Desmoplastic infantile ganglioglioma (DIG) is a rare, distinctive, supratentorial neoplasm with a generally favorable prognosis. Clinical, radiographic, and pathologic features can sometimes mimic those of a malignant tumor and other serious intracranial disorders. The author describes his experience with 3 cases of DIG, each of which initially masqueraded as another neurological disease with a very different prognosis. Case 1 was an infant boy referred for evaluation of a hemorrhagic infarction at birth. Case 2 was an infant girl referred for evaluation of a holohemispheric malignant neoplasm. Case 3 was an infant girl referred for evaluation of an intracranial mass believed to be a subdural empyema or possible sarcoma. In each case the lesion was resected and found to be a WHO grade I DIG. Each child has had a benign postoperative course. DIG can be mistaken for other serious neurological conditions including malignant neoplasm, cerebral infarction, and infection. It is prudent to consider this rare, low-grade resectable tumor in the differential diagnosis of atypical intracranial masses of childhood, as the impact on prognosis can be profound. The author discusses management strategies for DIG, including a role for molecular sequencing.
Roberta Rehder and Alan R. Cohen
William Sharpe was an intriguing figure in the history of American neurosurgery. He was an extraordinarily bright and gifted man who led a flamboyant, colorful, and unconventional life. He had an international impact on the field of neurosurgery during the first half of the 20th century, yet few practicing neurosurgeons know his name. In this report, the authors discuss Sharpe’s contributions to neurosurgery along with the remarkable quirkiness that came to define his professional and personal life.
Technical note and review of the literature
M. Samy Abdou and Alan R. Cohen
✓ The surgical technique for the endoscopic evacuation of colloid cysts of the third ventricle in 13 patients is described. The authors conclude that endoscopic resection of these lesions is a useful addition to the current surgical repertoire and a viable alternative to stereotactic aspiration or open craniotomy.
Case report and review of the literature
Shenandoah Robinson and Alan R. Cohen
Cowden disease is a rare autosomal-dominant phacomatosis and cancer syndrome that is associated with Lhermitte–Duclos disease (LDD), also called dysplastic cerebellar gangliocytoma.
In this review the authors summarize the additions to the literature during the past 5 years, with emphasis on new case reports and advances in imaging and molecular biology. Adult-onset LDD is now considered pathognomonic for Cowden disease.
Approximately 220 cases of LDD have been reported. Magnetic resonance imaging in patients with LDD is often diagnostic, and imaging studies have facilitated accurate diagnosis and contributed to the improved outcome in affected patients. Cowden disease and other rare, related disorders, such as Bannayan-Riley-Ruvalcaba, Proteus, and Proteus-like syndromes, are often caused by mutations of the PTEN gene.
Because of the high incidence of systemic cancer in patients with Cowden disease, it is important for neurosurgeons to recognize the association between this disease and LDD and to refer affected patients for appropriate cancer screenings and interventions.
Robin F. Koeleveld and Alan R. Cohen
✓ A case of primary embryonal-cell carcinoma of the parietal lobe is reported. The unusually chronic presentation of such a malignant tumor is described. The atypical computerized tomography and magnetic resonance imaging characteristics of this lesion are presented. Review of the literature yielded no previous reports of a lobar embryonal-cell carcinoma. The rarity of intracranial germ-cell tumors presenting off the midline is discussed.
Alan R. Cohen
Roberta Rehder, Subash Lohani and Alan R. Cohen
Donald Darrow Matson made seminal contributions to the field of pediatric neurosurgery. Born in 1913 in Fort Hamilton, New York, Matson was the youngest of four sons of an army colonel. He graduated from Cornell University and, years later, from Harvard Medical School. Matson selected Peter Bent Brigham Hospital for his neurosurgical training, which was interrupted during World War II. As a neurosurgeon, he worked close to the front lines under Brigadier General Elliot Cutler in Europe, earning a Bronze Star. Matson returned to Boston to become Franc Ingraham’s fellow and partner. He was a masterful surgeon and, with Ingraham, published Neurosurgery of Infancy and Childhood in 1954, the first pediatric neurosurgery textbook in the world. Upon Ingraham’s retirement, Matson became chairman of the department of neurosurgery at Boston Children’s Hospital and Peter Bent Brigham. In 1968, he became the inaugural Franc D. Ingraham Professor of Neurological Surgery at Harvard Medical School. Among his neurosurgical accomplishments, Matson served as President of the Harvey Cushing Society, later known as the American Association of Neurological Surgeons. He was unable to preside at the 1969 meeting that marked the 100th anniversary of Cushing’s birth, having contracted Creutzfeldt-Jakob disease. Matson died at the age of 55, surviving his mentor Ingraham by only 4 years.
Subash Lohani and Alan R. Cohen
In 1929, Franc D. Ingraham, Harvey Cushing's protégé, established the first pediatric neurosurgical unit in the world at Boston Children's Hospital and dedicated his career to the neurosurgical care of children. He trained with both Cushing and Dandy and spent 1 year working in Oxford with Sherrington, who considered Ingraham to be the finest operative surgeon ever to work in his laboratory. Ingraham was instrumental in developing novel treatments, which he compiled in his classic book, Neurosurgery of Infancy and Childhood. Although he was modest and shy, Ingraham loved to entertain children with magic and enjoyed photography in and out of the operating room. Unfortunately, his career was plagued by personal illness, and he died young in 1965 at the age of 67. Despite his prolific 36-year neurosurgical career, Ingraham remained an associate professor at Harvard at his retirement. To recognize his remarkable contributions, Harvard established an endowed chair in his name in 1967. Ingraham was a pioneer and a leader in the development of pediatric neurosurgery by virtue of his imagination, intelligence, and ability to lead and inspire others. Cushing has come to be regarded as the founder of neurosurgery. It is fair to conclude that Ingraham, his disciple, is the founder of pediatric neurosurgery.
Carl B. Heilman and Alan R. Cohen
✓ The fiberoptic endoscope has never gained popularity among neurosurgeons although it is ideally suited for navigating within the cerebral ventricles. Recent advances in optics and miniaturization make the application of endoscopy in neurosurgery more practical. The authors report eight children who underwent ventriculoscopic fenestration of symptomatic loculated cerebrospinal fluid (CSF) collections. These CSF collections were either isolated ventricular cysts or trapped lateral ventricles secondary to obstruction at the foramen of Monro. Cyst wall dissection was carried out with a “saline torch” dissector which was introduced through a working channel in the ventriculoscope. The torch was used to coagulate vessels and to sculpt large windows in cyst walls or in the septum pellucidum. Ventriculoscope-guided cyst fenestration can be performed safely and easily under direct vision. The technique may permit simplification of shunt systems in some patients and elimination of shunts in others.