Child ; Family ; Humans ; Neuroectodermal Tumors, Primitive/pathology* ; Neuroectodermal Tumors, Primitive, Peripheral/pathology* ; Oropharynx

Humans ; Kidney ; pathology ; Kidney Neoplasms ; pathology ; Neuroectodermal Tumors, Primitive ; pathology

Female ; Humans ; Neuroectodermal Tumors, Primitive ; pathology ; Ovary ; pathology

The MRI appearances of 7 adult patients with pathologically proven intracranial primitive neuroectodermal tumors (PNET) were retrospectively analyzed. The MRI features were compared with findings in pathology and surgery. In this group, the tumor masses were most commonly found in the semisphere of cerebrum and in the vermis of cerebellum. They were relatively large and 4 were in lobulated shape. All of them had well-defined margins. MR images showed the tumors to be mildly or obviously hypointense on T1-weighted images and hyperintense on T2-weighted images. Most masses had heterogeneous appearances with some cystic and necrotic areas. Intratumoral haemorrhage and focal calcification were occasionally seen. Mostly, there was no or only mild surrounding edema. Marked inhomogeneous contrast enhancement on MRI was seen in 6 cases except one. Two patients with multiple intracranial metastases were revealed on MR images. In this series, there was good correlation between MRI features and findings in pathology and surgery. These results showed that certain MRI features might suggest the diagnosis of intracranial PNET in adults. MRI is an effective technique to detect these tumors and is helpful to treatment planning and follow-up.
Brain Neoplasms/*diagnosis ; Brain Neoplasms/*pathology ; *Magnetic Resonance Imaging ; Neuroectodermal Tumors, Primitive/*diagnosis ; Neuroectodermal Tumors, Primitive/*pathology ; Retrospective Studies

Child ; Female ; Humans ; Lumbar Vertebrae ; Neuroectodermal Tumors, Primitive ; pathology ; therapy ; Spinal Neoplasms ; pathology ; therapy

Adolescent ; Child ; Humans ; Male ; Neuroectodermal Tumors, Primitive, Peripheral ; pathology ; Sarcoma, Ewing ; pathology

Diagnosis, Differential ; Humans ; Infant ; Male ; Neuroectodermal Tumors, Primitive ; pathology ; Orbital Neoplasms ; pathology

Primitive neuroectodermal tumor (PNET) is a small round cell malignancies, normally confined to the chest wall and soft tissues of children and young adults, and reports of this tumor existing in nasal cavity are scarce. We herein present a case of nasal PNET. A 35-year-old male patient presented with a chief complaint of stuffy nose was diagnosed with a primary nasal pPNET by the clinical, microscopic, immunohistochemical features. Since these tumors are highly aggressive and patient prognosis is typically very poor, early diagnosis, immediate surgical resection and re-resection if possible, early radiotherapy and chemotherapy and close follow-up are required. We also review literatures on pPNET.
Adult ; Humans ; Male ; Neuroectodermal Tumors, Primitive ; pathology ; Nose Neoplasms ; pathology ; Paranasal Sinuses ; Prognosis

Four children, with histopathologically confirmed posterior fossa primitive neuroectoderrnal tumors, were examined by plain radiography, computed tomography (CT) and cerebral angiography. The homogeneously well enhanced solid mass in the midline of the posterior fossa and hydrocephalus of various degree were seen on all CT scans. One case had calcifications and another case had low density areas in the tumor mass. Three cerebral angiograms showed vascular displacement without tumor vascularities. Unfortunately, these CT and angiography findings are and other tumors. when a well enhanced solid mass in the midline posterior fossa is seen on CT scan in children.
Angiography ; Cerebral Angiography ; Child ; Humans ; Hydrocephalus ; Medulloblastoma* ; Neuroectodermal Tumors, Primitive* ; Pathology* ; Radiography ; Tomography, X-Ray Computed

Medulloepithelioma, a rare tumor, arises from the epithelium of the medullary tube. In this article, we present a 3-year-old boy who suffered from secondary glaucoma, initially presumed the primary disease was endophthalmitis. Subconjunctival mass was later found, pathologically proved to be medulloepithelioma. We discuss the patient management with emphasis on the early signs of examination and the role of ultrabiomicroscopy (UBM) in evaluating pediatric secondary glaucoma and in influencing the management of patients with medulloepithelioma.
Child, Preschool ; Ciliary Body ; pathology ; Eye Neoplasms ; diagnosis ; Glaucoma ; Humans ; Male ; Neuroectodermal Tumors, Primitive ; diagnosis