Suprasellar Extension Research Articles

6400 An Unusual Presentation of Silent Corticotroph Pituitary Macroadenoma

Abstract Disclosure: N. Mon: None. V. Lohano: None. B. Williams: None. N. Lehman: None. R. Kulkarni: None. S.L. Mokshagundam: None. Silent Corticotroph Pituitary Adenomas (SCA) are a rare group of nonfunctioning pituitary adenomas (NFA) comprising 3-6% of all pituitary adenomas and up to 20% of corticotroph adenomas. SCA are biologically and clinically distinct from Cushing disease and NFA. SCA present with headache, visual field impairment, hypopituitarism, and pituitary apoplexy. We present a case of SCA with the initial presentation of syncope with undetectable ACTH and very low AM cortisol level.A 67 year-old-female was incidentally found to have a pituitary macroadenoma in MRI brain for syncope work up in 2017. MRI reported 1.4 x 1.2 x 1.3 cm sellar mass with suprasellar extension, minimal mass effect on the optic chiasm and no cavernous sinus invasion. Initial labs showed ACTH <5 (7.2-63.3 pg/mL), AM cortisol 0.8 (6-28ug/dl), Prolactin 101.1 (3.3-27 ng/mL), TSH 0.08 (0.34-5.60 uIU/mL), Free T4 0.6 (0.58-1.64 ng/dL), FSH 1.7 (2-5 mIU/mL), LH <0.2 (1-13 mIU/mL), IGF-1 33 ( 41-279 ng/mL). ACTH stimulation test: cortisol 0.5 ug/dl at baseline, 5.8 ug/dl at 60min. She was diagnosed with panhypopituitarism and hyperprolactinemia due to stalk effect. Hydrocortisone and Levothyroxine (LT4) were started. She underwent pituitary macroadenoma resection for optic chiasm decompression in 2017. Pathology reported pituitary neuroendocrine tumor (pitNET), but no immunostaining was done at that time. Follow up MRI in 2019 showed residual pituitary tumor. In 2023, she endorsed headaches, mood changes, fatigue and weight gain. Physical exam was unremarkable except BMI 34.75. Prolactin, Free T4, IGF-1 were normal. MRI in 2023 revealed recurrent and enlarging pituitary adenoma (1.9 x 1.4 x 1.6 cm) with suprasellar extension, abutting the optic chiasm, and left cavernous sinus invasion with internal carotid artery encasement. She underwent revision transsphenoidal resection of pituitary tumor to decompress the optic chiasm with plan for subsequent focused radiation to the cavernous sinus portion. Postoperative period was uneventful and hydrocortisone and LT4 were continued on discharge. Final pathology reported silent corticotroph lineage pitNET with low Ki67, immunostaining positive for TPIT, CAM5.2, and negative for ACTH. ACTH-negative SCA are a rare subtype and more likely to have lower ACTH levels, and multiple microcysts on MRI than ACTH-positive SCA. New WHO classification of pitNET highlights the histological typing and subtyping based on immunostaining of pituitary lineage transcription factors as the cornerstone for accurate classification. SCA are more biologically aggressive tumors with a high propensity of recurrence and resistance to conventional treatment. TPIT and ACTH immunostaining significantly improved the diagnosis of SCA and thereby close clinical follow up for early recognition and management of SCA recurrence. Absence of serum markers of recurrence makes this challenging. Presentation: 6/1/2024

8014 Cushing’s Syndrome: Two Sides of The Spectrum

Abstract Disclosure: M. Aguilera: None. A. Vu: None. J. Shakil: None. R. Al-Ward: None. Introduction: Cushing’s syndrome (CS) is a rare condition with an annual incidence of 2-8 per million. Endogenous CS is mainly due to corticotroph adenoma of the pituitary followed by excessive glucocorticoid production from adrenal and ectopic sources. CS has significant morbidity and mortality hence the need for timely diagnosis. The varied presentations often pose great challenges in identifying the source of hypercortisolism. We present two unusual cases of CS with distinct clinical presentations and atypical laboratory profiles. Case 1: 25-year-old male with no medical history presented with 9 months of facial puffiness, weight gain, fluid retention, hypertension and blurred vision. Physical examination revealed round facies, bilateral supraclavicular fat pads and wide abdominal striae. Laboratory workup showed markedly elevated ACTH 537 pg/mL [7.2-63 pg/mL], serum cortisol 8.5 ug/dL [6.2-19.4 ug/dl], 24-hour urine free cortisol (UFC) 30 [5-62 ug/24H] and normal late-night salivary cortisol (LNSC). Repeat workup a month later showed serum cortisol of 56ug/dL, ACTH 874 pg/dL, 24-hour UFC of 11880ug/24H and elevated LNSC. Pituitary MRI showed a large heterogenous 3.1 cm partially cystic sellar mass with suprasellar extension. Due to significantly elevated ACTH and short duration of symptoms, we suspected an ectopic source. Due to initial negative testing, except for high ACTH level, a silent corticotroph adenoma was also considered. The patient underwent transsphenoidal resection resulting in significant decrease of cortisol and ACTH postoperatively. Pathology showed a corticotroph adenoma with Ki-67 index of <1%. Case 2: 47-year-old woman with medical history of obesity, polycystic ovary syndrome and a stable 1 cm lung nodule since 2020, presented with fluid retention, worsening acne and resistant hypertension over a 1-year period. She required hospitalization for hypertensive emergency and hypokalemia. Laboratory data showed morning serum cortisol 28 ug/dL, ACTH of 90.1 pg/mL, 24-hour UFC 346 ug/24H, elevated LNSC on two occasions and cortisol 24.3 ug/dl after a 1mg dexamethasone suppression. MRI did not show a pituitary mass. PET dotatate scan revealed a tracer avid 1 cm right upper lobe pulmonary nodule. She underwent a right upper lobe lobectomy, resulting in a significant decrease in cortisol and ACTH levels on postoperative day 1. Pathology confirmed a typical carcinoid tumor. Discussion: High ACTH levels and a short symptom duration often point to ectopic production, paraneoplastic syndromes or corticotroph carcinomas. Cushing's disease typically exhibits mildly elevated ACTH levels, but our cases deviate from this norm, complicating source identification. This atypical presentation highlights the complexity of Cushing's syndrome, challenging conventional diagnostic paradigms. Presentation: 6/1/2024

12391 When Pressure Drops, Consequences Rise: A Case Of Pituitary Apoplexy And Watershed Infarcts

Abstract Disclosure: D. Moreno Watashi: None. H.R. Alkaissi: None. M.B. Sharma: None. Introduction: Pituitary apoplexy (PA) results from sudden hemorrhaging and infarction of the pituitary gland, typically within a pituitary adenoma. The primary symptom is a sudden, severe headache, often followed by acute endocrine dysfunction, ophthalmoplegia and visual or neurological disturbances. Case Description: A 68-year-old male with a past medical history of hypertension presented to the emergency department with acute chest and upper back pain, beginning one day prior. No history of smoking, alcohol intake or drug use. Blood pressure (BP) on arrival was 180/95 mmHg. Initial labs revealed mild electrolyte abnormalities. Troponins were normal, but ECG showed diffuse T wave inversion and ST depression. CT angiography revealed a type B aortic dissection. He was managed in the critical care unit with continuous esmolol intravenous (IV) infusion and planned for endovascular repair. During his stay, he became disoriented and hypotensive. MRI from the brain incidentally discovered a pituitary macroadenoma measuring 2.9x2.2x3cm and recent infarcts, with a distribution concerning for hypoperfusion. Fundoscopy concerning for optic chiasm compression. Labs indicated secondary hypothyroidism and hypogonadism (TSH 0.3 uUI/ml, free T4 0.88 ng/dL, total testosterone 56.3 ng/dL). Neurosurgical intervention was deferred until after aortic repair. Endovascular aortic repair was performed with episodes of hypotension. Post-procedure, the patient developed fever (39.5°C) and worsening mental status. Head CT revealed an increased suprasellar extension of the pituitary macroadenoma and vasogenic edema, consistent with hemorrhage of the lesion. He was started on stress doses of hydrocortisone, levothyroxine 125mcg and managed conservatively due to a high surgical risk. Discussion: PA's extrinsic risk factors include angiographic procedures, endocrinological dynamic tests, anticoagulation therapy, exogenous estrogen administration, orthopedic and cardiac surgeries (due to BP fluctuations). The intrinsic risk factors are non-functioning adenomas, macroadenomas and suprasellar extension. Treatment is focused on immediate correction of endocrine derangements and relieving local compression. Stress dosage of corticosteroids should be administered IV as soon as the diagnosis is confirmed to prevent life-threatening corticotropic deficiency. Neurosurgical decompression via a transsphenoidal approach is the definitive therapy to relieve local compression with severe visual loss, obtundation or rapidly progressive course. The potential outcomes are based on the extent of hemorrhage, necrosis, and edema. PA should be treated as a medical emergency in the setting of hemodynamic changes from acute corticotropic deficiency and irreversible neurological damage from acute intracranial compression. The risk factors for PA should be known to assist in early diagnosis. Presentation: 6/1/2024

8264 An Interesting Case of Hypophysitis in a Young Woman

Abstract Disclosure: A. Garcia: None. R. Sharma: None. C.M. Knight: None. Background Hypophysitis is an uncommon disorder and presents with clinical signs and symptoms along with biochemical abnormalities. Herein, we present a case of hypophysitis that responded well to high dose steroids. Case An 18 y.o black, adopted woman presented to hospital with complaints of headache and vision disturbances. Endocrinology was consulted for pituitary macroadenoma. Symptoms started a month prior. She had extensive work-up at an outside hospital including brain imaging, CSF analysis, and bloodwork. No definitive diagnosis was made and she was sent home on topiramate. Her symptoms continued to worsen prompting her to seek another opinion. No family history as she is adopted. She has a history of depression controlled with medication. MRI Brain showed 1.8x1.1x1.6 cm T2-enhancing mass with suprasellar extension deforming the midline optic chiasm. Initially, a diagnosis of macroadenoma was made and Endocrinology and Neurosurgery services consulted. Patient denied galactorrhea, menstrual abnormality, polyuria, polydipsia, cold intolerance, orthostasis, or weight gain. Bloodwork was significant for low TSH (0.045 uIU/ml; normal 0.35-4.94), low free T4 (0.58 ng/dl; normal 0.76-1.79). Thyroid functions were normal approximately one year prior. Cortisol was very low at 1.8 mcg/dL and ACTH inappropriately low-normal at 12 pg/ml (normal 6-50). LH and estradiol were low, while FSH was low normal. Prolactin, GH, IGF-1, sodium, glucose and creatinine were normal. Endocrine reviewed the previous MRI brain done four weeks ago that was reported as normal. Discussion with previous outside hospital radiologist suggested that pituitary was indeed not normal but was fuller with convexity towards chiasm although no definitive adenoma was found. Since it is unlikely that such adenoma would develop over 4 weeks, Endocrinology reached out to our radiologist who assessed this as pituitary enlargement due to likely hypophysitis, especially when compared to imaging 4 weeks ago. Further work up showed mildly-elevated ESR, and normal ACE, dsDNA, ANA, and Sjogren’s antibody levels. Tests for pregnancy, Lyme disease, tuberculosis, EBV and syphilis were negative. SARS-CoV-2 was negative for COVID. IgG4 levels were normal. CSF analysis was normal. She was started on levothyroxine and high-dose corticosteroids with improvement in her symptoms. Repeat imaging after 5 days of corticosteroids showed about 30% decrease in pituitary size. A diagnosis of likely lymphocytic hypophysitis was made and pituitary surgery averted for time being. Conclusion We present a case of possible lymphocytic hypophysitis in a young woman who avoided pituitary surgery due to diligent history and examination, in addition to a multidisciplinary team approach. It is very important to have prompt multidisciplinary approach if there is any doubt about a diagnosis as in this case. Presentation: 6/3/2024

8203 A Solitary Fibrous Tumor Mimicking a Non-functional Pituitary Macroadenoma

Abstract Disclosure: L. Bernacet Rivera: None. S. Omay: None. A. Huttner: None. S.E. Inzucchi: None. Background: A solitary fibrous tumor (SFT), also known as hemangiopericytoma (HPC), is a rare subset of soft tissue tumors of mesenchymal origin. This type of tumor tends to have aggressive behavior and a high risk of recurrence. Approximately less than 1% of central nervous system (CNS) tumors have been identified as part of this subtype. Case Presentation: A 73-year-old female with medical history of primary hypothyroidism, myelodysplastic syndrome (MDS), and IgM kappa MGUS initially presented to her primary care physician for evaluation of progressive visual loss over several weeks, accompanied by worsening headaches. Ophthalmologic exam revealed bitemporal hemianopsia which prompted urgent imaging. Pituitary MRI with IV contrast revealed a solid sellar mass with suprasellar extension measuring 1.8 x 1.3 x 1.9 cm. The lesion was noted to superiorly displace the optic chiasm as well as to cause mild impingement of the anterior cerebral arteries. Patient was urgently referred to neurosurgery and endocrinology for further evaluation. Initial neuroendocrine laboratory assessment, performed at 8 am, revealed a cortisol 16.5 ug/dL, free T4 1.2 ng/dL, TSH 0.77 mIU/L, prolactin 9.9 ng/mL, IGF-1 17 ng/mL, the latter value consistent with adult growth hormone deficiency. Her LH and FSH were 4.3 mIU/mL and 12.6 mIU/mL, respectively, lower than expected for a post-menopausal woman. She underwent a transnasal transsphenoidal resection of the sellar lesion. Surgery was difficult due to the fibrous nature of the tumor, for which complete resection was not achieved. Post-operative course was complicated by thrombocytopenia due to MDS. Hormonal workup following surgery was consistent with central adrenal insufficiency, hypogonadotropic hypogonadism, and central hypothyroidism. Pathology showed densely cellular spindle cell neoplasm with a collagenous background consistent with hemangiopericytoma. She underwent fractionated radiotherapy and tumor has since remained stable in size. Her visual symptoms resolved quickly following surgery. Discussion: SFT is an aggressive mesenchymal tumor which rarely appears in the sellar region. There are no identified brain MRI findings that would suggest the nature of this tumor. As a result, endocrinologists should be aware of this rare condition while evaluating pituitary neoplasms. Presentation: 6/3/2024

8110 Lymphocytic Hypophysitis secondary to a ruptured Rathke’s Cleft Cyst complicated by post surgical recurrence: A rare phenomenon

Abstract Disclosure: M.S. Maharaul: None. F. Thelmo: None. I. Ahmed: None. Introduction: Lymphocytic hypophysitis secondary to ruptured Rathke’s cleft cyst (RCC) is a rare entity. Rathke’s cleft cysts are benign fluid-filled intra-sellar cysts that originate from the remnants of Rathke’s pouch and contain mucoid material. Most are asymptomatic; some may get symptomatic during cyst expansion and rupture releasing liquid content and trigger local inflammation. Surgical resection followed by glucocorticoids is usually curative. Case: A 42-year-old female presented to the hospital with one week history of right sided frontal headache associated with blurred vision in right eye. MRI Brain showed a 15 mm complex cystic lesion in the sella with suprasellar extension associated with peripheral calcifications. On admission, endocrine laboratory investigations including pregnancy test were normal. Ophthalmic testing did not reveal any acute abnormalities. Transsphenoidal surgery was performed for tumor resection. Pathology examination revealed infiltration of massive lymphocytes, macrophages, plasma cells, and eosinophils that were diagnostic of lymphocytic hypophysitis in the anterior pituitary lobe. She was started on glucocorticoids and discharged home. Rheumatology and Infectious diseases workup were unrevealing. On follow up 3 weeks later, she had self-discontinued steroids. The following week, she presented to the emergency room again with a right sided headache and blurred vision. MRI Brain revealed a 16 mm recurrent inflammatory mass lesion in the sella. Endocrine panel demonstrated an 8 am serum cortisol level of 7.4 mcg/dl, ACTH 20 pg/ml, FSH 6.2 mIU/ml, LH 4.9 mIU/ml, free T4 1.1 ng/dl and prolactin 8 ng/ml. She underwent transsphenoidal resection of the mass again and was restarted on glucocorticoids. This time pathology examination revealed an epithelial lined cyst most compatible with Rathke’s cleft cyst. She continued to remain on a glucocorticoid taper during the follow up period. On re-evaluation, her endocrinology laboratory investigations continued to show a deficiency of gonadotropins for which she is following up with OBGYN, but the remaining pituitary hormones were all within normal limits. Conclusion: Rathke’s Cleft Cysts (RCCs) should be in the differential diagnoses for evaluation of hypophysitis. There have only been a handful of cases in the literature of hypophysitis associated with a ruptured RCC, and only one case of recurrence after surgical debulking. This case provides an insight into the clinical behavior, diagnosis, and timely management of lymphocytic hypophysitis due to a ruptured Rathke’s cleft cyst and management post-recurrence. Presentation: 6/3/2024

6619 An Unlikely Cause of Oligomenorrhea: Acromegaly Secondary to a Sparsely Granulated, Somatotroph-Lactotroph Adenoma

Abstract Disclosure: S.I. Alicea: None. Y. Wu: None. J. Marquez: None. C.S. Botero Suarez: None. M. Kinaan: None. Introduction: Acromegaly most commonly presents as a pituitary tumor that causes excessive production ofgrowth hormone (GH); and rarely due to excess of GHRH or ectopic production of GH. Approximately 15% of surgically removed pituitary adenomas represent GH-producing neoplasms, and about 5% are bihormonal, secreting both GH and prolactin (PRL). We present a case of an unexpected presentation of oligomenorrhea which ended up with the diagnosis of acromegaly secondary to plurihormonal mixed somatotroph-lactotroph sparsely granulated adenoma. Case Description A 32-year-old female with history of prediabetes was referred to our clinic by her gynecologistfor evaluation of oligomenorrhea which was initially attributed to polycystic ovarian syndrome.She reported menarche at age 10 and has experienced irregular menses and periods of amenorrhea over the years. Physical exam revealed macroglossia, a widened thickened nose, acanthosis nigricans in the neck skin folds, facial hair thickening, and significant thickening of all fingers, toes and soft tissues. Given physical exam findings, we evaluated the patient for acromegaly and lab testing revealed markedly high IGF I, mildly elevated prolactin, and hemoglobin A1C of 5.7%. An MRI of the brain with IV contrast revealed a heterogeneously enhancing pituitary macroadenoma measuring 2.9 x 2.9 x 2.2 cm with suprasellar extension resulting in mass effect on the optic chiasm without definite cavernous sinus extension. She was started on Cabergoline 0.5mg twice weekly temporarily and underwent pituitary transsphenoidal resection. Pathology results revealed tumor cells that were positive for Chromogranin and Cam5.2. About 10% of cells showed immunoreactivity for Prolactin and negative for TSH, ACTH, FSH, LH and immunostains. There was also weak focal (about 2%) immunoreactivity for GH. Occasional mitotic figures were present. The MIB-1 proliferation index was 3%. The reticulin stain demonstrates loss of acinar architecture, supporting the diagnosis. Aside from transient sodium fluctuations consistent with triphasic response, she had no long-lasting postoperative complications. One month after her surgery, her IGF I level and hemoglobin A1C are trending down and she reports symptomatic relief. She continues follow up in our clinic. Discussion This patient has acromegaly secondary to a mixed somatotroph-lactotroph sparsely granulated adenoma. Sparsely granulated adenomas are more common in younger populations, can be more aggressive, and are less responsive to somatostatin analogs. Early detection and treatment is imperative in the prevention of complications secondary to the disease. Our case will closely examine the differences in the clinical, prognostic, and pathologic features of sparsely- vs. densely granulated adenomas. We will also discuss the effects of acromegaly on gonadal function. Presentation: 6/1/2024

7025 Acute Psychosis and the Diagnosis of Cushing’s Syndrome: Lessons Learned from a Patient with a Long History of Obesity

Abstract Disclosure: S. Modi: None. L. Belalcazar: None. Background: Although the association of hypercortisolism and psychosis is well established, acute psychosis remains a very rare manifestation of Cushing’s syndrome, especially in the absence of previous psychiatric history. We present lessons learned from a patient in whom the diagnosis of Cushing’s syndrome was missed until the onset of acute psychosis helped identify his underlying endocrine disorder. Clinical Case: A 31-year-old male presented to the Emergency Department after being found down on the street. The patient had superficial lacerations that were suspected to be self-inflicted due to a suicide attempt, but later found to be the result of auditory hallucinations. He had no previous psychiatric history and no history of suicidal ideation. His medical history was positive for obesity since adolescence and a recent diagnosis of hypertension. His family noted that for the past several months he had been experiencing headache, fatigue, muscle weakness and, more recently, insomnia, restlessness, and confusion. Weight gain was noted, but of unknown magnitude. On admission, he was agitated and hallucinating. He was initially sedated with dexmedetomidine and started on quetiapine. Physical examination revealed Cushingoid features with truncal obesity (body mass index of 35 kg/m2), facial plethora, and dorsocervical fat pad; no abdominal striae were present. Labs showed mild hypokalemia. His AM cortisol was markedly elevated at > 123 ug/dL (normal 4.5-23.0 ug/dL) with an ACTH level at 144 pg/mL (normal 7.2-63.3 pg/mL). MRI Brain showed a 1.9 cm pituitary macroadenoma with small suprasellar extension. With a diagnosis of Cushing’s disease, later confirmed by pathology, he underwent transsphenoidal resection. AM cortisol on post-operative day 3 was 3.2 ug/dL (normal 4.5-23.0 ug/dL). Maintenance-dose steroids were started due to evidence of post-surgical corticotroph suppression. Notably, there was marked improvement in his anxiety, paranoia, and confusion post-operatively. Quetiapine and blood pressure medications were discontinued after discharge. Conclusion: In this patient, the diagnosis of Cushing’s syndrome was missed when initially presenting with new onset hypertension in the setting of long-standing obesity. Despite months of symptoms compatible with the diagnosis, it was not until he developed acute psychosis and a careful history was obtained, that the diagnosis of Cushing’s syndrome was entertained. Psychosis is present in only 3-8% of reported cases of Cushing’s syndrome. This case illustrates the importance of including Cushing’s syndrome in the differential diagnosis of acute psychosis, especially in patients without previous psychiatric history. It also highlights the need of excluding secondary causes of hypertension in young adults, with particular attention to Cushing’s syndrome in those who present with obesity, even if long-standing. Presentation: 6/1/2024

9234 A Case of Gigantism Due to An AIP Gene Mutation: Implications for Genetic Counseling and Preimplantation Genetic Diagnosis

Abstract Disclosure: M. Serebro: None. M. Yacobi Bach: None. N. Even Zohar: None. A. Reches: None. Y. Greenman: None. From the age of 17 the patient experienced accelerated growth, facial changes, and enlargement of hands and feet. Despite having a sister already diagnosed with acromegaly, the patient did not undergo endocrine evaluation until the age of 25, when he was diagnosed with gigantism. Magnetic resonance imaging (MRI) revealed the presence of a macroadenoma with suprasellar extension reaching the optic chiasm. The patient underwent transsphenoidal surgery and excision of a sparsely granulated somatotroph adenoma that was Pit1 positive and exhibited weak immunostaining for growth hormone (GH). Insulin-like growth factor 1 (IGF1) levels normalized after surgery, and a fasting GH level was 0.6 ng/ml. Given the family history and the early onset of the disease, genetic counseling was recommended. Genetic testing revealed a heterozygote likely pathogenic variant in the AIP gene (c.713G>A). This finding prompted the recommendation for genetic testing of all first-degree relatives. The patient and his wife, who desired to have children, were referred to obstetrics and gynecology for pregestational diagnosis. Family segregation testing indicated that the patient's mother also carried the same AIP variant. Preimplantation genetic diagnosis (PGD) was conducted, revealing that 11 out of 20 embryos were carriers of the AIP variant. The non-carrier embryos were selected for implantation, and pregnancy was achieved. Efforts to inform first-degree relatives about the importance of genetic testing were undertaken, and one sister was tested and found to be healthy. Other siblings have not yet been tested. The patient's mother, identified as an AIP variant carrier, underwent endocrine laboratory tests that showed GH, IGF1, and prolactin levels within the normal range. Brain MRI did not reveal a pituitary lesion.This case highlights the significance of genetic counseling and testing in patients with familial endocrine disorders, the potential implications of a molecular diagnosis of a pathogenic AIP variant, and the challenges faced in reproductive decision-making for affected individuals and their families. Presentation: 6/3/2024

8186 Hypoglycemia as an Unusual Presentation of Panhypopituitarism

Abstract Disclosure: P. Chalasani: None. M. Asfaw: None. M. Mansour: None. M. Hammoud: None. T. Altaweel: None. H. Ilyas: None. L. Lawrence: None. Introduction: Hypopituitarism refers to the diminished or complete absence of anterior pituitary function, which may arise from acquired or congenital factors. The symptoms depend on the patient’s age and the hormones affected. We report a case where hypoglycemia led to a new diagnosis of panhypopituitarism in an adult patient. Clinical Case:A 53-year-old female with a past medical history of hypertension was brought to the emergency department after syncope. Patient experienced diaphoresis and loss of consciousness associated with urinary incontinence. She did not have confusion, tongue biting, or muscular weakness after the episode. On presentation, the patient was hypoglycemic with blood glucose of 39 mg/dl and received IV dextrose. No focal neurologic deficits were noted on physical examination. She was mildly hypotensive with blood pressure 98/66 mmHg, and received hydrocortisone 100 mg IV once, and started on IV dextrose infusion. Hypoglycemia workup revealed elevated insulin 16.6 uIU/mL and C-peptide 4.8 ng/mL (1.1-4.4) levels for a blood glucose level of 132 mg/dl, with normal beta-hydroxybutyrate of 0.14 mmol/L (0.02-0.27). Sulfonylurea screen was negative. CT abdomen revealed no pancreatic lesions or abnormalities. Hyperinsulinemia was attributed to insulin resistance in this patient with a BMI of 34 kg/m2. CT head revealed a heterogeneous low-density expansile mass in the sella/suprasellar region measuring approximately 2.5 x 2.3 x 2 cm. MRI brain confirmed a heterogeneous sellar mass with suprasellar extension measuring 3.4 x 2.3 x 2.1 cm abuting the optic chiasm without displacement. Biochemical workup revealed panhypopituitarism with secondary adrenal insufficiency ACTH < 1.5 pg/mL (7.2-63.3) and cortisol 3.63 ug/dL (3.70-19.40); secondary hypothyroidism TSH 0.84 mIU/mL (0.27-4.20) and FT4 < 0.50 ng/dL (0.70-14.8); growth hormone deficiency with low IGF-1 23 ng/mL (65-216); secondary hypogonadism FSH < 1 mIU/mL (46-177), LH < 1 mIU/mL (7.7-59) and estrone < 6 pg/mL (0-125); with normal prolactin 14.1 ng/mL (4.8-23.3). Patient revealed she had been amenorrheic since 23 years old and conceived one child prior. Patient was transitioned to oral hydrocortisone 20 mg AM and 10 mg PM, and started on levothyroxine 50 mcg PO daily. No neurosurgical intervention was recommended. Subsequently, patient requested a second opinion and was transferred to a tertiary center. Conclusion: The link between hypoglycemia and hypopituitarism, may be attributed to hypocortisolism and growth hormone / IGF-1 deficiency. Intriguingly, this patient's initial clinical presentation centered around hypoglycemia, and further evaluation led to the identification of a sellar mass and new diagnosis of panhypopituitarism. This case demonstrates the need to include hypopituitarism in the differential when evaluating hypoglycemic patients. Presentation: 6/2/2024

Postural Change Assists Surgical Maneuverability During Endoscopic Transsphenoidal Surgery for Pituitary Macroadenoma

In endoscopic transsphenoidal surgery for pituitary adenoma with suprasellar extension, the prolapse of the stretched floppy diaphragma sellae can obstruct the surgical corridor, posing challenges during pituitary surgery. We have introduced a simple postural change technique to mitigate this issue and are sharing our clinical experience. Pituitary surgery is performed on an operating bed where the patient's position can be adjusted. During transsphenoidal pituitary surgery, raising the patient's head to a Fowler's position causes the diaphragma sellae to move upward, while lowering the head to a supine position causes it to descend. This simple adjustment minimizes hindrance from the floppy diaphragm sellae during surgery. We illustrate this technique in pituitary surgery, where diaphragm sellae relocation is necessary. A simple postural change technique effectively manages prolapsed floppy diaphragma sellae, enhancing visualization and surgical accessibility during endoscopic transsphenoidal pituitary surgery.

Evaluation and follow-up of patients diagnosed with hypophysitis: a cohort study.

Primary hypophysitis might be challenging to diagnose, and there is a lack of evidence regarding optimal treatment strategies due to rarity of the disease. We aim to investigate the clinical features and compare the outcomes of different management strategies of primary hypophysitis in a large group of patients recruited on a nationwide basis. A retrospective observational study. The demographic, clinical, and radiologic features and follow-up data were collected in study protocol templates and analyzed. One hundred and thirteen patients (78.8% female, median age: 36 years) were included. Lymphocytic (46.7%) and granulomatous hypophysitis (35.6%) were the prevailing subtypes out of 45 patients diagnosed after pathologic investigations. Headache (75.8%) was the most common symptom, and central hypogonadism (49.5%) was the most common hormone insufficiency. Of the patients, 52.2% were clinically observed without interventions, 18.6% were started on glucocorticoid therapy, and 29.2% underwent surgery at presentation. Headache, suprasellar extension, and chiasmal compression were more common among glucocorticoid-treated patients than who were observed. Cox regression analysis revealed higher hormonal and radiologic improvement rates in the glucocorticoid-treated group than observation group (hazard ratio, 4.60; 95% CI, 1.62-12.84 and HR, 3.1; 95% CI, 1.40-6.68, respectively). The main indication for surgery was the inability to exclude a pituitary adenoma in the presence of compression symptoms, with a recurrence rate of 9%. The rate of spontaneous improvement might justify observation in mild cases. Glucocorticoids proved superior to observation in terms of hormonal and radiologic improvements. Surgery may not be curative and might be considered in indeterminate, treatment-resistant, or severe cases.

Simultaneous combined keyhole mini-transcranial approach and endoscopic transsphenoidal approach to remove multi-lobulated pituitary neuroendocrine tumor with suprasellar extension

BackgroundTranssphenoidal surgery (TSS) is the main method to remove pituitary neuroendocrine tumor (PitNET), but large or multi-lobulated one is still challenging. ObjectiveThe less invasive simultaneous combined keyhole mini-transcranial approach and standard transsphenoidal approach was used to remove multi-lobulated or hourglass-shaped non-functioning PitNET to avoid the specific complications related to extended TSS. MethodsTwenty-one patients of large multi-lobulated or hourglass-shaped PitNET (6 patients were recurrent cases after TSS or transcranial approach) were treated by this simultaneous combined approach. The supraorbital or midline keyhole approach was performed under the operating microscope so that the fine arteries feeding optic apparatus were dissected from the tumor capsule safely and securely and the suprasellar part of the tumor was removed in the extra-capsular fashion in combination with transsphenoidal approach. ResultsVisual function improved after the surgery in 14 (74 %) of 19 patients with preoperative disturbance of the visual fields. Four patients (19 %) developed postoperative deterioration of pituitary endocrine functions requiring permanent hormonal replacement. One patient (4.2 %) suffered cortical laminar necrosis without permanent disorders and another patient (4.2 %) suffered cerebrospinal fluid rhinorrhea but neither was permanent. Postoperative computed tomography detected no subarachnoid hemorrhage in any patient. Gross total removal was achieved in 15 patients (71 %). Five patients (24 %) with subtotal removal and one patient (4.2 %) with partial removal had lateral tumor extension with Knosp classification 3 or 4. ConclusionThe combined method is an effective treatment option to remove complex PitNET safely and securely with dissecting fine arteries on the tumor capsule.

Hypopituitarism after gamma knife radiosurgery for pituitary adenomas: long-term results from a single-center experience

ObjectiveThe aim of this study was to investigate the incidence and risk factors of new-onset hypopituitarism after gamma knife radiosurgery (GKRS) for pituitary adenomas in a single center.MethodsIn this retrospective study, 241 pituitary adenoma patients who underwent GKRS from 1993 to 2016 were enrolled. These patients had complete endocrine, imaging, and clinical data before and after GKRS. The median follow-up time was 56.0 (range, 12.7–297.6) months.ResultsFifty patients (20.7%) developed new-onset hypopituitarism after GKRS, including hypogonadism (n = 22), hypothyroidism (n = 29), hypocortisolism (n = 20), and growth hormone deficiency (n = 4). The median time to new-onset hypopituitarism was 44.1 (range, 13.5–141.4) months. The rates of new-onset hypopituitarism were 7%, 16%, 20%, 39%, and 45% at 1, 3, 5, 10, and 15 years, respectively. For those patients treated with a single GKRS, sex (p = 0.012), suprasellar extension (p = 0.048), tumor volume (≥ 5 cm3) (p < 0.001), tumor progression (p = 0.001), pre-existing hypopituitarism (p = 0.011), and previous surgery (p = 0.009) were significantly associated with new-onset hypopituitarism in univariate analysis. In the multivariate analysis, tumor volume (≥ 5 cm3) and tumor progression were associated with new-onset hypopituitarism (hazard ratio [HR] = 3.401, 95% confidence interval [CI] = 1.708–6.773, p < 0.001 and HR = 3.594, 95% CI = 1.032–12.516, p = 0.045, respectively). For patients who received 2 or more times GKRS, no risk factors associated with new-onset hypopituitarism were found.ConclusionNew-onset hypopituitarism was not uncommon after GKRS for pituitary adenomas. In this study, large tumor volume (≥ 5 cm3) and tumor progression were associated with new-onset hypopituitarism after a single GKRS.

Aggressive Prolactinoma with Progression to Pituitary Carcinoma: A Case Report.

Adenomas of the pituitary gland, predominantly prolactinomas, can exhibit aggressive behavior. Aggressive prolactinomas are characterized by radiographic invasion, rapid growth, clinically significant progression despite standard therapies, and recurrence after surgery or radiotherapy. Pituitary carcinoma is rare (0.1-0.2% of pituitary tumors) [1, 2]. Case Presentation: In 2005, a 50-year-old man presented with bitemporal hemianopsia and severe asthenia due to a large pituitary tumor. Hormonal tests revealed hyperprolactinemia and panhypopituitarism; he received hormonal replacement and dopamine agonists (DA) therapy with a reduction in prolactin levels. Ten years later, he experienced tumor regrowth consistent with pituitary apoplexy and VI cranial nerve palsy. MRI showed a macroadenoma with suprasellar extension and compression of the optic structures. The patient underwent transsphenoidal surgery in view of the partially resistant disease. Histopathology showed a pituitary macroadenoma, and immunohistochemistry showed a high mitotic index (Ki-67 80%). In 2016, the patient developed a partial deficit of the left sixth cranial nerve. He underwent a new surgery but with incomplete resection. In view of the aggressive and resistant nature of the disease, he received radiotherapy. In 2020, prolactin levels began to increase again. MRI showed an occipital- temporal lesion. Subsequently, he underwent radiotherapy and started chemotherapy with temozolomide, resulting in the normalization of prolactin levels in the absence of DA therapy. The patient is currently in remission, with no evidence of tumor recurrence. Conclusion: It was found that 15% of prolactinomas are resistant to DAs, and resistance to DA may signal malignant transformation. Therefore, multimodality therapy and molecular analysis are critical for aggressive prolactinomas and pituitary carcinoma.

The key point of transsphenoidal surgery for infradiaphragmatic craniopharyngioma：Better sellar diaphragm resection

BackgroundCraniopharyngiomas have a high recurrence rate and a poor prognosis, and the key methods for reducing recurrences are unknown. The aim of this study was to explore the key points of microscopic or endoscopic transsphenoidal surgery used to treat infradiaphragmatic craniopharyngiomas. MethodsWe reviewed the medical records of patients with infradiaphragmatic craniopharyngiomas who were admitted to Peking Union Medical College Hospital between 2011 and 2018. ResultsWhen considering tumor location, all 34 patients had intrasellar tumors, with 19 of them exhibiting suprasellar extensions. Of the 34 patients, 24 patients underwent resection under the microscope and the remaining 10 patients underwent transsphenoidal endoscopic surgery. Gross total tumor resection was achieved in 16 patients. Twelve patients underwent invaded sellar diaphragm resection, while the remaining 22 patients were not. Cerebrospinal fluid leaks occurred during surgery in 18 patients. Visual acuity improved in two patients. After an average follow-up of 31.1 months, 13 patients experienced tumor recurrence. The short term recurrence rate in the sellar diaphragm resection group was significantly lower compared to the non-resected group (P < 0.001). Moreover, based on distinct surgical methods, the endoscope group displayed a reduced short term recurrence rate compared to the microscope group (P = 0.0048). ConclusionInvaded sellar diaphragm resection emerges as a pivotal maneuver in craniopharyngioma surgery, substantively influencing tumor recurrence. Capitalizing on the advantageous angled lens of endoscopes, surgeons can achieve heightened visualization. Significantly, the endoscopic approach exhibits a superior capacity to curtail recurrence, while effectively managing potential complications, when contrasted with the microscope group.

Endoscopic endonasal resection of Rathke cleft cysts: a single-institution analysis of 148 consecutive patients.

The traditional treatment of sellar Rathke cleft cysts (RCCs) generally involves transsellar drainage; however, suprasellar RCCs present unique challenges to appropriate management and technical complexity. Reports on overall outcomes for the endoscopic endonasal approach (EEA) for this pathology are limited. The EEA for RCCs allows three surgical techniques: marsupialization, fenestration, and fenestration with cyst wall resection. The authors performed a retrospective review of consecutive patients with RCCs that had been treated via an EEA at a single institution between January 2004 and May 2021. Marsupialization entailed the removal of cyst contents while maintaining a drainage pathway into the sphenoid sinus. Fenestration involved the removal of cyst contents, followed by separation from the sphenoid sinus, often with a free mucosal graft or vascularized nasoseptal flap. Cyst wall resection, either partial or complete, was added to select cases. A total of 148 patients underwent an EEA for RCC. Marsupialization or fenestration was performed in 88 cases (59.5%) and cyst wall resection in 60 (40.5%). Cysts were classified as having a purely sellar origin (43.2%), sellar origin with suprasellar extension (37.8%), and purely suprasellar origin (18.9%). Radiological recurrence was demonstrated in 22 cases (14.9%) at an average 39.7 months' follow-up (median 45 months, range 0.5-99 months), including 13 symptomatic cases (8.8%). Cases with cyst wall resection had no significantly different rate of recurrence (11.7% vs 15.9%, p = 0.48) or postoperative permanent anterior pituitary dysfunction (21.6% vs 12.5%, p = 0.29) compared to those of fenestrated and marsupialized cases. There was no significant difference in postoperative permanent posterior pituitary dysfunction based on technique, although such dysfunction tended to worsen with cyst wall resection (13.6% vs 4.0%, p = 0.09). Based on cyst location, purely suprasellar cysts were more likely to have a radiological recurrence (28.6%) than sellar cysts with suprasellar extension (12.5%) and purely sellar cysts (9.4%; p = 0.008). Most notably, of the 28 purely suprasellar cysts, selective cyst wall resection significantly improved the long-term (10-year) recurrence risk compared to fenestration alone (17.4% vs 80.0%, p = 0.0005) without any significant added risk of endocrinopathy. Endoscopic endonasal marsupialization or fenestration of sellar RCCs may be the ideal treatment strategy, whereas purely suprasellar cysts benefit from partial cyst wall resection to prevent recurrence. Selective cyst wall resection reduced long-term recurrence rates without significantly increasing rates of hypopituitarism.

P.090 Third ventricular pituicytoma: case report and review of the literature

Background: Pituicytoma is a rare, low grade tumour typically of the sellar region. Here we present a pituicytoma located in the third ventricle. Methods: 56 year old female presented with amenorrhea, hyperprolactinemia, and progressive bitemporal hemianopsia. MRI revealed a suprasellar mass located within the third ventricle and appearing separate from the pituitary. A supraciliary and translamina terminalis surgical approach to tumour resection was completed without complication. Post-operatively, she developed transient DI which resolved by post-opertaive day 3 and she was discharged hoem without any neurological deficits. Pathology revealed pituicytoma, WHO grade I. Results: Pituicytomas are rare tumours arising from neuroepithelial cells of the pituitary. The majority of cases are pure sellar or sellar with suprasellar extension, or at least have some connection to the pituitary. In many cases, imaging findings are synonymous to pituitary adenomas. We present a unique case in which the tumour was suprasellar but appeared separate from the pituitary. Surgical intervention is the most highly predictive factor of recurrence, as gross totoal reseciton can be curable. Conclusions: Here we present a unique location of pituictyoma. Due to the exceedingly rare nature of pituicytoma, unique presentations and management help to provide better understanding of the breadth of this disease presentation.

504 Anterior-posterior Diameter Is a Key Driver of Resectability and Complications for Pituitary Adenomas With Suprasellar Extension in Endoscopic Transsphenoidal Surgery

INTRODUCTION: As endoscopic transsphenoidal approaches are more routinely selected for larger pituitary adenomas with parasellar extension, understanding potential anatomical factors that limit resection and contribute to complications becomes increasingly important. METHODS: A single-center retrospective review of all endoscopic transsphenoidal surgeries for pituitary adenomas with suprasellar extension from 2015-2020 was performed. Preoperative MRIs were systematically assessed to assign a Knosp classification, a Zurich Pituitary Score (ZPS), and to measure lesional suprasellar dimensions. Univariate comparisons and multivariate regression models were employed to assess the influence of these factors on extent of resection (EOR) and postoperative complications. RESULTS: Of the 96 patients with suprasellar pituitary adenomas who underwent endoscopic transsphenoidal surgery, 74 patients (77%) had a gross total resection (GTR). Neither Knosp grade nor ZPS were associated with GTR (Knosp 3A-4 versus Knosp 0-2, p = 0.069; ZPS III-IV versus ZPS I-II, p = 0.079). Multivariate regression identified suprasellar anterior-posterior tumor diameter (SSAP) as the only significant predictor of EOR (OR 0.951, 95% CI 0.905-1.000, p = 0.048*). Higher SSAP also showed the strongest association with an increased overall complication rate (p = 0.0003*) and intraoperative CSF leaks (p = 0.0012*). Further analysis of the regression model for GTR suggested an optimal cut point value for SSAP of 23.7 mm, above which predictability for failing to achieve GTR carried a sensitivity of 89% and specificity of 41%. CONCLUSIONS: This study is unique in its examination of endoscopic transsphenoidal surgical outcomes for pituitary adenomas with suprasellar extension. Our findings suggest that previously established grading systems based on lateral extension into the cavernous sinus lose their predictive value in lesions with suprasellar extension and, more specifically, with increasing suprasellar anterior-posterior diameter.

The trans-sylvian trans-petrosal "half & half" approach-a how I do it.

Petroclival meningiomas are one of the most challenging tumors to be operated in the realm of neurosurgery. Many approaches have been developed over the years. The authors describe the Half & Half (H&H) approach whose main indication is petroclival meningiomas with suprasellar extension. The part of the tumor located above CN III and in the retrochiasmatic space is addressed through a trans-sylvian, while the petroclival portion is through an extradural anterior petrosectomy approach. The wide surgical corridor given by this approach allows extensive tumor resection while avoiding the risk associated with the manipulation of intracavernous neurovascular structures. The H&H approach is an effective strategy to maximize the safe resection of petroclival meningiomas.