Sinonasal teratocarcinosarcoma: a therapeutic dilemma.

Sinonasal teratocarcinosarcomas (SNTCSs) are rare and aggressive malignant tumours with histological features of the three embryonic layers. They have an elevated local recurrence rate, risk of metastasis and mortality. Moreover, the therapeutic options are limited, and optimal management is not yet clear. As fewer than 150 cases have been reported, therapeutic strategies remain a clinical challenge. Here, we discuss a case of a large SNTCS successfully treated with surgical resection followed by concurrent chemotherapy and radiation. Despite the significant size of the tumour and the inferred high recurrence risk, the patient has had no recurrence over the past 45 months. Although the optimal treatment of SNTCS is not clearly outlined, the very limited data suggests that a multidisciplinary approach with surgery, radiation and chemotherapy is the best option for patients.

The sellar barrier on preoperative imaging predicts intraoperative cerebrospinal fluid leak: a prospective multicenter cohort study.

BACKGROUND: The type of sellar barrier observed between a pituitary tumor and cerebrospinal fluid (CSF) on preoperative magnetic resonance imaging (MRI) may predict intraoperative CSF leak during endonasal pituitary surgery. This is the first multicentric prospective cohort trial to study the sellar barrier concept and CSF leak rate during endoscopic pituitary surgery. METHODS: This multi-center, international study enrolled patients operated for pituitary adenomas via fully endoscopic endonasal surgery over a period of 4 months. The independent variable was the subtype of sellar barrier observed on preoperative MRI (strong, mixed or weak); the dependent variable was the presence of an intraoperative CSF leak. The primary goal was to determine the association between a particular type of sellar barrier and the risk of intraoperative CSF leak. Appropriate statistical methods were then applied for data analysis. RESULTS: Over the study period, 310 patients underwent endoscopic endonasal surgery for pituitary tumor. Preoperative imaging revealed a weak sellar barrier in 73 (23.55%), a mixed sellar barrier in 75 (24.19%), and a strong sellar barrier in 162 (52.26%) patients. The overall rate of intraoperative CSF leak among all patients was 69 (22.26%). A strong sellar-type barrier was associated with significantly reduced rate of intraoperative CSF leak (RR = 0.08; 95% CI 0.03-0.19; p < 0.0001), while a weak sellar barrier associated with higher rates of CSF leak (RR = 8.54; 95% CI 5.4-13.5; p < 0.0001). CONCLUSIONS: The preoperative MRI of pituitary patients can suggest intraoperative CSF leak rates, utilizing the concept of the sellar barrier. Patients with a weak sellar barrier carry a higher risk for an intraoperative CSF leak, whereas a strong sellar barrier on MRI seems to mitigate intraoperative CSF leak. We propose that preoperatively assessment of the sellar barrier can prepare surgeons for intraoperative CSF leak repair.

Granular cell tumors of the sellar region: what should be done after subtotal resection? A systematic review.

PURPOSE: Granular cell tumors (GCT) are highly vascularized and adherent to adjacent structures, and so, complete resection represents a challenge. Adjuvant therapy decisions for residual GCTs currently relies on individual clinician decisions due to a paucity of systematic literature data. We present a comprehensive analysis about the impact of adjuvant therapy in reported cases of patients with incomplete GCT resection. METHODS: One database (PubMed) and crossed references were queried for GCT with incomplete resection or biopsy from 1962 to 2020. Literature review was performed according to the PRISMA guidelines. Also, two patients with residual GCT from our institutions are added to the analysis. Data regarding clinical presentation, surgical approach, use of adjuvant therapy, Ki-67 labeling, and follow up assessments were extracted and analyzed from selected publications. RESULTS: Thirty-three studies met the predetermined inclusion criteria and 53 patients were selected (including our two reported cases). The median of age was 49 [IQR, 39-60 years], with a slight male predominance (1.2:1). Among the surgical procedures, seven (13%) were biopsies alone. Adjuvant therapy was used in 18 patients (radiotherapy, 94.5%; chemotherapy, 5.5%) but there is no statistical correlation with adjuvant therapy and the progression of the remnant tumor (p = 0.33). Our institutions' patients did not receive adjuvant therapy and did not show tumor progression on MRI. CONCLUSION: Our systematic literature review suggests there is a limited role for chemo and/or radiotherapy in the management of incomplete GCT resection. It may be reasonable recommending close clinical follow up in patients with incomplete resection.

Minimally Invasive Approaches to the Lateral Cavernous Sinus and Meckel's Cave: Comparison of Transorbital and Subtemporal Endoscopic Techniques.

BACKGROUND: Transorbital and subtemporal keyhole approaches have recently been proposed to approach lesions in the lateral wall of the cavernous sinus (CS) and Meckel's cave (MC). Our goal was to compare these approaches and suggest indications for each of them. METHODS: Five cadaver heads (10 sides, 40 procedures) were used. The lateral transorbital approaches were carried out without and with the removal of the lateral orbital rim, herein referred to as the lateral transorbital approach (LTOA) and the lateral orbital wall approach (LOWA). The subtemporal approaches were performed without and with the removal of the zygomatic arch, referred to as the subtemporal approach (STA) and the subtemporozygomatic approach (STZA). Five targets were chosen and 2 triangles were created representing the lateral wall of the CS and MC. Stereotactic measurements were quantified to calculate angles of attack, surgical freedom, and temporal lobe retraction for each approach. RESULTS: LTOA presented the smaller horizontal angles of attack. LOWA increased the angles to the same level of STA and STZA. STA and STZA presented larger vertical angles of attack. The surgical freedom presented gradual increase from LTOA to LOWA, STA, and STZA. STA and STZA needed greater temporal lobe retraction for most targets. CONCLUSIONS: LTOA is a good option to biopsy a lesion in the lateral wall of the CS and LOWA increased the surgical corridor to work with microsurgical techniques. STA and STZA could be better options when wide exposure is necessary, but temporal lobe retraction should be taken into consideration.

ICAR: endoscopic skull-base surgery.

BACKGROUND: Endoscopic skull-base surgery (ESBS) is employed in the management of diverse skull-base pathologies. Paralleling the increased utilization of ESBS, the literature in this field has expanded rapidly. However, the rarity of these diseases, the inherent challenges of surgical studies, and the continued learning curve in ESBS have resulted in significant variability in the quality of the literature. To consolidate and critically appraise the available literature, experts in skull-base surgery have produced the International Consensus Statement on Endoscopic Skull-Base Surgery (ICAR:ESBS). METHODS: Using previously described methodology, topics spanning the breadth of ESBS were identified and assigned a literature review, evidence-based review or evidence-based review with recommendations format. Subsequently, each topic was written and then reviewed by skull-base surgeons in both neurosurgery and otolaryngology. Following this iterative review process, the ICAR:ESBS document was synthesized and reviewed by all authors for consensus. RESULTS: The ICAR:ESBS document addresses the role of ESBS in primary cerebrospinal fluid (CSF) rhinorrhea, intradural tumors, benign skull-base and orbital pathology, sinonasal malignancies, and clival lesions. Additionally, specific challenges in ESBS including endoscopic reconstruction and complication management were evaluated. CONCLUSION: A critical review of the literature in ESBS demonstrates at least the equivalency of ESBS with alternative approaches in pathologies such as CSF rhinorrhea and pituitary adenoma as well as improved reconstructive techniques in reducing CSF leaks. Evidence-based recommendations are limited in other pathologies and these significant knowledge gaps call upon the skull-base community to embrace these opportunities and collaboratively address these shortcomings.

Pituitary Adenoma Concomitant with Chiari I Malformation: Case Report and Literature Review.

BACKGROUND: Pituitary adenomas (PA) are usually benign neoplasms. Chiari I malformation (CIM) is an uncommon finding. Isolated cases associating functional PA and CIM have been reported. The concomitant presence of a nonfunctional PA and a CIM has not yet been described in the literature. CASE DESCRIPTION: We present the case of a 35-year-old patient whose symptoms were compatible with CIM. Magnetic resonance imaging of the brain, with and without contrast material, confirmed the CIM and incidentally encountered a PA. The patient was treated initially for her PA through an endoscopic endonasal approach with complete tumor resection, confirming a nonfunctional PA. Posterior fossa decompression was successfully accomplished 3 months later. The patient has been followed up for 5 years and has been free of symptoms, with no recurrence of her PA. This represents the first reported case of a nonfunctional PA with a concomitant CIM. CONCLUSION: This is the first reported case of a concomitant CIM and a nonfunctional PA. We discuss our successful management and conduct a systematic review of the literature to provide the most up-to-date guidance on managing these singular cases with concomitant pathologic conditions.

Potential Effect of Leukocyte-Platelet-Rich Fibrin in Bone Healing of Skull Base: A Pilot Study.

BACKGROUND: Reconstruction of surgical defects following cranial base surgery is challenging. Others have demonstrated that leukocyte-platelet-rich fibrin (L-PRF) stimulates tissue healing and bone regeneration. However, these studies have addressed mostly maxillofacial surgical wounds. OBJECTIVE: The objective of this study was to assess the possible adjuvant role of L-PRF in inducing neoossification of the surgical bone defect in anterior skull base surgery. METHODS: We identified patients who had undergone an endoscopic endonasal surgery of the anterior skull base in which L-PRF membranes were used for the reconstruction of the bone defect and who were followed up with postoperative CT scans. CT findings were then correlated with baseline scans and with the CT scans of a patient who had undergone imaging and histologic analysis after maxillofacial surgery in which L-PRF was used and in which we demonstrated bone formation. RESULTS: Five patients fulfilled the inclusion criteria. In four patients, the CT scan demonstrated closure of the bony defect by neoosteogenesis; however, the bone appeared less dense than the surrounding normal bone. A comparison with the control patient yielded similar radiological features. CONCLUSION: This case series suggests that L-PRF may induce bone healing and regeneration at the surgical site defect. Multi-institutional studies with a larger series of patients are required to confirm this possibility.

Morphometric analysis of the medial opticocarotid recess and its anatomical relations relevant to the transsphenoidal endoscopic endonasal approaches.

BACKGROUND: The medial opticocarotid recess (MOCR) is located in the posterior wall of the sphenoid sinus, medial to the junction of the optic canal (OC) and the carotid prominence (CP). There is controversy in the literature in relation to the presence of the MOCR and its constancy, which is relevant when approaching the skull base through an endoscopic route. METHODS: The morphometric relations of the MOCR with the surrounding structures were studied in 18 cadaveric specimens after endoscopic endonasal approach (EEA). RESULTS: The distance between both MOCR was 11.06 ± 1.14 mm; the distance between the MOCR and the lateral opticocarotid (LOCR) recess was 5.56 ± 0.85 mm; the distance between the MOCR and the suprasellar recess was 3.72 ± 0.49 mm; the angle between the MOCR plane and the OC 13.32 ± 2.30°; the angle between the MOCR plane and the CP 13.50 ± 2.68° and; the angle between the OC and the CP 26.81 ± 4.26°. All measurements showed low variability, with low standard deviation and interquartile range. No relations were found between any of the measurements. CONCLUSIONS: The MOCR may be used as a reference point for precise location of structures during EEA. Objective measurements may be especially useful in cases with distorted sphenoid bone anatomy.

To preserve or not to preserve the orbit in paranasal sinus neoplasms: a meta-analysis.

Context The effect on survival of orbital evisceration on patients with paranasal sinus neoplasms has not been well established. Objective To review systematically the available literature concerning survival in patients who undergo surgery for paranasal sinus neoplasm with and without preservation of the eye. Data Source A retrospective meta-analysis of English and non-English articles using Medline and the Cochrane database. Eligibility Criteria Studies analyzing 5-year survival rates in patients who had orbital evisceration compared with orbital preservation for the treatment of paranasal sinus neoplasms were included in the final analysis. Data Extraction Independent review by two authors using predefined data fields. Data Synthesis A meta-analysis of four articles involving 443 patients was performed using the DerSimonian-Laird random-effects method. Results Our analysis revealed a total effect size of 0.964 in favor of preservation of the eye; however, these results are not robust, having a true effect size anywhere from 0.785 to 1.142 with a 95% confidence interval. Limitations Only retrospective observational studies were included because a prospective randomized study cannot be performed in this population. Conclusion Our study supports the notion that in select patients preservation of the eye may yield a different outcome when compared with orbital evisceration.

Nuances in the treatment of malignant tumors of the clival and petroclival region.

Introduction Malignancies of the clivus and petroclival region are mainly chordomas and chondrosarcomas. Although a spectrum of malignancies may present in this area, a finite group of commonly encountered malignant pathologies will be the focus of this review, as they are recognized to be formidable pathologies due to adjacent critical neurovascular structures and challenging surgical approaches. Objectives The objective is to review the literature regarding medical and surgical management of malignant tumors of the clival and petroclival region with a focus on clinical presentation, diagnostic identification, and associated adjuvant therapies. We will also discuss our current treatment paradigm using endoscopic, open, and combined approaches to the skull base. Data Synthesis A literature review was conducted, searching for basic science and clinical evidence from PubMed, Medline, and the Cochrane Database. The selection criteria encompassed original articles including data from both basic science and clinical literature, case series, case reports, and review articles on the etiology, diagnosis, treatment, and management of skull base malignancies in the clival and petroclival region. Conclusions The management of petroclival malignancies requires a multidisciplinary team to deliver the most complete surgical resection, with minimal morbidity, followed by appropriate adjuvant therapy. We advocate the combination of endoscopic and open approaches (traditional or minimally invasive) as required by the particular tumor followed by radiation therapy to optimize oncologic outcomes.

Nuances in the Treatment of Malignant Tumors of the Clival and Petroclival Region

Introduction: Malignancies of the clivus and petroclival region are mainly chordomas and chondrosarcomas. Although a spectrum of malignancies may present in this area, a finite group of commonly encountered malignant pathologies will be the focus of this review, as they are recognized to be formidable pathologies due to adjacent critical neurovascular structures and challenging surgical approaches. Objectives: The objective is to review the literature regarding medical and surgical management of malignant tumors of the clival and petroclival region with a focus on clinical presentation, diagnostic identification, and associated adjuvant therapies. We will also discuss our current treatment paradigm using endoscopic, open, and combined approaches to the skull base. Data Synthesis A literature review was conducted, searching for basic science and clinical evidence from PubMed, Medline, and the Cochrane Database. The selection criteria encompassed original articles including data from both basic science and clinical literature, case series, case reports, and review articles on the etiology, diagnosis, treatment, and management of skull base malignancies in the clival and petroclival region. Conclusions: The management of petroclival malignancies requires a multidisciplinary team to deliver the most complete surgical resection, with minimal morbidity, followed by appropriate adjuvant therapy. We advocate the combination of endoscopic and open approaches (traditional or minimally invasive) as required by the particular tumor followed by radiation therapy to optimize oncologic outcomes...

Tecnica endoscopica endonasal: tratamento de lesoes malignas paranasais e da base do cranio anterior / Endoscopic endonasal technique: treatment of paranasal and anterior skull base malignancies

Inovações técnicas e tecnológicas têm liderado a expansão das indicações de uso das abordagens endoscópicas endonasais para a extirpação de lesões malignas do trato nasossinusal e base do crânio. OBJETIVO: Analisar criticamente a literatura disponível sobre o uso de abordagens endoscópicas endonasais incluindo indicações, limitações, técnicas cirúrgicas, desfecho oncológico e qualidade de vida. MÉTODO: Várias técnicas endoscópicas endonasais foram analisadas segundo a origem e extensão local das lesões malignas nasossinusais e da base do crânio, incluindo a porção anterior da base do crânio, nasofaringe, clívus e fossa infratemporal. A literatura disponível foi também avaliada em função dos desfechos. CONCLUSÃO: As abordagens endoscópicas endonasais são parte integrante do arsenal terapêutico desenvolvido para abordar neoplasias malignas do trato nasossinusais e da base do crânio. Em casos adequadamente selecionados, esta abordagem produz resultados oncológicos semelhantes com menor morbidade do que as abordagens abertas tradicionais. Não obstante, abordagens minimamente invasivas devem ser consideradas como complemento às abordagens abertas estabelecidas, ainda necessárias na maioria dos tumores mais avançados. .

Technical and technological innovations have spearheaded the expansion of the indications for the use of endoscopic endonasal approaches to extirpate malignancies of the sinonasal tract and adjacent skull base. OBJECTIVE: Critical review of the available literature regarding the use of endoscopic endonasal approaches including indications, limitations, surgical techniques, oncologic outcome, and quality of life. METHOD: Various endoscopic endonasal techniques are reviewed according to the origin and local extension of sinonasal and skull base malignancies including anterior cranial base, nasopharynx, clivus, and infratemporal fossa. In addition, the available literature is reviewed to assess outcomes. CONCLUSION: Endoscopic endonasal approaches are an integral part of the armamentarium for the treatment of the sinonasal tract malignancies and skull base. In properly selected cases, it affords similar oncologic outcomes with lower morbidity than traditional open approaches. Nonetheless, these minimal access approaches should be considered a complement to well-established open approaches, which are still necessary in most advanced tumors. .

Endoscopic endonasal technique: treatment of paranasal and anterior skull base malignancies.

UNLABELLED: Technical and technological innovations have spearheaded the expansion of the indications for the use of endoscopic endonasal approaches to extirpate malignancies of the sinonasal tract and adjacent skull base. OBJECTIVE: Critical review of the available literature regarding the use of endoscopic endonasal approaches including indications, limitations, surgical techniques, oncologic outcome, and quality of life. METHOD: Various endoscopic endonasal techniques are reviewed according to the origin and local extension of sinonasal and skull base malignancies including anterior cranial base, nasopharynx, clivus, and infratemporal fossa. In addition, the available literature is reviewed to assess outcomes. CONCLUSION: Endoscopic endonasal approaches are an integral part of the armamentarium for the treatment of the sinonasal tract malignancies and skull base. In properly selected cases, it affords similar oncologic outcomes with lower morbidity than traditional open approaches. Nonetheless, these minimal access approaches should be considered a complement to well-established open approaches, which are still necessary in most advanced tumors.

Presentación clínica y tomográfica de pacientes con displasia fibrosa craneofacial en la Clínica José A. Rivas / Clinical and radiological presentation in patients with fi brous dysplasia craniofacial in José A. Rivas clinic

Objetivo: describir las manifestaciones clínicas y radiológicas de los pacientes con diagnóstico de displasia fi brosa craneofacial en el Servicio de Rinología y Base de Cráneo de la Clínica José A. Rivas, entre enero del 2009 y enero del 2012. Diseño: estudio tipo serie de casos con análisis prospectivo Métodos: se incluyeron diez pacientes entre los ocho y 36 años con diagnóstico de displasia fi brosa craneofacial, en los que se tuvieron en cuenta variables demográfi cas, manifestaciones clínicas, presentación tomográfi ca, y se revisó el tipo de tratamiento realizado, al igual que la recidiva de la enfermedad. Resultados: se encontró que la manifestación preponderante ocurría en el sexo masculino (60%), con una media de 17 años, y la condición clínica común fue la asimetría facial (ocho pacientes). Además, el seno etmoidal fue el que evidenció mayor compromiso (60% de los casos); dos pacientes registraron lesión de la base del cráneo, uno de ellos reportó ceguera y compromiso del nervio óptico. En la tomografía, el tipo 2, con compromiso de más de dos senos paranasales, fue el que se halló con más frecuencia, y la variedad mixta, con respecto al tipo de lesión, tuvo mayor porcentaje que la de vidrio esmerilado, la homogénea y la quística. Al 90% de los pacientes se les sometió a tratamiento quirúrgico ciento por ciento endoscópico, y se detectó un 20% de recidivas.

Objective: To describe the clinical and radiological diagnosis of patients with craniofacial fibrous dysplasia in the Service of Rhinology and Skull Base Clinic José A. Rivas, between January 2009 and January 2012. Design: case series with prospective analysis. Methods: Ten patients between eight and 36 years diagnosed with craniofacial fibrous dysplasia, which took into account demographic, clinical, tomographic presentation, and revised the type of treatment, as well as recurrence of disease. Results: We found that the predominant manifestation occurred in males (60%), with a mean of 17 years, and the common clinical condition was facial asymmetry (eight patients). In addition, the ethmoid sinus was evident that greater commitment (60% of cases), two patients reported injury skull base, one of them reported blindness and optic nerve involvement. On CT, type 2, with involvement of more than two sinuses, was the one most frequently found, and the mixed variety, with respect to the type of injury, had the highest percentage of frosted glass, the homogeneous the cystic. 90% of patients underwent surgical treatment hundred percent endoscopic and detected 20% of recurrences. Conclusions: There are few studies in Colombia about craniofacial fibrous dysplasia. For this reason, we present statistics about the pathology own study, proposing a new tomographic classification as a basis for future research.

Endonasal endoscopic exposure of the internal carotid artery: an anatomical study.

OBJECTIVES/HYPOTHESIS: The aim of this work was to define the anatomical landmarks, limitations, and difficulties of obtaining internal carotid artery (ICA) exposure via endonasal endoscopic approaches (EEA). STUDY DESIGN: Cadaveric descriptive study. METHODS: The ICA was dissected via EEA in 10 cadaveric specimens (20 sides) prepared with intravascular injections of colored silicone. We carried the ICA dissection from the cavernous to the distal parapharyngeal segments through a transpterygoid corridor. RESULTS: The transpterygoid approach provided adequate exposure of the lacerum and horizontal petrous ICA. Additional exposure of the ICA and the infrapetrous area required resection of the eustachian tube (ET) and the fibrocartilaginous tissue of the foramen lacerum after a medial maxillectomy and resection of the pterygoid plates. The main anatomical landmarks to the corresponding ICA segment include: the vidian nerve that points to the lacerum and horizontal segments, the mandibular nerve (V3) that heralds the petrous segment, the foramen ovale and the ET that signal toward the carotid canal, and the posterior trunk of the mandibular nerve (V3) and the ET that mark the parapharyngeal segment. CONCLUSIONS: EEAs provide access to the ICA from its cavernous to the distal parapharyngeal segments. A stepwise approach is critical to its exposure and control. Surgeons must be aware of its frequently tortuous three-dimensional course and the intimate relation of the vessel to the carotid canal and the cartilage of the foramen lacerum.

Extracellular matrix composition of the cricopharyngeus muscle.

The aim of this study was to analyze the presence and distribution of total collagen, type I and type III collagen, elastic fibers, fibronectin, and versican in the endomysium of cricopharyngeus muscles from adults of various ages. The study was a cross-sectional analysis of human cricopharyngeus muscles. Twenty-seven muscles obtained from autopsies of men and women ranging in age from 28 to 92 years were analyzed with the Picrosirius method, oxidized Weigert resorcin-fuchsin, immunohistochemistry, and image analysis. Collagen had the highest density among the analyzed components. Elastic fibers surrounded each muscle cell; they were aligned longitudinally by their long axis and associated with traversing fibers, thereby forming a fiber network with embedded muscle cells. The fibronectin and versican contents varied widely among the specimens. We found no statistically significant differences between the proportion of extracellular matrix (ECM) components and factors such as gender and race. We conclude that the higher proportion of type I and type III collagen is compatible with the cricopharyngeus muscle's sphincteric behavior, and the arrangement of the elastic fibers may also contribute to the muscle's elasticity. We found no statistically significant correlation between the ECM components and age.