Clinical Analysis of Childhood Pancreatoblastoma Arising From the Tail of the Pancreas

Abstract

Pancreatoblastoma is a rare pancreatic tumor. In this study, 3 cases of childhood pancreatoblastoma that arise from the tail of the pancreas were reported. Abdominal pain and vomiting were observed in 1 case considering the huge size of the tumor. The other 2 patients, who were previously well, complained of a mass in the abdomen after a casual physical examination. Elevated serum α-fetoprotein levels were noted in all cases. Imaging findings indicated a well-defined heterogeneous large mass in the left retroperitoneal space. Exploratory laparotomy revealed a large mass, arising from the tail of the pancreas. Surgery alone with complete excision of the masses was performed. Immunohistochemical staining showed that only α-fetoprotein was positive in all cases. All of these 3 cases have a good outcome in the follow-up without adjuvant chemotherapy. These data suggest that the diagnosis of pancreatoblastoma is difficult and should be suspected at palpation of an abdominal mass. α-Fetoprotein may serve as a tumor marker for preoperative diagnosis and postoperative recurrence. Pancreatoblastoma arising from the tail of the pancreas is a curable tumor, and adjuvant chemotherapy may not be necessary if the tumor can be excised completely.

Pancreatic Tumors in Children

“…PBs comprises only 0.5% of pancreatic non-endocrine tumours and share similar histological and morphologic features with hepatoblastomas that secrete α-fetoprotein (AFP) (6,15). Although reported with high prevalence (6), in our study, we recorded only one case of this type of malignancy in a child presenting with a large abdominal mass (No.…”

“…PB is less aggressive in children when compared with adults, therefore surgery is the optimal treatment and complete resection of the tumour provides the best prognosis (6). Moreover, adjuvant chemotherapy is not recommended if the tumour can be completely excised (15). However, we performed a true-cut biopsy in case 2 who had a large mass located in the head of the pancreas and treated him by chemotherapy and radiotherapy.…”

“…Approximately 24 months later from the beginning of the treatment the remaining 1.5 cm residual mass was removed totally with enucleation. AFP levels may serve as a valuable tumour marker for preoperative diagnoses, and it can even indicate therapeutic effects and postoperative recurrences in PB patients (15). AFP was used as a marker in the follow-up period after residual mass excision and found to be normal.…”

A Rare Case of Pancreatoblastoma in a Pediatric Patient

“…Pancreatoblastoma is usually incidentally found because most of the time it is asymptomatic. In a study by Xu et al [9] where they reported three cases of PB, two of the three cases were only identified after finding an abdominal mass in rather a casual abdominal examination.…”

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

“…In this regard, it was suggested pancreatoblastoma originates in the ventral pancreatic bud as a result of abnormal embryological development [ 6 , 7 ], which would explain why it is typically found at the head of the pancreas. However, it can sometimes be found in the tail of the pancreas [ 8 ].…”