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Lymphoma - Non B cell neoplasms

T/NK cell disorders

Subcutaneous panniculitis-like T cell lymphoma


Reviewer: Dragos Luca, M.D. (see Reviewers page)
Revised: 29 October 2011, last major update August 2011
Copyright: (c) 2001-2011, PathologyOutlines.com, Inc.

Definition
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● Subcutaneous panniculitis-like T cell lymphoma (SPTCL) is a cytotoxic T cell lymphoma, which preferentially infiltrates subcutaneous tissue
● Composed of atypical lymphoid cells of varying size, typically with karyorrhexis of tumor cells and associated fat necrosis (WHO Classification of Tumours of Haematopoietic and Lymphoid Tissues, 4th Edition, Lyon 2008)

Terminology
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● γδ cases are now excluded from this category and reclassified as primary cutaneous γδ T cell lymphoma (in contrast to the 3rd edition of WHO classification)
● Most historical cases in the literature were diagnosed as histiocytic cytophagic panniculitis

Epidemiology
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● Rare, <1% of all non-Hodgkin lymphomas
● Slightly more common in females, median age 35 years, range 22-73 years
● 20% of cases under 20 years of age

Etiology
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● Up to 20% of patients have associated autoimmune disease (most frequently SLE, with lupus profundus panniculitis)
● No EBV

Sites
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● Multiple subcutaneous nodules on extremities and trunk

Clinical features
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● Subcutaneous nodules in extremities with associated hemophagocytic syndrome (15-20%)
● Nodules range from 0.5 to several cm in diameter; larger nodules may become necrotic but ulceration is rare
● Systemic symptoms in up to 50%
● Cytopenias, elevated liver function tests, may have hepatosplenomegaly (non-lymphomatous), no lymphadenopathy

Case reports
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● 38 year-old woman (Indian J Dermatol 2010;55:290)
● 43 year old woman with breast tumor (Appl Immunohistochem Mol Morphol 2009;17:301)
● 54 year old man with diffuse subcutaneous thoracic infiltration (Acta Derm Venereol 2009;89:427)

Treatment and prognosis
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● Course may be aggressive or indolent; estimated 5-year survival is 80%
● Dissemination to lymph nodes or other organs is rare
● Poor prognosis if accompanied by hemophagocytic syndrome
● Combination chemotherapy used traditionally; more conservative regimens (cyclosporine, prednisone, chlorambucil) may be effective
● Much better prognosis than primary cutaneous γδ T cell lymphoma

Postulated normal counterpart
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● Mature cytotoxic αβ T cell of the adaptive immune system

Gross description
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● Subcutaneous nodules on extremities and trunk ranging from 0.5 to several cm in diameter, possibly necrotic, rarely ulcerated

Gross images
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Thigh


Upper limb (1A) and abdomen (1B)


Gross, micro, and immuno

Micro description
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● Lesions confined to subcutis, monotonous infiltration of atypical pleomorphic lymphocytes infiltrating fat lobules, with/without sparing of the fibrous septae (resembling panniculitis)
● Atypia varies from minimal to marked, with irregular and hyperchromatic nuclei surrounded by a rim of pale cytoplasm
● Usually tumor necrosis/karyorrhexis, adipocyte rimming; variable vacuolated macrophages with lipid or nuclear debris, granulomatous reaction
● No epidermal or dermal involvement (usually); not primarily angiocentric but vascular invasion possible
● Other inflammatory cells typically absent, notably plasma cells (unlike lupus panniculitis)

Micro images
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Small and large atypical lymphocytes rimming adipocytes


CD8+

           

Various images


Rimming of fat cells


Panniculitis, rimming & karyorrhexis


CD3, CD8, CD30 and TIA1 (Fig.3)

Positive stains
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● CD3, CD8 (usually), CD43, Ki-67, perforin, granzyme B, TIA1, αβ type (βF1-positive)

Negative stains
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● CD56, CD30, EBV, granzyme M (metase)

Genetics and molecular
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● Clonal rearrangements of T cell receptor genes
● No specific cytogenetic abnormalities reported

Differential diagnosis
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● Cutaneous γδ T cell lymphoma: dermal/epidermal involvement, may have epidermal ulceration, CD56+, βF1-

Additional references
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WHO Classification of Skin Tumours, Lyon 2006
● Review articles: Am J Surg Pathol 2004;28:719, Mod Pathol 2002;15:625 (apoptosis and proliferation), Blood 2008;111:838, Cancer 2004;101:1404

End of Lymphoma - Non B cell neoplasms > T/NK cell disorders > Subcutaneous panniculitis-like T cell lymphoma


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