RESUMEN
Primary cutaneous CD30(+) lymphoproliferative disorders (CD30(+) LPDs) are the second most common form of cutaneous T-cell lymphomas and include lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma. Despite the anaplastic cytomorphology of tumor cells that suggest an aggressive course, CD30(+) LPDs are characterized by an excellent prognosis. Although a broad spectrum of therapeutic strategies has been reported, these have been limited mostly to small retrospective cohort series or case reports, and only very few prospective controlled or multicenter studies have been performed, which results in a low level of evidence for most therapies. The response rates to treatment, recurrence rates, and outcome have not been analyzed in a systematic review. Moreover, international guidelines for staging and treatment of CD30(+) LPDs have not yet been presented. Based on a literature analysis and discussions, recommendations were elaborated by a multidisciplinary expert panel of the Cutaneous Lymphoma Task Force of the European Organization for Research and Treatment of Cancer, the International Society for Cutaneous Lymphomas, and the United States Cutaneous Lymphoma Consortium. The recommendations represent the state-of-the-art management of CD30(+) LPDs and include definitions for clinical endpoints as well as response criteria for future clinical trials in CD30(+) LPDs.
Asunto(s)
Linfoma Anaplásico Cutáneo Primario de Células Grandes/terapia , Papulosis Linfomatoide/terapia , Estudios de Casos y Controles , Estudios de Cohortes , Unión Europea , Humanos , Antígeno Ki-1 , Linfoma Anaplásico Cutáneo Primario de Células Grandes/diagnóstico , Papulosis Linfomatoide/diagnóstico , Guías de Práctica Clínica como Asunto , Pronóstico , Sociedades Médicas , Estados UnidosAsunto(s)
Neoplasias Encefálicas/secundario , Neoplasias del Ojo/secundario , Linfoma de Células B Grandes Difuso/patología , Neoplasias Cutáneas/patología , Cuerpo Vítreo , Anciano , Neoplasias Encefálicas/terapia , Terapia Combinada , Neoplasias del Ojo/terapia , Resultado Fatal , Femenino , Humanos , Linfoma de Células B Grandes Difuso/química , Linfoma de Células B Grandes Difuso/terapia , Estadificación de Neoplasias , Neoplasias Cutáneas/química , Neoplasias Cutáneas/terapia , VitrectomíaRESUMEN
BACKGROUND: Granulomatous cutaneous T-cell lymphomas (CTCLs) are rare and represent a diagnostic challenge. Only limited data on the clinicopathological and prognostic features of granulomatous CTCLs are available. We studied 19 patients with granulomatous CTCLs to further characterize the clinicopathological, therapeutic, and prognostic features. OBSERVATIONS: The group included 15 patients with granulomatous mycosis fungoides (GMF) and 4 with granulomatous slack skin (GSS) defined according to the World Health Organization-European Organization for Research and Treatment of Cancer classification for cutaneous lymphomas. Patients with GMF and GSS displayed overlapping histologic features and differed only clinically by the development of bulky skin folds in GSS. Histologically, epidermotropism of lymphocytes was not a prominent feature and was absent in 9 of 19 cases (47%). Stable or progressive disease was observed in most patients despite various treatment modalities. Extracutaneous spread occurred in 5 of 19 patients (26%), second lymphoid neoplasms developed in 4 of 19 patients (21%), and 6 of 19 patients (32%) died of their disease. Disease-specific 5-year survival rate in GMF was 66%. CONCLUSIONS: There are clinical differences between GMF and GSS, but they show overlapping histologic findings and therefore cannot be discriminated by histologic examination alone. Development of hanging skin folds is restricted to the intertriginous body regions. Granulomatous CTCLs show a therapy-resistant, slowly progressive course. The prognosis of GMF appears worse than that of classic nongranulomatous mycosis fungoides.