Odion Binitie

Outcome of lower-limb preservation with an expandable endoprosthesis after bone tumor resection in children.

BACKGROUND The optimal treatment of malignant pediatric lower-extremity bone tumors is controversial. Expandable endoprostheses allow limb preservation, but the revision rate and limited function are considered barriers to their use. This study investigated the functional, emotional, and oncologic outcomes of thirty-eight patients treated with an expandable endoprosthesis. METHODS A retrospective chart review was performed, and surviving patients were asked to complete the Musculoskeletal Tumor Society (MSTS) outcomes instrument and the Pediatric Outcomes Data Collection Instrument (PODCI). Additional data including the range of hip and knee motion, limb-length discrepancy, and total lengthening were also obtained. RESULTS Thirty-eight patients were treated with an expandable endoprosthesis, and twenty-six of these patients were alive at the time of the study. The mean global MSTS score was 26.1, and the mean global PODCI score was 85.8. The mean emotional acceptance and happiness subscores were high. The mean sagittal-plane hip motion in patients who had undergone replacement of the proximal aspect of the femur was 103°. The mean knee motion in patients who had undergone replacement of the proximal aspect of the femur, the distal aspect of the femur, or the proximal aspect of the tibia was 127°, 97°, and 107°, respectively. The mean lengthening at the time of skeletal maturity was 4.5 cm, and the mean limb-length discrepancy was 0.7 cm. Forty-two percent of the patients experienced complications, with ten patients requiring prosthesis revision and two of these patients requiring amputation. CONCLUSIONS Current technology does not offer a single best reconstruction option for children. Previous studies and the present series have indicated that physical and emotional functioning in patients treated with an expandable endoprosthesis are good but that complication rates remain high. Amputation and rotationplasty are alternative treatments if patients and their families are amenable to these procedures. The literature supports no single superior treatment among these three options with regard to physical or emotional health.

Osteosarcoma in Pediatric Patients and Young Adults: A Single Institution Retrospective Review of Presentation, Therapy, and Outcome

Background. Little is known about how cumulative chemotherapy delivery influences the poorer outcome observed in young adult (YA, 18–40 years) versus pediatric (<18 years) osteosarcoma patients. Here, we retrospectively examined differences in presentation, therapy, including cumulative chemotherapy dose, and outcome in YA and pediatric patients. Methods. We reviewed 111 cases of high-grade osteosarcoma at Moffitt Cancer Center between 1988 and 2012. Presentation factors, therapies, and survival were compared between YA and pediatric cohorts. Results. The cohorts were equivalent with respect to metastatic status, gender, tumor size, tumor site, and histological subtype. We found that the YA patients tended to have poorer histologic response to neoadjuvant chemotherapy measured by necrosis with 55% and 35% of pediatric versus YA patients responding favorably (P = 0.06). Only 39% of YA patients achieved the typical pediatric dose of methotrexate, doxorubicin, and cisplatin. These patients had a 3-year EFS of 76% (CI 53–100%) versus 47% (CI 26–69%; P = 0.09) in those who received less chemotherapy. Conclusion. Age continues to be a prognostic factor in osteosarcoma. Our study suggests that presentation factors are not associated with prognosis, while poorer response to chemotherapy and lower cumulative dose of chemotherapy delivered to YA patients may contribute to poorer outcomes.

Do Surgical Margins Affect Local Recurrence and Survival in Extremity, Nonmetastatic, High-grade Osteosarcoma?

AbstractBackground Long-term survival for all patients with osteosarcoma using current aggressive adjuvant chemotherapy and surgical resection is between 60% and 70%. In patients who present with nonmetastatic, high-grade extremity osteosarcoma of bone, limb salvage surgery is favored, when appropriate, over amputation to preserve the limb, because limb salvage may lead to a superior quality of life compared with amputation. However, concern remains that in the attempt to preserve the limb, close or microscopically positive surgical margins may have an adverse effect on event-free survival.Questions/purposes(1) Does a positive or close surgical margin increase the likelihood of a local recurrence? (2) Does a positive or close surgical margin adversely affect the development of metastatic disease? (3) What is the relationship of surgical margin on overall survival?MethodsWith institutional review board approval, we retrospectively evaluated 241 patients treated at our institution between 1999 and 2011. Exclusion criteria included nonextremity locations, metastatic disease at initial presentation, low- or intermediate-grade osteosarcoma, treatment regimens that did not follow National Comprehensive Cancer Network (NCCN) guidelines, incomplete medical records, and any part of treatment performed outside of Moffitt Cancer Center or All Children’s Hospital. Fifty-one patients were included in the final analysis, of whom 31 (61%) had followup data at a minimum of 2 years or whose clinical status was known but had died before 2 years of followup. Margin status was defined as (1) microscopically positive; (2) negative ≤ 1 mm; and (3) negative > 1 mm. Margin status, histologic response (tumor percent necrosis), type of osteosarcoma, type of surgery, presence of local recurrence, metastatic disease, and overall survival were recorded for each patient. The mean age was 22 years (range, 12–74 years) and the mean followup was 3 years (range, 0.1–14 years). Margin status was positive in 10% (five of 51), negative ≤ 1 mm 26% (13 of 51), and negative > 1 mm 65% (33 of 51).ResultsLocal recurrence was noted to be 14% (seven of 51) at 3.4 years. After controlling for relevant confounding variables, the presence of a positive margin compared with a negative margin > 1 mm was the only independent predictor of local recurrence (hazard ratio [HR], 8.006; 95% confidence interval [CI], 1.314–48.781; p = 0.0241). At a mean of 3.4 years, 29% (15 of 51) of the patients developed metastatic disease with no difference with the numbers available in the probability of developing metastatic disease among the three margin groups (p = 0.614). Overall survival at 3.8 years was 75% (38 of 51). After controlling for relevant confounding variables, we found that patients with positive margins were more likely to die from disease than those with negative margins (HR, 6.26; 95% CI, 1.50–26.14; p = 0.0119); no other independent predictors of survival were identified.ConclusionsWith the numbers of patients we had, we observed that patients with extremity, nonmetastatic, high-grade osteosarcoma who had positive margins showed a higher probability of local recurrence in comparison to those with negative surgical margins. Given that positive margins appear to be associated with poorer survival in patients with high-grade osteosarcoma of the extremities, surgeons should strive to achieve negative margins, but larger studies are needed to confirm these findings.Level of EvidenceLevel III, therapeutic study.

Chondroblastoma of bone in the extremities: a multicenter retrospective study.

BACKGROUND Chondroblastoma is a rare benign cartilage tumor that commonly occurs in children and adolescents. This study was designed to review the epidemiologic characteristics and outcomes of surgical management in a large series of patients with extremity chondroblastoma. METHODS We performed a multicenter retrospective analysis of 199 patients with extremity chondroblastoma. Clinical data, radiographic images, histological findings, treatment, and outcome were analyzed. RESULTS There were 145 male patients and fifty-four female patients with a mean age of 18.0 years. The most commonly involved bone was the proximal part of the tibia (fifty-five patients [27.6%]), followed by the proximal part of the femur (fifty-two patients [26.1%]) and the distal part of the femur (thirty-eight patients [19.1%]). Prior to presentation, 73.4% (146 of 199 patients) experienced pain. The mean duration of pain and other symptoms was 8.7 months. The physis was open in 25.7%, it was closing in 22.2%, and it was closed in 52.1% of the patients at the time of presentation. One hundred and twenty-six patients had at least twenty-four months of follow-up; their mean follow-up duration was 62.1 months (range, twenty-four to 190 months). Initial treatment was curettage for 119 patients (94.4%) and en bloc resection for seven patients (5.6%). The local recurrence rate was 5.0% after curettage and 0% after resection. The only significant factor related to recurrence was the location of the lesion in the proximal part of the humerus (p = 0.001). CONCLUSIONS Chondroblastoma occurs most frequently in the proximal part of the tibia and the proximal part of the femur with significant male predilection. In this series, recurrence was most frequent in the proximal part of the humerus. Our results suggest that curettage and bone-grafting provide favorable local control and satisfactory functional outcome for patients with this disease. LEVEL OF EVIDENCE Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Minimally invasive sacroiliac fixation in oncologic patients with sacral insufficiency fractures using a fluoroscopy-based navigation system.

Study Design Surgical technique article with retrospective case series. Summary of Background Data Sacral insufficiency fractures are commonly encountered in oncologic patients and constitute a cause for persistent lower back and pelvic pain. Objective The aim of this study is to describe the modified technique of navigated percutaneous sacroiliac (SI) fixation using multiple long screws per level that cross both SI joints and engage bilateral iliac bones; furthermore to evaluate its safety and efficacy in oncologic patients with sacral insufficiency fractures. Methods Six oncologic patients (3 male, 3 female, mean age: 58.8 y) with sacral insufficiency fractures who had undergone additional radiation therapy were operated with navigated percutaneous fixation. Two patients had failed preoperative sacroplasty and 1 had failed SI pinning. Eighteen SI screws were placed (15 at S1 level and 3 at S2). In the majority of cases the screws were long enough to engage bilateral ilium and sacrum. Additionally, 1 patient underwent percutaneous iliolumbar instrumentation and in 4 patients we performed concomitant sacroplasty or polymethylmethacrylate screw augmentation. The patients were followed for 18.8 months in average (range: 12-30 mo). Outcome was assessed using the Karnofsky Performance Status score (KPS), pain scale (0-10) and detailed neurologic examination. Results In 1 case, a revision of a screw was required due to radiculopathy. There was no perioperative morbidity or mortality. No hardware failure was encountered. There was significant improvement in KPS (P=0.04) and pain levels (P=0.02). Conclusions These preliminary data suggest that navigated percutaneous SI screw fixation is a safe and effective intervention in terms of pain control and performance status improvement in oncologic patients with sacral insufficiency fractures. For optimal fixation, multiple long screws that engage both iliac bones may be inserted through the S1 level in a safe manner. The technique may be combined with sacroplasty or closed posterior instrumentation to augment the screw fixation. Further investigation is needed to compare this technique with other treatment modalities.

Consensus and controversies regarding the treatment of rhabdomyosarcoma

Optimal treatment of rhabdomyosarcoma (RMS) requires multidisciplinary approach, incorporating chemotherapy with local control. Although current therapies are built on cooperative group trials, a comprehensive standard of care to guide clinical decision making has been lacking, especially for relapsed patients. Therefore, we assembled a panel of pediatric and adolescent and young adult sarcoma experts to develop treatment guidelines for managing RMS and to identify areas in which further research is needed. We created algorithms incorporating evidence‐based care for patients with RMS, emphasizing the importance of clinical trials and close integration of all specialties involved in the care of these patients.

Reconstruction After Tumor Resection in the Growing Child

Limb preservation surgery has gained acceptance as a viable alternative to amputation for the treatment of extremity bone tumors in the growing child. There are several options for reconstructing the potential loss of a physis and the defect created by tumor excision. Metallic endoprosthesis, massive allograft, and allograft-prosthesis composites have been described in the skeletally immature population. With the development of expandable prostheses, even those far from skeletal maturity may be candidates for limb salvage. However, improvements in the literature are needed, including reporting surgical and functional outcomes in a rigorous manner, specific to age, anatomic location, and reconstruction.

Surgical and Functional Outcomes After Limb-Preservation Surgery for Tumor in Pediatric Patients: A Systematic Review.

Background: Limb‐salvage surgery and segmental reconstruction for the treatment of lower extremity osseous tumors in the pediatric population have been described in the literature, but there is little consensus regarding the optimal surgical treatment for this patient population. Methods: A systematic review of the literature was performed to identify studies focusing on limb‐salvage procedures in pediatric patients who were managed with one of three reconstructions with use of a metallic endoprosthesis, allograft, or allograft‐prosthesis composite. Data were segregated according to the excised and reconstructed anatomical location (proximal part of the femur, total femur, distal part of the femur, proximal part of the tibia) and were collated to assess modes of failure and functional outcomes of each reconstruction type for each anatomic location. Results: Sixty articles met the inclusion criteria; all were Level‐IV evidence, primarily consisting of small, retrospective case series. Infection was a primary mode of failure across all reconstruction types and locations, whereas allograft reconstructions were susceptible to structural failure as well. The rate of failure in the pediatric population correlated well with previously published results for adults. The incidence of subsequent amputation was lower in the pediatric population (5.2%) than has been reported in adults (9.5%) (p = 0.013). Meaningful growth of expandable metallic endoprostheses was reported in the literature, with an overall rate of leg‐length discrepancy of 13.4% being noted at the time of the latest follow‐up. The Musculoskeletal Tumor Society (MSTS) questionnaire was the most consistently used outcome measure in the literature, with average scores ranging from 71.0% to 86.8%, depending on reconstruction type and anatomic location. Conclusions: The current state of the literature detailing the surgical and functional outcomes of segmental reconstruction for the treatment of pediatric bone tumors is limited to Level‐IV evidence and is complicated by under‐segregation of the data by age and anatomical location of the reconstruction. Despite these limitations, pediatric limb‐salvage surgery demonstrates satisfactory initial surgical and functional outcomes. Level of Evidence: Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Recurrence and survival analysis of resected soft tissue sarcomas of pelvic retroperitoneal structures.

The purpose is to determine the clinicopathologic factors related to survival and recurrence of primary resected pelvic soft tissue sarcomas (STS).

Expandable Total Humeral Replacement in a Child with Osteosarcoma

Case. A right-handed 8-year-old female patient presented with a conventional, high-grade osteosarcoma involving her right humerus; through-shoulder amputation was recommended. After consultation, total humerus resection with expandable, total humeral endoprosthesis reconstruction was performed with a sleeve to encourage soft-tissue ingrowth. At three-year follow-up she has received one lengthening procedure and her functional scores are excellent. Conclusion. Total humeral resection and replacement in the pediatric population are rare and although early reports of expandable total humeral endoprosthesis outcomes demonstrate high failure rates, this patients success indicates that expandable total humeral replacement is a viable option.