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Objective:To investigate the clinical and pathological features and improve the acknowledgement of intraductal tubulopapillary neoplasm (ITPN) of pancreas.Methods:Six cases with ITPN in the Shandong Provincial Hospital Affiliated to Shandong First Medical University combined with 40 cases from PubMed and CNKI were retrospectively analyzed. There were 25 males and 21 females, aged (58.6±16.0) years. The clinical manifestations, pathological features, treatment and so on were analyzed.Results:All cases were treated with surgery. The main clinical symptoms were upper abdominal pain and discomfort (23 cases, 50.0%), followed by jaundice (9 cases, 19.6%). Seven cases (15.2%) had no clinical symptoms. Three cases (6.5%) had low back discomfort, chills and other rare symptoms, and 4 cases (8.7%) had no clinical symptoms mentioned in the literature. Tumors of 27 cases (58.7%) located in the head of the pancreas, 9 cases (19.6%) in the body and tail, 4 cases (8.7%) in the whole pancreas, 3 cases (6.5%) in the body, 2 cases (4.3%) in ampulla and 1 case (2.2%) in head and body. Most of the tumors located in the main pancreatic duct. Microscopically, back-to-back tubular glands were densely arranged, parts of them with papillary structure, with obvious cell atypia and many mitoses. Twenty-two cases (47.8%) of ITPN were completely confined to the pancreatic duct, and 24 cases (52.2%) were with associated invasive carcinoma. Tumor cells were positive for cytokeratin 7 and mucin 1, but negative for cytokeratin 20, synaptophysin, chromogranin and trypsin. Mucin 2, mucin 5AC and mucin 6 were negative in most cases. Ki-67 ranged from 10% to 70%.Conclusion:Pancreatic ITPNs were mostly located in the pancreatic head, confined to the main pancreatic duct, and were mostly manifested as pain and discomfort in the upper abdomen. Surgery was the main treatment. Tumors cells of ITPN were arranged in tubular and papillary, with severe epithelial atypia and special immunophenotype, parts of cases accompanied with associated invasive carcinoma.
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Objective:To study the clinical, imaging and pathological features of duodenal gangliocytic paraganglioma (DGP).Methods:The clinical, imaging and pathological data of patients with DGP treated at the Shandong Provincial Hospital Affiliated to Shandong First Medical University from January 2012 to October 2021 were retrospectively analyzed.Results:Of 8 patients with DGP, there were 7 males and 1 female, with a median age of 52 years (range 37 to 57 years). Five patients were asymptomatic and they were diagnosed on physical examination followed by investigations. Three patients presented with black stools. CT examination showed localized nodular thickening of the duodenum, with enhanced scanning showing obvious progressive contrast enhancement. Endoscopic ultrasonography showed a hypoechoic submucosal lesion in duodenal wall. Histologically, the neoplasm composed of three different cell types which included Schwann cells, epithelioid cells, and ganglioid cells. The Schwann cells expressed NF, NSE and S-100 proteins; the epithelioid cells expressed CK, NSE, Syn and CgA proteins; while the ganglioid cells expressed NSE, Syn, CgA and NF proteins. Endoscopic submucosal dissection was performed in 2 patients and surgical resection was performed in 6 patients.Conclusion:DGP is a rare benign neurogenic tumor which is most commonly found in the duodenum. It has a good prognosis. Imaging and endoscopic examinations demonstrated a submucosal mass. The main treatment are endoscopic resection and local surgical resection.
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OBJECTIVE@#To build information repository of the carrying rate of neonatal deafness gene in Shijiazhuang.@*METHOD@#Blood samples were collected from the heel in 3-days neonates. Mutations of the deafness related genes were detected by the method of fluorescent PCR. Neonates received the detection of 6 mutation sites from 3 genes, including GJB2 (235delC, 299-300delAT), SLC26A4 (IVS7-2A> G, 2168A> G), mitochondrial DNA12S rRNA(1494C>T,1555A>G).@*RESULT@#There were 384 neonates who carried mutations among 421 subjects and the carrying rate was 4.08%, 158 (1.68%) newborns carried heterozygous mutations and 1 (0.01%) case carried homogeneous mutation of GJB2 (235 delC), 55 (0.58%) neonates carried heterozygous mutations of GJB2 (299-300delAT); 133 (1.41%) neonates carried heterozygous mutations and 1 (0.01%) homogeneous of SLC26A4(IVS7-2A>G),19 (0.20%) newborns carried heterozygous mutations of SLC26A4 (2168A>G). The numbers of neonates who carried homogeneous and heterogeneous mutation of mitochondrial 12S rRNA gene were 14 and 3 with carring rates of 0.15% and 0.03%. Two newborns were found to carry more than one mutation. One carried 235delC, IVS7-2A>G and 1555A>G and another carried 235delC and IVS7-2A>G.@*CONCLUSION@#The main mutational patterns were 235delC from GJB2 gene and IVS7-2A>G from SLC26A4 gene in Shijiazhuang newborns. The carrying rate information repository of neonatal deafness gene has been built preliminarily.