Sclerosing epithelioid fibrosarcoma of the pancreas:A case report

BACKGROUND A sclerosing epithelioid fibrosarcoma(SEF)is a rare malignant fibroblastic soft tissue tumor that rarely occurs in intra-abdominal organs.A case of a SEF in the pancreatic head is reported herein,including its clinical manifestations,preoperative imaging features,gross specimen and pathological findings.CASE SUMMARY A 33-year-old male patient was admitted to Peking Union Medical College Hospital in December 2023 due to a one-year history of intermittent upper abdominal pain and the discovery of a pancreatic mass.The patient underwent an enhanced computed tomography scan of the abdomen,which revealed a welldefined,round mass with clear borders and calcifications in the pancreatic head.The mass exhibited progressive,uneven mild enhancement,measuring approximately 6.6 cm×6.3 cm.The patient underwent laparoscopic pylorus-preserving pancreaticoduodenectomy.Postoperative pathological examination revealed that the lesion was consistent with a SEF.At the 3-month postoperative follow-up,the patient did not report any short-term complications,and there were no signs of tumor recurrence.CONCLUSION SEFs are rare malignant fibrous soft tissue tumors.SEFs rarely develop in the pancreas,and its preoperative diagnosis depends on imaging findings,with confirmation depending on pathological examination and immunohistochemistry.Currently,only four cases of pancreatic SEF have been reported in studies written in English.This case is the first reported case of a pancreatic SEF by a clinical physician.

影响原发性腹膜后肿瘤手术患者预后的危险因素分析

背景原发性腹膜后肿瘤多为恶性肿瘤,其病因多种多样.分级、切除范围和肿瘤完整性等参数已被证明影响预后.目的探索影响原发性腹膜后肿瘤手术患者术后生存的危险因素.方法73例腹膜后肿瘤根治性手术的患者分为无瘤生存组(47例)、复发死亡组(26例).收集并分析影响患者术后无瘤生存的因素,包括年龄、肿瘤长径、淋巴结转移、首发症状、肿瘤类型、肿瘤分化程度以及是否合并其他系统疾病等.结果原发性腹膜后肿瘤术后5年无瘤生存率为64.4%(47/73).对影响原发性腹膜后肿瘤术后无瘤生存率的因素进行单因素分析结果显示:年龄(P<0.05),肿瘤长径(P<0.05),淋巴结转移(P<0.05),首发症状(P<0.05),肿瘤类型(P<0.05),肿瘤分化程度(P<0.05)共6项因素与腹膜后肿瘤术后无瘤生存率相关.多因素Cox回归分析结果提示首发症状(P<0.05)以及肿瘤类型(P<0.05)影响腹膜后肿瘤患者术后无瘤生存率的独立危险因素.结论首发症状以及肿瘤类型是影响腹膜后肿瘤患者术后无瘤生存率的关键.

SEVEN CASES OF EPITHELIAL OVARIAN CARCINOMA WITH BRAIN METASTASIS

Objective To summarize the clinical characteristics, treatment, and prognosis of brain metastasis in patients with epithelial ovarian carcinoma. Metbods Retrospective analysis was conducted in 7 cases of brain metastases of epithelial ovarian carcinoma from January 1986 to March 2007 in Peking Union Medical College Hospital for summarizing therapy results and prognosisaffecting factors. Results Incidence of brain metastases of epithelial ovarian carcinoma was about 0. 66% （7/1 055 ）. Serous adenocarcinoma was the predominant pathological type in 4 cases and the subsequent was adenocarcinoma in 3 cases. All the patients were diagnosed at late stage, 6 cases with the International Federation of Gynecology and Obstetrics （HGO） stage Ⅲc and 1 with FIGO stage IV. The mean duration from diagnosis of ovarian carcinoma to brain metastasis was 32.7 ± 20. 0 months （range, 23-73 months）. Single metastasis focus occurred in 43% of cases and multiple metastases in 57% of cases. Fifty-seven percent of patients presented extracranial metastasis. Serum CA125 played a role in monitoring reoccur- rence and brain metastases. The average survival time was about 12 months. Better treatment with prolonged survival could be achieved by combination of operation and chemotherapy or combination of radiotherapy with chemotherapy. Concltusions As a rare condition, brain metastasis of epithelial ovarian carcinoma is rising in incidence with improved treatment of ovarian carcinoma and prolonged survival. However, brain metastasis indicates bad prognosis which can be improved by combined therapy.

Mutations in GBA,SNCA,and VPS35 are not associated with Alzheimer's disease in a Chinese population:a case-control study

SNCA,GBA,and VPS35 are three common genes associated with Parkinson's disease.Previous studies have shown that these three genes may be associated with Alzheimer's disease(AD).However,it is unclear whether these genes increase the risk of AD in Chinese populations.In this study,we used a targeted gene sequencing panel to screen all the exon regions and the nearby sequences of GBA,SNCA,and VPS35 in a cohort including 721 AD patients and 365 healthy controls from China.The results revealed that neither common variants nor rare variants of these three genes were associated with AD in a Chinese population.These findings suggest that the mutations in GBA,SNCA,and VPS35 are not likely to play an important role in the genetic susceptibility to AD in Chinese populations.The study was approved by the Ethics Committee of Xiangya Hospital,Central South University,China on March 9,2016(approval No.201603198).

Novel ATL1 mutation in a Chinese family with hereditary spastic paraplegia: A case report and review of literature

BACKGROUND Hereditary spastic paraplegias (HSPs) refer to a group of heterogeneous neurodegenerative diseases characterized by lower limbs spasticity and weakness. So far, over 72 genes have been found to cause HSP (SPG1-SPG72). Among autosomal dominant HSP patients, spastic paraplegia 4 (SPG4/SPAST) gene is the most common pathogenic gene, and atlastin-1 (ATL1) is the second most common one. Here we reported a novel ATL1 mutation in a Chinese spastic paraplegia 3A (SPG3A) family, which expands the clinical and genetic spectrum of ATL1 mutations. CASE SUMMARY A 9-year-old boy with progressive spastic paraplegia accompanied by right hearing loss and mental retardation for five years was admitted to our hospital.Past history was unremarkable. The family history was positive, and his grandfather and mother had similar symptoms. Neurological examinations revealed hypermyotonia in his lower limbs, hyperreflexia in knee reflex, bilateral positive Babinski signs and scissors gait. The results of blood routine test, liver function test, blood glucose test, ceruloplasmin test and vitamin test were all normal. The serum lactic acid level was significantly increased. The testing for brainstem auditory evoked potential demonstrated that the right side hearing was impaired while the left was normal. Magnetic resonance imaging showed mild atrophy of the spinal cord. The gene panel test revealed that the proband carried an ATL1 c.752A>G p.Gln251Arg (p.Q251R) mutation, and Sanger sequencing confirmed the existence of family co-segregation. CONCLUSION We reported a novel ATL1 Q251R mutation and a novel clinical phenotype of hearing loss in a Chinese SPG3A family.

Nickel-catalyzed carbonylative four-component 1,4-dicarbofunctionalization of 1,3-enynes

An efficient Ni-catalyzed four-component 1,4-carbocarbonylation of 1,3-enynes with activated alkyl halides and arylboronic acids under atmospheric pressure of CO is presented.By tuning the electronic and steric effects of alkyl radicals,both electronrich and electron-deficient 1,3-enynes were compatible with this cascade.This protocol features mild conditions,broad substrate scope,excellent functional group compatibility and facile gram-scale synthesis,providing a practical approach to the quaternary carbon center-containing allenyl ketones.Mechanistic study revealed that the acyl-NiIIspecies plays an important role in both the coupling and the alkyl radical generation processes.

Photoinduced copper-catalyzed alkoxyl radical-triggered ring-expansion/aminocarbonylation cascade

A photoinduced copper-catalyzed alkoxyl triggered C-C bond cleavage/aminocarbonylation cascade is presented.Through adjusting the structure of alkoxyl radical precursors,functionalized lactones and ketoamides were synthesized with good yields and excellent functional group tolerance under redox-neutral conditions.Notably,this protocol enables the integration of lactone fragments with many amine drugs and drug fragments.

Global Phase Portraits of Uniform Isochronous Centers System of Degree Six with Polynomial Commutator

This paper studies the global phase portraits of uniform isochronous centers system of degree six with polynomial commutator.Such systems have the form x=-y+xf(x,y),y=x+yf(x,y),where f(x,y)=a_(1)x+a_(2)xy+a_(3)xy^(2)+a_(4)xy^(3)+a_(5)xy^(4)=xσ(y),and any zero of 1+a_(1)y+a_(2)y^(2)+a_(3)y^(3)+a_(4)y^(4)+a_(5)y^(5),y=y is an invariant straight line.At last,all global phase portraits are drawn on the Poincare disk.

Phosphodiesterase 10A inhibitor PF-2545920 as a prospective agent for the clinical promotion of sperm motility

Phosphodiesterase(PDE)inhibitors can improve sperm motility in patients with asthenozoospermia.However,the most commonly reported nonselective PDE inhibitor pentoxifylline and PDE5 inhibitor sildenafil have the disadvantages of requiring a high concentration and destroying sperm integrity.We examined the PDE10A inhibitor PF-2545920 to compare its ability to promote sperm motility with that of pentoxifylline and sildenafil.After seminal plasma was discarded,several semen samples were subjected to four treatments(control,PF-2545920,pentoxifylline,and sildenafil)to evaluate their ability to affect motility,viability,and spontaneous acrosome reactions.Intracellular calcium and adenosine triphosphate(ATP),mitochondrial membrane potential,and penetration through viscous medium were assessed by flow cytometry,luciferase,and hyaluronic acid after treatment with PF-2545920.Statistical analyses were performed using the analysis of variance statistical test.PF-2545920 elevated the percentage of motile spermatozoa compared to the control,pentoxifylline,and sildenafil groups at 10μmol l^(-1)(P<0.01).It is less toxic to GC-2spd mouse spermatocytes cells and spermatozoa and causes fewer spontaneous acrosomal reactions(P<0.05).PF-2545920 also increased mitochondrial membrane potential(P<0.001)and altered intracellular calcium(P<0.05)in a dose-dependent manner,including increasing sperm hyaluronic acid penetrating ability(P<0.05).Therefore,PF-2545920 might be an excellent choiceforstimulatingthe spermmotility.

Regioselective C-C bond cleavage/aminocarbonylation cascade under copper catalysis

A regioselective C-C bond cleavage/aminocarbonylation cascade is presented. In recent years, tremendous progress has been made in the alkoxyl radical-mediated C-C bond cleavage of unstrained monocarbocycles. In contrast, the deconstruction and functionalization of bicyclic skeletons has been less developed and has mainly focused on the ring expansion process. Inspired by the aromatization-driven C-C bond cleavage, here we demonstrate a ring-opening/aminocarbonylation cascade under copper catalysis, in which the formation of a stable γ-lactam or succinimide skeleton reverses the selectivity of C-C cleavage. Remarkably, the photo and thermal assistance is not required when the succinimide skeleton is formed during the ring opening process. DFT calculations revealed that this unexpected ring-opening process is thermodynamically and kinetically favourable.

Screening potential α-glucosidase inhibitors from Anemarrhena asphodeloides using response surface methodology coupled with grey relational analysis

Objective: To screen potential α-glucosidase inhibitors from Anemarrhena asphodeloides.Methods: Response surface methodology employing Box-Behnken design was used to optimize conditions for the extraction of α-glucosidase inhibitory active compounds from A. asphodeloides. The powders(20.0 g) of A. asphodeloides were extracted under the optimized conditions. The extract was applied to a D-101 macroporous resin column. It was eluted with ethanol and water to give six fractions. Compounds from the active fraction were identified by UPLC-Q-TOF-MS. The structure-activity relationship was discussed based on grey relational analysis.Results: The optimum extraction conditions were as follows: ethanol concentration, 100%; extraction temperature, 51 ℃; and solvent to solid ratio, 23 mL/g. It indicated that the active compounds were concentrated into 80% ethanol fraction. Twenty five steroid saponins from 80%ethanol fraction were identified by UPLC-Q-TOF-MS. Peaks 19 and 23 were tentatively identified as new structures. The predicted α-glucosidase inhibitory activities of the compounds were 7 > 2 > 1 > 22 > 23 > 3 > 9 > 21 > 24 > 4 > 13 > 8 > 14 > 16 > 17 > 25 > 6 > 19.Conclusion: The fraction eluted by 80% ethanol showed the best inhibitory activity. After analyzing the data of UPLC-Q-TOF-MS, 25 steroid saponins were tentatively identified in this fraction.

Solid-phase Decarburization Kinetics of High-carbon Ferromanganese Powders by Microwave Heating

Solid-phase decarburization of high-carbon ferromanganese powders （HCFPs） was conducted using calcium carbonate powders （CCPs） as a decarburizer by microwave heating. Solid-phase decarburization kinelSics was investi- gated by isothermal method. The results show that the HCFPs show excellent microwave absorption at a higher av- erage heating rate of 80 ℃/min, while CCPs exhibit poor microwave absorption at a lower heating rate of 5--20 ℃/min; the heating characteristics are in-between when HCFPs and CCPs are mixed. The average heating rates of the mix- ture are 32.14, 31.25, 31.43, and 30.77 ℃/rain when the mixture is heated up to 900, 1000, 1100, and 1200 ℃, respectively. The good microwave absorption property of the mixed material lays the foundation for the solid-phase decarburization of HCFPs containing CCPs. Solid-phase decarburization of HCFPs containing CCPs is a first-order reaction by microwave heating. Apparent activation energy of solid-phase decarburization is 55.07 kJ/mol, which is far less than that of ordinary carbon gasification reaction and that of solid-phase decarburization under the same de- carburization condition by conventional heating. It indicates that microwave heating not only produces thermal effect, but also has non-thermal effect.

Targeted inhibition of myeloid-derived suppressor cells in the tumor microenvironment by low-dose doxorubicin to improve immune efficacy in murine neuroblastoma

Background:High agglomeration of myeloid-derived suppressor cells(MDSCs)in neuroblastoma(NB)impeded therapeutic effects.This study aimed to investigate the role and mechanism of targeted inhibition of MDSCs by low-dose doxorubicin(DOX)to enhance immune efficacy in NB.Methods:Bagg albino(BALB/c)mice were used as tumor-bearing mouse models by injecting Neuro-2a cells,and MDSCs were eliminated by DOX or dopamine(DA)administration.Tumor-bearing mice were randomly divided into 2.5 mg/kg DOX,5.0 mg/kg DOX,50.0 mg/kg DA,and control groups(n=20).The optimal drug and its concentration for MDSC inhibition were selected according to tumor inhibition.NB antigen-specific cytotoxic T cells(CTLs)were prepared.Tumor-bearing mice were randomly divided into DOX,CTL,anti-ganglioside(GD2),DOX+CTL,DOX+anti-GD2,and control groups.Following low-dose DOX administration,immunotherapy was applied.The levels of human leukocyte antigen(HLA)-I,CD8,interleukin(IL)-2 and interferon(IFN)-γin peripheral blood,CTLs,T-helper 1(Th1)/Th2 cytokines,perforin,granzyme and tumor growth were compared among the groups.The Wilcoxon two-sample test and repeated-measures analysis of variance were used to analyze results.Results:The slowest tumor growth(F=6.095,P=0.018)and strongest MDSC inhibition(F=14.632,P=0.001)were observed in 2.5 mg/kg DOX group.Proliferation of T cells was increased(F=448.721,P<0.001)and then decreased(F=2.047,P=0.186).After low-dose DOX administration,HLA-I(F=222.489),CD8(F=271.686),Th1/Th2 cytokines,CD4^(+)and CD8^(+)lymphocytes,granzyme(F=2376.475)and perforin(F=488.531)in tumor,IL-2(F=62.951)and IFN-γ(F=240.709)in peripheral blood of each immunotherapy group were all higher compared with the control group(all ofP values<0.05).The most significant increases in the aforementioned indexes and the most notable tumor growth inhibition were observed in DOX+anti-GD2 and DOX+CTL groups.Conclusions:Low-dose DOX can be used as a potent immunomodulatory agent that selectively impairs MDSC-induced immunosuppression,thereby fostering immune efficacy in NB.

A Clinicopathological Study on Stage Ⅰ Ovarian Adult Granulosa Cell Tumors with Recurrence within 5 Years

To the Editor:Adult granulosa cell tumor (AGCT)of the ovary is a low-grade malignant,sex cord-stromal tumor with an indolent course and late recurrence.Recurrences usually occur more than 5 years after the first treatment.[1] However,a few cases of aggressive AGCT have been reported.[2,3] We retrospectively reviewed all 213 AGCT patients at Peking Union Medical College Hospital between 2000 and 2018 and identified six Stage I cases with recurrence <5 years.The Ethics Committee of Peking Union Medical College Hospital specifically approved this study (No.S-K413),and all included patients provided written informed consent to participate in the study.