• Title/Summary/Keyword: Rhabdoid

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Meningiomas with Rhabdoid or Papillary Components : Prognosis and Comparison with Anaplastic Meningiomas

  • Kim, Jeong-Kwon;Jung, Tae-Young;Jung, Shin;Lee, Kyung-Hwa;Kim, Seul-Kee;Lee, Eun Jung
    • 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.

Rhabdoid tumor in the gluteal region of a Shit-tzu dog

  • Chung, Jae-Yong;Do, Sun-Hee
    • Korean Journal of Veterinary Research
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    • v.49 no.4
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    • pp.361-363
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    • 2009
  • Rhabdoid tumor is an aggressive neoplasm of animals and human. It is similar with rhabdomyosarcoma histopathologically. But cellular origin of this neoplasm showed no striated muscle origin by immunohistological and ultrastructural studies. Castrated male Shit-tzu dog, 6 years old, had a mass in the left gluteal region near to the tail. The mass was examined histopathologically and immunohistologically. Histopathologically, the tumor was consisted of large polygonal cell with abundant eosinophilic cytoplasm. The nuclei in some cells were marked eccentrically located. Immunohistochemically, many neoplastic cells were positive for vimentin. These findings were similar to histopathological and immunohistological features of human rhabdoid tumor and few rhabdoid tumors in animals.

A Case of Rhabdoid Meningioma

  • Han, Jung-Ho;Seol, Ho-Jun;Kim, Dong-Gyu;Jung, Hee-Won
    • Journal of Korean Neurosurgical Society
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    • v.39 no.2
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    • pp.144-147
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    • 2006
  • Rhabdoid meningioma is an uncommon meningioma variant, which was initially described in 1998 by Perry et al. It is classified as a variant corresponding to grade III in the recent World Health Organization[WHO] classification of tumors of the nervous system, because of its unique histopathological features and its highly aggressive clinical course. To date there were still a lot of arguments about their developmental patterns and the treatment strategy especially for rhabdoid meningiomas lacking other histological features of malignancy. Although the exact causes of rhabdoid transformation of a benign meningioma are unknown, malignant transformation long duration after surgical resection under an underlying predilection for malignancy is suggested in our case. And we suggest that any histological rhabdoid features in recurrent meningiomas or even in primary cases seem to indicate the malignant clinical course and the need for aggressive treatments, because transformation from a benign or atypical one to a malignant one seems to occur at last.

A Case of Malignant Rhabdoid Tumor in the Anterior Mediastinum (전종격동에서 발생한 악성 횡문근양 종양 1예)

  • Oh, Kyung Jin;Lee, Ki Byung;Hong, Soon Won;Jung, Kyo Tae;Choi, Hong Kyu;Kim, Hyung Jung;Ahn, Chul Min;Chang, Yoon Soo
    • Tuberculosis and Respiratory Diseases
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    • v.67 no.5
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    • pp.462-466
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    • 2009
  • Malignant rhabdoid tumors arise primarily from the kidney. Extrarenal malignant rhabdoid tumors are rare, with the liver, central nervous system, and skin reported as the primary sites. Malignant rhabdoid tumors of the mediastinum are extremely rare among extrarenal malignant rhadoid tumors; only 3 cases have been reported to date, all characterized by aggressive clinical behavior. We experienced a 35-year-old woman diagnosed with malignant rhabdoid tumor in the anterior mediastinum with multiple metastases. The tumor was surgically unresectable, and treated with palliative radiation therapy. Three-month after radiation treatment, she died from dissemination of the malignant rabdoid tumor.

Imaging Findings of a Malignant Rhabdoid Tumor in the Stomach: A Case Report (위에 발생한 악성 횡문근양 종양의 영상 소견: 증례 보고)

  • Hye Ran Yoon;Dong Hee Park;Joonseog Kong
    • Journal of the Korean Society of Radiology
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    • v.82 no.2
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    • pp.493-497
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    • 2021
  • A malignant rhabdoid tumor is an aggressive tumor that occurs mainly in the kidney of infants and children. When it occurs in extrarenal sites, it is referred to as an extrarenal malignant rhabdoid tumor. Although a few cases of malignant rhabdoid tumor occuring in the central nervous system, liver, brain, skin, and soft tissue have been reported, it is rarely observed in the stomach. We report the imaging findings of a malignant rhabdoid tumor of the stomach that mimicked a gastric lymphoma in a patient who presented with melena.

Undifferentiated Adenocarcinoma with Rhabdoid Features in the Stomach: A Case Report (횡문근양 미분화 위선암 1예 보고)

  • Im, Myoung-Goo;Jun, Kyong-Hwa;Won, Yong-Sung;Jung, Ji-Han;Chin, Hyung-Min;Park, Woo-Bae;Chun, Chung-Soo
    • Journal of Gastric Cancer
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    • v.7 no.2
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    • pp.107-112
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    • 2007
  • Rhabdoid tumor has been considered to be a rare subtype of Wilm's tumor with Rhabdomyoma features. Since rhabdoid tumor that developed in the kidney was described for the first time in 1989, it has been reported in the gastrointestinal tract, although this is rare. The appropriate treatment is radical resection, and the effect of adjuvant chemotherapy has not yet been reported on. The outcome of extra-renal rhabdoid tumor is different from renal rhabdoid tumor and the former shows a poor prognosis. Among extra-renal rhabdoid tumors, undifferentiated gastric adenocarcinoma with rhabdoid features is very rare and its prognosis is poor. A 63 years old male patient underwent total gastrectomy for a tumor that developed in the greater curvature of the gastric body and this was diagnosed as undifferentiated gastric adenocarcinoma with rhabdoid features, according to the histopathology. We experienced an undifferentiated gastric adenocarcinoma with rhabdoid features that was diagnosed by immunohistochemical staining and we report here on this case.

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A Case of Lung Carcinoma with Rhabdoid Phenotype Mimicking an Aspergilloma in Patient with Recurrent Hemoptysis

  • Kim, Moo Woong;Rew, Soo Jung;Eun, Seo Joon;Lee, Ui Sin;Park, Chan Woo;Jeong, Jong Pil;Ko, Young Choon
    • Tuberculosis and Respiratory Diseases
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    • v.77 no.1
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    • pp.38-41
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    • 2014
  • Malignant rhabdoid tumor was first discovered in the kidney, and rhabdoid tumor of the lung was first reported in 1995. These were included as the variants of large-cell carcinoma, according to the 1999 World Health Organization classification of lung tumors. The rhabdoid tumor of the lung exhibits aggressive biological behavior and has a poor prognosis, and only a few reports of this tumor exist. We report a case of lung carcinoma with a rhabdoid phenotype, initially misdiagnosed as an aspergilloma, in a 48-year-old man who presented with recurrent hemoptysis. The chest computed tomography scans showed a huge consolidative lesion with an air crescent sign in the left upper lung and no contrast-enhancing lesion. An aspergilloma was diagnosed by the radiologist. However, after surgical excision and pathological examination, rhabdoid carcinoma was diagnosed. A surgical resection helps to make it possible to pathologically distinguish a malignancy from an aspergilloma.

Atypical Teratoid Rhabdoid Tumors in Adult Patient with Multiple Lesions

  • Oh, Young-Min;Koh, Eun-Jeong;Choi, Ha-Young
    • Journal of Korean Neurosurgical Society
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    • v.38 no.5
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    • pp.387-389
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    • 2005
  • Primary atypical teratoid/rhabdoid tumor[AT/RT] of the central nervous system is a recently described, highly malignant neoplasm that usually occur in the posterior fossa of children. Although AT/RT usually occurs in young children, AT/RT is being recognized in adults with increasing frequency. The authors report 49-year-old man with multiple AT/RT lesions [right lateral ventricle, right temporal lobe and right cerebellum]. Histopathologic findings showed typical rhabdoid cells with eccentric nuclei and prominent nucleoli. Eventhough the tumor was removed, a patient was dead in one month after surgery due to recurrence and rapid regrowth of the tumor.

Malignant Extrarenal Rhabdoid Tumor in Soft Tissue - A Case Report - (연부 조직에 발생한 악성 횡문근양 종양 - 증례 보고 -)

  • Lee, Sang-Hoon;Kim, Han-Soo;Oh, Joo-Han;Suh, Sung-Wook;Lee, Han-Koo
    • The Journal of the Korean bone and joint tumor society
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    • v.6 no.4
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    • pp.173-177
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    • 2000
  • Malignant rhabdoid tumor is a highly aggressive tumor of children, that often arises in the kidney. Some rhabdoid tumors have been reported in various extra-renal location including the central nervous system, liver, skin, and soft tissues. In case of arising in soft tissues, it may be misdiagnosed as rhabdomyosarcoma. It is important to distinguish malignant rabdoid tumor from rhadomyosarcoma, because malignant rhabdoid tumor has more aggressive behavior and poorer survival rate. And this differential diagnosis can be performed by several immunohistochemistry. Here we report a case of malignant rhabdoid tumor that arose in lower abdominal wall with related articles.

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Atypical Teratoid Rhabdoid Tumour : From Tumours to Therapies

  • Richardson, Elizabeth Anne;Ho, Ben;Huang, Annie
    • Journal of Korean Neurosurgical Society
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    • v.61 no.3
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    • pp.302-311
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    • 2018
  • Atypical teratoid rhabdoid tumours (ATRTs) are the most common malignant central nervous system tumours in children ${\leq}1year$ of age and represent approximately 1-2% of all pediatric brain tumours. ATRT is a primarily monogenic disease characterized by the bi-allelic loss of the SMARCB1 gene, which encodes the hSNF5 subunit of the SWI/SNF chromatin remodeling complex. Though conventional dose chemotherapy is not effective in most ATRT patients, high dose chemotherapy with autologous stem cell transplant, radiotherapy and/or intrathecal chemotherapy all show significant potential to improve patient survival. Recent epigenetic and transcriptional studies highlight three subgroups of ATRT, each with distinct clinical and molecular characteristics with corresponding therapeutic sensitivities, including epigenetic targeting, and inhibition of tyrosine kinases or growth/lineage specific pathways.