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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 14-day-old white male, born with a large primitive neuroectodermal tumor of the left cerebral hemisphere, was found to have a solitary rhabdoid tumor in the liver incidentally at autopsy. Cells resembling the liver rhabdoid cells were also found by histology, immunohistochemistry, and electron microscopy in the brain tumor. The concurrence of rhabdoid cells in the tumors of the brain and liver suggests a common histogenesis and further supports the previous suggestion that the rhabdoid tumor is of neuroectodermal origin. The rhabdoid tumor in the liver in this case is likely to be a metastatic tumor from the brain rather than a second primary tumor.
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PMID:Primitive neuroectodermal tumor of the brain associated with malignant rhabdoid tumor of the liver: a histologic, immunohistochemical, and electron microscopic study. 254 37

Malignant rhabdoid tumor (MRT), originally described as a sarcomatous variant of Wilms' tumor, is now recognized as a distinct, highly malignant entity. The authors describe, for the first time, a primary tumor of the orbit with histologic, immunohistochemical, and ultrastructural features of MRT. Their findings suggest both epithelial and mesenchymal differentiation of this unique tumor.
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PMID:Malignant rhabdoid tumor of the orbit. 269 50

This report documents, in seven infants younger than 2 years of age, a previously unrecognized association of a renal embryonal neoplasm (malignant rhabdoid tumor in six patients and a Wilms' tumor in one) with an embryonal primary tumor originating in the central nervous system. The neuroepithelial tumors included three cerebellar medulloblastomas, one pineoblastoma, one primitive neuroepithelial tumor (probably cerebral neuroblastoma), one malignant subependymal giant cell astrocytoma, and one cerebellar medulloepithelioma with divergent glial and neuronal differentiation. There is no evidence that this association is based on the selective neoplastic transformation of embryonal cells of similar histogenetic or cytogenetic origin. The relationship between these dissimilar, embryologically unrelated tumors remains enigmatic.
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PMID:The association of embryonal tumors originating in the kidney and in the brain. A report of seven cases. 609 60

The clinical, pathologic, and immunohistochemical features of a widely disseminated tumor with rhabdoid phenotype are described in nine infants < or = 3 months of age. Five neonates had tumor evident at birth, two of which had placental metastases. The average survival following diagnosis was < 6 weeks. None of the infants had an apparent primary tumor in either the kidney or brain. In four cases, the dominant mass involved the head and neck region, and in two cases, the primary mass was paraspinal. The histologic features were those of a high-grade, round cell neoplasm with abundant cytoplasm and containing cells with cytoplasmic filamentous inclusions. Immunohistochemical studies revealed polyphenotypic antigen expression. Genetic information was available from eight of nine cases. Karyotype analysis revealed abnormalities of chromosome band 22q11-12 in three of six tumors. Fluorescence in situ hybridization studies or molecular studies demonstrated 22q11.2 deletions in all five cases with available frozen tissue, two of which had translocations involving 22q by karyotype analysis. The similar clinical and pathologic findings in these rapidly fatal tumors in infants and the demonstration of abnormalities of chromosome 22q11 in a majority of the cases supports their histogenetic and nosologic relationship to the family of malignant rhabdoid tumors that typically occur in young children in several anatomic sites, including kidney, soft tissues, liver, and brain. Like neuroblastoma and rhabdomyosarcoma, malignant rhabdoid tumor can appear as disseminated disease at birth or shortly thereafter.
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PMID:Congenital disseminated malignant rhabdoid tumor: a distinct clinicopathologic entity demonstrating abnormalities of chromosome 22q11. 1007 13

Malignant rhabdoid tumor is a highly aggressive tumor of childhood that may present as a soft-tissue primary tumor. We report a soft-tissue neoplasm that was polyphenotypic by immunohistochemical expression of epithelial, mesenchymal, and neural markers and did not meet the criteria for any of the usual pediatric small round-cell tumors. The findings raised the diagnosis of rhabdoid tumor, leading to testing for WT1 mRNA and protein expression, which were positive, as has been reported for renal rhabdoid tumor. This tumor had the typical clinical behavior of rhabdoid tumor with therapy resistance and early tumor-related death. Multicolor spectral karyotyping of this neoplasm showed a balanced translocation between chromosomes 1 and 22 with breakpoints at 1p36 and 22q11-12. The latter region is commonly involved in rhabdoid tumor. This change was also identified by fluorescence in situ hybridization. This case suggests that studies of chromosome 22 may be required to distinguish rhabdoid tumor from other soft-tissue tumors.
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PMID:Analysis of chromosome 22q as an aid to the diagnosis of rhabdoid tumor: a case report. 1043 70

The staging of non-small lung cancer has to be performed in an interdisciplinary approach considering all clinical, radiological and histologic results. The staging using imaging procedures is done according to the TNM classification with T describing the extent of the primary tumor, N the presence and location of metastatic lymph nodes and M the presence or absence of distant metastases. It is important to remember that the individual stages of the TNM classification have undergone numerous revisions and thus need to be considered in their most recent version [Chest 111 (1997) 1718; Chest 111 (1997) 1710]. Noninvasive information about the stage of the disease is important for the planning and optimization of therapy. This may be done with imaging procedures such as, CT, MRT or PET.
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PMID:Imaging of lung cancer with CT, MRT and PET. 1174 Sep 88

In patients with cervical cancer of an unknown primary (CUP), no established concept exists for the necessary diagnostic procedures. In order to find the primary tumor, extensive diagnostic steps are generally recommended; however, they are often not performed consistently. In the current study, we consistently used a diagnostic algorithm and analyzed its consequences on patients' prognoses. We retrospectively studied 57 patients who were found to have a cervical metastasis of the upper- or midneck and an unknown primary tumor after routine examination of the head and neck region. Patients were analyzed for the value of applied diagnostic measures, tumor classification, survival rates and frequencies of subsequent lymph node or distant metastases after the initial treatment. Our results showed that a diagnostic algorithm (lymph node biopsy, rigid panendoscopy with systematic biopsies of suspect regions as well as blind biopsies of endoscopically inconspicuous regions, including the tongue base and nasopharynx and bilateral tonsillectomy) led to the detection of 14 occult oropharyngeal and 5 nasopharyngeal primary tumors in the patients. These tumors were primarily diagnosed as CUP. Oropharyngeal tumors either grew submucosally or were so small that only microscopic evaluation of the entire tonsil uncovered the tumor. Imaging procedures (X-ray, ultrasound, CT, MRT and FDG-PET) as well as gynecological, urological and gastroenterological consultations did not reveal the primary tumors in any of the cases. The 3-year survival rate for the patients with occult oropharyngeal primary tumors was 100% after treatment, while the patients in which our diagnostic schedule did not reveal a primary tumor showed a survival rate of 58%. The prognosis of all of the patients with cervical carcinoma metastasis was dependent on the initial nodal stage. Metachronous metastasis after completion of the initial treatment was prognostically infaust, while secondary detection of the primary tumor was worthwhile during follow-up as long as further treatment options were offered. The prognosis of patients with cervical carcinoma metastases of the upper- and midneck is much more favorable than that of patients with a CUP syndrome of other localizations. Identification of an occult pharyngeal tumor is prognostically relevant, since it opens up the possibility of specific locoregional treatment. In patients with cervical CUP, blind but systematic pharyngeal biopsies, including bilateral tonsillectomy, should be performed.
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PMID:Diagnostic strategies in cervical carcinoma of an unknown primary (CUP). 1211 82

Our aim is to evaluate the clinical features and outcomes of children with primary central nervous system (CNS) tumors who develop extraneural metastasis (ENM). We retrospectively evaluated children diagnosed with primary CNS tumors treated at our institution between 1972 and 2004. Of 1,011 patients these tumors, 10 (0.98%) developed ENM. The histopathologic diagnosis was medulloblastoma in six patients, germ cell tumors in two patients, and ependymoma and atypical teratoid rhabdoid tumor (ATRT) in one patient each. In six patients, the primary tumor was located in the posterior fossa; it had a supratentorial location in the patient with ATRT, was located in the sellar and suprasellar region in the two patients with germ cell tumors, and was found in the distal spinal cord in the patient with an ependymoma. In two patients ENM was detected at the time of diagnosis. In other patients ENM developed between 9 and 25 months after diagnosis. Metastatic sites included bone, bone marrow, lung, cervical lymph nodes, liver, and paranasal sinuses. Of the 10 patients who developed ENM, 8 died of their disease 0.27-16.2 months (median, 2.60 months) after it was detected. One patient with dysgerminoma is alive, without disease, 117.80 months after diagnosis of the ENM. One patient with germ cell tumor is alive with disease 11.3 months after diagnosis of the ENM. Systemic metastasis to other extraneural sites is extremely rare in children with intracranial tumors. In our series the rate of ENM is 0.98%. The liver and lung are the most common site for metastasis, followed by the bone and bone marrow. The outcome is poor in patients with CNS tumors with ENM.
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PMID:Extraneural metastasis in intracranial tumors in children: the experience of a single center. 1664 23

We examined the possibility of using microsatellite and mitochondrial DNA polymorphisms as markers to detect the clonal origin of tumor cells found in the same patient. We considered two children with complex tumor diseases: one with supratentorial primitive neuroectodermal tumors (PNET) and a hepatic rhabdoid tumor and another with brain and abdominal rhabdoid tumors. In the first patient we found an mtDNA cytosine insertion both in the normal tissue and in the primary tumor, whereas in the hepatic tumor we detected an insertion of 2 cytosine. In the second child, who had a constitutional mutation of hSNF5/INI-1, we identified the same mtDNA pattern both in normal tissue and in the abdominal tumor but not in the brain tumor, which presented three different mtDNA polymorphisms. Thus, we demonstrated the same clonal origin for tumors in the first patient and different clonal origins of the tumors in the second patient. At times it is very difficult to discriminate two neoplastic lesions or metastatic diseases by using only histopathologic techniques. Molecular examination of clonality is a useful tool to obtain information about the origin of synchronous and/or metachronous tumors found in the same patient.
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PMID:Clonality analysis of pediatric multiple tumors: two case reports and laboratory investigation. 1667 23

A new human malignant rhabdoid tumor (MRT) cell line (designated FRTK-1) was established from MRT of the kidney of an 18-month-old boy. The cell line is maintained for over 24 months with more than 100 passages. FRTK-1 cells in vitro showed 2 different growth patterns, adherent and non-adherent patterns. The FRTK-1 cells showed the same morphological and immunophenotypical characteristics as primary tumor cells of kidney. Cytogenetic and molecular analyses revealed a non-sense mutation in the hSNF5/INI1 gene and loss of expression of hSNF5/INI1 gene product protein. Epidermal growth factor receptor (EGFR) and cyclooxygenase-2 (COX-2) were expressed in the FRTK-1 cells. Until now, there has been no report of MRT cell lines with expression of COX-2. Therefore, FRTK-1 cell line might be useful for investigating biological behavior and developing new molecular targeting antitumor drugs for MRT with expression of EGFR or COX-2.
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PMID:Establishment and characterization of a new cell line, FRTK-1, derived from human malignant rhabdoid tumor of the kidney, with overexpression of epidermal growth factor receptor and cyclooxygenase-2. 1682 Sep 1


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