Sellar Lesions Research Articles

Magnetic resonance imaging characteristics and differential diagnosis of common sellar cystic lesions

Objective: To investigate magnetic resonance imaging (MRI) characteristic and differential diagnostic keypoints of common sellar regional cystic lesions with the purpose of improving differential diagnostic accuracy. Methods: In total, 174 cases of the pathologically diagnosed cystic lesions in sellar region between March 2016 and June 2019 were included in the current retrospective analysis. These cases included Rathke's cleft cyst (n=68), craniopharyngioma (n=48), pituitary adenoma (n=56) and arachnoid cyst (n=2) in the sellar region. The position, texture and morphology of leisions, signal of cystic and solid part, invasion of surrounding tissues and maximum diameter of cysts on the MRI images were evaluated and compared between different groups. SPSS 25.0 software was used for statistical analysis. Results: There were several specific changes on several MRI sequences in Rathke's cleft cysts, craniopharyngioma and pituitary adenoma groups. For Rathke's cleft cysts, round shape, rare polycystic structure, lack of solid part and maximum diameter of cysts which was significantly less than that of craniopharyngioma and pituitary adenoma groups ((17.37±6.12) mm vs (30.29±13.51) mm vs (28.18±11.13) mm, t value was 6.680, 5.838, respectively, all P<0.05), were favorable diagnostic criteria. While, the intracystic nodules were mostly found in craniopharyngioma. There was no high signal intensity in cystic wall on T1WI and T2WI of pituitary adenoma. MRI signal of arachnoid cyst in sellar region was basically the same as CSF signal, with low signal intensity on T1WI and DWI, high signal intensity on T2WI, without enhanced signal. Conclusions: Common sellar regional cystic lesions showed characteristic manifestations on different MRI sequences. Valuable information in the morphology, MRI signal intensity, maximum diameter measurements may improve accuracy of differential diagnosis for sellar regional cystic lesions.

Resection of Pituitary Tumor with Lateral Extension to the Temporal Fossa: The Toothpaste Extrusion Technique.

Transsphenoidal resection of the sellar and suprasellar lesions, whether microscopic or endoscopic, has been traditionally limited by tumors extending laterally to the carotid artery and cavernous sinus. Extended endoscopic or transmaxillary approaches may be warranted depending on these tumor extensions. We describe the use of an intraoperative Valsalva maneuver as a surgical adjunct to the transsphenoidal approach to improve the extent of resection for a favorable outcome. The patient was a 65-year-old woman who underwent resection of a giant pituitary tumor that extended laterally to the cavernous sinus to occupy a volume within the middle fossa. It was the senior author's impression that the lateral cavernous wall was intact at the time of surgery although this is difficult to determine definitively. After a transsphenoidal intrasellar resection of the intrasellar tumor, side-angled endoscopic visualization enabled identification of the breach in the medial cavernous wall where the tumor had invaded the cavernous sinus and ultimately grown into the middle fossa. A Valsalva maneuver was then applied, and the tumor was extruded from the cavernous sinus lateral to the carotid. The significant tumor was removed under direct visualization of the abducens nerve, which was well preserved. Postoperative imaging showed a sufficient extent of resection, and there were no postoperative complications. An intraoperative Valsalva maneuver can be a potentially useful technique for extending tumor resection in cases with a soft tumor and visualization of the opening within the cavernous sinus wall.

Pituitary apoplexy: A rare cause of acute isolated third nerve palsy

Isolated third cranial nerve palsies are most commonly due to microvascular ischemia in adults with atherosclerotic risk factors. Though rare, pituitary apoplexy causing ocular mononeuropathies must be recognized for prompt medical and surgical management. We highlight, with images, a 55-year old man with poorly controlled diabetes, who presents with a 3-day history of diplopia, right eye pain and ptosis. On examination, he had a third cranial nerve palsy, with a dilated pupil. Magnetic resonance imaging showed an enhancing mass in the sellar region extending laterally into the right cavernous sinus with a radiological suggestion of blood in the right cavernous sinus. Magnetic resonance angiogram showed no aneurysm, and there was no evidence of infection or hemorrhage on lumbar puncture. Hormonal testing revealed mild cortisol deficiency, but was otherwise unremarkable. Our patient was diagnosed with pituitary apoplexy. He initially elected for conservative management and was started on hydrocortisone replacement. Repeated neuroimaging 3 months’ later for non-resolving symptoms showed a persistent right sellar lesion. Endonasal endoscopic transphenoidal resection of the pituitary mass was then performed, resulting in gradual improvement in his third nerve palsy. Histology showed fibrous tissue with hemosiderin deposition, with no evidence of infection or malignancy. Our case highlights the importance of recognizing pituitary apoplexy as a rare cause of isolated third nerve palsy, even in patients with atherosclerotic risk factors. Surgical management can be considered to aid resolution of neurological complications.

A Study of Histopathological Spectrum of Sellar, Suprasellar and Parasellar Lesions of CNS at Tertiary Care Centre

A Study of Histopathological Spectrum of Sellar, Suprasellar and Parasellar Lesions of CNS at Tertiary Care Centre

Two Synchronous Pituitary Adenomas Causing Cushing Disease and Acromegaly

To report the first case of 2 synchronous pituitary adenomas, 1 corticotroph and 1 somatotroph, with distinct molecular lineages confirmed by differential hormone and S-100 protein expression. A case report followed by a literature review are presented. A 68-year-old woman presented for evaluation of resistant hypertension. Biochemical testing demonstrated adrenocorticotropic hormone (ACTH)-dependent hypercortisolemia and growth hormone (GH) excess. Pituitary magnetic resonance imaging (MRI) revealed a 2 cm left sellar lesion consistent with a pituitary macroadenoma. The patient therefore underwent transsphenoidal surgery for a presumed cosecreting ACTH and GH macroadenoma. Tumor immunohistochemical staining (IHC) was positive for ACTH, but negative for GH. Postoperative biochemical testing confirmed remission from Cushing disease, but the insulin-like growth factor 1 (IGF-1) level remained elevated. Postoperative MRI demonstrated a small right sellar lesion that, in retrospect, had been present on the preoperative MRI. Resection of the right lesion confirmed a GH-secreting adenoma with negative ACTH staining. After the second surgery, the IGF-1 level normalized and blood pressure improved. Further pathologic examination of both surgical specimens demonstrated differential expression of S-100 protein, a folliculostellate cell marker. Reverse transcription polymerase chain reaction of messenger ribonucleic acid from the left sellar lesion was positive for ACTH and negative for GH, confirming the IHC results. Germline mutations in genes known to be associated with pituitary adenoma syndromes (MEN1, CDC73, CDKN1A, CDKN1B, CDKN2B, CDKN2C, and AIP) were not detected. Although the pathogenesis of synchronous pituitary adenomas has not been fully elucidated, this case report suggests that they can have distinct molecular lineages.

Stepwise Algorithm for Skull Base Reconstruction in Endoscopic Endonasal Surgery: From the Simple to the Complex

Abstract INTRODUCTION The most frequent complication of endoscopic endonasal surgery (EES) is postoperative cerebrospinal fluid (CSF) leak. This study was designed to develop a step-wise algorithm for EES reconstruction across the spectrum of skull base defects: from free mucosal graft for uncomplicated pituitary adenomas to free flaps in complex cases with recurrent leaks. METHODS All patients with skull base pathologies who underwent EES between January 2017 and December 2018 were included and retrospectively analyzed. Tumor location, reconstruction method and postoperative CSF leak were reviewed and a step-wise algorithm based on size and location of defect was developed. RESULTS Location of skull base defects was categorized as follows: anterior fossa, suprasellar, sellar and posterior fossa. For all nonsellar sites, we performed a multilayer (collagen matrix + /- fascia lata + /− fat graft + vascularized flap) reconstruction. The nasoseptal flap (NSF) was the first choice for vascularized reconstruction when available. For all sellar lesions we employed a free mucosal graft unless a high-flow CSF leak was present, in which case a single-layer reconstruction with NSF was performed. When the NSF was not available, alternative local (lateral nasal wall flap) and regional (extracranial pericranial flap) pedicled flaps were successful choices. When patients failed multiple attempts at repair, regional or microvascular free flaps were options. Lumbar spinal drainage was employed for large anterior and posterior fossa defects and during secondary repair of postoperative CSF leaks. Of 347 patients, 4.6% had a postoperative CSF leak. Of 158 patients with an intraoperative leak (45.5%), 10.1% developed a postoperative CSF leak: 7.8% for sellar/suprasellar defects and 13% for anterior/posterior fossa defects. CONCLUSION This algorithm provides a standardized, stepwise approach to the reconstruction of all skull base defects after EES based on location.

Pituitary abscess following endoscopic endonasal drainage of a suprasellar arachnoid cyst: Case report and review of the literature

BackgroundArachnoid cysts are uncommon lesions of the sellar region, and only rarely require decompressive surgery to treat symptoms. Pituitary abscesses are another rare lesion, and are an uncommon complication of pituitary surgery. A previously healthy 45 year old woman presented with a new finding of bitemporal hemianopsia. Magnetic resonance imaging (MRI) showed a cystic sellar lesion with suprasellar extension and compression of the optic chiasm. Endoscopic transphenoidal surgery was performed and the lesion was found to be an arachnoid cyst. She was well immediately after the operation, but 1 week later presented with headaches, fever and worsening visual acuity. MRI showed a homogenous collection in the sellar region that was compressing the chiasm. The patient was treated with antibiotics, and a second transphenoidal operation was performed, with frank pus found in the pituitary fossa. Cultures of the fluid found during the operation grew Escherichia coli and Staphylococcus lugdunensis. Her symptoms resolved after the second operation, and formal visual fields and a pituitary hormone panel were normal at this time. We report on a rare case of a pituitary abscess complicating surgery for a sellar arachnoid cyst, and discuss management of these conditions.

Pituitary gland tumors

In this article, the most common pituitary gland tumors and the various differential diagnoses with focus on (neuro-)radiological diagnostic criteria are presented. An intensive, selective search of the literature in PubMed was carried out. Pituitary adenomas account for approximately 10-15% of all intracranial brain tumors and are the most common tumors of the sellar region. Beyond asize of 10 mm they are called macroadenomas, under 10 mm microadenomas. They can be distinguished into hormone-active and non-active adenomas. Most of the hormone-active adenomas secrete prolactin (50%), more rarely somatotropin (10%) or corticotropin (5%). Tumors in the sellar region can arise from various tissues. Due to the anatomically complex location, local adjacent structures can be affected or compressed by the tumors. Particularly in case of suprasellar extension, visual impairment due to pressure on the optic chiasm is common. Important differential diagnoses for sellar tumors include craniopharyngiomas, meningiomas, metastases, aneurysms and Rathke cleft cysts. The task of image diagnostics is the early detection of the lesions as well as the proliferation pattern into perifocal structures. Gold standard is the thin-section, contrast-enhanced MRI examination. Dynamic contrast administration is crucial for the diagnosis of the microadenoma and the specific enhancement characteristic of some other tumors. Ahighly focused imaging protocol is important for the diagnosis of sellar lesions such as pituitary tumors. The current favored modality is contrast-enhanced MRI, preferably with dynamic contrast-enhanced T1-weighted sequences. Early detection of the lesions and identification of the precise anatomical location are of great importance for diagnosis and therapy.

Single-fraction stereotactic radiosurgery for spindle cell oncocytoma: preliminary experience and systematic review of the literature.

Spindle cell oncocytoma (SCO) is a rare benign pituitary tumor. No patient series regarding stereotactic radiosurgery (SRS) for SCO has been published. We report the clinical outcomes of SCO treated with single-fraction SRS, as well as a systematic review of the literature. Retrospective cohort series and systematic literature review. Five patients (four male, one female) having single-fraction SRS for persistent or recurrent SCO between 2002 and 2018. Median age was 56 (range 54-79) years. Pre-SRS treatments included transsphenoidal resection (TSR) (n = 3), multiple TSR (n = 1), and TSR, radiotherapy, and craniotomy (n = 1). Median target volume was 4.7 (range 1.8-8.4) cm3, with a median tumor margin dose of 17 (range 14-20) Gy. Median follow-up was 24 (range 10-69) months. All radiation-naïve patients achieved tumor control after SRS; tumor progression was noted 24months after SRS in one patient who failed prior radiotherapy. No radiation-induced complications were observed after SRS. Systematic literature review of 43 cases in addition to the five cases presented here showed that tumor progression/recurrence was more frequent after STR compared to GTR (P < 0.001). Ten previous cases of radiotherapy for SCO have been reported, but most did not detail radiation volumes, doses, or outcomes. SCO are uncommon sellar lesions with a propensity for progression or recurrence. Based on the clinically aggressive course of these tumors, adjuvant SRS after STR or at the time of tumor recurrence should be considered. Further case accumulation and follow-up is required to better understand the long-term treatment outcomes after single-fraction SRS for these rare tumors.

Perioperative multidisciplinary management of endoscopic transsphenoidal surgery for sellar lesions: practical suggestions from the Padova model.

Perioperative management of patients with sellar lesion submitted to endoscopic transsphenoidal neurosurgery (TSS) lacks standardization and therefore it is committed to each center clinical practice. Although neurosurgical procedure remains the same for all sellar lesions, perioperative approach can require different measures depending on the underlying disease. With the aim of standardizing our perioperative procedures and sharing our experience with other centers involved in the management of pituitary disease, we developed a clinical care path for patients with sellar lesions candidate to endoscopic TSS. For the drafting of the following protocol, the national and international guidelines published in the last 5years have been evaluated and integrated with our center experience accumulated in decades of clinical practice. A steering committee including medical doctors involved in management of patients with pituitary masses at the Padua Hospital reviewed current knowledge on this topic. The committee developed a first draft which was shared with a broader group of medical doctors to reach a preliminary consensus; when it was reached, the clinical care assistance pathway was confirmed, validated, and published in the local web-based health service. We want to present and share our experience with colleagues involved in the perioperative management of pituitary diseasesin other centers.

Outcomes following endoscopic endonasal resection of sellar and supresellar lesions in pediatric patients

The endoscopic endonasal approach (EEA) is a credible surgical alternative for the resection of sellar and suprasellar lesions such as pituitary adenomas, craniopharyngiomas, and Rathke cleft cysts. However, its application to pediatric patients poses several unique challenges that have not yet been well evaluated. The authors evaluate the safety, efficacy, and outcomes associated with the use of the EEA for treatment of these pathologic entities in pediatric patients. Retrospective review of 30 patients between the ages of two and 24 who underwent endoscopic endonasal resection of sellar or suprasellar lesions between January 2010 and December 2015. Endocrinological and ophthalmological outcomes, as well as extent of resection and complications were all evaluated. Gross total resection was achieved in eight of the nine pituitary adenomas, nine of the 12 craniopharyngiomas, and six of the nine Rathke cleft cysts. Of the 30 patients, 22 remained disease free at last follow-up. A total of six patients developed hypopituitarism and five developed diabetes insipidus. Eleven patients experienced improved vision, sixteen experienced no change, and one patient experienced visual worsening. Postoperative cerebrospinal fluid leak was seen in a single case and later resolved, vasospasm/stroke was experienced by 10%of patients, and new obesity was recorded in 10% of patients. There were no perioperative deaths. Endoscopic endonasal resection is a safe and effective surgical alternative for the management of sellar and suprasellar pathologies in pediatric populations with excellent outcomes, minimal complications, and a low risk of morbidity.

Transnasal endoscopic approach for pediatric skull base lesions: a case series.

Transnasal endoscopic transsphenoidal approaches constitute an essential technique for the resection of skull base tumors in adults. However, in the pediatric population, sellar and suprasellar lesions have historically been treated by craniotomy. Transnasal endoscopic approaches are less invasive and thus may be preferable to craniotomy, especially in children. In this case series, the authors present their institutional experience with transnasal endoscopic transsphenoidal approaches for pediatric skull base tumors. The authors retrospectively reviewed pediatric patients (age ≤ 18 years) who had undergone transnasal endoscopic transsphenoidal approaches for either biopsy or resection of sellar or suprasellar lesions between 2007 and 2016. All operations were performed jointly by a team of pediatric neurosurgeons and skull base otolaryngologists, except for 8 cases performed by one neurosurgeon. The series included 42 patients between 4 and 18 years old (average 12.5 years) who underwent 51 operations. Headache (45%), visual symptoms (69%), and symptoms related to hormonal abnormalities (71%) were the predominant presenting symptoms. Improvement in preoperative symptoms was seen in 92% of cases. Most patients had craniopharyngiomas (n = 16), followed by pituitary adenomas (n = 12), Rathke cleft cysts (n = 4), germinomas (n = 4), chordomas (n = 2), and other lesion subtypes (n = 4). Lesions ranged from 0.3 to 6.2 cm (median 2.5 cm) in their greatest dimension. Gross-total resection was primarily performed (63% of cases), with 5 subsequent recurrences. Nasoseptal flaps were used in 47% of cases, fat grafts in 37%, and lumbar drains in 47%. CSF space was entered intraoperatively in 15 cases, and postoperative CSF was observed only in lesions with suprasellar extension. There were 8 cases of new hormonal deficits and 3 cases of new cranial nerve deficits. Length of hospital stay ranged from 1 to 61 days (median 5 days). Patients were clinically followed up for a median of 46 months (range 1-120 months), accompanied by a median radiological follow-up period of 45 months (range 3.8-120 months). Most patients (76%) were offered adjuvant therapy. In this single-institution report of the transnasal endoscopic transsphenoidal approach, the authors demonstrated that this technique is generally safe and effective for different types of pediatric skull base lesions. Favorable effects of surgery were sustained during a follow-up period of 4 years. Further refinement in technology will allow for more widespread use in the pediatric population.

The Angelina Dissectors: A Novel Design of Dissectors for Endoscopic Endonasal Approaches.

Background The description and refinement of the transsphenoidal approach would not be possible without new tools and technologies developed by surgeons to facilitate this approach, which is nowadays the standard procedure for more than 90% of sellar lesions. The latest major change in transsphenoidal surgery was the introduction of the rigid endoscope and the subsequent description of the endoscopic endonasal approach. Traditional bayoneted instruments, when used for this technique, were inadequate. New instruments designed, specifically for this technique, are necessary to facilitate the surgeon's work and improve patient outcome. Objective This study describes a novel design of dissectors created specifically for endoscopic endonasal approaches. Methods To develop and design the Angelina dissectors, we used our extensive surgical experience to identify the shortcomings of the available dissectors used for transsphenoidal surgery and created the Angelina dissectors. Results The Angelina dissector was designed with a unique shaft shape which facilitates endoscopic endonasal surgery. Conclusion Even though an endoscopic endonasal approach is possible using other instruments, the design of these dissectors aids the surgeon's work. It is our impression, based on personal experience that it allows more freedom of movement and dexterity during the procedure, which could translate as an improved patient outcome.

Abdominal Fat Graft for Sellar Reconstruction: Retrospective Outcomes Review and Technical Note.

The transsphenoidal approach is the standard of care for the treatment of pituitary adenomas and is increasingly employed for many anterior skull base tumors. Persistent postoperative cerebrospinal fluid (CSF) leaks can result in significant complications. To analyze our series of patients undergoing abdominal fat graft repair of the sellar floor defect following transsphenoidal surgery, describe and investigate our current, routine technique, and review contemporary and past methods of skull base repair. A recent consecutive series (2008-2017) of 865 patients who underwent 948 endonasal procedures for lesions of the sella and anterior skull base was retrospectively reviewed. Three hundred eighty patients underwent reconstruction of the sellar defect with an abdominal fat graft. The diagnoses of the 380 patients receiving fat grafts were the following: 275 pituitary adenomas (72.4%), 50 Rathke cleft cysts (13.2%), 12 craniopharyngiomas (3.2%), and a variety of other sellar lesions. Fourteen patients had persistent postoperative CSF leak requiring reoperation and included: 5 pituitary adenomas (1.3%), 4 craniopharyngiomas (1.1%), 2 arachnoid cysts (0.53%), 2 prior CSF leaks (0.53%), and 1 Rathke cleft cyst (0.26%). Four patients (1.1%) developed minor abdominal donor site complications requiring reoperation: 1 hematoma, 2 wound complications, and 1 keloid formation resulting in secondary periumbilical infection. Minimizing postoperative CSF leaks following endonasal anterior skull base surgery is important to decrease morbidity and to avoid a prolonged hospital stay. We present an evolved technique of abdominal fat grafting that is effective and safe and includes minimal morbidity and expense.

Early Basal Cortisol Level as a Predictor of Hypothalamic-Pituitary-Adrenal (HPA) Axis Function After Pituitary Tumor Surgery.

The purpose of this study was to evaluate the clinical relevance of the early postoperative basal cortisol level in assessing the postoperative hypothalamic-pituitary-adrenal (HPA) axis function after pituitary tumor surgery. We performed a prospective observational study that enrolled 83 patients operated for pituitary adenoma or other sellar lesions at the University Hospital Center Zagreb between December 2013 and April 2017 (44 nonfunctioning pituitary adenomas, 28 somatotropinomas, 5 craniopharyngiomas, 2 prolactinomas resistant to medical therapy and 4 other lesions - Rathke's cleft cyst, arachnoid cyst, chondroma and gangliocytoma). Exclusion criteria were Cushing's disease, chronic therapy with glucocorticoids prior to surgery and preoperative adrenal insufficiency. Early postoperative basal cortisol levels (measured on the second postoperative day) and the Synacthen stimulation test (performed 3 months after the surgery with the peak cortisol level of>500 nmol/L considered as a normal response) were analyzed to assess HPA axis function during follow-up. ROC analysis showed a cut-off of the basal cortisol level of ≥300 nmol/L measured on the second postoperative day to predict normal postoperative HPA axis function with the sensitivity of 92.31%, specificity of 87.14% and positive predictive value of 57.14%. The basal cortisol level on the second postoperative day is a valuable tool to predict integrity of the HPA axis after pituitary tumor surgery. Our data suggest that the cortisol level of ≥300 nmol/L accurately predicts adrenal sufficiency and that in these patients glucocorticoid therapy can be withdrawn.

Rathke's cleft cysts: a 6-year experience of surgery vs. observation with comparative volumetric analysis.

Rathke's cleft cysts (RCCs) are common sellar lesions. Their management remains controversial, particularly when small or asymptomatic. Herein we review a consecutive series of RCC patients managed with surgery or observation. All patients with a new diagnosis of presumed RCC, based on MRI, from February 2012-March 2018 were retrospectively divided into observational and surgical cohorts based on an intent-to-treat model. The cohorts were compared for clinical presentation, and cyst volume. The observational cohort was followed for change in cyst size. The surgical cohort was followed for changes in endocrinopathy, visual symptoms, headache and recurrence. Of 90 patients (mean age 36.7 ± 19.4years; 68% female), 60% (n = 54) were in the observational cohort and 40% (n = 36) in the surgical cohort. Average follow-up was 13 ± 23months in the observational cohort and 24 ± 19months in the surgical group. In comparing the cohorts, mean ages were similar with more women in the surgical group (81% vs. 56%, p = 0.04). Most patients in the observational cohort had incidentally-discovered RCCs (n = 50, 88%) as opposed to the surgical cohort (n = 6, 17%). The surgical cohort had higher rates of headache (89% vs 26%, p < 0.001), endocrinopathy (36% vs 0%, p < 0.001), and visual dysfunction (19% vs 0%, p = 0.001). Mean cyst volume and maximal cyst dimensions were greater in the surgical cohort (0.94 ± 0.77cm3 and 14.2 ± 4.1mm), compared to the observational cohort (0.1 ± 0.14cm3 and 6.4 ± 3mm), (p < 0.001). Among the 53% (n = 30/54) of patients in the observational group with follow-up, 3 (10%) had spontaneous RCC shrinkage, 1 (3%) had modest asymptomatic growth (at 10months from initial MRI), and 87% had stable cyst size. Of the 36 patients recommended to have surgery, 89% (n = 32) did so. Post-operatively, complete or partial resolution of headache, endocrinopathy and visual dysfunction were documented in 90% (n = 28/30), 75% (n = 10/12), and 100% (n = 7/7), respectively. On follow-up MRI, 8 (22%) patients had some cyst reaccumulation, of whom 3 (8%) were symptomatic and underwent uneventful reoperation. No major complications such as hematoma, CSF leak, new endocrinopathy or visual deficits occurred. From this consecutive series, a majority (60%) of RCCs do not appear to warrant surgical intervention and have a low risk of cyst progression. However, surgical cyst removal appears to be indicated and safe for patients with larger, symptomatic RCCs. Simple cyst drainage has a high rate of improvement in pituitary gland function, visual function and headache resolution with low complication rates and symptomatic recurrence risk. These findings stress the importance of careful case selection and potential utility of volumetric assessment for patients with RCCs.

Diffusion tensor imaging and visual evoked potentials in pediatric patients with sellar region lesions

Objiective: To evaluate the prognosis of visual function and the impact of surgery in pediatric patients with sellar mass lesions, as evidenced by diffusion tensor imaging (DTI) and visual evoked potentials. Methods: Twenty patients with sellar mass lesions were included in the study. DTI and visual evoked potentials were obtained before and after surgery. Fractional anisotropy (FA) and apparent diffusion coefficient (ADC) values were calculated for both optic nerves. DTI parameters and visual evoked potential amplitudes were compared for all patients to assess the correlation between DTI parameters and visual function. Results: The 20 patients were divided into two groups according the relationship between the lesions and the optic chiasm. The FA values increased significantly after operation, while the ADC values decreased (P<0.05). And the average amplitude of visual evoked potentials after operation was significantly higher than before operation (P<0.05). Conclusions: DTI assessments of the affected sides, with the resulting FA and ADC values, may help to estimate the visual improvement produced by surgical therapy in the early postoperative period. Surgical removal can improve visual function dramatically.

SAT-476 A Case of Rathke's Cleft Cyst Presenting with Hyperprolactinemia

CASE: A 15 year-old G0P0 female with no significant PMH was referred to Endocrinology for oligomenorrhea and hyperprolactinemia. Menarche was at age 13 and she subsequently had oligomenorrhea for a year. She endorsed headaches and fatigue upon climbing a flight of stairs but denied vision changes, galactorrhea, hirsutism, SOB, and constipation. She was not on any psychotropic medications.The patient was hemodynamically stable with a normal physical examination including affect and cognition. Labs revealed prolactin-402 ng/mL (N=3-24 ng/mL), monomeric prolactin-277 ng/mL (macroprolactin=31%), cortisol-9.2 ug/dL one hour after cosyntropin, and DHEA-54.8 ug/dL (N=110-433.2ug/dL). TSH, LH, FSH, sex hormone binding globulin, testosterone, HCG, insulin-like growth factor, 17OH-progestereone were all within normal limits. MRI with and without contrast revealed a 1.5 x 1.8 x 1.8cm sellar lesion with characteristics most consistent with Rathke’s cleft cyst (RCC) with mass effect on the infundibulum and optic chiasm. The patient was started on cabergoline 0.25mg twice a week and Neurosurgery was consulted. Surgical resection was recommended. DISCUSSION: We present a rare case of symptomatic hyperprolactinemia in an adolescent female with radiologic evidence of RCC. RCC is an uncommon and usually asymptomatic intrasellar lesion, but pituitary dysfunction has been reported in 39% to 100% of these cases with prolactin levels generally less than 200 ng/mL. This hyperprolactinemia has been postulated to occur due to pituitary stalk compression from the sellar mass causing a decrease in dopamine (prolactin inhibitor) delivery. Possible explanations for RCCs with grossly elevated prolactin levels include: (1) coexisting pituitary adenoma, (2) cystic adenoma, and (3) pituitary stalk effect. (1) and (2) are the simplest explanations but radiologic features of adenomas were not seen on MRI. In our case, prolactin was higher than the typical levels seen in stalk compression raising concern for either coexisting pituitary adenoma or cystic adenoma although these are rare. This finding combined with hypocorticolism and radiologic evidence of mass effect warranted surgical intervention. CONCLUSION: Symptomatic RCC is rare, but clinicians should be aware that hyperprolactinemia is common in these cases. The degree of hyperprolactinemia may help guide decisions about further diagnostic evaluation and treatment. REFERENCES: 1. Eguchi K, Uozumi T, Arita K, et al. Pituitary function in patients with Rathke’s cleft cyst: significance of surgical management. Endocrine Journal. 1994; 41: 535-540. 2. Isono M, Kamida T, Kobayashi H, et al. Clinical features of symptomatic Rathke’s cleft cyst. Clin Neurol Neurosurg. 2001; 96-100. 3. Voelker JL, Campbell RL, Muller J. Clinical, radiographic, and pathological features of symptomatic Rathke’s cleft cyst. J Neurosurg. 1991; 74: 535-544.

SUN-432 A Case of Sellar Plasmacytoma Masquerading as Pituitary Carcinoma with Hypopituitarism

Background: Sellar plasmacytomas are rare tumors arising from clonal plasma cells localized to the sellar/parasellar region. They may mimic other pituitary pathology such as pituitary adenomas, chordomas, meningiomas or metastatic carcinomas in both clinical manifestations and radiographic evidence. Certain features such as predominance of cranial nerve palsies and absence of hypopituitarism appear to be more common with sellar plasmacytomas. We present an interesting case of a sellar plasmacytoma, initially mistaken for pituitary carcinoma, with rapid development of hypopituitarism and systemic multiple myeloma. Case: A 75 year old healthy male presented with sudden onset left eye diplopia and retro-orbital pain. Left eye ptosis and diplopia with left lateral and upward gaze were noted on exam. Initial MRI brain revealed a 17mm soft tissue mass involving pituitary and clivus, extending into the left superior orbital fissure, concerning for pituitary carcinoma. With the exception of low LH (1.2) and testosterone (25), baseline pituitary hormone labs, complete blood count and metabolic panel were within normal limits. Within 1 month, he acutely presented with fever, confusion, weakness, and bony pains. Repeat pituitary labs showed low ACTH < 5, AM cortisol 4.3, TSH 0.07, FT4 0.5. New findings of acute renal injury, hypercalcemia, anemia with elevated Mspike, Kappa light chain 39.7 (0.3-2.0mg/dl), kappa/lamda ratio 66.2 (0.3-1.6) and beta2microglobulin 4484 were found. Repeat MRI brain noted multifocal osseous metastatic disease with left sellar and cavernous sinus lesion contiguous with clival osseous lesion displacing pituitary gland, concerning for plasmacytoma. PET/CT showed focal lytic lesions in clivus and posterior left iliac bone. Bone marrow biopsy confirmed 70% myeloma involvement with kappa monotypic plasma cell population. He was started on hydrocortisone, levothyroxine, chemotherapy and XRT. Conclusion: The rarity of plasmacytomas, combined with their clinical presentation and imaging characteristics similar to other sellar tumors, can often result in misdiagnosis. Accuracy of diagnosis is critical, as sellar plasmacytomas and underlying multiple myeloma (present in 50% of cases) require XRT and systemic chemotherapy respectively rather than surgery. Although pituitary function is usually preserved in plasmacytomas, our case shows that acute anterior pituitary dysfunction can be an early presenting sign.

SUN-433 Pituitary Dysfunction Secondary to a Large Cavernous Internal Carotid Artery Aneurysm

Background: Intracranial aneurysms extending into the sellar space and leading to pituitary dysfunction are exceedingly rare. This type of vascular lesions may be responsible for less than 0.2% of cases of hypopituitarism. Herein, we present a case of hypopituitarism and hyperprolactinemia secondary to a large unruptured left cavernous internal carotid artery aneurysm with intra- and suprasellar extension. Clinical case: An 81-year-old woman with past medical history of long-standing primary hypothyroidism was noted to have a suppressed serum TSH (0.007 uU/mL [0.400 - 5.500]) on routine laboratory studies. Prior to this, she had been on a stable dose of levothyroxine replacement for several years and her TSH level had been maintained within the normal reference range. Subsequent laboratory studies showed an elevated prolactin level (173.4 ng/mL [4.5 - 26.8]), inappropriately low gonadotropin levels for a post-menopausal woman (FSH 4.4 mU/mL [20 - 100]; LH 0.8 mU/mL [>20]), normal IGF-1 (96 ng/mL [27 - 170]), and a low morning cortisol level (2.5 ug/dL (5.3 - 22.5)] with an inappropriately normal ACTH level (12 pg/mL [<47]). Her 60-minute cortisol level was <20 ug/dL during an ACTH stimulation test. The patient was started on steroid replacement and was continued on levothyroxine. A brain MRI was performed and demonstrated an area of heterogeneous unenhanced T1-weighted signal intensity and T2 signal void in the sellar space, measuring approximately 15 mm in diameter. The radiological differentials included a proteinaceous pituitary cyst or a vascular aneurysm. A brain MRA was then obtained for better characterization of the lesion, which revealed a large left cavernous internal carotid artery aneurysm (13 x 17 x 16 mm), completely filling the sellar space and extending into the suprasellar region. The patient was referred to neurosurgery and underwent a successful pipeline embolization of the aneurysm. At two months follow-up, her TSH was 0.159 uU/mL and her prolactin level was 140.3 ng/mL. Conclusion: Aneurysms with intrasellar extension should always be considered in the differential diagnosis of sellar lesions. When compared with cases of nonfunctioning pituitary macroadenomas, intrasellar aneurysms may result in comparable dysfunction in pituitary hormone production and hyperprolactinemia. Endocrine abnormalities are usually irreversible and permanent hormone replacement therapy is necessary. Long-term follow-up may elucidate pituitary function recovery after effective aneurysm treatment.