Tumors of the Heart

, Judy W. Hung2 and Judy W. Hung3



(1)
Harvard Medical School Cardiology Division, Department of Medicine, Massachusetts General Hospital, Boston, MA, USA

(2)
Harvard Medical School, Boston, USA

(3)
Echocardiography, Cardiology Division, Department of Medicine, Massachusetts General Hospital, Boston, MA, USA

 



Introduction

Incidence

Classification [, ]

Primary Tumors (Benign or Metastatic)

Secondary Tumors (Metastatic)

Clinical Manifestations []

Diagnosis

Tumor Characteristics and Management [, ]

Basic Principles of Treatment

Ongoing Management

References


Abstract

Tumors of the heart, particularly primary cardiac tumors, are one of the least investigated subjects in oncology. As such cardiac tumors and their associated complications rarely gain clinical attention despite their potentially serious sequela. Early recognition and diagnosis of a cardiac tumor by a combination of clinical symptoms and the use of modern imaging tools, could lead to the institution of appropriate management in a timely manner. This in turn may lead to better morbidity and mortality outcomes for these patients.


Abbreviations


AV

Atrioventricular

CCT

Cardiac Computed Tomography

EKG

Electrocardiogram

RA

Right atrium

ESR

Erythrocyte Sedimentation Rate

HCM

Hypertrophic cardiomyopathy

IAS

Inter atrial septum

IL-6

Interleukin-6

IVC

Inferior vena cava

LA

Left atrium

LV

Left ventricle

MRI

Magnetic Resonance Imaging

MR

Mitral valve regurgitation

MS

Mitral valve stenosis

PET

Positron Emission Tomography

PR

Pulmonic valve regurgitation

PS

Pulmonic valve stenosis

PND

Paroxysmal nocturnal dyspnea

RV

Right ventricle

SOB

Shortness of breath

SVC

Superior vena cava

TEE

Transesophageal echocardiogram

TIA

Transient Ischemic Attack

TR

Tricuspid valve regurgitation

TS

Tricuspid valve stenosis

TTE

Transthoracic echocardiogram

VEGF

Vascular endothelial growth factor

WBC

White Blood Cell Count



Introduction


Tumors of the heart, particularly primary cardiac tumors, are one of the least investigated subjects in oncology. As such cardiac tumors and their associated complications rarely gain clinical attention despite their potentially serious sequela. Early recognition and diagnosis of a cardiac tumor by a combination of clinical symptoms and the use of modern imaging tools, could lead to the institution of appropriate management in a timely manner. This in turn may lead to better morbidity and mortality outcomes for these patients.

See Chap.​ 34 for images of cardiac tumors.


Incidence






  • Primary cardiac tumors



    • Rare <0.1 % prevalence (0.001–0.3 %) [1, 2]


    • 75 % are benign [3–5]


  • Secondary tumors (metastatic)



    • Secondary tumors are 20–40 times more common than primary cardiac tumors [6–8]


    • 15 % of cancer patients develop cardiac metastasis


Classification [8, 9]



Primary Tumors (Benign or Metastatic)






  • Tumor can arise from



    • Endocardium


    • Myocardium


    • Pericardium


    • Valve tissue


    • Cardiac connective tissue


Secondary Tumors (Metastatic)






  • Cardiac metastases can arise via:-



    • Blood dissemination of cancer cells


    • Direct extension via adjacent tissues


    • Propagation to the right atrium (RA) via superior vena cava (SVC) and inferior vena cava (IVC; classic: renal cancer)


  • Pericardium most commonly affected (69.4 %), followed by epicardium (34.2 %) and myocardium (41.8 %) then endocardium (5 %) [10]

Common metastatic cancers invading the heart and their prevalence (See Table 19-1 [10])


Table 19-1
Incidence of cardiac metastasis. Results are from 622 cardiac metastases out of 7,289 patients with malignant neoplasm, based on 18,751 autopsies Adapted from [10]












































































Cancer type
Number of patients
% with cardiac metastasis
Prevalence (all neoplasms) (%)
Prevalence (metastatic neoplasms) (%)

Mesothelioma
128
48.4
54.2
9.4

Melanoma
79
27.8
34.1
3.3

Adenocarcinoma of lung
460
21
26.1
14.6

Poorly differentiated carcinoma of lung
420
19.5
21.2
12.4

Squamous cell carcinoma of lung
428
18.2
23.4
11.8

Breast carcinoma
427
15.5
20.6
10.0

Ovarian carcinoma
106
10.3
11.6
1.5

Leukemia/lymphoma
711
9.4
17.3
10.1

Stomach carcinoma
360
8
9.8
4.4

Renal cell carcinoma
287
7.3
16.3
3.2


Adapted from Bussani et al. [10]

Melanoma cited as most common metastatic cancer [11]



  • Carcinoid Syndrome [8]



    • Lesions in the right heart chambers


    • Mainly affects cardiac valves


    • Formation of fibrous plaques result in thickening, shortening and decreased leaflet motion of tricuspid and/or pulmonic valve and regurgitation or stenosis


Clinical Manifestations [9]


Symptoms/signs dependent on location and size of tumor and not histopathology.



  • Systemic symptoms



    • Resemble vasculitis and connective tissue diseases


    • Fever, weight loss, fatigue, arthralgia, Raynaud’s phenomenon


    • Labs – anemia, ↑white blood cell count (WBC), ↑platelets, ↑erythrocyte sedimentation rate (ESR), hypergammaglobulinemia


    • Attributed to secretion of factors like interleukin-6 (IL-6), endothelin, vascular endothelial growth factor (VEGF)


  • Cardiovascular symptoms



    • Obstructive



      • For example, a left atrial tumor such as myxoma can present similarly to mitral stenosis


    • Heart failure


    • Arrhythmias


    • Atypical or non specific chest pain


  • Systemic embolization



    • Peripheral thromboembolism


    • Pulmonary embolism


    • Stroke


  • Symptoms due to metastasis



    • Pericardial disorders e.g. tamponade


    • Pulmonary symptoms (Table 19-2)


      Table 19-2
      Locations of tumor and likely associated signs/symptoms [14, 15]
























































      Location of tumor
      Symptoms/signs
      Mechanism for underlying ­symptoms/signs

      Left atrium
      Dyspnea
      Left heart failure resulting from:-

      Orthopnea
      1. Obstruction to circulation i.e. obstruction to atrial or ventricular filling

      Paroxysmal nocturnal dyspnea (PND)
      2. Impairment of mitral valve function (mitral regurgitation (MR))

      Pulmonary edema
      Systemic embolization

      Cough

      Hemoptysis

      Edema

      Fatigue

      Strokes/Transient Ischemic Attack (TIA)s

      Right atrium
      Fatigue
      Right heart failure resulting from:-

      Pulmonary edema
      1. Obstruction to circulation i.e. obstruction to atrial or ventricular filling

      Hepatomegaly
      2. Impairment of tricuspid valve function (tricuspid valve stenosis (TS))

      Ascites

      Prominent “a” waves in jugular veins

      L ventricle
      Arrhythmias
      Left heart failure resulting from:-

      Conduction defects
      1. Obstruction to circulation i.e. obstruction to atrial or ventricular filling
      < div class='tao-gold-member'>

      Only gold members can continue reading. Log In or Register to continue

      Related posts:

      1. Diseases of the Aorta
      2. Lipoprotein Disorders
      3. Nuclear Cardiology and Exercise Stress Testing
      4. Imaging Studies Section (Echocardiograms, Ventriculograms, Aortograms and Angiograms)

      Stay updated, free articles. Join our Telegram channel
      Tags:
      Jul 13, 2016 | Posted by in CARDIOLOGY | Comments Off on Tumors of the Heart

      Full access? Get Clinical Tree

       Get Clinical Tree app for offline access