Suprasellar Extension Research Articles

Spontaneous Near-Resolution of a Rathke’s Cleft Cyst

•Spontaneous near resolution of Rathke’s cleft cyst can occur•Conservative approach is recommended in the absence of mass effect or visual disturbance•The mechanism of spontaneous near resolution of RCC is poorly understood A 70-year-old Caucasian woman with history of chronic sinusitis presented to the Emergency department with 2-week history of headaches. Her physical examination was unremarkable including absence of galactorrhea, abnormal hair growth, or skin changes. Neurologic exam including visual fields were normal. Brain MRI revealed a 1.2 x 1.4x 1.3 cm ovoid, non-enhancing, cyst-like lesion with intrasellar and suprasellar components (Figure 1) with slight elevation and splaying of the optic chiasm and depression of the sella floor. Hormonal testing revealed normal values (Table 1). A conservative approach was chosen given the patient’s age, the lesion size, and lack of visual compromise or hormonal dysfunction.Table 1HormoneResultNormal RangeTSH0.90.280 – 3.980 ng/dlFree T40.600.58-1.64 ng/dlIGF-13741-279 ng/mlGrowth Hormone0.9<7.1 ng/mlProlactin19.272.74-19.64 ng/mLCortisol16.67-9a.m 4.0-22.0 mcg/dl Open table in a new tab At 1 month follow up she denied new symptoms. Repeat hormonal panel revealed a low FT4 0.5, and normal TSH 3.93, which prompted initiation of levothyroxine. The rest of the panel remained normal. A follow-up brain MRI revealed a hypoenhancing lesion within the sella with suprasellar extension measuring 1.1x 1.3 x0.9 cm (figure 2), contacting the optic chiasm without displacement. Ophthalmology evaluation reported normal visual fields. Neurosurgery recommended medical management. A year after initial presentation, TSH and Ft4 were normal on levothyroxine. Brain MRI revealed complete resolution of the previously characterized cystic lesion, with a normal-sized and shaped pituitary gland (figure 3). Patient continued to have intermittent headaches that were attributed to chronic sinusitis. The patient has not returned for a follow up visit, and we have been unable to reassess her pituitary/thyroid axis. What is the diagnosis? Answer: The clinical course and imaging findings are best explained by a rare case of a Rathke’s Cleft Cyst (RCC), with near spontaneous resolution. RCC are remnants of a failed regression of the developmental ectodermal pouch, characterized by unpredictable natural histories that can include spontaneous enlargement or less commonly decrease in size. The latter has been observed in up to 15% in studies evaluating the evolution and volumetry analysis of RCC (1Barkhoudarian G. Palejwala S.K. Ansari S. Eisenberg A.A. Huang X. Griffiths C.F. et al.Rathke’s cleft cysts: a 6-year experience of surgery vs. observation with comparative volumetric analysis.Pituitary [Internet]. 2019; 22 (Available from:): 362-371https://doi.org/10.1007/s11102-019-00962-yCrossref PubMed Scopus (19) Google Scholar). Symptoms may include headaches, pituitary dysfunction, and visual deficits from mass effect (2Altuwaijri N, Cote DJ, Lamba N, Albenayan W, Ren SP, Zaghloul I, et al. Headache Resolution After Rathke Cleft Cyst Resection: A Meta-Analysis. World Neurosurg [Internet]. 2018;111:e764–72. Available from: https://doi.org/10.1016/j.wneu.2017.12.170Google Scholar). There are no standardized management guidelines, however a conservative approach is preferred when there is absence of mass effect or visual disturbance as seen here(3Truong L.U.F. Bazin C. Gomis P. Decoudier B. Delemer B. Litré C.F. Surgery versus conservative care for Rathke’s cleft cyst.Neurochirurgie. 2021; 67: 104-111Crossref PubMed Scopus (4) Google Scholar). The mechanism of spontaneous resolution of RCC is poorly understood. This is a rare case of spontaneous near resolution of RCC in a patient that was followed by conservative therapy with regular clinical and radiologic follow up.

Review of first 60 pituitary adenoma cases of a skull base team, one center experience

Background: Endoscopic endonasal transsphenoid approach of pituitary gland is being effectively replacing microscopic approach. This approach need a lot of training and stepwise learning to reach perfection. This study discuss the experience and pitfalls of an evolving skull base team in one institute. Aim of the work: Analysis of first sixty pituitary adenomas patients operated by an evolving skull base teams in one institute, and highlighting modifications introduced in their technique. Methodology: This is a retrospective analytical study of the medical records of first 60 pituitary adenoma patients who had presented to one center and had endoscopic trans-nasal trans-sphenoidal approach by conjoined skull base team. Results: There were 60 cases of pituitary adenoma; 23 males, and 37 females. Mean age were 46 years old. 37 cases (61%) had temporal field defect, 22 cases had visual acuity loss, 41 patients (68%) had nonfunctioning adenomas, and 18% were prolactinomas. Fifty two cases (86.6%) were macroadenoma, and 2 patients (3.3%) were microadenoma. 48 cases (80%) had suprasellar extension, while 19 cases (19%) had parasellar extension. Three cases (5%) were recurrent. Trans-nasal cavity approach was adopted in 58 cases (96.6%). 4 cases needed middle turbinate resection. Mean operative time was 3 hours. Gross total removal of the tumor were achieved in 49 cases (81.6%). Intraoperative CSF leakage occurred in 31 patients (51%). Failure of reconstruction occurred in 4 cases (6.6%). Mean duration of hospital, and ICU stay was 8, 2 days respectively. Postoperative temporary diabetes insipidus occurred in 35 cases (58.3%), 5 of them (8.3%) had permanent DI. Major CNS complications occurred in 2 cases (3%). Conclusion: The more experience gained the more modifications introduced to improve surgical outcome; more gross total resection rate, less morbidity, and mortality rates, less operative time, better quality of life of patients and less time and effort exhaustion of the team work.

Anesthetic and hemodynamic management in patients with different approaches for multiple neurological pathologies. case report

A 54-year-old female presented with complaints of progressive visual loss and severe headaches for the past two months. The patient had a history of high blood pressure and was on medication for the same. On physical examination, she had a loss of peripheral vision and a visual acuity of 20/200 in her left eye. An urgent MRI scan was performed which revealed a 2 cm x 1.5 cm sellar mass with suprasellar extension and a concomitant incidental aneurysm in the left internal carotid artery ophthalmic segment. Further evaluation with hormonal profile revealed elevated levels of insulin-like growth factor 1 (IGF-1), which was suggestive of growth hormone-producing pituitary adenoma. A cerebral angiogram confirmed the presence of the incidental aneurysm in the left internal carotid artery ophthalmic segment.

Drivers of Complications and Outcomes in Patients with Pituitary Adenomas and Suprasellar Tumor Extension

Drivers of Complications and Outcomes in Patients with Pituitary Adenomas and Suprasellar Tumor Extension

Magnetic resonance imaging grading of pituitary macroadenoma – SIPAP classification revisited

BackgroundMagnetic resonance imaging (MRI) is regarded as the modality of choice in diagnosis of pituitary macroadenomas. Since surgery is the first line therapy for all pituitary adenomas, simple and reproducible MRI classification based on major directions of tumour growth is an essential tool. SIPAP MRI classification for pituitary adenoma describes tumor extension in parasellar, suprasellar, infrasellar, anterior and posterior directions. We, therefore, evaluated reproducibility of SIPAP classification in reporting of pituitary adenomas. MethodsForty-nine patients with biopsy-proven pituitary macroadenoma were graded according to SIPAP classification. Data was analyzed using Stata version 15. Interobserver variability was calculated using Cohen’s Kappa. Comparison between grading before and after treatment was performed by Chi-square test. P values < 0.05 were considered statistically significant. ResultsIndividual tumour extensions according to SIPAP for pre- and post-operative grading showed significant difference (p-value <0.001), except for anterior extension. For suprasellar extension, 67.3 % patients had pre-operative grade-3 and 63.3 % had post-operative grade-0. For infrasellar extension, 51.0 % had pre-operative grade-2 and 71.4 % had post-operative grade-0. Anterior, posterior and parasellar extensions showed increased frequency in grade-0 in post-operative stage compared to pre-operative. Substantial inter-observer agreement was achieved for Superior, Inferior, Anterior and Posterior extent with all Kappa statistics values above 0.7 (p-value <0.001). ConclusionWe propose incorporating simple and objective SIPAP classification in routine MR reporting for ideal pituitary tumour delineation, relationship to juxtasellar structures and tumour size, hence facilitating greater success rate in surgical and subsequent clinical management.

Cerebral vasospasm following subarachnoid hemorrhage: a rare complication after transsphenoidal surgery for pituitary macroadenoma.

Postoperative (PO) complications after transsphenoidal surgery (TSS) are rare when performed in pituitary referral centers. Partial hypopituitarism is more frequent and somewhat expected. Meningitis, cerebrospinal fluid leaks, and visual deficits are unusual. Cerebrovascular complications, including cerebral vasospasm are rare, usually under-appreciated and not mentioned to the patient prior to the surgery. This is a report of a 51-year-old male with a non-functioning pituitary macroadenoma presenting with partial hypopituitarism and visual field loss. The patient was submitted to an uneventful TSS. On the first PO day, he developed a left palpebral ptosis with unequal pupils and impaired consciousness (12 points on Glasgow Coma Scale). CT scan revealed a perimesencephalic subarachnoid hemorrhage (SAH) grade 1 according to the modified Fisher scale. High-dose dexamethasone (16 mg/day) was initiated and the patient became more alert (Glasgow 14). On the fifth PO day, due to progression of the neurological deficits (left III, IV, and VI cranial nerves palsy, ataxia, dysdiadochokinesia, right dysmetria, and dysarthria), a magnetic resonance angiography was obtained and revealed a recent mesencephalic infarct without evident vasospasm. Nevertheless, nimodipine 60 mg 4/4 h was initiated. No improvement was seen after 3 days of treatment. The patient was discharged and put on rehabilitation, returning to normal gait and balance after 7 months. This, therefore, is a case of an unexpected mesencephalic infarct probably due to vasospasm induced by minor SAH. Although exceptionally rare, informing the patient about this event prior to TSS is important due to its significant neurological impact. More data are needed considering preventive treatment with nimodipine as soon as SAH is detected after TSS and whether it would improve neurological outcomes. Whenever neurological deficits arise after transsphenoidal surgery (TSS), systemic infection, meningitis, electrolyte imbalance, and evident hemorrhage must be promptly investigated. Although rare, cerebral vasospasm (CVS) after TSS is associated with high morbidity and high mortality rates. Vigilance for vasospasm is necessary for patients undergoing TSS for pituitary adenoma, especially those with significant suprasellar extension. Informing this event to the patient prior to TSS is essential due to its significant morbidity and mortality. Post-TSS subarachnoid hemorrhage and hemiparesis may be important clues indicating CVS and infarction. There is limited evidence in the literature regarding post-TSS CVS surveillance and treatment strategies which could have an impact on clinical decisions.

A GIANT PROLACTINOMA: A CASE REPORT

A 38-years-old woman presented to our hospital 2 years ago with 5-year history of reduced vision, chronic cephaleea and secondary amenorrhea. Her baseline prolactin level was 46504 µIU/mL (reference range=127-637mUI/ml) with secondary hypogonadism, and pituitary magnetic resonance imaging revealed a giant prolactinoma (4 × 2.2 × 2.6 cm3) with suprasellar extension, optic chiasma and right cavernous sinus compression. She was initially treated with cabergoline in order to reduce the prolactin level and tumoral mass, but after 6 months of medical treatment she proceed to transsphenoidal tumor debulking surgery. Prolactin level dropped in the normal range only after the addition of cabergoline treatment after surgery (1 mg/day 3 times/per week). However, the extensive tumour was not completely resectable so she remained amenorrheic requiring hormone replacement therapy as well as thyroxine replacement due to the development of TSH deficiency (free T4 6.03 pmol/L).

Retinal nerve fiber layer thickness and its correlation with visual symptoms and radiological features in pituitary macroadenoma.

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. 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. 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). 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.

Outcomes in pituitary adenoma causing acromegaly following endoscopic endonasal transsphenoidal surgery.

The objectives of the study were to study the analysis of outcomes after endoscopic endonasal transsphenoidal surgery (EETSS) in acromegaly in terms of surgical complications, clinical improvement, endocrinological remission, achievement of prognostically critical growth hormone (GH) level, and requirement of additional treatment. The study included 28 acromegaly patients, who underwent EETSS. A 2010 consensus criterion was used for defining remission. Assessment of prognostically critical GH level (random value <2.5 ng/ml), the extent of resection and additional treatment, was done at post-operative week (POW) 12. All adenomas were macroadenomas; with a mean volume of 16.34 cm3 (range, 0.4-99 cm3). Most adenomas had high-grade extensions. Most common suprasellar, infrasellar, anterior, and posterior extension grades were 3 (n = 13), 1 (n = 16), 1 (n = 14), and 0 (n = 20), respectively. Knosp Grade 3 was common on both sides (right, n = 9 and left, n = 8). One patient had already been operated on with EETSS, 1.5 years back from current surgery. Sixteen patients were on hormonal support, preoperatively. Four patients died during follow-up. Post-operative common complications were diabetes insipidus (DI, n = 18), cerebrospinal fluid rhinorrhea (n = 10), surgical site hematoma (n = 3), meningitis (n = 3), hydrocephalus (n = 2), and syndrome of inappropriate antidiuretic hormone (n = 1). The mean hospital stay was 11.62 days and 12.17 months were the mean follow-up period. At 12 POW, no improvement was seen in body enlargement and visual complaints, but all other complaints improved significantly except perspiration. Adenomas were decreased in all extensions except posterior and mean adenoma volume was reduced from 16.34 cm3 to 2.92 cm3 after surgery. Sub-total resection (STR, n = 10), near-total resection (NTR, n = 7), gross-total resection (GTR, n = 5), and partial resection (PR, n = 2) were achieved. Endocrinological remission and prognostically critical GH levels were attained in 29.17% (n = 7) and 66.67% (n = 16), respectively. NTR, GTR, STR, and PR were associated with 57.14%, 40%, 10%, and 0% endocrinological remission, respectively. Additional treatment was required in a total of 17 patients, three in GTR, nine in STR, three in NTR, and two in PR. Ten were treated with Gamma Knife radiosurgery along with medical treatment and seven with medical treatment alone. A successful EETSS can reduce adenoma volume to achieve clinical improvement, endocrinologic remission, and prognostically critical GH level with some complications related to surgery. Pre-operative larger volume and higher extension grades affect these outcomes adversely.

A retrospective cohort study assessing geometrical parameters related to visual deterioration in pituitary macroadenoma

PurposeWe aim to identify specific parameters that will allow identification of those patients at greater risk of vision loss, specifically variables such as geometry, volume and size of the pituitary tumour. MethodologyVolume measurements were made with both manual (using the formula width × height × length × 0.5) and video size, sub-voxel interpolation using Brainlab software. The shape of the tumour was divided into four categories, ovoid, dumbbell, pyramidal and other. Chiasmal compression was divided into central and lateral compression and the chiasm location was classified based on pre/post and normal fixed chiasm in relation to the tuberculum sella. We used a visual impairment score ranging from (0–100), which was then further subdivided in to four grades: Grade I – 0–25 (mild); Grade II – 26–50 (moderate); Grade III – 51–75 (severe); Grade IV – 76–100 (subtotal or complete). ResultsUnivariate analysis of variables related to vision loss found tumour volume, suprasellar growth and lateral chiasmal compression related to pre-operative vision loss. On multivariate regression analysis tumour volume and lateral chiasmal compression remained significant (p < 0.008). In relation to post-operative vision, univariate analysis indicated that variables such as giant macroadenoma, pre-operative visual impairment score and suprasellar growth were significant. Multivariate analysis showed pre-operative visual impairment and suprasellar growth as the only statistically significant variables. ConclusionOur ROC analysis suggests tumours with suprasellar extension 16 mm (sensitivity 85 %, specificity 67 %) in sagittal plane are more likely to result in moderate to severe grade vision loss (VIS grade ≥ 2).

The HACKD Score-Predicting Extent of Resection of Pituitary Macroadenomas Through an Endoscopic Endonasal Transsphenoidal Approach.

Predicting extent of resection before pituitary surgery is imperative for operative planning and patient counseling. In the era of endoscopic endonasal transsphenoidal surgery (EEA), a specific, reliable tool for predicting resection of macroadenomas would have widespread applications. To identify factors that predict resection of pituitary macroadenomas through the EEA. A prospectively maintained, multicenter database of 277 consecutive macroadenomas resected through the EEA was analyzed. Multivariate logistic regression identified predictors of gross total resection (GTR). A simple scoring system, the Hardy, Age, Clival, Knosp, Depth (HACKD) Score, was developed and tested. GTR was achieved in 82.3% (228/277) of cases. Older than 50 years (odds ratio [OR] 2.96, P = .01), clival extension (OR 5.87, P < .01), Hardy grade C or D suprasellar extension (OR 3.91, P = .01), Knosp grade 3 or 4 cavernous sinus invasion (OR 7.62, P < .01), and tumor depth >20 mm (OR 5.14, P < .01) were all associated with subtotal resection. The HACKD score, awarding 1 point each for Hardy grade C or D, and older than 50 years, and 2 points each for clival extension, Knosp grades 3 or 4, and tumor depth >20 mm, demonstrated excellent discriminative ability (AUROC 0.887, 95% CI: 0.839-0.934). The rate of GTR progressively decreased with a higher HACKD score. Rates of GTR were 95.8% (182/190) for low (HACKD ≤2), 59.5% (44/74) for moderate (HACKD 3-5), and 15.4% (2/13) for high (HACKD 6+) HACKD scores. The HACKD score is a simple and accurate tool based on the largest study analyzing predictors of GTR in pituitary macroadenomas operated through the EEA.

LBODP076 Hypophysitis Misdiagnosed As Pituitary Macroadenoma During Pregnancy

Abstract Introduction Surgical treatment of nonfunctioning symptomatic pituitary macroadenoma may be associated with significant morbidity and the need for long-term medications. In late pregnancy and the postpartum periods, lymphocytic hypophysitis can present as a pituitary macroadenoma. We present a rare case of a pregnant patient who underwent pituitary surgery for lymphocytic hypophysitis. Case presentation A 37-year-old woman was evaluated for headaches and double vision during the third trimester of her pregnancy. A visual field exam showed bitemporal hemianopsia. Brain magnetic resonance imaging (MRI) showed a 2 cm intrasellar tumor with suprasellar extension, interpreted as a pituitary macroadenoma. The patient underwent a cesarean section. Labs after delivery showed IGF-1: 153 ng/ml (79-259), LH: &amp;lt; 0.2 mIU/ml (1.9-12.8), FSH: 6.4 mIU/ ml (2.3-12.6), Prolactin: 4.3 ng/ml (2.2-30.3), and a low morning cortisol: 2.4 ug/dl (4.2- 22.4). Pituitary MRI with intravenous contrast found a stable pituitary macroadenoma with suprasellar extension compressing the optic chiasm and prechiasmatic optic nerves without evidence of apoplexy. She underwent image-guided endoscopic transsphenoidal resection of the pituitary mass. Postoperatively, she developed diabetes insipidus that was treated with desmopressin. She was treated with steroids for secondary adrenal insufficiency and levothyroxine for central hypothyroidism. The pathology report showed hypophysitis; a lymphoplasmacytic infiltrate identified within the specimen with increased IgG4 expression. Differential diagnoses included IgG4-related hypophysitis and lymphocytic hypophysitis with an IgG4-expressing plasma cell component. Serum IGG 4 was 62 mg/dl (normal: 2 - 96 mg/dl); however, patient was already on steroids. She has been requiring hydrocortisone, levothyroxine, and desmopressin. Discussion Lymphocytic hypophysitis often occurs in late pregnancy or the postpartum period. MRI usually shows a pituitary mass, mimicking an adenoma. However, differences between lymphocytic hypophysitis and pituitary adenoma, using different imaging techniques, have been described. Lymphocytic hypophysitis should be considered in the differential diagnosis of women presenting with pituitary enlargement in the peripartum period. These patients may benefit from conservative treatment with glucocorticoids based on clinical suspicion. Presentation: No date and time listed

PMON109 A Case of Non-functioning Pituitary Adenoma With Unexpectedly High Prolactin Level

Abstract Introduction Lactotroph or prolactin (PRL)-secreting adenomas account for approximately 45% of all pituitary adenomas. They are classified by size as microprolactinomas (&amp;lt;10 mm) and macroprolactinomas (≥10 mm). A giant prolactinoma is arbitrarily defined as an adenoma &amp;gt; 4 cm. PRL levels are often proportional to the size of tumor ranging from &amp;lt;200 ng/mL in adenomas smaller than 1 cm, 200 ng/mL to 1000 ng/mL in 1 cm to 2 cm adenomas and more than 1000 ng/ml in tumors larger than 2 cm. Nonfunctioning pituitary adenomas (NFPA) can cause mild increases in PRL levels due to pituitary stalk compression, however, levels more than 6 times the upper limit of normal have not been reported. We present a rare case of NFPA with significantly elevated prolactin levels Case: 42 yo female presented with headache and left eye blurry vision worsening over 2 years. MRI revealed a pituitary macroadenoma measuring 4.1×4.7×3cm with supra-sellar extension, mass effect including severe compression and displacement of the optic apparatus, and potential cavernous sinus invasion. Initial biochemical profile: TSH 1.01 (0.30–4.20 uU/mL), FT4 0.9 (0.6–1.5 ng/dL), AM ACTH of 20 (7.2-20 pg/ml), peak cortisol 18.8 mg/dl after cosyntropin stimulation, FSH 14.6 mIU/mL, LH 2.4 mIU/mL, IGF-1 71 (52-328 ng/mL) and prolactin 452 (&amp;lt;25.0 ng/mL), confirmed by dilution. PRL levels were concerning for prolactinoma but did not correlate with tumor size making giant prolactinoma an unlikely diagnosis. Yet, levels were higher than those expected from stalk compression. An interdisciplinary decision was made to proceed with operative management. The patient underwent trans-sphenoidal pituitary resection (TSPR). Tumor pathology showed sparsely granulated gonadotroph adenoma with weak LH expression and negative PRL staining, confirming NFPA. PRL level one-month post op was 33.2 ng/mL and vision is improved. Conclusion Medical treatment with dopamine agonists is first line treatment for any size prolactinoma with a high remission rate of 90% and prescribed before TSPR even in those with vision disturbance or neurologic symptoms. Giant NFPAs with compressive symptoms are best surgically managed.To our knowledge, this is the first report of significant PRL elevation due to stalk compression. Not only was the PRL higher than that previously associated with stalk compression, but also well above 250 ng/mL, which is typically diagnostic of prolactinoma. This case also highlights the importance of assessing the relationship between PRL level and tumor size to determine the correct course of management. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

Intraoperative Prediction of Long-Term Remission in Acromegaly.

The features of long-term remission in acromegaly adenomectomy are incompletely understood. An intraoperative predictor for long-term outcome would be valuable for assessing resection of growth hormone (GH)-secreting tumors in real-time. To evaluate whether intraoperative GH measurement could predict long-term outcomes for acromegaly. In 47 patients, peripheral blood GH levels were measured thrice intraoperatively: once before tumor dissection, once during tumor dissection, and once after tumor dissection. Long-term remission was defined by age-appropriate, normalized insulin-like growth factor-1 at most recent follow-up and a random GH less than 1.0 ng/mL. Patients were only considered to be in long-term remission without the use of postoperative medical therapy for acromegaly or radiation therapy. The median length of follow-up was 4.51 (range: 0.78-9.80) years. Long-term remission was achieved in 61.7% (29/47) of operations. Like previous studies, cavernous sinus invasion (odds ratio [OR]: 0.060; 95% CI: 0.014-0.260; P value < .01), suprasellar extension (OR: 0.191; 95% CI: 0.053-0.681; P value<.01), and tumor size greater than 1 cm (OR: 0.177; 95% CI: 0.003-0.917; P value = .03) were associated with not being in long-term remission. The minimum GH measured intraoperatively predicted long-term outcome (area under the curve: 0.7107; 95% CI: 0.537-0.884; P value < .01). The odds ratio of remission in patients with the lowest quartile minimum intraoperative GH compared with patients with the highest quartile minimum intraoperative GH was 27.0 (95% CI: 2.343-311.171; P value < .01). Minimum intraoperative GH may predict long-term outcome for acromegaly, which in principle could provide the pituitary neurosurgeon with real-time feedback and inform intraoperative decision making.

PMON104 Sellar Gangliocytoma-Pituitary Neuroendocrine Tumor (PitNET) Presenting As Acromegaly

Abstract Growth hormone (GH)-secreting pituitary neuroendocrine tumors (PitNET) arising from somatotrophs is the most common cause of acromegaly. It may rarely develop in the setting of a growth hormone-secreting PitNET associated with a neuronal tumor composed of neoplastic ganglion cells, the so-called mixed gangliocytoma somatotroph PitNET. We present a case of acromegaly which proved to be due to a mixed gangliocytoma somatotroph PitNET on pathology.A 58-year-old, healthy woman presented to the ER with a 3-day history of left-sided vision loss and worsening headache. MRI revealed 4.5×4.2×3.8 cm heterogeneously enhancing bilobed mass consistent with a pituitary PitNET with suprasellar extension causing compression of the optic chiasm, invasion into the left cavernous sinus and partial encasement of the left internal carotid artery. She had coarse facial features, wide feet, multiple skin tags, and a history of multiple colonic polyps. Biochemical work up revealed an IGF-I of 664 ng/ml (50-317), AM cortisol 19.7 mg/dl, ACTH 30 pg/ml, prolactin 15.5 ng/ml, FSH 35.6 mIU/ml, LH 11.3 mIU/ml, and FT4 0.7 ng/dl (0.70-1.48). She underwent endoscopic transsphenoidal resection of the tumor. Histopathology demonstrated mature ganglion cells with neuropil-like areas, intermixed with small cell components. The neuronal population was immunopositive for NeuN (Neuronal nuclei antibody), synaptophysin, and NF (Neurofilament). PitNET cells were positive for GH, prolactin, Pit- 1 transcription factor and the somatostatin 5 receptor, histological features consistent with mixed gangliocytoma and sparsely granulated somatotroph pitNET. Some ganglion cells also showed weak immunostaining with Pit-1. A 3-month follow-up MRI showed a residual 3 cm lesion. IGF-I improved but was still elevated at 456 ng/ml associated with a GH 2.9 ng/ml. Medical treatment with pasireotide was recommended, but the patient temporarily declined further treatment to explore other options with her Naturopath.Gangliocytomas are a rare, benign, slow-growing, differentiated form of neuroblastic tumors of sympathetic nerve fibers, largely extracranial and rarely present in the sellar region, most frequently in association with a PitNET, forming the so-called mixed gangliocytoma PitNET. Definitive diagnosis is established by histological and immunohistochemical studies. Though PitNET components of mixed gangliocytoma PitNET are known to frequently secrete an excess hormone, the ganglionic components also show immunoreactivity for hypothalamic releasing hormones along with other neuroendocrine markers. The histological origin of these mixed tumors is controversial, but the small population of ganglionic cells that express the acidophilic lineage transcription factor, Pit-1, favors the theory of transdedifferentiation of neuroendocrine cells into neuronal elements. Treatment of mixed gangliocytoma- PitNET is similar to other PitNETs and the presence of neural components does not modify aggressiveness or risk of recurrence after surgical resection, as gangliocytomas have a low proliferative index. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

LBMON173 Non-functioning Seller Masses: Tumor Types, Surgical Management And Course Over Time

Abstract Introduction Pituitary adenomas (PA) are common. However, not all sellar tumors are PA. The specific diagnosis of a sellar mass can be difficult. Although there is abundant literature on PA, other types of sellar masses are not well described. In particular, their course over time and response to therapy are not well known. In this study, our objectives were to review non-functioning sellar masses treated with trans sphenoidal surgery (TSS) over the last 15 years to understand their types, success of surgery, and course over time. Patients and Methods We retrospectively reviewed all cases of non-functioning sellar masses managed at our hospital over the last 15 years (January 2007-December 2021). Out of 181 sellar masses that were resected by TSS during this period, 91 were functional PA and 90 were non-functional sellar masses. These sellar masses included non-functioning pituitary adenomas and other non-pituitary seller masses operated at our center during the above period and are the subject of this study. Results The patients included 29 (32%) females and 61 (68%) males with a median age of 41 years (range 13-75 years). The sellar masses were non-functional PA in 50 (55.6%)cases, atypical PA in 30 (33.3%) cases and non-PA in 10 (11%) cases. The 10 non-PA tumors included craniopharyngioma 3 cases, meningioma 3 cases, chordoma 1 case, giant cell tumor of the bone 1 case, spindle cell oncocytoma 1 case and Rathke cyst 1 case. The median largest tumor diameter was 3.15 cm (Interquartile range 2.2-4. 0). Only 5 cases (5.6%) were intrasellar, the other 85 cases (94.4%) had suprasellar (75 cases, 83%) and/or parasellar (32 cases, 35.6%) extension. Necrosis/cystic changes on MRI were present in 24 cases (26.7%). TSS was performed in 86 cases (95.6%) and transcranial surgery in 4 (4.4%) cases. The surgeries were complicated by transient central DI in 21 cases (23.3%), permanent CDI in 6 cases (6.7%), CSF leak in 8 cases (9%), bleeding in 2 (2.2%), stroke in 3 (3.3%) and partial or panhypopituitarism in 58 cases (64.4%). Twenty-two (24.4%) patients underwent a second TSS, 6 (6.7%) transcranial surgery, 32 (35.6%) external radiotherapy. At a median follow-up of 8.5 years (IQ range 4.63-12.18), only 19 (21%) patients had no evidence of residual tumor on follow-up MRI. The other 71 (79%) cases continued to have evidence of residual tumor, either completely intrasellar in 30 (33.3%) cases or with the suprasellar or parasellar residual component in 41 (45.6%) cases. Conclusions Non-functioning PA and other non-pituitary sellar tumors constitute 50% of sellar masses. Most are non-functioning PA, but atypical PA and other non-PA tumors can masquerade as PA. Most of these Non-functioning sellar masses are large with supra and parasellar extension. Surgery effectively reduces tumor size but is rarely curative and frequently complicated by hypopituitarism of variable severity and local complications. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

ODP298 Are there identifiable differences in pituitary adenoma width, extension, invasion, or cystic degeneration in subclinical compared to clinical pituitary apoplexy patients?

Abstract Background Pituitary apoplexy [PA] is defined as an infarction, haemorrhage, or both within the pituitary gland, and can be life threatening. (Onesti 1990) Typically, it presents with sudden onset headache, visual changes, loss of consciousness and potentially life-threatening panhypopituitarism. If so, it is defined as a 'clinical' PA. In absence of these symptoms, it is defined as a 'subclinical' PA. (Kim 2009) Subclinical PA occurs more frequently than clinical PA. (Kim 2009; Möller-Goede 2011; Liu 2010; Cinar 2013) The rationale for this is still unclear. Tumour size, site of invasion, and cystic degeneration are all risk factors for apoplexy. (Biousse 2001;Boellis 2014)It is important to understand whether these factors differ between these groups to understand why subclinical apoplexy is more common, and to improve prevention and treatment. Aim To identify whether underlying pituitary adenoma tumour size (width mm), site of invasion, or presence of cystic degeneration differed between clinical and subclinical populations with pituitary apoplexy (PA). Study design: This is a quantitative retrospective cohort study of patients attending Macquarie University Endocrinology Clinic and Macquarie Medical Imaging between 2011-2021. Methods Clinical and subclinical patients were divided based on the acuity of their presentation (clinical = sudden onset headache, nausea, vomiting, visual changes, subclinical = gradual onset, or lack of symptoms). Adenomas &amp;gt; 10mm were classified as macroadenomas and &amp;lt;10mm were microadenomas. Age, gender, presence of suprasellar extension, intrasellar invasion, lateral invasion, and cystic change were identified from neurosurgeon and neuroradiologist cross-reporting on MRI. Results 18 patients were included in the study aged between 19 years to 85 years with 13 females and 5 males. 55.6% presented subclinical and 44.4% clinically. 15 (83%) had macroadenomas and 3 (16%) had microadenomas. There were no significant differences in age, gender, width of adenoma, site of invasion, or presence of cystic degeneration between the two cohorts. However, the data supported previous literature which suggests that clinical pituitary apoplexy patients tend to have marginally larger adenomas and are more commonly associated with cystic degeneration. Conclusions Overall, this study highlights potential differences in tumour size and presence of cystic degeneration between subclinical and clinical apoplexy patients. However, further research is required with a larger cohort to determine the significance of these differences. The critical demarcation of the underlying pathology between patients experiencing subclinical versus clinical PA is not yet clear in the literature to date. When haemorrhage is secondary to an underlying lesion, regrowth of the pituitary tumour years after an episode may occur, and patients may require long-term clinical and imaging surveillance. Therefore, the importance of these research findings is that it may allow for increased predictability and prevention of pituitary apoplexy, which can be a life-threatening event. Presentation: No date and time listed

Surgical outcomes and longitudinal quality of life after endoscopic endonasal surgery for anterior skull base meningioma.

The objective of this study was to establish the effect of endoscopic endonasal surgery on longitudinal quality of life (QOL) in patients with anterior skull base meningioma. A prospectively collected cohort of consecutively operated anterior skull base meningiomas was analyzed. All cases were performed using the endoscopic endonasal approach (EEA). Sinonasal-specific and overall QOL were measured using the 22-Item Sinonasal Outcome Test and the Anterior Skull Base Questionnaire longitudinally (at 3 weeks, 6 weeks, and 3, 6, and 12 months) across the first postoperative year. The relationship between surgical and QOL outcomes to clinical and radiological characteristics was analyzed using multivariate regression. Fifty cases were available, with a mean age of 61.5 years and female predominance (74.0%). Visual dysfunction and headache were the most common presenting symptoms, and tumors commonly took origin from the planum (46.0%), tuberculum (44.0%), and olfactory groove (24.0%). Median tumor volume was 4.6 cm3. Visual improvement was noted in 73.1% of cases with preoperative field deficits, while nonimprovement was associated with greater tumor height (p = 0.04). Gross-total resection was not possible in patients with 360° vessel encasement and high-grade cavernous sinus extension with ophthalmoplegia. Postoperative diabetes insipidus was observed only in cases with suprasellar extension. Sinonasal-specific QOL worsened transiently after surgery but returned to baseline levels after 3 weeks. Olfaction and taste scores returned to preoperative baseline scores within the year. Overall QOL at presentation was worse in those with larger tumors (p = 0.04) and visual failure (p = 0.04) and better in those presenting with headache (p = 0.04). Transient worsening of QOL was seen in the first 3 weeks, which returned to baseline by 6 weeks, and then improved to above preoperative levels at 6 months and beyond. Worse QOL at baseline (p = 0.01) and visual improvement (p = 0.01) predicted QOL improvement after surgery. Longitudinal QOL in anterior skull base meningioma has been examined for the first time. Endoscopic endonasal surgery improves overall QOL after a transient 3-week worsening due to the sinonasal morbidity of the approach. Visual function is intimately tied to QOL, with worse vision associated with worse preoperative QOL, and QOL improving in parallel with visual restoration after surgery. The EEA is associated with better visual outcomes and should be the preferred approach in accessible tumors.

Risk factors and management associated with postoperative cerebrospinal fluid leak after endoscopic endonasal surgery for pituitary adenoma.

ObjectiveTo determine risk factors and management for the development of a postoperative cerebrospinal fluid (CSF) leak after an endoscopic endonasal surgery (EES) for pituitary adenomas.MethodsThe clinical data of 400 patients who underwent EES for resection of pituitary adenomas from December 2018 to November 2019 in the Department of Neurosurgery of Beijing Tiantan Hospital were retrospectively reviewed. Age, gender, body mass index (BMI), tumor size, Knosp grade, suprasellar extension grade, sellar floor erosion grade, repeated transsphenoidal surgery, intraoperative CSF leak, use of pedicled nasoseptal flap and lumbar drain were collected and analyzed.ResultsPostoperative CSF leak occurred in 14 of 400 patients (3.5%). Age, gender, BMI, tumor size, Knosp grade and repeated transsphenoidal surgery were not risk factors for CSF leak. Suprasellar extension grade (≥B 6.0% vs. <B 1.4%; p = 0.024), sellar floor erosion grade (≥III 5.7% vs. <III 0.6%; p = 0.020) and intraoperative CSF leak (Yes 7.5% vs. No 2.0%; p = 0.009) were factors associated with an increased postoperative CSF leak rate.ConclusionsHigher suprasellar extension grade, higher sellar floor erosion grade and intraoperative CSF leak were risk factors for postoperative CSF leak after endoscopic treatment of pituitary adenoma. Strict skull base reconstruction including use of a pedicled nasoseptal flap and perioperative lumbar drainage may avoid postoperative CSF leak.

Dumbbell-shaped pituitary adenomas: prognostic factors for prediction of tumor nondescent of the supradiaphragmal component from a multicenter series.

Dumbbell-shaped pituitary adenomas (DSPAs) are a subgroup of macroadenomas with suprasellar extension that are characterized by a smaller diameter at the level of the diaphragma sellae opening compared with the supradiaphragmal tumor component (SDTC). Hence, DSPAs may be particularly prone to a nondescending suprasellar tumor component and risk for residual tumor or postoperative bleeding. A multicenter retrospective cohort analysis of 99 patients with DSPA operated on via direct endoscopic endonasal transsphenoidal approach between 2011 and 2020 was conducted. Patient recruitment was performed at two tertiary care centers (Medical University of Vienna and University of Southern California) with expertise in endoscopic skull base surgery. DSPA was defined as having a smaller diameter at the level of the diaphragma sellae compared with the SDTC. On preoperative MRI, all DSPAs were macroadenomas (maximum diameter range 17-71 mm, volume range 2-88 cm3). Tumor descent was found in 73 (74%) of 99 patients (group A), and nondescent in 26 (26%) of 99 patients (group B) intraoperatively. DSPAs in group A had a significantly smaller diameter (30 vs 42 mm, p < 0.001) and significantly smaller volume (10 vs 22 cm3, p < 0.001) than those in group B. The ratio of the minimum area at the level of the diaphragmal opening in comparison with the maximum area of the suprasellar tumor component ("neck-to-dome area") was significantly lower in group A than in group B (1.7 vs 2.7, p < 0.001). Receiver operating characteristic curve analysis revealed an area under the curve of 0.75 (95% CI 0.63-0.87). At a cutoff ratio of 1.9, the sensitivity and specificity for a nondescending suprasellar tumor component were 77% and 34%, respectively. In the present study, the neck-to-dome area ratio was of prognostic value for prediction of intraoperative tumor nondescent in DSPAs operated on via a direct endonasal endoscopic approach. Pituitary adenoma SDTC nondescent carried the inherent risk of hemorrhagic transformation in all cases.