The diagnosis and management of pediatric sellar lesions is discussed in this paper. Craniopharyngiomas account for the majority of pediatric sellar masses, and pituitary adenomas are extremely uncommon during childhood. The diagnosis of sellar lesions involves a multidisciplinary effort, and detailed endocrinological, ophthalmological, and neurological testing is critical in the evaluation of a new sellar mass. The management of pituitary adenomas varies depending on the entity. For most tumors other than prolactinomas, transsphenoidal resection remains the mainstay of treatment. Less invasive methods, such as endoscopic transsphenoidal surgery and stereotactic radiosurgery, have shown promise as primary and adjuvant treatment modalities, respectively.
Jay Jagannathan, Aaron S. Dumont, John A. Jane Jr. and Edward R. Laws Jr.
Case report and review of the literature
Daniel M. Prevedello, Jay Jagannathan, John A. Jane Jr., M. Beatriz S. Lopes and Edward R. Laws Jr.
Pituitary adenomas are heterogeneous in growth rate, invasiveness, and recurrence. To understand the biological behavior of the individual adenoma more fully, cell proliferation markers such as monoclonal antibodies targeted against the Ki-67 antigen have been applied. The Ki-67 antigen is a protein related to cell proliferation and is expressed in cell nuclei throughout the entire cell cycle. The authors report the case of an extremely rapidly growing pituitary adenoma with cavernous sinus invasion. The lesion, which displayed a high Ki-67 labeling index (LI; 22%), was found in a 54-year-old woman who presented with diplopia and headaches. The patient underwent three transsphenoidal operations in less than 6 months and, ultimately, was treated with fractionated intensity-modulated radiation therapy. The relationships between high Ki-67 LIs and tumor recurrence, invasiveness, and growth velocity in pituitary adenomas are reviewed.
Jay Jagannathan, Daniel M. Prevedello, Vivek S. Ayer, Aaron S. Dumont, John A. Jane Jr. and Edward R. Laws
In this study the authors address the efficacy and safety of frameless stereotaxy in transsphenoidal surgery.
One thousand transsphenoidal operations were performed at the authors' institution between June 2000 and July 2005. This series consists of a retrospective review of 176 patients entered in a prospectively obtained database who underwent frameless stereotactic transsphenoidal surgery in which magnetic resonance (MR) imaging, computerized tomography (CT) scanning, or fluoroscopic guidance was used. Of the 176 patients, 104 (59%) had suprasellar extension of their tumor, 70 (40%) had involvement of the visual apparatus, and 65 (37%) had cavernous sinus involvement. All patients underwent detailed pre- and postoperative neurological, endocrinological, radiographic, and ophthalmological follow-up evaluations. Records were reviewed retrospectively for intraoperative and postoperative complications related to the surgical approach.
No instances of visual deterioration, carotid artery (CA) stenosis, or stroke were observed following transsphenoidal surgery. Only one patient sustained damage to the CA intraoperatively, and this was controlled in the operating room. Five patients (3%) required an intensive care unit stay postoperatively. Intraoperative cerebrospinal fluid leakage was encountered in 112 patients (64%) and was more frequently observed in tumors with suprasellar involvement.
Frameless stereotaxy is a safe and effective modality for the treatment of recurrent or invasive sellar masses. All three frameless stereotaxy modalities provided accurate information regarding the anatomical midline and the trajectory to the sella turcica. The MR imaging, CT scanning, and fluoroscopic stereotaxy modalities all have unique advantages as well as specific limitations.
Daniel M. Prevedello, Francesco Doglietto, John A. Jane Jr., Jay Jagannathan, Joseph Han and Edward R. Laws Jr.
✓The history of the endoscope exemplifies the manner in which technological advances influence medicine and surgery. Endoscopic systems have evolved and improved, and they currently provide detailed visualization of a variety of deep organ structures. Otorhinolaryngological surgeons have used the endoscope for more than 30 years. In the 1990s, a number of influential neurosurgeons and otorhinolaryngological surgeons began performing purely endoscopic pituitary surgery. Endoscopic transsphenoidal operations are now extending beyond the sella. The collaboration between otorhinolaryngologists and neurosurgeons has produced a new subspecialty of “endoscopic skull base surgery.” There is a great deal of progress still to be made in developing skills, instruments, and improving skull base repair. The extended skull base approaches allow surgical exposures from the olfactory groove to C-2 and to the infratemporal region and jugular fossa laterally. This article discusses the history of the endoscope, the pivotal technological advances, and the key figures in the burgeoning field of endoneurosurgery.
John A. Jane Jr., Joseph Han, Daniel M. Prevedello, Jay Jagannathan, Aaron S. Dumont and Edward R. Laws Jr.
Sellar tumors are most commonly approached through the transsphenoidal corridor, and tumor resection is most often performed using the operating microscope. More recently the endoscope has been introduced for use either as an adjunct to or in lieu of the microscope. Both the microscopic and endoscopic transsphenoidal approaches to sellar tumors allow safe and effective tumor resection. The authors describe their current endoscopic technique and elucidate the advantages and disadvantages of the pure endoscopic adenomectomy compared with the standard microscopic approach.
Jay Jagannathan, Jason P. Sheehan, Nader Pouratian, Edward R. Laws, Ladislau Steiner and Mary Lee Vance
In this study the authors address the efficacy and safety of Gamma Knife surgery (GKS) in patients with adrenocorticotropic hormone–secreting pituitary adenomas.
A review of data collected from a prospective GKS database between January 1990 and March 2005 was performed in patients with Cushing's disease. All but one patient underwent resection for a pituitary tumor, without achieving remission. Successful endocrine outcome after GKS was defined as a normal 24-hour urinary free cortisol (UFC) concentration posttreatment after a minimum of 1 year of follow up. Patient records were also evaluated for changes in tumor volume, development of new hormone deficiencies, visual acuity, cranial nerve neuropathies, and radiation-induced imaging changes. Ninety evaluable patients had undergone GKS, with a mean endocrine follow-up duration of 45 months (range 12–132 months). The mean dose to the tumor margin was 23 Gy (median 25 Gy).
Normal 24-hour UFC levels were achieved in 49 patients (54%), with an average time of 13 months after treatment (range 2–67 months). In the 49 patients in whom a tumor was visible on the planning magnetic resonance (MR) image, a decrease in tumor size occurred in 39 (80%), in seven patients there was no change in size, and tumor growth occurred in three patients. Ten patients (20%) experienced a relapse of Cushing's disease after initial remission; the mean time to recurrence was 27 months (range 6–60 months). Seven of these patients underwent repeated GKS, with three patients achieving a second remission. New hormone deficiencies developed in 20 patients (22%), with hypothyroidism being the most common endocrinopathy after GKS. Five patients experienced new visual deficits or third, fourth, or sixth cranial nerve deficits; two of these patients had undergone prior conventional fractionated radiation therapy, and four of them had received previous GKS. Radiation-induced changes were observed on MR images in three patients; one had symptoms attributable to these changes.
Gamma Knife surgery is an effective treatment for persistent Cushing's disease. Adenomas with cavernous sinus invasion that are not amenable to resection are treatable with the Gamma Knife. A second GKS treatment appears to increase the risk of cranial nerve damage. These results demonstrate the value of combining two neurosurgical treatment modalities—microsurgical resection and GKS—in the management of pituitary adenomas.