• Journal of Korean Neurosurgical Society
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• v.59 no.4
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• pp.357-362
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• 2016

Papillary and rhabdoid meningiomas are pathologically World Health Organization (WHO) grade III. Any correlation between clinical prognosis and pathologic component is not clear. We analyzed the prognoses of patients with meningiomas with a rhabdoid or papillary component compared to those of patients with anaplastic meningiomas. From 1994 to June 2013, 14 anaplastic meningiomas, 6 meningiomas with a rhabdoid component, and 5 meningiomas with papillary component were pathologically diagnosed. We analyzed magnetic resonance imaging (MRI) findings, extent of removal, adjuvant treatment, progression-free survival (PFS), overall survival (OS), and pathologic features of 14 anaplastic meningiomas (group A), 5 meningiomas with a predominant (${\geq}50%$) papillary or rhabdoid component (group B1), and 6 meningiomas without a predominant (<50%) rhabdoid or papillary component (group B2). Homogeneous enhancement on MRI was associated with improved PFS compared to heterogeneous enhancement (p=0.025). Depending on pathology, the mean PFS was $134.9{\pm}31.6\;months$ for group A, $46.6{\pm}13.4\;months$ for group B1, and $118.7{\pm}19.2\;months$ for group B2. The mean OS was $138.5{\pm}24.6\;months$ for group A and $59.7{\pm}16.8\;months$ for group B1. All recurrent tumors were of the previously diagnosed pathology, except for one tumor from group B1, which recurred as an atypical meningioma without a papillary component. Group B1 tumors showed a more aggressive behavior than group B2 tumors. In group B2 cases, the pathologic findings of non-rhabdoid/papillary portion could be considered for further adjuvant treatment.

• The Journal of the Korean bone and joint tumor society
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• v.7 no.2
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• pp.64-67
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• 2001

Epithelioid sarcoma has been well characterized as a distincitve entity. In the classic form, it occurs in the distal extremities of young adults. However, in the proximal form, it was found mostly in the pelvis, perineum, and genital tract of young to middle-aged adults. Morphologically, the tumor cells had promient epithelioid or rhabdoid features with marked cytologic atypia, and grew in a multinodular pattern. Immunohistochemically, cytokeratin, epithelial membrane antigen, and vimentin were positive in the tumor cells. This reports dealt with 32-year-old male lesion on his perineum. The tumor shows typical features of proximal type epithelioid sarcoma. We also discussed pathological differential diagnosis and prognosis on this particular disease.

• Molecules and Cells
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• v.45 no.12
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• pp.886-895
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• 2022

Malignant rhabdoid tumor (MRT) is a highly aggressive pediatric malignancy with no effective therapy. Therefore, it is necessary to identify a target for the development of novel molecule-targeting therapeutic agents. In this study, we report the importance of the runt-related transcription factor 1 (RUNX1) and RUNX1-Baculoviral IAP (inhibitor of apoptosis) Repeat-Containing 5 (BIRC5/survivin) axis in the proliferation of MRT cells, as it can be used as an ideal target for anti-tumor strategies. The mechanism of this reaction can be explained by the interaction of RUNX1 with the RUNX1-binding DNA sequence located in the survivin promoter and its positive regulation. Specific knockdown of RUNX1 led to decreased expression of survivin, which subsequently suppressed the proliferation of MRT cells in vitro and in vivo. We also found that our novel RUNX inhibitor, Chb-M, which switches off RUNX1 using alkylating agent-conjugated pyrrole-imidazole polyamides designed to specifically bind to consensus RUNX-binding sequences (5'-TGTGGT-3'), inhibited survivin expression in vivo. Taken together, we identified a novel interaction between RUNX1 and survivin in MRT. Therefore the negative regulation of RUNX1 activity may be a novel strategy for MRT treatment.

• The Korean Journal of Cytopathology
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• v.7 no.2
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• pp.218-224
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• 1996

We describe two cases of metastatic Wilms' tumor in the lung with emphasis on the cytologic features of specimens obtained by needle aspiration. One of them was extrarenal Wilms' tumor. The findings were correlated with the histopathologic features of the primary lesion. Cellular components in the fine needle aspiration cytology (FNAC) slides included blastemal, epithelial, stromal and inflammatory cells with immature tubular differentiation and rosette formation. Recognition of these cellular components in FNAC smears help in establishing FNAC diagnosis of Wilms' tumor. The blastemal cells were represented by small to medium sized cells with scanty cytoplasm having ill-defined borders and round to slightly oval nuclei with evenly dispersed chromatin and small marginated nucleoli. They were seen in our two cases. The differential diagnosis includes neuroblastoma, malignant lymphoma, malignant rhabdoid tumor, clear cell sarcoma, Ewing's sarcoma and embryonal rhabdomyosarcoma. In conclusion, making a definite cytologic diagnosis of metastatic Wilms' tumor may be possible by light and electron microscopy and immunohistochemical staining. The above findings may contribute to the diagnosis of FNAC of metastatic Wilms' tumor.

• Advances in pediatric surgery
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• v.13 no.2
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• pp.144-154
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• 2007

Liver tumors in children are rare, relatively complex, and encompass a broad spectrum of disease processes. This study reviews our experience of liver tumors during the last 10 years. Medical records of 36 cases of liver tumors in children, treated at Samsung Medical Centers, from October 1994 to December 2005, were reviewed in this study. We analyzed disease characters and survival rates as a whole and by specific disease. The median age was 3.6 years. Male and female ratio was 1:1. The most common symptom was the palpable mass in 15 cases. Others were abdominal distension in 9 cases, jaundice in 2, vomiting in 2, weight loss in 2, and pubic hair growth in 1. CT or US and liver biopsy were performed for diagnosis. There were 28 malignant tumors: malignant rhabdoid tumor (1 case), hepatocellular carcinoma (3 cases), hemangioendothelioma type II (3 cases), angiosarcoma (1 case), and hepatoblastoma (20 cases). Eight tumors were benign; hepatic adenoma (1 case), focal nodular hyperplasia (2 cases), hemangioendothelioma type I (2 cases), mesenchymal hamartoma (3 cases). In this study the clinical characteristics were not different from the other reports. Liver transplantation was performed in 3 cases-1 with hepatoblastoma and 2 with hepatocelleular carcinoma. Accurate and early diagnosis, and individualized multimodality therapeutic approaches might be important for better outcome.

• Investigative Magnetic Resonance Imaging
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• v.12 no.2
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• pp.188-196
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• 2008

Purpose : To describe the clinical, MR imaging, and pathologic findings of pediatric meningiomas. Materials and Methods : The authors retrospectively reviewed the medical records and MR images of 16 pediatric patients with pathologically proven meningioma. Mean patient age at diagnosis was 14 years (range, 3-18). MR images were reviewed for details of lesion sizes, locations, signal intensity (SI), marginal characteristics, internal architectures, enhancements, and dural and parenchymal changes. The findings of other imaging modalities and of pathological examinations were also analyzed. Results : Mean tumor size was 5.24 cm (range, 1.3-18.1 cm) and locations were supratentorial in 12 and infratentorial in 4. SI of masses were variable, that is, high in 9, iso in 4, and low in 3 on T2 weighted images (T2WI), and low (n=11), iso (n=4), or high (n=1) on T1WI images. All lesions were visualized as well-demarcated enhancing masses. Five of the tumors were heterogeneous with cystic or necrotic components. Dural attachment was observed in 11 patients and adjacent brain edema in 10. Tumors exhibited hyperdense (n=6) or isodense (n=4) on non-enhanced CT scans, and 3 of the 7 angiograms demonstrated blood supply from the internal carotid artery. Pathologic examinations revealed the following subtypes; transitional cell (n=4), meningotheliomatous (n=4), chordoid (n=2), fibrous (n=2), clear cell (n=1), hyalinized (n=1), rhabdoid papillary (n=1), and atypical (n=1). Conclusion : Pediatric meningiomas occur usually in teenagers, have diverse pathological types, and may produce atypical imaging findings, such as, a heterogeneous internal content or findings suggestive of intraaxial tumors.