Solitary fibrous tumor of the central nervous system: a clinicopathologic study of 24 cases.

OBJECTIVE: Solitary fibrous tumor is a rare, spindle-cell benign mesenchymal neoplasm and has a high recurrence rate. In this study, we reviewed our experience in the diagnosis and treatment of 24 patients with central nervous system solitary fibrous tumors. METHODS: Clinical data were retrieved from the medical records. Prognosis was assessed by clinic service and telephone interview. The specimens were stained with hematoxylin and eosin. Immunohistochemistry for CD34, CD99, EMA, HMB-45, Bcl-2, vimentin, GFAP, S-100, MBP, CK and MIB-1 was performed in all cases. Distributions of time to progression and recurrence were estimated using the Kaplan-Meier method and compared using the log-rank test. RESULTS: The 24 patients included 13 men and 11 women with a median age of 49.0 years. The most frequent initial symptoms were headache, dizziness, unstable walk and hearing loss. The most common location was cerebellar pontine angle (n = 6). Surgery reached gross total removal for 18 patients but subtotal removal for six patients on initial operation. Histopathologic examination showed spindle to oval cells were disposed in wavy fascicles between prominent, eosinophilic bands of collagen. Dense bands of collagen appeared in cross section as minute nodules that separated individual tumor cells. Cellular areas with a partial hemangiopericytoma pattern were noted in six cases. Atypical presentations were shown on initial operation in three cases. CD34, CD99 and vimentin were 100% positive; but EMA, CK, MBP, HBM-45 and GRAP were 100% negative. The positive in Bcl-2, RF and S-100 was 89%, 85% and 26%, respectively. Follow-up information was available for 23 patients. The median follow-up period was 36.0 months. Nine patients recurred and one patient died from the progression. Incomplete surgical resection was significantly associated with recurrence (p = 0.010). MIB-1 labeling index in recurrence was higher than in no recurrence (6.0% versus 3.4%, p = 0.029). All treated with subtotal removal only had subsequent tumor recurrence or progression; however, the two patients who were administered adjuvant radiosurgery after subtotal removal did not recur or progress. Adjuvant radiosurgery seemed to improve the prognosis (p = 0.028). CONCLUSIONS: Solitary fibrous tumor is a rare mesenchymal tumor with a propensity to recur. The most affected area is the cerebellopontine angle. Immunohistochemistry should be used to differentiate solitary fibrous tumor from other tumors. The extent of resection, MIB-1 labeling index and some anaplastic features might be predictive for recurrence. Postoperative radiosurgery might be an option in incompletely resected solitary fibrous tumor. Regular and long-term follow-up remains mandatory to monitor recurrence.

Spinal meningioma in childhood: clinical features and treatment.

OBJECT: The purpose of this retrospective study is to determine the clinical characteristics and the prognosis of the spinal meningioma in childhood (under 18 years of age) based on the treatment at our institution. METHODS: Ten spinal meningioma cases in children were treated during the last 9 years. The clinical data was retrospectively analyzed and the results were compared with those in the literature. RESULTS: The series included eight males and two females and the mean age was 13.2 years. The most common initial symptoms were pain (6/10) and the common signs were limb weakness (4/10) and gait disturbance (2/10) and distal paresthesia (1/10) and bladder disturbance (1/10). Four patients had other clinical signs of neurofibromatosis type II (NF-2) such as tumors elsewhere. All the tumors were located in cervical and thoracic vertebrae. Resection according to Simpson Grade I (6/10), II (2/10), III (1/10), and IV (1/10) were performed. Grade II meningiomas accounted for 3/10 in this series. All patients were followed up with mean follow-up period of 43 months. Seven patients had recurrence of the tumor in that period and one had died. CONCLUSIONS: Spinal meningioma is an uncommon pediatric neoplasm and has a poor prognosis. It has a male predominance and is inclined to be associated with NF-2, and those that are associated with higher pathologic subtypes and NF-2 have more unfavorable outcome. Every effort should be made to achieve total removal which may decrease the incidence of recurrence.

Imaging characteristics of Rosai-Dorfman disease in the central nervous system.

BACKGROUND AND PURPOSE: Rosai-Dorfman disease (RDD) is a rare, lymphoproliferative disorder of uncertain etiology. The Central Nervous System (CNS) is a very rare site for RDD and only a few imaging appearances have been described. The purpose of this study is to present the largest series of cases in the CNS imaging literature to increase familiarity with this entity and further identify features that may distinguish RDD from meningioma. MATERIALS AND METHODS: Findings from imaging examinations in 10 patients with pathologically confirmed RDD were retrospectively reviewed. Two radiologists evaluated the lesion location, shape, size, number, edge, cerebral edema, homogeneous or heterogeneous appearance, attenuation and signal intensity, degree of enhancement, and the relation between lesions and meninges. RESULTS: RDD in CNS showed similar features in imaging: an extra-axial, well-circumscribed, dura-based mass, isodense or hyperdense on CT, isointensity on T1-weighted imaging and isointensity with hypointensity on T2-weighted imaging. The mass enhanced markedly and homogeneously after the administration of contrast agent and demonstrated dural tail sign in all cases. Significant perifocal edema was associated with the masses. Remarkably, seven patients (77.8%) showed strong hypointensity within isointensity on T2-weighted or FLAIR images and no calcification was observed in CT images or pathologic specimens. CONCLUSIONS: Although RDD in the CNS is a rare process, it should be considered in the differential diagnoses for meningioma. We believe that a typical representation of hypointensity irrelevant to calcification on T2-weighted or FLAIR images can suggest the diagnosis of RDD.

Intracranial clear cell meningioma: a clinicopathologic study of 15 cases.

OBJECT: Clear cell meningioma (CCM) is a rare histological variant of meningioma. CCM has a high recurrence rate and aggressiveness. In this study, we reviewed our experience in the treatment of the lesion. METHODS: Here we present a series of 15 patients with intracranial CCM. The clinical data were retrieved from the records of our Neurosurgery Department and the patients' prognoses were attained by clinic service and telephone. Immunohistochemistry for epithelial membrane antigen (EMA), vimentin, glial fibrillary acidic protein (GFAP), CD10, and S-100 was done, and the MIB-1 labeling index was calculated in all cases. RESULTS: The 15 patients included eight males and seven females; the mean age was 34.8 years. The most frequent initial symptoms were headache and hearing loss. The most common location was the cerebellopontine angle (CPA) zone. Eleven patients had total removal and four patients underwent subtotal removal. Histological features of atypia were present in different proportions, from 6.7% to 100%, and six cases accorded with atypia. Three tumors showed brain invasion. EMA and vimentin were 100% positive, and CD10 was 100% negative. GFAP was 87% negative and S-100 was 93% negative. The mean follow-up period was 36.7 months. Three patients with brain invasion all recurred and five cases with atypia recurred. In 11 patients with total removal, six patients recurred. In four patients with subtotal removal, three patients recurred. Kaplan-Maier analysis showed that incomplete surgical resection was significantly associated with recurrence (p = 0.001). The MIB-1 labeling index for recurrence was 5.7 ± 2.7% versus 2.8 ± 1.5% for no recurrence (p = 0.036). CONCLUSIONS: CCM is a rare subtype of meningioma, with a tendency to present in younger patients and a propensity to recur. Immunohistochemistry plays a vital role in differentiating CCM from other tumors. Brain invasion, atypia and MIB-1 labeling index are likely to predict the recurrence. The extent of resection might be connected with the prognosis.