Endocrine Disorders in 66 Suprasellar and Pineal Tumors of Patients with Prepubertal and Pubertal Ages

Abstract

Tumor oncotypes, initial symptoms and endocrine disturbances before and/or 1 month after surgery were studied in 66 patients with prepubertal and pubertal ages having suprasellar or pineal intracranial tumors. Neoplasms found in patients of prepubertal age were: 15 craniopharyngiomas (CRA), 24 neuroepithelial-cell-derived tumors (NEC), 5 germ cell tumors (GERM) and 4 other lesions (OTHER). In patients of pubertal age, there were 7 CRA, 7 pituitary tumors (PIT), 2 NEC, 1 GERM and 1 OTHER. Approximately 90% of patients had visual abnormalities as one of the initial signs and symptoms, while 59% had increased intracranial pressure. Short stature was observed in only 10% of patients. Before surgery, somatotropic function was found to be deficient (by 2 pharmacological tests) in 90-100% of patients with CRA, PIT or GERM and in 40% of patients with NEC. Overt hypothyroidism was found in 5-25% of CRA, NEC or GERM but in 40% of PIT. Abnormal TSH responses to TRH were observed in 64% of CRA and in 29% of NEC. Low basal serum cortisol was found in 21 or 6% of patients with CRA or NEC, but in 100 or 60% of patients with PIT or GERM, respectively. Diabetes insipidus was diagnosed in 13.6% of all patients. Surgery produced few additional disturbances in endocrine function, except for the incidence of diabetes insipidus which was doubled. Gonadotropic deficiency was found in most patients of pubertal age with CRA and PIT. They were readily differentiated by the high prolactin or growth hormone (GH) levels of the latter. This study shows that: (1) CRA and NEC in the prepubertal age group, and CRA and PIT in the pubertal age group were the most frequent tumor oncotypes found; (2) visual abnormalities are the most frequent initial signs; (3) endocrine disturbances are highly frequent before surgery, while surgical intervention had little effect in increasing endocrine morbidity; (4) NEC, even though producing less endocrine dysfunctions than other tumor types, are an important etiology of endocrine sequelae because of their high relative frequency in children with prepubertal age.

Gliomas in the Sellar Turcica Region: A Retrospective Study Including Adult Cases and Comparison with Craniopharyngioma

“…This finding may explain why craniopharyngioma patients had a significantly lower rate of observable endocrine dysfunctions in the patients with altered hormone because GH deficiency is more likely to affect children compared with adults, and most patients in our craniopharyngioma series were adults. Other studies have also confirmed GH as the most frequently affected hormone in craniopharygioma [29,30]. …”

The Serum Growth Hormone (GH) Response to Provocative Tests Is Dependent on Type of Assay in Autosomal Dominant Isolated GH Deficiency because of an ARG183HIS (R183H) GH-I Gene Mutation

“…Although we used stimulation tests that are weak for adults (17 ), it is accepted that they are informative tests in children (18 ). We have used these tests in children for more than 20 years with good results (11 ). We have found that our patients responded normally to stimulation tests when total GH was determined but below normal when 22-kDa GH or bioactive GH was determined.…”

“…We propose designating SER 66/217 serum values as serum "total" GH and DELFIA 80/505 values as serum 22-kDa GH. We studied GH secretion, using pharmacologic stimulation with arginine and clonidine, as described previously (11 ) Serum GH bioactivity was analyzed in the Nb2 bioassay according to a modification of the method published by Radetti et al (12 ), using the CellTiter 96 ® AQ ueous Non-Radioactive Cell Proliferation Assay (Promega Corporation).…”

“…The recycling of folate cofactors is dependent on vitamin B 6 and B 2 , and vitamin B 2 is necessary for activating vitamin B 6 to pyridoxal 5Ј-phosphate (PLP) (9 ). Vitamin B 2 and B 6 status thus may have an impact on tHcy concentrations in patients taking AEDs, but few studies have reported on this matter (3,4,10,11 ). The methionine loading test detects individuals with impaired homocysteine metabolism not detected by fasting Hcy concentrations alone and is often considered primarily a test of the transsulfuration pathway (1,8,12 ).…”

Differences in Serum GH Cut-Off Values for Pharmacological Tests of GH Secretion Depend on the Serum GH Method

“…Conversion factors have been proposed to calculate equivalentvalues between polyclonal and monoclonal assays for serum concentrationsin adults and children. GH secretion was studied, using pharmacologicstimulation with arginine and clonidine, as described previously[12]. …”