A case with extraosseous Ewing's sarcoma: A late effect related to bone marrow transplantation for thalassemia or a component of a familial cancer syndrome?

Allogeneic bone marrow transplantation has proved to be a radical form of c ure in patients with beta-thalassemia major who have a human leukocyte anti gen identical donor Although malignant neoplasms are serious late complicat ions of bone marrow transplantation, very few reports describing the develo pment of malignant tumors after allografting for thalassemia appeared in th e literature. A case is presented here of extraosseous Ewing's sarcoma that developed 8 years after allogeneic bone marrow transplantation performed f or beta-thalassemia major. The phenotypic features of the patient's family fullfill the criteria for Li-Fraumeni syndrome. The patient was treated wit h chemotherapy and radiotherapy and died with recurrent disease. To the aut hors' knowledge, this is the first case of extraosseous Ewing's sarcoma aft er bone marrow transplantation for thalassemia. The possible contribution o f transplantation procedure and the genetic factors as well as the primary genetic hemoglobinopathy to the development of this malignant tumor are dis cussed.