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Objective@#To investigate the clinicopathologic and genetic features, pathologic diagnosis and differential diagnosis of angiofibroma of soft tissue(AFST).@*Methods@#The clinicopathologic characteristics of 24 cases diagnosed at Fudan University Shanghai Cancer Center from 2011 to 2017 were analyzed; immunohistochemical staining and interphase fluorescence in situ hybridization (FISH) were performed, and the literatures were also reviewed.@*Results@#There were 15 male and 9 female (male∶female=1.7∶1.0) patients with age of onset ranging from 8 to 68 years (mean, 45 years). Fourteen cases occurred in extremities, including upper limbs (n=3) and lower limbs (n=11); seven cases were in the trunk, and 1 case each was in the temporal region, retroperitoneum and liver, respectively. Clinically, the tumors usually presented as a slowly growing painless mass. Tumor sizes ranged from 0.8 to 14 cm (mean 4.6 cm). Microscopically, most lesions were well-circumscribed, with fibrous capsules. Few cases infiltrated the surrounding fibrofatty tissue focally. The tumors were mainly composed of sparse short spindle cells and numerous small, branching, thin-walled blood vessels distributed in amyxoid, fibromyxoid or collagenous matrix, often accompanied by medium-sized, round or irregular and ecstatic vessels at the tumor periphery.By immunohistochemistry, all tested cases expressed vimentin (5/5), and showed variable positivity for EMA (2/4), ER (1/2), PR (2/3), α-SMA (1/18)and desmin (1/10). Ki-67 proliferation index were all less than 5%. CD34, CD31 and ERG staining clearly outlined the contours of blood vessels in the stroma. Four cases were tested for NCOA2 gene rearrangement by FISH, of which three were positive. Follow-up data was available in 17 patients (range, 3 to 69 months; mean, 30 months) were all free of disease.@*Conclusions@#Soft tissue angiofibroma is a benign fibroblastic neoplasm characterized by a prominent and complex vasculature set in a myxoid-to-collagenous stroma, and cytogenetically a distinctive NCOA2 gene rearrangement. Caution should be exercised for the possibility of potentially misinterpretation of AFST as vascular tumors and other myxoid soft tissue tumors.
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Objective@#To investigate the role of JAZF1 gene rearrangement in the diagnosis and differential diagnosis of endometrial stromal sarcomas by fluorescence in situ hybridization (FISH).@*Methods@#JAZF1 gene rearrangement was analyzed by FISH in 129 cases of ESS diagnosed from January 2008 to December 2016 including 105 cases of low-grade endometrial stromal sarcoma (LG-ESS), 21 cases of high-grade endometrial stromal sarcoma (HG-ESS) and 3 cases of undifferentiated uterine sarcoma (UUS). Sixteen cases of the related tumours in uterus were also collected as control group. The results were compared with our previous studies of JAZF1/JJAZ1 fusion gene in ESS by RT-PCR.@*Results@#Detection of JAZF1 gene rearrangement by FISH was successfully analyzed in 144 cases. JAZF1 gene alteration was detected in 63 cases, all of which were LG-ESS, with an overall positivity of 60.6% (63/104), while no JAZF1 gene rearrangement was found in all other cases. JAZF1 gene rearrangement was present in LG-ESS with classic histology (69.3%, 52/75), smooth muscle differentiation (2/10), sex cord-like differentiation (4/5), fibromyxoid change (1/5), clear cell change (0/1), skeletal muscle differentiation (0/1), and schwannoma-like palisading pattern (0/1). The different components in all the cases of LG-ESS with variant histology had the clonal origin, with or without JAZF1 gene alteration. Compared to the results of JAZF1/JJAZ1 fusion gene by RT-PCR, the positive rate of JAZF1 gene rearrangement in LG-ESS by FISH (61.9%, 26/42) was significantly higher than that of RT-PCR (30.0%, 12/40; P<0.01).@*Conclusions@#JAZF1 gene rearrangement is present only in LG-ESS, but not in HG-ESS, UUS or other related tumours in uterus. The frequency of JAZF1 gene rearrangement varies between classic LG-ESS and different morphologic variants. It is frequently, but not consistently, present in classic LG-ESS and less often positive in variant cases. The results of JAZF1 gene alterations in LG-ESS with different morphologic variants support the contention that the endometrial stromal and their variant morphologic components have the same clonal origin. Detection of JAZF1 gene rearrangement by FISH is very useful for the diagnosis and differential diagnosis of ESS.
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Objective:To explore the relations among screen-based sedentary behaviors ( SSB) , family factors and body mass index ( BMI) of children, and to study how family factors have effect on BMI through influencing SSB. Methods:A total of 1 846 students aged 7-11 years from 12 primary schools in one district of Beijing were included. Their body weight and height were measured to calculate the BMI. The time of SSB and family factors were investigated by using questionnaires. The time of SSB was the total time of watching TV and videos, playing computer games and iPad each day during the past 7 days recalled by children. The family factors included the parents’ education, occupation, the parents’ time of SSB, whether the parents told their child the harm of SSB, the parents’ time limit for the children’ s SSB. The parents’ education and occupation were used for calculating the family socioeconomic score. Results:The median time of SSB for children was 1 hour/day, and the interquartile range was 1 hour/day. The BMI of the children with the parents’ time limit for the children’ s SSB less than 120 min/day were smaller than the children with the parents’ time limit not less than 120 min/day, in both the boys (1. 63 kg/m2,P<0. 001) and the girls (0. 85 kg/m2, P=0. 004). The family socioeconomic score, the parents’ SSB time, whether the parents told their children the harm of SSB were not related to the children’ s BMI . The mediation effects of SSB time for children on the association between the parents’ time limit for the children’s SSB and BMI were-0. 222 kg/m2(95%CI:-0. 432, -0. 095) for boys and-0. 187 kg/m2 (95%CI: -0. 507, -0. 049) for girls , which accounted for 13. 67% of the total effects for boys and 22. 11% for girls. Conclusion:The parents’ time limit for the children’ s SSB has effect on their BMI through influencing their SSB time. Parents’ supervision on the behaviors of children produces larger benefit for BMI than health education conveyed by parents. Therefore, parents’ participation in su-pervising the behaviors of children are indispensable for preventing and controlling childhood obesity.