Psasmacytoma, Arelatively uncommon presentation

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BARIK B.J.,PUSPARANI DAS., SAGNIKA TRIPATHY., SATHY R.C., BHAKTA S., PADHAN P.

ABSTRACT

We report a case of plasmacytoka in a middle – aged man who subsequently developed multiple myeloma. The pathophysiology, diagnosis and treatment of this condition are briefly reviewed.

Key woreds: Psasmacytoma, Multiplemyloma.

INTRODUCTION

Plasma cell disorders are either generalized or localized. Generalized psasma cell proliferation with osteolytic bone lesion and monoclonal component of protein in serum electrophoresis is commonly known as multiple myeloma (M.M). But 10%of all plasma cell disorders can present as localized plasmacytoma¹. plasmacytoma may be two types.

1.      Solitary osseous plasmacytoma (SOP)

2.      Extramedullary plasmacytoma (EMP)

Usually they present at the age of 50 years, about 20 years before the average of presentation of multiple myeloma ² . Though majority of SOP and about 50% EMP can progress to M.M. in long course, but their early detection is very important for the treatment point of view. Local radio theraphy with or without chemotheraphy can cure the patients.

CASE REPORT

            A 55 yr male was admitted on 25.06.03 with the complaints of swelling over sternal region, noted for last 6 months. It was gradually increase in size and became painful for last 4 months. He was also complaining of left shoulder pain. For last few days low back pain also started. This pain was nagging in character and increased with movement. His urine output normal, mild wt loss was also associated with it. He was mildly anemic and his cardiovascular; CNS and respiratory system had no abnormality. Hepatosplenomegaly was absent. His BP was 120/80 and pulse 80/min.

            Sternal swelling was firm, mildly tender, nonpulsatile, measuring »5cm x9cm x1.2cm.

            On the day of admission FNAC of swelling was done and it revealed plasmacytoma. Serum and urine were sent for M band and Bence Jonesproteins respectively, but both of them were negative. But skull X- ray revealed punch out lesions and bone marrow from Rt iliac crest detectedplasma cell infiltration (>85%). Hb%was 9.6gm, Urea 18 mg %creatinine 1.2mg %, liver function test normal and sedimentation rate 60mm in 1^st hr.

            Two days after amdmission, patient fell down in bathroom and the shaftof the humerus and femur were fractured. Plaster casttwas done on next day.

            Five days after admission, chemotherapy was started with cyclophosphamide 200 mg /m² and predinisolone 80 mg /m² for 5 days along with supportive therapy.

            Expect mild pain abdomen and chest pain no other complication developed. On follow up after 4 weeks, swelling over sternum regressed and sternal depression developed at that place due to destruction of mass along with ribs instability. No radiotherapy was given.

Discussion

            Solitary plasmacytoma is comparativelyan uncommon entity. When above- mentioned patient presented with sternal swelling it gives the suspicion of mediastinal pathology. But firm nature and ultimate FNAC report disclosed it as a plasmacytoma. Early age of presentation (55yrs) and few days H/O of low back pain also point out towards plasm,acytoma. But as every patient with plasmacytoma should be a suspected of multiple myeloma, B.M. examination and X- rayskull were done. This led to the diagnosis of the patient as a case of MM. M band negativity in this case tells about non – secretary nature of the tumor, which  is more aggressive. This can explain why a patient develops pathological fracture so early. b₂ Micro globulin can indicate about the prognosis of the patients, but not in this case due to lack of facility. As this case converted to M.M⁶, systemic chemotherapy was given instead of local irradiation.

            This is a case of plasmacytoma, which on long course, converted to multipla myeloma.

Conclusion:

             Although solitary plasmacytoma is rare, it should be considered for any abnormal bony shelling and they should be further followed up know their progression to  multiple myeloma.

Reference

1.                  Meis J.M. et al – Solitary plasmacytoma of bone and extramedullary plasma – cytoma : A clinicopathological and immunological study-Cancer 1987; 5: 1475-1785.

2.                  Alexamin R. L:ocalized and indolent myeloma 1980; 56-521-526.

3.                  Dimoponios MA et al – Chrability of silitary bone plasmacytome J. Clinical Oncology, 1992, 10:587-590.

4.                  Mouloponios LA et al – MRI instaging of solitary plasmasytoma of bones. J. of cli. Onco. 1993:11:1311-1315.

5.                  Aviles A et al – Serum b₃ microglobnulin in solitary plasmacytoma – Blood 1990-76:1663.

6.                  Wiltshend E, The natural history of EMP and its relation to solitary myeloma of bone and myelomatosis, medicine 1976--;55;217-2383.