Abstract
Pancreatoblastoma (PB), a rare malignant epithelial neoplasm, is the most common pancreatic neoplasm of childhood. It is exceptionally rare in the adult population and its occurrence is limited to case reports. Although the neoplastic cells of PB can have a number of different directions of differentiation, PB is defined by the combination of neoplastic cells with acinar differentiation and squamoid morules. We report a case of a female patient in her 70s who presented with elevated creatinine level, concerning a kidney disorder, and was found to have an abdominal mass on CT scan. Fine needle aspiration (FNA) showed cellular smears with numerous 3-dimentional clusters of acinar cells and scattered squamoid morules. A cell block showed sheets of cells, some of which formed acini. Numerous squamoid morules were noted and were highlighted by nuclear labelling with antibodies to B-catenin in the cell block. The FNA diagnosis was rendered as “carcinoma with acinar differentiation, favour pancreatoblastoma.” Subsequent histological findings confirmed the PB diagnosis. Next generation sequencing detected a CTNNB1 mutation. Given the wide usage of FNA in the preoperative diagnosis of pancreatic masses, the cytopathologist needs to be aware of the morphological features of PB and its cytological differential diagnosis, even in an elderly patient. The differential diagnosis includes acinar cell carcinoma, pancreatic neuroendocrine tumour, and solid pseudopapillary neoplasm. In conclusion, the cytological finding of neoplastic cells with acinar differentiation combined with squamous morules and/or mesenchymal elements in the smears and more commonly in the cell blocks appears to be the most specific finding for the diagnosis of PB.
| Original language | English (US) |
|---|---|
| Journal | Cytopathology |
| DOIs | |
| State | Accepted/In press - 2022 |
Keywords
- cytopathology
- pancreas
- pancreatic neuroendocrine tumor
- Pancreatoblastoma
ASJC Scopus subject areas
- Pathology and Forensic Medicine
- Histology
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Saoud, C., Wu, A. A., Fishman, E. K., Hruban, R. H. (Accepted/In press). Pancreatoblastoma in an elderly woman: A case report simulating a pancreatic neuroendocrine tumour with emphasis on cytomorphological features. Cytopathology. https://doi.org/10.1111/cyt.13203
Pancreatoblastoma in an elderly woman : A case report simulating a pancreatic neuroendocrine tumour with emphasis on cytomorphological features. / Saoud, Carla; Wu, Annie A.; Fishman, Elliot K. et al.
In: Cytopathology, 2022.
Research output: Contribution to journal › Article › peer-review
Saoud, C, Wu, AA, Fishman, EK, Hruban, RH 2022, 'Pancreatoblastoma in an elderly woman: A case report simulating a pancreatic neuroendocrine tumour with emphasis on cytomorphological features', Cytopathology. https://doi.org/10.1111/cyt.13203
Saoud C, Wu AA, Fishman EK, Hruban RH, Ali SZ. Pancreatoblastoma in an elderly woman: A case report simulating a pancreatic neuroendocrine tumour with emphasis on cytomorphological features. Cytopathology. 2022. doi: 10.1111/cyt.13203
Saoud, Carla ; Wu, Annie A. ; Fishman, Elliot K. et al. / Pancreatoblastoma in an elderly woman : A case report simulating a pancreatic neuroendocrine tumour with emphasis on cytomorphological features. In: Cytopathology. 2022.
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abstract = "Pancreatoblastoma (PB), a rare malignant epithelial neoplasm, is the most common pancreatic neoplasm of childhood. It is exceptionally rare in the adult population and its occurrence is limited to case reports. Although the neoplastic cells of PB can have a number of different directions of differentiation, PB is defined by the combination of neoplastic cells with acinar differentiation and squamoid morules. We report a case of a female patient in her 70s who presented with elevated creatinine level, concerning a kidney disorder, and was found to have an abdominal mass on CT scan. Fine needle aspiration (FNA) showed cellular smears with numerous 3-dimentional clusters of acinar cells and scattered squamoid morules. A cell block showed sheets of cells, some of which formed acini. Numerous squamoid morules were noted and were highlighted by nuclear labelling with antibodies to B-catenin in the cell block. The FNA diagnosis was rendered as “carcinoma with acinar differentiation, favour pancreatoblastoma.” Subsequent histological findings confirmed the PB diagnosis. Next generation sequencing detected a CTNNB1 mutation. Given the wide usage of FNA in the preoperative diagnosis of pancreatic masses, the cytopathologist needs to be aware of the morphological features of PB and its cytological differential diagnosis, even in an elderly patient. The differential diagnosis includes acinar cell carcinoma, pancreatic neuroendocrine tumour, and solid pseudopapillary neoplasm. In conclusion, the cytological finding of neoplastic cells with acinar differentiation combined with squamous morules and/or mesenchymal elements in the smears and more commonly in the cell blocks appears to be the most specific finding for the diagnosis of PB.",
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AB - Pancreatoblastoma (PB), a rare malignant epithelial neoplasm, is the most common pancreatic neoplasm of childhood. It is exceptionally rare in the adult population and its occurrence is limited to case reports. Although the neoplastic cells of PB can have a number of different directions of differentiation, PB is defined by the combination of neoplastic cells with acinar differentiation and squamoid morules. We report a case of a female patient in her 70s who presented with elevated creatinine level, concerning a kidney disorder, and was found to have an abdominal mass on CT scan. Fine needle aspiration (FNA) showed cellular smears with numerous 3-dimentional clusters of acinar cells and scattered squamoid morules. A cell block showed sheets of cells, some of which formed acini. Numerous squamoid morules were noted and were highlighted by nuclear labelling with antibodies to B-catenin in the cell block. The FNA diagnosis was rendered as “carcinoma with acinar differentiation, favour pancreatoblastoma.” Subsequent histological findings confirmed the PB diagnosis. Next generation sequencing detected a CTNNB1 mutation. Given the wide usage of FNA in the preoperative diagnosis of pancreatic masses, the cytopathologist needs to be aware of the morphological features of PB and its cytological differential diagnosis, even in an elderly patient. The differential diagnosis includes acinar cell carcinoma, pancreatic neuroendocrine tumour, and solid pseudopapillary neoplasm. In conclusion, the cytological finding of neoplastic cells with acinar differentiation combined with squamous morules and/or mesenchymal elements in the smears and more commonly in the cell blocks appears to be the most specific finding for the diagnosis of PB.
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FAQs
How serious is a pancreatic neuroendocrine tumor? ›
These cancers tend to grow slowly and can possibly spread to other parts of the body. Cancers that are grade 3 are called pancreatic neuroendocrine carcinomas (NECs). These cancers tend to grow and spread quickly and can spread to other parts of the body.
How long can you live with neuroendocrine pancreatic cancer? ›The 5-year relative survival rate for a pancreas NET that has not spread to other parts of the body from where it started is 95%. If the tumor has spread to nearby tissue or the regional lymph nodes, the 5-year relative survival rate is 72%.
What is the most common cause of pancreatic neuroendocrine tumor? ›Inherited gene mutations
Multiple Endocrine Neoplasia Type 1 (MEN1) syndrome: Most inherited cases of PNETs are due to changes in the MEN1 gene. This syndrome can cause cancer in the pancreas, parathyroid glands, and pituitary glands. These tumors usually happen at younger ages and tend to be non-functioning.
Pancreatic neuroendocrine tumors (NETs) may be benign (not cancer) or malignant (cancer). When pancreatic NETs are malignant, they are called pancreatic endocrine cancer or islet cell carcinoma. Pancreatic NETs are much less common than pancreatic exocrine tumors and have a better prognosis.