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Lipase Hypersecretion Syndrome

MedGen UID:
363934
•Concept ID:
C1881406
•
Disease or Syndrome

Definition

A rare disorder resulting from neoplastic exocrine excess. It is typically seen in 10-15 % of patients with pancreatic acinar cell carcinoma. Clinical signs include subcutaneous fat necrosis, polyarthralgia and osteolytic lesions. Clinical course may proceed to non-bacterial thrombotic endocarditis. Clinical prognosis is poor due to its usual onset with advanced disease. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVLipase Hypersecretion Syndrome

Recent clinical studies

Etiology

Acinar cell carcinoma: a report of 19 cases with a brief review of the literature.
Wang Y, Wang S, Zhou X, Zhou H, Cui Y, Li Q, Zhang L
World J Surg Oncol 2016 Jun 28;14(1):172. doi: 10.1186/s12957-016-0919-0. PMID: 27352960Free PMC Article
Acinar cell carcinoma of the pancreas: an institutional series of resected patients and review of the current literature.
Seth AK, Argani P, Campbell KA, Cameron JL, Pawlik TM, Schulick RD, Choti MA, Wolfgang CL
J Gastrointest Surg 2008 Jun;12(6):1061-7. Epub 2007 Oct 24 doi: 10.1007/s11605-007-0338-1. PMID: 17957440

Diagnosis

A rare case of lipase hypersecretion syndrome.
Sofela J, Hunt WTN, Chaytor R, Stephens M, Sofela A, Harmse D, Chave T
Clin Exp Dermatol 2022 Mar;47(3):635-638. Epub 2021 Dec 10 doi: 10.1111/ced.15023. PMID: 34890070
Lipase hypersecretion syndrome: A distinct form of paraneoplastic syndrome specific to pancreatic acinar carcinomas.
Taskin OC, Adsay V
Semin Diagn Pathol 2019 Jul;36(4):240-245. Epub 2019 Jul 5 doi: 10.1053/j.semdp.2019.07.001. PMID: 31300257
Acinar cell carcinoma of the pancreas: an institutional series of resected patients and review of the current literature.
Seth AK, Argani P, Campbell KA, Cameron JL, Pawlik TM, Schulick RD, Choti MA, Wolfgang CL
J Gastrointest Surg 2008 Jun;12(6):1061-7. Epub 2007 Oct 24 doi: 10.1007/s11605-007-0338-1. PMID: 17957440

Therapy

Acinar cell carcinoma: a report of 19 cases with a brief review of the literature.
Wang Y, Wang S, Zhou X, Zhou H, Cui Y, Li Q, Zhang L
World J Surg Oncol 2016 Jun 28;14(1):172. doi: 10.1186/s12957-016-0919-0. PMID: 27352960Free PMC Article

Prognosis

Acinar cell carcinoma: a report of 19 cases with a brief review of the literature.
Wang Y, Wang S, Zhou X, Zhou H, Cui Y, Li Q, Zhang L
World J Surg Oncol 2016 Jun 28;14(1):172. doi: 10.1186/s12957-016-0919-0. PMID: 27352960Free PMC Article
Acinar cell carcinoma of the pancreas: an institutional series of resected patients and review of the current literature.
Seth AK, Argani P, Campbell KA, Cameron JL, Pawlik TM, Schulick RD, Choti MA, Wolfgang CL
J Gastrointest Surg 2008 Jun;12(6):1061-7. Epub 2007 Oct 24 doi: 10.1007/s11605-007-0338-1. PMID: 17957440

Clinical prediction guides

Acinar cell carcinoma: a report of 19 cases with a brief review of the literature.
Wang Y, Wang S, Zhou X, Zhou H, Cui Y, Li Q, Zhang L
World J Surg Oncol 2016 Jun 28;14(1):172. doi: 10.1186/s12957-016-0919-0. PMID: 27352960Free PMC Article

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