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Cushing disease and acromegaly are common endocrine disorders caused by excessive cortisol and growth hormone production, respectively. Both conditions can co-occur due to functioning pituitary adenomas, which are typically benign pituitary gland tumors. This report discusses a 30-year-old woman with hyperpituitarism leading to treatment-resistant Cushing disease and acromegaly caused by a functional pituitary macroadenoma. A 30-year-old woman presented with a history of excessive weight gain, facial puffiness, fatigue, persistent headaches, and visual disturbances. Clinical examination revealed features consistent with Cushing disease and acromegaly, including a moon face, central obesity, and large hands and feet—the ophthalmologic evaluation identified bitemporal hemianopia, suggesting optic chiasm compression. Laboratory results showed elevated ACTH, IGF-1, and prolactin levels, alongside confirmed hypercortisolism. The patient also had secondary diabetes mellitus and galactorrhea—initial treatment with octreotide provided limited benefit, with persistent hormone elevations and insufficient symptom control. The patient underwent endonasal endoscopic transsphenoidal resection of the pituitary macroadenoma, leading to marked symptomatic and hormonal improvements. This underscores the diagnostic challenge and treatment complexity of such cases. Early diagnosis is critical for optimizing outcomes in patients with hyperpituitarism and mitigating complications. This case highlights the importance of multidisciplinary management and the necessity of long-term follow-up to monitor for recurrence and ensure sustained remission.

The diagnosis of pituitary microprolactinomas is often obscured by relatively low levels of elevated prolactin compared to macroprolactinomas. This may lead to varying patterns of medical therapy versus observation. We sought to correlate prolactin levels in suspected microprolactinomas with tumor volumes and clinical outcomes. This was a multicenter retrospective study of patients with pituitary microadenomas with baseline prolactin levels > 18ng/ml for males and > 30ng/ml for females. A linear-mixed model was used to depict changes in tumor volume over time. There were 65 patients with a mean tumor volume of 95.9mm3 and mean prolactin level of 59.4ng/ml. There were significantly higher prolactin levels in patients with tumors above the mean volume versus below (74.0 versus 53.4ng/ml, p = 0.027). 26 patients were observed, 31 were treated with anti-dopaminergic therapy, and 8 had surgery. There were significantly greater baseline prolactin levels for patients who were treated surgically (mean 86.4ng/ml) than those treated medically (mean 61.7 g/ml) or observed (mean 48.5ng/ml) (p = 0.02). Among the 26 patients who were surveilled, 13 patients demonstrated spontaneous tumor shrinkage, 12 remained stable, and 1 patient's tumor grew but was lost to follow-up. Linear mixed modeling demonstrated a statistically significant rate of tumor shrinkage over time of 3.67mm3/year (p = 0.03). When analyzing patients who were observed versus those requiring surgery after initially being surveilled, there were significantly greater baseline PRL/volume ratios in surgical patients versus those observed (8.1 ng/ml/mm3 versus 2.4 ng/ml/mm3, p = 0.025). Suspected microprolactinomas may demonstrate more convincingly elevated prolactin levels when measuring over 95.9mm3. Tumors with baseline prolactin levels over 50ng/ml may be more inclined to undergo medical treatment. In tumors with levels below 50ng/ml, it may be reasonable to undergo surveillance as these tumors tend to spontaneously shrink over time. In tumors that are surveilled, an elevated baseline PRL/volume ratio of > 8 ng/ml/mm3 may be indicate serial tumor growth that may necessitate medical and/or surgical intervention. [ABSTRACT FROM AUTHOR]

Essential thrombocythemia (ET) is a malignant hematological disease that has the ability to progress to acute leukemia or transform into other myeloproliferative neoplasms (MPNs). The coexistence of ET and other MPNs with pituitary adenoma (PA) is rare. There are no reports of cases of secretory PA of prolactin hormone in combination with ET. This case was reported in a young woman from Saudi Arabia who had the secretory PA present in combination with ET. The 20-year-old patient was treated for a micro-PA with hyperprolactinemia. The patient was referred to the hematological service for thrombocytosis. There was no history of thrombosis, cardiovascular risk factors, or constitutional symptoms. Laboratory tests showed that platelet levels consistently lagged from 700 × 103/µL to 1000 × 103/µL for 1 year. Hemoglobin 13 g/dL, white blood cells (WBCs) 6 × 103/µL, and normal WBC differential. The peripheral blood smear was inconclusive. A peripheral blood sample was sent for the cytogenetic study of myeloproliferative diseases, which came to be positive for c.1849G>T p.(V617F) mutation in the EXON 14 Janus kinase 2 (JAK2) gene. The patient was diagnosed with a MPN, ET with positive exon 14 JAK2, at low-risk category. Evidence suggests that MPNs in combination with endocrinological diseases are rare. However, there is a high incidence of MPN and unrelated tumors such as PA. Further research is recommended to thoroughly investigate endocrine tumors and look beyond secondary thrombocytosis that leads to thrombocythemia as in ET.

Traditionally considered non-functional low proliferative benign neuroendocrine proliferations measuring less than 5 mm, pancreatic (neuro)endocrine microadenomas are now classified as pancreatic neuroendocrine microtumors in the 2022 WHO classification of endocrine and neuroendocrine tumors. This case report discussed the features of an incidentally identified 4.7-mm glucagon-expressing pancreatic neuroendocrine microtumor with MEN1 mutation only, chromosomally stable and an epigenetic alpha-like phenotype. The tumor was associated with an unexplained increased proliferation rate in Ki-67 of 15%. There was no associated DAXX/ATRX deficiency. The presented case challenges the conventional thought of a low proliferative disease of the so-called "pancreatic neuroendocrine microadenomas" and provides additional support to the 2022 WHO classification that also requires grading of these neoplasms. Despite exhibiting molecular features of less aggressive behavior, the case also underscores the biological complexity of pancreatic neuroendocrine microtumors. By recognizing the heterogenous spectrum of neuroendocrine neoplasms, the current case also contributes to ongoing discussions on how to optimize the clinical management of such tumors. [ABSTRACT FROM AUTHOR]

Essential thrombocythemia (ET) is a malignant hematological disease that has the ability to progress to acute leukemia or transform into other myeloproliferative neoplasms (MPNs). The coexistence of ET and other MPNs with pituitary adenoma (PA) is rare. There are no reports of cases of secretory PA of prolactin hormone in combination with ET. This case was reported in a young woman from Saudi Arabia who had the secretory PA present in combination with ET. The 20-year-old patient was treated for a micro-PA with hyperprolactinemia. The patient was referred to the hematological service for thrombocytosis. There was no history of thrombosis, cardiovascular risk factors, or constitutional symptoms. Laboratory tests showed that platelet levels consistently lagged from 700 × 103/µL to 1000 × 103/µL for 1 year. Hemoglobin 13 g/dL, white blood cells (WBCs) 6 × 103/µL, and normal WBC differential. The peripheral blood smear was inconclusive. A peripheral blood sample was sent for the cytogenetic study of myeloproliferative diseases, which came to be positive for c.1849G>T p.(V617F) mutation in the EXON 14 Janus kinase 2 (JAK2) gene. The patient was diagnosed with a MPN, ET with positive exon 14 JAK2, at low-risk category. Evidence suggests that MPNs in combination with endocrinological diseases are rare. However, there is a high incidence of MPN and unrelated tumors such as PA. Further research is recommended to thoroughly investigate endocrine tumors and look beyond secondary thrombocytosis that leads to thrombocythemia as in ET. [ABSTRACT FROM AUTHOR]

Background: The first-line surgical management of an adrenocorticotropic hormone (ACTH)--secreting pituitary adenoma causing Cushing's disease (CD) is endoscopic transsphenoidal resection of the tumor. This study was performed to assess postoperative (postop) complications and remission in endoscopic surgically resected cases of CD. Methods: Data of patients who underwent endoscopic transsphenoidal surgery (ETSS) for CD were collected from the neurosurgery department at a tertiary care center in a retrospective manner from January 2015 to February 2022 and analyzed. Postoperative remission was categorized as - early morning serum cortisol <138 nmol/L within 7 days of the surgery, as per the Endocrine Society Guidelines, with significant clinical improvement in features of hypercortisolism in the operated patient and strict cutoff rate of <50 nmol/L at postop day 3 was also utilized, to look for the early identification of remission. Results: A total of 41 patients were identified who underwent 44 ETSS during the same timeframe. Preoperative magnetic resonance imaging localized an adenoma in all 41 patients, out of which 32 were microadenoma, and nine were macroadenoma (2 with cavernous sinus invasion). Intrapetrosal sinus sampling was performed in 35 (85%) patients. The rate of remission for the initial surgery was 85.4% using the standard criteria and 68.3% using strict criteria. Three patients underwent early repeat surgery for the persistent disease as the day 3 cortisol was high (306-555 nmol/L). Once the outcome of this surgery was also included, the overall rate of remission was 90.2% (37/41). None of the patients had meningitis, cerebrospinal fluid leakage, visual deterioration, or vascular injury. Permanent and transient diabetes insipidus (DI) occurred in 9.75% and 26.8% following the first ETSS, respectively. We also noted a single case of CD recurrence in 9 months during the total follow-up period of 84 months. Conclusion: ETSS has satisfactory rates of remission for the primary treatment of CD, with rates being higher for microadenomas. A long follow-up period is needed to assess the rates of recurrence. Patients must be counseled regarding the risk of postop DI, whether transient or permanent, as a possible complication. [ABSTRACT FROM AUTHOR]

Simple Summary: The pituitary gland is crucial in regulating metabolic processes. Tumors in this gland can be subtle, meaning that establishing reference values for the pituitary dimensions is pivotal. Computed tomography is commonly used to identify pituitary alterations, plan surgery or radiation therapy, and monitor treatment responses. This study aimed to determine the normal pituitary size, and the pituitary-to-brain ratio, in a group of domestic short-haired cats by computed tomography. The study also aimed to explore the correlations between body weight, age, sex, and pituitary dimensions, and assess the inter- and intra-agreement between operators in measuring pituitary dimensions. The study showed that the normal range for pituitary dimensions is wider than previously reported. The study also showed a low correlation between body weight, age, and pituitary dimensions. The intra-operator agreement in measuring pituitary dimensions was good/excellent, but the inter-operator agreement was moderate/good, likely due to differences in expertise. The reference values obtained from this study could help in better evaluating the pituitary gland in domestic short-haired cats with suspected pituitary neoplastic lesions. The detection of subtle changes in the pituitary dimensions has relevant clinical implications. In cats, a few studies have established the cut-off values of the pituitary gland's dimensions using small and inhomogeneous samples. The aims of this study were: to determine by computed tomography (CT) the pituitary linear dimensions and the pituitary-to-brain (P:B) ratio in a sample of domestic short-haired (DSH) cats; to assess the effects of sex, age, and weight on pituitary dimensions; and to evaluate the inter- and intra-observer agreement for such measurements. All skull CTs of DSH cats performed over four years using a multidetector CT and a standardized protocol were retrospectively reviewed. The exclusion criteria were: clinical, laboratory, or CT alterations of the pituitary gland, brain diseases, fractures of the neurocranium, and diabetes. The pituitary dimensions and brain area were assessed by two different observers using multiplanar reconstructions and automated segmentation tools. Fifty-one cats were included in the final sample. The intraclass correlation coefficients for intra- and inter-observer reliability were good/excellent, and moderate/good, respectively. No differences between sexes were detected, and negligible correlations were found between age and weight. According to this study, a pituitary gland with a height > 4 mm or a P:B ratio > 0.49 mm should be considered enlarged. [ABSTRACT FROM AUTHOR]

Purpose: The optimal management approach for presumed non-functioning pituitary microadenomas (microNFPAs) remains unclear. Our aim was to capture current UK practice and identify changes with time. Methods: Two online surveys investigating clinicians' approaches were performed in 2009–2010 and 2021–2022 (advertised through Society for Endocrinology UK). Results: 150 and 214 clinicians participated in the 2021 and 2009 survey, respectively (response rates 31.2% and 35.4%, respectively). At baseline, 2021 survey respondents were more likely to measure IGF-1 (96.0% vs 74.1%, p < 0.001) and morning cortisol (87.9% vs 62.6%, p < 0.001), and less likely GH (26.2% vs 42.6% p = 0.002), 24 h urine free cortisol (3.4% vs 23.2%, p < 0.0001) or dynamically assess adrenal reserve (11.4% vs 30.4%, p < 0.001). 47.2% of clinicians in 2021 would reassess pituitary function annually until discharge (in absence of tumour growth/symptoms). The 2021 survey respondents were more likely to stop imaging at or before 3 years (81.7% vs 44.3%, p < 0.001) and at or before 5 years (86.6.% vs 72.9%, p = 0.002), whilst 2009 survey respondents were more likely to continue imaging beyond 5 years (24% vs 7%, p < 0.001). Responses on imaging frequency/intervals showed notable variability in both surveys. Conclusions: Diagnostic and management approaches for microNFPAs have evolved in the UK. Biochemical investigations are performed in accord with consensus guidelines, though many clinicians perform annual biochemical surveillance without tumour growth/symptoms. A small number of clinicians request imaging beyond 5 years, but the frequency of imaging intervals until discharge remains variable. Robust evidence on the long-term natural history of microNFPAs is necessary to unify clinician approach. [ABSTRACT FROM AUTHOR]

Acromegaly is a clinical syndrome with isolated cases globally. Patients may remain undiagnosed for several years after the onset of initial signs and symptoms. Timely diagnosis and initiation of its treatment play a crucial role in patients with acromegaly. In this article, we present physical, biochemical, and radiological characteristics in a set of 6 cases that may aid in prompt diagnosis and appropriate management of acromegaly. [ABSTRACT FROM AUTHOR]

Abstract Background Pituitary adenomas are benign brain tumors that cause considerable morbidity and neurological symptoms. SOX9 as a regulatory transcriptional mediator affects normal and tumor cell growth with an undefined role in pituitary adenomas pathogenesis. Thus, in the present study, the expression pattern of SOX9 in GH-secreting pituitary tumors and normal pituitary tissues is investigated. Methods The SOX9 gene expression level was evaluated in 60 pituitary tissues including different types of GH-secreting adenomas and normal pituitary tissues through Real-Time PCR. The protein level of SOX9 was assessed using immunohistochemistry. The correlations of SOX9 gene and protein expression level with the patient’s clinical and pathological features were considered. Results The SOX9 over-expression was detected in GH-secreting adenomas tumor tissues compared to normal pituitary tissues which were accompanied by overexpression of SOX9 protein in tumor tissues. The over-expression of SOX9 had a significant impact on GH-secreting adenomas tumor incidence with the odds ratio of 8.4 and the diagnostic value of SOX9 was considerable. The higher level of SOX9 expression was associated with invasive and macro tumors in GH-secreting pituitary adenoma patients. The positive correlation of SOX9 gene and protein level was observed and the tumor size and tumor invasive features were valuable in predicting SOX9 expression level in GH-producing pituitary tumors. Conclusion The study provided the first shreds of evidence regarding the expression pattern of SOX9 in the GH- secreting pituitary adenomas at both gene and protein levels which may emphasize the possible involvement of SOX9 as a mediator in pituitary adenoma tumor formation also open up new intrinsic molecular mechanism regarding pituitary adenoma pathogenesis.

Objective: Cushing disease is a potentially fatal endocrine disorder caused by adrenocorticotropin (ACTH)‐secreting microadenomas in the pituitary gland. Accurate detection and localization of the adenomas is the key to clinical treatment. This study analysed the value of contrast‐enhanced Sampling Perfection with Application‐optimized Contrasts using different flip angle Evolutions (SPACE) sequence in magnetic resonance imaging (MRI) assessment of ACTH‐secreting pituitary microadenomas. Design and Patients: We carried out a retrospective study in which 45 patients with ACTH‐secreting pituitary microadenomas were enrolled. Dynamic contrast‐enhanced (DCE) coronal T1‐SE sequence was performed. A contrast‐enhanced coronal SPACE sequence was added immediately after DCE MRI finished. Two independent observers assessed the tumour existence and location, then the results were compared with surgical findings. Results: Twenty‐four lesions (53.3%) were detected by the DCE T1‐SE sequence alone, while 35 lesions (80.0%) were detected with the addition of contrast‐enhanced SPACE sequence. The sensitivity (58.5% vs. 85.3%; p <.05) and best diagnostic accuracy (62.0% vs. 84.4%; p <.05) were significantly better for addition with SPACE sequence than DCE‐SE images alone in detection of ACTH‐secreting pituitary microadenomas. For lesions <5 mm, the detected numbers were 4 (16.6%) versus 10 (27.8%) by DCE T1‐SE sequence and combined DCE T1‐SE with SPACE sequence. Conclusions: A combination of contrast‐enhanced SPACE with DCE T1‐SE sequence could improve the detection of ACTH‐secreting pituitary microadenomas. Contrast‐enhanced SPACE sequence could be a supplementary sequence for imaging of ACTH‐secreting pituitary adenomas when T1‐SE sequence provides negative or equivocal findings. [ABSTRACT FROM AUTHOR]

Context: Characterization of the clinical features and natural history of nonfunctioning pituitary microadenomas (NFPmAs) is limited by heterogeneous and small-scale studies. Objective: To characterize the clinical presentation and natural history of NFPmAs and evaluate if imaging follow-up interval can be extended. Methods: Retrospective single-center cohort study (years 2006-2021) of conservatively managed patients with NFPmAs. Initial symptoms, pituitary function, and tumor size were assessed. A change in NFPmA size =2 mm, as determined by pituitary or brain magnetic resonance imaging (MRI), was considered significant. Results: There were 347 patients in the study cohort. Headache (78.4%) and fatigue (70.0%) were commonly reported despite no evidence of mass effect or significant pituitary hypofunction. Pituitary deficiencies at baseline were rare, with hypogonadism being most common (5.1%). During a median imaging follow-up period of 29 months (range 3-154), 8.1% of NFPmAs grew. Growth incidence was 2.1 per 100 person-years with a mean and median time to growth of 38.1 (SD ± 36.4) and 24.5 (interquartile range 12.0-70.8) months, respectively. Tumor growth was mild and not associated with new pituitary deficiencies or visual deficits. Conclusion: These data indicate that the natural history of NFPmAs is overall benign. Consequently, we propose that the initial MRI follow-up timeline for NFPmAs can be extended up to 3 years unless a lesion is close to the optic chiasm, there are worrisome mass effect symptoms, or new pituitary deficiencies. [ABSTRACT FROM AUTHOR]

ACTH secreting pituitary adenomas are rare occurrences in autoptical, surgical and neuroradiological series. We herewith describe a series of four patients of Cushing’s disease due to ACTH secreting pituitary microadenoma. All patients presented with signs and symptoms of hypercortisolism. Hormonal testing and bilateral inferior petrosal sinus sampling were indicative of pituitary-dependent Cushing’s syndrome. Dynamic sellar MRI was suggestive of asymmetric pituitary, with suspect pituitary microadenoma in all cases. Three patients were operated for endoscopic endonasal transsphenoidal surgery, while one patient refused surgery and was managed with ketoconazole. During postoperative period, remission of hypercortisolism was ensured in two patients, while one patient continued to have raised levels of cortisol. One patient with raised levels of cortisol during postoperative period was prescribed Ketoconazole for 6 months. Histopathology confirmed the presence of pituitary adenoma. Adenoma displayed ACTH immunoreactivity. Our cases indicate that ACTH secreting pituitary microadenoma may occur in patients with Cushing’s disease. Careful radiological, surgical and pathological examination is necessary to recognize this condition and avoid surgical failure.

La hiperprolactinemia es un diagnóstico frecuente en la consulta de endocrinología y de los adenomas hipofisarios los prolactinomas son el subtipo más frecuente causantes de hiperprolactinemia. Se revisó pacientes con hiperprolactinemia idiopática y prolactinomas evaluados en el servicio de endocrinología del Centro Médico Docente La Trinidad. Se realizó un estudio descriptivo, retrospectivo, de carácter observacional en el que se explorararon posibles relaciones estadísticamente significativas, entre la presencia de hiperprolactinemia idiopática versus tumores hipofisiario (prolactinoma) e hiperprolactinemia y características demográficas o de presentación y presentació clínica. Se encontró: predominio de hiperprolactinemia en el sexo femenino, con una proporción de 4:2, pacientes con prolactinomas la proporción fue 3 mujeres por cada hombre afectado, el promedio de edad al momento del diagnóstico en las mujeres resultó 37 años y 45 años en los hombres. Los prolactinomas eran responsables de la hiperprolactinemia en un 67.44 %, siendo los microadenomas los tumores más frecuentes con un 68,9 %. Los macroadenomas se presentaron en 9 pacientes (31,03 %) de los cuales 8 fueron tratados con neurocirugía. Las manifestaciones clínicas y comorbilidades en los 43 pacientes con hiperprolactinemia principales fueron trastornos menstruales, dislipidemia y sobrepeso. La concentración media de prolactina estuvo directamente relacionada con las dimensiones de los tumores. Por último, se observó que la totalidad de los pacientes recibió tratamiento con cabergolina y 8 ameritaron neurocirugía, presentando déficits secuelares más inherentes al tumor que a la cirugía.

Objective  Pituitary adenomas are historically classified into microadenoma or macroadenomas based on size less than or greater than/equal to 1c m. "Giant" adenomas describe tumors ≥4 cm. The aim of this study is to present an evidence-based approach to size classification based on national trends. Design  The design involved is multi-institutional retrospective study. Participants  A total of 29,651 patients were studied from National Cancer Institute's SEER program from 2004 to 2016 across the United States. Main Outcome Measures  The main outcome measures include demographics, treatment characteristics, and overall survival in the population. Results  At the 20-mm threshold, the likelihood of operation exceeds the likelihood of nonoperative management. Patients with adenoma size 1 to 19 mm had significantly longer overall survival compared with 20 to 50 mm (Log rank: p  < 0.0001). No survival difference was found between size 20 to 29 mm and larger. There was no significant difference in the rate of surgery between 30 to 39 mm and 40 to 50 mm tumors(p  = 0.5035). Surgery group had a higher overall survival compared with nonsurgically managed patients (Log rank: p  < 0.0001). Conclusion  Microadenoma has classically been used to describe pituitary tumors less than 1 cm, though no clinical significance of this threshold has been demonstrated. The current study suggests a size cut-off of 20 or 30 mm as more clinically relevant. Still, future studies are warranted to examine the significance of this classification by specific tumor type, and subclassified as appropriate. There is no difference in the rate of surgery or survival for adenomas between 30 and 50 mm, challenging the 4-mm cutoff threshold for "giant" adenoma. [ABSTRACT FROM AUTHOR]

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Purpose: Adrenocorticotropic hormone (ACTH)-dependent Cushing's disease accounts for 75% cases of the endogenous Cushing's syndrome. The size of lesion is usually very small, which results in false-negative magnetic resonance imaging (MRI) even after biochemical confirmation of the disease. Corticotrophin-releasing hormone (CRH) the key controller of hypothalamus-pituitary--adrenal axis binds to CRH receptor R1 and R2. CRH R1 is overexpressed in pituitary adenomas. The present study aims to target these overexpressed receptors with 68Ga-DOTA-CRH for noninvasive imaging of ACTH-dependent pituitary adenomas. Materials and Methods: Custom-synthesized 1,4,7,10-tetraazacyclododecane-1,4,7,10-tetraacetic acid (DOTA)-CRH peptide was purified by high performance liquid chromatography (HPLC) and characterized by mass spectra. Postradiolabeling optimization with 68Ga, quality control tests were carried out to ensure the suitability of 68Ga DOTA-CRH for intravenous administration. A pilot study consisting of 15 patients including 6 known cases of macroadenoma underwent 68Ga-DOTA-CRH regional brain positron emission tomography/computed tomography (PET/CT). The optimal imaging time and biodistribution studies were performed in five patients' whole-body and serial brain PET/CT imaging. Lesion activity was determined as SUVmax and correlated with CE-MRI and histopathology of excised tissue. Results: A retention time of 11.3 min and mass of 5145 Da was observed on HPLC and mass spectra. Radiolabeling yield of >98% was achieved under optimized conditions using 25-100 μg of conjugated peptide for 10-22 mCi of 68Ga. The quality control results were in agreement with acceptable criteria. 68Ga-DOTA-CRH was able to delineate ACTH secreting corticotropinoma in all 15 patients. Physiological uptake of radiotracer was observed in liver and spleen with diffused marrow activity. Excretion was noted by renal route. Imaging results were in correlation with CE-MRI and histopathology of excised tissue. Conclusion:68Ga-DOTA-CRH PET/CT is a promising molecular imaging modality for detection of ACTH-dependent microadenoma. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the validity of electrophysiological tests in the early diagnosis of a ganglion cells and/or optic nerve dysfunction in patients with pituitary microadenoma. Methods: 66 eyes, from 33 patients with microadenoma with no evidence of the optic chiasm compression in magnetic resonance imaging (MRI) and the visual impairment in the routine ophthalmological examination, standard static perimetry (24-2 white on white) and optical coherence tomography (HD-OCT), were analysed. The pattern electroretinogram (PERG), standard pattern visual evoked potentials (PVEPs) and multichannel visual evoked potentials (mVEPs) (ISCEV standards) were performed. The results obtained from the electrophysiological tests were compared to the same number of age-matched healthy controls. Results: Statistically significant differences between the patients with microadenoma and healthy controls were detected in all electrophysiological tests (p < 0.001). The most frequent abnormalities were observed in mVEPs (25/33 patients, 75.8%; 43/66 eyes, 65.2%). The most frequent features registered in this test were: (1°4′)—an increase in the P100wave latency from uncrossed fibres (13/33 patients, 39.39%; 21/66 eyes, 31.8%) and (0°16′)—an amplitude reduction of this wave from the crossed fibres (11/33 patients, 33.33%; 19/66 eyes, 28.8%). The changes in PVEPs (15/33 patients, 45.5%; 25/66 eyes, 37.9%) and PERG (10/33 patients, 30.3%; 15/66 eyes, 22.7%) were also registered. Of all the tests and parameters analysed in the study, the greatest diagnostic value in detecting the visual pathway dysfunction in this group of patients was the amplitude of P100 wave from the crossed fibres of the mVEPs (1°4′) with a sensitivity of 60.6% and a specificity of 93.8%. These parameters suggest that this type of dysfunction is downstream to the chiasm and can also indicate the visual pathway dysfunction severity. Conclusions: In patients with microadenoma, the abnormalities in the electrophysiological tests are registered even without clinical evidence of visual impairment from the routine ophthalmological examination, SAP, OCT and chiasmal compression in MRI. The mVEPs have the most significant role in the diagnosis of the visual pathway dysfunction in patients with microadenoma. [ABSTRACT FROM AUTHOR]

Purpose: We aimed to assess clinical characteristics of apoplexy of pituitary microadenomas compared to macroadenomas. Methods: We retrieved clinical records of patients > 18 years old, hospitalized in Rambam hospital between January 2001 and October 2017, with pituitary apoplexy and follow-up of at least one year. We compared clinical course and outcomes of apoplexy between patients with microadenomas and macroadenomas, and between patients who received conservative or surgical treatment. Statistical analysis was done using Fisher's exact and Mann–Whitney tests. Results: Twenty-seven patients with pituitary apoplexy were included in the final analysis: mean age was 40.7 ± 12.5 years, 13(48%) were female, 7(26%) had microadenomas, and 21(78%) harbored clinically nonfunctioning pituitary adenomas. Upon admission, hyponatremia, random cortisol level of < 200 nmol/L, and secondary hypothyroidism, were evident in 6/20, 8/18, and 4/18 patients with macroadenoma and 1/5, 2/5, and 1/6 patients with microadenoma, respectively (P = 1.0). Hypogonadotropic hypogonadism was evident in 9/12 men with macroadenoma. In 12 macroadenoma patients, the tumor abutted the optic chiasm, of whom eight had visual field defects. Fifteen patients with macroadenoma and two with microadenoma underwent transsphenoidal surgery. Median follow-up was 3 years. At last follow-up visit, patients with microadenoma had lower rates of corticotropic deficiency or secondary hypothyroidism compared to macroadenoma patients (1/7 vs. 13/20 respectively, p = 0.033). Only two patients with macroadenomas had persistent visual field defects. Outcomes were comparable between conservative and surgical treatment groups. Conclusions: Long term pituitary hormone deficiencies are more common in pituitary apoplexy patients with macroadenomas. Apoplexy of pituitary microadenoma carries a more favorable prognosis. [ABSTRACT FROM AUTHOR]

Background: Pituitary adenomas are benign brain tumors that cause considerable morbidity and neurological symptoms. SOX9 as a regulatory transcriptional mediator affects normal and tumor cell growth with an undefined role in pituitary adenomas pathogenesis. Thus, in the present study, the expression pattern of SOX9 in GH-secreting pituitary tumors and normal pituitary tissues is investigated. Methods: The SOX9 gene expression level was evaluated in 60 pituitary tissues including different types of GH-secreting adenomas and normal pituitary tissues through Real-Time PCR. The protein level of SOX9 was assessed using immunohistochemistry. The correlations of SOX9 gene and protein expression level with the patient's clinical and pathological features were considered. Results: The SOX9 over-expression was detected in GH-secreting adenomas tumor tissues compared to normal pituitary tissues which were accompanied by overexpression of SOX9 protein in tumor tissues. The over-expression of SOX9 had a significant impact on GH-secreting adenomas tumor incidence with the odds ratio of 8.4 and the diagnostic value of SOX9 was considerable. The higher level of SOX9 expression was associated with invasive and macro tumors in GH-secreting pituitary adenoma patients. The positive correlation of SOX9 gene and protein level was observed and the tumor size and tumor invasive features were valuable in predicting SOX9 expression level in GH-producing pituitary tumors. Conclusion: The study provided the first shreds of evidence regarding the expression pattern of SOX9 in the GH- secreting pituitary adenomas at both gene and protein levels which may emphasize the possible involvement of SOX9 as a mediator in pituitary adenoma tumor formation also open up new intrinsic molecular mechanism regarding pituitary adenoma pathogenesis. [ABSTRACT FROM AUTHOR]

Introduction: Prolactinoma account to the most common pituitary adenomas and current therapy regime constitutes of dopamine agonist therapy (DA) and surgery in selected cases [17]. Due to tumor fibrosis induced by previous DA therapy, surgical removal can be challenging though. Therefore, this study investigates how preoperative DA usage influences perioperative treatment and surgical outcome in prolactinoma and aims to ascertain whether a specific subgroup of prolactinoma patients could derive greater benefit from exclusive surgical intervention., Methods: We retrospectively analyzed n = 159 surgically treated and histologically confirmed prolactinomas in the sella region from 2013-2022 in our institution. Clinical, radiological and surgical features were analyzed. Univariate and multivariate analyses were performed., Results: Out of total of 159 prolactinoma patients, 83.6% received previous treatment with DA followed by surgery, while only 16.4% received exclusive surgery. Both groups presented similar initial tumor volumes (1.9cm 3 vs. 1.5cm 3 , p = 0.59) and equal preoperative prolactin levels (PRL) (199.7 µg/l vs. 191.0 µg/l, p = 0.44). Surgical procedures took significantly longer when patients received prior DA treatment (79 min. vs. 70 min., p = 0.0479). Six months after surgery, pretreated patients revealed significantly higher PRL compared to non-treated (107 g/l vs. 8.64 µg/, p = 0.0009). Additionally, untreated microprolactinoma presented a remission of 100%, whereas pretreated exhibited a remission rate of 88.75%., Conclusion: The current study demonstrates that prior DA treatment is associated with significantly longer surgeries, higher recurrence rates and lower rates of normalization of PRL levels after surgery, particularly in microprolactinomas and support the latest recommendations of the Pituitary Society's Consensus Statement 2023, which favors the option of surgery alone as first-line therapy for microprolactinomas., (© 2024. The Author(s).)

Purpose: To report the results of a series of patients undergoing the endoscopic subperichondrial transseptal (STRAS) approach for pituitary surgery and to evaluate the efficiency and the safety of this approach. Methods: This is a single-centre retrospective study including all patients undergoing pituitary lesion resection through the STRAS approach from January 2002 to December 2017 by a multidisciplinary surgical team (ENT and neurosurgeon). Demographic data, tumour type, complication rate and pre- and post-operative visual, endocrine and tumour status were retrospectively analysed. Results: 119 patients were included in the study, 80 (67%) presenting macroadenoma, 24 (20%) microadenoma (20%) and 6 (5%) giant adenoma. 61 (51%) patients had secreting adenoma and 51 (42%) patient had non-functioning adenoma. The STRAS approach allowed a good visualization of intrasphenoidal and intrasellar anatomical landmarks in all cases and no patient needed turbinate resection. No patient died or had neurological deficit. Endocrine remission or control was achieved in 90.5% of hormone-secreting microadenomas and in 84.2% of hormone-secreting macroadenomas. Gross-total resection was achieved for 39 patients (48.8%) of the 80 macroadenomas. Nasal complication rate was very low, with no septal perforation and two epistaxis (1.7%) medically treated. Conclusion: The STRAS approach is an elegant approach to the sphenoid sinus that enables a good exposure of the intrasphenoidal anatomical landmarks with a maximal preservation of the nasal mucosa. This approach allows an intrasellar work with great comfort and safety for the surgeon using a two-hand or a four-hand technique. [ABSTRACT FROM AUTHOR]

Purpose: Carney complex (CNC) is a rare autosomal dominant syndrome, characterized by mucocutaneous pigmentation, cardiac, cutaneous myxomas and endocrine overactivity. It is generally caused by inactivating mutations in the PRKAR1A (protein kinase cAMP-dependent type I regulatory subunit alpha) gene. Acromegaly is an infrequent manifestation of CNC, reportedly diagnosed in 10% of patients. Methods: We here report the case of a patient who was concomitantly diagnosed with Carney complex, due to a new mutation in PRKAR1A ((NM_002734.3:c.80_83del, p.(Ile27Lysfs*101 in exon 2), and acromegaly. In parallel, we conducted an extensive review of published case reports of acromegaly in the setting of CNC. Results: The 43-year-old patient was diagnosed with an acromegaly due to a GH-secreting pituitary microadenoma resistant to somatostatin analogs. He underwent transsphenoidal surgery in our tertiary referral center, which found a pure GH-secreting adenoma. In the literature, we identified 57 cases (24 men, 33 women) of acromegaly in CNC patients. The median age at diagnosis was 28.8 ± 12 year and there were 6 cases of gigantism. Acromegaly revealed CNC in only 4 patients. 24 patients had a microadenoma and two carried pituitary hyperplasia and/or multiple adenomas, suggesting that CNC may result in a higher proportion of microadenoma as compared to non-CNC acromegaly. Conclusions: Although it rarely reveals CNC, acromegaly is diagnosed at a younger age in this setting, with a higher proportion of microadenomas. [ABSTRACT FROM AUTHOR]

Hypogonadism is a result of testicular/ovarian failure and/or insufficient pituitary stimulation. Various hypothalamo-pituitary abnormalities or lesions can contribute to hypogonadism. Benign or malign tumoural lesions of cellar or paracellar region, may lead to hypogonadism. Therefore, pituitary magnetic resonance imaging (MRI) is needed in patients with hypogonadism. In our study, we aimed to investigate our hypothalamo-pituitary MRI findings of hypogonadal patients. We evaluated 42 isolated hypogonadal patients followed in our clinic. These patients pituitary MRI findings were analyzed retrospectively. 80.9 % of isolated hypogonadotrophic patients were male, 19.1 % were female. Pituitary MRI findings of isolated hypogonadotrophic patients revealed that; 59.5% (n=25) were normal, 16.7 % (n=7) pituitary microadenoma, 11.9% (n=5) partial empty sella, 4.7% (n=2) pituitary macroadenoma, 2.4% empty sella (n=1), 2.4% (n=1) ectopic neuropituitary, and 2.4% (n=1) empty sella plus ectopic neuropituitary together. In half of the patients with isolated hypogonadotrophic hypogonadism, pituitary MRI findings may be normal. In these patients, if clinic and laboratory results are harmonious, to determine the diagnosis dynamic tests are required and appropriate therapy must be done, even if pituitary MRI is normal. [Med-Science 2015; 4(4.000): 2857-68]

Background This study investigated the outcome of transsphenoidal surgery (TSS) for Cushing disease (CD) and the influence of our surgical strategy on remission rates and postoperative pituitary function. Patients and Methods We retrospectively reviewed data from 71 patients with CD who underwent microscope navigation TSS (MN-TSS) in Saint-Luc Hospital between 1996 and 2017. True remission was defined as normal fasting cortisol level, normal 24-hour urinary free cortisol, or continued need for hydrocortisone replacement for 1 year after surgery. Results Overall remission rate after 1 or repeated MN-TSS was 83%. Highest remission rate was found in patients with macroadenomas (92%). Successful first MN-TSS was correlated with a high final remission rate (95%), whereas failed first MN-TSS was correlated with a low final remission rate (36%). Although day 1 cortisol levels were significantly lower in patients with long-term remission, high levels were still observed in a few patients, especially those who had had CD for many years. We found a low rate of postoperative pituitary long-term hypofunction (9.7%). Conclusions MN-TSS is a safe and effective procedure to treat CD, allowing remission rates of 83%. One-year remission period after first surgery is correlated with a final remission rate of 95%. Although day 1 morning cortisol value is the most significant predictor for long-term remission, some patients with CD for many years may keep high postoperative cortisol levels and be in later remission, likely because of secondary adrenal hyperplasia. Our focused approach with microscope navigation resulted in low rates of postoperative pituitary hypofunction and kept a recurrence rate comparable to that in the literature. Highlights • The overall remission rate in our center (mainly 1 surgeon) was 83%. Highest remission rate in patients with macroadenomas (92%). • Successful first MN-TSS was correlated with a high final remission rate (95%), failed first MN-TSS remained associated with a low final remission rate (36%) even after additional surgery. • For patients with CD for many years, a high-level day 1 cortisol level was regularly observed even if followed by long-term remission. • Less than 10% of postoperative pituitary long-term hypofunction. [ABSTRACT FROM AUTHOR]

Pancreatic neuroendocrine microadenomas (NEMAs) are non-functioning neuroendocrine tumors < 0.5 cm with a low proliferation rate and are considered benign. We report on a pancreatic NEMA with lymph node metastasis. A male in his 70s had pylorus-preserving pancreaticoduodenectomy for a distal bile duct carcinoma, which was a 2.1 cm well-differentiated-infiltrating adenocarcinoma with invasion limited to the bile duct wall. An incidental separate 0.4 cm well-differentiated NEMA was found in the pancreatic head with metastatic well-differentiated neuroendocrine tumor in one peripancreatic lymph node. Both neuroendocrine tumors in the pancreatic head and in the lymph node were composed of nests of uniform neoplastic cells with a fine chromatin pattern. The Ki-67 labeling index of NEMA was 0.85%. The neoplastic neuroendocrine cells in both the pancreas and node were diffusely positive for synaptophysin, chromogranin, and insulin. Therefore, this unusual case provides an exception to the current classification system which regards NEMAs as benign lesions. [ABSTRACT FROM AUTHOR]

Background & Importance: The aim of this study was to demonstrate the result of transsphenoid microsurgery for Cushing disease in our center and comparing the results in details with other articles, to achieve the best decision for patients management in future. Case Presentation: Between 1991 to 2015, 50 patients suffering from Cushing disease were operated. Before operation, endocrine tests and neuroimaging examinations were done for all patients and all of them were operated via transsphenoid approach. They were followed up in clinic with endocrine tests and imaging in regular intervals. During this study, 50 Cushing cases including 47 primary and three secondary tumors were operated. Thirty four (68%) tumors were microadenoma and 16 (32%) macroadenoma. A patient died due to postoperative fulminant meningitis (mortality=2%). Mean of follow-up was five years. Overall, 40 (80%) patients had remission after operation and 9 (18%) patients achieved no remission, three of them had macroadenoma and six microadenoma. During follow-up period, three (6%) patients had recurrence which needed second operation. In secondary tumor and in macroadenoma with cavernous sinus involvement, the result was not good (33% and 0% remission, respectively). Conclusion: Transsphenoid surgery is a safe and effective first choice method to manage Cushing disease compared to other treatment modalities. In recurrent or resistant cases, other modalities could help to manage patients.