Abstract
BackgroundHepatoid carcinoma of the pancreas is extremely rare. This article tries to summarize the clinical features and outcomes of pancreatic hepatoid carcinoma.MethodsThe data pool for analysis includes the case we encountered with hepatoid carcinoma of the pancreas and the reported cases in the literature.ResultsTwenty-three cases of hepatoid carcinoma of the pancreas were analyzed. This tumor occurred more frequently in male than in female patients (69.6 vs. 30.4 %). Tumor sizes range from 0.5 to 11.0 cm with median of 6.0 cm. The most common symptom was epigastric pain (36.4 %). When the tumor locates at pancreatic head, nausea/vomiting (62.5 %) is more common, followed by jaundice and epigastric pain (50.0 %). For those at pancreatic body-tail, 42.9 % of the patients presented no symptom. Alpha-fetoprotein (AFP) was abnormally elevated in 60 % of the cases. Hepatoid carcinoma in the pancreas could be either pure form or mixed form with other malignancy (40.9 %), with the most common coexisted pathology of malignant neuroendocrine tumor (22.7 %). Metastasis occurred in 36.4 % of the cases at the diagnosis of this tumor, including liver metastasis in 31.8 % and lymph node metastasis in 21.1 %. The overall 1-year survival rate was 71.1 % and 5-year 40.4 %, with a median of 13.0 months. Unresectability, hepatic, and lymph node metastases are associated with negative impact on survival outcome.ConclusionsElevation of serum AFP may be a clue leading to the diagnosis of pancreatic hepatoid carcinoma. This tumor could be mixed form with other malignancy. Surgical resection should be the treatment of choice whenever possible.
Highlights
Hepatoid carcinoma of the pancreas is extremely rare
To identify the relevant articles dealing with hepatoid carcinoma of the pancreas in the English literature, a computerized search was performed on the PubMed electronic database, covering data from 1987 to 2014
A total of 23 cases of hepatoid carcinoma of the pancreas were collected for this study, including 22 cases from case reports in literature and 1 from our institute
Summary
Hepatoid carcinoma of the pancreas is extremely rare. This article tries to summarize the clinical features and outcomes of pancreatic hepatoid carcinoma. The first case reported by Ishikura et al in 1985 was described in the stomach [9], while Hruban et al reported the first case in the pancreas in 1987 [5, 10] Documentation of this unique histopathologic feature has been made in other extrahepatic sites including the esophagus, papilla of Vater, colon, lung, gallbladder, adrenal gland, kidney, urinary bladder, ovary, uterus, vagina, and testicle [1, 5,6,7,8,9, 11,12,13,14,15,16,17,18,19,20,21,22,23]. An attempt is made to clarify the characteristics, clinical presentations, managements, and survival outcomes of this rare tumor
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