Successful treatment of metastatic pancreatoblastoma in an adult with autologous hematopoietic cell transplant.

Abstract

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:

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:

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:

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.

• Authors: Omar Elghawy, John S Wang, Rachel M Whitehair, William Grosh, Tamila L Kindwall-Keller
• Journal: Pancreatology : official journal of the International Association of Pancreatology (IAP) ... [et al.] (Pancreatology) Vol. 21 Issue 1 Pg. 188-191 (Jan 2021) ISSN: 1424-3911 [Electronic] Switzerland
• PMID: 33199137 (Publication Type: Case Reports, Journal Article)
• Copyright: Copyright © 2020 IAP and EPC. Published by Elsevier B.V. All rights reserved.

Chemical References

• Antineoplastic Agents

Topics

• Antineoplastic Agents (therapeutic use)
• Combined Modality Therapy
• Female
• Hematopoietic Stem Cell Transplantation (methods)
• Humans
• Neoplasm Metastasis
• Neoplasm Recurrence, Local
• Pancreatic Neoplasms (drug therapy, pathology, therapy)
• Remission Induction
• Treatment Outcome
• Young Adult