OBJECTIVETo investigate the clinicopathological characteristics, diagnosis, treatment and prognosis of gastric hepatoid adenocarcinoma(HAS).

METHODSRetrospective analysis of clinicopathological data of 24 cases with gastric HAS diagnosed by surgery and pathology in Chinese PLA General Hospital from January 2013 to May 2016 were carried out. All the patients underwent preoperative serum alpha fetoprotein (AFP), carcinoembryonic antigen (CEA), endoscopy and imaging examination (CT or B-mode ultrasonography), and those with elevated AFP were excluded from liver cancer, cirrhosis, endodermal sinus tumor and other diseases. The follow-up ended in June 2016 or the death of the patient. The survival was from the operation to the death of the patient or the end of follow-up.

RESULTSThere were a total of 24 cases with gastic HAS, accounting for 1.03%(24/2 326) of the total number of patients with gastric cancer diagnosed at the same time in our center. There were 19 males and 5 females, the ratio of male to female was 3.8:1.0, the mean age of the patients was 55.9 (31 to 72) years, and 2 of them had liver metastasis. The first symptom in 50% (12/24) patients was epigastric pain, in 25%(6/24) was abdominal distension with vomiting, and the rest included dysphagia (12.5%, 3/24), hematemesis (8.3%, 2/24) and melena (4.2%, 1/24). Preoperative serum levels of AFP and CEA were elevated in 10 (41.7%) and 5 patients (20.8%) respectively. All the patients underwent surgical treatment, including 23 cases with D2 radical resection of gastric cancer and R0 resection, and the other of palliative gastrojejunostomy. Lesions of HAS located in gastric antrum in 11 cases (45.8%), in cardia in 7 cases (29.2%), and in gastric body in 6 cases (25%). Tumor maximum diameter of 10 cases was larger than 5 cm, and the average diameter was 5.7(1.0 to 12.0) cm. Postoperative pathological TNM staging: stage I(b was in 1 cases, stage II( in 7 cases, stage III( in 14 cases, stage IIII( in 2 cases; lymph node metastasis in stage N1-3 in 20 cases (83.3%). Poorly-differentiated tumors were found in 21 cases (87.5%), and no well-differentiated tumors were found. There were 11 cases (45.8%) with formation of intravascular tumor thrombi. In immunohistochemistry, AFP positive expression was found in 11 cases(45.8%). Seventeen HAS cases (70.8%, 17/24) received postoperative SOX(oxaliplatin + S-1) or XELOX (oxaliplatin + capecitabine) as adjuvant chemotherapy, 5 cases postoperative immune therapy, and 2 cases postoperative traditional Chinese medicine. All the patients were followed up for 0.7 to 42.0 months (median 8 months). A total of 9 patients died, of whom, 1 case underwent gastrojejunostomy because of liver and peritoneal metastasis before operation, and died 20 days after surgery; 4 cases died of multiple liver metastases after 1.5 to 12.0 months with survival of 3 to 18 months; 3 cases presented local recurrence after 2 to 17 months with survival of 6 to 22 months; 1 cases had peritoneal metastasis after 12 month with survival of 26 months.

CONCLUSIONSHAS is a rare type of gastric cancer with poor prognosis. The diagnosis is mainly based on histopathology, and radical resection is the mainstay treatment.

Objective: To study the clinicopathologic features of lymphoproliferative disease by lymph node core needle biopsy(CNB)and to evaluate the diagnostic significance of CNB for lymphoproliferative disease. Methods: The annual distribution, entity constitute, clinical finding, gross feature, morphologic change, affiliate study and repeat biopsy diagnosis of 1 013 cases of lymph node CNB diagnosed at West China Hospital of Sichuan University from January 2009 to December 2015 were investigated. Results: (1) Proportion of lymph node CNB in total amount of biopsy specimens increased from 0.2% in 2009 to 0.8% in 2015.(2) The study cohort included 471 lymphomas, 12 atypical lymphoid hyperplasia (ALH), 136 suspected lymphomas, 372 benign lesions, and 22 cases of descriptive diagnoses. The most common types were diffuse large B cell lymphoma and T-lymphoblastic lymphoma. (3) Majority of patients were adolescents and children younger than 20 years or the elderly older than 60 years. 53.1% CNB tumor specimen consisted of ≥4 tissue cores and 40.5% were >2 cm in length. (4) 104 CNB cases with previous history of excision biopsy was included 45 carcinomas(no metastatic carcinoma was found), 32 lymphomas for treatment observation.1/14 suspicious lymphomas, 1/1 ALH and 3/22 cases benign lesions were diagnosed as lymphoma by repeat biopsy respectively. (5) 217 CNB cases were diagnosed as lymphoma by subsequent CNB (70), or subsequent excision biopsy (147) including 78.5%(73/93) suspected lymphomas, 5/7 ALH and 32.3%(20/62)benign lesions. Conclusions Lymph node CNB has certain clinical indications, although limited for the diagnosis of lymphoproliferative disorders. Suspected lymphomas and ALH diagnosed by CNB should be followed by repeat tissue biopsy. For the benign lesions by CNB it does not rule out additional biopsy to further investigate the lesion.

OBJECTIVE: To summarize the clinical features and biological characteristics of Helsmoortel Van der Aa syndrome (HVDAS) due to hotspot mutations of the ADNP gene in order to facilitate early diagnosis. METHODS: Clinical data and result of genetic testing for a girl with HVDAS due to hotspot mutation of the ADNP gene was summarized. Related literature was also reviewed. RESULTS: The patient, a 2-year-old girl, had presented with growth retardation, facial dysmorphism, psychomotor and language delay and recurrent respiratory infections. Whole exome sequencing revealed that she has harbored a heterozygous c.2496_2499delTAAA (p.Asn832Lysfs*81) variant of the ADNP gene, which was not found in either of her parents. CONCLUSION Although the typical features of the HVDAS have included intellectual disability and autism spectrum disorders, growth retardation and premature primary tooth eruption may also be present. In addition, the phenotypic difference among individuals carrying hot spot variants of the ADNP gene was not prominent.
Humans ; Female ; Child, Preschool ; Intellectual Disability/genetics* ; Homeodomain Proteins/genetics* ; Nerve Tissue Proteins/genetics* ; Abnormalities, Multiple/genetics* ; Mutation ; Rare Diseases ; Growth Disorders/genetics*

Twelve compounds were isolated from the ethanol extract of Fructus Gleditsiae Abnormalis by macroporous resin, silica gel, Sephadex LH-20, MCI and ODS column chromatographies. Their structures were identified on the basis of physicochemical properties and spectral data as gleditsioside A(1), gleditsioside B(2), gleditsioside H(3), gleditsioside I(4), gleditsioside J(5), gleditsioside K(6), gleditsia saponins C′(7), tamarixetin-7-O-β-D-glucopyranoside(8), neohesperidin(9), chrysoeirol-7-O-neohesperidoside(10); syringaresinol- O-β-D-glucopyranoside(11), liriodendrin(12). Compounds 8-12 were firstly isolated from this genus.

Objective: To explore the clinical effect and safety of minor liver resection for hilar cholangiocarcinoma (HC) of Bismuth-Corlette type Ⅲ and Ⅳ. Methods: From May 2007 to May 2017, the clinical data of 108 patients with Bismuth-Corlette type Ⅲ and Ⅳ HC underwent hepatectomy were collected and analyzed retrospectively.There were 56 males and 52 females, aged (57.2±5.3) years (ranged 48-76 years) .Among the 108 cases, there were 51 cases of type Ⅲa, 40 cases of type Ⅲb and 17 cases of type Ⅳ. Small-scale hepatectomy (≤3 hepatectomy) was performed in 70 cases, including 8 cases of 4b segment resection, 28 cases of 4b segment+5 segment resection, and 34 cases of partial 4 segment+partial 7 segment+partial 1 segment resection. Large-scale hepatectomy was performed in 38 cases (>3 segments) , of which 30 cases were treated with 2 segments+3 segments+4 segments+1 segment, and 8 cases were treated with 5 segments+7 segments+8 segments+1 segment. t′ test was used to analyze the data which did not conform to the normal distribution, and χ² test was used to calculate the incidence of postoperative complications and the 1, 3, and 5-year cumulative overall survival rate. Results: (1) The operation time of minor liver resection group ((180±25)minutes) was shorter than that of major liver resection group ((210±35)minutes) (t′=4.676, P<0.05) , the amount of blooding operation time of minor liver resection group ((310±80)ml) was less than that of major liver resection group ((500±110)ml)in the operation (t′=9.385, P<0.05) , and the difference was statistically significant. (2) The incidence of complications was lower in minor liver resection group and major liver resection group, and the difference was statistically significant (χ²=5.230, P<0.05) . (3) The actual 1-, 3- and 5-year survival rates were 87.1%, 58.4%, 30.0% and 84.2%, 57.9%,31.6%, respectively. There were no significant differences in survival rates in two groups in 1-, 3- and 5-year survival rates (χ²=0.177, P=0.674; χ²=0.005, P=0.946; χ²=0.029, P=0.865) . Conclusions Compared to patients with major liver resection, Minor liver resection for selected patients with HC of Bismuth-Corlette Ⅲ and Ⅳaccording to our criteria achieved better long-term outcomes. Chen′s biliojejunostomy is a simple, effective and safe method, which can be widely used when there are multiple biliary intestinal anastomosese.