What Is an Infantile Sinusoidal Tumour of the Liver?An infantile sinusoidal tumour of the liver is a rare and peculiar liver tumour primarily affecting infants and very young children. This tumour is characterized by its unique histological appearance, showing an abundance of blood-filled spaces or sinusoids that are lined by tumour cells. These tumours can range from benign to highly malignant, making accurate diagnosis and timely treatment crucial.
SymptomsSymptoms of an infantile sinusoidal tumour can vary but often include:
- Abdominal distension or swelling
- A palpable mass in the abdomen
- Weight loss or failure to thrive
- Jaundice (yellowing of the skin and eyes)
- Vomiting or poor feeding
- Anemia or other blood-related issues due to the tumour’s high vascularity
Disease Aetiology (Causes)The exact cause of infantile sinusoidal tumours of the liver is unknown. These tumours do not have a clear association with genetic syndromes or environmental factors, unlike some other types of childhood cancers. Research continues to explore potential cellular or molecular origins to better understand these rare tumours.
DiagnosisDiagnosing an infantile sinusoidal tumour involves a combination of imaging, laboratory tests, and biopsy:
- Imaging tests such as ultrasound, computed tomography (CT) scans, and magnetic resonance imaging (MRI) can help identify the presence and extent of the tumour.
- Blood tests, including liver function tests and markers that may indicate liver damage or dysfunction.
- A biopsy of the liver tumour is crucial for a definitive diagnosis, allowing for microscopic examination of the tumour cells and the unique sinusoidal structures.
TreatmentTreatment strategies for infantile sinusoidal tumours depend on the tumour’s size, location, and whether it is considered benign or malignant:
- Surgery is often the primary treatment option, aiming to remove the tumour completely.
- Chemotherapy may be used, especially if the tumour is malignant or if complete surgical resection is not possible.
- Liver transplantation might be considered in cases where the tumour is unresectable and liver function is significantly compromised.
- Supportive care to manage symptoms and improve quality of life, including nutritional support and management of any complications.