Primary hepatic neuroendocrine tumor with a suspicious pulmonary nodule:A case report and literature review

BACKGROUND Primary hepatic neuroendocrine tumors(PHNETs)are extremely rare tumors originating from neuroendocrine cells.Due to lack of neuroendocrine symptoms and specific radiographic characteristics,PHNETs are challenging to differentiate from other liver tumors.CASE SUMMARY This case involved a 67-year-old male who was admitted with a discovered hepatic mass and a suspicious lung lesion.Primary hepatic carcinoma was initially speculated based on the characteristic magnetic resonance imaging findings.The patient underwent a laparoscopic right partial hepatectomy,and subsequent immunohistochemical examination revealed a HNET.To exclude other potential origins,a positron emission tomography-computed tomography scan and gastrointestinal endoscopy were performed,leading to a final diagnosis of PHNETs.Then we conducted a literature review using the PubMed database,identifying 99 articles and 317 cases related to PHNETs.The characteristics,diagnostic methods,and treatment of PHNETs have been described.Finally,we elaborate on the presumed origins,pathological grades,clinical features,diagnosed methods,and treatments associated with PHNETs.CONCLUSION The diagnosis of PHNETs was primarily an exclusionary process.A definitive diagnosis of PHNETs relied mainly on immunohistochemical markers(chromogranin A,synaptophysin,and cluster of differentiation 56)and exclusion of primary foci in other organs.Radical surgery was the preferred treatment for early-stage tumors.

Primary coexisting adenocarcinoma of the colon and neuroendocrine tumor of the duodenum: A case report and review of the literature

BACKGROUND Neuroendocrine tumors(NETs)arise from the body’s diffuse endocrine system.Coexisting primary adenocarcinoma of the colon and NETs of the duodenum(D-NETs)is a rare occurrence in clinical practice.The classification and treatment criteria for D-NETs combined with a second primary cancer have not yet been determined.CASE SUMMARY We report the details of a case involving female patient with coexisting primary adenocarcinoma of the colon and a D-NET diagnosed by imaging and surgical specimens.The tumors were treated by surgery and four courses of chemothe-rapy.The patient achieved a favorable clinical prognosis.CONCLUSION Coexisting primary adenocarcinoma of the colon and D-NET were diagnosed by imaging,laboratory indicators,and surgical specimens.Surgical resection com-bined with chemotherapy was a safe,clinically effective,and cost-effective treat-ment.

Treatment of primary nasal tuberculosis with anti-tumor necrosis factor immunotherapy:A case report

BACKGROUND Primary nasal tuberculosis(TB)is a rare form of extrapulmonary TB,particularly in patients receiving anti-tumor necrosis factor(TNF)immunotherapy.As a result,its diagnosis remains challenging.CASE SUMMARY A 58-year-old male patient presented to the ear,nose,and throat department with right-sided nasal obstruction and bloody discharge for 1 month.He was diagnosed with psoriatic arthritis and received anti-TNF immunotherapy for 3 years prior to presentation.Biopsy findings revealed chronic granulomatous inammation and a few acid-fast bacilli,suggestive of primary nasal TB.He was referred to our TB management department for treatment with oral anti-TB agents.After 9 months,the nasal lesions had disappeared.No recurrence was noted during follow-up.CONCLUSION The diagnosis of primary nasal TB should be considered in patients receiving TNF antagonists who exhibit thickening and crusting of the nasal septum mucosa or inferior turbinate,particularly when pathological findings suggest granulomatous inflammation.

Clinical analysis of multiple primary gastrointestinal malignant tumors:A 10-year case review of a single-center

BACKGROUND Multiple primary malignant tumors(MPMTs)was first described by Billroth as early as 1889,with the first report published by Warren and Gates in 1932.Since then,numerous cases have been reported.A literature review of 1104269 patients with cancer revealed that the incidence of MPMTs ranged from 0.73 to 11.7%.In recent years,however,there has been a significant upward trend in the incidence of this phenomenon,which may be associated with many different factors,including the advancement of modern diagnostic procedures facilitating the examination and diagnosis of more MPMTs,increased exposure to chemotherapy and radiotherapy that exacerbate the risk of new malignant tumors in patients with cancer,and prolonged survival of patients with cancer allowing sufficient time for the development of new primary cancers.AIM To analyze the incidence,clinical features,treatment factors,prevalence,and prognosis of patients with MPMTs in the gastrointestinal tract treated in a single center.Additionally,we analyzed the different tumor combinations,time interval between the occurrence of tumors,and staging.METHODS This retrospective cohort study analyzed 8059 patients with pathologically confirmed gastrointestinal malignant tumors treated at the Gansu Province Hospital in Lanzhou,Gansu,China between June 2011 and June 2020.Of these,85 patients had MPMTs.The clinical features,treatment factors,prevalence,and prognosis of this latter cohort were analyzed.RESULTS The incidence of MPMTs in patients with gastrointestinal malignant tumors was 1.05%(85/8059),including 83 double primary malignant tumors and two triple primary malignant tumors of which 57(67.06%)were synchronous MPMTs(SMPMTs)and 28(32.94%)were metachronous MPMTs(MMPMTs).The most frequent associations were found between the rectum colon cancers within the SMPMT category and the gastric-colon cancers within the MMPMT category.For the MMPMTs,the median interval was 53 months.The overall 1-,3-and 5-year survival rates from diagnosis of the first primary cancer were 91.36%,65.41%,and 45.97%,respectively;those from diagnosis of the second primary cancer were 67.90%,29.90%,and 17.37%,respectively.CONCLUSION MPMTs in the gastrointestinal tract have a high incidence and poor prognosis.Thus,it is necessary to perform both gastroscopy and colonoscopy in patients with gastrointestinal tumors.Multidisciplinary comprehensive diagnosis and treatment may improve the diagnosis rate and treatment efficiency of MPMTs.

Early screening to identify and diagnose primary nasal tuberculosis in patients with tumor necrosis factor inhibitors

In this editorial,we comment on the article by Liu et al.Based on our analysis of a case report,we consider that early screening and recognition of primary nasal tuberculosis are crucial for patients undergoing treatment with tumor necrosis factor inhibitor(TNFi).While TNFi therapy increases the risk of reactivating latent tuberculosis,primary nasal tuberculosis remains rare due to the protective mechanisms of the nasal mucosa.Risk factors for primary nasal tuberculosis include minimally invasive nasal surgery,diabetes,and human immunodefi ciency virus.Patients with early symptoms such as nasal congestion,rhinorrhea,altered olfaction,epistaxis,or ulceration,and unresponsive to conventional antibiotics and antihistamines should undergo early rhinoscopy,possibly followed by repeated tissue biopsies and acid-fast bacilli culture when necessary.When diagnosis is challenging,it is essential to consider local tuberculosis epidemiology and the efficacy of diagnostic antituberculosis treatment.The preferred method for tuberculosis screening is the Interferon Gamma Release Assay,with a general recommendation for screening at 3 and 6 months after initial treatment and then every six months.However,the optimal frequency is not yet consensus-driven and may be increased in economically viable settings.

Expect the unexpected:Brown tumor of the mandible as the first manifestation of primary hyperparathyroidism

Hyperparathyroidism(HPT)is a condition in which one or more parathyroid glands produce increased levels of parathyroid hormone(PTH),causing disturbances in calcium homeostasis.Most commonly HPT presents with asymptomatic hypercalcemia but the clinical spectrum may include disturbances reflecting the combined effects of increased PTH secretion and hypercalcemia.Brown tumors are rare,benign,tumor-like bone lesions,occurring in 1.5%to 4.5%of patients with HPT,as a complication of an uncontrolled disease pathway,and are nowadays rarely seen in clinical practice.The tumor can appear either as a solitary or multifocal lesion and usually presents as an asymptomatic swelling or a painful exophytic mass.Furthermore,it can cause a pathological fracture or skeletal pain and be radiologically described as a lytic bone lesion.The diagnosis of a brown tumor in HPT is typically confirmed by assessing the levels of serum calcium,phosphorus,and PTH.Although when present,brown tumor is quite pathognomonic for HPT,the histologic finding often suggests a giant cell tumor,while clinical presentation might suggest other more frequent pathologies such as metastatic tumors.Treatment of brown tumors frequently focuses on managing the underlying HPT,which can often lead to regression and resolution of the lesion,without the need for surgical intervention.However,in refractory cases or when dealing with large symptomatic lesions,surgical treatment may be necessary.

Clinical characteristics and outcome of primary hepatic neuroendocrine tumors after comprehensive therapy

BACKGROUND Primary hepatic neuroendocrine tumors(PHNETs),a group of neuroendocrine neoplasms,are extremely rare.There are only few case reports about PHNETs in the literature.The lack of large samples and multicenter research results in poor diagnostic and therapeutic approaches.AIM To discuss the clinical characteristics,diagnosis,and treatment of PHNETs and risk factors related to survival.METHODS We retrospectively analyzed the clinical data,imaging features,immunohistochemistry data,and treatment efficacy of 40 patients who were pathologically diagnosed with PHNETs and admitted to The First Affiliated Hospital of Zhengzhou University from January 1,2014 to November 15,2019.Finally,survival analysis was performed to identify the risk factors for survival.RESULTS The main symptoms and signs included intermittent abdominal pain(19 patients,47.5%)and bloating(8 patients,20.0%).The positive rates of tested tumor markers were recorded as follows:Carbohydrate antigen 19-9(CA19-9)(6 patients,15.0%),CA72-4(3 patients,7.5%),carcinoembryonic antigen(7 patients,17.5%),and alpha-fetoprotein(6 patients,15.0%).Immunohistochemical staining results showed positivity for Syn in 38(97.4%)of 39 patients,for chromogranin A in 17(65.4%)of 26 patients,for CD56 in 35(94.6%)of 37 patients,for AE1/AE3 in 28(87.5%)of 32 patients,and for Ki-67 in all 40(100.0%)patients.The overall survival rate was significantly related to the tumor grade,AE1/AE3,and Ki-67.tumor number,tumor size,metastasis,and treatment)and overall survival.CONCLUSION Higher grade,negative AE1/AE3,and higher Ki-67 are associated with a worse survival rate.Kinds of treatment and other parameters have no significant influence on overall survival.

Influence of primary tumor location and resection on survival in metastatic colorectal cancer

BACKGROUND Patients with right sided colorectal cancer are known to have a poorer prognosis than patients with left sided colorectal cancer, whatever the cancer stage. To this day, primary tumor resection(PTR) is still controversial in a metastatic, non resectable setting.AIM To explore the survival impact of PTR in patients with metastatic colorectal cancer(mCRC) depending on PTL.METHODS We retrospectively collected data from all consecutive patients treated for mCRC at the Centre Georges Francois Leclerc Hospital. Univariate and multivariate Cox proportional hazard regression models were used to assess the influence of PTR on survival. We then evaluated the association between PTL and overall survival among patients who previously underwent or did not undergo PTR. A propensity score was performed to match cohorts.RESULTS Four hundred and sixty-six patients were included. A total of 153(32.8%) patients had unresected synchronous mCRC and 313(67.2%) patients had resected synchronous mCRC. The number of patients with right colic cancer, left colic cancer and rectal cancer was respectively 174(37.3%), 203(43.6%) and 89(19.1%). In the multivariate analysis only PTL, PTR, resection of hepatic and or pulmonary metastases and the use of oxaliplatin, EGFR inhibitors or bevacizumab throughout treatment were associated to higher overall survival rates. Survival evaluation depending on PTR and PTL found that PTR improved the prognosis of both left and right sided mCRC. Results were confirmed by using a weighted propensity score.CONCLUSION In mCRC, PTR seems to confer a higher survival rate to patients whatever the PTL.

Pyrotinib is effective in both trastuzumab-sensitive and primary resistant HER2-positive breast tumors

Objective: Primary resistance to trastuzumab frequently occurs in human epidermal growth factor receptor 2(HER2)-positive(+) breast cancer patients and remains a clinical challenge. Pyrotinib is a novel tyrosine kinase inhibitor that has shown efficacy in the treatment of HER2+ breast cancer. However, the efficacy of pyrotinib in HER2+ breast cancer with primary trastuzumab resistance is unknown.Methods: HER2+ breast cancer cells sensitive or primarily resistant to trastuzumab were treated with trastuzumab, pyrotinib, or the combination. Cell proliferation, migration, invasion, and HER2 downstream signal pathways were analyzed. The effects of pyrotinib plus trastuzumab and pertuzumab plus trastuzumab were compared in breast cancer cells in vitro and a xenograft mouse model with primary resistance to trastuzumab.Results: Pyrotinib had a therapeutic effect on trastuzumab-sensitive HER2+ breast cancer cells by inhibiting phosphoinositide 3-kinase(PI3K)/protein kinase B(AKT) and rat sarcoma virus(RAS)/rapidly accelerated fibrosarcoma(RAF)/mitogen-activated protein kinase(MAPK)/extracellular-signal regulated kinase(ERK)pathways. In primary trastuzumab-resistant cells, pyrotinib inhibited cell growth, migration, invasion, and HER2 downstream pathways, whereas trastuzumab had no effects. The combination with trastuzumab did not show increased effects compared with pyrotinib alone. Compared with pertuzumab plus trastuzumab, pyrotinib plus trastuzumab was more effective in inhibiting cell proliferation and HER2 downstream pathways in breast cancer cells and tumor growth in a trastuzumab-resistant HER2+ breast cancer xenograft model.Conclusions: Pyrotinib-containing treatments exhibited anti-cancer effects in HER2+ breast cancer cells sensitive and with primary resistance to trastuzumab. Notably, pyrotinib plus trastuzumab was more effective than trastuzumab plus pertuzumab in inhibiting tumor growth and HER2 downstream pathways in HER2+ breast cancer with primary resistance to trastuzumab. These findings support clinical testing of the therapeutic efficacy of dual anti-HER2 treatment combining an intracellular small molecule with an extracellular antibody.

Liver transplantation and liver resection as alternative treatments for primary hepatobiliary and secondary liver tumors: Competitors or allies?

Although Starzl designed in the 1960’s liver transplantation(LT)to treat unresectable primary and also secondary liver tumors,transplantation still occupies a(too)small place in the respective therapeutic algorithms[1].Due to the lack of(any)selection criteria,the concept of transplantation became rapidly challenged because of the prohibitively high incidence of tumor recurrence[1–3].

Neuroendocrine liver metastases: Contributions of endoscopy and surgery to primary tumor search

Neuroendocrine tumors (NETs) are diagnosed with increasing frequency and patients often present with liver metastases at the time of diagnosis. Apart from treatment of the metastases, resection of the primary tumor at an early phase is recommended to prevent complications, although it may be difficult to locate, especially in patients with functionally inactive NETs. Small and multifocal tumors in the jejunum and ileum represent a particular challenge. Primary hepatic neuroendocrine carcinoma is extremely rare and is diagnosed only after exclusion of other primary tumors. Therefore, some uncertainty may remain, as small non-hepatic primary tumors may escape detection. Diagnostic work-up in these patients includes biochemical assays and imaging modalities (also comprising specific techniques of scintigraphy and positron emission tomography). This editorial highlights the contributions of endoscopy and operative exploration to the search for the primary tumor. Besides esophagogastro-duodenoscopy, endoscopic ultrasonography, colonoscopy and bronchoscopy, special endoscopic techniques such as balloon enteroscopy or capsule endoscopy are used with growing experience. Compared with balloon enteroscopy, capsule endoscopy is noninvasive and better tolerated, but it cannot localize a lesion precisely and does not allow biopsy or removal of lesions. Before proceeding to surgery, a discussion of the findings by a tumor board should be a standard procedure. Improvements in diagnostic tools have created new perspectives for the detection of obscure primary tumors in patients with neuroendocrine liver metastases, and these searches are best coordinated by a multidisciplinary team.

High incidence combination of multiple primary malignant tumors of the digestive system

BACKGROUND Clinical reports of multiple primary malignant tumors(MPMTs)in the digestive system are increasing.In China,although the survival rate of patients with MPMTs is increasing,the quality of life is very low.Many patients have reached the advanced stage when the second primary tumor is found,resulting in no early intervention and treatment.This is due to the misunderstanding of MPMTs by clinicians,who treat such tumors as metastases.Therefore,before a patient has a second primary tumor,doctors should understand some common combinations of digestive system MPMTs to provide clinical guidance to the patient.AIM To explore the high incidence combination of digestive system MPMTs under heterochronism and synchronization.METHODS A total of 1902 patients with MPMTs at Peking Union Medical College Hospital were analyzed retrospectively.They were divided into metachronous MPMT and synchronous MPMT groups,and then the high incidence combinations of the first primary cancer and the second primary cancer in metachronous cancer and synchronous cancer were sorted.Sex and age differences between metachronous and synchronous tumors were tested by the chi square test and t test,respectively.A P value<0.05 was considered as statistically significant,and SPSS version 26.0(SPSS Inc.,Chicago,Illinois,United States)was used for statistical analysis.RESULTS Among the 1902 patients with MPMTs confirmed by pathology,1811(95.2%)cases were secondary primary cancers,89(4.7%)cases were tertiary primary cancers,and 2(0.1%)cases were quaternary primary cancers.Most(88.2%)of the secondary primary cancers were identified as metachronous multiple primary cancers six months after diagnosis of the first primary cancer.The top ten most common MPMTs in the first primary cancer group ranged from high to low as follows:Breast cancer,thyroid cancer,nonuterine cancer,lung cancer,colon cancer,kidney cancer,uterine cancer,bladder cancer,rectal cancer,and gastric cancer.The highest incidence rate of the first primary cancer in male metachronous cancer was lung cancer(11.6%),the highest incidence rate of the second primary cancer was still lung cancer(24.9%),the highest incidence rate of the first primary cancer in female metachronous cancer was breast cancer(32.7%),and the highest incidence rate of the second primary cancer was lung cancer(20.8%).Among them,breast cancer,nonuterine cancer and uterine cancer were female-specific malignant tumor types,and thyroid cancer also accounted for 79.6%of female patients.The top five metachronous cancer combinations,independent of female-specific malignant tumor types and thyroid cancer,were colon cancer and lung cancer(26 cases),kidney cancer and lung cancer(25 cases),rectal cancer and lung cancer(20 cases),gastric cancer and lung cancer(17 cases),and bladder cancer and lung cancer(17 cases).The most common synchronous cancer combination was colon cancer and rectal cancer(15 cases).CONCLUSION Screening for lung cancer should be performed six months after the detection of colon cancer while rectal cancer screening should be performed within six months.

Primary Malignant Renal Tumors in Infancy and Childhood: CT Appearances

Objective： To investigate the imaging manifestation of primary malignant renal tumor with CT. Methods： Forty-three cases of surgically and pathologically confirmed primary malignant renal tumor were retrospectively reviewed. Un-contrast and contrast CT was performed in all 43 patients in which 15 patients received MRI examination. Results： The residual normal renal tissue of 29 cases out of 34 cases of Wilms＇ tumor was enhanced and manifested ＂crescent sign＂ or ＂ring sign＂. Four cases of malignant rabdoid tumor （RTK） manifested as large mass with notable necrosis and subcapsular fluid collection; Two cases of clear cell sarcoma （CCS） showed metastases to the skull which could indicate the diagnosis; Renal cell carcinoma （RCC） （n=3） showed calcification in 1 case. Conclusion： CT can precisely delineate the location, size, extent of involvement, imaging characteristics and metastases of renal tumor, which can provide information necessary to the clinical staging, therapy planning and prognosis of the tumors.

Primary Endodermal Sinus Tumor in the Posterior Cranial Fossa:Clinical Analysis of 7 Cases

Objective To clarify the clinical features,therapeutic method and outcomes of the primary endodermal sinus tumors(ESTs)in the posterior cranial fossa.Methods The English literatures on EST in the posterior cranial fossa were retrieved from PubMed and reviewed.And a 4-year-old boy diagnosed with EST in our hospital was reported.The clinical manifestations,therapy,pathologic features,and prognosis of these cases were analyzed.Results Only seven cases of the ESTs in the posterior cranial fossa were enrolled in this review,including six cases searched from the PubMed and one case from our hospital.Six patients were boy and one patient’s gender was not available from the report.Ages ranged from 1 to 5 years(mean 3.14 years).The mean tumor size in our cohort was 4.4 cm.Six cases came from East Asia.Schiller-Duval bodies were found in all seven neoplasms.All tumors were positive for alpha-fetoprotein.The alpha-fetoprotein level in serum was increased to a very high level before therapy and depressed quickly after the effective chemotherapy.The mean follow-up time was 24.4 months(range 5-52 months).Six tumors were totally removed,and four of them recurred.Three cases died including one whose tumor was partially removed.Conclusions The serum alpha-fetoprotein level is well correlated with the severity of the tumor.A combination of operation and chemotherapy might be the effective management for EST in the posterior cranial fossa.The prognosis of extragonadal intracranial EST is poor.

Liver collision tumor of primary hepatocellular carcinoma and neuroendocrine carcinoma:A rare case report

BACKGROUND Hepatocellular carcinoma(HCC)can occasionally develop with other non-HCC cell types,either in a combined type or collision type.A collision tumor is defined as two histopathologically distinct tumors of the same organ lacking a clear transition zone.Hepatic collision tumors are rare.Among them,“hepatocellular carcinoma-hepatic neuroendocrine carcinoma”(HCC-NEC)collision tumors are especially rare and information about them is rarely published.CASE SUMMARY A 48-year-old man with typical findings of HCC underwent consecutive therapies,including radiofrequency ablation and embolization prior to resection.Diagnosis of the HCC-NEC collision tumor in the right liver and another HCC in the left liver was established following surgical resection.The patient displayed NEC metastasis following resection and succumbed to septicemia after 2 more rounds of chemotherapy.To our knowledge,this is the 25th reported case of mixed HCC-NEC tumor.The rarity of HCC-NEC collision tumors and the absence of diagnostic criteria make it difficult to differentiate this condition from simple liver tumors,especially in patients with chronic liver disease.CONCLUSION Our case highlights the difficulty in accurately diagnosing HCC-NEC in the absence of histological evidence.The prognosis is poor for this condition,although ultrasound-guided liver biopsy can be helpful to establish a prompt diagnosis.Further accumulation of such cases could help establish an accurate diagnosis earlier.Early discovery of NEC may allow for better treatment strategies and better prognoses.

Clinical Significance of Serum IL-18 and IL-18BP in Patients with Benign or Malignant Primary Liver Tumors

OBJECTIVE To study the relationship between the serumlevels of IL-18 and IL-18BP in the development and growth ofprimary liver cancer,benign liver tumors and liver cirrhosis and todetermine the value of serum IL-18 and IL-18BP in the diagnosisof primary liver cancer.METHODS The serum levels of IL-18 and IL-18BP in 36 patientswith primary hepatic carcinoma(PHC)were detected.Eighteenpatients were diagnosed with various benign liver tumors and 21patients with cirrhosis of liver(LC),determined by using an ELISAassay.The serum levels of AFP in 36 patients with primary livercancer were examined.The relationship among levels of serumIL-18,IL-18BP and AFP in the primary liver cancer was explored.RESULTS The sIL-18 levels in PHC were significantly lower thanin control group,the benign liver tumor group and the LC group.The sIL-18BP in PHC was significantly higher than that in controlgroup,benign liver tumor group and LC group(P<0.001).Therewas a close correlation between the levels of IL-18,IL-18BP andclinical stage in PHC:the later clinical stages had lower levels ofIL-18 and higher levels of IL-18BP while the earlier clinical stageshad higher levels of IL-18 and lower levels of IL-18BP.There was anegative correlation between serum levels of IL-18 and AFP in thePHC group(r = -0.7152,n=36,P<0.01),and there was a positivecorrelation between serum levels of IL-18 BP and AFP in thepatients with PHC(r=0.6315,n=36,P<0.01).The IL-18 and IL-18BP in the patients with various benign liver tumors or LC weresignificantly higher than those in control group.The differenceswere statistically significant(P<0.01).CONCLUSION Serum levels of IL-18 and IL-18BP can reflectthe immune function of patients with primary liver cancer,withvarious benign liver tumors or with LC and can also be indicativeof the clinic stage of primary liver cancer.It can be used to assistin making a diagnosis and in determining the clinical stage ofPHC.Detecting AFP concurrently can help make the diagnosis ofprimary liver cancer more precise.

Genes of tumor necrosis factors and their receptors and the primary open angle glaucoma in the population of Central Russia

AIM：To examine the association of genetic polymorphisms（-308）G/A TNFα,（＋250）A/G Ltα,（＋36）A/G TNFR1,（＋1663）A/G TNFR2 with the development of primary open angle glaucoma（POAG）among people in Central Russia.METHODS：The study sample included 443 individuals,of which 252 patients with POAG and 191 individuals in the control group.Genotyping of（-308）G/A TNFα,（＋250）A/G Ltα,（＋36）A/G TNFR1,（＋1663）A/G TNFR2 was performed using polymerase chain reaction.The distribution of alleles and genotypes of the studied DNA markers in the groups was examined by 2×2 contingency tables andχ2with the Yates’s correction for continuity and odds ratios（OR）with95%confidence intervals（CI）.RESULTS：Allele（-308）G TNFα（Р=0.01,OR=1.78,95%CI1.12-2.85）was identified as a risk factor for POAG.Homozygotes（-308）AA TNFαare at a lowest risk for development of the disease（Р=0.01,OR=0.0005）.The following combination of genetic variants of cytokines were associated with a reduced risk of POAG：（＋1663）A TNFR2 and（＋250）G Ltα（OR=0.34）CONCLUSION：Genetic polymorphisms（-308）G/A TNFα,（＋250）A/G Ltα,（＋1663）A/G TNFR2 associated with the development of POAG in the population of Central Russia.

Primary primitive neuroectodermal tumor in the pericardium-a focus on imaging findings:A case report

BACKGROUND Primitive neuroectodermal tumors(PNETs)are rare,sporadic malignant tumors of the peripheral nervous system,bone,or soft tissues.However,to the best of our knowledge,only three cases of PNET in the pericardium have been reported in the English literature,and their magnetic resonance imaging findings have not previously been described.CASE SUMMARY A 3-year-old boy was hospitalized with a 1-wk history of recurrent vomiting and weakness.Detailed history-taking revealed no evidence of heart disease.Computed tomography demonstrated a soft tissue mass in the left pericardial cavity with heterogeneous contrast enhancement.The border between the mass and the heart was poorly defined.Thoracotomy revealed a mass invading the left ventricle,with a high risk of bleeding.The mass was considered inoperable.A biopsy was performed,and the histological and immunohistochemical findings confirmed the diagnosis of primary PNET of the pericardium.The patient received four cycles of standard chemotherapy.Chest magnetic resonance imaging 3 mo after the initiation of chemotherapy revealed that the tumor in the pericardium still existed,but its volume had slightly decreased.The patient was lost to follow-up,and the final outcome was therefore unknown.CONCLUSION Medical imaging plays an important role in defining the pericardial origin of PNET and understanding its characteristics.Magnetic resonance imaging can provide more information on the tumor than computed tomography and may thus aid therapeutic planning.

Primary Malignant Musculosqueletal Tumors of Members in Adult in Togo

Purpose: Primary malignant musculosqueletal tumors in adult are rare affections group and its treatment is still a real challenge today. The aim of this work was to describe the epidemiologic and treatment aspects of these tumors in the national reference center of our country. Materiel and Methods: It was a retrospective review of primary malignant musculosqueletal tumors treated in orthopedics and trauma unit of Sylvanus Olympio teaching hospital of Lomé, Togo from January 2000 to December 2014. Results: During the study period, 28 cases were selected and reviewed. There were 17 men (60.71%) and 11 women (39.29%). The average age was 32.7 years. There were 20 cases (71%) of primary malignant bone tumors (PMBT) and 8 cases (29%) of primary malignant soft tissues tumors (PMSTT). There were 6 (30%) of osteosarcoma. The tumor was located in the bones of the forearm and wrist/hand in 2 (10%) patients for each anatomical site;for the lower limb, around knee in 7 (35%) patients. Eight patients had PMSTT (28, 57%). There were three cases of rhabdomyosarcoma, 2 cases of fibrosarcoma. Tumors were located around knee in 4 cases and around ankle/foot in 3 cases. In the two groups of tumor, tumor resection was performed in 5 patients (17.86%) and limb amputation indicated in 23 patients (82.14%), was performed in 15 (53.57%) and 8 patients (37.78%), rejected it and left hospital against medical advice. Conclusion: Malignant musculoskeletal tumors are relatively rare in Togo. Their treatment is based on radical surgery which is often not supervised by adjuvant therapies. Ignorance, poverty of the population and embryonic state of diagnostic and treatment infrastructures are the obstacles to their care. Education and awareness must be integrated to care and fight against this group of diseases.

Primary Cardiac Tumors Operated on in Côte d’Ivoire: They Are Almost All Myxomas

Objective: Through this surgical series, we present the epidemiological and anatomical-clinical aspects and the surgical results concerning patients operated on for a primary cardiac tumor at the Abidjan Heart Institute. Materials and Method: This is a retrospective descriptive study covering the period of January 1982 to December 2022, based on the medical records of patients operated on for a primary cardiac tumor at the Abidjan Heart Institute. Results: Twenty-seven (27) patients underwent surgery for a primary cardiac tumor, including 14 women and 13 men with a mean age of 41.5 years (range 19 - 76 years). The main circumstances of discovery were exertional dyspnea, palpitation and syncope or pseudo-syncope. The main site was the septal wall of the left atrium. The diagnosis of myxoma was confirmed by pathological examination of the surgical specimen in 96.3% (n = 24) of the patients and it was a malignant large cell immunoblastic lymphoma of the myocardium in 3.7% (n = 1) of the patients. The mean largest diameter was 46.1 mm. The postoperative course was marked by an ischaemic stroke (n = 1);recurrence of a left atrial myxoma 5 years after the first tumor removal (n = 1). Two cases of death were noted, one due to the evolution of immunoblastic large cell lymphoma and the other due to an extracorporeal circulation accident. Conclusion: Almost all primary cardiac tumors operated on in Abidjan are myxomas. The circumstances of the discovery of these cardiac tumors are multiple and varied but dominated by exertional dyspnea, palpitation and syncope. Whatever their histological type, primary cardiac tumors are serious affections, in view of the haemodynamic and rhythmic disorders they cause.