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Solitary extramedullary plasmacytoma of the palpebral conjunctiva.

A 63-year-old man had a solitary extramedullary plasmacytoma of the conjunctiva for several years with no history of ocular inflammation or systemic abnormalities. Immunoperoxidase staining was consistent with a neoplastic lesion. This is the only well documented such case in the recent literature to the best of our knowledge. This rare condition is contrasted with reactive plasma cell granuloma, a benign inflammatory condition, which comprises most of the cases previously reported as plasmacytomas. Since extraskeletal plasmacytomas may be the first manifestation of multiple myeloma, these patients deserve appropriate diagnostic evaluation and close follow-up.

Conjunctival Neoplasms↗

Malignant epibulbar melanoma: natural history and treatment by radiotherapy.

One hundred and eighty-four cases of malignant melanoma of the lids and epibulbar region were seen in the Radiotherapy Department of the Royal Marsden Hospital during the period 1943-74. Some of these tumours were radiosensitive and could thus be treated by radiotherapy without loss of the affected eye. Their clinical and histological features are described. The treatment policy adopted was based on radiotherapy first, and surgery reserved for the failures. A policy of observation is advised for precancerous melanosis, and active treatment should be undertaken only if malignancy supervenes. The contraindications for radiotherapy, the factors governing radiosensitivity, and the incidence and site of metastases are reported, and the possible effect on the metastatic and survival rates of leaving the eye are discussed. Active treatment of the node-free neck is not advised. Results are given according to site, histology, and treatment method, and the frequency with which the eye was lost is shown, since the sole advantage of radiotherapy over surgery is the possibility of saving the eye without worsening the prognosis for survival.

Adult↗

Conjunctival myxoma: a case report.

A rare case of conjunctival myxoma in an 18-year-old female is reported. Clinically it presented as a painless mass located in the nasal bulbar conjunctiva. It was composed of spindle and stellate shaped cells in a loose mucoid stroma. Some of the cells had intracytoplasmic vacuoles consistent with dilated rough endoplasmic reticulum and/or intranuclear vacuoles of nuclear membrane invaginations. Mast cells were also seen in the stroma. No recurrence has been reported eight months postoperatively.

Adolescent↗

Ocular involvement in sinus histiocytosis with massive lymphadenopathy.

Sinus histiocytosis with massive lymphadenopathy is a distinct clinical entity, with ophthalmic involvement in 10% of patients. Orbital as well as eyelid lesions have been described as part of the extra-lymph node involvement of this disorder. We recently examined a young boy with sinus histiocytosis with a bulbar conjunctival mass and chronic dacryocystitis.

Adolescent↗

Malignant lymphoma of the conjunctiva following Hodgkin's disease.

A 64-year-old woman with mixed cellularity Hodgkin's disease diagnosed in 1972 developed a malignant lymphoma of the conjunctiva 14 years later. She had undergone combined chemotherapy and radiation therapy for Hodgkin's disease. Non-Hodgkin's lymphomas developing after Hodgkin's disease have been reported with increasing frequency in recent years. It is important to recognise a separate malignant lymphoma in a patient with Hodgkin's disease because of the different treatment offered for each of these diseases.

Conjunctiva↗

Therapeutic outcome of patients suffering from malignant melanomas of the conjunctiva.

Eighty-one cases of conjunctival melanoma treated between 1960 and 1988 were studied to determine factors that might affect outcome in patients with such lesions. The therapeutic procedures performed were local excision (16), local excision followed by brachytherapy with Sr-90/Y-90 (32), local excision followed by cryotherapy with liquid nitrogen (16), brachytherapy with Sr-90/Y-90 (12), local excision followed by external beam irradiation (3), and local excision followed by brachytherapy and cryotherapy (2). The median follow-up period was 5.5 years (longest 26, shortest 1 year). Sixty two patients (76.5%) showed a complete regression of the melanoma, 19 (23.5%) developed recurrences, and 15 (18.5%) died from metastases. The melanomas had developed with almost equal frequency from a pre-existing naevus (25.9%), from primary acquired melanosis (25.9%), and 'de novo' (30.9%). Small tumours had a higher chance of regressing (80.6%) than larger ones (68.6%). The cumulative survival rate was 76% after five years and 60% after 10 years from any causes of death and 87.6% after five years and 76.3% after 10 years from deaths caused by metastases. Most deaths from metastases occurred within 5 years. At 88.5%, the cumulative survival rate of patients with small tumours (less than one quadrant of the bulbar conjunctiva and less than 2 mm thickness) was significantly higher than that of patients with larger tumours (more than one quadrant of the bulbar conjunctiva and/or more than 2 mm thickness) with 65% after eight years. Local excision followed by beta ray irradiation (Sr-90/Y-90) or cryotherapy can be recommended as the treatment of choice. Nevertheless the behaviour of conjunctival melanomas remains unpredictable in individual cases.

Adult↗

Malignant fibrous histiocytoma of the conjunctiva.

Malignant fibrous histiocytoma (MFH) of the conjunctiva is an extremely rare tumour, and only three previous cases have been reported. We describe two patients with MFH of the conjunctiva: a 58-year-old white male with epibulbar tumour who had exenteration and is alive after five years' follow-up, and a 3 1/2-year-old African girl with xeroderma pigmentosum and an MFH of her right eye conjunctiva, the first reported case of this association. The characteristics and the methods of diagnosis of MFH are discussed.

Child, Preschool↗

Premalignant melanosis of the conjunctiva and the cornea in xeroderma pigmentosum.

Xeroderma pigmentosum is a rare autosomal recessive dermatosis. The neoplastic changes in sunlight-exposed areas of the skin and eyes may be related to the impaired replication of ultraviolet radiation-damaged DNA. A 38-year-old Greek woman is reported with a mild form of xeroderma pigmentosum and primary acquired melanosis with atypia of her right limbal conjunctiva and cornea. The development of this precursor of conjunctival malignant melanoma in a xeroderma pigmentosum patient may support the putative role of sunlight exposure in malignant transformation of conjunctival melanocytes.

Adult↗

Resection of intraocular squamous cell carcinoma.

A patient with recurrent squamous cell carcinoma of the conjunctiva was referred with 20/20 vision in an eye with obvious intraocular extension. A modified iridocyclochoroidectomy was performed and the tumour was removed. Three and a half years later the patient's vision is 20/30 and there is no recurrence. This is the first case in which an eye has been successfully salvaged with documented intraocular squamous cell carcinoma of the conjunctiva.

Carcinoma, Squamous Cell↗

Multifocal amelanotic conjunctival melanoma and acquired melanosis sine pigmento.

Clinical and histopathological features of four cases of multifocal amelanotic malignant melanoma of the conjunctiva in association with 'acquired melanosis sine pigmento' are reported. The absence of conjunctival pigmentation in this extremely rare combination of lesions prevented early diagnosis and clinical monitoring. As a result orbital exenteration was required in three cases. This multicentric non-pigmented variety of conjunctival malignant melanoma tends to present later than pigmented forms and may require exenteration of the orbit as a primary procedure.

Adult↗

Impression cytology of conjunctival melanosis and melanoma.

Impression cytology using cellulose acetate paper has been used in various ocular surface disorders as a simple non-invasive diagnostic test. To assess its value in differentiating melanocytic tumours, 24 patients with a range of pigmented lesions of the conjunctiva were examined using this technique. Cytological and histological diagnoses were compared in 23 cases. In 73% of cases impression cytology predicted the histological diagnosis by detection of superficial atypical melanocytes and their proportion relative to benign epithelial cells. This pilot study shows impression cytology to be a useful diagnostic aid in the differentiation of pigmented tumours of the bulbar conjunctiva.

Adult↗

Pigmented epithelial tumours of the conjunctiva.

Two out of 60 conjunctival epithelial tumours reviewed between 1973 and 1989 were found to be pigmented. One tumour was a pigmented papilloma and the other a pigmented squamous cell carcinoma. The melanin pigment was found in epithelial tumour cells as well as in macrophages, dendritic melanocytes, and Langerhans cells. The distinction between the latter two types of cells was possible in one of the tumours only. Both tumours were found in dark-skinned white patients without any evidence of conjunctival acquired melanosis.

Adult↗

Nasal and orbital recurrence of conjunctival melanoma 21 years after exenteration.

In our experience 5% of invasive malignant melanomas of the conjunctiva arising from areas of primary acquired melanosis with atypia spread to the ipsilateral nasal cavity and paranasal sinuses. Twenty one years after orbital exenteration for multicentric conjunctival melanoma an 82-year-old man was seen with an orbital recurrence, which had extended to the nasal cavity and paranasal sinuses through the nasolacrimal duct without invading the mucosa. This previously undescribed way of spread after the longest symptom-free interval following exenteration ever reported is illustrated.

Aged↗

Clinical predictive factors for development of recurrence and metastasis in conjunctival melanoma: a review of 68 cases.

Sixty eight cases of histologically proved conjunctival melanoma were reviewed in order to determine the clinical factors that were predictive of local recurrence and distant metastasis. All patients were treated with surgical excision and most had supplemental cryotherapy. The mean follow up was 7.5 years. Histopathologically, the conjunctival melanoma arose from primary acquired melanosis in 56%, from naevus in 26%, and de novo in 18%. Of the 68 patients, 38 (56%) developed at least one local tumour recurrence and 22 (32%) developed more than one recurrence. The method of initial treatment and the eventual development of metastasis were the two parameters statistically associated with tumour recurrence. Those patients treated initially with tumour excision alone had a statistically significant higher recurrence rate than those treated initially with excision and supplemental cryotherapy (p = 0.001). Fourteen patients (21%) developed metastasis and the mean period between treatment and metastasis was 3.6 years. Twelve (18%) died from metastatic melanoma with a mean interval of 4.4 years from the time of initial surgery until death. The only clinical parameter that was statistically associated with distant metastasis was local tumour recurrence (p = 0.015). Based on these observations, the authors make recommendations regarding the treatment of conjunctival malignant melanoma. It appears that initial complete excision of the tumour with supplemental cryotherapy offers the patient the best chance of remaining free of recurrence and metastasis.

Adult↗

Prognostic factors in primary malignant melanoma of the conjunctiva: a clinicopathological study of 256 cases.

A series of 256 consecutive cases of invasive primary conjunctival malignant melanomas was examined to identify clinical and histopathological prognostic factors. The follow up period varied between 0.3 and 45.9 years (mean 9 years, median 6.3 years). The 5 year survival rate was estimated at 82.9%, the 10 year survival rate at 69.3%. Multiple regression analysis with the Cox proportional hazards model was used to assess sex, age, and a number of baseline features of conjunctival malignant melanoma as possible prognostic factors influencing melanoma related mortality. In assessing each potential prognostic factor, the effects of all other factors were taken into account in the modelling process. Tumours in unfavourable locations--that is, those involving the palpebral conjunctiva, fornices, plica, caruncle, and lid margins, were associated with 2.2 times higher mortality compared with (epi)bulbar melanomas. Patients with mixed cell type tumours had about three times higher mortality compared with those with pure spindle cell melanomas, and histological evidence of lymphatic invasion by tumour cells was also a prognostic feature, carrying a fourfold increase in the death rate. Multifocal tumours were associated with a fivefold increase in mortality among those with tumours in favourable (epi)bulbar locations, but were not prognostic in patients with melanomas in unfavourable sites. The death rate was significantly higher in those with initial tumour thickness of more than 4 mm, but only among patients with unfavourably located melanomas. Sex, age, and clinical origin of the tumour (primary acquired melanosis, pre-existing naevus, or de novo) were not useful prognostic indicators in this study.

Adult↗