• Title/Summary/Keyword: Undifferentiated pleomorphic sarcoma

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Undifferentiated Pleomorphic Sarcoma of the Descending Thoracic Aorta Mimicking Pseudoaneurysm with Periaortic Hematoma: a Case Report

  • Kim, Minsu;Bae, Young-A;Byeon, Sun-Ju;Choi, Jung-Ah
    • Investigative Magnetic Resonance Imaging
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    • v.23 no.2
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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.

Undifferentiated pleomorphic sarcoma of the mandible

  • Benites, Bernar Monteiro;Miranda-Silva, Wanessa;Fonseca, Felipe Paiva;Oliveira, Claudia Regina Gomes Cardim Mendes de;Fregnani, Eduardo Rodrigues
    • Journal of the Korean Association of Oral and Maxillofacial Surgeons
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    • v.46 no.4
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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.

Undifferentiated Pleomorphic Sarcoma in Mandible

  • Kim, Chul-Hwan;Jang, Jong-Won;Kim, Moon-Young;Kim, Yong-Hwan;Kim, Hang-Gul;Kim, Joo-Hwan
    • Maxillofacial Plastic and Reconstructive Surgery
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    • v.36 no.6
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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.

Undifferentiated Pleomorphic Sarcoma of the Thoracic Aorta Presenting with Ruptured Saccular Aneurysm: A Case Report (소낭성 동맥류 파열로 발현된 흉부 대동맥에서 기원한 미분화성 다형성 육종: 증례 보고)

  • Do Woo Kim;Young Hwan Kim;Ung Rae Kang;Jun Woo Cho;Jae Seok Jang
    • Journal of the Korean Society of Radiology
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    • v.81 no.5
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    • pp.1204-1209
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    • 2020
  • Cases of undifferentiated pleomorphic sarcoma of the thoracic aorta are rare, and usually present with embolic events, renovascular hypertension, or back pain. Mural-based undifferentiated pleomorphic sarcomas that present as ruptured saccular aneurysms are extremely rare and are difficult to differentiate from mycotic aneurysms or penetrating atherosclerotic ulcers. Herein, we report a case of histopathologically proven undifferentiated pleomorphic sarcoma arising from the wall of the descending thoracic aorta that manifested as a mass after thoracic endovascular aortic repair for the treatment of a ruptured saccular aneurysm. We present findings obtained by CT and PET to provide helpful information for the accurate diagnosis and appropriate treatment of future cases.

Undifferentiated Pleomorphic Sarcoma in the Mandibular Ramus and Post-Operative Recurrence: A Case Report (하악지 영역에 발생한 미분화성 다형성 육종: 수술 후 재발한 증례)

  • Ahn, Jaemyung;Jang, Seung-Il;Hong, Jongrak;Paeng, Jun Young
    • The Korean Journal of Oral and Maxillofacial Pathology
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    • v.42 no.5
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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.

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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    • v.56 no.4
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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.

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

  • Sung Oh Song;Min Seon Kim;Kyung Hee Lee;Suk Jin Choi
    • Journal of the Korean Society of Radiology
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    • v.82 no.5
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    • pp.1304-1309
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    • 2021
  • Undifferentiated pleomorphic sarcoma (UPS) is a high-grade soft tissue sarcoma that arises from mesenchymal tissue. Primary UPS of the small intestine is extremely rare, and only a few cases have been reported in the literature. Its presentation is usually nonspecific; however, it may occasionally present as intussusception with intermittent abdominal pain. It is a highly aggressive tumor with a propensity for early distant metastasis to the peritoneum, lymph nodes, other abdominal organs, lungs and brain. To our knowledge, there are no reported cases of endobronchial metastasis from small intestine UPS. We report a rare case of UPS of the small intestine with endobronchial metastasis presenting as intussusception.

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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    • v.52 no.5
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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.

Malignant Inflammatory Fibrous Histiocytoma in a Pointer Dog

  • Do, Sun-hee;Jeong, Kyu-shik
    • Proceedings of the Korean Society of Veterinary Pathology Conference
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    • 2003.10a
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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)

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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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    • v.15 no.22
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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.