Staging criteria for mycosis fungoides / Sézary syndrome (MF / SS) is different from non mycosis fungoides / Sézary syndrome lymphomas (including primary cutaneous B cell lymphomas [CBCL], other primary cutaneous T cell lymphomas [CTCL] and NK cell lymphomas) (Blood 2007;110:1713, Blood 2007;110:479)
Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T cell lymphoma
Primary cutaneous acral CD8+ T cell lymphoma
Primary cutaneous CD4+ small / medium T cell lymphoproliferative disorder
Primary cutaneous peripheral T cell lymphoma, NOS
Cutaneous B cell lymphomas
Primary cutaneous marginal zone lymphoma
Primary cutaneous follicle center lymphoma
Primary cutaneous diffuse large B cell lymphoma, leg type
Contributed by Henry K. Wong, M.D., Ph.D.
Mycosis fungoides, patch stage
Mycosis fungoides, plaque stage
Mycosis fungoides, tumor stage
Peripheral smear images
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Sézary cells, peripheral smear
Diagnosis and staging of cutaneous lymphoma
Board review style question #1
A 45 year old woman presents with new onset violaceous plaques and tumors on the lower leg and foot with rapid progression (see picture above). The lesions measure 3.2 cm and 4.9 cm in greatest dimension, respectively. No other abnormalities are found on physical exam. Biopsy of the lesions shows sheet of large, atypical cells that stain positively with CD20, MUM1 and BCL2. A diagnosis of diffuse large B cell lymphoma, leg type is made. What is the correct T stage of the disease?
Board review style answer #1
B. T2a. Multiple lesions of diffuse large B cell lymphoma, leg type are present on 2 contiguous regions of the body, hence, the T stage is T2a using the non mycosis fungoides / Sézary syndrome primary cutaneous lymphoma staging criteria.
A 68 year old man presents with erythema of the entire back, chest, abdomen and bilateral extremities. On further inspection, there is a 1.8 cm raised ulcerated lesion present on the left upper thigh and multiple enlarged palpable lymph nodes in the inguinal region. Biopsy of the thigh lesion shows tagging of CD4 + T cells along the dermal epidermal junction and epidermotropism. The cells show loss of CD7 and CD26. What is the most likely diagnosis and correct T stage of the disease?
Mycosis fungoides; stage T1a
Mycosis fungoides; stage T2b
Mycosis fungoides; stage T3
Mycosis fungoides; stage T4
Board review style answer #2
D. Mycosis fungoides; stage T4. The erythema covers > 80% of body surface which is stage T4 according to the mycosis fungoides / Sézary syndrome primary cutaneous lymphoma staging criteria.
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