Pancreatoblastoma in a paediatric patient: anatomo-pathological aspects of a case with multiple hepatic metastases

CAO, GABRIEL FERNANDO

Ecancermedicalscience

Bristol, UK : ecancer

Lugar: Bristol; Año: 2018 p. 861 - 866

1754-6605

Pancreatoblastoma is a rare paediatric malignant neoplasm. The treatment of choice is complete surgical resection. However, it is oftenunresectable due to its large size, local infiltration or distant metastasis. Since the condition is rare, there is currently no standard treatmentregimen. We outline the case of a 4-year-old child who presented with abdominal pain and distention, together with an enlarged liver andelevated serum α-fetoprotein levels. Imaging studies showed the presence of an abnormal pancreatic tumour and multiple nodular lesionsin 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 pancreaticlesion was resected, obtaining a circumscribed and nodular specimen measuring 7 × 6 cm and weighing 150 g. Given the extent of themetastasis, the child is currently awaiting a liver transplant.