External validation of a machine learning based algorithm to differentiate hepatic mucinous cystic neoplasms from benign hepatic cysts.

PURPOSE: To externally validate an algorithm for non-invasive differentiation of hepatic mucinous cystic neoplasms (MCN) from benign hepatic cysts (BHC), which differ in management. METHODS: Patients with cystic liver lesions pathologically confirmed as MCN or BHC between January 2005 and March 2022 from multiple institutions were retrospectively included. Five readers (2 radiologists, 3 non-radiologist physicians) independently reviewed contrast-enhanced CT or MRI examinations before tissue sampling and applied the 3-feature classification algorithm described by Hardie et al. to differentiate between MCN and BHC, which had a reported accuracy of 93.5%. The classification was then compared to the pathology results. Interreader agreement between readers across different levels of experience was evaluated with Fleiss' Kappa. RESULTS: The final cohort included 159 patients, median age of 62 years (IQR [52.0, 70.0]), 66.7% female (106). Of all patients, 89.3% (142) had BHC, and the remaining 10.7% (17) had MCN on pathology. Agreement for class designation between the radiologists was almost perfect (Fleiss' Kappa 0.840, p < 0.001). The algorithm had an accuracy of 98.1% (95% CI [94.6%, 99.6%]), a positive predictive value of 100.0% (95% CI [76.8%, 100.0%]), a negative predictive value of 97.9% (95% CI [94.1%, 99.6%]), and an area under the receiver operator characteristic curve (AUC) of 0.911 (95% CI [0.818, 1.000]). CONCLUSION: The evaluated algorithm showed similarly high diagnostic accuracy in our external, multi-institutional validation cohort. This 3-feature algorithm is easily and rapidly applied and its features are reproducible among radiologists, showing promise as a clinical decision support tool.

Outcome and Management of Serous Tubal Intraepithelial Carcinoma Following Opportunistic Salpingectomy: Systematic Review and Meta-Analysis.

OBJECTIVE: Serous ovarian cancer is the most common subtype of epithelial ovarian carcinoma-the most prevalent type of ovarian cancer. High-grade serous ovarian carcinoma (HGSOC) is thought to arise from the distal fallopian tube, with a precursor lesion known as serous tubal intraepithelial carcinoma (STIC). STICs are found in the final pathology of a salpingectomy specimen in 10%-20% of women with a BRCA gene mutation and 1%-7% of women without a mutation. However, there is currently no official guideline and a paucity of data on the management of STICs. DATA SOURCES: We performed a systematic review following PRISMA guidelines. Five databases were searched for relevant studies on STICs. STUDY SELECTION: Two independent reviewers performed the abstract and full-text screening and data extraction, with conflicts resolved through discussion with the third reviewer. The risk of bias of each study was assessed using the Newcastle-Ottawa scale. DATA EXTRACTION AND SYNTHESIS: Fourteen articles were included. Ninety-nine patients who were diagnosed with STIC and subsequently followed for a mean period of 55.5 months were included in this analysis. Eighty-three patients (83.9%) were BRCA mutation carriers. After the diagnosis of isolated STIC, 7 patients (7.3%) received chemotherapy and 25 (26%) underwent surgical staging. Three of the 25 patients were diagnosed with HGSOC based on the staging surgery. Nine patients were later diagnosed with HGSOC during follow-up, with an average duration of follow-up of 58.5 months between the diagnosis of STIC and the diagnosis of HGSOC. CONCLUSION: Based on our review of the literature, there is a 10.7% risk of having concurrent HGSOC at the time of STIC diagnosis, and the risk of developing a subsequent HGSOC is 14.5%. BRCA mutation status should be determined in cases of isolated STIC, as 83.9% of patients included in this study were found to carry BRCA mutations. We believe it is necessary to further investigate the role of surgical staging following the diagnosis of STIC.

Medical imaging in misdiagnosing serous cystic neoplasms of the pancreas with pancreatic duct dilatation as other pancreatic lessions / 中华肝胆外科杂志

Objective:To analyze the medical imaging in misdiagnosing serous cystic neoplasm(SCN) of the pancreas with pancreatic duct dilatation as other pancreatic lesions.Methods:Data of 21 patients with SCN and pancreatic duct dilatation who underwent surgical resection from January 2011 to November 2021 at the First Affiliated Hospital of Naval Medical University were retrospectively analyzed. There were 9 males and 12 females with ages ranging from 25 to 74, mean ± s. d. (57.4±13.4) years. The clinical features, surgical treatments, CT and MRI imaging features, and misdiagnosis were analyzed.Results:Of 11 patients who presented with abdominal pain, 1 patient had backache, 1 patient was jaundice, 1 patient had weight loss, 1 patinet had fatigue and 6 patients were asymptomatic. Ten patients were operated using pancreaticoduodenectomy, 8 distal pancreatectomy, 2 segmental pancreatectomy and 1 total pancreatectomy. For 11 patients, the lesion was located in the head of pancreas, and for 10 patients in the body and tail of pancreas. The tumor size was 23.0-92.0 (45.8±17.8) mm. All 21 patients had upstream pancreatic duct dilatation but no downstream pancreatic duct dilatation. The inner diameter of the pancreatic duct was 4.0-11.0(7.1±2.0) mm. Of 13 patients showed a low signal intensity on T 1-weighted imaging, 18 patients showed a markedly high signal intensity on T 2-weighted imaging, 13 patients showed no limitation on diffusion weighted imaging. Among the 11 patients who underwent CT examination, 5 patients were diagnosed to have intraductal papillary mucinous neoplesm (IPMN), 3 SCN, 1 pancreatic neuroendocrine tumor, 1 pancreatic cancer and 1 cyst. The misdiagnotic rate of CT was 72.7% (8/11). Among the 18 patients who underwent MRI examination, 9 patients were diagnosed to have IPMN, 3 mucinous cystic neoplasm, 3 SCN, 2 pancreatic cancer and 1 solid pseudopapillary tumor. The misdiagnosis rate of MRI was 83.3% (15/18). Conclusion:SCN with pancreatic duct dilatation was easily misdiagnosed as IPMN or other pancreatic solid tumors. The difference between SCN with pancreatic duct dilatation and IPMN was that the downstream pancreatic duct of SCN was normal. SCN showed a markedly high signal intensity on T 2-weighted imaging and no limitation on diffusion weighted imaging, which can help to distinguish SCN from other pancreatic solid tumors.

Neoplasia quística mucinosa hepática: reporte de caso y revisión de la literatura

La neoplasia quística mucinosa (NQM) primaria de hígado es una neoplasia poco usual de tipo epitelial que se caracteriza por la formación de quistes y que típicamente no tienen comunicación con los ductos biliares. Representa menos del 5% de todas las lesiones quísticas del hígado y existen solo 250 casos en la literatura mundial. Presentamos el caso de una paciente mujer de 23 años con una lesión de 13,5 x 10,2 cm, hipodensa, lobulada, con múltiples tabiques de hasta 2,5 mm de espesor y pequeñas imágenes quísticas en su interior, que condiciona dilatación de la vía biliar intrahepática a predominio izquierdo y del colédoco. El estudio anatomopatológico concluyó que la tumoración correspondía a una neoplasia quística mucinosa de hígado.

Mucinous cystic neoplasm (MCN) of the liver is an unusual cyst-forming epithelial neoplasm, typically showing no communication with the bile ducts. This neoplasm represents less than 5% of all cystic lesions of the liver and there are only 250 cases in the world literature. We present the case of a 23-year-old female with a 13.5 x 10.2 cm lesion, hypodense, lobulated, with multiple septa up to 2.5 mm thick and small cystic images inside, which produces intrahepatic bile duct and common bile duct dilatation. The pathological study concluded that the tumor corresponded to a mucinous cystic neoplasm of the liver.

A nomogram for predicting pancreatic mucinous cystic neoplasm and serous cystic neoplasm.

OBJECTIVES: To develop and validate a nomogram for the preoperative prediction of pancreatic serous cystic neoplasm (SCN) and mucinous cystic neoplasm (MCN) based on multidetector computed tomography (MDCT). MATERIALS AND METHODS: In this retrospective study, the data of 227 patients with SCN and MCN were analyzed. Each patient underwent MDCT and surgical resection. A multivariable logistic regression model was developed using a training set consisting of 129 patients with SCN and 38 patients with MCN who were admitted between October 2012 and April 2019. The model was validated in 60 consecutive patients, 44 of whom had SCN and 16 of whom had MCN, admitted between May 2019 and April 2020. The regression model was adopted to establish a nomogram. Nomogram performance was determined by its discriminative ability and clinical utility. RESULT: The multivariable logistic regression model included sex, size, location, shape, cyst characteristic, and cystic wall thickening. The individualized prediction nomogram showed good discrimination in the training sample (AUC 0.89; 95% CI 0.83-0.95) and in the validation sample (AUC 0.81; 95% CI 0.70-0.94). If the threshold probability is between 0.03 and 0.9, and > 0.93 in the prediction model, using the nomogram to predict SCN and MCN is more beneficial than the treat-all-patients as SCN scheme or the treat-all-patients as MCN scheme. The prediction model showed better discrimination than the radiologists' diagnosis (AUC = 0.68). CONCLUSION: The nomogram could predict SCN and MCN preoperatively and may aid clinical decision-making.

A rare cecal subepithelial tumor in a Crohn´s Disease patient

Appendiceal tumors comprise a variety of histologic types, including appendiceal mucinous neoplasms, which can be grouped as premalignant lesions, tumors of uncertain malignant potential, and malignant lesions. The appendiceal mucinous neoplasms are characterized by mucinous epithelial proliferation with extracellular mucin and pushing tumor margins, commonly an incidental finding during operative exploration. We report the case of a low-grade appendiceal mucinous neoplasm presenting as a subepithelial lesion in Crohn´s Disease patient. The diagnosis was not straightforward, and only surgical resection allowed an accurate diagnosis. Although Inflammatory Bowel Disease is a risk factor for the development of colorectal neoplasms, the absolute risk for appendiceal tumors is uncertain. The frequency of progression to malignancy remains to be determined.

[Appendiceal diverticulosis, a potential mimic of low grade appendiceal mucinous neoplasm: analysis of 20 cases].

Objective: To study the clinicopathologic features and the key points of differential diagnosis of appendiceal diverticulosis (AD) and low-grade mucinous neoplasm (LAMN) to avoid over-diagnosis. Methods: The clinical data, pathologic features and follow-up information of 20 patients with AD, who were diagnosed in the Peking University Third Hospital from January 2010 to November 2019 were collected and compared with 44 cases of LAMN which were diagnosed during the same period. Results: Among the 20 cases of AD, hypermucinous epithelium, filiform villi or undulating epithelium and mucosa atrophy were observed in 10 (50.0%), 4 (20.0%) and 14 (70.0%) cases, respectively, however, focally loss of lamina propria and mucosa/submucosa fibrosis were observed only in 1 (5.0%) and 4 (20.0%) cases, respectively. Extramural mucin deposits were seen in 11 (55.0%) cases, all were acellular mucin. Mucosal Schwann cell hyperplasia were present in 12 (60.0%) cases. Nine (45.0%) and 5 (25.0%) cases were associated with acute diverticulitis or acute suppurative appendicitis, respectively. In comparison with AD, LAMN cases more frequently showed hypermucinous epithelium (42/44, 95.5%), filiform villi or undulating epithelium (43/44, 97.7%), loss of lamina propria (43/44, 97.7%) and fibrosis and hyalinization of appendiceal wall (44/44, 100.0%), whereas mucosal atrophy (4/44, 9.1%) and Schwann cell hyperplasia(11/44, 25.0%) were less frequently seen (P<0.05). Follow-up information was available for 10 AD patients and 27 LAMN patients; all were alive without evidence of recurrence. Conclusions: Epithelial hyperplasia, loss of lamina propria, fibrosis of the appendiceal wall and extramural mucin deposits may occur focally in AD and should be distinguished from LAMN. The preservation of normal appendiceal mucosa architecture, lack of diffuse appendiceal wall fibrosis and hyalinization, and no definite neoplastic epithelium are the key point for preventing over-diagnosis.

[Expression of ARID1A in ovarian seromucinous neoplasms and its clinicopathological significance].

Objective: To investigate the clinical, pathological and immunohistochemical features of seromucinous neoplasms, including seromucinous cystadenoma, borderline tumour and seromucinous carcinomas of the ovary. Methods: A retrospective review of the seromucinous neoplasms collected between June 2006 and December 2018 was conducted at the First Medical Center of PLA General Hospital. EnVision immunohistochemical staining was used to detect the expression of CK7, PAX8, ER, PR, WT1, p16, p53 and Baf250a which was encoded by the ARID1A gene. Results: A total of 75 ovarian seromucinous neoplasms were included. There were 30 cases of benign seromucinous cystadenoma, whose patients aged 12 to 83 years (mean, 36 years). The tumor histologically composed of endocervical-type mucinous epithelium and serous-type cells, each of which accounted for more than 10%. Among the 34 cases of seromucinous borderline tumour including 7 cases with concurrent endometriosis, the patients aged 21 to 72 years (mean, 39 years). Characteristic histologic features were broad papilla structure and an admixture of cell types, predominant endocervical-like mucinous cells (non-intestinal, no goblet cells), eosinophilic cells and others such as clear cells, hobnail cells, ciliated cells, and endometrioid cells. The larger papillae tended to have oedematous stroma containing neutrophils. In the 11 cases of seromucinous carcinomas including 2 cases with concurrent endometriosis, patients aged 26 to 61 years (mean, 40 years). Seromucinous carcinomas exhibited a predominant papillary architecture with smaller components of confluent glandular, microglandular and solid structure, expansive stromal invasion pattern, and sometimes locally destructive infiltration. An admixture of epithelial cell types was in seromucinous carcinomas, as well as borderline tumour. Immunohistochemically, the tumours were positive for CK7, PAX8, p16, estrogen receptor and progesterone receptor (positive in 10% to 80% of the cases). They were negative for WT1, while p53 staining showed a "wild-type" pattern. The Ki-67 positive rate was 20% to 60%. Loss of ARID1A-encoded protein Baf250a staining was observed in 6 (30%) of the 20 seromucinous borderline tumors, and 2 of the 11 seromucinous carcinomas. According to FIGO 2014 staging system, there were 4 cases of ⅠA, 3 cases of ⅡA and 4 cases of ⅢC. Follow-up information was available in 9 patients of seromucinous carcinomas, and 2 lost to follow-up. Eight were alive (follow-up for 6 to 108 months), including 2 patients with relapse, but 1 patient who initially presented with a stage ⅢC tumor died of disease 60 months after the cancer diagnosis. Thirty-four patients of borderline tumour were all alive at the end of follow-up, including 1 with relapse. Conclusions: Seromucinous neoplasms have characteristic histopathological and immunopathological features. Both borderline tumors and carcinomas have complex structures and cellular components. ARID1A as a tumor-suppressor gene plays a role in the oncogenesis of ovarian seromucinous neoplasms. The loss of staining with ARID1A-encoded Baf250a and wild-type p53 in seromucinous neoplasms together support that seromucinous neoplasms could be type Ⅰ tumor of dualistic model of epithelial ovarian cancer, with favourable prognosis.

[Clinical analysis and prognosis factors of malignancy in the patients with mucinous cystic neoplasms of the pancreas].

Objective: To examine clinic pathological features of mucinous cystic neoplasms (MCN) of the pancreas and explore the prognosis factors associated with malignant transformation of MCN of the pancreas. Methods: This multicenter retrospective study included all patients with pancreatic MCN underwent surgery at Department of Pancreatic Surgery, Zhongshan Hospital of Fudan University between January 2008 and December 2018 and patients with MCN who confirmed by postoperative pathology from Multicenter Pancreatic Cystic Tumor Database. There were 50 males (14.4%) and 297 females (85.6%) and the mean age was 48.6 years (range: 24-77 years). According to the pathological results, all patients were divided into benign lesion group (including MCN and which associated with low/medium grade dysplasia) and malignant lesion group (including MCN with high-grade dysplasia or invasive carcinoma) . The preoperative clinical pathology and imaging features of the two groups were analyzed, and the risk factors associated with malignant transformation of MCN were statistically analyzed. Results: This multicenter retrospective study included 347 patients. Twenty-four of the 347 patients were malignant, including 7 males and 17 females. Univariate analysis showed that age, gender, carcino-embryonic antigen (CEA) , CA19-9, CA125, tumor maximum diameter, and tumor location were remarkably different in the two groups (P<0.05) . Logistic regression analysis found that the preoperative tumor maximum diameter (OR=1.023, 95% CI: 1.002-1.045, P=0.035) was an independent risk factor for MCN malignant transformation. Conclusions: Age, gender, CEA, CA19-9, CA125, tumor maximum diameter, and tumor location are important features of MCN malignant lesions.The maximum diameter of the preoperative tumor is an independent risk factor for MCN malignant transformation.

A rare cecal subepithelial tumor in a Crohn´s Disease patient.

Appendiceal tumors comprise a variety of histologic types, including appendiceal mucinous neoplasms, which can be grouped as premalignant lesions, tumors of uncertain malignant potential, and malignant lesions. The appendiceal mucinous neoplasms are characterized by mucinous epithelial proliferation with extracellular mucin and pushing tumor margins, commonly an incidental finding during operative exploration. We report the case of a low-grade appendiceal mucinous neoplasm presenting as a subepithelial lesion in Crohn´s Disease patient. The diagnosis was not straightforward, and only surgical resection allowed an accurate diagnosis. Although Inflammatory Bowel Disease is a risk factor for the development of colorectal neoplasms, the absolute risk for appendiceal tumors is uncertain. The frequency of progression to malignancy remains to be determined.

The many faces of pseudomyxoma peritonei: a radiological review based on 30 cases / As múltiplas faces do pseudomixoma peritonial: uma revisão radiológica baseada em 30 casos

Abstract Objective: To determine the most common imaging features of pseudomyxoma peritonei (PMP), as well as the histologic subtypes of the primary tumors. Materials and Methods: We reviewed 30 cases of women with pathologically confirmed PMP. Only computed tomography scans were available. All cases were retrospectively studied by four radiologists, working independently. We identified the most common imaging findings, the predominant primary site of the disease, and the growth pattern. The most common sites of recurrence were also analyzed. Results: The most common computed tomography finding was peritoneal/omental nodules (including "omental caking"), followed by visceral scalloping and non-mucinous ascites. The most common site of the primary tumor was the appendix (in 63.3%), followed by the ovaries (in 16.6%), and 16.6% of the tumors were of undetermined origin. There was one case of synchronous appendiceal and ovarian tumors. Low-grade mucinous neoplasm was the most common histologic subtype, accounting for 84.2% of the appendiceal tumors and 40% of the ovarian tumors. Conclusion: Although PMP is a relatively rare entity, radiologists must be aware of its possible imaging findings, common locations, and possible patterns of recurrence. The origin of the primary tumor should also be investigated. Future studies are needed in order to determine which preoperative imaging findings predict surgical outcomes and to characterize the main findings of radiological recurrence.

Resumo Objetivo: Determinar as características de imagem mais frequentes do pseudomixoma peritonial (PMP), bem como os subtipos histológicos dos tumores primários. Materiais e Métodos: Foram revisados 30 casos confirmados patologicamente de PMP em mulheres. Somente a tomografia computadorizada estava disponível. Todos os casos foram estudados de forma independente e retrospectiva por quatro radiologistas. Os autores relataram os achados de imagem mais frequentes, a localização predominante da doença primária e o padrão de crescimento. Os locais de recorrência mais comuns também foram analisados. Resultados: Os achados tomográficos mais frequentes foram nódulos peritoniais/omentais (incluindo "bolo omental"), seguidos por scalloping visceral e ascite não mucinosa. Os padrões de localização mais comuns do PMP também foram documentados. A grande maioria dos tumores primários foi de origem apendicular (63,3%), seguida de origem ovárica (16,6%) e indeterminada (16,6%). Houve um caso síncrono de tumor apendicular e ovário. A neoplasia mucinosa de baixo grau foi o subtipo histológico mais frequente, representando 84,2% dos tumores do apêndice e 40% dos tumores primários ovarianos. Conclusão: Embora o PMP continue sendo uma entidade relativamente rara, o radiologista deve estar ciente de seus possíveis achados de imagem, locais comuns e possíveis padrões de recorrência. A investigação do tumor primário também deve ser encorajada. Futuros estudos são necessários para prever o resultado cirúrgico da imagem pré-operatória e caracterizar os principais achados de recorrência radiológica.

[Cystic lesions of the pancreas : Differential diagnostics and treatment]. / Zystische Raumforderungen des Pankreas : Differenzialdiagnostik und Therapie.

Cystic space-occupying lesions of the pancreas represent incidental findings in most cases. As there is a potential risk of malignant transformation further evaluation of the lesions as well as a follow-up of these patients is usually recommended. Before this work-up is initiated the clinical situation of the patient as a whole and comorbidities, age and personal preferences have to be taken into account. So far there are no biomarkers that reliably predict the risk of malignant transformation. Imaging by magnetic resonance tomography (MRI) in combination with magnetic resonance cholangiopancreatography (MRCP) is more accurate than computed tomography to identify worrisome features. During follow-up, endoscopic ultrasound (EUS) can be used as complementary method to MRI/MRCP. Using contrast enhancement or endoscopic fine needle aspiration (EUS-FNA) may influence the therapeutic strategy in some patients. Whereas for some cystic pancreatic lesions consensus has been reached, varying recommendations exist for intraductal papillary mucinous neoplasms (IPMN). There is consensus that in main-duct as well as in mixed-type IPMN surgery is recommended. The management of branch-duct type IPMN, however, remains controversial. A multidisciplinary expert panel including gastroenterologists, visceral surgeons, radiologists and pathologists is essential to discuss all cases of patients with cystic pancreatic lesions and to guarantee an optimal, patient-centered treatment recommendation.

The many faces of pseudomyxoma peritonei: a radiological review based on 30 cases.

OBJECTIVE: To determine the most common imaging features of pseudomyxoma peritonei (PMP), as well as the histologic subtypes of the primary tumors. MATERIALS AND METHODS: We reviewed 30 cases of women with pathologically confirmed PMP. Only computed tomography scans were available. All cases were retrospectively studied by four radiologists, working independently. We identified the most common imaging findings, the predominant primary site of the disease, and the growth pattern. The most common sites of recurrence were also analyzed. RESULTS: The most common computed tomography finding was peritoneal/omental nodules (including "omental caking"), followed by visceral scalloping and non-mucinous ascites. The most common site of the primary tumor was the appendix (in 63.3%), followed by the ovaries (in 16.6%), and 16.6% of the tumors were of undetermined origin. There was one case of synchronous appendiceal and ovarian tumors. Low-grade mucinous neoplasm was the most common histologic subtype, accounting for 84.2% of the appendiceal tumors and 40% of the ovarian tumors. CONCLUSION: Although PMP is a relatively rare entity, radiologists must be aware of its possible imaging findings, common locations, and possible patterns of recurrence. The origin of the primary tumor should also be investigated. Future studies are needed in order to determine which preoperative imaging findings predict surgical outcomes and to characterize the main findings of radiological recurrence.

Efficacy evaluation of the indications for surgery in different guidelines for predicting malignancy of mucinous pancreatic cystic neoplasms / 中华胰腺病杂志

Objective To evaluate the performance of the Sendai Guidelines,Fukuoka Guidelines and Pancreatic Cystic Lesions Management Guidelines (Chinese guidelines) in predicting malignant mucinous pancreatic cystic neoplasms (PCN).Methods A retrospective analysis of 196 patients,who received surgery and were pathologically identified as PCN or intraductal papillary mucinous neoplasms (IPMN),underwent surgical resection in Ruijin Hospital affiliated with Shanghai Jiao Tong University from January 2003 to April 2017 was performed.The differences on clinical and pathological parameters between malignant mucinous and benign mucinous PCN were compared.The accuracy,sensitivity,specificity,positive predictive value (PPV)and negative predictive value (NPV) of the indications for surgery in the Sendai,Fukuoka and Chinese Guidelines in predicting malignant mucinous PCN were calculated.Results Of 196 patients,39 patients (19.9％) were confirmed as malignant tumors and 157 patients (80.1％) were confirmed as benign tumors by pathology.There were significant differences on age,symptoms (abdominal pain,jaundice or pancreatitis),tumor solid composition,pancreatic duct diameter,tumor site,tumor diameter ＞3 cm,and serum CA199 level between malignant and benign patients (all P ＜0.05).But there were no significant differences on gender distribution,tumor diameter,mural nodules and the proportion of mucinous cystic neoplasm (MCN)and intra-ductal papillary mucinous neoplasm (IPMN).165 patients (84.2％) met the Sendai Guidelines,153 patients (78.1％) met the Chinese guideline,and only 61 patients (31.1％) met the Fukuoka Guidelines.All 39 patients with malignant tumors met the indications in Sendai Guidelines and Chinese guidelines,and only 35 patients had the indication for surgery in the Fukuoka Guidelines.The accuracy,sensitivity,specificity,PPV and NPV of the Fukuoka Guidelines for predicting the malignancy were 84.7％,89.7％,83.4％,57.4％ and 97.0％,compared to 35.7％,100％,19.8％,23.6％ and 100％ for the Sendai and 41.8％,100％,27.4％,25.5％ and 100％ for the Chinese guidelines,respectively.Conclusions The performance of the Chinese guideline is slightly better than the Sendai Guidelines,while both of them can lead to a larger number of patients undergoing unnecessary surgical resection.Though the rate of missed diagnosis could reach 10.3％,the Fukuoka Guidelines gets the highest accuracy.

Tratamento cirúrgico de lesões císticas do pâncreas: uma experiência unicêntrica / Surgical management of cystic lesions of the pancreas: a single-centre experience

ABSTRACT BACKGROUND: Cystic lesions of the pancreas represent a group of pancreatic diseases with great histological heterogeneity, varying from benign lesions, some of them with malignant potential, to overt malignant lesions. OBJECTIVE: To describe the cases of cystic lesions of the pancreas which underwent surgical intervention at a tertiary university hospital. METHODS: This is a retrospective population-based study (historical cohort) which was carried out enrolling individuals attended at the Outpatient service of Pancreas Surgery of the Hospital de Clínicas of Unicamp. The individuals underwent surgical procedures performed from January 2012 through December 2016. RESULTS: In the period evaluated, 39 cases of cystic lesions of the pancreas which underwent surgery were identified, 26 (66.6%) of which were female. The average age at diagnosis was 47.4±16.4 years (range, 18-73). In regards to symptoms, 35 (89.7%) were symptomatic. The average length of hospital stay was 10 days (range 4-76). Surgeries performed to treat the lesions depended on the localization and type of the lesions: cystojejunostomy (41%), distal pancreatectomy (36%), pancreaticoduodenectomy (15.4%), drainage of ruptured and/or infected pseudocyst (5.2%) and central pancreatectomy (2.6%). CONCLUSION: Cystic lesions of the pancreas are a group of lesions with a highly varying presentation and diagnostic approach and may require an also highly variable surgical treatment. An appropriate preoperative imaging diagnosis is essential for their management.

RESUMO CONTEXTO: As lesões císticas do pâncreas representam um grupo de doenças pancreáticas com grande heterogeneidade histológica, variando desde lesões benignas, algumas com potencial pré-maligno, até outras degeneradas para formas malignas. OBJETIVO: Descrever os casos de LCPs submetidos à intervenção cirúrgica em um hospital universitário terciário. MÉTODOS: Trata-se de um estudo retrospectivo populacional (coorte histórica) realizado com a participação de indivíduos atendidos no Ambulatório de Cirurgia do Pâncreas do Hospital de Clínicas da Unicamp. Os indivíduos foram submetidos a procedimentos cirúrgicos realizados no período de janeiro de 2012 a dezembro de 2016. RESULTADOS: No período avaliado, foram identificados 39 casos de lesões císticas do pâncreas operados, sendo 26 (66,6%) do sexo feminino. A idade média no diagnóstico foi de 47,4±16,4 anos. Em relação aos sintomas, 35 (89,7%) eram sintomáticos. O tempo médio de internação foi de 10 dias (variação de 4-76). As cirurgias realizadas para o tratamento das lesões dependeram da localização e do tipo das lesões: derivação pseudocisto-jejunal (41%), pancreatectomia distal (36%), pancreaticoduodenectomia (15,4%), drenagem de pseudocistos rotos e/ou infectados (5,2%) e pancreatectomia central (2,6%). CONCLUSÃO: As lesões císticas do pâncreas são um grupo de lesões cuja apresentação e abordagem diagnóstica são altamente heterogêneas e que podem requerer um tratamento cirúrgico altamente complexo e variável. Um diagnóstico pré-operatório adequado é essencial para definir o seu tratamento.

[Morphologic features of fallopian tubal epithelium in pelvic high-grade serous carcinoma].

Objective: To study the pathologic features of fallopian tubal epithelium in patients with pelvic high-grade serous carcinoma (HGSC), to investigate its role in pelvic serous carcinogenesis and to reclassify the primary site of HGSC based on recently proposed criteria. Methods: The fallopian tubes in 58 cases of pelvic HGSC (54 cases of ovarian primary, 3 cases of tubal primary, 1 case of peritoneum) and 25 cases of pelvic non-HGSC (5 cases of ovarian low-grade serous cancer, 9 cases of endometrioid cancer, and 11 cases of clear cell ovary carcinoma) were collected from June 2015 to December 2016, and serially examined under light microscope (SEE-FIM protocol). Immunostaining for p53 and Ki-67 was performed to evaluate the presence of p53 signature, serous tubal intraepithelial lesion (STIL), serous tubal intraepithelial carcinoma (STIC) and invasive carcinoma in these fallopian tubes. Meanwhile, primary site of HGSC based on the recently proposed diagnostic criteria were also reclassified. Results: Among the study group, the frequencies of p53 signature, STIL, STIC and invasive HGSC were 27.6% (16/58), 43.1% (25/58), 36.2% (21/58) and 67.2% (39/58), respectively, while in control group, the proportions were 24.0% (6/25), 0, 0 and 8.0% (2/25), respectively. The continuum of epithelial changes in the process of serous neoplasia including p53 signature, STIL, STIC and invasive carcinoma was identified in 8 cases of pelvic HGSC. The proportions of STIL, STIC and invasive carcinomas in HGSC group were higher than that in non-HGSC group (P<0.01). About 80.0% (20/25) of STIL and 85.7% (18/21) of STIC were present unilaterally. Diagnostically, the study group contained the 17 cases of ovarian HGSC, 40 cases of tubal HGSC, and 1 case of peritoneal HGSC after reclassification of the cancer primary. Conclusions: Continuous changes of tubal epithelium including p53 signature, STIL, STIC and invasive carcinomas are identified in patients with HGSC, supporting the current understanding that the fallopian tube is likely the cellular source of the majority HGSC. STIL and STIC may be specific to pelvic HGSC and may act as a target for future research on the early detection and prevention of this disease. The newly proposed diagnostic criteria for pelvic HGSC will lead us to more accurate classification of cancer primary sites. Correct classification of HGSC may have potential impacts for cancer prevention and improve our understanding of ovarian serous carcinogenesis.

An appraisal of pancreatic cyst fluid molecular markers

Pancreatic malignancy is the third leading cause of cancer related death in the United States with limited viable screening options. By the end of this decade, cancers are poised to become the leading cause of death with pancreatic cancer projected to be the second leading cause of cancer related mortality. Pancreatic cystic lesions (PCLs) are found in approximately 5%–14% of patients due to the increased utilization of cross-sectional imaging, with approximately 8%–10% of pancreatic cancers originating as PCLs. Current screening guidelines have shown discrepancies between morphologic characteristics of PCLs and identifying advanced pancreatic disease. Molecular analysis has emerged as a novel technology to aid in adequate diagnosis and management decisions of PCLs. Mucinous cysts including intraductal papillary mucinous neoplasms (IPMNs) or mucinous cystic neoplasms have similar oncogenic mutations including KRAS, TP53, SMAD4, PIK3CA, PTEN, or CKDN2A, while GNAS and RNF43 mutations are specific only to IPMNs. Serous cystadenomas have been associated with a loss of tumor suppressor gene VHL, while solid-psuedopapillary neoplasms have an oncogenic mutation CTNNB1. A specific molecular marker to diagnose existing high-grade dysplasia or impending malignant transformation is yet to be identified. Moving forward it is important to advance technology in isolating and identifying high-risk molecular markers from cyst fluid while considering their increased utilization in the evaluation of PCLs.

Morphologic features of fallopian tubal epithelium in pelvic high-grade serous carcinoma / 中华病理学杂志

Objective@#To study the pathologic features of fallopian tubal epithelium in patients with pelvic high-grade serous carcinoma (HGSC), to investigate its role in pelvic serous carcinogenesis and to reclassify the primary site of HGSC based on recently proposed criteria.@*Methods@#The fallopian tubes in 58 cases of pelvic HGSC (54 cases of ovarian primary, 3 cases of tubal primary, 1 case of peritoneum) and 25 cases of pelvic non-HGSC (5 cases of ovarian low-grade serous cancer, 9 cases of endometrioid cancer, and 11 cases of clear cell ovary carcinoma) were collected from June 2015 to December 2016, and serially examined under light microscope (SEE-FIM protocol). Immunostaining for p53 and Ki-67 was performed to evaluate the presence of p53 signature, serous tubal intraepithelial lesion (STIL), serous tubal intraepithelial carcinoma (STIC) and invasive carcinoma in these fallopian tubes. Meanwhile, primary site of HGSC based on the recently proposed diagnostic criteria were also reclassified.@*Results@#Among the study group, the frequencies of p53 signature, STIL, STIC and invasive HGSC were 27.6% (16/58), 43.1% (25/58), 36.2% (21/58) and 67.2% (39/58), respectively, while in control group, the proportions were 24.0% (6/25), 0, 0 and 8.0% (2/25), respectively. The continuum of epithelial changes in the process of serous neoplasia including p53 signature, STIL, STIC and invasive carcinoma was identified in 8 cases of pelvic HGSC. The proportions of STIL, STIC and invasive carcinomas in HGSC group were higher than that in non-HGSC group (P<0.01). About 80.0% (20/25) of STIL and 85.7% (18/21) of STIC were present unilaterally. Diagnostically, the study group contained the 17 cases of ovarian HGSC, 40 cases of tubal HGSC, and 1 case of peritoneal HGSC after reclassification of the cancer primary.@*Conclusions@#Continuous changes of tubal epithelium including p53 signature, STIL, STIC and invasive carcinomas are identified in patients with HGSC, supporting the current understanding that the fallopian tube is likely the cellular source of the majority HGSC. STIL and STIC may be specific to pelvic HGSC and may act as a target for future research on the early detection and prevention of this disease. The newly proposed diagnostic criteria for pelvic HGSC will lead us to more accurate classification of cancer primary sites. Correct classification of HGSC may have potential impacts for cancer prevention and improve our understanding of ovarian serous carcinogenesis.

Which is the best current guideline for the diagnosis and management of cystic pancreatic neoplasms? An appraisal using evidence-based practice methods.

BACKGROUND AND AIM: Cystic pancreatic neoplasms (CPNs) are an increasingly diagnosed entity. Their heterogeneity poses complex diagnostic and management challenges. Despite frequently encountering these entities, particularly in the context of the increased imaging of patients in modern medicine, doctors have to rely on incomplete and ambiguous published literature. The aim of this project was to review the guidelines relating to CPNs using evidence-based practice (EBP) methods. METHODS: A search of both the primary and secondary literature was performed. Five sets of guidelines were identified which were then methodologically appraised by the AGREE II instrument, a validated and widely utilised tool for guideline development assessment. RESULTS: The 2014 'Italian consensus guidelines for the diagnostic work-up and follow-up of cystic pancreatic neoplasms' were found to be the most methodologically sound guidelines, on the basis of both the overall score and average weighted domain score. CONCLUSIONS: The current best guidelines were identified. The Appraisal of Guidelines for Research and Evaluation (AGREE II) instrument can be used for retrospective review of published guidelines or as a roadmap for guideline-writing groups. All guidelines found were methodologically limited. Further longitudinal/prospective studies are required to improve the level of evidence. KEY POINTS: • Cystic pancreatic neoplasms (CPNs) are an increasingly encountered entity in modern medicine. • Clinical uncertainty remains with regard to optimal diagnostic and management strategies. • The Italian consensus guidelines for cystic pancreatic neoplasms are currently the best guidelines.

Italian consensus guidelines for the diagnostic work-up and follow-up of cystic pancreatic neoplasms.

This report contains clinically oriented guidelines for the diagnostic work-up and follow-up of cystic pancreatic neoplasms in patients fit for treatment. The statements were elaborated by working groups of experts by searching and analysing the literature, and then underwent a consensus process using a modified Delphi procedure. The statements report recommendations regarding the most appropriate use and timing of various imaging techniques and of endoscopic ultrasound, the role of circulating and intracystic markers and the pathologic evaluation for the diagnosis and follow-up of cystic pancreatic neoplasms.