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Table 1 Pure Large Cell Hepatobiliary Neuroendocrine Carcinoma

From: Biliary tract large cell neuroendocrine carcinoma: current evidence

CaseRef.Age (yr)SexSitePresentationMetastasisChemoSurgeryOutcomea
1[6]65MGBSymptomatic CholelithiasisLiver (4mn after CCY)Chemo NSCCY
18 mo
2[16]55MGBAbdominal Pain + JaundiceMultiple LNChemo NS
(1 cycle)
Celiac LN Bx; unresectDied
1 mo
3[16]67FGBAbdominal PainLiverChemo NS
(3 cycles)
Liver/GB Bx, unresectDied
10 mo
4[15]58FGB18mo hx Suggestive of GallstoneRegional LNCis + ETP
(5 cycles, adjuvant)
CCY → 2 mo later radical GB Bed Clearance, Partial LR, CBD excision, and LND up to coeliac nodesAlive
(16 mo post-CCY)
5[17]64FGBAbdominal PainLiver / BrainHepatic Arterial Chemo Infusion: Cis/ETP + CAV + Pre-op 3D RT
Post-op Chemo with Cis/ETP
Triseg; Cerebell +γ-knife
69mo after 1st diagnosis (3 yr without recurrence since the last γ-knife)
Abdominal Pain + JaundiceMultiple LN; Liver; Lung; Vertebra, PeritoneumCis + ETP (1 cycle)UnresectDied
<  1 mo
7[9]56MGBExophthalmosLiver; Ileum; Kidneys; Adrenal Glands; Diaphragm; Epicardium; Left Orbit; Abdominal LNChemo-radiotherapy NSUnresectDied
36 mo
8[18]64MGBAbdominal FullnessLiver; Multiple LN; BoneCis + TXT→
Axillary LN + GB Bx
22 mo
9[8]76MCBDJaundiceMultiple LN; GBNoneRHepLob; Bile Duct Resection, CCYDied
21 days
10[13]75FCBDNausea JaundiceLN; → recurrence at HJ site; Liver; Portocaval Area5-FU+ EPR + CIS (adjuvant)CBD excision with LN dissectionDied
12 mo
11[11]76FGBAbdominal Pain with CholelithiasisRegional LN; LiverCis + ETP → CBDCA + ETP; SST (no carcinoid sxs)CCYDied
5 mo
12[19]72MCBDJaundiceLiverCis + ETPRHepLob+ extra-hepatic BD and PV resection after Percu/ Trans-hepatic PV EmboAlive 7mo post-surgery
  1. aDuration from diagnosis
  2. Ref. Reference number
  3. M male, F female
  4. GB gallbladder, CBD common bile duct
  5. hx history, CCY Cholecystectomy, LN lymph node, LR Liver resection
  6. NS not specified, HJ hepaticojejunostomy
  7. Cis Cisplatin, ETP Etoposide, CAV Cyclophosphamide/Adriamycin/Vincristine, RT radiation therapy, TXT Docetaxel, CBDCA Carboplatin, 5-FU 5-fluorouracil, EPR epirubicin, SST Somatostatin analog
  8. Unresect unresectable, LND lymphadenectomy, RHepTriseg right hepatic trisegmentectomy, cerebell +γ-knife cerebellectomy with γ-knife irradiation, RHepLob Right hepatic lobectomy, BD Bile Duct, PV Portal Vein, Percu/Trans-hepatic Percutaneous Transhepatic, Embo embolization
  9. mo month, Bx biopsy, sxs symptoms