Clinicopathological features of combined hepatocellular-cholangiocarcinoma with sarcomatous change: Case report and literature review

Abstract

Rationale: Combined hepatocellular-cholangiocarcinoma (cHCC-CC) is a rare subtype of primary liver malignancy comprising <1.5% of all primary liver tumors. Sarcomatoid changes in cHCC-CC are even rarer. Due to the rarity of this subtype, its clinicopathological feature is poorly understood. Therefore, here we report 2 tumors.

Patient concerns: The first patient was a 44-year-old man with 5-year history of hepatitis B-induced cirrhosis. The resection of right liver revealed a 2.5 × 2.5 × 2 cm tumor mass. Histologically, the tumor showed areas of the typical moderately differentiated HCC. An intermingled adenocarcinoma with pleomorphic and spindle-shaped cells was also identified. The second case involved a 54-year-old man with a history of hepatitis B-induced cirrhosis. A 3.5 × 3 × 3 cm mass was found in the middle left of falciform ligament. Microscopically, the tumor consisted of spindle-shaped sarcomatoid carcinoma cells mixed with typical well-differentiated HCC and well-differentiated CC.

Diagnoses: According to the clinicopathological features, diagnosis of cHCC-CC with sarcomatous change was made.

Interventions: In the first case, right lobectomy of the liver was performed. The second patient underwent laparoscopic, hepatic left lateral lobectomy.

Outcomes: The first patient was alive and well 10 years after the surgical resection without additional treatment. In second case, at 8 months after surgical resection, there was no evidence of recurrence or metastasis.

Lessons: In this report, we describe 2 rare cases of cHCC-CC with sarcomatous change, and findings are helpful for the pathologists would like to further identify the clinicopathological features of this rare tumor.

Figure 1

(A–B) The first case, HCC component. (A) Typical, moderately differentiated hepatocellular carcinoma, trabeculae were variable thick, the tumor shows round, oval, inconsistent cells. (HE, 200X×). (B) Tumor cells were negative for S-100. (IHC, 200×). (C–F) The first case, CC component. (C–D) Cholangiocarcinoma component, composed of distorted tubular glands with an abundant fibrous stroma, tumor cells had vesicular nuclei, obvious nucleoli and slightly eosinophilic cytoplasm. (HE, 100×, 400×). (E–F) Tumor cells were positive for CK19, CK7. (IHC, 100×). (G–H, K) The first case, mixed area. (G–H) Sarcomatoid component mixed with cholangiocarcinoma, cholangiocarcinoma cells, and spindle cells were positive for CK19 (IHC, 200×; HE, 200×). (K) CC cells and some spindle cells secrete acid mucopolysaccharides (blue). (AB-PAS, 200×). (I—J) The first case, sarcomatoid component. (I) The sarcomatoid component was composed of short shuttle-like cells with clear nucleoli and few mitotic figures (HE, 200×). (J) Tumor cells were positive for VIM. (IHC, 200×).

Figure 2

(A–C) The second case, HCC component. (A) Compact trabecula was 2 to 3 thickness (HE, 200×). (B–C) tumor cells were positive for Heppar-1 and sinusoid-like blood spaces stained positive for CD34. (IHC, 200×, 100×). (D–F) The second case, pseudoglandular component. (D) Pseudoglandular admixed with the trabecular pattern, the structure is formed by a single layer of cuboidal tumor cells. (HE, 100×). (E–F) Tumor cells were positive for Heppar-1 and negative for CK7. (IHC,100×). (G–K) The second case, CC component. (I, K) Cholangiocarcinoma component consisted of a single layer of tumor cells with vesicular nuclei, obvious nucleoli (HE, 100×, 200×). (G–H, J) Tumor cells were positive for CK19, CK7, and Moc-31 (IHC, 200×). (L–M) The second case, transitional forms. (L) The tumor cells change their morphology from atypia, bizarre shape to typical spindle shape. (HE, 200×). (M) Transition zone cells were positive for CK (IHC, 200X). (N–P) The second case, sarcomatoid component. (N) Extensive spindling of neoplastic cells is obvious, whirls and bundles are irregularly arranged and contain elongated cells (HE, 200×). (O–P) Tumor cells were positive for VIM and negative for CK (IHC, 200×).