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 Table of Contents  
REVIEW ARTICLE
Year : 2018  |  Volume : 4  |  Issue : 3  |  Page : 180-183

Primary epithelioid leiomyosarcoma of left atrium: Pathologist clinches it all


1 Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
2 Department of Cardiac Radiology, All India Institute of Medical Sciences, New Delhi, India
3 Department of Cardiothoracic and Vascular Surgery, All India Institute of Medical Sciences, New Delhi, India

Date of Web Publication11-Jan-2019

Correspondence Address:
Dr. Sudheer Arava
Department of Pathology, All India Institute of Medical Sciences, Ansari Nagar, New Delhi - 110 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jpcs.jpcs_44_18

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  Abstract 

Primary cardiac neoplasms are rare with an estimated prevalence of 0.001%–0.2%. Cardiac tumors are mostly benign and myxomas are the commonest. Malignant tumors are rare in which metastasis and infiltration from the adjacent malignant tumor is more common than the primary ones. Amongst the primary malignant tumors sarcomas are the commonest. Primary epithelioid leiomyosarcoma of the heart is extremely rare. Here, we present a case of a 67-year-old female patient who was admitted with acute onset of pulmonary edema; on investigation, the patient was found to have left atrial mass. A provisional diagnosis of large left atrial myxoma with left ventricular inflow obstruction was made. Biopsy taken showed features of leiomyosarcoma which was confirmed by immunohistochemistry. Most of the patients with cardiac sarcoma have a high-grade tumor at the time of diagnosis with distant metastasis in the lungs (35.7%), lymph node (14.2%) followed by liver (7.14%) and they potent a poor prognosis. At present, only a few hundred primary cardiac sarcomas have been reported, most of which are based on autopsy series. Primary cardiac epithelioid leiomyosarcoma is a rare entity that can pose a diagnostic dilemma. However, atypical presentation of “atrial myxoma” being the common differential diagnosis. Histopathological evaluation is confirmatory to diagnose such a lesion. The pathologist should be aware of such an entity which can be attended by morphological evaluation including immunohistochemistry.

Keywords: Cardiac sarcomas, cardiac tumors, leiomyosarcoma


How to cite this article:
Phulware RH, Barwad A, Kumar S, Bhoje AK, Chandrashekhara S H, Arava S, Ray R. Primary epithelioid leiomyosarcoma of left atrium: Pathologist clinches it all. J Pract Cardiovasc Sci 2018;4:180-3

How to cite this URL:
Phulware RH, Barwad A, Kumar S, Bhoje AK, Chandrashekhara S H, Arava S, Ray R. Primary epithelioid leiomyosarcoma of left atrium: Pathologist clinches it all. J Pract Cardiovasc Sci [serial online] 2018 [cited 2019 Mar 19];4:180-3. Available from: http://www.j-pcs.org/text.asp?2018/4/3/180/249933


  Introduction Top


Primary cardiac tumors are rare and most of them are benign. Most of the primary malignant tumors are sarcomas and epithelioid leiomyosarcoma is extremely rare. These tumors can pose a diagnostic dilemma due to atypical clinical presentation and varied radiological features. Histopathological examination is the gold standard in confirming the diagnosis. The present case has been highlighted due to its rarity. Hence, knowledge regarding this neoplasm is must for the clinicican, radiologist and pathologist for proper diagnosis, patient care and management.


  Case Report Top


A 67-year-old female presented to the cardiology outpatient department with the clinical signs and symptoms of acute onset pulmonary edema. Echocardiographic evaluation showed a large left atrial mass with heterogeneous appearance, attached to the lower and anterior interatrial septum and atrial aspect of the anterior mitral valve leaflet (AML) without any evidence of calcification. The mass was prolapsing into the left ventricle (LV) with the only free movement of AML tip. There was moderate mitral stenosis, LV inflow obstruction with a mean diastolic gradient of 12 mm of Hg. The posterior mitral leaflet, other valvular morphology was normal with normal bi-ventricular function. Computed tomography (CT) angiography showed dilated left artrium (LA) with large hypo dense nonenhancing mass lesion measuring 58 mm × 37 mm [Figure 1]. It was abutting the AML causing restriction of the valve movement. The lung field showed bilateral plural effusion with passive atelectasis. All the coronary vessels were unremarkable without any evidence of significant luminal occlusion. With these radiological features, the possibility of left atrial clot or left atrial myxoma with dilated LA was considered as differentials.
Figure 1: Contrast-enhanced computed tomography four-chamber view of the heart showing heterogeneously enhancing mass lesion (*) with broad base attachment toward interatrial septum, infiltrating the posterior left atrial wall and involving mitral apparatus with extension into left ventricle, bilateral pleural effusion (#), collapse left lung segment (+) (RA: Right atrium, LA: Left atrium, RV: Right ventricle, LV: Left ventricle).

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The patient was planned for surgical excision of the tumor. Intraoperatively, the operating surgeon felt that the lesion was unlikely to be a myxoma due to its firm consistency and local infiltrative nature. Complete excision could not be possible, hence multiple biopsies were taken and the tissue was sent for histopathological evaluation. After the biopsy, interatrial septum was closed in two layers, adequate homeostasis was achieved and routine skin and sternal closure was done. In the postoperative period, the patient was discharged on the 10th day with an advice for regular follow-up.


  Discussion Top


Thefirst primary cardiac tumor was reported by Columbus in 1559, and cardiac myxoma wasfirst described by King in 1845. Cardiac tumor wasfirst diagnosed by clinical examination and electrocardiography in 1934. In thefirst two decades, until the 1950s, cardiac tumors remained merely a curiosity. More recently, with the development of echocardiography, CT, and magnetic resonance imaging (MRI), preoperative diagnosis is possible at least in some cases. Since then, a large variety of benign and malignant primary cardiac tumors have been reported.[1],[2]

Incidence

Primary cardiac tumors are rare with an overall incidence ranging from 0.001%–0.2%. Approximately ¾th of them are benign and cardiac myxomas being the commonest. Malignant tumors are rare and constitute mainly metastasis from other distant sites or local infiltration from the adjacent malignant tumor than the primary ones.[3] Among the primary malignant tumors, cardiac sarcomas are the most common with a female preponderance (2:1).[4]

Manifestations

Cardiac neoplasms often remain clinically silent until they reach an advanced stage, thereby limiting the therapeutic options for the majority of these patients, especially those with malignant tumors. Patients with cardiac tumors can present with a variety of symptoms such as dyspnea, atypical chest pain, or congestive heart failure. The severity of clinical symptoms is due to the size and site of tumor origin.

Due to the slow and asymptomatic growth and lack of specific biochemical markers, imaging methods play an important role in the diagnosis of cardiac tumor. Chest radiographs are often abnormal and trigger the suspicion leading to further evaluation; however, they are seldom diagnostic. They are more likely to demonstrate anatomic changes that reflect hemodynamic consequences of the tumor. Echocardiography is helpful in reaching a diagnosis, demonstrating a cardiac mass, while transesophageal echocardiography is used to differentiate LA masses from thrombi. CT scanning remains an important adjunct to echocardiogram in the diagnosis of these patients. The tumor may be visible on contrast-enhanced scans and in addition it is able to evaluate the mediastinum and lungs for the presence of metastasis. MRI and magnetic resonance angiography have shown to be useful in assessing contiguous extracardiac involvement in metastatic disease.[5]


  Classification Top


The primary cardiac tumors are derived either from mesenchymal or epithelial origin.[2]

WHO classification of tumors of the heart (2015) is summarized in [Table 1].
Table 1: Classification

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Types of tumors

Metastatic cardiac tumors or direct tumor invasion are more common than primary lesions. Benign cardiac tumors constitute 75%, of which atrial myxomas are the most common.[6] Malignant cardiac tumors constitute approximately 25%, among which angiosarcoma being the most frequent, followed by rhabdomyosarcoma, mesothelioma, fibrosarcoma, malignant peripheral nerve sheath tumor (MPNST), and leiomyosarcoma.[4]

Pathology

The cardiac tumor biopsy specimen was sent in 10% buffered formalin as multiple friable soft-tissue fragments altogether measuring 0.4 cm × 0.3 cm × 0.1 cm. Sections made from paraffin-embedded tissue block showed a malignant tumor composed of spindle-shaped cells with moderate pleomorphism and atypia. The tumor cells were arranged in an intersecting fascicular pattern with focal areas of necrosis and atypical mitosis. The individual tumor cells were plump, spindle-to-round nucleus (epithelioid nature) with moderate amount of eosinophilic cytoplasm. Slit-like vascular channels, hemosiderin deposition, and intracytoplasmic lumina, suggestive of angiosarcoma, were not identified. The tumor was mitotically active with an average mitotic count of 5-10/high power field. A panel of immunohistochemical stains done revealed diffuse immunopositivity for desmin, vimentin and focal positivity for smooth muscle actin. The tumor cells were immunonegative for myogenin, CD31, CD34, S-100, Bcl-2, and Mic-2 [Figure 2]. On the basis of the above histomorphological and immunohistochemical features, a final diagnosis of epithelioid leiomyosarcoma of heart was offered. A thorough systemic examination did not reveal any evidence of primary lesion elsewhere in the body. Hence, the final diagnosis of primary cardiac leiomyosarcoma was considered.
Figure 2: (a) Microscopic examination shows bundles of spindle cells arranged in short interlacing fascicles. (b) On higher magnification, tumor cell showing epithelioid morphology with irregular round nuclear contour with mild-to-moderate cytoplasm and brisk atypical mitosis (arrow). (c) The tumor cells are immunopositive for smooth muscle actin. (d) The tumor cells are diffusely immunopositive for desmin.

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Significance

In the present case, based on light microscopy findings, the differential diagnosis of high-grade spindle cell sarcoma was considered which includes leiomyosarcoma, MPNST, synovial sarcoma, spindle cell rhabdomyosarcoma, and angiosarcoma [Table 2] and [Table 3].
Table 2: Differential diagnosis of spindle cell tumors

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Table 3: Interpretation of immunohistochemical markers

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Cardiac sarcomas

At present, only a few hundred primary cardiac sarcomas have been reported, most of which are based on autopsy series. Similar to their soft-tissue counterparts, cardiac sarcomas constitute a variety of histologic types. Primary cardiac sarcomas can occur at any age but more frequently between the third and fifth decades with a mean age of 41 years. Left-sided cardiac involvement is more common than right-sided involvement. The clinical presentation of patients with cardiac sarcoma depends on the size as well as the location of the tumor and infiltration into the adjacent structures.[7],[8]

Primary leiomyosarcomas

The commonest primary malignant cardiac tumor is angiosarcoma. Epithelioid leiomyosarcoma is an uncommon type of cardiac malignancy with smooth muscle differentiation that constitutes only 8%–9% of all cardiac sarcomas. It occurs commonly in the LA with few reports mentioning its occurrence from the LV, right atria, and the right ventricle. It most likely originates from smooth muscle bundles lining the subendocardium or pulmonary vein and artery. Affected patients commonly present in the fourth decade of life. Unlike myxoma, cardiac leiomyosarcoma usually arise from the posterior wall of the LA and appear as lobulated, irregular, low-attenuation masses.[9]

Preoperative diagnosis of leiomyosarcoma is difficult, a histopathological examination remains gold standard for the diagnosis. Due to the rarity of the lesion, the optimal treatment modality of primary cardiac epithelioid leiomyosarcoma is not known. According to the available literature, it requires a rapid multimodality approach. Cardiac transplantation is a viable option. Surgical resection or debulking followed by adjuvant radiation therapy and/or chemotherapy is fundamental of treatment.[4],[10],[11]

Prognosis

Most of the patients with cardiac sarcoma have a high-grade tumor at the time of diagnosis with distant metastasis in the lungs (35.7%), lymph node (14.2%) followed by liver (7.14%). They carry a poor prognosis.[12]

Following surgical removal, patients may remain well for some time; however, long-term survival remains poor and most patients will die within 1 year of diagnosis.[4],[8] Therefore, immediate and complete surgical resection of the tumor along with adjuvant therapy provides the best optimism for palliation and longer survival outcome.[4],[7] According to some reports, cardiac transplantation for primary cardiac sarcomas does not offer impressive outcome.[11],[13]

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Arava S, Bagmar S, Jain P, Kumaran M, Kumar S, Ray R, et al. Primary intracardiac malignant peripheral nerve sheath tumor: A rare case report. Indian J Pathol Microbiol 2015;58:531-3.  Back to cited text no. 1
[PUBMED]  [Full text]  
2.
Shapiro LM. Cardiac tumours: Diagnosis and management. Heart 2001;85:218-22.  Back to cited text no. 2
    
3.
Engberding R, Daniel WG, Erbel R, Kasper W, Lestuzzi C, Curtius JM, et al. Diagnosis of heart tumours by transoesophageal echocardiography: A multicentre study in 154 patients. European cooperative study group. Eur Heart J 1993;14:1223-8.  Back to cited text no. 3
    
4.
Hattori Y, Iriyama T, Watanabe K, Negi K, Takeda I, Sugimura S, et al. Primary cardiac sarcoma: Two case reports. Jpn Circ J 2000;64:222-4.  Back to cited text no. 4
    
5.
Simpson L, Kumar SK, Okuno SH, Schaff HV, Porrata LF, Buckner JC, et al. Malignant primary cardiac tumors: Review of a single institution experience. Cancer 2008;112:2440-6.  Back to cited text no. 5
    
6.
Patel J, Sheppard MN. Pathological study of primary cardiac and pericardial tumours in a specialist UK centre: Surgical and autopsy series. Cardiovasc Pathol 2010;19:343-52.  Back to cited text no. 6
    
7.
Neragi-Miandoab S, Kim J, Vlahakes GJ. Malignant tumours of the heart: A review of tumour type, diagnosis and therapy. Clin Oncol (R Coll Radiol) 2007;19:748-56.  Back to cited text no. 7
    
8.
Zhang PJ, Brooks JS, Goldblum JR, Yoder B, Seethala R, Pawel B, et al. Primary cardiac sarcomas: A clinicopathologic analysis of a series with follow-up information in 17 patients and emphasis on long-term survival. Hum Pathol 2008;39:1385-95.  Back to cited text no. 8
    
9.
Araoz PA, Eklund HE, Welch TJ, Breen JF. CT and MR imaging of primary cardiac malignancies. Radiographics 1999;19:1421-34.  Back to cited text no. 9
    
10.
Dein JR, Frist WH, Stinson EB, Miller DC, Baldwin JC, Oyer PE, et al. Primary cardiac neoplasms. Early and late results of surgical treatment in 42 patients. J Thorac Cardiovasc Surg 1987;93:502-11.  Back to cited text no. 10
    
11.
Putnam JB Jr., Sweeney MS, Colon R, Lanza LA, Frazier OH, Cooley DA, et al. Primary cardiac sarcomas. Ann Thorac Surg 1991;51:906-10.  Back to cited text no. 11
    
12.
Mayer F, Aebert H, Rudert M, Königsrainer A, Horger M, Kanz L, et al. Primary malignant sarcomas of the heart and great vessels in adult patients – A single-center experience. Oncologist 2007;12:1134-42.  Back to cited text no. 12
    
13.
Vander Salm TJ. Unusual primary tumors of the heart. Semin Thorac Cardiovasc Surg 2000;12:89-100.  Back to cited text no. 13
    


    Figures

  [Figure 1], [Figure 2]
 
 
    Tables

  [Table 1], [Table 2], [Table 3]



 

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