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.

Clinical and imaging findings in a rare case of sinus pericranii.

BACKGROUND: Sinus pericranii is a rare, usually asymptomatic condition that is characterized by an abnormal communication between the intra- and extracranial venous drainage pathways. The etiology is unknown but both congenital and post-traumatic etiologies have been proposed. Treatment is primarily surgical but newer minimally invasive endovascular approaches have been reported and is indicated due to cosmesis, hemorrhage, and air embolism. ILLUSTRATIVE CASE: We present a case of an 11-month-old boy having sinus pericranii, who was referred for a slowly growing tumor located frontally in the midline on his scalp since 6 months of age. CT-scan with three-dimensional CT (3D-CT) reconstruction and magnetic resonance imaging along with venography was performed which confirmed the diagnosis. Simple surveillance was decided because of a limited esthetic prejudice and the absence of any functional disorder. CONCLUSION: The prognosis is nearly always good with a low risk of bleeding. A simple follow-up is often proposed because of the usual absence of complications.

Management of spinal cord compression caused by extramedullary hematopoiesis in beta-thalassemia.

Thalassemia is a chronic hemolytic anemia, endemic around the Mediterranean basin. Extramedullary hematopoiesis (EMH) is a normal compensatory reaction that can involve many organs or tissues, including the epidural space, leading to a spinal cord compression syndrome. In almost all cases, the clinical and MR patterns are those of a lower dorsal spinal cord progressive compression secondary to an epidural expanding process. The management still remains controversial, including mainly blood transfusions, decompressive surgery and radiotherapy. We present a case of EMH in a thalassemic adolescent boy presenting with a T12 level of compression, treated successfully with blood transfusion, surgical decompression and radiotherapy.