N Cassoux

Conservative treatment of retinoblastoma: a prospective phase II randomized trial of neoadjuvant chemotherapy followed by local treatments and chemothermotherapy

PurposeIntraocular retinoblastoma treatments often combine chemotherapy and focal 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 and diode laser. In order to decrease the possible long-term toxicity of chemotherapy due to etoposide, a randomized neoadjuvant phase II protocol was conducted using vincristine–carboplatin vs etoposide–carboplatin.Patients and methodsThe study was proposed when initial tumor characteristics did not allow front-line local treatments. Patients included in this phase II noncomparative randomized study of neoadjuvant chemotherapy received vincristin–carboplatin (new arm) vs etoposide–carboplatin (our reference arm). They were subsequently treated by local treatments and chemothermotherapy. Primary end point was the need for secondary enucleation or external beam radiotherapy (EBRT) not exceeding 40% at 2 years.ResultsA total of 65 eyes in 55 children were included in the study (May 2004 to August 2009). Of these, 32 eyes (27 children) were treated in the arm etoposide–carboplatin and 33 eyes (28 children) in the arm vincristin–carboplatin. At 2 years after treatment, 23/33 (69.7%) eyes were treated and salvaged without EBRT or enucleation in the arm vincristin–carboplatin and 26/32 (81.2%) in the arm etoposide–carboplatin.ConclusionEven if the two treatment arms could be considered as sufficiently active according to the study decision rules, neoadjuvant chemotherapy by two cycles of vincristine–carboplatin followed by chemothermotherapy appear to offer less optimal local control than the etoposide–carboplatin combination.

Extériorisation extra-sclérale d’un mélanome choroïdien via un orifice de sclérotomie

Une patiente de 81 ans est adressée en consultation d’oncologie oculaire pour la prise en charge d’une volumineuse masse choroïdienne de l’œil droit. Une chirurgie endoculaire (vitrectomie avec mise en place d’huile de silicone) a été réalisée un mois auparavant pour un diagnostic initial d’hématome choroïdien associé à un décollement de rétine. L’analyse histologique d’un prélèvement réalisé pendant la chirurgie a conclu à un mélanome choroïdien. L’examen ophtalmologique retrouve un œil non voyant en postopératoire ; à la lampe à fente on visualise plusieurs nodules sous-conjonctivaux pigmentés dont l’un est situé au niveau d’un orifice de sclérotomie (Fig. 1). Il existe une volumineuse masse choroïdienne dont les dimensions ne sont pas évaluables en échographie oculaire du fait de la présence de silicone dans la cavité vitréenne. Une énucléation primaire est décidée en raison de la taille de la lésion tumorale. L’examen histologique du globe énucléé confirme le diagnostic de mélanome choroïdien de type mixte (à cel-

Conjunctival Melanoma in a Black Patient

A black woman in her early 40swithanunremarkablemedicalhistory presentedwithapigmentedconjunctival lesionoftherighteyethathad beenpresentsincechildhoodbuthadbeenincreasinginsizeforthepast few months (Figure, A). Tumor excision was performed under general anesthesia (using a “no-touch technique” as described previously1,2). Histological analysis showed conjunctival melanoma (Figure, B). The clinical stage of the tumor was T1aN0M0 according to the seventh editionoftheAmericanJointCommitteeonCancerstagingsystemforconjunctival melanoma.2 No mutations were found on the BRAF gene (exon 15), NRAS gene (codons 12, 13, 61, 117, and 146), or C-KIT gene (exons 11, 13, and 17). Adjuvant brachytherapy with iodine plaque was performed because of the invasion of the substantia propria.

Retrospective study of combined treatments for conjunctival melanoma

Purpose Since 2OOO, we have been using proton beam therapy after surgery for all invasive conjunctival melanomas. Patients with associated primary acquired melanosis also receive additional treatment with mitmycin 0, 04% drops at least two cycles. We have made a retrospective study to evaluate the results.

The treatment of retinoblastoma with intravenous chemotherapy + local treatment

Patients and method: Treatments protocols applied since 1995 included intraveinous chemotherapy at various dosages with local ophthalmological treatment by transpupillary thermotherapy, cryotherapy and iodine 125 plaque brachytherapy. The data concerning initial findings, treatments used, and results are registered in our database in a prospective way. We performed a retrospective study of children treated between January 1995 and December 2009. Results: There were 317 patients with bilateral retinoblastoma and 411 patients with unilateral retinoblastoma. The median follow up is 72 months. Median age at diagnosis was 7 month for bilateral and 20 months for unilateral. In bilateral patients, 104 had conservative management of both eyes, 204 had a conservative management of one eye and enucleation of the fellow eye and 9 had bilateral enucleation. Among the 312 eyes treated conservatively, 83 eyes were irradiated and 13 had secondary enucleation. (Success rate 70%) Among 411 unilateral retinoblastoma, 87(21%) were treated conservatively. 71 eyes were preserved (67 without external beam and 4 with external beam) (success rate 77%) Conclusion: Chemotherapy associated to local ophthalmological treatments is efficient in the

Fine needle aspiration biopsy in uveal melanoma : the Curie experience

Purpose In order to posssibly include uveal melanoma patients in a randomized trial on adjuvant therapy by Fotemustine we have started in 2009 to propose fine needle aspiration biopsy for genetic studies during clip or plaque positioning

524 Le léiomyome de l’uvée : un diagnostic différentiel difficile du mélanome

Introduction Les leiomyomes sont des tumeurs benignes derivees des cellules musculaires lisses. La localisation intraoculaire des leiomyomes est rare et exclusivement uveale. Materiels et Methodes Etude retrospective des dossiers de 3 patients ayant presente un leiomyome intraoculaire pris en charge dans un centre specialise en oncologie oculaire. Resultats 3 patients : 2 hommes et 1femme, âges de 18 ans, 20 ans et 22 ans au diagnostic. Ils presentaient une masse tumorale saillante choroidienne de pigmentation variable, mesurant plus de 10 mm d’epaisseur en echographie B. Une IRM avec injection de gadolinium a ete realisee chez les 3 patients et montre une volumineuse tumeur intraoculaire rehaussee apres injection de produit de contraste. Dans deux cas, le diagnostic initial a ete celui de melanome choroidien. Pour le troisieme cas il existait un doute diagnostic (lesion totalement achrome) et l’accessibilite de la masse a conduit a la realisation d’une resection partielle par sclero-uvectomie. L’etude anatomopathologique a montre que ces trois tumeurs etaient des leiomyomes dont deux de type meso-ectodermiques (les trois tumeurs exprimaient la desmine et l’actine musculaire lisse et pas la proteine S 100, ni l’HBM45). Discussion Les leiomyomes de l’uvee sont des tumeurs tres rares (environ une cinquantaine de cas ont ete rapportes dans la litterature) survenant le plus souvent chez des sujets jeunes. Aucun argument clinique ne permet avec certitude le diagnostic differentiel avec d’autres tumeurs intraoculaires. L’examen anatomopathologique est donc essentiel et necessite bien souvent une etude immuno histochimique. Conclusion Les leiomyomes de l’uvee sont des tumeurs intraoculaires benignes de diagnostic tres difficile et pour lesquels le diagnostic differentiel avec le melanome de l’uvee est particulierement delicat etproblematique en raison des sanctions therapeutiques.