Lymphoma - Non B cell neoplasms - Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T cell lymphoma

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Lymphoma - Non B cell neoplasms

T/NK cell disorders

Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T cell lymphoma

Reviewer: Dragos Luca, M.D. (see Reviewers page)
Revised: 31 October 2011, last major update August 2011
Copyright: (c) 2001-2011, PathologyOutlines.com, Inc.

Definition
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● A cutaneous T cell lymphoma characterized by proliferation of epidermotropic CD8+ cytotoxic T cells and aggressive clinical behavior (provisional entity, WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, 4th Edition, Lyon 2008)

Terminology
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● Similar (? identical) to the generalized pagetoid reticulosis of the Ketron-Goodman type

Epidemiology
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● Rare, <1% of all cutaneous TCL’s
● Mainly adults

Sites
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● Generalized skin lesion in most patients

Clinical features
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● Localized or disseminated papules, nodules and tumors with central ulceration and necrosis, or superficial hyperkeratotic patches and plaques
● Extracutaneous dissemination possible: lung, testis, CNS, oral mucosa (lymph nodes often spared)

Case reports
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● 33 year old man with Steinert myotonic dystrophy (Ann Dermatol Veneorol 2006;133:991)
● 58 year old woman with HTLV-1 (Acta Derm Venereol 2010;90:324)
● Simulating pyoderma gangrenosum (Clin Exp Dermatol 2009;34:e261)

Treatment and prognosis
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● Aggressive clinical course
● Median survival 32 months

Postulated normal counterpart
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● Skin-homing CD8+ cytotoxic T cells of αβ type

Gross description
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● Localized or disseminated papules, nodules and tumors with central ulceration and necrosis, or superficial hyperkeratotic patches and plaques

Gross images
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Image (Fig. 5A)

Micro description
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● Very variable appearance
● Lichenoid pattern with marked pagetoid epidermotropism and subepidermal edema
● Deep nodular infiltrates
● Epidermal acanthosis or atrophy with necrosis, ulceration, blister formation; invasion and destruction of skin adnexal structures
● Small, medium or large tumor cells with pleomorphic or blastic nuclei; angiocentricity and angioinvasion may be present

Micro images
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Image (Fig. 5B)

Positive stains
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● CD3, CD8, βF1, granzyme B, perforin, TIA1, CD45RA (may be negative), CD7 (may be negative)

Negative stains
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● CD45RO, CD4, CD5, CD2 (may be positive), EBV

Genetics and Molecular
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● Clonal TCR gene rearrangements
● No specific cytogenetic abnormalities

Differential diagnosis
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● Other types of CD8+ cytotoxic T cell lymphomas: blastic NK cell lymphoma, cutaneous gamma/delta T cell lymphoma, extranodal NK-T cell lymphoma-nasal type, subcutaneous panniculitis-like T cell lymphoma are differentiated based on clinical presentation, behavior, lack of marked epidermotropism with epidermal necrosis (Am J Surg Pathol 2004;28:719)
● Mycosis fungoides (sometimes CD8+): no overt destruction and necrosis, more protracted course
● Lymphomatoid papulosis (proposed type D): much better prognosis, different clinical course and management (Am J Surg Pathol 2010;34:1168, Am J Clin Pathol 2006;125:490)

Additional references
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● Am J Pathol 1999; 155:483, Blood 2005;105:3768

End of Lymphoma - Non B cell neoplasms > T/NK cell disorders > Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T cell lymphoma

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