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PURPOSE: To evaluate the outcome of patients with retinoblastoma and postlaminar optic nerve invasion (PLONI). DESIGN: Retrospective interventional case series. PARTICIPANTS: Sixty-one consecutive patients included in 3 successive protocols were analyzed. METHODS: Pathologic review was done in each case. Patients were stratified into 2 risk groups: the high-risk group included those with concomitant full choroidal and/or scleral invasion and were given adjuvant chemotherapy. Those without these features were considered low risk and chemotherapy was withheld after 1994. MAIN OUTCOME MEASURES: Extraocular relapse and survival according to stratification. RESULTS: The probability of event-free survival (pEFS) was 0.91 and the probability of overall survival (pOS) was 0.94 at 5 years. Patients in the high-risk group (n = 22) had pEFS of 0.86. Three had extraocular relapse (involving the central nervous system; all died of disease). Microscopic scleral invasion was associated to extraocular relapse (P = 0.05). Lower risk patients (n = 39) had a pEFS of 0.94 and pOS of 1. Eighteen received postenucleation chemotherapy and none relapsed. Twenty-one received no adjuvant therapy and 2 had a systemic relapse but were successfully retrieved. Relapsing patients had a higher ratio of affected optic nerve (>25% of it overall length; P = 0.02). CONCLUSIONS: Patients with PLONI have an excellent outcome with current therapy. Risk stratification according to the presence of concomitant choroidal and/or scleral invasion may help in the decision of giving adjuvant therapy.

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There is general agreement that bone marrow (BM) examination for staging in patients with retinoblastoma should be limited to cases with advanced disease. However, there are limited data about the yield of sampling multiple sites with aspirations and biopsies and immunocytology. Our policy for BM examination included: 2 aspirates and 2 biopsies at the posterior iliac crest scheduled only for cases with postlaminar optic nerve extension (n=56), scleral invasion (n=10) or orbital (n=5) or metastatic disease at diagnosis (n=7) or at extraocular relapse (n=18). Immunocytology with the antibodies 3A7 or 3F8 for the ganglioside GD2 was performed. From 1/1994 to 3/2005, 277 newly diagnosed patients and 5 at extraocular relapse were included. BM invasion was not found in any of the 66 patients enucleated with disease confined to the globe, but was found in 11/27 of those with overt extraocular disease. There were 2/11 cases with at least 1 negative aspirate with positive biopsy and/or immunocytology for GD2. GD2 positivity was found in 9/9 cases. A more aggressive BM evaluation has a low yield in enucleated patients with high-risk features but disease limited to the globe. However, in cases with overt extraocular dissemination, the use of BM biopsy and immunocytology for GD2 allowed for the detection of cases that would have been missed by aspirations alone. GD2 was intensively expressed and it may also be used to monitor disease response and the presence of minimal residual disease.

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Gastric lymphoepithelioma--like carcinoma, undifferentiated with lymphoid or medullary stroma, constitutes a 3.8% of gastric carcinomas. Microscopically it is similar to other lymphoepitheliomas, it has an expansive growth and better prognosis than other histologic types. A possible relation to Epstein Barr virus is presumed. A 56 year old female, gastrectomized due to a cancer (Billroth II) 31 years before, showed an ulcer lesion close to the anastomosis at endoscopy. A biopsy was taken with positive result and gastrectomy was performed. The surgical specimen included gastric stump, jejunoanastomosis, omentum and lymph nodes. It had a 4.5 cm, protruded ulcerated lesion, located in anterior wall, 1.5 cm from stomy. It was fixed in buffered formalin, routinely processed and stained with Hematoxilin--Eosin. Immunohistochemistry techniques for CKAE1-AE3, EMA, CEA, CD45, CD20, CD3 and CD45Ro, and PCR for Epstein Barr virus were performed. Microscopically it was constituted by an expansive proliferation of polygonal cells with barely eosinophylic cytoplasm and vesicular nuclei, arranged in small clusters or isolated, positive for CKAE1-AE3, EMA and CEA, with dense mature lymphoid infiltrate, both follicular and diffuse, positive for CD45, CD45Ro, CD3 and CD20. PCR technique for Epstein Barr virus was positive. This has been the first case of this type in our department since 1989. In addition to the peculiar characteristics of this variety, whose differential diagnosis must be made with lymphomas, the origin from gastric stump, where this complication appears with variable rates, the risk being related to the type of surgery and the time elapsed are relevant.

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El carcinoma de tipo linfoepitelioma, indiferenciado con estroma linfoide o medular, constituye un 3,8% de los carcinomas gástricos. Microscópicamente es similar a linfoepiteliomas de otras localizaciones, tiene crecimiento expansivo y mejor pronóstico que otros tipos histológicos. Se postula probable relación con el virus de Epstein Barr. Una mujer de 56 años gastrectomizada por cáncer hacía 31 años (Billroth) mostró en la endoscopía lesión ulcerada próxima a neboca. Se biopsió con resultado positivo y se realizó gastrectomía. Se recibió pieza que incluía muñón gástrico, yeyuno anastomosis, epiplón y glanglios. Presentaba lesión sobreelevada, ulcerada, de 4,5 cm ubicada en cara anterior a 1,5 cm de neoboca. Se efectuó fijación en formol buffer, procesamiento según métodos habituales y coloración con Hematoxilina-Eosina. Se realizaron técnicas de Inmunohistoquímica para CKAE1-AE3, EMA, CEA, CD45, CD20, CD3 y CD45Ro, y de PCR para virus de Epstein Barr. Microscópicamente estaba constituida por una proliferación expansiva de células poligonades con citoplasma ligeiramente eosinófilo y núcleos vesiculares, dispuestas en pequeños nidos o asiladas, positivas para CKES1-AE3, EMA y CEA, con denso infiltrado linfoide maduro, de disposición folicular y difuso, positivo para CD45, CD45Ro, CD3 y CD20. La técnica de PCR fue positiva. Este es el primer caso de este tipo registrado en nuestro servicio desde 1989. Además de las características peculiares de esta variedad, cuyo diagnóstico diferencial debe haceres con los linfomas. es relevante su origen en muñón gástrico, donde esta complicación aparece con cifras variables, estando el riesgo vinculado al tipo de cirgía y tiempo transcurrido.(AU)

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El carcinoma de tipo linfoepitelioma, indiferenciado con estroma linfoide o medular, constituye un 3,8% de los carcinomas gástricos. Microscópicamente es similar a linfoepiteliomas de otras localizaciones, tiene crecimiento expansivo y mejor pronóstico que otros tipos histológicos. Se postula probable relación con el virus de Epstein Barr. Una mujer de 56 años gastrectomizada por cáncer hacía 31 años (Billroth) mostró en la endoscopía lesión ulcerada próxima a neboca. Se biopsió con resultado positivo y se realizó gastrectomía. Se recibió pieza que incluía muñón gástrico, yeyuno anastomosis, epiplón y glanglios. Presentaba lesión sobreelevada, ulcerada, de 4,5 cm ubicada en cara anterior a 1,5 cm de neoboca. Se efectuó fijación en formol buffer, procesamiento según métodos habituales y coloración con Hematoxilina-Eosina. Se realizaron técnicas de Inmunohistoquímica para CKAE1-AE3, EMA, CEA, CD45, CD20, CD3 y CD45Ro, y de PCR para virus de Epstein Barr. Microscópicamente estaba constituida por una proliferación expansiva de células poligonades con citoplasma ligeiramente eosinófilo y núcleos vesiculares, dispuestas en pequeños nidos o asiladas, positivas para CKES1-AE3, EMA y CEA, con denso infiltrado linfoide maduro, de disposición folicular y difuso, positivo para CD45, CD45Ro, CD3 y CD20. La técnica de PCR fue positiva. Éste es el primer caso de este tipo registrado en nuestro servicio desde 1989. Además de las características peculiares de esta variedad, cuyo diagnóstico diferencial debe haceres con los linfomas. es relevante su origen en muñón gástrico, donde esta complicación aparece con cifras variables, estando el riesgo vinculado al tipo de cirgía y tiempo transcurrido.

Asunto(s)

RESUMEN

Gastric lymphoepithelioma--like carcinoma, undifferentiated with lymphoid or medullary stroma, constitutes a 3.8

of gastric carcinomas. Microscopically it is similar to other lymphoepitheliomas, it has an expansive growth and better prognosis than other histologic types. A possible relation to Epstein Barr virus is presumed. A 56 year old female, gastrectomized due to a cancer (Billroth II) 31 years before, showed an ulcer lesion close to the anastomosis at endoscopy. A biopsy was taken with positive result and gastrectomy was performed. The surgical specimen included gastric stump, jejunoanastomosis, omentum and lymph nodes. It had a 4.5 cm, protruded ulcerated lesion, located in anterior wall, 1.5 cm from stomy. It was fixed in buffered formalin, routinely processed and stained with Hematoxilin--Eosin. Immunohistochemistry techniques for CKAE1-AE3, EMA, CEA, CD45, CD20, CD3 and CD45Ro, and PCR for Epstein Barr virus were performed. Microscopically it was constituted by an expansive proliferation of polygonal cells with barely eosinophylic cytoplasm and vesicular nuclei, arranged in small clusters or isolated, positive for CKAE1-AE3, EMA and CEA, with dense mature lymphoid infiltrate, both follicular and diffuse, positive for CD45, CD45Ro, CD3 and CD20. PCR technique for Epstein Barr virus was positive. This has been the first case of this type in our department since 1989. In addition to the peculiar characteristics of this variety, whose differential diagnosis must be made with lymphomas, the origin from gastric stump, where this complication appears with variable rates, the risk being related to the type of surgery and the time elapsed are relevant.