• Title/Summary/Keyword: rhabdomyosarcoma

Search Result 87, Processing Time 0.021 seconds

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
    • /
    • v.6 no.4
    • /
    • pp.173-177
    • /
    • 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.

  • PDF

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
    • /
    • v.15 no.2
    • /
    • pp.1011-1014
    • /
    • 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.

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

  • Son, Hwa-young;Kang, Boo-hyon;Han, Sang-seop;Jun, Moo-hyung;Cho, Sung-whan
    • Korean Journal of Veterinary Research
    • /
    • v.32 no.4
    • /
    • pp.609-627
    • /
    • 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.

  • PDF

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

  • Kim, Jisung;Yeon, Je Yeob;Park, Jae-Hong;Lee, Yong-Moon;Lee, Dong Wook
    • Korean Journal of Head & Neck Oncology
    • /
    • v.29 no.2
    • /
    • pp.75-78
    • /
    • 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.

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

  • Lee, Jong-Seok;Jeon, Dae-Geun;Lee, Soo-Yong;Kim, Sug-Jun;Jung, Dong-Whan;Park, Hyun-Soo
    • The Journal of the Korean bone and joint tumor society
    • /
    • v.3 no.2
    • /
    • pp.89-97
    • /
    • 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.

  • PDF

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

  • Im, Ra-Joo;Kim, Hae-Sol;Kim, Tae-Seok;Lee, Cheol-Gu;Seo, Jeong-Meen;Lee, Suk-Koo
    • Advances in pediatric surgery
    • /
    • v.13 no.2
    • /
    • pp.155-161
    • /
    • 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.

  • PDF

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

  • Chung, Eun-Ji;Suh, Chang-Ok;Kim, Gwi-Eon;Lyu, Chuhl-Joo;Kim, Byung-Soo
    • Radiation Oncology Journal
    • /
    • v.13 no.3
    • /
    • pp.267-275
    • /
    • 1995
  • Radiotherapy and chemotherapy can effectively control cancer but can also cause new second cancers to develop as long-term complications especially in childhood cancer. We experienced two patients with second malignant solid neoplasm who had been treated with radiation and chemotherapy for childhood cancers One female patient with rhabdomyosarcoma of the right popliteal fossa was treated with radiotherapy at total dose of 54Gy Three years and seven months later, osteosarcoma developed in the field of radiation therapy The other male patient with non-Hodgkin's lymphoma of the small bowel was treated with radiotherapy and leiomyosarcoma developed in the field of radiotherapy 18 years later. We reviewed the literature of the second malignant neoplasm in children in respect of risk factors. The risk for a second primary cancer following radiotherapy or chemotherapy emphasizes the need for life long follow-up of patients receiving such treatments, particularly patients treated for childhood cancers.

  • PDF

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
    • /
    • v.44 no.3
    • /
    • pp.435-441
    • /
    • 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.

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

  • Bahk, Won-Jong;Chang, Ju-Hai;Kang, Yong-Koo;Song, Seok-Whan;Moon, Myung-Sang;Kim, Jung-Man;Woo, Young-Kyun;Lee, Seung-Koo;Kim, Hyoung-Min;Kim, Yun-Sil;Chang, Jee-Young;Yoon, Se-Chul
    • The Journal of the Korean bone and joint tumor society
    • /
    • v.1 no.1
    • /
    • pp.84-90
    • /
    • 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.

  • PDF

CASE REPORT OF PILOMAIRICOMA (Pilomatricoma 환아에 대한 증례 보고)

  • Seo, Hyun-Woo;Kim, Ji-Hyuck;Park, Ho-Won
    • Journal of the korean academy of Pediatric Dentistry
    • /
    • v.31 no.1
    • /
    • pp.79-84
    • /
    • 2004
  • Pilomatricoma is a benign soft tissue tumor of hair follicle origin. They occur most commonly in the head and neck region and are usually found in girls during the first two decades of life. Although malignant transformation has been described, it is exceedingly rare. The clinical presentation is typically that of an asymptomatic, superfical, solitary, firm mass that is often accompanied by a reddish-blue discoloration of the overlying skin. Histopathologically pilomatricoma are seen as epithelial islands embedded in a cellular stroma. The epithelial component consist of two main cell types : basaloid cells and ghost cells. Intracellular and stromal calcification is reported in many cases. Diagnosis is usually suspected based on palpation of a superficial, rock-hard mass and confirmed by histopathologic examination. Surgical excision is both curative and the treatment of choice. Recurrence is rare. In this case, the radiographs showed a dense calcification, measuring about 1cm diameter. So simple excision was peformed intraorally, and resultantly the lesion was proved to be a pilomatricoma by histological examination.

  • PDF