Recommendations for the use of pegylated interferon-α in the treatment of classical myeloproliferative neoplasms.

The classical myeloproliferative neoplasms (MPN) are uncommon clonal haemopoietic malignancies characterised by excessive production of mature blood cells. Clinically, they are associated with thrombosis, haemorrhage, varying degrees of constitutional disturbance and a risk of progression to myelofibrosis or acute myeloid leukaemia. Many of the disease manifestations may be ameliorated by treatment with interferon-α (IFN), but its use in Australian MPN patients has been limited due to the inconvenience of frequent injections and side-effects. The pegylated form of IFN is a long-acting preparation, which is better tolerated, and its Pharmaceutical Benefits Scheme listing is likely to lead to increased usage. We review the literature on risks and benefits of IFN treatment for MPN, suggest criteria for patient selection in each of these diseases and discuss strategies to manage the side-effects of pegylated IFN.

Limb salvage in osteosarcoma using autoclaved tumor-bearing bone.

BACKGROUND: Tumor prostheses currently give the best short- and medium-term results for limb-salvage reconstruction procedures in the treatment of bone tumors. However, in developing countries, the cost of a tumor prosthesis is beyond the reach of much of the population. We report the use of autoclaved tumor-bearing bone in 10 patients, as an affordable alternative to the use of prostheses. METHODS: This is a case series of 10 patients (mean age 19 years) with osteosarcoma who were treated at our hospital from 1998 to 2008, and followed up for a mean of 35 months (range 14 to 8). The femur was involved in six cases, the humerus in three cases, and the ulna in one case. The mean length of the autoclaved bone was 150 mm (range 60-210). RESULTS: Bone union occurred in seven patients over an mean duration of 12 months (range 8-17). Three patients had non-union. Two of these had associated implant failure, with one of them also developing chronic infection, and the third is still being followed up. Two other patients had local recurrence. CONCLUSION: The use of autoclaved tumor grafts provides an inexpensive limb-salvage option without sacrificing appropriate oncologic principles. A painless and stable limb is achievable, and the use of this technique can be further refined.

Extraskeletal myxoid chondrosarcoma of the thigh in a child: a case report.

Extraskeletal myxoid chondrosarcoma (EMC) is aggressive in children. The condition in children differs to that in adults and to skeletal myxoid chondrosarcoma. We report on a 9-year-old girl with EMC in her left thigh. She underwent above-knee amputation. Five months later, a small mass was noted at the right lower lobe of the lung. The patient underwent one course of ifosfamide, carboplatin, and etoposide chemotherapy, followed by resection of the mass and 8 more courses of chemotherapy. At the 2-year follow-up, she was in remission radiologically.

Functional and oncologic outcomes after excision of the total femur in primary bone tumors: Results with a low cost total femur prosthesis.

BACKGROUND: The extent of tumor may necessitate resection of the complete femur rarely to achieve adequate oncologic clearance in bone sarcomas. We present our experience with reconstruction in such cases using an indigenously manufactured, low-cost, total femoral prosthesis (TFP). We assessed the complications of the procedure, the oncologic and functional outcomes, and implant survival. MATERIALS AND METHODS: Eight patients (four males and four females) with a mean age of 32 years, operated between December 2003 and June 2009, had a TFP implanted. The diagnosis included osteogenic sarcoma (5), Ewing's sarcoma (1), and chondrosarcoma (2). Mean followup was 33 months (9-72 months) for all and 40 months (24-72 months) in survivors. They were evaluated by Musculoskeletal Tumor Society score, implant survival as well as patient survival. RESULTS: There was one local recurrence and five of seven patients are currently alive at the time of last followup. The Musculoskeletal Tumor Society score for patients ranged from 21 to 25 with a mean of 24 (80%). The implant survival was 88% at 5 years with only one TFP needing removal because of infection. CONCLUSIONS: A TFP in appropriately indicated patients with malignant bone tumors is oncologically safe. A locally manufactured, cost-effective implant provided consistent and predictable results after excision of the total femur with good functional outcomes.

Initial symptoms and delayed diagnosis of osteosarcoma around the knee joint.

PURPOSE: To identify initial symptoms of osteosarcoma around the knee joint. METHODS: Records of 19 men and 11 women aged 9 to 34 (mean, 17) years with osteosarcoma around the knee joint were reviewed. Each patient was interviewed (for a mean of 1.5 hours), and a detailed history of symptom onset recorded. RESULTS: In all patients, the first symptom was pain, which was more pronounced on weight bearing in 16 (53%) of them and was worse at night in 11 (37%). Swelling was noticed in 28 patients (a mean of 7 weeks after pain onset). 16 (53%) of the patients had a history of minor trauma around the time of symptom onset. 20 (67%) of the patients had a limp, in 9 of whom it was not associated with pain on weight bearing; only 2 (7%) had a pathological fracture. The mean interval from the onset of symptoms (pain) to presentation was 10 (range, 0-49) weeks. The mean interval from presentation to radiography was 3 (range, 0-20) weeks; in 8 patients this interval exceeded 3 weeks despite a noticeable swelling. The mean interval from radiography to referral to our hospital was 2 (range, 0-14) weeks. Together with the 2 weeks for diagnostic workup and biopsy, the mean total delay was 17 (range, 4-55) weeks. CONCLUSION: Physicians should be aware of the symptoms of osteosarcoma and promptly refer patients to tumour centres, especially adolescents, those who present with pain that may be worse at night and on weight bearing, and those in whom a swelling and/or a limp is evident.