Large desmoid tumors in familial adenomatous polyposis: a successful outcome

Desmoid tumors develop from connective tissue, fasciae, and aponeuroses, and may occur in the context of familial adenomatous polyposis or may arise sporadically; also, they may be extra-abdominal, intra-abdominal, or located in the abdominal wall. These benign tumors have a great aggressiveness with a high rate of local recurrence. Familial adenomatous polyposis is an inherited condition with autosomal dominant transmission, and is characterized by the development of multiple colonic and rectal adenomatous polyps, as well as desmoid tumors. We present the case of a 54-year-old woman with germline APC gene mutation, who underwent a total colectomy, subsequently developing two large infiltrative solid intra-abdominal lesions consistent with desmoid tumors. Medical treatment with Cox-2 inhibitors was initiated without result. She was submitted to resection for intestinal obstruction, but developed local recurrence. The lesions were also unresponsive to tamoxifen, and chemotherapy was initiated with dacarbazine plus doxorubicin, switching to vinorelbine plus methotrexate, achieving a good response in all lesions after 12 months. The approach to these intra-abdominal lesions should be progressive, beginning with observation, then a medical approach with non-steroidal anti-inflammatory drugs or with an anti-hormonal agent. Afterwards, if progression is still evident, chemotherapy should be started. Surgery should be reserved for resistance to medical treatment, in palliative situations, or for extra-abdominal or abdominal wall desmoids tumors.

Large desmoid tumors in familial adenomatous polyposis: a successful outcome.

Desmoid tumors develop from connective tissue, fasciae, and aponeuroses, and may occur in the context of familial adenomatous polyposis or may arise sporadically; also, they may be extra-abdominal, intra-abdominal, or located in the abdominal wall. These benign tumors have a great aggressiveness with a high rate of local recurrence. Familial adenomatous polyposis is an inherited condition with autosomal dominant transmission, and is characterized by the development of multiple colonic and rectal adenomatous polyps, as well as desmoid tumors. We present the case of a 54-year-old woman with germline APC gene mutation, who underwent a total colectomy, subsequently developing two large infiltrative solid intra-abdominal lesions consistent with desmoid tumors. Medical treatment with Cox-2 inhibitors was initiated without result. She was submitted to resection for intestinal obstruction, but developed local recurrence. The lesions were also unresponsive to tamoxifen, and chemotherapy was initiated with dacarbazine plus doxorubicin, switching to vinorelbine plus methotrexate, achieving a good response in all lesions after 12 months. The approach to these intra-abdominal lesions should be progressive, beginning with observation, then a medical approach with non-steroidal anti-inflammatory drugs or with an anti-hormonal agent. Afterwards, if progression is still evident, chemotherapy should be started. Surgery should be reserved for resistance to medical treatment, in palliative situations, or for extra-abdominal or abdominal wall desmoids tumors.

Hepatic perivascular epithelioid tumor (PEComa). A case report.

A 37-year-old female had liver nodules found in an abdominal ultrasound scan. The radiological features were suggestive of hepatocellular adenomas, although there were some atypical findings. Two years later, one of the nodules showed dimensional progression and intralesional hemorrhage. The patient underwent a left hepatectomy and the postoperative course was uneventful. The histological exam and the immunohistochemistry were consistent with the diagnosis of PEComa. The PEComa is a mesenchymal tumor rarely described in the liver. The preoperative diagnosis of PEComa is very difficult and, with this report, the authors intend to increase the limited knowledge relating to natural history and optimal treatment of this rare condition.

Malignant Small Bowel Tumors: Diagnosis, Management and Prognosis.

PURPOSE: Despite being rare entities, the incidence of malignant small bowel tumors seems to be rising. The development of capsule endoscopy and balloon assisted enteroscopy provided an advance in the assessment of small bowel lesions. We aim to describe the clinical and pathological characteristics of patients with small bowel cancer and ascertain what roles these endoscopic techniques currently have. MATERIAL AND METHODS: A retrospective study of patients diagnosed with small bowel cancer, from January 2010 to October 2014, was performed. The data was submitted to statistical analysis. RESULTS: Of the 28 diagnosed patients, 54% were female. The mean age at diagnosis was 61 years. Adenocarcinoma was the most frequent tumor (n = 11), followed by sarcoma (n = 6), lymphoma (n = 6) and neuroendocrine tumors (n = 3). The main form of presentation was related to blood loss or intestinal obstruction. By the time of diagnosis, 46% of patients had distant metastasis/ unresectable cancer. Most of the tumors were diagnosed by endoscopic (41%) or imaging techniques (35%). In the first year after diagnosis, 29% of patients died. In multivariate analysis, adenocarcinoma remained an independent factor for worse survival. DISCUSSION: Patients with adenocarcinoma presented at late stages and with unresectable tumors, contributing to a worse outcome. A high degree of clinical suspicion for the diagnosis of small bowel cancer is necessary. CONCLUSION: The characteristics of the patients were generally consistent with those described in the literature. Capsule endoscopy and balloon assisted enteroscopy are useful in the diagnosis, management and surveillance of small bowel cancer.

Introdução: Apesar de entidades raras, a incidência dos tumores malignos do intestino delgado parece estar a aumentar. O desenvolvimento da cápsula endoscópica e da enteroscopia assistida por balão permitiram um avanço na avaliação das lesões do intestino delgado. Temos como objetivo descrever as características clínicas e patológicas dos doentes com cancro do intestino delgado e averiguar o papel que estas técnicas endoscópicas assumem atualmente. Material e Métodos: Foi realizado um estudo retrospetivo dos doentes diagnosticados com cancro do intestino delgado, desde janeiro de 2010 até outubro de 2014. Os dados foram submetidos a análise estatística. Resultados: Dos 28 doentes diagnosticados, 54% eram do sexo feminino. A idade média ao diagnóstico foi de 61 anos. O tumor mais frequente foi o adenocarcinoma (n = 11), seguido do sarcoma (n = 6), linfoma (n = 6) e tumores neuroendócrinos (n = 3). A principal forma de apresentação esteve relacionada com perdas hemáticas ou obstrução intestinal. Ao diagnóstico, 46% dos doentes tinhammetástases distantes/tumor irressecável. A maioria dos tumores foi diagnosticada por técnicas endoscópicas (41%) ou imagiológicas (35%). No primeiro ano após o diagnóstico, 29% dos doentes faleceram. Na análise multivariada, o adenocarcinoma permaneceu fator independente para pior sobrevida. Discussão: Os doentes com adenocarcinoma apresentaram-se em estádios tardios e com tumores irressecáveis, contribuindo para um pior prognóstico. Ã necessário um elevado grau de suspeita clínica para o diagnóstico de cancro do intestino delgado. Conclusão: As características dos doentes foram globalmente consistentes com o descrito na literatura. A cápsula endoscópica e a enteroscopia assistida por balão são úteis no diagnóstico, gestão e vigilância do cancro do intestino delgado.

Laparoscopic cholecystectomy in acute cholecystitis in the elderly.

BACKGROUND/AIMS: Benefits of using laparoscopic cholecystectomy (LC) in the elderly with acute cholecystitis (AC) is still questionable. Our aim is to carry out a comparative analysis of that intervention in two groups of patients: under 65 years old, and over or equal to 65 years old. METHODOLOGY: This study corresponds to 249 LCs carried out during 4 years at the Emergency and Surgery Department of Hospital de S. João; 2 groups of individuals were considered: group A--under 65 years old, and group B--65 years old or over. The first consisted of 168 cases (88 men and 80 women, mean age 48.34 years (+/- 11.80)); and the second included 81 patients (40 men and 41 women, mean age 75.73 years (+/- 6.87)). The diagnosis was made on the basis of clinical symptoms, leukocytosis and ultrasound. RESULTS: The comparative analysis between these two groups provided the following results: 1) Mortality: 0% in A vs. 4.76% in B (P = 0.007); 2) Overall postoperative complications: 5.36% in A vs. 22.2% in B (P < 0.001); 3) Surgical complications: 4.76% in A vs. 14.8% in B (P = 0.468); 4) Intraoperative complications: 4.76% in A vs. 4.94% in B (P = 0.007); 5) Reoperations: 2.98% in A vs. 7.41% in B (P = 0.022); 6) Conversion: 12.50% in A vs. 17.28% in B (P = 0.447); 7) Lesion of the Main Bile Duct: 1.79% in A vs. 2.47% in B (P = 0.447); 8) Hospital Stay, equal to or less than 4 days: 72.62% in A vs. 27.16% in B (P <0.001). CONCLUSIONS: LC is a safe and efficient intervention in the treatment of acute cholecystitis in the elderly, although with greater morbidity and longer hospital stay, when compared with younger patients.

[Laparoscopic cholecystectomy and open cholecystectomy in acute cholecystitis: critical analysis of 520 cases]. / Colecistectomia por Laparoscopia e por Laparotomia na Colecistite Aguda: Análise Crítica de 520 Casos.

INTRODUCTION: Despite the skepticism with which it was initially seen, laparoscopic cholecystectomy is now the technique of choice for acute cholecystitis. It is, however, important to evaluate the results in comparison with classic cholecystectomy, since the latter is still used by some surgeons in certain situations. MATERIAL AND METHODS: Our research corresponds to the analysis of 520 patients operated on for acute cholecystitis performed in the department of surgery at the SÉo JoÉo Hospital in Oporto - 412 (79.2%) laparoscopic cholecystectomies and 108 (20.8%) open cholecystectomies - from 2007 to 2013. We evaluated comorbidities, leukocytosis, time between diagnosis and surgery, ASA, per and postoperative complications, mortality, reoperations, lesion of main bile duct, conversion rate and hospital stay, in order to compare these two techniques. The conversion group was included in laparoscopic cholecystectomy. Statistical analysis was based on descriptive statistic procedures and the evaluation of contrast between groups was based on Fishers' exact test. Significant values were considered for p < 0.05. RESULTS: Laparoscopic Cholecystectomy versus Open Cholecystectomy: Mortality: 0.7% vs 3,7% (p = 0.0369); Peroperative complications: 3.6% vs 12.9% (p = 0.0006); Surgical postoperative complications: 7.7% vs 17.5% (p = 0.0055); Medical postoperative complications: 4.3% vs 5.5% (p = 0.6077); Lesion of the main bile duct: 0.9% vs 1.8% (p = 0.6091); Reoperation: 2.9% vs 5.5% (p = 0.2315); Hospital stay up to 4 days after surgery: 64.8% vs 18.5% (p < 0.001). The convertion rate was of 10.7%: 8.8% in early surgery (before 4 days after de diagnosis) and 13.7% in the late surgery (after this time but in the same stay) (p = 0.1425). Multiple causes led to convertion: surgical complications (biliary lesions, iatrogenic lesion of the small bowel, perfurations of the gallbladder with spillage of stones); complications during the pneumoperitoneum, unclear anatomy and scoliosis. Postoperative complications in laparoscopic cholecystectomies converted group vs non-converted: surgical 20.4% vs 6.2% (p = 0.0034) and medical 6.8% vs 4.1% (p = 0.4484). DISCUSSION: There are few investigations concerning the comparison of laparoscopic cholecystectomy vs acute cholecystitis in patients with acute cholecystitis, corresponding mostly to multicenter studies. For this reason, we carry out an analysis inherent to 520 patients operated on with that disease in the surgery department of Hospital S. JoÉo in Oporto of which 412 were by laparoscopic cholecystectomy and 108 by acute cholecystitis. We found better results in laparoscopic cholecystectomy than in acute cholecystitis with respect to mortality, per and post-operative surgical complications and hospital stay. The incidence of main bile duct injury, medicalcomplications and reoperations, although less evident in laparoscopic cholecystectomy, were not statistically significant. There were more complications in the group of laparoscopic cholecystectomy converted than in those where it was not be necessary the conversion. This raises the need, in complications during the laparoscopic cholecystectomy, not to perform the conversion too late. The analysis of this study, therefore, properly values laparoscopic cholecystectomy in the surgery of patients with acute cholecystitis. CONCLUSION: The results justify the frequency with which laparoscopic cholecystectomy is performed in acute cholecystitis, in comparison to open surgery, thus taking an increasingly prominent place in the treatment of this disease.

IntroduçÉo: Apesar do cepticismo com que inicialmente foi encarada, a colecistectomia laparoscópica é hoje a técnica de eleiçÉo na colecistite aguda. Torna-se, porém, importante avaliar os seus resultados, em comparaçÉo com a colecistectomia clássica, uma vez que esta última ainda é seguida por alguns cirurgiões em determinadas situações.Material e Métodos: No nosso estudo foram incluídos 520 doentes com colecistites agudas operados no Serviço de Cirurgia Geral do Hospital de S. JoÉo, entre 2007 e 2013, dos quais 412 (79,2%) por laparoscopia e 108 (20,8%) por via aberta, com uma incidência de conversÉo de 10,7%. Procedeu-se ao estudo relativo às doenças coexistentes, leucocitose, tempo decorrido entre o diagnóstico na urgência e a cirurgia, classificaçÉo ASA, complicações intra e pós-operatórias, mortalidade, reintervenções, lesÉo biliar e estadia hospitalar. Os doentes convertidos foram incluídos no grupo das colecistectomias laparoscópicas. A análise estatística baseou-se em processos descritivos e a avaliaçÉo das diferenças entre grupos foi realizada com base no teste exato de Fisher, sendo considerados valores significativos para p < 0,05.Resultados: Colecistectomia laparoscópica versus Colecistectomia aberta: Mortalidade: 0,7% vs 3,7% (p = 0,0369); Complicações per-operatórias: 3,6% vs 12,9% (p = 0,0006); Complicações pós-operatórias cirúrgicas: 7,7% vs 17,5% (p = 0,0055); Pós-operatórias médicas: 4,3% vs 5,5% (p = 0,6077); LesÉo da via biliar principal: 0,9% vs 1,8% (p = 0,6091); Reintervenções: 2,9% vs 5,5% (p = 0,2315); Internamento hospitalar inferior ou igual a quatro dias: 64,8% vs 18,5% (p < 0,0001). Na colecistectomia laparoscópica houve 10,7% de conversões: nas precoces (intervenções realizadas antes das 96 h após o diagnóstico na urgência) esta taxa foi de 8,8% e nas tardias (após aquele período de tempo mas no mesmo internamento) de 13,7% (p = 0,1425); Complicações nos doentes convertidos vs nÉo convertidos: nas cirúrgicas 20,4% vs 6,2% (p = 0,0034) e nas médicas 6,8% vs 4,1% (p = 0,4484). As causas de conversÉoforam condicionadas por complicações cirúrgicas (lesões biliares, lacerações entéricas, perfurações vesiculares com a disseminaçÉo de cálculos), intoler'ncia ao pneumoperitoneo, indefiniçÉo do pedículo biliar e escoliose.DiscussÉo: Há poucas investigações relativas à comparaçÉo da colecistectomia laparoscópica vs colecistectomia aberta nos doentes com colecistectomia aberta, correspondendo a maior parte delas a estudos multicêntricos. Por esta razÉo, julgamos de interesse proceder a uma análise inerente a 520 operados com aquela doença no Serviço de Cirurgia Geral do Hospital de S. JoÉo dos quais 412 por colecistectomia laparoscópica e 108 por colecistectomia aberta. Verificamos na colecistectomia laparoscópica melhores resultados do que na colecistectomia aberta no que se refere à mortalidade, complicações per e pós-operatórias cirúrgicas e estadia hospitalar. A incidência da via biliar principal, complicações médicas e reintervenções, embora menos evidentes na colecistectomia laparoscópica, nÉo se revelaram com significado estatístico. Merece referência o maior número de complicações no grupo das colecistectomias laparoscópicasconvertidas do que naquelas em que tal nÉo foi necessário confirmando-se, assim, o já referido em estudos multicêntricos citados na literatura. Este facto levanta a necessidade de, mediante complicações ocorridas durante a colecistectomia laparoscópica, nÉo se proceder à conversÉo tardiamente. A análise do presente estudo valoriza, assim, devidamente a colecistectomia laparoscópica na cirurgia dos doentes com colecistite aguda.ConclusÉo: Os resultados obtidos justificam a frequência com que a colecistectomia laparoscópica é realizada na colecistite aguda, em comparaçÉo com a via aberta, ocupando cada vez mais, um lugar primordial, no tratamento desta doença.