive than those in adults. There are also some signifant differences in their histopathologic appearance.

Patients with continuous expansion of either giant lung cysts or areas of localized bullous emphysema will eventually deteriorate into a state of extreme respiratory distress due to compression of the remaining healthy pulmonary tissue. Thirty-one patients with these 2 types of lung disease having very poor respiratory function underwent surgery for the release of compromised healthy lung by resection of the expanding processes. A rational investigation program including respiratory tests, ventilation and perfusion scintigraphy and pulmonary angiography was gradually developed and analyzed. Surgical mortality was 12.9% in unilateral operations, probably somewhat higher in later contralateral interventions. The operative risk was higher in the emphysema group than in the group with giant lung cysts. Follow-up has demonstrated encouraging results in both groups concerning increased pulmonary function as well as improved working capacity.

Because previous work has suggested that NK cells may be important in host resistance against the intracellular parasite Toxoplasma gondii we examined whether human NK cells and lymphokine-activated killer (LAK) cells have activity against trophozoites and cysts of this organism in vitro. A method to radiolabel Toxoplasma trophozoites with 51Cr was developed and direct cytotoxic activity was determined by using modifications of the standard 51Cr release assay. Viability of 51Cr-labeled trophozoites assessed by both methylene blue staining and trypan blue exclusion was greater than 90%. Significantly more 51Cr was released by anti-Toxoplasma antibody and C than by antibody in the absence of C. Incubation of trophozoites with freshly isolated human NK cells or NK cells activated with either rIL-2 or rIFN-alpha did not result in significant release of 51Cr (specific lysis was 0 to 2.3%). In contrast, the average specific lysis of radiolabeled trophozoites by LAK cells was significant (specific lysis was 7.8% +/- 1.1, p less than 0.01). In a series of separate experiments, preincubation of radiolabeled trophozoites with heat-inactivated normal or Toxoplasma antibody-positive human serum increased the cytotoxicity of LAK cells from a mean specific lysis of 15% +/- 4.5 to 39% +/- 8.5, respectively (p less than 0.05), as assessed by 51Cr release. Because previous work has shown that radioisotope release from parasites may be nonspecific, separate experiments were performed to determine the cytotoxicity of LAK cells against antibody-coated trophozoites by using ethidium bromide-acridine orange staining to assess effector cell damage. LAK cells had a mean specific lysis of 51% against antibody-coated trophozoites by ethidium bromide-acridine orange staining. Preincubation with heat-inactivated Toxoplasma-antibody positive human serum did not increase activity of rIL-2-activated NK cells against 51CR-labeled trophozoites. Neither human NK cells (freshly isolated or activated by rIL-2 or rIFN-alpha) nor LAK cells were cytotoxic for purified preparations of cysts of Toxoplasma isolated from the brains of chronically infected mice.