A 16-year-old student presented with a 4-week history of progressive shortness of breath, loss of appetite, and occasional blood-tinged sputum. The chest X-ray revealed massive right-sided pleural effusion with cardiomegaly. An echocardiogram revealed a large pericardial mass with massive pericardial effusion. Subsequent computed tomography of the thorax revealed a large heterogeneous mass in the right lung with extension into the pericardium. Lung biopsy revealed primitive neuroectodermal tumor (PNET) with small round blue cells, Homer-Wright rosettes, and CD99 positivity. We discuss pericardial metastases of PNET and its implication in this patient.
Ewing's sarcoma is a rare tumor first discovered by James Ewing in 1921. It is more common in bone or skeletal component compared to soft tissue or extraosseous Ewing's sarcoma. Among soft tissue Ewing’s, spinal cord involvement is rarer with only nine cases reported. We report a case of nine-year-old Malay girl who presented with low back pain for two months following a fall with progressive neurological deficits of bilateral lower limb. Magnetic resonance imaging was suggestive of a well-defined margin of intradural extramedullary tumor. With nerve sheath tumor in mind, surgical excision with laminectomy L2-S1 was performed. Intraoperative finding was an extradural mass from L3-L5 with extension to bilateral neuroforamen. Histopathology report defined a round cell tumour of Ewing’s sarcoma from the mass.
Epigenetics is the study of heritable changes in gene expression that do not involve changes to the underlying DNA sequence. It is a change in phenotype without changing in genotype which in turn affects how cellsread the genes. The epigenetic change is a regular occurrence but can also be influenced by several factors including age, environment, lifestyle, and disease state. It may have damaging effects that result in diseases like cancer. At least three systems including DNA methylation, histone modification and RNA-associated gene silencing are currently considered to initiate and sustain epigenetic change. New and ongoing research is continuously uncovering the role of epigenetics in a variety of diseases including in childhood solid cancer such as Ewing sarcoma, neuroblastoma, Wilms tumours, brain tumours and rhabdomyosarcoma. A better understanding of epigenetic changes in childhood cancers can guide towards future therapy and diagnosis.