Primary cutaneous anaplastic large cell lymphoma

Name
Primary cutaneous anaplastic large cell lymphoma
ICD-O-3 Morphology
9718/3
Effective 2001 and later
Reportable
for cases diagnosed 2001 and later
Primary Site(s)
C440-C449, C510-C512, C518-C519, C600-C602, C608-C609, C632
Cutaneous (skin) lymphoma which presents with lesions on the skin of the trunk, face, extremities, and buttocks. See Module 7.

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Grade
5 - T-cell
Module Rule
None
Alternate Names
C-ALCL
Primary cutaneous CD30+ large T-cell lymphoma
Primary cutaneous CD30+ T-cell lymphoproliferative disorder
Definition
A spectrum of related conditions originating from transformed or activated CD30+ T lymphocytes. Distinction of C-ALCL from systemic ALCL with cutaneous involvement is important.

This is part of the Primary cutaneous CD30 positive T-cell lymphoproliferative disorders WHO group, which also includes lymphomatoid papulosis (see 9718/1).
Abstractor Notes
Most patients present with solitary or localized nodules or tumors, and sometimes papules, and often show ulceration.

Extracutaneous dissemination occurs in <10% of the patients and mainly involves the lymph nodes.
Definitive Diagnostic Methods
Genetic testing
Histologic confirmation
Immunophenotyping
Genetics Data
T-cell receptor (TCR) genes are clonally rearranged
Immunophenotyping
CD2-
CD4+ T-cell phenotype
CD30+
Most express cutaneous lymphomacyte antigen (CLA)
Treatments
Chemotherapy
Radiation
Transformations to
There are no known transformations
Transformations from
There are no known transformations
Corresponding ICD-9 Codes
202.7 Peripheral T-cell lymphoma
Corresponding ICD-10 Codes
C84.4 Peripheral T-cell lymphoma
Corresponding ICD-10-CM Codes (effective October 1, 2015 U.S. only)
C86.6 Primary cutaneous CD30-positive T-cell proliferations
Signs and Symptoms
Drenching night sweats
Fatigue
Fever (for no known reason)
Pain in the chest, abdomen, or bones (for no known reason)
Painless swelling in the lymph nodes
Solitary or localized nodules or tumors
Ulcerated skin lesions
Weight loss (for no known reason)
Diagnostic Exams
CT (CAT) scan
Complete blood count (CBC)
MRI (magnetic resonance imaging)
PET (positron emission tomography) scan
Recurrence and Metastases
Multifocal lesions are seen in about 20% of cases. These lymphomas frequently relapse in the skin.
Epidemiology and Mortality
Age: 60 years median age (can occur in children, although rare)
Incidence: second most common type of cutaneous T-cell lymphoma
Race: female predominance
Survival: 90% 10 year survival