What is notochord remnant?
Remnants of the notochord usually remain near the midline and are entrapped in bone. Chordomas are a malignant tumor that can develop from the notochord remnants. The nucleus pulposus of the intervertebral discs represent residual notochord.
What is notochordal cell tumor?
Benign notochordal cell tumor, or giant vertebral notochordal rest (GVNR), is a new and evolving term used to describe benign notochordal tissue that may produce a macroscopic vertebral lesion, with only a few cases reported in the literature. The distinction between GVNR and cordoma relies mostly on imaging findings.
Can a chordoma be benign?
because they are relatively slow growing and they most often recur locally rather than spreading throughout the body. Because chordomas are low-grade, sometimes they are mistakenly called benign. Benign tumors do not invade other tissue, do not metastasize, and usually do not return after being removed.
What is Ecchordosis Physaliphora?
Ecchordosis physaliphora (EP) is a benign lesion which arises from ectopic notochordal remnants, lying along the midline craniospinal axis from the clivus to the sacrococcygeal region. It has been described as a small, gelatinous nodule, varying in size from a few millimetres to 2 cm, exhibiting a slow growth pattern.
Why is the notochord important?
The notochord is the defining structure of the chordates, and has essential roles in vertebrate development. It serves as a source of midline signals that pattern surrounding tissues and as a major skeletal element of the developing embryo.
What does the notochord become in adults?
In all vertebrates other than hagfish, the notochord develops into the vertebral column, becoming vertebrae and the intervertebral discs the center of which retains a structure similar to the original notochord.
What is a sacral chordoma?
Sacral chordoma is a rare tumour that represents the most common malignancy of the sacral region. Its diagnosis can be delayed because of unclear clinical manifestation. This tumour can involve surrounding anatomical structure such as the rectum, and its surgical treatment is still challenging.
Does chordoma show on CT scan?
On CT scans, chordomas appear homogeneous, with a density comparable to that of muscles. The tumor appearance on contrast enhancement is heterogeneous. Calcification is found in less than one half of patients, and differentiation from sequestered bone fragments is difficult.
How common is Ecchordosis Physaliphora?
Unlike chordomas, which are often symptomatic due to brainstem or cranial nerve compression, patients with ecchordosis physaliphora are usually asymptomatic. They are found in 0.5-2% of autopsies [2].