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Skin - Nonmelanocytic tumors

Lymphoma and related disorders

Primary cutaneous lymphoma

Reviewer: Christopher Hale, M.D. (see Reviewers page)
Revised: 25 August 2013, last major update July 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.

Primary cutaneous B cell lymphoma

● < 20% of primary cutaneous lymphomas
● By definition, no evidence of extracutaneous disease is identified for 6 months after initial diagnosis
Marginal zone lymphomas often associated with Borrelia burgdorferi infection

Similar morphology to nodal disease, but more indolent Subtypes include:
      (a) primary cutaneous follicle center lymphoma (PCFCL)
      (b) primary cutaneous marginal zone lymphoma (PCMZL)
      (c) intravascular large B-cell lymphoma, diffuse large B-cell lymphoma, leg type (DLBCLLT)
      (d) diffuse large cell B-cell lymphoma-other;

● PanB-cell markers are CD20, CD79a (J Clin Aesthet Dermatol 2010;3:21)

Primary Cutaneous Follicle Center Lymphoma:
● Positive for CD20 and CD79a with follicular pattern
● Follicular and diffuse patterns of PCFCL typically BCL6 positive
● CD10 expressed in follicular pattern and typically negative in diffuse pattern
● BCL2, CD5, and CD43 negative
● BCL2 positivity suggests nodal origin with skin involvement
● t(14;18) is rare in cutaneous follicular lymphoma, but is characteristic of nodal follicular lymphoma

Primary Cutaneous Marginal Zone Lymphoma:
● Positive for CD20, CD79a, and BCL2; negative staining for CD5, CD10, CD43, BCL6

Diffuse Large B-cell Lymphoma, leg-type:
● BCL2 positive (negative staining suggests diffuse large B-cell lymphoma, other)
● Positive for CD20, CD79a, MUM-1/IRF4 and usually BCL6 or CD10

Subcutaneous, blastic NK, NK/T cell or other cytotoxic T cell lymphoma

● These cases include patients with skin and non-skin disease at diagnosis, as well as skin only
● Excludes mycosis fungoides and peripheral T cell lymphoma
● All CD30 negative with medium/large cells or subcutaneous panniculitis-like

(a) Subcutaneous panniculitis-like T cell lymphoma:
● Indurated, erythematous and discolored plaques on extremities
● Alpha/beta CD8+ cytotoxic T cells, with almost exclusive involvement of subcutaneous tissue resembling lobular panniculitis
● Recommend to exclude cases with epidermal involvement from this category
● Tumor cells have pleomorphic nuclei and adipocyte rimming (not specific for this diagnosis)
● CD3+, CD8+, TIA1+, EBV-, estimated 5 year survival is 80% with systemic steroid therapy

(b) Blastic NK cell lymphoma:
● Usually multiple bruise-like deep-red plaques/tumors
● Involves dermis and surrounding adnexa, grenz zone present, subcutis but no epidermal involvement
● Monomorphous medium sized cells with fine chromatin resembling blasts of acute myelogenous leukemia (although AML is CD56-)
● May actually derive from common myeloid and NK precursor called plasmacytoid type 2 dendritic cell
● CD3-, CD4+, CD8-, CD56+, TIA1-, TdT variable, EBV-
● Estimated 5 year survival is 0%
● Eventually become leukemia

(c) NK/T cell lymphoma, nasal type:
● Multiple patches, plaques or nodules
● May involve epidermis, dermis or subcutis
● Medium-large pleomorphic or blastic nuclei
● CD3epsilon+, TIA1+, EBV+
● Estimated 5 year survival is 0%

(d) Epidermotrophic CD8+ T cell lymphoma:
● Multiple plaques and tumors, similar to disseminated pagetoid reticulosis
● Ulceration common
● Alpha/beta negative, CD8+ cytotoxic T cells, with predominant involvement of epidermis, also dermis, adnexae and subcutis
● CD3+, CD8+; TIA1+, betaF1+; EBV-
● Estimated 5 year survival is 0%
● Must rule out mycosis fungoides

(e) Cutaneous gamma/delta T cell lymphoma:
● Multiple plaques and tumors, similar to disseminated pagetoid reticulosis
● Epidermal involvement with necrosis, interface dermatitis, adnexal involvement, gamma/delta T cells
● CD4-, CD8-, CD56+, TIA1+, EBV-, estimated 5 year survival is 0%

(f) Cutaneous alpha/beta pleomorphic T cell lymphoma:
● Solitary or multiple plaques and tumors
● Often epidermal necrosis and adnexal involvement
● Alpha/beta T helper cells (CD8-) with expression of cytotoxic markers betaF1 and TIA1, different from subcutaneous panniculitis-like T cell lymphoma and epidermotrophic CD8+ T cell lymphoma
● Estimated 5 year survival is 0%

(g) Cutaneous medium/large pleomorphic T cell lymphoma, not otherwise specified:
● Multiple plaques and tumors, donít fit other categories

Clinical images

Small or medium pleomorphic T cell lymphoma with solitary reddish nodule on the cheek

Micro images

Cutaneous CD8 epidermotropic T cell lymphoma

Additional references

J Clin Aesthet Dermatol 2010;3:21, J Clin Pathol 59;8:813, Am J Surg Pathol 2004;28:719
● Mutational analysis (Mod Path 2004;17:623)

End of Skin - Nonmelanocytic tumors > Lymphoma and related disorders > Primary cutaneous lymphoma

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