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Results for “Neuroectodermal Tumors, Primitive, Peripheral”

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Imaging of soft tissue tumors with Tc(V)-99m dimercaptosuccinic acid. A new tumor-seeking agent.

Tumor scintigraphy, using Tc(V)-99m dimercaptosuccinic acid (Tc(V)-DMS) was performed in 58 patients with soft tissue tumors, and the results were compared with that of Ga-67 citrate. Tc(V)-DMS was found to have a sensitivity of 90% for malignant tumors including aggressive fibromatosis compared to that of Ga-67 citrate, which was 56%. However, the specificity of Tc(V)-DMS for these tumors was 71% but with Ga-67 citrate the specificity was 80%. The imaging accuracy in soft tissue tumors with Tc(V)-DMS and Ga-67 citrate was 78% and 71%, respectively. Although the accumulation of Tc(V)-DMS has been detected in some benign soft tissue tumors, the reduced accumulation in inflammatory lesions compared to Ga-67 citrate was recognized, and Tc(V)-DMS could be of great use in the detection of extension or location of malignant soft tissue tumors.

Abscess↗

Esthesioneuroblastoma imaged with bone scintigraphy.

An esthesioneuroblastoma, a rare malignancy of the olfactory nerve, was identified with bone imaging in a four year old male who presented with a history of headaches and proptosis of the right eye. Correlation with CT is provided.

Bone and Bones↗

Primitive neuroectodermal tumor arising in an ovarian mature cystic teratoma: immunohistochemical and electron microscopic studies.

The occurrence of a malignant neuroectodermal tumor in a mature cystic teratoma is extremely rare. Five cases of ovarian primitive neuroectodermal tumors (PNET) or neuroepitheliomas have been reported. In two, the tumor was adjacent to foci of a mature teratomatous element. We present the immunohistochemical profile and electron microscopic study of an ovarian mature cystic teratoma with PNET or malignant neuroepithelioma. The transition between the mature neural elements and the malignant PNET presents a model for monodermic neuroepithelial differentiation and explains the histogenesis of this unusual tumor.

Adult↗

MR imaging of esthesioneuroblastoma.

The CT, magnetic resonance (MR), and histological features of a case of esthesioneuroblastoma, an uncommon tumor arising from the olfactory epithelium of the nasal vault are reported. The response to radiotherapy was followed up by MR imaging.

Adult↗

Fine structure of medulloepithelioma.

A survey of published cases of medulloepitheliomas indicates that this tumor is highly malignant (average course 6 months), occurs in early childhood (average age 4,4 years) and has no sex predilection. Although it may originate in any part of the central nervous system, it is most common in the cerebral hemispheres. The first case studied with the electron microscope is reported here. The fine structure of the tumor is similar to that of the fetal neural tube in showing a primitive epithelium having uniform nuclei rich in chromatin but little cytoplasm and sparse organelles; it rests on a distinct basement membrane. The epithelial surface lacks cilia or microvilli and is covered with an irregular amorphous coat which is probably the cause of the light-microscopic misinterpretation of an inner limiting membrane. The lateral cell surfaces lack interdigitations but show extensive primitive forms of cell junctions. The case presented is also unusual in being the second case on record originating in the cerebellum and the first showing massive seeding along the spinal canal.

Cell Membrane↗

Neuronal and astrocytic differentiation in human neuroepithelial neoplasms. An immunohistochemical study.

Neuroepithelial neoplasms of childhood were examined immunohistochemically using antibodies against a neurofilament polypeptide and glial fibrillary acidic protein. Ninety-one cases, including 11 controls, were examined. Positively reacting cells, indicating neuronal and glial differentiation, were found in 59 of the 80 tumors. The study supports a neuroepithelial origin for medulloblastomas, central neuroblastomas, and primitive neuroectodermal tumors of childhood. The results also indicate that only a small number of the tumor cells differentiate along either neuronal or glial cell lines.

Astrocytoma↗

Cerebral medulloepithelioma with bone, cartilage, and striated muscle. Light microscopic and immunohistochemical study.

A two-and-a-half-year-old girl had a cerebral medulloepithelioma with histologic evidence of ependymal, astroglial, oligodendroglial, and neuroblastic differentiation, as well as islands of cartilage and bone, and a microscopic focus of striated rhabdomyoblasts. Immunohistochemical staining for glial fibrillary acidic protein and S-100 protein with the peroxidase-antiperoxidase technique revealed positive staining in the various differentiating neural elements of the tumor. Mesectodermal differentiation, or the production by neuroectoderm of tissues usually considered to be mesodermal, is being increasingly recognized in the fields of embryology and tissue culture. Although this is the likely explanation for the composition of this tumor, other possibilities are considered as well.

Bone and Bones↗

Patterns of epithelial metaplasia in malignant gliomas. I. Papillary formations mimicking medulloepithelioma.

Adenoid-like formations resembling ducts and glands or forming a cribriform pattern have previously been described in malignant gliomas, resulting in some cases in a confusion with metastatic adenocarcinoma. The interpretation of these structures as being composed of anaplastic glial cells rests partly on the presence of transitions to more differentiated neoplastic astrocytes and partly on the positivity of some of these cells for glial fibrillary acidic protein. In this report two cases are presented in which the adenoid pattern was associated with papillary formations mimicking the arrangement of a medulloepithelioma. These structures represent a form of aberrant neoplastic differentiation in a malignant glioma rather than the expression of an embryonal neuroepithelial neoplasm.

Adult↗

Early outcome and complications of the extended subcranial approach to the anterior skull base.

OBJECTIVES: To present the technique of the extended subcranial approach to the anterior skull base and to review the results in 55 patients who underwent the procedure. STUDY DESIGN: Retrospective review of the records of 55 patients who underwent the extended subcranial approach to the anterior skull base between 1994 and 1998 for the treatment of various neoplasms originating in the nasal cavity, nasopharynx, paranasal sinuses, orbit, or meninges, as well as for the repair of complex craniofacial trauma and/or cerebrospinal fluid (CSF) leak. Preoperative patient evaluation and the surgical technique are also reviewed. METHODS: Patient records were retrospectively reviewed and tabulated for age, sex, and indications for procedure, with special focus on early outcome and complications. RESULTS: Twenty-six patients underwent oncologic resections, 22 patients had reduction of complex fronto-naso-orbital and skull base fractures, and seven patients had repair of CSF leak. Significant complications in the oncologic group consisted of one hematoma requiring needle aspiration and two cases of temporary nontension pneumocephalus. In the fracture group, one patient died because of extensive intracerebral damage and multiorgan failure, and one patient had nontension pneumocephalus coupled with CSF leakage and one patient had temporary nontension pneumocephalus. The most common late complication in all three groups was anosmia. CONCLUSIONS: Based on their review, the authors conclude that the extended subcranial approach to the anterior skull base is a safe, versatile, and effective procedure for the surgical treatment of various pathological conditions involving the anterior skull base.

Adolescent↗

Esthesioneuroblastoma with intracranial extension.

The authors present five cases of olfactory neuroblastoma with intracranial extension operated on in the Department of Neurosurgery in collaboration with otorhinolaryngologists. This tumor is most frequently reported as growing inside the nasal cavities, and it can extend to the paranasal sinuses. Cases presenting initially as intracranial tumors have been infrequently reported. At present, histological diagnosis of this tumor is aided by the use of electron microscopy and immunocytochemistry. Total resection combined with radiation therapy remains the most satisfactory treatment.

Adolescent↗

Primary intramedullary primitive neuroectodermal tumor of the spinal cord: case report and review of the literature.

OBJECTIVE AND IMPORTANCE: Primary intraspinal primitive neuroectodermal tumors (PNETs) are rare. We report a case and review the literature. CLINICAL PRESENTATION: A 22-year-old woman presented with rapidly progressive paraparesis and neurogenic bladder. INTERVENTION: Preoperative computed tomography myelograms revealed a complete block at T12-L1, consistent with an intramedullary lesion. An urgent operation was performed with gross total tumor removal. The pathological findings were consistent with a PNET. Recurrence was noted within 10 weeks of surgery and has been somewhat responsive to chemotherapy and radiotherapy thus far. A review of the English literature shows that only 13 cases of primary intraspinal PNETs have been reported to date, and the present case is the second one in which the tumor was purely intramedullary. Most of the reported patients survived less than 2 years. CONCLUSION: Primary intraspinal PNETs are rare tumors and carry a poor prognosis.

Adult↗