Initial assessment of “Gissell's stain”: A novel histopathological method for the identification of Helicobacter pylori / Valoración inicial de la tinción de Gissell, un nuevo método histopatológico en la identificación del Helicobacter pylori

Introduction and Objectives: The histopathological identification of Helicobacter pylori using the routine method (haematoxylin–eosin) is not only very difficult but also has low sensitivity. Giemsa staining is often used in addition, but different protocols do not produce homogeneous results. Furthermore, the Gold Standard recommended by the European Helicobacter Pylori Study Group has been applied in very few studies, thus resulting in uncertain outcomes. Therefore, a new staining method is required to overcome these limitations. The aim of this study was to evaluate the diagnostic capacity and inter-observer agreement of “Gissell's stain”. Material and Methods: A cross-sectional study evaluated 99 gastric paraffin blocks from a private laboratory. Three sections were prepared from each block, and haematoxylin–eosin (HE), Giemsa and “Gissell's stain” methods were applied. The kappa statistics, sensitivity, specificity, and predictive values were calculated. Results: “Gissell's stain” obtained the highest inter-observer agreement (kappa=0.87) compared to the other two methods (HE, kappa=0.51; Giemsa, kappa=0.83). It also obtained the best sensitivity and negative predictive value (97.1% and 98.3%, respectively) compared with the other two methods (HE: 68.6% and 85.1%, respectively; Giemsa: 88.6% and 93.9%, respectively). Conclusions: Given its unique characteristics (fast, cheap, accessible, and easy to use), in addition to its statistical reliability, “Gissell's stain” has great potential for routine use in the identification of H. pylori.(AU)

Introducción y objetivos: La identificación histopatológica del Helicobacter pylori, utilizando hematoxilina-eosina (HE) como método de rutina es frecuentemente dificultosa y con una baja sensibilidad. El método de Giemsa es usado a veces de modo adicional, pero diferentes protocolos no producen resultados homogéneos. Además, el método recomendado por el grupo europeo de estudio de Helicobacter pylori ha sido empleado en pocos estudios, resultando en datos discrepantes. Por tanto, un método alternativo eficaz podría superar estas limitaciones. El presente estudio valora la agudeza diagnostica y el acuerdo interobservador en la aplicación del método de Gissell. Material y métodos: Un estudio interobservador de 99 biopsias gástricas provenientes de la practica privada. De cada caso se prepararon tres muestras teñidas correspondientemente con: HE, Giemsa y Gissell. Se procedió mediante estadística kappa a valorar la sensibilidad, especificidad y valores predictivos. Resultados: La técnica de Gissell obtuvo el mayor acuerdo interobservador con un kappa con 0,87, comparado con la HE (kappa = 0,51) y Giemsa (kappa = 0,83). Además, obtuvo la mayor sensibilidad y valor predictivo negativo comparado con los otros dos métodos. Conclusiones: Dadas sus características prácticas, tales como rapidez, economía, accesibilidad y facilidad de uso, además de su fiabilidad estadística, el método de Gissell posee un gran potencial para la identificación rutinaria del Helicobacter pylori.(AU)

Initial assessment of "Gissell's stain": A novel histopathological method for the identification of Helicobacter pylori.

INTRODUCTION AND OBJECTIVES: The histopathological identification of Helicobacter pylori using the routine method (haematoxylin-eosin) is not only very difficult but also has low sensitivity. Giemsa staining is often used in addition, but different protocols do not produce homogeneous results. Furthermore, the Gold Standard recommended by the European Helicobacter Pylori Study Group has been applied in very few studies, thus resulting in uncertain outcomes. Therefore, a new staining method is required to overcome these limitations. The aim of this study was to evaluate the diagnostic capacity and inter-observer agreement of "Gissell's stain". MATERIAL AND METHODS: A cross-sectional study evaluated 99 gastric paraffin blocks from a private laboratory. Three sections were prepared from each block, and haematoxylin-eosin (HE), Giemsa and "Gissell's stain" methods were applied. The kappa statistics, sensitivity, specificity, and predictive values were calculated. RESULTS: "Gissell's stain" obtained the highest inter-observer agreement (kappa=0.87) compared to the other two methods (HE, kappa=0.51; Giemsa, kappa=0.83). It also obtained the best sensitivity and negative predictive value (97.1% and 98.3%, respectively) compared with the other two methods (HE: 68.6% and 85.1%, respectively; Giemsa: 88.6% and 93.9%, respectively). CONCLUSIONS: Given its unique characteristics (fast, cheap, accessible, and easy to use), in addition to its statistical reliability, "Gissell's stain" has great potential for routine use in the identification of H. pylori.

Enfermedad de Paget extramamario primario de vulva concurrente con Lesión intraepitelial escamosa vulvar: reporte de caso / Concurrence of primary extramammary Paget's disease of the vulva and vulval intra-epithelial neoplasia: A case report

La enfermedad de Paget extramamario y la neoplasia intraepitelial vulvar son lesiones comunes de la vulva. No obstante, su ocurrencia sincrónica es una manifestación sumamente rara en la misma localización. Presentamos el caso de una paciente de 77años, quien hace 16meses aqueja de prurito y sarpullido en la región vulvar con sangrado escaso que empezó a incrementarse en frecuencia y en cantidad. Es sometida a hemivulvectomía derecha y vulvectomía simple izquierda. El informe de patología reportó la presencia concurrente de enfermedad de Paget vulvar y neoplasia intraepitelial vulvar de alto grado.(AU)

Extramammary Paget's disease and intraepithelial vulvar neoplasia are common lesions in the vulva. However, their simultaneous occurrence is extremely rare. We present the case of a 77year-old woman who presented with a 16month history of pruritus and a rash in the vulvar region with gradually increasing bleeding. She underwent a right hemivulvectomy and a left simple vulvectomy. The histopathology revealed a coexistence of both Paget's disease and high grade intraepithelial vulvar neoplasia.(AU)

[Pancreatic lipomatous hamartoma: A case report and literature review]. / Hamartoma lipomatoso pancreático: Reporte de caso y revisión de la literatura.

Pancreatic hamartomas (PH) are extremely unusual non-neoplastic tumor-like lesions and accounts for <1% of all hamartomas. Moreover, there is a distinct variant of PH denominated Pancreatic lipomatous hamartoma (PLH), that is even rarer, with only 5 cases, including the present case, reported in the literature. PLH lacks well-defined features and clinically can be mistaken with other lipomatous lesions of the pancreas, including lipoma, pancreatic lipomatosis, PEComa, liposarcoma, and malignant tumors with lipomatous components. Here, we describe a case of PLH in a 70-year-old male with abdominal pain and a lesion, which was preoperatively diagnosed as a pancreatic no functional low-grade neuroendocrine tumor, and subsequent underwent a laparoscopic enucleation of the tumor. The postoperative pathology and immunohistochemical analyses confirmed the diagnosis of PLH.

[Concurrence of primary extramammary Paget's disease of the vulva and vulval intra-epithelial neoplasia: A case report]. / Enfermedad de Paget extramamario primario de vulva concurrente con Lesión intraepitelial escamosa vulvar: reporte de caso.

Extramammary Paget's disease and intraepithelial vulvar neoplasia are common lesions in the vulva. However, their simultaneous occurrence is extremely rare. We present the case of a 77year-old woman who presented with a 16month history of pruritus and a rash in the vulvar region with gradually increasing bleeding. She underwent a right hemivulvectomy and a left simple vulvectomy. The histopathology revealed a coexistence of both Paget's disease and high grade intraepithelial vulvar neoplasia.

Hamartoma lipomatoso pancreático: Reporte de caso y revisión de la literatura

Los hamartomas pancreáticos (HP) son lesiones no neoplásicas extremadamente inusuales y representan < 1% de todos los hamartomas. Además, existe una variante distintiva denominada Hamartoma Lipomatoso Pancreático (HLP), que es aún más raro, con solo 5 casos, incluyendo el presente reporte, descritos en la literatura. HLP carecen de características específicas y clínicamente puede ser confundido con otras lesiones lipomatosas pancreáticas, como lipoma, lipomatosis pancreática, PEComa, liposarcoma y tumores malignos con componentes lipomatosos. El presente reporte describe un caso de HLP en un paciente varón de 70 años, que aquejaba de dolor y masa abdominal, preoperatoriamente diagnosticado como tumor neuroendocrino de bajo grado no funcionante pancreático. Subsecuentemente, fue sometido a enucleación laparoscópica del tumor. El reporte patológico postoperatorio y los estudios de inmunohistoquímica confirmaron el diagnóstico de HLP.

Pancreatic hamartomas (PH) are extremely unusual non-neoplastic tumor-like lesions and accounts for <1% of all hamartomas. Moreover, there is a distinct variant of PH denominated Pancreatic lipomatous hamartoma (PLH), that is even rarer, with only 5 cases, including the present case, reported in the literature. PLH lacks well-defined features and clinically can be mistaken with other lipomatous lesions of the pancreas, including lipoma, pancreatic lipomatosis, PEComa, liposarcoma, and malignant tumors with lipomatous components. Here, we describe a case of PLH in a 70-year-old male with abdominal pain and a lesion, which was preoperatively diagnosed as a pancreatic no functional low-grade neuroendocrine tumor, and subsequent underwent a laparoscopic enucleation of the tumor. The postoperative pathology and immunohistochemical analyses confirmed the diagnosis of PLH.

Gonadoblastoma in a patient with 45,X/46XY mosaicism.

45,X/46,XY mosaicism is a sex development disorder with an estimated incidence of less than 1 in 15,000 live births. Various studies have shown there is an increased risk of germ cell tumours forming in Mosaic Turner syndrome. This includes gonadoblastoma, a clinically benign mixed germ-stromal cell tumour. However, this can later develop into one or several malignant germ cell neoplasms, for which early prophylactic gonadectomy is often recommended in patients with 45,X/46,XY mosaicism. The study presents the case of an 11-year-old patient diagnosed with a Mosaic Turner syndrome karyotype, who underwent prophylactic bilateral gonadectomy.

Carcinoma coloide pancreático y tumor del estroma gastrointestinal del estómago sincrónicos: reporte de caso y revisión de la literatura / Colloid Carcinoma of the pancreas and synchronous gastrointestinal stromal tumour of stomach. A case report and review of the literature

El carcinoma coloide (CC) es una variante histológica inusual del adenocarcinoma ductal del páncreas, el cual se caracteriza por la presencia de grandes lagos de mucina extracelular que contiene células neoplásicas y está asociado a una mejor sobrevida a los cinco años, comparada con los adenocarcinomas ductales (DAC) tubulares o no tipo especial (NOS).Presentamos el caso de una paciente mujer de 74 años con una lesión multiquística de tabiques finos en cola de páncreas con diagnóstico clínico radiológico sugestivo de cistoadenoma seroso vs. neoplasia mucinosa. En el acto operatorio se evidenció una lesión de 10 x 6 cm, que infiltraba hilio esplénico y mesocolon transverso. Por otro lado, se identificaron dos nódulos en la pared del fondo gástrico que también fue resecado en conjunto. El estudio anatomopatológico concluyó que la tumoración del páncreas correspondía a un CC asociado incidentalmente a un tumor del estroma gastrointestinal (GIST) de fondo gástrico.(AU)

Colloid carcinoma (CC) is a rare histological type of adenocarcinoma of the pancreatic duct and is characterized by the presence of large lakes of extracellular mucin containing neoplastic cells. Its 5 year prognosis is more favourable than that of ductal, tubular or not otherwise specified (NOS) adenocarcinomas.We present the case of a 74-year-old woman with a thin walled, multicystic lesion in the tail of the pancreas, radiologically suggestive of a serous cystadenoma as opposed to a mucinous neoplasm. Surgery revealed a 10 x 6 cm lesion invading the splenic hilum and transverse mesocolon. Two nodes on the wall of the gastric fundus were also removed. Histopathology showed the pancreatic tumour to be a colloid carcinoma with a synchronous gastrointestinal stromal tumour of the gastric fundus.(AU)

Tumor neuroectodérmico maligno del tracto gastrointestinal: Reporte de 2 casos y revisión de la literatura / Malignant gastrointestinal neuroectodermal tumor: A report of 2 cases and a review of the literature

El tumor neuroectodérmico maligno del tracto gastrointestinal (GNET) es una neoplasia maligna sumamente rara, descrita por primera vez por Zambrano et al. en 2003 como tumor similar al sarcoma de células claras del tracto gastrointestinal, pues a diferencia del sarcoma de células claras posee células gigantes osteoclásticas y positividad difusa e intensa para S-100 con ausencia inmunohistoquímica y ultraestructural de diferenciación melanocítica. La presente publicación busca aportar los 2 primeros casos de GNET reportados en nuestro país, Perú, y América Latina. Reportamos 2 casos de GNET, en paciente mujer y en varón, ambos en la sexta década de la vida, cuyos casos llegaron a nuestra institución para reevaluación diagnóstica. Uno de los casos continuó tratamiento en nuestro instituto con evolución desfavorable. El conocimiento de su existencia y criterios diagnósticos por los patólogos es necesario para evitar confundirla y maldiagnosticarla con alguna otra neoplasia gastrointestinal no epitelial.(AU)

Malignant gastrointestinal neuroectodermal tumour (GNET) is an extremely rare neoplasm first described by Zambrano in 2003 as clear cell sarcoma like tumor of the gastrointestinal tract. In contrast to clear cell sarcoma, it has giant osteoclast cells and shows diffuse and intense positivity for S-100 with no immunohistochemical or ultrastructural melanocyte differentiation. We present the first cases of GNET reported in South America, occurring in Peru. Two cases of GNET, one in a female and one in a male, both between 60 and 70 years of age, were referred to our hospital for reevaluation. One underwent further treatment in our centre, but with an unfavourable evolution. Pathologists should be aware of the diagnostic criteria for GNET in order to avoid misdiagnosis due to confusion with other non-epithelial gastrointestinal neoplasms.(AU)

Carcinoma hepatocelular ectópico: reporte de caso y revisión de la literatura / Ectopic hepatocellular carcinoma. A case report

El hígado ectópico (HE) es una entidad infrecuente, debida a la migración aberrante de las células hepáticas durante la embriogénesis. La incidencia se estima entre el 0,24 y el 0,47%. Puede presentarse en numerosas localizaciones, siendo la vesícula la localización más común. Existen alrededor de 100 casos publicados de HE, de los cuales 28 han desarrollado carcinoma hepatocelular (CHC); mientras que el hígado ortotópico no presenta ninguna alteración; todos provienen de la literatura asiática y caucásica. Reportamos el caso de un paciente de 42 años con lesión sólida de 17×12×12cm, aparentemente dependiente de la pared posterior del cuerpo gástrico, y con desplazamiento de numerosas vísceras, sugiriendo como posibilidad diagnóstica GIST. Mientras que, otras estructuras como el hígado y las vías biliares fueron topográficamente normales. El estudio anatomopatológico concluyó que la tumoración descrita correspondía a un CHC originado en un HE.(AU)

Ectopic liver (EL) is a rare developmental anomaly caused by the migration of hepatic cells to other locations during embryogenesis. Its incidence is thought to be between 0.24 and 0.47% and can occur in various sites, although the gall bladder is the most frequent. Approximately 100 cases of EL have been published, of which 28 developed hepatocellular carcinoma, whilst the orthotopic liver was unaffected. All cases are reported in Asian and Caucasian patients.We report the case of a 42-year-old patient with a solid lesion measuring 17×12×12cm apparently arising from the posterior wall of the stomach, displacing various organs and suggestive of a gastrointestinal stromal tumour (GIST). The liver, biliary tract and other structures were unremarkable. Histopathology revealed a diagnosis of hepatocellular carcinoma in ectopic liver tissue.(AU)

[Colloid Carcinoma of the pancreas and synchronous gastrointestinal stromal tumour of stomach. A case report and review of the literature]. / Carcinoma coloide pancreático y tumor del estroma gastrointestinal del estómago sincrónicos: reporte de caso y revisión de la literatura.

Colloid carcinoma (CC) is a rare histological type of adenocarcinoma of the pancreatic duct and is characterized by the presence of large lakes of extracellular mucin containing neoplastic cells. Its 5 year prognosis is more favourable than that of ductal, tubular or not otherwise specified (NOS) adenocarcinomas. We present the case of a 74-year-old woman with a thin walled, multicystic lesion in the tail of the pancreas, radiologically suggestive of a serous cystadenoma as opposed to a mucinous neoplasm. Surgery revealed a 10 x 6 cm lesion invading the splenic hilum and transverse mesocolon. Two nodes on the wall of the gastric fundus were also removed. Histopathology showed the pancreatic tumour to be a colloid carcinoma with a synchronous gastrointestinal stromal tumour of the gastric fundus.

[Malignant gastrointestinal neuroectodermal tumor: A report of 2 cases and a review of the literature]. / Tumor neuroectodérmico maligno del tracto gastrointestinal: Reporte de 2 casos y revisión de la literatura.

Malignant gastrointestinal neuroectodermal tumour (GNET) is an extremely rare neoplasm first described by Zambrano in 2003 as clear cell sarcoma like tumor of the gastrointestinal tract. In contrast to clear cell sarcoma, it has giant osteoclast cells and shows diffuse and intense positivity for S-100 with no immunohistochemical or ultrastructural melanocyte differentiation. We present the first cases of GNET reported in South America, occurring in Peru. Two cases of GNET, one in a female and one in a male, both between 60 and 70 years of age, were referred to our hospital for reevaluation. One underwent further treatment in our centre, but with an unfavourable evolution. Pathologists should be aware of the diagnostic criteria for GNET in order to avoid misdiagnosis due to confusion with other non-epithelial gastrointestinal neoplasms.

[Ectopic hepatocellular carcinoma. A case report]. / Carcinoma hepatocelular ectópico: reporte de caso y revisión de la literatura.

Ectopic liver (EL) is a rare developmental anomaly caused by the migration of hepatic cells to other locations during embryogenesis. Its incidence is thought to be between 0.24 and 0.47% and can occur in various sites, although the gall bladder is the most frequent. Approximately 100 cases of EL have been published, of which 28 developed hepatocellular carcinoma, whilst the orthotopic liver was unaffected. All cases are reported in Asian and Caucasian patients. We report the case of a 42-year-old patient with a solid lesion measuring 17×12×12cm apparently arising from the posterior wall of the stomach, displacing various organs and suggestive of a gastrointestinal stromal tumour (GIST). The liver, biliary tract and other structures were unremarkable. Histopathology revealed a diagnosis of hepatocellular carcinoma in ectopic liver tissue.

Intestinal obstruction due to small intestinal metastasis from primary Merkel cell carcinoma of the gluteal region.

Merkel cell carcinoma (MCC) is a rare neoplasm of unknown multifactorial origin first described in 1972. It occurs most often in older Caucasian males and is typically associated with sun-exposed areas of skin. However, cases have also been reported in other areas, such as the trunk and the gluteal region. Metastatic disease will occur in up to one-third of cases at onset or during the course of the disease, including metastases to the abdominal organs. We present the case of a 53-year-old male with a history of primary MCC of the right buttock and local resection surgery. Eighteen months later, he presented with a small bowel obstruction and had an emergency segmental bowel resection. Pathology examination with immunohistochemistry concluded that findings were consistent with metastatic MCC.

[Mucinous cystic neoplasm of the liver: a case report and review of the literature]. / Neoplasia quística mucinosa hepática: reporte de caso y revisión de la literatura.

Mucinous cystic neoplasm (MCN) of the liver is an unusual cyst-forming epithelial neoplasm, typically showing no communication with the bile ducts. This neoplasm represents less than 5% of all cystic lesions of the liver and there are only 250 cases in the world literature. We present the case of a 23-year-old female with a 13.5 x 10.2 cm lesion, hypodense, lobulated, with multiple septa up to 2.5 mm thick and small cystic images inside, which produces intrahepatic bile duct and common bile duct dilatation. The pathological study concluded that the tumor corresponded to a mucinous cystic neoplasm of the liver.

Carcinoma adenoescamoso de colon: reporte de caso y revisión de la literatura / Adenosquamous carcinoma of the colon: a case report and review of the literature

RESUMEN El cáncer colorectal es el tercer tipo de neoplasia maligna más frecuentemente diagnosticada para ambos sexos. El adenocarcinoma es el tipo histológico que constituye el 86% de todos los casos de carcinoma colónico; sin embargo, existen otros subtipos menos frecuentes, como el carcinoma adenoescamoso, una neoplasia sumamente rara, pero con peor pronóstico y menor sobrevida. Presentamos el caso de una paciente mujer de 68 años con un tiempo de enfermedad de 2 años, caracterizado por anemia, hematoquezia y dolor abdominal. El estudio colonoscópico reveló la presencia de tumoración a nivel de colon ascendente, el cual luego de la resección quirúrgica y estudios anatomopatológicos, resultó corresponder a un carcinoma adenoescamoso primario colónico.

ABSTRACT Colorectal cancer is the third most frequently diagnosed cancer in both men and women. Adenocarcinoma is the most common pathologic subtype of colon cancer and constitutes 86% of all colon cancers. Nontheless, there are other less frequent sybtyes of colorectal carcinomas, such as adenosquamous carcinoma, an extremely rare form of colon cancer, but with worse prognosis and greater potential of metástasis. We present the case of a 68-year-old female presented with a two-month history of anemia, hematochezia and abdominal pain. The colonoscopic study presented a tumor in the ascending colon, which after surgical resection and pathological studies, turned out to be a primary colonoc adenosquamous carcinoma.

Neoplasia quística mucinosa hepática: reporte de caso y revisión de la literatura

La neoplasia quística mucinosa (NQM) primaria de hígado es una neoplasia poco usual de tipo epitelial que se caracteriza por la formación de quistes y que típicamente no tienen comunicación con los ductos biliares. Representa menos del 5% de todas las lesiones quísticas del hígado y existen solo 250 casos en la literatura mundial. Presentamos el caso de una paciente mujer de 23 años con una lesión de 13,5 x 10,2 cm, hipodensa, lobulada, con múltiples tabiques de hasta 2,5 mm de espesor y pequeñas imágenes quísticas en su interior, que condiciona dilatación de la vía biliar intrahepática a predominio izquierdo y del colédoco. El estudio anatomopatológico concluyó que la tumoración correspondía a una neoplasia quística mucinosa de hígado.

Mucinous cystic neoplasm (MCN) of the liver is an unusual cyst-forming epithelial neoplasm, typically showing no communication with the bile ducts. This neoplasm represents less than 5% of all cystic lesions of the liver and there are only 250 cases in the world literature. We present the case of a 23-year-old female with a 13.5 x 10.2 cm lesion, hypodense, lobulated, with multiple septa up to 2.5 mm thick and small cystic images inside, which produces intrahepatic bile duct and common bile duct dilatation. The pathological study concluded that the tumor corresponded to a mucinous cystic neoplasm of the liver.

[Adenosquamous carcinoma of the colon: a case report and review of the literature]. / Carcinoma adenoescamoso de colon: reporte de caso y revisión de la literatura.

Colorectal cancer is the third most frequently diagnosed cancer in both men and women. Adenocarcinoma is the most common pathologic subtype of colon cancer and constitutes 86% of all colon cancers. Nontheless, there are other less frequent sybtyes of colorectal carcinomas, such as adenosquamous carcinoma, an extremely rare form of colon cancer, but with worse prognosis and greater potential of metástasis. We present the case of a 68-year-old female presented with a two-month history of anemia, hematochezia and abdominal pain. The colonoscopic study presented a tumor in the ascending colon, which after surgical resection and pathological studies, turned out to be a primary colonoc adenosquamous carcinoma.

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

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 extraabdominal (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.

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.