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HANDOUT 3 RARITIES CASE 10: DIAGNOSIS SYRINGOTROPIC CUTANEOUS T-CELL LYMPHOMA CLINICAL •Rare form of cutaneous lymphoma (only ~12 cases reported to date) •Mostly males (10/12 cases) •Single or multiple scaly patches of plaques •Alopecia usual •Hypoaesthesia & anhidrosis in some •Historical cases of syringolymphoid hyperplasia probably the same thing PATHOLOGY •No epidermotropism •Dense lymphocytic infiltrate •Hyperplasia and infiltration of sweat duct epithelium •Probably a variant of mycosis fungoides Sweat duct infiltration seen in some cases of follicular MF FURTHER READING Tannous et al. J AM Acad Dermatol 1999; 41: 303 Zelger et al. Br J Dermatol 1994; 130: 765 Ah-Weng et al. Br J Dermatol 2003; 148: 349 CASE 11: ADDITIONAL FINDINGS IMMUNOPHENOTYPE •CD3, CD5, CD7, CD8 positive •CD2, CD4, CD30, CD45RO, CD56 negative PCR •Monoclonal TCR-g re-arrangement DIAGNOSIS PRIMARY CUTANEOUS CD8-POSITIVE EPIDERMOTROPIC T-CELL LYMPHOMA CD8 EXPRESSION IN PRIMARY CTCL Majority of CTCL CD3+CD4+CD8- phenotype CD8 expressed in proportion of cases of well defined entities more frequently CD4+: •Pagetoid reticulosis •Mycosis fungoides •CD30+ lymphoproliferations •CD30- large T-cell lymphoma •CD30- small/medium pleomorphic CTCL 50% rare rare 12% 21% NO effect on prognosis except perhaps for CD30small/medium pleomorphic CTCL and only if localised CD8 TYPICALLY EXPRESSED IN: •Subcutaneous panniculitis-like T-cell lymphoma •Primary cutaneous CD8-positive epidermotropic cytotoxic T-cell lymphoma PRIMARY CUTANEOUS CD8-POSITIVE EPIDERMOTROPIC T-CELL LYMPHOMA •NO history of previous or concurrent MF •Eruptive papules, nodules, tumours •Central ulceration •Aggressive clinical course •Spread to other extranodal sites rather than lymph nodes •Median survival 32 months in one recent series PATHOLOGY •Band-like or nodular infiltrates •Prominent epidermotropism with follicular and sweat duct involvement •Small, medium or large lymphocytes IMMUNOPHENOTYPE •CD3, CD8, TIA-1 positive •CD7, CD56 ++/- •CD2, CD5 --/+ •CD4, CD45RO negative CD7 positive cases said to have poorer prognosis than CD7 negative cases FURTHER READING Berti et al. Am J Pathol 1999; 155: 483 Agnarsson et al. J Am Acad Dermatol 1990; 22: 569 CASE 12: ADDITIONAL FINDINGS SPINDLE CELLS POSITIVE WITH ANTIBODIES TO: •CD45, CD20, CD10, BCL-6 SPINDLE CELLS NEGATIVE WITH ANTIBODIES TO: •Cytokeratin, •S-100/melanA, •CD23, CD35 •Pan-actin, SMA, desmin •CD34 DIAGNOSIS SPINDLE-CELL B-CELL LYMPHOMA SPINDLE-CELL LYMPHOMA •A pattern of growth NOT a distinct entity •Variety of lymphoma subtypes in a variety of sites •Often associated with dense fibrous tissue e.g. bone, mediastinum •Very rare in absence of sclerosis •In skin majority B-cell type and follicle centre cell origin DIFFERENTIAL DIAGNOSIS Other cutaneous spindle cell tumours •True sarcomas – primary or secondary •Spindle cell squamous carcinoma •Spindle cell melanoma •Atypical fibroxanthoma RECOGNITION EASY WITH IMMUNO PROVIDED POSSIBILITY CONSIDERED CD21 IMMUNOSTAINING Typically used as a marker for FDC (& FDC sarcoma) A normal B-cell differentiation antigen •C3d receptor •Said not to be detectable in routinely processed, paraffin embedded tissues •CAN be detected with newer more sensitive antigen retrieval techniques FURTHER READING Cerroni et al. Am J Dermatopathol 2000; 22: 299 Goodlad. Br J Dermatol 2001; 145: 313 Wang et al. Histopathology 2001; 39: 476