Secondary Extramedullary Plasmacytoma Diagnosed by Fine Needle Aspiration Cytology

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Information about Secondary Extramedullary Plasmacytoma Diagnosed by Fine Needle...

Published on September 30, 2015

Author: Apollo_Hospitals

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1. Secondary Extramedullary Plasmacytoma Diagnosed by Fine Needle Aspiration Cytology

2. Case Report INTRODUCTION A plasmacytoma is a discrete, solitary mass of neoplastic monoclonal plasma cells found in either bone marrow or a soft tissue site. It can occur as a solitary lesion or could be an extramedullary presentation of multiple myeloma. A diagnosis of plasmacytoma should prompt further clinical, biochemical and radiologic investigations to determine whether the lesion is truly solitary or in fact a localized presentation of multiple myeloma [1]. The diagnosis of extramedullary plasmacytoma on Fine Needle Aspiration Cytology (FNAC) is difficult, but recently few cases have been reported. We would like to share a similar case report of secondary extramedullary (subcutaneous) plasmacytoma diagnosed by FNAC in our institute. On further investigations, it revealed systemic involvement by the disease (multiple myeloma). CASE REPORT A 33-year old female was referred to our hospital with a previous history of fatigue, bone pain, nausea and vomiting for which she was admitted in Patna in January’09. A Bone Marrow Aspiration carried out there was reported as? Megaloblastic Anemia/? Myelodysplasia with excess blasts. A PET scan performed in July’09 showed multiple skeletal metastases. Tumor markers (CEA, CA-125, CA-19.9) were within normal limits. In November’09, she presented to our institute with complaint of multiple subcutaneous lumps over the abdomen and right thigh. These lumps were soft, mobile, non-tender and slowly increasing in size since the past few months. Her biochemical investigations revealed normal renal and liver Function Tests. The serum Total Protein, Albumin and Globulin were 6.9 g/dL, 3.4 g/dL and 3.5 g/ dLrespectively. FNAC was performed from the subcutaneous nodules present over the anterior abdominal wall and right thigh. All the three sites showed similar cytomorphology. Smears were richly cellular, composed of sheets of dispersed pleomorphic plasmacytoid cells with occasional clustering. Cells were of medium to large size, oval in shape with dense blue cytoplasm. Nuclei were round, eccentrically placed with coarse chromatin and inconspicuous nucleoli. Binucleate and multinucleate forms were also present. Occasional mitotic figures were seen. Background was hemorrhagic. On Immunocytochemistry, the tumor cells showed focal positivity with CD138 (Fig.1). A final diagnosis of Secondary Extramedullary Plasmacytoma was made on cytology. A bone marrow aspiration (BMA), Immunoglobulin Profile and Serum Protein Electrophoresis studies were advised (Table 1, Fig.2). SPE showed the presence of a sharp ‘M spike’ in the gamma globulin region (1.89 gm/dL). Ascitic fluid examination was given as “Positive for suspicious (plasmacytoid) cells”. Bone marrow aspiration SECONDARY EXTRAMEDULLARY PLASMACYTOMA DIAGNOSED BY FINE NEEDLE ASPIRATION CYTOLOGY Neha Kapoor*, Sheefa Khan* and Mridula Guleria** Post graduate student*, Senior Consultant **, Department of Cytopathology, Indraprastha Apollo Hospitals, Sarita Vihar, New Delhi 110 076, India. Correspondence to: Dr Mridula Guleria, Senior Consultant, Department of Cytopathology, Indraprastha Apollo Hospitals, Sarita Vihar, New Delhi 110 076, India. Key words: Plasmacytoma, Multiple Myeloma, Aspiration Cytology Table 1. Immunoglobulin profile Immunoglobulin Result Reference Value IgA 13.0 85-450 mg/dL IgG 2,333.0 800-1700 mg/dL IgM 11.0 50-320 mg/dL Apollo Medicine, Vol. 7, No. 3, September 2010 220

3. Case Report 221 Apollo Medicine, Vol. 7, No. 3, September 2010 Sheets of singly dispersed plasmacytoid cells Pap Pap Smear (1) Binucleate and multinucleate forms present Pre Pap Smear (2) Mitotic figures evident Pap Pap Smear (3)

4. Apollo Medicine, Vol. 7, No. 3, September 2010 222 Case Report and biopsy along with Flow Cytometry analysis was reported to be “Consistent with Multiple Myeloma”. DISCUSSION Plasma cell neoplasms represent autonomous proliferations of plasma cells and can manifest as diffuse myeloma with systemic involvement (plasma cell myeloma or multiple myeloma), monoclonal gammopathy of undetermined significance (MGUS), or as variants of plasma cell myeloma such as indolent myeloma, smoldering myeloma, osteosclerotic myeloma, plasma cell leukemia and non-secretory myeloma [2]. Multiple myeloma comprises about 1% of malignant tumors, 10-15% of hematopoietic neoplasms and causes 20% of deaths from hematologic malignancies. It is more common in men than women (1.4:1) and 90% cases occur over the age of 50yrs [3]. A localized neoplastic proliferation of plasma cells presents as solitary plasmacytoma of bone or extramedullary soft tissue plasmacytoma. Extramedullary plasmacytomas are known to occur in many different organs, such as lymph node, skin, lung, pleura, gastrointestinal tract, spermatic cord, ovary, kidney, pancreas, thyroid and urinary bladder, but are most common in the head and neck region [4,5]. Occasional cases in the breast have also been reported [4]. These usually represent secondary involvement by a systemic process [6,7] and can at times be the sole initial manifestation of the underlying disorder. In recent years, Fine Needle Aspiration Cytology has emerged as the first line investigation for evaluation of clinically palpable masses. Cytological evaluation of such lesions offers rapid and reliable diagnosis on which prompt treatment decisions can be made [2]. When applied to plasmacytomas, FNAC offers the opportunity for a non-invasive and rapid diagnosis along with verification of widespread extramedullary involvement in a known case of multiple myeloma. The clinical course of these patients with extramedullary plasmacytomas depends on whether the lesion is solitary or part of a disseminated myeloma 4. This distinction requires clinical correlation with radiology, bone marrow biopsy and analysis of serum immunoglobulin levels [4,6]. Further investigations such as flow cytometric analysis, protein electrophoresis and immunofixation are also required to confirm a diagnosis of multiple myeloma [2]. Nuclear pleomorphism seen CD138 – Focally positive Fig.2. Serum Protein Electrophoresis (SPE) Fig.1

5. Case Report 223 Apollo Medicine, Vol. 7, No. 3, September 2010 Diagnostic criteria for Symptomatic Plasma Cell Myeloma [3]: 1. M-protein in serum or urine (>30g/L) 2. Bone marrow clonal plasma cells (usually >10%) or plasmacytoma 3. Related organ or tissue impairment (hypercalcemia, renal insufficiency, anemia, bone lesions) Plasmacytomas are often a late manifestation of the disease and their appearance in a known case of myeloma indicates failure of apparently successful therapy [4,8]. Knowledge of atypical and unusual presentations is therefore essential in arriving at a correct diagnosis of myeloma in clinically unsuspected cases. The overall prognosis is significantly worse in patients with systemic involvement as compared to those with solitary plasmacytoma, consequently early cytological diagnosis of extramedullary involvement in multiple myeloma helps in timely institution of appropriate treatment [2]. REFERENCES 1. Shariff S, Abdulla A, Huda J. Lymph node Involvement in Plasmacytoma. Bahrain Med Bull 2005; 27(4). 2. Sharma A, Kaushal M, Chaturvedi N. Cytodiagnosis of multiple myeloma presenting as orbital involvement: a case report. CytoJournal 2006, 3:19. 3. Swerdlow S, Harris N, Campo E. WHO Classification of tumors of Hematopoietic and Lymphoid Tissues. 4th edition 2008. 200-213. 4. Pai R, Kini H, Ghartimagar D. Secondary extramedullary plasmacytoma of the breast: Diagnosed by fine needle aspiration cytology. Kathmandu University Medical Journal 2007: 5(4); 538-540. 5. Enver A, Cemal G, Hasan K. Stage II multiple extramedullary plasmacytoma, synchronous the nasal polyps of the head & neck. A case report and review of the literature. Tr. J. of Medical Sciences1998; 28: 693- 695. 6. Ross JS, King TM, Spector JI, Zimbler H, Basile RM. Plasmacytoma of the breast: an unusual case of recurrent myeloma. Archives Intern Med 1987; 147: 1838-1840. 7. Bassett WB, Weiss RB. Plasmacytoma of the breast: an unusual manifestation of multiple myeloma. South Med Journal 1979; 72: 1492-1494. 8. Pai RR, Raghuveer CV. Extramedullary plasmacytoma diagnosed by fine needle aspiration cytology. A report of four cases. Acta Cytol 1996; 40(5): 963-966.

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