Clinical and Radiologic Follow-Up in Ecchordosis Physaliphora: A Case Series and Literature Review

Abstract

Objective: This study endeavors to assess the clinical and radiologic findings of ecchordosis physaliphora (EP) in patients under long-term observation at our clinic as well as in cases reviewed from the existing literature.

Methods: In our study, we evaluated EP lesions in a total of 16 patients who underwent follow-up and treatment in the neurosurgical unit. We conducted a retrospective review using magnetic resonance imaging and computed tomography studies to confirm the diagnoses as EP. We conducted a systematic literature review following the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, encompassing a database search from inception to January 2024. We included confirmed cases of EP from both surgically and conservatively reported studies.

Results: Our study included a total of 16 patients, consisting of 9 (56.25%) men and 7 (43.75%) women, with an average age of 45 ± 17.3 years. Among them, 7 (43.75%) patients presented with headaches, while 3 (18.75%) reported hearing loss. Incidental EP was detected in 6 (37.5%) patients in the study during imaging performed for different indications. The key radiological features of EP comprised hypointensity on T1, hyperintensity on T2, and an absence of magnetic resonance imaging gadolinium enhancement. In 1 out of the 16 cases, we employed an endoscopic endonasal approach for resection, and there was no recurrence observed over an average postoperative follow-up period of 24 months. Among the 15 patients who underwent conservative follow-up, 12 (80%) had the classical type B EP, one (10%) patient exhibited benign notochordal tumor in the C2 vertebra, and another (10%) patient presented with a variant type EP.

Conclusions: Utilizing a combination of imaging modalities, ensuring a clear radiological distinction between EP and chordoma, can offer substantial advantages in this context. Given that EP might be incidentally discovered and nonresistant symptoms may resolve on their own, considering conservative treatment before surgery may be a viable option in all cases.

Keywords: Chordoma; Clivus; Ecchordosis physaliphora; FLAIR; Retroclival mass.