Surgical management of intracranial gliomas--biopsy, resection, or watchful waiting.
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Biopsy↗
Biomedical subjects
E R Laws
Publications and source records attributed to E R Laws.
Cerebral atrophy in Cushing's disease.
BACKGROUND: Cushing's disease causes significant pathological changes throughout the body as a result of elevated cortisol levels. Very few systematic investigations have focused on the morphologic effects of hypercortisolism on the central nervous system. The validity of using premature cerebral atrophy as a diagnostic tool for Cushing's disease remains unknown. METHODS: This study includes 63 patients with Cushing's disease who were evaluated and treated at the University of Virginia Medical Center. Radiologists randomly compared these individuals with age- and sex-matched controls in a blinded protocol, assessing the degree of cerebral atrophy on computed tomography and magnetic resonance scans. RESULTS: Patients with Cushing's disease showed significant premature atrophy when compared with controls. This trend continued after subdividing the groups based on age and duration of symptoms except in the following groups: age greater than 60, duration of symptoms less than 1 year, and symptoms lasting between 4-5 years. CONCLUSIONS: Excluding the three aforementioned groups, the hypercortisolemic state manifested in patients with Cushing's disease promotes the premature development of cerebral atrophy, which can be identified on routine radiologic imaging and may assist in the clinical diagnosis of the condition.
Adult↗
Double pituitary lesions in three patients with Cushing's disease.
Double pituitary adenomas are rare in surgical specimens and the most common clinical feature in reported patients has been acromegaly. We report 3 cases of double pituitary lesions in patients who presented with Cushing's disease. In a 22-year-old man (case 1) with delayed puberty and low testosterone levels, mild hyperprolactinemia was diagnosed and treated with dopamine agonist therapy that reduced the prolactin (PRL) levels to normal. Over a 1-year period Cushing's disease developed gradually and was confirmed with classical endocrine testing. In a 27-year-old woman (case 2) who initially presented with severe depression and morbid obesity there was a gradual onset of Cushing's disease; initially she had minimally elevated serum PRL. In a 33-year-old woman (case 3) there was a 2-year history of Cushing's disease characterized by hirsutism, hypertension and weight gain; serum PRL was normal. Magnetic resonance imaging in all 3 patients revealed a microadenoma that was successfully removed by transsphenoidal pituitary surgery. Histology and immunocytochemistry in case 1 and case 3 revealed a corticotroph cell adenoma and a PRL cell adenoma in separate areas of the pituitary. In case 3 the PRL cell adenoma was "silent" but in case 1 the PRL cell adenoma may have been the cause of the mild hyperprolactinemia. In case 2 nodular corticotroph hyperplasia was the cause of Cushing's disease and a "silent" PRL cell adenoma was also identified. We conclude from these cases and a literature review that double pituitary lesions may occur in patients with Cushing's disease. The corticotroph part of the double lesion may consist of a corticotroph cell adenoma or, as reported in this study, of corticotroph nodular hyperplasia. The counterpart of the double lesion may consist either of a "silent" PRL cell adenoma or a functional PRL cell adenoma causing hyperprolactinemia.
Adenoma↗
Long-term outcomes for surgically resected craniopharyngiomas.
OBJECTIVE: This retrospective study critically analyzed the long-term functional outcomes and tumor recurrence rates for surgically treated craniopharyngiomas. METHODS: This study used an outcome classification system that included functioning vision, independent versus dependent living, Karnofsky Performance Scale scores, academic levels, work status, and psychological status. Tumor recurrence rates were analyzed with respect to the extent of surgical resection and adjunctive radiotherapy. RESULTS: For 121 patients, with a mean follow-up period of 10 years, the overall "good outcome" rate was 60.3%. Factors associated with poor outcomes included lethargy at presentation, visual deterioration, papilledema, tumor calcification, hydrocephalus, and tumor adhesiveness at surgery. Gross total resection was associated with good outcomes (P = 0.017) and decreased risk of recurrence (P = 0.024). Subtotal resection was associated with increased risk of tumor recurrence (P = 0.0235). The highest risk of recurrence was in the subtotal resection/no radiation group (P = 0.0001). There were no differences in outcomes or recurrence rates between pediatric and adult patients. There were also no differences in outcomes or recurrence rates between papillary and adamantinous tumors. Approximately one-third of patients exhibited morbid obesity, and permanent diabetes insipidus was observed for 25 patients. CONCLUSION: A rigorous evaluation of outcomes for tumors such as craniopharyngiomas must consider not only the extent of resection, as judged by postoperative imaging, but also the long-term physical, intellectual, and psychological functioning of the patients.
Adolescent↗
Transsphenoidal surgery for Cushing's disease: outcome in patients with a normal magnetic resonance imaging scan.
OBJECTIVE: Transsphenoidal surgery for Cushing's disease from a pituitary adenoma is an effective and safe treatment. Definitive preoperative diagnosis of Cushing's disease caused by a pituitary adenoma is often difficult, particularly in patients with normal imaging studies and a normal sella turcica. We present the outcome of transsphenoidal surgery in patients with presumed Cushing's disease and a normal pituitary magnetic resonance imaging scan. METHODS: Between January 1992 and December 1997, 105 patients underwent transsphenoidal surgery for Cushing's disease at our institution. The criteria for inclusion in this study were clinical and biochemical studies strongly suggestive of Cushing's disease, a normal magnetic resonance imaging scan with normal sella and sellar contents, no previous pituitary surgery, and transsphenoidal surgery performed at this institution. Eighteen patients fulfilled these criteria, and their results were analyzed retrospectively. RESULTS: The average age of the patients was 47.8 years; there were 13 women and 5 men. Inferior petrosal sinus sampling with and without corticotropin-releasing hormone stimulation was performed in 16 patients with correct localization of the lesion in 13 (81%). During surgery, the surgeon identified and removed 17 pituitary tumors; 15 patients had selective adenomectomies, one had a hemihypophysectomy, and two had total hypophysectomies. Thirteen discrete adrenocorticotropic hormone-secreting adenomas were proven histologically, and one pituitary gland had diffuse involvement with tumor. Complications occurred in five patients. Sixteen patients who were followed up for an average of 21.6 months had sustained remission, 12 of whom were profoundly hypocortisolemic immediately after surgery. CONCLUSION: In patients with Cushing's disease and a normal magnetic resonance imaging scan, an experienced surgeon can perform transsphenoidal surgery resulting in effective removal of very small microadenomas, with clinical and biochemical remission in the majority. Inferior petrosal sinus sampling is helpful in localizing the adenoma.
Adenoma↗
Results of transsphenoidal surgery for Cushing's disease in patients with no histologically confirmed tumor.
OBJECTIVE: Pathological confirmation of surgical resection of an adenoma for Cushing's disease is not always achieved. We reviewed our experience to determine the prognostic significance of this lack of confirmation regarding outcome, and we evaluate explanations for this situation. METHODS: The records of all patients undergoing transsphenoidal surgery for Cushing's disease from 1992 to 1998 were reviewed, and those with no histological confirmation of tumor were identified. Information regarding preoperative and postoperative hormonal levels and clinical symptoms, preoperative magnetic resonance imaging data, intraoperative findings, and the number of reoperations were recorded. RESULTS: There were 29 patients with no confirmation of tumor. Nineteen (66%) of these patients were cured with surgery and only one had a recurrence of disease, with an average follow-up of 38 months. An abnormality thought to represent an adenoma at the time of surgery was removed in 26 patients (90%). Preoperative magnetic resonance imaging suggested a discrete lesion in 21 patients (72%). Neither intraoperative impression nor magnetic resonance imaging appearance was correlated with outcome. CONCLUSION: Patients with no histological confirmation of tumor after transsphenoidal surgery for Cushing's disease are likely to have a good outcome. The results do not differ significantly from reported cure rates in patients with confirmed adenomas. Possible explanations for this situation are discussed.
Adenoma↗
Clinically silent corticotroph tumors of the pituitary gland.
OBJECTIVE: To determine the clinical presentation, imaging characteristics, microscopic and ultrastructural characteristics, and treatment outcomes of patients with clinically silent pituitary corticotroph adenomas. METHODS: All silent corticotroph adenomas diagnosed at the Mayo Clinic during the years 1975 through 1997 were selected from the files of the Mayo Tissue Registry. RESULTS: We studied 23 cases, occurring in 16 male and 7 female patients (age range, 11-79 yr; mean age, 48 yr), who presented with headaches (50%), visual field defects (61%), extraocular muscle paresis (13%), hypopituitarism (26%), and galactorrhea/amenorrhea (43%/29% of the female patients). No patients exhibited clinical hypercortisolism. All tumors were macroadenomas (2.4+/-0.8 cm; range, 1.5-4.0 cm) and exhibited suprasellar extension in 87% of the cases and hemorrhage, necrosis, and/or cystic changes in 61%. All tumors stained were variably periodic acid-Schiff-, adrenocorticotropic hormone-, and beta-endorphin-positive, particularly Subtype I lesions. Ultrastructural classification was performed in 19 cases. In a comparison of Subtype I and II tumors, differences were observed with respect to sex (male/female, 1.4:1 versus 6:1), preoperative hyperprolactinemia (5 of 16 versus 0 of 6 cases), preoperative hypopituitarism (9 of 16 versus 5 of 7 cases), radiographic or gross invasion (7 of 16 versus 5 of 7 cases), and partial or total postoperative pituitary failure (6 of 16 versus 6 of 6 cases). The overall median postoperative follow-up period was 4.9 years (range, 0.3-16.6 yr); 54% of the patients had persistent or recurrent tumors. CONCLUSION: Clinically silent corticotroph adenomas behave in an aggressive manner and are characterized by the following: lack of clinical signs or symptoms of Cushing's syndrome and normal cortisol levels; no or only minor elevations of serum adrenocorticotropic hormone levels; macroadenomas with hemorrhagic infarction; and presentation dominated by mass effect symptoms. The high persistence/recurrence rate underscores the need for long-term follow-up.
Adenoma↗
Pituitary surgery for the management of acromegaly.
Active acromegaly is almost always the result of a benign growth hormone (GH)-secreting adenoma of the pituitary gland. Because the same pituitary stem cell can produce both GH and prolactin (PRL), many acromegalic patients also have hyperprolactinemia. The advantages of surgical excision of pituitary adenomas associated with acromegaly include: (1) prompt decrease in GH; (2) reliable and immediate relief of the mass effect from the tumor (decompression of the optic nerves and chiasm), and (3) the opportunity to obtain tumor tissue for characterization and investigative study. Currently, more than 97% of operations for removal of pituitary tumors associated with acromegaly are done using the transsphenoidal approach rather than craniotomy. Technical advances to make the surgery safer continue to evolve, and include endoscopic approaches, computer-guided image-based intraoperative visualization, and intraoperative magnetic resonance imaging. Criteria for satisfactory remission of acromegaly after surgery are the same as those used for medical management. They include normal insulin-like growth factor (IGF)-I and suppression of GH to undetectable levels (<1.0 ng/ml) during an oral glucose tolerance test (OGTT). Data from a recent series of 86 patients operated upon for acromegaly at the University of Virginia and followed for more than 1 year have been reviewed. In patients receiving surgery as the initial procedure, 67% had a normal IGF-I, and 52% suppressed to <1.0 ng/ml in an OGTT. There was one true recurrence of disease diagnosed 81 months after surgery. Results are best in patients with noninvasive microadenomas. Gamma knife radiosurgery has been a valuable adjunct in those patients who fail to achieve postoperative remission. Pathological evaluation of the tumors revealed that 16% expressed GH only, 25% stained for GH and glycoprotein hormones (follicle stimulating hormone, thyroid hormone, thyroid stimulating hormone, alpha-subunit), 21% for GH and PRL, and 33% for GH, PRL and glycoprotein hormones. There was one acidophil stem cell tumor and 10% had the mammosomatotroph subtype. This contemporary series was free of mortality or serious complications. One patient had a transient cerebrospinal fluid leak and 3 developed transient SIADH with hyponatremia. Surgical treatment remains an important aspect of the combined management of patients with acromegaly.
Acromegaly↗
Intrasellar chordomas mimicking pituitary adenoma.
OBJECT: Whereas chordomas involving the sellar region are uncommon, largely or entirely intrasellar examples are rare. The goal in this study was to present examples of these rare tumors as a guide to their proper diagnosis and treatment. METHODS: The authors report three cases in which the chordomas filled the pituitary fossa and presented as nonfunctioning pituitary adenomas. All lesions exhibited the typical histological patterns and immunophenotype of chordoma. One tumor, studied ultrastructurally and subjected to DNA analysis, was shown to have a diploid histogram. The authors present a clinicopathological study of these three cases and review the literature on intrasellar chordomas. CONCLUSIONS: Although these tumors are easily misdiagnosed and therefore may not receive optimal treatment, aggressive surgical resection can yield a favorable prognosis in lesions with a limited extent.
Adenoma↗
Radiosurgery for Cushing's disease after failed transsphenoidal surgery.
OBJECT: Although transsphenoidal surgery has become the standard of care for Cushing's disease, it is often unsuccessful in normalizing cortisol production. In this study the authors investigate the safety and efficacy of gamma knife radiosurgery (GKRS) for Cushing's disease after failed transsphenoidal surgery. METHODS: The records of all patients who underwent GKRS at the authors' institution after unsuccessful transsphenoidal surgery for Cushing's disease were retrospectively reviewed. Successful treatment was considered a normal or below-normal 24-hour urinary free cortisol (UFC) level. Records were also evaluated for relapse, new-onset endocrine deficiencies, interval change in tumor size, and visual complications. Forty-three patients underwent 44 gamma knife procedures with follow up ranging from 18 to 113 months (mean 39.1, median 44 months). Normal 24-hour UFC levels were achieved in 27 patients (63%) at an average time from treatment of 12.1 months (range 3-48 months). Three patients had a recurrence of Cushing's disease at 19, 37, and 38 months, respectively, after radiosurgery. New endocrine deficiencies were noted in seven patients (16%). Follow-up magnetic resonance images obtained in 33 patients revealed a decrease in tumor size in 24, no change in nine, and an increase in size in none of the patients. One patient developed a quadrantanopsia 14 months after radiosurgery despite having received a dose of only 0.7 Gy to the optic tract. CONCLUSIONS: Gamma knife radiosurgery appears to be safe and effective for the treatment of Cushing's disease refractory to pituitary surgery. Delayed recurrences and new hormone deficiencies may occur, indicating the necessity for regular long-term follow up.
Adenoma↗
Conventional radiotherapy for pituitary tumors.
Radiosurgery for pituitary adenomas is clearly attaining a more prominent position in the spectrum of management of these lesions. It is our belief that improvements in radiosurgical technology can significantly enhance the outcome for patients with these lesions.
Adenoma↗
The decade of the brain: 1990 to 2000.
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Animals↗
Case problems conference: Cushing's disease and parasellar aneurysm.
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Adult↗
From child to adult.
This essay discusses a number of aspects of the neurosurgical problems that occur as a child progresses into adulthood. Some typical disorders may not present until a patient reaches adulthood ("pediatric" brain tumors, hydrocephalus, Arnold-Chiari malformation). The neurosurgeon will be confronted with disorders of maturation - children who are prematurely adult and adults who remain childlike. A variety of neurological disorders may present with these disorders of maturation; and they are discussed. Finally, the neurosurgeon must be prepared to follow-through for adults who have been treated for neurological problems when they were children. These include patients with shunts for hydrocephalus, patients with spinal deformities, and patients with secondary effects of adjunctive therapy.
Adolescent↗
Small cell lung carcinoma causing epidural hematoma: case report.
BACKGROUND: Epidural hematoma usually stems from trauma, yet it may occur from other etiologies, including neoplasms. We present a case of small cell lung carcinoma with focal hemorrhagic central nervous system metastasis producing an epidural hematoma, and review the associated literature. CLINICAL PRESENTATION: A 67-year-old man was undergoing chemotherapy for small cell carcinoma of the lung. Acute neurologic deterioration resulted from a large parietal epidural hematoma of non-traumatic origin. INTERVENTION: The clot was evacuated via craniotomy with marked improvement in his clinical state. Metastatic tumor was present in the scalp, muscle, bone, and dura. No gross brain invasion was apparent. CONCLUSIONS: This case illustrates the wisdom of including metastatic disease in the differential diagnosis of intracranial hemorrhage. Even epidural hematoma may result from metastatic cancer. The prevalence of lung carcinoma and the aging of the population may produce an increased appearance of this phenomenon. Appropriate evaluation and rapid intervention will aid the patient in both the acute and long-term phases, and should improve the quality of survival.
Aged↗
Third ventricular lesion masquerading as suprasellar disease.
We discuss the case of a patient who presented with a bitemporal visual field disturbance thought to arise from chiasmatic compression secondary to a suprasellar mass. The patient was ultimately diagnosed with medulloblastoma with diffuse intraventricular disease. Careful review of magnetic resonance (MR) findings in this case demonstrate the apparent suprasellar mass to be within the suprachiasmatic recess of the third ventricle. The role of MR imaging in distinguishing between suprasellar disease involving the third ventricle and primary third ventricular lesions is discussed.
Adult↗