Blastomas of the digestive system in adults: A review.
Adult blastomas
Digestive system
Gastroblastoma
Hepatoblastoma
Pancreatoblastoma
Journal
World journal of gastrointestinal surgery
ISSN: 1948-9366
Titre abrégé: World J Gastrointest Surg
Pays: United States
ID NLM: 101532473
Informations de publication
Date de publication:
27 Apr 2024
27 Apr 2024
Historique:
received:
01
01
2024
revised:
03
02
2024
accepted:
25
03
2024
medline:
1
5
2024
pubmed:
1
5
2024
entrez:
1
5
2024
Statut:
ppublish
Résumé
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.
Identifiants
pubmed: 38690053
doi: 10.4240/wjgs.v16.i4.1030
pmc: PMC11056657
doi:
Types de publication
Journal Article
Review
Langues
eng
Pagination
1030-1042Informations de copyright
©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.
Déclaration de conflit d'intérêts
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.