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MicroRNA (miR)-200b-3p has been associated with many tumors, but its involvement in pituitary adenoma is unclear. This study investigated the molecular mechanism underlying miR-200b-3p regulation in pituitary adenomas to provide a theoretical basis for treatment. Bioinformatics was used to analyze pituitary adenoma-related genes and screen new targets related to RECK and miRNA. As well, the relationship between miR-200b-3p and RECK protein was verified using a double-luciferase reporter gene assay. The expression of miR-200b-3p in clinical samples was analyzed by in situ hybridization. Transfection of the miR-200b-3p inhibitor and small interfering-RECK (si-RECK) was verified by qPCR. GH3 cell viability and proliferation were detected using CCK8 and EdU assays. Apoptosis was detected by flow cytometry and western blotting. Wound healing and Transwell assays were used to detect cell migration and invasion. The effects of miR-200b-3p and RECK on GH3 cells were verified using salvage experiments. miR-200b-3p was highly expressed in pituitary tumor tissue. Inhibitors of miR-200b-3p inhibited cell proliferation promoted cell apoptosis, inhibited invasion and migration, and inhibited the expression of matrix metalloproteinases. Interestingly, miR-200b-3p negatively regulated RECK. The expression of RECK in pituitary adenoma tissues was lower than that in neighboring tissues. Si-RECK rescued the function of miR-200b-3p inhibitors in the above cellular behaviors, and miR-200b-3p accelerated the development of pituitary adenoma by negatively regulating RECK expression. In summary, this study investigated the molecular mechanism by which miR-200b-3p regulates the progression of pituitary adenoma through the negative regulation of RECK. The findings provide a new target for the treatment of pituitary adenoma. [ABSTRACT FROM AUTHOR]

Background: Large and giant pituitary adenomas (defined as tumors of maximal diameter ≥ 3–3.9 cm and ≥ 4 cm, respectively) present considerable surgical challenges regarding the extent of resection and perioperative morbidity. Endoscopic endonasal resection is considered the most effective treatment for pituitary adenomas. It allows for better visualization, maneuverability, and access to distant and lateral tumor compartments, ultimately enhancing the extent of resection. This article evaluates our initial experience with endoscopic endonasal resection of large and giant pituitary adenomas. The clinical outcomes, perioperative complications, and extent of tumor resection would be specifically addressed. Patients and methods: The primary goal of surgery was to decompress the optic pathways, and the secondary goals were to achieve maximal safe resection and hormonal control in hormone-secreting adenomas. The degree of tumor resection was classified as gross-total resection (100%), near-total resection (90–100%), subtotal resection (70–90%), and partial resection (< 70%). Results: 42 patients were included in this study. A good visual outcome achieved with 80% improvement in visual symptoms. Gross-total resection (GTR) was achieved in 19 patients (45.2%), near-total resection (NTR) was achieved in 12 patients (28.6%), subtotal resection (STR) in 6 patients (14.3%), and partial resection in the remaining 5 patients (11.9%). Subgroup analysis revealed that GTR, NTR rates were higher in large, compared to giant tumors. GTR, NTR rates of large adenomas were 59.3%, and 29.6%, compared to 20%, and 26.7% in giant adenomas respectively (p-value: 0.01428). Surgical complications were observed in 19 patients (45.2%) with CSF leakage being the most common complication (11 patients, 26.2%). Post-operative diabetes insipidus was observed in 5 patients (11.9%), major vascular injury in one case (2.4%), transient post-op 6th nerve palsy observed in 3 patients (7.1%), while two patients (4.8%) presented with post-operative paranasal sinuses infection. Conclusions: Endoscopic endonasal transsphenoidal resection of large and giant pituitary adenomas is a safe and efficient procedure. Large adenomas (3–3.9 cm) have excellent resection rates and lower complications than giant adenomas (≥ 4 cm), which may require extending our approach to achieve more tumor resection rates in the future. However, only adequate resection of these giant adenomas can be enough to achieve the main surgical goals of visual improvement, hormonal control, and decompression of surrounding structures. [ABSTRACT FROM AUTHOR]

Endonasal endoscopic transsphenoidal surgery (TSS) and resection of pituitary adenomas are considered the gold standard treatment for Cushing disease (CD). Even with various recent advances in management, disease persistence and recurrence are common in these patients. The remission rate in the global population after surgery has been reported to vary widely from 64% to 93%. This study aims to determine the various clinical, biochemical, radiological, and histological factors that correlate with persistence and recurrence in patients with CD. This study also aims to understand the clinicopathological significance of EGFR-MAPK, NF-κB, and SHH pathway activation and to study the protein expression of activation markers of these pathways (i.e., c-Fos, c-Jun, GLI-1, pMEK, NR4A1, and p44) in functioning corticotroph pituitary adenomas. From January 2009 to September 2022, the clinical data of 167 patients who underwent surgical treatment (n = 174 surgeries) for CD with a median follow-up of 8.1 years (range, 1–13.29 years) were ambispectively analyzed. The preoperative clinical, biochemical, and radiological features, operative findings, postoperative clinical and biochemical data, and histopathological and molecular profiles were retrieved from the electronic medical records. The patients were followed up to assess their remission status. Among the 174 surgeries performed, 140 were primary surgeries, 22 were revision surgeries, 24 surgeries were for pediatric patients, and 12 surgeries were for patients with Nelson syndrome. In the primary surgery cohort, 74.3% were female, and the average age was 28.73 ± 10.15 years. Of the primary surgery cohort, 75% of the patients experienced remission compared with 47.4% after revision surgery. The remission rate for the pediatric patients was 55.5%. The postoperative day 1 plasma cortisol (P < 0.001; area under the curve, 0.8894; range, 0.8087–0.9701) and adrenocorticotropic hormone (P < 0.001; area under the curve, 0.9; range, 0.7386–1) levels were seen to be strong independent predictors of remission in the primary surgery cohort. The remission rate after endoscopic TSS was greater than that after microscopic TSS in patients undergoing primary surgery (81.08% vs. 57.14%; P = 0.008). The presence of adenoma on histopathological examination (HPE) was also a strong predictor of disease remission (P = 0.020). On stratifying by surgical approach and HPE, microscopically operated patients without histopathological evidence of adenoma had significantly higher odds of nonremission (odds ratio, 38.1; 95% confidence interval, 4.2–348.3) compared with endoscopically operated patients with adenoma found on HPE. A lower immunoreactivity score for NR4A1 was found to correlate with higher remission rates (P = 0.074). However, none of the molecular markers studied (i.e., c-Fos, c-Jun, GLI-1, pMEK, and p44) showed a significant correlation with the preoperative cortisol values. The remission rate after primary surgery is higher than that after revision surgery and is lower for pediatric patients than for adults. The postoperative day 1 plasma cortisol and adrenocorticotropic hormone levels are strong independent predictors of remission in the primary surgery cohort. An endoscopic approach with histopathological evidence of adenoma is associated with a higher remission rate; thus, endoscopy should be the approach of choice for these patients with the goal of identification of an adenoma on HPE. [ABSTRACT FROM AUTHOR]

Background: Invasion of the CS is one of the limiting factors for total resection for PitNet tumors with cure rates less than 30%. Extended approaches may be considered in selective and well-studied cases of secreting adenomas. Method: We describe the key steps of the endoscopic transcavernous approach for functional pituitary adenomas with a video illustration. The surgical anatomy is described along with the advantages and limitations of this approach. Conclusion: A detailed knowledge of CS anatomy and familiarity with this surgical approach acquired in the laboratory is essential. Proper instrumentation is critical to decrease the risks of vascular injury. [ABSTRACT FROM AUTHOR]

Background The coexistence of two histologically distinct neoplasms in the same area without histological admixture or an intermediate cell population zone represents a rare tumor type called collision tumor. Collision tumor of pituitary adenoma and meningioma has been reported years later following irradiation to pituitary adenoma. However, collision tumor of pituitary adenoma and meningioma in absence of irradiation therapy is extremely uncommon. Case Description We report an unusual case of collision tumor involving diaphragma sella meningioma and pituitary adenoma in a 50-year-old lady without prior radiation therapy. She presented with visual blurring and impaired field of vision. Her preoperative magnetic resonance imaging (MRI) was suggestive of pituitary adenoma. Total excision of the lesion was performed through endoscopic transsphenoidal route. Histological diagnosis was consistent with collision tumor of pituitary adenoma and meningioma. Conclusion Collision tumor comprising of nonfunctioning pituitary adenoma and meningioma is extremely rare. Preoperative MRI may not always be able to distinguish these histologically distinct neoplasms. Hence, histopathological examination is necessary to establish the diagnosis. Endoscopic transsphenoidal approach may suffice in excision of these collision tumors. Close follow-up is necessary to detect tumor recurrence. Though the association of these tumors can be coincidental, casual relationship between the occurrence of collision tumors cannot be totally excluded. [ABSTRACT FROM AUTHOR]

The new World Health Organization nomenclature of pituitary tumors was introduced in the year 2022 after much deliberation. This nomenclature clearly demarcates the anterior lobe (adenohypophyseal), posterior lobe (neurohypophyseal), and hypothalamic tumors. There is also focus on other tumors arising in the sellar region. The nomenclature has also advocated the routine use of immunohistochemistry in describing the pituitary transcription factors that plays a fundamental role in distinguishing the cell lineage of these tumors. However, the nomenclature is complex in understanding due to inclusion of pathological correlates like transcription factors, hormones, biomarkers, and various controversies that have emerged regarding the renaming of pituitary adenomas (PA) as PiTNETs ("Pituitary Neuroendocrine tumors") because majority of the adenomas are benign and have rare metastatic behavior while classifying them as PiTNETs will create unnecessary misinterpretation of these as aggressive tumors that will lead to apprehension among the patients. The new classification gives deeper insight into the histological picture of the various pituitary tumors but other than contributing to the follow-up strategy and postsurgery management, this classification does not add anything new that could be advantageous for the neurosurgeons in clinical practice and decision making, especially in deciding the plan of action for surgery. Hence, there is need of a more comprehensive, integrated, neuroradiological-based classification with more emphasis on the invasiveness of these tumors that would assist the neurosurgeons in planning the treatment strategy and managing patients of pituitary tumors. [ABSTRACT FROM AUTHOR]

Purpose: To identify differences in the presentation and surgical outcomes between very large (30–39 mm) and giant (≥ 40 mm) (LARGE group) pituitary adenomas (PAs) compared to the smaller group (< 30 mm) (non-LARGE group). Methods: Eighty patients with very large (n = 44) or giant (n = 36) PAs and 226 patients in the non-LARGE group who underwent tumor resection by pituitary surgery between 2008 and 2023 were studied. Hormonal, radiological, ophthalmological, and pathological data, and surgical outcomes were evaluated. Results: Preoperatively, patients of the LARGE group presented more frequently with visual impairment (82.5% vs. 22.1%, P < 0.001) and with pituitary apoplexy (15.0% vs. 2.7%, P < 0.001) than the non-LARGE group. Moreover, the LARGE group were more commonly associated with preoperative panhypopituitarism (28.8% vs. 6.2%, P < 0.001). This group presented cavernous sinus invasion more frequently (71.3% vs. 23.9%, P < 0.001). The non-LARGE group achieved surgical cure more often than the LARGE group (79.7% vs. 50.0%, P < 0.001), and the rate of major complications was higher in the latest (8.8% vs. 1.3%, P < 0.004). Conclusions: PAs ≥ 30 mm are most frequently accompanied by hormonal dysfunction, cavernous sinus invasion, and visual impairment. All this implies lower resection rates and higher postoperative complications than the smaller adenomas, posing a real surgical challenge. [ABSTRACT FROM AUTHOR]

Tumours of the anterior part of the pituitary gland represent just 1% of all childhood (aged <15 years) intracranial neoplasms, yet they can confer high morbidity and little evidence and guidance is in place for their management. Between 2014 and 2022, a multidisciplinary expert group systematically developed the first comprehensive clinical practice consensus guideline for children and young people under the age 19 years (hereafter referred to as CYP) presenting with a suspected pituitary adenoma to inform specialist care and improve health outcomes. Through robust literature searches and a Delphi consensus exercise with an international Delphi consensus panel of experts, the available scientific evidence and expert opinions were consolidated into 74 recommendations. Part 1 of this consensus guideline includes 17 pragmatic management recommendations related to clinical care, neuroimaging, visual assessment, histopathology, genetics, pituitary surgery and radiotherapy. While in many aspects the care for CYP is similar to that of adults, key differences exist, particularly in aetiology and presentation. CYP with suspected pituitary adenomas require careful clinical examination, appropriate hormonal work-up, dedicated pituitary imaging and visual assessment. Consideration should be given to the potential for syndromic disease and genetic assessment. Multidisciplinary discussion at both the local and national levels can be key for management. Surgery should be performed in specialist centres. The collection of outcome data on novel modalities of medical treatment, surgical intervention and radiotherapy is essential for optimal future treatment. Evidence and guidance for the care of pituitary adenomas in children and adolescents is currently lacking. Part 1 of this two-part consensus guideline presents 17 general recommendations for diagnosis and management of pituitary adenomas in children and young people under 19 years of age. [ABSTRACT FROM AUTHOR]

Background: Pituitary adenomas are benign brain tumors that impose a heavy burden on patients worldwide. The local burden of disease is yet to be established due to scarcity of data. In line with this, this study aims to present the challenges and gaps in the treatment of pituitary adenomas in the Philippines. Methods: A scoping review of available relevant literature on epidemiology, clinical experience with treatment, health financing, and healthcare delivery system based on the Preferred Reporting Items for Systematic reviews and Meta-analysis guidelines extension for Scoping Reviews was conducted. Results: The scarcity of updated local clinical data, inequity of distribution of resources, inadequate government support, and lack of affordable diagnostic testing, medications, and neurosurgical procedures are the factors that hinder provision of adequate care of pituitary adenomas in the Philippines. Conclusion: There are notable treatment gaps in the management of pituitary adenomas in the Philippines, which may be addressed by strengthening universal healthcare. Strategies to address these gaps were proposed, including improving public-private insurance coverage, increasing manpower, enhancing accessibility to resources, and spreading more awareness. [ABSTRACT FROM AUTHOR]

Background: Coexistence of TSH-secreting pituitary adenoma (TSHoma) and Graves' disease (GD) is rare and complicates the management decision. Methods: We present a case of the co-existence of TSHoma and GD. In addition, we systematically searched articles describing TSHoma and GD in the same patient published until 20th March 2023, using Pubmed, Scopus and Embase. Case presentation: A 46-year-old man presented with symptoms of thyrotoxicosis. His thyroid function tests showed serum TSH 3.35 (reference range 0.3–4.2) mIU/L, FT3 19.7 (3.7–6.4) pmol/L, and FT4 68.9 (11-23.3) pmol/L. The serum TSH receptor antibody was 11.5 mIU/L (positive at ≥ 1.75 mIU/L). Pituitary magnetic resonance imaging showed macroadenoma compressing the optic chiasm. The patient underwent trans-sphenoidal resection of pituitary adenoma. Postoperatively, he remained on maintenance carbimazole and octreotide. Results: Fourteen articles comprising 15 patients were identified from the systemic search. A total of 16 patients (including the current case) were included in the systematic review. The mean (± SD) age at diagnosis was 41 ± 13.6 years. The majority were females (75%). The median (IQR) TSH was 1.95 (0.12–5.5) mIU/L, the median (IQR) free T3 was 11.7 (7.6–19.7) pmol/L and the median (IQR) free T4 level was 47.6 (33.3–64.4) pmol/L. Ten (76.9%) patients had positive TSH receptor antibody levels. 84.6% had pituitary macroadenoma. Pituitary surgery was performed in 12 (75%) patients. At the last follow-up, 4 (25%) patients had complete resolution of symptoms after pituitary surgery, 3 (18.7%) were on maintenance treatment with thionamides for GD, 1 (6.25%) on beta-blockers and 1 (6.25%) on somatostatin analog. Conclusion: TSHoma and GD can co-exist, and it is essential to identify this rare association as it can significantly impact treatment strategies. [ABSTRACT FROM AUTHOR]

Anterior pituitary cell function requires a high level of protein synthesis and secretion which depend heavily on mitochondrial adenosine triphosphate production and functional endoplasmic reticula. Obesity adds stress to tissues, requiring them to adapt to inflammation and oxidative stress, and adding to their allostatic load. We hypothesized that pituitary function is vulnerable to the stress of obesity. Here, we utilized a 10- to 15-week high-fat diet (HFD, 60%) in a thermoneutral environment to promote obesity, testing both male and female FVB.129P mice. We quantified serum hormones and cytokines, characterized the metabolic phenotype, and defined changes in the pituitary transcriptome using single-cell RNA-sequencing analysis. Weight gain was significant by 3 weeks in HFD mice, and by 10 weeks all HFD groups had gained 20 g. HFD females (15 weeks) had increased energy expenditure and decreased activity. All HFD groups showed increases in serum leptin and decreases in adiponectin. HFD caused increased inflammatory markers: interleukin-6, resistin, monocyte chemoattractant protein-1, and tumor necrosis factorα. HFD males and females also had increased insulin and increased TSH, and HFD females had decreased serum prolactin and growth hormone pulse amplitude. Pituitary single-cell transcriptomics revealed modest or no changes in pituitary cell gene expression from HFD males after 10 or 15 weeks or from HFD females after 10 weeks. However, HFD females (15 weeks) showed significant numbers of differentially expressed genes in lactotropes and pituitary stem cells. Collectively, these studies reveal that pituitary cells from males appear to be more resilient to the oxidative stress of obesity than females and identify the most vulnerable pituitary cell populations in females. [ABSTRACT FROM AUTHOR]

Bilateral thalamic stroke is usually due to compromised artery of Percheron, an anatomical variation of the vascular supply of the thalamus. The stroke in this area is very uncommon, and is mainly due to top of the basilar syndrome. Other causes are extremely rare. We describe the case of a patient with a pituitary adenoma who underwent surgery and later presented with a bilateral thalamic infarct, suggesting compromise of the artery of Percheron. This would be the third case published in the literature about this complication. We present a literature review about the vascular supply of the thalamus, the artery of Percheron, and its involvement in pituitary surgery. [ABSTRACT FROM AUTHOR]

This review presents a comprehensive analysis of pituitary adenomas, a type of brain tumor with diverse behaviors and complexities. We cover various treatment approaches, including surgery, radiotherapy, chemotherapy, and their integration with newer treatments. Key to the discussion is the role of biomarkers in oncology for risk assessment, diagnosis, prognosis, and the monitoring of pituitary adenomas. We highlight advances in genomic, epigenomic, and transcriptomic analyses and their contributions to understanding the pathogenesis and molecular pathology of these tumors. Special attention is given to the molecular mechanisms, including the impact of epigenetic factors like histone modifications, DNA methylation, and transcriptomic changes on different subtypes of pituitary adenomas. The importance of the tumor immune microenvironment in tumor behavior and treatment response is thoroughly analyzed. We highlight potential breakthroughs and innovations for a more effective management and treatment of pituitary adenomas, while shedding light on the ongoing need for research and development in this field to translate scientific knowledge into clinical advancements, aiming to improve patient outcomes. [ABSTRACT FROM AUTHOR]

Cushing's disease (CD) is an uncommon yet serious disorder, linked to increased morbidity and mortality. Diagnosis of these patients are complex, and emphasis should be placed on early detection for better management of these patients. The objective of this case report is to evaluate the diagnostic elements in the identification of a subcentimeter adrenocorticotrophic hormone (ACTH)-dependent pituitary adenoma. We report a case of a 26-year-old man who presented with extensive superficial fungal infection of the skin, unintentional weight gain, hypertension, dyslipidaemia as well as cushingoid appearance suspicious of hypercortisolism. Raised midnight plasma cortisol and 24-hour urinary cortisol levels warranted further investigation. Overnight and 48-hour low-dose dexamethasone suppression test (LDDST) were non-suppressed. Subsequent bilateral inferior petrosal sinus sampling (BIPSS) was not only efficient in differentiating pituitary from ectopic Cushing's syndrome (CS) but was shown to be superior to magnetic resonance imaging (MRI) in lateralisation of the pituitary adenoma. [ABSTRACT FROM AUTHOR]

Fractionated stereotactic radiotherapy (FSRT) is a common modality used to treat pituitary adenomas with good control rates. It is not known whether FSRT should be performed early or delayed until progression occurs. We compared FSRT in treating nonfunctional pituitary adenomas (NFPA) as an adjuvant (ADJ) or on-progression (PRG) therapy. A retrospective review of patients who underwent FSRT for an NFPA between January 2004 and December 2022 at a single institution was performed. We compared endocrinologic, ophthalmologic, and radiographic outcomes in FSRT delivered as ADJ and PRG treatment. Seventy-five patients were analyzed, with a median follow-up of 53 months. FSRT was administered to 35 and 40 patients as ADJ and PRG, with a median time to treatment of 5.5 and 40 months, respectively. The tumor control rate was 94.3% for ADJ and 95.0% for PRG. Treatment resulted in 4 (11.4%) versus 7 (17.5%) new endocrinopathies and 2 (5.7%) versus 1 (2.5%) new visual deficits for ADJ versus PRG. A survival analysis of time to new endocrinopathy showed no difference between the 2 cohorts. The median time from surgery to new endocrinopathy was significantly different between ADJ and PRG, at 15.5 and 102.0 months, respectively. FSRT is effective in treating NFPA for residual and progressive tumors, with excellent tumor control rates and a low risk of developing new endocrinopathies and visual deficits. Delaying treatment delayed the development of new endocrinopathies, suggesting that observation with FSRT on tumor progression may delay the onset of hypopituitarism and maintain similar effectiveness in tumor control. [ABSTRACT FROM AUTHOR]

We successfully removed a rare ectopic pituitary adenoma from a 64-year-old female patient initially misdiagnosed with a chordoma. The tumor was located in the slope region and posed a challenge due to its rarity and location. Using neuro-navigation and neuro-endoscopic techniques, we performed a transnasal transsphenoidal resection and confirmed postoperatively that the tumor was a non-functional pituitary adenoma. Although the patient experienced temporary cortisol deficiency after surgery, she recovered well. To accurately diagnose ectopic pituitary adenomas of clivus, doctors must carefully evaluate the patient's imaging results and hormone levels. If necessary, nuclear medicine scans can also aid in diagnosis. Endoscopic transnasal-transsphenoidal surgery is the preferred treatment method, and preoperative multimodal imaging evaluation and intraoperative neuro-navigation technology are essential for a successful surgery. [ABSTRACT FROM AUTHOR]

Purpose: To clarify the invasiveness to surrounding structures and recurrence rate of each subtype of nonfunctioning pituitary neuroendocrine tumor (Pit-NETs) according to the WHO 2022 classification. Methods: This retrospective study utilized data from 292 patients with nonfunctioning Pit-NETs treated with initial transsphenoidal surgery. Recurrence was evaluated on 113 patients who were available for a magnetic resonance imaging follow-up ≥ 60 months. All tumors were assessed by immunohistochemical staining for Pit-1, T-PIT, and GATA3. Invasiveness to surrounding structures was evaluated based on intraoperative findings. Results: Cavernous sinus invasion was found in 47.5% of null cell tumors, 50.0% of Pit-1 lineage tumors, 31.8% of corticotroph tumors, and 18.3% of gonadotroph tumors. Dura mater defects in the floor of sellar turcica, indicating dural invasion, were found in 44.3% of null cell tumors, 36.4% of corticotroph tumors, 16.7% of Pit-1 lineage tumors, and 17.3% of gonadotroph tumors. In logistic regression analysis, Pit-1 (OR 5.90, 95% CI 1.71–20.4, P = 0.0050) and null tumors (OR 4.14, 95% CI 1.86–9.23, P = 0.0005) were associated with cavernous sinus invasion. Recurrence was found in 8 (4.9%) patients, but without significant differences between tumor subtypes. The presence of cavernous sinus invasion was correlated with recurrence (HR = 1.95, 95% CI 1.10–3.46, P = 0.0227). Conclusion: Among nonfunctioning Pit-NETs, Pit-1 lineage tumors tend to invade the cavernous sinus, corticotroph tumors may produce dura mater defects, and null cell tumors tend to cause both. Pit-NETs with cavernous sinus invasion require a careful attention to recurrence. [ABSTRACT FROM AUTHOR]

An ectopic pituitary adenoma (EPA) is an uncommon type of pituitary adenoma, accounting for only 2% of all pituitary adenomas. EPAs are benign tumors that can occur anywhere along the migratory embryonic path of the pituitary gland and have no relationship to intrasellar elements. They are usually hormonally active and have a minor female predominance. The clinical features of EPAs are highly dependent on its hormonal activity, anatomical location, and its local mass effect. Appropriate radiological imaging is essential for the evaluation of EPAs. Imaging investigations show a normal pituitary gland and sellar turcica, provide details on the size of the tumor, its margins, and extent, and help with surgical planning. The criteria for diagnosing an ectopic pituitary adenoma depend on detailed histopathological examination. EPA management should be individualized. We present a case of a 71-year-old male who presented with a 9-month history of left nasal obstruction, purulent nasal discharge, and intermittent anterior epistaxis. The patient was being managed by his general practitioner for chronic rhinosinusitis but failure of his symptoms to resolve prompted a visit to the otorhinolaryngologist. The patient was diagnosed with a null-cell ectopic pituitary adenoma through histological analysis of a biopsy specimen that showed adenohypophyseal cells without cell-type-specific differentiation. The patient subsequently underwent an endoscopic endonasal excision and had an uneventful hospital stay. [ABSTRACT FROM AUTHOR]

This article describes a case of a 13-year-old boy who was diagnosed with resistance to thyroid hormone (RTH) and also had a nonfunctioning pituitary microadenoma. This is the first reported case of a pediatric patient with both RTH and a nonfunctioning pituitary microadenoma. RTH is a rare syndrome characterized by elevated levels of free iodothyronine and non-suppressed thyroid stimulating hormone (TSH), and it is usually caused by mutations in the THRB gene. Accurate differential diagnosis is important to determine the appropriate treatment, as patients with RTH do not require treatment while patients with TSHoma (TSH-secreting pituitary adenoma) may require surgical removal. The patient in this case had a THRB gene mutation and a nonfunctioning pituitary microadenoma, and his symptoms improved with the appropriate treatment. [Extracted from the article]

Primary hyperparathyroidism secondary to ectopic parathyroid (in anterior mediastinum) is rare in children. We report the case of a 12-year-old girl with a history of multiple fractures, renal calculi and limb deformities. She was diagnosed with hyperparathyroidism secondary to an intrathymic parathyroid adenoma. Sestamibi scan showed a lesion in the anterior mediastinum. A biochemical evaluation revealed hypercalcaemia, elevated alkaline phosphatase and parathormone levels. The lesion was marked with radioisotope and confirmed intraoperatively using a gamma camera. The child underwent thoracoscopic left thymectomy with the adenoma. Immediate decrease in calcium and parathyroid hormone values were noted intraoperatively and serial monitoring showed a downward trend. On follow-up, the child is doing well. Ectopic parathyroid adenoma is very rare. CT with radioisotope scans is helpful in diagnosis. Thoracoscopic excision of ectopic adenoma is safe in children. [ABSTRACT FROM AUTHOR]

The development of adenohypophysis by differentiating and detaching from the pharyngeal roof results in formation of a functional ectopic pituitary tissue around the Sella turcica. Of the ectopic sites in which pituitary adenoma occurs, clival adenomas are rare and the majority secrete prolactin. We report a case of ACTH-dependent Cushing syndrome. Magnetic resonance imaging revealed a clival mass with sphenoid sinus infiltration and 68Gallium (Ga) Dodecane tetra acetic acid—NaI3—octreotide positron emission tomography-computed tomography showed intense uptake in the region. Postoperative immunohistochemistry revealed ACTH and T-box Protein in T-cell positivity. With literature review, we found 5 reported cases of clival ectopic pituitary adenoma with Cushing syndrome. Clinical characteristics, imaging features, histology, and management of such masses have been discussed. In conclusion, ACTH-producing clival ectopic pituitary adenomas are rare, require differentiation from neuroendocrine tumors, and remit by multimodal therapy. [ABSTRACT FROM AUTHOR]

Purpose: The main purpose of this study is to understand the characteristics and management of sinonasal small round blue cell tumors and also to emphasise the role of immunohistochemistry in their diagnosis and on the outcomes after endoscopic/open excision in these patients. Methods: This is a retrospective study conducted at a tertiary care referral centre in India which included 38 patients with sino nasal for a period of 5 years. All the patients were evaluated clinically and radiologically. All cases were confirmed diagnostically with histopathological examination and immunohistochemistry following surgical excision either by endoscopic or open approach. Some of the cases underwent post operative radiotherapy. Results: In our study, among 176 cases diagnosed with Sino nasal malignancies, 38 (21.6%) cases were diagnosed with sinonasal small round blue cell tumors with male to female ratio 1.4:1. Most common histopathological type among all the sinonasal small round blue cell tumors that presented to us was esthesioneuroblastoma i.e., 8 (21%) patients followed by pituitary macroadenoma in 7(8.4%) patients. Other types are undifferentiated squamous cell carcinoma 10(13.1%), craniopharyngioma 8(10.5%), lymphoma 3(7.9%), synovial/spindle cell sarcoma, malignant melanoma and adenocarcinoma 1(2.6%) each. Schwannoma, rhabdomyosarcoma, neuroendocrine carcinoma and neurofibroma 2 (5.2%) each. Conclusion: Sinonasal small round blue cell tumors are extremely rare tumours. Histopathological diagnosis with immunohistochemistry is characteristic of various tumors and is conclusive for diagnosis. Knowledge of these tumor entity is essential as early diagnosis helps in further management in preventing spread to vital structures and improving outcome. Most of the tumors have a multimodality treatment approach which includes surgical excision, radiotherapy and chemotherapy. [ABSTRACT FROM AUTHOR]

Importance: Benchmarks aid in improve outcomes for surgical procedures. However, best achievable results that have been validated internationally for transsphenoidal surgery (TS) are not available. Objective: We aimed to establish standardized outcome benchmarks for TS of pituitary adenomas. Design: A total of 2685 transsphenoidal tumor resections from 9 expert centers in 3 continents were analyzed. Setting: Patients were risk stratified, and the median values of each center's outcomes were established. The benchmark was defined as the 75th percentile of all median values for a particular outcome. The postoperative benchmark outcomes included surgical factors, endocrinologyspecific values, and neurology-specific values. Results: Of 2685 patients, 1149 (42.8%) defined the low-risk benchmark cohort. Within these benchmark cases, 831 (72.3%) patients underwent microscopic TS, and 308 (26.8%) patients underwent endoscopic endonasal resection. Of all tumors, 799 (29.8%) cases invaded the cavernous sinus. The postoperative complication rate was 19.6% with mortality between 0.0% and 0.8%. Benchmark cutoffs were ≤2.9% for reoperation rate, ≤1.9% for cerebrospinal fluid leak requiring intervention, and ≤15.5% for transient diabetes insipidus. At 6 months, benchmark cutoffs were calculated as follows: readmission rate: ≤6.9%, new hypopituitarism ≤6.0%, and tumor remnant ≤19.2%. Conclusions: This analysis defines benchmark values for TS targeting morbidity and mortality and represents the best outcomes in the best patients in expert centers. These cutoffs can be used to assess different centers, patient populations, and novel surgical techniques. It should be noted that the benchmark values may influence each other and must be evaluated in their own context. [ABSTRACT FROM AUTHOR]

Prolactinomas are the most common pituitary tumor histotype, with microprolactinomas being prevalent in women and macroprolactinomas in men. Hyperprolactinemia is among the most common causes of hypogonadotropic hypogonadism in both sexes, prompting medical advice for hypogonadism (infertility, oligo-amenorrhea, impotence, osteoporosis/osteopenia) in both sexes, and for signs and symptoms of mass effects (hypopituitarism, visual loss, optic chiasm compression, cranial nerve deficits, headaches) predominantly in men. Diagnostic workup involves a single prolactin measurement and pituitary imaging, but some laboratory artifacts (ie, the "hook effect" and macroprolactin) can complicate or delay the diagnosis. The treatment of choice for prolactinomas is represented by dopamine agonists, mainly cabergoline, which are able to induce disease control, restore fertility in both sexes, and definitively cure one-third of patients, thus permitting treatment discontinuation. Pregnancy and menopause may promote spontaneous prolactin decline and anticipate cabergoline discontinuation in women. Surgery and/or radiotherapy are indicated in case of resistance to cabergoline not overcome by the increase in drug dose up to the maximally tolerated or the patient's personal choice of surgery. The evidence of resistance to cabergoline in invasive and proliferative tumors may indicate biological aggressiveness, thus requiring alternative therapeutic approaches mainly based on temozolomide use as monotherapy or combined with radiotherapy. In uncontrolled patients, new medical approaches (alternative hormonal treatments, cytotoxic drugs, peptide receptor radionuclide therapy, mTOR/Akt inhibitors, tyrosine kinase inhibitors, or immunotherapy) may be offered but the experience collected to date is still very scant. This article reviews different facets of prolactinomas and discusses approaches to the condition in more common clinical situations. [ABSTRACT FROM AUTHOR]

We report a case of a 75-year-old patient with hypopituitarism, bitemporal visual field deficits and a parasellar mass on pituitary MRI. During surgery, suspicion was raised that a non-functioning pituitary adenoma was accompanied by an abutting diaphragm sellae meningioma, which was confirmed at pathological examination. In retrospect, the initial MRI suggested two separate tumours on the basis of differing densities but this distinction was not seen on the last preoperative MRI. [ABSTRACT FROM AUTHOR]

Only two aneurysm formations in the internal carotid artery after gamma knife radiosurgery (GKRS) for pituitary adenomas are reported so far. Here, out of the 482 patients who underwent GKRS for pituitary adenomas at our institute, at least five developed aneurysms within the area of high single-dose irradiation. Three patients presented with epistaxis due to aneurysmal rupture and one presented with abducens paralysis due to nerve compression, while one was asymptomatic. The interval between irradiation and aneurysmal detection ranged from 14 to 21 years. Aneurysm formation in those conditions may be higher than previously thought. [ABSTRACT FROM AUTHOR]

Functional gonadotroph adenomas (FGAs) are rare, manifesting symptoms like menstrual irregularities or ovarian hyperstimulation syndrome (OHSS). We present a case of OHSS caused by an FGA during the follow-up of a pituitary tumor initially considered nonfunctioning. The patient presented with lower abdominal pain, abdominal swelling, and dyspnea. Magnetic resonance imaging (MRI) revealed 15 cm enlarged ovarian cysts and pleural effusion. Laboratory examination showed an elevated serum estradiol (E2) level (5741.4 pmol/L [1564.0 pg/mL]), suppressed luteinizing hormone, and nonsuppressed follicular-stimulating hormone (FSH). However, no pituitary hormone disorders were observed when a 19 mm pituitary tumor was discovered 11 months prior. Given the absence of human chorionic gonadotropin (hCG) administration, OHSS due to the FGA was suspected. Cabergoline, known for alleviating the severity of OHSS, was administered, but the ovarian cysts continued to enlarge. Subsequently, endoscopic transsphenoidal surgery was performed, and immunohistochemical analysis confirmed the diagnosis of the FSH-producing adenoma. Follow-up MRI scans showed reduced ovarian cysts and successful pituitary tumor resection with a reduced E2 level. This case highlights the importance of considering FGAs when encountering OHSS without hCG administration or following up on pituitary tumors in premenopausal female patients to take appropriate measures for accurate diagnosis and management. [ABSTRACT FROM AUTHOR]

Pituitary carcinoma is a rare disease with surgical, radiotherapeutic, and chemotherapeutic treatment options. We present the case of a female patient diagnosed with a nonfunctioning pituitary adenoma who underwent several surgical procedures, radiations, and chemotherapeutic treatments with various substances. Sixteen years after the first diagnosis, a cranial and spinal metastatic spread of the tumor occurred. We opted for an individual therapy based on anecdotal evidence. Unfortunately, the recommended off-label treatment with a somatostatin analog substance was never given due to bureaucratic delays. This case report is about the challenging aspects of individual decision-making in rare neurosurgical diseases. [ABSTRACT FROM AUTHOR]

Growth hormone deficiency (GHD) is a common complication in survivors of cancer and patients with tumors of the pituitary region. Growth hormone replacement therapy (GHT) has proven beneficial effects, including increased growth velocity, positive effects on body composition and skeletal integrity, and increased quality of life. However, due to known pro-proliferative, angiogenic, and anti-apoptotic properties of growth hormone, there are still some concerns about the safety of GHT in survivors. This narrative review aims to provide an overview of the long-term sequelae, and subsequently long-term safety, of GHT in survivors of (childhood) cancer and patients with tumors of the pituitary region. We identified predominantly reassuring results regarding the safety of survivors with GHT, although we must take into account the shortcomings of some studies and limited information on adult cancer survivors. Besides the already increased risk for second neoplasms, recurrences, or mortality in survivors due to host-, disease-, and treatmentrelated factors, we could not identify an increased risk due to GHT in particular. Therefore, we support the consensus that GHT can be considered in survivors after careful individual risk/benefit analysis and in open discussion with the patients and their families, taking into account the known morbidity of untreated GHD in cancer survivors and the positive effects of GHT. [ABSTRACT FROM AUTHOR]

'Pituitary tumours' is an umbrella term for various tumours ori ginating from different regions of the hypothalamic--pituitary system. The vast majority of pituitary tumours are pituitary adenomas, also recently referred to as pituitary neuroendocrine tumours. The prevalence of clinically relevant pituitary adenomas is approximately 1 in 1000; other pituitary tumours such as craniopharyngioma and pituicytoma are comparatively very rare. This review addresses the molecular and genetic aspects of pituitary adenomas. We first discuss the germline genetic variants underlying familial pituitary tumours, which account for approximately 5% of all pituitary adenoma cases. This includes variants in established pituitary adenoma/hyperplasia predisposition genes (MEN1, PRKAR1A, AIP, CDKN1B, GPR101, SDHA, SDHB, SDHC, SDHD, SDHAF2) as well as emerging genetic associations. In addition, we discuss McCune--Albright syndrome which lies between the germline and somatic pituitary tumour genes as the causative GNAS mutations are postzygotic rather than being inherited, and the condition is associated with multiglandular features due to the involvement of different cell lines rather than being limited to the pituitary. By contrast, somatic GNAS mutations contribute to sporadic acromegaly. USP8 is the only other gene where somatic driver mutations have been established in sporadic pituitary tumorigenesis. However, there are now known to be a variety of other somatic genetic and molecular changes underpinning sporadic pituitary adenomas which we review here, namely: copy number variation, molecular changes in signalling and hypoxia pathways, epithelial--mesenchymal transition, DNA repair, senescence, the immune microenvironment and epigenetics. [ABSTRACT FROM AUTHOR]

Objectives: Pituitary adenomas can exhibit aggressive behavior, characterized by rapid growth, resistance to conventional treatment, and early recurrence. This study aims to evaluate the clinical value of shape-related features combined with textural features based on conventional MRI in evaluating the aggressiveness of pituitary adenomas and develop the best diagnostic model. Methods: Two hundred forty-six pituitary adenoma patients (84 aggressive, 162 non-aggressive) who underwent preoperative MRI were retrospectively reviewed. The patients were divided into training (n = 193) and testing (n = 53) sets. Clinical information, shape-related, and textural features extracted from the tumor volume on contrast-enhanced T1-weighted images (CE-T1WI), were compared between aggressive and non-aggressive groups. Variables with significant differences were enrolled into Pearson's correlation analysis to weaken multicollinearity. Logistic regression models based on the selected features were constructed to predict tumor aggressiveness under fivefold cross-validation. Results: Sixty-five imaging features, including five shape-related and sixty textural features, were extracted from volumetric CE-T1WI. Forty-seven features were significantly different between aggressive and non-aggressive groups (all p values < 0.05). After feature selection, four features (SHAPE_Sphericity, SHAPE_Compacity, DISCRETIZED_Q3, and DISCRETIZED_Kurtosis) were put into logistic regression analysis. Based on the combination of these features and Knosp grade, the model yielded an area under the curve value of 0.935, with a sensitivity of 94.4% and a specificity of 82.9%, to discriminate between aggressive and non-aggressive pituitary adenomas in the testing set. Conclusion: The radiomic model based on tumor shape and textural features study from CE-T1WI might potentially assist in the preoperative aggressiveness diagnosis of pituitary adenomas. Key Points: • Pituitary adenomas with aggressive behavior exhibit rapid growth, resistance to conventional treatment, and early recurrence despite gross resection and may require multiline treatments. • Shape-related features and texture features based on CE-T1WI were significantly correlated with the Ki-67 labeling index, mitotic count, and p53 expression, and the proposed model achieved a favorable prediction of the aggressiveness of PAs with an AUC value of 0.935. • The prediction model might provide valuable guidance for individualized treatment in patients with PAs. [ABSTRACT FROM AUTHOR]

Context. Pituitary adenomas are benign tumors, usually found in men in their 3rd and 5th decades of life, representing 10-15% of all intracranial tumors. The clinical manifestations include important endocrinological disturbances and visual impairment. Objective. This study aimed to determine the most suitable neurosurgical approach regarding the dimensions, extensions and invasiveness of tumor extensions. Design. This was a systematic review of the literature from 2002-2022, focused on clinical outcome, especially endocrinological state according to the surgical approach. Subjects and Methods. We performed an advanced search on Web of Science and PubMed databases on October 10th, 2022. The literature showed 300 studies in the last 20 years, and after we applied the inclusion and exclusion criteria’s, 19 studies were fully read and analyzed. Results. Postoperative complications were reviewed in each surgical approach group, including visual impairment, new endocrinological disturbances, diabetes insipidus and cerebrospinal fluid leakage. Analyze of the endocrinological findings did not determined differences in transcranial groups from transsphenoidal groups. Overall complications were identified in the transcranial cohorts, while cerebrospinal fluid leakage still represent the main problem in transsphenoidal groups. The majority of studies found included extended endoscopic transsphenoidal approach, which shows results of great potential. Conclusions. For the surgical treatment of pituitary adenoma, transsphenoidal procedure with or without extended approaches is preferred, but they’re cases when a craniotomy is mandatory for a feasible gross tumor resection. Combined “above and below” simultaneous procedure or a two-staged intervention is recommended for giant pituitary adenoma, to maximize tumor resection and lower the risk of cerebrospinal fluid leakage [ABSTRACT FROM AUTHOR]

Pituitary metastases are rare. Until now, few cases have been reported; about 50% of pituitary metastases originate from breast or lung cancers. We describe the clinical case of a primary colon carcinoma first presenting with a pituitary metastasis. A 76-year-old woman, with no history of malignancy, presented with headache, dizziness, and diplopia, at the Emergency Department. The neurologic examination was remarkable for complete left ophthalmoplegia with sensitivity deficit on the left side of the face. Radiologic investigations documented a voluminous sellar and suprasellar lesion, with extension in the left cavernous sinus and temporal lobe. Pituitary hormone levels were suggestive of anterior hypopituitarism and mild hyperprolactinemia. Subtotal surgical removal of the lesion was achieved through a trans-sphenoidal endoscopic endonasal approach. The histological examination disclosed a metastasis of gastrointestinal adenocarcinoma. A subsequent colonoscopy identified right colon cancer. A contrasted total-body computerized tomography ruled out other metastases. Postsurgical MRI showed a stable parasellar residual tumor. Conventional radiotherapy was scheduled. This case underlines the importance of considering pituitary metastases in the differential diagnosis of aggressive pituitary lesions, which should be managed in a pituitary tumor center of excellence through a multidisciplinary approach, for the complexity in diagnosis and therapeutic management of this rare condition. [ABSTRACT FROM AUTHOR]

Pituitary adenomas are benign brain tumors that comprise  10%–20% of all central nervous system neoplasms. In recent years, stereotactic radiosurgery (SRS) has emerged as a highly effective treatment option in the management of functioning and nonfunctioning adenomas. It is associated with tumor control rates frequently ranging from 80% to 90% in published reports. While permanent morbidity is uncommon, potential side effects include endocrine dysfunction, visual field deficits, and cranial nerve neuropathies. In patients where single fraction SRS would pose an unacceptable risk (e.g. large lesion size or close proximity to the optic apparatus), hypofractionated SRS delivered in 1–5 fractions is a potential treatment option; however, available data are limited. A comprehensive literature search of PubMed/MEDLINE, CINAHL, Embase, and the Cochrane Library was conducted to identify articles reporting on the use of SRS in functioning and nonfunctioning pituitary adenomas. [ABSTRACT FROM AUTHOR]

Background: Thyroid-stimulating hormone (TSH)-secreting pituitary adenomas (TSHomas) are rare and usually present with hyperthyroidism. Calcification in pituitary tumors is an infrequent finding. Herein, we report an extremely rare case of TSHoma with diffuse calcification. Case description: A 43-year-old man was admitted to our department with a complaint of palpitations. An endocrinological examination revealed elevated serum levels of TSH, free triiodothyronine (FT3), and free thyroxin, whereas the physical examination revealed no obvious abnormality. Computerized tomography (CT) showed a sellar mass with diffuse calcification. Contrastenhanced T1-weighted images revealed a less-enhancing tumor without obvious suprasellar or parasellar expansion. The tumor was completely removed via endoscopic transnasal-sphenoidal surgery. Microscopically, nests of cells were inconspicuous among the diffuse psammoma bodies. Expression of TSH was patchy, and only several TSH-positive cells were observed. Postoperatively, the serum levels of TSH, FT3, and FT4 decreased to their normal range. Follow-up MR images showed no evidence of residual tumor or regrowth after the resection. Conclusions: Herein, we report a rare case of TSHoma with diffuse calcification that presented with hyperthyroidism. A correct and early diagnosis was made according to the European Thyroid Association guidelines. This tumor was completely removed via endoscopic transnasal-transsphenoidal surgery (eTSS), and thyroid function was normalized after the operation. [ABSTRACT FROM AUTHOR]

Background: Pituitary apoplexy is acute infarction with or without hemorrhage of the pituitary gland. It is a rare but potentially life-threatening emergency that most commonly occurs in the setting of pituitary adenoma. The mechanisms underlying pituitary apoplexy are not well understood, but are proposed to include factors of both hemodynamic supply and adenoma demand. In the case of patients with known pituitary macroadenomas undergoing major surgery for other indications, there is a theoretically increased risk of apoplexy in the setting of "surgical stress." However, risk stratification of patients with nonfunctioning pituitary adenomas prior to major surgery is challenging because the precipitating factors for pituitary apoplexy are not completely understood. Here we present a case in which intraoperative hypovolemia is a possible mechanistic precipitating factor for pituitary apoplexy. Case presentation: A 76-year-old patient with a known hypofunctioning pituitary macroadenoma underwent nephrectomy for renal cell carcinoma, during which there was significant intraoperative blood loss. He became symptomatic with ophthalmoplegia on the second postoperative day, and was diagnosed with pituitary apoplexy. He was managed conservatively with cortisol replacement therapy, and underwent therapeutic anticoagulation 2 months after pituitary apoplexy for deep vein thrombosis. His ophthalmoplegia slowly resolved over months of follow-up. Pituitary apoplexy did not recur with therapeutic anticoagulation. Conclusions: When considering the risk of surgery in patients with a known pituitary macroadenoma, an operation with possible high-volume intraoperative blood loss may have increased risk of pituitary apoplexy because intraoperative hypovolemia may precipitate ischemia, infarction, and subsequent hemorrhage. This may be particularly relevant in the cases of elective surgery. Additionally, we found that we were able to therapeutically anticoagulate a patient 2 months after pituitary apoplexy for the management of deep vein thrombosis without recurrence of pituitary apoplexy. [ABSTRACT FROM AUTHOR]

Object: This study aimed to explore the relationship between the aggressiveness and immune cell infiltration in pituitary adenoma (PA) and to provide the basis for immuno-targeting therapies. Methods: One hundred and three patients with PA who underwent surgery at a single institution were retrospectively identified. The infiltration of macrophages and T-lymphocytes was quantitatively assessed. Results: The number of CD68+ macrophages was positively correlated with Knosp (p = 0.003) and MMP-9 expression grades (p = 0.00). The infiltration of CD163+ macrophages differed among Knosp (p = 0.022) and MMP-9 grades (p = 0.04). CD8+ tumor-infiltrating lymphocytes (TILs) were also positively associated with Knosp (p = 0.002) and MMP-9 grades (p = 0.01). Interestingly, MGMT expression was positively correlated with MMP-9 staining extent (p = 0.000). The quantities of CD8+ TILs (p = 0.016), CD68+ macrophages (p = 0.000), and CD163+ macrophages (p = 0.043) were negatively associated with MGMT expression levels. The number of CD68+ macrophages in the PD-L1 negative group was significantly more than that in the PD-L1 positive group (p = 0.01). The rate of PD-L1 positivity was positively correlated with the Ki-67 index (p = 0.046) and p53 expression (p = 0.029). Conclusion: Targeted therapy for macrophages and CD8+ TILs could be a helpful treatment in the future for aggressive PA. Anti-PD-L1 therapy may better respond to PAs with higher Ki-67 and p53 expression and more infiltrating CD68+ macrophages. Multiple treatment modalities, especially combined with immunotherapy could become a novel therapeutic strategy for aggressive PA. [ABSTRACT FROM AUTHOR]

Pituitary metastasis (PM) is an uncommon manifestation of systemic malignant tumours. It is the least common site of intracranial metastases. As PM has no clinical or radiological pathognomonic features, their diagnosis is challenging. Herein, we present a rare case of a PM unveiling lung cancer. A 60‐year‐old male with no medical history of malignancy was admitted with a sudden headache, retro‐orbital pain, and a severe loss of both eyes' visual acuity. After proper investigations and endoscopic resection of the sellar mass, the diagnosis was confirmed to be pituitary metastasis of lung carcinoma. PM can be the initial presentation of an otherwise unknown malignancy. Their diagnosis and management are complex and depend on many factors. Endoscopic surgical resection provides histopathological proof, helps with symptomatic relief, and improves the quality of life but has no effect on survival. [ABSTRACT FROM AUTHOR]

Objective: The aim of the study was to evaluate the association of the thickness of retinal nerve fiber layer (RNFL) with (i) visual symptoms and (ii) suprasellar extension defined by magnetic resonance imaging (MRI) in patients with pituitary macroadenoma. Materials and Methods: RNFL thickness of 50 consecutive patients operated for pituitary macroadenoma between July 2019 and April 2021 were compared with standard visual examination findings and MRI measurements such as optic chiasm height, distance between the optic chiasm and adenoma, suprasellar extension, and chiasmal lift. Results: The study group included 100 eyes of 50 patients operated for pituitary adenomas with suprasellar extension. RNFL thinning predominantly involved the nasal (84.26 ± 16.43 µm) and temporal quadrants (70.72 ± 14.80 µm) and correlated well with the visual field deficit (P < 0.001). Patients with moderate-to-severe deficit in visual acuity had a mean RNFL thickness <85 µm and patients with severe disc pallor had extremely thin RNFLs (<70 µm). Suprasellar extension defined as Wilsons Grade C, D, and E and Fujimotos Grades 3 and 4 were significantly associated with thin RNFLs <85 µm (P < 0.01). Chiasmal lift more than 1 cm and tumor chiasm distance of <0.5 mm were associated with thin RNFL (P < 0.002). Conclusion: RNFL thinning correlates directly with the severity of visual deficits in patients with pituitary adenoma. Wilsons Grade D and E, Fujimoto Grade 3 and 4, chiasmal lift more than 1 cm, and chiasm tumor distance <0.5 mm are strong predictors of RNFL thinning and poor vision. Pituitary macro adenoma and other suprasellar tumors need to be excluded in patients with preserved vision but having obvious RNFL thinning. [ABSTRACT FROM AUTHOR]

Pituitary metastases are rare. Clinical presentation could range from asymptomatic to panhypopituitarism or local symptoms. We present a case report of a 43‐year‐old male patient with a new onset headache, visual disturbances, and panhypopituitarism. The investigation led to the diagnosis of pituitary metastasis as the first manifestation of underlying lung cancer. With rising incidence of breast and lung malignancy, headache/visual field disturbance or signs of pituitary hormone deficiency might be the presenting feature of pituitary metastatic disease. A rapid growth of a sellar lesion and/or the presence of diabetes insipidus along with a sellar mass should raise the suspicion for malignancy. [ABSTRACT FROM AUTHOR]

Objective: When the lesions in the sellar region are large, they can involve both the inside and outside the sella, which brings challenges to the differential diagnosis of pituitary macroadenoma and lesions other than macroadenoma. Therefore, this study explored the diagnostic value of an ectopic posterior pituitary hyperintense signal (EPPHS) in pituitary macroadenoma and its possible causes. Methods: The clinical and imaging data of 131 patients with sellar tumors or tumor-like lesions involving both intrasellar and extrasellar regions in the Affiliated Hospital of Guizhou Medical University from February 2011 to December 2021 were analyzed retrospectively. The diagnostic value of EPPHS in pituitary macroadenoma was analyzed. The differences in clinical and imaging indexes between the EPPHS-positive group and the EPPHSnegative group were compared. Results: These 131 cases of sellar tumors or tumor-like lesions involving both intrasellar and extrasellar regions included 91 cases of pituitary macroadenoma and 40 cases of lesions other than macroadenoma. The receiver operator characteristic (ROC) curve analysis suggested that EPPHS had a diagnostic value in diagnosing pituitary macroadenoma [area under the curve (AUC) = 0.857, P = 0.0001]. Compared with the EPPHS negative group, the median prolactin level in the EPPHS positive group was significantly higher (P < 0.05). Through ROC curve analysis, prolactin value was found to be of diagnostic value for EPPHS (AUC = 0.612, P = 0.0312). Conclusion: In sellar tumors or tumor-like lesions involving both intrasellar and extrasellar regions, the appearance of EPPHS is helpful in the diagnosis of pituitary macroadenoma. The formation of EPPHS may be related to injuries to the pituitary stalk. [ABSTRACT FROM AUTHOR]

To analyze surgical outcomes and tumor characteristics of 49 patients with thyrotropin-secreting pituitary adenoma, a rare functional pituitary adenoma subtype with challenging surgery, who underwent endoscopic endonasal transsphenoidal surgery. In this single-center study, clinical, radiological, surgical, and endocrinological data of 49 patients diagnosed with thyrotropin-secreting pituitary adenoma were retrospectively reviewed. Mean tumor size was 21.12 mm (6 microadenomas, 41 macroadenomas, 2 giant adenomas). Cavernous sinus invasion was present in 12 (24.48%) patients. Tumor consistency was firm in 23 (46.93%) patients. Plurihormonal secretion characteristics were present in 9 patients (5 growth hormone + thyrotropin and 4 growth hormone + prolactin + thyrotropin). Mean follow-up duration was 51.73 months. Of the cases with firm tumors, 14 patients had a history of antithyroid drug use; there was no statistically significant correlation between the antithyroid medication and the firmness of the tumor. Gross total resection was achieved in 32 (65.30%) cases, near-total resection was achieved in 14 (28.57%) cases, and subtotal resection was achieved in 3 (6.12%) cases. The analysis showed that the negative effect of >2 cm tumor size and cavernous sinus invasion on resection rate was statistically significant (P < 0.001). Although early remission was achieved in 33 (67.34%) patients, 44 (89.79%) patients were in euthyroid state at >3 months' follow-up. Complications were 4 new-onset pituitary deficiencies, 3 cases of permanent diabetes insipidus, 1 thyrotoxic crisis, and 1 cerebrospinal fluid leak. Endoscopic endonasal transsphenoidal surgery is the first-line treatment in thyrotropin-secreting pituitary adenoma and should be performed in a tertiary center. Gross total resection is the goal, and early diagnosis is essential for surgical success. [ABSTRACT FROM AUTHOR]

Clinical endocrinology entails an understanding of the mechanisms involved in the regulation of tumors that occur in the endocrine system. The exact cause of endocrine cancers remains an enigma, especially when discriminating malignant lesions from benign ones and early diagnosis. In the past few years, the concepts of personalized medicine and metabolomics have gained great popularity in cancer research. In this systematic review, we discussed the clinical metabolomics studies in the diagnosis of endocrine cancers within the last 12 years. Cancer metabolomic studies were largely conducted using nuclear magnetic resonance (NMR) and mass spectrometry (MS) combined with separation techniques such as gas chromatography (GC) and liquid chromatography (LC). Our findings revealed that the majority of the metabolomics studies were conducted on tissue, serum/plasma, and urine samples. Studies most frequently emphasized thyroid cancer, adrenal cancer, and pituitary cancer. Altogether, analytical hyphenated techniques and chemometrics are promising tools in unveiling biomarkers in endocrine cancer and its metabolism disorders. [ABSTRACT FROM AUTHOR]

Background: Pituitary metastasis accounts for a very low percentage of cases of brain metastasis from lung cancer, and there are uncertainties and challenges in diagnosis and treatment. We hope to shed some light on the diagnosis and treatment by reporting a case of ALK fusion mutation-positive lung cancer pituitary metastasis. Case presentation: We report a 48-year-old female patient with an initial diagnosis of stage IVB lung adenocarcinoma with ALK fusion. The patient developed headache, dizziness, hypopituitarism and hyperprolactinemia one year after treatment with crizotinib. Later, the patient underwent neurosurgical resection of the pituitary tumor and then symptomatic relief. Postoperative pathology suggested pituitary metastasis, and the next-generation gene sequencing conducted on the pituitary metastasis indicated that secondary drug resistance mutation ALK-I1171s occurred after the ALK fusion gene. Conclusion: In this article, we present a patient with suspected pituitary metastases with lung cancer. The progression to pituitary mass resection and next-generation gene sequencing of the pituitary metastasis are suggestive for further diagnosis and treatment. [ABSTRACT FROM AUTHOR]

Objective: To describe clinical and pathological characteristics and treatment outcomes in a large cohort of aggressive pituitary tumours (APT)/pituitary carcinomas (PC). Design: Electronic survey August 2020-May 2021. Results: 96% of 171 (121 APT, 50 PC), initially presented as macro/giant tumours, 6 were microadenomas (5 corticotroph). Ninety-seven tumours, initially considered clinically benign, demonstrated aggressive behaviour after 5.5 years (IQR: 2.8-12). Of the patients, 63% were men. Adrenocorticotrophic hormone (ACTH)-secreting tumours constituted 30% of the APT/PC, and the gonadotroph subtypes were under-represented. Five out of 13 silent corticotroph tumours and 2/6 silent somatotroph tumours became secreting. Metastases were observed after median 6.3 years (IQR 3.7-12.1) from diagnosis. At the first surgery, the Ki67 index was =3% in 74/93 (80%) and =10% in 38/93 (41%) tumours. An absolute increase of Ki67 = 10% after median of 6 years from the first surgery occurred in 18/49 examined tumours. Tumours with an aggressive course from outset had higher Ki67, mitotic counts, and p53. Temozolomide treatment in 156/171 patients resulted in complete response in 9.6%, partial response in 30.1%, stable disease in 28.1%, and progressive disease in 32.2% of the patients. Treatment with bevacizumab, immune checkpoint inhibitors, and peptide receptor radionuclide therapy resulted in partial regression in 1/10, 1/6, and 3/11, respectively. Median survival in APT and PC was 17.2 and 11.3 years, respectively. Tumours with Ki67 = 10% and ACTH-secretion were associated with worse prognosis. Conclusion: APT/PCs exhibit a wide and challenging spectrum of behaviour. Temozolomide is the first-line chemotherapy, and other oncological therapies are emerging. Treatment response continues to be difficult to predict with currently studied biomarkers. [ABSTRACT FROM AUTHOR]

Li-Fraumeni syndrome is a cancer predisposition condition associated with various tumor types. We present the case of a 6-year-old boy who initially presented with a pituitary adenoma that was successfully treated with surgery. It ultimately recurred, requiring further surgical intervention followed by proton beam therapy. He later developed a medulloblastoma, and genetic testing revealed TP53 germline mutation. The patient underwent gross total resection of this medulloblastoma, followed by proton-based craniospinal irradiation and adjuvant chemotherapy. He remained disease-free 12 months after radiation and 7 months after chemotherapy. Current literature does not report pituitary adenoma as the initial central nervous manifestation in Li-Fraumeni syndrome. Early genetic testing should be considered in pediatric patients who present with such rare tumor types to help identify cancer predisposing conditions. Furthermore, as evidenced by our case, the management of multiple brain tumors in the pediatric population poses challenges. A multidisciplinary approach involving neurosurgery, pediatric oncology, pathology, and radiation oncology remains crucial to optimize patient outcomes. [ABSTRACT FROM AUTHOR]