Radiological Diagnosis of a Rare Prepontine Lesion: Ecchordosis Physaliphora

Ecchordosis physaliphora (EP) is a notochordal remnant tissue rarely encountered during routine clinical practice. These lesions usually do not produce any significant symptoms as they are slow-growing and mostly small in size. Symptoms are due to mass effects on adjacent structures when they are large or extra-tumoral hemorrhage. Because of histological similarity with chordoma, diagnosis is challenging, and this differentiation is essential as the disease course and treatment differ significantly. Imaging plays a crucial role in identifying and distinguishing these lesions. We report the case of a 16-year-old male who presented with intermittent headache and neck pain for six months. His routine clinical examinations were within normal limits. On neurological assessment, there was no focal neurodeficit. Evaluation of cranial nerves did not reveal any evidence of palsy. Routine hematological tests were also normal. A computed tomography (CT) scan of the brain revealed a mass in front of the pons. Magnetic resonance imaging (MRI) for further evaluation revealed a T1 hypointense and T2/fluid-attenuated inversion recovery hyperintense lesion in the pre-pontine cistern. There was no enhancement in the mass either in the post-contrast CT or MRI scans. There was no bony erosion and clivus was normal. Based on the location and characteristic imaging features, a diagnosis of EP was made. There may be several other lesions that may present as a mass in the pre-pontine region. Histopathological tests may find it difficult to distinguish between lesions that originate from notochord remnants. Imaging studies play a vital role in confirming the diagnosis and help in planning treatment and follow-up.