Pituitary adenomas in childhood and adolescence

1994 ◽  
Vol 80 (2) ◽  
pp. 209-216 ◽  
Author(s):  
Michael D. Partington ◽  
Dudley H. Davis ◽  
Edward R. Laws ◽  
Bernd W. Scheithauer

✓ Pituitary adenomas are uncommon in childhood. Between 1975 and 1988, 36 patients underwent trans-sphenoidal resection at the Mayo Clinic before their 17th birthday. Fifteen (41.7%) had prolactin (PRL)-secreting tumors, 16 (44.4%) had adrenocorticotropic hormone (ACTH)-secreting tumors (including two patients with Nelson-Salassa syndrome), and three (8.3%) had tumors secreting growth hormone (GH); two patients (5.6%) had clinically nonfunctioning tumors. Of the 36 patients, 21 (58.3%) were girls, and girls predominated in every group except that with ACTH-secreting tumors, where they accounted for only 37.5% of the patients. The average age at the time of surgery was 14.7 ± 2.2 years, the youngest patient being 7.3 years old. The ACTH-secreting tumors were associated with an earlier onset of symptoms than the other tumor types: six (37.5%) of 16 patients became symptomatic before 10 years of age, compared with two (10.0%) of the 20 other patients. Presenting symptoms primarily reflected endocrine dysfunction, with neurological presentation (visual field deficits) occurring in only two patients. There were no deaths in this patient group. Significant operative morbidity included steroid-induced psychosis in one patient and diabetes insipidus in three, which resolved in two. Plurihormonal tumors, broadly defined as tumors producing more than one hormone, were common, comprising five (36%) of 14 PRL-secreting tumors, all three GH-secreting tumors, and one of two clinically nonfunctioning tumors, or 25.7% overall. Macroadenomas accounted for 89% of plurihormonal tumors. Long-term follow-up review (median > 5 years) revealed good control of PRL-secreting tumors (although five of 15 patients had received postoperative radiotherapy), contrasted with a 25% late recurrence rate for ACTH-secreting tumors, which had an 80% initial remission rate. The majority of patients had good endocrine function, including reproductive function. It is concluded that: 1) although pediatric pituitary adenomas occur primarily in adolescence, Cushing's disease is found at any age; 2) transsphenoidal surgery is feasible and safe in this age group; 3) plurihormonal tumors occur more frequently in the pediatric age group than in adults; and 4) long-term control rates in PRL- and ACTH-secreting tumors are probably similar to those seen in adults.

2005 ◽  
Vol 102 (Special_Supplement) ◽  
pp. 119-123 ◽  
Author(s):  
Tatsuya Kobayashi ◽  
Yoshimasa Mori ◽  
Yukio Uchiyama ◽  
Yoshihisa Kida ◽  
Shigeru Fujitani

Object. The authors conducted a study to determine the long-term results of gamma knife surgery for residual or recurrent growth hormine (GH)—producing pituitary adenomas and to compare the results with those after treatment of other pituitary adenomas. Methods. The series consisted of 67 patients. The mean tumor diameter was 19.2 mm and volume was 5.4 cm3. The mean maximum dose was 35.3 Gy and the mean margin dose was 18.9 Gy. The mean follow-up duration was 63.3 months (range 13–142 months). The tumor resolution rate was 2%, the response rate 68.3%, and the control rate 100%. Growth hormone normalization (GH < 1.0 ng/ml) was found in 4.8%, nearly normal (< 2.0 ng/ml) in 11.9%, significantly decreased (< 5.0 ng/ml) in 23.8%, decreased in 21.4%, unchanged in 21.4%, and increased in 16.7%. Serum insulin-like growth factor (IGF)—1 was significantly decreased (IGF-1 < 400 ng/ml) in 40.7%, decreased in 29.6%, unchanged in 18.5%, and increased in 11.1%, which was almost parallel to the GH changes. Conclusions. Gamma knife surgery was effective and safe for the control of tumors; however, normalization of GH and IGF-1 secretion was difficult to achieve in cases with large tumors and low-dose radiation. Gamma knife radiosurgery is thus indicated for small tumors after surgery or medication therapy when a relatively high-dose radiation is required.


2000 ◽  
Vol 93 (2) ◽  
pp. 194-200 ◽  
Author(s):  
Naoko Sanno ◽  
Akira Teramoto ◽  
R. Yoshiyuki Osamura

Object. Thyrotropin-secreting pituitary adenomas are rare lesions of the endocrinological system. Although introduction of a hypersensitive radioimmunoassay for thyrotropin enables the recognition of inappropriate secretion of this hormone, the aforementioned lesions remain uncommon and unfamiliar to most neurosurgeons. It has been reported previously that surgical cure of thyrotropin-secreting adenomas is more difficult than in other functional adenomas because of the large size and invasive features of the former. However, the long-term outcome after surgery has not been well documented. The authors report on a surgical series of 16 patients with thyrotropin adenoma and the results of long-term follow up.Methods. Sixteen patients ages 23 to 62 years (12 women and four men) underwent transsphenoidal removal of thyrotropin adenomas between 1983 and 1999. These patients had the syndrome of inappropriate thyrotropin secretion (SITS) with pituitary mass lesions. Four of the patients had undergone previous subtotal thyroidectomy and/or radioiodine thyroid ablation, and 11 had been treated with antithyroid medication. Radiological investigations demonstrated macroadenomas in 14 patients, and 10 of those had cavernous sinus invasion. Surgical findings showed unusually fibrous and firm tumors in 13 (81.2%) of 16 patients. Preoperative octreotide administration was revealed to be effective for serum thyrotropin reduction as well as tumor shrinkage. Transsphenoidal surgery was performed with no morbidity resulting. Surgical remission was achieved in 10 (62.5%) of 16 patients, and total remission was achieved in 14 patients (87.5%) with a combination of additional radiation or medical therapy. In the other two patients, SITS persisted because of tumor rests in the cavernous sinus. Therefore, radiation and/or antithyroid therapy was administered. In the mean follow-up period of 7.5 years (range 11 months–15.8 years), no recurrence of tumor was observed on magnetic resonance images, whereas recurrence of SITS was found in two patients with no tumor regrowth. In addition, coexistent primary hyperthyroidism was found in two other patients despite remission of SITS after surgery.Conclusions. Transsphenoidal surgery can achieve a good long-term outcome in patients with thyrotropin-secreting pituitary adenomas if surgery is performed before these become larger, invasive tumors. In the authors' experience, thyrotropin-secreting adenomas are fibrous and firm, which makes it difficult to achieve surgical remission. In addition, even satisfactory resection of the tumor sometimes results in recurrence of SITS or hyperthyroid symptoms due to coexistent primary hyperthyroidism. It is emphasized that a careful follow-up review is necessary after surgery, especially in patients with a long preoperative history of hyperthyroidism.


1998 ◽  
Vol 89 (3) ◽  
pp. 353-358 ◽  
Author(s):  
Pamela U. Freda ◽  
Sharon L. Wardlaw ◽  
Kalmon D. Post

Object. The results of surgical therapy for acromegaly were assessed using carefully conducted endocrinological testing in 115 patients who underwent transsphenoidal surgery from 1981 to 1995. Methods. Ninety-nine of the 115 patients could be contacted for follow-up review; in 57 of the patients an endocrinological assessment was performed by the authors. Biochemical cure was strictly defined as a normalization of the insulin-like growth factor(IGF)—I level (obtained in 100 patients) and/or a basal or glucose-suppressed growth hormone (GH) level of 2 ng/ml or less. The mean length of follow up from transsphenoidal surgery to laboratory testing was 5.4 years with a maximum of 15.7 years. After transsphenoidal surgery alone, 61% of the patients achieved a biochemical remission; the remission rate was 88% for patients with microadenomas and 53% for those with macroadenomas. Tumor size and preoperative GH level tended to correlate negatively with outcome of surgery. Early postoperative GH level tended to correlate with long-term outcome; in cases in which the early postoperative GH level was lower than 3 ng/ml, the chance of long-term remission was 89%. Thirty-two patients received postoperative radiotherapy: in 10 (31%) of these patients the disease is currently in remission after surgery and radiotherapy only and in three others the disease is in remission with the addition of medical therapy. The overall complication rate was 6.9% with no cerebrospinal fluid leaks, meningitis, permanent diabetes insipidus, or new hypopituitarism. The overall recurrence rate was low at 5.4%. Conclusions. This series shows, based on IFGF—I measurements and strict GH supression criteria to define remission, that transsphenoidal surgery provides an excellent chance for long-term cure in patients with microadenomas. Surgery alone is successful in most patients with noninvasive macroadenomas; however, most patients with invasive macroadenomas will require adjunctive therapy. Recurrences are uncommon when biochemical remission is clearly documented postoperatively.


2005 ◽  
Vol 102 (4) ◽  
pp. 650-657 ◽  
Author(s):  
Indro Chakrabarti ◽  
Arun P. Amar ◽  
William Couldwell ◽  
Martin H. Weiss

Object. The authors report on a cohort of patients with craniopharyngioma treated principally through transnasal (TN) resection and followed up for a minimum of 5 years. More specifically, they evaluate the role of the TN approach in the management of craniopharyngioma. Methods. Between 1984 and 1994, 68 patients underwent TN resection of craniopharyngiomas at the University of Southern California. The tumor was at least partially cystic in 88% of cases. Four tumors were purely intrasellar, 53 had intra- and suprasellar components, and 11 were exclusively suprasellar. During the same period, 18 patients underwent transcranial (TC) resection of purely suprasellar craniopharyngiomas. Long-term neurological, visual, and endocrine outcomes were reviewed for all patients. In 61 (90%) of 68 patients in the TN group, total resection was achieved, according to 3-month postoperative magnetic resonance images, although four patients suffered a recurrence. Three (43%) of the seven tumors that had been partially resected were enlarged on serial imaging. Fifty-four (87%) of 62 patients with preoperative visual loss experienced improvement in one or both eyes, but two patients (3%) with exclusively suprasellar tumors experienced postoperative visual worsening in one or both eyes. New instances of postoperative endocrinopathy (that is, not present preoperatively) occurred as follows: hypogonadism (eight of 22 cases), growth hormone (GH) deficiency (four of 18 cases), hypothyroidism (11 of 49 cases), hypocortisolemia (nine of 52 cases), and diabetes insipidus (DI; four of 61 cases). One case each of hypocortisolemia and hypothyroidism resolved after surgery. Hyperphagia occurred in 27 (40%) of 68 patients. One patient had short-term memory loss. Postoperative complications included one case of cerebrospinal fluid leak. Among the 18 patients in the TC group, 11 had complete resections. In one case (9%) the tumors recurred. Three (43%) of the seven subtotally resected tumors grew during the follow-up interval. Vision improved in 11 (61%) of 18 cases and worsened in three (17%) as a result of surgery. New instances of postoperative endocrinopathy occurred as follows: hypogonadism (one of six cases), GH deficiency (four of seven cases), hypothyroidism (11 of 14 cases), hypocortisolemia (eight of 15 cases), and DI (nine of 16 cases). No instance of preoperative endocrinopathy was corrected through TC surgery. Four patients (22%) exhibited short-term memory loss and 11 (61%) had hyperphagia after surgery. When compared with those in the TC group, patients in the TN group had shorter hospital stays. Conclusions. Use of the TN approach can render good outcomes in properly selected patients with craniopharyngioma, particularly when the tumor is cystic. Even in mostly suprasellar cases, an extended TN approach can afford complete resection. Note that endocrine function often worsens after surgery and that postoperative obesity can be a significant problem.


1989 ◽  
Vol 70 (3) ◽  
pp. 420-425 ◽  
Author(s):  
Fredric B. Meyer ◽  
Thoralf M. Sundt ◽  
Nicolee C. Fode ◽  
Michael K. Morgan ◽  
Glen S. Forbes ◽  
...  

✓ In this study, 24 aneurysms occurring in 23 patients under the age of 18 years (mean 12 years) are analyzed. The male:female ratio was 2.8:1, and the youngest patient was 3 months old. Mycotic lesions and those associated with other vascular malformations were excluded. Forty-two percent of the aneurysms were located in the posterior circulation, and 54% were giant aneurysms. Presenting symptoms included subarachnoid hemorrhage in 13 and mass effect in 11. Several of these aneurysms were documented to rapidly increase in size over a 3-month to 2-year period of observation. All aneurysms were surgically treated: direct clipping was performed in 14; trapping with bypass in four; trapping alone in four; and direct excision with end-to-end anastomosis in two. The postoperative results were excellent in 21 aneurysms (87%), good in two (8%), and poor in one. The pathogenesis of cerebral aneurysms is reviewed.


1986 ◽  
Vol 64 (5) ◽  
pp. 713-719 ◽  
Author(s):  
Michael J. Ebersold ◽  
Lynn M. Quast ◽  
Edward R. Laws ◽  
Berndt Scheithauer ◽  
Raymond V. Randall

✓ Little has been written about the long-term results of transsphenoidal treatment for clinically nonfunctioning pituitary adenomas. The records of 100 patients who had undergone a transsphenoidal procedure for excision of such tumors were reviewed. Immunocytology for pituitary hormones was performed in all cases. The group consisted predominantly of null-cell adenomas, although a small number of prolactinomas and gonadotropic tumors were found. The mean diameter of the tumors at the time of detection was slightly more than 2 cm. In most cases, the presenting symptoms were due to the mass effect of the tumor (that is, visual symptoms in 72 patients, hypopituitarism in 61, headache in 36, and cranial nerve disturbance other than visual loss in 10). Radiation therapy was recommended for patients in whom subtotal removal of the adenoma was expected. Six patients developed symptomatic tumor recurrence, and 10 patients demonstrated radiographic recurrence during the 48 to 100 months (mean 73.4 months) of follow-up observation. Only three of 10 deaths during the follow-up period were due to pituitary disease or treatment.


1975 ◽  
Vol 43 (4) ◽  
pp. 437-439 ◽  
Author(s):  
Humberto Ortiz-Suarez ◽  
Donald L. Erickson

✓ A review of four cases of chromophobe adenomas in adolescents suggests that extracapsular extension with invasion of parasellar structures is more common in this age group than in adults. A syndrome of adolescent obesity, oculomotor palsies, and plain x-ray changes of the sella characterizes this group.


1998 ◽  
Vol 89 (1) ◽  
pp. 60-68 ◽  
Author(s):  
Richard B. Schwartz ◽  
B. Leonard Holman ◽  
Joseph F. Polak ◽  
Basem M. Garada ◽  
Marc S. Schwartz ◽  
...  

Object. The study was conducted to determine the association between dual-isotope single-photon emission computerized tomography (SPECT) scanning and histopathological findings of tumor recurrence and survival in patients treated with high-dose radiotherapy for glioblastoma multiforme. Methods. Studies in which SPECT with 201Tl and 99mTc-hexamethypropyleneamine oxime (HMPAO) were used were performed 1 day before reoperation in 47 patients with glioblastoma multiforme who had previously been treated by surgery and high-dose radiotherapy. Maximum uptake of 201Tl in the lesion was expressed as a ratio to that in the contralateral scalp, and uptake of 99mTc-HMPAO was expressed as a ratio to that in the cerebellar cortex. Patients were stratified into groups based on the maximum radioisotope uptake values in their tumor beds. The significance of differences in patient gender, histological characteristics of tissue at reoperation, and SPECT uptake group with respect to 1-year survival was elucidated by using the chi-square statistic. Comparisons of patient ages and time to tumor recurrence as functions of 1-year survival were made using the t-test. Survival data at 1 year were presented according to the Kaplan—Meier method, and the significance of potential differences was evaluated using the log-rank method. The effects of different variables (tumor type, time to recurrence, and SPECT grouping) on long-term survival were evaluated using Cox proportional models that controlled for age and gender. All patients in Group I (201Tl ratio < 2 and 99mTc-HMPAO ratio < 0.5) showed radiation changes in their biopsy specimens: they had an 83.3% 1-year survival rate. Group II patients (201T1 ratio < 2 and 99mTc-HMPAO ratio of ≥ 0.5 or 201Tl ratio between 2 and 3.5 regardless of 99mTc-HMPAO ratio) had predominantly infiltrating tumor (66.6%); they had a 29.2% 1-year survival rate. Almost all of the patients in Group III (201Tl ratio > 3.5 and 99mTc-HMPAO ratio ≥ 0.5) had solid tumor (88.2%) and they had a 6.7% 1-year survival rate. Histological data were associated with 1-year survival (p < 0.01); however, SPECT grouping was more closely associated with 1-year survival (p < 0.001) and was the only variable significantly associated with long-term survival (p < 0.005). Conclusions. Dual-isotope SPECT data correlate with histopathological findings made at reoperation and with survival in patients with malignant gliomas after surgical and high-dose radiation therapy.


2005 ◽  
Vol 102 (1) ◽  
pp. 6-9 ◽  
Author(s):  
Simone A. Betchen ◽  
Jane Walsh ◽  
Kalmon D. Post

Object. Vestibular schwannomas (VSs) are now amenable to resection with excellent hearing preservation rates. It remains unclear whether immediately postoperative hearing is a durable result and will not diminish over time. The aim of this study was to determine the rate of long-term preservation of functional hearing following surgery for a VS and to examine factors influencing hearing preservation. Methods. All patients eligible for hearing preservation (Gardner—Robertson Class I or II) who had undergone resection of a VS by a single surgeon were reviewed retrospectively. Follow-up audiograms and magnetic resonance images were obtained. Of 142 patients deemed eligible for hearing preservation surgery, 38 had immediate postoperative hearing confirmed by an audiogram. In these patients with preserved hearing, the audiographic results demonstrated functional hearing in 30 (85.7%) of 35 patients who underwent repeated testing at a mean follow-up time of 7 years. Delayed hearing loss occurred in five (14.3%) of the 35 patients and did not correlate significantly with the size of the tumor. Hearing improved one Gardner—Robertson class postoperatively in three (7.9%) of the 38 patients. Conclusions. Long-term functional hearing was maintained in 85.7% of patients when it was preserved immediately postoperatively and the result was independent of tumor size. The results of this study emphasize that long-term preservation of functional hearing is a realistic goal following VS surgery and should be attempted in all patients in whom preoperative hearing is determined to be Gardner—Robertson Class I or II.


2002 ◽  
Vol 97 (1) ◽  
pp. 3-11 ◽  
Author(s):  
Rémy Van Effenterre ◽  
Anne-Laure Boch

Object. This work is devoted to a 25-year retrospective study of 122 cases of craniopharyngiomas in adults and children treated and followed by the same neurosurgeon (R.V.E.). In this homogeneous series, the aim was total microsurgical removal of the tumor, without postoperative radiotherapy. Methods. The operation was performed via a frontopterional approach in 112 cases and a transsphenoidal approach in 10 cases. The tumor removal was considered total in 59%, subtotal in 29%, and partial in 12%. The surgical mortality rate was 2.5%. Even when tumor removal was not complete, radiotherapy was not systematically administered; it was reserved for cases of recurrence. The authors have studied clinical signs, operative characteristics, and ophthalmological, endocrinological, and functional outcomes, as well as recurrence risk and long-term patient survival. The mean follow-up period was 7 years. The functional results in these patients were excellent in 85%, good in 9%, fair in 5% (usually because of ophthalmological sequelae), and poor in 1%. Tumors recurred in 29 patients, but the salvage treatment, by operation or radiotherapy, was successful in 83%. The actuarial patient survival rate was 92% after 5 years and 85% after 10 years. Conclusions. These results compared favorably with the data reported in the literature, suggesting that radical surgery of craniopharyngiomas allows good outcome in terms of survival, full recovery, and quality of life for both adults and children.


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