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

Primary primitive neuroectodermal tumors (PNET) are rare malignant tumors, affecting mostly children and adolescents. Only three cases of primary breast PNETs have been reported in the medical literature, with none in Korea. We present a case of a primary PNET of the breast in a 33-year-old woman, with imaging and immunohistopathology findings.
Adult ; Breast Neoplasms/*diagnosis/pathology ; Female ; Humans ; Immunohistochemistry ; Mammography ; Neuroectodermal Tumors/*diagnosis/pathology ; Positron-Emission Tomography

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

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

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.
Adult ; Brain Neoplasms ; diagnosis ; pathology ; Female ; Humans ; Magnetic Resonance Imaging ; Male ; Neuroectodermal Tumors, Primitive ; diagnosis ; pathology ; Retrospective Studies

Humans ; Infant, Newborn ; Male ; Mediastinal Neoplasms ; diagnosis ; pathology ; Neuroectodermal Tumors, Primitive ; diagnosis ; pathology ; Tomography, X-Ray Computed

OBJECTIVETo study clinicopathologic features, immunohistochemical profile, diagnosis and differential diagnosis of childhood central nervous system primitive neuroectodermal tumors (CNS PNETs) with the features of ependymoblastoma and neuroblastoma.

METHODSThe clinical data, morphologic and immunohistochemical features were analyzed in 4 cases of pediatric CNS PNETs with features of ependymoblastoma and neuroblastoma. EnVision method immunohistochemistry was applied.

RESULTSFour patients including three boys and one girl presented at the age from 12 month to 4 years and three tumors located in cerebrum, one in brain stem. All tumors showed typical combined histological patterns of ependymoblastoma and neuroblastoma, demonstrating zones of true rosettes, occasional pseudovascular rosettes, and undifferentiated neuroepithelial cells in a prominent background of mature neuropils. There was focal expression of glial fibrillary acidic protein (GFAP) consistent with glial differentiation and epithelial membrane antigen (EMA) consistent with ependymal differentiation. Necrosis was seen in three cases and calcification was present in one case. Immunohistochemically, the rosettes and undifferentiated neuroepithelial cells were positive for vimentin, partially positive for GFAP and EMA but negative for synaptophysin. The tumor cells were also positive for synaptophysin in neuropils. The Ki-67 label index ranged from 20% to 60%.

CONCLUSIONSCNS PNETs with the features of ependymoblastoma and neuroblastoma is a rare tumor with poor prognosis. The tumor primarily occurs in childhood, especially infant and belongs to the family of embryonal tumors of the CNS. The morphologic, immunohistochemical and genetic features are important in differential diagnosis from other tumors of the CNS.

Antigens, Neoplasm ; metabolism ; Central Nervous System ; pathology ; Child ; Female ; Glial Fibrillary Acidic Protein ; metabolism ; Humans ; Immunohistochemistry ; Infant ; Male ; Mucin-1 ; metabolism ; Neuroblastoma ; diagnosis ; pathology ; Neuroectodermal Tumors, Primitive ; diagnosis ; pathology ; Neuroectodermal Tumors, Primitive, Peripheral ; diagnosis ; pathology ; Synaptophysin ; metabolism ; Vimentin ; metabolism

OBJECTIVETo review the clinical characteristics, diagnosis and treatments of primitive neuroectodermal tumor (PNET) in head and neck.

METHODSA retrospective review of the medical records for the cases of PNET in head and neck from 2004 to 2014. General clinical information including diagnosis and treatments was obtained and analyzed. A literature review was also conducted.

RESULTSA total of 5 cases diagnosed with PNET were included. Of 5 patients, one patient with lesion in the temporal field was treated with radical resection, followed by radiotherapy, and a 24-month follow-up showed no recurrence. Three patients presented with maxillary, infratemporal fossa or cheek invasion respectively, after diagnosed with pathological examination, one patient received preoperation radiotherapy plus radical resection and postoperative chemotherapy, and the lesions had no obvious progress with follow-up of 106 months; one patient was applied with preoperative chemotherapy plus extensive resection and postoperation radiotherapy, showed recurrence 15 months later, and was lost to follow-up; and another patient underwent chemotherapy plus radiotherapy and extensive resection, presented with pulmonary metastasis 5 months later, and died of brain metastasis within 25 months. One foreign patient presented with tumor involved submaxillary, mouth and tongue, the tumor was reduced obviously after chemotherapy, but he was lost to follow-up after getting home.

CONCLUSIONSPNET in head and neck is rare and the clinical profile of PNET is the presence of occupying and compression. The pathology examination is an only way to confirm the diagnosis of PNET. The combination of excision, radiotherapy and chemotherapy is the treatment choice.

Brain Neoplasms ; secondary ; Head and Neck Neoplasms ; diagnosis ; pathology ; therapy ; Humans ; Lost to Follow-Up ; Male ; Neoplasm Recurrence, Local ; Neuroectodermal Tumors, Primitive ; diagnosis ; pathology ; therapy ; Retrospective Studies

Brain Neoplasms ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Humans ; Infant ; Magnetic Resonance Imaging ; Male ; Neoplasm Recurrence, Local ; Nestin ; metabolism ; Neuroectodermal Tumors, Primitive ; metabolism ; pathology ; surgery ; Temporal Lobe ; Vimentin ; metabolism

OBJECTIVETo study the clinicopathological features and diagnostic criteria of Ewing's sarcoma/primitive neuroectodermal tumor (Ewing's sarcoma/PNET) in oral and maxillofacial region.

METHODSThere were 15 patients with Ewing's sarcoma/PNET in the last 35 years at our hospital, aged 1-49 years and mean 14.5 years. The most common manifestation was swelling of the affected region. The cases were analyzed and histological and immunohistochemical studies were also conducted to examine CD99 (12E7), Vimentin, NSE, S-100, Syn, CD45 (LCA), desmin.

RESULTS(1) The most common histological pattern of Ewing's sarcoma/PNET was a lobular arrangement of uniform, small, hyperchromatic cells in a fibrous background. Some of these tumors were rich in cytoplasmic glycogen. (2) Of the 7 cases, IHC was positive for CD99 (12E7) and Vimentin and negative for lymphoid (CD45), muscle (desmin) markers. S-100 was positive in 4 cases and NSE negative in 3, Syn positive in 1. NSE, S-100, Syn were all negative in 2 cases.

CONCLUSIONSEwing's sarcoma/PNET is more common in teenagers and young people. Immunohistochemistry is essential to distinguish Ewing's sarcoma/PNET from other small round cell tumors. Immunohistochemistry is useful in the diagnosis.

Adolescent ; Adult ; Child ; Child, Preschool ; Diagnosis, Differential ; Facial Neoplasms ; diagnosis ; pathology ; Female ; Humans ; Infant ; Jaw Neoplasms ; diagnosis ; pathology ; Male ; Middle Aged ; Neuroectodermal Tumors, Primitive, Peripheral ; diagnosis ; pathology ; Sarcoma, Ewing ; diagnosis ; pathology ; Young Adult