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CASE REPORT
Year : 2009  |  Volume : 5  |  Issue : 4  |  Page : 297-299

Pulmonary and nodal multiple myeloma with a pleural effusion mimicking bronchogenic carcinoma


Department of Pulmonary Medicine, C.S.M. Medical University (Erstwhile King George,s Medical University), UP, Lucknow (INDIA)-226003 & G.S.V.M. Medical College, Kanpur, India

Date of Web Publication11-Feb-2010

Correspondence Address:
R.A.S Kushwaha
Department of Pulmonary Medicine, C.S.M. Medical University (Erstwhile King George,s Medical University), UP, Lucknow (INDIA)-226003 & G.S.V.M. Medical College, Kanpur
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0973-1482.59915

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 > Abstract 

Multiple myeloma (MM) is a hematological malignancy characterized by the occurrence of plasma cell tumor within the bone marrow. Extramedullary plasmacytomas form a small percentage of plasma cell tumors, and although 80-90% of extramedullary lesions occur in the head and neck, pulmonary plasmacytomas are found to be a very uncommon clinical entity. Hereby, we describe a case of a patient with pulmonary plasmacytoma, who developed nodal and pulmonary MM with a pleural effusion, the radiological appearance of which mimicked bronchogenic carcinoma.

Keywords: Bronchogenic carcinoma, extramedullary, multiple myeloma


How to cite this article:
Kushwaha R, Verma SK, Mehra S, Prasad R. Pulmonary and nodal multiple myeloma with a pleural effusion mimicking bronchogenic carcinoma. J Can Res Ther 2009;5:297-9

How to cite this URL:
Kushwaha R, Verma SK, Mehra S, Prasad R. Pulmonary and nodal multiple myeloma with a pleural effusion mimicking bronchogenic carcinoma. J Can Res Ther [serial online] 2009 [cited 2019 Nov 21];5:297-9. Available from: http://www.cancerjournal.net/text.asp?2009/5/4/297/59915


 > Introduction Top


Multiple myeloma (MM) represents a malignant disorder of plasma cells and accounts for 1% of all malignancies and 10% of hematologic malignancies. There are two important variants of MM: solitary bone plasmacytoma and less common extramedullary plasmacytoma. Despite a widespread distribution of plasma cells in the body, the tumor extension is primarily within the bone and bone marrow. [1] While the dissemination of extramedullary plasmacytoma in lungs found to be exceptionally rare.


 > Case Report Top


A 60-year-old male, bidi smoker (pack year 18) was admitted to our department with complaints of left-sided chest pain for 2-month duration; dry cough and breathlessness for 1-month duration; and about 6-kg weight loss over 4 weeks. The pain was moderate in intensity, constant, and localized primarily to the upper part of the chest wall both anteriorly and posteriorly. The pain increased to some extent on movement.

Since the last 2 years, he was diabetic and was on oral hypoglycemic drugs. Physical examination revealed mild pallor.

His chest radiograph revealed opacity in the left lung with a widened mediastinum and obliteration of the left costophrenic angle and erosion of the left second and third rib [Figure 1]. A computed tomographic scan of the thorax (CT thorax) revealed mass in the anterior segment of the left upper lobe with irregular margins measuring 4 × 5 × 3 cm in size and a pleural effusion on the left side. The lesion was infiltrating the pleura. There was also present a large mass with irregular margins in the prevascular region of the anterior mediastinum infiltrating the mediastinal pleura. Multiple mediastinal lymph nodes were present and fewer of them conglomerated suggestive of extracapsular spread of the disease. There was an osteolytic lesion in the body of D10 vertebra [Figure 2].

Biopsy of the lung mass revealed atypical plasma cells arranged in sheets along with pulmonary parenchymal cells suggestive of malignant myeloma. Fine-needle aspiration cytology (FNAC) of the osteolytic lesion of the body of D10 vertebra revealed plasma cell infiltration. The patient had also complained of backache and hence we carried out an MRI of the spine which revealed a diffuse and patchy altered signal intensity with the collapse of sixth cervical and first lumbar vertebra, with the picture suggestive of diffuse marrow infiltration indicative of MM [Figure 3]a and b.

His skull X-ray revealed multiple lytic lesions. Serum protein electrophoresis was done which revealed raised total proteins (11 gm/dl) (normal 6.5-8.5 gm/dl) with normal albumin, alfa-1, alfa-2, and beta globulin but markedly raised gamma globulin (5 gm/dl) (normal 0.75-1.8 gm/dl), and the electrophoresis showed an M-spike. Serum immunoglobulin determination revealed markedly raised IgG immunoglobulin (3000 gm/dl) (normal 1700 gm/dl). Beta-2 microglobulin was 3815 ng/ml (normal 1010-1730 ng/ml). Urinary examination for Bence Jones' proteins was negative. Bone marrow biopsy revealed a hypocellular marrow with more than 25% plasma cells.

Thus, diagnosis of MM with extramedullary dissemination into the lung was made on the basis of plasma cell infiltration of the bone marrow, the lytic bone lesions, the presence of monoclonal immunoglobulins in the serum, and the myeloma plasma cells in the lung mass.

The patient was planned to be referred to the oncology department for chemotherapy but was not willing to undergo any form of definitive treatment; he ultimately expired.


 > Discussion Top


MM may manifest as a diffuse bony disease (myelomatosis), as a solitary plasmacytoma of bone, or as extramedullary (extraosseous) plasmacytoma. Myeloma cells found at the extramedullary site may thus either be due to extramedullary plasmacytoma or due to extramedullary dissemination of MM. [1] Extramedullary plasmacytoma is a variant of MM. It usually involves submucosal lymphoid tissues of nasopharynx or paranasal sinuses without bone-marrow involvement. It is highly responsive to local irradiation and has excellent prognosis. [2]

Extramedullary dissemination of MM occurs in advanced disease but is a rare phenomenon. The sites of extramedullary dissemination reported in the literature are spleen, liver, lymph nodes, kidneys, thyroid gland, adrenal gland, ovary, testes, lung, pleura, pericardium, intestinal tract, and skin. [2] Lung involvement is extremely rare. In fact, it is so rare that in one large case series of 869 cases, there was no mention of lung involvement. [3] Shin et al. had described two cases of extramedullary plasmacytoma involving the lung parenchyma. [4] The first case had a mass lesion in the right infrahilar region, while the second case had reticulonodular opacities in the lower zones of both lungs. The first case did not have generalized myelomatosis, but he developed it 5 years after treatment, namely, local radiotherapy, while the second case had generalized myelomatosis,

Because it is a disorder of the aged population, with its severe clinical course and heterogeneous symptoms, the diagnosis is difficult. This disorder presents primarily in the elderly, with organ dysfunctions and symptoms such as bone pains, renal dysfunction, anemia, susceptibility to infections, hypercalcemia, hyperviscosity, and neurological manifestations. The most frequent thoracic involvement by MM is pulmonary infiltrate secondary to an infectious process.

In our patient, after encountering atypical plasma cells in lung parenchyma, we first investigated in order to find out whether he was having extramedullary plasmacytoma or MM with extra-medullary dissemination, since the treatment and prognosis of these two conditions are vastly different. For the diagnosis of MM, at least two of the following three criteria should be present: marrow plasmacytosis, lytic bone lesions, and M component, i.e., monoclonal immunoglobulin in blood and/or urine. [5] In our patient, all the three were present.

In a recent study to investigate the causes, and the frequency and the effects of prognosis of the pulmonary involvement, it was found that the cases with pulmonary involvement were associated with progressive diseases, which included mainly renal failure and pathological bone fractures.

Despite advances in the management of MM in the past few decades, it remains an incurable disease. The disease follows a relapsing course in majority of patients, regardless of the treatment regimen or initial response to treatment. Traditionally, newly diagnosed myeloma patients have been classified as either transplant or nontransplant candidates. This classification is based on a number of factors including age, performance status, comorbid medical conditions, and patient performance. Transplant candidates were typically treated with nonalkylating agents to prevent marrow damage. Nontransplant candidates often received alkylating agent-based therapy (most oncologists prefer cyclophosphamide or melphalan as an alkylating agent with prednisolone). However, cyclophosphamide is preferred more because it is less toxic to the bone marrow. These agents are administered over 4 days every 4 weeks till the disease reaches a plateau phase which is defined as the phase when patient's hemoglobin, paraprotein, and beta-2 microglobulin levels have become stable, and the patient is asymptomatic or minimally symptomatic over a period of at least 3 months. [5] Lately, a high-dose alkylating agent with hematopoietic support has been used. It has improved the remission rate from less than 10% to 50%. However, such therapy is suitable for younger patients only. [2] Recently, the management of patients with MM has been transformed by introduction of three novel agents: thalidomide, lenalidomide, and bortezomib.

We have reported the above case in order to demonstrate variability in the presentation of MM.



 
 > References Top

1.Pinto RG, Mandreker S, Vernekar JA. Multiple myeloma presenting as a subcutaneous nodule on the chest wall: Diagnosis by fine needle aspiration. Acta Cytol 1997;41:1233-4.   Back to cited text no. 1
[PUBMED]  [FULLTEXT]  
2.Longo DL. Plasma cell disorders. In: Eugene Braunwald et al. Ed, Harrison's Principles of Internal Medicine, 14 th ed, New York: McGraw Hill; 1998. p. 713-7.  Back to cited text no. 2
    
3.Kyle RA. Multiple myeloma: review of 869 cases. Mayo Clin Proc 1975;50:29-40.   Back to cited text no. 3
[PUBMED]  [FULLTEXT]  
4.Shin MS, Carcelen MF, Ho KJ. Diverse roentgenographic manifestations of the rare pulmonary involvement in myeloma. Chest 1992;102:946-8.  Back to cited text no. 4
[PUBMED]  [FULLTEXT]  
5.Mackie MJ, Ludlam CA. Diseases of blood. In: Davidson's Principles and Practice of Medicine, 17 th ed. Edinburgh;Churchill Liringstone:1995. p.821-3.  Back to cited text no. 5
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]


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