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

Fatal Outcome ; Humans ; Magnetic Resonance Imaging ; Male ; Mediastinal Neoplasms ; complications ; diagnostic imaging ; pathology ; surgery ; Mediastinum ; diagnostic imaging ; pathology ; Neuroectodermal Tumors, Primitive, Peripheral ; complications ; diagnostic imaging ; pathology ; surgery ; Radiography ; Spinal Cord Diseases ; diagnostic imaging ; etiology ; pathology ; Young Adult

Malignant kidney neoplasms are the most frequently encountered solid kidney masses. Although renal cell carcinoma is the major renal malignancy, other solid malignant renal masses should be considered in the differential diagnosis of solid renal masses that do not contain a macroscopic fatty component. In this pictorial essay, we present the imaging findings of a primitive neuroectodermal tumor, primary liposarcoma of the kidney, primary neuroendocrine tumor, leiomyosarcoma, synovial sarcoma, malignant fibrous histiocytoma, sclerosing fibrosarcoma and renal metastasis of osteosarcoma.
Bone Neoplasms/secondary ; Carcinoma, Renal Cell/pathology/radiography ; Diagnosis, Differential ; Fibrosarcoma/radiography ; Histiocytoma/radiography ; Humans ; Kidney Neoplasms/*pathology/radiography ; Leiomyosarcoma/pathology/radiography ; Magnetic Resonance Imaging ; Middle Aged ; Neuroectodermal Tumors, Primitive/pathology/radiography ; Osteosarcoma/pathology ; Sarcoma ; Sarcoma, Synovial/radiography ; Tomography, X-Ray Computed

OBJECTIVETo analyze the magnetic resonance imaging (MRI) features of supratentorial primitive neuroectodermal tumors (sPNET) and improve the diagnosis of this disease.

METHODSMRI manifestations of 14 cases of PNET were retrospectively analyzed and compared with their pathological features.

RESULTSThe supratentorial lesions involved the occipital lobe in 4, frontal lobe in 3, fronto-occipital lobe in 2, temporo-occipital lobe in 3, lateral ventricle in 1 case and the saddle region in 1. All the lesions were large in volume and most of them presented heterogeneous signals in MRI. Of the 14 cases, 12 showed cystic degeneration and necrosis, 2 had hemorrhage and 6 showed signs of emptied small blood vessels. Twelve cases had heterogeneous enhancement and 2 had moderate enhancement. Pathologically, 10 cases of neuroblastomas were identified, along with 3 ganglioneuroblastomas and 1 atypical rhabdoid tumor.

CONCLUSIONMRI findings of PNET are rather characteristic to assist in the diagnosis and treatment of the disease, but a final definite diagnosis still relies on pathological examination.

Adolescent ; Adult ; Aged ; Child ; Child, Preschool ; Female ; Humans ; Magnetic Resonance Imaging ; methods ; Male ; Middle Aged ; Neuroectodermal Tumors, Primitive ; diagnosis ; diagnostic imaging ; pathology ; Radiography ; Retrospective Studies ; Sensitivity and Specificity ; Supratentorial Neoplasms ; diagnosis ; diagnostic imaging ; pathology ; Young Adult

OBJECTIVE: To comprehensively analyze the spectrum of imaging features of the primary tumors and metastatic patterns of the Extraskeletal Ewing sarcoma family of tumors (EES) in adults. MATERIALS AND METHODS: We performed a computerized search of our hospital's data-warehouse from 1996 to 2013 using codes for Ewing sarcoma and primitive neuroectodermal tumors as well as the demographic code for > or = 18 years of age. We selected subjects who were histologically confirmed to have Ewing sarcoma of extraskeletal origin. Imaging features of the primary tumor and metastatic disease were evaluated for lesion location, size, enhancement pattern, necrosis, margin, and invasion of adjacent organs. RESULTS: Among the 70 patients (mean age, 35.8 +/- 15.6 years; range, 18-67 years) included in our study, primary tumors of EES occurred in the soft tissue and extremities (n = 20), abdomen and pelvis (n = 18), thorax (n = 14), paravertebral space (n = 8), head and neck (n = 6), and an unknown primary site (n = 4). Most primary tumors manifested as large and bulky soft-tissue masses (mean size, 9.0 cm; range, 1.3-23.0 cm), frequently invading adjacent organs (45.6%) and showed heterogeneous enhancement (73.7%), a well-defined (66.7%) margin, and partial necrosis/cystic degeneration (81.9%). Notably, 29 patients had metastatic disease detected at their initial diagnosis. The most frequent site of metastasis was lymph nodes (75.9%), followed by bone (31.0%), lung (20.7%), abdominal solid organs (13.8%), peritoneum (13.8%), pleura (6.9%), and brain (3.4%). CONCLUSION: Primary tumors of EES can occur anywhere and mostly manifest as large and bulky, soft-tissue masses. Lymph nodes are the most frequent metastasis sites.
Adolescent ; Adult ; Aged ; Asian Continental Ancestry Group ; Bone Neoplasms/*pathology/radiography ; Brain Neoplasms/pathology/radiography ; Female ; Humans ; Lymph Nodes/pathology ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Neoplasm Metastasis ; Neuroectodermal Tumors, Primitive/pathology/radiography ; Positron-Emission Tomography ; Sarcoma, Ewing/*pathology/radiography ; Tomography, X-Ray Computed ; Young Adult

OBJECTIVETo investigate the CT and MRI features of peripheral primitive neuroectodermal tumors (pPNETs) and evaluate its diagnostic value.

METHODSThe clinicopathological data of 9 surgically treated patients with peripheral primitive neuroectodermal tumors confirmed by pathology were collected, spiral CT (4/9) and MRI (6/9) plain scanning and dynamic enhancement scanning were performed preoperatively. Both CT and MRI scannings were performed in 1 case. Those CT and MR images were retrospectively reviewed and analyzed together with clinicopathological findings.

RESULTSThe 9 lesions were located in skeletal muscles (n = 6), pelvic cavity (n = 2) and thoracic cavity (n = 1). The tumor size was 7.4-18.3 cm in diameter with a mean diameter of 11.6 cm. The shape of those lesions was round or ellipse (4 lesions) and irregular (5 lesions). The tumor usually presented as ill-defined masses, with homogeneous (n = 2) or inhomogeneous density (n = 7). Seven cases, including the 3 lesions located in the chest and pelvis, showed obvious necrosis and multilocular cyst formation. The tumors showed iso-density as that of the adjacent muscles on CT plain scans and moderate heterogeneous enhancement after intravenous injection of contrast agents. The features of the tumors on the MRI including slightly low signal intensity on SE T1-weighted imaging, iso-signal intensity or slightly high signal intensity on FSE T2-weighted imaging and heterogeneous dynamic delayed contrast-enhancement with obvious necrosis in most of them. Six cases had a lesion in the skeletal muscles, presented as a giant ill-defined masse surrounding bone and extended along neural route with bone destruction to varying degrees.

CONCLUSIONPrimitive neuroectodermal tumor is a kind of malignant tumor with proliferation of small, undifferentiated neuroectodermal cells, usually occurring in children or adolescent and frequently located in the extremities, chest cavity, pelvic cavity and chest wall. It typically presents as a large, ill-defined masse extending along neural route with heterogeneous and obvious enhancement after intravenous injection of contrast agents. The tumors located in the chest and pelvic cavities and some in the extremities show obvious necrosis and multilocular cyst formation, while some of the tumors in the extremities appear as homogeneous solid masses.

Adolescent ; Adult ; Child ; Diagnostic Errors ; Female ; Histiocytoma, Malignant Fibrous ; diagnosis ; Humans ; Male ; Muscle Neoplasms ; diagnosis ; diagnostic imaging ; pathology ; Muscle, Skeletal ; Neuroectodermal Tumors, Primitive, Peripheral ; diagnosis ; diagnostic imaging ; pathology ; Pelvic Neoplasms ; diagnosis ; diagnostic imaging ; pathology ; Radiography ; Retrospective Studies ; Thoracic Neoplasms ; diagnosis ; diagnostic imaging ; pathology ; Young Adult

Adult ; Antineoplastic Agents ; therapeutic use ; Benzenesulfonates ; therapeutic use ; Female ; Humans ; Kidney Neoplasms ; diagnostic imaging ; drug therapy ; Neuroectodermal Tumors, Primitive ; diagnostic imaging ; drug therapy ; Niacinamide ; analogs & derivatives ; Phenylurea Compounds ; Pregnancy ; Pyridines ; therapeutic use ; Radiography ; Thrombosis ; diagnostic imaging ; pathology ; Vena Cava, Inferior ; diagnostic imaging ; pathology