The History of Arachne Through Historic Descriptions of Meningiomas with Hyperostosis: From Prehistory to the Present.

BACKGROUND: Intracranial meningiomas are brain tumors that have probably been known the longest, largely because of the occasional production of grotesque cranial deformities that have attracted the attention and interest of humankind. Because of the tendency of some intracranial meningiomas to cause skull deformation and thickening, these tumors have given rise to various speculations and theories related to their origin, starting in prehistoric times up to the present. METHODS: From the Steinheim skull and "pharaonic meningiomas" to the first meningioma monograph and the first explanations of Harvey Cushing regarding the mechanism of hyperostosis, this review aims to weave again the story of Arachne. We identify the main contributors who have tried to understand and explain the tendency of some of these tumors to cause hyperostosis or other skull bone involvements. CONCLUSIONS: The contribution of neurosurgeons or pathologists over the centuries is of undeniable importance and is the basis for understanding future molecular mechanisms.

Meningioma: The Tumor That Taught Us Neurosurgery.

The history of neurosurgery is ever fascinating. The journey has been tedious; nevertheless, in the landscape of success and failures we have become more efficient and polished. Skills were learned, innovations were made, and in the process we evolved. The immense contribution of meningioma surgery in this maturation process is attested by history itself. Countless stories that testify the momentous role of meningioma in the process of evolution and reformation of neurosurgical techniques exist in the literature. With every step and every attempt at conquering this tumor, we reformed to be better surgeons, more skilled and more precise. In this paper we have walked down the lane of neurosurgery and paid a due tribute to this "necessary evil."

Felix Platter and a historical perspective of the meningioma.

INTRODUCTION: Felix Platter is one of the pioneer anatomists and physicians of the 16th century who described various human diseases including meningioma. In this historical article, we present the details of Platter's life and his pioneering work on meningioma. FIRST CASE OF MENINGIOMA: In 1614, Dr. Platter described the first case of meningioma. He described the tumor as a round, fleshy mass shaped like an acorn and as large as a medium-sized apple, and full of holes. The tumor was covered with its own membrane, had no connection with the matters of the brain, and left behind a cavity after removal. This first clear description of an intracranial tumor is most consistent with encapsulated meningioma. The succeeding scholar, Harvey Cushing, coined the term "meningioma" for this tumor; neurosurgeons today describe the tumor as "parasagittal or falcine meningioma." OTHER CONTRIBUTIONS: In addition to his contribution to meningioma study, Dr. Platter was also the first to describe Dupuytren's disease, hypertrophy of the thalamus, and the retina as the sensory organ of the eye. He contributed to the germ theory of disease and gave substantial accounts of mental illnesses, gynecological disorders, and certain dermatological conditions. PUBLICATIONS: Dr. Platter published numerous accounts on various diseases. In 1614 he reported the case of meningioma in the book entitled "Platerus Observations in Hominis". Additionally, Dr. Platter published his work, 'Praxeos Medicae,' which contains his most important contribution on psychiatry and his classification of psychiatric diseases. CONCLUSION: Because of his many contributions to neuroscience, particularly his identification of meningioma, Dr. Platter should be highly credited as a pioneer in the field of neurosurgery.

High-grade meningiomas: new avenues for drug treatment?

PURPOSE OF REVIEW: For standard first-line treatment of high-grade meningiomas, surgical resection and radiotherapy are regarded as standard of care. In the recurrent setting after exhaustion of all local treatment options, no effective therapies are known and several drugs have failed to show efficacy, but novel compounds may offer hope for better disease control. RECENT FINDINGS: Upregulation of proangiogenic molecules and dysregulation of some signaling pathways such as the platelet-derived growth factor and mammalian target of rapamycin are recurrently found in high-grade meningiomas. Furthermore, in-vitro studies and single patient experience indicate that trabectedin may be an effective therapy in this tumor type. Unfortunately, so far there is a lack of conclusive clinical trials to draw definite conclusions of efficacy of these approaches. SUMMARY: There remains a significant unmet need for defining the role of medical therapy in recurrent high-grade meningioma, and more basic research and multicentric well designed trials are needed in this rare and devastating tumor type. Potentially promising novel therapeutics include antiangiogenic drugs, molecular inhibitors of signaling cascades, immunotherapeutics or trabectedin. However, more basic research is required to identify more promising drug targets. VIDEO ABSTRACT AVAILABLE: See the Video Supplementary Digital Content 1 (http://links.lww.com/CONR/A22).

Vittorio Marchi, MD (1851-1908): an unsung pioneer of neuroscience.

Vittorio Marchi was an Italian neuroscientist who lived during the second half of the 19th century. He is generally remembered for developing a novel staining method to trace the degeneration of nervous fibers in lesions of the central nervous system. This osmium-based method was used worldwide for approximately a century and continues to be cited in modern textbooks. Despite several important original scientific contributions, Marchi was never awarded a university chair. Discouraged, he left to practice neurology in a rural area but continued to write outstanding articles, and in 1897, he became a pioneer of meningioma surgery. Today, he is remembered only in specialized contexts. We hope this article can provide the recognition that Marchi deserves.

[Meningioma challenge: analysis of 80-year experience of Burdenko Neurosurgical institute and future perspectives].

The paper describes the history of meningioma surgery at Burdenko Neurosurgical Institute, analyses of accumulated series of patients and assesses effectiveness of different techniques for meningioma diagnosis and treatment of. We analyzed 15413 patients with meningiomas operated in Burdenko Neurosurgical Institute from 1932 to 2011. Mathematical analysis was performed using Statistica 6.0 program. Mortality rate during World War II reached a disastrous level of 45,8%, in 21st century it is below 1%. Temporary and permanent morbidity has also decreased. While in 1961 Karnofsky performance score was 71, in 2011 it became 83. The probability of postoperative tumour recurrence also diminished, from 40% in 1960-s to 29% in 1996 and presumably to 25% nowadays. Independent factors that influence the risk of recurrence are primary gross total resection, grade of anaplasia and radiation therapy. However, unreasonably aggressive surgery leads to significant increase of morbidity. Further improvement of surgical results in patients with meningiomas depends on implementation of new technologies for neurovisualization, abandoning extensive surgical approaches whenever possible, finding the "balance" between radical removal and expected functional outcome, wider application of radiosurgery and three-dimensional conformal radiation therapy.

A meningioma and its consequences for American history and the rise of neurosurgery.

The case of General Leonard Wood is notable both for its contribution to the field of neurosurgery and its historical significance. As one of Harvey Cushing's first successful brain tumor operations in 1910, Wood's surgery was part of the case series that culminated in Cushing's monograph Meningioma. This case was important to the rise of Cushing's career and his recognition as a member of the next generation of neurosurgeons who did not settle for mere bony decompression to taper intracranial tension but who dared to pursue intradural resections-operations that had been performed by surgeons for decades but were frowned upon because of the attendant risks. Cushing's operation to remove a recurrent brain tumor ended Wood's life in 1927. The authors discuss the effects the tumor may have had on Wood's life and career, explore an alternate explanation for the cause of Wood's death, and provide a brief account of the life of General Wood, highlighting events in his military and administrative career juxtaposed against the progression of his illness. Furthermore, the case history of the General is reviewed, using information drawn from the original patient notes and recently discovered images from the Cushing Brain Tumor Registry that elucidate more details about General Wood's story, from the injury that caused his first tumor to his final surgery, leading to his demise.

Friederich Nietzsche and the seduction of Occam's razor.

Friedrich Nietzsche developed dementia at the age of 44 years. It is generally assumed that the cause of his dementia was neurosyphilis or general pareisis of the insane (GPI). Others have proposed frontal-based meningioma as the underlying cause. We have reviewed Nietzsche's medical history and evaluated the evidence from the medical examinations he underwent by various physicians. We have viewed the possible diagnosis of GPI or meningioma in light of present neuro-ophthalmic understanding and found that Nietzsche did not have the neurological or neuro-ophthalmic symptoms consistent with a diagnosis of GPI. The anisocoria which was assumed to be Argyll Robertson pupil was present since he was six years of age. He did not have tongue tremor, lacked progressive motor features and lived at least 12 years following the onset of his neurological signs. Furthermore, the headaches that have been attributed to a frontal-based tumour were present since childhood and the pupil abnormality that has been interpreted as an "afferent pupillary defect" had the characteristics of an abnormality of the efferent pupillary innervation. None of the medical records or photographs suggest there was any ocular misalignment. We concluded that neither diagnosis of GPI nor frontal-based meningioma is convincing. It is likely that Nietzsche suffered from migraines, his blindness in his right eye was a consequence of high progressive myopia associated with retinal degeneration, his anisocoria explained by unilateral tonic pupil, and his dementia by an underlying psychiatric disease.

Clinical management of petroclival meningiomas and the eternal quest for preservation of quality of life: personal experiences over a period of 20 years.

BACKGROUND: Within the realm of neurosurgery, petroclival meningiomas are regarded as probably the most difficult tumour to be treated by microsurgery. This is due to the not infrequently large size of the tumours which, although predominantly located in the posterior fossa, may occupy more than one cranial compartment, with often significant space-occupying effect and brain stem compression. Frequent tight brain stem adherence as well as encasement of the basilar artery, its perforators and cranial nerves adds to the sometimes extreme difficulties of surgical tumour removal. Counselling patients as well as pre- and intraoperative decision making in petroclival meningiomas is even more difficult because upon clinical and radiological tumour detection, despite sometimes surprisingly large tumours, clinical symptoms are often only mild. Summarising the complicated development of petroclival meningioma surgery over the last 60 years, this paper represents the conceptual thinking of the author in regard to the treatment of petroclival meningiomas which has evolved over more than two decades, based on a special interest in these treacherous tumours, and accumulated experiences in the treatment of over 150 patients. Surgical concepts and the operative decision-making process are demonstrated in four illustrative cases. METHODS: Over a period of slightly over 20 years, between January 1988 and December 2008, 161 patients with petroclival meningiomas were managed clinically by the author or under his direct surveillance in four academic neurosurgical institutions. The observation period ranged from 4 to 242 months. Thirteen patients were lost to follow-up so, all together, complete data were available for 148 patients. In 119 patients (80%), the tumour was large. Giant tumours accounted for 7% and 11 patients, medium-sized tumours were found in 12 patients (8%) and small tumours in only six patients (4%). Sixty-two percent of the patients had invasion of Meckel's cave or some part of the cavernous sinus, mainly the posterior region to different degrees. All giant tumours and one third of the large tumours extended into more than one cranial fossa. RESULTS: The treatment modalities in the 148 patients were as follows: microsurgery alone was performed in 71 patients (48%), microsurgery and adjuvant radiosurgery in 22 patients (15%) so in 93 patients (63%), altogether, microsurgery was the primary treatment. Twenty-nine patients (20%) underwent radiosurgery as their only treatment, and two patients (1%), during the very early phase of the study period, received radiotherapy. Twenty-four patients (16%) were only observed without any additional therapy. Gross total resection was achieved in 34 patients (37%), and subtotal resection, defined as removal of more than 90% of the tumour volume, was performed in another 36 patients (39%). Radical tumour removal was possible in 76% of the patients. There was no procedure-related death within 3 months post-surgery; the early post-op surgical complication rate was 31% with new neurological deficits or worsening of pre-existing deficits. During the observation period, almost all patients recovered significantly bringing the percentage of permanent neurological deficits, again mainly cranial nerve deficits, down to 22%. CONCLUSIONS: Based on the experiences of the author, the following treatment principles in petroclival meningiomas are proposed: small tumours in asymptomatic patients should be observed. If tumour growth is detected on serial magnetic resonance imaging or treatment is desired by the patient, surgery should be the first choice. Radiosurgery in growing small tumours should be reserved to patients with advanced age or significant co-morbidities. In medium-sized tumours and symptomatic patients, radical surgery should be attempted, if possible by judicious intraoperative judgement. In large and giant petroclival meningiomas, tumour resection as radical as possible judged intraoperatively with decompression of neural structures should be performed, followed by observation and, in the case of growing tumour remnants, radiosurgery. Thus, by a combined application of advanced microsurgical techniques, thoughtful, intraoperative decision making with limited surgical aggressively and, in selected patients, with small tumours or small tumour remnants simple observation or alternative or adjunct radiosurgery, excellent results as measured by tumour control and preservation of quality of life can be achieved.

The madness of Dionysus: a neurosurgical perspective on Friedrich Nietzsche.

OBJECTIVE: To examine the possibility that an intracranial mass may have been the etiology of the headaches and neurological findings of the philosopher Friedrich Nietzsche (1844-1900) and the cause of his ultimate mental collapse in 1889. METHODS: The authors conducted a comprehensive English and German language literature search on the topic of Nietzsche's health and illness, examining Nietzsche's own writings, medical notes from his physicians, contemporary medical literature, biographical texts, and past attempts at pathography. We also examined archived portraits and engravings of the philosopher from 1864 onward. An English language search in the modern literature on the topic of psychiatric presentations of intracranial mass lesions was also conducted. RESULTS: From his late 20s onward, Nietzsche experienced severe, generally right-sided headaches. He concurrently suffered a progressive loss of vision in his right eye and developed cranial nerve findings that were documented on neurological examinations in addition to a disconjugate gaze evident in photographs. His neurological findings are consistent with a right-sided frontotemporal mass. In 1889, Nietzsche also developed a new-onset mania which was followed by a dense abulia, also consistent with a large frontal tumor. CONCLUSION: A close examination of Nietzsche's symptomatic progression and neurological signs reveals a clinical course consistent with a large, slow growing, right-sided cranial base lesion, such as a medial sphenoid wing meningioma. Aspects of his presentation seem to directly contradict the diagnosis of syphilis, which has been the standard explanation of Nietzsche's madness. The meningioma hypothesis is difficult, though not impossible, to prove; imaging studies of Nietzsche's remains could reveal the bony sequelae of such a lesion.

The development of techniques for resection of spinal cord tumors by Harvey W. Cushing.

Harvey Cushing's refinement of Halsted's meticulous surgical techniques facilitated safe resection of intradural spinal tumors. Although Cushing focused his attention on brain tumors at the Peter Bent Brigham Hospital, his numerous contributions to the treatment of intradural spinal tumors include the description of these tumors' natural histories and their histological classifications. The application of his experienced intracranial techniques to the resection of spinal tumors improved outcomes. The authors review selected operative notes and sketches to demonstrate his technique in the excision of the spinal cord tumors.

Probable destructive meningioma in an archaeological adult male skull from Alaska.

Several intracranial pathological conditions can affect the bones of the skull. The most common cause of these conditions is tumor, but infection and other diseases are also known to affect the bones of the skull. Distinguishing between the various causes of intracranial skeletal pathology in archaeological human remains is usually a challenging exercise, and a specific diagnosis will often be impossible. Meningiomas are tumors that arise in arachnoid tissues embedded in the outer layer of the dura. Because of this association, they occur almost exclusively in the skull and vertebral column. Usually meningiomas are slow-growing tumors that do not metastasize to other organs and tissues of the body. However, rare cases can be malignant and, even when meningiomas are benign, their presence and growth can adversely affect the nervous and vascular supply to other tissues in the skull and vertebral column. Their effect on adjacent bone tissue varies from stimulating bone-forming lesions to causing highly destructive lesions. A few examples of meningioma have been described in the paleopathological literature. Most of these cases are bone-stimulating meningiomas. The case presented here is a probable example of a highly destructive meningioma of the skull base, with unilateral extension into the left side of the cranium. This case is compared with a modern clinical case of destructive intracranial meningioma that was documented both radiographically and pathologically. Destructive meningiomas can be confused with other pathological conditions, including benign and malignant tumors. Criteria for differentiating the diagnostic options are reviewed.

Harvey Cushing's Meningiomas text and the historical origin of resectability criteria for the anterior one third of the superior sagittal sinus.

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."

[Peripheral, central neurofibromatosis and multiplex meningiomas (a historical review)]. / TT

Despite that the neurofibromatosis and the multiplex meningiomas have been known for centuries, it is hardly during the last decade that a significant progress has been achieved in the understanding of their etiology and pathogenesis. The development of the concept for NF is being followed from the time of the first descriptions till today.