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.

Effects of lung cancer cell-associated B7-H1 on T-cell proliferation in vitro and in vivo

B7 homolog 1 (B7-H1) is the most potent immunoinhibitory molecule in the B7 family. In this study, we examined the effects of tumor-associated B7-H1 on T-cell proliferation in lung cancer. The expression of B7-H1 in human adenocarcinoma A549 and mouse Lewis lung carcinoma (LLC) cells were examined by flow cytometry. To assess the in vitro effect of tumor-associated B7-H1 on T-cell proliferation, we isolated T cells from peripheral blood mononuclear cells (PBMCs) of healthy individuals, labeled them with carboxyfluorescein succinimidyl ester, and co-cultured them with A549 cells in the absence or presence of anti-B7-H1 antibody. For in vivo analysis, LLC cells were subcutaneously injected into mice treated or not with anti-B7-H1 antibody. T-cell proliferation in both in vitro and in vivo assays was analyzed by flow cytometry. In vitro, co-culturing T cells with A549 cells significantly inhibited the proliferation of the former compared with the proliferation of T cells alone (P<0.01), and the addition of B7-H1 blocking antibody dramatically reversed the inhibition of T-cell proliferation by A549 cells. Similarly, in mice bearing LLC-derived xenograft tumors, in vivo administration of anti-B7-H1 antibody significantly increased the total number of spleen and tumor T cells compared to levels in control mice that did not receive anti-B7-H1 antibody. Functionally, in vivo administration of anti-B7-H1 antibody markedly reduced tumor growth. Tumor-associated B7-H1 may facilitate immune evasion by inhibiting T-cell proliferation. Targeting of this mechanism offers a promising therapy for cancer immunotherapy.

Distal pancreatectomy with splenectomy for the management of splenic hilum metastasis in cytoreductive surgery of epithelial ovarian cancer / 부인종양

OBJECTIVE: Distal pancreatectomy with splenectomy may be required for optimal cytoreductive surgery in patients with epithelial ovarian cancer (EOC) metastasized to splenic hilum. This study evaluates the morbidity and treatment outcomes of the uncommon procedure in the management of advanced or recurrent EOC. METHODS: This study recruited 18 patients who underwent distal pancreatectomy with splenectomy during cytoreductive surgery of EOC. Their clinicopathological characteristics and follow-up data were retrospectively analyzed. RESULTS: All tumors were confirmed as high-grade serous carcinomas. The median diameter of metastatic tumors located in splenic hilum was 3.5 cm (range, 1 to 10 cm). Optimal cytoreduction was achieved in all patients. Eight patients (44.4%) suffered from postoperative complications. The morbidity associated with distal pancreatectomy and splenectomy included pancreatic leakage (22.2%), encapsulated effusion in the left upper quadrant (11.1%), intra-abdominal infection (11.1%), pleural effusion with or without pulmonary atelectasis (11.1%), intestinal obstruction (5.6%), pneumonia (5.6%), postoperative hemorrhage (5.6%), and pancreatic pseudocyst (5.6%). There was no perioperative mortality. The majority of complications were treated successfully with conservative management. During the median follow-up duration of 25 months, nine patients experienced recurrence, and three patients died of the disease. The 2-year progression-free survival and overall survival were 40.2% and 84.8%, respectively. CONCLUSION: The inclusion of distal pancreatectomy with splenectomy as part of cytoreduction for the management of ovarian cancer was associated with high morbidity; however, the majority of complications could be managed with conservative therapy.

Linfoma esplénico de zona marginal como causa de esplenomegalia masiva: reporte de caso clínico y revisión de la literatura / Splenic marginal zone lymphoma as cause for massive splenomegaly: a clinical case and literature review

Massive splenomegaly is in the which the growth of the spleen has spread to other quadrants of the abdomen. It is produced by a limited number of pathologies, both benign and malignant. It is presented a case of a 62 year-old woman who is consulting for four years of progressive increase in her abdominal volume, associated to the feeling of abdominal fullness, dyspnea on moderate exertion and lower extremities edema. At the physical examination was observed massive splenomegaly and jaundice. The hemogram showed pancytopenia and a lymphocyte count of80 percent. The myelogram revealed marrow infiltration by lymphocytes of mature appearance. Flow cytometry of peripheral blood showed 70 percent of lymphocytes, which expressed B cells markers CD19, CD20, CD23and FMC7 in addition to Kappa light chain restriction, suggesting marginal splenic zone lymphoma. The bone marrow biopsy showed lymphoid small cells infiltrate with positive markers CD20, CD5,CD23 and negative cyclin D1 study. BCL-2 was also positive. It was considered unfit to receive chemotherapy and was treated with 4 cycles of rituximab, with significant decrease of splenic size.

Utilidad y complicaciones de la biopsia percutánea esplénica con aguja tru-cut guiada por imágenes / Ultrasound guided needle biopsy of the spleen: review of 13 procedures

Background: Needle biopsies of the spleen were avoided due to the fear of bleeding in a highly vascularized organ. However their safety, even using 18 gauge needles, has been demonstrated. Aim: To report the experience with ultrasound guided needle biopsies of the spleen. Material and Methods: Retrospective review of records of ultrasound guided biopsies of the spleen using Tru-cutTM needles, performed between 2005 and 2009. Results: Thirteen procedures performed in 12 patients were identified. A specific diagnosis was achieved in nine (69 percent) procedures (lymphoma in four, melanoma in 2, sarcoma in 1, extremedullary erythropoiesis in one and splenic cryptococcosis in one. Two patients with negative results were subjected to a new biopsy, which yielded the diagnosis of lymphoma. A third patient was studied elsewhere, finding a malignant tumor. Two patients had complications, one had a vagal reaction and other had a perisplenic hematoma without clinical repercussion. Conclusions: Ultrasound guided needle biopsy of the spleen is a safe and useful procedure.

Objetivo: Reportar la experiencia de biopsias percutáneas esplénicas con aguja tru-cut guiadas por imágenes. Materiales y Métodos: Revisión retrospectiva de biopsias esplénicas con aguja tru-cut guiadas por ultrasonido (US) y tomografía computada (TC) realizadas en nuestro hospital desde Enero de 2005 a Abril de 2009. Resultados: Se identificaron un total de 13 procedimientos. La biopsia percutánea logró un diagnóstico específico en 9 (69 por ciento) de las 13 intervenciones. Los diagnósticos fueron linfoma (n = 4), melanoma (n = 2), sarcoma (n = 1), hematopoyesis extramedular (n = 1) y criptococosis esplénica (n = 1). De las biopsias no diagnósticas 3 casos correspondieron a patología neoplásica y uno a patología benigna. Se reportaron 2 complicaciones (15 por ciento). Discusión: La biopsia esplénica percutánea guiada por imágenes con aguja tru-cut es un procedimiento útil y seguro, capaz de determinar el diagnóstico definitivo en la mayoría de los pacientes y evitar la mayoría de las esplenectomías diagnósticas.

Quiste esplénico gigante con elevación del CA19-9: reporte de un caso / Giant splenic cyst with Ca-19-9 elevated: report of a case

Los quistes esplénicos verdaderos constituyen una rareza, es por ello que presentamos el caso de una paciente de 21 años de edad tratada por un quiste esplénico gigante (30 cm) con elevación del Ca 19-9 (1670U/mL) a quien se le realizó esplenectomía con evolución satisfactoria y sin complicaciones. La biopsia definitiva reportó quiste esplénico verdadero, siendo estas lesiones pocofrecuentes, más aun cuando cursan con elevación del Ca19-9, encontramos que se han publicado en la literatura consultada alrededor de 30 casos.

The true cyst spleen are a poor known pathology, for these reason we report the case of a 21-year-old woman with a giant true spleen cyst (30 cm) with a high CA 19-9 serum level (16 70 U/mL). The patient underwent splenectomy without complications and she has a successful postoperative course. The histopathological diagnosis was a true splenic cyst being these lesions very rare; approximately 30 cases of benign true splenic cysts with a high CA 19-9 serum level have been published in the literature.

CT and MRI Findings of Sclerosing Angiomatoid Nodular Transformation of the Spleen: Spoke Wheel Pattern

Sclerosing angiomatoid nodular transformation of the spleen is a recently described benign pathologic entity that is characterized by round shaped vascular spaces that are lined by endothelial cells, and the spaces are circumscribed by granulomatoid structures. Microscopically, all the reported cases had multiple angiomatoid nodules in a fibrosclerotic stroma. Each angiomatoid nodule was made up of slit-like, round or irregular shaped vascular spaces that were lined by endothelial cells and interspersed ovoid cells. We present here the CT and dynamic gadolinium-enhanced MR findings of a patient with sclerosing angiomatoid nodular transformation. The spoke-wheel pattern that was observed on MRI in this case may be an important imaging clue for making the correct diagnosis of this benign lesion.

Angiosarcoma primario del bazo: Caso clínico / Primary angiosarcoma of the spleen: Report of one case

Primary angiosarcoma of the spleen is rare and almost always fatal. The pathogenesis is unknown. It has an aggressive behavior and frequently presents with hematological abnormalities or metastatic disease. We report a 49 year-old male that presented with spleen and lymph node enlargement. He was subjected to a splenectomy and the biopsy disclosed an angiosarcoma of the spleen. Metastases were detected in the lung and bones and the patient was considered beyond any therapeutic option, dying fifteen months later.

Nódulos fibro-histiocíticos no baço de uma cadela: relato de caso / Splenic fibroistiocytic nodules in a bitch: case report

Descreve-se a ocorrência de múltiplos nódulos fibro-histiocíticos no baço de uma cadela Rottweiler, de sete anos. O diagnóstico foi feito por histopatologia e confirmado por imunoistoquímica positiva para ED1, CD18, vimentina e lisozima. O animal foi sacrificado três meses após o diagnóstico, por severo agravamento dos sinais clínicos.

Multiple fibrohistiocytic nodules in the spleen of a 7-year-old female Rottweiler were reported. The diagnosis was made by histology and immunohistochemistry; cells were positive for ED1, CD18, vimentin and lysozyme. The dog was euthanatized three months after the tumor was diagnosed, due to severe clinical signs.

Primary transitional cell carcinoma of spleen.

Transitional cell carcinoma arises from the lining of the urogenital tract and ovary. We report a 39-year-old lady with transitional cell carcinoma of the spleen that presented as a complex cystic lesion. Exhaustive search for another primary was negative. She is doing well 18 months after splenectomy was performed.

Comprometimento de orgãos sólidos abdominais por linfoma / Lymphoma of the solid abdominal viscera

Este estudo visa demonstrar as diversas formas de apresentação do comprometimento linfomatoso das vísceras sólidas abdominais. Realizamos estudo retrospectivo no Hospital São Paulo - Escola Paulista de Medicina e no Hospital e Maternidade São Luiz, selecionando casos de pacientes com comprometimento pelo linfoma de órgãos sólidos abdominais, como fígado, pâncreas, baço, rins, adrenais e ovários, nas mais diversas formas de apresentação. Bago e fígado são os órgãos sólidos mais freqüentemente acometidos, seguidos dos rins, pâncreas, adrenais, útero e ovários (raramente comprometidos). As formas de infiltração destes órgãos são: a) aumento volumétrico homogêneo; b) nódulos múltiplos; c) massa única, geralmente associada a linfonodomegalia intra e retroperitoneal. 0 conhecimento das diversas formas de infiltração linfomatosa extranodal abdominal permite suspeitar da sua presença, facilita o diagnóstico diferencial e aprimora o estadiamento da doença.

Primary angiosarcoma of the spleen--a case report.

Primary splenic angiosarcomas are rare neoplasms. They have aggressive behavior that frequently present with hematological abnormalities and metastatic disease. The diagnosis can only be made at surgery. Prognosis of this disease is poor. We present a rare case of primary splenic angiosarcoma with metastases in liver.

Epstein-Barr Virus-Associated Peripheral T-Cell Lymphoma involving Spleen in a Renal Transplant Patient

The incidence of posttransplantation lymphoproliferative disorders (PTLDs) has increased in recent years. Although rare, various types of T-cell lymphoma have been reported and their association with Epstein-Barr virus (EBV) has been compared with B-cell PTLDs. We report a case of splenic peripheral T-cell lymphoma occurring in a 47-yr-old male patient 7 yr after renal allograft transplantation. The spleen showed sinusoidal proliferation of focal CD30 positive, large, atypical lymphoid cells. Positivity for CD3 and cytolytic granule-associated proteins was also demonstrated in the tumor cells, while anaplastic large cell lymphoma kinase (ALK) and CD8 were not expressed. Strong nuclear signals for EBV mRNA were noted by EBER1 in situ hybridization. A molecular genetic study demonstrated a rearrangement of the gamma T-cell receptor gene. To our knowledge, this case is unique in terms of a posttransplant T-cell lymphoma that shows focal CD30, cytolytic granule-associated proteins, and EBV positivity.

Splenic haemangioma and Kasabach-Merritt Syndrome as differential diagnosis for abdominal mass in a newborn: case report

Apresentamos um caso raro de hemangioma esplênico em um recém-nascido do sexo feminino, apresentando-se como massa abdominal, coagulopatia e trombocitopenia. No ato operatório observou-se uma massa tumoral vascular do pólo inferior do baço. A paciente encontra-se em acompanhamento ambulatorial. O diagnóstico e as opções de tratamento foram revistas e discutidas. Os autores revisaram a literatura sobre hemangioma esplênico em recém-nascidos e observaram ser este o terceiro caso de associação entre hemangioma esplênico e Síndrome de Kasabach-Merritt. O hemangioma esplênico é uma doença rara no diagnóstico diferencial das massas abdominais em recém-nascidos. O hemangioma é a neoplasia benigna mais freqüente do baço. A anemia, a trombocitopenia e a coagulopatia são vistos com freqüência em hemangiomas cavernosos grandes associados à Síndrome de Kasabach-Merritt (KMS). O hemangioma cavernoso esplênico associado com esta síndrome é extremamente raro.

A case of epidermoid cyst of the intrapancreatic accessory spleen

A 54-year-old woman presented with a huge palpable mass on left upper quadrant of the abdomen. After preoperative work-up, a cystic disease of pancreatic tail or accessory spleen was initially suspected. We performed exploratory laparotomy and resected both the spleen and a 15 x 11 cm-sized huge cystic mass containing a part of solid component which extended continuously to the pancreatic tail. The solid component, comprising the upper portion of the resected cyst, was reddish brown and granular like as normal splenic tissue. The inner surface of the cyst was smooth and was filled with yellowish white material. Histologic examination showed an epidermoid cyst originating in the accessory spleen of the pancreatic tail lacking hair or skin appendages.