Carcinoma of Unknown Primary Presenting as Large Right ...

HEART VIEWSApr-Jun 14 Issue 2 / Vol 15

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ABSTRACT

We report a 65-year-old male patient who presented with right heart failure and a large mobile right atrial and ventricular mass on echocardiography. His computed tomography demonstrated bilateral supraclavicular/mediastinal lymphadenopathy, right atrial and ventricular mass with right pulmonary artery segmental embolism, and multiple liver hypodense lesions. His tumor markers were negative. However, fine-needle aspiration cytology of supraclavicular lymph node revealed metastatic carcinoma suggestive of squamous cell carcinoma. He was suspected to have carcinoma of unknown primary origin. This case illustrates a rare presentation of carcinoma of unknown primary origin with disseminated tumor thrombosis primarily manifesting in heart and other sites.

Key words: Carcinoma of unknown primary, cardiac metastasis, right atrium, tumor thrombosis

Carcinoma of Unknown Primary Presenting as Large Right Atrial and Ventricular Mass Secondary to

Disseminated Tumor Thrombosis

Prashanth Panduranga, Zahid Kazmi, Najib Al‑Rawahi, Zulfikar Habibulla1, Nabil Al‑Lawati1, Faiza Al‑Kindi2

Departments of Cardiology, 1Chest Medicine, 2Radiology, Royal Hospital, Muscat, Sultanate of Oman

INTRODUCTION

Secondary cardiac metastatic tumors are more common than primary cardiac tumors.[1,2] Cardiac metastases is predominantly in the sixth and

seventh decade of life with no gender disparity.[1] Cardiac metastases are found in up to 25% of post-mortem patients with malignancy and are usually found late and rarely seen as first site of metastases.[1,2] We report a 65-year-old male patient who presented with right heart failure and a large mobile right atrial and ventricular mass on echocardiography that turned out to be tumor thrombosis.

CASE REPORT

A 65-year-old man, non-smoker, presented to a regional hospital with nonspecific chest pain and exertional shortness of breath for one week duration. He has history of long‑standing hypertension and atrial fibrillation on oral warfarin therapy. He gave history of generalized fatigue, weight loss, over two-month period. He was diagnosed to have heart failure and treated.

He underwent a transthoracic echocardiogram, which

revealed a multi‑lobulated mass measuring 5.1 × 2.5 cm freely mobile in the right atrium (RA) [Figure 1a and b, arrowheads, Video]. In addition, there was another mass measuring 1.4 × 2.8 cm in the right ventricle [Figure 1c, arrowheads, Video]. This was interpreted as either thrombus or cardiac tumor. His international normalized ratio (INR) was 2.6. He was referred to our center for transesophageal echocardiogram. Clinically he was afebrile, looking sick, emaciated with palpable hard right supraclavicular lymph node. Chest and cardiac examinations were unremarkable. There was no evidence of deep vein thrombosis. His chest X-ray showed cardiomegaly with mild bilateral pleural effusions. Electrocardiograph (ECG) demonstrated atrial fibrillation with controlled ventricular rate. On enquiry, he gave history of generalized fatigue, weight loss, over 2 month’s period.

Case Report

How to cite this article: Panduranga P, Kazmi Z, Al-Rawahi N, Habibulla Z, Al-Lawati N, Al-Kindi F. Carcinoma of unknown primary presenting as large right atrial and ventricular mass secondary to disseminated tumor thrombosis. Heart Views 2014;15:54-6. © Gulf Heart Association 2014.

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DOI:

10.4103/1995-705X.137506 Address for correspondence: DR. Prashanth Panduranga, Department of Cardiology, Royal Hospital, PB 1331, Muscat‑111, Sultanate of Oman. E‑mail: [email protected]

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Panduranga, et al.: Mobile right atrial and ventricular mass


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Repeat echocardiogram confirmed the previous echocardiogram findings Biventricular function was good and valves were normal. There was minimal posterior pericardial effusion.

Computed tomography (CT) of the neck, chest and abdomen/pelvis showed bilateral supraventricular lymph node enlargement with large superior and anterior mediastinal lymphadenopathy [Figure 2a, arrowhead], right upper lobe pulmonary artery intraluminal filling defects [Figure 2c, arrowheads], and multiple hypodense liver lesions in left lobe.

He was suspected to have metastatic carcinoma of unknown primary origin (CUP). Blood investigation revealed normal full blood count, urea, electrolytes, creatinine, negative blood and urine cultures, lactate dehydrogenase was 321 IU/L (n = 95-190), alkaline phosphatase 149 IU/L (n = 30-110), albumin 22 gm/L (n = 35-50), C-reactive protein (CRP) 174 mg/L (n < 5),

and erythrocyte sedimentation rate (ESR) 34 mm/Hr (n = 0-20). Tumor markers like prostate-specific antigen, cancer antigen 125/19-9, alpha-fetoprotein, carcinoembryonic antigen and Beta-2 microglobulin were all within normal limits. Bone scan did not reveal any bone metastasis. Pleural fluid aspiration was negative for malignant cells. However, supraclavicular lymph node fine-needle aspiration cytology showed metastatic carcinoma suggestive of squamous cell carcinoma. He was seen by multidisciplinary team and found to be in advanced stage of cancer and ineligible for any specific treatment. He died after 2 weeks of admission.

DISCUSSION

The most common tumors metastazing to heart are: Malignant melanoma, carcinomas of the lung, breast and

Figure 2: Computed tomography chest showing large superior and anterior mediastinal lymphadenopathy (a, arrowheads), filling defects within RA and right ventricle (b, arrowheads), and right upper lobe pulmonary artery intraluminal filling defects (c, arrowheads) in a patient with carcinoma of unknown primary origin

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Figure 1: Transthoracic echocardiogram showing a multilobulated mass measuring 5.1 × 2.5 cm freely mobile in the right atrium (a and b, arrowheads) in a patient with carcinoma of unknown primary origin. Note another mass measuring 1.4 × 2.8 cm in the right ventricle (c, arrowheads). RA: Right atrium, LA: Left atrium, RV: Right ventricle, LV: Left ventricle

cba




Panduranga, et al.: Mobile right atrial and ventricular mass


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esophagus, malignant lymphoma, and leukemia.[1] They commonly spread to pericardium and myocardium and less to endocardium.[3] Metastatic spread reaches the heart by lymphatic or hematogenous route, or by direct or transvenous extension (pulmonary veins, superior or inferior vena cava).[1] The RA is commonly involved through inferior vena cava from sub-diaphragmatic malignancy and carcinoma of the lung and thyroid gland spread through the superior vena cava into the RA.[1]

The predominant causes of intracardiac mass includes benign and malignant primary cardiac tumors as well as thrombus or a vegetation. Primary thrombi develop within the RA (in situ thrombi) and are usually immobile and attached to atrial wall as in atrial fibrillation. Secondary thrombi result from venous embolization, are mobile and temporarily lodge in the RA or right ventricle (emboli-in-transit) as noted in this patient. Secondary tumors presenting as intra-cardiac mass are very rare and often covered by thrombotic material leading to tumor thrombosis as demonstrated in this patient.[1]

Cardiac metastases are commonly silent, but when manifest they present with pericardial effusion with or without tamponade, heart failure or valve dysfunction (marantic vegetations), tachy- or brady-arrhythmias and systemic or pulmonary embolism.[1] Diagnosis of cardiac metastasis is done primarily with the use of echocardiography (transthoracic or transesophageal) and imaging techniques (CT or Magnetic Resonance Imaging).[3] Treatment options, in most of the cases, is palliative as it manifests at advanced stage of the tumor as in this case.[1] Generally, in this advanced stage many patients would have

undergone either surgery (for the tumor of origin) or radio- or chemotherapy. However, in this patient, unknown malignancy presented with cardiac metastasis and disseminated tumor thrombosis at an advanced stage with a rapidly progressive downhill course leading to his death. Surgical resection of cardiac tumor is advised, if only solitary intra-cardiac mass is present causing chamber or valvular obstruction and if overall prognosis is better.

In the case presented, chest symptoms were the first manifestation of unknown malignancy, leading to the diagnosis of right atrial/ventricular mass and pulmonary artery embolism indicating progressive showering of tumor emboli from the RA tumor thrombus. We were unsuccessful in identifying the primary tumor, despite extensive blood investigations and imaging studies indicating carcinoma of unknown primary origin (CUP). This case illustrates a rare first presentation of CUP origin with disseminated tumor thrombosis at multiple sites.

REFERENCES

1. Reynen K, Kockeritz U, Strasser RH. Metastases to the heart. Ann Oncol 2004;15:375‑81.

2. Pinho T, Rodrigues‑Pereira P, Araújo V, Oliveira NP, Macedo F, Graça A, et al.Cardiacmetastasisofmelanomaasfirstmanifestationof disease. Rev Port Cardiol 2009;28:633‑9.

3. Ekmektzoglou KA, Samelis GF, Xanthos T. Heart and tumors: Location, metastasis, clinical manifestations, diagnostic approaches, and therapeutic considerations. J Cardiovasc Med (Hagerstown) 2008;9:769‑77.

Source of Support: Nil, Conflict of Interest: None declared.

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