Eur J Cardiothorac Surg 2000;18:619-621
© 2000 Elsevier Science NL
The primitive neuroectodermal tumor of the heart
Kaz
m Be
irlia,
Caner Arslana,
Hasan Tüzüna,
Büge Özb
a Department of Thoracic and Cardiovascular Surgery, Cerrahpa
a Faculty of Medicine, University of 
stanbul, stanbul, Turkey
b Department of Pathology, Cerrahpaa Faculty of Medicine, University of stanbul, stanbul, Turkey
Received 11 April 2000;
received in revised form 25 July 2000;
accepted 15 August 2000.
Corresponding author. Tel.: +90-212-633-2991; fax: +90-212-632-8474
e-mail: kamil{at}edu.istanbul.tr
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Abstract
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A young man was admitted to hospital with dyspnea, malaise, chest pain and night sweating. Investigative studies revealed a cystic mass lesion originating from the heart. Surgical exploration of the tumor showed that it was unresectable and pathology of the biopsy material was primitive neuroectodermal tumor. Medical literature concerning this unusual type of tumor is reviewed.
Key Words: Primitive neuroectodermal tumor • Heart
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1. Introduction
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Primitive neuroectodermal tumor (PNET) derives from a carcinogenic alteration of pluripotent neural crest cells caused by a balanced reciprocal translocation t (11;22) (q24;q12). It is the second most common type of sarcoma in the first 2 decades of life. It is typically seen in the soft tissues of the chest wall and paraspinal region and rarely presents as an organ based neoplasm. A 31-year-old man who was first examined for constitutional symptoms exhibits a case of cardiac primitive neuroectodermal tumor. In this report, management of patient and literature regarding this unusual type of cardiac tumor is reviewed.
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2. Case report
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A 31-year-old man was admitted to Cerrahpaa Medical Faculty Hospital on October 15, 1998 with the history of dyspnea on exertion, malaise, night sweating, left lateral chest pain and weight loss. One year ago, he had been treated for pulmonary tuberculosis. His above mentioned complaints had begun 3 months before admission to hospital and aggravated during last month and he lost 3 kg of weight. On physical examination, his arterial tension was 90/60 mmHg, pulse rate, 80/min. He had no fever, lenfadenopathy and organomegaly. Faintness of the heart sounds and feebleness of cardiac impulse were noted. There was no abnormality on blood and urine chemistry. Chest X-ray showed enlarged heart shadow, enlargement being greater in the transverse diameter than longitudinal with a short vascular pedicle and the electrocardiogram had low voltage. Pericardial effusion was suspected and transthoracic echocardiography revealed a mass lesion with cystic septations compressing the right atrium in addition to large volume of fluid around the heart.
There was no finding suggesting cardiac tamponade. Computerized tomography (CT) of the chest revealed massive pericardial effusion and non-homogenous mass lesion containing cystic and solid spaces and originating from right side of the heart, compressing the superior vena cava. It was reaching 11x8x9 cm in dimensions (Fig. 1)
. Cardiac tumor was taken into consideration. Cystic spaces in the mass reminded us cardiac hydatic cyst but, enzyme-linked immunosorbent assay (ELISA) IgM-IgG and indirect hemagglutination test for echinococcus granulosus was negative and there was no pathology on abdomino-pelvic CT. Whole body bone scintigraphy was normal. Surgical intervention was performed to evaluate operability and absolute diagnosis. After median sternotomy, pericardium was opened left to the midline and nearly 1.5 l of hemorrhagic defibrinated fluid was aspirated from the pericardial cavity. On the right side, tumoral mass originated from the right atrium, occupying the space between the aortic root and the atrioventricular groove was seen. The tumor was solid towards the interatrial groove and cystic in front. It was 10–15 cm. in diameter. Adhesions to the pericardium were separated by blind and sharp dissections. Both serous and bloody fluid were aspirated from the cytic parts of the mass located anteriorly. For pathologic examination 1 cm cyst wall was excised and the defect was closed with a pericardial patch. Frozen section examination revealed a malign tumor with round cells. Tumor was accepted as unresectable and operation was ended.
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Fig. 1. Computerized tomography of the chest, massive pericardial effusion and non-homogenous mass lesion containing solid and cystic spaces are seen.
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On pathological examination of the biopsy material, sheets of primitive round cells and formation of Homer–Wright type rosettes were principal microscopic features (Fig. 2)
. Immunohistochemically, the tumor cells were intensely positive for neuron specific enolase and MIC-2 gene product and were consistently negative for leukocyte common antigen, cytokeratin (CAM 5, 2) and smooth muscle actin and ß human chorionic gonadotropin. Balanced reciprocal translocation, t (11;22) (q24;q12), was demonstrated on chromosomal analysis of the tumor cells. The diagnosis was primitive neuroectodermal tumor.
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Fig. 2. Histologic features of the small round tumor cells. Tumor cells have small prominent nucleoli and very scanty cytoplasm. Note rosette formation and cellular necrosis. (Hematoxylin & eosin x100).
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Chemotherapy was planned but the patient refused any further treatment and was discharged from the hospital with his own wish. Although our patient had refused additional treatments, he was still alive, had no respiratory distress and his heamodynamics was stable and no dissemination was seen on physical examination at out patient clinic after 17 months from his discharge. Pericardial effusion recurred at two times during this period and drainage was performed under echocardiography at another institution. He didn't accept radiologic examinations and was lost for follow-up.
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3. Discussion
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In the PNETs of chest wall, retroperitoneum and paravertebral region, after biopsy proven diagnosis, primary chemotherapy which in great portion of cases leads to remission, allows excision of remainder of the tumor without mutilation and avoidance of intraoperative tumor cell dissemination [1]. Some authors have been using cytokine treatment (experimental) and autologous stem cell in addition to chemotherapy and radiotherapy to patients with metastatic PNET of kidney and taking good results [2]. PNETs of hand, kidneys, retroperitoneum, bone, uterus, lung and are reported, [3–6]. Only one cardiac case report was seen in literature review. In this case diagnosis of PNET was made on endomyocardial biopsy and confirmed in sections from myocardial tumor found within the heart explanted during cardiac transplant. In 1-year follow up, no malignancy was seen in this patient [7]. We could not have any chance of further treatment and follow up longer than 17 months in our case.
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References
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- Zimmermann T., Blutters S.R., Berghauser K.H., Cristiansen H., Padberg W. Der primitive neuroektodermale tumor. Chirurg 1997;68:732-737.[Medline]
- Marley E.F., Liapis H., Humphry P.A., Nadler R.B., Siegel C.L., Zhu X., Brandt S.M., Dehner L.P. Primitive neuroectodermal tumor of the kidney another enigma: a pathologic, immunohistochemical and molecular diagnostic study. Am J Surg Pathol 1997;21:354-359.[Medline]
- Daw J.L., Wiedrich T.A., Bauer B.S. Congenital primitive neuroectodermal tumor of the hand: a case report. J Hand Surg Am 1997;22:743-746.[Medline]
- Fukumoto S., Takamuro K., Nakanishi K., Yamoto M., Inaba S., Makimura S., Yoshikawa T., Terai T. Peripheral primitive neuroectodermal tumor involving paravertebral and retroperitoneal regions. Int Med 1997;36:424-429.
- Fragetta F., Magro G., Vasquez E. Primitive neuroectodermal tumor of the uterus with focal cartilaginous differentiation. Histopathology 1997;30:483-485.[Medline]
- Catalan R.L., Murphy T. Primitive neuroectodermal tumor of the lung. Am J Roentgenol 1997;169:1201-1202.[Medline]
- Charney D.A., Charney J.M., Ghali V.S., Teplitz C. Primitive neuroectodermal tumor of the myocardium: a case report, review of the literature, immunohistochemical and ultrastructural study. Hum Pathol 1996;27:1365-1369.[Medline]
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Abstract
1. Introduction
