Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primitive neuroectodermal tumor (PNET) is a small round cell malignancy arising in soft tissue and bone, predominantly in older children and adolescents. We report the cytomorphologic features and findings of ancillary studies of eight fine needle aspiration (FNA) biopsies from three patients (7-year-old male, 12-year-old female, 9-year-old female). Two of the biopsies suggested the initial diagnosis of PNET of the chest wall, while the remaining six documented recurrent or metastatic disease. In one of these cases the primary diagnosis made by FNA biopsy enabled the pediatric oncologists to give specific therapy for the unresectable tumor and achieve remission. Local recurrences included the chest wall (two cases), pleura (one case) and pericardium (one case), while metastatic disease involved the supraclavicular lymph node and breast. All the cases consisted of small malignant cells with a high nuclear/cytoplasmic ratio and hyperchromatic nuclei without prominent nucleoli. Homer Wright rosettes were seen in only two of the aspirates, and neuropil and ganglion cells were not present. Ancillary studies, including electron microscopy (two cases), immunocytochemistry (four aspirates from two cases) and cytogenetics (11/22 translocation, one case) performed on the aspirated material were aids in making a specific diagnosis and excluded other small round cell tumors of childhood, such as malignant lymphoma, rhabdomyosarcoma and Ewing's sarcoma. The differential diagnosis between PNET and neuroblastoma can be difficult on the basis of an FNA biopsy alone, although light microscopic morphologic differences exist. Clinical features (e.g., age, primary site, metastatic patterns), catecholamine levels, electron microscopy and cytogenetics are necessary in establishing the correct diagnosis.
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PMID:Fine needle aspiration cytology of primitive neuroectodermal tumors. A report of these cases. 132 99

We sought temporal trends in the demographic, clinical, histologic feature, diagnostic class, and quality of life data over the interval 1930-1979 in the Childhood Brain Tumor Consortium database. The proportion of children younger than eight years old declined from 72% to 55% and the proportion of those older than ten more than doubled from 12% to 27%. The relative frequency of tumors in the supratentorial compartment increased significantly, while infratentorial tumors decreased. We found significant declines in supratentorial ependymomas and pilocytic astrocytomas. Similarly, some infratentorial tumors, especially ependymomas, decreased and brain stem tumors increased. Infratentorial medulloblastoma (primitive neuroectodermal tumor) increased significantly. Some individual histologic features which are markers of anaplasia increased in frequency in both supratentorial and infratentorial tumors. There was a significant increase in biopsies that contained nonneoplastic neural tissue in addition to tumor for both compartments and among supratentorial tumors there was a marked increase in the proportion of cases containing an indistinct neural tissue boundary. The probability of postoperative death declined, but the probability of survival five or ten years after surgery did not improve significantly for children who had tumors in either compartment. Among children who survived five years after the initial craniotomy, the proportion who had significant long term deficits increased. Most of this increase occurred in the last decade (1970-79). In this decade, the proportion of children for whom no deficits were reported five years following operation was 4% if they had a supratentorial tumor and 27% if they had an infratentorial tumor. The proportions of children alive five years following first surgery who had arachnoidal metastases increased significantly for infratentorial tumors.
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PMID:Temporal trends among childhood brain tumor biopsies. The Childhood Brain Tumor Consortium. 143 31

Occurrence of embryonal kidney tumors in patients with primitive neuroectodermal tumors, so-called central nervous system-renal neoplasia has been reported. An infant who presented with masses in the right lateral ventricle and the cerebellar vermis is reported. Histological examination showed primitive neuroectodermal tumors. Further investigation revealed tumors in the bilateral kidneys, which were removed subtotally and pathologically shown to be Wilms' tumors. The patient was then treated with anticancer drugs and irradiation. However, he developed lung metastases from the renal tumors and expired. At autopsy, a small tumor was found in the inferior horn of the left lateral ventricle. Histological finding showed a primitive neuroectodermal tumor. Also, bilateral large masses of recurrent Wilms' tumors, multiple metastases to the lungs and peritoneal dissemination were found. There is no evidence that this association is based on the selective neoplastic transformation of embryonal cells of similar histogenetic or cytogenetic origin. Several reports demonstrate the presence of embryonal cells in the nervous tissue which could imply a neuroepithelial origin for Wilms' tumors.
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PMID:[Primitive neuroectodermal tumor with Wilms' tumor. Case report]. 172 36

Of 29 consecutive children treated for malignant primary tumors of the central nervous system (CNS) at this institution, postoperative examination showed radiographic or cytologic evidence of neuraxis dissemination in 10 (34%). Given the historically poor results in disseminated CNS tumors treated with surgery and radiation therapy alone, these ten patients were treated prospectively with an investigational Phase II protocol consisting of preirradiation cisplatin (90 mg/m2 on day 1) and etoposide (150 mg/m2 on days 3 and 4). The diagnoses included medulloblastoma (n = 4), malignant glioma (n = 3), cerebral primitive neuroectodermal tumor (n = 1), pineoblastoma (n = 1), and mixed glioma of the brainstem (n = 1). Postoperative neuraxis scanning with computed tomography, magnetic resonance imaging, or spinal myelography showed measurable intracranial or spinal metastases in all children. The cerebrospinal fluid (CSF) cytologic examination was positive for tumor cells in five. The best responses, based on serial imaging of neuraxis metastases, included two complete responses, four partial responses, and three stable disease states. One patient had progressive disease at the primary site despite stable disease in the spine; progressive neuraxis disease was documented in only one patient during chemotherapy. Clearance of tumor cells from the CSF was documented in three patients. The adverse effects of chemotherapy, consisting of transient myelosuppression and mild ototoxicity, were minimal. Reversible neurologic deterioration occurred in two patients; one patient became acutely quadriplegic after a prolonged convulsive seizure without radiographic evidence of tumor progression.
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PMID:Neuraxis dissemination in pediatric brain tumors. Response to preirradiation chemotherapy. 173 73

The authors reviewed the hospital charts of 415 pediatric patients treated for benign or malignant primary brain tumors over the past 20 years at the Children's Hospital Medical Center, Seattle. Patients' ages ranged from the neonatal period to 18 years. A shunt was placed in 152 patients (37%), 45 before and 94 after surgery. Confirmation of extraneural metastases was based on clinical and diagnostic examination. Factors analyzed as possibly influencing the occurrence of extraneural metastases were: 1) the shunt: type, valve, location, filter, and revisions; 2) extent of resection; 3) pathology; and 4) treatment regimen. Eight of the 415 patients developed extraneural metastases during life. All eight patients had a medulloblastoma (cerebellar primitive neuroectodermal tumor). These eight patients were separated into Group A (without a shunt) and Group B (with a shunt). In Group A (five patients), the mean interval from primary diagnosis to metastasis was 15 months. Two children had gross total resection of the tumor. The predominant location of metastases in Group A was: bone (two cases); cervical lymph nodes (one); lung/bone (one); and retroperitoneal pelvic mass (one). Three Group A patients had a simultaneous central nervous system (CNS) recurrence. Of the three Group B patients, two had a ventriculoperitoneal (VP) shunt and one a ventriculoatrial (VA) shunt; all were placed postoperatively. One Group B patient had a simultaneous CNS recurrence. No shunt revisions were performed in these three patients. The mean time from primary diagnosis to metastasis was 25 months. One patient had a total tumor resection. The predominant location of metastases was bone (one case), retroperitoneal pelvic mass (one), and abdominal cavity with ascites (one case). Only one patient in the entire series had a filter placed; this resulted in shunt obstruction and was removed 1 month following placement. It is concluded that cerebrospinal fluid shunts, regardless of type, location, revision rate, or filter insertion, do not predispose pediatric patients with brain tumors to develop extraneural metastases. A diagnosis of shunt-related metastases should be based on the development of intra-abdominal (VP shunt) or pulmonary (VA shunt) dissemination primarily with or without additional sites. The diagnosis of medulloblastoma is an important factor related to metastasis occurrence while the extent of resection and postoperative therapy are not influential.
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PMID:The risks of metastases from shunting in children with primary central nervous system tumors. 203 46

A cerebral primitive neuroectodermal tumor with astrocytic differentiation and extracranial metastases in a 28-day-old infant is reported. The infant presented with a progressively enlarged head, cutaneous lesions in the neck, and enlarged cervical lymph nodes. A computed tomography brain scan demonstrated a giant thalamic tumor with subarachnoid dissemination and hydrocephalus. Biopsy material from the cervical lesions showed a picture of glioma with anaplastic astrocytes. The patient received a ventriculoperitoneal shunt operation and palliative chemotherapy, but died at 3 months of age. Autopsy was performed. Histological studies, which included immunohistochemical stains of the thalamic tumor, showed small, round, primitive, neoplastic cells with focal astrocytic differentiation.
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PMID:Congenital cerebral primitive neuroectodermal tumor with astrocytic differentiation and extracranial metastases. 216 35

A primitive neuroectodermal tumor (PNET) presented as a cerebral hemispheric mass in a 33-year-old man. Bone marrow metastases were discovered 11 months later. A cell line (CHP707m) was derived from these metastases. In culture, the cells showed features of neuronal differentiation, forming short neurites and synthesizing low-molecular-weight neurofilament protein. Northern blotting showed the tumor cells express nerve growth factor (NGF) receptor messenger RNA, and fluorescence-activated cell-sorting demonstrated NGF receptors on the cell surface. Western blotting showed CHP707m NGF receptors are truncated. The receptors are functional; they bind iodine 125-labeled mouse NGF with an affinity of 1.6 x 10(-9) M, and short-term treatment with NGF induces expression by the tumor cells of the proto-oncogene, c-fos. Although CHP707m is the first central nervous system PNET cell line proven to express NGF receptors, immunohistological survey of tissue sections prepared from human central nervous system PNETs showed that 13 of 35 contained NGF receptor-positive tumor cells. Thus, more than one-third of such tumors might be responsive to the effects of NGF.
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PMID:Human central nervous system primitive neuroectodermal tumor expressing nerve growth factor receptors: CHP707m. 217 17

Osseous metastases from primary central nervous system (CNS) tumors are rare. The CNS tumors that most frequently metastasize to bone are the glioblastoma multiforme and the medulloblastoma. In this report, a 22-year-old woman sought treatment for a lytic lesion in her right proximal femur 18 months after a craniotomy to remove a pinealoblastoma, a rare primitive neuroectodermal tumor of the pineal gland. An extensive evaluation demonstrated no other primary tumors. A biopsy of the femoral lesion revealed that it was morphologically identical to the pinealoblastoma, and special immunohistochemical stains were performed that verified neuronal differentiation. This may be the first report of a pinealoblastoma associated with an extracranial skeletal metastasis.
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PMID:Extracranial skeletal metastasis from a pinealoblastoma. A case report and review of the literature. 267 97

A 69-year-old man had a melanotic primitive neuroectodermal tumor of the medulla displaying various neuroepithelial elements including undifferentiated neuroepithelial cells forming Homer Wright's rosettes as well as neoplastic neuroglia resembling those seen in medulloblastoma. The neuroglial tumor cells were verified by demonstrating glial fibrillary acidic protein (GFAP) in the cells. These findings support the concept that the primitive neuroectodermal tumor and medulloblastoma are similar neoplasms. They have been described by such diverse names as melanotic medulloblastomas and progonomas. Review of 18 reported cases of intracranial melanotic primitive neuroectodermal tumors, including the present one, reveals that they have common pathologic features, are most frequent in the cerebellum and fourth ventricle, often metastasize widely within the neuraxis or even systemically, occur more frequently in children than adults, and strike males more often than females.
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PMID:Melanotic primitive neuroectodermal (neuroepithelial) tumor of medulla. 301 24

In a retrospective study 820 tumors were immunohistochemically examined with anti-GFAP. All 224 astrocytomas and 105 of 112 glioblastomas were, at least focally, positive. 72% of ependymomas and 64% of oligodendrogliomas contained tumor cells which expressed GFAP. In such entities the reaction is dependent on the histologic subtype. Only 26 of 114 medulloblastomas (22.8%) demonstrated scattered GFAP positive cells. GFAP was also demonstrated in the CNS in gangliogliomas, monstrocellular sarcomas, 3 of 6 PNET, one non-classifiable tumor in a child, 1 plexus papilloma, in scattered stromal cells in 15 of 26 hemangioblastomas as well as in the mature glial component of intracranial germ cell tumors. Outside of the CNS there was evidence of GFAP in 3 cases with nasal glial heterotopy and in the myxoidal part of a pleomorphic salivary gland adenoma. Neoplasms which proved negative to GFAP in our series included purely neural differentiated tumors meningioma, neurolemmomas, chordomas, paragangliomas, sarcomas, lymphomas, melanomas and carcinoma metastases. Separating GFAP-positive reactive astrocytes from the actual tumor cells has proved to be a problem in the routine use of GFAP in differential diagnosis. Absence of an immunohistochemical response does not exclude a tumor of glial origin. Tissue samples which are too small, particularly in the case of anaplastic astrocytomas and glioblastomas can give false negative results.
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PMID:[Significance of immunohistochemistry for neuro-oncology. VI. Occurrence, localization and distribution of glial fibrillary acid protein (GFAP) in 820 tumors]. 342 11


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