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Clinical features and outcome of primary pancreatic lymphoma.

ABSTRACT: 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.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.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 %.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).

SUBMITTER: Sadot E

PROVIDER: S-EPMC4574284 | biostudies-literature | 2015 Apr

REPOSITORIES: biostudies-literature

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Clinical features and outcome of primary pancreatic lymphoma.

Sadot Eran E Yahalom Joachim J Do Richard Kinh Gian RK Teruya-Feldstein Julie J Allen Peter J PJ Gönen Mithat M D'Angelica Michael I MI Kingham T Peter TP Jarnagin William R WR DeMatteo Ronald P RP

Annals of surgical oncology 20141024 4

<h4>Introduction</h4>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.<h4>Methods</h4>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 ...[more]

PMID: 25341750

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Project description:ObjectivesTo characterize the clinical features and outcomes of childhood-onset primary Sjögren's syndrome (pSS).MethodsPatients less than 18 years old who were diagnosed with pSS by paediatric rheumatologists were included, and all patients were applied the 2002 American-European Consensus Group (ACEG) criteria, the 2016 American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria for pSS, or the 1999 proposed juvenile pSS criteria. The electronic medical records of patients with pSS from 2013 to 2020 were collected and analysed.ResultsThirty-nine patients were included. Of them, 27 (69.2%), 38 (97.4%) and 35 (89.7%) patients fulfilled the AECG criteria, ACR/EULAR criteria and proposed juvenile pSS criteria, respectively. The female:male ratio was 3.9:1. The median ages at first signs or symptoms and at diagnosis were 9.2 (4.7, 14.5) years and 10.9 (6.3, 15.0) years, respectively. The main clinical manifestations were rash or purpura (20, 51.3%), followed by fever (12, 30.8%), glandular enlargement/recurrent parotitis (10, 25.6%), and dry mouth and/or dry eyes (9, 23.1%). Twenty-eight (56.4%) patients had systemic damage, the most common of which was haematological involvement (14, 35.9%), followed by hepatic (13, 33.3%) and renal involvement (8, 20.5%). Thirty-eight (97.4%) patients underwent labial minor salivary gland biopsy, and all exhibited focal lymphocytic sialadenitis. All patients had a global ESSDAI score ≥ 1 at diagnosis, and the median total score at diagnosis was 8 (2, 31). Thirty-six (92.3%) patients were followed up for a median time of 23.6 (7.9, 79.5) months, and three patients developed systemic lupus erythematosus (SLE) at follow-up times of 13.3, 38.8 and 63.8 months.ConclusionsThe presentation of childhood-onset pSS is atypical, and extraglandular manifestations and systemic involvement are more common than in adult-onset pSS. Labial salivary gland biopsy is vital for patients with probable pSS. Some patients may develop SLE over time.

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Clinical features and outcome of primary pancreatic lymphoma.

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Clinical features and outcome of primary pancreatic lymphoma.

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