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.

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.

Neoplasia sólida pseudopapilar del páncreas / Solid pseudopapillary tumor of the pancreas

Antecedentes: la neoplasia sólida pseudopapilar (NSSP) del páncreas es un raro tumor pancreático que se da comúnmente en mujeres jóvenes. Por lo general, son poco sintomáticos y el pronóstico es bueno ante cirugía resectiva. Objetivo: describir 6 casos tratados en 3 centros diferentes y hacer una revisión actualizada del tema. Material y métodos: análisis de historias clínicas, biopsias y protocolos quirúrgicos. Período enero de 2014 hasta abril de 2017. Resultados: encontramos 6 casos (5 mujeres y 1 hombre); el promedio de edad fue 39,7 años (rango 28 a 54 años). En 3 casos se trató de hallazgos incidentales. En todos los casos se utlizaron Ecografia y tomografia computarizada (TC) para el diagnóstico y en 2 casos se realizó resonancia magnética (RM). Todos los tumores se encontraron en el cuerpo o la cola del páncreas o en ambos. En 2 cirugías se realizó un abordaje videolaparoscópico; el tempo quirúrgico promedio fue de 91,17 minutos y el promedio de días de hospitalización fue de 5,5, con 3 pacientes que reingresaron por complicaciones durante el posoperatorio (un absceso y 2 fistulas pancreáticas). Conclusiones: se trata de tumores poco frecuentes; sin embargo, creemos que el avance en métodos por imágenes permitrá aumentar el diagnóstico y tratamiento de esta patología por lo que su incidencia aumentará. Queremos destacar la importancia del abordaje videolaparoscópico para este tpo de patología, ya que en la mayoría de los casos resulta factible con buenos resultados. Estas cirugías deben realizarse en centros donde puedan manejarse sus complicaciones para evitar reintervenciones.

Background: Solid Pseudopapillary Neoplasia (NSSP) pancreas is a rare pancreatic tumor commonly found in young women. They are usually not very symptomatic and the prognosis is good in resective surgery. Objective: to describe 6 cases from 3 diferent centers and to make an updated review of the topic. Materials and methods: analysis of clinical histories, biopsies and surgical protocols. Period from January 2014 to April 2017. Results: we found 6 cases, with 5 women and 1 male; the mean age was 39.7 years (range 28 to 54 years). In 3 cases we dealt with incidental findings. In all cases, echography and CAT were used for the diagnosis and in 2 cases, MRI was performed. All tumors were found in the body and / or tail of the pancreas. In 2 surgeries a videolaparoscopic approach was performed, the mean surgical tme was 91.17 minutes and the mean number of days of hospitalizaton was 5.5, with 3 patents reentered for postoperative complicatons (one abscess and two pancreatic fistulas). Conclusions: these tumors are rare; however we believe that the advances in imaging methods will increase the diagnosis and treatment of this pathology so that its incidence will increase. We want to emphasize the importance of the videolaparoscopic approach for this type of pathology since in most cases it is feasible with good results. These surgeries should be performed in a center where their complicatons can be managed to avoid reinterventons.

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 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 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 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.

Neoplasia papilar cística do pâncreas / Papyllary cystic tumor of the pancreas

Papyllary cystic tumor of the pancreas, so-called Frantz's tumor, is rare. Clinical presentation of this disease is usually a slowly growing abdominal mass with or without abdominal pain, affecting predominantly young females. Its pathogenesis is still unknown . Surgical resection is usually curative, and prognosis is excellent. The authors report two pancreatic tumor cases(Frantz's tumor) in women aged 26 and 31 years old. Pre operative assessment showed a solid-cystic tumor of the tail and body of the pancreas. An extended distal pancreatectomy was performed without splenic preservation.