Typical carcinoids and neuroendocrine carcinomas of the stomach: differing clinical courses and prognoses

Background

Gastric endocrine tumors are usually classified as 3 types of well-differentiated endocrine tumors (typical carcinoids or carcinoids) and poorly differentiated carcinomas (neuroendocrine carcinomas [NECs]).

Methods

From 1993 to 2008, 97 patients (73 men and 24 women) were diagnosed with gastric neuroendocrine tumors at the Asan Medical Center.

Results

Of the 45 patients with typical carcinoids, 37 underwent surgery (eg, endoscopic resection). Of the 52 patients with NECs, 43 underwent surgery (eg, radical gastrectomy). One patient died of recurrence of the typical carcinoids, whereas 26 patients with NECs died of related diseases (P < .05). The rates of survival and recurrence did not significantly differ by type of typical carcinoid (P > .05).

Conclusions

Regardless of the type, carcinoids that are not yet advanced can be effectively treated with minimal endoscopic or laparoscopic surgery. However, all NECs and advanced carcinoids should be treated with radical gastrectomy.     

Bilateral Wilms' tumors with progressive or nonresponsive disease

Background

To provide guidelines for future cooperative group trials, we reviewed the outcomes of children with bilateral Wilms' tumors (BWTs) treated on National Wilms Tumor Study-4 (NWTS-4) who had progressive or nonresponsive disease (PNRD).

Methods

NWTS-4 enrolled 3335 patients from August 1986 to September 1994 including 188 patients with BWT (5.6%). Treatment and outcome data were collected on patients with BWT. Treatment guidelines were outlined in the protocol, but patients were not on study.

Results

Thirty-eight children with BWT had PNRD. Preoperative chemotherapy was given for a median of 7 months (range, 2-29 months) before definitive resection. After the initial chemotherapy regimen, 36 children went on to a second regimen, and of these, 21 children received a third regimen before resection. Eleven patients received irradiation to one or both kidneys. Pathology at resection revealed previously undiagnosed anaplasia in 3 patients (2 diffuse and 1 focal) treated for 14, 15, and 15 months before resection. A fourth patient developed a diffusely anaplastic tumor 13 months after therapy. Other pathological findings included rhabdomyomatous (4 patients) or differentiated stromal elements (10 patients) and complete necrosis (1 patient). Ten kidneys from 7 patients lacked biopsy at presentation or pathology review of those specimens.

Conclusions

BWT patients with PNRD received prolonged courses of chemotherapy. Early and sequential biopsies to establish the reason for failure to respond should be obtained. This will identify anaplastic tumors managed best by early nephrectomy and intensive chemotherapy and will also distinguish differentiated tumors that are best managed with early resection, but less intensive therapy after nephrectomy.     

Donor/recipient algorithm for management of the middle hepatic vein in right graft live donor liver transplantation

Background

The aim of this study was to delineate an algorithm for donor and recipient criteria and middle hepatic vein (MHV) management in right-graft live-donor liver transplantation (LDLT) on the basis of computerized 3-dimensional computed tomographic image analysis.

Methods

Data on 94 consecutive right-graft LDLTs were prospectively collected. Graft and remnant data for the first 23 cases were retrospectively evaluated by means of 3-dimensional computed tomographic reconstructions, and on the basis of that preliminary series, a graft selection algorithm using 3 parameters—hepatic vein dominance classification, graft and remnant graft volume/body weight ratios, and congestion volumes—was created. It was subsequently applied to the next 71 right-graft LDLTs.

Results

Fifty-nine right grafts contained the MHV. Four of the 12 grafts with no MHVs required MHV reconstructions. In 18 cases, small liver grafts were used. The postoperative function of liver grafts and remnants with versus without MHVs was not statistically different.

Conclusions

The proposed algorithm favored the inclusion of the MHV with the right grafts. It also allowed for the procurement of grafts that were potentially small for size without compromising donor or recipient safety.     

Urinary diversion in children with pelvic tumors

Purpose

The aim of this study was to investigate children presenting with malignant pelvic tumors obstructing the upper urinary tract.

Methods

Seventeen children with upper urinary tract obstruction by a malignant tumor were reviewed. A nephrostomy tube or Double J (DJ) stent was inserted into each obstructed urinary system and removed after tumor shrinkage and/or hydronephrosis regression.

Results

There were 9 boys and 8 girls in the study; the mean age and median follow-up were 5.7 years and 2.5 years, respectively. The most common obstructing tumor was rhabdomyosarcoma. Twelve children underwent diversion by nephrostomy tubes and 3 by DJ stents; 2 patients underwent resection of the tumors with ureteroureterostomy. Complications after the insertion of the stents included febrile urinary tract infections (UTI) or pyelonephritis in 4 of the children with DJ stents. In the nephrostomy group, febrile UTI developed in 3 and the tube fell out in 1, and was blocked in another. Of the 17 children, 9 have no evidence of disease, 2 are currently under treatment, and 6 died of cancer.

Conclusions

The prognosis of children with malignant pelvic tumor obstructing the upper urinary system justifies urgent and optimal upper tract diversion, enabling chemotherapy to be started immediately.     

Influence of maternal parity, age, and ethnicity on risk of esophageal atresia in the infant in a population-based study

Background

The etiology of esophageal atresia (EA) is virtually unknown. We hypothesized that the maternal factors low parity, high age, and white ethnicity are involved.

Methods

A Swedish nationwide, population-based, case-control study was nested in a cohort of newborn children in 1982 to 2004. Among 2,305,858 deliveries, 722 cases of EA and 3610 controls were included. Conditional logistic regression was used to calculate odds ratios (ORs) and 95% confidence intervals (CIs). Matching, stratification, and multivariable regression were used to adjust for potential confounding.

Results

A more than 30% decreased risk of EA was found for mothers delivering their second (OR, 0.68; 95% CI, 0.56-0.83) or third child (OR, 0.64; 95% CI, 0.49-0.83) compared to primiparous mothers. Children of women giving birth when 35 to 40 years and older than 40 years showed a 2-fold (OR, 2.09; 95% CI, 1.09-3.99) and 3-fold (OR, 3.04; 95% CI, 1.37-6.74) increased risk of EA, respectively, compared to those of mothers younger than 20 years. This association remained when chromosomal abnormality cases were excluded (P = .004). There was a 66% increase in risk of isolated EA in children of mothers of white (OR, 1.66; 95% CI, 1.06-2.61), compared to mothers who are not of white ethnicity.

Conclusions

This study indicates an increased risk of EA in children of mothers having their first delivery, of older age, and of white ethnicity.     

Three-dimensional visualization and virtual simulation of resections in pediatric solid tumors

Purpose

Three-dimensional visualization of solid tumors is possible because of high-resolution computed tomography and magnetic resonance imaging scans. However, additional preoperative information is often desirable in complex malignancies. For the first time, the authors present a model of preoperative 3-dimensional visualization and virtual resections in pediatric solid tumors.

Methods

Image analysis of various pediatric tumors was performed using the research software HepaVision2 (MeVis, Bremen). Organs, tumors, and the vascular system were extracted from multislice computed tomography scans. After hierarchical analysis of the vascular system, territories supplied or drained by the major vascular branches were calculated. Results were explored and virtual resections of organs were carried out using the research software InterventionPlanner (MeVis, Bremen). Data were correlated to intraoperative findings.

Results

Four hepatic malignancies, 4 renal tumors, and 3 other neoplasms were analyzed. The technique of 3-dimensional visualization was feasible for all investigated children (mean age 5 years and 9 months). Spatial relations between physiological and pathological structures were identified, and anatomical structures (vessels, tumor tissue, and organ parenchyma) were determined using colorimetric encoding. Virtual simulations of tumor resection were used successfully for planning of surgical procedures in the hepatic and renal tumors.

Conclusions

The technique of 3-dimensional tumor visualization and virtual simulation of tumor resections provides the basis for a successful planning of complex tumor resections in children. The efficiency of these techniques should be further analyzed in series with higher numbers and differentiations of tumors.     

Primary hepatic sarcomas in children--a single-center experience over 19 years

Background/ Purpose

Primary hepatic sarcomas are rare and account for about 13% of primary hepatic neoplasms. There are few reported series of pediatric hepatic sarcomas, and the aim was to review our experience.

Methods

A retrospective analysis of cases managed from 1988 to 2007 by the pediatric liver unit in Birmingham, UK, was conducted.

Results

Nineteen children were identified. These presented with sudden abdominal pain (n = 6), obstructive jaundice (n = 3), incidental mass (n = 3), and chronic pain/distension (n = 3). Vascular involvement was identified in 3, and 6 had pulmonary metastases. Three patients had primary resection, and 3 only a biopsy. Thirteen had a biopsy followed by chemotherapy and resection. Surgery included extended hepatectomy (n = 11), hepatectomy (n = 3), and nonanatomical resections (n = 2). There was 1 major intraoperative complication. Median inpatient stay was 7 days. One biliary leak developed 4 weeks postoperatively. Five of the 16 patients who underwent resection of the primary tumor died. Eleven were alive at a median follow-up of 3 years.

Conclusion

This is a challenging group of patients. Local control remains pivotal to successful treatment. Good results can be achieved in a specialist center with multidisciplinary approach.     

Differences in Early Postoperative Complications in Elective and Emergency Heart Transplantation

Introduction and Objectives

The immediate postoperative period is a critical phase in heart transplantation. Severe complications occur that may influence short-term and medium-term morbidity and mortality in these patients. The aim of this study was to analyze the incidence of severe complications in emergency and nonemergency transplantations.

Materials and Methods

We studied 152 patients who underwent heart transplantation between 2001 and 2007. Combined transplantations and retransplantations were excluded. Two groups were considered: emergency transplantations (36 patients, 24%) and elective transplantations. We compared survival and occurrence of infection, primary graft failure (PGF), renal and hepatic failure, respiratory complications, cardiac tamponade, arrhythmias, reoperation, and intensive care unit (ICU) stay.

Results

The emergency transplantation group had a greater number of ischemic patients, with a more prolonged cardiopulmonary bypass time, and a larger proportion of donors were women. Overall mortality in the intensive care unit was 2.6%, with no differences between groups. However, emergency procedures were significantly associated with a higher incidence of PGF, need for intraaortic balloon pump, and a more prolonged mechanical ventilation time, as well as a greater number of bacterial infections and a significantly longer ICU stay.

Conclusions

In our series, emergency transplantation showed no greater perioperative mortality. We observed a greater number of severe complications, such as PGF, bacterial infection, and more prolonged mechanical ventilation time.     

Tumours in End-Stage Kidney

Objective

Patients with end-stage kidney disease (ESKD) show a greater risk for renal cell carcinoma (RCC), which tends to be multifocal and bilateral. The malignant potential is unclear. The question is whether to remove both kidneys in patients with a tumor on one side only diagnosed by computed tomography (CT).

Materials and Methods

Kidney tumors were found in 14 patients with ESKD from January 2002 to December 2006. One was unfit for surgery. Thirteen patients underwent nephrectomy and 6 a bilateral procedure of whom only 2 had bilateral tumors on CT, 3 multiple tumors on the contralateral side, and 1 uncontrollable hypertension with tumors as an incidental finding. Tumors were found in all 19 specimens.

Results

In 13 kidneys (68.4%), the tumors were multiple; in 6 (31.6%), solitary. The types of tumor were: 13 (68.4%) papillary RCCs (PRCC), 9 (47.4%) clear RCCs (CRCC), a combination of PRCC and CRCC in 4 (21.0%), and myxoid liposarcoma (with solitary PRCC contralaterally). The mean follow-up was short (19 ± 15 months; maximum, 54 months). Only 1 patient died due to a tumor at 16 months after operation.

Conclusions

There is a high risk for bilateral involvement. Patients who undergo unilateral nephrectomy must be regularly followed and contralateral nephrectomy carefully considered, mainly in transplanted patients on immunosuppression. Further studies are needed to give a definitive answer about the indications for surgery and the indications for contralateral nephrectomy as well. To date, prophylactic contralateral nephrectomy should not be a therapeutic standard.     

Radionuclide imaging study of long-term pulmonary function after lobectomy in children with congenital cystic lung disease

Purpose

We evaluated the long-term pulmonary function after lobectomy for congenital cystic lung disease, in both infants and children, using radionuclide imaging (RI).

Methods

We performed a retrospective review of 93 patients who underwent resection of cystic lung lesions between 1974 and 2001. The results of postoperative lung volume/perfusion scintigraphy at 1 (n = 64), 5 (n = 32), and 10 years (n = 18) after surgery (V1, 5, 10/Q1, 5, 10) and mean transit time (MTT—a marker for air-trapping) at 1, 5, and 10 years after surgery (MTT1, 5, 10) were compared with respect to age at operation, preoperative infection, underlying disease, and type of surgery.

Results

Patients who were younger than 1 year at the time of surgery showed a significantly lower MTT5 (1.09 ± 0.08) and MTT10 (1.15 ± 0.11) than patients who were older than 1 year at the time of surgery (MTT5, 1.49 ± 0.67; MTT10, 1.54 ± 0.33). The noninfected group had significantly higher Q10 and lower MTT10 values (P < .05) compared to the infected group. No significant differences were observed between patients with single lobe vs multiple lobe resection.

Conclusions

The optimal age for surgery in patients with congenital cystic lung disease appears to be less than 1 year.     

Distractions and surgical proficiency: an educational perspective

Background

Surgery training requires residents to focus on tasks while minimizing the effect of distractions. There is a need to develop training methodologies that can enable surgical residents to hone this ability.

Methods

Fourteen surgical residents were divided into 2 groups. They were trained to perform simulated tasks in a noiseless environment and subsequently performed these tasks in a distractive one. In a follow-up experiment, an experimental group was trained in noisy and distractive conditions and was compared with a control group trained in noiseless conditions.

Results

Residents who trained in noiseless environments possessed decreased surgical proficiency when performing the identical tasks in realistic environments (P < .05). Pretraining in a noisy environment improves surgical proficiency.

Conclusions

Noise and distractions can significantly impede performance of surgical residents, but this effect can be nullified by introduction of noise and distractions in the training environment.     

The extent of lower extremity occlusive disease predicts short- and long-term patency following endovascular infrainguinal arterial intervention

Background

Endovascular revascularization of the femoral-politeal arterial segment has gained acceptance despite lower patency than surgical bypass due to lower morbidity. Choosing patients that are ideal candidates for endovascular therapy remains controversial. We have assessed hemodynamic factors that might predict longer primary patency after endovascular therapy.

Methods

Ninety-nine limbs were treated with endovascular therapy from January 2001 to January 2005 with a mean and median follow-up of 338 and 293 days. Primary patency was considered lost when recurrent symptoms developed, ankle-brachial index (ABI) decreased following initial improvement, or a subsequent procedure was required. Kaplan-Meier analysis was used to evaluate patency.

Results

Patients with an ABI ≥.5 prior to intervention had longer primary patency compared to those with an ABI less than .5 (P = .043). Having 1 or more patent tibial runoff vessels was associated with improved patency for the first 24 months post-procedure (P = .001).

Conclusions

Patients with an ABI ≥.5 or at least 1 patent tibial vessel runoff have significantly higher hemodynamic and clinical success following endovascular therapy of the femoral-popliteal arterial segment.     

Hyperglycemia during hepatic resection: continuous monitoring of blood glucose concentration

Background and aims

This study assessed glucose metabolism via continuous intraoperative monitoring of blood glucose in patients undergoing hepatic resection.

Methods

Thirty patients who underwent hepatic resection were enrolled. During hepatic resection, blood glucose concentration was continuously measured by an artificial pancreas.

Results

Glucose concentrations followed a similar up-and-down pattern in all patients during the Pringle maneuver series. The concentration decreased marginally during the first clamping of the hepatoduodenal ligament but showed a rapid increase after unclamping. However, this increase declined with the number of Pringle cycles (P < .01). Patients with liver cirrhosis showed smaller elevations in glucose concentration after the first unclamping compared with patients without liver cirrhosis (P < .05).

Conclusions

The present study showed a rapid and profound transition in glucose concentration during hepatic resection. The mechanism underlying the transition of blood glucose concentration may involve glycogen break down within hepatocytes because of hypoxia.     

Is congenital lobar emphysema a surgical disease?

Background

The management of congenital lobar emphysema has traditionally been surgical. Because of increased use of imaging, this lesion is frequently found in asymptomatic and mildly symptomatic children, prompting us to adopt a more conservative approach to these children.

Methods

All children with congenital lobar emphysema presenting between 1995 and 2002 were included. Medical records, imaging files, and pathology reports were reviewed.

Results

Twenty children (0-17 years) were identified. Eight were diagnosed antenatally. Fourteen were managed without surgery. Of the 11 symptomatic children, 6 showed spontaneous improvement.

Conclusions

The favorable outcome of both asymptomatic and mildly symptomatic children suggests that a nonoperative approach should be considered in these patients.     

Cure of multifocal panhepatic hepatoblastoma: is liver transplantation always necessary?

Purpose

Multifocal panhepatic hepatoblastoma (HB) without extrahepatic disease is generally considered as an indication for total hepatectomy and liver transplantation. However, after initial chemotherapy, downstaging of the tumor sometimes allows complete macroscopic resection by partial hepatectomy. This procedure is no longer recommended because of the risk of persistent viable tumor cells in the hepatic remnant. We report our experience with conservative surgery in such cases.

Method

Between 2000 and 2005, 4 children were consecutively referred to our unit with multinodular pan-hepatic HBs (classification PRETEXT IV of the International Society of Pediatric Oncology Liver Tumor Study Group SIOPEL). Three of them had extrahepatic disease at diagnosis. All patients were treated according to SIOPEL 3 and 4 protocols.

Results

Extrahepatic metastases were still viable in 2 of 3 patients after initial chemotherapy. These patients eventually died of tumor recurrence. In the 2 patients without residual extrahepatic disease, liver tumors had regressed, and complete macroscopic excision of hepatic tumor remnants could be achieved by conservative surgery. These 2 children are alive and well and free of tumor 7 years after diagnosis.

Conclusions

Conservative surgery may be curative in some multinodular PRETEXT IV HB patients, with a good response to preoperative chemotherapy and complete excision of all macroscopic tumor remnants. However, because of the lack of reliable predictors of sterilization of the microscopic disease in the residual liver, with subsequent poor prognosis, total hepatectomy and liver transplantation remain currently recommended in patients with multinodular PRETEXT IV HB without extrahepatic disease, even though some of these children are probably overtreated.     

Acquired intestinal aganglionosis after a lytic infection with varicella-zoster virus

Background and Purpose

In this report, we present the first case of an immunologically impaired child surviving a lytic varicella-zoster virus infection affecting the enteric nervous system. In histological findings, myenteric and submucous enteric ganglia were nearly completely absent owing to virus infection.

Methods

A 3-year-old girl with acute lymphoblastic leukemia and generalized varicella-zoster infection developed an ileus. She underwent multiple laparotomies in which histological sections of the entire small intestine could be obtained.

Results

The histological evaluation of these samples showed a generalized aganglionosis with inflammatory residuals. A more detailed immunohistochemical analysis using neuronal (PGP, enolase), glial (S100), and lymphocytic (LCA) antibodies demonstrated a nearly complete neuronal loss.

Conclusion

To our knowledge, this is the first case of a secondary intestinal aganglionosis after varicella-zoster virus infection.     

Cancellations in ambulatory day surgery: Ten years observational study

Background

Reasons for cancellation of booked procedures in ambulatory surgery need a detailed analysis in order to introduce corrective measures to lessen them.

Methods

Cancellations occurring the day before operation without patient replacement and procedures cancelled on the day of operation in 10 500 patients scheduled to be operated on in a multidisciplinary ambulatory surgery unit were analysed. Data were obtained from the incident register sheets and the database of the unit.

Results

A total of 424 patients were cancelled (4%). Reasons for cancellation were: acute medical conditions in 23.3% of cases, personal decision of the patient to refuse programming in 22.2%, non-attendance in 2.1%, failure to follow pre-operative guidance in 23.3% and unavailability of resources in 29%. These causes were preventable or possibly preventable in 57.1% of cases, difficult to prevent in 29% and not preventable in 13.9%.

Conclusion

More than half the cases of cancellation could be prevented. A rapid response of surgical departments to substitute patients, campaigns to increase the awareness of the population about the cost of health services and the implementation of pre-operative assessment guidelines must be considered.     

Management of the primary tumor in patients with metastatic pancreatic neuroendocrine tumor: a contemporary single-institution review

Background

Pancreatic nonfunctioning neuroendocrine tumors (PNFNETs) are an uncommon malignancy and often present with metastatic disease. There is a lack of information on the management of the primary tumor in patients who present with unresectable synchronous hepatic metastases.

Methods

A retrospective review (2001-2008) of PNFNETs was conducted. Patients were divided into 3 groups: PNFNET without evidence of hepatic metastasis (group A), PNFNET with metastatic disease involving less than 50% of the liver (group B), and PNFNET with metastatic disease involving more than 50% of the liver (group C). Clinical data and outcomes were analyzed.

Results

Thirty-five patients with PNFNET were identified (group A = 15, group B = 11, group C = 9). Resection of the pancreatic tumor was performed in 26 patients. With a mean follow-up period of 30 months, death from disease progression occurred in 1 patient in group A, none in group B, and in 7 in group C.

Conclusions

In selected patients, resection of the primary pancreatic tumor even in the setting of unresectable but limited hepatic metastases may be indicated.     

Pilomatrixoma--accuracy of clinical diagnosis

Background/Purpose

Pilomatrixoma is a common tumor of skin appendages in children. The aim of the study was to assess the accuracy of clinical diagnosis and factors contributing to misdiagnosis.

Methods

A retrospective case note review of patients who had pilomatrixoma excised during a 5-year period in a tertiary referral children's hospital in the UK.

Results

From 75 patients, 78 pilomatrixomata were excised. The diagnosis was achieved preoperatively in 46% of patients. Other diagnoses included sebaceous and dermoid cysts, foreign body reaction, calcification in lymph gland, and fat necrosis.

Conclusion

Factors contributing to misdiagnosis include cystic lesions with varying consistency, punctum-like appearance, atypical location, and absence of clinically recognizable calcification. Despite close excision, the recurrence rate is low.