Primitive neuroectodermal tumor of the pericardium: a case report and literature review

Abstract

Background The primitive neuroectodermal tumors (PNETs) are a family of highly malignant tumors with a multidirectional differential potential. The tumors are characterized by aggressive small round tumor cells that originate from the spinal cord of the central and sympathetic nervous systems. Cases involving the pericardium are extremely rare. Herein, we present a case of peripheral primitive neuroectodermal tumor (pPNET) that originated in the pericardium. Case presentation A 23-year-old woman presented with cough and progressive dyspnea for 1 month, followed by eyelid and facial edema for 10 days, without any apparent cause. Significantly elevated tumor markers were detected in her blood. A cardiac ultrasound revealed a 74 mm × 61 mm spherical mass that was attached to the left pericardium, as well as massive pericardial effusion. Positron emission tomography-CT (PET-CT) showed focal hypermetabolism in the left pericardium. Via histopathology and immunohistochemistry, the spherical mass was identified as PNETS. The patient was successfully treated with a combination of surgical resection via thoracotomy and postoperative chemotherapy, and she was disease-free for 7 years at follow-up. Unfortunately, at 7 years after the treatment, the patient’s pPNET recurred. Positron emission tomography-MRI (PET-MRI) and 64-slice coronary CTA revealed that the aorta and multiple coronary arteries were involved. Subsequently, the patient refused a heart transplant and voluntarily left the hospital. Conclusions This paper reports on a rare and recurrent case of PNET in the parietal pericardium. With respect to the different biologic characteristics and prognoses of pPNETs (compared to other known pericardium tumors), it is essential to consider this entity as a differential diagnosis in pericardium tumors.

A rare radiotherapy-sensitive primitive neuroectodermal tumor with APC gene amplification in an adult: a case report and literature review

“…It is a high-grade malignant tumor with early metastasis and an extremely poor prognosis. PNET can reportedly develop anywhere in the body, including the orbit, nasal cavity, mediastinum, stomach, bone, uterus, kidney, neck, chest, pancreas, gingiva, paraspinal region, ovaries, breast, testes, and thymus; 1 – 4 , 6 – 9 however, it is rarely found in the lung. Only 15 related cases were retrieved from the literature from 2012 to 2022 ( Table 1 ).…”

“…As a member of the Ewing sarcoma family, PNET can be classified into central or peripheral subtypes, both of which can affect organs other than those in the neurological, soft tissue, and skeletal systems in children and adolescents. 1 – 4 The diagnosis of PNET largely depends on pathological and molecular examinations. Given the rarity and extremely poor prognosis of PNET with pulmonary invasiveness, 1 we believe that it is clinically valuable to report the clinical features, treatments, follow-up procedures, histological characteristics, and genomic variants of such cases to expand the knowledge of this disease.…”