Malignant haemangioendothelioma of bone in a HbSC disease patient--a case report
W A Shokunbi1, O B Campbell, J O Ogunbiyi
1Department of Haematology, University College Hospital, Ibadan, Nigeria.
We report here a 35-year-old man with sickle cell disease (SCD), who presented in 1989 with pain in the (R) hip of 7 years duration and swelling of the (R) calf of 3 months duration. Clinical examination revealed a hard tender mass in the (R) calf. Histology of the (R) calf mass revealed haemangioendothelioma (HE), similar to the histology of the (R) iliac bone mass obtained in another institution previously. He was treated with external radiotherapy with the 1.25 megavoltage beam to antero-posterior fields of the (R) hemipelvis and (R) calf, with good response. Chemotherapy was subsequently administered using 6 cycles of VAC regime. The patient remained in remission for 12 months. In 1991 he had lymphoedema of (R) lower limb and received further radiotherapy and chemotherapy after an isotope bone scan had revealed disease activity in the (R) hemipelvis, (R) femur and (L) upper tibia. He responded again with complete regression of the lymphoedema and remained well until April 1993 when the lymphoedema recurred. He died while being evaluated for further treatment. Although there is no evidence to suggest that SCD confers any protection from development of neoplasms, the co-existence of SCD with a neoplasm is not common. We consider the occurrence of HE of bone, a rare malignancy, in a HbSC patient worthy of reporting.
We report here a 35-year-old man with sickle cell disease (SCD), who presented in 1989 with pain in the (R) hip of 7 years duration and swelling of the (R) calf of 3 months duration. Clinical examination revealed a hard tender mass in the (R) calf. Histology of the (R) calf mass revealed haemangioendothelioma (HE), similar to the histology of the (R) iliac bone mass obtained in another institution previously. He was treated with external radiotherapy with the 1.25 megavoltage beam to antero-posterior fields of the (R) hemipelvis and (R) calf, with good response. Chemotherapy was subsequently administered using 6 cycles of VAC regime. The patient remained in remission for 12 months. In 1991 he had lymphoedema of (R) lower limb and received further radiotherapy and chemotherapy after an isotope bone scan had revealed disease activity in the (R) hemipelvis, (R) femur and (L) upper tibia. He responded again with complete regression of the lymphoedema and remained well until April 1993 when the lymphoedema recurred. He died while being evaluated for further treatment. Although there is no evidence to suggest that SCD confers any protection from development of neoplasms, the co-existence of SCD with a neoplasm is not common. We consider the occurrence of HE of bone, a rare malignancy, in a HbSC patient worthy of reporting.
