Visual outcomes and predictors in optic pathway glioma: a single centre study.

BACKGROUND/AIMS: Optic pathway gliomas (OPGs) may cause progressive visual loss despite chemotherapy. Newer, less toxic treatments might be given earlier, depending on visual prognosis. We aimed to investigate the prognostic value of visual evoked potentials (VEP) and optical coherence tomography (OCT). METHODS: A retrospective study of OPG patients (treated 2003-2017) was conducted. Primary outcome was PEDIG category visual acuity in better and worse eyes (good < = 0.2, moderate 0.3-0.6 and poor > = 0.7 logMAR). Binary logistic regression analysis was used to identify predictors of these outcomes. RESULTS: 60 patients (32 Neurofibromatosis type 1 [NF1] and 28 sporadic) had median presentation age 49 months (range 17-183) (NF1) and 27 months (range 4-92) (sporadic). Median follow up was 82 months (range 12-189 months). At follow up 24/32 (75%) of NF1 children and 14/28 (50%) of sporadic children had good better eye visual acuity and 11/32 (34%) of NF1 children and 15/28 (54%) of sporadics had poor worse eye acuity. Mean peripapillary retinal nerve fibre layer (RNFL) thickness predicted good better eye final acuity (OR 0.799, 95%CI 0.646-0.987, p = 0.038). Presenting with visual symptoms (OR 0.22 95% CI 0.001-0.508, p = 0.017) and poorer VEP scores (OR 2.35 95% CI 1.1-5.03, p = 0.027) predicted poor worse eye final acuity. 16 children had homonymous hemianopias at follow up, predicted by poor presenting binocular VEP score (OR 1.449 95%CI 1.052-1.995, p = 0.02). CONCLUSIONS: We found that both RNFL thickness on OCT and VEP were useful in predicting future visual acuity and vision and potentially in planning treatment. We had a high prevalence of homonymous hemianopia.

A rare case of transmural endometriosis in primary adenocarcinoma of the rectum.

Intestinal endometriosis of the rectum and sigmoid colon, occurring in up to 34% of pelvic endometriosis, mimics a wide number of conditions that are difficult to differentiate from inflammatory or malignant diseases. Herein we report the first case of transmural endometriosis concomitant with advanced primary rectal adenocarcinoma, presenting with obstructive symptoms. Correct diagnosis based on morphological identification and immunohistochemical characterization of the two entities is crucial for treatment.

External validation of a simplified BCLC staging system for early hepatocellular carcinoma.

BACKGROUND AND AIMS: The aim was to externally validate the capability of a simplified Barcelona Clinic Liver Cancer (s-BCLC) staging system in allocating patients to hepatic resection (HR) and the effect on survival: S-BCLC was defined by only 2 groups: AA included BCLC A1 + A2 classes with alpha-fetoprotein (AFP) ≤ 20 ng/ml and AB included A1 + A2 with AFP > 20 ng/ml plus A3 + A4 subgroups. METHODS: This study compared a training group (TG) with hepatocellular carcinoma (HCC) submitted to hepatic resection (HR) in Milan with another group of patients, the validation group (VG) in Creteil. All patients underwent ultrasound-guided anatomical resection (<3 segments). RESULTS: Overall survival got worse from A1 to A4 (p = 0.0271) in TG (n = 132), as well as in VG (n = 100) (p = 0.0044) with a more important overlapping of each curves. According s-BCLC classification, the survival curves of TG (p = 0.0001) and VG (p = 0.0250) showed a definitive separation in two different staging groups. The s-BCLC provided the best predictive accuracy and it also presented the highest separability index and C-statistics in both TG and VG. On the other hand, in the evaluation of discriminatory ability for death, measured by ROC curve areas, the s-BCLC system gave better results than the others. CONCLUSION: This experience stressed the high value of BCLC system in staging of HCC, but the s-BCLC system seems to be more useful for therapeutic decision making.

Unusual neuroimaging in a young boy with cerebral X-linked adrenoleukodystrophy.

We describe the case of a 7-year-old boy with cerebral X-linked adrenoleukodystrophy presenting with neurobehavioral symptoms. Attention deficit with hyperactive behavior was followed by progressive memory retrieval deficits and difficulty in writing. A detailed review of the clinical history, clinical examination and contrast-enhanced brain nuclear magnetic resonance imaging disclosed indeed an atypical cerebral form of X-linked adrenoleukodystrophy with diffuse anterior and posterior white matter involvement with a typical rim of contrast enhancement. A severe clinical deterioration occurred despite dietary treatment and hormonal replacement therapy. These rare but severe forms of atypical cerebral X-linked adrenoleukodystrophy can more often mimic attention deficit/hyperactivity disorder and should, therefore, be considered in young males with progressive neurobehavioral symptoms, particularly when possibly related to frontal white matter involvement. An accurate clinical history and examination should determine whenever a contrast-enhanced head MRI should be indicated.

Intraoperative ultrasound patterns predict recurrences after surgical treatments for hepatocellular carcinoma().

INTRODUCTION: Hepatocellular carcinoma (HCC) is associated with a high incidence of postoperative recurrence, despite high rates of complete necrosis with radiofrequency ablation (RFA) and curative hepatic resections (HR). The aim of this study was to identify intraoperative ultrasound patterns observed during HR or RFA that predicting intrahepatic HCC recurrence. MATERIALS AND METHODS: From January 1997 through August 2008, we treated 377 patients with HCC (158 with HR and 219 with surgical RFA). All patients underwent intraoperative ultrasound (IOUS) examination. Primary HCCs was classified according to diameter, HCC pattern (nodular or infiltrative), echogenicity (hyper- or hypo-), echotexture (homogeneous or inhomogeneous), capsular invasion, mosaic pattern, nodule-in-nodule appearance, and infiltration of portal vessels. Number of HCC nodules was also considered. Comparisons between the groups of possible factors for intrahepatic recurrence of treated tumors were performed using the Kaplan-Meier method and compared using the log-rank test. RESULTS: Patients were followed for 9-127 months (median: 18.6 months), and intrahepatic recurrence was observed in 198 (52.5%). In 138 patients (36.5%), recurrences were located in different segments with respect to the primary tumor. In 60 HCC tumors (16%), local recurrences were found in the same segment as the primary tumor. At univariate analysis, primary HCC echogenicity and mosaic pattern were the only factors not significant associated with intrahepatic recurrences. CONCLUSION: IOUS is an accurate staging tool for use during "surgical" resection or RFA. This study shows that IOUS patterns can also be used to estimate the risk of post-treatment HCC recurrence. In patients at high risk for this outcome, closer follow-up and use of adjuvant therapies could be useful.

Bariatric surgery in obesity: changes of glucose and lipid metabolism correlate with changes of fat mass.

BACKGROUND AND AIM: Bariatric surgery induces significant weight loss and improves glucose metabolism in obese patients (BMI>35 kg/m(2)). Our aim was to compare restrictive (LAGB, laparoscopic gastric banding) and malabsorptive approaches (BIBP, biliary-intestinal bypass) on the loss of fat-free mass (FFM), fat mass (FM), and on changes of glucose and lipid metabolism. METHODS AND RESULTS: Body composition (bio-impedance analysis, BIA), blood glucose (BG), insulin, triglycerides, total- and HDL-cholesterol, liver enzymes (AST and ALT) were measured at baseline and 1 year after surgery in patients undergoing LAGB, BIBP, and in diet-treated control patients. In the main study, with patients matched for initial BMI (43-55 kg/m(2), LAGB=24, BIBP=12, controls=6), decreases of BMI, FM, BG and cholesterol were greater in patients with BIBP than with LAGB (p<0.01), while decreases of FFM, insulin, HOMA-IR and triglycerides were similar. No effects on BMI, FM, FFM, BG, insulin, HOMA-IR or cholesterol were observed in the control patients. Decreases of BG, insulin, HOMA-IR, cholesterol and triglycerides correlated with FM but not with FFM decrease. Similar results were obtained in an additional study in patients with a different initial BMI (LAGB=25, BIBP=6, controls=24) and when considering all subjects together. A decrease of liver enzymes (ALT) was greater with LAGB than with BIBP, and HDL-cholesterol increased with LAGB and decreased with BIBP. CONCLUSION: BMI, FM, BG and cholesterol decrease more with malabsorptive than with restrictive surgery, while FFM, insulin, HOMA-IR and triglycerides decrease in a similar way. FFM loss is of low entity. Changes of glucose and lipid metabolism are proportional to a decrease of fat mass but not of fat-free mass.

Laparoscopic liver resections for hepatocellular carcinoma. Is it a feasible option for patients with liver cirrhosis?

BACKGROUND: Laparoscopic surgery has gained growing acceptance, but this does not hold for laparoscopic surgery of the liver, above all for patients with hepatocellular carcinoma (HCC) and cirrhosis. This approach mainly includes diagnostic procedures and interstitial therapies. However, we believe there is room for laparoscopic liver resections in well-selected cases. The aim of this study is to assess: (a) the risk of intraoperative bleeding and postoperative complications, (b) the safety and the respect of oncological criteria, and (c) the potential benefit of laparoscopic ultrasound in guiding liver resection. METHODS: A prospective study of laparoscopic liver resections for hepatocellular carcinoma was undertaken in patients with compensated cirrhosis. Hepatic involvement had to be limited and located in the left or peripheral right segments (segments 2-6), and the tumor had to be 5 cm or smaller. Tumor location and its transection margins were defined by laparoscopic ultrasound. RESULTS: From January 1997, 22 out of 250 patients with HCC (9%) underwent laparoscopic liver resections. The mean patient age was 61.4 years (range, 50-79 years). In three patients, conversion to laparotomy was necessary. The laparoscopic resections included five bisegmentectoies (2 and 3), nine segmentectomies, two subsegmentectomies and three nonanatomical resections for extrahepatic growing lesions. The mean operative time, including laparoscopic ultrasonography, was 199 +/- 69 min (median, 220; range, 80-300). Perioperative blood loss was 183 +/- 72 ml (median, 160; range, 80-400 ml). There was no mortality. Postoperative complications occurred in two out of 19 patients: an abdominal wall hematoma occurred in one patient and a bleeding from a trocar access in the other patient requiring a laparoscopic re-exploration. Mean hospital stay of the whole series was 6.5 +/- 4.3 days (median, 5; range, 4-25), while the mean hospital stay of the 19 laparoscopic patients was 5.4 +/- 1 (median, 5; range, 4-8). CONCLUSION: Laparoscopic treatment should be considered in selected patients with HCC and liver cirrhosis in the left lobe or segments 5 and 6 of the liver. It is clear that certain types of laparoscopic resection are feasible and safe when carried out by adequately skilled surgeons with appropriate instruments.

Laparoscopic radiofrequency of hepatocellular carcinoma using ultrasound-guided selective intrahepatic vascular occlusion.

BACKGROUND: The optimal treatment for hepatocellular carcinoma (HCC) is surgical resection. However, only a small percentage of patients are operative candidates. The authors aimed to assess a novel operative combination of laparoscopic radiofrequency (LRF) with a selective intrahepatic vascular occlusion (SIHVO) to obtain an increased rate of total necrosis and a reduced rate of local HCC recurrences. METHODS: For this study, 37 patients with HCC in liver cirrhosis were submitted to LRF with SIHVO. An LRF was indicated for patients not amenable to liver resection who evidenced at least one of the following criteria: severe impairment of the coagulation tests, large tumors (but <5 cm) or multiple lesions requiring repeated punctures, superficial lesions adjacent to visceral structures, deep-sited lesions with a very difficult or impossible percutaneous approach, and short-term recurrence of HCC after percutaneous loco-regional therapies. RESULTS: Laparoscopic ultrasound identified seven new malignant lesions (19%) undetected by preoperative imaging. There was no operative mortality. Of the 37 patients, 31 experienced no complications (84%). Computed tomography (CT) evaluation 1 month after treatment showed that a complete response with 100% necrosis had been achieved for all the patients (100%). During the follow-up period (mean, 11.8 +/- 8.2 months), new malignant nodules developed in 14 patients (42%), and 36% of these recurrences were located in the same treated segment of the HCC. CONCLUSIONS: The combined LRF and SIHVO procedure proved to be a safe and effective technique at least in the short and mid term. In fact, it permitted the treatment of lesions not treatable using the percutaneous approach with a complete clearance, and it had a low morbidity rate.

Laparoscopic radiofrequency ablation of hepatocellular carcinoma: A critical review from the surgeon's perspective.

BACKGROUND: The treatment of choice for hepatocellular carcinoma (HCC) is surgical resection but only a small percentage of patients are operative candidates. Percutaneous radiofrequency interstitial thermal ablation (RFA) has proved to be effective in the treatment of unresectable HCC. However, there is a sub-group of patients who may benefit from a laparoscopic rather than a percutaneous approach. Laparoscopic RFA offers the combined advantages of improved tumor staging based on the intracorporeal ultrasound examination and safer access to liver lesions that are difficult or impossible to treat with a percutaneous approach. The aim of our review was to evaluate the advantages and limitations of the laparoscopic approach, according to the criteria of evidence-based medicine. CONCLUSIONS: Laparoscopic RFA of HCC proved to be a safe and effective technique, at least in terms of the short- and mid-term results. This technique may be indicated in selected cases of HCC when percutaneous RFA is very difficult or contraindicated.

Impact of intraoperative ultrasonography in laparoscopic liver surgery.

BACKGROUND: Laparoscopic surgery has gained growing acceptance, but this does not hold for laparoscopic surgery of the liver. This mainly includes diagnostic procedures, interstitial therapies, and treatment of liver cysts. However, the authors believe there is room for a laparoscopic approach to the liver in selected cases. METHODS: A prospective study of laparoscopic liver resections was undertaken with patients who had preoperative diagnoses of benign lesion and hepatocellular carcinoma with compensated cirrhosis. The inclusion criteria required that hepatic involvement be limited and located in the left or peripheral right segments (segments 2-6), and that the tumor be 5 cm or smaller. The location of the tumor and its transection margin were defined by laparoscopic ultrasound (LUS). RESULTS: From December 1996, 17 (5%) of 313 liver resections were included in the study. There were 5 benign lesions and 12 hepatocellular carcinomas in cirrhotic patients. The mean age of the study patients was 59 years (range, 29-79 years). The LUS evaluation identified the presence of new hepatocellular carcinoma nodules in two patients (17%). The resections included 1 bisegmentectomy, 8 segmentectomies, 3 subsegmentectomies, and 3 nonanatomic resections. The mean operative time, including laparoscopic ultrasonography, was 156 +/- 50 min (median, 150 min; range, 60-250 min), and the perioperative blood loss was 190 +/- 97 ml. There was no mortality. Conversion to laparotomy was necessary for two patients. Postoperative complications were experienced by 3 of 15 patients, all of them cirrhotics. One of the patients had a wall hematoma, and the remaining two patients had bleeding from a trocar access requiring a laparoscopic reexploration. The mean hospital stay for the whole series was 6.9 +/- 4.9 days (median, 6 days; range, 2-25 days) and 5.6 +/-1.4 days (median, 6 days; range, 2-8 days) for the 15 laparoscopic patients. CONCLUSION: Laparoscopic treatment should be considered for selected patients with benign and malignant lesions in the left lobe or frontal segments of the liver. Evaluation by LUS is indispensable to guarantee precise determination of the segmental tumor location and the relationship of the tumor to adjacent vascular or biliary structures, excluding adjacent or adjunctive new lesions. The evolution of laparoscopic hepatectomies probably will depend on the development of new techniques and instrumentations.

Laparoscopic ultrasound-guided resection of gastric submucosal tumors.

BACKGROUND: The laparoscopic resection of gastric stromal tumors is being performed with increased frequency. Wedge resection of anterior wall lesions is generally performed. The treatment of posterior wall lesions is still controversial. METHODS: We report three cases of gastric submucosal tumors treated by a laparoscopic wedge resection of the stomach. All lesions were localized anterior gastric wall by intraoperative ultrasound on the. In the first patient the resection was performed with an endoscopic stapler; in the other patients, ultrasonic coagulation in association with an intracorporeal suture has been used. RESULTS: All patients were successfully treated laparoscopically; there were no conversions to open surgery. In all cases the operative course was uneventful. The postoperative hospital stay ranged from 6 to 8 days. CONCLUSIONS: The results suggest that laparoscopic surgery is an adequate strategy for gastric submucosal neoplasms including gastrointestinal stromal tumors (GIST). Intraoperative ultrasound is very useful in the selection of the technical approach with or without the endoscopic stapler.

Hepatobiliary cystadenoma: diagnostic uncertainty.

BACKGROUND: Hepatobiliary cystadenoma is a rare tumour that can be clinically 'silent' and only discovered as an incidental finding on ultrasonography (US). It can also be symptomatic with abdominal pain and jaundice or develop internal bleeding and (exceptionally) malignant degeneration. Therefore the treatment of choice is liver resection. CASE OUTLINE: A 77-year-old woman was admitted with mild jaundice and right hypochondrial pain. For 7 years she had been known to have a silent liver lesion, always considered to be a simple cyst on US. US and CT scan revealed a multi-septate mass involving segments IV, V and VIII of the liver, with thick walls, no calcifications and no contrast enhancement. US-guided aspiration showed the presence of old blood-stained material. The patient was operated with a suspected diagnosis of bleeding into a simple cyst. A Lin fenestration was performed with wide excision of the anterior wall of the cyst. Pathological examination demonstrated a mucinous hepatobiliary cystadenoma. The postoperative course was uneventful, and follow-up at 2 years confirmed no recurrence. DISCUSSION: Imaging will normally help to distinguish the occasional hepatobiliary cystadenoma from the common simple cyst. Otherwise, when a complication occurs, preoperative differentiation may become impossible, and requires histological examination of the cyst after surgical removal.

Laparoscopy with laparoscopic ultrasound for pretreatment staging of hepatocellular carcinoma: a prospective study.

Laparoscopy with laparoscopic ultrasound (L-LUS) has proved to be superior to conventional CT imaging in the staging of hepatocellular carcinoma (HCC). The aim of our prospective study was to evaluate the efficacy of L-LUS as compared with currently available imaging techniques (spiral CT or Lipiodol CT) in patients with HCC and liver cirrhosis. From January 1998 to May 2000, 70 consecutive patients (50 men and 20 women; mean age 67 +/- 7 years) were enrolled. Liver cirrhosis was related to chronic hepatitis C virus infection in 55, hepatitis B virus infection in seven, and alcohol abuse in eight patients. Preoperative diagnostic workup included the following: 70 ultrasound examinations of the liver, 23 CT scans after Lipiodol arteriography, and 53 spiral CT scans. A single lesion was found in 39 patients, two lesions in 20 patients, and three lesions in 11 patients. L-LUS was performed in all patients under general anesthesia using a two- to three-trocar technique. The examination was completed in 68 patients (97%); in two cases extensive adhesions prevented the L-LUS examination. L-LUS yielded additional information in 39 patients (57%). New histologically proved HCC lesions were detected in 14 patients (in the same liver segment in 4 cases and in different liver segments in 10 cases), and an adrenal metastasis was seen in one patient. In 23 patients, benign nodules were identified as regenerative macronodules, low-grade dysplastic nodules, or small hemangiomas. In 10 patients, correct localization of the primary lesion was detected by L-LUS in comparison with the preoperative liver location. In our experience, L-LUS is a safe and reliable procedure. It provides superior information (intraoperative histologic confirmation) for the diagnosis and pretreatment staging of HCC in patients with cirrhosis when compared with current radiologic imaging techniques.

Radiofrequency interstitial thermal ablation of hepatocellular carcinoma in liver cirrhosis. Role of the laparoscopic approach.

BACKGROUND: The laparoscopic approach to radiofrequency interstitial thermal ablation (RITA) of hepatocellular carcinoma (HCC) with intraoperative ultrasound guidance has been proposed with the aim of obtaining additional information for a better neoplastic staging and a complete and effective treatment of the liver lesions in patients with a difficult percutaneous approach. METHODS: In this pilot study, 29 patients with HCC in liver cirrhosis were submitted to laparoscopic RITA under sonographic guide. Most of these patients were in Child's A class of liver function. Patients with large tumors (> 5 cm), portal vein thrombosis, or severe liver disease (Child's C class) were excluded from the study. RESULTS: The laparoscopic RITA procedure was completed in 27 of 29 patients (93% feasibility rate). The laparoscopic ultrasound examination identified new malignant liver nodules in five patients (18.5%). A total of 44 lesions were treated. The mean operative time was 75.8 +/- 20.5 min (range, 45-120 min), and the mean RITA time was 18 +/- 10 min (range, 10-56 min). There was no operative mortality, and postoperative morbidity was low (four cases) without any mortality. A complete tumor necrosis was observed in 90% of the patients via spiral computed tomography (CT) 1 month after treatment. CONCLUSIONS: Laparoscopic RITA of hepatocellular carcinoma proved to be a safe and effective technique, at least in the short term. Its role in the treatment of HCC needs to be defined in larger series.

Laparoscopic radiofrequency of hepatocellular carcinoma (HCC) in liver cirrhosis.

BACKGROUND/AIMS: In this report, the feasibility and efficacy of laparoscopic radiofrequency interstitial thermal ablation of hepatocellular carcinoma has been evaluated in 20 patients. METHODOLOGY: The laparoscopic approach with the use of intraoperative ultrasonography allowed us to obtain additional information regarding liver nodules and a complete treatment of the liver lesions. RESULTS: The complication rate was low and there was no operative mortality. A complete necrosis has been obtained in 90% of the cases at 1 month dynamic computed tomography following the treatment. CONCLUSIONS: Laparoscopic radiofrequency thermal ablation of hepatocellular carcinoma proved to be a safe and effective technique; its use may be proposed in selected patients. Larger series are needed to accurately assess its role among the other ablative therapies of hepatocellular carcinoma.

Fractional allelic loss in non-end-stage cirrhosis: correlations with hepatocellular carcinoma development during follow-up.

Hepatocellular carcinoma (HCC) is usually preceded by cirrhosis whose genetic background is still poorly understood. The aim of this study was to evaluate, in non-end-stage cirrhosis, the fractional allelic loss (FAL) at loci mostly reported to be altered in HCC and the microsatellite instability (MSI). Twenty cases of cirrhosis were retrospectively selected. Eleven had developed an HCC during the follow-up (HCC-prone group), while 9 remained HCC-free (HCC-free group). Microdissected hepatocellular cirrhotic nodules from basal liver biopsies, were studied at 20 loci (on the chromosomal arms 1p and 1q, 3p, 4q, 6q, 7q, 8p, 13q, and 18q) and with the mononucleotide repeats BAT26 and TGFbIIR. Genetic changes were detected in both groups. Overall, the FAL index was statistically increased in the HCC-prone group (0.213) as compared to the HCC-free group (0.094; P =.044). Allelic loss at chromosomal arms 1p, 4q, 13q, 18q, and concurrent losses at more than 3 loci were confined to the HCC-prone group. In both groups, MSI was never ascertained using BAT26 and TGFbIIR. In conclusion, an increased FAL index and the lack of MSI characterize the non-end-stage cirrhosis of patients undergoing HCC during follow-up. These data emphasize the role of early clonal changes in chronic liver disease, and their potential predictive significance for clinical use.

Isolated splenic metastasis from gastric carcinoma: value of CEA and CA 19-9 in early diagnosis: report of two cases.

We report two cases of an isolated splenic metastasis, occurring 5 and 3 years, respectively, after gastrectomy for gastric carcinoma. Serum carcinoembryonic antigen and CA 19-9 levels were evaluated preoperatively, postoperatively, and during the oncologic follow-up. The patients underwent splenectomy for solitary splenic metastasis. We conclude that the use of serum carcinoembryonic and CA 19-9 values may help in the early diagnosis of these recurrences and splenectomy allows radical treatment in patients with no evidence of disseminated disease.

Development of a high grade dysplastic nodule in a case of congenital hepatic fibrosis.

A 43-year-old male with a history of congenital hepatic fibrosis associated with large liver cell dysplasia developed a sizable lesion in the right lobe of the liver, which was, after a follow-up of 4 years, surgically removed on account of a suspected malignant transformation. Pathological examination showed an unencapsulated nodule with both architectural and cytological dysplastic changes, arising in a background of congenital hepatic fibrosis harbouring extranodular foci of large liver cell dysplasia. This report concerns the development of a high grade dysplastic nodule, a sizable hepatocellular lesion with suspected preneoplastic significance, in a patient with congenital hepatic fibrosis associated with large liver cell dysplasia.