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A case of atypical teratoid/rhabdoid tumor (AT/RT) presenting as unusual cutaneous lesion
( Seung Gi Hong ),( Sun Young Jo ),( Ki Woong Ro ),( Eun Phil Heo ) 대한피부과학회 2018 대한피부과학회 학술발표대회집 Vol.70 No.1
Atypical teratoid/rhabdoid tumor (AT/RT) is a rare malignant tumor that occurs in children especially in the first few years of life. It is a tumor with high malignancy, rapid growth, poor response to treatment, and very poor prognosis. Skin metastasis has been reported very rarely and appears to be a hamartoma pattern mimicking skin tag. Immunohistochemistry is a complex result that is expressed differently in different parts of the body depending on histologic features. Integrase Interactor-1 (INI-1) immunostaining and tumoral and leukocytes INI-1 gene sequencing assays are used as confirmatory tests in cerebral tumors. In skin biopsies, INI-1 immunostaining is used as a confirmatory test. In immunohistochemical staining, loss of INI-1 is characteristic. In our case, a skin examination revealed a cluster of multiple papules with pale erythema on the right side of the back. At 2 months of age, chondromyxoid fibroma was seen on histologic examination. Two months later, he was admitted to the hospital with seizure. After surgery, AT/RT was diagnosed by INI-1 loss of expression in immunochemical staining. Then, additional immunochemical staining was performed on the skin specimens, and INI-1 loss of expression was detected, In patients with macrocephaly, hydrocephalus, and intracranial tumors, loss of expression through INI-1 immunostaining may be helpful in the diagnosis of unusual skin lesions such as polypoid soft fibroma.
중추 신경계에서 발생한 비정형 기형/횡문근양 종양 -증례보고-
한성록 ( Seong Rok Han ),윤상원 ( Sang Won Yoon ),이기택 ( Gi Taek Yee ),손문준 ( Moon Jun Sohn ),주미 ( Mee Joo ),황충진 ( Choong Jin Whang ) 대한뇌종양학회 2004 대한뇌종양학회지 Vol.3 No.2
Atypical teratoid/Rhabdoid tumors(AT/RT) are aggressive neoplasm that afflict infants and young children and are the new entity among malignant pediatric brain tumors. These tumors show similar histological and radiological features to primitive neuroectodermal tumor-medulloblastoma(PNET-MB). So, these tumors have been misdiagnosed previously as PNET-MB. Separation of these two tumor types is crucial. Because the prognosis of a patient with an AT/RT is worse than that of a PNET-MB despite aggressive surgical treatment with or without adjuvant chemotherapy and radiation therapy. Recently, we experience a 5-month girl with a primary intracranial AT/RT in the posterior fossa. We report the case with a special emphasis on the differential diagnosis from PENT-MB.
성인에서 발생한 터어키안 상부 Atypical Teratoid/Rhabdoid Tumor -증례보고-
이종명 ( Jong Myong Lee ),정신 ( Shin Jung ),정태영 ( Tae Young Jung ),김인영 ( In Young Kim ),강삼석 ( Sam Suk Kang ),이민철 ( Min Chul Lee ) 대한뇌종양학회 2004 대한뇌종양학회지 Vol.3 No.2
Atypical teratoid/rhabdoid tumor(AT/RT) of the central nervous system is a rare and extremely aggressive malignancy, mostly found in early childhood and the posterior fossa appears to be a common location for AT/RT. We report a case of suparsellar AT/RT in an adult patient. A 19-year-old man was admitted with decreased visual acuity and visual field defects for three years. Magnetic resonance image showed a heterogenously enhancing multiseptated mass in the suprasellar area and thickened pituitary stalk. A gross total resection was performed by the subfrontal translamina terminalis approach. Histological diagnosis revealed it as an atypical teratoid/rhabdoid tumor. Local radiotherapy in the suprasellar region was given with total dose of 5400cGy in 27 fractions. The patient has attended regular follow-ups and took hormonal medications without the recurrence of tumor for one year after the surgery.
김기수(Ki Soo Kim),정철구(Chul Ku Jung),김윤미(Yoon-Mee Kim),고경옥(Kyong-Og Ko),이영혁(Young Hyuk Lee) 대한소아신경학회 2004 대한소아신경학회지 Vol.12 No.2
중추신경계의 비전형 기형/횡문 종양은 주로 영유아기에 많이 발생하는 매우 희귀한 종양으로 구토, 기면, 보챔 등의 비특이적 증상이나 두통, 뇌신경마비등의 증상이 나타날 수 있다. 진단은 대뇌전산화 다층촬영과 핵자기 공명 영상을 통하여 할 수 있고 조직검사를 통해 확진할 수 있다. 이 종양은 원시 신경 외피 종양-수모세포종과 매우 유사하여 흔히 잘못 지단되는 경우가 많으나 비전형 기영/횡문 종양이 좀더 어린 연령에서 발생하고 소뇌에 발생한느 경우가 더 적으며 치료에 대한 반응이 매우 나쁘다. 치료는 수술, 항암 화학요법, 방사선치료 등으로 구성되어 있고 치료에 대한 예후는 매우 나쁘다. 저자들은 중추형 안면 신경마비를 주소로 내원한 2세 된 여아에서 비전형 기형/횡문 종양 1례를 경험하였기에 문헌 고찰과 함께 보고하는 바이다. Primary central nervous system atypical teratoid/rhabdoid tumors are rare and extremely aggressive malignancies of early childhood. These tumors are most common in infants less than 2 years of age. Diagnosis is based on distinctive light microscopic and immunohistochemical findings, coupled with a molecular genetic analysis. A histologic features of these tumors are epithelial and/or mesenchymal components in addition to rhabdoid, with or without neuroepithelial fields. The expression of the epithelial membrane antigen, vimentin, and the smooth muscle actin are characteristic of these tumors. Treatment includes surgery, chemotherapy, and radiotherapy. Prognosis is poor despite of an aggressive therapy. We report one case of an atypical teratoid/rhabdoid tumor in a young child. She is an 18 month-old-girl who presented with central type facial palsy. After extensive surgery she improved transiently but relapsed immediately. Her condition was not permitted to receive radiotherapy or chemotherapy. Thereafter, with phrenic nerve palsy, she suffered from recurrent episodes of pneumonia and respiratory difficulties. Finaly, she was expired three months after the diagnosis and treatment.
유아에서 발생한 비정형 기형/횡문근양 종양 -증례보고-
문종언 ( Jong Un Moon ),김태성 ( Tae Sung Kim ),박봉진 ( Bong Jin Park ),임영진 ( Young Jin Lim ) 대한뇌종양학회·대한신경종양학회·대한소아뇌종양학회 2007 대한뇌종양학회지 Vol.6 No.2
Atypical teratoid/rhabdoid tumor(AT/RT) is extremely rare and highly malignant tumor of early childhood. AT/RT shows similar histopathological and radiological features to primitive neuroectodermal tumor(PNET) but more aggressive behavior than PNET. We report a case of supratentorial AT/RT with review of literatures. A 11-month-old female presented with a 2-weeks history of left side hemiparesis and convulsion. On neurologic examinations, there were no specific findings on developments and activities, except left side hemiparesis and intermittent seizure. Brain magnetic resonance images(MRI) revealed PNET such as primary cerebral neuroblastoma at right frontotemporal lobe with midline shift to the left side and suggestive of seeding nodule at occipital horn of left lateral ventricle. There were no evidences of spinal metastasis. Right frontotemporal mass was subtotally removed. Symptoms and signs were mildly improved but seizure was continued. Histopathological examination showed findings of AT/RT. In spite of our recommendation of radiation therapy and chemotherapy, her parents refused further treatment. On postoperative brain computed tomography(CT) scan, subtotal removal state of right frontotemporal lobe mass with marked reduction of mass effect and midline shift were shown. On follow-up radiologic examinations at postoperative 4 months, residual tumor was grown and hydrocephalus was progressed aggressively. AT/RT of the cerebrum is an extremely rare and aggressive tumor of early childhood. We suggest that aggressive management may be required even though tumor should be progressed rapidly and shows poor prognosis.
Atypical Teratoid Rhabdoid Tumors in Adult Patient with Multiple Lesions
Oh, Young-Min,Koh, Eun-Jeong,Choi, Ha-Young The Korean Neurosurgical Society 2005 Journal of Korean neurosurgical society Vol.38 No.5
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.
홍승기 ( Seung Gi Hong ),조선영 ( Sun Young Jo ),노기웅 ( Ki Woong Ro ),허은필 ( Eun Phil Heo ) 대한피부과학회 2019 大韓皮膚科學會誌 Vol.57 No.5
An atypical teratoid/rhabdoid tumor (AT/RT) is a rare malignancy occurring in the first few years of life. This tumor shows rapid growth, a poor response to treatment, and poor prognosis. Cutaneous metastases presents as hamartomatous lesions mimicking skin tags. Immunohistochemical examination shows varied patterns of expression based on the sites of the body affected. Integrase interactor-1 (INI-1) gene sequencing and loss of expression of INI-1 observed with immunostaining can confirm AT/RT. In our patient, the skin lesion was identified at birth. Histopathological examination of the skin lesion could not establish an accurate diagnosis. Two months later, the patient presented with a brain tumor. Immunohistochemical examination of the brain lesion revealed complete loss of INI-1 expression in tumor cells, and the lesion was diagnosed as AT/RT. After that, we can detect the loss of INI-1 expression in the skin on the back. We report a rare case of AT/RT affecting the brain with cutaneous metastasis diagnosed with immunohistochemical staining. (Korean J Dermatol 2019;57(5):274∼278)