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PubMed2003

Medulloblastoma.

Medulloblastomas, primitive neuroectodermal tumors of the cerebellum, comprise 20% of all pediatric brain tumors and are the most common solid neoplasm in children. Primitive neuroectodermal tumors are believed to arise from cerebellar granule cell precursors. Occasionally, medulloblastoma occurs in children with genetically linked disorders, such as Turcot's syndrome or Gorlin's syndrome, which is also called basal cell nevus syndrome. Several genes have been implicated in the development of medulloblastoma in children, including Patched-1 and Smoothened. The protein products of these genes function within the sonic hedgehog molecular signaling pathways, which are important in neural development and disease. Through analysis of several well-designed multi-institutional trials, much has been learned about the clinical factors that influence outcome in children with medulloblastomas. Age younger than 3 years, bulky residual disease postoperatively, and metastasis constitute adverse prognostic features and indicate patients who are considered "high risk" for recurrence with standard therapy using 3600 cGy craniospinal radiation in conjunction with a posterior fossa dose of 5400 cGy. Patients lacking these features are considered "standard risk." Chemotherapeutic trials have been developed to assess the safety and efficacy of various multi-agent therapies to improve the poor results of high-risk patients and to allow reduction in the dose of radiation needed to cure standard-risk patients, which may allow a decrease in late cognitive sequelae. Currently, it is policy to evaluate all children with posterior fossa tumors characteristic of medulloblastoma with preoperative, staging neuroimaging studies of the craniospinal axis. Surgical resection is undertaken with the goal of gross total resection. Postoperative neuroimaging studies are compared with preoperative studies to determine the amount of residual disease. Cerebrospinal fluid is obtained from a lumbar puncture done at the conclusion of the surgical resection or 2 weeks after surgery in order to determine microscopic leptomeningeal spread. Children with tumor histopathology consistent with medulloblastoma are enrolled, when possible, in open clinical trials. Chemotherapy and radiation are given as per protocol. The goal of current treatment approaches is to tailor therapy based on clinical risk factors, with intensification of treatment for children with high-risk disease and reduction of radiation therapy for those with standard-risk disease. Evaluation of biologic predictors of outcome, which may further refine treatment stratification, is in progress.

Journal Article↗
PubMed2001

A case of undifferentiated glioma in a 70-year-old woman.

The present case involved a 70-year-old woman who was diagnosed with a right cerebral hemorrhage. Excisional surgery of the hematoma was performed. Grossly, a whitish, solid tumor (1 x 1 x 0.8 cm in size) was recognized in the hematoma. Histologically, the tumor was composed of large, polygonal cells and small undifferentiated cells in a jumbled architectural arrangement with a cartilage component. The large, polygonal cell component was conspicuous and somewhat rhabdoid in appearance and appeared to be an astrocytic tumor showing glial differentiation. The small, undifferentiated cell component resembled tumor cells of a primitive neuroectodermal tumor (PNET). Clinical follow-up of the patient for 2 months after the first operation revealed recurrence with rapid growth. A second operation was performed, but the patient died 8 months after the first operation (2 months after the second). Immunohistochemically, the tumor cells suggesting glial differentiation were positive for glial fibrillary acidic protein (GFAP), S-100, neuron-specific enolase (NSE), and vimentin. PNET-like components in the primary tumor were positive for NSE, GFAP, and S-100, and weakly positive for vimentin and synaptophysin. Each tumor cell was negative for epithelial membrane antigen (EMA), keratin, desmin, actin, myoglobin, neurofilament (NF), and MIC2 protein. The recurrent tumor revealed predominantly PNET-like components; however, only a few tumor cells were positive for GFAP. This appearance suggested that this brain tumor might originate from a common multipotential stem cell. Considering its histopathological and immunohistochemical characteristics, the primary tumor was finally regarded as an undifferentiated glioma with dedifferentiation of the glial component in the recurrent tumor.

Aged↗
PubMed1993

The Ewing's sarcoma EWS/FLI-1 fusion gene encodes a more potent transcriptional activator and is a more powerful transforming gene than FLI-1.

EWS/FLI-1 is a chimeric protein formed by a tumor-specific 11;22 translocation found in both Ewing's sarcoma and primitive neuroectodermal tumor of childhood. EWS/FLI-1 has been shown to be a potent transforming gene, suggesting that it plays an important role in the genesis of these human tumors. We now demonstrate that EWS/FLI-1 has the characteristics of an aberrant transcription factor. Subcellular fractionation experiments localized the EWS/FLI-1 protein to the nucleus of primitive neuroectodermal tumor cells. EWS/FLI-1 specifically bound in vitro an ets-2 consensus sequence similarly to normal FLI-1. When coupled to a GAL4 DNA-binding domain, the amino-terminal EWS/FLI-1 region was a much more potent transcriptional activator than the corresponding amino-terminal domain of FLI-1. Finally, EWS/FLI-1 efficiently transformed NIH 3T3 cells, but FLI-1 did not. These data suggest that EWS/FLI-1, functioning as a transcription factor, leads to a phenotype dramatically different from that of cells expressing FLI-1. EWS/FLI-1 could disrupt normal growth and differentiation either by more efficiently activating FLI-1 target genes or by inappropriately modulating genes normally not responsive to FLI-1.

3T3 Cells↗
PubMed1993

Ewing sarcoma 11;22 translocation produces a chimeric transcription factor that requires the DNA-binding domain encoded by FLI1 for transformation.

The 11;22 chromosomal translocation specifically linked to Ewing sarcoma and primitive neuroectodermal tumor results in a chimeric molecule fusing the amino-terminal-encoding portion of the EWS gene to the carboxyl-terminal DNA-binding domain encoded by the FLI1 gene. We have isolated a fourth EWS-FLI1 fusion cDNA that is structurally distinct from the three forms previously described. To determine the transforming activity of this gene, alternative forms of the EWS-FLI1 fusion were transduced into NIH 3T3 cells. Cells expressing either type 1 or type 4 fusion constructs formed foci in culture and colonies in soft agar, indicating that EWS-FLI1 is a transforming gene. EWS-FLI1 deletion mutants were created to map functionally the critical regions within the chimera. Deletion of either the EWS domain or the FLI1 corresponding to the DNA-binding domain totally abrogated the ability for EWS-FLI1 to transform 3T3 cells. These data indicate that the oncogenic effect of the 11;22 translocation is caused by the formation of a chimeric transcription factor. Formation of chimeric transcription factors has now been demonstrated to promote tumors of both neuroectodermal and hematopoietic origin, suggesting that this may be a common mechanism in human carcinogenesis.

Amino Acid Sequence↗
PubMed2002

Phase II study of irinotecan (CPT-11) in children with high-risk malignant brain tumors: the Duke experience.

A phase II study of irinotecan (CPT-11) was conducted at Duke University Medical Center, Durham, NC, to evaluate the activity of this agent in children with high-risk malignant brain tumors. A total of 22 children were enrolled in this study, including 13 with histologically verified recurrent malignant brain tumors (glioblastoma multiforme [GBM] 4, anaplastic astrocytoma 1, ependymoma 5, and medulloblastoma/primitive neuroectodermal tumor 3), 5 with recurrent diffuse pontine glioma, and 4 with newly diagnosed GBM. All patients with recurrent tumor had prior chemotherapy and/or irradiation. Each course of CPT-11 consisted of 125 mg/m ( 2 ) per week given i.v. for 4 weeks followed by a 2-week rest period. Patients with recurrent tumors received therapy until disease progression or unacceptable toxicity. Patients with newly diagnosed tumors initially received 3 cycles of treatment to assess tumor response and then were allowed radiotherapy at physician's choice; patients who demonstrated a response to CPT-11 prior to radiotherapy were allowed to continue the drug after radiation until disease progression or unacceptable toxicity. A 25% to 50% dose reduction was made for grade III-IV toxicity. Responses were assessed after every course by gadolinium-enhanced MRI of the brain and spine. Twenty-two patients received a median of 2 courses of CPT-11 (range, 1-16). Responses were seen in 4 of 9 patients with GBM or anaplastic astrocytoma (44%; 95% confidence interval, 11%-82%) (complete response in 2 patients with recurrent GBM lasting 9 months and 48+ months; partial response in one patient with a newly diagnosed midbrain GBM lasting 18 months prior to radiotherapy; and partial response lasting 11 months in 1 patient with recurrent anaplastic astrocytoma), 1 of 5 patients with recurrent ependymoma (partial response initially followed by stable disease lasting 11 months), and none of 5 patients with recurrent diffuse pontine glioma. Two of 3 patients with medulloblastoma/primitive neuroectodermal tumor had stable disease for 9 and 13 months. Toxicity was mainly myelosuppression, with 12 of 22 patients (50%) suffering grade II-IV neutropenia. Seven patients required dose reduction secondary to neutropenia. CPT-11, given in this schedule, appears to be active in children with malignant glioma, medulloblastoma, and ependymoma with acceptable toxicity. Ongoing studies will demonstrate if activity of CPT-11 can be enhanced when combined with alkylating agents, including carmustine and temozolomide.

Adolescent↗
PubMed2006

Immunohistochemistry of primary malignant neuroepithelial tumors of the kidney: a potential source of confusion? A study of 30 cases from the National Wilms Tumor Study Pathology Center.

Ewing sarcoma/peripheral primitive neuroectodermal tumor (pPNET) is a rare primary tumor of the kidney with morphologic features similar to those of other primitive tumors. Previous studies have shown that these tumors frequently stain positively with immunostains against CD99 and FLI-1 and negatively with stains against WT-1, suggesting that these markers may be used for the distinction between Wilms tumor and pPNET. We present 30 cases of primary malignant neuroepithelial tumor with immunohistochemical profiles and reverse transcriptase polymerase chain reaction (RT-PCR) analysis and show that immunophenotypic overlap exists between Wilms tumor and pPNET. A subset of 30 neuroepithelial tumors from the National Wilms Tumor Study originally categorized as putative pPNETs of the kidney was stained with FLI-1, WT-1, and thyroid transcription factor-1. Bicolor fluorescence in situ hybridization studies were performed on 19 of the cases. Other data on these tumors were available from a previous study (Am J Surg Pathol 2001;25:133). Of 7 primary tumors that had the EWS/FLI-1 fusion transcript by RT-PCR, 6 exhibited strong immunopositivity for FLI-1. Nine that were negative by RT-PCR stained positively with the FLI-1 stain. Five fusion-negative cases stained with both FLI-1 and WT-1. Three fusion-negative cases were negative for FLI-1 but positive for WT-1. Five fusion-negative cases were negative for both FLI-1 and WT-1. Of the 30 cases, 29 were positive for CD99. Seven cases that were negative for the EWS-FLI-1 fusion by RT-PCR were positive by fluorescence in situ hybridization. All cases were negative for thyroid transcription factor-1. Reliance upon immunohistochemistry as the sole means of ancillary diagnosis in renal pPNET can lead to confusing results. We recommend molecular fusion studies for clarification of primitive renal tumors with unexpected immunophenotypic results.

Adult↗
PubMed2004

Establishing a germ cell origin for metastatic tumors using OCT4 immunohistochemistry.

BACKGROUND: There are diverse morphologic manifestations of metastatic tumors. The determination of tumor origin is critical for patient management, and it is especially important when the differential diagnosis includes metastatic germ cell tumor, which is a highly treatable condition. OCT4 is a nuclear transcription factor that is expressed in pluripotent embryonic germ cells. In this study, the author sought to determine the usefulness of OCT4 immunohistochemistry in the diagnosis of metastatic germ cell tumors. METHODS: Sixty-two retroperitoneal lymph node dissection specimens from patients with histories of testicular germ cell tumors were stained using the antibodies against OCT4. In addition, 84 metastatic, nongerm cell lesions from men with known primary tumors were studied in parallel for OCT4 immunohistochemistry. RESULTS: All embryonal carcinoma components (n = 29 specimens) and seminoma components (n = 18 specimens) from retroperitoneal lymph node dissection specimens showed strong, intense, diffuse nuclear staining for OCT4. Yolk sac tumors (n = 12 tumors), choriocarcinomas (n = 4 tumors), mature teratomas (n = 16 tumors), and primitive neuroectodermal tumors (n = 5 tumors) were negative for OCT4 staining. Metastatic, nonsmall cell carcinomas from the lung (n = 14 tumors), colon (n = 12 tumors), stomach (n = 5 tumors), pancreas (n = 7 tumors), prostate (n = 12 tumors), kidney (n = 3 tumors), and urinary bladder (n = 15 tumors) all were found to be negative immunohistochemically for OCT4, as were metastatic small cell carcinomas (n = 4 tumors) and metastatic melanomas (n = 7 tumors). In addition, malignant lymphomas (n = 5 tumors) also were negative for OCT4. CONCLUSIONS: Immunohistochemical detection of OCT4 is highly sensitive and specific for the diagnosis of seminoma and embryonal carcinoma metastatic from the testis. Establishing germ cell origin for metastatic tumors has important implications for assessing patient prognosis and treatment options.

Biomarkers, Tumor↗
PubMed1985

Vimentin and glial fibrillary acidic protein in human brain tumors.

Using the peroxidase-antiperoxidase (PAP) technique, we examined 35 primary brain tumors for expression of vimentin and GFAP. Both low-grade and high-grade astrocytomas contained vimentin-positive and GFAP-positive cells. Ependymomas also stained for both markers. In gliosarcomas, the glioblastomatous portions stained like astrocytomas, while only vimentin stain was seen in the fibrosarcomatous portions. Medulloblastomas and oligodendrogliomas were negative for both vimentin and GFAP, while meningiomas contained scattered areas of vimentin-positive cells. These results suggest that the expression of vimentin and GFAP is mostly confined to glial-derived tumors, and that vimentin can potentially be a useful marker for distinguishing undifferentiated GFAP-negative glial tumors and mesenchymal tumors from primitive neuroectodermal tumors.

Astrocytoma↗
PubMed1992

Stress-response (heat-shock) protein 72 expression in tumors of the central nervous system: an immunohistochemical investigation.

This report deals with the expression of stress-response (heat-shock) protein 72 (srp 72) in a series of 95 primary human brain tumors and 21 carcinoma metastases to the central nervous system (CNS). Immunohistochemical procedures were employed; cells of the human cervical cancer line HeLa S3 were used as positive controls. The protein was detected in 14/22 meningiomas and in 6/13 glioblastomas. Tumor cells expressing srp 72 were also found in 4/17 astrocytomas, 2/9 pituitary tumors, 2/14 primitive neuroectodermal tumors and 1/10 medulloblastomas. Whereas the majority (8/10) of the breast carcinoma metastases had tumor cells that expressed srp 72, only 2/11 lung tumor metastases were positively stained. These results document srp 72 expression by a variety of primary and metastatic tumors of the CNS.

Antibodies, Monoclonal↗
PubMed1991

Application of an anti-myosin antibody for scintigraphic differential-diagnosis of infantile tumors.

Immunoscans with the Fab-fragment of the monoclonal anti-myosin antibody R11D10 labeled with 111In showed significant uptake in rhabdomyosarcoma, rhabdoid tumor, and primitive neuroectodermal tumor. The diagnosis of an antimyosin positive or negative tumor with the aid of the immunoscan was superior to clinical findings in combination with ultrasound and CT, and to histological diagnosis in tumor biopsies. In addition chemotherapeutically induced renal impairment can be diagnosed by diminished renal uptake of antimyosin.

Adolescent↗
PubMed2004

Causation of nervous system tumors in children: insights from traditional and genetically engineered animal models.

Pediatric neurogenic tumors include primitive neuroectodermal tumors (PNETs), especially medulloblastoma; ependymomas and choroid plexus papillomas; astrocytomas; retinoblastoma; and sympathetic neuroblastoma. Meningiomas and nerve sheath tumors, although uncommon in childhood, are also significant because they can result from exposures of children to ionizing radiation. Specific chromosomal loci and specific genes are related to each of these tumor types. Virtually all these genes appear to act as tumor suppressor genes, which are inactivated in tumor cells by mutations or by chromosomal loss. In genetically engineered mice, some genes that are clearly associated with specific human tumors (e.g., RB1 in retinoblastoma and NF2 in meningiomas and schwannomas) have no such effect. Other genetic constructs in mice involving the genes p53, ptc1, and Nf1 have produced tumors remarkably similar to some of the human pediatric neoplasms. Some of these tumors become clinically apparent after only a few weeks, while the mice are still juveniles, especially when two or more tumor suppressor genes are inactivated in the same genetic construct. Conversely, at least one genetic pathway in rodents involving point mutation in the coding region of a transforming gene (neu in malignant schwannomas) does not appear to operate in any human tumors. The nervous system is markedly susceptible to experimental carcinogenesis during early life in rodents, dogs, primates, and other nonhuman species, and there is no obvious reason why this generalization should not also apply to humans. However, except for therapeutic ionizing radiation, no physical, chemical, or biological cause of human pediatric nervous system tumors is known. The failure of experimental transplacental carcinogenesis to mirror human pediatric experience more closely may reflect the need for multiple mutational events in target cells, and for experimental carcinogens that are capable of causing the full spectrum of mutations that occur in cancer-related genes in pediatric neurogenic tumors.

Adult↗
PubMedDate not supplied

AIM-2: a novel tumor antigen is expressed and presented by human glioma cells.

Antigen isolated from Immunoselected Melanoma-2 (AIM-2) was recently identified using melanoma-reactive CD8 T cells. AIM-2 antigen is expressed in a wide variety of tumor types, including neuroectodermal tumors, as well as breast, ovarian and colon carcinomas. In this study, we analyzed AIM-2 expression in glioblastoma multiforme (GBM) in primary cultured cells and established GBM cell lines. We found that the primary GBM cell lines expressed 88.4% and 93.0% of non-spliced and spliced AIM-2, respectively. Five out of seven of the established GBM cell lines expressed both non-spliced and spliced AIM-2. Furthermore, the C9 CTL clone, which is specific for AIM-2 peptide (RSDSGQQARY), efficiently recognized GBM tumor cells in an antigen-specific and HLA-A1 restricted manner. IFN-gamma treatment of the GBM tumor cells dramatically increased HLA-A1 expression levels and, consequently, increased CTL recognition of the treated tumor cells. More importantly, seven out of 12 HLA-A1 and AIM-2 positive patients from our dendritic cell clinical trial generated AIM-2 specific CTL activity in their PBMC after vaccinations. These data indicate that AIM-2 could be used as a tumor antigen target for monitoring vaccine trials or to develop antigen specific active immunotherapy for glioma patients.

Antigens, Neoplasm↗
PubMed2002

The expression of WT1 in the differentiation of rhabdomyosarcoma from other pediatric small round blue cell tumors.

The WT1 gene encodes a transcription factor implicated in normal and neoplastic development. The purpose of this study was to evaluate the diagnostic utility of a commercial WT1 antibody on a variety of pediatric small round blue cell tumors (SRBCT). A mouse monoclonal antibody (clone: 6F-H2, DAKO) raised against the N-terminal amino acids 1-181 of the human WT1 protein was tested. Microscopic sections from 66 specimens were stained using an antigen retrieval protocol with trypsin. The tumors included peripheral neuroectodermal tumors (PNET/Ewing's), neuroblastomas, desmoplastic small round cell tumors (DSRCT), lymphomas, Wilms' tumors, and rhabdomyosarcomas (RMS). One RMS case was investigated by Western blot analysis and RT-PCR to confirm the antibody specificity. A strong cytoplasmic staining was demonstrated in all RMS (11/11). The Western blot analysis confirmed the WT1 protein in the tissue, and the RT-PCR confirmed the presence of WT1 mRNA in the peripheral blood and tissue of one RMS patient. The Wilms' tumors had a variable nuclear and/or cytoplasmic positivity in most (17/24) cases. All PNET/Ewing's were negative. The nuclei of two lymphoblastic lymphomas stained strongly. A weak nuclear or cytoplasmic staining was reported in a few DSRCT (3/5), lymphomas (2/10), and neuroblastomas (2/8). This is a useful antibody in the differentiation of RMS from other SRBCTs. A strong cytoplasmic staining favors an RMS, and a strong nuclear staining is suggestive of a Wilms' tumor. A role for WT1 in the pathogenesis of rhabdomyosarcomas is raised. The limited sampling precludes any conclusions regarding the value of tissue or peripheral blood analysis for WT1 mRNA in patients with rhabdomyosarcoma.

Animals↗
PubMedDate not supplied

Magnetic resonance analysis of suprasellar tumors of childhood.

A retrospective analysis of the magnetic resonance (MR) images in 53 pediatric patients with pathologically proven suprasellar tumors was performed, in an attempt to identify the characteristic MR features of these tumors and assess the capability of MR to predict a histologic differentiation. The tumors analyzed included 29 astrocytomas, 11 craniopharyngiomas, 4 germinomas, 3 pituitary adenomas with suprasellar extension, 2 teratomas, 1 spindle cell tumor, 1 primitive neuroectodermal tumor, 1 arachnoid cyst and 1 chordoma of the clivus with suprasellar extension. Thirty patients received intravenous Gd-DTPA as part of their MR exam. Certain MR features, while not pathognomonic, are quite helpful in the differentiation of craniopharyngiomas from chiasmatic/hypothalamic astrocytomas. Presence of a high signal intensity component on T1-weighted images, cyst formation with macrocystic predominance, irregular, heterogeneous solid portion, and smooth ring cyst wall enhancement represent the key characteristics of craniopharyngiomas. Solid predominance with microcysts, long T1 and T2 relaxation times, intense enhancement after contrast administration and extension along the posterior optic pathways are the typical MR findings of chiasmatic/hypothalamic astrocytomas. Presence of diabetes insipidus in correlation with the MR findings of a well-marginated, round or lobular tumor with prolonged T1 and T2 relaxation times, which enhances strongly after Gd-DTPA administration may be the clue in the diagnosis of suprasellar germinomas. Teratomas can be separated from other pediatric suprasellar neoplasms on the basis of internal heterogeneity with presence of fat, calcium and various soft tissue densities. Tumors invading the suprasellar cistern by extension are easily differentiated by identification of the primary site of origin. The above features, while not pathognomonic, are quite helpful in making a specific diagnosis.

Adenoma↗
PubMed1988

Enhancement of growth of primary metastatic fresh human tumors of the nervous system by epidermal growth factor in serum-free short term culture.

Using the adhesive tumor cell culture system, we studied the effect of epidermal growth factor (EGF) on 16 primary and 7 metastatic fresh human tumors of the nervous system cultured in serum-free and serum-supplemented media at low cell density. In serum-free conditions, EGF significantly enhanced the growth of glial tumor cells. This positive effect was less pronounced for metastases to the brain. No significant enhancement was observed for the other tumor types (primitive neuroectodermal tumors and various tumors of neuroepithelial/mesenchymal origin). The addition of serum obscured this effect of EGF, even at the lowest cell densities. In 7 tumors, the simultaneous addition of platelet-derived growth factor did not enhance the EGF response. Subtypes of brain tumors respond to EGF in vitro under serum-free conditions.

Brain Neoplasms↗
PubMed2005

Current treatment for Ewing's sarcoma.

Ewing's sarcoma is the second most common primary bone tumor seen in children and adolescents, and was described by James Ewing in 1921 as a diffuse endothelioma of bone. It is one of the differential diagnoses of pediatric small round blue cell tumors. This is not a single condition, but a group of morphologically and clinically closely related disorders with similar molecular biology -- expression of tumor-specific chimeric oncoproteins through balanced chromosomal translocations involving the EWS gene -- often referred to as the Ewing family of tumors. This includes Ewing's sarcoma of bone, extra-osseous Ewing's sarcoma, Askin tumor and peripheral neuroectodermal tumor. These are aggressive neoplasms with almost 25% of patients having clinically evident metastases at presentation. Ewing's sarcoma has therefore been considered as a systemic disease necessitating local as well as systemic treatment. An aggressive multidisciplinary approach has resulted in significant improvement in prognosis for patients with these tumors. Despite aggressive treatment, 20-40% of patients with localized disease and almost 80% of patients with metastatic disease at presentation succumb to the illness. Advances in understanding the molecular biology of these tumors will hopefully result in the development of novel treatment approaches. The aim of this article is to review the existing treatment methods and to highlight the more recent approaches to the treatment of this condition.

Adolescent↗
PubMed2000

[Retinoblastoma].

Hereditary retinoblastoma is different from non-hereditary retinoblastoma in the following ways. It has a germinal mutation of Rb gene located in 13q14. Its inheritance is autosomally dominant with 80% penetrance. Retinal tumors are present usually multiply (average number is three) in both eyes. The patients are found at a younger age than those non-hereditary retinoblastoma. About 5% of them are accompanied by a pineal tumor or paracellular neuroectodermal tumor, in such cases they are called trilateral retinoblastoma. Secondary malignancies such as osteosarcoma and rhabdomyosarcoma are more frequent, and they are the main causes of death of patients with hereditary retinoblastoma. Considering these facts, education of patients and their family members and development of new modalities of treatment for preserving eyeballs without inducing secondary malignancies are important.

Age Factors↗
PubMedDate not supplied

Establishment and characterization of malignant rhabdoid tumor of the kidney.

Malignant rhabdoid tumor of the kidney (MRTK) is a highly aggressive tumor which occurs in childhood and which is histologically characterized by the existence of eosinophilic intracytoplasmic inclusions. We established and characterized a cell line from this tumor with histological, immunohistochemical and cytogenetical analysis. Histologically, the tumor cells demonstrate typical eosinophilic inclusions, while immunohistochemically the cells demonstrate common mesenchymal and epithelial differentiation. Although the conventional karyotyping of this tumor lacked the abnormalities of 22q chromosome, Southern blot analysis and microsatellite analysis verified abnormalities of the BCR gene and of the hSNF5/INI1 gene. Despite the variety of locations, these common genetic abnormalities appear to contribute to distinguish rhabdoid tumor from such other small round cell tumors as primitive neuroectodermal tumor, rhabdomyosarcoma, poorly differentiated synovial sarcoma and desmoplastic small round cell tumor.

Animals↗