Case of primary hepatic leiomyosarcoma successfully treated with laparoscopic right hepatectomy

Vella, Sandro; Cortis, Kelvin; Pisani, David; Pocock, James; Aldrighetti, Luca

doi:10.1136/bcr-2019-233567

Cited by 6 publications

(4 citation statements)

References 51 publications

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“…Hepatic leiomyosarcomas are generally metastatic, arising from the GI tract, uterus, retroperitoneum, or lungs, requiring careful staging[ 2 ]. To the best of our knowledge, only about 70 cases, including case series, have been published internationally, including our patient (Tables 1 and 2 )[ 1 ].…”

Section: Discussionmentioning

confidence: 99%

“…Sarcomas from the liver are rare, constituting only 1%-2% of all primary hepatic malignant tumors[ 1 , 2 ]. Leiomyosarcoma accounts for 8%-10% of all hepatic sarcomas, whereas other hepatic sarcomas including angiosarcoma, fibrosarcoma, liposarcoma, embryonal sarcoma, malignant fibrous histiocytoma, carcinosarcoma, and epithelioid hemangioendothelioma collectively comprise nearly 70%-80% of hepatic sarcomas[ 3 ].…”

Section: Discussionmentioning

confidence: 99%

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Primary hepatic leiomyosarcoma: A case report and literature review

Hassan¹,

Bari²,

Sheikh³

et al. 2022

World J Hepatol

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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.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Primary hepatic leiomyosarcoma: A case report and literature review

Hassan¹,

Bari²,

Sheikh³

et al. 2022

World J Hepatol

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“…Laparoscopic right hepatectomy and transcatheter arterial chemoembolisation (TACE) are reported to be successful in the early stages, although more studies are needed. [6][7][8][9] Some of these measures also consider residual liver function, tumour margins, and vascularity. Liver transplantation, chemotherapy, and radiotherapy benefits for PHL remain unclear.…”

Section: Discussionmentioning

confidence: 99%

A rare primary liver tumour case report: 53-year-old man with primary hepatic leiomyosarcoma

Pramana

Kusnanto

Darmayani

et al. 2022

JKKI

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Primary hepatic leiomyosarcoma (PHL) is a very rare primary liver tumour. These tumours usually arise from intrahepatic vascular structures, gallbladder, or ligamentum teres. The pathogenesis of this disease is still unknown. We report a 53-year-old man with complaints of intermittent right upper abdominal pain for one month, decreased appetite, nausea, weight loss, and tea-coloured urination. The patient denied any complaints of defecation pattern. On physical examination, jaundice was present in both eyes and the whole-body skin, tenderness in the right hypochondriacal region, and enlarged liver with a lumpy surface. The laboratory examination demonstrated increased transaminase enzymes and bilirubin, while hepatitis B and C were negative. The patient underwent several tests for tumour markers, such as CA 19-9 50 (<37 U/mL), AFP 1.23 (<5.81 IU/mL), and CEA 0.83 (<3 ng/mL). Ultrasound demonstrated an enlarged liver, multiple liver nodules, and cholelithiasis. Meanwhile, the abdomen’s computerised tomography (CT) scan shows a solid heterogeneity image with an irregular border in segments 1 and 4b and a tumoral thrombus in the inferior vena cava. The diagnosis was confirmed by biopsy and immunohistochemistry (IHC); vimentin and smooth muscle actin (SMA) results were positive, while CD 34, CD 117, and cytokeratin were negative. Our patient was diagnosed with PHL stage IVa (T4N2M0). PHL is a particularly rare tumour with a poor prognosis. The patient died after one month of diagnosis. Diagnosis of PHL is challenging. It was based on clinical features, physical examinations, laboratory examinations, and other supporting investigations.

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“…1 Demografische, tumorspezifische Eigenschaften und Therapie der "häufigeren" primären Lebersarkome anhand der Literaturrecherche. Leiomyosarkom [190,191,192,193,194,195,196,197,198,199,200,201…”

Section: Kaposi-sarkomunclassified