Primary cutaneous follicle centre lymphoma pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Sowminya Arikapudi, M.B,B.S. [2]

Overview

Primary cutaneous follicle center lymphoma is a tumor of neoplastic follicle centre cells, including centrocytes and variable numbers of centroblasts, with a follicular and diffuse growth pattern that generally presents on the head or trunk. This is the most common type of primary cutaneous B-cell lymphoma. Primary cutaneous follicle center lymphoma (PCFCL) may be associated with borrelia burgdorferi, hepatitis C, and human herpesvirus 8. On gross pathology,solitary or grouped erythematous papules, plaques and tumor lesions, mostly non-ulcerated are characteristic findings of primary cutaneous follicle center lymphoma. On microscopic histopathological analysis, centroblasts (large noncleaved cells), centrocytes (small and large cleaved cells), and reactive T cells are characteristic findings of primary cutaneous follicle center lymphoma.

Pathophysiology

Primary cutaneous follicle center lymphoma (PCFCL) is characterized by a proliferation of follicle center cells (centrocytes and centroblasts) with a follicular, follicular and diffuse, or diffuse growth pattern.

Genetics

Genes involved in the pathogenesis of primary cutaneous follicle centre lymphoma include:

• Amplification of C-REL gene
• BCL-2 rearrangements

Association

Primary cutaneous follicle center lymphoma (PCFCL) may be associated with:

• Borrelia burgdorferi
• Hepatitis C
• Human herpesvirus 8

Gross Pathology

On gross pathology, solitary or grouped erythematous papules, plaques and tumor lesions, mostly non-ulcerated are characteristic findings of primary cutaneous follicle center lymphoma.

Multinodular mass on the scalp (A) and spectacular response after the second chemotherapy course (B).

A 56-year old male patient with numerous erythematous and livid infiltrates before superficial radiotherapy.

A 56-year old male patient with numerous erythematous and livid infiltrates before superficial radiotherapy.

Microscopic Pathology

On microscopic histopathological analysis, centroblasts (large noncleaved cells), centrocytes (small and large cleaved cells), and reactive T cells are characteristic findings of primary cutaneous follicle center lymphoma.

The epidermis is preserved, with diffuse and nodular infiltrates of lymphoid cells.

The epidermis is preserved, with diffuse and nodular infiltrates of lymphoid cells.

Histopatological fingings. Biopsy of the mass of the scalp showing a dense and diffuse dermal infiltrate with nodular growth pattern (A) consisted of large atypical lymphocytes (H&E) (B), which are positive for CD20 (C) and Bcl6 (D) and negative for Bcl2 (E) on immunohistochemical staining. Bone marrow biopsy showing a hypercellular bone marrow with proliferation of megakaryocytes with hyperlobulated nuclei, sometimes in loose clusters (H&E) (F), positive for CD61 on immunohistochemical staining (G).

References

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