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Primary Cutaneous Follicle Center Lymphoma | Basicmedical Key

Primary Cutaneous Follicle Center Lymphoma

Primary Cutaneous Follicle Center Lymphoma

Aaron Auerbach, MD, PhD

There is a single large raised red nodule image on the scalp, 5 cm in largest dimension. Microscopically, the nodule was diagnosed as PCFCL. (Courtesy M. Tomaszewski, MD.)

Low-power magnification shows PCFCL with a multinodular image dermal infiltrate that is separated from the epidermis by a grenz zone image. (Courtesy M. Tomaszewski, MD.)



  • Primary cutaneous follicle center lymphoma (PCFCL)


  • Follicular lymphoma of skin

  • Crosti disease (reticulohistiocytoma of dorsum)

    • Plaques/tumors surrounded by erythematous macules/papules


  • Mature B-cell lymphoma of follicle center cells, primary in skin

    • Disease limited to skin for 6 months after diagnosis

  • Distinct disease from systemic follicular lymphoma

    • Better prognosis

    • Fewer BCL2 rearrangements than systemic follicular lymphoma



  • Incidence

    • 0.1-0.2 cases per 100,000 people per year

    • Most common primary cutaneous B-cell lymphoma

      • ˜ 20% of all skin lymphomas

      • ˜ 60% of all B-cell skin lymphomas

  • Age

    • Usually adults

      • Median age: 60 years

      • But can also be seen in childhood

  • Gender

    • Male:female = 1.5:1


  • Usually head and neck, especially scalp

    • Less commonly trunk


  • Usually single lesion

    • ˜ 15% multifocal

  • Plaques, nodules, or tumors of differing sizes

    • From < 1 cm to > 40 cm in greatest dimension

  • Rarely ulcerates


  • Observation, surgical removal or local radiation

  • Chemotherapy only if extensive disease or extracutaneous disease


  • Good

    • Much better than systemic follicular lymphoma

    • Usually complete remission with treatment

    • ˜ 95% 5-year survival

  • Not affected by

    • Grade or growth pattern

    • Bcl-2 expression or t(14;18) status

  • ˜ 35% recurrence (often proximal to original lesion); extracutaneous spread ˜ 10%

  • ˜ 10% disseminate to extracutaneous sites


Histologic Features

  • Dermal B-cell infiltrate, often extends into subcutis (˜ 75%)

    • No overlying epidermotropism

  • Growth pattern

    • Nodular, diffuse, or nodular and diffuse

    • May be classified as follicular (> 75% follicular architecture), follicular and diffuse (25-75% follicular architecture), or diffuse (< 25% follicular architecture) growth pattern

  • Follicles

    • Often not well-defined

    • Usually seen in small lesions

    • Lack mantle zones

    • Lack tingible body macrophages

    • Contain follicular dendritic cells

  • Tumor cells

    • Mostly centrocytes (small to medium-sized, cleaved)

    • Variable numbers of centroblasts (larger in size)

  • Cases with ↑ numbers of centroblasts

    • Diagnose as PCFCL if nodular or nodular and diffuse growth pattern

    • Diagnose as diffuse pattern PCFCL if large cells and only diffuse growth pattern

  • Grading of follicular lymphoma

    • Not necessary or of prognostic value for PCFCL

    • Is necessary for systemic follicular lymphoma

      • Grade 1: < 5 centroblasts per high-power field; grade 2: 6-15 centroblasts per high-power field; grade 3: > 15 centroblasts per high-power field

      • Grades 1 and 2 show minimal differences in long-term outcome

      • Thus, 2008 WHO classification lumps cases with few centroblasts as “follicular lymphoma grade 1-2 (low grade)” in systemic follicular lymphoma, and does not advise grading of PCFCL

Cytologic Features

  • Small cleaved cells with coarse chromatin and 1 or more indistinct nucleoli on peripheral smear



Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Primary Cutaneous Follicle Center Lymphoma
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