• 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.

• Asian Pacific Journal of Cancer Prevention
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• 제14권5호
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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.

• Journal of Chest Surgery
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• 제30권12호
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• pp.1214-1218
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• 1997

폐전이 골 연부조직 육종에 대한 폐절제 및 합병치료의 성적을 분석하였다. 1986년부터 1996년까지 14례 (남자 11례, 여자 3례)에서 15회의 폐절제술을 시행하였는데, 1례에서는 폐전이 육종의 재발로 2회의 폐절제술을 시행하였다. 절제된 폐전이 육종의 수는 1∼5개였다. 폐전이 육종의 병리조직학적 소견상 4례는 골육종 이었으며, 10례는 연부조직 육종이었다 폐절제술후 평균 생존기간은 29.2개월이었으며, Kaplan-Meier식에 의 한 5년생존율은 33.2%였다. 14례중 3례의 종양 자유기간이 3년이상으로 평균 생존기간은 52.6개월이었으며, 3년 이하의 11례는 추적기간중 모두 사망하여 이들의 평균 생존기간은 17.3개월이었다. 결론적으로 폐전이 육종에 대한 폐절제술은 뚜렷한 수명 연장 효과가 있을 것으로 생각된다.

• 대한골관절종양학회지
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• 제8권3호
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• pp.69-75
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• 2002

워너 증후군은 상염색체 열성 유전을 하는 희귀한 성인 조로 질환이다. 이 질환은 조기 노화를 보일 뿐만 아니라, 악성 종양의 발생 빈도도 증가하게 되는데, 골연부 조직의 종양이 많이 발생하게 된다. 그러나 악성 종양의 발생 원인은 단순한 조기 노화 현상으로만 보기보다는 하나의 종양 증후군으로 보는 경향이 있으며, 그 이유는 워너 증후군 환자에서 발생하는 종양의 위치, 병리적 소견, 나이 등에서 정상인과는 많은 차이를 보였기 때문이다. 최근의 분자 유전학적 연구들에 의해서 워너 증후군은 DNA의 복제, 복구, 재생에 관여하는 Werner helicase의 유전자 변이와 관련이 있음이 밝혀졌다. 워너 증후군의 유전자 이상은 이러한 DNA 복구과정에 문제를 일으키고, 유전자의 불안정성을 증가시켜 종양의 발생 가능성을 높이게 된다. 육종의 발생과 워너 증후군의 연관성에 관한 향후의 연구들은 정상적인 노화의 과정과 육종의 발생 기전에 관한 많은 정보를 제공할 수 있을 것으로 사료된다.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제36권3호
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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.

• 대한정형외과학회지
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• 제55권3호
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• pp.271-275
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• 2020

외상은 횡문근육종을 포함한 일부 육종의 발생에 영향을 미친다고 알려져 있고 수술 후 반흔이나 화상 흉터, 골절 부위, 금속 내고정물 주위에서 수년 간의 잠복기를 거쳐 육종을 일으킨 사례들이 드물게 보고되고 있다. 저자들은 컨베이어 벨트에 의해 압궤상을 입은 전완부에 11년 뒤 발생한 횡문근육종의 사례를 경험하였다. 조직 손상으로 인한 염증 반응, 많은 방사선 촬영에 대한 노출, 금속 내고정물, 감각 및 운동 마비로 인한 무의식적이고 반복적인 손상이 육종의 발생에 영향을 미쳤으리라고 생각된다. 이러한 광범위한 조직 손상 후에는 육종 발생 가능성을 염두에 두고 육종 유발 요인에 대한 노출에 주의를 기울여야 하겠다.

• Journal of Chest Surgery
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• 제50권6호
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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.

• Journal of Korean Neurosurgical Society
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• 제29권11호
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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.

• Journal of Korean Neurosurgical Society
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• 제57권6호
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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.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제38권4호
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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.