By: Prof. Dr.: Fawzy Megahed Commentary case 15-2-2017 By: Prof. Dr.: Fawzy Megahed

A 63-year-old woman presented to hospital with 2 months of progressively worsening ataxia, vertigo, and frequent vomiting.

Which of the following is most valuable in determining the cause of her presentation : Lipid profile & Hb A1c. Assay of thiamine level. Neuro-imaging. Pelvi-abdominal ultrasonography. Carotid duplex. Toxicological screening including lead & arsenic

Magnetic resonance imaging (MRI) revealed a 3-cm cerebellar mass with surrounding edema and a 7-mm midline shift at the level of the fourth ventricle.

Initial therapy should be: Antiplatelets. LMW heparin. Referal to neurosurgery. High-dose dexamethasone. L-carnitine and cyanocobalamin. Referal for physio-therapiest.

High-dose dexamethasone therapy was initiated.

The neurologic symptoms resolved after 48 hours.

The best next step is Reassurance with close follow up Correction of any metabolic derangement CT abdomen , pelvis and chest with contrast Antinuclear antibody assay Esophagogastrodudenoscopy & colonscopy Electroencephalography Stereotactic brain biopsy

A computed tomography (CT) scan of the chest with intravenous contrast showed severe emphysematous changes and a left hilar mass invading the mediastinum and the left atrium. The mass occupied more than 60% of the left atrium (Figure 1).

What do you suggest to be next ?

Further imaging with transthoracic echocardiography revealed an immobile mass measuring 4.2 * 3.9 cm attached to the posterior wall of the left atrium (Figure 2).

The left ventricular ejection fraction was 60% to 65%, with a calculated pulmonary artery systolic pressure of 72 mm Hg and a dilated, noncollapsible inferior vena cava.

Cardiac MRI showed a heterogeneously enhancing lesion measuring 5.5 * 4.4 * 3.3 cm filling the majority of the left atrium and the atrial appendage with involvement of the left superior pulmonary vein, upper lobe, and lingular lobe segmental veins (Figure 3).

At that time patient complained of left sided paresis, difficulty with micturation and dysartheria .

A second MRI of the brain showed scattered punctuate lesions consistent with embolic phenomenon.

The the next step in treatment is Surgical resection of the mass . Regional radiotherapy followed by Surgical resection Chemotherapy followed by whole brain radiation None of the above

Cardiothoracic surgery was consulted to evaluate for surgical resection of the atrial mass.

However, given the patient’s severe pulmonary hypertension and emphysema, as well as extensive pulmonary venous involvement of the mass suggestive of an aggressive neoplasm, surgical resection was not pursued.

What to do next?

A CT-guided biopsy of the tumor in the left pulmonary vein showed small round blue cells (Figure 4) consistent with a diagnosis of extensive-stage small cell lung cancer.

The best for this patient is: Radiotherapy on mediastinum followed by chemotherapy Chemotherapy followed by whole brain radiation Radiotherapy on mediastinum with whole brain radiation Chemotherapy alone

The patient received cisplatin and etoposide with 10 fractions of whole brain radiation before being discharged.

DISCUSSION

Cardiac tumors : incidence & types Primary cardiac tumors are rare, and approximately 75% of these tumors are benign. The most common benign tumors are myxomas and papillary fibroelastomas; other examples include rhabdomyomas, fibromas, lipomas, and atrioventricular nodal tumors.

Of primary malignant tumors, the majority are sarcomas; other rare types include cardiac lymphoma and pericardial mesothelioma.

Secondary cardiac tumors are 20 to 1000 times more common than primary cardiac tumors.

The cancers that typically have a predilection for metastasizing to the heart or pericardium include melanoma, lung cancer, leukemia, lymphoma, sarcoma, breast cancer, and esophageal cancer, among others.

Cardiac tumors : presentation Many patients with cardiac tumors are asymptomatic. Complications depend on the location of the tumor, degree of obstruction, and invasion into myocardium and pericardium.

These complications include heart failure, arrhythmias, cardiac tamponade, embolic phenomena, and constitutional symptoms such as fever, arthralgias, and weight loss. As was the case in our patient, embolic stroke may be the presenting feature of cardiac tumors.

Characteristics of tumors more likely to be associated with embolic phenomena included involvement of the aortic valve (most significant predictor), left atrial tumors, absence of mitral regurgitation, New York Heart Association class I/II, and tumor volume <13.3 cm2.

Cardiac tumors : work up Diagnostic workup begins with imaging, which can help to differentiate types of cardiac tumors and define surgical planning.

Imaging with echocardiography is essential in the initial evaluation of a cardiac mass and allows information on size, valvular disease, and cardiac function.

Compared with echocardiography, MRI may be more sensitive in detecting cardiac masses, characterizing the cardiac mass (eg, benign or malignant tumors, or tumor mimics such as thrombus), and appreciating extracardiac involvement.

Gated cardiac CT also may be useful to identify local invasion and other complications.

Cardiac tumors : management Although some patients may be monitored without intervention, many patients benefit from early surgical resection of cardiac tumors.

In primary benign tumors, prognosis after surgical resection is excellent, even if embolic phenomena are present. Prognosis is poor in primary malignant tumors with a median survival often less than 1 year.

Surgical resection, chemotherapy, or radiotherapy may be performed for palliation in some cases. For metastatic disease, as in our patient, surgical outcomes may be palliative, but long-term outcomes remain poor.

Although surgical lung and left atrial resection for T4 lung cancer invading the left atrium may have acceptable outcomes, metastatic disease has a poor prognosis and a nonsurgical approach is usually warranted.