Desmoid-type fibromatosis of the mesentery: a clinicopatho-logical and genetic analysis of 9 cases / 浙江大学学报·医学版

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.

Gastrointestinal inflammatory myofibroblastic tumor: a clinicopathologic study / 中华病理学杂志

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.

Gastric glomus tumors expressing synaptophysin: clinicopathologic and immunohistochemical analyses / 中华病理学杂志

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.

Neuroendocrine differentiation and Wilms' tumor protein-1 expression in breast mucinous carcinoma and their significance / 浙江大学学报·医学版

<p><b>OBJECTIVE</b>To investigate neuroendocrine differentiation and Wilms' tumor protein-1 (WT-1) expression in breast mucinous carcinoma and their clinicopathological significance.</p><p><b>METHODS</b>The 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.</p><p><b>RESULTS</b>The 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).</p><p><b>CONCLUSION</b>Hypercellular 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.</p>

Expression of IgG4 in Rosai-Dorfman disease and its significance / 中华病理学杂志

<p><b>OBJECTIVE</b>To 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).</p><p><b>METHODS</b>The 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.</p><p><b>RESULTS</b>Nine 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.</p><p><b>CONCLUSIONS</b>Some 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.</p>

Clinicopathological analysis of langerhans cell histiocytosis / 中华全科医师杂志

The clinicopathological data of 13 patients with Langerhans cell histiocytosis (LCH) from January 2004 to January 2014 were retrospectively analyzed.There were 8 males and 5 females with a mean age of 35 (6-63)years.For all 13 cases,there were 15 lesions.Histologically,there was a diffuse distribution of Langerhans cells accompanied by a variable number of eosinophils and some other inflammatory cells.Immunohistochemical study showed tumor cells were positive for S-100 protein,CD1a and Langerin.During a follow-up period of 6-101 months,all survived.The diagnosis of LCH was assisted by immunophenotypic analyses for S-100,CD1a and Langerin.Combination therapy was effective.Most patients had an excellent prognosis.