Cases reported "Conjunctival Neoplasms"

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1/24. Primary intraepithelial sebaceous gland carcinoma of the palpebral conjunctiva.

    Sebaceous gland carcinoma usually arises from meibomian or Zeis glands deep within the eyelid, but it can rarely arise within the conjunctival epithelium without a deep component. We describe a woman with a history of chronic blepharoconjunctivitis unresponsive to topical medications. Examination disclosed confluent papillary hypertrophy of the upper palpebral conjunctiva and deposits of white flaky material. Tarsoconjunctival punch biopsy revealed intraepithelial sebaceous gland carcinoma. Management consisted of frozen section-controlled complete tumor excision with removal of the entire posterior lamella of the right upper eyelid, cryotherapy to the margins, and reconstruction. Histopathologic analysis confirmed primary sebaceous gland carcinoma localized to the conjunctival epithelium without involvement of underlying meibomian or Zeis glands or the caruncle. patients with unexplained chronic unilateral blepharoconjunctivitis or papillary hypertrophy of the palpebral conjunctiva should be considered for biopsy to rule out neoplasia, even when there is no sign of an underlying eyelid mass.
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2/24. Sentinel node biopsy for orbital and ocular adnexal tumors.

    PURPOSE: To describe a technique for sentinel node mapping and biopsy in patients with orbital or adnexal tumors. methods: Five patients with orbital and adnexal tumors were studied. Two patients had malignant eyelid melanomas (one of the skin and one of the conjunctiva), one with orbital invasion. Two patients had sebaceous gland carcinoma, and one patient had a mucoepidermoid carcinoma of the conjunctiva; 500 microCi of technetium-99m sulfur nanocolloid (Nycomed Amersham, Princeton, NJ) diluted to 1.0 mL was injected intradermally at the lateral canthus. The patients were positioned as they would be during surgery. lymphoscintigraphy was performed by means of anterior, lateral, and oblique views. The tracer was followed to the first lymphatic basin, and the sentinel node was identified. Cutaneous markers were placed to denote the site. During surgery, lymphoscintigraphy scans and a hand-held gamma probe were used to locate the sentinel node. Once excised, the sentinel node was sent for histopathology. Frozen sectioning confirmed the presence of lymphoid tissue. Permanent sections with immunohistochemical markers were performed to examine for metastatic disease. RESULTS: The sentinel node biopsy technique was applied to 5 patients with orbital and adnexal tumors. All lymph nodes were free of tumor on histopathologic examination. CONCLUSIONS: Sentinel node mapping and biopsy are possible for orbital and adnexal tumors. The morbidity of elective lymph node dissection and adjuvant radiotherapy can be avoided. Our results are preliminary, and further work must be done to identify the lymphatic basins of the orbit and ocular adnexa.
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3/24. Pre- and posttreatment MR imaging in AIDS-related Kaposi sarcoma of the conjunctiva and lacrimal gland.

    AIDS-related Kaposi sarcoma can affect the eye: the (bulbar) conjunctiva and lacrimal gland being rare sites of occurrence. We present a case of AIDS-related Kaposi sarcoma of the conjunctiva in which MR imaging was effective in suggesting the diagnosis and aiding therapeutic strategy. We also discuss advances in imaging of ocular and orbital malignancies presented in the recent literature.
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4/24. Sebaceous gland adenoma of the tarsal conjunctiva in a patient with muir-torre syndrome.

    PURPOSE: To highlight the recognition and diagnosis of muir-torre syndrome (MTS) in patients with sebaceous tumors of the eyelid/conjunctiva and to determine the role of immunohistochemical markers as a screening test in patients with muir-torre syndrome. DESIGN: Single interventional case report. methods: The clinical and family history was reviewed from the records of a 41-year-old man who had a sebaceous gland adenoma of the tarsal conjunctiva of the left upper eyelid. The lesion was completely excised and submitted for histopathologic examination. Immunohistochemical stains using an antibody to MSH2 were performed on the paraffin-embedded sections of the tumor. RESULTS: Histopathologic examination of the tumor showed a lobular pattern with basaloid cells at the periphery of the lobules with central areas of sebaceous differentiation. Immunohistochemical stains showed a lack of MSH2 expression in the tumor, which is highly consistent with MTS. CONCLUSIONS: muir-torre syndrome should be considered in patients initially seen with sebaceous tumors of the eyelid. immunohistochemistry for MSH2 is a practical initial approach to screen for MTS in patients with sebaceous tumors.
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5/24. Malignant lymphoma of the ocular adnexa associated with the benign lymphoepithelial lesion of the parotid glands. Report of two cases.

    BACKGROUND: The benign lymphoepithelial lesion of the salivary/lacrimal glands is generally regarded as a lymphoproliferative disorder that may be associated with Sjogren syndrome. Although lymphomatous transformation in patients with Sjogren syndrome is well documented, few reports have appeared describing similar findings in patients with a benign lymphoepithelial lesion. methods: The authors report the unusual occurrence of malignant lymphoma involving the ocular adnexa in two patients with a histopathologically documented benign lymphoepithelial lesion of the parotid glands. RESULTS: Both patients developed B-cell lymphomas. The eyelid tumor in case 1 expressed IgM/kappa cell surface markers by flow cytometry, and the orbital/conjunctival masses in case 2 showed neoplastic nodules of B cells that were only immunoreactive to lambda light chains (avidin-biotin complex method). CONCLUSION: To the authors' knowledge, this is the first report of ocular adnexal lymphoma arising in patients with a benign lymphoepithelial lesion of the parotid glands. This report establishes that ocular adnexal lymphoma may arise in a patient with a preexisting benign lymphoepithelial lesion.
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6/24. Orbitotemporal neurofibromatosis. Clinical features and surgical management.

    PURPOSE: To classify the periorbital deformities of adult orbitotemporal neurofibromatosis (NF) and describe new clinical findings, and to recommend guidelines for surgical treatment and management of surgical complications. DESIGN: Retrospective noncomparative case series. PARTICIPANTS: Thirty-three patients over age 16 with orbitotemporal NF. methods: Retrospective surgical case record and serial photographic review recording the laterality and the severity of periorbital involvement, the presence of complications from previous surgery, the surgical techniques undertaken, and the surgical outcome and complications. MAIN OUTCOME MEASURES: Comparison of preoperative and postoperative level of deformities. RESULTS: New classification of periorbital deformities: (1) brow ptosis, (2) upper lid infiltration with ptosis, (3) lower lid infiltration, (4) lateral canthal disinsertion, and (5) conjunctival and lacrimal gland infiltration. Two patients had bilateral and 31 patients (94%) had unilateral orbitotemporal NF. All patients had upper and 19 patients (58%) had lower lid involvement. Six (18%) patients had significant brow infiltration. Fourteen (42%) patients had a dropped lateral canthus requiring surgical reattachment, 28 (85%) required anterior levator resection for ptosis, and 28 (85%) had lid-debulking surgery. New findings included severe brow infiltration, lacrimal gland involvement, and functional nasolacrimal duct obstruction. Complications from previous surgery included residual ptosis, ptosis overcorrection, poor lid contour, dry eye, corneal exposure, and upper and lower lid entropion/ectropion. CONCLUSIONS: The periorbital appearance and comfort of patients with NF type 1 who have orbitotemporal NF can be significantly improved through oculoplastic surgery.
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7/24. Sebaceous gland carcinoma of the eyelid presenting as a conjunctival papilloma.

    An unusual presentation of sebaceous carcinoma of the eyelid is described in a 96-year-old man who presented with a large papillomatous palpebral conjunctival lesion in the left upper eyelid. The patient underwent a shave excision of the lesion, followed by a full thickness excision with paraffin section margin control. Histopathology revealed a sebaceous gland carcinoma with no evidence of pagetoid spread. Although rare, sebaceous gland carcinoma should be considered in the differential diagnosis of a conjunctival papilloma.
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8/24. A spectrum of bilateral squamous conjunctival tumors associated with human papillomavirus type 16.

    Three patients with bilateral tumors presenting as multiple keratinizing and verrucous lesions of the bulbar and tarsal conjunctiva were determined by dna amplification and hybridization studies to harbor human papillomavirus type 16 (HPV-16). Results of biopsy in two patients showed infiltrating squamous cell carcinoma in one eye and dysplasia or carcinoma in situ in the fellow eye. In the third patient, focal, inflamed, hypertrophic, papillary lesions with pseudoglandular invaginations of the surface epithelium were found in the tarsal conjunctivae of both eyes. These are the first documented cases of bilateral conjunctival tumors associated with human papillomavirus.
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9/24. Peribulbar oncocytoma: high-frequency ultrasound with histopathologic correlation.

    The clinical, ultrasonographic, and histopathologic characteristics of an oncocytoma of the conjunctiva are described. A 49-year-old woman presented with a red-orange lesion adjacent to the caruncle in her left eye. A clinical examination including high-frequency ultrasound was performed prior to excisional biopsy of her tumor. High-frequency ultrasound demonstrated an epibulbar tumor with low internal reflectivity and a cystic component. There was no involvement of the underlying sclera. Multiple hypoechoic components of the tumor stroma were correlated to multiple cystic glandular structures on histopathologic evaluation. The excised tumor was diagnosed to be an oncocytoma.
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10/24. multiple endocrine neoplasia type III.

    The multiple endocrine neoplasia (men) syndromes are autosomally dominant inherited disorders in which hyperplastic or neoplastic changes occur in a wide variety of tissues. The specific syndromes are classified according to the endocrine glands affected. men type I consists of an aggregation of tumors of parathyroid, pancreatic, and pituitary glands. The association of medullary carcinoma of the thyroid (MCT) and pheochromocytoma is called men type II or type IIA, and if combined with mucosal neuromas, intestinal ganglioneuromatosis, and prominent corneal nerves, is named men type III or type IIB. Individuals afflicted with men type III are characterized by a marfanoid habitus, mucosal neuromas involving oral and ocular tissues, and a number of ophthalmologic findings including prominent corneal nerves, thickened eyelids, and subconjunctival neuromas. These features are easily recognized during the ocular exam, allowing the ophthalmologist to make an early diagnosis of this syndrome prior to the onset of life-threatening manifestations like medullary thyroid carcinoma and pheochromocytoma.
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