Undifferentiated liver embryonal sarcoma in adults: A report of four cases and literature review

AIM: To evaluate the undifferentiated embryonal sarcoma of liver (UESL) in adults in order to improve its diagnosis and treatment. METHODS: Four primary and one recurrent cases of UESL were clinicopathologically evaluated and immunohistochemically investigated with a panel of antibodies using the EnVision+ system. Relevant literature about UESL in adults was reviewed. RESULTS: Three males and one female were enrolled in this study. Their chief complaints were abdominal pain, weight loss, or fever. Laboratory tests, imaging and pathological features of UESL in adults were similar to those in children. Immunohistochemistry showed evidence of widely divergent differentiation into mesenchymal and epithelial phenotypes. The survival time of patients who underwent complete tumor resection followed by adjuvant transcatheter arterial chemoembolization (TACE) was significantly longer than that of those who underwent surgical treatment alone.CONCLUSION: UESL in adults may undergo pluripotential differentiation and its diagnosis should be made based on its morphological and immunohistochemical features. Complete tumor resection after adjuvant TACE may improve the survival time of such patients.

Undifferentiated embryonal sarcoma of the liver:Clinical characteristics and outcomes

BACKGROUND Undifferentiated embryonal sarcoma of the liver(UESL)is a rare liver malignancy originating from primary mesenchymal tissue.The clinical manifestations,laboratory tests,and imaging examinations of the disease lack specificity and the preoperative misdiagnosis rate is high.The overall prognosis is poor and survival rate is low.AIM To investigate the diagnosis,treatment,and prognosis of UESL.METHODS We performed a retrospective,single-center cohort study in Shengjing Hospital of China Medical University,which is a central hospital in northeast China.From 2005 to 2017,we recruited 14 patients with pathologically confirmed UESL.We analyzed the clinical manifestations,laboratory tests,imaging examinations,pathological examinations,therapy,and prognosis of these patients.RESULTS There were nine males and five females aged 2-60 years old included in the study.The major initial symptoms were abdominal pain(71.43%)and fever(57.14%).Preoperative laboratory tests revealed that seven patients had increased leukocyte levels,four showed a decrease in hemoglobin levels,seven patients had increased glutamyl transpeptidase levels,nine had increased lactate dehydrogenase levels,and three showed an increase in carbohydrate antigen 199.There was no difference in the rate of misdiagnosis in preoperative imaging examinations of UESL between adults and children(6/6 vs 5/8,P=0.091).The survival rate after complete resection was 6/10,while that after incomplete resection was 0/4(P=0.040),suggesting that complete resection is important to improve survival rate.In total,five out of the eight children achieved survival.During the follow-up,the maximum survival time was shown to be 11 years and minimum survival time was 6 mo.Six adult patients relapsed late after surgery and all of them died.CONCLUSION Preoperative imaging examination for UESL has a high misdiagnosis rate.Multidisciplinary collaboration can improve the diagnostic accuracy of UESL.Complete surgical resection is the first choice for treatment of UESL.

Undifferentiated embryonal sarcoma of the liver presenting as a hemorrhagic cystic tumor in an adult

BACKGROUND:Undifferentiated embryonal sarcoma (UES) of the liver is a rare,highly malignant neoplasm with a poor prognosis occurring almost exclusively in late childhood (6-10 years of age).Only a few cases have been reported in adults accounting for less than 1% of all primary liver neoplasms.METHODS:A 47-year-old woman presented with a palpable mass in the left upper abdomen.Magnetic resonance imaging revealed a 12×10 cm cystic mass with hemorrhage in the left lateral segment of the liver.The initial impression was a hemorrhagic cystic tumor of the liver.The patient underwent a left lateral sectionectomy of the liver.Histopathology and immunohistochemistry helped make a diagnosis of UES.RESULTS:The patient recovered uneventfully and received systemic chemotherapy.Radiologic examination for follow-up revealed a metastatic lesion in the lumbar spine (L5).She was subjected to radiotherapy at the lumbar spine.She survived 48 months.CONCLUSION:Although hepatic cyst as UES of the liver is difficult to diagnose because of its rarity in adults and lack of specific findings,it should be considered in a differential diagnosis.

Embryonal rhabdomyosarcoma in the maxillary sinus with orbital involvement in a pediatric patient:Case report

This report presents a case of embryonal rhabdomyosarcoma(e RMS) located in the left maxillary sinus and invading the orbital cavity in a ten-year-old male patient who was treated at a referral hospital.The images provided from the computed tomography showed a heterogeneous mass with soft-tissue density,occupying part of the left half of the face inside the maxillary sinus,and infiltrating and destroying the bone structure of the maxillary sinus,left orbit,ethmoidal cells,nasal cavity,and sphenoid sinus.An analysis of the histological sections revealed an undifferentiated malignant neoplasm infiltrating the skeletal muscle tissue.The immunohistochemical analysis was positive for the antigens:MyoD 1,myogenin,desmin,and Ki67(100% positivity in neoplastic cells),allowing the identification of the tumour as an eR MS.The treatment protocol included initial chemotherapy followed by radiotherapy and finally surgery.The total time of the treatment was nine months,and in 18-mo of follow-up period did not show no local recurrences and a lack of visual impairment.

Primary Embryonal Lung Carcinoma and Testicular Seminoma in the Same Patient Ten-Years Later: Case-Report and Literature Review

Primary germ cell tumors of lung are extremely rare. The prognosis is usually poor, with various symptoms seriously affecting quality of life. In this paper we describe the unique case of a patient affected by an embryonal carcinoma of lung and a testicular seminoma after ten years. We also report literature about pulmonary extragonadal germ cell tumors.

Neoadjuvant transcatheter arterial chemoembolization and systemic chemotherapy for the treatment of undifferentiated embryonal sarcoma of the liver in children

BACKGROUND Undifferentiated embryonal sarcoma of the liver(UESL)is a rare and aggressive mesenchymal tumor in children.Herein,we describe our experience in neoadjuvant therapy(NAT)and subsequent surgery for the treatment of UESL in children.AIM To evaluate the efficacy of NAT and explore a new choice for successful operation of UESL in children.METHODS We retrospectively analyzed six patients newly diagnosed with unresectable UESL who received NAT and then surgery at our center between January 2004 and December 2019.The tumor was considered unresectable if it involved a large part of both lobes of the liver or had invaded the main hepatic vessels or inferior vena cava.The NAT included preoperative transcatheter arterial chemoembol ization(TACE)and systemic chemotherapy.The patients were 4 boys and 2 girls with a mean age of 7 years.The longest tumor at presentation ranged from 8.6 to 14.8 cm(mean,12 cm).Extrahepatic metastases were present in 2 cases.Preoperative systemic chemotherapy was administered 3 wk after TACE.Tumor resection was performed 3 wk after one or two cycles of NAT.The patients received systemic chemotherapy after surgery.RESULTS All patients successfully underwent NAT and complete resection.The tumor volumes decreased by 18.2%–68.7%,with a mean decrease of 36%after 1 cycle of NAT(t=3.524,P=0.017).According to the Response Evaluation Criteria In Solid Tumors criteria,4 patients had a partial response and underwent surgery,while 2 had stable disease and received another cycle of NAT before surgery.Massive tumor necrosis was seen on pathological examination of the surgical specimen:>90%necrosis in two,>50%necrosis in three,and 25%necrosis in 1,with an average of 71.8%.Post-NAT complications included fever,nausea and vomiting,and mild bone marrow suppression.Elevated alanine transaminase levels occurred in all patients,which returned to normal within 7–10 d after treatment.No cardiac or renal toxicity,severe hepatic dysfunction,bleeding and nontarget embolization were observed in the patients.The median follow-up period was 8 years with an overall survival of 100%.CONCLUSION NAT effectively reduced tumor volume,cleared the tumor margin,and caused massive tumor necrosis.This may be a promising choice for successful surgery of UESL in children.

Recurrent undifferentiated embryonal sarcoma of the liver in adult patient treated by pembrolizumab: A case report

BACKGROUND Undifferentiated embryonal sarcoma of the liver(UESL)is a neoplasm that rarely develops in adults.The main treatments for UESL are upfront gross total surgical resection and adjuvant multiagent chemotherapy.Here,we report a case of recurrent UESL in an adult treated with pembrolizumab and discuss a method to identify proper candidates for antibody of programmed cell death protein 1(anti-PD-1)treatment.CASE SUMMARY A 69-year-old woman was admitted for abdominal pain that developed for 1 wk.Computed tomography showed a 16 cm mass in the right lobe of the liver.Right hemihepatectomy and lymphadenectomy were performed,and histological diagnosis was UESL.Six months later,the patient suffered from painless obstructive jaundice,and positron emission tomography-computed tomography revealed multiple metastases.Then,percutaneous transhepatic cholangial drainage was applied to reduce jaundice,and radiofrequency ablation was used to control the lesion near the hepatic hilum.However,the patient suffered from a serious fever caused by the tumor.The patient received treatment with pembrolizumab,and the prescribed dosage was 2 mg/kg every 3 wk.After the seventh dose,positron emission tomography-computed tomography revealed that the multiple metastases had nearly disappeared.Radiologic exam was used to evaluate the disease state,and no new lesions were found.Next-generation sequencing and immunohistology were applied to determine the reason why the patient had such a favorable response to pembrolizumab.Tumor mutation burden,microsatellite instability,and programmed death ligand 1 expression can be combined to predict the effect of PD-1 antibodies. When every one of thesebiomarkers are detected in a tumor patient, the patient may be a proper candidatefor PD-1 antibodies.CONCLUSION Anti-PD-1 treatment for tumors needs further research to identify indications andproper biomarkers.

Our Experience with Embryonal Rhabdomyosarcoma Presenting as Aural Polyp

Aural polyps are a common clinical entity encountered by otorhinolaryngologist in daily practice. Polyps are frequently seen in paediatric patients, usually inflammatory in nature. In children rhabdomyosarcomas (RMS) can mimic all the cinical features of chronic suppurative otitis media which usually present as external auditory canal mass or polyp. Here we present a case where a male child presented with recurrence of polyp in left ear which was finally diagnosed as embryonal rhabdomyosarcoma which is a rare and invariably fatal disease in children. Through this article we intend to highlight the failures and delay committed in attaining diagnosis in this patient in spite of multispecialty evaluation involving repeated imaging and histopathological correlation. Our experience with embryonal rhabdomyosarcoma throws light on the high vigilance required in handling the aural polyps in pediatric population as early diagnosis and treatment are the key elements for successful outcomes.

胎儿的心脏的间充质的干细胞表示 embryonal 标记并且展出区别进所有三细菌层的房间

AIM:To study the expression of embryonal markers by fetal cardiac mesenchymal stem cells(fC-MSC)and their differentiation into cells of all the germ layers. METHODS:Ten independent cultures of rat fCMSC were set up from cells derived from individual or pooled fetal hearts and studies given below were carried out at passages 3,6,15 and 21.The phenotypic markers CD29,CD31,CD34,CD45,CD73,CD90, CD105,CD166 and HLA-DR were analyzed by flow cytometry.The expression of embryonal markers Oct-4, Nanog,Sox-2,SSEA-1,SSEA-3,SSEA-4,TRA-1-60 and TRA 1-81 were studied by immunocytochemistry.The fC-MSC treated with specific induction medium were evaluated for their differentiation into(1)adipocytes and osteocytes(mesodermal cells)by Oil Red O and Alizarin Red staining,respectively,as well as by expression of lipoprotein lipase,PPARγ2 genes in adipocytes and osteopontin and RUNX2 genes in osteocytes by reverse-transcription polymerase chain reaction(RT- PCR);(2)neuronal(ectodermal)cells by expression of neuronal Filament-160 and Glial Fibrillar Acidic Protein by RT-PCR and immunocytochemistry;and(3)hepa- tocytic(endodermal)cells by expression of albumin by RT-PCR and immunocytochemistry,glycogen deposits by Periodic Acid Schiff staining and excretion of urea into the culture supernatant. RESULTS:The fC-MSC expressed CD29,CD73,CD90, CD105,CD166 but lacked expression of CD31,CD34, CD45 and HLA-DR.They expressed embryonal markers,viz.Oct-4,Nanog,Sox-2,SSEA-1,SSEA-3,SSEA-4, TRA-1-81 but not TRA-1-60.On treatment with specific induction media,they differentiated into adipocytes and osteocytes,neuronal cells and hepatocytic cells. CONCLUSION:Our results together suggest that fCMSC are primitive stem cell types with a high degree of plasticity and,in addition to their suitability for cardiovascular regenerative therapy,they may have a wide spectrum of therapeutic applications in regenerative medicine.

Real case of primitive embryonal duodenal carcinoma in a young man

We report here the case of a young man suffering from a rare germ cell tumour. The patient was a 25-yearold man who was referred to our centre for asthenia, stinging epigastric pain, and an iron deficiency anaemia. Gastroscopy revealed a circumferential vegetating lesion on the second portion of the duodenum. The lesion was indurated at the third portion of the duodenum, responsible for a tight stenosis. A computerized tomography-scan of the chest, abdomen and pelvis, and a pancreatic MRI showed a circumferential lesion with a bi-ductal dilatation(i.e., of the common bile duct and Wirsung's duct) without metastatic localisation. The patient underwent a pancreaticoduodenectomy with lymph node dissection including all cellular adipose tissues of the hepatic pedicle from the hepatic common artery and of the retroportal lamina. Histological findings were suggestive of a duodenal embryonal carcinoma with pancreatic infiltration. This is the second published case highlighting the duodenal primitive localisation of an embryonal carcinoma with pancreatic infiltration.

A case of prostate embryonal rhabdomyosarcoma in an adult patient

Prostate embryonal rhabdomyosarcoma(ERMS) is characterized by a high degree of malignancy, both local rapid growth with formation of large pelvic masses, often leading to renal failure due to urethra obstruction, and systemic spread, commonly to the lungs, liver, and bone. ERMS of the prostate is a commonly occurring tumor in infants and children. It is rarely seen in adults. Here, we report on a case of the prostate ERMS in a 27-year-old man.

Embryonal Rhabdomyosarcoma of the Uterine Cervix: Two Cases Report and Literature Review

Introduction:?Embryonal rhabdomyosarcoma (RMS) of the uterine cervix is a rare and extremely aggressive malignant entity. However, prognosis seems to be improved with the use of multimodality approach treatment. Cases: We report the cases of 50- and 51-year-old women, presenting with vaginal bleeding and mass. The two patients underwent radical surgery. Histological examination revealed RMS of uterine cervix. Treatment was effective in the first case but despite the chemotherapy, the second patient died two months later. Discussion and Conclusions: The prognosis of the cervical embryonal RMS depends on clinical and histological features. The current treatment protocols are based on trials done on pediatric patients. Studies on embryonal RMS treatment and outcomes are limited in women over forty years. In patients with unfavorable prognosis characteristics, the multimodality approach including surgery, adjuvant chemo and radiotherapy can be effective. Otherwise, surgery alone can effectively be proposed.

Overview of embryonal rhabdomyosarcoma of cervix in women over 40-year-old

The literature on cervical embryonal rhabdomyosarcoma(RMS) is reviewed here to identify management guidelines for middle-aged women diagnosed with this rare type of gynecologic cancer. Specifically, the Pub Med, Web of Science and Google Scholar databases, were searched to find published case series on cervical embryonal RMS reporting on four or more patients, of whom at least one was > 40-year-old. The χ2 test was used to assess heterogeneity. Five articles published between 1986 and 2013 were identified, reporting on a total of 47 patients, of whom 22(46.8%) were older and 25(53.2%) younger than 40-year-old. Although the two age groups did not differ significantly by stage of disease or radiotherapy treatment, the older age groupreceived less chemotherapy(55% vs 90%, P = 0.008)and had more hysterectomy(86% vs 43%, P = 0.009).Follow-up data was missing for 18/47(38.3%) patients.Among the 29 patients with follow-up data, survival was shorter in the older group, with 8/12(67%) alive and3 with disease at a median follow-up of 2.6 years, as compared with the younger group that had 15/17(88%)alive and none with disease at a median follow-up of 3.5years. The longest survivals among the older women were observed in those who received radiotherapy,including one case with a resected lung metastasis.A prospective multi-institutional collaboration and better follow-up are needed to determine the optimal management of cervical embryonal RMS. Long-term survival appears feasible if management is accompanied by chemotherapy and radiotherapy.

Embryonal Rhabdomyosarcoma of Middle Ear and Temporal Bone: A Rare Case Report

Introduction: Rhabdomyosarcoma accounts for up to 60% of childhood soft tissue sarcoma, occurring mostly in the head and neck region but rarely in the ear and temporal areas. Case Report: A 4-year-old boy came to our hospital due to a purulent discharge, bleeding from his left ear. Also seen mass in the left ear along with facial weakness and diplopia. After histopathologic evaluation, it was diagnosed as embryonal rhabdomyosarcoma. Conclusion: Rhabdomyosarcoma should be kept in mind as a differential diagnosis in all children presenting with otitis media with mastoiditis. Biopsy should be considered who are not responding to medications and those worsening condition.

Diagnosis and therapy of primary undifferentiated embryonal sarcoma of the liver

Primary undifferentiated embryonal sarcoma （UES） of the liver, also called malignant mesenchymoma of the liver, is a rare hepatic malignancy. It arises from the mesoderm and comprises more than two types of sarcomas. UES can occur at any site of the body; common sites include the retroperitoneum and the lower extremities. Only a few cases of UES have been reported worldwide.1 In this study, we retrospectively examined the clinical data of 11 patients with UES between January 2000 and November 2011 at the People＇s Liberation Army General Hospital.

Adult primary undifferentiated embryonal sarcoma of the liver： a case report

Undifferentiated embryonal sarcoma of the liver （UESL） was first described by Stocker and Ishak in 1978. It is a rare tumor that most often presents in childhood between 6 to 10 years of age.2 The adult patients diagnosed with UESL often show nonspecific symptoms with normal laboratory results. Ultrasonography, CT, or MRI often reveals a cystic mass. Complete surgical resection plus pre- and/or post-operative systemic chemotherapy offers the possibility of a cure. We report a case of primary UESL in an adult with no specific symptoms, tumor markers, or image results. The diagnosis of the case was based on the resulting pathology from surgery.

Characteristics and prognosis of embryonal rhabdomyosarcoma in children and adolescents:An analysis of 464 cases from the SEER database

Importance:As the most common subtype of pediatric rhabdomyosarcoma(RMS),the prognosis of embryonal RMS has rarely been investigated solely.Objective:To perform a population-based study to characterize the prognosis of embryonal RMS in children and adolescents.Methods:Demographic and clinical features were retrospectively evaluated in selected patients with embryonal RMS registered in the Surveillance,Epidemiology,and End Results(SEER)program from 1988 to 2016.Survival curves were compared using the log-rank test.A multivariate Cox proportional hazards model was developed to assess the impact of each factor on the overall survival.A nomogram was constructed based on the results of Cox regression model.Results:A total of 464 patients were included in the analysis,among which 64.6%were male and 70.2%were white patients.About 38.6%and 26.3%of the patients were at 1-4 years and 5-9 years,respectively.Cox analysis showed that patients at age group 5-9 years had the lowest risk of mortality(hazard ratio[HR],0.277;95%confidential interval[CI],0.123-0.620),compared with patients diagnosed at less than 1-year-old,and age group 1-4 years had the second-best prognosis.Patients having distant tumors had significantly higher mortality risk(HR,4.842;95%CI,2.804-8.362)than the patients with localized tumor.Compared with receiving no surgery or radiotherapy,receiving any combination of surgery and radiotherapy would lower the risk of mortality significantly(for surgery without radiotherapy:HR,0.418;for radiotherapy without surgery:HR,0.405;and for surgery plus radiotherapy:HR,0.410).Interpretation:Age,stage at diagnosis,and treatment received were found to be the most important predictors of the overall survival of pediatric embryonal RMS.

The combined application of stem cells and three-dimensional bioprinting scaffolds for the repair of spinal cord injury

Spinal cord injury is considered one of the most difficult injuries to repair and has one of the worst prognoses for injuries to the nervous system.Following surgery,the poor regenerative capacity of nerve cells and the generation of new scars can make it very difficult for the impaired nervous system to restore its neural functionality.Traditional treatments can only alleviate secondary injuries but cannot fundamentally repair the spinal cord.Consequently,there is a critical need to develop new treatments to promote functional repair after spinal cord injury.Over recent years,there have been seve ral developments in the use of stem cell therapy for the treatment of spinal cord injury.Alongside significant developments in the field of tissue engineering,three-dimensional bioprinting technology has become a hot research topic due to its ability to accurately print complex structures.This led to the loading of three-dimensional bioprinting scaffolds which provided precise cell localization.These three-dimensional bioprinting scaffolds co uld repair damaged neural circuits and had the potential to repair the damaged spinal cord.In this review,we discuss the mechanisms underlying simple stem cell therapy,the application of different types of stem cells for the treatment of spinal cord injury,and the different manufa cturing methods for three-dimensional bioprinting scaffolds.In particular,we focus on the development of three-dimensional bioprinting scaffolds for the treatment of spinal cord injury.

Cardiac differentiation is modulated by anti-apoptotic signals in murine embryonic stem cells

BACKGROUND Embryonic stem cells(ESCs)serve as a crucial ex vivo model,representing epiblast cells derived from the inner cell mass of blastocyst-stage embryos.ESCs exhibit a unique combination of self-renewal potency,unlimited proliferation,and pluripotency.The latter is evident by the ability of the isolated cells to differ-entiate spontaneously into multiple cell lineages,representing the three primary embryonic germ layers.Multiple regulatory networks guide ESCs,directing their self-renewal and lineage-specific differentiation.Apoptosis,or programmed cell death,emerges as a key event involved in sculpting and forming various organs and structures ensuring proper embryonic development.How-ever,the molecular mechanisms underlying the dynamic interplay between diffe-rentiation and apoptosis remain poorly understood.AIM To investigate the regulatory impact of apoptosis on the early differentiation of ESCs into cardiac cells,using mouse ESC(mESC)models-mESC-B-cell lym-phoma 2(BCL-2),mESC-PIM-2,and mESC-metallothionein-1(MET-1)-which overexpress the anti-apoptotic genes Bcl-2,Pim-2,and Met-1,respectively.METHODS mESC-T2(wild-type),mESC-BCL-2,mESC-PIM-2,and mESC-MET-1 have been used to assess the effect of potentiated apoptotic signals on cardiac differentiation.The hanging drop method was adopted to generate embryoid bodies(EBs)and induce terminal differentiation of mESCs.The size of the generated EBs was measured in each condition compared to the wild type.At the functional level,the percentage of cardiac differentiation was measured by calculating the number of beating cardiomyocytes in the manipulated mESCs compared to the control.At the molecular level,quantitative reverse transcription-polymerase chain reaction was used to assess the mRNA expression of three cardiac markers:Troponin T,GATA4,and NKX2.5.Additionally,troponin T protein expression was evaluated through immunofluorescence and western blot assays.RESULTS Our findings showed that the upregulation of Bcl-2,Pim-2,and Met-1 genes led to a reduction in the size of the EBs derived from the manipulated mESCs,in comparison with their wild-type counterpart.Additionally,a decrease in the count of beating cardiomyocytes among differentiated cells was observed.Furthermore,the mRNA expression of three cardiac markers-troponin T,GATA4,and NKX2.5-was diminished in mESCs overexpressing the three anti-apoptotic genes compared to the control cell line.Moreover,the overexpression of the anti-apoptotic genes resulted in a reduction in troponin T protein expression.CONCLUSION Our findings revealed that the upregulation of Bcl-2,Pim-2,and Met-1 genes altered cardiac differentiation,providing insight into the intricate interplay between apoptosis and ESC fate determination.

Early embryonic failure caused by a novel mutation in the TUBB8 gene:A case report

BACKGROUND This study aimed to explore the relationship between gene mutations and early embryonic development arrest and to provide more possibilities for the diagnosis and treatment of repeated implantation failure.CASE SUMMARY Here,we collected and described the clinical data of a patient with early embryonic development stagnation after repeated in vitro fertilization attempts for primary infertility at the Department Reproductive Center of Zaozhuang Maternal and Child Healthcare Hospital.We also detected the whole-exon gene of the patient's spouse and parents,and conducted bioinformatics analysis to determine the pathogenesis of the gene.CONCLUSION A novel mutant of the TUBB8 gene[c.602G>T(p.C201F)]was identified,and this mutant provided new data on the genotype-phenotype relationships of related diseases.