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Background Plexiform neurofibromas (PN) are the most frequent tumors associated with Neurofibromatosis type 1 (NF-1). PN can cause significant complications, including pain, functional impairment, and disfigurement. There is no efficient medical treatment and, surgical resection of large PN is frequently infeasible. Selumetinib (AZD6244/ARRY-142886) is a mitogen-activated protein kinase enzyme (MEK1/2) inhibitor and works by targeting the MAPK pathway. It is an investigational treatment option for inoperable symptomatic PN associated with NF-1. Herein, we describe a single institutional experience with selumetinib for inoperable PN in NF-1. Methods Case series study of demographics, clinical, baseline characteristics, treatment effect, and follow-up of consecutive genetically confirmed NF1 patients with inoperable PN associated with significant or potential significant morbidity treated with selumetinib (April 2018 to April 2019). Results Nineteen patients were treated with selumetinib. Predominant target locations were head and neck (31.6%, 6/19), chest (26.3%, 5/19) and pelvis (21%, 4/19) and the most important comorbidities were disfigurement (47.4%, 9/19) and pain (26.3%, 5/19). The mean follow-up time was 223 days (range 35-420 days). All but one had sustained clinical improvement, mainly in the first 60-90 days of treatment. In one patient, the treatment was suspended after 168 days (lack of clear benefit and left ventricular ejection fraction drop). There were no adverse effects leading to treatment suspension. Conclusions In the first observational study of selumetinib for NF-1 associated PN we showed that the drug was associated with clinical and radiological improvement. Our study also confirms the safety described in the clinical trials.

The ideal therapeutic regimen in primary mediastinal B-cell lymphoma (PMBCL) is controversial and may include consolidation radiotherapy (RT). An adequate strategy is essential in a population where long-term effects of RT are significant. We evaluated the prognostic value of end-of-chemotherapy (EOT) FDG-PET in 50 patients receiving rituximab and anthracycline-containing chemotherapy and its implications for consolidative RT. Thirty patients (60%) obtained complete metabolic response (CMR), 5 received consolidation RT. The remaining patients had partial response (14) and progression (6). Of these, 12 received mediastinal RT, 6 salvage chemotherapy and 2 no further treatment. Five-year progression free survival (PFS) was 100% and 48% (95%CI 30-77%) in patients with negative and positive EOT FDG-PET respectively (p

Well-differentiated thyroid cancer (WDTC) is the most common endocrine neoplasia, and its incidence is rising. Studies have reported an increased risk of second primary cancer (SPC) in WDTC survivors, but its relationship with radioiodine treatment (RAIT) and other risk factors remains controversial. This study evaluated whether RAIT is an independent risk factor for SPC in WDTC patients.This was a retrospective single-center study. A total of 2031 patients with WDTC diagnosed between 1998 and 2009, treated and followed at the authors' tertiary cancer center, were included.The median age of patients was 48 years (range 5-90 years); 83% were women and 77% underwent RAIT. The median follow-up was 8.8 years (range 5.0-17.0 years). A total of 130 SPC were diagnosed: 108/1570 (6.9%) received RAIT (RAIT+) and 22/461 (4.8%) did not (RAIT-). The most common SPC was breast cancer (31%), followed by genitourinary and gastrointestinal cancer (18% each). The 10-year cumulative incidence of SPC was 8.2% in RAIT+ and 4.5% in RAIT-. The absolute risk increase in the RAIT+ group versus the RAIT- group at 10 years of follow-up was 0.039 [confidence interval (CI) 0.011-0.067] per patient-year. The number needed to harm (NNH) was 25.6 [CI 15.0-87.2], indicating that on average during a 10-year follow-up period, there is one additional case of SPC for every 26 patients receiving RAIT. When controlling for age, sex, and familial and personal histories of cancer, there was an 84% increase in the risk of SPC in the RAIT+ group compared to the RAIT- group (p = 0.026; relative risk = 1.84 [CI 1.02-3.31]). There was an association between SPC incidence and total cumulative activity administered, which was statistically significant200 mCi. The incidence of SPC was higher in both the WDCT and the RAIT+ cohorts compared to the general population (standardized incidence ratios = 1.32 and 1.40, respectively).These results indicate that in spite of the low incidence of SPC in WDTC patients, the risk is increased after RAIT, particularly for activities200 mCi. Thus, considering the excellent survival of patients with WDTC, clinicians need to weigh the risks and benefits of RAIT, especially in patients with low-risk thyroid cancer.

In recent years, with the higher median life expectancy, the number of hip and knee replacements has increased. Clinical examination and morphological studies are essential to evaluate patients with a painful arthroplasty. Nuclear medicine examinations also play an important role, their main usefulness being the exclusion of prosthesis complications. Nevertheless, conventional examinations, namely bone scan and white blood cell scintigraphy, can also identify complications, such as loosening and infection. This study describes the normal and pathologic patterns of a bone scan and exemplifies ten common situations that can cause pain in patients with hip or knee arthroplasty, other than loosening and infection, which can be disclosed on a bone scintigraphy. The ten situations that should be considered and looked for when analysing a bone scan are: referred pain, patellofemoral pain syndrome, fractures, fissures, abscess/haematoma, bone insert behaviour, heterotopic ossification, greater trochanter pseudarthrosis, osteoarthritis extension in a knee with an unicompartmental prosthesis, and systemic disease with bone involvement.

To evaluate factors associated with non identification of the sentinel lymph node (SLN) in lymphoscintigraphy of breast cancer patients and analyze the relationship with SLN metastases.A single-center, cross-sectional and retrospective study was performed. Forty patients with lymphoscintigraphy without sentinel lymph node identification (negative lymphoscintigraphy - NL) were enrolled. The control group included 184 patients with SLN identification (positive lymphoscintigraphy - PL). Evaluated factors were age, body mass index (BMI), tumor size, histology, localization, preoperative breast lesion hookwire (harpoon) marking and SLN metastases. The statistical analysis was performed with uni- and multivariate logistic regression models and matched-pairs analysis.Age (p=0.036) or having BMI (p=0.047) were the only factors significantly associated with NL. Being ≥60 years with a BMI ≥30 increased the odds of having a NL 2 and 3.8 times, respectively. Marking with hookwire seems to increase the likelihood of NL, but demonstrated statistical significance is lacking (p=0.087). The other tested variables did not affect the examination result. When controlling for age, BMI and marking with the harpoon, a significant association between lymph node metastization and NL was not found (p=0.565).The most important factors related with non identification of SLN in the patients were age, BMI and marking with hook wire. However, only the first two had statistical importance. When these variables were controlled, no association was found between NL and axillary metastases.

© 2014 Elsevier España, S.L.U. and SEMNIM. All rights reserved., Objectives: In 2009-2010 a Portuguese consortium was created to implement the methodologies proposed by the Dose Datamed II (DDM2) project, aiming to collect data from diagnostic X-ray and nuclear medicine (NM) procedures, in order to determine the most frequently prescribed exams and the associated ionizing radiation doses for the Portuguese population. The current study is the continuation of this work, although it focuses only on NM exams for the years 2011 and 2012. Material and methods: The annual frequency of each of the 28 selected NM exams and the average administered activity per procedure was obtained by means of a nationwide survey sent to the 35 NM centres in Portugal. Results: The results show a reduction of the number of cardiac exams performed in the last two years compared with 2010, leading to a reduction of the annual average effective dose of Portuguese population due to NM exams from 0.08 mSv ± 0.017 mSv/caput to 0.059 ± 0.011 mSv/caput in 2011 and 0.054 ± 0.011 mSv/caput in 2012. Portuguese total annual average collective effective dose due to medical procedures was estimated to be 625.6 ± 110.9 manSv in 2011 and 565.1 ± 117.3 manSv in 2012, a reduction in comparison with 2010 (840.3 ± 183.8 manSv). Conclusions: The most frequent exams and the ones that contributed the most for total population dose were the cardiac and bone exams, although a decrease observed in 2011 and in 2012 was verified. The authors intend to perform this study periodically to identify trends in the annual Portuguese average effective dose and to help to raise awareness about the potential dose optimization.

A cobertura do solo é prática importante na produção de hortaliças que também auxilia no controle de plantas daninhas. Praticamente, não existem pesquisas para o controle alternativo de plantas daninhas em beterraba, e são poucos os herbicidas indicados para a cultura. Neste trabalho, objetivou-se avaliar o efeito da cobertura do solo com palha de café ou bagaço de cana-de-açúcar e de doses de lodo de lagoa de decantação de águas residuárias de suinocultura na incidência de plantas daninhas e na produtividade de beterraba, cultivar Early Wonder. O experimento foi realizado na Fazenda Experimental da EPAMIG, em Oratórios-MG. Utilizou-se o esquema de parcelas subdivididas no delineamento experimental de blocos casualizados, com quatro repetições. Nas parcelas foram dispostos três tipos de cobertura do solo: bagaço de cana-de-açúcar, palha de café e ausência de cobertura, e nas subparcelas utilizaram-se cinco doses de lodo de lagoa de decantação de águas residuárias de suinocultura (0, 10, 20, 40 e 60 t ha-1). Aos 45 dias após o transplantio (DAT) das mudas foi avaliada a população de plantas daninhas, e aos 70 DAT realizou-se a colheita. A palha de café e o bagaço de cana-de-açúcar foram eficazes na redução da massa fresca total das plantas daninhas, devido ao efeito supressor da cobertura morta sobre as dicotiledôneas. Todavia, a massa seca de tiririca aumentou com o uso de palha de café, enquanto a massa seca das outras monocotiledôneas não apresentou diferença entre os três tratamentos. O aumento das doses de lodo promoveu redução linear da massa seca de monocotiledôneas, exceto de tiririca. A cobertura com palha de café proporcionou maior massa unitária de raiz e maior produtividade de raízes comerciais, independentemente das doses de lodo.

Introducao: A terapeutica com litio esta indicada no tratamento da doenca bipolar. Para alem de uma maior incidencia de bocio e de hipotiroidismo, o uso prolongado de litio esta associado a varias consequencias metabolicas, nomeadamente a hipercalcemia e o hiperparatiroidismo (HPT). Caso clinico: Mulher, 64 anos, com antecedentes de doenca bipolar e HTA. Medicada com litio, quetiapina, bisoprolol e enalapril. Recorre a nossa consulta em Agosto de 2008 por BMN conhecido desde ha varios anos e por niveis de calcio elevados. Sem historia conhecida de litiase renal. Na consulta e-lhe confirmado um BMN (com citologia benigna dos nodulos) e um hiperparatiroidismo primario (calcio 10,6, fosforo 2,06, PTH 256, clearance creatinina 73 ml/min, calciuria e funcao tiroideia sem alteracoes). Cintigrafia das paratiroides com sestamibi negativa. Osteodensitometria ossea com evidencia de osteopenia (Tscore -2,0) ao nivel da extremidade distal do radio. Medicada com acido alendronico 70 mg/semana. A hipotese de diagnostico mais provavel foi BMN e HPT secundarios ao litio (HPTL). Por nao ser possivel parar o litio, decidiu-se manter vigilância. Apos 1 ano, verifica-se franco agravamento da hipercalcemia (calcio: 12,8). Repete sestamibi, que mostra um aumento da captacao do radiofarmaco ao nivel da paratiroide inferior esquerda, razao pela qual e proposta para cirurgia. Em Jan/2010 e submetida a tiroidectomia total e paratiroidectomia inferior esquerda. O DH revelou um adenoma da paratiroide com 13 × 12 × 8 mm e uma hiperplasia folicular, multinodular da tiroide. Pos operatorio com normalizacao do calcio e reducao da PTH. Inicia terapeutica com levotiroxina 12 5ug/dia e mantem litio. Durante 1 ano, a doente manteve niveis de calcio normais e de PTH discretamente elevados. Repetiu sestamibi que foi negativo. Atualmente, mantem-se em vigilância na consulta de Endocrinologia, mantendo terapeutica com litio. Discussao: O HPTPL pode afectar uma ou varias paratiroides, pelo que o tratamento cirurgico nao e consensual. A individualizacao da terapeutica e essencial, sendo frequentemente necessaria uma exploracao bilateral. O rastreio atempado e vigilância do HPTL e essencial em doentes sob terapeutica com litio, permitindo uma reducao das comorbilidades associadas.

Differentiated thyroid carcinoma of follicular origin (DTCFO), although not very frequent, has registered a raising incidence in the last decades. In the majority of the cases, DTCFO is a curable disease when treated and monitored by experienced, multidisciplinary teams. These factors contribute to an increasing number of DTCFO survivors requiring life-long monitoring, due to the possibility of occurrence of recurrences many years after the initial treatment. Several aspects of the treatment and management of these patients are still controversial. The present protocol represents the consensus of the members of the Grupo de Estudo da Tiróide of the Sociedade Portuguesa de Endocrinologia, Diabetes e Metabolismo. It aims to define guidelines, in agreement with the current state of the art and contemplating the necessary adaptations to local constrains, that ensure decreased mortality and protection of patients' quality of life, avoiding unnecessarily aggressive or ineffective treatments, optimizing the use of the available resources.