Search Database ICD-O-3 Code Lists

Name

Primary cutaneous peripheral T-cell lymphoma (NOS) (pcPTCL)

ICD-O-3 Morphology

9709/3: Cutaneous T-cell lymphoma, NOS
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
Cutaneous (skin) lymphoma which presents with generalized skin lesions.

Assign C449 if specific primary site cannot be determined.

See abstractor notes

Coding Manual: Hematopoietic Coding Manual (PDF)

Abstractor Notes

Primary cutaneous peripheral T-cell lymphoma (NOS) (pcPTCL) is part of the Mature T-cell and NK-cell neoplasms lineage table in the WHO 5th edition of Hematolymphoid Tumors. (See Appendix B in the Hematopoietic Manual, Table B20)

For primary cutaneous peripheral T-cell lymphoma, disseminated skin lesion are predominantly location on the trunk and limbs, whereas solitary tumor are more commonly found on the skin of the legs, and arms. This diagnosis is usually a diagnosis of exclusion.

For primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma, patients present with generalized or localized skin lesions. The oral mucosa may be involved.

Primary cutaneous acral CD8-positive T-cell lymphoproliferative disorder is commonly found in the ears (most common site), nose, hands, and feet.

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

None

Alternate Names

Cutaneous lymphoma, NOS [OBS]
Cutaneous T-cell lymphoma, NOS (CTCL, NOS)
Primary cutaneous acral CD8 positive T-cell lymphoproliferative disorder
Primary cutaneous acral CD8 positive T-cell lymphoma [OBS]
Primary cutaneous CD8 positive aggressive epidermotropic cytotoxic T-cell lymphoma (PCAETL)
Primary cutaneous peripheral T-cell lymphoma, unspecified

Definition

Primary cutaneous peripheral T-cell lymphoma (pcPTCL) NOS is a poorly characterized group of T-cell lymphomas not meeting the criteria for any specifically defined primary cutaneous T-cell lymphoma entity, i.e. it is a diagnosis of exclusion. (WHO 5th edition).

Primary cutaneous CD8-positive aggressive epidermotropic cytotoxic T-cell lymphoma (PCAETL) is a neoplastic proliferation of T lymphocytes often expressing CD8 along with cytotoxic molecules, characterized by epidermal necrosis, a high proliferation index, and aggressive clinical behavior. (WHO 5th edition)

Primary cutaneous acral CD8-positive T-cell lymphoproliferative disorder is characterized by dermal, non-epidermotropic infiltrates of clonal medium-sized CD8+ cytotoxic lymphocytes, preferentially located at acral sites. (WHO 5th edition)

Definitive Diagnostic Methods

Clinical diagnosis
Cytogenetics
Genetic testing
Histologic confirmation
Immunohistochemistry
Immunophenotyping

Genetics Data

Gain of function mutations in JAK2, STAT3 and STAT5B (PCAETL)
Upregulated JAK-2 signaling (PCAETL)

Immunophenotyping

CD2+ (expression/positive)
CD3+ (expression/positive)
CD4+ (expression/positive)/CD8- (no expression/negative)
CD4- (no expression/negative) (CD8-positive T-cell lymphoproliferative disorder)
CD8+ (expression/positive) (CD8-positive T-cell lymphoproliferative disorder)
CD30- (no expression/negative) (CD8-positive T-cell lymphoproliferative disorder)

Treatments

Chemotherapy
Hematologic Transplant and/or Endocrine Procedures
Hormone therapy
Immunotherapy
Other therapy
Radiation therapy
Surgery

Transformations to

Transformations from

None

Same Primaries

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

C84.5 Other and unspecified T-cell lymphomas

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

C84.A_ Cutaneous T-cell lymphoma, unspecified (effective October 01, 2015)
C84.AA Cutaneous T-cell lymphoma, unspecified, in remission (effective October 01, 2024)

Signs and Symptoms

Drenching night sweats
Fatigue
Fever (for no known reason)
Pain in the chest, abdomen, or bones (for no known reason)
Ulcerated/disseminated eruptive papules, nodules and tumors
Weight loss (for no known reason)

Diagnostic Exams

Blood chemistry studies
Bone marrow aspiration and biopsy
CT (CAT) scan
Flow cytometry
Immunohistochemistry
Immunophenotyping
PET (positron emission tomography) scan
Skin biopsy

Progression and Transformation

None

Epidemiology and Mortality

Incidence: extremely rare (pcPTCL-NOS)
Age: 6th decade of lie (pcPTCL-NOS)
Sex: M:F ratio 3:1 (pcPTCL-NOS)
Survival: 27 months to 5.6 years (pcPTCL-NOS)
Age: Adults (PCAETL)
Sex: Male predilection (PCAETL)
Survival: Very aggressive, median survival 12 months (PCAETL)
Age: Median age 56 (CD8-positive T-cell lymphoproliferative disorder)
Sex: M:F ratio 2:1 (CD8-positive T-cell lymphoproliferative disorder)
Survival: Excellent (CD8-positive T-cell lymphoproliferative disorder)

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: Primary cutaneous T-cell lymphoid proliferations and lymphomas
Pages: Part B: 685-687, 705-709

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 Mycosis Fungoides and Other Cutaneous T-Cell Lymphomas Treatment. Bethesda, MD: National Cancer Institute. Updated <02/19/2025>. Available at: https://www.cancer.gov/types/lymphoma/hp/mycosis-fungoides-treatment-pdq. Accessed <03/31/2025>. [PMID: 2638928
Section: Mycosis Fungoides and Other Cutaneous T-Cell Lymphomas Treatment (PDQ®)–Health Professional Version
Pages: https://www.cancer.gov/types/lymphoma/hp/mycosis-fungoides-treatment-pdq
Glossary