Malignant teratoma in Klippel-Feil syndrome: a case report and review of the literature.

INTRODUCTION: Klippel-Feil syndrome is characterized by a congenital fusion of cervical vertebrae. Intracranial teratomas are nongerminomatous germ cell tumors and they account for 0.3 to 0.9% of all intracranial tumors. Teratomas with malignant transformation refer to lesions which give rise to malignant cancer of somatic type. The association between tumors of dermoid origin and Klippel-Feil malformation is extremely rare. Only 23 other cases have so far been reported, and only one case of dermoid tumor with areas of dedifferentiation on squamous cell carcinoma has been described. CASE PRESENTATION: We report the case of a 72-year-old white man with a 2-year history of gait and balance disturbances. A brain magnetic resonance imaging revealed a fourth ventricle neoplastic process with infiltrative features. He was operated through a suboccipital craniectomy with a C1 laminotomy and bilateral vertebral artery transposition. At 6-months follow-up, magnetic resonance imaging showed an early regrowth of the fourth ventricle tumor, with the same radiological features. CONCLUSIONS: Patients with Klippel-Feil malformation could develop posterior fossa dermoid tumors. The malignant potential of such tumors must be considered and surgery is recommended. Particular attention must be focused on the histopathological analysis in order to identify possible foci of malignant transformation.

First demonstration of the effectiveness of peptide receptor radionuclide therapy (PRRT) with 111In-DTPA-octreotide in a giant PRL-secreting pituitary adenoma resistant to conventional treatment.

In prolactin-secreting giant adenomas, cabergoline treatment is the first line approach. Surgery and/or radiotherapy are indicated when the tumour is resistant to medical treatment and continues growing, causing visual field impairment. Data concerning other therapeutic approach are scanty. Although PRL-secreting tumours may express somatostatin receptors type 2, 3 and 5, somatostatin analogs treatment is generally ineffective and peptide receptor radionuclide therapy (PRRT) has never been reported. A 58 year-old woman complaining of severe neurological symptoms caused by a giant prolactinoma, relapsing after surgery and not-responding to dopamine-agonists and octreotide LAR treatment, underwent four cycles of PRRT with 111-Indium-DTPA-octreotide with remarkable tumour shrinkage and a significant improvement in clinical conditions. No side effects were reported. This is the first report on the effectiveness and safety of PRRT with radio-labelled somatostatin analogs in a patient with aggressive giant prolactinoma resistant to conventional treatment.

Non-traumatic sphenoidal intradiploic arachnoid cyst as a cause of trigeminal neuralgia. A case report.

Non-traumatic intradiploic arachnoid cyst is a rare condition. We describe a young man with typical trigeminal neuralgia and intradiploic arachnoid cyst at the greater wing of the sphenoid. The patient was successfully treated with medical therapy. To our knowledge, this is the first case report of a possible correlation between trigeminal neuralgia and intraosseous arachnoid cyst. We describe the clinical case, the possible pathogenetic mechanism and briefly review the literature.

Five-level one-piece laminoplasty for extensive tumors of the lumbar spine.

The authors describe the surgical method and results of nonexpansive one-piece multivel laminoplasty of the whole lumbar spine, for microsurgical resection of extensive spinal tumors. This technique allows a very comfortable approach to the dura and intradural content, as well as a perfect replacement of the posterior spinal arch with supporting elements and an optimal reconstruction of the spinal anatomy. A nonexpansive whole lumbar one-piece laminoplasty was performed for resection of extensive multilevel lumbar tumors. The authors report an illustrative case of a patient who initially presented with a three-year history of numbness on both legs and progressive difficulty in walking. Two months before admission, he complained of bilateral sciatica and rectourinary dysfunction. A spinal magnetic resonance imaging (MRI) documented an intradural tumor extending from L2 to S1. The patient underwent a nonexpansive whole lumbar one-piece laminoplasty and microsurgical removal of the intradural lesion. The postoperative course was uneventful, the sensory disturbances and bilateral sciatica early recovered while rectourinary disturbance gradually improved up to a complete resolution at one year follow-up. The authors believe that multilevel laminoplasty rather than laminectomy is the technique of choice as a posterior procedure for extensive lumbar spinal tumors. With this technique, it is possible to obtain a very confortable approach to the dura and intradural content, as well as a perfect replacement of the posterior spinal arch with supporting elements and an optimal reconstruction of the normal spine. Moreover, this method prevents postoperative instability and deformity and avoids the so called post-laminectomy epidural membrane.

Large sphenocavernous meningiomas: is there still a role for the intradural approach via the pterional-transsylvian route?

BACKGROUND: Large-sized sphenocavernous meningiomas represent a surgical challenge. Although the role of skull base techniques with combined extra- and intradural steps has been recently emphasized, pure intradural resection tactics via the pterional route constitute the traditional microsurgical approach for resection of such tumours. METHOD: We report the application of the pterional-transsylvian approach in 13 patients with sphenocavernous meningiomas. This series is unique because it includes only patients with tumours exceeding 5 cm in their greatest dimension. FINDINGS: A gross total resection was accomplished in 10 patients (77%). Eight patients had a good outcome, one had a persistent mild hemiparesis, and one died. No recurrences occurred in this group. Three patients (23%) had subtotal resections owing to invasion of the cavernous sinus in one instance and encasement of the middle cerebral artery in the others. Two had a good outcome and one died. In these patients minimal asymptomatic tumour progression was seen 3 and 6 years after surgery. The overall surgical outcome was good in 10 patients (77%), fair in one, and death in two. INTERPRETATION: In our experience, large sphenocavernous meningiomas may be operated on adopting pure intradural resection tactics via the pterional-transsylvian route with rates of gross total removal and surgical complications related to brain retraction or vascular manipulation comparable to those of extensive skull base approaches. The traditional intradural pterional transsylvian approach continues to have a place in the treatment of these lesions.

Radicular compression by lumbar intraspinal epidural gas pseudocyst in association with lateral disc herniation. Role of the posterior longitudinal ligament.

Among unusual abnormalities of the lumbar spine reported since the introduction of Computed Tomography (CT), the presence of gas lucency in the spinal canal, known as vacuum phenomenon, is often demonstrated. On the contrary, epidural gas pseudocyst compressing a nerve root in patients with a lateral disc herniation has rarely been reported. We report a case of a 44-year-old man who experienced violent low back pain and monolateral sciatica, exacerbated by orthostatic position, one week before admission. A lumbosacral spine CT showed the presence of vacuum phenomenon associated with a degenerated disc material and a capsulated epidural gas collection with evidence of root compression. A microsurgical interlaminar approach was carried out and, before the posterior longitudinal ligament was entered, a spherical "bubble" compressing the nerve roots was observed. The capsulated pseudocyst was dissected out, peeled off and excised en bloc. A large part of the posterior longitudinal ligament and the lateral disc herniation were removed. Postoperatively the patient was completely free of symptoms. The mechanism of exacerbation of pain was probably due to the increased radicular compression in the upright posture and, besides the presence of a lateral disc herniation, could be related to a pneumatic squeezing of gas from the intervertebral space into the well capsulated sac by the solicitated L4-L5 motion segment. Histological study of the wall of the pseudocyst showed the presence of fibrous tissue identical to the ligament. We conclude that, in case of a lumbar disc herniation, it is recommended to perform a complete microdiscectomy and an accurate removal of the involved portion of posterior longitudinal ligament in order to prevent pseudocystic formations.

Trigeminal pain caused by a cerebellopontine-angle lipoma. Case report and review of the literature.

A 16-year-old girl complained of 2-year history of right facial pain, episodic vertigo and progressive hearing loss in the right ear. The facial pain was described as an episodic lancinating event confined to the second and third branch of the right trigeminal nerve. Computed tomography and magnetic resonance imaging revealed a 2 cm lesion in the right cerebellopontine-angle. At surgery, a soft, yellowish mass was found incorporating the 7th and 8th cranial nerves. The anterior-inferior cerebellar artery (AICA) was displaced medially and pushed into the sensory portion of the trigeminal nerve root, causing vascular compression. The hearing loss remained unchanged. The trigeminal pain disappeared over a period of several weeks. Patients can be harmed in an attempt to remove these neurovascular nonmalignant, generally non growing, fatty vascular lumps. Only a partial, meticulous removal should be performed with a maximum effort to decompress the affected nerve.

Spasmodic torticollis due to neurovascular compression of the spinal accessory nerve by the anteroinferior cerebellar artery: case report.

OBJECTIVE AND IMPORTANCE: Spasmodic torticollis is a neuromuscular disorder characterized by uncontrollable clonic and intermittently tonic spasm of the neck muscles. We report a case of spasmodic torticollis attributable to neurovascular compression of the right XIth cranial nerve by the right anteroinferior cerebellar artery (AICA). CLINICAL PRESENTATION: A 72-year-old man with a 2-year history of right spasmodic torticollis underwent magnetic resonance imaging, which demonstrated compression of the right XIth cranial nerve by an abnormal descending loop of the right AICA. INTERVENTION: The patient underwent microvascular decompression surgery. During surgery, it was confirmed that an abnormal loop of the right AICA was compressing the right accessory nerve. Compression was released by the interposition of muscle between the artery and the nerve. CONCLUSION: The patient's postoperative course was uneventful, and his symptoms were fully relieved at the 2-year follow-up examination. This is the first reported case of spasmodic torticollis attributable to compression by the AICA; usually, the blood vessels involved are the vertebral artery and the posteroinferior cerebellar artery.

Pituitary metastasis from uterine cervical carcinoma: a case presenting as diabetes insipidus.

The authors report a case of symptomatic pituitary metastasis from an adenocarcinoma of the uterine cervix. Only two cases of intrasellar metastasis from an adenocarcinoma of the uterine cervix have been previously reported; both were identified at autopsy. Our patient presented with clinical features of diabetes insipidus and a right sixth nerve palsy. Trans-sphenoidal surgery was performed and partial removal of the tumour was achieved. The tumour was an epidermoidal carcinoma of the uterine cervix located primarily in the posterior lobe of the pituitary. The patient's neurological symptoms were relieved following surgery. The possibility of a secondary location must always be considered in a differential diagnosis when treating an intrasellar lesion in a patient with a documented primary malignancy.

In vivo evidence that erythropoietin has a neuroprotective effect during subarachnoid hemorrhage.

To ascertain in vivo whether recombinant human erythropoietin has a neuroprotective effect on the cortex during subarachnoid hemorrhage, 56 rabbits were divided into the following groups: Group 1 control sham operated plus placebo (n=14; saline solution - NaCl 0.9%); Group 2 control sham operated plus recombinant human erythropoietin (n=14); Group 3 subarachnoid hemorrhage plus placebo (n=14); Group 4 subarachnoid hemorrhage plus recombinant human erythropoietin (n=14; intraperitoneal administration of recombinant human erythropoietin immediately after inducing subarachnoid hemorrhage). In none of the Groups 1 and 2 animals was subarachnoid hemorrhage induced. In Group 3 rabbits, an increase in locomotor activity (open field apparatus) was observed 24, 48 and 72 h after surgery, and the mortality rate was 42.9% within 72 h after surgery, and, no increase in locomotor activity was observed in Group 4 rabbits, which survived for at least 72 h. Our findings suggest that recombinant human erythropoietin may be of benefit in the treatment of subarachnoid hemorrhage.

Transsphenoidal microsurgical selective removal of multiple (triple) adenomas of the pituitary gland.

This is the first case of multiple (triple) pituitary micro-adenomas documented by magnetic resonance imaging (MRI) in a living patient and treated by a transsphenoidal microsurgical approach. The patient, a 37-year-old woman, complained of a long history of bifrontal headache, weight gain and oligomenorrhea. Physical examination revealed moderate hirsutism and a slight fat pad overlying the vertebrae. Routine laboratory studies and endocrinological biochemical investigations were normal. A gadolinium-enhanced MRI of the pituitary region revealed three intrapituitary micro-adenomas. A transsphenoidal microsurgical approach to the pituitary gland was carried out and micro-adenomas were completely removed one at a time. One year follow-up showed complete resolution of clinical symptoms and signs and normal biochemical parameters of pituitary function.

Unusual MRI finding of multiple adenomas in the pituitary gland: a case report and review of the literature.

The simultaneous occurrence of multiple adenomas in the pituitary gland is a rare event. We report the coexistence of three non functioning pituitary microadenomas in a 37-year-old woman, referred to us for oligomenorrhea and headache. Biochemical evaluation revealed prolactin (131 U/liters), follicle-stimulating hormone (4.1 U/liters), luteinizing hormone (3.9 U/liters), 17beta-estradiol (74 pg/mL), free (2.0 pg/mL) and total testosterone (0.5 ng/mL), dehydroepiandrosterone-sulfate (3.5 microg/mL), 17OH-progesterone (0.8 ng/mL), cortisol (13.1 microg/dL), free triiodothyronine (4.8 pmol/L), free thyroxine (18.5 pmol/liters), thyrotropin (1.6 mU/L), and growth hormone (0.2 ng/mL) levels in the normal range, as for as the response to dynamic endocrine tests. MRI showed an enlarged sella turcica, occupied by three distinct hypointense areas that measured less than 5 mm in diameter in the left, medium and right side of the pituitary, respectively. This finding was confirmed 6 months later by a second MRI that revealed also a light increase in microadenomas dimensions. The patient, therefore, underwent neurosurgery by transfenoidal approach. Histologic examination showed no morphologic differences between the specimens obtained from the different microadenomas. Immunohistochemistry evaluation revealed a positive staining for the common alpha-subunit of glycoproteic hormones and negative for the other pituitary hormones tested, while electron microscopy showed cells with a poor secretory apparatus and a variable grade of cell differentiation. In conclusion, we report the fifth case described with multiple pituitary adenomas diagnosed in vivo and the first with three coexisting tumors revealed by MRI before neurosurgery. The occurrence of multiple pituitary tumors emphasizes the role of pituitary and extrahypophiseal factors in the clonal expansion of genetically altered cells.

Giant olfactory meningiomas: the pterional approach and its relevance for minimizing surgical morbidity.

Olfactory groove meningiomas may attain surprisingly large size. The subfrontal approach is currently the route preferred by most neurosurgeons for their excision. The pterional-transsylvian route represents an alternate exposure for microsurgery of frontobasal tumors. Although this approach has been already described for olfactory meningiomas, tumors of giant size were not specifically addressed in the literature. We report the application of the pterional-transsylvian approach in six patients with giant olfactory meningiomas. This series is unique because it includes only patients with tumors exceeding 6 cm in diameter with bilateral symmetrical development. A radical removal was achieved in all patients and all of them made a full recovery. To investigate the relevance of the pterional-transsylvian approach for minimizing surgical morbidity, a magnetic resonance imaging protocol was designed to characterize even subtle postoperative frontal lobe structural changes. These changes, limited to the frontal lobe ipsilateral to exposure and localized in specific anatomical domains of the prefrontal area, included cystic degenerative alterations, parenchymal gliosis, and associated persistent white matter edema. Results from the present series strengthen the usefulness of the pterional-transsylvian approach as a safe surgical route for lesions affecting the anterior skull base, even with huge bilateral symmetrical expansion, such as giant olfactory meningiomas.

Asymptomatic aneurysms. Literature meta-analysis and indications for treatment.

Autopsy and angiographic studies indicate that cerebral aneurysms occur in an estimated 0.2% to 8.9% of the general population. Modern noninvasive imaging techniques now detect these lesions in ever increasing numbers, and neurosurgeons are faced with a difficult decision about whether to recommend surgical treatment of incidentally discovered asymptomatic unruptured aneurysms (AA). While in the past an expectant management was generally recommended for AA, elective surgery is now advocated because of the risk of bleeding of the untreated aneurysm and the low operative morbidity and mortality rates of modern microneurosurgery. We performed a meta-analysis of the literature on morbidity and mortality from elective surgery for AA to obtain a more generalizable estimate of operative risk than is currently available. Our meta-analysis contained 19 series consisting of 343 cases of elective surgery for AA. There was a total of 3 deaths for a combined mortality rate of 0.87%, and a total of 15 deficits for a combined morbidity rate of 4.3%. These results compare favorably with the 10.3% natural history cumulative risk of mortality for unruptured aneurysms. The authors' personal experience with AA management was also reviewed and a particularly significant illustrative case is briefly reported. We recommend treatment for all asymptomatic aneurysms incidentally discovered particularly if: a) the aneurysm size is greater than 5 mm and arterial hypertension is present; b) the patient is otherwise healthy; c) the estimated life expectancy is > or = 10 years.

Primary cervical melanoma with brain metastases. Case report and review of the literature.

Primary intramedullary melanoma is a very rare tumor that occurs most frequently in the middle or lower thoracic spinal cord. The authors present a case of primary cervical cord melanoma that developed in a 62-year-old man who was surgically treated and subsequently underwent radiation therapy. Clinical and histogenetic features of this neoplasm and results of chemo-. radio-, and immunotherapy are reported. Both "dysembryogenetic" and "mesodermal" hypotheses on the origin of primary spinal melanoma are discussed.