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LETTER TO THE EDITOR
Year : 2015  |  Volume : 52  |  Issue : 3  |  Page : 428-429
 

A rare case of extramedullary plasmacytoma arising from posterior mediastinum


1 Department of Medical Oncology, Bhagwan Mahaveer Cancer Hospital and Research Center, Jaipur, Rajasthan, India
2 Department of Radiation Oncology, Bhagwan Mahaveer Cancer Hospital and Research Center, Jaipur, Rajasthan, India

Date of Web Publication18-Feb-2016

Correspondence Address:
D Gupta
Department of Medical Oncology, Bhagwan Mahaveer Cancer Hospital and Research Center, Jaipur, Rajasthan
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-509X.176717

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How to cite this article:
Gupta D, Goyal J. A rare case of extramedullary plasmacytoma arising from posterior mediastinum. Indian J Cancer 2015;52:428-9

How to cite this URL:
Gupta D, Goyal J. A rare case of extramedullary plasmacytoma arising from posterior mediastinum. Indian J Cancer [serial online] 2015 [cited 2019 Jun 26];52:428-9. Available from: http://www.indianjcancer.com/text.asp?2015/52/3/428/176717


Sir,

Extra medullary plasmacytoma (EMP) is a plasma cell tumor, which involves soft-tissues, without any signs of systemic involvement. It may originate in any site although most frequently it occurs in the upper respiratory tract and oral cavity.[1],[2] Rarely may it present with mediastinal mass lesion without any other organ involvement.

A 43-year-old male presented to us with a history of mediastinal mass for 2 years although patient was asymptomatic. Before presenting to us he had a history of cough and breathlessness 2 years back, for which chest X-ray was carried out elsewhere, which showed mediastinal widening with small heterogeneous patch in the left lower lung. He was advised contrast enhanced computed tomography (CECT) of chest for that which showed mediastinal mass. Subsequently, he underwent computed tomography guided fine needle aspiration cytology, which was inconclusive so repeat biopsy was performed, which also was inconclusive. His bronchoscopy also showed no mass lesion and bronchoalveolar lavage showed only chronic bronchitis. He received conservative treatment and was kept on follow-up. One year later repeat CECT scan was carried out, which showed mediastinal mass of the same size and location. His serum alpha fetoprotein and beta-human chorionic gonadotropin levels were normal. At current presentation, his latest CECT of the chest showed infiltrating mass in the middle and posterior mediastinum encasing trachea, carina and both main stem bronchi and esophagus without intraluminal extension. Bilateral lung fields were normal and there was no pleural effusion [Figure 1]a. This time, repeat biopsy was attempted in view of persistent mass lesion although patient had no symptoms. Repeat biopsy showed plasma cell tumor with CD 38/138 positivity and cytokeratin/CD20 negative. His beta2-microblobulin was 1.91 mg/L and serum protein electrophoresis showed an M band of 4.94 g/dL. His bone marrow aspiration and biopsy did not show any infiltration by plasma cells and he had no organ dysfunction required for multiple myeloma work-up. He was treated with external beam radiation therapy, 50 Gy in 25 fractions over 5 weeks. After 2 months of radiation, his CECT chest showed a significant reduction in mass lesion [Figure 1]b and his serum protein electrophoresis showed M spike of 1.12 g/dL. He has been kept on follow-up at a regular interval.
Figure 1: (a) Computed tomography scan of thorax in sagittal section and coronal section; (b) showing mass lesion in posterior mediastinum predominantly encasing trachea and esophagus

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EMP accounts for approximately 3% of plasma cell neoplasms and it occurs in the upper aero-digestive tract in 80% of patients, mostly in the nasal cavity, paranasal sinuses, larynx, tonsils. Additional characteristics are male predominance and advanced age, usually above 40 years, at presentation.[1],[2] Diagnostic criteria of EMP are tissue biopsy indicating monoclonal plasma cell infiltration, bone marrow plasma cells <5% of nucleated cells and absence of increased calcium, renal failure, anemia and multiple bone lesions features. Some patients may have serum M band, but usually it is of low quantity.[3],[4] Rarely EMP may present with primary mediastinal mass lesions with or without lymph nodes or lung lesions.[5],[6],[7],[8],[9] Usually, these patients with mediastinal mass are having minimal symptoms of chest discomfort, cough or mild breathlessness as was in our case, but they may have compressing symptoms of superior vena cava syndrome,[10] myasthenia gravis due to thymus involvement,[11] pleural effusion [12] or may be diagnosed coexistent with multiple myeloma.[13] EMP are highly radiosensitive with 80-20% local control rates and approximately 50-65% patients remain free of disease at 10 years.[2],[14] Surgery is usually not feasible in the head and neck area, but surgery may be considering for other sites like gastrointestinal sites. Considering the high cure rates of EMP with radiotherapy and lack of adequate data on chemotherapy, radiotherapy remains the standard of care and adjuvant chemotherapy should be reserved for non-responders or relapses to myeloma.

To conclude, EMP arising in mediastinum is a rare entity and radiotherapy remains the mainstay of therapy.

 
  References Top

1.
Dores GM, Landgren O, McGlynn KA, Curtis RE, Linet MS, Devesa SS. Plasmacytoma of bone, extramedullary plasmacytoma, and multiple myeloma: Incidence and survival in the United States, 1992-2004. Br J Haematol 2009;144:86-94.  Back to cited text no. 1
    
2.
Weber DM. Solitary bone and extramedullary plasmacytoma. Hematology Am Soc Hematol Educ Program 2005;1:373-6.  Back to cited text no. 2
    
3.
Baghmar S, Mohanti BK, Sharma A, Kumar L, Prakash G, Kumar S, et al. Solitary plasmacytoma: 10 years's experience at all India institute of medical sciences, New Delhi. Leuk Lymphoma 2013;54:1665-70.  Back to cited text no. 3
    
4.
Kilciksiz S, Karakoyun-Celik O, Agaoglu FY, Haydaroglu A. A review for solitary plasmacytoma of bone and extramedullary plasmacytoma. Scientific World Journal 2012;2012:895765.  Back to cited text no. 4
    
5.
Miyazaki T, Kohno S, Sakamoto A, Komori K, Sasagawa I, Futagawa S, et al. A rare case of extramedullary plasmacytoma in the mediastinum. Intern Med 1992;31:1363-5.  Back to cited text no. 5
    
6.
Luh SP, Lai YS, Tsai CH, Tsao TC. Extramedullary plasmacytoma (EMP): Report of a case manifested as a mediastinal mass and multiple pulmonary nodules and review of literature. World J Surg Oncol 2007;5:123.  Back to cited text no. 6
    
7.
Lee SY, Kim JH, Shin JS, Shin C, In KH, Kang KH, et al. A case of extramedullary plasmacytoma arising from the posterior mediastinum. Korean J Intern Med 2005;20:173-6.  Back to cited text no. 7
    
8.
Muller C, Chantelot JM, Kemeny JL, Terrioux P, de Saint-Florent G, Blanchon F. Plasmacytoma of the mediastinum. A new case. Rev Pneumol Clin 1988;44:39-42.  Back to cited text no. 8
    
9.
Yamaguchi H, Yamaga S, Nisikawa K, Kugiyama K. A case of extramedullary plasmacytoma in the mediastinum. Nihon Kokyuki Gakkai Zasshi 2005;43:536-40.  Back to cited text no. 9
    
10.
Vanquaethem H, Salles Y, Quinquenel ML, Cuguillière A. A rare cause of superior vena cava syndrome: Extramedullary plasmocytoma. Rev Pneumol Clin 2011;67:325-8.  Back to cited text no. 10
    
11.
Ahmed AR, Marchbank AJ, Nicholson AG, Wotherspoon AC, Ladas GP. Extramedullary plasmacytoma presenting with myasthenia gravis and mediastinal mass. Ann Thorac Surg 2000;70:1390-2.  Back to cited text no. 11
    
12.
Raci-Wetherbee E, Dincer HE. IgG myeloma presenting as a large mediastinal mass and pleural effusion. J Bronchology Interv Pulmonol 2012;19:65-7.  Back to cited text no. 12
    
13.
Masood A, Hudhud KH, Hegazi A, Syed G. Mediastinal plasmacytoma with multiple myeloma presenting as a diagnostic dilemma. Cases J 2008;1:116.  Back to cited text no. 13
    
14.
Galieni P, Cavo M, Pulsoni A, Avvisati G, Bigazzi C, Neri S, et al. Clinical outcome of extramedullary plasmacytoma. Haematologica 2000;85:47-51.  Back to cited text no. 14
    


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