SH/EAHP Workshop 2011 Los Angeles, California, USA

Transcription

1 SH/EAHP Workshop 2011 Los Angeles, California, USA October 27-29, 2011 Session 3 Non-Mycosis Fungoides CTCL Patty Jansen & Rein Willemze

2 Introduction Submitted: 101 cases + 7 cases group 1: 108 Deactivated or not evaluable: 7 cases Total number of evaluable cases: 101

3 Session 3: Diagnosis (oral) PCSM-TCL/pseudo-T-cell lymphoma: 20 (3) Subcutaneous panniculitis-like TCL: 7 (2) Cutaneous gamma/delta TCL: 5 (2) Aggressive CD8+ CTCL: 10 (1+) Extranodal NK/T-cell lymphoma: 12 (2) Hydroa vacciniforme(-like TCL): 3 (2) Angioimmunoblastic T-cell lymphoma: 5 (2) Cutaneous PTCL, NOS: 18 (2) T-PLL: 6 (1) Adult T-cell leukemia/lymphoma: 4 (1)

4 Session 3: Diagnosis Other: Lymphomatoid papulosis (CD8+) (2 cases) PTLD, T/NK type Clonal EBV+ T-cell lymphoproliferation Sezary syndrome Atypical MF (TCRϒ positive) Precursor T-lymphoblastic lymphoma/leukemia Hepatosplenic T-cell lymphoma Aggressive CTCL NOS (CD79+)

5 Session 3: Selection orals (n=18) Sufficient biopsy sections / immunostainings Sufficient clinical data (preferably clinical pictures) to allow clinicopathologic correlation. Teaching value Food for discussion. Real beauties (too many of them).

6 Session 3: Selection orals (n=18) PCSM-TCL or pseudo-t (204,113,136) SPTCL versus CGD-TCL (274,161,122,213) CD8+ CTCL (308, 89, 305). Extranodal NK/T (3, 132) Hydroa vacciniforme(-tcl) (56, 177) AITL (83, 185) PTCL, NOS (160, 278) ATLL (314) T-PLL (224)

7 Session 3 Case 204 Submitted by Karen Grogg, MD, Mayo Clinic, Rochester as: Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma Panel diagnosis: Comes within 10 minutes

8 PCSM-TCL / pseudo-t (n=20) No Solitary Head/neck Clonal Band-like (drug-associated) 2 2/2 0/2 2/2 Nodular/diffuse 16 15/16 15/16 15/16 Perivascular/ periadnexal 2 0/2 0/2 0/1 In common: many admixed B, CD8+ T, histiocytes; low proliferative fraction; PD-1 expression; excellent prognosis.

9 Session 3 Case 204 Submitted by Karen Grogg, MD, Mayo Clinic, Rochester as: Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma Panel diagnosis: cutaneous CD4+ small/medium-sized pleomorphic T-cell proliferation

10 Session 3 Case 113 Submitted by Trent B. Marburger, MD, cleveland clinic as: Cutaneous atypical lymphoid hyperplasia (secondary to prolonged anticonvulsant therapy) Panel diagnosis: Lymphomatoid drug reaction

11 Session 3 Case 136 Submitted by Sarah L. Ondrejeka, MD, Cleveland Clinic as: Primary cutaneous CD4+ small/medium-sized pleomorphic T-cell lymphoma Panel diagnosis: Cutaneous CD4+ small/medium-sized pleomorphic T-cell proliferation

12 Cutaneous CD4+ S/M pleomorphic T- cell proliferation Solitary lesion (head/neck) Both for cases with a band-like pattern and cases with a nodular/diffuse pattern. Predominance CD4+ s/m pleomorphic T-cells Admixed CD4+ blast cells (<30%) (PD-1+) Many admixed B, CD8+ T and histiocytes. Clonal T-cells (90%) NO marker loss (except CD7). MANAGEMENT: No staging; non-aggressive tx; FU up to 2 yrs

13 Term PCSM-TCL maintained for: Cases that are different, e.g.: Other clinical presentation (eg. rapidly growing tumors) Marker loss (except CD7) High proliferative fraction (>50%) Few admixed B-cells, histiocytes, CD8+ T- cells.

14 Session 3 Case 274 Submitted by Mark Cappel, MD, Mayo Clinic, Florida as: Subcutaneous panniculitis-like T-cell lymphoma Panel diagnosis: Subcutaneous panniculitis-like T-cell lymphoma

15 Session 3 Case 161 Submitted by Alison Huppmann, MD, NCI/NIH, Bethesda: as: Subcutaneous panniculitis-like T-cell lymphoma Panel diagnosis: Subcutaneous panniculitis-like T-cell lymphoma

16 Session 3 Case 122 Submitted by Joo Y. Song, MD, NCI/NIH, Bethesda: Primary cutaneous gamma/delta T-cell lymphoma Panel diagnosis: Primary cutaneous gamma/delta T-cell lymphoma

17 Session 3 Case 213 Submitted by Alden R. Webb, MD, Indiana University School of Medicine as: Primary cutaneous gamma/delta T-cell lymphoma Panel diagnosis: Primary cutaneous gamma/delta T-cell lymphoma

18 Session 3 Case 308 Submitted by Paul Rodriguez-Waitkus, MD, Baylor College of Medicine, Houston as: Cutaneous CD8+ cytotoxic T-cell lymphoma, with overlap features between cutaneous aggressive epidermotropic CD8+ cytotoxic T cell lymphoma and small/medium size pleomorphic T cell lymphoma (CD8 phenotype). Panel diagnosis: Primary cutaneous CD8+ aggressive epidermotropic cytotoxic T-cell lymphoma

19 Session 3 CD8+ CTCL Case 89 Case 305

20 L U M C Aggressive epidermotropic CD8+ CTCL Definition: Proliferation of epidermotropic CD8+ cytotoxic T- cells and an aggressive clinical behavior No signs or history of MF or CD30+ LPD (LyP)

21 L U M C Aggressive epidermotropic CD8+ CTCL Clinical features: Generalized plaques and tumors; ulceration common Dissemination to unusual visceral sites Poor prognosis; median survival < 3 years Histologic features: Nodular to diffuse infiltrates; strong epidermotropism Small to large pleomorphic cells or blast cells Phenotype: βf1+, CD2-/+, CD3+, CD4-, CD5-, CD7+/-, CD8+, TIA-1+, CD45RA+/-

22 L U M C Aggressive epidermotropic CD8+ CTCL

23 L U M C Aggressive epidermotropic CD8+ CTCL 9 / /1993

24 L U M C Aggressive epidermotropic CD8+ CTCL CD8

25 L U M C Aggressive epidermotropic CD8+ CTCL Februari 1994 April 1994

26 L U M C Aggressive epidermotropic CD8+ CTCL Februari 1995

27 L U M C Aggressive epidermotropic CD8+ CTCL HE CD8 TIA-1 TIA-1 Berti E. et al; Am J Pathol 1999;155:

28 L U M C CTCL with a CD8+ T-cell phenotype Aggressive epidermotropic cytotoxic CD8+ CTCL Classical MF (ca. 5%) # Transformed MF (CD4+ CD8+) Pagetoid reticulosis (ca. 50%) # C-ALCL and LyP (ca. 5%) # SPTCL (> 90%) # the same clinical course as CD4+ cases

29 Session 3 Case 89 Sylvia Hartmann 1, Sylvie Pätzold 2, Martin-Leo Hansmann 1 1 Department of Pathology, 2 Department of Dermatology Goethe University Hospital, Frankfurt Germany 56-year-old male caucasian patient with a history of cutaneous CD8 + T cell lymphoma since Since that time waxing and waning appearance of erythematous nodules which resolve spontaneously within several months Staging: no lymph node or bone marrow involvement The patient refused treatment During that time lesions of different age were biopsied

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33 Plaque at 1 month

34 Plaque at 1 month

35 CD30 CD3 CD8 Ki 67 Plaque at 1 month

36 Plaque at 4 months

37 Plaque at 4 months

38 CD30 CD3 Plaque at 4 months CD8 Ki 67

39 Session 3 Case 89 Sylvia Hartmann 1, Sylvie Pätzold 2, Martin-Leo Hansmann 1 1 Department of Pathology, 2 Department of Dermatology Goethe University Hospital, Frankfurt Germany Panel diagnosis: Lymphomatoid papulosis (CD8+)

40 L U M C EORTC workshop on 63 CD8+ CTCL Aggressive epidermotropic CD8+ CTCL (n=20): 13 M; 7 F; median age: 55 yrs (range yrs). Follow-up: 16/20 died of lymphoma Median survival: 12 months Prognostic factors: Unknown, but: 3 of 4 survivors had presented with a solitary skin lesion (?) One of the survivors had peculiar clinicopathologic features and was planned to be excluded.

41 Session 1 Case 305 Rein Willemze and Patty Jansen, LUMC, Leiden, The Netherlands Man, 51 years Infiltrated plaque right ear for 6 months. No other skin lesions or symptoms.

42 L U M C Patient 38 (PA: R )

43 L U M C Patient 38 (PA: R )

44 L U M C Patient 38 (PA: R ) CD3 CD5 CD8 TIA-1

45 L U M C Histology: Patient 38 (PA: R ) diffuse atypical non-epidermotropic infiltrate. Bandfree zone beneath epidermis medium-sized to large pleomorphic T-cells with prominent nucleoli. Phenotype: CD2+, CD3+, CD4-, CD5-, CD8-, CD56-, TIA-1+, GrB-, CD30- Genetics: clonal TCR gene rearrangement Conclusion: diffuse large T-cell lymphoma; DD: - PTCL, NOS; transformed MF - agressive CD8+ CTCL (non-epidermotr.) Clinic: not consistent with MF; disappeared after biopsy.

46 L U M C Course: Staging: Patient 38 (PA: R ) indolent not performed Diagnosis: Lymphomatoid reaction (pseudo-t-cell lymphoma)? DD: PTCL, NOS (indolent course) Follow-up: no relapse 24 months after diagnosis.?

47 L U M C Indolent CD8-positive lymphoid proliferation of the ear. A distinct primary cutaneous T-cell lymphoma? Petrella T. et al; Am J Surg Pathol 2007;31:

48 Session 1 Case 305 Panel diagnosis: Indolent CD8-positive lymphoid proliferation of the ear

49 L U M C CTCL with a CD8+ T-cell phenotype Aggressive epidermotropic CD8+ CTCL. Classical MF (ca. 5%) # Transformed MF (CD4+ CD8+) Pagetoid reticulosis (ca. 50%) # C-ALCL and LyP (ca. 5%) # SPTCL (ca. 90%) Indolent CD8-positive lymphoid proliferation of the ear. # the same clinical course as CD4+ cases

50 Session 3 Case 3 Submitted by Rebecca King, MD, University of Pennsylvania as: Extranodal NK/T-cell lymphoma, nasal type Panel diagnosis: Extranodal NK/T-cell lymphoma, nasal type

51 Session 3 Case 132 Submitted by Wesley Greaves, MD, MD Anderson Cancer Center as: Extranodal NK/T-cell lymphoma, nasal type Panel diagnosis: Extranodal NK/T-cell lymphoma, nasal type

52 Session 3 Case 56 Submitted by Nidhi Aggarwal, MD, University of Pittsburgh Medical Center as: Hydroa vacciniforme Panel diagnosis: Hydroa vacciniforme

53 Session 3 Case 177 Submitted by Carlos Torres-Cabala, MD, MD Anderson Cancer Center as: Hydroa vacciniforme-like cutaneous T-cell lymphoma Panel diagnosis: Hydroa vacciniforme-like T-cell lymphoma

54 Session 3 Case 83 Submitted by Laetitia Quintanilla-Fend, MD, University of Tübingen, Germany as: Angioimmunoblastic T-cell lymphoma Panel diagnosis: Angioimmunoblastic T-cell lymphoma

55 Session 3 Case 185 Submitted by Nicolas Ortonne, MD, Hopital Henri Mondor, Creteil, France as: Angioimmunoblastic T-cell lymphoma Panel diagnosis: Angioimmunoblastic T-cell lymphoma

56 Session 3 Case 160 Submitted by Armin Jegalian, MD, Cleveland Clinic as: Peripheral T-cell lymphoma, NOS Panel diagnosis: Peripheral T-cell lymphoma, NOS

57 Session 3 Case 278 Submitted by Rashmi Kanagel Shamanna, MD, MD Anderson Cancer Center as: Peripheral T-cell lymphoma, primary from nasal area, with presentation and recurrence in skin of lower extremities Panel diagnosis: Peripheral T-cell lymphoma, NOS

58 Session 3 Case 314 Submitted by Rahel Mathew, MD, University of South Florida as: Adult T-cell leukemia/lymphoma Panel diagnosis: Adult T-cell leukemia/lymphoma

59 Session 3 Case 224 Submitted by Joshua Coleman, MD, Cleveland Clinic as: T-cell Prolymphocytic Leukemia Panel diagnosis: T-cell Prolymphocytic Leukemia