Jejunal stromal tumor and neurofibromatosis / Tumor estromal de jejuno e neurofibromatose

Abstract Gastrointestinal stromal tumors, although rare, are the most common primary mesenchymal neoplasms of the gastrointestinal tract and originate from the interstitial cells of Cajal. They present slow growth and symptoms such as bleeding, abdominal pain or discomfort, and the presence of an abdominal mass. The most affected organs are the stomach and small intestine. Differential diagnoses for gastrointestinal stromal tumor include adenocarcinoma and small intestine lymphoma, metastasis, and carcinoid tumor. Gastrointestinal stromal tumors have been associated with familial syndromes such as type 1 neurofibromatosis, considered a predisposing factor for tumors in the small intestine. This study aimed to report a case of gastrointestinal stromal tumor in the jejunal region in a patient with type 1 neurofibromatosis, followed-up for two years, who underwent laparoscopic segmental enterectomy and diagnosis determined by histopathology and immunohistochemistry. The diagnosis of small intestine gastrointestinal stromal tumor is challenging because of its low incidence, nonspecific symptoms, relative inaccessibility of the small intestine to conventional endoscopic examination, broad spectrum of radiological appearances, and the fact that the nature of the mass is difficult to determine with imaging examinations of the abdomen alone. Thus, the small intestine gastrointestinal stromal tumor may be erroneously diagnosed as pancreatic, gynecological, or mesenteric tumors. The literature does not present many reports on the association of jejunal gastrointestinal stromal tumor with neurofibromatosis. Understanding the tumoral behavior of small intestine gastrointestinal stromal tumor in this subgroup of patients would allow better follow-up.

Resumo Os tumores estromais gastrointestinais, embora raros, são as neoplasias mesenquimais primárias mais comuns do trato gastrointestinal e originam-se das células intersticiais de Cajal. Apresentam crescimento lento e manifestam sintomas como sangramento, dor ou desconforto abdominal e presença de massa abdominal. Os órgãos mais acometidos são estômago e intestino delgado. Os diagnósticos diferenciais para tumores estromais gastrointestinais incluem adenocarcinoma e linfoma de intestino delgado, metástases e tumor carcinoide. Os tumores estromais gastrointestinais têm sido associados a síndromes familiares como a neurofibromatose tipo 1, considerada um fator predisponente para tumores no intestino delgado. O objetivo desse trabalho é relatar um caso de tumor estromal gastrointestinal em região jejunal em paciente portadora de neurofibromatose tipo 1, com 2 anos de seguimento, submetida a enterectomia segmentar laparoscópica e diagnóstico determinados pela histopatologia e imuno-histoquímica. O diagnóstico de tumor estromal gastrointestinal do intestino delgado é desafiador, devido a sua baixa incidência, sintomas inespecíficos, relativa inacessibilidade do intestino delgado ao exame endoscópico convencional, amplo espectro de aparências radiológicas e difícil determinação da natureza da massa apenas com exames de imagens do abdome. Assim, tumor estromal gastrointestinal no intestino delgado podem ser erroneamente diagnosticados como tumores pancreáticos, tumores ginecológicos, ou tumores do mesentério. A descrição científica da associação de tumor estromal gastrointestinal de jejuno com neurofibromatose é incomum. Tais descrições permitem melhor seguimento dos pacientes a partir do momento que se entende o comportamento tumoral do tumor estromal gastrointestinal de intestino delgado nesse subgrupo de pacientes.

Most small bowel cancers are revealed by a complication / A maioria dos cânceres de intestino delgado são revelados por uma complicação

ABSTRACT Objective To characterize the pattern of primary small bowel cancers in a tertiary East-European hospital. Methods A retrospective study of patients with small bowel cancers admitted to a tertiary emergency center, over the past 15 years. Results There were 57 patients with small bowel cancer, representing 0.039% of admissions and 0.059% of laparotomies. There were 37 (64.9%) men, mean age of 58 years; and 72 years for females. Out of 57 patients, 48 (84.2%) were admitted due to an emergency situation: obstruction in 21 (38.9%), perforation in 17 (31.5%), upper gastrointestinal bleeding in 8 (14.8%), and lower gastrointestinal bleeding in 2 (3.7%). There were 10 (17.5%) duodenal tumors, 21 (36.8%) jejunal tumors and 26 (45.6%) ileal tumors. The most frequent neoplasms were gastrointestinal stromal tumor in 24 patients (42.1%), adenocarcinoma in 19 (33.3%), lymphoma in 8 (14%), and carcinoids in 2 (3.5%). The prevalence of duodenal adenocarcinoma was 14.55 times greater than that of the small bowel, and the prevalence of duodenal stromal tumors was 1.818 time greater than that of the small bowel. Obstruction was the complication in adenocarcinoma in 57.9% of cases, and perforation was the major local complication (47.8%) in stromal tumors. Conclusion Primary small bowel cancers are usually diagnosed at advanced stages, and revealed by a local complication of the tumor. Their surgical management in emergency setting is associated to significant morbidity and mortality rates.

RESUMO Objetivo Caracterizar o padrão de neoplasias malignas primárias do intestino delgado em um hospital terciário de Leste Europeu. Métodos Estudo retrospectivo de pacientes com câncer de intestino delgado, internados em um hospital terciário e de emergência, ao longo dos últimos 15 anos. Resultados Foram avaliados 57 pacientes com neoplasias malignas gastrintestinais, o que representou 0,039% das admissões e 0,059% das laparotomias realizadas. Total de 37 (64,9%) pacientes masculinos, média de idade de 58 anos, e de 72 anos para mulheres. Dentre os 57 pacientes, 48 (84,2%) foram internados em situação de emergência: obstrução intestinal em 21 (38,9%), perfuração em 17 (31,5%), hemorragia digestiva alta em 8 (14,8%), e hemorragia digestiva baixa em 2 (3,7%). Houve 10 (17,5%) tumores duodenais, 21 (36,8%) jejunais e 26 (45,6%) ileais. As neoplasias mais frequentes foram tumor estromal gastrintestinal, em 24 (42,1%) pacientes, adenocarcinoma em 19 (33,3%), linfoma em 8 (14%) e carcinoides em 2 (3,5%). A prevalência de adenocarcinoma duodenal foi 14,55 vezes maior do que a do intestino delgado, e a prevalência de tumores estromais duodenais foi 1,818 vez maior do que a do intestino delgado. A obstrução intestinal foi complicação do adenocarcinoma em 57,9% dos casos, e a perfuração foi a principal complicação local (47,8%) dos tumores estromais. Conclusão As neoplasias malignas primárias do intestino delgado foram geralmente diagnosticadas em estado avançado e reveladas por uma complicação local do tumor. O tratamento cirúrgico em situação de emergência está associado à significativa morbimortalidade.

Hemorragia digestiva baja recurrente secundaria a GIST de yeyuno / Recurrent gastrointestinal bleeding caused by a jejunal GIST

We report a 39 years old male with a history of three episodes of hematochezia and severe anemia. Upper and lower gastrointestinal endoscopies were normal. Labeled red blood cell scan, selective angiography and abdominal CAT scan identified a bleeding solid jejunal mass that was excised. The pathological report showed a gastrointestinal stromal tumor measuring 6 cm in diameter, with medium malignancy (two mitoses in 50 high magnification fields). Immunohistochemistry showed that it was CD 117 positive and CD 34 negative, smooth muscle actin and S 100 were positive in the malignant cells. After eight years of follow up, the patient is disease free.

La hemorragia digestiva baja (HDB) representa la forma clínica de presentación más frecuente de los tumores del estroma gastrointestinal (GIST) del segmento yeyuno-íleon. El diagnóstico es complejo por las dificultades para acceder a este nivel del tubo digestivo y habitualmente se efectúa después de varias hospitalizaciones. Se presenta el caso de un paciente varón de 39 años con tres episodios de hDb con anemia intensa (Hto 18 por ciento) cuyo estudio endoscópico alto y bajo no reveló anormalidades. La cintigrafía con glóbulos rojos marcados con Tc 99, la arteriografía selectiva y la tomografía computada de abdomen permitieron identificar una masa sólida de 5 cm en la zona paravertebral izquierda a nivel de L5, la que fue intervenida con el diagnóstico probable de GIST de intestino delgado. Se efectuó una pequeña resección yeyunal con anastomosis manual y el estudio histopatológico confirmó la presencia de un GIST de malignidad intermedia (6 cm, 2 mitosis en 50 campos de aumento mayor); IHQ compatible: CD 117: positivo; CD 34 negativo; Actina músculo liso y S 100: positivo focal en las células neoplásicas). Evolución alejada sin complicaciones luego de 8 años de seguimiento. El método diagnóstico más relevante en la actualidad es el Angio-TAC.

GIST Yeyunal con Sangrado Digestivo de Origen Oscuro / Jejunal GIST with obscure gastrointestinal bleeding

Reportamos el caso de una mujer de 84 años con antecedentes de arritmia cardiaca y hemorroides. Tenía múltiples hospitalizaciones y transfusiones por anemia ferropénica sintomática; los estudios endoscópicos solo demostraron divertículos y pequeños pòlipos de colon. Posteriormente fue hospitalizada por presentar heces sanguinolentas de color rojo vinoso; la endoscopia alta indicó gastritis y la colonoscopia mostró pequeñas úlceras colónicas, un pólipo colónico y múltiples divertículos. Meses después, reingresó con hemorragia de origen oscuro; en esa ocasión se demostraron: gastritis, erosiones antrales, pequeños pólipos colónicos y úlceras colónicas en vías de cicatrización; la cápsula endoscópica mostró probable angiodisplasia en yeyuno medio, la enteroscopia anterógrada detectó en yeyuno proximal algunas lesiones eritematosas sin evidencia de sangrado activo. Volvió a ser hospitalizada por melena y dolor abdominal, la endoscopia alta mostró angiodisplasias gástricas y duodenales que fueron tratadas. El último ingreso indicó un tiempo de enfermedad de dos años, el episodio se caracterizó por presentar deposiciones rojo vinosas y anemia. La endoscopia mostró angiodisplasia gástrica, que fue tratada con termocoagulación con argón plasma. En el examen no presentó signos de descompensación hipovolémica. Enfocado como un problema de hemorragia digestiva de origen oscuro se repitieron varios exámenes endoscópicos sin resultados. Resangró estando hospitalizada, se realizó cápsula endoscópica que demostró sangrado agudo en yeyuno, se complementó con nueva enteroscopia anterógrada que mostró lesiones ulceradas de yeyuno, se marcó el área con tinta china y se indicó laparotomía exploratoria. En la intervención quirúrgica se encontró en borde antimesentérico de yeyuno, una tumoración redondeada (6x6x4.5 cm) de crecimiento extraluminal, que comprometía la pared sin adherirse a otras estructuras; se realizó resección del tumor y anastomosis yeyuno-yeyunal. El estudio histológico -con inmunohistoquímica- del espécimen mostró que se trataba de un Tumor Estromal Intestinal (GIST), de riesgo intermedio, patrón histológico fusiforme, con escasas mitosis, dependiente de la capa muscular propia del intestino delgado. En conclusión el presente caso se trata de una mujer con un GIST yeyunal cuya presentación clínica fue una hemorragia de origen oscuro que constituyó un problema diagnóstico y que gracias al advenimiento de los nuevos procedimientos endoscópicos (enteroscopia y cápsula endoscópica) fue localizada y posteriormente extirpada quirúrgicamente.

We report the case of a woman of 84 years with a history of cardiac arrhythmia and hemorrhoids. She had multiple hospitalizations and transfusions for symptomatic iron deficiency anemia, endoscopic studies showed only small diverticula and colon polyps. He was later hospitalized with bloody stools red wines, upper endoscopy and colonoscopy showed gastritis, small colonic ulcers, colonic polyp and multiple diverticula. Readmitted with bleeding of obscure origin, on that occasion showed gastritis, antral erosions, small ulcers, colon polyps and colon ulcers in the process of healing, capsule endoscopy showed angiodysplasia in jejunum, anterograde enteroscopy detected some erythematous lesions in proximal jejunum without evidence of bleeding. Again hospitalized for melena and abdominal pain, upper endoscopy revealed gastric and duodenal angiodysplasia were treated. The last entry indicated a time of two years disease, the current episode with wine-red colored stools, Hb: 8.4 g, for which he received two units of PG. Endoscopy showed gastric angiodysplasia, which was treated with thermocoagulation (argon plasma). In the entrance examination showed no signs of hypovolaemic decompensation. Approached as a problem of obscure gastrointestinal bleeding were repeated several endoscopic examinations without results. She re-bled being hospitalized, capsule endoscopy was performed showing acute bleeding in the jejunum, complemented by new anterograde enteroscopy that showed ulcerated lesions of the jejunum, the area was marked with indian ink. Exploratory laparotomy was indicated. In the surgical intervention it was in edge antimesentérico of yeyuno, a round tumor (6x6 cm) of extraluminal growth, which compromised the wall without sticking to other structure, Resection of the tumor and jejuno-jejunal anastomosis was realized. The histological study with immunohistochemistry showed an Intestinal Stromal Tumor (GIST), intermediate risk, histological pattern fusiform, with scarce mitosis; the lesion was dependent on the muscularis propria of the small intestine. In conclusion, this case involves a woman with a jejunal GIST whose clinical presentation was hemorrhage of unknown origin which was a diagnostic problem and thanks to the advent of new endoscopic procedures (enteroscopy and capsule endoscopy) could locate the place of injury and subsequent surgery.

Hemorragia digestiva baja e intususcepción subsecuente a tumor del estroma gastrointestinal / Gastrointestinal bleeding and intussusception due to gastrointestinal stromal tumor (GIST)

Introducción: Los tumores del intestino delgado representan 25 % de las neoplasias gastrointestinales, de ellos 0.2 y 1 % corresponde a tumores del estroma gastrointestinal. Su presentación clínica más frecuente es la hemorragia digestiva, siendo los tumores del estroma gastrointestinal responsables en 1 % de los casos. Este tipo de neoplasias también puede ser el origen de intususcepción, proceso patológico infrecuente en la edad adulta por representar únicamente 5 % de todas las obstrucciones intestinales. Caso clínico: Mujer que acudió al servicio de urgencias por dolor abdominal y hemorragia digestiva baja. Las pruebas complementarias no resultaron concluyentes. Tras laparotomía exploradora se diagnosticó intususcepción y hemorragia digestiva baja subsecuente a tumor del estroma gastrointestinal. Conclusiones: La cirugía debe ser el último recurso diagnóstico y terapéutico, pero es necesaria en ocasiones debido a la dificultad para determinar la causa etiológica de la hemorragia y la obstrucción intestinal.

BACKGROUND: Small bowel tumors comprise 25% of gastrointestinal (GI) neoplasms, of which only between 0.2 and 1% correspond to gastrointestinal stromal tumors (GIST). GI bleeding is the most common presentation of GIST, being responsible in 1% of the cases. This type of neoplasm can also be the origin of an intussusception, which is an infrequent process during adult age, representing only 5% of all intestinal obstructions. CLINICAL CASE: We report a case of a patient who arrived at the emergency department due to abdominal pain and lower GI bleeding. The diagnostic process was not conclusive. After an exploratory laparotomy, the diagnosis was GI bleeding and intussusception due to GIST. CONCLUSIONS: Surgery must be the last diagnostic and therapeutic resource, but it is sometimes necessary to localize bleeding and intestinal obstruction site.

Small Intestinal Perforation Caused by Primary Jejunal MALT Lymphoma / 대한소화기학회지

No abstract available.

Jejunal Intussusception with Gastrointestinal Bleeding Caused by Metastatic Lung Cancer / 대한소화기학회지

Intestinal intussusception caused by metastatic tumor is uncommon. Symptomatic small bowel metastases from lung cancer have been rarely reported. Here we report a case of intussusception with gastrointestinal bleeding induced by jejunal metastasis of non-small cell lung cancer with a review of the literature. A 52-year-old man was admitted to our hospital because of melena. He had underwent right pneumonectomy and received systemic chemotherapy with radiotherapy for squamous cell lung cancer. Esophagogastroduodenoscopy and colonoscopy failed to reveal bleeding focus. Abdominal CT scan revealed jejunal intussusception and histologic examination of resected jejunum showed metastatic mass from lung cancer. In patients with small bowel obstruction and history of malignancies, possibility of small bowel metastatic tumor should be considered.

Intussusception Secondary to Jejunal Lymphangioma in a 13-year-old Girl / 대한소화기학회지

Lymphangioma is a benign tumor composed of numerous small or large thin-walled lymphatic spaces. It shows a predilection for head, neck, and axilla. Jejunal lymphangioma in children is extremely rare. We present a case of 13-year-old girl with jejuno-jejunal intussusception secondary to lymphangioma in jejunum. To our knowledge, this is the second report of jejunal lymphangioma causing intussusception in a child.

[ case report of metastatic choriocarcinoma to jejunum with severe lower GI bleeding]

A 33-year-old female presented with intermittent abdominal pain and severe attacks of lower gastrointestinal [GI] bleeding since 2 weeks. She has had history of irregular menses in last 3 months. In the labeled RBC-Scan study, the bleeding site was detected in left upper quadrant [LUQ]. In surgery, a tumor with the diameter of 2x1cm in a 65 cm distance of ligament of Trietz was detected. In pathological assessment jejunal metstatic choriocarcinoma was reported. Generally, choriocarcinoma is very rare among the intestinal metastatic tumors, including less than 5% of these tumors. Its presentation is acute lower GI bleeding and organ rupture, especially splenic rupture. These tumors will respond appropriately to the treatment in initial stages. Chemotherapy is the treatment of choice in these tumors

Segmental Jejunal Lipomatosis: A Rare Cause of Intestinal Obstruction

A rare case of a segmental small intestinal (jejunal) lipomatosis is described. A 33-year-old male was admitted with a clinical diagnosis of an acute intestinal obstruction. A plain erect abdominal x-ray showed multiple air-fluid levels. On an exploratory laparotomy, a jejunojejunal intussusception was found secondary to a segmental submucosal lipomatosis. This was treated by a segmental resection and anastomosis, which resulted in a complete cure. Here we present this case with a review of the relevant literature.

Small bowel leiomyoma: an unusual presentation.

Dual pathology having similar symptomatology is uncommon. We present a patient whose symptoms, although not absolutely typical of inguinal hernia, were attributed to bilateral irreducible inguinal hernias and were treated surgically. He remained symptomatic and later, was found to have an intraluminal leiomyoma of the jejunum, which was then removed. We suggest that the presence of any unusual clinical features should prompt further investigation to detect an alternative causative pathology.

Leiomiomas de yeyuno como causa de hemorragia digestiva baja masiva: análisis de 2 casos / Jejunal leiomyomas as cause of masive low digestive hemorrhage: analysis of 2 cases

La hemorragia digestiva baja masiva sigue siendo un problema difícil de resolver, a pesar de los avances en técnicas diagnósticas. Los tumores de intestino delgado constituyen sólo el 1-3 porciento de los tumores del tracto digestivo, y las lesiones intestinales en general conforman el 5 por ciento del total de las causas de hemorragias digestivas bajas. A pesar de su baja frecuencia, estos tumores, cuando se oresentan, plantean dificultad en su diagnóstico y tratamiento. Dado lo infrecuente de su aparición y por el desafío diagnóstico y terapéutico que plantean, se presentan dos casos de leiomiomas de yeyuno que debutaron como hemorragia digestiva masiva de origen desconocido y que fueron resueltas finalmente en forma quirúrgica

Congenital jejunal leiomyoma in a neonate.

Leiomyomas of the small intestine are rare in neonates. We report a neonate with jejunal leiomyoma, who presented with acute intestinal obstruction.

Tumores Jejuno-Ileais primários / Jejunum-ileal primary tumors

O propósito deste trabalho é fazer uma revisão detalhada da grande diversidade tumoral que acomete o segmento jejuno-ileal do tubo digestivo, buscando subsídios para um diagnóstico quanto possível precoce e um tratamento mais eficaz. Fazem parte deste estudo somente pacientes com localização tumoral primária no segmento jejuno-ileal do tubo digestivo (mestastáticos) e os com tumores duodenais e da junção íleo-cecal; excluíram-se também pacientes com quadros de hiperplasias, assim como os portadores de tumores de natureza puramente inflamatória.