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Can J Ophthalmol 2021 Jun 4. Epub 2021 Jun 4.

Department of Ophthalmology and Visual Sciences, McGill University, and McGill Academic Eye Centre, Montreal, Que.. Electronic address:

Radiol Case Rep 2021 Jul 19;16(7):1840-1844. Epub 2021 May 19.

Departments of Radiology, M.D. PGY1 Radiology Resident at King Khalid University Hospital, Riyadh, Saudi Arabia.

Chordoma is a rare and aggressive intracranial bone tumor that is difficult to diagnose and resect with a peak incident between the ages of 20-40 years old and high recurrence rate when not completely resected. We present the case of clival chordoma in an 11-year-old female patient, who reported with a chronic right-sided headache, progressive loss of vision, hoarseness of voice, and slurred speech. Fluid-fluid level on fluid-attenuated inversion recovery magnetic resonance imaging sequence can be an atypical radiological sign for clival chordoma. Read More

Immunotherapy 2021 Jun 3. Epub 2021 Jun 3.

Department of Hematology-Oncology, Faculty of medicine, Saint-Joseph University, Beirut, Lebanon.

Cancers (Basel) 2021 May 30;13(11). Epub 2021 May 30.

Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Bethesda, MD 20892, USA.

Background: Chordoma is a rare bone cancer with an unknown etiology. TBXT is the only chordoma susceptibility gene identified to date; germline single nucleotide variants and copy number variants in TBXT have been associated with chordoma susceptibility in familial and sporadic chordoma. However, the genetic susceptibility of chordoma remains largely unknown. Read More

Cancers (Basel) 2021 May 25;13(11). Epub 2021 May 25.

Department of Radiation Oncology, Gunma University Graduate School of Medicine, 3-39-15, Showa-machi, Maebashi 371-8511, Gunma, Japan.

Carbon-ion radiotherapy (CIRT) represents a definitive treatment for inoperable bone and soft tissue sarcoma (BSTS). This prospective study analyzed 61 patients with inoperable BSTS who were treated with CIRT to evaluate QOL, functional outcomes, and predictive factors in patients with inoperable BSTS treated with definitive CIRT. The Musculoskeletal Tumor Society (MSTS) scoring system and the Short Form (SF)-8 questionnaire were completed before and at 1, 3, 6, 12, and 24 months after CIRT. Read More

Cancers (Basel) 2021 May 17;13(10). Epub 2021 May 17.

Department of Neurological Surgery, The University of Texas Southwestern Medical Center, Dallas, TX 75235, USA.

Chordomas and chondrosarcomas are rare but devastating neoplasms that are characterized by chemoradiation resistance. For both tumors, surgical resection is the cornerstone of management. Immunotherapy agents are increasingly improving outcomes in multiple cancer subtypes and are being explored in chordoma and chondrosarcoma alike. Read More

Acta Otorhinolaryngol Ital 2021 Apr;41(Suppl. 1):S30-S41

Department of Neurosciences DNS, Neurosurgery Section, Padua University, Padua, Italy.

Introduction: The endoscopic endonasal transsphenoidal approach to the sella and parasellar regions is now increasingly used for removal of a variety of lesions localized in the ventral skull base. The advantage of the endoscope is enhanced visualization and improved panoramic view that can result in more complete removal of the tumor. An extensive knowledge of the anatomy is mandatory to approach this region. Read More

Carcinogenesis 2021 May 31. Epub 2021 May 31.

Key Laboratory of Genomic and Precision Medicine, Beijing Institute of Genomics, Chinese Academy of Sciences, China National Center for Bioinformation, Beijing 100101, China.

Chordoma is a rare bone tumor arising from notochordal remnants, but the underlying mechanism remains elusive. By integrated mRNA and microRNA analyses, we found significant downregulation of TGFB3 along with upregulation of its inhibitor, miR-29 family in chordoma comparing with notochord. Somatic copy number gains of miR-29 loci in chordoma highlighted a mechanism of inactivation of TGFB3 signaling in tumor formation. Read More

Onco Targets Ther 2021 12;14:3109-3118. Epub 2021 May 12.

Department of Neurosurgery, Tangshan People's Hospital, Tangshan, Hebei, People's Republic of China.

Objective: MicroRNAs have been implicated in the progression of various cancers. However, the role of microRNAs in chordoma remains to be further elucidated. Here, we purposed to character the role of two microRNAs, miR-17 and miR-93, and their potential mechanisms in chordoma. Read More

Semin Diagn Pathol 2021 May 12. Epub 2021 May 12.

Department of Pathology and Laboratory Medicine, Division of Bone and Soft tissue, University of Miami, Sylvester Comprehensive Cancer Center, and Jackson Memorial Hospitals, Miami, FL, USA. Electronic address:

Primary malignant bone tumors are uncommon and their accurate classification requires careful correlation of clinical, radiological, and pathologic findings. It is a heterogeneous group of tumors with a wide spectrum of morphology and their biological potential can be of low- or high-grade, depending on their risk for developing metastases. Over the past several decades, the classification of bone sarcomas has remained largely constant. Read More

Global Spine J 2021 Jun;11(5):740-750

Department of Neurological Surgery, 4002The University of Texas MD Anderson Cancer Center, Houston, TX, USA.

Study Design: Retrospective case series.

Objective: To determine predictive factors of overall survival (OS) and local recurrence (LR), report complications, and assess the impact of complications on survival, recurrence, and function in patients undergoing en bloc resection of sacral chordoma.

Methods: This retrospective case series was obtained from a prospective database (1995-2016). Read More

J Korean Neurosurg Soc 2021 May 28. Epub 2021 May 28.

Department of Neurosurgery, Seoul National University Hospital, Seoul, Korea.

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. Read More

J Family Med Prim Care 2021 Feb 27;10(2):1047-1049. Epub 2021 Feb 27.

Department of Pathology and Lab Medicine, All India Institute of Medical Sciences, Jodhpur, Rajasthan, India.

Follicular thyroid carcinoma (FTC) presenting as an isolated spinal metastasis with features of compression myelopathy is extremely rare with <10 cases reported in world literature. FTC is the second most common thyroid cancer with an indolent course. It predominantly occurs in females. Read More

Photodiagnosis Photodyn Ther 2021 May 23:102346. Epub 2021 May 23.

Department of Neurosurgery, Medical Faculty, Heinrich Heine University, Düsseldorf, Germany. Electronic address:

Background: Chordoma are uncommon aggressive tumors of the skeleton. Surgical resection is often subtotal and adjuvant treatment possibilities are limited as chordomas are highly chemo- and radioresistant. In the present study we examined the impact of 5-ALA PDT on different human chordoma cell lines. Read More

J Orthop Sci 2021 May 22. Epub 2021 May 22.

Department of Orthopedic Surgery, Yamaguchi University Graduate School of Medicine, 1-1-1 Minami-Kogushi, Ube, Yamaguchi 755-8505, Japan.

BMC Neurol 2021 May 22;21(1):207. Epub 2021 May 22.

Department of Neurosurgery, Tohoku University Graduate School of Medicine, Sendai, Miyagi, Japan.

Background: Treating chordoma through surgery alone is often ineffective. Thus, surgery often performed with irradiation, with a reported 5-year survival rate of 60-75%. The clinical course varies, and disease rarity prevents larger number of clinical investigations. Read More

Respir Physiol Neurobiol 2021 May 18;291:103693. Epub 2021 May 18.

Department of Otolaryngology Head and Neck Surgery, The Second Affiliated Hospital of Xi'an Jiaotong University, Xi'an, Shaanxi, 710004, China. Electronic address:

Objectives: To study the effect of endoscopic endonasal surgery on nasal function for the treatment of clival chordoma.

Methods: Pre and post-operative computed tomography (CT) scans of a case of chordoma treated with an endoscopic endonasal approach (EEA) were collected retrospectively, and models of the nasal cavity were reconstructed so that a subsequent numerical simulation of nasal airflow characteristics, warming, and humidification could be conducted.

Results: Middle turbinectomy resulted in redistribution of airflow within the nasal cavity, and the most significant changes occurred in the middle section. Read More

Front Oncol 2021 29;11:649958. Epub 2021 Apr 29.

Department of Neurosurgery, The First Affiliated Hospital of Xiamen University, School of Medicine, Xiamen University, Xiamen, China.

Background: Diagnosing the well-known concomitant Rathke's cleft cyst (RCC) and differentiating it from other sellar lesions are difficult because RCC is and other sellar lesions are closely related and represent a continuum from simple RCCs to more complex lesions. The purpose of this study is to better understand the adeno- and neurohypophysis adjacent to the par intermedia remnants and their role in the origin of the coexistence of these two distinct tumor neoplasias; to assess the incidence in different age groups; to categorize the pathohistological subtype, which can be incorporated in predictive/prognostic models; and finally, to evaluate the current evidence on collision tumors of the sellar embryonic remnant tract in terms of their biological behavior and pathology.

Methods: Utilizing the PubMed database, data were collected from 1920 to 2019. Read More

Ann Diagn Pathol 2021 May 10;53:151760. Epub 2021 May 10.

Pathology Departments, University Hospital of Saint-Etienne, France. Electronic address:

Introduction: Chordomas are rare malignant midline tumors, presumed to arise from notochordal remnants. This was further suggested by the discovery of the brachyury in chordomas pathogenesis. Its immunohistochemical expression has become the principal adjunct in the diagnosis of chordomas. Read More

Neurosurgery 2021 May 14. Epub 2021 May 14.

Department of Neurosurgery, Lariboisière Hospital, Assistance Publique - Hôpitaux de Paris, University of Paris, Paris, France.

Background: Currently, different postoperative predictors of chordoma recurrence have been identified. Tumor growth rate (TGR) is an image-based calculation that provides quantitative information of tumor's volume changing over time and has been shown to predict progression-free survival (PFS) in other tumor types.

Objective: To explore the usefulness of TGR as a new preoperative radiological marker for chordoma recurrence. Read More

Bioorg Med Chem Lett 2021 Jul 2;43:128078. Epub 2021 May 2.

Department of Pharmacology, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, NC 27599, USA. Electronic address:

Heteroatom rich 1,2,3-dithiazoles are relatively underexplored in medicinal chemistry. We now report screening data on a series of structurally diverse 1,2,3-dithiazoles and electronically related 1,2,4-dithiazines with the aim of identifying interesting starting points for potential future optimisation. The 1,2,3-dithiazoles, were obtained via a number of different syntheses and screened on a series of cancer cell lines. Read More

Cancers (Basel) 2021 Apr 30;13(9). Epub 2021 Apr 30.

Pathology Department, University Hospital of Saint-Etienne, 42055 Saint-Etienne, France.

Chordomas are notably resistant to chemotherapy. One of the cytoprotective mechanisms implicated in chemoresistance is autophagy. There are indirect data that autophagy could be implicated in chordomas, but its presence has not been studied in chordoma tissues. Read More

Neurosurg Focus 2021 May;50(5):E17

1Department of Neurosurgery, Johns Hopkins University School of Medicine, Baltimore, Maryland.

Objective: Long-term local control in patients with primary chordoma and sarcoma of the spine and sacrum is increasingly reliant upon en bloc resection with negative margins. At many institutions, adjuvant radiation is recommended; definitive radiation is also recommended for the treatment of unresectable tumors. Because of the high off-target radiation toxicities associated with conventional radiotherapy, there has been growing interest in the use of proton and heavy-ion therapies. Read More

Oper Neurosurg (Hagerstown) 2021 Apr 30. Epub 2021 Apr 30.

Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.

Chordoma is not a benign disease. It grows invasively, has a high rate of local recurrence, metastasizes, and seeds in the surgical field.1 Thus, chordoma should be treated aggressively with radical resection that includes the soft tissue mass and the involved surrounding bone that contains islands of chordoma. Read More

Oper Neurosurg (Hagerstown) 2021 Apr 30. Epub 2021 Apr 30.

Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.

Epidermoid tumors arise from misplaced squamous epithelium and enlarge through the accumulation of desquamated cell debris.1 Notwithstanding the prevailing conservative attitudes to minimize morbidity, optimal treatment consists of total removal of the capsule2,3; therefore, giant and multicompartmental tumors are particularly challenging. The utilization of simultaneous endoscopic microscopic techniques by tandem endoscopic and microscopic dissection to overcome the shortcomings of both modalities, markedly enhances the ability of radical removal,4 thus eliminating or at least long-delaying inevitable recurrences with subsequent accumulated morbidity. Read More

Acta Neurochir (Wien) 2021 Apr 26. Epub 2021 Apr 26.

Division of Neurosurgery, Department of Biotechnology and Life Sciences, University of Insubria, Varese, Italy.

Objective: This paper highlights the management of 5 patients affected by symptomatic ecchordosis physaliphora (EP), treated via endoscopic endonasal transsphenoidal-transclival approach and contextual multilayer skull base reconstruction. A detailed analysis of each case is provided, along with the review of the current body of literature.

Methods: A retrospective review of patients treated by means of endoscopic endonasal approach for EP from 2010 to 2020 in the Otolaryngology and Neurosurgery Departments of a tertiary-care referral center for endoscopic skull base surgery was analyzed. Read More

Radiol Case Rep 2021 Jun 1;16(6):1320-1324. Epub 2021 Apr 1.

Department of Endocrinology & Diabetes, Royal Brisbane and Women's Hospital, Brisbane, Queensland, Australia.

Ectopic pituitary adenomas are a rare clinical entity and are frequently mistaken for other base of skull lesions on imaging. We report the clinical presentation and management of a woman presenting with an ectopic prolactinoma located in the clivus. A 66-year-old female presented with a 6-month history of headaches and light-headedness. Read More

Target Oncol 2021 05 24;16(3):325-337. Epub 2021 Apr 24.

Brain Tumor Stem Cell Laboratory, Department of Neurologic Surgery, Mayo Clinic, 4500 San Pablo Rd. S, Jacksonville, FL, 32224, USA.

Background: Chordoma is a rare but devastating tumor that arises in the cranial skull base or spine. There are currently no US Food and Drug Administration-approved targeted therapies for chordoma, and little understanding of whether using more than one therapy has benefit over monotherapy.

Objective: The objective of this study was to systematically review the current status of clinical trials completed for patients with chordoma to determine if multimodal therapy offers a benefit in progression-free survival over monomodal therapy. Read More

Oper Neurosurg (Hagerstown) 2021 Apr;20(5):E344-E345

Department of Neurosurgery, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts.

Recurrent skull base chordomas are challenging lesions. They already had maximum radiation, and in the absence of any effective medical treatment, surgical resection is the only treatment.1,2 Surgery on recurrent previously radiated chordomas, however, carries much higher risk and the likelihood of subtotal resection. Read More

Acta Neurochir (Wien) 2021 Apr 13. Epub 2021 Apr 13.

Department of Neurosurgery, Microsurgical Neuroanatomy Laboratory, Cerrahpasa Medical Faculty, Istanbul University-Cerrahpasa, Istanbul, Turkey.

Background: Surgical access to the ventral pontomedullary junction (PMJ) can be achieved through various corridors depending on the location and extension of the lesion. The jugular tubercle (JT), a surgically challenging obstacle to access the PMJ, typically needs to be addressed in transcranial exposures. We describe the endoscopic endonasal transclival approach (EETCA) and its inferolateral transtubercular extension to assess the intradural surgical field gained through JT removal. Read More