Treatment of chest wall sarcomas: a single-institution experience over 20 years.

OBJECTIVE: To evaluate the impact on the outcome of radiation therapy and chemotherapy in the treatment of localized chest wall sarcomas. METHODS: A retrospective review of 65 patients with stage IIB and III chest wall sarcomas seen over 20 years at the University of Washington Medical Center. Overall and disease-free survival outcomes were analyzed on the basis of the treatment received: surgery alone; surgery and radiation therapy; surgery and chemotherapy; and surgery, radiation therapy, and chemotherapy. RESULTS: Disease recurrence was observed in 32.3%, and, of these, 33.3% were local only, 42.9% distant only, and 23.8% were both local and distant. As compared with surgery alone, disease-free survival at both 5 and 10 years improved by 92% with the addition of radiation therapy to surgery, by 82% with the addition of chemotherapy to surgery, and by 89% and 90% with the addition of both chemotherapy and radiation therapy at 5 and 10 years, respectively. Overall survival also improved with radiation therapy, chemotherapy, or the combination of both, with the greatest improvement seen in patients treated with both radiation therapy and chemotherapy, which showed reduced mortality at 5 and 10 years of 49% and 45%, respectively, compared with surgery alone. CONCLUSIONS: The addition of radiation therapy, chemotherapy, or both to surgery in localized chest wall sarcoma improves outcome and should strongly be considered for patients with acceptable comorbidities. A trend toward improvement in overall survival was also shown with the use of radiation therapy and chemotherapy. As chest wall sarcomas are rare and histologically heterogenous, larger studies are necessary to elucidate which histologic subtypes may gain the most benefit from radiation therapy and chemotherapy.

Benign parosteal osteochondromatous proliferation of the hand originally diagnosed as osteochondroma: a report of two cases and review.

Bizarre parosteal osteochondromatous proliferation (BPOP) is a rare cartilaginous neoplasm that often presents in the long bones of the hands and feet. BPOP is a benign but locally aggressive fibro-osseous mass that has striking clinical, radiographic, and histologic similarities with osteochondroma. Differentiating between the two lesions is important as BPOP often requires more extensive surgical resection and has a higher recurrence rate compared to osteochondroma. This report presents two cases of BPOP where initial clinical diagnosis of osteochondroma was made even after appropriate imaging and histologic samples were evaluated. This report reviews clinical, radiographic, and histologic characteristics that can differentiate between the two lesions.

Megaprosthesis versus Condyle-sparing intercalary allograft: distal femoral sarcoma.

UNLABELLED: Although functionally appealing in preserving the native knee, the condyle-sparing intercalary allograft of the distal femur may be associated with a higher risk of tumor recurrence and endoprosthetic replacement for malignant distal femoral bone tumors. We therefore compared the risk of local tumor recurrence between patients in these two types of reconstruction groups. We retrospectively reviewed 85 patients (mean age, 22 years; range, 4-82 years), 38 (45%) of whom had a condyle-sparing allograft and 47 (55%) of whom had endoprostheses. The minimum followup for both groups was 2 years (mean, 7 years; range, 2-19 years). Local recurrences occurred in 11% (five of 47) of the patients having implants versus 18% (seven of 38) of the patients having allografts. Using time to local recurrence as an end point, the Kaplan-Meier survivorship of the implant group was similar to that of the condyle-sparing allograft group at 2, 5, and 10 years (93% versus 87% at 2 years, 87% versus 81% at 5 years, and 87% versus 81% at 10 years, respectively). The condyle-sparing allograft procedure offers the potential advantage of retaining the native knee in a young patient population while incurring no greater risk of local recurrence as those offered the endoprosthetic procedure. LEVEL OF EVIDENCE: Level IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

Oncologic approaches to pediatric limb preservation.

Preservation of limb function in the pediatric oncology patient is uniquely challenging. Treatment must be strictly prioritized in terms of the patient's life, the limb, its function, length equalization, and cosmetic appearance. At the same time, social, socioeconomic, and cultural factors must be understood and respected to achieve the most advantageous outcome for both the patient and family. Given these considerations, as well as the relative rarity of many oncologic diagnoses and the myriad of presentation scenarios, drafting generalized treatment recommendations is difficult. Instead, orthopaedic intervention in the care of children and young adults with oncologic conditions must be individualized, with the broad goal being optimization of limb function rather than rigid advocacy of limb salvage.

Musculoskeletal manifestations of Hurler syndrome: long-term follow-up after bone marrow transplantation.

Bone marrow transplantation (BMT) is effective in ameliorating many of the clinical manifestations of Hurler syndrome. However, long-term data on the natural history of the musculoskeletal disorders of Hurler syndrome after BMT are limited. The authors report the orthopaedic outcomes in seven patients with Hurler syndrome who were successfully engrafted between 1990 and 1999, and have been followed for a mean of 7.6 years since transplantation. Medical records, clinical examinations, and imaging studies were reviewed to assess the development and management of hip dysplasia, genu valgum, spine abnormalities, hand abnormalities, and joint range of motion. BMT does not appear to alter the natural history of the musculoskeletal disorders in Hurler syndrome, although there may be a beneficial effect on upper extremity joint mobility.