Copyright ©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastrointest Surg. Apr 27, 2024; 16(4): 1030-1042
Published online Apr 27, 2024. doi: 10.4240/wjgs.v16.i4.1030
Blastomas of the digestive system in adults: A review
Yu Liu, Tony El Jabbour, Jonathan Somma, Yukihiro Nakanishi, Saverio Ligato, Hwajeong Lee, Zhi-Yan Fu
Yu Liu, Jonathan Somma, Zhi-Yan Fu, Department of Pathology, LSU Health Sciences Center School of Medicine, New Orleans, LA 70112, United States
Tony El Jabbour, Saverio Ligato, Department of Pathology, Hartford HealthCare, Hartford, CT 06102, United States
Yukihiro Nakanishi, Department of Pathology, Moffitt Cancer Center, Tampa, FL 33612, United States
Hwajeong Lee, Department of Pathology and Laboratory Medicine, Albany Medical Center, Albany, NY 12208, United States
Author contributions: Liu Y and Fu ZY are acknowledged for their substantial contributions to drafting the work, conducting a comprehensive literature review for all discussed sections, revising critically important intellectual content, and giving final approval to the published version; Jabbour T and Lee H are credited for providing guidance on the topic; Jabbour T, Nakanishi Y, and Ligato S are acknowledged for their contributions to pathology imaging; Somma J is credited with aiding in the final polishing of content language; All authors have actively participated in the creation of the manuscript and agree with the final version of the manuscript.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: Https://
Corresponding author: Zhi-Yan Fu, MD, Attending Doctor, Department of Pathology, LSU Health Sciences Center School of Medicine, 1901 Perdido St, New Orleans, LA 70112, United States.
Received: January 1, 2024
Peer-review started: January 1, 2024
First decision: January 25, 2024
Revised: February 3, 2024
Accepted: March 25, 2024
Article in press: March 25, 2024
Published online: April 27, 2024
Processing time: 112 Days and 6.2 Hours

Blastomas, characterized by a mixture of mesenchymal, epithelial, and undifferentiated blastematous components, are rare malignant neoplasms originating from precursor blast cells. This review focuses on digestive system blastomas in adult patients, including gastroblastoma, hepatoblastoma, and pancreatoblastoma. Gastroblastoma is a biphasic, epitheliomesenchymal tumor, with only sixteen cases reported to date. In addition to the characteristic histology, metastasis-associated lung adenocarcinoma transcript 1 - glioma-associated oncogene homolog 1 gene fusion is typical, although recently novel ewing sarcoma breakpoint region 1 - c-terminal binding protein 1 and patched 1 - glioma-associated oncogene homolog 2 fusions have been described. Hepatoblastoma is exceptionally rare in adults and can show a variety of histologic patterns which may cause diagnostic difficulty. Pancreatoblastoma, primarily a pediatric tumor, displays acinar differentiation and squamoid nests with other lines of differentiation also present, especially neuroendocrine. Diagnostic approaches for these blastomas include a combination of imaging modalities, histopathological examination, and molecular profiling. The treatment generally involves surgical resection, which may be supplemented by chemotherapy or radiotherapy in some cases. Prognoses vary with gastroblastoma generally showing favorable outcomes post-surgery whereas hepatoblastoma and pancreatoblastoma often have poorer outcomes, particularly in the setting of metastases. This review highlights the complexity of diagnosing and managing these rare adult blastomas as well as the need for ongoing research to better understand their pathogenesis and improve treatment strategies.

Keywords: Adult blastomas, Gastroblastoma, Hepatoblastoma, Pancreatoblastoma, Digestive system

Core tip: Adult digestive system blastomas, including gastroblastoma, hepatoblastoma, and pancreatoblastoma, are rare neoplasms with less than a hundred cases reported for each type. They have nonspecific clinical presentations and imaging findings. Gastroblastoma diagnosis hinges on biphasic histology and the metastasis-associated lung adenocarcinoma transcript 1 - glioma-associated oncogene homolog 1 gene fusion. Hepatoblastoma recapitulates the different embryological stages of liver development and can be epithelial or mixed epitheliomesenchymal. Pancreatoblastoma demonstrates multidirectional differentiation and contains squamoid nests. Surgical resection is the primary treatment for these tumors with adjuvant chemotherapy or radiotherapy in some cases. Prognoses vary, with gastroblastoma typically having better post-surgical outcomes. In contrast, hepatoblastoma and pancreatoblastoma frequently recur and metastasize.