RESUMO
OBJECTIVES: The resection margin (RM) status and microscopic vascular invasion (MVI) are known prognostic factors for intrahepatic cholangiocarcinoma (ICC). An enhanced understanding of their impact on long-term prognosis is required to improve oncological outcomes. METHODS: A total of 711 consecutive patients who underwent curative liver resection for hepatitis B virus-related ICC were retrospectively analyzed. The different impact of the RM status (narrow, <1 cm, or wide, ≥1 cm) and MVI (positive, +, or negative, -) on overall survival (OS) and recurrence-free survival (RFS) were analyzed. RESULTS: The 1-, 3-, and 5-year OS rates were 67.6%, 42.5%, and 33.2% in wide RM & MVI (-), 58.0%, 36.1%, and 26.5% in narrow RM & MVI (-), 51.0%, 27.0%, and 24.3% in wide RM & MVI (+), and 39.0%, 20.4% and 14.3% in narrow RM & MVI (+) (p < 0.001). Multivariate analysis showed that RM & MVI were independent risk factors for the OS and RFS. CONCLUSION: Combined analysis of RM and MVI can better stratify the risks of postoperative death and recurrence in patients with HBV-related ICC, which may help subsequent adjuvant therapy and closer follow-up.
Assuntos
Neoplasias dos Ductos Biliares , Colangiocarcinoma , Vírus da Hepatite B , Neoplasias Vasculares , Adulto , Idoso , Idoso de 80 Anos ou mais , Neoplasias dos Ductos Biliares/mortalidade , Neoplasias dos Ductos Biliares/patologia , Neoplasias dos Ductos Biliares/cirurgia , Neoplasias dos Ductos Biliares/virologia , Colangiocarcinoma/mortalidade , Colangiocarcinoma/patologia , Colangiocarcinoma/cirurgia , Colangiocarcinoma/virologia , Feminino , Hepatectomia/mortalidade , Antígenos de Superfície da Hepatite B , Humanos , Metástase Linfática , Masculino , Margens de Excisão , Pessoa de Meia-Idade , Invasividade Neoplásica , Estudos Retrospectivos , Análise de Sobrevida , Neoplasias Vasculares/mortalidade , Neoplasias Vasculares/patologia , Neoplasias Vasculares/cirurgia , Neoplasias Vasculares/virologia , Adulto JovemRESUMO
Epstein-Barr virus-associated smooth muscle tumour (EBV-SMT) is a rare disease occurring in immunosuppressed patients, such as those with AIDS, post-transplantation immunodeficiency and congenital immunodeficiency. Intracranial EBV-SMT after solid organ transplantation has been reported. However, intracranial lesions after bone marrow transplantation are extremely rare. We report the case of a 47-year-old man with a history of acute myeloid leukaemia and bone marrow transplantation. He had symptoms of trigeminal neuralgia, and MRI revealed a left cavernous sinus tumour. He started taking oral gabapentin, but his symptoms did not improve. We performed transnasal endoscopic surgery. Postoperative MRI showed complete removal of the cavernous sinus lesion. Pathological examination showed spindle-shaped cells positive for smooth muscle markers and EBV-encoded small RNA in situ hybridisation. EBV-SMT was pathologically diagnosed. His symptoms improved after surgery. No tumour recurrence was noted on follow-up MRI after 15 months without adjuvant radiation or chemotherapy.
Assuntos
Endoscopia/métodos , Infecções por Vírus Epstein-Barr/diagnóstico , Herpesvirus Humano 4/genética , Hospedeiro Imunocomprometido , Neoplasias Vasculares/diagnóstico , Biópsia , Seio Cavernoso , Diagnóstico Diferencial , Infecções por Vírus Epstein-Barr/virologia , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , RNA Viral/análise , Ultrassonografia Doppler , Neoplasias Vasculares/cirurgia , Neoplasias Vasculares/virologiaRESUMO
BACKGROUND: Intravascular large B-cell lymphoma (IVLBCL) is an extremely rare subtype of diffuse large B-cell lymphoma that most commonly involves the central nervous system, skin, and bone marrow. To our knowledge, Epstein-Barr virus (EBV)-positive IVLBCL in the liver has never been reported in the literature. CASE PRESENTATION: We report a case of a 65-year-old Chinese man with complaint of fever for 18 days. No obvious abnormality was found by physical examination. Laboratory findings were notable for anemia, thrombocytopenia, and elevated level of serum lactate dehydrogenase. Bone marrow on smear, biopsy, and flow cytometry revealed no lymphoma. Imaging studies showed a slightly lower density lesion in the liver with high fluorodeoxyglucose uptake and hepatosplenomegaly. Percutaneous liver biopsy revealed clustering of large atypical lymphocytes within the hepatic sinusoids. Immunohistochemically, these lymphoma cells were positive for CD20, PAX-5, MUM-1, BCL-6 and CD5, but negative for CD3 and CD10. Besides, Epstein-Barr virus-encoded RNA was detected in tumor cells by in situ hybridization. BCL-2, BCL-6 and MYC genes were intact tested by fluorescence in situ hybridization analysis. The patient was diagnosed as IVLBCL and died after 1 month of hospitalization without receiving immunochemotherapy. CONCLUSIONS: IVLBCL of the liver is a highly rare lymphoma with nonspecific manifestations and dismal prognosis. Full recognition of its clinicopathological features will help to better diagnose this disease.
Assuntos
Capilares/patologia , Infecções por Vírus Epstein-Barr/complicações , Neoplasias Hepáticas/patologia , Linfoma Difuso de Grandes Células B/patologia , Neoplasias Vasculares/patologia , Idoso , Humanos , Neoplasias Hepáticas/virologia , Linfoma Difuso de Grandes Células B/virologia , Masculino , Neoplasias Vasculares/virologiaAssuntos
Rim/irrigação sanguínea , Linfoma Extranodal de Células T-NK/patologia , Neoplasias Vasculares/patologia , Idoso , Antígenos CD/análise , Infecções por Vírus Epstein-Barr/complicações , Herpesvirus Humano 4/isolamento & purificação , Humanos , Rim/patologia , Rim/virologia , Linfoma Extranodal de Células T-NK/virologia , Masculino , Neoplasias Vasculares/virologiaRESUMO
Intravascular lymphoma (IVL) is a rare entity. Most cases are a variant of extranodal diffuse large B cell lymphoma, and fewer than 10% of the published cases are of T cell origin. Only intravascular B cell lymphoma is recognized as a distinct entity in the most recent World Health Organization (WHO) classification of lymphoproliferative disorders. We describe a case of cutaneous natural killer (NK)/T IVL, with a cytotoxic immunophenotype and Epstein-Barr virus (EBV) positivity. However, our case was immunohistochemically negative not only for T cell receptor (TCR)-ßF1 and TCR-γ (TCR-silent), but also for CD56, making it the first triple-negative NK/T IVL case to be described. We urge recognition of this NK/T cell lineage intravascular lymphoma due to its particular immunophenotypical profile and its unvarying relationship with EBV. Its occurrence should not be considered a coincidence, but rather a key aspect of the pathogenic background of this haematological neoplasm.
Assuntos
Infecções por Vírus Epstein-Barr/complicações , Herpesvirus Humano 4/isolamento & purificação , Linfoma Cutâneo de Células T/classificação , Neoplasias Cutâneas/classificação , Neoplasias Vasculares/classificação , Idoso de 80 Anos ou mais , Infecções por Vírus Epstein-Barr/patologia , Infecções por Vírus Epstein-Barr/virologia , Humanos , Imunofenotipagem , Linfoma Cutâneo de Células T/patologia , Linfoma Cutâneo de Células T/virologia , Masculino , Células T Matadoras Naturais/patologia , Células T Matadoras Naturais/virologia , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/virologia , Neoplasias Vasculares/patologia , Neoplasias Vasculares/virologiaRESUMO
Intravascular selective growth of neoplastic B lymphocytes is a characteristic finding of intravascular large B-cell lymphoma (IVLBCL). However, because neoplastic B cells of IVLBCL grow merely in the lumina of capillaries or small vessels, primary IVLBCL of the great vessels is considered exceptional. To our knowledge, only 2 primary B-cell lymphomas in the lumina of the vena cava have been reported. However, there has been no report of primary B-cell lymphoma with intra-aortic growth. We describe a novel manifestation of primary Epstein-Barr virus-positive large B-cell lymphoma mainly affecting the lumina of the aorta and its major branches in a 76-year-old man. He had a long-term fever that was refractory to antibiotics and aortic mural thrombosis with visceral embolization. Because he had no detectable mass suggesting a malignancy, it was difficult to diagnose while he was alive. He died without anticancer treatment, and the confirmed diagnosis was made at autopsy.
Assuntos
Aorta/patologia , Linfoma Difuso de Grandes Células B/patologia , Neoplasias Vasculares/patologia , Idoso , Infecções por Vírus Epstein-Barr/complicações , Herpesvirus Humano 4 , Humanos , Linfoma Difuso de Grandes Células B/virologia , Masculino , Trombose/etiologia , Neoplasias Vasculares/virologiaRESUMO
Classical Kaposi sarcoma (KS) usually appears on lower extremities accompanied or preceded by local lymphedema. However, the development in areas of chronic lymphedema of the arms following mastectomy, mimicking a Stewart-Treves syndrome, has rarely been described. We report an 81-year-old woman who developed multiple, erythematous to purple tumors, located on areas of post mastectomy lymphedema. Histopathological examination evidenced several dermal nodules formed by spindle-shaped cells that delimitated slit-like vascular spaces with some red cell extravasation. Immunohistochemically, the human herpesvirus type 8 (HHV-8) latent nuclear antigen-1 was detected in the nuclei of most tumoral cells confirming the diagnosis of KS. Lymphedema could promote the development of certain tumors by altering immunocompetence. Although angiosarcoma (AS) is the most frequent neoplasia arising in the setting of chronic lymphedema, other tumors such as benign lymphangiomatous papules (BLAP) or KS can also develop in lymphedematous limbs. It is important to establish the difference between AS and KS because their prognosis and treatment are very different. Identification by immunohistochemistry of HHV-8 is useful for the distinction between KS and AS or BLAP.
Assuntos
Herpesvirus Humano 8/isolamento & purificação , Linfedema/patologia , Mastectomia/efeitos adversos , Sarcoma de Kaposi/patologia , Neoplasias Vasculares/patologia , Idoso de 80 Anos ou mais , Diagnóstico Diferencial , Feminino , Hemangiossarcoma/diagnóstico , Hemangiossarcoma/patologia , Hemangiossarcoma/virologia , Herpesvirus Humano 8/imunologia , Humanos , Linfangiossarcoma/diagnóstico , Linfangiossarcoma/patologia , Linfedema/virologia , Sarcoma de Kaposi/virologia , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/virologia , Neoplasias Vasculares/virologiaRESUMO
BACKGROUND: Intravascular NK-cell lymphoma (IVNKL) is an extremely rare variant of non-Hodgkin lymphoma. To our knowledge, there are only a few cases reported in the English literature. Here, a case of a 29-year-old male with IVNKL involving the skin of the trunk and 4 extremities and liver is presented. A comprehensive literature review is undertaken to summarize the clinical and pathological features of this disorder. FINDINGS: In our case, large neoplastic lymphoid cells are restricted to the lumen of small vessels and exhibit the phenotype of a true NK cell. The morphology and immunophenotype, positivity of EBER and NK-cell origin are similar to other IVNKL cases. In addition, some cases including ours carry a poor prognosis as multiple systems or vital organs are involved. CONCLUSION: In summary, we report a case of an unusual intravascular lymphoma of NK-cell lineage that displays both clinical and pathological features and compare it with other differential diagnoses. It is important to recognize this rare entity to make an appropriate diagnosis and achieve a better understanding regarding the treatment and prognosis.
Assuntos
Linfoma Extranodal de Células T-NK/patologia , Células T Matadoras Naturais/patologia , Neoplasias Vasculares/patologia , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biomarcadores Tumorais/análise , Biomarcadores Tumorais/genética , Biópsia , Linhagem da Célula , Diagnóstico Diferencial , Evolução Fatal , Genes Codificadores dos Receptores de Linfócitos T , Herpesvirus Humano 4/genética , Humanos , Imuno-Histoquímica , Linfoma Extranodal de Células T-NK/tratamento farmacológico , Linfoma Extranodal de Células T-NK/genética , Linfoma Extranodal de Células T-NK/imunologia , Linfoma Extranodal de Células T-NK/virologia , Masculino , Células T Matadoras Naturais/imunologia , Células T Matadoras Naturais/virologia , Fenótipo , Valor Preditivo dos Testes , RNA Viral/genética , Tomografia Computadorizada por Raios X , Resultado do Tratamento , Neoplasias Vasculares/tratamento farmacológico , Neoplasias Vasculares/genética , Neoplasias Vasculares/imunologia , Neoplasias Vasculares/virologiaRESUMO
Intravascular large cell lymphoma is a rare subtype of extranodal large cell lymphoma characterized by the presence of neoplastic cells within the lumina of small vessels. Most cases of intravascular large cell lymphoma have a B-cell phenotype. To date, 12 cases of intravascular natural killer (NK/)/T-cell lymphoma (IVNKL) have been reported. Our case is A 47-year-old female presented with erythematous patches and plaques on the lower extremities mimicking panniculitis clinically. A skin biopsy revealed intravascular lymphoma (IVL) with a NK/T cell phenotype (positive for CD3, and granzyme B and negative for CD20, CD4, CD8, CD5). The lymphoma cells were also positive for Epstein-Barr virus by Epstein-Barr virus-encoded RNA in situ hybridization test. Because this type of lymphoma is extremely rare, our case is documented and compared with the previously reported cases.
Assuntos
Linfoma Extranodal de Células T-NK/patologia , Linfoma Cutâneo de Células T/patologia , Paniculite/patologia , Neoplasias Cutâneas/patologia , Neoplasias Vasculares/patologia , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biomarcadores Tumorais/análise , Biópsia , Diagnóstico Diferencial , Feminino , Herpesvirus Humano 4/genética , Humanos , Imuno-Histoquímica , Linfoma Extranodal de Células T-NK/química , Linfoma Extranodal de Células T-NK/tratamento farmacológico , Linfoma Extranodal de Células T-NK/virologia , Linfoma Cutâneo de Células T/química , Linfoma Cutâneo de Células T/tratamento farmacológico , Linfoma Cutâneo de Células T/virologia , Pessoa de Meia-Idade , Valor Preditivo dos Testes , RNA Viral/genética , Neoplasias Cutâneas/química , Neoplasias Cutâneas/tratamento farmacológico , Neoplasias Cutâneas/virologia , Resultado do Tratamento , Neoplasias Vasculares/química , Neoplasias Vasculares/tratamento farmacológico , Neoplasias Vasculares/virologiaRESUMO
Intravascular lymphomas are rare and aggressive hematolymphoid tumors. Here, we describe a human herpesvirus type-8 (HHV-8)/Kaposi sarcoma-associated herpesvirus-positive and Epstein-Barr virus (EBV)-positive intravascular lymphoma. The patient was a 59-year-old human immunodeficiency virus-positive man who presented with diarrhea, abdominal pain, fevers, night sweats, and weight loss. Radiographic studies of the abdomen and pelvis revealed numerous subcentimeter nodules within the subcutaneous fat that lacked connection to the skin. An excisional biopsy demonstrated large atypical cells within vessels in the deep subcutaneous fat, and many of the vessels contained extensive organizing thrombi. The atypical cells lacked strong expression of most B-cell markers but were positive for MUM-1 and showed partial expression of several T-cell markers. An immunohistochemical stain for HHV-8 and an in situ hybridization for EBV were both positive in the neoplastic cells. The disease had a rapidly progressive and fatal course. This lymphoma appears to represent an entirely intravascular form of primary effusion lymphoma and highlights the propensity for HHV-8 and EBV-positive lymphoid neoplasms to show aberrant expression of T-cell markers, illustrates the utility of skin biopsies for the diagnosis of intravascular lymphoma, and suggests that biopsies to evaluate for intravascular lymphoma should be relatively deep and include subcutaneous fat.
Assuntos
Coinfecção , Infecções por Vírus Epstein-Barr/virologia , Infecções por Herpesviridae/virologia , Herpesvirus Humano 4/isolamento & purificação , Herpesvirus Humano 8/isolamento & purificação , Linfoma de Efusão Primária/virologia , Neoplasias Vasculares/virologia , Biomarcadores Tumorais/análise , Biópsia , DNA Viral/isolamento & purificação , Progressão da Doença , Infecções por Vírus Epstein-Barr/metabolismo , Infecções por Vírus Epstein-Barr/patologia , Evolução Fatal , Infecções por Herpesviridae/metabolismo , Infecções por Herpesviridae/patologia , Herpesvirus Humano 4/genética , Humanos , Imuno-Histoquímica , Hibridização In Situ , Linfoma de Efusão Primária/química , Linfoma de Efusão Primária/patologia , Masculino , Pessoa de Meia-Idade , Prognóstico , Fatores de Tempo , Tomografia Computadorizada por Raios X , Neoplasias Vasculares/química , Neoplasias Vasculares/patologiaAssuntos
Linfoma Extranodal de Células T-NK/diagnóstico , Linfoma Cutâneo de Células T/diagnóstico , Neoplasias Cutâneas/diagnóstico , Neoplasias Vasculares/diagnóstico , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biópsia , DNA Viral/análise , Feminino , Herpesvirus Humano 4/genética , Humanos , Imunofenotipagem , Hibridização In Situ , Linfoma Extranodal de Células T-NK/tratamento farmacológico , Linfoma Extranodal de Células T-NK/patologia , Linfoma Extranodal de Células T-NK/virologia , Linfoma Cutâneo de Células T/tratamento farmacológico , Linfoma Cutâneo de Células T/patologia , Linfoma Cutâneo de Células T/virologia , Pele/patologia , Pele/virologia , Neoplasias Cutâneas/tratamento farmacológico , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/virologia , Resultado do Tratamento , Neoplasias Vasculares/tratamento farmacológico , Neoplasias Vasculares/patologia , Neoplasias Vasculares/virologiaRESUMO
OBJECTIVE: To study the clinicopathologic features and disease outcome of intravascular natural killer-cell lymphoma (IVNKL). METHODS: The histologic features, immunohistochemical findings and results of in-situ hybridization for Epstein-Barr virus-encoded RNA (EBER) were analyzed in 2 novel cases of IVNKL. Seven cases of IVNKL previously reported in the literature were reviewed. RESULTS: The patients were a 68-year-old woman and a 22-year-old man. They both presented with erythematous patches and nodules on their trunk and extremities. Skin biopsies confirmed the diagnosis of IVNKL. The tumor cells were positive for CD3, CD56, granzyme B and EBER. Both patients died 2 months after the diagnosis. Amongst the 9 reported cases, including those from the literature, the male was 4 cases, the female was 5 cases. The mean age of the patients was 45.7 years and the median age was 47 years. Skin lesions represented the commonest clinical manifestations. Multiple organ involvement was found in 7 cases and central nervous system was involved in 3 cases. Six patients died during 2 to 17 months of follow-up. The median survival was 9 months and the one-year survival rate was (35.6±18.6)%. The clinical outcome of the patients with multiple organ involvement was worse than that with skin manifestations only. The difference however was not statistically significant (P=0.083). CONCLUSIONS: IVNKL is a rare disease. Diagnosis should be made according to typical histologic findings, immunophenotype and EBER in-situ hybridization results. The overall prognosis of IVNKL is poor. Early diagnosis and treatment before multiorgan involvement may be helpful in improving the clinical outcome.
Assuntos
Células Matadoras Naturais/patologia , Linfoma não Hodgkin/patologia , Neoplasias Vasculares/patologia , Idoso , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Complexo CD3/metabolismo , Antígeno CD56/metabolismo , Ciclofosfamida/uso terapêutico , Doxorrubicina/uso terapêutico , Feminino , Seguimentos , Granzimas/metabolismo , Humanos , Células Matadoras Naturais/metabolismo , Células Matadoras Naturais/virologia , Linfoma não Hodgkin/tratamento farmacológico , Linfoma não Hodgkin/metabolismo , Linfoma não Hodgkin/virologia , Masculino , Pessoa de Meia-Idade , Prednisona/uso terapêutico , RNA Viral/metabolismo , Neoplasias Vasculares/tratamento farmacológico , Neoplasias Vasculares/metabolismo , Neoplasias Vasculares/virologia , Vincristina/uso terapêutico , Adulto JovemRESUMO
The majority of cases of intravascular lymphomatosis (IVL) is derived from B cells. However, IVL may also arise from T cells, or more rarely NK cells. The clinicopathological findings in six cases of NK-cell IVL (NK-IVL), including one new case, were summarised and compared with B-cell IVL (B-IVL) and T-cell IVL (T-IVL). Earlier onset of disease and female predominance were found in NK-IVL. NK-IVL was typically Epstein-Barr virus (EBV)-positive, whereas EBV was rarely detected in B-IVL. Cutaneous manifestations were common in NK-IVL with constant EBV infection. B-IVL showed a more favourable prognosis than T- or NK-IVL. Irrespective of immunophenotype, however, IVL showed a less favourable prognosis than ordinary lymphomas within the same immunophenotype. In summary, IVL of the B-, T- and NK-cell phenotypes is clinicopathologically distinct and shows similarities to their more common counterparts, i.e. diffuse large B-cell lymphoma, peripheral T-cell lymphoma, unspecified and extranodal NK/T-cell lymphoma, nasal type.
Assuntos
Células Matadoras Naturais/patologia , Linfoma não Hodgkin/patologia , Neoplasias Vasculares/patologia , Adulto , Idoso , Antineoplásicos/uso terapêutico , Feminino , Humanos , Linfoma de Células B , Linfoma não Hodgkin/tratamento farmacológico , Linfoma não Hodgkin/virologia , Linfoma de Células T , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento , Neoplasias Vasculares/tratamento farmacológico , Neoplasias Vasculares/virologiaRESUMO
Most cases of intravascular large cell lymphoma have a B-cell phenotype, but rare T-cell and natural killer (NK)-cell variants have been reported. We describe the clinicopathologic features of 4 patients (M:F=3:1; age range: 63 to 87; median age: 65) with intravascular large NK/T-cell lymphoma. The skin was the site of presentation in all patients (leg: 1 case; trunk: 1 case; trunk and extremities: 2 cases). Two patients had lesions confined to the skin; in 1 case concomitant involvement of the brain was detected and in 1 case no further studies were carried out. Immunohistology showed positivity for cytotoxic markers in 3/4 cases. One case had an NK phenotype similar to NK/T-cell lymphoma, nasal-type, whereas the other cases could not be precisely classified into specific categories (peripheral T-cell lymphoma, NOS). One of these cases was negative for cytotoxic markers and was positive only for CD2 and CD3 epsilon. Association with Epstein-Barr virus (EBV) was demonstrated in 2 cases by in situ hybridization, whereas 1 case was negative. All our patients had aggressive disease and died between 2 weeks and 7 months from presentation. Analysis of our cases and of those published in the literature shows that intravascular large NK/T-cell lymphoma is a rare, aggressive lymphoma with variable phenotypic features, frequent expression of cytotoxic proteins, true NK-cell phenotype and association with Epstein-Barr virus infection, and common presentation in the skin. Homogeneous studies on larger number of patients and reevaluation of cases published with incomplete phenotypic data would be necessary to gather more information on this extremely rare type of lymphoma.
Assuntos
Infecções por Vírus Epstein-Barr/virologia , Linfoma Extranodal de Células T-NK/patologia , Linfoma de Células T/patologia , Neoplasias Vasculares/patologia , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Linfoma Extranodal de Células T-NK/virologia , Linfoma de Células T/virologia , Masculino , Pessoa de Meia-Idade , Fenótipo , Neoplasias Vasculares/virologiaAssuntos
Síndrome da Imunodeficiência Adquirida/complicações , Neoplasias Encefálicas/virologia , Infecções por Vírus Epstein-Barr/virologia , Pericitos/patologia , Neoplasias Vasculares/virologia , Síndrome da Imunodeficiência Adquirida/virologia , Adulto , Neoplasias Encefálicas/patologia , Diagnóstico Diferencial , Endotélio Vascular/patologia , Infecções por Vírus Epstein-Barr/patologia , Hemangiopericitoma/patologia , Herpesvirus Humano 4 , Humanos , Imuno-Histoquímica , Hibridização In Situ , Leiomioma/patologia , Neoplasias Hepáticas/secundário , Masculino , Miócitos de Músculo Liso/patologia , Pericitos/virologia , Neoplasias Vasculares/patologiaRESUMO
BACKGROUND: Based on the spectrum of histological features, Kaposi's sarcoma (KS) is grouped into patch, plaque and nodular stages. The histological changes overlap, especially with lesional evolution. To date, intravascular KS is undocumented. METHODS: A clinicopathological description of six cases of intravascular KS. RESULTS: Clinical: There were four men and two women (mean age = 65 years). Four patients, who presented clinically with classic (sporadic) KS, developed solitary violaceous nodules on the extremities. Two patients with acquired immune deficiency syndrome-related KS had disseminated cutaneous KS lesions in all stages of evolution. Six months to 3 years follow-up showed no evidence of systemic KS in any of the patients. Histopathology: Exclusive intravascular growth was seen in five patients. The vascular channels, highlighted by mural immunostaining with desmin and anti-smooth muscle actin, had the histological features of veins. Intravascular growth was characterized by interlacing fascicles of human herpesvirus 8, CD31 and CD34-positive spindle cells with formation of cleft-like spaces, erythrocyte extravasation, hyaline globules and a lymphoplasmacytic infiltrate. One patient had a proliferation of irregular, vascular channels in the desmin in addition to the intravenous growth. CONCLUSION: Intravascular KS is a peculiar hitherto unrecognized morphological variant of KS that does not seem to be associated with an increased risk of aggressive behaviour.
Assuntos
Sarcoma de Kaposi/patologia , Neoplasias Cutâneas/patologia , Neoplasias Vasculares/patologia , Idoso , Idoso de 80 Anos ou mais , Feminino , Herpesvirus Humano 8 , Humanos , Masculino , Pessoa de Meia-Idade , Sarcoma de Kaposi/metabolismo , Sarcoma de Kaposi/virologia , Pele/irrigação sanguínea , Pele/patologia , Pele/virologia , Neoplasias Cutâneas/metabolismo , Neoplasias Cutâneas/virologia , Neoplasias Vasculares/metabolismo , Neoplasias Vasculares/virologia , Veias/metabolismo , Veias/patologiaRESUMO
Cattles suffering from chronic enzootic haematuria frequently develop urinary bladder tumours of both epithelial and mesenchymal origin mainly haemangioma and its malignant counterpart. The role of the bovine papillomavirus type-2 (BPV-2) and of its major transforming oncoprotein in naturally occurring urothelial carcinogenesis has been recently clarified. E5 interacts in vivo as in vitro with the beta receptor for the platelet-derived growth factor (PDGF). However, studies regarding tumours of mesenchymal origin such as those arising from blood vessels are lacking. We show that the BPV-2 is present in 100% of the vascular tumours of the urinary bladder examined. Twenty-six out of twenty-seven tumour samples (96%) expressed E5 while 20 out of 27 (74%) tumour samples expressed E7. The two viral oncoproteins were not expressed in normal endothelial cells. Additionally, they co-localize in neoplastic endothelial cells as demonstrated by confocal immunofluorescence. PDGFbeta receptor was also shown to be expressed and co-localizes with E5 in neoplastic blood vessels. Our results demonstrate, for the first time, that the BPV-2 is present in high percentage in tumours of mesenchymal origin arising in its natural host. Furthermore, the expression of the two viral oncoproteins confirm that the virus may have a causative role in the neoplastic process.
Assuntos
Papillomavirus Bovino 1/genética , DNA Viral/metabolismo , Neoplasias da Bexiga Urinária/veterinária , Neoplasias da Bexiga Urinária/virologia , Neoplasias Vasculares/genética , Neoplasias Vasculares/virologia , Animais , Bovinos , Doenças dos Bovinos , Proteínas Oncogênicas/metabolismo , Proteínas Oncogênicas Virais , Proteínas E7 de Papillomavirus , Receptor beta de Fator de Crescimento Derivado de Plaquetas/metabolismoRESUMO
Intravascular lymphoma (IVL) is a rare variant of non-Hodgkin lymphoma with a predilection for skin and brain. Except a few cases of T-cell lineage, most of the reported cases were large B-cell lymphomas. We encountered a case of cutaneous IVL in a 71-year-old woman presenting with multiple erythematous patches and nodules on her trunk and extremities. The intravascular large cells showed an immunophenotype of CD3epsilon(+);, CD5(-), CD20(-), CD30(-), CD56(+), and TIA-1(+). The lymphoma cells were also positive for Epstein-Barr virus by Epstein-Barr virus-encoded RNA in situ hybridization test and the T-cell receptor gene was germline. This IVL differs from nasal type NK/T-cell lymphoma only by its intravascular nature. Only 3 cases of intravascular NK-cell lymphoma have been reported before. Because this variant is extremely rare, our case is documented and compared with the 3 previously reported cases.
Assuntos
Herpesvirus Humano 4/isolamento & purificação , Células Matadoras Naturais , Linfoma não Hodgkin/virologia , Neoplasias Cutâneas/virologia , Neoplasias Vasculares/virologia , Idoso , Feminino , Herpesvirus Humano 4/genética , Humanos , Imunofenotipagem , RNA Viral/análiseRESUMO
Primary leiomyosarcoma of the pulmonary artery is extremely uncommon and its cause remains unclear. We document a case of pulmonary artery leiomyosarcoma expressing Epstein-Barr virus DNA sequences in an immunocompetent patient 4 years after symptomatic Epstein-Barr virus infection.
Assuntos
Infecções por Vírus Epstein-Barr/complicações , Leiomiossarcoma/virologia , Artéria Pulmonar , Neoplasias Vasculares/virologia , Adulto , DNA Viral/análise , Evolução Fatal , Feminino , Herpesvirus Humano 4/genética , Humanos , Imuno-Histoquímica , Leiomiossarcoma/metabolismo , Leiomiossarcoma/cirurgia , Pneumonectomia , Artéria Pulmonar/cirurgia , Fatores de Tempo , Neoplasias Vasculares/metabolismo , Neoplasias Vasculares/cirurgiaRESUMO
A 42-year-old female underwent hysterectomy because of a huge uterine mass. Histologically, she was diagnosed as having intravascular lymphoma co-existing with myoma uteri. Lymphoma cells were large in size and were positive for CD5, CD20, CD45, CD79a, lambda light chain, and EBV but were negative for CD3 and cyclin D1. No other organs except for the adjoining bilateral ovaries seemed to be affected by the lymphoma cells. She received the combination chemotherapy (cyclophosphamide, doxorubicin, vincristine, and prednisolone) together with rituximab and has been well without definite disease progression. So far, this is the first case of CD5+ EBV+ intravascular large B-cell lymphoma (CD5+ EBV+ IVLBL) in the uterus of a patient who was incidentally diagnosed and successfully treated.