Pancreatoblastoma is a rare tumor of the pancreas

Pancreas

The pancreas is a gland organ in the digestive and endocrine system of vertebrates. It is both an endocrine gland producing several important hormones, including insulin, glucagon, and somatostatin, as well as a digestive organ, secreting pancreatic juice containing digestive enzymes that assist...

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It occurs mainly in childhood and has a relatively good prognosis.

Symptoms

Children with pancreatoblastoma rarely present with early-stage disease, instead, most present with locally advanced or metastatic disease. Common presenting symptoms include abdominal pain

Abdominal pain

Abdominal pain can be one of the symptoms associated with transient disorders or serious disease. Making a definitive diagnosis of the cause of abdominal pain can be difficult, because many diseases can result in this symptom. Abdominal pain is a common problem...

, emesis, and jaundice

Jaundice

Jaundice is a yellowish pigmentation of the skin, the conjunctival membranes over the sclerae , and other mucous membranes caused by hyperbilirubinemia . This hyperbilirubinemia subsequently causes increased levels of bilirubin in the extracellular fluid...

. A multidisciplinary approach

Multidisciplinary approach

A multidisciplinary approach to problem solving involves drawing appropriately from multiple disciplines to redefine problems outside of normal boundaries and reach solutions based on a new understanding of complex situations....

 including good clinical history

Medical history

The medical history or anamnesis of a patient is information gained by a physician by asking specific questions, either of the patient or of other people who know the person and can give suitable information , with the aim of obtaining information useful in formulating a diagnosis and providing...

, state of the art

State of the art

The state of the art is the highest level of development, as of a device, technique, or scientific field, achieved at a particular time. It also refers to the level of development reached at any particular time as a result of the latest methodologies employed.- Origin :The earliest use of the term...

imaging, and careful pathology is often needed to establish the correct diagnosis.

Pathology

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).

Treatment

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.