Clinical features

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

Fig. 9.1 Cutaneous follicle centre cell lymphoma. Clustered tumours on the scalp.
Fig. 9.2 Cutaneous follicle centre cell lymphoma (early lesions). Two papular lesions on the scalp.
Fig. 9.3 Cutaneous follicle centre cell lymphoma (early lesions). Clustered papules on the right eyebrow.

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. 9.4 Cutaneous follicle centre cell lymphoma. Large erythematous tumour on the shoulder. Note infiltrated erythematous patches and papules in the surroundings.
Fig. 9.5 Cutaneous follicle centre cell lymphoma. Large erythematous tumours surrounded by plaques and papules covering a large area of the back (so-called 'reticulohistiocytoma of the dorsum', 'Crosti's lymphoma').

Fig. 9.6 Cutaneous follicle centre cell lymphoma. Erythematous papules and plaques on the back (early lesions of 'Crosti's lymphoma')

  1. 9.7 Cutaneous follicle centre cell lymphoma. Large tumours on the left side of the back. Note small papules located at the right paravertebral area (arrow).
  2. 9.6 Cutaneous follicle centre cell lymphoma. Erythematous papules and plaques on the back (early lesions of 'Crosti's lymphoma')
  3. 9.7 Cutaneous follicle centre cell lymphoma. Large tumours on the left side of the back. Note small papules located at the right paravertebral area (arrow).

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.

  1. 9.8 Cutaneous follicle centre cell lymphoma, early lesion. (a) Histology reveals small nodular infiltrates throughout the dermis. (b) Cytomorphology shows small lymphocytes admixed with centrocytes and centroblasts.
  2. 9.8 Cutaneous follicle centre cell lymphoma, early lesion. (a) Histology reveals small nodular infiltrates throughout the dermis. (b) Cytomorphology shows small lymphocytes admixed with centrocytes and centroblasts.

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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