Patients are adults of both genders. Cutaneous follicle centre cell lymphoma presents clinically with erythematous papules, plaques and tumours, usually non-ulcerated. Lesions are located mostly on the head and neck and on the trunk (Figs 9.1-9.6). A distinct clinical presentation with plaques
and tumours on the back surrounded by erythematous macules and papules expanding centrifugally around the central tumours has been described in the past as 'reticulo-histiocytoma of the dorsum' or 'Crosti's lymphoma' (Fig. 9.5) [24,25].
Lesions are usually clustered at a single site but may be multiple at different sites. Although there are no clear-cut differences in clinical presentation between the diffuse and follicular variants of cutaneous follicle centre cell lymphoma, cases with a follicular pattern have a predilection for the head and neck region, whereas the so-called Crosti's lymphoma corresponds in the majority of cases to a follicle centre cell lymphoma with a diffuse pattern of growth [18,24].
In some patients with the clinical presentation of Crosti's lymphoma, small erythematous papules located far from the main lesions can be observed (Fig. 9.7). These papules represent early manifestations of the disease and reveal histo-pathologically specific features of follicle centre cell lymphoma (Fig. 9.8). The question arises as to whether local
Fig. 9.6 Cutaneous follicle centre cell lymphoma. Erythematous papules and plaques on the back (early lesions of 'Crosti's lymphoma')
radiotherapy is the more appropriate treatment modality for these patients, and what the radiation field should be. The relatively high incidence of local recurrences observed in Crosti's lymphoma may be caused, at least in part, by the presence of early lesions far from the main tumour, which had not been identified clinically at the time of the planning of treatment.
Association with infections such as Borrelia burgdorferi, hepatitis C or human herpesvirus 8 has been described in sporadic patients but does not seem to be a major aetiological factor for cutaneous follicle centre cell lymphoma [26-29].
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