Long-Term Follow-up of High-Dose Chemotherapy with Autologous Stem Cell Transplantation in Children and Young Adults with Metastatic or Relapsed Ewing Sarcoma: A Single-Institution Experience.

Forty-seven patients with metastatic disease at diagnosis or recurrent Ewing sarcoma (EWS) received high-dose chemotherapy (HDC) followed by tandem (n = 20, from February 13, 1997, to October 24, 2002) or single (n = 27, from October 1, 2004, to September 5, 2018) autologous hematopoietic stem cell transplantation (ASCT). To our knowledge, this is the largest single-institution study with sustained long-term follow-up exceeding 10 years. All patients who underwent single ASCT received a novel conditioning regimen with busulfan, melphalan, and topotecan. The overall survival (OS) and disease-free survival (DFS) were 46% and 37% at 10 years and 42% and 37% at 15 years, respectively. Disease status at transplant and the time to disease relapse prior to ASCT were identified as important prognostic factors in OS, DFS, and risk of relapse. At 10 years, patients who underwent transplantation in first complete response (1CR) had an excellent outcome (OS 78%), patients in 1CR/second complete response (2CR)/first partial response (1PR) had an OS of 66%, and patients at third or more complete response, second or more partial response, or advanced disease had an OS of 26%. Ten-year OS for patients without a history of relapse, with late relapse (≥2 years from diagnosis), or with early relapse (<2 years from diagnosis) was 75%, 50%, and 18%, respectively. Selected patients in 1CR, 2CR, 1PR, and with late relapse had excellent, sustained 10- and 15-year OS and DFS.

Is claviculo pro humeri of value for limb salvage of pediatric proximal humerus sarcomas?

BACKGROUND: There are several options for proximal humerus reconstruction in young children after resection of a malignant tumor and no one technique has been definitively shown to be superior to others, leaving the decision to surgeon and patient choice. Claviculo pro humeri (CPH) is a biologic reconstruction of the proximal humerus using the patient's ipsilateral clavicle as a rotational osseous flap. CPH represents a potential option for this complicated clinical problem in very young children, but little is known about it because the indications for its use are so uncommon. QUESTIONS/PURPOSES: The purposes of this study were to (1) assess the oncologic outcomes of CPH at a minimum of 2 years in a small series of patients; (2) elicit the complications associated with this procedure; and (3) show the Musculoskeletal Tumor Society (MSTS) functional score of these patients. METHODS: Four patients (average age, 5 years 11 months; range, 4 years 5 months to 8 years 9 months at the time of surgery) were treated with CPH for reconstruction after resection of a proximal humerus sarcoma; this represented all of the patients treated with this approach for this problem between January 2008 and April 2011 at one institution. During this period, the general indications for using CPH were the need to reconstruct a proximal humerus defect in a child younger than 10 years of age. During this time, CPH was used for all patients treated for proximal humerus sarcomas meeting these criteria. Patient demographics, diagnosis, tumor size and extent, operative details, radiographs and MRIs, complications, and functional outcomes were assessed. RESULTS: All are alive with no evidence of disease at a minimum followup of 31 months (average, 43 months; range, 31-58 months). Two patients developed nonunion and underwent revision surgery. Osseous union and a stable neoshoulder articulation were ultimately obtained in all patients. Limited shoulder motion was the only functional deficit noted with forward elevation ranging between 30° and 90°. MSTS functional scores were excellent with a range of 87% to 90%. CONCLUSIONS: This is a rarely used procedure in North America but we achieved functional limb salvage in all four patients. Consistent with prior literature, nonunion was the major complication in this series. The two nonunions were successfully treated without interruption of chemotherapy or significant bone graft donor site morbidity. Based on these results, the authors suggest that this procedure is a reasonable reconstruction option to consider after proximal humerus resection in patients younger than 10 years of age. Further followup will be required to assess long-term results and to determine how this procedure compares with the alternatives. LEVEL OF EVIDENCE: Level IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

A dual-center review of compressive osseointegration for fixation of massive endoprosthetics: 2- to 9-year followup.

BACKGROUND: Aseptic failure of massive endoprostheses used in the reconstruction of major skeletal defects remains a major clinical problem. Fixation using compressive osseointegration was developed as an alternative to cemented and traditional press-fit fixation in an effort to decrease aseptic failure rates. QUESTIONS/PURPOSES: The purpose of this study was to answer the following questions: (1) What is the survivorship of this technique at minimum 2-year followup? (2) Were patient demographic variables (age, sex) or anatomic location associated with implant failure? (3) Were there any prosthesis-related variables (eg, spindle size) associated with failure? (4) Was there a discernible learning curve associated with the use of the new device as defined by a difference in failure rate early in the series versus later on? METHODS: The first 50 cases using compressive osseointegration fixation from two tertiary referral centers were retrospectively studied. Rates of component removal for any reason and for aseptic failure were calculated. Demographic, surgical, and oncologic factors were analyzed using regression analysis to assess for association with implant failure. Minimum followup was 2 years with a mean of 66 months. Median age at the time of surgery was 14.5 years. RESULTS: A total of 15 (30%) implants were removed for any reason. Of these revisions, seven (14%) were the result of aseptic failure. Five of the seven aseptic failures occurred at less than 1 year (average, 8.3 months), and none occurred beyond 17 months. With the limited numbers available, no demographic, surgical, or prosthesis-related factors correlated with failure. CONCLUSIONS: Most aseptic failures of compressive osseointegration occurred early. Longer followup is needed to determine if this technique is superior to other forms of fixation.

Extra-abdominal desmoid tumors associated with familial adenomatous polyposis.

Extra-abdominal desmoid tumors are a significant cause of morbidity in patients with familial adenomatous polyposis syndrome. Understanding of the basic biology and natural history of these tumors has increased substantially over the past decade. Accordingly, medical and surgical management of desmoid tumors has also evolved. This paper analyzes recent evidence pertaining to the epidemiology, molecular biology, histopathology, screening, and treatment of extra-abdominal desmoid tumors associated with familial adenomatous polyposis syndrome.

At-risk populations for osteosarcoma: the syndromes and beyond.

Osteosarcoma is the most common primary malignancy of bone. Most cases are sporadic without a known genetic or environmental cause. Heritable genetic predisposition syndromes are associated with a small percentage of osteosarcomas. Study of these rare disorders has provided insight into the molecular pathogenesis of osteosarcoma. Screening of at-risk families and surveillance of affected individuals for these syndromes may permit earlier diagnosis and more effective treatment of osteosarcoma in these populations. This paper reviews the genetic and clinical features of the known osteosarcoma predisposition syndromes.

A double-blind, prospective, randomized controlled trial comparing highly cross-linked and conventional polyethylene in primary total hip arthroplasty.

Highly cross-linked polyethylene was developed to reduce articular bearing wear in total hip arthroplasty. In vitro studies have shown reduced wear in comparison with conventional polyethylene. A double-blind, prospective, randomized trial was performed comparing highly cross-linked and conventional polyethylene in 119 patients. The primary outcome variables were linear, 3-dimensional, and volumetric wear as determined by analysis of digitized radiographs using validated wear measurement software. Linear, 3-dimensional, and volumetric wear rates were significantly less in the highly cross-linked polyethylene group between 6 months and 4 years postoperatively (P < .05). The data presented here show that highly cross-linked polyethylene reduces short-term polyethylene wear. The intermediate and long-term clinical results of highly cross-linked polyethylene remain unknown pending further follow-up.

Effects of increased surgical volume on total knee arthroplasty complications.

The effect of increased total knee arthroplasty (TKA) surgical volume on the incidence of knee complications within a single surgeon's practice was determined. Data were collected prospectively on 600 primary TKAs. Major complications required knee reoperation. Minor complications were wound concerns requiring increased surveillance or oral antibiotics. Twelve major (2.0%) and 40 minor (6.7%) complications occurred at minimum 2-year follow-up. Surgical volume increased from 57 to 150 procedures per 6 months through the course of the study. Multivariate analysis determined that increased surgical volume was associated with lower rates of both major and minor complications, adjusted odds ratio 0.62 (95% confidence interval [CI], 0.40-0.96) and 0.56 (95% CI, 0.43-0.73), respectively. For each additional 50 procedures performed within a 6-month interval, major complications were reduced by 38% (P = .03), and minor complications were reduced by 44% (P < .0001). All complications were significantly decreased when the surgeon was performing 112 +/- 44 TKAs compared with 85 +/- 49 TKAs per 6-month interval.

The use of arthroscopy in the athlete with knee osteoarthritis.

Arthroscopy is an important technique in the diagnosis, classification, and treatment of the athlete with osteoarthritis (OA). Reliability of the current classification systems improves with training and experience. Arthroscopy remains superior to imaging in the diagnosis of OA. Arthroscopic lavage and debridement provide benefit in a significant percentage of patients. The reasons for improvement are not fully defined. Arthroscopic treatment of OA is not curative, and results deteriorate with time. Variability in the use of medical management, arthroscopy, osteotomy, and arthroplasty remains among different practitioners. Indications for arthroscopy require further clarification based upon empiric evidence.