|Year : 2019 | Volume
| Issue : 3 | Page : 241-243
Primary cardiac angiosarcoma with extensive visceral metastases: Utility of 18F-fluorodeoxyglucose positron emission tomography-computed tomography in response assessment to sorafenib
Sarthak Tripathy, Madhavi Tripathi, Girish Kumar Parida, Chandrasekhar Bal, Shamim Ahmed Shamim
Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, India
|Date of Web Publication||20-Jun-2019|
Dr. Shamim Ahmed Shamim
Room No. 4, Department of Nuclear Medicine, AIIMS, Rajkumari Amritkaur OPD, New Delhi - 110 029
Source of Support: None, Conflict of Interest: None
| Abstract|| |
Primary cardiac angiosarcomas although rare neoplasms remain the most common primary malignant neoplasms affecting the heart. We discuss the sequential positron-emission tomography–computed tomography findings of a 50-year-old man who was diagnosed with the metastatic cardiac angiosarcoma at the outset and developed progressive disease despite sorafenib therapy.
Keywords: Cardiac angiosarcoma, metastases, positron-emission tomography–computed tomography, sorafenib
|How to cite this article:|
Tripathy S, Tripathi M, Parida GK, Bal C, Shamim SA. Primary cardiac angiosarcoma with extensive visceral metastases: Utility of 18F-fluorodeoxyglucose positron emission tomography-computed tomography in response assessment to sorafenib. Indian J Nucl Med 2019;34:241-3
|How to cite this URL:|
Tripathy S, Tripathi M, Parida GK, Bal C, Shamim SA. Primary cardiac angiosarcoma with extensive visceral metastases: Utility of 18F-fluorodeoxyglucose positron emission tomography-computed tomography in response assessment to sorafenib. Indian J Nucl Med [serial online] 2019 [cited 2019 Aug 23];34:241-3. Available from: http://www.ijnm.in/text.asp?2019/34/3/241/260748
A 50-year-old man diagnosed with primary cardiac angiosarcoma underwent 18F fluorodeoxyglucose positron-emission tomography–computed tomography (FDG PET–CT) for initial staging and metastatic work-up. The scan findings revealed an enlarged right atrium with increased FDG uptake in the right atrial appendage [Figure 1]a solid black arrow, c and d]. FDG avid metastases were seen in the bilateral lungs [Figure 2]a, adrenals [Figure 2]c, and spleen [Figures 1a dashed-red line and 2c]. He was started on sorafenib therapy and a followup of PET–CT scan was done after 3 months that revealed progressive metastatic disease in bilateral lungs [Figure 1]b and [Figure 2]b, liver [Figure 1]b solid purple arrow and [Figures 2]d], spleen [Figure 1]b solid red arrow and [Figures 2]d], abdominal lymph nodes, and bones [Figure 1]b solid blue arrows and [Figure 2]f]. Angiosarcoma accounts for <1% among sarcomas and primary angiosarcoma of the heart and the great vessels accounts for only 3% of all the angiosarcomas  with an autopsy prevalence of 0.001%–0.28%. Most of these tumors primarily arise in the right atrium, involving the lateral wall of the right atrium and mostly sparing the septum which results in delay in the initial diagnosis. Frequent sites of extra-cardiac metastases include lung, liver, brain, bone, lymph nodes, adrenal glands, spleen, and intestine.,, Our case had metastatic disease involving the lungs, adrenals, spleen, liver, and bones with all of them progressing on follow-up PET–CT scan. Utility of 18-F-FDG PET–CT in initial staging as a part of extra-cardiac metastatic work-up and response assessment to chemo or radiotherapy; although, scarce has been reported in the literature by Hod et al., Tan et al., Jain et al., and Dhull et al., respectively.,,, Due to aggressive and rapidly progressive behavior of the primary and metastatic disease, we advocate extensive use of 18-F-FDG PET-CT as a “one-stop shop” for immaculate assessment of the disease burden during the initial staging and restaging purposes.
|Figure 1: (a) Pretherapy 18F fluorodeoxyglucose positron-emission tomography–computed tomography maximum intensity projection image showing focal increased fluorodeoxyglucose uptake in the thorax region (solid black arrow) suggestive of cardiac angiosarcoma. Dashed-red arrows show increased fluorodeoxyglucose uptake in the spleen suggestive of metastases. (b) Posttherapy 18F fluorodeoxyglucose positron-emission tomography–computed tomography maximum intensity projection image showing increased fluorodeoxyglucose uptake in the liver, spleen, thorax, and pelvis suggestive of metastases. (c) Trans-axial computed tomography image showing soft-tissue lesion in the right atrial appendage (d) Fused trans-axial positron-emission tomography–computed tomography image showing increased fluorodeoxyglucose uptake in the right atrial appendage suggestive of cardiac angiosarcoma|
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|Figure 2: Fused axial pre-therapy positron-emission tomography–computed tomography image showing nodules in the right lung (a) which increased in size and fluorodeoxyglucose uptake in post-therapy scan. (b) Fused axial pre-therapy image (c) showing mildly fluorodeoxyglucose avid hypodense lesion in the spleen which increases in size and fluorodeoxyglucose avidity in post-therapy scan. (d) However, no significant change in Fluorodeoxyglucose avid liver lesion in segment V is seen. No significant fluorodeoxyglucose uptake is seen in the sternum (e) whereas post-therapy scan (f) shows increased fluorodeoxyglucose uptake in the sternum with no cortical changes suggestive of marrow metastases|
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There are no conflicts of interest.
| References|| |
Lantz DA, Dougherty TH, Lucca MJ. Primary angiosarcoma of the heart causing cardiac rupture. Am Heart J 1989;118:186-8.
Barreiro M, Renilla A, Jimenez JM, Martin M, Al Musa T, Garcia L, et al.
Primary cardiac tumors: 32 years of experience from a Spanish tertiary surgical center. Cardiovasc Pathol 2013;22:424-7.
McMannus B. Primary tumors of heart. In: Bonow RO, Mann DL, Zipes DP, Libby P, editors. Braunwald's Heart Disease: A Textbook of Cardiovascular Medicine. 9th
ed. Philadelphia: Elsevier's Saunders; 2012. p. 163850.
Hou CH, Shen DH, Lin LF, Gao HW, Hsu YC, Cheng CY. Aggressive right atrial tumor with extensive FDG-avid metastases in a case of cardiac angiosarcoma. Ann Nucl Med Mol Imaging 2012;25:201-5.
Sabolek M, Bachus-Banaschak K, Bachus R, Arnold G, Storch A. Multiple cerebral aneurysms as delayed complication of left cardiac myxoma: A case report and review. Acta Neurol Scand 2005;111:345-50.
Ramadhan A, Willen H, Thor A. Angiosarcoma of the mandible: Metastasis from a primary tumor of the right atrium of the heart. Case Rep Clin Med 2013;2:537.
Hod N, Shalev A, Levin D, Anconina R, Ezroh Kazap D, Lantsberg S, et al.
FDG PET/CT of cardiac angiosarcoma with pulmonary metastases. Clin Nucl Med 2018;43:744-6.
Tan H, Jiang L, Gao Y, Zeng Z, Shi H. 18F-FDG PET/CT imaging in primary cardiac angiosarcoma: Diagnosis and follow-up. Clin Nucl Med 2013;38:1002-5.
Jain A, Simon S, Elangovan I. (18)F-fluoro-deoxyglucose positron emission tomography-computed tomography in initial assessment and diagnosis of right atrial angiosarcoma with widespread visceral metastases: A rare case report and review of the literature. Indian J Nucl Med 2015;30:51-4.
] [Full text]
Dhull VS, Sharma P, Mukherjee A, Jana M, Bal C, Kumar R, et al.
18F-FDG PET-CT for evaluation of cardiac angiosarcoma: A case report and review of literature. Mol Imaging Radionucl Ther 2015;24:32-6.
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