Cystic endosalpingiosis of the appendix.
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Aged↗
Biomedical subjects
Cord Langner
Publications and source records attributed to Cord Langner.
Juvenile polyposis of the stomach causing recurrent upper gastrointestinal bleeding.
We present the case of a 42-year-old man who suffered from recurrent severe upper gastrointestinal bleeding starting from February 2003. Endoscopy showed multiple glassy polyps in the stomach, which corresponded to a diffuse mucosal thickening detected by endosonography. The duodenum was normal. In February 2006, life-threatening acute gastrointestinal bleeding prompted total gastrectomy. The resection specimen showed the gastric mucosa carpeted by numerous glassy pedunculated polyps, measuring 2 cm in largest diameter. Histologically, the polyps were characterized by an abundant loose stroma and by elongated, twisting foveolae, covered by hyperplastic epithelium. Colonoscopy including the terminal ileum revealed a single tubulovillous adenoma, but no hamartomatous polyps, rendering a final diagnosis of juvenile polyposis of the stomach. This case represents the first description of juvenile polyposis causing life-threatening gastric haemorrhage. Thus, although rare, the disease has to be considered in the differential diagnosis of patients with acute upper gastrointestinal tract bleeding.
Adenomatous Polyposis Coli↗
Klebsiella oxytoca as a causative organism of antibiotic-associated hemorrhagic colitis.
BACKGROUND: Antibiotic-associated hemorrhagic colitis is a distinct form of antibiotic-associated colitis in which Clostridium difficile is absent. Although the cause is not known, previous reports have suggested a role of Klebsiella oxytoca. METHODS: We studied 22 consecutive patients who had suspected antibiotic-associated colitis and who were negative for C. difficile. Patients underwent diagnostic colonoscopy, and among those who received a diagnosis of antibiotic-associated hemorrhagic colitis, stool samples were cultured for K. oxytoca. We isolated K. oxytoca strains and tested them for cytotoxin production using a tissue-culture assay. In addition, we also cultured stool samples obtained from 385 healthy subjects for K. oxytoca. An in vivo animal model for antibiotic-associated hemorrhagic colitis was established with the use of Sprague-Dawley rats. RESULTS: Of the 22 patients, 6 had findings on colonoscopy that were consistent with the diagnosis of antibiotic-associated hemorrhagic colitis, and 5 of these 6 patients had positive cultures for K. oxytoca. No other common enteric pathogens were found in the five patients. Before the onset of colitis, all five were receiving penicillins, and two were also taking nonsteroidal antiinflammatory drugs (NSAIDs). All isolated K. oxytoca strains produced cytotoxin. K. oxytoca was found in 1.6% of the healthy subjects. In the animal model, K. oxytoca was found only in the colon of rats that received amoxicillin-clavulanate in addition to being inoculated with K. oxytoca. In these rats, infection with K. oxytoca induced a right-sided hemorrhagic colitis that was not observed in uninfected animals that received amoxicillin-clavulanate, indomethacin (an NSAID), or both. CONCLUSIONS: Our fulfillment of Koch's postulates for cytotoxin-producing K. oxytoca suggests that it is the causative organism in at least some cases of antibiotic-associated hemorrhagic colitis. Infection with K. oxytoca should be considered in patients with antibiotic-associated colitis who are negative for C. difficile.
Adult↗
CD24 expression in urothelial carcinoma of the upper urinary tract correlates with tumour progression.
Expression of the mucin-like adhesion molecule CD24 has been implicated in the progression of several types of cancer and has been identified as new prognostic factor. We evaluated CD24 expression in 268 consecutive cases of upper urinary tract urothelial carcinoma with respect to associations with tumour stage, grade, angioinvasion and infiltrative growth pattern using a tissue microarray technique and correlated data with patient outcome. CD24 expression was demonstrated in 161/259 (62%) evaluable tumours and was associated with high tumour stage [77/139 (55%) pTa/pT1 vs 84/120 (70%) pT2-pT4; P=0.02] and high tumour grade [68/139 (49%) low vs 93/120 (78%) high grade; P<0.001] as well as presence of angioinvasion (P=0.002) and infiltrative pattern of invasion (P=0.007). Patients with CD24-positive tumours tended to have a higher risk of disease progression (P=0.065). Multivariate analysis, however, proved pT stage >1 [P<0.001, risk ratio (RR)=5.87, 95% confidence interval (CI)=2.88-11.95] and high tumour grade (P<0.001, RR=3.30, 95% CI 1.75-6.22) as only independent predictors of metastatic disease. In conclusion, CD24 expression in upper urinary tract urothelial cancer is associated with advanced tumour stage and high tumour grade as well as histopathological features indicative of aggressive tumour behaviour, but it lacks independent impact on patient outcome.
Adult↗
Biologic significance of fascin expression in clear cell renal cell carcinoma: systematic analysis of primary and metastatic tumor tissues using a tissue microarray technique.
OBJECTIVES: To investigate the biologic significance of fascin, a globular actin cross-linking protein, involved in cell adhesion and motility, in primary and metastatic renal cell carcinoma (RCC). METHODS: A total of 136 primary clear cell RCCs and 54 clear cell RCC metastases were stained immunohistochemically using a tissue microarray technique. Distinct cytoplasmic staining was considered positive, and the staining results were associated with the pT stage, Fuhrman grade, tumor size, sarcomatoid morphology, and metastasis-free survival. For multivariate testing, Cox's proportional hazards regression model was used. RESULTS: Fascin expression was noted in 13 (10%) of 136 primary and 25 (46%) of 54 metastatic RCC specimens (P <0.001). Fascin expression was associated with high tumor stage (2 [3%] of 70 pT1 versus 11 [17%] of pT2/pT3; P = 0.008), high tumor grade (3 [3%] of 88 grade 1-2 versus 10 [21%] of 48 grade 3-4; P = 0.002), and large tumor size (P <0.001). In addition, 8 (62%) of 13 RCCs with sarcomatoid morphology expressed fascin compared with 5 (4%) of 123 RCCs without sarcomatoid transformation (P <0.001). Metastatic disease was noted in 10 (77%) of 13 patients with fascin-positive RCC compared with 26 (21%) of 121 patients with fascin-negative RCC (P <0.001). Multivariate analysis revealed pT Stage 1 or greater (P <0.001, risk ratio [RR] 8.6, 95% confidence interval [CI] 2.8 to 26.5), Fuhrman grade greater than 2 (P <0.001, RR 12.7, 95% CI 4.6 to 35.4), fascin expression (P <0.001, RR 7.2, 95% CI 3.0 to 17.4), and female gender (P = 0.02, RR 2.5, 95% CI 1.1 to 5.5) as independent predictors of metastatic disease. CONCLUSIONS: Fascin immunoreactivity in RCC proved to be an independent predictor of metastatic disease and was demonstrated in almost one half of RCC metastases. Thus, fascin may be a promising molecular target for future cancer therapy.
Adult↗
Hypermethylation of hMLH1, HPP1, p14(ARF), p16(INK4A) and APC in primary adenocarcinomas of the small bowel.
Small bowel adenocarcinoma (SB-AC) is a very rare tumor entity. Epigenetic alterations, including hypermethylation of DNA mismatch repair genes and tumor suppressor genes, seem to be important for carcinogenesis in tumors of the gastrointestinal tract, but have not yet been investigated in SB-AC. In the current study, the prevalence of hypermethylation in a panel of genes involved in gastrointestinal carcinogenesis (hMLH1, HPP1, p14(ARF), p16(INK4A), APC) was determined in a series of SB-AC. Paraffin-embedded tumor samples from 56 patients with SB-AC who underwent surgical resection between January 1985 and December 2003 were investigated for hypermethylation by means of methylation-specific real-time PCR, and compared with our findings in a previously investigated series of 50 gastric adenocarcinomas. In comparison with adenocarcinomas of the stomach, SB-AC revealed a significantly higher rate of hypermethylation of HPP1 (86% versus 54%, p = 0.0003), p16(INK4A) (32% versus 10%, p = 0.0006), and a significantly lower rate of hypermethylation of APC (48% versus 84%, p = 0.0001). Hypermethylation of hMLH1 and p14(ARF) was present in 23% and 9% of SB-AC, respectively. Locally advanced tumor categories (pT3/4) showed a higher rate of hypermethylation of HPP1 (90%) than did early tumor categories (pT1/2 categories, 40%; p = 0.0036). This was also reflected by the correlation between the HPP1 hypermethylation and high UICC stage (p = 0.02). No correlation was found between hypermethylation and other clinicopathologic parameters such as age, tumor grade and nodal status. Our findings suggest that hypermethylation of hMLH1, HPP1, p16(INK4A) and APC is frequent in primary adenocarcinomas of the small bowel. The differences in the hypermethylation spectrum of small bowel and stomach cancer indicate significant epigenetic differences between these tumors.
Adaptor Proteins, Signal Transducing↗
Serum levels of vascular endothelial growth factor (VEGF) and endostatin in renal cell carcinoma patients compared to a control group.
OBJECTIVES: Renal cell carcinoma (RCC) is a vascularised neoplasm. The importance of the angiogenic process in its growth and metastatic spreading is widely recognised. We assessed serum levels of endogenous endostatin and vascular endothelial growth factor (VEGF) in RCC patients and healthy volunteers, and evaluated the factors' prognostic role for patients' survival, distinguishing histologic subtypes with respect to correlation with tumour stage, grade, and size. METHODS: We considered 146 consecutive patients with RCC and 110 healthy volunteers. Serum samples from all subjects were analysed for endostatin and VEGF by using competitive enzyme immunoassays. RCC samples were compared with serum from the control group and with clinicopathologic factors and clinical outcome. RESULTS: Mean age was 63 years (range: 37-85 years) in RCC patients and 62 years (range: 23-88 years) in the control group. VEGF levels (median: 3.6 ng/ml+/-6.97; range: 0-48.4 ng/mL) were significantly higher in RCC patients, compared with controls (p=0.001). Endostatin levels did not differ significantly between the two groups (p=0.9) without correlation between endostatin and VEGF levels (p=0.09). No significant difference was found in the endostatin levels among the histologic subtypes (p=0.973) and VEGF (p=0.232). The median follow-up was 27 months (range: 1-57 months). CONCLUSIONS: Serum VEGF levels are elevated in RCC patients, compared with controls, and do not correlate significantly with circulating endostatin levels. No difference in preoperative serum VEGF and endostatin levels among the different histologic subtypes was found. In multivariate analysis VEGF and endostatin failed to be prognostic; only tumour stage and grade remained independent predictors of survival.
Adult↗
No association of primary adenocarcinomas of the small bowel with Epstein-Barr virus infection.
Epstein-Barr Virus (EBV) infection is considered to play an etiologic role in human malignancies, including a subset of gastric and cardiac cancers. Adenocarcinomas of the small bowel comprise a very rare entity, with little knowledge about molecular properties and etiological aspects. We have investigated the prevalence of EBER expression (EBV-encoded small RNAs) in a series of small bowel adenocarcinomas (n=56) utilizing RNA in situ hybridization (EBER-RISH). The patients had undergone primary surgical resection at either the Technical University of Munich or at the University of Graz. A surgical series of 82 primary resected gastric (n=36) or cardiac (n=46) adenocarcinomas (TU Munich) was used as control group. None of the 56 small bowel carcinomas exhibited EBER expression whereas in the control group the rate of EBER expression accounted for 4.4% in the group of cardia carcinomas and 8.6% in the group of gastric cancers. These results indicate that EBV infection plays no etiologic role in primary small bowel adenocarcinomas.
Adenocarcinoma↗
Tumor necrosis as prognostic indicator in transitional cell carcinoma of the upper urinary tract.
PURPOSE: The clinicopathological features predictive of outcome in patients with upper urinary tract transitional cell carcinoma are not clearly established. We analyzed the prognostic impact of tumor necrosis with respect to metastasis-free survival. MATERIALS AND METHODS: The presence of focal (10% or less of the tumor area) and extensive (greater than 10% of the tumor area) coagulative tumor necrosis was retrospectively reevaluated in 268 cases of consecutive upper urinary tract transitional cell carcinoma and correlated with outcome. RESULTS: Overall tumor necrosis was noted in 113 of 268 (42.2%) cases of transitional cell carcinoma with 63 (23.5%) showing focal and 50 (18.7%) showing extensive necrosis, respectively. Tumor necrosis was associated with high tumor stage (p <0.001) and high tumor grade (p <0.001). In addition, tumor necrosis was more common in pelvic tumors compared with ureteral tumors (p <0.001). Actuarial 5-year metastasis-free survival rates for patients with transitional cell carcinoma with extensive, focal and lacking necrosis were 24%, 45% and 78%, respectively (log rank test p <0.001). Multivariate analysis proved pT stage greater than 1 (p <0.001, RR 6.04, 95% CI 2.82-12.93), high tumor grade (p <0.001, RR 3.37, 95% CI 1.65-6.89) and extensive tumor necrosis as independent predictors of poor patient outcome (p = 0.02, RR 1.82, 95% CI 1.09-3.05). CONCLUSIONS: The presence of extensive tumor necrosis proved to be an additional histological variable with an independent influence on metastasis-free survival in patients with upper urinary tract transitional cell carcinoma. Its assessment is readily applicable in routine sections and should thus be commented upon separately in the pathology report.
Adult↗
pT classification, grade, and vascular invasion as prognostic indicators in urothelial carcinoma of the upper urinary tract.
Clinicopathologic features predictive of patient outcome in upper urinary tract urothelial carcinoma are not well defined. The aim of this study was to assess the role of pT classification, tumor grade, and vascular invasion in predicting metastasis-free survival. A total of 190 consecutive invasive upper urinary tract urothelial cancers operated between 01/1984 and 12/2004 were re-evaluated with respect to pT classification, tumor grade (according to the three-tiered WHO 1973 and the recent two-tiered grading system following the WHO/ISUP consensus classification), as well as presence of lymph and/or blood vessel invasion. Prognostic impact was analyzed using the Kaplan-Meier method and the Log-Rank test. For multivariate testing, a Cox's proportional hazards regression model was used. pT1 was present in 81 (43%), pT2 in 29 (15%), pT3 in 73 (38%), and pT4 in seven (4%) cases. There were 12 (6%) G1, 96 (51%) G2, and 82 (43%) G3 tumors or 84 (44%) low-grade and 106 (56%) high-grade tumors according to the two-tiered system. The presence of vascular invasion in 72/190 (38%) tumors was associated with high pT classification (P<0.001) and high tumor grade (P<0.001). Disease progression occurred in 39% of patients, with 5- and 10-year metastasis-free survival rates of 56 and 45%, respectively. On univariate analysis, all investigated parameters showed prognostic significance. The negative influence of vascular invasion on patient outcome was strikingly strong in high pT classification and high-grade cancers. On multivariate analysis, pT classification (P<0.001) and vascular invasion (P<0.001) proved to be independent prognostic factors, whereas tumor grade according to the two-tiered system missed statistical significance (P=0.06). In conclusion, pT classification and vascular invasion are independent prognostic factors with respect to metastasis-free survival and should be used to guide adjuvant therapy strategies in affected patients. Presence (or absence) of vascular invasion should be commented upon separately in the pathology report.
Adult↗
Bladder tumour development after urothelial carcinoma of the upper urinary tract is related to primary tumour location.
OBJECTIVE: To better define the predictors of bladder tumour development in patients operated for upper urinary tract urothelial cancer (UT-UC). PATIENTS AND METHODS: Surgical specimens from 191 consecutive patients with no history of bladder cancer and operated for UT-UC were chosen for analysis. Bladder tumour development was assessed in relation to UT-UC location, tumour multiplicity, stage and grade, margin status, mode of operation, age and gender. RESULTS: Overall, 51 of 191 (27%) patients developed subsequent bladder tumours, including 25 of 123 (20%) with pelvic, 19 of 47 (40%) with ureteric and seven of 21 (33%) with multifocal tumours (P = 0.04 for all subgroups; P = 0.01 for pelvic vs ureteric). There was no influence of the other variables. The median (mean, range) time to recurrence was 12 (18, 3-64) months. In a multivariate analysis, ureteric tumour location was an independent predictor (P = 0.02; risk ratio, RR, 2.0, 95% confidence interval, CI, 1.1-3.7). After excluding 68 patients with systemic disease progression, bladder tumour development was noted in 39 of 123 (32%), including 18 of 76 (24%) with pelvic, 16 of 34 (47%) with ureteric and five of 13 with multifocal tumours (P = 0.06 for all subgroups; P = 0.02 for pelvic vs ureteric). In a multivariate analysis, ureteric location (P = 0.03; RR 2.1, 95% CI 1.1-4.2) and high tumour grade (P = 0.04; RR 2.2, 95% CI 1.03-4.7) were independent predictors of subsequent bladder tumour development. CONCLUSION: The risk of developing a bladder tumour after surgery for UT-UC is significantly related to ureteric tumour location and high tumour grade. Clinical trials to evaluate a possible reduction of bladder cancer risk by intraoperative ureteric ligation and/or peri-operative topical intravesical chemotherapy instillation are justified.
Adult↗
Puumala virus RNA in patient with multiorgan failure.
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Austria↗
Alpha-methylacyl-CoA racemase (AMACR/P504S) protein expression in urothelial carcinoma of the upper urinary tract correlates with tumour progression.
Alpha-methylacyl-CoA racemase (AMACR/P504S) is a useful biomarker of prostate cancer. We evaluated the expression of AMACR in upper urinary tract urothelial carcinomas with respect to associations with tumour stage, grade and metastasis-free survival. A total of 268 tumours were investigated immunohistochemically using a tissue microarray technique. AMACR expression was noted in 127 of 261 (48.7%) evaluated tumours and was associated with high tumour stage [58 of 139 (41.7%) pTa/pT1 vs. 69 of 122 (56.6%) pT2-pT4, P=0.019] and high tumour grade [44 of 137 (32.1%) low vs. 83 of 124 (66.9%) high grade, P<0.001]. In addition, AMACR expression was associated with the presence of tumour necrosis (P<0.001) and marked stromal desmoplasia (P=0.0026). This correlation indicates that increased AMACR expression might be related to hypoxia-induced changes in cancer cell metabolism, such as increased dependence on fatty acid oxidation for energy generation. Progressive disease was observed in 73 of 183 (39.9%) patients with solitary invasive carcinomas and was associated with AMACR expression (P=0.017). Multivariate analysis, however, proved only pT-stage >1 (P<0.001) and high tumour grade (P<0.001) to be independent predictors of patient outcome. In conclusion, AMACR expression correlated with advanced tumour stage and grade and may serve as an additional prognostic indicator in upper urinary tract urothelial cancer.
Adult↗
Analysis of trefoil factor family protein 1 (TFF1, pS2) expression in chronic cholecystitis and gallbladder carcinoma.
Trefoil factor family protein 1 (TFF1, pS2) interacts with mucins to protect gastrointestinal epithelium against injury and contributes to mucosal repair by promoting epithelial cell migration and restitution. Moreover, TFF1 has antiproliferative and anti-apoptotic effects and promotes cell scattering and invasion. We investigated TFF1 expression in healthy and inflamed non-neoplastic gallbladder mucosa as well as in gallbladder carcinomas (n=57) and corresponding metastases (n=18), using a tissue microarray technique. TFF1 immunoreactivity was absent in healthy mucosa, focally observed in epithelium with inflammatory changes and present in 35% of primary and 24% of metastatic cancer tissues. Immunoreactivity significantly decreased with increasing tumour stage (P=0.009) and increasing tumour grade (P=0.001). Patients with TFF1 positive tumours showed a more favourable outcome compared to patients with TFF1 negative tumours in univariate analysis (P=0.006). However, multivariate analysis proved resection status and tumour grade as the only independent prognostic factors. In conclusion, TFF1 is expressed in inflamed non-neoplastic gallbladder epithelium and in low stage and low grade gallbladder carcinomas. Thus, TFF1 may be the missing link between gallstones, chronic cholecystitis and gallbladder cancer. Further studies are needed to evaluate whether TFF1 immunostaining can be used as a diagnostic tool to identify patients with a more favourable outcome.
Adenocarcinoma↗
CAR and PXR agonists stimulate hepatic bile acid and bilirubin detoxification and elimination pathways in mice.
Induction of hepatic phase I/II detoxification enzymes and alternative excretory pumps may limit hepatocellular accumulation of toxic biliary compounds in cholestasis. Because the nuclear xenobiotic receptors constitutive androstane receptor (CAR) and pregnane X receptor (PXR) regulate involved enzymes and transporters, we aimed to induce adaptive alternative pathways with different CAR and PXR agonists in vivo. Mice were treated with the CAR agonists phenobarbital and 1,4-bis-[2-(3,5-dichlorpyridyloxy)]benzene, as well as the PXR agonists atorvastatin and pregnenolone-16alpha-carbonitrile. Hepatic bile acid and bilirubin-metabolizing/detoxifying enzymes (Cyp2b10, Cyp3a11, Ugt1a1, Sult2a1), their regulatory nuclear receptors (CAR, PXR, farnesoid X receptor), and bile acid/organic anion and lipid transporters (Ntcp, Oatp1,2,4, Bsep, Mrp2-4, Mdr2, Abcg5/8, Asbt) in the liver and kidney were analyzed via reverse-transcriptase polymerase chain reaction and Western blotting. Potential functional relevance was tested in common bile duct ligation (CBDL). CAR agonists induced Mrp2-4 and Oatp2; PXR agonists induced only Mrp3 and Oatp2. Both PXR and CAR agonists profoundly stimulated bile acid-hydroxylating/detoxifying enzymes Cyp3a11 and Cyp2b10. In addition, CAR agonists upregulated bile acid-sulfating Sult2a1 and bilirubin-glucuronidating Ugt1a1. These changes were accompanied by reduced serum levels of bilirubin and bile acids in healthy and CBDL mice and by increased levels of polyhydroxylated bile acids in serum and urine of cholestatic mice. Atorvastatin significantly increased Oatp2, Mdr2, and Asbt, while other transporters and enzymes were moderately affected. In conclusion, administration of specific CAR or PXR ligands results in coordinated stimulation of major hepatic bile acid/bilirubin metabolizing and detoxifying enzymes and hepatic key alternative efflux systems, effects that are predicted to counteract cholestasis.
Animals↗
Enterolithiasis in jejunal diverticulosis, a rare cause of obstruction of the small intestine: a case report.
Jejunal diverticula are rare and usually asymptomatic; they occur twice as frequently in men. They are discovered incidentally during small-bowel enteroclysis, CT scan or laparotomy. Complications include diverticulitis, perforation, hemorrhage and enterolith formation. Intestinal obstruction due to enterolithiasis is uncommon. We present the association of enterolithiasis and jejunal diverticulosis causing obstruction of the small intestine in a 74-year-old female who was admitted for abdominal cramps, nausea and vomiting. On physical examination, there was discomfort on palpation of the upper abdomen. Laboratory tests revealed mild elevation of leucocytes and C-reactive protein. CT scan demonstrated dilatated loops of proximal jejunum with thickening of the wall, suggesting ingestion of a foreign body. Clinical and radiological findings did not indicate conservative therapy; our patient underwent minilaparotomy, and pronounced jejunal diverticulosis was identified. An enterotomy was performed and a cylindrical enterolith, 10cm long and 3cm in diameter, was removed. The operative and postoperative course was uneventful. Enterolithiasis must be considered as a potential source of intestinal obstruction. The differential diagnosis should take gallstone ileus and ingestion of a foreign body into consideration. Initial therapy is nonoperative; if this management fails, surgery is indicated.
Aged↗
Congenital absence of the portal vein in an adult woman: a case report.
Congenital absence of the portal vein is a rare malformation. Mesenteric and splenic venous blood bypasses the liver and drains into the renal veins or directly into the inferior vena cava. The malformation occurs predominantly in females and children and is often associated with other anomalies such as hepatic tumors, cardiac malformations and skeletal abnormalities. We describe a 23-year-old female with congenital absence of the portal vein associated with variant origin of the hepatic artery from the superior mesenteric artery. Additional anomalies were not present. The patient is in good condition. Laboratory tests showed elevated total bilirubin; other liver parameters were within normal range. The absence of the portal vein was confirmed by MR angiography, which revealed that the upper part of the superior mesenteric vein and the splenic vein drained into the left renal vein. A liver biopsy showed no signs of cirrhosis. Yearly follow-up with laboratory tests and ultrasonography was recommended to our patient. According to the literature, the prognosis of this patient depends on the presence of cardiac defects and liver tumors. There is little information on the outcome of patients with congenital absence of the portal vein; only long-term follow-up investigations will provide answers to the numerous remaining questions.
Abdominal Pain↗
Surgical therapy options in polycystic liver disease.
INTRODUCTION: Polycystic liver disease (PLD) is a rare affliction frequently observed in association with polycystic kidney disease. Only symptomatic patients require treatment, which can be conservative or surgical, i.e. laparoscopic or conventional. We report the results of our experience in the surgical management of polycystic liver disease. METHODS: Between 1994 and 2003, 19 patients (16 female, 3 male) were referred to our center for the management of PLD. Their median age was 50 years (range 33-72). All were symptomatic and their cysts had a median diameter of 11 cm (range 5-22). RESULTS: Laparoscopic management was undertaken in eight patients, with one conversion to open technique because of bleeding from a superficial hepatic vein. An open procedure was performed in 11 patients: one left hemihepatectomy, deroofing in two patients, segment resection 2/3 plus deroofing in six patients, and segment resection 5/6 plus deroofing in two patients. Four patients had complications: one case of biliary leakage was managed conservatively; two patients had pneumothorax caused by the cava catheter inserted for anesthesia, and one patient's abdominal drain tore off and had to be removed by relaparotomy on the fourth postoperative day. Median follow-up of all patients was 49 months (range 7-98). In one patient there was symptomatic recurrence with hepatomegaly and compression of the inferior vena cava 84 months after the first operation. CONCLUSIONS: Careful selection of patients and meticulous surgical technique are recommended in the management of PLD. The treatment of choice for symptomatic Gigot or Morino type 1 PLD is laparoscopic surgery, and for advanced stage PLD combined hepatic resection and cyst fenestration.
Adult↗