Stereotactic Radiosurgery with the Cobalt-60 Gamma Unit in the Treatment of Growth Hormone-Producing Pituitary Tumors

Neurosurgery ◽  
1991 ◽  
Vol 29 (5) ◽  
pp. 663-668 ◽  
Author(s):  
Marja Thorén ◽  
Tiit Rähn ◽  
Guo Wan-Yuo ◽  
Werner Sigbritt

Abstract Stereotactic radiosurgery on the pituitary given with the cobalt-60 gamma unit was used in the treatment of 21 patients with growth hormone (GH)-producing pituitary adenomas and acromegaly. All but one patient had locally invasive macroadenomas, and in the majority of cases, there was parasellar growth. Radiosurgery was the initial treatment for seven patients. Fourteen patients were previously treated by pituitary surgery, eight of whom had undergone conventional external pituitary irradiation as well. All patients had clinical signs of active acromegaly before radiosurgery. The radiation doses given to the previously untreated patients or those who only underwent surgery were 40 to 70 Gy in each of one to three irradiations. The patients with previous external irradiation received a lower dose of 30 to 50 Gy in each of one or two irradiations. The patients were observed during a period of 1 to 21 years from the first radiosurgical session. Two young patients had a clinical remission with a substantial decline of GH levels to near normal serum profiles. Another eight patients obtained reduction of GH levels and clinical activity. More than half of the patients (11 of 21) had minor or no effects from the treatment. There were no complications from the radiosurgery except the development of pituitary insufficiency in 2 of 13 patients who did not undergo previous conventional external irradiation. The remission rates were lower than the results previously reported by us for radiosurgery for Cushing's disease. This may be a result of the predominance in the present study of invasive macroadenomas and single treatments and to the lower radiation doses used in the patients who underwent conventional irradiation previously. In the future, the effect of higher radiation doses will be evaluated. The presently available stereotactic magnetic resonance imaging with contrast enhancement, which improves the visualization of the target volume, may aid in improving future radiosurgical results in the treatment of GH-producing pituitary tumors.

Neurosurgery ◽  
2001 ◽  
Vol 49 (2) ◽  
pp. 284-292 ◽  
Author(s):  
Charlotte Höybye ◽  
Eva Grenbäck ◽  
Tiit Rähn ◽  
Marie Degerblad ◽  
Marja Thorén ◽  
...  

Abstract OBJECTIVE To study retrospectively long-term outcomes of patients with adrenocorticotropic hormone-producing pituitary tumors that were treated with stereotactic Leksell gamma knife unit radiosurgery. METHODS Eighty-nine patients aged 5 to 67 years were treated between 1976 and 1985. Eighteen patients aged 18 to 68 years (mean age, 41 yr) were followed in detail. Fifteen patients were women. None had previously received conventional radiotherapy, but pituitary microsurgery had been performed in two patients, and one patient had had an adrenalectomy. In the remaining 15 patients, radiosurgery was the primary therapy. RESULTS Sixty-four patients had one stereotactic treatment, and 25 patients had two or more treatments. No complications were observed during treatment and the immediate follow-up period. At follow-up, 17 patients had died 1 to 20 years after the first treatment. No deaths were related to the treatment. In our 18 patients, the follow-up time after the first radiosurgical treatment was 12 to 22 years (mean follow-up period, 17 yr). Urinary cortisol levels gradually normalized in 83% of the patients. No recurrences were observed. Pituitary hormone insufficiencies developed in about two of every three patients and occurred even more than 10 years after treatment. Eight patients had transient hyperprolactinemia. The patients' vision and visual fields were unaffected, and none of them had signs of radiation-induced side effects such as brain tumors or brain necrosis. CONCLUSION Stereotactic radiosurgery is a safe and effective method in the treatment of patients with adrenocorticotropic hormone-producing pituitary tumors, and the effect of treatment is long-lasting. Stereotactic radiosurgery is mainly a complement to microsurgery because of its gradually appearing effect and the occurrence of pituitary insufficiency. New pituitary deficiencies may be found more than 10 years after treatment.


2016 ◽  
Vol 19 (1) ◽  
pp. 48-56 ◽  
Author(s):  
Maria C Jugan ◽  
John R August

Objectives The aim of the study was to evaluate ultrasonographic changes in the small intestine of cats with clinical signs of gastrointestinal disease and low or low–normal serum cobalamin concentrations. Methods Records for client-owned cats presenting to the small animal hospital with signs of gastrointestinal disease and in which serum cobalamin concentrations were measured from 2000–2013 were reviewed. Inclusion criteria were cobalamin concentrations <500 ng/l, abdominal ultrasound within 1 month of cobalamin testing and definitive diagnosis. Results Of 751 serum cobalamin measurements, hypocobalaminemia or low–normal cobalamin was identified in 270 cats, abdominal ultrasound was performed in 207 of those cats and a diagnosis was available for 75 of them. Small intestinal ultrasound changes were detected in 49/75 (65%) cats. Abnormalities included thickening, loss of wall layer definition, echogenicity alterations and discrete masses. Serum cobalamin concentrations <500 ng/l were observed with diagnoses of inflammatory disease, neoplasia, infectious disease and normal histopathology. Cobalamin concentration was significantly lower in cats with lymphoma or inflammatory bowel disease compared with other gastrointestinal neoplasia ( P = 0.031). No difference was found between cobalamin concentration and the presence of ultrasound abnormalities, specific ultrasound changes or albumin concentration. Conclusions and relevance One-third of symptomatic cats with hypocobalaminemia or low–normal cobalamin concentrations may have an ultrasonographically normal small intestine. For the majority of cats in this study, histopathologic abnormalities were observed in the small intestine, regardless of ultrasound changes. These findings suggest gastrointestinal disease should not be excluded based on low–normal cobalamin concentrations, even with a concurrent normal ultrasound examination. Additional studies are needed in cats with low–normal serum cobalamin concentrations, as a definitive diagnosis was not pursued consistently in those cats. However, data from this study suggest that careful monitoring, histopathologic evaluation and future cobalamin supplementation may be warranted.


2020 ◽  
Author(s):  
Marcin Adamczak ◽  
Piotr Kuczera ◽  
Andrzej Wiecek

Kidneys play the major role in the synthesis and degradation of several hormones. Different coexisting conditions such as inflammation, malnutrition and metabolic acidosis and applied treatment may also cause endocrine abnormalities in chronic kidney disease (CKD) patients. A tendency towards decreased thyroxin and triiodothyronine with normal serum concentrations of reversed triiodothyronine (as opposed to other chronic non-thyroid, non-kidney diseases) and thyroid stimulating hormone are observed. As far as the somatotopic axis is concerned, in CKD normal serum concentration of growth hormone and its effector – the insulin-like growth factor are observed. Nevertheless, due to the phenomenon of GH/IGF-1 “resistance” CKD patients usually present a phenotype resembling GH deficiency. Serum prolactin concentrations are often elevated in CKD women and men. This leads to the dysregulation of the pituitary-gonadal axis causing hypogonadism and it’s clinical consequences regardless of patient’s gender. The alterations in hormones of gonadal origin caused by uremia, together with hyperprolactinemia lead to the development of sexual dysfunction and infertility in men and women. The alterations of thyroid, pituitary gland and gonads associated with CKD are discussed in this chapter. This review contains 4 tables, and 64 references. Keywords: chronic kidney disease, hypothyroidism, hyperthyroidism, growth hormone, recombinant human GH, insulin-like growth factors, hemodialysis


2013 ◽  
Vol 09 (01) ◽  
pp. 66
Author(s):  
Sanne E Franck ◽  
Aart Jan van der Lely ◽  
Sebastian Neggers ◽  
◽  
◽  
...  

After the introduction of somatostatin analogs (LA-SMSA) and the growth hormone (GH) receptor antagonist, pegvisomant (Peg-v) normal serum insulin-like growth factor-1 (IGF-1) concentrations in virtually every patients with acromegaly is possible. The impact of these products on the GH–IGF1 axis is completely different. We advocate that LA-SMSA may normalize serum IGF1 levels in the presence of elevated GH actions in extrahepatic tissues. This results in persistent peripheral disease activity that we call ‘extra-hepatic acromegaly’. Peg-v competitively blocks systemic GH action and results in a GH serum level increase. Therefore high doses of Peg-v are necessary to control IGF-1. Since the mode of action differs between these products, it is questionable if identical IGF-1 levels, during Peg-v or LA-SMSA are really identical representations of the biochemical situation. With the traditional biomarkers medical treatment is therefore difficult to monitor with the traditional biomarkers. Additionally, Peg-v and LA-SMSA could be ideal combination since they have different mode of actions. We believe that the time has come to challenge the existing concepts of treatment and monitoring of patients with acromegaly.


2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A263-A263
Author(s):  
Kathryn Dahir ◽  
Eric Rush ◽  
Daniel Beltran ◽  
Scott Eisenbeis ◽  
Britt Johnson ◽  
...  

Abstract X-linked hypophosphatemia (XLH), a dominant disorder caused by a disease-associated variant in the PHEX gene, affects males and females of all ages. Rickets and osteomalacia may be present along with short stature, lower limb deformity, muscle pain and/or weakness/fatigue, bone pain, joint pain/stiffness, hearing difficulty, enthesopathy, osteoarthritis, and dental abscesses. Patients with XLH have below-normal serum phosphate and elevated serum FGF23. XLH is one of multiple etiologies of hypophosphatemia; depending on genetic cause, management may differ. Acquired hypophosphatemia (e.g. tumor induced osteomalacia) is non-hereditary in nature. This program provides a no-charge genetic test to confirm a clinical XLH diagnosis or to aid suspected genetic hypophosphatemia diagnosis. Patients aged &gt;/= 6 months with either a clinical XLH diagnosis or suspicion of genetic hypophosphatemia, as evidenced by 2 or more clinical signs/ symptoms, were eligible for testing. The next generation sequencing panel includes 13 genes: ALPL, CLCN5, CYP2R1, CYP27B1, DMP1, ENPP1, FAH, FAM20C, FGF23, FGFR1, PHEX, SLC34A3 and VDR. Copy number variant detection was performed. 831 unrelated individuals were tested as of June 30, 2020. 569 (68.5%) of these subjects had a PHEX variant: 519 (91.2%) were either pathogenic or likely pathogenic (P/LP) and 50 (8.8%) were variants of uncertain significance (VUS). Of the 312 (37.5%) cases where no PHEX molecular diagnosis was found, 38 (12.2%) had molecular diagnoses associated with other genes/disorders: 4 had a variant (P/LP) in FGF23 (autosomal dominant [AD] hypophosphatemic rickets), 2 had two variants (P/LP) in CYP27B1 (autosomal recessive [AR] vitamin D dependent rickets), 1 had P/LP variants in ENPP1 (AR hypophosphatemic rickets Type 2). There were 27 cases with single P or LP variants in ALPL (AD hypophosphatasia, HPP); 4 cases carried two variants (P/LP) in ALPL (AR form). Of 237 unique P/LP PHEX variants detected: 59 were deletions, duplications or insertions; 37 were copy number variants; 52 were splice-site variants; 89 were single nucleotide variants. Additional family member testing/clinical information resulted in 48 cases having VUS reclassified to P/LP, highlighting the value of cascade family testing/clinical info to resolve VUS. RNA analyses to resolve VUS may further improve molecular diagnostic yield. Program results demonstrate a high diagnostic yield for XLH/ genetic hypophosphatemia and new insight into XLH-associated PHEX variants.


2021 ◽  
Vol 62 (11) ◽  
pp. 1459-1464
Author(s):  
Je Sang Lee ◽  
Si Hyung Lee ◽  
Bo Yeon Kim ◽  
Sun Young Jang

Purpose: To study the relationship between the levels of serum thyroid-stimulating hormone (TSH)-receptor autoantibodies (TRAbs) and thyroid eye disease (TED) activity and severity scores.Methods: A cross-sectional study was performed. The medical records of 315 patients diagnosed with TED between March 2014 and December 2019 were reviewed. The clinical activity score (CAS) was used to assess TED activity and a modified NOSPECS score was used for severity grading. The serum TRAb level was measured using two assays: a TSHR binding inhibitory immunoglobulin (TBII) assay and thyroid stimulating immunoglobulin (TSI) bioassay.Results: The TBII and TSI assay results were significantly positively correlated with the CAS (R = 0.113 and 0.211, respectively; p < 0.05), modified NOSPECS score (R = 0.173 and 0.316, respectively; p < 0.05), and proptosis (R = 0.136 and 0.167, respectively; p < 0.05). Both assays demonstrated significant differences in the level of TRAb between patients with and without compressive optic neuropathy or corneal epithelial defects.Conclusions: The levels of TRAbs according to both TBII and TSI assays showed significant correlations with clinical signs of corneal involvement, optic neuropathy, and TED activity and severity.


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