Primitive Neuroectodermal Tumor of the Heart

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  • Primitive Neuroectodermal Tumorof the HeartNnHu











    Puamdi Nwaejike, MD, Doris Rassl, FRCPath,go Ford, MD, and Stephen R. Large, FRCS

    partments of Cardiothoracic Surgery, Histopathology, andcology, Papworth Hospital, Cambridge, United Kingdom

    e present a case of primitive neuroectodermal tumor ofleft atrium with involvement of the coronary sinus.e initial presentation was of cardiac tamponade result-from the size of the tumor. There was no evidence ofor elsewhere, and after complete resection and with-

    t adjuvant chemotherapy the patient is well at 2-yearlow-up. There has been no evidence of tumor recur-ce. This is a rare reported case of resection of a cardiacimitive neuroectodermal tumor without adjuvant che-therapy. Other cases in the literature have beenated by orthoptic transplantation and resection withemotherapy.

    (Ann Thorac Surg 2012;93:e279) 2012 by The Society of Thoracic Surgeons

    rimary cardiac tumors are rare; the incidence rangesfrom 0.001% to 0.28%. Malignant tumors account for

    % of primary cardiac tumors [1]. Primary primitiveuroectodermal tumor (PNET) derives from carcino-nic alteration of pluripotent neural crest cells andely presents as an organ-based neoplasm [2]. Weesent a rare case of primary PNET of the heart.

    42-year-old woman presented with a 6-week history ofogressively worsening breathlessness, abdominal dis-tion, and peripheral edema. Eighteen weeks earliere had delivered a healthy baby by cesarean section.e had had 3 previous pregnancies (1 termination ofegnancy, 1 miscarriage, and 1 cesarean section). Shes taking sertraline 100 mg (Zoloft) for postnatal de-ession and lansoprazole 15 mg (Prevacid) for gastro-phageal reflux disease. She gave up smoking in 1993pack years smoking history) and her mother had

    east cancer at age 40 years. There was no other historymalignancy in her immediate family. Echocardiogra-y demonstrated a 7 cm 8 cm mass behind the heart,large pericardial effusion, and good left ventricularction. Two liters of blood-stained pericardial fluidre drained by pericardiocentesis. The exudate had aotein content of 36 g/dL and cytologic examinationealed mesothelial cells and no malignant cells. Car-c magnetic resonance imaging confirmed a 7 cm 8mass adherent to the posterior wall of the heart.

    ere was obstruction of the inferior vena cava (Fig 1).hole-body computed tomography showed no tumorewhere. This solid seemingly encapsulated mass washerent to the first 2 cm of the coronary sinus andpeared to arise from the posterior free wall of the leftium.Cardiopulmonary bypass was achieved by ascendingrtic arterial return and superior vena cava and right

    epted for publication Aug 15, 2011.

    dress correspondence to Dr Large, Department of Cardiothoracicgery, Papworth Hospital, Papworth Everard, Cambridge CB38RE,ited Kingdom; e-mail:

    2012 by The Society of Thoracic Surgeonsblished by Elsevier Incoral venous drainage. With appropriate cardiac pro-tion the inferior vena cava was clamped and discon-cted, and division of the right superior and inferiorlmonary vein pair afforded good access to the poste-r aspect of the heart. A vascular, nonhomogeneousss was seen to be densely adherent to the posteriorll of the left atrium and the coronary sinus. It was notached to the pericardium nor was there any macro-pic evidence of pericardial or epicardial involvement.tisfactory clearance of the tumor was achieved. Theronary sinus was reconstructed with an oval patch ofvine pericardium (6 cm 2cm) using 5-0 polypropyl-e, as was the left atrium (4 cm 4 cm square bovinericardial patch) (Supple Peri-Guard, Bio-Vascular, Inc,int Paul, MN). The final tumor clearance was satisfac-y and the inferior vena cava and right pulmonary veinir were reattached. The pericardial space was washedt with water in an attempt to remove any remaininglignant cells. The patient made an uneventful recov-and was discharged on the sixth postoperative day for

    cologic review. A postoperative magnetic resonanceage confirmed tumor clearance and no evidence ofor involvement elsewhere (Fig 2).

    The tumor infiltrated the myocardium and was com-sed of solid sheets of atypical cells with vesicularclei, variably visible small nucleoli, and eosinophilictoplasm. Mitoses were seen and there was focal tumorcrosis. An associated brisk lymphocytic infiltrate wasesent in places. No evidence of gland formation, mucinoduction, or keratinization was apparent. Immuno-ining revealed the tumor to be focally positive forncytokeratin (MNF116) (Fig 3), and diffuse staining forentin was also present. Positive staining was appar-

    t for the neuroendocrine marker PGP9.5 (Fig 4) andally for synaptophysin, but other stains for neuroen-crine differentiation, including chromogranin, neuron-ecific enolase (NSE), and CD56 appeared negative. Theor was also negative for lymphoid markers (leuko-

    te common antigen [LCA], CD3, CD20, CD79a, CD30,15), BCL-2, CD1a, endothelial markers (CD31, CD34,n Willebrand factor [VWF]), estrogen receptor, smoothscle actin, epithelial membrane antigen, TTF-1, otherithelial markers (AE1/AE3, BerEP4, AUA1, CK5/6,7, and CK20), mesothelial markers (calretinin, WT1),lanocyte markers (S100 and HMB45), and germ cellrkers (human chorionic gonadotropin [HCG], placen-alkaline phosphatase [PLAP], -fetoprotein [AFP]).68 stained infiltrating macrophages, but the tumor

    1. Cardiac magnetic resonance image showing the mass com-ssing the posterior heart wall.


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    e28 CASE REPORT NWAEJIKE ET AL Ann Thorac SurgPRIMITIVE NEUROECTODERMAL TUMOR OF THE HEART 2012;93:e279ls did not stain. Further immunostaining revealedriable positivity for CD99.In view of the immunoprofile, including variable pos-ity for CD99, the possibility of a primitive neuroecto-rmal tumor was considered and a block of formalin-ed paraffin-embedded tumor tissue was sent fortogenetic analysis to Great Ormond Street revealed an EWSR1 rearrangement on fluorescencesitu hybridization analysis, and an EWS-FLII type IInscript was detected by real-time polymerase chainction analysis, findings consistent with a diagnosis ofET. Analysis for the SS18 rearrangement was negative,cluding synovial sarcoma.


    e present a case of primary PNET of the heart success-ly treated by resection alone.After surgical resection the patient was free of recur-ce after a 2-year surveillance and remains so to date.mor has never been found elsewhere and we proposet this represents a primary PNET of the left atrial freell with involvement of the coronary sinus.

    2. Postoperative cardiac magnetic resonance image showing ap-rance after resection of mass.wares3. Slides stained with MNF116. (Original magnification 100.)Primary cardiac tumors are rare; the incidence rangesm 0.001% to 0.28%. Malignant tumors account for 25%primary cardiac tumors [1]. The most frequent primarylignant cardiac tumors are angiosarcomas.Primitive neuroectodermal tumor derives from carci-genic alteration of pluripotent neural crest cellsough a balanced reciprocal translocation t(11;22)(q24;2) [2]. It rarely presents as an organ-based neoplasmd is typically seen in the soft tissues of the chest walld paraspinal region [2]. There is no clear guidance ontreatment of PNET of the heart and we could find

    ly 1 other report of successful resection of the primaryor with no adjuvant therapy [3]. Other management

    tcomes include 1 unresectable tumor [2], 1 case ofatment by chemotherapy [4], and 2 cases of treatmentorthoptic transplantation [5, 6].We acknowledge the possibility that this could be adiac metastasis behaving like a primary lesion be-se primary tumors can undergo spontaneous regres-n after treatment of metastases [7]. In a 10-year-periodiew of 18,751 postmortem examinations, Bussani andociates [1] discovered 7,289 cases (39%) of previouslydiagnosed malignant neoplasm. Six hundred andenty-two of these showed evidence of cardiac metas-is (9.1% of malignant tumors). However the heart wasnd to be the only target of metastasis in 10 cases/7,289 0.14%). The highest rates of cardiac metasta-are seen from pleural mesothelioma (48.4%), mela-

    ma (27.8%), lung adenocarcinoma (21%), undifferenti-d carcinoma (19.5%), lung squamous cell carcinoma.2%), and breast carcinoma (15.5%) [1].n our patient, 2-year follow-up with oncologic reviews shown no evidence of recurrence of local tumor or oflate primary tumor. Follow-up was initially every 6eks then every 6 months, and considering the sideects of adjuvant chemotherapy on her young familyd the complete surgical resection, it was decided tonsider adjuvant chemotherapy only if there were anyns of recurrence. We thus conclude that this tumor

    4. Slides stained with PGP9.5. (Original magnification 50.)s a primary PNET of the heart successfully treated byection alone.

  • References

    1. Bussani R, De-Giorgio F, Abbate A, Silvestri F. Cardiacmetastases. J Clin Pathol 2007;60:2734.

    2. Besirli K, Arslan C, Tuzun H, Oz B. The primitive neuroecto-dermal tumor of the heart. Eur J Cardiothorac Surg 2000;18:61921.

    3. Kath R, Krack A, Schneider C, Katenkamp D, Hoffken K.Cardiac manifestations of peripheral primitive neuroectoder-mal tumor (pPNET): a rare case. Dtsch Med Wochenschr2000;125:11924.

    4. Rajappa S, Gundeti S, Varadpande L, Bethune N, Rao S,Digumarti R. Cardiac primitive neuroectodermal tumor pre-

    senting as acute coronary syndrome. J Clin Oncol2007;25:44951.

    5. Charney DA, Charney JM, Ghali VS, Teplitz C. Primitiveneuroectodermal tumor of the myocardium: a case report,review of the literature, immunohistochemical, and ultra-structural study. Hum Pathol 1996;27:13659.

    6. Chai Y, Huang L, Yue L. Peripheral primitive neuroectoder-mal tumour of left ventricular wall origin: a rare case. ActaCardiol 2007;62:5234.

    7. Fairlamb DJ. Spontaneous regression of metastases of renalcancer: a report of two cases including the first recordedregression following irradiation of a dominant metastasis andreview of the world literature. Cancer 1981;47:21026.


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