Primary B cell lymphoma of the external auditory canal.

Abstract

Temporal bone lymphomas are rare and typically metastatic neoplasms. We describe a case of primary B cell lymphoma that originated in the external auditory canal of an elderly woman. The diagnosis was based on histopathologic examination supplemented by immunophenotypic analysis. The patient was treated with external-beam radiation and remained disease-free throughout 9 years of follow-up. We also point out that the presence of non-Hodgkin's lymphoma in an unusual site may be an indication that the patient has an acquired immunodeficiency syndrome.

Introduction

Although lymphomas account for the largest number of nonepithelial malignancies of the head and neck, these lymphoproliferative neoplasms seldom present in the temporal bone area. During our review of the literature, it became incontrovertibly clear that isolated case reports of temporal bone lymphomas generally represented either metastatic foci from distant primaries or encroachment from contiguous locations. (1,2) Sporadic cases of primary extranodal lymphoproliferative malignancies originating in the temporal bone area have been reported; potential sites of origin include the middle ear, (3-5) mastoid, (5,6) internal auditory canal, (7) and external auditory canal. (8,9) In this article, we report a case of primary B cell lymphoma that originated in the external auditory canal, a site reported only rarely in the literature.

Case report

An 83-year-old woman presented with a 6-week history of fullness and diminished hearing in the left ear. She denied any ear pain or discharge, and she could not recall any antecedent upper respiratory infection or recent ear trauma. Her medical history was limited to hypertension and Paget's disease.

Routine and microscopic examinations of the left ear canal revealed the presence of a blue-hued, circumferential swelling that prevented clear visualization of the tympanic membrane. Multiple needle aspirations of the lesion yielded no aspirate and caused no notable decompression of the lesion. Audiometric testing revealed a severe to profound, high-frequency sensorineural bearing loss in the right ear and a mixed loss in the left ear. Prior to a planned biopsy, computed tomography (CT) of the temporal bone clearly delineated the outline of a soft-tissue lesion confined entirely to the left ear canal (figure 1).

[FIGURE 1 OMITTED]

Histologic examination of the biopsy specimen revealed that it was a diffuse large-cell lymphoma (figure 2). Partial infiltration of the fibroconnective tissue by atypical mononuclear cells was seen. The cells had large nuclei, frequent multiple nucleoli, and a moderate amount of cytoplasm--histologic features that are consistent with diffuse large-cell lymphoma. The surrounding tissue exhibited evidence of inflammation. Immunophenotypic analysis of the tumor was performed via a three-step immunoperoxidase method. The paraffin tissue sections were stained with antibodies to CD20 (L26), CD3, CD68 (PGM-1), lysozyme, CD43 (Leu 22), CAM 5.2, and AE1/AE3.

[FIGURE 2 OMITTED]

The tumor cells were negative for CD3 (T cells), CD68, lysozyme (myeloid cells), CAM 5.2 (epithelial cells), and AEI/AE3, which effectively ruled out carcinoma, granulocytic sarcoma, and T cell lymphoma. However, the tumor cells were positive for CD20 (L26), indicating that they were of B cell origin. In addition, the tumor cells coexpressed CD43. Coexpression of CD43 and a B cell marker is compatible with a B cell lymphoma. A staging workup was negative for any other site of involvement.

The patient was treated with external-beam radiation to 5,040 cGy. She remained disease-free throughout 9 years of follow-up.

Discussion

Lymphomas typically present as solid, malignant neoplasms that arise from lymphocytes and their respective precursor cells. These malignancies are generally grouped into one of two major categories: Hodgkin's disease and non-Hodgkin's lymphoma. These two entities have distinctive clinical and histologic features. Irrespective of cell type or classification, the single most striking clinical feature of these lymphoproliferative neoplasms is clearly the presence of lymphadenopathy. Extranodal involvement, although rare in Hodgkin's disease, may be present in as many as 30% of patients with non-Hodgkin's lymphoma; the head and neck region is second only to the gastrointestinal tract as the most common site of involvement. (10) Lymphadenopathy is generally associated with extranodal involvement at some point during the course of the disease. Should an extranodal site prove to be the sole locus of pathology, the lesion would likely be identified as a primary extranodal lymphoma. (5)

Although uncommon, a number of cases of temporal bone lymphomas have been reported. (1,2) In most of them, the lymphoma was metastatic. As mentioned, sites of primary lymphomas originating in the temporal bone area have been reported to include the middle ear, (3-5) mastoid, (5,6) internal auditory canal, (7) and external auditory canal. (8,9) Of these sites, the middle ear and mastoid appear to be the most common.

Symptoms commonly associated with these temporal bone neoplasms tend to be site-specific. Although patients may present with any combination of typical ear complaints, certain circumstances--such as facial nerve paralysis or an infection that fails to respond to appropriate medical intervention--should raise the specter of a possible underlying malignancy. (5)

A rise in the incidence of non-Hodgkin's lymphoma is well recognized in patients whose immune status is altered. Non-Hodgkin's lymphoma has taken on added relevance because of its prominence as a malignancy related to acquired immunodeficiency syndrome (AIDS). With an estimated 200-fold increase beyond expected rates in patients infected with human immunodeficiency virus (HIV), non-Hodgkin's lymphoma has emerged as the second most common malignancy among this group. Virtually all cases of HIV-associated non-Hodgkin's lymphoma are of B cell origin; approximately half of these cases are extranodal in presentation. (11) These neoplasms typically progress rapidly and generally display high-grade histopathology. A wide spectrum of uncommon, unexpected extranodal sites has been described in cases of AIDS-related non-Hodgkin's lymphoma. (11,12) It would clearly serve us well not to overlook the possibility of AIDS in a patient with non-Hodgkin's lymphoma in an unusual extranodal site.

References

(1.) Paparella MM, el-Fiky FM. Ear involvement in malignant lymphoma. Ann Otol Rhinol Laryngol 1972;81:352-63.

(2.) Kobayashi K, Igarashi M, McBride RA, et al. Temporal bone pathology of metastatic T-cell lymphoma. Acta Otolaryngol Suppl 1988;447:113-19.

(3.) Gapany-Gapanavicius B, Chisin R, Weshler Z. Primary presentation of malignant lymphoma in middle ear cleft. Ann Otol Rhinol Laryngol 1980;89:180-3.

(4.) Lang EE, Walsh RM, Leader M. Primary middle-ear lymphoma in a child. J Laryngol Otol 2003;117:205-7.

(5.) Tucci DL, Lambert PR, Innes DJ Jr. Primary lymphoma of the temporal bone. Arch Otolaryngol Head Neck Surg 1992;118: 83-5.

(6.) Lewis WB, Patel U, Roberts JK, et al. Angiocentric T-celllymphoma of the temporal bone. Otolaryngol Head Neck Surg 2002;126: 85-6.

(7.) Angeli SI, Brackmann DE, Xenellis JE, et al. Primary lymphoma of the internal auditory canal. Case report and review of the literature. Ann Otol Rhinol Laryngol 1998;107:17-21.

(8.) Fish BM, Huda R, Dundas SA, Lesser TH. B-cell lymphoma of the external auditory meatus. J Laryngol Otol 2002;116:39-41.

(9.) Shuto J, Ueyama T, Suzuki M, Mogi G. Primary lymphoma of bilateral external auditory canals. Am J Otolaryngol 2002;23: 49-52.

(10.) Hanna E, Wanamaker J, Adelstein D, et al. Extranodal lymphomas of the head and neck. A 20-year experience. Arch Otolaryngol Head Neck Surg 1997;123:1318-23.

(11.) Biggar RJ, Rabkin CS. The epidemiology of AIDS-related neoplasms. In: Krown SE, von Roenn JH, eds. Hematologic and Oncologic Aspects of HIV Infection. Philadelphia: W.B. Saunders; 2996:1002.

(12).Knowles DM. Etiology and pathogenesis of AIDS-related nonHodgkin's lymphoma. In: Krown SE, von Roenn JH, eds. Oncologic Complications of HIV Infection. Philadelphia: W.B. Saunders; 2003:787.

Sheldon P. Hersh, MD; Winston G. Harrison, MD; David J. Hersh, MD

From the Department of Surgery/Otolaryngology (Dr. S.P. Hersh) and the Department of Pathology (Dr. Harrison), New York Hospital Medical Center of Queens, Flushing, N.Y., and the Department of Medicine, Lenox Hill Hospital, New York City (Dr. D.J. Hersh).

Reprint requests: Sheldon P. Hersh, MD, 110-11 72nd Ave., Suite 1B, Forest Hills, NY 11375. Phone: [phone]; fax: [phone]; e-mail: [e-mail]