Paul Moriarty

Ocular adnexal lymphoma—comparison of MALT lymphoma with other histological types

AIMS To correlate histological features of ocular adnexal lymphoma using the revised European American lymphoma classification (REAL), with stage of disease at presentation, treatment modalities, and patient outcome. MALT lymphoma defines an extranodal marginal zone B cell lymphoma as outlined in the REAL classification. Comparison groups of patients included those with primary ocular adnexal MALT lymphoma versus primary ocular adnexal lymphomas of other types, MALT lymphoma versus non-MALT lymphomas (primary and secondary), and primary ocular adnexal lymphoma (MALT lymphomas and other types) versus secondary ocular adnexal lymphomas. METHODS A retrospective review of the National Ophthalmic Pathology Laboratory records identified 20 cases of ocular adnexal lymphoma over a 10 year period which were reclassified using appropriate immunohistochemical stains. Patients’ medical records were examined for data including stage of the disease at presentation, mode of treatment, and patient outcome. RESULTS Among the 20 cases identified 14 had primary ocular adnexal lymphomas. 10 of the primary lymphomas had histological features of MALT lymphoma. One case was a primary ocular adnexal T cell lymphoma, one a follicular centre, follicular B cell lymphoma, and two were large cell B cell lymphomas. Six cases had systemic disease, four large B cell, one follicular centre, follicular B cell, and one mantle cell. A significantly higher proportion of patients with MALT lymphomas had early disease (p = 0.005), initially required local treatment (p = 0.005) and were alive at last follow up (p = 0.001) than those without. Two patients with MALT lymphoma had recurrence of lymphoma which responded to further treatment. CONCLUSIONS Patients with primary ocular adnexal MALT lymphomas present with localised disease requiring local treatment and have a better outcome compared with patients with other types. As a small percentage of these tumours recur, patients should be followed up indefinitely.

Long term follow up of nasolacrimal intubation in adults

Background/aims: The authors have previously reported a short term mean 15 month follow up of nasolacrimal intubation in adults. The effectiveness of this procedure for long term (mean 78 months) control of epiphoria is assessed here. Methods: 65 eyes from 40 patients who underwent nasolacrimal intubation were followed. Mean age at intubation was 59.2 years. The mean follow up period was 6.2 years. The results were based on long term symptomatic improvement. Results: Complete long term resolution of symptoms was reported in 50.7%. A partial improvement was reported in 38.5%, and no improvement in 10.7%. A better outcome was associated with a canalicular than nasolacrimal duct obstruction. On long term follow up 16.9% required dacrocysto-rhinostomy (DCR). Conclusion: Nasolacrimal intubation, a minimally invasive procedure is successful in the long term control of epiphora. Selection of patients with canalicular duct obstruction gives higher success rates with fewer patients subsequently requiring the DCR procedure.

Differential Expression of Fourteen Proteins between Uveal Melanoma from Patients Who Subsequently Developed Distant Metastases versus Those Who Did Not

PURPOSE To compare the proteomic profiles of two categories of primary uveal melanoma tissue samples; those from patients who have subsequently developed metastatic disease and those who have not. METHODS Two-dimensional difference gel electrophoresis (2D DIGE) was performed on 25 uveal melanoma tissue specimens (minimum follow-up of 7 years) comparing nine uveal melanoma tumors from patients who developed metastatic disease and 16 from those who did not. Most of the tumors which metastasized also exhibited chromosome 3 monosomy. Selected differentially expressed proteins were further followed up by immunohistochemistry and functional validation in vitro using siRNA. RESULTS Proteomic analysis revealed 14 statistically significant differentially expressed proteins, with nine showing increased expression (PDIA3, VIM/HEXA, SELENBP1, ENO1, CAPZA1, ERP29, TPI1, PARK7, and FABP3) and five showing decreased expression (EIF2S, PSMA3, RPSA, TUBB, and TUBA1B) in uveal melanomas that subsequently metastasized compared with those that did not. Immunohistochemical analysis was performed for six of the differentially expressed proteins and gave similar results to the 2D DIGE study for two of these proteins, fatty acid-binding protein, heart-type (FABP3) and triosephosphate isomerase (TPI1). siRNA knockdown in the 92.1 uveal melanoma cell line confirmed a functional role for FABP3 and TPI1 in invasion in vitro. CONCLUSIONS Proteomic analysis identified proteins differentially expressed in uveal melanoma that will subsequently metastasize, some of which appear to have a functional role in invasion. These results may contribute to better predictive tests (along with genetic analysis) and to the identification of new therapeutic targets.

Rosai Dorfman disease or sinus histiocytosis with massive lymphadenopathy of the orbit.

Sinus histiocytosis with massive lymphadenopathy (SHML) or Rosai Dorfman syndrome is a rare benign proliferative histiocytic disease of unknown origin. It predominantly affects the lymph nodes. The head and neck region usually in association with lymph node involvement, represents one of the most common extranodal areas affected by SHML. The other common extra nodal site is skin. Rarely, there is widespread dissemination with liver, kidney, respiratory organs, orbit, and eyeball involvement.1 The mean age of onset is 20 years (birth to 74 years). A 57 year old woman with a 6 month history of double vision was referred to the Royal Victoria Eye and Ear Hospital, Dublin. She was found to have proptosis, ptosis, diplopia due to inferior rectus dysfunction, and restriction of elevation of the left eye. Her visual acuity was normal. Relevant investigations showed a high erythrocyte sedimentation rate (ESR) of 44 mm in the first hour, C reactive protein of 1.9 (normal less …

Proteomics in uveal melanoma.

Uveal melanoma is the most common primary intraocular malignancy in adults, with an incidence of 5-7 per million per year. It is associated with the development of metastasis in about 50% of cases, and 40% of patients with uveal melanoma die of metastatic disease despite successful treatment of the primary tumour. The survival rates at 5, 10 and 15 years are 65%, 50% and 45% respectively. Unlike progress made in many other areas of cancer, uveal melanoma is still poorly understood and survival rates have remained similar over the past 25 years. Recently, advances made in molecular genetics have improved our understanding of this disease and stratification of patients into low risk and high risk for developing metastasis. However, only a limited number of studies have been performed using proteomic methods. This review will give an overview of various proteomic technologies currently employed in life sciences research, and discuss proteomic studies of uveal melanoma.

Beware the empty curette

BACKGROUND AND OBJECTIVE Chalazions are a common occurrence in the eyelids due to chronic inflammation in the tarsal plate. Treatment of non-resolving cases may involve incision and curettage. Chalazions that are recurrent should arouse suspicion. We present a case of a pleomorphic adenoma of the palpebral portion of the lacrimal gland, which was misdiagnosed as a chalazion. PATIENT AND METHODS A 41-year-old Caucasian lady initially presented with a swelling in the outer part of her right upper eyelid. A diagnosis of chalazion was made and two attempts at incision and curettage failed to resolve the lesion. She was then referred to the oculoplastics/adnexal unit. A more detailed examination revealed involvement of the palpebral part of her lacrimal gland. A CT-scan confirmed this and she proceeded to have a lateral orbitotomy to remove the tumour. RESULT Histological confirmation of a pleomorphic adenoma of the palpebral portion of the lacrimal gland was obtained. The lacrimal gland had been completely excised. CONCLUSION Persistent or recurrent meibomian cyst should be treated with suspicion. A careful orbital examination including lid eversion should always be performed to outrule a more sinister pathology.

Primary mucinous meningioma of bone.

Introduction Meningiomas represent the most frequent intracranial neoplasms in adults. The vast majority of cases are characterised by distinctive morphologic features, which permits reliable diagnosis and classification by conventional histological techniques. In the present paper, we report an unusual variant of a primary intraosseous meningioma in which clusters of cells were surrounded by extensive mucinous stroma. The unusual appearance of the lesion initially suggested a metastatic mucin-secreting carcinoma, resulting in an extensive search for a primary lesion. The diagnosis of primary mucinous intraosseous meningioma was confirmed with immunohistochemical studies.

Bilateral orbital Kimura's disease

The authors present the case of a 62-year-old man who was shown to have bilateral Kimuras disease of the orbit. The patient complained of bilateral lid swelling for two years prior to his presentation. Orbital biopsies demonstrated angiolymphoid hyperplasia with eosinophilia-Kimuras disease. He subsequently was started on oral steroids but the disease process was recalcitrant, and required orbital radiation. This latter modality proved more effective in controlling his symptoms, and the amount of proptosis. Debulking surgery, however, was also required. This is the second reported case of bilateral orbital Kimuras discase in a Caucasian.