Melanotic progonoma of temporal and occipital bones: A case report.

Melanotic progonoma is a rare tumor that primarily affects the maxilla of infants during the first year of life. Involvement in the skull is rare and can mimick other benign or malignant tumors affecting the infant's skull. The authors report a case of melanotic progonoma of right occipital and temporal bones in a 7-months' girl and discuss the histological features, immunohistochemistry study, differential diagnosis and management of this tumor.

[Atypical metastatic sites for adenocarcinoma of the lung]. / Localisations métastatiques inhabituelles d'adénocarcinome pulmonaire.

Lung carcinomas have a high propensity to metastasize to the liver, adrenal, bone or brain. However, certain atypical intracranial sites may occur. We report two cases of adenocarcinoma of the lung discovered by peculiar brain metastases. The first patient presented with an orbital apex syndrome due to a metastasis to the cavernous sinus with intraorbital extension. The second patient presented with headache and visual loss due to metastasis to the pituitary. Intrasellar and parasellar metastases are rare sequelae of pulmonary neoplasms. Etiological diagnosis is based on imaging and sampling or biopsy. They are often associated with a poor prognosis. The differential diagnosis of an intracranial process in the area of the sella should include metastasis even in the absence of a known primary cancer.

A rare case of adult medulloblastoma with spinal metastasis.

Medulloblastoma is a relatively common malignant brain tumor of childhood and relatively rare in adulthood, with a propensity for neuraxial spread via cerebrospinal fluid pathways. Osseous extraneural metastasis is uncommon and when it happens, radiologic findings are of sclerotic (60%), lytic (35%), and mixed patterns (5%) (Algra et al. (1992)). In this paper, we present a case of medulloblastoma metastiaszing to the lumbar spine and describe the magnetic resonance appearance, with emphasis on the image findings mimicking spondylodiscitis.

[Solitary intrasellar plasmocytoma revealed by a diplopia: a case report]. / Plasmocytome solitaire intrasellaire révélé par une diplopie : à propos d'un cas.

INTRODUCTION: Plasmocytomas rarely invade the skull base. It can be solitary or multiple. The clinical presentation mainly consists in a progressive neuropathy involving many cranial nerves, or may simply present as an optochiasmatic syndrome. OBSERVATION: The authors report a case of a female patient aged 68 years presenting with an optochiasmatic syndrome with an anosmia and paralysis of the right sixth (VI) cranial nerve progressing over two years prior to her hospitalisation. Cerebral MRI showed a voluminous intrasellar lesion, isointense on T1 and hyperintense on T2, enhancing intensely after gadolinium injection with evidence of invasion of the sphenoid and cavernous sinuses. The endocrinologic assay was normal. A sphenoidal biopsy by the rhinoseptal route permitted the diagnosis of a plasmocytoma. A complete radiologic and laboratory assessment of the patient was accomplished, the patient benefited from local radiotherapy. DISCUSSION: Plasmocytomas are malignant tumors that are essentially osseous. The cervicocephalic region is rarely afflicted (1%). Extension to the cranial base seldom occurs. Less than 30 cases have been described in the literature simulating the other numerous neoplastic intrasellar lesions. CONCLUSION: Plasmocytomas of the cranial base revealing a myeloma represent a very rare entity. Nevertheless, whether solitary or multiple, a plasmocytoma must be considered amongst the differential diagnoses in the face of any invasive lesion of the sphenoid sinus.

[Orbital exenteration]. / L'exentération orbitaire.

PURPOSE: Orbital exenteration is a disfiguring surgery. The surgery is mostly performed for advanced neoplasms of the eyelid in an attempt to achieve cure with tumor free margins. Reconstruction is a real challenge, especially in elderly patients with significant comorbidities. PATIENTS AND METHODS: We operated 15 patients presenting with palpebral and orbital tumors, between January 2000 and December 2007. We collected the clinical data concerning patients, tumor, treatment, and recurrences. RESULTS: Ten male and five female patients with a mean age of 56 years at diagnosis presented with ulcerative palpebral malignant tumor, and impaired ocular motility. Basal cell carcinoma was the most common (80%). All patients underwent exenteration, (subtotal three, total eight, and extended four patients). The cavity was filled with a temporal muscle flap in ten cases, Mustardé flap in three cases, latissimus dorsi myocutaneous free flap in one case, and a jugal V-Y flap in one case. The mean follow-up was 23 months with good healing without radiotherapy tissue alteration. Four patients had a recurrence and one patient died from metastases. DISCUSSION: The goals of reconstruction are functional and esthetic. Given the initial tumoral extension, we choose to use a regional or microsurgical flap for functional reconstruction. The flap provides a good cutaneous coverage, rapid healing, closure of orbital nasal and sinus communications, or of orbital and cranial communications. It is not damaged by radiotherapy.

Intracranial meningioma in children: different from adult forms? A series of 21 cases.

OBJECTIVE: Intracranial meningiomas are very rare in children, comprising only 0.4 to 4.1% of pediatric tumors and only 1.5 to 1.8% to all intracranial meningiomas. The goal of this study of pediatric meningiomas was to establish their epidemiological profile as well as their clinical and radiological features, to assess the long-term outcome, and compare this result with adult meningioma. PATIENT AND METHODS: We conducted a retrospective study from June 1983 to June 2007; during this period 521 patients underwent surgery for primary meningioma at the Rabat Hospital, Department of Neurosurgery. Twenty-one patients were under 16 years of age (4%). The clinical charts and imaging data were reviewed. RESULTS: The mean age was 10.3 years (range: 2 to 16 years), with 13 boys and eight girls. In one patient a neurofibromatosis was associated. The mean delay to diagnosis was 4.6 months (range: 1 to 12 months). The most common clinical sign was raised intracranial pressure (90%). Of the meningiomas diagnosed, 47% were convexity meningiomas while 24% were parasagittal and 19% were skull-base meningiomas; in two cases (9.5%) the location was intraventricular. The mean tumor diameter was 6.6 cm (range: 3 to 10 cm). A large cystic component was found in 24% of the cases. Surgery achieved a Simpson grade I resection in 47%; 62% of the tumors were grade I and 24% were grade II based on World Health Organization pathological classification. The mean follow-up period was 33 months (range: 6 to 120 months). The recurrence rate was 33%. CONCLUSION: Pediatric meningiomas are larger than those found in the adult population; there is a male predominance with high incidence of a cystic component and high-grade meningiomas, thus explaining the increased recurrence rate despite the multimodal treatment.

[A case of cystic craniofacial fibrous dysplasia]. / Un cas de dysplasie fibreuse kystique craniofaciale.

INTRODUCTION: Fibrous dysplasia is a benign, idiopathic, fibro-osseous disease. CLINICAL CASE: A 17-year-old girl had presented with left proptosis for the previous two years, associated to homolateral hemicranial pain. Clinical examination was normal except for non-reducible axile exophthalmia. Computed tomography and magnetic resonance imaging of the head revealed an extensive cystic tumor of the left fronto-ethmoido-sphenoidal region, with compression of the left frontal lobe and medial orbital wall. The radiological appearance suggested a mucocele but histological examination, after surgery, proved a fibrous dysplasia. DISCUSSION: Fibrous dysplasia with fronto-sphenoido-ethmoidal localization may be misdiagnosed as mucocele. Histology proves the diagnosis.

[Posterior fossa extradural and extracranial hydatid cyst]. / Kyste hydatique intra- et extracrânien de la fosse cérébrale postérieure (à propos d'un cas).

Hydatidosis is an endemic disease in Morocco. Cerebral echinococcosis is a relatively rare entity accounting for only 1-2% of all hydatid cysts in humans. Extradural hydatid cyst of the posterior fossa is a very uncommon site for the disease: only four cases have been reported in the literature. We report the case of a 37-year-old admitted for high intracranial pressure. Brain MRI showed an extradural and extracranial posterior fossa cyst without enhancement after contrast medium injection. Multiple hydatid cysts were removed and the histological examination of the tissue sample confirmed the diagnosis. The patient was given albendazole postoperatively with good follow-up 6 months later.

Magnetic resonance imaging for spinal cord tumors. A report of twenty cases.

This paper discusses spinal cord tumors including imaging characteristics with emphasis on magnetic resonance imaging and advances in treatment. This is a retrospective study of 20 cases patients with neoplasms arising from the spinal cord. All of our cases were explored by magnetic resonance imaging (1.5T) using T1-weighted imaging (Spin Echo), T2-weighted imaging (Spin Echo) and T1-weighted imaging with Gadolinium administration. Pain is the earliest symptom, characteristically occurring at night when the patient is supine. Ependymoma were observed in 11 cases. Astrocytoma was noted in five cases. Other uncommon tumors were identified in four cases: oligodendroglioma (n=1), epidermoid cyst (n=1), hemangioblastoma and metastasis (n=1). In MRI most tumors are isointense or slightly hypointense compared to the normal cord signal with homogenous or irregular enhancement. We describe the characteristic magnetic resonance findings and differential diagnosis of spinal cord tumors. Spinal cord lesions comprise approximately 2-4% of all central nervous system neoplasms. Magnetic resonance imaging plays a central role in the imaging of spinal cord neoplasms.

[Radiosurgery in trigeminal neuralgia: long-term results and influence of operative nuances]. / La radiochirurgie dans le traitement de la névralgie trigéminale : résultats à long terme et influence des nuances techniques.

Stereotactic radiosurgery is an alternative to conventional surgery for the treatment of trigeminal neuralgia. To better define the safety of radiosurgery and optimal technical choices, we reviewed our patient records and the literature. A total of 334 patients presenting with trigeminal neuralgia were treated between December 1992 and September 2005. A minimum of 1 year of follow-up was available for 262 patients. The mean age was 68 years (range: 30-90); 128 patients were male and 134 female. A neurovascular conflict was clearly visualized on MRI in 167 patients. Twenty-one had a past history of multiple sclerosis and 110 had already received conventional surgical treatment for trigeminal neuralgia. The intervention consisted of gamma knife radiosurgery (GKS) to the retrogasserian cisternal portion of the Vth cranial nerve. The median maximal dose used was 85Gy (range: 70-90). Actuarial curves show a plateau at 5 years for both the risk of hypoesthesia and recurrence. At 5 years, 58% of the patients remain pain-free and 83% have no trigeminal nerve disturbance. The median delay for pain cessation was 15 days. The initial pain-relief rate was 89%. None of the complications reported for the other techniques were observed. Patient selection (typical versus atypical, age, past surgery, multiple sclerosis) and details of operative technique (maximum dose, volume of nerve treated, target location, etc.) had a major influence on the probability of pain relief and toxicity risk. The details of operative technique are turning out to have a major influence on the clinical results. In our experience, high-dose (80-90Gy) retrogasserian (7-8mm from the brainstem) GKS provides the patient with a better chance of long-term pain relief and a lower risk of trigeminal nerve functional disturbance. GKS applied to the cisternal anterior trigeminal nerve using high doses provided safe and effective treatment for trigeminal neuralgia over the long term.

Sphenoid sinus aspergillosis simulating pituitary tumor in immunocompetent patient.

Aspergillosis of the sphenoid sinus is rare in immunocompetent patients. It may be mistaken for a sellar region tumor. A 65-year-old, human immunodeficiency virus-negative man presented with a 3-week history of cranial nerve III paresis and visual deterioration. The patient had a long-term history of tobacco snuff abuse. CT scans and MRI demonstrated a space-occupying lesion of the sellar and sphenoid sinus region. Presumptive diagnosis of pituitary macroadenoma was made and the patient was operated on via a transnasal-transsphenoidal approach. After the sphenoid sinus was opened, a yellow-brownish gluey material with crumbly debris extruded and was aspirated. The dura was intact. Histopathology revealed numerous Aspergillus hyphae without tissue invasion. Postoperatively, the cranial nerve III paresis resolved in a few days and visual acuity improved. Sphenoid sinus aspergillosis should be included in the differential diagnosis of sellar region processes, even in immunocompetent patients. Early diagnosis and transsphenoidal removal provides good results without the need for systemic antifungal therapy in non-invasive aspergillosis.

Tuberculoma of the cavernous sinus mimicking a meningioma: case report and review of the literature.

Cavernous sinus involvement of tuberculosis is rare; only seven cases have been reported in the literature to date. Many factors make the diagnosis difficult in this location, necessitating a surgical procedure. We report the case of a 45-year-old man who presented with a left cavernous sinus syndrome clinically. The lesion mimicked a meningioma of the cavernous sinus in radiological examinations. It was entirely excised through a subtemporal approach. Histopathological examination revealed caseating granuloma and the diagnosis of tuberculoma was established. Postoperative antituberculous drugs completed the treatment and led to complete resolution of neurological symptoms. In our paper, we emphasize the rarity of the lesion in this location, we review all the cases previously published underlining the main epidemiological, clinical, radiological, therapeutic and prognostic features.

[Radiosurgery for drug-resistant epilepsies: state of the art, results and perspectives]. / Rôle de la radiochirugie Gamma Knife dans le traitement des épilepsies pharmacorésistantes: situation actuelle, résultats et perspectives.

BACKGROUND: There is growing interest in the use of radiosurgery in epilepsy. We analyzed our experience in this field in an attempt to define the potential of radiosurgery in epileptology. MATERIAL AND METHODS: [corrected] Our local clinical experience (134 patients), accumulated over the last 15 years, mainly includes treatment of temporal lobe epilepsy without space-occupying lesions (59 patients), including 53 with pure MTLE, 61 cases of hypothalamic hamartoma, two cases of callosotomy, and 12 other types of epilepsy. RESULTS: The analysis of our material, as well as other clinical and experimental data, suggest that the use of radiosurgery is beneficial only to patients in whom a strict preoperative definition of the extent of the epileptogenic zone (or network) has been achieved and strict rules of dose planning have been applied. As soon as these principles are not observed, the risk of treatment failure and/or side effects increases dramatically. Long-term outcome data are now available and published for MTLE but not yet for other types of epilepsy. Long-term safety and efficacy in MTLE are comparable to surgical resection but radiosurgery has the advantage of sparing verbal memory in patients operated by Gamma Knife (GK) on the dominant side. In small hamartomas, the efficacy is comparable to microsurgery but with a dramatic reduction in risk. CONCLUSION: The vast amount of clinical materiel and long-term evaluation now support the use of GK surgery in small hypothalamic hamartomas and MTLE when the patient is at risk of verbal memory loss.

[Paraganglioma of the latero-sellar area. Case report]. / Paragangliome latéro-sellaire. A propos d'un cas.

Paraganglioma of the sellar and latero-sellar area is extremely rare. We report a case of latero-sellar and suprasellar paraganglioma of a 58-year-old women which caused deterioration of visual acuity and left exophthalmia without endocrine dysfunction. Magnetic resonance imaging showed a large tumor in the sellar and parasellar area, which extended to the left cavernous sinus and infiltrated the left orbit, the ethmoid, the sphenoidal sinus and the left pterygomaxillary fossa. Surgery by transcranial, left frontotemporal approach, enabled subtotal removal. Definitive histologic examination revealed a paraganglioma with signs of anaplasis. We report our clinical findings and present a review of literature.

[Cerebellar primary germinoma. Case report]. / Le germinome cérébelleux primitif.

Germinoma cell tumors account for 1% of all primary cerebral tumors. They occur in pineal and in supra-sellar regions, and rarely, in basal ganglia and thalami. Germinoma cell tumors originating in the posterior fossa are very rare. We report a case of primary germinoma found in the cerebellar hemisphere in a 45-year-old immunocompetent man presented with intracranial hypertension and cerebellar ataxia. The CT scan revealed a heterogeneous mass in the cerebellar right hemisphere with a cystic component. Intense and heterogeneous enhancement after contrast infusion was observed. Two other nodules were identified in the frontal and occipital lobe. The pineal region was normal. Total resection was performed. The histological finding were a germinoma. Extensive systemic examination indicated that the intracranial lesion had not metastasized from a primary extracranial tumor.