A Detailed Examination of Retroperitoneal Undifferentiated Pleomorphic Sarcoma: A Case Report and Review of the Existing Literature
Abstract
:1. Introduction
2. Case Presentation
3. Discussion
3.1. Histogenetic Evolution of Pleomorphic Undifferentiated Sarcoma
3.2. Risk Factors Associated with Undifferentiated Pleomorphic Sarcoma
3.3. Anatomical Localization of Undifferentiated Pleomorphic Sarcoma
3.4. Molecular Pathogenesis of Undifferentiated Pleomorphic Sarcoma
3.5. Signaling Pathways and Molecular Drivers in Undifferentiated Pleomorphic Sarcoma
3.6. The Role of the Peritumoral Microenvironment in Undifferentiated Pleomorphic Sarcoma
3.7. Clinical Presentation of Retroperitoneal Undifferentiated Pleomorphic Sarcoma
3.8. Diagnostic Challenges and Imaging Features of Undifferentiated Pleomorphic Sarcoma
3.9. Histopathological Profile of Undifferentiated Pleomorphic Sarcoma
3.10. Immunohistochemistry, Molecular Diagnostics, and WHO Criteria in the Diagnosis of Undifferentiated Pleomorphic Sarcoma
3.11. Biomarkers in Retroperitoneal Undifferentiated Pleomorphic Sarcoma
3.12. Multidisciplinary Approach in the Treatment of Retroperitoneal UPS
3.13. Surgical Treatment of Undifferentiated Pleomorphic Sarcoma
3.14. The Role of Radiation Therapy in the Management of Undifferentiated Pleomorphic Sarcoma
3.15. Adjuvant Therapy for Undifferentiated Pleomorphic Sarcoma
3.16. Targeted Therapy in the Management of Undifferentiated Pleomorphic Sarcoma
3.17. Prognostic Factors and Survival Outcomes in Undifferentiated Pleomorphic Sarcoma
3.18. Importance of Collaborative Research and Data-Sharing
3.19. Comprehensive Summary of Retroperitoneal UPS Characteristics
4. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Acknowledgments
Conflicts of Interest
References
- Mohsin, J.; Jamal, A.; Mubeen, N.-u.; Syed, A.A. Retroperitoneal Soft-Tissue Sarcoma: Retrospective Study from a Cancer Hospital in Pakistan. Eur. J. Surg. Oncol. 2022, 48, e166. [Google Scholar] [CrossRef]
- Nakamura, M.; Yamanaka, K.; Kato, T.; Hatano, K.; Kakuta, Y.; Kawashima, A.; Fukuhara, S.; Nonomura, N. A retroperitoneal primary undifferentiated pleomorphic sarcoma. Urol. Case Rep. 2024, 53, 102664. [Google Scholar] [CrossRef]
- Mack, T.; Purgina, B. Updates in Pathology for Retroperitoneal Soft Tissue Sarcoma. Curr. Oncol. 2022, 29, 6400–6418. [Google Scholar] [CrossRef] [PubMed]
- El-Naggar, A.M.; Leprivier, G.; Sorensen, P.H. Chapter 22—Soft Tissue Sarcomas. In Cancer Genomics; Dellaire, G., Berman, J.N., Arceci, R.J., Eds.; Academic Press: Boston, MA, USA, 2014; pp. 377–395. [Google Scholar]
- Fletcher, C.D. The evolving classification of soft tissue tumours—An update based on the new 2013 WHO classification. Histopathology 2014, 64, 2–11. [Google Scholar] [CrossRef]
- Yu, K.; Wang, L.; Bu, F.; Zhang, J.; Hai, Y.; Hu, R.; Lu, J.; Shi, X. Retroperitoneal undifferentiated pleomorphic sarcoma with total nephrectomy: A case report and literature review. Front. Surg. 2023, 10, 1166764. [Google Scholar] [CrossRef] [PubMed]
- Weiss, S.W.; Enzinger, F.M. Malignant fibrous histiocytoma: An analysis of 200 cases. Cancer 1978, 41, 2250–2266. [Google Scholar] [CrossRef]
- Pavlović, M.; Milošević, B.; Radovanović, D.; Cvetković, A.; Trifunović, B.; Čanović, D.; Mitrović, S.; Jovanović, M.; Spasić, M.; Vulović, M.; et al. Malignant fibrous histiocytoma of the right upper leg: A case report. Vojnosanit. Pregl. 2018, 75, 5. [Google Scholar] [CrossRef]
- Wang, J.; Dong, S.; Zhang, J.; Gao, S.; Li, Z.; Li, P.; Yuan, J.; Tian, Z. Undifferentiated pleomorphic sarcoma with neoplastic fever: A retrospective study. Cancer Manag. Res. 2021, 13, 8481–8487. [Google Scholar] [CrossRef]
- Gherzi, L.; Ferrari, M.; Pardolesi, A. Lung metastases from sarcoma: Multidisciplinary approach—A narrative review. AME Surg. J. 2021, 2. [Google Scholar] [CrossRef]
- Müller-Richter, U.D.; Kohlhof, J.K.; Reichert, T.E.; Roldán, J.C. Undifferentiated pleomorphic sarcoma of the orbital region. Br. J. Oral. Maxillofac. Surg. 2008, 46, 325–327. [Google Scholar] [CrossRef]
- O’Brien, J.E.; Stout, A.P. Malignant fibrous xanthomas. Cancer 1964, 17, 1445–1455. [Google Scholar] [CrossRef] [PubMed]
- Çilesiz, N.C.; Erdoğan, N.; Özkan, A.; Kalkanlı, A.; Hazar, A.; Balcı, M.B.C.; Aydın, M.; Özdemir, E. Pleomorphic undifferentiated sarcoma: A case of a giant renal mass. Urol. Case Rep. 2018, 17, 44–47. [Google Scholar] [CrossRef] [PubMed]
- Schneider, J.A.; König, H.; Hertel, E. Malignant fibrous histiocytoma of the renal capsule. Br. J. Urol. 1992, 69, 435–436. [Google Scholar] [CrossRef] [PubMed]
- Chen, S.; Huang, W.; Luo, P.; Cai, W.; Yang, L.; Sun, Z.; Zheng, B.; Yan, W.; Wang, C. Undifferentiated Pleomorphic Sarcoma: Long-Term Follow-Up from a Large Institution. Cancer Manag. Res. 2019, 11, 10001–10009. [Google Scholar] [CrossRef] [PubMed]
- Kelleher, F.C.; Viterbo, A. Histologic and genetic advances in refining the diagnosis of “undifferentiated pleomorphic sarcoma”. Cancers 2013, 5, 218–233. [Google Scholar] [CrossRef] [PubMed]
- Araki, Y.; Yamamoto, N.; Tanzawa, Y.; Higashi, T.; Kuchiba, A.; Hayashi, K.; Takeuchi, A.; Miwa, S.; Igarashi, K.; Endo, M.; et al. Family cancer history and smoking habit associated with sarcoma in a Japanese population study. Sci. Rep. 2022, 12, 17129. [Google Scholar] [CrossRef] [PubMed]
- Schiavi, A.; Lavigne, J.; Turcotte, R.; Kasprzak, L.; Dumas, N.; Chong, G.; Freeman, C.; Alameldin, M.; Galiatsatos, P.; Palma, L.; et al. Using a family history questionnaire to identify adult patients with increased genetic risk for sarcoma. Curr. Oncol. 2015, 22, 317–325. [Google Scholar] [CrossRef] [PubMed]
- McDuffie, H.H.; Pahwa, P.; Karunanayake, C.P.; Spinelli, J.J.; Dosman, J.A. Clustering of cancer among families of cases with Hodgkin Lymphoma (HL), Multiple Myeloma (MM), Non-Hodgkin’s Lymphoma (NHL), Soft Tissue Sarcoma (STS) and control subjects. BMC Cancer 2009, 9, 70. [Google Scholar] [CrossRef] [PubMed]
- Beane, J.D.; Yang, J.C.; White, D.; Steinberg, S.M.; Rosenberg, S.A.; Rudloff, U. Efficacy of adjuvant radiation therapy in the treatment of soft tissue sarcoma of the extremity: 20-year follow-up of a randomized prospective trial. Ann. Surg. Oncol. 2014, 21, 2484–2489. [Google Scholar] [CrossRef]
- Xu, F.; Zhao, F.; Feng, X.; Li, C.; Han, D.; Zheng, S.; Liu, Y.; Lyu, J. Nomogram for predicting cancer-specific survival in undifferentiated pleomorphic sarcoma: A Surveillance, Epidemiology, and End Results -based study. Cancer Control 2021, 28, 10732748211036775. [Google Scholar] [CrossRef]
- Kodera, K.; Hoshino, M.; Takahashi, S.; Hidaka, S.; Kogo, M.; Hashizume, R.; Imakita, T.; Ishiyama, M.; Ogawa, M.; Eto, K. Surgical management of primary undifferentiated pleomorphic sarcoma of the rectum: A case report and review of the literature. World J. Surg. Oncol. 2022, 20, 199. [Google Scholar] [CrossRef]
- Mellas, S.; Bouchikhi, A.A.; Tazi, M.F.; Khallouk, A.; Elammari, J.E.; El Fassi, M.J.; Mellas, N.; Farih, M.H. Primary Pleomorphic Undifferentiated Sarcoma-a Rare Renal Localization: A Case Report. Case Rep. Urol. 2012, 2012, 862493. [Google Scholar] [CrossRef] [PubMed]
- Karaca, L.; Sade, R.; Yüce, I.; Ogul, H.; Bayraktutan, G.; Kantarci, M. A rare localization: Primary pleomorphic undifferentiated sarcoma. Spine J. 2015, 15, e59–e60. [Google Scholar] [CrossRef] [PubMed]
- Pathrose, G.; John, N.T.; Manojkumar, R. A rare case of malignant fibrous histiocytoma/ pleomorphic undifferentiated sarcoma of the kidney. J. Clin. Diagn. Res. 2015, 9, PD27–PD29. [Google Scholar] [CrossRef]
- Gupta, R.; Gupta, S.; Aggarwal, D.; Singh, S. Primary pleomorphic undifferentiated sarcoma of the kidney: A rare renal tumor. Indian J. Pathol. Microbiol. 2008, 51, 573–576. [Google Scholar] [CrossRef] [PubMed]
- Kumar, S.; Bansal, P.; Tiwari, P.; Kundu, A.K. Renal pleomorphic undifferentiated sarcoma: A rarity. Saudi J. Kidney Dis. Transpl. 2012, 23, 1241–1242. [Google Scholar] [CrossRef]
- Sun, H.; Liu, J.; Hu, F.; Xu, M.; Leng, A.; Jiang, F.; Chen, K. Current research and management of undifferentiated pleomorphic sarcoma/myofibrosarcoma. Front. Genet. 2023, 14, 1109491. [Google Scholar] [CrossRef]
- Ali, N.M.; Niada, S.; Brini, A.T.; Morris, M.R.; Kurusamy, S.; Alholle, A.; Huen, D.; Antonescu, C.R.; Tirode, F.; Sumathi, V.; et al. Genomic and transcriptomic characterisation of undifferentiated pleomorphic sarcoma of bone. J. Pathol. 2019, 247, 166–176. [Google Scholar] [CrossRef]
- Cui, Y.; Han, L.; Shang, J.; Fang, W.; Zhao, M.; Chen, D.; Liu, H. Primary cardiac undifferentiated pleomorphic sarcoma is associated with TP53 mutation during lack of MDM2 amplification, and targeted sequencing analysis reveals potentially actionable targets. Hum. Pathol. 2022, 123, 113–122. [Google Scholar] [CrossRef]
- Li, G.Z.; Okada, T.; Kim, Y.M.; Agaram, N.P.; Sanchez-Vega, F.; Shen, Y.; Tsubokawa, N.; Rios, J.; Martin, A.S.; Dickson, M.A.; et al. Rb and p53-Deficient Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma Require Skp2 for Survival. Cancer Res. 2020, 80, 2461–2471. [Google Scholar] [CrossRef]
- Okada, T.; Lee, A.Y.; Qin, L.X.; Agaram, N.; Mimae, T.; Shen, Y.; O’Connor, R.; López-Lago, M.A.; Craig, A.; Miller, M.L.; et al. Integrin-α10 Dependency Identifies RAC and RICTOR as Therapeutic Targets in High-Grade Myxofibrosarcoma. Cancer Discov. 2016, 6, 1148–1165. [Google Scholar] [CrossRef] [PubMed]
- Lazar, A.J.; McLellan, M.D.; Bailey, M.H.; Miller, C.A.; Appelbaum, E.L.; Cordes, M.G.; Fronick, C.C.; Fulton, L.A.; Fulton, R.S.; Mardis, E.R.; et al. Comprehensive and Integrated Genomic Characterization of Adult Soft Tissue Sarcomas. Cell 2017, 171, 950–965.e28. [Google Scholar] [CrossRef]
- Zheng, B.; Qu, Y.; Wang, J.; Shi, Y.; Yan, W. Pathogenic and targetable genetic alterations in resected recurrent undifferentiated pleomorphic sarcomas identified by targeted next-generation sequencing. Cancer Genom. Proteom. 2019, 16, 221–228. [Google Scholar] [CrossRef]
- Ye, S.; Liu, Y.; Fuller, A.M.; Katti, R.; Ciotti, G.E.; Chor, S.; Alam, M.Z.; Devalaraja, S.; Lorent, K.; Weber, K.; et al. TGFβ and Hippo Pathways Cooperate to Enhance Sarcomagenesis and Metastasis through the Hyaluronan-Mediated Motility Receptor (HMMR). Mol. Cancer Res. 2020, 18, 560–573. [Google Scholar] [CrossRef]
- Schmitz, K.; Koeppen, H.; Binot, E.; Fassunke, J.; Künstlinger, H.; Ihle, M.A.; Heydt, C.; Wardelmann, E.; Büttner, R.; Merkelbach-Bruse, S.; et al. MET gene copy number alterations and expression of MET and hepatocyte growth factor are potential biomarkers in angiosarcomas and undifferentiated pleomorphic sarcomas. PLoS ONE 2015, 10, e0120079. [Google Scholar] [CrossRef]
- Pazzaglia, L.; Novello, C.; Conti, A.; Pollino, S.; Picci, P.; Benassi, M.S. miR-152 down-regulation is associated with MET up-regulation in leiomyosarcoma and undifferentiated pleomorphic sarcoma. Cell. Oncol. 2017, 40, 77–88. [Google Scholar] [CrossRef]
- Wong, P.; Hui, A.; Su, J.; Yue, S.; Haibe-Kains, B.; Gokgoz, N.; Xu, W.; Bruce, J.; Williams, J.; Catton, C.; et al. Prognostic microRNAs modulate the RHO adhesion pathway: A potential therapeutic target in undifferentiated pleomorphic sarcomas. Oncotarget 2015, 6, 39127–39139. [Google Scholar] [CrossRef]
- Serrano, C.; Romagosa, C.; Hernández-Losa, J.; Simonetti, S.; Valverde, C.; Moliné, T.; Somoza, R.; Pérez, M.; Vélez, R.; Vergés, R.; et al. RAS/MAPK pathway hyperactivation determines poor prognosis in undifferentiated pleomorphic sarcomas. Cancer 2016, 122, 99–107. [Google Scholar] [CrossRef] [PubMed]
- Shiraishi, D.; Fujiwara, Y.; Horlad, H.; Saito, Y.; Iriki, T.; Tsuboki, J.; Cheng, P.; Nakagata, N.; Mizuta, H.; Bekki, H.; et al. CD163 Is Required for Protumoral Activation of Macrophages in Human and Murine Sarcoma. Cancer Res. 2018, 78, 3255–3266. [Google Scholar] [CrossRef]
- Keung, E.Z.; Burgess, M.; Salazar, R.; Parra, E.R.; Rodrigues-Canales, J.; Bolejack, V.; Van Tine, B.A.; Schuetze, S.M.; Attia, S.; Riedel, R.F.; et al. Correlative Analyses of the SARC028 Trial Reveal an Association Between Sarcoma-Associated Immune Infiltrate and Response to Pembrolizumab. Clin. Cancer Res. 2020, 26, 1258–1266. [Google Scholar] [CrossRef]
- Hu, Q.; Zhou, S.; Hu, X.; Zhang, H.; Huang, S.; Wang, Y. Systematic screening identifies a 2-gene signature as a high-potential prognostic marker of undifferentiated pleomorphic sarcoma/myxofibrosarcoma. J. Cell Mol. Med. 2020, 24, 1010–1021. [Google Scholar] [CrossRef]
- Pollack, S.M.; He, Q.; Yearley, J.H.; Emerson, R.; Vignali, M.; Zhang, Y.; Redman, M.W.; Baker, K.K.; Cooper, S.; Donahue, B.; et al. T-cell infiltration and clonality correlate with programmed cell death protein 1 and programmed death-ligand 1 expression in patients with soft tissue sarcomas. Cancer 2017, 123, 3291–3304. [Google Scholar] [CrossRef]
- Xu, G.; Shi, X.; Shao, G. An unusual case of metastasis of a pulmonary undifferentiated pleomorphic sarcoma to the right ventricle: A case report. J. Med. Case Rep. 2013, 7, 165. [Google Scholar] [CrossRef] [PubMed]
- Brennan, C.; Kajal, D.; Khalili, K.; Ghai, S. Solid malignant retroperitoneal masses-a pictorial review. Insights Imaging 2014, 5, 53–65. [Google Scholar] [CrossRef]
- Sun, S.; Bonaffini, P.A.; Nougaret, S.; Fournier, L.; Dohan, A.; Chong, J.; Smith, J.; Addley, H.; Reinhold, C. How to differentiate uterine leiomyosarcoma from leiomyoma with imaging. Diagn. Interv. Imaging 2019, 100, 619–634. [Google Scholar] [CrossRef]
- Alley, J.G., Jr. Musculoskeletal imaging: The requisites, 2nd ed. Acad. Radiol. 2003, 10, 1469. [Google Scholar] [CrossRef]
- MRI of Bone and Soft Tissue Tumors and Tumorlike Lesions. Differential Diagnosis and Atlas. AJNR Am. J. Neuroradiol. 2009, 30, e16. [Google Scholar]
- Yoo, H.J.; Hong, S.H.; Kang, Y.; Choi, J.Y.; Moon, K.C.; Kim, H.S.; Han, I.; Yi, M.; Kang, H.S. MR imaging of myxofibrosarcoma and undifferentiated sarcoma with emphasis on tail sign; diagnostic and prognostic value. Eur. Radiol. 2014, 24, 1749–1757. [Google Scholar] [CrossRef]
- Lefkowitz, R.A.; Landa, J.; Hwang, S.; Zabor, E.C.; Moskowitz, C.S.; Agaram, N.P.; Panicek, D.M. Myxofibrosarcoma: Prevalence and diagnostic value of the “tail sign” on magnetic resonance imaging. Skeletal Radiol. 2013, 42, 809–818. [Google Scholar] [CrossRef]
- Lindberg, M.R.; Lucas, D.; Cassarino, D.S.; Gardner, J.M.; Stallings-Archer, K. (Eds.) Undifferentiated Pleomorphic Sarcoma. In Diagnostic Pathology: Soft Tissue Tumors, 2nd ed.; Elsevier: Philadelphia, PA, USA, 2016; pp. 718–721. [Google Scholar]
- Goldblum, J.R. An approach to pleomorphic sarcomas: Can we subclassify, and does it matter? Mod. Pathol. 2014, 27 (Suppl. S1), S39–S46. [Google Scholar] [CrossRef]
- Thway, K.; Fisher, C. Undifferentiated and dedifferentiated soft tissue neoplasms: Immunohistochemical surrogates for differential diagnosis. Semin. Diagn. Pathol. 2021, 38, 170–186. [Google Scholar] [CrossRef] [PubMed]
- Mairal, A.; Terrier, P.; Chibon, F.; Sastre, X.; Lecesne, A.; Aurias, A. Loss of chromosome 13 is the most frequent genomic imbalance in malignant fibrous histiocytomas. A comparative genomic hybridization analysis of a series of 30 cases. Cancer Genet. Cytogenet. 1999, 111, 134–138. [Google Scholar] [CrossRef] [PubMed]
- Hofvander, J.; Tayebwa, J.; Nilsson, J.; Magnusson, L.; Brosjö, O.; Larsson, O.; Vult von Steyern, F.; Mandahl, N.; Fletcher, C.D.; Mertens, F. Recurrent PRDM10 gene fusions in undifferentiated pleomorphic sarcoma. Clin. Cancer Res. 2015, 21, 864–869. [Google Scholar] [CrossRef] [PubMed]
- Jo, V.Y.; Fletcher, C.D. WHO classification of soft tissue tumours: An update based on the 2013 (4th) edition. Pathology 2014, 46, 95–104. [Google Scholar] [CrossRef] [PubMed]
- Tsagozis, P.; Augsten, M.; Zhang, Y.; Li, T.; Hesla, A.; Bergh, J.; Haglund, F.; Tobin, N.P.; Ehnman, M. An immunosuppressive macrophage profile attenuates the prognostic impact of CD20-positive B cells in human soft tissue sarcoma. Cancer Immunol. Immunother. 2019, 68, 927–936. [Google Scholar] [CrossRef] [PubMed]
- Pillozzi, S.; Bernini, A.; Palchetti, I.; Crociani, O.; Antonuzzo, L.; Campanacci, D.; Scoccianti, G. Soft Tissue Sarcoma: An Insight on Biomarkers at Molecular, Metabolic and Cellular Level. Cancers 2021, 13, 3044. [Google Scholar] [CrossRef] [PubMed]
- Guled, M.; Pazzaglia, L.; Borze, I.; Mosakhani, N.; Novello, C.; Benassi, M.S.; Knuutila, S. Differentiating soft tissue leiomyosarcoma and undifferentiated pleomorphic sarcoma: A miRNA analysis. Genes Chromosomes Cancer 2014, 53, 693–702. [Google Scholar] [CrossRef] [PubMed]
- Welsch, K.; Breuninger, H.; Metzler, G.; Sickinger, F.; Eberle, F.C.; Häfner, H.M.; Schnabl, S.M. Patterns of infiltration and local recurrences of various types of cutaneous sarcomas following three-dimensional histology. JDDG J. Dtsch. Dermatol. Ges. 2018, 16, 1434–1442. [Google Scholar] [CrossRef]
- Sampo, M.; Tarkkanen, M.; Huuhtanen, R.; Tukiainen, E.; Böhling, T.; Blomqvist, C. Impact of the smallest surgical margin on local control in soft tissue sarcoma. Br. J. Surg. 2008, 95, 237–243. [Google Scholar] [CrossRef]
- Fujiwara, T.; Stevenson, J.; Parry, M.; Tsuda, Y.; Tsoi, K.; Jeys, L. What is an adequate margin for infiltrative soft-tissue sarcomas? Eur. J. Surg. Oncol. 2020, 46, 277–281. [Google Scholar] [CrossRef]
- Kainhofer, V.; Smolle, M.; Szkandera, J.; Liegl-Atzwanger, B.; Maurer-Ertl, W.; Gerger, A.; Riedl, J.; Leithner, A. The width of resection margins influences local recurrence in soft tissue sarcoma patients. Eur. J. Surg. Oncol. EJSO 2016, 42, 899–906. [Google Scholar] [CrossRef]
- Vodanovich, D.A.; Spelman, T.; May, D.; Slavin, J.; Choong, P.F.M. Predicting the prognosis of undifferentiated pleomorphic soft tissue sarcoma: A 20-year experience of 266 cases. ANZ J. Surg. 2019, 89, 1045–1050. [Google Scholar] [CrossRef]
- Keung, E.Z.; Tsai, J.W.; Ali, A.M.; Cormier, J.N.; Bishop, A.J.; Guadagnolo, B.A.; Torres, K.E.; Somaiah, N.; Hunt, K.K.; Wargo, J.A.; et al. Analysis of the immune infiltrate in undifferentiated pleomorphic sarcoma of the extremity and trunk in response to radiotherapy: Rationale for combination neoadjuvant immune checkpoint inhibition and radiotherapy. Oncoimmunology 2018, 7, e1385689. [Google Scholar] [CrossRef] [PubMed]
- Kamat, N.V.; Million, L.; Yao, D.H.; Donaldson, S.S.; Mohler, D.G.; van de Rijn, M.; Avedian, R.S.; Kapp, D.S.; Ganjoo, K.N. The Outcome of Patients With Localized Undifferentiated Pleomorphic Sarcoma of the Lower Extremity Treated at Stanford University. Am. J. Clin. Oncol. 2019, 42, 166–171. [Google Scholar] [CrossRef] [PubMed]
- Ozcelik, M.; Seker, M.; Eraslan, E.; Koca, S.; Yazilitas, D.; Ercelep, O.; Ozaslan, E.; Kaya, S.; Hacibekiroglu, I.; Menekse, S.; et al. Evaluation of prognostic factors in localized high-grade undifferentiated pleomorphic sarcoma: Report of a multi-institutional experience of Anatolian Society of Medical Oncology. Tumour Biol. 2016, 37, 5231–5237. [Google Scholar] [CrossRef]
- Di Marco, J.; Kaci, R.; Orcel, P.; Nizard, R.; Laredo, J.D. Radiation-induced undifferentiated pleomorphic sarcoma after radiation therapy for a desmoid tumour. Cancer Radiother. 2016, 20, 36–38. [Google Scholar] [CrossRef] [PubMed]
- Patel, R.; Hu, J.; Chopra, S.; Lee, C. Neoadjuvant chemotherapy for radiation-associated soft-tissue sarcoma: A case report. Rare Tumors 2019, 11, 2036361318821763. [Google Scholar] [CrossRef]
- Maruzzo, M.; Rastrelli, M.; Lumachi, F.; Zagonel, V.; Basso, U. Adjuvant and neoadjuvant chemotherapy for soft tissue sarcomas. Curr. Med. Chem. 2013, 20, 613–620. [Google Scholar] [CrossRef]
- Young, R.J.; Litière, S.; Lia, M.; Hogendoorn, P.C.; Fisher, C.; Mechtersheimer, G.; Daugaard, S.; Sciot, R.; Collin, F.; Messiou, C. Predictive and prognostic factors associated with soft tissue sarcoma response to chemotherapy: A subgroup analysis of the European Organisation for Research and Treatment of Cancer 62012 study. Acta Oncol. 2017, 56, 1013–1020. [Google Scholar] [CrossRef]
- Crago, A.M.; Cardona, K.; Koseła-Paterczyk, H.; Rutkowski, P. Management of Myxofibrosarcoma and Undifferentiated Pleomorphic Sarcoma. Surg. Oncol. Clin. N. Am. 2022, 31, 419–430. [Google Scholar] [CrossRef]
- Martinez-Cruzado, L.; Tornin, J.; Rodriguez, A.; Santos, L.; Allonca, E.; Fernandez-Garcia, M.T.; Astudillo, A.; Garcia-Pedrero, J.M.; Rodriguez, R. Trabectedin and campthotecin synergistically eliminate cancer stem cells in cell-of-origin sarcoma models. Neoplasia 2017, 19, 460–470. [Google Scholar] [CrossRef] [PubMed]
- Gronchi, A.; Palmerini, E.; Quagliuolo, V.; Martin Broto, J.; Lopez Pousa, A.; Grignani, G.; Brunello, A.; Blay, J.-Y.; Tendero, O.; Diaz Beveridge, R. Neoadjuvant chemotherapy in high-risk soft tissue sarcomas: Final results of a randomized trial from Italian (ISG), Spanish (GEIS), French (FSG), and Polish (PSG) Sarcoma Groups. J. Clin. Oncol. 2020, 38, 2178–2186. [Google Scholar] [CrossRef] [PubMed]
- Tanaka, K.; Mizusawa, J.; Fukuda, H.; Araki, N.; Chuman, H.; Takahashi, M.; Ozaki, T.; Hiruma, T.; Tsuchiya, H.; Morioka, H. Perioperative chemotherapy with ifosfamide and doxorubicin for high-grade soft tissue sarcomas in the extremities (JCOG0304). Jpn. J. Clin. Oncol. 2015, 45, 555–561. [Google Scholar] [CrossRef]
- Gronchi, A.; Stacchiotti, S.; Verderio, P.; Ferrari, S.; Martin Broto, J.; Lopez-Pousa, A.; Llombart-Bosch, A.; Dei Tos, A.P.; Collini, P.; Jurado, J.C.r.u.z.; et al. Short, full-dose adjuvant chemotherapy (CT) in high-risk adult soft tissue sarcomas (STS): Long-term follow-up of a randomized clinical trial from the Italian Sarcoma Group and the Spanish Sarcoma Group. Ann. Oncol. 2016, 27, 2283–2288. [Google Scholar] [CrossRef]
- Cheung, L.S.; Chen, L.; Oke, T.F.; Schaffer, T.B.; Boudadi, K.; Ngo, J.T.; Gross, J.M.; Kemberling, H.; Diaz, L.A.; Lipson, E. Anti-PD-1 elicits regression of undifferentiated pleomorphic sarcomas with UV-mutation signatures. J. Immunother. Cancer 2021, 9, e002345. [Google Scholar] [CrossRef] [PubMed]
- Toulmonde, M.; Penel, N.; Adam, J.; Chevreau, C.; Blay, J.-Y.; Le Cesne, A.; Bompas, E.; Piperno-Neumann, S.; Cousin, S.; Grellety, T. Use of PD-1 targeting, macrophage infiltration, and IDO pathway activation in sarcomas: A phase 2 clinical trial. JAMA Oncol. 2018, 4, 93–97. [Google Scholar] [CrossRef]
- Boxberg, M.; Steiger, K.; Lenze, U.; Rechl, H.; von Eisenhart-Rothe, R.; Wörtler, K.; Weichert, W.; Langer, R.; Specht, K. PD-L1 and PD-1 and characterization of tumor-infiltrating lymphocytes in high grade sarcomas of soft tissue–prognostic implications and rationale for immunotherapy. Oncoimmunology 2018, 7, e1389366. [Google Scholar] [CrossRef]
- You, Y.; Guo, X.; Zhuang, R.; Zhang, C.; Wang, Z.; Shen, F.; Wang, Y.; Liu, W.; Zhang, Y.; Lu, W. Activity of PD-1 inhibitor combined with anti-angiogenic therapy in advanced sarcoma: A single-center retrospective analysis. Front. Mol. Biosci. 2021, 8, 747650. [Google Scholar] [CrossRef]
- Tian, Z.; Dong, S.; Yang, Y.; Gao, S.; Yang, Y.; Yang, J.; Zhang, P.; Wang, X.; Yao, W. Nanoparticle albumin-bound paclitaxel and PD-1 inhibitor (sintilimab) combination therapy for soft tissue sarcoma: A retrospective study. BMC Cancer 2022, 22, 56. [Google Scholar] [CrossRef]
- Oh, C.R.; Hong, J.Y.; Kim, J.H.; Lee, J.S.; Kim, H.S.; Kim, T.W.; Ahn, J.-H.; Kim, J.E. Real-world outcomes of pazopanib treatment in Korean patients with advanced soft tissue sarcoma: A Multicenter Retrospective Cohort Study. Target. Oncol. 2020, 15, 485–493. [Google Scholar] [CrossRef]
- Chi, Y.; Fang, Z.; Hong, X.; Yao, Y.; Sun, P.; Wang, G.; Du, F.; Sun, Y.; Wu, Q.; Qu, G. Safety and efficacy of anlotinib, a multikinase angiogenesis inhibitor, in patients with refractory metastatic soft-tissue sarcoma. Clin. Cancer Res. 2018, 24, 5233–5238. [Google Scholar] [CrossRef]
- Sun, D.X.; Liao, G.J.; Liu, K.G.; Jian, H. Endosialin-expressing bone sarcoma stem-like cells are highly tumor-initiating and invasive. Mol. Med. Rep. 2015, 12, 5665–5670. [Google Scholar] [CrossRef] [PubMed]
- Eisinger-Mathason, T.S.; Zhang, M.; Qiu, Q.; Skuli, N.; Nakazawa, M.S.; Karakasheva, T.; Mucaj, V.; Shay, J.E.; Stangenberg, L.; Sadri, N.; et al. Hypoxia-dependent modification of collagen networks promotes sarcoma metastasis. Cancer Discov. 2013, 3, 1190–1205. [Google Scholar] [CrossRef] [PubMed]
- Tawbi, H.A.; Burgess, M.; Bolejack, V.; Van Tine, B.A.; Schuetze, S.M.; Hu, J.; D’Angelo, S.; Attia, S.; Riedel, R.F.; Priebat, D.A. Pembrolizumab in advanced soft-tissue sarcoma and bone sarcoma (SARC028): A multicentre, two-cohort, single-arm, open-label, phase 2 trial. Lancet Oncol. 2017, 18, 1493–1501. [Google Scholar] [CrossRef]
- Kim, S.; Dodd, R.D.; Mito, J.K.; Ma, Y.; Kim, Y.; Riedel, R.F.; Kirsch, D.G. Efficacy of phosphatidylinositol-3 kinase inhibitors in a primary mouse model of undifferentiated pleomorphic sarcoma. Sarcoma 2012, 2012, 680708. [Google Scholar] [CrossRef] [PubMed]
- Tokumoto, H.; Setoguchi, T.; Saitoh, Y.; Sasaki, H.; Nagano, S.; Maeda, S.; Tanimoto, A.; Taniguchi, N. Neurotensin receptor 1 is a new therapeutic target for human undifferentiated pleomorphic sarcoma growth. Mol. Carcinog. 2019, 58, 2230–2240. [Google Scholar] [CrossRef]
- Berlow, N.E.; Grasso, C.S.; Quist, M.J.; Cheng, M.; Gandour-Edwards, R.; Hernandez, B.S.; Michalek, J.E.; Ryan, C.; Spellman, P.; Pal, R. Deep functional and molecular characterization of a high-risk undifferentiated pleomorphic sarcoma. Sarcoma 2020, 2020, 6312480. [Google Scholar] [CrossRef]
- Salcedo Hernández, R.A.; Lino-Silva, L.S.; de León, D.C.; Herrera-Gómez, Á.; Martínez-Said, H.; Meneses-García, A. Importance of tumor size in soft tissue sarcomas: A proposal for a nomogram based on a score system to staging soft tissue sarcomas in the postoperative setting. Med. Oncol. 2014, 31, 873. [Google Scholar] [CrossRef] [PubMed]
- Song, B.; Lee, K.; Lee, C.; Moon, K.C. Prognostic significance of microscopic tumor extension in local recurrence of myxofibrosarcoma and undifferentiated pleomorphic sarcoma. Pathol. Int. 2018, 68, 509–516. [Google Scholar] [CrossRef]
- Lou, Y.; Wan, W.; Wu, Z.; Yang, J.; Xu, K.; Huang, Q.; Liu, T.; Wei, H.; Yang, X.; Xiao, J. Prognostic Factors for Patients With Undifferentiated High Grade Pleomorphic Sarcoma of the Spine. Spine 2019, 44, E539–E548. [Google Scholar] [CrossRef]
- Hu, C.Y.; Pan, Z.Y.; Yang, J.; Chu, X.H.; Zhang, J.; Tao, X.J.; Chen, W.M.; Li, Y.J.; Lyu, J. Nomograms for predicting long-term overall survival and cancer-specific survival in lip squamous cell carcinoma: A population-based study. Cancer Med. 2019, 8, 4032–4042. [Google Scholar] [CrossRef] [PubMed]
- Dineen, S.P.; Roland, C.L.; Feig, R.; May, C.; Zhou, S.; Demicco, E.; Sannaa, G.A.; Ingram, D.; Wang, W.L.; Ravi, V.; et al. Radiation-Associated Undifferentiated Pleomorphic Sarcoma is Associated with Worse Clinical Outcomes than Sporadic Lesions. Ann. Surg. Oncol. 2015, 22, 3913–3920. [Google Scholar] [CrossRef] [PubMed]
- Oda, Y.; Tamiya, S.; Oshiro, Y.; Hachitanda, Y.; Kinukawa, N.; Iwamoto, Y.; Tsuneyoshi, M. Reassessment and clinicopathological prognostic factors of malignant fibrous histiocytoma of soft parts. Pathol. Int. 2002, 52, 595–606. [Google Scholar] [CrossRef] [PubMed]
Characteristic | Description |
---|---|
Incidence and Prevalence | Rare, accounting for about 1–2% of all solid tumors. Retroperitoneal occurrence is particularly uncommon. |
Age of Onset | Predominantly affects middle-aged and elderly males, presenting in the sixth to seventh decades of life. |
Symptoms | Indirect and related to the tumor’s impact on surrounding tissues, including nonspecific abdominal pain, weight loss, and anemia. |
Diagnostic Challenges | Diagnosing UPS is complex due to its rarity and nonspecific imaging characteristics. Requires exclusionary diagnostic methods often involving advanced imaging like CT and MRI. |
Histopathological Features | Displays a complex and highly variable architecture with a predominance of spindle, pleomorphic, and epithelioid cell types. Frequent features include necrosis, hemorrhage, and a high number of mitotic figures. |
Molecular and Genetic Profile | Characterized by a variety of genetic and epigenetic alterations. Notable for the presence of TP53 mutations, deletions, and other chromosomal aberrations contributing to tumor behavior. |
Treatment Approaches | Primarily surgical resection with an emphasis on achieving negative margins due to the high risk of recurrence and metastasis. Radiation and chemotherapy are used based on tumor location and stage. |
Prognosis and Survival Factors | Prognosis varies significantly based on tumor size, location, and surgical outcomes. Deep-seated tumors and inadequate surgical margins are associated with poorer prognosis. |
Disclaimer/Publisher’s Note: The statements, opinions and data contained in all publications are solely those of the individual author(s) and contributor(s) and not of MDPI and/or the editor(s). MDPI and/or the editor(s) disclaim responsibility for any injury to people or property resulting from any ideas, methods, instructions or products referred to in the content. |
© 2024 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license (https://creativecommons.org/licenses/by/4.0/).
Share and Cite
Balovic, G.; Stojanovic, B.S.; Radovanovic, D.; Lazic, D.; Ilic, M.; Jovanovic, I.; Svilar, D.; Stankovic, V.; Sibalija Balovic, J.; Markovic, B.S.; et al. A Detailed Examination of Retroperitoneal Undifferentiated Pleomorphic Sarcoma: A Case Report and Review of the Existing Literature. J. Clin. Med. 2024, 13, 3684. https://doi.org/10.3390/jcm13133684
Balovic G, Stojanovic BS, Radovanovic D, Lazic D, Ilic M, Jovanovic I, Svilar D, Stankovic V, Sibalija Balovic J, Markovic BS, et al. A Detailed Examination of Retroperitoneal Undifferentiated Pleomorphic Sarcoma: A Case Report and Review of the Existing Literature. Journal of Clinical Medicine. 2024; 13(13):3684. https://doi.org/10.3390/jcm13133684
Chicago/Turabian StyleBalovic, Goran, Bojana S. Stojanovic, Dragce Radovanovic, Dejan Lazic, Milena Ilic, Ivan Jovanovic, Dejan Svilar, Vesna Stankovic, Jelena Sibalija Balovic, Bojana Simovic Markovic, and et al. 2024. "A Detailed Examination of Retroperitoneal Undifferentiated Pleomorphic Sarcoma: A Case Report and Review of the Existing Literature" Journal of Clinical Medicine 13, no. 13: 3684. https://doi.org/10.3390/jcm13133684
APA StyleBalovic, G., Stojanovic, B. S., Radovanovic, D., Lazic, D., Ilic, M., Jovanovic, I., Svilar, D., Stankovic, V., Sibalija Balovic, J., Markovic, B. S., Dimitrijevic Stojanovic, M., Jovanovic, D., & Stojanovic, B. (2024). A Detailed Examination of Retroperitoneal Undifferentiated Pleomorphic Sarcoma: A Case Report and Review of the Existing Literature. Journal of Clinical Medicine, 13(13), 3684. https://doi.org/10.3390/jcm13133684