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Background: Benign liver tumours (e.g., hepatocellular adenoma (HCA), focal nodular hyperplasia (FNH), and haemangioma) are occasionally resected for alleged symptoms, although data on long-term outcomes is lacking. The aim of this cross-sectional study was to assess long-term outcomes of surgical intervention., Methods: Forty patients with benign tumours (HCA 20, FNH 12, giant haemangioma 4, cysts 4) were included. Patients filled in Validated McGill Pain Questionnaires, preoperatively and after a median of 54 months after resection. Outcomes were evaluated using paired sample t-test and (M) ANOVA., Results: Relief of symptoms sustained in 30/40 patients, within a follow-up of 54 (24-148) months after resection. VAS scores were reduced from 5.5 preoperatively to 1.6 postoperatively (p < 0.001). Patients with left-sided tumours had higher postoperative Pain Rating Index (PRI), compared to patients with right-sided tumours: 15.3 vs. 5.8 (p = 0.018). If patients could reconsider undergoing surgery, 34/38 would again choose resection. Discomfort at the operative scar was the most common complaint: 8/40 patients, all after open surgery, of whom 3/40 had an incisional hernia. 7/40 patients had a laparoscopic resection., Conclusion: Resection relieved symptoms in 30/40 patients. The operative scar was a frequent source for remaining postoperative complaints, suggesting an advantage for a laparoscopic approach when feasible., (Copyright © 2016 International Hepato-Pancreato-Biliary Association Inc. Published by Elsevier Ltd. All rights reserved.)

Objectives: The clinical management of hepatocellular adenoma (HCA) and focal nodular hyperplasia (FNH) is still subject to controversy, especially with respect to patient selection for surgery. The aim of this prospective cohort study was to assess the outcomes of surgical intervention., Methods: Between January 2008 and September 2012, patients diagnosed with FNH or HCA based on magnetic resonance imaging or computed tomography were enrolled in this prospective study. Resection was undertaken in patients with HCA of > 5 cm or symptomatic lesions. Lesion characteristics, extent of liver resection (minor: fewer than three segments; major: three or more segments), morbidity (by Dindo-Clavien class), mortality, postoperative length of stay and symptoms [McGill Pain Questionnaire, including a visual analogue scale (VAS)] were evaluated., Results: A total of 110 patients (106 female; median age: 39 years) were included; 51 patients had HCA and 59 had FNH. Of the 110 patients, 49 underwent resection (33 HCA patients; 16 FNH patients). Laparoscopic minor resection was performed in five HCA and five FNH patients; open minor resection was performed in 19 HCA and seven FNH patients, and open major resection was performed in nine HCA and four FNH patients. Severe postoperative complications were observed in four patients (Grade III, n = 3; Grade IV, n = 1). Median baseline scores on the VAS were 6 in FNH patients and 7 in HCA patients; the median VAS score after resection was 0 (P = 0.008)., Conclusions: If patients with HCA and FNH require surgery, limited resection can be carried out with low morbidity and without mortality. Patients with preoperative symptoms show a high rate of postoperative symptom relief., (© 2013 International Hepato-Pancreato-Biliary Association.)

Background: Chemoradiotherapy is increasingly applied in patients with oesophageal cancer. The aim of the present study was to determine whether 3D-CT volumetry is able to differentiate between responding and non-responding oesophageal tumours early in the course of neoadjuvant chemoradiotherapy., Patients and Methods: Serial CT before and after two weeks of neoadjuvant chemoradiotherapy was performed in the multimodality treatment arm of a randomised trial including patients with oesophageal carcinoma. CT response was measured with the change in tumour volume between baseline and after 14 days of neoadjuvant therapy. Receiver Operating Characteristic (ROC) analysis was used to evaluate the ability of 3D-CT as an early imaging marker of response., Results: CT response analysis was performed in 39 patients, of whom 26 patients were histopathological responders. Median tumour volume increased between baseline and after 14 days of chemoradiotherapy in histopathological responders as well as in non-responders, though changes were not statistically significant. The area under the ROC curve was 0.71., Conclusion: Tumour volume changes after 14 days of neoadjuvant chemoradiotherapy as measured by 3D-CT were not associated with histopathological tumour response. CT volumetry should not be used for early response assessment in patients with potentially curable oesophageal cancer treated with neoadjuvant chemoradiotherapy., (Copyright © 2011 Elsevier Ltd. All rights reserved.)

Background: Diagnostic laparoscopy has been generally accepted in staging of patients with a periampullary malignancy. In our institution diagnostic laparoscopy was routinely used since 1992. However, in 1998 it was eliminated from the protocol since in a prospective study a yield of only 13% was found with a histologically proven accuracy of 60% for distant metastases. The effect of implementation of the new protocol on the occurrence of unnecessary laparotomies and the outcome after bypass surgery was assessed., Methods: Between January 1999 and December 2001, 186 consecutive patients with a potentially resectable periampullary carcinoma after radiological staging without diagnostic laparoscopy underwent explorative laparotomy with the intention to perform a curative pancreatoduodenectomy. Incidence of unresectability and outcome of palliative surgery were assessed., Results: Resection could not be performed in 65 patients who underwent laparotomy because of metastatic disease (29 patients) and loco-regional tumour ingrowth (34 patients). These patients underwent a bypass procedure with a median survival of 216 days., Conclusion: At laparotomy distant metastases were detected in 16% of the patients. Considering the fact that the detection rate of diagnostic laparoscopy is lower than 100%, the use of staging laparotomy is too limited to justify it as a routine procedure.

Previous studies have shown that enzyme supplementation therapy with alglucerase for type 1 Gaucher's disease is effective at doses of 30-130 U/kg per month. Since both the clinical presentation and the response to therapy in Gaucher's disease are highly variable, individual dosing seems indicated. This notion, as well as the high costs of alglucerase and the unknown long-term side-effects, led us to investigate the efficacy of an individualised very low dose of alglucerase. Twenty-five adults with symptomatic type 1 Gaucher's disease (thirteen splenectomised) received alglucerase 1.15 U/kg three times a week (15 U/kg per month). Every 6 months, the dose was halved, maintained, or doubled, according to the response (based on haematological variables and liver and spleen volume). After 6 months of treatment, eighteen (72%) patients had a response (seventeen moderate, one good). After 12 months (in nineteen patients) and 18 months (in seven patients), all had sustained improvement. Severe splenomegaly resulted in slower haematological responses. Our results are similar to those obtained by others with higher-dose regimens and better than a low-dose regimen of 10U/kg every 2 weeks. We conclude that very low initial doses of alglucerase, when administered frequently, are effective and cost-saving in the treatment of type 1 Gaucher's disease.