Hepatoid carcinoma of the pancreas: A case report and review of the literature

Table 1 Summary of clinical features of hepatoid carcinomas of the pancreas reported in the English language literature

Ref.	Age	Sex	Clinical presentation	Serum AFP levels at diagnosis	Serum CEA levels at diagnosis	Location	Tumor size in cm	Associated component	Site of metasta-sis	Treatment	Clinical follow-up in mo
Hruban et al[11], 1987	53	F	Subcutaneous fat necrosis and polyarthritis	Normal	Not available	Tail	1	Acinar cell carcinoma	Liver	Chemotherapy (5-FU, Adriamycin)	Died of disease (2.75)
Tanno et al[12], 1999	65	F	Epigastric and back pain, anorexia, and weight loss	Elevated	Elevated	Bodytail	6 × 5	Ductal adenocarcinoma	Liver, right supraclavicular, and paraaortic lymph node	Palliative care	Died of disease (6)
Yano et al[13], 1999	57	M	Jaundice, epigastric pain, vomiting and fever	Elevated	Elevated	Head	9 × 7 ×5	Ductal adenocarcinoma	No	Surgery (pancreatoduodenectomy)	Died of disease (3)
Paner et al[14], 2000	28	M	Severe abdominal and back pain	Elevated	Elevated	Multifocal	8 × 8 × 6	Ductal adenocarcinoma	Widespread (gastric, ileal, and colonic mucosa)	Debulking of the tumor plus chemotherapy	Died of disease (14)
Paner et al[14], 2000	57	M	Vomiting, diarrhea, weight loss, diffuse skin rashes and diabetes mellitus	Elevated	Elevated	Tail	6 × 4 × 3.5	Neuroendocrine neoplasms (glucagonoma)	Liver	Surgery (distal pancreatectomy with splenectomy) plus chemotherapy	Died of disease (102)
Lam et al[15], 2001	64	F	Hypoglycemia and recurrent nocturnal sweating	Elevated	Not available	Tail	7 × 4 × 4	Insulinoma	Liver	Distal pancreatectomy with splenectomy plus regional embolization and systemic chemotherapy	Died of disease (22)
Cuilliere et al[16], 2002	70	M	Incidental (asymptomatic)	Normal	Normal	Body	3	Serous microcystic adenoma	No	Distal pancreatectomy with splenectomy	Alive with no evidence of recurrence (12)
Hughes et al[17], 2004	51	M	Incidental finding	Normal	Normal	Tail	6 × 5.5 × 5.5	No	No	Total pancreatectomy	Alive with no evidence of recurrence (14)
Matsueda et al[18], 2006	49	F	Weight loss	Elevated	Normal	Widespread	Not available	No	Liver (detected after 12 mo)	Surgery (total pancreatectomy), chemotherapy (gemcitabine) and liver lobectomy	Alive with no evidence of recurrence (48)
Shih et al[19], 2006	32	M	Incidental (asympto-matic)	Normal	Elevated	Tail	7	No	No	Surgery (distal pancreatectomy with splenectomy)	Alive with no evidence of recurrence (18)
Oh et al[20], 2006	21	M	Incidental (asymptomatic)	Elevated	Not available	Head	3 × 3 × 3	Neuroendocrine neoplasm	No	Surgery (pancreatoduodenectomy)	Alive with no evidence of recurrence (7)
Cardona et al[21], 2007	58	M	Back and flank pain	Normal	Not available	Body	3.3 × 2.5 × 2.5	No	No	Surgery (distal pancreatectomy with splenectomy)	Alive with no evidence of recurrence (15)
Kubota et al[22], 2007	56	M	Diabetes	Not available	Not available	Tail	6.3 × 6.2	No	No	Surgery (distal pancreatectomy with splenectomy)	Alive with no evidence of recurrence (36)
Hameed et al[23], 2007	41	F	Gastro-esophageal reflux, jaundice, and abdominal pain	Elevated	Elevated	Head	4.5 × 4 × 3	Neuroendocrine neoplasm	Liver	Pancreaticoduodenectomy plus Chemotherapy with cisplatin and irinotecan	Died of disease (27)
Liu et al[24], 2007	80	M	Nausea, diarrhea weight loss and epigastric palpable mass	Normal	Not available	Head	5 × 5 × 6	No	Transverse colon	Simple tumorectomy and partial transverse colon resection	Alive with no evidence of recurrence (8)
Zhang et al[25], 2007	37	F	Upper abdominal pain, anorexia, and emaciation	Elevated	Not available	Widespread	9	Neuroendocrine neoplasm	No	Surgery (pancreatoduodenectomy)	Died of disease (3)
Jung et al[26], 2010	46	M	Dyspepsia and epigastric palpable mass	Elevated	Elevated	Head	8 × 9	Neuroendocrine neoplasm	No	Radical pancreatoduodenectomy	Alive with no evidence of recurrence (4)
Petrelli et al[27], 2012	37	F	Epigastric abdominal mass	Not available	Normal	Body	11	No	Liver, mediastinal lymph nodes, and lungs	Chemotherapy with sorafenib	Died of disease (12)
Kelly et al[28], 2012	53	F	Severe epigastric pain	Elevated	Not available	Bodytail	Not available	No	Liver	Distal pancreatectomy and adjuvant chemotherapy (carboplatin and gemcitabine)	Alive with no evidence of recurrence (22)
Kai et al[29], 2012	79	F	Incidental (asymptomatic)	Elevated	Elevated	Tail	7	No	Stomach, left adrenal gland, and liver	Distal pancreatectomy with splenectomy, combined with resection of the left adrenal gland and total gastrectomy	Died of disease (2)
Huang et al[30], 2012	52	M	Jaundice, anorexia and epigastric pain	Not available	Elevated	Head	0.5 nodule	Neuroendocrine tumor	No	Pancreaticoduodenectomy plus chemotherapy with sunitinib	Alive with no evidence of recurrence (16)
Majumder et al[31], 2013	69	M	Left upper quadrant pain, jaundice and nausea	Normal	Not available	Head	5.8 × 6.0	No	Liver	Biliary drainage plus chemotherapy with gemcitabine	Died of disease (3)
Steen et al[32], 2013	61	F	Incidental (asymptomatic)	Normal	Not available	Tail	5.3 × 3.5	No	No	Distal pancreatectomy with splenectomy	Alive with no evidence of recurrence (60)
Xin et al[33], 2014	33	F	Incidental (asymptomatic)	Elevated	Normal	Head	2 × 1.4 × 1.8	Neuroendocrine neoplasm	No	Pancreaticoduodenectomy plus chemotherapy with gemcitabine	Alive with no evidence of recurrence (46)
Vanoli et al[34], 2015	57	F	Jaundice	Elevated	Elevated	Head	3.5 × 3 × 3	No	No	Pancreatoduodenectomy and adjuvant chemotherapy (gemcitabine)	Alive with no evidence of recurrence (10)
Soofi et al[35], 2015	69	M	Atypical chest pain	Elevated	Normal	Body and tail	5.9	No	No	Distal pancreatectomy with splenectomy	Alive with no evidence of recurrence (4)
Antonini et al[36], 2015	59	M	Weight loss and abdominal discomfort	Normal	Normal	Body	6 × 5	No	No	Chemotherapy with sorafenib	Died of disease (4)
Kuo et al[37], 2015	67	M	Incidental (asymptomatic)	Normal	Normal	Tail	2 × 2	No	No	Distal pancreatectomy with spleen preservation	Alive with no evidence of recurrence (6)
Veerankutty et al[38], 2015	47	M	Incidental	Not available	Normal	Tail	3.1 × 2.9 × 2.6	Serous cystadenoma	No	Distal pancreatectomy with splenectomy (laparoscopic)	Alive with no evidence of recurrence (8)
Williams et al[39], 2015	71	M	Pancreatitis and melena (oozing ulcer at the ampulla)	Normal	Normal	Head	5	No	Duodenal muscularis propria	Pancreaticoduodenectomy and cholecystectomy	Not available
Stamatova et al[40], 2016	78	M	Incidental (asymptomatic)	Normal	Normal	Head	8 × 6	No	No	Pancreaticoduodenectomy	No evidence of recurrence but died of heart attack (2)
Chang et al[41], 2016	61	M	Incidental (asymptomatic)	Not available	Not available	Body and tail	1.3	No	No	Distal pancreatectomy	Alive with no evidence of recurrence (6)
Akimoto et al[42], 2016	59	M	Incidental (asymptomatic)	Normal	Normal	Body	5.0 × 3.5	No	No	Middle pancreatectomy	Alive with no evidence of recurrence (12)
Pellini Ferreira et al[43], 2017	43	M	Jaundice, epigastric pain, and watery diarrhea	Normal	Normal	Tail	9.0	Neuroendocrine neoplasm	Spleen and liver	Chemotherapy with capecitabine and temozolomide	Alive with no evidence of recurrence (16)
Ma et al[44], 2017	75	M	Weight loss	Elevated	Elevated	Tail	7.8	No	Liver	Chemotherapy with mFOLFIRINOX (oxaliplatin + leucovorin + irinotecan + 5-fluorouracil) plus surgery (distal pancreatectomy with splenectomy and wedge resection of the liver)	Alive with no evidence of recurrence (10)
Yang et al[45], 2018	83	M	Abdominal pain	Not available	Not available	Body	2.7 × 2.5 × 1.5	No	No	Laparoscopic distal pancreatectomy and splenectomy	Alive with no evidence of recurrence (107)
Yang et al[45], 2018	72	M	Severe back pain	Not available	Not available	Tail	12.0 × 10.5 × 4.5	No	No	Distal pancreatectomy with splenectomy	Died of pulmonary embolism (1)
Yang et al[45], 2018	54	M	Incidental (asymptomatic)	Elevated	Normal	Body and tail	10.0 × 9.0 × 9.0	No	Adjacent transverse colon	Distal pancreatectomy along with splenectomy and left hemicolectomy	Died of disease (29)
Our case	36	M	Progressive jaundice, weight loss and epigastric palpable mass	Elevated	Normal	Widespread	6.0 × 7.0	No	Celiac trunk, root of transverse mesocolon, and upper mesojejunum	Palliative care	Died of disease (4)

AFP: Alpha-fetoprotein; CEA: Carcinoembryonic antigen; F: Female; M: Male.

Table 2 Outline of main features characterizing presentation of the 39 cases of hepatoid carcinoma of the pancreas

Variable	n (%) or median (IQR)
Sex
Female	12 (30.77)
Male	27 (69.23)
Age in yr
Median (range)	57 (21-83)
Symptoms
Asymptomatic	13 (33.33)
Pain: Abdominal/back	13 (33.33)
Gastrointestinal symptoms: Vomiting, diarrhea, and dyspepsia	9 (23.08)
Weight loss	8 (20.51)
Jaundice	7 (17.95)
Epigastric mass	3 ( 7.69 )
Location
Tail	13 (33.33)
Head	11 (28.21)
Body	6 (15.38)
Body and tail	5 (12.82)
Diffuse or multifocal	4 (10.26)
Size of longest diameter in cm
Median (range)	6 ( 1-12 )
Metastasis	17 (43.59)
Liver metastasis	12 (30.77)
Associated component: Mixed form	15 (38.46)
Elevated AFP	18 (46.15)
Elevated CEA	11 (28.21)

AFP: Alpha-fetoprotein; CEA: Carcinoembryonic antigen; IQR: Interquartile range.