• Journal of Korean Neurosurgical Society
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• 제59권4호
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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.

• 대한골관절종양학회지
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• 제7권2호
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• pp.64-67
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• 2001

유상피육종은 전형적인 형태인 경우에는 비교적 젊은 사람의 손이나 발과 같은 말단부에 호발하는 종양이다. 그러나 근위형의 경우는 주로 청장년층의 골반, 회음부 또는 생식기에 발생한다. 형태학적으로 종양세포는 뚜렷한 유상피 형태를 취하거나, rhabdoid형태를 보이며, 심한 세포학적 이형증과 함께, 다결절 형태로 성장한다. 면역조직화학적 검사에서는 특징적으로 cytokeratin, epithelial membrane antigen, vimentin이 양성이다. 본 보고는 32세 남자의 회음부에 발생한 근위형 유상피육종을 기술하였다. 형태학적으로 전형적인 근위형 유상피 육종의 조직학적 소견을 관찰하였으며, 병리조직학적 감별점과, 환자의 예후에 관하여 토론하였다.

• Molecules and Cells
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• 제45권12호
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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.

• 대한세포병리학회지
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• 제7권2호
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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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• 제13권2호
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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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• 제12권2호
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• pp.188-196
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• 2008

목적: 이 연구의 목적은 소아 수막종의 임상적, 영상의학적, 그리고 병리학적 특징을 기술함에 있다. 대상 및 방법: 병리학적으로 진단된 수막종을 가진 16예의 소아환자를 대상으로 임상기록과 자기공명영상을 후향적으로 분석하였다. 평균연령은 14세 (3-18세)였다. 자기공명영상은 병변의 크기, 신호강도, 경계부의 특징, 내부 구조, 조영증강, 그리고 경막과 뇌실질의 변화를 검토하였고 다른 영상 방법 및 병리소견을 함께 분석하였다. 결과: 종양의 평균 크기는 5.24 cm (1.3-18.1 cm)였으며 12예는 천막상부에 4예는 천막하부에 위치하였다. T2 강조 영상에서 높은 신호강도를 보이는 종양이 9예 있었고 4예에서 동신호강도를, 3예에서 낮은 신호강도를 보였다. T1 강조 영상에서는 낮은 신호강도 11예, 동신호강도 4예, 그리고 높은 신호강도 1예가 있었다. 모든 종양의 경계가 좋았고 조영증강을 보였다. 5예에서 종양은 균질한 양상을 보였으며 나머지에는 낭종이나 괴사에 의해 비균질한 양상을 보였다. 경막 부착 소견이 11예에서, 뇌실질 부종이 10예에서 동반되었다. 컴퓨터 단층촬영 소견은 6예에서 밝은 음영을 보였으며 5예에서 석회화를 동반하였다. 혈관 조영술 상 3예는 내경동맥에서, 4예는 외경동맥으로부터 혈액 공급이 이루어졌다. 병리검사결과 종양의 아형은 이행성 (4예), 수막세포성 (4예), 척삭성 (2예), 섬유성 (2예), 투명세포성 (1예), 유리질화성 (1예), 횡문근양 유두모양 (1예), 그리고 비정형(1예) 수막종으로 진단되었다. 결론: 소아의 수막종은 주로 10대에 발생하며 다양한 병리학적 아형을 보일뿐만 아니라 비정형적인 영상 소견으로 인해 축내 종양으로 오인될 수 있다.