• 제목/요약/키워드: rhabdomyosarcoma

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

  • 이상훈;김한수;오주한;서성욱;이한구
    • 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.

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Increasing Frequency of Soft Tissue Sarcomas in Vojvodina - Comparison with the Literature

  • Dugandzija, Tihomir;Mikov, Marica Miladinov;Solajic, Nenad;Nikolin, Borislava;Trifunovic, Jasna;Ilic, Maja
    • Asian Pacific Journal of Cancer Prevention
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    • 제15권2호
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    • pp.1011-1014
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    • 2014
  • Background: Soft tissue sarcomas (STS) represent 1% of all malignant lesions. In this study the authors analyzed the incidence of STS in Vojvodina (the north region of Serbia) in the period from 1985 to 2009. A number of studies conducted worldwide indicate that STS incidence rates are tending to increase. Materials and Methods: On the basis of data from the Cancer Registry of Vojvodina, age standardized STS incidence rates were established as well as their linear trend, with data on histological structure, age, gender and STS distribution at specific locations. Results: The total number of registered patients was 1,308. Average age standardized rate was 1.90/100,000 per year. The investigated period showed a slight increase in the incidence rate (average annual percent increase=0.77%). The most frequent histological type was sarcoma not otherwise specified-NOS (27%), followed by leiomyosarcoma (21%), liposarcoma (14%), rhabdomyosarcoma (11%) and malignant fibrous histiocytoma (9%). The male/female ratio was 0.73:1. Every fifth patient was younger than 39. Conclusions: Comparison among eight international STS epidemiology studies show that the incidence rate range is between 1.4/100,000-5.0/100,000, though our finding is closer to the lower limit. Furthermore, the incidence rate increase was lower than that characteristic for the half of the analyzed studies. A partial explanation for that should be looked for among changes in diagnostic criteria and STS classifications.

SPF Ktc : ICR 마우스의 자연발생 종양에 대한 병리학적 연구 (A pathological study of spontaneous tumors in SPF Ktc : ICR mice)

  • 손화영;강부현;한상섭;전무형;조성환
    • 대한수의학회지
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    • 제32권4호
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    • pp.609-627
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    • 1992
  • The study was carried out to obtain the basic data for types, incidence and histopathological features of the spontaneous tumors of the specific pathogen free (SPF) Ktc : ICR mice bred in a barrier system in Technology Research Center, Korea Research Institute of Chemical Technology. One hundred of the mice consisted of 50 males and 50 females were examined for 18 months. 1. The overall incidence rate of spontaneous tumors was 51(51%) of 100 heads tested. The male mice showed slightly higher incidence(28%) than the female(23%), and the incidence rate and the number of affected organs were increased with the increasing age of mice. 2. The incidence rate of primary tumor was 59(59%) of 100 heads tested, consisted of 30 cases (50.8%) of benign tumors and 29 cases(49.2%) of malignant tumors. Among the malignant tumors twenty cases were metastasized to various organs. 3. In tumor incidence rates by systems and organs, the male mice showed the high incidence rate in the liver (18%), hematopoietic system (16% ) and lung (14%), while the female mice, in the hematopoietic system(18%), lung(12%), liver(8%) and uterus(8%). 4. The tumors showing the particularly low incidence rates (<1.0%) were rhabdomyosarcoma in the skeletal muscle, malignant schwannoma in the peripheral nerve, cortical adenoma in the adrenal gland, transitional cell carcinoma in the urinary bladder, tubular cell adenoma in the kidney and adenoma in the pituitary gland and harderian gland.

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영아의 설근부에 발생한 폐포성 횡문근육종 1예 (Alveoloar Rhabdomyosarcoma of Tongue Base in an Infant : A Case Report)

  • 김지성;연제엽;박재홍;이용문;이동욱
    • 대한두경부종양학회지
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    • 제29권2호
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    • pp.75-78
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    • 2013
  • 영아의 설근부에 발생하는 악성 종양은 매우 드물다. 저자들은 연하곤란과 폐쇄성 수면 무호흡증으로 의뢰된 17개월 남아의 설근부에 발생한 횡문근육종을 경험하였기에 문헌고찰과 함께 보고하는 바이다. 외래에서 실시한 이학적 검사 상 설근부 전체가 돌출되어 있었으며 단단한 종괴가 촉지 되었다. 조직검사와 기관절개술을 시행하였으며 컴퓨터단층촬영, 양전자 방출 단층 촬영 그리고 뼈 스캔과 함께 염색체 분석을 실시하였다. 조직검사 결과 폐포성 횡문근육종이 확인되었으며 전이의 증거는 없었다. 염색체 분석상 폐포성 횡문근육종에 상응하는 PAX7-FKHR 유전자 전좌가 발견되었다. 8 회의 항암화학요법과 방사선 치료 후 촉지되는 설근부의 종괴는 없었으며 환자가 호소하는 증상도 개선되었다. 추적 관찰 시 시행된 자기공명영상 결과 확연한 종괴 크기의 감소를 확인할 수 있었다. 횡문근육종은 매우 드문 악성 종양으로 수술과 함께 항암화학요법, 방사선치료 등 여러 치료 방법이 동원되지만 전이나 재발이 있을 시 예후는 매우 불량하다. 그러므로 영아에서 연하곤란, 호흡 곤란 등의 증상이 있을 시에는 설근부를 포함한 상부호흡소화관을 적극적으로 검사하여야 하며 악성 종양의 가능성을 염두에 두어야 하겠다.

연부 육종의 예후 인자 - 205례 분석 - (Prognostic Factors of Soft Tissue Sarcomas - analysis of 205 cases -)

  • 이종석;전대근;이수용;김석준;정동환;박현수
    • 대한골관절종양학회지
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    • 제3권2호
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    • pp.89-97
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    • 1997
  • Twenty hundred and five out of 266 patients who were registered in Korea Cancer Center Hospital from Mar. 1985 to Jan. 1994, were analyzed in the aspect of survival and local recurrence. Fifty one patients were excluded due to inadequate data and follow up. Prognostic factors for survival were evaluated statistically. One hundred and four cases were male, 101 female. Average age was 39.7(range 1 to 77) year with a peak incidence around 4th decade. The most frequent diagnosis was malignant fibrous histiocytoma(MFH)(24.1%). Liposarcoma, synovial sarcoma, rhabdomyosarcoma, malignant peripheral nerve sheath tumor and fibrosarcoma were relatively common diagnostic entities, in decreasing order. In location, extremity was 179(87.3%) and trunk 26(12.7%). Average follow up period was 7.5 years(6 months to 10 years). Actuarial 5 years and 10 years survival rate were 64.0% and 40.8% respectively. In univariate analysis with log-lank test, significant differences in survival rate were noted in histopathological diagnosis, size(10 cm), stage and metastasis. Age, sex, tumor location, tumor depth and local recurrence didn't affect the survival rate. Adjuvant chemotherapy and/or radiotherapy did not affect overall survival rate, but lowered the local recurrence rate when compared with surgery only. Surgical margin did not affect the survival rate, but local recurrence rate was different according to each margin; 5.7% in more than wide; 39.5% in marginal; and 60.0% in intralesional excision. In multivariate analysis for results of univariate analysis with Cox's propotional model, metastasis was a meaningful factor for survival of soft tissue sarcoma.

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소아 췌장종양의 임상적 고찰 (Clinical Experiences of Pancreatic Tumors in Children)

  • 임라주;김해솔;김태석;이철구;서정민;이석구
    • Advances in pediatric surgery
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    • 제13권2호
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    • pp.155-161
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    • 2007
  • Pancreatic tumors in children are very rare but have a better prognosis compared with that in adult. Pediatric pancreatic tumors are more often benign and easier to resect. To evaluate the characteristics and prognosis, the records of 13 patients who underwent pancreatic resection, from June 1997 to May 2005, at Samsung Medical Center were reviewed. The mean follow up period was 48 months. The male to female ratio was 1: 1.6. Mean age was 10.3 years. Signs and symptoms included abdominal pain (7), abdominal palpable mass (5), jaundice (1), hypoglycemic (1), and non-specific GI symptoms (4). The commonly used diagnostic tools were CT and abdominal sonography. In addition, MRI, ERCP, EEG, and hormone test were also done when indicated. Surgical procedures included distal pancreatectomy (5), pylorus preserving pancreaticoduodenectomy (4), tumor excision (3), and subtotal pancreatectomy (1). Locations of lesions in pancreas were head (4), tail (5), and body and tail (4). Postoperative complications developed in 3 cases; postoperative ileus (1), wound problem (1), and pancreatitis (1). The pathologic diagnosis included solid-pseudopapillary tumor (6), congenital simple cyst (1), pancreatic duplication cyst (1), serous oligocystic adenoma (1), mucinous cystadenocarcinoma (1), rhabdomyosarcoma (1), insulinoma (1), and pancreatoblastoma (1). Three cases received adjuvant chemotherapy and radiotherapy. Overall survival rate was 81 %. One patient with a mucinous cystadenocarcinoma died. In this study, pancreatic tumors in children were resectable in all patients and had good survival. Surgery of pancreatic tumors should be regarded as the gold standard of treatment and a good prognosis can be anticipated in most cases of benign and malignant tumors.

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소아암의 방사선치료후 발생한 이차 악성 고형 종양 - 증례보고 및 문헌고찰 - (Second Malignant Solid Neoplasms in Children Treated with Radiotherapy - Report of Two Cases and Review of Literature -)

  • 정은지;서창옥;김귀언;유철주;김병수
    • Radiation Oncology Journal
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    • 제13권3호
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    • pp.267-275
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    • 1995
  • 방사선치료와 항암화학요법 등 암의 치료법이 점차 발전해감에 따라 치료후 장기간 생존하는 환자들이 많아지고 특히 소아암 치료후 장기 생존자가 증가하면서 여러가지 치료로 인한 합병증 및 문제점들이 발생하고 있다. 그중 중요한 하나가 이차암의 발생인데 본과에서 이차 악성 고형 종양 발생 환자 2예를 경험하였기에 보고하고자 한다. 한 예는 우측 슬와부에 발생한 rhab-domyosarcoma group II로 수술후 방사선치료 및 항암화학요법을 시행받았는데 3년7개월 후 방사선치료부위에서 osteosarcoma가 발생하였고 또 다른 한 예는 우측 하복부에 소장 악성 림프종이 발생하여 방사선치료 및 항암화학요법을 시행받았고 18년후에 방사선치료부위에 leimyosarcoma가 발생하였다. 문헌 고찰을 통해 소아암 치료후 이차암 발생의 위험 요인들을 고찰하였고 이차암에 대한 인식 및 세밀한 추적 조사가 필요함을 확인하였다.

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Ginsenoside Rb1 and Rb2 upregulate Akt/mTOR signaling-mediated muscular hypertrophy and myoblast differentiation

  • Go, Ga-Yeon;Jo, Ayoung;Seo, Dong-Wan;Kim, Woo-Young;Kim, Yong Kee;So, Eui-Young;Chen, Qian;Kang, Jong-Sun;Bae, Gyu-Un;Lee, Sang-Jin
    • Journal of Ginseng Research
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    • 제44권3호
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    • pp.435-441
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    • 2020
  • Background: As a process of aging, skeletal muscle mass and function gradually decrease. It is reported that ginsenoside Rb1 and Rb2 play a role as AMP-activated protein kinase activator, resulting in regulating glucose homeostasis, and Rb1 reduces oxidative stress in aged skeletal muscles through activating the phosphatidylinositol 3-kinase/Akt/Nrf2 pathway. We examined the effects of Rb1 and Rb2 on differentiation of the muscle stem cells and myotube formation. Methods: C2C12 myoblasts treated with Rb1 and/or Rb2 were differentiated and induced to myotube formation, followed by immunoblotting for myogenic marker proteins, such as myosin heavy chain, MyoD, and myogenin, or immunostaining for myosin heavy chain or immunoprecipitation analysis for heterodimerization of MyoD/E-proteins. Results: Rb1 and Rb2 enhanced myoblast differentiation through accelerating MyoD/E-protein heterodimerization and increased myotube hypertrophy, accompanied by activation of Akt/mammalian target of rapamycin signaling. In addition, Rb1 and Rb2 induced the MyoD-mediated transdifferentiation of the rhabdomyosarcoma cells into myoblasts. Furthermore, co-treatment with Rb1 and Rb2 had synergistically enhanced myoblast differentiation through Akt activation. Conclusion: Rb1 and Rb2 upregulate myotube growth and myogenic differentiation through activating Akt/mammalian target of rapamycin signaling and inducing myogenic conversion of fibroblasts. Thus, our first finding indicates that Rb1 and Rb2 have strong potential as a helpful remedy to prevent and treat muscle atrophy, such as age-related muscular dystrophy.

연부조직 육종의 수술후 방사선치료 (Postoperative Radiation for Soft Tissue Sarcoma)

  • 박원종;장주해;강용구;송석환;문명상;김정만;우영균;이승구;김형민;김연실;장지영;윤세철
    • 대한골관절종양학회지
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    • 제1권1호
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    • pp.84-90
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    • 1995
  • To evaluate the role of radiation and chemotherapy after limb-saving operation in the management of soft tissue sarcoma, the authors analysed retrospectively 33 patients treated in department of Orthoaepdic Surgery and Radiation Therapy, Catholic University Medical College, in terms of survival rate, local recurrence rate and prognostic factors. There were 16 males and 17 females. The age distribution ranged from 16 to 81 years with mean age of 48. The follow-up period ranged from 2 to 10 years with average of 5.5 years. The histologic diagnoses were 9 liposarcoma(27.2%), 8 malignant fibrohistiocytoma(24.2%), 7 unclassifiable(21.2%), 3 rhabdomyosarcoma(9.1%), 2 malignant schwannoma, 2 synovral sarcoma, and 2 fibrosarcoma(6.1%) in orders. While marginal and intralesional margins were gained in 24 patients(72.7%), wide and radical margins were obtained only in 9 patients(27.3%). On postoperative 3 weeks, local irradiation of 5000-7000 cGy was delivered to all patients by shrinking field technique for 5-8 weeks. Of 33 patients, 16(45.5%) patients were received adjuvant chemotherapy in combination of adriamycin, cyclophosphamide & vicristine, or VP16 & ifosfamide based on histologic type and obtained surgical margin. The survival rates by direct method at 2 years and 5 years were 58% and 37% respectively. Local recurrences occured in 15 patients(45.5%) at average 16 months after operation. Survival rates at 2 years and 5 years were 37% and 22% in case of intralesional and manginal excision, 75% and 47% in case of wide and radical excision respectively with statistical significance(p<0.05). They were 25% and 17% in the presence of local recurrence, 67% and 42% in the absence of local recurrence respectively with statistical significance(p<0.05). Even though there was no statistical correlation between survival rate and tumor size(p>0.05), the authors considered tumor size as a significant prognostic factors as well as surgical margin and the presence of local recurrence.

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Pilomatricoma 환아에 대한 증례 보고 (CASE REPORT OF PILOMAIRICOMA)

  • 서현우;김지혁;박호원
    • 대한소아치과학회지
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    • 제31권1호
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    • pp.79-84
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    • 2004
  • Pilomatricoma(Calcifying epithelioma of Malherbe)는 모낭 세포 기원의 양성 연조직 종양으로, 보통 머리와 목 부위에 나타나며, 20세 이전의 여성들에게서 호발하고, 악성으로의 전이가 보고되기도 하나, 이는 극히 드물다. 임상적으로, 무통성, 표재성, 고형성이며 종종 적청색을 나타내고, 크기는 보통 $0.5{\sim}3.0cm$ 정도로 다양하게 나타난다. 조직학적으로, 세포기질 내에 상피조직이 존재하는 형태이며 상피조직에서는 기저세포(basaloid cell)와 유령세포(ghost cell)가 특징적으로 나타나고, 세포내와 기질내 석회화가 종종 관찰되기도 한다. 진단은, 초진시 고형 물질의 촉진으로 병소를 의심해볼 수 있고, 조직병리학적 검사로 확진할 수 있다. 감별진단으로는 아가미낭(branchial cyst), 혈관종(hemangioma), 횡문근육종(rhabdomyosarcoma), 표피포함낭(epidermal inclusion cyst), 모낭(pilar cyst), 이하선 종양(parotid gland tumor), 피부의 골종(osteomas of the skin), 림프절의 석회화(calcified lymph nodes), 죽종(atheromas) 등이 있다. 치료로는 외과적 절제술이 추천되며 재발은 드물다. 본 증례는 우측 하악각 부위에 결절이 촉진되는 6세 여자 환아에게서 임상 검사와 방사선 검사 후 병소의 외과적 절제술을 시행하였으며, 조직병리학적 검사를 통해 pilomatricoma로 확진되었고, 양호한 치료 결과를 얻었기에 이에 보고하는 바이다.

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