Abstract
We report the case of a 59-year-old female with progressive bilateral painful swelling of the thighs. MRI revealed multiple intramuscular necrotic masses with similar morphologic patterns. Whole-body CT and 18-FDG PET-CT scans demonstrated additional hypermetabolic muscular masses and a lobulated lesion within the left atrial cavity. As biopsy of a muscular mass was compatible with a poorly differentiated sarcoma with MDM2 oncogene amplification, two diagnoses were discussed: a dedifferentiated liposarcoma with muscle and heart metastases or a primary cardiac sarcoma, mainly a cardiac intimal sarcoma, with muscular metastases, which was finally confirmed by array-comparative genomic hybridization (aCGH) in a sarcoma reference center. This case emphasizes the potential for intimal sarcoma to disseminate in skeletal muscle prior to any other organ and the need for a genomic approach in addition to classical radiopathologic analyses to distinguish primary from secondary locations facing simultaneous tumors of the heart and skeletal muscles with MDM2 amplification.
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References
Neuville A, Collin F, Bruneval P, Parrens M, Thivolet F, Gomez-Brouchet A, et al. Intimal sarcoma is the most frequent primary cardiac sarcoma: clinicopathologic and molecular retrospective analysis of 100 primary cardiac sarcomas. Am J Surg Pathol. 2014;38(4):461–9.
Sparrow PJ, Kurian JB, Jones TR, Sivananthan MU. MR imaging of cardiac tumors. Radiographics. 2005;25(5):1255–76.
Butany J, Nair V, Naseemuddin A, Yau T, et al. Cardiac tumours: diagnosis and management. Lancet Oncol. 2005;6(4):219–28.
Butany J, Leong SW, Carmichael K, Komeda M. A 30-year analysis of cardiac neoplasms at autopsy. Can J Cardiol. 2005;21(8):675–80.
Mayer F, Aebert H, Hartmann JT, et al. Primary malignant sarcomas of the heart and great vessels in adult patients—a single-center experience. Oncologist. 2007;12(9):1134–42.
Agaimy A, Rösch J, Weyand M, Strecker T. Primary and metastatic cardiac sarcomas: a 12-year experience at a German heart center. Int J Clin Exp Pathol. 2012;5(9):928–38.
Rashid A, Molloy S, Butt S, et al. Metastatic pulmonary intimal sarcoma presenting as cauda equina syndrome: first report of a case. Spine (Phila Pa 1976). 2008;33(15):E516–20.
Shimizu H, Tanibuchi A, Yozu R, et al. Stroke due to undifferentiated aortic intimal sarcoma with disseminated metastatic lesions. Circulation. 2009;120(25):e290–2.
Yamashiro K, Funabe S, Hattori N, et al. Primary aortic sarcoma: a rare but critical cause of stroke. Neurology. 2015;84(7):755–6.
Hoiczyk M, Iliodromitis K, Erbel R, et al. Intimal sarcoma of the pulmonary artery with unusual findings: a case report. Clin Res Cardiol. 2012;101:397–401.
Binh MB, Sastre-Garau X, Coindre JM, et al. MDM2 and CDK4 immunostainings are useful adjuncts in diagnosing well-differentiated and dedifferentiated liposarcoma subtypes: a comparative analysis of 559 soft tissue neoplasms with genetic data. Am J Surg Pathol. 2005;29(10):1340–7.
Coindre J-M, Pédeutour F, Aurias A. Well-differentiated and dedifferentiated liposarcomas. Virchows Arch. 2010;456(2):167–79.
Molina JE, Edwards JE, Ward HB. Primary cardiac tumors: experience at the University of Minnesota. Thorac Cardiovasc Surg. 1990;38(Suppl 2):183–91.
Burke AP, Cowan D, Virmani R. Primary sarcomas of the heart. Cancer. 1992;69(2):387–95.
Simpson L, Kumar SK, Okuno SH, Schaff HV, Porrata LF, Buckner JC, et al. Malignant primary cardiac tumors: review of a single institution experience. Cancer. 2008;112(11):2440–6.
Glockner JF, White LM, Sundaram M, McDonald DJ. Unsuspected metastases presenting as solitary soft tissue lesions: a fourteen-year review. Skelet Radiol. 2000;29(5):270–4.
Surov A, Hainz M, Holzhausen H-J, Arnold D, Katzer M, Schmidt J, et al. Skeletal muscle metastases: primary tumours, prevalence, and radiological features. Eur Radiol. 2010;20(3):649–58.
Djaldetti M, Sredni B, Zigelman R, Verber M, Fishman P. Muscle cells produce a low molecular weight factor with anti-cancer activity. Clin Exp Metastasis. 1996;14(3):189–96.
Kurek JB, Nouri S, Kannourakis G, Murphy M, Austin L. Leukemia inhibitory factor and interleukin-6 are produced by diseased and regenerating skeletal muscle. Muscle Nerve. 1996;19(10):1291–301.
Bar-Yehuda S, Barer F, Volfsson L, Fishman P. Resistance of muscle to tumor metastases: a role for a3 adenosine receptor agonists. Neoplasia N Y N. 2001;3(2):125–31.
Weiss L. Biomechanical destruction of cancer cells in skeletal muscle: a rate-regulator for hematogenous metastasis. Clin Exp Metastasis. 1989;7(5):483–91.
Pretorius ES, Fishman EK. Helical CT of skeletal muscle metastases from primary carcinomas. Am J Roentgenol. 2000;174(2):401–4.
Ote EL, Oriuchi N, Endo K, et al. Pulmonary artery intimal sarcoma: the role of 18F-fluorodeoxyglucose positron emission tomography in monitoring response to treatment. Jpn J Radiol. 2011;29(4):279–82.
Lee DH, Jung TE, Choi JH et al. Pulmonary artery intimal sarcoma: poor 18F–fluorodeoxyglucose uptake in positron emission computed tomography. J Cardiothorac Surg. 2013:7;8:40.
Acknowledgements
The authors would like to thank Agnès Ribeiro, Pr. Jean-Michel Coindre and Dr. Frederic Chibon for their contribution and expertise concerning the pathologic analysis as well as Mrs. Estelle Ternamian for medical writing services.
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Drs. Crombé and Lintingre jointly directed this work.
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Crombé, A., Lintingre, PF., Le Loarer, F. et al. Multiple skeletal muscle metastases revealing a cardiac intimal sarcoma. Skeletal Radiol 47, 125–130 (2018). https://doi.org/10.1007/s00256-017-2768-5
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DOI: https://doi.org/10.1007/s00256-017-2768-5