Congenital hepatic cyst with intracystic hemorrhage: Two rare cases reports in the Chinese Han population

Abstract

Introduction: Fast-growing congenital hepatic cysts with intracystic hemorrhage are rare in clinical practice. Additionally, the clinical manifestations of and laboratory and imaging findings for this condition are often nonspecific and are particularly difficult to differentiate from those of hepatobiliary cystadenoma and cystadenocarcinoma, thus posing great challenges for diagnosis and treatment. The 2 case reports presented here aim to analyze the diagnosis and treatment of 2 rare cases of congenital hepatic cysts with intracystic hemorrhage in the Chinese Han population to provide an important reference for the clinical diagnosis and treatment of this condition.

Diagnoses: These 2 case reports present 2 rare cases of congenital hepatic cysts with intracystic hemorrhage. Case 1 involved a 31-year-old patient with a very large, fast-growing hepatic cyst with intracystic hemorrhage and elevated carbohydrate antigen 199. Case 2 involved a patient with intense, paroxysmal right upper abdominal pain; computed tomography suggested a hepatic cyst with intracystic hemorrhage and possibly hepatobiliary cystadenoma.

Outcomes: Both patients underwent liver resection. Postoperative follow-up showed that for both patients, the symptoms improved, the laboratory findings returned to normal levels, and the surgical outcomes were satisfactory.

Conclusion: Liver resection is an ideal treatment for patients with congenital hepatic cysts with intracystic hemorrhage, and especially those with fast-growing, symptomatic hepatic cysts or hepatic cysts that are difficult to differentiate from hepatobiliary cystadenoma and cystadenocarcinoma.

Figure 1

Imaging examination. (A, B) CT showed 2 masses (approximately 13.2 × 19.9 cm and 8.3 × 10.4 cm in size) of slightly low density. Septa were visible in the larger cyst, with enhancement of the cystic wall and septa and no significant enhancement inside the cyst. (C, D) MRCP showed dilation of the left intrahepatic bile ducts (possibly related to compression of the hilar bile ducts) and unclear extrahepatic bile ducts. (E) MRA showed compression and displacement of the hilar vessels, clear images of the trunk and branches of the portal vein, and compression and displacement of the hepatic veins. (F) CTA showed compression and displacement of the hilar vessels, clear images of the trunk and branches of the portal vein, a clear right hepatic vein (compressed and displaced), and an unclear left hepatic vein. (G, H, I, J) CT measurement showed that the overall liver volume was 4590 mL, and the FLV was 1030 mL: the FLV of the right liver was 455 mL, and the FLV of the left liver was 575 mL. CT = computed tomography, CTA = computed tomographic arteriography, FLV = future liver volume, MRA = magnetic resonance angiography.

Figure 2

Intraoperative exploration and gross examination of the surgical specimen. (A, B) Intraoperative exploration showed a huge cystic mass of approximately 25 × 20 cm in size in the middle lobe, extending down to the pelvis and adhering to the gallbladder and the first porta hepatis. Moreover, another cystic mass, approximately 10 × 10 cm in size, was present in the lower right lobe immediately adjacent to the huge cystic mass. (C, D, E) Gross examination of the surgical specimen showed that the specimen was 22.3 × 16 × 8.4 cm in size; the cystic cavity was 20 × 11 cm in size (as observed based on the specimen surface and sections); the cavity was filled with brown turbid liquid; the interior cystic wall was rough and 0.2 to 0.7 cm thick, with a small amount of bleeding in part of the wall; and no apparent cirrhosis was observed in the remaining liver tissue.

Figure 3

Histopathological examination. (A, B) Microscopic examination showed that the cystic wall was covered with a single layer of squamous epithelial cells, with hyperplasia of the fibrous tissue (especially elastic fibers) beneath the epithelial cells, infiltration of a small number of inflammatory cells (hematoxylin and eosin stain: A, magnification × 100; B, magnification × 40). (C, D) Microscopic examination showed that the cystic wall was covered with a single layer of squamous epithelial cells, with hyperplasia of the fibrous tissue underneath the epithelial cells, infiltration of inflammatory cells. (Hematoxylin and eosin stain: C, magnification × 100; D, magnification × 40).

Figure 4

Imaging examination and postoperative specimen. (A, B) CT showed a right-lobe mass of approximately 11 × 8 cm in size. Enhanced CT showed a cystic mass at the edge of the liver parenchyma, with uneven enhancement during the arterial phase. The density of the lesion was lower than that of the surrounding normal liver parenchyma during the portal and delayed phases. (C, D) MRCP showed a mass with high T1 and T2 signals, approximately 10 × 13 cm in size, with multiple nodules on the wall in the right anterior lobe. CT = computed tomography, MRCP = magnetic resonance cholangiopancreatography.