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Salomon Cohen-Cohen, Giuseppe Lanzino, and Leonardo Rangel-Castilla

The extended retrosigmoid approach provides an excellent corridor to the lateral aspect of the pontomedullary junction (PMJ)., This video demonstrates a microsurgical resection of a progressive enlarging cavernous malformation (CM) of the PMJ. The patient is a 33-year-old woman with progressive symptoms, including right facial droop, left hemianesthesia, diplopia, and nystagmus. The patient underwent a right extended retrosigmoid approach with intraoperative neuronavigation and neuromonitoring. Lower cranial nerve dissection allowed access to the lateral PMJ. A longitudinal corticotomy was performed above the glossopharyngeal. The CM was removed in a piecemeal fashion. Postoperative MRI confirmed gross-total resection and the patient remained neurologically stable.

The video can be found here: https://youtu.be/K_TtiTo1RsQ.

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Salomon Cohen-Cohen, Paul A. Gardner, Joao T. Alves-Belo, Huy Q. Truong, Carl H. Snyderman, Eric W. Wang, and Juan C. Fernandez-Miranda

OBJECTIVE

Pituitary adenomas often invade the medial wall of the cavernous sinus (CS), but this structure is generally not surgically removed because of the risk of vascular and cranial nerve injury. The purpose of this study was to report the surgical outcomes in a large series of cases of invasive pituitary adenoma in which the medial wall of the CS was selectively removed following an anatomically based, stepwise surgical technique.

METHODS

The authors’ institutional database was reviewed to identify cases of pituitary adenoma with isolated invasion of the medial wall, based on an intraoperative evaluation, in which patients underwent an endoscopic endonasal approach with selective resection of the medial wall of the CS. Cases with CS invasion beyond the medial wall were excluded. Patient complications, resection, and remission rates were assessed.

RESULTS

Fifty patients were eligible for this study, 15 (30%) with nonfunctional adenomas and 35 (70%) with functional adenomas, including 16 growth hormone–, 10 prolactin-, and 9 adrenocorticotropic hormone (ACTH)–secreting tumors. The average tumor size was 2.3 cm for nonfunctional and 1.3 cm for functional adenomas. Radiographically, 11 cases (22%) were Knosp grade 1, 23 (46%) Knosp grade 2, and 16 (32%) Knosp grade 3. Complete tumor resection, based on intraoperative impression and postoperative MRI, was achieved in all cases. The mean follow-up was 30 months (range 4–64 months) for patients with functional adenomas and 16 months (range 4–30 months) for those with nonfunctional adenomas. At last follow-up, complete biochemical remission (using current criteria) without adjuvant treatment was seen in 34 cases (97%) of functional adenoma. No imaging recurrences were seen in patients who had nonfunctional adenomas. A total of 57 medial walls were removed in 50 patients. Medial wall invasion was histologically confirmed in 93% of nonfunctional adenomas and 83% of functional adenomas. There were no deaths or internal carotid artery injuries, and the average blood loss was 378 ml. Four patients (8%) developed a new, transient cranial nerve palsy, and 2 of these patients required reoperation for blood clot evacuation and fat graft removal. There were no permanent cranial nerve palsies.

CONCLUSIONS

The medial wall of the CS can be removed safely and effectively, with minimal morbidity and excellent resection and remission rates. Further follow-up is needed to determine the long-term results of this anatomically based technique, which should only be performed by very experienced endonasal skull base teams.

Restricted access

Salomon Cohen-Cohen, Ahmed Helal, Ziying Yin, Matthew K. Ball, Richard L. Ehman, Jamie J. Van Gompel, and John Huston III

OBJECTIVE

Pituitary adenoma is one of the most common primary intracranial neoplasms. Most of these tumors are soft, but up to 17% may have a firmer consistency. Therefore, knowing the tumor consistency in the preoperative setting could be helpful. Multiple imaging methods have been proposed to predict tumor consistency, but the results are controversial. This study aimed to evaluate the efficacy of MR elastography (MRE) in predicting tumor consistency and its potential use in a series of patients with pituitary adenomas.

METHODS

Thirty-eight patients with pituitary adenomas (≥ 2.5 cm) were prospectively evaluated with MRI and MRE before surgery. Absolute MRE stiffness values and relative MRE stiffness ratios, as well as the relative ratio of T1 signal, T2 signal, and diffusion-weighted imaging apparent diffusion coefficient (ADC) values were determined prospectively by calculating the ratio of those values in the tumor to adjacent left temporal white matter. Tumors were classified into three groups according to surgical consistency (soft, intermediate, and firm). Statistical analysis was used to identify the predictive value of the different radiological parameters in determining pituitary adenoma consistency.

RESULTS

The authors included 32 (84.21%) nonfunctional and 6 (15.79%) functional adenomas. The mean maximum tumor diameter was 3.7 cm, and the mean preoperative tumor volume was 16.4 cm3. Cavernous sinus invasion was present in 20 patients (52.63%). A gross-total resection was possible in 9 (23.68%) patients. The entire cohort’s mean absolute tumor stiffness value was 1.8 kPa (range 1.1–3.7 kPa), whereas the mean tumor stiffness ratio was 0.66 (range 0.37–1.6). Intraoperative tumor consistency was significantly correlated with absolute and relative tumor stiffness (p = 0.0087 and 0.007, respectively). Tumor consistency alone was not a significant factor for predicting gross-total resection. Patients with intermediate and firm tumors had more complications compared to patients with soft tumors (50.00% vs 12.50%, p = 0.02) and also had longer operative times (p = 0.0002).

CONCLUSIONS

Whereas other MRI sequences have proven to be unreliable in determining tumor consistency, MRE has been shown to be a reliable tool for predicting adenoma consistency. Preoperative knowledge of tumor consistency could be potentially useful for surgical planning, counseling about potential surgical risks, and estimating the length of operative time.

Free access

Salomon Cohen-Cohen, Desmond A. Brown, Benjamin T. Himes, Lydia P. Wheeler, Michael W. Ruff, Brittny T. Major, Naykky M. Singh Ospina, John L. D. Atkinson, Fredric B. Meyer, Irina Bancos, William F. Young Jr., and Jamie J. Van Gompel

OBJECTIVE

Multiple endocrine neoplasia type 1 (MEN1) is a rare, autosomal-dominant tumor disorder characterized by the development of pituitary tumors and other endocrine neoplasms. Diagnosis is made clinically based on the development of 2 or more canonical lesions (parathyroid gland, anterior pituitary, and enteropancreatic tumors) or in family members of a patient with a clinical diagnosis of MEN1 and the occurrence of one of the MEN1-associated tumors. The goal of this study was to characterize pituitary tumors arising in the setting of MEN1 at a single institution. The probability of tumor progression and the likelihood of surgical intervention in patients with asymptomatic nonfunctional pituitary adenomas were also analyzed.

METHODS

A retrospective review of a prospectively maintained institutional database was performed for patients with MEN1 diagnosed from 1970 to 2017. Data included patient demographics, tumor characteristics, treatment strategies, and outcomes.

RESULTS

A review of the database identified 268 patients diagnosed with MEN1, of whom 158 (59%) were female. Among the 268 patients, 139 (51.8%) had pituitary adenomas. There was a higher prevalence in women than in men (65% vs 35%, p < 0.005). Functional adenomas (57%) were more common. Prolactin-secreting adenomas were the most common functional tumors. Macroadenomas were seen in 27% of patients and were more likely to be symptomatic and locally aggressive (p < 0.001). Forty-nine patients (35%) underwent transsphenoidal resection at some point during their disease course. In 52 patients who were initially observed with MEN1 asymptomatic nonfunctional adenomas, only 5 (10%) progressed to need surgery. In MEN1 patients, an initial parathyroid lesion is most likely followed in order by pituitary, pancreatic, adrenal, and, finally, rare carcinoid tumors.

CONCLUSIONS

Asymptomatic nonfunctional pituitary adenomas in patients with MEN1 may be followed safely with MRI. In this series, parathyroid tumors developed at the lowest median age of all cardinal tumors, and development of additional cardinal MEN1 lesions followed a predictable pattern. This pattern of disease progression could have significant implications for disease surveillance in clinical practice and may help to target clinical resources to the lesions most likely to develop next. This may aid with early detection and treatment and warrants further study.

Free access

Salomon Cohen-Cohen, Desmond A. Brown, Benjamin T. Himes, Lydia P. Wheeler, Michael W. Ruff, Brittny T. Major, Naykky M. Singh Ospina, John L. D. Atkinson, Fredric B. Meyer, Irina Bancos, William F. Young Jr., and Jamie J. Van Gompel

OBJECTIVE

Multiple endocrine neoplasia type 1 (MEN1) is a rare, autosomal-dominant tumor disorder characterized by the development of pituitary tumors and other endocrine neoplasms. Diagnosis is made clinically based on the development of 2 or more canonical lesions (parathyroid gland, anterior pituitary, and enteropancreatic tumors) or in family members of a patient with a clinical diagnosis of MEN1 and the occurrence of one of the MEN1-associated tumors. The goal of this study was to characterize pituitary tumors arising in the setting of MEN1 at a single institution. The probability of tumor progression and the likelihood of surgical intervention in patients with asymptomatic nonfunctional pituitary adenomas were also analyzed.

METHODS

A retrospective review of a prospectively maintained institutional database was performed for patients with MEN1 diagnosed from 1970 to 2017. Data included patient demographics, tumor characteristics, treatment strategies, and outcomes.

RESULTS

A review of the database identified 268 patients diagnosed with MEN1, of whom 158 (59%) were female. Among the 268 patients, 139 (51.8%) had pituitary adenomas. There was a higher prevalence in women than in men (65% vs 35%, p < 0.005). Functional adenomas (57%) were more common. Prolactin-secreting adenomas were the most common functional tumors. Macroadenomas were seen in 27% of patients and were more likely to be symptomatic and locally aggressive (p < 0.001). Forty-nine patients (35%) underwent transsphenoidal resection at some point during their disease course. In 52 patients who were initially observed with MEN1 asymptomatic nonfunctional adenomas, only 5 (10%) progressed to need surgery. In MEN1 patients, an initial parathyroid lesion is most likely followed in order by pituitary, pancreatic, adrenal, and, finally, rare carcinoid tumors.

CONCLUSIONS

Asymptomatic nonfunctional pituitary adenomas in patients with MEN1 may be followed safely with MRI. In this series, parathyroid tumors developed at the lowest median age of all cardinal tumors, and development of additional cardinal MEN1 lesions followed a predictable pattern. This pattern of disease progression could have significant implications for disease surveillance in clinical practice and may help to target clinical resources to the lesions most likely to develop next. This may aid with early detection and treatment and warrants further study.

Full access

Sivashanmugam Dhandapani, Harminder Singh, Hazem M. Negm, Salomon Cohen, Mark M. Souweidane, Jeffrey P. Greenfield, Vijay K. Anand, and Theodore H. Schwartz

OBJECTIVE

Craniopharyngiomas can be difficult to remove completely based on their intimate relationship with surrounding visual and endocrine structures. Reoperations are not uncommon but have been associated with higher rates of complications and lower extents of resection. So radiation is often offered as an alternative to reoperation. The endonasal endoscopic transsphenoidal approach has been used in recent years for craniopharyngiomas previously removed with craniotomy. The impact of this approach on reoperations has not been widely investigated.

METHODS

The authors reviewed a prospectively acquired database of endonasal endoscopic resections of craniopharyngiomas over 11 years at Weill Cornell Medical College, NewYork-Presbyterian Hospital, performed by the senior authors. Reoperations were separated from first operations. Pre- and postoperative visual and endocrine function, tumor size, body mass index (BMI), quality of life (QOL), extent of resection (EOR), impact of prior radiation, and complications were compared between groups. EOR was divided into gross-total resection (GTR, 100%), near-total resection (NTR, > 95%), and subtotal resection (STR, < 95%). Univariate and multivariate analyses were performed.

RESULTS

Of the total 57 endonasal surgical procedures, 22 (39%) were reoperations. First-time operations and reoperations did not differ in tumor volume, radiological configuration, or patients' BMI. Hypopituitarism and diabetes insipidus (DI) were more common before reoperations (82% and 55%, respectively) compared with first operations (60% and 8.6%, respectively; p < 0.001). For the 46 patients in whom GTR was intended, rates of GTR and GTR+NTR were not significantly different between first operations (90% and 97%, respectively) and reoperations (80% and 100%, respectively). For reoperations, prior radiation and larger tumor volume had lower rates of GTR. Vision improved equally in first operations (80%) compared with reoperations (73%). New anterior pituitary deficits were more common in first operations compared with reoperations (51% vs 23%, respectively; p = 0.08), while new DI was more common in reoperations compared with first-time operations (80% vs 47%, respectively; p = 0.08). Nonendocrine complications occurred in 2 (3.6%) first-time operations and no reoperations. Tumor regrowth occurred in 6 patients (11%) over a median follow-up of 46 months and was not different between first versus reoperations, but was associated with STR (33%) compared with GTR+NTR (4%; p = 0.02) and with not receiving radiation after STR (67% vs 22%; p = 0.08). The overall BMI increased significantly from 28.7 to 34.8 kg/m2 over 10 years. Six months after surgery, there was a significant improvement in QOL, which was similar between first-time operations and reoperations, and negatively correlated with STR.

CONCLUSIONS

Endonasal endoscopic transsphenoidal reoperation results in similar EOR, visual outcome, and improvement in QOL as first-time operations, with no significant increase in complications. EOR is more impacted by tumor volume and prior radiation. Reoperations should be offered to patients with recurrent craniopharyngiomas and may be preferable to radiation in patients in whom GTR or NTR can be achieved.

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Hazem M. Negm, Rafid Al-Mahfoudh, Manish Pai, Harminder Singh, Salomon Cohen, Sivashanmugam Dhandapani, Vijay K. Anand, and Theodore H. Schwartz

OBJECTIVE

Regrowth of the lesion after surgical removal of pituitary adenomas is uncommon unless subtotal resection was originally achieved in the first surgery. Treatment for recurrent tumor can involve surgery or radiotherapy. Locations of residual tumor may vary based on the original approach. The authors evaluated the specific sites of residual or recurrent tumor after different transsphenoidal approaches and describe the surgical outcome of endoscopic endonasal transsphenoidal reoperation.

METHODS

The authors analyzed a prospectively collected database of a consecutive series of patients who had undergone endoscopic endonasal surgeries for residual or recurrent pituitary adenomas after an original transsphenoidal microscopic or endoscopic surgery. The site of the recurrent tumor and outcome after reoperation were noted and correlated with the primary surgical approach. The chi-square or Fisher exact test was used to compare categorical variables, and the Mann-Whitney U-test was used to compare continuous variables between surgical groups.

RESULTS

Forty-one patients underwent surgery for residual/recurrent pituitary adenoma from 2004 to 2015 at Weill Cornell Medical College. The previous treatment was a transsphenoidal microscopic (n = 22) and endoscopic endonasal (n = 19) surgery. In 83.3% patients (n = 30/36) there was postoperative residual tumor after the initial surgery. A residual tumor following endonasal endoscopic surgery was less common in the sphenoid sinus (10.5%; 2/19) than it was after microscopic transsphenoidal surgery (72.7%; n =16/22; p = 0.004). Gross-total resection (GTR) was achieved in 58.5%, and either GTR or near-total resection was achieved in 92.7%. Across all cases, the average extent of resection was 93.7%. The rate of GTR was lower in patients with Knosp-Steiner Grade 3–4 invasion (p < 0.0005). Postoperative CSF leak was seen in only one case (2.4%), which stopped with lumbar drainage. Visual fields improved in 52.9% (n = 9/17) of patients and were stable in 47% (n = 8/17). Endocrine remission was achieved in 77.8% (n = 14/18) of cases, 12 by surgery alone and 2 by adjuvant medical (n = 1) and radiation (n = 1) therapy. New diabetes insipidus occurred in 4.9% (n = 2/41) of patients—in one of whom an additional single anterior hormonal axis was compromised—and 9.7% (n = 4/41) of patients had a new anterior pituitary hormonal insufficiency.

CONCLUSIONS

Endonasal endoscopic reoperation is extremely effective at removing recurrent or residual pituitary adenomas that remain after a prior surgery, and it may be preferable to radiation therapy particularly in symptomatic patients. Achievement of GTR is less common when lateral cavernous sinus invasion is present. The locations of residual/recurrent tumor were more likely sphenoidal and parasellar following a prior microscopic transsphenoidal surgery and sellar following a prior endonasal endoscopic surgery.