Search Database ICD-O-3 Code Lists

Name

Primary cutaneous follicle centre lymphoma (PCFCL)

ICD-O-3 Morphology

9597/3: Primary cutaneous follicle center lymphoma
Effective 2001 and later

Reportable

for cases diagnosed 1978 and later

Primary Site(s)

C440-C449 , C510-C512 , C518-C519 , C600-C602 , C608-C609 , C632
Most common sites of involvement: Cutaneous (skin) lymphoma, primarily on the scalp, forehead or trunk.

Assign C449 if specific primary site cannot be determined.

See abstractor notes.

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

(This code is effective for cases diagnosed 2010 and later. For cases diagnosed prior to 2010, see codes 9690/3 or 9709/3.)

Primary cutaneous follicle centre lymphoma (PCFCL) is part of the Mature B-cell neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B15)

PCFCL characteristically manifests with solitary or localized skin lesions on the head/neck or the trunk. Less common include skin lesions on the legs. Multiple skin lesions may also be present.

Differences between follicle center lymphoma and follicular lymphoma:
1. Follicle center lymphoma is a cutaneous malignancy with only rare involvement of regional lymph nodes.
2. Follicular lymphoma commonly occurs in nodes and extranodal sites.

Note: If there is involvement of lymph node(s) that are not regional for the skin site involved, or involvement of bone marrow or organ(s), do not code histology primary cutaneous follicle center cell lymphoma and do not code skin as the primary site. Code the histology to follicular lymphoma (See histology codes: 9690, 9691, 9695 or 9698).

Systemic therapy is only given for patients with very extensive cutaneous disease, extremely thick skin tumors, or with extracutaneous disease.

Diagnostic Confirmation

This histology can be determined by positive histology (including peripheral blood) with or without genetics and/or immunophenotyping. Review the Definitive Diagnostic Methods, Immunophenotyping and Genetics Data sections below, and the instructions in the Hematopoietic Manual for further guidance on assigning Diagnostic confirmation.

Module Rule

Module 6: PH12

Alternate Names

B-cell lymphoma, follicle type
Crosti's disease (lymphoma)
Reticulohistiocytoma of the dorsum

Definition

Primary cutaneous follicle center lymphoma (PCFCL) is a tumor of follicle center cells, including centrocytes and variable numbers of centroblasts, with a follicular, follicular and diffuse, or diffuse growth pattern, that generally occurs in the skin of the head or trunk. (WHO 5th edition)

Definitive Diagnostic Methods

Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

BCL2 rearrangements lacking
CREBBP mutations
FOXP1 negative
IRF4 (MUM1) negative
KMT2D mutations

Immunophenotyping

BCL6+ (expression/positive)
CD5- (no expression/negative)
CD10+ (expression/positive)
CD43- (no expression/negative)

Treatments

Chemotherapy
Radiation therapy

Transformations to

There are no known transformations

Transformations from

There are no known transformations

Same Primaries

Corresponding ICD-10 Codes (Cause of Death codes only)

C82.7 Other types of follicular non-Hodgkin lymphoma

Corresponding ICD-10-CM Codes (U.S. only)

C82.5_ Diffuse follicle center lymphoma (effective October 01, 2015)
C82.5A Diffuse follicle center lymphoma, in remission (effective October 01, 2024)
C82.6_ Cutaneous follicle center lymphoma (effective October 01, 2015)
C82.6A Cutaneous follicle center lymphoma, in remission (effective October 01, 2024)

Signs and Symptoms

Drenching night sweats
Fatigue
Fever (for no known reason)
Firm erythematous or violaceous plaques, nodules or tumors of varying sizes
Pain in the chest, abdomen, or bones (for no known reason)
Painless swelling in the lymph nodes
Skin rash or itchy skin
Weight loss (for no known reason)

Diagnostic Exams

Blood chemistry studies
Bone marrow aspiration and biopsy
CT (CAT) scan
Cytogenetic analysis
Flow cytometry
Immunohistochemistry
Immunophenotyping
Laparoscopy (rarely performed)
Laparotomy (rarely performed)
Lymph node biopsy
PET (positron emission tomography) scan

Progression and Transformation

Cutaneous relapses noted in ~30% of patients
Recurrences usually proximate to the initial site of the tumor
Untreated skin lesions increase in size, rarely metastases to other sites

Epidemiology and Mortality

Age: 51 years median age
Sex: slight male predominance
Survival: 5 year survival over 95%

Sources

WHO Classification of Tumours Editorial Board. Haematolymphoid tumours. Lyon (France): International Agency for Research on Cancer; 2024. (WHO classification of tumours series, 5th ed.; vol. 11). https://publications.iarc.who.int/637.
Section: Follicular lymphoma
Pages: Part B: 440-442

International Classification of Diseases for Oncology, 3rd edition (including revisions). Geneva: World Health Organization, 2001, 2011, 2020.
Section: ICD-O-3.2 (2020) Morphological Codes
Pages: http://www.iacr.com.fr/index.php?option=com_content&view=category&layout=blog&id=100&Itemid=577

PDQ® Adult Treatment Editorial Board. PDQ Indolent B-Cell Non-Hodgkin Lymphoma Treatment. Bethesda, MD: National Cancer Institute. Updated <02/18/2025>. Available at: https://www.cancer.gov/types/lymphoma/hp/indolent-b-cell-lymphoma-treatment-pdq. Accessed <03/04/2025>. [PMID: 37437080]
Section: Indolent B-Cell Non-Hodgkin Lymphoma Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Glossary