Primary high-grade neuroendocrine carcinoma of the heart

Gustavo E Guajardo-Salinas1, Javier E Anaya-Ayala, David C Rice

  • 1Department of Cardiothoracic Surgery, University of Texas MD Anderson Cancer Center, Methodist DeBakey Heart & Vascular Center, Houston, Texas 77030, USA.

Insights

This case study details the successful palliative resection of a rare, high-grade neuroendocrine heart tumor. Surgical intervention prolonged survival and improved outcomes for a patient with advanced cardiac obstruction.

Area of Science:

  • Cardiology
  • Oncology
  • Surgical Pathology

Background:

  • Primary cardiac neuroendocrine tumors are exceedingly rare.
  • High-grade neuroendocrine carcinoma of the heart presents a significant surgical challenge due to potential for rapid growth and obstruction.

Observation:

  • A 70-year-old male presented with progressive dyspnea due to a large, solitary, high-grade neuroendocrine carcinoma.
  • The tumor extensively involved the right atrium, superior and inferior vena cavae, aortic root, and interatrial septum.

Findings:

  • Palliative tumor resection, excluding the aortic root and trigone, was performed, followed by reconstruction of the right atrium and interatrial septum.
  • The patient recovered well with only minor local recurrence at 18 months post-surgery.

Implications:

  • Palliative resection of large neuroendocrine cardiac tumors can achieve favorable outcomes and extend patient survival.
  • This case highlights the feasibility of surgical management for primary high-grade neuroendocrine cardiac tumors, even with extensive involvement.

Related Concept Videos

Cushing Syndrome II: Pathophysiology01:19

Cushing Syndrome II: Pathophysiology

Cortisol production is normally governed by the hypothalamic–pituitary–adrenal (HPA) axis, which maintains hormonal balance through tightly regulated feedback mechanisms. Disruption of this regulatory system is central to the development of Cushing syndrome, whether the excess cortisol originates from external medications or internal pathology. Persistent cortisol elevation alters metabolism, immune function, and endocrine signaling, producing the characteristic clinical features of the...
Cardiomyopathy III: Hypertrophic Cardiomyopathy01:29

Cardiomyopathy III: Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy, or HCM, is an autosomal dominant genetic disorder characterized by asymmetric left ventricular hypertrophy without ventricular dilation. It is more common in men and is typically diagnosed in young, athletic adults.EtiologyHCM is primarily genetic and is caused by mutations in genes encoding sarcomeric proteins. Researchers have identified over 1400 mutations across at least 11 different genes. Among these, the most frequently occurring mutations are found in the...
Hyperthyroidism II: Pathophysiology01:27

Hyperthyroidism II: Pathophysiology

Hyperthyroidism is a hypermetabolic state caused by elevated levels of thyroid hormones, triiodothyronine (T3) and thyroxine (T4). It results from dysregulation at the thyroid, pituitary, or immune system level and affects multiple organ systems.PathophysiologyThe most common cause of hyperthyroidism is Graves’ disease, an autoimmune disorder in which antibodies, specifically thyroid-stimulating antibodies (TSAb), a subtype of TSH receptor antibodies (TRAb), bind to and activate TSH receptors...
Heart Failure II: Pathophysiology01:29

Heart Failure II: Pathophysiology

Systolic Heart Failure and Compensatory MechanismsSystolic heart failure (also termed HFrEF, Heart Failure with Reduced Ejection Fraction) is the most prevalent type of heart filure. It results in a decreased volume of blood being pumped from the ventricle. The aortic arch and carotid sinuses have baroreceptors that detect reduced blood pressure, triggering the sympathetic nervous system (SNS) to release epinephrine and norepinephrine. Initially, this response aims to boost heart rate and...
Cardiomyopathy I: Introduction and Classification01:25

Cardiomyopathy I: Introduction and Classification

Cardiomyopathy, or CMP, is a group of diseases affecting the myocardial structure, impairing its ability to pump blood effectively. This condition can lead to arrhythmias, heart failure, or sudden cardiac death.Cardiomyopathies are classified into primary and secondary categories:Primary Cardiomyopathy refers to conditions involving only the heart muscle that are often idiopathic (of unknown cause) or genetic. They primarily affect the myocardium without the involvement of other systemic...
Heart Failure IV: Classification and Diagnostic Evaluation01:30

Heart Failure IV: Classification and Diagnostic Evaluation

Heart failure can be classified in various ways, with the most common classifications based on physical activity limitations, disease progression, severity, and treatment strategies.The Functional Classification of Heart Failure divides patients into four categories based on physical activity limitation due to symptom burden.Class I: Patients in this class have cardiac disease but no physical activity limitations. Ordinary activities like walking, climbing stairs, or routine tasks do not cause...