• Title/Summary/Keyword: Rhabdomyosarcomas

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구강의 횡문근육종

  • Cho, Han-Kuk;Kim, Hun-Il;Yeuh, In-Haeng;Shin, Mu-Soo
    • The Journal of the Korean dental association
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    • v.12 no.12
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    • pp.913-918
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    • 1974
  • The authors have observed clinically and histopathologically on two cases of rhabdomyosarcomas occurred in the oral cavity. The results were as follow: 1. A case of adult pleomorphic rhabdomyosarcoma, involving the right mandibular third molar area of 52 year old male, revealed pathological fracture of the right mandibular angle area. Microscopically, sections of this tumor showed cross striations by staining of phosphotungstic acid hematoxylin. 2. A case of embryonal alveolar rhabdomyosarcoma, involving the right buccal area of one year and ten months old female, revealed severe bone destruction on the right mandibular body area. Microscopically, sections of this tumor did not show cross striations.

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Embryonal Rhabdomyosarcoma of Urinary Bladder Diagnosed by Urine Cytology - A Case Report - (뇨세포검사로 진단된 방광의 배아성 횡문근육종 - 1 증례 보고 -)

  • Kim, Joo-Heon;Lee, Ho;Kang, Myoung-Jae;Lee, Dong-Geun;Kim, Sang-Ho
    • The Korean Journal of Cytopathology
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    • v.5 no.1
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    • pp.71-73
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    • 1994
  • Rhabdomyosarcomas are found mainly in young patients, but rare in adults. A correlated cytological and histologic study of one case of embryonal rhabdomyosarcoma is presented. The cytologic appearance of the urine smear corresponded well with the histologic findings. Cytologically, two main cell types were distinguished ; a predominant primitive, small round cell with scant cytoplasm and a large cell with abundant cytoplasm. The cytologic feature proving rhabdomyoblastic differentiation, such as cross-striation, was absent.

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A Case of Laryngeal Rhabdomyosarcoma (후두부횡문근육종의 1례)

  • 이순철;김석도;김시원
    • Proceedings of the KOR-BRONCHOESO Conference
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    • 1981.05a
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    • pp.9.3-9
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    • 1981
  • Rhabdomyosarcomas are relatively rare and highly malignant tumors which originated from mesenchymal cells. A 50-year-old man, suffered from hoarseness for 2 months, visited to the Dept. of ENT, Seoul Red Cross Hospital. By indirect laryngoscopic examination, a sessile based whitish polypoid mass (0.5cm$\times$0.3cm) was noted at the one third portion of right true vocal cord. Under general anesthesia, local excision was performed. Biopsy revealed rhabdomyosarcoma of the embryonal type. To our knowledge, only 24 cases of laryngeal rhabdomyosarcoma have been reported in the world literature.

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Cytologic Features and Distribution of Primary Sites of Malignant Cells in Cerebrospinal Fluid - Analysis of 1,438 Specimeus - (뇌척수액내 종양세포의 원발부위 분석 및 세포학적 특징 - 총 1,438예 분석 -)

  • Kim, Yeon-Mee;Jeon, Mi-Yeong;Chi, Je-Geun
    • The Korean Journal of Cytopathology
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    • v.11 no.2
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    • pp.65-73
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    • 2000
  • Cytologlc evaluation of cerebrospinal fluid(CSF) is an effective mean for diagnosing many disorders involving the central nervous systems(CNS). One of the most important reasons for cytologic examination of CSF is to detect metastatic or primary neoplasms of the CNS. We did a retrospective study of 1,438 CSF specimens obtained between 1992 and 1996. A total of 1,205 adult and 233 pediatric CSF specimens from 947 patients were accessed at the Department of Pathology of Seoul National University Hospital and Children's Hospital, respectively. Among 1,438 CSF cytology specimens, 169 cases(11.8%, 77 patients) including 135 adult cases(59 patients) and 34 pediatric cases(18 patients) were positive for malignant cells. Diagnoses included 50 metastatic carcinomas(adult, 60; pediatric, 0); 46 malignant lymphomas(adult, 44; pediatric, 2); 21 leukemias(adult, 20; pediatric, 1); 4 retinoblastomas(adult, 0; pediatric 4); 2 rhabdomyosarcomas(adult, 0; pediatric, 2); 1 multiple myeloma(adult, 1; pediatric, 0), and 35 primary CNS neoplasms(adult, 10; pediatric, 25). The most commonly identified metastatic carcinomas in adults were adenocarcinoma. Their primary sites were the lung, gastrointestinal tract, and breast in order of frequency. The most common primary CNS neoplasm in children was medulloblastoma.

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A Case of Fibrous Mass Diagnosed as Spidle Cell Rhabdomyosarcoma in the Vocal Fold (방추세포성 횡문근육종으로 진단된 성대 내 섬유성 종물 1예)

  • Kim, Dae Young;Hwang, Jun-Ha;Park, In Suh;Lim, Jae-Yol
    • Journal of the Korean Society of Laryngology, Phoniatrics and Logopedics
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    • v.27 no.2
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    • pp.126-129
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    • 2016
  • Rhabdomyosarcoma is an uncommon type of soft tissue malignant neoplasm characterized by undifferentiated mesodermal tissue. Sarcomas account for approximately 1% of all laryngeal neoplasm and rhabdomyosarcomas are the rarest sarcoma found in the larynx. When the sarcoma involves the larynx, radical surgery such as laryngectomy has been considered. With recent advances of combined therapy, however, it can be treated by conservative surgeries followed by postoperative radiotherapy and/or pulse chemotherapy. With reviews of literature, we report a 47-year-old patient complaining of husky voice and throat discomfort who was finally diagnosed as rhabdomyosarcoma of the vocal fold and successfully treated by laser cordectomy followed by adjuvant chemoradiotherapy.

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Treatment Results of Rhabdomyosarcomas of Head and Neck (두경부의 횡문근육종에서 다방면요법의 효과)

  • Ahn Ki-Jung;Suh Chang-Ok;Kim Gwi-Eon
    • Korean Journal of Head & Neck Oncology
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    • v.7 no.1
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    • pp.45-51
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    • 1991
  • 최근 횡문근육종을 치료함에 있어서 수술적 요법, 방사선 치료, 강암 약물요법을 적절히 병행함으로써 치료에 따르는 후유증을 극소화하면서 괄목할만한 생존율 향상을 가져오게 되었다. 특히 두경부의 횡문근육종은 소아에서 다발하고 병소의 위치에 따라서 각각 다른 임상적 특성을 가지며 수술적 절제를 했을 때 기능 및 외견상 결손이 크므로 별도의 연구 대상이 되어 왔으며 수술적 방법보다는 방사선 치료 및 항암 약물요법이 강조되고 있다. 저자들은 1976년부터 1987년까지 두경부 횡문근육종으로 진단받고 연세 암센터에서 항암 약물요법 및 방사선 치료를 받은 22명의 환자를 대상으로 하여 발생 부위, 조직 병리, 병기별 분포 및 생존율, 치료 방법에 따른 생존율을 후향성으로 분석하였다. 22명의 환자중 10 세 미만이 12 예로 가장 많았고, 13명이 배아세포형이였으며, 임상병기는 병기 III 이 가장 많아서 14 예였다. 병소의 위치는 안와가 6명으로 가장 많았고, 뇌수막주변부가 7명 (상악동 3명, 중이도 2명, 비강 l명, 비인강 1명), 기타 9명 (경부 5명, 이하선 2명, check 2명) 이었다. 5년 무병 생존율은 안와 종양에서 가장 높아서 50% 였고, 안와외 두경부가 37.5%, 뇌수막주변부가 16.7% 로 가장 낮았으며, 낮은 병기(병기 III), 배아세포형, 항암 약물 요법 병용군에서 상대적으로 높은 생존율이 관찰되었다.

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Clinical Assessment of Intracranial Mesenchymal Tumors with Relevant to Non-meningothelial Tumors (두개강내 원발성 간엽성 종양에 대한 임상 고찰)

  • Yi, Hyeong Joong;Kim, Choong Hyun;Bak, Koang Hum;Kim, Young Soo;Kim, Jae Min;Ko, Yong;Oh, Suck Jun;Kim, Kwang Myung
    • Journal of Korean Neurosurgical Society
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    • v.29 no.1
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    • pp.44-50
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    • 2000
  • Objective : A few anecdotal cases of mesenchyme-derived tumors which grow into a cranial cavity have been reported that disclosed a dismal prognosis, due to their critical location, aggressive biological behavior, and high rate of surgical morbidity. The aim of this study is to search clinical factors related to these tumors. Methods : Eight patients who underwent surgical removal of intracranial mesenchymal tumors between January 1993 and December 1997 were studied retrospectively. The tumors included are three chordomas, two chondrosarcomas, two rhabdomyosarcomas, and one hemangiopericytoma. Authors compared clinical features, treatment, and results of our cases with reported cases. The mean follow-up period was 20.5 months. Results : All cases showed nonspecific, location-related clinical findings and arose from sphenopetroclival region. Single stage operation was performed in 4 cases, and skull base approaches in 3 cases. Adjuvant therapies were done in 2 cases. Recurrence was seen in 3 cases(37.5%), and 3 patients died. Interdisciplinary approach with otologic surgeon was done in 2 cases. Conclusion : Recent advancement of refined tactics has made these tumors amenable and provides prolongation of progression-free survival. These are modified skull base approaches, multi-modality treatment options, and inter-disciplinary team approaches. Good results may be expected for these mesenchymal tumors by aggressive resection and adjuvant therapies according to their biological nature.

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Treatment Outcome and Predictors of Survival in Thai Adult Rhabdomyosarcoma Cases

  • Sookprasert, Aumkhae;Ungareewittaya, Piti;Manotepitipongse, Areesa;Wirasorn, Kosin;Chindaprasirt, Jarin
    • Asian Pacific Journal of Cancer Prevention
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    • v.17 no.3
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    • pp.1449-1452
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    • 2016
  • Background: Adult rhabdomyosarcoma (RMS) is a rare and aggressive disease with limited data compared with pediatric RMS. The goal of this study was to determine the treatment outcome and identify factors related to survival outcome in Thailand. Materials and Methods: Adult patients (${\geq}15$ years old) with the pathological diagnosis of RMS between 1985 and 2010 were reviewed. The data were retrospectively reviewed from the pathological results and medical records from Srinagarind Hospital, Khon Kaen University. Results: A total of 34 patients were identified in the study. The median age at diagnosis was 35.5 years and the most common primary sites were extremity and the head and neck region. The incidence of pleomorphic RMS increased with age and none was found in those aged younger than 20 years old. The median survival time was 9.33 months (95%CI: 5.6-13.1). The 1- and 5- year survival rates were 38.2% (22.3-54.0) and 20.6% (9.1-35.3). On multivariate analysis, age and size of tumor did not predict better outcome while chemotherapy and surgery were significantly associated with longer survival. Conclusions: Outcome of adult RMS was poor. Surgery and chemotherapy are strongly associated with better prognosis and multimodality treatment should be incorporated in the clinic.

Compound HRAS/PIK3CA Mutations in Chinese Patients with Alveolar Rhabdomyosarcomas

  • Liu, Chun-Xia;Li, Xiao-Ying;Li, Cheng-Fang;Chen, Yun-Zhao;Cui, Xiao-Bin;Hu, Jian-Ming;Li, Feng
    • Asian Pacific Journal of Cancer Prevention
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    • v.15 no.4
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    • pp.1771-1774
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    • 2014
  • The rhabdomyosarcoma (RMS) is the most common type of soft tissue tumor in children and adolescents; yet only a few screens for oncogenic mutations have been conducted for RMS. To identify novel mutations and potential therapeutic targets, we conducted a high-throughput Sequenom mass spectrometry-based analysis of 238 known mutations in 19 oncogenes in 17 primary formalin-fixed paraffin-embedded RMS tissue samples and two RMS cell lines. Mutations were detected in 31.6% (6 of 19) of the RMS specimens. Specifically, mutations in the NRAS gene were found in 27.3% (3 of 11) of embryonal RMS cases, while mutations in NRAS, HRAS, and PIK3CA genes were identified in 37.5% (3 of 8) of alveolar RMS (ARMS) cases; moreover, PIK3CA mutations were found in 25% (2 of 8) of ARMS specimens. The results demonstrate that tumor profiling in archival tissue samples is a useful tool for identifying diagnostic markers and potential therapeutic targets and suggests that these HRAS/ PIK3CA mutations play a critical role in the genesis of RMS.

Paratesticular Alveolar Rhabdomyosarcoma with Multiple Lymph Nodes Metastasis Successfully Treated with Chemotherapy (전이성 고환주위 폐포성 횡문근육종 1예)

  • Lee, Ha-Young;Hyun, Myung-Soo;Lee, Kyung-Hee;Kim, Min-Kyoung;Koh, Sung-Ae;Sohn, Se-Hoon;Park, Sung-Woo;Kim, Dong-Geun;Kim, Myung-Jin;Jang, Hyo-Jin;Kim, Mi-Jin
    • Journal of Yeungnam Medical Science
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    • v.28 no.1
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    • pp.70-76
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    • 2011
  • Rhabdomyosarcomas are soft tissue sarcomas; while extremely rare in adults, they are one of the most common neoplasms in children and adolescents. Histologically, they can be classified into embryonal (ERMS), alveolar (ARMS), pleomorphic, and undifferentiated types. The ARMS type is very rare, and is associated with a poor prognosis. Common primary sites of ARMS are the trunk and extremities. We report on a case of paraaortic, supraclavicular, and axillary lymph node metastasis from paratesticular ARMS treated with VAC (vincristine, dactinomycin, cyclophosphamide)/ IE(ifosfamide, etoposide) chemotherapy in a young adult. Administration of six cycles of chemotherapy with VAC/IE resulted in complete remission. The patient has maintained complete remission over the past 27 months.

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