ABSTRACT Objective To investigate the expression of human papillomavirus (HPV), p16, p53, and p63 in non-schistosomiasis-related squamous cell carcinoma of the bladder and to develop an accurate and automated tool to predict histological classification based on clinicopathological features. Methods Twenty-eight patients with primary bladder pure squamous cell carcinoma who underwent cystectomy or transurethral resection of bladder tumor (TURBT) for bladder cancer between January 2011 and July 2017 were evaluated. Clinical data and follow-up information were obtained from medical records. Formalin-fixed, paraffin-embedded surgical specimens were used for immunohistochemical staining for p16, p53, and p63. Human papillomavirus detection was evaluated by PCR. Statistical analysis was performed, and statistical significance was set at p<0.05. Finally, decision trees were built to classify patients’ prognostic features. Leave-one-out cross-validation was used to test the generalizability of the model. Results Neither direct HPV detection nor its indirect marker (p16 protein) was identified in most cases. The absence of p16 was correlated with less aggressive histological grading (p=0.040). The positive p16 staining detection found only in pT1 and pT2 cases in our sample suggests a possible role for this tumor suppressor protein in the initial stages of bladder squamous cell carcinoma. The decision trees constructed described the relationship between clinical features, such as hematuria/dysuria, the level of tumor invasion, HPV status, lymphovascular invasion, gender, age, compromised lymph nodes, and tumor degree differentiation, with high classification accuracy. Conclusion The algorithm classifier approach established decision pathways for semi-automatic tumor histological classification, laying the foundation for tailored semi-automated decision support systems for pathologists.

Abstract Introduction The deficiency of ADA2 (DADA2) is a rare autoinflammatory disease provoked by mutations in the ADA2 gene inherited in a recessive fashion. Up to this moment there is no consensus for the treatment of DADA2 and anti-TNF is the therapy of choice for chronic management whereas bone marrow transplantation is considered for refractory or severe phenotypes. Data from Brazil is scarce and this multicentric study reports 18 patients with DADA2 from Brazil. Patients and methods This is a multicentric study proposed by the Center for Rare and Immunological Disorders of the Hospital 9 de Julho - DASA, São Paulo - Brazil. Patients of any age with a confirmed diagnosis of DADA2 were eligible for this project and data on clinical, laboratory, genetics and treatment were collected. Results Eighteen patients from 10 different centers are reported here. All patients had disease onset at the pediatric age (median of 5 years) and most of them from the state of São Paulo. Vasculopathy with recurrent stroke was the most common phenotype but atypical phenotypes compatible with ALPS-like and Common Variable Immunodeficiency (CVID) was also found. All patients carried pathogenic mutations in the ADA2 gene. Acute management of vasculitis was not satisfactory with steroids in many patients and all those who used anti-TNF had favorable responses. Conclusion The low number of patients diagnosed with DADA2 in Brazil reinforces the need for disease awareness for this condition. Moreover, the absence of guidelines for diagnosis and management is also necessary (t).

Resumo Introdução A qualidade das informações dos Registros Hospitalares de Câncer (RHC) necessita de avaliação quanto à cobertura, completitude e concordância da causa básica(CB) com o Sistema de Informações sobre Mortalidade (SIM). Objetivo Avaliar a qualidade dos RHC nas duas unidades hospitalares do Instituto Mário Penna: Hospitais Mário Penna (HMP) e Luxemburgo (HL), Belo Horizonte, Minas Gerais, em 2016 e 2017, nos atributos mencionados. Método Por captura-recaptura (RHC x RHC), avaliaram-se, por unidade, cobertura, completitude da variável “óbito por câncer” e concordância da a (CB) com a causa da pesquisa (CP). Por relacionamento determinístico (RHC x SIM) avaliaram-se cobertura e concordância da CB. Resultados A cobertura dos RHC foi boa eexcelente (88,8% e 95,3%); a completitude foi ruim (34,6% e 32,6%) no HMP e HL respectivamente; por capítulo da CID-10, não houve concordância da CB com a CP. Observaram-se excelentes cobertura (94,7%) e concordância (94,5%) entre CP e SIM; observou-se sub-registro de 38 neoplasias no SIM, com reclassificação de causas pouco úteis. Conclusão A aplicação das técnicas de captura-recaptura e relacionamento determinístico contribuiu para a melhora da qualidade da informação dos RHC, com redução da incompletude nos RHC e correção da CB nos RHC e no SIM.

Abstract Bakground The quality of information from the Hospital Cancer Records (HRC) needs to be evaluated regarding coverage, completeness and agreement between the underlying cause (UC) as registered in the HRC and the Mortality Information System (SIM). Objective To assess the quality of the HRC in the two Instituto Mário Penna hospitals: Mário Penna (HMP) and Luxemburgo (HL) in Belo Horizonte, Minas Gerais, between 2016-2017. Method By capture-recapture (RHC x RHC), we assessed coverage, completeness of the “cancer death” variable and agreement between underlying cause (UC) with the cause of the research (CR), in each hospital. Deterministic relationship (RHC x SIM) was used to asses UC coverage and agreement between systems. Results The coverage of deaths at the HRC was good/excellent (88.8% and 95.3%); completeness was poor (34.6% and 32.6%) in HMP and HL respectively; per ICD-10 chapter, there was no agreement between CB and CP. Excellent coverage (94.7%) and agreement (94.5%) of CR and SIM were observed; 38 neoplasms were under-reported in the SIM, with reclassification of less useful causes. Conclusion Applying capture-recapture and deterministic linkage techniques contributed in improving the quality of information in the HRC, with a reduction in incompleteness in the HRC and correction of the UC in both HRC and SIM.

ABSTRACT Introduction and Objective Radical cystectomy (RC) with pelvic lymph node dissection is the standard treatment for muscle invasive bladder cancer and the oncologic outcomes following it are directly related to disease pathology and surgical technique. Therefore, we sought to analyze these features in a cohort from a Brazilian tertiary oncologic center and try to identify those who could negatively impact on the disease control. Patients and Methods We identified 128 patients submitted to radical cystectomy, for bladder cancer treatment, from January 2009 to July 2012 in one oncology tertiary referral public center (Mario Penna Institute, Belo Horizonte, Brazil). We retrospectively analyzed the findings obtained from their pathologic report and assessed the complications within 30 days of surgery. Results We showed similar pathologic and surgical findings compared to other large series from the literature, however our patients presented with a slightly higher rate of pT4 disease. Positive surgical margins were found in 2/128 patients (1.5%). The medium number of lymph nodes dissected were 15. Major complications (Clavien 3 to 5) within 30 days of cystectomy occurred in 33/128 (25.7%) patients. Conclusions In the management of invasive bladder cancer, efforts should focus on proper disease diagnosis and staging, and, thereafter, correct treatment based on pathologic findings. Furthermore, extended LND should be performed in all patients with RC indication. A critical analysis of our complications in a future study will help us to identify and modify some of the factors associated with surgical morbidity.

OBJETIVO: Pesquisar se o tempo de compressão venosa de até 120 horas pós-escleroterapia é suficiente para evitar reperfusão nas veias tratadas; se há relação entre a intensidade inflamatória na parede venosa e tecidos adjacentes e o tamanho do trombo venoso; se a intensidade da inflamação pós-escleroterapia varia com o tempo de compressão; se há relação entre a presença de hemossiderina nos tecidos adjacentes ao vaso esclerosado e coágulo venoso. MÉTODOS: Utilizaram-se 28 coelhos, machos, distribuídos em quatro grupos (0, 24, 72 e 120). Em todos os animais foram administrados 0,25 ml de solução de polidocanol 1% e, como controle, 0,25 ml de solução de cloreto de sódio 0,9% na veia marginal dorsal das orelhas direita e esquerda, respectivamente. Realizou-se compressão mecânica em trecho da veia perfundida, exceto nos animais do grupo 0. O tempo de compressão variou de 0 a 120 horas nos grupos. Realizou-se exame anatomopatológico de trecho das veias marginais dorsais direita e esquerda de todos os animais. RESULTADOS: Não houve diferenças significativas nos diversos tempos de compressão, tanto no grau de trombose venosa como na intensidade inflamatória, em ambas as orelhas, nos diversos grupos. Observou-se correlação positiva e significativa entre intensidade inflamatória e tamanho do trombo e na ocorrência de trombos e hemossiderina. CONCLUSÕES: O tempo de até 120 horas de compressão não é suficiente para evitar reperfusão nos vasos esclerosados. A intensidade da inflamação nos tecidos tem relação com o tamanho do trombo, mas não com o tempo de compressão. A presença de hemossiderina nos tecidos adjacentes ao vaso submetido à esclerose está relacionada com a presença de coágulo venoso.

PURPOSE: Research whether a post-sclerotherapy venous compression period of up to 120 hours is sufficient to avoid reperfusion in treated veins; whether there is a relationship between the inflammatory intensity in venous walls and adjacent tissue and the size of venous thrombosis; whether the intensity of the post-sclerotherapy inflammation varies with the period of compression; whether there is a relationship between the presence of hemosiderin in the tissues adjacent to the sclerosing blood vessels and venous blood clots. METHODS: Twenty eight rabbits, all male, were utilized, distributed into four groups (0, 24, 72 and 120). All the animals were administered with 0.25 ml of 1% polidoconal solution and, as a control, 0.25 ml of 0.9% sodium chloride solution in the marginal dorsal vein of the right and left ears, respectively. Mechanical compression was applied to the perfused stretch of the vein, except for the animals in group 0. The period of compression varied from 0 to 120 hours in the groups. An anatomopathological examination of the section of the right and left marginal dorsal veins of all the animals was conducted. RESULTS: There was no significant difference among the various compression periods, both in terms of the degree of vein thrombosis and in the inflammatory intensity in both ears of the various groups. A positive and significant correlation was observed between the inflammatory intensity and the size of the thrombus and in the occurrence of thrombi and hemosiderin. CONCLUSIONS: A compression period of up to 120 hours is not sufficient to prevent reperfusion in sclerosing blood vessels. The intensity of tissue inflammation is related to the size of the thrombus, but not to the compression period. The presence of hemosiderin in the tissues adjacent to the vessels subjected to sclerosis is related to the presence of venous coagulation.

OBJETIVO: Determinar os fatores prognósticos associados com a recorrência local nos pacientes submetidos a tratamento de sarcoma de tecidos moles em extremidades. MÉTODOS: Foram avaliados retrospectivamente 30 pacientes tratados por ressecção cirúrgica de sarcoma de tecidos moles localizados em extremidades, com seguimento 36,5 ± 12,2 meses. Foram avaliados como fatores prognósticos: idade, sexo, localização, profundidade, localização em compartimentos anatômicos, tamanho, manipulação prévia ao tratamento definitivo, margens cirúrgicas, grau de malignidade histológica, presença de necrose e invasão vascular à histologia. Estes fatores foram correlacionados com a ocorrência de recidiva local do tumor por uma análise univariada e multivariada, sendo considerados significativos valores de p = 0,05. RESULTADOS: A recorrência local apresentou correlação significativa, tanto na análise univariada como multivariada, com a localização extra-compartimental (p = 0,001) e com o alto grau de malignidade (p = 0,024). Não houve associação com a idade (p = 1,000), com o sexo (p = 1,000), com a localização em segmentos corporais (p = 0,544), com a profundidade (p = 0,288), com o tamanho (p = 0,694), com as margens cirúrgicas (p = 0,384), com a manipulação prévia (p = 0,461), com a presença de necrose (p = 0,461) ou invasão vascular (p = 1,000). CONCLUSÃO: A recorrência local apresentou correlação com a localização extra-compartimental e com o alto grau de malignidade dos tumores.

OBJECTIVE: To determinate the prognostic factors associated with the local relapse in patients treated for soft tissue sarcoma in extremities. METHODS: Retrospectively, 30 patients were evaluated after being treated with surgical resection of soft tissue sarcoma located in extremities, with a outcome of 36,5 ± 12,2 months. The prognostic factors evaluated were: age, sex, location, depth, location in the anatomic compartments, size, tumor previous manipulation, surgical margins, grade of histological malignance, presence of histological necrosis or histological vascular invasion. These factors were correlated with the local relapse of the tumor with a univariated and multivariated analysis with significance when p = 0,05. RESULTS: Local relapse presented significant correlaction in univariated and multivariated analysis with extra-compartimental location (p = 0,001) and with high grade histological malignance (p = 0,001). There was no significant association with age (p = 1,000), sex (p = 1,000), body location (p = 0,544), depth (p = 0,288), size (p = 0,694), surgical margins (p = 0,384), previous manipulation (p = 0,461), necrosis (p = 0,461) or with the presence of vascular invasion (p = 1,000). CONCLUSION: Local relapse presents correlaction with extra-compartimental location and with high grade malignancy tumors.

OBJETIVO: Avaliar um colar de isolamento crânio-cervical, para uso em coelhos, quanto aos seguintes aspectos: eficácia da fixação crânio-cervical em até 120 h; facilidade de manuseio; liberdade do animal para deambular e ingerir alimentos; integridade física do animal durante e depois do uso do dispositivo. MÉTODOS: A partir do colar elizabetano confeccionou-se um colar de isolamento crânio-cervical para uso em coelhos, com as seguintes modificações: amplo corte no aparelho, em forma de meia lua, coincidindo com a boca do animal; divisão interna do dispositivo em dois compartimentos, para isolar e imobilizar as orelhas, individualmente; perfurações múltiplas no compartimento superior para melhor ventilação; adaptação de correia cérvico-torácica, para fixar o colar na região cervical, mantendo-se as presilhas de fechamento no dorso do animal. O dispositivo foi utilizado em 18 coelhos machos da raça Nova Zelândia, por período de até 120 horas consecutivas. RESULTADOS: O aparelho foi eficaz no isolamento crânio-cervical e das orelhas de coelhos, não interferiu nas funções respiratórias do animal, na deglutição de alimentos e não causou trauma físico, permitindo a livre deambulação e conforto no repouso. Foi de fácil e rápido manuseio na colocação e retirada, permanecendo no local sem necessidade de reposicionamento por até 120 horas. CONCLUSÃO: O colar de isolamento crânio-cervical modificado possibilitou a realização do experimento com isolamento das orelhas dos coelhos, com facilidade no manuseio, sem causar limitações ao animal em relação à ingestão de alimentos e deambulação.

PURPOSE: To evaluate the craniocervical isolation collar, for use in rabbits, with regard to the following aspects: effectiveness of the craniocervical attachment for up to 120 hours; ease of handling; freedom of the animal to move about and ingest food; and the physical integrity of the animal during and after use of the device. METHODS: Starting from an Elizabethan collar, a craniocervical collar was prepared for use in rabbits with the following modifications: a wide cut in the device in the shape of a half-moon, coinciding with the animal's mouth; inner division of the device into two compartments to individually isolate and immobilize the ears; multiple perforations in the upper compartment for better ventilation; adaptation of the cervical-thoracic band to attach the collar to the cervical region, keeping the fasteners on the animal's back. The device was used on 18 male New Zealand rabbits for up to 120 consecutive hours. RESULTS: The device was effective in the craniocervical isolation and separation of ears in rabbits without interfering with the animals' respiration, ability to swallow food, or causing physical trauma, while allowing the animals to move about freely and rest comfortably. Attaching and removing the device was easy and quick and it remained in place for up to 120 hours without the need to reposition it. CONCLUSION: The modified craniocervical isolation collar made it possible to conduct the experiment with isolation of the rabbits' ears, ease of handling, and without causing limitations in relation to the animals' ability to eat and move about freely.

OBJETIVOS: O crescimento de um rim em resposta à perda do outro rim recebe o nome de hipertrofia renal compensatória. Entretanto, os mecanismos que regulam esse processo ainda não foram esclarecidos. Neste estudo, avaliaram-se os efeitos da icterícia obstrutiva na morfologia e na função do rim remanescente após nefrectomia unilateral. MÉTODOS: Foram estudados 40 ratos Wistar (250g-300 g), distribuídos nos seguintes grupos: Grupo 1 - operação simulada; Grupo 2 - nefrectomia direita; Grupo 3 - ligadura do ducto biliar; Grupo 4 - ligadura do ducto biliar e nefrectomia direita. Os animais foram mortos após 20 dias, quando foi colhido sangue para exames laboratoriais e feita a retirada dos rins e do fígado. Os rins foram pesados para avaliação do crescimento compensatório. Rins e fígado foram estudados histologicamente por microscopia óptica. RESULTADOS: Não houve diferença no aumento do peso renal relativo entre os animais dos Grupos 2 e 4 (p = 0,4). Os animais submetidos à ligadura do ducto biliar isoladamente (Grupo 3) apresentaram maior peso renal relativo quando comparados com os animais submetidos à operação simulada (Grupo 1) (p = 0,011). As alterações histológicas encontradas foram dilatação capilar da camada medular nos rins hipertrofiados e modificações típicas de colestase nos fígados dos animais ictéricos. Cirrose foi encontrada apenas nos animais submetidos à nefrectomia. CONCLUSÃO: O peso e a função do rim remanescente após nefrectomia unilateral não são afetados pela icterícia obstrutiva.

OBJECTIVE: The removal of one kidney results in a compensatory growth of the remnant kidney. However, the mechanism that underlies this hypertrophic response is not understood. In this study we assessed the influence of obstructive jaundice on renal morphology and function after unilateral nephrectomy. METHODS: Forty Wistar rats (250-300 g) were randomly divided into four groups: Group 1 - sham operation, Group 2 - right nephrectomy, Group 3 - common bile duct ligation, Group 4 - common bile duct ligation and right nephrectomy. After 20 days, blood was collected for biochemical studies and the animals were killed. Their kidneys and livers were removed and weighed. Histological evaluation of both organs was carried out. RESULTS: No difference in the kidney/body weight ratio was observed for animals undergoing right nephrectomy (Groups 2 and 4) (p = 0.4). Animals submitted to bile duct ligation only (Group 3) presented a higher kidney/body weight ratio than animals submitted to sham operation (Group 1) (p = 0.011). Vasodilatation was identified in the medulla of the hypertrophied kidneys and histological alterations were found in the livers of the jaundiced animals. Cirrhosis occurred only in nephrectomized rats. CONCLUSION: Cholestasis does not affect the weight, function or the compensatory renal growth of the remnant kidney after unilateral nephrectomy.

OBJETIVO: Determinar os fatores prognósticos relacionados com o desenvolvimento de metástases a distância nos pacientes com diagnóstico de sarcoma de tecidos moles (STM) em extremidades. MÉTODOS: Foram avaliados 30 pacientes tratados por ressecção cirúrgica de STM, com seguimento médio de 36,5 ± 12,2 meses, utilizando como fatores prognósticos: idade, sexo, localização, profundidade, localização em compartimentos anatômicos, tamanho, manipulação prévia ao tratamento definitivo, margens cirúrgicas, grau de malignidade histológica, presença de necrose e invasão vascular à histologia. Esses fatores foram correlacionados com o desenvolvimento de metástases por análise univariada e multivariada, sendo considerados significativos valores de p < 0,05. RESULTADOS: Na análise univariada houve correlação do desenvolvimento de metástases com a localização extracompartimental (p = 0,002), com o tamanho > 10cm (p = 0,007), com o alto grau de malignidade (p = 0,007), com a presença de necrose (p = 0,002) e com a presença de invasão vascular (p = 0,034). A idade (p = 1,000), o sexo (0,709), a localização em segmentos corporais (p = 0,298), a profundidade (p = 0,288), a margem cirúrgica (p = 0,419) e a manipulação prévia do tumor (p = 1,000) não apresentaram correlação com a ocorrência de metástases a distância. Na análise multivariada apenas a localização extracompartimental (p = 0,008), o tamanho (p = 0,018) e a presença de invasão vascular (p = 0,043) foram significativos. CONCLUSÃO: O desenvolvimento de metástases a distância nos pacientes com sarcoma de tecidos moles depende da localização extracompartimental, do tamanho (> 10cm), do alto grau de malignidade histológica, da presença de necrose e da invasão vascular à histologia.

OBJECTIVE: To determine the prognostic factors related to the development of remote metastases in patients with the diagnosis of soft tissue sarcoma (STS) in the extremities. METHODS: 30 patients treated with surgical resection of STS with a mean follow-up of 36.5 ± 12.2 months were evaluated using the following prognosis factors: age, gender, location, depth, location in anatomic compartments, size, handling before final treatment, surgical borders, degree of histological malignancy, presence of necrosis, and histologically-verified vascular invasion. Such factors were correlated to the development of metastases by univariate analysis, values of p < 0.05 being considered significant. RESULTS: The univariate analysis showed a correlation of the development of metastases and the extra-compartmental location (p = 0.002), size > 10 cm (p = 0.007), the high degree of malignancy (p = 0.007), the presence of necrosis (p = 0.002) and the presence of vascular invasion (p = 0.034). Age (p = 1.000), gender (0.709), location in body segments (p = 0.298), depth (p = 0,288), surgical border (p = 0.419), and prior handling of the tumor (p = 1.000) did not show a correlation with the occurrence of remote metastases. In the multivariate analysis, only the extra-compartmental location (p = 0.008), size (p = 0.018), and the presence of vascular invasion (p = 0.043) were significant. CONCLUSION: The development of remote metastases in patients with soft tissue sarcoma depends on the extra-compartmental location, on the size (> 10 cm), on the high degree of malignancy seen in histology, on the presence of necrosis, and on vascular invasion seen in histology.

É relatado caso de elastólise da derme média em paciente do sexo feminino, de 37 anos, que apresentava áreas de enrugamento fino da pele, principalmente nas flexuras e pápulas perifoliculares com aspecto em peau d´orange na região paravertebral, conferindolhe aspecto precocemente envelhecido. A histologia mostrava fragmentação e rarefação das fibras elásticas restritas à porção média da derme.

A case of mid-dermal elastolysis is reported in a 37-year-old female patient, who presented skin areas with fine wrinkles, mainly in fold areas and also perifollicular papules similar to peau d'orange in the paravertebral area, lending her a prematurely elderly appearance. Histological examination revealed fragmentation and rarefaction of elastic fibers, restricted to the mid-dermis portion.

É relatado caso de paciente de 24 anos, portador de traço falciforme, com imagem sólida no rim direito, submetido a nefrectomia radical que revelou tumor, cujo exame anatomopatológico permitiu o diagnóstico de carcinoma medular do rim. Os autores discutem aspectos dessa neoplasia, tais como freqüência, patogênese, apresentação clínica, histopatologia e evolução.

We report the case of a 24-year-old patient who presented a left kidney tumor that was diagnosed as a medullary renal cell carcinoma. The following aspects of this neoplasia are discussed in this communication: frequency, pathogenesis, clinical presentation, histopathological findings, differential diagnosis and follow-up.

É relatado o caso de paciente de 20 anos com hepatoesplenomegalia, febre e grave insuficiência hepática. Estudos histopatológicos e imunohistoquímicos de fragmentos hepáticos obtidos em necropsia permitiram o diagnóstico de leishmaniose visceral e hepatite fulminante pelo vírus B. Os autores apontam possível influência da resposta imunitária relacionada com a leishmaniose visceral no desenvolvimento de grave lesão hepática pelo vírus B da hepatite.

The case of a 20-year-old man with hepatoesplenomegaly, fever and severe hepatic insufficiency is reported. Histopathological and immunohistochemical studies of tissue specimens obtained at autopsy led to diagnosis of visceral leishmaniasis and fulminant hepatitis B. The authors pointed out a possible influence of immunological response related with visceral leishmaniasis in development of severe hepatic involvement by hepatitis B virus