Primary Cutaneous Follicle Center Lymphoma Presenting as a Solitary Nodule on the Forearm of an Adolescent Girl: A Case Report and Literature Review

Figures & data

Table 1 Summary of the Clinical Information on Reported Cases of Pediatric and Adolescent PCFCL (Less Than 20 Years of Age)

Case No.	Age	Sex	Lesion Site and Description	Immunohistochemical Result	Treatment	Follow Up and Duration of Remission	Reference
1	20 years	F	Not reported	Not reported	Not reported	Alive and recurrent at 13 months follow up.	Regina Fink-Puchws et al^Citation1 (2004)
2	16 years	M	Smooth surfaced erythematous nodule with hard-elastic consistency. Nose and left nasolabial fold, spread to the left cheek, left maxillary sinus and soft palate.	CD20, CD79a, BCL6 positive. CD5, CD10, CD30, BCL2, CD21 negative.	6 cycles of polychemotherapy (cyclophosphamide, doxorubicin, etoposide, prednisone, cytarabine, bleomycin, vincristine, and methotrexate)	Alive and disease free at 41 months follow up.	Massimo Ghislanzoni et al^Citation4 (2005)
3	8 years	M	Firm, alopecic, skin colored, smooth nodule. Right frontal scalp.	CD20, CD10, CD21 positive. BCL2, CD30 negative.	Surgical excision	Alive and disease free at 48 months follow up.	Tania Condarco et al^Citation5 (2008)
4	17 years	F	Localized annular plaque. Scalp.	CD20, BCL6, BCL2, CD30 positive. CD10 negative.	Surgical excision and electron beam therapy	Alive and disease free at 25 months follow up.	Amitay-Laish et al^Citation6 (2009)
5	16 years	M	Rubbery, mobile nodule. Left parietal scalp.	CD20, BCL6 positive. BCL2 negative.	Surgical excision	Not reported	Wael N. Sayaj et al^Citation7 (2019)
6	16 years	F	Indurated erythematous plaque. Posterior arm.	CD20, BCL6, CD10 positive. BCL2, CD30 negative.	Not reported	Not reported	Nicole Edmonds et al^Citation8 (2020)
7–11	<20 years	3 M 2 F	Head and neck.	Not reported	Not reported	Alive during a median follow up of 48 months	David Bomze et al^Citation3 (2021)
12	11 years	M	Medial canthus of the right eye.	CD20, BCL6, CD10, BCL2 positive.	Surgical excision	Alive and disease free at 24 months follow up.	Paul D’Alessandro et al^Citation9 (2022)

Figure 1 A solitary non scaly erythematous nodule over the left forearm with a firm consistency measuring 1.5 cm x 1 cm.

Figure 2 (A) A dense dermal infiltrate that shows a follicular growth pattern sparing the epidermis and separated by a Grenz zone (arrow) (H&E, 4x). (B) The infiltrate focally extends to the subcutaneous fat (arrow) (H&E, 4x). (C and D) A predominance of small cells with irregular, elongated or angulated nuclei with inconspicuous nucleoli. Larger cells with more rounded nuclei, open chromatin, and peripheral nucleoli are demonstrated (H&E, 40x).

Figure 3 (A) The neoplastic cells are diffusely and strongly positive for CD20 (4x). (B) They showed variable staining for BCL6 (40x). (C) Uniquely, the neoplastic cells in this case were diffusely positive for BCL2 (4x). (D) CD21 highlights expanded follicular dendritic meshwork (4x).

Fink-Puches R, Chott A, Ardigó M, et al. The spectrum of cutaneous lymphomas in patients less than 20 years of age. Pediatr Dermatol. 2004;21(5):525–533. doi:10.1111/j.0736-8046.2004.21500.x

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Ghislanzoni M, Gambini D, Perrone T, Alessi E, Berti E. Primary cutaneous follicular center cell lymphoma of the nose with maxillary sinus involvement in a pediatric patient. J Am Acad Dermatol. 2005;52(5 Suppl 1):S73–S75. doi:10.1016/j.jaad.2004.05.025

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Sayej WN, Isakoff MS, DiGiuseppe JA, et al. Primary cutaneous follicle center lymphoma in a 16-year-old boy with Crohn's disease exposed to infliximab and methotrexate. J Pediatr Gastroenterol Nutr. 2020;70(2):e49–e50. doi:10.1097/MPG.0000000000002578

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Edmonds N, Hernández-Pérez M, Holsinger M, Gru AA. Primary cutaneous follicle center lymphoma in a 16-year-old girl. J Cutan Pathol. 2021;48(5):663–668. doi:10.1111/cup.13939

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D’Alessandro PR, Lo AC, Spencer MH, et al. Primary cutaneous follicle center lymphoma of the medial canthus of the eye in an 11-year old. Pediatr Blood Cancer. 2022;69(9):e29630. doi:10.1002/pbc.29630

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