BACKGROUND: Invasive nonfunctioning pituitary adenomas (NFPAs) remain challenging due to their high complication rate and poor prognosis. We aimed to identify the distinctive molecular signatures of invasive NFPAs, compared with noninvasive NFPAs, using gene expression profiling by RNA sequencing. METHODS: We obtained frozen fresh tissue samples from 14 patients with NFPAs who underwent primary transsphenoidal surgery. Three non-invasive and 11 invasive NFPAs were used for RNA sequencing. The bioinformatics analysis included differential gene expression, gene ontology analysis, and pathway analysis. RESULTS: A total of 700 genes were differentially expressed (59 up-regulated and 641 down-regulated genes) between invasive and non-invasive NFPAs (false discovery rate <0.1, and |fold change| ≥2). Using the down-regulated genes in invasive NFPAs, gene ontology enrichment analyses and pathway analyses demonstrated that the local immune response was attenuated and that transforming growth factor-β (TGF-β) RII-initiated TGF-β signaling was down-regulated in invasive NFPAs. The overexpression of claudin-9 (CLDN9) and the down-regulation of insulin-like growth factor-binding protein 5 (IGFBP5), death-associated protein kinase 1 (DAPK1), and tissue inhibitor of metalloproteinase-3 (TIMP3) may be related with invasiveness in NFPAs. CONCLUSION: Invasive NFPAs harbor different gene expression profiles relative to noninvasive NFPAs. In particular, local suppression of the immune response and TGF-β signaling can make PAs prone to invasiveness.
Computational Biology ; Death-Associated Protein Kinases ; Down-Regulation ; Gene Expression ; Gene Expression Profiling ; Gene Ontology ; Humans ; Pituitary Neoplasms ; Prognosis ; Sequence Analysis, RNA ; Tissue Inhibitor of Metalloproteinase-3 ; Transcriptome

The endoscopic endonasal approach (EEA) has been popularized in adults and has been applied to an expanding range of surgical modules and indications in this population. However, its clinical application in pediatric neurosurgery has been impeded by the differences in anatomical features and the relatively low incidence of diseases to which it is applicable. In this review article, we mainly discuss the surgical indications, feasibility, and complications of EEA for suprasellar lesions in children based on a review of the literature, focusing especially on the age-related anatomical features of the nasal cavity, various pathologic entities, and the impact of EEA on long-term craniofacial growth.
Adult ; Child* ; Humans ; Incidence ; Nasal Cavity ; Neurosurgery

OBJECTIVE: In this study, we retrospectively evaluate the surgical outcome of the cervical fracture-dislocation in order to define the criteria for the operative approach selection based on fracture characteristics. METHODS: Thirty one consecutive patients (29 males and 2 females) who underwent operation for the cervical fracture-dislocation between 1997 and 2001 at a single institute were included in this study. Plain X-ray, computed tomography, and magnetic resonance imaging studies were performed in all patients. Injuries were characterized using Denis's three-column plain X-ray model, Daffer's computed tomography, and Oner's magnetic resonance imaging classifications. The Frankel classification was used for neurological deficits. The mean postoperative follow-up period was 11.93 months (range 1-61 months). RESULTS: The anterior approach was performed in 14 and the posterior approach in 10 of the 31 patients, respectively. Both anterior and posterior fixation was performed electively in 7 of the 31 patients. Daffer and Oner's classification was found to be able to predict surgical failures by either the anterior or the posterior approach. Three-column injury was misinterpreted as two-column injury only by plain radiography. No differences in neurological outcome, pain relief, or bone fusion rate were observed between the anterior approach and the posterior approach. However, the posterior approach was associated more frequently with postoperative kyphosis and instability. CONCLUSION: Surgical approach is usually determined on the basis of whether the compression is ventral or dorsal. Anterior fixation only may be an alternative to both anterior and posterior fixation in three-column injury, but posterior fixation alone is not.
Classification ; Follow-Up Studies ; Humans ; Kyphosis ; Magnetic Resonance Imaging ; Male ; Radiography ; Retrospective Studies

The small pituitary mass was incidentally found in 40-years-old women with renal cell carcinoma. The endocrinological and ophthalmological evaluation revealed no deficit and the short-term follow-up was recommended. In 6 months later, the visual disturbance was reported and the size of mass was increased. The tumor was removed totally via the trans-sphenoid approach. The post-operative endocrinological insufficiency was not noticed. During one year of follow-up period, there was no evidence of recurrence without adjuvant radiotherapy. The clinical features of pituitary metastasis from renal cell carcinoma were similar to those of pituitary adenoma. The possibility of pituitary metastasis should be kept in mind in patients with sellar mass and renal cell carcinoma.
Carcinoma, Renal Cell ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Neoplasm Metastasis ; Pituitary Neoplasms ; Radiotherapy, Adjuvant ; Recurrence

Idiopathic granulomatous hypophysitis (IGH), a rare disease, requires differentiation from more common mass lesions of the sella such as pituitary adenoma, craniopharyngioma, Rathke's cleft cyst, or pituitary tuberculoma. IGH usually presents with an insidious onset of visual defects and headaches. On the other hand, rapid onset of neurologic and visual symptoms in an IGH patient is exceptionally rare. Here, we present a biopsy-proven case of IGH with rapid onset and satisfactory outcome after high dose steroid treatment.
Adult ; Autoimmune Hypophysitis ; Craniopharyngioma ; Endoscopy ; Glucocorticoids ; Hand ; Headache ; Humans ; Pituitary Gland ; Pituitary Neoplasms ; Rare Diseases ; Sella Turcica ; Tuberculoma

Objective: : The skull base reconstruction step, which prevents cerebrospinal fluid (CSF) leakage, is one of the most challenging steps in endoscopic skull base surgery (ESS). The purpose of this study was to assess the outcomes and complications of a reconstruction technique for immediate CSF leakage repair using multiple onlay grafts following ESS. Methods: : A total of 230 consecutive patients who underwent skull base reconstruction using multiple onlay grafts with fibrin sealant patch (FSP), hydroxyapatite cement (HAC), and pedicled nasoseptal flap (PNF) for high-flow CSF leakage following ESS at three institutions were enrolled. We retrospectively reviewed the medical and radiological records to analyze the preoperative features and postoperative results. Results: : The diagnoses included craniopharyngioma (46.8%), meningioma (34.0%), pituitary adenoma (5.3%), chordoma (1.6%), Rathke’s cleft cyst (1.1%) and others (n=21, 11.2%). The trans-planum/tuberculum approach (94.3%) was the most commonly adapted surgical method, followed by the trans-sellar and transclival approaches. The third ventricle was opened in 78 patients (41.5%). Lumbar CSF drainage was not performed postoperatively in any of the patients. Postoperative CSF leakage occurred in four patients (1.7%) due to technical mistakes and were repaired with the same technique. However, postoperative meningitis occurred in 13.5% (n=31) of the patients, but no microorganisms were identified. The median latency to the diagnosis of meningitis was 8 days (range, 2–38). CSF leakage was the unique risk factor for postoperative meningitis (p<0.001). Conclusion : The use of multiple onlay grafts with FSP, HAC, and PNF is a reliable reconstruction technique that provides immediate and complete CSF leakage repair and mucosal grafting on the skull base without the need to harvest autologous tissue or perform postoperative CSF diversion. However, postoperative meningitis should be monitored carefully.

Objective: : The skull base reconstruction step, which prevents cerebrospinal fluid (CSF) leakage, is one of the most challenging steps in endoscopic skull base surgery (ESS). The purpose of this study was to assess the outcomes and complications of a reconstruction technique for immediate CSF leakage repair using multiple onlay grafts following ESS. Methods: : A total of 230 consecutive patients who underwent skull base reconstruction using multiple onlay grafts with fibrin sealant patch (FSP), hydroxyapatite cement (HAC), and pedicled nasoseptal flap (PNF) for high-flow CSF leakage following ESS at three institutions were enrolled. We retrospectively reviewed the medical and radiological records to analyze the preoperative features and postoperative results. Results: : The diagnoses included craniopharyngioma (46.8%), meningioma (34.0%), pituitary adenoma (5.3%), chordoma (1.6%), Rathke’s cleft cyst (1.1%) and others (n=21, 11.2%). The trans-planum/tuberculum approach (94.3%) was the most commonly adapted surgical method, followed by the trans-sellar and transclival approaches. The third ventricle was opened in 78 patients (41.5%). Lumbar CSF drainage was not performed postoperatively in any of the patients. Postoperative CSF leakage occurred in four patients (1.7%) due to technical mistakes and were repaired with the same technique. However, postoperative meningitis occurred in 13.5% (n=31) of the patients, but no microorganisms were identified. The median latency to the diagnosis of meningitis was 8 days (range, 2–38). CSF leakage was the unique risk factor for postoperative meningitis (p<0.001). Conclusion : The use of multiple onlay grafts with FSP, HAC, and PNF is a reliable reconstruction technique that provides immediate and complete CSF leakage repair and mucosal grafting on the skull base without the need to harvest autologous tissue or perform postoperative CSF diversion. However, postoperative meningitis should be monitored carefully.

Pituitary surgery has advanced considerably in recent years with the exploration and development of various endoscopic approaches and techniques. Different endoscopic skull base approaches are being applied to access sellar tumors in different locations. Moreover, extracapsular dissection and cavernous sinus exploration have enabled gross total resection of sellar tumors where it could not have been achieved in the past. Techniques for skull base reconstruction have also progressed, allowing surgeons to remove larger and more complicated tumors than before. This review article discusses different endoscopic skull base approaches, surgical techniques for removing pituitary adenomas, and reconstruction methods for repairing postoperative low-flow and high-flow cerebrospinal fluid leakage.

Giant cell tumors (GCTs) are locally aggressive primary bone tumors of osteoclast-like cells. Most GCTs occur within the long bones, and primary GCTs involving the clivus are extremely rare. We present the case of an 18-year-old boy with binocular horizontal diplopia with an insidious onset who was found to have a hypointense enhancing mass involving the clivus and left side dorsum sellae on magnetic resonance images. The tumor was completely resected via an endoscopic endonasal transclival approach, and histopathologic examination via immunohistochemistry indicated a GCT. The patient’s left abducens nerve palsy improved slightly after surgery. Because of the rarity of GCTs, there is no consensus about the definitive treatment protocol. However, we suggest that gross total resection is the treatment of choice, and denosumab plays a critical role in patients with subtotal resection.