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Journal of Clinical Oncology, Vol 26, No 18 (June 20), 2008: pp. 3089-3091 © 2008 American Society of Clinical Oncology. DOI: 10.1200/JCO.2008.16.1208
Primary Pulmonary PlasmacytomaNassau University Medical Center, East Meadow, NY A 55-year-old black female with no past medical history came to the emergency department with right-sided chest pain and shortness of breath for the last 8 days. The patient also had anorexia, 5-lb weight loss during 1 week, and cough productive of yellow sputum for the last few days. The patient denied fever, nausea, vomiting, abdominal pain, recent travel, or sick contacts. She did not take any medications and never had a surgery. Her only hospitalization was for a vaginal delivery 30 years ago. The patient denied smoking, alcohol use, or illicit drug use. Her parents had a history of cancer but the patient did not remember the type of cancer or age at the time of their deaths. Vital signs on admission were blood pressure, 110/80 mmHg; regular heart rate, 100 beats/min; respiratory rate, 20 breaths/min; temperature, 98.6°F. Respiratory system examination revealed decreased air entry on the right side of the chest. The rest of the physical examination was unremarkable. Routine laboratory work-up showed WBC count, 8,500/µL; hemoglobin, 10.2 g/dL; platelet count, 506,000/µL; sodium, 131 mmol/L; potassium, 3.8 mmol/L; chloride, 102 mmol/L; bicarbonate, 21 mmol/L; blood urea nitrogen, 12 mg/dL; creatinine, 0.9 mg/dL; glucose, 108 mg/dL; calcium 8.6 mg/dL; albumin, 3.4 gm/dL; total protein, 10.5 gm/dL; AST, 41 IU/L; and lactate dehydrogenase, 211 IU/L. Chest x-ray showed moderate to large right pleural effusion, possible underlying mass, and infiltrate or atelectasis with mild mediastinal shift to the left. The patient underwent chest tube placement on the right side with significant decrease in the pleural effusion. Computed tomography scan of the chest revealed large (approximately 10 cm in its greatest diameter) heterogeneously enhancing right lung mass (Fig 1A). The patient underwent a percutaneous computed tomography–guided core biopsy of the right lung mass, which showed plasmacytoma, well differentiated (Fig 2, hematoxylin and eosin stain). Immunostaining showed tumor cells were positive for CD138, multiple myeloma oncogene 1 lambda (Fig 3), and immunoglobulin G (IgG), but negative for cytokeratins, thyroid transcription factor-1 protein, CD3, and CD20; these results suggested that the tumor was of plasma cell origin with light chain restriction. Serum protein electrophoresis showed M spike in the gamma region. Serum immunofixation showed abnormal IgG (lambda). Bone marrow biopsy showed 1.64% CD38 bright plasma cell (Fig 4). Quantitative Ig assay showed IgG, 5,160 mg/dL (range, 613 to 1,295 mg/dL); IgA, 113 mg/dL (range, 69 to 309 mg/dL); and IgM, 28 mg/dL (range, 53 to 334 mg/dL). The very high monoclonal protein concentration in our patient was likely due to the enormous tumor mass. Light chains were absent in the urine. Skeletal survey showed no evidence of lytic or blastic lesion and no evidence of fracture. Diagnosis of primary pulmonary plasmacytoma (PPP) was made and the patient received a total of 59.4 Gy of radiation therapy with complete resolution of the tumor (Fig 1B). On the last follow-up visit 6 months after the treatment, the patient was free from signs and symptoms of the disease. Quantitative Ig assay showed IgG, 3,010 mg/dL; IgA, 122 mg/dL; and IgM, 39 mg/dL, which indicated improvement.
A plasmacytoma is a discrete, solitary mass of neoplastic monoclonal plasma cells. Soft tissue extramedullary plasmacytoma (SEP) represents approximately 3% of all plasma cell neoplasms. It can arise in any part of the body. Wiltshaw1 reported that although SEP can arise in almost any organ, by far the commonest site of origin of SEP is the subepithelial tissues of the mucous membranes of the upper air passages, including the paranasal sinuses. Approximately 80% of cases of SEP involve the paranasal sinuses, pharynx, nasal cavity, or gums and oral mucosa.1-5 PPP is a rare tumor; to our knowledge, fewer than 30 cases have been reported in the literature and verified by immunohistochemistry.6 Typically, they present as a solitary nodule or less commonly as lobar consolidation or diffuse pulmonary infiltrates. Our case is a unique presentation of PPP as it presents with IgG/lambda monoclonal gammopathy. To our knowledge, three other cases7 have been reported in the literature to date, with similar presentation. Diagnostic criteria for SEP are as follows4: tissue biopsy showing monoclonal plasma cell histology; bone marrow plasma cell infiltration not exceeding 5% of all nucleated cells; absence of osteolytic bone lesions or other tissue involvement (no evidence of myeloma); absence of hypercalcemia or renal failure; and low serum M protein concentration, if present. On the basis of the documented radiation sensitivity of plasma cell tumors, one of the accepted treatments is radiation therapy; other treatment modalities are resection alone or a combination of surgery with chemotherapy. Our patient received radiation therapy with complete resolution of the tumor. Chemotherapy is indicated for patients with refractory and/or relapsed disease. Therapy as for multiple myeloma is indicated. Adjuvant chemotherapy should be considered in patients with tumors larger than 5 cm and those with high-grade tumors.8 For our patient, we did not use adjuvant chemotherapy because the tumor was well differentiated and responded well to radiation therapy, and on follow-up, the patient had decreasing immunoglobulin levels. A review article of 19 cases of PPP9 showed that most patients with PPP are middle aged or older (range, 14 to 49 years), with mean and median age of 57 and 55 years, respectively. In the 19 patients, the male-to-female ratio was 1.4:1. The overall 2- and 5-year survival was 66% and 40%, respectively. Nine of 22 patients (40%) ultimately developed multiple myeloma. Therefore, it is essential that a thorough evaluation be undertaken to exclude systemic disease at initial presentation; these patients should be monitored by close clinical follow-up. AUTHORS’ DISCLOSURES OF POTENTIAL CONFLICTS OF INTEREST The author(s) indicated no potential conflicts of interest.
REFERENCES 1. Wiltshaw E: The natural history of extramedullary plasmacytoma and its relation to solitary myeloma of bone and myelomatosis. Medicine (Baltimore) 55:217-238, 1976[Medline] 2. Alexiou C, Kau RJ, Dietzfelbinger H, et al: Extramedullary plasmacytoma: Tumor occurrence and therapeutic concepts. Cancer 85:2305-2314, 1999[CrossRef][Medline] 3. Dimopoulos MA, Kiamouris C, Moulopoulos LA: Solitary plasmacytoma of bone and extramedullary plasmacytoma. Hematol Oncol Clin North Am 13:1249-1257, 1999[CrossRef][Medline] 4. Galieni P, Cavo M, Pulsoni A, et al: Clinical outcome of extramedullary plasmacytoma. Haematologica 85:47-51, 2000 5. Liebross RH, Ha CS, Cox JD, et al: Clinical course of solitary extramedullary plasmacytoma. Radiother Oncol 52:245-249, 1999[CrossRef][Medline] 6. Edelstein E, Gal AA, Mann KP, et al: Primary solitary endobronchial plasmacytoma. Ann Thorac Surg 78:1448-1449, 2004 7. Chang C, Chang Y, Lee L, et al: Primary pulmonary plasmacytoma with immunoglobulin G/lambda light chain monoclonal gammopathy. J Thorac Cardiovasc Surg 132:984-985, 2006 8. Soutar R, Lucraft H, Jackson G, et al: Guidelines on the diagnosis and management of solitary plasmacytoma of bone and solitary extramedullary plasmacytoma. Br J Haematol 124:717-726, 2004[CrossRef][Medline] 9. Koss MN, Hochholzer L, Moran CA, et al: Pulmonary plasmacytomas: A clinicopathologic and immunohistochemical study of five cases. Ann Diagn Pathol 2:1-11, 1998[Medline]
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Copyright © 2008 by the American Society of Clinical Oncology, Online ISSN: 1527-7755. Print ISSN: 0732-183X
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