Carcinosarcoma of gallbladder: A world review

Table 1 Summary of 76 reported cases of gallbladder carcinosarcoma from 1970 to 2021

No.	Year	Ref.	Age/sex	Risk factors for GB CA (stones)	Clinical presentation	Liver function tests	Position of CA	Tumor markers (CEA, AFP, CA 19-9)	Size (mm)	Initial diagnosis	Confirmatory diagnosis (mode)	Stage (UICC)	Survival (mo)	IHC positives	Further management
1	2020	Khurram et al[4]	64/F	No stones	RUQ pain, intermittent fever, abdominal distension	AST, GGT elevated	Fundus	Normal	132 × 97 × 110	Hepatic abscess	Cholecystectomy	NA	NIL mentioned	CK	NA
2	2020	Ayoub et al[5]	66/M	NA	RUQ pain	Normal	Body	Normal	150 × 80 × 60	Gallbladder malignancy	Cholecystectomy and lymphadenectomy	IVA	12+	Vimentin, CK	NA
3	2020	Kaneko et al[6]	70/F	NA	Obstructive jaundice	NA	NA	Normal	110 × 70 × 34	Gallbladder malignancy	Cholecystectomy	NA	44+	CK, Ki-67	NA
4	2020	Siddiqui et al[7]	57/M	NA	Abdominal pain, nausea, LOW, LOA	ALP, total bilirubin elevated	Fundus	NA	620	Gallbladder malignancy	ERCP (unsuccessful), PTC with internal-external biliary drainage catheter	NA	NA	Vimentin	NA
5	2020	Mochizuki et al[8]	88/F	Gallstones	Chills, tremors, vomiting	NA	Body	NA	60 × 25	Acute cholecystitis	Cholecystectomy	NA	10 +	Ki-67	NA
6	2019	Varshney et al[9]	50/M	Gallstones	RUQ pain, obstructive jaundice	AST, ALT, bilirubin elevated	Fundus	Normal	65 × 55	Gallbladder malignancy	radical cholecystectomy with standard lymphadenectomy	NA	6+	Vimentin, CK	Adjuvant chemotherapy
7	2019	Aldossary et al[10]	40/M	Gallstones	RUQ pain	Normal	Entire gallbladder	Normal	115 × 92 × 50	Gallbladder malignancy	Open lap, radical cholecystectomy, extended R hemi w IC anastomosis, liver resection	IVB	6	Vimentin	Adjuvant chemotherapy
8	2019	Aldossary et al[10]	52/F	No stones	RUQ pain	ALT, AST elevated	Fundus	CA19-9 level of 154.3 IU/mL, with normal levels of AFP and CEA	136 × 120 × 95	Gallbladder Malignancy	Open lap, radical CCY, transverse chole, Roux en Y + distal gastrectomy	IVB	3	Vimentin, CK	NA
9	2019	Aldossary et al[10]	62/F	Gallstones	RUQ pain, nausea, anorexia	Normal	Body	Normal	27 × 9	Gallbladder malignancy	Lap CCY	II	86+	Vimentin, CK	Adjuvant chemotherapy
10	2019	Alratroot et al[11]	52/F	Xanthogranulomatous cholecystitis	RUQ pain	GGT elevated	Fundus	CA 19-9 154.33 IU/mL	110 × 60	Gallbladder malignancy	Laparotomy with radical cholecystectomy, transverse colectomy, distal gastrectomy, omentectomy and liver bed resection	III	1.5+	Vimentin, CK	Adjuvant chemotherapy
11	2019	Matsubayashi et al[12]	72/F	Pancreaticobiliary maljunction	RUQ pain	ALP, GGT elevated	Entire gallbladder	Normal	90 × 85	Gallbladder malignancy	Laparotomy and extended cholecystectomy	IIIA	73+	Vimentin, CK	NA
12	2018	Doniparthi et al[13]	49/M	NA	Epigastric pain	AST, ALT, lipase elevated	NA	Normal	32	Acute cholecystitis	Lap cholecystectomy, followed up by robotic liver resection and lymphadenectomy	NA	NA	NA	NA
13	2018	Koustav et al[14]	40/F	NA	RUQ pain		NA	CA19-9 elevated	43 × 51	Gallbladder malignancy	Staging laparoscopy + extended cholecystectomy	NA	NA	NA	NA
14	2018	Trautman et al[15]	73/F	Chronic cholecystitis	Abdominal distension, constipation, vomiting, LOW	AST, ALT, ALP elevated	NA	Beta-HCG elevated		Gallbladder malignancy	Diagnostic laparoscopy	NA	0.5	Vimentin	Palliative (NM)
15	2017	Furuya et al[16]	61/F	NA	RUQ pain	Normal	NA	Normal	15 × 15	Chronic cholecystitis with stone	Cholecystectomy	NA	NA	NA	NA
16	2016	Hu et al[17]	68/F	Cholelithiasis	RUQ pain, fever	Normal	Body	CA19-9 elevated	16 × 15 × 13	Gallbladder malignancy	Cholecystectomy	NA	1	NA	NA
17	2016	Cruz et al[18]	52/F	Gallstones	RUQ pain	ALT AST elevated	Entire gallbladder	Normal	170 × 125	Gallbladder malignancy	Cholecystectomy	NA	1	Vimentin, CK	Palliative (NM)
18	2016	Dong et al[19]	61/M	NA	Abdominal distension	NA	NA	Normal	180	Gallbladder malignancy	Resection (not specified)	NA	NIL mentioned	Ki-67	NA
19	2016	Gupta et al[20]	46/F	NA	RUQ pain	NA	Fundus	All normal	350 × 250 × 200	Gallbladder malignancy	Radical cholecystectomy with hepato-duodenal ligament lymph node clearance and segment 4b/5 liver resection	NA	15 (still alive)	Vimentin, CK	Adjuvant chemotherapy
20	2016	Wong et al[21]	52/F	NA	Abdominal pain	NA	Entire gallbladder	CA19-9 elevated	75	NA	Autopsy	III	6	Vimentin, CK	Adjuvant chemotherapy
21	2016	Ansari et al[22]	50/F	NA	RUQ pain	Normal	Entire gallbladder	Normal	50 × 40	NA	Radical cholecystectomy	II	13 mo (still alive)	Vimentin, CK, Ki-67	Adjuvant chemotherapy
22	2015	Gao et al[23]	62/M	Chronic cholecystitis	RUQ pain	Normal	Entire gallbladder	Normal	50 × 40	Gallbladder malignancy	Simple cholecystectomy	II	0	Vimentin, CK	NA
23	2015	Tonouchi et al[24]	87/M	No stones	Abdominal pain	NA	NA	NA	60 × 55	Diffuse peritonitis	Cholecystectomy with partial transverse colectomy around the fistula	NA	Lost to follow-up	Vimentin, CK	NA
24	2015	Faujdar et al[25]	60/F	NA	RUQ pain, fever	Normal	Entire gallbladder		120 ×70 × 60	Gallbladder malignancy	Cholecystectomy	NA	60+	Vimentin, CK	NA
25	2014	Wada et al[26]	68/M	NA	Right flank pain	GGT elevated	NA	Normal	85 × 70	Gallbladder malignancy	Extended right hepatectomy with portal thrombectomy with hepatoduodenal ligament lymphadenectomy	NA	51+	Vimentin, CK, Ki-67	Adjuvant chemotherapy
26	2014	Kishino et al[27]	70s/F	NA	Referred for suspected GB cancer (presenting complaint not mentioned)	NA	Fundus	NA	68	Gallbladder malignancy	Cholecystectomy	NA	1.5+	Vimentin, CK	NA
27	2013	Wang et al[28]	68/F	Chronic cholecystitis, cholecystolithiasis	RUQ pain, jaundice, fever	ALT, ALP elevated	NA	CEA, CA19-9, AFP elevated	100 × 70 × 50	Gallbladder malignancy	Cholecystectomy with liver segmentectomy (S4a+S5) and a lymph node dissection, followed by resection of the extrahepatic bile duct and a Roux-en-Y type hepatic cholangiojejunostomy	NA	6+	Vimentin, CK	NA
28	2013	Khanna[29]	45/F	NA	RUQ pain	Normal	Body		60 × 40	Gallbladder malignancy	Laparotomy and simple cholecystectomy with wedge resection	NA	3	Vimentin, CK, Ki-67	NA
29	2013	Li et al[30]	64/M	Chronic cholecystitis	RUQ pain	NA	NA	CEA, CA19-9 elevated	40 × 30 × 30	NA	Cholecystectomy, R hemicolectomy, resection of multiple hepatic metastases	NA	3+	Vimentin, CK, Ki-67	NA
30	2012	Kim et al[31]	72/F	Gallstones	Abdominal pain	Normal	Fundus	Normal	65 × 45 × 45	Gallbladder malignancy	Radical cholecystectomy with wedge resection of liver combined with hepatoduodenal ligament lymphadenectomy	NA	4	NA	Adjuvant chemotherapy
31	2012	Kim et al[31]	81/M	NA	Epigastric pain	Normal	Fundus	Normal		Gallbladder malignancy	Cholecystectomy with liver segmentectomy (S4a,5) and lymph node dissection	NA	13	Vimentin, CK	NA
32	2012	Sadamori et al[32]	80/M	NA	RUQ pain, fever		Entire gallbladder		76 × 27	Gallbladder malignancy	Cholecystectomy with liver segmentectomy (S4a and S5) and lymph node dissection	NA	2+		Adjuvant chemotherapy
33	2012	Kataria et al[33]	55/F	NA	RUQ pain	Normal	Fundus	Normal	70 × 50 × 30	NA	Cholecystectomy, wedge resection of liver with resection of transverse colon and paraduodenal lymph node	NA	6	Vimentin, CK	NA
34	2012	Parreira et al[34]	59/F	NA	RUQ pain	Normal	NA	NA	NA	NA	Conventional cholecystectomy	NA	2	NA	NA
35	2012	Park et al[35]	77/F	NA	RUQ pain	AST, ALT elevated	NA	CA19-9, CA-125 elevated	78 × 55 × 12	Gallbladder malignancy	Laparotomy, followed by cholecystectomy and lymph node dissection	IIIB	10+	NA	NA
36	2012	Ishida et al[36]	62/F	NA	Incidental finding on radiograph for left calcaneal fracture	Normal	Entire gallbladder	Normal	52 × 38	Gallbladder malignancy	Open cholecystectomy	NA	8	NA	NA
37	2011	Lee et al[37]	77/F	No stones	RUQ pain	Not mentioned	Body	CA19-9, CA-125 elevated	80 × 70 × 30	Gallbladder malignancy	Cholecystectomy	NA	1.5+	Vimentin, CK	NA
38	2011	Pu et al[38]	59/F	Cholecystolithiasis	RUQ pain, fever	Normal	Body	CA19-9 elevated	120 × 25 × 60	Gallbladder malignancy	Exploratory laparotomy, followed by radical LN resection and hepatocholangojejunostomy Roux-En-Y	II	0	CK	Adjuvant chemotherapy
39	2011	Krishnamurthy et al[39]	83/M	No stones	Abdominal pain	NA	NA	NA	NA	NA	Laparoscopic cholecystectomy	NA	48+	Vimentin, CK	NA
40	2009	Kohtani et al[40]	84/M	Chronic cholecystitis	RUQ pain	Serum glutamic oxaloacetic transaminase, GGT elevated	Neck	NA		Gallbladder malignancy	Open cholecystectomy	II	3+	Vimentin, CK, Ki-67	Adjuvant chemotherapy
41	2009	Agarwal et al[41]	60/F	NA	RUQ pain, fever	Normal	Neck	NA	70 × 50 × 40	Gallbladder malignancy	Staging laparoscopy, laparotomy, simplex cholecystectomy	NA	24+	Vimentin, CK	NA
42	2009	Magata et al[42]	78/F	NA	RUQ pain	NA	Body	CEA elevated	115 x 40 x 35	Gallbladder malignancy	Whole-layer cholecystectomy with regional lymph node dissection	NA	6+	Vimentin, CK	NA
43	2009	Shimada et al[43]	69/M	Choledocholithiasis	Fever	Normal	Entire gallbladder	AFP elevated	90 × 50	Gallbladder malignancy	Laparotomy, cholecystectomy, lymph node dissection	NA	54+	Vimentin, CK, Ki-67	NA
44	2009	Uzun et al[44]	70/M	NA	RUQ pain	Normal	Fundus	Normal	100 × 60 × 30	Gallbladder malignancy	Radical cholecystectomy, wedge resection of liver-gallbladder bed with hepatoduodenal ligament lymphadenectomy	NA	8	CK, Ki-67	NA
45	2006	Kubota et al[45]	72/M	NA	RUQ pain, fever	AST, ALT, ALP elevated	NA	Normal	70 × 55 × 40	Gallbladder malignancy	En bloc resection of the gallbladder and segments 4a and 5 of the liver, partial colectomy, and lymph node dissection	NA	6	NA	NA
46	2005	Akatsu et al[46]	76/F	Gallstones	Incidental finding on follow-up for cholelithiasis	Normal	NA	Normal		Gallbladder malignancy	Extended cholecystectomy, liver 4b and 5 resection	NA	2	Vimentin, CK	NA
47	2005	Huguet et al[47]	64/F	Cholecystitis	RUQ pain, fever	Normal	Entire gallbladder	Normal	120 × 100 × 70	Gallbladder malignancy	A cholecystectomy with wedge resection of the gallbladder fossa (involving liver segments 4 and 5), extrahepatic bile duct excision, non–pylorus-preserving pancreaticoduodenectomy with excision of 15 cm of proximal jejunum, and right hemicolectomy	NA	60	Vimentin, CK	NA
48	2005	Sodergren et al[48]	64/F	NA	Malaise and LOA	ALP Elevated	NA	NA	20 × 12 × 12	NA	Extrahepatic radical bile duct resection with hepatic and coeliac lymph node clearance followed by right hepaticodochojejunostomy to a jejunal Roux loop	NA	5	Vimentin, CK	NA
49	2005	Sodergren et al[48]	60/F	NA	Painless jaundice	NA	NA	NA	90	Gallbladder malignancy	Cholecystectomy and extrahepatic bile duct resection with lymph node clearance	NA	2	Vimentin, CK	Palliative (NM)
50	2004	Takahashi et al[49]	84/F	NA	RUQ pain	NA	Body	CEA, CA19-9 elevated	84 × 40 × 30	Gallbladder malignancy	Cholecystectomy and transverse colon partial colectomy	NA	2	Vimentin, CK
51	2003	Kim et al[50]	61/F	No stones	RUQ pain	Normal	Neck	Normal	45 × 40 × 40	Gallbladder malignancy	Cholecystectomy with common bile duct resection	NA	2	Vimentin	Palliative (NM)
52	2002	Al-Sheneber et al[1]	68/F	Acute cholecystitis, gallstones	RUQ pain	Normal	NA	CEA elevated	148 × 80	Gallbladder malignancy	CT guided needle biopsy of the upper abdominal mass	NA	7	Vimentin, CK	NA
53	2002	Hotta et al[51]	53/M	Chronic cholecystitis, gallstones	RUQ Pain	Normal	NA	Normal	1100	Gallbladder malignancy	Cholecystectomy with resection of subsegmentectomy of liver S5 and a resection of transverse colon at the second operation	II	2	NA	Adjuvant chemotherapy
54	2002	Ajiki et al[52]	69/F	Gallstones, left renal tumor	Epigastric pain	Normal	NA	CA19-9 elevated	NA	Double cancers of the left kidney and gallbladder	Left renal excision, cholecystectomy with liver segmentectomy (S4a, S5), and lymph node dissection	NA	NA	NA	Adjuvant chemotherapy
55	2000	Yavuz et al[53]	50/F	NA	RUQ pain	NA	Body	NA	80 × 60 × 60		Exploratory laparotomy -> cholecystectomy, liver wedge biopsy	NA	NA	NA	NA
56	1999	Eriguchi et al[54]	65/F	Gallstones	RUQ pain	Normal	Entire gallbladder	NA		Gallbladder malignancy	Cholecystectomy	I	16+	NA	NA
57	1997	Ryś et al[55]	67/F	Gallstones	Abdominal pain, LOW	NA	Fundus	NA	10 × 15	Gallbladder malignancy	Hemicolectomy and cholecystectomy	NA	2	Vimentin	NA
58	1996	Nakagawa et al[56]	60/F	NA	Abdominal pain, fever	Normal	Body	NA	70 × 40	Gallbladder malignancy	Mass reduction surgery	NA	NA	NA	NA
59	1994	Fagot et al[57]	83/F	Gallstones	Vomiting, fever, right RHC pain	Total bilirubin elevated	Fundus	NA	45	NA	Surgery (not defined)	NA	12+	NA	NA
60	1992	Nakazawa et al[58]	63/F	NA	Nausea	Normal	Body	Normal	30 × 30	Gallbladder malignancy	Pancreaticoduodenectomy	NA	NA	NA	NA
61	1990	Ishihara et al[59]	58/F	NA	Abdominal pain	NA	Fundus	NA	50 × 80	Gallbladder malignancy	Cholecystectomy	NA	11+	Vimentin	NA
62	1988	Lumsden et al[60]	81/F	Gallstones	RUQ pain, LOW, LOA	Total bilirubin, ALP, GOT elevated	NA	NA	50 × 20 × 20	Biliary neoplasm	Cholecystectomy	NA	12+	NA	NA
63	1987	Hasegawa et al[61]	61/M	NA	RUQ pain	Normal	Entire gallbladder	NA	150	Gallbladder malignancy	Resection (not specified)	NA	6	NA	NA
64	1987	Herrera-Goepfert et al[62]	60/F	Gallstones	Abdominal pain, jaundice, LOW		Entire gallbladder	NA	70 × 40	Gallbladder malignancy	Autopsy	NA	NA	NA	NA
65	1986	Inoshita et al[63]	53/M	Gallstones	RUQ pain, jaundice	Total bilirubin, ALP, GOT, GPT elevated	Neck	NA	NA	Choledocholithiasis	Open laparotomy	NA	17	NA	NA
66	1985	Lopez et al[64]	78/F	No stones	Anorexia, LOW	Normal	NA	NA	NA	Gallbladder empyema	Open laparotomy	NA	NA	NA	NA
67	1984	Born et al[65]	90/F	Gallstones	Anorexia, nausea, vomiting	Amylase elevated	NA	NA	150 × 150 × 10	NA	Exploratory laparotomy	NA	3	NA	NA
68	1982	von Kuster et al[66]	91/F	Gallstones	RUQ pain, fever	GOT, ALP elevated	NA	NA	20	Gallbladder empyema	Exploratory laparotomy	NA	0	NA	NA
69			77/F	NA	Bleeding in GI tract (lower)	Normal	NA	NA	30	NA	Exploratory laparotomy	III	31+	NA	NA
70	1982	Aldovini et al[67]	75/F	Gallstones	Abdominal pain	ALP, SGT elevated	NA	NA	90	NA	Cholecystectomy	NA	8+	NA	NA
71	1982	Yamagiwa et al[68]	78/F	NA	RUQ pain	NA	NA	NA	NA	NA	NA	NA		NA	NA
72	1980	Mansori et al[69]	81/M	Gallstones	Abdominal pain	GOT, ALP elevated	NA	NA	NA	NA	Exploratory laparotomy	NA	0.5	NA	NA
73	1973	Higgs et al[70]	77/M	Gallstones	Jaundice	ALP, GOT elevated	NA	NA	NA	NA	Cholecystectomy and CBDE	NA	1	NA	NA
74	1971	Mehrotra et al[71]	45/F	Gallstones	RUQ pain	Normal	Neck	NA	50 × 40 × 30	NA	Open laparotomy	NA	4	NA	NA
75	1970	Appelman et al[72]	91/M	Gallstones, chronic cholecystitis	Obstructive jaundice	AST, ALT, ALP elevated	Fundus	NA	NA	Pancreatic cancer	Autopsy	NA	0.5	NA	NA
76	1970	Appelman et al[72]	75/F	Gallstones, chronic cholecystitis	RUQ pain	ALP elevated	Fundus	NA	50 × 50 × 20	NA	Cholecystectomy	NA	1	NA	NA

GBCA: Gallbladder cancer; CEA: Carcinoembryonic antigen; AFP: Alpha-fetoprotein; CA 19-9: Carbohydrate antigen 19-9; UICC: Union for International Cancer Control; IHC: Immunohistochemistry; RUQ: Right upper quadrant; AST: Aspartate aminotransferase; ALT: Alanine aminotransferase; ALP: Alkaline phosphatase; GGT: Gamma-glutamyl transferase; ERCP: Endoscopic retrograde cholangiopancreatography; PTC: Percutaneous transhepatic cholangiogram; CCY: Cholecystectomy; NA: Not available; NM: Not mentioned; NIL: None; CR: Complete response; LOA: Loss of appetite; LOW: Loss of weight; R hemi w C: Right hemihepatectomy with cholecystectomy; M: Male; F: Female; Lap: Laparoscopic; Chole: Cholecystectomy; LN: Lymph node; CT: Computerized tomography; RH: Right hepatectomy; GI: Gastrointestinal; CBDE: Common bile duct exploration; HCG: Human chorionic gonadotropin; SGOT: Serum glutamic-oxaloacetic transaminase; SGPT: Serum glutamic-pyruvic transaminase.