Primary rhabdoid tumour of the brain

Jakate, S.; Marsden, H. B.; Ingram, Lori A.

doi:10.1007/bf00750268

Cited by 69 publications

(26 citation statements)

References 12 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…15,31) The presence of vimentinpositive cytoplasmic globular inclusions and membranous EMA reactivity are characteristic features of RTs at any site. Adult RTs may also be positive for S-100 protein, 13,31) GFAP, 31) cytokeratin, 11,22) and a-smooth muscle antigen, 5,17,18) and negative for desmin, neurofilament, and myoglobin. 11) In our patient, the tumor specimen was positive for vimentin, EMA, a-smooth muscle actin, neurofilament, and S-100 protein, diffusely positive for GFAP, and negative for cytokeratin AE1/AE3, desmin, and oligo2.…”

Section: Discussionmentioning

confidence: 99%

Malignant Brain Tumor With Rhabdoid Features in an Adult -Case Report-

Mutou

Hirose

Ikeda

et al. 2011

Neurol. Med. Chir.(Tokyo)

View full text Add to dashboard Cite

Rhabdoid tumor (RT) of the central nervous system is an uncommon and aggressive neoplasm that usually affects pediatric patients. Currently, these tumors are classified as malignant RT or atypical teratoid/RT. Another entity of intraparenchymal brain tumor with a rhabdoid component is the extremely rare rhabdoid glioblastoma. A 23-year-old woman presented with a malignant RT in the right thalamus. The tumor was adjacent to the right lateral ventricle and was partially resected. Histological examination revealed prominent proliferation of rhabdoid cells, which is consistent with a diagnosis of malignant RT; the typical features of glioblastoma were not observed. The tumor cells stained positively for integrase interactor-1 and glial fibrillary acidic protein. Therefore, the tumor may have originated from glial components. Genetic analysis using comparative genomic hybridization showed a deoxyribonucleic acid copy-number gain on chromosome 7 but not on chromosome 22. The tumor did not respond to chemotherapy or radiotherapy, and the patient survived for only 4 months after surgery. The present case of malignant RTs shows certain similarities with those of rhabdoid glioblastoma. Further accumulation and analysis of data, including data from genetic analyses, may lead to the identification of a new type of malignant RT.

show abstract

Section: Discussionmentioning

confidence: 99%

Malignant Brain Tumor With Rhabdoid Features in an Adult -Case Report-

Mutou

Hirose

Ikeda

et al. 2011

Neurol. Med. Chir.(Tokyo)

View full text Add to dashboard Cite

show abstract

“…There is substantial report of rhabdoid morphology's being observed in a broad spectrum of tumor types, such as malignant tumors reported as extrarenal rhabdoid tumors arising in various extrarenal sites (7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23), and a variety of malignant neoplasms of known histogenesis, including either epithelial or nonepithelial malignant tumors (24 -37). Concerning prognosis in cases with rhabdoid morphology, Wick et al determined that less than 50% of patients with extrarenal rhabdoid tumors have survived for 5 years without tumor recurrence (9).…”

Section: Discussionmentioning

confidence: 99%

“…Extrarenal malignant rhabdoid tumors have been proposed to exist at several sites, including soft parts. However, there have been doubts as to the existence of such an entity, because of the lack of clinicopathologic uniformity among the cases (7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17)(18)(19)(20)(21)(22)(23). Furthermore, the presence of rhabdoid cells has been reported in various types of specific malignant neoplasms, either mesenchymal or epithelial (24 -37).…”

mentioning

confidence: 99%

Rhabdoid Features in Leiomyosarcoma of Soft Tissue: With Special Reference to Aggressive Behavior

et al. 2000

View full text Add to dashboard Cite

The presence of rhabdoid cells has been reported in various types of malignant neoplasms and has been determined to be a predictor of aggressive behavior of neoplasms regardless of tumor histogenesis. One hundred and thirteen cases of leiomyosarcoma, selected from 1800 soft tissue sarcomas, were reviewed on hematoxylin and eosin sections, and immunohistochemical staining when available, and seven cases with rhabdoid features were retrieved. Clinicopathologic differences were analyzed to compare between cases with rhabdoid features and those without rhabdoid features. In the seven cases with rhabdoid features, two were intra-abdominal, and the others arose in external soft tissues including muscle, subcutis, and cutis. Patient age ranged from 33 to 84 years, three were female, and four were male. Tumor size ranged from 3 to 22 cm. Clinical evidence showed no differences from those cases without rhabdoid features. Histologically, one of the abdominal cases was epithelioid leiomyosarcoma. Two of the 7 cases were better subclassified as pleomorphic leiomyosarcoma, in which rhabdoid cells are diffusely scattered. In cases other than those with pleomorphic leiomyosarcomas, foci of anaplastic areas were observed, and collections of rhabdoid cells were present in those areas. Immunohistochemical examination of the cases confirmed myogenic differentiation, and showed rhabdoid cells being positive for vimentin and desmin in the inclusion bodies, and diffusely so for muscle actin in the cytoplasm. After dividing all the cases of leiomyosarcoma by their location, prognostic analysis was performed. Leiomyosarcoma of external soft tissue with rhabdoid cells showed a tendency for poorer prognoses than cases without rhabdoid features. On the contrary, retroperitoneal cases did not. This study indicates that rhabdoid features are associated with aggressive biological behavior in leiomyosarcoma of the external soft tissue.

show abstract

“…MRT also has an extremely poor prognosis because of its high potential for metastasis (1). Primary MRT has been described in the CNS, pelvis, and paravertebral regions (2)(3)(4)(5)(6)(7). A characteristic feature of MRT cells is the presence of a large eosinophilic inclusion in the cytoplasm (8,9).…”

mentioning

confidence: 99%

TRAIL/Apo2L Ligands Induce Apoptosis in Malignant Rhabdoid Tumor Cell Lines

et al. 2003

View full text Add to dashboard Cite

Tumor necrosis factor-related apoptosis-inducing ligand (TRAIL/Apo2L) is a potent inducer of apoptosis in various cancer cells, whereas normal cells are not sensitive to TRAILmediated apoptosis. Four TRAIL/Apo2L receptors (DR4, DR5, DcR1, and DcR2) have been identified. DR4 and DR5 have a death domain, whereas DcR1 and DcR2 are called decoy receptors because of their incomplete or lack of a death domain. Malignant rhabdoid tumor (MRT) is an aggressive neoplasm showing a poor prognosis because of its resistance to chemotherapeutic agents. In this study, we examined whether TRAIL could induce apoptotic cell death in MRT cell lines. We found that although half of the MRT cell lines examined were sensitive to TRAIL/Apo2L, Western blot analysis revealed that the expression of DcR2 was low in TRAIL-sensitive MRT cells. We examined the effect of doxorubicin on the expression levels of TRAIL receptors and its enhancement on the susceptibility of MRT cell lines to TRAIL. Western blot and flow cytometric analyses revealed that doxorubicin significantly increased the expression of DR5, and somewhat up-regulated the expression of Abbreviations MRT, malignant rhabdoid tumor TNF, tumor necrosis factor TRAIL/Apo2L, tumor necrosis factor-related apoptosis-inducing ligand or Apo2 ligand NF-B, nuclear factor-B PI3-kinase, phosphatidylinositol 3-kinase PI, propidium iodide MTT, 3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium bromide MRTs were first described in the kidney as a rare variant of Wilms' tumor with a rhabdomyosarcomatoid pattern. Unfortunately, there is no effective treatment for this disease because it is largely resistant to both chemotherapy and radiotherapy. MRT also has an extremely poor prognosis because of its high potential for metastasis (1). Primary MRT has been described in the CNS, pelvis, and paravertebral regions (2-7). A characteristic feature of MRT cells is the presence of a large eosinophilic inclusion in the cytoplasm (8, 9). In the brain, the tumors may present as a mixture of rhabdoid tumor with primitive neuroectodermal tumors, mesenchymal, or epithelial element, an entity referred to as atypical teratoid/rhabdoid tumors (10, 11). Most atypical teratoid/rhabdoid tumors demonstrate monosomy 22 or deletions of chromosome band 22q11 with alterations of the hSNF5/INI1 gene (12). Recently study revealed that MRT has truncated mutations and homozygous deletions of hSNF5/INI1 gene, suggesting a tumor suppressor gene (13).TNF-related apoptosis-inducing ligand or Apo2 ligand (TRAIL/Apo2L) was recently identified as a member of the TNF superfamily of cell death-inducing ligands. This ligand is a type II transmembrane protein that triggers apoptosis mainly in tumor cells (14,15). TRAIL induces apoptotic cell death in many cancer or transformed cells both in vitro and in various in vivo tumor models (16 -20). However, its proapoptotic effects are minimal in normal human cells in vitro and in TRAIL-treated animals (14,(21)(22)(23)(24)(25). TRAIL mRNA is constitutively present in many tissues, unlike the r...

show abstract

Primary rhabdoid tumour of the brain

Cited by 69 publications

References 12 publications

Malignant Brain Tumor With Rhabdoid Features in an Adult -Case Report-

Malignant Brain Tumor With Rhabdoid Features in an Adult -Case Report-

Rhabdoid Features in Leiomyosarcoma of Soft Tissue: With Special Reference to Aggressive Behavior

TRAIL/Apo2L Ligands Induce Apoptosis in Malignant Rhabdoid Tumor Cell Lines

Contact Info

Product

Resources

About