Roberto Pinto Paes

Cranial vault lymphoma: a systematic review of five patients

Bone involvement is a common finding in many types of lymphoma (Clin Oncol 9(3): 195–196, 1997). However, cranial vault affliction has been regarded as an exceedingly rare presentation, particularly in the case of primary lymphoma (J Neurosurg 108(5): 1018–1020, 2008). Our objective is to describe a series of five immunocompetent patients with histologically confirmed cranial vault lymphoma (CVL), and to conduct a systematic review of the current literature. Our review points out identical imaging patterns in most of the lesions for all reported CVL cases, despite their different histological subtypes. This typical pattern can be seen on computed tomography (CT) scans and magnetic resonance imaging (MRI) as an expansive tumor that affects all three compartments of the cranial vault, including the scalp, skull bone, and pachymeninges, even in the absence of osteolysis. We argue that the absence of osteolysis might enhance diagnostic capability. In the appropriate clinical setting, these features represent important disease characteristics that may help with an earlier diagnosis. Large B-cell lymphoma was the most common subtype of primary CVL.

World Health Organization Classification of tumors of hematopoietic and lymphoid tissues, 4th edition, 2008 - major changes from the 3rd edition, 2001

The World Health Organization (WHO) classification of Tumors of Hematopoietic and Lymphoid Tissues (4th edition, 2008) represents an update of the 3rd edition, 2001.2 A summary of these changes in myeloproliferative disorders, myelodisplastic syndrome, acute myeloid leukemias, B and T precursor cell neoplasms, and mature B, T and NK cell neoplasms is presented below. Understanding the molecular genetic changes and the results achieved with innovative therapeutic approaches in these groups of diseases require continuous reassessment of its classification, justifying the major changes discussed here 1,3-5 .

Secondary infiltration of the central nervous system in patients with diffuse large B-cell lymphoma

Objective To investigate the incidence and risk factors of infiltration of the central nervous system after the initial treatment of diffuse large B-cell lymphoma in patients treated at Santa Casa de Misericórdia de São Paulo. Methods A total of 133 patients treated for diffuse large B-cell lymphoma from January 2001 to April 2008 were retrospectively analyzed in respect to the incidence and risk factors of secondary central nervous system involvement of lymphoma. Intrathecal prophylaxis was not a standard procedure for patients considered to be at risk. This analysis includes patients whether they received rituximab as first-line treatment or not. Results Nine of 133 (6.7%) patients developed central nervous system disease after a mean observation time of 29 months. The median time to relapse or progression was 7.9 months after diagnosis and all but one patient died despite the treatment administered. Twenty-six (19.5%) patients of this cohort received rituximab as first-line treatment and nine (7.1%) received intrathecal chemoprophylaxis. Of the nine patients that relapsed, seven (77.7%) had parenchymal central nervous system involvement; seven (77.7%) had stage III or IV disease; one (11.1%) had bone marrow involvement; two (22.2%) had received intrathecal chemoprophylaxis; and 3 (33.3%) had taken rituximab. In a multivariate analysis, the risk factors for this infiltration were being male, previous use of intrathecal chemotherapy and patients that were refractory to initial treatment. Conclusion Central nervous system infiltration in this cohort is similar to that of previous reports in the literature. As this was a small cohort with a rare event, only three risk factors were important for this infiltration

Classificação dos tumores hematopoéticos e linfoides de acordo com a OMS: padronização da nomenclatura em língua portuguesa, 4ª edição

INTRODUCTION: The World Health Organization (WHO) classification of hematopoietic and lymphoid tissue (4th edition, 2008) tumors constitutes an updated review of the 3rd edition published in 2001. The translation of the nomenclature used to describe the entities should be clear, precise and uniform so that clinicians, pathologists and researchers involved in the onco-hematopathological area may identify them accurately. OBJECTIVE: With this purpose, the authors present an updated proposal and a terminological standardization in Portuguese based on WHO/2008

Frequency of EBV associated classical Hodgkin lymphoma decreases over a 54-year period in a Brazilian population

The epidemiology of classical Hodgkin lymphoma varies significantly in populations with different socioeconomic conditions. Among other changes, improvement in such conditions leads to a reduction in the association with EBV infection and predominance of the nodular sclerosis subtype. This study provides an overview of the epidemiology of 817 cases of classical Hodgkin lymphoma diagnosed in five reference hospitals of the State of Sao Paulo, Brazil, over 54 years (1954–2008). The cases were distributed in 3 periods (1954–1979; 1980–1999; and 2000–2008). EBV-positive cases decreased from 87% to 46%. In children and adolescents (<15 years) and in young adults (15–45 years), EBV-positive cases decreased respectively from 96% to 64%, and from 85% to 32%. The percentage of male patients declined from 80% to 58%. In older patients (>45 years), the decrease in EBV infection was not significant. Nodular Sclerosis was the most common subtype in all periods. These results support the hypothesis that, in the Brazilian State of Sao Paulo, classical Hodgkin lymphoma has changed and now shows characteristics consistent with Pattern III observed in populations that experienced a similar socioeconomic transition.

Classificação da Organização Mundial da Saúde para os tumores dos tecidos hematopoético e linfoide, 4a edição, 2008 - principais modificações introduzidas em relação à 3a edição, 2001

The World Health Organization (WHO) Classification of tumors of hematopoietic and lymphoid tissues (4th edition, 2008)1 presents an updated version of the 3rd edition published in 20012. A summary of these changes relates to the groups of chronic myeloproliferative disorders, myelodisplasia, acute myeloid leukemias, neoplasms of precursor B and T cells and neoplasms derived of mature B, T and NK cells. A better understanding of molecular genetic changes and results achieved with innovative therapeutic approaches in these groups of diseases requires constant reassessment of the classifications, supporting the major changes discussed here, including interesting comments from literature1, 3-5.

Manifestação bucal de linfoma difuso de grandes células B

O linfoma de celulas B acomete cerca de 90% de todos os casos de linfoma. O aparecimento da lesao bucal deste tipo de linfoma e pouco comum e raras vezes se manifesta em regiao anterior de mandibula. Neste relato de caso, o paciente de 22 anos, sexo masculino, com diagnostico de linfoma de grandes celulas B em mediastino e pleura, apos um ano de tratamento apresentou lesao nodular em regiao anterior de mandibula, comprometendo os dentes incisivos inferiores. O diagnostico histologico e imuno-histoquimico confirmou a forma secundaria do linfoma. O paciente foi submetido a tratamento quimioterapico com regressao completa da lesao mandibular e esta sob acompanhamento odontologico reabilitador.

Neuroendocrine tumors: An epidemiological study of 250 cases at a tertiary hospital

OBJECTIVE To compare the frequency of neuroendocrine tumors in our service with that reported in the literature considering age, gender, location, degree of differentiation and increase in incidence by means of a retrospective study. METHOD Search of variables from a database of neuroendocrine tumor cases diagnosed at the Department of Pathological Sciences, Hospital da Santa Casa de São Paulo over the past 10 years, relating them to epidemiological data such as gender, age, distribution across organs, most-used immunohistochemical markers and presence or absence of either lymph node or distant metastases. RESULTS In all, 250 cases were reviewed, 133 involving females, predominantly in the 61-70 age range. The lung was the most frequent site, followed by the stomach. CD56, synaptophysin and chromogranin were the immunohistochemical markers used most often and to a lesser extent Ki67, a marker of cell proliferation that indicates a higher or lower degree of histological malignancy. Metastases, either in lymph nodes and/or distant sites, were found in 44 cases (17.6%). CONCLUSION The results were largely consistent with those in the literature, including age group, gender and location. Most metastases originated from high-grade tumors, with high Ki67 levels and greater impairment of the liver. However, only 36.4% of the cases had Ki67 index. Reevaluation of the Ki67 proliferative index using image analysis in doubtful cases will allow for a correlation between progression and prognosis.

Recidiva tardia de linfoma da zona marginal

O linfoma de zona marginal e um linfoma de baixo grau com curso clinico indolente e potencial de recidiva.1,2 Apresentamos um caso de recidiva tardia apos 25 anos de aparente remissao completa, levantando a possibilidade de recidiva de doenca preexistente ou desenvolvimento de novo clone neoplasico.