Figure 1
Native pre-biopsy abdominal CT scan
Abdominal CT scan. Intrahepatic tumour mass (arrow).
A rare case of primary liver leiomyosarcoma
SectionAbdominal imaging
Case TypeClinical Cases
AuthorsMikhail Cherkashin, Ekaterina Sokolova, Mikhail Anishkin, Denis Puchkov, Natalia Berezina
Connected authors
39 years, male
Clinical History
A 39-year-old man presented with weight loss and abdominal pain for the past 8 months. Abdominal CT was performed in May 2017 and showed a hypodense liver mass (12x10x7 cm) in left lobe and segment IV. Surgical direct biopsy was performed and revealed carcinoma. Chemotherapy was initiated.
Imaging Findings
CT (June 17) showed a large heterogeneous hepatic tumour (15x16x15 cm), without noticeable enhancement or great vessels invasion (Figure 1 - arterial phase, Figure 2 - venous). PET-CT showed the liver focus (SUV 12.6), but no distant lesions. Based on clinical and radiology data and disease history (local progression despite conventional chemotherapy), gastrointestinal stromal tumour was suspected. For pathology verification CT-guided core needle biopsy (16G) was performed (Figures 3 and 4). Pathology (27.06.2017): polymorphic cell tumour, immunohistological examination Vim (+), SMA (+), Caldesmon (+), S100 (-/+), Melan A (-), PCK (-), HMB-45 (-), CD 34 (-), CD 117 (-).
Discussion
Background:
Hepatic leiomyosarcoma is an extremely rare tumour, with non-specific presentations, laboratory studies, or images; it stands for only 0.2–2% of primary hepatic malignant tumours [1, 2].
Leiomyosarcoma is an aggressive tumour. Response to treatment with chemotherapy is poor. The main treatment is surgical removal with neoadjuvant and/or adjuvant chemotherapy. Whenever resection is impossible, radiotherapy is an option for local control [3].
Imaging perspective:
Radiology findings in case of liver sarcomas are non-specific, however, they are usually very large at presentation. Contrast enhancement is poor, different from that of hepatocellular carcinoma, but somehow similar to many secondary deposits. Therefore pathological confirmation is mandatory, including immune-staining, as demonstrated by the failure of surgical biopsy in this patient.
Outcome:
Current time gemcitabine and 5-fluoruracil is continuing and the next steps will include treatment response radiology assessment and stereotactic radiation therapy for palliative local control.
Learning points:
Primary hepatic leiomyosarcoma is an extremely rare aggressive tumour. Imaging presentation is non-specific. Image-guided core needle biopsy is mandatory, and should include immunostaining.
Hepatic leiomyosarcoma is an extremely rare tumour, with non-specific presentations, laboratory studies, or images; it stands for only 0.2–2% of primary hepatic malignant tumours [1, 2].
Leiomyosarcoma is an aggressive tumour. Response to treatment with chemotherapy is poor. The main treatment is surgical removal with neoadjuvant and/or adjuvant chemotherapy. Whenever resection is impossible, radiotherapy is an option for local control [3].
Imaging perspective:
Radiology findings in case of liver sarcomas are non-specific, however, they are usually very large at presentation. Contrast enhancement is poor, different from that of hepatocellular carcinoma, but somehow similar to many secondary deposits. Therefore pathological confirmation is mandatory, including immune-staining, as demonstrated by the failure of surgical biopsy in this patient.
Outcome:
Current time gemcitabine and 5-fluoruracil is continuing and the next steps will include treatment response radiology assessment and stereotactic radiation therapy for palliative local control.
Learning points:
Primary hepatic leiomyosarcoma is an extremely rare aggressive tumour. Imaging presentation is non-specific. Image-guided core needle biopsy is mandatory, and should include immunostaining.
Differential Diagnosis List
Primary liver leiomyosarcoma
Hepatocellular carcinoma
Gastrointestinal stromal tumour
Liver leiomyosarcoma
Final Diagnosis
Primary liver leiomyosarcoma
References
[1] Carriaga MT, Henson DE. (1995) Liver, gallbladder, extrahepatic bile ducts, and pancreas. Cancer 75:171–190 (PMID: 8000995)
[2] Iida T, Maeda T, Amari Y, Yurugi T, Tsukamoto Y, Nakajima F. (2017) Primary hepatic leiomyosarcoma in a patient with autosomal dominant polycystic kidney disease. CEN Case Rep May;6(1):74-78. (PMID: 28509136)
[3] Bae S, Crowe P, Gowda R, Joubert W, Carey-Smith R, Stalley P, Desai J. (2016) Patterns of care for patients with advanced soft tissue sarcoma: experience from Australian sarcoma services. Clin Sarcoma Res Jul 11;6:11 (PMID: 27403280)
Case information
| URL: | https://eurorad.org/case/14947 |
| DOI: | 10.1594/EURORAD/CASE.14947 |
| ISSN: | 1563-4086 |
License
This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.
Figure 2
CT-guided core needle biopsy
CT-guided core needle biopsy. Coaxial 16 G needle placed into the tumour (arrow).
Figure 3
Diagnostic contrast-enhanced CT. Arterial phase
Arterial phase. Large vessels are not revealed within the tumour mass.
Figure 4
Diagnostic contrast-enhanced CT. Venous phase
Venous phase. Strong enhancing and slow contrast outflow.
Native pre-biopsy abdominal CT scan
Abdominal CT scan. Intrahepatic tumour mass (arrow).
Cherkashin M. Medical institute n.a Berezin Sergey, Saint-Petersburg, Russia
Cherkashin M. Medical institute n.a Berezin Sergey, Saint-Petersburg, Russia
CT-guided core needle biopsy
CT-guided core needle biopsy. Coaxial 16 G needle placed into the tumour (arrow).
Cherkashin M. Medical institute n.a Berezin Sergey, Saint-Petersburg, Russia
Cherkashin M. Medical institute n.a Berezin Sergey, Saint-Petersburg, Russia
Diagnostic contrast-enhanced CT. Arterial phase
Arterial phase. Large vessels are not revealed within the tumour mass.
Cherkashin M., MIBS, SPb, Russia
Cherkashin M., MIBS, SPb, Russia
Diagnostic contrast-enhanced CT. Venous phase
Venous phase. Strong enhancing and slow contrast outflow.
Cherkashin M., MIBS, SPb, Russia
Cherkashin M., MIBS, SPb, Russia