A retrospective review of the effect of surgeon specialty on the management of 190 benign and malignant pediatric and adolescent adnexal masses.

STUDY OBJECTIVE: To investigate the impact of operating surgeon specialty on rates of ovarian preservation, and to explore differences in surgical management when malignant lesions are identified. DESIGN: Retrospective study. SETTING: Education and research hospitals. PARTICIPANTS: Between January 1, 2003 and January 1, 2009, all female patients ≤ 20 years of age undergoing surgery with pathologically confirmed ovarian or fallopian tube tissues removed were evaluated. INTERVENTIONS: Demographic, operative, and pathologic data were abstracted. MAIN OUTCOME MEASURES: Rates of ovarian preservation with benign lesions, and rates of appropriate surgical staging when malignant lesions were identified. RESULTS: The mean age was 11.9 ± 4.4 years. Malignant lesions were larger than benign masses, 17.3 ± 7.1 cm versus 8.8 ± 7.1 cm respectively (P < .001). Torsion was associated with oophorectomy with a relative risk (RR) of 1.86 and 95% confidence interval (CI) of 1.35-2.57 (P = 0.033). Postmenarchal patients were less likely to undergo ovarian sacrificing procedures (RR 0.62, 95% CI 0.45-0.84, P < .001). The relative risk of incomplete surgical staging with malignant lesions was reduced in the presence of a gynecologic oncologist (RR 0.14, 95% CI 0.02-0.89, P = .003). CONCLUSION: Ovarian conservation should be prioritized in cases with benign lesions, whereas complete and accurate surgical staging is imperative when malignancy is identified.

Pediatric chest wall Ewing's sarcoma.

BACKGROUND: Chest wall tumors of primitive neuroectodermal origin (PNET, Ewing's sarcoma [ES]) are rare and have a poor prognosis. Multimodality therapy has improved survival results, and long-term survival is possible. Whether adjuvant radiation therapy is uniformly beneficial remains unclear. METHODS: A retrospective analysis of the authors' institutional experience between 1979 and 1998 was performed. RESULTS: Twenty consecutive patients with PNET-ES of the chest wall were identified. The median age was 12 years (range, 2.5 to 21 years). Median follow-up was 3 years (range, 7 months to 19.4 years). Seven patients presented with a mass, 12 with pain, 1 with respiratory distress, and 1 with a neuropathy. Initial therapy consisted of biopsy and neoadjuvant chemotherapy followed by chest wall resection in 12 patients. Of the remaining 8 patients, 6 underwent biopsy, followed by chest wall resection and adjuvant chemotherapy, 1 underwent biopsy, chemotherapy, and resection of a lung nodule, and 1 underwent biopsy, chemotherapy, and a laminectomy and decompression procedure. All 20 patients were included in institutional-based trials using multiagent chemotherapy. Fifteen patients received radiation therapy with a median dose of 3,000 cGy. At last follow-up, 11 patients are alive and disease free, with a median survival of 7.5 years (range, 7 months to 19.4 years). Seven of 11 (64%) survivors had neoadjuvant therapy followed by chest wall resection. Seven of 11 (64%) survivors had radiation therapy. There was no surgical mortality. Twelve patients had treatment-related complications, 3 of which were related to surgical resection. There were no survivors among patients with recurrent disease. Three of the patients who died of disease had both local and distant recurrences, 4 patients had distant recurrence only, and one patient had local recurrence only. Only 4 of 9 (44%) patients who died were treated initially with chemotherapy followed by chest wall resection. All but 1 of those that died (89%) received initial radiation therapy. All 9 patients who did not survive received additional salvage radiotherapy as well. CONCLUSIONS: Long-term survival is possible with ES-PNET after complete chest wall resection. This may be facilitated by neoadjuvant chemotherapy. Long-term survival without radiation therapy is possible, and consideration of radiation therapy should be made on a case-by-case basis.

Diagnosis of mediastinal masses in pediatric patients using mediastinoscopy and the Chamberlain procedure.

BACKGROUND/PURPOSE: Mediastinal masses commonly are referred to the pediatric surgeon and can be difficult diagnostic problems. Various techniques have been used to perform biopsy in the mediastinum, but there are few reports of mediastinoscopy or Chamberlain procedure in children in the literature. The authors reviewed their experience with these techniques in a pediatric oncology population. METHODS: The medical records of all patients on the pediatric surgical service between 1987 and 1997, inclusive, who underwent mediastinoscopy or Chamberlain procedure were reviewed. Demographic data, diagnostic accuracy, complications, operating time, and blood loss were recorded. RESULTS: Sixteen consecutive patients underwent 13 Chamberlain procedures and six mediastinoscopies over the above period. Diagnostic accuracy was found to be 95% overall (100% for Chamberlain procedure, 83% for mediastinoscopy). Five complications occurred in the Chamberlain group and none in the mediastinoscopy group. No complication required thoracotomy or sternotomy. Among patients whose sole reason for admission was diagnosis of a mediastinal mass, the mean hospital stay was 1.7+/-0.8 days (n = 7) for those who underwent Chamberlain procedure and 1.4+/-0.9 days (n = 4) for those who underwent mediastinoscopy. One mediastinoscopy was performed as an ambulatory procedure. CONCLUSION: Mediastinoscopy and the Chamberlain procedure are effective and safe techniques for biopsy of mediastinal masses in this age group.

Childhood melanoma survival.

BACKGROUND: Melanoma in childhood is uncommon. Some believe that melanoma among children is associated with a better prognosis than among adults. METHODS: The authors reviewed their institutional experience with melanoma in 40 patients younger than 18 years treated between 1950 and 1984. All slides were reviewed by a single dermatopathologist who was blinded to clinical outcomes. Long term follow-up was available for all but three patients. RESULTS: There were 26 girls and 14 boys. The median age at diagnosis was 15 years (range, 3-17 years). Eleven patients (28%) were younger than 12 years. Fifteen patients (38%) had melanoma arise in a congenital nevus (2 had bathing trunk nevi. The most common site was the extremity (n = 23), followed by the trunk (n = 10) and the head and neck (n = 7). Seventeen patients (43%) initially were considered to have benign lesions, and 23 patients (57%) were diagnosed correctly with melanoma at initial presentation. Only 21 of 37 evaluable patients (57%) were alive at last follow-up with a median follow-up of 18 years (range, 2-48 years). Fifteen patients (41%) died of their disease, with a median survival of 12 months (range, 6-60 months). One patient died of breast carcinoma 14 years after treatment for melanoma. Disease free survival was 57% at 5 and 10 years. Of the 15 patients who died of disease, 12 were female (P = 0.09) and 10 had melanoma arising in a congenital nevus (P < 0.05). Five-year overall survival was 78% for patients who presented with localized disease (n = 23) and 30% for patients who presented with regional metastasis (n = 16, P < 0.001). There were no survivors among those who presented with systemic disease (n = 1). CONCLUSIONS: Children with melanoma are at significant risk of dying of their disease. Survival is similar to that seen among adults and depends on stage at presentation. The survival advantage observed for adult females is not seen among children.

Neovascularity and clinical outcome in high-grade extremity soft tissue sarcomas.

BACKGROUND: Increased tumor neovascularity has been shown to correlate with poor prognosis in solid tumors. METHODS: Microvessels were identified by factor VIII immunohistochemical staining. Analysis of microvessel counts, tumor characteristics, and resection details was performed on 119 primary, high-grade extremity soft tissue sarcomas (STS) and correlated with clinical outcome. RESULTS: Tumor characteristics and resection details were analyzed and patient outcome was examined with respect to local recurrence, distant metastasis, and disease-specific survival. Factors found to be significant on univariate analysis for all outcome variables were positive microscopic margin and tumor size. A positive microscopic margin was found to be a significant risk factor for local recurrence (P = .03), distant metastasis (P = .006), and disease-specific survival (P = .004). A primary tumor greater than 10 cm in diameter was a poor prognostic factor for distant metastasis (P = .03) and disease-specific survival (P = .006) when compared to tumors smaller than 10 cm. Microvessel count did not correlate with survival nor did it predict distant metastasis or local recurrence. Histologic subtypes of STS that have a prominent vascular pattern as a diagnostic criterion (i.e., angiosarcoma, liposarcoma, hemangiopericytoma) form a subgroup of all STS. Neovascularity in these subtypes showed no relationship to clinical outcome. CONCLUSIONS: These data confirm the prognostic importance of microscopic margin and tumor size in high-grade extremity STS. Neovascularity measured by factor VIII staining had no prognostic significance in these mesenchymal tumors, in contradistinction to carcinomas. Alternatively, microvessel counts may not accurately represent the angiogenic capacity of STS. Therefore, patients with STS who are eligible for anti-angiogenesis clinical trials cannot be identified solely by microvessel count.

Pediatric rhabdomyosarcoma of the head and neck.

PURPOSE: Survival for pediatric rhabdomyosarcoma has improved with the use of multidrug chemotherapy and external beam radiotherapy. This study was performed to determine survival in a cohort of patients treated on one of three multidrug treatment protocols for head and neck rhabdomyosarcoma and to identify factors that place patients at risk for treatment failure. METHODS: Pertinent prognostic variables including age, sex, subsite of origin, resectability, and TNM stage were analyzed by the Kaplan-Meier methods with comparisons between variables performed using the Prentice-Wilcoxon test statistic. RESULTS: Overall 5-year survival was 74% (95% confidence interval 64% to 84%). Local failure accounted for the cause of death in 10 patients, and 8 died of disseminated disease. On univariate analysis, each variable contributing to the TNM staging system was significant in determining survival; invasiveness (P = 0.01), size (P = 0.02), nodal metastases (P <0.01), and distant disease (P <0.01). CONCLUSION: Survival has improved for head and neck rhabdomyosarcoma treated with multimodality therapy. Patients with advanced-stage disease are at greatest risk for treatment failure and require the most aggressive therapy.

Chest wall rhabdomyosarcoma.

BACKGROUND: Rhabdomyosarcoma is the most common soft tissue sarcoma in the pediatric age group. The primary tumor site is an important prognostic determinant. Axial lesions are associated with decreased survival and provide a clinical challenge. METHODS: A retrospective analysis of the authors' institutional experience between 1972 and 1996 was performed. Patients were from a data base of 302 consecutive cases. RESULTS: Fifteen consecutive patients with chest wall rhabdomyosarcoma were identified. The median age was 16 years (range, 6 months-25 years). Median follow-up was 6.6 years (range, 10 months-18.5 years). Nine patients presented with a mass, six with pain, two with respiratory distress, and one with ulnar neuropathy. The median lesion size was 7 cm (range, 3-16 cm). A surgical procedure was the initial therapy for 13 of 15 patients. Fourteen patients received radiation therapy with a median dose of 4400 cGy. All but one were included in institutional-based trials using multiagent chemotherapy. At last follow-up, 10 patients were alive and disease free, with a median survival of 123 months (range, 51-221 months). Seven of ten survivors underwent a complete resection as their initial therapy. There was no surgical mortality, and only two patients had treatment-related complications. Of the five patients who died, two underwent complete resection as their initial therapy. All five patients had invasive tumors. Four were > 10 cm, 3 were of alveolar subtype, and 2 were embryonal. CONCLUSIONS: Complete resection of chest wall rhabdomyosarcoma is recommended. However, survival is possible for patients with microscopically positive surgical margins with the addition of chemotherapy and radiation.

The application of minimal access procedures in infants, children, and young adults with pediatric malignancies.

OBJECTIVE: In this study, we sought analysis of minimal access procedures in pediatric and young adult oncology patients. METHODS: Between 1990 and 1997, 84 patients underwent 93 minimal access procedures. Clinical, pathological, and operative details were analyzed. RESULTS: There were 32 females and 52 males with a median age of 14 years (range 3 months to 31 years). The median body weight was 50 kg (range 6-94 kg). There were 47 thoracoscopic procedures and 46 laparoscopic procedures. Laparoscopic procedures included liver biopsy (21), diagnostic tumor biopsy (13), lymph node biopsy (4), cholecystectomy (4), oophoropexy (3), and kidney biopsy (1). Median hospital stay was 2 days (range 1-14 days). Six patients had their procedure converted to an open procedure (13%). Thoracoscopic procedures included diagnostic lung biopsy (22), mediastinal mass biopsy or resection (4), pleural biopsy (5), and pleurodesis (4). Eleven were converted to open thoracotomy (23%). Median hospital stay was 4 days (range 2-35 days). There were two complications after laparoscopy (4%) and three disease-related deaths. There were six complications after thoracoscopy (13%), and three disease-related deaths. Adequate tissue was obtained in all biopsy procedures. CONCLUSIONS: Children with cancer require operations for diagnosis and staging. Minimal access procedures are safe and effective and allow adjuvant therapy to begin earlier.

Posterior mediastinal masses.

Solid mediastinal masses in infancy and childhood occur most frequently in the posterior mediastinum. From 1972 to 1989, 63 patients presented with a posterior mediastinal mass. The median age at diagnosis was 6 years (range, 1 day to 26 years). Thirty patients were female. Forty-five percent of the patients presented with respiratory symptoms or chest pain; 13% had neurologic symptoms, one half of which were related to spinal cord compression; and 5% had a palpable mass. In 32% of patients the mass was an incidental finding. The tumors were of neurogenic origin in 89% of patients, of which neuroblastoma was the most common. Of all patients with posterior mediastinal masses, 60% had malignant tumors. Median follow-up for 62 of 63 evaluable patients was 45 months (range, 1 to 289 months). One patient was lost to follow-up. Of the 62 patients followed, 84% are alive and free of disease. All but 4 of the 32 patients with neuroblastoma are alive and free of disease with a median follow-up of 73 months (range, 7 to 289 months). Patients with neuroblastoma who were diagnosed in the first year of life had a significantly better survival pattern than those presenting after the first year. There were seven deaths in the series: four from neuroblastoma, two from primitive neuroectodermal tumor, and one from malignant schwannoma. Preoperative diagnostic evaluation of a posterior mediastinal mass should include posteroanterior and lateral chest roentgenograms, and either CT or MRI of the chest and abdomen to assess the extent of the mass.(ABSTRACT TRUNCATED AT 250 WORDS)

Reduction of smooth muscle hyperplasia in vein grafts in athymic rats.

While developing an animal model of a vascular malformation, we found that two doses of cyclosporin A significantly reduced the smooth muscle cell hyperplasia observed in vein-arterial interposition grafts in Sprague-Dawley rats. Therefore, we hypothesized that T cells may either produce or augment a mitogen for vascular smooth muscle cells. To further investigate this, we quantitated the extent of smooth muscle cell hyperplasia in the vein grafts of athymic nude rats that lack mature, functioning T cells. Male Sprague-Dawley rats (275 to 350 gm) and athymic nude rats were anesthetized, and a segment of the superficial epigastric vein was placed into the transected femoral artery using microsurgical techniques. Sprague-Dawley rats (N = 9) and athymic nude rats (N = 5) undergoing vein grafting received 30 mg/kg cyclosporin A intraperitoneally, intraoperatively and 24 hours later. Sprague-Dawley rats (N = 7) and athymic nude rats (N = 6) that had vein grafts were not treated with cyclosporin A. Animals were killed at either 3 weeks or 6 weeks and histologic sections were taken from the middle of the graft to avoid clamp-induced trauma. At 3 weeks, untreated vein grafts in Sprague-Dawley rats exposed to arterial pressure exhibited a nine-fold increase in smooth muscle hyperplasia compared with the preoperative vein. Treatment of Sprague-Dawley rats with cyclosporin A resulted in a 57% reduction of smooth muscle hyperplasia (p less than 0.05). Vein grafts from athymic nude rats exhibited a 51% reduction in smooth muscle hyperplasia (p less than 0.05). Sprague-Dawley rats killed at 6 weeks revealed a recovery of smooth muscle hyperplasia equivalent to an untreated Sprague-Dawley vein graft at 3 weeks. Inhibition of smooth muscle hyperplasia persisted for 6 weeks in the athymic nude rats. Cyclosporin A administration or T cell deficiency in athymic nude rats decreases the smooth muscle hyperplasia observed in venous grafts exposed to arterial pressure. This finding provides evidence for a possible role of T cells in the regulation of cell growth in the vascular wall.

Experience with colorectal carcinoma metastatic to the liver.

The predilection of colorectal cancer metastases for the liver is probably the result of several factors, including the blood supply, the "homing" characteristics of the tumor cells, and the state of the liver. Five-year survival rates after hepatic resection for colorectal cancer metastases range from 20 to 40 per cent, and some other patients obtain palliative benefit. The authors discuss the three presentations of liver metastases, operative techniques, and prognostic factors.