Department of Rheumatology and Histopathology, The Royal London Hospital, Bancroft Road, London E1 4DG, UK
Correspondence to: A. S. M. Jawad. E-mail: alismjawad1{at}hotmail.com
SIR, We report the case history of a Bangladeshi man with Paget's disease of bone who presented with pelvic pain, thrombocytopenia and pyrexia and was found to have developed angiosarcoma.
A 58-yr-old Bangladeshi man was admitted with a 4-week history of bilateral buttock pain. There was no history of preceding trauma. Six months previously, he was noted to have thrombocytopenia and following bone marrow aspiration and biopsy, immune thrombocytopenia was diagnosed. His antiplatelet antibodies, however, were negative. Physical examination showed a temperature of 36.8°C. There were no palpable lymph glands, clear chest, normal heart sounds and no organomegaly. There was tenderness in the left femur and pelvis with weakness and wasting of the left quadriceps muscle. His urine was clear.
Investigations showed his haemoglobin was 12.5 g/dl, white cell count 6.9 x 109/l, platelet count 54 x 109/l, serum alkaline phosphatase 249 IU/l (normal <118), C-reactive protein 152 mg/l and erythrocyte sedimentation rate 119 mm/h. His prostatic specific antigen, serum protein electrophoresis, thyroid function, clotting profile and autoantibody screen were normal.
Plain radiographs of his pelvis and femur showed pagetoid changes in the left hemipelvis and femur with a lytic lesion in the mid-shaft of the left femur. CT-guided biopsy of the lytic lesion confirmed Paget's disease with no evidence of malignancy. Radionuclide bone scan showed hot spots suggestive of Paget's disease or metastasis at the left hemipelvis, femurs, L4 and 5 and the distal right tibia.
Initially, he was started on risedronate at 30 mg/day. Two days later, he became pyrexial at 38°C. The risedronate was stopped, but the fever continued. Blood, urine and throat swab cultures failed to grow any organisms. Abdominal ultrasound, echocardiogram, hepatitis screen, malarial screen and Heaf test were normal or negative.
This patient's pain and temperature remained persistent and a CT scan of thorax, abdomen and pelvis were requested. This revealed a soft tissue mass causing destruction at the lesser trochanter of left femur and inferior portion of the sacrum, with noted Paget's disease in the left hemipelvis and scapula (Fig. 1).
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Our patient's disease was considered too advanced for surgery and instead he received palliative radiotherapy. He died 3 months later.
Paget's disease of bone is characterized by an accelerated rate of bone turnover, with excessive resorption and formation of bone resulting in a mosaic lamellar bone with increased vascularity and fibrous tissue formation in the adjacent bone marrow [1]. It affects both sexes equally, and occurs in 2% of the population over the age of 50. It is more common in European and rare in Asian and Scandinavian people. Our patient was originally from Bangladesh.
It has been observed that patients with Paget's disease produce large quantities of interleukin (IL)-6 [2]. IL-6 production is also increased in patients with bone metastasis. Bisphosphonates are also known to stimulate the secretion of IL-6 [1]. We postulate that the pyrexia in our patient must have been induced by the risedronate and was later maintained by the secretion of IL-6 by the pagetoid and tumour tissue.
Angiosarcoma is a rare anaplastic tumour. It comprises 0.11.0% of primary maligant bone tumours [3,4]. Malignant transformation occurs in 0.71% of patients with Paget's disease of bone. The commonest complicating malignant tumour is osteosarcoma [1, 2].
The 5-yr survival of patients with angiosarcoma is 08% [5] and this is a reflection of the aggressive nature of the tumour, the delay in diagnosis and the more advanced age of the patients. The last often contributes to the patient being unfit for aggressive surgery.
There have been around 300 reports of angiosarcoma alone, but only three case reports of angiosarcoma in patients with Paget's disease, two arising from pagetoid bone and one arising separately [3,4]. Patients with Paget's disease and angiosarcoma present with pain often out of proportion to the Paget's disease itself. Our patient had severe pain and pyrexia.
In summary, our patient was unusual in several aspects. He was originally from Bangladesh and Paget's disease of bone is very rare in patients from the Asian subcontinent [1]. Malignant transformation occurs in 0.71% of patients with Paget's disease, and angiosarcoma arising from pagetoid bone has only been reported in two patients. Though the fever may have been triggered by the risedronate, it is more likely to have been caused by IL-6 secretion by the angiosarcoma. The low platelet count is most likely to have been caused by physical destruction of the platelets in the vascular spaces of the angiosarcoma. His antiplatelet antibodies were negative with a normal bone marrow.
The authors have declared no conflicts of interest.
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