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Malignant tumours of the ear are rare. The most common malignant tumours are squamous cell carcinomas and adenocarcinomas. Lymphoma in the ear is rare.

Malignant lymphoma of the external ear usually presents as part of a generalized process. Rarely, primary B-cell non-Hodgkin's lymphoma may occur in the external and middle ear.

Cauliflower-like tumours may be located in both the external auditory canals.

Epidermotropic CD8+ cutaneous T-cell lymphoma has been reported, presenting as an ulcerated lesion of the pinna.

A case of CD5-positive diffuse large B-cell lymphoma of the temporal bone has been reported presenting as a mass in the left external auditory canal.

In a case of therapy-resistant otitis and/or peripheral facial paralysis malignancy should be excluded by computer tomography of the mastoid.  For histopathological diagnosis unfixed specimens are preferable.      Visit: Ear Pathology Online

                  

Primary cutaneous aggressive epidermotropic CD8 positive cytotoxic T-cell lymphoma of the ear.J Laryngol Otol. 2007 May;121(5):503-5.

We report a case of an epidermotropic CD8+ cutaneous T-cell lymphoma which initially presented as an ulcerated lesion of the pinna. Although T-cell lymphomas may present as cutaneous lesions, the ear is rarely involved. This uncommon presentation and the need for multiple biopsies means that the diagnosis of these lesions may be delayed or missed. A high index of suspicion is required when evaluating cutaneous lesions in the head and neck area.

Primary B cell lymphoma of the external auditory canal.Ear Nose Throat J. 2006 Sep;85(9):597-9.

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.

De novo CD5-positive diffuse large B-cell lymphoma of the temporal bone presenting with an external auditory canal tumor. Intern Med. 2006;45(11):733-7.

We report a 74-year-old woman with primary CD5-positive diffuse large B-cell lymphoma (DLBCL) of the temporal bone. The patient was admitted because of a mass in the left external auditory canal. She was treated with eight courses of CEOP therapy (rituximab was added from the sixth course) followed by radiotherapy of 40 Gy, and complete remission was achieved. The occurrence of malignant lymphoma in the temporal bone, which is an extremely unusual site, may have depended on the peculiarity of CD5-positive DLBCL.

Primary middle-ear lymphoma in a child.J Laryngol Otol. 2003 Mar;117 (3):205-7.

The case of a five year old boy who presented with a lower motor neurone facial nerve palsy secondary to primary non-Hodgkin's lymphoma (NHL) of the middle ear is discussed. Any child who presents with a facial nerve palsy and conductive hearing loss requires thorough evaluation to exclude the possibility of temporal bone malignancy.

Systemic B-cell lymphoma presenting as an isolated lesion on the ear.Clin Exp Dermatol. 2001 Mar;26(2):166-9.

We report a case of systemic B-cell lymphoma that presented as an isolated cutaneous lesion on the ear, mimicking a primary cutaneous B-cell lymphoma. Although there was no clinical evidence of systemic disease, bone marrow involvement was found on further investigation and subsequent immunoglobulin gene analysis revealed an identical clone in the skin lesion and bone marrow aspirate. Evidence of a t(14 : 18) translocation was not identified. This case is unusual for several reasons. First, involvement of the pinna as a presenting feature of systemic lymphoma has not been reported previously. Second, the cutaneous lesion had been present for 3 years prior to diagnosis and there has been no clinical progression of systemic lymphoma during 2 years of follow-up. Third, the lymphoma does not correspond exactly to any of the entities in the REAL classification of systemic B-cell lymphoma. This case underlines the indolent nature of some systemic B-cell lymphomas and the need to investigate thoroughly patients with disease apparently confined to the skin.

Lymphoma in the ear.ORL J Otorhinolaryngol Relat Spec. 2000 Sep-Oct;62(5):274-7.

BACKGROUND: Malignant tumors of the ear are rare. The most common malignant tumors are squamous cell carcinomas and adenocarcinomas. Lymphoma in the ear is rare. METHODS: We report 2 cases of a primary presentation of a lymphoma of the ear. The literature since 1947 is reviewed. RESULTS: An 83-year-old woman with an anaplastic large cell lymphoma of the skin of the external auditory meatus and a 75-year-old man with a B-cell non-Hodgkin's lymphoma of the mastoid process are presented. The literature review shows that only 16 cases of lymphomas of the ear have been reported so far. CONCLUSIONS: In a case of therapy-resistant otitis and/or peripheral facial paralysis malignancy should be excluded by computer tomography of the mastoid. For histopathological diagnosis unfixed specimens are preferable. Uniform treatment of lymphomas of the ear has not been established.

Lymphoma of the tympanic membrane in acquired immunodeficiency syndrome.Auris Nasus Larynx. 1998 Jan;25(1):89-94.

Lymphoproliferative disease is more common in the immunocompromised host and can occur at unusual sites. Lymphomas of the temporal bone are rare. We present the first case of a large B-cell Lymphoma of the tympanic membrane in a patient with acquired immunodeficiency syndrome. The tympanic membrane is a site rich with antigen-presenting dendritic cells that may play an etiologic role in neoplastic transformation at this site. The staging, treatment and prognosis of an immunocompromised host afflicted with lymphoma is discussed. Future directions in improving survival include better therapy for the primary viral infection and less toxic therapy for the lymphoma.

Petrous bone tumors: primary non-Hodgkin lymphoma of the inner ear canal and cerebellopontile angle.Laryngorhinootologie. 1997 Oct;76(10):625-8.

BACKGROUND: We report about a primary Non-Hodgkin Lymphoma (NHL) of the internal auditory canal. The only previously known manifestations of a NHL in the temporal bone have been infiltrations or hemorrhagic complications due to a late manifestation or advanced systemic disease. Involvement of both temporal bones is typical. CLINICAL CASE: The 60-year-old female patient complained of an acute one-sided deafness, accompanied by a high-pitched tinnitus, rotating vertigo, and paralysis of the left half of the face. RESULTS: We found a deafness in the left ear, spontaneous nystaxis, which was interpreted as a deficiency in excitement of the vestibular organ, and a complete peripheral facial paralysis. Diagnostic imaging studies revealed a large, intrameatal solid mass in the temporal bone, measuring 1.2 x 0.8 cm. Histologic examination after translabyrinthine tumor removal demonstrated a centroblastic Non-Hodgkin Lymphoma. The following extensive interdisciplinary staging examination showed no other tumor manifestations; the CSF analysis was negative. CONCLUSIONS: The uniqueness of this case lies in the detection of a primary nongeneralized centroblastic lymphoma of the internal auditory canal. In contrast to infiltrations of systemic NHL in the same location, in which the advanced disease is responsible for the bad prognosis, this isolated lymphoma of the internal auditory canal seems analogous to extranodal MALT Lymphomas with a better prognosis. The primary extranodal NHL of the temporal bone, not reported in previous studies, is discussed with regard to clinical symptoms, differential diagnoses, and therapeutic strategies.

Primary lymphoma of the internal auditory canal. Case report and review of the literature.Ann Otol Rhinol Laryngol. 1998 Jan;107(1):17-21.

We report a rare case of a primary intracranial B cell lymphoma originating in the internal auditory canal. The clinical manifestations were indistinguishable from those of other, more common tumors of the same region. We achieved total gross tumor removal with preservation of the facial nerve. A detailed histologic examination and a systemic workup confirmed the primary nature of this tumor. To our knowledge, this is the second case reported in the literature of a primary malignant lymphoma originating in the internal auditory canal. This is the first instance that includes immunohistochemical and cytometric studies of fresh tissue. We discuss the management of primary lymphomas of the central nervous system, with special emphasis on their association with acquired immunodeficiency syndrome and other immune system diseases. Awareness of primary central nervous system lymphomas is important, since a greater occurrence of these rare tumors in the cerebellopontine angle is probable in the future.

Angiolymphoid Hyperplasia with Eosinophilia of the External Ear ; Neoplasms of the External Ear ; Squamous Cell Carcinoma of the External Ear ; Verrucous Carcinoma of the External Ear ;   Basal cell carcinoma of the External Ear ; Ceruminous Adenoma of the External Ear ; Pleomorphic Adenoma of the External Ear ; Syringocystadenoma Papilliferum of the External Ear ; Cylindroma of the External Ear ; Ceruminous Adenocarcinoma of the External Ear ; Adenoid Cystic Carcinoma of the External Ear ;Melanocytic Tumours of the External Ear ;Rhabdomyosarcoma of the External Ear ;Benign Fibro-Osseous Lesion of the External Ear ;Osteoma of the Ear (external auditory canal and middle ear) ;Langerhans Cell Histiocytosis of the Ear
 
September 2009

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Primary lymphoma of bilateral external auditory canals.Am J Otolaryngol. 2002 Jan-Feb;23(1):49-52.

We report a rare case of primary lymphoma originating in the bilateral external auditory canals. A 49-year-old man presented with cauliflower-like tumors on both of his external auditory canals. Immunohistopathologic examination and immunoglobulin heavy chain gene rearrangement studies led to a diagnosis of B-cell lymphoma. He had no other systemic involvement. After chemotherapy, total gross tumor removal by surgery was achieved, and no recurrence has been detected during 1 year of follow-up. To our knowledge, this is the third case of malignant lymphoma originating in the external auditory canal to be reported in the literature, and this is the first report that includes immuno histochemical findings of fresh tissue.

Primary lymphoma of the temporal bone. Arch Otolaryngol Head Neck Surg. 1992 ;118(1):83-5.

Involvement of the temporal bone by lymphoreticular neoplasm is rare; all reported cases have been of secondary involvement. This article presents what we believe to be the first two reported cases of primary temporal bone lymphoma. The patients, an elderly man and a boy, both presented with infection of the ear, hearing loss, and facial nerve paresis. In both cases, facial paresis resolved after appropriate chemotherapeutic treatment. Patient presentation and clinical course are discussed in light of published work on temporal bone malignancy. Further investigation, including computed tomography and biopsy, should be considered for patients who present with an apparent middle ear infection unresponsive to medical therapy. The development of facial paralysis in such a patient warrants heightened suspicion of malignancy.

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