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Case Report Malignant phyllodes tumor of the left atrium

Anupam Bhambhani a,*, Sudha Ayyagari b, Tushar Mohapatra c, Syed Abdul Rehman b, Milap Shah b, Sudhakar Rao e, Vital Rangashamanna d, V. Rajasekhar a, Santosh Chittimilla f

a Senior Consultant, Department of Cardiology, Yashoda Hospital, Secunderabad, India b Consultant, Department of Pathology, Yashoda Hospital, Secunderabad, India c Consultant, Department of Nuclear Medicine, Yashoda Hospital, Secunderabad, India d Consultant, Department of Radiology, Yashoda Hospital, Secunderabad, India e Consultant Cardiothoracic Surgery, Yashoda Hospital, Secunderabad, India f Registrar ICCU, Yashoda Hospital, Secunderabad, India

article info abstract

Article history: Metastatic tumors to the heart usually involve right sided chambers. We report a rare case Received 26 May 2013 of malignant phyllodes tumor of breast with metastatic involvement of left atrium Accepted 5 February 2014 occurring through direct invasion from mediastinal micro-metastasis and presenting as a Available online 1 March 2014 left atrial mass causing arrhythmia. Copyright ª 2014, Cardiological Society of India. All rights reserved. Keywords: Left atrial tumor Cardiac metastasis Carcinoma phyllodes

A 50-year-old lady presented to the casualty department, with septum. The LA appendage was free of thrombus. The mass history of an episode of sudden onset New York Heart Asso- was intermittently protruding through the mitral orifice. ciation (NYHA) class IV dyspnea and transient palpitation, Remaining echocardiographic assessment was unremarkable. occurring about 3 h prior to presentation and subsiding In view of recent history of resection of malignant breast spontaneously. Her Electrocardiogram done in a primary tumor, it was considered essential to rule out malignant na- hospital showed supra-ventricular tachycardia. ture of the LA mass and also to look for local metastases in the At presentation, she was asymptomatic, with unremark- surrounding thoracic viscera before planning definitive able physical examination. There was past history of excision treatment. Hence, probabilities of LA myxoma, breast metas- of right breast lump with axillary clearance for a malignant tasis or LA thrombus were considered and computed tomog- mass a year ago, followed by radiation therapy. On echocar- raphy (CT) of thorax with contrast was advised to further diography, a large LA mass was seen occupying almost whole characterize the mass. CT scan was performed on dual source of the LA cavity, with probable attachment to a pulmonary dual energy 64-slice MDCT scanner before and after injection vein (Fig. 1A). The mass had no attachment to inter atrial of 75 ml non-ionic iodinated contrast. It revealed a large

* Corresponding author. Department of Cardiology, Yashoda Hospital, S.P. Road, Secunderabad 500003, India. Tel.: þ91 9989027102. E-mail address: [email protected] (A. Bhambhani). 0019-4832/$ e see front matter Copyright ª 2014, Cardiological Society of India. All rights reserved. http://dx.doi.org/10.1016/j.ihj.2014.02.008 242 indian heart journal 66 (2014) 241e243

Fig. 1 e A: Echocardiogram in parasternal long axis view showing large mass in left atrium (arrow heads); MV -mitral valve, LV- left ventricle. B: 64-slice MDCT with contrast showing filling defect in left atrium. C: Surgical specimen excised from left atrium. D: H&E stain sections of breast tumor showing features of malignant Phyllodes having components of spindle cells (D1) as well as neoplastic chondrocytes with high mitotic activity (D2) (arrows). E: H&E stain sections from left atrial mass (E1) & breast mass (E2) showing histo-morphologically similar spindle cell components indicating common origin for both neoplasms. F: PET scan showing abnormal tracer activity involving soft tissue mass occupying entire left superior pulmonary vein and partially entering adjacent atrial cavity (large arrow). Small arrow indicates focal abnormal tracer activity in the anterior wall of ascending aorta suggesting malignant embolism. G: Tomographic scan one month after atrial tumor resection revealed pulmonary nodule (arrow) gaining access into the adjacent pulmonary vein (arrow head). H: Positron emission tomography after tumor excision e the Maximum Intensity Projection image showing increased metabolic activity in left atrium, extending into left superior pulmonary vein (arrow), corresponding to residual malignant atrial tumor. Metastatic activity in left axillary lymph node (arrow head) is also visible. The midline shows metabolically active sternotomy scar suggestive of inflammation.

(55 39 41 mm) hypo dense filling defect lying along mediastinum, thrombus was considered more likely, but the postero-lateral wall and roof of LA (Fig. 1B), extending from scan did not allow for clear differentiation between bland left superior pulmonary vein. The diagnostic possibilities of thrombus and tumor tissue. Similar attenuation value for thrombus and metastatic tumor were considered. In view of thrombus and tumor on CT scan is a known pitfall during absence of metastatic nodules in lung parenchyma or in tumor evaluation.1 In view of potential for systemic indian heart journal 66 (2014) 241e243 243

embolization and/or acute mitral valve obstruction, early involvement of other anatomically related structures to surgical resection was performed. Left atriotomy revealed a explain the route of metastasis. In this case the metastatic large tumor mass attached to left superior pulmonary vein, lesions of lung and mediastinum were too small to be detected occupying LA chamber and partially protruding into left ven- even by CT scan at the time of diagnosis of cardiac tumor and tricular inflow. Presence of patent foramen ovale was ruled could be detected only by increased metabolic activity on PET out intra-operatively, since, it could be a potential explanation scan. In all other cases reporting pulmonary vein invasion for the metastatic route from breast to left atrium without from mediastinum, the primary tumor was large enough to involving right heart, as revealed in CT scan. The mass was explain local invasion, before extending into the pulmonary resected and sent for histopathological examination (Fig. 1C). vein.7 Thirdly, although extension of metastases into the left The post-operative recovery was uneventful. Meanwhile, her atrium by invasion of the pulmonary veins is well docu- previous pathological records were reviewed, which revealed mented for primary pulmonary malignancies, this phenom- the following. A breast tumor measuring 3.5 3 3 cm in size enon is uncommon for lung or mediastinal metastatic was resected a year ago with axillary clearance. The base of lesions.4 the mass and soft tissue margins were free of abnormal tissue. In our case, the probable route of metastases was hema- All the resected lymph nodes were free of tumor. On histologic togenous into the contra lateral axillary lymph nodes, lung examination, the breast lump had features of malignant and mediastinal lymph nodes. From the latter, it invaded phyllodes having components of spindle cells (Fig. 1D e 1) as directly through the left superior pulmonary vein. Malignant well as neoplastic chondrocytes with high mitotic activity phyllodes are known to metastasize by the hematogenous (Fig. 1D e 2). Immunohistochemical profile was negative for ER route rather than lymphatic. This also explains the reason for (Estrogen receptor), PR (Progesterone receptor) and Her2eu. negative nodes on initial axillary dissection. The cardiac tumor revealed histomorphologic features of a Cardiac metastases are rare but important cardiovascular sarcoma (Fig. 1E e 1) with features very similar to that of the diagnoses. Their potentially lethal course and the possibility spindle cell component of the breast mass (Fig. 1E e 2), indi- of avoiding major complications with excision, make their cating common origin for both the tumors. diagnosis consequential. Malignant tumors may reach heart A subsequently performed whole body positron emission through atypical routes. Strategic strong index of suspicion tomography (PET) scan with F18 e fluorodeoxy glucose and thorough investigation of every case may avoid missing revealed an area of abnormal tracer activity in a soft tissue the diagnosis. mass occupying entire left superior pulmonary vein and encroaching into adjacent LA wall (Fig. 1F e large arrow). A one month later performed PET scan showed a single pul- Conflicts of interest monary nodule with abnormal tracer activity abutting the left superior pulmonary vein, suggesting vein erosion and access All authors have none to declare. into cardiac chamber (Fig. 1G and H). It is noteworthy that this nodule was not visualized in previous scan. There was no other hypermetabolic lesion in rest of the lungs. Interestingly, references another focus of increased radiotracer activity was found in ascending aorta suggesting tumor embolization (Fig. 1F e small arrow). The whole right breast and right axilla were free of malignant activity; however, two left axillary lymph nodes 1. Gross BH, Glazer GM, Francis IR. CT of intracardiac and intrapericardial masses. AJR Am J Roentgenol. 1983 revealed hypermetabolic lesions (Fig. 1H). In addition, there May;140:903e907. was a small subcutaneous lesion in right anterior abdominal 2. Jackson CE, Gardner RS, Connelly DT. A novel approach for a wall (Fig. 1H). novel combination: a trans-septal biopsy of left atrial mass in Cystosarcoma phyllodes, is a rare, locally aggressive and recurrent phyllodes tumour. Eur J Echocardiogr. 2009 recurrent tumor accounting for approximately 1% of breast Jan;10:171e172. Epub 2008 Aug 1. tumors. While metastases to heart are uncommon, cardiac 3. Majano-Lainez RA. Cardiac tumors: a current clinical and e involvement is known.2 The pathways of metastatic invasion pathological perspective. Crit Rev Oncog. 1997;8:293 303. 4. Schwentner L, Kurzeder C, Kreienberg R, Wo¨ ckel A. Focus on of heart in descending order of frequency are tumor emboli- haematogenous dissemination of the malignant cystosarcoma zation through veins, hematogenous spread, lymphatic spread phylloides: institutional experience. Arch Gynecol Obstet. 2011 3 and direct invasion. Although relatively rare, hematogenous Mar;283:591e596. metastasis is associated with poor prognosis.4 Other rare pre- 5. Nakatsu T, Koshiji T, Sakakibara Y, et al. Pulmonary artery sentations of this tumor are RV metastasis causing pulmonary obstruction due to a metastatic malignant phyllodes tumor of e embolism, RVOT obstruction5 or even cardiogenic shock.6 the breast. Gen Thorac Cardiovasc Surg. 2010 Aug;58:423 426. The present case is interesting for many reasons. First, 6. Garg N, Moorthy N, Agrawal SK, Pandey S, Kumari N. Delayed cardiac metastasis from phyllodes breast tumor presenting as Cystosarcoma Phyllodes metastasizing to heart is a rare en- cardiogenic shock. Tex Heart Inst J. 2011;38:441e444. tity. Secondly, the tumor presented as a substantially large 7. Davies MJ. Tumours of the heart and pericardium. In: intracavitary cardiac mass causing decompensation and sig- Pommerance A, Davies MJ, eds. The Pathology of the Heart. nificant immediate risk potential, without easily recognizable Oxford: Blackwell Scientific Publications; 1975:413e439.