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Copyright: ©Author(s) 2026. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution-NonCommercial (CC BY-NC 4.0) license. No commercial re-use. See permissions. Published by Baishideng Publishing Group Inc.
World J Gastrointest Oncol. Jul 15, 2026; 18(7): 120564
Published online Jul 15, 2026. doi: 10.4251/wjgo.v18.i7.120564
Primary hepatic adenoid cystic carcinoma: A case report and review of literature
Han-Qi Xing, Tao-Ming Mo, Jie He
Han-Qi Xing, Jie He, Department of Radiology, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou 310000, Zhejiang Province, China
Tao-Ming Mo, Department of Pathology, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou 310000, Zhejiang Province, China
Author contributions: Xing HQ contributed to manuscript writing, revising the final manuscript and follow-up of the patient; Mo TM contributed to collection of figures, editing pictures and pathological diagnosis; Xing HQ and He J collected the clinical history and checked and confirmed the authenticity of all the raw data; He J conceived the idea and contributed to supervision. All authors have read and approved the final manuscript.
Informed consent statement: Informed written consent was obtained from the patient for publication of this report and any accompanying images.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
Corresponding author: Jie He, MD, PhD, Department of Radiology, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, No. 3 East Qingchun Road, Hangzhou 310000, Zhejiang Province, China. 3414024@zju.edu.cn
Received: March 2, 2026
Revised: March 31, 2026
Accepted: April 20, 2026
Published online: July 15, 2026
Processing time: 127 Days and 20.5 Hours
Abstract
BACKGROUND

Primary adenoid cystic carcinoma (ACC) of the liver is an exceedingly rare malignancy, with only two previously documented cases in the English-language literature. ACC most commonly arises in the salivary glands, and primary hepatic involvement is highly unusual. The scarcity of reported cases highlights the importance of each new case in advancing understanding of its clinical features and treatment strategies.

CASE SUMMARY

A 59-year-old woman presented with a mass in the right hepatic lobe, which was incidentally detected on contrast-enhanced abdominal computed tomography performed for low back pain. She had no specific symptoms. The lesion had initially been suspected to be a liver abscess at another hospital. She had a history of acute hepatitis C. Imaging studies revealed a cystic-solid mass in segment VII of the liver. Serum tumor marker levels were within normal limits. The diagnosis of ACC was confirmed by liver biopsy, supported by immunohistochemistry. A thorough systemic workup ruled out the possibility of metastatic disease. The patient underwent laparoscopic partial hepatectomy. Postoperative pathological examination confirmed hepatic ACC. Postoperatively, radiotherapy was omitted because the surgical margins were negative; however, given the delayed metastatic potential of ACC, adjuvant chemotherapy with oral capecitabine was administered. At the 7-month follow-up, no evidence of tumor recurrence or metastasis was observed.

CONCLUSION

As the third reported case, this report provides important insights into the diagnosis and management of primary ACC of the liver.

Keywords: Primary adenoid cystic carcinoma; Liver; Diagnosis; Delayed metastasis; Case report

Core Tip: The imaging features were nonspecific and could be mistaken for more common entities, such as a liver abscess. Primary adenoid cystic carcinoma of the liver typically presents with insidious clinical manifestations and lacks specific serological markers, contributing to a high rate of misdiagnosis. This case suggests that, for liver lesions of unknown nature, pathological examination should be considered to establish the diagnosis, even when imaging findings are nonspecific and tumor marker levels are within normal limits. As an aggressive malignancy with the potential for delayed metastasis, primary adenoid cystic carcinoma of the liver warrants increased clinical awareness to facilitate early diagnosis and improve patient prognosis.

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