Pancreatoblastoma

A rare malignant epithelial pancreatic neoplasm, most often found in children, which usually presents with the non-specific symptoms of a palpable mass, vomiting abdominal pain, jaundice, and weight loss/failure to thrive, and is characterized histologically by multiple lines of differentiation (acinar, ductal, mesenchymal, neuroendocrine) and the presence of squamoid nests.



Input patient's signs and symptoms


Narrow down the case reports



Total: 75 (papers)

  


(per page)
Matched Phenotype  Gene  Mutation  MeSH
Rank
(Similarity)
PMID
(PMCID)
1
(48.9%)
29285688
Pancreatoblastoma: an unusual diagnosis in an adult patient.
Nunes G, Coelho H, Patita M, Barosa R, Pinto Marques P, Roque Ramos L, Brito MJ, Tomaz A, Fonseca J.
Clin J Gastroenterol. 2018;11(2):161-166.
Weight loss Anemia Hepatomegaly
Adult Echo Endoscopy Fatal Outcome Homo sapiens Magnetic Resonance Imaging Male Pancreatic Neoplasm
2
(43.7%)
20632017
Successful treatment of advanced pancreatoblastoma by a pylorus-preserving pancreatoduodenectomy after radiation and high-dose chemotherapy.
Souzaki R, Tajiri T, Kinoshita Y, Tanaka S, Koga Y, Suminoe A, Hara T, Kohashi K, Oda Y, Taguchi T.
Pediatr Surg Int. 2010;26(10):1045-8.
Anemia Fever Vomiting
AFP
Antineoplastic Agents Child Females Follow-Up Studies Homo sapiens Pancreatic Neoplasm Pancreaticoduodenectomy Pylorus Radiotherapy, Adjuvant
3
(42.5%)
25851961
Successful emergency combined therapy with partial splenic arterial embolization and endoscopic injection therapy against a bleeding duodenal varix in a child.
Sunakawa H, Tokuhara D, Yamamoto A, Sugimori S, Morotomi Y, Taniguchi S, Nakaya M, Cho Y, Matsui K, Kasuga S, Sakae Y, Yamato K, Nishida N, Tokimasa S, Shintaku H.
Clin J Gastroenterol. 2015;8(3):138-42.
Portal hypertension Anemia
Child Combined Modality Therapy Duodenum Embolization, Therapeutic Gastrointestinal Hemorrhage Homo sapiens Male Pancreatic Neoplasm Portal Hypertension Postoperative Complications Sclerotherapy
4
(36.0%)
1328584
A 20-year review of pediatric pancreatic tumors.
Jaksic T, Yaman M, Thorner P, Wesson DK, Filler RM, Shandling B.
J Pediatr Surg. 1992;27(10):1315-7.
Jaundice Hypoglycemia Abdominal mass
Carcinoma Carcinoma, Papillary Child Child, Preschool Follow-Up Studies Homo sapiens Infant Infant, Newborn Islet Cell Adenoma Pancreas Pancreatectomy Pancreatic Cyst Pancreatic Neoplasm Postoperative Complications X-Ray Computed Tomography
5
(32.6%)
23143293
Multicystic adenomatoid pancreatic hamartoma in a child: Case report and literature review.
Sueyoshi R, Okazaki T, Lane GJ, Arakawa A, Yao T, Yamataka A.
Int J Surg Case Rep. 2013;4(1):98-100.
Leukocytosis Abdominal mass
6
(32.2%)
7954063
Pancreatoblastoma: a case report.
Tzou JS, Wu TC, Yang CM, Wang JS, Wei CF, Hwang B.
Zhonghua Yi Xue Za Zhi (Taipei). 1994;54(3):198-203.
Jaundice Hepatosplenomegaly
Females Homo sapiens Pancreatic Neoplasm
7
(30.0%)
2845376
Congenital pancreatoblastoma in Beckwith-Wiedemann syndrome: an emerging association.
Drut R, Jones MC.
Pediatr Pathol. 1988;8(3):331-9.
Hemihypertrophy Umbilical hernia Hypoglycemia Visceromegaly
Beckwith-Wiedemann Syndrome Homo sapiens Infant, Newborn Male Neoplasms, Germ Cell and Embryonal Pancreatic Neoplasm
8
(29.8%)
18090935
Long-term survival of pancreatoblastoma in children.
Lee YJ, Hah JO.
J Pediatr Hematol Oncol. 2007;29(12):845-7.
Jaundice Abdominal mass
AFP
Antineoplastic Combined Chemotherapy Protocols Child Child, Preschool Combined Modality Therapy Females Homo sapiens Neoplasm Invasiveness Pancreatic Neoplasm Survivors
8
(29.8%)
15632705
Clinical and biologic analysis of pancreatoblastoma.
Sheng L, Weixia Z, Longhai Y, Jinming Y.
Pancreas. 2005;30(1):87-90.
Jaundice Abdominal mass
Electron Microscopy Flow Cytometry G2 Phase Homo sapiens Male Pancreas Pancreatic Neoplasm S Phase
8
(29.8%)
9752756
Clinical course and management of pancreatoblastoma in children.
Kataria R, Bhatnagar V, Agarwala S, Sharma MC, Gupta AK, Mitra DK.
Trop Gastroenterol. 1998;19(2):67-9.
Jaundice Abdominal mass
Antineoplastic Combined Chemotherapy Protocols Child Child, Preschool Combined Modality Therapy Females Homo sapiens Pancreas Pancreatic Neoplasm
        

Phenotype(s) retrieved from Orphanet

    Total: 9

HPO ID Term Frequency
HP:0001824 Weight loss Frequent (79-30%)
HP:0002013 Vomiting Frequent (79-30%)
HP:0002014 Diarrhea Frequent (79-30%)
HP:0002027 Abdominal pain Frequent (79-30%)
HP:0003270 Abdominal distention Frequent (79-30%)
HP:0005213 Pancreatic calcification Frequent (79-30%)
HP:0005984 Elevated maternal serum alpha-fetoprotein Frequent (79-30%)
HP:0000952 Jaundice Occasional (29-5%)
HP:0002733 Abnormality of the lymph nodes Occasional (29-5%)


Phenotype(s) retrieved from case reports

    Total: 19

HPO ID Term # of case reports
HP:0002664 Neoplasm 13
HP:0030731 Carcinoma 13
HP:0002835 Aspiration 6
HP:0031500 Abdominal mass 4
HP:0000952 Jaundice 3
HP:0030405 Pancreatic endocrine tumor 3
HP:0001824 Weight loss 2
HP:0000822 Hypertension 1
HP:0001409 Portal hypertension 1
HP:0001508 Failure to thrive 1
HP:0001903 Anemia 1
HP:0002014 Diarrhea 1
HP:0002027 Abdominal pain 1
HP:0002028 Chronic diarrhea 1
HP:0002249 Melena 1
HP:0002894 Neoplasm of the pancreas 1
HP:0006278 Ectopic pancreatic tissue 1
HP:0031274 Hypovolemic shock 1
HP:0032165 Placental mesenchymal dysplasia 1


Causative gene(s) retrieved from Orphanet

    Total: 0

Gene Symbol Gene Name Entrez Gene ID