Intrasellar chordoma associated with a primitive persistent trigeminal artery.

Abstract

Chordomas located primarily in the sellar region are uncommon, and may be misdiagnosed non-functioning pituitary adenoma. Furthermore, the association of a persistent primitive trigeminal artery (PPTA) with an intrasellar chordoma is extremely rare, and no similar cases have been reported in the literature to date. The coexistence of intrasellar chordoma (ISC) and PPTA makes safe and complete tumor resection challenging, and preoperative endovascular occlusion of this artery may be helpful.We report a case of a 32-year-old man who developed right hemifacial paresthesias and a cranial nerve six palsy. MRI study demonstrated the presence of a primary ISC associated with a PPTA. Angiographic balloon test occlusion of the PPTA revealed no neurological changes, so this vessel was endovascularly occluded by coiling. The lesion was subtotally removed through a sublabial transsphenoidal approach, without intraoperative bleeding complications. Histological examination of the lesion was consistent with the diagnosis of chordoma.Detailed preoperative neurovascular evaluation may be useful to detect vascular anomalies associated with intracranial chordomas, such as PPTA. In this report we emphasize the importance of appropriate treatment of vascular anomalies prior intrasellar lesions resection that may facilitate surgery and avoid potential hazardous intraoperative bleeding complications. Sağ hemifasiyal parestezi ve kraniyal altıncı sinir palsisi gelişen 32 yaşında bir erkek olgu bildiriyoruz. MRG çalışması PPTA ile ilişkili bir primer ISC varlığını gösterdi. PPTA'nın anjiyografik balon test oklüzyonu nörolojik değişikliğe neden olmadığından sarmal (koil) ile bu damar endovasküler olarak tıkandı. Lezyon sublabial transsfenoidal yaklaşımla intraoperatif bir kanama komplikasyonu olmadan subtotal olarak çıkartıldı. Lezyonun histolojik incelemesi kordoma tanısıyla uyumluydu.İntrakraniyal kordomalarla ilişkili PPTA gibi vasküler anomalileri saptamak için ayrıntılı preoperatif nörovasküler değerlendirme faydalı olabilir. Bu raporda, intrasellar lezyonların rezeksiyonundan önce cerrahiyi kolaylaştırabilecek ve tehlikeli intraoperatif kanama komplikasyonlarından kaçınmayı sağlayacak şekilde vasküler anomalilerin uygun tedavisinin önemini vurguluyoruz.

Persistent Trigeminal Artery in Endonasal Resection of Skull Base Tumors: A Systematic Review

“…As such, the medial type has an intrasellar course and has been implicated as a potential vascular risk during transsphenoidal skull base surgery. 1,10,13 PTA is usually an incidental finding; however, studies have shown an association with wide-ranging cerebrovascular pathologies, including PHACE syndrome, arteriovenous malformation, trigeminal cavernous fistulas, intracranial aneurysms, carotid/vertebral/aortic arch anomalies, and trigeminal nerve compression. 21,24 Even more rare is the association of PTA with skull base tumors such as meningioma, pituitary adenoma, chordoma, and hemangioblastoma.…”

“…21,24 Even more rare is the association of PTA with skull base tumors such as meningioma, pituitary adenoma, chordoma, and hemangioblastoma. 10,14 Only two cases of meningioma have involved the primitive trigeminal artery despite being the most common of all primary brain tumors ($36.1%; ►Table 1). 25 Surgical resection is the mainstay of treatment with adjuvant external beam radiotherapy utilized for recurrent, atypical, or inaccessible disease or in patients with significant comorbidities.…”

“…9 Its association with skull base tumors is rarer still, but cases of concurrent pituitary adenomas, meningiomas, chordomas, acoustic neuroma, and hemangioblastomas have been reported. 1,[10][11][12][13][14][15][16][17][18] To date, only 13 such cases have been documented in the literature, including the current study. We additionally report a case of recurrent skull base meningioma and discuss its unique surgical considerations in the context of a coexisting PTA.…”

Persistent primitive trigeminal artery as a cause of trigeminal neuralgia and persistent primitive trigeminal artery associated with pituitary adenoma: Two case reports

“…Four cases of functional macro-adenoma and one case of non-functional adenoma accompanying PPTA have been reported in the literature [6][7][8][9]. PPTA and accompanying prolactinoma is an extremely rare condition and previously only one case has been reported in the English literature [10].…”

“…Although patients are usually asymptomatic, it has been reported that it may accompany third and sixth cranial nerve palsies, aneurysms, vascular anomalies and hemifacial spasm [2][3][4]. The pituitary lesions accompanying PPTA and trigeminal neuralgia (TN) associated with a PPTA are rarely seen [5][6][7][8][9][10]. Knowledge of the typical imaging findings of PPTA prevents major surgical complications [8].…”