Extremely high alpha-fetoprotein-producing adrenal hepatoid adenocarcinoma

Abstract

Hepatoid adenocarcinoma (HAC) is a rare tumour that produces an alpha-fetoprotein (AFP) mimicking hepatocellular carcinoma (HCC). Adrenal HAC is exceedingly rare. Here we report extremely high AFP-producing adrenal HAC, the first case in Thailand. A 47-year-old man presented with left flank pain and weight loss for 2 months. A palpably huge left flank mass was observed on physical examination. CT revealed a 7 cm enhanced mass involving the left adrenal gland and multiple contrast-enhanced hypodense masses in both liver lobes. The largest was a 3.7 cm at liver segment-VII without cirrhotic background, with an AFP level of 321 495 ng/mL. Both adrenal and liver biopsies were performed. This patient received a diagnosis of advanced adrenal HAC. Unfortunately, the tumour progressed, causing massive upper gastrointestinal bleeding and death. Adrenal HAC is challenging to diagnose, which multifocal HCC, pheochromocytoma and adrenocortical carcinoma should be excluded. Surgical resection is preferred among resectable patients. However, no systemic therapy has been standardised.

Keywords: endocrine cancer; hepatic cancer; pathology; urological cancer.

Figure 1

(A) CT scan with four-phase contrast shows an irregular infiltrative heterogeneous enhanced mass involving the left hemidiaphragm and left adrenal gland mass with several hypodense lesions scattered throughout the lobes of the liver. No radiological evidence supports liver cirrhosis. (B) The CT study shows an interval increase in the left adrenal gland mass, now measuring about 11.2×15.2×5.5 cm. Interval increased extension of necrotic soft tissue and multiple matted necrotic nodes involving the left hemidiaphragm, pericardial fat pad, pericardium, periaortic, gastrohepatic and peripancreatic regions, which are unprecedented. This lesion shows the direct invasion of the distal oesophagus and gastric cardia, causing proximal oesophageal dilation. Increased extension of tumour thrombus in inferior vena cava extending into the right hepatic vein ascends to the right atrium and extension in the left inferior pulmonary vein reaching into the left atrium. These conditions progress within 3 weeks after the first CT study.

Figure 2

(A) Sections of liver nodule core biopsies show an epithelial neoplasm consisting of the proliferated polygonal epithelial cells arranged in 6–7 cell thick trabeculae and nested separately by flat endothelial lining sinusoidal spaces. The neoplastic cells contain vesicular and slightly pleomorphic irregular thick nuclear membrane nuclei, prominent nucleoli and rare mitoses. Intranuclear cytoplasmic inclusions are noted. Adjacent normal liver parenchyma is present on the right side of the picture. These biopsies appear to have positive stain for AE1/AE3, arginase-1, glypican-3 and CAM5.2, but a negative stain for CK7, CK20, inhibin, S100, synaptophysin or chromogranin A. Hepatocellular carcinoma or hapatoid adenocarcinoma is suggested from these results. (B) Sections of adrenal mass core biopsies show an epithelial neoplasm within a desmoplastic stroma. The adrenal mass shares similar histological features to the liver nodule; positive stain for AE1/AE3, arginase-1, glypican-3 and CAM5.2 and negative stain for CK7, CK20, inhibin, S100, synaptophysin or chromogranin A. No residual non-neoplastic adrenal tissue was present in the core biopsies. Thus, we can conclude the resemblance of both liver nodules and adrenal mass.