Mycosis Fungoides

This schema is for Mycosis Fungoides (MF) and Sezary Disease (SD). The major difference between the two histologies is that for Sezary Disease, high amounts of cancerous T-cells are found in the blood. The distinction between the two histologies should be made by the pathologist or managing physician, not the registrar.

There are several phases for Mycosis Fungoides, patch (reddened, eczema-like rash), plaque/papules (small, raised bumps) and tumors on the skin (which may develop ulcers and become infected)

Lymph node involvement is based on clinically abnormal lymph nodes, and if confirmed pathologically, are further separated into clone negative, clone positive, and clone unknown

Metastatic disease includes visceral (non-cutaneous, extranodal) organ involvement, and carcinomatosis (clinically or pathologically confirmed)

There is one SSDI for Mycosis Fungoides and that is peripheral blood involvement. This is the “B” category that refers to the percentage of peripheral blood lymphocytes that are atypical. The percentage cutoffs are less than or equal to 5%, more than 5%, along with clone negative, clone positive, and clone unknown.

Treatment for Mycosis Fungoides and Sezary Disease includes photodynamic therapy, biological therapy, chemotherapy, targeted therapy, and checkpoint inhibitors

Data Item Criteria/Comments
Histologies 9700/3: Mycosis Fungoides, 9701/3: Sezary Syndrome
Primary Sites C440-C449, C510-C512, C518-C519, C600-C602, C608-C609, C632
EOD Primary Tumor Patches only, or papules/plaques
Tumor: less than 1 cm OR equal to 1 cm or greater
Generalized erythroderma
EOD Regional Nodes Includes regional AND distant lymph nodes
Clinical enlarged nodes (negative pathology or no pathology)
Clone positive, clone negative, clone unknown
Dutch grade and NCI LN grade: 1, 2, 3, 4 or unknown
EOD Mets Carcinomatosis (clinically or pathologically), peripheral blood, bone marrow, other metastatic involvement (excluding distant lymph nodes)
Summary Stage Code 1: Patches, papules or plaques only (size irrelevant)
Code 2: Cutaneous tumors, erythroderma
Code 3: Patches, papules, plaques and regional lymph nodes
Code 4: Cutaneous tumors, erythroderma and regional lymph nodes
Code 7: Bone marrow, visceral organs, distant lymph nodes, other metastases
Peripheral Blood Involvement (SSDI) The B category is required for staging and is based on the percentage of peripheral blood lymphocytes
See the SSDI manual for further information

Updated: December 2, 2025