Pancreatic Pathology Online

Pathology of Pancreatoblastoma of the Pancreas

Dr Sampurna Roy MD             

 

Syn: Pancreaticoblastoma ; Infantile Pancreatic Carcinoma.

Pancreaticoblastoma is a rare malignant tumour occurring in infants and children.

Paediatric Pancreatic Tumours.

Pancreatoblastoma, comprising 0.5% of epithelial tumours of the pancreas usually affects children at 1-8 years of age, with quite equal  sex ratio.

A few cases have been reported in adults.

Morphology appears analogous to nephroblastoma and hepatoblastoma.

Gross features:

The tumours arise in the head or body of the pancreas as a large, soft, round, encapsulated mass,measuring 5-20 cm in diameter.

Cut surface shows conspicuous hemorrhage, necrosis and cystic changes.

Microscopic features:

The tumour consists of mainly epithelial elements but rarely a mesenchymal component is also present.

The epithelial element is characterized by monomorphic polygonal cells, with PAS-positive material in either the cytoplasm or glandular lumen, growing in a solid, trabecular or acinar pattern.

Mitoses are frequent present. Consistently present are squamoid corpuscles - circumscribed, whorled nests of plump spindle cells with a squamous appearance and occasional keratinization.

The stroma is usually moderate to abundant and frequently quite cellular (especially in the pediatric cases).

Mesenchymal elements, if present, may consist of spindle-shaped cells with stromal hyalinization, and myxoid or cartilaginous changes.

Immunohistochemistry: The cells also show positive immunostaining with lipase, trypsin, chymotrypsin and alpha1-antitrypsin.

Scattered neuroendocrine or alpha-fetoprotein positive cells can be found.

Electron microscopic features: The tumour shows acinar features with zymogen granules in many epithelial cells.

Prognosis: Prognosis of this rare well encapsulated tumour is good in paediatric patients, when resected completely.

Prognosis is poorer, when there is metastasis (usually in adult patients) or when the tumour is inoperable.

 

Further reading:

Adrenocorticotropin-secreting pancreatoblastoma.

Diagnosis and treatment of pancreatoblastoma in China.

A case of pancreatoblastoma prenatally diagnosed as intraperitoneal cyst.

Pancreatoblastoma in an adult: case report and review of the literature.

Pancreatoblastoma: a rare tumour accidentally found.

Clinical and biologic analysis of pancreatoblastoma.

Fine-needle aspiration cytology of pancreato blastoma in a young woman: report of a case and review of the literature.

Pancreatoblastoma associated with incomplete Beckwith-Wiedemann syndrome: case report and review of the literature.

Expression pattern of keratin subclasses in pancreatoblastoma with special emphasis on squamoid corpuscles.

Pediatric pancreatoblastoma: histopathologic and cytogenetic characterization of tumor and derived cell line.

Diagnosis, treatment and outcome of pancreatoblastoma.

Ampullary pancreato blastoma in an elderly patient: a case report and review of the literature.

 
 
 
 
 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)


 

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