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Caruncular lesions in Denmark 1978-2002: a histopathological study with correlation to clinical referral diagnosis.

PURPOSE: To carry out a retrospective clinicopathological evaluation of caruncular lesions. METHODS: Data were collected from all surgically removed and histopathologically evaluated caruncular lesions registered by Danish pathology departments during the 25-year period 1978-2002. RESULTS: A total of 574 caruncular lesions were identified. The number of caruncular lesions increased significantly during the 25-year period. This was due to an increase in the number of benign lesions, whereas the number of premalignant and malignant lesions remained constant. A total of 550 (96%) of the lesions were benign. Naevus (n = 248, 43%) and papilloma (n = 131, 23%) were the most common neoplasms. Premalignant lesions (n = 10, 1.7%) were dominated by primary acquired melanosis (PAM) with atypia and epithelial dysplasia. Malignant lesions constituted a total of 14 neoplasms (2.4%), with basal cell carcinoma (n = 4, 0.7%) and lymphoma (n = 4, 0.7%) being the most frequent. The preoperative clinical diagnosis was correct in 286 (50%) of cases. CONCLUSION: Caruncular lesions are predominately benign but the lesions are rare and diverse, making clinical diagnosis difficult. Referral of excised lesions for pathological examination is recommended.

Adult↗

Ocular adnexal lymphoid neoplasms: clinical, histopathologic, electron microscopic, and immunologic characteristics.

Clinicopathologic analysis of 400 ocular adnexal lymphoid neoplasms has demonstrated that the orbital lymphoid neoplasms occur primarily in the sixth and seventh decades of life; that their benignancy or malignancy is generally indistinguishable clinically; that the orbital malignant lymphomas are most commonly small cell lymphomas; that the orbital "histiocytic" lymphomas almost always represent an anomalous deposit of disseminated lymphoma; and that the percentage of patients with orbital lymphoma who develop systemic disease varies with the histopathology: two thirds of cases of poorly differentiated lymphocytic lymphomas, as defined cytomorphologically, have associated systemic disease. Prospective correlative clinicopathologic and immunologic analysis of 25 cases has shown that cell marker analysis divides the ocular adnexal lymphoid infiltrates into immunologically polyclonal proliferations, which show diverse but benign histopathologic features, and immunologically monoclonal B cell proliferations, which have the histologic features of malignant lymphomas. The benign, polyclonal ocular pseudolymphomas recapitulate the cell marker profile of a benign reactive lymph node with similar variations in the T cell:B cell ratio. The ocular adnexal and nodal B cell lymphomas are analogous in that they most commonly express surface IgM heavy chains and kappa light chains, express Ia antigens in parallel with SIg, and occasionally contain neoplastic B cells at various developmental stages--i.e., Ia+SIg+ and Ia+SIg-. Correlative immunologic and ultrastructural studies have demonstrated that electron microscopy is a reliable and reproducible technique for indirectly assessing the mono- or polyclonality of an ocular adnexal lymphoid neoplasm. This study is focused on the use of hybridoma-derived monoclonal antibodies, which are capable of detecting maturational stages of B and T cell differentiation and functionally distinct T cell subsets, in order to investigate the interactional and immunoregulatory defects that participate in the generation of the ocular adnexal lymphoid proliferations.

Alkaline Phosphatase↗

Mucoepidermoid carcinoma of the conjunctiva: a series of three cases.

OBJECTIVE: To detail the clinical presentation and outcomes of currently available treatments for mucoepidermoid carcinoma of the conjunctiva (MCC). DESIGN: Retrospective noncomparative case series. PARTICIPANTS: Three patients ranging from 40 to 63 years of age with MCC participated. INTERVENTION: Excisional biopsies and various therapies were performed. MAIN OUTCOME MEASURES: Clinical and surgical outcomes were measured. RESULTS: Patient 1 is a 55-year-old man with right temporal MCC. He underwent two local excisions with adjuvant cryotherapy and has had no recurrence at 31 months follow-up. Patient 2 is a 63-year-old man with right temporal MCC who underwent fractionated iodine 125 plaque radiotherapy. He had a recurrence approximately 8 months after plaque treatment and subsequently underwent enucleation of the right eye. Clinical follow-up examinations revealed no further recurrence at 17 months. Patient 3 is a 40-year-old woman treated for right MCC with carbon dioxide laser with recurrence at 3 weeks. She subsequently underwent radiation treatment with the development of regional lymph node metastases 16 months later. CONCLUSIONS: MCC is a rare neoplasm that displays an extraordinary capacity for aggressive local invasion. This series of three case reports demonstrates the high recurrence rate of MCC and the response of this tumor to different current modalities of treatment. Extended follow-up is required with this tumor because distant metastases can occur very late.

Adult↗

Mucoepidermoid carcinoma of the conjunctiva with intraocular invasion.

A 70-year-old man developed intraocular invasion from a recurrent tumor of the nasal perilimbal conjunctiva of the right eye six months after the original tumor had been resected. Examination of the enucleated right globe disclosed massive invasion of the nasal one-half of the anterior segment by a mucoepidermoid carcinoma of the conjunctiva. The neoplasm demonstrated mainly epidermoid features, including the production of a substantial amount of intraocular keratin. However, a segment of tumor along and within the iris stroma showed almost exclusively mucus-secretory features with a mucinous material within individual tumor cells and cysts lined by tumor cells. The original biopsy specimen demonstrated only the epidermoid and not the mucoid component of the carcinoma. Mucoepidermoid carcinoma of the conjunctiva appears to be more locally aggressive than conventional squamous cell carcinoma as is characterized by its tendency for early recurrence associated with a high incidence of intraocular and orbital invasion.

Aged↗

Antimetabolites in ocular surface neoplasia.

Malignant neoplasms of the ocular surface are uncommon but consist of squamous and melanocytic tumors. Surgical excision has been the mainstay of treatment and is perhaps the "gold standard." However, in those circumstances in which surgical intervention is not feasible, adjunctive treatments have been advocated. Recent attention has been given to the use of mitomycin C and 5-fluorouracil for the treatment of ocular surface squamous neoplasia. This article reviews the major publications relating to the use of antimetabolites in ocular surface neoplasia and highlights the most recent contributions to the ophthalmic literature.

Antimetabolites, Antineoplastic↗

Mucoepidermoid carcinoma of the conjunctiva.

Mucoepidermoid carcinoma of the conjunctiva (MECa) is a rare neoplasm. It resembles squamous cell carcinoma (SCCa), clinically and histologically. Nevertheless, it is characterized by a high degree of local aggressiveness. We reviewed all cases of MECa reported in the English literature for epidemiological data, characteristics of the tumor, treatment, and outcome. We found that MECa, reported predominantly in aging males (median age 71 years), arises in the limbal and perilimbal areas. Simple excision is followed by rapid recurrence, and most tumors will require enucleation or exenteration for local control. Histological stains directed to the production of mucin are essential for the diagnosis. The recommended treatment is wide local excision. Enucleation or exenteration is recommended for tumors invading the globe or orbit.

Carcinoma, Mucoepidermoid↗

Epibulbar subconjunctival fibroma. A tumor possibly arising from Tenon's capsule.

A fibroma, a benign neoplasm arising from the periorbita or tendon sheaths, is described in a 74-year-old woman as an indolent, lobular tumor situated in the episcleral and subconjunctival connective tissues with superior limbal fixation. Because most fibromas arise from tendon sheaths or fascial planes, we speculate that our lesion arose from Tenon's capsule. The rarity of periocular fibroma is documented by a review of the modern ophthalmic literature. Histopathologically, the tumor was composed of a paucicellular population of fibroblasts, sometimes displaying hyperchromatic nuclei and widely separated by abundant broad bundles of collagen. Mast cells were infrequently dispersed in the lesion. A wide local excision was performed, and no recurrence has developed during an 18-month follow-up period.

Aged↗

Epibulbar granular cell tumor. Report of a case and review of the literature.

BACKGROUND: The granular cell tumor (GCT) is a common, usually benign, neoplasm of uncertain histogenesis that may affect any organ in the body. It occurs rarely in ophthalmic sites, where orbital location is most frequent. The purpose of this report is to describe a unique case of an epibulbar, subconjunctival GCT in a child. METHOD: Histopathologic examination of the excised tumor included standard and immunohistochemical staining of formaldehyde-fixed, paraffin-embedded tissue. RESULTS: Histopathologic study showed the characteristic morphology of GCT. Immunohistochemistry was strongly positive for vimentin, weakly and partly positive for neuron-specific enolase, and negative for S-100 and HAM-56. CONCLUSIONS: Granular cell tumor must be added to the differential diagnosis of epibulbar masses. Immunohistochemistry of the tumor in this case suggests an uncommitted mesenchymal cell origin.

Antibodies↗

Survival after malignant tumors of the orbit and periorbit treated by exenteration.

PURPOSE: In order to further assess the survival value of orbital exenteration in malignant orbital and periorbital tumors. METHODS: The charts of 44 patients exenterated for a neoplasm of the orbit or periorbit were reviewed in a retrospective study. RESULTS: The overall 4-year survival was 45%. 26 patients had free margins on histological examination and 24 patients had developed local recurrence or metastasis. Local recurrence or metastasis were significantly more common in the group with transected margins than in the group with free-margins (p= 0.01). Survival between the group of patients with local recurrence or metastasis and the group without local recurrence or metastasis showed statistically significant difference (p=0.0025). In contrast, survival between the group of patients with free margins and the group with transected margins did not show statistically significant difference (p=0.13). CONCLUSION: Surgical free margins section is a key element in successful cancer surgery but seems not the only prognosis variable.

Adolescent↗

Lymphoproliferative disease of the ocular adnexa: a clinical and pathologic study with statistical analysis of 69 patients.

PURPOSE: To evaluate the clinical features, treatment, and outcomes of a cohort of patients with ocular adnexal lymphoproliferative disease classified according to the World Health Organization modification of the Revised European-American Classification of Lymphoid neoplasms and to perform a robust statistical analysis of these data. METHODS: Sixty-nine cases of ocular adnexal lymphoproliferative disease, seen in a tertiary referral center from 1992 to 2003, were included in the study. Lesions were classified by using the World Health Organization modification of the Revised European-American Classification of Lymphoid neoplasms classification. Outcome variables included disease-specific survival, relapse-free survival, local control, and distant control. RESULTS: Stage IV disease at presentation, aggressive lymphoma histology, the presence of prior or concurrent systemic lymphoma at presentation, and bilateral adnexal disease were significant predictors for reduced disease-specific survival, local control, and distant control. Multivariate analysis found that aggressive histology and bilateral adnexal disease had significantly reduced disease-specific survival. CONCLUSIONS: The typical presentation of adnexal lymphoproliferative disease is with a painless mass, swelling, or proptosis; however, pain and inflammation occurred in 20% and 30% of patients, respectively. Stage at presentation, tumor histology, primary or secondary status, and whether the process was unilateral or bilateral were significant variables for disease outcome. In this study, distant spread of lymphoma was lower in patients who received greater than 20 Gy of orbital radiotherapy.

Adolescent↗

[Optimization of methodology and extension of indications for beta therapy of neoplasms of the eyelids, conjunctiva and cornea].

The paper offers a theoretical foundation and results after clinical approbation of the method developed by the authors for beta therapy by enlarged single doses using, if necessary, a speeded-up fractionating and split courses of radiation. Beta therapy was used in 220 patients with tumors of the eye lids, conjunctiva and cornea, single doses of radiation, 40 Gr daily or each other day. By their morphologic type the tumors were mostly malignant epithelial and pigmented new-formations (sarcoma, melanoma). In the follow-up period, from 1 to 6 years, the following results were recorded: the treatment proved effective for epithelial tumors in 97%, melanocytic--in 73%; recurrences appeared in 3%; the number of postradiation complications made up 6.6%, of them disturbances in visual functions--2%: death from metastases in melanoma--8.7% of cases. The proposed method of radiation allowed to increase indications for beta therapy of this pathology to 5-6 mm of the tumor protrusion, to decrease the number of complications and terms of treatment. As a result of computer analysis of clinical results, a new method for calculation of optimal summary doses of beta radiation was worked out, that can be used by practitioners in conducting beta therapy for tumors of the eye lids, conjunctiva and cornea.

Beta Particles↗

[Inverted transitional cell papilloma of the conjunctiva with peripheral carcinomatous transformation].

BACKGROUND: Inverted papilloma is a benign epithelial neoplasm of mucous membrane, usually arising in the nose and paranasal sinuses, much rarer of the conjunctiva. Histologically ribbons or bulbous expansions of the epithelium invaginate into the underlying connective tissue. In opposite to their sinonasal counterparts the few described papillomas of the conjunctiva til now have not shown any signs of malignant transformation or recurrence after local excision. PATIENT: A 96-year old woman presented with a solid tumour on the nasal bulbar conjunctiva of her right eye, which had persisted at least over 6 months. Ocular examination showed a 25 x 15 x 15 mm reddish tumour with papillary surface. Histologic features of the excised specimen were consistent with inverted papilloma, but here were significant carcinomatous foci in peripheral areas. Four months later there was a tumour recurrence on the bulbar and tarsal conjunctiva. CONCLUSIONS: Inverted papilloma of the conjunctiva is an unusual diagnosis and to date there is no previous report of malignant transformation. With only seven cases described so far the biologic behavior remains uncertain, but it seems to be more aggressive than assumed so far. Complete excision is the treatment of choice, but patients should be carefully observed and adjuvant therapy could be necessary to ensure tumour control.

Aged↗

Conjunctival 'MALToma' with systemic recurrence.

We report the recurrence of a localized conjunctival lymphoma arising in mucosal-associated lymphoid tissue (MALToma) in the psoas muscle, 18 months after initial treatment with radiotherapy. Findings from systemic investigations demonstrated MALToma recurrences in the psoas muscle and the stomach. Ocular adnexal MALTomas typically manifest as localized tumors, which respond well to radiotherapy, but a proportion may recur in typical MALT sites. To our knowledge, this is the first report of a recurrence in the psoas muscle, and the clinical implication is that all patients with ocular adnexal lymphomas need to be followed up for an indefinite period.

Antineoplastic Combined Chemotherapy Protocols↗

Primary basal cell carcinoma of the caruncle.

We describe a 24-year-old man with primary basal cell carcinoma of the caruncle. Clinically the lesion was a whitish, slightly prominent nodule surrounded by fine vessels. No associated cutaneous lesion and no connection to the surrounding skin was present. The lesion was subsequently completely excised, and histopathological examination revealed a solid-cystic basal cell carcinoma of the caruncle. Primary basal cell carcinoma of the caruncle is an extremely rare but distinct entity. To our knowledge, review of the literature has not demonstrated a previous photographically documented case of primary basal cell carcinoma of the caruncle.

Adult↗

Intraocular neoplastic cyst from mucoepidermoid carcinoma of the conjunctiva.

An 89-year-old woman with recurrent conjunctival mucoepidermoid carcinoma developed intraocular inflammation and an elevated fundus lesion that simulated choroidal detachment in her affected right eye. Intraocular invasion of squamous cell carcinoma was suspected and the eye was enucleated. Pathologic examination of the enucleated eye showed intraocular invasion by conjunctival mucoepidermoid carcinoma that formed a suprauveal cyst lined with malignant epithelial cells. The patient developed an orbital recurrence 1 year later and underwent orbital exenteration. She died 2 years later from an unrelated cause. Conjunctival mucoepidermoid carcinoma can exhibit intraocular invasion and produce an intraocular neoplastic cyst.

Aged↗

Four-eyelid sebaceous cell carcinoma following irradiation.

BACKGROUND: Sebaceous cell carcinoma is a distinctive tumor of the eyelid associated with a high rate of metastasis and mortality. Involvement of both upper and lower lids has been described; however, involvement of all 4 eyelids is extremely rare. OBJECTIVE: To describe the evaluation and diagnosis of the clinicopathologic features in a 74-year-old patient with 4-eyelid sebaceous carcinoma and a history of whole face irradiation for eczema. METHODS: Bilateral eyelid, conjunctiva, map, and fine needle biopsy of enlarged, submandibular lymph node specimens were stained with hematoxylin-eosin and oil-red-O for light microscopy and fresh-frozen sections were evaluated by electron microscopy. RESULTS: The biopsy specimens from the eyelids and conjunctiva showed sebaceous carcinoma cells in the meibomian glands and scattered within the conjunctival epithelium. The cells were moderately well differentiated. Similar cells stained with oil-red-O were found in the submandibular lymph nodes. CONCLUSIONS: The appearance of bilateral sebaceous carcinoma supports the concept of simultaneous occurrence of multiple primaries in specific patients, especially after radiation therapy. Patients with a history of facial irradiation and atypical eyelid lesions should be evaluated for malignant tumors such as sebaceous carcinoma.

Adenocarcinoma, Sebaceous↗

Conjunctival dermoid cyst seen on examination as a chronically red eye.

We describe a patient who was seen with a unilateral red eye and purulent discharge refractory to antibiotic treatment with multiple antibiotic regimens over the previous 4 months. Initially it was believed to be a bacterial conjunctivitis, but when conventional treatments failed it was thought to be a viral conjunctivitis with bacterial superinfection. Cultures yielded only Enterococcus. There was a small mass with a draining fistulous tract at the lateral canthus. Symptoms persisted despite courses of topical and systemic antibiotics followed by a tapering dose of steroids. The patient was taken to the operating room for an orbitotomy through the conjunctiva at the lateral canthus. Findings from pathologic examination of the excised mass demonstrated a dermoid cyst of the conjunctiva. This clinical appearance obscured the diagnosis.

Adult↗