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PubMed1996

Expression of BCL-2 gene product in embryonal tumors of the central nervous system.

BACKGROUND: The expression of bcl-2 is associated with suppression of programmed cell death and prolonged cell survival. Recently, immunoreactivity to the bcl-2 gene product has been reported not only in a variety of embryonal and adult nonhematopoietic tissues, but also in neuroblastoma. However, the study of bcl-2 expression has not been performed in brain tumors. METHODS: In this study, we examined the incidence and significance of bcl-2 expression in 25 cases of embryonal tumors of the central nervous system, including medulloblastoma, neuroblastoma, ependymoblastoma, and PNET (primitive neuroectodermal tumor), which has the possibility of neuronal differentiation. RESULTS: The results demonstrated that 13 tumors (52%) positive for bcl-2 belonged to early differentiated and neuronal types, and 7 negative tumors (28.0%) mostly belonged to undifferentiated type. CONCLUSIONS: These results have led us to speculate on the possibility that tumor cells begin expressing bcl-2 along with their neuronal differentiation from primitive cells.

Adolescent↗
PubMed2003

Intraperitoneal dissemination of Ad12-induced undifferentiated neuroectodermal hamster tumors: de novo methylation and transcription patterns of integrated viral and of cellular genes.

The intramuscular (i.m.) injection of human adenovirus type 12 (Ad12) into newborn Syrian hamsters caused widespread dissemination of up to 15 tumors over the entire peritoneal cavity in 70-90% of the animals within 30-50 days. Subcutaneous (s.c.) injections led to local tumor formation only. Independent of location, tumor histology revealed Homer-Wright rosette-like structures typical for primitive neuroectodermal tumors (PNET). All tumor cells showed markers indicative of neuroectodermal and mesenchymal derivations. Each Ad12-induced tumor cell carried multiple copies of integrated Ad12 genomes at one chromosomal site which was different for each tumor. For Ad12 tumor induction in hamsters, the patterns of Ad12 viral and cellular gene expression were important and were affected by changes in DNA methylation, both in the integrated Ad12 DNA and the cellular genome. By applying the bisulfite protocol, the de novo DNA methylation in the integrated Ad12 genomes was determined. These patterns were complex, characterized by regional initiation and by excluding genome segments in the E1A and E1B promoters. In all tumors, the Ad12 segments E1A, E1B, E2A, parts of E3 and E4 were similarly transcribed, as shown by the RT-PCR and DNA microarray methods. Changes in the transcription of a large number of cellular genes was assessed by using mouse gene microarrays encompassing about 1980 different mouse genes with 87-96% homology to hamster genes. Similarities and differences existed in the transcription of cellular genes of different functional classes among the different Ad12-induced tumors. These alterations in cellular gene transcription may be an important parameter in the oncogenic transformation by Ad12.

Adenoviruses, Human↗
PubMed1986

Evaluation of polyamine levels in cerebrospinal fluid of children with brain tumors.

Cerebrospinal fluid (CSF) polyamine levels were analyzed retrospectively in 21 pediatric patients with different types of intracranial malignant tumors to determine the benefit of following these markers during the clinical management of brain tumors. The tumors included 16 medulloblastomas and 1 each of germinoma, ependymoma, primitive neuroectodermal tumor, astrocytoma, and malignant teratoma. The clinical course of each patient was followed by neurologic examination, cranial computed tomography, CSF cell count, and cytology after cytocentrifugation. The correlation of CSF putrescine and spermidine levels with the clinical course of the brain tumors was analyzed. The following results were obtained: (1) A significant increase in CSF putrescine levels was observed in children with medulloblastoma when there was recurrent or metastatic disease in the sites close to the CSF pathway compared with the children whose disease status was stable after successful treatment (P less than 0.005). (2) The increase of CSF putrescine levels was the earliest predictor of recurrence or metastasis near the CSF pathway. (3) In tumors other than medulloblastoma, the levels of polyamines were not predictive of disease activity with the possible exception of germinoma. (4) Spermidine levels in the CSF were of limited clinical importance for patients with brain tumors. CSF putrescine levels may be the earliest and most sensitive quantitative marker of the progression of medulloblastoma, and their evaluation should be included in the diagnostic work-up and follow-up examination of children with medulloblastoma.

Astrocytoma↗
PubMed2005

[Mesenchymal tumors of the pancreas. Surprising, but not uncommon].

Mesenchymal tumors of the pancreas are rare. They are resected because a solid or cystic pancreatic tumor is suspected. Benign mesenchymal tumors comprise lymphangiomas, hemangiomas, schwannomas, solitary fibrous tumors, adenomatoid tumors, clear cell tumors, and hamartomas. Inflammatory pseudotumors are a special case. Malignant mesenchymal tumors include leiomyosarcomas, malignant peripheral nerve sheath tumors (MPNST), liposarcomas, malignant fibrous histiocytomas, Ewing's sarcomas, and primitive neuroectodermal tumors (PNET). It is important to differentiate these tumors from anaplastic carcinomas and retroperitoneal tumors that infiltrate pancreatic tissue.

Adenoma↗
PubMed2006

PMS2 mutations in childhood cancer.

Until recently, the PMS2 DNA mismatch repair gene has only rarely been implicated as a cancer susceptibility locus. New studies have shown, however, that earlier analyses of this gene have had technical limitations and also that the genetic behavior of mutant PMS2 alleles is unusual, in that, unlike MLH1 or MSH2 mutations, PMS2 mutations show low heterozygote penetrance. As a result, a dominantly inherited cancer predisposition has not been a feature reported in families with PMS2 mutations. Such families have instead been ascertained through childhood-onset cancers in homozygotes or through apparently sporadic colorectal cancer in heterozygotes. We present further information on the phenotype associated with homozygous PMS2 deficiency in 13 patients from six families of Pakistani origin living in the United Kingdom. This syndrome is characterized by café-au-lait skin pigmentation and a characteristic tumor spectrum, including leukemias, lymphomas, cerebral malignancies (such as supratentorial primitive neuroectodermal tumors, astrocytomas, and glioblastomas), and colorectal neoplasia with an onset in early adult life. We present evidence for a founder effect in five families, all of which carried the same R802-->X mutation (i.e., arginine-802 to stop) in PMS2. This cancer syndrome can be mistaken for neurofibromatosis type 1, with important management implications including the risk of the disorder occurring in siblings and the likelihood of tumor development in affected individuals.

Adenosine Triphosphatases↗
PubMed1980

Computed tomography of pineal, parapineal, and histologically related tumors.

Thirty-one of 32 pineal and related tumors were diagnosed by computed tomography (CT). There were 7 germinomas, 4 primary pineal tumors, 4 embryonal cell carcinomas, one teratoma, one epidermoid, one primitive neuroectodermal tumor, 8 astrocytomas and 6 lipomas. Histologic confirmation was obtained in 22. The CT characteristics allow differentiation of benign tumors such as teratomas and epidermoids from malignant germinal lesions such as germinoma and embryonal cell carcinoma. Abnormal calcification occurs most frequently in the primary pineal tumors, pineoblastoma and pineocytoma. The CT characteristics and consideration of the patient's age and sex often lead to histologic prediction and better treatment planning.

Adolescent↗
PubMedDate not supplied

Recurrent intrapulmonary malignant small cell tumor of the thoracopulmonary region with metastasis to the oral cavity: review of literature and case report.

Malignant small cell tumor of the thoracopulmonary region (MSCT) was first described in 1979 and has been referred to as the Askin tumor. This malignant neoplasm is a member of the peripheral primitive neuroectodermal tumor (PPNET) family and typically involves the periosteum, soft tissue, and extrapulmonary tissue of the thoracic wall. MSCT may also involve the lung parenchyma by local extension or may arise de novo in peripheral lung tissue. Local recurrence, abdominal involvement by tumor extravasation across the diaphragm, and skeletal metastatic disease are relatively common. However, metastasis to the head and neck region and in particular to the oral cavity is extremely rare. We present a recurrent intrapulmonary MSCT with metastasis to the oral cavity in an adolescent Hispanic boy, and review the literature regarding this member of the PPNET family. Differentiation from neuroblastoma may be made based on immunoreactivity for beta 2 microglobulin and HBA71 and lack of immunoreactivity for chromogranin in PPNET and MSCT. Ultrastructural features commonly seen in MSCT and PPNET are round to ovoid tumor cells with occasional cytoplasmic processes with relatively few pleomorphic dense core granules. These tumors lack the gangliocytic and Schwann cell differentiation that is characteristic of neuroblastoma. MSCT and PPNET have a common reciprocal cytogenetic translocation [t(11;22)q(24;q12)], which is shared with Ewing's sarcoma. Prognosis in MSCT is quite dismal, with a 2-year survival of 38% and a 6-year survival of only 14%.

Adolescent↗
PubMed1992

Expression of tumor necrosis factor-alpha cachectin in primary brain tumors of astrocytic lineage.

The tumor necrosis factor alpha (TNF-alpha) is a cytokine released within central nervous system from activated astrocytes and macrophages and involved in several pathologic processes including AIDS-myelopathy, multiple sclerosis and myelin dilatation in panencephalic type of Creutzfeldt-Jakob disease. We studied the expression of the TNF-alpha in brain tumors. Only tumors of astrocytic lineage like astrocytomas and glioblastomas, or tumors of mixed lineage as oligo-astrocytomas and multipotential primitive neuroectodermal tumors (PNET) expressed TNF-alpha-like immunoreactivity. We conclude that this lymphokine is expressed in neoplastic astrocytes. We postulate that TNF-alpha may participate in neoplastic transformation of astrocytes via cascade of interactions with receptor for TNF-alpha.

Astrocytoma↗
PubMed1999

Trilateral retinoblastoma: is the location of the intracranial tumor important?

BACKGROUND: Trilateral retinoblastoma refers to bilateral retinoblastoma associated with an intracranial primitive neuroectodermal tumor in the pineal or suprasellar region. The purpose of this study was to review patient and tumor characteristics and treatment outcome in patients with trilateral retinoblastoma and to determine whether there is a difference in presentation or outcome according to the location of the intracranial tumor. METHODS: A MEDLINE search of all English language articles pertaining to trilateral retinoblastoma published between 1977-1997 was performed. A total of 94 different cases were identified and analyzed. RESULTS: The gender was male in 39 patients (41.5%), female in 50 patients (53.2%), and unknown in 5 patients (5.3%). Family history for retinoblastoma was positive in 44 patients (46.8%), negative in 39 patients (41.5%), and unknown in 11 patients (11.7%). The median age at the time of diagnosis of retinoblastoma was 6 months. The median time interval to the development of an intracranial tumor from the time of diagnosis of retinoblastoma was 21 months. In 78 patients (83.0%) the intracranial tumor was in the pineal region and in 16 patients (17.0%) it was in the suprasellar region. The median time interval from the time of diagnosis of retinoblastoma to the development of a pineal region tumor was 24 months whereas the median time interval for the development of a suprasellar region tumor was 1 month. At 6 months after the diagnosis of intraocular tumors, 6 of 61 children with pineal region tumors and 10 of 14 patients with suprasellar region tumors had developed intracranial disease (P = 0.005). Unilateral intraocular retinoblastoma associated with intracranial tumor was more likely to occur in patients with suprasellar region tumors than pineal region tumors (P < 0.015). The median survival after the diagnosis of an intracranial tumor was 6 months regardless of the location of the intracranial tumor. For patients who received no treatment for the intracranial tumor the median survival was 1 month whereas it was 8 months for those who received treatment. Children who were asymptomatic at the time of diagnosis of the intracranial tumor had a better overall survival than those who were symptomatic (P = 0.002). CONCLUSIONS: The prognosis of children who develop trilateral retinoblastoma is dismal with current treatment strategies. Tumors of the suprasellar region present earlier than tumors of the pineal region after the diagnosis of intraocular tumors. Because patients who were asymptomatic at the time of diagnosis of intracranial disease had a better overall survival than those who were symptomatic, screening for intracranial tumors may be a valuable strategy in the management of patients with bilateral and/or hereditary retinoblastoma. [See editorial on pages 3-5, this issue.]

Adolescent↗
PubMed1985

Cross-reactivity between human hemopoietic cells and brain tumors as defined by monoclonal antibodies.

A battery of 24 monoclonal antibodies raised against human hemopoietic cells was tested in an indirect immunofluorescence technique on frozen sections of a variety of human neurogenic and non-neurogenic tumors. Twelve antibodies demonstrated some type of labeling of neurogenic tumors, frequently in patterns characteristic for benign and/or malignant gliomas and/or primitive neuroectodermal tumors (PNETs). Although also some cross-reactivity occurred in non-neurogenic tumors, the apparent operational specificity of some of our antibodies within the nervous system promises some aid in neuropathological tumor diagnosis; this was also demonstrated by combined use of some antibodies on smear preparations in which diagnosis by conventional stains was uncertain. This study confirms and expands previous data that sharing of antigenic determinants by hemopoietic cells and nervous system tumors is common. The significance of these cross-reactivities is at present a matter of speculation; cross-reacting autoantibodies might interfere with immune regulation in tumor patients, and an immune response might be initiated when glioma cells bearing Ia antigens present tumor-associated antigens to T cells.

Adolescent↗
PubMed1998

Prognostic value of proton MR spectroscopy of cerebral hemisphere tumors in children.

We studied 14 young people with newly diagnosed hemisphere tumors, aged from 3 to 20 years (average 10 years). All underwent surgery following MR imaging (MRI) and spectroscopy (MRS). The tumors studied were three glioblastomas, one each of ganglio-glioblastoma, primitive neuroectodermal tumor (PNET), rhabdoid teratoid tumor, pilocytic astrocytoma, ependymoma, anaplastic ependymoma, and gliomatosis cerebri, and four gangliogliomas. Four patients died; ten patients are alive (five with stable residual tumor, five with no evident tumor). Images and spectra were acquired on a 1.5-T imager. Proton MRS was performed before gadolinium injection in all but one case. Single-voxel techniques were utilized in all cases, using a spin-echo or STEAM sequence with a long echo time (135 or 270 ms). Peak areas of N-acetyl aspartate (NAA), choline (Cho), and creatine and phosphocreatine (Cr) were assessed. The NAA/Cho peak-area ratio was very low in the patients who died (mean +/- s.d. 0.20 +/- 0.14), and higher in the patients who are alive (0.74 +/- 0.47; P = 0.007 by two-tailed t-test). The Cr/Cho peak-area ratio also followed a similar trend for the two groups (mean +/- s.d. 0.17 +/- 0.07 and 0.49 +/- 0.30, respectively; P = 0.01 by two-tailed t-test).

Brain↗
PubMed2003

Vasopressin-SV40 T antigen expression in transgenic mice induces brain tumor and lymphoma.

In order to understand the importance of various cis-acting elements in regulating VP gene expression, transgenic mice regulated by VP constructs were produced containing 3.8 kb of the 5' flanking region and all the exons and introns in the mouse VP gene, which was fused at the end of exon 3 to an SV40 T antigen (Tag). In the transgenic mice by the pVPSV.IGR3.6 construct, all the six transgenic mice died at the age of 2-6 weeks. In the transgenic mice by pVPSV.IGR2.1, 21% of them had brain tumors at 5 weeks and 100% of the mice had brain tumors after 24 weeks. Histological analysis of the transgenic mice revealed primitive neuroectodermal tumors (PNET) in the brain and lymphoma in the spleen and lymph nodes. The phenotype differences between the two transgenic mice suggest that tissue-specific expression might be regulated by cis-acting elements in the 1.5-kb of the 3(') flanking region, which are not contained in pVPSV.IGR2.1. In conclusion, pVPSV.IGR2.1 mice will be a valuable mouse model system for investigating PNET tumorigenesis in the brain and lymphoma in the lymph nodes and spleen.

Animals↗
PubMed1999

Constitutional mutations of the hSNF5/INI1 gene predispose to a variety of cancers.

Biallelic, truncating mutations of the hSNF5/INI1 gene have recently been documented in malignant rhabdoid tumor (MRT), one of the most aggressive human cancers. This finding suggests that hSNF5/INI1 is a new tumor-suppressor gene for which germline mutations might predispose to cancer. We now report the presence of loss-of-function mutations of this gene in the constitutional DNA from affected members but not from healthy relatives in cancer-prone families. Furthermore, a constitutional mutation is documented in a patient with two successive primary cancers. In agreement with the two-hit model, the wild-type hSNF5/INI1 allele is deleted in the tumor DNA from mutation carriers. In all tested cases, DNA from parents demonstrated normal hSNF5/INI1 sequences, therefore indicating the de novo occurrence of the mutation, which was shown to involve the maternal allele in one case and the paternal allele in two other cases. These data indicate that constitutional mutation of the hSNF5/INI1 gene defines a new hereditary syndrome predisposing to renal or extrarenal MRT and to a variety of tumors of the CNS, including choroid plexus carcinoma, medulloblastoma, and central primitive neuroectodermal tumor. This condition, which we propose to term "rhabdoid predisposition syndrome," may account for previous observations of familial and multifocal cases of the aforementioned tumor types. It could also provide the molecular basis for cases of Li-Fraumeni syndrome without p53 germline mutations.

Chromosomal Proteins, Non-Histone↗
PubMed2001

CD99 immunoreactivity in ependymoma.

The expression of CD99 in normal ependymal cells and ependymoma has been reported. However, only limited numbers of tumors have been studied, and the pattern of CD99 expression has not been described. The authors' purpose was to investigate CD99 immunoreactivity in ependymoma and its use for differential diagnosis. Twenty-five ependymomas were immunostained with antibody directed at CD99. The result of immunostaining of ependymomas was compared with 63 nonependymal tumors that histologically resemble ependymal neoplasms. The nonependymal tumors included 19 astrocytic tumors, 6 oligodendroglial tumors, 8 choroid plexus neoplasms, 2 central neurocytomas, 5 medulloblastomas, 10 primitive neuroectodermal tumors (PNET), and 13 pituitary adenomas. All ependymomas showed strong expression of CD99 in membranous pattern with intracytoplasmic or intercellular dots (ICDs). The expression pattern of CD99 was not correlated with histologic type or grade of ependymomas. Among 63 nonependymal tumors, 11 (17.5%) showed incomplete membrane staining for CD99; diffuse in 4 PNETs and focal in 5 choroid plexus neoplasms (3 papillomas and 2 carcinomas) and one each of pituitary adenoma and oligodendroglioma. The ICD was not found in nonependymal tumors except a case of choroid plexus papilloma. However, membrane staining or ICD for CD99 was not distinctive in nonependymal tumors. In conclusion, the characteristic pattern of anti-CD99 antibody, i.e., diffuse strong membranous immunostaining with ICDs, is useful in distinguishing ependymomas from the central nervous system tumors that histologically mimic ependymoma.

12E7 Antigen↗
PubMed1997

Pediatric leptomeningeal metastases: outcome following combined therapy.

Fifteen children ranging in age from 4 to 18 years with leptomeningeal metastases were evaluated for extent of tumor and treated with radiotherapy or chemotherapy. Histologic diagnosis included: acute lymphoblastic leukemia (4); anaplastic astrocytoma (1); ependymoma (2); nonseminomatous germ cell tumor (1); non-Hodgkin's lymphoma (1); pineoblastoma (1); and primitive neuroectodermal tumor not otherwise specified (2). Pretreatment imaging studies demonstrated recurrent intracranial parenchymal disease in nine, spinal disease in four, and abnormal radioisotope ventriculography in six. Systemic disease was seen in four children (3 leukemia; 1 non-Hodgkin's lymphoma). All children were treated with systemic chemotherapy and intraventricular chemotherapy. Nine children received radiotherapy to bulky or symptomatic leptomeningeal disease. Median survival was 6 months (range, 4-18 months). Children with hematologic malignancies had superior outcomes compared to children with solid tumors. Three children with leukemic or lymphomatous meningitis are alive and disease free whereas all children with carcinomatous meningitis died. In conclusion, leptomeningeal metastases in children portends a limited survival, and therapies at this time remain palliative except in children with hematologic malignancies.

Adolescent↗
PubMed1993

Posterior dural arteriovenous malformation and medulloblastoma in an infant: case report.

A newborn is described who presented with heart failure from a posterior dural arteriovenous malformation and had a coexisting congenital medulloblastoma. There have been sporadic reports of arteriovenous malformation and brain neoplasms in older children and adults, and these have generally been glial tumors. This is the first known case of a combined congenital primitive neuroectodermal tumor and arteriovenous malformation in an infant.

Cerebellar Neoplasms↗
PubMed1990

Congenital brain tumors: a review of 45 cases.

Forty-five pathologically proved cases of neonatal brain tumors (diagnosed in neonates within 60 days after birth) were reviewed from the neuroradiology archives dating back to 1964. CT was performed in 24 cases, MR in five, sonography in six, and angiography in seven. Two-thirds of the lesions were supratentorial. The most common histology was a tumor composed of primitive or poorly differentiated tissues: 12 teratomas and 12 primitive neuroectodermal tumors, four of which were typical medulloblastomas. In addition, there were nine astrocytomas (grades I-III); four cases of glioblastoma multiforme (astrocytoma grade IV); three choroid plexus papillomas; and single cases each of ependymoma, medulloepithelioma, germinoma, angioblastic meningioma, and ganglioglioma. The dominant CT appearance, regardless of histology, was a large heterogeneous lesion with associated hydrocephalus. Coarse calcification was a constant feature in the teratomas. Prognosis was poor overall, with the longest survival seen in choroid plexus papilloma and astrocytoma. Imaging studies are most valuable in identifying and distinguishing potentially curable lesions such as choroid plexus papillomas (variably sized intraventricular lesions with homogeneous enhancement) from rapidly fatal tumors such as teratomas (large heterogeneous lesions with coarse calcifications and associated hydrocephalus).

Astrocytoma↗