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Metastatic adrenocortical carcinoma of the interventricular septum

Yunqi Zhu, MD, PhD,ª Xiaoya Lu, MD, PhD,b,c Lili Lin, MD,ª Tingting Zhang, MD,ª Donghe Chen, MD,ª Kui Zhao, MD,ª and Xinhui Su, MD, PhDª

a PET Center, Department of Nuclear Medicine, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou , Zhejiang, China

Department of Clinical Laboratory, Zhejiang Provincial People’s Hospital, People’s Hospital of Hangzhou Medical College, Hangzhou , Zhejiang, China

” Key Laboratory of Tumor Molecular Diagnosis and Individualized Medicine of Zhejiang Province, Zhejiang Provincial People’s Hospital, People’s Hospital of Hangzhou Medical College, Hangzhou , Zhejiang, China

Received Jul 2, 2022; accepted Jul 2, 2022 doi:10.1007/s12350-022-03072-8

A 52-year-old woman presented with shortness of breath after exertion for 3 months. She had a history of a left adrenalectomy due to adrenal adenoma 9 years ago. Transthoracic echocardiogram revealed a large mass in the mid-basal portion of interventricular septum (IVS) causing severe narrowing of the right ventricular outflow tract (RVOT, Figure 1). Contrast-enhanced cardiac CT demonstrated the presence of a solid mass in the mid- basal portion of IVS (Figure 2), confirming findings observed on echocardiography. Positron emission tomography/computed tomography (PET/CT) demon- strated intense 18F-fluorodeoxyglucose (18F-FDG) accumulation of the mass (Figure 3A through C), sug- gestive of malignancy. In addition, 18F-FDG PET/CT revealed multiple bilateral pulmonary nodules with mildly increased 18F-FDG accumulation (Figure 3D and E), suggestive of pulmonary metastases.

Percutaneous interventricular tumor biopsy and palliative radiofrequency ablation was performed.

Immunohistochemical analysis of the biopsy showed positive staining of Inhibin-a, Melan-A, and Syn, with a high proliferation index (Ki-67) of 20%, leading to the diagnosis of metastatic adrenocortical carcinoma (ACC).

Adrenocortical carcinoma is a rare malignant neo- plasm with an annual incidence of 0.7 to 2 cases per million per year.1 The most common metastatic sites of ACC are the liver, lungs, and lymph nodes. A number of cases with inferior vena cava and right atrial involve- ment have been reported, which is considered as direct tumor invasion of the inferior vena cava and extension into right atrium rather than distant metastasis.2 Myocardial metastasis of ACC is extremely rare, with only a single prior reported case.3 This case highlights that whole-body FDG PET/CT is a valuable imaging modality for investigation and detection of primary tumor, local invasion, and distant metastases in cases of ACC.

Reprint requests: Xinhui Su, MD, PHD, PET Center, Department of Nuclear Medicine, The First Affiliated Hospital, Zhejiang University School of Medicine, Hangzhou 310003, Zhejiang, China; suxinhui@zju.edu.cn

J Nucl Cardiol 2023;30:2191-3. 1071-3581/$34.00

Copyright @ 2022 The Author(s) under exclusive licence to American Society of Nuclear Cardiology

Figure 1. Transthoracic echocardiogram images demonstrating the interventricular septum (IVS) mass. The parasternal long axis apical view (A) and four-chamber view (B) showing a large mass in the IVS (asterisk, 57 x 51 x 50 mm3). Blood flow passed right ventricular outflow tract (RVOT) at the velocity of 4.17 m/s (C). Mild tricuspid regurgitation was also detected in the four-chamber view (D).

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Figure 2. Contrast-enhanced cardiac CT demonstrating a large mass (asterisk) in the IVS with significant RVOT obstruction (arrows).

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Figure 3. Maximum intensity projection PET (A) and fused 18F-FDG PET/CT images showing increased 18F-FDG uptake of the IVS mass (B and C, asterisk, SUVmax of 13.1). Multiple bilateral pulmonary nodules with mildly increased 18F-FDG accumulation were also observed (D and E, arrows, SUVmax of 1.6).

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Disclosures

Authors declare that there is no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the Ethical Standards of the Institutional and National Research Committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Informed consent was obtained from all individual participants included in the study.

References

1. Lam AK. Adrenocortical carcinoma: Updates of clinical and pathological features after renewed World Health Organisation Classification and pathology staging. Biomedicines 2021;9:175.

2. 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 (Baltimore) 2017;96:e6751.

3. Xie X, Zhou Y, Wu B, Guo X. Percutaneous transmyocardial ablation of a metastatic adrenocortical carcinoma invading the interventricular septum. Eur Heart J 2021;42:2023.

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