Sarcomatoid carcinoma of the pancreas — a rare tumor with an uncommon presentation and course: A case report and review of literature

Table 1 Summary of reported cases of sarcomatoid carcinoma of the pancreas

Ref.	Age (yr)/ gender	Involved part of the pancreas	Tumor extension	Therapeutic schedule	Tumor size, cm	Sarcomatoid component	Follow-up time/results
Cresson et al[31], 1987	69/male	Head and tail	NA	Mitomycin, adriamycin, and 3000 rads of external radiation to the stomach	NA	Tubular structures, desmosomes, and hemijunctions under electron microscope	5 mo/hemorrhage after surgery of metastasis in the jejunum
Higashi  et al[19], 1999	74/male	Head	Head of the pancreas and the adjacent duodenum, with blood vessel and perineural sarcomatoid	Pylorus preserving pancreatoduodenectomy	4.5 × 4 × 3	CK AE1/AE3 (+), EMA (+), MUC1 (-), ARA (+), S100 (+), SMA (+), desmin (–), vimentin (–)	3 mo/died after surgery of peritoneal carcinomatosis
Darvishian et al[32], 2002	74/male	Head	Peripancreatic adipose tissue and the duodenal wall.	Pancreatoduodenectomy	4.0 × 3.0	Vimentin (+), CK (+), CEA (+), SMA (+), desmin (+) and CD68 (-)	4 mo/alive and well
De la Riva et al[33], 2006	72/female	Head	NA	NA	NA	CK and vimentin (+)	9 mo/deceased with hepatic metastasis
Kim et al[21], 2006	73/female	Body and tail	Local invasion. With retroperitoneal lymph node with metastasis	Pancreatectomy with splenectomy and colonic segmental resection	20 ×15 ×13	CK (-), Vimentin (+), CD68 (+)	4 mo/deceased secondary to hepatic and peritoneal metastases
Ren et al[13], 2013	48/male	Tail	Free surgical margins	Surgery N/A. Digital subtraction angiography interventional chemotherapy was then implemented. Gemcitabine, oxaliplatin, and floxuridine were intravenously injected via the superior mesenteric artery and celiac trunk artery.	10 cm × 8 cm × 3.5	Vimentin, α-1-antichymotrypsin, CK-19, CK-18, and pan-CK (+). CD68 and lysozyme (-)	36 mo/alive and well
Yao et al[15], 2013	48/male	Tail	Free surgical margins	Laparoscopic spleen-preserving left pancreatectomy, adjuvant gemcitabine 1 cycle	10 × 8 × 5	CK 18 and vimentin (+)	3 mo/tumor recurrence and death
Kane et al[9], 2014	85/male	Body	Local invasion with free surgical margins	A distal pancreatectomy, splenectomy, and partial gastrectomy	3.3 × 3.0 × 2.6	Pan-CK, CK5.2 (+), S100, SMA, EMA (-)	26 mo/alive and well
Lai et al[34], 2015	55/male	Body and tail	NA	Distal pancreatectomy, splenectomy, and colonic segmental resection	14	CK, CK7, and vimentin (+)	NA
Nambiar et al[35], 2017	41/male	Head and uncinate	Liver metastasis	Gemcitabine	2.2 × 2.1	CK (+) and vimentin (+)	1 mo/on chemotherapy when reported
Ruess et al[36], 2017	73/female	Head of pancreas	Free surgical margins	Extended pylorus-preserving pancreatoduodenectomy	4.2	Pan-CK1/3 (+), CK7 (+), CK19 (+). Vimentin (+). S100 (+)	4 mo/death after surgery
Xie et al[16], 2018	63/male	Head of pancreas	Invasion of the distal common bile duct. Local invasion of the peripheral nerves. The lymph nodes, blood vessels, and resection margins were free from tumor tissue.	Pancreatoduodenectomy. 15 d of thymopeptides (1 mg per day).	2.5 × 2 × 1.8 cm	Vimentin (+), CK7 (+), and CK19 (+)	16 mo/hepatic metastasis
Bukhari and Joudeh[17], 2019	64/male	Head	Free surgical margins	Pancreatoduodenectomy with cholecystectomy and adjuvant gemcitabine	2.4 × 2 × 1.9	CAM 5.2 (-), vimentin (+)	19 mo/alive and well
Zhou et al[14], 2019	59/male	Head	Pancreatic head with extension into the main pancreatic duct. Free surgical margins. Three out of 23 lymph nodes were positive for metastasis	Pancreatoduodenectomy	2.5 × 2.5 × 2.0	CK19 (+) and vimentin (+)	6 mo/liver metastasis and peritoneal metastasis
Kimura et al[10], 2020	58/male	Body	Three lymph nodes out of 40 with direct invasion	Distal pancreatectomy with splenectomy. A six-month course of gemcitabine	5	CK (+) and vimentin (+). PSmad2/3, snail, and fibronectin	120 mo (10 yr)/alive and well
Omrani et al[18], 2020	73/male	Tail	NA	En bloc resection of the tail of the pancreas, spleen, a part of the stomach, and postoperative adjuvant chemotherapy with gemcitabine	10	OCG were positive for CK19 and CK7	120 mo (10 yr)/colonic metastasis
Our case	61/female	Tail	Free surgical margins, one lymph node compromised	Distal pancreatectomy and en-bloc splenectomy	3.2 × 2.9	Pan-CK (+) and vimentin (+)	35 mo/alive and well

CEA: Carcinoembryonic antigen; (-): No positivity; (+): Positivity; CK: Cytokeratin; EMA: Epithelial membrane antigen; NA: Not available; OCG: Osteoclastic giant cells; SMA: Smooth muscle actin; MUC1-ARA: Apoprotein MUC1.