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?
Ecchordosis physaliphora is a rare congenital benign hamartomatous lesion, found in approximately 2% of autopsies [1,2]. It originates from nodal cord remnants [1].
What is a chordoma?
Chordoma is a slow growing cancer of tissue found inside the spine. Chordoma can happen anywhere along the spine. It is most often found near the tailbone (called a sacral tumor) or where the spine meets the skull (called a clival tumor). Chordoma is also called notochordal sarcoma.
Can you survive chordoma?
Chordomas are malignant and potentially life threatening tumors. Currently the median survival in the United States is about 7 years. The overall survival rates are 68% at 5 years and 40% at 10 years. Complete surgical resection offers the best chance for long-term survival.
Is there a cure for chordoma?
Because of the risk of chordomas spreading, a successful first surgery is very important. Operative intervention provides the best chance for cure and control of the tumor. Depending on the location, size and appearance of your chordoma, your surgery team might involve multiple surgeons.
Are all chordomas malignant?
Chordomas form from remnants of the notochord — embryonic tissue that eventually forms the center of spinal disks. These tumors are considered malignant and may metastasize, though they typically grow slowly.
Can ecchordosis physaliphora be confused with clival chordoma?
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.
What is the morphology of ecchordosis?
Morphologically, ecchordosis closely resembles notochord and chordoma. All three consist of vacuolated cells that contain acid mucopolysaccharides. Under the electron microscope, the intracellular vacuoles have a smooth limiting membrane whereas the extracellular vacuoles are lined by microvilli and pinocytotic vesicles.
What is the history of ecchordosis?
Herbert Luschka was the first to describe a lesion matching the characteristics of ecchordosis physaliphora at the level of the clivus in 1856. One year later, Rudolf Virchow named a similar lesion ‘ecchordosis physaliphora’, after mistakenly considering that it originates from cartilage tissue.
Can ecchordosis physaliphora cause cerebrospinal fluid rhinorrhoea and meningitis?
•Clivus defects can be associated with cerebrospinal fluid rhinorrhoea and meningitis if they are not recognised and treated •This study presents three cases where ecchordosis physaliphora was the cause of cerebrospinal fluid rhinorrhoea and meningitis