Joao Paulo Almeida and Fred Gentili
Mazda K. Turel, Georgios Tsermoulas, Lior Gonen, George Klironomos, Joao Paulo Almeida, Gelareh Zadeh and Fred Gentili
The treatment of recurrent and residual craniopharyngiomas is challenging. In this study the authors describe their experience with these tumors and make recommendations on their management.
The authors performed an observational study of adult patients (≥ 18 years) with recurrent or residual craniopharyngiomas that were managed at their tertiary center. Retrospective data were collected on demographics and clinical, imaging, and treatment characteristics from patients who had a minimum 2-year follow-up. Descriptive statistics were used and the data were analyzed.
There were 42 patients (27 male, 15 female) with a mean age of 46.3 ± 14.3 years. The average tumor size was 3.1 ± 1.1 cm. The average time to first recurrence was 3.6 ± 5.5 years (range 0.2–27 years). One in 5 patients (8/42) with residual/recurrent tumors did not require any active treatment. Of the 34 patients who underwent repeat treatment, 12 (35.3%) had surgery only (transcranial, endoscopic, or both), 9 (26.5%) underwent surgery followed by adjuvant radiation therapy (RT), and 13 (38.2%) received RT alone. Eighty-six percent (18/21) had a gross-total (n = 4) or near-total (n = 14) resection of the recurrent/residual tumors and had good local control at last follow-up. One of 5 patients (7/34) who underwent repeat treatment had further treatment for a second recurrence. The total duration of follow-up was 8.6 ± 7.1 years. The average Karnofsky Performance Scale score at last follow-up was 80 (range 40–90). There was 1 death.
Based on this experience and in the absence of guidelines, the authors recommend an individualized approach for the treatment of symptomatic or growing tumors. This study has shown that 1 in 5 patients does not require repeat treatment of their recurrent/residual disease and can be managed with a “scan and watch” approach. On the other hand, 1 in 5 patients who had repeat treatment for their recurrence in the form of surgery and/or radiation will require further additional treatment. More studies are needed to best characterize these patients and predict the natural history of this disease and response to treatment.
Joao Paulo Almeida, Zachary Cappello, Hamid Borghei-Razavi, Pablo F. Recinos, Raj Sindwani and Varun R. Kshettry
Petroclival chondrosarcomas are a formidable surgical challenge given the close relationship to critical neurovascular structures. The endoscopic endonasal approach can be utilized for many petroclival chondrosarcomas. However, tumors that extend to the inferior petrous apex require working behind the internal carotid artery (ICA). We present a case of a 33-year-old with a 1-year history of complete abducens palsy, with imaging showing an enhancing mass centered at the left petroclival fissure and inferior petrous apex behind the paraclival carotid artery and extending down into the nasopharynx abutting the cervical ICA. In this video, we describe the surgical steps of the endoscopic endonasal translacerum approach with ICA skeletonization and mobilization. We also highlight the relevant surgical anatomy with anatomical dissections to supplement the surgical video. The patient did well without complications. Postoperative MRI demonstrated complete resection and pathology revealed grade II chondrosarcoma. He underwent adjuvant proton beam radiotherapy.
The video can be found here: https://youtu.be/80QXALJW9ME.
Mohammed J. Asha, Hirokazu Takami, Carlos Velasquez, Selfy Oswari, Joao Paulo Almeida, Gelareh Zadeh and Fred Gentili
Transsphenoidal surgery is advocated as the first-line management of growth hormone (GH)–secreting adenomas. Although disease control is defined by strict criteria for biochemical remission, the length of follow-up needed is not well defined in literature. In this report, the authors present their long-term remission rate and identify various predictive factors that might influence the clinical outcome.
The authors conducted a single-institute retrospective analysis of all transsphenoidal procedures for GH-secreting adenomas performed from January 2000 to June 2016. The primary outcome was defined as biochemical remission according to the 2010 consensus criteria and measured at the 1-year postoperative mark as well as on the last recorded follow-up appointment.
Secondary variables included recurrence rate, patterns of clinical presentation, and outcome of adjuvant therapy (including repeat surgery). Subgroup analysis was performed for patients who had biochemical or radiological “discordance”—patients who achieved biochemical remission but with incongruent insulin-like growth factor 1 (IGF-1)/GH or residual tumor on MRI. Recurrence-free survival analysis was conducted for patients who achieved remission at 1 year after surgery.
Eighty-one patients (45 female and 36 male) with confirmed acromegaly treated with transsphenoidal surgery were included. In 62 cases the patients were treated with a pure endoscopic approach and in 19 cases an endoscopically assisted microscopic approach was used.
Primary biochemical remission after surgery was achieved in 59 cases (73%) at 1 year after surgery. However, only 41 patients (51%) remained in primary surgical remission (without any adjuvant treatment) at their last follow-up appointment, indicating a recurrence rate of 31% (18 of 59 patients) over the duration of follow-up (mean 100 ± 61 months). Long-term remission rates for pure endoscopic and endoscopically assisted cases were not significantly different (48% vs 52%, p = 0.6). Similarly, no significant difference in long-term remission was detected between primary surgery and repeat surgery (54% vs 33%, p = 0.22).
Long-term remission was significantly influenced by extent of resection, cavernous sinus invasion (radiologically as well as surgically reported), and preoperative and early postoperative GH and IGF-1 levels (within 24–48 hours after surgery) as well as by clinical grade, with lower remission rates in patients with dysmorphic features and/or medical comorbidities (grade 2–3) compared to minimally symptomatic or silent cases (grade 1).
The long-term surgical remission rate appears to be significantly less than “early” remission rates and is highly dependent on the extent of tumor resection. The authors advocate a long-term follow-up regimen and propose a clinical grading system that may aid in predicting long-term outcome in addition to the previously reported anatomical factors. The role of repeat surgery is highlighted.
Jackson A. Gondim, João Paulo Almeida, Lucas Alverne F. de Albuquerque, Erika Gomes, Michele Schops and Jose Italo Mota
With the increase in the average life expectancy, medical care of elderly patients with symptomatic pituitary adenoma (PA) will continue to grow. Little information exists in the literature about the surgical treatment of these patients. The aim of this study was to present the results of a single pituitary center in the surgical treatment of PAs in patients > 70 years of age.
In this retrospective study, 55 consecutive elderly patients (age ≥ 70 years) with nonfunctioning PAs underwent endoscopic transsphenoidal surgery at the General Hospital of Fortaleza, Brazil, between May 2000 and December 2012. The clinical and radiological results in this group were compared with 2 groups of younger patients: < 60 years (n = 289) and 60–69 years old (n = 30).
Fifty-five patients ≥ 70 years of age (average age 72.5 years, range 70–84 years) underwent endoscopic surgery for treatment of PAs. The mean follow-up period was 50 months (range 12–144 months). The most common symptoms were visual impairment in 38 (69%) patients, headache in 16 (29%) patients, and complete ophthalmoplegia in 6 (10.9%). Elderly patients presented a higher incidence of ophthalmoplegia (p = 0.032) and a lower frequency of pituitary apoplexy before surgery (p < 0.05). Tumors with cavernous sinus invasion were treated surgically less frequently than in younger patients. Although patients with an American Society of Anesthesiologists score of 3 were more common in the elderly group (p < 0.05), no significant difference regarding surgical time, extent of resection, and hospitalization were observed. Elderly patients presented with more complications than patients < 60 years (32.7% vs 10%, p < 0.05). Complications observed in the elderly group included 5 CSF leaks (9%), 2 permanent diabetes insipidus cases (3.6%), 4 postoperative refractory hypertension cases (7.2%), 1 myocardial ischemia (1.8%), and 1 death (1.8%). Postoperative new anterior pituitary deficit was more common in the younger group (< 60 years old: 17.7%) than in the elderly (≥ 70 years old: 12.7%); however, there was no statistical difference.
Endoscopic transsphenoidal surgery for elderly patients with PAs may be associated with higher complication rates, especially secondary to early transitory complications, when compared with surgery performed in younger patients. Although the worst preoperative clinical status might be observed in this group, age alone is not associated with a worst final prognosis after endoscopic removal of nonfunctioning PAs.
Joao Paulo Almeida, Dennis Tang, Varun R. Kshettry, Raj Sindwani and Pablo F. Recinos
This is the case of a 25-year-old woman who had had a previous rupture of a dermoid cyst and now presented with recent MRI scans suggesting further growth of her dermoid cyst. Her lesion was located in the suprasellar space and extended into the interpeduncular fossa and prepontine cistern. Considering the location of the tumor, an endoscopic pituitary hemitransposition was selected for its resection. In this video we present the technical nuances and illustrate the anatomy used for an endoscopic endonasal pituitary hemitransposition for resection of a suprasellar dermoid cyst with extension into the interpeduncular fossa. In this case, a near-total resection was achieved, with no complications and no additional hormonal deficit after surgery.
The video can be found here: https://youtu.be/BHtNf5invUI.
Joao Paulo Almeida, Carlos Velásquez, Claire Karekezi, Miguel Marigil, Mojgan Hodaie, James T. Rutka and Mark Bernstein
International collaborations between high-income (HICs) and low- and middle-income countries (LMICs) have been developed as an attempt to reduce the inequalities in surgical care around the world. In this paper the authors review different models for international surgical education and describe projects developed by the Division of Neurosurgery at the University of Toronto in this field.
The authors conducted a review of models of international surgical education reported in the literature in the last 15 years. Previous publications on global neurosurgery reported by the Division of Neurosurgery at the University of Toronto were reviewed to exemplify the applications and challenges of international surgical collaborations.
The most common models for international surgical education and collaboration include international surgical missions, long-term international partnerships, fellowship training models, and online surgical education. Development of such collaborations involves different challenges, including limited time availability, scarce funding/resources, sociocultural barriers, ethical challenges, and lack of organizational support. Of note, evaluation of outcomes of international surgical projects remains limited, and the development and application of assessment tools, such as the recently proposed Framework for the Assessment of International Surgical Success (FAIRNeSS), is encouraged.
Actions to reduce inequality in surgical care should be implemented around the world. Different models can be used for bilateral exchange of knowledge and improvement of surgical care delivery in regions where there is poor access to surgical care. Implementation of global neurosurgery initiatives faces multiple limitations that can be ameliorated if systematic changes occur, such as the development of academic positions in global surgery, careful selection of participant centers, governmental and nongovernmental financial support, and routine application of outcome evaluation for international surgical collaborations.
Jackson A. Gondim, João Paulo Almeida, Lucas Alverne F. de Albuquerque, Erika Gomes, Michele Schops and Tania Ferraz
Acromegaly is a chronic disease related to the excess of growth hormone (GH) and insulin-like growth factor–I secretion, usually by pituitary adenomas. Traditional treatment of acromegaly consists of surgery, drug therapy, and eventually radiotherapy. The introduction of endoscopy as an additional tool for surgical treatment of pituitary adenomas and, therefore, acromegaly represents an important advance of pituitary surgery in the recent years. The aim of this retrospective study is to evaluate the results of pure transsphenoidal endoscopic surgery in a series of patients with acromegaly who were operated on by a pituitary specialist surgeon. The authors discuss the advantages, outcome, complications, and factors related to the success of the endoscopic approach in cases of GHsecreting adenomas.
The authors retrospectively analyzed data from cases involving patients with GH-secreting adenomas who underwent pure transsphenoidal endoscopic surgery at the Department of Neurosurgery of the General Hospital in Fortaleza, Brazil, between 2000 and 2009. Tumors were classified according to size as micro- or macroadenomas, and tumor extension was analyzed based on suprasellar/parasellar extension and sella floor destruction. All patients were followed up for at least 1 year. The criteria of disease control were GH levels < 1 ng/L after oral glucose tolerance test and normal insulin-like growth factor–I levels for age and sex.
During the study period, 67 patients underwent pure endoscopic transsphenoidal surgery for treatment of acromegaly. Disease control was obtained in 50 cases (74.6%). The rate of treatment success was higher in patients with microadenomas (disease control achieved in 12 [85.7%] of 14 cases) than in those with larger lesions. Suprasellar/parasellar extension and high levels of sella floor erosion were associated with lower rates of disease control (p = 0.01 and p = 0.02, respectively). Complications related to the endoscopic surgery included epistaxis (6.0%), transitory diabetes insipidus (4.5%), and 1 case of seizure (1.5%).
Endoscopic transsphenoidal surgery represents an effective option for treatment of patients with acromegaly. High disease control rates and a small number of complications are some of the most important points related to the technique. Factors related to the success of the endoscopic surgery are lesion size, suprasellar/parasellar extension, and the degree of sella floor erosion. Although presenting important advantages, there is no conclusive evidence that endoscopy is superior to microsurgery in treatment of GH-secreting adenomas.
João Paulo Almeida, Armando S. Ruiz-Treviño, Buqing Liang, Sacit B. Omay, Sathwik R. Shetty, Yu-Ning Chen, Vijay K. Anand, Kartikey Grover, Paul Christos and Theodore H. Schwartz
Surgery is generally the first-line therapy for acromegaly. For patients with residual or recurrent tumors, several treatment options exist, including repeat surgery, medical therapy, and radiation. Reoperation for recurrent acromegaly has been associated with poor results, with hormonal control usually achieved in fewer than 50% of cases. Extended endonasal endoscopic approaches (EEAs) may potentially improve the results of reoperation for acromegaly by providing increased visibility and maneuverability in parasellar areas.
A database of all patients treated in the authors’ center between July 2004 and February 2016 was reviewed. Cases involving patients with acromegaly secondary to growth hormone (GH)–secreting adenomas who underwent EEA were selected for chart review and divided into 2 groups: first-time surgery and reoperation. Disease control was defined by 2010 guidelines. Clinical and radiological characteristics and outcome data were extracted. A systematic review was done through a MEDLINE database search (2000–2016) to identify studies on the surgical treatment of acromegaly. Using PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses) guidelines, the included studies were reviewed for surgical approach, tumor size, cavernous sinus invasion, disease control, and complications. Cases were divided into reoperation or first-time surgery for comparative analysis.
A total of 44 patients from the authors’ institution were included in this study. Of these patients, 2 underwent both first-time surgery and reoperation during the study period and were therefore included in both groups. Thus data from 46 surgical cases were analyzed (35 first-time operations and 11 reoperations). The mean length of follow-up was 70 months (range 6–150 months). The mean size of the reoperated tumors was 14.8 ± 10.0 mm (5 micro- and 6 macroadenomas). The patients’ mean age at the time of surgery was younger in the reoperation group than in the first-time surgery group (34.3 ± 12.8 years vs 49.1 ± 15.7 years, p = 0.007) and the mean preoperative GH level was also lower (7.7 ± 13.1 μg/L vs 25.6 ± 36.8 μg/L, p = 0.04). There was no statistically significant difference in disease control rates between the reoperation (7 [63.6%] of 11) and first-time surgery (25 [71.4%] of 33) groups (p = 0.71). Univariate analysis showed that older age, smaller tumor size, lower preoperative GH level, lower preoperative IGF-I level, and absence of cavernous sinus invasion were associated with higher chances of disease control in the first-time surgery group, whereas only absence of cavernous sinus invasion was associated with disease control in the reoperation group (p = 0.01). There was 1 case (9%) of transient diabetes insipidus and hypogonadism and 1 (9%) postoperative nasal infection after reoperation. The systematic review retrieved 29 papers with 161 reoperation and 2189 first-time surgery cases. Overall disease control for reoperation was 46.8% (95% CI 20%–74%) versus 56.4% (95% CI 49%–63%) for first-time operation. Reoperation and first-time surgery had similar control rates for microadenomas (73.6% [95% CI 32%–98%] vs 77.6% [95% CI 68%–85%]); however, reoperation was associated with substantially lower control rates for macroadenomas (27.5% [95% CI 5%–57%] vs 54.3% [95% CI 45%–62%]) and tumors invading the cavernous sinus (14.7% [95% CI 4%–29%] vs 38.5% [95% CI 27%–50%]).
Reoperative EEA for acromegaly had results similar to those for first-time surgery and rates of control for macroadenomas that were better than historical rates. Cavernous sinus invasion continues to be a negative prognostic indicator for disease control; however, results with EEA show improvement compared with results reported in the prior literature.
Sacit Bulent Omay, João Paulo Almeida, Yu-Ning Chen, Sathwik R. Shetty, Buqing Liang, Shilei Ni, Vijay K. Anand and Theodore H. Schwartz
Craniopharyngiomas arise from the pituitary stalk, and in adults they are generally located posterior to the chiasm extending up into the third ventricle. The extended endonasal approach (EEA) can provide an ideal corridor between the bottom of the optic chiasm and the top of the pituitary gland (chiasm-pituitary corridor [CPC]) for their removal. A narrow CPC in patients with a prefixed chiasm and a large tumor extending up and behind the chiasm has been considered a contraindication to EEA, with a high risk of visual deterioration and subtotal resection.
A database of all patients treated in the authors’ center (Weill Cornell Medical College, NewYork-Presbyterian Hospital) between July 2004 and August 2016 was reviewed. Patients with craniopharyngiomas who underwent EEA with the goal of gross-total resection (GTR) were included in the study. Patients with postfixed chiasm or limited available preoperative imaging were excluded. Using preoperative contrast-enhanced T1-weighted sagittal midline MR images, the authors calculated the CPC as well as the distance from the chiasm to the top of the tumor (CTOT). From these numbers, they calculated a ratio of the CPC to the CTOT as a measure of difficulty in removing the tumors through the EEA and called this ratio the corridor index (CI). The relationship between the CI and the ability to achieve GTR and visual outcome were measured.
Thirty-four patients were included in the study. The mean CPC was 10.1 mm (range 5.2–19.1 mm). The mean CTOT was 12.8 mm (range 0–28.3 mm). The median CI was 0.8; the CI ranged from 0.4 to infinity (for tumors with a CTOT of 0). Thirty-two patients had GTR (94.1%) and 2 had subtotal resection. The CPC value had no relationship with our ability to achieve GTR and no effect on visual or endocrine outcome.
EEA for craniopharyngioma is generally considered the first-line surgical approach. Although a narrow corridor between the top of the pituitary gland and the bottom of the chiasm may seem to be a relative contraindication to surgery for larger tumors, the authors’ data do not bear this out. EEA appears to be a successful technique for the majority of midline craniopharyngiomas.