• Title/Summary/Keyword: Sarcomas

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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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    • v.15 no.2
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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.

Extraskeletal Ewing Sarcomas in Late Adolescence and Adults: A Study of 37 Patients

  • Tao, Hai-Tao;Hu, Yi;Wang, Jin-Liang;Cheng, Yao;Zhang, Xin;Wang, Huan;Zhang, Su-Jie
    • Asian Pacific Journal of Cancer Prevention
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    • v.14 no.5
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    • pp.2967-2971
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    • 2013
  • Background: Extraskeletal Ewing sarcoma (EES)/primitive neuroectodermal tumours (PNET) are rare soft tissue sarcomas. Prognostic factors and optimal therapy are still unconfirmed. Materials and Methods: We performed a retrospective analysis on patients to explore the clinic characteristics and prognostic factors of this rare disease. A total of 37 patients older than 15 years referred to our institute from Jan., 2002 to Jan., 2012 were reviewed. The characteristics, treatment and outcome were collected and analyzed. Results: The median age was 28 years (range 15-65); the median size of primary tumours was 8.2 cm (range 2-19). Sixteen patients (43%) had metastatic disease at the initial presentation. Wide surgical margins were achieved in 14 cases (38%). Anthracycline or platinum-based chemotherapy was performed on 29 patients (74%). Radiotherapy was delivered in 13 (35%). At a median follow-up visit of 24 months (range 2-81), the media event-free survival (EFS) and overall survival (OS) were 15.8 and 30.2 months, respectively. The 3-year EFS and OS rates were 24% and 43%, respectively. Metastases at presentation and wide surgical margins were significantly associated with OS and EFS. Tumour size was significantly associated with OS but not EFS. There were no significant differences between anthracycline and platinum based chemotherapy regarding EFS and OS. Conclusions: EES/PNET is a malignant tumour with high recurrence and frequent distant metastasis. Multimodality therapy featuring wide surgical margins, aggressive chemotherapy and adjuvant local radiotherapy is necessary for this rare disease. Platinum-based chemotherapy can be used as an adjuvant therapy.

Surgical Treatment for Metastatic Pulmonary Sarcoma (폐전이 골 연부조직 육종에 대한 외과적 치료)

  • 박재길;이선희
    • Journal of Chest Surgery
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    • v.30 no.12
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    • pp.1214-1218
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    • 1997
  • The therapeutic results of pulmonary resection for metastatic bone and soft tissue sarcomas were analyzed. From 1986 to 1996, 14 patients(11 male and 3 female) underwent 15 pulmonary resections for metastatic sarcomas. One(7.1%) patient had 2 thoracotomies for recurrences. The number of metastatic tumors were from one to five. The primary malignant tumors were from bone in 4 and from soft tissues in 10. Mean survival time after thoracotomy was 29.2 months, and Kaplan-Meier's 5-year survival rate from the first metastasectomy was 33.2%. Three patients who had the tumor free interval period over 3 years were alive(mean survival period 52.6 months), whereas eleven patients of the less than 3 years were dead with disease(mean survival period 17.3 months). These results suggested that pulmonary. metastasectomy in bone and soft tissue sarcoma may prolong the survival rate.

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The Musculoskeletal Tumors of Werner's Syndrome (워너 증후군에 발생하는 근골격계 종양)

  • Lee, Sang-Hoon;Suh, Sung-Wook;Yoo, Kwang-Hyun;Kim, Han-Soo;Ishikawa, Yuichi;Goto, Makoto
    • The Journal of the Korean bone and joint tumor society
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    • v.8 no.3
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    • pp.69-75
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    • 2002
  • Werner's syndrome is a rare autosomal recessive disorder manifesting as premature aging. It is also known to be characterized by a high frequency of malignant tumors, especially sarcomas. However, Werner's syndrome may be not only a premature aging disease but also a cancer syndrome, because the malignant tumors in these patients are different from those of normal population with respect to involved site, histological type, and age of onset. Recent studies found Werner's syndrome was caused by a mutation of Werner helicase suggesting that WRN helicase may participate in metabolism and repair of DNA. And a dysfunction of WRN helicase may induce the genomic instability causing somatic mutations. Further studies of Werner's syndrome associated with sarcoma might give much informations about the normal aging process and the pathogenesis of sarcomas.

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Epithelial-mesenchymal transition in osteogenic sarcoma of the neck following oral squamous cell carcinoma (구강 편평세포암종 제거 후 발생한 경부 골육종에서의 상피간엽이행)

  • Kim, Hyun-Sil;Kim, Nam-Hee;Han, Seon-Hui;Cha, In-Ho;Seo, Dong-Jun;Park, Won-Se;Yook, Jong-In;Kim, Hyung-Jun
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • v.36 no.3
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    • pp.172-176
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    • 2010
  • Postirradiation extraosseous osteogenic sarcomas are uncommon in the head and neck, despite the extensive use of high-dose radiation. It has been described as de novo radiation-induced neoplasm. We present a 73-year-old male who had been treated by radiotherapy for gingival cancer 7 years earlier and later developed extraosseous osteogenic sarcomas (EOSs) of the neck. Microscopically, the neck mass was composed with mesenchymal malignant cells with cartilaginous and osteogenic differentiation. Immunohistochemical stain demonstrated strong positivity of tumor cells for Snail, the one of major epithelial-mesenchymal transition (EMT) inducer. The E-cadherin expression was scarce, showing inverse relationship to Snail expression. Compared with previous squamous cell carcinoma (SCC) of the gingiva, the present EOS sample revealed the remained epithelial cells on cytokeratin immunohistochemistry, suggesting the tumor arise from the cells of epithelial origin. We have also reviewed the previous 6 cases of head and neck EOSs carefully. The clinicopathologic features of the unusual lesion suggest that it is an incomplete EMT of precedent epithelial malignancy rather than de novo pathology.

Rhabdomyosarcoma Following Traumatic Injury (외상 후 발생한 횡문근육종)

  • Kwon, Dae Gyu;Park, Sung Jun;Yoon, Young Hoon;Lee, Tong Joo
    • Journal of the Korean Orthopaedic Association
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    • v.55 no.3
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    • pp.271-275
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    • 2020
  • Trauma is frequently implicated in the development of sarcomas, including rhabdomyosarcoma. Rare soft tissue sarcomas have been reported to arise in scar tissue following surgical procedures or thermal or acid burns, at fracture sites, and in the vicinity of plastic or metal implants, usually after a latent period of several years. The authors encountered a case of a rhabdomyosarcoma arising from the forearm crushed by a conveyor belt 11 years ago. Several possible etiological factors for sarcoma development were identified in this patient, including tissue damage and inflammation, as well as the presence of metal implants and the limb's exposure to radiation during multiple imaging tests. After severe soft tissue damage, the occurrence of a sarcoma should be considered and more attention should be paid to the causative factors for sarcoma.

Recurrent Mediastinal Sarcoma in the Aortic Arch

  • Pyo, Won Kyung;Kim, Ho Jin;Kim, Joon Bum
    • Journal of Chest Surgery
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    • v.50 no.6
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    • pp.463-466
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    • 2017
  • As mediastinal sarcomas commonly present as large tumors invading adjacent vital structures, complete resection is frequently challenging. For such tumors, aggressive surgical strategies, such as the resection and reconstruction of the invaded vital structures under cardiopulmonary bypass, may be required to achieve complete resection and to improve survival. Herein, we report a case of recurrent mediastinal sarcoma invading the aortic arch and arch vessels that was successfully removed by total arch replacement.

Epidural Granulocytic Sarcoma Causing Cord Compression at Thoracic Region in Acute Myelogenous Leukemia - A Case Report - (급성 골수성 백혈병에서 발생한 흉추부 경막외 과립구육종 - 증례보고 -)

  • Park, Woo-Min;Jang, Jee-Soo;Rhee, Chang-Hun;Gwak, Ho-Shin;Lee, Seung-Hoon
    • Journal of Korean Neurosurgical Society
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    • v.29 no.11
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    • pp.1533-1537
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    • 2000
  • Granulocytic sarcomas are solid tumors resulting from the localized proliferation of myelogenous leukions cells. Epidural involvement of granulocytic sarcoma is very rare in acute myelogenous leukemia(AML). We report a patient with a thoracic epidural granulocytic sarcoma whose presentation with acute paraparesis led to the diagnosis of relapsing of alleged AML. Early recognition of the etiology of the paraparesis and treatment with emergency decompressive, laminectomy, radiation therapy and chemotherapy resulted in an excellent neurological and hematological outcome.

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Intracranial Undifferentiated Sarcoma Arising from a Low-Grade Glioma : A Case Report and Literature Review

  • Kim, Bum-Joon;Kim, Jong-Hyun;Chung, Hung-Seob;Kwon, Taek-Hyun
    • Journal of Korean Neurosurgical Society
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    • v.57 no.6
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    • pp.469-472
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    • 2015
  • Undifferentiated sarcomas are rarely identified in the intracranial region. A 23-year-old man was admitted with a chief complaint of headache. Initial magnetic resonance images showed signs of low-grade glioma in the frontal lobe. Stereotactic biopsy was performed, and a diagnosis of diffuse astrocytoma was confirmed. Three months later, the patient presented with a high-grade tumor as seen on imaging studies. He underwent total resection of the tumor and histopathological tests identified an undifferentiated sarcoma. The patient died eight months later due to massive tumor bleeding. To the best of our knowledge, this is the first report of undifferentiated sarcoma arising from low-grade glioma without any chemotherapy or radiotherapy.

Myofibroblastic sarcoma of the mandible: a case report

  • Park, Kyung-Ran;Jang, Hyo-Won;Won, Ji-Hoon;Kim, Hyun-Sil;Cha, In-Ho;Kim, Hyung-Jun
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • v.38 no.4
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    • pp.240-244
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    • 2012
  • Myofibroblastic sarcoma is a rare tumor that mostly develops in the soft tissues of the head and neck. Within the oral cavity, a tongue lesion is the most common. A myofibroblastic sarcoma tends to recur locally instead of metastasizing. We encountered a myofibroblastic sarcoma of the mandible of a 9-year-old male and performed mass excision and additional marginal alveolectomy. So far, there is neither recurrence nor metastasis. We report this case because of the uncommon location of this tumor type and its surgical approach compared to other forms of sarcomas.