Prognostic impact of conservative surgery for pancreatic IPMNs

BackgroundThe extent of pancreatic resection for intraductal papillary mucinous neoplasms (IPMNs) remains an unresolved issue. The study aims at analyzing the prognostic impact of conservative surgery (CS) i.e. of pancreatoduodenectomy or distal pancreatectomy, versus total pancreatectomy (TP), for pancreatic IPMNs.MethodsWe retrospectively analyzed and compared data of patients who had undergone pancreatic resection for IPMNs at our center between November 2007 and April 2019. Patients were divided into two main groups based on the extent of surgery: TP-group and CS-group. Subsequently, the perioperative and the long-term outcomes were compared. Moreover, a sub-group analysis of patients with IPMN alone and patients with malignant IPMN, based on preoperative indications to surgery and post-operative histopathological findings, was also performed.ResultsFifty-three patients were included in the TP-group and 73 in the CS-group. In 50 (39.7%) cases the frozen section changed the pre-operative surgical planning, with an extension of the pancreatic resection, in 43 (34.1%) cases up to a total pancreatectomy. Twenty-six patients (20.6%) with low-grade dysplasia at the frozen section underwent CS, while twenty (15.8%) underwent TP. Comparing these two sub-groups no differences were found in surgical IPMN recurrence, nor progression. The rate of overall postoperative complications was 56.6% in the TP-group and 57.5% in the CS-group (p = 0.940). Fifteen patients (20.5%) developed diabetes in the CS-group.None of the patients treated with CS developed a surgical IPMN recurrence or progression during the follow-up period. Comparing OS and DFS of the two groups, we did not find any statistically significant difference (p = 0.619 and 0.315).ConclusionA timely CS can be considered an appropriate and valid strategy in the surgical treatment of the majority of pancreatic IPMNs, as it can avoid the serious long-term metabolic consequences of TP in patients with a long-life expectancy. On the contrary, TP remains mandatory in case of PDAC or high-risk features involving the entire gland.     

Cutaneous histiocytoses in children

Cutaneous histiocytoses constitute a heterogeneous group of diseases characterised by the cutaneous accumulation of cells with the cytological and phenotypic features of macrophages or dendritic cells. The clinical spectrum ranges from self-resolving, skin-limited conditions to severe, multiorgan disease with a high morbidity rate. Until recently, cutaneous histiocytoses were classified according to the immunophenotype of the pathological cells, with differentiation between Langerhans cell histiocytosis (LCH) [CD1a+, CD207 (langerin)+] and non-Langerhans cell histiocytosis (CD68+, CD163+, CD1a?, CD207?). Over the last 12 years, a number of new pathophysiological findings (in particular, molecular pathology results) regarding histiocytoses have contributed to a new classification based on molecular alterations, as well as on clinical and imaging characteristics and the phenotype. The most frequent entities in children are juvenile xanthogranuloma and LCH.     

Appendiceal mucinous neoplasm mimics ovarian tumors: Challenges for preoperative and intraoperative diagnosis and clinical implication

ObjectiveThe aim of this study was to investigate the clinicopathological characteristics of appendiceal mucinous neoplasm that had been preoperatively misdiagnosed as a mucinous ovarian tumor and to discuss the clinical impacts of misdiagnosis.MethodsSeventy-eight patients with a final pathologic diagnosis of appendiceal mucinous neoplasm during a 10-year period were retrospectively reviewed. All patients were diagnosed with ovarian tumor before treatment. A univariate analysis was performed to evaluate predictors of the diagnostic accuracy of a frozen section.ResultsThe patients’ median age was 61 years (range, 21–82 years), and most were diagnosed as low-grade appendiceal mucinous neoplasm (LAMN) (84.62%). The diagnostic concordance between the frozen section and the final pathology was 56.92%. The most consistent diagnosis was LAMN (64.14%). Univariate analysis indicated that maximal diameter of the ovarian tumor (unilateral), laterality of the ovarian tumors (unilateral or bilateral), and frozen section site (appendix or extra-appendix) significantly correlated with the accuracy of frozen section diagnosis (all p < 0.05). Although the diagnostic discordance between the frozen section and the final pathology was 43.08%, only one patient was clinically impacted because of suboptimal surgery.ConclusionAppendiceal mucinous neoplasm should be considered as a differential diagnosis of pelvic mass in women. For patients who do not require fertility-sparing surgery, excision and frozen section of the bilateral ovaries and appendix regardless of the appearance of the appendix might improve the diagnosis. For older patients with peritoneal dissemination, appropriate cytoreductive surgery is recommended to reduce the clinical impact of misdiagnosis.     

乳腺黏液癌的MRI表现及与临床病理特征相关性分析

目的:探讨分析乳腺黏液癌（mucinous breast carcinoma，MBC）的MRI影像学特征及临床病理特征。方法:收集2010年1月至2016年12月间临床资料完整的乳腺黏液癌患者75例，其中单纯型乳腺黏液癌（Pure MBC）患者54例，混合型乳腺黏液癌（Mixed MBC）患者21例。分析比较两组患者的MRI影像学表现和临床病理特征。结果:在两组乳腺癌患者中，单纯型乳腺黏液癌与混合型乳腺黏液癌比较，混合型乳腺黏液癌更多表现为淋巴结阳性(P=0.002)；在年龄、月经状况、家族史、雌激素受体（ER）、孕激素受体（PR）、人表皮生长因子受体2（HER2）水平方面差异无统计学意义（P<0.05）；在MRI检查方面，肿块平均大小及肿块边缘、形态、强化方式、强化程度及表观弥散系数(ADC)差异具有统计学意义(P<0.001)。结论:了解乳腺黏液癌的MRI影像学及临床病理特征，可帮助临床医师预测乳腺黏液癌及其亚型和患者的预后，有利于制定最优的治疗方案。     

Gastric-type mucinous carcinoma of the cervix and its precursors – historical overview

The emerging concept of gastric-type mucinous carcinoma (GAS) of the uterine cervix has been accepted worldwide because of its aggressive clinical behaviour and the absence of high-risk human papillomavirus (HPV). GAS is included as a variant of mucinous carcinoma in the 2014 World Health Organization classification, and its recognition has provoked a discussion on endocervical adenocarcinoma as a single entity such that endocervical adenocarcinoma is now divided into HPV-associated and HPV-independent groups. This article reviews historical and conceptual aspects of GAS and its precursors, starting with minimal deviation adenocarcinoma (MDA), through the ensuing confusion, up to the recent paradigm shift in cervical adenocarcinoma subclassification. The gastric immunophenotype of MDA was demonstrated by a Japanese group in 1998 using the HIK1083 antibody, which recognises gastric pyloric gland mucin, and this elucidated the pathogenesis of this particular tumour. However, this information resulted in overdiagnosis of lobular endocervical glandular hyperplasia (LEGH), first described in 1999 and which represents pyloric gland metaplasia (PGM), as malignant. In the early 2000s the relationship between MDA and LEGH/PGM became a matter of controversy. In 2007 HIK1083 immunohistochemistry extended the morphological spectrum of endocervical adenocarcinoma showing gastric differentiation beyond MDA, which resulted in the proposal of GAS as a distinct entity including MDA as its very well-differentiated subtype. GAS is now considered to be an aggressive and chemoresistant neoplasm that is not related to high-risk HPV. The LEGH/PGM–GAS sequence is currently regarded as an HPV-independent pathway of carcinogenesis. Understanding the underlying molecular events in this process is key to the development of biomarkers for early detection and molecular targeted therapy.     

胰腺囊性肿瘤诊疗及进展

胰腺囊性肿瘤(PCNs)是少见肿瘤，发病原因尚不明确，不良生活习惯（吸烟、饮酒、重咖啡、高脂高蛋白饮食等）、慢性胰腺炎、环境污染因素及遗传因素等是潜在致病因素。PCNs分为浆液性囊性肿瘤（SCN）、黏液性囊性肿瘤（MCN）、胰腺导管内乳头状黏液肿瘤(IPMN)和实性假乳头状瘤（SPN）四种类型。发病症状常不典型，早期诊断难。PCNs具有典型影像特点，单个影像检查技术对PCNs的准确性和局限性不同，CT检查在胰腺病变中仍是最基本、最主要的检查方式。MRI对于小的囊性病灶比CT更有优势。超声内镜（EUS）充分结合了内镜和超声检查的优势，与CT、MRI检查相辅相成，同时还可进行细针穿刺取病理及囊液分析。尽管PCNs大部分为良性，但只要达到切除标准，均应推荐患者进行手术治疗，严格遵循PCNs诊治流程，制订个体化PCNs治疗策略，使患者利益最大化。