Primary cutaneous follicle center lymphoma in a 16‐year‐old girl

Abstract

In the pediatric and adolescent age group, primary cutaneous lymphomas are rare, especially cutaneous B‐cell lymphomas. According to the World Health Organization, the three main subtypes of primary cutaneous B‐cell lymphomas are primary cutaneous marginal zone B‐cell lymphoma (PCMZL), primary cutaneous follicle center lymphoma (PCFCL), and primary cutaneous diffuse large B‐cell lymphoma, leg type. We present an extraordinary case of PCFCL arising in a 16‐year‐old female, only the sixth case of PCFCL to be reported in the literature in a patient less than 20 years old. Although PCMZL was considered in this case, the finding of lambda light chain restriction in the BCL‐6 and CD10 positive population of lymphocytes established the diagnosis of primary cutaneous follicle center lymphoma. Not many data currently exist on the prognosis of PCFCL in young individuals, but adult PCFCL has a good prognosis with an indolent course and 5‐year survival rates over 95%. Because of its uncommon manifestation in young patients, the diagnosis of PCFCL is often delayed or missed. This case is presented to raise awareness of PCFCL in the pediatric/ adolescent population and to contribute to the ongoing research of this condition.

Primary Cutaneous Follicle Center Lymphoma of the Eyelid in a Young Man

“…al. 15 state that there have only been 6 cases of this entity in patients younger than 20 years, the youngest one being 8 years old, and none of them reported an eyelid lesion.…”

“…al. 15 state that there have only been 6 cases of this entity in patients younger than 20 years, the youngest one being 8 years old, and none of them reported an eyelid lesion. Some OAL series do not report cases of eyelid DLBCL, 3,7,10 while others have reported this histologic type in 5.4%-9% of eyelid lymphomas.…”

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

“…3 In this age group, PCFCL is exceptionally rare with only 12 cases having been reported in the literature (Table 1). 1,[3][4][5][6][7][8][9] In this study, we report a case of PCFCL in a 16-year-old girl with unusual clinical and immunohistochemical presentation.…”

“…3 Pediatric PCFCL seems to be extremely rare with only 12 individuals less than 20 years of age reported in literature (Table 1). 1,[3][4][5][6][7][8][9] The underlying pathogenesis of PCFCL is not well understood and has been linked to chronic antigenic viral stimulation including Epstein Barr virus (EBV), Human Herpes Virus type 8 (HHV8) and Hepatitis B virus (HBV). 12 Primary cutaneous follicle center lymphoma (PCFCL) presents clinically as a solitary or grouped of plaques or nodules with a predilection of scalp, forehead, and trunk.…”

“…3 Follow up of the twelve published cases of pediatric PCFCL showed 9 cases were alive and disease free over a range of 42 weeks, one case disease recurred at 13 months follow-up, the remaining two patients' disease course was not reported (Table 1), our patient was referred to a specialist hospital for further management. 1,[3][4][5][6][7][8][9]…”