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.
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].
What is Clival chordoma?
Clival chordomas are locally invasive tumors that arise in the base of the skull. Clival chordomas are ideally treated with maximal safe surgical removal followed by focused radiotherapy. Fortunately, the majority of clival chordomas can be removed via the nose using an endoscopic endonasal approach.
What is a Notochordal remnant?
Notochordal remnants are occasionally found in the fetal and adult vertebrae on autopsy. Giant vertebral notochordal rest and notochordal hamartoma are terms that have been used to describe what is now collectively referred to as benign notochordal cell tumors.
What is a chordoma?
Chordomas are tumors that can occur anywhere within the spine or the base of the skull. The two most common locations for chordomas are the lower back (sacral area — approximately one-third to one-half of chordomas) and the base of the skull (approximately one-third of chordomas).
What causes Clival chordoma?
The underlying causes of chordoma are unknown. Most cases arise spontaneously and are not due to an inherited genetic change. A prevailing theory is that acquired genetic abnormalities or mutations result in cancerous growth of notochordal remnants.
What is notochord in human?
notochord, flexible rodlike structure of mesodermal cells that is the principal longitudinal structural element of chordates and of the early embryo of vertebrates, in both of which it plays an organizational role in nervous system development. In later vertebrate development, it becomes part of the vertebral column.
What is an Adamantinoma?
Adamantinoma is a rare bone cancer. Most of the time, adamantinoma grows in the lower leg. It often starts as a lump in the middle of the shinbone (tibia) or the calf bone (fibula). Adamantinoma can also occur in the jaw bone (mandible) or, sometimes, the forearm, hands, or feet.
Can a clival lesion be benign?
Although most of the lesions show a benign behaviour, clival diseases can be potentially devastating and life-threatening [3,4]. These tumours usually present as incidental imaging findings. Patients may complain of headache or cranial neuropathies, especially diplopia due to abducens nerve palsy.
Where does ecchordosis physaliphora occur on the Clivus?
Ecchordosis physaliphora (EP) is a distinct clinical entity defined as a notochordal remnant found on the dorsal surface of the clivus, occurring in about 2 % of autopsies. The aim of this study is to introduce typical and atypical imaging features of EP, which can be confused with those of clival chordoma.
Is the intradural chordoma the same as ecchordosis physaliphora?
There has been some controversy as to whether intradural chordoma and large ecchordosis physaliphora are different entities. Some authors (such as Wolfe et al.) proposed the name ‘intradural chordoma’ for all intradural notochordal remnant lesions 8.
How many cases of retroclival ecchordosis were found?
RESULTS: Five cases with retroclival EP were found (incidence, 1.7%). In all cases, the ecchordoses was hyperintense on T2-weighted images and hypointense on T1-weighted images.
Who was the first person to diagnose ecchordosis?
Hubert von Luschka (1820-1875), a German pathologist, first described the finding of pathologic ectopic notochordal tissue at the posterior clivus in 1856. The differential diagnosis of retroclival intradural lesions consists mainly of 4: