Carcinoma epidermoide metastásico asociado a epidermólisis ampollosa distrófica / Metastasic squamous cell carcinoma in dystrophic epidermolysis bullosa

Caso clínico: femenino de 18 años con diagnóstico de epidermolisis ampollosa distrófica (EAD) quién desarrolló una neoformación nodular sobre una úlcera crónica. Se diagnosticó carcinoma epidermoide (CE) invasor al que se realizó resección. Sin embargo, 5 meses después del tratamiento quirúrgico presentó metástasis a ganglios, pulmón e hígado con desenlace fatal. Comentarios: el CE es la causa más importante de muerte en pacientes con EAD. Suele ser agresivo y metastásico. Se recomienda una vigilancia cada 3 a 6 meses para realizar diagnóstico y tratamiento oportunos (AU)

Case report: 18-year-old female patient with dystrophic epidermolysis bullosa (DEB) who developed a tumor over a chronic ulcer. She was diagnosed with invasive squamous cell carcinoma (SCC) and underwent surgical resection. However, 5 months later she presented metastases to the lymph nodes, lung and liver with a fatal outcome. Comments: SCC is the most important cause of death in patients with DEB. It is usually aggressive and metastatic. Surveillance every 3 to 6 months is recommended for prompt diagnosis and treatment (AU)

Deaths related to differentiated thyroid cancer: a rare but real event

ABSTRACT Objective The present study describes the clinical and tumor characteristics of patients that died from differentiated thyroid cancer and reports on the cause and circumstances of death in these cases. Subjects and methods Retrospective analysis of all the differentiated thyroid cancer (DTC) related deaths at a single institution over a 5-year period, with a total of 33 patients. Results Most of the patients were female (63.6%), with a mean age at diagnosis of 58.2 years. The most common histologic type was papillary (66.7%) and 30.3% were follicular. The distribution according to the TNM classification was: 15.4% of T1; 7.7% T2; 38.4% T3; 19.2% of T4a and 19.2% of T4b. Forty-four percent of cases were N0; 20% N1a and 36.6% of N1b. Twelve patients were considered non-responsive to radioiodine. Only one of the patients did not have distant metastases. The most common metastatic site was the lung in 69.7%. The majority of deaths were due to pulmonary complications related to lung metastases (17 patients, 51.5%), followed by post-operative complications in 5 cases, neurological disease progression in 3 cases, local invasion and airway obstruction in one patient. Median survival between diagnosis and death was reached in 49 months while between disease progression and death it was at 22 months. Conclusion Mortality from DTC is extremely rare but persists, and the main causes of death derive from distant metastasis, especially respiratory failure due to lung metastasis. Once disease progression is established, median survival was only 22 months.

Sunitinib treatment in patients with advanced renal cell cancer: the Brazilian National Cancer Institute (INCA) experience

ABSTRACT Purpose: The aim of this study was to assess the impact of sunitinib treatment in a non-screened group of patients with metastatic renal cell cancer (mRCC) treated by the Brazilian Unified Health System (SUS) at a single reference institution. Material and Methods: Retrospective cohort study, which evaluated patients with mRCC who received sunitinib between May 2010 and December 2013. Results: Fifty-eight patients were eligible. Most patients were male 41 (71%), with a median age of 58 years. Nephrectomy was performed in 41 (71%) patients with a median interval of 16 months between the surgery and initiation of sunitinib. The most prevalent histological subtype was clear cell carcinoma, present in 52 (91.2%) patients. In 50 patients (86%), sunitinib was the first line of systemic treatment. The main adverse effects were fatigue (57%), hypothyroidism (43%), mucositis (33%) and diarrhea (29%). Grade 3 and 4 adverse effects were infrequent: fatigue (12%), hypertension (12%), thrombocytopenia (7%), neutropenia (5%) and hand-foot syndrome (5%). Forty percent of patients achieved a partial response and 35% stable disease, with a disease control rate of 75%. Median progression free survival was 7.6 months and median overall survival was 14.1 months. Conclusion: Sunitinib treatment was active in the majority of patients, especially those with low and intermediate risk by MSKCC score, with manageable toxicity. Survival rates were inferior in this non-screened population with mRCC treated in the SUS.

The impact of erlotinib use in non-small-cell lung cancer patients treated in a private reference general hospital and in a private cancer clinic from 2005 to 2011 / O impacto do uso do erlotinibe em pacientes portadores de neoplasia de pulmão de não pequenas células tratados em um hospital geral de referência e clínica particular de oncologia no período de 2005 a 2011

ABSTRACT Objective: To report the demographic data and clinical outcomes of non-small-cell lung cancer patients exposed to erlotinib in any line of treatment. Methods: This was a retrospective cohort study of nonsmall-cell lung cancer patients from a reference general hospital and a private oncology clinic, who received erlotinib from 2005 to 2011. Statistical analysis was performed and we evaluated demographic data and response to treatment, by correlating the results of this first cohort published in Brazil with results of current literature. Results: A total of 44 patients were included; 65.9% were diagnosed with adenocarcinoma, and 63.6% had metastatic disease. The mean age was 63.3 years. The median follow-up was 47.9 months. Epidermal growth factor receptor mutation screening was performed in 22.7% of patients (n=10), with mutation present in 30% of patients. The median overall survival was 46.3 months, and there was a higher probability of survival at 60 months for females compared to males (29.4% versus 15.8%; p=0.042). The other variables did not present significant statistical difference. Conclusion: We collected the largest cohort of patients with non-small-cell lung cancer who have used erlotinib in Brazil to date, and demonstrated that outcomes of patients treated at our clinic during the study period were consistent with the results of current literature in similar patients. .

RESUMO Objetivo: Relatar as características demográficas e a evolução de pacientes com neoplasia de pulmão de não pequenas células que receberam erlotinibe em qualquer linha de tratamento. Métodos: Coletamos retrospectivamente dados de pacientes portadores de neoplasia de pulmão de não pequenas células que receberam erlotinibe em qualquer linha de tratamento em um hospital geral de referência e em uma clínica particular de oncologia em São Paulo, no período de 2005 a 2011. Foi realizada a análise estatística e foram avaliados aspectos demográficos e resposta ao tratamento estabelecido, correlacionando os resultados dessa primeira coorte publicada no Brasil com resultados da literatura vigente. Resultados: Foram avaliados 44 pacientes, dos quais 65,9% eram portadores de adenocarcinoma e 63,6% tinham doença metastática. A média de idade foi de 63,3 anos. O seguimento mediano foi de 47,9 meses. A pesquisa de mutação do receptor do fator de crescimento epidérmico foi realizada em 22,7% dos pacientes (n=10), resultando positiva em 30% dos avaliados. A sobrevida global mediana foi de 46,3 meses, e observou-se uma probabilidade maior de sobrevida em 60 meses para o grupo feminino, quando comparado ao grupo masculino (29,4% versus 15,8%; p=0,042). As demais variáveis não apresentaram diferença estatística significativa. Conclusão: Coletamos a maior sequência de pacientes com neoplasia de pulmão de não pequenas células que fizeram uso de erlotinibe no Brasil até a data vigente e demonstramos que a evolução dos pacientes tratados no período avaliado teve resultados concordantes com os da literatura vigente em pacientes semelhantes. .

Carga de doença no Brasil: um olhar sobre o álcool e a cirrose não viral / Disease burden in Brazil: an investigation into alcohol and non-viral cirrhosis

O uso/dependência de álcool é importante fator de risco para o desenvolvimento da cirrose. O objetivo deste artigo é descrever e analisar o DALY (Disability Adjusted Life Years), o YLL (Years of Life Lost) e o YLD (Years Lived with Disability) de uso/dependência de álcool e da cirrose de etiologia não viral no Brasil, em 2008. O DALY foi calculado pela soma do YLL e do YLD. Para o YLL, foi utilizada a média dos óbitos de 2007-2009 no país. Através da revisão de dados epidemiológicos e do uso da ferramenta DisMod, a prevalência de cada um dos agravos foi modelada, gerando dados de incidência para o cálculo do YLD. O álcool e a cirrose foram responsáveis, respectivamente, por 3% e 1% do DALY total. Considerando-se as dez primeiras causas de DALY para homens, o uso/ dependência de álcool ocupou a segunda, terceira e sexta posições nas idades de 15-29, 30-44 e 45-59 anos, respectivamente. A cirrose ocupou a oitava posição no grupo de 30-44 anos; a quinta, no de 45-59 e a oitava, no de 60-69. A distribuição dos agravos por faixa etária sugere que intervenções direcionadas ao uso/dependência de álcool terão efeitos na carga de cirrose alcoólica no país.

Alcohol use/dependence are an important risk factor for cirrhosis of the liver. The article aims to describe and conduct a comparative analysis of Disability Adjusted Life Years (DALY), Years of Life Lost (YLL) and Years Lived with Disability (YLD) of alcohol use disorders and non-viral cirrhosis in Brazil in 2008. DALY was calculated as the sum of YLL and YLD. For YLL estimates, the mean number of deaths from 2007- 2009 in the country was considered. After revision of epidemiological data, prevalence of each disease was modelled with the DisMod tool, which generated incidence data for YLD estimates. Alcohol and non-viral cirrhosis were responsible for 3% and 1% of total DALYs, respectively. In both diseases, men contributed to a greater proportion of DALYs. Among the first ten causes of DALYs, alcohol use disorders occupied the second, third and sixth positions at the ages of 15-29, 30-44 and 45- 59, respectively. Non-viral cirrhosis was the eighth cause of DALY in the 30-44 age group in men; the fifth, in the 45-59 group and the eighth, in the 60-69 group. Age distribution suggests that interventions directed against alcohol use/dependence would have effects on the burden of alcoholic cirrhosis in the country.

A socialização da medicina na era do adhemarismo / The socialization of medicine in the era of São Paulo Governor Adhemar de Barros

A análise observa as conexões entre o processo de profissionalização dos médicos paulistas e políticas de saúde do governo estadual de Adhemar de Barros em São Paulo (1947-1951), em meio a amplas mudanças na área de saúde denominadas pelos médicos paulistas “socialização da medicina”. Reconhecemos aspectos ambivalentes para o profissionalismo médico diante desse governo populista, como: a luta médica pela equiparação ante os advogados servidores públicos estaduais; a criação de uma secretaria de saúde estadual; e certos elos contraditórios entre a área de saúde adhemarista e a ideologia e a organização profissionais da medicina paulista. Nesse particular, o artigo aprofunda a análise de manifestações ideológicas de importantes lideranças médicas paulistas.

The article analyzes how the process of the professionalization of physicians in São Paulo related to healthcare policy under the administration of São Paulo governor Adhemar de Barros (1947-1951) during a period of broad change in the realm of health known by São Paulo physicians as the “socialization of medicine.” Medical professionalism confronted certain ambivalences under this populist administration, including doctors’ struggle to achieve pay equal to that of state public attorneys; the establishment of a state health department; and some contradictory ties between the area of health under Adhemar and the professional ideology and organization of medicine in São Paulo. The article undertakes a more in-depth analysis of the ideological manifestations of important leaders in the state’s medical community.

Response to sorafenib treatment in advanced metastatic thyroid cancer / Resposta ao tratamento com sorafenibe em pacientes com carcinoma metastático de tireoide

Objective: To investigate the efficacy of sorafenib in progressive radioiodine resistant metastatic thyroid carcinoma.Subjects and methods: Off-label observational study. Sorafenib 400 mg twice daily was evaluated. Therapy duration was 12 ± 3 months (range 6-16 months).Results: Eight patients were included (seven papillary, one insular variant). The eight patients meeting study criteria received sorafenib 400 mg orally twice a day until disease progression or unacceptable toxicity developed. One patient showed a partial response with tumor regression of -35%, six months after the beginning of the treatment; five patients exhibited stable disease and two patients had progressive disease and died. Thyroglobulin decreased within 4 weeks in all patients by 50% ± 23%.Adverse events: one patient had heart failure, and recovered after sorafenib withdrawal. However, she died five months later of sudden death.Conclusion: These data suggest a possible role for sorafenib in the treatment of progressive metastatic DTC. Adverse event are usually manageable, but severe ones may appear and these patients should be strictly controlled.

Objetivo: Investigar a eficácia do sorafenibe no carcinoma de tireoide metastático progressivo e refratário à iodoterapia.Sujeitos e métodos: Estudo observacional do efeito do sorafenibe off-label administrado 400 mg duas vezes ao dia. A duração da terapia foi de 12 ± 3 meses (variação de 6-16 meses).Resultados: Oito pacientes foram incluídos (sete com variante papilífera e um com variante insular). Os oito pacientes que preencheram os critérios do estudo receberam o sorafenibe 400 mg por via oral duas vezes por dia até progressão da doença ou toxicidade inaceitável. Um paciente apresentou uma resposta parcial com regressão tumoral da lesão alvo de 35% seis meses após o início do tratamento; cinco pacientes apresentaram doença estável e dois pacientes progrediram e morreram. A tireoglobulina diminuiu 50% ± 23% em 4 semanas em todos os pacientes.Eventos adversos: um paciente teve insuficiência cardíaca e morreu por morte súbita cinco meses após a retirada do sorafenibe.Conclusão: Esses dados sugerem um possível papel para sorafenibe para o tratamento do CDT metastático progressivo.

Descriptive analysis of and overall survival after surgical treatment of lung metastases / Analise descritiva e sobrevida global do tratamento cirurgico das metastases pulmonares

OBJECTIVE: To describe demographic characteristics, surgical results, postoperative complications, and overall survival rates in surgically treated patients with lung metastases. METHODS: This was a retrospective analysis of 119 patients who underwent a total of 154 lung metastasis resections between 1997 and 2011. RESULTS: Among the 119 patients, 68 (57.1%) were male and 108 (90.8%) were White. The median age was 52 years (range, 15-75 years). In this sample, 63 patients (52.9%) presented with comorbidities, the most common being systemic arterial hypertension (69.8%) and diabetes (19.0%). Primary colorectal tumors (47.9%) and musculoskeletal tumors (21.8%) were the main sites of origin of the metastases. Approximately 24% of the patients underwent more than one resection of the lesions, and 71% had adjuvant treatment prior to metastasectomy. The rate of lung metastasis recurrence was 19.3%, and the median disease-free interval was 23 months. The main surgical access used was thoracotomy (78%), and the most common approach was wedge resection with segmentectomy (51%). The rate of postoperative complications was 22%, and perioperative mortality was 1.9%. The overall survival rates at 12, 36, 60, and 120 months were 96%, 77%, 56%, and 39%, respectively. A Cox analysis confirmed that complications within the first 30 postoperative days were associated with poor prognosis (hazard ratio = 1.81; 95% CI: 1.09-3.06; p = 0.02). CONCLUSIONS: Surgical treatment of lung metastases is safe and effective, with good overall survival, especially in patients with fewer metastases. .

OBJETIVO: Descrever características demográficas, resultados operatórios, complicações pós-operatórias e taxa de sobrevida global em pacientes com metástases pulmonares tratados cirurgicamente. MÉTODOS: Análise retrospectiva de 119 pacientes submetidos a um total de 154 cirurgias de ressecção de metástase pulmonar entre 1997 e 2011. RESULTADOS: Do total de 119 pacientes, 68 (57,1%) eram do sexo masculino, e 108 (90,8%) eram brancos. A mediana de idade foi de 52 anos (variação, 15-75 anos). Nessa amostra, 63 pacientes (52,9%) apresentaram comorbidades, sendo as mais frequentes hipertensão arterial sistêmica (69,8%) e diabetes (19,0%). Tumores primários colorretais (47,9%) e musculoesqueléticos (21,8%) foram os principais sítios de origem das metástases. Aproximadamente 24% dos pacientes foram submetidos a mais de uma ressecção das lesões, e 71% fizeram tratamento adjuvante prévio à metastasectomia. A taxa de recidiva de metástase pulmonar foi de 19,3%. A mediana do intervalo livre de doença foi de 23 meses. A principal via de acesso usada foi toracotomia (78%), e o tipo de ressecção mais frequente foi em cunha e segmentectomia (51%). O índice de complicações pós-operatórias foi de 22% e o de mortalidade perioperatória foi de 1,9%. As taxas de sobrevida global em 12, 36, 60 e 120 meses foram, respectivamente, de 96%, 77%, 56% e 39%. A análise de Cox confirmou que complicações nos primeiros 30 dias pós-operatórios associaram-se a pior prognóstico (hazard ratio = 1,81; IC95%: 1,09-3,06; p = 0,02). CONCLUSÕES: O tratamento cirúrgico das metástases pulmonares oriundas de diferentes sítios tumorais é efetivo e seguro, com boa sobrevida global, especialmente nos casos com um menor número de lesões ...

Resección de metástasis pulmonares / Resection of lung metastases

Surgical treatment of lung metastases (LM) is a therapy used for several types of metastatic cancers. However, the type of surgery and its outcome is controversial. Aim: To describe a series of patients with LM undergoing surgical treatment, their results and long-term follow up. Material and Methods: We retrospectively reviewed all the medical records of patients with the diagnosis of LM, of whatever origin, operated at our hospital between the years 2002 and 2008. We analyzed their clinical characteristics, treatment, morbidity, mortality and results. Results: During this period 24 patients with LM were operated. The mean age was 52.8 +/- 16.6 years. Most patients had kidney cancer as primary tumour. The usual clinical presentation was multiple bilateral nodules. The surgical approach was in general a video-thoracoscopy (40.9 percent) associated with a wedge resection of the lesions (81.8 percent). In 83.4 percent of cases, there were no post-operative complications. Seventy six percent of the patients relapse, in an average of 16.7 +/- 12 months and 68.7 percent were reoperated for a new resection. The probability of surviving more than 3 and 5 years post metastasectomy was 57 percent and 36 percent respectively. Conclusions: In this series the LM were mostly secondary to renal cancer. The video-thoracoscopy is an emergent and safe technique in the treatment of LM, being the main type of surgical approach in our patients. Despite the high percentage of reoperations, the survival rate is comparable to the rest of the published series.

El tratamiento quirúrgico de las metástasis pulmonares (MP) constituye una terapia usada para varios tipos de cánceres metastásicos. Sin embargo, el tipo de cirugía y sus resultados es controversial, por lo que hemos decidido estudiarla. Objetivo: Describir clínicamente una serie de pacientes con MP sometidos a tratamiento quirúrgico, sus resultados y seguimiento a largo plazo. Material y Método: Se revisaron retrospectivamente las fichas de pacientes con el diagnóstico de MP, de cualquier origen, operados en nuestro hospital entre los años 2002 y 2008. Se analizaron las características clínicas, tratamiento, morbimortalidad y resultados. Resultados: Durante este período se operaron 24 pacientes por MP. El promedio de edad fue 52,8 +/- 16,6 años. La mayoría de los pacientes tenía un cáncer renal como tumor primario. La forma habitual de presentación fueron múltiples nodulos bilaterales. El abordaje quirúrgico más frecuente fue por videotoracoscopía (40,9 por ciento) asociado a una resección en cuña de las lesiones (81,8 por ciento). En el 83,4 por ciento no hubo complicaciones post operatorias. El 76,4 por ciento de los pacientes recidivó, en promedio a los 16,7 +/-12 meses y el 68,7 por ciento fue reintervenido para una nueva resección. La probabilidad de sobrevivir más de 3 y 5 años post me-tastasectomía fue de 57 por ciento y 36 por ciento respectivamente. Conclusiones: En esta serie las MP fueron principalmente secundarias a cáncer renal. La videotoracoscopía es una cirugía emergente y segura en el tratamiento de las MP, siendo la principal forma de abordaje quirúrgico en nuestros pacientes. A pesar del elevado porcentaje de reintervenciones, la sobrevida es comparable al resto de las series publicadas.

Carcinoma de tireóide na infância e adolescência / Thyroid carcinoma in children and adolescents

Aproximadamente 10 por cento das neoplasias de tireóide aparecem abaixo dos 21 anos e representam 3 por cento das neoplasias da infância, com predomínio em meninas (2F:1M). São papilíferos em 90 por cento casos e mais freqüentemente bilaterais, multifocais e de maior dimensão. Invasão de cápsula e metástases ganglionar ou pulmonar são mais freqüentes que no adulto. O efeito carcinogênico da radiação é especialmente importante na criança pré-púbere. Em 5 por cento dos casos de PTC existe história familial. Genes como o RET/PTC, RAS e BRAF estão habitualmente envolvidos na gênese do processo. A apresentação clínica pode ser nódulo cervical (90 por cento casos) sem que represente um fator de pior prognóstico. O US e a PAAF são procedimentos diagnósticos valiosos. O tratamento deve ser cirúrgico (tireoidectomia total com esvaziamento ganglionar) seguido de PCI e radioterapia ablativa. A substituição com L-tiroxina deve objetivar a supressão crônica do TSH. O prognóstico ao longo prazo é melhor que no adulto. A quantificação da Tg plasmática também é útil na detecção da doença residual.

Approximately 10 percent of thyroid cancers are present in patients less than 21 years of age, representing 3 percent of all cancers of children and adolescents, with predominance in females 2:1 in relation to males. Thyroid cancers in this age group are usually papillary (90 percent), bilateral, multifocal and bigger in size compared to adults. Capsule invasion and lymphatic and pulmonary metastases are more frequent in children. Radiation sensitivity seems to represent an important factor in prepubertal patients. Familial history is reported in 5 percent of the cases. Genes such as RET/PTC, RAS and BRAF are usually involved in thyroid carcinogenesis in this age group. Cervical adenomegaly is a common clinical presentation, but does not represent a poor prognostic factor in children. Ultrasound and fine needle aspiration biopsy are valuable diagnostic procedures. Surgery is the preferred treatment including thyroidectomy and ganglionary excision, followed by ablative radioiodine therapy. L-thyroxine replacement with suppressive dosage should be employed targeting chronic TSH suppression. Long-term prognosis is usually better in children when compared with adults. Plasma thyroglobulin measurement is also useful to detect residual thyroid cancer disease.

Metástases pulmonares em homem: localização incomum do tumor primário / Pulmonary metastases in men: primary tumor in an unusual location

Os autores relatam caso de câncer de mama em um homem de 72 anos achado acidentalmente durante a pesquisa do tumor primário para investigação de metástases pulmonares. São abordados aspectos epidemiológicos, diagnóstico, tratamento e prognóstico desta patologia no sexo masculino.

We report a case of breast cancer identified in a 72-year-old male as an accidental finding during the course of the investigation of a primary tumor and the search for pulmonary metastases. We address aspects related to the epidemiology, diagnosis, treatment and prognosis of this condition in males.

5mCi pretreatment scanning does not cause stunning when the ablative dose is administered within 72 hours

OBJETIVO: Determinar a interferência de uma dose traçadora de 5mCi de 131I. PACIENTES E MÉTODO: Nós analisamos retrospectivamente 145 pacientes que receberam o primeiro tratamento ablativo em nosso serviço. Eles foram divididos de acordo com o estadio da doença determinado pelo rastreamento pós-terapêutico (101 pacientes com remanescentes tireoidianos e 44 com metástases pulmonares) e scanning de corpo inteiro antes da ablação (realizada em 69 indivíduos). Todos os pacientes com remanescentes tireoidianos foram tratados com uma dose ablativa de 100mCi e aqueles com metástases receberam 200mCi. RESULTADOS: Nos pacientes com remanescentes apenas (n= 41) ou metastases (n= 28) submetidos ao scanning diagnóstico, a captação encontrava-se aparentemente aumentada na maioria dos patientscasos (71 e 73%, respectivamente) 7 dias após a terapia, enquanto captação reduzida (visual) não foi observada em nenhum paciente. A eficácia da ablação foi similar nos grupos submetidos ou não ao rastreamento diagnóstico: 71 e 80% em pacientes sem metástases (p= 0,28), respectivamente, e 43 e 50% naqueles com envolvimento pulmonar (p= 0,64). CONCLUSAO: Esses resultados indicam que o rastreamento diagnóstico usando uma dose de 5mCi de 131I não interefere com a captação da dose ablativa ou com a eficácia do tratamento quando a ablação é realizada dentro de 72h.