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.     

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.     

Evaluation of total oxidative and antioxidative status in pediatric patients undergoing laparoscopic surgery

Objective

Reactive oxygen species (ROS) induced by several diseases can trigger oxidative stress. During laparoscopy, increased intraabdominal pressure caused by pneumoperitoneum may lead to splanchnic ischemia followed by reperfusion because of deflation. Because ischemia reperfusion creates oxidative stress, in this study, we aimed to investigate the oxidative-antioxidative status of the pediatric patients with laparoscopic surgery.

Methods

The children underwent laparoscopic procedures under general anesthesia, and they were mechanically ventilated. Blood samples were obtained after induction of anesthesia, at the end of the surgery, and were centrifuged at 3000 revolutions per minute for 10 minutes to separate plasma. The plasma total antioxidant status (TAS), total oxidant status (TOS), and oxidative stress index (OSI) were determined.

Results

Plasma TOS and OSI levels were significantly higher at the end of the surgery than after induction of anesthesia (P < .05 and P < .01, respectively). On the other hand, plasma TAS levels were lower in the end of the surgery than after induction of anesthesia (P < .05).

Conclusions

These results suggest that ROS are generated during the laparoscopic procedure, possibly as a result of an ischemia-reperfusion phenomenon induced by the inflation and deflation of the pneumoperitoneum, which causes oxidative stress and consume plasma antioxidants.     

Combined resection of superior vena cava for lung carcinoma: prognostic significance of patterns of superior vena cava invasion

Background

Combined resection of the superior vena cava (SVC) for lung carcinoma remains challenging in terms of technical aspect and prognosis. We attempted to clarify the surgical outcome of combined resection and reconstruction of the SVC for lung carcinoma.

Methods

Between March 1980 and May 2001, among 3,499 lung resections, 40 (1.1%) patients underwent combined resection of the SVC. Thirty-four were men and 6 were women. Ages ranged from 37 to 77 years, with median of 64 years. Lobectomy and pneumonectomy was performed in 19 and 21 patients, respectively. The SVC system was totally resected and reconstructed with grafts in 11 patients, and partially resected in 29 patients. For the latter patients, autologous pericardial patches were used in 8 patients, and a running direct suture was performed in 21 patients. The survival curves were constructed by the method of Kaplan-Meier, and the curves were compared using the log-rank test.

Results

Thirty-day mortality was 10%. The 5-year survival rate was 24%, with the median follow-up period for living patients 67 months (actual 5-year survivors were 7). The prognoses were compared between patients with SVC invasion by metastatic nodes (n = 15) and those with SVC invasion by a direct tumor extension (n = 25), and the survival difference was statistically significant (5-year survival rate, 6.6% versus 36%; p = 0.05).

Conclusions

The pattern of SVC invasion was considered to be a significant prognostic factor, and this factor should be taken into consideration for evaluating the outcome of clinical trials for T4 lung cancer.     

Clinicopathologic characteristics and prognosis of synchronous multifocal gastric carcinomas

Background

We sought to define differences between multifocal and solitary gastric carcinoma to decrease the risk of missing a cancer while resecting another more evident carcinoma.

Methods

We retrospectively examined clinicopathologic characteristics of multifocal gastric carcinoma including anatomic distribution and postoperative survival.

Results

Multifocal gastric carcinoma was seen more frequently when patients were older and when the largest tumor was small and at an early stage. More than half of accessory lesions were located near the main tumor. No significant difference in postoperative survival was seen between patients with multifocal and solitary carcinoma, whether early or advanced.

Conclusions

The entire stomach should be examined carefully before and during resection, especially when local or endoscopic surgery is performed.     

Long-term survival after aggressive resection of pulmonary metastases among children and adolescents with osteosarcoma

Purpose

Although survival without resection of pulmonary metastases from osteosarcoma is unlikely, not all surgeons agree on an aggressive surgical approach. We have taken an approach to attempt surgical resection if at all feasible regardless of number of metastases and disease-free interval (DFI). This study presents information on long-term follow-up after this aggressive approach to resection.

Methods

A single-institution retrospective cohort study of osteosarcoma patients younger than 21 years with pulmonary metastases, limited to the contemporary chemotherapeutic period (1980-2000), was conducted.

Results

In 137 patients, synchronous (23.4%) or metachronous (76.6%) pulmonary nodules were identified. The median follow-up was 2.0 years (5 days to 20.1 years) for all patients. Overall survival among patients who had pulmonary nodules was 40.2% and 22.6% at 3 and 5 years, respectively. Ninety-nine patients underwent attempted pulmonary metastasectomy (mean survival, 33.6 months; 95% confidence interval, 25.1-42.1) and 38 patients did not (mean survival, 10.1 months; 95% confidence interval, 6.5-13.6; P < .001, t test). Characteristics that were associated with an increased likelihood of 5-year overall survival after pulmonary resection were primary tumor necrosis greater than 98% after neoadjuvant chemotherapy (P < .05) and DFI before developing lung metastases more than 1 year (P < .001). No statistically significant difference in overall survival or disease-free survival was found based on the number of pulmonary metastases resected. Characteristics including primary tumor size, site, or extension; chemotherapy; early vs late metastases; unilateral vs bilateral metastases; and resection margins did not significantly affect survival.

Conclusions

Most patient and tumor characteristics commonly used by surgeons to determine utility of resection of pulmonary metastases among patients with osteosarcoma are not associated with outcome. Biology of the particular tumor (response to preoperative chemotherapy, measured by tumor necrosis percentage, and DFI), as opposed to tumor burden, appears to influence survival more significantly. We would advocate considering repeat pulmonary resection for patients with recurrent metastases from osteosarcoma.     

Surgical management of thoracic malignancies invading the heart or great vessels

Background

Surgical resection of thoracic malignancies involving either the heart or great vessels is uncommonly performed because of the potential morbidity and mortality for an unknown probability of significant palliation or cure. We reviewed our experience of 10 patients treated surgically, either primarily or as a component of multimodality therapy, to assess feasibility and results.

Methods

A retrospective review of the results in 10 patients who underwent resection of thoracic malignancies that included either great vessel or the heart was conducted.

Results

Histologic diagnoses included soft tissue sarcoma (n = 7), squamous cell carcinoma (n = 1), malignant thymoma (n = 1), and mediastinal teratoma (n = 1). Three patients underwent induction chemotherapy. Cardiopulmonary bypass was used in 7 patients. Structures resected included superior vena cava (n = 5), left atrium (n = 4), right atrium (n = 2), descending aorta (n = 1), and main pulmonary artery (n = 1). Concomitant anatomic pulmonary resections were performed in 3 patients. Seven patients had an R0 or R1 resection. There were no perioperative deaths. All symptomatic patients had immediate and sustained palliation of their presenting symptoms. The median length of stay was 6 days (range, 4 to 43 days). Six patients underwent postoperative systemic therapy. The overall median survival was 21.7 months (range, 3.2 to 69 months) and was 33.3 months (range, 3.7 to 69 months) for patients who had an R0 or R1 resection.

Conclusions

Resection of the heart and great vessels involved by thoracic malignancies can be performed with acceptable morbidity and mortality and results in significant palliation and, in some cases, prolonged survival.     

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.     

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.     

An injectable tissue-engineered embolus prevents luminal recanalization after vascular sclerotherapy

Background/Purpose

Sclerotherapy for vascular malformations is often limited by luminal recanalization. This study examined whether an injectable tissue-engineered construct could prevent this complication in a rabbit model of venous sclerotherapy.

Methods

Ethanol sclerotherapy of a temporarily occluded jugular vein segment was performed in 46 rabbits, which were then divided into 3 groups. Group I (n = 16) had no further manipulations. In groups II (n = 15) and III (n = 15), 0.5 mL collagen hydrogel was injected intraluminally, respectively, devoid of and seeded with autologous fibroblasts. At 1, 4, and 20 to 24 weeks postoperatively, vein segments were examined for patency and resected for histological evaluation. Statistical analysis was by Fisher's Exact test.

Results

All vein segments were occluded at 1 and 4 weeks in all groups, despite histological evidence of progressive endothelial ingrowth. However, at 20 to 24 weeks, angiography demonstrated restoration of vessel patency in groups I (3/6) and II (3/5), but not in group III (0/6; P = .043), in which histology confirmed an obliterated lumen for all vessels.

Conclusion

An injectable, fibroblast-based, engineered construct prevents midterm to long-term recanalization in a leporine model of vascular sclerotherapy. This novel therapeutic approach may prevent recurrence of vascular malformations after sclerotherapy, thus reducing the need for repeated procedures and morbid operative resections.     

Bronchioloalveolar carcinoma of the lung 3 centimeters or less in diameter: a prognostic assessment

Background

Bronchioloalveolar carcinoma (BAC) of the lung is a subtype of adenocarcinoma for which the incidence is actually rising, but the histologic definition of BAC has been recently changed by the revised World Health Organization (WHO) classification in 1999. The clinical features of patients with BAC diagnosed according to the recently revised WHO classification have not yet been clarified. In this retrospective study, we investigated the pattern of recurrence and survival outcome for patients with resected BAC by pathology review, compared with those in patients who had adenocarcinoma other than BAC.

Methods

From 1985 through 2002, 108 patients underwent surgical resection for pulmonary adenocarcinoma 3 cm or less in diameter at the University of Yamanashi, Japan. All of the resected specimens of these 108 patients were pathologically reviewed again to confirm the diagnosis as BAC or adenocarcinoma other than BAC. The tumor was defined as BAC when the adenocarcinoma lesion had a pure bronchioloalveolar growth pattern and no evidence of stromal, vascular, or pleural invasion according to the WHO classification (third edition).

Results

Twenty-five patients (23%) had a diagnosis of BAC, and 83 (77%) had a diagnosis of other adenocarcinoma. There was a female predominance among both patients with BAC and those with other adenocarcinoma. Lymph node involvement was seen for 30 lesions (36%) of adenocarcinoma other than BAC, but not for any BAC lesions. The median duration of follow-up after surgery was 5.1 years. There was no recurrence in the postoperative course in patients with BAC for a 5-year disease-free survival rate of 100%, whereas the 5-year disease-free survival rate for other adenocarcinoma was 63.5%.

Conclusions

The patients with resected BAC, which is defined as a noninvasive adenocarcinoma by the revised WHO classification, had an excellent prognosis. However, these results may depend on a strictly accurate pathology diagnosis as BAC. Limited resection might be curative in patients with focal BAC based on evidence of pathologic noninvasive features.     

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.     

Preoperative hematologic markers as independent predictors of prognosis in resected pancreatic ductal adenocarcinoma: neutrophil-lymphocyte versus platelet-lymphocyte ratio

Background

The objective of this study was to investigate whether the preoperative hematologic markers, the platelet-lymphocyte ratio (PLR), or the neutrophil-lymphocyte ratio (NLR) ratio are significant prognostic indicators in resected pancreatic ductal adenocarcinoma.

Methods

A total of 84 patients undergoing pancreatoduodenectomy for pancreatic ductal adenocarcinoma over a 10-year period were identified from a retrospectively maintained database.

Results

The preoperative NLR was found to be a significant prognostic marker (P = .023), whereas PLR had no significant relationship with survival (P = .642) using univariate Cox survival analysis. The median overall survival in patients with an NLR of ≤3.0 (n = 55) was 13.7, 17.0 months in those with an NLR of 3.0 to 4.0 (n = 17) and 5.9 months in patients with a value of >4.0 (n = 12) (log rank, P = .016). The NLR retained its significance on multivariate analysis (P = .039) along with resection margin status (P = .001).

Conclusion

The preoperative NLR represents a significant independent prognostic indicator in patients with resected pancreatic ductal adenocarcinoma, whereas PLR does not.     

Oncologic outcomes after resection of rectal cancer: Laparoscopic versus open approach

Background

This study examined outcomes of laparoscopic and open rectal cancer surgery in a community hospital setting.

Methods

A community health care system cancer registry was reviewed retrospectively (2004-2007) for rectal cancer patients undergoing surgical treatment. Primary end points were rates of recurrence and survival.

Results

Both open and laparoscopic resection groups had similar demographic, treatment, and tumor characteristics. Most patients in the open resection and laparoscopic resection populations experienced no recurrence (79% vs 83%, respectively; P = .5). Overall, the groups had similar mean (88% vs 96%, respectively; P = .4) and disease-free (21 and 23 months, respectively; P = .5) survival.

Conclusions

In a community hospital setting, laparoscopic resection of rectal cancer was found to be as safe and effective as open resection in selected patients.     

Study of the factors associated with recurrence in children with sacrococcygeal teratoma

Purpose

The aim of this study was to explore effects of (1) histological involvement of resection margins with microscopic residue, (2) incomplete removal of coccyx, and (3) tumor spillage on recurrence and on survival in children operated upon for sacrococcygeal teratoma (SCT).

Methods

Retrospective review of 70 patients treated between 1960 and 2003.

Results

Fifty-four girls and 16 boys presented with SCT diagnosed prenatally (12), at birth (37), or later (21). Thirty-six percent of tumors were Altman type I, 27% type II, 18% type III, and 18% type IV. Histologically, mature teratoma was observed in 48 patients, immature teratoma in 11, yolk sac tumor (YST) in 9, embryonal carcinoma in one, and mixed tumor in one. Eighty-four percent of patients solely underwent surgical extirpation. Six (8.5%) patients died. However, mortality for the group of 42 patients treated during the past 15 years was as low as 2.5%. Tumor recurrence was observed in 5 patients, 2 of whom died. Of 3 patients with initially mature teratoma, 1 showed local immature recurrence and 2 malignant recurrences. One of the latter died. Of 2 patients with initially immature teratoma grade I, one relapsed with a benign lesion and one with YST leading to death. Possible eliciting factors had been demonstrated in 3 patients. Histological analysis of resection margins showed tumoral involvement in 11 patients (and also in one patient after resection of a recurrent tumor). Only one of those with YST focus in the resection margin showed recurrence. Intraoperative tumor spillage presented in 2 patients, who both died of metastatic disease. Spillage of tumoral cyst fluid occurred in 6, none developed recurrence. One of 5 patients whose coccyx had not been removed died of metastatic disease. One with immature teratoma developed a benign recurrent tumor. The other 3 showed no recurrence.

Conclusions

Microscopic involvement of the resection margins of mature or immature SCT is rarely associated with recurrence, provided there are no YST foci in the resection margins. A conservative attitude then appears to be justified. Spillage of cyst fluid was never associated with recurrence, unlike spillage of tumor and absence of removal of coccyx.     

The ratio between metastatic and examined lymph nodes is an independent prognostic factor for patients with resectable middle and distal bile duct carcinoma

Background

The lymph node ratio, defined as the ratio between the number of lymph node metastasis and the total number of lymph nodes examined, has been reported to be an important prognostic factor in other gastrointestinal carcinomas except middle and distal bile duct carcinomas.

Methods

Between 1991 and 2004, 62 consecutive patients who underwent surgery for middle and distal bile duct carcinoma were retrospectively analyzed concerning prognostic factors.

Results

The median number of lymph nodes examined was 12 (range 5 to 38). The overall 5-year survival rates of patients with lymph node ratio of 0, lymph node ratio of 0 to .2, and lymph node ratio >.2 were 62%, 41%, and 0%, respectively. A multivariate analysis revealed that a lymph node ratio >.2 and perineural invasion were independent predictive factors for survival.

Conclusions

Lymph node ratio >.2 is an important factor to predict survival after resected middle and distal bile duct carcinoma.     

Resection of pulmonary metastases in pediatric patients with Ewing sarcoma improves survival

Background

Ewing sarcoma (ES) is the second most common bone tumor in children, and survival of those with metastatic ES has not improved. Previous studies have shown a survival benefit to whole lung irradiation in patients with pulmonary metastases and may be given either before, after, or instead of surgical pulmonary metastasectomy (PM). The contribution of surgery compared with irradiation in ES has not previously been studied.

Methods

A retrospective review of patients younger than 21 years (median age, 16 years) treated at a single institution (1990-2006) was performed. Kaplan-Meier survival curves were compared using log-rank test and a multivariate Cox proportional hazards model. P ≤ .05 was regarded as significant.

Results

Eighty patients with ES were identified. Of these, 31 (39%) had pulmonary metastases. Nine patients had incomplete details of their full treatment regimen, but the following groups could be defined from the remainder: resection alone (n = 5), radiation alone (n = 3), radiation and resection (n = 3), or chemotherapy alone (n = 11). There were 24 deaths overall, with a median overall survival (OS) of 2.7 (95% confidence interval [CI], 1.7-5.2) years. Patients who had PM had the best OS (80%), whereas those who underwent radiation to the lung without PM compared with chemotherapy only for pulmonary metastasis both had similar OS of 0% at 5 years (P = .002). Patients who had radiation followed by PM for lung metastasis had a 5-year OS of 65%. Patients with PM had a longer OS compared with those without lung resection (P < .0001).

Conclusion

These data suggest a possible benefit for ES patients who undergo surgical resection of lung metastases.     

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.     

The use of free flap for limb salvage in children with tumors of the extremities

Background

The purpose of this study was to evaluate long-term functional outcomes in pediatric oncology patients who underwent limb salvage using free flaps.

Methods

All 22 pediatric oncology patients treated with a free flap for extremity salvage were included in the study from 1999 to 2008.

Results

The median patient age was 13.5 years. All but one patient had sarcoma, which involved lower extremity in 45% and upper extremity in 55%. The median bone defect length was 13.9 cm: reconstructed with vascularized free fibula in 68% (in 23% osseous allograft was also used) and used fibula growth plate transfer in 23%. The mean soft tissue defect area was 108 cm²: reconstructed with latissimus dorsi flap in 4 patients, vertical rectus abdominus muscle flap in 2, and anterolateral thigh flap in 1. The majority of the complications were nonunion (14%) and wound infection/dehiscence (14%). The median Musculoskeletal Tumor Society score was 70. Patients with tumors in the upper extremity had significantly higher Musculoskeletal Tumor Society scores compared with lower extremity tumor patients (80 vs 50, P = .04); and among those with lower extremity tumors, patients with distal defects had better outcomes than patients with proximal defects (70 vs 40, P = .03).

Conclusion

In pediatric oncology patients who need limb salvage, use of free flaps can result in good long-term functional outcomes.     

The impact of surgical excision in chest wall rhabdomyosarcoma: a report from the Children's Oncology Group

Aims

Rhabdomyosarcoma (RMS) is the most common soft tissue tumor of childhood. Patient age, size, histologic finding, and site of the tumor are primary determinants of prognosis in RMS. Chest wall RMS is a site in which the limitations of surgical excision are realized. We aim to determine the impact of surgical excision in chest wall RMS.

Methods

A retrospective chart review was conducted of all 130 pediatric patients enrolled in the Intergroup Rhabdomyosarcoma Study (IRS) with chest wall rhabdomyosarcoma from the first (I) through fourth (IV) IRS with follow-up to June 2005. Median follow-up was 12.1 years (4.6-27.2 years).

Results

There was a significant improvement in failure-free survival (FFS) and overall survival (OS) between the first IRS study, I, and IRS-IV. The estimated FFS and OS at 5 years in IRS I was 30% and 40%, respectively, compared to 68% and 78%, respectively, in IRS-IV (P = .03 and P = .05, respectively). There was no association between histologic finding or size and FFS or OS. However, all patients who presented without metastasis had an FFS and OS of 49% and 61%, respectively, compared with metastatic patients, 7% and 7%, respectively (P < .001). Five-year FFS of group I, II, and III patients was 52%, 52%, and 45%, respectively, and OS was 65%, 60%, and 59%, respectively. There was no significant difference in 5-year FFS or OS in patients who had a complete resection (group I), complete resection with positive microscopic margins (group II), or biopsy or partial resection only (group III). In groups I to III patients, the local and regional failure rate at 5 years is 25% and 6%, respectively.

Conclusions

The most significant impact on outcome in chest wall RMS patients is metastatic disease at diagnosis. The locoregional failure rate is high but does not appear to impact survival. Alternative treatment strategies are needed for chest wall RMS, but aggressive surgical excision may not be necessary.