Successful treatment of metastatic pancreatoblastoma in an adult with autologous hematopoietic cell transplant.
Autologous hematopoietic cell transplantation
Pancreas Neoplasm
Pancreatoblastoma
Journal
Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.]
ISSN: 1424-3911
Titre abrégé: Pancreatology
Pays: Switzerland
ID NLM: 100966936
Informations de publication
Date de publication:
Jan 2021
Jan 2021
Historique:
received:
30
08
2020
revised:
18
10
2020
accepted:
27
10
2020
pubmed:
18
11
2020
medline:
21
10
2021
entrez:
17
11
2020
Statut:
ppublish
Résumé
Pancreatoblastoma is a rare pediatric malignant neoplasm characterized by its histological resemblance to fetal pancreatic tissue and poor clinical outcomes. Preoperative diagnosis of the neoplasm is difficult due to its rarity, variable clinical presentation, and its lack of distinct laboratory markers. Current mainstay of treatment is surgical resection of the tumor, although a standard of care has not yet been established. Data were collected on one patient admitted to the University of Virginia Hospital System. Radiology, hematopoietic cell transplant, and biopsy data were collected according to the best clinical practice. Herein, we describe the case of an adult patient with pancreatoblastoma treated with high-dose chemotherapy and autologous peripheral blood hematopoietic cell transplantation. 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. 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. Our patient is currently living 57 months after diagnosis despite the average survival rate being less than 18 months.
Sections du résumé
BACKGROUND
BACKGROUND
Pancreatoblastoma is a rare pediatric malignant neoplasm characterized by its histological resemblance to fetal pancreatic tissue and poor clinical outcomes. Preoperative diagnosis of the neoplasm is difficult due to its rarity, variable clinical presentation, and its lack of distinct laboratory markers. Current mainstay of treatment is surgical resection of the tumor, although a standard of care has not yet been established.
METHODS
METHODS
Data were collected on one patient admitted to the University of Virginia Hospital System. Radiology, hematopoietic cell transplant, and biopsy data were collected according to the best clinical practice.
RESULTS
RESULTS
Herein, we describe the case of an adult patient with pancreatoblastoma treated with high-dose chemotherapy and autologous peripheral blood hematopoietic cell transplantation. 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.
CONCLUSIONS
CONCLUSIONS
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. Our patient is currently living 57 months after diagnosis despite the average survival rate being less than 18 months.
Identifiants
pubmed: 33199137
pii: S1424-3903(20)30799-7
doi: 10.1016/j.pan.2020.10.049
pii:
doi:
Substances chimiques
Antineoplastic Agents
0
Types de publication
Case Reports
Journal Article
Langues
eng
Sous-ensembles de citation
IM
Pagination
188-191Informations de copyright
Copyright © 2020 IAP and EPC. Published by Elsevier B.V. All rights reserved.