CARDIOVASCULAR IMAGES
Metastatic Adrenocortical Carcinoma Causing Profound Right Ventricular Outflow Tract Obstruction
An Improvement Following Surgical Resection
A drenocortical carcinoma (ACC) very rarely metastasizes to the right heart; its extension beyond the tricuspid valve is exceptional. We report a complex case of a 59-year-old gentleman with ACC who developed significant right ven- tricular outflow tract (RVOT) obstruction for nearly 10 years following initial diagnosis.
Nitin Chandramohan, BMBS, MRCP Rebecca Rose Davies, BMBS Nada Al-Sakini, MBChB, MRCP, PhD
Our patient was initially referred to an endocrinologist with gynecomastia and significant unintentional weight loss. Computerized tomography (CT) scan showed a 17-centimeter left-sided adrenal mass (Figure 1). The patient underwent a com- bined left nephrectomy and adrenalectomy, and tissue histology was consistent with ACC. Over the next 3 years, there was development of liver metastases and a small 5 mm lung nodule at the left costophrenic angle requiring a hemihepatec- tomy and resection of lung nodule, respectively.
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The following year, an interval CT scan had 2 main findings: first, a mass that was contiguous with the apex of the heart and second, a recurrence of pulmonary nodules. The patient was symptomatic at this time and complained of chest pain and breathlessness on exertion. A transthoracic echocardiogram showed an echo- genic epicardium with a thickened and hypertrophied right ventricle (RV; Figure 2A and 2B). Three months later, repeat transthoracic echocardiogram demonstrated a very large mass measuring 3.3x2.9 cm obstructing a significant part of the RV and extending through pulmonary valve obstructing the RVOT (Figure 3A through 3C; Movie I through III in the Data Supplement). The patient was commenced on a chemotherapy regimen consisting of etoposide, doxorubicin, and cisplatin. Despite this, our patients’ symptoms continued to worsen, and a CT pulmonary angiogram showed an increase in the dimensions of the cardiac metastasis, with 70% occlusion of RVOT and worsening apical mass (Figure 4A through 4C). Our patient was referred for tumor debulking surgery with the aim to reduce symptom load. Intraoperatively, there was a considerable amount of bulky tumor in pulmonary artery protruding through the pulmonary valve but not displacing it. There was also a significant dis- ease relating to the free wall of the right atrium. RV and right atrium metastases were excised, as well a large tumor protruding from the noncoronary sinus to the right atrium. Histology from the myocardium was in keeping with metastatic adreno- cortical carcinoma. Following surgery, our patients’ symptom burden had markedly improved, with reductions in both feelings of breathlessness and chest pain.
Two months postoperatively, transthoracic echocardiogram revealed residual RV mass extending from the apex to the midcavity, also involving the inferior vena cava. Mild concentric thickening of the left ventricle was also noted. There was, however, a significant improvement in the degree of RVOT obstruction (Figure 3D through 3F); this is further evidenced by a reduction in peak pulmonary valve veloc- ity postoperatively (Figure 5A and 5B). A trial of palliative radiotherapy targeting the residual intraventricular mass was started. A follow-up interval CT scan showed the presence of right atrium and RV thrombus for which our patient was initiated
Key Words: adrenalectomy
adrenocortical carcinoma @ etoposide
” hepatectomy ” weight loss
@ 2020 American Heart Association, Inc.
https://www.ahajournals.org/journal/ circimagin
on anticoagulation. Thirteen months postcardiac sur- gery, our patient was admitted to hospital following an episode of collapse. It was thought that he likely had a non-ST-segment-elevation myocardial infarction. A CT scan also demonstrated progression of metastatic dis- ease with new lesions in the liver, brain, and bones. The patient continued to deteriorate and subsequently died. The medical certificate of cause of death indicated met- astatic adrenocortical carcinoma and non-ST-segment- elevation myocardial infarction as the cause of death.
ACC is an aggressive tumor that tends to metasta- size to the liver, lungs, and kidneys. Tumor dissemina- tion occurs in 82% of patients with ACC, but cardiac metastases are rare. Patient wishes should be at the forefront of determining the management of ACC with cardiac metastases, but as it stands, surgical manage- ment is the sole life-prolonging treatment available.1,2 To our knowledge, this is the longest surviving case (13 months) following successful surgical resection of sig- nificant RVOT disease in the context of ACC.1,3,4
ARTICLE INFORMATION
The Data Supplement is available at https://www.ahajournals.org/doi/suppl/ 10.1161/CIRCIMAGING.119.010058.
Correspondence
Nada Al-Sakini, MBChB, MRCP, PhD, Cardiology Registrar, Department of Car- diology, The Royal Cornwall Hospital NHS Trust, Treliske, Truro, Cornwall, TR1 3LQ. Email nadariadh@yahoo.com
Affiliations
Department of Medicine (N.C., R.R.D.) and Department of Cardiology (N.A .- S.), Royal Cornwall Hospital NHS Trust.
Disclosures
None.
REFERENCES
1. Alghulayqah A, Alghasab N, Amin T, Alkahtani N, Farhat R, Alzahrani AS. Long-term recurrence-free survival of adrenocortical cancer extending into the inferior vena cava and right atrium: case report and literature review. Medicine. 2018;96:e6751. doi: 10.1097/MD.0000000000006751
2. Rosen B, Rozenman Y, Harpaz D. Extension of adrenocortical carcinoma into the right atrium - echocardiographic diagnosis: a case report. Cardio- vasc Ultrasound. 2003;1:5. doi: 10.1186/1476-7120-1-5
3. Carlin BW, Dianzumba SB, Wendel C, Joyner CR. Right ventricular inflow and outflow obstruction due to adrenal carcinoma. Catheter Cardio Diag. 1986;12:51-54. doi: 10.1002/ccd. 1810120113
4. Abdelnabi M, Almaghraby A, Saleh Y, Abd ElSamad S. Right atrial and ventricular invasion by adrenal carcinoma: a case report. J Cardiol Cases. 2019;20:42-44. doi: 10.1016/j.jccase.2019.03.010
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