1. HANDOUT 3
RARITIES

2. CASE 10: DIAGNOSIS
SYRINGOTROPIC CUTANEOUS T-CELL LYMPHOMA

3. 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

4. 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

5. 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

6. CASE 11: ADDITIONAL FINDINGS
IMMUNOPHENOTYPE
CD3, CD5, CD7, CD8 positive
CD2, CD4, CD30, CD45RO, CD56 negative
PCR
Monoclonal TCR-g re-arrangement

7. PRIMARY CUTANEOUS CD8-POSITIVE EPIDERMOTROPIC T-CELL LYMPHOMA
DIAGNOSIS
PRIMARY CUTANEOUS CD8-POSITIVE
EPIDERMOTROPIC T-CELL LYMPHOMA

8. 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 50%
Mycosis fungoides rare
CD30+ lymphoproliferations rare
CD30- large T-cell lymphoma 12%
CD30- small/medium pleomorphic CTCL 21%
NO effect on prognosis except perhaps for CD30- small/medium pleomorphic CTCL and only if localised

9. CD8 TYPICALLY EXPRESSED IN:
Subcutaneous panniculitis-like T-cell lymphoma
Primary cutaneous CD8-positive epidermotropic cytotoxic T-cell lymphoma

10. 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

11. PATHOLOGY Band-like or nodular infiltrates
Prominent epidermotropism with follicular and sweat duct involvement
Small, medium or large lymphocytes

12. 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

13. FURTHER READING Berti et al. Am J Pathol 1999; 155: 483
Agnarsson et al. J Am Acad Dermatol 1990; 22: 569

14. 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

15. DIAGNOSIS
SPINDLE-CELL B-CELL LYMPHOMA

16. 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

17. 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

18. 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

19. 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