Primary Cutaneous Follicle Center Lymphoma of the Eyelid: A Case Report and Review of the Literature in Light of Recent Changes to the WHO Classification of Lymphoid Neoplasms

Abstract

Primary cutaneous follicle center lymphoma (PCFCL) is a unique entity that represents up to 11% of all cutaneous lymphomas. PCFCL is associated with an indolent course and excellent 5-year survival rates, but can progress to secondary systemic involvement if left untreated. Histopathologic features of PCFCL can vary depending on the size, duration, and clinical stage of the lesion, making diagnosis somewhat challenging. Here, we present a case of a 50-year-old woman with an eyelid lesion that was initially classified as an inflamed cyst based on biopsy, but 1 year later, was determined to be PCFCL after repeat biopsy revealed different histology. In light of the recent changes to the WHO classification of lymphoid neoplasms, we review the unique clinical and histopathologic features of PCFCL that distinguish it from other more aggressive forms of cutaneous lymphoma in terms of course, prognosis, and management.

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

“…Both DLBCL and PCFCL are infrequent type of lymphoma in the ocular adnexa, with a prevalence ranging from 7% to 11.4% in multiple series for DLBCL, 3,[6][7][8] and few reports for PCFCL. 9 DLBCL represents a primary disease in 38%-54% 10,11 of patients and has a high mortality rate, with a 5-year survival of 36%-52.6%. 6,11 PCFCL represents 11% of cutaneous lymphomas, has a more indolent course, and has a better prognosis with 5-year survival rates of 95%.…”

“…11,16 PCFCL cases are not reported in these series; we found no reported incidence of primary follicle center lymphoma of the eyelid, with only 1 other case reported in the literature. 9 A recent systematic review found 199 eyelid lymphomas, of which 18 cases were DLBCL, 10 were follicular lymphoma and none was PCFCL. 16 In our case, the 4-week duration of symptoms before the presentation is also a salient feature.…”

Eyelid involvement of primary cutaneous lymphomas: A clinical perspective

“…Infiltrated, ill‐defined erythematous patches or flat plaques surrounding a large nodule or tumour represent a distinctive appearance of this PCL 8,9 . Eyelid involvement as solitary non‐ulcerated nodules was reported in two patients of whom one was 11 years old 65,66 …”

Primary cutaneous follicle center lymphoma of the breast: Management of an exceedingly rare malignancy

“…Temporary resolution of the skin changes with or without topical treatment may occur, resulting in a delayed biopsy. A repeated biopsy should be considered in selected cases with discordance between clinical and pathological findings (9). In our patient, the biopsy was performed with a seven-month delay, as the tumor had been initially misdiagnosed as a benign lesion at another institution.…”

“…On immunohistochemical analysis, the neoplastic lymphocytes express B-cell markers such as CD19, CD20, CD22, CD79A, and PAX5 and at least one follicle center marker which is BCL-6 and less commonly CD10 (8,9).…”