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PPoma

MedGen UID:
577392
Concept ID:
C0346407
Neoplastic Process
Synonyms: pancreatic polypeptide neoplasm; Pancreatic Polypeptide Tumor; pancreatic polypeptide tumor; pancreatic polypeptide tumour; Pancreatic polypeptidoma; pancreatic polypeptidoma
SNOMED CT: Pancreatic polypeptidoma (255039001)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0019957
Orphanet: ORPHA97278

Definition

PPoma is a type of pancreatic endocrine tumor (see this term) that hypersecretes pancreatic polypeptide (PP) but that does not cause a hypersecretion syndrome (is non-functioning) and instead presents with only non-specific symptoms such as weight loss, abdominal pain, jaundice, diarrhea and/or an abdominal mass, hence leading to a late diagnosis. PPoma can be associated with multiple endocrine neoplasia 1 (MEN-1; see this term). [from ORDO]

Professional guidelines

PubMed

Fiasse R, Pauwels S, Rahier J, Jamar F, Ketelslegers JM, Hassoun A, de Longueville M
Acta Gastroenterol Belg 1993 May-Aug;56(3-4):279-91. PMID: 8266771

Recent clinical studies

Etiology

Talmon GA, Wren JD, Nguyen CL, Pour PM
Pancreas 2017 Jul;46(6):820-824. doi: 10.1097/MPA.0000000000000844. PMID: 28609372
Poma PA
J Reprod Med 2000 Mar;45(3):213-8. PMID: 10756499
Poma PA
Obstet Gynecol 1998 Jun;91(6):1013-8. doi: 10.1016/s0029-7844(98)00077-5. PMID: 9611015
Fiasse R, Pauwels S, Rahier J, Jamar F, Ketelslegers JM, Hassoun A, de Longueville M
Acta Gastroenterol Belg 1993 May-Aug;56(3-4):279-91. PMID: 8266771

Diagnosis

Talmon GA, Wren JD, Nguyen CL, Pour PM
Pancreas 2017 Jul;46(6):820-824. doi: 10.1097/MPA.0000000000000844. PMID: 28609372
Tomita T
Islets 2010 Mar-Apr;2(2):82-8. doi: 10.4161/isl.2.2.10807. PMID: 21099299
Kuo SC, Gananadha S, Scarlett CJ, Gill A, Smith RC
World J Surg 2008 Aug;32(8):1815-22. doi: 10.1007/s00268-008-9499-7. PMID: 18521664
Metz DC
Ital J Gastroenterol Hepatol 1999 Oct;31 Suppl 2:S153-9. PMID: 10604121
Bloom SR, Polak JM, Welbourn RB
World J Surg 1979 Sep 20;3(5):587-95. doi: 10.1007/BF01654765. PMID: 516776

Therapy

Talmon GA, Wren JD, Nguyen CL, Pour PM
Pancreas 2017 Jul;46(6):820-824. doi: 10.1097/MPA.0000000000000844. PMID: 28609372
Mortenson M, Bold RJ
Int J Gastrointest Cancer 2002;32(2-3):153-6. doi: 10.1385/IJGC:32:2-3:153. PMID: 12794252
Poma PA
J Reprod Med 1999 Aug;44(8):657-68. PMID: 10483534
Keymeulen B, Bossuyt A, Peeters TL, Somers G
Ann Nucl Med 1995 Aug;9(3):149-52. doi: 10.1007/BF03165043. PMID: 8534589
Fiasse R, Pauwels S, Rahier J, Jamar F, Ketelslegers JM, Hassoun A, de Longueville M
Acta Gastroenterol Belg 1993 May-Aug;56(3-4):279-91. PMID: 8266771

Prognosis

Talmon GA, Wren JD, Nguyen CL, Pour PM
Pancreas 2017 Jul;46(6):820-824. doi: 10.1097/MPA.0000000000000844. PMID: 28609372
Metz DC
Ital J Gastroenterol Hepatol 1999 Oct;31 Suppl 2:S153-9. PMID: 10604121
Poma PA
J Reprod Med 1999 Aug;44(8):657-68. PMID: 10483534
Fiasse R, Pauwels S, Rahier J, Jamar F, Ketelslegers JM, Hassoun A, de Longueville M
Acta Gastroenterol Belg 1993 May-Aug;56(3-4):279-91. PMID: 8266771

Clinical prediction guides

Poma PA
J Reprod Med 1999 Aug;44(8):657-68. PMID: 10483534
Keymeulen B, Bossuyt A, Peeters TL, Somers G
Ann Nucl Med 1995 Aug;9(3):149-52. doi: 10.1007/BF03165043. PMID: 8534589

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