RESUMO
BACKGROUND: Bleeding episodes in severe hemophilia may occur more frequently and spontaneously after mild trauma or daily activities. An inadequate treatment of that bleeding in hemophilia may result in pseudotumor, usually in the muscle adjacent to the bone. We reported haemophilic pseudotumor treated with various surgical interventions. METHODS: This study was conducted inthe Department of Orthopaedic and Traumatology at a government hospital over a period of 7 years(2010 -2017). Patients Perioperative management was done in accordance with the Integrated Hemophilia Team of our institution protocol.Diagnosis and management planning of hemophilic pseudotumor was confirmed via Integrated HemophiliaTeam meeting. After the surgery, all patients were asked to come for routine follow up. RESULTS: We reported six Haemophilia-A patients with pseudotumor in the pelvis, proximal femur and lower leg. One case in pelvic bone underwent hematoma evacuation, acetabular reconstruction using the Harrington procedure, and total hip arthroplasty.Two cases, a case in the proximal femur and another case in the distal fibula, were treated with amputation, other two cases, one was soft tissue psedotumor in the pelvic region and was treated by hematoma evacuation, and the remaining casewas managed with wide excision and followed by defect closure. CONCLUSION: Surgery is a preferable treatment for pseudotumors that have been present for years.It's associated with the best outcomes especially when selected as the primary line ofwith preventable and manageable bleeding complication. As previously published by many authors, this paper confirms that surgical excision is the treatment of choice but should only be carried out in major hemophilia centers by a multidisciplinary surgical team.
RESUMO
BACKGROUND: Nasopharyngeal carcinoma (NPC) is endemic in Indonesia and 20% of the patients are diagnosed before the age of 31. This study evaluates presentation and treatment outcome of young patients in Jakarta, in a tertiary referral centre. METHODS: Forty-nine patients under the age of 31, diagnosed with NPC between July 2004 and January 2007, were evaluated. Baseline data included histological type, stage of disease and presenting symptoms. We intended to follow all patients after diagnosis to reveal treatment outcome and overall survival (OS). RESULTS: All but two patients had advanced stage disease (94%), 7 (14%) had distant metastasis. The median interval between start of complaints and diagnosis was 9 months. Forty-two patients were planned for curative intent treatment. Eleven patients (26%) never started treatment, 2 patients did not complete treatment and 3 patients did not return after finishing treatment. Four patients died before radiation could start. Three patients died within 4 months after treatment. Nine patients (21%) had a complete response. Due to the high number of patients who were lost to follow-up (LFU), OS was analyzed as follows: a best-case (patients censored at last contact) and a worst-case scenario (assuming that patients who did not finish treatment or had disease at last contact would have died). The 2-year OS for patients without distant metastases was 39-71%. CONCLUSION: Treatment outcome for young patients with NPC in this institute was poor. Improvement can be achieved when NPC is diagnosed at an earlier stage and when there is better treatment compliance.