Objective To summarize the current progress in diagnosis and treatment of polycystic liver disease, and provide ideas for further research direction and clinical practice of polycystic liver disease. Method The domestic and foreign literature about polycystic liver disease was reviewed, screened, and summarized. Results The diagnosis, evaluation, and classification of polycystic liver disease were mainly performed clinically by abdominal ultrasound and CT. Surgical treatment was the main treatment, including aspiration sclerotherapy, fenestration, segmental hepatectomy, and liver transplantation. Conclusions The classification and evaluation scheme of polycystic liver disease needs to be improved, and its medical treatment still needs further research. Estrogen receptor and gonadotropin-releasing hormone receptor are promising therapeutic targets.
ObjectiveTo summarize the clinicopathological features of microencapsulated/reticular schwannoma.MethodsTwo cases of microencapsulated/reticular schwannoma treated in West China Hospital of Sichuan University were retrospectively collected. The histological and immunohistochemical features were summarized, and the related literatures were reviewed.ResultsTwo cases of microcystic/reticular neurilemmoma diagnosed and treated in West China Hospital of Sichuan University were male. The clinical manifestations were “dull pain in the right lower abdomen” and “mass of left index finger”. Microscopically, the characteristic reticular or cribriform structures were found in all cases, and lymphocytic sheath was seen in 1 case. Immunohistochemical staining showed S-100, glial fibrillary acidic protein (GFAP) and calretinin positive. By February 2020, only 41 cases were reported in the literatures （there were no cases of this group),there was no significant gender difference, the median age was 55 years (11–93 years), and the median size of the lesion was 2.1 cm (0.4–13.0 cm). Most of them were found by accident, without obvious symptoms and signs, and no history of neurofibromatosis type 1 or 2. Microencapsulated/reticular schwannoma in this patients mainly occurs in the viscera, especially in the gastrointestinal tract (mostly in the the colorectal of lower gastrointestinal tract ), most of them were solitary nodular masses without capsule. Microscopically, the boundary was clear, and the characteristic microcapsules and reticular structures were seen. The tumor cells were diffusely expressing S-100 protein, and GFAP and calretinin were expressed in varying degrees. Electron microscopy showed the characteristics of Schwann cells.ConclusionsMicrocystic/reticular schwannoma is a rare subtype of schwannoma, which needs to be differ-entiated from multiple benign and malignant neoplasms. The diagnosis of the tumor is a challenge for the pathologist.
Objective To investigate the surgical technique of reduced sized liver transplantation. Methods A reduced size liver transplantation was successfully performed on a 11-year old girl with incurable caroli′s disease. Results The recovery of liver graft function was good after the operation in this patients without complications. Conclusion Reduced size liver transplantation is a safe and effective technique for pediatric liver transplantation to provide liver graft. Authors introduced their experiences of surgical technique in this patient.
Fibropolycystic liver diseases (FLDs) is a rare genetic disorder, including bile duct hamartomas, congenital hepatic fibrosis, polycystic liver disease, Caroli’s disease, and choledochal cysts. Fibropolycystic liver diseases has received little clinical attention and exhibits a variety of imaging manifestations, leading to a high likelihood of missed diagnosis and misdiagnosis. Through this case, we delineate the characteristic imaging manifestations of the disease and its underlying pathological mechanisms. Our objective is to enhance readers' comprehension of the disease and thereby reduce the rate of missed diagnosis and misdiagnosis of the disease.
目的 全面介绍先天性囊性腺瘤样畸形(CCAM)其可能的病因，临床、病理特点及诊断手段，循证探讨针对CCAM的治疗方法及预后。 方法 对我院2011年11月收治的1例罕见的CCAM患者的临床资料进行分析，并对相关文献进行复习。 结果 患者数次误诊后最终诊断为CCAM，予手术治疗后痊愈，随访1年无复发。 结论 CCAM是一种少见的、非遗传性的、错构瘤样的肺发育异常，为一种良性的肺部畸形，其特点是局部肺终末呼吸性细支气管过度生长。CCAM多通过产前影像学检查、活组织检查或术后病检诊断。手术为治愈该病的最根本、最重要措施。
【摘要】目的探讨乳腺囊性肿块的临床特点及诊治经验。方法对我院1988年4月至2003年5月期间收治的220例乳腺囊性肿块患者的临床资料进行回顾性分析。结果经均手术切除及病理学检查，本组病例中乳腺囊性上皮增生症162例，积乳性乳腺囊肿26例，单纯性乳腺囊肿23例，乳腺叶状囊肉瘤5例，大导管内乳头状瘤4例。结论乳腺囊性肿块具有一定的共性和个性特点，术前B超检查和诊断性穿刺对乳腺囊性肿块的诊断和鉴别诊断有一定价值，但确诊有赖于病理学检查，手术可以明确诊断和治愈疾病。
ObjectiveTo explore the diagnostic value of exhaled volatile organic compounds (VOCs) for cystic fibrosis (CF). MethodsA systematic search was conducted in PubMed, EMbase, Web of Science, Cochrane Library, CNKI, Wanfang, VIP, and SinoMed databases up to August 7, 2024. Studies that met the inclusion criteria were selected for data extraction and quality assessment. The quality of included studies was assessed by the Newcastle-Ottawa Scale (NOS), and the risk of bias and applicability of included prediction model studies were assessed by the prediction model risk of bias assessment tool (PROBAST). ResultsA total of 10 studies were included, among which 5 studies only identified specific exhaled VOCs in CF patients, and another 5 developed 7 CF risk prediction models based on the identification of VOCs in CF. The included studies reported a total of 75 exhaled VOCs, most of which belonged to the categories of acylcarnitines, aldehydes, acids, and esters. Most models (n=6, 85.7%) only included exhaled VOCs as predictive factors, and only one model included factors other than VOCs, including forced expiratory flow at 75% of forced vital capacity (FEF75) and modified Medical Research Council scale for the assessment of dyspnea (mMRC). The accuracy of the models ranged from 77% to 100%, and the area under the receiver operating characteristic curve ranged from 0.771 to 0.988. None of the included studies provided information on the calibration of the models. The results of the Prediction Model Risk of Bias Assessment Tool (PROBAST) showed that the overall bias risk of all predictive model studies was high, and the overall applicability was unclear. ConclusionThe exhaled VOCs reported in the included studies showed significant heterogeneity, and more research is needed to explore specific compounds for CF. In addition, risk prediction models based on exhaled VOCs have certain value in the diagnosis of CF, but the overall bias risk is relatively high and needs further optimization from aspects such as model construction and validation.
ObjectiveTo investigate the diagnosis and treatment of pancreatic cystic neoplasm. MethodsThe clinical data of 40 cases of pancreatic cystic neoplasm from October 2001 to October 2013 in our hospital were retrospec-tively analyzed. ResultsPatients with pancreatic cystic neoplasm had no specific clinical feature. Ultrasonography and computed tomography displayed a cystic tumor in 57.5%(23/40) and 72.5%(29/40) of all patients, respectively, but could not distinguish the histological types. All of the patients had been operated, among them 2 cases were misdia-gnosed as pseudocysts and internal drainage; another 38 patients were undergoing the distal pancreatectomy. Pathologic examination results after operation showed 23 cases of serous cystadenoma, 9 cases of mucinous cystadenoma, 3 cases of intraductal papillary mucinous adenoma, and 5 cases of mutinous cystadenocarcinoma. Thirty five patients were followed-up. The followed-up time range from 2 months to 8 years, verage(74.2±12.8) months. Among the 3 patients with mucinous cystadenocarcinoma, 1 patient alived with no evidence of recurrence, the other 2 patients died of tumor invasion and metastasis in 4 months and 7months after operation. The others were still alive now with no evidence of recurrence. ConclusionSurgical resection is the most effective treatment for pancreatic cystic tumor, even if the patients with no any symptoms.