NCOG-31. GIANT PITUITARY ADENOMA (GPA) SCORE: A NOVEL SCORING SYSTEM TO PREDICT POSTOPERATIVE OUTCOMES OF GIANT PITUITARY ADENOMAS

BACKGROUND Giant pituitary adenomas (GPA) are uncommon and highly variable in morphology and extension. There is no scoring system that considers all the dimensions of adenoma invasion. We developed a new Giant Pituitary Adenoma score and report our surgical experience and evaluate outcomes after resection of these tumors in accordance with the preoperative score. METHODS We developed a novel scoring system for classifying giant pituitary adenomas, and 11-year data of GPA surgery at our center was collected retrospectively, based on this scoring system. GPA Score considered tumor’s parasellar extension, encasement of cavernous internal carotid artery (ICA), suprasellar extension > 2 cm, suprasellar extension > 4cm and retrosellar extension. Maximum possible score was 9. The scoring system was applied to 53 patients of GPA who underwent surgical resection between January 1, 2006, and December 2017. The Lundin-Pederson (ABC/2) method was used to calculate the tumor volume both pre- and post-resection and linear regression was used to assess the relationship between extent of tumor resection and GPA score. RESULTS The median age of the study population was 42.08 ± 16.49 years. The mean maximum diameter of the pituitary adenomas was 5.0 cm (range 4.0 cm-8.5cm) while the mean volume of the adenomas was 27.3 cm3 (range 10 cm3-149 cm3). There were 3 cases of score 2, 5 cases of score 3, 13 cases of score 4, 20 cases of score 5, 9 cases of score 6 and 3 cases of score 7. The range of tumor volumes of tumors for scores from 2-7 was 17.3 cm3 to 65.8 cm3 and GPA score was correlated with the percent residual tumor using linear regression that was statistically significant (p= 0.001). CONCLUSION GPA Score is a reliable scoring system to predict the extent and subsequent difficulty in tumor resection in GPA.

Identification of the Extradural and Intradural Extension of Pituitary Adenomas to the Suprasellar Region: Classification, Surgical Strategies, and Outcomes

ObjectiveSuprasellar pituitary adenomas (PAs) can be located in either extradural or intradural spaces, which impacts surgical strategies and outcomes. This study determined how to distinguish these two different types of PAs and analyzed their corresponding surgical strategies and outcomes.MethodsWe retrospectively analyzed 389 patients who underwent surgery for PAs with suprasellar extension between 2016 to 2020 at our center. PAs were classified into two main grades according to tumor topography and their relationships to the diaphragm sellae (DS) and DS-attached residual pituitary gland (PG). Grade 1 tumors were located extradurally and further divided into grades 1a and 1b, while grade 2 tumors were located intradurally.ResultsOf 389 PAs, 292 (75.1%) were surrounded by a bilayer structure formed by the DS and the residual PG and classified as grade 1a, 63 (16.2%) had lobulated or daughter tumors resulting from the thinning or absence of the residual PG and subsequently rendering the bilayer weaker were classified as Grade 1b, and the remaining 34 (8.7%) PAs that broke through the DS or traversed the diaphragmic opening and encased suprasellar neurovascular structures were classified as Grade 2. We found that the gross total removal of the suprasellar part of grade 1a, 1b, and 2 PAs decreased with grading (88.4%, 71.4%, and 61.8%, respectively). The rate of major operative complications, including cerebrospinal fluid leakage, hemorrhage, and death, increased with grading.ConclusionsIt is essential to identify whether PAs with suprasellar extension are located extradurally or intradurally, which depends on whether the bilayer structure is intact. PAs with an intact bilayer structure were classified as grade 1. These were extradural and usually had good surgical outcomes and lower complications. PAs with no bilayer structure surrounding them were classified as grade 2. These were intradural, connected to the cranial cavity, and had increased surgical complications and a lower rate of gross total removal. Different surgical strategies should be adopted for extradural and intradural PAs.

Characteristics of Pituitary Tumors Including Non-Functioning Tumors - a Tertiary Care Center Experience

Introduction: Pituitary tumors compromise 10-20% of intracranial tumors. Non-functioning pituitary adenomas (NFPAs) are benign neoplasms which constitutes about 1/3rd of the pituitary tumors. There is few data on the characteristics of non-functioning tumors comparing with functioning tumors. Wide spread availability of MRI has eased the diagnosis of non-functioning tumors and early diagnosis. Objectives: To study the characteristics of pituitary tumors and the incidence of non-functioning tumors and compare with functioning tumors presenting to a tertiary care center. MethodsA retrospective study including 146 individuals with pituitary tumor were included from 2014 to 2020. Results: The study included 62(42.5%) male and 84(57.5%) female. The mean age was 41.97 + 13.93 years in males and 39.88 + 16.12 years in females. The mean duration of symptoms prior to presentation was 7.52 + 7.12 months and 10.37 + 11.60 months in males and females respectively. Macroadenoma (size >1cm) incidence was more with 55(88.7%) in males and 58(69%) in females. More number of males (87.1%) underwent surgery when compared to females (64.3%)(p – 0.002). There was more number of suprasellar and invasion of pituitary tumors seen in males compared to females(P- <0.01). There was no significant difference between various parameters like TSH, T4, LH, FSH, prolactin, Electrolytes, cortisol, ACTH, GH and IGF1 between males and females(p- NS). Seventy percent (103) of them were non-functioning tumors. Functioning tumors included 7(4.8%) acromegaly, 8(5.5%) cushings disease, 2(1.4%) gonadotropinoma, 24(16.4%) prolactinoma and 2(1.4%) posterior fossa tumor. Mean age in non- functioning tumors was 43.74 + 15.5 years. Mean diameter of tumor in non-functioning tumors was 21.04 + 11.54 cm when compared to functioning tumors (17.03 + 10.33 cm)(p- 0.61). Most of the patients with non-functioning tumors who underwent surgery received post operatively hydrocortisone and levothyroxine when compared to patients with functioning tumors(p < 0.01). There was no significant difference in terms of gender, suprasellar extension, invasion and pituitary hormones(p-NS). ConclusionMost of the pituitary tumors in our study were macroadenomas with more number of males undergoing surgery as most of them had suprasellar extension and invasion. Non- functioning tumors should be followed up regularly as most of them required hydrocortisone and levothyroxine post-operatively.

Pituitary infundibular epidermoid cyst: a rare cause of hypopituitarism

A 53-year-old man presented with 6 months history of weight loss associated with nausea, fatigue, dizziness and headache. On arrival he was in adrenal crisis. Biochemistry revealed anterior hypopituitarism with low cortisol, thyroxine, testosterone and a slightly raised prolactin. He was commenced on steroids, thyroxine and testosterone. MRI pituitary gland was reported to have a 9.4 mm microadenoma. Cabergoline was started for a possible microprolactinoma. Follow-up MRI showed increase in the size of complex cystic lesion causing chiasmal compression raising a possibility of craniopharyngioma. Visual fields assessment was normal. In view of the rapid enlargement, to protect vision and obtain a tissue diagnosis he underwent endoscopic trans-sphenoidal surgery. A cystic lesion was noted intraoperatively originating from pituitary stalk with intrasellar and suprasellar extension. It was filled with white caseous material and fluid. Histology revealed epidermoid cyst. His headache resolved postoperatively.

Comparing Mononostril, Binostril, and One and a Half Nostril Endoscopic Transsphenoidal Approach for Treating Pituitary AdenomaPituitary Adenoma

Background and Aim: EETA (Endoscopic Endonasal Transsphenoidal Approach) is a preferred choice for pituitary tumors. EETA offers minimal invasiveness, fewer complications, and better outcomes than the sublabial or transseptal microscopic approach. EETA has three approaches: mononostril endoscopic transsphenoidal approach, binostril endoscopic transsphenoidal approach, and one and a half nostril approach. This study aims to compare three different EETAs and compare between microscopic transsphenoidal approach, transcranial approach and EETA. Methods and Materials/Patients: To provide up-to-date information, we concisely reviewed these three EETAs. Using the keywords of “neuroendoscopy”, “META” (Mononostril Endoscopic Transsphenoidal Approach), “OETA” (One and a half nostril Approach), “BETA” (Binostril Endoscopic Transsphenoidal Approach), “pituitary adenoma”, “EETA”, “endoscopy”, “transsphenoidal approach”, “transcranial approach for pituitary adenoma” and “microscopic transsphenoidal approach”. We retrieved all the relevant articles from Google Scholar, PubMed, and Medline. Then, we reviewed them and critically analyzed them. Results: In BETA there is free and easy movement of surgical instruments in the surgical field and a broader view of the sphenoid sinus and it is an excellent approach to resect large tumors. The META is suitable in tumors with limited involvement of the intra-sellar and supra-sellar area. The mononostril approach is not suitable and has some limitations for the following situations: a crowded narrow nasal cavity, a harder tumor with the invasive appearance or significant suprasellar extension, and lesions other than pituitary adenomas. The OETA provides a sufficient surgical corridor for a 2-surgeon/4 or 3-hands technique with a minimal injury of the nose and good operative results, free and easy movement of surgical instruments in the surgical field, low post-operative morbidity, and good post-operative quality of life. Conclusion: In EETA, knowing the size and consistency of tumor, general versus invasive pituitary adenoma, and the extent of parasellar and suprasellar extension, is essential. If we cannot reach a pituitary macroadenoma with a trans-sphenoidal approach, then we can use a transcranial approach for the removal of the tumor. The microscopic transsphenoidal approach is suitable for pediatric pituitary adenoma

Pegvisomant in combination or pegvisomant alone after failure of somatostatin analogs in acromegaly patients: an observational French ACROSTUDY cohort study

Objective After surgery, when somatostatin analogs (SAs) do not normalise IGF-I, pegvisomant (PEG) is indicated. Our aim was to define the medical reasons for the treatment of patients with PEG as monotherapy (M) or combined with SA, either as primary bitherapy, PB (PEG is secondarily introduced after SA) or as secondary bitherapy, SB (SAs secondarily introduced after PEG). Methods We retrospectively analysed French data from ACROSTUDY. Results 167, 88 and 57 patients were treated with M, PB or SB, respectively, during a median time of 80, 42 and 70 months. The median PEG dose was respectively 15, 10 and 20 mg. Before PEG, the mean IGF-I level did not differ between M and PB but the proportion of patients with suprasellar tumour extension was higher in PB group (67.5% vs. 44.4%, P = 0.022). SB regimen was used preferentially in patients with tumour increase and IGF-I level difficult to normalise under PEG. In both secondary regimens, the decrease of the frequency of PEG’s injections, compared to monotherapy was confirmed. However, the mean weekly dose of PEG between M and PB remained the same. Conclusions The medical rationale for continuing SAs rather than switching to PEG alone in patients who do not normalise IGF-I under SAs was a tumour concern with suprasellar extension and tumour shrinkage under SA. A potential explanation for introducing SA in association with PEG appears to be a tumour enlargement and difficulties to normalise IGF-I levels under PEG given alone. In both regimens, the prospect of lowering PEG injection frequency favoured the choice.

Intracranial And Intraorbital Rosai Dorfman Disease

Rosai Dorfman disease is a rare histiocytic disorder. It is also known as Sinus Histiocytosis. It is with massive lymphadenopathy involves an overproduction of a type of white blood cell. The disease is rarely associated with intracranial and intraorbital involvement. Intracranial Rosai-Dorfman can mimic meningioma. Other pathologies also underline its pathologies. Here, we report a nine-year-old boy with a history of proptosis of the right eye and presenting with multiple skull lesions. Histopathological study revealed Sphenopetroclival lesion, which features that of Rosai Dorfman Disease. His MRI scan of the brain was taken, which showed evidence of right optic nerve meningioma with sella and suprasellar extension, causing severe proptosis. The child underwent right frontotemporal craniotomy with petrosectomy and Transylvanian, subtemporal approach to multicompartmental Rosai-Dorfmans lesion. After four months, the patient had a recurrence of the disease on which chemotherapy and steroids were started, which also did not show much response while taking an MRI scan. A corticosteroid is a useful option in the Central Nervous System Rosai Dorfman disease treatment. But this patient showed a negative outcome to the treatment.

Incidence and risk factors associated with reoperation for sellar hematoma following endoscopic transsphenoidal pituitary surgery

OBJECTIVEPostoperative sellar hematoma is an uncommon complication of endonasal endoscopic transsphenoidal surgery (EETS) for pituitary adenoma that often requires emergency surgical evacuation. Sellar hematomas can cause mass effect and compress parasellar structures, leading to clinically significant symptoms such as visual impairment and severe headache. The objective of this study was to determine the incidence and risk factors associated with reoperation for postoperative hematoma after EETS for pituitary adenoma.METHODSThe authors reviewed a prospectively acquired database of EETS for pituitary adenoma over 13 years at Weill Cornell Medicine, NewYork-Presbyterian Hospital and identified cases that required reoperation for confirmed hematoma. They also reviewed clinical and radiographic data of a consecutive series of patients undergoing EETS for pituitary adenoma who did not have postoperative hematoma, which served as the control group. Demographic data and risk factors were compared between the groups using univariate and multivariate analyses via binary logistic regression.RESULTSAmong a cohort of 583 patients undergoing EETS for pituitary adenoma, 9 patients (1.5%) required operation for sellar hematoma evacuation. All 9 patients with reoperation for sellar hematoma presented with worsening in their vision, and severe headache was present in 67%. New postoperative endocrine dysfunction developed in 78%. Clot evacuation improved vision in 88%. The mean time to hematoma evacuation was 4.5 days. The median length of stay for patients with sellar hematoma was 8 days (range 4–210 days) compared with a median length of stay of 3 days (range 1–32 days) for the control patients (p < 0.005). Significant risk factors in univariate analysis were tumor diameter ≥ 30 mm (p < 0.005), suprasellar extension (p < 0.005), tumor volume (p < 0.005), cavernous sinus invasion (p < 0.05), gonadotroph histology (p < 0.05), antiplatelet use (p < 0.05), and elevated BMI (p < 0.05). On multivariate analysis, tumor diameter ≥ 30 mm (OR 4.555, CI 1.30–28.90; p < 0.05) and suprasellar extension (OR 1.048, CI 1.01–1.10; p < 0.05) were found to be the only independent predictors of sellar hematoma. The incidence of hematoma in tumors ≥ 30 mm was 5% (7/139).CONCLUSIONSPostoperative sellar hematoma requiring reoperation is a rare phenomenon after transsphenoidal surgery, often presenting with visual loss and headache. Clot evacuation results in improvement in vision, but long-term endocrinopathy often ensues. Tumor diameter ≥ 30 mm and suprasellar extent are the most reliable risk factors. Close postoperative scrutiny should be given to patients at high risk.

Preoperative surgical risk assessment by evaluation of tumor blood flow using perfusion imaging by computed tomography in transsphenoidal surgery for pituitary adenoma

Background: Complete removal of large and giant pituitary adenomas (PAs) with suprasellar extension by transsphenoidal surgery (TSS) is sometimes difficult. Swelling and bleeding of a residual suprasellar tumor after incomplete removal via TSS can compress the surrounding neurovascular structures, resulting in postoperative complications. We measured PA blood flow by perfusion computed tomography (PCT) and assessed its usefulness in the preoperative prediction of postoperative hemorrhagic complications after TSS.Method: We performed PCT in 28 patients with PAs with suprasellar extension before TSS. Perfusion studies were performed with a 320-row multidetector computed tomography system. We measured cerebral blood volume (CBV) and cerebral blood flow (CBF) of the tumor (CBVt and CBFt) and normal-appearing white matter (CBVw and CBFw).Results: In all patients, CBVt was greater than CBVw. There was a statistically significant difference between CBFt and CBFw (p < 0.0001). The patients were divided into 2 groups according to CBFt: high CBF (CBFt > mean value) and low CBF (CBFt < mean value). There were no statistically significant differences between the 2 groups in terms of gender, age, maximum tumor diameter, suprasellar extension grade, and extent of resection. Two of the 7 patients in the high CBF group suffered from visual acuity deterioration and visual field stenosis due to postoperative hemorrhage, while no patients in the low CBF group did (p = 0.014).Conclusions: PCT may be useful in the preoperative prediction of postoperative residual tumor hemorrhage, which can be a hazardous postoperative complication after TSS for large and giant PAs.

Preoperative Surgical Risk Assessment by Evaluation of Tumor Blood Flow Using Perfusion Imaging by Computed Tomography in Transsphenoidal Surgery for Pituitary Adenoma

Background: Complete removal of large and giant pituitary adenomas (PAs) with suprasellar extension by transsphenoidal surgery (TSS) is sometimes difficult. Swelling and bleeding of a residual suprasellar tumor after incomplete removal via TSS can compress the surrounding neurovascular structures, resulting in postoperative complications. We measured PA blood flow by perfusion computed tomography (PCT) and assessed its usefulness in the preoperative prediction of postoperative hemorrhagic complications after TSS.Method: We performed PCT in 28 patients with PAs with suprasellar extension before TSS. Perfusion studies were performed with a 320-row multidetector computed tomography system. We measured cerebral blood volume (CBV) and cerebral blood flow (CBF) of the tumor (CBVt and CBFt) and normal-appearing white matter (CBVw and CBFw).Results: In all patients, CBVt was greater than CBVw. There was a statistically significant difference between CBFt and CBFw (p < 0.0001). The patients were divided into 2 groups according to CBFt: high CBF (CBFt> mean value) and low CBF (CBFt< mean value). There were no statistically significant differences between the 2 groups in terms of gender, age, maximum tumor diameter, suprasellar extension grade, and extent of resection. Two of the 7 patients in the high CBF group suffered from visual acuity deterioration and visual field stenosis due to postoperative hemorrhage, while no patients in the low CBF group did (p = 0.014).Conclusions: PCT may be useful in the preoperative prediction of postoperative residual tumor hemorrhage, which can be a hazardous postoperative complication after TSS for large and giant PAs.