• Investigative Magnetic Resonance Imaging
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• 제23권2호
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• pp.162-166
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• 2019

Undifferentiated pleomorphic sarcoma (UPS) arising from the descending thoracic aorta is a rare type of tumor. To our knowledge, only a few cases have been reported in the literature. We present computed tomography (CT) and magnetic resonance imaging findings of a 43-year-old male patient with undifferentiated pleomorphic sarcoma of the descending thoracic aorta, which showed enhancement on only magnetic resonance imaging (MRI). MRI with contrast enhancement may be useful in differentiating an aortic tumor from atherosclerotic disease.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• 제46권4호
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• pp.282-287
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• 2020

Undifferentiated pleomorphic sarcoma (UPS) is a high-grade neoplasm that is usually located in the extremities and retroperitoneum. In the past, UPS was considered the most common soft tissue sarcoma in adults; due to improvements in diagnostic techniques, most cases have been reclassified as other lineage-specific tumors. Gnathic bones are rarely affected, and the clinicopathological characteristics of this neoplasm when diagnosed in the jaw remain to be better described. In this report, we present a rare case of mandibular UPS affecting an 88-year-old female who demonstrated a painful swelling on the right side of the mandible that was accompanied by a pathological fracture. Microscopic examination revealed a pleomorphic spindle-cell neoplasm with mitotic figures and necrosis. The patient underwent surgery and adjuvant radiotherapy but experienced metastasis after 12 months of follow-up and died. Diagnosis of UPS is challenging, and oral pathologists must be aware of this entity when dealing with aggressive undifferentiated neoplasms.

• Maxillofacial Plastic and Reconstructive Surgery
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• 제36권6호
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• pp.303-307
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• 2014

Undifferentiated pleomorphic sarcoma (UPS), previously known as malignant fibrous histiocytoma, occurs commonly in the soft tissues in adult, but is rare in the maxillofacial region. It consists of undifferentiated mesenchymal tumor cells resembling histiocytes and fibroblasts. The purpose of this article is to report a case of UPS in the mandible. A 44-year-old patient presented with a painful growing mass in the mandible of two months' duration. Computed tomography and positron emission tomography-computed tomography revealed an ill-defined heterogenous, hypermetabolic mass about 4 cm in size in the left mandible invading adjacent soft tissues. A left mandiblulectomy and reconstruction with a fibular free flap were performed. Immunohistochemical study gave a diagnosis of UPS. The patient was referred for adjuvant chemotherapy after surgical removal of the tumor.

• 대한영상의학회지
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• 제81권5호
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• pp.1204-1209
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• 2020

흉부 대동맥 미분화성 다형성 육종은 매우 드문 질환으로 색전 사례, 신혈관성 고혈압, 등 통증 등의 증상을 보일 수 있다. 특히 대동맥 벽에서 기원하여 소낭 동맥류 파열로 보고된 경우는 극히 드물며 진균성 동맥류나 관통성 죽상경화 궤양과 감별이 어렵다. 이에 저자들은 소낭성 동맥류 파열로 혈관 내 흉부 대동맥 치료를 시행한 후 종괴가 발생하여 수술적 생검으로 확진된 미분화성 다형성 육종의 전산화단층촬영과 양전자방출단층촬영 소견을 보고하고자 하며 이를 통해 정확한 진단과 적절한 치료에 도움을 주고자 한다.

• 대한구강악안면병리학회지
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• 제42권5호
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• pp.135-143
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• 2018

Undifferentiated pleomorphic sarcoma (UPS) in the mandible region is a rare malignant neoplasm. We encountered the case of a patient who had been diagnosed with deep benign fibrous histiocytoma (DBFH) prior to surgery, but the final biopsy revealed the presence of tumor tissues in the resected margin, which indicated a UPS. Therefore, radiotherapy was conducted post-operatively, but the tumor recurred approximately 3 years later and a second surgery was performed. The difference between the initial and final diagnoses was considered to be due to the characteristics of the tumor, as it involved both benign and malignant components. DBFH and UPS can occur in young women. DBFH can transform to a malignancy and UPS may also appear as a benign tumor during early biopsy. Hence, attention should be paid to diagnosis and treatment methods, and ongoing close observation is required.

• 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.

• 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.

• 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.

• 한국수의병리학회:학술대회논문집
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• 한국수의병리학회 2003년도 추계학술대회초록집
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• pp.54-54
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• 2003

Malignant fibrous histiocytomas (MFHs) is the most common type of soft tissue sarcoma in the old animal with a aggressiveness, a high local recurrence rate and significant metastatic rate, which associated with a poor prognosis. In most histologic and immunohistological studies, the tumor cells raised from a fibroblastic and/or myofibroblastic phenotype, presumably from undifferentiated mesenchymal cell origin. MFHs are usually firm and invasive, arising in the subcutis; metastasis depends on tumor grade (many are grade 3) [1,2]. The primary tumor cells are pleomorphic, varying in appearance from fusiform to round. Often nucleoli are prominent and irregular [5]. Extracellular amorphous eosinophilic material may be prominent and likely represents reactive collagen production by the tumor [5]. (omitted)

• 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.