DUSP22 rearrangement in primary cutaneous T cell lymphoma with features intermediate between mycosis fungoides, anaplastic large-cell lymphoma and lymphomatoid papulosis

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Background/Objectives: Primary cutaneous lymphomas are rare in pediatric patients. The clinical and histopathological manifestations may differ from those in adults. Due to their low frequency and the insidious clinical picture, the diagnosis is usually delayed. The Spanish Primary Cutaneous Lymphoma Registry was initiated in 2016 as a multicenter registry that would allow better insight into the epidemiological, clinical, histopathological, and treatment response characteristics of patients with primary cutaneous lymphomas. Methods:We conducted a prospective observational cohort study of primary cutaneous lymphomas in pediatric patients participating in the Spanish Academy of Dermatology and Venereology (AEDV) Primary Cutaneous Lymphoma Registry.Results: At the time of the analysis, 10 patients under 18 years of age out of 799 allage cases (1.25%) had been included in the registry (7 males, 3 females). The mean age at diagnosis was 9.7 years (SD: 4.8). Seven (70%) had mycosis fungoides, 2 of them had the folliculotropic variant; and 3 (30%) had primary cutaneous marginal zone B-cell lymphoma.Conclusions: Primary cutaneous lymphomas are extremely rare in pediatric patients and usually have a good prognosis. Therefore, a high level of suspicion is necessary for the diagnosis. We suggest management by experienced physicians and follow-up into adulthood.

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Background: Mycosis fungoides is rarely associated to B-cell malignancies, and the few reported cases are mainly internal lymphomas involving secondarily the skin (ie, chronic lymphocytic leukemia). Objectives:The aim of our study is to describe the clinical and histopathological features of 4 patients presenting with 2 concurrent primary cutaneous lymphomas and review the pertinent literature. Methods:We identified 4 cases of concurrent primary cutaneous lymphomas in our institutions. An extracutaneous lymphoma was ruled out on the basis of a complete work out. We performed a PubMed search to identify reported cases of primary cutaneous composite or concurrent lymphomas.Results: Eleven cases of primary cutaneous concurrent lymphomas have been described in the literature. Counting all together (our cases and the cases previously described in the literature), mycosis fungoides was the most frequent primary cutaneous T-cell lymphoma (TCL) (13/15), followed by 1 case of peripheral TCL-NOS and 1 case of subcutaneous panniculitis-like TCL. Regarding the associated primary cutaneous B-cell lymphomas, 8/15 cases consisted of low-grade B-cell lymphomas [that is, 5 marginal zone lymphoma (in the most recent classification reclassified as marginal zone lymphoproliferative disorder, MZLD, 2 follicular-center B-cell lymphoma (primary cutaneous follicle-center lymphoma) and 1 lowgrade NOS B-cell lymphoma]; 4/15 were associated to Epstein-Barr virus; 1 case consisted of a methotrexate-associated lymphoproliferative disease, and 2 cases consisted of primary cutaneous diffuse large B-cell lymphoma-leg type.Conclusions: Primary cutaneous concurrent lymphomas are exceptional. Clinicopathological correlation and a complete workout to reach the correct diagnosis may guide the appropriate treatment in each case.