Pleomorphic undifferentiated sarcoma: A case of a giant renal mass

Çilesiz, Nusret Can; Erdoğan, Nusret; Özkan, Arif; Kalkanli, Arif; Hazar, Aydin Ismet; Balcı, Mustafa Bahadır Can; Aydın, Memduh; Özdemir, Enver

doi:10.1016/j.eucr.2018.01.002

Cited by 3 publications

(4 citation statements)

References 4 publications

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“…In this case, the patient presented with gastritis because the mass was located in the posterior peritoneum and pressed forward on the stomach, and some patients may report to the clinic with respiratory symptoms due to tumor metastasis in the lungs. In similar cases and this case report, common symptoms included abdominal pain (lumbago) in five cases (83.3%) ( 5 , 6 , 8 , 9 ), weight loss in four cases (66.7%) ( 5 , 6 , 8 ), anemia in three cases (50%) ( 6 , 8 , 9 ), fever in two cases (33.3%) ( 8 , 9 ), and difficulty urinating in one case (16.7%) ( 8 ).…”

Section: Discussionsupporting

confidence: 76%

“…The disease was first reported in 1964 as malignant fibrous histiocytoma ( 3 ), which has since been renamed undifferentiated pleomorphic sarcoma ( 4 ). The occurrence of this tumor in the retroperitoneum is less reported, and only eight cases have been described, of which five tumors originated in the kidney ( 5 – 9 ), two were documented in a clinical study by A. Pirayesh ( 10 ), and one was a paraspinal primary ( 11 ). We report a case of undifferentiated pleomorphic sarcoma arising in the retroperitoneum in a 59-year-old man and review the available medical literature on undifferentiated pleomorphic sarcoma to summarize the epidemiology, etiology, clinical presentation, radiologic features, diagnosis, and treatment options, including radiotherapy, chemotherapy, and targeted therapy for this rare tumor.…”

Section: Introductionmentioning

confidence: 99%

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Retroperitoneal undifferentiated pleomorphic sarcoma with total nephrectomy: a case report and literature review

Wang

Fan

et al. 2023

Front. Surg.

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BackgroundUndifferentiated pleomorphic sarcoma (UPS) is a highly malignant soft tissue sarcoma with a poor prognosis and no clear effective clinical means for treatment, and there has been no significant progress in research within this field in recent years. This study aimed to investigate the epidemiology, etiology, clinical features, diagnostic modalities, various treatment modalities, and prognosis of retroperitoneal undifferentiated pleomorphic sarcoma and to contribute to the clinical management of this type of disease. In this study, we report a case of undifferentiated pleomorphic sarcoma with a primary origin in the retroperitoneum. Undifferentiated pleomorphic sarcoma occurring in the retroperitoneum is rarely reported.Case descriptionA 59-year-old man with abdominal distension and pain for 4 months presented to our hospital after the failure of conservative treatment. A 9.6 cm by 7.4 cm mass in the left retroperitoneum was found on a CT scan of the whole abdomen with three degrees of enhancement. After surgical treatment, the tumor and the left kidney were completely removed, and pathological examination and genetic sequencing showed an apparent undifferentiated pleomorphic sarcoma. The patient subsequently declined follow-up treatment and is currently alive and well.ConclusionsAt the current level of clinical technology, the treatment of undifferentiated pleomorphic sarcoma is still in the exploratory stage, and the scarcity of clinical cases of this disease may have hindered the acquisition of clinical trials and research data for this disease. At present, the first choice of treatment for undifferentiated pleomorphic sarcoma is still radical resection. In the existing clinical studies, there are no strong data to support the effect of preoperative neoadjuvant chemoradiotherapy and adjuvant chemoradiotherapy in clinical practice. Similar to other diseases, the use of radiotherapy and chemotherapy before and after surgery may be a potential treatment for this disease in the future. Targeted therapy for this disease still needs further exploration, and we need more reports on related diseases to promote future treatment and research on this disease.

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Section: Discussionsupporting

confidence: 76%

Section: Introductionmentioning

confidence: 99%

Retroperitoneal undifferentiated pleomorphic sarcoma with total nephrectomy: a case report and literature review

Wang

Fan

et al. 2023

Front. Surg.

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“…Approximately 50% of all UPS cases are found in the lower extremities, and another 20% in the upper extremities, predominantly affecting middle-aged to elderly males [21,22]. Conversely, the occurrence of UPS in the retroperitoneum is notably rare, with our case representing only the eighth documented instance [6,13,[23][24][25][26][27].…”

Section: Anatomical Localization Of Undifferentiated Pleomorphic Sarcomamentioning

confidence: 98%

“…The histogenetic origins of UPS, previously recognized as MFH, have long been a subject of controversy [11]. Initially defined in 1964 by O'bren and Stout, the tumor was characterized by cellular indicators suggestive of both mesenchymal and mononuclear phagocytic system origins [12,13]. The nomenclature and classification of these tumors have evolved significantly over the decades.…”

Section: Histogenetic Evolution Of Pleomorphic Undifferentiated Sarcomamentioning

confidence: 99%

A Detailed Examination of Retroperitoneal Undifferentiated Pleomorphic Sarcoma: A Case Report and Review of the Existing Literature

Balovic,

Stojanovic,

Radovanovic

et al. 2024

JCM

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This detailed review focuses on retroperitoneal undifferentiated pleomorphic sarcoma (UPS), a particularly aggressive soft-tissue sarcoma that poses unique diagnostic and therapeutic challenges due to its rarity and complex presentation. By documenting a new case of retroperitoneal UPS and conducting a comprehensive review of all known cases, this article aims to expand the existing body of knowledge on the epidemiology, molecular pathogenesis, and treatment strategies associated with this rare disease. The complexity of diagnosing UPS is emphasized given that it rarely occurs in the retroperitoneal space and its histological and molecular complexity often complicates its recognition. This review highlights the need for specialized diagnostic approaches, including advanced imaging techniques and histopathological studies, to accurately diagnose and stage the disease. In terms of treatment, this paper advocates a multidisciplinary approach that combines surgery, radiotherapy and chemotherapy and tailors it to individual patients to optimize treatment outcomes. This review highlights case studies that illustrate the effectiveness of surgical intervention in the treatment of these tumors and emphasize the importance of achieving clear surgical margins to prevent recurrence. Furthermore, this review discusses the potential of new molecular targets and the need for innovative therapies that could bring new hope to patients affected by this challenging sarcoma.

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Metastatic Undiff erentiated Pleomorphic Sarcoma of Kidney: Presenting as Hematuria - A Case Report

Gurwale,

Iqbal,

Choudhury

et al. 2023

Journal of Medical Sciences

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Pleomorphic undifferentiated sarcoma (PUS) is an extremely aggressive soft-tissue sarcoma that occurs most commonly in the trunk and the extremities but is extremely rare in the kidney. This entity has no distinctive clinical or radiological signs for diagnosis. It has high local recurrence and distant metastasis rates along with poor prognosis. We describe a case of PUS of the left kidney with urinary bladder metastases. Radiological investigations suggested an initial diagnosis of multifocal urothelial carcinoma; however, a definitive and confirmatory diagnosis of PUS was made on the basis of histopathological and immunohistochemical examination. Subsequently, left nephroureterectomy along with bladder cuff excision was performed and postoperative histopathology revealed PUS. Follow-up investigations revealed metastatic deposits of tumor in the base of the urinary bladder for which an endoscopic surgery of transurethral resection of the bladder tumor was performed. The histopathological findings were consistent with metastatic high-grade PUS of the kidney with metastasis to the bladder.

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Pleomorphic undifferentiated sarcoma: A case of a giant renal mass

Cited by 3 publications

References 4 publications

Retroperitoneal undifferentiated pleomorphic sarcoma with total nephrectomy: a case report and literature review

Retroperitoneal undifferentiated pleomorphic sarcoma with total nephrectomy: a case report and literature review

A Detailed Examination of Retroperitoneal Undifferentiated Pleomorphic Sarcoma: A Case Report and Review of the Existing Literature

Metastatic Undiff erentiated Pleomorphic Sarcoma of Kidney: Presenting as Hematuria - A Case Report

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