Search

Diagnosis of choroidal hematoma, either spontaneous or associated with age-related macular degeneration, is clinical. In some cases of expansive or posterior lesion, hematoma may be misdiagnosed as a tumor. MRI and color Doppler imaging (CDI) are important in ruling out uveal melanoma in these cases.We reviewed the clinical, MRI, and ultrasonographic characteristics of 95 patients sent to the Curie Institute for suspected uveal melanoma between 1998 and 2006, whose final diagnosis was a choroidal hematoma. Imaging differences with melanomas are discussed.A total of 95 patients with a diagnosis of hematoma were seen; the age varied from 54 to 92 years with a median age of 77 years; there was a history of macular degeneration in 27 cases and 11 patients were taking an anticoagulant. Intravitreous hemorrhage was noted in 18 cases and the lesion was located in the posterior pole in 28 cases. The thickness of the lesions measured by B scan ultrasonography varied between 1 and 7.8mm, with a mean thickness of 2.86mm. MRI was performed in 27 cases and CDI in ten cases. On CDI, hematomas appeared as linear or regular bulging lesions with no intralesional blood flow. On MRI, hematomas appeared as a high-intensity signal on T1-weighted images, heterogeneous on T2-weigted images in relation to the progression of the clot, but no contrast enhancement was noted inside the lesion. Follow-up examinations showed the progressive involvement of the clot and delayed decreasing size of the lesion.The diagnosis of choroidal hematoma is usually made by fundus examination. In some posterior locations with pseudo-tumoral appearance, CDI and MRI are useful to rule out a uveal tumor. more...

Purpose To evaluate the treatment of conjunctival melanoma at the Institut Curie in term of local recurrences, ocular preservation, distant metastasis and survival Methods Retrospective study of patients with conjunctival melanoma treated for their first tumoral localisation between January 2000 and December 2005. The clinical records, histologic features, ocular treatments were rewiewed. The rate of local recurrences, ocular preservation, regional and distant metastasis, and survival were recorded. Results During the study period, 91 patients were referred for conjunctival melanoma. 67 of them were treated for a first occurrence of malignant melanoma (38 women and 29 men). The mean age was 62 years (25 – 90). 32/67 patients (47 %) presented a primary acquired melanosis (PAM). Most cases (55 patients) were treated by surgical excision and adjunctive radiotherapy (Iodine plaque, protonbeam or external beam irradiation), 5 cases were treated by surgery alone, and the last 6 by surgery and adjunctive cryotherapy or adjunctive topical chemotherapy. 3 patients were lost of follow-up. With a mean follow-up of 52 months (15-101months) 20/64 patients developped at least one local recurrence (between 1 and 7 recurrences). 16 of them presented a PAM. An enucleation was finally necessary for 1 patient and an exenteration for 2 patients. 7 patients developped metastatic disease (all but one presented a PAM) and 6 of them died. Conclusion Local recurrences, metastatic disease and tumor related death appear to occur at higher frequency when melanoma is associated with PAM. This risk must be taken into account in the therapeutic approach of conjunctival melanoma. more...

Background Uveal melanoma is characterised by a high prevalence of liver metastases and a poor prognosis. Aim To review the evolving surgical management of this challenging condition at a single institution over a 16-year period. Patients and Methods Between January 1991 and June 2007, among 3873 patients with uveal melanoma, 798 patients had liver metastases. We undertook a detailed retrospective review of their clinical records and surgical procedures. The data was evaluated with both uni- and multivariate statistical analysis for predictive survival indicators. Results 255 patients underwent surgical resection. The median interval between ocular tumour diagnosis and liver surgery was 68 months (range 19–81). Liver surgery was either microscopically complete (R0; n = 76), microscopically incomplete (R1; n = 22) or macroscopically incomplete (R2; n = 157). The median overall postoperative survival was 14 months, but increased to 27 months when R0 resection was possible. With multivariate analysis, four variables were found to independently correlate with prolonged survival: an interval from primary tumour diagnosis to liver metastases >24 months, comprehensiveness of surgical resection (R0), number of metastases resected (≤4) and absence of miliary disease. Conclusions Surgical resection, when possible, is able to almost double the survival and appears at present the optimal way of improving the prognosis in metastatic uveal melanoma. Advances in medical treatments will be required to further improve survival. more...

Detail the most frequent problems encountered in the differential diagnosis of retinoblastoma.and method: We conducted a retrospective study on the children referred to the Curie Institute for suspicion of retinoblastoma between 2000 and 2006. Diagnosis was made by fundus examination using the indirect ophthalmoscope, ultrasonography, and MRI.Of the 486 children seen during this period, 408 had unilateral or bilateral retinoblastoma and 78 (16%) had another lesion: Coats disease (20 children, 25%), congenital malformations (23 children, 30%; coloboma, PHPV, microphthalmia, isolated or associated with retinal dysplasia), other tumors (10 children, 13%; astrocytomas and medulloepithelioma), combined hamartomas (six children, 8%), inflammatory diseases (six children, 8%) (Toxocara canis, cat scratch eye disease, or toxoplasmosis), and other diseases (13 children, 16%; corneal opacities, congenital cataract, or retinal detachment).Compared to previous series, this study shows the proportion of erroneous diagnosis has lowered (16%) compared to earlier studies by Balmer (1986; 30%), and Shields (1991; 42%). No cases of retinopathy of prematurity were seen in our series, demonstrating that screening is good or of a lower frequency in France. The frequency of PHPV has dropped. Coats disease remains a frequent and sometimes difficult diagnosis to make, particularly in advanced stages of the disease. more...

To describe the evolution and management over time of scleral necrosis as a complication after radiation therapy for uveal melanomas.Retrospective nonrandomized case series.Twenty-three patients (12 male, 11 female; median age 56.4 years; range, 30-73 years) treated with a single plaque therapy (cobalt-60 (Co(60)) or iodine-125 (I(125)) (1.5% of treated patients) or proton beam radiotherapy (0.45% of treated patients) for choroidal or ciliary body melanoma, presenting scleral necrosis as a post-radiation complication, were studied. Tumor characteristics, intraocular pressure, location and size of scleral necrosis were recorded during visits after radiation therapy.The median maximal tumour base and thickness at diagnosis were 14.6 +/- 4.4 mm and 7.2 +/- 2.3 mm respectively. Median radiation dose to tumor base and tumor apex was 223 Gy and 88.3 Gy respectively. The mean time of scleral necrosis onset after radiation therapy was 70.4 months (range 11-257 months); the mean scleral necrosis diameter at its onset was 4.4 mm (range 1-10 mm). Out of 23 affected eyes, 17 presented a stability of scleral necrosis; in three cases there was partial regression of scleral necrosis; in two cases there was a progressive extension of scleral necrosis until a pre-perforation stade. In two cases, a secondary enucleation was necessary due to a severe neovascular glaucoma.Scleral necrosis is a rare complication of radiotherapy for uveal melanomas. Relapse with extrascleral extension of the primary tumor has to be ruled out in order to avoid unnecessary enuclations. In most patients, the scleral necrosis remains stable and does not need any further treatment. more...

Purpose: Introduction : Patients with peripheral choroidal melanoma are often misdiagnosed as uveal melanoma Methods: We have reviewed the clinical , MRI and ultrasonographic characteristics of patients sent to Curie institute for suspected uveal melanoma between 1998 and 2006 and of whom final diagnosis was choroidal hematoma Results: 95 patients were seen with this diagnosis ( for 2357 patients treated for uveal melanoma during the same period); the age varies between 54 and 92 years with a median age of 77 years; there was a history of macular degeneration in 27 cases and posterior pole drusen in 3 cases.11 patients were taking anticoagulant or antiaggregant medications .There was sudden loss of vision in 35 cases and a vitreous haemorrhage in 18 cases. The lesion was located in the posterior pole in 28 cases and in the periphery in 67 cases. The thickness measured by B scan ultrasonography varied between 1 mm and 7, 8 mm with a mean thickness of 2, 86 mm. MRI was necessary in 27 cases and color doppler ultrasonography in 10 cases. These exams usually show peripheral enhancement with gadolinium (compared to full thickness gadolinium enhancement in melanomas) and no vessels with doppler (compared to visible vascularisation in melanomas) A follow up was performed in 42 patients showing progressive fibrin scarring Conclusions: These peripheral hematomas can be due to peripheral exsudative chorioretinopathy or to ruptured retinal macroanevrysm; they are more frequent in elderly patients with vascular diseases . The diagnosis of choroidal hematoma is generally made by fundus examination but MRI and color doppler imaging can be usefull more...

Udgivelsesdato: 2005

Uveal melanoma is very rare in children, and in both adults and children it can in rare cases develop intralesional cavities resembling an intraocular cyst. The presence of a solid mass at the base and a thick wall surrounding the cavity can assist in differentiating cavitary melanoma from a benign cyst. We report the case of a 5-year-old girl who presented with a large intraocular pigmented mass in the left eye, showing multiple hollow cavities on ocular ultrasonography, CT scan, and MRI. The patient was treated by enucleation and the pathology confirmed the diagnosis of choroidal melanoma. FISH revealed no aberration in chromosome 3. more...

Introduction La localisation orbitaire du rhabdomyosarcome (RMS) represente 10 % de l’ensemble des RMS et est a l’origine de 3 a 4 % des exophtalmies de l’enfant. L’objectif de cette etude est de decrire les caracteristiques cliniques, therapeutiques et les sequelles a long terme des enfants atteints de RMS orbitaire ainsi que d’identifier des facteurs pronostics propres a cette localisation. Materiels et Methodes Etude retrospective monocentrique des dossiers de 59 enfants atteints de RMS orbitaires traites entre 1980 et 1999. Resultats L’exophtalmie est initialement presente dans 42 cas et il existe une atteinte para meningee dans 16 cas. En fin de traitement initial (ayant compris une chimiotherapie dans tous les cas, une radiotherapie dans 81 % des cas, une tentative d’exerese tumorale au diagnostic dans 24 cas et une chirurgie mutilante dans 3 cas), 83 % des enfants etaient en premiere remission complete. 38 % des 18 enfants ayant rechute ou n’ayant pas ete sensibles a la chimiotherapie premiere, ont repondu a un traitement de deuxieme ligne. Au total (traitements de 1re ligne et rechute), 91 % des enfants ont ete irradies. Apres un suivi median de 8 ans, la survie globale (SG) des 59 enfants est de 81 ± 5 % et l’intervalle libre sans evenement de 66 ± 7 %. Le seul facteur ayant une influence significative sur la SG est l’existence d’une atteinte para meningee (p = 0,02). Les autres facteurs etudies (âge, sexe, exophtalmie au diagnostic, taille de la tumeur, stade clinique, periode de traitement et modalites de la radiotherapie) n’influencent pas la SG. Au total, 4 enfants ont ete exenteres. Les sequelles post-radiques restent importantes : cataracte (37 %), baisse de l’acuite visuelle (50 %), asymetrie faciale, hypoplasie orbitaire, enophtalmie (22 a 33 %). Discussion Malgre une forte dose de radiotherapie, le taux de sequelles est moindre que dans les autres series publiees. Conclusion L’exophtalmie fait partie dans 71 % des cas du tableau clinique revelateur du RMS orbitaire. Dans cette localisation, le pronostic vital du RMS est actuellement bon avec cependant des sequelles radiques qui restent importantes justifiant d’adapter les traitements a l’importance de la maladie initiale, l’atteinte para meningee etant un des principaux facteurs pronostique pejoratif. more...

9538 Background: intraocular retinoblastoma treatments often associate chemotherapy and focal treatments. The protocols vary and may combine two or three drugs, and different number of cycles associated to the local adjuvant treatments. A first prospective protocol of conservative treatments in our institution showed the efficacy of the use of two courses of chemoreduction with etoposide and carboplatin, followed by chemothermotherapy using carboplatin, as a single agent. In order to decrease the possible long term toxicity of chemotherapy due to etoposide, a prospective randomized phase II chemo reduction protocol was conducted, using vincristine and carboplatin vs. etoposide carboplatin. Methods: the study was proposed when initial tumour size or location did not allow the use of front-line local treatments. The phase II randomized study of reduction chemotherapy used vincristin carboplatin or etoposide carboplatin, followed by local treatment including chemothermotherapy using the combination carboplatin and laser diode hyperthermia. Primary endpoint was the need for secondary enucleation or EBRT not exceeding 40% at 2 years. The new treatment was considered sufficiently effective if 10 events or less were observed among 33 eyes. Results: 55 children, 65 eyes were included in the study (May 2004- August 2009).32 eyes (27 children) were treated conservatively in the arm etoposide-carboplatin and 33 (28 children) eyes in the arm vincristin carboplatin.At two years after treatment 23/33 (69.7%) eyes were treated and salvaged without EBRT or enucleation in the arm vincristin-carboplatin and 26/32 (81.3%) in the arm etoposide and carboplatin. Conclusions: neoadjuvant chemotherapy by two cycles of vincristine and carboplatin followed by chemothermotherapy (using the combination carboplatin, as a single drug, and laser diode hyperthermia) does not seem to offer an optimal local control. more...

Contexte Le risque de recurrence chez les enfants de parent atteint d’un retinoblastome (Rb) familial (transmission autosomique dominante a forte penetrance) justifie un depistage des la periode neonatale par examen du fond d’œil. Objectif Evaluer les benefices du depistage precoce des formes familiales de Rb. Materiel et methodes Etude retrospective monocentrique. Comparaison du devenir des enfants selon que le depistage a ete debute des la premiere semaine de vie (G1), avant l’âge de 6 mois (G2) ou au-dela (G3) entre 1995 et 2004 a l’Institut Curie. Resultats Une enucleation a ete realisee respectivement pour 0/16, 2/23 et 13/20 des enfants des groupes G1, G2 et G3 ( p p Conclusion Le depistage neonatal du Rb familial diminue le risque d’enucleation et de RTE. Le recours a la chimiotherapie et l’AV finale ne semble pas significativement differents bien que cela necessite d’etre etudie sur une plus grande cohorte. more...