Hepatoblastoma is a malignant liver cancer occurring in infants and children and composed of tissue resembling fetal liver cells, mature liver cells, or bile duct cells. They usually present with an abdominal mass. The disease is most commonly diagnosed during a childs first three years of life. Alpha-fetoprotein (AFP) levels are commonly elevated, but when AFP is not elevated at diagnosis the prognosis is poor. Signs and symptoms
Patients are usually asymptomatic at diagnosis. As a result, disease is often advanced at diagnosis. Pathophysiology
Hepatoblastomas originate from immature liver precursor cells, are typically unifocal, affect the right lobe of the liver more often than the left lobe, and can metastasize. They are categorized into two types: "Epithelial Type" and "Mixed Epithelial / Mesenchymal Type. "Individuals with familial adenomatous polyposis (FAP), a syndrome of early-onset colonic polyps and adenocarcinoma, frequently develop hepatoblastomas. Also, beta-catenin mutations have been shown to be common in sporadic hepatoblastomas, occurring in as many as 67% of patients.Recently, other components of the Wnt signaling pathway have also demonstrated a likely role in constitutive activation of this pathway in the causation of hepatoblastoma. Accumulating evidence suggests that hepatoblastoma is derived from a pluripotent stem cell.Syndromes with an increased incidence of hepatoblastoma include Beckwith–Wiedemann syndrome, trisomy 18, trisomy 21, Acardi syndrome, Li–Fraumeni syndrome, Goldenhar syndrome, von Gierke disease, and familial adenomatous polyposis. Diagnosis
The most common method of testing for hepatoblastoma is a blood test checking the alpha-fetoprotein level. Alpha-fetoprotein (AFP) is used as a biomarker to help determine the presence of liver cancer in children.
Isolated cases of gallstone formation have been associated with use of lanreotide, particularly over long periods of time. Pharmacology
Lanreotide is a synthetic analogue of somatostatin, a naturally occurring inhibitory hormone which blocks the release of several other hormones, including growth hormone, thyroid-stimulating hormone (TSH), insulin and glucagon. Lanreotide binds to the same receptors as somatostatin, although with higher affinity to peripheral receptors, and has similar activity. However, while somatostatin is quickly broken down in the body (within minutes), lanreotide has a much longer half-life, and produces far more prolonged effects. Formulations
Lanreotide is available in two formulations: a sustained release formulation (sold under the trade name Somatuline LA), which is injected intramuscularly every ten or fourteen days, and an extended release formulation (UK trade name Somatuline Autogel, or Somatuline Depot in the US), which is administered subcutaneously once a month. Self-assembling properties
Lanreotide has been shown to spontaneously self-assemble into monodisperse nanotubes of 24.4 nm diameter and has been thereafter used as a fruitful and versatile model system in several biophysical studies. References
External links
"Lanreotide". Drug Information Portal. U.S. National Library of Medicine.