Jian-Kang Shen

Temporal bone chondroblastoma: A review

The objective of this paper was to review temporal bone chondroblastomas in regard to their presentation, radiographic findings, histopathology, and treatment. A case report of a 38‐year‐old man who presented with the left‐sided hearing impairment and temporal swelling was reviewed. A CT scan revealed an osteolytic lobulated expansile mass. MRI depicted two cystic components with fluid–fluid level and enhanced solid mass. Immunohistochemical study of S‐100 was performed using avidin‐biotin‐complex method. The tumor was totally removed, with eroded squamous bone and temporal muscle, via the left zygomatic‐extended middle fossa approach. The pathology of the tumor showed that the tumor cell was spindle‐shaped, along with multinucleated giant cells. These cells had oval to polygonal nuclei; some cells showed grooved nuclei. Intercelluar calcification and hemorrhagic components were also observed in the tumor. Tumor cells were strongly positive for S‐100 protein. Temporal bone chondroblastomas are extremely rare osseous tumors with only 45 cases previously reported in the published literature. They may be confused with more common lesions seen in the temporal bone. Diagnostic radiology, including CT and/or MRI, as well as immunohistochemical staining with S‐100 protein, may assist in making the diagnosis. Treatment is complete surgical excision with preservation of vital neurovascular structures.

Molecular genetics alterations and tumor behavior of sporadic vestibular schwannoma from the People’s Republic of China

Objectives: To analyze the molecular genetic alteration of sporadic vestibular schwannomas from the People’s Republic of China and to correlate these alterations with the tumor behaviors. Methods: Four highly polymorphic microsatellite DNA markers were used to observe the frequency of loss of heterozygosity (LOH) in chromosome 22. The NF2 gene mutations were detected by Polymerase chain reaction-single-strand conformation polymorphism (PCR-SSCP) and DNA sequencing. The schwannomin/merlin (S/M) expression was examined using anti-NF2 (A-19) IgG under immunohistochemistry and western blot. The proliferative index (LI) of vestibular schwannoma was evaluated by proliferative cell nuclear antigen investigation. Results: Sixteen vestibular schwannomas (44.4%) showed allele loss. We found 22 mutations in 36 schwannomas. The LI and the growth rate of schwannomas with LOH or mutation were significantly higher than those without LOH or mutation. All of these vestibular schwannomas showed no immunoreaction to anti-NF2(A-19) IgG by immunohistochemistry. By immunoblotting technique, reduced expression of S/M was found in 31 cases (86%). The growth index of schwannomas with severely reduced expression of S/M was significantly higher than those with moderately reduced or normal expression. Conclusion: The molecular genetic changes in sporadic vestibular schwannomas from Chinese patients were similar to the previous reports. We demonstrate the relationship between tumor behaviors and genetic alteration (including LOH and mutation of NF2 gene). We propose that inactivation of S/M, may be an important step in tumorigenesis of sporadic vestibular schwannoma.

Diagnosis and therapy for Cushing’s disease with negative dynamic MRI finding: a single-centre experience

Objective  Most patients with Cushing’s disease (CD) will have evidence of an adenoma on high‐resolution magnetic resonance imaging (MRI). However, a significant minority of patients with CD and biochemical hypercortisolaemia have no visible adenoma on MRI. The purpose of this study was to evaluate the diagnostic value of preoperative biochemical assessment and accuracy of MRI findings on surgical outcome.

Cauda equina hemangioblastoma at L5 vertebral level related to von Hippel-Lindau disease.

Hemangioblastoma (HB) is uncommon, with only a few cases of hemangioblastoma with von Hippel–Lindau disease (VHL) located in cauda equina previously reported. We describe a case of hemangioblastoma misdiagnosed as nerve sheath tumour in the cauda equina.

Volumetric MRI Analysis in Patients with Short-Term Remission of Cushing's Disease

The data on patients with short‐term remission of Cushings disease (CD) might provide information that is not available from previous long‐term remission studies. We aimed to investigate structural changes in the brain in these patients and to examine whether these changes were associated with clinical characteristics.

Altered spontaneous brain activity in Cushing's disease: a resting‐state functional MRI study

Cushings disease (CD) provides a unique and naturalist model for studying the influence of hypercortisolism on the human brain and the reversibility of these effects after resolution of the condition. This cross‐sectional study used resting‐state fMRI (rs‐fMRI) to investigate the altered spontaneous brain activity in CD patients and the trends for potential reversibility after the resolution of the hypercortisolism. We also aim to determine the relationship of these changes with clinical characteristics and cortisol levels.

Altered gray and white matter microstructure in Cushing’s disease: A diffusional kurtosis imaging study

Exposure to chronic hypercortisolism has multiple adverse effects on brain biology in humans. Cushings disease (CD) represents a unique and natural human model for examining the effects of hypercortisolism on the brain. This cross-sectional study used Diffusional Kurtosis Imaging (DKI) to investigate the microstructure alterations in both white matter (WM) and gray matter (GM) of CD patients and to determine the relationship of these changes with clinical characteristics. DKI images were obtained from 15 active CD patients. DKI parametric maps were estimated through voxel-based analyses (VBA) and compared with 15 healthy controls matched for age, sex and education. In addition, correlations were analyzed between the altered DKI parameters and clinical characteristics. Compared with healthy controls, CD patients mainly exhibited significantly altered diffuse parameters in the GM and WM of the left medial temporal lobe (MTL). The mean values of increased radial diffusivity (RD) of CD patients in GM of the left hippocampus/parahippocampal gyrus correlated positively with the clinical severity of CD. Additionally, we also found altered kurtosis parameters in the cerebellum and frontal lobe. DKI imaging of CD patients could represent complementary information in both white matter and gray matter. The impairment of the left MTL might explain some part of the memory and cognition impairments in CD patients.

Surgical Approaches and Techniques for Tuberculum Sellae Meningiomas

Objective To inquire surgical approaches and techniques of tuberculum sellae meningiomas in order to improve the operative effect.Methods A retrospective analysis was made on 47 cases of tuberculum sellae meningioma operated.The tumors operated on with 4 different surgical approaches were classified into large,middle and small types depending upon its size on CT or MRI,with the comparison of total resection rate,operative results and complications among the types.Results Of 47 cases,tumor was total removed in 44cases,subtotal in 3cases.Total resection rate is no relationship with size of tumors and operative approaches(P0.05).Postoperatively,patients recovered well in 43 cases, mild disable in 2 cases,severe disable in 1 case,and dead in 1 case,with mortality of 2.1%.Operative results are no relationship with size of tumors and operative approaches(P0.05).Conclusion Tuberculum sellae meningiomas can be removed completely and safely with higher total resection rate,less postoperative complications and better operative results in majority of cases.The more appropriate approaches are the supraorbital keyhole approach in small and middle types,the unilateral subfrontal or pterional approach in middle and large types and the frontotemporal orhitozygomatic approach in large type. Whichever operative approach was used,the techniques of removing meningioma were same,and were the key to total remove it.