Sellar Lesions Research Articles

A giant invasive macroprolactinoma with recurrent nasal bleeding as the first clinical presentation: case report and review of literature

BackgroundGiant prolactinoma (> 4 cm in dimension) is a rare disorder. Invasive macroprolactinoma has the potential to cause base of skull erosion and extend into the nasal cavity or even the sphenoid sinus. Nasal bleeding caused by intranasal tumor extension is a rare complication associated with invasive giant prolactinoma.We report a case of giant invasive macroprolactinoma with repeated nasal bleeding as the initial symptom.Case presentationA 24-year-old man with an invasive giant prolactinoma in the nasal cavity and sellar region who presented with nasal bleeding as the initial symptom, misdiagnosed as olfactory neuroblastoma. However, markedly elevated serum prolactin levels (4700 ng/mL), and a 7.8-cm invasive sellar mass confirmed the diagnosis of invasive giant prolactinoma. He was treated with oral bromocriptine. Serum prolactin was reduced to near normal after 6 months of treatment. Follow-up magnetic resonance imaging showed that the sellar lesion had disappeared completely and the skull base lesions were reduced.ConclusionThis case is notable in demonstrating the aggressive nature of untreated invasive giant prolactinomas which can cause a diagnostic difficulty with potential serious consequences. Early detection of hormonal levels can avoid unnecessary nasal biopsy. Early identification of pituitary adenoma with nasal bleeding as the first symptom is particularly important.

Opportunities and challenges in the diagnosis and treatment of optic chiasm lesions: a clinical and research perspective

The optic chiasm is a critical component of the visual pathway, and lesions in the pituitary and sellar regions can cause irreversible damage to a patient's visual function, resulting in a significant decrease in their quality of life. As a result, neuro-ophthalmology evaluation is a crucial part of the multidisciplinary treatment of pituitary diseases. However, due to the significant variation in the anatomical structure of the optic chiasm and the sellar region, as well as the complexity of the injury mechanism, chiasm injury can result in diverse manifestations and severity levels, which can make it difficult to correlate with anatomical parameters. In recent years, research has increasingly focused on the early recognition of optic chiasm compression, the prediction of visual function after intervention, and the long-term neurodegenerative effects, while optical coherence tomography (OCT), electrophysiological examinations, and functional magnetic resonance imaging are currently the most commonly used methods for evaluating sellar region lesions. However, the role of these methods, represented by OCT, in clinical diagnosis and treatment, still lacks high-level clinical evidence support, and the evaluation and prediction of optic chiasm function remain key areas for further study. In addition to compression lesions, lesions such as inflammation, infiltration, and demyelination in the sellar region, caused by systemic multi-system diseases, can also lead to visual function damage and require recognition in clinical practice.

Basal interhemispheric approach for sellar and juxta-sellar lesions

BackgroundSellar and juxta-sellar lesions represent a surgical challenge due their deep and complex relationships to surrounding important neurovascular structures. The basal interhemispheric (BIH) approach, as a midline approach, has an advantage over the lateral approaches as it allows for the safe removal of these challenging tumors.AimTo describe the technique of BIH approach in a prospective cohort of 17 patients with sellar and juxta-sellar lesions with different pathologies presented to Alexandria Main University and affiliated hospitals. As well as the clinical outcome and complications of this technique, these cases were followed up for a mean period of 20 weeks.ResultsGross total resection was achieved in 9 cases (52.9%), subtotal resection was achieved in 5 cases (29.5%), while partial resection was carried out in 3 cases (17.6%). Visual status improved following surgery in 10 patients (58.8%), remained stationary in 5 patients (29.4%) and worsened in 2 cases. Post-operative shunt was needed in three cases, and severe water and electrolyte disturbance was recorded in 3 cases and pan-hypopituitarism in a single case. A single mortality was recorded, but it was due to pulmonary embolism and was not related to the approach.ConclusionBIH could be a panoramic safe approach that allows tumor removal from multiple corridors with identification of the regional anatomy of the tumor thus elaborating the surgical strategy for gross total tumor removal.

Transphenoidal surgery for sellar and suprasellar lesions: a prospective analysis of factors affecting inpatient length of stay

Searchable abstracts of presentations at key conferences in endocrinology ISSN 1470-3947 (print) | ISSN 1479-6848 (online)

Abstract #1408202: A Case of Rathke's Cleft Cyst with Multiple Associated Endocrinopathies

Rathke Cysts are cystic sellar or suprasellar lesions deriving from the remnants of Rathke’s pouch. During fetal development, Rathke’s pouch migrates from the oral ectoderm to what eventually develops as the sellar region. The pharyngohypophyseal stalk connecting Rathke’s pouch to the stomodeum is obliterated by growth of the sphenoid bone. Inadequate obliteration of the pharyngohypohyseal stalk results in formation of the rathke cyst. While most cases of Rathke cyst are asymptomatic, they may present with headache, vision loss, pituitary hormone deficiency, and diabetes insipidus. We present the case of a 44-year-old female with a history of cerebral palsy, secondary amenorrhea beginning at age 30, and blindness who presented to the hospital with generalized weakness and altered mental status. The degree and nature of visual impairment could not be assessed in the inpatient setting due to the patient’s altered mental status. She was found to have a urinary tract infection and was started on antibiotic therapy. Hospital course was complicated by hypotension refractory to fluid resuscitation. Further workup demonstrated low AM cortisol responsive to cosyntropin stimulation, and hypotension resolved with IV scheduled hydrocortisone. Pituitary lab follow-up demonstrated low estradiol with inappropriately normal gonadotropin levels, low Free T4 with inappropriately normal TSH, low IGF-1, and normal prolactin. MRI revealed a 2.4x1.7x2.2cm suprasellar mass consistent with Rathke cyst, with obliteration of the optic chiasm, pituitary parenchyma, and infundibulum. The patient’s adrenal insufficiency, panhypopituitarism, blindness, and amenorrhea were likely caused by mass effect on the optic chiasm and pituitary gland. Secondary adrenal insufficiency was managed with physiologic hydrocortisone dosage and secondary hypothyroidism was managed with oral levothyroxine. Outpatient neurosurgical intervention was recommended for cyst resection. This case represents a rare, symptomatic presentation of a Rathke cyst with vision loss and multiple endocrinopathies. Visual symptoms, which may include visual field defects, decreased visual acuity or oculomotor palsies, arise from mass effect on the optic chiasm or cavernous sinus. For these patients, surgical resection may recover visual function or prevent irreversible vision loss. Rathke cyst may also present with hypopituitarism. Restoration of pituitary function following resection is less common than resolution of visual symptoms. In patients presenting with visual disturbances, adrenal insufficiency, and hypopituitarism, a diagnosis of symptomatic Rathke cyst or other sellar mass should be considered.

Abstract #1398468: Lymphocytic Hypophysitis Successfully Treated with Prednisone

Lymphocytic Hypophysitis is an inflammatory disorder of the pituitary gland that has classically been described in pregnant and postpartum women. It is the most common type of Primary Hypophysitis which refers to inflammation of the pituitary gland that is not secondary to other systemic diseases or drugs. This is a rare group of disorders with an estimated annual incidence of 2.4 in 10 million. We present the case of a young woman diagnosed with Lymphocytic Hypophysitis and successfully treated with prednisone. A 27-year-old previously healthy female presented with daily headaches and fatigue that began 4 months after an uncomplicated vaginal delivery. She was amenorrheic despite cessation of breast milk production. Physical exam was within normal limits. Laboratory evaluation revealed an AM cortisol of 1 mg/dl (4-22), Free T4 of 0.4 ng/dL (0.8-1.8), LH of 0.5 mIU/mL (1.9-12.5), and estrogen of 30 pg/ml (12.5-166, follicular), consistent with adrenal insufficiency, hypothyroidism, and hypogonadism. Prolactin was normal. She had no evidence of Diabetes Insipidus. MRI pituitary with and without contrast revealed an intensely and homogenously enhancing sellar mass measuring 2 x 2 x 1.3 cm with suprasellar extension and mass effect on the optic chiasm. She did not have any abnormalities in her visual fields or acuity. Lymphocytic Hypophysitis was felt to be the most likely diagnosis based on her clinical presentation and imaging features. Via shared decision-making, she was started on 40 mg of oral prednisone daily in addition to levothyroxine and oral combined contraceptive pills. On reevaluation 2 months later, her symptoms improved and a repeat MRI revealed a dramatic improvement in the appearance of the lesion following which prednisone was reduced to 30 mg with a plan for revaluation after 2 more months. Lymphocytic Hypophysitis should always be suspected in pregnant and postpartum women with neurological complaints of headaches or visual abnormalities and pituitary hormonal deficiency. The investigation involves obtaining an anterior pituitary hormonal profile including TSH with free T4, ACTH with cortisol, FSH, LH, estradiol, and prolactin. Serum sodium with plasma and urine osmolality can be used to evaluate diabetes insipidus in suspected cases. MRI pituitary with and without contrast frequently reveals a symmetric sellar lesion that enhances intensely and homogeneously with gadolinium. A pituitary adenoma, in contrast, enhances less than the normal pituitary tissue. A tool based on clinical and radiological features called the Gutenberg score can also aid in differentiating lymphocytic hypophysitis from an adenoma. Treatment involves management of hormonal deficiencies including Diabetes Insipidus and relief of symptoms of mass effect. Glucocorticoids can induce remission and possible recovery of hormonal function. Follow-up with serial imaging in a few months after initiation of glucocorticoids is essential. Diagnosis needs to be reconsidered if there is no improvement in the appearance of the lesion and a pituitary biopsy may be indicated.

Opinion article: Neurosurgical treatment for neuro-ophthalmologic conditions.

A significant role of the neuro-ophthalmologist is to counsel patients on appropriate management and anticipated visual prognosis for conditions affecting the afferent and efferent visual systems, including those requiring neurosurgical treatment. However, the literature regarding anticipated neuro- ophthalmologic prognosis after neurosurgical intervention for cerebral aneurysms, sellar lesions, optic pathway tumors, and elevated intracranial pressure is limited with many key questions unanswered. For example, if a cerebral aneurysm is equally amenable to clipping or endovascular coiling, is there a preferred approach in terms of visual prognosis based on aneurysm location? Is dural venous sinus stenting (VSS) for idiopathic intracranial hypertension (IIH) superior, equivalent or inferior to shunting in terms of visual recovery and safety profile? Landmark studies on pituitary tumors using pre-operative optical coherence tomography (OCT) imaging of the optic nerve head to predict visual recovery after surgical decompression of the optic chiasm have changed neuro-ophthalmologic practice and enabled patients to be better informed regarding expected visual outcomes. 1,2 In order to optimize an interdisciplinary team approach to patient care, further studies of visual outcomes for neuro- ophthalmologic conditions requiring neurosurgical intervention are needed.

Anatomical variations of the cavernous Internal Carotid Artery: Validation of an MRI-based classification

ObjectiveVariations of the cavernous Internal Carotid Artery (ICA) angulation (C4-bend) have been classified into 4 anatomical subtypes with particular surgical relevance, as a very angulated ICA comes in closest contact with the pituitary gland leading to higher risk of iatrogenic vascular injury. This study aimed to validate this classification using current routine imaging techniques. MethodsThe different cavernous ICA bending angles were measured on 109 MRI TOF sequences, within a retrospective database of patients with no sellar lesions. Each ICA was classified into one of 4 anatomical subtypes as already defined in a previous study [1]. Interrater agreement was assessed by a Kappa Correlation Coefficient. ResultsKappa Correlation Coefficient was of 0.90 [0.82–0.95], validating the strong level of agreement between all observers when using the present classification. ConclusionClassification of the cavernous ICA in 4 subtypes appears statistically valid on routinely used pre-operative MRI, providing an efficient tool to estimate the vascular iatrogenic risk prior to endoscopic endonasal transsphenoidal surgery.

Nasal Crust-Related Morbidity and Debridement After Endoscopic Skull Base Surgery.

Introduction: Nasal crust after endoscopic skull base surgery can cause nasal congestion, obstruction, and pain, which can affect quality of life. The use of debridement aims to provide symptomatic relief and improve quality of life. Generally, most adult patients tolerate office-based debridement, except in a few select patients that require further sedation in the operating room for a debridement. The study sought to determine the rate of symptomatic crust-related morbidity and the rate of debridement in both the office and the operating room. Methods: Premorbid, operative, and postoperative data of adult patients who had endoscopic skull base surgery in our institution from 2014 to 2018 were reviewed retrospectively. The characteristics of nasal symptoms in the postoperative period were determined and the number of debridements in the office and the operating room were analyzed. Results: Two hundred and thirty-four (234) patients with 244 surgeries were included in the study. The majority, 68.9%, had a sellar lesion and a free mucosa graft (FMG) was the most common skull base reconstruction at 53.5%. One hundred and twenty (49.0%) had crust-related symptoms during the postoperative period and 11 patients (4.5%) required the operating room for debridement. The use of a pedicled flap, anxiety, and preoperative radiotherapy were significantly associated with intolerance to in-office debridement ( p -value=0.05). Conclusions: The use of a pedicled flap or anxiety may predispose patients to require an OR debridement. Previous radiotherapy also influenced the tolerance to the in-office debridement.

347 Management of Rathke Cleft Cysts in Children: The Rady Children’s Hospital Approach

INTRODUCTION: Rathke cleft cysts (RCCs) are benign, epithelial-lined sellar lesions that arise from remnants of the craniopharyngeal duct. Due to their rarity in the pediatric population, data are limited regarding the natural history and management of growing or symptomatic RCCs. METHODS: We performed a retrospective study of RCC patients age ≤18 years treated between 2006-2022 at our institution. RESULTS: 567 patients with a diagnosis of pituitary mass or cyst were identified. Of these, 31 underwent surgery and had a histopathological diagnosis of RCC, 58% female and 42% male. Mean age was 13.2 +/- 4.2 years. Presenting symptoms included headache (58%), visual changes (35%), and endocrinopathies or growth delay (26%); 13% were identified incidentally and grew significantly on serial imaging. Six percent presented with intralesional hemorrhage. Surgical approach was transsphenoidal for 90% of patients and orbitozygomatic for 10%. Preoperative headaches resolved in 61% of patients after surgery. New pituitary axis deficits were seen in 9.7%. Only two complications occurred from a first-time surgery: one cerebrospinal fluid leak requiring lumbar drain placement, and one case of epistaxis requiring cauterization. No patients experienced new visual or neurological deficits. Patients were followed postoperatively with serial imaging. Mean follow-up was 62.9 +/- 58.4 months. Recurrence requiring reoperation occurred in 32% of patients (transsphenoidal n = 8, pterional n = 2). Five-year progression-free survival was 47.9%. Except for one patient with multiple neurological deficits from a concurrent tectal glioma, all patients had a modified Rankin Scale score of 0 or 1 (good outcome) at last follow-up. CONCLUSIONS: Due to their secretory epithelium, RCCs may demonstrate rapid growth and recurrence. Incidental RCCs can be managed conservatively, whereas symptomatic or growing lesions may be fenestrated or resected. Due to their dynamic and variable nature, careful patient selection and close postoperative surveillance are critical to optimizing patient outcomes.

Headache in Patients with Sellar Disease: Clinicomorphological Predictors of Headache and the Outcome of Endoscopic Transsphenoidal Surgery.

Objectives Sellar pathologies are frequently found on imaging performed to investigate headache. However, both headache and incidental sellar lesions are common. Hence, this study prospectively examined headache prevalence, phenotype, and severity in patients with sellar pathologies and the impact of transsphenoidal surgery on headache. Methods Patients undergoing transsphenoidal resection of sellar lesions were consecutively recruited. At baseline, participants were defined as having headache or not and headache phenotype was characterized using validated questionnaires. Headache severity was assessed at baseline and 6 months postoperatively using the Headache Impact Test-6 (HIT-6) and Migraine Disability Assessment Score (MIDAS). Tumor characteristics were defined using radiological, histological, and endocrine factors. Primary outcomes included baseline headache prevalence and severity and headache severity change at 6 months postoperatively. Correlation between headache and radiological, histological, and endocrine characteristics was also of interest. Results Sixty participants (62% female, 47.1 ± 18.6 years) were recruited. Sixty-three percent possessed baseline headache. HIT-6 scores were higher in patients with primary headache risk factors, including younger age (R 2 = -0.417, p = 0.010), smoking history (63.31 ± 7.93 vs 54.44 ± 9.21, p = 0.0060), and family headache history (68.13 ± 7.01 vs 54.94 ± 9.11, p = 0.0030). Headaches were more common in patients with dural invasion (55.70 ± 12.14 vs 47.18 ± 10.15, p = 0.027) and sphenoid sinus invasion (58.87 ± 8.97 vs 51.29 ± 10.97, p = 0.007). Postoperative severity scores improved more with higher baseline headache severity (HIT-6: R 2 = -0.682, p < 0.001, MIDAS: R 2 = -0.880, p < 0.0010) and dural invasion (MIDAS: -53.00 ± 18.68 vs 12.00 ± 17.54, p = 0.0030). Conclusion Headaches in sellar disease are likely primary disorders triggered or exacerbated by sellar pathology. These may respond to surgery, particularly in patients with severe headache and dural invasion.

Differentiation of pure cystic sellar lesions on magnetic resonance imaging.

Cystic pituitary adenomas and cystic craniopharyngiomas may mimic Rathke cleft cysts when there is no solid enhancing component on magnetic resonance imaging (MRI). This study aims to investigate the efficiency of MRI findings in differentiating Rathke cleft cysts from pure cystic pituitary adenoma and pure cystic craniopharyngioma. 109 patients were included in this study (56 Rathke cleft cysts, 38 pituitary adenomas, and 15 craniopharyngiomas). Preoperative magnetic resonance images were evaluated using 9 imaging findings. These findings include intralesional fluid-fluid level, intralesional septations, midline /off-midline location, suprasellar extension, an intracystic nodule, a hypointense rim on T2-weighted images, ≥ 2mm thickness of contrast-enhancing wall, T1 hyperintensity and T2 hypointensity. p < 0.01 was considered statistically significant. There was a statistically significant difference among groups for these 9 findings. Intracystic nodule and T2 hypointensity were the most specific MRI findings in differentiating Rathke cleft cyst from the others (98.1% and 100%, respectively). Intralesional septation and thick contrast-enhancing wall were the most sensitive MRI findings ruling out Rathke cleft cysts with 100% sensitivity. Rathke cleft cysts can be distinguished from pure cystic adenoma and craniopharyngioma with the presence of an intracystic nodule, T2 hypointensity, the absence of the thick contrast-enhancing wall, and absence of intralesional septations.

Trends in Stereotactic Radiosurgery for Intracranial and Spinal Pathologies: Analysis of the Top 100 Most Cited Articles.

There is a plethora of papers on the role of stereotactic radiosurgery (SRS) in various benign and malignant intracranial tumors, and it is possible to overlook the most important and landmark studies. Thus, the necessity of citation analysis arises, which reviews the most cited articles and recognizes the impact made by these articles. Utilizing the 100 most cited articles describing the use of SRS for intracranial and spinal pathologies, this article aims to provide meaningful information regarding the historical trends and recent directions in which this field is headed. We performed a search of the Web of Science database using the keywords "stereotactic radiosurgery," "gamma knife," "GKRS," "gamma knife radiosurgery," "LINAC," and "Cyberknife" on May 14, 2022. Our search retrieved a total of 30,652 articles published between the years 1968 and 2017. The top 100 cited articles were arranged in descending order based on citation count (CC) and citation per year (CY). The journal with the largest number of publications as well as citation count was the International Journal of Radiation Oncology Biology Physics (n = 33), followed by Journal of Neurosurgery (n = 25). The most cited article was authored by Andrews, which was published in 2004 in The Lancet (1699 CC, 89.42 CY). Flickinger, with 25 papers and 7635 total citations, was the author with the highest impact. Lunsford, with 25 publications and total citations of 7615, was a close second. The USA was the leading country with the maximum number of total citations (n = 23,054). Ninety-two articles described the use of SRS for intracranial pathologies (metastases, n = 38; AVM, n = 16; vestibular schwannoma, n = 9; meningioma, n = 8; trigeminal neuralgia, n = 6; sellar lesion, n = 2; glioma, n = 2; functional, n = 1; and procedure related, n = 10). Eight studies describing spinal radiosurgery were included, out of which four were on spinal metastases. Citation analyses of the top 100 articles revealed that the focus of research in the field of SRS started with functional neurosurgery and progressed to benign intracranial tumors and AVMs. More recently, central nervous system (CNS) metastases have received the maximum attention with 38 articles, including 14 randomized controlled trials finding a place in the top 100 cited articles. Presently, the use of SRS is concentrated in developed countries. Efforts need to be made for more widespread use in developing nations to bring the maximum possible benefits of this focused noninvasive treatment to a wider population.

Assessing national patterns and outcomes of pituitary surgery: is hospital administrative data good enough?

Purpose Patterns of surgical care, outcomes, and quality of care can be assessed using hospital administrative databases but this requires accurate and complete data. The aim of this study was to explore whether the quality of hospital administrative data was sufficient to assess pituitary surgery practice in England. Methods The study analysed Hospital Episode Statistics (HES) data from April 2013 to March 2018 on all adult patients undergoing pituitary surgery in England. A series of data quality indicators examined the attribution of cases to consultants, the coding of sellar and parasellar lesions, associated endocrine and visual disorders, and surgical procedures. Differences in data quality over time and between neurosurgical units were examined. Results A total of 5613 records describing pituitary procedures were identified. Overall, 97.3% had a diagnostic code for the tumour or lesion treated, with 29.7% (n = 1669) and 17.8% (n = 1000) describing endocrine and visual disorders, respectively. There was a significant reduction from the first to the fifth year in records that only contained a pituitary tumour code (63.7%–47.0%, p < .001). The use of procedure codes that attracted the highest tariff increased over time (66.4%–82.4%, p < .001). Patterns of coding varied widely between the 24 neurosurgical units. Conclusion The quality of HES data on pituitary surgery has improved over time but there is wide variation in the quality of data between neurosurgical units. Research studies and quality improvement programmes using these data need to check it is of sufficient quality to not invalidate their results.

Gastrointestinal carcinoma first presenting as pituitary mass: recognition of a rare case and multidisciplinary approach for the management of pituitary metastases

Abstract Introduction PMs (pituitary metastases) are rare and only a few cases are reported in the literature. In half of the documented cases, PMs originate from breast and lung cancers, rarely from other sites. They can grow rapidly and present local invasiveness, leading to acute onset of neurological symptoms, such as headache, visual and oculomotor disturbances, or endocrinological signs, such as diabetes insipidus and hypopituitarism. Their rarity and the lack of specific clinical and radiological features often impede their differentiation from other more common sellar area lesions, particularly when history of malignancy is absent. As PMs can be life-threatening, they must be recognized and treated promptly. Management of PMs may also be difficult because the prognosis depends on the course of the primary neoplasm. The case presented below is one of the few documented cases in the world of a pituitary metastasis from a primary gastrointestinal carcinoma. Clinical Case A 76-year-old woman, with no history of malignancy, presented with headache, dizziness and diplopia. Neurologic examination was remarkable for complete left ophthalmoplegia associated with sensitivity deficit in the left half face. Radiologic investigations documented a voluminous sellar and suprasellar formation, with extension in the ipsilateral cavernous sinus, compressing the optic chiasm and ipsilateral temporal lobe. The mass appeared heterogeneous as component of apoplectic infarction. Left cavernous sinus syndrome in apoplectic macroadenoma was diagnosed. Surgical removal of the lesion was performed through a transsphenoidal endoscopic endonasal approach. The histological exam disclosed a metastasis of adenocarcinoma with widespread necrotic-haemorrhagic elements. The immunohistochemical profile suggested an origin from a primary gastrointestinal carcinoma. Hormone replacement therapy and adjuvant therapy were instituted, although radiological investigations failed to identify the site of the primary gastrointestinal carcinoma. Postoperative MRI showed a parasellar residual tumor, which was radiologically stable at 6-month follow-up. The patient will undergo a radiotherapy and neurosurgical evaluation. Conclusions Clinicians should consider the possibility of PMs in patients with symptoms and signs that may suggest the presence of a pituitary mass. Since PMs can be cancer first manifestation, the recognition of PMs determines the referral to adequate oncological therapy. Prognosis is usually poor and it's difficult to estimate as it varies significantly depending on the systemic disease and the primary histology. Recognition and management of PMs remain complex and require a multidisciplinary team of endocrinologists, oncologists, neurosurgeons and neuroradiologists.

GH-Producing pituitary adenoma and concomitant Rathke's cleft cyst: A case report

Abstract Introduction Rathke cleft cysts (RCCs) are commonly believed to be cysts formed from remnants of Rathke's pouch. It is usually asymptomatic, &amp;lt; 3mm in diameter, and found in 13–33% of normal pituitary glands. The coexistence of pituitary adenoma (PA) and RCC is rare (incidence rate of 0.51%-1.7%), and growth hormone hypersecretion has also been observed in a limited number of cases in these patients. Therefore, we aim to present a rare case of growth hormone- (GH-) producing PA with concomitant RCC. Clinical Case A 29-year-old female patient, due to primary amenorrhea, was diagnosed as having 46XY chromosomal Swyer syndrome from her previous visit, and she had bilateral salphengoopherectomy afterward. In clinical follow-ups, due to the presence of growth complaints in the hands and feet, insulin-like growth factor-1 (IGF-1) was found to be high. The basal serum GH value was 8.89 ng/mL, and IGF-1 was 369 ng/mL. The 75 gr oral glucose tolerance test (OGTT) result showed that the serum GH level was not suppressed below 1.0 μg/L, and the test was repeated twice. Other anterior pituitary hormone axes are normal. In the pituitary magnetic resonance imaging revealed a low-signal 4 mm Rathke cleft cyst is observed in the T1A image in the midsection of the pituitary gland and a high-signal intra-cystic nodule is observed within the cyst on the T1A image (Figure 1A). The fluid-fluid level is observed in the cyst on the T2A image (Figure 1B). Conclusion The relationship between RCC and PA is unclear and theoretical. The theory is “transitional pituitary tumor,” defined as the Rathke pouch forming the anterior lobe of the pituitary gland with a proliferation of the anterior wall. Moreover, PAs originate from the anterior lobe of the pituitary gland via clonal changes. This shows that RCCs originated from the remnant of the Rathke pouch, and PA has a common embryonic origin. Nevertheless, they rarely occur at the same time. Only 14 patients with GH-producing PA and concomitant RCC have been reported in the literature. Preoperative correct diagnosis of PA and RCC coexistence is difficult, because RCCs’ imaging characteristics (size, position, and intensity) are highly variable, and cystic degeneration occurs in 5% to 18% of PA cases. RCCs are often surrounded by the normal pituitary gland and are located in the nearby midline of the sellar region. MRI characteristics of sellar lesions for PAs existing concomitant with RCCs may present with variable intensity on T1-weighted imaging. If radiographic imaging shows that typical RCC and PA are not obvious at first glance, the possibility of concomitant PA still needs to be considered. If the PA in this patient had been nonfunctional, correct diagnosis of the concomitant PA would have been much more difficult. This case offers a reminder that although rare, RCC can accompany PA, and performing contrast-enhanced MRI is useful to detect RCC.

Endoscopic 1½-transseptal approach for pituitary surgery.

We previously introduced the one-and-a-half (1½) nostril endoscopic transsphenoidal approach (OETA) to reduce the damage to the nasal structures. Here, we reported the modified approach which is called the endoscopic 1½-transseptal approach (EOTA) for pituitary surgery by combining the OETA and the microscopic transseptal approach to simplify intranasal procedures and protect nasal mucosa. In EOTA, we removed the sellar lesions in a corridor that is composed of the right submucosal space and the anterior left ½ nasal cavity. We introduced EOTA with a detailed technical description and preliminary clinical outcomes. A total of 128 patients who underwent EOTA for pituitary surgery from July 2018 to September 2020 were reviewed for evaluation of the safety and efficacy of this approach. EOTA had a high gross total resection (GTR) rate and a 1ow complication rate. GTR was achieved in 106 (82.8%) patients, with 81.4% for pituitary adenomas and 93.3% for other non-adenomatous lesions. Post-operative complications included 3 patients (2.3%) with postoperative cerebrospinal fluid leak, 3 patients (2.3%) with diabetes insipidus, 5 patients (3.9%) with anterior pituitary insufficiency and 2 patients (1.6%) with meningitis. In addition, EOTA simplified the intranasal procedures, which led to shortened operation time (67.8 minutes). The results of ASK nasal-12, the Lund-Kennedy score, and the odor identification test showed that patients who underwent EOTA recovered quickly after surgery and the nasal cavity returned to the preoperative condition both apparently and physiologically one month after surgery. EOTA is a simple, safe and effective approach for pituitary lesions, which provides not only a sufficient surgical corridor for 2-surgeon/4- or 3-hands technique but also minimally invasive access to the sellar region.

Defining the Clival Recess Surgical Corridor and Clival Classification System for Approach to Sellar Pathology.

Sellar masses within the pars intermedius, bordered anteriorly by normal pituitary gland/stalk, and/or with ectatic cavernous carotid anatomy are challenging and high risk when approached through the endonasal standard direct/anterior sellar approach. This approach portends itself to a higher risk of pituitary gland/stalk injury and subtotal resection with the aforementioned anatomic variants. To describe the indirect clival recess corridor approach to sellar lesions. This corridor is a "silent" point of access to lesions in this region endoscopically. While skull base teams may have used this approach to some degree, it has not yet been described in the literature to our knowledge. We defined the clival recess surgical corridor with skull base craniometric measurements and use a case example with aberrant anatomy to illustrate the approach. We cross-sectionally reviewed 42 patients with sellar and suprasellar masses. To describe the approach's anatomy, we devised and defined the terms dorsum sella plumb line, anatomic corridor, angle of osseous, and operative corridor. Created novel clival aeration grade informing surgical planning. Classified clival aeration as Grade 1 (100%-75% aeration), Grade 2 (75%-50% aeration), Grade 3 (50%-25% aeration), and Grade 4 (25%-0% aeration). This classification system determines extent of drilling of the clivus required to optimize the clival recess corridor approach and its limitations. The clival recess surgical corridor is effective for accessing pituitary lesions within the sella. Consider the indirect approach when a standard direct/anterior sellar approach has high risk for vascular injury and/or endocrinological dysfunction.

Predictors of visual outcome following surgery in sellar lesions

ABSTRACT Purpose: The purpose of the study is to find out the factors affecting visual outcome after surgery in patients with sellar lesions. Methods: We prospectively analyzed 36 patients diagnosed with sellar tumors who underwent surgery between July 2018 and January 2020. Clinical and demographic data including age, sex, pre- and post-operative visual acuity, visual field, and fundus examination was done in these patients. Size of the tumor was also measured radiologically. The size of the tumor was categorized as &lt;2 and ≥2 cm. Results: Larger tumors had significant association with optic disc changes and visual field defects. The mean visual acuity (LogMAR) and field defects (mean deviation) improved postsurgery (paired t-test). Five of the six patients with peripheral constriction showed an improvement in the visual fields after surgery while only one of 15 patients with temporal hemianopia showed improvement in visual fields after surgery (P = 0.009). Patients with a tumor size &lt;2 cm had statistically significant improvement in visual acuity (paired t-test P &lt; 0.001) and visual fields (paired t-test P = 0.008. Conclusion: Tumor size &lt;2 cm was found to be a good predictor of visual outcome after surgery.

Role of Magnetic Resonance Imaging in the Evaluation of Sellar, Parasellar and Suprasellar Lesions

Introduction: Magnetic resonance imaging (MRI) is now widely available, and considerable body of experiencehas been accumulated in using it to evaluate this region. MRI is now accepted as the imaging procedure of choicein the evaluation of sellar and parasellar pathology.The aims and objectives are to know the characteristic MRIfeatures of the sellar, parasellar and suprasellar lesions and to describe the incidence of the various lesions andtheir age and sex distribution in the study population.Methodology: Prospective study of 30 patients referred for imaging having clinical features raising suspicion ofsellar, parasellar and suprasellar lesions. They underwent magnetic resonance imaging of the brain-sella using1.5 TESLA PHILIPS ACHIEVA MRI MACHINE in the Department of Radiodiagnosis, KIMS &amp; RF, Amalapuram.Results: Out of 30 cases 4 cases were pituitary macroadenoma, 3 cases each of pituitary microadenoma,craniopharyngioma, meningioma and aneurysm. 2 cases of each hypothalamic hamartoma, Pituitary colloidcyst, Rathke’s cleft cyst, Arachnoid cyst and primary empty sella were found. And 1 case of each Suprasellartuberculoma, Trigeminal schwannoma, Suprasellar retinoblastoma and PNS rhabdomyosarcoma were found.Conclusion: MR imaging characteristics of the most common lesions like pituitary macroadenoma, microadenoma,craniopharyngioma, meningioma and aneurysm were sufficiently distinct to allow them to be differentiatedfrom each other and from most other entities. Other characteristics such as extrasellar versus intrasellar location,nature of contrast material enhancement, the presence of cystic components, and clinical findings permit furtherdifferentiation among the various other abnormalities. The superior resolution and multiplanar capacity of MRimaging best depicts the extent of sellar, parasellar and suprasellar lesions