• 제목/요약/키워드: undifferentiated sarcoma

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New established cell lines from undifferentiated pleomorphic sarcoma for in vivo study

  • Eun-Young Lee;Young-Ho Kim;Md Abu Rayhan;Hyun Guy Kang;June Hyuk Kim;Jong Woong Park;Seog-Yun Park;So Hee Lee;Hye Jin You
    • BMB Reports
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    • 제56권4호
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    • pp.258-264
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    • 2023
  • As a high-grade soft-tissue sarcoma (STS), undifferentiated pleomorphic sarcoma (UPS) is highly recurrent and malignant. UPS is categorized as a tumor of uncertain differentiation and has few options for treatment due to its lack of targetable genetic alterations. There are also few cell lines that provide a representative model for UPS, leading to a dearth of experimental research. Here, we established and characterized new cell lines derived from two recurrent UPS tissues. Cells were obtained from UPS tissues by mincing, followed by extraction or dissociation using enzymes and culture in a standard culture environment. Cells were maintained for several months without artificial treatment, and some cell clones were found to be tumorigenic in an immunodeficient mouse model. Interestingly, some cells formed tumors in vivo when injected after aggregation in a non-adherent culture system for 24 h. The tissues from in vivo study and tissues from patients shared common histological characteristics. Pathways related to the cell cycle, such as DNA replication, were enriched in both cell clones. Pathways related to cell-cell adhesion and cell-cell signaling were also enriched, suggesting a role of the mesenchymal-to-epithelial transition for tumorigenicity in vivo. These new UPS cell lines may facilitate research to identify therapeutic strategies for UPS.

장중첩증으로 발현된 기관지 내 전이를 동반한 소장의 미분화성 다형성 육종: 증례 보고 (Undifferentiated Pleomorphic Sarcoma of the Small Intestine with Distant Endobronchial Metastasis Presenting as Intussusception: A Case Report)

  • 송성오;김민선;이경희;최석진
    • 대한영상의학회지
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    • 제82권5호
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    • pp.1304-1309
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    • 2021
  • 미분화성 다형성 육종은 중간엽조직에서 기원하는 고등급의 연조직 육종이다. 소장에서 기원한 미분화성 다형성 육종은 매우 드물며 산발적인 증례 보고가 대부분이다. 소장 미분화성 다형성 육종의 증상은 대개는 비특이적이며, 일부에서는 장중첩증으로 발현되어 간헐적 복통을 일으키기도 한다. 미분화성 다형성 육종은 공격적인 성장을 보이는 종양으로 진단 당시에 원격 전이를 동반한 경우가 많다. 가장 흔한 원격 전이 부위는 복막이며, 그 외에도 임파선, 복강 내 고형 장기, 폐나 뇌 등으로 전이할 수 있다. 저자들이 아는 한도에서는, 기관지 내 전이는 현재까지 보고된 바 없다. 저자들은 장중첩증으로 발현한 소장의 미분화성 다형성 육종이 기관지 내로 전이한 드문 증례를 경험하였기에 이를 보고하고자 한다.

좌심방에 발생한 원발성 미분류 방추세포 육종 1례 (A Case of Primary Unclassified Spindle Cell Sarcoma of the Left Atrium)

  • 조정환;홍그루;송인욱;손창우;정선영;남종호;김미진
    • Journal of Yeungnam Medical Science
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    • 제26권2호
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    • pp.108-113
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    • 2009
  • Primary cardiac tumors are very uncommon. Seventy five percent of them are benign tumors and these are mostly myxomas. The malignant cardiac tumors, the majority of which are undifferentiated sarcomas, comprise up to 25% of all cardiac tumors. A primary malignant sarcoma of the myocardium is exceedingly rare. Thus, there have been very few such cases reported in the literature. We present here a case of a 15 year old man who had complaints of orthopnea and increasing exercise intolerance over a one month period. Transthoracic echocardiography demonstrated a well demarcated huge mass with left ventricular inflow obstruction on the posterior wall of the left atrium. The patient's symptoms were relieved by surgery. The histological diagnosis was an unclassified spindle cell sarcoma.

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Primary Chest Wall Sarcoma: Surgical Outcomes and Prognostic Factors

  • Park, Ilkun;Shin, Sumin;Kim, Hong Kwan;Choi, Yong Soo;Kim, Jhingook;Zo, Jae Ill;Shim, Young Mog;Cho, Jong Ho
    • Journal of Chest Surgery
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    • 제52권5호
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    • pp.360-367
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    • 2019
  • Background: Primary chest wall sarcoma is a rare disease with limited reports of surgical resection. Methods: This retrospective review included 41 patients with primary chest wall sarcoma who underwent chest wall resection and reconstruction from 2001 to 2015. The clinical, histologic, and surgical variables were collected and analyzed by univariate and multivariate Cox regression analyses for overall survival (OS) and recurrence-free survival (RFS). Results: The OS rates at 5 and 10 years were 73% and 61%, respectively. The RFS rate at 10 years was 57.1%. Multivariate Cox regression analysis revealed old age (hazard ratio [HR], 5.16; 95% confidence interval [CI], 1.71-15.48) as a significant risk factor for death. A surgical resection margin distance of less than 1.5 cm (HR, 15.759; 95% CI, 1.78-139.46) and histologic grade III (HR, 28.36; 95% CI, 2.76-290.87) were independent risk factors for recurrence. Conclusion: Long-term OS and RFS after the surgical resection of primary chest wall sarcoma were clinically acceptable.

소아악성고형종의 진단에 있어서 chimeric transcript의 유용성 (Usefulness of Chimeric Transcript in the Diagnosis of Pediatric Solid Tumors)

  • 최승훈
    • Advances in pediatric surgery
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    • 제5권1호
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    • pp.45-52
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    • 1999
  • Pediatric solid tumors have many histologic similarity. These tumors contained small round cell types, and cause frequent diagnostic problems in pediatric pathology. An important advance in the differentiation of these small round cell tumors has been the identification of consistent chromosomal translocations associated with several types of tumors. Eighteen patients with soft tissue sarcoma were available for review. Seventeen cell lines were also included in this study. The RNA from the specimens were analyzed by reverse transcriptase-polymerase chain reaction (RT-PCR). PAX3-FKHR fusion was present in four of five alveolar rhabdomyosarcoma and PAX7-FKHR fusion was detected in one of five alveolar rhabdomyosarcoma. None of the specimens expressed more than one chimeric transcript. EWS-FLI1 or EWS-ERG fusions were detected in all seven Ewings' sarcoma. No specimens showed EWS-WT1 fusion. These results corresponded well to the histopathologic diagnosis. There were no differences in the histologic appearances of tumors with the more frequent PAX3-FKHR or EWS-FLI1 fusions compared with those containing the variant PAX7-FKHR or EWS-ERG fusions. RT-PCR assay for chimeric transcript is a useful tool for rapid and objective diagnosis of pediatric solid tumors. Through these tools, we can approach genetically to the differential diagnosis of undifferentiated small round tumors.

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

  • 김대영;황준하;박인서;임재열
    • 대한후두음성언어의학회지
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    • 제27권2호
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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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18F-FDG PET/CT를 통해 진단된 주폐동맥 협착 소견의 폐동맥 육종 (Pulmonary artery sarcoma manifesting as a main pulmonary artery stenosis diagnosed by 18F-FDG PET/CT)

  • 이훈희;박한빛;조윤경;안정민;이상민;이재승;김대희
    • Journal of Yeungnam Medical Science
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    • 제34권2호
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    • pp.279-284
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    • 2017
  • Pulmonary artery sarcoma (PAS) is a rare and fatal disease that often mimics chronic thromboembolic pulmonary hypertension (CTEPH); therefore, diagnosis of PAS is often delayed. Herein, a healthy 74-year-old man was presented with a 4-month history of dyspnea. Chest computed tomography showed wall thickening and stenosis in the main pulmonary artery as well as in both proximal pulmonary arteries. In order to differentiate between unusual CTEPH, vasculitis, and PAS, we performed right heart catheterization and pulmonary angiography. The mean pulmonary arterial pressure was 21 mmHg, and there was severe pulmonary artery stenosis. Thrombi on the pulmonary arterial wall lesions were observed in intravascular ultrasound and optical coherence tomography. Furthermore, the patient had a history of deep vein thrombosis. Therefore, we diagnosed unusual CTEPH. After 6 months of rivaroxaban anticoagulation therapy, a chest X-ray revealed a left lower lobe lung mass, and a positron emission tomography later showed hypermetabolic lesions in the main pulmonary artery wall, in both pulmonary arteries walls, in the lung parenchyma, and in the bones. A biopsy of the right proximal humerus lesion revealed undifferentiated intimal sarcoma. Pulmonary sarcoma is rare, but should be considered when differentially diagnosing main pulmonary artery wall thickening and stenosis. A positron emission tomography may aid in this diagnosis.

Uterine Sarcoma: Clinical Presentation, Treatment and Survival Outcomes in Thailand

  • Potikul, Chalermrat;Tangjitgamol, Siriwan;Khunnarong, Jakkapan;Srijaipracharoen, Sunamchok;Thavaramara, Thaovalai;Pataradool, Kamol
    • Asian Pacific Journal of Cancer Prevention
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    • 제17권4호
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    • pp.1759-1767
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    • 2016
  • Background: Uterine sarcoma is a group of rare gynecologic tumors with various natures, and different lines of treatment. Most have a poor treatment outcome. This study targeted clinical characteristics, treatment, overall survival (OS), progression-free survival (PFS), and prognostic factors in uterine sarcoma patients in one tertiary center for cancer care. Materials and Methods: Uterine sarcoma patients who were treated at the Department of Obstetrics and Gynecology, Faculty of Medicine Vajira Hospital between January 1994 and December 2014 were identified. Clinico-pathological data were analyzed. Prognostic outcomes were examined by Kaplan-Meier curves and Cox regression analysis. Results: We identified 46 uterine sarcoma patients: 25 carcinosarcoma (CS) (54.3%), 15 leiomyosarcoma (LMS) (32.6%), and 6 undifferentiated uterine sarcoma (UUS) (13.1%) cases. Mean age was $54.0{\pm}11.9years$ (range 25-82 years). Abnormal uterine bleeding was the most common presenting symptom (63.0%). Among 33 patients (71.7%) who had pre-operative tissue collected, diagnosis of malignancy was correct in 29 (87.9%). All patients received primary surgery and retroperitoneal lymph nodes were resected in 34 (73.9%). After surgery, 5 (10.9%) had gross residual tumors. Stage I disease was most commonly found (56.5%). Adjuvant treatment was given to 27 (58.7%), most commonly chemotherapy. After a median follow-up of 16.0 months (range 0.8-187.4 months), recurrence was encountered in 22 patients (47.8%). Median time to recurrence was 5.8 months (range1.0-105.5 months). Distant metastasis was more common than local or locoregional failure. The 2-year PFS was 45.2% (95% confidence interval [CI], 30.6%-59.7%) and the 2-year OS was 48.3% (95% CI, 33.3%-60.7%). Multivariable analyses found residual disease after surgery as a significant factor only for PFS. Conclusions: Uterine sarcoma is a rare tumor entity. Even with multimodalities of treatment, the prognosis is still poor. Successful cytoreductive surgery is a key factor for a good survival outcome.

Long-Term Treatment Results in Soft Tissue Sarcomas of the Thoracic Wall Treated with Pre-or-Postoperative Radiotherapy - a Single Institution Experience

  • Oksuz, Didem Colpan;Ozdemir, Sevim;Kaydihan, Nuri;Dervisoglu, Sergulen;Hiz, Murat;Tuzun, Hasan;Mandel, Nil Molinas;Koca, Sedat;Dincbas, Fazilet Oner
    • Asian Pacific Journal of Cancer Prevention
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    • 제15권22호
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    • pp.9949-9953
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    • 2014
  • Objective: To evaluate the long term results among patients with soft tissue sarcoma of the thoracic wall. Materials and Methods: Twenty-six patients who were treated with pre-or postoperative radiotherapy between December 1980-December 2007, with a diagnosis of soft tissue sarcoma of the thoracic wall were retrospectively evaluated. Results: The median age was 44 years (14-85 years) and 15 of them were male. A total of 50% of patients were grade 3. The most common histologic type of tumor was undifferentiated pleomorphic sarcoma (26.9%). Tumor size varied between 2-25 cm (median 6.5 cm). Seventeen of the cases had marginal and 9 had wide local resection. Four cases received preoperative radiotherapy and 22 postoperative radiotherapy. Six of the patients with large and high grade tumors received chemotherapy. Median follow-up time was 82 months (9-309 months). Local recurrence and metastasis was detected in 34.6% and 42.3% of patients, respectively. Five-year local control (LC), disease-free survival (DFS), overall survival (OS), and disease-specific survival (DSS) were 62%, 38%, 69%, and 76% respectively. On univariate analysis, the patients with positive surgical margins had a markedly lower 5-year LC rate than patients with negative surgical margin, but the difference was not significant (43% vs 78%, p=0.1). Five-year DFS (66% vs 17%) and DSS (92% vs 60%) rates were significantly worse for the patients who had high grade tumors (p=0.01, p=0.008 respectively). Conclusions: Tumor grade and surgical margin are essential parameters for determining the prognosis of thoracic wall soft tissue sarcoma both in our series and the literature.