# Primary cutaneous follicle centre lymphoma

Source: https://onco.cc/cancers/primary-cutaneous-follicle-centre-lymphoma/  
OnCo record `primary-cutaneous-follicle-centre-lymphoma` (Cancer). Data CC BY-NC 4.0, attribute "Data from OnCo (onco.cc)"; commercial use needs a licence.

## TL;DR

Primary cutaneous follicle centre lymphoma is a slow-growing lymphoma of germinal-centre B cells that stays in the skin, usually as lumps on the head or trunk. Its outlook is excellent, with about 95 in 100 people alive at five years, and radiotherapy or excision is usually all that is needed; the important thing is not to mistake it for the aggressive leg-type large B-cell lymphoma.

## Summary

WHO-HAEM5 lists primary cutaneous follicle centre lymphoma among the cutaneous B-cell lymphomas as a distinct entity from follicular lymphoma, defined by follicle centre cells in the skin with no extracutaneous disease at staging (Alaggio 2022). It has a five-year overall survival of about 95 percent against about 50 percent for primary cutaneous large B-cell lymphoma, leg type, and the two are separated in practice by IgM staining, present in all 40 leg-type cases and only 5 of 53 follicle centre cases in the defining study (Am J Surg Pathol 2010). Its molecular landscape differs from classic follicular lymphoma: 27 percent lack CD10 but all express MEF2B and HGAL, TNFRSF14 is the most commonly mutated gene (40 percent, with a further 10 percent carrying 1p36 deletions), followed by CREBBP, TNFAIP3, KMT2D, SOCS1, EP300, STAT6 and FOXO1, and BCL2 rearrangements are usually absent (Human Pathology 2020).

How it differs from its parent: it is skin-confined, rarely carries the t(14;18) that defines nodal follicular lymphoma, is not graded or staged as follicular lymphoma is, and is cured locally in most cases; the parent's chemoimmunotherapy is reserved for the rare disseminated case.

How common: no registry share in the sources read.

Treatment: local radiotherapy or excision for solitary or localised lesions; rituximab for multifocal skin disease; the parent's systemic pathways only for extracutaneous spread; skin relapses are common and treated locally again (Am J Surg Pathol 2010 for the survival figures).

## Fields

- Kind: Cancer
- Last checked: 2026-09-24
- Also known as: Primary cutaneous follicular lymphoma; Primary cutaneous follicle center lymphoma; PCFCL; Crosti lymphoma; Reticulohistiocytoma of the dorsum
- Tags: subtype-page; wave4; haematologic; rare
- Group: haematologic
- Burden: Rare; the largest series in the sources read hold 53 patients (Am J Surg Pathol 2010) and 22 (Human Pathology 2020). Five-year overall survival is about 95 percent (Am J Surg Pathol 2010). No registry share is in the sources read.
- Subtypes: Primary cutaneous follicle centre lymphoma, follicular growth pattern; Primary cutaneous follicle centre lymphoma, diffuse growth pattern (large centrocytes; still indolent); Primary cutaneous follicle centre lymphoma of the leg (must be separated from leg-type large B-cell lymphoma)
- Biomarkers: BCL6 positive; CD10 variable; MEF2B and HGAL positive; IgM negative (positive in leg-type large B-cell lymphoma); TNFRSF14 mutation or 1p36 deletion; Usually no BCL2 rearrangement

## Sections of this record

The page is a hub with ten sections in reading order; large sections have their own page. The same plan as JSON: https://onco.cc/api/v1/cancers/primary-cutaneous-follicle-centre-lymphoma/sections.json

- Overview (on the hub): The TL;DR, the family this cancer belongs to, the organ, who gets it and what the state of the art is. https://onco.cc/cancers/primary-cutaneous-follicle-centre-lymphoma/#overview
- What it is (on the hub): Anatomy, the subtypes and how they differ, how it is staged, and where advanced disease spreads. https://onco.cc/cancers/primary-cutaneous-follicle-centre-lymphoma/#what-it-is [3 subtypes]
- Finding it (on the hub): How it shows itself, how it is confirmed, what screening exists, and the biomarkers clinicians test for. https://onco.cc/cancers/primary-cutaneous-follicle-centre-lymphoma/#finding-it [4 biomarkers]
- Treating it (on the hub): The standard of care by setting, the medicines, surgery and radiotherapy named in it, and the regimens behind them. https://onco.cc/cancers/primary-cutaneous-follicle-centre-lymphoma/#treating-it [2 settings]
- Evidence (on the hub): Trials recruiting now, the landmark trials, the key papers and what they mean, the latest literature, and the milestones year by year. https://onco.cc/cancers/primary-cutaneous-follicle-centre-lymphoma/#evidence
- The science (on the hub): The molecular landscape: the targets and how often each appears, the pathways, the mechanics stages and the preclinical models. https://onco.cc/cancers/primary-cutaneous-follicle-centre-lymphoma/#science [1 target]
- Where you are (own page): Cases by country, the UK and NHS pathway and other country lenses, and the expert centres with trials on record. https://onco.cc/cancers/primary-cutaneous-follicle-centre-lymphoma/where-you-are/
- Living with it (on the hub): The decisions you may face, the aids that walk through them, the warnings on record, the first sixty days and the questions to ask. https://onco.cc/cancers/primary-cutaneous-follicle-centre-lymphoma/#living-with-it [9 questions, 2 red cards]
- What is coming (own page): Everything in development, the open problems and what is being done about them, the roadmaps, and what changed on this record. https://onco.cc/cancers/primary-cutaneous-follicle-centre-lymphoma/coming/ [1 medicine]
- Data (own page): Every connected record, the notes, the JSON, Markdown and RDF twins, and where the record came from and when it was checked. https://onco.cc/cancers/primary-cutaneous-follicle-centre-lymphoma/data/ [8 connected records]

## Standard of care

- Solitary or localised: Local radiotherapy or excision. ([Follicular lymphoma](https://onco.cc/cancers/follicular-lymphoma/))
- Multifocal or extracutaneous: Rituximab for multifocal skin disease; the parent's systemic pathways for the rare extracutaneous spread. ([Rituximab](https://onco.cc/drugs/rituximab/), [Follicular lymphoma](https://onco.cc/cancers/follicular-lymphoma/))

## Sources

- Wikipedia: https://en.wikipedia.org/wiki/Primary_cutaneous_follicle_center_lymphoma
- NCI PDQ: adult non-Hodgkin lymphoma treatment: https://www.cancer.gov/types/lymphoma/patient/adult-nhl-treatment-pdq
- Alaggio 2022, Leukemia: the 5th edition WHO classification of haematolymphoid tumours, lymphoid neoplasms: https://doi.org/10.1038/s41375-022-01620-2
- Am J Surg Pathol 2010: IgM distinguishes primary cutaneous large B-cell lymphoma, leg type from follicle centre lymphoma: https://doi.org/10.1097/pas.0b013e3181e5060a
- Human Pathology 2020: molecular landscape of primary cutaneous follicle centre lymphoma: https://doi.org/10.1016/j.humpath.2020.09.014

## Connected records

- cancers: [Cutaneous T-cell lymphoma (mycosis fungoides and Sezary syndrome)](https://onco.cc/cancers/cutaneous-t-cell-lymphoma/), [Diffuse large B-cell lymphoma](https://onco.cc/cancers/dlbcl/), [Follicular lymphoma](https://onco.cc/cancers/follicular-lymphoma/), [Primary cutaneous marginal zone lymphoma](https://onco.cc/cancers/primary-cutaneous-marginal-zone-lymphoma/)
- drugs: [Rituximab](https://onco.cc/drugs/rituximab/)

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JSON: https://onco.cc/api/v1/entities/primary-cutaneous-follicle-centre-lymphoma.json