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Image focus
IMAGE FOCUS
doi:10.1093/ehjci/jeu223 Online publish-ahead-of-print 11 November 2014
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Extracavitary cardiac carcinoid presenting with right ventricular outflow tract obstruction Hesham R. Omar1*, Devanand Mangar2, Tony Fattouch3, Julie D. Gibbs4, and Enrico M. Camporesi5 1 Internal Medicine Department, Mercy Medical Center, 1410 N. Fourth Street, Clinton, IA 52732, USA; 2Tampa General Hospital and FGTBACEO and Regional Medical Director, TEAMHealth, Tampa, FL, USA; 3Cardiothoracic Imaging Department, University of South Florida, Tampa, FL, USA; 4Pathology Department, University of South Florida, Tampa, FL, USA; and 5University of South Florida and TEAMHealth, Tampa, Florida, USA
* Corresponding author. Tel: +1 3127149272, Email: [email protected]
Conflict of interest: none declared. Published on behalf of the European Society of Cardiology. All rights reserved. & The Author 2014. For permissions please email: [email protected].
Downloaded from http://ehjcimaging.oxfordjournals.org/ at The University of British Colombia Library on October 4, 2015
A 67-year-old male with known metastatic carcinoid to the liver diagnosed 3 years earlier treated with somatostatin and hepatic artery embolization started developing dyspnoea and obstructive cardiac symptoms, and workup was suggestive of cardiac carcinoid. Transoesophageal echocardiogram (TEE) revealed a 6 × 4 cm subpulmonic mass within the free wall of the right ventricle (RV) encroaching on the RV outflow tract (RVOT) without any valvular involvement (Panel D). Cardiac magnetic resonance (CMR) showed a mass centred in the epicardial fat and RV free wall extending towards the RVOT region (Panels A–C). External beam radiation therapy was given without reduction in the size of the mass; in addition, the patient developed episodes of ventricular tachycardia (VT), so he was referred for surgical excision. After median sternotomy, and cardiopulmonary bypass, the tumour was visible on the free wall of the RV without extension through the endocardial surface. It was possible to separate the tumour with sharp dissection and circumferentially resect it (Panels H and I) followed by patch repair of the right ventriculotomy site. Surgical pathology revealed synaptophysin and chromogranin positivity (Panels F and G) confirming the diagnosis of metastatic carcinoid. Postoperative TEE showed no evidence of residual tumour. At follow-up 3 months later, the patient cardiac symptoms improved and no further episodes of VT were recorded. The case is unique because of the myocardial involvement by metastatic carcinoid disease causing RVOT obstruction in the absence of the usual valvular or endocardial affection. (Panels A–I) CMR short-axis view (Panel A) and three-chamber view (Panel B) T2 images showing a smooth, solid, hyperintense mass centred on the right ventricular (RV) wall and encroaching on the RV outflow track. (Panel C) illustrates CMR T1 fat saturation image, showing that the mass is hyperintense indicating the lack of fat. (Panel D) is a TEE of the RV and pulmonary valve showing the mass is progressively invading RV. (Panel E) H&E stain showing that the margin is free of tumour, which appears as monotonous tumour cells arranged in a trabecular or ‘ribbon-like’ pattern with numerous luminal rosettes and has finely stippled ‘salt and pepper’ nuclear chromatin without nucleoli and low mitotic rate (low grade). (Panels F and G) Tumour cells positive for synaptophysin and chromogranin cytoplasmic staining. The Ki-67 (not shown) demonstrated a mild-to-moderate proliferation index. (Panel H) A direct view showing the mass completely dissectible from the outer wall of the RV. (Panel I) The excised 6 × 4 cm tan brown mass.
Image focus
IMAGE FOCUS
doi:10.1093/ehjci/jeu223 Online publish-ahead-of-print 11 November 2014
.............................................................................................................................................................................
Extracavitary cardiac carcinoid presenting with right ventricular outflow tract obstruction Hesham R. Omar1*, Devanand Mangar2, Tony Fattouch3, Julie D. Gibbs4, and Enrico M. Camporesi5 1 Internal Medicine Department, Mercy Medical Center, 1410 N. Fourth Street, Clinton, IA 52732, USA; 2Tampa General Hospital and FGTBACEO and Regional Medical Director, TEAMHealth, Tampa, FL, USA; 3Cardiothoracic Imaging Department, University of South Florida, Tampa, FL, USA; 4Pathology Department, University of South Florida, Tampa, FL, USA; and 5University of South Florida and TEAMHealth, Tampa, Florida, USA
* Corresponding author. Tel: +1 3127149272, Email: [email protected]
Conflict of interest: none declared. Published on behalf of the European Society of Cardiology. All rights reserved. & The Author 2014. For permissions please email: [email protected].
Downloaded from http://ehjcimaging.oxfordjournals.org/ at The University of British Colombia Library on October 4, 2015
A 67-year-old male with known metastatic carcinoid to the liver diagnosed 3 years earlier treated with somatostatin and hepatic artery embolization started developing dyspnoea and obstructive cardiac symptoms, and workup was suggestive of cardiac carcinoid. Transoesophageal echocardiogram (TEE) revealed a 6 × 4 cm subpulmonic mass within the free wall of the right ventricle (RV) encroaching on the RV outflow tract (RVOT) without any valvular involvement (Panel D). Cardiac magnetic resonance (CMR) showed a mass centred in the epicardial fat and RV free wall extending towards the RVOT region (Panels A–C). External beam radiation therapy was given without reduction in the size of the mass; in addition, the patient developed episodes of ventricular tachycardia (VT), so he was referred for surgical excision. After median sternotomy, and cardiopulmonary bypass, the tumour was visible on the free wall of the RV without extension through the endocardial surface. It was possible to separate the tumour with sharp dissection and circumferentially resect it (Panels H and I) followed by patch repair of the right ventriculotomy site. Surgical pathology revealed synaptophysin and chromogranin positivity (Panels F and G) confirming the diagnosis of metastatic carcinoid. Postoperative TEE showed no evidence of residual tumour. At follow-up 3 months later, the patient cardiac symptoms improved and no further episodes of VT were recorded. The case is unique because of the myocardial involvement by metastatic carcinoid disease causing RVOT obstruction in the absence of the usual valvular or endocardial affection. (Panels A–I) CMR short-axis view (Panel A) and three-chamber view (Panel B) T2 images showing a smooth, solid, hyperintense mass centred on the right ventricular (RV) wall and encroaching on the RV outflow track. (Panel C) illustrates CMR T1 fat saturation image, showing that the mass is hyperintense indicating the lack of fat. (Panel D) is a TEE of the RV and pulmonary valve showing the mass is progressively invading RV. (Panel E) H&E stain showing that the margin is free of tumour, which appears as monotonous tumour cells arranged in a trabecular or ‘ribbon-like’ pattern with numerous luminal rosettes and has finely stippled ‘salt and pepper’ nuclear chromatin without nucleoli and low mitotic rate (low grade). (Panels F and G) Tumour cells positive for synaptophysin and chromogranin cytoplasmic staining. The Ki-67 (not shown) demonstrated a mild-to-moderate proliferation index. (Panel H) A direct view showing the mass completely dissectible from the outer wall of the RV. (Panel I) The excised 6 × 4 cm tan brown mass.
