JAFES-Booklet (English - pdf - 1103 Kb) - MEMS

Cardiac Paraganglioma In A Familial Paraganglioma Syndrome -
Coronary Artery embolization For Nonresectable Tumor.
Zanariah H 1 , Alfazir O 2 , Rosli MA 2
1 Endocrinology Unit, Department of Medicine, Hospital Putrajaya
2 Department of Cardiology, National Heart Institute (IJN)
Primary cardiac paraganglioma is a very rare tumor with less than 60 reported cases in literature to
date. Clinical presentation is variable,most commonly manifested by hypertension and symptoms
related to catecholamine excess such as palpitations, headache and sweating. Most are benign and
most commonly arise from the left atrial wall. The tumor is often poorly encapsulated and local
invasion can occur with metastases reported in up to 10% of cases. Surgical excision is the treatment
of choice, often complex and necessitating cardiopulmonary bypass. It is often difficult to detect
preoperatively the degree of extension of the tumor and its resectability. Patients with non-resectable,
intracardiac tumors have been considered for cardiac transplantation. Embolization of feeding coronary
arteries to the tumor has been performed, mainly as a preoperative procedure to reduce perioperative
bleeding.
We describe a case of non-resectable functioning cardiac paraganglioma who underwent embolization
of coronary arteries with subsequent improvement in clinical status, normalization of urinary
catecholamines and reduction in tumor size.
A 42-year-old Chinese male presented with recent onset hypertension and diabetes with an abnormal
chest radiograph. He was otherwise asymptomatic. He was on metformin monotherapy with a HbA1c
of 7.6% and had severe hypertension of 180/140 mmhg with a postural drop to 150/120 mmHg at
presentation to our center. He had previous surgical excision of bilateral carotid paragangliomas. His
sister was recently diagnosed to have abdominal paraganglioma with liver metastases.
Initial CT scan noted a mediastinal mass adjacent to the main pulmonary artery measuring 6.3 x 3.9
cm and normal appearance of both adrenal glands. Coronary angiogram revealed a highly vascular
cardiac mass adjacent to pulmonary artery with feeding vessels from the Right coronary (RCA) and
Left anterior descending (LAD) arteries. Twenty-four hour urinary noradrenaline levels were elevated
at 19236nmol (normal 40 – 780 nmol) with normal urinary adrenaline levels. MIBG scan revealed a
faint tracer uptake in the heart and otherwise physiological uptake elsewhere.
Cardiothoracic consultation concluded that the tumor was inoperable. The patient was referred to the
cardiologist for consideration of tumor embolization. He was prepared with oral phenoxybenzamine
and later addition of propranolol. Coronary angiogram was performed with alcohol ablation of the
tumor and placement of a helical coil in the main feeder artery to the tumor. On follow-up, blood pressure
gradually normalized and antihypertensives were withdrawn. Urinary catecholamines had
normalized at three months follow-up with CT scan showing tumor size reduction.
Follow-up to detect regrowth of cardiac tumor and development of additional paragangliomas at
other sites is important. He most likely has a succinate dehydrogenase (SDH) gene mutation giving
rise to familial and multifocal disease. Genetic studies are necessary for him and his family to enable
early diagnosis and disease prognostication.
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