Adenomiosis apendicular: reporte de dos casos / Appendicular adenomiosis: report of two cases

Resumen Los adenomiomas son tumoraciones benignas constituidas por un agregado nodular de músculo liso, glándulas endometriales y estroma endometrial. La presencia de adenomiomas fuera del útero es un hallazgo infrecuente. Presentamos dos casos de adenomiomas extrapélvicos localizados en el apéndice. El estudio histológico resultó esencial para el diagnóstico.

Abstract Adenomyomas are a benign tumor compound of smooth muscle nodular aggregate, endometrial glands and endometrial stroma. Adenomyomas presenting outside uterus are a rare finding. Here we report two extrapelvic adenomyomas of the appendix. Histological examination was essential for diagnosis.

Primary malignant neoplasms of the cecal appendix: unusual findings in acute appendicitis

Introduction: To evaluate the prevalence of appendix neoplasia correlating with patient profile, histological types and frequency. Methods: Data collection was performed in the Pathology Department of a General Hospital, with the objective of identifying patients diagnosed with malignant cecal appendix tumors by histopathologic study of specimens from acute appendicitis. Results: The prevalence of malignant primary epithelial neoplasia of the appendix was 1%. Fifty percent of the cases were neuroendocrine tumors, 35% were mucinous, and 15% were adenocarcinomas. The mean age at diagnosis was 41.3 (SD, 20.4) years (range 16-81), with a women/men ratio of 3:1. Discussion: Appendiceal neoplasms are rare and should be suspected manly in women over 40 years of age with suggestive symptoms of acute appendicitis. The size, location, extent, margins and presence of mucin are essential findings for the treatment of these patients. (AU)

Fibromatosis tipo desmoide de apéndice cecal: reporte de caso inusual y revisión de la literatura / Appendicular desmoid tumor: unusual case and review of the literature

RESUMEN Antecedente : La fibromatosis tipo desmoide es un proceso neoplásico benigno no encapsulado localmente invasivo y agresivo, que se origina de la proliferación de fibroblastos y miofibroblastos aparentemente normales. La localización más frecuente de la fibromatosis es extra-abdominal (60%), pared abdominal (25%) e intra-abdominal (8-15%), en raras ocasiones puede originarse en las vísceras (0,73%), como el páncreas, unión gastroesofágica, diafragma y apéndice. La incidencia anual de tumor desmoide se estima de 2 a 5 casos por millón. En el presente artículo, reportamos un caso de presentación inusual, originado en el apéndice cecal. Caso : Paciente de sexo femenino de 41 años con dolor pélvico agudo, que ingresa a sala de operaciones con diagnóstico clínico y ecográfico de probable tumor de ovario a pedículo torcido. En el intraoperatorio se evidenció una tumoración sólida de 15 cm de diámetro que dependía del apéndice cecal, correspondiendo el estudio anatomopatológico a tumor desmoide de apéndice cecal. Conclusiones : El tumor desmoide puede originarse en diversas localizaciones extra e intra-abdominales, siendo esta última la más rara y agresiva. El diagnóstico preoperatorio exacto es muy difícil y casi siempre los pacientes ingresan al quirófano con sospecha diagnostica de tumoración intraabdominal de etiología desconocida. Los factores de riesgos asociados a su aparición aún no se encuentran caracterizados, siendo la resección quirúrgica completa del tumor -con márgenes quirúrgicos libres (R0)- el tratamiento de elección; sin embargo, el riesgo de recurrencia es alto incluso con la remoción óptima del tumor.

ABSTRACT Background : Desmoid type fibromatosis is a benign neoplastic process non-encapsulated locally invasive and aggressive, which arises from a proliferation of bland-looking fibroblasts and myofibroblasts. The most frequent location of fibromatosis is extra- abdominal (60%), abdominal wall (25%) and intra-abdominal (8-15%), rarely can originate in the viscera (0.73%), such as the pancreas, gastroesophageal junction, diaphragm and appendix. The annual incidence of desmoid tumor is estimated at 2 to 5 cases per million. In this article, we report a case of unusual presentation, originated in the cecal appendix. Case : A 41-year-old female patient with acute pelvic pain, admitted to surgical ward with a clinical and ultrasound diagnosis of probably ovarian pedicle tumor. An explorative laparotomy revealed a solid mass of 15 cm in diameter arising from the cecal appendix, with the anatomopathological study corresponding to a desmoid tumor of the cecal appendix. Conclusions : The desmoid tumor can arise from several extra and intra-abdominal locations, the latter being the rarest and most aggressive. Accurate preoperative diagnosis is very difficult and almost always patients enter to surgery with suspected diagnosis of intraabdominal tumor of unknown etiology. The risk factors associated to its appearance are not yet characterized. The complete surgical resection of the tumor -with free surgical margins (R0)- is the treatment of choice; however, the risk of recurrence is high even with optimal tumor removal.

Cistoadenocarcinoma mucinoso de apéndice cecal / Mucinous cystoadenocarcinoma of the appendix

Objetivo Exposición de 3 casos tratados quirúrgicamente en nuestra clínica. Casos clínicos Caso 1: paciente mujer de 78 años con diagnóstico de quiste complejo de ovario derecho, se realizó la cirugía y se evidencia tumoración de apéndice cecal. Caso 2: paciente varón de 38 años con dolor abdominal crónico. Presenta marcador tumoral antígeno carcinoembrionario elevado, laparoscopia exploratoria evidencia líquido mucinoso peritoneal y tumoración apendicular. Caso 3: paciente mujer de 42 años con dolor crónico en fosa iliaca derecha con marcadores tumorales normales. Laparoscopia exploratoria evidencia tumoración dependiente del apéndice cecal con localización retrocecal ascendente. Discusión Se debe considerar a los mucoceles apendiculares dentro del diagnóstico diferencial de dolor en cuadrante inferior derecho del abdomen. Es preferible realizar una hemicolectomia radical para lesiones grandes y/o perforadas por el riesgo de existir cistoadenocarcinoma. El abordaje laparoscópico es una buena alternativa de manejo.

Objective We present three surgically treated cases in our hospital Case Reports Case 1: A 78 years old woman with a diagnosis of complex ovarian cyst. During surgery, an appendicular tumor was found. Case 2: A 38 years old male with chronic abdominal pain with high levels of carcinoembryonic antigen. During exploratory laparoscopy an appendicular tumor and mucinous peritoneal fluid were found. Case 3: A 42 years old woman with chronic pain in the right lower abdomen, negative tumor markers. During exploratory laparoscopy a retrocecal appendicular tumor was found. Discussion Appendiceal mucoceles should be considered in the differential diagnosis of right lower abdominal pain. If the lesions are big or perforated, a radical hemicolectomy should be performed due to the risk of a cystadenocarcinoma. Laparoscopic approach is a good alternative for management.

Appendiceal Immunoglobulin G4-Related Disease Mimicking Appendiceal Tumor or Appendicitis: A Case Report

Immunoglobulin G4 (IgG4)-related disease is an autoimmune disease that forms tumorous lesions. Several cases involving various organs are reported, however, IgG4-related disease involving appendix has not been reported yet. In this report, we presented a case of IgG4-related disease of appendix, which raised a suspicion of appendiceal tumor or usual appendicitis and, therefore, led to unnecessary surgical resection. IgG4-related disease should be considered in the differential diagnosis for a mass-like swelling of the appendix, in order to avoid unnecessary surgery.

Appendiceal Neuroendocrine Tumor with Lymph Node Metastasis in a Teenager / 대한소화기학회지

Neuroendocrine tumor (NET) is a cancer-like tumor that occurs mostly in the gastrointestinal system. Within the gastrointestinal tract, NET most commonly occurs in the rectum whereas appendix is very rarely involved. In most cases of appendiceal NET, it is found at a relatively early stage compared to other NETs because appendiceal NET frequently presents with acute appendicitis because appendiceal NET frequently presents with acute appendicitis even when the size is smaller than 1 cm. Therefore, it is very rare for lymph node metastasis to occur in a young adult. Herein, we report a rare case of grade 1 appendiceal NET with lymph node metastasis which developed in a teenage male.

Small cell carcinoma metastatic to the appendix, with acute suppurative appendicitis

Malignancy of the appendix is uncommon. A small subset of these lesions is actually metastatic cancers. Metastasis to the appendix is very rare and infrequently symptomatic. Metastatic lesions can cause obstruction,

Appendiceal pseudomyxoma peritonei in a pregnant woman

One in 1000 cancers occur during pregnancy. Epithelial tumors of the appendix occur in approximately 1% of all colorectal tumors, and the occurrence of this neoplasm during pregnancy is extremely rare. We report an unusual case of pseudomyxoma peritonei [PMP] in a 41-year-old full-term pregnant women. Cesarean section was carried out, and the abdomen was found to contain an appendiceal mucinous tumor with large volume PMP. This case illustrates the incidental nature of the disease and its occurrence in a pregnant women with no clinical features during the antenatal period. A review of recent literature is also documented. Pseudomyxoma peritonei is commonly discovered during surgery for other conditions and a high index of suspicion is required to make a diagnosis. Diagnostic modalities such as ultrasound, CT, and MRI can provide some evidence for PMP, but the definitive diagnosis is only made laparoscopically or by exploratory laparotomy

primary diffuse large B-cell lymphoma of appendix

Primary appendiceal lymphomas are exceedingly rare neoplasms mostly ignored until histological examination of appendices. We report a case of primary appendiceal lymphomas in a 22-year-old man. Appendectomy was performed under diagnosis of acute appendicitis. Macroscopically a fleshy intramural mass in middle third portion of appendix was observed. Microscopic examination revealed a diffuse large B-cell lymphoma inducing acute appendicitis. Further medical workups of the patient was unremarkable and the disease was restricted to appendix. After initial simple appendectomy, the patient underwent adjuvant chemotherapy and was healthy after two years. This case emphasized great value of detailed histological examination of all appendices

Carcinoide apendicular en paciente pediátrica: reporte de caso / Appendicular carcinoid in a pediatric patient: case report

Los carcinoides son tumores neuroendocrinos de baja incidencia en la población general. Aparecen con mayor frecuencia en apéndice cecal, seguido por intestino delgado, recto y bronquios. Los tumores carcinoides apendiculares son habitualmente asintomáticos, por lo que la mayoría de las veces sólo se descubren en las biopsias de la pieza operatoria de las apendicetomías. Su tratamiento es quirúrgico y tienen un buen pronóstico. Se presenta el caso de una paciente de 14 años de edad con cuadro clínico de apendicitis aguda, a quien se le realizó apendicectomía. El resultado de estudio anatomopatológico confirmó un tumor de células endocrinas (argentafinoma, tumor carcinoide) en el tercio distal del órgano, que infiltraba hasta la serosa y apendicitis aguda supurada. Por lo inusual de estos tumores en edades tempranas y por lo que puede representar para el niño una conducta no consecuente, se presenta este caso, enfatizando que es extremadamente importante el seguimiento de las conclusiones del examen histológico de pacientes sometidos a apendicectomía...

Tumor carcinoide apendicular / Carcinoid of the appendix. A case report

Tumors of the appendix constitute an heterogeneous group of malignancies with a variable evolution and prognosis...The aim of this reporte was to determine the prevalence of carcinoid tumors of the cecal appendix and its relationship with age, sex, clincal features and introspctive suspicion.

Incidencia de tumores apendiculares malignos en apendicectomías / Incidence of malignant tumors in appendectomy

Antecedentes: Los tumores apendiculares son una patología infrecuente. Representan el 0.5% de los tumores digestivos. Su diagnostico prequirúrgico es excepcional y constituye, generalmente un hallazgo incidental en el estudio anatomopatológico de la pieza quirúrgica. Objetivo: Evaluar la incidencia y tipo histológico de los tumores apendiculares, en apendicetomías realizadas en nuestra institución. Diseño: Retrospectivo, descriptivo. Material y Métodos: Se evaluaron retrospectivamente historias clínicas de pacientes apendicectomizados en un periodo de 10 años, año 1997 al 2007. Se excluyeron aquellas en las cuales no se había realizado estudio anatomopatológico. Se evaluaron 380 historias clínicas, estudiando aquellas con diagnostico de tumor apendicular maligno, registrando edad, sexo, clínica, tipo de tumor y tratamiento. Resultados: Se diagnosticaron 5 tumores malignos (1,3 %), 4 tumores carcinoides (1%), y 1 adenocarcinoma (0.3 %), 3 de sexo masculino (60 %) y 2 de sexo femenino (40%), con un promedio de edad de 41,6 años. La indicación quirúrgica fue por apendicitis en el 60 %. La sospecha intra-operatoria se planteo en 1 de los pacientes (20%). Conclusión: Los tumores apendiculares son neoplasias muy poco frecuentes, diagnosticadas generalmente en el estudio anatomopatológico, de allí la impor tancia del mismo. Estos tumores deben estar presentes en los diagnósticos diferenciales de la patología apendicular, su conocimiento permitirá realizar un tratamiento y manejo opor tuno del mismo.

Cistoadenoma de apêndice: relato de caso e revisão da literatura sobre tumores mucinosos do apêndice vermiforme / Cyst adenoma of appendix: case report and literature review about mucinous vermiform appendix neoplasms

INTRODUÇÃO: Mucocele do apêndice (MA) é uma lesão rara, caracterizada pelo acúmulo de secreção mucoíde em sua luz. Diagnóstico precoce é fundamental para evitar o extravasamento de secreção mucoíde na cavidade peritoneal, causando pseudomixoma peritoneal. RELATO DE CASO: Paciente sexo masculino, 62 anos com história de dor abdominal em fossa ilíaca direita há cerca de 6 meses. Realizou uma ultra-sonografia do abdome que evidenciou no flanco direito pequena estrutura cística compatível com MA. Colonoscopia, exames laboratoriais e dosagem do CEA encontravam-se sem alterações. O paciente foi submetido à laparotomia exploradora com ressecção do apêndice e do seu meso em monobloco. O exame histológico mostrou tratar-se de cistoadenoma do apêndice vermiforme. O paciente evoluiu sem intercorrências. DISCUSSÃO: A MA é um achado raro. As principais causas patológicas incluem cisto de retenção, mucocele secundária a epitélio hiperplásico, cistoadenomas e cistoadenocarcinomas. A doença é geralmente assintomática e o diagnóstico pré-operatório é raro. O tratamento para mucocele do apêndice é cirúrgico e a preocupação principal do cirurgião é evitar o extravasamento do conteúdo da mucocele na cavidade abdominal. CONCLUSÃO: A MA é uma entidade incomum e com alto potencial de complicação, usualmente curável com tratamento cirúrgico adequado.

INTRODUCTION: Appendiceal mucocele (AM) is a rare lesion that is characterized by an appendiceal lumen distended with mucus. Early diagnosis and prompt surgical intervention are mandatory to prevent spillage of the mucinous content into the peritoneal cavity, causing pseudomyxoma peritonei. CASE REPORT: A 62-year-old male was presented with a 6-months history of recurrent right lower quadrant abdominal pain. Ultrasonography showed a lesion in the right lumbar region compatible with AM. Colonoscopy, routine laboratory tests and CEA were normal. The patient underwent an appendectomy and complete excision of the cystic lesion. Pathologic examination revealed a mucinous cyst adenoma of appendix. The patient was symptom free after treatment. DISCUSSION: AM is a rare condtion. Mucoceles are histologically subdivided into retention mucocele, mucocele secondary a hiperpalsic epithelium, mucinous cystadenoma and mucinous cystadenocarcinoma. This disease is often asymptomatic and pre-operative diagnosis is rare. The treatment is essentially surgical and the surgeon need to avoid spillage of the mucinous content into the peritoneal cavity. CONCLUSION: AM is an uncomum entity and with high pontential of complication, usually curable with adequate surgical aproach.

Peritoneal pseudomyxoma associated with synchronic malignant mucinous neoplasias of the cecum, appendix and rectum: case report and review of the literature

Peritoneal pseudomyxoma is a pathological condition that compromises the peritoneum, characterized by the production of large quantities of mucinous liquid, which progressively fills the peritoneal cavity, originating in general from mucinous appendicular or ovarian tumors. We report a peritonial pseudomyxoma associated with mucinous adenocarcinoma of the appendix synchronic with adenocarcinoma of the rectum in 44 years old patient, where the initial diagnosis was rectal adenocarcinoma. Tumour of the appendix and peritonial pseudomyxoma were incidental and found intraoperativelly. We focus the histological patterns of the lesions, diagnosis and the treatment, with revision of the literature.

O pseudomixoma peritoneal é uma condição patológica que acomete o peritoneo, caracterizada pela produção de grandes quantidades de líquido mucinoso, que progressivamente preenche a cavidade peritoneal, tendo em geral como origem tumores mucinosos apendiculares ou de ovário. Relatamos a ocorrência de um pseudomixoma peritoneal associado a adenocarcinoma mucinoso do apêndice sincrônico e adenocarcinoma do reto em paciente de 44 anos, cujo diagnóstico inicial foi de adenocarcinoma do reto. A neoplasia do apêndice e o pseudomixoma peritonial foram achados incidentais, intra-operatórios. Enfocamos as principais características anatomo-patológicas das lesões, o diagnóstico e tratamento, através de ampla revisão da literatura.

Tumores del apéndice cecal: análisis anatomoclínico y evaluación de la sobrevida alejada / Appendiceal tumors: retrospective review of 67 patients

Introducción: Los tumores del apéndice cecal corresponden al 0,5 por ciento de las neoplasias gastrointestinales y su pronóstico depende del tipo de tumor. Objetivos: Analizar las características de los tumores apendiculares y los factores que influyen en su sobrevida. Material y Métodos: Estudio retrospectivo de los pacientes con un tumor apendicular operados entre 1981 y 2006 en el Hospital de la P. Universidad Católica. Se evaluaron las características clínicas, anatomopatológicas (AP) y la sobrevida alejada. Se utilizó la prueba de Fisher, el test de Wilcoxon y curvas de Kaplan-Meier. Resultados: Se operaron 67 enfermos, 39 mujeres y 28 hombres con una edad de 45,3 + 19,6 años. En 59 enfermos la operación se realizó por sospecha de apendicitis aguda, la que se confirmó en el estudio AP en 37 de ellos. En 36 pacientes se demostró un carcinoma neuroendocrino, en 18 un tumor benigno, en 7 un adenocarcinoma, en 5 un adenocarcinoide y en uno un carcinoma neuroendocrino y un cistoadenoma mucinoso. Se realizó una hemicolectomía en un segundo tiempo operatorio a 9 enfermos: 4 con un carcinoide, 3 con un adenocarcinoma, 1 con un adenocarcinoide y uno con un pseudomixoma peritoneal. La sobrevida a 5 años fue de 100 por ciento en los enfermos con un tumor benigno, 97 por ciento en los con un carcinoma neuroendocrino, 75 por ciento en los con un adenocarcinoide y 62 por ciento en los con un adenocarcinoma (p<0,05). Conclusiones: En la mayoría de los enfermos el diagnóstico de tumor se obtuvo como un hallazgo del estudio anatomopatológico en apendicectomías indicadas por sospecha de apendicitis aguda. En la sobrevida influyó fundamentalmente el tipo histológico del tumor.

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.

Adenocarcinoma de apêndice: relato de dois casos e revisão da literatura / Adenocarcinoma of the appendix: two cases report and literature review

O adenocarcinoma apendicular é enfermidade rara, geralmente identificada com um quadro clínico de apendicite aguda. O diagnóstico pré-operatório é difícil e incomum. Objetivo - apresentar dois casos de adenocarcinoma apendicular, singulares por situarem-se em faixa etária jovem, associado à revisão de literatura / The appendicular adenocarcinoma is a rare disease, generally identifiedd as an acute appendicitis clinical presentation. Pre-operative diagnosis is extremely difficult and uncommon. Aim - to present two cases of appendicular adenocarcinoma in young people, and literature review...

Rectal and appendiceal inflammatory myofibroblastic tumors

Inflammatory myofibroblastic tumors are neoplasms characterized by spindle cell proliferation and a fiboinflammatory vascular stroma. Herein, we presented the successful treatment of a rectal inflammatory myofibroblastic tumor in an 11-year-old boy who presented with diarrhea and abdominal pain of 11/2 months duration and an appendiceal inflammatory myofibroblastic tumor in a 29-year-old man presented with recurrent abdominal pain of two months duration with associated tenderness and rebound tenderness in the right lower abdomen. Histologically, our cases had inflammatory myofibroblastic tumors very similar to that of other sites; the spindle cells were positive for vimentin and muscle-specific actin