In recent years,new advances have been achieved for the research on the pathogenesis,diagnosis,and treatment of primary biliary cholangitis(PBC).Accurate diagnosis,prognosis assessment,and risk stratification based on the clinicopathological features of patients are important for the treatment of PBC.This article summarizes the advances in the clinicopathological features and treatment of PBC.

Nine cases of mesenteric desmoid-type fibromatosis were diagnosed and treated in Taizhou Hospital, Wenzhou Medical University between January 2010 and May 2022, including 2 females and 7 males, aged 16 to 59 years. The lesions were in the mesentery of small intestine with 7 cases, ileocecal junction with 1 cases and transverse colon with 1 case. The tumors had an unclear boundary and no envelope, the section was solid, gray and tough. The mean maximum diameter was (10.7±8.5) cm (range 3.5-33.0 cm). Microscopically, fusiform fibroblasts and myofibroblasts were parallel, bunched or staggered, buried in a large amount of extracellular collagen. The cell morphology was relatively consistent, without obvious atypia, and mitosis was rare. Immunohistochemistry showed that the tumor cells were positive for vimentin (9/9), β-catenin (9/9), while smooth muscle actin (5/9) stains were focally positive. Ki-67 proliferation index was 1%-10%. Cytokeratin Pan, S-100, STAT6, CD117, DOG1, CD34, desmin and anaplastic lymphoma kinase stains were negative. Genetic analysis showed that there were 7 cases of c.121G>A(p.Thr41Ala) mutation of CTNNB1 gene, 1 case of c.121G>A(p.Thr41Ala) and 1 case of c.134C>T(p.Ser45Phe) double mutation, and 1 case of wild type. Tumors were surgically resected in all 9 cases. Eight cases had no recurrence or metastasis, 1 case had recurrence 6 months later, and no recurrence or metastasis after additional surgical resection.
Male ; Female ; Humans ; Fibromatosis, Aggressive/diagnosis* ; Immunohistochemistry ; Fibroblasts/metabolism* ; Mesentery/pathology* ; beta Catenin/analysis*

O bjective To understand the effects and clinical significance of the 2019 guidelines for the human epidermal growth factor receptor 2 (HER2) detection. Methods:According to the 2014 guidelines, 548 cases of invasive breast cancer with equivocal HER2 (2+) detected by immunohistochemistry (IHC) in Taizhou Enze Medical Center, Zhejiang Province, China from 2013 to 2019 were selected. The results of IHC and HER2/CEPl7 double-probe were reevaluated and divided into groups according to the 2019 guidelines for the comparative analysis.Results:Among the 548 IHC HER2 (2+) invasive breast cancers, the number of positive, equivocal and negative cases for HER2 were 96 (17.52%), 81 (14.78%) and 371 (67.70%), respectively, according to the 2014 guidelines. However, according to the 2019 guidelines, 10 cases (1.82%) were reclassified as IHC 1+, 2 cases in the group 2 were reclassified as negative, and all the originally equivocal cases in group 4 were reclassified as negative. Finally, the total number of positive and negative cases for HER2 were 94 (17.15%) and 454 (82.85%), respectively.Conclusions:After applying the 2019 guidelines, the number of IHC 2+ cases decreases, and the positive rate for HER2 also decreases slightly due to the reevaluation change in groups 2 and 4, leading to reclassification of the cases that were deemed equivocal according to the 2014 guidelines. In general, the new 2019 guidelines are more reasonable and easier to use.

Objective: To study the clinicopathologic characteristics, immunophenotype and ALK gene alterations of gastrointestinal inflammatory myofibroblastic tumor. Methods: Clinical data, histological features and immunohistochemical results were analyzed in 7 cases of gastrointestinal inflammatory myofibroblastic tumor at Zhejiang Province Taizhou Hospital from January 2005 to December 2016. ALK gene status was investigated by ALK fluorescence in situ hybridization. Results: There were 4 female and 3 male patients. The age of patients ranged from 1 to 72 years (median age=53 years and mean age=40 years). The tumor was located in stomach (n=4), left hemicolon (n=1), right hemicolon (n=1) and rectum (n=1). Histologically, the tumors consisted of spindle fibroblast and myofibroblast cells growing in bundles with inflammatory infiltration primarily composed of plasma cells and lymphocytes. Immunohistochemical study showed spindle tumor cells were positive for vimentin (7/7), SMA (7/7), but were negative for CD34, CKpan, CD117, DOG1, S-100 and desmin. Two cases expressed ALK protein and fluorescence in-situ hybridization revealed the presence of ALK gene rearrangement in the both cases. Conclusions Gastrointestinal inflammatory myofibroblastic tumor is a rare neoplasm that is easily misdiagnosed. Its surgical removal is a reliable treatment. ALK may be a potential novel therapeutic target for inflammatory myofibroblastic tumor.

Objective To investigate the expression of paired box 2 (Pax2) and E-cadherin in human renal cell carcinoma (RCC) and their correlation with clinicopathologic parameters.Methods The RCC tissue microarrays containing 85 renal cell carcinoma specimens and 35 normal kidney tissue specimens were used to detect Pax2 and E-cadherin expressions by immunohistochemistry.Results The positive expression rate of Pax2 was 77.6％ (66/85) in RCC specimens,which was significantly higher than that in 35 normal kidney tissue specimens (P ＜ 0.01).The positive expression rate of E-cadherin was 30.6％ (26/85) in RCC specimens,which was significantly lower than that in 35 normal kidney tissue specimens (P ＜ 0.01).The expression of Pax2 in RCC was significantly related to histological classification and pathological grade (P ＜ 0.05),while it was not related to the size of tumor and clinical stage (P ＞ 0.05).The expression of E-cadherin in RCC was significantly related to the size of tumor,histological classification,pathological grade and clinical stage (P ＜ 0.05).The positive expression rate of E-cadherin in survival group was significantly higher than that in death group (P ＜ 0.05).Conclusions The abnormal expressions of Pax2 and E-cadherin may play a crucial stage in development of human RCC.Detection of the expressions of Pax2 and E-cadherin can be used in the evaluation of malignant degree and prognosis of RCC.

Objective To investigate the expression of epidermal growth factor receptor (EGFR) and multidrug resistance protein, and it's relationship with efficacy of chemotherapy in NSCLC.MethodsThe immunohistochemical (EnVision method) was used to detect the expression of EGFR and LRP, P-gp, MRP, GST-π in 95 cases of NSCLC tissues and 15 cases of normal lung samples.ResultsEGFR in NSCLC tissues was significantly correlated with the history of smoking, pathological types of cancer(squamous cell carcinoma vs adenocarcinoma), presence of lymph node metastasis, and TNM stages (P< 0.05).The positive expression rate of LRP and MRP in adenocarcinoma were higher than squamous cell Carcinoma (P< 0.05), and MRP in high differentiate cancer was higher than low differentiate cancer (P< 0.05).The expression of MRP and LRP, PGP and GST-π in NSCLC were positive correlation(r=0.341, P=0.001;r=0.213, P=0.038), there is no correlation between EGFR with LRP, P-gp, MRP, GST-π.EGFR-negative chemotherapy efficiency (62.5%) was significantly higher than EGFR-positive(37.5%, P< 0.05).ConclusionEGFR and multi drug resistance proteins are overexressed in NSCLC tissues.multi drug resistance proteins have a synergistic to drug resistance.EGFR could be used to objectively predict chemotherapy effect and prognosis of NSCLC.

Objective: To investigate the clincopathologic and immunohistochemical features of gastric glomus tumors and their differences from gastric neuroendocrine neoplasms. Methods: Six cases of gastric glomus tumors, 8 cases of glomus tumors in other sites and 7 cases of gastric neuroendocrine neoplasms were collected from the Department of Pathology, Taizhou Hospital. The clinicopathological and immunohistochemical characteristics of these tumors were analyzed retrospectively. Results: The gastric glomus tumors were located in the muscularis propria of the antrum and most cases strongly expressed synaptophysin (5/6). However, no synaptophysin expression was seen in glomus tumors of other organs.Most gastric neuroendocrine neoplasms were located in the mucosa or submucosa of the fundus and corpus. In addition to the strong expression of synaptophysin (7/7), CgA (6/7) and CD56(5/7) were strongly positive, although SMA was negative. Conclusions Gastric glomus tumors and neuroendocrine neoplasms have similar morphological characteristics and both show strongly expression of synaptophysin. However, the location and immunohistochemical characteristics of gastric glomus tumors differ from those of the neuroendocrine neoplasms.

OBJECTIVETo investigate neuroendocrine differentiation and Wilms' tumor protein-1 (WT-1) expression in breast mucinous carcinoma and their clinicopathological significance.

METHODSThe clinicopathological data of 65 patients with breast mucinous carcinoma, including 31 cases of mixed mucinous carcinoma, 23 cases of hypocellular pure mucinous carcinoma and 11 cases of hypercellular pure mucinous carcinoma, admitted in Taizhou Hospital from January 2010 to June 2015 were retrospectively reviewed. The expression of neuroendocrine markers and WT-1 was detected by immunohistochemistry staining in all cases.

RESULTSThe mixed mucinous carcinomas and hypercelluar pure mucinous carcinomas had higher incidence of axillary lymph node metastasis and human epidermal recepter 2 (HER-2) positive than hypocellular pure mucinous carcinoma (all (P<0.01). However, the difference was not significant between mixed mucinous carcinomas and hypercellular pure mucinous carcinomas (all P>0.05). The expression of neuroendocrine marker was stronger in hypercellular mucinous carcinoma than that in mixed mucinous carcinoma and hypocellular mucinous carcinoma (all (P<0.05), but the difference was not statistically significant between mixed mucinous carcinoma and hypocellular pure mucinous carcinoma (P>0.05). The expression of WT-1 was weaker in mixed mucinous carcinoma than that in hypercellular and hypocellular pure mucinous carcinoma(all (P<0.05), but the difference was not statistically significant between hypercellular and hypocellular pure mucinous carcinoma (P>0.05). The mucinous carcinomas with lymph node metastasis had lower expression of neuroendocrine markers than those without lymph node metastasis ((P<0.01). The expression of WT-1 in breast mucinous carcinoma with lymph node metastasis trended lower than that in those without lymph node metastasis, but the difference was not statistically significant (P>0.05).

CONCLUSIONHypercellular pure mucinous breast carcinoma has higher rates of lymph node metastasis and HER-2 amplification than hypocellular pure mucinous carcinoma, the sub-classification of breast pure mucinous carcinoma should be considered. Neuroendocrine differentiation and WT-1 expression may be helpful in distinguishing the subtypes of breast mucinous carcinoma. Breast mucinous carcinoma with neuroendocrine differentiation trends to have less lymph node metastasis.

Adenocarcinoma, Mucinous ; classification ; diagnosis ; pathology ; Axilla ; Breast Neoplasms ; classification ; diagnosis ; pathology ; Female ; Humans ; Immunohistochemistry ; Incidence ; Lymph Nodes ; pathology ; Lymphatic Metastasis ; Neuroendocrine Tumors ; diagnosis ; pathology ; Receptor, ErbB-2 ; metabolism ; Retrospective Studies ; WT1 Proteins ; metabolism

Bone Neoplasms ; pathology ; Carcinoma, Neuroendocrine ; pathology ; Humans ; Osteosarcoma ; pathology ; Ureter ; pathology ; Ureteral Neoplasms ; pathology

OBJECTIVETo study the prevalence of IgG4-positive plasma cells in Rosai-Dorfman disease and to assess the association between Rosai-Dorfman disease and IgG4-related sclerosing disease (IgG4-SD).

METHODSThe clinicopathologic features of 12 tissue samples of Rosai-Dorfman disease (11 extranodal and one nodal) from nine patients were reviewed. The degree of fibrosis and occlusive phlebitis was studied by HE staining. The expression of IgG4 and IgG in plasma cells were studied by immunohistochemistry (EnVision) and quantitatively analyzed by medical image analysis system.

RESULTSNine tissue samples showed different degree of fibrosis (four tissue samples were mild, one tissue sample was moderate and four tissue samples were severe) and two tissue samples showed occlusive phlebitis in the lesional tissue. Immunohistochemical study showed marked infiltration by IgG4-positive plasma cells (> 50 per high-power field) in four tissue samples, moderate infiltration (30 to 50 per high-power field) in two tissue samples, mild (10 to 29 per high-power field) in three cases and negative infiltration (< 10 per high-power field) in three tissue samples (P < 0.01). Three tissue samples fulfilled the diagnostic criteria of IgG4-SD (> 50 IgG4-positive plasma cells per high-power field and IgG4-to-IgG ratio > 40%), including one tissue sample each of Rosai-Dorfman disease in the left facial skin, above the left eye socket, and in the right parotid.

CONCLUSIONSSome cases of Rosai-Dorfman disease fulfill the diagnostic criteria and show the histologic features of IgG4-SD. They may represent members of the IgG4-SD spectrum. The detection of IgG4-positive plasma cells in the lesional tissues of Rosai-Dorfman disease may have clinical pathological significance.

Fibrosis ; Histiocytosis, Sinus ; diagnosis ; immunology ; Humans ; Immunoglobulin G ; chemistry ; Immunohistochemistry ; Phlebitis ; pathology ; Plasma Cells ; chemistry