INTRODUCTION AND OBJECTIVE: Neoadjuvant and adjuvant therapies for soft tissue sarcomas of the extremities are still controversial. The aim of this study was to analyze the results of a protocol of neoadjuvant chemoradiation therapy for extremity sarcomas. METHODS: A retrospective analysis was carried out in a consecutive series of 49 adult patients with advanced extremity soft tissue sarcomas that could not be resected with adequate margins during the primary resection. All patients were treated with a protocol of preoperative radiation therapy at a total dose of 30 Gy, concomitant with doxorubicin (60 mg/m²) chemotherapy. The main endpoints assessed were local recurrence-free survival, metastasis-free survival and overall survival. The median follow-up time was 32.1 months. RESULTS: The five-year local recurrence-free survival, metastasis-free survival and overall survival rates were 81.5%, 46.7% and 58.3%, respectively. For high-grade tumors, the five-year metastasis-free and overall survival rates were only 36.3% and 41.2%, respectively. Severe wound complications were observed in 41.8% of the patients who underwent surgery. These complications precluded adjuvant chemotherapy in 73.7% (14/19) of the patients eligible to receive it. CONCLUSIONS: In this study, neoadjuvant chemoradiation therapy was associated with a good local control rate, but the distant relapse-free rate and overall survival rate were still poor. The high rate of wound complications modified the planning of adjuvant treatment in most patients.

A síndrome de Lynch representa de 1-7% de todos os casos de câncer colorretal. É uma síndrome de herança autossômica dominante que predispõe ao câncer e é causada por mutações nos genes de reparo de ácido desoxirribonucléico (DNA). Desde a descoberta dos principais genes com função de reparo de DNA, mutações nos genes MSH2, MLH1, MSH6, PMS2 e PMS1 estão relacionadas com a susceptibilidade à síndrome de Lynch. Outro gene, MLH3, tem sido proposto como tendo papel na predisposição à síndrome de Lynch, porém mutações de significância clínica nesse gene não são claras. De acordo com o banco de dados InSiGHT (International Society for Gastrointestinal Hereditary Tumors), aproximadamente 500 diferentes mutações associadas à síndrome de Lynch são conhecidas, envolvendo primeiramente MLH1 (50%), MSH2 (40%) e outros (10%). Grandes progressos têm ocorrido para nosso entendimento das bases moleculares da síndrome de Lynch. A caracterização molecular será a forma mais precisa para definirmos a síndrome de Lynch e irá fornecer informações preditivas mais precisas sobre o risco de câncer colorretal e extra-colônico, além de permitir regimes otimizados de manejo.

Lynch syndrome represents 1-7% of all cases of colorectal cancer and is an autosomal-dominant inherited cancer predisposition syndrome caused by germline mutations in deoxyribonucleic acid (DNA) mismatch repair genes. Since the discovery of the major human genes with DNA mismatch repair function, mutations in five of them have been correlated with susceptibility to Lynch syndrome: mutS homolog 2 (MSH2); mutL homolog 1 (MLH1); mutS homolog 6 (MSH6); postmeiotic segregation increased 2 (PMS2); and postmeiotic segregation increased 1 (PMS1). It has been proposed that one additional mismatch repair gene, mutL homolog 3 (MLH3), also plays a role in Lynch syndrome predisposition, but the clinical significance of mutations in this gene is less clear. According to the InSiGHT database (International Society for Gastrointestinal Hereditary Tumors), approximately 500 different LS-associated mismatch repair gene mutations are known, primarily involving MLH1 (50%) and MSH2 (40%), while others account for 10%. Much progress has been made in understanding the molecular basis of Lynch Syndrome. Molecular characterization will be the most accurate way of defining Lynch syndrome and will provide predictive information of greater accuracy regarding the risks of colon and extracolonic cancer and enable optimal cancer surveillance regimens.

A esofagite de refluxo associada ao epitélio de colunar do esôfago predispõe ao adenocarcinoma, cuja incidência vem aumentando nos últimos anos. Entre 1976 e 1993, os autores trataram 11 pacientes com adenocarcinoma primário do esôfago. Em dois casos, a neoplasia desenvolveu-se em epitélio colunar ectópico no esôfago cervical e torácico. Nos demais casos, ocorreu no terço distal do esôfago em epitélio colunar de Barrett, em pacientes com sintomas clínicos de esofagite de refluxo, dos quais sete eram portadores de hérnia de hiato e refluxo gastroesofágico previamente documentados. Nove pacientes foram submetidos a esofagectomia transiatal com esofagogastroplastia, um foi submetido a esofagectomia distal com interposição de jejuno e o último a esofagogastroplastia retroestemal sem esofagectomia. A exceção de três pacientes, os demais tiveram operações consideradas curativas. Cinco doentes encontravam-se em estádios mais iniciais, ainda sem comprometimento linfonodal. Não houve mortalidade operatória, sendo que as principais complicações foram a fístula da anastomose esofagogástrica e a abertura da cavidade pleural, ambas ocorrendo em dois pacientes. A sobrevida média dos pacientes foi de 40,5 meses. Três pacientes permanecem vivos e sem evidência de doença (estádio 0, I e IIA) com 64, 94 e 117 meses de seguimento. Concluiu-se que a esofagectomia neste tipo de tumor é um procedimento seguro e que a sobrevida a longo prazo é possível quando os tumores em estadio inicial são tratados adequadamente.

Esophagitis associated with Barretts esophagus is a recognized predisponent factor for the development of adenocarcinoma. its incidence has been raising through the last years. Between 1976 and 1993, eleven patients with primary adenocarcinoma of the esophagus were treated. In two cases, the neoplasia occurred in an aberrant gastric mucosa in the cervical and thoracic esophagus. in the remaining cases, the tumor occurred in the distal third of the organ. in columnar-lined (Barretts) esophagus, in patients with hiatal hernia and gastroesophageal refluxo Nine patients were submitted to transhiatal esophagectomy. one to distal esophagectomy with interposition of jejunum. and one to retrosternal esophagogastroplasty without esophagectomy. All but three patients had curative operations. Five patients had early stage disease, without limphonode involvement. There was no operative mortality and the main complications were anastomotic leackage and openning of pleural cavity, both of them occurring, in two patients. The average survival of these patients was 40.5 months. Three patients remain alive and with no evidence of recurrence (Stage 0, I and IIA), with a follow-up of 64, 94 and 117 months. Patients with gastroesophagic reflux and Barretts esophagus must be properly treated and they need endoscopic surveillance for the evaluation of progressive dysplasia or adenocarcinoma. The authors conclude that esophagectomy is a safe procedure and long term survival is possible when these esophageal tumors are treated in the early stages.