Extramedullary plasmacytoma manifesting as a palpable mass in the nasal cavity. (Original Article).

Abstract

We report a rare case of plasmacytoma of the nasal cavity in a 60-year-old man. The patient had a history of a steadily growing and palpable mass in the opening of the left nostril. The tumor was found to be localized, and it was identified as an extramedullary plasmacytoma. The patient underwent therapy with 55 Gy of radiation. Six months following the cessation of radiotherapy, the size of the tumor had not changed. The mass was then completely excised under microscopic vision. Histopathologic examination identified the growth as a plasmacytoma with monotypic light-chain expression. To optimize the management of patients with an extramedullary plasmacytoma of the head and neck, interdisciplinary management is mandatory.

Introduction

Depending on their site of development and clinical features, malignant tumors of monoclonal antibody-producing plasma cells are classified as either multiple myelomas, solitary plasmacytomas, or extramedullary plasmacytomas. (1) Extramedullary plasmacytomas account for 4% of all nonepithelial tumors of the nasal cavity, paranasal sinuses, and nasopharynx, and they are found in 0.4% of all head and neck malignancies. (2) These tumors have been reported to occur simultaneously at different sites in the head and neck in 10 to 20% of patients. (2-4) Approximately 80% of extramedullary plasmacytomas are localized in the submucosa of the upper respiratory tract, and they usually occur in patients between the ages of 50 and 60 years. (4, 5)

The postdiagnosis clinical course is unpredictable and varies among individuals. (3) Between 10 and 32% of all patients develop multiple myeloma following diagnosis, which dramatically reduces the mean survival time from 8.3 years to 20 months. (2, 3, 6) Recurrent extramedullary plasmacytomas are often successfully treated only with surgery. (7)

In this article, we describe a case of extramedullary plasmacytoma in the nasal cavity that was diagnosed by chance and treated initially with radiation therapy and later with surgery.

Case report

A 60-year-old man was referred to our clinic after he had palpated a nasal mass at the piriform aperture on the left side (figure 1). His breathing was only slightly impaired, and he experienced no spontaneous bleeding. Endoscopic examination confirmed the presence of the dark-red submucosal mass at the piriform aperture on the left side of the floor of the nasal cavity. No cervical lymph nodes were palpable or detected by ultrasound.

Histopathologic examination of a biopsy specimen identified a malignant lymphoma. Further analysis revealed that the mass was an extramedullary plasmacytoma with expression of [lambda] light chains; no heavy-chain expression was detected (figure 2). The tumor cells were positive for CD138 and syndecan and negative for CD20 (a pan-B-cell marker). Intermingled cells were positive for CD3 (a pan-T-cell marker). A bone marrow biopsy, chest x-ray, total-body skeletal survey, and 99m technetium scintigraphy did not detect any systemic lesion. Tests for Bence Jones protein in the urine and for serum myeloma protein were negative. Computed tomography (CT) of the head and neck demonstrated that the paranasal sinuses and adjacent bones were not affected. These findings confirmed the diagnosis of a stage IA extramedullary plasmacytoma (Dune and Salmon classification).

The tumor was irradiated with 55 Gy (usually at 2 Gy/day), but it did not shrink (figure 3). Six months following the cessation of radiotherapy, the mass was completely excised under microscopic vision and examined histologically. Vital tumor residues were analyzed immunohistochemically because of their altered, partly pleomorphic appearance (figure 4). Monotypic [lambda] light chains could be detected. Because of the anaplastic character of the tumor, remaining tumor cells could be detected using polyclonal antibodies to [lambda] and [kappa] light chains. At the 1-year follow-up, there was no sign of recurrence.

Discussion

Plasma cells are mature immunocompetent cells that are derived from B lymphocytes, and they provide specific noncellular immunity within the immune system. They evade blood vessels after a short time of circulation and produce specific antibodies against antigens in different tissues. Larger populations are seen in the mucosa of the nasal and paranasal sinuses. Plasmacytomas arise from a neoplastic proliferation of these antibody-producing plasma cells. The three types of plasmacytomas--multiple myelomas, solitary plasmacytomas, and extramedullary plasmacytomas--are further classified as either localized (stage I), localized including local lymph nodes (stage II), or generalized (stage III), according to their clinical manifestation. (4)

The first case of extramedullary plasmacytoma was reported in 1905. (8) Approximately 80% of cases occur in the upper respiratory tract, and 10 to 20% manifest as multiple lesions. (2-5) The most frequently affected areas are the nasal cavity and paranasal sinuses, followed by the nasopharynx, tonsils, and pharynx. (2) A systematic method of staging this disease--including measurements of the complete blood count, renal and liver function, serum and urinary protein electrophoresis, and serum immunoglobulin levels; a skeletal survey and bone marrow examination; and CT of the tumor region--must be performed in order to exclude systemic involvement before the diagnosis can be made.

The treatment of localized extramedullary plasmacytoma is still a matter of some debate; some clinicians favor radiotherapy, (4,7,9) others prefer surgical management, (2) and some advocate a combination of both (10) As was the case in our patient, radiotherapy does not always reduce the size of the tumor, perhaps because of an abundant deposition of amyloid within the mass. (11) Fortunately, the tumor in our patient was resectable, but this is not always the case. In any event, regular examinations by an otolaryngologist should be performed on every patient with an extramedullary plasmacytoma of the head and neck as long as the patient lives.

Chemotherapy is advisable only for patients who have a generalized extramedullary plasmacytoma. The 5-year survival rate among these patients has been reported to range between 53 and 75%. (3) Because each individual clinical course is unpredictable and because the development of multiple myelomas has been observed in 10 to 32% of patients 28 to 36 years later, lifetime follow-up is again mandatory. (2,12,13) A 10-year survival rate of about 50% can be expected. (12)

The optimal therapy for patients with an extramedullary plasmacytoma of the head and neck can be achieved only by an interdisciplinary approach.

Acknowledgment

The authors thank Prof. Stathis Philippou, chairman of the Department of Pathology at Augusta-Kranken-Anstalt in Bochum, Germany, for his excellent consultation.

From the Department of Otorhinolaryngology--Plastic Head and Neck Surgery, St. Anna Hospital, Duisburg, Germany (Dr. Windtuhr), and the Department of Pathology, University Hospital, Wuerzburg. Germany (Dr. Ott).

Reprint requests: Jochen P. Windfuhr, MD, Department of Otorhinolaryngology--Plastic Head and Neck Surgery. St. Anna Hospital, Albertus Magnus Str. 33,47259 Duisburg, Germany. Phone: [phone]; tax: [phone]; e-mail: [e-mail]

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