Pancreatic Acinar Cell Carcinoma in Childhood

Abstract

A 9-year-old Chinese boy with abdominal pain and an abdominal mass was found at laparotomy to have carcinoma of the pancreas. Histologically, this was an acinar cell carcinoma, which is an extremely rare condition, especially in childhood. The patient survived for ten months after pancreatoduodenectomy and choledochojejunostomy. A review of the literature showed that in contrast to adults, jaundice was an uncommon finding in children with pancreatic carcinoma, and that an enlarging abdomen or an abdominal mass was the initial feature in the majority of patients. Prognosis was poor even with treatment, the longest survivor having lived for five years after pancreatoduodenectomy.

Genetic and Immunohistochemical Analysis of Pancreatic Acinar Cell Carcinoma

“…mas, another rare but distinctive pancreatic malignancy. Although pancreatoblastomas occur predominantly in the pediatric population and ACCs predominantly in adults, occasional cases of ACCs in children (2 in this series of 21 ACCs) 1,48,49 and pancreatoblastomas in adults 27,50 -54 are encountered, and some investigators regard pancreatoblastomas as the pediatric counterpart of ACCs. 27 We have recently characterized genetic alterations in pancreatoblastomas and have demonstrated high frequencies of both allelic loss on chromosome 11p and mutations in the APC/␤-catenin pathway, a molecular genotype that is distinct from that of pancreatic ductal adenocarcinoma.…”

Chronic Pancreatitis and Obstructive Jaundice from a Round‐Cell Tumor Involving the Pancreas

“…Nausea, vomiting, anorexia, and weight loss are commonly present, as in this case. Obstructive jaundice occurs in only 25% of pediatric patients versus -50% of adults with pancreatic malignancies (5). Elevation of the serum amylase or lipase , associated with epigastric pain radiating to the back , may suggest an initial diagnosis of chronic pancreatitis, as in this case (l ,4).…”

Acinar cell carcinoma of the pancreas in childhood

“…In addition, Dall'igna et al [6] reviewed 21 patients aged 18 years or younger with pancreatic tumors reported in Italy between 2000 and 2009, including 2 cases of ACC. Table 2 summarizes the 26 reported pediatric cases, including the patients from the above reports and our case [5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21]. Although the details are often unclear, the following characteristics of pediatric ACC can be suggested: (1) the tumors tend to be very large at diagnosis, (2) distant metastasis is rare and in most cases lesions are (9).…”