Cushing's syndrome caused by giant Ewing's sarcoma of the kidney:A case report and review of literature

BACKGROUND Primary renal Ewing’s sarcoma(ES)is extremely rare,and only two cases causing Cushing’s syndrome(CS)have been reported to date.We report that the case of an 18-year-old patient is diagnosed primary renal ES with typical CS characterized by purple stripes,weight gain,and hypertension.CASE SUMMARY CS was first diagnosed by laboratory testing.A huge tumor was revealed in the kidney following an imaging examination.Moreover,brain and bone metastases were observed.After comprehensive treatment,primarily based on surgery,primary renal ES was pathologically diagnosed with a typical EWSR1-FLI1 genetic mutation through genetic testing.Furthermore,the glucocorticoid level returned to normal.By the ninth postoperative month of follow-up,the patient was recovering well.Cushing-related symptoms had improved,and a satisfactory curative effect was achieved.CONCLUSION Primary renal ES,a rare adult malignant tumor,can cause CS and a poor prognosis.

EWING’S SARCOMA CAN EXPRESS BONE MORPHOGENETIC PROTEIN

Paraffin sections from 6 cases of Ewing's sarcoma were immunostained by ABC method using BMP -McAb for investigating the existence of BMP in Ewing' s sarcoma. All cases were positive. The result was coincided with human tumor transplants in athymic nude mouse by Bauer. It is shown that Ewing' s sarcoma can express BMP. So we support the hypothesis that Ewing' s sarcoma originates from primitive multipotential cells and can pluripotentially differentiate.

An unusual biconvex epidural lesion:acutely presenting extraosseous intracranial Ewing's sarcoma

Background:Ewing's sarcoma family of tumors consists of small round cell neoplasms,inclusive of primitive neuroectodermal tumor(PNET),Askin's tumor,and PNET of the bone.Extraosseous Ewing's sarcoma occurs commonly at bones of lower extremities and paravertebral region of the spine.It rarely presents as a primary intracranial lesion.When intracranial,it can be misdiagnosed as central PNET(e.g.,medulloblastoma,pinealoblastoma,or supratentorial PNET),other intracranial lesions,or even as an epidural hematoma.Case presentation:We report the case of a 20-year-old patient who presented to the emergency department with complaints of drowsiness,headache,and fever for 1 day.On initial computed tomography(CT)scan of the brain,a right temporal biconvex epidural lesion involving squamous-temporal bone with periosteal reaction was noted.The patient underwent urgent craniotomy,and a tumor was found and excised.Biopsy report confirmed Ewing's sarcoma.Conclusion:Ewing's sarcoma is a rare intracranial malignancy with only a few cases reported in literature.In a young patient with a biconvex epidural lesion,in the absence of trauma or ongoing infection,the possibility of Ewing's sarcoma should be considered as well.

A transcription assay for EWS oncoproteins in Xenopus oocytes

Aberrant chromosomal fusion of the Ewing's sarcoma oncogene(EWS)to several different cellular partners produces the Ewing's family of oncoproteins(EWSfusion-proteins,EFPs)and associated tumors(EFTs).EFPs are potent transcriptional activators,dependent on the N-terminal region of EWS(the EWS-activationdomain,EAD)and this function is thought to be central to EFT oncogenesis and maintenance.Thus EFPs are promising therapeutic targets,but detailed molecular studies will be pivotal for exploring this potential.Such studies have so far largely been restricted to intact mammalian cells while recent evidence has indicated that a mammalian cell-free transcription system may not support bona fide EAD function.Therefore,the lack of manipulatable assays for the EAD presents a significant barrier to progress.Using Xenopus laevis oocytes we describe a plasmid-based micro-injection assay that supports efficient,bona fide EAD transcriptional activity and hence provides a new vehicle for molecular dissection of the EAD.