Hepatic Sclerosing Hemangioma Simulating Gallbladder Carcinoma: A Rare Case

Abstract

Sclerosing hemangiomas of the liver are rare benign tumors of the liver. Although hepatic hemangiomas are the most common benign liver tumor, they are mostly cavernous in nature. A hepatic sclerosing hemangioma is defined by presence of fibrosis and hyalinization as a result of degenerative changes in a cavernous hemangioma. The radiological features of sclerosing hepatic hemangioma can resemble those of cholangiocarcinoma, fibrolamellar carcinoma, or metastasis. We present a case of a hepatic sclerosing hemangioma in which an unusual magnetic resonance imaging (MRI) and computed tomography (CT) appearance lead to radiographic concern for gallbladder carcinoma. To the best of our knowledge, this is the first case in the literature of a hepatic sclerosing hemangioma mimicking a gallbladder carcinoma.

We present an interesting case of a sclerosing hemangioma of the liver, which clinically and radiologically mimicked gallbladder carcinoma. A 69-year-old female, with a history of intermittent abdominal pain over the past year, was found to have elevated Carbohydrate antigen 19-9 (CA 19-9) of 70.4 U/ml (normal range 0–35 U/ml) on routine blood work. Computed tomography (CT) imaging was performed and revealed a mild enhancing soft tissue mass of size approximately 3.2 × 1.8 cm with punctate calcification along the lateral wall of the gallbladder having minimal intraluminal extension (Figure 1). Further workup included a magnetic resonance imaging (MRI) that confirmed a mild T2 hyperintense exophyic hepatic mass of size 3.6 × 3.0 cm along the anteroinferior aspect of the gallbladder, with minimal enhancement on postcontrast image (Figure 2). Both CT and MRI showed a noncirrhotic liver with a few simple cysts and no evidence of metastatic disease.

Figure 1.

CECT whole abdomen. (A) Axial, (B) sagittal, and (C) coronal images show a mild enhancing soft tissue mass of size approximately 3.2 × 1.8 cm, with punctate calcification along the lateral wall of the gallbladder having minimal intraluminal extension (white arrows). CECT, contrast-enhanced computed tomography.

Figure 2.

MRI abdomen. (A) Axial T2, (B) coronal T2, (C) sagittal T2, and (D) coronal T1 postcontrast images demonstrate a mild T2 hyperintense exophytic hepatic mass of size 3.6 × 3 cm, along the anteroinferior aspect of the gallbladder (white arrows), which shows minimal enhancement on post contrast image (orange arrow). MRI, magnetic resonance imaging.

She underwent a diagnostic laparoscopy with open partial liver resection and open cholecystectomy. The partial hepatectomy specimen consisted of segments 4 and 5 was sent for intraoperative frozen consultation. Gross examination revealed a mass of size 4.0 × 3.5 × 1.5 cm based in the liver. A portion of the specimen was evaluated in the frozen section, which microscopically revealed a benign lesion containing admixed areas of fibrosis and blood-filled spaces. On further gross examination by serial sectioning of the specimen, it was found that the mass was composed of heterogeneous areas of dense sclerosis and blood-filled spongy and cystic components. Histologically, the lesion consisted of variably sized, thin-walled vascular spaces, lined by benign endothelial cells. Many of the vascular spaces were obliterated by fibrous tissue, consistent with sclerosing hemangioma. The sclerosing hemangioma pushed and attenuated the surrounding hepatocytes (Figure 3). No further surgical resection was performed. The gallbladder showed chronic cholecystitis. The gallbladder wall was uninvolved by sclerosing hemangioma.

Figure 3.

Histopathology sclerosing hemangioma. Low power images (25×). (A) (B) Fibrosis and hyalinization surrounded by variably sized blood vessels and areas of calcification. (C) A large bile duct (black arrow) is seen at the periphery of the lesion composed of variably sized vascular spaces. (D) Attenuated hepatocytes (black arrow) are seen at the periphery of the lesion.

Discussion

Cavernous hemangiomas are the most common benign tumor of the liver. Although most of the cavernous hemangiomas are discovered incidentally, those that are greater than 4 cm in size are likely to be brought to clinical attention due to symptoms associated with the lesion. Grossly, hemangiomas are typically spongy, red to brown in color. Microscopically, they are characterized by multiple clusters of blood-filled cavities, lined by a single layer of benign endothelial cells. The walls of the blood-filled spaces show more fibrous tissue rather than organized layer of muscle or elastic tissue.¹ Hepatic sclerosing hemangiomas are rare lesions, believed to result from the degeneration of cavernous hemangiomas secondary to thrombosis, necrosis, sclerosis, and infarction, among other regressive changes. They are mostly solitary and show admixture of variably sized cavernous spaces and stromal fibrosis. The fibrosis tends to appear in the center of the lesion surrounded by patent vessels in the periphery in most of the cases. Some tumors can demonstrate recent or old thrombus. Grossly, sclerosing hemangiomas show variable areas of dark red hemorrhagic parenchyma and gray white areas corresponding to fibrosis. In extreme cases in which extensive sclerosis obliterates the vasculature, the term sclerosed hemangioma is used. They are firm and tan white on gross appearance.² Sclerosing hemangiomas are more common in women than in men. The median age of diagnosis is 65 years.³

CT and MRI are becoming increasingly used in the evaluation of patients with abdominal symptoms.⁴ MRI has a high sensitivity and specificity in diagnosing hemangiomas. Typically, hemangiomas are hypoattenuating lesions, with nodular peripheral enhancement in the arterial phase and central enhancement in the delayed phase.⁵ The characteristic appearance is due to sluggish blood flowing through the vascular spaces.⁶ However, in sclerosing hemangioma of the liver, the presence of sclerosis and degeneration gives an unusual appearance on radiology which could be confused with primary lesions such as hepatocellular carcinoma, cholangiocarcinoma, and even metastasis.⁷ There are overlapping radiographic features between sclerosing and cavernous hemangiomas. There are some radiographic features of sclerosing hemangiomas, however, that can help in leading to its diagnosis. This includes having a typical geographic pattern, demonstrating capsular retraction, the presence of transient hepatic attenuation difference in the arterial phase, rim enhancement, and nodular regions of enhancement. There are no pathognomonic features, however, to definitively diagnose sclerosing hemangiomas on radiology. MRI features of sclerosing hemangiomas based on reports in the literature include hypointense signal on T1-weighted images and intermediate-to-high signal on T2-weighted images. Areas of hypointense T2 signal were found to correspond to histologic findings of sclerosis. Nodular enhancement was noted on postcontrast images. CT features include an irregular low attenuated mass with patchy irregular enhancement and punctate calcifications.^{7, 8}

In our case, radiology and laboratory findings supported a diagnosis of gallbladder malignancy. On imaging, the soft tissue mass appeared to be arising from the gallbladder wall and appeared exophytic to the liver parenchyma. Thus, at the time of interpretation, the findings mimicked those of a gallbladder malignancy. However, in reality, the mass was based in the liver, without any involvement of the gallbladder wall. Also, the CA19-9 in this case was elevated at 70.4 U/ml, further raising the possibility of a malignancy.

CA19-9 is a tumor-associated antigen and a useful additional test in evaluation of patients with pancreaticobiliary malignancies. However, CA19-9 has a varying sensitivity (50%–90%) and specificity (54%–98%)⁹ and is often falsely elevated in benign diseases such as gallbladder stone disease, chronic pancreatitis, hepatitis, liver cirrhosis, renal failure, duodenal ulcer, gastric polyps, colonic polyps, and renal cyst.¹⁰

Hemangiomas are benign, mostly asymptomatic and found incidentally, whereas gallbladder carcinomas are typically lethal tumors and the fifth most common malignancy of the gastrointestinal tract in the United States.¹¹ Precise preoperative imaging evaluation is of great importance in planning patient management and surgical extent. In cases of gallbladder carcinoma, common patterns on imaging includes focal or diffuse gallbladder wall thickening, a mass replacing the gallbladder or a polypoid mass.^{12, 13} Radiological evaluation may be difficult, and many conditions may be mistaken for gallbladder carcinomas. Some of the most common mimickers of gallbladder carcinoma are xanthogranulomatous cholecystitis, adenomyomatosis, polyps, adenomas, and metastatic disease. Our case is the first case of sclerosing hemangioma mimicking gallbladder carcinoma described in the literature and illustrates the importance of including sclerosing hemangiomas in the differential diagnosis of gallbladder lesions as patient management is totally different. Fortunately, in our case, no further radical resection was required after the frozen section evaluation.

Conflicts of interest

All authors have no conflicts of interest to declare.