PPoma

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).



Input patient's signs and symptoms


Narrow down the case reports



Total: 5 (papers)

   


(per page)
Matched Phenotype  Gene  Mutation  MeSH
Rank
(Similarity)		 PMID
(PMCID)
1
(4.0%)		 28609372
 Pancreatic Polypeptide Cell Proliferation in the Pancreas and Duodenum Coexisting in a Patient With Pancreatic Adenocarcinoma Treated With a GLP-1 Analog.
Talmon GA, Wren JD, Nguyen CL, Pour PM.
Pancreas. 2017;46(6):820-824.
Diabetes mellitus
GLP1R PPY
Adenocarcinoma Cell Proliferation Chemotherapy, Adjuvant Diabetes Mellitus, Non-Insulin-Dependent Duodenum Fatal Outcome Homo sapiens Hyperplasia Hypoglycemic Agents Immunohistochemistry Male Pancreas Pancreatic Neoplasm Pancreatic Polypeptide-Secreting Cells Pancreaticoduodenectomy Time Factors
1
(4.0%)		 28515932
 Single-stage laparoscopic adrenalectomy for pheochromocytoma and enucleation of a pancreatic neuroendocrine tumor in Von Hippel-Lindau disease: A case report.
Casaccia M, Macina S, Fornaro R.
Mol Clin Oncol. 2017;6(5):799-801.
Pheochromocytoma
VHL
1
(4.0%)		 18521664
 Sporadic pancreatic polypeptide secreting tumors (PPomas) of the pancreas.
Kuo SC, Gananadha S, Scarlett CJ, Gill A, Smith RC.
World J Surg. 2008;32(8):1815-22.
Abdominal pain
Adult Females Homo sapiens Immunohistochemistry Male Middle Aged Pancreatic Neoplasm Pancreatic Polypeptide X-Ray Computed Tomography
1
(4.0%)		 12794252
 Symptomatic pancreatic polypeptide-secreting tumor of the distal pancreas (PPoma).
Mortenson M, Bold RJ.
Int J Gastrointest Cancer. 2002;32(2-3):153-6.
Pancreatitis
Cholelithiasis Diarrhea Differential Diagnosis Females Homo sapiens Hormones Immunohistochemistry Middle Aged Multiple Endocrine Neoplasia Type 1 Pancreatic Neoplasm Pancreatic Polypeptide Pancreatic Pseudocyst Pancreatitis
1
(4.0%)		 9324611
 [Pancreatic polypeptide secreting endocrine pancreas tumor associated with multiple stomach and duodenal ulcers].
Mehring UM, Jager HJ, Kloppel G, Hasse FM.
Langenbecks Arch Chir. 1997;382(3):134-7.
Neoplasm
GAST
Biomarkers, Tumor Differential Diagnosis Females Homo sapiens Middle Aged Neuroendocrine Tumors Pancreas Pancreatic Neoplasm Pancreatic Polypeptide
        

Phenotype(s) retrieved from Orphanet

    Total: 32

HPO ID Term Frequency
HP:0002894 Neoplasm of the pancreas Very frequent (99-80%)
HP:0100833 Neoplasm of the small intestine Very frequent (99-80%)
HP:0001438 Abnormality of abdomen morphology Frequent (79-30%)
HP:0001824 Weight loss Frequent (79-30%)
HP:0002014 Diarrhea Frequent (79-30%)
HP:0002017 Nausea and vomiting Frequent (79-30%)
HP:0002019 Constipation Frequent (79-30%)
HP:0002039 Anorexia Frequent (79-30%)
HP:0002240 Hepatomegaly Frequent (79-30%)
HP:0002574 Episodic abdominal pain Frequent (79-30%)
HP:0004396 Poor appetite Frequent (79-30%)
HP:0030144 Hypoactive bowel sounds Frequent (79-30%)
HP:0001046 Intermittent jaundice Occasional (29-5%)
HP:0001081 Cholelithiasis Occasional (29-5%)
HP:0001406 Intrahepatic cholestasis Occasional (29-5%)
HP:0001541 Ascites Occasional (29-5%)
HP:0002239 Gastrointestinal hemorrhage Occasional (29-5%)
HP:0005214 Intestinal obstruction Occasional (29-5%)
HP:0006723 Intestinal carcinoid Occasional (29-5%)
HP:0012334 Extrahepatic cholestasis Occasional (29-5%)
HP:0030145 Lack of bowel sounds Occasional (29-5%)
HP:0000820 Abnormality of the thyroid gland Very rare (4-1%)
HP:0000837 Increased circulating gonadotropin level Very rare (4-1%)
HP:0000845 Growth hormone excess Very rare (4-1%)
HP:0000870 Prolactin excess Very rare (4-1%)
HP:0001031 Subcutaneous lipoma Very rare (4-1%)
HP:0001578 None Very rare (4-1%)
HP:0002893 Pituitary adenoma Very rare (4-1%)
HP:0002897 Parathyroid adenoma Very rare (4-1%)
HP:0003072 Hypercalcemia Very rare (4-1%)
HP:0008200 Primary hyperparathyroidism Very rare (4-1%)
HP:0008256 Adrenocortical adenoma Very rare (4-1%)


Phenotype(s) retrieved from case reports

    Total: 4

HPO ID Term # of case reports
HP:0002027 Abdominal pain 1
HP:0002666 Pheochromocytoma 1
HP:0006725 Pancreatic adenocarcinoma 1
HP:0500167 Hypergastrinemia 1


Causative gene(s) retrieved from Orphanet

    Total: 0

Gene Symbol Gene Name Entrez Gene ID