What is Pancreatoblastoma?
Pancreatoblastoma is a rare type of pancreatic cancer primarily affecting children. It can originate from various pancreatic cell types, including acinar, ductal, and neuroendocrine tissues. This cancer often spreads rapidly, making surgical removal of the tumour the most critical treatment option.
Causes
The exact cause of pancreatoblastoma remains unknown, but some cases are associated with hereditary conditions, including:
- Beckwith-Wiedemann Syndrome: A disorder characterised by tissue overgrowth.
- Familial Adenomatous Polyposis (FAP): A condition that increases the risk of several cancers.
- Genetic changes, such as alterations in chromosome 11p, may also play a role.
Signs and Symptoms
Common signs and symptoms include:
- Abdominal pain or discomfort
- Bloating or a persistent feeling of fullness
- Constant fatigue
- Persistent fever
- Unintentional weight loss
- Loss of appetite
- Cancer spread to the liver, lymph nodes, or lungs
- Physical changes such as:
- Round face and abdominal weight gain
- Stretch marks on the breasts, hips, or abdomen
- A fatty hump between the shoulders
Diagnosis
Diagnosing pancreatoblastoma involves several steps:
1. Imaging Procedures:
CT scans or MRIs are used to locate and assess the tumour.
2. Blood Tests:
- Elevated alpha-fetoprotein (AFP) levels are often seen in children with pancreatoblastoma.
- Increased levels of carcinoembryonic antigen (CEA) may also be present.
3. Tumour Biopsy:
A biopsy involves removing a small portion of the tumour for examination. Diagnosing can be challenging due to the tumour’s varied cell types.
Treatment
Treatment typically includes:
- Surgery: Complete removal of the tumour is the primary approach.
- Chemotherapy: Administered after surgery to reduce the risk of recurrence or metastasis.
- Radiation Therapy: Used in cases of metastasis or when surgical removal is incomplete.
Prognosis and Outcomes
The prognosis largely depends on the tumour’s complete removal.
- About 65% of patients survive five years after successful tumour removal.
- If the tumour cannot be fully removed, survival rates are significantly lower.
Pancreatoblastoma in adults tends to be more advanced, leading to a worse prognosis compared to children. Early diagnosis and complete surgical resection are crucial for improving survival rates and outcomes.
Pancreatoblastoma is a challenging condition requiring prompt diagnosis and a multidisciplinary approach for effective treatment and management.
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