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.

Primitive Neuroectodermal Tumor as the Second Malignant Neoplasm in a Long-Term Survivor Child of Acute Lymphoblastic Leukemia: A Case Report

Acute lymphoblastic leukemia(ALL)is a common pediatric cancer.The second malignant neoplasms(SMNs)in long-term survivors of pediatric ALL are relatively rare.Herein we report a 10-year-old girl who was diagnosed as primitive neuroectodermal tumor(PNET)5 years after the initial diagnosis of ALL with radiotherapy・free treatment.PNET is an exceedingly rare neoplasm in SMNs of survivors of childhood ALL.It is predisposed to be misdiagnosed and the pathogenesis is unclear.The outcome is poor.Long-term follow-up is necessary for the survival children of ALL.

Primitive neuroectodermal tumor of the prostate in a 58-year-old man:A case report

BACKGROUND Primitive neuroectodermal tumor(PNET),especially located in the prostate,is a rare tumor that mainly occurs in young men.Bladder or rectum invasion and distant metastasis are strongly associated with a poor prognosis.Combination therapy,including radical surgery,adjuvant chemotherapy,and radiotherapy,is available.We present a case of prostatic PNET and a review of 17 cases identified in the literature.CASE SUMMARY A 58-year-old man was admitted complaining of dysuria for 2 years.Computed tomography and magnetic resonance imaging showed a large cystic-solid mass in the pelvic cavity compressing the surrounding bladder and rectum.The mass was iso-to hyperintense on T1-weighted imaging(WI)and heterogeneously hyperintense on T2WI.Cystic degeneration and necrosis were seen in the tumor,and solid tissues within the mass enhanced on contrast-enhanced scan.The patient underwent robot-assisted laparoscopic pelvic tumor resection.Histologically,the presence of many small round cells that were positive for expression of CD99,vimentin,and synaptophysin established the diagnosis of PNET in the prostate after surgery.The patient underwent adjuvant chemotherapy.During 34 mo of follow-up,the patient had no signs or symptoms of recurrence or residual disease.CONCLUSION We present the case of the oldest prostatic PNET patient,who has a good prognosis.This illustrates how older men with prostatic PNET may also benefit from the combination therapy,like younger adults,and achieve a long-term survival.As always,PNET should be considered in the differential diagnosis of aggressive prostatic tumors in young men.

Total pelvic exenteration and a new model of diversion for giant primitive neuroectodermal tumor of prostate: A case report and review of the literature

The present study reports a rare primitive neuroectodermal tumor (PNET) of prostate.A 27-year-old male was admitted to Harbin Medical University Cancer Hospital (Harbin,China) for dysuria and dyschezia. Magnetic resonance imaging (MRI) revealed a large mass thatmay involve the bladder and rectum next to the prostate. Histopathological analysis of biopsyof prostate indicated mesenchymal origin tumor, and immunohistochemistric stainingconfirmed diagnosis of PNET of prostate. En bloc total pelvic exenteration and double barrelsigmoidostomy were performed. Double stomas in the skin incision were used for fecal andurinary diversion, respectively. Short-term outcome is satisfactory, while long-term efficacyremains to be poor. Clinical features of PNET of prostate should be paid much more attentionand radical surgery and adjuvant chemotherapy should be recommended.

Ewing sarcoma/primitive neuroectodermal tumor of the ureter:A case report and literature review

Ewing sarcoma/primary neuroectodermal tumors are rare,invasive,and small round blue cell tumors.There are few reports of its occurrence in the urinary system.Here,we present the first middle-aged female patient whose Ewing sarcoma primary site was in the ureter.The main clinical manifestation was intermittent hematuria.She was in good health after complete surgical resection and adjuvant radiotherapy.To date,there has been no recurrence or metastasis.Accurate early diagnosis and appropriate treatment can help prolong survival.18F-fluorodeoxyglucose positron emission tomography/computed tomography is expected to be an effective means of evaluating treatment effects and detecting metastasis and recurrence.In this article,besides introducing a case of Ewing sarcoma/primitive neuroectodermal tumor of the ureter,we review the literature to discuss the current status of diagnosis and treatment.

Diagnosis and treatment of spinal primitive neuroectodermal tumor

Objective To sum up the clinical experience in diagnosis and treatment of spinal primitive neuroectodermal tumor(PNET). Methods Thirteen patients with spinal PNET were included in the study from 1999 to 2009.There were 8 males and 5

Clinical pathologic analysis of urologic primary primitive neuroectodermal tumor

Objective To explore clinico-pathological features,immunophenotype,treatment and prognosis of urologic primary primitive neuroectodermal tumor ( PNET) . Methods The clinical data of 3 patients with urologic PNET were analyzed retrospectively. All patients were male,aged 29,32 and 75 years respectively.

Peripheral Primary Neuroectodermal Tumour of the Ileum: A Case Report and Review of the Literature

Background: Ewing’s Sarcoma (ES)/peripheral Primitive Neuroectodermal Tumor (pPNET) is a rare aggressive malignant small round cell tumour. Most of them arise in bony sites, and they represent the second commonest primary osseous malignancy in adolescence and young adults. The localization of the small bowel is very rare, to our knowledge only 35 cases of ES/pPNET of the small bowel have been reported in the literature. Case Presentation: We report a case of pPNET of the ileum in a 26-year-old female, presented abdominal pain with a transit disorder. The clinical examination was unremarkable. The histological and immunohistochemical study confirmed the diagnosis of peripheral primary neuroectodermal tumours. The patient was treated by tumor resection followed by adjuvant chemotherapy. The evolution was good, without recurrence with a follow-up of 12 months. Conclusion: PNET of the ileum is very rare. We report this case to enrich the database of this rare clinical entity and to improve the management of patients with PNET of the ileum.

Imaging Findings of Hepatic Ewing’s Sarcoma on Computed Tomography and Gadobenate Dimeglumine-enhanced Magnetic Resonance Imaging: A Case Report and Literature Review

Ewing’s sarcoma(ES)is a tumor that often occurs in the long bones and rarely arises from visceral organs primarily.Here,we report a case of primary hepatic ES,discuss its computed tomography(CT)and gadobenate dimeglumineenhanced magnetic resonance(MRI)features.This is the first Chinese and fifth primary hepatic ES case reported,based on a literature review.Imaging examinations showed that the tumor was solid,with necrosis and hemorrhage.Contrast-enhanced images showed that the tumor was hypervascular and especially had heterogeneous signal intensity on hepatobiliary phase MRI images.Intratumoral vessels and vascular invasion were also present.