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Annals of Surgical Oncology 05/2013; · 4.17 Impact Factor
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ABSTRACT: BACKGROUND: Chemotherapy has improved the outcome of patients with newly diagnosed osteosarcoma, but its role in relapsed disease is unclear. METHODS: We reviewed the records of all patients who were treated for relapsed high-grade osteosarcoma at our institution between 1970 and 2004. Postrelapse event-free survival (PREFS) and postrelapse survival (PRS) were estimated, and outcome comparisons were made using an exact log-rank test. RESULTS: The 10-year PREFS and PRS of the 110 patients were 11.8% ± 3.5% and 17.0% ± 4.3%, respectively. Metastasis at initial diagnosis (14%), and relapse in lung only (75%) were not significantly associated with PREFS or PRS. Time from initial diagnosis to first relapse (RL1) ≥18 months (43%), surgery at RL1 (76%), and ability to achieve second complete remission (CR2, 56%) were favorably associated with PREFS and PRS (P ≤ 0.0002). In patients without CR2, chemotherapy at RL1 was favorably associated with PREFS (P = 0.01) but not with PRS. In patients with lung relapse only, unilateral relapse and number of nodules ( ≤ 3) were associated with better PREFS and PRS (P ≤ 0.0005); no patients with bilateral relapse survived 10 years. The median PREFS after treatment with cisplatin, doxorubicin, methotrexate, and ifosfamide was 3.5 months (95% confidence interval, 2.1-5.2), and the median PRS was 8.2 months (95% confidence interval, 5.2-15.1). CONCLUSIONS: Late relapse, surgical resection, and unilateral involvement (in lung relapse only) favorably impact outcome after relapse. Surgery is essential for survival; chemotherapy may slow disease progression in patients without CR2. These data are useful for designing clinical trials that evaluate novel agents. Cancer 2013;. © 2013 American Cancer Society.
Cancer 04/2013; · 4.77 Impact Factor
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ABSTRACT: 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.
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.
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.
Thoracoscopic resection of preoperatively localized small lung nodules is a safe and effective procedure in children.
Journal of Pediatric Surgery 04/2013; 48(4):750-6. · 1.45 Impact Factor
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ABSTRACT: 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.
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).
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.
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.
Journal of Pediatric Surgery 10/2012; 47(10):1806-10. · 1.45 Impact Factor
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ABSTRACT: Ewing sarcoma (ES) is the most common chest wall malignancy in adolescents. Current therapy incorporates chemotherapy to treat systemic disease and radiotherapy to assist with local control. We sought to evaluate the timing of surgery and role of adjuvant radiotherapy.
We reviewed the St. Jude Children's Research Hospital chest wall ES experience from 1979 to 2009. Patient demographics, tumor characteristics, treatment variables, and outcomes were analyzed with respect to timing of surgery and use of adjuvant radiotherapy.
Our cohort consisted of 36 patients with chest wall ES; median follow-up was 14.2 years, and 15-year estimate of overall survival was 66 %. In patients with localized disease, the timing of surgery (up-front vs. delayed) did not impact margin negativity or the use of adjuvant radiotherapy, but it did decrease the extent of chest wall resection. When considering radiotherapy in patients with localized disease, we found that patients who did not receive radiotherapy had smaller tumor size (median 6 vs. 10 cm) (p = 0.04) and were more likely to have had negative margins (p = 0.01) than patients who received adjuvant radiotherapy. One patient in each group developed a locoregional recurrence. The 15-year estimated of overall survival for patients who received adjuvant radiotherapy was 80 versus 100 % for those who did not.
Delayed surgery decreased the extent of chest wall resection and helped define a patient population with favorable tumor biology. Patients with complete pathologic responses to chemotherapy, and those with tumors <8 cm and negative surgical margins may be spared adjuvant radiotherapy without any decrement in overall survival.
Annals of Surgical Oncology 07/2012; 19(12):3809-15. · 4.17 Impact Factor
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ABSTRACT: 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.
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.
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.
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.
Journal of Pediatric Surgery 06/2012; 47(6):1250-4. · 1.45 Impact Factor
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ABSTRACT: The purpose of this study was to describe the use of lymphoscintigraphy and sentinel lymph-node biopsy (SLNB) for the management of children with melanoma and sarcomas. We report the experience of two children's hospitals that utilize this technique to identify sentinel lymph nodes for lymph-node biopsy and dissection.
We identified 56 patients (median age 10.8 years) who underwent 58 lymphoscintigraphy procedures. There were 33 patients with melanoma and melanocytic lesions, and 23 with sarcomas.
Of 58 lymphoscintigraphy procedures, sentinel lymph nodes were identified in 52 (90% success rate). Using the combination of intraoperative blue dye injection and lymphoscintigraphy, the success rate was 95% (55/58). Metastatic disease was found in 14 sentinel lymph nodes (13 patients with melanoma and melanocytic lesions, and 1 patient with rhabdomyosarcoma).
We have found that lymphoscintigraphy with SLNB is an effective method to identify patients who may benefit from more extensive lymph-node dissection and to identify those patients who are unlikely to benefit from further lymph-node exploration.
Pediatric Surgery International 04/2012; 28(6):571-8. · 1.25 Impact Factor
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ABSTRACT: 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.
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.
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.
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.
Journal of Pediatric Surgery 11/2011; 46(11):2071-5. · 1.45 Impact Factor
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ABSTRACT: Hemangiopericytoma (HPC) is a heterogeneous, highly vascularized malignant soft-tissue neoplasm with 2 different clinical presentations: adult-type and infantile-type HPC. Intracranial HPC represents a special subtype with a high proclivity toward recurrence and metastasis.
The authors have reviewed the clinical features, response to treatment, and outcomes of 17 patients with HPC treated at St Jude Children's Research Hospital from 1962 to 2009.
At diagnosis, 11 patients were older than 1 year (subgroup A) and 6 patients were younger than 1 year (subgroup B). Subgroup A: median age at diagnosis 13.5 years, (range, 4 to 20 y). Primary sites were intracranial (n=5), thigh (n=3), calf (n=1), foot (n=1), and scalp (n=1). One patient who presented with a thigh HPC had metastatic disease at diagnosis, and 3 patients with head location had unresectable tumors. Two patients with thigh location experienced objective responses to chemotherapy. Six patients died of disease progression, 4 of them had an intracranial location. The remaining 5 children are alive at follow-up of 12 to 32 years. Subgroup B: median age at diagnosis 0.5 months (range, 0 to 3 mo). Primary sites were thigh (n=2), calf (n=1), perianal (n=1), forearm (n=1), and lung (n=1). Three patients with limb location had unresectable disease at diagnosis, 2 of them experienced excellent responses to neoadjuvant chemotherapy and 1 did not show any response to chemotherapy and a staged resection was performed. All 6 infants are alive without evidence of disease at follow-up of 2 to 27 years.
Infantile HPC is characterized by a better clinical behavior than the adult type, which requires an aggressive multimodality therapy. Chemoresponsiveness and spontaneous regression have been reported in children younger than 1 year, suggesting that a more conservative surgical approach should be used. Intracranial HPC is considered as an aggressive tumor because of its propensity for recurrence and metastasis.
Journal of Pediatric Hematology/Oncology 07/2011; 33(5):356-9. · 1.16 Impact Factor
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ABSTRACT: The standard treatment of osteosarcoma includes cisplatin and high-dose methotrexate (HDMTX); both agents exert significant toxicity, and HDMTX requires complex pharmacokinetic monitoring and leucovorin rescue. In the previous OS91 trial, the treatment of localized disease with carboplatin, ifosfamide, doxorubicin, and HDMTX yielded outcomes comparable to those of cisplatin-based regimens and caused less toxicity. To build on this experience, the authors conducted a multi-institutional trial (OS99) that evaluated the efficacy of carboplatin, ifosfamide, and doxorubicin without HDMTX in patients with newly diagnosed, localized, resectable osteosarcoma.
Treatment was comprised of 12 cycles of chemotherapy administered over 35 weeks: 3 cycles of carboplatin (dose targeted to area under the concentration-time curve of 8 mg/mL × min on Day 1) and ifosfamide (at a dose of 2.65 g/m(2) daily ×3 days) and 1 cycle of doxorubicin (at a dose of 25 mg/m(2) daily ×3 days) before surgical resection, followed by 2 additional cycles of the combination of carboplatin and ifosfamide and 3 cycles each of doxorubicin (25 mg/m(2) daily ×2 days) combined with ifosfamide or carboplatin.
A total of 72 eligible patients (median age, 13.4 years) were enrolled between May 1999 and May 2006. Forty of the 66 (60.6%) evaluable patients had good histologic responses (>90% tumor necrosis) to preoperative chemotherapy. The estimated 5-year event-free survival rate was 66.7% ± 7.0% for the OS99 trial compared with 66.0% ± 6.8% for the OS91 trial (P = .98). The estimated 5-year survival rate was 78.9% ± 6.3% for the OS99 trial and 74.5% ± 6.3% for the OS91 trial (P = .40).
The regimen used in the OS99 trial was found to produce outcomes comparable to those of cisplatin-containing or HDMTX-containing regimens. This therapy offers a good alternative for patients, particularly those who demonstrate an intolerance of HDMTX, and for institutions that cannot provide pharmacokinetic monitoring for MTX.
Cancer 06/2011; 117(12):2770-8. · 4.77 Impact Factor
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ABSTRACT: Predictors of outcome have not been established for pediatric visceral and body wall nonrhabdomyosarcoma soft tissue sarcomas (NRSTS).
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.
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).
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.
Journal of Pediatric Surgery 10/2009; 44(10):1965-71. · 1.45 Impact Factor
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Matthew J Krasin,
Andrew M Davidoff,
Xiaoping Xiong,
Shengjie Wu,
Chia-Ho Hua,
Fariba Navid,
Carlos Rodriguez-Galindo, Bhaskar N Rao,
Kelly A Hoth,
Michael D Neel,
Thomas E Merchant,
Larry E Kun,
Sheri L Spunt
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ABSTRACT: To demonstrate the safety and efficacy of limited margin radiotherapy in the local control of pediatric and young adult patients with high-grade nonrhabdomyosarcoma soft tissue sarcoma (NRSTS).
Pediatric patients with high-grade NRSTS requiring radiation were treated on an institutional review board approved prospective institutional study of conformal/intensity-modulated/interstitial brachytherapy using a 2-cm anatomically constrained margin.
A total of 32 patients (median age, 15.3 years; range, 2-22 years) received adjuvant (27 patients) or definitive (5 patients) irradiation. With a median follow-up of 32 months, the 3-year cumulative incidence of local failure was 3.7% for patients undergoing irradiation after surgical resection. Four patients experienced local failure; the mean dose to the volume of recurrence was >or=97% of the prescribed dose.
Delivery of limited margin radiotherapy using external beam or brachytherapy provides a high rate of local tumor control without marginal failure. Further follow-up is required to determine whether normal tissue effects are minimized using this approach.
International journal of radiation oncology, biology, physics 08/2009; 76(3):874-8. · 4.59 Impact Factor
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Pediatric Blood & Cancer 07/2009; 53(3):310-1. · 1.89 Impact Factor
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ABSTRACT: 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.
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.
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).
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.
Journal of Pediatric Surgery 05/2009; 44(4):665-71. · 1.45 Impact Factor
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ABSTRACT: Pediatric colorectal carcinoma (CRC) is rare, but the available data suggest that it is more likely than adult CRC to be advanced at presentation and to have a poor outcome. We sought to better characterize pediatric CRC.
We reviewed the clinical and pathologic features, prognostic factors, and outcome of CRC in 77 children and adolescents (ages 7 to 19 years) referred to St Jude Children's Research Hospital between 1964 and 2003.
At presentation, 76 patients had one or more signs or symptoms of CRC (abdominal pain, altered bowel habits, weight loss, anemia). Tumors were evenly distributed between the right and left colon; 62% were mucinous adenocarcinoma. At presentation, 86% of patients had advanced-stage disease; more than half had distant metastases. Overall outcome was poor. Advanced stage and mucinous histology were significant predictors of adverse outcome. Stage-specific survival at 10 years was 67% +/- 27% (stage 1), 38% +/- 15% (stage 2), 28% +/- 11% (stage III), and 7% +/- 4% (stage 4). Although no patient had a diagnosis of polyposis syndrome before diagnosis of CRC, 17 (22%) had colon polyps and eight (including two who previously underwent pelvic radiotherapy) had multiple polyps.
Initial signs and symptoms of CRC are similar in pediatric and adult patients. The strikingly higher frequency of mucinous histology suggests that the biology of CRC differs in pediatric and adult patients and may contribute to poor outcomes. Children should be included in prospective clinical trials for CRC.
Journal of Clinical Oncology 01/2008; 25(36):5808-14. · 18.37 Impact Factor
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Jill P Ginsberg,
Shesh N Rai,
Claire A Carlson,
Anna T Meadows,
Pamela S Hinds,
Elena M Spearing,
Lijun Zhang,
Lulie Callaway,
Michael D Neel, Bhaskar N Rao,
Victoria G Marchese
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ABSTRACT: Comparison of functional mobility and quality of life is performed in patients with lower-extremity bone sarcoma following either amputation, limb-sparing surgery, or rotationplasty with four different types of outcome measures: (1) an objective functional mobility measure that requires patients to physically perform specific tasks, functional mobility assessment (FMA); (2) a clinician administered tool, Musculoskeletal Tumor Society Scale (MSTS); (3) a patient questionnaire, Toronto Extremity Salvage Scale (TESS); and (4) a health-related quality of life (HRQL) measure, Short Form-36 version 2 (SF-36v.2).
This is a prospective multi-site study including 91 patients with lower-extremity bone sarcoma following amputation, limb-sparing surgery, or rotationplasty. One of three physical therapists administered the quality of life measure (SF-36v.2) as well as a battery of functional measures (FMA, MSTS, and TESS).
Differences between patients who had amputation, limb-sparing surgery, or rotationplasty were consistently demonstrated by the FMA. Patients with limb sparing femur surgery performed better than those patients with an above the knee amputation but similarly to a small number of rotationplasty patients. Several of the more conventional self-report measures were shown to not have the discriminative capabilities of the FMA in these cohorts.
In adolescents with lower-extremity bone sarcoma, it may be advantageous to consider the use of a combination of outcome measures, including the FMA, for objective functional mobility assessment along with the TESS for a subjective measure of disability and the SF-36v.2 for a quality-of-life measure.
Pediatric Blood & Cancer 01/2008; 49(7):964-9. · 1.89 Impact Factor
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ABSTRACT: Reliability and validity of a new tool, Functional Mobility Assessment (FMA), were examined in patients with lower-extremity sarcoma. FMA requires the patients to physically perform the functional mobility measures, unlike patient self-report or clinician administered measures.
A sample of 114 subjects participated, 20 healthy volunteers and 94 patients with lower-extremity sarcoma after amputation, limb-sparing, or rotationplasty surgery. Reliability of the FMA was examined by three raters testing 20 healthy volunteers and 23 subjects with lower-extremity sarcoma. Concurrent validity was examined using data from 94 subjects with lower-extremity sarcoma who completed the FMA, Musculoskeletal Tumor Society (MSTS), Short-Form 36 (SF-36v2), and Toronto Extremity Salvage Scale (TESS) scores. Construct validity was measured by the ability of the FMA to discriminate between subjects with and without functional mobility deficits.
FMA demonstrated excellent reliability (ICC [2,1] >or=0.97). Moderate correlations were found between FMA and SF-36v2 (r = 0.60, P < 0.01), FMA and MSTS (r = 0.68, P < 0.01), and FMA and TESS (r = 0.62, P < 0.01). The patients with lower-extremity sarcoma scored lower on the FMA as compared to healthy controls (P < 0.01).
The FMA is a reliable and valid functional outcome measure for patients with lower-extremity sarcoma. This study supports the ability of the FMA to discriminate between patients with varying functional abilities and supports the need to include measures of objective functional mobility in examination of patients with lower-extremity sarcoma.
Pediatric Blood & Cancer 08/2007; 49(2):183-9. · 1.89 Impact Factor
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PT Victoria G. Marchese PhD,
Shesh N. Rai PhD,
BSN Claire A. Carlson RN,
Pamela S. Hinds PhD, RN, FAAN,
Elena M. Spearing MA, DPT, PT, PCS,
Lijun Zhang MS,
MS Lulie Callaway PT,
Michael D. Neel MD,
Bhaskar N. Rao MD,
Jill P. Ginsberg MD,
Victoria G. Marchese,
Shesh N. Rai,
Claire A. Carlson,
Pamela S. Hinds,
Elena M. Spearing,
Lijun Zhang,
Lulie Callaway,
Michael D. Neel, Bhaskar N. Rao,
Jill P. Ginsberg
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ABSTRACT: Background
Reliability and validity of a new tool, Functional Mobility Assessment (FMA), were examined in patients with lower-extremity sarcoma. FMA requires the patients to physically perform the functional mobility measures, unlike patient self-report or clinician administered measures.ProcedureA sample of 114 subjects participated, 20 healthy volunteers and 94 patients with lower-extremity sarcoma after amputation, limb-sparing, or rotationplasty surgery. Reliability of the FMA was examined by three raters testing 20 healthy volunteers and 23 subjects with lower-extremity sarcoma. Concurrent validity was examined using data from 94 subjects with lower-extremity sarcoma who completed the FMA, Musculoskeletal Tumor Society (MSTS), Short-Form 36 (SF-36v2), and Toronto Extremity Salvage Scale (TESS) scores. Construct validity was measured by the ability of the FMA to discriminate between subjects with and without functional mobility deficits.ResultsFMA demonstrated excellent reliability (ICC [2,1] ≥0.97). Moderate correlations were found between FMA and SF-36v2 (r = 0.60, P < 0.01), FMA and MSTS (r = 0.68, P < 0.01), and FMA and TESS (r = 0.62, P < 0.01). The patients with lower-extremity sarcoma scored lower on the FMA as compared to healthy controls (P < 0.01).Conclusion
The FMA is a reliable and valid functional outcome measure for patients with lower-extremity sarcoma. This study supports the ability of the FMA to discriminate between patients with varying functional abilities and supports the need to include measures of objective functional mobility in examination of patients with lower-extremity sarcoma. Pediatr Blood Cancer 2007;49:183–189. © 2006 Wiley-Liss, Inc.
Pediatric Blood & Cancer 07/2007; 49(2):183 - 189. · 1.89 Impact Factor
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ABSTRACT: Advances in systemic and local therapies have improved outcomes for patients with the Ewing sarcoma family of tumors (ESFT). As new treatments are developed, a critical review of data from past treatment eras is needed to identify clinically relevant risk groups.
The authors reviewed the records of 220 patients with ESFT who were treated on protocols at St. Jude Children's Research Hospital from 1979 to 2004. Two treatment eras were defined. Factors predictive of outcome were analyzed to identify distinct risk groups.
The median age at diagnosis was 13.7 years (range, 1.1-25.2 years). Metastatic disease was associated with tumors measuring >8 cm (P = .002) and axial location (P = .014). The 5-year overall survival (OS) estimate (63.5% +/- 3.5%) did not appear to differ by protocol. Tumor stage and size were found to be the only independent predictors of outcome. Treatment era and type of local control therapy were found to influence the outcome of patients with localized disease. Four risk groups were defined: favorable risk (age <14 years with localized, nonpelvic tumors), intermediate risk (localized, age >/=14 years, or pelvic tumors), unfavorable-pulmonary (isolated lung metastases), and unfavorable-extrapulmonary (extrapulmonary metastases). The 5-year OS estimates for these groups were 88.1% +/- 4.4%, 64.9% +/- 5.2%, 53.8% +/- 9.4%, and 27.2% +/- 7.3%, respectively (P < .001). The incidence of therapy-related leukemia was significantly higher during the second treatment era, when more intensified regimens were used (6.1% +/- 2.7% vs 0% +/- 0%; P = .005).
Risk stratification schemes such as this should be used to prospectively evaluate novel risk-based therapies. Studies of biologic pathways may help to refine this model.
Cancer 07/2007; 110(2):375-84. · 4.77 Impact Factor
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ABSTRACT: Pediatric oncology patients who have undergone placement of multiple central venous catheters may have thrombosis or stenosis in the upper venous system. The purpose of this study was to identify factors that predict venous thrombosis or stenosis and to evaluate the role of Doppler ultrasonography in assessing the upper venous system of pediatric patients requiring multiple central vascular catheters.
The medical records of eligible patients were reviewed with regard to demographics, primary disease, type of catheter, duration of previous central venous access, association with infection, operative notes, and Doppler ultrasonographic findings.
Our evaluation criteria were met in 50 cases (47 patients). In 10 cases, Doppler ultrasonography revealed abnormality in the upper venous system. Patient demographics, primary disease, type of catheter, duration of previous central venous access, or association with infection were not found to significantly predict the abnormality in the upper venous system. Placement of central venous access device was performed without difficulty when the site of placement was chosen on the basis of ultrasonographic findings.
Doppler ultrasonography is useful in diagnosing thrombosis or stenosis in asymptomatic pediatric patients requiring placement of multiple central venous catheters.
Pediatric Blood & Cancer 06/2007; 48(5):527-31. · 1.89 Impact Factor
