Isolated cardiac peripheral primitive neuroectodermal tumor: A case report

Abstract

Background: Peripheral primitive neuroectodermal tumor isolated in the heart, presenting as a primary cardiac tumor is considered as extremely rare.

Methods: We present a 53-year-old Chinese female with a cardiac tumor which was discovered by CT.

Results: A hypo-intense tumorous mass was shown extending from the left ventricle by Cardiac CT, and fused FDG positron emission tomography demonstrated no other abnormal FDG active lesions in the body. We performed a total resection surgery of the tumor subsequently and the patient recovered well and discharged from hospital 6 d after surgery.

Conclusion: The pathological diagnosis was primary cardiac peripheral primitive neuroectodermal tumor. No tumor recurrence was shown by echocardiography during the 24 months follow-up visits.

Keywords: Cardiac tumor; computed tomographymalignant; no recurrence; pericardial effusion; peripheral primitive neuroectodermal tumor; resection surgery.

Figure 1.

(A) Four chamber view of CT showing a hypo-intense tumorous mass extending from the left ventricle with the coronary artery infiltrating (arrow). FDG PET-CT shows a high uptake lesion (40 mm × 30 mm) located in the posterior-inferior of the left ventricle (arrows in B, (C) and D) with severe focal FDG activity (SUV max 9.0).

Figure 2.

Surgical exploration reveals a firm 40 mm × 30 mm × 30 mm mass with coronary infiltrated.

Figure 3.

Post-operative histological examination showing small cell malignant tumor and highly suspicious of malignant peripheral primitive neuroectodermal tumor with CD99 and NSE intense positive identification. Magnification, A: x100, B: x200.

Figure 4.

MAP kinase pathways and their mammalian ETS-domain transcription factor targets. ERK, p38 and JNK pathways are activated by distinct upstream kinases in response to a wide variety of extracellular signals such as growth factors, cytokine factors, and stress. Various substrates in the cytoplasma and the nucleus of the cell were activated, including transcription factors. These downstream targets control cellular responses.