Objective To investigate the efficacy of different radical surgical procedures for the treatment of hilar cholangiocarcinoma.Methods The clinical data of 207 patients with hilar cholangiocarcinoma who were treated at the Southwest Hospital from June 2007 to June 2012 were retrospectively analyzed.Local resection or hemihepatectomy combined with caudate lobectomy was applied to patients with Bismuth type Ⅰ hilar cholangiocarcinoma; dumbbell type radical resection was applied to patients with Bismuth type Ⅱ hilar cholangiocarcinoma or some patients with type Ⅲ a,Ⅲ b and Ⅳ hilar cholangiocarcinoma; hemihepatectomy or extended hemihepatectomy combined with caudate lobectomy was applied to patients with Bismuth type Ⅲ a,Ⅲ b and Ⅳ hilar cholangiocarcinoma.The patients were followed up every 3 months postoperatively till December 2012.All data were analyzed using the chi-square test or Fisher exact probability test,the survival curve was drawn by Kaplan-Meier method,and the survival was analyzed using the Log-rank test.Results Of the 207 patients,124 received radical resection,including 14 received local resection,23 received dumbbell type resection,87 received lobectomy + caudate lobectomy,49 received palliative resection; 34 received biliary drainage.Four patients died perioperatively.The incidences of complications of dumbbell type radical resection,left hemihepatectomy + caudate lobectomy,right hemihepatectomy + caudate lobectomy were 21.7％ (5/23),46.6％ (27/58) and 48.3％ (14/29),respectively.The incidence of complications after dumbbell type radical resection was significantly lower than left hemihepatectomy + caudate lobectomy and right hemihepatectomy + caudate lobectomy (x2 =4.42,3.90,P ＜ 0.05).One hundred and seventy patients were followed up.The median survival time of the 112 patients who received radical radical resection was 26.5 months,and the 1-,3-,5-year survival rates were 75.9％ (85/112),42.9％ (24/56) and 28.9％ (11/38),respectively.The median survival time of the 38 patients who received palliative resection was 8.5 months,and the 1-,3-year survival rates were 31.6％ (12/38) and 0.The survival time of 20 patients who received biliary drainage was 4.0 months,and the l-year survival rate was 0.The survival rate of patients who received radical resection was significantly higher than those who received palliative resection (x2=65.32,P ＜ 0.05).There was a significant difference in the survival rate between patients who received surgical treatment and those who received biliary drainage (x2=99.97,P ＜ 0.05).Of the 112 patients who received radical resection,the median survival time of 10 patients who received local resection of tumor was 47.0 months,the 1-year survival rate was 10/10,and 4 patients survived at the end of the follow-up; the median survival time of 23 patients who received dumbbell type radical resection was 32.0 months,and the 1-,3-year survival rates were 95.7％ (22/23) and 7/15,and the survival time of 6 patients was longer than 5 years; the median survival time of 54 patients who received left hemihepatectomy or extended left hemihepatectomy + caudate lobectomy was 27.6 months,and the 1-,3-year survival rates were 42.1％ (24/57) and 38.7％ (12/32),and the survival time of 9 patients was longer than 5 years,3 patients survived at the end of the follow-up ; the median survival time of 25 patients who received right hemihepatectomy or extended right hemihepatectomy + caudate lobectomy was 28.3 months,and the 1-,3-year survival rates were 45.8％ (11/24) and 6/15,and the survival time of 6 patients was longer than 5 years,2 patients survived at the end of follow-up.The median survival time of 35 patients (patients with Bismuth type Ⅰ,Ⅱ hilar cholangiocarcinoma and Bismuth Ⅲ a and Ⅲ b hilar cholangiocarcinoma which did not invade the secondary bile duct) who received hemihepatectomy + caudate lobectomy was 32.0 months,and the 1-,3-,5-year survival rates were 91.4％ (32/35),45.8％ (11/24) and 5/16,which were not different from the survival rate of patients who received dumbbell type radical resection (x2 =0.17,P ＞ 0.05).The 5-year survival rate of patients with lymph node metastasis was 4/19,which was significantly lower than 30.4％ (7/23) of patients without lymph node metastasis (x2 =23.40,P ＜ 0.05).Conclusion Joint lobectomy and standardized lymph node dissection could help to improve the efficacy of surgical treatment for patients with hilar cholangiocarcinoma.

OBJECTIVE: To explore the pathological characteristics and significance of RET proto-oncogene screening in multiple endocrine neoplasia type 2A (MEN2A) with cutaneous lichen amyloidosis (CLA). METHODS: Clinical data of 51 members from 7 unrelated pedigrees of MEN2A-CLA were collected. Systemic clinical investigations including biochemical testing, imaging examination, germline RET variant screening and histopathological examination were carried out. RESULTS: RET gene variants were detected in 28 patients with MEN2A (C634G/F/R/S/W and C611Y) including 12 males and 16 females, with the mean age of diagnosis being (41.1 ± 18.3) years old, which were consistent with their clinical manifestations. The incidence of medullary thyroid carcinoma (MTC), pheochromocytoma (PHEO), hyperparathyroidism (HPTH) and CLA among 28 MEN2A patients were 89.3%, 28.6%, 7.1% and 28.6%, respectively. Comparison of the incidence of MTC/PHEO/HPTH and CLA between C611Y and C634G/F/R/S/W, only PHEO and CLA in C611Y were lower than those in C634G/F/R/S/W (P < 0.05; P < 0.05). Among 8 patients with CLA, the male to female ratio was 2 : 6. The clinical features included pruritus in the interscapular region and presence of dry, thickened, scaly, brown pigment, clustered or desquamate-like plaques. The mean onset age of CLA [(18.4 ± 4.6) years] versus the mean age at diagnosis of CLA or MEN2A were significantly different (P < 0.001; P < 0.001). CONCLUSION MEN2A-CLA may be the early clinical manifestation of MEN2A and most frequently occurred along with RET-C634 variant. To facilitate the recognition of MEN2A-CLA, to combine family investigation and screening of RET variant are helpful for early diagnosis and standardized treatment, which can improve the long-term outcome of MEN2A-specific tumors.
Adolescent ; Adrenal Gland Neoplasms ; Adult ; Amyloidosis, Familial ; Carcinoma, Neuroendocrine ; China ; Female ; Humans ; Lichens ; Male ; Middle Aged ; Multiple Endocrine Neoplasia Type 2a/genetics* ; Pheochromocytoma ; Proto-Oncogene Proteins c-ret/genetics* ; Skin Diseases, Genetic ; Thyroid Neoplasms/genetics* ; Young Adult

Animal model of hydrocephalus is an important object to study the mechanism, pathological characteristics, and treatment of hydrocephalus. A stable and controllable animal model in accordance with clinical development of hydrocephalus can help to develope hydrocephalus related basic research and clinical translational application. According to the study purpose and genetic and physiological characteristics of experimental animals, a variety of animals have been used to establish different types of hydrocephalus animal models. The methods for congenital hydrocephalus models include gene edition and metabolic induction, while secondary hydrocephalus models can be induced by blocking the circulation of cerebrospinal fluid and interfering cerebrospinal fluid absorption. The hydrocephalus models constructed by different methods are also different in progression, neurofunctional changes, and histopathological characteristics. This paper reviews the construction methods and pathological characteristics of various hydrocephalus models in order to provide references for selection of animal models for hydrocephalus-related research.