Epidemiology of cutaneous lymphomas

Primary cutaneous lymphomas represent the second most frequent type of extranodal non-Hodgkin lymphomas (NHLs) after gastrointestinal lymphomas [9]. The worldwide annual incidence of primary cutaneous lymphomas is estimated to be 1:100,000 [1, 6, 9]. Despite the existence of several large, often clinic-based registries dealing with cutaneous lymphomas (Netherlands Lymphoma Registry, Lymphoma Registry Graz/Austria, Lymphoma Registry Stanford/USA, German Registry for Cutaneous Lymphomas, National Cancer Institute’s Surveillance, Epidemiology, and End Results Program (NCI SEER)) there is a paucity of comparable population-based data concerning this group of diseases. Until 2005, one of the main obstacles for the correct reporting was the lack of appropriate classification to cover all clinicopathological entities, when the new WHO-EORTC was introduced [23]. The comparison of age distribution in different lymphoma registries shows that primary cutaneous lymphomas most commonly arise in older adults, with a median onset time after 60 years of age [1]. One exception to this distribution is lymphomatoid papulosis, which often develops in young adults and can even manifest in childhood [2]. Males seem to be more affected than females, with a male/female ratio reaching up to 4:1 [7, 11, 27]

Cutaneous lymphoma, leukemia and related disorders

Mycosis fungoides (MF) is a general indolent peripheral T-cell lymphoma initially and preferentially present in the skin and showing distinct clinical, histological (except in early stages), immunophenotypical, and genotypical features. It is the most common cutaneous lymphoma [75, 100], characterized by the sequential appearance of patches, developing into plaques and finally into tumors, which tend to ulcerate

Evaluation of lymphangiogenic markers in Sézary syndrome

Sézary syndrome (SS) is regarded as a leukemic, aggressive subtype of cutaneous T-cell lymphoma (CTCL) characterized by the accumulation of malignant T-cells in the skin, as well as by blood and lymph node involvement. To date there have been no data on the extent of lymphangiogenesis in SS or erythrodermic mycosis fungoides (eMF). Lymphangiogenesis represents the de novo formation of lymphatic vasculature and has been associated with the occurrence of metastatic disease and poor prognosis. In this study we investigated lymphangiogenesis in skin biopsies from patients with SS and eMF. The expression of VEGFR-3 was significantly higher in patients with SS (p = 0.0285) as compared to patients with eMF. LYVE-1, podoplanin (PDPN), and VEGF-C stainings showed a similar tendency. The number of PDPN-expressing lymphatic vessels (p = 0.025) as well as CD31-positive blood vessels (p = 0.0065) correlated with disease progression in patients with SS. We show for the first time a non-vascular pattern of VEGF-C and VEGFR-3, i.e. their epidermal expression in erythrodermic CTCLs, suggesting their role in lymphocyte trafficking to the skin

Non-melanoma skin cancer

Even though surgery represents the gold standard for the treatment of many types of non-melanoma skin cancer, medical treatment options have been developed for superficial tumors and actinic keratoses. Drug treatment for non-melanoma skin cancer encompasses topical use of 5-fluorouracil, diclofenac, retinoids, and imiquimod. For patients who are not candidates for surgical excision or who are not amenable to surgery, intralesional injections using interferons may be a therapeutic option (see Chap. 4.1.3)

Efficacy and safety of oral alitretinoin in severe oral lichen planus - results of a prospective pilot study

BACKGROUND: Patients with severe oral lichen planus refractory to standard topical treatment currently have limited options of therapy suitable for long-term use. Oral alitretinoin (9-cis retinoic acid) was never systematically investigated in clinical trials, although case reports suggest its possible efficacy. OBJECTIVES: To assess the efficacy and safety of oral alitretinoin taken at 30 mg once daily for up to 24 weeks in the treatment of severe oral lichen planus refractory to standard topical therapy. METHODS: We conducted a prospective open-label single arm pilot study to test the efficacy and safety of 30 mg oral alitretinoin once daily for up to 24 weeks in severe oral lichen planus. Ten patients were included in the study. Primary end point was reduction in signs and symptoms measured by the Escudier severity score. Secondary parameters included pain and quality of life scores. Safety parameters were assessed during a follow-up period of 5 weeks. RESULTS: A substantial response at the end of treatment, i.e. >50% reduction in disease severity measured by the Escudier severity score, was apparent in 40% of patients. Therapy was well tolerated. Adverse events were mild and included headache, mucocutaneous dryness, musculoskeletal pain, increased thyroid-stimulating hormone and dyslipidaemia. CONCLUSIONS: Alitretinoin given at 30 mg daily reduced disease severity of severe oral lichen planus in a substantial proportion of patients refractory to standard treatment, was well tolerated and may thus represent one therapeutic option for this special group of patients

Systemic corticosteroids for subcutaneous panniculitis-like T-cell lymphoma

BACKGROUND Primary cutaneous γ/δ T-cell lymphoma (PCGD-TCL) is aggressive and has a poor prognosis. In contrast, subcutaneous panniculitis-like T-cell lymphoma (SPTCL) of the α/β T-cell receptor phenotype is known to follow an indolent course and have a more favourable prognosis. In the past, PCGD-TCL and SPTCL were often considered to be a manifestation of the same disease, and aggressive systemic polychemotherapy has commonly been the first-line therapy for both. Given the understanding that SPTCL is a separate and less aggressive entity, clinical data exclusively evaluating the efficacy of conservative treatment in SPTCL are needed. OBJECTIVES To assess the overall clinical response to systemic corticosteroids in the treatment of SPTCL. METHODS This was a retrospective cross-sectional study based on a patient data repository from two tertiary care university hospitals in Zürich (Switzerland) and Tübingen (Germany). The repository spanned 13 years. RESULTS In four of the five patients (80%) with SPTCL, treatment with systemic corticosteroids induced a complete remission. CONCLUSIONS Systemic corticosteroids may be an excellent first-line single-agent therapy for SPTCL

Radiotherapy as a Treatment Option for Local Disease Control in Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type.

Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL, LT) is an aggressive lymphoma variant. Anthracycline-based chemotherapy with rituximab is recommended as first-line treatment. Radiotherapy (RT) has been considered as a therapeutic option for local disease control in patients with solitary or localized lesions. We report the results of a retrospective analysis of PCDLBC, LT patients treated either with RT alone or with physician's decision as first-line treatment, aiming to assess disease progression and/or first recurrence in these treatment groups. We retrospectively analyzed 20 patients treated either with RT alone (n = 8) or with investigator's choice treatment (n = 12), which included chemotherapy alone or combined with local therapy (RT and wide local excision). Complete response (CR) was achieved in 8 patients from the first group and 9 patients from the second group, with 1 treatment failure. Six patients treated with RT alone progressed with a median time to progression (TTP) of 12.5 months. In the second group, 5 patients progressed with a median TTP of 5.2 months. RT showed good local disease control in both groups without any skin relapses during the follow-up period. RT as first-line monotherapy followed by watchful waiting did not significantly improve the overall risk of disease progression but resulted in good local disease control. After progression, RT could still easily be combined with systemic treatment. The strength of this analysis needs to be evaluated in a larger patient cohort