Primary lymphoma of the lacrimal sac: an EORTC ophthalmic oncology task force study.

AIM: To define the clinical and histopathological characteristics of primary lacrimal sac lymphoma in a predominantly white population. METHODS: Specimens of lacrimal sac lymphoma and follow up data were solicited from members of the Ophthalmic Oncology Task Force of the European Organization for Research and Treatment of Cancer (EORTC) and the European Ophthalmic Pathology Society (EOPS). Specimens were stained with haematoxylin and eosin and an immunohistochemical panel against leucocyte antigens was applied. Diagnosis was reached by consensus of five experienced pathologists according to the World Health Organization classification system. The histopathological findings were correlated with the clinical data. RESULTS: Of 15 primary lacrimal sac lymphomas, five (33%) were diffuse large B cell lymphoma (DLBCL), five (33%) were extranodal marginal zone B cell lymphoma of mucosa associated lymphoid tissue (MALT lymphoma), three were classified as "transitional MALT lymphoma," being in transition from MALT lymphoma to DLBCL, and two were unclassified B cell lymphomas. Nine of the patients were female, and the median age at the time of diagnosis was 71 years (range 45-95 years). The most frequent presenting symptoms were epiphora (85%), swelling in the region of the lacrimal sac (79%), and dacryocystitis (21%). All but one patient presented in stage I. Systemic spread occurred in three of nine patients (33%). The 5 year overall survival was 65%. CONCLUSIONS: DLBCL and MALT lymphoma are equally common in the lacrimal sac in contrast with the remaining periorbital and/or orbital region where MALT lymphoma predominates.

[Apropos of 5 cases of optic nerve tumors diagnosed during a 6-year-period]. / À propos de 5 cas de tumeurs du nerf optique sur une période de 6ans.

INTRODUCTION: Optic nerve tumors are uncommon and rarely described. By way of five patients treated at the University Hospital of Clermont-Ferrand, France, over a six-year-period, we discuss their clinical and radiological characteristics, their treatment and their course. OBSERVATIONS: We report two cases of optic nerve glioma in small children, two cases of optic nerve sheath meningioma and a 57-year-old patient treated for lung cancer with an optic nerve metastasis and choroidal and brain metastases. The diagnosis was confirmed by imaging in all patients and histologically for the cases of optic nerve glioma. RESULTS: The children with glioma were exclusively treated with chemotherapy, the two women with meningioma exclusively with radiation therapy, and the man with the optic nerve metastasis by chemotherapy and radiotherapy. The tumors were stabilized in all cases except for a progression in one case of glioma, one year after treatment. DISCUSSION: Gliomas and meningiomas are the most frequent tumors; other tumors are rare. Through a literature review and many illustrations, we discuss epidemiologic, clinical and radiologic characteristics, treatment and course of these uncommon tumors. CONCLUSION: The diagnosis of optic nerve tumors must be considered in cases of anterior or posterior progressive optic neuropathy. Treatment must be a compromise between effective treatment of the tumor and preservation of visual function. Decisions are made through multidisciplinary consultations, in which the role of the ophthalmologist is crucial for the diagnosis and success of the treatment.

[Ocular involvement with Candida albicans: report of 2 cases]. / Atteinte oculaire à Candida albicans : à propos de 2 cas.

INTRODUCTION: Ocular involvement by Candida albicans is rare and may present as endogenous endophthalmitis or choroiditis. It occurs in the context of C. albicans septicemia, in the context of intensive care unit hospitalization or intravenous drug use. We report two cases referred to our department with different characteristics, background, diagnostic modalities and different courses. OBSERVATIONS: A 37-year-old woman, with a history of intravenous drug use, presented with C. albicans endophthalmitis. Intravenous combination antifungal therapy was begun, but vitrectomy and intravitreal amphotericin B were performed due to worsening of the endophthalmitis. The second case was a 53-year-old man who was hospitalized in the intensive care unit for C. albicans septicemia with a left macular chorioretinitis. Intravenous antifungal therapy was initiated and allowed regression of the ocular lesion. DISCUSSION: Our cases illustrate both types of ophthalmic involvement by candidiasis requiring different treatments with well-described recommendations: in the case of endophthalmitis, the use of vitrectomy and intravitreal amphotericin B injection in association with intravenous antifungal treatment, whereas parenteral antifungal treatment is often sufficient in the case of chorioretinitis. CONCLUSION: Early detection, initiation of treatment and ophthalmologic monitoring are difficult but necessary in these populations non-compliant with follow-up or in intensive care units. The management of ocular candidiasis requires good collaboration between the ophthalmology, infectious diseases and intensive care unit departments.

[Intraocular lymphoma associated with primary malignant lymphoma of the central nervous system: Seven-year experience of a tertiary center]. / Lymphome intraoculaire associé au lymphome primitif non hodgkinien du système nerveux central : expérience d'un centre tertiaire sur une durée de sept ans.

INTRODUCTION: Primary intraocular lymphoma (PIOL), associated with primary central nervous system lymphoma (PCNSL), is a rare malignancy disease. By way of a seven-year experience of a tertiary center, we discuss the presentation and we review the diagnostic and therapeutic modalities. OBSERVATIONS: We report six cases of PIOL associated with PCNSL. For all patients, the clinical presentation was a vitreoretinal syndrome. The diagnosis was histologically confirmed by vitreal sample or brain biopsy. Five patients developed a diffuse large B-cell lymphoma. Only one patient developed a T-cell lymphoma. The treatment consisted of conformational radiation therapy, systemic chemotherapy and intravitreal injections of methotrexate. The median survival after the diagnosis was 24 months. DISCUSSION: PIOL, associated with PCNSL, is the most common type of ocular lymphoma. In most cases, ocular manifestations inaugurate the disease. PIOL is often fatal because of ultimate central nervous system presentation. The role of the ophthalmologist consists in early diagnosis. Typical clinical findings include vitroretinal tumor syndrome but can mascarade other eye pathologies. Diagnosis requires histology. The majority of PIOL is diffused large B-cell lymphoma. Decisions are made through multidisciplinary consultation. PIOL exhibits high responsiveness to methotrexate. CONCLUSION: Through a literature review and many illustrations, we discuss epidemiological, clinical, histological, radiological and treatment characteristics of PIOL associated with PCNSL.

[DSAEK after failed penetrating keratoplasty]. / DSAEK après échec de kératoplastie perforante.

PURPOSE: To report the anatomic and functional outcomes of Descemet's stripping automated endothelial keratoplasty (DSAEK) in 32 eyes after failed penetrating keratoplasty (PK). SUBJECTS AND METHODS: This retrospective, single-center observational study was performed on 32 eyes of 26 patients with failed PK grafts who underwent DSAEK between June 2009 and June 2013 at Clermont-Ferrand University Medical Center. Primary outcomes measured were: graft survival rates, functional improvement (visual acuity), and complications. RESULTS: Mean follow-up was 16.45 months [6-36]. Graft survival at 12 months was 76.1 % [68.1-82.3]. PK grafts cleared in 75 % of cases. Eight primary DSAEKs detached and/or decentered early (prior to day 8, average 6.22 days): 3 underwent repeat DSAEK with a new graft, and 3 underwent conversion to PK. Results were available for 26 patients at 6 months. Mean preoperative best-corrected visual acuity (BCVA) in logMAR increased from 1.48 (counting fingers at 2 meters) to 0.996 logMAR (1/10) at 6 months. BCVA improved in 17 patients (68 %). BCVA improved an average of 5.2 lines (P=0.0006). Eight patients had a large number of comorbid conditions that limited final visual acuity. DISCUSSION: Anatomic outcomes are similar to other studies. Final average BCVA was limited by severe comorbid conditions and performance of DSAEK solely for comfort. Many surgical strategies have been described to decrease graft dislocation and primary graft failure. Adapting graft trephine diameter to the host cornea as measured by AS-OCT, using a nomogram based on posterior corneal curvature obtained on AS-OCT, may be an interesting approach. CONCLUSION: DSAEK allows for quick visual recovery by preserving the anterior corneal curvature. It is an essential option for restoring corneal clarity in PK failure due to endothelial decompensation. A longer follow-up would allow a comparison of graft survival for the two techniques in this context.

Results of proton therapy of uveal melanomas treated in Nice.

PURPOSE: To present the first results of uveal melanomas treated with the Medicyc Cyclotron 65 MeV proton beam facility in Nice, analyzing the factors that affect the cause-specific survival (CSS), metastatic rate, and reporting the visual outcome. METHODS AND MATERIALS: This study concerns 538 patients referred by French institutions between June 1991 and December 1996. The eye and tumor parameters were measured using ultrasonography and angiography. Since 1994, CT scans were performed in most patients to help determine the axial length and the shape of the ocular globe. Tantalum clips were inserted around the tumor by the referring ophthalmologist. There were 349 posterior pole tumors (64.9%), 130 equatorial tumors (24.1%), and 59 ciliary body tumors (11%). Two hundred four patients (37.9%) had T1 or T2 tumors, and 334 patients (62.1%) had T3 or T4 tumors. The median tumor diameter was 14.6 mm, and the median tumor height was 5.1 mm. All patients received 52 Gy (57.20 Gy Co-equivalent dose) on 4 consecutive days. The data were analyzed by December 1997. RESULTS: The CSS was 77.4% at 78 months, the overall survival was 73.8% and the local control was 89.0%. The CSS was not influenced by the patient age or the site of the tumor. It was 81.5% for T1 and T2 tumors, versus 75% for T3 and T4 tumors (P = 0.035). It was found that the tumor diameter, rather than the height, was the most important parameter affecting outcome. The metastatic rate was 8%. It depended on the T stage, tumor diameter and thickness, but not the tumor site. Thirty-eight enucleations were performed, most of them due to tumor progression and/or glaucoma. One-third of the patients in whom visual acuity was adequately scored before and after treatment had a stable, if not improved vision, and half the patients retained useful vision after treatment. CONCLUSION: The outcome of patients suffering from uveal melanoma and treated with high-energy protons compares favorably with other techniques of treatment. The tumor dimensions affected CSS and metastatic rate. Even though two-thirds of patients had posterior pole tumors, half of them retained useful vision.

Phase II scintigraphic clinical trial of malignant melanoma and metastases with iodine-123-N-(2-diethylaminoethyl 4-iodobenzamide).

Preclinical studies established [125I]-N-(2-diethylaminoethyl) 4-iodobenzamide (BZA) as a potential radiopharmaceutical in the management of patients with malignant melanoma. External detection of both murine and human melanotic melanomas was possible after intravenous injection of 125I-BZA in tumor-bearing mice. This article reports a Phase II clinical trial evaluating 123I-BZA as an imaging agent of primary melanomas and metastases. A total of 110 patients with a history of melanoma were investigated in two nuclear medicine departments. Subjects were imaged from 20 to 24 hr after the intravenous injection of 3.5 mCi (130 MBq) of 123I-BZA. After injection, no short-term or long-term side effects were noted. Calculated on a lesion-site basis, diagnostic sensitivity was 81%, accuracy was 87% and specificity was 100%. The melanoma nature of previously occult lesions was confirmed by clinical criteria and/or additional investigations in follow-up studies. The scintigraphies were normal in 44 patients in clinical remission after treatment of malignant melanoma and in seven patients with nonmelanoma disease. No false positive results were observed. Iodine-123-BZA scintigraphy appears to be a safe and useful agent for the detection and follow-up of patients with malignant melanoma. BZA also allowed the detection of unsuspected lesions and the evaluation of the results of various therapeutic procedures such as surgery, chemotherapy, immunobiology, biological therapy or radiotherapy.

Establishment of IPC 227 cells as human xenografts in rabbits: a model of uveal melanoma.

This study was designed in order to evaluate the feasibility of establishing an animal model of human uveal melanoma. IPC227, a cell line established from the biopsy of a patient with a spindle cell ciliary body melanoma, was transplanted into the anterior chamber of the eye in immunosuppressed New Zealand rabbits. In a second step, a tumour fragment from the anterior chamber was implanted transclerally into the posterior choroid. Complete ophthalmological examinations were then performed on the animals. Characteristic growth patterns were noted depending on the location of implantation. In the anterior chamber, diffuse, flat, heavily pigmented tumours appeared 8 days after the injection of the cell suspension that covered the iris and the angle by day 25, with a success rate of 100%. Nodular, lightly pigmented tumours were obtained 6-7 weeks after subchoroidal implantation, with a 25% success rate. Clinical examination, including fundus photography, ultrasound and magnetic resonance imaging, demonstrated the same characteristics as those of human uveal melanoma, confirming the value of this model for the evaluation of new therapeutic and diagnostic methods in the management of uveal melanoma.

[One case of dacryolithiasis of the lacrimal sac (author's transl)]. / Un cas de calcul du sac lacrymal. Observation anatomo-clinique.

Report is given of one case of dacryolithiasis of a diverticulum of the lacrimal sac. The case was demonstrated by dacryocystography and spontaneous passage of the case through the nose. Histopathological examination revealed this cast to be composed of layers of cellular debris, accompanied by an infiltration of leucocytes. There was a mycotic infection most likely with candida. The diverticulum of the sac demonstrated squamous metaplasia of the epithelium and mild inflammation. The responsibility of keratinized cells and mycotic infection in the pathogenesis of dacryolith is discussed.

[Intraocular fibrosis after perforating injury of the posterior segment. Experimental study]. / Fibrose intra-oculaire après traumatisme perforant du segment postérieur. Etude expérimentale.

Double perforating injuries and linear wounds were produced in the posterior segment of the rabbit eye in order to study post-traumatic intraocular proliferation. After observation for one month, the animals were sacrificed and the eyes treated for histologic examination. Transvitreous strands and preretinal membranes slowly developed, sometimes inducing traction retinal detachment. Histologic studies demonstrated the new-formed tissue to be fibrovascular, mainly containing collagenous fibers and fibroblast-like cells. This fibroblastic proliferation closed the wound through the ocular membranes and invaded the vitreous using vitreous fibers as a scaffold. The authors discuss the signification of this results comparing them to literature data on different animals and on man.

[Unilateral iris metastasis of cutaneous malignant melanoma]. / Métastase irienne unilatérale d'un mélanome malin cutané.

We describe a case of cutaneous malignant melanoma metastatic to the iris and the angle 31 months after excision of the primary tumor in a 37-year-old caucasian female patient. The patient who had numerous metastases (lung, liver, cerebrum, skin) suffered from secondary glaucoma and died 5 months after the discovery of the intraocular metastases. The glaucoma was treated with medications and cyclocryotherapy. There was no response of the iris metastases to cyclical combined chemotherapy.

[Use of contact lenses during argon laser iridectomies (author's transl)]. / Intérêt du verre de contact pour l'iridectomie par photocoagulation au laser à l'argon.

Advantages appear to be gained by the use of a specific contact lens during argon laser iridectomy. On the one hand, the energy level is increased on the iris and diminished on the retina. On the other hand, coagulation is directed near the iris base and far from the posterior pole. Specifications of a new contact lens are presented.

[Treatment of post-traumatic cyclodialysis using by direct cyclopexy]. / Traitement des cyclodialyses post-traumatiques par cyclopexie directe.

INTRODUCTION: We report 2 cases of posttraumatic cyclodialysis treated by direct cyclopexy. We decided to operate because of the presence of a macular and disc oedema after an ocular chronic hypotony. PATIENTS: The case was a blunt ocular trauma and the other case a perforating injury. For the latter, a cycloplegic treatment was ineffective. The ocular hypotony persisted 2 months in the first case and 2 and a half years in the second. In both cases, the hypotony was major (0mmHg) and the cyclodialysis cleft was extended to 90 degrees (in the superior temporal quadrant and the inferior nasal quadrant). A direct cyclopexy was performed with cilioscleral sutures with 10-0 monofilament. RESULTS: One patient presented a moderate and temporary postoperative hypertony. The maximal follow-up was 9 months: ocular tonus was normalized (10 and 11mmHg), the fundus aspect was improved, and visual acuity was either the same (hand movement perception) or increased from 0.1, 14 to 1.0, 2 on the Parinaud scale. DISCUSSION: The direct cyclopexy was effective in both cases. It precisely defined the cyclodialysis limits, it allowed the suprachoroïdal fluid to drain and restored the anatomic features. Moreover, it allowed treating larger cyclodialysis for which a simple laser treatment would be incomplete. On the other hand, it was invasive intraocular surgery which presented risks of hemorrhage, infection and retinal detachment. CONCLUSION: The direct cyclopexy is an advisable method in the treatment of cyclodialysis. It normalized an ocular tonus and improved of hypotonus retinopathy. However, vision remained sometimes limited because of the posttraumatic sequels are or ocular hypotony that was too lengthy.

[The diagnosis of isolated choroidal hemangioma (author's transl)]. / Un diagnostic difficile: l'angiome choroïdien isolé.

The authors report clinical and laboratory data setting the diagnosis of isolated choroidal hemangioma. Main elements appear to be biomicroscopy and angiography after fluorescein and cardiogreen injection. The clinical history of two non typical cases is reported and discussed.

[Prognostic study of malignant melanoma of the uvea treated by enucleation]. / Etude pronostique des mélanomes malins de l'uvée traités par énucléation.

In order to evaluate the clinical and histopathologic prognosis indicators of uveal melanomas treated with enucleation, 59 patients, followed up during 1 to 18 years were included in a retrospective study. Kaplan Meier survival curves were constructed and compared using the log rank test. The overall 5 and 10 years probabilities of survival were 74.9% and 48.5% respectively. Metastases were, or might have been, responsible for death in 83.3% of the cases. The median survival was 4 years 9 months (range less than 1 to 11 years). Fifty five per cent of the metastases occurred within 4 years of the enucleation and 90% within 8 years. For the patients with spindle cell melanomas, the 5 year probability of survival was 90% against 38% for the patients with epithelioid cell tumors. The median time to tumor related death was 5 years for the first group against 3 years 8 months for the second. The analysis of our data demonstrated the tumor volume to be a statistically significant clinical prognosis indicator. The following histopathologic parameters were also statistically significant prognosis indicators: the cell type (p less than or equal to 0.001), the mitotic activity, the state of the sclera, the presence of blood lakes. Statistical adjustments and determination of the instantaneous relative risk of death showed the histopathologic factors, specially the cell type, to be the most important indicators.

[Late metastases of malignant choroidal melanoma after curietherapy: two case reports]. / Métastases tardives après curiethérapie efficace de mélanome malin de la choroïde. A propos de deux cas.

PURPOSE: The technical advancements now provide conservative treatment of choroidal melanoma. The course of the disease remains marked by the metastases that usually set in within 3 years. The aim of this paper is to report two unusual cases of hepatic metastases of choroidal melanoma that set in 11 and 18 Years after curietherapy. METHODS: A 40-year-old woman and a 50-year-old man presented with T2 and T3 choroidal melanomas and were treated by cobalt plaque. The tumors decreased within the year and the patients were monitored every 3 months for 5 years. RESULTS: Metastases set in, respectively, 11 and 18 years after treatment. DISCUSSION AND CONCLUSION: These two observations show that the metastases had already spread at the moment of diagnosis and that the cell doubling time of the melanoma was very long. These findings indicate the difficulties in monitoring patients with choroidal melanoma.

[Multiple decubitus ophthalmological operating table]. / Table operatoire d'ophtalmologie à decubitus multiples.

A new ophthalmological operating table is presented which is adapted to the most recent developments in microsurgery and for the treatment of the most difficult cases of vitreoretinal pathology. The head of the table is mobile in the sagittal plane, movements being effected under remote-control by the surgeon at a rate of 2 mm/sec. The same movement can be applied to a horseshoe shaped armrest, which is solidly attached around the headpiece and is adjustable for height. A respiratory shield can be attached during local anesthesia, and the table can be positioned in proclivity or declivity. During vitreoretinal surgery, the table enables rotation of the patient in ventral decubitus along a longitudinal axis, rotation being possible manually or electrically. Proclivity and declivity positions can be obtained by rotation on a transversal axis. These functions assist exchanges between ocular fluids and substances used for internal packing, as well as passage into the anterior chamber of foreign bodies in the vitreal cavity.

[External beam radiation in treatment of subfoveal vascularized pigment epithelium detachment in age-related macular degeneration. Long-term results of a pilot study]. / Radiothérapie externe dans le traitement des décollements vascularisés rétrofovéolaires de l'épithélium pigmentaire de la DMLA. Résultats à long terme d'une étude pilote.

PURPOSE: The purpose of this study was to contribute to the evaluation of long-term external beam radiation treatment in patients with subfoveal occult choroidal neovessels complicated with pigment epithelium detachment in age-related macular degeneration. MATERIALS AND METHODS: This was a retrospective study of ten patients with a mean age of 75 years and a mean follow-up of 18.7 months. External beam radiation of 14.4 Gy was administered with a daily dose of 1.8 Gy. The efficacy of the treatment was assessed based on visual acuity, the size of the membrane and the persistence or not of neovascular activity. RESULTS: We observed stabilization of visual acuity in 44% of the cases at 6 months but only in 20% at 19 and 30 months. The mean initial visual acuity was measured at 0.2 at presentation and 0.1 at the final evaluation. At the end of the study, 60% of patients attained the level of legal blindness; 90% of patients developed a disciform scar, with persistence of neovascular activity in 27% of the cases. On fluorescein angiography, the size of pigment epithelium detachment increased more than one optic disc diameter in 20% of the cases at 1 year. On the other hand, no radiation complication was observed. DISCUSSION: The visual and anatomical results of our study are similar to the natural progression of occult neovascular membrane. External beam radiation at a dose of 14.4 Gy did not seem to provide a long-term benefit in the treatment of subfoveal occult neovessels associated with pigment epithelium detachment.

[Giant peripheral tear of the retinal pigmented epithelium]. / Déchirure géante et périphérique de l'épithélium pigmentaire rétinien.

The authors present a case of gigantic tear of the retinal pigmented epithelium. This tear was situated in the middle periphery and seems to have appeared spontaneously together with a carotid-cavernous fistula. The functional outcome was favorable. The authors deal with the various possible patho-physiological mechanisms.

[A case of intraocular recurrence at a distant site of malignant melanoma of the choroid treated by cobalt 60 disk]. / Un cas de récidive intraoculaire située à distance d'un mélanome malin de la choroïde traité par disque de cobalt 60.

Twenty months after cobalt 60 plaque therapy of a choroidal malignant melanoma, a patient developed an intraocular recurrence at a site distant from and non contiguous to the original lesion that seemed controlled. The characteristics and the possible explanations of this unusual recurrence are discussed.