Name

ICD-O-3 Morphology

9718/3: Primary cutaneous CD30-positive T-cell lymphoproliferative disorders
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.

Grade

Not Applicable

Module Rule

None

Alternate Names

C-ALCL
Primary cutaneous CD30+ large T-cell lymphoma [OBS]
Primary cutaneous CD30+ T-cell lymphoproliferative disorder, regressing atypical histiocytosis [OBS]

Definition

Primary cutaneous anaplastic large cell lymphoma (C-ALCL) is composed of large cells with an anaplastic, pleomorphic, or immunoblastic cytomorphology, the majority of which express the CD30 antigen.

Patients with C-ALCL should not have the clinical evidence of mycosis fungoides with large cell transformation, which may be CD30-postive or CD30 negative. The disease must also be distinguished from systemic anaplastic large cell lymphoma with cutaneous involvement, which is a separate disease with different cytogenetics, clinical features, and outcomes.

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

TR genes are clonally rearranged
6q16-21 loss
7q31
13q34 loss

Immunophenotyping

CD2 variable loss
CD3 variable loss
CD4 positive T-cell phenotype
CD5 variable loss
CD15 expression
CD30 positive
Granzyme B expression
Perforin expression
TIA1 expression

Treatments

Chemotherapy
Radiation therapy

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 (U.S. only)

C86.6 Primary cutaneous CD30-positive T-cell proliferations (effective October 01, 2015)

Signs and Symptoms

Drenching night sweats
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

Progression and Transformation

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

Sources

Swerdlow SH, Campo E, Harris NL, Jaffe ES, Pileri SA, Stein H, Thiele J (Eds):
WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues (Revised 4th edition)
IARC: Lyon 2017
Section: Mature T- and NK-cell neoplasms
Pages: 395-396

International Classification of Diseases for Oncology, Third Edition, First Revision. Geneva: World Health Organization, 2013.
Section: ICD-O-3.1 (2011) Morphological Codes
Pages: http://codes.iarc.fr/codegroup/2

National Cancer Institute
Section: General Information About Adult Non-Hodgkin Lymphoma (NHL)
Pages: https://www.cancer.gov/types/lymphoma/hp/adult-nhl-treatment-pdq
Glossary