Ahmed H, Bari H, Nisar Sheikh U, Basheer MI. Primary hepatic leiomyosarcoma: A case report and literature review . World J Hepatol 2022; 14(9): 1830-1839 [PMID: 36185726 DOI: 10.4254/wjh.v14.i9.1830]
Corresponding Author of This Article
Hassaan Bari, FCPS, MBBS, Surgeon, Department of Surgical Oncology, Shaukat Khanum Memorial Cancer Hospital and Research Centre, 7-A, Khayaban-e-Firdousi, Block R3, M A Johar Town, Lahore 54782, Pakistan. hassaanbari@skm.org.pk
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Case Report
Open-Access Policy of This Article
This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
World J Hepatol. Sep 27, 2022; 14(9): 1830-1839 Published online Sep 27, 2022. doi: 10.4254/wjh.v14.i9.1830
Primary hepatic leiomyosarcoma: A case report and literature review
Hassan Ahmed, Hassaan Bari, Umer Nisar Sheikh, Muhammad Irfan Basheer
Hassan Ahmed, Hassaan Bari, Department of Surgical Oncology, Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore 54782, Pakistan
Umer Nisar Sheikh, Muhammad Irfan Basheer, Department of Pathology, Shaukat Khanum Memorial Cancer Hospital and Research Centre, Lahore 54782, Pakistan
Author contributions: Ahmed H and Bari H were the patient’s surgeons, reviewed the literature, and contributed to manuscript drafting; Sheikh UN and Basheer MI performed the histopathological analyses and interpretation; All authors issued final approval for the version submitted.
Informed consent statement: Informed written consent was obtained from the patient to publish this report and any accompanying images.
Conflict-of-interest statement: The authors have no conflicts of interest to declare.
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).
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Hassaan Bari, FCPS, MBBS, Surgeon, Department of Surgical Oncology, Shaukat Khanum Memorial Cancer Hospital and Research Centre, 7-A, Khayaban-e-Firdousi, Block R3, M A Johar Town, Lahore 54782, Pakistan. hassaanbari@skm.org.pk
Received: June 1, 2022 Peer-review started: June 1, 2022 First decision: June 23, 2022 Revised: July 15, 2022 Accepted: September 13, 2022 Article in press: September 13, 2022 Published online: September 27, 2022
Abstract
BACKGROUND
Primary hepatic leiomyosarcoma (PHL) is a rare tumor with a very low incidence of about 0.2%.
CASE SUMMARY
A 48-year-old diabetic, hypertensive, and morbidly obese female patient presented with a history of abdominal pain and weight loss for 2 mo. She had no history of fever, jaundice, or other liver disease(s). Clinical examination revealed a palpable mass in the epigastrium. Imaging evaluation with a contrast-enhanced computed tomography (CT) scan of the abdomen and pelvis revealed an ill-defined enhancing hyper vascular hepatic mass of 9.9 cm × 7.8 cm occupying the left hepatic lobe with evidence of central necrosis, compression effect on the left hepatic vein, and partial wash-out on delayed images. On further workup, the maximum standardized uptake value on positron emission computed tomography scan was 6.4, which was suggestive of malignancy. The remaining part of the liver was normal without any evidence of cirrhosis. Ultrasound-guided biopsy of the mass showed smooth muscle neoplasm suggestive of leiomyosarcoma. After optimization for co-morbidities, an extended left hepatectomy was planned in a multidisciplinary team meeting. On intraoperative ultrasound, the left hepatic lobe was entirely replaced by a large tumor extending to the caudate lobe with a compression effect on the middle and left hepatic veins. Final histopathology showed nodular and whorled white tumor comprised of spindled/fascicular cells with moderate to severe pleomorphism and focal necrosis. The mitotic index was greater than 20 mitoses per 10 high-power fields. The resection margins were free of tumor. Immunohistochemistry (IHC) depicted a desmin-positive/ caldesmon-negative/discovered on gastrointestinal stromal tumor 1-negative/ cluster of differentiation 117-negative profile, confirming the definitive diagnosis as PHL.
CONCLUSION
This case report highlights the rare malignant mesenchymal hepatic tumor. To confirm PHL diagnosis, one requires peculiar histopathological findings with ancillary IHC confirmation. Management options include adequate/complete surgical resection followed by chemotherapy and/or radiotherapy.
Core Tip: Primary hepatic leiomyosarcoma is an extremely rare tumor among all primary hepatic malignancies, with approximately 70 cases worldwide, including our case. The rare nature of the disease has precluded its underlying pathogenesis. Clinical manifestations are usually nonspecific, and tumors are generally asymptomatic until they significantly increase in size. They have a relatively poor prognosis and aggressive metastatic potential. The preferred type of treatment is surgical excision, which is sometimes combined with adjuvant chemotherapy and radiotherapy; however, little is known about their effectiveness because of the disease rarity. In-depth studies are needed to shed light on this uncommon clinical entity.
