Table 1.
Patient details, neoplasm localization, pathology, treatment, and follow-up of reported functional gallbladder neuroendocrine neoplasms
| Case | Ref. | Age in yr, sex | Presentation | Physical examination | Primary site | Pathology | Treatment | Follow-up |
| 1 | Monier et al[12] | 60, M | Epigastric pain, jaundice, flushing, cough, wheezing | NA | fundus of GB | Size: NA; PI: ~ 60%; IHC: Chromogranin A (+), synaptophysin (+), CD56 (+), CK7 (+), polyclonal carcinoembr-yonic antigen (+), Caudal-type homeobox transcription factor 2 (+), S-100 (−), CK20 (−), CD10 (−), CK19 (−), prostate-specific antigen (−),thyroid transcription factor-1 (−), HepParl (−) | NA | NA |
| 2 | Lin et al[13] | 65, F | 3 mo progressive generalized weakness and anorexia | Proximal muscle weakness, hirsutism, moon face, buffalo hump | Body of GB | Size: NA; PI: NA; IHC: Chromogranin (+), synaptophysin (+), adrenocortico-tropic hormone -stain (+) | Cholecystect-omy and wedge-shaped liver resection | Hepatic metastasis 2 mo after surgery |
| 3 | Howlett et al[14] and Coates et al[15] | 68, F | NA | Cushing’s syndrome | GB | Size: NA; PI: NA; IHC: Neuron specific enolase immunostain-ing (+) | NA | Survived for 1 mo following presentation |
| 4 | Ahn et al[16] | 52, F | Episodic hypoglycemia of 10 mo duration | NA | GB | Size: 10.3 cm × 7 cm × 5.3 cm; PI: NA; IHC: CD56 (+), chromogranin (+), synaptophysin (+), immunochem-ical staining for insulin (+) | Hepatic segmentectomy, cholecystecto-my, pancreaticosp-lenectomy | NA |
| 5 | Salimi et al[17] | 79, M | Watery diarrhea, abdominal pain, and weakness for several weeks | NA | GB | Size: 0.5 cm in thickness and 1.5 cm in diameter; PI: NA; IHC: Neuron specific enolase immunostain-ing (+), chromogranin (+) | NA | The patient died on his 3rd day of hospitalization from multiple organ failure |
| 6 | Present case | 65, M | A history of flushing for 2 mo | The skin over face, neck, upper chest and limbs flushed and was scaly | neck of GB | Size: 4.0 cm × 2.0 cm; PI: Ki-67 (80%+++); IHC: Chromogranin A (+), synaptophysin (+), pan-CK (+), CK19 (+), CD56 (+), thyroid transcription factor-1 (a few +), CK7 (−), glypican-3 (−) | En bloc cholecystect-omy, resection of hepatic segment IVb and V, Whipple and regional lymphadenec-tomy were performed | The skin became flushed in 3rd month after surgery and the patient passed away in 7th month |
M: Male; F: Female; NA: Not available; GB: Gallbladder; PI: Proliferation index; IHC: Immunohistochemistry; CD: Cluster of differentiation; CK: Cytokeratin.