Angioma de células litorales esplénico. Presentación de caso clínico / Littoral cell angioma of the spleen. Report on a clinical case / Angioma de células litoraneas do baço: apresentação de um caso clínico

El angioma de células litorales del bazo es una neoplasia vascular benigna poco frecuente, de presentación clínica, paraclínica e imagenológica inespecífica. Se realizó en nuestro paciente la esplenectomía, el tratamiento indicado y curativo, y se logró el diagnóstico a través de la histología e inmunohistoquímica de la pieza de anatomía patológica.

Littoral cell angioma of the spleen is rather an unusual benign vascular neoplasm with non-specific clinical, paraclinical and imaging presentation. Our patient underwent splenectomy, performed as the appropriate and remedial treatment. Diagnosis was made by means of histology and immunohistochemistry techniques of the pathologic anatomy of the surgical piece.

O angioma de células litoraneas do baço é uma neoplasia vascular benigna rara com apresentação clínica, laboratorial e de imagem inespecíficas. A esplenectomia foi realizada em nosso paciente, sendo esse o tratamento indicado e curativo, obtendo-se o diagnóstico através da histologia e imunohistoquímica da peça anatomopatológica.

Un absceso esplénico por endocarditis / Abscess due to endocardities

Este artículo se publicó solamente con imágenes.

Angioma de células litorales del bazo: un diagnóstico de lesión focal esplénica: caso clínico / Littoral cell angioma of the spleen: report of one case

Splenic vascular neoplasms are the most common form of spleen tumors. Among them, littoral cell angioma is rare and it is frequently an incidental finding in imaging studies. It has no specific clinical, laboratory or imaging findings. Splenectomy allows definitive diagnosis throughout a histopathological examination. We report a 52-year-old man presenting with asthenia and abdominal distension. Computed tomography with intravenous contrast showed multiple splenic hypodense masses and a prostatic enlargement. Presuming a lymphoma, a laparoscopic splenectomy was performed. Histopathologic examination diagnosed littoral cell angioma. During urological follow-up, a prostate adenocarcinoma was diagnosed.

Diagnóstico Histopatológico e Imuno-histoquímico de Linfoma de Baço com Apresentação Clínica Atípica: Relato de Caso / Histopathological and Immunohistochemical Diagnosis of Spleen Lymphoma with Atypical Clinic Presentation: Case Report / Diagnóstico Histopatológico e Inmunohistoquímico de Linfoma de Bazo con Presentación Clínica Atípica: Relato de Caso

Introdução: Tanto o angiossarcoma esplênico quanto o linfoma não Hodgkin (LNH) de células B não só são raros como apresentam desafios diagnósticos. Relato do caso: Paciente do sexo masculino, 45 anos, obeso, sudorese noturna há um ano, e dor abdominal cíclica há dois, com clínica inicial de angiossarcoma, contudo, sob o exame histopatológico e imuno-histoquímico, mostrou tratar-se de LNH de células B com apresentação atípica. A ultrassonografia (US) de abdome total realizada ao início do sintoma de sudorese noturna não teve alterações. Após um ano, a US apresentou massa esplênica. Na ressonância magnética da região abdominal, constatou-se tumoração esplênica expansiva heterogênea no aspecto anterior, suspeita de angiossarcoma esplênico. Após esplenectomia total, pancreatectomia caudal e linfadenectomia retroperitoneal, o exame histopatológico mostrou neoplasia maligna pouco diferenciada extensamente necrótica substituindo o parênquima esplênico. O estudo imuno-histoquímico foi positivo para os marcadores CD20 em diversos focos, para o BCL2 e para o KI67 (em 95% dos núcleos das células neoplásicas). A histopatologia e a imuno-histoquímica foram compatíveis com o diagnóstico de LNH difuso de alto grau, com imunofenótipo B do baço, sem sinal de infiltração de tecidos adjacentes. Após a esplenectomia total e quatro ciclos de quimioterapia, o paciente estava livre do linfoma e sem sintomas. Conclusão: É de suma importância o reconhecimento do diagnóstico correto de neoplasias raras como a relatada. Tanto a clínica quanto os exames de imagem, mesmo que fundamentais, podem ser imprecisos, reiterando a importância dos exames histológico e imuno-histoquímico complementares

Introduction: Both the splenic angiosarcoma and the B-cell non-Hodgkin's lymphoma (NHL) are not only rare but are challenging to diagnose. Case report: Male patient, 45 years old, obese, night sweats for one year, and cyclic abdominal pain for two years, with an initial angiosarcoma clinic, however after immunohistochemistry test, it was revealed a B-cell NHL with atypical presentation. The total abdomen ultrasound (US) performed at the onset of the night sweating symptom showed no alterations. After one year, the US showed a splenic mass. Magnetic resonance imaging of the abdominal region revealed a heterogeneous expansive splenic lesion in the anterior aspect with suspected splenic angiosarcoma. After full splenectomy, caudal pancreatectomy, and retroperitoneal lymphadenectomy, histological analysis showed an area of poorly differentiated necrotic malignancy infiltrated in the splenic parenchyma. Immunohistochemical analysis was positive for CD20 reagents in several foci, BCL2, and for KI67 (in ninety percent of the neoplastic cell nucleus). Histopathology and immunohistochemistry are consistent with high-grade, diffuse, NHL of immunophenotype B in the spleen, with no sign of metastasis to adjacent tissues. After total splenectomy and four rounds of chemotherapy, the patient had remission of the tumor and was asymptomatic. Conclusion: It is extremely important to recognize the relevance of the correct diagnosis of rare neoplasms such as the one reported. Both clinical and imaging tests, although important, can be inaccurate, reiterating the importance of complementary histologic and immunohistochemical tests

Introducción: El angiosarcoma esplénico e el linfoma no Hodgkin (LNH) de células B no solo son raros sino que presentan un reto diagnóstico. Relato del caso: Paciente masculino de 45 años, obeso, con sudoración nocturna durante un año y dolor abdominal cíclico durante dos años, con clínica inicial de angiosarcoma, pero bajo inmunohistoquímica mostró LNH de células B con presentación atípica. En la ecografía de abdomen total realizada al comienzo del síntoma de sudoración nocturna no hubo cambios. Después de un año, la ecografía enseñó una masa esplénica. La resonancia magnética de la región abdominal reveló una lesión esplénica expansiva heterogénea en la cara anterior, sospechosa de angiosarcoma esplénico. Tras la esplenectomía total, la pancreatectomía caudal y la linfadenectomía retroperitoneal, el análisis histológico mostró un área de neoplasia maligna necrótica poco diferenciada infiltrada en el parénquima esplénico. El análisis inmunohistoquímico fue positivo para los reactivos CD20 en varios focos, BCL2 y KI67 en el 95% del núcleo celular, la histología y la inmunohistoquímica son consistentes con LNH difuso de alto grado de inmunofenotipo B en el bazo, sin signos de metástasis. Tras esplenectomía total y cuatro rondas de quimioterapia, el paciente presentó remisión del tumor, así como disminución de síntomas. Conclusión: Es de suma importancia reconocer el diagnóstico de neoplasias raras como la reportada. Tanto las pruebas clínicas como las de imagen, aunque fundamentales, pueden ser inexactas, reiterando la importancia de las pruebas histopatológicas e inmunohistológicas

Manejode linfoma primario de bazo en paciente con ViH-Sida: reporte de caso / Management of primary lymphoma of the spleen in a patient with HIV-AIDS: case report

Los linfomas primarios de bazo afectan al bazo y/o sus ganglios linfáticos inicialmente. Representan <1% de los linfomas e implican un reto diagnóstico, por baja prevalencia y sintomatología inusual. Por lo tanto, el objetivo de este trabajo fue describir el manejo y evolución de un paciente con VIH-SIDA y la presentación de un Linfoma primario de bazo.En este reporte presentamos el caso de un paciente masculino de 52 años con VIH sin apego a tratamiento debuta con dolor abdominal, fiebre y pérdida de peso de 2 meses de evolución. La tomografía computarizada properatoria revela esplenomegalia y lesiones hipodensas sospechosas de neoformación. Es intervenido, realizándose una laparotomía exploratoria, con esplenectomía. El estudio histopatológico reporta linfoma no Hodgkin.El linfoma primario de bazo es poco frecuente. Es necesario su conocimiento para su sospecha diagnóstica y un diagnóstico diferencial en pacientes con esplenomegalia.Primary splenic lymphomas involve the spleen and/or its lymph nodes. They represent <1% of lymphomas and they imply a diagnostic challenge due to its poor symptomatology and low prevalence.There for, the objects this work describe the management and evolution of a patient with HIV-AIDS and presentation of a primary lymphoma of the spleenIn this report we present the case of a 52-year-old male patient with HIV infection without treatment presented with abdominal pain, fever and a 2-month weight loss. The pre-operative computed tomography showed splenomegaly and hypodense lesions suspected to be tumoral. He was operated on, performing an exploratory laparotomy, performing an splenectomy. The histopathological study reported a non-Hodgkin lymphoma.Primary splenic lymphoma is a non frequent entity. It is necessary to be acquainted with it in order to suspect it.

Primary splenic lymphomas involve the spleen and/or its lymph nodes. They represent <1% of lymphomas and they imply a diagnostic challenge due to its poor symptomatology and low prevalence.There for, the objects this work describe the management and evolution of a patient with HIV-AIDS and presentation of a primary lymphoma of the spleenIn this report we present the case of a 52-year-old male patient with HIV infection without treatment presented with abdominal pain, fever and a 2-month weight loss. The pre-operative computed tomography showed splenomegaly and hypodense lesions suspected to be tumoral. He was operated on, performing an exploratory laparotomy, performing an splenectomy. The histopathological study reported a non-Hodgkin lymphoma.Primary splenic lymphoma is a non frequent entity. It is necessary to be acquainted with it in order to suspect it.

Hemangioma esplénico / Splenic Hemangioma

RESUMEN Los tumores primitivos del bazo son relativamente raros, y entre ellos, los hemangiomas constituyen la neoplasia benigna más común en los adultos. Se presenta un paciente de 22 años de edad, piel blanca, con antecedentes de hipertensión arterial. El paciente refirió molestia en el hipocondrio izquierdo y en el ultrasonido abdominal se detectó esplenomegalia y litiasis renal. Ante el planteamiento de hiperesplenismo se indicó esplenectomía diagnóstica y terapéutica. La principal y más importante complicación que puede originar esta afección es la rotura espontánea del bazo en la cuarta parte de los casos informados, habitualmente en los de gran tamaño. El tratamiento de elección es la esplenectomía, principalmente para evitar el riesgo de rotura esplénica.

ABSTRACT Primitive tumours of the spleen are relatively uncommon, and among them, hemangiomas are the most common benign neoplasm in adults. We present a 22-year-old white-skinned patient with a history of high blood pressure. The patient reported discomfort in the left hypochondrium and abdominal ultrasound detected splenomegaly and renal lithiasis. Diagnostic and therapeutic splenectomy was indicated given the medical approach of hypersplenism. The main and most important complication that can originate this condition is the spontaneous rupture of the spleen in a quarter of the reported cases, usually in the large ones. The treatment of choice is splenectomy, mainly to avoid the risk of splenic rupture.

Bazo metastásico de un adenocarcinoma de endometrio. Presentación de un caso / Splenic metastasis from endometrial adenocarcinoma. A case presentation

Introducción: La metástasis esplénica de un carcinoma endometrial es un acontecimiento clínico raro, con solo 13 casos documentados en la literatura, revisada. La evolución de esta metástasis, en una paciente atendida, en nuestra Institución, fue el motivo para publicar este trabajo. Hay otras enfermedades oncológicas que en su evolución de progresión o recaída cursan con este tipo de cuadro clínico donde el tratamiento quirúrgico es fundamental y así complementar con tratamiento de quimioterapia. Se hizo una revisión en publicaciones cubanas no se encontró reporte del tema. Objetivo: Presentar un caso con un adenocarcinoma de endometrio tipo endometroide que metastiza al bazo con histología de células claras. Presentación: Paciente de 45 años, con diagnóstico de adenocarcinoma de endometrio con estadiamiento quirúrgico pT3A Nx Mo etapa IIIA Grado 2; este estadiamiento es el anterior a 2009, llevó su tratamiento quirúrgico y radioterapia complementaria, controlada por 21 meses. En consulta de seguimiento se diagnostica metástasis al bazo, por lo que se realiza esplenectomía y es tratada con quimioterapia; fue atendida por el equipo multidisciplinario de ginecología oncológica; las investigaciones realizadas estuvieron basadas en inmuhistoquimica, imageneología y tratamiento de soporte cuando lo necesitó. Conclusiones: La metástasis esplénica por un cáncer de endometrio es rara, es el primero reportado en Cuba, los estudios inmuhistoquímicos y de imágenes son fundamentales(AU)

Introduction: Splenic metastasis from endometrial carcinoma is a rare clinical event with only 13 documented cases in the literature reviewed. The evolution of a patient with this metastasis attended in our institution was the reason that motivated us to publish this work. There are other oncological diseases that are accompanied by this clinical picture during their evolution of progression where surgical treatment complemented with chemotherapy treatment is essential. A literature review was carried out in Cuban publications, but no reports on the topic were found. Objective: The aim of this work is to present an endometroid type case endometrium adenocarcinoma which metastasizes to the spleen with clear cell histology. Case presentation: Forty-five-year-old patient with diagnosis of endometrium adenocarcinoma with surgical stage pT3a Nx Mo stage IIIA Grade 2; this quantification was defined before 2009. The patient underwent surgical treatment which was complemented with radiotherapy and then followed for 21 months. In the follow-up consultation, spleen metastasis was diagnosed; so she underwent splenectomy and was treated with chemotherapy. She was treated by the multidisciplinary gynecologic oncology team; the investigations performed were based on immuhistochemistry, imaging, and supportive treatment whenever needed. Conclusions: Splenic metastasis from endometrial cancer is rare; it is the first case reported in Cuba. Immuhistochemical and imaging studies are essential(AU)

Status and progress in the treatment for locally resectable progressive gastric cancer and metastatic gastric cancer / 中南大学学报(医学版)

For resectable gastric cancer, although radical surgery is still the main treatment, methods of operation and the curative effect of operation are still in the stage of exploration for metastatic gastric cancer. Radiotherapy, chemotherapy and molecular targeted therapy also play an important role in prolonging the survival period of patients with gastric cancer. Postoperative radiotherapy and chemotherapy can prolong the survival time, but for patients with locally advanced gastric cancer, the preoperative radiotherapy and chemotherapy can also further improve the survival period of patients compared with direct operation. In addition, with the development and using of molecular targeted drugs, such as antiangiogenic agents, immunosuppressive drugs and so on, the survival period of patients with gastric cancer has been further extended.

The prognostic significance of POD24 in 106 cases with splenic marginal lymphoma with bone marrow invasion / 中华血液学杂志

Objective: To explore the effect of progression of disease within 24 months (POD24) on overall survival (OS) of splenic marginal lymphoma (SMZL) with bone marrow invasion, and to compare the clinical characteristics between POD24 SMZL with non-POD24 SMZL patients. Methods: The SMZL patients with bone marrow invasions were retrospectively analyzed between January 2002 and January 2017 treated in our institute, and the patients with sufficient follow-up time to judge POD24 were evaluated the clinical characteristics and prognosis, patients who died of non-progressive factors were excluded. Results: 106 patients were enrolled with a median age of 57 (25-79) years old. ①Clinical characteristics: All patients presented with bone marrow invasion and splenomegaly, 59.4% (63/106) with huge spleen, 14.8% (15/101) with hepatomegaly. Complex karyotype were found in 22.7% (18/79) patients; 13q deletion, 11q (ATM) deletion, 17p (TP53) deletion, and CEP12 abnormality patients presented with the percentage of 5.1% (4/78) , 1.3% (1/72) , 2.5% (2/80) , and 7.5% (4/53) , respectively.②Survival analysis: Univariate analysis showed that POD24, HGB less than 100 g/L and FISH detection of trisomy 12 were poor prognostic factors of OS. Multivariate analysis showed that only POD24 had independent prognostic significance[HR=20.116 (95%CI 2.226-181.820) , P=0.008]. ③Subgroup features: Patients with POD24 had significantly higher rates of mediastinal lymphadenopathy (63.6%vs 18.9%, P=0.005) and complex karyotype (50.0%vs 17.9%, P=0.024) than those without POD24. While the incidence of abdominal lymphadenopathy, anemia, thrombocytopenia, the lower albumin, and the increasing lactate dehydrogenase were higher in POD24 patients, but with no statistically difference. Conclusion: POD24 is an independent prognostic factor of the OS in SMZL. SMZL patients with mediastinal lymphadenopathy and complex karyotypes when diagnosed have a higher risk of POD24.

Lesions in 224 spleens of splenectomized dogs and evalution of alternative techniques for previous microscopic diagnosis / Lesões em 224 baços de cães esplenectomizados e avaliação de técnicas alternativas para diagnóstico microscópico prévio

When detecting a proliferative splenic lesion, veterinarians usually choose splenectomy before a conclusive diagnosis, which can provide a deleterious effect to the dog. The most appropriate would be to perform splenectomy as a therapeutic procedure only in cases with real surgical indication, which can be established after defining microscopic diagnosis and prognosis. The objectives of this study were: to determine the frequency of different types of lesions in spleens of splenectomized dogs in the period of 12 years (2006-2017); determine the representativity of neoplastic lesions (benign and malignant) and non-neoplastic lesions; to evaluate and compare the safety and efficiency of fine needle aspiration biopsy (FNA) and ultrasound-guided Tru-cut needle biopsy for cytological and histopathological diagnosis, respectively, of splenic nodular lesions. In the studied period 224 cases of lesions were found in splenectomized spleens. The frequency of non-neoplastic lesions (50,45%, 113/224) and neoplastic lesions (49,55%, 111/224) was very similar. Among the neoplastic lesions, the malignant ones were more frequent (79,27%, 88/111), and the hemangiosarcoma was the most common (52,25%, 58/111). The possibility of malignant neoplasm was about 74% greater than a benign one. In summary, it was verified that 60.71% (136/224) of the cases corresponded to benign lesions without indication to splenectomy. FNA techniques and Tru-cut biopsy showed a low risk of complications. Regarding the diagnostic efficacy, the FNA obtained 71.43% (15/21) of conclusive diagnoses, 60% (9/15) of which were compatible with the final result of the histopathological evaluation, after splenectomy (gold standard). The Tru-cut biopsy obtained 71.43% (5/7) of conclusive diagnoses and 28.57% (2/7) of inconclusive diagnoses. Among the conclusive one, in 100% of the cases the diagnosis was compatible with the gold standard. Thus, since the possibility of benign splenic lesions in dogs is 1.52 times greater than malignancies, splenectomy should be recommended as a therapeutic procedure only in cases with proven surgical indication, which can be established after definition of microscopic diagnosis and prognosis. The use of FNA and Tru-cut biopsy should be recommended, especially for small and focal splenic lesions, since such techniques are good alternatives for establishing diagnosis previously to splenectomy, which may reduce the number of unnecessary splenectomies. The importance of recommending such techniques is emphasized, especially for dogs with focal splenic lesions smaller than three centimeters.(AU)

Rotineiramente, frente à detecção de uma lesão proliferativa esplênica, médicos veterinários costumam optar pela esplenectomia antes de se obter o diagnóstico definitivo da lesão esplênica, o que pode trazer consequências deletérias ao cão. O mais apropriado seria, contudo, a sua adoção, como medida terapêutica, apenas nos casos com comprovada indicação cirúrgica, o que pode ser estabelecido após o diagnóstico microscópico e prognóstico definidos. Os objetivos desse estudo foram: determinar a frequência dos diferentes tipos de lesão em baços de cães esplenectomizados no período de 12 anos (2006-2017); determinar a representatividade das lesões neoplásicas (benignas e malignas) e não neoplásicas; avaliar e comparar a segurança e a eficiência da punção aspirativa por agulha fina (PAAF) e da biópsia por agulha Tru-cut guiadas por ultrassom para o diagnóstico citológico e histopatológico, respectivamente, de lesões nodulares esplênicas. No período estudado foram encontrados 224 casos de lesões em baços esplenectomizados. A frequência de lesões não neoplásicas (50,45%, 113/224) e neoplásicas (49,55%, 111/224) esplênicas foi muito semelhante. Entre as neoplásicas, as de caráter maligno foram mais frequentes (79,27%, 88/111) e o hemangiossarcoma o mais comum (52,25%, 58/111). A possibilidade de ocorrência de uma neoplasia maligna foi cerca de 74% maior do que uma benigna. Em suma, verificou-se que em 60,71% (136/224) dos casos estudados, tratava-se de lesões benignas que não teriam indicação de esplenectomia. As técnicas de PAAF e a biópsia por agulha Tru-cut demonstraram ter baixo risco de complicações. Quanto à eficácia diagnóstica, pela PAAF obteve-se 71,43% (15/21) de diagnósticos conclusivos, sendo 60% (9/15) desses compatíveis com o resultado final realizado pela avaliação histopatológica, após a esplenectomia (padrão ouro). Já a biópsia por Tru-cut obteve 71,43% (5/7) de diagnósticos conclusivos e 28,57% (2/7) de inconclusivos. Dentre os conclusivos, em 100% dos casos o diagnóstico foi compatível com o padrão ouro. Desta maneira, visto a possibilidade de ocorrência de lesões esplênicas benignas em cães serem 1,52 vezes maior do que as malignas deve ser recomentado a adoção de esplenectomia, como medida terapêutica, apenas nos casos com comprovada indicação cirúrgica, o que pode ser estabelecido após o diagnóstico microscópico e prognóstico definidos. O uso da PAAF e da biópsia por Tru-cut deve ser recomentado, sobretudo, para lesões esplênicas pequenas e focais, uma vez que tais técnicas representam boas alternativas para o estabelecimento do diagnóstico prévio a esplenectomia, o que pode reduzir o número de esplenectomias desnecessárias. Enfatiza-se a importância da recomendação de tais técnicas, sobretudo para cães com lesões esplênicas focais menores do que três centímetros.(AU)

Metastasis esplénica de un adenocarcinoma de colon derecho / Splenic metastasis of a right colon adenocarcinoma

Introducción: Las metástasis únicas en bazo de origen colorrectal son extremadamente infrecuentes. Sin embargo, debido el control postoperatorio más estricto de la patología oncológica con estudios por imágenes modernos, la incidencia ha ido aumentando en los últimos años. Objetivo: Exponer el caso de una paciente con una metástasis esplénica de un tumor colónico primario. Caso clínico: Paciente de sexo femenino, de 85 años con antecedentes de colectomía derecha laparoscópica. La anatomía patológica informó: adenocarcinoma de colon T2 N0, sin requerir tratamiento quimioterápico adyuvante. En el seguimiento postoperatorio, durante el segundo año intercurrió con aumento del antígeno carcinoembrionario (CEA). Se realizó PET-TC que evidenció nódulo hipercaptante único de 3 cm. en bazo. Se programó la esplenectomía laparoscópica, evolucionando satisfactoriamente. La anatomía patológica definitiva informó adenocarcinoma metastásico de origen colónico. Conclusión: La metástasis esplénica de un tumor colónico es extremadamente infrecuente, existiendo solamente casos aislados en la literatura. En pacientes sin otro compromiso sistémico la esplenectomía debe recomendarse. (AU)

Introduction: Isolated splenic metastases are highly uncommon. The incidence has been increasing during the last years due to better medical imaging and long term follow up. Objective: We report a case of a patient who developed isolated splenic metastasis from right colon cancer. Case presentation: Female, 85 years old who underwent a laparoscopic right colectomy. The pathologic investigation confirmed: colon adenocarcinoma staged as T2 N0, without need of oncologic adjuvant therapy. In the postoperative follow up, during the second year, an increased serum carcinoembryonic antigen level was noticed. A PET TC was carried out and demonstrated a 3 cm focus of high radiotracer uptake in the spleen. Laparoscopic splenectomy was performed, postoperative course was uneventful. Pathologic exam demonstrated splenic parenquima invaded with adenocarcinoma originated in colon. Conclusion: Isolated splenic metastases is a very rare clinical entity. Only few case reports can be found in the literature. If they are no other sites of disease, splenectomy should be the optimal approach.

Hemangioma esplênico no adulto associado à síndrome de Kasabach-Merritt / Kasabach-Merritt Syndrome-associated adult splenic hemangioma

O hemangioma esplênico, tumor benigno mais frequente do baço, porém, por vezes de difícil diagnóstico, pode integrar a síndrome de Kasabach-Merritt, afecção rara e potencialmente fatal, descrita como associação entre hemangioma, trombocitopenia e coagulopatia de consumo. Apresentamos um caso de paciente de 73 anos em acompanhamento com a hematologia por trombocitopenia crônica a princípio idiopática, que evoluiu com dor abdominal, anemia e derrame pleural. Foi realizada tomografia computadorizada de abdome, que evidenciou massa esplênica sólida heterogênea. A paciente foi submetida a esplenectomia total videolaparoscópica e evoluiu com melhora da dor abdominal e normalização da série plaquetária, sem recorrência do derrame pleural após o procedimento. A raridade e a complexidade do caso, somadas à dificuldade diagnóstica e à abordagem terapêutica, foram fatores que corroboraram para a apresentação desse caso. (AU)

Splenic hemangioma, the most common, but sometimes hard to diagnose, benign tumor of the spleen may integrate Kasabach- Merritt syndrome, a rare and potentially fatal condition described as an association ofhemangioma, thrombocytopenia and consumption coagulopathy. We present a case of a 73-year-old female patient being monitoring, with Hematology due to chronic idiopathic thrombocytopenia who progressed with abdominal pain, anemia and pleural effusion; a computed tomography scan of the abdomen was performed, showing a heterogeneous solid splenic mass. The patient underwent total videolaparoscopic splenectomy and progressed with improvement of abdominal pain and normalization of the platelet series, with no recurrence of pleural effusion after the procedure. The rarity and complexity of the case added to the diagnostic difficulty and therapeutic approach were factors that corroborated for the presentation of this case. (AU)

Metástasis solitaria intraparenquimatosa del bazo estudios clínicos patológicos e inmunohistoquímico / Intraparenchymal solitary spleen metastasis pathological and immunohistochemical clinical studies

Las metástasis esplénicas intra-parenquimatosas de tumores sólidos son tumores muy raros, y si se trata de nódulos únicos, son excepcionales. Presentamos dos casos de metástasis intra-parenquimatosas. El primer caso se trató de una mujer de 66 años con antecedentes de un adenocarcinoma endometrial. El segundo caso se trató de un paciente de sexo masculino con antecedente de un adenocarcinoma prostático, quien se presentó con un adenocarcinoma mucinoso en el bazo, como segunda neoplasia. También realizamos una revisión de los registros de biopsias de 14 años, del servicio de anatomía patológica del instituto(AU)

The spleen parenchymal metastasis of the solid tumors, are very rare, but the isolated metastasis to the spleen are considered exceptional. We herein present two cases of the isolated splenic metastases. The first case was a female a 66 year old female with a diagnosis endometrial adenocarcinoma. The second cases was a 71 years old male, with a history of a prostatic adenocarcinoma, and a spleen metastasis of a ucinous adenocarcinoma, as a second malignancy. We also made a review of 14 years, biopsies records from the pathology service, of the institute(AU)

Hemangioendotelioma esplénico en paciente pediátrico. Presentación de caso / Splenic Hemangioendothelioma in Pediatric Patient. Case Report

Los hemangioendoteliomas esplénicos son neoplasias de origen vascular poco frecuentes, se presentan en pacientes entre 3 y 54 años. En edad pediátrica se han descrito pocos casos. Se caracteriza por presentar lesiones bien circunscritas, debidamente separadas del parénquima esplénico sano, moderada atipia celular, poca actividad mitótica y una apariencia histológica intermedia entre el hemangioma y el angiosarcoma.

Splenic hemangioendothelioma are rare vascular neoplasms that occur in patients between the ages of 3 and 54. Few pediatric cases have been described. It is characterized by well-circumscribed lesions, properly separated from the healthy splenic parenchyma, moderate cellular atypia, little mitotic activity, and an intermediate histological appearance between hemangioma and angiosarcoma.

Histopathological and immunophenotypical assessment of canine primary splenic lymphoma according to the World Health Organization / Avaliação histopatológica e imunofenotípica segundo a classificação da Organização Mundial da Saúde do linfoma esplênico primário em cães

Although there are several studies addressing multicentric lymphoma in dogs, data regarding splenic lymphoma remains scarce. The diagnosis of splenic lymphoma using the World Health Organization (WHO) classification system can aid prognostic characterization of splenic lymphoma. The aim of this study was to evaluate the most common histological types of splenic lymphoma in dogs from Brazil according to the WHO classification. We assessed 33 cases of splenic lymphoma diagnosed by histopathologic and immunohistochemical (IHC) analysis submitted to VETPAT- Pathology Laboratory, Campinas-SP, Brazil. IHC was performed using antibodies against CD3 for T-cell and CD79α for B-cell identification . Mean age of patients with splenic lymphoma was 9.8 years. The most affected breeds were mixed breed dogs (33%) followed by Pit bulls and Yorkshires (9.0%). The most prevalent histological type was marginal zone B-cell lymphoma (60.7%) followed by diffuse large B-cell lymphoma (12.1%) and lymphoblastic T-cell lymphoma (12.1%). Histological and immunohistochemical characterization of splenic lymphoma is important due to the high prevalence of indolent lymphomas such as marginal zone, which may be less aggressive and thus have different prognostic and distinct forms of treatment when compared to high-grade lymphomas.(AU)

Embora existam diversos estudos a respeito do linfoma multicêntrico em cães, os dados sobre linfoma esplênico primário são escassos. O diagnóstico do linfoma esplênico utilizando a classificação da Organização Mundial da Saúde (OMS) pode melhorar a caracterização da doença. O objetivo do estudo foi avaliar os principais tipos de linfoma esplênico primário em cães no Brasil de acordo com a classificação da OMS. Foram avaliados 33 casos de linfoma esplênico diagnosticados por histopatologia e imuno-histoquímica submetidos ao Laboratório de Patologia Veterinária (VETPAT, Campinas/SP). A imuno-histoquímica foi realizada utilizando os anticorpos CD3 para linfomas T, CD79α para linfomas B. A média de idade dos pacientes com linfoma esplênico foi de 9,8 anos. Os animais sem raça definida (SRD) foram os mais acometidos (33%) seguidos de PitBulls e Yorkshire (9,0%). O tipo histológico mais comum foi o linfoma de zona marginal representando 60,7% dos casos seguido do linfoma difuso de grandes células B (12,1%) e linfoma linfoblástico T (12,1%). A caracterização histopatológica e imuno-histoquímica do linfoma esplênico é importante devido à alta prevalência de linfomas indolentes como o linfoma de zona marginal, que devido ao seu comportamento indolente apresenta prognóstico e tratamento distintos quando comparado aos linfomas de alto grau.(AU)

Linfoma no Hodgkin folicular primario del bazo / Follicular Non-Hodgkin Primary Splenic Lymphoma

Se presenta el caso de una paciente de 79 años que desde hace 3 meses presenta marcada sudoración nocturna, dolor abdominal en zona de hipocondrio y flanco izquierdo y ligera pérdida de peso. Al examen físico se observa palidez de piel y mucosas, esplenomegalia que rebasa 7 cm el reborde costal izquierdo, superficie lisa, superficie regular. El resto del examen físico fue normal. Se realizan múltiples investigaciones básicas sin poderse identificar el diagnostico. Por la presencia de gran esplenomegalia y la pancitopenia se decide realizar esplenectomía confirmándose un linfoma no Hogkin folicular primario del bazo(AU)

We present the case of a 79-year-old patient who had marked night sweats, abdominal pain in the hypochondrium area and left flank and slight weight loss for 3 months. Physical examination showed pale skin and mucous membranes, splenomegaly that exceeds the left costal margin by 7 cm, smooth surface, and regular surface. The rest of the physical examination was normal. Multiple basic studies were conducted out but the diagnosis was not identified. Due to the presence of great splenomegaly and pancytopenia, it was decided to perform splenectomy, confirming a follicular non-Hodgkin primary splenic lymphoma(AU)

Splenic marginal zone lymphoma: a literature review of diagnostic and therapeutic challenges

ABSTRACT Splenic marginal zone lymphoma (SMZL) is a low-grade B-cell non-Hodgkin's lymphoma characterized by massive splenomegaly, moderate lymphocytosis with or without villous lymphocytes, rare involvement of peripheral lymph nodes and indolent clinical course. As a rare disease, with no randomized prospective trials, there is no standard of care for SMZL so far. Splenectomy has been done for many years as an attempt to control disease, but nowadays it has not been encouraged as first line because of new advances in therapy as rituximab, that are as effective with minimal toxicity. Facing these controversies, this review highlights advances in the literature regarding diagnosis, prognostic factors, treatment indications and therapeutic options.

Inflammatory pseudotumor-like follicular dendritic cell tumor: an underdiagnosed neoplasia

Follicular dendritic cell (FDC) tumor is an uncommon neoplasm. It generally presents as a slow-growing, painless mass, without systemic symptoms. Histological features usually include low grade spindle cell proliferation. This tumor occurs primarily in lymph nodes, especially cervical and axillary, however, involvement of extranodal sites such as the tonsils, spleen, liver, and gastrointestinal tract has been reported. Inflammatory pseudotumor-like follicular dendritic cell tumor (IPT-like FDCT) is a rare, distinctive histological subtype of this low-grade malignant neoplasm, with consistent Epstein-Barr virus (EBV) association. The differential diagnosis with other fibro-inflammatory tumor proliferations, as inflammatory pseudotumor (IPT) and inflammatory myofibroblastic tumor (IMT), may be challenging. In the present article, two cases of IPT-like FDCT of the spleen are presented, with a broad overview of the literature: one 77-year-old male and one 70-year-old female. A large immunohistochemical panel should be used for diagnosis, as no single specific and totally sensitive markers are available, including markers for CD21, CD23, CD35, CNA42, and clusterin. Individual cases may express one or more of these markers, so that all of them should be investigated. In situ hybridization for EBV is constantly positive. Immunostaining for ALK should be negative, as it is present in roughly half of the cases of IMT. This panel should be used in combination of clinical, laboratory, and topographic evidences. Importantly, inclusion of this lesion as a possible option in clinical and pathological investigation represents the basis for a correct diagnosis (AU)

Splenic diffuse red-pulp small B-cell lymphoma associated with hepatitis B virus: a report of two cases / Linfoma esplênico difuso da polpa vermelha, de linfócitos B pequenos, associado ao vírus da hepatite B: relato de dois casos

ABSTRACT CONTEXT: Splenic diffuse red-pulp small B-cell lymphoma is a rare disease, representing less than 1% of all non-Hodgkin lymphomas (NHL). This entity is characterized by involvement of bone marrow sinusoids and peripheral blood. The majority of cases are at an advanced stage when diagnosed. Its pathogenesis is still poorly understood. CASE REPORTS: We report on two patients with chronic non-replicating hepatitis B virus (HBV) who developed splenic diffuse red-pulp small B-cell lymphoma. Both of them were in stage IV at diagnosis and evolved with aggressive disease. Both of them achieved a complete response through chemotherapy, but one of them died due to infectious complications during bone marrow transplantation. The other decided not to undergo transplantation and continues not to show any evidence of disease today (three years after treatment). Some studies have shown a possible association between B-cell NHL and HBV. Nonetheless, the mechanism through which this oncogenic virus interacts with B-cell NHL is still poorly understood. HBV is lymphotropic and may insert into the host's genome, thus causing overexpression of oncogenes and downregulation of tumor suppressor genes. Therefore, chronic stimulation by HBV can increase B-cell proliferation, which promotes monoclonal expansion of these cells and results in malignancy. CONCLUSION: HBV may be implicated in the pathogenesis of this lymphoma, although no direct association between these two entities could be proved in the present study. Further investigations are necessary.

RESUMO CONTEXTO: Linfoma esplênico difuso da polpa vermelha, de linfócitos B pequenos, é uma doença rara, representando menos do que 1% de todos os linfomas não Hodgkin. Essa entidade é caracterizada por envolvimento de sinusoides da medula óssea e sangue periférico. A maioria dos casos está em estádio avançado ao diagnóstico. Sua patogênese ainda é pouco compreendida. RELATOS DE CASOS: Reportamos dois pacientes com vírus da hepatite B (HBV) crônica não replicante que desenvolveram linfoma esplênico difuso da polpa vermelha, de linfócitos B pequenos. Ambos estavam em estádio IV ao diagnóstico e evoluíram com doença agressiva. Ambos alcançaram resposta completa com a quimioterapia, porém um deles evoluiu a óbito por intercorrências infecciosas durante o transplante de medula óssea e o outro optou por não realizar o transplante e encontra-se sem evidência de doença até os dias atuais (três anos após tratamento). Alguns estudos demonstraram a possível associação entre linfomas não Hodgkin B e HBV. Entretanto, o mecanismo pelo qual esse vírus oncogênico interage com linfoma não Hodgkin B ainda é pouco compreendido. HBV é linfotrópico e pode se inserir no genoma do receptor, causando superexpressão de oncogenes e downregulation de genes supressores tumorais. Portanto, o estímulo crônico pelo HBV pode aumentar a proliferação de células B, promovendo expansão monoclonal dessas células, resultando em malignidade. CONCLUSÃO: HBV pode estar implicado na patogênese desse linfoma, entretanto, uma associação direta entre essas duas entidades não pôde ser provada no presente estudo e investigações adicionais são necessárias.