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Background: Review of the first documented case of aortic wall metastasis from a limb sarcoma. Case presentation: In a 56-year-old woman with a diagnosis of a high-grade limb fibrosarcoma, an aortic metas- tasis was revealed by a fast growing aneurysm of the descending thoracic aorta. This was managed with an endoprosthesis. Conclusion: The presence of an aneurysm in a patient with a sarcoma with a high potential for metastasis and poor cardiovascular risk factors should alert physicians. Keywords: Soft tissue sarcoma, Aortic aneurysm, Aortic metastasis Background processes. There are no clear current guidelines regard - Soft-tissue sarcoma is a rare entity (it represents less than ing management of metastatic sarcoma . 1% of malignant tumors ). These tumors have a pro - Typically, metastatic sarcomas are considered incur- pensity for locally destructive growth and recurrence, able and palliative treatment, including chemotherapy, and 20% to almost 100% of them have a significant risk is performed (for performance status = 0–2). Tumors of distant metastasis, depending on their histological spread primarily through the vascular system, and lungs type and grade. One-tenth of soft-tissue sarcomas are are the most common site for distant metastasis. To our already in a metastatic state when discovered . Due to knowledge, an aortic metastasis of a sarcoma has not yet the poor prognosis in the case of metastatic neoplasia , been described as primary intimal sarcomas are most a baseline CT scan is recommended for tumor staging, commonly reported in the literature . In this report, especially for sarcomas measuring more than five cen - we describe a case of aortic metastasis of a limb fibrosar - timeters. Recent studies also suggest that positron emis- coma revealed by a descending thoracic aortic aneurysm, sion tomography (PET) may be clinically indicated to and its management. evaluate biological activity in soft tissue masses , but this technique can lead to false positives due to its low Case report specificity in the case of infectious or active inflammatory A 56-year-old nonsmoking woman consulted for a pain- ful mass in her left thigh. Her past medical history was only significant for untreated autoimmune hepatitis. MRI of her left thigh showed a posterolateral muscu- lar mass measuring five centimeters, with both necrotic *Correspondence: email@example.com Department of Interventional Radiology, Centre Léon Bérard, 28 rue and enhanced portions (Fig. 1a). Histologic analysis per- Laennec, 69008 Lyon, France formed on biopsy samples showed evidence of malignant Full list of author information is available at the end of the article © The Author(s) 2018. This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creat iveco mmons .org/licen ses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. The Creative Commons Public Domain Dedication waiver (http://creat iveco mmons .org/ publi cdoma in/zero/1.0/) applies to the data made available in this article, unless otherwise stated. Delerce et al. Clin Sarcoma Res (2018) 8:22 Page 2 of 5 Fig. 1 a Coronal gadolinium-enhanced T1-weighted image with fat saturation shows a posterolateral muscular mass of the left thigh with both necrotic and enhanced portions (white star); Saccular aneurysm of the descendant thoracic aorta (white head arrows): arterial phase CT scan of the aneurysm with axial view (b), coronal reconstruction (c) and 3D reconstruction (d). e PET-CT image fusion shows FDG uptake (white arrows) around the aneurysm. f Coronal maximum intensity projection (MIP) reconstruction of postoperative CT shows the endoprosthesis (transparent head arrows) and the excluded aneurysmal cavity (transparent arrow) pleomorphic proliferation suggesting a diagnosis of high- for endovascular aortic repair with an endoprosthesis grade fibrosarcoma. However, the results were not totally (Fig. 1f) measuring 28 × 164 mm (Relay NBS Bolton conclusive because of pan-cytokeratin AE1/AE3 expres- Medical). The presence of an atypical epigastric artery sion that could also be found in sarcomatoid carcinomas. angiogram led to an artery biopsy during the procedure, The diagnosis of pleomorphic fibrosarcoma was finally which found no evidence of dysplasia. Despite negative maintained due to the lack of epithelial marker CK7, blood samples taken near the aneurysm, the possibility of CK5/6 and p63. (French Federation of Cancer Centers an infectious location was discussed due to the patient’s Sarcoma Group grading system [FNCLCC] = Differen - poor dental condition. Postoperative probabilistic anti- tiation: 3; Necrosis: 2; mitosis: 10 High-Power Field: 35; biotic treatment was started with oxacillin and ofloxacin, Mitotic Index: 3; Grade: 3). and then amoxicillin. A thoracoabdominal CT scan was performed for Surgical resection of the primary tumor was rejected tumor staging and found a 35-mm saccular aneurysm of due to histological confirmation of a right gluteal metas - the descending thoracic aorta (Fig. 1b–d). The patient, tasis (contralateral). Initially, the patient received con- despite her age, had no cardiovascular risk factors. A ventional chemotherapy by doxorubicin and ifosfamide, PET-CT performed 1 month later showed a suspicious but the treatment was quickly switched to cisplatin and contralateral limb metastasis and abnormal aortic FDG paclitaxel due to tumor progression. The patient was uptake around the aneurysm, which could be attributed then included in a clinical trial (NCT01308034 Study of to an infectious or tumorous process (Fig. 1e). Follow- Continuous Dosing of Sunitinib in Non GIST Sarcomas up CT scans showed quick growth of the aneurysm with Concomitant Radiotherapy) and treatment by suni- from 35 to 49 mm. The patient was then transferred tinib was introduced with concomitant radiotherapy. Due Delerce et al. Clin Sarcoma Res (2018) 8:22 Page 3 of 5 to adverse effects that led to a deep thrombocytopenia Sarcoma survival rates are closely linked to their poten- (50 G/L), sunitinib was stopped. tial metastasis risk. Since 2002, the World Health Organi- Follow up CT scans (Fig. 2a–c) showed the appear- zation (WHO) Classification of Tumors of Soft Tissue ance of a suspicious lesion near the excluded aneurys- and Bone divided these tumors into three risk groups: mal cavity with contrast-enhanced portions (Fig. 3a, b). benign, intermediate (locally aggressive or rarely metas- Aortic MRI and contrast-enhanced ultrasound con- tasizing) and malignant. This classification helps phy - firmed the presence of tumor tissue instead of throm - sicians adapt treatment according to the risk of tumor botic material within the aortic aneurysm sac (Fig. 3c–f ). metastasis. There are no current guidelines regarding A transparietal biopsy of the large mass was performed initial tumor staging, but the WHO clearly recommends using ultrasound guidance by a left posterior paraverte- multidisciplinary decision-making. Sarcomas spread pri- bral approach, and histological examination found pleo- marily through the vascular system, and the lungs are the morphic spindle cells with pan cytokeratin and smooth most common site for distant metastasis. Patients at high muscle actin positivity which confirmed the diagnosis risk of metastasis usually have an initial chest CT scan; a of sarcoma metastasis (FNCLCC grade 3). Palliative thoracoabdominal CT scan may be also performed. management was decided. The patient was included in Sarcoma rarely metastasizes to the skin, soft tissues, another clinical trial (NCT02406781-PEMBROSARC) liver or lymph nodes (3%). Primary aortic sarcomas are and received four injections of pembrolizumab during rare tumors, with approximately 30 cases reported in the a 3-month period, associated with cyclophosphamide. literature . The patient died due to mediastinal tumor progression Typically, patients with aortic intimal sarcomas have 17 months after initial sarcoma diagnosis. symptoms caused by tumor emboli including pulseless extremities or abdominal pain, rather than symptoms Discussion directly related to the primary tumor . In our case, the The luminal-type intimal sarcoma (IS) is the most com - patient was asymptomatic and there was no evidence of mon sarcoma of the aorta . In the early stages, tumor aortic lumen obstruction. aneurysms are very similar to atherosclerotic aneurysms [18F]-2-Fluorodeoxyglucose (FDG) is the most com- on CT findings, which often leads to a delay in diagno - monly used tracer for PET imaging. This is a glucose sis. Tumor etiology must be considered in the case of low analog that accumulates in cells and FDG uptake reflects cardiovascular risk factors, atypical imaging findings or a tissue metabolic activity. In oncology, it is used to detect rapidly evolving aneurysm. In the late stages, multiplanar metastases by virtue of their metabolic differences com - imaging often helps to discriminate tumor tissues from pared to surrounding normal tissue. False positives atherosclerotic materials and shows enhancing lesions commonly occur with active inflammatory or infectious around the aneurysm and tumor extending into the aor- lesions and false negatives occur with low metabolic tic wall or adjacent structures. activity tumors or small lesions (< 1 cm), due to limited Fig. 2 Follow-up CT scans shows rapid progression of the lateral aortic metastasis (white head arrows) near the endoprosthesis (white arrows). a Initial postoperative CT scan shows a non-enhancing lateral aortic lesion which is difficult to differentiate from an excluded aneurysmal cavity. b One month later CT scan shows an important tumor’s size increase with a minimal peripheral enhancement (b). c Six months later, CT scan shows an important tumor progression Delerce et al. Clin Sarcoma Res (2018) 8:22 Page 4 of 5 Fig. 3 CT scan and MRI performed 6 months after endoprosthesis placement show a large lateral aortic tumor mass (white stars) with a progressive contrast enhancement (white arrows). CT scan shows a minimal contrast enhancement on portal phase (a) and an important one on delayed phase (b). Axial non-contrast (c) and gadolinium-enhanced T1-weighted images with fat saturation on arterial phase (d), portal phase (e) and delayed phase (f) show also a progressive tumor enhancement Delerce et al. Clin Sarcoma Res (2018) 8:22 Page 5 of 5 Competing interests resolution. In our case, PET-CT was used in conjunction The authors declare that they have no competing interests. with initial CT scan as a complementary diagnostic tool and showed intense FDG uptake around the aneurysm. Availability of data and materials The datasets used and/or analysed during the current study available from the However, in the case of rapidly evolving atherosclerotic corresponding author on reasonable request. aneurysms, FDG uptake can also be seen. An aortic met- astatic localization was not initially considered to be the Consent for publication Informed consent was obtained from all individual participants included in most likely diagnosis in our case. Follow-up CT scans the study. and MRI findings found enhanced tissues around the aneurysm. Ethics approval and consent to participate All procedures performed in studies involving human participants were in The endovascular treatment of aortic aneurysms is now accordance with the ethical standards of the institutional and/or national the gold standard to avoid the risk of rupture beyond research committee and with the 1964 Helsinki declaration and its later 5 cm. In this case, simple monitoring was impossible in amendments or comparable ethical standards. view of rapid progression, and the multidisciplinary team Funding judged open surgery to be inappropriate and too invasive. Not applicable. To our knowledge, an aortic metastasis of a sarcoma has not yet been reported in the literature. Immunohis- Publisher’s Note tochemical tests are needed to discriminate primary inti- Springer Nature remains neutral with regard to jurisdictional claims in pub- lished maps and institutional affiliations. mal sarcomas from other aortic tumors. Indeed, intimal sarcomas do not express epithelial, smooth muscle, or Received: 5 October 2017 Accepted: 6 October 2018 schwannoma-like markers and are positive for mesen- chymal marker, vimentin. In our case, pan cytokeratin and smooth muscle actin markers were positive, similar to the primitive limb sarcoma. References 1. Hallahan DE, Vogelzang NJ, Borow KM, Bostwick DG, Simon MA. 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Acknowledgements Not applicable.
Clinical Sarcoma Research – Springer Journals
Published: Oct 26, 2018
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