1.Ewing's sarcoma of central nervous system: a clinicopathological analysis of six cases.
Q Y YANG ; H LI ; C LIU ; M L BAO ; Q H FAN ; M H PAN
Chinese Journal of Pathology 2023;52(12):1249-1254
Objective: To investigate the clinicopathological characteristics, pathological diagnosis of Ewing's sarcoma of the central nervous system. Methods: Six cases of Ewing's sarcoma of the central nervous system diagnosed at the First Affiliated Hospital of Nanjing Medical University, Nanjing, China from 2015 to 2022 were collected. The clinical manifestations, histological morphology, immunophenotype and molecular genetics of these cases were analyzed. The related literature was reviewed. Results: There were four males and two females, with a male to female ratio of 2∶1. The onset age was 17-40 years, with a median age of 23 years. All 6 tumors were located in the spinal cord (2 cases of cervical vertebra, 1 case of thoracic vertebra, 2 cases of lumbar vertebra, and 1 case of sacral vertebra). The patients' clinical manifestations were mostly lumbago, weakness and numbness of lower limbs/limbs. In 1 case, the tumor recurred and metastasized to the suprasellar region and the third ventricle. Microscopically, the tumor showed diffuse infiltrative growth. In some cases, the tumor was closely related to the spinal meninges. The tumor cells were arranged in sheet, lobular, thin-rope, and nest-like patterns. Homer-Wright rosette was visible. The tumor cells were small to medium in size, and most of them had scant cytoplasm. A few cells had clear cytoplasm. Some areas were rhabdoid. The tumor cell nuclei showed focal mild pleomorphism. The chromatin was uniform and delicate while the nucleoli were not obvious. Mitosis was commonly seen. The tumor was separated by fibrous connective tissue and may be accompanied by mucinous degeneration. Immunohistochemistry showed that all tumors were positive for CD99, NKX2.2, Fli1, ERG. ATRX, H3K27me3, INI1 and BRG1 were all retained. Immunohistochemical stains for EMA, GFAP and Olig2 were negative. The Ki-67 proliferation index was 30%-70%. EWSR1 break-apart FISH test was positive. Conclusions: Ewing's sarcoma is rare in the central nervous system and needs to be distinguished from a variety of neoplasms with primitive undifferentiated small cell morphology. Immunohistochemistry and molecular genetics may be required for a proper diagnosis.
Humans
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Male
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Female
;
Young Adult
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Adult
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Adolescent
;
Sarcoma, Ewing/pathology*
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Proto-Oncogene Protein c-fli-1
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Immunohistochemistry
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Biomarkers, Tumor/genetics*
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Central Nervous System/pathology*
2.Burden of cardiovascular diseases attributable to metabolism disorders, in Jiangsu province.
H YU ; Z Q FAN ; P F LUO ; J SU ; R Q HAN ; J Y ZHOU
Chinese Journal of Epidemiology 2018;39(12):1596-1601
Objective: To quantify the burden of cardiovascular disease (CVD) deaths that attributed to metabolic disorders in population aged ≥25 years in Jiangsu province. Methods: The data we used were from the following three sources: 1) 2015 Jiangsu Chronic Disease Risk Factor and Nutrition Survey, 2) death surveillance, 3) results of the 2016 Global Burden of Disease Study, based on population attributable fractions (PAF), to analyze related parameters as mortality, years of life lost (YLL), life expectancy (LE) and premature mortality. Results: Most people died from ischemic stroke (IS) showed the standard mortality as 87.48/100 000. High SBP appeared as the major cause on CVD deaths. PAF with high cholesterol and high BMI decreased along with the increase of age while high fasting plasma glucose increased. Deaths due to ischemic heart diseases, IS or hemorrhagic stroke that attributed to metabolism disorders would reduce the LE by 1.08, 1.07 or 0.55 years, respectively. Males appeared to have higher YLL than females and were more likely to die from premature CVD, as the consequence of having metabolism disorders. Conclusions: Blood pressure control should be considered an important approach to reduce the burden of CVD. According to the characteristics of gender-related risks and the distinct impact of age-related metabolism disorders on different CVD diseases, stratified strategies should be strengthened for comprehensive prevention and control of CVD, in Jiangsu province.
Adult
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Blood Pressure
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Cardiovascular Diseases/epidemiology*
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Chronic Disease
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Cost of Illness
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Female
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Humans
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Life Expectancy
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Male
;
Metabolic Diseases/epidemiology*
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Mortality/trends*
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Mortality, Premature
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Risk Factors
3.Primary hepatic angiosarcoma: a clinicopathological analysis of nine cases.
S ZHAO ; Y ZHU ; S Y MA ; Q H FAN ; Q X GONG
Chinese Journal of Pathology 2023;52(11):1132-1137
Objective: To investigate the clinical manifestations, histomorphology, and differential diagnosis of primary hepatic angiosarcoma. Methods: Nine cases of primary hepatic angiosarcoma diagnosed in the Department of Pathology, the First Affiliated Hospital of Nanjing Medical University from January 2014 to December 2021 were collected, including biopsy and surgical specimens. The histomorphology, clinical, and radiologic findings were analyzed. The relevant literature was also reviewed. Results: There were six males and three females, aged 30 to 73 years (mean 57 years). Grossly, the growth pattern of the tumor was classified as either mass formation or non-mass formation (sinusoidal). Microscopically, the mass-forming primary hepatic angiosarcoma were further subdivided into vasoformative or non-vasoformative growth patterns; and those non-vasoformative tumors had either epithelioid, spindled, or undifferentiated sarcomatoid features. Sinusoidal primary hepatic angiosarcoma on the other hand presented with markedly dilated and congested blood vessels of varying sizes, with mild to moderately atypical endothelial cells. Follow-up in all nine cases revealed 8 mortality ranging from 1 to 18 months (mean 5 months) from initial diagnosis. One patient was alive with disease within a period of 48 months. Conclusions: Primary hepatic angiosarcoma is a rare entity with a wide spectrum of histomorphology, and often misdiagnosed. It should be considered when there are dilated and congested sinusoids, with overt nuclear atypia. The overall biological behavior is aggressive, and the prognosis is worse.
Male
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Female
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Humans
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Hemangiosarcoma/diagnosis*
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Endothelial Cells/pathology*
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Liver Neoplasms/surgery*
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Prognosis
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Biopsy
4.Traditional Chinese medicine for treatment of echinococcosis: a review.
Q CHEN ; C LIU ; C LI ; B ZHANG ; H FAN
Chinese Journal of Schistosomiasis Control 2023;35(4):398-406
Echinococcosis is a zoonotic parasitic disease caused by infection with Echinococcus species. As the drug of first choice for treatment of echinococcosis, albendazole suffers from problems of large doses and remarkable adverse reactions in clinical therapy. Development of novel drugs against echinococcosis is of urgent need. Recently, great advances have been achieved in the research on traditional Chinese medicine for treatment of echinococcosis. This review summarizes the progress of researches on traditional Chinese medicine for treatment of echinococcosis, aiming to provide insights into development of anti-echinococcosis drugs.
Animals
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Humans
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Medicine, Chinese Traditional
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Echinococcosis/parasitology*
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Albendazole/therapeutic use*
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Zoonoses
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Echinococcus multilocularis
5.Malignant peripheral nerve sheath tumor: a clinicopathological analysis.
W PENG ; Q X GONG ; Q H FAN ; Y LIU ; G X SONG ; Y Z WEI
Chinese Journal of Pathology 2023;52(9):924-930
Objective: To investigate the clinicopathological, immunophenotypic, and genetic features of malignant peripheral nerve sheath tumor (MPNST). Methods: Twenty-three cases of MPNST were diagnosed at the Jiangsu Province Hospital (the First Affiliated Hospital of Nanjing Medical University), China, between January 2012 and December 2022 and thus included in the study. EnVision immunostaining and next-generation sequencing (NGS) were used to examine their immunophenotypical characteristics and genomic aberrations, respectively. Results: There were 10 males and 13 females, with an age range of 11 to 79 years (median 36 years), including 14 cases of neurofibromatosis type I-associated MPNST and 9 cases of sporadic MPNST. The tumors were located in extremities (7 cases), trunk (4 cases), neck and shoulder (3 cases), chest cavity (3 cases), paraspinal area (2 cases), abdominal cavity (2 cases), retroperitoneum (1 case), and pelvic cavity (1 case). Morphologically, the tumors were composed of dense spindle cells arranged in fascicles. Periphery neurofibroma-like pattern was found in 73.9% (17/23) of the cases. Under low magnification, alternating hypercellular and hypocellular areas resembled marbled appearance. Under high power, the tumor cell nuclei were irregular, presenting with oval, conical, comma-like, bullet-like or wavy contour. In 7 cases, the tumor cells demonstrated marked cytological pleomorphism and rare giant tumor cells. The mitotic figures were commonly not less than 3/10 HPF, and geographic necrosis was often noted. Immunohistochemically, tumor cells were positive for S-100 (14/23, 60.9%) and SOX10 (11/23, 47.8%). The loss of the CD34-positive fibroblastic network encountered in neurofibromas was observed in 14/17 of the MPNST cases. The loss of H3K27me3 expression was observed in 82.6% (19/23) of the cases. Moreover, SDHA and SDHB losses were presented in one case. NGS revealed that NF1 gene loss of function (germline or somatic) were found in all 5 cases tested. Furthermore, four cases accompanied with somatic mutations of SUZ12 gene and half of them had somatic mutations of TP53 gene, while one case with germline mutation in SDHA gene and somatic mutations in FAT1, BRAF, and KRAS genes. Available clinical follow-up was obtained in 19 cases and ranged from 1 to 67 months. Four patients died of the disease, all of whom had the clinical history of neurofibromatosis type Ⅰ. Conclusions: MPNST is difficult to be differentiated from a variety of spindle cell tumors due to its wide spectrum of histological morphology and complex genetic changes. H3K27me3 is a useful diagnostic marker, while the loss of CD34 positive fibroblastic network can also be a diagnostic feature of MPNST. NF1 gene inactivation mutations and complete loss of PRC2 activity are the common molecular diagnostic features, but other less commonly recurred genomic aberrations might also contribute to the MPNST pathogenesis.
Female
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Male
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Humans
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Child
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Adolescent
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Young Adult
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Adult
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Middle Aged
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Aged
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Neurofibrosarcoma
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Neurofibromatosis 1
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Histones
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Genes, p53
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Nerve Sheath Neoplasms
6.Low-fat and fat-free spindle cell lipoma: a clinicopathological analysis of six cases.
H J HUA ; Q Y YANG ; M N LI ; Y LI ; Y DING ; Q H FAN ; H LI
Chinese Journal of Pathology 2023;52(10):1028-1030
7.Epidemiological characteristics of molecular transmission cluster among reported HIV/AIDS cases in Jiaxing city, Zhejiang province, 2017.
M Y LUO ; X H PAN ; Q FAN ; J F ZHANG ; R GE ; J JIANG ; W J CHEN
Chinese Journal of Epidemiology 2019;40(2):202-206
Objective: To understand the epidemiological characteristics of one large HIV molecular transmission cluster in Jiaxing city, Zhejiang province, 2017 in order to select those people under high-risk and providing basis for programs on prevention. Methods: During 2017, newly diagnosed HIV/AIDS cases in this city were recruited. Plasma samples were collected from subjects, followed by RNA extraction, RT-PCR and nest-PCR for pol gene amplification, before being sequenced and aligned. Mega 6.0 software was used to construct phylogenetic tree, and Cytoscape 3.6.0 software was used to identify HIV molecular transmission clusters. Cases within the large transmission clusters were investigated, using a field-epidemiology-questionnaire. Data related to socio-demographics and previous sexual behaviors were collected and EpiData 3.0 and SPSS 20.0 software were used. Results: In the large transmission cluster with subtype identified as CRF07_BC, in Jiaxing, 2017, 26 cases of the total 30 cases were investigated. A total of 80.8% (21/26) could be identified as newly infected within the last two years and 30.8%(8/26) could be identified as newly infected within the last one year, including 22 cases infected locally. Among several infected cases who were at age 45 years or older, they admitted that they had experienced unprotected sexual contacts in local city for long time and having had more than 10 disclosed sexual contacts within the last two years at the local venues. Conclusions: This molecular cluster had been formed and scaled up quickly in recent two years, it has played an important role in promoting and scaling up the HIV transmission. Three cases identificed as high risk played an importantrde role in scaling up this cluster.
Amplified Fragment Length Polymorphism Analysis
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China/epidemiology*
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Genes, pol
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Genotype
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HIV Infections/transmission*
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HIV-1/isolation & purification*
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Humans
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Molecular Epidemiology
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Phylogeny
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Polymerase Chain Reaction
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RNA, Viral/blood*
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Sexual Behavior
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pol Gene Products, Human Immunodeficiency Virus
8.Epidemiological characteristics and molecular typing of typhoid and paratyphoid in China, 2009-2013.
Y CAO ; Y Y HAN ; F F LIU ; Q H LIAO ; J LI ; B W DIAO ; F X FAN ; B KAN ; M Y YAN
Chinese Journal of Epidemiology 2018;39(3):337-341
Objective: To understand the epidemiological and molecular characteristics of typhoid and paratyphoid in China from 2009 to 2013, and provide evidence for the prevention and control of typhoid and paratyphoid, the development and improvement of surveillance strategies. Methods: Epidemiological analysis was conducted on the incidence data of typhoid and paratyphoid, and related public health emergencies in China during 2009-2013. Pathogen isolation and culture, serologic test were conducted for the typhoid and paratyphoid cases from 13 national surveillance sites. The isolates were subjected to antimicrobial susceptibility testing. Pulsed-field gel electrophoresis (PFGE) was performed for the molecular typing of these isolates. Results: The average incidence of typhoid and paratyphoid in China during this period was 1.03/100 000. The reported case number and incidence decreased with year. The provinces reporting high case numbers were Yunnan, Guizhou, Guangxi, Hunan, Zhejiang, Guangdong and Xinjiang. The incidence of age group 0-4 years was highest. The proportion of farmers and children outside child care settings showed an increasing tendency over time. The annual incidence peak was during July-August. Twenty five outbreaks occurred during 2009-2013. The results of pathogen isolation and culture showed that the positive rate was 3.00% (940/31 322), among the positive isolates, the proportion of Salmonella paratyphi A accounted for higher proportion (68.19%, 641/940) compared with Salmonella typhi (31.60%, 297/940). The drug resistances of Salmonella typhi and Salmonella paratyphi varied, but their resistances to nalidixic acid were highest (50.22% and 85.33%) respectively. A certain amount of Salmonella typhi isolates showed the resistance to the 3rd generation cephalosporins. PFGE analysis showed divergent patterns of Salmonella typhi compared with limited patterns of Salmonella paratyphi A. Conclusion: The epidemic level of typhoid and paratyphoid in China was relatively low, but the outbreak occurred occasionally. It is necessary to enhance the laboratory-based surveillance, particularly the capability of etiological diagnosis, outbreak investigation, response and antibiotic resistance monitoring, and conduct risk factor investigation in provinces with high incidences in recent years.
Child
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Child, Preschool
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China/epidemiology*
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Disease Outbreaks
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Drug Resistance, Bacterial/genetics*
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Electrophoresis, Gel, Pulsed-Field
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Epidemics
;
Farmers
;
Humans
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Incidence
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Infant
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Molecular Typing
;
Paratyphoid Fever/microbiology*
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Population Surveillance
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Salmonella paratyphi A/isolation & purification*
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Salmonella typhi/isolation & purification*
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Typhoid Fever/microbiology*