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1/9. Mucosa-associated lymphoid tissue lymphoma of the lacrimal gland.

    PURPOSE: Mucosa-associated lymphoid tissue lymphoma recently has been defined as a distinct subtype of non-Hodgkin's lymphoma with characteristic clinicopathologic features. A 37-year-old woman with systemic lupus erythematosus and unilateral mucosa-associated lymphoid tissue lymphoma of the lacrimal gland is described. methods: The tumor was totally excised by lateral orbitotomy. Immunohistochemical studies were performed with UCHL-1, CD20 (L26), leukocyte common antigen, cytokeratin (CAM5), and kappa and lambda light chain antibodies. RESULTS: The tumor was composed of centrocyte-like lymphocytes, cells with plasmacytoid features, and lymphoepithelial lesions. Most of the cells expressed the CD20 protein and were positive for immunoglobulin kappa light chain. The patient received no supplemental therapy. No systemic dissemination or local recurrence occurred during a follow-up of 26 months. CONCLUSION: The features of this case support the association between systemic diseases and the subsequent development of mucosa-associated lymphoid tissue lymphoma.
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2/9. myoepithelioma of the lacrimal gland: report of a case with potentially malignant transformation.

    myoepithelioma of the lacrimal gland is extremely rare and only four cases, one of which was malignant, have been reported in detail. The present report describes a case of lacrimal gland myoepithelioma in a Japanese male with histological features suggestive of potentially malignant transformation. The excised tumor consisted of two components, a central nodular component and a peripheral component surrounding the former. These components were separated by a fibrous tissue. Microscopically, both components were comprised almost entirely of spindle-shaped cells, but with some epithelioid cells containing glycogen granules. Extracellular spaces in the peripheral component were filled with eosinophilic materials with the occasional crystalloid structures, which were immunoreactive for collagen type i. Neoplastic cells were immunoreactive focally for vimentin and S-100, but negative for cytokeratins, epithelial membrane antigen, muscle actin, smooth muscle actin, desmin, myosin, and glial fibrillary acidic protein. The neoplastic cells in the central component showed nuclear pleomorphism and atypia with a higher frequency of mitotic figures, and higher labelings of proliferation markers than those in the peripheral component. Neither invasion, necrosis, nor hemorrhage was observed in the tumor. From these findings we proposed a diagnosis of potentially malignant myoepithelioma.
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3/9. Giant cell angiofibroma of the nasolacrimal duct.

    PURPOSE: To describe clinical and histologic features of the first case, to our knowledge, of giant cell angiofibroma located in the nasolacrimal duct region in a 28-year-old woman. methods: Interventional case report. A left nasolacrimal duct tumor was excised en bloc by lateral rhinotomy. Histopathologic examination was performed with the use of light microscopy. Immunohistochemical staining included S-100 protein, muscle-specific actin, desmin, myoglobin, vimentin, and CD34. RESULTS: The lesion was characterized by haphazardly arranged oval to spindled cells, a myxoid and collagenous stroma, multinucleated giant cells, prominent blood vessels, and pseudovascular spaces. Tumor cells were strongly positive for vimentin and CD34 and were negative for other antigens. After excision, there has been no recurrence over 4 years of follow-up. CONCLUSIONS: Originally described as an orbital tumor, giant cell angiofibroma also may occur in the nasolacrimal duct and lacrimal sac region. This mesenchymal neoplasm should be included in the differential diagnosis of lacrimal drainage system tumors.
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4/9. Angiotropic lymphoma occurring in a lacrimal sac oncocytoma.

    This report describes a case of angiotropic variant of diffuse large B cell lymphoma within a benign oncocytoma of the lacrimal sac. The occurrence of this rare lymphoma within a benign neoplasm has not been documented previously. An 87 year old woman presented with a swelling over the area of the left lacrimal sac, which histological examination revealed to be an oncocytoma. Many small blood vessels within the tumour were filled with large cytologically atypical cells, which stained positively for leucocyte common antigen and a B cell antigen, CD20, confirming the presence of a large B cell non-Hodgkin's lymphoma of angiotropic type. Angiotropic lymphoma is a very rare and usually highly aggressive variant of non-Hodgkin's lymphoma, which classically involves the central nervous system and skin, but has been described within most organs. Its occurrence within a benign neoplasm is probably coincidental, although a close association between oncocytic epithelium and normal lymphoid cells is recognised in Warthin's tumour of salivary and lacrimal glands.
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5/9. adult extrarenal rhabdoid tumor of the lacrimal gland.

    A 50-year-old man presented with a rapidly growing mass in the area of the right lacrimal gland. An initial erroneous histopathologic diagnosis of a pleomorphic adenoma made on a small-incisional biopsy was later corrected to a malignant rhabdoid tumor when a wide local excision of the tumor was performed. The tumor was composed predominantly of dyscohesive, globoid, and eosinophilic cells, which frequently contained cytoplasmic inclusions. These were demonstrated to be composed of whorls of intermediate vimentin filaments. The tumor cells expressed epithelial membrane antigen as well as cytokeratin. Ultrastructurally, they displayed intercellular junctions and interrupted segments of linear basement membrane material. These findings, together with the development of the lesion within the parenchyma of the lacrimal gland, are suggestive of an epithelial origin. The patient was treated with radical surgery and adjunctive radiotherapy and chemotherapy, which are the recommended treatment modalities for this highly malignant tumor.
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6/9. Pleomorphic adenoma arising in an accessory lacrimal gland of Wolfring.

    PURPOSE: To describe a patient with pleomorphic adenoma arising in an accessory lacrimal gland of Wolfring in the lower lid and to illustrate the immunohistochemical and molecular cytogenetics. DESIGN: Single interventional case report. methods: A 62-year-old man presented with a 20-year history of a painless slowly growing mass at the temporal part of the right lower eyelid. Histological, immunohistochemical, and fluorescence in situ hybridization studies of the excised tumor were performed. RESULTS: Histological evaluation showed many glandular elements embedded in a myxoid stroma. The tumor was situated beneath an area of a normal accessory lacrimal gland of Wolfring and in close association with normal meibomian glands. Myoepithelial tumor cells in the myxoid stroma reacted strongly with an antibody against glial fibrillary acidic protein, which did not bind to normal lacrimal gland tissue. Tumor cells with both epithelial and myoepithelial morphologies reacted positively for both pleomorphic adenoma gene-1 and high-mobility group A2 proteins. fluorescence in situ hybridization analysis showed no evidence of clonal translocations or numerical abnormalities involving chromosome 8 or 12. CONCLUSIONS: Pleomorphic adenoma of the accessory lacrimal gland is an exceedingly rare tumor of the ocular adnexa. glial fibrillary acidic protein seems to be a tumor-associated antigen. Genetically, this case of pleomorphic adenoma arising from an accessory lacrimal gland of Wolfring is identical with those originating from salivary glands.
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7/9. Clear cell epithelial-myoepithelial carcinoma arising in pleomorphic adenoma of the lacrimal gland.

    BACKGROUND: A 63-year-old man had an 8-year history of painless proptosis, which had noticeably increased over the last 2 months. A mass was palpable in the left lateral canthus. Computed tomographic studies showed a globular mass with small foci of calcification involving the lacrimal gland. After an incisional biopsy, a histologic diagnosis of clear cell epithelial-myoepithelial carcinoma was made and an orbital exenteration was performed. FINDINGS: Results of histologic examination of the mass showed a partially encapsulated, clear cell epithelial-myoepithelial carcinoma with an associated pleomorphic adenoma (benign mixed tumor). Immunohistochemical studies disclosed strong immunoreactivity to cytokeratin (AE1/AE3), epithelial membrane antigen, S-100 protein, and alpha-actin. CONCLUSION: Although a clear cell myoepithelial carcinoma rarely has been reported in association with a pleomorphic adenoma of the submandibular gland, to the authors' knowledge, this combination has never been reported in the lacrimal gland, nor has a clear cell epithelial-myoepithelial carcinoma ever been reported in this anatomic location. The differential diagnosis of lesions with prominent clear cells involving the lacrimal gland is extensive and includes clear cell variants of acinic cell carcinoma and oncocytoma, mucoepidermoid carcinoma, and others.
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8/9. An Epstein-Barr virus positive undifferentiated carcinoma in the lacrimal sac.

    We report a Chinese patient who presented with metastatic undifferentiated carcinoma in the cervical lymph node of unknown primary origin. Despite a raised IgA titre against Epstein-Barr virus capsid antigen, examination and biopsy of the nasopharynx were negative. radiotherapy was given to the head and neck region with the orbit shielded. There was complete resolution of the metastatic lymph nodes. She developed proptosis of her left eye one year afterward when a large tumour was found in the lacrimal sac region. review of the initial computerized tomography revealed a small soft tissue mass in the same region. The tumour was composed of undifferentiated carcinoma cells associated with dense lymphoid infiltrate. in situ hybridization for Epstein-Barr virus EBER rna showed strong positive signals in the malignant cells. This is the first reported case of an Epstein-Barr virus positive undifferentiated carcinoma with lymphoid stroma in the lacrimal sac. In addition to the nasopharynx, salivary glands, nasal cavity and paranasal sinuses, the lacrimal sac should be considered as a potential primary site for Epstein-Barr virus positive metastatic undifferentiated carcinoma in the cervical lymph nodes.
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9/9. Solitary fibrous tumor of the lacrimal gland fossa.

    BACKGROUND: solitary fibrous tumors are rare spindle cell neoplasms that most commonly arise in the pleura. Recently, solitary fibrous tumors of the orbital soft tissues have been described. The authors report the first case of solitary fibrous tumor arising in the lacrimal gland fossa and discuss features that may distinguish solitary fibrous tumor from other lacrimal fossa lesions. methods: A left lacrimal gland fossa tumor was excised via lateral orbitotomy from a 76-year-old man and analyzed by light microscopy. Immunohistochemical studies were performed using antibodies to CD34, vimentin, epithelial membrane antigen, cytokeratins AE1-3 and Cam5.2, S-100 protein, and actin. RESULTS: The histopathologic features of the tumor were typical of solitary fibrous tumor. A well-circumscribed, variably cellular proliferation of bland spindle cells displayed both "patternless" and storiform growth patterns. Immunohistochemical studies showed that the tumor cells expressed vimentin and CD34 but did not express epithelial membrane antigen, cytokeratins, S-100 protein, or actin. The patient is free of tumor recurrence after 1 year of follow-up. CONCLUSION: The authors report the first case of solitary fibrous tumor arising in the lacrimal gland fossa. The clinical presentation of this tumor was similar to that of pleomorphic adenoma (benign mixed tumor) of the lacrimal gland; fortunately, the treatment for both of these lacrimal gland fossa neoplasms is en bloc excision. The histopathologic appearance of solitary fibrous tumor is varied, and immunohistochemical analysis may help in distinguishing solitary fibrous tumor from other lacrimal fossa lesions. Careful follow-up of patients with solitary fibrous tumor is important because recurrence may present years after excision of the primary tumor.
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