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PubMed2006

Uveal and conjunctival malignant melanoma in denmark 1943-97: observed and relative survival of patients followed through 2002.

PURPOSE: To evaluate the observed and relative survival of patients diagnosed with a malignant melanoma in the ocular region in Denmark during the period 1943-97. METHODS: The study included 2,504 patients (1,292 men and 1,212 women) diagnosed with a melanoma in the ocular region, of which 2,434 cases could be topographically subclassified into 2,178 in the choroid/ciliary body, 141 in the iris, and 115 in the conjunctiva. The patients were followed through 2002 and the observed survival proportions and relative survival ratios were estimated. RESULTS: For the total ocular region and the choroid/ciliary body, the observed survival did not vary statistically significantly with the year of diagnosis. A statistically insignificant higher observed survival for women than men was found for tumors in the ocular region and the subgroups choroid/ciliary body, iris, and conjunctiva. During the 55-year study period, the 5- and 10-year relative survival remained stable for the ocular region for men at 67% and 57% and for women at 71% and 60%, respectively, and stable for the choroid/ciliary body for men at 66% and 55% and for women at 69% and 57%, respectively. The 5- and 10-year relative survival for the iris was for men 90% and 85% and for women 99% and 101%, respectively, and for the conjunctiva for men 83% and 70% and for women 93% and 82%, respectively. CONCLUSION: The observed and relative survival of patients diagnosed with a melanoma in the ocular region and choroid/ciliary body in Denmark during the period 1943-97 and followed through 2002 has remained stable. The highest observed and relative survival was found for iris melanomas, the lowest for choroid/ciliary body melanomas, and intermediate for conjunctival melanomas.

Adolescent↗
PubMed2002

Effectiveness of local radiotherapy in primary extranodal marginal zone B-cell lymphoma of MALT or MALT lymphoma of conjunctiva: study of four cases.

Primary lymphomas of the conjunctiva are extremely infrequent and usually belong to extranodal marginal zone B-cell lymphoma of MALT or MALT lymphoma. Radiotherapy with lens shielding is one of the most employed therapeutic options. Four patients with MALT lymphomas of the conjunctiva with complete and maintained response to radiotherapy are reported. The effectiveness as well as the lack of significant toxicity of radiotherapy are emphasized.

Adolescent↗
PubMed2006

Conjunctival low-grade non-Hodgkin's lymphoma: a large single-center study of initial characteristics, natural history and prognostic factors.

To define the initial characteristics and prognostic factors of patients with conjunctival low-grade malignant lymphoma, all patients treated for low-grade lymphoma with initial conjunctival involvement were reviewed. Forty-nine cases were selected, including 45 cases with exclusive ophthalmologic conjunctival involvement. Pathologic review showed 55% of mucosa-associated lymphoid tissue type lymphoma, and 23% of lymphoplasmocytic lymphoma. Initial characteristics were median age of 62 years, nodal involvement in 17% of cases, and stage IV in 22% of patients with 10% of bone marrow involvement. With a median follow-up of 75 months, the 5-year disease-free survival (DFS) and overall survival were 65% and 83%, respectively. On multivariate analysis, nodal involvement was the only factor with a pejorative impact on DFS. Our patient cohort represents one of the largest published series defining the characteristics and prognostic factors of primary conjunctival low-grade malignant lymphoma.

Age Factors↗
PubMed2001

Modified responses to PGE2 in polyamine biosynthesis by T lymphocytes of gastric- and conjunctiva basal cell-carcinoma patients.

The response to Prostaglandin (PG) E2 of T cells from gastric carcinoma (GC)- and conjunctiva-basal cell carcinoma (conjunctiva-BCC)-bearing patients has been studied in relation to polyamine metabolism. Polyamines are crucial co-factors in cell growth as well as differentiation and many works report that lymphocyte spermine (SP), spermidine (SPD) and putrescine (PUT) levels may be related to tumor proliferation. The present work aims to detect the basal and PGE2 induced concentrations of these polyamines and cAMP, ornithine decarboxylase (ODC), spermine N1-acetyltransferase (SAT) activities of T lymphocytes drawn from patients suffering from GC and conjunctiva-BCC since many carcinomas are characterized by high levels of PGE2. Data obtained from lymphocytes of neoplastic subjects were compared with those derived from PGE2-treated control lymphocytes. Results highlight a very significant increase of all the polyamine metabolites in PGE2-treated T cells from neoplastic patients in respect to the untreated and PGE2-treated control lymphocytes. Therefore, it is conceivable that the PGE2 content increase, often occurring during the epithelial tumour development, may contribute, through enhancement of polyamine metabolism, to tumor progression.

Acetyltransferases↗
PubMed1984

Lymphocytic infiltrates of the conjunctiva and orbit: immunohistochemical staining of 16 cases.

The authors have performed frozen section immunologic stains on 16 cases of ocular lymphocytic infiltrates and correlated the results with clinical and histologic findings. Their cases included inflammatory pseudotumor (3), reactive lymphoid hyperplasia (3), atypical lymphocytic infiltrate (9), and small cleaved cell lymphoma (1). Seven of the nine cases with an atypical lymphocytic infiltrate expressed one immunoglobulin light chain, while only one of six considered reactive on histologic evaluation had immunologic results suggestive of a neoplastic B cell proliferation. The case of follicular small cleaved cell lymphoma expressed B lineage antigens but did not express immunoglobulin; this patient died of disseminated lymphoma two years after conjunctival involvement. Percentages and subset ratios of T lymphocytes were quantitated and showed similar results in reactive and neoplastic lesions. There is no apparent difference in clinical presentation or follow-up information between patients with reactive lesions and those having an atypical lymphocytic infiltrate with monotypic immunoglobulin.

Adult↗
PubMed2006

Clinical implications of hepatitis C virus infection in MALT-type lymphoma of the ocular adnexa.

BACKGROUND: A pathogenic link between hepatitis C virus (HCV) and MALT-type lymphomas has been suggested. However, studies assessing the role of HCV infection separately in different forms of MALT lymphomas are not available. PATIENTS AND METHODS: The prevalence and clinical implications of HCV seropositivity were analyzed in 55 patients with ocular adnexa lymphoma (OAL) of MALT-type. RESULTS: HCV seropositivity was detected in seven (13%) patients. At presentation, HCV infection was significantly associated with concomitant extra-orbital disease, lymph node dissemination and involvement of additional extranodal organs. HCV seropositivity was associated also with a higher relapse rate and worse progression-free survival. In fact, 16 patients experienced relapse after first-line treatment: five (71%) were HCV-seropositive and 11 (23%) were HCV-seronegative, with a median TTP of 31 and 50+ months (P = 0.01), and a 5-year progression-free survival of 43 +/- 18% and 77 +/- 7% (P = 0.005), respectively. HCV-seropositive patients experienced frequent relapses despite further lines of therapy; relapses were systemic in all cases but one; multiple subcutaneous nodules were common at relapse. CONCLUSIONS: HCV seropositivity is present in 13% of OAL of MALT-type. Concomitant HCV infection is associated with more disseminated disease and aggressive behavior in OAL, with a consequent potential negative impact in patients managed with radiotherapy alone.

Adult↗
PubMed2004

TP53 mutations in squamous-cell carcinomas of the conjunctiva: evidence for UV-induced mutagenesis.

Squamous cell carcinoma of the conjunctiva is associated with sun exposure and often occurs in HIV-positive individuals. We have analysed TP53 mutations in 21 cases of squamous cell carcinoma and 22 controls with benign conjunctival lesions from a region (Uganda, Africa) with a high prevalence of heavy sun exposure and HIV infection. TP53 mutations were detected in 11 cases (52%) and 3 controls (14%). Seven of the mutations (6 in cases and 1 in controls) were CC-->TT transitions, a molecular signature of mutagenesis by solar UV rays. A similar prevalence (56%) of TP53 mutations was found in 18 squamous cell carcinoma cases positive for epidermodysplasia verruciformis human papillomavirus types. The prevalence of CC-->TT transitions reported here is the highest observed in any cancer type and matches that of skin cancers in subjects with xeroderma pigmentosum, an inherited disease with hypersensitivity to UV damage. These results confirm at the molecular level the causal role of solar UV rays in the aetiology of squamous cell carcinoma of the conjunctiva and suggest that infection with epidermodysplasia verruciformis types of human papillomavirus may act as a cofactor to increase the sensitivity of conjunctiva cells to UV-induced mutagenesis.

Adult↗
PubMed1995

Pathology of ocular melanomas.

Primary ocular melanomas usually arise in the uvea, in the choroid and ciliary body. They metastasize primarily and initially exclusively, to the liver. Metastasis and survival is determined by the maximum tumour dimension, the number of epithelioid cells present within the tumour, vascular patterns within the tumour and nucleolar size and activity. Ganglioside and integrin profiles differ from cutaneous melanomas. Iris melanocytic lesions tend not to metastasize, most being naevi of varying degrees of aggressiveness which may cause glaucoma and corneal decompensation. Conjunctival melanoma is a rare unilateral tumour arising either in primary acquired melanosis or de novo rather than within a naevus. Survival of the patient depends on the location of the tumour and the histological subtype. Tumours not arising in the bulbar or limbal conjunctiva have a much poorer prognosis as do eyelid (cutaneous) melanomas if they involve the lid margin.

Conjunctival Neoplasms↗
PubMed1995

Management of ocular melanoma.

Most melanomas in the orbital region are primary tumours. The uveal tract is the commonest site though the skin of the eyelids, the conjunctiva and, very occasionally, the orbit itself may be involved. Direct spread may cause the tumour to present in an adjacent structure so that a uveal tract melanoma with an extrascleral nodule may appear to arise in the conjunctiva or in the orbit whilst a conjunctival melanoma may first be apparent in the adjacent eyelid skin. Metastatic deposits from cutaneous melanoma may occasionally present in the eye or ocular adnexa.

Conjunctival Neoplasms↗
PubMed2002

Long-term follow-up of patients with conjunctival melanoma.

Data from long-term follow-up examinations of patients with conjunctival melanoma are limited. A retrospective study of survival rates and local tumor relapse rates was performed on 85 patients initially treated between 1958 and 1993. Therapeutic procedures were local excision, local excision followed by brachytherapy, local excision combined with cryotherapy, and local excision followed by either irradiation or cryotherapy and adjuvant mitomycin C (MMC) application. The Kaplan-Meier method was used to estimate cumulative survival rates and event rate curves. Clinical parameters of the patients and the tumors were obtained and analyzed for their relation to tumor recurrence and death from metastatic melanoma using the multivariate Cox hazards modeling. The median follow-up duration among the surviving patients was 13.1 years (mean 13.8 years). The cumulative 10-year survival rate of the 85 patients based on all causes of death was 62.5%, and that based on tumor-related death was 77.7%. Patient age greater than 55 years, higher TNM category, and unfavorable tumor location (palpebral conjunctiva, fornix, caruncle, corneal stroma, eyelid) were identified as prognostic factors for death from metastatic melanoma. Tumors with unfavorable location, higher TNM grade, and excision alone as initial therapy showed a higher cumulative probability of local relapse than favorably located (bulbar and limbal conjunctiva) tumors, lower TNM grade, and excision plus adjuvant therapy. The behavior of conjunctival melanomas remains unpredictable in individual cases. To minimize local recurrence rate surgical excision should be combined with an adjunctive procedure such as irradiation, cryotherapy, or local chemotherapy with MMC. Randomized prospective multicentric studies are required.

Combined Modality Therapy↗
PubMed1998

CD5+ low-grade marginal zone B-cell lymphomas with localized presentation.

Marginal zone B-cell lymphomas (MZBCLs) are low-grade lymphomas that characteristically lack CD5 expression. However, rare cases of MZBCL have been described in which the lymphomatous B cells coexpress CD5 (CD5+ MZBCL). In 7 of 9 reported CD5+ MZBCLs, there was evidence of widespread disease. We report four additional cases of CD5+ MZBCL. Three cases were low-grade B-cell lymphomas of mucosa-associated lymphoid tissue (MALT) involving the lungs, the conjunctiva (bilateral), and the uterus. The remaining case represented a monocytoid B-cell lymphoma involving a posterior cervical lymph node. Southern blot hybridization did not show rearrangements of bc11 or bc12 in the three cases analyzed. All four patients had localized disease and normal peripheral blood counts. Staging of bone marrow biopsies from three patients did not show evidence of bone marrow involvement. The remaining patient had bilateral conjunctival lesions that were present for 15 years without progression. These four additional cases of CD5+ MZBCL show that this group of low-grade B-cell lymphomas occasionally may exhibit an atypical phenotype. Furthermore, in this study, the CD5+ MZBCLs were clinically localized at presentation, in contrast to most other reported cases, which have had dissemination to bone marrow or peripheral blood.

Aged↗
PubMed1990

Dermolipoma surgery, or, "an ounce of prevention is worth a pound of cure".

The surgery of dermolipoma is fraught with dangers. It too often is followed by damage to the lacrimal secretory system, intractable diplopia, blepharoptosis, and perhaps other undesirable complications. Since these tumors are benign, the smallest amount of surgery that debulks the tumor and leads to a cosmetically acceptable result is the best surgery for the patient's satisfaction and for the surgeon's peace of mind. By being aware of these factors, the surgeon can avoid most complications. Some methods of preventing and relieving the symptoms of potential complications of dermolipoma surgery are briefly considered.

Blepharoptosis↗
PubMed1992

Oncocytoma of the caruncle.

Oncocytomas of the ocular adnexa are rare, but when they occur, are most commonly located on the caruncle. Oncocytic carcinomas have been reported but the majority of caruncular oncocytomas are benign. The lesions present as small, cystic, tan-to-red lesions. Histologically, the oncocytes are arranged in an adenomatous pattern. The cells have central nucleoli surrounded by eosinophilic cytoplasm. Electron microscopically, the cytoplasm is filled with mitochondria. As the packing of mitochondria increases, the nucleoli become eccentric and pyknotic, possibly suggesting an end-stage process. A typical clinical case is presented.

Adenoma↗
PubMed1998

Squamous cell tumors and ocular prostheses.

Conjunctival squamous cell carcinoma is an infrequent tumor. It has been reported to occur in association with actinic damage and chronic irritation. To the authors' knowledge, however, this tumor has not been reported secondary to poorly fitting ocular prostheses. Two patients were studied in whom conjunctival squamous cell carcinoma had developed. In both instances, the patient had been enucleated and fitted with an ocular prosthesis more than 40 years before tumor development. Histopathologic evaluation of each tumor revealed its squamous cell origin. In one of the patients, the tumor was found to be metastatic to the ipsilateral parotid gland, an uncommon finding. The authors attempted to identify risk factors that may have contributed to the development of these tumors. Aside from the poor fit of the prostheses, neither patient had significant risk factors for the development of conjunctival squamous cell carcinoma. It is concluded that a new, sanguineous conjunctival discharge or focal eyelid swelling after years of prosthetic wear may not be due to mechanical irritation alone. The onset of these symptoms, especially years after the initial fitting of an ocular prosthesis, should prompt a thorough investigation of its cause.

Aged↗
PubMed1998

Surgical management of circumscribed conjunctival melanomas.

Circumscribed conjunctival melanoma usually arises in the bulbar conjunctiva and less often in the forniceal or palpebral conjunctiva. After simple superficial removal, employed by many ophthalmologists, these tumors have an increased tendency toward local recurrence and distant metastasis. A surgical procedure designed to remove the tumors completely and minimize the changes of recurrence would be desirable. The authors employed a surgical approach to conjunctival melanoma excision, which they believe insures more complete tumor removal and decreases the chances of recurrence and metastasis. The surgical management of melanoma in the limbal region of the bulbar conjunctiva consists of localized alcohol epitheliectomy, removal of the mass by a partial lamellar scleroconjunctivectomy, and supplemental double freeze-thaw cryotherapy to the adjacent remaining conjunctiva by a specific technique. For tumors located in the forniceal or palpebral conjunctiva, wide surgical resection with alcohol treatment to the scleral base and cryotherapy to the surrounding conjunctiva is performed. A "no touch" technique is employed and direct manipulation of the tumor is strictly avoided in an effort to prevent tumor cell seeding into a new area. The technique currently employed has evolved from experience with circumscribed conjunctival melanoma excision during a 20-year period. About 80 patients had circumscribed conjunctival melanoma unassociated with appreciable primary acquired melanosis. Although it is not the purpose of this article on surgical technique to provided a detailed statistical analysis of the results, the authors currently believe that this technique should be employed in all cases of circumscribed lesions in which conjunctival melanoma is a diagnostic consideration. Incisional biopsy and frozen sections are generally not advisable. Preliminary observations suggest that this method decreases the chances of local recurrence.

Conjunctival Neoplasms↗
PubMed1998

A caruncular dermoid with contiguous eyelid involvement: embryologic implications.

An 11-year-old girl with a mass arising from the caruncle and the upper eyelid was examined for spontaneous epibulbar bleeding. Excision of the lesion involved reconstruction of the proximal nasolacrimal system, lid margin, and conjunctiva. The histopathologic diagnosis was consistent with a dermoid of the caruncle. This case is notable not only for a dermoid accompanied by epibulbar bleeding but for its caruncular origin and attachment to the upper eyelid. This case suggests that the caruncle and the upper eyelid arise from similar embryologic structures.

Child↗