Hemangiopericytoma of the Cerebellopontine Angle: A Wolf in Sheep's Clothing

Primary meningeal hemangiopericytoma (HPC) is a rare, aggressive dura based tumor that remarkably mimics a meningioma clinically and radiologically. Its occurrence within the cerebellopontine angle (CPA) is exceptional, and establishing the exact diagnosis is of the utmost importance since total resection remains the cornerstone of treatment. A 42-year-old man presented with a three-month history of progressively worsening vertigo and difficulty in walking. On admission, his neurological examination revealed a right peripheral facial palsy, right abducens palsy and left hemiparesis, suggesting the diagnosis of Millard-Gubler syndrome. Computed tomography and magnetic resonance imaging demonstrated a homogeneously enhancing dura based lesion of the right CPA causing major brain stem compression. There was no widening of the ipsilateral internal auditory canal. A standard retrosigmoid craniotomy was performed to access the right CPA. Exposure of the lesion revealed a well-encapsulated, gray, fibrous lesion, which appeared to originate from the tentorium. Gross total resection was achieved and confirmed radiologically. The microscopic features and the immunohistochemical profile confirmed the diagnosis of a HPC, and adjuvant radiation therapy was administered. Ten years later, the patient presented with a severe neurological deficit due to a local recurrence, but at that time refused any second intervention. He died three months later. HPC can locate within the CPA and present as a Millard-Gubler syndrome. The diagnosis should be kept in mind in case of a CPA dura based tumor. Radical surgery plus radiation therapy can maximize the recurrence-free survival and close follow-up remains mandatory to spot recurrences early.

Hemangiopericytoma of the Cerebellopontine Angle: A Wolf in Sheep's Clothing

Primary meningeal hemangiopericytoma (HPC) is a rare, aggressive dura based tumor that remarkably mimics a meningioma clinically and radiologically. Its occurrence within the cerebellopontine angle (CPA) is exceptional, and establishing the exact diagnosis is of the utmost importance since total resection remains the cornerstone of treatment. A 42-year-old man presented with a three-month history of progressively worsening vertigo and difficulty in walking. On admission, his neurological examination revealed a right peripheral facial palsy, right abducens palsy and left hemiparesis, suggesting the diagnosis of Millard-Gubler syndrome. Computed tomography and magnetic resonance imaging demonstrated a homogeneously enhancing dura based lesion of the right CPA causing major brain stem compression. There was no widening of the ipsilateral internal auditory canal. A standard retrosigmoid craniotomy was performed to access the right CPA. Exposure of the lesion revealed a well-encapsulated, gray, fibrous lesion, which appeared to originate from the tentorium. Gross total resection was achieved and confirmed radiologically. The microscopic features and the immunohistochemical profile confirmed the diagnosis of a HPC, and adjuvant radiation therapy was administered. Ten years later, the patient presented with a severe neurological deficit due to a local recurrence, but at that time refused any second intervention. He died three months later. HPC can locate within the CPA and present as a Millard-Gubler syndrome. The diagnosis should be kept in mind in case of a CPA dura based tumor. Radical surgery plus radiation therapy can maximize the recurrence-free survival and close follow-up remains mandatory to spot recurrences early.

[Central nervous system dysgerminoma. A clinicopathological study of 3 cases]

Intracranial germ cell tumors are rarely seen and typically localize in the pineal or suprasellar region. The largest category of germ cell tumors is dysgerminoma. To describe clinicopathological features and immunohistochemical profile of dysgerminomas. We report three cases of central nervous system dysgerminomas. There were two young women and a man who were 6, 11 and 23 year-old. They presented with symptoms of insipidus diabetes [n=3] with association to visual field defects in the third case. Radiological findings showed a supra seller lesion in two cases. Double localization in the pineal and suprasellar regions was seen in the third case. Histologic examination and immunohistochemical study of surgical specimen were consistent with primary central nervous system dysgerminoma

Benign ethmoidal polyposis as an unusual cause of CSF leakage and recurrent meningitis

Cerebrospianl fluid [CSF] leak is usually post-traumatic, but rarely it can be tumourous or spontaneous complications of nasal polyposis are exceptional. The association between CSF leak and nasal polyposis has been rarely described. Herein, we report the case of a woman with a history of recurrent meningitis and CSF leak, secondary to a huge benign nasal plyposis treated in a single neurosurgical procedure

Surgical management of symptomatic suprasellar arachnoid cysts

The true incidence of intracranial arachnoid cysts is unknown because many may be asymptomatic throughout life. However, with the advent of CT and MRI the frequency of the diagnosis of arachnoid cyst has significantly increased. In adults they represent 9% of all the arachnoid cysts while in paediatric population this percentage reaches 15%.The various surgical options for treating suprasellar arachnoid cysts [SSAC] include craniotomy with fenestration or marsupialization of the cyst, cystoperitoneal shunt and percutaneous ventriculocysto-cisternostomy. Ten patients with SSAC were operated on in our institution since 1992. The aim of our work consists of describing the clinical manifestations, the radiological features and the different surgical methods to treat SSAC. The patients ranged in age from one month to 33 years [mean age = 8 yrs]. The signs and symptoms are referable to the size of the cyst. All patients with SSAC underwent preoperative CT, cisternography or MRI. In 7 patients, marsupialization of the cyst was performed; in 5 patients, a cystoperitoneal shunt was placed and ventricuhocysto-cisternostomy performed in only 2 patients. Five patients were operated more than one time undergoing two different methods of surgery

Hypothalamic hamartoma causing precocious puberty treated by surgery

Hypothalamic hamartomas are benign turnouts that are often associated with central precocious puberty. Resection of hypothalamic hamartoma has been recommended as a treatment option for selected cases of pedenculated lesions, especially in young children. We report a case of a four-year-old boy whose onset of puberty began at approximately 10-months of age when his parents noticed incipient pubic hair and a husky voice. Magnetic resonance imaging demonstrated a pedenculated isodense mass below the tuber cinereum associated with a pituitary microadenoma. The hamartoma was totally removed microsurgically. Postoperatively, the signs and symptoms of precocious puberty began to disappear. Clinical presentation, mechanism and treatment of precocious puberty caused by hypothalamic hamartoma are fully discussed