Primary cutaneous follicle centre lymphoma

Name
Primary cutaneous follicle centre lymphoma
ICD-O-3 Morphology
9597/3
Effective 2010 and later
Reportable
for cases diagnosed 2010 and later
Primary Site(s)
C440-C449, C510-C512, C518-C519, C600-C602, C608-C609, C632

Help me code for dx year :

Grade
6 - B-cell
Module Rule
Module 6: PH12
Alternate Names
B-cell lymphoma, follicle type
Crosti's disease
Follicle cell lymphoma
PCFCL
PCFCL, leg type
Primary cutaneous follicle center lymphoma
Reticulohistiocytoma of the dorsum
Definition
This 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. It mainly affects adults.

All variants of follicular lymphoma (NOS, grade 1, grade 2, and grade 3) were once called “follicle centre lymphoma.” Although that term is obsolete, it is sometimes used to describe follicular lymphoma. You will also see “follicle centre” in the pathology reports for follicular lymphoma. However, the primary site and other sites of involvement will differ between follicular lymphoma and follicle centre lymphoma. Follicle centre lymphoma is a cutaneous malignancy with only rare involvement of regional lymph nodes. Follicular lymphoma commonly occurs in nodes and extranodal sites. (See the Heme DB Abstractor Notes for both neoplasms for information on clinical presentation and common primary sites.
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.) This disease usually presents with solitary or localized skin lesions on the scalp, the forehead or the trunk. Also presents on the leg with a less favorable prognosis. Although it is not common, the regional lymph nodes may be involved. Disease dissemination to distant nodes or other organs/tissue is rare and usually happens in the late stages of the disease. Patient presents with firm erythematous to violaceous plaques, nodules or tumors of variable size. Systemic therapy is only given for patients with very extensive cutaneous disease, extremely thick skin tumors, or with extracutaneous disease.

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 centre cell lymphoma and do not code skin as the primary site.
Definitive Diagnostic Methods
FISH
Histologic confirmation
PCR
Genetics Data
Amplification of C-REL gene
BCL2 rearrangements
Immunophenotyping
BCL2
BCL6
CD10+
CD20
CD79A
Ig negative
Treatments
Chemotherapy
Radiation
Transformations to
None
Transformations from
None
Corresponding ICD-9 Codes
202.0 Nodular lymphoma
Corresponding ICD-10 Codes
C85.7 Other specified types of non-Hodgkin's lymphoma
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C82.6 Cutaneous follicle center lymphoma
Signs and Symptoms
None
Diagnostic Exams
None
Recurrence and Metastases
None
Epidemiology and Mortality
None