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.

Mucinous cystadenoma of the liver with pathological-radiological correlation.

The paper presents a pathological-radiological correlation of the manifestation of mucosal cystadenoma with ovarian stroma of the liver with examination and correlation with the new stroma nomenclature and differential diagnostic dilemmas of radiologists and pathologists.

Case 300: Ruptured Mucinous Cystadenoma of the Pancreas.

History Part one of this case appeared 4 months previously and may contain larger images. A 40-year-old woman presented to the outpatient department of our hospital with a 2-year history of abdominal pain, which had worsened in the past few days. There were no other constitutional symptoms; in particular, there was no weight loss or loss of appetite. The serum amylase and serum lipase levels were always within normal limits, even during the current episode. There was no history of pancreatitis, alcohol abuse, drug use, or trauma. The patient was evaluated at an outside clinic 2 years ago and underwent imaging, including US and dedicated pancreatic CT, which revealed a complex multiseptated noncalcified pancreatic cystic lesion. After CT, the patient underwent endoscopic US, which showed the lesion was suspected to be an infected parasitic cyst; fine-needle aspiration and cytology were not attempted due to the risk of iatrogenic rupture. The patient was advised to undergo surgical resection but declined the procedure and was lost to follow-up for 2 years before her current visit to our facility. On clinical examination, there were no clinically important findings, aside from mild to moderate pain on deep palpation and some shifting dullness that was suggestive of ascites. The patient always remained hemodynamically stable. US-guided ascitic fluid analysis was performed, which revealed amylase levels of 869 IU/L (normal range, 4-234 IU/L). Considering prior CT and endoscopic US reports, the patient was further evaluated with a pancreatic MRI protocol for lesion characterization and to explain the new-onset abdominal pain.

The Coexistence of Two Different Epithelial Ovarian Tumors: A Rare Case.

BACKGROUND: Epithelial tumors are the most common subgroup and are seen in 60-70% of all ovarian tumors. Serous cystadenoma and mucinous cystadenoma are the most common benign epithelial tumors. Serous cystadenomas are ovarian tumors with the highest bilateral incidence. The coexistence of tumors with different histopathology in the ovaries is extremely rare and has only been reported in a few cases in the literature. We present a case of bilateral ovarian tumor that was diagnosed as serous and mucinous cystadenoma after laparoscopic surgery. CASE REPORT: A 45-year-old female patient was admitted to our center with swelling in the pelvic region and pain in the left lumbar region. US imaging showed a cystic lesion in the right adnexal area, 4x2 cm in size, well-circumscribed, containing a few thin septa, and a low echo fluid content. A cystic lesion with 6x4cm sized multilocular, well-circumscribed, slightly high echo fluid content was observed in the left adnexal area. On CT, a complex cystic lesion measuring 6x4cm was observed in the left adnexal area, pushing the left ureter laterally and causing the hydroureter. In addition, a 4x2 cm cystic lesion was observed in the right adnexal area and hydroureter was observed on the right side proximal to this lesion. Both lesions were removed by surgery. On histopathologic examination, the left-sided cystic lesion was diagnosed as mucinous cystadenoma, and the right-sided cystic lesion was diagnosed as serous cystadenoma. CONCLUSION: The coexistence of different ovarian tumor subtypes is rare. In this article, we presented a case in which serous and mucinous cystadenoma lesions were seen together for the fourth time in the literature, according to our knowledge.

Computed Tomography and Clinical Analysis of Ovarian Mucinous Tumors in Adolescent Patients.

STUDY OBJECTIVE: To investigate the clinical and computed tomography features of ovarian mucinous tumors in children and adolescents. DESIGN, SETTING, AND PARTICIPANTS: A retrospective analysis of clinical and preoperative computed tomography (CT) data was performed in 59 patients who were 20 years or younger with ovarian mucinous tumors confirmed by histopathology. Patients' age, medical history, symptoms, tumor marker levels, and CT imaging findings were recorded. INTERVENTIONS: None. MAIN OUTCOME MEASURES: Identification of the clinical and CT features of ovarian mucinous tumors in pediatric and adolescent patients. RESULTS: There were 41 mucinous cystadenomas in 39 cases, 12 borderline mucinous cystadenomas in 11 cases, and 9 mucinous cystadenocarcinomas in 9 patients. A total of 55 tumors were multilocular (55/62, 88.7%), including fewer than 10 loculations in 23 tumors, 10-20 loculations in 17 tumors, and over 20 loculations in 15 tumors. Eleven borderline mucinous cystadenomas were multilocular (11/12, 91.7%), with over 10 loculations in 7 tumors (7/12, 58.3%). Twelve tumors appeared as multilocular cystic-solid (12/62, 19.4%), and a case of mucinous cystadenocarcinoma was predominantly solid (1/62, 1.6%). Seventeen tumors showed honeycomb sign and stained glass appearance. Six mural nodules, with sizes ranging from 1.1 to 3.5 cm (average: 2.8 cm), were found in borderline mucinous cystadenoma and mucinous cystadenocarcinoma. CONCLUSIONS: The CT findings of ovarian mucinous tumors in children are characteristic. The preoperative CT is helpful in making differential diagnoses.

MRI is useful to suggest and exclude malignancy in mucinous cystic neoplasms of the pancreas.

OBJECTIVES: To evaluate the value of MRI in differentiating benign (b-MCN) and malignant (m-MCN) MCN. European guidelines suggest that certain mucinous cystic neoplasms (MCN) of the pancreas can be conservatively managed. METHODS: A retrospective single-center study of consecutive patients with resected MCN. MRIs were independently reviewed by two readers blinded to the pathological results. The authors compared b-MCN (i.e., mucinous-cystadenoma comprising high-grade dysplasia (HGD)) and m-MCN (i.e., cystadenocarcinoma). RESULTS: Sixty-three patients (62 women [98%]) with 63 MCN (6 m-MCN, 2 HGD) were included. m-MCN tumors had a tendency to be larger than b-MCN (median 86 [25-103] vs. 45 [17-130] mm, p = .055). The combination of signal heterogeneity on T2-weighted imaging, wall thickness ≥ 5 mm, the presence of mural nodules ≥ 9 mm, and enhancing septa had an area under the ROC curve of 0.97 (95% CI 0.91-1.00) for the diagnosis of m-MCN. A total of 24 (37%), 20 (32%), 10 (16%), 5 (8%), and 4 (6%) out of 63 MCNs showed 0, 1, 2, 3, and 4 of these features, respectively. The corresponding rate of m-MCN was 0%, 0%, 10%, 20%, and 100%, respectively, with a good-to-excellent inter-reader agreement. Patterns with a high NPV for m-MCN included an absence of enhancing septa or walls (NPV 97% and 100%, respectively), wall thickness < 3 mm (NPV 100%), and no mural nodules (NPV 100%). CONCLUSIONS: A combination of 4 imaging features suggests malignant MCN on MRI. On the other hand, visualization of a thin non-enhancing wall with no mural nodules suggests benign MCN. KEY POINTS: • A heterogenous signal on T2-weighted MRI, a ≥ 5-mm-thick wall, mural nodules ≥ 9 mm, and/or enhancing septa suggest malignant MCNs. • A thin non-enhancing wall with no mural nodules suggests benign MCNs. • MRI should be performed in the pre-therapeutic evaluation of MCN to help determine the therapeutic strategy in these patients.

Ovarian Cystadenomas: Growth Rate and Reliability of Imaging Measurements.

OBJECTIVES: To evaluate the growth rate of benign ovarian cystadenomas and the degree of variability in ultrasound measurements. METHODS: Two independent retrospective cohorts of women found to have benign cystadenomas at surgery were identified. To assess growth rate, ultrasounds on women in a community-based health system were reviewed and the growth rate was determined based on the maximum reported size dimension using a mixed effect model. To assess measurement variability, two radiologists independently measured presurgical adnexal imaging findings for women in a tertiary care referral setting. Interobserver, intra-observer, and intermodality (cine clip versus still images) variability in measurements was determined using correlation coefficients (CC) and Bland-Altman analysis, with the proportion of measurements varying by more than 1 cm calculated. RESULTS: For growth rate assessment, 405 women with 1412 ultrasound examinations were identified. The median growth rate was 0.65 cm/year with mucinous cystadenomas growing faster at 0.83 cm/year compared to 0.51 cm/year for serous cystadenomas (median test P < .0001). To evaluate measurement variability, 75 women were identified with 176 ultrasound studies. The within-subject standard deviations for ultrasound measurements were 0.74 cm for cine clip images and 0.41 cm for static images, with 11% of measurements overall differing by more than 1 cm. CONCLUSIONS: Cystadenomas grow on average 0.65 cm/year, which is similar in magnitude to the inherent error observed in measurement on ultrasound, suggesting that repeat ultrasound at intervals of longer than a year will often be needed to accurately assess growth if a cyst represents a benign cystadenoma.

Primary peritoneal mucinous cystadenocarcinoma mimicking possible recurrent ovarian mucinous cystadenoma: coincidental pathology or a spectrum of disease?

We present the case of a 32-year-old woman with a previous surgical history of benign mucinous cystadenoma resected in 2012 who underwent magnetic resonance cholangiopancreatography following her second pregnancy in 2020. This demonstrated a large cystic mass in the left subdiaphragmatic space. Histopathology confirmed a well-differentiated primary peritoneal mucinous cystadenocarcinoma displaying cells of a Mullerian origin. We subsequently discuss the aetiology of these conditions separately and explore the possibility of a connection between the two regarding origin or potential malignant transformation that may otherwise have occurred coincidentally in this young patient. We also acknowledge a paucity of evidence regarding subsequent management strategies.

Mucocoele of the appendix: A case report.

Mucinous cystadenoma is a rare tumour of the appendix. It accounts for only 0.4% of the gastrointestinal tract malignancies and is reported rarely in the literature. Therefore, surgical management is not yet established. Here we report the case of a 65-year-old female who presented with a dragging sensation and a feeling of a mass in the right iliac fossa. Her computed tomography (CT) suggested formation of an abscess in the parietal peritoneum. She was scheduled for laparotomy and upon exploration, a mass was found arising from the tip of the retroperitoneal appendix. The whole of the appendix was studded with mucoid material. Right hemicolectomy was performed and histopathology of the appendix showed mucinous cystadenoma with no evidence of malignant changes. The recovery was uneventful and the patient was discharged on the fourth post-operative day. The unusual presentation of retroperitoneal pseudomyxoma without any intraperitoneal pathology prompted us to report this case.

[A case of mucinous cystadenoma of the pancreas changed from multilocular to unilocular with cyst in cyst structure with an 8-year follow-up].

A 57-year-old woman presented with multilocular cysts like a bunch of grapes, 30mm in diameter, in the tail of the pancreas. The number of cysts has increased, and each one had grown. Eventually, they turned into a unilocular cyst with a cyst in the cyst structure of about 50mm in diameter. Laparoscopic distal pancreatectomy was performed, and the resected specimen was diagnosed with mucinous cystadenoma. We report the rare morphological change in this case and consider the mechanism of its occurrence based on pathological considerations.

Multiparameter Analysis Using 18F-FDG PET/CT in the Differential Diagnosis of Pancreatic Cystic Neoplasms.

Purpose: To evaluate multiparametric analysis in differential diagnosis between pancreatic serous cystic neoplasms (SCNs) and mucinous cystic neoplasms (MCNs) as well as the differentiation of the benign and malignant MCNs with 18F-FDG (18-fluorodeoxyglucose) PET/CT (positron emission tomography). Methods: Forty patients with total of 41 lesions (SCNs: 27/41; MCNs: 14/41), who were preoperatively examined with 18F-FDG PET/CT, were retrospectively analyzed. Multiple quantitative parameters using conventional and texture features were included. The combined model was established with complementary PET/MR parameters. The differential diagnostic efficacy of each independent parameter and the combined model were evaluated with receiver operating characteristic (ROC) analysis. Integrated discriminatory improvement (IDI) and net reclassification improvement (NRI) were used to evaluate improvement of diagnostic efficacy by using combination of multiple parameters. Results: Among all independent parameters, the percentile 5th (0.88 ± 0.38 vs 0.47 ± 0.23, P < 0.001) showed the highest discriminative diagnostic value. The combination of multiple parameters can improve the differential diagnostic efficacy of SCNs and MCNs (sensitivity = 71.4%, specificity = 77.8%, and AUC = 0.788), and the addition of texture parameters to the conventional parameters allowed a significant reclassification with IDI = 0.236 (95% CI: 0.095-0.377) and categorical NRI = 0.434 (95% CI: 0.030-0.838). SURmax (tumor to normal pancreas ratio, T/P) and SURmax (tumor to aorta ratio, T/A) both showed the highest discriminative diagnostic value (sensitivity = 100.0%, specificity = 70.0%, AUC = 0.900, and Youden index = 0.700) in the differential diagnosis of benign and malignant MCNs, with the cutoff values of 0.84 and 0.90, respectively. Conclusion: Combination of multiple parameters using 18F-FDG PET/CT could further improve differentiation between pancreatic SCNs and MCNs. SURmax (T/P) and SURmax (T/A) could improve differential diagnosis of benign and malignant MCNs.

Pulmonary Mucinous Cystadenoma - A Rare Pathology.

BACKGROUND: Pulmonary cystadenoma is a very rare benign tumor of the lung with slow growth rate and most often, asymptomatic. CASE REPORT: We present the case of a 58-year-old patient admitted in the hospital for coughing with hemoptoic sputum. Standard thoracic radiography revealed a 4/5 cm macronodular opacity in the right inferior lobe, paracardiac. Thoracic computed tomography (CT) with contrast discovered a 3.8/4.7 cm homogenous mass in the right inferior lobe. After intraoperative assessment of the lesion a lower right lobectomy with mediastinal and local lymphadenectomy was performed. CONCLUSION: Pulmonary mucinous cystadenoma is one of the primary pulmonary mucinous cystic neoplasia (PMCT) alongside PMCT of low malignancy and pulmonary mucinous cystadenocarcinoma (PMCAC). Because of this and because of the clinical and imagistic similarities between these main entities, establishing a preoperative diagnosis becomes very difficult. Therefore, histopathological and immunohistochemistry studies are mandatory in order to establish the correct diagnosis.

Mucinous cystadenoma arising from a pancreatic heterotopia.

Mucinous pancreatic cystadenoma (MCA) is a premalignant lesion usually located in the body and tail of the pancreas, characteristically in females in their fifties and sixties. The definitive diagnosis relies on the presence of columnar mucin-producing epithelium and ovarian-type stroma. MCA arising from a pancreatic heterotopia (PH) has been rarely reported.

Dilation of the biliary tract secondary to intrahepathic biliary mucinous cystadenoma with ovarian stroma.

We present the case of a 55-year-old female with no history of interest and asymptomatic, who was admitted to perform studies due to findings of an abdominal ultrasound. A dilated left intrahepatic bile duct and a segment of the common hepatic duct, the proximal bile duct, was identified with irregular thickening of the wall, with an increased caliber (12 mm). A cystic lesion of 6 x 5.5 x 8 cm was also identified in the left liver lobe, with multiple septa. Computed tomography (CT) and magnetic resonance imaging (MRI) showed overlapping findings. Endoscopic retrograde cholangiopancreatography (ERCP) identified a filling defect in the common hepatic duct-left intrahepatic duct, which was not pulled with a Fogarty balloon, although it passed through it.

An intrapancreatic bronchogenic cyst simulating a mucinous cystadenoma. A case report.

We present the case of a 61-year-old male with a long-term epigastric mass and pain, high CA 19.9 levels and no weight loss. A computerized tomography was performed and a solid-cystic pancreatic mass was observed. The lesion measured 10 x 8 cm with well-defined margins, a macroscopic calcification and an enhanced solid component.