Chordomas are rare, malignant bone tumors of the skull-base and axial skeleton. Until recently, there was no consensus among experts regarding appropriate clinical management of chordoma, resulting in inconsistent care and suboptimal outcomes for many patients. To address this shortcoming, the European Society of Medical Oncology (ESMO) and the Chordoma Foundation, the global chordoma patient advocacy group, convened a multi-disciplinary group of chordoma specialists to define by consensus evidence-based best practices for the optimal approach to chordoma. In January 2015, the first recommendations of this group were published, covering the management of primary and metastatic chordomas. Additional evidence and further discussion were needed to develop recommendations about the management of local-regional failures. Thus, ESMO and CF convened a second consensus group meeting in November 2015 to address the treatment of locally relapsed chordoma. This meeting involved over 60 specialists from Europe, the United States and Japan with expertise in treatment of patients with chordoma. The consensus achieved during that meeting is the subject of the present publication and complements the recommendations of the first position paper.

✓The history of the endoscope exemplifies the manner in which technological advances influence medicine and surgery. Endoscopic systems have evolved and improved, and they currently provide detailed visualization of a variety of deep organ structures. Otorhinolaryngological surgeons have used the endoscope for more than 30 years. In the 1990s, a number of influential neurosurgeons and otorhinolaryngological surgeons began performing purely endoscopic pituitary surgery. Endoscopic transsphenoidal operations are now extending beyond the sella. The collaboration between otorhinolaryngologists and neurosurgeons has produced a new subspecialty of “endoscopic skull base surgery.” There is a great deal of progress still to be made in developing skills, instruments, and improving skull base repair. The extended skull base approaches allow surgical exposures from the olfactory groove to C-2 and to the infratemporal region and jugular fossa laterally. This article discusses the history of the endoscope, the pivotal technological advances, and the key figures in the burgeoning field of endoneurosurgery.

Aim: To gather data regarding factors predicting responsiveness to pasireotide in acromegaly.Patients and methods: SSTR2a, SSTR3, SSTR5, AIP, Ki-67 and the adenoma subtype were evaluated in somatotroph adenomas from 39 patients treated post-operatively with somatostatin analogues (SSAs). A standardized SSTR scoring system was applied (scores 0–3). All patients received first-generation SSAs, and 11 resistant patients were subsequently treated with pasireotide LAR.Results: None of the patients with negative or cytoplasmic-only SSTR2a expression (scores 0–1) were responsive to first-generation SSAs, as opposed to 20% (score 2) and 50% of patients with a score of 3 (P=0.04). None of the patients with an SSTR5 score of 0–1 were responsive to pasireotide, as opposed to 5/7 cases with a score of 2 or 3 (P=0.02). SSTR3 expression did not influence first-generation SSAs or pasireotide responsiveness. Tumours with low AIP were resistant to first-generation SSAs (100 vs 60%; P=0.02), while they had similar responsiveness to pasireotide compared to tumours with conserved AIP expression (50 vs 40%; P=0.74). Tumours with low AIP displayed reduced SSTR2 (SSTR2a scores 0–1 44.4 vs 6.7%; P=0.006) while no difference was seen in SSTR5 (SSTR5 scores 0–1 33.3 vs 23.3%; P=0.55). Sparsely granulated adenomas responded better to pasireotide compared to densely granulated ones (80 vs 16.7%; P=0.04).Conclusion: The expression of SSTR5 might predict responsiveness to pasireotide in acromegaly. AIP deficient and sparsely granulated adenomas may benefit from pasireotide treatment. These results need to be confirmed in larger series of pasireotide-treated patients.

Since its inception, one of the major issues in transsphenoidal surgery has been the adequate visualization of anatomical structures. As transsphenoidal surgery evolved, technical advancements improved the surgical view of the operative field and the orientation. The operating microscope replaced Cushing's headlight and Dott's lighted speculum retractor, and fluoroscopy provided intraoperative imaging. These advances led to the modern concept of micro-surgical transsphenoidal procedures in the early 1970s. For the past 30 years the endoscope has been used for the treatment of diseases of the sinus and, more recently, in the surgical treatment of pituitary tumors. The collaboration between neurological and otorhinolaryngological surgeons has led to the development of novel surgical procedures for the treatment of various pathological conditions in the skull base. In this paper the authors review the history of the endoscope—its technical development and its application—from the first endoscope described by Philipp Bozzini to the First World Congress of Endoscopic Skull Base Surgery held in 2005 in Pittsburgh, Pennsylvania. Specifically, in this review the history of endoscopy and its application in endonasal neurosurgery are presented.

OBJECTIVE: The endoscope has recently been applied to the supradiaphragmatic transsphenoidal approach, but only case reports dealing with different pathological features have been described. The authors present their experience with this technique in 10 patients with craniopharyngiomas. METHODS: A pure endoscopic endonasal technique was used. From November 1998 through May 2005, four males and six females with a craniopharyngioma, either purely supradiaphragmatic (six patients) or with a significant suprasellar component (four patients), were treated. The tumors had a mean diameter of 2.9 cm (range, 1–4 cm); four patients had a major prechiasmatic component and six had a retrochiasmatic one. RESULTS: Seven total, one subtotal, and two partial resections were obtained. Vision symptoms improved significantly in six out of eight patients. Endocrine function did not improve after surgery, and diabetes insipidus was the most frequent deficit, although it was transient in five out of eight patients. Cerebrospinal fluid leak was the most frequent complication and required reoperation in two patients. Postoperative obesity occurred in two patients. No recurrence has yet been documented in the total resection group. The mean follow-up period is 37 months (range, 3–75 mo). CONCLUSION: The endoscopic technique allows results comparable with the best microscopic series. We think that this technique increases the safety of the procedure because of improved vision. Further studies are required to better define the exact location of the tumor with respect to the arachnoidal plane, the extra-arachnoidal craniopharyngioma being the most suitable for a radical removal using a transsphenoidal supradiaphragmatic approach.

Transorbital endoscopic approaches are increasing in popularity as they provide corridors to reach various areas of the ventral skull base through the orbit. They can be used either alone or in combination with different approaches when dealing with the pathologies of the skull base. The objective of the current study is to evaluate the surgical anatomy of transorbital endoscopic approaches by cadaver dissections as well as providing objective clinical data on their actual employment and morbidity through a systematic review of the current literature. Four cadaveric specimens were dissected, and step-by-step dissection of each endoscopic transorbital approach was performed to identify the main anatomic landmarks and corridors. A systematic review with pooled analysis of the current literature from January 2000 to April 2020 was performed and the related studies were analyzed. Main anatomical landmarks are presented based on the anatomical study and systematic review of the literature. With emphasis on the specific transorbital approach used, indications, surgical technique, and complications are reviewed through the systematic review of 42 studies (19 in vivo and 23 anatomical dissections) including 193 patients. In conclusion, transorbital endoscopic approaches are promising and appear as feasible techniques for the surgical treatment of skull base lesions. Surgical anatomy of transorbital endoscopic approaches can be mastered through knowledge of a number of anatomical landmarks. Based on data available in the literature, transorbital endoscopic approaches represent an important complementary that should be included in the armamentarium of a skull base team.