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Background
Extramedullary plasmacytoma (EMP) is a neoplastic proliferation of plasma cells in non-osseous sites without the involvement of bone marrow. It accounts for about 3% of all the plasma cell neoplasms. The majority of EMPs occur in the head and neck region 1 and retroperitoneal involvement is rare. 2 3 They often pose a diagnostic challenge because of their unusual location and absence of symptoms until advanced stages. A high index of suspicion is required for the diagnosis of this condition at an earlier stage. Small and confined EMPs are usually highly radiosensitive. 4 5
Case presentation
A 65-year-old Caucasian woman, a heavy smoker with a medical history of EMP of the retroperitoneum, presented with a 1-month long history of progressively worsening abdominal pain, discomfort and generalised weakness. Two years ago, she was diagnosed with retroperitoneal plasmacytoma. On diagnosis, the tumour measured 4.9 cmx3.8 cm and involved mainly the left side. She had a complete response to radiotherapy.
Currently, she denied any history of fever, night sweats, nausea or vomiting. However, she did have significant anorexia along with a 15 kg weight loss over the past 3 months. She denied any alcohol or drug use. There was no family history of malignancy in any of her first-degree relatives.
Physical examination revealed an ill-appearing woman with stable vital signs. There was no icterus, pallor or lymphadenopathy. Heart and lung examination were within normal limits. Abdominal examination revealed distension and fullness in the left flank region. The abdomen was soft, non-tender without any hepatosplenomegaly. Bowel sounds were normal. Neurological examination was grossly intact.
Investigations
Laboratory tests revealed white cell count of 4900/[micro]L, haemoglobin 10.4 g/dL and platelet count 375 000/[micro]L. Glucose, electrolytes, renal and liver function tests were within normal limits. Urinalysis revealed no Bence-Jones proteinuria. CT of the abdomen and pelvis ( figure 1 ) showed a 10 cmx16 cm left paraspinal soft tissue mass at the level of left renal hilum with significant hydronephrosis. CT of the chest showed a 6.1 cmx4.6 cm left hilar mass ( figures 2 and 3 ). Bone marrow biopsy revealed normocellular marrow with trilineage haematopoiesis with less than 10% plasma cells, non-diagnostic for multiple myeloma. Fine-needle aspiration cytology of the left retroperitoneal mass was positive...