
==== Front
Radiol Case Rep
Radiol Case Rep
Radiology Case Reports
1930-0433
Elsevier

S1930-0433(24)00693-9
10.1016/j.radcr.2024.07.107
Case Report
Primary hepatic leiomyosarcoma with adrenal and hepatic metastasis: Case report and literature review
Faraj Chaymae MD faraj.chaymae@gmail.com
a⁎
Mahdi Youssef PhD b
Essetti Sara MD a
Chait Fatima MD a
Essaber Hatim MD a
El Bakkari Asaad MD a
Omor Youssef PhD a
Latib Rachida PhD a
Amalik Sanae PhD a
El Khannoussi Basma PhD b
a Radiology department, National Institute of Oncology, Faculty of Medicine and Pharmacy, Mohammed V University, Rabat, Morocco
b Pathology department, National Institute of Oncology, Faculty of Medicine and Pharmacy, Mohammed V University, Rabat, Morocco
⁎ Corresponding author. faraj.chaymae@gmail.com
17 8 2024
11 2024
17 8 2024
19 11 49504954
16 7 2024
17 7 2024
18 7 2024
© 2024 The Authors. Published by Elsevier Inc. on behalf of University of Washington.
2024

https://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Primary hepatic leiomyosarcoma (PHL) is a rare malignant tumor, which originates from smooth muscles. The imaging features are nonspecific and the diagnosis is often delayed until the tumor reaches a large size, which leads often to a dismal prognosis. We report a case of a 46-year-old male patient who was complaining about abdominal pain for 2 months. The imaging revealed the presence of a large mass in the liver with adrenal and liver metastasis. Diagnosis of PHL was confirmed by histopathological and immunohistochemical examinations. In this case report, we review the epidemiological, clinical, and paraclinical aspects of the disease, as well as the treatment modalities.

keywords

Leiomyosarcoma
Liver
Metastasis
==== Body
pmcIntroduction

Primary hepatic leiomyosarcoma (PHL) is an uncommon mesenchymal hepatic tumor which originates from smooth muscles. clinical manifestations are often nonspecific and the patient remains asymptomatic until there is a significant increase in tumor size causing a mass effect.

These tumors are extremely rare, which has limited our understanding of them and consequently left the standard of treatment unclear. Herein, we report a case of a bulky primary hepatic leiomyosarcoma in a 68-year-old female patient.

Case report

A 46 years old male patient, with no medical history, had been a chronic smoker for 28 years and had weaned himself off alcohol 1 year ago. The history of the disease dates back 2 months, with the onset of abdominal pain with no other associated signs. The patient was put on symptomatic treatment but, given the persistence of the pain, an ultrasound was performed which revealed the presence of a hepatic mass with a right adrenal nodule.

An abdominal MRI revealed a voluminous hepatic mass in segments II, III and IV, measuring 91 × 80 × 110 mm. On T1-weighted imaging (WI), the mass was well defined and showed slightly a heterogeneous hypointensity, and heterogeneous hyperintensity on T2WI. On diffusion-WI, the mass displayed hyperintensity. In the arterial phase of dynamic contrast-enhanced imaging, the mass showed no evident enhancement and in the 3-min delayed imaging, it showed a heterogeneous marked enhancement (Fig. 1). Imaging also found a right adrenal nodule (Fig. 1) and a nodular hepatic lesion in segment VIII (Fig. 2), suggesting secondary localization. A biopsy of the hepatic mass was performed, and the histological and immunohistochemical study were in favor of an hepatic localization of a leiomyosarcoma (Fig. 3, Fig. 4).Fig. 1 MRI findings of primary liver leiomyosarcoma. (A) On T1-weighted imaging (WI), the mass (red arrow) is well defined and shows slightly a heterogeneous hypointensity. (B) On T2WI, the mass (red arrow) is heterogeneously hyperintense. (C and D) On diffusion-WI, the mass (red arrow) display hyperintensity. (E) In the arterial phase of dynamic contrast-enhanced imaging, the mass (red arrow) shows no evident enhancement. (F) In the 3-min delayed imaging, the mass (white arrow) shows a heterogeneous marked enhancement. The adrenal gland (green arrow) metastasis is also enhanced.

Fig 1:

Fig. 2 MRI findings showing an intrahepatic metastasis (blue arrows) hyperintense on T2 weighted images (A), DWI (B) and enhanced on contrast enhanced imaging (C).

Fig 2:

Fig. 3 Representative micrographs of the tumor: Tumor is composed of atypical spindle cells (A). Tumor cells have an enlarged hyperchromatic nuclei and eosinophilic cytoplasm (B). (Hematoxylin-eosin; A : x100, B : x200).

Fig 3:

Fig. 4 Immunohistochemical profile of the lesion : Tumor cells are positive for smooth muscle actin (A) and h-caldesmon (B). They are negative for AE1/AE3 cytokeratines (C) and CD 34 (with blood vessels as positive control) (D).

Fig 4:

Since the patient had hepatic and adrenal metastasis, surgery was ruled out, the patient was put on chemotherapy.

Discussion

Primary hepatic leiomyosarcomas (PHL) include 6%-16% of the primary hepatic sarcomas which in turn represent 0.2%-2% of primary hepatic cancers [1]. Leiomyosarcoma potentially originates from the smooth muscle cells in the round ligament, intrahepatic blood vessels, and bile ducts [2].

Among the cases described to date, including our patient, there is no evident sex predisposition with an approximate male-to-female ratio of 1:1 in the literature review. Age ranges from 5 months to 86 years old (mean age of 51.3 years) [2].

The underlying pathogenetic mechanisms have not been identified yet. Nonetheless, there have been isolated instances of immunosuppressed patients developing primary hepatic leiomyosarcoma, with two of those cases including acquired immunodeficiency syndrome. A patient diagnosed with acquired immunodeficiency syndrome additionally had an Epstein-Barr virus infection.

Another patient with primary hepatic leiomyosarcoma was under immunosuppressive treatment after renal transplantation. Additionally, 2 patients with chronic hepatitis have been reported to have primary hepatic leiomyosarcoma: one who had hepatitis B, and another patient with hepatitis C [3].

PHL doesn't have any particular clinical signs, and tumors usually don't cause any symptoms until they increase in size. Common symptoms include nausea, vomiting, jaundice, and abdominal pain; physical examination often reveals hepatomegaly and a palpable mass [4]. One infrequent symptom seen in PHL patients is acute bleeding secondary to tumor rupture [5]. Serological indicators, including α-fetoprotein, are normal, but the findings of liver function tests may be abnormal. Diagnosis is frequently delayed due to nonspecific clinical symptoms and the lack of serological markers [4].

Ultrasonography usually shows hypoechoic or heterogeneous echogenic mass [3].

Computed tomography usually reveals a hypodense and often heterogeneous mass with inhomogeneous and often peripheral enhancement after administration of intravenous contrast, which may show regions of cystic degeneration [3,6,7].

Magnetic resonance imaging characteristically displays homogeneous or heterogeneous hypointense T1-weighted images and hyperintense T2-weighted images [8,9], with occasional observation of encapsulation [7]. In the patient reported by LV and al, the tumor was large and displayed hypointensity on T1-weighted SE images and hyperintensity on T2 weighted SE images. Furthermore, in the dynamic contrast-enhanced MRI, the masse was not evidently enhanced during the arterial and portal venous phases. However, the masse was markedly enhanced during the 5-min delayed imaging [4] . Those similar features were observed in our case.

Histological examination of primary hepatic leiomyosarcomas shows spindle-shaped cells with fascicular arrangement. Immunohistochemistry reveals expression of smooth muscle actin, desmin and vimentin, whereas ytokeratins, neuron-specific enolase and S-100 protein are not expressed [3].

Hepatocellular carcinoma and cholangiocarcinoma are the main differential diagnoses. Indeed, tumor biopsy is the only mean to achieve formal diagnosis of PHL [1].

PHL has aggressive metastatic potential and is usually diagnosed in situations of locally advanced or metastatic disease. Therapeutic options vary depending upon the tumor size and/or stage on initial presentation [2]. For nonmetastatic cases, such as ours, hepatic resection (wedge resection, segmentectomy, lobectomy, or prolonged hepatectomy with the goal of R0 resection) remains the only potentially curative treatment [10].

Adjuvant chemotherapy, according to some authors, included a variety of medications, such as ifosfamide and doxorubicin, which contribute to a longer life time following total resection. Furthermore, as part of a combined adjuvant treatment with chemotherapy, 3 cases have received radiotherapy treatment [11].

In certain cases, transarterial chemoembolization and transarterial infusion of carboplatin and epirubicin were also documented as PHL therapy modalities [12].

Conclusion

In conclusion, PHL is a rare malignant disease which diagnosis is challenging and often delayed until reaching a large size, resulting in extremely poor prognosis. The preferred treatment type is surgical resection, sometimes in combination with adjuvant chemotherapy and/or radiotherapy.

Guarantor of submission

The corresponding author is the guarantor of submission.

Patient consent

Written informed consent for the publication of this case report was obtained from the patient.

Acknowledgments: Not applicable.

Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
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References

1 Esposito F Lim C Baranes L Salloum C Feray C Calderaro J Primary leiomyosarcoma of the liver: two new cases and a systematic review Ann Hepatobiliary Pancreat Surg 24 1 2020 63 67 32181431
2 Ahmed H Bari H Nisar Sheikh U Basheer MI Primary hepatic leiomyosarcoma: a case report and literature review World J Hepatol 14 9 2022 1830 1839 36185726
3 Feretis T Kostakis ID Damaskos C Garmpis N Mantas D Nonni A Primary hepatic leiomyosarcoma: a case report and review of the literature Acta Medica (Hradec Kralove) 61 4 2018 153 157 30664449
4 Lv WF Han JK Cheng DL Tang WJ Lu D. Imaging features of primary hepatic leiomyosarcoma: a case report and review of literature Oncol Lett 9 5 2015 2256 2260 26137052
5 Jeong TY Kim YS Park KJ Lee JS Huh JG Ryu SH [A case of primary leiomyosarcoma of the liver presenting with acute bleeding] Korean J Gastroenterol 51 3 2008 194 198 18451694
6 Ferrozzi F Bova D Zangrandi A Garlaschi G. Primary liver leiomyosarcoma: CT appearance Abdom Imaging 21 2 1996 157 160 8661764
7 Yu RS Chen Y Jiang B Wang LH Xu XF. Primary hepatic sarcomas: CT findings Eur Radiol 18 10 2008 2196 2205 18463872
8 Soyer P Blanc F Vissuzaine C Marmuse JP Menu Y. Primary leiomyosarcoma of the liver MR findings Clin Imaging 20 4 1996 273 275 8959367
9 Shivathirthan N Kita J Iso Y Hachiya H Kyunghwa P Sawada T Primary hepatic leiomyosarcoma: case report and literature review World J Gastrointest Oncol 3 10 2011 148 152 22046492
10 Maeda T Yugawa K Kinjo N Imai D Sanefuji K Kawata K Large surgically resected leiomyosarcoma of the liver: a case report Surg Case Rep 6 1 2020 168 32648231
11 Almogy G Lieberman S Gips M Pappo O Edden Y Jurim O Clinical outcomes of surgical resections for primary liver sarcoma in adults: results from a single centre Eur J Surg Oncol 30 4 2004 421 427 15063896
12 Zhu KL Cai XJ. Primary hepatic leiomyosarcoma successfully treated by transcatheter arterial chemoembolization: a case report World J Clin Cases 7 4 2019 525 531 30842965
