Atypical Adenoma Research Articles

Comparative analysis of intraoperative and imaging features of invasive growth in pituitary adenomas.

Most pituitary adenomas (PAs), also termed pituitary neuroendocrine tumors, are benign in nature and can be treated effectively by surgical resection, medical treatment, and in special cases by radiotherapy. However, invasive growth can be an important feature of a more aggressive behavior and adverse prognosis. The extension of PAs into the cavernous sinus can be categorized according to the Knosp criteria on magnetic resonance imaging (MRI). Comparative analyses of MRI features and intraoperative findings of invasive growth regarding different clinical factors are still scarce. We performed a retrospective single-center analysis of 764 PAs that were surgically treated between October 2004 and April 2018. Invasive growth was assessed according to the surgical reports and preoperative MRI (Knosp criteria). Clinical data, such as patient age at diagnosis and gender, histopathological adenoma type, and extent of resection, were collected. Invasive features on MRI were seen in 24.4% (Knosp grades 3A-4, 186/764) of the cases. Intraoperatively, invasion was present in 42.4% (324/764). Complete resection was achieved in 80.0% of adenomas and subtotal resection, in 20.1%. By multivariate analysis, invasion according to intraoperative findings was associated with the sparsely granulated corticotroph (SGCA, P = .0026) and sparsely granulated somatotroph (SGSA, P = .0103) adenoma type as well as age (P = .0287). Radiographic invasion according to Knosp grades 3A-4 correlated with age (P = .0098), SGCAs (P = .0005), SGSAs (P = .0351), and gonadotroph adenomas (P = .0478). Both criteria of invasion correlated with subtotal resection (P = .0001, respectively). Both intraoperative and radiographic signs of invasive growth are high-risk lesions for incomplete extent of resection and occur more frequently in older patients. A particularly high prevalence of invasion can be found in the SGCA and SGSA types. Cavernous sinus invasion is also more common in gonadotroph adenomas. Usage of the Knosp classification is a valuable preoperative estimation tool.

Operative Nuances of Capsular Dissection Techniques and Capsule Morphology in Pituitary Adenomas: Past 1-Year Case Experiences of a Tertiary Center.

The widespread use of endoscope and increased surgical experiences in pituitary adenomas (PAs) has raised the expectations for higher resection and cure rates. The subject that will meet this expectation in all types of adenomas is the capsule and its dissection. The purpose of this study was to go beyond the commonly mentioned pseudocapsule definition for small-sized adenomas in previous studies and to describe the capsule structure in different morphologies present in pituitary adenomas of all sizes. This includes detailing the nuances of capsular dissection (CD) and presenting postoperative surgical outcomes through the experiences of a high-volume tertiary center. In our center, 534 patients underwent endoscopic trans-sphenoidal surgery because of pituitary adenoma in 2022 by the same surgeon. The data of the patients were examined retrospectively. The surgical techniques applied were grouped as adenoma resection only, capsular resection after internal debulking, and direct extracapsular resection. CD was performed in 275 (51.5%) patients. The gross total resection (GTR) rate in nonsecretory adenomas with and without CD was 90.7% (97/107) and 90.7% (97/107), respectively. The remission rate in secretory adenomas with and without CD was 81.0% (136/168) and 44.0% (67/152), respectively. When the patients were examined in 2 groups as those who underwent CD and those who did not, the application of CD had a positive predictive value in terms of GTR/remission (P: .036). Capsule thickness was not found to be statistically significant depending on tumor subtype, size, and aggressiveness, but capsule thickness was statistically significant in terms of total capsular resection (P: .045). The morphology of the capsule, tumor size/subtype/consistency, and patient-specific factors are crucial for the selection of the surgical technique to be applied. It is possible to increase the GTR/remission rates in adenomas of all sizes by capsule dissection. Moreover, performing CD does not contribute significantly to the development of potential complications in such cases.

Primary hyperparathyroidism due to parathyroid cancer or atypical adenoma. a third-level spanish hospital experience

Primary hyperparathyroidism due to parathyroid cancer or atypical adenoma. a third-level spanish hospital experience

Temozolomide on Aggressive Pituitary Adenomas: About 4 Cases and Review of the Literature

Pituitary adenomas represent 15 to 20% of all brain tumors . They affect 1/1000 of the world population, some pituitary adenomas with high Ki-67 indexes exhibit aggressive behaviors, such as rapid growth, early and frequent recurrence, and resistance to conventional treatment, even in the absence of metastasis . Many trials have focused on the role of chemotherapy in the treatment of these aggressive and atypical adenomas The objective: Monitoring of patients on temozolomide . we report the follow-up of 4 patients (pts) of 10 pts treated in our department during the period (2012-2019), 2 women / 2 men presenting an aggressive pituitary macro-adenoma, having benefited from excision surgery, radiotherapy and received medical treatment with temozolomide initiated with clinical, biological and radiological evaluation of the therapeutic response and search for p53 and ki 67 in the diagnosis. A clinical improvement was noted in our 4 pts; Radiologically: a partial response was obtained in 1pts, two stabilities and one progression . The average duration of treatment is 26 months (12 - 40) with manageable side effects .Temozolomide appears to be effective in the treatment of aggressive pituitary adenomas and may currently constitute a therapeutic alternative when all resources are exhausted, thus allowing control of the disease.

Immunohistochemical and Clinicopathologic Profiling of Pituitary Adenomas at a Nigerian Tertiary Hospital

Background: Pituitary adenoma is the most common pituitary tumour and the third most common intracranial tumour worldwide. Only a few studies have been done in Africa on the immunohistochemical expression pattern and clinicopathologic presentations of the different hormonal subtypes of pituitary adenomas. Objective: To evaluate the immunohistochemical hormonal profiles and clinicopathologic behaviour of pituitary adenomas diagnosed within ten years at the Lagos University Teaching Hospital, Lagos, Nigeria. Methods: In this 10-year retrospective, cross-sectional study, all cases of pituitary adenomas diagnosed at the hospital mentioned above between 1 January 2009 and 31 December 2018 were analysed. Their paraffin-embedded tissue blocks were stained with haematoxylin and eosin and then with immunostains for growth hormome (GH), adrenocorticotrophic hormone (ACTH), thyroid stimulating hormone (TSH), follicular stimulating hormone (FSH), luteinizing hormone (LH), and prolactin (PRL), using their standard immunohistochemistry protocols. Results: Seventy pituitary adenoma cases were studied. The male-to-female ratio of the patients was 1.6:1. The median age was 45 years, and the peak age of occurrence was the fifth decade for both sexes. Most were null cell adenomas (44.7%) and FSH-beta gonadotrophs (22.9%). The least diagnosed were thyrotroph (1.4%) and corticotroph adenomas (1.4%). Most of the adenomas were macroadenomas, and the most common presenting symptom was visual impairment. Conclusion: Pituitary adenoma is commoner in males in their 5th decade of life at the Lagos University Teaching Hospital, Lagos, Nigeria. The null cell adenoma is the commonest type of pituitary adenoma diagnosed in the center, and the majority of the patient diagnosed with pituitary adenoma present late with complications of visual impairment.

Clinical characteristics and outcomes of patients operated for primary hyperparathyroidism at Tampere University Hospital in 2017-2018.

Studies on the outcomes of parathyroid surgery are scarce. The aim was to report the outcomes and to study the association between pre- and peri-operative information with the outcomes of patients operated for primary hyperparathyroidism. This was a retrospective, descriptive study with unselected patients treated surgically for primary hyperparathyroidism from a catchment population of 704,500 in Finland. Data were acquired from the electronic hospital registers based on parathyroid surgery procedure codes between 1 January 2017 and 31 December 2018. Preoperative data, surgical data, preoperative and postoperative laboratory values, histopathological findings, and postoperative clinical data were recorded. During the 2-year study period, 149 patients with primary hyperparathyroidism were treated surgically with a 97% remission rate. Surgical complications included postoperative bleeding in two patients (1%) and vocal cord paralysis in one patient (0.6%). No postoperative infections were reported. Three patients (2%) developed postoperative hypoparathyroidism necessitating the use of alfacalcidol more than 1 month after surgery. Ionized calcium measured 0-1 days after surgery was not statistically significantly associated with remission or postoperative hypoparathyroidism. Serum parathyroid hormone (PTH) assessed 0-1 days postoperatively was associated with persistent disease, but not with postoperative hypoparathyroidism. The histopathological diagnosis was adenoma or hyperplasia in 112 patients (75%), atypical adenoma in 28 patients (19%), and carcinoma in five patients (3%). Patients with parathyroid carcinoma had higher preoperative ionized calcium and PTH values than those with adenoma or hyperplasia. Most patients who were operated due to primary hyperparathyroidism achieved normocalcemia after surgery, and the frequency of complications was low. Ionized calcium taken 0-1 days after surgery was not associated with remission of hyperparathyroidism or postoperative hypoparathyroidism. High postoperative serum PTH predicted persistent disease.

SpaceANOVA: Spatial Co-occurrence Analysis of Cell Types in Multiplex Imaging Data Using Point Process and Functional ANOVA.

Multiplex imaging platforms have enabled the identification of the spatial organization of different types of cells in complex tissue or the tumor microenvironment. Exploring the potential variations in the spatial co-occurrence or colocalization of different cell types across distinct tissue or disease classes can provide significant pathological insights, paving the way for intervention strategies. However, the existing methods in this context either rely on stringent statistical assumptions or suffer from a lack of generalizability. We present a highly powerful method to study differential spatial co-occurrence of cell types across multiple tissue or disease groups, based on the theories of the Poisson point process and functional analysis of variance. Notably, the method accommodates multiple images per subject and addresses the problem of missing tissue regions, commonly encountered due to data-collection complexities. We demonstrate the superior statistical power and robustness of the method in comparison with existing approaches through realistic simulation studies. Furthermore, we apply the method to three real data sets on different diseases collected using different imaging platforms. In particular, one of these data sets reveals novel insights into the spatial characteristics of various types of colorectal adenoma.

Improvement of metabolic syndrome and its components in patients who underwent transsphenoidal resection for pituitary adenoma.

Pituitary adenomas (PA) are neoplasms of pituitary adenohypophyseal cell lineage, which are the third most common cause of brain tumors among adults. Due to hormone secretion, PAs are closely related to metabolic syndrome (MetS). However, the relationship between these two entities has been scarcely studied to date. This paper aims to evaluate changes in the metabolic status of patients with PA before and after surgical treatment and to look for differences in metabolic outcomes among patients according to the adenoma type and the surgery success rate. We assessed patients with PA who went through transsphenoidal surgery for its treatment, documenting metabolic parameters before and after surgery, analyzed whole sample changes, and then stratified them according to adenoma type (nonfunctioning, somatotroph, lactotroph, and corticotroph), and surgery success (total resection, near-total resection, partial resection, subtotal resection). A total of 214 patients were enrolled for this study. The prevalence of MetS with AACE criteria went from 51.52% before surgery to 28.99% after surgery (P < 0.001). Hyperglycemia (HG) was the most beneficial component; it went from 56% pre-surgery to 40.51% post-surgery (P = 0.03). The total resection group had the best improvement, with a significant decrease of prevalence in MetS from 83 to 16% (P < 0.001), and every component, except hypoalphalipoproteinemia (HA): obesity went from 96 to 67% (P < 0.001), arterial hypertension (AH) 59 to 24% (P < 0.001), HG 74 to 23% (P < 0.001), and hypertriglyceridemia (HTG) from 81 to 54% (P < 0.001). According to MetS prevalence, there was no difference in the improvement according to PA type. Surgical treatment in patients with PA is associated with MetS improvement.

Low Colorectal Cancer Risk After Resection of High-Risk Pedunculated Polyps

IntroductionPost-Faecal Immunochemical Test (FIT) colonoscopy represents a setting with an enriched prevalence of advanced adenomas. Due to an expected higher risk of Colorectal Cancer (CRC), post-polypectomy surveillance is recommended, generating a substantially increased load on endoscopy services. The aim of our study was to investigate post-polypectomy CRC risk in a screening population of FIT+ subjects after resection of low- (LRA) or high-risk adenomas (HRA). MethodsWe retrieved data from a cohort of patients undergoing post-polypectomy surveillance within a FIT-based CRC screening program in Italy between 2002 and 2017 and followed-up to December 2021. Main outcomes were post-polypectomy CRC incidence and mortality risks according to type of adenoma (LRA/HRA) removed at colonoscopy as well as morphology, size, dysplasia and location of the index lesion. We adopted as comparators FIT+/colonoscopy negative and FIT negative patients. The absolute risk was calculated as the number of incident CRCs per 100,000 person-years of follow-up. We used Cox multivariable regression models to identify associations between CRC risks and patient- and polyp-related variables. ResultsOverall, we included 87,248 post-FIT+ colonoscopies (133 endoscopists). Of these, 42,899 (49.2%) were negative, 21,650 (24.8%) had a LRA and 22,709 (26.0%) a HRA. After a median follow-up of 7.25 years, a total of 635 CRCs were observed. For patients with LRAs, CRC incidence (Hazard Ratio [HR] 1.18, 95% Confidence Interval [CI] 0.92-1.53) was not increased compared to the FIT+/colonoscopy negative group, while for HRAs a significant increase in CRC incidence (HR 1.53, 95% CI 1.14-2.04) was found. The presence of one or more risk factors among proximal location, non-pedunculated morphology and high-grade dysplasia explained most of this excess CRC risk in the HRA group (HR 1.85, 95% CI 1.36-2.52). Patients with only distal pedunculated polyps without HGD, representing 39.2% of HRA, did not have increased risk compared to the FIT-negative group (HR 0.87, 95% CI 0.59 - 1.28). ConclusionsCRC incidence is significantly higher in patients with high-risk adenomas diagnosed at colonoscopy. However, such excess risk does not appear to apply to patients with only distal pedunculated polyps without HGD, an observation that could potentially reducethe burden of surveillance in FIT programs.

Predictive factors and model validation of post-colon polyp surgery Helicobacter pylori infection.

Recently, research has linked Helicobacter pylori (H. pylori) stomach infection to colonic inflammation, mediated by toxin production, potentially impacting colorectal cancer occurrence. To investigate the risk factors for post-colon polyp surgery, H. pylori infection, and its correlation with pathologic type. Eighty patients who underwent colon polypectomy in our hospital between January 2019 and January 2023 were retrospectively chosen. They were then randomly split into modeling (n = 56) and model validation (n = 24) sets using R. The modeling cohort was divided into an H. pylori-infected group (n = 37) and an H. pylori-uninfected group (n = 19). Binary logistic regression analysis was used to analyze the factors influencing the occurrence of H. pylori infection after colon polyp surgery. A roadmap prediction model was established and validated. Finally, the correlation between the different pathological types of colon polyps and the occurrence of H. pylori infection was analyzed after colon polyp surgery. Univariate results showed that age, body mass index (BMI), literacy, alcohol consumption, polyp pathology type, high-risk adenomas, and heavy diet were all influential factors in the development of H. pylori infection after intestinal polypectomy. Binary multifactorial logistic regression analysis showed that age, BMI, and type of polyp pathology were independent predictors of the occurrence of H. pylori infection after intestinal polypectomy. The area under the receiver operating characteristic curve was 0.969 [95% confidence interval (95%CI): 0.928-1.000] and 0.898 (95%CI: 0.773-1.000) in the modeling and validation sets, respectively. The slope of the calibration curve of the graph was close to 1, and the goodness-of-fit test was P > 0.05 in the two sets. The decision analysis curve showed a high rate of return in both sets. The results of the correlation analysis between different pathological types and the occurrence of H. pylori infection after colon polyp surgery showed that hyperplastic polyps, inflammatory polyps, and the occurrence of H. pylori infection were not significantly correlated. In contrast, adenomatous polyps showed a significant positive correlation with the occurrence of H. pylori infection. Age, BMI, and polyps of the adenomatous type were independent predictors of H. pylori infection after intestinal polypectomy. Moreover, the further constructed column-line graph prediction model of H. pylori infection after intestinal polypectomy showed good predictive ability.

O-01 Case report: Temozolomide treatment of refractory prolactinoma

Abstract Introduction Treatment of refractory prolactinoma is challenging. Temozolomide (TMZ), an oral chemotherapeutic agent, has been recommended as an effective drug for refractory pituitary adenomas or carcinomas. Clinical Case We report a 53-year-old male who presented with diplopia, left eye proptosis, conjunctival congestion, and headache. Magnetic resonance imaging (MRI) showed a macroadenoma 20×17 mm in diameter with compression of the optic chiasm and invasion of both cavernous sinuses. Hormonal evaluation revealed hyperprolactinemia of 2382 ug/L, 120x above the upper limit of normal (ULN), with central hypothyroidism and hypogonadism. Cabergoline was recommended as an initial treatment. Despite the maximum cabergoline dose of 1.5 g daily, tumor volume increased with visual disturbances and headaches. Prolactin (PRL) remained 60x above ULN so the patient underwent transsphenoidal surgery. Tumor histology was consistent with an atypical pituitary adenoma, positively stained for prolactin and sporadically for growth hormone, Ki-67 of 6.4%, no mitosis and negative p53. Postoperatively, the PRL level was still high (25x above ULN) with tumor remnants in both cavernous sinuses. Due to the tumor resistance to dopamine agonist treatment and uncomplete surgical resection, patient underwent stereotactic gamma knife radiosurgery (GK prescription dose 22 Gy, isodose 49%) simultaneously with TMZ treatment. After 2 cycles of TMZ (1. cycle TMZ 300mg/5days, 2. cycle TMZ 400 mg/5days), the PRL level decreased significantly (126 ug/L). The plan is to apply a total of 6 cycles of temozolomide. Conclusion Clinical evidence for the efficacy of TMZ in refractory prolactinoma remains limited, but early use of this agent may be helpful in some cases.

O-05 A novel mutation of MEN1 gene in familial Multiple Endocrine Neoplasia Type 1 (MEN1): A report of two cases

Abstract Introduction Multiple Endocrine Neoplasia Type 1 (MEN1) is a rare (1-9/100000) autosomal dominant disease. It is characterized by primary hyperparathyroidism (90%), enteropancreatic tumor (60-70%), non-functional adrenal adenomas (30%) and pituitary tumors (10-20%). Herein, we present two cases in the same family with MEN1 syndrome caused by a novel mutation. Clinical Case 1: 49-year-old male presented to our clinic with fasting hypoglycemia. Fasting serum glucose (FSG) was 51 mg/dl and serum cortisol was 18 ug/dl. During the hypoglycemia in prolonged fasting test, the serum levels of glucose, insulin and C-peptide were 37 mg/dl, 17.3 mu/l, 7.75 ng/ml respectively (Table 1). Abdominal MRI revealed mass lesions in the pancreas (Figure 1). Ultrasonography of the neck and MIBI scintigraphy performed because of the high levels of calcium and parathyroid hormone (PTH), were consistent with parathyroid adenoma (Table 2). Pituitary MRI showed a 12 mm macroadenoma. He underwent parathyroidectomy and distal pancreatectomy simultaneously. The resected specimens revealed a well-differentiated neuroendocrine tumor (NET) of the pancreas and parathyroid adenoma. The patient had no new hypoglycemic episodes after surgery and serum calcium remained within the normal range (Table 2). Central hypothyroidism and hypogonadotropic hypogonadism which was resulted from the pressure of the adenoma was managed medically. Genetic testing revealed a heterozygous mutation in NM_001370251.1:c.949del(HET). First degree relatives of the patient were screened for MEN. Clinical Case 2: The son of case 1, an 18-year-old male, presented with fasting hypoglycemia. He had a BMI of 37 kg/m2 and underwent gastric botox 3 months prior to his presentation. FPG was 52 mg/dl and serum cortisol was 20 ug/dl. Prolonged fasting test confirmed the diagnosis of insulinoma (Table 1). Abdominal MRI showed a pancreatic tail mass 37×22 mm in size. Gallium 68 PET/CT DOTATATE revealed increased uptake in multiple lesions in the pancreas. Ultrasonography and MIBI scintigraphy showed a parathyroid adenoma. A nonfunctional microadenoma was detected in pituitary MRI. The patient underwent distal pancreatectomy. Following pancreatectomy, right superior and left inferior parathyroidectomies were performed. Since his serum calcium levels started to increase in 48 hours after the operation, the patient has had second operation for left superior parathyroidectomy. After the operation, the serum calcium and parathyroid hormone levels were 9.2 mg/dl, 58 pg/ml respectively. Specimens revealed well-differentiated NET and atypical parathyroid adenoma. The patient was found to have the same genetic mutation with his father and during the follow up the serum glucose and calcium levels remained within the normal range (Table 2). Conclusion In the genetic examination the heterozygous mutation NM_001370251.1:c.949del(HET) in exon 7/11 of the MEN1 gene found in our patients represents a novel mutation.Figure 1.MRI showing pancreatic masses Table 1.Laboratory findings Table 2.Prolonged fasting test

Fine needle aspiration biopsy of parathyroid; is it meaningful? A cytologic study of 81 cases with histological and clinical correlations.

Recognizing the parathyroid gland and distinguishing the parathyroid from thyroid lesions in fine needle aspiration (FNA) is challenging. This study aimed to identify cytomorphologic features suggestive of parathyroid origin and to assess the utility of cytopathology in conjunction with ancillary tests in the identification of parathyroid glands. Ultrasound (US) guided FNA of parathyroid gland and lesions in 81 patients were reviewed concerning clinical history and correlated to histopathologic findings in available cases. FNA smears were evaluated for cellularity, architectural patterns, cellular and nuclear features, and background of the smears. In 78 cases, FNA was supplemented by a measurement of parathormone (PTH) levels in the needle washout fluid (FNA-PTH assay) and/or GATA3/PTH/chromogranin-A immunostainings. Sixty-four cases were diagnosed cytologically as parathyroid lesions in conjunction with FNA-PTH assay and/or immunocytochemical examinations. In an additional nine cases, a diagnosis of parathyroid lesions was rendered after repeated FNA with FNA-PTH assay. The histolopathologic diagnosis of surgically excised cases (n = 75) included parathyroid adenoma (60 cases), atypical parathyroid adenoma (4 cases), parathyroid hyperplasia (10 cases), and parathyroid carcinoma (1 case). Major cytological findings of parathyroid tissue included high cellularity, scattered naked nuclei, cribriform and three-dimensional clusters, stippled chromatin, and oxyphilic cytoplasm while papillary pattern or colloid-like material was identified in three cases respectively. No nuclear grooves or inclusions were seen in any case. High cellularity scattered naked nuclei, cribriform and three-dimensional patterns, stippled chromatin and oxyphilic cytoplasm are cytomorphologic features that favour parathyroid origin. A combination of these features with FNA-PTH assay and/or GATA3, PTH, and chromogranin-A immunostainings on cytologic specimens aid in the identification of parathyroid glands and the distinguishing of parathyroid from thyroid lesions.

Clinically functioning gonadotropin-secreting pituitary adenoma.

Gonadotroph adenomas are the most common type of nonfunctional pituitary adenomas. However, functioning gonadotroph adenomas causing clinical manifestations are rare. We present the case of a 42-year-old man with an incidental finding of a pituitary gland mass. A pituitary MRI revealed a 3 cm macroadenoma, and laboratory investigations revealed elevated follicle-stimulating hormone (FSH) and total testosterone levels. A diagnosis of functioning FSH-secreting pituitary adenoma was considered, with possible concomitant luteinizing hormone secretion, given the elevated testosterone, prompting further evaluation. Testicular ultrasound showed bilaterally enlarged testicles, and visual field testing revealed a monocular superior temporal defect. Transsphenoidal resection of pituitary adenoma was the treatment of choice. Histopathology assessment confirmed the diagnosis of gonadotroph-secreting adenoma, with positive staining for FSH. Within the 12-week postoperative period, FSH and testosterone levels normalized, and the patient experienced significant improvement in vision, along with the resolution of macroorchidism. While functional gonadotroph adenomas are rare, patients can present with a wide range of symptoms that are often unnoticeable due to their slow development. Careful evaluation can help guide multidisciplinary management to achieve full remission. Endocrine evaluation is indicated in all cases of pituitary incidentalomas to determine functional status. Clinically functioning gonadotroph adenomas, while rare, pose a diagnostic challenge and require careful clinical evaluation. Transsphenoidal surgery is the mainstay of treatment of functioning gonadotroph adenomas, with the involvement of a multidisciplinary team to achieve desirable outcomes.

Oxyntic Gland Neoplasms - From Adenoma to Advanced Gastric Cancer: A Review of 29 Cases.

Oxyntic gland neoplasm (OGN) is a rare condition that can be classified as oxyntic gland adenoma (OGA) or gastric adenocarcinoma of fundic-gland type (GA-FG). GA-FG primarily presents as early gastric cancer, with only a few reported cases of advanced gastric cancer (AGC). We aimed to investigate the clinicopathological features of OGN and describe an aggressive variant. We investigated a total of 29 cases, including a patient with double primary cases, diagnosed with OGN or differentiated-type adenocarcinoma with GA-FG morphology, between November 2016 and April 2022. We analyzed 54 pathological specimens and reviewed their clinicopathological, endoscopic, and histological features. The lesions were reclassified as OGA or GA-FG, and immunohistochemical (IHC) staining for MUC-5AC and MUC-6 was performed on available resected GA-FG cases. The median patient age was 65 years and males accounted for 58.6% of patients. Most cases occurred in the body horizontally (69.0%) and on the greater curvature side cross-sectionally (48.3%). Endoscopically, type 0-IIa (41.4%) and a subepithelial tumor-like appearance (24.1%) were the most common findings. Histologically, there were 8 cases of OGA (27.6%) and 21 cases of GA-FG (72.4%). In GA-FG, MUC-6 was positive in 13 cases (81.3%), whereas MUC-5AC was positive in 8 cases (50.0%). Three cases presented as AGCs. Although OGNs are generally considered low-grade, they can present as AGCs and may exhibit features of lymphovascular or perineural invasion. Recognizing the clinicopathological features and accurately diagnosing OGN are important for providing adequate treatment.

THE PLACE OF SURGERY IN THE MANAGEMENT OF PROLACTIN SECRETING ADENOMAS.

Surgery has lost a lot of ground as the main therapy of most prolactinomas as it is clear from the current guidelines in most prolactin secreting adenomas, even in the setting of optic compression. However, we believe that surgery is still an important part in the treatment of this type of adenomas. This study is aimed to define what is the role of pituitary surgery in the current setup of prolactinoma management. In this retrospective, single-center study we analyzed 12 consecutive patients who underwent primary endoscopic transsphenoidal surgery for prolactinomas, between 2013 and 2022. Surgical indication, previous dopamine agonist (DA) treatment, remission rates, surgical complications, pituitary function and imagistic appearance are presented. Of the 12 patients included, 4 had giant PRL and 8 macroadenomas, while 9 of them had previous DA treatment. The main surgical indication was pituitary apoplexy in 5 patients followed by CSF leak after DA treatment, 3 cases, and DA resistance in 3 cases. The main surgical complications were transitory diabetes insipidus in 7 cases. Normalization of prolactin levels was achieved in 2 patients. Surgical intervention should be strongly considered in all patients with neurologic symptoms referable to the lesion, resistance to medical therapy, other treatment failure or with complications after DA treatment. The endoscopic endonasal surgery offers good surgical outcomes with low rates of surgical complications and should remain an open option for specific cases.

Survival determinants of patients with uncertain behavior pituitary tumors: A Surveillance, Epidemiology, and End Results (SEER) database study

IntroductionThe “borderline” pituitary tumor category between typical benign adenomas and pituitary carcinomas is a subject of ongoing controversy. Surveillance, Epidemiology, and End Results (SEER) database classifies uncertain behavior pituitary tumors (UPTs) under the “borderline” category including atypical adenomas with presumed propensity for aggressive behavior. Very little is known about UPT patient survival prognostic factors. We aimed to characterize survival risk factors of patients with UPT according to the SEER database. MethodsSEER 17 database was queried for pituitary tumors and further separated by SEER classification of “Borderline” under the category “ICD-O-3 Hist/behav, malignant”. Patient demographics, tumor characteristic, extent of disease and treatment data were extracted and analyzed. A nomogram was built assessing survival. ResultsThree hundred and thirty-two patients diagnosed between 2004 and 2019 were included. The survival rates at 1, 5 and 10 years were 86.8 %, 78.6 % and 65.1 %, respectively. Female sex and age <65 years correlated with a better prognosis (HR 2.41, 95 % CI [1.57–3.68], p = 4.00E-05 and HR 5.47; 95 % CI [3.53–8.45], p = 3.00E-15). Patients undergoing surgery had a better prognosis than those pursuing non-surgical management (HR 0.43, 95 % CI [0.19–0.97], p = 0.02). A nomogram incorporating sex, age and surgical versus non-surgical treatment status was created. The nomogram demonstrated acceptable accuracy in estimating the survival probability at 1- and 2-years, with a C-index of 0.751 (95 % CI: 0.641–0.861). ConclusionFemale sex, age <65 years and surgical resection are significant factors determining UPT patient survival based on SEER data. The proposed nomogram achieved good potential for predicting UPT patient survival.

Primary hyperparathyroidism in children

Primary hyperparathyroidism (PHPT) is an endocrine disorder characterized by excessive secretion of parathyroid hormone (PTH) with upper-normal or elevated blood calcium levels due to primary thyroid gland pathology. PHPT is a rare pathology in children, with a prevalence of 2-5:100,000 children according to the literature. Due to the non-specificity of clinical manifestations at onset (nausea, vomiting, abdominal pain, emotional lability), the disease may remain undiagnosed for a long time. To study the features of the course and molecular genetic basis of primary hyperparathyroidism in children. Retrospective observational study of 49 patients diagnosed with primary hyperparathyroidism. All patients underwent a comprehensive laboratory-instrumental and molecular genetic study at the Institute of Pediatric Endocrinology, Endocrinology Research Center of Russia in the period 2014-2022. The first clinical symptoms of PHPT were noted at the age of 13.8 years [10.6; 1 5.2], among which fatigue, headaches, dyspepsia, lower limb pain, and fractures were the most common. The age of diagnosis was 15.81 years [13.1; 16.8], all children were found to have high levels of PTH, total and ionized calcium, with hypophosphatemia in 93.9% of patients (n=46) and hypercalciuria in 43% (n=21). Five out of 49 patients (10.2%) were found to have ectopy of the thyroid: 3 showed an intrathyroidal location, 2 in the mediastinal region. Molecular genetic study revealed mutations in 32.7% of patients (n=16, CI (21; 47)), mutations inMEN1being the most frequent (n=11). Pathogenic variants inCDC73were detected in 3patients,RET -in 2. Among the operated 39 patients, adenoma of the thyroid was detected in 84.6% of cases (n=33), hyperplasia in 7.7% (n=3), atypical adenoma in 5.1% (n=2), carcinoma in 5.1% of cases (n=2). The paper presents the peculiarities of the course and the results of molecular genetic study of pediatric PHPT. This sample is the largest among those published in the Russian Federation.

H - 67 Socially Responsible Neuropsychology (SRN) in Action: Considering Culture, Brain Structure, and Function Longitudinally in an Atypical Pituitary Adenoma Case.

Research on the neuropsychological profile associated with pituitary adenoma (PA) in diverse populations is limited. Clinically, PAs are categorized by size (micro-vs-macro) and hormone secretion levels (functional/secreting-vs-nonfunctional/non-secreting [NFPA]). Macro-NFPA are associated with visual-spatial, memory, and executive functioning declines, likely due to mass effects. No previous reports of cognitive declines in micro-NFPA were found in the literature. We present a clinical case illustrating the application of an SRN model in a 75-year-old, Spanish-English bilingual male, with cognitive declines, diagnosed with micro-NFPA. An SRN model was applied to promote equitable care through evidence-based consideration of neuropsychological syndromes in bilinguals, neurodiagnostics, and complex psychosocial factors. The patient underwent longitudinal language-congruent neuropsychological assessment and structural/functional neuroimaging. Educational background, linguistic proficiencies, acculturation level, social/behavioral comportment, and limitations in available neuropsychological tools/norms were integrated to reflect the complexity in conducting a reliable bilingual assessment and formulating differential diagnoses. The SRN model guided complex clinical decision-making. Neuropsychological profile (frontal-subcortical-vs-cortical-involvement) varied across language and time-point. Within/between variable performances in memory, executive functioning, processing speed, and verbal fluency were inconsistent with structural neuroimaging at assessment points, increasing diagnostic ambiguity. Advocated-for functional neuroimaging revealed pituitary hypometabolism. To our knowledge, this is the first reported case of neurocognitive impairment in micro-NFPA, and the first reported neuropsychological profile of a bilingual individual with PA. The findings suggest the need for further research on neurocognitive declines in NFPA in diverse populations. It also highlights the importance of assessing in all relevant languages when conducting longitudinal assessments to boost sensitivity and specificity in differential diagnoses.

SAT201 A Rare Case Of A Mediastinal Atypical Parathyroid Adenoma Resulting In Severe Hypercalcemia And Brown Tumor

Abstract Disclosure: O.A. Aluko: None. D. Franey: None. R. Quiros: None. W. Burfeind: None. R.E. Amori: None. Introduction: Primary hyperparathyroidism is most often from parathyroid adenomas located usually in close association to the thyroid gland. Ectopic parathyroid glands result in significant variability in the position of the gland, however rarely in the mediastinum. We report a case of a patient with severe hypercalcemia from a large, ectopic mediastinal parathyroid adenoma. Case Report: A 53-year old male with a past medical history of hypercalcemia and nephrolithiasis was sent to the ER for evaluation of hypercalcemia after he was found to have a corrected calcium of 12.5 mg/dl (n8.4 - 10.2 mg/dl) on routine labs. Repeat blood work at presentation confirmed hypercalcemia with a corrected calcium 11.9 mg/dl and elevated ALP of 211 U/l (n 43 - 122 U/L), elevated initial PTH of799 pg/ml (n 18.4 - 80.1 pg/ml), Vit D 32.7 ng/ml (n 30 - 100 ng/ml), and low phosphorus 2.4 mg/dl (n2.7 - 4.5 mg/dl). Calcium slightly improved to 11.7 mg/dl after IV hydration. Chest CT showed a large mediastinal mass compressing trachea and esophagus, suspicious for parathyroid origin, with a smaller left sided lesion suspicious for parathyroid adenoma. CT also showed multiple bone lesions likely brown tumors. He underwent complete robotic resection of the mediastinal mass. Immediately prior to surgery, PTH was 1002.8 pg/ml, and fell to 131.1 pg/ml and 44.1 pg/ml following removal of the mediastinal mass. Adherence of the mass to surrounding structures made resection challenging, resulting in a right vocal cord paralysis. After surgery, he was empirically started on IV calcium and calcitriol for high clinical concern for hungry bone syndrome, both later discontinued as serum calcium remained within normal limits. Pathologic findings reported as highly suspicious, however not definitive for parathyroid carcinoma with no evidence of perineural or lymphovascular invasion. Given rarity, he was referred to a tertiary cancer center where he was diagnosed with Atypical parathyroid Adenoma (APA). He underwent a right pubic biopsy with pathology diagnosis of brown tumor of hyperparathyroidism. He started on cinacalcet 30 mg daily after surgery. There is a future plan for resection of the smaller left sided parathyroid adenoma. Conclusion: This case highlights two rare entities; a mediastinal ectopic parathyroid and atypical parathyroid adenoma. Atypical Parathyroid Adenoma is a not-well defined entity which could mimic a parathyroid carcinoma as seen in our case. The location of the mass in the superior mediastinum is unusual, making it more difficult to diagnose, and could increase risk of complications during resection. Delayed diagnosis of these entities can lead to severe hypercalcemia and skeletal complications infrequently seen in common primary hyperparathyroidism cases. Presentation: Saturday, June 17, 2023