POEMS syndrome - a unique presentation of a rare paraneoplastic syndrome

Abstract

POEMS (peripheral neuropathy, organomegaly, endocrinopathy, M protein, skin changes) syndrome is a rare multisystem paraneoplastic disorder. A 40-year-old male with a history of peripheral neuropathy and erectile dysfunction presented with a pathological fracture of the neck of the femur, found to be a solitary plasmacytoma. Additional unusual features included splenomegaly, hyperprolactinaemia and skin changes. The patient had a total hip replacement at a specialist orthopaedic hospital and is due to undergo radiotherapy to the femoral lesion and autologous stem cell transplantation.

Figure 1

Radiograph of the proximal right femur. There is an intertrochanteric pathological fracture through a lytic lesion in the femur. There is well-defined endosteal scalloping of the femur and a sclerotic margin is seen medially to the lesion.

Figure 2

Coronal T1 weighted image showing a pathological fracture through the tumour in the proximal right femur. There is an intermediate signal tumour in the bone with some expansion of femur and endosteal scalloping. There is marginal sclerosis around the margins of the tumour, most obvious medially. There is a very small extraosseous mass at the medial margin of the tumour, closely related to the lesser trochanter.

Figure 3

Fluorodeoxyglucose-positron emission tomography/CT image. There is high metabolite avidity in the tumour within the femur. No other avid lesions were seen.