Multiple sclerosing hemangiomas mimicking hepatocellular carcinoma: A case report

Abstract

Hepatocellular carcinoma is a prevalent malignant tumor affecting the liver, and surgical resection and liver transplantation are the primary treatment options for early-stage HCC patients. However, the presence of benign hepatic tumors with similar imaging characteristics to HCC poses challenges in diagnosing and treating the disease, often resulting in misdiagnosis and inappropriate treatment. This case report presents a 52-year-old female patient who exhibited space-occupying liver lesions on abdominal CT and MRI scans. Based on pathological sections from other hospitals, liver malignancy was highly suspected, and hepatocellular tumor was diagnosed preoperatively. But the tumor markers of the patient were all within the normal range. After evaluating the overall condition of the patient, we finally chose the diagnosis and treatment of dissection and partial hepatectomy. Surprisingly, the final diagnosis of postoperative pathology was sclerosing hemangioma. The patient recovered well and was discharged 2 weeks later. Hepatic sclerosing hemangioma is an extremely rare disease that can be easily mistaken for malignant liver tumors due to absence of typical imaging presentations. The diagnosis also needs to be differentiated from other benign tumors, such as liver adenoma and liver abscess, according to the medical history, symptoms, and auxiliary examinations. Therefore, special attention should be given to the diagnosis and treatment of sclerosing hemangioma.

Introduction

Hepatic sclerosing hemangioma is an infrequent benign condition, with sclerosis believed to be a potential complication of common liver hemangiomas. ¹ While typical hemangiomas can be reliably identified by using ultrasound, CT, and MRI, sclerosing hemangiomas exhibit a variety of other degenerative changes that often obscure the underlying nature of the disease. ² Patients with sclerosing hemangioma frequently undergo partial hepatectomy as the imaging presentation is often misinterpreted as primary hepatocellular carcinoma. Pathological findings after surgery are necessary to establish an accurate diagnosis.^1,3 Further consideration should be given to the standardization of diagnostic and treatment approaches for sclerosing hemangioma, aiming to prevent unnecessary invasive surgical procedures for patients with this condition.

Case presentation

A middle-aged woman aged 52 was found to have multiple occupying lesions in the liver on CT scan during her hernia repair surgery at an outside hospital. The patient was referred to our hospital with pathological tissue sections from an outside liver puncture. On admission, the patient experienced mild abdominal discomfort symptoms, primarily abdominal distension. Laboratory tests on admission indicated significantly elevated levels of alanine transaminase: 361.8 U/L, aspartate aminotransferase: 475.4 U/L, alkaline phosphatase: 127 U/L, and gamma-glutamyl transpeptidase: 52.8 U/L. Conversely, bilirubin indicators and tumor markers including methemoglobin, carcinoembryonic antigen, glycoantigen 19-9, and hepatitis B virus DNA results fell within the normal range. CT scans revealed hypodense foci in the right lobe of the liver measuring approximately 9.7*8.6 cm, accompanied by poorly defined hypodense foci in the left inner lobe of the liver. MRI findings suggested the presence of three occupying lesions in the liver, with the largest mass measuring approximately 89*74 mm. The mass exhibited a predominantly mixed signal shadow on long T1-long T2 sequences along with multiple segregated enhancing shadows at the edge and inside. Significant enhancement was observed during the arterial phase and continuous enhancement during the venous and delayed phases. Other imaging observations included an 11 mm nodule in the s4 segment of the liver with delayed enhancement and a tiny round long T2 signal without significant enhancement (Figure 1). Based on the above imaging examinations, a preliminary diagnosis of neoplastic lesion (sarcoma), hepatocellular carcinoma, intrahepatic metastasis of hepatocellular carcinoma or atypical hemangioma was made. Liver biopsy revealed a large area of coagulated necrotic tissue surrounded by vascular granulation tissue proliferation, inflammatory cell infiltration, and iron-containing hemoglobin deposition. Exploratory laparotomy was performed, revealing multiple intrahepatic occupancies. After the resection of the liver mass, the excised specimen was a solid, soft, circular mass with a complete capsule from the right lobe of the liver, measuring 10*6.5*6.5 cm. The cut surface was grayish-white, partially grayish-yellow. Additionally, a grayish-white nodule with a diameter of 0.4 cm was identified in the inner left lobe of the liver (Figure 2). Moreover, another grayish-white nodule measuring 2.5*2.5*1 cm was located beneath the falciform ligament of the liver. Intraoperative rapid pathology indicated a spindle cell lesion with interstitial small vessel hyperplasia in the right posterior lobe of the liver, fibrous tissue hyperplasia with chronic inflammatory cell infiltration in the left inner lobe of the liver, and a tumor of vascular origin in the mass below the sickle ligament. Postoperative conventional pathology suggested vascular-derived lesions but failed to confirm the disease definitively. Therefore, immunohistochemical staining was performed on the sections, revealing positive CD31, CD34, and ERG staining in all three lesions, leading to the final diagnosis of sclerosing hemangiomas. The patient’s postoperative recovery was uneventful, and she was discharged on the 11th day after surgery.

Figure 1.

An abdominal magnetic resonance imaging scan of a 52-year-old woman showed multiple space-occupying lesions in the liver. The irregular mass in the right lobe of the liver showed multiple separate enhancement shadows in the interior and margin of the mass, with continuous enhancement of the arteries, veins, and delayed phase.

Figure 2.

The gross manifestation of a large mass in the right lobe of liver was 10*6.5*6.5 cm, the capsular was complete, and multiple gray and white areas were visible on the tumor section.

Discussion

Hemangiomas are the most prevalent benign liver tumors, with large anatomical studies estimating an incidence rate ranging from 0.7% to 7% in the general population. Ultrasound examinations effectively identify hemangiomas smaller than 1.5 cm displaying strong echogenic masses that differentiate them from primary and metastatic hepatocellular carcinoma. However, larger hemangiomas are challenging to visualize using ultrasound and often necessitate the utilization of CT and the more sensitive MRI for accurate identification.^2,4 Although CT and MRI exhibit high sensitivity in detecting cavernous sinusoidal hemangiomas, their sensitivity for sclerosing hemangiomas is significantly lower, with some microscopic lesions evading CT visualization. Hepatic sclerosing hemangioma represents a rare benign tumor characterized by degenerative changes, including hemorrhage, iron-containing heme deposits, interstitial myofibrillar hyperplasia, thrombosis, necrosis, and scar formation. ⁵ The presence of these degenerative changes results in diverse imaging manifestations of sclerosing hemangioma. Previous case reports have indicated that most patients with this condition exhibit ring enhancement on CT scans, while MRI images lack specificity, often leading to misdiagnosis as liver malignancies, such as metastases, hepatocellular carcinoma, and cholangiocarcinoma, during preoperative assessments (Table1).^5–13 Notably, serological indicators and tumor markers typically fall within the normal range for most patients with sclerosing hemangioma. Hence, clinicians must exercise caution when diagnosing cases with ambiguous imaging features and normal tumor-related indexes, considering the inclusion of rare benign liver tumors into the identification of liver malignant tumors, such as hepatic sclerosing hemangioma, in the differential diagnosis of liver malignancies. More importantly, different diagnostic results determine the appropriate interventions. The patient in this case report was preoperatively diagnosed with hepatocellular carcinoma, which is the most prevalent primary liver cancer. HCC has the highest incidence in East Asia and Africa. ¹⁴ With the increasing incidence of hepatocellular carcinoma, early diagnosis and treatment are of paramount importance. Surgical resection is one of the most critical treatment modalities for early-stage patients, while hepatectomy is the preferred approach for hepatocellular carcinoma patients without cirrhosis. ¹⁵ On the other hand, smaller asymptomatic cirrhotic hemangiomas can be managed conservatively for an extended period, and surgical intervention is only necessary when the tumor reaches a certain size. ⁸ Therefore, it is essential to be cautious when diagnosing rare diseases to ensure accurate diagnosis, which can spare patients from unnecessary harm and guide appropriate treatment measures. In cases where the imaging diagnosis is inconclusive and malignancy is suspected, liver aspiration biopsy retains its diagnostic significance when contraindications to puncture are mostly excluded. The final decision on whether to proceed with surgery should be made by considering the biopsy results, characteristics of the mass (such as size and location), and the physical-psychological impact on the patient.

Table 1.

Literature review of sclerosing hemangioma cases in the liver.

Year	Author(s)	Age/Sex	Size (mm)	Presentation(s)	Tumor marker	Serological diagnosis	CT	MRI(T1/T2)	Preoperative diagnosis	Operation	Postoperative pathological diagnosis
2000	Yamashita, Y et al 6	67 F	50	Fatigue and emaciation	Normal	Hb(4.9 g/dl)	Low-density liver mass	High/high	Metastasis	Partial hepatectomy	HSH
2011	Yong Moon Shin 7	50 M	100	Emaciation	Normal	Normal	Patchy irregular enhancement	/High	HSH	Needle biopsy	SCH
2013	Song, Ji Soo et al 8	63 F	90*71/30*21	Abdominal pain	Normal	Normal	Patchy irregular enhancement	N	HSH/SCH	Partial hepatectomy	HSH
2015	Miyamoto, Shunsuke et al 9	76 M	59	Upper abdominal pain	Normal	Normal	RE	RE/high	ICC	Laparoscopic	HSH
2015	Wakasugi, Masaki et al 10	67 F	28	Hematochezia	Normal	N	RE	High/	Metastasis	Laparoscopic	HSH
2016	Behbahani, Siavash et al 5	70 M	N	Weakness and emaciation	N	Glu 722 mg/dL	RE	N	Metastasis	Needle biopsy	HSH
2019	Xu, Li et al 11	65 M	45	No	Normal	Normal	N	N	SCH	RF ablation	HSH
2018	Kyohei Yugawa et al 1	48 M	67*53	No	Normal	ALT/AST/ALP/GGT↑	Delayed	Low/high	HCC	Partial hepatectomy	HSH
2021	Jia, Cuiyu et al 13	63 M	N	No	CA19-9 ↑	Normal	Enhancement	Enhancement	ICC	Needle biopsy	HSH
2021	Jia, Cuiyu et al 13	56 F	N	No	N	N	RE	RE	Probably malignancy	Needle biopsy	HSH

F = female, M = male, N = not mentioned, CA19-9 = carbohydrate antigen 199, RE = ring enhancement, HSH = Hepatic sclerosed hemangioma, SCH = Sclerosing cavernous hemangioma, ICC = Intrahepatic cholangiocarcinoma.

Conclusion

Accurate preoperative diagnosis is essential for the treatment of liver tumors. Some rare benign diseases, such as sclerosing hemangioma, which have confusing imaging features, still cannot be ignored in the differential diagnosis of malignant liver masses. For the diagnosis of such rare diseases, the help of pathological examinations is indispensable, and a comprehensive analytical consideration of the disease can help us in the final characterization.