Surgical resection of pulmonary metastatic lesions in children with hepatoblastoma

Background

Although the most common site of metastasis for hepatoblastoma is the lung, the role of thoracotomy and surgical resection of pulmonary metastasis remains uncertain. In this study, we aimed to determine the long-term outcome of patients with hepatoblastoma who have a surgical resection of pulmonary metastatic lesions.

Methods

Patients with hepatoblastoma in the Children's Oncology Group INT-0098 were reviewed. Detailed reports enabling comprehensive review were available for 175 of 181 eligible patients. Prognostic factors (histology, tumor margin, surgical complications, α-fetoprotein) were also reviewed.

Results

Thoracotomy for initial pulmonary metastasis: 38 patients presented with pulmonary metastasis, Children's Oncology Group stage IV. Nine of these 38 underwent thoracotomy and pulmonary metastectomy either before (2), simultaneous (5), or after (2) resection of their primary liver tumor. Eight of these 9 patients with metastectomy were long-term survivors. Thoracotomy for tumor relapse: 20 patients who had previously achieved complete tumor clearance experienced subsequent pulmonary relapse of their tumor (11 stage I or III, 9 stage IV). All 20 patients with pulmonary relapse had salvage chemotherapy; 13 also had thoracotomy and pulmonary metastectomy (8) or thoracotomy and biopsy (5). Only 4 of 13 were long-term survivors: 2 were stage I and 2 were stage IV.

Conclusions

As pediatric surgeons, we varied tremendously in our timing and surgical approach to the management of pulmonary metastasis in hepatoblastoma. This large multicenter review suggests that thoracotomy should be used cautiously in the management of pulmonary relapse and perhaps more aggressively in the management of metastases present at diagnosis that persist after neoadjuvant chemotherapy.     

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.     

Outcome of central hepatectomy for hepatoblastomas

Background/Purpose

Central hepatoblastomas (CHBL) involving liver segments (IV + V) or (IV + V + VIII) are in contact with the portal bifurcation. Their resection may be achieved by central hepatectomy (CH) with thin resection margins on both sides of the liver pedicle, by extended right or left hepatectomy with thin resection margins on one side, or by liver transplantation with thick free margins. The aim of this study is to assess the operative and postoperative outcome of CH for hepatoblastoma.

Methods

This was a retrospective monocentric study of 9 patients who underwent CH for CHBL between 1996 and 2008.

Results

The operative time was 4 hours 50 minutes (2 hours 20 minutes to 7 hours), vascular clamping lasted 30 minutes (0-90 minutes), and the amount of blood cell transfusion was 250 mL (0-1800 mL). Two patients had biliary leakage requiring percutaneous drainage. Median follow-up time was 27 months (14-120 months). All of 8 nonmetastatic patients are alive and disease-free; 1 metastatic patient died of recurrent metastases at last follow-up. Although 3 of 9 patients had surgical margins less than 1 mm, none, including the patients who died from metastases, had local recurrence.

Conclusions

Our study demonstrates the feasibility of CH for CHBL without operative mortality or local recurrence. Central hepatectomy is an alternative to extensive liver resections in selected patients.     

Survival after liver transplantation for hepatoblastoma: a 2-center experience

Purpose

Complete resection with adjuvant chemotherapy is the accepted treatment for hepatoblastoma. The aim of this study is to evaluate our results of liver transplantation (LT) for tumors still unresectable after adequate chemotherapy.

Methods

All patients transplanted for hepatoblastoma from 2 institutions between 1990 and 2004 were included. Variables reviewed to determine impact on survival included the following: previous tumor resection, metastatic disease at diagnosis, microscopic vascular invasion, α-fetoprotein (AFP) levels at diagnosis and at transplant, tumor histology, and administration of posttransplantation chemotherapy. Effectiveness of pretransplantation chemotherapy was defined as a drop of more than 99% in peak AFP levels.

Results

Fourteen patients were transplanted: 9 boys and 5 girls (age range, 18 months-13 years; mean age, 57 ± 48 months). Patients were transplanted a mean of 4 ± 1 months after diagnosis. Overall survival was 71% (10/14) with a mean follow-up of 46 months. All deaths were secondary to recurrent tumor. Of 10 patients who underwent a primary LT, 9 survived compared to only 1 of 4 transplanted for unresectable tumor recurrence after primary resection (90% vs 25%; P = .02). Decline in peak AFP of more than 99% was also associated with better survival (100% vs 56%; P = .08). Similarly, patients who received posttransplantation chemotherapy had 100% survival compared with 56% without chemotherapy (P = .08). Other variables had little effect on survival.

Conclusions

Liver transplantation is a successful treatment option for children with unresectable hepatoblastoma with a 90% survival rate for primary transplantation. Rescue LT for recurrent hepatoblastoma after previous resection has a poor survival outcome and should be considered a relative contraindication. Posttransplantation chemotherapy improves survival. A prospective multicenter collaboration to validate these findings with a larger patient population is necessary. Until that time, patients who receive rescue transplants should receive posttransplantation chemotherapy.     

Effect of postoperative chemotherapy on the serum alpha-fetoprotein level in hepatoblastoma

Purpose

It is generally accepted that postoperative chemotherapy does not affect the serum alpha-fetoprotein (AFP) level. The authors report on 3 patients who supposedly showed chemotherapy-related changes in their AFP levels after operation.

Methods

This study included 3 patients with hepatoblastoma (1 case of PRETEXT III and 2 cases of PRETEXT IV).

Results

One patient with PRETEXT III underwent a complete tumor resection, and the postoperative AFP level decreased until it reached the normal range. However, he consistently exhibited a transient, 2- to 3-fold increase in the AFP after each course of chemotherapy for 3 courses. The chemotherapy regimen had to be stopped because of drug-induced encephalopathy, but he has been followed up for 5 years without any evidence of recurrence, and his AFP level has also remained stable and in the normal range. Two patients with PRETEXT IV, who underwent a curative tumor resection, also showed similar chemotherapy-related changes in AFP levels. Both of these cases were observed only after the administration of routine postoperative chemotherapy instead of administering further high-dose chemotherapy. The AFP level remained stable for 17 months and 7 months after the cessation of chemotherapy in 2 cases, respectively.

Conclusions

Regarding the postoperative chemotherapy of hepatoblastoma, we have to pay close attention to both the AFP status during chemotherapy as well as the absolute AFP level.     

Living-donor hepatectomy

Background

Organ transplantation from deceased donors is still far below the need. Because of this deficiency, liver transplantations are performed mostly from live donors in many transplant centers in our country. Living-donor liver transplantation (LDLT) has evolved dramatically over the past decade. The aim of this study was to present our clinical experience with living-donor hepatectomy.

Methods

We retrospectively analyzed all patients who underwent donor hepatectomy between March 2000 and September 2010. We reviewed demographic data, operation type, operation and cold ischemia times, duration of hospital stay, and postoperative complications.

Results

During the study period, 140 living donors underwent operations for liver transplantation. We performed 108 right hepatectomies, 17 left hepatectomies, and 15 left lateral hepatectomies. The mean age of the donors was 30.8 years. There was no operative or postoperative mortality. Overall morbidity rate was 13.57% (n = 19). Nine patients had biliary leakages, 4 biliomas; 2 urinary tract infections, and 1 each inferior vena caval injury, pneumonia, portal vein thrombosis, and acute tubular necrosis. Reoperation was not required in any of these patients.

Conclusions

Living-donor liver transplantation is a valuable alternative for patients awaiting a cadaver organ. Live-donor hepatectomy can be performed with low morbidity. The greatest disadvantage of this procedure is the risk of the surgical operation for the individual who will experience no medical benefit from this procedure.     

Surgical treatment of hepatic tumors in children: lessons learned from liver transplantation

Purpose

Hepatectomy remains a complex operation even in experienced hands. The objective of the present study was to describe our experience in liver resections, in the light of liver transplantation, emphasizing the indications for surgery, surgical techniques, complications, and results.

Methods

The medical records of 53 children who underwent liver resection for primary or metastatic hepatic tumors were reviewed. Ultrasonography, computed tomographic (CT) scan, and needle biopsy were the initial methods used to diagnose malignant tumors. After neoadjuvant chemotherapy, tumor resectability was evaluated by another CT scan. Surgery was performed by surgeons competent in liver transplantation. As in liver living donor operation, vascular anomalies were investigated. The main arterial anomalies found were the right hepatic artery emerging from the superior mesenteric artery and left hepatic artery from left gastric artery. Hilar structures were dissected very close to liver parenchyma. The hepatic artery and portal vein were dissected and ligated near their entrance to the liver parenchyma to avoid damaging the hilar vessels of the other lobe. During dissection of the suprahepatic veins, the venous infusion was decreased to reduce central venous pressure and potential bleeding from hepatic veins and the vena cava.

Results

Fifty-three children with hepatic tumors underwent surgical treatment, 47 patients underwent liver resections, and in 6 cases, liver transplantation was performed because the tumor was considered unresectable. There were 31 cases of hepatoblastoma, with a 9.6% mortality rate. Ten children presented with other malignant tumors—3 undifferentiated sarcomas, 2 hepatocellular carcinomas, 2 fibrolamellar hepatocellular carcinomas, a rhabdomyosarcoma, an immature ovarian teratoma, and a single neuroblastoma. These cases had a 50% mortality rate. Six children had benign tumors—4 mesenchymal hamartoma, 1 focal nodular hyperplasia, and a mucinous cystadenoma. All of these children had a favorable outcome. Hepatic resections included 22 right lobectomies, 9 right trisegmentectomies, 8 left lobectomies, 5 left trisegmentectomies, 2 left segmentectomies, and 1 case of monosegment (segment IV) resection. The overall mortality rate was 14.9%, and all deaths were related to recurrence of malignant disease. The mortality rate of hepatoblastoma patients was less than other malignant tumors (P = .04).

Conclusion

The resection of hepatic tumors in children requires expertise in pediatric surgical practice, and many lessons learned from liver transplantation can be applied to hepatectomies. The present series showed no mortality directly related to the surgery and a low complication rate.     

Improved survival outcome for hepatoblastoma based on an optimal chemotherapeutic regimen--a report from the study group for pediatric solid malignant tumors in the Kyushu area

Background/purpose

The survival outcome for patients with hepatoblastoma normally depends on the resectability of the tumor. In Japan, the pre and/or postoperative chemotherapy protocol using a combination of cisplatin (CDDP) and tetrahydropyranyl-Adriamycin (THP-ADR) has been the standard treatment since 1991. This study aims to assess exactly what influence the establishment of this chemotherapy protocol has had on both the tumor resectability and the outcome of patients with hepatoblastoma.

Methods

From 1982 to 1997, 60 patients with hepatoblatoma were treated in the Kyushu area, Japan. Based on the pretreatment extent of disease (PRETEXT), the outcome and tumor resectability were compared between group A (1982 to 1990, n = 27, PRETEXT I:5, II:8, III:6, IV:8) and group B (1991 to 1997, n = 33, PRETEXT I:9, II:9, III:5, IV:10).

Results

The 5-year survival rates (group A and group B) were 33% and 73% for all cases (P < .01), 100% and 89% for PRETEXT I, 38% and 89% for II (P < .05), 17% and 80% for III (P < .01), and 0% and 40% for IV (P < .01), respectively. The 5-year survival rates for patients with metastases were 0% for group A (n = 5) and 57% for group B (n = 7; P < .01). The rates of a complete resection of primary tumor were 48% for group A and 67% for group B. In particular, a significant difference was found regarding the complete resection rate between groups A and B in the patients with PRETEXT III (17% for group A and 80% for group B; P < .01). In the patients with an incomplete tumor resection (14 for group A, 11 for group B), the 5-year survival rates were 0% for group A and 45% for group B (P < .01).

Conclusions

The optimal chemotherapeutic regimen of CDDP and THP-ADR was thus found to greatly contribute to the improved survival rate of hepatoblastoma patients. Preoperative chemotherapy resulted in an increased resectability of the tumor, whereas postoperative chemotherapy played an important role in the increased cure rate of cases with either an incomplete tumor resection or metastasis. However, refractory cases with PRETEXT IV or metastasis may still require the development of an even more effective treatment modality, including the use of blood stem cell transplantation.     

Hepatocellular Carcinoma in Kidney Transplant Recipients

Objective

A kidney transplant is a suitable surgical management for end-stage renal disease patients; however, posttransplantation malignancy is an unwanted outcome. In Taiwan, hepatocellular carcinoma (HCC) is a major malignancy not only among the general population but also in the post-kidney transplant group. Therefore, regular imaging studies for posttransplantation follow-up are necessary. We examined the imaging characteristics and the efficacy of radiologic diagnostic criteria and the American Joint Committee on Cancer (AJCC) staging system in post-kidney transplantation HCC.

Patients and Methods

We retrospectively reviewed 15 patients with post-transplantation HCC among 554 hospital-based kidney transplant recipients. From 1988 to 2008 we analyzed the patient profiles, imaging studies, histopathologic diagnosis, treatment methods, and outcomes. The 6th-edition AJCC radiologic staging system was applied for validation in this study.

Results

Using the AJCC staging system, all 15 patients with histopathologically confirmed HCC were enrolled as stage I (n = 7), stage II (n = 2), stage IIIA (n = 5), or stage IV (n = 1) cases. The 5-year survival rates were 71.4% in stage I, 50% in stage II, 20% in stage IIIA, and 0% in stage IV. Over one-half of post-kidney transplantation HCC were sized 2.5-6.0 cm in diameter with mixed echogenicity. The positive diagnostic rate for radiologic criteria was 83.3%.

Conclusions

The AJCC staging system and the radiologic diagnostic criteria were validated in post-kidney transplantation HCC. Surgical resection and transcatheter arterial embolization for early-stage HCC in kidney transplant recipients showed satisfactory outcomes. A noncirrhotic liver in a kidney transplant recipient makes surgical resection the treatment of choice because of the better prognosis.     

SERUM CYFRA 21–1 IN PATIENTS WITH INVASIVE BLADDER CANCER AND ITS RELEVANCE AS A TUMOR MARKER DURING CHEMOTHERAPY

Purpose:

Previous studies have shown that serum levels of the degradation products of cytokeratins could be used as surrogate markers in the diagnosis and followup of patients with solid tumors, including tumors of the bladder.

Materials and Methods:

The soluble cytokeratin 19 fragment CYFRA 21-1 was measured by solid phase radioimmunoassay in the serum of 142 patients with invasive transitional cell cancer of the bladder. Of the patients 56 had clinical stage I to III locally confined disease (T1-4aN0M0) and 86 had stage IV metastatic disease with lymph node and/or distant metastases. A control group consisted of 33 healthy volunteers. In a subgroup of 49 patients with metastatic disease receiving combined platinum based chemotherapy serum CYFRA 21-1 was determined prior to the initiation of therapy and after the documentation of response.

Results:

Abnormal CYFRA 21-1 was observed in 7% of patients with locally invasive disease and in 66% of those with metastatic disease (p <0.0001). There was no correlation of CYFRA 21-1 with tumor differentiation. Patients with abnormal CYFRA 21-1 showed statistically significant worse median overall survival. Moreover, in the subgroup of patients with metastatic disease receiving chemotherapy CYFRA 21-1 levels correlated with the response to treatment.

Conclusions:

Patients with transitional cell cancer of the bladder with evidence of distant metastases showed a significant increase in serum CYFRA 21-1. During chemotherapy CYFRA 21-1 appears to be a potentially sensitive and useful indicator for monitoring treatment response.     

Outcomes of primary liver cancer in children: an appraisal of experience

Introduction

Hepatoblastoma (HB) and hepatocellular carcinoma (HCC) are the most common primary liver cancers in children. Recent advances in management of pediatric liver cancer have improved disease-specific survival (DSS). This is a review of our experience with childhood liver malignancy over the past 3 decades.

Materials and Methods

A retrospective chart review from 1975 to 2005 identified patients who were 18 years old or younger with a histologically confirmed diagnosis of primary liver cancer. Patients were staged according to the Children's Cancer Group and Pediatric Oncology Group (CCG/POG) system. Patients were followed up prospectively through clinic visits and mail correspondence. Standard statistical methods were used for comparison, risk, and survival analyses.

Results

Fifty-two patients were confirmed to have primary liver cancers, where 24 (46%) patients had HB, 22 (42%) had HCC, 3 (6%) had sarcomas, and 3 (6%) had other histologies. Mean ages at presentation for HB and HCC were 3.2 and 13.1 years old, respectively. The most common presentations were abdominal mass (67%) and pain (40%). Most patients underwent major liver resection (n = 45, 87%), including: lobectomy (n = 25, 48%), and trisegmentectomy (n = 11, 21%). Three patients underwent liver transplantation (n = 3, 6%) for advanced local disease. Forty-five (87%) received primary or neoadjuvant and/or adjuvant chemotherapy. Patients had the following CCG/POG stages: I (n = 31, 60%), II (n = 6, 11.5%), III (n = 9, 17%), and IV (n = 6, 11.5%). Complete gross resection (stage I and II) was achieved in 37 (71%) patients. The perioperative mortality and morbidity rates were 0% and 29%, respectively. Patients with complete resection had significantly better 5-year DSS and median survival compared with incomplete gross resection: 62% vs 9% and 216 vs 18 months, P < .001. Patients treated during the period 1995-2005 had better 5-year DSS and median survival compared with those treated during 1975-1994: 68% vs 32% and 117 vs 27 months, P = .032. All 3 patients who underwent transplantation for conventionally unresectable disease are alive without disease recurrence (follow-up period, 1-15 years).

Conclusion

Complete resection of the pediatric primary liver tumors remains the cornerstone of treatment to achieve cure. Major liver resection can be performed with minimal perioperative mortality and morbidity. Patients with HB appeared to have better survival compared with patients with HCC, and there was significant improvement in the DSS of children treated in the recent decade. Liver transplantation in conjunction with chemotherapy may have an increasing role in the management of locally advanced primary liver cancers.     

Donor quality of life after living donor liver transplantation: single-institute experience

Aim

Living donor liver transplantation (LDLT) has been widely accepted because of the severe shortage of hepatic grafts. However, the healthy donor is exposed to risks of morbidity and mortality. In this study, we analyzed medical, functional, and psychological outcomes of donors after hepatectomy for liver donation.

Patients and methods

Among 41 donor hepatectomy cases for LDLT performed in our institute from January 1994 to May 2011, we reviewed the medical records (liver function tests, complications, etc) of 27 subjects who donated to recipients older than 12 years. We also performed a questionnaire survey based on the Japanese Short Form-36 version 2 Health Survey scales as a measure of physical and mental health, to which 31 subjects responded.

Results

Six of the 27 donors experienced prolonged jaundice. Their ratios of graft volume/standard donor liver volume (GV/SDLV) were higher than those of the 21 donors without prolonged jaundice (60.0% vs 41.5%). According to the questionnaires, social functioning among those having undergone emergency hepatectomy as well as general health perceptions declined in those with postoperative complications. Physical component summary declined among those having undergone emergency hepatectomy and with postoperative complications.

Conclusion

In liver donation from a living donor, massive hepatectomy should be avoided. A ratio of GV/SDLV around 50% seems reasonable. Donors with emergency transplantations or postoperative complications must be more carefully followed after donor hepatectomy.     

Association between surgical margins and long-term outcome in advanced hepatoblastoma

Background

Many patients with hepatoblastoma present with unresectable disease. Neoadjuvant therapy has improved resectability rates to as high as 70% to 90%. Despite this improvement, many patients will be left with tumors that are of borderline resectability. The authors hypothesize that favorable outcomes may be achieved even with resection margins less than 1 cm thus sparing the need for liver transplantation.

Methods

Between January 1981 and March 2003, 23 patients age less than 16 years with a diagnosis of hepatoblastoma undergoing surgical resection were identified. The clinical characteristics, pathologic resection margins, and survival status were reviewed.

Results

Eighteen (78%) of the patients were alive with no evidence of recurrence at last follow-up. Thirteen (56.5%) had ≥1 cm resection margins, whereas 10 (43.5%) had resection margins less than 1 cm. Eleven (47.8%) presented with PRETEXT III tumors. There was no significant difference in survival rate between resection margins less than 1 cm and ≥1 cm (P = .13; 95% CI 0.91 to 2.61). Thirteen patients (56.5%) presented with synchronous pulmonary metastatic disease, where survival was significantly worse (P = .04; 95% CI 1.10 to 2.50). Subgroup analysis confirmed that margins less than 1 cm did not significantly affect survival after controlling for pulmonary metastatic disease (P = .56; 95% CI 0.71 to 3.61).

Conclusions

Surgical resection with margins less than 1 cm are associated with survival that is equivalent to resection with margins ≥1 cm. Our findings suggest it is preferable to preserve key structures with a small resection margin and therefore spare the need for liver transplantation in patients with advanced hepatoblastoma.     

Liver Regeneration in 120 Consecutive Living-Related Liver Donors

Background

Living-related liver transplantation for pediatric patients has become an acceptable, low-risk treatment option. The aim of this study was to assess the extent of donor liver regeneration.

Materials and Methods

Between October 1999 and January 2008, 120 living-related donors provided 109 grafts consisting of segments II and III and 11 grafts consisting of segments II, III, and IV. Volumetric assessment of the donor liver and selected segments was performed using computed tomography. After procurement every graft was weighed. At 7 and 30 days, as well as 12 months after the operation the donor liver remnant was evaluated for differences in volume.

Results

A significant correlation was observed between the liver graft mass and its volume as assessed by computed tomography (r = 0.781; P < .05). Twelve months after procurement, the average regeneration index was significantly higher among donors of segments II, III, and IV (144 ± 23%) versus donors of segments II and III (114 ± 15%; P < .05).

Conclusion

Liver regeneration after procurement of selected liver segments from living donors is a consistent finding. Computed tomography is an accurate imaging modality to track changes in liver volume. This study showed a positive correlation between the size of the liver graft and the regeneration of the liver remnant in the donor.     

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.     

Postoperative biochemical liver function after major hepatic resection in children

Background/Purpose

There are no detailed reports of the profile of biochemical liver function tests (LFTs) after partial hepatectomy in children. The study aims to establish normal profiles of standard LFTs after major liver resection in noncirrhotic children; the effects of preoperative chemotherapy were also analyzed.

Methods

Clinical and biochemical data were collected from a consecutive series of children who had undergone a primary major liver resection for a hepatic tumor. Chemotherapy details were recorded. Children who had more than 4 liver segments resected were compared with those undergoing lesser resections. Those with and without preoperative chemotherapy were also compared.

Results

A total of 22 children underwent major liver resection at a median age of 24 months (range, 2 weeks to 16 years). Fifteen received preoperative chemotherapy. Peak derangements in all standard LFTs occurred on day 1 to day 2 postoperatively. Normal plasma levels of bilirubin and albumin were present by day 5, international normalized ratio and alkaline phosphatase by day 7, and alanine aminotransferase by 1 to 2 weeks. Peak alanine aminotransferase and international normalized ratio values tended to be higher in children having more extensive liver resections. Preoperative chemotherapy given up to 3 weeks before surgery had no major effect on LFT recovery profiles. Hypophosphatemia was maximal on day 2.

Conclusions

Postoperative LFTs showed a more rapid resolution than typically seen after partial hepatectomy in adults. Preoperative chemotherapy had no major effects on postresection LFT profiles.     

Incidental appendectomy in donors undergoing hepatectomy for living-donor liver transplantation

Background

The aim of this study was to investigate the morbidity associated with appendectomy in living liver donors undergoing hepatectomy.

Methods

The medical records of 338 donors who underwent hepatectomies for living-donor liver transplantation between 2008 and 2010 were reviewed retrospectively. The patients were divided into 2 groups on the basis of appendectomy: patients in group A (n = 126) received incidental appendectomies in conjunction with donor hepatectomy, and those in group B (n = 212) underwent hepatectomy alone.

Results

No significant difference in age, gender, or body mass index was found between groups. The wound infection rate (P = .037) and length of hospital stay (P = .0038) were higher in group A than in group B. Intraoperative findings in 126 donors in group A were subserosal (n = 4), retrocecal (n = 6), or hard nodular (n = 11) appendix; hyperemic appendix with edema (n = 9); appendix length ≥8 cm (n = 18); and palpable fecalith (n = 78). Histopathologic examination of appendix specimens revealed lymphoid hyperplasia with a fecalith (n = 32), fecalith only (n = 32), acute appendicitis (n = 20), normal anatomy (n = 18), fibrous obliteration (n = 9), lymphoid hyperplasia (n = 9), Enterobius vermicularis (n = 3), appendiceal neuroma (n = 1), carcinoid tumor (n = 1), and mucoceles (n = 1).

Conclusion

Although incidental appendectomy increased the wound infection rate and length of hospital stay, this procedure is necessary for the prevention of potential complications due to appendicitis when the exploration of the ileocecal region in patients undergoing donor hepatectomy reveals one or more of the following: appendix length ≥8 cm; dropsical, hyperemic, subserosal, nodular, and/or retrocecal appendix; and/or palpable fecaloma.     

Using the Clavien Grading System to Classify the Complications of Right Hepatectomy in Living Donors

Introduction

The ratios of complications for living related liver donors after right hepatectomy differ widely among numerous single institutions. This study sought to use the Clavien classification system to define and graded the severity of these complications.

Materials and methods

This study retrospectively analyzed the outcomes of 160 consecutive living donor right hepatectomies performed between July 2002 and February 2008. Complications among living donors for liver transplantation after right hepatectomy were stratified according to the Clavien classification of postoperative surgical complications.

Results

Fifty-two living donors displayed one or more perioperative complications Grade 1 complications were recorded in 18.1%; grade 2 in 6.3%; grade 3a in 5%; and grade 3b in 3.1%. Biliary complications were the most frequent. No donor mortality was present in this series.

Conclusions

The Clavien grading system is useful to comparise surgical outcomes. This study demonstrated that donor right hepatectomy was a relatively safe procedure, but reducing donor complications after right hepatectomy has to be the first priority during the entire process of living related transplantation.     

Aggressive surgical therapy and radiotherapy for patients with high-risk neuroblastoma treated with rapid sequence tandem transplant

Background/Purpose

The treatment approach for patients with high-risk neuroblastoma has been one of dose intensification chemotherapy and aggressive treatment of the primary tumor. Local tumor control is examined in high-risk patients treated with tandem stem cell transplant, aggressive surgery, and selected radiation therapy (XRT).

Methods

Seventy-six patients with high-risk stage III/IV neuroblastoma were treated on a standard protocol incorporating aggressive surgical resection with or without local XRT followed by tandem high-dose chemotherapy and stem cell rescue. Patients were evaluated for degree of surgical resection, site of progression, and outcome.

Results

Overall event-free survival for the series is 56%. Forty-eight had gross total resection, 12 had greater than 90% resection, 10 had 50% to 90% resection, and 6 had biopsy only or no surgery. Surgical complications occurred in 29% with no deaths. There were no isolated local failures. Two patients had local recurrence after gross total resection. Surgeon assessment of completeness of resection agreed with postoperative radiological findings 66% of the time.

Conclusion

Aggressive surgical treatment with local XRT and myeloablative chemotherapy with stem cell rescue provides excellent local control in high-risk neuroblastoma, although distant failures, particularly osseous, remain a problem. Poor correlation exists between the surgeon's perception of completeness of resection and findings on postoperative imaging studies.     

Survival after laser surgery for twin-to-twin transfusion syndrome: when are they out of the woods?

Purpose

Severe, progressive twin-to-twin transfusion syndrome (TTTS) is associated with near-100% mortality if left untreated. Endoscopic laser ablation of placental vessels (ELA) is associated with 75% to 80% survival of at least one twin. The actuarial risk of fetal demise after ELA has not yet been described.

Study Design

A retrospective cohort study from 2 centers on a consecutive series of 163 sets of monochorionic twins with severe TTTS (18 Quintero stage I, 55 stage II, 71 stage III, 19 stage IV) who underwent ELA. Actuarial survival was calculated and stratified for donor vs recipient and according to stage.

Results

Median gestational age at diagnosis was 20.1 weeks; median operative time was 60 minutes. Overall survival was 63%, and survival of at least one twin was seen in 76% of pregnancies. Of fetal demises, 10% occurred within 48 hours after ELA, and 90% of all fetal demises occurred within 1 month. There was a 10% survival advantage of recipients over donors. Survival was similar for stages I, II, and IV (75%-80%), compared with 55% for stage III.

Conclusions

Actuarial survival curves for TTTS confirms a greater burden on donor than on recipient but not at a previously reported 2:1 ratio. The current staging system does not accurately reflect post-ELA mortality risk. The unexpected higher mortality in stage III may reflect a more acute progression of the disorder in this group, an adverse effect of LA on an as yet unknown subgroup with stage III or, alternatively, preoperative demise of fulminant stage IV patients, leaving a stage IV subgroup with a more benign course and better outcome.