Adult hepatoblastoma: A case report and literature review.

INTRODUCTION: Adult hepatoblastoma is a rare malignant liver neoplasm. Surgery is the only cure, but recurrence is common even after complete resection. No therapeutic strategy has been established. PRESENTATION OF CASE: A 22-year-old man presented with a rapidly expanding right hypochondrial mass. Pain preceded the appearance of the mass. No definitive diagnosis was established in the referring hospital. In addition, two attempts of embolization failed to reach the tumor due to its large size and vascular displacement. Clinical examination revealed a 26cm×23cm mass occupying the right hypochondrium and epigastrium as far as the right iliac fossa, compressing the stomach, spleen, kidneys and liver. The preoperative diagnosis was gastrointestinal stromal tumor because it appeared to originate from the stomach. During surgery, we found a mass arising from the liver, adhering to the omentum, stomach, and left hemidiaphragm, and infiltrating the pericardium. The tumor was completely resected off the inferior vena cava and pericardium. The histopathological diagnosis was a 30cm×30cm hepatoblastoma weighing 4kg. The postoperative treatment course went smoothly until day 10, when the patient developed complications like bilateral atrial thrombi and left ventricular hypokinesia and expired on day 16. DISCUSSION: Due to the rarity of hepatoblastoma in adults and non-specific initial symptoms, hepatoblastoma is often overlooked as a diagnosis. Early detection may lead to improved prognosis and survival. CONCLUSION: We report here the first case of adult hepatoblastoma in the Middle East and the largest such tumor ever reported in literature.

Endogenous endothelin increases gallbladder tone and leads to acute cholecystitis in the Australian possum.

Endothelins are bioactive peptides produced by gallbladder epithelial cells. We aimed to determine the role of endothelins in acute cholecystitis. Escherichia coli lipopolysaccharide vs saline (sham) was instilled into the gallbladder lumen of Australian possums. Some animals received the non-selective endothelin antagonist, tezosentan. At 4 or 24 h, plasma and gallbladder endothelins and white blood cell count (WBCC) were determined. Acute cholecystitis was assessed using a histopathology score. In other animals gallbladder tone was determined. At 4h, a dose-dependent 60-fold increase in gallbladder endothelin level occurred (P = 0.001) but other parameters remained comparable with sham animals. Epithelial cells were endothelin-immunoreactive. At 24 h, the WBCC rose (P < 0.007), and severe cholecystitis developed. Gallbladder but not plasma endothelin levels remained elevated. Tezosentan pre-treatment resulted in a histologically normal gallbladder, but the WBCC and gallbladder endothelin levels were elevated. Lipopolysaccharide or saline instillation also caused a time-dependent increase in gallbladder tone over 4 h (P < 0.001), but not in control animals. This increase was reduced by tezosentan treatment. Gallbladder endothelin production is an early event in acute cholecystitis, increases gallbladder tone and plays a crucial role in the inflammatory process.

Secretin induces variable inhibition of motility in different parts of the Australian possum sphincter of Oddi.

The sphincter of Oddi (SO) may not function as a single structure. We aimed to determine the response of the proximal and distal segments of the bile duct (BD-SO) and pancreatic duct (PD-SO) components of the SO to secretin, with and without neural blockade with tetrodotoxin (TTX). In anaesthetized Australian possums, separate manometry catheters were placed in the proximal and distal BD-SO or PD-SO segments to record motility. Secretin, 50-1000 ng kg(-1), was administered, followed by TTX, and re-administration of secretin, 500 and 1000 ng kg(-1). Changes in the motility index (MI, frequency x mean amplitude) were determined. Statistical analysis utilized repeated-measures ANOVA. Secretin produced a dose-dependent decrease in MI from the proximal and distal BD-SO and PD-SO (all P < 0.001). The maximum inhibition, at 1000 ng kg(-1), was 21 +/- 4%, 33 +/- 6% and 42 +/- 5% of control (mean +/- SEM), for proximal and distal BD-SO, and distal PD-SO, respectively. The proximal PD-SO MI, however, was inhibited to 62 +/- 6% of control, at 1000 ng kg(-1). TTX enhanced the secretin-induced response to the same level at the four sites (P < 0.02). We conclude that secretin inhibits the motility of the possum SO in a nonuniform manner and is modulated by neural activity.

Inhibitory motor innervation of the gall bladder musculature by intrinsic neurones containing nitric oxide in the Australian brush-tailed possum (Trichosurus vulpecula).

BACKGROUND: Gall bladder functions are modulated by neurones intrinsic to the organ. Data are available on the neurochemical composition of intrinsic and extrinsic nerves innervating the gall bladder but are lacking on specific functional classes of gall bladder neurones. AIMS: To characterise the intrinsic motor neurones of the gall bladder and identify their roles using pharmacological techniques. METHODS: Retrograde tracing from the possum gall bladder muscle in vitro allowed identification of intrinsic motor neurones. Subsequently, their content of choline acetyltransferase and nitric oxide synthase, markers of acetylcholine and nitric oxide containing neurones, was established using immunohistochemical techniques. Organ bath pharmacology was used to evaluate neurotransmission by acetylcholine and nitric oxide in gall bladder muscle strips. RESULTS: Innervation of the gall bladder musculature by neurones of both the muscular and serosal plexuses was demonstrated. A large proportion (62%) of these motor neurones were immunoreactive for nitric oxide synthase. All gall bladder neurones showed immunoreactivity for choline acetyltransferase. Organ bath pharmacology confirmed the neuroanatomical data, showing acetylcholine and nitric oxide mediating neurotransmission to the gall bladder musculature. CONCLUSIONS: Neurones containing acetylcholine and nitric oxide, located within the muscular and serosal plexuses, provide excitatory and inhibitory motor innervation of the gall bladder, respectively. The large inhibitory innervation suggests active relaxation of the gall bladder during filling, mediated by intrinsic nerves.

Endothelin-1 induces contraction of human and Australian possum gallbladder in vitro.

BACKGROUND: Endothelin-1 (ET-1) stimulates guinea pig gallbladder (GB) muscle strip contractility; however, the role and source of ET-1 in the GB remains to be elucidated. AIMS: To determine the effect of ET-1 on human and possum GB muscle strip contractility and evaluate whether ET-1 is present in GB tissue. METHODS: GB muscle strips were mounted in organ baths to measure isometric tension. ET-1 was added cumulatively with and without pretreatment with the neural blocker tetrodotoxin (TTX) or the ET receptor antagonists BQ-123, BQ-788, and tezosentan. Immunohistochemical localization of ET was performed on freshly fixed and cultured GBs. RESULTS: ET-1 induced concentration-dependent increases in tone in human and possum GB strips (p<0.05). This response was unaffected by BQ-123, BQ-788, and TTX but antagonized by BQ-123+BQ-788 in the human tissue only. Tezosentan (10(-4) mol/l) blocked the ET-1-induced response in human and possum GB strips (p<0.001). Although ET immunoreactivity was absent in freshly fixed possum GB, immunoreactivity was observed in the GB epithelium of freshly fixed human tissue and in both possum and human tissue following 24 h of organ culture. CONCLUSION: ET-1 acts directly on human and possum GB smooth muscle producing contractions, possibly via ET-B receptors. ET may be present under pathophysiological conditions altering GB function.