{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,21]],"date-time":"2026-09-21T14:00:47Z","timestamp":1789999247833,"version":"4.0.1"},"reference-count":62,"publisher":"MDPI AG","issue":"18","license":[{"start":{"date-parts":[[2026,9,19]],"date-time":"2026-09-19T00:00:00Z","timestamp":1789776000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cancers"],"abstract":"<jats:p>Background: Primary cutaneous lymphomas (PCLs) represent a heterogeneous group of extranodal non-Hodgkin lymphomas with distinct clinical, histological, and molecular profiles. Recent advancements in genomic profiling have led to significant revisions in diagnostic frameworks and therapeutic paradigms. Objective: This review aims to summarize the current landscape of primary cutaneous B-cell and T-cell lymphomas and lymphoproliferative disorders (LPDs), focusing on the diagnostic updates in the WHO 5th edition and the International Consensus Classification (ICC 2022), alongside evolving risk-adapted treatment strategies. Methods: A comprehensive review of the recent literature and major international consensus guidelines was conducted, evaluating diagnostic shifts, disease reclassifications, and novel therapeutic interventions across various PCL subtypes. Results: The latest WHO 5th and ICC 2022 classifications emphasize the clinical behavior of indolent entities, shifting terminology from \u201clymphoma\u201d to \u201clymphoproliferative disorder\u201d for conditions such as primary cutaneous acral CD8+ T-cell LPD and primary cutaneous CD4+ small\/medium T-cell LPD to prevent overtreatment. Several previously provisional categories (e.g., primary cutaneous gamma\/delta T-cell lymphoma and EBV-positive mucocutaneous ulcer) have achieved definitive diagnostic status. Parallel to these revisions, therapeutic management has shifted toward toxicity-reducing, risk-adapted strategies. For indolent subtypes, ultra-low-dose radiotherapy (4 Gy) and skin-directed therapies remain highly effective. In aggressive or refractory subtypes, such as Mycosis Fungoides and primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL, LT), precision oncology\u2014utilizing targeted agents (e.g., brentuximab vedotin, mogamulizumab, lenalidomide, ibrutinib) and cellular\/immunotherapies\u2014is rapidly changing outcomes. Conclusions: Integrating the updated diagnostic classifications with modern risk-stratified and targeted treatment paradigms is crucial for optimizing clinical management and avoiding overtreatment in indolent PCLs while improving survival in aggressive subtypes.<\/jats:p>","DOI":"10.3390\/cancers18183038","type":"journal-article","created":{"date-parts":[[2026,9,21]],"date-time":"2026-09-21T12:50:03Z","timestamp":1789995003000},"page":"3038","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Primary Cutaneous Lymphomas and Lymphoproliferative Disorders: Current Insights for Classification and Management"],"prefix":"10.3390","volume":"18","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1852-8957","authenticated-orcid":false,"given":"U\u011fur","family":"Hatipo\u011flu","sequence":"first","affiliation":[{"name":"Department of Hematology and Bone Marrow Transplantation Center, University of Health Sciences, Ankara Oncology Training and Research Hospital, Ankara 06200, T\u00fcrkiye"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5083-6306","authenticated-orcid":false,"given":"Mert","family":"Seyhan","sequence":"additional","affiliation":[{"name":"Department of Hematology and Bone Marrow Transplantation Center, University of Health Sciences, Ankara Oncology Training and Research Hospital, Ankara 06200, T\u00fcrkiye"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hakan","family":"Eminoglu","sequence":"additional","affiliation":[{"name":"Department of Hematology and Bone Marrow Transplantation Center, University of Health Sciences, Ankara Oncology Training and Research Hospital, Ankara 06200, T\u00fcrkiye"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9332-663X","authenticated-orcid":false,"given":"Turgay","family":"Ula\u015f","sequence":"additional","affiliation":[{"name":"Department of Hematology and Bone Marrow Transplantation Center, University of Health Sciences, Ankara Oncology Training and Research Hospital, Ankara 06200, T\u00fcrkiye"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5994-2735","authenticated-orcid":false,"given":"Mehmet Sinan","family":"Dal","sequence":"additional","affiliation":[{"name":"Department of Hematology and Bone Marrow Transplantation Center, University of Health Sciences, Ankara Oncology Training and Research Hospital, Ankara 06200, T\u00fcrkiye"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,9,19]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1090","DOI":"10.1093\/bjd\/ljaf312","article-title":"European Organisation for Research and Treatment of Cancer, United States Cutaneous Lymphoma Consortium and International Society for Cutaneous Lymphomas consensus recommendations for management and treatment of cutaneous lymphoproliferative disorders","volume":"193","author":"Willemze","year":"2025","journal-title":"Br. 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