Introduction

Mantle-cell lymphoma (MCL) is a rare B-cell malignancy that usually presents in stage III-IV disease (classical) with poor outcomes even with aggressive therapy. Rarely, patients present with localized disease and the role of localized or systemic therapy remains a clinical choice.

Purpose

To assess outcomes in our patients with early stage MCL with a focus on treatment approaches.

Methods

Patients with early stage (Ann Arbor Stage I or II) between 1992 and 2012 were identified through our MCL database. Demographic , treatment, and outcomes data was collected for each patient. Cox regression was used to identify risk factors for progression and survival. Kaplan-Meier (KM) method was used to estimate median time to progression (TTP) and overall survival (OS), and comparisons were estimated using the log rank test. A p-value of <0.05 was considered significant.

Results

From our database of 300 patients with MCL, 34 were identified to have stage I or II disease (Table 1). 7 received local therapy, consisting of either radiation or resection. 20 received systemic therapy, including 1 who received only rituximab. 7 received a combination of local therapy and systemic therapy, including 2 who received rituximab with radiation.

Table 1

Demographic data in patients with early stage MCL

Characteristics (N=34)  Result 
Age in years (median)  64 (46-84) 
WBC in k/uL (median)  7.6 (4.9-53.2) 
% Male  74% 
% Stage I  56% 
% B symptoms  6% 
% Bulky (>5cm)  12% 
% Blastoid/Pleomorophic  24% 
% Extranodal
GI
NP/OP
Testicular
Lung/Breast 
 62%
38%
38%
14%
10% 
ECOG 0-1  100% 
MIPI Int/High  42%/16% 
Staging CT
PET
Marrow
Endoscopy 
100%
71%
85%
38% 
Treatment Local
Systemic
Combined 
21%
59%
21% 
Characteristics (N=34)  Result 
Age in years (median)  64 (46-84) 
WBC in k/uL (median)  7.6 (4.9-53.2) 
% Male  74% 
% Stage I  56% 
% B symptoms  6% 
% Bulky (>5cm)  12% 
% Blastoid/Pleomorophic  24% 
% Extranodal
GI
NP/OP
Testicular
Lung/Breast 
 62%
38%
38%
14%
10% 
ECOG 0-1  100% 
MIPI Int/High  42%/16% 
Staging CT
PET
Marrow
Endoscopy 
100%
71%
85%
38% 
Treatment Local
Systemic
Combined 
21%
59%
21% 

GI: stomach/intestines; NP: nasopharynx, OP: oropharynx; MIPI: Mantle Cell International Prognostic Index

With a median followup of 73.7 months, TTP was 22.8 months and OS was 83.8 months. MIPI could be determined for 56% of the cohort, however, was neither prognostic for TTP or OS. WBC was available for 74% of the cohort, and was prognostic for OS only [HR 1.46 (95%CI 1.02-2.08, p-value=0.04)]. None of the remaining MIPI covariates were prognostic. Ki67 was reported for only 8 patients limiting analysis as a continuous variable. Blastoid/Pleomorphic histology, alternatively, was prognostic for OS [HR 24.5 (95%CI 1.22-25.72, p-value=0.03)], but not TTP. Locally directed therapy was not associated with a decrease in OS or TTP, with some patients showing no evidence of relapse with extended followup (figure 1).

Conclusions

In our series patients with early stage MCL demonstrated a longer TTP and OS than those with extensive stage disease. The MIPI failed to stratify patients, likely related to small sample size. Aggressive histology may confer a worse prognosis, though, it remains unclear whether this can be abrogated by more intensive management, as all with blastoid/pleomorphic histology received some form of systemic therapy. Notably, as described by others, locally directed therapy did not compromise outcomes, with some showing extended TTP and OS.

Disclosures:

No relevant conflicts of interest to declare.

Author notes

*

Asterisk with author names denotes non-ASH members.

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