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Introduction: Up to 5% of all pituitary tumors are hereditary e.g. due to MEN1 or aryl hydrocarbon receptor-interacting protein ( AIP ) genes mutations., Objectives: The study was aimed at the assessment of the frequency and characteristics of AIP -mutation related tumors in patients with apparently sporadic pituitary macroadenomas in the Polish population., Materials and Methods: The study included 131 patients (57 males, 74 females; median age 42 years) diagnosed with pituitary macroadenomas, and with a negative family history of familial isolated pituitary adenoma (FIPA) or multiple endocrine neoplasia type 1 (MEN1) syndromes. Sanger sequencing was used for the assessment of AIP gene variants. The study was approved by the Ethics Board of JUMC., Results: AIP variants were identified in five of the 131 included subjects (3.8%): one diagnosed with Cushing's disease, two with acromegaly, and two with non-secreting adenomas. Patients harboring hereditary AIP gene alterations did not differ from the rest of the study group in median age at diagnosis (41.0 vs. 42.5 years, P=0.8), median largest tumor diameter (25 vs. 24 mm, P=0.6), gender distribution (60.0% vs. 56.3% females, P=0.8), secreting tumor frequency (60.0% vs. 67.5%, P=0.7), or acromegaly diagnosis frequency (40.0% vs.37.3%, P=0.9)., Conclusions: In our series of apparently sporadic pituitary macroadenomas, AIP gene variant carriers did not differ substantially from patients with negative genetic testing. A risk factor-centred approach to AIP genetic screening may result in missing germline variants. Considering the clinical impact of such genetic variants and their relatively low penetrance, it is, however, doubtful if general genetic screening benefits the whole cohort of pituitary macroadenoma patients and their families., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Trofimiuk-Müldner, Domagała, Sokołowski, Skalniak and Hubalewska-Dydejczyk.)

Introduction: We studied changes of pituitary adenoma volumes in patients treated with octreotide LAR (SSLAR) over 12 months prior to surgery., Materials and Methods: 26 patients (22 female and 4 male, mean age 57.5±15.0 years) were treated with SSLAR: 5 patients with microadenoma - during 6 months, and 21 patients with macroadenoma - during 12 months before surgery. Concentration of hGH and IGF-1 was evaluated at 0, 3, 6 and 12 months, while MRI images were taken at 0, 6 and 12 months prior to surgery. The volume of pituitary adenoma, estimated as that of an ellipsoid, was based on MRI image projections. Basic statistics, Shapiro-Wilk and Wilcoxon tests were applied., Results: Median values of hGH and IGF-1 concentrations prior to treatment were 19.0 ng/ml (IQR=40.7) and 766.4 ng/ml (IQR=787.7), respectively. After 6 and 12 months the median value of hGH concentration decreased to 6.3 ng/ml (IQR=9.0) and 3.45 ng/ml (IQR=3.7), respectively, while the median values of IGF-1 became 535.0 ng/ml (IQR=652.8) and 287.0 (IQR=275.7), respectively. All differences were statistically significant (p<0.05) as compared to basal value. The median volumes of adenomas differed significantly and were estimated at: 1.1 cm (3) (IQR=2.5) prior to treatment, 0.5 cm (3) (IQR=1.5) after 6 months, and 0.35 cm (3) (IQR=2.4) after 12 months of SSLAR administration., Conclusion: Treatment of acromegalic patients with somatostatin analogues not only decreases the concentration of hGH and IGF-1, but also appears to decrease the size of the tumour in about 50% of patients treated, leading to general enhancement of the outcome of surgery., (© J. A. Barth Verlag in Georg Thieme Verlag KG Stuttgart · New York.)

Background: We evaluated the predictive value of IGF-1 against hGH in the treatment outcome., Material and Methods: A prospective study was undertaken of 47 patients (mean age 41.1 ± 12.9 years; 44 with macroadenoma and 3 with microadenoma), requiring treatment with octreotide LAR (SSLAR) following incomplete surgery. Concentrations of hGH and IGF-1 were measured three months after surgery and three, six, nine, 12, 18, and 24 months after introducing SSLAR., Results: Following surgery, respective median values of hGH and IGF-1 concentrations were 5.55 ng/mL (IQR = 7.1) and 512.7 ng/mL (IQR = 379.5). After six, 12, and 24 months of SSLAR treatment, median values of hGH decreased significantly: to 2.95 ng/mL (IQR = 5.5, p < 0.05), 2.95 ng/mL (IQR = 4.4, p < 0.05) and 2.00 ng/mL (IQR = 3.6, p < 0.001), respectively. After six, 12, and 24 months of SSLAR treatment, the respective median IGF-1 concentrations significantly decreased to 384.5 ng/mL (IQR = 312.2, p < 0.01), 323.0 ng/mL (IQR = 230.3, p < 0.001) and 334.0 ng/mL (IQR = 328.9, p < 0.01). The differences between median hGH and IGF-1 concentrations at 12 and 24 months were not significant. A statistically significant correlation was found between IGF-1 concentration prior to and after surgery (R = 0.61, p < 0.05) and prior to SSLAR treatment and IGF-1 concentration 24 months later (R = 0.49, p < 0.05). No such correlation was observed for hGH., Conclusions: The level of IGF-1 prior to surgery and prior to SSLAR treatment is a better predictor of the treatment outcome than hGH. Octreotide LAR was most effective over the first 12 months of treatment. No further significant decrease of hGH or IGF-1 levels was observed past this period.

Introduction: A particularly challenging case of concurrent acromegaly and follicular thyroid carcinoma in a patient of the Clinic of Endocrinology, UJCM in Krakow is discussed., Case Description: A 59-year-old male with post total thyroidectomy performed in 2005 and histopathologically confirmed metastases of the follicular thyroid carcinoma to the lungs was admitted to the Clinic in April 2006 for complementary ¹³¹I treatment. Acromegaly was treated in 1996 by trans-sphenoidal surgery. In December 2005 a relapse of pituitary adenoma was shown by MRI, which correlated with increased levels of hGH and IGF-1. Biochemical control of acromegaly was achieved with Sandostatin LAR. Pre-therapeutic whole-body scintigraphy (WBS) revealed numerous conjoined hot spots of ¹³¹I accumulation in both lungs and in thyroid remnants. In May and November 2006 the patient received ¹³¹I treatment. Post-therapeutic WBS in November 2006 revealed complete ablation of the thyroid remnants. Laboratory tests confirmed lowering of thyroglobulin concentration. In the years 2007, 2008, and 2009 the patient was qualified for therapy with ¹³¹I aided by rhTSH, achieving further reduction of Tg levels. Post-therapeutic WBS performed in 2009 revealed weak bilateral tracer uptake in the lung parenchyma. In 2010, chest CT revealed fibrosis in left lung segments, no infiltrative changes, and no lymph node enlargement. Patient follow-up continues at our Department., Conclusions: Disseminated thyroid cancer in a patient with pituitary insufficiency may be successfully treated by rhTSH-supported ¹³¹I treatment.

Introduction: In acromegalic patients, the prevalence of certain benign and malignant neoplasms is higher than that in the healthy population. We retrospectively evaluated the prevalence of tumours in acromegalic patients treated at our department: the regional centre for acromegalic patients for the Małopolskie voivodeship in Poland., Material and Methods: During the years 1983-2008, a hundred and one acromegalic patients (30 males and 71 women), of mean age 51.8 +/- 15.4 years, were diagnosed and treated. Pituitary macroadenoma and microadenoma were stated in 63.4% and 25.7% of these patients, respectively. In 10.9% of these patients no data on tumour diameter were available. The mean observation period was 9.4 +/- 6.5 years. The median levels of hGH and IGF-1 prior to neurosurgery were 20.2 (IQR = 34.9) ng/ml and 764.5 (IQR = 569.6) ng/ml, respectively., Results: In the studied group of patients, we found the following prevalence of various tumours: nodular goitre - 64/101 patients (63.0%), polyps of the colon - 13/101 patients (13.0%); uterine polyps - 4/101 patients (4.0%); and prostate adenoma - 2/101 patients (2.0%). Among malignant tumours, thyroid cancer, endometrium and cervix cancer were the most frequent, each of these occurring in 3 patients (3.0%). Colon cancer prevalence was 2.0% (in 2 patients)., Conclusions: From our retrospective study, we suggest an overall increase of tumour incidence in acromegalic patients. Prospective multicentre studies are required to resolve the significance of this observation. In our study group, the number of malignant neoplasms was significantly higher in patients with long-lasting uncontrolled disease (over 5 years), compared to patients with controlled disease. (Pol J Endocrinol 2010; 61 (1): 29-34).

Since acromegaly is associated with high rates of comorbidities and increased mortality risk compared to the general population, over the last few years somatostatin analogues have been used to treat acromegaly patients who, following surgery, have not fulfilled cure criteria (basal hGH < 2.5 ng/ml, IGF-1 below normal ranges for age and sex and hGH < 1.0 ng/ml in the 120th min of the OGTT test). We assessed the efficacy of Octreotide LAR (OCT-LAR) in managing such patients. 72 patients underwent diagnostic tests to qualify them for Octreotide LAR treatment. Treatment efficacy evaluation was based on measuring the concentration of hGH and IGF-1 prior to and 3 and 12 months and performing control MRI 6 and 12 months after the beginning of OCT-LAR treatment. The dose of O ctreotide LAR was 20 mg/month, increased to 30 mg/month if unsatisfactory response was observed. We evaluated the efficacy of Octreotide LAR in 48 acromegaly patients (66.7% of 72 evaluated), in whom criteria of postsurgery cure were not fulfilled. 24 patients (33.3%) did not require further treatment. After 3 months of OCT-LAR treatment, hGH < 2.5 ng/ml was stated in 37.0% of patients, median value--3.4 ng/ml (IQR = 5.3), as compared to median value of 5.5 ng/ml (IQR = 5.6) before treatment (p < 0.05). After 3 months of treatment IGF-1 below normal ranges for age and sex was stated in 55.5% of patients, median value--336.8 ng/ml (IQR = 290.0), as compared to median value of 520.0 ng/ml (IQR = 351.0) prior to OCT-LAR treatment (p < 0.05). After 12 months hGH < 2.5 ng/l ml and IGF-1 below normal ranges for age and sex were found in 63.0% and 54.5% of patients, respectively. In control MRI recurrence, correlated with enhanced concentration of IGF-1, was stated in 7 patients (14.6%). Thus, we conclude that satisfactory acromegaly control, in terms of hGH and IGF-1 levels, was obtained in above 50% of patients treated with Octreotide LAR. Since in the studied group hGH secretion had achieved cure criteria after 3 months in 37.5% as compared to 63.0% after 12-months, assessment of OCT-LAR treatment should be extended over periods exceeding 3 months.