Cardiac Primitive Neuroectodermal Tumor (PNET) with Metastasis at Presentation

Case Report

Ann Hematol Oncol. 2017; 4(6): 1156.

Cardiac Primitive Neuroectodermal Tumor (PNET) with Metastasis at Presentation

Jawahar A*, Hibbeln J and Goldberg A

Department of Radiology, Loyola University Medical Center, USA

*Corresponding author: Jawahar A, Department of Radiology, Loyola University Medical Center, 2160, South 1st Avenue, Maywood, Illinois-60153, USA

Received: April 25, 2017; Accepted: May 31, 2017; Published: June 22, 2017


Primitive neuroectodermal tumors (PNET) are rare tumors and PNET of cardiac origin are extremely rare, with only 6 cases reported since 1996. Cardiac PNET cases have been associated with poor prognosis with extensive metastasis at presentation, and warrant aggressive treatment.

We present a 19-year-old male patient who presented with headache and falls; workup demonstrated cerebellar lesions which were resected and noted to be metastatic PNET, and his preoperative transthoracic echocardiography showed an irregular cardiac mass protruding into the interventricular septum which was later identified as the primary malignant mass. Despite the mass being of the cardiac primary origin, he did not have cardiac symptoms until 15 months after his initial symptoms.

Keywords: PNET; Cardiac tumors; Metastasis; Neuroectodermal tumors; Cardiac transplantation


Cardiac PNET is an extremely rare tumor, with only 6 cases reported in literature to date since it was first described by Charney, et al. in 1996 [1]. Cardiac PNETs have poor prognosis with extensive metastasis at presentation, and have been felt to warrant aggressive treatment with chemotherapy and radiotherapy, with limited surgical options [2-4]. Though recurrences have been commonly reported, there have been two cases described in which complete resection of tumor in patients without extracardiac or metastatic spread was shown to be beneficial, without recurrence in subsequent short follow up. The role of imaging is to identify the primary tumor, its invasion into its surrounding structures, and metastatic foci which can make these patients non-surgical candidates, more appropriately treated with chemotherapy and radiotherapy.

Case Presentation

A 19-year-old male presented to our institution in November 2015 with complaints of worsening headache for 2 months and subsequent imbalance with falls. A CT head (Figure 1A,B) showed three ill-defined cerebellar lesions suspicious for malignancy and underwent craniotomy with excision of the masses with negative margins. Immunohistochemical stains of the lesions were positive for cytokeratin CAN 5.2, GFAP, vimentin and CD56, and negative for translocation characteristic of synovial sarcoma. Histopathology was reported as metastatic primitive neuroectodermal tumor and he received post-operative radiotherapy for 3 weeks. Preoperative transthoracic echocardiography (Figure 2A) showed an approximately 5 cm irregular mass involving and protruding from the interventricular septum, with involvement of both ventricles. The uninvolved myocardium contracted normally; left ventricular ejection was 55%. Cardiac MRI (Figure 2B, 2C, 2D) confirmed a 5.2x4.9 cm irregular mass infiltrating the mid interventricular septum with extension into both ventricular cavities. The mass was isointense on T1W images, hyperintense on T2W images, and showed robust perfusion on T1 post-contrast imaging. Delayed hyperenhancement was also seen. No obvious obstruction of cardiac blood flow was noted.