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Gautam U. Mehta, Franco DeMonte, Shirley Y. Su, Michael E. Kupferman, Ehab Y. Hanna and Shaan M. Raza

Chondrosarcomas of the skull base are malignant tumors for which surgery is the primary therapeutic option. Gross-total resection has been demonstrated to improve survival in patients with these tumors. Chondrosarcomas arising from the petroclival synchondrosis harbor particularly unique anatomical considerations that have long been a barrier to achieving such a resection. Endoscopic endonasal transpterygoid approaches have been recently used to gain improved access to such lesions; however, these approaches have classically relied on a medial to lateral transclival trajectory, which provides limited exposure for complete resection of lateral disease. In this paper the authors describe an endoscopic endonasal transpterygoid transnasopharyngeal approach that provides comprehensive access to the petroclival region through dissection of the eustachian tube with resection of the cartilaginous torus tubarius. Of note, the authors have previously demonstrated the superior outcomes and validity of this approach relative to other cranial base techniques for petroclival chondrosarcomas. Surgical outcomes in 5 cases of chondrosarcoma without medial extension are detailed. Gross-total resection was achieved in 4 of 5 patients. Postoperative complications included transient palatal numbness in all patients and eustachian tube dysfunction due to the approach. With tympanostomy tube placement, no patient had persistent hearing loss. Overall, this approach appears to be a safe and effective technique for resection of petroclival chondrosarcomas.

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Jack Phan, Courtney Pollard III, Paul D. Brown, Nandita Guha-Thakurta, Adam S. Garden, David I. Rosenthal, Clifton D. Fuller, Steven J. Frank, G. Brandon Gunn, William H. Morrison, Jennifer C. Ho, Jing Li, Amol J. Ghia, James N. Yang, Dershan Luo, He C. Wang, Shirley Y. Su, Shaan M. Raza, Paul W. Gidley, Ehab Y. Hanna and Franco DeMonte

OBJECTIVE

The objective of this study was to assess outcomes after Gamma Knife radiosurgery (GKRS) re-irradiation for palliation of patients with trigeminal pain secondary to recurrent malignant skull base tumors.

METHODS

From 2009 to 2016, 26 patients who had previously undergone radiation treatment to the head and neck received GKRS for palliation of trigeminal neuropathic pain secondary to recurrence of malignant skull base tumors. Twenty-two patients received single-fraction GKRS to a median dose of 17 Gy (range 15–20 Gy) prescribed to the 50% isodose line (range 43%–55%). Four patients received fractionated Gamma Knife Extend therapy to a median dose of 24 Gy in 3 fractions (range 21–27 Gy) prescribed to the 50% isodose line (range 45%–50%). Those with at least a 3-month follow-up were assessed for symptom palliation. Self-reported pain was evaluated by the numeric rating scale (NRS) and MD Anderson Symptom Inventory–Head and Neck (MDASI-HN) pain score. Frequency of as-needed (PRN) analgesic use and opioid requirement were also assessed. Baseline opioid dose was reported as a fentanyl-equivalent dose (FED) and PRN for breakthrough pain use as oral morphine-equivalent dose (OMED). The chi-square and Student t-tests were used to determine differences before and after GKRS.

RESULTS

Seven patients (29%) were excluded due to local disease progression. Two experienced progression at the first follow-up, and 5 had local recurrence from disease outside the GKRS volume. Nineteen patients were assessed for symptom palliation with a median follow-up duration of 10.4 months (range 3.0–34.4 months). At 3 months after GKRS, the NRS scores (n = 19) decreased from 4.65 ± 3.45 to 1.47 ± 2.11 (p < 0.001); MDASI-HN pain scores (n = 13) decreased from 5.02 ± 1.68 to 2.02 ± 1.54 (p < 0.01); scheduled FED (n = 19) decreased from 62.4 ± 102.1 to 27.9 ± 45.5 mcg/hr (p < 0.01); PRN OMED (n = 19) decreased from 43.9 ± 77.5 to 10.9 ± 20.8 mg/day (p = 0.02); and frequency of any PRN analgesic use (n = 19) decreased from 0.49 ± 0.55 to 1.33 ± 0.90 per day (p = 0.08). At 6 months after GKRS, 9 (56%) of 16 patients reported being pain free (NRS score 0), with 6 (67%) of the 9 being both pain free and not requiring analgesic medications. One patient treated early in our experience developed a temporary increase in trigeminal pain 3–4 days after GKRS requiring hospitalization. All subsequently treated patients were given a single dose of intravenous steroids immediately after GKRS followed by a 2–3-week oral steroid taper. No further cases of increased or new pain after treatment were observed after this intervention.

CONCLUSIONS

GKRS for palliation of trigeminal pain secondary to recurrent malignant skull base tumors demonstrated a significant decrease in patient-reported pain and opioid requirement. Additional patients and a longer follow-up duration are needed to assess durability of symptom relief and local control.

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Rohan Ramakrishna, Shaan M. Raza, Michael Kupferman, Ehab Hanna and Franco DeMonte

OBJECT

Adenoid cystic carcinoma (ACC) is a locally aggressive tumor of salivary gland origin. Little data exist to guide treatment when this tumor extends to involve the structures of the skull base.

METHODS

Fifty-one patients with a diagnosis of ACC affecting the skull base were identified from a prospective database at MD Anderson Cancer Center (from 1992 to 2010).

RESULTS

Median follow-up for study patients was 6.75 years. The 5- and 10-year overall survival (OS) rates were 78% and 50%, respectively. Sixty-six percent of patients had progression of their disease. The 5- and 10-year progression-free survival (PFS) rates were 46.7% and 21.0%, respectively. Gross-total resection was achieved in 75% of patients, with 49% having microscopically negative margins at the time of first operation. On univariate analysis, resections with microscopically negative margins were associated with a significant OS advantage (20.1 ± 3.3 years) compared with resections that left residual disease, even if microscopic (10.3 ± 1.6 years, p = 0.035). In patients who underwent reoperation, the effect persisted, with improved OS in those with negative margins (21.4 ± 0.0 vs 16.7 ± 4.0 years, p = 0.06). The use of adjuvant radiotherapy was associated with an OS advantage (16.2 ± 2.5 vs 5.5 ± 2.2 years, p = 0.03) at initial diagnosis and improved PFS (7.8 ± 1.0 vs 2.1 ± 0.62 years, p = 0.005), whereas repeat irradiation provided no benefit. The use of adjuvant chemotherapy at diagnosis or at recurrence was not associated with any significant advantage. Multivariate analysis revealed margin-negative resection at initial operation and at recurrence retained OS significance, even after controlling for age, radiation therapy, and T stage.

CONCLUSIONS

ACC of the skull base is best treated with a multidisciplinary approach aimed at maximal, safe resection. Adjuvant radiotherapy should be offered, whereas chemotherapy does not confer benefit.

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Shaan M. Raza, Rohan Ramakrishna, Randal S. Weber, Michael E. Kupferman, Paul W. Gidley, Ehab Y. Hanna and Franco DeMonte

OBJECT

A relative paucity of information exists regarding outcomes from craniofacial resection for advanced nonmelanoma skin cancers involving the skull base. In light of advances in surgical technique and adjuvant therapy protocols, the authors reviewed their surgical experience to determine disease control rates, overall survival (OS), morbidity, and mortality.

METHODS

A retrospective review of 24 patients with nonmelanoma cutaneous cancers with skull base involvement treated with craniofacial resection at The University of Texas MD Anderson Cancer Center from 1994 to 2012 was performed. Of these patients, 19 (79%) had squamous cell carcinoma (SCC), 4 (17%) had basosquamous carcinoma (BSCC), and 1 patient (4%) had adenocarcinoma. Factors as assessed were prior treatment, TNM staging, tumor involvement, extent of intracranial extension, margin status, postoperative complications, recurrence, disease status at last follow-up, and long-term survival. The majority of tumors were T4 (67%) according to the TNM classification; perineural extension was noted in 58%, cavernous sinus involvement in 25%, and dural involvement in 29%.

RESULTS

Postoperative complications occurred in 4 patients (17%) including 1 death. Kaplan-Meier estimates were calculated for OS and progression-free survival (PFS). Median OS was 43.2 months with an 82% 1-year OS and 37% 5-year OS; the median PFS was 91.2 months. Margin status was positively associated with median OS in SCC (91 months [for negative margins] vs 57 months, p = 0.8) and in BSCC (23.7 vs 3.2 months, p < 0.05). Postoperative radiotherapy was associated with improved median OS (43.2 vs 22 months, p = 0.6). Brain involvement was uniformly fatal after 1 year, while cavernous sinus involvement (31 vs 43 months, p = 0.82), perineural disease (31 vs 54 months, p = 0.30), and T4 stage (22 vs 91.2 months, p = 0.09) were associated with worsened OS. Similar associations were found with median PFS.

CONCLUSIONS

Aggressive multimodality management with surgery and postoperative radiotherapy can positively impact locoregional control and OS. With improvements in technique and adjuvant therapy protocols, treatment can still be considered in situations of perineural disease and cavernous sinus involvement and as a salvage option for patients in whom prior treatment has failed. As patients with advanced NMSCs often have few options, craniofacial resection, as part of a coordinated multimodal management plan, is justified if it can be performed safely.

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Shaan M. Raza and Franco DeMonte

This video describes the surgical management of an epidermoid cyst within the cerebellopontine angle and petroclival region with involvement of cranial nerves V through XI and the vertebrobasilar system. A retrosigmoid craniotomy was performed for gross total resection of the lesion. The key steps of the procedure are discussed, including: positioning, soft tissue dissection, craniotomy, microsurgical dissection/resection, closure. Additionally, surgical nuances with regards to the safe maximal resection of such lesions are detailed.

The video can be found here: http://youtu.be/VEROVO5cYdU.

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Franco DeMonte and Paul W. Gidley

Object

In the early 1960s William F. House developed the middle fossa approach for the removal of small vestibular schwannomas (VSs) with the preservation of hearing. It is the best approach for tumors that extend laterally to the fundus of the internal auditory canal, although it does have the potential disadvantage of increased facial nerve manipulation, especially for tumors arising from the inferior vestibular nerve. The aim of this study was to monitor the hearing preservation and facial nerve outcomes of this approach.

Methods

A prospective database was constructed, and data were retrospectively reviewed.

Results

Between December 2004 and January 2012, 30 patients with small VSs underwent surgery via a middle fossa approach for hearing preservation. The patients consisted of 13 men and 17 women with a mean age of 46 years. Tumor size ranged from 7 to 19 mm. Gross-total resection was accomplished in 25 of 30 patients. Preoperative hearing was American Academy of Otolaryngology–Head and Neck Surgery (AAO-HNS) Class A in 21 patients, Class B in 5, Class C in 3, and undocumented in 1. Postoperatively, hearing was graded as AAO-HNS Class A in 15 patients, Class B in 7, Class C in 1, Class D in 2, and undocumented in 5. Facial nerve function was House-Brackmann (HB) Grade I in all patients preoperatively. Postoperatively, facial nerve function was HB Grade I in 28 patients, Grade III in 1, and Grade IV in 1. There were 3 complications: CSF leakage in 1 patient, superficial wound infection in 1, and extradural hematoma (asymptomatic) in 1. The overall hearing preservation rate of at least 73% and HB Grade I facial nerve outcome of 93% in this cohort are in keeping with other contemporary reports.

Conclusions

The middle fossa approach for the resection of small VSs with hearing preservation is a viable and relatively safe option. It should be considered among the various options available for the management of small, growing VSs.

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Wael Hassaneen, Nicholas B. Levine, Dima Suki, Abhijit L. Salaskar, Alessandra de Moura Lima, Ian E. McCutcheon, Sujit S. Prabhu, Frederick F. Lang, Franco DeMonte, Ganesh Rao, Jeffrey S. Weinberg, David M. Wildrick, Kenneth D. Aldape and Raymond Sawaya

Object

Multiple craniotomies have been performed for resection of multiple brain metastases in the same surgical session with satisfactory outcomes, but the role of this procedure in the management of multifocal and multicentric glioblastomas is undetermined, although it is not the standard approach at most centers.

Methods

The authors performed a retrospective analysis of data prospectively collected between 1993 and 2008 in 20 patients with multifocal or multicentric glioblastomas (Group A) who underwent resection of all lesions via multiple craniotomies during a single surgical session. Twenty patients who underwent resection of solitary glioblastoma (Group B) were selected to match Group A with respect to the preoperative Karnofsky Performance Scale (KPS) score, tumor functional grade, extent of resection, age at time of surgery, and year of surgery. Clinical and neurosurgical outcomes were evaluated.

Results

In Group A, the median age was 52 years (range 32–78 years); 70% of patients were male; the median preoperative KPS score was 80 (range 50–100); and 9 patients had multicentric glioblastomas and 11 had multifocal glioblastomas. Aggressive resection of all lesions in Group A was achieved via multiple craniotomies in the same session, with a median extent of resection of 100%. Groups A and B were comparable with respect to all the matching variables as well as the amount of tumor necrosis, number of cysts, and the use of intraoperative navigation. The overall median survival duration was 9.7 months in Group A and 10.5 months in Group B (p = 0.34). Group A and Group B (single craniotomy) had complication rates of 30% and 35% and 30-day mortality rates of 5% (1 patient) and 0%, respectively.

Conclusions

Aggressive resection of all lesions in selected patients with multifocal or multicentric glioblastomas resulted in a survival duration comparable with that of patients undergoing surgery for a single lesion, without an associated increase in postoperative morbidity. This finding may indicate that conventional wisdom of a minimal role for surgical treatment in glioblastoma should at least be questioned.

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Susan L. McGovern, Kenneth D. Aldape, Mark F. Munsell, Anita Mahajan, Franco DeMonte and Shiao Y. Woo

Object

Despite a favorable outcome for most patients with WHO Grade I meningiomas, a subset of these patients will have recurrent or progressive disease that advances to a higher grade and requires increasingly aggressive therapy. The goal of this study was to identify clinical characteristics associated with the recurrence of benign meningiomas and their acceleration to atypical and malignant histological types.

Methods

Records of 216 patients with WHO Grade I, II, or III meningioma that were initially treated between 1965 and 2001 were retrospectively reviewed. Median follow-up was 7.2 years.

Results

Patients with non–skull base cranial meningiomas (82 of 105 [78%]) were more likely to have undergone a gross-total resection than patients with skull base meningiomas (32 of 78 [41%]; p < 0.001). Consequently, patients with Grade I non–skull base cranial meningiomas had better 5-year recurrence-free survival (69%) than patients with Grade I skull base meningiomas (56%) or Grade II or III tumors at any site (50%; p = 0.005). Unexpectedly, patients with non–skull base tumors who experienced a recurrence (8 of 22 [36%]) were more likely than patients with skull base tumors (1 of 19 [5%]) to have a higher grade tumor at recurrence (p = 0.024). Furthermore, the median MIB-1 labeling index of Grade I non–skull base cranial meningiomas (2.60%) was significantly higher than that of Grade I skull base tumors (1.35%; p = 0.016).

Conclusions

Cranial meningiomas that occur outside of the skull base are more likely to have a higher MIB-1 labeling index and recur with a higher grade than those within the skull base, suggesting that non–skull base cranial tumors may have a more aggressive biology than skull base tumors.

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Sarfaraz Sadruddin, L. Jeffrey Medeiros and Franco DeMonte

The rare occurrence of T-cell lymphoblastic lymphoma as a primary tumor in the cavernous sinus is described. The patient, a 17-year-old girl, presented with right-sided ophthalmic and maxillary neuropathy and diplopia due to neuropathies of cranial nerves III and VI. An enhancing mass in the cavernous sinus was identified on MR imaging. Dexamethasone was prescribed but did not provide symptomatic relief. Rapid progression of symptoms led to open biopsy, and a diagnosis of T-cell lymphoblastic lymphoma was made. The patient promptly underwent aggressive chemotherapy in which a modified hyper–cyclophosphamide, vincristine, and dexamethasone without doxorubicin regimen with concurrent radiotherapy was used. The patient achieved complete remission and is currently completing the 2-year maintenance phase of chemotherapy.