Pancreatic Hepatoid Carcinoma: A Review of the Literature
Department of Surgery, Verona University Hospital, Verona, Italy. Digestive surgery
(Impact Factor: 2.16).
11/2013; 30(4-6):425-433. DOI: 10.1159/000355442
Hepatoid carcinomas (HCs) are extrahepatic neoplasms exhibiting features of hepatocellular tumors in terms of morphology and immunohistochemistry. They have been described in several organs, most notably in the stomach and ovary. They can present in pure forms or in association with other morphological aspects, such as endocrine tumors or ductal adenocarcinomas. The aim of this review is to describe aspects of hepatoid adenocarcinoma of the pancreas with regard to epidemiology, diagnosis, and treatment.
The PubMed database was searched for publications addressing hepatoid adenocarcinoma of the pancreas. We have searched for articles including the following keywords: 'pancreatic hepatoid carcinoma', 'ectopic liver cancer' and 'rare pancreas neoplasm' published to date. As references, we used case reports and review articles.
Pancreatic forms of HCs are extremely uncommon: only 22 cases have been reported.
The possibility of an HC of the pancreas should be considered in the differential diagnosis of an uncommon pathological mass of the pancreas. Treatment seems to be related to the association with other neoplasms, tumor extension at the time of diagnosis and the possibility to perform a radical resection. The common embryologic origin of the pancreas and liver, together with peculiar environmental factors, may explain the development of pancreatic HCs.
Available from: Jon Arne Søreide
- "Although the term hepatoid adenocarcinoma (HAC) of the stomach was introduced in 1985 by Ishikura et al., with the observation of high serum a-fetoprotein (AFP) levels in seven patients with gastric adenocarcinoma,[1,2]an AFPproducing gastric tumour was first reported by Bourreille et al.in 1970. HAC may also originate from other gastrointestinal localisations,[4,5]including the pan- creas.[6,7]Moreover, this extrahepatic hepatoid cancer type has also been diagnosed in the ovary,uterus,lungs,[10,11]and several other organs.[4,12]The "
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ABSTRACT: Objective The term hepatoid adenocarcinoma (HAC) of the stomach was introduced three decades ago with the observation of high serum α-fetoprotein (AFP) levels in some gastric adenocarcinoma patients. This very rare gastric cancer patient subgroup is likely frequently misdiagnosed. Material Two patients who were recently diagnosed with HAC of the stomach at our institution are presented. We also performed a structured literature search and reviewed pertinent articles to provide knowledge to improve the proper identification, diagnosis and management of patients with gastric HAC. Results HAC is a rare subgroup of gastric carcinoma with poor prognosis. Clinical management of this population may be challenging. The scientific literature is largely based on very small patient series or case reports, and the evidence for proper decision making and management is considered weak. Conclusion All physicians involved in the diagnosis and treatment of patients with gastric cancer should pay attention to this rare subgroup to improve identification.
Available from: Shih-Chin Chen
- "Clinically, the incidence of hepatocellular carcinoma spreading to the pancreas is low, only 2.7–5.6 %, and the metastasis is usually a late finding[1,44]. Long-term follow-up is needed to exclude the possibility that the pancreatic tumor represents a metastasis from the liver. If bile production cannot be identified histologically, then the differential diagnosis should also include other primary pancreatic tumors with large eosinophilic tumor cells, such as intraductal oncocytic papillary neoplasms, pancreatoblastoma, poorly differentiated pancreatic adenocarcinoma, islet cell tumors, and acinar cell carcinoma, but should be easily distinguished by histological examinations for their own characteristics of morphology and immunohistochemistry[1,34528].With limited experience due to its rarity, the treatment approach is far from being standardized. "
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ABSTRACT: Hepatoid carcinoma of the pancreas is extremely rare. This article tries to summarize the clinical features and outcomes of pancreatic hepatoid carcinoma.
The data pool for analysis includes the case we encountered with hepatoid carcinoma of the pancreas and the reported cases in the literature.
Twenty-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.
Elevation 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.
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Diagnosis of primary gastrointestinal and liver neoplasms is usually straightforward. Immunohistochemistry is most helpful to differentiate metastatic carcinomas with morphologic similarity and to resolve tumors of unknown origin. Recently, several new markers highly sensitive and specific for primary liver and gastrointestinal tumors have been discovered. Their potential diagnostic application has not been widely appreciated by general practicing pathologists. In addition, a new trend in immunohistochemistry application has started, focusing on assessing predictive markers (such as human epidermal growth factor receptor 2) and mutation-specific markers (v-raf murine sarcoma viral oncogene homolog B V600E) to directly guide clinical management. Practicing pathologists need to be aware of and prepared for this evolving trend.
To summarize the usefulness of several recently discovered immunohistochemical markers in the study of gastrointestinal and liver tumors; to suggest the most current and effective immunohistochemical panels addressing common diagnostic challenges for these tumors; to share practical experience and useful tips for human epidermal growth factor receptor 2 testing in gastric and gastroesophageal junction adenocarcinoma and v-raf murine sarcoma viral oncogene homolog B V600E immunohistochemistry in colorectal carcinoma.
Sources include literature review, and authors' research data and practice experience. The cases illustrated are selected from the pathology archives of the Geisinger Medical Center (Danville, Pennsylvania).
Application of immunohistochemistry in gastrointestinal and liver tumors continues to evolve. New tumor-specific markers constantly emerge and help pathologists to further improve diagnostic accuracy. Assessment of predictive and prognostic markers by immunohistochemistry in routine pathologic diagnosis is a new trend and will greatly facilitate the advancement of personalized cancer therapy.
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