Three-Dimensional Volumetric Changes in Posterior Vault Distraction With Distraction Osteogenesis.

OBJECT: Distraction osteogenesis (DO) may allow for maximal volumetric expansion in the posterior vault (PV) by overcoming viscoelastic forces of overlying soft tissues. Little evidence exists regarding surgical planning and anticipated 3D volumetric changes pre- and post-operatively. We aim to study the volumetric changes occurring in PV distraction in lambdoid craniosynostosis. METHODS: From 2007 to 2019, a single institution retrospective review revealed 232 craniosynostosis patients. Fourteen demonstrated lambdoid synostosis (6%), and of those, 11 patients were included in the study due to treatment with PVDO or representative sample. Six patients had unilateral synostosis and 5 had bilateral synostosis. Imaging protocol for PVDO patients included preoperative head CT within 1 month of surgery and 8 weeks following distraction cessation with weekly skull plain films. 3D volumetric analyses were performed on pre and postoperative head CT using 3D Slicer software. RESULTS: Posterior fossa volume (PFV) increased by 38.7% and foramen magnum area increased by 26.9% postoperatively. Unilateral lambdoid craniosynostosis patients had greater increases in PFV versus bilateral lambdoid craniosynostosis patients (63.5% versus 8.9%, P = 0.007). Osteotomy to the asterion was more effective in increasing PFV versus osteotomy to foramen magnum (P = 0.050). Placement of distractor in the inferior third of the lambdoid suture is more effective in increasing PFV versus placement in the middle or top third of the suture (P = 0.041). CONCLUSIONS: Highest volumetric increases are seen in unilateral lambdoid synostosis. Extending osteotomy beyond the asterion is not necessary for maximal PV volumetric gain. Placement of distractor in the inferior third of the suture leads to maximal PV volumetric gains.

Brachytherapy in the treatment of recurrent aggressive falcine meningiomas.

Recurrent aggressive falcine meningiomas are uncommon tumors that recur despite receiving extensive surgery and radiation therapy (RT). We have utilized brachytherapy as a salvage treatment in two such patients with a unique implantation technique. Both patients had recurrence of WHO Grade II falcine meningiomas despite multiple prior surgical and RT treatments. Radioactive I-125 seeds were made into strands and sutured into a mesh implant, with 1 cm spacing, in a size appropriate to cover the cavity and region of susceptible falcine dura. Following resection the vicryl mesh was implanted and fixed to the margins of the falx. Implantation in this interhemispheric space provides good dose conformality with targeting of at-risk tissue and minimal radiation exposure to normal neural tissues. The patients are recurrence free 31 and 10 months after brachytherapy treatment. Brachytherapy was an effective salvage treatment for the recurrent aggressive falcine meningiomas in our two patients.

Development of a Modified Bayonet Forceps for Improving Steerability of Paddle Lead Electrodes During Spinal Cord Stimulator Surgery: A Technical Note.

BACKGROUND AND OBJECTIVE: Despite recent advancements in spinal cord stimulation (SCS) technology, the surgical instrumentation for placement of SCS paddle leads remains suboptimal. Therefore, we developed a novel instrument to improve the steerability of SCS paddle leads during surgical placement. METHODS: A review of existing literature was performed to analyze workflow deficiencies in the standard instrumentation of SCS paddle lead placement. After a period of adaptation and iterative feedback with a medical instrument company, a new instrument was developed, tested at benchtop, and successfully incorporated into the surgical routine. RESULTS: A standard bayonet forceps was modified to include hooked ends and a ribbed surface, providing the surgeon with greater control over the paddle lead. The new instrument also included bilateral metal tubes starting approximately 4 cm proximal from the edge of the forceps. The bilateral metal tubes, through which the SCS paddle lead wires are passed, serve as anchors to keep the wires away from the incision site. In addition, it permitted the paddle lead to assume a bent configuration, reducing its overall size and allowing it to be placed through a smaller incision and laminectomy. The modified bayonet forceps was successfully used intraoperatively for placement of SCS paddle lead electrodes in several surgeries. CONCLUSION: The proposed modified bayonet forceps increased steerability of the paddle lead, facilitating optimal midline placement. The bent configuration of the device facilitated a more minimally invasive surgical approach. Future studies are needed to validate our single-provider experience and evaluate the impact of this new instrument on operating room efficiency.

A Rare Case of Life-Threatening Multicompartmental Spontaneous Intracranial Hemorrhage From a Grade 1 Convexity Meningioma.

Meningiomas are slowly growing benign tumors. The incidence of hemorrhage associated with intracranial meningiomas is in the 0.5%-2.4% range. However, intracranial meningiomas with hemorrhagic presentation are associated with higher rates of overall major morbidity (36%) and mortality (21.1%). We report a case of a convexity meningioma presenting with intraparenchymal hematoma and bilateral acute subdural hematomas (SDH) in a comatose patient (Glasgow Coma Scale (GCS) score: 7) who had a history of recurrent episodes of headaches over the past few months. Hemorrhagic presentation of a meningioma is a rare but potentially devastating event. Early recognition of the potential underlying meningioma as a cause of bleeding followed by rapid appropriate additional imaging is crucial to direct treatment plans to achieve the best outcome.

Radical Resection of a Giant Epidermoid Tumor Associated With Miniature Chordoma Utilizing a Combined Endoscopic-Microscopic Technique: 2-Dimensional Operative Video.

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. The transmastoid approach by skeletonizing and reflexing the transverse-sigmoid sinus offers wide exposure of the cerebellopontine angle avoiding cerebellar retraction and allowing 4-hands dissection.5 The patient is a 17-yr-old male with a giant epidermoid tumor in the cerebellopontine angle, extending through the incisura. The patient underwent surgical resection with maximum pursuit of the epithelial capsule. After removing the epidermoid tumor, a miniature intra and extradural midclival tumor was encountered and removed with a proven pathology of chordoma. Patient did well postoperatively with relief of his hemifacial spasms. Patient consented for surgery and photograph publication. Image at 1:23, ©1997, O. Al-Mefty, used with permission. All rights reserved.

Microsurgical Resection of a Parasellar Meningioma Invading the Cavernous Sinus, Bone, and Optic Canal: 2-Dimensional Operative Video.

Parasellar meningiomas, regardless of the initial origin, frequently involve the optic canal and cavernous sinus, leading to visual loss and ocular movement dysfunction.1 Hyperostotic bony invasion to the surrounding skull base is common.2 Visual acuity prognosis can be improved by surgical resection through different technical maneuvers, including opening the optic canal and the preservation of the visual apparatus vascular supply by developing the intra-arachnoidal dissection planes.1,3,4 To achieve radical dissection and reduce recurrences, the extradural invaded bone is thoroughly resected. Likewise, the soft and nonadherent invasive tumor around the cavernous carotid can be extensively removed.1,3,4 New or worsening of the ophthalmoplegia is frequent after cavernous sinus exploration and tumor resection. However, it is usually temporary and improves progressively in 80% of patients.1,3,4 Safely approaching these lesions requires extensive anatomic knowledge through cadaveric dissection and training, allowing us to achieve tumor control, preserve or improve visual function, and avoid or delay irradiation therapy.1,3,4 Pituitary function preservation is also possible in many patients through surgery, which is at higher risk with irradiation. We demonstrate the technical maneuvers through a case of a 49-yr-old woman who presented with a parasellar meningioma involving the cavernous sinus, both optic canals, and extensive bony invasion. The patient consented to the surgery and publication of her images. Image at 1:14 and middle and right image at 1:31 reprinted with permission from Al-Mefty O, Operative Atlas of Meningiomas. Vol 1, ©LWW, 1998. Left image at 1:31 reprinted from Arnautovic et al,5 by permission from JNSPG.

Is Falcine Meningioma a Diffuse Disease of the Falx? Case Series and Analysis of a "Grade Zero" Resection.

BACKGROUND: Falcine meningiomas have unique characteristics including their high rates of recurrence, association with high grade pathology, increased male prevalence, and potential for diffuse involvement of the falx. OBJECTIVE: To address these issues in a substantial series of falcine meningiomas and report on the impact of extent of resection for this distinct meningioma entity. METHODS: Retrospective analysis of characteristics and outcomes of 59 falcine meningioma patients who underwent surgery with the senior author. A "Grade Zero" category was used when an additional resection margin of 2 to 3 cm from the tumor insertion was achieved. RESULTS: For de novo falcine meningiomas, gross total resection (GTR) was associated with significantly decreased recurrence incidence compared with subtotal resection (P ≤ .0001). For recurrent falcine meningiomas, median progression-free survival (PFS) was significantly improved for GTR cases (37 mo vs 12 mo; P = .017, hazard ratio (HR) .243 (.077-.774)). "Grade Zero" resection demonstrated excellent durability for both de novo and recurrent cases, and PFS was significantly improved with "Grade Zero" resection for recurrent cases (P = .003, HR 1.544 (1.156-2.062)). The PFS benefit of "Grade Zero" resection did not achieve statistical significance over Simpson grade 1 during the limited follow-up period (mean 2.8 yr) for these groups. CONCLUSION: The recurrence of falcine meningiomas is related to the diffuse presence of tumor between the leaflets of the falx. Increased extent of resection including, when possible, a clear margin of falx surrounding the tumor base was associated with the best long-term outcomes in our series, particularly for recurrent tumors.

The Currarino triad: What pediatric surgeons need to know.

PURPOSE: We report our experience in managing a group of patients with Currarino syndrome, highlighting diagnostic challenges, surgical techniques, in addition to a review of current neurosurgical options. PATIENTS AND METHODS: The study included patients with Currarino syndrome who presented to our pediatric surgery department during the period 2010 through 2016. The 'sacral scimitar' in plain X-ray provided the clue for the diagnosis; while MRI examination was essential to define the nature of the presacral mass and associated spinal anomalies. RESULTS: The study included 17 patients (13 girls and 4 boys). Their age at presentation ranged from 7months to 10years. We used posterior sagittal approach to correct anorectal anomalies, and excise presacral cysts that were subjected to histopathological examination. Two cases presented with a pelvic abscess (infected presacral dermoid cyst), which were initially drained followed by excision. The presacral mass consisted of either lipomyelocele (6), lipomyelomeningocele (3), or a developmental (dermoid) cyst (8). Tethering of the spinal cord was a common association (70%) CONCLUSION: Apart from diagnostic challenges, the management of Currarino syndrome is similar to the usual management of ARM regarding the surgical approach and probably the prognosis that mainly depends on degree of associated sacral dysplasia. LEVEL OF EVIDENCE: This is a case series with no comparison group (level IV).

Utility of dynamic computed tomography angiography in the preoperative evaluation of skull base tumors.

OBJECT: The anatomical complexity of skull base tumors mandates detailed preoperative planning for safe resection. In particular, the location of critical vascular and bony structures can influence the surgical approach. Traditional methods, such as MRI, MR angiography and/or venography (MRA/MRV), CT angiography and/or venography (CTA/CTV), and digital subtraction angiography, each have their limitations. One alternative that combines the benefits of both detailed anatomy compatible with intraoperative image guidance and visualization of the vascular flow is the 320-detector row dynamic volume CTA/CTV. The authors investigated this technique's impact on the surgical approach used in a series of complex intracranial tumors. METHODS: All patients with complex intracranial tumors who had undergone preoperative dynamic CTA/CTV as well as MRI in the period from July 2010 to June 2012 were retrospectively reviewed. Those in whom only routine CTA/CTV sequences had been obtained were excluded. Clinical records, including imaging studies, operative reports, and hospital course, were reviewed. Ease in detecting specific major arterial and venous tributaries using dynamic CTA/CTV was graded for each case. Furthermore, 2 skull base neurosurgeons projected a desired surgical approach for each tumor based on MRI studies, independent of the CTA/CTV sequences. The projected approach was then compared with the ultimately chosen surgical approach to determine whether preoperative awareness of vasculature patterns altered the actual operative approach. RESULTS: Sixty-four patients were eligible for analysis. Dynamic CTA/CTV successfully demonstrated circle of Willis arteries, major draining sinuses, and deep internal venous drainage in all cases examined. The superior petrosal sinus, vein of Labbé, tentorial veins, and middle fossa veins were also identified in a majority of cases, which played an important role in preoperative planning. Visualization of critical vascular-especially venous-anatomy influenced the surgical approach in 39% (25 of 64) of the cases. CONCLUSIONS: Dynamic CTA/CTV has been applied to few neurosurgical disease pathologies to date. This noninvasive technology offers insight into vascular flow patterns as well as 3D anatomical relationships and provides thin-cut sequences for intraoperative navigation. The authors propose dynamic CTA as an addition to the preoperative planning for complex skull base tumors.

Giant intracranial epidermoids: is total removal feasible?

OBJECT: Epidermoid tumors arise from misplaced squamous epithelium and enlarge through the accumulation of desquamated cell debris. Optimal treatment consists of total removal of the capsule; therefore, giant and multicompartmental tumors are particularly challenging. A conservative attitude in handling the tumor capsule is common given concerns about capsule adherence to neurovascular structures, and thus the possibility of recurrence is accepted with the intent of minimizing complications. This study focuses on the outcome of surgery in patients with giant epidermoid tumors for which total capsule removal was the aim. METHODS: The authors conducted a retrospective analysis of all patients with giant epidermoid tumors treated by the senior author (O.A.), who pursued total removal of the capsule through skull base approaches. Patients were divided into 2 groups: one including patients with de novo tumors and the other consisting of patients who presented with recurrent tumors. RESULTS: Thirty-four patients had undergone 46 operations, and the senior author performed 38 of these operations in the study period. The average tumor dimensions were 55 × 36 mm, and 25 tumors had multicompartmental extensions. Total removal of the tumor and capsule was achieved with the aid of the microscope in 73% of the 26 de novo cases but in only 17% of the 12 recurrent tumor cases. The average follow-up among all patients was 111 months (range 10-480 months), and the average postsurgical follow-up was 56.8 months (range 6-137 months). There were 4 recurrences in the de novo group, and every case had had a small piece of tumor capsule left behind. One patient died after delayed rupture of a pseudoaneurysm. In the de novo group, the average preoperative Karnofsky Performance Scale (KPS) score was 71.42%, which improved to 87.14% on long-term follow-up. In the group with recurrences, the KPS score also improved on long-term follow-up, from 64.54% to 84.54%. In the de novo group, 3 cases (11.5%) had permanent cranial nerve deficits, and 4 cases (15.4%) had a CSF leak. In the recurrence group, 3 cases (25%) had new, permanent cranial nerve deficits, and 1 (8.3%) had a CSF leak. Two patients in this group developed hydrocephalus and required a shunt. CONCLUSIONS: Total removal of the capsule of giant epidermoid tumors was achieved in 73% of patients with de novo tumors and was associated with improved function, low morbidity and mortality, and a lower risk of recurrence. Surgery in patients with recurrent tumors was associated with higher morbidity and persistence of the disease.

"Live cadavers" for training in the management of intraoperative aneurysmal rupture.

OBJECT: Intraoperative rupture occurs in approximately 9.2% of all cranial aneurysm surgeries. This event is not merely a surgical complication, it is also a real surgical crisis that requires swift and decisive action. Neurosurgical residents may have little exposure to this event, but they may face it in their practice. Laboratory training would be invaluable for developing competency in addressing this crisis. In this study, the authors present the "live cadaver" model, which allows repetitive training under lifelike conditions for residents and other trainees to practice managing this crisis. METHODS: The authors have used the live cadaver model in 13 training courses from 2009 to 2014 to train residents and neurosurgeons in the management of intraoperative aneurysmal rupture. Twenty-three cadaveric head specimens harboring 57 artificial and 2 real aneurysms were used in these courses. Specimens were specially prepared for this technique and connected to a pump that sent artificial blood into the vessels. This setting created a lifelike situation in the cadaver that simulates live surgery in terms of bleeding, pulsation, and softness of tissue. RESULTS: A total of 203 neurosurgical residents and 89 neurosurgeons and faculty members have practiced and experienced the live cadaver model. Clipping of the aneurysm and management of an intraoperative rupture was first demonstrated by an instructor. Then, trainees worked for 20- to 30-minute sessions each, during which they practiced clipping and reconstruction techniques and managed intraoperative ruptures. Ninety-one of the participants (27 faculty members and 64 participants) completed a questionnaire to rate their personal experience with the model. Most either agreed or strongly agreed that the model was a valid simulation of the conditions of live surgery on cerebral aneurysms and represents a realistic simulation of aneurysmal clipping and intraoperative rupture. Actual performance improvement with this model will require detailed measurement for validating its effectiveness. The model lends itself to evaluation using precise performance measurements. CONCLUSIONS: The live cadaver model presents a useful simulation of the conditions of live surgery for clipping cerebral aneurysms and managing intraoperative rupture. This model provides a means of practice and promotes team management of intraoperative cerebrovascular critical events. Precise metric measurement for evaluation of training performance improvement can be applied.

The combined microscopic-endoscopic technique for radical resection of cerebellopontine angle tumors.

OBJECT: The combined microscopic and endoscopic technique has shown significant advantages in the management of various lesions through different approaches. Endoscopic-assisted techniques have frequently been applied to cerebellopontine angle (CPA) surgery in the context of minimally invasive craniotomies. In this paper the authors report on the use of the endoscope in the CPA as a tool to increase the extent of resection, minimize complications, and preserve the function of the delicate CPA structures. They also describe a technique of the simultaneous use of the microscope and endoscope in the CPA and dissection of CPA tumors under tandem endoscopic and microscopic vision to overcome the shortcomings of introducing the endoscope alone in the CPA. The reliability of using the microscope alone in dissecting CPA tumors is evaluated, as is the effectiveness of the combined technique in increasing the resectability of various types of CPA tumors. METHODS: The authors conducted a retrospective analysis of 50 patients who underwent combined microscopic-endoscopic resection of CPA tumors by the senior author over a period of 3 years (February 2011 to February 2014) at Brigham and Women's Hospital, Harvard Medical School. The reliability of the extent of microscopic removal was evaluated with endoscopic exploration. Additional resection was performed with both microscopic and the combined microscopic-endoscopic technique. Endoscopically verified total resection was validated by intraoperative or postoperative MRI. The function of the cranial nerves was evaluated to assess the impact of the combined technique on their function. RESULTS: A tumor remnant was endoscopically identified in 69% of the 26 patients who were believed to have microscopic total resection. The utilization of the endoscopic visualization and dissection increased endoscopically verified total removal to 38 patients, and 82% of these patients had no sign of residual tumor on postoperative imaging. The technique was most effective with epidermoid tumors. There were a total of 17 new cranial nerve deficits in 10 patients. Preoperative fifth cranial nerve deficits improved in 52% and hearing improved in 29% of patients after surgery. CONCLUSIONS: This method provides simultaneous microscopic and endoscopic visualization and dissection techniques through skull-base approaches to CPA tumors. It overcomes some of the shortcomings of endoscopic-assisted surgery, further extends the surgical field, and increases the radicality of tumor resection with good functional outcomes.

True petroclival meningiomas: results of surgical management.

OBJECT: The relentless natural progression of petroclival meningiomas mandates their treatment. The management of these tumors, however, is challenging. Among the issues debated are goals of treatment, outcomes, and quality of life, appropriate extent of surgical removal, the role of skull base approaches, and the efficacy of combined decompressive surgery and radiosurgery. The authors report on the outcome in a series of patients treated with the goal of total removal. METHODS: The authors conducted a retrospective analysis of 64 cases of petroclival meningiomas operated on by the senior author (O.A.) from 1988 to 2012, strictly defined as those originating medial to the fifth cranial nerve on the upper two-thirds of the clivus. The patients' average age was 49 years; the average tumor size (maximum diameter) was 35.48 ± 10.09 mm (with 59 tumors > 20 mm), and cavernous sinus extension was present in 39 patients. The mean duration of follow-up was 71.57 months (range 4-276 months). RESULTS: In 42 patients, the operative reports allowed the grading of resection. Grade I resection (tumor, dura, and bone) was achieved in 17 patients (40.4%); there was no recurrence in this group (p = 0.0045). Grade II (tumor, dura) was achieved in 15 patients (36%). There was a statistically significant difference in the rate of recurrence with respect to resection grade (Grades I and II vs other grades, p = 0.0052). In all patients, tumor removal was classified based on postoperative contrast-enhanced MRI, and gross-total resection (GTR) was considered to be achieved if there was no enhancement present; on this basis, GTR was achieved in 41 (64%) of 64 patients, with a significantly lower recurrence rate in these patients than in the group with residual enhancement (p = 0.00348). One patient died from pulmonary embolism after discharge. The mean Karnofsky Performance Status (KPS) score was 85.31 preoperatively (median 90) and improved on follow-up to 88, with 30 patients (47%) having an improved KPS score on follow-up. Three patients suffered a permanent deficit that significantly affected their KPS. Cerebrospinal fluid leak occurred in 8 patients (12.5%), with 2 of them requiring exploration. Eighty-nine percent of the patients had cranial nerve deficits on presentation; of the 54 patients with more than 2 months of follow-up, 21 (32.8%) had persisting cranial nerve deficits. The overall odds of permanent cranial nerve deficit of treated petroclival meningioma was 6.2%. There was no difference with respect to immediate postoperative cranial nerve deficit in patients who had GTR compared with those who had subtotal resection. CONCLUSIONS: Total removal (Grade I or II resection) of petroclival meningiomas is achievable in 76.4% of cases and is facilitated by the use of skull base approaches, with good outcome and functional status. In cases in which circumstances prevent total removal, residual tumors can be followed until progression is evident, at which point further intervention can be planned.

Transmastoid retrosigmoid approach to the cerebellopontine angle: surgical technique.

BACKGROUND: The traditional suboccipital craniotomy in the retrosigmoid approach gives limited exposure to the cerebellopontine angle (CPA) structures and necessitates cerebellar retraction, whereas the addition of drilling of the mastoid process with reflection of venous sinuses offers wider exposure of the CPA and avoids cerebellar retraction. We describe the details of the surgical technique and provide radiological measurements substantiating the advantages of this approach. OBJECTIVE: To validate the usefulness of partial mastoidectomy in the retrosigmoid approach and to evaluate the complications of this maneuver. METHODS: Radiological CPA measurements on computed tomography bone window films were made on the last consecutive 20 patients who underwent CPA surgery via the transmastoid retrosigmoid approach. We measured the distance and angle of work by this approach and compared the measurements with those using the traditional retrosigmoid approach if that would have been used in each case. We also reviewed 432 patients from the records of the senior author to evaluate possible complications of this approach. RESULTS: The mean working distance for the transmastoid approach was 23.06 mm, whereas the working distance in the traditional approach was 46.44 mm. The mean increase in the angle of work after drilling of the mastoid was 25.39 degrees, and the simple average of increased distance in lateral exposure was 26.66 mm. CONCLUSION: The transmastoid retrosigmoid approach increases the exposure and gives better access to the CPA targets. This approach alleviates cerebellar retraction, facilitates surgery in the supine position, promotes the use of the endoscope, and is associated with negligible complications.

Primary calcified rhabdoid meningioma of the cranio-cervical junction: A case report and review of literature.

Rhabdoid meningioma (RM) is a relatively new, rare, and aggressive subtype of meningioma, classified as Grade III malignancy in 2000, 2007 versions of WHO classification of the central nervous system. We reviewed the data available from all published cases of RMs. To the best of our knowledge, there are more than 100 published cases of RMs; none have documented extensive calcification or origin from the cranio cervical junction. We report the first case of a totally calcified (stony mass), primary RM, at the cranio cervical junction. Also, we highlighted the role of the transcondylar approach to achieve microscopic total removal of such a challenging lesion. A 37 year old female, allergic to erythromycin, presented with 5 years of progressive right upper extremity numbness and weakness, right facial numbness, and occipital pain. Imaging demonstrated a large calcified mass at the right posterior-lateral margin of the cranio cervical junction, encasing the right vertebral artery and right PICA loop. Patient underwent microscopic total resection of the lesion. Pathological diagnosis was confirmed as RM with atypical features. Subsequently, the patient received postoperative intensity modulated radiotherapy (IMRT) on the tumor bed, and close follow up imaging showed no recurrence 2 years after surgery. We report the first case of a primary RM originating from the cranial cervical junction; also, it is the first case to present with extensive calcification in this morphological subtype. We also conclude that RM has now become a feature of newly diagnosed cases and not only a disease of recurrent cases as it was thought in the past. Since RMs are typically considered aggressive, total surgical resection with close follow up and postoperative adjuvant radiation should be considered. However, the adjuvant therapy of each separate case of RM should be tailored according to its particular histopathologic profile.