Pulmonary artery sarcoma is a rare tumor with lethal prognosis. Its etiology is unknown; most frequently the pulmonary trunk and its main branches are affected. Magnetic resonance imaging with gadolinium and positron emission tomography are complementary studies that can guide the diagnosis, but this can only be established with the histological study. We present this case because of the low incidence and its importance in the differential diagnosis of pulmonary thromboembolism.

El sarcoma de la arteria pulmonar es una patología rara y de pronóstico letal. Se desconoce su etiología, pero con mayor frecuencia afecta al tronco de la arteria pulmonar y a sus ramas principales. Los estudios complementarios mediante resonancia magnética con gadolinio y tomografía por emisión de positrones son de utilidad para orientar el diagnóstico, pero este sólo se puede establecer al realizar un estudio histológico. Presentamos este caso dada la baja incidencia y su importancia para el diagnóstico diferencial del tromboembolismo pulmonar.

As metástases a distância em carcinoma diferenciado de tireoide são raras. Pulmão e ossos são os lugares mais frequentemente atingidos. As metástases em tecidos moles são extremamente infrequentes. Neste artigo, descrevemos dois casos de pacientes com câncer diferenciado de tireoide com metástases em tecidos moles. Ambos os pacientes padeciam de enfermidade avançada. As metástases em partes moles foram assintomáticas e detectadas mediante 18-Fluordeoxyglucose positron emission tomography/computed tomography (18F FDG PET-CT), sendo confirmadas por citologia e/ou histopatologia. Esses achados ressaltam o valor do 18F FDG PET-CT para a avaliação correta da extensão da enfermidade e a utilidade do método para avaliar regiões do corpo que não são exploradas nos estudos de rotina.

Distant metastases of differentiated thyroid cancer are unusual; lung and bones are the most frequently affected sites. Soft tissue metastases (STM) are extremely rare. We describe two cases of patients with differentiated thyroid cancer metastasizing to soft tissues. Both patients had widespread metastatic disease; clinically asymptomatic soft tissue metastases were found by 18-Fluordeoxyglucose positron emission tomography/computed tomography (18F FDG PET-CT), and confirmed by cytological and/or histopathological studies. These findings underscore the ability of 18F FDG PET-CT in accurately assessing the extent of the disease, as well as the utility of the method to evaluate regions of the body that are not routinely explored.

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.

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.

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.

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.

Signet ring cell lymphoma is a rare neoplasm caracterized 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 oropharingeal 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.

El linfoma de células en anillo de sello es una neoplasia poco frecuente, caracterizada por la presensencia de grandes células de citoplasma vacuolado, claro, similar a los encontrados en adeno-carcinomas mucosecretantes. Se ha descripto en ganglios linfáticos y tejidos extralinfáticos. Se presenta el caso de un paciente varón de 59 años con linfoma de células en anillo de sello localizado en pared lateral de orofaringe. Esta localización creemos que no ha sido descripta anteriormente. La histología mostró células en anillo de sello y el inmunofenotipo tumoral fue: vimentina, CD45, CD20, Ig M y cadenas kappa positivos, siendo la fracción de crecimiento tumoral alta (Ki 67:70%). El paciente evolucionó desfavorablemente y falleció a los dos meses del diagnóstico sin recibir tratamiento.