Pay attention to the detection and evaluation of CD30 in pathological diagnosis of lymphoma / 中华病理学杂志

CD30 is a transmembrane glycoprotein of tumor necrosis factor receptor family, which is expressed differently in various lymphomas. It has specific effects on the proliferation and function of tumor cells. However, the significance of CD30 expression has not been fully recognized by clinicians and pathologists, and the detection and evaluation of CD30 have not been standardized. The role of CD30 in the diagnosis of lymphoma, detection methods and reporting standards are summarized in the paper to improve the understanding of CD30.

Uncommon anatomical sites and anatomopathological presentations of lymphoma in dogs / Sítios anatômicos e apresentações anatomopatológicas incomuns do linfoma em cães

Lymphomas are the tumors most frequently associated with the death or euthanasia of dogs in most parts of the world. In dogs, they almost always occur as disseminated (multicentric lymphoma), gastrointestinal (alimentary lymphoma), or nodal mediastinal (mediastinal lymphoma) diseases. However, other uncommon presentations can occasionally occur. This study aimed to establish the prevalence of these unusual types of lymphoma and demonstrate to veterinary pathologists how they present pathologically. From a total of 100 cases of lymphoma in dogs diagnosed between 1965 and 2017, 16 cases (16/100) were considered by us as non-traditional presentations of the disease: follicular lymphoma, (5/100), peripheral T-cell lymphoma, NOS (2/100), angiocentric lymphoma (2/100), intravascular large T-cell lymphoma (2/100), lymphomatoid granulomatosis (1/100), anaplastic large-cell lymphoma (1/100), hepatosplenic T-cell lymphoma (1/100), and chronic small B-cell lymphocytic lymphoma, intermediate type (1/100). We hope that the results presented here can help veterinary pathologists to recognize such cases of "atypical lymphoma" in their diagnostic routines.(AU)

Linfomas são os tumores mais associados a morte ou eutanásia de cães na maior parte do mundo. Nessa espécie animal ocorrem quase sempre como uma doença disseminada (linfoma multicêntrico), gastrintestinal (linfoma alimentar) ou nodal mediastinal (linfoma mediastínico), entretanto, ocasionalmente, outras apresentações bem menos comuns podem ser encontradas. O objetivo deste artigo é estabelecer a prevalência desses pouco usuais tipos de linfoma e demonstrar aos patologistas veterinários como eles se apresentam anatomopatologicamente. De um total de 100 casos de linfoma em cães diagnosticados entre os anos de 1965 e 2017, 16 casos (16/100) foram considerados como apresentações não tradicionais da doença: linfoma folicular (5/100), linfoma de células T periférico inespecífico (2/100), linfoma angiocêntrico (2/100), linfoma intravascular de grandes células T (2/100), granulomatose linfomatoide (2/100), linfoma de grandes células anaplásicas (1/100), linfoma hepatoesplênico de células T (1/100) e linfoma linfocítico crônico de pequenas células B - tipo intermediário (1/100). Esperamos que os resultados aqui demonstrados auxiliem patologistas veterinários a reconhecerem tais casos de "linfomas atípicos" em suas rotinas diagnósticas.(AU)

NADPH Oxidase 5 upregulation is associated with lymphoma aggressiveness

SUMMARY OBJECTIVES Lymphomas are a heterogeneous set of malignant neoplasias of lymphoid B and NK/T mature and immature cells at various stages of differentiation. Genetic and molecular biology tools are used to appropriately classify the type and prognosis of the lymphomas, which have implications in therapeutic effectiveness. Among them, the nicotinamide adenine dinucleotide phosphate-oxidase (NADPH) oxidase (NOX5) enzymes have been explored. This study analyzed the expression of NADPH oxidase 5 in lymphoma tissue according to the degree of tumor aggressiveness. METHODS Slides from 64 patients with lymphoma who had paraffin-embedded tissue available were reviewed by two independent, experienced pathologists. They classified tumors according to the WHO classification (2017). NOX5 expression in tissues was assessed by immunohistochemical staining using a tissue microarray. The assay was interpreted using a scoring system of 0, 1, 2, and 3, for cytoplasmic staining of NOX5 corresponding to negative, weak, intermediate, and strong staining, respectively. We compared the expression of NOX5 in patients with aggressive versus non-aggressive lymphomas. RESULTS NOX5 expression was positive in 100% (27/27) of aggressive lymphomas and in 19% (7/37) of non-aggressive ones. The seven patients with positive expression of NOX5 presented intermediate staining (2); strong staining (3) was observed only in tissues of aggressive lymphomas, and negative and weak staining (0 and 1) were observed only in non-aggressive lymphomas. CONCLUSIONS Aggressive lymphomas overexpress NOX5 protein. The higher NOX5 expression in aggressive lymphomas can suggest an involvement of this enzyme on the acquisition of an aggressive phenotype in lymphoid neoplasia.

RESUMO OBJETIVOS Os linfomas são um grupo heterogêneo de neoplasias malignas de células linfoides B e NK/T maduras e imaturas em vários estágios de diferenciação. Ferramentas de biologia molecular e genética são usadas para classificar adequadamente o tipo e o prognóstico dos linfomas, os quais têm implicações na eficácia terapêutica. Entre eles, as enzimas nicotinamida adenina dinucleótido fosfato oxidase (NADPH) oxidase (NOX5) foram exploradas. Este estudo analisou a expressão da NADPH oxidase 5 em linfomas de acordo com o grau de agressividade tumoral. MÉTODOS As lâminas de 64 pacientes com linfoma, que tinham tecido embebido em parafina disponível, foram revisadas por dois patologistas experientes independentemente. Eles utilizaram a classificação da OMS (2017). A expressão de NOX5 nos tecidos foi avaliada por coloração imuno-histoquímica utilizando microarray de tecido. O ensaio foi interpretado com um sistema de pontuação de 0, 1, 2 e 3, para coloração citoplasmática de NOX5 correspondente à coloração negativa, fraca, intermediária e forte, respectivamente. Comparamos a expressão de NOX5 em pacientes com linfomas agressivos versus não agressivos. RESULTADOS A expressão de NOX5 foi positiva em 100% (27/27) dos linfomas agressivos e em 19% (7/37) dos linfomas não agressivos. Os sete pacientes com expressão positiva de NOX5 apresentaram coloração intermediária (2); coloração forte (3) foi observada apenas em tecidos de linfomas agressivos, e negativos e fracos (0 e 1) observados apenas em linfomas não agressivos. CONCLUSÕES Linfomas agressivos superexpressam a proteína NOX5. A expressão aumentada de NOX5 em linfomas agressivos pode sugerir um envolvimento dessa enzima na aquisição de um fenótipo agressivo na neoplasia linfoide.

Ileítis como presentación de linfoma en síndrome de Wiskott-Aldrich / Ileitis as presentation of lymphoma in Wiskott-Aldrich syndrome

El síndrome de Wiskott-Aldrich (SWA) es un raro síndrome de inmunodeficiencia primaria ligado al cromosoma X que se asocia con aumento de incidencia de infecciones, trastornos autoinmunes y neoplasias. Se presenta el caso de un varón de 41 años con diagnóstico de síndrome de Wiskott-Aldrich y cuadro de ileítis como forma de presentación de un síndrome linfoproliferativo. La ileítis, en el contexto del paciente, representa un problema clínico dado el gran número de diagnósticos diferenciales (enfermedad inflamatoria intestinal, infecciones, neoplasias y enfermedades linfoproliferativas) por lo que suele requerir diagnóstico anatomopatológico y consideraciones particulares respecto al posterior tratamiento específico.

Wiskott-Aldrich syndrome is a rare X chromosome-linked primary immunodeficiency syndrome associated with an increased incidence of infections, autoimmune disorders and neoplasms. We present the case of a 41-year-old man with a diagnosis of Wiskott-Aldrich syndrome with ileitis as a form of presentation of a lymphoproliferative syndrome. The ileitis, in the context of the patient, represents a clinical challenge given the large number of differential diagnoses (inflammatory bowel disease, infections, neoplasms and lymphoproliferative diseases), so it usually requires anatomopathological diagnosis and particular considerations regarding the subsequent specific treatment.

Acute kidney injury and cardiac arrhythmia as the presenting features of widespread diffuse large B-cell lymphoma

Diffuse Large B-cell Lymphoma, the most common adult non-Hodgkin lymphoma, is a proliferative neoplasm of enlarged B cells. Patients may be asymptomatic on presentation, but if present, symptoms often correlate with direct organ dysfunction resulting from the site of involvement. While the gastrointestinal system is the most common site of extranodal involvement, virtually any part of the body can be infiltrated by malignant lymphocytes. Here, we present an unusual case of cardiac and bilateral renal involvement by Diffuse Large B-cell Lymphoma in a 78-year-old male with a relatively unremarkable medical history. This combination of organ involvement and the resulting clinical symptoms are uncommonly described in the literature. The patient was treated for his symptoms prior to death, but the underlying cause that explained his presentation was not identified until performance of an autopsy. As such, this case demonstrates the utility of the medical autopsy, a gold standard in diagnostic medicine that can provide a variety of benefits in today's healthcare system.

Feline lymphoma in the nervous system: pathological, immunohistochemical, and etiological aspects in 16 cats / Linfoma no sistema nervoso de felinos: aspectos patológicos, imuno-histoquímicos e etiológicos em 16 gatos

The pathological, immunohistochemical (IHC), and etiological features of lymphoma involving the nervous system (NS) in cats were analyzed through a retrospective study (2004-2017) in Rio Grande do Sul State, Brazil. The NS involvement was observed in 16 (12.2%) of 125 felines with lymphoma. Young cats were mainly affected, with a median of 24 months old. Most cases were secondary central NS lymphoma, whereas in three cats, the NS involvement was primary. IHC revealed 14 (87.5%) FeLV-positive, six FIV-positive, and one FeLV/FIV-negative cats. Distribution of feline lymphoma in the NS was 8/16 in the spinal cord, 7/16 in the brain, and 1/16 in the paravertebral nerves and ganglia (neurolymphomatosis). The lymphoma pattern in the spinal cord was exclusively extradural, often focal (6/8), and located in the lumbar (3/6), sacral (1/6), thoracic (1/6), and cervical segments (1/6). Brain neuroanatomical patterns were: leptomeningeal lymphomatosis (4/7), lymphomatous choroiditis (2/7), and intradural lymphoma (1/7). The feline with primary neurolymphomatosis presented a marked thickening of paravertebral nerves and ganglia from the sacral region. B-cell lymphoma (75%) was often diagnosed, and diffuse large B-cell lymphoma (DLBCL) (11/16) was the main subtype. T-cell lymphoma (25%) was less commonly observed and was classified as peripheral T-cell lymphoma (PTCL) (3/16) and T-cell lymphoblastic lymphoma (T-LBL) (1/16).(AU)

Os aspectos patológicos, imuno-histoquímicos (IHQ) e etiológicos do linfoma envolvendo o sistema nervoso de felinos foram analisados através de um estudo retrospectivo (período de 2004-2017) no Estado do Rio Grande do Sul, Brasil. O envolvimento do sistema nervoso foi observado em 16 (12,2%) dos 125 felinos com linfoma desse estudo e afetou principalmente, jovens com idade mediana de 24 meses. A grande maioria dos casos o linfoma era secundário no sistema nervoso central e somente em três gatos o linfoma foi primário do sistema nervoso. Na IHQ, 14 (87,5%) casos foram positivos para FeLV, seis (37,5%) para FIV, e um foi negativo para ambos. A distribuição do linfoma no sistema nervoso foi em 8/16 felinos na medula espinhal, 7/16 no encéfalo e em 1/16 em nervos e gânglios paravertebrais (neurolinfomatose). Na medula espinhal, o padrão do linfoma foi exclusivamente extradural e frequentemente focal (6/8), localizadas nos segmentos lombares (3/6), sacrais (1/6), torácicos (1/6) e cervicais (1/6). No encéfalo, os padrões neuroanatômicos observados foram: linfomatose leptomeningeal (4/7), coroidite linfomatosa (2/7), linfoma intradural (1/7). No felino diagnosticado com neurolinfomatose primária, foi observado acentuado espessamento dos nervos e gânglios paravertebrais da região sacral. Os linfomas de células de células B (75%) foram os mais frequentes e o principal tipo foi o linfoma difuso de grandes células B (11/16). Os linfomas de células T (25%), menos observados, foram classificados como linfomas de células T periférico inespecífico (3/16) e linfoma linfoblástico T (1/16).(AU)

Tumores hepáticos primarios malignos no hepatocarcinoma ni colangiocarcinoma / Primary malignant hepatic tumors other than hepatocarcinoma or cholangiocarcinoma: a series of cases

Background: There are several types of primary malignant hepatic tumors (PMHT) other than hepatocellular carcinoma (HCC) and cholangiocarcinoma (CC): they are infrequent and poorly known. Imaging studies could help characterize the lesions and may guide the diagnosis. However, the definitive diagnosis of PMHT is made by pathology. Aim: To review a registry of liver biopsies performed to diagnose hepatic tumors. Patients and Methods: Review of a pathology registry of liver biopsies performed for the diagnosis of liver tumors. Among these, 25 patients aged 57 ± 17 years, 60% males, in whom a liver tumor other than a HCC or CC was diagnosed, were selected for review. The medical records of these patients were reviewed to register their clinical characteristics, imaging and the pathological diagnosis performed during surgery and/ or with the percutaneous liver biopsy. Results: Ten patients (40%) had neuroendocrine tumors, six (24%) had a lymphoma and four (16%) had hepatic hemangioendothelioma. Angiosarcoma and sarcomatoid carcinoma were diagnosed in one patient each. In 22 patients (88%), neither clinical features nor imaging studies gave the correct diagnosis. Four patients (16%) had chronic liver disease. The most frequent symptoms were weight loss in 28% and abdominal pain in 24%. Conclusions: The most common PMHT other than HCC and CC were neuroendocrine tumors and lymphomas. Imaging or clinical features were not helpful to reach the correct diagnosis.

Outcomes after surgical resection of primary non-myxoma cardiac tumors

Abstract Objective: Primary cardiac tumors are rare lesions with different histological type. We reviewed our 17 years of experience in the surgical treatment and clinical results of primary non-myxoma cardiac tumors. Methods: Between July 2000 and February 2017, 21 patients with primary cardiac tumor were surgically treated in our institution. The tumors were categorized as benign non-myxomas and malignants. Data including the demographic characteristics, details of the tumor histology and grading, cardiac medical and surgical history, surgical procedure of the patients were obtained from the hospital database. Results: Eleven patients were diagnosed with benign non-myxoma tumor (male/female:7/4), ranging in age from 10 days to 74 years (mean age 30.9±26.5 years). Papillary fibroelastoma was the most frequent type (63.6%). There were two early deaths in benign group (all were rhabdomyoma), and mortality rate was 18%. The mean follow-up period was 69.3±58.7 months (range, 3 to 178 months). All survivals in benign group were free of tumor-related symptoms and tumor relapses. Ten patients were diagnosed with malignant tumor (sarcoma/lymphoma:8/2, male/female:3/7), ranging in age from 14 years to 73 years (mean age 44.7±18.9 years). Total resection could be done in only three (30%) patients. The mean follow-up period was 18.7±24.8 months (range, 0-78 months). Six patients died in the first 10 months. Conclusion: Complete resection of the cardiac tumors, whenever possible, is the main goal of surgery. Surgical resection of benign cardiac tumors is safe, usually curative and provides excellent long-term prognosis. On the contrary, malignant cardiac tumors still remain highly lethal.

The role of surgery in primary central nervous system lymphomas / El rol de la cirugía en los linfomas primarios del sistema nervioso central

ABSTRACT Background Primary central nervous system lymphomas (PCNSL) are infrequent. The traditional treatment of choice is chemotherapy. Complete resections have generally not been recommended, because of the risk of permanent central nervous system deficits with no proven improvement in survival. The aim of the current study was to compare survival among patients with PCNSL who underwent biopsy versus surgical resection. Methods A retrospective study was conducted on 50 patients with a confirmed diagnosis of PCNSL treated at our center from January 1994 to July 2015. Results Patients in the resection group exhibited significantly longer median survival time, relative to the biopsy group, surviving a median 31 months versus 14.5 months; p = 0.016. Conclusions In our series, patients who had surgical resection of their tumor survived a median 16.5 months longer than patients who underwent biopsy alone.

RESUMO Introducción Los linfomas primarios del sistema nervioso central (LPSNC) son infrecuentes. Tradicionalmente el tratamiento de elección es la quimioterapia. Existe un paradigma de no indicar resección, por el riesgo de déficit neurológico sin aumento de la sobrevida. El objetivo del presente estudio es comparar la sobrevida de pacientes con LPSNC sometidos a biopsia versus resección. Métodos Estudio retrospectivo que incluye 50 pacientes con diagnóstico confirmado de LPSNC tratados en nuestra Institución desde enero de 1994 hasta julio de 2015. Resultados Los pacientes del "grupo resección" mostraron una sobrevida media significativamente mayor respecto a los del "grupo biopsia"; 31 meses versus 14,5 meses respectivamente, p = 0,016. Conclusiones En nuestra serie, los pacientes que con resección quirúrgica de su tumor tuvieron una sobrevida media de 16,5 meses superior que los pacientes biopsiados.

Surgical treatment of gastric cancer: a 10-year experience in a high-volume university hospital

OBJECTIVES: Surgery remains the cornerstone treatment modality for gastric cancer, the fifth most common type of tumor in Brazil. The aim of this study was to analyze the surgical treatment outcomes of patients with gastric cancer who were referred to a high-volume university hospital. METHODS: We reviewed all consecutive patients who underwent any surgical procedure due to gastric cancer from a prospectively collected database. Clinicopathological characteristics, surgical and survival outcomes were evaluated, with emphasis on patients treated with curative intent. RESULTS: From 2008 to 2017, 934 patients with gastric tumors underwent surgical procedures in our center. Gastric adenocarcinoma accounted for the majority of cases. Of the 875 patients with gastric adenocarcinoma, resection with curative intent was performed in 63.5%, and palliative treatment was performed in 22.4%. The postoperative surgical mortality rate for resected cases was 5.3% and was related to D1 lymphadenectomy and the presence of comorbidities. Analysis of patients treated with curative intent showed that resection extent, pT category, pN category and final pTNM stage were related to disease-free survival (DFS) and overall survival (OS). The DFS rates for D1 and D2 lymphadenectomy were similar, but D2 lymphadenectomy significantly improved the OS rate. Additionally, clinical factors and the presence of comorbidities had influence on the OS. CONCLUSIONS: TNM stage and the type of lymphadenectomy were independent factors related to prognosis. Early diagnosis should be sought to offer the optimal surgical approach in patients with less-advanced disease.

Intravascular large B-cell lymphoma with multi-organ failure presenting as a pancreatic mass: a case with atypical presentation and definite diagnosis postmortem

Intravascular large B-cell lymphoma (IVLBCL) is a very rare extra nodal lymphoma that tends to proliferate within small blood vessels, particularly capillaries and postcapillary venules while sparing the organ parenchyma. The cause of its affinity for the vascular bed remains unknown. Because of its rarity and unremarkable clinical presentation, a timely diagnosis of IVLBCL is very challenging. Here, we describe a case of IVLBCL presenting as pancreatic mass that was ultimately diagnosed at autopsy. A 71-year-old Caucasian female presented with a 3-month history of fatigue, abdominal pain, and weight loss. She was referred to the emergency room with a new diagnosis of portal vein thrombosis and lactic acidosis. During her hospital course she was found to have a 1.9 × 1.8 cm lesion in the pancreatic tail on imaging; The cytologic specimen on the mass showed a high-grade lymphoma. A bone marrow biopsy showed no involvement. The patient's condition rapidly deteriorated and she, later, died due to multi-organ failure. An autopsy revealed diffuse intravascular invasion in multiple organs by the lymphoma cells. Based on our literature review­and to the best of our knowledge­there are virtually no reports describing the presentation of this lymphoma with a discernible tissue mass and associated multi-organ failure. The immunophenotypic studies performed revealed de novo CD5+ intravascular large B-cell lymphoma, which is known to be aggressive with very poor prognosis. Although it is a very rare lymphoma, it should be considered as a potential cause of multi-organ failure when no other cause has been identified. A prompt tissue diagnosis, appropriate high-dose chemotherapy and stem cell transplantation remain the only viable alternative to achieve some kind of remission.

Colonic basidiobolomycosis with liver involvement masquerading as gastrointestinal lymphoma: a case report and literature review

Abstract Basidiobolomycosis is an unusual fungal skin infection that rarely involves the gastrointestinal tract. This study reported a 5-year-old boy with gastrointestinal basidiobolomycosis that had been misdiagnosed as gastrointestinal lymphoma. He was treated by surgical resection and a combination of posaconazole and amphotericin B deoxycholate with an acceptable response and no recurrence.

Mucinose folicular primária ­ relato de caso / Primary follicular mucinosis ­ case report

De etiologia desconhecida, a mucinose folicular primária ou idiopática se caracteriza clinicamente como afecção inflamatória, com placas mais ou menos infiltradas e descamativas, com ou sem perda de pelos. Na sua forma secundária, costuma apresentar lesões mais numerosas e difusas, com morfologia variá- vel, desde placas até nódulos ulcerados. É representada por depósitos localizados ou difusos de mucina na pele ou nos folículos pilosos. Objetivo: Apresentar um caso incomum de mucinose folicular primária, de importância da diferenciação com a forma secundária de mucinose folicular, discutir os aspectos clínicos e histopatológicos utilizados no diagnóstico, suas características e classificações, bem como as possíveis escolhas terapêuticas.(AU)

Of unknown etiology, acute follicular mucinosis is clinically characterized as an inflammatory disease coursing with more or less infiltrated and scaly plaques, with or without hair loss. In the chronic form, it usually has more numerous and diffuse lesions with variable morphology, from plaques to ulcerated nodules. It is characterized by localized or diffuse deposits of mucin in the skin or hair follicles. Objective: To present an unusual case of primary follicular mucinosis, the importance of differentiation from the secondary form of follicular mucinosis, to discuss the clinical and histopathological aspects used in the diagnosis, its characteristics and classifications as well as the possible therapeutic choices.(AU)

Biological therapy and development of neoplastic disease in patients with juvenile rheumatic disease: a systematic review / Uso de imunobiológicos e desenvolvimento de doenças neoplásicas em pacientes com doenças reumáticas juvenis: revisão sistemática

Abstract Juvenile rheumatic diseases affect the musculoskeletal system and begin before the age of 18. These conditions have varied, identifiable or unknown etiologies, but those of an autoimmune inflammatory nature have been associated with an increased risk of development of cancer, regardless of treatment. This study aims to assess, through a systematic review of the literature according to Prisma (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) quality criteria, the risk of cancer in patients with juvenile rheumatic disease, and its association with biological agents. The criteria described by the Strengthening the Reporting of Observational Studies in Epidemiology initiative were used in order to assess the methodological quality of those individual items selected in this study. We analyzed nine publications, from a total of 251 papers initially selected. There was an increase in cancer risk in the population with juvenile rheumatic disease versus the general population. Most specified cancers were of a lymphoproliferative nature. Seven studies did not specify the treatment or not defined an association between treatment and cancer risk. Only one study has suggested this association; in it, their authors observed high risk in patients diagnosed in the last 20 years, a period of the advent of new therapies. One study found an increased risk in a population not treated with biological agents, suggesting a disease in its natural course, and not an adverse effect of therapy. Studies have shown an increased risk of malignancy associated with juvenile rheumatic disease, and this may be related to disease activity and not specifically to the treatment with biological agents.

Resumo As doenças reumáticas juvenis afetam o sistema musculoesquelético e se iniciam antes dos 18 anos. Apresentam etiologia variada, identificável ou desconhecida, porém as de natureza inflamatória autoimune têm sido associadas ao maior risco de desenvolvimento de neoplasias, independentemente do tratamento. Este artigo propõe avaliar, por meio de revisão sistemática da literatura de acordo com os critérios de qualidade Prisma (Preferred Reporting Items for Systematic Reviews and Meta- Analyses), o risco de câncer em pacientes com doenças reumáticas juvenis e sua associação com imunobiológicos. Os critérios descritos pela iniciativa Strengthening the Reporting of Observational Studies in Epidemiology foram usados para avaliar a qualidade metodológica individual dos artigos selecionados no presente estudo. Foram analisadas nove publicações, de 251 incialmente selecionadas. Houve aumento no risco de câncer na população com doença reumática juvenil comparada com a população em geral. A maioria dos cânceres especificados foi de natureza linfoproliferativa. Sete estudos não especificaram a terapêutica ou não definiram associação entre ela e o risco de câncer. Apenas um estudo sugeriu essa associação e observou maior risco em pacientes diagnosticados nos últimos 20 anos, período de advento de novas terapias. Um estudo constatou maior risco em uma população não tratada com imunobiológicos, sugeriu tratar-se da evolução natural da doença, e não do efeito adverso da terapêutica. Os estudos demonstram aumento no risco de malignidade associada a doenças reumáticas juvenis que pode estar relacionada à atividade da doença, e não especificamente ao tratamento com imunobiológicos.

Small clonal B-cell population in the bone marrow as a possible tool in the diagnosis of occult primary parotid lymphoma / Pequeña población de células B monoclonales en medula ósea como posible herramienta diagnóstica en el linfoma oculto primario de parótida

Case Description: An 82-years old Hispanic woman with a past medical history significant for pulmonary thromboembolism on oral anticoagulation, rheumatoid arthritis, and hypertension developed a new onset thrombocytopenia. Clinical Findings: Small clonal B-cells populations (SCBP) also known as monoclonal B-cell lymphocytosis was found as part of the workup for an idiopathic thrombocytopenia and lead ultimately to the diagnosis of parotid primary follicular lymphoma coexisting with Warthin tumor involving the bone marrow in a small extent and oncocytic papilloma located in the maxillary sinus. Treatment and Outcome: Patient was treated with Rituximab monotherapy with improvement on her platelet count. Clinical relevance: Although it is unclear the role of this clonal cells, they may work as a possible diagnostic tool for occult lymphomas. Further prospective studies are needed to confirm this possible association.

Descripción de caso: Mujer hispana de 82 años con una historia médica significativa de tromboembolismo pulmonar en anticoagulación, artritis reumatoide e hipertensión, la cual desarrolló recientemente una trombocitopenia. Hallazgos clínicos: Una pequeña población de células B monoclonales también conocida como linfocitosis monoclonal de células B fue encontrado dentro del estudio de una trombocitopenia idiopática que conllevó al diagnóstico de un linfoma folicular primario de parótida coexistiendo con un tumor de Warthin y un papiloma oncocítico localizado en el seno maxilar. Tratamiento y resultado: La paciente fue tratada con monoterapia de Rituximab con una mejoría en su conteo de plaquetas. Relevancia clínica: Aunque el rol de las pequeñas poblaciones B monoclonales no está completamente dilucidado, podrían tener una aplicación como herramienta diagnóstica. Futuros estudios prospectivos son necesarios para confirmar esta posible asociación.

Survival analysis in patients with metastatic spinal disease: the influence of surgery, histology, clinical and neurologic status / Análise de sobrevida em pacientes com doença metastática espinhal: influência da cirurgia, histologia, status clínico e neurológico

Spine is the most common site for skeletal metastasis in patients with malignancy. Vertebral involvement quantification, neurological status, general health status and primary tumor histology are factors to set surgical planning and therapeutic targets. We evaluated the impact of general clinical and neurological status, histologic type and surgery in survival. Method : The study sample consisted of consecutive patients admitted from July 2010 to January 2013 for treatment. Results : Sixty eight patients were evaluated. 23 were female and 45 were male. Main primary neoplasic sites were: breast, prostate, lung/pleura and linfoproliferative. Thirty three out of 68 received surgical treatment, 2 received percutaneous biopsy and 33 had nonsurgical treatment. Survival : Log Rank curves revealed no statistical significant difference according to histological type, surgical approach and Frankel Score. Karnofsky Score was statistically different. Conclusion : Histological type and clinical status were statistically associated with life expectancy in vertebral metastatic disease. .

A coluna vertebral é o sítio mais comum de metastases ósseas. A quantificação do acometimento vertebral, o status neurológico, status clínico e histologia do tumor primário são fatores importantes para planejamento cirúrgico e metas terapêuticas. Nós avaliamos o impacto do status clinico geral e neurológico, tipo histológico e cirurgia na sobrevida de pacientes com metástases espinhais. Método : A amostra consistiu de pacientes consecutivamente admitidos de Julho de 2010 a Janeiro de 2013. Resultados : Sessenta e oito pacientes foram avaliados. 23 eram mulheres e 45 eram homens. Os principais sítios primários foram mama, próstata, pulmão e linfoproliferativos. Trinta e três realizaram tratamento cirúrgico, 2 realizaram biópsia percutânea e 33 tiveram tratamento conservador e radioterapia. Conclusão As curvas Log Rank não revelaram significância quanto à cirurgia e escore de Frankel, mas revelaram associação com Karnofsky e tipo histológico. .

Whole-body diffusion-weighted magnetic resonance imaging versus FDG-PET/CT for initial lymphoma staging: systematic review on diagnostic test accuracy studies / Ressonância magnética de corpo total com difusão comparada à FDG-PET/CT no estadiamento inicial do linfoma: revisão sistemática de estudos de acurácia diagnóstica

CONTEXT AND OBJECTIVE: Positron emission tomography with [18]F-fluoro-2-deoxyglucose (FDG-PET/CT) has been advocated as the method of choice for lymphoma staging, since it enables whole-body analysis with high sensitivity for detection of affected areas and because it combines capacities for anatomical and functional assessment. With technological advances, magnetic resonance imaging (MRI) has emerged as an alternative to FDG-PET/CT. This systematic review with meta-analysis aimed to compare whole-body diffusion-weighted MRI (WB-MRI) with FDG-PET/CT for lymphoma staging. DESIGN AND SETTING: Systematic review on diagnostic test accuracy studies conducted at a public university. METHODS: The Medline, Scopus, Embase and Lilacs databases were searched for studies published up to September 2013 that compared WB-MRI and FDG-PET/CT for lymphoma staging. The reference lists of included studies were checked for any relevant additional citations. RESULTS: Six studies that evaluated the initial lymphoma staging in 116 patients were included. WB-MRI and FDG-PET/CT agreed in 90.5% of the cases (κ = 0.871; P < 0.0001). In most of the studies, when there was disagreement between the methods, WB-MRI overstaged in relation to FDG-PET/CT. The sensitivity of WB-MRI and FDG-PET/CT, in comparison with the clinical-radiological standard, ranged from 59 to 100% and from 63 to 100% respectively. CONCLUSION: WB-MRI is a highly sensitive method for initial lymphoma staging. It has excellent agreement with FDG-PET/CT and is a great alternative for managing lymphoma patients, without using ionizing radiation or an intravenous contrast agent. .

CONTEXTO E OBJETIVO: A tomografia por emissão de pósitrons com 2-[18F]-fluoro-2-deoxi-D-glicose (FDG-PET/CT) tem sido defendida como método de escolha para o estadiamento do linfoma por realizar o estudo do corpo inteiro com boa sensibilidade para detecção das áreas acometidas e por combinar as capacidades de avaliação anatômica e funcional. Com os avanços tecnológicos, a ressonância magnética tem se apresentando como alternativa à FDG-PET/CT. Esta revisão sistemática com metanálise visa comparar a ressonância magnética de corpo inteiro (WB-MRI) com difusão com a FDG-PET/CT no estadiamento do linfoma. TIPO DE ESTUDO E LOCAL: Revisão sistemática de estudos de acurácia diagnóstica conduzida em universidade pública. MÉTODOS: Foi conduzida uma busca nos bancos de dados Medline, Embase, Scopus e Lilacs por estudos publicados até setembro de 2013 comparando a WB-MRI com a FDG-PET/CT no estadiamento do linfoma. As referências bibliográficas dos estudos incluídos foram checadas com a finalidade de encontrar citações adicionais relevantes. RESULTADOS: Foram incluídos seis estudos que avaliaram o estadiamento inicial do linfoma de 116 pacientes. A WB-MRI e a FDG-PET/CT concordaram em 90,5% dos casos (κ = 0,871; P < 0,0001). Na maioria dos estudos, quando houve discordância, a WB-MRI estabeleceu estadiamento superior à FDG-PET/CT. A sensibilidade da WB-MRI e da FDG-PET/CT, em relação ao padrão clínico-radiológico, variou de 59% a 100% e de 63% a 100%, respectivamente. CONCLUSÃO: A WB-MRI apresenta alta sensibilidade no estadiamento inicial do linfoma, excelente concordância com a FDG-PET/CT e representa uma ótima alternativa no manejo de pacientes com linfoma, sem utilizar radiação ionizante ou meio de contraste intravenoso. .