Symptomatic ecchordosis physaliphora of the upper clivus: an exceedingly rare entity

Spontaneous rhinorrhea: a possible concealing initial symptom of ecchordosis physaliphora. Illustrative case

“…The intraosseous growth of EP weakens the clivus, making it vulnerable to fractures with even minor trauma or spontaneous fractures. 5,[17][18][19] This "locus of minor resistance" can lead to the formation of a pseudo-meningocele or a CSF leak into the nasal cavity, increasing the patient's risk of developing pneumocephalus and meningitis. 20,21 The patient may experience various symptoms, such as headache, stiffness in the neck, sensitivity to sound and light, and a decline in consciousness due to irritation of the meninges.…”

“…According to the literature, headache is the most common presenting symptom followed by cerebrospinal fluid (CSF) leakage, meningitis, diplopia, dizziness, sixth cranial nerve palsy, facial pain, and hearing loss. 5,6 Rarely, fatal subarachnoid and intratumoral hemorrhages have also been reported. 4,[7][8][9] Differential diagnoses for EP include chordoma, other benign notochord tumors (BNCTs), dermoid cysts, and epidermoid cysts.…”

“…4,[7][8][9] Differential diagnoses for EP include chordoma, other benign notochord tumors (BNCTs), dermoid cysts, and epidermoid cysts. 1,5,10 T1-and T2-weighted magnetic resonance imaging (MRI) typically shows a hypodense lesion without restricted diffusion, whereas T2 fluid-attenuated inversion recovery (FLAIR) reveals CSF-like features. However, neither MRI nor computed tomography (CT) show enhancement.…”

“…The Ki-67 index is usually less than 5%, but no significant clinical implications have been found. 1,5,12 The endoscopic approach is currently the preferred method for treating EP; however, other approaches such as the transoral, transpalatal, transmaxillary, suboccipital, retrolabyrinthine presigmoid, frontotemporal, and trans-third ventricle techniques are also viable options. 5,[13][14][15] In addition to surgical interventions, 1 case of EP recurrence in a child has been reported to be successfully treated with proton therapy.…”

Letter: Chordoma and Ecchordosis Physaliphora: 2 Sides of the Same Coin

“…Each year, 1 person in a million is diagnosed with chordoma, but only 45 cases of EP have been reported. [1][2][3][4][5][6][7][8][9][10][11][12] In 1857, Virchow invented the acronym EP for the newly discovered dorsum sella lesion, believing it was cartilage related. A year later, Muller discovered notochord rests in the synchondrosis sphenooccipitalis and hypothesized that these were the source of the EP dorsum sella masses.…”