Search

We present a case of a rare tumor--melanotic schwannoma of trigeminal nerve root and gasserian ganglion. Differential diagnosis between metastatic melanoma and melanotic schwannoma (MS) is associated with serious difficulties and high responsibility. Metastatic melanoma is a high grade tumor while most MS are benign lesions with good outcome. By the date 105 cases of these tumors are described in the world literature, 3 of them originated from trigeminal nerve root and gasserian ganglion. MS predominantly occur in relatively young patients, they are characterized by presence of Carney's complex and psammomatous bodies and absence of primary focus. MS and metastatic melanoma have similar appearance on MRI due to presence of melanin granules. Indirect signs evident for MS include cystic structure and dumbbell-shaped growth. Metastatic melanoma of cranial nerves is more typical in people older than 40, primary focus in the face in the zone of innervation of affected nerve is common. In case of absence of the listed features differential diagnosis is based on immunohistochemical analysis and electron microscopy of tissue samples.

Cellular schwannomas rarely involve the cranial nerves, being more common in the spinal and peripheral nerves. A rare case of cellular schwannoma involving the gasserian ganglion, a hitherto unreported site, that extended infratentorially to present as a cerebellopontine angle tumor is reported. It is important to recognize that cellular schwannomas can histologically mimic malignant peripheral nerve sheath tumors because of their high cellularity and mitotic activity, but they are relatively benign tumors with a tendency to recur but not metastasize.

In a 25-year-old woman with episodic periorbital-temporal pain who eventually developed a sixth nerve palsy, magnetic resonance imaging revealed a lesion predominantly in the Meckel cave that was found to be a capillary hemangioma arising from the mandibular division of the trigeminal nerve. Hemangiomas of the Meckel cave must be considered in cases of facial pain with a sixth nerve palsy. even if there are no clinical findings of trigeminal neuropathy.

Objective and Importance: Idiopathic inflammatory trigeminal sensory neuropathy (IITSN) is a disorder with the dominant clinical features of trigeminal sensory disturbance; this idiopathic condition follows a benign course in most cases. Recent reports have shown that transient abnormalities, which may mimic those of trigeminal neuromas, can be observed in magnetic resonance imaging scans. Presented here is a case of IITSN that was diagnosed, with cytological and histopathological verification, during the active inflammatory phase of the disease (the first such attempt, to our knowledge)., Clinical Presentation: A 20-year-old female patient was referred to our hospital with a 2-month history of numbness of the left side of her face, headache, and hemifacial pain attacks. Cranial magnetic resonance imaging scans revealed a mass above and below the foramen ovale, extending into the cavernous sinus., Intervention: A percutaneous biopsy procedure through the foramen ovale was performed; the pathological examination revealed lymphocytes, macrophages, and endothelial cells but no evidence of neoplastic cells. A few days later, the patient was surgically treated using a cranial base approach, the gasserian ganglion was exposed, and the lesion was removed. Pathological examination of the specimens revealed inflammatory changes and fibrosis of the nerve fibers and ganglion cells. Disruption of the myelin around the nerve bundles was detected. Therefore, IITSN was pathologically confirmed during the early stage of the disease. During 3 months of follow-up monitoring, the patient experienced no serious clinical problems., Conclusion: IITSN should be suspected in cases of tumors involving the cavernous sinus, and a percutaneous biopsy through the foramen ovale should be performed as part of the differential diagnosis in such cases. This procedure might obviate unnecessary aggressive surgery. In the current case, no neoplastic cells were observed during the examination; only lymphocytes, macrophages, and endothelial cells were observed, on a background of erythrocytes. Lymphocyte-dominant inflammatory infiltration, fibrotic changes, and demyelinization are cardinal histopathological findings observed during the active phase of IITSN.

We report a large trigeminal neurinoma in an 18-month-old child involving the Gasserian ganglion and all three divisions in a plexiform pattern. The tumour in the ophthalmic division extended all the way to the supraorbital nerve. The child presented with proptosis which had been observed since she was 4 months of age. There was a family history of neurofibromatosis.

From 1980 through 1993 a series of 44 patients with trigeminal neurinomas was treated. Five of them were operated on for the remainder of the tumour as they had undergone previous surgery elsewhere, 35 were operated on for the first time, and 4 were not operated on for various reasons. An epidural approach to the neurinomas originating in the branches of the Vth nerve peripherally to the Gasserian ganglion (GG) was used. In the neurinomas originating in the GG or in the root of the Vth nerve, either an epidural-transdural approach or an epidural-transdural-transpetrous approach was used. All tumours operated on using the approach described in this article were completely removed. In 10 patients, the Vth nerve sensory deficits increased in comparison with preoperative deficits; in 9 their state remained unaltered; and in 11 the sensory function of the Vth nerve improved. In those patients who had experienced pre-operative atypical trigeminal pain, the pain disappeared after surgery. There was no additional treatment: radiosurgery, irradiation or chemotherapy. Histopathological examination did not reveal any malignant changes in the tumours in any of the patients. Based on our own experience and on the published data it is believed that the best treatment for trigeminal neurinomas is complete microsurgical removal of the lesion.

Neoplastic involvement of the trigeminal nerve was observed in 73 patients operated on in our institution for extra-axial tumours of the posterior and middle cranial fossae. It was defined as contact, compression, or infiltration. The nerve root was involved in 58 patients, the ganglion and/or the peripheral divisions in 9, all portions of the Vth nerve system in 6. A clinical trigeminal dysfunction was present in 44 patients (60%). Anatomico-surgical findings are correlated with clinical features and with tumour type. Typical trigeminal neuralgia was the complaint in 7 subjects; all of them presented an involvement of the sensory root. Post-operatively, 11 patients were relieved of their symptoms. The outcome is correlated with the anatomical findings and with the extent of surgical removal of the tumours. The importance of a careful evaluation of patients with trigeminal symptomatology is stressed.

We report on diagnosis, treatment and clinical course of a solitary schwannoma of the trigeminal nerve with consecutive metastatic spread in a 45-year-old woman. The patient presented a temporal lobe syndrome with psychomotor seizures. Trigeminal nerve function was intact apart from a transient hypaesthesia in the mandibular branch. Assuming a meningioma the tumor was removed via a temporal approach. Secondary to the definitive histological diagnosis radiation therapy was performed. After that the patient was symptom-free. 4 months later a large recurrent tumor was found involving the cavernous sinus and the pterygopalatine fossa. In a second operation the tumor was resected intra- and extradurally through an infratemporal preauricular approach in cooperation with an oral and maxillo-facial surgeon. At this time multiple pulmonary metastases developed showing no response to polychemotherapy (EVI). The patient died 13 months after onset of the disease. Hitherto, only 5 cases of a primary malignant schwannoma of the trigeminal nerve have been published in the world literature.

Two cases of Gasserian ganglion neurinomas in children are described. The onset in both cases was insidious and the tumors became very large before the first clinical signs appeared. Blindness of one eye by direct compression of the homolateral optic nerve was the main symptom in both instances. In one child homolateral exophthalmus was also present. The clinical course of both cases was completely painless and without any other sign of involvement of the trigeminal nerve. Abnormal vessels related to the tumor were visible in the arterial phase of carotid angiography. This angiographic finding (unique in appearance and distribution) may be considered as pathognomonic for tumors of the Gasserian ganglion.

A rare tumor closely associated with the trigeminal ganglion produced a syndrome of atypical facial pain in a 44-year-old man. Two previous reports have designated similar tumors as "fibro-osseous lesions." The distinctive morphological appearance is illustrated and possible histogenesis discussed.