cbcl an overview and newer approaches - mcgill.ca
TRANSCRIPT
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CBCL An Overview and Newer Approaches
Steven M. Horwitz M.D.Lymphoma ServiceMemorial Sloan Kettering Cancer Center
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Classification of Cutaneous Lymphomas
Blood 2005;105:3768-85
CUTANEOUS T/NK LYMPHOMAS
Mycosis fungoides
Sezary Syndrome
ATLL
Primary cutaneous CD 30+
Anaplastic large cell
Lymphomatoid papulosis
Subcutaneous panniculitis-like T-cell
Extranodal NK/T-cell
Primary cutaneous peripheral T-cell
Epidermotropic CD8+ T-cell
Cutaneous T-cell
CD4+ pleomorphic T-cell
CUTANEOUS B-CELL LYMPHOMAS
Primary cutaneous marginal zone
Primary cutaneous follicle center
Primary cutaneous diffuse large cell
Leg type
Intravascular
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Primary Cutaneous B Cell Lymphoma
Background
Subtypes
Prognosis
NCCN
Clinical scenarios:
Do we need to stage low grade CBCL?
Use of low dose radiation
PCFCL in nodes
Unusual cases of PC-MZL
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Primary Cutaneous B Cell Lymphoma
Definition:
– Lymphoma presenting in the skin
– No evidence of extracutaneous disease on complete staging at time of diagnosis
Approximately 20-25% of all cutaneous lymphomasPrimary cutaneous B-cell lymphomas have some unique clinical features
– Indolence-slow growth-majority- MZL/FC– Relapses are common, regardless of the treatment-not assoc with
worse prognosis– Relapses are largely confined to the skin
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Primary Cutaneous Marginal Zone B-cell Lymphoma
Clinical Features: M>F (1.4 :1), median age=53
• Red papules, plaques, nodules, rare ulcerations
• Upper extremities > trunk > head
• Slow growth/ spontaneous regression
• Association with Borrelia burgdorferi ? (Europe)– Anecdotal responses to antibiotics
• Therapy-observation, local, topical
• Anecdotes/small series with rituximab, antibiotics, interferon
• Topical corticosteroids, nitrogen mustard
• 5-year survival > 95%
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Primary Cutaneous Marginal Zone B-cell Lymphoma
Pathology
• Non-epidermotropic
• Nodular-diffuse infiltrates
• Some large cells
• Reactive T-cells
• CD20, CD79a,
• CD5, CD10, bcl-6-negative
• Light chain restriction, IGH rearranged
• Cases showing rearrangement 14:18 – IGH on 14 and MLT gene on 18
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CD20 (B-cell)
CD3 (T-cell)
Primary Cutaneous Marginal Zone Lymphoma
Kappa LambdaLight chainrestriction
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PC Follicle Center Lymphoma
• Solitary, grouped, or multifocal plaques or tumor nodules
• Scalp/forehead> trunk >extremities
• Less common disseminated lesions
• Slow growth/ Spontaneous regression
• Rare extracutaneous spread
• Most common; M>F (2.1:1)median age=58
• Excellent prognosis
• 5-yr DSS 95%
Senff NJ, Noordijk EM, Kim YH, et al. Blood 2008;112:1600
WHO monogram, 4th Edition, 2008
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Primary Cutaneous Follicle Center Lymphoma
In contrast:PCLBCL-LT:Monotonous diffuse centroblasts & immunoblastsExtension to subcutaneous fat; MUM-1+, Bcl-2 + Frequent mitosis
Mix of centrocytes and centroblastsEpidermal sparingVarying amounts of large cellsFollicular or diffuseFrequent reactive T-cells
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Immunohistochemistry/Genetics
• Tumor cells express CD20 and CD79
• Tumor cells will express HGAL, bcl-6, and LMO2 consistently
• PC FCL do not consistently express bcl-2 or CD10
• T(14;18) and bcl-2 gene rearrangement not present in most cases of PC FCL
Weinberg O et al. Am J Surg Pathol. 2009 Apr;33(4):591-8.
Xie X et al. Mod Pathol. 2008 Jun;21(6):653-9.
Morales A et al. Am J Dermatopathol. 2008 Oct;30(5):425-30.
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Cutaneous MZL/FC LymphomaIndolent histologies
Treatment N CR Relapse Relapse:EC OS
5/10y
ALL 416 90% 44% 6-11% 96/90%
XRT 217 98% 49%
Chemo +/- XRT
(Variety)
96 80% 52%
Excision 103 97% 35%
Zinzani et al. J Clin Oncol 24:1376-1382. March 2006
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Cutaneous MZL/FC LymphomaLocal therapy: XRT/Excision
All DFS
Treatment N 5 year 10 year
Single 247 64% 51% p<.001
Regional 125 43% 27%
Disseminated 57 45% 36%
Zinzani et al. J Clin Oncol 24:1376-1382. March 2006Reddy et al, ASCO 2007, a8028
Indolent N Relapse
Localized 65 34 52%
Generalized 19 13 68%
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Primary Cutaneous Diffuse Large B-cell Lymphoma, Leg Type
• Rare; rapid growth
Lower extremities> anywhere else
• Especially elderly women(F>M 2:1; age >70)
• Extracutaneous involvement more common
than other lymphomas
• 5 year over-all survival 67%
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Pathology• Dense diffuse infiltrate of large B cells, with obliteration of existing
adnexal structures
• Extends significantly into subcutaneous tissue
• Monomorphous large B cells, with appearance of immunoblasts
• Express CD79, CD20, MUM-1, FOXP1, IgM and IgD
• In some series, strong uniform expression of bcl-2 with variable expression of bcl-6
• Does not usually express CD10
Sundram U et al. J Cutan Pathol. 2005 Mar;32(3):227-34.Kodama K et al. Blood. 2005 Oct 1;106(7):2491-7.
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MUM-1
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CD20
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Bcl-2
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Bcl-6
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Hoefnagel et al Blood 2005;105:3671-3678
Willemze et al Blood, 2005;105:3768-3785
de Leval et al Am J surg Pathol. 2001;25:732-741
Hoefnagel et al Br J Dermatol. 2003:149:1183-1191
Child et al Br J Dermatol. 2001;144:735-744
Cerroni et al J Invest Dermatol. 1994; 102:231-235
FCL LBC-L
CD20/CD 79a + +
Bcl-2 -/+ ++
Bcl-6 + +
CD10 -/+ -
MUM-1 - +
t(14:18) - -
Trans myc (fish) - +/-
ABC GC
Follicle center -Diffuse large B-cell, leg typePathology
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DLBC-Leg Type
Treatment N CR Relapse Relapse:EC OS
5/10y
ALL 51 82% 55% 17% 73/47%
XRT 28 23% 52%
Chemo +/- XRT
(CHOP)
20 80% 63%
Excision 3 100% 33%
Zinzani et al. J Clin Oncol 24:1376-1382. March 2006
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Senff, N. J. et al. Arch Dermatol 2007;143:1520-1526.
disease-specific survivalrelapse-free survival
Results after Primary Radiation for Cutaneous B-cell Lymphoma
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Koens et al. Journal of Investigative DermatologyVolume 134, Issue 1, January 2014, Pages 290-292
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JAMA Dermatol. 2014;150(11):1173-1179. doi:10.1001/jamadermatol.2014.821
MYD88 mutation was not present in 24 patients and was present in 34 patients.(P = .002).
Overall Survival of 58 Patients With Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg-Type According to the MYD88 Mutation
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MarieBeylot-Barry J. Invest Derm Volume 138, Issue 9, September 2018, Pages 1982-1989
Lenalidomide for Cutaneous Diffuse Large B-Cell Lymphoma, Leg-Type
Anecdote with BTK inhibitor
6 had CR and 5 had PR
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What is the utility of initial staging for low grade CBCL?
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Utility of imaging in cutaneous marginal zone lymphoma and cutaneous follicle center lymphoma
Meenal Kheterpal, Shamir Geller, MD1, Julia Dai, Melissa Pulitzer , Patricia Myskowski, Alison Moskowitz, Ai Ni, Jinah Kim, MD Richard Hoppe, MD, Youn H. Kim, Steven M. Horwitz
1Memorial Sloan Kettering Cancer Center, Stanford University School of Medicine
Retrospective review:Patients with cutaneous presentation of FCL (CFCL) diagnosed between 1997-2016
evaluated at MSKCC or at Stanford UniversityPatients with cutaneous presentation of MZL (CMZL) diagnosed between 1998-2016,
evaluated at MSKCC or at Stanford University
Inclusion criteria:CMZL/CFCL confirmed by pathologists at MSKCC / StanfordSkin was the initial site of presentation of the cutaneous lymphomaImaging study (positron emission tomography scan and/or chest, abdomen and pelvic
computed tomography scan) within 12 months as part of the initial workup.
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MZL
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Marginal Zone LymphomaClinical characteristics of patients with systemic involvement at
presentation
45.4
63.6
36.4
0
36.4
9.1
45.536.4
18.227.3
9.10
27.3
54.5
0
10
20
30
40
50
60
70
% o
f p
atie
nts
Clinical Characteristics
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FCCL
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Follicular lymphoma presenting in the skinClinical characteristics of patients with systemic
involvement at presentation89.4
21.1
5.215.8
57.9
21.1 21.16.3
17.6 15.8 21.4
0
33 38.9
0102030405060708090
100%
of
pat
ien
ts
Clinical characteristics
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Conclusions• 3.6% of patients with CMZL vs 8.8% of patients with CFCL histology presenting in the skin
have concurrent extracutaneous disease most consistent with systemic B-cell lymphoma
• Of the patients with systemic involvement at presentation, imaging alone detected 81.8% (9/11) of MZL cases and 89.4% (17/19) of FCL cases.
• Due to the rarity of extracutaneous disease, imaging had a low yield in CMZL in follow up
• Bcl2 positivity is suggestive of systemic nodal B-cell lymphoma in patients with CFCL Additionally, subcutaneous involvement, elevated LDH and lower extremity involvement may be associated with a higher likelihood of systemic disease.
• Large lesions with deep subcutaneous involvement in patients presenting with CMZL may be suggestive of systemic B-cell lymphoma although prospectively collected data is needed for validation
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Use of low dose radiation for low grade CBCL
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Boom Boom Radiation
Treatment
Karen Chau, Joanna Yang, Monica Chelius, Carla Hajj, and Joachim Yahalom
2Gy x 2 RT for Patients with Indolent Lymphomas
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Background• Boom boom is 2Gy x 2 RT
– Option for patients with radiosensitive and indolent lymphomas
• Initially published in 1994 by Ganem, et al.1
– 89% of patients had a response
• Literature– Netherlands study (Haas, et al.2)
• 109 patients (304 sites)• Overall response rate was 92% (61% CR and 31% PR)• Median time to local progression: 42 months
– FORT trial (next slide)1 Ganem, GéRard, et al. "Potential role for low dose limited‐field radiation therapy (2× 2 grays) in advanced low‐grade
non‐Hodgkin's lymphomas." Hematological oncology 12.1 (1994): 1-8.2 Haas, R. L. M., et al. "High response rates and lasting remissions after low-dose involved field radiotherapy in indolent
lymphomas." Journal of clinical oncology 21.13 (2003): 2474-2480.
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FORT trial• 4Gy versus 24Gy radiotherapy for patients with indolent lymphoma
(FORT): a randomized phase 3 non-inferiority trial
– Eligibility
• 18+ years old with FL or MZL and no previous treatment for at least 1 month pre-RT
– Between April 7, 2006, and June 8, 2011
• 24Gy: 299 sites
• 4Gy: 315 sites
– Median f/u: 26 months (range: 0.39 – 75.4)
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FORT trial– Outcomes
• Initial response measured at 12 weeks
– 24Gy: 236 (91%) had a CR/PR
– 4Gy: 227 (81%) had a CR/PR
24Gy 4Gy
Complete response 176 (68%) 137 (49%)
Partial response (>30%) 60 (23%) 90 (32%)
Stable disease (including <30% regression) 22 (8%) 44 (16%)
Progression 2 (<1%) 10 (4%)
Total 260 281
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FORT trial– Outcomes
• 91 local progressions (24Gy group: 21; 4Gy group: 70)• Time to local POD with 4Gy was not non-inferior to 24Gy• 24Gy is more effective than 4Gy but 4Gy remains useful alternative
for palliative Tx
• Difference in local progression-free interval at 3 years: 20%
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Boom Boom: Retrospective Review of Our Experience at MSKCC
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Patient selection• Received 2Gy x 2 RT between 2006 – 2017 at MSKCC
• ≥18 years of age at Dx
• MSKCC confirmed pathology of indolent lymphoma prior to RT
–Follicular lymphoma
–Marginal zone lymphoma
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Histology65%
Follicular Lymphoma33% Marginal Zone
Lymphoma
2% Other
Cohort characteristics
54%Early stage
46%Advanced stageInitial stage (early vs. advance)
40%Stage I
13%Stage II
18%Stage III
29%Stage IVInitial stage (breakdown)
First line treatment66%
Other34%
Boom boom RT
Lesion type (nodal vs. extranodal)60%
Extranodal35%
Nodal
5% Both
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Timeline
2Gy x 2
(Radiation therapy)
Follow-up at 2 months
(Evaluation of initial response)
Further treatment or
f/u
(if needed)
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Response at 2 month f/u (by histology)
CR
PR
NR
Follicular Lymphoma
Response rate: 85%
111 sites
60%(n = 67)
25%(n = 28)
15%(n = 16)
CR
PR
NR
Response rate: 90%
57 sites
72%(n = 41)
18%(n = 10)
10%(n = 6)
Marginal Zone Lymphoma
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CR
PR
NR
Nodal
Response rate: 75%60 sites
58%(n = 35)17%
(n = 10)
25%(n = 15)
Response at 2 month f/u (by lesion type)
CR
PR
NR
Response rate: 94%103 sites
71%(n = 73)
23%(n = 24)
6%(n = 6)
Extranodal
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In-field progression (pts with initial CR/PR)
n = 109
n = 38
p < 0.001
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Conclusion• Patients with FL and MZL in this series appeared to have excellent initial
response
–ORR was 87%
• A complete response is durable
–2 year FFLF: 92%
–5 year FFLF: 85%
• A partial response remains stable/disease-free approximately 50% of the time
• Very low dose radiation appears to be a viable option for pts with PCBCL
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How to approach PC-FCCL when it involves lymph nodes?
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Cutaneous Large B-cell LymphomaOS by Site
Grange et al J Clin Oncol 19:3602-3610, 2001
2
5
5
0
7
5
10
0
%
0 1 2 3 4 5Years
Leg
Non-Leg
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80 yo M with PCFCCL• Multiple lesions – chest and abdomen, 3 mo duration
• Bx outside (1/10): PCFCCL (Large Cells, CD20+ BCL-2+/- BCL-6+ focally, MUM-1 -, CD10+), confirmed also at MSK
• Workup: PET (2/25/2010) : No evidence of FDG-avid lymphoma. CBC, LDH – wnl
• F/U – PR with topical CS, RT was given to a tumor lesion on the back. Local recurrences treated with IL CS
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PC-FCCL• Indolent course whether cells large or small
• Large cells in node (same cells) will be often called DLBCL
• Unclear best way to tell whether at that point its an aggressive or indolent lymphoma
• Clinically?
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Unusual presentations of PC-MZL?
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CD20
CD3
Bcl-2
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PC-MZL
• Antigen stimulation
• Secondary to external trauma/stimulation?
• Bicycle helmet straps
• Right leg prosthesis
• Laser hair removal
• Chandelier
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Primary Cutaneous B Cell Lymphoma
Overview
Do we need to stage low grade CBCL?
Use of low dose radiation-”Boom- Boom”
PCFCL in nodes
Unusual cases of PC-MZL
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MSKCC Cutaneous LymphomaAlison MoskowitzPatricia MyskowskiSarah NoorShamir GellerMeenal KhetterpalJoachim Yahalom-XRT
Dermatopathology
Melissa Pulitzer
Klaus Busam
Travis Hollman
Acknowledgements
Thank you