Neoplasias del intestino delgado / Small intestinal neoplasms

Small intestine tumors are infrequent lesions during the routine clinical practice. They appear sporadically, in association with genetic diseases (e.g familiar adenomatous polyposis or Peutz-Jeghers syndrome), or associated to chronic inflammatory diseases (e.g Crohn’s disease or celiac disease). Benign tumors of small intestine (e.g leiomyomas, lipomas, adenomas, hamartomas or desmoid tumors) are generally asymptomatic, and may show up with intussusception. Primary malignant small intestine tumors (e.g adenocarcinoma, leiomyosarcoma, carcinoid tumor and lymphoma), can appear with intestinal obstruction, jaundice, digestive bleeding or abdominal pain. Small intestine metastatic lesions can appear by nearness, peritoneal metastasis or by hematological way. This last dissemination type is infrequent and more typically of melanoma. Because of its low prevalence, unspecific symptomatology and relative inaccessibility by conventional endoscopy, the diagnostic of small intestine neoplasm is often made several months after the first symptoms. Enteroclysis is a useful imaging technique towards the small intestine neoplasm suspicion. The endoscopic capsule and enteroscopy are actually the best diagnostic and therapeutic methods for this type of neoplasm. The treatment depends in the type of neoplasm, being the tumoral resection the first-line therapy.

Los tumores de intestino delgado son lesiones infrecuentes en la práctica clínica habitual. Aparecen de forma esporádica en asociación con enfermedades genéticas (por ej., poliposis adenomatosa familiar o síndrome de Peutz-Jeghers), o bien asociados a enfermedades inflamatoria crónicas intestinales (por ej., enfermedad de Crohn o enfermedad celíaca). Los tumores benignos de intestino delgado (por ej., leiomiomas, lipomas, adenoma, hamartoma o tumor desmoide) son generalmente asintomáticos, pudiendomanifestarse con intususcepción. Los tumores malignos primarios de intestino delgado (por ej.,adenocarcinoma, leiomiosarcoma, carcinoide y linfoma), pueden presentarse con obstrucción intestinal, ictericia, sangramiento digestivo o dolor abdominal. Las lesiones metastásicas de intestino delgado pueden aparecer por contigüidad, metástasis peritoneal o por vía hematógena. Este último tipo de diseminación es infrecuente y más típico del melanoma. Debido a su baja prevalencia, sintomatología inespecífica y relativa inaccesibilidad por endoscopía convencional, el diagnóstico de las neoplasias de intestino delgado es realizado a menudo varios meses después de iniciado los síntomas. La enteroclisis es una técnica de imagen útil frente a la sospecha de neoplasia de intestino delgado. La cápsula endoscópica y la enteroscopía son los métodos actualmente de mayor rendimiento para el diagnóstico y eventual terapia de este tipo de neoplasias. El tratamiento depende del tipo de neoplasia, siendo la resección tumoral la terapia de primera línea.

Neoplasias duodenais primárias: análise de casuística e conduta cirúrgica / Primary duodenal tumors: clinicopathologic experience and surgical treatment

São apresentados 18 casos de neoplasias primárias do duodeno, salientando-se sua raridade e as dificuldades diagnósticas, a despeito dos modernos recursos endoscópicos e de identificação por imagem. A literatura revela que de 1% a 10% de todos os tumores do aparelho digestório se situam no intestino delgado. A localização duodenal é, por isso, muito pouco freqüente, possui sintomatologia indefinida e implica conduta terapêutica mais complexa do que quando o tumor se situa em outros segmentos do intestino delgado. Os sintomas mais comuns são dor, náusea, vômito e hemorragia. A presente casuística consiste de 11 neoplasias malignase sete benignas. Entre as primeiras o adenocarcinoma é o mais freqüente, sendo os lipomas os mais comuns dosneoplasmas benignos. Enquanto nestes a exérese local representa a conduta mais adequada, a duodenopancreatectomiacefálica foi a cirurgia de escolha nos tumores malignos da segunda porção duodenal, exibindo bons resultados. As lesões malignas da terceira porção e principalmente da quarta porção do duodeno foram tratadas pela ressecção duodenojejunal, também com resultados satisfatórios.

Eighteen primary duodenal tumors, including 11 malignant and 8 benign, are presented, stressing their poorly defined natural history and rare frequency. The most common histological diagnosis was adenocarcinoma. There were 6 adenocarcinomas, 2 carcinoids, 1 linfoma, 1 anaplastic and 1 neuroectodermic carcinoma. Benign lesions were occasionally found during a gastroduodenal or a biliary surgery (n = 4) and during the autopsies (n = 3). They consisted of lipomas (n = 4), adenomatous polyps (n = 2) and leiomyoma (n = 1). Concerning the malignant lesions, 6 pancreaticoduodenectomies, 3 duodenojejunal segmentary resection and one paliation consisting of gastrojejunostomy and biopsy were performed. One carcinoid lesion was found at the duodenal bulb during a gastroduodenectomy for a gastric ulcer. Pancreaticoduodenectomy can be done with acceptable risk and it seems that patients with duodenal adenocarcinoma are more likely to survive longer after radical surgery than those with cancer of the head of pancreas. Every patient of this series treated by pancreaticoduodenectomy or duodenojejunal segmentary resection had an uneventful recovery and the follow-up ranged from 16 months to 7 years. As for the duodenal carcinoids, local resection is usually followed by recurrence, so an agressive surgical management is the best approach. Benign tumors must be treated by local excision when disclosed.