A case of non-necrotising herpetic retinitis.

In developed countries, the main etiology of posterior uveitis is of infectious origin, Herpes is the most common viral agent as it has a wide spectral of ocular manifestations. These manifestations could depend on the patient's immunologic state, ranging from a mild focal form of non-necrotizing herpetic renitis (NNHR) to a severe form of Acute retinal necrosis (ARN). We present a case of NNHR by VHS 2 and the different differential diagnostics previous to its diagnosis: atypical ocular toxoplasmosis, ocular tuberculosis and ARN. During its evolution, it presented a drastic drop in the visual acuity despite of the antiviral treatment, and with systemic corticoids and the injury's clinical improvement. This event led to reevaluate the suspected entities, establishing the NNHR as a definitive diagnostic by exclusion that constituted a challenging diagnostic.

Un caso de retinitis herpética no necrosante / A case of non-necrotising herpetic retinitis

En países en vías de desarrollo la principal etiología de uveítis posterior es de origen infeccioso, siendo el herpes virus el agente viral más común, con un amplio espectro de manifestaciones oculares que puede depender del estado inmunológico del paciente: desde una forma leve focal de retinitis herpética no necrosante (RHNN) hasta una forma severa de necrosis retiniana aguda. Presentamos un caso de RHNN por VHS 2 y los diferentes diagnósticos diferenciales planteados previos a su diagnóstico: toxoplasmosis ocular atípica, tuberculosis ocular y necrosis retiniana aguda. Durante su evolución presentó una caída súbita de la agudeza visual a pesar del tratamiento antiviral y con corticoides sistémicos y de la mejoría clínica de la lesión. Este evento conllevó a replantear las entidades sospechadas, estableciendo la RHNN como diagnóstico definitivo por exclusión, lo cual constituyó un reto diagnóstico. (AU)

In developed countries, the main origin of posterior uveitis is an infection. Herpes is the most common viral agent, as it has a wide spectrum of ocular manifestations. These manifestations may depend on the immunological state of the patient, and range from a mild focal form of non - necrotising herpetic retinitis (NNHR) to a severe form of acute retinal necrosis (ARN). A case of NNHR due to Herpes simplex virus type 2 (HSV-2) is reported, along with the different differential diagnostics prior to its diagnosis: atypical ocular toxoplasmosis, ocular tuberculosis, and ARN. During its course, despite the antiviral treatment, systemic corticoids and the clinical improvement of the injury, there was a drastic drop in the visual acuity. This event led to the re-evaluation of the suspected entities, establishing the NNHR as a definitive diagnostic by exclusion, which was a diagnostic challenge (AU)

A case of non-necrotising herpetic retinitis. / Un caso de retinitis herpética no necrosante.

In developed countries, the main origin of posterior uveitis is an infection. Herpes is the most common viral agent, as it has a wide spectrum of ocular manifestations. These manifestations may depend on the immunological state of the patient, and range from a mild focal form of non - necrotising herpetic retinitis (NNHR) to a severe form of acute retinal necrosis (ARN). A case of NNHR due to Herpes simplex virus type 2 (HSV-2) is reported, along with the different differential diagnostics prior to its diagnosis: atypical ocular toxoplasmosis, ocular tuberculosis, and ARN. During its course, despite the antiviral treatment, systemic corticoids and the clinical improvement of the injury, there was a drastic drop in the visual acuity. This event led to the re-evaluation of the suspected entities, establishing the NNHR as a definitive diagnostic by exclusion, which was a diagnostic challenge.

Beyond the vascular profile: conventional DWI, IVIM and kurtosis in the assessment of hepatocellular carcinoma.

OBJECTIVE: To describe the role of the Diffusion Weighted Imaging (DWI) in the assessment of hepatocellular carcinoma (HCC). MATERIALS AND METHODS: Several electronic databases were evaluated in the present review. The search included articles published from January 2010 to May 2019. The references of all articles were also evaluated. All titles and abstracts were assessed, and only the studies of DWI in patients with HCC were retained. RESULTS: HCC is the most common primitive hepatic cancer. The non-invasive radiological criteria for HCC diagnosis are based on the presence of the specific vascular profile characterized by contrast uptake during arterial phase, defined as arterial hyperenhancement, followed by washout in the venous/portal phase. However, arterial hyperenhancement and wash out appearance have a sensitivity rate of 50-60% in lesion smaller than 2 cm. Therefore, other functional parameters have been introduced in the detection and characterization of HCC nodules. DWI has been applied to liver imaging as an excellent tool for detection and characterization of focal liver lesions, increasing clinical confidence and decreasing false positives. The assessment of DW images can be done qualitatively and quantitatively, through the apparent diffusion coefficient (ADC) map. Intravoxel incoherent motion (IVIM) is a more sophisticated analysis, a biexponential model, to better defining the relationship between signal attenuation and increasing b value that separately reproduces tissue diffusivity and tissue perfusion. Traditionally DWI approach to analyze data is founded on the hypothesis that water molecules diffuse within a voxel following a single direction with a Gaussian behavior without any restriction. However, according to the presence of microstructures, water molecules within biologic tissues exhibits a non-Gaussian phenomena proposed by Jensen in 2005 called Diffusion Kurtosis Imaging (DKI). This approach assesses the kurtosis coefficient (K) that shows the deviance of diffusion from a Gaussian approach, and the diffusion coefficient (D) with the correction of non-Gaussian bias. DKI is an advanced DWI model that quantifies non-Gaussian behavior of diffusion and provides both a corrected ADC, as well as the excess kurtosis of tissue, a measure of the extent to which tissue diffusion deviates from a Gaussian pattern. It is believed that the DKI model is more sensitive to tissue microstructural complexity than standard DW. CONCLUSIONS: DWI should be an integral part of study protocol for HCC patients, considering the great advantages due to DWI and DWI-based approaches in detection and characterization of HCC.

Electrochemotherapy of cholangiocellular carcinoma at hepatic hilum: a case report.

OBJECTIVE: Cholangiocarcinoma (CCA) is the second most common primary hepatic malignancy after hepatocellular carcinoma. The current standard palliative treatment, chemotherapy regimen with gemcitabine and cisplatin, prolongs overall survival only of a few months. Established locoregional therapies are not a curative option or an alternative to surgery in the treatment of CCA. We report a case of a patient affected by a cholangiocellular carcinoma at hepatic hilum treated by Electrochemotherapy (ECT) at our oncologic center. CASE PRESENTATION: A 71 years old male affected by a CCA at hepatic hilum was treated with ECT according to ESOPE guidelines. No complications occurred during ECT procedure. The patient was discharged after 10 days. The functional MR evaluation at 2 and at 4 months post-treatment showed a significant response without significant post-treatment adverse events. The Computed tomography (CT) assessment after 18 months did not show progression of disease. CONCLUSIONS: ECT is safe and its use could be suggested as a palliative treatment of advanced neoplastic lesions in which radical surgical treatment is not possible.

Neoadjuvant FOLFIRI+bevacizumab in patients with resectable liver metastases from colorectal cancer: a phase 2 trial.

BACKGROUND: Preoperative treatment of resectable liver metastases from colorectal cancer (CRC) is a matter of debate. The aim of this study was to assess the feasibility and activity of bevacizumab plus FOLFIRI in this setting. METHODS: Patients aged 18-75 years, PS 0-1, with resectable liver-confined metastases from CRC were eligible. They received bevacizumab 5 mg kg(-1) followed by irinotecan 180 mg m(-)(2), leucovorin 200 mg m(-)(2), 5-fluorouracil 400 mg m(-)(2) bolus and 5-fluorouracil 2400 mg m(-)(2) 46-h infusion, biweekly, for 7 cycles. Bevacizumab was stopped at cycle 6. A single-stage, single-arm phase 2 study design was applied with 1-year progression-free rate as the primary end point, and 39 patients required. RESULTS: From October 2007 to December 2009, 39 patients were enrolled in a single institution. Objective response rate was 66.7% (95% exact CI: 49.8-80.9). Of these, 37 patients (94.9%) underwent surgery, with a R0 rate of 84.6%. Five patients had a pathological complete remission (14%). Out of 37 patients, 16 (43.2%) had at least one surgical complication (most frequently biloma). At 1 year of follow-up, 24 patients were alive and free from disease progression (61.6%, 95% CI: 44.6-76.6). Median PFS and OS were 14 (95% CI: 11-24) and 38 (95% CI: 28-NA) months, respectively. CONCLUSION: Preoperative treatment of patients with resectable liver metastases from CRC with bevacizumab plus FOLFIRI is feasible, but further studies are needed to define its clinical relevance.

An experimental study of tissue reaction to hyaluronic acid (Restylane) and polymethylmethacrylate (Metacrill) in the mouse.

The aging skin is a challenge for medical science. Plastic surgeons and dermatologists are called every day to solve problems like filling wrinkles or folds. The material used must be biocompatible because abnormal reactions may cause catastrophic results. This study analyzes the biological behavior of polymethylmethacrylate (Metacrill) and hyaluronic acid (Restylane), using a histopathologic study in mice. A prospective study was performed using 40 mice for each substance: polymethylmethacrylate or hyaluronic acid was injected into the right ear, the left ear been used as a control. Histopathologic analyses of the right ear, liver, and kidney were performed at intervals during the study and revealed the development of a granulomatous reaction with fibrosis and absorption of spheres and signs of liver and kidney sistematization for polymethylmethacrylate. A discrete cellular reaction, with less formation of fibrosis, and no giant cells were seen in the mice injected with hyaluronic acid.

Expression of laminin-5 γ2 chain in cutaneous pseudocarcinomatous hyperplasia.

BACKGROUND: Since the use of laminin-5 as a marker of invasiveness has been proposed by several authors, our objective was to compare the expression of this protein in pseudocarcinomatous hyperplasia and squamous cell carcinoma (SCC). METHODS: Sixty-four paraffin-embedded skin biopsy samples with diagnosis of epidermal hyperplasia (non-pseudocarcinomatous), pseudocarcinomatous hyperplasia, actinic keratosis/carcinoma in situ, microinvasive and frankly invasive SCC were obtained for immunohistochemical study. RESULTS: Adjacent normal epithelium and epidermal hyperplasia (non-pseudocarcinomatous) showed no staining. In pseudocarcinomatous hyperplasia, laminin-5 was positive, at least focally, in 14 of 16 (87.5%) samples and was concentrated in peripheral cells of elongated rete pegs and in migrating cells in dermis. In samples of microinvasive carcinoma (n = 7), the expression was observed in all cases and was concentrated in the leading edge of the tumor. All cases (n = 21) of frankly invasive SCC showed cells expressing laminin-5, at least focally. Well-differentiated areas of the tumor presented a pattern of expression in peripheral cells of tumor nests while a diffuse pattern of expression was observed in less differentiated areas. CONCLUSION: We showed that cytoplasmic laminin-5 expression should not be used as a criterion of malignancy and is not useful in distinguishing pseudocarcinomatous hyperplasia from microinvasive and well-differentiated SCC.

Can we tell the site of origin of metastatic squamous cell carcinoma? An immunohistochemical tissue microarray study of 194 cases.

To the workup of metastatic squamous cell carcinoma (SCC) of unknown primary, we studied an immunohistochemical panel including thyroid transcription factor (TTF-1), napsin A, villin, CDX-2, K903, CK5/6, p63, p16, CK7, and CK20. Using tissue microarray, we compared 194 SCC cases from the following sites: 35 lung, 34 skin, 14 cervix, 4 vagina, 16 vulva, 8 penis, 9 anus, 3 rectum, 10 esophagus, 4 bladder/urethra, and 57 SCC from various head and neck sites. p63 and K903 stained positively in 100% of cases, and CK5/6 in nearly 100% of cases, with the exception of 1 lung. CK7 was positive in 31.6% of all cases, with varying positivity according to the site. CK20 was negative in all cases except 1 lung. Napsin A was positive in 25.8% of lung, 7.7% of skin, 37.5% of penis, and 13.3% of tongue, and negative in all other sites. TTF-1 was positive only in 1 lung. p16 positivity ranged from 21.43% in vulva, to 75% in vagina and anus, and it was negative in lung, penis, bladder/urethra, and some head and neck. CDX-2 was negative in all cases except 1 vulva. Villin was negative in all cases. We conclude that immunohistochemistry has very limited value in determining the primary site of metastatic SCC. If lung is in the differential versus head and neck, esophagus, anorectal, or genital SCC, a panel including TTF-1, napsin A and p16 may be helpful, since positive TTF-1 and/or napsin A would favor lung primary, and positive p16 would favor an extrapulmonary site.

Comprometimento do sistema nervoso central na paracoccidioidomicose em paciente imunocompetente: relato de caso / Central nervous system involvement of paracoccidioidomycosis in an immunocompetent patient: case report

JUSTIFICATIVA E OBJETIVOS: A paracoccidioidomicose é uma doença granulomatosa crônica causada por um fungo e acomete principalmente os pulmões e a pele. A clínica é variada e o envolvimento do sistema nervoso central (SNC) é raro. Quando não diagnosticada e tratada oportunamente pode levar às formas disseminadas graves e letais. O objetivo desse estudo foi demonstrar as características clínicas da rara apresentação dessa doença. RELATO DO CASO: Paciente do sexo masculino, 26 anos, imunocompetente, apresentava paracoccidiodomicose recidivante com envolvimento do SNC. CONCLUSÃO: Apesar de ser uma doença rara, deve-se ter em mente que a neuroparacoccidiodomicose pode atingir pacientes imunocompetentes. Portanto deve-se fazer o diagnóstico e o tratamento de forma precoce.

Management of colorectal liver metastases in patients with peritoneal carcinomatosis.

We have taken into consideration papers published in the last 10 years on the treatment of patients with peritoneal carcinomatosis and hepatic metastasis from colorectal cancer and the pre-operative prognostic factors needed to consider these subjects eligible for surgical treatment. Peritoneal carcinomatosis should not be considered an absolute contraindication to hepatic resection if it is possible to perform a complete resection of all peritoneal and liver disease.

Doença de Weber Christian: relato de caso / Weber Christian Disease: case report

JUSTIFICATIVA E OBJETIVOS: A doença de Weber Christianou paniculite lobular idiopática é uma doença rara, que se apresenta com inflamação recorrente da camada adiposa da pele. O objetivo deste estudo foi descrever o quadro clínico dessa doença e informar sobre sua presença e impacto na qualidade de vida dos pacientes. RELATO DO CASO: Paciente do sexo feminino, portadora dessa doença, que ficou 20 anos sem diagnóstico, mas quando se detectou sua presença foi possível a remissão. CONCLUSÃO: A importância do caso descrito está em demonstrar como a falta de diagnóstico interfere na qualidade devida dos pacientes, portanto é bom lembrar que doenças raras acontecem e seu diagnóstico só é feito quando se pensam em hipóteses e diagnósticos diferenciais, quesitos esses indispensáveis para o exercício da clínica médica.

BACKGROUND AND OBJECTIVES: Weber Christian disease or idiopathic lobular pannicullitis is a skin condition that features recurring inflammation in the fat layer of the skin. The aim of this article is to describe this rare pathology, the clinical findings and remember your presence and their impact on patient's quality of life. CASE REPORT: Female patient, we report a case of patient with Weber Christian disease that was undiagnosed for 20 years and, when it was done it was possible to remission of the disease. CONCLUSION: The importance of the case described is to demonstrate how the lack of diagnosis interfere with the quality of life of patients and we always remember that rare diseases occurand their diagnosis is only made when thinking about chance and differential diagnosis, these questions essential to the exercise of clinical practice.

Variable pathological and clinical features of a large Brazilian family harboring a mutation in the aryl hydrocarbon receptor-interacting protein gene.

BACKGROUND: Germline aryl hydrocarbon receptor-interacting protein (AIP) mutations occur in 15% of familial isolated pituitary adenoma (FIPA) cases. To date, studies have focused on the identification of such mutations in large international cohorts. Detailed genetic and clinical studies within AIP mutation-positive families have been limited. AIM: To undertake a comprehensive study of a large Brazilian FIPA kindred with an E174 frameshift (E174fs) AIP mutation to assess clinical, hormonal, and radiological features in mutation carriers. METHODS: The kindred included 122 subjects across six generations; all underwent clinical examination. Genetic studies were performed to identify E174fs mutation carriers. E174fs-positive subjects underwent magnetic resonance imaging (MRI) and hormonal assessments. RESULTS: Of the ten germline AIP mutation carriers, three had pituitary tumors, while seven were asymptomatic carriers. Three patients with pituitary tumors showed variability in terms of tumor phenotype (two with acromegaly, one with prolactinoma, or mixed prolactin/GH-secreting tumor) and age at diagnosis; both patients with acromegaly had poor responses to octreotide. Tumor AIP immunohistochemistry from the operated patient showed decreased expression when compared with normal tissue. Two adult subjects with normal MRI had elevated IGF-I in the absence of other causes. A 2-year-old child with the E174fs mutation and a normal MRI had premature thelarche, ovarian development, and advanced bone age in the absence of other underlying causes. CONCLUSIONS: The penetrance of pituitary tumors in AIP mutation-positive adult subjects was 33.3%, while clinical/hormonal features were variable. The features noted in AIP-mutation carriers in this kindred suggest that clinical characteristics of such carriers may extend beyond pituitary tumors.

Câncer de boca: expressão imuno-histoquímica de c-erbB-2, Bcl-2 e EGFR - estudo comparativo com leucoplasia e hiperplasia inflamatória / Oral cancer: immunohistochemical expression of c-erbB-2, Bcl-2 and EGFR - study with leukoplakia and inflammatory hyperplasia

Anormalidades em genes que regulam a proliferação e morte celular podem provocar inúmeras doenças entre elas o carcinoma epidermóide de boca. Tem sido relatado que alterações genéticas nas células tumorais predizem a agressividade biológica dos tumores. Marcadores genéticos como c-erbB-2, Bcl-2 e EGFR são considerados indicadores promissores de prognósticos para as lesões cancerizáveis e as neoplasias. Objetivo: Avaliar a expressão imuno-histoquímica das proteínas c-erbB-2, Bcl-2 e EGFR (oncoproteínas envolvidas na vias de proliferação celular). Material e Métodos: cento e cinco blocos de parafina contendo fragmentos de biopsias incisionais, sendo 54 de carcinomas epidermóides, 25 blocos de leucoplasias e 26 blocos de hiperlasias obtidos do Laboratório de Patologia de Boca da Universidade Federal de Mato Grosso do Sul (UFMS). A expressão das proteínas foi verificada através da técnica imuno-histoquímica utilizando a estreptoavidina-biotina-peroxidase no Laboratório de Patologia da Universidade de Brasília (UNB). Resultados: Os resultados revelaram diferença estatisticamente significante da proteína EGFR para os carcinomas epidermóides de boca e para as demais proteínas não houve diferença estatisticamente significante entre as lesões. Conclusões: Os resultados sugerem que o EGFR pode ser utilizado como marcador em carcinoma de boca podendo contribuir para a progressão da neoplasia, porém, sendo insuficiente na predição da carcinogênese

Multidisciplinary approach to locally advanced rectal cancer: results of a single institution trial.

Locally advanced rectal cancer carries out a dismail prognosis despite optimal surgery in terms of local and distant relapses. Neoadjuvant chemoradiation offers good results with tumor downstaging and downsizing and leads to more radical surgery with conservative intent. Selection of patients and an intensive chemotherapy may improve long term results. Our experience with a combined polichemotherapy and radiotherapy for low advanced rectal cancer is presented.

Mefloquina no tratamento da leishmaniose cutânea em uma área endêmica de Leishmania (Viannia) braziliensis / Efficacy of mefloquine in the treatment of skin leishmaniasis in an endemic area of Leishmania (Viannia) braziliensis

O objetivo deste trabalho foi avaliara eficácia da mefloquina numa regiäo endêmica de leishmaniose cutânea por Leishmania (Viannia) braziliensis, considerando que esta droga de administraçäo oral, eficaz no tratamento da malária, com meia vida prolongada e efeitos colaterais pouco freqüentes poderia ser menos tóxica e de mais fácil administraçäo, quando comparadas com os antimoniais pentavalentes. Em Corte de Pedra, no litoral sul do Estado da Bahia, foram tratados, aleatoriamente, dez pacientes portadores de lesöes leishmanióticas, subdivididos em dois grupos. O primeiro grupo recebeu mefloquina pela via oral, dose de 250mg/dia, durante seis dias, repetindo-se o mesmo esquema após intervalo de três semanas. O segundo grupo recebeu antimoniato de meglumina (Glucantime©) diariamente, pela via endovenosa, na dose de 20mg/kg por20 dias. Do grupo da mefloquina só um paciente apresentou cicatrizaçäo depois do segundo ciclo. Um desses, com quatro lesöes apresentou nova lesöo durante o primeiro ciclo de tratamento. A evoluçäo dos outros três foi lenta sendo que em nove semanas nenhum deles tinha cicatrizado as úlceras que permaneciam com grande infiltraçäo e sinais evidentes de atividade. O grupo tratado com Glucantime© apresentou evidente melhora

Estudo terapêutico aberto com sulfato de aminosidine na leishmaniose mucosa causada por Leishmania (Viannia) braziliensis / Open therapeutic study with aminosidine sulfate in mucosal leishmaniasis due to Leismania (Viannia) braziliensis

From September to November 1994. 21 patients with active mucosal leishmaniasis were treated with aminosidine sulphate 16 mg/kg/day by intramuscular injection for 20 days. They were principally adult male agricultural workers. Thirteen patients had not received specific treatment and eight had failed to respond to Glucantime therapy. Diagnosis was based on clinical and epidemiological observations, a search for the parasite, leishmanin skin sensitivity and indirect fluorescent antibody serological tests. Sixty seven percent of patients had leishmania parasites isolated from inoculated hamsters or visualized in imprints or histopathological sections. The mean follow-up period was 12.6 months. All patients completed treatment. Side effects were pain at the injection site (86 per cent); mild proteinuria (24 per cent), elevated serum creatinine (.5 per cent) and subclinical bearing loss in one of two patients who did audiometric tests. Clinical cure was achieved in 48 per cent and the accumulated relapse rate was 29 per cent(4/14).

Estudo comparativo entre antimoniato de meglumina, isotianato de pentamidina e sulfato de aminosidine, no tratamento de lesöes cutâneas primárias causadas por Leishmania (Viannia) braziliensis / Comparative study between meglumine antimoniato, pentamidine isothianate and aminosidine sulphate, in the treatment of cutaneous primary lesions, caused by Leishmania (Viannia) braziliensis (L(V)b)

With the aim of comparing the therapeutic efficacy, tolerability and toxicity of meglumine antimoniate, aminosidine sulphate and pentamidine isethionate, a field study was conducted on randomized treatment of patients with primary cutaneous leishmaniasis due to Leishmania (Viannia) braziliensis (L(V)b), in Corte de Pedra, BA, from October 1992 up to January 1993. Forty six patients were treated and distributed into three groups, two with 15 and one with 16 subjects. All patients were submitted to clinical examination, histopathological and immunological investigations, as diagnostic criterium. All patients were treated by intramuscular route. Group 1 received pentamidine 4 mg/kg/every 2 days, for 8 applications; Group 2 received aminosidine 20 mg/kg/day, for 20 days, and Group 3 received meglumine 10 mg Sbv/kg/day, for 20 days. Failure of therapy was defined as ulceration of the skin lesion four months after treatment. Such failure occurred in five cases as follows: two cases in patients of group 1 one case in patients of group 2, and two cases in group 3, after the first year of follow up. In the evaluation after three years we reviewed fifteen patients, five in each group; except for one in Group 3, all of them were cured. Statistical significance of the results between the three schedules used was not verified.