Abstract
Pancreatoblastoma is a rare paediatric malignant neoplasm. The treatment of choice is complete surgical resection. However, it is often unresectable due to its large size, local infiltration or distant metastasis. Since the condition is rare, there is currently no standard treatment regimen. We outline the case of a 4-year-old child who presented with abdominal pain and distention, together with an enlarged liver and elevated serum α-fetoprotein levels. Imaging studies showed the presence of an abnormal pancreatic tumour and multiple nodular lesions in the liver, the biopsies from which led to a diagnosis of pancreatoblastoma. In this case, the patient received cycles of neoadjuvant chemotherapy, combining cisplatin and doxorubicin. The patient subsequently underwent scheduled surgery in which the primary pancreatic lesion was resected, obtaining a circumscribed and nodular specimen measuring 7 × 6 cm and weighing 150 g. Given the extent of the metastasis, the child is currently awaiting a liver transplant.
Highlights
Pancreatoblastoma is the term given to rare malignant neoplasms [1] that mimic foetal pancreatic development at 7 weeks of gestation [2]
The rarity of the condition and the lack of specificity in its symptoms make pancreatoblastoma a real diagnostic challenge for oncologists and surgeons, especially since standardised guidelines are not available for this purpose [1, 4]. Since it is a biologically aggressive neoplasm, it may be unresectable at the time of diagnosis, instead of requiring neoadjuvant chemotherapy to shrink the tumour
Pancreatoblastoma is a rare malignant neoplasm, which usually presents as a slow-growing abdominal mass and with high serum levels of α-fetoprotein
Summary
Pancreatoblastoma is the term given to rare malignant neoplasms [1] that mimic foetal pancreatic development at 7 weeks of gestation [2]. This was followed by an abdominal and pelvic CT scan with and without contrast dye: this showed a heterogeneous tumour in the body and tail of the pancreas that had defined borders, hypodense areas inside (suggestive of necrosis) and an apparent pseudocapsule. This measured: anteroposterior diameter 75 × transverse diameter 57 × longitude 101 mm, displacing the left kidney and the splenic artery in the head and rear directions, the left ureter in the dorsal direction and the bowel in the caudal direction. The patient is receiving medical follow-up and is in a liver transplant programme
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