Anaplastic Classic Kaposi Sarcoma: PD-L1 Expression and Response to Immunotherapy: A Case Report and Review of the Literature

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Ivan Lolli Oncology Unit of National Institute of Gastroenterology “S. De Bellis,” Research Hospital Castellana Grotte, Bari, Italy

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Anna Maria Valentini Histopathology Unit of National Institute of Gastroenterology “S. De Bellis,” Research Hospital Castellana Grotte, Bari, Italy

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Angela Dalia Ricci Oncology Unit of National Institute of Gastroenterology “S. De Bellis,” Research Hospital Castellana Grotte, Bari, Italy

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Raffaele Armentano Histopathology Unit of National Institute of Gastroenterology “S. De Bellis,” Research Hospital Castellana Grotte, Bari, Italy

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Anaplastic classic Kaposi sarcoma (CKS) is an extremely rare pathologic variant of CKS characterized by high aggressiveness and poor prognosis. We report the clinical course of this malignant histologic form in an otherwise healthy 67-year-old male from Apulia in Southern Italy. The anaplastic progression arose during a long history of CKS and developed after multiple local and systemic treatments. The extremely aggressive and chemorefractory nature of the disease dictated amputation of a lower limb and, later, surgery for metastatic pulmonary involvement. At subsequent relapse, therapy with the anti–PD-1 inhibitor pembrolizumab was started. The immunotherapy was selected based on the PD-L1 expression in the tumor and tumor microenvironment. Remarkably, PD-1 blockade induced a complete and durable response in the patient, with a disease-free survival that has exceeded 18 months, and follow-up is still ongoing.

Submitted March 14, 2022; final revision received October 20, 2022; accepted for publication October 20, 2022. Published online April 7, 2023.

Disclosures: The authors have disclosed that they have not received any financial considerations from any person or organization to support the preparation, analysis, results, or discussion of this article.

Correspondence: Ivan Lolli, MD, Oncology Unit of National Institute of Gastroenterology “S. De Bellis,” 70013 Via Turi 27, Castellana Grotte, Bari, Italy. Email: ivanlolli53@gmail.com

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  • 1.

    Vangipuram R, Tyring SK. Epidemiology of Kaposi sarcoma: review and description of the nonepidemic variant. Int J Dermatol 2019;58:538542.

  • 2.

    Grayson W, Pantanowitz L. Histological variants of cutaneous Kaposi sarcoma. Diagn Pathol 2008;3:31.

  • 3.

    Siozopoulou V, Domen A, Zwaenepoel K, et al. Immune checkpoint inhibitory therapy in sarcomas: is there light at the end of the tunnel? Cancers (Basel) 2021;13:360.

  • 4.

    Kim YJ, Jung CJ, Won CH, et al. PD-1 and PD-L1 expression in Kaposi sarcoma: a comparative study according to the pathological stage and clinical characteristics. J Cutan Pathol 2021;48:221228.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 5.

    Kulangara K, Zhang N, Corigliano E, et al. Clinical utility of the combined positive score for programmed death ligand-1 expression and the approval of pembrolizumab for treatment of gastric cancer. Arch Pathol Lab Med 2019;143:330337.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 6.

    Eisenhauer EA, Therasse P, Bogaerts J, et al. New response evaluation criteria in solid tumours: revised RECIST guideline (version 1.1). Eur J Cancer 2009;45:228247.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 7.

    Seymour L, Bogaerts J, Perrone A, et al. iRECIST: guidelines for response criteria for use in trials testing immunotherapeutics. Lancet Oncol 2017;18:e143152.

  • 8.

    Charfi S, Chaabane H, Chaari I, et al. De novo anaplastic Kaposi sarcoma in a HIV-negative man: a case report and review of the literature. Our Derm Online 2017;8:296299.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 9.

    Tourlaki A, Recalcati S, Boneschi V, et al. Anaplastic Kaposi’s sarcoma: a study of eight patients. Eur J Dermatol 2013;23:382386.

  • 10.

    Chapalain M, Goldman-Lévy G, Kramkimel N, et al. Anaplastic Kaposi’s sarcoma: 5 cases of a rare and aggressive type of Kaposi’s sarcoma. Ann Dermatol Venereol 2018;145:2128.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 11.

    Satta R, Cossu S, Massarelli G, et al. Anaplastic transformation of classic Kaposi’s sarcoma: clinicopathological study of five cases. Br J Dermatol 2001;145:847849.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 12.

    Gambassi G, Semeraro R, Suma V, et al. Aggressive behavior of classical Kaposi’s sarcoma and coexistence with angiosarcoma. J Gerontol A Biol Sci Med Sci 2005;60:520523.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 13.

    Cerimele D, Carlesimo F, Fadda G, et al. Anaplastic progression of classic Kaposi’s sarcoma. Dermatology 1997;194:287289.

  • 14.

    Galanina N, Goodman AM, Cohen PR, et al. Successful treatment of HIV-associated Kaposi sarcoma with immune checkpoint blockade. Cancer Immunol Res 2018;6:11291135.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 15.

    Delyon J, Bizot A, Battistella M, et al. PD-1 blockade with nivolumab in endemic Kaposi sarcoma. Ann Oncol 2018;29:10671069.

  • 16.

    Saller J, Walko CM, Millis SZ, et al. Response to checkpoint inhibitor therapy in advanced classic Kaposi sarcoma: a case report and immunogenomic study. J Natl Compr Canc Netw 2018;16:797800.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 17.

    Delyon J, Biard L, Renaud M, et al. PD-1 blockade with pembrolizumab in classic or endemic Kaposi’s sarcoma: a multicentre, single-arm, phase 2 study. Lancet Oncol 2022;23:491500.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 18.

    Noguchi T, Ward JP, Gubin MM, et al. Temporally distinct PD-L1 expression by tumor and host cells contributes to immune escape. Cancer Immunol Res 2017;5:106117.

  • 19.

    Maleki Vareki S, Garrigós C, Duran I. Biomarkers of response to PD-1/PD-L1 inhibition. Crit Rev Oncol Hematol 2017;116:116124.

  • 20.

    Shklovskaya E, Rizos H. Spatial and temporal changes in PD-L1 expression in cancer: the role of genetic drivers, tumor microenvironment and resistance to therapy. Int J Mol Sci 2020;21:7139.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 21.

    Xu-Monette ZY, Zhang M, Li J, et al. PD-1/PD-L1 blockade: have we found the key to unleash the antitumor immune response? Front Immunol 2017;8:1597.

  • 22.

    Vilain RE, Menzies AM, Wilmott JS, et al. Dynamic changes in PD-L1 expression and immune infiltrates early during treatment predict response to PD-1 blockade in melanoma. Clin Cancer Res 2017;23:50245033.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 23.

    Ugurel S, Schadendorf D, Horny K, et al. Elevated baseline serum PD-1 or PD-L1 predicts poor outcome of PD-1 inhibition therapy in metastatic melanoma. Ann Oncol 2020;31:144152.

    • PubMed
    • Search Google Scholar
    • Export Citation
  • 24.

    Subbiah V, Solit DB, Chan TA, et al. The FDA approval of pembrolizumab for adult and pediatric patients with tumor mutational burden (TMB) ≥10: a decision centered on empowering patients and their physicians. Ann Oncol 2020;31:11151118.

    • PubMed
    • Search Google Scholar
    • Export Citation
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