Introduction:
Pancreatic tumours are rare and pose a significant challenge to the paediatric surgeons and pathologist.2 In order to ensure good outcome complete surgical resection is necessary.6,7 The child might also have to be subjected to neoadjuvant chemotherapy in order to ensure complete surgical removal.8 The chemo sensitivity and radio sensitivity is a debatable issue but lack of any standardized protocol makes it necessary to individualize the management.9We describe here a child diagnosed as pancreatoblastoma, who had unresectable disease but responded well to chemotherapy and complete resection was possible. On postoperative histopathology it turned out to be acinar cell carcinoma which is a rare tumour especially in this age group.
Case summary :
A four year old child presented with history of fall during playing one day back followed by pain in abdomen epigastric region. He had non bilious vomiting. Mother gave history of progressive distention of abdomen since last two months. Child also had transient jaundice 2 months back. There was no history of fever. On examination there was a large lump palpable in epigastric and umbilical region.
Ultrasound revealed large heterogeneous mass in head of pancreas with suspicious breach of capsule and hemoperitoneum. There was infiltration into the root of mesentry. Tumour caused widening of ā€˜C’ loop of duodenum with displacement of 2nd and 3rd part of duodenum. Diagnosis of pancreatic tumour possibly pancreatoblastoma was considered. CT scan confirmed the findings. The tumour was large heterogenous and arising from head, neck and uncinate process of pancreas with size of 8.4 x 9.7 x 9.9 cm. The mass was reaching up to anterior abdominal wall and posteriorly compressing Inferior vena cava. It was encasing portal vein at its origin. There was breach in capsule. CT chest showed mild bilateral pleural effusion. Serum alpha fetoprotein was raised (12170.87 ng/ml).CT guided biopsy revealed tumour arranged in sheets and nests, expressing Creatine Kinase. The cytopathological features were consistent with pancreatoblastoma.
After initial management of pain and stabilization patient received chemotherapy, Cisplatin and Doxorubicin (PLADO). After 4 cycles of PLADO the tumour size decreased to 6.5 x 6.4 x 2.6 cm and previously seen encased portal vein was now free from tumour. The child was taken up for surgery. The tumour was situated in head of pancreas. (Fig 1). The Superior mesenteric vein and portal vein were free. (Fig 2). The tumour could be excised with a margin of compressed pancreatic tissue around it. (Fig 3) There was a small breach in capsule during removal with resultant spill of tumour locally. Postoperatively patient recovered well. Patient was tolerating full orally by 5thpostoperative day. On 14th postoperative day patient developed pain in abdomen. Serum Lipase was raised (2190.5 U/lit) and ultrasound revealed heterogeneously echogenic cystic lesion in head of pancreas 3.7 x 4.1 cm size with perilesional edema. The child responded well to conservative management and serum Lipase decreased along with cystic lesion. Postoperatively the serum Alpha-fetoprotein decreased to 11 ng/ml. The child is now one month postop and is scheduled to receive abdominal radiation.
The histopathological report showed malignant epithelial tumour showing acinar differentiation. Margins were tumour free with compressed benign pancreatic tissue seen around the tumour. The pathology report had a comment that the earlier diagnosis of pancreatoblastoma was made considering the tumour cell characteristics and the age of the child. However absence of squamous rest in the multiple sections studied rule out the possibility of pancreatoblastoma. Possibility of neuroendocrine cell tumour was also ruled out by absence of synaptophysin and CD 56 expression and insignificant chromogranin expression.