*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: email@example.com
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
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
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
Plasmacytomas are tumors of monoclonal plasma cells. There is a
wide variance in the incidence of primary pulmonary plasmacytomas in
the literature.2–3 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.2–3 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.2–3
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.2–3 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.2–3 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
This case is unique in the presentation of renal failure with
monoclonal gammopathy, and a negative result for the initial multiple
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