1
|
Liu Y, El Jabbour T, Somma J, Nakanishi Y, Ligato S, Lee H, Fu ZY. Blastomas of the digestive system in adults: A review. World J Gastrointest Surg 2024; 16:1030-1042. [PMID: 38690053 PMCID: PMC11056657 DOI: 10.4240/wjgs.v16.i4.1030] [Citation(s) in RCA: 1] [Impact Index Per Article: 1.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/01/2024] [Revised: 02/03/2024] [Accepted: 03/25/2024] [Indexed: 04/22/2024] Open
Abstract
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.
Collapse
Affiliation(s)
- Yu Liu
- Department of Pathology, LSU Health Sciences Center School of Medicine, New Orleans, LA 70112, United States
| | - Tony El Jabbour
- Department of Pathology, Hartford HealthCare, Hartford, CT 06102, United States
| | - Jonathan Somma
- Department of Pathology, LSU Health Sciences Center School of Medicine, New Orleans, LA 70112, United States
| | - Yukihiro Nakanishi
- Department of Pathology, Moffitt Cancer Center, Tampa, FL 33612, United States
| | - Saverio Ligato
- Department of Pathology, Hartford HealthCare, Hartford, CT 06102, United States
| | - Hwajeong Lee
- Department of Pathology and Laboratory Medicine, Albany Medical Center, Albany, NY 12208, United States
| | - Zhi-Yan Fu
- Department of Pathology, LSU Health Sciences Center School of Medicine, New Orleans, LA 70112, United States
| |
Collapse
|
2
|
De Stefano FA, Morell A, Marks K, Fernandez S, Smith G, Mayo T, Merenzon M, Shah AH, Eichberg DG, Luther E, Ivan ME, Komotar RJ. Brain Metastasis from Pancreatic Cancer: Our Experience and Systematic Review. World Neurosurg 2022; 166:e590-e598. [PMID: 35863644 DOI: 10.1016/j.wneu.2022.07.060] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/02/2022] [Accepted: 07/13/2022] [Indexed: 12/15/2022]
Abstract
OBJECTIVE To systematically review existing literature on the neurosurgical management and outcomes of brain metastasis from pancreatic cancer in comparison with our institutional experience of this patient cohort. METHODS Following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, a systematic literature review was performed using PubMed, Ovid Embase, Scopus, and Web of Science databases from date of database inception to March 2022. Studies were selected based on predetermined inclusion and exclusion criteria. Simultaneously, a retrospective analysis was conducted of patients who underwent neurosurgical evaluation and treatment for intracranial metastatic lesions from pancreatic origin at a single institution. RESULTS The original literature search yielded 292 articles, of which 17 studies comprising 23 patients with brain metastases of pancreatic origin were ultimately selected. Median overall survival from primary diagnosis of pancreatic cancer was 22 months (interquartile range: 3-84) and 3 months (interquartile range: 1-36) after diagnosis of brain metastasis. In our institutional cohort, 4 patients were identified with a median overall survival of 30.5 months (interquartile range: 2-108). Our institutional cohort experienced a prolonged median overall survival (3 months vs. 30.5 months, P = 0.03) compared with the literature. CONCLUSIONS Brain metastasis from pancreatic cancer is rare and associated with a fatal outcome. However, based on the data presented in this review, patient-specific and treatment-related factors could signal better prognosis. Further studies are needed to elucidate multimodal therapy and survival to suggest a more personalized decision-making process.
Collapse
Affiliation(s)
- Frank A De Stefano
- Department of Neurological Surgery, University of Kansas, Kansas City, Kansas, USA.
| | - Alexis Morell
- Department of Neurological Surgery, University of Miami, Miami, Florida, USA
| | - Katya Marks
- School of Medicine, University of Oxford, Oxford, United Kingdom
| | - Sophia Fernandez
- School of Medicine, Florida International University, Miami, Florida, USA
| | - Grace Smith
- School of Medicine, Morehouse College, Atlanta, Georgia, USA
| | - Timothy Mayo
- Department of General Surgery, Washington University School of Medicine, St. Louis, Missouri, USA
| | - Martin Merenzon
- Department of Neurological Surgery, University of Miami, Miami, Florida, USA
| | - Ashish H Shah
- Department of Neurological Surgery, University of Miami, Miami, Florida, USA
| | - Daniel G Eichberg
- Department of Neurological Surgery, University of Miami, Miami, Florida, USA
| | - Evan Luther
- Department of Neurological Surgery, University of Miami, Miami, Florida, USA
| | - Michael E Ivan
- Department of Neurological Surgery, University of Miami, Miami, Florida, USA
| | - Ricardo J Komotar
- Department of Neurological Surgery, University of Miami, Miami, Florida, USA
| |
Collapse
|
3
|
Omiyale AO. Adult pancreatoblastoma: Current concepts in pathology. World J Gastroenterol 2021; 27:4172-4181. [PMID: 34326617 PMCID: PMC8311526 DOI: 10.3748/wjg.v27.i26.4172] [Citation(s) in RCA: 19] [Impact Index Per Article: 4.8] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/30/2021] [Revised: 03/23/2021] [Accepted: 06/22/2021] [Indexed: 02/06/2023] Open
Abstract
Adult pancreatoblastoma is an exceptionally rare malignant tumour of the pancreas that mimics other solid cellular neoplasms of the pancreas, which may pose diagnostic difficulties. Because of its rarity, little is known about its clinical and pathologic features. This article reviews the clinical and pathologic features of pancreatoblastoma in adults including differential diagnosis, treatment, and follow-up. Although pancreatoblastoma commonly occurs in childhood, there have now been more than 70 adult pancreatoblastomas described in the literature. There is a slight male predominance. There are no symptoms unique to pancreatoblastomas and adult patients are frequently symptomatic. The most common presenting symptom is abdominal pain. Grossly, the tumours are often large and well-circumscribed. Microscopically, pancreatoblastomas are composed of neoplastic cells with predominantly acinar differentiation and characteristic squamoid nests. These tumours are positive for trypsin, chymotrypsin, lipase, and BCL10. Loss of heterozygosity on chromosome 11p is the most common molecular alteration in pancreatoblastomas. Adult pancreatoblastomas are aggressive tumours with frequent local invasion, recurrence, and distant metastasis. Treatment consists of surgical resection. Chemotherapy and radiotherapy may have a role in the treatment of recurrent, residual, unresectable, and metastatic disease. It is important to distinguish pancreatoblastomas from morphological mimics such as acinar cell carcinomas, solid pseudopapillary neoplasms, and pancreatic neuroendocrine neoplasms.
Collapse
Affiliation(s)
- Ayo O Omiyale
- Department of Cellular Pathology, Imperial College Healthcare NHS Trust, London W6 8RF, United Kingdom
| |
Collapse
|