Pancreatoblastoma

A rare malignant epithelial neoplasm that originates in the pancreas and is characterized by acinar differentiation, the formation of squamoid corpuscles, and the formation of stromal bands. Most cases occur in childhood.

Networked: 83 relevant articles (7 outcomes, 5 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Acinar Cell Carcinoma
2.	Headache (Headaches)
3.	Neoplasms (Cancer)
4.	Rare Diseases (Rare Disease)
5.	Adenocarcinoma

Experts

1.	Tanaka, Yukichi: 4 articles (01/2018 - 02/2003)
2.	Ghavamzadeh, Ardeshir: 3 articles (02/2013 - 01/2009)
3.	Ijiri, Rieko: 3 articles (06/2005 - 02/2003)
4.	Kato, Keisuke: 3 articles (06/2005 - 02/2003)
5.	Kigasawa, Hisato: 3 articles (06/2005 - 02/2003)
6.	Nishimata, Shigeo: 3 articles (06/2005 - 02/2003)
7.	Fujimura, Junya: 2 articles (01/2018 - 04/2010)
8.	Hama, Asahito: 2 articles (01/2018 - 05/2012)
9.	Taguchi, Tomoaki: 2 articles (01/2018 - 10/2010)
10.	Bhagat, Monica: 2 articles (04/2016 - 10/2015)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Pancreatoblastoma:

1.	Keratins (Keratin)IBA 06/01/2005 - "Expression pattern of keratin subclasses in pancreatoblastoma with special emphasis on squamoid corpuscles." 01/01/1994 - "Findings which distinguished the pancreatoblastoma and separated it from acinar cell carcinoma were the negativity of the solid components ("squamoid corpuscles") for neuroendocrine markers and their very weak keratin positivity. " 10/01/1987 - "The pancreatoblastoma demonstrated positive reactions to alpha-fetoprotein (AFP) (67%: 2/3), alpha-1-antitrypsin (AAT) (100%: 3/3), carcinoembryonic antigen (CEA) (67%: 2/3) and keratin (33%: 1/3), although CEA was only weakly positive in both cases. " 10/01/1992 - "An acinar cell carcinoma and a pancreatoblastoma had positive results for AAT, lysozyme, cytokeratin, and AFP but negative results for vimentin. "
2.	CateninsIBA 05/01/2012 - "Congenital pancreatoblastoma associated with β-catenin mutation." 01/01/2021 - "Pancreatoblastoma is a rare malignant epithelial neoplasm of the pancreas that mainly occurs in children and involves abnormalities in the WNT/β-catenin pathway, such as CTNNB1 mutation. " 01/01/2021 - "Case Report: Two Cases of Metastatic Pancreatoblastoma in Adults: Efficacy of Folfirinox and Implication of the Wnt/β-Catenin Pathway in Genomic Analysis."
3.	VimentinIBA 10/01/1992 - "An acinar cell carcinoma and a pancreatoblastoma had positive results for AAT, lysozyme, cytokeratin, and AFP but negative results for vimentin. "
4.	Muramidase (Lysozyme)IBA 10/01/1992 - "An acinar cell carcinoma and a pancreatoblastoma had positive results for AAT, lysozyme, cytokeratin, and AFP but negative results for vimentin. "
5.	Etoposide (VP 16)FDA LinkGeneric 10/01/2020 - "A 28-year-old woman with a history of pancreatoblastoma that had been in remission for 51 months after treatment with cisplatin, doxorubicin (Adriamycin), and etoposide had presented to the emergency room with chronic recurrent headaches. "
6.	Doxorubicin (Adriamycin)FDA LinkGeneric 10/01/2020 - "A 28-year-old woman with a history of pancreatoblastoma that had been in remission for 51 months after treatment with cisplatin, doxorubicin (Adriamycin), and etoposide had presented to the emergency room with chronic recurrent headaches. "
7.	Cisplatin (Platino)FDA LinkGeneric 10/01/2020 - "A 28-year-old woman with a history of pancreatoblastoma that had been in remission for 51 months after treatment with cisplatin, doxorubicin (Adriamycin), and etoposide had presented to the emergency room with chronic recurrent headaches. "
8.	folfirinoxIBA 01/01/2021 - "Case Report: Two Cases of Metastatic Pancreatoblastoma in Adults: Efficacy of Folfirinox and Implication of the Wnt/β-Catenin Pathway in Genomic Analysis."
9.	Fluorodeoxyglucose F18 (Fludeoxyglucose F 18)FDA Link 12/29/2022 - "CT, MRI, and 18F-FDG PET/CT imaging features of seven cases of adult pancreatoblastoma." 01/01/2020 - "The objective of this study was to illustrate the clinical, CT, MRI, and 18F-FDG PET/CT features of adult pancreatoblastoma, an extremely rare disease. " 01/01/2020 - "Adult Pancreatoblastoma With Liver Metastasis Diagnosed by 18F-FDG PET/CT and 2 Years' Postoperative Follow-up." 08/01/2021 - "This case showed the metabolic and biological features of pancreatoblastoma on the 18F-FDG and 68Ga-DOTATATE PET/MR." 08/01/2021 - "Detection of Adult Pancreatoblastoma by 18F-FDG and 68Ga-DOTATATE PET/MR."
10.	beta CateninIBA 02/01/2003 - "This study concerns the significance of aberrant (nuclear/cytoplasmic) expression of beta-catenin in pancreatoblastoma (PBL). " 01/01/2023 - "Up-regulation of ALK is associated with altered Wnt/beta-catenin pathway in adult pancreatoblastoma." 02/01/2003 - "Significance of aberrant (cytoplasmic/nuclear) expression of beta-catenin in pancreatoblastoma." 05/01/2009 - "This latter includes pancreatic endocrine tumor (PET) as SPT may show neuroendocrine differentiation, and pancreatic acinar cell carcinoma (ACC) and pancreatoblastoma (PB) that may often show nuclear beta-catenin staining. "

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 01/01/2017 - "As no standard chemotherapy exists for pancreatoblastoma (PB), surgical resection is the most effective way of achieving complete remission. " 05/01/2010 - "Three of the four patients with pancreatoblastoma had advanced disease at diagnosis and were given chemotherapy; at the time of this report, three patients were alive in first remission, while one died due to treatment toxicity. " 02/01/2020 - "The size of the pancreatoblastoma was gradually reduced along systemic chemotherapy, thus we decided to perform surgery to remove it completely. " 02/13/2018 - "We recently treated an adult patient with pancreatoblastoma by chemotherapy. " 01/01/2017 - "Complete Resection of Pancreatoblastoma With Portal Vein Obstruction After High-dose Chemotherapy: A Case Report."
2.	Stem Cell Transplantation 01/01/2013 - "Autologous stem cell transplantation as first line treatment after incomplete excision of pancreatoblastoma." 09/01/2010 - "Autologous stem cell transplantation as treatment modality in a patient with relapsed pancreatoblastoma." 01/01/2009 - "Stem cell transplantation was performed for various diseases including acute myelogenous leukemia, acute lymphoblastic leukemia, chronic myelogenous leukemia, chronic lymphoblastic leukemia, thalassemia major, sickle cell thalassemia, sickle cell disease, multiple myeloma, myelodysplasia, mucopolysaccharidosis, paroxysmal nocturnal hemoglobinuria, non-Hodgkin's lymphoma, Hodgkin's disease, severe aplastic anemia, plasma cell leukemia, Niemann-Pick disease, Fanconi anemia, severe combine immunodeficiency, congenital neutropenia, leukocyte adhesion deficiencies, Chediak-Higashi syndrome, osteopetrosis, histiocytosis X, Hurler syndrome, amyloidosis, systemic sclerosis, breast cancer, Ewing's sarcoma, testicular cancer, germ cell tumors, neuroblastoma, medulloblastoma, renal cell carcinoma, nasopharyngeal carcinoma, ovarian cancer, Wilms' tumor, rhabdomyosarcoma, pancreatoblastoma, and multiple sclerosis. " 02/01/2013 - "Stem cell transplantation was performed for treatment of various diseases such as acute myelogenous leukemia, acute lymphoblastic leukemia, chronic myelogenous leukemia, chronic lymphoblastic leukemia, beta-thalassemia major, sickle- cell thalassemia, sickle- cell disease, multiple myeloma, myelodysplasia, mucopolysaccharidosis, paroxysmal nocturnal hemoglobinuria, non-Hodgkin's lymphoma, Hodgkin's disease, severe aplastic anemia, plasma cell leukemia, Niemann-Pick disease, Fanconi anemia, severe combined immunodeficiency, congenital neutropenia, leukocyte adhesion deficiencies, Chediak-Higashi syndrome, osteopetrosis, histiocytosis X, Hurler syndrome, amyloidosis, systemic sclerosis, breast cancer, Ewing's sarcoma, testicular cancer, germ cell tumors, neuroblastoma, medulloblastoma, renal cell carcinoma, nasopharyngeal carcinoma, ovarian cancer, Wilms' tumor, rhabdomyosarcoma, pancreatoblastoma, and multiple sclerosis. "
3.	Radiotherapy 10/15/1987 - "This case suggests a role for radiation therapy in the management of children with pancreatoblastoma." 10/15/1987 - "The authors report a case of recurrent pancreatoblastoma in a 3-year-old girl who was successfully treated with radiotherapy. " 01/01/2013 - "This is the first case reported of pancreatoblastoma that was treated with autologous hematopoietic stem cell transplantation as first line treatment without radiotherapy at the site of the microscopic disease." 10/15/1987 - "Radiotherapy for locally recurrent infantile pancreatic carcinoma (pancreatoblastoma)." 07/01/2001 - "Preoperatively the diagnosis of a pancreatoblastoma was suspected and an explorative laparotomy was performed followed by radical tumor extirpation and intraoperative radiotherapy. "
4.	Hematopoietic Stem Cell Transplantation 01/01/2013 - "This is the first case reported of pancreatoblastoma that was treated with autologous hematopoietic stem cell transplantation as first line treatment without radiotherapy at the site of the microscopic disease."
5.	Cell Transplantation 01/01/2021 - "While it is difficult to draw conclusions based on a single case, we would like to highlight the success of this treatment modality in the management of our patient with a 51-month remission and open further discussion into exploring the use of autologous hematopoietic cell transplantation for pancreatoblastoma. " 01/01/2021 - "To the authors' knowledge, this is the first documented successful treatment of pancreatoblastoma using autologous hematopoietic cell transplantation in the United States, and the first successful treatment in an adult patient worldwide. " 01/01/2021 - "Herein, we describe the case of an adult patient with pancreatoblastoma treated with high-dose chemotherapy and autologous peripheral blood hematopoietic cell transplantation. "