Transcavernous sinus pituitary gland transposition: how I do it.

BACKGROUND: Pituitary transposition preserving gland function is possible when approaching superior clival region tumors. Clinical experience along with detailed anatomical knowledge makes this technique safe and effective. METHOD: We present a step by step description of our technique based on the most recent anatomical references to get a pituitary transposition through the different compartments of the cavernous sinus. By this technique, we achieve minor gland manipulation and a better surgical view of this area. We support this technique with an anatomical analysis on cadaveric specimens and clarifying dissection images. CONCLUSIONS: Transcavernous sinus pituitary gland transposition is an easily feasible technique and allows gland shifting preserving pituitary function.

How I do it: endoscopic endonasal approach to the inferior third of the clivus.

BACKGROUND: Nowadays, endoscopic endonasal expanded approach targeting for the clival lower third is well described in literature. Nonetheless, great variations can be found among surgical groups, specially during the earlier stages of this procedure. METHOD: We present a step by step description of the clival lower third approach until entering the dural space, setting its bony limits. We describe the basipharyngeal flap tailoring as a helpful option for latter reconstruction. The study of cadaveric specimens adds clarifying dissections. CONCLUSIONS: The expansion in the coronal plane is providential in most of the intradural lesions of the inferior clivus. Basipharyngeal flap may help seal the surgical defects in this area.

How I do it? Cranial setup for cranial settling.

BACKGROUND: Expanded endonasal endoscopic techniques allow us to treat several pathologies related to the odontoid process and craniocervical junction. Cases such as giant basilar invagination represent a surgical challenge. METHODS: The authors provide technical nuances and describe how to complete an endoscopic endonasal odontoidectomy and release the craniocervical junction with the aim of restoring a correct sagittal balance in cases with giant basilar invagination. The study of cadaveric specimens adds clarifying dissections. CONCLUSIONS: Endonasal endoscopic odontoidectomy and craniocervical junction joint release allow the treatment of irreducible basilar invagination and restoration of better sagittal balance before posterior cervical occipitocervical fusion.

Endoscopic radical hypophysectomy: how I do it.

BACKGROUND: Total hypophysectomy it is a classical procedure that currently has many indications especially in patients with Cushing syndrome without good endocrine control. Expanded endonasal endoscopic techniques grant us an alternative standpoint to the classic trans-sphenoidal microscopic approach and a comprehensive assessment of the process METHOD: The author provides technical nuances and describe step by step the radical endoscopic hypophysectomy. The study of cadaveric specimens adds clarifying dissections. CONCLUSIONS: Radical hypophysectomy is an easily replicable and safe procedure. The most important morbidity is the intraoperative cerebrospinal fluid (CSF) leakage, which is inherent to this technique and can be successfully prevented with a pedicled nasoseptal flap reconstruction.

Clinical Applicability of the Sellar Barrier Concept in Patients with Pituitary Apoplexy: Is It Possible?

There is evidence of association between sellar barrier thickness and intraoperative cerebrospinal fluid (CSF) leakage, impacting the postoperative prognosis of the patients. The aim of this study is to analyze the clinical applicability of the sellar barrier concept in a series of operated patients with pituitary apoplexy (PA). A retrospective study was conducted including 47 patients diagnosed with PA who underwent surgical treatment through a transsphenoidal approach. Brain magnetic resonance imaging (MRI) of the patients were evaluated and classified utilizing the following criteria: strong barrier (greater than 1 mm), weak barrier (less than 1 mm), and mixed barrier (less than 1 mm in one area and greater than 1 mm in another). The association between sellar barrier types and CSF leakage was analyzed, both pre- and intraoperatively. The preoperative MRI classification identified 10 (21.28%) patients presenting a weak sellar barrier, 20 patients (42.55%) with a mixed sellar barrier, and 17 patients (36.17%) exhibiting a strong sellar barrier. Preoperative weak and strong sellar barrier subtypes were associated with weak (p ≤ 0.001) and strong (p = 0.009) intraoperative sellar barriers, respectively. Strong intraoperative sellar barrier subtypes reduced the odds of CSF leakage by 86% (p = 0.01). A correlation between preoperative imaging and intraoperative findings in the setting of pituitary apoplexy has been observed.

[Esthesioneuroblastoma. Transcribiform-transfovea ethmoidalis endonasal expanded approach. Technical note]. / Estesioneuroblastoma. Abordaje endonasal expandido transcribiforme-transfovea etmoidalis. Nota técnica.

INTRODUCTION: We report a fully endoscopic transcribiform-transfovea ethmoidalis endonasal expanded approach (EEA) for the treatment of esthesioneuroblastoma and review the literature about this entity available in English, establishing a precise surgical technique and describing our intraoperative experience. CASE REPORT: Our patient was a 65-year old female with anosmia and cognitive deterioration. Cranial MRI showed a large tumoral lesion with solid and cystic components involving the nasal cavity, with diagnostic suspicion of intracranial malignant sinonasal tumour. The patient underwent a fully endoscopic transcribiform-transfovea ethmoidalis EEA, achieving total resection and tumour-free margins. Surgery was followed by radiotherapy. DISCUSSION: Craniofacial resection enables total removal of sinonasal malignancies, even when the intracranial cavity is involved, and allows for subarachnoid space isolation from the nasal cavity. New advances in endoscopic skull base surgery have achieved comparable oncological results and sufficient reconstructive capacity, leading to less morbidity and better tolerance. CONCLUSION: EEA may become the first treatment option for skull base malignancies in an immediate future, provided that the anatomical limits of the extended approach are not exceeded by the lesion.

[Transpterygoid expanded endonasal approach in fibrous dysplasia of the skull base: case report and technical note]. / Abordaje endonasal expandido transpterigoideo en la displasia fibrosa de base de cráneo: caso clínico y nota técnica.

INTRODUCTION: Expanded endonasal approaches (EEA) are becoming a first-level technique for the treatment of skull base pathologies. In some cases, the endoscopic procedures make it possible to dissect structures manipulated with greater difficulty in the classic approaches. We report a full endoscopic transpterygoid EEA for the treatment of a fibrous dysplasia (FD) of the skull base. In addition, we reviewed the English literature available on FD and transpterygoid EEA, establishing an exact surgical technique and showing our intraoperative experience. CASE REPORT: A 42-year-old male with right sixth cranial nerve palsy. Cranial MRI and CT showed a central skull base lesion with diagnostic suspicion of FD. Patient underwent a full endoscopic transpterygoid EEA, achieving a wide skull base neurovascular decompression. Neuronavigation and the vidian canal landmark resulted mandatory during intraoperative procedure. DISCUSSION: The transpterygoid EEA is a safe technique consistently supported in the literature. It may reduce the morbidity associated to the classic transcranial approaches, since it permits maximum resection with minimum craniofacial distortion. The vidian hole and canal are the landmarks used to locate and avoid injury to the lacerum segment of the carotid injury. The surgical treatment indication in FD cases must be established in symptomatic patients. CONCLUSION: Transpterygoid EEA for treatment of FD of the skull base is a safe and effective procedure, thanks to the guide that the vidian canal provides in finding the lacerum segment of the carotid artery.

The Quadrangular Space, Endonasal Access to the Meckel Cave: Technical Considerations and Clinical Series.

OBJECTIVE: An anteromedial corridor via an expanded endoscopic endonasal approach to the Meckel cave (MC) was described more than a decade ago. However, few clinical series or endoscopic endonasal technical contributions exist concerning this type of approach to this complex region. METHODS: We present a detailed description of the surgical technique for this approach reviewing the original technique and adding clarifying conceptual notions. We conducted a multicenter retrospective study selecting patients who underwent endonasal endoscopic surgery for lesions exclusively limited to the MC in the past 6 years. Intraoperative and postoperative complications were analyzed. The study of 10 cadaveric specimens provides additional information. RESULTS: We performed a fully endoscopic anteromedial corridor to the MC in 18 patients. The most prevalent pathologic finding was schwannoma of the V nerve in 4 patients. Sixth cranial nerve palsy (13 patients) and trigeminal dysfunction (10 patients) were the predominant preoperative clinical signs. There were no remarkable intraoperative complications. Corneal keratopathy caused by dry eye syndrome affected 3 patients and V2 residual neuralgia appeared postoperatively in 2 patients. Six patients recovered from sixth cranial nerve palsy, and 2 showed improvement in preoperatively referred facial pain. CONCLUSIONS: The front door to the MC via the endonasal anteromedial corridor could be a good option. Understanding of the anatomy and the concept of the quadrangular space is crucial to performing this technique safely, which has few complications in experienced hands. Recovery from sixth nerve palsy is possible with this approach. Corneal keratopathy in these patients is a potential complication.

Sex Hormone Receptor Expression in Craniopharyngiomas and Association with Tumor Aggressiveness Characteristics.

Craniopharyngiomas (CPs) are rare tumors of the sellar and suprasellar regions of embryonic origin. The primary treatment for CPs is surgery but it is often unsuccessful. Although CPs are considered benign tumors, they display a relatively high recurrence rate that might compromise quality of life. Previous studies have reported that CPs express sex hormone receptors, including estrogen and progesterone receptors. Here, we systematically analyzed estrogen receptor α (ERα) and progesterone receptor (PR) expression by immunohistochemistry in a well-characterized series of patients with CP (n = 41) and analyzed their potential association with tumor aggressiveness features. A substantial proportion of CPs displayed a marked expression of PR. However, most CPs expressed low levels of ERα. No major association between PR and ERα expression and clinical aggressiveness features was observed in CPs. Additionally, in our series, ß-catenin accumulation was not related to tumor recurrence.

Usefulness of endonasal flaps and grafts in skull base surgery. Consensus document.

INTRODUCTION: Skull base reconstruction is one of the greatest challenges extended endonasal endoscopic surgery. Many grafts and flaps from the endonasal fossa have been demonstrated to be useful in the control of complications such a cerebrospinal fluid leaks. Review and analysis of these resources are necessary in skull base recontruction to improve outcomes. OBJECTIVES: The target is to create a consensus document on the use of different endonasal flaps and grafts in the skull base surgery. MATERIAL AND METHODS: Literature review of the most relevant free grafts and vascularized flaps from the endonasal fossa. Analysis using the Delphi method on the use of the different endonasal resources for endoscopic repair of skull base defects. RESULTS: We obtained two results: 1) A selection of the most representative flaps and grafts from the endonasal fossa, describing origin, surface and indications, based on a literature review. 2) A consensus document, using Delphi methodology, with general considerations (2), recommendations (10) and limitations (6) of the different endonasal flaps and grafts. CONCLUSIONS: We present the first consensus document in the field of extended endonasal endoscopic surgery using the Delphi method as a working tool. We highlight the usefulness of the nasoseptal flap together with other endonasal flaps and grafts for skull base reconstruction.

Middle turbinate vascularized flap for skull base reconstruction after an expanded endonasal approach.

INTRODUCTION: The expanded endonasal approaches to the skull base are modular approaches that arise from the sphenoidal sinus. The reconstructive techniques in these approaches are key to avoid postoperative complications. Available flaps for reconstruction include the pedicled nasoseptal flap, the transpterygoid temporoparietal fascia flap, and the posterior pedicle inferior turbinate flap (PPITF), among others. Recently, the middle turbinate flap has been described in a cadaveric study. We report our preliminary experience in the use of this middle turbinate vascularized flap for skull base reconstruction after expanded endonasal approaches. MATERIAL AND METHODS: Ten patients underwent reconstructive procedures with the mucoperiostial vascularized middle turbinate flap. Capability to cover the defect, closure success, operative time and complications related to the procedure are retrospectively analyzed. RESULTS: A satisfactory closure was obtained in all procedures, and there were no complications related to the technique. Required operative time was similar to the time employed for the nasoseptal flap. CONCLUSIONS: The vascularized middle turbinate flap is a reliable reconstructive technique for the reconstruction of moderate-sized skull base defects. It can be considered either as the first choice of closure or as an alternative to the nasoseptal flap when this is not available. Different flap combinations may facilitate skull base defect reconstruction.

Our experience in prolactinomas larger than 60mm.

INTRODUCTION: Giant prolactinomas (tumor size larger than 40mm) are a rare entity of benign nature. Prolactinomas larger than 60mm are usually underrepresented in published studies and their clinical presentation, outcomes and management might be different from smaller giant prolactinomas. PATIENTS AND METHODS: We retrospective collected data from patients with prolactinomas larger than 60mm in maximum diameter and prolactin (PRL) serum levels higher than 21,200µIU/mL in our series of prolactinomas (283). Data were collected from January 2012 to December 2017. We included three patients with prolactinomas larger than 60mm. RESULTS: At diagnosis, two patients presented neurological symptoms and one nasal protrusion. All patients received medical treatment with dopamine agonists. No surgical procedure was performed. Median prolactin levels at diagnosis was 108,180 [52,594-514,984]µIU/mL. Medical treatment achieved a marked reduction (>99%) in prolactin levels in all cases. Tumor size reduction (higher than 33%) was observed in all cases. In one patient cerebrospinal fluid (CSF) leak was observed after tumor shrinkage. CONCLUSIONS: Dopamine agonists appear to be an effective and safe first-line treatment in prolactinomas larger than 60mm even in life-threatening situations. More studies with a higher number of patients are necessary to obtain enough data to make major recommendations.

Basilar Artery Injury During Endonasal Surgery: Stepwise to Control Bleeding.

BACKGROUND: Intraoperative injury during endoscopic endonasal surgery of the carotid artery has been previously described in the literature. However, the accidental damage of the basilar artery in such scenario is not defined. OBJECTIVE: To define the protocol of action for massive bleeding from an artery in the posterior fossa. METHODS: The reported patient was diagnosed with a partially calcified clival chordoma featured by a huge intradural component. An endoscopic endonasal transpterygoid transclival approach was selected for the treatment of this tumor. During the surgical procedure, the basilar artery injury was injured, causing intense bleeding. We present and discuss the surgical maneuvers that could save a patient's life after this dramatic complication. RESULTS: Different techniques were performed in order to control the massive bleeding, including injection of hemotastic matrix with thrombin (Floseal©), bipolar coagulation, and vessel reconstruction by means of a vascular clip. Finally, an autologous muscle graft reinforced with an overlying fibrin sealant patch (Tachosil©) was chosen and was an effective technique. Afterwards, the patient was treated with a flow diverter device to occlude an iatrogenic pseudoaneurysm. A monoplegia of the right upper limb was the only remarkable sequel 6 mo after surgery. CONCLUSION: The muscle graft together with the coordinated action with interventional neuroradiology for the reconstruction of the vessel are possibly the best options to try to preserve the neurological function. In such a scenario, the assumption of potential ischemic events prevails over the intraoperative death of the patient.

Usefulness of endonasal flaps and grafts in skull base surgery. Consensus document. / Utilidad de los colgajos e injertos endonasales en cirugía endoscópica nasosinusal y de base de cráneo. Documento de consenso.

INTRODUCTION: Skull base reconstruction is one of the greatest challenges extended endonasal endoscopic surgery. Many grafts and flaps from the endonasal fossa have been demonstrated to be useful in the control of complications such a cerebrospinal fluid leaks. Review and analysis of these resources are necessary in skull base recontruction to improve outcomes. OBJECTIVES: The target is to create a consensus document on the use of different endonasal flaps and grafts in the skull base surgery. MATERIAL AND METHODS: Literature review of the most relevant free grafts and vascularized flaps from the endonasal fossa. Analysis using the Delphi method on the use of the different endonasal resources for endoscopic repair of skull base defects. RESULTS: We obtained two results: 1) A selection of the most representative flaps and grafts from the endonasal fossa, describing origin, surface and indications, based on a literature review. 2) A consensus document, using Delphi methodology, with general considerations (2), recommendations (10) and limitations (6) of the different endonasal flaps and grafts. CONCLUSIONS: We present the first consensus document in the field of extended endonasal endoscopic surgery using the Delphi method as a working tool. We highlight the usefulness of the nasoseptal flap together with other endonasal flaps and grafts for skull base reconstruction.

Fully Endoscopic Transforaminal-Transchoroidal Approach for Tectal Area Tumor Removal.

BACKGROUND: The surgical approaches to lesions located in the tectal area have remained controversial. The essential functions in the surrounding areas and the difficulties in obtaining a good surgical view during tumor removal have made these procedures risky and challenging. Endoscopic transforaminal approaches have been previously described for biopsy and intraventricular tumor removal. However, the endoscopic transforaminal-transchoroidal gross resection technique for such cases has barely been described. METHODS: The endoscopic entry points and trajectories were planned using preoperative magnetic resonance imaging. Once the endoscope was inside the ventricular system, the angles of work and tumor exposure of the upper posterior part of the third ventricle were carefully evaluated. If the angle of work was insufficient for tumor removal, the choroidal fissure was opened using endoscopic bipolar electrode and dissectors. Tumor removal was performed using an endoscopic ultrasonic aspirator. We have presented a 3-case series of patients affected by tectal tumors that were treated using a fully endoscopic transforaminal-transchoroidal approach. RESULTS: Total gross resection of the tumors was achieved in 2 patients. Subtotal resection was achieved in the third patient. No major complications had developed in relationship to the procedure. No new cognitive impairment was reported secondary to this technique. CONCLUSIONS: In our experience, a fully endoscopic transforaminal-transchoroidal approach was a suitable treatment for this complex pathological entity. Opening of the choroidal fissure added an extra angle of work and improved the exposure of the upper posterior part of the third ventricle.

Endoscopic endonasal superomedial orbitectomy: How far is safe and possible?

OBJECTIVES: During the endoscopic endonasal approach (EEA) to the anterior cranial base, the lateral boundaries are the lamina papyracea (medial orbital walls) bilaterally but further extension in the coronal plane is possible by performing a superomedial orbitectomy. The aims of this study are to describe the technique of the endoscopic endonasal transethmoidal supraorbital approach to the anterior cranial base and to calculate the extension in the coronal plane added with the superomedial orbitectomy. METHODS: Thirty superomedial orbitectomies via EEA were completed in 15 fresh-frozen heads. After finishing the procedure, a bifrontal craniotomy with removal of both frontal lobes was performed in order to measure the width of the supraorbital EEA in the coronal plane. We divided the anterior cranial base into five zones related to distinct anatomical segments: sinusal zone, post-sinusal zone, anterior ethmoidal, inter-ethmoidal zone, and posterior ethmoidal zone. Measurements of each segment of the anterior cranial base were taken. RESULTS: In all specimens, it was possible to perform a superomedial orbitectomy without excessive retraction of the orbital contents. The inter-ethmoidal zone is the segment where the lateral extension was widest. The mean total width in this area was 45.4 mm. The superomedial orbitectomy added a mean of 8 mm on each side to the total anterior skull base exposure. CONCLUSION: The endoscopic endonasal superomedial orbitectomy added important extension in the coronal plane during an EEA to the anterior cranial base. The inter-ethmoidal zone has shown the greatest lateral extension. LEVEL OF EVIDENCE: N/A Laryngoscope, 130:1151-1157, 2020.

Dynamic Chess-Table Cranial Expansion for Treatment of Craniocerebral Disproportion: Technical Note and Volumetric Results.

OBJECTIVE: Craniocerebral disproportion (CCD) is a challenging disease, and several expansile procedures have been used for its treatment. This report describes the dynamic chess-table cranial expansion technique and reports on 9 patients with primary and secondary CCD treated with this procedure. METHODS: Nine patients affected by CCD were treated with chess-table cranial expansion and reviewed. Symptoms of increased intracranial pressure (ICP) and radiologic findings were analyzed. ICP was monitored using epidural or telemetric sensors. Intracranial volume was measured using computed tomography image processing tools before the surgery, 24 hours after the surgery, and 1 month later. A mathematical model was developed to explain the clinical and surgical results. RESULTS: Five patients had secondary CCD and 4 had primary CCD. The mean age for cranial expansion was 16.78 years. The most frequent symptoms were headaches, nausea/vomiting, and decreased consciousness. Slit ventricles and sutural sclerosis were observed in 33.3% and 55.6% of patients. The mean ICP before the procedure was 48.67 mm Hg. Progressive cranial expansion was seen in all patients after surgery. The mean ICP decreased to 11 mm Hg and mean intracranial volume expansion was 85.8 cm3 at 1 month after surgery. There were no serious complications after surgery. All patients improved their symptoms, and no patient needed additional cranial expansion procedures. CONCLUSIONS: Chess-table cranial expansion is a safe and effective procedure and may be used as an alternative treatment for CCD. Progressive expansion of the intracranial volume is related to a decrease in ICP recordings and an improvement of symptoms.

Optic Nerve Atrophy Due to Long-Standing Compression by Planum Sphenoidale Meningioma.

PURPOSE: In this study we report an uncommon endoscopic endonasal image of an atrophic optic nerve as seen after surgical removal of a suprasellar meningioma. The peculiarity of this case is the long-lasting underestimated ocular symptomatology of the patient who reported a 15-year history of impairment of vision on her left eye. METHODS: A 51-year-old woman was admitted to our hospital complaining of a 15-year history of impairment of vision on her left eye. After making serendipitously the diagnosis of a suprasellar mass, we performed endoscopic endonasal surgery. RESULTS: The tumor was reached from below and removed safely, without manipulation of the optic pathways. At the end of tumor removal, the impressive left optic nerve atrophy due to enduring local tumor compression was visualized. CONCLUSIONS: To the best of our knowledge, no endoscopic endonasal image with such features has been provided in the pertinent literature. Possibly, this contribution will help identify damaged optic nerves during endoscopic endonasal surgery.

Refining the anatomic boundaries of the endoscopic endonasal transpterygoid approach: the "VELPPHA area" concept.

OBJECTIVE: The endoscopic endonasal transpterygoid route has been widely evaluated in cadavers, and it is currently used during surgery for specific diseases involving the lateral skull base. Identification of the petrous segment of the internal carotid artery (ICA) is a key step during this approach, and the vidian nerve (VN) has been described as a principal landmark for safe endonasal localization of the petrous ICA at the level of the foramen lacerum. However, the relationship of the VN to the ICA at this level is complex as well as variable and has not been described in the pertinent literature. Accordingly, the authors undertook this purely anatomical study to detail and quantify the peri-lacerum anatomy as seen via an endoscopic endonasal transpterygoid pathway. METHODS: Eight human anatomical specimens (16 sides) were dissected endonasally under direct endoscopic visualization. Anatomical landmarks of the VN and the posterior end of the vidian canal (VC) during the endoscopic endonasal transpterygoid approach were described, quantitative anatomical data were compiled, and a schematic classification of the most relevant structures encountered was proposed. RESULTS: The endoscopic endonasal transpterygoid approach was used to describe the different anatomical structures surrounding the anterior genu of the petrous ICA. Five key anatomical structures were identified and described: the VN, the eustachian tube, the foramen lacerum, the petroclival fissure, and the pharyngobasilar fascia. These structures were specifically quantified and summarized in a schematic acronym-VELPPHA-to describe the area. The VELPPHA area is a dense fibrocartilaginous space around the inferior compartment of the foramen lacerum that can be reached by following the VC posteriorly; this area represents the posterior limits of the transpterygoid approach and, of utmost importance, no neurovascular structures were observed through the VELPPHA area in this study, indicating that it should be a safe zone for surgery in the posterior end of the endoscopic endonasal transpterygoid approach. CONCLUSIONS: The VELPPHA area represents the posterior limits of the endoscopic endonasal transpterygoid approach. Early identification of this area can enhance the safety of the endoscopic endonasal transpterygoid approach expanded to the lateral aspect of the skull base, especially when treating patients with poorly pneumatized sphenoid sinuses.

Staged Management of Intracranial Masson Tumor: An Unexpected Gauntlet: Case Report and Review of the Literature.

BACKGROUND: Intravascular papillary endothelial hyperplasia (IPEH), also known as Masson tumor, is a benign lesion consisting of a reactive proliferation of endothelial cells with papillary formations related to thrombi. It has been reported in many different anatomic areas. Gross total resection is the elected treatment. Intracranial IPEH is rare, and only a few cases have been reported. This article reports a complicated case of cavernous sinus Masson tumor. CASE DESCRIPTION: A 51-year-old woman presented because of hemicraneal headache, left facial paresthesia, and diplopia (due to a slight left ocular external rectum muscle paresis) that she had experienced the previous 60 days. She had previously received a diagnosis of neurofibromatosis type I. Contrast-enhanced magnetic resonance imaging showed a 3.5-cm contrast-enhanced tumor adjacent to the left cavernous sinus involving the Meckel cave that extended around the distal petrous portion of the left internal carotid artery. Two possibilities as a differential diagnosis were suggested: meningioma or neurogenic tumor. After a staged surgical procedure, the histopathologic findings were unexpected and showed IPEH (Masson tumor) as the cause of the mass. Despite having benign features, the IPEH showed recurrences over time, so adjuvant 3-dimensional conformal radiation therapy was initiated. CONCLUSIONS: IPEH is prone to recurrences after subtotal resection. In the present case, successful surgical treatment and adjuvant radiotherapy showed an excellent outcome. To date, no adjuvant therapy has been established as a go-to option.