Chordoma, a rare bone tumor that occurs along the spine, has led scientists on a fascinating journey of discoveries. In this historical vignette, the authors track these discoveries in diagnosis and treatment, noting events and clinicians that played pivotal roles in our current understanding of chordoma. The study of chordoma begins in 1846 when Rudolf Virchow first observed its occurrence on a dorsum sellae; he coined the term “chordomata” 11 years later. The chordoma’s origin was greatly disputed by members of the scientific community. Eventually, Müller’s notochord hypothesis was accepted 36 years after its proposal. Chordomas were considered benign and slow growing until the early 1900s, when reported autopsy cases drew attention to their possible malignant nature. Between 1864 and 1919, the first-ever symptomatic descriptions of various forms of chordoma were reported, with the subsequent characterization of chordoma histology and the establishment of classification criteria shortly thereafter. The authors discuss the critical historical steps in diagnosis and treatment, as well as pioneering operations and treatment modalities, noting the physicians and cases responsible for advancing our understanding of chordoma.
Ronald Sahyouni, Khodayar Goshtasbi, Amin Mahmoodi and Jefferson W. Chen
Brandon M. Lehrich, Nolan J. Brown, Shane Shahrestani, Ronald Sahyouni and Frank P. K. Hsu
Dr. James Tait Goodrich was an internationally renowned pediatric neurosurgeon who pioneered the neurosurgical procedures for the multistage separation of craniopagus twins. As of March 2020, 59 craniopagus separations had been performed in the world, with Goodrich having performed 7 of these operations. He was the single most experienced surgeon in the field on this complex craniofacial disorder. Goodrich was a humble individual who rapidly rose through the ranks of academic neurosurgery, eventually serving as Director of the Division of Pediatric Neurosurgery at the Children’s Hospital at Montefiore Medical Center in the Bronx, New York. In this historical vignette, the authors provide context into the history of and sociocultural attitudes toward conjoined twins; the epidemiology and classification of craniopagus twins; the beginnings of surgery in craniopagus twins; Goodrich’s neurosurgical contributions toward advancing treatment for this complex craniofacial anomaly; and vignettes of Goodrich’s unique clinical cases that made mainstream news coverage.
Ali O. Jamshidi, André Beer-Furlan, Daniel M. Prevedello, Ronald Sahyouni, Mohamed A. Elzoghby, Mina G. Safain, Ricardo L. Carrau, John A. Jane Jr. and Edward R. Laws
The endoscopic endonasal approach has been proposed as a primary surgical strategy for select craniopharyngiomas. However, those tumors that arise from the sella have not been classified with the other craniopharyngioma subtypes in terms of surgical nuances, intraoperative findings, and postoperative outcomes. The authors describe their experience with a select subtype of craniopharyngioma arising within the sella subjacent to the diaphragma sellae and refer to these tumors as type 0.
After obtaining IRB approval, three institutions retrospectively reviewed their data collected from 2005 to 2017. Patients eligible for inclusion in the study were those who had tumors that originated from the sella inferior to the diaphragma sellae. Demographic, clinical, radiological, surgical, and follow-up data were examined and analyzed.
Twenty-eight patients (average age 19.3 years, range 3–60 years) were included in this study. Sixteen patients (57%) were younger than 18 years of age. All patients had characteristic imaging features of an expanded sella. Seventy-five percent of the patients presented with some form of visual field deficit (89% had radiographic optic apparatus compression) and 39% with hypopituitarism. The average maximal tumor diameter in the axial, coronal, or sagittal plane was 3.1 cm. Gross-total resection was achieved in 82% of the patients. Twenty-one percent of patients experienced an iatrogenic complication, and there were only two cases (7%) of postoperative cerebrospinal fluid (CSF) leakage. Only two patients (7%) required the use of a nasoseptal flap as part of their original reconstruction. Pathology was uniformly the adamantinomatous subtype. Postoperative objective visual outcomes were improved in 71% of the patients with visual symptoms or visual field deficits on presentation and stable in 24%. Mean follow-up was 45.1 months (range 3–120 months) with an 18% recurrence rate at a mean of 44.4 months (range 10–84 months). One patient was lost to follow-up. Thirty-six percent of patients received postoperative radiation to treat recurrence or residual tumor. Endocrine data are also presented.
Craniopharyngiomas that originate within the sella below the diaphragma sellae are a select subtype characterized by 1) an enlarged sella, 2) an intact diaphragma sellae at surgery, and 3) an adamantinomatous pathology. These tumors can be treated transnasally without the absolute need for neurovascular flap reconstruction, as there is a low risk of CSF leakage.