-
Expert Review of Neurotherapeutics Mar 2018With an annual incidence of 5/100,000, meningioma is the most frequent primary tumor of the central nervous system. Risk factors are radiotherapy and hormone intake.... (Review)
Review
With an annual incidence of 5/100,000, meningioma is the most frequent primary tumor of the central nervous system. Risk factors are radiotherapy and hormone intake. Most meningiomas are grade I benign tumors, but up to 15% are atypical and 2% anaplastic according to the WHO 2016 histological criteria. Areas covered: This review details the current standard therapy based on international guidelines and recent literature, and describes new approaches developed to treat refractory cases. First-line treatments are observation and surgery, but adjuvant radiotherapy/radiosurgery is discussed for atypical and indicated for anaplastic meningiomas. The most problematic cases include skull base meningiomas that enclose vasculo-nervous structures and surgery- and radiation-refractory tumors that present with significant morbidity and mortality. The treatment of recurrent tumors is based on radiotherapy and repeated surgery. Systematic therapies are not effective in general but several clinical trials are ongoing. Expert commentary: Molecular characterization of the tumors, based on genetic mutations such as NF2, SMO, TERT, TRAF7, and on the methylation profile are developing, completing the histological classification and giving new insights into prognosis and treatment options.
Topics: Humans; Meningeal Neoplasms; Meningioma; Mutation; Neoplasm Grading; Neoplasm Recurrence, Local; Prognosis; Radiosurgery; Radiotherapy, Adjuvant; Risk Factors; Skull Base Neoplasms
PubMed: 29338455
DOI: 10.1080/14737175.2018.1429920 -
Veterinary and Comparative Oncology Sep 2014Osteosarcoma in dogs is a heterogeneous disease entity with regard to its histologic, clinical and biologic behaviour. Differences in behaviour are associated with... (Review)
Review
Osteosarcoma in dogs is a heterogeneous disease entity with regard to its histologic, clinical and biologic behaviour. Differences in behaviour are associated with tumour location. Oral and maxillofacial osteosarcomas are typically reported as a component of the broader classifications of axial osteosarcoma or osteosarcoma of flat bones to differentiate them from appendicular osteosarcoma. Similar to human oral and maxillofacial osteosarcoma, in dogs, these also appear to have less aggressive behaviour than appendicular osteosarcoma. Ideally, local control is achieved with wide surgical resection that results in tumour-free margins. Failure of local control is the most common contributor to poor prognosis. Chemotherapy and radiation treatment are reported to have variable outcomes. The aim of this article is to review the literature on oral and maxillofacial osteosarcoma in dogs in comparison to appendicular and axial osteosarcoma. Similarities and differences between oral and maxillofacial osteosarcoma in humans are addressed.
Topics: Animals; Dog Diseases; Dogs; Facial Bones; Humans; Maxillary Neoplasms; Mouth Neoplasms; Osteosarcoma; Prognosis; Skull Neoplasms
PubMed: 22935032
DOI: 10.1111/j.1476-5829.2012.00352.x -
Neuroimaging Clinics of North America May 2022Many different benign and malignant processes affect the central skull base and petrous apices. Clinical evaluation and tissue sampling are difficult because of its deep... (Review)
Review
Many different benign and malignant processes affect the central skull base and petrous apices. Clinical evaluation and tissue sampling are difficult because of its deep location, leaving imaging assessment the primary means for lesion evaluation. Skull base lesions demonstrate a variety of confusing appearances on imaging, creating diagnostic dilemmas. It is important to be familiar with imaging appearances of common mimickers of malignant neoplasm in the skull base. This article familiarizes readers with imaging characteristics of various anatomic variants and benign pathologies that mimic malignant neoplasms, in hopes of increasing confidence of diagnosis, decreasing unnecessary procedures, and allaying patient fear.
Topics: Humans; Magnetic Resonance Imaging; Petrous Bone; Skull Base; Skull Base Neoplasms; Tomography, X-Ray Computed
PubMed: 35526960
DOI: 10.1016/j.nic.2022.02.001 -
Advances in Oto-rhino-laryngology 2020Posttreatment imaging surveillance in patients treated for anterior skull base tumors is a multifaceted issue which - as a first step - requires tailoring of the... (Review)
Review
Posttreatment imaging surveillance in patients treated for anterior skull base tumors is a multifaceted issue which - as a first step - requires tailoring of the selection of imaging technique and acquisition protocol to the clinical scenario. As a general rule, acute symptoms suggesting the onset of a complication of treatment require prompt diagnosis through an easily accessible technique, such as CT; on the other hand, monitoring recurrences in asymptomatic patients is best achieved with MRI, exploiting the inherently higher contrast resolution. The interpretation of follow-up imaging studies is challenging. First of all, it is essential to be aware of specific characteristics of the natural history that may differ significantly between histologies, influencing the pattern and timing of recurrences. Additionally, resection of the lesion and reconstruction of the defect (as well as radiation treatment) produce complex anatomical changes, which may mislead inexperienced radiologists; this concept emphasizes the centrality of collecting accurate information on treatment modalities and procedures applied before scanning in each patient. This enables the pattern of expected posttreatment changes to be anticipated and, consequently, recurrences or complications to be more easily identified.
Topics: Humans; Magnetic Resonance Imaging; Neoplasm Recurrence, Local; Postoperative Complications; Radiotherapy; Skull Base; Skull Base Neoplasms; Tomography, X-Ray Computed
PubMed: 32731227
DOI: 10.1159/000457941 -
Current Pain and Headache Reports Feb 2019Sellar and parasellar lesions are numerous and varying in terms of their patholphysiology and physical and radiographic characteristics but often incite pain syndromes... (Review)
Review
PURPOSE OF REVIEW
Sellar and parasellar lesions are numerous and varying in terms of their patholphysiology and physical and radiographic characteristics but often incite pain syndromes that are similar in semiology. The goal of this review was to familiarize the reader with a variety of sellar and parasellar lesions grouped together based on common clinical symptomatology, with a focus on important imaging characteristics that are often distinguishing features diagnostically.
RECENT FINDINGS
In most cases, tissue acquisition via surgical resection or stereotactic biopsy are the mainstay for definitive diagnosis of sellar and parasellar lesions. With advances in MRI technology in particular in terms of resolution and the inclusion of new techniques including dynamic imaging with delayed contrast, imaging studies of lesions in the sellar and parasellar regions have become increasingly important for diagnostic purposes, with pituitary adenomas and schwannomas as prime examples. In the case of chordoid gliomas, molecular features of the tumor also help distinguish it from other disease processes similar in presentation, which have dramatic impacts on management. Advances in surgical approaches and radiation techniques offer more precise and targeted therapy to lesions in an area with increased risk of clinical morbidity given the high concentration of critically important structures that must be spared during treatment. Sellar and parasellar lesions have the potential to cause significant morbidity and mortality, highlighting the importance of clinical recognition of warning signs/symptoms, obtaining high-quality imaging studies in various modalities for diagnostic purposes, and prompt management which often involves a multimodal approach that includes surgical resection, radiation, and/or medical therapy. Future advanced imaging techniques will only improve presurgical diagnostic accuracy and lead to more prompt and efficient management.
Topics: Diagnosis, Differential; Humans; Magnetic Resonance Imaging; Pain; Pituitary Gland; Pituitary Neoplasms; Sella Turcica; Skull Neoplasms; Syndrome
PubMed: 30712067
DOI: 10.1007/s11916-019-0740-x -
Current Treatment Options in Oncology Mar 2021Management of chordoma along the cranial-spinal axis is a major challenge for both skull base and spinal surgeons. Although chordoma remains a rare tumor, occurring in... (Review)
Review
Management of chordoma along the cranial-spinal axis is a major challenge for both skull base and spinal surgeons. Although chordoma remains a rare tumor, occurring in approximately 1 per 1 million individuals, its treatment poses several challenges. These tumors are generally poorly responsive to radiation and chemotherapy, leading to surgical resection as the mainstay of treatment. Due to anatomic constraints and unique challenges associated with each primary site of disease, gross total resection is often not feasible and is associated with high rates of morbidity. Additionally, chordoma is associated with high rates of recurrence due to the tumor's aggressive biologic features, and postoperative radiation is increasingly incorporated as a treatment option for these patients. Despite these challenges, modern-day surgical techniques in both skull base and spinal surgery have facilitated improved patient outcomes. For example, endoscopic endonasal techniques have become the mainstay in resection of skull base chordomas, improving the ability to achieve gross total resection, while reducing associated morbidity of open transfacial techniques. Resection of spinal chordomas has been facilitated by emerging techniques in preoperative imaging, intraoperative navigation, spinal reconstruction, and radiotherapy. Taken collectively, the treatment of chordoma affecting the skull base and spinal requires a multidisciplinary team of surgeons, radiation oncologists, and medical oncologists who specialize in the treatment of this challenging disease.
Topics: Chordoma; Humans; Natural Orifice Endoscopic Surgery; Neoplasm Recurrence, Local; Radiotherapy, Adjuvant; Plastic Surgery Procedures; Skull Base Neoplasms; Spinal Neoplasms; Surgery, Computer-Assisted; Treatment Outcome
PubMed: 33743089
DOI: 10.1007/s11864-021-00838-z -
Clinical and Translational Medicine Oct 2023Skull base chordoma is a rare and aggressive tumour of the bone that has a high likelihood of recurrence. The fundamental differences in single cells between primary and...
BACKGROUND
Skull base chordoma is a rare and aggressive tumour of the bone that has a high likelihood of recurrence. The fundamental differences in single cells between primary and recurrent lesions remain poorly understood, impeding development of effective treatment approaches.
METHODS
To obtain an understanding of the differences in single cells between primary and recurrent chordomas, we performed single-cell RNA sequencing and T-cell/B-cell receptor (BCR) sequencing. This allowed us to delineate the differences between the two types of tumour cells, tumour-infiltrating lymphocytes, myeloid cells, fibroblasts and B cells. Copy number variants (CNVs) were detected and compared between the tumour types to assess heterogeneity. Selected samples were subjected to immunohistochemistry to validate protein expression. Fluorescence in situ hybridisation experiments, Transwell assays and xenograft mouse models helped verify the role of fibronectin 1 (FN1) in chordoma.
RESULTS
Promoting natural killer (NK) cell and CD8_GZMK T-cell function or inhibiting the transformation of CD8_GZMK T cells to CD8_ZNF683 T cells and promoting the transformation of natural killer T (NKT) cells to NK cells are promising strategies for preventing chordoma recurrence. Additionally, inhibiting the M2-like activity of tumour-associated macrophages (TAMs) could be an effective approach. Antigen-presenting cancer-associated fibroblasts (apCAFs) and dendritic cells (DCs) with high enrichment of the antigen-presenting signature were enriched in primary chordomas. There were fewer plasma cells and BCR clonotypes in recurrent chordomas. Remarkably, FN1 was upregulated, had more CNVs, and was more highly secreted by tumours, macrophages, CD4 T cells, CD8 T cells and fibroblasts in recurrent chordoma than in primary chordoma. Finally, FN1 enhanced the invasion and proliferation of chordomas in vivo and in vitro.
CONCLUSION
Our comprehensive picture of the microenvironment of primary and recurrent chordomas provides deep insights into the mechanisms of chordoma recurrence. FN1 is an important target for chordoma therapy.
Topics: Humans; Animals; Mice; Chordoma; Fibronectins; Neoplasm Recurrence, Local; Treatment Outcome; Skull Base Neoplasms; Head and Neck Neoplasms; Skull Base; Tumor Microenvironment
PubMed: 37784253
DOI: 10.1002/ctm2.1429 -
Journal of Neuro-oncology Dec 2020The purpose of this review is to assess the recent evidence regarding the management of squamous cell carcinoma of the skull-base and to discuss the implications of... (Review)
Review
PURPOSE
The purpose of this review is to assess the recent evidence regarding the management of squamous cell carcinoma of the skull-base and to discuss the implications of these findings on clinical practice.
METHOD
Free text Medline and MeSH term search of publications relating to Squamous Cell Carcinoma & Skull-base and Skull base, Neoplasm respectively. Multidisciplinary clinical guidelines were also reviewed.
RESULTS
The primary search yielded a total of 271 papers which following initial review was reduced to 28. Secondary search yielded 56 papers. There were no randomised controlled trials relating to squamous cell carcinoma of the skull-base and as such this review is based on cohort studies, case series and expert opinion.
CONCLUSION
Squamous cell carcinoma (SCC) is the most common cancer occurring in the Head and Neck. Squamous cell carcinoma is also the most common cancer arising within the nose and sinuses of which skull-base squamous cell carcinoma is a rare subgroup. Evidence relating to the management and survival of skull-base SCC is based on expert opinion and. retrospective analyses Clinical examination and biopsy, imaging and a broad multidisciplinary team are key to the management of skull-base SCC. The information gathered should be used to guide informed discussion by suitably trained experts with patients regarding surgical approach, post-operative recovery and adjuvant or neoadjuvant treatments. The standard of care is currently to perform skull base resection with or without additional craniotomy, pedicled or free flap reconstruction in multiple layers and post-operative radiation (usually photons or protons). Open approaches have traditionally been the mainstay, however in certain cases endoscopic approaches can yield equivalent results and offer many advantages. Despite advances in care survival remains poor with a nearly one in five risk of nodal recurrence within two years.
Topics: Animals; Carcinoma, Squamous Cell; Combined Modality Therapy; Disease Management; Humans; Skull Base Neoplasms
PubMed: 32504403
DOI: 10.1007/s11060-020-03545-1 -
The Journal of Craniofacial Surgery Sep 2018The authors describe a case of congenital calvarial hemangioma successfully managed using propranolol therapy. Presenting symptoms, radiological and pathological...
OBJECTIVES
The authors describe a case of congenital calvarial hemangioma successfully managed using propranolol therapy. Presenting symptoms, radiological and pathological features, differential diagnosis, and management of this rare congenital mass are described.
CASE PRESENTATION
A 2-year-old boy presented with a 1-year history of a growing right parietal skull mass. No obvious etiology was apparent. No focal neurological deficits or associated craniofacial anomalies were identified. Plain film imaging demonstrated focal thickening of the right parietal bone with internal trabeculations in a sunburst appearance. Computed tomography (CT) scan showed bone thickening with coarsening of the bony trabeculae, minor irregularity of the outer table, unaffected inner table, and no evidence of aggressive features. A diagnostic biopsy of the lesion was performed in the operating room. Microscopic examination was consistent with hemangioma. Based on histological and radiological features of the lesion, it was identified as a cavernous hemangioma. Medical treatment utilizing propranolol was initiated for over 3 years with interval reduction in the lesion size. MRI head following treatment with propranolol demonstrated reduction of the mass compared to preoperative imaging.
CONCLUSIONS
Although a rare entity, it is important to consider congenital calvarial hemangioma in the differential diagnosis of slow growing skull lesions due to the possibility of complications as a result of the hemangioma's intracranial extension, and the potential for treatment. En bloc resection has classically been described as a treatment for such lesions, although our case demonstrates that medical treatment with propranolol therapy may be appropriate in certain situations.
Topics: Child, Preschool; Hemangioma, Cavernous; Humans; Male; Parietal Bone; Propranolol; Skull Neoplasms; Vasodilator Agents
PubMed: 29742579
DOI: 10.1097/SCS.0000000000004613 -
Neurocirugia (Asturias, Spain) 2017Schwannomas are nerve sheath tumours that originate in Schwann cells. They are usually solitary and sporadic and manifest on peripheral, spinal or cranial nerves.... (Review)
Review
Schwannomas are nerve sheath tumours that originate in Schwann cells. They are usually solitary and sporadic and manifest on peripheral, spinal or cranial nerves. Intracranial schwannomas tend to manifest on the eighth cranial nerve, particularly in patients with neurofibromatosis type2. Anterior skull-base schwannomas represent less than 1% of all intracranial schwannomas. They are more frequent in young people and are typically benign. These tumours represent a diagnostic challenge due to their rarity and difficult differential diagnosis, and numerous theories have been postulated concerning their origin and development. In this article, we present the case of a 13-year-old male with a single anterior cranial-base tumour not associated with neurofibromatosis who presented with headache, papilloedema, eye pain and loss of visual acuity. Complete resection of the tumour was performed, which was histopathologically diagnosed as a schwannoma. The patient made a complete clinical recovery with abatement of all symptoms. We conducted a review of the literature and found 66 cases worldwide with this diagnosis. We describe the most relevant epidemiological and clinical characteristics of this kind of tumour and its relation with the recently discovered and similar olfactory schwannoma.
Topics: Adolescent; Humans; Male; Neurilemmoma; Skull Base Neoplasms
PubMed: 28655484
DOI: 10.1016/j.neucir.2017.04.002