Blastomas of the digestive system in adults: A review.

Autor:	Liu Y; Department of Pathology, LSU Health Sciences Center School of Medicine, New Orleans, LA 70112, United States., El Jabbour T; Department of Pathology, Hartford HealthCare, Hartford, CT 06102, United States., Somma J; Department of Pathology, LSU Health Sciences Center School of Medicine, New Orleans, LA 70112, United States., Nakanishi Y; Department of Pathology, Moffitt Cancer Center, Tampa, FL 33612, United States., Ligato S; Department of Pathology, Hartford HealthCare, Hartford, CT 06102, United States., Lee H; Department of Pathology and Laboratory Medicine, Albany Medical Center, Albany, NY 12208, United States., Fu ZY; Department of Pathology, LSU Health Sciences Center School of Medicine, New Orleans, LA 70112, United States. zfu@lsuhsc.edu.
Jazyk:	angličtina
Zdroj:	World journal of gastrointestinal surgery [World J Gastrointest Surg] 2024 Apr 27; Vol. 16 (4), pp. 1030-1042.
DOI:	10.4240/wjgs.v16.i4.1030
Abstrakt:	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. Competing Interests: Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article. (©The Author(s) 2024. Published by Baishideng Publishing Group Inc. All rights reserved.)
Databáze:	MEDLINE
Externí odkaz:	Zobrazit plný text záznamu