Imaging Pearls ❯ Pancreas ❯ Pancreatoblastoma
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- Pancreatoblastoma is a rare type of malignant pancreatic tumour more commonly found in childhood in comparison to adults [1,2]. They form 25% of pancreatic neoplasm cases that are diagnosed in the first decade of life [3]. This makes them one of the most common malignant pancreatic tumours in children, with the mean age of diagnosis being four years [3,4]. With pancreatoblastomas making up less than 1% of pancreatic neoplasms, adult cases are exceedingly rare; to date only 74 cases have been reported in literature [4]. Its presentation is often varied and non-specific, with the most common presenting complaint being abdominal pain [5]. There are no symptoms specific to pancreatoblastoma and other signs include weight loss, abdominal masses, diarrhoea and less commonly jaundice [3,6,7]. Imaging can also present with similar findings to those seen in malignant and benign neoplasms [8]. Tumour markers such as alphafetoprotein (AFP), carcinoembryonic antigen (CEA) and Ca 19-9 and are often normal in adults, and whilst elevated AFP and Ca 19-9 can occur, it is rare [4,6,8]. The often non-specific presentation of the disease inconjunction with the fact that tumour markers are not likely to contribute to a diagnosis can make pancreatoblastoma a difficult diagnosis to achieve without biopsy and subsequent histology.
Adult Pancreatoblastoma: An Uncommon Pancreatic Malignancy.
Hussain A, Farrukh J
(October 31, 2023) Cureus 15(10): e48063. DOI 10.7759/cureus.48063 - “Pancreatoblastomas are slow-growing tumours that originate from the exocrine cells of the pancreas. They frequently present in a non-specific manner and given their slow growth by the time patients often present, the tumours can grow to a significant size. As a result, symptoms such as abdominal pain or distension can often be the primary presenting complaint. Whilst less common in adults they have predominantly been shown to have poorer outcomes in comparison to paediatric cases . Cavallini et al. highlight an average prognosis of 18 months in adults.”
Adult Pancreatoblastoma: An Uncommon Pancreatic Malignancy.
Hussain A, Farrukh J
(October 31, 2023) Cureus 15(10): e48063. DOI 10.7759/cureus.48063 - “While pancreatoblastoma is a rare malignancy especially in adults, given its non-specific presentation both on clinical and imaging examination it can be very difficult to diagnose without a successful biopsy. Therefore, it must be considered as a differential diagnosis when suspecting atypical pancreatic malignancies, in order to achieve as early detection and diagnosis as possible. Early detection is critical, as often patients will present once the tumour has grown to a substantial size and achieved a significant degree of local infiltration. This can pose a challenging scenario for surgeons, as was shown in this case with the malignancy being deemed inoperable.”
Adult Pancreatoblastoma: An Uncommon Pancreatic Malignancy.
Hussain A, Farrukh J
(October 31, 2023) Cureus 15(10): e48063. DOI 10.7759/cureus.48063 - Pancreatoblastoma: Facts
- Usual age is 1-8 years
- Usually a heterogeneous tumor
- Aggressive tumor with adjacent and distant metatstases
- Mean size around 10 cm
- “Pancreatoblastoma is the most common pancreatic tumor in children within the first decade, whereas it is exceptional in adults. Its etiology is unknown, however, it has been reported in association with genetic syndromes such as familial adenomatous polyposis syndrome and Beckwith-Weidemann syndrome. In contrary to the children population, there is no male predominance in adults. Pancreatoblastoma involves the pancreatic head in almost half of the cases.”
Imaging features of rare pancreatic tumors M. Barrala, S.A. Faraound, E.K. Fishman, A. Dohan, C. Pozzesserea, M.-A. Berthelina,P. Bazeries, M. Barat, C. Hoeffel, P. Soyer Diagnostic and Interventional Imaging (2016) (in press) - “On imaging, pancreatoblastoma appears as a large, heterogeneous, exophytic multiloculated and well- circumscribed necrotic lesion with enhancing septa. Of interest, higher-grade tumors may have progressively less-defined margins. Rim-shaped or clustered calcifications, biliary and pancreatic ductal dilatation,
ascites, hepatic and pelvic metastases, adenopathy and vascular invasion may be present.”
Imaging features of rare pancreatic tumors M. Barrala, S.A. Faraound, E.K. Fishman, A. Dohan, C. Pozzesserea, M.-A. Berthelina,P. Bazeries, M. Barat, C. Hoeffel, P. Soyer Diagnostic and Interventional Imaging (2016) (in press)
- Pancreatic Tumors in Children
- Pancreatoblastoma
- Pseudopapillary tumor (SPEN)
- Islet cell tumor (insulinoma)
- Pancreatoblastoma: Facts
- Most common pancreatic tumor in young children
- Mean age is 4.5 years
- Slight male predominance
- More common in Asians
- Pancreatoblastoma: CT Findings
- Large mass (5 cm or greater)
- Smooth and multiloculated
- Calcifications not uncommon
- Liver metastases not uncommon and hypovascular