Neuroendocrine tumors (NETS) of the Gallbladder or the biliary tree are rare. Most of the current guidelines and protocols are derived from the experience of managing Lung small cell neuroendocrine carcinoma (SCNEC) or gastrointestinal (GI) NETS. But the overall outcome of Gallbladder NETS (GB NETS) seems worse than similarly staged lung NETS and adenocarcinoma of the gallbladder. This may be due to its rarity and lack of literature for a focused approach towards its treatment. Hence the need for a specifically designed approach might help improve the results of treatment for these rare tumours. We share our experience of two patients with GB NETS and their 5-year outcome.
A 14-year-old boy presented with a painful swelling topped by a bruise overlying the skin of the right inguinal region without peritonitis. This was the area of impact of bicycle handlebar while riding 6 days ago. On contrast-enhanced CT scan, we found a traumatic abdominal wall hernia (handlebar hernia) near the right deep ring without any solid organ, bowel or urinary bladder injury. Inguinal exploration revealed a defect in transversus abdominis and internal oblique muscle which was repaired and meshplasty was done.Delayed presentation and ignorance towards ‘handlebar sign’ is associated with visceral injury (haematoma/perforation) will incur the risk of rising morbidity and mortality. With CT scan we can assess the abdominal cavity to rule out associated visceral or vascular injury. Surgical repair for restoring disrupted anatomy with or without meshplasty is the preferred approach.
A 56-year-old woman with a medical history of hypertension presented to our hospital with back pain, abdominal pain, vomiting and elevated blood pressure. The laboratory parameters including evaluation for secondary hypertension were within normal ranges at the time of presentation. During her hospitalisation, fluctuations in her blood pressure and pulse were observed which were attributed to autonomic disturbances, the cause of which was unknown. On the seventh day after presentation to the hospital, the patient developed focal seizures and slurred speech which was believed to be secondary to hyponatraemia detected at that time. Hyponatraemia improved with hypertonic saline and she experienced no further seizures. On the eighth day of her admission, she developed acute flaccid paralysis of all her limbs and respiratory distress. We concluded this to be secondary to Guillain-Barre syndrome (GBS). She responded to plasmapheresis.The presence of dysautonomia and hyponatraemia before the onset of paralysis makes this a rare presentation of GBS.
An 18-year-old man presented with acute onset right-sided abdominal pain associated with an episode of unconsciousness for a period of 30-40 min. The laboratory values were notable for elevated total bilirubin (3.8 mg/dL; normal range 0.2 to 0.8 mg/dL) and serum ammonia (77 mmol/L; normal range 21-50 mmol/L). Transabdominal ultrasound showed coarse heterogeneous liver. Contrast-enhanced computedn tomography revealed nodular liver and anomalous drainage of the portal vein (PV) directly into the right atrium (RA) (a). The IVC had attenuated contrast flow, suggesting major blood shunting through the PV (b). His 2D echo and pulmonary artery pressure were normal. He was treated with 30 mL (20 g) of lactulose PO 2 times a day and 550 mg of rifaximin tablet twice a day. He experienced recurrent episodes of hepatic encephalopathy. His family refused a shunt closure procedure after being explained the potential complications. We present an atypical presentation of Abernethy malformation where the portal blood is directly shunted into systemic (here, the RA) circulation (c). Available classifications describe portal venous blood draining into systemic circulation through a shunt or directly into the IVC, renal or iliac veins; but there is no subtype describing this pattern of anomaly. This case presents a unique presentation of Abernethy malformation that would be difficult to treat using conventional surgical techniques. (Informed consent was obtained from the patient to publish these images.
A 36-year-old woman was diagnosed with compensated cirrhosis of liver secondary to Budd-Chiari syndrome (BCS) and had undergone stenting of a thrombosed left hepatic vein. Eight months later, she presented with jaundice and right upper quadrant pain. CT revealed multiple focal lesions in the liver, which on biopsy proved to be hepatic hemangioendothelioma (HHE). Her liver disease and ascites progressively increased. Four months later, magnetic resonance cholangiopancreatography showed an advanced stage of HHE with infiltration of the common bile duct and vascular invasion with a blocked stent, with metastasis to the spleen and dorsolumbar vertebrae. We believe this is the first reported case of an HHE developing in the background of BCS.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.