Xiaohui Ren, Junmei Wang, Mengqing Hu, Haihui Jiang, Jun Yang and Zhongli Jiang
Intracranial and intraspinal malignant peripheral nerve sheath tumors (MPNSTs) are rarely reported because of their extremely low incidence. Knowledge about these tumors is poor. In this study the authors aimed to analyze the incidence and clinical, radiological, and pathological features of intracranial and intraspinal MPNSTs.
Among 4000 cases of intracranial and intraspinal PNSTs surgically treated between 2004 and 2011 at Beijing Tiantan Hospital, cases of MPNST were chosen for analysis and were retrospectively reviewed. To determine which parameters were associated with longer progression-free survival (PFS) and overall survival (OS), statistical analysis was performed.
Malignant PNSTs accounted for 0.65% of the entire series of intracranial and intraspinal PNSTs. Twenty-four (92.3%) of these 26 MPNSTs were primary. Radiologically, 26.9% (7 of 26) of the MPNSTs were misdiagnosed as nonschwannoma diseases. Twenty-one patients were followed up for 1.5 to 102 months after surgery. Twelve patients experienced tumor recurrence, and median PFS was 15.0 months. The 2- and 3-year PFS rates were 47.7% and 32.7%, respectively. Five patients died of tumor recurrence, and median OS was not available. The 2- and 3-year OS rates were 74.7% and 64.0%, respectively. Univariate analysis revealed that female sex, total tumor removal, and primary MPNSTs were significantly associated with a better prognosis. Multivariate analysis revealed that only total removal was an independent prognostic factor for both PFS and OS.
Malignant PNST within the skull or spinal canal is a rare neoplasm and is seldom caused by benign schwannomas. Radiologically, intracranial or intraspinal MPNST should be differentiated from meningioma, chordoma, fibrous dysplasia of bone, and ear cancer. Total resection whenever possible is necessary for the prolonged survival of patients, especially males.
Zhiqin Lin, Chengjun Wang, Zhenwen Gao, Xiangrong Li, Folin Lan, Tianqing Liu, Yongzhi Wang and Zhongli Jiang
Trapped temporal horn (TTH) is a rare subtype of loculated hydrocephalus that is often managed surgically. The natural history of TTH is not well understood, and there are few data on the outcomes of conservative management of this condition. The aim of this study was to analyze the clinical features and outcomes of conservatively and surgically managed cases of TTH.
The authors retrospectively reviewed the clinical data for 19 consecutive cases of TTH that developed after microsurgical resection of lateral ventricular trigone meningioma between 2011 and 2015.
The 19 cases involved 6 male and 13 female patients (mean age [± SD] 39.9 ± 13.8 years). The mean time interval from tumor resection to onset of TTH was 3.2 ± 3.0 months (range 3 days–10 months). Symptoms of intracranial hypertension were the most common complaints at presentation. The mean Karnofsky Performance Scale (KPS) score at onset was 52.1 ± 33.3 (range 10–90). Midline shift was observed in 15 cases (78.9%), and the mean amount of midline shift was 6.0 ± 4.8 mm (range 0–15 mm). Eleven cases (57.9%) were managed with surgical intervention, while 8 cases (42.1%) were managed conservatively. All patients (100%) showed improved clinical status over the course of 4.8 ± 1.0 years (range 2.8–6.3 years) of follow-up. The mean KPS score at last follow-up was 87.9 ± 11.3 (range 60–100). Eighteen patients (94.7%) showed signs of radiographic improvement, and 1 patient (5.3%) exhibited stable size of the temporal horn. Significant differences were observed between the surgical and nonsurgical cohorts for the following variables: KPS score at onset, presence of intracranial hypertension, and midline shift. The mean KPS score at onset was greater (better) in the nonsurgical group than in the surgical group (82.5 ± 8.9 vs 30 ± 25.7, p = 0.001). A greater proportion of patients in the surgical group presented with symptoms of intracranial hypertension (81.8% vs 0%, p = 0.001). The extent of midline shift was greater in the surgical group than in the nonsurgical group (9.0 ± 3.8 mm vs 2.0 ± 2.4 mm, p = 0.001).
The majority of patients with TTH presented in a delayed fashion. TTH is not always a surgical entity. Spontaneous resolution of TTH may be under-reported. Conservative management with clinical and radiological follow-up is effective in selected patients.
Xiaohui Ren, Song Lin, Zhongcheng Wang, Lin Luo, Zhongli Jiang, Dali Sui, Zhiyong Bi, Yong Cui, Wenqing Jia, Yan Zhang, Lanbing Yu and Siyuan Chen
Most intracranial epidermoid cysts typically present with long T1 and T2 signals on MR images. Other epidermoid cysts with atypical MR images are often misdiagnosed as other diseases. In this study the authors aimed to analyze the incidence and the clinical, radiological, and pathological features of atypical epidermoid cysts.
Among 428 cases of intracranial epidermoid cysts that were surgically treated between 2002 and 2008 at Beijing Tiantan Hospital, cases with an atypical MR imaging appearance were chosen for analysis. Clinical and pathological parameters were recorded and compared in patients with lesions demonstrating typical and atypical MR appearance.
An atypical epidermoid cyst accounts for 5.6% of the whole series. Radiologically, 58.3% of atypical epidermoids were misdiagnosed as other diseases. Compared with a typical epidermoid cyst, atypical epidermoid lesions were significantly larger (p = 0.016, chi-square test). Pathologically, hemorrhage was found in 21 patients with atypical epidermoid cyst and is significantly correlated with granulation (p = 0.010, Fisher exact test). Old hemorrhage was found in 13 cases and was significantly correlated with cholesterol crystals. Twenty-one patients were followed up for 1.3–8.6 years after surgery. The 5- and 8-year survival rates were both 100%. Three patients experienced cyst recurrence. The 5- and 8-year recurrence-free rates were 95% and 81.4%, respectively.
Radiologically, an atypical epidermoid cyst should be differentiated from dermoid cyst, teratoma, schwannoma, glioma, craniopharyngioma, and cavernous angioma. A tendency toward spontaneous hemorrhage is confirmed in atypical epidermoid cysts, and a hypothesis was proposed for spontaneously intracystic hemorrhage in atypical epidermoid cysts. Follow-up confirmed long-term survival of patients with atypical epidermoid cysts.