Stereotactic cyst aspiration directly followed by Gamma Knife radiosurgery for large cystic brain metastases.

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.

Biliary Atresia-associated Cholangitis: The Central Role and Effective Management of Bile Lakes.

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.

Prediction of Late Death or Disability at Age 5 Years Using a Count of 3 Neonatal Morbidities in Very Low Birth Weight Infants.

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.

Intravascular embolization therapy in patients with enlarged polycystic liver.

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.

Peri-operative challenges and long-term outcomes in liver transplantation for polycystic liver disease.

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.

Comparison of survival between unilocular cystic and purely solid renal cell carcinoma.

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.

Patients with isolated polycystic liver disease referred to liver centres: clinical characterization of 137 cases.

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.

Clinical outcomes of liver transplantation for polycystic liver disease: a single center experience.

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.

Perinatal outcomes of fetal abdominal cysts and comparison of prenatal and postnatal diagnoses.

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.

[Primitive renal synovial sarcoma: a cystic tumor in young patients]. / Synovialosarcome primitif rénal: une tumeur kystique chez des patients jeunes.

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.

Factors Influencing Cyst Infection in Autosomal Dominant Polycystic Kidney Disease.

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.

Living donor liver transplantation in polycystic liver disease.

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.

Non-thrombotic Pulmonary Embolism Due to Liver Hydatic Cyst: A Case Report.

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.

Outcomes and Durability of Hepatic Reduction after Combined Partial Hepatectomy and Cyst Fenestration for Massive Polycystic Liver Disease.

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.

Survival after arterial embolization therapy in patients with polycystic kidney and liver disease.

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.

Survival after end-stage renal disease in autosomal dominant polycystic kidney disease: contribution of extrarenal complications to mortality.

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.

Thymus related tumors and tumor-like lesions in childhood with rapid clinical progression and death.

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.

Surgery for adult polycystic liver disease.

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.

Management of cystic craniopharyngiomas with phosphorus-32 intracavitary irradiation.

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.