Neoplasia quística mucinosa gigante del páncreas tratada con pancreatectomía corporocaudal y preservación esplénica / Giant mucinous cystic neoplasm of the pancreas undergoing pancreatectomy with splenic preservation

RESUMEN: La neoplasia quística mucinosa del páncreas (NQMP) es un tumor infrecuente, que afecta predominantemente a mujeres (90-95 %), afectando especialmente entre la quinta y séptima década de la vida; cuyo descubrimiento suele ser incidental. Por lo general, son lesiones solitarias, sin compromiso del conducto pancreático principal; poco sintomáticas y asociadas a malignidad (10 % a 40 %). El objetivo de este manuscrito fue reportar un caso de neoplasia quística mucinosa gigante del páncreas intervenida quirúrgicamente y revisar la evidencia existente respecto de sus características morfológicas, terapéuticas y pronósticas. Mujer de 29 años, con masa abdominal poco sintomática. El diagnóstico se verificó por medio de ultrasonografía, tomografía axial computarizada y resonancia nuclear magnética. Se intervino quirúrgicamente, realizándose pancreatectomía corporocaudal con preservación esplénica, sin incidentes. La paciente fue dada de alta al quinto día, sin complicaciones, y evolucionó de forma adecuada, sin complicaciones postoperatorias. La NMQP es una lesión compleja, que puede asociarse a malignidad, pero el diagnóstico preoperatorio de malignidad no puede establecerse con seguridad. El pronóstico depende de un diagnóstico precoz y un tratamiento oportuno.

SUMMARY: Mucinous cystic neoplasm of the pancreas (MCNP) are variable types of tumors, which predominantly affect women (90-95 %), and usually appear incidentally in the 5th to 7th decade of life. They are generally solitary lesions, with no involvement of the main pancreatic duct, rarely symptomatic and are associated with malignancy (10 % to 40 %). The aim of this manuscript was to report a case of giant mucinous cystic neoplasm of the pancreas surgically treated and review the existing evidence regarding its morphological, therapeutic and prognosis characteristics. Patient: A 29-year-old woman with a slightly symptomatic abdominal mass. The diagnosis was verified with ultrasound, computed axial tomography and magnetic nuclear resonance. The patient underwent surgery; an uneventful corporocaudal pancreatectomy with splenic preservation was performed. She was discharged on the fifth day, and has evolved adequately, without postoperative complications. MCNP is a complex lesion, which can be associated with malignancy, but the preoperative diagnosis of malignancy cannot be established with certainty. Its prognosis depends on early diagnosis and timely treatment.

Cistoadenoma mucinoso de la válvula ileocecal: tumoración intestinal infrecuente en un niño de 1 año. Reporte de caso / Mucinous cystadenoma of the ileocecal valve. Uncommon intestinal tumor in a oneyear-old boy. Case report

RESUMEN El cistoadenoma mucinoso se encuentra usualmente en el ovario, páncreas y el apéndice, pero su presentación en el intestino es extremadamente rara. En este reporte de caso, presentamos a un niño con obstrucción parcial intestinal debido a un cistoadenoma mucinoso en la válvula ileocecal. En la cirugía se retiró el íleo terminal, válvula ileocecal, ciego y apéndice, seguido de anastomosis ileocecal. El paciente evolucionó favorablemente en el postoperatorio y se recuperó sin contratiempos. A nuestro entender, este es el primer reporte de presentación de este tumor en dicha localización.

ABSTRACT Mucinous cystadenoma is usually found in the ovary, pancreas and appendix but its presentation in the intestine is extremely rare. In this case report we present an infant with partial intestinal occlusion due to a mucinous cystadenoma of the ileocecal valve. We performed an excision of the terminal ileum, ileocecal valve, cecum and appendix, followed by ileocolic anastomosis. The patient did well after the procedure and recovered uneventfully. To our knowledge, this is the first case report of this tumor in this location.

Giant appendiceal mucinous cystadenoma treated by laparoscopy: a case report and review of the literature / Cistadenoma mucinoso apendicular gigante tratado por laparoscopia: relato de caso e revisão de literatura

ABSTRACT Appendiceal mucinous cystadenoma is a rare entity, which causes appendicular mucocele. It is more frequent in women over 50 years old. In half of the cases it is asymptomatic. Tomography of the abdomen is the gold standard in its preoperative diagnosis. The treatment is surgical, with good prognosis, the complete resection evolves without appendicular rupture and extravasation. We report a case of a 64-year-old man with appendiceal mucinous cystadenoma. A laparoscopic right hemicolectomy was performed. This therapy that can be safely used to treat appendiceal mucocele, as long as it is cautious.

RESUMO O cistadenoma mucinoso apendicular é entidade rara que causa mucocele apendicular, sendo mais frequente em mulheres acima dos 50 anos. Em metade dos casos, o cistadenoma mucinoso apendicular é assintomático. A tomografia do abdome é o padrão-ouro para um diagnóstico pré-operatório. O tratamento é cirúrgico e tem bom prognóstico; a ressecção completa evolui sem ruptura apendicular e sem extravasamento. Relatamos um caso de paciente homem de 64 anos com cistadenoma mucinoso apendicular. Foi realizada hemicolectomia laparoscópica direita. Esse é um procedimento que pode ser usado com segurança no tratamento de mucocele apendicular, desde que seja executado com cautela.

Neoplasia mucinosa gigante de páncreas: presentación de caso clínico y revisión de la literatura / Giant mucinous neoplasm of the pancreas: presentation of a case and review of the literature

Female, 34 year-old patient with diffuse abdominal pain, bloating, and early satiety. Results from a study performed show a complex cystic mass in the pancreas with positive pancreatic mucinous cystadenoma histology. Cystadenoma/Mucinous cystadenocarcinoma represent 10 percent of cystic tumors within the pancreas, which occurr almost exclusively in women. Grossly they are uni or multilocular and contain dense, rich in mucinous matter. Histologically they exhibit columnar epithelium with presence of ovarian stroma. They vary in size, with a 10 cm average. Given their malignancy risk, surgical resection is recommended. Ultrasonography (US) helps to identify these lesions, however fails to provide a definitive diagnosis. Computed tomography (CT) and Magnetic resonance imaging (MRI) have higher accuracy, helping to identify aggressive nature in 75-90 percent. MRI is the test of choice for its resolution capacity. Endoscopic US allows for better characterization and for conducting fine-needle aspiration (FNA) for biochemical and cytological analysis of the liquid, achieving a significant increase in the diagnosis specificity. The differential diagnosis must be performed with other cystic lesions, considering that the final diagnosis is histological. The American College of Radiology suggests the use of surgical resection of mucinous neoplasms of the pancreas, Intraductal papillary or mucinous tumors the IPMT main duct and branch (if symptomatic, growing progressively, > 3 cm, have solid component and/or main duct dilation associated)...

Paciente 34 años, femenino, con dolor abdominal difuso, meteorismo y saciedad precoz. Se realiza estudio con imágenes que demuestran una gran masa quística compleja del páncreas cuya histología fue positiva para cistoadenoma mucinoso de páncreas. Los cistoadenomas/cistoadenocarcinomas mucinosos representan el 10 por ciento de los tumores quísticos del páncreas y se presentan casi exclusivamente en mujeres. Macroscópicamente son uni o multicavitados y contienen material denso, rico en mucina. Histológicamente presentan epitelio cilíndrico con presencia de estroma ovárico. Su tamaño es variable con promedio de 10 cm. Dado el riesgo de malignización se prefiere resección quirúrgica. La ultrasonografía (US) identifica estas lesiones, sin embargo, no llega a diagnóstico definitivo. La tomografía computarizada (TC) y la resonancia magnética (RM), presentan una precisión mayor, ayudando a diferenciar el carácter agresivo en 75-90 por ciento. La RM es el examen de elección por su capacidad de resolución. La US endoscópica permite una mejor caracterización y la realización de punción-aspiración con aguja fina (PAAF) para el estudio bioquímico y citológico del líquido, consiguiendo aumentar considerablemente la especificidad del diagnóstico. El diagnóstico diferencial debe realizarse con otras lesiones quísticas, teniendo en cuenta que el diagnóstico definitivo es histológico. El American College of Radiology sugiere resección quirúrgica de las neoplasias mucinosas de páncreas, y de los tumores papilares mucinosos intraductales (TPMI) de ducto principal y de rama (si son sintomáticos, crecen en forma progresiva, presentan un diámetro > 3 cm, presentan componente sólido y/o se asocian dilatación del conducto principal)...

large unilocular mucinous cystadenoma in third trimester of pregnancy

Ovarian malignancy is the second most common gynaecological malignancy diagnosed during pregnancy. A grand multipara, aged 30 years presented with gestational amenorrhea with abdominal discomfort and breathlessness for last 15 days. Sonographic examination demonstrated a huge, unilocular ovarian cyst and an alive fetus of about 30 weeks gestation. Intraoperative findings were huge left ovarian cyst [42x40x20 cm] with straw coloured mucinous fluid. Left salpingo-oophorectomy was performed followed by peritoneal washings and omental biopsy. Histopathology revealed mucinous cystadenomas with inflammatory changes in omentum and no malignant cells in peritoneal washings. She delivered vaginally a female baby of 3.5 kg at 38 weeks with good Apgar score

Cistoadenoma mucinoso de pâncreas durante a gravidez / Mucinous cystadenoma of the pancreas developing during pregnancy

Cistoadenoma mucinoso de pâncreas durante a gravidez é extremamente raro e relatado somente cinco vezes na literatura. Nós descrevemos caso de uma mulher de 26 anos, na sua segunda gestação, com um acentuado aumento de volume intra-abdominal por massa cística, cuja gestação foi interrompida durante a trigésima primeira semana por severa pré-eclâmpsia. A cirurgia de retirada do cisto ocorreu 26 dias após o parto, com enucleação total da lesão. O exame anatomopatológico evidenciou o cisto como cistoadenoma mucinoso, que mediu 23x20x15 cm e pesou 9070 g. Até onde é do nosso conhecimento, é um dos maiores cistoadenomas mucinosos pancreáticos relatados durante a gestação

Mucinous cystadenoma of the pancreas during pregnancy is an extremely rare condition and was reported only five times in the literature. We report the case of a 26-year-old female who, in her second pregnancy, had a sharp intra-abdominal volume increase due to cystic mass, whose pregnancy was interrupted at the thirty-first week of gestation because of severe pre-eclampsia. The surgery to remove the cyst was performed 26 days after delivery, with total enucleation of the lesion. The anatomico-pathological examination showed a mucinous cystadenoma that measured 23x20x15 cm and weighed 9,070 g. To the best of our knowledge this is one of the largest pancreatic mucinous cystadenomas reported during pregnancy

Cistoadenoma mucinoso de apéndice: informe de un caso / Mucinous cystadenoma of the appendix: case report

Introducción: La patología apendicular representa más de 50% de la actividad quirúrgica general, y dentro de ésta hay que considerar las raras, entre las que se incluyen los mucoceles, cuya incidencia es mayor en mujeres (4/1) y en pacientes con edad superior a 50 años. El objetivo de este trabajo es informar un caso de cistoadenoma mucinoso de apéndice. Caso clínico: Hombre de 34 años de edad con diagnóstico a los nueve años de edad de artritis reumatoide juvenil; con prótesis bilateral de cadera a los 22 años. Presenta amiloidosis renal desde 1998 y se encuentra en tratamiento con esteroides hasta el momento de este informe. El cuadro clínico se inició un año atrás, cuando en una tomografía axial computarizada de abdomen de control se encontró tumoración en la fosa iliaca derecha, confirmada por colonoscopia en ciego. Por diagnóstico presuntivo de lipoma fue programado para cirugía; en el transoperatorio se encontró gran tumoración de pronóstico reservado, por lo que se decidió hemicolectomía derecha. El informe de patología de la pieza quirúrgica fue cistoadenoma mucinoso. Conclusiones: La sintomatología del cistoadenoma es inespecífica. Los métodos de diagnóstico incluyen rayos X, ultrasonido, tomografía axial computarizada y colonoscopia. El cistoadenoma es la forma más común de los mucoceles apendiculares y el protocolo de diagnóstico es igual que para patología de apéndice cecal. El tratamiento de elección es la cirugía y depende del tamaño del mucocele. El pronóstico es bueno con el tratamiento adecuado.

BACKGROUND: Pathology of the appendix represents >50% of surgical activity. It is necessary to consider rare pathologies such as mucoceles. These have a high frequency in females with a F/M ratio of 4:1, as well as in patients >50 years of age. The objective of this study is to report on a case of mucinous cystadenoma of the appendix. CLINICAL CASE: We present the case of a 34-year-old male with a history of juvenile rheumatoid arthritis diagnosed at 9 years of age. At 22 years of age, the patient underwent bilateral hip arthroplasty. Since 1998, the patient has presented with renal amyloidosis and has been under steroid treatment to date. The current problem evolved 1 year ago during control studies for his disease. Computed tomography (CT) of the abdomen was done, demonstrating a tumor in the right iliac fossa. Colonoscopy confirmed a tumor with smooth edges located in the cecum. Surgery was recommended with a diagnosis of lipoma of the cecum. During surgery, a large tumor was found, indicating a poor prognosis. Right hemicolectomy was done. Surgical specimen was sent to pathology with a report of mucinous cystadenoma. CONCLUSIONS: The group of symptoms of cystadenoma is nonspecific. Diagnostic methods include X-rays, ultrasound, CT and colonoscopy. Mucinous cystadenoma is the most common form of mucocele of the appendix. Diagnostic protocols are the same for benign appendix pathology. Treatment is surgical and type of surgery depends on the size of the mucocele. Mucoceles of the appendix are pathologies with a favorable prognosis when appropriate treatment is done.

Primary retroperitoneal mucinous cystadenoma

Primary mucinous neoplasms of the retroperitoneum, including mucinous cystadenocarcinomas, mucinous borderline tumors, and mucinous cystadenomas are uncommon tumors found exclusively in women. Since the retroperitoneum does not contain mucinous epithelium, the origin, and histogenesis of these tumors remain unclear. It is speculated that these tumors can arise from teratomas, supernumerary ovaries, or mucinous metaplasia of the retroperitoneal mesothelium. We describe a case of a primary mucinous cystadenoma of the retroperitoneum in a 44 year-old female that presented as a palpable abdominal mass. There was no evidence of recurrence 16 months after complete laparoscopic excision of the tumor. The morphology and immunohistochemical analysis in this case support the hypothesis that mucinous metaplasia of the retroperitoneal mesothelium overlying a preceding inclusion cyst can give rise to retroperitoneal mucinous tumors

Neoplasias quísticas pancreáticas / Cystic neoplasms of the pancreas

BACKGROUND: Cystic neoplasms of the pancreas represent between 5% and 10% of all pancreatic tumors. Due to their malignant potential, surgery is indicated. Because of the uncommon nature of this pathology, the aim of this study was to present a series of six patients who were operated on for a cystic tumor and to analyze the morphological characteristics, surgical experience and follow-up. METHODS: This was a retrospective study. Clinical files were studied, as well as surgical and pathological registries from patients diagnosed with cystic neoplasms of the pancreas. Five females and one male were included with a mean age of 54 +/- 15 years. The main symptom was abdominal pain. RESULTS: Clinical course and CT scan results were the criteria for suspecting the diagnosis. In 4/6 patients, the tumor was located within the body and tail of the pancreas. The most frequent intervention was distal pancreatectomy. There was no mediate operative mortality in a 30-day period. Histopathological diagnoses were three serum cystadenomas, two mucinous cystadenomas and one cystadenocarcinoma. After a 4-year follow-up, no tumor recurrences have been reported in the surviving patients. CONCLUSIONS: The natural history of cystic neoplasms of the pancreas is generally benign, but complications may occur by direct invasion of neighboring structures. Clinical course, tomography, pancreatography and punction are fundamental for its treatment.

Tumor borderline mucinoso ovárico gigante con microinvasión / Borderline mucinous ovarian tumor with microinvasion

Se presenta caso clínico de tumor borderline mucinoso gigante de ovario derecho operado. Se describe una breve revisión de la literatura, incluyendo los conceptos de microinvasión, arquitectura micropapilar, implantes, así como algunas características de su patogenia y tratamiento.

Tumor de ovario borderline mucinoso: a propósito de un caso clínico / Mucinous borderline ovarian tumor: a clinical case

Los tumores de ovario bordeline son neoplasias de bajo grado de malignidad, representan el 10 a 15 por ciento del total de los tumores de ovario. Suelen diagnosticarse en estadios tempranos, tienen baja probabilidad de recurrencia y buen pronóstico. Se presenta un caso clínico de una paciente con diagnóstico de tumor mucinoso bordeline a partir del cual se hace una revisión destacando puntos relevantes de la patología, diagnóstico y diferentes modalidades de terapéutica.

Cistadenoma mucinoso primário de trompa de Fallopio: apresentaçäo de caso / Mucinous cystadenoma of Fallopian tube: case report

As lesöes mucinosas da trompa de Fallopio compreendem uma grande quantidade de processos que incluem metaplasia mucinosa, cistadenoma mucinoso, tumores mucinosos com bixo potencial de malignidade (tumores mucinosos "boderline") e adenocarcinoma mucinoso. As neoplasias mucinosas benignas primárias da trompa de Fallopio säo estremamente raras. Há poucos casos descritos na literatura mundial, e o grande interesse gira em torno da possível associaçäo destas, com neoplasias múltiplas do trato genital, lesöes mucinosas do apêndice e síndrome de Peutz-Jeghers. O presente relato descreve a ocorrência de um cistadenoma mucinoso primário de trompa de Fallopio em uma paciente de 50 anos, menopausada há dois anos, cujo cisto foi identificado inicialmente como de ovário, por ecografia transvaginal, realizada para investigaçäo de um sangramento vaginal. A paciente foi submetida a laparotomia exploradora e teve o diagnóstico feito por exame transoperatório de congelaçäo, confirmado posteriormente por exame anatomopatológico convencional

Tumores quísticos del páncreas / Cystic tumors of the pancreas

Los tumores quísticos del páncreas son raros y constituyen sólo 10 a 15 por ciento de las lesiones quísticas del páncreas y 1 por ciento de las neoplasias malignas. Pueden ser benignas o malignos y generalmente son circunscritos y bien localizados. Su identificación, el diagnóstico diferencial y el tratamiento son difíciles y un ejemplo de ello es que hasta una tercera parte pueden confundirse con seudoquistes del páncreas. Los más importantes son los cistadenomas microquísticos serosos, los cistadenomas mucinosos, los cistadenocarcinomas mucinosos, los adenocarcinomas productos de mucina y los adenocarcinomas asociados a seudoquiste o a quiste simple. Los estudios de mayor utilidad para el diagnóstico son el ultrasonido, la tomografía axial computada, la colangiopancreatografía retrógrada endoscópica, la punción guiada del quiste con estudio del líquido y la biopsia. La elección del tipo de tratamiento depende de la variedad del tumor de que trate, de su localización y extensión, del riesgo quirúrgico, de la experiencia del cirujano y de los recursos institucionales. En general, el pronóstico de estos tumores es más favorable que el del adenocarcinoma ductal, aun cuando sean malignos

Cistoadenoma mucinoso; informe de un caso y revisión de la literatura / Mucinous cystoadenoma; case report and review

Informamos el caso de una paciente con una tumoración abdominal, remitida al hospital de área "Juan Manuel Galvez" (Gracias Lempira) con el diagnóstico de embarazo. En base a los estudios clínicos y laboratoriales se postuló el diagnóstico de "tumor gigante de ovario(T.G.O.)". La laparotomía exploradora demostró un tumor gigante de ovario izquierdi que por estudios histopatológicos resultó ser un cistoadenoma mucinoso benigno de ovario. Lo avanzado de la enfermedad en ésta paciente deja al descubierto nuestra pobre cobertura de salud asi como el bajo nivel sociocultural de la población. Se enfatiza que el diagnóstico diferencial de éste tumor no es el embarazo sino otras neoplasias abdominales y pélvicas.

Retirada de um cisto mucinoso por vídeo-laparoscopia / Extraction of a mucinous extraction by videolaparoscopy

Os autores relatam a retirada de um cisto mucinoso através de videolaparoscopia e enfatizam: cicatriz mínima, menor permanência hospitalar e rápido retorno às atividades habituais. Devem ser obedecidos vários critérios importantes, tais como: afastar-se possível doença maligna e evitar-se extravasamento de conteúdo mucoso com conseqüente formaçao de pseudomixoma peritoneal.