Table 1 Literature review and analysis of pathological and clinical data

Tsui et al. [1]/1993

Female

74

RUQ pain

Liver function test normal

Wedge resection

Positive: cam5.2, AE1/3, EMA, CEA

Negative: Chromo, S100, desmin, AFP, NSE

No recurrence after 20 years

No

Parada et al. [3]/1997

Female

49

RUQ pain

Liver function test normal

Partial hepatectomy

Monosomy 22

No recurrence

No

Akin et al. [6]/2002

Male

25

Abdominal enlargement, RUQ pain

RUQ palpable mass

Right lobectomy

–

Recurrence and pulmonary metastasis after 3 years

Yes

Garduño-López et al. [19]/2002

Female

68

RUQ pain, vomiting, diarrhea and jaundice

Liver enlargement

Elevated CA19-9; hepatitis B surface Ag, ALP, total biliary, GGT, AFP normal

Left hepatectomy

Stained CA19-9

No recurrent after 30 months

No

Varnholt et al. [20]/2003

Female

47

RUQ pain, weight gain of 5 kg

–

Incomplete resection

Epithelial: positive for D10, p53 (50% to 75%), AE1/3, cam 5.2, CK7, CK19, CEA, EMA; negative for 1F6; Ki67 < 10%

Stroma: positive for vimentin and SMA; negative for desmin

No metastasis or significant growth after 3 years

No

Gurrera et al. [31]/2010

Male

79

Vague abdominal pain

Blood tests and AFP normal

Partial resection

Epithelial: positive for CK7,CK8,CK9,CK19,EMA; negative for CEA,CK5/6,P53,calretinin, HBME-1, beta-catenin

Stroma: positive for vimentin and SMA; negative for desmin

No recurrence after 7 years

No

Kai et al. [7]/2012

Male

40

Upper abdominal pain

Hematological, coagulation test,CEA, Ca19-9, AFP normal;

carrier for HBV

Right hepatectomy

Positive: CK19, Ca19-9, MUC1

Negative: CEA, MUC2, MUC 5AC, p53, ER, PR, GCDFP15

Ki67 5% to 10%

Dying of fulminant hepatitis B 8 months after surgery

Yes

Nguyen et al. [8]/2012

Female

53

Incidentally found

Elevated CA-125; liver function tests, clotting profile, AFP, CA 19–9, and CEA normal

Segmental resection

Positive: CK7,CK19

No recurrent after 12 months

Yes

Tsutsui et al. [9]/2014

Female

69

Asymptomatic

General examination normal

Complete blood count, chemistry, urinalysis, tumor markers, and coagulation normal

Partial liver resection

Epithelial: positive for CK7, CK19, CAM5.2, CKAE1/AE3, p53; negative for CEA, a-SMA;

Ki67:10–15% in the dysplastic epithelia, 1–2% in non-dysplastic epithelia. Stroma: positive for vimentin and SMA; negative for desmin

No recurrent after 4 years

Yes

Jacobs et al .[10]/2015

Female

57

Incidentally found

Modest left costovertebral angle tenderness

Mild leukocytosis, liver enzymes, CEA, AFP normal

Preoperative embolization and surgical resection

–

No recurrent after 5 years

Yes

Elpek et al. [32]/2016

Male

23

Asymptomatic

Physical examination normal

Tumor markers, hematologic and coagulation normal

Partial hepatectomy

Positive: CK7, CK19, CK 18 and EMA

Negative: AFP, PLAP, HCG, Hepatocyte, CK20, CD30, OCT4 and MUC2

–

No

Godambe et al. [11]/2016

Female

71

Bilateral upper abdominal pain

Liver function testing, serum alpha fetoprotein, CEA, and CA199 normal

Left hepatectomy

Positive: CK7 and CK19; brisk Ki67

P53(25% to 50%)

Stroma: negative for Ki67 and p53

Negative: CD10, polyclonal CEA, Inhibin and PAX8

–

Yes

Thai et al. [12]/2016

Male

77

Fever, lumbosacral pain disorientation, and nocturnal agitation

Hyponatriemia, increase in inflammatory markers and anemia

Left lobectomy

Positive: CK7, CK19, CA19.9, CEA and MUC1

–

Yes

Thompson et al. [13]/2016

Male

71

Incidentally found

AFP, CA 19–9, Liver function tests, serology for HBV and HCV normal

Left hepatectomy

–

Dying for primary lung malignancy after 9 years

Yes

Thompson et al. [13]/2016

Male

71

Incidentally found

AFP, CA 19–9, Liver function tests, serology for HBV and HCV normal

Caudate lobe resection

Positive: CK7

Negative: CDX-2, CK20

CDKN2A mutation

No recurrent after 4 weeks

Yes

Kaminsky et al. [14]/2016

Female

37

Postprandial nausea, vomiting, and epigastric pain

–

Excising with wide local margins

Positive: CK7, CK19, synaptophysin, CD56

Negative: chromogranin, CK20, CDX2, heppar1, and p53

Ki67: 10% to 15% in BAF and 50% in CC

No recurrence after 4 months

Yes

Arnason et al. [4]/2017

4 females and 2 males

46 to 83

Abdominal pain (4 patients); incidental findings(2 patients)

–

Surgical resection(5 patients)

Positive: CKAE1/3, CK7, CK19,CA19-9

Ki67: less than 10% in the epithelial component, < 1% in the stromal component

Amplifications of CCND1 and ERBB2

No recurrence in 3 patients after 3, 20, and 21 years

Local hepatic recurrences in 2 patients after 1 and 6 years

No(series of 6 including 2 cases above)

Chua et al. [15]/2018

Female

66

Asymptomatic

AFP normal

Segmentectomy and adjuvant chemotherapy

Positive: CK7

Negative: CK20 and CDX2 Ki67: 2% BAF; CC 30%;

P53 positive in BAF and CC

No recurrence after 6 weeks

Yes

Esteban et al. [33]/2018

Female

26

Jaundice and pruritus

Scleral icterus and generalized jaundice

Elevated serum total bilirubin and alkaline phosphatase; AST, ALT, hepatitis serologies normal

Left hepatectomy

–

No recurrence after 3 months

No(co-existent BAF and hepatobiliary MCN)

Meguro et al.[5]/2018

Male

63

Found by MRI examination for liver cirrhosis

–

–

Epithelial: positive for CK7, CK19; negative for P53

Stroma: positive for vimentin, CD44, CD56,CD73,CD271;negative for P53, desmin, SMA

Oseoblasts: positive for BMP-2

Dying for liver failure after 21 days

Yes(adenosarcoma)

Lee et al.[17]/2019

Male

63

Asymptomatic

Liver function tests,protein induced by vitamin K, AFP, antagonist-II, CA 19–9, and CEA normal

Bisegmentectomy

Epithelial: positive for CK7, CK19;P53(focally positive);Ki67(< 2%)

Stroma: positive for SMA

No recurrence after 41 months

No

Lee et al.[17]/2019

Male

38

Asymptomatic

physical examination and tumor markers normal

Left lateral section

–

No recurrence after 39 months

No

Sturm et al. [16]/2019

Female

63

unspecific abdominal complaints

The physical examination and AFP, CEA, and CA 19–9 normal

Left hemihepatectomy

Epithelial: positive for CK7, Cadherin 17, CD56,Muc1

Stroma: positive for SMA

Negative: inhibin, calretinin, S100P, ERG, Muc2, Muc4, Muc5, and Muc6

MLH1, MSH2, MSH6, and PMS2: nuclear expression

Ki67: 5–10% in biliary adenofibroma; 20–30% in the adenocarcinoma

Different polymorphisms in the encoded TP53 and KIT

No recurrence after 24 months

Yes

Present case

Female

64

RUQ pain

Complete blood count, chemistry, coagulation, liver function test, tumor markers(AFP、CA125、CA19-9、CA15-3 and CA72-4)normal

Hepatitis B surface antigen, e antibody, core antibody positive

partial resection

Positive: CK7、CK19、CEA

KI67: less than 10% in benign part; 20–30% in malignant part

No recurrence after 9 months

Yes