Busulfan/cyclophosphamide/melphalan

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Inflammatory myofibroblastic tumour: case report A 15-year-old boy developed inflammatory myofibroblastic tumour (IMT)after treatment with busulfan, cyclophosphamide and melphalan as induction chemotherapy for bone marrow transplantation. The boy was hospitalised due to several weeks of isolated persistent serotine fever (current presentation), which was unresponsive to empirical treatment with unspecified antibiotics. At the age of 8 years, he was diagnosed with acute myeloid leukaemia, and had undergone bone marrow transplantation from HLA identical brother. The induction therapy included busulfan, cyclophosphamide and melphalan [dosages and routes not stated]. Subsequent haematological workup was free from relapse or transplant rejection. At the time of current presentation, his physical examination and blood parameters were noted as normal. Later, a transthoracic echocardiography revealed a dishomogeneous mass of 52×25mm in the main pulmonary artery. The origin of mass was from the left pulmonary valve cusp, causing mild regurgitation. His biventricular function was normal along with normal cardiac anatomy. Angio-CT scan confirmed presence of a mass of 25×15mm at the origin of the main pulmonary artery with multiple distal defects of perfusion in the lobar pulmonary arteries. Several parenchymal nodules were also noted in both the lungs whose aetiology was not clear. After multidisciplinary evaluation, he underwent surgical excision of the mass in main pulmonary artery on cardiopulmonary bypass on beating heart. Histopathological examination of the resected mass was consistent for IMT. The mass also showed an ALK mutation. Post operative course was uneventful, and he was discharged on postoperative day 6 with no echocardiographic evidence of any residual cardiac mass. Two months after the surgery his CT and PET scan were completely normal. However, due to chances of recurrence he was initiated on crizotinib. Five months after the surgery, he again presented with haemoptysis. Second angio-CT scan showed increasing multiple perfusion defects in the right lobar and sublobar pulmonary arteries, and parenchymal nodules remains unchanged. He was initiated with heparin to prevent further thrombosis. Subsequently, a second elective surgery was performed and the mass was removed along with suspicious right pulmonary nodule. Both the masses were confirmed for IMT. He was discharged on postoperative day 11. After two months of discharge, he was free from any other symptoms, and the crizotinib therapy was continued. Ponzoni M, et al. Recurrent pulmonary embolization of inflammatory myofibroblastic tumor: a case report. Cardiovascular Pathology 50: 107270, Feb 2021. Available from: 803504905 URL: http://doi.org/10.1016/j.carpath.2020.107270

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Reactions 3 Oct 2020 No. 1824

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