Linfoma de células T/NK extranasal primario en íleon / Natural Killer/T cell primary extranasal ileum lymphoma

RESUMEN El linfoma extranodal de células T/NK extranasal (NKTL) primario del tracto gastrointestinal es poco frecuente y tiene carácter agresivo. Presentamos el caso de un paciente inmunocompetente de 51 años que ingresa por dolor abdominal de dos meses de evolución. En la colonoscopía, se hallaron ulceraciones ileales, por lo que se le realizaron estudios para descartar enfermedad de Crohn y tuberculosis intestinal, posteriormente presentó obstrucción intestinal. En la laparotomía exploratoria, se encontró un conglomerado ganglionar en mesenterio. La anatomía patológica confirmó el diagnóstico de NKTL primario en íleon. Se inició quimioterapia y el paciente pidió alta voluntaria dada su precaria condición. Dos meses después del alta el paciente fallece. Este linfoma ha sido reportado principalmente en Asia y posiblemente sea el primer caso reportado en Perú. Tiene un pronóstico funesto con una supervivencia global de ocho meses. Por ello, es necesario un diagnóstico precoz e iniciar la terapia oportunamente.

ABSTRACT Primary extranodal Natural Killer / T cell lymphoma (NKTL) on gastrointestinal tract is an uncommon and aggressive neoplasm. We present the case of a 51-year-old immunocompetent patient with a 2-month history of abdominal pain. Colonoscopy findings showed ileal ulcerations, so studies were carried out to rule out Crohn's disease and intestinal tuberculosis. Later, he developed intestinal obstruction. Exploratory laparotomy found a nodal conglomerate in the mesentery. Anatomical pathology confirmed the diagnosis of primary NKTL on the ileum. Chemotherapy was initiated but the patient asked for voluntary discharge because of his precarious condition. Two months after discharge the patient died. This lymphoma has been reported mainly in Asia and is possibly the first case in Peru. It has a dismal prognosis with overall survival of 8 months. Therefore, it is necessary to get an early diagnosis and begin therapy in a timely manner.

Ileítis como presentación de linfoma en síndrome de Wiskott-Aldrich / Ileitis as presentation of lymphoma in Wiskott-Aldrich syndrome

El síndrome de Wiskott-Aldrich (SWA) es un raro síndrome de inmunodeficiencia primaria ligado al cromosoma X que se asocia con aumento de incidencia de infecciones, trastornos autoinmunes y neoplasias. Se presenta el caso de un varón de 41 años con diagnóstico de síndrome de Wiskott-Aldrich y cuadro de ileítis como forma de presentación de un síndrome linfoproliferativo. La ileítis, en el contexto del paciente, representa un problema clínico dado el gran número de diagnósticos diferenciales (enfermedad inflamatoria intestinal, infecciones, neoplasias y enfermedades linfoproliferativas) por lo que suele requerir diagnóstico anatomopatológico y consideraciones particulares respecto al posterior tratamiento específico.

Wiskott-Aldrich syndrome is a rare X chromosome-linked primary immunodeficiency syndrome associated with an increased incidence of infections, autoimmune disorders and neoplasms. We present the case of a 41-year-old man with a diagnosis of Wiskott-Aldrich syndrome with ileitis as a form of presentation of a lymphoproliferative syndrome. The ileitis, in the context of the patient, represents a clinical challenge given the large number of differential diagnoses (inflammatory bowel disease, infections, neoplasms and lymphoproliferative diseases), so it usually requires anatomopathological diagnosis and particular considerations regarding the subsequent specific treatment.

Cistoadenoma mucinoso de la válvula ileocecal: tumoración intestinal infrecuente en un niño de 1 año. Reporte de caso / Mucinous cystadenoma of the ileocecal valve. Uncommon intestinal tumor in a oneyear-old boy. Case report

RESUMEN El cistoadenoma mucinoso se encuentra usualmente en el ovario, páncreas y el apéndice, pero su presentación en el intestino es extremadamente rara. En este reporte de caso, presentamos a un niño con obstrucción parcial intestinal debido a un cistoadenoma mucinoso en la válvula ileocecal. En la cirugía se retiró el íleo terminal, válvula ileocecal, ciego y apéndice, seguido de anastomosis ileocecal. El paciente evolucionó favorablemente en el postoperatorio y se recuperó sin contratiempos. A nuestro entender, este es el primer reporte de presentación de este tumor en dicha localización.

ABSTRACT Mucinous cystadenoma is usually found in the ovary, pancreas and appendix but its presentation in the intestine is extremely rare. In this case report we present an infant with partial intestinal occlusion due to a mucinous cystadenoma of the ileocecal valve. We performed an excision of the terminal ileum, ileocecal valve, cecum and appendix, followed by ileocolic anastomosis. The patient did well after the procedure and recovered uneventfully. To our knowledge, this is the first case report of this tumor in this location.

Multiple Neuroendocrine Tumor of the Distal Ileum / 대한소화기학회지

No abstract available.

A Case of Ileal Mucosa-associated Lymphoid Tissue Lymphoma Accompanied by Luminal Stricture and Arterial Spurting / 대한소화기학회지

Primary small intestinal lymphoma is relatively uncommon. Small bowel tumors are difficult to diagnose, because they are usually asymptomatic in the initial phase, and they are not easily detected by traditional methods of investigating the small intestine. This case shows a successfully detected and treated gastrointestinal bleeding from rare ileal mucosa-associated lymphoid tissue lymphoma, using double balloon endoscopy.

Tumor neuroendocrino de Íleon: a propósito de un caso / Ileum's neuroendocrine tumor: report of a case

Los tumores neuroendocrinos (TNE) del aparato gastrointestinal representan menos del 5% de las neoplasias del aparato gastrointestinal siendo el intestino delgado, en especial el íleon, donde se presentan mayor frecuencia (30%). Se originan del sistema endocrino difuso gastrointestinal y tienen capacidad de secretar péptidos y aminas bioactivas, principalmente la 5-hidroxitriptamina (5-HT) o serotonina. La Organización mundial de la salud (OMS) los clasifica de acuerdo al grado de diferenciación y conducta biológica en 5 grados. Las manifestaciones clínicas pueden estar ausentes o ser inespecíficas, su hallazgo, como en el caso de nuestra paciente, puede ser casual. Se reporta el caso de paciente femenino de 56 años la cual consultó por distensión abdominal y dolor anorrectal, realizándose colonoscopia con ileoscopia encontrando tumor subepitelial en íleon terminal con inmunohistoquímica que reportó: Tumor neuroendocrino bien diferenciado. El tratamiento de los TNE varía desde la resección quirúrgica en los estadíos menos avanzados hasta tratamiento quirúrgico asociado a quimioterapia y radioterapia en los más avanzados con una tasa de supervivencia a los 5 y 10 años de 73 y 65% para tumores bien diferenciado. De allí la importancia del diagnóstico precoz. Asimismo resaltar la canulación de retiro de la válvula ileocecal durante la colonoscopia de pesquisa.

The gastrointestinal neuroendocrine tumors (NET) represent less than 5% of the gastrointestinal tract neoplasms, being the small bowel, specially ileum, the most frequent location (30%). They originate from the diffuse endocrine gastrointestinal system and are able to secrete peptides and bioactive amines, mainly the 5-hidroxitryptamine (5-HT) or serotonine. The World Health Organization (WHO) classifies them according to its differentiation grade and biological behavior in 5 grades. The clinical manifestations could be absent or non specifics. Its finding, as in our patient case, can be casual. We report a case of a 56 years old female patient who complained of abdominal distention and anorectal pain. A colonoscopy with ileoscopy was performed finding a subepithelial tumor in the terminal ileum which inmunohistochemestry reported: Well differentiated neuroendocrine tumor. The treatment of NET varies from surgical resection alone in the less advanced stages, to surgical treatment associated to chemotherapy and radiotherapy in the most advanced stages, with a survival rate of 73 and 65% in the 5 and 10 years for the well differentiated tumors, being very important its early diagnosis. Equally must be highlighted the canulation of ileum cecal valve during a screening colonoscopy.

Fibrogenesis and carcinoid tumor: a case report

Carcinoid tumors are rare. They may appear in the entire gastrointestinal and respiratory tracts, with single or multiple occurrences. Prognosis is dependent on the size and location. Symptoms may appear in carcinoid syndrome, related to active substances, especially serotonin. One important aspect associated with these tumors and usually ignored is fibrogenesis. This is a case report of a patient with carcinoid tumor of the terminal ileum, treated by laparoscopy, associated with fat and fibrosis infiltration. (AU)

Tumores carcinoides são pouco frequentes, podem surgir em todo o trato gastrointestinal e respiratório, podem ser únicos ou múltiplos. O prognóstico depende do tamanho e da localização do tumor. Podem ocorrer sintomas relacionados à síndrome carcinoide, decorrente da produção de substâncias ativas, em especial serotonina. Um aspecto comumente ignorado associado a estes tumores é a estimulação da fibrogênese. Relatamos um caso de tumor carcinoide de íleo, tratado por videolaparoscopia, associado à infiltração fibroadiposa. (AU)

Linfoma de Celulas Grandes B Difuso Gastrointestinal del Anciano Asociado al Virus Epstein Barr: Reporte de Cinco Casos y Revision de la Literatura / Gastrointestinal Epstein Barr Virus positive diffuse large B cell lymphoma in elderly: report of five cases and review of literatura

El Linfoma de Células Grandes B difuso del anciano asociado al Epstein Barr es una nueva entidad incluida provisionalmente en la más reciente clasificación de neoplasias linfoides de la WHO. Usualmente afecta ancianos y tiene pobre sobrevida. El objetivo de este reporte fue evaluar las característica clínicas, endoscópicas y sobrevida de cinco pacientes portadores de esta entidad y compromiso gastrointestinal. Tres casos tuvieron infiltración gástrica y dos casos tuvieron compromiso del ileón y el ciego.

EBV-positive diffuse large B-cell lymphoma (DLBCL) in elderly is a new entity included provisionally in the most recent WHO Classification of lymphoid neoplasms. It usually affects elderly patients and has a poor survival. The goal of this report was to evaluate clinical, endoscopic characteristics and survival of five patients with this entity and gastrointestinal afectation. From five cases, three cases had gastric infiltration, one ileon and one in cecum.

Adenocarcinoma in an ileal duplication.

Alimentary tract duplications are a rare congenital anomalies that usually present in childhood and occasionally in adults. To our knowledge, since 1884, there have been only 14 reported cases of ileal duplications in adults of which 3 patients had developed malignancy in the duplicated ileum. We report here such an extremely rare case of isolated ileal duplication in an elderly male in whom malignancy occurred and perforated.

Synchronous carcinoid tumour of the small intestine and appendix in the same patient / Tumor carcinoide sincrónico del intestino delgado y el apéndice en el mismo paciente

Carcinoid tumours have been reported in a wide range of organs but most frequently involve the gastrointestinal tract. Many of these carcinoid tumours are associated with metachronous and synchronous lesions of another histological type. Primary carcinoid tumours of the different organ in the same patient is rare. In this paper, the authors present a case with synchronous carcinoid tumour of the small intestine and appendix in the same patient.

Se han reportado tumores carcinoides en una amplia variedad de órganos, pero el lugar de detección más frecuente ha sido el tracto gastrointestinal. Muchos de estos tumores carcinoides se hallan asociados con lesiones metacrónicas y sincrónicas de otro tipo histológico. La presencia de tumores carcinoides primarios de un órgano diferente en el mismo paciente es rara. En este trabajo, los autores presentan un caso con tumores carcinoides sincrónicos del intestino delgado y el apéndice en el mismo paciente.

A Case of Malignant Gastrointestinal Stromal Tumor of Ileum with Liver Abscess / 대한소화기학회지

Gastrointestinal stromal tumor (GIST) is an uncommon mesenchymal tumor of the gastrointestinal tract and is generally located in the stomach and small intestine. They usually present with abdominal pain, gastrointestinal bleeding, and palpable mass. Some patients present with rare symptoms that are more common in malignant GIST. Malignant GIST combined with a liver abscess has not been reported yet in the literatures. We report a case of 67-year-old woman who suffered from liver abscess combined by malignant GIST with central necrosis and fistula in the ileum. She complained of fever, chills, and abdominal pain. Abdominal CT scan showed huge liver abscess and ileal mass with air pocket. Small bowel series showed contrast material filling into the ileal GIST mass. An operation was performed and the final diagnosis was malignant GIST of the ileum with invasion into the sigmoid colon and urinary bladder.

A Case of Small Intestinal Signet Ring Cell Carcinoma in Crohn's Disease / 대한소화기학회지

Crohn's disease and ulcerative colitis are well known risk factors of intestinal cancer in relation to the extent and duration of disease. Rarely, small bowel cancer can develop after a longstanding inflammation of Crohn's disease with a relatively higher incidence than the general population. Signet ring cell carcinoma is a rare condition among intestinal cancers, and the diagnosis or detection is more difficult if the cancer originates from the small bowel. We report a case of a 30-year old female in whom signet ring cell carcinoma of ileum was diagnosed after a 15-year history of Crohn's disease.

Lipoma de la válvula ileocecal: informe de un caso clínico y revisión de la literatura / Ileocecal valve lipoma: case report and review of the literature

Introducción: los lipomas son los tumores benignos más frecuentes en tubo digestivo y hasta 50 % se encuentra en el colon. La mayoría son submucosos y varían de acuerdo a su incidencia, localización, sintomatología y patogénesis, lo que dificulta su diagnóstico. Nuestro objetivo fue conocer las diferentes manifestaciones clínicas causadas por un lipoma en la válvula ileocecal, y hacer una revisión de la literatura de esta localización poco frecuente. Caso clínico: mujer de 78 años que presentó cuadros repetidos de oclusión intestinal parcial con distensión abdominal, constipación y obstipación. Se realizó colonoscopia que mostró tumoración submucosa de 30 mm en válvula ileocecal; la tomografía computarizada de abdomen mostró tumoración nodular de densidad grasa en válvula ileocecal. La paciente fue sometida a laparotomía exploradora y cecotomía, donde se identificó y resecó tumoración submucosa, la cual fue enviada a estudio transoperatorio; dado que se trató de una tumoración benigna, se respetó la válvula ileocecal y se realizó cierre primario. El reporte histopatológico definitivo fue lipoma, negativo para malignidad. Conclusiones: es importante conocer las distintas formas de presentación de los lipomas, ya que fácilmente pueden confundirse con lesiones malignas (sobre todo si existe ulceración de la mucosa) y someter al paciente a procedimientos mayores. Sólo deben resecarse si producen síntomas o son mayores a 2 cm.

BACKGROUND: Lipomas are the most frequent benign tumors of the digestive tract and 50% are localized in the colon. Most are found submucosally and may vary in their incidence, localization, symptoms and pathogenesis, making diagnosis difficult. We undertook this study to learn the different presentations of an ileocecal valve (ICV) lipoma and conduct a review of the literature due to this uncommon location. CASE PRESENTATION: We present the case of a 78-year-old female with intermittent small bowel occlusion, abdominal distention and constipation. Colonoscopy showed a submucous tumor at the ICV, CT scan showed a tumor at the ICV with fat density. Laparotomy was performed with primary resection of the lesion by cecotomy, frozen section was reported as nonmalignant so a primary closure without ICV involvement was achieved. Definitive pathology revealed a benign lipoma. CONCLUSION: It is important to know the different presentations of these benign tumors because if there is mucosal ulceration they can be mistaken for a malignant lesion and lead to greater resections. Resection is necessary if they are symptomatic or >2 cm.

Primary small bowel melanoma: a case report.

The small intestine is the most common site of gastrointestinal metastasis from cutaneous melanoma. However, the small intestine itself is rarely the site of a primary melanotic tumour. We describe a case of primary small bowel melanoma in a 70-year-old man, and discuss the features of primary and metasttic melanoma.

Complicaciones agudas del linfoma yeyuno-ileal / Acute complications of jeyuno-ileal lymphoma

El linfoma de intestino delgado es una entidad cuya incidencia oscila entre el 1 y 4 por ciento de todos los tumores malignos del aparato digestivo. Es habitual su presentación clínica como cuadros agudos de abdomen, lo que determina que el cirujano se enfrente a los mismos en los servicios de urgencia. En estas circunstancias, y con un diagnóstico etiológico no siempre establecido, se emprende el tratamiento quirúrgico. Su confirmación anátomo patológica, permite emprender el tratamiento adyuvante en base a quimio y radioterapia. Se analizan en forma retrospectiva seis casos clínicos que se presentaron como cuadros agudos de abdomen y fueron sometidos a cirugía de urgencia, sin complicaciones ni mortalidad operatoria.

Ganglioneuroma of small intestine presenting with perforation peritonitis.

We report a 42-year-old man with benign solitary small intestinal ganglioneuroma presenting with perforation peritonitis. The patient had no evidence of MEN IIB syndrome. Simple segmental resection was done; the patient is well on follow up one year later.

Linfoma de región íleo-cecal: reporte de un caso / Ileocecal lymphoma

El linfoma de la región íleo-cecal es una forma poco frecuente de presentación de linfoma no Hodgkin (LNH) extranodal, patología poco frecuente en nuestro medio, desde el punto de vista de las imágenes y su evolución clínico imagenológica. Presentamos un paciente de sexo masculino, de 47 años de edad, con síndrome de FID, de aproximadamente un mes de evolución, con dolor, fiebre y tumefacción en la zona

Leiomyoblastoma of small intestine.

Gastrointestinal tumours are rare in children and leiomyoblastoma of small intestine is still rarer. We report a case of leiomyoblastoma of small intestine in a child who presented with acute intestinal obstruction.

Bleeding ileal schwannoma.

We report a case of ileal schwannoma who presented with bleeding per rectum. Three-vessel angiogram was the only diagnostic investigation, and revealed tumor blush in the mid-ileal region. On exploration, an intraluminal mass was found in the mid-ileal region along with a feeding vessel in the mesentery. Resection of the involved ileum with end-to-end anastomosis was done. Histopathology showed benign schwannoma of ileum involving the submucosa, muscularis propria and serosa.