Primary cutaneous lymphomas (PCLs) are a heterogenous group of non-Hodgkin lymphomas arising in the skin from T- or B-lymphocytes, for which there is limited epidemiological data available. To describe the disease characteristics and estimate annual incidence rates (IRs) and temporal trends of PCLs and their subtypes in Attica, Greece. A retrospective analysis of all PCL patients, diagnosed in Attica\'s main haemopathology referral centre from 2009 to 2021, was conducted. In total, 1,189 patients were included; 725 males and 464 females (males__females=1.56). The median age at diagnosis was 62 years. The annual IR was 2.2 new cases per 100,000 individuals. Most patients (n=979, 82.3%) were diagnosed with cutaneous T-cell lymphoma (CTCL) with a crude IR of 1.8 new cases per 100,000 person-years. Mycosis fungoides (MF) was the most common subtype (n=817, 68.7%), followed by lymphomatoid papulosis (LyP) (n=59, 5.0%). The crude IR for MF was 1.5 new cases per 100,000 person-years. Cutaneous B-cell lymphomas (CBCLs) accounted for 17.6% (n=210) of all PCLs (IR: 0.4 new cases per 100,000 person-years). PCL, CTCL and MF incidence rates increased from 2009 to 2019, followed by a decrease in 2020-2021. The incidence rate of CBCL increased steadily during the study period. The annual IRs of PCL in Greece were higher than those reported in other studies from Europe, America and Asia. The increase in IRs from 2009 to 2019 may reflect physicians\' improved diagnostic efficiency. The COVID-19 pandemic may be the reason for the decline in PCL, CTCL and MF diagnoses from 2020 to 2021.

Eyelids may be affected in systemic, ocular adnexal and primary cutaneous lymphomas (PCLs). The frequency of eyelid involvement in PCLs is still not well known and it is not a predilection site for any type. While primary cutaneous T-cell lymphomas (CTCLs) are more commonly seen than primary cutaneous B-cell lymphomas (CBCLs), especially mycosis fungoides (MF) as by far the most frequent type, B cell lymphomas are reported to be the commonest type in eyelid localization on the contrary. PCLs may be located on the eyelids, as the sole manifestation or in association with the involvement of other parts of the eye and elsewhere of the body. MF may present with a rich spectrum of clinical features on the eyelids mostly seen in folliculotropic subtype and advanced-stage disease. Erythematous scaly patches or plaques representing the most commonly encountered eyelid MF lesions may mimic many other dermatological conditions. Diffuse thickening, oedema, poikilodermic changes, atrophy and wrinkling are other suggestive findings of eyelid MF. Milia-like papules, madarosis and ectropion are also seen in the folliculotropic variant of MF, as ectropion is more typical for Sezary syndrome. Eyelids are also a typical location for tumoural MF which has been suggested as a poor prognostic indicator in MF. Papulonodular lesions, large tumours, ulceration, diffuse infiltration, oedema and subcutaneous atrophy on the eyelids may also be seen in other types of PCLs. Keep in mind, the rich clinical spectrum of PCLs on the eyelids may be crucial in early diagnosis in this special localization.

Primary cutaneous anaplastic large cell lymphoma (PCALCL) is a subtype of non-Hodgkin lymphoma (NHL) that is localized to the skin. Disseminated disease is rare, and visceral organ involvement is even more so. We report a unique case of PCALCL with gastric metastasis. A 75-year-old man with a history of cutaneous left lower extremity PCALCL status post radiation therapy initially presented with abdominal pain and was found to have diffuse celiac axis and retroperitoneal lymphadenopathy. Endoscopy, initially done to biopsy an involved lymph node (LN), demonstrated a friable gastric nodular lesion with telangiectasias. Biopsy of the lesion and LN revealed anaplastic large cell lymphoma, identical in pathology to the known skin lesion. The patient was treated with systemic chemotherapy with a good response. PCALCL has been thought of as a localized malignancy with a good prognosis and low potential for extracutaneous spread. To our knowledge, this is the first instance of metastatic PCALCL involving the stomach.

The Coronavirus disease 2019 (COVID-19) pandemic spreads quickly all over the world. There are no sufficient data in the literature about COVID-19 infection and cutaneous lymphomas. This review sheds the light on what is known so far about COVID-19 with a cutaneous lymphoma perspective. Cutaneous T-cell lymphoma (CTCL) diagnosis does not represent a predisposing factor to viral infections and most of CTCL patients have indolent disease. However, physicians should be cautious with patients with aggressive primary cutaneous lymphomas and advanced CTCL. Different treatment strategies for cutaneous lymphomas should be taken into consideration during the COVID-19 pandemic. Thus, it is highly needed to estimate the benefit-to-risk ratio on a case-by-case basis.

Time to next treatment (TTNT) is an emerging endpoint in clinical studies of primary cutaneous T-cell lymphomas (CTCL), with utility as a surrogate marker for the \"duration of clinical benefit\". TTNT provides a highly clinically meaningful endpoint that uniquely reflects not only the duration of treatment efficacy on disease and symptom control, but also incorporates the patient experience by accounting for patient compliance and tolerance to the studied therapy(s). Given the distinct challenges of pin-pointing the exact date of progression in patients with multi-compartmental CTCL, TTNT overcomes many of the shortcomings of conventional, disease-focused, clinical endpoints in primary CTCL research. Although widely accepted in clinical research for numerous other incurable malignancies, TTNT currently lacks a standardised definition. In this paper, we describe the value of TTNT as a clinical endpoint, review the applications of TTNT in primary CTCL research, and propose a standardised definition of TTNT to be applied in future clinical research of primary CTCL therapies.

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Therapeutic progress in primary cutaneous lymphomas continues to be largely dominated by the T-cell lymphomas, towards which the great majority of recent therapeutic innovations have been directed. The latter include local treatments consisting either of relatively classical but \"revamped\" approaches involving different pharmaceutical forms (example: chlormethine gel) or else lower but seemingly equally effective dosages (electron therapy), or of more innovative approaches (example: UVA-1, dynamic phototherapy, imiquimod, resimiquimod). However, significant progress has been made chiefly in terms of systemic treatments with the emergence of \"targeted\" drugs that directly and specifically target tumour cells (monoclonal antibodies directed against CD30, CCR4 or CD158k) and the further development of \"small\" molecules such as histone deacetylase inhibitors and new cytostatics. Immunotherapies, which have proven so effective in other areas of oncodermatology, are also of great interest, while allogeneic haematopoietic stem-cell transplantation has clearly shown its superiority over autologous transplantation and now constitutes a significant component of the therapeutic arsenal in advanced disease. While the innovations in terms of B-cell lymphomas are certainly less significant, mention must also be made of the value of rituximab combined with polychemotherapy (CHOP) and of lenalidomide (as second-line therapy) in primary cutaneous diffuse large B-cell lymphoma, leg type, along with the development of localized (very) low-dose radiotherapy in unilesional or paucilesional indolent forms.

BACKGROUND: Nonmycosis fungoides/Sézary syndrome (non-MF/SS) primary cutaneous lymphomas (PCL) are currently categorized under the 2005-World Health Organization/European Organization for Research and Treatment of Cancer (WHO-EORTC) classification for PCL. These differ in behavior from secondary cutaneous lymphomas (SCL) and to lymphomas limited to the oral cavity (primary oral lymphomas [POL]) both categorized under the 2016-WHO classification for lymphoid neoplasms.
OBJECTIVE: This study aims to report the first series of non-MF/SS PCL, SCL, and POL in a Mexican cohort, examine the applicability of current classification systems and compare our findings with those from foreign cohorts.
METHODS: Eighteen non-MF/SS PCL, four SCL, and two POL with available tissue for morphology and immunophenotypic assessment were reclassified according to the 2005-WHO/EORTC and 2016-WHO classifications.
RESULTS: Non-MF/SS PCLs were primarily of T-cell origin (61%) where CD30+ lymphoproliferative disorders predominated, followed by Epstein-Barr virus-induced lymphomas, and peripheral T-cell lymphomas, not otherwise specified. Primary cutaneous B-cell lymphomas (BCL) were primarily of follicle center cell origin followed by postgerminal lymphomas of the diffuse large BCL variety.
CONCLUSIONS: Most non-MF/SS PCL, SCL, and POL can be adequately categorized according to the 2005-WHO/EORTC and 2016-WHO classification systems, even when dealing with clinically atypical cases. The relative frequencies in our cohort hold closer similarities to Asian registries than from those of Europe/USA, supporting the concept of individual and/or racial susceptibility, and the notion of geographical variances in the rate of lymphomas. In particular, such disparity may arise from viral-induced lymphomas which might show partial geographical restriction.

The primary cutaneous lymphomas are a heterogeneous group of T-, Natural Killer- and B- cell neoplasms with a wide range of clinical and pathological presentations, and with very different prognoses compared to systemic lymphomas. Recent studies have shown that the skin microenvironment, which is composed of various immune cell subsets as well as their spatial distribution and T-cell interactions through different chemokines and cytokines, has an important role in the development and pathogenesis of cutaneous lymphomas and has assisted in the development of novel and more effective immunotherapies. The following review will focus on the major subtypes of primary cutaneous lymphomas, including the clinical and histological patterns, molecular hallmarks, and current and future treatment strategies.

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