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1/34. Recurrent lacrimal sac papilloma: case report.

    Tumors of the lacrimal sac are rare. Benign papillomas comprise approximately 40% of all neoplasms of the lacrimal drainage system. They often present insidiously with symptoms of dacryostenosis or dacryocystitis. Recurrent bouts of dacryocystitis and nasolacrimal duct obstruction were reported in a 35-year-old man over a period of 13 years. A medial canthal mass was noted in the 6th year after the onset of symptoms. A tumor was discovered incidentally during surgical intervention for presumed dacryostenosis. Surgical removal of the tumor and dacryocystorhinostomy were performed. The histopathologic report turned out to be benign papiloma. Local recurrences occurred during the follow-up period. In addition to surgical excision, we applied cryotherapy and CO2 laser to prevent further recurrence. This case we presented the characteristic recurrence of lacrimal sac papilloma and implied the possibility of tumor occurrence in a patient with recurrent dacryocystitis. We must bear in mind that a recurrent dacryocystitis may be a presentation of a lacrimal sac tumor, because early diagnosis and aggressive treatment can prevent recurrence and result in a cure.
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2/34. 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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3/34. Acinic cell carcinoma of the lacrimal gland with intracranial extension: a case report.

    PURPOSE: To describe a patient with acinic cell carcinoma of the lacrimal gland presenting with atypical manifestations including erosion of the orbital roof and intracranial extension. We compare his clinical manifestations with two previously reported cases. methods: Case report. RESULTS: A 51-year-old man presented with a firm, tender, palpable mass in the superior temporal portion of his right orbit. CT scan demonstrated a large homogeneously enhancing mass displacing the globe inferiorly and eroding the roof of the orbit. MRI of the orbit showed intracranial extension of tumor. biopsy revealed an acinic cell carcinoma of the lacrimal gland. CONCLUSIONS: Acinic cell carcinoma is a rare neoplasm occurring primarily in the major and minor salivary glands.
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4/34. Extranodal marginal zone B-cell lymphoma of the lacrimal gland associated with crystal-storing histiocytosis.

    OBJECTIVE: Crystal-storing histiocytosis (CSH) is a very rare immunoglobulin (Ig) deposition disorder that can be associated with B-cell neoplasms as well as some reactive Ig-secreting lymphoproliferative disorders. This article reports the clinical, histopathologic, and molecular biologic findings of CSH in association with an extranodal marginal zone lymphoma (EMZL) of the lacrimal gland. DESIGN: Interventional case report with clinicopathologic correlation. INTERVENTION: Treatment consisted of conjunctival and orbital biopsies, as well as low-dose radiation of the left orbit. methods: Histopathologic findings of the conjunctival and orbital biopsies were compared. Further, extensive immunohistochemistry, polymerase chain reaction (PCR) for the detection of Ig heavy chain (IgH) gene rearrangement, Gene Scan analysis, and dna sequencing were performed on all tissues. RESULTS: The tumor manifestations in the specimens demonstrated a similar morphologic and immunophenotypic characteristics consistent with the diagnosis of EMZL. Immunoglobulin H PCR and Gene Scan analysis showed B cells derived from the same clone. In association with the orbital EMZL were large accumulations histiocytes filled with refractile crystals, consistent with the diagnosis of CSH. CONCLUSIONS: The current case describes an EMZL of the lacrimal gland appearing 5 years after excision and low-dose radiation of a conjunctival lymphoma. The orbital recurrence was associated with surrounding CSH, a rare Ig storage disorder more often reported in patients with multiple myeloma or lymphoplasmacytic lymphoma or immunocytoma. Ophthalmic pathologists should be aware of the diagnosis when reviewing orbital biopsies, because difficulties may arise when the crystal-storing macrophages occupy more tissue space than the Ig-secreting cells or when they resemble cell types such as rhabdomyoblasts.
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5/34. Pleomorphic adenoma of the lachrymal gland: presentation of a clinical case of relapse.

    Pleomorphic adenoma affecting the lachrymal gland requires a well-grounded clinical and therapeutic protocol to avoid the risk of malignant transformation or disease recurrence, which is quite dangerous at this site. The authors present a clinical case of pleomorphic adenoma recurrence affecting the right lachrymal gland in a patient who first underwent a biopsy, with subsequent resection of the neoplasm. The authors also review the clinical and radiological features enabling a differential diagnosis and describe the radical "exenteratio orbitae" surgery for pleomorphic adenoma recurrence 7 years after the first operation, which was performed because of total derangement of the orbital cavity. The patient remains disease-free 5 years after this surgical treatment. Therefore, it turns out that an incisional biopsy carries a higher biological cost for the patient.
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6/34. neurofibroma of the lacrimal sac.

    neurofibroma is an extremely rare neural tumor of the lacrimal sac. We present a case of neurofibroma of the lacrimal sac in a 45-year-old lady. Based on the history of intermittent epiphora, presence of a mass in the lacrimal sac region, dacryocystogram suggestive of space occupying lesion within the lacrimal sac and computed tomographic image of a tumor lying in the lacrimal fossa region, a tentative diagnosis of lacrimal sac neoplasm was made. Excision of the tumor with repair of the lacrimal sac was performed. Histopathology proved it to be neurofibroma of the lacrimal sac. Until now, only four cases have been reported worldwide. The paucity of reported cases limits our knowledge regarding the natural course, treatment, results and prognosis of this tumor.
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7/34. 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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8/34. Primary sebaceous carcinoma of the lacrimal gland treated by carbon ion radiotherapy.

    Sebaceous carcinoma is a rare primary neoplasm of the lacrimal gland and, to the best of our knowledge, only six cases have been reported previously. Sebaceous carcinoma of the orbit more commonly occurs as secondary invasion from the eyelid, but may occur by way of metastatic spread from elsewhere in the body. We describe a patient who presented with a rapidly growing neoplasm of the lacrimal gland which, histologically, was a sebaceous carcinoma. The eyelid was entirely normal on examination. In this patient we found a single tumor cell line within the normal lacrimal gland. In this article we describe the diagnosis and treatment with carbon ion radiotherapy of primary sebaceous carcinoma of the lacrimal gland, a condition not previously reported in japan.
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9/34. hemangiopericytoma of the lacrimal sac.

    hemangiopericytoma is a rare soft-tissue neoplasm composed histologically of pericytes. hemangiopericytoma of the lacrimal sac is exceedingly rare. This case provided the first known computed tomographic image of a hemangiopericytoma of the lacrimal sac prior to surgical intervention.
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10/34. Calcified orbital cyst simulating a malignant lacrimal gland tumor in an adult.

    A 56-year-old woman with left periocular pain was discovered on CT to have a partially calcified, circumscribed cystic lesion in the lacrimal gland fossa, suggestive of a malignant lacrimal gland tumor. Adjacent bone fossa formation without erosion was noted. Histopathologic examination of the excised mass disclosed a cyst with chronic granulomatous inflammation and calcification of the cyst wall. The combination of chronic pain, cystic changes, and calcification in a lacrimal gland fossa mass in an adult should suggest a malignant neoplasm, but a long-standing orbital cyst with calcification of its rim can produce the same features.
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