Hepatoid Carcinoma of the Pancreas: Case Report, Next-Generation Tumor Profiling, and Literature Review

Abstract

Fewer than 25 cases of hepatoid carcinoma of the pancreas have been reported in the literature. We present a case in a 61-year-old male with a remote history of Hodgkin’s lymphoma and gastric neuroendocrine cell hyperplasia. On surveillance endoscopic ultrasound, an 8 × 5 mm cystic lesion was seen in the tail of the pancreas. MRI showed a focal pancreatic duct cut-off with mild ductal dilation. Fine needle aspiration was performed, which was concerning for acinar cell carcinoma. The patient underwent distal pancreatectomy and recovered uneventfully. Final pathology demonstrated a 1.3-cm hepatoid carcinoma of the pancreas, with a final clinicopathological stage of T1N0M0. Next-generation nucleic acid sequencing of the tumor did not suggest a viable adjuvant chemotherapeutic agent, and no adjuvant therapy was administered. The patient has no evidence of disease 6 months following resection. A further characterization and description of the outcomes of these rare tumors is warranted to help guide providers and counsel patients.

Primary Hepatoid Carcinoma of the Pancreas: A Clinicopathological Study of 3 Cases With Review of Additional 31 Cases in the Literature

“…Serum AFP level was elevated in 6 (mean = 179.7 ng/mL; range = 11-623 ng/mL), 10,15,23,26,30,31 normal in 9, † and not measured in 7 patients. 12,18,24,33,36 This subtype shows similar morphological features to orthotopic HCC. Intracytoplasmic bile and bile plug within canalicular and pseudoglandular spaces were identified in 12/22 (55%) tumors.…”

“…Hepatoid carcinomas of the pancreas are often positive for hepatocellular immunohistochemical markers (Table 4) such as AFP (18/27 or 67%), Hep Par1 (25/26 or 96%), glypican-3 (7/9 or 78%), and arginase-1 (3/4 or 75%). 9-38 Hepatoid carcinomas with pure HCC-like morphology are less likely to be immunohistochemically positive for AFP (9/17 or 53%) than those with other differentiation (9/9 or 100%; P = .007). Among the hepatoid carcinomas with neuroendocrine differentiation, those with homogeneous tumor cells show AFP 22,38 and Hep Par1 22,34 in the same group of cells, whereas those with 2 different populations of cells show positive AFP and Hep Par1 in the hepatoid component but not in the neuroendocrine component.…”

“…50 So far, none of the reported PHCs, including our 3 cases, occurred in children. 9-38 Pancreatic neuroendocrine tumors (islet cell tumors) may contain oncocytic cytoplasm 51 and should be distinguished from hepatoid carcinoma with neuroendocrine differentiation. Pancreatic neuroendocrine tumors are negative for glypican-3.…”

“…According to the stem cell theory, liver and pancreas share the same embryonic derivation, and the genes controlling hepatocytic differentiation are normally repressed in the pancreatic stem cells; these genes could be activated during carcinogenesis. 9,30 Ectopic liver tissue has been reported to be associated with an increased risk of developing HCC 54 ; however, so far, ectopic hepatic tissue has not been demonstrated in the pancreas or in any reported PHC 9-38 even though one study claimed that the hepatoid carcinoma arose from ectopic live tissue. 17 In the literature, there is only 1 reported hepatic adenoma in the pancreas.…”

“…3-8 The first primary pancreatic hepatoid carcinoma (PHC) was reported by Hruban et al in 1987. 9 So far, only 31 primary PHCs have been reported in the English literature, 9-38 and the vast majority of them are single case reports except 1 study with 2 cases. 12 In addition, the vast majority of these reports were in nonpathology journals, * and therefore, the pathological features of these tumors and their correlation with prognosis have not been analyzed.…”