✓ Plexiform neurofibroma of the cauda equina has been reported only twice previously. The authors report the first pediatric patient in whom such a tumor has been found. A 4-year-old boy presented with low-back pain that radiated bilaterally into the L-4 and L-5 dermatomes. A dermal sinus noted at the midthoracic level was surrounded by a hemangiomatous lesion. Magnetic resonance imaging confirmed the presence of the dermal sinus and revealed a well-defined lumbosacral mass that showed heterogeneous intensity with irregular enhancement. Intraoperatively, a solid mass, which engulfed the entire cauda equina, could not be dissected from the roots. The dermal sinus tract, however, was excised from the thoracic spine. The patient underwent radiotherapy to control the tumor and relieve his pain. Plexiform neurofibromas of the cauda equina are characterized by an insidious and progressive clinical course. The tumor mass may engulf all the roots of the cauda equina. No plexiform neurofibroma of the cauda equina has been reported to be associated with neurofibromatosis Type 1. The authors assume that the thoracic-level dermal sinus observed in this child was an incidental finding.
Trimurti D. Nadkarni, Harold L. Rekate and Stephen W. Coons
Robert W. Ryan, Tamir Wolf, Robert F. Spetzler, Stephen W. Coons, Yoel Fink and Mark C. Preul
The CO2 laser has an excellent profile for use in neurosurgery. Its high absorption in water results in low thermal spread, sparing adjacent tissue. Use of this laser has been limited to line-of-sight applications because no solid fiber optic cables could transmit its wavelength. Flexible photonic bandgap fiber technology enables delivery of CO2 laser energy through a flexible fiber easily manipulated in a handheld device. The authors examined and compared the first use of this CO2 laser fiber to conventional methods for incising neural tissue.
Carbon dioxide laser energy was delivered in pulsed or continuous wave settings for different power settings, exposure times, and distances to cortical tissue of 6 anesthetized swine. Effects of CO2 energy on the tissue were compared with bipolar cautery using a standard pial incision technique, and with scalpel incisions without cautery. Tissue was processed for histological analysis (using H & E, silver staining, and glial fibrillary acidic protein immunohistochemistry) and scanning electron microscopy, and lesion measurements were made.
Light microscopy and scanning electron microscopy revealed laser incisions of consistent shape, with central craters surrounded by limited zones of desiccated and edematous tissue. Increased laser power resulted in deeper but not significantly wider incisions. Bipolar cautery lesions showed desiccated and edematous zones but did not incise the pia, and width increased more than depth with higher power. Incisions made without using cautery produced hemorrhage but minimal adjacent tissue damage.
The photonic bandgap fiber CO2 laser produced reliable cortical incisions, adjustable over a range of settings, with minimal adjacent thermal tissue damage. Ease of application under the microscope suggests this laser system has reached true practicality for neurosurgery.
Rami O. Almefty, Tammy L. Tyree, David J. Fusco, Stephen W. Coons and Peter Nakaji
Histiocytic sarcoma is a rare malignancy with only 10 reports confirmed primarily involving the CNS. The diagnosis is dependent on the finding of malignant cells with histiocytic morphology and immunophenotype. The authors report a case of pathologically proven HS of the CNS. A 16-year-old boy presented with headaches, emesis, and altered sensorium. Noncontrast head CT scanning demonstrated a left parietal mass consistent with a tumor. Surgery was undertaken. Intraoperative findings revealed green-yellow exudates consistent with an abscess. Cultures were obtained and broad-spectrum antibiotics were started. The patient subsequently underwent multiple surgical procedures, including drainage and debulking of abscesses and hemicraniectomy. Two months after initial presentation, the patient's diagnosis of histiocytic sarcoma was confirmed. Pathological examination demonstrated necrotizing inflammation with preponderant neutrophil infiltration, variably atypical mononuclear and multinucleate histiocytes, and numerous mitoses. Additional immunohistochemistry studies confirmed immunoreactivity for CD68, CD45, CD45RO, and CD15 and were negative for CD3, CD20, melanoma cocktail, CD30, CD1a, CD34, HMB-45, and melan-A. Once the diagnosis of histiocytic sarcoma was confirmed, antibiotics were stopped and radiation therapy was undertaken. Despite treatment, the patient's neurological status continued to decline and the patient died 126 days after initial presentation.
This case represents a rare confirmed example of CNS histiocytic sarcoma. A profound inflammatory infiltrate seen on pathology and green exudates seen intraoperatively make the condition difficult to distinguish from an abscess. Immunohistochemistry showing a histiocytic origin and negative for myeloid, dendritic, or other lymphoid markers is essential for the diagnosis. Further research is needed to establish consensus on treatment.
Zaman Mirzadeh, Robert Bina, Yael Kusne, Stephen W. Coons, Robert F. Spetzler and Nader Sanai
After complete resection and radiation therapy, the 10-year overall survival rates for adult patients with posterior fossa ependymomas approach 85%. This favorable outcome profile emphasizes the critical importance of functional preservation to this patient population. Here, the authors identify predictors of functional outcome following microsurgical resection of adult posterior fossa ependymomas.
The authors identified adult patients with newly diagnosed WHO Grade II posterior fossa ependymomas who underwent microsurgical resection at the Barrow Neurological Institute from 1990 to 2011. Clinical and radiographic variables were collected, including volumetric extent of resection, foramen of Luschka extension, cystic changes, peritumoral T2 signal changes, Karnofsky Performance Scale (KPS) score, National Institutes of Health Stroke Scale (NIHSS) score, progression-free survival (PFS), and overall survival (OS).
Forty-five patients were identified, with a median clinical follow-up of 103 months. The median PFS and OS were 6.8 and 8.6 years, respectively. Extent of resection and adjuvant radiotherapy were predictive of improved PFS (p = 0.005) and were nonsignificantly associated with improved OS. Univariate analysis revealed that tumor size (p < 0.001), cystic changes (p < 0.01), postoperative T2 signal (p < 0.01), and CSF diversion (p = 0.048) predicted functional and neurological recovery rates, based on KPS and NIHSS scores, respectively. Multivariate regression analysis identified tumor size (p < 0.001), cystic changes (p = 0.01), and CSF diversion (p = 0.02) as independent predictors of slower functional recovery, while only tumor size (p = 0.007) was an independent predictor of neurological recovery. Specifically, by 6 weeks postoperatively, baseline KPS score was recovered by only 43.8% of patients with tumors larger than 30 cm3 (vs 72.4% patients with tumors < 30 cm3), 35.3% of patients with cystic tumors (vs 78.6% of patients with noncystic tumors), and 46.7% of patients requiring CSF diversion (vs 70% of patients not requiring CSF diversion).
Greater extent of resection and adjuvant radiotherapy significantly improve PFS in adult patients with posterior fossa ependymomas. Tumor size, cystic changes, and the need for CSF diversion were independent predictors of the rate of functional recovery in this patient population. Taken together, these functional outcome predictors may guide preoperative estimations of recovery following microsurgical resection.
Report of 3 cases
Eric M. Horn, Peter Nakaji, Stephen W. Coons and Curtis A. Dickman
Spinal meningeal melanocytomas are rare lesions that are histologically benign and can behave aggressively, with local infiltration. The authors present their experience with intramedullary spinal cord melanocytomas consisting of 3 cases, which represents the second largest series in the literature. A retrospective chart review was performed following identification of all spinal melanocytomas treated at the author's institution, based on information obtained from a neuropathology database. The charts were reviewed for patient demographics, surgical procedure, clinical outcome, and long-term tumor progression. Three patients were identified in whom spinal melanocytoma had been diagnosed between 1989 and 2006. The patients' ages were 37, 37, and 48 years, and the location of their tumor was C1–3, T9–10, and T-12, respectively. All 3 had complete resection with no adjuvant radiotherapy during follow-up periods of 16, 38, and 185 months, respectively. One patient demonstrated a recurrence 29 months after resection and the other 2 patients have demonstrated asymptomatic recurrences on imaging studies obtained at 16 and 38 months following resection.
With these cases added to the available literature, the evidence strongly suggests that complete resection is the treatment of choice for spinal melanocytomas. Even with complete resection, recurrences are common and close follow-up is needed for the long term in these patients. Radiation therapy should be reserved for those cases in which complete resection is not possible or in which there is recurrence.
L. Fernando Gonzalez, Gregory P. Lekovic, Jennifer Eschbacher, Stephen Coons, Randall W. Porter and Robert F. Spetzler
✓Cavernous hemangiomas that occur within the cavernous sinus (CS) are different from cerebral cavernous malformations (CMs) clinically, on imaging studies, and in their response to treatment. Moreover, CMs are true vascular malformations, whereas hemangiomas are benign vascular tumors. Because of these differences, the authors suggest that these two entities be analyzed and grouped separately. Unfortunately, despite these differences, much confusion exists in the literature as to the nature, behavior, and classification of these two distinct lesions. This confusion is exacerbated by subtle histological differences and the inconsistent use of nomenclature. The authors use the term “cavernous malformation” to refer to intraaxial lesions only; they prefer to use the term “cavernous sinus hemangioma” to refer to extraaxial, intradural hemangiomas of the CS.
Report of four cases
Michael T. Lawton, Joseph E. Heiserman, Stephen W. Coons, Bruce D. Ragsdale and Robert F. Spetzler
✓ Juvenile active ossifying fibroma is a rare lesion seldom seen by neurosurgeons. It originates in the paranasal sinuses during childhood, grows slowly, and encroaches on adjacent orbital and cranial compartments. In the past 3 years, four patients with this lesion were seen (three men and one woman; mean age 28 years). The clinical presentations were different with each patient: sinusitis, meningitis, periorbital pain, and a unique case of a juvenile active ossifying fibroma presenting with high-grade internal carotid artery stenosis and ischemic symptoms. Three patients were treated by transfacial approaches: two with a transfrontal—nasal approach and one with a transfrontal—nasoorbital approach. Two open resections resulted in gross-total excision and no recurrence as of the 2-year follow-up review. In the third patient, the tumor-encased carotid artery was preserved at the expense of a complete resection; that patient underwent superficial temporal artery—middle cerebral artery bypass and remains without ischemic symptoms or tumor recurrence at 2 years. The fourth patient underwent three subtotal endoscopic resections and is also without symptomatic recurrence at 2 years.
Three points must be made concerning these lesions. First, the clinical and radiographic characteristics of juvenile active ossifying fibroma may not be easily recognized by neurosurgeons, which could lead to misdiagnosis and mismanagement of these lesions. Second, this tumor can encase the carotid artery and cause severe stenosis or occlusion. Third, complete resection of the tumor is required to effect a cure, and transfacial approaches, which give wide exposure of the sinuses, appear to yield better, more radical resections than endoscopic procedures.
M. Yashar S. Kalani, Sudarshan Iyer, Stephen W. Coons and Kris A. Smith
Intradural spinal teratomas are rare tumors of the spinal cord that are infrequently encountered in children. Although the mechanistic basis for the formation of these tumors is unclear, several lines of evidence suggest that a dysembryogenic process in the embryo results in their formation. The authors present a case of spinal intradural teratoma in an 18-year-old, previously healthy man and review the literature linking the development of these tumors to defects in neurulation and embryogenesis.
Paul W. Detwiler, Randall W. Porter, Stephen W. Coons, Robert F. Spetzler, Carlos A. Carrion and Harold L. Rekate
✓ Infantile myofibromatosis involving the skull is a benign disease if there is a solitary lesion. However, the multifocal form with skull involvement may portend a lethal course in the 1st year of life if there is involvement of the heart, lungs, or gastrointestinal tract. The authors report the case of a 3-year-old boy with an enlarging left parietal skull lesion that had been present since infancy. Increasing pain and the need to obtain tissue for diagnosis led to resection of the lesion by means of a small craniectomy. Further evaluation revealed no other lesions. A distinctly rare disease is presented, and the need for staging in children younger than 2 years of age is suggested to rule out cardiac, pulmonary, or gastrointestinal involvement.
Kathryn E. Fenton, Nikolay L. Martirosyan, Mohammed G. Abdelwahab, Stephen W. Coons, Mark C. Preul and Adrienne C. Scheck
For patients with glioblastoma multiforme, median survival time is approximately 14 months. Longer progression-free and overall survival times correlate with gross-total resection of tumor. The ability to identify tumor cells intraoperatively could result in an increased percentage of tumor resected and thus increased patient survival times. Available labeling methods rely on metabolic activity of tumor cells; thus, they are more robust in high-grade tumors, and their utility in low-grade tumors and metastatic tumors is not clear. The authors demonstrate intraoperative identification of tumor cells by using labeled tumor-specific antibodies.
GL261 mouse glioma cells exhibit high expression of a membrane-bound protein called second tyrosinase-related protein (TRP-2). The authors used these cells to establish an intracranial, immunocompetent model of malignant glioma. Antibodies to TRP-2 were labeled by using Alexa Fluor 488 fluorescent dye and injected into the tail vein of albino C57BL/6 mice. After 24 hours, a craniotomy was performed and the tissue was examined in vivo by using an Optiscan 5.1 handheld portable confocal fiber-optic microscope. Tissue was examined ex vivo by using a Pascal 5 scanning confocal microscope.
Labeled tumor cells were visible in vivo and ex vivo under the respective microscopes.
Fluorescently labeled tumor-specific antibodies are capable of binding and identifying tumor cells in vivo, accurately and specifically. The development of labeled markers for the identification of brain tumors will facilitate the use of intraoperative fluorescence microscopy as a tool for increasing the extent of resection of a broad variety of intracranial tumors.