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Primary Pulmonary Plasmacytoma*: A Case Report FREE TO VIEW

James N. Wise, MD; Robert F. Schaefer, MD; Raymond C. Read, MD, FCCP
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*From the Department of Radiology (Dr. Wise), Massachusetts General Hospital, Boston, MA; and the Departments of Anatomic Pathology (Dr. Schaefer) and General Thoracic Surgery (Dr. Read), Central Arkansas Veterans Healthcare Center, Little Rock, AK.

Correspondence to: James N. Wise, MD, Department of Radiology, Massachusetts General Hospital, 55 Fruit St, FND 216, Boston, MA 02114; e-mail: jnwise@hotmail.com



Chest. 2001;120(4):1405-1407. doi:10.1378/chest.120.4.1405
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Most extramedullary plasmacytomas are associated with the upper respiratory tract. Primary pulmonary plasmacytomas are rare, and the treatment is surgical excision. This malignancy advances to multiple myeloma in a minority of patients. The patient in our case report is the first to experience renal failure due to hematologic monoclonal gammopathy. Postoperatively, serum protein electrophoresis reverted to normal.

Figures in this Article

Multiple myeloma is a plasma cell malignancy that typically presents in the bone marrow. Extramedullary plasmacytomas are unusual tumors that are closely associated with the upper respiratory tract in 80% of cases.1 Plasmacytomas of the lower respiratory tract are very rare. Gordon and Walker reported the first in 1944. A search of Index Medicus reveals 44 cases. We report another.

A 65-year-old man with a history of end-stage renal disease, coronary artery disease, and hypertension initially presented to the hospital in renal failure. Serum protein electrophoresis demonstrated a spike of IgG κ chains. The results of multiple myeloma investigations were negative. A diagnosis of renal failure secondary to hematologic monoclonal gammopathy was made. Ten months later, the patient was being evaluated for a change of his peritoneal dialysis catheter when a right hilar mass was noted on chest radiography (Fig 1 ). It was confirmed by CT scan examination. He was then referred to the thoracic surgery department.

The patient reported a 75-pack-year history of smoking and a family history of lung cancer. A physical examination was unremarkable. An arterial blood gas examination showed the following: pH, 7.39; Pco2, 46 mm Hg; Po2, 58 mm Hg; and pulse oximetry, 91% on room air. Pulmonary function testing demonstrated an FVC of 3.65 L and an FEV1 of 2.56 L. The results of serum chemistry measurements, testing of coagulation times, and CBC were within normal limits. A bronchoscopy procedure was not diagnostic.

The patient then underwent a right thoracotomy with right upper lobectomy. Operatively, a tumor invading the right upper lobe bronchus was identified (Fig 2 ). Right lobar lymph node No. 10 and right paraesophageal node No. 8 were noted to be enlarged and were resected. By histology, these nodes were noted to have metastatic small oval cells present with hyperchromatic nuclei and occasional mitoses. Because of its bronchial location and staining properties, this mass was first identified as a carcinoid. However, initial immunohistochemical stains here suggested a plasmacytoma. Paraffin blocks and slides then were sent to the Armed Forces Institute of Pathology for consultation, and this diagnosis was confirmed.

Postoperatively, the results of a radiographic survey of the patient’s skeleton did not demonstrate any osteolytic sites. The results of testing a bone marrow biopsy specimen revealed that it was hypocellular with only 3% plasma cells. An MRI examination of the head, pelvis, and spine was negative for myeloma as well. Two months postoperatively, the patient’s M-protein spike disappeared and it has not returned. Fifteen months postoperatively, he continues to do well without evidence of recurrence.

Plasmacytomas are tumors of monoclonal plasma cells. There is a wide variance in the incidence of primary pulmonary plasmacytomas in the literature.23 This is because the verification of their monoclonal nature by serum M protein or immunohistochemical stains often is lacking. Without these two techniques, a plasmacytoma is easily confused with the much more common plasma cell granuloma.23 Even though 44 cases have been reported in the literature, only 23 cases have been verified by immunohistochemical techniques or by serologic evidence of M protein.23 Unlike other extramedullary plasmacytomas, which have a male/female ratio of 3:1 to 5:1, there is no sexual predilection for primary pulmonary plasmacytoma.23 The average age of presentation is 54 years.

Primary pulmonary plasmacytoma presents as a pulmonary nodule most commonly in the hilum (6 of 23 cases). However, presentations as lobar consolidations and bilateral diffuse infiltrates have been noted. The right and left lungs are equally affected, with the upper lobes being more commonly affected.2Primary pulmonary plasmacytoma, unlike solitary myeloma, advances to multiple myeloma only in a minority of cases.3There are six cases of primary pulmonary plasmacytoma in which the M-protein spike was present. The M-protein level is directly related to tumor burden and clinical course.4 The M-protein component usually consists of Ig Gκ chains.2This case is the third in which the patient’s M-protein level returned to normal after resection. Diagnosis is usually made after the resection of the tumor.3 After resection, immunohistochemical markers are essential to identify monoclonal plasma cells. Negative results of a postoperative myeloma survey, and negative results of testing a bone marrow biopsy specimen are essential for ruling out multiple myeloma.

The treatment for primary pulmonary plasmacytoma is usually resection. Our patient, like the majority of other patients who have undergone resections (14 of 23 cases), was considered to be treated and is not receiving adjunctive chemotherapy or radiation.23 Two patients have received surgery and radiation therapy. One patient has received surgery and chemotherapy. Three patients received radiation therapy and chemotherapy. One patient received radiation therapy only. One patient received chemotherapy only. There is no difference in survival among any of these treatment modalities.1The 2-year and 5-year survival rates are 66% and 40%, respectively.2 Two patients have been reported to survive for 9 years, and two patients have been reported to survive for > 20 years.2

This case is unique in the presentation of renal failure with monoclonal gammopathy, and a negative result for the initial multiple myeloma investigation.

Figure Jump LinkFigure 1. Preoperative plain chest radiograph showing the suprahilar mass in the right upper lobe.Grahic Jump Location
Figure Jump LinkFigure 2. Gross section of the resected right upper lobe demonstrating invasion of the right upper lobe bronchus with adjacent lymph nodes.Grahic Jump Location
Wiltshaw, E (1976) The natural history of extramedullary plasmacytoma and its relation to solitary myeloma of bone and myelomatosis.Medicine (Baltimore)55,217-238. [PubMed] [CrossRef]
 
Koss, MN, Hochholzer, L, Moran, CA Pulmonary plasmacytomas: a clinicopathologic and immunohistochemical study of five cases.Ann Diagn Pathol1998;2,1-11. [PubMed]
 
Joseph, G, Pandit, M, Korfhage, L Primary pulmonary plasmacytoma.Cancer1993;71,721-724. [PubMed]
 
Salmon, SE Immunoglobulin synthesis and tumor kinetics of multiple myeloma.Semin Hematol1973;10,135-147. [PubMed]
 

Figures

Figure Jump LinkFigure 1. Preoperative plain chest radiograph showing the suprahilar mass in the right upper lobe.Grahic Jump Location
Figure Jump LinkFigure 2. Gross section of the resected right upper lobe demonstrating invasion of the right upper lobe bronchus with adjacent lymph nodes.Grahic Jump Location

Tables

References

Wiltshaw, E (1976) The natural history of extramedullary plasmacytoma and its relation to solitary myeloma of bone and myelomatosis.Medicine (Baltimore)55,217-238. [PubMed] [CrossRef]
 
Koss, MN, Hochholzer, L, Moran, CA Pulmonary plasmacytomas: a clinicopathologic and immunohistochemical study of five cases.Ann Diagn Pathol1998;2,1-11. [PubMed]
 
Joseph, G, Pandit, M, Korfhage, L Primary pulmonary plasmacytoma.Cancer1993;71,721-724. [PubMed]
 
Salmon, SE Immunoglobulin synthesis and tumor kinetics of multiple myeloma.Semin Hematol1973;10,135-147. [PubMed]
 
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