• 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.

• 대한수의학회지
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• 제49권4호
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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.

• Journal of Korean Neurosurgical Society
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• 제39권2호
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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.

• Tuberculosis and Respiratory Diseases
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• 제67권5호
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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.

• 대한영상의학회지
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• 제82권2호
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• pp.493-497
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• 2021

악성 횡문근양 종양은 주로 영유아의 신장에서 발생하는 공격적인 종양이다. 신장 이외의 조직에서 발생한 경우 신외성 악성 횡문근양 종양이라 하고 중추신경계, 간, 뇌, 피부, 연조직 등에서 드물게 보고되었다. 위에서 발생한 악성 횡문근양 종양은 매우 드물다. 저자들은 흑색변을 주소로 내원한 72세 남자의 위에서 발견된 악성 신외성 횡문근양 종양이 림프종과 유사한 영상 소견을 보인 예를 경험하여 보고한다.

• Journal of Gastric Cancer
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• 제7권2호
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• pp.107-112
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• 2007

횡문근양 종양은 윌름씨 종양의 횡문근육종 형태의 드문 변종 아형으로 여겨져 왔으나, 1989년에 신장에서 발생한 황문근양 종양이 처음으로 기술된 이후 위장관에서도 드물게 보고되고 있다. 적절한 치료는 근치적 절제술이여 술후 보조 항암요법의 효과는 보고되지 않았다. 신장외 횡문근양 종양은 신장에서 발생한 횡문근양 종양과는 경과가 다르며 불량한 예후를 보인다. 신장외 횡문근양 종양 중에서도 횡문근양 형태의 미분화 위 선암은 매우 드물며 예후가 불량하다. 63세 남자 환자가 위 체부의 대만부에 발생한 종괴로 위전절제술을 시행받았으며, 병리조직 결과에서 횡문근양 형태의 미분화 위 선암이 진단되었다. 이에 본 저자들은 면역조직화학적으로 진단된 횡문근양 형태를 보이는 미분화 위 선암을 경험하여 증례를 보고하는 바이다.

• Tuberculosis and Respiratory Diseases
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• 제77권1호
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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.

• Journal of Korean Neurosurgical Society
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• 제38권5호
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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.

• 대한골관절종양학회지
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• 제6권4호
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• pp.173-177
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• 2000

횡문근양 종양은 주로 소아기의 신장에서 발생하는 신생물로, 침습적인 양상을 보이는 비교적 드문 종양이다. 최근에 신장 외의 다양한 부위에서 발생한 예들이 보고되고 있으며, 연부 조직에 발생한 경우에는 미분화된 횡문근 육종으로 오진되기 쉽다. 횡문근양 종양은 횡문근 육종보다 침습적이고 나쁜 예후를 보이는 종양으로, 횡문근 육종과의 조직학적인 정확한 감별이 필요하며, 면역조직화학 검사가 그 감별에 도움을 줄 수 있다. 저자들은 치골 부위와 하복벽 부위에서 발생한 횡문근양 종양을 경험하여 문헌의 고찰과 함께 보고하고자 한다.

• Journal of Korean Neurosurgical Society
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• 제61권3호
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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.