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Published online: 2024-01-12

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Clear cell sarcoma from diagnosis and multidisciplinary treatment to clinical trials

Anna M. Czarnecka1, Paulina Chmiel1, Paweł Sobczuk12, Mateusz Spałek3, Anna Szumera-Ciećkiewicz4, Ewa Bartnik56, Marcin Zdzienicki1, Tomasz Świtaj1, Monika Dudzisz-Śledź1, Piotr Rutkowski1
DOI: 10.5603/ocp.98279

Abstract

Clear cell sarcoma (CCS), formerly called soft tissue melanoma, is a rare malignant soft tissue sarcoma (STS) characterized by a propensity for lymphatic spread and poor prognosis. Clear cell sarcoma can be distinguished by a t(12; 22) (q13; q12) translocation, which in addition to diagnostic implications may be important for targeted treatment in the future. Clear cell sarcoma occurs mainly on the extremities, most often the shin (in the feet and ankle area), in the tendons, and aponeurosis, often at a young age. Considering the significant ability to develop metastases to regional lymph nodes (about 30% of cases), a sentinel node biopsy (SLNB) should be considered in diagnosis, with possible subsequent radical lymphadenectomy (LND) in the case of metastases. Treatment of localized disease is limited to radical local excision with optional complementary radiotherapy. Due to the resistance to classical chemotherapy and the presence of characteristic molecular abnormalities, research focusing on the use of molecular targeted therapies in this group of cancers is ongoing. In clinical trials, MET inhibitors, and tyrosine kinase inhibitors (TKI) were evaluated. Clear cell sarcoma was also one of the subtypes of tumors assessed in the CREATE clinical trial with crizotinib and IMMUNOSARC with checkpoint inhibitors. However, a poor understanding of the biology and natural course of this sarcoma requires further research to develop an effective treatment and unify clinical guidelines. 

Keywords: sarcomaclear cellSLNBMETimmunotherapy

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References

  1. Cornillie J, van Cann T, Wozniak A, et al. Biology and management of clear cell sarcoma: state of the art and future perspectives. Expert Rev Anticancer Ther. 2016; 16(8): 839–845.
    CrossRefPubMed
  2. ENZINGER FM. CLEAR-CELL SARCOMA OF TENDONS AND APONEUROSES. AN ANALYSIS OF 21 CASES. Cancer. 1965; 18: 1163–1174, doi: 10.1002/1097-0142(196509)18:9<1163::aid-cncr2820180916>3.0.co;2-0.
    PubMed
  3. Chung EB, Enzinger FM. Malignant melanoma of soft parts. A reassessment of clear cell sarcoma. Am J Surg Pathol. 1983; 7(5): 405–413.
    CrossRefPubMed
  4. Lucas DR, Nascimento AG, Sim FH. Clear cell sarcoma of soft tissues. Mayo Clinic experience with 35 cases. Am J Surg Pathol. 1992; 16(12): 1197–1204.
    CrossRefPubMed
  5. Clark MA, Johnson MB, Thway K, et al. Clear cell sarcoma (melanoma of soft parts): The Royal Marsden Hospital experience. Eur J Surg Oncol. 2008; 34(7): 800–804.
    CrossRefPubMed
  6. Malchau SS, Hayden J, Hornicek F, et al. Clear cell sarcoma of soft tissues. J Surg Oncol. 2007; 95(6): 519–522.
    CrossRefPubMed
  7. Bianchi G, Charoenlap C, Cocchi S, et al. Clear cell sarcoma of soft tissue: a retrospective review and analysis of 31 cases treated at Istituto Ortopedico Rizzoli. Eur J Surg Oncol. 2014; 40(5): 505–510.
    CrossRefPubMed
  8. Kawai A, Nakayama R, Matsumine A, et al. Clear cell sarcoma of tendons and aponeuroses: An analysis of 75 cases. J Clin Oncol. 2006; 24(18_suppl): 9572–9572.
    CrossRef
  9. Chen G, Sun S, Du Z, et al. Intra-Extracranial Primary Clear Cell Sarcoma: The First Report and Review of the Literature. World Neurosurg. 2019; 126: e1140–e1146.
    CrossRefPubMed
  10. Bali A, Roy M, Chikkannaiah P, et al. Cutaneous clear cell sarcoma: a rare aggressive tumor with potential diagnostic challenge. J Lab Physicians. 2012; 4(1): 53–55.
    CrossRefPubMed
  11. Hantschke M, Mentzel T, Rütten A, et al. Cutaneous clear cell sarcoma: a clinicopathologic, immunohistochemical, and molecular analysis of 12 cases emphasizing its distinction from dermal melanoma. Am J Surg Pathol. 2010; 34(2): 216–222.
    CrossRefPubMed
  12. Jin L, Sui Y, Zhu H, et al. Primary mediastinal clear cell sarcoma: a case report and review of the literature. Diagn Pathol. 2017; 12(1): 5.
    CrossRefPubMed
  13. Abdollahi A, Khatami F, Tavangar SM, et al. Clear Cell Sarcoma: A Case Report and Review of Literature. Int J Hematol Oncol Stem Cell Res. 2018; 12(1): 65–68.
  14. Hoppin JA, Tolbert PE, Flanders WD, et al. Occupational risk factors for sarcoma subtypes. Epidemiology. 1999; 10(3): 300–306.
    PubMed
  15. Kawai A, Hosono A, Nakayama R, et al. Japanese Musculoskeletal Oncology Group. Clear cell sarcoma of tendons and aponeuroses: a study of 75 patients. Cancer. 2007; 109(1): 109–116.
    CrossRefPubMed
  16. Gonzaga MI, Grant L, Curtin C, et al. The epidemiology and survivorship of clear cell sarcoma: a National Cancer Database (NCDB) review. J Cancer Res Clin Oncol. 2018; 144(9): 1711–1716.
    CrossRefPubMed
  17. Hocar O, Le Cesne A, Berissi S, et al. Clear cell sarcoma (malignant melanoma) of soft parts: a clinicopathologic study of 52 cases. Dermatol Res Pract. 2012; 2012: 984096.
    CrossRefPubMed
  18. Yang XL, Lu SJ, Xue J, et al. Clear cell sarcoma of the right lumbar region: A case report and review of the literature. Oncol Lett. 2014; 8(4): 1625–1627.
    CrossRefPubMed
  19. Rao V, Rekhi B. Cytomorphological spectrum, including immunohistochemical results of 16 cases of clear cell sarcoma of soft tissue, along with positive EWSR1 gene rearrangement result in two cases. Cytopathology. 2020; 31(4): 280–287.
    CrossRefPubMed
  20. Auerbach A, Cassarino DS. Clear Cell Tumors of Soft Tissue. Surg Pathol Clin. 2011; 4(3): 783–798.
    CrossRefPubMed
  21. Mrózek K, Karakousis CP, Perez-Mesa C, et al. Translocation t(12;22)(q13;q12.2-12.3) in a clear cell sarcoma of tendons and aponeuroses. Genes Chromosomes Cancer. 1993; 6(4): 249–252.
    CrossRefPubMed
  22. Reeves BR, Fletcher CD, Gusterson BA. Translocation t(12;22)(q13;q13) is a nonrandom rearrangement in clear cell sarcoma. Cancer Genet Cytogenet. 1992; 64(2): 101–103.
    CrossRefPubMed
  23. Davis IJ, Kim JJ, Ozsolak F, et al. Oncogenic MITF dysregulation in clear cell sarcoma: defining the MiT family of human cancers. Cancer Cell. 2006; 9(6): 473–484.
    CrossRefPubMed
  24. Panagopoulos I, Mertens F, Dêbiec-Rychter M, et al. Molecular genetic characterization of the EWS/ATF1 fusion gene in clear cell sarcoma of tendons and aponeuroses. Int J Cancer. 2002; 99(4): 560–567.
    CrossRefPubMed
  25. Fisher C. The diversity of soft tissue tumours with EWSR1 gene rearrangements: a review. Histopathology. 2014; 64(1): 134–150.
    CrossRefPubMed
  26. Mavrogenis Af, Bianchi G, Stavropoulos Na, et al. Clinicopathological features, diagnosis and treatment of clear cell sarcoma/melanoma of soft parts. Hippokratia. 2013; 17(4): 298–302.
    PubMed
  27. Rossi S, Szuhai K, Ijszenga M, et al. EWSR1-CREB1 and EWSR1-ATF1 fusion genes in angiomatoid fibrous histiocytoma. Clin Cancer Res. 2007; 13(24): 7322–7328.
    CrossRefPubMed
  28. Patel RM, Downs-Kelly E, Weiss SW, et al. Dual-color, break-apart fluorescence in situ hybridization for EWS gene rearrangement distinguishes clear cell sarcoma of soft tissue from malignant melanoma. Mod Pathol. 2005; 18(12): 1585–1590.
    CrossRefPubMed
  29. Yang L, Chen Y, Cui T, et al. Identification of biomarkers to distinguish clear cell sarcoma from malignant melanoma. Hum Pathol. 2012; 43(9): 1463–1470.
    CrossRefPubMed
  30. Davis IJ, McFadden AW, Zhang Y, et al. Identification of the receptor tyrosine kinase c-Met and its ligand, hepatocyte growth factor, as therapeutic targets in clear cell sarcoma. Cancer Res. 2010; 70(2): 639–645.
    CrossRefPubMed
  31. Negri T, Brich S, Conca E, et al. Receptor tyrosine kinase pathway analysis sheds light on similarities between clear-cell sarcoma and metastatic melanoma. Genes Chromosomes Cancer. 2012; 51(2): 111–126.
    CrossRefPubMed
  32. Mae H, Outani H, Imura Y, et al. Targeting the Clear Cell Sarcoma Oncogenic Driver Fusion Gene by HDAC Inhibition. Cancer Res Commun. 2023; 3(7): 1152–1165.
    CrossRefPubMed
  33. Pearl ML, Inagami M, McCauley DL, et al. Mesna, doxorubicin, ifosfamide, and dacarbazine (MAID) chemotherapy for gynecological sarcomas. Int J Gynecol Cancer. 2002; 12(6): 745–748.
    CrossRefPubMed
  34. Hisaoka M, Ishida T, Kuo TT, et al. Clear cell sarcoma of soft tissue: a clinicopathologic, immunohistochemical, and molecular analysis of 33 cases. Am J Surg Pathol. 2008; 32(3): 452–460.
    CrossRefPubMed
  35. Meis-Kindblom JM. Clear cell sarcoma of tendons and aponeuroses: a historical perspective and tribute to the man behind the entity. Adv Anat Pathol. 2006; 13(6): 286–292.
    CrossRefPubMed
  36. Mariappan D, Shashikala Dr. Molecular genetics, diagnosis and management of clear cell sarcoma of hand. IOSR Journal of Dental and Medical Sciences. 2016; 15(07): 15–22.
    CrossRef
  37. Deyrup AT, Althof P, Zhou M, et al. Paraganglioma-like dermal melanocytic tumor: a unique entity distinct from cellular blue nevus, clear cell sarcoma, and cutaneous melanoma. Am J Surg Pathol. 2004; 28(12): 1579–1586.
    CrossRefPubMed
  38. Folpe A, Goodman Z, Ishak K, et al. Clear Cell Myomelanocytic Tumor of the Falciform Ligament/Ligamentum Teres. Am J Surg Pathol. 2000; 24(9): 1239–1246.
    CrossRefPubMed
  39. Thyvalappil A, Sudhamani B, Kizhakkethara G, et al. Paraganglioma-like dermal melanocytic tumor. Indian J Dermatol. 2015; 60(1): 80–81.
    CrossRefPubMed
  40. De Beuckeleer LH, De Schepper AM, Vandevenne JE, et al. MR imaging of clear cell sarcoma (malignant melanoma of the soft parts): a multicenter correlative MRI-pathology study of 21 cases and literature review. Skeletal Radiol. 2000; 29(4): 187–195.
    CrossRefPubMed
  41. Tordjman M, Dubois M, de Malherbe M, et al. Clear Cell Sarcoma of the Tongue on MRI and PET/CT. Clin Nucl Med. 2018; 43(4): e118–e121.
    CrossRefPubMed
  42. Nguyen BaD, Roarke MC, Ram PC. PET monitoring of clear cell sarcoma of tendons and aponeuroses. Clin Nucl Med. 2007; 32(5): 415–417.
    CrossRefPubMed
  43. Zhang X, Kang F, Zheng H, et al. Melanin-targeted [F]-PFPN PET imaging may shed light for clear cell sarcoma. Eur J Nucl Med Mol Imaging. 2023 [Epub ahead of print].
    CrossRefPubMed
  44. Nagano S, Yahiro Y, Yokouchi M, et al. Doppler ultrasound for diagnosis of soft tissue sarcoma: efficacy of ultrasound-based screening score. Radiol Oncol. 2015; 49(2): 135–140.
    CrossRefPubMed
  45. Zamora E, Zamora MA. Ultrasound-based screening and characterization of an atypical clear-cell sarcoma of the hand: A case report. J Med Imaging Radiat Sci. 2021; 52(2): 312–315.
    CrossRefPubMed
  46. Hocar O, Cesne ALe, Terrier P, et al. Clear cell sarcoma (CCS) or malignant melanoma of soft parts: A retrospective clinicopathologic study of 52 cases. J Clin Oncol. 2008; 26(15_suppl): 10576–10576.
    CrossRef
  47. Pasquali S, Gronchi A. Neoadjuvant chemotherapy in soft tissue sarcomas: latest evidence and clinical implications. Ther Adv Med Oncol. 2017; 9(6): 415–429.
    CrossRefPubMed
  48. Boglione A, Bergnolo P, Canton OD, et al. Systemic therapy in clear cell sarcoma (CCS). Ann Oncol. 2016; 27: iv99.
    CrossRef
  49. Jones RL, Constantinidou A, Thway K, et al. Chemotherapy in clear cell sarcoma. Med Oncol. 2011; 28(3): 859–863.
    CrossRefPubMed
  50. Yalcin S, Barista I, Tekuzman G, et al. Dramatic Response to Ifosfamide, Mesna and Doxorubicin Chemotherapy Regimen in an Adult With Clear Cell Sarcoma of the Kidney. J Urol. 1996; 155(6): 2024–2024.
    CrossRef
  51. Tazzari M, Palassini E, Vergani B, et al. Melan-A/MART-1 immunity in a EWS-ATF1 translocated clear cell sarcoma patient treated with sunitinib: a case report. BMC Cancer. 2015; 15: 58.
    CrossRefPubMed
  52. Tinoco G, Husain M, Kim H, et al. Clear cell sarcoma: The OSU experience. J Clin Oncol. 2023; 41(16_suppl): e23554–e23554.
    CrossRef
  53. Firmin N, Boudou-Rouquette P, Duliege D, et al. Outcome of 91 clear cell sarcoma tumor patients: A retrospective study from the French Sarcoma Group (GSF-GETO). J Clin Oncol. 2018; 36(15_suppl): 11552–11552.
    CrossRef
  54. Rutkowski P, Koseła-Paterczyk H, Kozak K, et al. Postępowanie diagnostyczno-terapeutyczne u chorych na mięsaki tkanek miękkich u dorosłych — zalecenia ekspertów. Onkol Prakt Klin Edu. 2023; 9(3): 149–180.
  55. Juel J, Ibrahim RM. A case of clear cell sarcoma-A rare malignancy. Int J Surg Case Rep. 2017; 36: 151–154.
    CrossRefPubMed
  56. Abdellah A, Soufiane B, Amine B, et al. Clear cell sarcoma of tendons and aponeuroses of the parapharyngeal space: an unusual localization of a rare tumor (a case report and review of the literature). Pan Afr Med J. 2014; 19: 147.
    CrossRefPubMed
  57. Matsuoka M, Onodera T, Yokota I, et al. Amputation surgery associated with shortened survival in patients with localized soft tissue sarcoma. J Orthop Sci. 2023 [Epub ahead of print].
    CrossRefPubMed
  58. van Akkooi ACJ, Verhoef C, van Geel AN, et al. Sentinel node biopsy for clear cell sarcoma. Eur J Surg Oncol. 2006; 32(9): 996–999.
    CrossRefPubMed
  59. Al-Refaie WB, Ali MW, Chu DZ, et al. Clear cell sarcoma in the era of sentinel lymph node mapping. J Surg Oncol. 2004; 87(3): 126–129.
    CrossRefPubMed
  60. Andreou D, Boldt H, Werner M, et al. Sentinel node biopsy in soft tissue sarcoma subtypes with a high propensity for regional lymphatic spread--results of a large prospective trial. Ann Oncol. 2013; 24(5): 1400–1405.
    CrossRefPubMed
  61. Alhatem A, Nudelman M, Schwartz RA, et al. Primary Cutaneous Clear Cell Sarcoma, Clinical Outcome With Sentinel Lymph Nodes Status. Am J Clin Pathol. 2020; 153(6): 799–810.
    CrossRefPubMed
  62. Keung EZ, Krause KJ, Maxwell J, et al. Sentinel Lymph Node Biopsy for Extremity and Truncal Soft Tissue Sarcomas: A Systematic Review of the Literature. Ann Surg Oncol. 2023; 30(2): 958–967.
    CrossRefPubMed
  63. Weller JH, Westermann C, Patel P, et al. Trends of lymph node sampling and metastasis in pediatric and young adult patients with clear cell, epithelioid, and synovial sarcomas. Pediatr Blood Cancer. 2022; 69(6): e29455.
    CrossRefPubMed
  64. Tong TR, Chow Tc, Chan OWh, et al. Clear-cell sarcoma diagnosis by fine-needle aspiration: cytologic, histologic, and ultrastructural features; potential pitfalls; and literature review. Diagn Cytopathol. 2002; 26(3): 174–180.
    CrossRefPubMed
  65. Ferrari A, Casanova M, Bisogno G, et al. Clear cell sarcoma of tendons and aponeuroses in pediatric patients: a report from the Italian and German Soft Tissue Sarcoma Cooperative Group. Cancer. 2002; 94(12): 3269–3276.
    CrossRefPubMed
  66. Picciotto F, Zaccagna A, Derosa G, et al. Clear cell sarcoma (malignant melanoma of soft parts) and sentinel lymph node biopsy. Eur J Dermatol. 2005; 15(1): 46–48.
    PubMed
  67. Nishida Y, Yamada Y, Tsukushi S, et al. Sentinel lymph node biopsy reveals a positive popliteal node in clear cell sarcoma. Anticancer Res. 2005; 25(6C): 4413–4416.
    PubMed
  68. Fantini F, Monari P, Bassissi S, et al. Sentinel lymph node biopsy in clear cell sarcoma. J Eur Acad Dermatol Venereol. 2007; 21(9): 1271–1272.
    CrossRefPubMed
  69. Teterycz P, Czarnecka A, Szmajdzinska A, et al. Prognostic and predictive factors for the outcomes of clear cell sarcoma (CCS) multidisciplinary treatment: The role of lymph node involvement. J Clin Oncol. 2020; 38(15_suppl): e23554–e23554.
    CrossRef
  70. Gronchi A, Miah AB, Tos APD, et al. Soft tissue and visceral sarcomas: ESMO–EURACAN–GENTURIS Clinical Practice Guidelines for diagnosis, treatment and follow-up☆. Ann Oncol. 2021; 32(11): 1348–1365.
    CrossRefPubMed
  71. Deroose JP, Eggermont AMM, van Geel AN, et al. Long-term results of tumor necrosis factor alpha- and melphalan-based isolated limb perfusion in locally advanced extremity soft tissue sarcomas. J Clin Oncol. 2011; 29(30): 4036–4044.
    CrossRefPubMed
  72. Deenik W, Mooi WJ, Rutgers EJ, et al. Clear cell sarcoma (malignant melanoma) of soft parts: A clinicopathologic study of 30 cases. Cancer. 1999; 86(6): 969–975.
    PubMed
  73. Constantinidou A, Jones RL, Al-Muderis O, et al. Systemic therapy in clear cell sarcoma. J Clin Oncol. 2010; 28(15_suppl): 10098–10098.
    CrossRef
  74. Smrke A, Frezza AM, Giani C, et al. Systemic treatment of advanced clear cell sarcoma: results from a retrospective international series from the World Sarcoma Network. ESMO Open. 2022; 7(3): 100522.
    CrossRefPubMed
  75. Stacchiotti S, Palassini E, Negri T, et al. Clear cell sarcoma (CCR): Clinical behavior and response to chemotherapy. J Clin Oncol. 2010; 28(15_suppl): 10096–10096.
    CrossRef
  76. Cojocaru E, Thway K, Fisher C, et al. Efficacy of Gemcitabine-based Chemotherapy in Clear Cell Sarcoma of Soft Tissue. Anticancer Res. 2020; 40(12): 7003–7007.
    CrossRefPubMed
  77. Fujimoto M, Hiraga M, Kiyosawa T, et al. Complete remission of metastatic clear cell sarcoma with DAV chemotherapy. Clin Exp Dermatol. 2003; 28(1): 22–24.
    CrossRefPubMed
  78. Tran H, Asfour V, Chang C, et al. Clear cell sarcoma arising from the iliac wing: case report. I J Oncol. 2009; 6(2).
    CrossRef
  79. Kumar Kushwaha A, Soni S. Metastatic Clear Cell Sarcoma Role of Temazolamide. Journal of Dental and Medical Sciences. 2016; 15(12): 8–9.
  80. Katz D, Palmerini E, Pollack SM. More Than 50 Subtypes of Soft Tissue Sarcoma: Paving the Path for Histology-Driven Treatments. Am Soc Clin Oncol Educ Book. 2018; 38: 925–938.
    CrossRefPubMed
  81. Goldberg J, Demetri GD, Choy E, et al. Preliminary results from a phase II study of ARQ 197 in patients with microphthalmia transcription factor family (MiT)-associated tumors. J Clin Oncol. 2009; 27(15_suppl): 10502–10502.
    CrossRef
  82. Wagner AJ, Goldberg JM, Dubois SG, et al. Tivantinib (ARQ 197), a selective inhibitor of MET, in patients with microphthalmia transcription factor-associated tumors: results of a multicenter phase 2 trial. Cancer. 2012; 118(23): 5894–5902.
    CrossRefPubMed
  83. Schöffski P, Wozniak A, Stacchiotti S, et al. Activity and safety of crizotinib in patients with advanced clear-cell sarcoma with MET alterations: European Organization for Research and Treatment of Cancer phase II trial 90101 'CREATE'. Ann Oncol. 2017; 28(12): 3000–3008.
    CrossRefPubMed
  84. Wagner AJ, Goldberg JM, Dubois SG, et al. Tivantinib (ARQ 197), a selective inhibitor of MET, in patients with microphthalmia transcription factor-associated tumors: results of a multicenter phase 2 trial. Cancer. 2012; 118(23): 5894–5902.
    CrossRefPubMed
  85. U.S., C.g.N. NCT03132155: QUILT-3.031: AMG 337 in subjects with advanced or metastatic clear cell sarcoma. https://clinicaltrials.gov/ct2/show/NCT03132155 (11.2023).
  86. George S, Merriam P, Maki RG, et al. Multicenter phase II trial of sunitinib in the treatment of nongastrointestinal stromal tumor sarcomas. J Clin Oncol. 2009; 27(19): 3154–3160.
    CrossRefPubMed
  87. Mir O, Boudou-Rouquette P, Larousserie F, et al. Objective response to sorafenib in advanced clear-cell sarcoma. Ann Oncol. 2012; 23(3): 807–809.
    CrossRefPubMed
  88. Stacchiotti S, Grosso F, Negri T, et al. Tumor response to sunitinib malate observed in clear-cell sarcoma. Ann Oncol. 2010; 21(5): 1130–1131.
    CrossRefPubMed
  89. Nishida Y, Urakawa H, Nakayama R, et al. Phase II trial of pazopanib in patients with metastatic or unresectable chemoresistant sarcomas: A Japanese Musculoskeletal Oncology Group study. Cancer Sci. 2020; 111(9): 3303–3312.
    CrossRefPubMed
  90. Chi Y, Fang Z, Hong X, et al. Safety and Efficacy of Anlotinib, a Multikinase Angiogenesis Inhibitor, in Patients with Refractory Metastatic Soft-Tissue Sarcoma. Clin Cancer Res. 2018; 24(21): 5233–5238.
    CrossRefPubMed
  91. Tang L, Wang Y, Zhang J, et al. Efficacy and safety of anlotinib in advanced soft tissue sarcoma: results from one of multi-centers in a phase IIB trial (ALTER0203). J Clin Oncol. 2019; 37(15_suppl): e22518–e22518.
    CrossRef
  92. Zhang RS, Liu J, Deng YT, et al. The real-world clinical outcomes and treatment patterns of patients with unresectable locally advanced or metastatic soft tissue sarcoma treated with anlotinib in the post-ALTER0203 trial era. Cancer Med. 2022; 11(11): 2271–2283.
    CrossRefPubMed
  93. Chuk MK, Widemann BC, Minard CG, et al. A phase 1 study of cabozantinib in children and adolescents with recurrent or refractory solid tumors, including CNS tumors: Trial ADVL1211, a report from the Children's Oncology Group. Pediatr Blood Cancer. 2018; 65(8): e27077.
    CrossRefPubMed
  94. Akshintala S, Widemann B, Barkauskas D, et al. Phase 2 trial of cabozantinib in children and young adults with refractory sarcomas, Wilms tumor, and rare tumors: Children's Oncology Group Study (ADVL1622). J Clin Oncol. 2021; 39(15_suppl): 10010–10010.
    CrossRef
  95. Steger GG, Wrba F, Mader R, et al. Complete remission of metastasised clear cell sarcoma of tendons and aponeuroses. Eur J Cancer. 1991; 27(3): 254–256.
    CrossRefPubMed
  96. Lauro S, Bordin F, Trasatti L, et al. Concurrent chemoimmunotherapy in metastatic clear cell sarcoma: a case report. Tumori. 1999; 85(6): 512–514.
    CrossRefPubMed
  97. Sidlik Muskatel R, Pillar N, Godefroy J, et al. Case report: Robust response of metastatic clear cell sarcoma treated with cabozantinib and immunotherapy. Front Pediatr. 2022; 10: 940927.
    CrossRefPubMed
  98. Wang J, Gao S, Yang Y, et al. Clinical Experience with Apatinib and Camrelizumab in Advance Clear Cell Sarcoma: A Retrospective Study. Cancer Manag Res. 2021; 13: 8999–9005.
    CrossRefPubMed
  99. Marcrom S, De Los Santos JF, Conry RM. Complete response of mediastinal clear cell sarcoma to pembrolizumab with radiotherapy. Clin Sarcoma Res. 2017; 7: 14.
    CrossRefPubMed
  100. Scheinberg T, Lomax A, Tattersall M, et al. PD-1 blockade using pembrolizumab in adolescent and young adult patients with advanced bone and soft tissue sarcoma. J Clin Oncol. 2017; 35(15_suppl): 3060–3060.
    CrossRef
  101. Goldberg JM, Fisher DE, Demetri GD, et al. Biologic Activity of Autologous, Granulocyte-Macrophage Colony-Stimulating Factor Secreting Alveolar Soft-Part Sarcoma and Clear Cell Sarcoma Vaccines. Clin Cancer Res. 2015; 21(14): 3178–3186.
    CrossRefPubMed
  102. Tsukahara T, Emori M, Murata K, et al. The future of immunotherapy for sarcoma. Expert Opin Biol Ther. 2016; 16(8): 1049–1057.
    CrossRefPubMed
  103. Broto J, Hindi N, Grignani GE, et al. IMMUNOSARC: A collaborative Spanish (GEIS) and Italian (ISG) sarcoma groups phase I/II trial of sunitinib plus nivolumab in advanced soft tissue and bone sarcomas: Results of the phase II- soft-tissue sarcoma cohort. Ann Oncol. 2019; 30: v684.
    CrossRef
  104. Martin-Broto J, Hindi N, Grignani G, et al. Nivolumab and sunitinib combination in advanced soft tissue sarcomas: a multicenter, single-arm, phase Ib/II trial. J Immunother Cancer. 2020; 8(2).
    CrossRefPubMed
  105. Palmerini E, Lopez-Pousa A, Grignani G, et al. IMMUNOSARC: a collaborative Spanish (GEIS) and Italian (ISG) sarcoma groups phase I/II trial of sunitinib and nivolumab in advanced soft tissue and bone sarcoma: Results from the phase II part, bone sarcoma cohort. J Clin Oncol. 2020; 38(15_suppl): 11522–11522.
    CrossRef
  106. Jones AL, Joon A, Haydu LE, et al. Outcomes of melanoma soft parts/clear cell sarcoma (MSP/CCS) patients (pts) with immune and targeted therapies. J Clin Oncol. 2019; 37(15_suppl): e21046–e21046.
    CrossRef
  107. Kuiper DR, Hoekstra HJ, Veth RPH, et al. The management of clear cell sarcoma. Eur J Surg Oncol. 2003; 29(7): 568–570.
    CrossRefPubMed
  108. Abdellah A, Soufiane B, Amine B, et al. Clear cell sarcoma of tendons and aponeuroses of the parapharyngeal space: an unusual localization of a rare tumor (a case report and review of the literature). Pan Afr Med J. 2014; 19: 147.
    CrossRefPubMed
  109. Lewis JJ, Leung D, Heslin M, et al. Association of local recurrence with subsequent survival in extremity soft tissue sarcoma. J Clin Oncol. 1997; 15(2): 646–652.
    CrossRefPubMed
  110. Fujiwara T, Kunisada T, Nakata E, et al. Factors associated with survival in patients with clear cell sarcoma. Bone Joint J. 2023; 105-B(11): 1216–1225.
    CrossRefPubMed
  111. Chen S, Luo P, Yang L, et al. Prognostic analysis of surgically treated clear cell sarcoma: an analysis of a rare tumor from a single center. Int J Clin Oncol. 2019; 24(12): 1605–1611.
    CrossRefPubMed
  112. Agulnik M, Davis E, Albert C, et al. Phase 1/2 study of devimistat in combination with hydroxychloroquine (HCQ) in patients with relapsed or refractory (R/R) clear cell sarcoma (CCS). J Clin Oncol. 2022; 40(16_suppl): TPS11595–TPS11595.
    CrossRef
  113. NCT04274023, C.g. Study on TSR-042 in advanced clear cell sarcoma (ACCeSs). 2023. https://clinicaltrials.gov/ct2/show/NCT04274023 (11.2023).
  114. ClinicalTrials.gov. NCT04458922: Testing Atezolizumab in patients > 2–17 years old with newly diagnosed, Unresectable, or metastatic clear cell sarcoma or chondrosarcoma. https://clinicaltrials.gov/ct2/show/NCT04458922.
  115. NCT03600649, Clinical Trial of SP-2577 (Seclidemstat) in Patients With Relapsed or Refractory Ewing or Ewing-related Sarcomas. https://clinicaltrials.gov/study/NCT03600649.

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