Neoplasia quística mucinosa hepática, reporte de un caso y análisis de la patología / Hepatic mucinous cystic neoplasm, case report and pathology analysis / Neoplasia cística mucinosa hepática, relatório de um caso e análise da patologia

Desde los primeros reportes en la bibliografía, la nomenclatura de las lesiones quísticas hepatobiliares se ha ido modificando, habiéndose descripto dos tipos de lesiones: las serosas y las mucinosas. En 2010 la Organización Mundial de la Salud estableció una nueva clasificación donde los términos cistoadenomas y cistoadenocarcinomas hepatobiliares son reemplazados por entidades más específicas como la neoplasia mucinosa quística y los tumores quísticos intraductales (neoplasia papilar intraductal, neoplasma tubulopapilar intraductal y neoplasma oncocitico papilar). En cuanto a la neoplasia mucinosa quística, la presencia de estroma ovárico le confiere características distintivas en lo patológico y biológico, siendo esto un requisito en la clasificación de la OMS. Esta característica lo diferencia de los hamartomas biliares, los quistes congénitos y la enfermedad de Caroli. Dichas neoplasias son infrecuentes, con una incidencia menor al 5% de las lesiones quísticas hepáticas y ocurren casi exclusivamente en mujeres, frecuentemente perimenopáusicas. Su potencial de malignización ha sido descrito, siendo éste la indicación de tratamiento quirúrgico resectivo. Presentamos el caso clínico de una paciente portadora de una neoplasia quística mucinosa hepática, catalogada como cistoadenoma hepático según la antigua clasificación.

Since the early reports in the literature, the nomenclature of hepatobiliary cystic lesions has been modified, with two types of lesions being described: serous and mucinous. In 2010, the World Health Organization established a new classification in which the terms hepatobiliary cystadenomas and cystadenocarcinomas were replaced by more specific entities such as mucinous cystic neoplasms and intraductal cystic tumors (intraductal papillary neoplasm, intraductal tubulopapillary neoplasm, and intraductal oncocytic papillary neoplasm). Regarding mucinous cystic neoplasms, the presence of ovarian stroma confers distinctive pathological and biological characteristics, which is a requirement in the WHO classification. This characteristic differentiates it from biliary hamartomas, congenital cysts, and Caroli's disease. Such neoplasms are rare, with an incidence of less than 5% of hepatic cystic lesions, and occur almost exclusively in women, often perimenopausal. Their potential for malignancy has been described, and this is the indication for surgical resection treatment. We present a clinical case of a patient with a mucinous cystic hepatic neoplasm, classified as a hepatic cystadenoma according to the old classification.

Desde os primeiros relatos na literatura, a nomenclatura das lesões císticas hepatobiliares tem sido modificada, sendo descritos dois tipos de lesões,asserosas e as mucinosas. Em 2010, a Organização Mundial da Saúdeestabeleceuuma nova classificação, naqual os termos cistoadenomas e cistoadenocarcinomas hepatobiliares foramsubstituídos por entidades mais específicas, como a neoplasia mucinosa cística e os tumores císticos intraductais (neoplasia papilar intraductal, neoplasma tubulopapilar intraductal e neoplasma oncocítico papilar). Em relação à neoplasia mucinosa cística, a presença de estroma ovarianoconfere características distintas do ponto de vista patológico e biológico, sendoesseum requisito naclassificação da OMS. Essa característica a diferencia dos hamartomas biliares, cistoscongênitos e doença de Caroli. Essas neoplasias são raras, comumaincidência menor que 5% das lesões císticas hepáticas, e ocorremquase exclusivamente em mulheres, frequentementeperimenopáusicas. Seu potencial de malignizaçãotem sido descrito, sendoesta a indicação para tratamentocirúrgicoressectivo. Apresentamos o caso clínico de uma paciente portadora de uma neoplasia cística mucinosa hepática, classificada como cistoadenoma hepático de acordocom a antigaclassificação.

Neoplasia cística mucinosa do pâncreas / Mucinous cystic neoplasia of the pancreas

Introdução: Neoplasia cística mucinosa é tumor mucinoso benigno (cistoadenoma mucinoso) ou maligno (cistoadenocarcinoma mucinoso), que não se comunica com os ductos pancreáticos. Objetivo: Apresentar revisão da literatura sobre o tema. Método: Ênfase nas diretrizes das principais sociedades médicas mundiais na orientação do diagnóstico, tratamento e a vigilância da neoplasia cística mucinosa. Resultado: A quase totalidade dessas neoplasias ocorre no gênero feminino de 40-50 anos de idade. Como raras exceções, esta neoplasia é encontrada na cauda/corpo do pâncreas. Para estabelecer o diagnóstico é necessário a presença de estroma similar ao do ovário na parede do cisto no exame patológico. Exames de imagem de alta resolução, como tomografia, ressonância magnética e ecoendoscopia apresentam elevada precisão para identificar esta neoplasia. O tratamento cirúrgico consiste na pancreatectomia distal com linfadenectomia e esplenectomia. A via laparoscópica ou robótica é preferida para tumores <5-7 cm. Devido a possibilidade de rotura do tumor e disseminação da neoplasia, as lesões >5-7 cm devem ser submetidos à ressecção laparotômica. Conclusão: Não existe uniformidade internacional na conduta terapêutica. O tratamento cirúrgico deve ser indicado para todos os pacientes com condições cirúrgicas e que apresentam neoplasia ≥3-4 cm, dependendo do consenso.

Introduction: Mucinous cystic neoplasia is a benign mucinous tumor (mucinous cystadenoma) or malignant (mucinous cystadenocarcinoma), which does not communicate with the pancreatic ducts. Objective: To present a review of the literature on the topic. Method: Emphasis on the guidelines of the main global medical societies in guiding the diagnosis, treatment and surveillance of mucinous cystic neoplasia. Result: Almost all of these neoplasms occur in females aged 40-50 years. As a rare exception, this neoplasm is found in the tail/body of the pancreas. To establish the diagnosis, the presence of stroma similar to that of the ovary in the cyst wall is necessary on pathological examination. High-resolution imaging exams, such as tomography, magnetic resonance imaging and endoscopic ultrasound, are highly accurate in identifying this neoplasm. Surgical treatment consists of distal pancreatectomy with lymphadenectomy and splenectomy. The laparoscopic or robotic route is preferred for tumors <5-7 cm. Due to the possibility of tumor rupture and dissemination of the neoplasm, lesions >5-7 cm must undergo laparotomic resection. Conclusion: There is no international uniformity in therapeutic conduct. Surgical treatment should be indicated for all patients with surgical conditions and who have neoplasia ≥3-4 cm, depending on the consensus.

Mucinous cystic neoplasm of the liver

A 55-year-old woman was investigated for occasional epigastric pain and weight loss. T2-weighted abdominal magnetic resonance imaging and magnetic resonance cholangiography revealed a multilocular cyst with multiple septa and a solid component in the liver, measuring 6.1 × 4.8 × 6.5 cm. Given the patient's symptoms and malignant potential, a laparoscopic segmentectomy with partial resection of segments IV B and V was performed to completely remove the cystic lesion, associated with cholecystectomy. Histopathology demonstrated a cyst lined by columnar mucinous epithelium. Therefore, the diagnosis was mucinous cystic neoplasm of the liver. This article presents a case report and literature review of this entity.

Características de la neoplasia quística mucinosa del páncreas en pacientes atendidos en un Hospital Nacional del Perú / Characteristics of pancreatic mucinous cystic neoplasm in patients treated at a National Hospital in Peru

RESUMEN Con el objetivo de analizar las características clínico, patológicas y quirúrgicas de la Neoplasia Quística Mucinosa de páncreas (NQM), se realizó un análisis de los pacientes del servicio de Cirugía de Páncreas, Bazo y Retroperitoneo del Hospital Nacional Guillermo Almenara Irigoyen en Lima, Perú desde enero del 2009 hasta octubre del 2018. La presencia del estroma ovárico se usó como criterio diagnóstico de NQM. De diez pacientes con edad promedio de 47,8 años, nueve fueron mujeres, las lesiones estuvieron localizadas en el páncreas distal, el tamaño tumoral promedio fue de 88,6 mm. En todos los pacientes se realizó una pancreatectomía distal siendo tres laparoscópicas, no hubo reoperaciones ni fallecidos, dos pacientes tuvieron carcinoma invasor asociado. En conclusión, la presentación de NQM es mayor en mujeres de edad media siendo la localización en el páncreas distal y el porcentaje de malignidad bajo. La cirugía laparoscópica es una alternativa de manejo.

ABSTRACT In order to analyze the clinical, pathological, and surgical characteristics of pancreatic mucinous cystic neoplasm (MCN), an analysis of the patients from the Pancreas, Spleen, and Retroperitoneal Surgery Service of the Guillermo Almenara Irigoyen National Hospital in Lima, Peru, was performed from January 2009 to October 2018. The presence of ovarian stroma was used as a diagnostic criterion for MCN. From ten patients with an average age of 47.8 years, nine were women; the lesions were located in the distal pancreas, and the average tumor size was 88.6 mm. All patients underwent a distal pancreatectomy, three of which were laparoscopic; there were no reoperations or deaths; two patients had associated invasive carcinoma. In conclusion, the frequency of MCN is higher in middle-aged women, being the location in the distal pancreas and the percentage of malignancy is low. Laparoscopic surgery is a disease management option.

Neoplasias quísticas pancreáticas: enfoque diagnóstico / Diagnostic Approach to Cystic Pancreatic Neoplasms

Resumen Los quistes pancreáticos comprenden una amplia variedad de lesiones cada vez más frecuentemente diagnosticadas debido tanto al empleo creciente de técnicas de imagen como al envejecimiento de la población. Entre ellas, las neoplasias quísticas mucinosas son especialmente relevantes por su potencial de malignización. Aunque la ecografía abdominal, la tomografía axial computarizada y la resonancia magnética suelen ser las pruebas diagnósticas de imagen iniciales, muchas veces los hallazgos morfológicos no son suficientes para su diferenciación. La ecoendoscopia se ha convertido en la mejor prueba para su caracterización ya que permite realizar estudio morfológico y también del líquido obtenido mediante punción, aunque su precisión diagnóstica para la detección de quistes de estirpe mucinosa y de malignidad sigue siendo baja. La importancia de la adecuada caracterización radica tanto en la detección precoz de las lesiones preneoplásicas y malignas como en evitar cirugías innecesarias. La indicación de ecoendoscopia, de tratamiento quirúrgico y de seguimiento varía entre las distintas guías de práctica clínica estando actualmente en duda especialmente el tratamiento y seguimiento de las neoplasias quísticas mucinosas papilares intraductales de rama lateral por el menor riesgo de degeneración y su asociación con el cáncer de páncreas.

Abstract Cystic pancreatic lesions comprise a wide variety of lesions that are being increasingly diagnosed due to the more frequent use of imaging techniques and the aging of the population. Among these lesions, mucinous cystic neoplasms are especially relevant because of their malignant potential. Although abdominal ultrasound, computerized tomography and magnetic resonance imaging are usually the initial diagnostic imaging tests, morphological findings are often not enough for their differentiation. Endoscopic ultrasound has become the best test for their characterization because it allows morphological study and fluid analysis obtained by puncture of the lesion, although its diagnostic accuracy for the detection of mucinous and malignant cysts remains low. The importance of proper characterization is the early detection of preneoplastic as well as malignant lesions and to avoid unnecessary surgery. Clinical practice guidelines differ about the indications for endoscopic ultrasound, surgical treatment and follow-up of these lesions. Questions specially remains in the management of side-branch intraductal papillary neoplasm because of their lower risk of degeneration and their association with pancreatic cancer.

Coexisting Mucinous Cystic Neoplasm of the Pancreas and Type 1 Autoimmune Pancreatitis

Type 1 autoimmune pancreatitis (AIP1) is an IgG4-related systemic disease that mimics tumors. We report a rare case of AIP1 accompanied by mucinous cystic neoplasm (MCN). A pancreatic lesion was incidentally detected in a woman in her 60s. After 6 years of follow-up, the lesion abruptly increased in size. Computed tomography showed a 3.5 cm unilocular cyst in the tail of the pancreas and distal pancreatectomy was performed. On microscopic examination, the cyst was lined by mucinous and non-mucinous epithelial cells with mild cytologic atypia. The surrounding stroma comprised ovarian-type spindle cells with progesterone receptor positivity. The pericystic pancreas exhibited multifocal lymphoid follicles, lymphoplasmacytic infiltrations, obliterative phlebitis, and storiform fibrosis. IgG4-positive plasma cell infiltration (215 cells high-power field) and the IgG4/IgG ratio (57%) were increased. Cases of MCN coexisting with AIP1 are extremely rare; only two such cases have been reported in the English-language literature. This third case featured low-grade MCN with AIP1.

Observation or surgical resection: treatment strategies of pancreatic cystic tumor / 中华消化外科杂志

With the popularization of the aging population and imaging examination,detection rate of the pancreatic cystic neoplasm are increasing in recent years,which commonly includes intraductal papillary mucinous neoplasm (IPMN),mucinous cystic neoplasm (MCN),serous cystic neoplasm (SCN) and solid pesudopapillary tumor (SPT).The differential diagnosis is essential to decide surgical resection or observation.The diagnosis of pancreatic cystic tumor relies primarily on imaging examination.For patients who need to long-term follow-up,the MRI should be recommended.The preoperative precision diagnosis that is performed by traditional imaging,tumor markers,endoscopic ultrasonography and contribute to make the individualized therapy plan.The follow-up strategy is optimal for majority of patients with SCN.According to patients' condition,the surgical resection or observation will be selected for patients with IPMN or MCN after finding malignant transformation-related high risk factors.SPT has been classified as the malignant tumor,and if patients are suspected to have SPT by imaging examination,surgical resection should be recommended.

Mucinous Cystic Neoplasm of the Pancreas Presenting with Acute Pancreatitis, Initially Misdiagnosed as a Pseudocyst / 대한내과학회지

Pancreatic cystic lesions include retention cysts (congenital cysts), pseudocysts, and cystic neoplasms. Pancreatic cystic neoplasms have recently been diagnosed more commonly, possibly due to advances in imaging and widespread screening programs. Cystic neoplasms of the pancreas account for 10-20% of pancreatic tumors. Mucinous cystic neoplasms (MCN) and intraductal papillary mucinous neoplasms are regarded as premalignant lesions, whereas serous cystadenoma is not. In the clinical setting of acute pancreatitis, pancreatic cystic lesions are usually diagnosed as pseudocysts. However, cystic neoplasms of the pancreas should be considered in the differential diagnosis of pancreatic cysts, even in patients with a history of pancreatitis. In the Korean literature, MCN combined with acute pancreatitis has rarely been reported. Here, we report a case of MCN presenting with acute pancreatitis in a 22-year-old female, which was initially misdiagnosed as pancreatic pseudocyst.

Mucinous cystadenocarcinoma arising in an ectopic kidney simulating a retroperitoneal dermoid cyst: a rare tumour presenting as a diagnostic dilemma

Primary mucinous cystic neoplasms are rare tumours of the kidney, with a very few case reports in the literature. They arise from metaplasia of renal pelvic urothelium. We describe here a 45-year-old male who presented with pain in the abdomen and a lump in the left iliac fossa for two months. Ultrasound and CT scan showed a large, complex, heterogenous mass in the central abdomen and left iliac fossa, suggesting the possibility of dermoid cyst. Excision of the mass showed an enlarged multicystic kidney fi lled with mucin, destruction of renal parenchyma and a small viable area of grey white tumour. Histopathology revealed a peripherally located mucinous cystadenocarcinoma arising in the background of chronic pyelonephritis and mucinous metaplasia. We report this case for the rarity of the lesion and the associated clinical and radiological diagnostic dilemma.

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

A Case of Mucinous Cystic Neoplasm of the Pancreas Communicating with the Main Pancreatic Duct / 대한소화기내시경학회지

Diagnoses of cystic lesions in the pancreas are increasing in clinical practice because of the wider use of imaging studies. The selection of appropriate treatment depends on the ability to distinguish between benign and malignant cysts. However, cystic pancreatic neoplasms are sometimes misdiagnosed as pseudocysts and treated improperly. We experienced a case of mucinous cystic neoplasm of the pancreas misdiagnosed as a pseudocyst, which had a communication between the cyst and the main pancreatic duct.

Biological Markers for Making the Differential Diagnosis between Intraductal Papillary Mucinous Tumor and Mucinous Cystic Tumor of the Pancreas

PURPOSE: Although intraductal papillary mucinous tumor (IPMT) and mucionus cystic tumor (MCT) share many common features, they are 2 clinically different disease entities. The aims of this study were to compare the clinicopathological characteristics of IPMT and MCT and to find molecular markers for making the differential diagnosis between IPMT and MCT METHODS: Forty-one patients with IPMT and 29 MCT patients who all underwent resection between 1994 and 2003 were enrolled in this study. After one gastrointestinal pathologist reviewed the pathological slides, we compared the clinico-pathological features of the two diseases. Immunohistochemical staining with using 17 biological markers was performed to find useful molecular markers for making the differential diagnosis. RESULTS: Besides the clinical features like gender, the tumor size and location, the patient's age and the radiological images, we found differences of the expressions of PR(p<0.001), ER(p<0.001), MUC2(p=0.038) and MUC5A(p=0.001) between IPMT and MCT. CONCLUSION: PR, ER, MUC2 and MUC5A can be useful in making the final differential diagnosis between IPMT and MCT. Considering the different gene expressions, further studies are needed to clarify the different pathogenesis of these two diseases entities.

Clinicopathologic Review of 41 Cases of Pancreatic Mucinous Cystic Neoplasms / 대한소화기학회지

BACKGROUND/AIMS: Intraductal papillary mucinous neoplasms (IPMN) and mucinous cystic neoplasms are included in mucin-producing pancreatic tumors. The reports about IPMN are not uncommon but those about the mucinous cystic neoplasms are relatively few. The aims of this study were to define the natural history of resected mucinous cystic neoplasms of the pancreas and to identify the findings which suggest malignancy. METHODS: The authors retrospectively evaluated the clinical outcomes of 41 patients with mucinous cystic neoplasms who were surgically resected at Asan Medical Center between 1995 and 2004. RESULTS: Women (n=33) were more frequently affected than men (n=8). Thirty three patients (80.6%) had adenoma, 1 (2.4%) borderline malignancy, 1 (2.4%) carcinoma in situ, and 6 (14.6%) invasive mucinous cystadenocarcinoma. The most frequent symptom was abdominal pain (39%). About half of the enrolled patients were asymptomatic. Unilocular type (79%) was more frequent than the multilocular type (21%) on gross morphology. The tumor size of invasive mucinous cystic neopolasms was larger than that of non-invasive mucinous cystic neoplalsms (p=0.01). Abdominal pain was more frequent in invasive mucinous cystic neoplasms (p=0.026). On gross morphology, mural nodules were detected in 4 of 6 patients with invasive mucinous cystic neoplasms. However, they were not detected in any patients with non-invasive mucinous cystic neoplasms. Recurrence developed in none of the 35 patients with non-invasive mucinous cystic neoplasms, however 2 of the 6 patients with invasive mucinous cystic neoplasms died within 5 years. CONCLUSIONS: Clinical predictors of invasive mucinous cystic neoplasms are suggested to be tumor size and abdominal pain. The prognosis of the non-invasive mucinous cystic neoplasms is excellent when curative resection is performed.

Precursor Lesions of Pancreatic Cancer

This review article describes morphological aspects, gene abnormalities, and mucin expression profiles in precursor lesions such as pancreatic intraepithelial neoplasia (PanIN), intraductal papillary mucinous neoplasm (IPMN), and mucinous cystic neoplasm (MCN) of the pancreas, as well as their relation to pancreatic ductal adenocarcinoma (PDAC). The gene abnormalities in precursors of PDAC are summarized as follows: (1) KRAS mutation and p16/CDKN2A inactivation are early events whose frequencies increase with the dysplasia grade in both PanIN and IPMN; (2) TP53 mutation and SMAD4/DPC4 inactivation are late events observed in PanIN3 or carcinomatous change of IPMN in both PanIN and IPMN, although the frequency of the TP53 mutation is lower in IPMN than in PDAC; and (3) also in MCN, KRAS mutation is an early event whose frequency increases with the dysplasia grade, whereas TP53 mutation and SMAD4/DPC4 inactivation are evident only in the carcinoma. The mucin expression profiles in precursors of PDAC are summarized as follows: (1) MUC1 expression increases with the PanIN grade, and is high in PDAC; (2) the expression pattern of MUC2 differs markedly between the major subtypes of IPMN with different malignancy potentials (i.e., IPMN-intestinal type with MUC2+ expression and IPMN-gastric type with MUC2- expression); (3) MUC2 is not expressed in any grade of PanINs, which is useful for differentiating PanIN from intestinal-type IPMN; (4) de novo expression of MUC4, which appears to increase with the dysplasia grade; and (5) high de novo expression of MUC5AC in all grades of PanINs, all types of IPMN, MCN, and PDAC.

Clinical Analysis for Mucinous Cystic Neoplasms of the Pancreas

PURPOSE: Mucinous cystic neoplasms are at best potentially malignant and present a spectrum of neoplasia ranging from benign neoplasms to invasive carcinoma. The purpose of this study was to investigate the clinicopathologic characteristics relevant to the specific diagnosis of mucinous cystic neoplasms of the pancreas. METHODS: We retrospectively reviewe the clinical features and histopathologic findings of 34 cases who underwent op-erations with mucinous cystic neoplasms of the pancreas from October 1994 to March 2005. RESULTS: There were 25 mucinous cystadenoma (73.5%), 6 mucinous cystic tumor of borderline malignant potential (17.6%), 1 in situ mucinous cystadenocarcinoma (2.9%), 2 invasive mucinous cystadenocarcinoma (5.9%). Their ages ranged from 23 to 76 years with a mean of 44.2. The 88.2% of the patients were women. The 94.1 % of the tumors were located in the body or tail of the pancreas. The tumor size ranged from 2 to 17 cm, especially the mean size of invasive mucinous cystadenocarcinoma was 13.5 cm. The accuracy of CT for diagnosing was 81.8% and that of US was 53.3%. After a follow-up duration from 5 to 103 months, all patients are still alive. However, recurrent disease developed in all of the 2 patients with invasion. CONCLUSION: Our results suggest that complete surgical resection is certainly the gold standard treatment for mucinous cystic neoplasms. A histologic diagnosis of invasive cysta-denocarcinoma portends a dismal prognosis, so close long term follow up is necessary due to tumor recurrence.

Fine Needle Aspiration Cytology of Mucinous Cystic Carcinoma of the Pancreas: A Case Report / 대한세포병리학회지

Mucious cystic neoplasm of pancreas is a cystic neoplasm composed of columnar, mucin-producing epithelium and is supported by ovarian-type stroma. The key to the cytologic evaluation of pancreatic cystic lesions is to recognize the cytologic components as being diagnostic of a mucin-producing cystic neoplasm, as all of these neoplasms need to be resected. We report the use of fine needle aspiration cytology in the diagnosis of an invasive mucinous cystic carcinoma confirmed by partial pancreatectomy. The cytologic specimen showed a abundant mucin background and sheets or papillae of neoplastic cells. There are mucin-containing columnar cells that show a variable degree of cytologic atypia.