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Ernest Joseph Barthélemy, Christopher A. Sarkiss, James Lee and Raj K. Shrivastava

The historical origin of the meningioma nomenclature unravels interesting social and political aspects about the development of neurosurgery in the late 19th century. The meningioma terminology itself was the subject of nationalistic pride and coincided with the advancement in the rise of medicine in Continental Europe as a professional social enterprise. Progress in naming and understanding these types of tumor was most evident in the nations that successively assumed global leadership in medicine and biomedical science throughout the 19th and 20th centuries, that is, France, Germany, and the United States. In this vignette, the authors delineate the uniqueness of the term “meningioma” as it developed within the historical framework of Continental European concepts of tumor genesis, disease states, and neurosurgery as an emerging discipline culminating in Cushing's Meningiomas text.

During the intellectual apogee of the French Enlightenment, Antoine Louis published the first known scientific treatise on meningiomas. Like his father, Jean-Baptiste Louis, Antoine Louis was a renowned military surgeon whose accomplishments were honored with an admission to the Académie royale de chirurgie in 1749. His treatise, Sur les tumeurs fongueuses de la duremère, appeared in 1774. Following this era, growing economic depression affecting a frustrated bourgeoisie triggered a tumultuous revolutionary period that destroyed France's Ancien Régime and abolished its university and medical systems. The resulting anarchy was eventually quelled through legislation aiming to satisfy Napoleon's need for qualified military professionals, including physicians and surgeons. These laws laid the foundations for the subsequent flourishing of French medicine throughout the mid-19th century. Subsequent changes to the meningioma nomenclature were authored by intellectual giants of this postrevolutionary period, for example, by the Limogesborn pathologist Jean Cruveilhier known for the term “tumeurs cancéreuses de la duremère,” and the work of histopathologists, such as Hermann Lebert, who were influenced by Pasteur's germ theory and by Bernard's experimental medicine.

The final development of the meningioma nomenclature corresponded to the rise of American neurosurgery as a formal academic discipline. This historical period of growth is chronicled in Cushing's text Meningiomas, and it set the scientific stage for the modern developments in meningioma research and surgery that are conducted and employed today.

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Raj K. Shrivastava, Chandranath Sen, Peter D. Costantino and Robert Della Rocca

Object

Sphenoorbital meningiomas (SOMs) are complex tumors involving the sphenoid wing, orbit, and cavernous sinus, which makes their complete resection difficult or impossible. Sphenoidal hyperostosis that results in incomplete resection makes these tumors prone to high rates of recurrence with postoperative morbidity resulting in a nonfunctional globe. High-dose radiation therapy has often been described as the only treatment capable of achieving tumor control, although often at the expense of the patient's progressive visual deterioration.

Methods

This series consisted of 25 patients who were retrospectively analyzed over a 12-year period. Visual function was evaluated pre- and postoperatively in all patients. A standardized surgical approach to a frontotemporal craniotomy and orbitozygomatic osteotomy with intra- and extradural drilling of the optic canal and all the hyperostotic bone was performed. Orbital and cranial reconstruction was performed in all patients. The follow-up period was 6 months to 12 years (average 5 years).

The patients presented with the classic triad of SOM: proptosis (86%), visual impairment (78%), and ocular paresis (20%). A gross-total resection was achieved in 70% of patients with surgery limited by the superior orbital fissure and the cavernous sinus. Proptosis improved in 96% of patients with 87% improvement in visual function. Ocular paresis improved in 68%, although 20% of patients experienced a temporary ocular paresis postoperatively. There were no perioperative deaths or morbidity related to the surgical approach or reconstruction. Ninety-five percent of patients reported an improved functional orbit. There was tumor recurrence in 8% of patients; in one case recurrence was delayed for longer than 11 years.

Conclusions

Sphenoorbital meningiomas are a distinct category of tumors complicated by potentially extensive hyperostosis of the skull base. Successful resection requires extensive intra- and extradural surgery, necessitating drilling of the optic canal and an orbital osteotomy within anatomical limitations. The bone resection requires reconstruction with autograft, allografts, or alloplast for improved orbital function. All aspects of the clinical triad improved. A radical resection can be achieved with low morbidity, providing a significantly improved clinical outcome in the long-term period.

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Raj K. Shrivastava, Marc S. Arginteanu, Wesley A. King and Kalmon D. Post

Object. Giant prolactinomas are rare tumors whose treatment and outcome has only been addressed in isolated case reports. The authors document the long-term follow-up findings and clinical outcome in a group of patients with giant prolactinomas.

Methods. This study is a retrospective chart and clinical review of more than 2000 cases of pituitary tumors treated at the authors' institution, of which 10 met the criteria for inclusion (prolactin level > 1000 ng/ml, diameter > 4 cm on neuroimaging studies, and clinical signs of hyperprolactinemia/mass effect). The average follow-up duration was 6.7 years after initial treatment with either bromocriptine or transsphenoidal resection. In more than 90% of the patients in this series the disease was controlled by medical treatment with bromocriptine alone; the other 10% required early surgery via transsphenoidal resection. All patients had improvement in visual symptoms. All tumors had extrasellar components, five of which exhibited frank invasion of the cavernous sinus. Tumor volume on magnetic resonance imaging was decreased on average by 69%; this occurred at a faster rate and in larger amounts when treated with bromocriptine than has been reported in the literature for macroprolactinomas.

Conclusions. According to long-term follow-up findings, giant prolactinomas are exquisitely responsive to dopamine agonist therapy. In giant prolactinomas the prolactin level does not correlate with size. The main indication for early surgery is intratumoral hematoma, whereas our main indications for late surgery are cerebrospinal fluid leakage caused by medical treatment, or an increasing prolactin level despite medical therapy. Checking prolactin levels in suspicious sellar and/or suprasellar lesions may be diagnostic and prevent unnecessary surgery.

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Raj K. Shrivastava, Salomao Segal, Martin B. Camins, Chandranath Sen and Kalmon D. Post

✓ The search for the origin of the commonly held principle in current neurosurgery regarding the resectability of the anterior one third of the superior sagittal sinus unravels the many fascinating developments that occurred in neurosurgery during the early 20th century. All these occurrences can be traced back to, and are uniquely contextualized in, Harvey Cushing's seminal text, Meningiomas, Their Classification, Regional Behaviour, Life History, and Surgical End Results. Written with Louise Eisenhardt and published in 1938, Meningiomas is a monograph of incredible description and detail. The meticulous categorization of meningiomas, their presentation, clinical outcome, and surgical therapies are even further supplemented by Cushing's personal commentary, questions, and recollections. Cushing's genius was evident in his ability not only to make insightful clinical observations, but also to synthesize these ideas within the neurosurgical context of his era. As he says in Meningiomas, “Thus the pathological curiosity of one day becomes in its proper time a commonplace … most of which are one and the same disorder—had, for their interpretation, to await the advent of the Neurosurgeon.”

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Chandranath Sen, Aymara I. Triana, Niklas Berglind, James Godbold and Raj K. Shrivastava

Object

Chordomas are rare malignant neoplasms arising predominantly at the sacrum and skull base. They are uniformly lethal unless treated with aggressive resection and proton beam irradiation. The authors present results of the surgical management of a large number of patients with clivus chordomas. Factors that influence the surgeon's ability to achieve radical tumor resection are also evaluated.

Methods

Between 1991 and 2005, 71 patients with clivus chordomas underwent surgery. The average follow-up was 66 months (median 60 months, range 3–189 months). Sixty-five patients had complete records that were analyzed in the present report. Thirty-five percent of them had undergone surgery before being treated by the authors. They were evaluated with MR imaging and CT scanning and underwent surgery utilizing a variety of skull base techniques aimed at achieving radical excision. Many also underwent postoperative radiation, usually in the form of proton beam therapy. The patients were followed up with serial imaging at regular intervals as well as with neurological evaluation.

Results

Radical tumor resection was achieved in 58% of the group. The overall 5-year survival rate was 75%. Radical resection had a positive impact on survival. The ability to achieve radical resection was dependent on the preoperative tumor volume and the number of anatomical areas involved by the tumor. Cranial nerve impairment and CSF leakage were the most frequent postoperative complications.

Conclusions

Radical excision is the ideal surgical goal in the treatment of clival chordomas and can be achieved with reasonable risks. Several different surgical approaches may be necessary to accomplish this.

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Karan M. Kohli, Joshua Loewenstern, Remi A. Kessler, Margaret Pain, Christina A. Palmese, Joshua Bederson and Raj K. Shrivastava

OBJECTIVE

With increasing general use of antidepressants (ADs), multiple studies have noted a small protective effect of ADs for patients with glioma, but their impact on meningioma has not been established. This study aims to evaluate the role of ADs in the context of additional clinical factors in relation to long-term risk of meningioma recurrence.

METHODS

One hundred five patients with an intracranial meningioma presenting from 2011–2014 with at least 3 years of follow-up (median 4.2 years) after resection were reviewed. AD use along with demographics, tumor characteristics, and outcomes were recorded. Multivariate logistic regression was used to analyze the association of AD use with tumor recurrence, including other clinical measures significantly associated with recurrence as covariates.

RESULTS

Twenty-nine patients (27.4%) were taking ADs (27 selective serotonin reuptake inhibitors, 2 norepinephrine-dopamine reuptake inhibitors) prior to tumor recurrence. Their tumors largely affected the frontal (31.0%) or parietal lobe (17.2%) and were located in convexity, parasagittal, or falcine (CPF) areas more frequently than skull base areas relative to the tumors of non-AD users (p = 0.035). AD use was found to be an independent predictor of recurrence, in addition to subtotal resection and WHO grade II/III classification (p values < 0.05). The median time from AD prescription to tumor recurrence was 36.6 months (interquartile range [IQR] = 20.9–62.9 months) and median length of AD use was 41.4 months (IQR = 24.7–62.8 months).

CONCLUSIONS

AD use was an independent predictor of meningioma recurrence. This association may be due to mood or affective changes caused by tumor location in CPF regions that may be a sign of early recurrence. The finding calls attention to AD use in the management of patients with meningioma, and warrants further exploration of an underlying relationship.

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Raj K. Shrivastava, Fred J. Epstein, Noel I. Perin, Kalmon D. Post and George I. Jallo

Object. Intramedullary spinal cord tumors (IMSCTs) in the older-age adult population pose complex management issues regarding the extent of resection and functional outcome, especially in terms of quality of life. Historically, IMSCTs in the older adult population were treated with irradiation alone because it was assumed that functional recovery would be poor. The authors examined their IMSCT database and report the first large series of IMSCTs in patients older than 50 years of age.

Methods. In this retrospective clinical and chart review there were 30 cases meeting inclusion criteria drawn from databases at three different institutions. A modified McCormick Scale was used to assess functional levels in all 30 patients pre- and postoperatively. The mean age of patients in this cohort was 59.8 years (range 50–78 years), and the mean follow- up period was 10.6 years (range 2–16 years).

Ependymoma was the most common tumor (83%), and 55% were located in the thoracic spine. The most common presenting symptom was sensory dysesthesia, with rare motor loss. The prodromal period to treatment was 19.4 months. Based on the McCormick Scale score at last follow-up examination 67% of patients were clinically functionally the same, 9% were worse, and 24% were improved after surgery. There were two deaths due tumor progression (both malignant tumors) and one recurrence (anaplastic astrocytoma). All three patients in whom malignant astrocytomas were diagnosed underwent postoperative radiation therapy.

Conclusions. In the population of patients older than age 50 years, thoracic ependymomas are the most common IMSCTs that present characteristically with sensory symptoms. The longer prodromal period in the older adult population may reflect the fact that their diagnosis and workup is inadequate. There was no significant increase in the length of stay in the neurosurgical ward. The authors recommend motor evoked potential-guided aggressive microsurgical resection, because the long-term outcome of benign lesions is excellent (good functional recovery and no tumor recurrence).

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Thomas F. Barrett, Corey M. Gill, Brett A. Miles, Alfred M. C. Iloreta, Richard L. Bakst, Mary Fowkes, Priscilla K. Brastianos, Joshua B. Bederson and Raj K. Shrivastava

Squamous cell carcinoma of the head and neck (HNSCC) affects nearly 500,000 individuals globally each year. With the rise of human papillomavirus (HPV) in the general population, clinicians are seeing a concomitant rise in HPV-related HNSCC. Notably, a hallmark of HPV-related HNSCC is a predilection for unique biological and clinical features, which portend a tendency for hematogenous metastasis to distant locations, such as the brain. Despite the classic belief that HNSCC is restricted to local spread via passive lymphatic drainage, brain metastases (BMs) are a rare complication that occurs in less than 1% of all HNSCC cases. Time between initial diagnosis of HNSCC and BM development can vary considerably. Some patients experience more than a decade of disease-free survival, whereas others present with definitive neurological symptoms that precede primary tumor detection. The authors systematically review the current literature on HNSCC BMs and discuss the current understanding of the effect of HPV status on the risk of developing BMs in the modern genomic era.

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John W. Rutland, Javin Schefflein, Annie E. Arrighi-Allisan, Daniel Ranti, Travis R. Ladner, Akila Pai, Joshua Loewenstern, Hung-Mo Lin, James Chelnis, Bradley N. Delman, Raj K. Shrivastava and Priti Balchandani

OBJECTIVE

Predicting vision recovery following surgical decompression of the optic chiasm in pituitary adenoma patients remains a clinical challenge, as there is significant variability in postoperative visual function that remains unreliably explained by current prognostic factors. Available literature inadequately characterizes alterations in adenoma patients involving the lateral geniculate nucleus (LGN). This study examined the association of LGN degeneration with chiasmatic compression as well as with the retinal nerve fiber layer (RNFL), pattern standard deviation (PSD), mean deviation (MD), and postoperative vision recovery. PSD is the degree of difference between the measured visual field pattern and the normal pattern (“hill”) of vision, and MD is the average of the difference from the age-adjusted normal value.

METHODS

A prospective study of 27 pituitary adenoma patients and 27 matched healthy controls was conducted. Participants were scanned on a 7T ultra–high field MRI scanner, and 3 independent readers measured the LGN at its maximum cross-sectional area on coronal T1-weighted MPRAGE imaging. Readers were blinded to diagnosis and to each other’s measurements. Neuro-ophthalmological data, including RNFL thickness, MD, and PSD, were acquired for 12 patients, and postoperative visual function data were collected on patients who underwent surgical chiasmal decompression. LGN areas were compared using two-tailed t-tests.

RESULTS

The average LGN cross-sectional area of adenoma patients was significantly smaller than that of controls (13.8 vs 19.2 mm2, p < 0.0001). The average LGN cross-sectional area correlated with MD (r = 0.67, p = 0.04), PSD (r = −0.62, p = 0.02), and RNFL thickness (r = 0.75, p = 0.02). The LGN cross-sectional area in adenoma patients with chiasm compression was 26.6% smaller than in patients without compression (p = 0.009). The average tumor volume was 7902.7 mm3. Patients with preoperative vision impairment showed 29.4% smaller LGN cross-sectional areas than patients without deficits (p = 0.003). Patients who experienced improved postoperative vision had LGN cross-sectional areas that were 40.8% larger than those of patients without postoperative improvement (p = 0.007).

CONCLUSIONS

The authors demonstrate novel in vivo evidence of LGN volume loss in pituitary adenoma patients and correlate imaging results with neuro-ophthalmology findings and postoperative vision recovery. Morphometric changes to the LGN may reflect anterograde transsynaptic degeneration. These findings indicate that LGN degeneration may be a marker of optic apparatus injury from chiasm compression, and measurement of LGN volume loss may be useful in predicting vision recovery following adenoma resection.