Gallbladder Neuroendocrine Tumors: Is There a Need for a Specific Approach?

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 or gastrointestinal 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 toward its treatment. Hence, the need for a specifically designed approach might help improve results of treatment for these rare tumors. We share our experience of 2 patients with GB-NETs and their 5-year outcome.

Ileal cavernous haemangioma in an adult presenting as a rare cause of small bowel obstruction.

Haemangiomas of the small bowel are a very rare entity and rarely considered as an aetiology for an intestinal obstruction. Contrast-enhanced CT is the investigation of choice but the lesion can be confused with malignancy or rarely a neuroendocrine tumour. Commonly it presents as abdominal pain with anaemia and/or melaena. With patients presenting without obstruction or acute gastrointestinal bleed, capsule endoscopy has shown to be a useful diagnostic tool.We present here our experience of managing a case of a patient with ileal haemangioma who presented with a subacute small bowel obstruction and underwent a laparoscopic-assisted ileal segmental resection with side-to-side anastomosis. The lesion was a cavernous haemangioma on histopathological analysis.

'Little old lady's hernia' (obturator hernia): A deceptive encounter.

Obturator hernia (OH) is rare which not only carries high mortality amongst all abdominal hernia, but also known for the difficulty in diagnosing it. Howship-Romberg sign is a clinical sign to diagnose OH, but due to the lower-limb muscle contractures, it was not possible in our case. Computed tomography scan becomes the investigation of choice in this situation. A laparoscopic approach can be used safely.

Successful treatment of extensive splanchnic vein thrombosis in a patient with mycosis fungoides.

A 33-year-old man of a Middle Eastern origin presented to us with abdominal pain and distension secondary to refractory ascites of 1-month duration. The patient had a history of taking oral retinoic acid 25 mg for 4 months for mycosis fungoides. Investigations revealed thrombosis of hepatic veins with extensive thrombosis of the porto-mesenteric axis. A combination of transjugular intrahepatic portosystemic shunt, balloon angioplasty and thrombolysis with recombinant tissue plasminogen activator was successfully used to treat his condition.

Left-sided sinistroposition of the gall bladder: incidental presentation during elective cholecystectomy.

A 59-year-old male patient presented with mild gallstone pancreatitis. He underwent laparoscopic cholecystectomy during the same admission, where we encountered a left-sided gall bladder (GB). This was managed during laparoscopic surgery by modifying the laparoscopic port positions, and we did not encounter any other variations in the biliary anatomy. Thorough knowledge regarding anatomical variations of the GB will help in managing rare cases and avoid injuries to vital structures.

Primary hepatic hemangioendothelioma in a patient with Budd-Chiari syndrome.

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.

Castleman's disease in carcinoma gall bladder.

Castleman's disease (CD) also known as angiofollicular lymph node hyperplasia represents a group of uncommon non-clonal lymphoproliferation. We herein report a case of CD associated with carcinoma of the gall bladder. To the best of our knowledge, it is the first of its kind and has not been reported in the past. The hypothesis regarding the aetiopathogenesis of CD is that it is associated with interleukin-6 surges. This may be explained by the overproduction of IL-6 by tumour cells or IL-6 production due to a long-standing/smouldering localised inflammatory response. An important question raised by this observation is whether the finding of CD has any effect on the outcome of this patient of carcinoma of the gall bladder. Further research is required in this matter.