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.     

Arterial invasion predicts early mortality in stage I non-small cell lung cancer

Background

A retrospective study was performed to evaluate the association between arterial invasion and survival in patients with stage I non-small cell lung cancer.

Methods

One hundred patients were identified who had undergone complete anatomic resection as definitive treatment for stage I non-small cell lung cancer. The tumors were reviewed for the presence or absence of arterial invasion. Five-year survival data were obtained for all patients.

Results

The 100 patients had an overall 5-year survival of 61%. There were 64 stage IA patients with a 62% 5-year survival and 36 stage IB patients with a 58% 5-year survival. The 39 patients identified with arterial invasion had a 38% 5-year survival compared with a 73% 5-year survival in the 61 patients without arterial invasion (p < 0.001), with an unadjusted hazard ratio of 3.5 (p < 0.001). Multivariate analysis by stage IA versus IB and by size greater or less than 2 cm demonstrated hazard ratios of 3.5 and 4.0, respectively (p < 0.001). This difference was independent of demographic characteristics, tumor type, or grade. Subgroup analysis revealed a hazard ratio of 5.8 in patients with stage IA non-small cell lung cancer (p < 0.001) and 19.8 in patients with tumors ≤ 2 cm (p = 0.006).

Conclusions

Arterial invasion is present in a substantial percentage of patients with stage I non-small cell lung cancer and is adversely associated with survival.     

Long-term survival after resection of primary adenoid cystic and squamous cell carcinoma of the trachea and carina

Background

Tracheal resection for primary carcinoma may extend survival. We evaluated survival after surgical resection or palliative therapy to identify prognostic factors.

Methods

We conducted a retrospective study of patients diagnosed with primary adenoid cystic carcinoma (ACC) or squamous cell carcinoma (SCC) of the trachea between 1962 and 2002. Laryngotracheal, tracheal, or carinal resection was performed when distant metastasis and invasion of adjacent mediastinal structures were absent and tumor length permitted. Radiotherapy was administered after operation (54 Gy), except in superficial tumors, or as palliation (60 Gy).

Results

Of 270 patients with ACC or SCC (135 each), 191 (71%) were resected. Seventy-nine were not resected due to tumor length (67%), regional extent (24%), distant metastasis (7%), or other reasons (2%). Overall operative mortality was 7.3% (14/191) and improved each decade from 21% to 3%. Tumor in airway margins was present in 40% (17/191) of resected patients (ACC 59% versus SCC 18%) and lymph node metastasis in 19.4% (37/191). Overall 5- and 10-year survival in resected ACC was 52% and 29% (unresectable 33% and 10%) and in resected SCC 39% and 18% (unresectable 7.3% and 4.9%). Multivariate analysis of long-term survival found statistically significant associations with complete resection (p < 0.05), negative airway margins (p < 0.05), and adenoid cystic histology (p < 0.001), but not with tumor length, lymph node status, or type of resection.

Conclusions

Locoregional, not distant, disease determines resectability in primary tracheal carcinoma. Resection of trachea or carina is associated with long-term survival superior to palliative therapy, particularly for patients with complete resection, negative airway margins, and ACC.     

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.     

Outcome of adrenocortical tumors in children

Purpose

This study reviews adrenocortical tumors in children to determine factors that significantly affect outcome.

Methods

An institutional review board-approved retrospective review from 1976 to 2005 identified 23 patients younger than 19 years old with histologic confirmation of adrenocortical carcinoma (ACC) and adenomas.

Results

The mean age of the 23 children was 9.0 ± 1.6 years; girls predominated (female-to-male ratio = 1.9:1) as did cancers (ACC 16, adenoma 7); tumor hormone production (74%); and advanced stage for disease (66%). All malignancies were more than 2.5 cm. Adrenalectomy, including en bloc resection of adjacent structures (35%) achieved grossly negative margins in 70% of patients. Three patients received chemotherapy or chemoradiation as primary treatment without surgery. There was no perioperative mortality; morbidity was 10% (pneumothorax, acute renal failure, chylous ascites, and thrombocytosis). Surgical cure without adjuvant therapy was achieved for all adenomas and ACC stages I and II. For ACC stage III and IV, median survival was 21 months, 5-year survival was 0%. All advanced-staged ACC received adjuvant therapy. Surgically negative margins conferred a survival advantage.

Conclusions

Children, especially females with ACC present with large advanced-staged tumors. Surgically negative margins with or without en bloc resection improves survival. The high percentage of children with functioning tumors suggests earlier detection is possible.     

Predictors of outcome in children and adolescents with rhabdomyosarcoma of the trunk--the St Jude Children's Research Hospital experience

Purpose

The aim of this study was to determine predictors of outcome in childhood truncal rhabdomyosarcoma.

Methods

Retrospective chart review evaluating the impact of demographic features, disease characteristics, and the extent and timing of surgical intervention on outcome was performed.

Results

Thirty-three patients with a median age of 8 years were identified. Most had advanced Intergroup Rhabdomyosarcoma Study group III (n = 13) or group IV (n = 9) disease. Primary site included 20 (61%) chest wall, 6 (21%) paraspinal, 5 (15%) periscapular, and 1 (3%) abdominal wall. Most tumors were embryonal (n = 21), larger than 5 cm (n = 27), and locally invasive (n = 13); 7 had positive nodes.Tumor size, nodal status, and gross total tumor resection (upfront or delayed) were significant predictors of event-free and overall survival. Tumors 5 cm or smaller were amenable to upfront surgical resection (P = .007). In patients with tumors larger than 5 cm, resection at any time was associated with a 10-year overall survival 57% ± 13% compared with 8% ± 5% in those who had no surgery (P = .003). Tumor recurrence was local in 44% of cases, and survival after local recurrence was rare (1 of 8).

Conclusion

Tumor size, nodal status, and gross total resection at any time are significant predictors of outcome in truncal rhabdomyosarcoma. Gross tumor excision should be the goal of surgical intervention in this disease.     

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.     

Successful liver transplant for unresectable hepatoblastoma

Background

Treatment of children with stage III and IV hepatoblastoma has shown little improvement with 5-year survival rates of 64% and 25%, respectively (J Clin Oncol 2000;18:2665-75). A timely and organized treatment program including preoperative chemotherapy combined with living donor liver transplantation and postoperative chemotherapy has been used seeking improved long-term survival in stage III and IV cases.

Methods

A retrospective review of 8 patients with stage III and IV hepatoblastoma unresectable by conventional resection were treated with complete hepatectomy and transplantation. Approval was obtained from our institutional review board.

Results

Since August of 2001, we have treated 6 patients with stage III hepatoblastoma and 2 patients with initial stage IV hepatoblastoma. These patients (age, 23 months-9 years) had all received extensive chemotherapy or prior resections. After chemotherapy, none had gross tumor documented outside of the liver at time of transplantation. All underwent hepatectomy including vena cava resection, in selected cases, with living donor orthotopic liver transplantation. All patients had at least 2 cycles of postoperative chemotherapy. Of 8 patients, 6 are alive and well with normalized alpha-fetoprotein levels. There were 2 late deaths from recurrent disease. Length of follow-up ranged from 7 to 53 months.

Conclusion

Complete hepatectomy with living donor liver transplantation provides optimal surgical treatment in unresectable stage III and initial stage IV disease confined to the liver at resection. This series indicates that children tolerate complete hepatectomy, transplantation, and postoperative chemotherapy well. Referral to a transplant center during the first 3 cycles of chemotherapy appears to offers the best opportunity for long-term survival.     

Loss of carcinoembryonic antigen-related cell adhesion molecule 1 expression predicts metachronous pulmonary metastasis and poor survival in patients with hepatoblastoma

Purpose

Carcinoembryonic antigen-related cell adhesion molecule 1 (CEACAM1) is a member of the carcinoembryonic antigen family of immunoglobulin-like adhesion molecules. The aim of this study was to test the hypothesis that loss of CEACAM1 expression in hepatoblastoma cells may promote hematogeneous metastasis and function as an adverse prognostic factor.

Methods

Immunohistochemical expression of CEACAM1 in surgically resected specimens from 19 patients with hepatoblastoma was examined retrospectively. The CEACAM1 expression in the epithelial area of the tumor was classified into 2 categories as follows: diffuse expression, characterized by positive staining throughout the tumor specimen, or loss of expression, in which there were distinct areas of negative staining within the tumor specimen.

Results

Of the 19 patients, 12 were classified as having tumors with diffuse expression, and 7 had loss-of-expression tumors. Survival after treatment was significantly worse in patients with tumors with loss of CEACAM1 expression (cumulative 5-year survival rate, 29%) than in patients with diffuse CEACAM1 expression (cumulative 5-year survival rate, 92%; P = .0062). Loss of CEACAM1 expression was a significant risk factor for metachronous pulmonary metastases (P = .0105).

Conclusions

Loss of CEACAM1 expression may reflect a high metastatic potential and thus indicate a poor prognosis for patients with hepatoblastoma.     

The necessity of chemotherapy in T3N0M0 colon cancer without risk factors

Background

For patients with T3N0M0 colon cancer without known risk factors, the necessity of chemotherapy has no definite consensus.

Methods

From 1994 to 2004, we retrospectively analyzed 247 patients who had pathologically confirmed T3N0M0 colon cancer without risk factors.

Results

Among the 247 patients, 198 (80.2%) received chemotherapy and 49 (19.8%) underwent surgery alone. The 5-year survival rate was 92.8% for patients with T3N0M0 colon cancer without risk factors. Only chemotherapy increased survival (P = .032) by multivariate analysis. The 5-year survival rates were 85.4% in the surgery-only group and 94.2% in the group that received surgery with chemotherapy. There was no difference in the therapeutic efficacy of 5-fluorouracil, capecitabine, and uracil/tegafur (P = .424) for survival.

Conclusion

The results of this study showed that chemotherapy improved the survival of patients with T3N0M0 colon cancer without risk factors.     

Hepatoblastoma--evolution of management and outcome and significance of histology of the resected tumor. A 31-year experience with 40 cases

Background/purpose

The aim of this study was to retrospectively evaluate and compare the clinical features, treatment strategy, pathology, and outcome of all patients with hepatoblastoma treated at an African hospital over a 31-year period (1970 to 2001).

Methods

Forty patients with hepatoblastoma were divided into 3 groups according to the treatment given. Group I (1970 to 1983, 14 patients) had no protocol therapy; group II (1984 to 1988, 6 patients) received protocol treatment according to Children’s Study Group (CCSG) guidelines; group III (1989 to 2001, 20 patients) received SIOPEL protocol therapy. All available clinical, surgical, radiologic, and pathologic data were reviewed and analyzed.

Results

Overall patient survival was as follows: group I, 14%; group II, 50%, and group III, 80%. Deaths in group II were caused by chemotherapy-induced immunosuppression only. Prognostic data for group III showed that all tumor-related deaths could be predicted by identifying multifocal disseminated growth patterns (P = .001) or vascular invasion (P = .001) in resected tumors. Of the 40 diagnostic tumor biopsies performed, 2 significant complications (1 death, 1 intraperitoneal tumor seeding) occurred. Histologic criteria evaluating these biopsies were not predictive of overall survival.

Conclusions

The introduction of protocol therapy has resulted in a marked improvement in survival. Immunosuppression-related sepsis in our setting resulted in unacceptable mortality in patients treated according to CCSG guidelines. A diagnostic biopsy in hepatoblastoma is of value but not without complications. Preoperative chemotherapy followed by complete surgical excision according to International Society of Paediatric Oncology guidelines yields excellent results with a current survival rate of 80%.     

Circumferential margin plays an independent impact on the outcome of rectal cancer patients receiving curative total mesorectal excision

Background

The aim of this study was to determine the impact of the circumferential resection margin on the outcomes of patients with rectal cancer undergoing total mesorectal excision.

Methods

Medical records from July 2004 to June 2008 were prospectively reviewed, and 348 patients who underwent potentially curative surgery for rectal cancer were identified. The influence of the circumferential resection margin on local recurrence, distant metastasis, and 5-year cancer-specific survival was assessed.

Results

Of 348 patients, 13 (3.7%) had positive circumferential resection margins. During a median follow-up period of 58.0 months, 8 patients (2.3%) had local recurrence and 53 (15.2%) developed distant metastases. Local recurrence rates and distant metastasis rates in patients with positive circumferential resection margins were 15.4% and 61.5%, respectively, significantly higher than in those with negative circumferential resection margins (1.8% and 13.4%, respectively) (P < .001). The 5-year cancer-specific survival rates were 75.8% and 0% for patients with tumors having negative and positive circumferential resection margins, respectively (P < .001).

Conclusions

A circumferential resection margin of ≤1 mm adversely affects cancer-specific survival, local recurrence, and distant metastasis.     

Defining hepatoblastoma responsiveness to induction therapy as measured by tumor volume and serum α-fetoprotein kinetics

Purpose

Hepatoblastoma is commonly unresectable at presentation, necessitating induction chemotherapy before definitive resection. To refine the paradigm for timing of resection, we questioned whether a plateau in hepatoblastoma responsiveness to neoadjuvant therapy could be detected by calculating tumor volume (TV) and serum α-fetoprotein (sAFP) kinetics.

Methods

To calculate TV and sAFP as measures of treatment responsiveness over time, infants having initially unresectable epithelial-type hepatoblastomas were identified at a single institution (1996-2008). Effects of therapy type, therapy duration, and lobe of liver involvement on TV, sAFP, margin status, and toxicity were analyzed.

Results

Of 24 infants treated for epithelial-type hepatoblastoma during this interval, 5 were resected primarily, and 15 had complete digital films for kinetics analysis. Both TV and sAFP decreased dramatically over time (P < .0001). No statistically significant difference in mean TV or sAFP was detected after chemotherapy cycle 2. Left lobe tumors had greater presenting levels of and significantly slower decay in sAFP compared with right lobe tumors (P = .005), although no statistically significant differences in TV existed between liver lobes. Resection margins did not change with therapy duration.

Conclusions

Measuring TV and sAFP kinetics accurately reflects hepatoblastoma responsiveness to induction therapy. Treatment toxicities may be reduced by earlier resection and tailoring of chemotherapeutic regimens.     

Chest-wall desmoid tumors: results of surgical intervention

Background

We are analyzing our experience with treatment of desmoid tumors of the chest wall and adjacent structures.

Methods

A retrospective review was undertaken of the records of all patients who underwent surgical management for a desmoid tumor of the chest between January 1980 and December 2001 at one institution. Fifty-three patients (24 men and 29 women) were identified, whose median age was 39 years (range 10 to 78 years).

Results

The desmoid tumor involved the chest wall exclusively in 25 patients (47%) and both the chest wall and adjacent structures in 28 (53%). Twenty-eight patients (53%) had previous resections for a desmoid tumor. Three patients also had previous radiation therapy. A wide radical resection was attempted in all 53 patients; resection was complete in 44. Seven patients had positive microscopic margins and 2 had gross residual disease. Complications were noted in 6 patients (11%); no operative deaths occurred. Median hospitalization was 6 days (range 1 to 124 days). Nineteen patients (36%) had postoperative radiation therapy (12 had complete resection and 7 had positive margins). Follow-up was complete in 51 patients (96%) and ranged from 2 weeks to 21 years (median 53 months). At the end of the review 46 patients were alive with no recurrence; 3 were alive with local recurrence and 2 died (1 from metastatic breast cancer and 1 from unknown cause). Five-year overall probability of developing a local recurrence was 37.5% (95% confidence interval, 20.2% to 53.3%). Recurrence occurred in 8 of 9 patients with positive margins (89%) and 8 of 44 with negative margins (18%). Factors adversely affecting the rate of postoperative recurrence were reoperation (p = 0.0199), positive margins (p < 0.0001), and postoperative radiation therapy (p = 0.0027). Eleven patients (22%) required reoperation at a median of 24.6 months postoperatively (range 11 to 78 months).

Conclusions

Desmoid tumors involving the chest and adjacent structures are locally aggressive tumors with a high recurrence rate. Wide radical resection should be attempted whenever possible. Positive margins at resection, reoperation and postoperative radiation are associated with a high risk of local recurrence.     

Residual tumor size and IGCCCG risk classification predict additional vascular procedures in patients with germ cell tumors and residual tumor resection: a multicenter analysis of the German Testicular Cancer Study Group

Background

Residual tumor resection (RTR) after chemotherapy in patients with advanced germ cell tumors (GCT) is an important part of the multimodal treatment. To provide a complete resection of residual tumor, additional surgical procedures are sometimes necessary. In particular, additional vascular interventions are high-risk procedures that require multidisciplinary planning and adequate resources to optimize outcome.

Objectives

The aim was to identify parameters that predict additional vascular procedures during RTR in GCT patients.

Design, setting, and participants

A retrospective analysis was performed in 402 GCT patients who underwent 414 RTRs in 9 German Testicular Cancer Study Group (GTCSG) centers. Overall, 339 of 414 RTRs were evaluable with complete perioperative data sets.

Measurements

The RTR database was queried for additional vascular procedures (inferior vena cava [IVC] interventions, aortic prosthesis) and correlated to International Germ Cell Cancer Collaborative Group (IGCCCG) classification and residual tumor volume.

Results and limitations

In 40 RTRs, major vascular procedures (23 IVC resections with or without prosthesis, 11 partial IVC resections, and 6 aortic prostheses) were performed. In univariate analysis, the necessity of IVC intervention was significantly correlated with IGCCCG (14.1% intermediate/poor vs 4.8% good; p = 0.0047) and residual tumor size (3.7% size <5 cm vs 17.9% size ≥5 cm; p < 0.0001). In multivariate analysis, IVC intervention was significantly associated with residual tumor size ≥5 cm (odds ratio [OR]: 4.61; p = 0.0007). In a predictive model combining residual tumor size and IGCCCG classification, every fifth patient (20.4%) with a residual tumor size ≥5 cm and intermediate or poor prognosis needed an IVC intervention during RTR. The need for an aortic prosthesis showed no correlation to either IGCCCG (p = 0.1811) or tumor size (p = 0.0651).

Conclusions

The necessity for IVC intervention during RTR is correlated to residual tumor size and initial IGCCCG classification. Patients with high-volume residual tumors and intermediate or poor risk features must initially be identified as high-risk patients for vascular procedures and therefore should be referred to specialized surgical centers with the ad hoc possibility of vascular interventions.     

Determinants of Tc-99m sestamibi SPECT scan sensitivity in primary hyperparathyroidism

Background

The aim of this study was to evaluate the influence of patient and adenoma characteristics on ^99mTc-methoxy isobutyl isonitrile (MIBI) scan performance in individuals diagnosed with primary hyperparathyroidism (PHP).

Methods

Records of patients undergoing parathyroidectomy for PHP over 6 years at a single center were reviewed.

Results

The overall true-positive (TP) rate for ^99mTc-MIBI scans was 56%. Adenomas sized 1.9 to 3.5 cm were more likely to have TP scans than 0.3-cm to 1.8-cm adenomas (74% vs 40%, P < .001). Preoperative ionized calcium levels between 1.49 and 1.72 mmol/L were more likely to have TPs than levels between 1.27 and 1.48 mmol/L (65% vs 47%, P < .05). No single class of medication was shown to significantly effect TP rates. A decrease in TP rate was observed for larger adenomas in patients on ≥1 medication (74% vs 65%, P = .05).

Conclusions

In PHP, ^99mTc-MIBI scan positivity is most related to adenoma size and preoperative ionized calcium level.     

Rectal cancer patients benefit from implementation of a dedicated colorectal cancer center in a Veterans affairs medical center

Background

A dedicated colorectal cancer (CRC) center was created in a Veterans Affairs Medical Center with the intent of improving quality of patient care and multidisciplinary cooperation.

Methods

Retrospective and prospective databases before and after creation of the CRC center, respectively, were created. Patients entered in each database included those requiring surgical intervention for CRC treatment. Statistical analyses included Fisher's exact, chi-square, and unpaired Student t tests as well as analysis of variance.

Results

The overall quality of care of CRC patients has improved as evidenced by a larger percentage of complete, margin-negative resections (P <.05) as well as an increase in the number of lymph nodes excised at surgery (P <.0001). Furthermore, a multidisciplinary approach is clearly beneficial as evidenced by the increased number of CRC patients receiving appropriate multidisciplinary therapy (P <.0001).

Conclusions

A dedicated CRC center has significantly improved quality of care for CRC patients.     

Factors affecting postoperative course and survival after en bloc resection for esophageal carcinoma

Background

To identify factors affecting postoperative course and survival after esophagectomy for cancer and reasons for improved survival over time.

Methods

Complete esophageal resection was attempted for middle and lower third esophageal carcinomas in 386 consecutive patients between January 1982 and January 2002. Two study periods were analyzed: 1982 to 1993 and 1994 to 2002. Prognostic factors were identified by multivariate analysis and the two periods compared.

Results

Hospital mortality rate decreased from 5.4% to 2.9% (p = 0.245). Both anastomotic leakage and pulmonary complications rates decreased from 9.8% to 2.2% (p = 0.001) and 24.1% to 19.3% (p = 0.295), respectively. An increased proportion of patients had R0 resection in the latter period, 78.5% versus 67.0%, (p = 0.028). Five-year survival rate after R0 resection increased from 29% to 46% (p = 0.001), with a decreased recurrence rate from 65.8% to 44.3% (p = 0.002). Three favorable prognostic factors were identified: low pT stage, pN0 stage, and operation during the 1994 to 2002 study period.

Conclusions

Short-term outcome and survival of patients with resected esophageal cancer have improved over time. Advances in perioperative technique, staging methods, and surgical management combined with higher patient selection and use of neoadjuvant chemoradiation may be responsible for this progress.     

The experience of a regional pediatric intestinal failure program: Successful outcomes from intestinal rehabilitation

Background

The aim of this study was to evaluate the clinical experience of a regional multidisciplinary intestinal failure program for children established in 2005.

Methods

Data were collected from a prospective internal database. Univariate analyses were performed to compare pre- and post-treatment outcomes. Median values are reported.

Results

Forty-nine children were referred at an age of 7 months. Remnant small bowel length was 29 cm. With follow-up of 14 months, overall patient survival was 88%. Thirteen bowel-lengthening procedures were performed, thereby increasing small bowel length from 83 to 132 cm (P < .05). Enteral autonomy was achieved in 22 patients (45%), and the caloric requirement for parenteral nutrition was decreased from 100% to 41% (P < .01). Conjugated bilirubin was reduced from 4.1 to 0 mg/dL (P < .05).

Conclusion

A multidisciplinary approach to pediatric intestinal failure that prioritizes intestinal rehabilitation can achieve successful enteral feeding advancement, improved liver function, and excellent survival in intermediate-range follow-up.     

The impact of gross total resection on local control and survival in high-risk neuroblastoma

Background/purpose

Gross total resection of the primary tumor in treatment of high-risk neuroblastoma remains controversial. Furthermore, there are few reports of the effect of primary tumor resection on local control as opposed to overall survival. The authors reviewed their institutional experience to assess the effect of primary tumor resection on local control and overall survival.

Methods

A total of 141 patients were treated on protocol between November 1, 1979 and June 25, 2002 and are the subject of this report. Gross total resection was assessed by review of operative notes, postoperative computerized axial tomograms, and postoperative meta-iodobenzyl guanidine (MIBG)¹ scans when available.

Results

The median age was 3.3 years, and all patients were International Neuroblastoma Staging System (INSS) stage 4 with 79% having metastases to cortical bone. The primary site was the adrenal gland in 74%, the central abdominal compartment in 13%, the posterior mediastinum in 7%, and other sites in 6%. Gross total resection was accomplished in 103 (73%) but was more than 90% for the last 3 protocols. Five kidneys were lost overall. The probability of local progression was 50% in unresected patients compared with 10% in patients undergoing gross total resection (P < .01). Overall survival rate in resected patients was 50% compared with 11% in unresected patients (P < .01).

Conclusions

Our data indicate that local control and overall survival rate are correlated with gross total resection of the primary tumor in high-risk neuroblastoma. Gross total resection should be part of the management of stage 4 neuroblastoma in patients greater than 1 year of age.