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 external auditory canal
See ear 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 acquired immunodeficiency syndrome, see AIDS. .
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 mastoid /mas·toid/ (mas´toid)
2. mastoid process.
3. pertaining to the mastoid process.
The mastoid process. , (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.
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 Noun 1. upper respiratory infection - infection of the upper respiratory tract
respiratory infection, respiratory tract infection - any infection of the respiratory tract 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 as·pi·rate
To take in or remove by aspiration.
A substance removed by aspiration.
The removal by suction of a fluid from a body cavity using a needle. and caused no notable decompression of the lesion. Audiometric au·di·om·e·ter
An instrument for measuring hearing activity for pure tones of normally audible frequencies. Also called sonometer.
au testing revealed a severe to profound, high-frequency sensorineural sensorineural /sen·so·ri·neu·ral/ (-noor´al) of or pertaining to a sensory nerve or mechanism; see also under deafness.
adj. 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 mononuclear /mono·nu·cle·ar/ (-noo´kle-er)
1. having but one nucleus.
2. a cell having a single nucleus, especially a monocyte of the blood or tissues.
adj. cells was seen. The cells had large nuclei, frequent multiple nucleoli nucleoli
plural form of nucleolus. , 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 lysozyme: see immunity.
An enyme that was first identified and named by Alexander Fleming, who recognized its bacteriolytic properties. , CD43 (Leu Leu leucine.
leucine. 22), CAM 5.2, and AE1/AE3.
[FIGURE 2 OMITTED]
The tumor cells were negative for CD3 (T cells), CD68, lysozyme (myeloid myeloid /my·eloid/ (mi´e-loid)
1. medullary; pertaining to, derived from, or resembling bone marrow or the spinal cord.
2. having the appearance of myelocytes, but not derived from bone marrow. 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 work·up
n. Abbr. w/u
A thorough medical examination for diagnostic purposes. 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.
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 lymphadenopathy /lym·phad·e·nop·a·thy/ (-op´ah-the) disease of the lymph nodes.
angioimmunoblastic lymphadenopathy , angioimmunoblastic lymphadenopathy with dysproteinemia . 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 Facial nerve paralysis is a common problem that involves the paralysis of any structures innervated by the facial nerve. The pathway of the facial nerve is long and relatively convoluted, and so there are a number of causes that may result in 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 human immunodeficiency virus
Human immunodeficiency virus (HIV)
A transmissible retrovirus that causes AIDS in humans. (HIV HIV (Human Immunodeficiency Virus), either of two closely related retroviruses that invade T-helper lymphocytes and are responsible for AIDS. There are two types of HIV: HIV-1 and HIV-2. HIV-1 is responsible for the vast majority of AIDS in the United States. ), 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 his·to·pa·thol·o·gy
The science concerned with the cytologic and histologic structure of abnormal or diseased tissue.
The study of diseased tissues at a minute (microscopic) level. . 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.
(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 hematological, hematologic
pertaining to or emanating from blood cells.
total and differential white cell counts, hematocrit estimation, erythrocyte count. 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 Lenox Hill Hospital, on Manhattan's Upper East Side, is a 652-bed, acute care hospital and a major teaching affiliate of NYU Medical Center. Founded in 1857 as the German Dispensary, today's 10-building Lenox Hill Hospital complex has occupied its present site since 1868 when it , New York City New York City: see New York, city.
New York City
City (pop., 2000: 8,008,278), southeastern New York, at the mouth of the Hudson River. The largest city in the U.S. (Dr. D.J. Hersh).
Reprint requests: Sheldon P. Hersh, MD, 110-11 72nd Ave., Suite 1B, Forest Hills, NY 11375. Phone: (718) 261-9000; fax: (718) 268-0504; e-mail: email@example.com