Neoplasia pseudopapilar sólida del páncreas (tumor de Frantz) en una joven de 13 años / Solid pseudopapillary tumor of the pancreas (Frantz tumor) in a 13-year-old girl

RESUMEN El tumor sólido pseudopapilar de páncreas (TSP) es un tumor infrecuente, de bajo grado de malignidad, que representa el 1-3% de todas las neoplasias pancreáticas, con predilección por el sexo femenino, y es el tumor sólido de páncreas más frecuente en la infancia. Mujer de 13 años que consultó por dolor abdominal de 5 días de evolución, en hipocondrio derecho e irradiado a espalda, con náuseas y vómitos. TSP es diagnosticado con ecografía, tomografía computarizada (TC) y resonancia magnética (RM). Se realizó duodenopancreatectomía cefálica. La paciente fue dada de alta. El examen anatomopatológico informó un TSP de páncreas. El abordaje quirúrgico de estos tumores, abierto o laparoscópico, permite una excelente supervivencia alejada.

ABSTRACT Solid pseudopapillary tumor (SPT) of the pancreas is a rare neoplasm with a low malignant potential and represents 1- 3% of all pancreatic tumors. They usually occur in women and are the most common solid pancreatic tumor in children. A 13-year-old girl visited the emergency department due to abdominal pain in the right hypochondrium radiating to the back, nausea and vomiting lasting 5 days. A SPT was diagnosed by ultrasound, computed tomography (CT) scan and magnetic resonance imaging (MRI). The patient underwent cephalic pancreaticoduodenectomy and was discharged. The pathological study reported a SPT of the pancreas. The surgical approach of these tumors through open surgery or laparoscopy offers excellent long-term survival.

Aspectos anatomo-clínicos de un caso de neoplasia sólida seudopapilar de páncreas: (tumor de Frantz) / Solid pseudopapillary neoplasm of the pancreas: (Frantz´s tumor) anatomo-clinical aspects / Aspectos anátomo-clínicos de um caso de neoplasia sólida pseudopapilar do pâncreas: (tumor de Frantz)

El tumor pseudopapilar de páncreas, conocido como tumor de Frantz, es excepcional; comprende entre el 0.2 y 2.7 % de los carcinomas de páncreas. Su etiología es desconocida y predomina en mujeres jóvenes. Son tumores de bajo potencial maligno, que cursan asintomáticos y dan síntomas por compresión regional: dolor gravativo y/o masa abdominal palpable. La probabilidad de una forma definidamente maligna es de 15 %. Anatómicamente se localizan con mayor frecuencia en la cola del páncreas, seguido por la cabeza y el cuerpo. El tratamiento es la resección quirúrgica y su pronóstico es excelente, con sobrevida a 5 años de casi 100 %. El diagnóstico es anátomo-patológico, considerando el aspecto microscópico y el perfil inmunohistoquímico. Se presenta un caso de una mujer de 21 años, en la cual se realizó una cirugía de excéresis completa del tumor y que se mantiene a asintomática a 24 meses de operada.

Pseudo papillary tumor of the pancreas, also called Frantz´s tumor, is a very rare disease; between 0.2 and 2.7 % of pancreatic carcinomas. Ethiology is unknown; is observed in young women. It´s a tumors with low malignant potential, usually of asymptomatic evolution by long time and the symptoms are due to regional compression: pain or abdominal mass. The possibility of a malignant form is around 15 %. Anatomically, they are most frequently located in the tail of the pancreas, followed by the head and body. The treatment is the surgical resection; with an excellent prognosis and a 5-year survival almost 100 %. The diagnosis is with pathological study, considering the microscopic appearance and the immunohistochemical profile. Its perform an analysis of the literature and once case of a 21 years old, woman is reported. A complete resection surgery of the tumor was performed and remains asymptomatic at 24 months of follow-up.

O tumor pseudopapilar do pâncreas, conhecido como tumor de Frantz, é excepcional; compreende entre 0,2 e 2,7 % dos carcinomas pancreáticos. Sua etiologia é desconhecida e predomina em mulheres jovens. São tumores de baixo potencial maligno, que são assintomáticos e apresentam sintomas devido à compressão regional: dor intensa e/ou massa abdominal palpável. A probabilidade de uma forma definitivamente maligna é de 15 %. Anatomicamente, eles estão localizados com mais frequência na cauda do pâncreas, seguido pela cabeça e corpo. O tratamento é a ressecção cirúrgica e seu prognóstico é excelente, com sobrevida em 5 anos de quase 100 %. O diagnóstico é anátomo-patológico, considerando o aspecto microscópico e o perfil imuno-histoquímico. Apresenta-se o caso de uma mulher de 21 anos submetida a cirurgia de excisão completa do tumor e que permanece assintomática 24 meses após a cirurgia.

Carcinoma pseudopapilar sólido del páncreas / Solid pseudo-papillary carcinoma of the pancreas

El tumor sólido pseudopapilar del páncreas, conocido también como tumor de Frantz, es una enfermedad rara: neoplasia bien delimitada, de lento crecimiento, no agresiva pero maligna, habitualmente con pronóstico favorable. El tratamiento de elección es quirúrgico. Aunque algunos de ellos son agresivos a nivel local, la mayoría de los pacientes se curan con la resección completa del tumor. Se reportó el caso de una mujer de 30 años, ingresada en el Servicio de Cirugía General del Hospital Universitario Clínico Quirúrgico Comandante Faustino Pérez Hernández, con diagnóstico presuntivo clínico e imagenológico, de tumor pseudopapilar sólido del páncreas, con confirmación histológica tras la resección quirúrgica. Este infrecuente tumor debe ser considerado en el diagnóstico diferencial de los tumores pancreáticos, fundamentalmente en mujeres jóvenes(AU)

The solid pseudo-papillary carcinoma, also known as Frantz´s tumor, is a rare disease. It is a well-defined neoplasia, of low growth, non-aggressive but malignant, usually with a favorable prognosis. The elective treatment is the surgery. Although some of them are locally aggressive, most patients are healed with the complete tumor resection. The authors reported the case of a woman, aged 30 years who entered the Service of General Surgery of the University Hospital Comandante Faustino Pérez Hernández, with a presumptive clinical and imaging diagnosis of pancreas solid pseudo-papillary tumor, histologically confirmed after surgical resection. This infrequent tumor should be taken into account in the differential diagnosis of pancreatic tumors, mainly in young women(AU)

Solid pseudopapilar neoplasm of the pancreas.

Solid pseudopapillary tumor of the pancreas is a rare entity, more frequent in women between the 2nd and 4th decades. The diagnosis is usually incidental and it can be reached by computed tomography or magnetic resonance imaging. Subsequent pathological confirmation is necessary for an adequate treatment. A retrospective study of six cases was carried out. All the patients were female, between 14 and 56 years of age, in which 50% the tumor were an incidental finding. We had three cases located in the head and three in the body of the pancreas. We performed three pancreaticoduodenectomies and three distal pancreatectomies with splenic preservation, without disease recurrence.

El tumor sólido-quístico de páncreas es poco frecuente y predomina en mujeres entre la segunda y la cuarta décadas de la vida. Los pacientes son generalmente asintomáticos. El diagnóstico se realiza por imágenes con tomografía o resonancia magnética, y con la posterior confirmación patológica para poder ofrecer un tratamiento adecuado. Presentamos una serie de seis casos. Todas las pacientes fueron de sexo femenino, de entre 14 y 56 años. El 50% fueron un hallazgo incidental. Tuvimos tres casos localizados en la cabeza y tres en el cuerpo del páncreas. Se realizaron tres duodenopancreatectomías cefálicas y tres pancreatectomías distales con preservación esplénica, con buena evolución y sin recidiva.

Tumor papilar sólido-quístico pancreático / Solid pseudopapillary tumor of the pancreas

RESUMEN Antecedentes: la neoplasia sólida pseudopapilar del páncreas es una entidad rara, que típicamente se presenta en mujeres jóvenes. Suele presentar síntomas abdominales inespecíficos. Es un tumor maligno de bajo grado de malignidad. Objetivos : el objetivo del siguiente informe de serie de casos es presentar 9 casos tratados en un cen tro y realizar una revisión bibliográfica del tema. Material y métodos: estudio retrospectivo descriptivo de los casos con diagnóstico anatomopatoló gico de neoplasia sólida pseudopapilar en el Servicio de Cirugía General, desde febrero de 2013 hasta septiembre de 2019. Se contemplaron como variables: edad, sexo, localización del tumor, tratamiento quirúrgico realizado, tiempo operatorio, complicaciones, estancia hospitalaria y seguimiento alejado. Resultados: fueron 9 casos, todos de sexo femenino con media de edad de 30 años (rango 20 a 70 años). La localización más frecuente fue en cola de páncreas en 4 casos (45%). Todas las pacientes fueron sometidas a cirugía, con abordaje laparoscópico en el 60% de los casos (n = 5); la resección pancreática distal con preservación esplénica fue la conducta más utilizada (n = 6). Se constataron tres complicaciones, de las cuales dos fueron colecciones abdominales como consecuencia de una fístula pancreática que se abordaron por vía percutánea, y la restante fue un retardo del vaciamiento gástrico por lo cual la paciente requirió internación prolongada. Conclusión: la neoplasia sólida pseudopapilar pancreática es una enfermedad poco frecuente, ma ligna pero con bajo riesgo de malignidad. Presenta buena sobrevida cuando se somete a cirugía de carácter curativo; la laparoscopia es la vía de abordaje de elección en centros con experiencia.

ABSTRACT Background: Solid pseudopapillary tumor of the pancreas is a rare condition that affects young women. The most common symptom is unspecific abdominal pain. It is a malignant tumor of low malignant potential. Objective: The aim of this study is to report a case series of patients treated in a single center and perform a bibliographic review. Material and methods: We conducted a retrospective study of the cases with pathological diagnosis of solid pseudopapillary tumor of the pancreas treated in the Department of General Surgery between February 2013 and September 2019. The following variables were analyzed; age, sex, tumor location, surgical treatment, operative time, complications, length of hospital stay and long-term follow-up. Results: Nine patients were included; all of them were women with mean age of 30 years (range: 20 - 70 years). The most common location of the tumor was the tail of the pancreas (n = 4; 45%). Surgery was performed in all the cases; five cases underwent video-assisted laparoscopy and spleen-preserving distal pancreatectomy was the technique more commonly used (n = 6). Three complications were recorded: two abdominal collections due to biliary leaks were trated by percutaneous approach and the other patient presented delayed gastric emptying and required prolonged hospitalization. Conclusion: Solid pseudopapillary tumor pf the pancreas is a rare low-grade malignant neoplasm. The prognosis is favorable after surgery and laparoscopy is the preferred approach in centers with experience.

Pancreatoduodenectomy for solid pseudopapillary tumor of the pancreas: a multi-institution study / Duodenopancreatectomia para o tumor pseudopapilar sólido do pâncreas: estudo multi-institucional

ABSTRACT Background: Solid pseudopapillary tumor of the pancreas is a rare low-grade malignant neoplasm. Most patients present with nonspecific symptoms until the tumor becomes large. Complete surgical resection by pancreatoduodenectomy is the treatment of choice for tumors located in the head of the pancreas Aim: To analyzed the clinicopathologic features, management, and outcomes of patients who had solid pseudopapillary tumor of the head pancreas and underwent surgical resection. Methods: Were analyzed 16 patients who underwent pancreatoduodenectomy for this condition. Results: Mean age was 25.7 years old, and 15 patients were female (93.7%). Nonspecific abdominal pain was present in 14 (87.5%). All underwent computed tomography and/or magnetic resonance imaging as part of diagnostic workup. The median diameter of the tumor was 6.28 cm, and surgical resection was performed with open or laparoscopic pancreatoduodenectomy without neoadjuvant chemotherapy. Postoperative complications occurred in six patients (37.5%) and included pancreatic fistula without mortality. The mean of hospital stay was 10.3 days. Median follow-up was 3.6 years, and no patient had local recurrence or metastatic disease. Conclusion: For these patients surgical resection with pancreatoduodenectomy is the treatment of choice showing low morbidity, no mortality, and good long-term survival.

RESUMO Racional: Tumor sólido pseudopapilar do pâncreas é neoplasia maligna rara, de baixo grau de malignidade. A maioria dos pacientes apresenta sintomas inespecíficos até que o tumor aumente de tamanho. A ressecção cirúrgica completa através a duodenopancreatectomia é o tratamento de escolha para os localizados na cabeça do pâncreas. Objetivo: Analisar as características clinicopatológicas, tratamento e resultados de pacientes com tumor sólido pseudopapilar do pâncreas localizado na cabeça do pâncreas submetidos à ressecção cirúrgica. Método: Foram analisados 16 pacientes com duodenopancreatectomia devido a esse tumor localizado na cabeça do pâncreas. Resultados: Havia 15 mulheres (93,7%) e a média de idade era de 25,7 anos. Dor abdominal não específica esteve presente em 14 pacientes (87,5%). Todos realizaram tomografia computadorizada do abdome e/ou ressonância nuclear magnética como parte da investigação. O diâmetro médio do tumor era de 6,28 cm e a ressecção cirúrgica foi realizada por duodenopancreatectomia, tanto por laparotomia quanto por videolaparoscopia, com ou sem quimioterapia neoadjuvante. As complicações pós-operatórias ocorreram em seis pacientes (37,5%) e incluíram fístula pancreática, sem mortalidade. O tempo médio de internação hospitalar foi de 10,3 dias. O tempo médio de seguimento foi de 3,6 anos e nenhum paciente apresentou recorrência local ou doença metastática. Conclusões: A ressecção cirúrgica através da duodenopancreatectomia é o tratamento de escolha para estes pacientes. Os resultados mostraram baixa morbidade, nenhuma mortalidade e boa sobrevida em longo prazo.

Tumor sólido pseudopapilar de páncreas: Tumor de Frantz / Solid pseudopapillary tumor of the pancreas: Frantz´s tumor

El tumor pseudopapilar de páncreas, también conocido como tumor de Frantz, es una enfermedad muy rara en nuestro medio, comprende menos del 3 % de los tumores pancreáticos (1). Su etiología es desconocida, y su incidencia se observa en mujeres jóvenes con predominancia de la tercera década de la vida. Presentamos el caso de una mujer de 23 años que ingresa por epigastralgia urente desde hace 3 meses, además de llenura precoz. Al examen presenta dolor a la palpación profunda. Cuenta con perfil bioquímico, hepático, marcadores tumorales dentro de valores normales, la tomografía espiral multicorte (TEM) abdomino-pélvica reporta lesión neoformativa sólida quística de morfología redondeada de bordes bien definidos, la patología confirma tumor sólido pseudopapilar de páncreas. La paciente se somete a resección de tumor, con evolución favorable.

The solid pseudopapillary tumor of the pancreas, also known as Frantz's tumor, is a very rare disease in our country, comprising less than 3 % of pancreatic tumors (1). Its etiology is unknown and it is predominantly found in young women in their third decade of life. We present the case of a 23-year-old woman who was admitted due to a 3-month burning epigastralgia and early satiety. On examination, she had pain with deep palpation. Her biochemical profile, liver profile, and tumor markers were within normal values. An abdomen and pelvis multislice helical/spiral computed tomography (CT) scan showed a solid cystic neoformative lesion with rounded structure and well-defined borders. Pathology confirmed a solid pseudopapillary tumor of the pancreas. The patient underwent a tumor resection with favorable evolution.

Tumor de Frantz. presentacion de 2 casos / Frantz´s tumor. a two cases presentation

La neoplasia sólida pseudopapilar de páncreas es una neoplasia maligna de bajo grado, relativamente poco frecuente, que representa entre 0.9 y 2.7 % de todas las neoplasias malignas pancreáticas. Afecta principalmente mujeres jóvenes (89 % de los casos), con una edad promedio al diagnóstico de 28 años. La mayoría presentan sintomatología inespecífica relacionada a una masa intrabdominal y más de un tercio suelen ser descubiertas incidentalmente. Presentamos dos casos (una paciente de 19 años de edad diagnosticada de una neoplasia localizada en cuerpo de páncreas y una paciente de 28 años con tumor en la cabeza, que correspondían a tumor de Frantz. Analizamos las características biológicas de estos tumores, sus formas de presentación y las pruebas complementarias recomendadas para su diagnóstico, así como una búsqueda en la literatura.

The solid pseudopapillary neoplasm of the pancreas is a low-grade malignancy, relatively rare, representing between 0.9 and 2.7% of all pancreatic malignancies. It mainly affects young women (89% of cases), with a mean age at diagnosis of 28 years. Most have specific symptoms related to intra-abdominal mass and more than one third are usually discovered incidentally. We report two cases ( a 19-years-old woman diagnosed of a neoplasm in the tail and another 28 years old woman , with neoplasm in the head of the pancreas which could be classified as Frantz’s tumor. We analyze the biological characteristics of these tumors, their forms of presentation and the recommended explorations for the diagnostic.

Tumor sólido-cístico pseudopapilar do pâncreas (tumor de Frantz): relato de dois casos e revisão de literatura / Pseudopapillary solid-cystic tumor of pancreas (Frantz's tumor): two cases report and literature review

O tumor sólido-cístico pseudopapilar do pâncreas (TSCPP) - tumor de Frantz - é uma neoplasia rara do pâncreas que ocorre principalmente em pacientes jovens e apresenta um bom prognóstico. Pouco mais de 700 casos foram relatados na literatura mundial. A maioria das publicações estudadas relatam casos deste tumor em pacientes do sexo feminino, que ao diagnóstico apresenta-se oligossintomáticas ou assintomáticas, com massa abdominal palpável, em que a tomografia computadorizada evidencia presença de massa sólidocística. Dois terços destes tumores ocorrem no corpo e cauda do pâncreas e, apesar do crescimento excessivo para fora dos limites pancreáticos, raramente invadem estruturas vasculares ou órgãos adjacentes. A maioria dos pacientes são curados com a ressecção completa do tumor, porém 10 a 15% apresentam metástase sincrônica ou metacrônica. Em nossa casuística, um deles apresentou-se de forma clássica: mulher jovem, oligossintomática, submetida à pâncreatectomia corpo-caudal com preservação do baço, que obteve cura com a ressecção da lesão. No segundo caso, paciente do sexo masculino, sexagenário, submetido a ressecção corpo-caudal do pâncreas, apresentou recidiva hepática dois anos após tratamento da lesão primária e foi submetido à ressecção da lesão metastática do fígado. Ambos atualmente em acompanhamento e sem evidência de doença. Enfatiza-se a necessidade de se considerar o tumor de Frantz no diagnóstico diferencial de massa abdominal, sólido-cística em topografia pancreática e com crescimento indolente e oligossintomático. O objetivo deste trabalho é mostrar dois casos tumor sólido-cístico psudopapilar do pâncreas, sendo um caso típico e outro atípico: homem, sexagenário que evoluiu com lesão metastática

The pseudopapillary solid-cystic tumor of pancreas (TCPP) - Frantz tumor - is a rare neoplasm of the pancreas that occurs mainly in young patients and has a good prognosis. Just over 700 cases have been reported in the world literature. The majority of studied publications reported cases of female patients, introducing itself oligosymptomatic or asymptomatic with palpable abdominal mass, where the CT scan shows the presence of solid-cystic mass. Two-thirds of these tumors occur in the body and tail of the pancreas, and despite the excessive growth beyond pancreatic limits, it rarely invaded adjacent organs or vascular structures. Most of patients are cured with complete tumor resection, but 10 to 15% have synchronous or metachronous metastasis. In our series, one of them have presented a classical form: young woman, oligosymptomatic, submitted to pancreatectomy body-caudal with preservation of the spleen, which achieved cure with resection of the lesion. In the second case, the male patient in his sixties, subjected to body-tail pancreatic resection, liver showed recurrence two years after treatment of the primary lesion and underwent resection of metastatic liver damage. Currently both are in monitoring and have no evidence of disease. It emphasizes the need to consider the Frantz tumor in the differential diagnosis of abdominal mass, especially when in pancreatic topography and indolent growth and oligosymptomatic. The objective of this study is to show two cases solid-cystic psudopapilar tumor of the pancreas, one tipycal case and other atypical: man, in his sixties who developed metastatic lesion

La neoplasia sólida-pseudopapilar del páncreas como entidad enigmática / The solid tumor-the pancreas pseudopapillary as enigmatic entity

El tumor sólido-pseudopapilar del páncreas, conocido también como tumor de Frantz, es una rara enfermedad. Afecta principalmente a mujeres jóvenes, no blancas. Tiene componentes sólidos y quísticos. Constituye una neoplasia maligna con un pronóstico, generalmente alentador. Realizamos una revisión de la literatura con el objetivo de actualizar algunos puntos clave acerca del tema. Se incluyeron para la búsqueda las siguientes palabras claves: tumor sólido-quístico, tumor quístico papilar, pseudopapilar, sólido y quístico, tumor de páncreas, tumor pancreático, neoplasia del páncreas, neoplasia pancreática, sólido pseudopapilar y tumor de Frantz. Los acápites que desarrollamos son patogénesis, biología molecular, patología, características clínicas, imaginología y diagnóstico, tratamiento y por último pronóstico. Existen algunos aspectos no bien dilucidados en estos tumores. Por ejemplo, se necesitan más investigaciones en áreas como patogénesis, biología molecular y tratamiento no quirúrgico(AU)

Solid-pseudopapillary of the pancreas, also known as Frantz tumor, is a rare disease. It mainly affects non-Caucasian young women; it has solid and cystic components and represents a malignant neoplasm with generally encouraging prognosis. To this end, a literature review was made to update some key points on the subject. The following keywords were used: solid-cystic turmor, papillary cystic tumor, pseudopapillary, solid and cystic, pancreas tumor, pancreatic tumor, pancreas neoplasm, pancreatic neoplasm, solid-pseudopapillary and Frantz' tumor. The sections that we further developed were pathogenesis, molecular biology, pathology, clinical characteristics, imaging and diagnosis, treatment and finally prognosis. There are some poorly clarified in these tumors, so more research work is needed in pathogenesis, molecular biology and non-surgical treatment(AU)

Tumor solido pseudopapilar de pancreas. tumor de frantz: Reporte de un caso / Pancreas pseudopapillary solid tumor. frantz tumor: Report of a case

El tumor solido pseudopapilar de páncreas o tumor de Frantz, descrito por primera vez en 1959 por V.K. Frantz; constituye menos del 1% de las neoplasias pancreáticas. Es más frecuente en mujeres jóvenes aunque también ha sido descrita en hombres. Es una neoplasia epitelial maligna de bajo grado, de comportamiento benigno por su bajo poder metastático y muy difícil de diagnosticar. La resección quirúrgica completa tienen una sobrevida del 93% a cinco años. En este artículo se presentan un caso de una paciente femenina de 33 años intervenida quirúrgicamente por tumor abdomin al en Noviembre de 2010 cuyo diagnostico histológico final fue tumor solido pseudopapilar de páncreas o tumor de frantz.

The pancreatic solid pseudopapillary tumor, also called tumor Frantz, first described in 1959 by V.K. Frantz; constitutes less than 1% of pancreatic tumors. It is more common in young women but has also been described in men. It is a malignant epithelial neoplasm of low-grade, benign behavior due to their low metastatic and very difficult to diagnose. Complete surgical resections have a survival of 93% at five years. In this paper we present a case of a female patient of 33 years underwent surgery for abdominal tumor in November, 2010 whose final histological diagnosis was pancreatic solid pseudopapilar tumor or tumor Frantz.

Tumor sólido pseudopapilar del páncreas en la IV región / Solid-pseudopapillary tumor of pancreas

Introduction: The solid-pseudopapillary tumor of pancreas (STP) is a low-grade malignant neoplasm. In Chile, 21 cases have been reported since 2008, most of them treated in Santiago. The present series contributes to the national case-load and has the purpose to describe the experience with this uncommon neoplasm in the IV Region. Patients and Methods: From January 2004 to March 2014, a total of 38 benign and malignant pancreatic neoplasms have been informed in the data-base of our Regional Pathology Service. We selected the biopsies informed as STP and 5 cases (13 percent) were found. The clinical records of these patients were retrospectively reviewed. The results were reported using descriptive statistics with central tendency measures and dispersion. Results: Most patients were women with a mean age of 44.8 years. All patients were studied with either abdominal ultrasound; computed tomography or magnetic resonance. In 2 patients the STP was located in the pancreatic tail, and they were submitted to distal pancreatectomy. The other 3 patients had the STP in the pancreatic head and were resolved by pancreatoduodenectomy. All tumors were confirmed by immunohistochemistry. Late follow-up showed malignant behavior in 1 patient while the other 4 patients are currently free of disease. Conclusions: The general characteristics of STP in the IV Region are similar to what is currently known. They present a high incidence compared with the habitually published incidence.

Introducción: El tumor sólido pseudopapilar del páncreas (TSP) es una neoplasia con bajo potencial maligno. En Chile se han reportado 21 casos desde el año 2008, 18 de ellos tratados en instituciones de Santiago. La presente serie además de contribuir a la casuística nacional, tiene como objetivo la descripción de la experiencia de la IV Región con el manejo de estos tumores. Pacientes y Métodos: Entre enero de 2004 y marzo de 2014 se informaron 38 biopsias de tumores pancreáticos benignos y malignos en la base de datos del Servicio de Patología Regional. Se seleccionaron las biopsias informadas como TSP, las que constituyen 5 casos (13 por ciento) y se revisaron las fichas clínicas en forma retrospectiva. Para el informe de los resultados se utilizó estadística descriptiva con medidas de tendencia central y dispersión. Resultados: El promedio de edad fue 44,8 años, siendo la mayoría mujeres. Los pacientes fueron estudiados con ecografía, tomografía computarizada y resonancia magnética. En 2 pacientes el TSP se localizaba en la cola del páncreas, estos pacientes fueron sometidos a pancreatectomía distal. En los otros 3 casos el TSP se encontraba en la cabeza del páncreas y fueron resueltos mediante pancreatoduodenectomía. Los TSP fueron confirmados por inmunohistoquímica. El comportamiento posterior fue benigno en 4 casos y maligno en 1 caso. Conclusiones: Las características de los TSP en la IV Región son similares a las conocidas y presentan una alta incidencia comparada con la habitualmente reportada.

Tumor sólido pseudopapilar del páncreas / Solid pseudopapillary tumor of the pancreas

El tumor sólido pseudopapilar del páncreas es una patología de baja incidencia, correspondiendo el 1 -2% de los tumores pancreáticos, es de naturaleza benigna y con bajo potencial de malignización. El tratamiento quirúrgico por lo general otorga larga supervivencia, alrededor del 90% a los 5 años siendo esta conducta mandatorio. Se reporta el caso de un paciente operado en el servicio.

Solid pseudopapillary tumor of the pancreas is a disease of low incidence, where 1 -2% of pancreatic tumors is benign and low malignant potential. Surgical treatment usually gives long survival, about 90% at 5 years as this conduct mandatory. We report the case of a patient operated on service.

Tumor de Frantz: neoplasia sólida pseudopapilar de páncreas / Frantz tumor: a pancreatic pseudopapillary solid neoplasia

Nosotros reportamos un caso de neoplasia sólida pseudopapilar de páncreas en una mujer de 35 años. Ella refirió haber iniciado desde hace tres meses una enfermedad progresiva caracterizada por dolor epigástrico y baja de peso. En el examen físico solo mostró dolor moderado a la palpación profunda de mesogastrio, no se palpó alguna masa abdominal. Los exámenes auxiliares mostraron hemoglobina 13.3gr; leucocitos, plaquetas, perfil de coagulación, bioquímica, electrolitos, albúmina, globulinas, bilirrubinas, transaminasas y fosfatasa alcalina fueron normales. Examen de orina: leucocitos 14-16 por campo, urocultivo: negativo, Ca 19.9: 21.2 (0-37). Arco V (anticuerpos IgG E. granulosus):, 17.3 (nivel dudoso), Inmunoblot quiste hidatídico: negativo. La ecografía abdominal mostro entre cuerpo y cola de páncreas una imagen hipoecogénica de aspecto sólido. En la tomografía abdominal se detectó en el cuerpo del páncreas la presencia de dos nódulos hipodensos con densidad promedio de 25 U.H. y que medían 50 y 22 mm de diámetro. Las imágenes por resonancia magnetica nuclear determinaron en el cuerpo del páncreas un quiste bilobulado de 45 y 25 mm de diámetro, con isointensidad en T1, hiperintensidad en T2, sin cambios tras la saturación grasa, con realce periférico por el contraste. Intervenida quirúrgicamente, se encontró una tumoración quística en el cuerpo del páncreas; sin signos de infiltración a otros órganos. Se realizó pancreatectomía distal y esplenectomía. La tumoración era ovalada y medía 60x46x35 mm, encapsulada, de consistencia irregular, que al corte dejaba fluir material de aspecto necrótico. El estudio histológico demostró el típico patrón pseudopapilar con estroma perivascular hialinizado; en los estudios de inmunohistoquimica se observo inmunoreactividad positiva a ciclina D1 y al receptor de progesterona; también hubo positividad para vimentina, enolasa neuronoespecífica y CD-56...

We report a case of solid and papillary epithelial neoplasm of the pancreas in a 35-year-old female. She presented with epigastric pain and weight loss, these symptoms developed 3 months before. Physical examination revealed mild pain on deep palpation of mesogastrium, without palpable abdominal mass; rest of the examination was normal. The laboratory data showed hemoglobin 13.3gr/dl; WBC, platelets, blood coagulation test, blood biochemistry, electrolytes, liver function test were normals. Urine test: 14-16 leukocytes per field, urocultive negative, Ca 19.9: 21.2 (0-37). Her serology for hydatic cyst (arc V and inmunoblot) was negative. Abdominal ultrasound showed between body and tail of the pancreas a solid hypoechoic image. In abdominal CT was detected in the body of the pancreas the presence of two hypodense lesions with average density of 25 UH and mediate 50 and 22 mm in diameter. The nuclear magnetic resonance imaging identified the body of the pancreas, bilobed cyst of 45 and 25 mm in diameter, with isointensity in T1, hyperintensity in T2, unchanged after fat saturation, with peripheral contrast enhancement. Operation findings showed a cystic tumor in the body of pancreas without signs of infiltration to other organs. Therefore, distal pancreatectomy and splenectomy were carried out. The tumor was 60x46x35 mm and a half oval, encapsulated, irregular consistency, which the court left necrotic-looking material flow. The study showed the pattern typical hyalinized perivascular pseudopapillary with stroma, in immunohistochemical studies positive imunoreactivity was observed in cyclin D1 and progesterone receptor, also were positive for vimentin, enolase neuronoespecífica and CD-56. The proliferation index assessed by Ki-67 was less than 1% in tumor cells...

Pancreatectomia corpo-caudal com preservação esplênica para o tratamento de tumor sólido pseudopapilar de pâncreas (Tumor de Frantz) / Corpo-caudal pancreatectomy with splenic preservation in the treatment of a solid pseudopapillary tumor (Frantz tumor)

Objetivos: descrever um caso de tumor sólido pseudopapilar de pâncreas (tumor de Frantz), neoplasia infrequente, que entretanto tem sido cada vez mais diagnosticada e relatada na literatura. Descrição do Caso: uma adolescente de18 anos consultou com queixa de dor abdominal persistente em hipocôndrio esquerdo. Ecografia abdominal e tomografia computadorizada de abdome revelaram lesão sólido-cística na transição entre o corpo e a cauda do pâncreas. A paciente foi submetida a pancreatectomia corpo-caudal com preservação esplênica. O exame anatomopatológico revelou tratar-se de um tumor sólido pseudopapilar de pâncreas. Houve excelente evolução pós-operatória, com resolução completa das queixas, estando a paciente assintomática seis meses após o procedimento. Conclusões: o tumor sólido pseudopapilar de pâncreas é uma neoplasia rara, ocorre predominantemente em mulheres jovens e é considerada uma lesão de baixo grau de malignidade. Sintoma de dor abdominal inespecífica é a apresentação clínica característica, e o diagnóstico é realizado por exames de imagem. Ressecção cirúrgica completa do tumor é o tratamento de escolha.

Aims: To present a case of solid pseudopapillary tumor (Frantz tumor), an infrequent pancreatic neoplasm, which has been increasingly diagnosed and reported in the literature. Case Description: A 18-year old woman presented with pain and tenderness in the left upper abdominal quadrant. Abdominal ultrasound and computed tomography scan revealed a solid and cystic lesion in the body of the pancreas. The patient underwent a corpo-caudal pancreatectomy with splenic preservation. Pathologic analysis of the specimen revealed a solid pseudopapillary tumor. Postoperative course was uneventful with complete resolution of the symptoms, and the patient is completely asymptomatic six months after the operation. Conclusions: Pancreatic solid pseudopapillary tumor is a rare neoplasm that predominantly occurs in young women, and is generally considered a low-grade malignant tumor. Its clinical presentation is nonspecific abdominal symptoms, and the diagnosis is performed by image tests. Complete surgical resection of primary tumor is the treatment of choice.

Tumor sólido pseudopapilar de páncreas: presentación de un caso y revisión de la literatura / Solid pseudopapillary tumor of the pancreas: case report and literature review

Introducción: El tumor sólido pseudopapilar del páncreas es un tumor epitelial de baja malignidad que afecta a mujeres jóvenes y corresponde de 1 a 2 % de todas las neoplasias del páncreas. Presentamos un caso tratado en el Hospital General de Tijuana, y revisión de la literatura. Caso clínico: Mujer de 37 años de edad, con síntomas de saciedad temprana y distensión abdominal. Durante una colecistectomía abierta se identificó un tumor en el cuerpo del páncreas, se tomó biopsia y estableció el diagnóstico de tumor sólido pseudopapilar páncreas. Fue tratada con pancreatectomía distal y esplenectomía. Conclusiones: El tumor sólido pseudopapilar del páncreas es la neoplasia de etiología desconocida más frecuente en mujeres jóvenes, de diagnóstico diferencial ante tumor en páncreas, aunque por su rareza no debe ser la primera opción a descartar. Las manifestaciones clínicas incluyen dolor abdominal, sensación de plenitud o saciedad temprana, masa abdominal, náusea y vómito, entre otras. Los exámenes de laboratorio por lo general son normales. La tomografía axial computarizada puede revelar masa heterogénea grande con una cápsula. El diagnóstico se realiza mediante biopsia y la cirugía por sí sola tiene un nivel de curación excelente y se debe intentar en todos los casos.

BACKGROUND: Solid pseudopapillary tumor of the pancreas is an epithelial tumor of low malignancy that primarily affects young women and represents approximately 1-2% of all pancreatic neoplasms. We present a case of this type of tumor treated in the General Hospital of Tijuana, Mexico, as well as a review of the literature. CLINICAL CASE: We present the case of a 37-year-old female with symptomatology of early satiety and abdominal distension. During open cholecystectomy we found a tumor in the body of the pancreas. Biopsy was done, establishing the diagnosis of solid pseudopapillary tumor of the pancreas. The patient was treated successfully with distal pancreatectomy and splenectomy. DISCUSSION: Solid pseudopapillary tumor of the pancreas is a rare neoplasm. It is more frequent in young women and has an unknown etiology. Clinical manifestations include abdominal pain, sensation of plenitude or early satiety, abdominal mass, nausea and vomiting. Laboratory tests are usually normal. Computerized axial tomography may show a large encapsulated heterogeneous mass. Diagnosis is established through biopsy and surgery is the best treatment for this pathological entity. CONCLUSIONS: One may conclude that the solid pseudopapillary tumor is a differential diagnosis in the presence of pancreatic tumors, although due to its rarity it is not the first option to discard. Surgery represents the best treatment for this pathological entity and should be attempted in all cases, independent of the size of the pancreatic injury.

Tumor de Frantz: presentación de un caso / Frantz's tumor: report of a case

El tumor papilar sólido-quístico de páncreas es una neoplasia muy poco frecuente y de origen incierto. Suele afectar a mujeres jóvenes, cursa con escasa sintomatología y es considerado en general como una neoplasia de bajo grado de malignidad. Su tratamiento es quirúrgico, presentando un excelente pronóstico tras resección completa incluso si existen metástasis. Presentamos el caso de una paciente de 14 años de edad diagnosticada de una neoplasia localizada en cola de páncreas que correspondía a un tumor de Frantz. Analizamos las características biológicas y ultraestructurales de estos tumores, sus formas de presentación y las pruebas complementarias recomendadas para su diagnóstico.

Solid-cystic-papillary tumor of pancreas is an uncommon neoplasm and unknown origin. It generally occurs in young women, the symptomatology is limited and is considered a low-grade malignant tumor. Treatment is surgical and the prognosis is usually good after complete resection, even if metastatic dissemination exists. We report a case of a 14-years-old woman diagnosed of a neoplasm in the tail of the pancreas which could be classified as Frantz's tumor. We analyze the biological and ultrastructural characteristics of these tumors, their forms of presentation and the recommended explorations for the diagnostic.