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Background Osteosarcoma (OS) and the Ewing sarcoma family of tumors (ESFT) are the most common primary pediatric bone malignancies. We sought to assess the diagnostic accuracy of initial tumor biopsies in patients with OS or ESFT at a pediatric cancer center. Methods All biopsies performed at initial presentation of patients with OS or ESFT at our institution from 2003 to 2012 were retrospectively reviewed. Diagnostic accuracy and incidence of complications were correlated with study variables using logistic regression analysis. Results One hundred forty-two biopsies were performed in 105 patients (median age 13.4 years, range: 1.8-23.0), 104 (73.2%) OS and 38 (27.8%) ESFT. Thirty-one (21.8%) were performed on metastatic sites. Eighty-five (76.6%) of 111 primary site biopsies were open procedures, and 26 were percutaneous (23.4%). Primary site biopsies were successful in 94.1% of open and 73.1% of percutaneous procedures. Odds of obtaining a successful diagnostic specimen were 7.8 times higher with open approach (CI: 1.6-36.8). Metastatic site biopsies were successful in 66.7% of percutaneous and 100% of open and thoracoscopic procedures. Conclusion Biopsy of metastatic sites was equal to primary site in obtaining diagnostic material with the added benefit of accurate staging, with few adverse events and high diagnostic yield. [ABSTRACT FROM AUTHOR]

Abstract: Purpose: Detection and treatment of small lung nodules are important in managing pediatric cancer. We studied the effectiveness of preoperative localization of pulmonary nodules by CT-guided needle hook wire placement followed by thoracoscopic resection in children with cancer. Methods: We reviewed records of patients who underwent thoracoscopic resection of lung nodules localized preoperatively with CT-guided needle and hook wire placement at our hospital between March 1999 and April 2010 for nodule characteristics and outcomes of procedure. Results: Thirty-seven patients (median age, 14years) with osteosarcoma or other cancers underwent thoracoscopic resection of needle-localized lung nodules. Lesion (median nodule size, 4mm) location was left lung (n=11), right lung (n=19), and bilateral (n=7). The procedure was successful in 36 (97.3%) patients. Five patients had a pneumothorax after localization but none required chest tube placement before thoracoscopy. All patients underwent thoracoscopy, but 4 required conversion to open thoracotomy. During thoracoscopic inspection, the hook wire slipped out of the lesion in 6 patients, of whom 1 needed thoracotomy to locate nodule. Lesions (malignant in 13 patients) were removed in all patients. Five patients with benign lesions had recurrent malignant lung nodules. Conclusions: Thoracoscopic resection of preoperatively localized small lung nodules is a safe and effective procedure in children. [Copyright &y& Elsevier]

Abstract: Background/Purpose: Bones of the hands and feet are uncommon sites for Ewing sarcoma. In this study, we reviewed our experience in the management of these tumors. Methods: We retrospectively reviewed clinical presentation, management, and outcome of patients with Ewing sarcoma of the bones of hands and feet treated at our institution (1981-2006). Results: The cohort included 6 males and 3 females (8 white, 1 African American; median age at diagnosis, 15 years). Primary tumor site was the hand in 6 and the foot in 3 patients. Three patients had distant metastatic disease at diagnosis (lung [n = 2]; ipsilateral axillary lymph node[(n = 1]). All patients had painful swelling at the primary site, and 2 (22%) had pathological fracture. All patients received chemotherapy and local control measures (surgery [n = 6], radiation [n = 2], surgery and radiation [(n = 1]). Three patients received radiotherapy for distant metastases. Three patients had systemic recurrence (lungs [n = 2], lung and brain [n = 1]); none had local tumor recurrence. Median follow-up was 5 years. Five patients (55.6%) are alive at last follow-up. Conclusions: Chemotherapy and surgical excision of primary tumor are the mainstays of treatment. Radiotherapy is recommended for local control of lesions in the hand for patients declining excisional therapy. [Copyright &y& Elsevier]

Abstract: Background/Purpose: The purpose of this study is to determine if patients with osteosarcoma (OS) with metachronous metastatic pulmonary disease presenting with a single pulmonary nodule (SPN) on computed tomography (CT) were found to have other lesions at the time of thoracotomy. Methods: Data were collected retrospectively on consecutive patients with OS treated at our institution from 1982 to 2007. Patients with no evidence of disease at the end of initial therapy who subsequently relapsed in the lung were identified. Results: In our study, 16 (8%) of 198 patients with OS with metachronous metastatic pulmonary disease presented with a SPN on CT scan. In all patients, only 1 metastatic nodule for OS was found at the time of thoracotomy. The median time between diagnosis and first lung relapse was 23.8 months (range, 4-80 months). Eleven patients (68.7%) subsequently had a second lung relapse, but only 3 patients had involvement of the ipsilateral lung (mean time interval between first and second pulmonary relapses of 17 months; range, 2-44 months). Five-year overall survival from diagnosis was 56.2%. Seven patients (43.8%) died of disease progression. Conclusions: In our experience, patients with OS with metachronous metastatic pulmonary disease presenting with a SPN on CT were not found to have additional malignant lesions at the time of thoracotomy. Consideration should be given in this group of selected patients to use a minimally invasive approach to nodule removal with image-guided localization, if needed, rather than open thoracotomy because ipsilateral metastases are not likely to be found. [Copyright &y& Elsevier]

Abstract: Background/Purpose: The aim of this study was to retrospectively analyze the clinical presentation, histology, treatment, and outcomes of children with vaginal tumors who were treated at a single institution. Methods: A retrospective review of medical records and pathologic materials of all children with vaginal tumors treated at St Jude Children''s Research Hospital between 1970 and 2009 was conducted. Results: Eighteen patients (median age, 3.7 years; range, 0.1-15 years) were identified. Three different histologies were found: rhabdomyosarcoma (RMS; n = 13), germ cell tumor (n = 3), and clear cell adenocarcinoma (n = 2). Bleeding or blood-tinged discharge was the most common clinical presentation (66%), followed by a protruding mass (39%). Vaginal and uterine salvage was 44.4% (8 of 18 patients). Thirteen patients (72.2%) remain disease-free, with a median follow-up of 23.2 years (range, 2-39 years). Four patients (22.2%) died of disease progression (1 RMS, 2 germ cell tumor, and 1 clear cell adenocarcinoma), and 1 patient with RMS died of colon cancer 12 years after the primary diagnosis had been made. Conclusions: Vaginal tumors are extremely rare in the pediatric population. Early recognition of symptoms like bleeding and a protruding vaginal mass may prevent morbidity and mortality. Our findings confirm the good prognosis of vaginal RMS. [Copyright &y& Elsevier]

Abstract: Background: Predictors of outcome have not been established for pediatric visceral and body wall nonrhabdomyosarcoma soft tissue sarcomas (NRSTS). Methods: The study used a retrospective review of clinical features and outcome of 61 patients with visceral and body wall NRSTS evaluated at our institution between March 1962 and December 1999. Results: Median age at diagnosis was 9.9 years (range, birth to 17.4 years). Tumors were greater than 5 cm in 43 (70%), high grade in 33 (54%), invasive in 25 (41%), and metastatic at presentation in 14 (23%) patients. Visceral tumors (n = 27) were more likely than body wall tumors (n = 34) to be greater than 5 cm (93% vs 53%; P < .001) and invasive (70% vs 18%; P < .001) and were less likely to be resected at diagnosis (44% vs 85%; P = .001). Estimated 10-year event-free survival (EFS) and overall survival (OS) for the entire cohort were 45.5% ± 6.9% and 56.8% ± 6.7%, respectively. The 10-year EFS and OS were better for patients with body wall sites than for those with visceral sites (61.8% ± 8.5% and 67.5% ± 8.2% vs 24.2% ± 9.4% and 43.0% ± 10.3%; P = .004 and P = .004). The 10-year estimated cumulative incidence (CI) of local recurrence was higher for patients with visceral sites than for those with body wall sites (64.3% ± 9.8% vs 26.5% ± 7.7%; P = .004), whereas CI of distant recurrence was similar for the 2 sites (15.2% ± 7.2% vs 23.5% ± 7.4%; P = .39). Conclusions: Pediatric patients with visceral NRSTS are more likely to have invasive, large, and unresectable tumors compared to those with body wall tumors. More than two thirds of visceral NRSTS recur locally, and fewer than half of patients with visceral tumors survive. [Copyright &y& Elsevier]

Abstract: Purpose: The incidence of pediatric nonrhabdomyosarcoma soft tissue sarcomas (NRSTSs) of the groin and axilla is unknown, and the optimal surgical approach to these patients is unclear. Methods: We conducted a retrospective study of patients treated at St Jude Children''s Research Hospital from January 1962 to March 2007 for NRSTSs of the groin and axilla. Demographic variables, tumor pathology, clinical management, and outcome were reviewed. Results: Of the 300 patients treated for NRSTSs, only 10 had tumors of the axilla or groin (6 of whom had synovial sarcoma). Surgical interventions included wide resection of the tumor (n = 7), marginal resection (n = 1), subtotal resection (n = 1), and biopsy only (n = 1). Six patients underwent lymph node sampling; all were negative for tumor. Short- and long-term surgical complications were rare. Four patients received adjuvant chemotherapy (n = 3) and/or radiotherapy (n = 2). At a median follow-up of 8.5 years, 7 of the 10 were surviving free of disease. Two of these patients died of tumor progression (1 with metastases at diagnosis and 1 with an unresectable tumor at diagnosis), and one patient who was free of NRSTS died of secondary breast carcinoma. Conclusions: Pediatric NRSTSs of the axilla and groin are rare, but outcomes are similar to those of other patients with NRSTS. Wide local excision of the tumor with preservation of good limb function should be the surgical goal and may be sufficient therapy in some cases. [Copyright &y& Elsevier]

Abstract: Purpose: Children presenting with osteosarcoma and pulmonary metastases have poor survival rates. The standard approach to treating unilateral metastases is ipsilateral thoracotomy with complete resection of the metastases whenever possible. We analyzed whether contralateral exploratory thoracotomy is beneficial in these patients. Methods: We reviewed the records of all osteosarcoma patients presenting with or developing early pulmonary metastases (within 24 months of diagnosis) at the St Jude Children''s Research Hospital (Memphis, Tenn) between June 1980 and September 2005. Demographics, imaging results, treatment protocols, surgical procedures, and recurrence, survival, and timeline data were assessed. Results: Of the 109 patients, initial pulmonary involvement was radiographically identified as unilateral in 81 (74%) and bilateral in 28 (26%) patients. Of the patients presenting with unilateral pulmonary metastases, 2 years later, 13 (16%) had recurrence in the ipsilateral and 19 (23%) in the contralateral lung. In 2 (2.4%) patients, metastases recurred bilaterally. There was no evidence of statistically significant difference between the incidence of recurrence in the ipsilateral and contralateral lung (P = .18). Conclusions: Children with osteosarcoma and unilateral pulmonary metastases had similar incidence of recurrence in the ipsilateral and contralateral lung. Their survival rates were also not significantly different. Therefore, contralateral exploratory thoracotomy in patients presenting with unilateral pulmonary metastases will probably not improve survival. [Copyright &y& Elsevier]

Abstract: 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. [Copyright &y& Elsevier]

Abstract: Epithelioid sarcoma is a rare soft tissue sarcoma with a propensity for local aggressiveness, regional nodal spread, and pulmonary metastases. We report a case of epithelioid sarcoma in a neonate with bilateral optic nerve hypoplasia who developed liver, kidney, and bone metastases. The unusual presenting features and pattern of disease progression in this patient suggest that a different disease evaluation strategy should be considered for infants with epithelioid sarcoma. [Copyright &y& Elsevier]