Ectopic recurrence of craniopharyngiomas: case report.

OBJECTIVE: Craniopharyngiomas are benign tumors that originate from squamous cell rests of the embryonal hypophyseal-pharyngeal duct located along the pituitary stalk. After their surgical resection, recurrence usually occurs in the region of the original tumor bed. Ectopic recurrence of craniopharyngiomas is extremely rare. It usually occurs either along the surgical route, because of direct surgical seeding, or at a distal location in the subarachnoid space, because of seeding along the cerebrospinal fluid pathways. We present 3 examples of ectopic recurrences of craniopharyngiomas. CLINICAL PRESENTATION: The first patient was a 52-year-old woman with a history of resected suprasellar craniopharyngioma presenting 15 years later with a history of balance problems and new onset of double vision. Her magnetic resonance imaging scan revealed a tumor in the prepontine cistern. The second patient was a 41-year-old man with a history of a resected suprasellar craniopharyngioma presenting 9 years later with headache, dizziness, and disequilibrium. He was noted by his family to have an altered behavior with progressively increasing indifference. His magnetic resonance imaging scan showed a right frontal lesion in the vicinity of the sylvian fissure. The third patient was a 24-year-old man with a history of suprasellar craniopharyngioma resection, followed by conventional radiotherapy 12 years before his recent presentation with headache, numbness of the right side of his face, and increased drowsiness. His magnetic resonance imaging scan showed a bilateral cystic cerebellopontine angle lesion. INTERVENTION: The first patient underwent operation via a petrosal approach with subtotal resection of the tumor and decompression of the brainstem; this patient had an uneventful postoperative course. The tumor in the second patient was surgically resected through a pterional craniotomy, with an uneventful postoperative course. The third patient's right-sided cerebellopontine angle lesion was microsurgically resected, and the patient was given a single-dose gamma knife for the left-side and residual small right-side tumor. The histological diagnosis of all 3 lesions was craniopharyngioma. CONCLUSION: Although ectopic recurrence of a craniopharyngioma is very rare, it should always be considered in the differential diagnosis of what appears to be a new tumor in a patient with a history of previously resected craniopharyngiomas. Long-term follow-up of patients with resected craniopharyngioma is very important.

Cystic giant prolactinoma in childhood.

In childhood and adolescence, pituitary adenomas are rare and half are prolactinomas. However, cystic giant prolactinoma in prepuberty is extremely rare. In this report, we present a 10-year-old boy with a cystic giant prolactinoma who was treated with two-stage surgery as the tumor was dumbbell shaped. To our knowledge, this is the second reported case of a cystic giant prolactinoma in a prepubertal child.

Management of bone-invasive, hyperostotic sphenoid wing meningiomas.

OBJECT: The hyperostosis frequently associated with sphenoid wing meningiomas is actual invasion of bone by the tumor. The intracranial portion of the tumor is usually thin with en plaque spread, and the tumor tends to invade the orbit through the superior orbital fissure. METHODS: The authors reviewed the records of 67 patients with sphenoid wing meningiomas who underwent surgery at the University of Arkansas for Medical Sciences between 1994 and 2004. In all 67 cases, the surgery was performed by the senior author. Seventeen of the patients had the distinguishing characteristics of hyperostotic sphenoid wing meningiomas-extensive bone invasion, en plaque dural involvement, and a minimal intracranial mass with minimal orbital involvement. In all patients, hyperostosis was determined on the basis of preoperative neuroimaging. Histopathological evaluation of bone specimens was performed in 14 cases. Estrogen and progesterone receptor expression and Ki 67 labeling were evaluated in all specimens. Chromosome analysis was performed in all tumors resected since 2001 (seven cases). Particular attention was paid to removing all involved bone and dura mater. RESULTS: Total removal was achieved in 14 cases (82.3%), with only one recurrence (7.1%) over a mean follow-up period of 36 months (range 5-72 months). Radical resection was followed by cranioorbital reconstruction to prevent enophthalmos and to obtain good cosmetic results. No deaths or serious complications occurred in association with surgery. Proptosis was corrected in all cases and visual acuity improved in seven (70%) of 10 cases. Revision of the orbital reconstruction was required because of postoperative enophthalmos (two cases) or restricted postoperative ocular movement (one case). CONCLUSIONS: Sphenoid wing meningiomas frequently invade bone, although such invasion does not represent malignancy. These lesions are generally histologically benign. Total removal with a prospect for cure and visual preservation should be the goal of treatment. This requires extensive drilling of the invaded bone and extensive excision of the involved dura. When the optic canal is involved, it should be decompressed. Extensive bone resection should be followed by cranioorbital reconstruction for good cosmesis and to prevent enophthalmos.

Arteriovenous fistula originating from proximal part of the anterior cerebral artery.

Intracerebral arteriovenous fistulas of the brain may be misdiagnosed as arteriovenous malformation. There are only a few reports in the literature about this concept and to the best of our knowledge this is the first case arising from the proximal part of the anterior cerebral artery in association with an aneurysm that ruptured. A 64-year-old man presented with subarachnoid hemorrhage. The patient was neurologically intact. Cerebral angiography and magnetic resonance imaging of the patient revealed a small arteriovenous malformation of the brain involving the region of gyrus rectus and the posteromedial aspect of the orbitofrontal gyrus in addition to an aneurysmal dilatation within the malformation. An arteriovenous fistula arising from the junction of the A1 and A2 segments of the right anterior cerebral artery connected to a vein that has tributaries spreading over the fronto-orbital gyrus and gyrus rectus, were observed during surgery. The patient was successfully treated with clip ligatation of the fistula. Intracerebral arteriovenous fistulas are rare lesions. They may easily be misdiagnosed as arteriovenous malformations. There preoperative consideration in the differential diagnosis can help to decide the most appropriate treatment option.

Results of microsurgical clipping of 50 high complexity basilar apex aneurysms.

OBJECTIVE: Complex basilar aneurysms (large size, wide base, low bifurcation, and dysmorphic posteriorly projecting domes) frequently fail endovascular treatment. We report our experience using the pretemporal transzygomatic transcavernous approach with 50 complex basilar aneurysms. METHODS: Using the pretemporal transcavernous route, opening the occulomotor trigone, and removing the anterior clinoid and the posterior clinoid when necessary, a wide exposure of the interpeduncular fossa is achieved. Temporary clips are applied to a perforator-free zone of the basilar trunk, proximal to the superior cerebellar artery. Complexity criteria in the 50 aneurysms included large or giant size in 27 patients, wide dysmorphic base in 18 patients, low bifurcation in 21 patients, posteriorly projecting dome in 11 patients, and dolichoectasia of the apex in three patients. RESULTS: Twenty-five patients presented with subarachnoid hemorrhage. There were 14 men and 36 women between the ages of 32 and 76 years (mean, 52.2 yr). Forty-nine aneurysms (98%) were successfully clipped. There was no procedure-related mortality. Two patients died (one from delayed bowel ischemia and one from a vasospasm-related complication). There were three ischemia-related events, two of which were procedure-related (medial thalamic lacunar infarct, superior cerebellar distribution ischemia) and one which was a third distal middle cerebral cardiac embolus after stopping Coumadin (DuPont Pharmaceuticals, Wilmington, DE) for atrial fibrillation. Transient partial or complete occulomotor palsies occurred in all patients with full recovery as the rule, except in one patient. At discharge, Glascow Outcome Scale scores were 4 or 5 in 88% of the patients. At the 6-month follow-up examination, Rankin Outcome Scale scores were 0 to 2 in 92% of the patients. CONCLUSION: Our experience reintroduces microsurgery as a safe and more durable treatment option for the management of complex basilar apex aneurysms that tend to have a higher rate of failure with endovascular therapy.

Distal anterior cerebral artery aneurysms: report of 26 cases.

Distal anterior cerebral artery (ACA) aneurysms are rare, and constitute approximately 1.5% to 9% of all intracranial aneurysms. They show some unique features compared with other aneurysms in the cerebral circulation and are frequently treated with a different technique. Twenty-six of 364 patients with cerebral aneurysms treated at our department between 1996 and 2004 had distal ACA aneurysms (7.1%). Twenty-three of the 26 patients were treated through an anterior interhemispheric approach and two with a pterional approach. All saccular aneurysms were successfully clipped except one which was embolized after the surgery. The only fusiform aneurysm spontaneously thrombosed and resolved with parent artery occlusion. Two of the 26 patients had multiple aneurysms. The surgical mortality was 8%. Distal ACA aneurysms have higher mortality and morbidity than other anterior circulation aneurysms. They should be aggressively treated even if very small because of the tendency to rupture. Endovascular treatment is an alternative in the management of these aneurysms. The most important factors affecting the outcome are grade on admission and the neurosurgeon's experience.

Cerebellopontine angle lipoma with extracranial extension.

Lipomas of the cerebellopontine angle (CPA) are rare. A recent literature review identified only 98 reported cases of CPA lipoma. We present here a case of CPA lipoma in a 28-year-old woman who was admitted to our hospital with hearing loss in her left ear. Computed tomography scan and magnetic resonance imaging revealed a CPA mass lesion with extracranial extension around the left internal carotiol artery. The patient was operated on in the sitting position via a right suboccipital craniectomy. The intracranial part of the mass was partially removed. Histopathological examination resulted in a diagnosis of lipoma. Surgical treatment of CPA lipomas is rarely indicated, and the aim of surgery must be decompression of neural structures.

Distal anterior cerebral artery mirror aneurysms and middle cerebral artery aneurysms.

A 52-year-old woman presented with bilateral distal anterior cerebral artery (ACA) mirror aneurysms, in addition to two right middle cerebral artery (MCA) aneurysms. The left distal ACA and right MCA aneurysms were clipped through right interhemispheric and right pterional craniotomies. The right distal ACA aneurysm was thrombosed. Distal ACA aneurysms tend to be multiple, but mirror aneurysms are rare, especially in the absence of congenital vascular anomaly. Single stage unilateral craniotomy is recommended for surgical treatment.

Spontaneous cerebrospinal fluid rhinorrhoea due to temporosphenoidal encephalocele.

A 57-year old man was referred to our hospital with spontaneous cerebrospinal fluid (CSF) rhinorrhoea of 6 years duration. He had an episode of meningitis 2 months previously. CT cisternography and cranial MRI revealed a defect in the lateral wall of the sphenoid sinus, with an anteromedial temporosphenoidal encephalocele. Surgery was performed transcranially through a pterional approach. The temporal encephalocele was amputated, the sphenoid sinus obliterated and the dural defect repaired. Lumbar drainage was used for 5 days after surgery. Spontaneous CSF rhinorrhoea is only infrequently due to temporal encephalocele. Anteromedial temporosphenoidal encephaloceles are the least common type of temporal encephalocele, with only 12 reported in the literature.

Recurrent solitary fibrous tumour in the cerebellopontine angle.

Solitary fibrous tumours (SFT) of the central nervous system are rare. They resemble meningioma in clinical presentation, imaging features and appearance at surgery. Schwannoma, hemangiopericytoma and other spindle cell mesenchymal neoplasms should also be considered in the differential diagnosis. Although the histogenesis of this tumour is still debated, strong CD34 reactivity of the tumour cells suggests that SFT is mesenchymal. We present the clinical, radiological, and pathological features of an SFT located in the cerebellopontine angle (CPA). A 55-year-old female presented with 6 months of headache. The MRI scan showed a contrast enhancing ovoid mass in the left CPA. At craniotomy, the tumour was completely resected. Histolopathological diagnosis was of meningioma. Three years later, the symptoms recurred and an MRI scan demonstrated tumour recurrence. A repeat craniotomy was performed and the lesion was again completely excised. Tumour morphology on histopathology and immunoreactivity for CD34 of the tumour cells supported the diagnosis of SFT. Review of the original tumour also disclosed immunoreactivity for CD34. Ki67 labeling indices were less than 1% in both tumours.

Intradiploic epidermoid cysts of the skull: a report of four cases.

Epidermoid cysts are uncommon, benign and slow-growing lesions. They may often reach an enormous size without producing neurological symptoms. Intradiploic epidermoid cysts are nearly 25% of all epidermoids. They are derived from ectodermal remnants that stay within the cranial bones during embryonic development. Intradiploic epidermoid cysts can be located in any part of the skull. Two of our four cases were located in left occipital bone and the others were in the frontal bone. These tumours can occur at any age from the first to the seventh decade of life. Our cases had a mean age of 38.75 years (19-55 years). They may reach great sizes before the initial diagnosis and may produce major neurological signs. For good long-term prognosis, correct radiological assessment and complete removal of the tumour with its capsule are essential. We report four cases of intradiploic epidermoid cysts of the skull and analyse the clinical, radiological features and treatment of these lesions in the light of relevant literature.

Tension pneumocyst after transsphenoidal surgery for Rathke's cleft cyst: case report.

OBJECTIVE AND IMPORTANCE: Tension pneumocephalus is a rare but well-described complication of transsphenoidal surgery. It is usually associated with postoperative cerebrospinal fluid fistulae causing lower intracranial pressure, with air located in the subdural, subarachnoid, or intraventricular space. We report a case of suprasellar tension pneumocyst that caused visual deterioration to develop after an operation for a Rathke's cleft cyst. Only one similar case has been reported previously. CLINICAL PRESENTATION: A 54-year-old woman with a cystic sellar-suprasellar mass compressing the chiasm was operated on via a standard transsphenoidal approach. The intraoperative diagnosis was Rathke's cleft cyst, and the floor of sella was left open to avoid recurrence. The sphenoid sinus was filled with a fat graft, and the rostrum of the sphenoid was reconstructed with a bone fragment. The patient's postoperative course was uneventful, and her vision improved. Ten days after discharge, the patient was readmitted to the emergency service with headache and visual impairment. Emergent computed tomography confirmed a suprasellar tension pneumocyst. INTERVENTION: The patient underwent immediate reoperation via an endonasal endoscopic approach. After the trapped air was evacuated, the sella was closed with fascia lata and muscle using fibrin glue. The patient's vision improved postoperatively. CONCLUSION: Suprasellar tension pneumocyst is an extremely rare complication of transsphenoidal surgery. To avoid this complication, the sellar floor should be repaired in a watertight fashion, and patients should be instructed to avoid blowing the nose, sneezing, straining, and coughing postoperatively.