Ann Hepatobiliary Pancreat Surg.  2018 Aug;22(3):287-291. 10.14701/ahbps.2018.22.3.287.

Adrenal metastasis in sequentially developed combined hepatocellular carcinoma-cholangiocarcinoma: A case report

• Nugroho A
• Lee KW
• Lee KB
• Kim HS
• Kim H
• Yi NJ
• Suh KS

• KMID: 2420619
• DOI: http://doi.org/10.14701/ahbps.2018.22.3.287

Abstract

The incidence of combined hepatocellular carcinoma-cholangiocarcinoma (cHCC-CC) in a single patient accounts for only 0.4 to 14% of all primary liver cancer. However, the prognosis of its intrahepatic cholangiocarcinoma (ICC) component is poor. We experienced a unique case of a sequentially developed cHCC-CC with adrenal metastasis as the primary presentation and a hidden primary hepatocellular carcinoma. A 65-year-old female with a history of jaundice and abdominal discomfort was diagnosed with S4 ICC measuring 5 cm in diameter, and characterized histologically as papillary adenocarcinoma with intraductal growth, but without any evidence of malignant hepatocyte. S4 segmentectomy with hepaticojejunostomy revealed no additional masses. A follow-up CT scan 3 months after surgery showed a right adrenal mass with markedly increased serum AFP (4950 ng/mL), which was treated with right adrenalectomy. Histopathology revealed a metastatic hepatocellular carcinoma testing positive for AFP, glypican-3, and hepatocytes, but negative for CD-10, inhibin-α, EMA, S-100, and cytokeratin-7. Serum AFP level immediately plummeted to 4.1 ng/mL upon adrenal mass removal. A recurrent S7 liver mass was suspected 1 year later with serum AFP value of 7.6 ng/mL, and characteristic CT imaging of HCC. TACE was performed with good response. Adrenal metastasis may manifest as the primary focus of hepatocellular carcinoma in sequentially developed cHCC-CC patients with hidden primary HCC. cHCC-CC should be considered in the differential diagnosis of cholangiocarcinoma with elevated AFP.

Keyword

Adrenal metastasis; Combined hepatocellular-cholangiocarcinoma; Extrahepatic-recurrence

MeSH Terms

Adenocarcinoma, Papillary
Adrenalectomy
Aged
Carcinoma, Hepatocellular
Cholangiocarcinoma
Diagnosis, Differential
Female
Follow-Up Studies
Glypicans
Hepatocytes
Humans
Incidence
Jaundice
Keratin-7
Liver
Liver Neoplasms
Mastectomy, Segmental
Neoplasm Metastasis*
Prognosis
Tomography, X-Ray Computed
Glypicans
Keratin-7

Figure

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  Fig. 1 Initial preoperative CT scan showing a multiple intra-ductal gradually enhancing lesions within the left intrahepatic duct.

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  Fig. 2 Liver S4 resection specimen with intraductal tumor infiltration suggesting intrahepatic cholangiocarcinoma.

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  Fig. 3 Post liver resection imaging studies showing a metastatic right adrenal mass.

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  Fig. 4 Metastatic hepatocellular carcinoma of adrenal gland.

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  Fig. 5 Levels of AFP (ng/mL) and prothrombin in vitamin K absence II (PIVKA-II) PIVKA-II (mAU/mL) in the disease (logarithmic scale).

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  Fig. 6 CT at 15 months post right adrenalectomy showed intrahepateic recurrence near resected right adrenal, 17 months after transarterial chemoembolization (TACE) showed a small viable HCC and 19 months after radiofrequency ablation (RFA) showed no viable HCC.

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