[Orbital and ocular adnexal lymphomas. Clinico-pathological correlation in 25 cases]. / Linfomas de la órbita y anexos oculares. Correlación clínico patológica de 25 casos.

Clinical, histological features and outcome of a cohort of patients with orbital and adnexal lymphoproliferative tumors were evaluated. Twenty-five cases in an oncologic referral center from 1995 to 2008, were included in the study. Each case had detailed immunophenotypic analysis using a panel of monoclonal antibodies (CD45, CD20, CD3, CD5, CD23, BCL2, BCL6, BCL10, Ki67, CD30, CD15, BCL1, Kappa, Lambda, CD138). Lesions were classified by using WHO (2008) lymphomas classification. Twenty-three patients were found to have primary and two secondary lymphomas. Histological subtypes were: 16 patients with marginal zone B cell lymphoma of mucosa-associated lymphoid tissue (MALT) lymphoma, four diffuse large B cell lymphomas, two mantle cell lymphomas, two follicular lymphomas, and one Hodgkin lymphoma. Among the 25 patients studied, 22 had localized stage. Extranodal marginal zone lymphoma was the most frequent type of primary orbital and adnexal lymphoma. In this study localized disease was observed in most cases, and distant spread of the lymphomas was infrequent.

Individual patient risk of progression of urinary bladder papillary tumors estimated from biomarkers at initial transurethral resection of bladder tumor.

OBJECTIVE: To determine if individual, instead of group, patient progression risk could be predicted using p53, Ki67 and CK20 biomarker percentage values at initial transurethral resection of bladder tumor specimens. METHODS: This was an observational study where biomarkers were measured with no knowledge of tumor outcome. Initial bladder tumor specimens were classified as non-invasive and invasive to sub-epithelium (pT1). Percentages of stained biomarker cells were tested as progression predictors from non-invasive to pT1 and pT1 to pT2. Progression probability was correlated with biomarker percentages resulting in a regression equation. RESULTS: We studied 112 patients (median age = 67, range 37-91, males 83/112 (73%), with median follow-up of 39 months (range 1.7-140). Mean biomarker values were higher in stage pT1 than in non-invasive (all p < 0.001). Cut-off points separating progression from non-progression groups in stage pT1 were higher than in non-invasive for all biomarkers. Correlation R values for progression probability vs. biomarker percentages varied from 0.7 to 0.9 (all p < 0.001), regression slopes from 0.1 to 0.8 and intercepts from 11 to 35. A novel individual progression probability was calculated as the product of biomarker percentage of stained cells and slope, plus the prevalence-adjusted intercept. CONCLUSIONS: Identification of individual risk of progression in patients with non-muscle-invasive bladder tumors was possible using p53- and Ki67-derived progression probability using a regression equation. Combining biomarker-derived progression probability to tumor stage pT1 improves progression to pT2 predictive accuracy.

[Hematodermic CD4+ CD56+ neoplasm in childhood]. / Neoplasia hematodérmica CD4+ CD56+ en la infancia.

Hematodermic CD4+ CD56+ neoplasm with plasmacytoid dendritic cell phenotype is a rare and aggressive neoplasm recently recognized by the WHO-EORTC classification. It generally appears in elderly adults, exceptionally in childhood. We present a 12-year-old girl with severe mental retardation, genetic clinical features and multiple nodular cutaneous lesions on legs and arms. Histologically the nodules showed diffuse dermal infiltrate of medium and small cells and expression of CD4, CD56, CD43, S100 and plasmacytoid dendritic markers: CD123, BDCA-2 under flow cytometry study. Peripheral blood and bone marrow were not involved. Clinical remission of cutaneous lesions was observed after two weeks of acute lymphoblastic leukemia therapy.

[Signet ring cell lymphoma mimicking mucin-producing carcinoma]. / Linfoma de células en anillo de sello que simula carcinoma mucosecretante.

Signet ring cell lymphoma is a rare neoplasm characterized by large, vacuolated and clear cells mimicking mucin-producing adenocarcinoma. It is localized in nodal and extranodal sites. A case of a 59 years old male, with a diffuse lymphoma signet ring cell type localized on oropharyngeal mucosa is reported. The histopathology study showed signet ring cells and the immunophenotype was: vimentine(+), CD45(+), CD20(+), Ig M(+), Kappa chain(+) and high index proliferative activity of neoplastic cells (Ki 67:70%). After a review of the literature and previous reports, we could not find a similar case in this anatomic site. The patient had a unfavourable clinical course and died two months after the diagnosis without receiving any treatment.

Primary NK/T cell lymphoma nasal type of the colon.

Since nasal NK/T-cell lymphoma and NK/T-cell lymphoma nasal type are rare diseases, colonic involvement has seldom been seen. We report a case of a patient with a primary NK/T-cell lymphoma nasal type of the colon. The patient had no history of malignant diseases and was diagnosed after exhaustive study in the context of fever of unknown origin. The first therapeutic approach followed the DA-EPOCH-protocol: etoposide, prednisone, doxor-rubicin, vincristine and cyclophosphamide. The persistence of constitutional symptoms after the first treatment course motivated the switch to a second line following the SMILE-protocol: dexamethasone, metotrexate, ifosfamide, E.coli L-asparaginase, and etoposide. Despite intensive chemotherapy, the patient died 2 months after the diagnose of an extranodal NK/T-cell lymphoma of the colon and 4 months after the first symptomatic appearance of disease.

Linfomas de la órbita y anexos oculares: Correlación clínico patológica de 25 casos / Orbital and ocular adnexal lymphomas: Clinico-pathological correlation in 25 cases

Se evaluaron las características clínicas, histológicas y la evolución de una cohorte de pacientes con linfomas de la órbita y anexos oculares. Entre 1995 y 2008 se estudiaron 25 casos de linfomas de la órbita y anexos oculares en un centro oncológico de referencia. En cada caso se analizó el inmunofenotipo usando un panel de anticuerpos monoclonales (CD45, CD20, CD3, CD5, CD23, BCL2, BCL6, BCL10, Ki67, CD30, CD15, BCL1, Kappa, Lambda, CD138). Las lesiones fueron evaluadas utilizando el sistema de clasificación de linfomas (OMS, 2008). Se analizaron 23 linfomas primarios y dos secundarios. Los subtipos histológicos fueron: 16 linfomas B de la zona marginal asociados a las mucosas (MALT), cuatro linfomas difusos de células grandes B, dos linfomas foliculares y un paciente con linfoma Hodgkin. De los 25 casos estudiados, 22 presentaron estadios localizados. El linfoma MALT fue el subtipo más frecuente. En este estudio se observó enfermedad localizada en la mayoría de los casos y con baja progresión a distancia.

Clinical, histological features and outcome of a cohort of patients with orbital and adnexal lymphoproliferative tumors were evaluated. Twenty-five cases in an oncologic referral center from 1995 to 2008, were included in the study. Each case had detailed immunophenotypic analysis using a panel of monoclonal antibodies (CD45, CD20, CD3, CD5, CD23, BCL2, BCL6, BCL10, Ki67, CD30, CD15, BCL1, Kappa, Lambda, CD138). Lesions were classified by using WHO (2008) lymphomas classification. Twenty-three patients were found to have primary and two secondary lymphomas. Histological subtypes were: 16 patients with marginal zone B cell lymphoma of mucosa-associated lymphoid tissue (MALT) lymphoma, four diffuse large B cell lymphomas, two mantle cell lymphomas, two follicular lymphomas, and one Hodgkin lymphoma. Among the 25 patients studied, 22 had localized stage. Extranodal marginal zone lymphoma was the most frequent type of primary orbital and adnexal lymphoma. In this study localized disease was observed in most cases, and distant spread of the lymphomas was infrequent.

Neoplasia hematodérmica CD4+ CD56+ en la infancia / Hematodermic CD4+ CD56+ neoplasm in childhood

La neoplasia hematodérmica CD4+ CD56+ con fenotipo de célula dendrítica plasmocitoide es una rara y agresiva neoplasia recientemente reconocida por la WHO-EORTC classification. Afecta adultos de edad media y ancianos, siendo muy pocos los casos descriptos en niños. Presentamos el caso de una niña de 12 años con grave retraso mental, estigmas genéticos y múltiples lesiones cutáneas localizadas en miembros inferiores y superiores. Histológicamente se observó un infiltrado dérmico difuso de células pequeñas y medianas con expresión de CD4, CD56, CD43 y S100 así como de marcadores dendríticos plasmocitoides: CD 123 y BDCA-2 confirmados por citometría de flujo, sin compromiso de sangre periférica ni médula ósea. Cumpliendo dos semanas de tratamiento para leucemia linfoblástica aguda evolucionó con remisión clínica de las lesiones cutaneas.

Hematodermic CD4+ CD56+ neoplasm with plasmacytoid dendritic cell phenotype is a rare and aggressive neoplasm recently recognized by the WHO-EORTC classification. It generally appears in elderly adults, exceptionally in childhood. We present a 12-year-old girl with severe mental retardation, genetic clinical features and multiple nodular cutaneous lesions on legs and arms. Histologically the nodules showed diffuse dermal infiltrate of medium and small cells and expression of CD4, CD56, CD43, S100 and plasmacytoid dendritic markers: CD123, BDCA-2 under flow cytometry study. Peripheral blood and bone marrow were not involved. Clinical remission of cutaneous lesions was observed after two weeks of acute lymphoblastic leukemia therapy.

Linfoma gástrico primario anaplásico de células grandes CD30 (Ki-1) positivo / Primary gastric CD30 (Ki-1)-positive anaplastic large cell lymphoma

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Glomerulonefritis membranoproliferativa con semilunas y depósitos masivos de IgA asociada a HBsAg / Membranoproliferative glomerulonephritis with semilunar forms and massive deposits of IgA associated with HBsAg

Se presenta un joven coreano de 21 años con diagnóstico de ingreso al hospital de síndrome nefrótico, hipertensión arterial, microhematuria e insuficiencia renal, requiriendo hemodiálisis. El paciente era portador HBsAg y HBeAg sin enfermedad hepática clínica ni humoral, tampouco antecedentes de transfusiones ni adicción a drogas de uso intravenoso. Se le efectuó biopsia renal percutánea pudiendo diagnosticarse glomérulonefritis membrano-proliferativa en estado avanzado con formación de semilunas fibroepiteliales en el espacio de Bowman. La inmunofluorescencia mostró depósitos masivos de IgA con un patrón granular, en mesangio y capilares glomerulares. Mediante técnica de inmunoperoxidasas se comprobó depósitos de HBsAg en la pared de los capilares glomerulares, citoplasma de las células glomerulares, mesangio y túbulos renales. Se trataría del primer caso de glomérulonefritis membranoproliferativa con semilunas y depósitos masivos de IgA, insuficiencia renal progresiva con necesidad de hemodiálisis crónica, en un portador de HBsAg y HBeAg