Pancreatoblastoma

Definition

Definition of Pancreatoblastoma
Pancreatoblastoma is an extremely rare pancreatic tumor in childhood. Children with pancreatoblastoma usually present late with upper abdominal pain and many have a palpable mass in the epigastrium. Mechanical obstruction of the upper duodenum and gastric outlet by tumor in the head of the pancreas may be associated with vomiting, jaundice and gastrointestinal haemorrhaging.

Symptoms

Symptoms of Pancreatoblastoma
Children with pancreatoblastoma rarely present with early-stage disease, instead, most present with locally advanced or metastatic disease. Common presenting symptoms include abdominal pain, emesis, and jaundice. A multidisciplinary approach including good clinical history, state of the art imaging, and careful pathology is often needed to establish the correct diagnosis.

Pathology

Pathology of Pancreatoblastoma
Resected pancreatoblastomas can be quite large, ranging from 2 centimeters to 20 centimeters in size (1 to 8 inches). They are typically solid, soft masses. Under the microscope, at least two cell types are seen- 1) cells with “acinar” differentiation, and cells forming small “squamoid” nests. The cells with acinar differentiation have some features of the normal acinar cell of the pancreas (the most common cell in the normal pancreas).

Diagnosis

Diagnosis of Pancreatoblastoma
Preoperative imaging with US, CT and/or MRI will usually suggest a correct diagnosis of pancreatoblastoma.

Prognosis

Prognosis of Pancreatoblastoma
Overall survival is at least 80% in children with completely resectable tumors at diagnosis. By contrast the outlook for children with metastatic disease, usually hepatic or skeletal, is very poor.

Treatment

Treatment of Pancreatoblastoma
If the tumor is operable, the first line of therapy should be surgical resection. Then, after surgical resection,adjuvant chemotherapy should be given, even in stage I disease. In patients with inoperable disease, chemotherapy alone should be given. A multi-disciplinary approach to the treatment, including surgeons, oncologists, pathologists, radiologists, and radiation oncologists, is often the best approach to managing these patients.

Prevention

Prevention of Pancreatoblastoma
Consult with your doctor.