Liver metastasis from hepatoid adenocarcinoma of the esophagus mimicking hepatocellular carcinoma

Abstract

Alpha-fetoprotein (AFP)-producing adenocarcinoma, histologically mimicking hepatocellular carcinoma (HCC), is a distinct entity known as hepatoid adenocarcinoma (HAC). Reported cases of HAC arising from the esophagus are extremely rare. Due to common liver metastasis and elevated AFP levels in patients with esophageal HAC, differentiation of HAC with liver metastasis from HCC could be challenging. We describe a case of esophageal HAC that presented with a liver mass showing hepatoid features and elevated serum AFP levels. Initial presentation was suspicious for HCC. Upon further diagnostic work-up, the patient was diagnosed with esophageal HAC with liver metastasis. The distinction between these two entities is particularly important because HAC is more aggressive, and its therapeutic options are very limited.

Keywords: alpha-fetoproteins; hepatocellular carcinoma; hepatoid adenocarcinoma.

Figure 1.

Computed tomography scan reveals a 12.6 × 11.2 cm liver mass with central necrosis and heterogeneous enhancement (white arrow) and esophageal wall thickening (gray arrow) with a large luminal mass (black arrow) almost obstructing the lumen.

Figure 2.

Endoscopic findings. (A) Lower esophagus showed two columns of tumor extending approximately 5 cm upstream from the gastroesophageal junction. (B) A bulky tumor causing a ball valve-like effect, obstructing the gastroesophageal junction. (C) Debulking was performed using a mini snare and a spiral snare. (D) Tumor burden was significantly decreased.

Figure 3.

Histopathology and immunohistochemistry studies. (A) Liver specimens show polygonal tumor cells arranged mainly in a trabecular pattern and areas of glandular formations with abundant eosinophilic cytoplasm and round nuclei (hematoxylin and eosin stain, original magnification × 100). (B) Esophageal specimens show neoplastic hepatocyte-like cells arranged in nests and trabecular pattern with intervening fibrovascular stroma. Adjacent intestinal metaplasia (Barrett’s esophagus) is evident (hematoxylin and eosin stain, original magnification × 100). (C) Neoplastic cells from esophageal tumor were positive for alpha-fetoprotein staining (original magnification × 200). (D) Esophageal specimens show positive staining for glypican-3 (original magnification × 200). (E) Carcinoembryonic antigen (polyclonal) staining of esophageal specimens, although positive, did not show the definite canalicular staining pattern of hepatocellular carcinoma (original magnification × 400). (F) Esophageal specimens were positive for SALL4 staining (original magnification × 200).

Figure 4.

Positron emission/computed tomography scan reveals a large, intense metabolically active, low-density lesion with central photopenic area in the liver and an intense metabolically active area at the gastroesophageal junction.