CARDIOVASCULAR IMAGES
Massive Migration of an Adrenocortical Carcinoma Thrombus
A 41-year-old woman presented with a 4-month history of left flank abdomi- nal pain associated with anorexia, weight loss, hirsutism, peripheral edema, ascites, and a well-delimited petrous mass on palpation of the upper left abdominal quadrant. Abdominal ultrasonography detected a large mass between the left kidney and spleen. Thoracoabdominal magnetic resonance angiography defined a solid 16×12-cm retroperitoneal mass (Figure 1A, asterisk) displacing the left kidney and pancreas, with thrombosis of the left renal vein and inferior vena cava into the right atrium (RA; Figure 1A through 1D, arrows). Elevated plasma levels of dehydroepiandrosterone sulfate (>27.1 umol/L) and testosterone (3.25 ug/L) completed the suspected diagnosis of pure androgen-secreting adrenocorti- cal carcinoma (ACC).
Hassan Kassem, MD Sergio Moral, MD, PhD Manel Morales, MD Esther Ballesteros, MD Ramon Brugada, MD, PhD
Combined surgery guided by transesophageal echocardiography was proposed to achieve tumor and thrombus excision. During surgical intervention preparation, the patient presented with sudden systemic arterial hypotension together with quick desaturation. Transesophageal echocardiography showed the rapid progres- sion of the thrombus into the right heart chambers, leading to a total occupation and cardiac arrest (Figure 1E; Movies I and II in the Data Supplement). Despite advanced cardiopulmonary resuscitation maneuvers, the patient died. Postmortem histopathologic study confirmed the diagnosis of ACC and the tumoral nature of the thrombus.
ACCs are rare, aggressive carcinomas with an annual incidence of 0.5 to 2 cases per million.1 Hormone secretion has been described in 76% of adults with ACC, but only 5% are pure androgen-secreting tumors and usually adenomas rather than carcinomas.2 Complete surgical resection remains the most effec- tive treatment and, along with an early staging, is among the strongest predic- tors of overall survival.3 Thrombus extension into the inferior vena cava is also infrequent but about 26% of thrombi reach the RA.3 Right-sided ACC will more typically extend to the RA because of the direct course of the right adrenal vein to the inferior vena cava. Nevertheless, left-sided ACC, as in our case, can also reach the right heart cavities through thrombosis of the left renal vein; this should be included in the differential diagnosis of right-sided cardiac mass cause (Figure 2).
The main clinical manifestations of inferior vena cava and RA extension of this tumor include peripheral edema, ascites, and hepatomegaly. Forensic studies have attributed some sudden deaths in ACC to massive migrations obstructing the right ventricular inflow,4 but this mechanism has not been confirmed because of the difficulty of establishing the temporal sequence between the event and the thrombosis of the right heart cavities. As our case demonstrates, massive thrombus migration in ACC can be fatal. Thus, when a thrombus reaches the RA in ACC, due to its large size, it must be considered not only an extratu-
Key Words: adrenocortical carcinoma kidney magnetic resonance angiography testosterone
thrombosis
@ 2019 American Heart Association, Inc.
https://www.ahajournals.org/journal/ circimaging
moral extension but also a high-risk sign of a poten- tially fatal complication that necessitates rapid surgical intervention.
ARTICLE INFORMATION
The Data Supplement is available at https://www.ahajournals.org/doi/suppl/ 10.1161/CIRCIMAGING.119.008855.
Correspondence
Sergio Moral, MD, PHD, Cardiology Department, Hospital Universitari Doctor Josep Trueta, Avenida França, S/N, 17007, Girona, Spain. Email moral.sergio@ yahoo.es
Affiliations
Cardiology Department, Hospital Universitari Doctor Josep Trueta, CIBERCV, Gi- rona, Spain (H.K., S.M., M.M., R.B.). Radiology Department, Centre d’Atenció Primaria Pare Claret, Institut Català de la Salut, Barcelona, Spain (E.B.).
Disclosures
None.
REFERENCES
1. Kerkhofs TM, Verhoeven RH, Van der Zwan JM, Dieleman J, Kerstens MN, Links TP, Van de Poll-Franse LV, Haak HR. Adrenocortical carcinoma: a population-based study on incidence and survival in the Netherlands since 1993. Eur J Cancer. 2013;49:2579-2586. doi: 10.1016/j.ejca.2013.02.034
2. Abiven G, Coste J, Groussin L, Anract P, Tissier F, Legmann P, Dousset B, Bertagna X, Bertherat J. Clinical and biological features in the prognosis of adrenocortical cancer: poor outcome of cortisol-secreting tumors in a se- ries of 202 consecutive patients. J Clin Endocrinol Metab. 2006;91:2650- 2655. doi: 10.1210/jc.2005-2730
3. Chiche L, Dousset B, Kieffer E, Chapuis Y. Adrenocortical carcinoma extending into the inferior vena cava: presentation of a 15-patient series and review of the literature. Surgery. 2006;139:15-27. doi: 10.1016/j.surg.2005.05.014
4. Dickens P, Poon CS, Wat MS. Sudden death associated with solitary intracavitary right atrial metastatic tumour deposit. Forensic Sci Int. 1992;57:169-173.
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A, Right-sided adrenocortical carcinoma (ACC) reaching the right atrium (RA) through thrombosis of the right adrenal vein (RAV; black arrow) to the inferior vena cava (IVC; representing =80% of described cases). B, Left-sided ACC reaching the RA from the thrombosis of IVC: note the longer distance that the tumoral thrombus must travel before reaching the RA through thrombosis of the left adrenal vein (LAV; white arrow), left renal vein, and IVC. RV indicates right ventricle; and SVC, superior vena cava.
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