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?
Km. Uysal et al., 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?, PED HEM ONC, 17(5), 2000, pp. 415-419
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.