Primary Malignant Tumours of the Trigeminal Nerve

Abstract

ABSTRACT:We report a case of primary malignant schwannoma of the trigeminal nerve, and review the 5 previously reported cases. Clinical presentation, along with radiological and pathological features are discussed. RESUME: Des tumeurs primaires malins du nerf trijumeau Un sixieme cas de neurinome primaire malin du trijumeau est presente avec une revue de la litterature. La presentation clinique et les manifestations radiologiques et pathologiques sont discutees.Can. J. Neurol. Sci. 1986; 13:146-148 Schwannomas of the trigeminal nerve are rare; 120 cases had been described up to 1972.12 Malignant schwannoma is very rare, with only five cases having been reported.3,5 These malignant tumours, which are believed to arise from schwannoma cells, 6,7 have been termed malignant schwannoma, neurogenic sarcoma, malignant neurilemmoma, neurofibrosarcoma.8 As experience with these tumours is limited, their management is a subject of controversy.In this paper, we present a new case of malignant schwannoma of the trigeminal nerve and review the five previously reported cases. CASE REPORTA 49 year old woman was admitted in April 1984 with a one month history of left sided facial and periorbital pain, and a two week history of diplopia. The only abnormality on examination was left lateral rectus paresis. Investigations which included an edrophonium test to rule out myasthenia gravis, and a CT head scan, were negative. It was postulated that the sixth nerve palsy may have resulted from a small hemorrhage into the abducens nerve as a complication of long term anticoagulant therapy which she had been taking for lower limb thrombophlebitis. Anticoagulant therapy was discontinued.At follow-up in August 1984 she reported that the facial pain had increased in severity and there was no improvement in diplopia. There was loss of sensation in the territory of the first and second divisions of the left trigeminal nerve, and absent left corneal reflex, decreased taste in the anterior two-thirds of the left side of the tongue, left masseter weakness, and a persistent left sixth nerve palsy.A CT head scan (Figure 1), performed in the coronal plane, showed destructive bone change involving the superior aspect of the clivus on the left side, and the left side of the dorsum sella. A mass lesion demonstrated in this region extended into the sphenoid sinus on the left side, and had also breeched the posterior aspect of the floor of the sphenoid sinus to extend to the right of the sphenoid septum. There was lateral extension to involve the carotid canal, as well as involvement of the cavernous sinus (Figure 2).At surgery, through a transnasal-transphenoidal approach, part of the tumour, which was in the posterior aspect of the sphenoid sinus, was removed. It was hard, avascular, and whitish. Detailed histologic examination using multiple stains including hematoxylin and eosin. PAS, PTAH. Masson's, Holmes and reticulin revealed a highly cellular spindle-celled tumour (Figure 3). The cells showed bipolar fibrillary components and were arranged i...

Malignant peripheral nerve sheath tumors of the trigeminal nerve: a systematic review of 36 cases

“…These 29 articles provided 35 cases, to which we added 1 additional case from our institu tion. 1,2,[4][5][6][7][8]10,11,[14][15][16][17][18][19][20][21]24,[26][27][28][29][30][32][33][34][35][36][37] The complete results of our analysis of 36 cases are detailed in Table 1.…”

“…The majority of patients pre sented with altered facial sensation and/or facial pain dis tributed along the affected divisions. 1,4,6,7,11,14,16,17,20,21,24,33,37 Furthermore, weakness of the mastication muscles was also seen in a number of patients. 17,20,33 The involvement of other cranial nerves was relatively common among the population we studied, and we found that it was typically due to spread of malignant tumor through the cavernous sinus and other intracranial or extracranial compartments.…”

“…Diplopia is expected to be seen in tumors involving the cavernous sinus, as tumor involvement here can affect the nerves coursing through the sinus. 1,5,11,16,20,24 Hearing loss was also seen, often as the result of local compression or nerve destruction in posterior fossa tumors. 7,17,19 Distant metastasis of trigeminal MPNSTs was also reported, with one patient having involvement of the clavicle.…”

Trigeminal nerve schwannoma

“…3 This pain is usually described as burning in nature. Sensory paresthesias and a diminished corneal reflex also may be seen.…”

“…Growth within the cavernous sinus may further lead to dysfunction of cranial nerves III, IV, and VI, and enlargement within the prepontine cistern may lead to compressive effects on cranial nerves VII, VIII, and IX. 3,4 Trigeminal schwannomas are a type of peripheral intracranial nerve sheath tumor that develop at the skull base and originate from the Schwann cells. Schwann cells are a type of glial cell that helps protect the transmission of messages and instructions by neurons in the peripheral nervous system.…”