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.

[Primary malignant tumors of the sternum]

Primary tumors of the sternum are rare and account only 0.5% of all primary bone tumors. They are often malignant, osteolytic and aggressive. They often present difficulties in management. To determine clinical, pathological and therapeutic characteristics for primary malignant tumors of the sternum. We report a series of six cases of primary malignant tumors of the sternum, collected in our institution between 1993 and 2009. There were 4 men and 2 women with a mean age of 69, 5 years. Parietal swelling was the most frequent symptom. Imaging showed a sternal lytic lesion. Three tumors were treated surgically. Associated treatments were neoadjuvant chemotherapy [1 case] and postoperative radiotherapy [1 case]. A medical treatment [radiotherapy alone or chemotherapy] was performed in 3 cases. These tumors were divided as below: 3 plasmacytoma, 1 chondrosarcoma, 1 osteosarcoma and 1 large B cell lymphoma. The management of primary malignant tumors of the sternum is multidisciplinary. It depends on the histological type, the possibility of surgical treatment and the distant and local aggressiveness

[Vasculitis in sweet's syndrome]

Also called acute febrile neutrophilic dermatosis. Sweet's syndrome is non-infective dermatoses that exhibit a predominantly neutrophilic inflammatory infiltrate. Absence of vasculitis is a histologic criterion for diagnosis. However, recent reports suggest that vasculitis should not exclude the diagnosis. To describe their clinical, pathological and therapeutic characteristics. We report a series of 47 cases of Sweet's syndrome, collected in our institution between 1997 and 2011. The patient population consisted of 11 males and 36 females. The mean age was 47 years [28-74]. An associated disease process was seen in 10 patients: inflammatory disease [3 cases], inflammatory bowel disease [2 cases], tuberculosis [3 cases] and diabetes [3 cases]. One case of pregnancy was observed. In the prodromal phase, functional symptoms were reported in 38 cases [80.8%]. Cutaneous lesions consisted of erythematous plaques or nodules. Lesions were located mainly on the upper or lower extremities. All biopsy specimens demonstrated a dermal infiltrate composed predominately of neutrophis. Fibrinoid necrosis and intramural inflammation were observed in 8 cases. The dermatosis can precede, follow, or appear concurrent with the diagnosis of the patient's diseases which requires careful surveillance