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Table 1 Pure Large Cell Hepatobiliary Neuroendocrine Carcinoma

From: Biliary tract large cell neuroendocrine carcinoma: current evidence

Case

Ref.

Age (yr)

Sex

Site

Presentation

Metastasis

Chemo

Surgery

Outcomea

1

[6]

65

M

GB

Symptomatic Cholelithiasis

Liver (4mn after CCY)

Chemo NS

CCY

LR

Died

18 mo

2

[16]

55

M

GB

Abdominal Pain + Jaundice

Multiple LN

Chemo NS

(1 cycle)

Celiac LN Bx; unresect

Died

1 mo

3

[16]

67

F

GB

Abdominal Pain

Liver

Chemo NS

(3 cycles)

Liver/GB Bx, unresect

Died

10 mo

4

[15]

58

F

GB

18mo hx Suggestive of Gallstone

Regional LN

Cis + ETP

(5 cycles, adjuvant)

CCY → 2 mo later radical GB Bed Clearance, Partial LR, CBD excision, and LND up to coeliac nodes

Alive

(16 mo post-CCY)

5

[17]

64

F

GB

Abdominal Pain

Liver / Brain

Hepatic Arterial Chemo Infusion: Cis/ETP + CAV + Pre-op 3D RT

Post-op Chemo with Cis/ETP

RHep

Triseg; Cerebell +γ-knife

Died

69mo after 1st diagnosis (3 yr without recurrence since the last γ-knife)

6

[10]

67

F

GB

CBD

Abdominal Pain + Jaundice

Multiple LN; Liver; Lung; Vertebra, Peritoneum

Cis + ETP (1 cycle)

Unresect

Died

<  1 mo

7

[9]

56

M

GB

Exophthalmos

Liver; Ileum; Kidneys; Adrenal Glands; Diaphragm; Epicardium; Left Orbit; Abdominal LN

Chemo-radiotherapy NS

Unresect

Died

36 mo

8

[18]

64

M

GB

Abdominal Fullness

Liver; Multiple LN; Bone

Cis + TXT→

CBDCA + TXT

Axillary LN + GB Bx

Unresect

Died

22 mo

9

[8]

76

M

CBD

Jaundice

Multiple LN; GB

None

RHepLob; Bile Duct Resection, CCY

Died

21 days

10

[13]

75

F

CBD

Nausea Jaundice

LN; → recurrence at HJ site; Liver; Portocaval Area

5-FU+ EPR + CIS (adjuvant)

CBD excision with LN dissection

Died

12 mo

11

[11]

76

F

GB

Abdominal Pain with Cholelithiasis

Regional LN; Liver

Cis + ETP → CBDCA + ETP; SST (no carcinoid sxs)

CCY

Died

5 mo

12

[19]

72

M

CBD

Jaundice

Liver

Cis + ETP

RHepLob+ extra-hepatic BD and PV resection after Percu/ Trans-hepatic PV Embo

Alive 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