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Published in: Annals of Surgical Oncology 4/2015

01-04-2015 | Pancreatic Tumors

Clinical Features and Outcome of Primary Pancreatic Lymphoma

Authors: Eran Sadot, MD, Joachim Yahalom, MD, Richard Kinh Gian Do, MD, PhD, Julie Teruya-Feldstein, MD, Peter J. Allen, MD, Mithat Gönen, PhD, Michael I. D’Angelica, MD, T. Peter Kingham, MD, William R. Jarnagin, MD, Ronald P. DeMatteo, MD

Published in: Annals of Surgical Oncology | Issue 4/2015

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Abstract

Introduction

Primary pancreatic lymphoma (PPL) is a rare tumor that is often misdiagnosed. Clinicopathologic features, optimal therapy, and outcomes are not well defined. We reviewed our institutional experience with PPL.

Methods

Search of our institutional database identified that between 1987–2012, 21,760 patients with lymphoma and 11,286 patients with a primary pancreatic tumor were evaluated. There were 44 patients with pathologically confirmed PPL. Clinical data were obtained by chart review and survival distributions were estimated using the Kaplan–Meier method and compared using the log-rank test.

Results

At baseline, LDH was elevated in 55 % of the patients, CA 19-9 in 25 %, and CEA in 20 %. Imaging characteristics included large, unresectable tumors (67 %), and lymphadenopathy inferior to the renal vein (50 %). Twenty-three patients underwent surgery for resection (5), diagnosis (13), or palliation (5). Chemotherapy alone achieved a 75 % complete response rate. Eight patients experienced relapse, 88 % of which occurred at distant sites. Median overall survival was 6.1 years and 10-year disease-specific survival (DSS) was 69 %. Patients with a low risk International Prognostic Index (IPI) and those with a follicular histologic subtype demonstrated 5-year DSS of 100 %.

Conclusions

Chemotherapy for PPL results in a high complete response rate and long DSS, which is similar to nodal non-Hodgkin’s lymphoma (NHL). A favorable outcome is expected for IPI low risk patients and follicular histologic subtype. Systemic therapy should generally be the initial therapy when the diagnosis is known. Prolonged follow up is recommended to detect relapses. Surgery alone should be reserved for non-curative intent (i.e. diagnostic or palliative).
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Literature
1.
go back to reference Baylor SM, Berg JW. Cross-classification and survival characteristics of 5,000 cases of cancer of the pancreas. J Surg Oncol. 1973;5(4):335–358.CrossRefPubMed Baylor SM, Berg JW. Cross-classification and survival characteristics of 5,000 cases of cancer of the pancreas. J Surg Oncol. 1973;5(4):335–358.CrossRefPubMed
2.
go back to reference Freeman C, Berg JW, Cutler SJ. Occurrence and prognosis of extranodal lymphomas. Cancer. 1972;29(1):252–260.CrossRefPubMed Freeman C, Berg JW, Cutler SJ. Occurrence and prognosis of extranodal lymphomas. Cancer. 1972;29(1):252–260.CrossRefPubMed
3.
go back to reference Behrns KE, Sarr MG, Strickler JG. Pancreatic lymphoma: is it a surgical disease? Pancreas. 1994;9(5):662–667.CrossRefPubMed Behrns KE, Sarr MG, Strickler JG. Pancreatic lymphoma: is it a surgical disease? Pancreas. 1994;9(5):662–667.CrossRefPubMed
4.
go back to reference Dawson IM, Cornes JS, Morson BC. Primary malignant lymphoid tumours of the intestinal tract: report of 37 cases with a study of factors influencing prognosis. Br J Surg. 1961;49:80–89.CrossRefPubMed Dawson IM, Cornes JS, Morson BC. Primary malignant lymphoid tumours of the intestinal tract: report of 37 cases with a study of factors influencing prognosis. Br J Surg. 1961;49:80–89.CrossRefPubMed
6.
go back to reference Zucca E. Extranodal lymphoma: a reappraisal. Ann Oncol. 2008;19 Suppl 4:iv77–80.PubMed Zucca E. Extranodal lymphoma: a reappraisal. Ann Oncol. 2008;19 Suppl 4:iv77–80.PubMed
7.
go back to reference Seymour JF. X. Extra-nodal lymphoma in rare localisations: bone, breast and testes. Hematol Oncol. 2013;31 Suppl 1:60–63.CrossRefPubMed Seymour JF. X. Extra-nodal lymphoma in rare localisations: bone, breast and testes. Hematol Oncol. 2013;31 Suppl 1:60–63.CrossRefPubMed
8.
go back to reference Zucca E, Conconi A, Mughal TI, et al. Patterns of outcome and prognostic factors in primary large-cell lymphoma of the testis in a survey by the International Extranodal Lymphoma Study Group. J Clin Oncol. 2003;21(1):20–27.CrossRefPubMed Zucca E, Conconi A, Mughal TI, et al. Patterns of outcome and prognostic factors in primary large-cell lymphoma of the testis in a survey by the International Extranodal Lymphoma Study Group. J Clin Oncol. 2003;21(1):20–27.CrossRefPubMed
9.
go back to reference Gundrum JD, Mathiason MA, Moore DB, Go RS. Primary testicular diffuse large B-cell lymphoma: a population-based study on the incidence, natural history, and survival comparison with primary nodal counterpart before and after the introduction of rituximab. J Clin Oncol. 2009;27(31):5227–5232.CrossRefPubMed Gundrum JD, Mathiason MA, Moore DB, Go RS. Primary testicular diffuse large B-cell lymphoma: a population-based study on the incidence, natural history, and survival comparison with primary nodal counterpart before and after the introduction of rituximab. J Clin Oncol. 2009;27(31):5227–5232.CrossRefPubMed
10.
go back to reference Battula N, Srinivasan P, Prachalias A, Rela M, Heaton N. Primary pancreatic lymphoma: diagnostic and therapeutic dilemma. Pancreas. 2006;33(2):192–194.CrossRefPubMed Battula N, Srinivasan P, Prachalias A, Rela M, Heaton N. Primary pancreatic lymphoma: diagnostic and therapeutic dilemma. Pancreas. 2006;33(2):192–194.CrossRefPubMed
11.
12.
go back to reference Webb TH, Lillemoe KD, Pitt HA, Jones RJ, Cameron JL. Pancreatic lymphoma. Is surgery mandatory for diagnosis or treatment? Ann Surg. 1989;209(1):25–30.CrossRefPubMedCentralPubMed Webb TH, Lillemoe KD, Pitt HA, Jones RJ, Cameron JL. Pancreatic lymphoma. Is surgery mandatory for diagnosis or treatment? Ann Surg. 1989;209(1):25–30.CrossRefPubMedCentralPubMed
13.
go back to reference Koniaris LG, Lillemoe KD, Yeo CJ, et al. Is there a role for surgical resection in the treatment of early-stage pancreatic lymphoma? J Am Coll Surg. 2000;190(3):319–330.CrossRefPubMed Koniaris LG, Lillemoe KD, Yeo CJ, et al. Is there a role for surgical resection in the treatment of early-stage pancreatic lymphoma? J Am Coll Surg. 2000;190(3):319–330.CrossRefPubMed
14.
go back to reference Bouvet M, Staerkel GA, Spitz FR, et al. Primary pancreatic lymphoma. Surgery. 1998;123(4):382–390.CrossRefPubMed Bouvet M, Staerkel GA, Spitz FR, et al. Primary pancreatic lymphoma. Surgery. 1998;123(4):382–390.CrossRefPubMed
15.
go back to reference Mishra MV, Keith SW, Shen X, Bar Ad V, Champ CE, Biswas T. Primary pancreatic lymphoma: a population-based analysis using the SEER program. Am J Clin Oncol. 2013;36(1):38–43.CrossRefPubMed Mishra MV, Keith SW, Shen X, Bar Ad V, Champ CE, Biswas T. Primary pancreatic lymphoma: a population-based analysis using the SEER program. Am J Clin Oncol. 2013;36(1):38–43.CrossRefPubMed
16.
go back to reference Swerdlow SH, Campo E, Harris NL, et al. WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon: IARC Press; 2008. Swerdlow SH, Campo E, Harris NL, et al. WHO classification of tumours of haematopoietic and lymphoid tissues. Lyon: IARC Press; 2008.
17.
go back to reference Lister TA, Crowther D, Sutcliffe SB, et al. Report of a committee convened to discuss the evaluation and staging of patients with Hodgkin’s disease: Cotswolds meeting. J Clin Oncol. 1989;7(11):1630–1636.PubMed Lister TA, Crowther D, Sutcliffe SB, et al. Report of a committee convened to discuss the evaluation and staging of patients with Hodgkin’s disease: Cotswolds meeting. J Clin Oncol. 1989;7(11):1630–1636.PubMed
18.
go back to reference A predictive model for aggressive non-Hodgkin’s lymphoma. The International Non-Hodgkin’s Lymphoma Prognostic Factors Project. N Engl J Med. 1993;329(14):987–994. A predictive model for aggressive non-Hodgkin’s lymphoma. The International Non-Hodgkin’s Lymphoma Prognostic Factors Project. N Engl J Med. 1993;329(14):987–994.
19.
go back to reference Cheson BD, Pfistner B, Juweid ME, et al. Revised response criteria for malignant lymphoma. J Clin Oncol. 2007;25(5):579–586.CrossRefPubMed Cheson BD, Pfistner B, Juweid ME, et al. Revised response criteria for malignant lymphoma. J Clin Oncol. 2007;25(5):579–586.CrossRefPubMed
20.
go back to reference Allen PJ. Pancreatic adenocarcinoma: putting a hump in survival. J Am Coll Surg. 2007;205(4 Suppl):S76–80.CrossRefPubMed Allen PJ. Pancreatic adenocarcinoma: putting a hump in survival. J Am Coll Surg. 2007;205(4 Suppl):S76–80.CrossRefPubMed
21.
22.
go back to reference Kamisawa T, Chari ST, Lerch MM, Kim MH, Gress TM, Shimosegawa T. Recent advances in autoimmune pancreatitis: type 1 and type 2. Gut. 2013;62(9):1373–1380.CrossRefPubMed Kamisawa T, Chari ST, Lerch MM, Kim MH, Gress TM, Shimosegawa T. Recent advances in autoimmune pancreatitis: type 1 and type 2. Gut. 2013;62(9):1373–1380.CrossRefPubMed
24.
go back to reference Ahmad SS, Idris SF, Follows GA, Williams MV. Primary testicular lymphoma. Clin Oncol. 2012;24(5):358–365.CrossRef Ahmad SS, Idris SF, Follows GA, Williams MV. Primary testicular lymphoma. Clin Oncol. 2012;24(5):358–365.CrossRef
25.
26.
go back to reference Gholam D, Bibeau F, El Weshi A, Bosq J, Ribrag V. Primary breast lymphoma. Leuk Lymphoma. 2003;44(7):1173–1178.CrossRefPubMed Gholam D, Bibeau F, El Weshi A, Bosq J, Ribrag V. Primary breast lymphoma. Leuk Lymphoma. 2003;44(7):1173–1178.CrossRefPubMed
27.
go back to reference Ferrucci PF, Zucca E. Primary gastric lymphoma pathogenesis and treatment: what has changed over the past 10 years? Br J Haematol. 2007;136(4):521–538.CrossRefPubMed Ferrucci PF, Zucca E. Primary gastric lymphoma pathogenesis and treatment: what has changed over the past 10 years? Br J Haematol. 2007;136(4):521–538.CrossRefPubMed
28.
go back to reference Habermann TM, Weller EA, Morrison VA, et al. Rituximab-CHOP versus CHOP alone or with maintenance rituximab in older patients with diffuse large B-cell lymphoma. J Clin Oncol. 2006;24(19):3121–3127.CrossRefPubMed Habermann TM, Weller EA, Morrison VA, et al. Rituximab-CHOP versus CHOP alone or with maintenance rituximab in older patients with diffuse large B-cell lymphoma. J Clin Oncol. 2006;24(19):3121–3127.CrossRefPubMed
29.
go back to reference Ziepert M, Hasenclever D, Kuhnt E, et al. Standard International Prognostic Index remains a valid predictor of outcome for patients with aggressive CD20+B-cell lymphoma in the rituximab era. J Clin Oncol. 2010;28(14):2373–2380.CrossRefPubMed Ziepert M, Hasenclever D, Kuhnt E, et al. Standard International Prognostic Index remains a valid predictor of outcome for patients with aggressive CD20+B-cell lymphoma in the rituximab era. J Clin Oncol. 2010;28(14):2373–2380.CrossRefPubMed
31.
go back to reference Maurer MJ, Ghesquieres H, Jais JP, et al. Event-free survival at 24 months is a robust end point for disease-related outcome in diffuse large B-cell lymphoma treated with immunochemotherapy. J Clin Oncol. 2014;32(10):1066–1073.CrossRefPubMed Maurer MJ, Ghesquieres H, Jais JP, et al. Event-free survival at 24 months is a robust end point for disease-related outcome in diffuse large B-cell lymphoma treated with immunochemotherapy. J Clin Oncol. 2014;32(10):1066–1073.CrossRefPubMed
32.
go back to reference Ryan G, Martinelli G, Kuper-Hommel M, et al. Primary diffuse large B-cell lymphoma of the breast: prognostic factors and outcomes of a study by the International Extranodal Lymphoma Study Group. Ann Oncol. 2008;19(2):233–241.CrossRefPubMed Ryan G, Martinelli G, Kuper-Hommel M, et al. Primary diffuse large B-cell lymphoma of the breast: prognostic factors and outcomes of a study by the International Extranodal Lymphoma Study Group. Ann Oncol. 2008;19(2):233–241.CrossRefPubMed
33.
go back to reference Lee AY, Connors JM, Klimo P, O’Reilly SE, Gascoyne RD. Late relapse in patients with diffuse large-cell lymphoma treated with MACOP-B. J Clin Oncol. 1997;15(5):1745–1753.PubMed Lee AY, Connors JM, Klimo P, O’Reilly SE, Gascoyne RD. Late relapse in patients with diffuse large-cell lymphoma treated with MACOP-B. J Clin Oncol. 1997;15(5):1745–1753.PubMed
34.
go back to reference Larouche JF, Berger F, Chassagne-Clement C, et al. Lymphoma recurrence 5 years or later following diffuse large B-cell lymphoma: clinical characteristics and outcome. J Clin Oncol. 2010;28(12):2094–2100.CrossRefPubMed Larouche JF, Berger F, Chassagne-Clement C, et al. Lymphoma recurrence 5 years or later following diffuse large B-cell lymphoma: clinical characteristics and outcome. J Clin Oncol. 2010;28(12):2094–2100.CrossRefPubMed
Metadata
Title
Clinical Features and Outcome of Primary Pancreatic Lymphoma
Authors
Eran Sadot, MD
Joachim Yahalom, MD
Richard Kinh Gian Do, MD, PhD
Julie Teruya-Feldstein, MD
Peter J. Allen, MD
Mithat Gönen, PhD
Michael I. D’Angelica, MD
T. Peter Kingham, MD
William R. Jarnagin, MD
Ronald P. DeMatteo, MD
Publication date
01-04-2015
Publisher
Springer US
Published in
Annals of Surgical Oncology / Issue 4/2015
Print ISSN: 1068-9265
Electronic ISSN: 1534-4681
DOI
https://doi.org/10.1245/s10434-014-4176-6

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