Case Report
Primary cutaneous follicular center cell lymphoma of the nose with maxillary sinus involvement in a pediatric patient

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We report a primary cutaneous follicular center cell lymphoma (PCFCCL) that arose on the nose and the left naso-labial fold, spread to the left cheek, the left maxillary sinus, and the soft palate in a 16-year-old boy. Polychemotherapy was performed and the patient is disease-free after 41 months. This case is unusual because PCFCCL rarely arises on the nose, only occasionally disseminates to extracutaneous sites, and, to our knowledge, has never been described in pediatric patients.

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Case report

In August 2000, a 16-year-old boy had an erythematous nodule on the left nasal ala and on the left naso-labial fold. Four years before, the cutaneous lesion had been diagnosed at another hospital as B-cell pseudolymphoma, with the histologic features of lymphoid hyperplasia. Molecular studies revealed polyclonality of immunoglobulin heavy chain (IgH) gene and T-cell receptor (TCR) gene, and cranial computed tomography (CT) was negative. The tumor had been surgically removed 4 times with

Discussion

In the pediatric age the most frequent lymphomas are Burkitt's lymphoma, lymphoblastic B-cell lymphoma, and CD30+ anaplastic large cell lymphoma,3, 4 which arise in extracutaneous sites and may secondarily involve the skin. Primary cutaneous lymphomas, except CD30+ disorders,5 mycosis fungoides,6 and panniculitis-like T-cell lymphoma,7 are rare in children and PCFCCL has never been described, to our knowledge.

PCFCCL has been reported as an indolent neoplasm of B-cell origin, which rarely

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    It is also male predominant and tends to occur in middle-aged and older patients, with a median age in the 50s.14–16 It rarely occurs in children, with only 3 previously reported pediatric cases.35–38 Patients with PCFCL present with either single or multiple papules, plaques, or tumors with pink to purple coloration and may display peripheral erythema; surrounding plaques and papular lesions may also be seen (Fig. 3).

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    Primary cutaneous follicle center lymphoma accounts for approximately 50–60% of primary cutaneous B-cell lymphomas5,7,8 and occurs in middle aged-to older men and women, with a cited median age generally in the 50 s,7–9,11 and a slight male predominance in some studies.8 The disease is extremely rare in children, with only three cases reported in the literature;32–35 however, it can be seen in young adults.36 Patients usually present with erythematous papules, plaques or tumors most commonly on the head, neck or trunk (Fig. 1), although any site can be involved, including the leg.

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This supplement is made possible through the generous support of Stiefel Laboratories for the American Academy of Dermatology.

Funding sources: None.

Conflicts of interest: None identified.

The case has been presented at the 1st National Congress of Dermatology and Venereology ADOI-SIDEV June 2001, Rome, Italy.

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