Hemangiolinfangioma mesentérico: reporte de un caso / Mesenteric hemangiolymphangioma: case report

El hemangiolinfangioma es un tipo muy raro de malformación del sistema vascular, caracterizado histológicamente por la presencia de vasos venosos y linfáticos dilatados quísticamente, cuyas células endoteliales de revestimiento son positivas para marcadores de inmunohistoquímica como CD31, CD34 y D2-40. El compromiso extenso retroperitoneal y del tracto gastrointestinal es infrecuente. Se presenta el caso de una paciente femenina de 24 años con antecedente de dolor pélvico crónico, con exacerbación de síntomas. El diagnóstico imagenológico mostró una masa retroperitoneal multiquística. Se hizo hemicolectomía derecha y resección de la masa, encontrándose que dicha lesión estaba íntimamente adherida al mesenterio con compromiso extenso del tracto gastrointestinal, y cuyo estudio histopatológico reveló un hemangiolinfangioma, con mejoría clínica posterior a la resección quirúrgica. Aportamos a la literatura mundial, la caracterización de los hallazgos clínicos, imagenológicos e histopatológicos de este tipo de malformaciones

Hemangiolymphangioma is a very rare type of malformation of the vascular system, characterized histologically by the presence of cystically dilated venous and lymphatic vessels, whose lining endothelial cells are positive for immunohistochemical markers such as CD31, CD34 and D2-40. Extensive retroperitoneal and gastrointestinal tract involvement is uncommon. We present the case of a 24-yearold female patient with a history of chronic pelvic pain with exacerbation of symptoms. The imaging diagnosis revealed a multicystic retroperitoneal mass. A right hemicolectomy and resection of the mass was performed, finding that the lesion was intimately adherent to the mesentery with extensive involvement of the gastrointestinal tract, and whose histopathological study revealed a hemangiolymphangioma, with clinical improvement after surgical resection. We contribute to the world literature with the characterization of the clinical, imaging and histopathological findings of this type of malformations

Mesotelioma peritoneal multiquístico benigno: reporte de un caso y revisión de la literatura / Benign multicystic peritoneal mesothelioma: case report and literature review

INTRODUCCIÓN: El mesotelioma peritoneal multiquístico benigno (MPMB) es una entidad con muy baja incidencia que se presentan con mayor frecuencia en mujeres jóvenes. CASO CLÍNICO: Paciente de 17 años que consulta por amenorrea secundaria. Además, refería pérdida de peso, estreñimiento y molestias abdominales. La exploración fue normal y en las pruebas de imagen se objetivó una pelvis ocupada por una masa multiquística, de unos 20 cm de diámetro máximo, de paredes finas y contenido anecoico. Se solicitaron marcadores tumorales de los cuales sólo se encontró elevado el CA 19.9 (35,2 U/mL). Se decidió realizar una laparoscopia exploradora para establecer un diagnóstico y ante los hallazgos y al considerarse la masa fácilmente resecable, en el mismo acto quirúrgico se extirpó por completo para su estudio anatomopatológico, que confirmó un MPMB. Se desestimaron tratamientos adicionales y actualmente se encuentra en seguimiento y asintomática. CONCLUSIÓN: La patogénesis del MPMB no está clara. Algunos autores defienden que se trata de una reacción peritoneal a un daño inflamatorio, otros apoyan que se trata de una neoplasia benigna. Esta naturaleza incierta, junto con la escasa experiencia, hacen que el manejo resulte complejo. Es necesario individualizar cada caso e intentar ser conservadores especialmente en pacientes jóvenes. Si se opta por un manejo activo, lo más acertado es la citorreducción completa seguida de quimioterapia hipertérmica peritoneal. El seguimiento, en cualquier caso, debe ser exhaustivo y multidisciplinar principalmente con pruebas de imagen.

BACKGROUND: Benign Multicystic Peritoneal Mesothelioma (BMPM) is an entity with a very low incidence that occurs more frequently in young women. CASE REPORT: A 17-year-old woman attended for secondary amenorrhea. She also referred to weight loss, constipation and abdominal discomfort. The examination was normal and echography revealed a pelvis occupied by a 20 centimeters multicystic mass, with thin walls and anechoic content. A preoperative study with tumor markers was requested in which only a CA 19.9 elevation was observed (35.2 U/ml 0-27). It was decided to perform an exploratory laparoscopy to establish a diagnosis. Due to the findings and because of the fact that the mass was considered easy to remove, it was completely removed in the same surgical act. The pathological result confirmed a BMPM. Additional treatments have been dismissed and are the patient is currently being followed up without symptoms. CONCLUSION: The pathogenesis of BMPM is unclear. Some authors argue that it is a peritoneal reaction to inflammatory damage, others support that it is a benign neoplasm. This uncertain nature coupled with limited experience make management complex. It is necessary to evaluate each case and try to be conservative especially in young patients. If surgery management is chosen, complete cytoreduction followed by hyperthermic peritoneal chemotherapy is the most successful option. The follow-up in any case must be exhaustive and multidisciplinary, mainly with imaging tests.

Quimioterapia intraperitoneal en aerosol presurizado (PIPAC) / Pressurized intraperitoneal aerosol chemotherapy (PIPAC)

RESUMEN La técnica PIPAC se presenta como una variante de tratamiento para los pacientes con carcinomato sis peritoneal que no son candidatos a una resección. Se describen de manera detallada los pasos y el procedimiento quirúrgico para la administración de quimioterapia intraperitoneal presurizada con dispositivo PIPAC.

ABSTRACT Pressurized intraperitoneal aerosol chemotherapy (PIPAC) is a therapeutic option for patients with unresectable peritoneal carcinomatosis. The steps and the surgical technique of the PIPAC technique are thoroughly described.

Peritoneal carcinomatosis treated with cytoreductive surgery and intraperitoneal chemotherapy / Carcinomatose peritoneal tratada com cirurgia citoreductiva e quimioterapia intraperitoneal

ABSTRACT Introduction: To evaluate the combined treatment with cytoreductive surgery and intraperitoneal chemotherapy for peritoneal carcinomatosis arising from colorectal cancer, pseudomyxoma peritonei and mesothelioma. Methods: Data were obtained from 73 patients with peritoneal carcinomatosis arising from colorectal cancer (52.1%), pseudomyxoma peritonei (41.1%) or mesothelioma (6.8%) between 2002 and 2011. We reported the morbidity grade (II, III and IV), mortality and survival rates of the candidates after cytoreductive surgery and intraperitoneal chemotherapy. Results: 41 (56.2%) women participated, and the median age was 50 years. Thirty-nine patients (53.4%) underwent complete cytoreductive surgery and intraperitoneal chemotherapy. Patients who underwent a complete cytoreduction received intraperitoneal chemotherapy with mitomycin C, from which only 16/39 (41%) had hyperthermic intraperitoneal chemotherapy (41-42 °C). The overall morbidity rate was 23.3% and the grade III/IV complication rate was 12.3%. The overall mortality rate was 5.5%. The univariate analysis showed that cytoreductive surgery and intraperitoneal chemotherapy (p = .029), a blood transfusion (p = .002) and the operative time (p = .001) were significant for the occurrence of postoperative complications. Patients with peritoneal carcinomatosis from colorectal cancer who underwent complete cytoreductive surgery and intraperitoneal chemotherapy had overall survival rates of 81.3%, 12.5% and 12.5% at 1, 3 and 5 years, respectively. Patients with peritoneal carcinomatosis from pseudomyxoma peritonei who underwent complete cytoreductive surgery and intraperitoneal chemotherapy had overall survival rates of 84.2%, 77.7% and 77.7% at 1, 3 and 5 years, respectively. Conclusion: The combined treatment for peritoneal carcinomatosis may be performed safely with acceptable morbidity and mortality in a specialized unit setting. Although over half of patients underwent normothermic intraperitoneal chemotherapy, our results were comparable to results from others centers.

RESUMO Introdução: O objetivo foi avaliar o tratamento combinado da cirurgia citorredutora e quimioterapia intraperitoneal em pacientes com carcinomatose peritoneal secundária ao câncer colorretal, pseudomixoma peritoneal e mesotelioma. Métodos: Foram obtidos dados de 73 pacientes com carcinomatose peritoneal secundária ao cirurgia citorredutora (52.1%), pseudomixoma peritoneal (41,1%) ou mesotelioma (6,8%). Foram avaliados o grau de morbidade, a taxa de mortalidade e as taxas de sobrevida após a cirurgia citorredutora e quimioterapia intraperitoneal. Resultados: 41 (56,2%) pacientes do sexo feminino participaram, com média de idade de 50 anos. 39 pacientes (53,4%) foram submetidos a cirurgia citorredutora completa e quimioterapia intraperitoneal. Todos esses receberam Mitomicina C, sendo 16/39 (41%) quimioterapia intraperitoneal hipertérmica (41-42°C). A morbidade global foi 23,3%, com taxa de mortalidade global de 5,5%. A análise univariada mostrou que câncer colorretal e quimioterapia intraperitoneal (p = .029), transfusão sanguínea (p = .002) e tempo operatório (p = .001) foram associados com complicações pós-operatórias. Pacientes com carcinomatose peritoneal secundária ao cirurgia citorredutora submetidos a cirurgia citorredutora completa e quimioterapia intraperitoneal tiveram sobrevida global de 81,3%; 12,5% e 12,5% em 1, 3 e 5 anos, respectivamente. Os pacientes com pseudomixoma peritoneal que foram submetidos a cirurgia citorredutora completa e quimioterapia intraperitoneal tiveram sobrevida global de 84,2%; 77,7% e 77.7% em 1, 3 e 5 anos, respectivamente. Conclusão: O tratamento combinado para carcinomatose peritoneal é seguro quando realizado em centros terciários com experiência no procedimento. Embora mais da metade dos pacientes tenham sido submetidos a quimioterapia intraperitoneal normotérmica após a cirurgia citorredutora completa, os resultados podem ser comparados a de outros centros que utilizam exclusivamente a quimioterapia hipertérmica.

Quimioterapia Aerossolizada e Pressurizada Intraperitoneal (PIPAC) por monoportal: aplicação alternativa no controle das metástases peritoneais / Pressurized Intraperitoneal Aerosol Chemotherapy (PIPAC) through a single port: alternative delivery for the control of peritoneal metastases

RESUMO A carcinomatose peritoneal é a evolução natural das neoplasias gastrointestinais, ginecológicas e primárias do peritônio. Nos últimos anos, a carcinomatose passou a ser considerada uma doença confinada ao peritônio, e não mais uma doença disseminada. Desta forma, a associação de citorredução cirúrgica associada à quimioterapia intraperitoneal se tornou o ponto chave no controle das metástases peritoneais. Tradicionalmente, a quimioterapia intraperitoneal é aplicada utilizando soluções líquidas. Uma nova modalidade de infusão da quimioterapia na cavidade abdominal surge como uma alternativa ao método tradicional. A chamada PIPAC (Pressurized Intraperitoneal Aerosol Chemotherapy) transforma a solução terapêutica líquida em um spray aerossolizado, potencializando a distribuição e penetração da quimioterapia intraperitoneal. Este relato tem por objetivo descrever essa nova técnica cirúrgica inovadora, realizada pela primeira vez por um monoportal no Brasil, alterando a forma descrita originalmente para a aplicação da PIPAC.

ABSTRACT Peritoneal carcinomatosis is the natural course of gastrointestinal, gynecologic, and primary peritoneal neoplasms. In recent years, our understanding of carcinomatosis has changed; it is no longer considered a disseminated condition, but rather a disease confined to the peritoneum. Thus, the combination of cytoreductive surgery and intraperitoneal chemotherapy has become the cornerstone of control of peritoneal metastases. Traditionally, intraperitoneal chemotherapy is delivered in the form of liquid solutions. However, a new mode of chemotherapy delivery to the abdominal cavity has arisen as an alternative to the conventional method. In Pressurized Intraperitoneal Aerosol Chemotherapy (PIPAC), the liquid solution is aerosolized into a spray, potentiating the distribution and penetration of the chemotherapeutic agent intraperitoneally. The present study aims to describe a novel form of this innovative surgical technique performed for the first time in Brazil, in a modification of the technique originally described for PIPAC: delivery through a single-port device.

Effects of nivolumab in peritoneal carcinamatosis of malign melanoma in mouse model

Abstract Purpose: To evaluate the efficacy of nivolumab and comparison with dacarbazine (DTIC) on peritoneal carcinomatosis of malignant melanoma in mouse model. Methods: Mouse skin melanoma cells was injected under the capsule of the peritoneal surface in the left side of the abdomen. On postoperative day ten, mouses randomised into three groups. Group 1: Control, Group 2: HIPEC (Hyperthermic intraperitoneal chemotherapy) with DTIC and Group 3: HIPEC with Nivolumab. After the sacrification on postoperative day fifteen, peritoneum evaluated macroscopically and histopathologically by using peritoneal regression grading score (PRGS). Results: In the 15th day exploration, all animals developed extensive intraperitoneal tumor growth in Group 1. In Group 2 and Group 3 median tumor size was 0.7±0.3cm and 0.3±0.2cm respectively (p: 0.023). Peritoneal carcinomatosis index (PCI) were significantly lower in Group 3 than other groups (p: 0.019). The lowest total tumor nodules in group 3 was 4 ± 2. The PGRS score was found significantly lower in Group 3 than other groups (p: 0.03). Lymphocytic response rate was found higher in the Group 3. Conclusions: It has been found that nivolumab significantly better than DTIC on peritoneal metastases of malign melanoma in mouse models. Nivolumab treatment gives promising results with pathological evidence in the treatment of metastatic disease of malignant melanoma.

Citorreduccion con quimioterapia / Citorreduction with chemotherapy

Hasta no hace mucho tiempo el cancer en la superficio peritoneal estaba asociado con un pesimo pronostico. Esta forma extrema de presentacion de algunos tumores de origen peritoneal, digestivo o ginecologico, que se diseminan por vía transcelomica, tenía terapias solamente paliativas. En la actualidad algunos pacientes con diseminacion peritoneal del cancer pueden beneficiarse de un tratamiento que asocia cirugia de citorreduccion y quimoterapia intraperitoneal para brindar una alternativa terapeutica que puede mejorar el tiempo de supervivencia y la calidad de vida de las personas. En este trabajo intento demostrar el desarrollo y propositos de una cirugía desafiante en lo que respecta a nuestro rol como instrumentadores, la citorreducción. La misma consiste en la reseccion de toda enfermedad macroscopica, la que es visible al ojo humano sin ayuda de microscopio y luego la deliminación de la enfermedad microscopica mediante la utilización intraperitoneal de farmacos quimioterapicos a alta temperatura. En la actualidad el Sanatorio Allende es el unico centro de salud de la provincia de Cordoba y unos pocos en el pais, que tiene un programa de estas caracteristicas.

Leiomiomatosis benigna metastásica: reporte de caso clínico / Benign intraperitoneal metastatic leiomyomatosis: a case report

Benign intraperitoneal metastatic leiomyomatosis is a rare benign disease that is observed when a leiomyoma is present in the peritoneal surface. Women who have undergone hysterectomy for leiomyomas are most commonly affected. Patients are usually asymptomatic at presentation, being frequently an incidental finding in imaging studies. Ultrasound and CT play an important role in the diagnosis. The lesions are histologically identical to their uterine counterparts. There are different theories about the pathogenesis of the disease, including peritoneal seeding after laparoscopic hysterectomy. Others support the hypothesis of multiple independent foci of smooth muscle proliferation. Treatment, as in uterine leiomyomatosis, is generally conservative. We report a 53-year-old hysterectomized woman with intraperitoneal leiomyomas detected in a routine physical examination as mobile abdominal masses who underwent successful laparoscopic resection.

Neurofibroma del mesenterio en un paciente sin enfermedad de Von Recklinghausen / Mesenteric neurofibroma seen in a patient without Von Recklinghausen´disease

Los neurofibromas son tumores benignos del sistema nervioso que pueden ser solitarios o múltiples: la neurofibromatosis o enfermedad de Von Recklinghausen. Estos tumores en general son de consistencia blanda, frecuentes a nivel de partes blandas, su localización en el aparato digestivo es infrecuente y no son encapsulados. El objetivo del presente trabajo fue presentar el caso de una paciente con neurofibroma mesentérico sin enfermedad de Von Recklinghausen. Se presenta el caso de una paciente de 41 años que fue ingresada por dolor abdominal y una tumoración palpable en la región entre flanco e hipocondrio izquierdo. En los estudios imaginológicos realizados se informó como quiste del mesenterio, lo que fue confirmado en el acto quirúrgico. Se realizó exéresis de la tumoración informada por Anatomía Patológica como neurofibroma secundariamente enquistado benigno. El neurofibroma mesentérico es una tumoración que puede verse en pacientes sin enfermedad de Von Recklinghausen.

Neurofibromas are benign tumors of the nervous system that can be solitary or multiple, the neurofibromatosis or Recklinghausen's disease. In general, these tumors are soft, non-encapsulated, more frequent in soft parts of the body and infrequent in the digestive system. The objective of the present paper was to present a female patient with mesenteric neurofibroma and without Von Recklinghausen disease. Here is a 41 years-old female patient who was admitted to the hospital because she suffered abdominal pain and a palpable tumor at the region between left flank and hypochondrium. The imaging studies revealed a mesenteric cyst, which was confirmed in surgery. The tumor was classified as a benign cyst neurofibroma by pathological anatomy and finally removed. The mesenteric neurofibroma is a tumor that can be found in patients without Von Recklinghausen disease.

Mesotelioma benigno como causa de dolor abdominal inespecífico en un paciente con Enfermedad de Crohn / Benign multicystic peritoneal mesothelioma in a patient with Crohn disease

Benign multicystic peritoneal mesothelioma is an uncommon lesion arising from the peritoneal mesothelium. It is asymptomatic or presents with unspecific symptoms. Imaging techniques may reveal it, however the final diagnosis can only be made by histopathology. Surgery is the only effective treatment considering its high recurrence rate. We report a 19 years old male with Crohn’s disease. Due to persistent abdominal pain, an abdominal magnetic resonance imaging was performed, showing a complex cystic mass in the lower abdomen. The patient underwent surgery and the lesion was completely resected. The pathological study reported a benign multicystic peritoneal mesothelioma.

Rol de la cirugía citoreductora y quimioterapia hipertérmica en el tratamiento de la carcinomatosis peritoneal / Cytoreductive surgery and hyperthermic chemotherapy role in peritoneal carcinomatosis treatment

El objetivo de este artículo es ofrecer una revisión sobre algunas estrategias terapéuticas actuales para pacientes con carcinomatosis peritoneal (CP), la que históricamente ha sido considerada como una condición terminal, sólo susceptibles de tratamientos y cuidados paliativos; y ocasionalmente de qui-mioterapia sistémica aislada con mala respuesta y supervivencias promedio inferiores a los 6 meses. La combinación de cirugía de citoreductora (CCR), que implica peritonectomía y resecciones multiviscerales con hipertermia y quimioterapia intraperitoneal intraoperatoria (HIPEC), es un concepto relativamente nuevo en el tratamiento de la enfermedad microscópica residual intraperitoneal. La indicación de este método se ha ido extendiendo de forma progresiva, para pacientes con CP muy bien seleccionados; y tiene base en varios estudios de fase III, cuya conclusión es que se puede obtener suficiente citoreducción. Sin embargo, hasta el momento existe cierto consenso basado en la evidencia, en que estaría indicada en pacientes portadores de pseudomixoma peritoneal, mesotelioma peritoneal y cáncer colorrectal; y en CP de otro origen, dependiendo de cada caso en particular.

The aim of this article is to provide a review of some current therapeutic strategies for patients with peritoneal carcinomatosis (PC), which historically has been considered a terminal condition, only amenable to palliative care, and occasionally systemic chemotherapy alone with poor response and lower average survival. The combination of cytoreductive surgery (CRS), which involves peritonectomy and multivisceral resections and intraoperative hyperthermic intraperitoneal chemotherapy (HIPEC) is a relatively new intraperitoneal treatment of microscopic residual disease concept. The indication of this method has been extended progressively to highly selected patients with PC and it is based in several phase III studies, which concluded that it is possible to obtain sufficient cytoreduction. However, until now there is some consensus based on evidence, in which HIPEC would be indicated in patients with peritoneal PC originated in pseudomyxoma, peritoneal mesothelioma and colorectal cancer; and CP from another source, depending on each particularcase.

Tumoración pélvica en una paciente con síndrome de li fraumeni / Pelvic tumor in a patient with li-fraumeni syndrome

El síndrome de Li Fraumeni (SLF) es una rara enfermedad hereditaria asociada con un riesgo incrementado de padecer ciertos tumores malignos. Presentamos el caso de una paciente con diagnóstico de SLF con antecedentes de sarcoma de glúteo con metástasis pulmonares y cáncer de mama bilateral metacrónico. Acudió al Servicio de Urgencias por distensión y dolor abdominal. Se objetivó una masa pélvica y se pensó en un probable origen ovárico de la misma. La paciente fue intervenida en el Servicio de Ginecología, y durante la intervención se descartó dicho origen ya que la tumoración dependía del epiplón. El diagnóstico final fue metástasis de sarcoma.

Li-Fraumeni syndrome (LFS) is a rare, inherited syndrome associated with increased risk of various malignant tumors. We present the case of a patient diagnosed LSF with a history of gluteal sarcoma with lung metastases and metachronous bilateral breast cáncer. She came to the emergency department for abdominal bloating and pain. She had a pelvic mass and we had thought probable ovarian dependence. The patient was operated on at the Department of Gynecology, and during the intervention we realized that the tumor depended on the omentum. The final diagnosis was a metastatic of sarcoma.

Staging laparoscopy in gastroesophageal and gastric adenocarcinoma: First experience from Pakistan.

CONTEXT: Current NCCN guidelines do not consider staging laparoscopy mandatory for detection of metastasis in gastroesophageal junction (GEJ) and gastric cancer. AIMS: To determine the rate of detection of metastasis on staging laparoscopy in GEJ and gastric cancer in Pakistani population and determine the prognostic significance of cytology versus biopsy positive metastatic disease. SETTINGS AND DESIGN: Retrospective study conducted from January 2005 to June 2013. MATERIALS AND METHODS: Demographics, clinicopathological characteristics and laparoscopic findings of 149 patients were compared. STATISTICAL ANALYSIS USED: Categorical variables were represented as frequencies and percentages and significance was determined using Chi square test. Overall survival was calculated from the date of staging laparoscopy to the date of death/ last follow‑up. Survival for cytology versus biopsy positive metastatic disease was calculated using Kaplan Meier curves and significance determined with Log rank test. RESULTS: Overall, metastases were detected in 40% of patients on staging. Laparoscopy detected metastasis in significantly high number of gastric cancers (48% versus 28%) (P = 0.01). Peritoneal nodules were more frequent with gastric tumors (40% versus 23%) and also were more likely to be malignant (58% versus 35%). Expected one year survival in patients with positive cytology (peritoneal washing/ascitic fluid) was significantly higher than patients with a positive peritoneal nodule biopsy (29% versus 0) (P = 0.04). On univariate analysis this was the only significant factor for increased risk of death (P = 0.03, HR = 2.5, CI = 1.04‑5.98). CONCLUSIONS: Staging laparoscopy detects metastatic disease in a significant number of patients deemed non metastatic on preoperative imaging. Prognostically, cytology positive metastatic cancer may be different from biopsy positive cancer.

Teratoma inmaduro con gliomatosis peritoneal y embarazo / Immature teratoma with peritoneal gliomatosis in pregnancy

Presentamos el caso clínico de una paciente de 18 años sin antecedentes a destacar. Cursando las 30 semanas de embarazo se constata una tumoración abdominal que requirió tratamiento quirúrgico y se diagnosticó un teratoma inmaduro grado 3, asociado a gliomatosis peritoneal. Se realizó una cesárea al término y se instauró el tratamiento con quimioterapia presentando una recidiva a los siete meses que requirió tratamiento quirúrgico conservador y nuevo plan de quimioterapia estando actualmente libre de enfermedad y en seguimiento. Lo infrecuente de esta patología y asociada a embarazo, motivó su comunicación.

We report the case of a healthy 18-year old patient with adnexal mass diagnosed by her 30th week of pregnancy. It required surgical treatment and a grade 3 immature teratoma associated with peritoneal gliomatosis was diagnosed. A cesarean section was performed at term and subsequently chemotherapy was established. The patient presented a recurrence seven months later, which required fertility-sparing surgery and a new chemotherapy plan. The patient is currently disease-free and under surveillance. The infrequency of this disease and associated with pregnancy, led to their communication.

Tumor del estroma extra gastrointestinal (E-GIST) gigante de omento / Extra gastrointestinal stromal tumor (E-GIST) of the omentum

Introduction: Tumors that are pathologically and immunohistochemically similar to gastrointestinal stromal tumors (GIST), but coming from soft tissues of the mesentery or retroperitoneum are called extra gastrointestinal stromal tumors (E-GISTs) and are more aggressive than GISTs. Clinical case: We report a 46 years old woman operated for a uterine sarcoma with a liver metastasis of the tumor, subsequently subjected to radiotherapy. A new CAT scan showed a 21 x 12 cm tumor. Radiotherapy was discontinued and the patient refused chemotherapy. Four years later, the patient consulted in the emergency room for a partial bowel obstruction. A new CAT scan showed a tumor that occupied the abdominal and pelvic cavity. The patient was operated, finding a highly vascularized and multisystem tumor that was fused together with the omentum, which was excised. The pathological diagnosis of the surgical piece was a high risk GIST.

Introducción: Los tumores del estroma gastrointestinal (GIST) fueron clasificados inicialmente dentro de otros grupos de tumores (leiomioma, leiomioblastoma, leiomiosarcoma), llamándolos leiomiomas bizarros o leiomiomas celulares debido a su apariencia histológica similar. Con el advenimiento de la microscopia electrónica se demostró que sólo algunos de estos tumores presentaban evidencias ultraestructurales de diferenciación muscular lisa. Posteriormente, los avances en la biología molecular y la inmunohistoquímica han permitido diferenciarlos de otras neoplasias digestivas e identificarlos como una patología clínica e histopatológica propia. Los tumores que histopatológicamente e inmunohistoquímicamente son similares a los GIST, pero proceden de los tejidos blandos del mesenterio o el retroperitoneo sin presentar conexión, ni siquiera tenue, con la pared ni con la superficie serosa de las vísceras, son denominanados tumores del estroma extragastrointestinal (E-GIST) y han mostrado tener un comportamiento más agresivo que los GIST originados a nivel gástrico y similar a los intestinales. Reporte del caso: Paciente de 47 años quien desarrolló durante 8 años un tumor abdominal, que resultó ser un tumor del estroma gastrointestinal de omento, sometida a tratamiento quirúrgico. En 18 meses no ha presentado recidiva tumoral.

Linfangiomatosis mesentérica como causa de abdomen agudo en la tercera edad / Mesenteric lymphangiomathosis presenting as an acute abdomen in an older subject

We report a 71 years old male who consulted in the emergency room for abdominal pain lasting 12 hours. On physical examination there was pain on abdominal palpation and signs of peritoneal irritation. An abdominal CT scan showed a thickening of the medial and distal ileum, multiple adjacent collections and signs of peritonitis. The patient was operated, observing multiple white tumors in the mesentery and serosa of small bowel, one of these lesions, adhered to the bowel wall, had a hemorrhagic infarct. One of the lesions was punctured, obtaining a milky fluid. Biopsies were obtained and the infarcted lesion was excised. The pathological study reported a Mesenteric Multilocular Lymphangioma. The patient had an uneventful postoperative evolution.

Presentamos el caso clínico de un hombre de 71 años, sin antecedentes mórbidos, salvo apendicectomía y colecistectomía hace más de 20 años. Consulta en servicio de urgencias por dolor abdominal de 12 horas de evolución, sin otros síntomas asociados. Al examen físico destacaba dolor abdominal e irritación peritoneal a la palpación de hipocondrio izquierdo. Exámenes destacan aumento de parámetros inflamatorios, Ia tomografía computada de abdomen y pelvis revela engrosamiento de íleon medio-distal, con múltiples colecciones adyacentes y signos de peritonitis. Se realiza laparotomía exploradora, evidenciándose múltiples tumoraciones blanquecinas en mesenterio y serosa de todo el intestino delgado, una de las cuales se aprecia con infarto hemorrágico adherida a Ia pared abdominal. Punción de lesiones da salida a líquido lechoso. Se toman biopsias y se reseca lesión infartada. Paciente evoluciona favorablemente, dado de alta al tercer día. La histología reveló un Linfangioma Quístico Multilocular Mesentérico. Pese a ser una patología infrecuente, debe ser considerada dentro de los diagnósticos diferenciales de abdomen agudo, siendo extremadamente rara su presentación en Ia tercera edad.

Linfangioma mesentérico / Mesentery lymphangioma

El linfangioma representa el 6 por ciento de los tumores benignos, y su localización más frecuente es la región cervical, seguida de la axila en el 95 por ciento de los casos. Solo un 5 por ciento tiene localización en otro sitio. Dentro de estos, los abdominales representan entre el 2 y el 5 por ciento , y los más frecuentes son los del mesenterio. Se presenta el caso de una paciente de 45 años con el diagnóstico de metrorragia disfuncional, que a pesar del tratamiento hormonal, aumentó en intensidad. Se le practicó un legrado diagnóstico, cuyo resultado fue hiperplasia adenomatosa con ligera atipia, y se le indicó una histerectomía abdominal, que se realizó sin dificultad. En el transcurso de esta intervención se llevó a cabo la extirpación de un tumor del mesenterio del yeyuno, cuyo diagnóstico histológico definitivo fue linfangioma mesentérico(AU)

The lymphangioma accounts for the 6 percent of the benign tumors and its more frequent location is the cervical region, followed by the axilla in the 95 percent of cases. Only the 5 percent is located in other site including the abdominal ones accounting for the 2 and the 5 percent and the more frequent are those of mesentery. This is the case of a patient aged 45 diagnosed with dysfunctional metrorrhagia that despite the hormonal treatment increased its intensity. A diagnostic curettage was made whose result was an adenomatous hyperplasia with a slight atypia prescribing an abdominal hysterectomy carried out without problem. During this intervention a yeyunal mesenteric tumor was removed with a definitive histological diagnosis of mesenteric lymphangioma(AU)