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  Table of Contents    
CASE REPORT  
Year : 2015  |  Volume : 58  |  Issue : 4  |  Page : 531-533
Primary intracardiac malignant peripheral nerve sheath tumor: A rare case report


1 Department of Pathology, All India Institute of Medical Sciences, New Delhi, India
2 Department of Medical Oncology, All India Institute of Medical Sciences, New Delhi, India
3 Department of Cardio-thoracic Surgery, All India Institute of Medical Sciences, New Delhi, India
4 Department of Cardiac Radiology, All India Institute of Medical Sciences, New Delhi, India

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Date of Web Publication4-Nov-2015
 

   Abstract 

Myxomas are the most common benign cardiac tumors constituting approximately 75% of all the cardiac tumors. Rest 25% are malignant and sarcomas being the commonest. Among the sarcomas primary cardiac malignant peripheral nerve sheath tumors are extremely rare. They usually arise in relation to the branches of vagus or phrenic nerves, 5-42% being associated with neurofibromatosis type 1. Clinical signs and symptoms depend on the location and extent of involvement. Complete resection is the treatment of choice but local recurrence is common.

Keywords: Left atrium, malignant peripheral nerve sheath tumor, primary cardiac sarcoma

How to cite this article:
Arava S, Bagmar S, Jain P, Kumaran M, Kumar S, Ray R. Primary intracardiac malignant peripheral nerve sheath tumor: A rare case report. Indian J Pathol Microbiol 2015;58:531-3

How to cite this URL:
Arava S, Bagmar S, Jain P, Kumaran M, Kumar S, Ray R. Primary intracardiac malignant peripheral nerve sheath tumor: A rare case report. Indian J Pathol Microbiol [serial online] 2015 [cited 2019 Nov 15];58:531-3. Available from: http://www.ijpmonline.org/text.asp?2015/58/4/531/168867



   Introduction Top


Primary cardiac tumors are rare with an overall incidence of 0.001–0.28%.[1] Approximately 75% of these are benign, of which myxomas being the commonest. Remaining 25% are malignant and nearly all of them are sarcomas.[2] Amongst the sarcomas, malignant peripheral nerve sheath tumor (MPNST) of the heart is extremely rare with uniform fatality. Till now only few cases have been reported in the literature. Due to its rarity, exact incidence, management and prognosis of this tumor is not clear. Hence, this case has been highlighted because of its rarity and occurrence in a young female.


   Case Report Top


An 18-year-old female with no significant past medical history presented with an eight months history of gradual onset of exertional dyspnea, palpitations, chest pain, low-grade fever and weight loss. She started to experience dyspnea and palpitations even on rest. She denied any orthopnea or paroxysmal nocturnal dyspnea. Clinical examination revealed signs of raised jugular venous pressure and ankle edema. The area of cardiac dullness was expanded. There were no stigmata of neurofibromatosis type 1.

Chest X-ray showed cardiomegaly with an oligemic right lung field. Electrocardiogram (EKG) was suggestive of right ventricular hypertrophy. Transthoracic echocardiography (TTE) demonstrated large left atrial (LA) mass ~ 6 cm × 4 cm × 3 cm, wide based attached to posterior LA wall and posterior mitral valve (MV) leaflet. It was almost obstructing MV inlet with dilated LA, mild pulmonary regurgitation, severe tricuspid regurgitation (TR), severe pulmonary arterial hypertension and normal left ventricular function (LVF). Computerized tomography (CT) scan of the thorax, [Figure 1]a confirmed a 2.1 cm × 1.9 cm predominantly calcified lesion along posterior superior LA wall protruding into LA cavity and showing mild heterogeneous enhancement. Magnetic resonance imaging (MRI) of the heart showed the same findings as seen on CT scan [Figure 1]b. Other cardiac chambers, myocardium, pericardium were normal with no evidence of any pleural or pericardial effusions. With the suspicion of atrial myxoma, she was taken up for surgery.
Figure 1: (a) Computerized tomography scan shows a 2.1 cm × 1.9 cm predominantly calcified lesion along posterior superior left atrial (LA) wall protruding into LA cavity and showing mild heterogeneous enhancement (b) cardiac magnetic resonance imaging confirmed a 2.1 cm × 1.9 cm predominantly calcified lesion along posterior superior LA wall protruding into LA cavity and showing mild heterogeneous enhancement. Other cardiac chambers, myocardium, pericardium were normal with no evidence of any pleural or pericardial effusion

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Median sternotomy and vertical pericardiotomy were done. Right atrium was opened, and a vertical incision was made on interatrial septum (IAS). IAS was retracted, the soft tissue mass was excised completely and base was cauterized. On follow-up, patient is presently asymptomatic. Postoperative TTE was suggestive of mild mitral regurgitation and mild TR with normal LVF.

Histopathology

Microscopic examination of the tumor showed densely cellular areas with intervening hypocellular and myxoid areas composed of spindle-shaped tumor cells arranged in intersecting fascicles and loose storiform pattern. These cells have wavy nuclei with regular nuclear contour [Figure 2]a. Areas showing brisk atypical mitosis with necrosis was also identified [Figure 2]b. The proliferation index (Ki 67) of the tumor at the highest proliferating area was approximately 20 percent [Figure 2]c. To differentiate from other spindle cell tumors a panel of immunohistochemical stains comprising of S100, neurofilament, desmin, myogenin, CD31, smooth muscle actin, epithelial membrane antigen and Pancytokeratin were done. As the tumor showed focal nuclear immunopositivity for S100 [Figure 2]d with background neurofilament positivity and negative for all other above mentioned immunomarkers, a final diagnosis of MPNST was made.
Figure 2: Malignant peripheral nerve sheath tumor heart: (a) Malignant spindle cell tumor with brisk mitotic figures and (b) areas of necrosis. The tumor has (c) high proliferative index with (d) occasional cells showing S100 nuclear positivity

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   Discussion Top


Primary cardiac sarcomas are defined as tumors that are confined to the heart or pericardium with no evidence of extra cardiac primary neoplasm. MPNST is an aggressive neoplasm of neural origin occurring typically in the age group of second to fifth decade.[3] Usually, these tumors arise in association with major peripheral nerve trunks. Common sites being proximal limb, trunk, and head and neck region.[4] In cardiac cases, it originates from the cardiac plexus, and from the branches of vagus or phrenic nerves.[5] They can either arise de-novo or from an existing neurofibroma or schwannoma. 5–42% of the cases are associated with neurofibromatosis type 1 (NF).[6] Clinical signs and symptoms depend on the location and extent of involvement. Symptoms do not develop until the tumor interferes with cardiac function. Common symptoms reported in the literature are dyspnea, fatigue, fever, cough, chest pain, anorexia, weight loss, arrhythmia and peripheral embolism.[5] Signs of superior vena caval obstruction and congestive cardiac failure have also been reported.

Chest X-ray findings may be normal but more usually cardiomegaly, abnormalities of cardiac contour, pleural effusion and signs of heart failure are evident. EKG may be entirely normal. Heart block, arrhythmia, and ST-T wave changes indicate myocardial invasion and damage. Diagnosis is greatly facilitated by echocardiography, CT and transesophageal echocardiography. High contrast, spatial resolution, and multiplanar capabilities allow MRI to localize the tumor precisely, including its relationship with cardiac chambers, contiguous structures and myocardial involvement. MRI is especially important in determining the resectability and surgical approach.[5]

Histopathologically, these tumors have to be differentiated from other malignant spindle cell tumors such as leiomyosarcoma, fibrosarcoma, solitary fibrous tumor, monophasic synovial sarcoma, angiosarcoma, Spindle cell melanoma, and spindle cell variant of carcinoma and mesothelioma.[7],[8] A panel of antibodies that either eliminate the other possibilities or provide additional evidence of nerve sheath differentiation are helpful in the final diagnosis. Before considering it as primary tumor, all possible causes of metastasis should be ruled out by thorough clinical and radiological evaluation. Though most of the cases of MPNST are associated with neurofibromatosis type 1 (NF1), it was not true for our case.

Due to its rarity, general strategies for management and prognosis of such tumors are not clear. Like sarcomas in any other location, resection should be the procedure of choice.[8] All reported series on cardiac sarcomas claimed improved survival after complete resection. Local recurrence is common even after complete excision of the tumor. Aggressive surgery significantly improves disease-free survival. Adjuvant chemotherapy and radiotherapy do not prolong patient survival, but it is effective as a palliative option. The prognosis of MPNST is poor, with survival being influenced by tumor size, location, and association with NF-1 mutation. Median survival ranges from 3 months to 1-year and prognosis correlates with tumour grading but not histological type.[9]


   Conclusion Top


Primary MPNST of the heart is extremely rare. Clinical symptoms and signs depend on the site, location and extent of involvement. CT and high-resolution MRI are helpful for exact localization of the tumor and to determine the surgical resectability. As histopathology reveals malignant spindle cell tumor, a panel of immunohistochemical stains is necessary to differentiate it from others. Surgical resection with adjuvant chemo-radiotherapy will improve the patient survival.

Acknowledgements

We acknowledge Dr. Sameer Bhakhshi and Dr. Soumya Ranjan Mallick for their contribution in the patient care, diagnosis and preparation of the manuscript.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
   References Top

1.
Valeviciene N, Mataciunas M, Tamosiunas A, Petrulioniene Z, Briediene R. Primary heart angiosarcoma detected by magnetic resonance imaging. Acta Radiol 2006;47:675-9.  Back to cited text no. 1
    
2.
Rahman M, Cook DS, Ellis G, O'keefe PA. Malignant peripheral nerve sheath tumor of the heart. Asian Cardiovasc Thorac Ann 2006;14:425-7.  Back to cited text no. 2
    
3.
Enzinger FM, Weiss SW. Soft Tissue Tumors. 3rd ed. St. Louis: Mosby; 1995. p. 889-900.  Back to cited text no. 3
    
4.
Panigrahi S, Mishra SS, Das S, Dhir MK. Primary malignant peripheral nerve sheath tumor at unusual location. J Neurosci Rural Pract 2013;4:S83-6.  Back to cited text no. 4
    
5.
Morishita T, Yamazaki J, Ohsawa H, Uchi T, Kawamura Y, Okuzumi K, et al. Malignant schwannoma of the heart. Clin Cardiol 1988;11:126-30.  Back to cited text no. 5
    
6.
D'agostino AN, Soule EH, Miller RH. Sarcomas of the peripheral nerves and somatic soft tissues associated with multiple neurofibromatosis (Von Recklinghausen's Disease). Cancer 1963;16:1015-27.  Back to cited text no. 6
    
7.
Putnam JB Jr, Sweeney MS, Colon R, Lanza LA, Frazier OH, Cooley DA. Primary cardiac sarcomas. Ann Thorac Surg 1991;51:906-10.  Back to cited text no. 7
    
8.
Pauwels P, Dal Cin P, Sciot R, Lammens M, Penn O, van Nes E, et al. Primary malignant peripheral nerve sheath tumour of the heart. Histopathology 1999;34:56-9.  Back to cited text no. 8
    
9.
Swanson PE, Scheithauer BW, Wick MR. Peripheral nerve sheath neoplasms: Clincopathologic and immunochemical observations. In: Rosen PP, Fechner RE, editors. Pathology Annuel, Part 2. Vol. 30. Connecticut: Appleton and Lange; 1995. p. 1-82.  Back to cited text no. 9
    

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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: 10.4103/0377-4929.168867

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