Sellar Mass Lesions Research Articles

PMON155 Intraoperative, confocal laser endomicroscopy in pituitary surgery

Abstract Introduction Fluorescence imaging, such as confocal laser endomicroscopy (CLE) is a fast modality of image acquisition, which offers the possibility of early intraoperatively diagnosis and may be used to improve surgical decision making. Objective Within a 3-month prospective trail we used the Zeiss CONVIVO CLE in transspehnoidal surgery for different sellar pathologies. CLE is a miniaturized portable, handheld, fiber optic-based, confocal laser scanning microscope used in conjunction with a 488-nm excitable fluorophore. During the trial period we investigated feasibility, diagnostic accuracy and usability in terms of differential diagnosis in transsphenoidal surgeries of various pituitary tumours (15 pituitary adenomas, 2 craniopharyngiomas, 1 sellar metastasis). Method: Patients were randomly selected. Inclusion criteria were a pituitary macroadenoma or a sellar mass lesion exceeding 10 mm in diameter. A transsphenoidal approach was used and fluorescin was injected upon opening the sellar floor. CLE was carried out immediately after dural opening and during tumour resection, whenever possible. Additionally, the microprobe was used ex vivo with the resected tumor material. Histopathologic features of CLE images and H&E-stained permanent sections were assessed and compared postoperatively. Result: We were able to obtain CLE in-vivo images in all cases. Using the microscopic approach, no problems occurred regarding space for the microprobe. To obtain in-vivo images we had to rest the probe within a mount. Wiggling of the surgeon's hand, pulse synchronous movements of the mass and mild intraoperative venous bleeding led to inexact images. To overcome these problems, we used CLE as early as possible at the beginning of tumor resection to avoid bleeding interferences. Additionally we investigated resected tumor specimens ex-vivo within the OR. Since no bleeding and no interference with movements occurred image quality and acquisition time improved. Histopathologic features of CLE images and H&E-stained permanent sections showed, that except for the sellar metastasis, a diagnosis could be made on the basis of the intraoperative CLE images, whereas the ex-vivo images were more precise. Conclusion CLE is a potential imaging modality for transnasal assessment of skull base and pituitary lesions. It may help to establish an early diagnosis, to identify pituitary microadenomas, especially in cushing´s disease and to distinguish the various sellar pathologies. CLE is a fascinating possibility to verify a suspected diagnoses already intraoperatively. Presentation: Monday, June 13, 2022 12:30 p.m. - 2:30 p.m.

Nocardia farcinica pituitary abscess in an immunocompetent patient: illustrative case.

Pituitary abscess is a rare clinical entity, typically precipitated by Staphylococcus, Pseudomonas, or Aspergillus infection. Although Nocardia species-associated central nervous system abscesses have been documented in immunocompromised patients, no case of Nocardia pituitary abscesses has been previously reported. A 44-year-old man presented with hemoptysis and was found to have a cavitary right lung nodule, which was presumed histoplasmosis, prompting antifungal treatment. Several months later, he developed panhypopituitarism. Magnetic resonance imaging identified a pituitary mass, which subsequently underwent transsphenoidal endoscopic biopsy. Infectious workup was negative, and the patient was discharged on intravenous ertapenem therapy. Over several months, he developed progressive headaches, and updated imaging confirmed interval enlargement of the mass with new cavernous sinus invasion. He underwent repeat endoscopic biopsy, which yielded positive cultures for Nocardia farcinica and prompted successful treatment with trimethoprim-sulfamethoxazole and linezolid. The current study highlights a previously unreported clinical entity, the first pituitary abscess attributable to Nocardia sp. or N. farcinica, which arose in a young, immunocompetent individual. Although rare, atypical infections represent an important component in the differential diagnosis for sellar mass lesions.

Stereotactic radiosurgery for Rathke's cleft cysts: an international multicenter study.

Rathke's cleft cysts (RCCs) are sellar collections from an incompletely regressed Rathke's pouch. Common symptoms of RCCs can include headaches, visual loss, and endocrinopathy. Surgery is required in some cases of symptomatic or growing RCCs. Recurrence after surgery is common (range 10%-40%). Stereotactic radiosurgery (SRS) has been used in an attempt to control growth and symptoms, but outcomes are not well known. The authors sought to study the outcomes of RCCs following Gamma Knife surgery for both salvage and initial treatment. The outcomes of 25 patients with RCCs who underwent SRS between 2001 and 2020 were reviewed. Four patients received initial SRS and 21 were treated with salvage SRS. Diagnosis was based on imaging or histopathology. Cyst control was defined as stability or regression of the cyst. Kaplan-Meier analysis was used to determine time to recurrence and determine potential factors for recurrence. The respective median clinical follow-up and margin dose were 6.5 years and 12 Gy. Overall control was achieved in 19 (76%) of 25 patients, and 4 recurrences required further intervention. The average time to recurrence was 35.6 months in those RCCs that recurred. Visual recovery occurred in 14 (93.3%) of 15 patients and no new post-SRS visual deficits occurred. The presence of a pretreatment visual deficit was often an indicator of RCC regrowth. All 3 patients with pretreatment hyperprolactinemia experienced resolution after SRS. New endocrinopathy related to SRS was noted in 5 (20%) of 25 patients, all of which were thyroid and/or cortisol axis related. Upfront SRS was used in 4 patients. No new endocrinopathies or visual deficits developed after upfront SRS, and the 1 patient with a pretreatment visual deficit recovered. One of the 4 patients with upfront SRS experienced a recurrence after 7.5 years. SRS produced effective recovery of visual deficits and carries a low risk for new visual deficits. Cyst control was achieved in approximately three-fourths of the patients. Following SRS, patients without pretreatment visual deficits are less likely to have RCC regrowth. Endocrinopathy can occur after SRS, similar to other sellar mass lesions. Initial SRS shows the potential for long-term cyst control, with improvement of symptoms and a low risk for complications.

A Case of Unusual Initial Presentation of Panhypopituitarism From Metastatic Adenocarcinoma of Lungs

Introduction: Metastatic disease of the pituitary gland account for about 1 to 2 percent of the sellar masses with suprasellar extension. Approximately 7 percent of the patients are symptomatic with varying symptoms based on the location and extent of metastases. We present you a case in which patient presented with features of Panhypopituitarism starting with severe hypothyroidism in a previously undiagnosed lung cancer. The Patient also suffered with some serious complications such as diabetes insipidus, adrenal insufficiency and complete vision loss. Clinical Case: A 60 y/o male with a past medical history of Hypertension, Chronic Obstructive Pulmonary Disease, smoking 2 packs of cigarettes per day for 40 years, second-degree heart block requiring a pacemaker, Chronic kidney disease 3b presented to the emergency department with complaints of left ankle pain, swelling, and syncope. The Patient was admitted for further workup and Endocrinology was consulted for concerns of hypothyroidism with severe myxedema and adrenal insufficiency. CT head was done, which showed sellar and suprasellar mass lesion measuring 1.8 x 2.2 x 3.0 cm. Finding were confirmed on MRI. Pertinent labs were PTH 57.7 pg/ml, TSH 0.33 uIU/ml, Free T4 0.4 ng/dl, ACTH 8 pg/ml, Cortisol 2.5 ug/dl, FSH 0.5 MIU/ml, LH 0.1 MIU/ml, testosterone 4 ng/dl, Prolactin 17.7 ng/ml, Insulin-like GFBP-3: 2.1 mg/L. The Patient was started on high dose Hydrocortisone, IV Levothyroxine T4 and desmopressin for Diabetes Insipidus. The Patient complained of peripheral vision loss. Neurosurgery partially resected the sellar mass through the transsphenoidal approach. Histopathology came back with Metastatic adenocarcinoma. Further clinical course was complicated by complete vision loss from increase in the sellar mass size after 8 weeks. Patient received radiation therapy but unfortunately there was no significant improvement in the vision. Conclusion: This case highlights some serious complication from metastatic disease of Pituitary gland from lung cancer. There is a need for continued annual screening for dysfunction of the hypothalamic-pituitary axis to monitor therapy. This case also highlights the importance of widespread screening of smokers in accordance with the standard lungs cancer screening recommendations. This can potentially prevent some of the serious complications of metastatic lung disease. Reference: Ross, D. Cooper, D. Mulder, J. (2020, September) Central Hypothyroidism. https://www.uptodate.com/contents/central-hypothyroidismSnyder, P. Cooper, D. Martin, K. (2020, September) Causes, presentation, and evaluation of sellar masses. https://www.uptodate.com/contents/causes-presentation-and-evaluation-of-sellar-masses

Clinical efficacy of optical coherence tomography in sellar mass lesions: a meta-analysis.

Although optical coherence tomography (OCT) of the eyes has been studied to detect and monitor sellar masses, there is no recommendation for selecting the most effective measurement of OCT in clinical practice. Thus, we conducted a meta-analysis to examine the efficacy of OCT in sellar mass lesions. We conducted a literature search in PubMed and EMBASE through April 26, 2020. The primary outcomes were the thickness of the peripapillary retinal nerve fiber layer (pRNFL) and the macular ganglion cell complex (mGCC). The secondary outcomes included the thickness of the macular ganglion cell and inner plexiform layer (mGCIPL) and macular thickness. Random-effects models were used in all meta-analyses. Additionally, we conducted meta-regressions and subgroup analyses. We included 22 studies, involving 1347 eyes of patients and 1198 eyes of controls. When compared with the control group, the reductions in pRNFL, mGCC and macular thickness in the patient group were significantly different, whereas significant thinning of the mGCIPL was restricted to the nasal hemiretina. Furthermore, we found that before visual field (VF) defects occurred, significant thinning of the pRNFL and mGCC thickness could be detected by OCT. The change in OCT parameters also showed different patterns in different types of pituitary adenomas. Sellar mass lesions were associated with the changes in OCT measurements. The characteristic patterns of the OCT parameters may refine the diagnostic accuracy. Moreover, the alterations of OCT metrics before VF defects indicate the efficacy of OCT in early detection. Different types of pituitary adenomas may vary in OCT measurements, and their specific features warrant further research efforts.

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.

Utility of optical coherence tomography in the evaluation of sellar and parasellar mass lesions.

Anterior visual pathway compression is a common feature of sellar region masses. We review the visual pathway neuroanatomy pertaining to sellar and parasellar lesions and describe recent advances in optical coherence tomography (OCT) imaging that have provided a novel quantitative perspective in the evaluation and management of such patients. Ultrastructural measurements of optic nerve integrity using OCT, namely peripapillary retinal nerve fiber layer (pRNFL) and the ganglion cell and inner plexiform layer (GCIPL) thicknesses, have been shown to correlate with visual acuity and visual field deficits on perimetry in patients with compressive sellar region masses. In some cases, OCT can visualize early signs of anterior visual pathway involvement in the absence of clinically evident visual field loss or optic disc pallor. OCT is particularly useful when assessing patients who demonstrate less reliable visual field testing. Furthermore, there is growing awareness that pRNFL and GCIPL thinning preoperatively correlate with worse visual recovery following chiasmal decompression, highlighting the prognostic utility of OCT in this patient population. OCT provides a complimentary, yet critical, role in quantitatively assessing ultrastructural retinal injury in patients with sellar and parasellar lesions compressing the anterior visual pathway and should be incorporated into routine evaluation.

The solitary sellar plasmacytoma: a diagnostic challenge.

SummarySolitary sellar plasmacytomas are exceedingly rare and difficult to distinguish from other pituitary tumors. We report a case of a 62-year-old woman presenting with blurred vision of the right eye and tenderness of the right temporal region, which was interpreted as temporal arteritis. MRI revealed a pituitary mass lesion (20mm×14mm×17mm) without compression of the optic chiasm and her pituitary function was normal. Pituitary surgery was undertaken due to growth of the lesion, and histopathological examination showed a highly cellular neoplasm composed of mature monoclonal plasma cells. Subsequent examinations revealed no evidence of extrasellar myeloma. The patient received pituitary irradiation and has remained well and free of symptoms apart from iatrogenic central diabetes insipidus. Until now, only eight cases of solitary sellar plasmacytoma have been reported. Most frequent symptoms stem from compression of the cranial nerves in the cavernous sinus (III, IV, V), whereas the anterior pituitary function is mostly intact.Learning pointsA solitary plasmacytoma is a rare cause of a sellar mass lesion.The radiological and clinical features are nonspecific, but cranial nerve affection and intact pituitary function are usually present.The diagnosis is made histologically and has important therapeutic implications.

Ipilimumab-induced autoimmune hypophysitis: a differential for sellar mass lesions.

SummaryAutoimmune hypophysitis (AH) has been previously described in a typical demographic population, primarily women in the reproductive age group and perinatal period. The era of immune modulation using anti-cytotoxic T-lymphocyte-associated antigen 4 biological therapy (ipilimumab) against advanced cancers like metastatic melanomas has now resulted in a new form of hypophysitis being increasingly recognised under a spectrum of immune-related adverse events. Drug-related AH often presents with subtle symptoms and a pituitary mass, with the potential for fatality necessitating wide awareness and a high index of clinical suspicion given that it is usually treatable. We describe below two cases of AH within the last three months at our centre, which were treated with different regimens and produced good endocrine outcomes.Learning points AH is a new and defined clinical entity occurring as a side effect of ipilimumab, which enhances immune-mediated destruction of metastatic melanoma.It can present insidiously and have life-threatening complications related to hypocortisolism, hence a high index of clinical suspicion must be exerted by treating physicians, and seems to result in resolution of pituitary masses and variable improvements of pituitary function.Clinical improvement, radiological resolution of pituitary masses and variable normalisation of pituitary function are possible with early treatment with high-dose oral or i.v. steroids and hormone replacement therapy, although duration and dosing protocols are unclear at this stage.Ipilimumab should continue to be prescribed as treatment for metastatic melanoma; however, close clinical observation of patient's progress must be maintained while they are on this drug.Predictive factors for onset of AH remain unclear and it is imperative that AH is distinguished from pituitary metastases.Further studies are required to determine the safety of continuing therapy with ipilimumab in patients who have developed AH while on treatment.

Manifestation of a sellar hemangioblastoma due to pituitary apoplexy: a case report

IntroductionHemangioblastomas are rare, benign tumors occurring in any part of the nervous system. Most are found as sporadic tumors in the cerebellum or spinal cord. However, these neoplasms are also associated with von Hippel-Lindau disease. We report a rare case of a sporadic sellar hemangioblastoma that became symptomatic due to pituitary apoplexy.Case presentationAn 80-year-old, otherwise healthy Caucasian woman presented to our facility with severe headache attacks, hypocortisolism and blurred vision. A magnetic resonance imaging scan showed an acute hemorrhage of a known, stable and asymptomatic sellar mass lesion with chiasmatic compression accounting for our patient's acute visual impairment. The tumor was resected by a transnasal, transsphenoidal approach and histological examination revealed a capillary hemangioblastoma (World Health Organization grade I). Our patient recovered well and substitutional therapy was started for panhypopituitarism. A follow-up magnetic resonance imaging scan performed 16 months postoperatively showed good chiasmatic decompression with no tumor recurrence.ConclusionsA review of the literature confirmed supratentorial locations of hemangioblastomas to be very unusual, especially within the sellar region. However, intrasellar hemangioblastoma must be considered in the differential diagnosis of pituitary apoplexy.

Pituitary Magnetic Resonance Imaging for Sellar and Parasellar Masses: Ten-Year Experience in 2598 Patients

Sellar and parasellar masses present with overlapping clinical and radiological features ranging from asymptomatic incidental presentations and hormonal effects to compressive local mass effects. Pituitary masses are diagnosed with increased frequency with magnetic resonance imaging (MRI) advancements and availability, but indications and diagnostic outcomes of MRI screening for sellar lesions are not defined. Although pituitary adenomas are the most frequently encountered sellar mass lesions, other etiologies should be considered in the differential diagnosis of a sellar mass. The study was conducted at a tertiary pituitary center. This study was a retrospective review of 2598 subjects undergoing at least one pituitary MRI scan from 1999 to 2009. Prevalence and diagnosis of specific sellar and parasellar masses as screened by pituitary MRI. The most common indications for pituitary imaging, excluding known mass follow-up, were for evaluation of hyperprolactinemia or hypogonadism. A normal pituitary gland was reported in 47% of subjects undergoing pituitary MRI. The most common pituitary adenomas initially identified by MRI included prolactinoma (40%), nonfunctioning adenoma (37%), and GH adenoma (13%). Nonadenomatous sellar masses accounted for 18% of visible lesions, of which the most common were Rathke's cleft cyst (19%), craniopharyngioma (15%), and meningioma (15%). Metastases accounted for 5% of nonpituitary lesions and breast cancer was the most common primary source. Half of all pituitary MRI scans performed in a large patient population yielded no visible lesion. Nonadenomatous pituitary lesions should be considered in the diagnosis of sellar masses observed on MRI, and a high clinical suspicion is required to exclude the presence of a nonfunctioning pituitary adenoma.

Role of PROPELLER diffusion weighted imaging and apparent diffusion coefficient in the diagnosis of sellar and parasellar lesions

Objective To evaluate the role of the apparent diffusion coefficient (ADC) using periodically rotated overlapping parallel lines with enhanced reconstruction (PROPELLER) diffusion weighted imaging (DWI) in the differentiation between sellar and parasellar mass lesions. Materials and methods The study protocol was approved by our institutional review board. We retrospectively studied 60 patients with sellar and parasellar lesions who had undergone PROPELLER DWI on a 3-T MR imager. Conventional MRI findings were expressed as the ratio of signal intensity (SI) in the lesions to the normal white matter and the degree of contrast enhancement. ADC values were calculated as the minimum (ADC-MIN), mean (ADC-MEAN), and maximum (ADC-MAX). All patients underwent surgery and all specimens were examined histologically. Logistic discriminant analysis was performed by using the SI ratios on T1- and T2-weighted images (T1-WI, T2-WI), the degree of enhancement, and absolute ADC values as independent variables. Results ADC-MIN of hemorrhagic pituitary adenomas was lower than of the other lesions with similar appearance on conventional MRI (non-hemorrhagic pituitary adenomas, craniopharyngiomas, Rathke's cleft cysts; accuracy 100%); the useful cut-off value was 0.700 × 10 −3 mm 2/s. ADC-MAX of meningiomas was lower than of non-hemorrhagic pituitary adenomas (accuracy 90.3%; p < 0.01). ADC-MIN of craniopharyngiomas was lower than of Rathke's cleft cysts (accuracy 100%; p < 0.05). Conclusion As PROPELLER DWI is less sensitive to susceptibility artifacts than single-shot echoplanar DWI, it is more useful in the examination of sellar and parasellar lesions. Calculation of the ADC values helps to differentiate between various sellar and parasellar lesions.

Imaging spectrum of Erdheim–Chester disease—An extended report of two cases

Erdheim–Chester disease (ECD) is a rare sporadic systemic histiocytic disease of unknown etiology that affects multiple organ systems. We report two cases of ECD with musculoskeletal, orbital/periorbital involvement with dural and sellar mass lesions. Case 1 showed the unusual feature of ECD with pituitary mass and both anterior and posterior pituitary hypofunction. Proton magnetic resonance spectroscopy (1H MRS) was attempted from the orbital mass in case 2 by placing a voxel, in the orbital mass which showed isolated lipid resonance at 1.3 ppm that can be attributed to the presence of lipid-laden foamy macrophages seen in this condition. Application of 1H MRS in orbital lesions is unique to the present report. The magnetic resonance imaging (MRI) of brain and orbit including proton magnetic resonance spectroscopy (1H MRS) findings are discussed with a short review of the available literature.

Idiopathic granulomatous hypophysitis presenting as non-functioning pituitary adenoma: description of six cases and review of literature

The aim of this study was to analyse the complete profile and outcome of patients with idiopathic giant cell granulomatous hypophysitis. Six consecutive cases of idiopathic giant cell granulomatous hypophysitis were studied from 1993 to 2002. Headache and visual disturbances were the most frequent presenting symptoms. All patients had hypogonadism, four had hypoadrenalism and three were hypothyroid at presentation. None of them had diabetes insipidus preoperatively. A sellar mass with suprasellar extension on MRI with loss of the posterior pituitary 'bright spot' was a consistent observation in all patients. All patients underwent surgical excision of the mass lesion with histopathological confirmation of giant cell granulomatous hypophysitis. Other systemic granulomatous diseases were excluded by appropriate investigations. Postoperatively, all patients became hypothyroid and hypogonad, five patients had adrenal insufficiency, while two developed permanent diabetes insipidus. The clinical presentation of giant cell granulomatous hypophysitis is that of an expanding sellar mass lesion with a varying degree of endocrine dysfunction. Preoperative diagnosis of 'hypophysitis' is usually difficult; however, stalk thickening and loss of posterior pituitary 'bright spot' on MR imaging are clues to the diagnosis.

Space occupying processes of the sellar region with emphasis on tumor-like lesions

Most space occupying lesions of the sellar region are tumors (e.g., pituitary adenomas, craniopharyngiomas, but also meningiomas). In approximately 8% of patients with sellar mass lesions, non-neoplastic disorders (e.g., inflammations, cysts, hyperplasia) are causal. Cystic lesions of the pituitary region are classified as Rathke's cysts, colloid cysts, arachnoidal cysts and epidermoid cysts. These have to be differentiated from cystic craniopharyngeomas or cystic pituitary adenomas. Autochthonous inflammations (lymphocyctic hypophysitis, idiopathic granulomatous hypophysitis, xanthomatous and peritumorous hypophysitis) have to be distinguished from generalized diseases involving the pituitary (e.g., sarcoidosis, tuberculosis). Hyperplasia can result in a doubling of pituitary size. Lactotroph and corticotroph hyperplasia are the most common and may be of a diffuse and nodular type. The nodular form of pituitary hyperplasia may show transition into adenoma. Other hyperplasias may be found adjacent to adenomas or other tumours. Furthermore, vascular (aneurysms) and bony lesions (especially fibrous dysplasia) can appear as space occupying lesions of the sellar region.

Hipofisitis granulomatosa idiopática. Estudio morfológico e inmunohistoquímico de una observación

Inflammatory diseases of the pituitary gland constitute a group of interest because of their scarce frequency, because the disorder presents with symptoms of hypopituitarism and expanding sellar mass and because of their therapeutics implications. We present one case of idiopathic granulomatous hypophysitis, in a 55-years-old patient with daily headaches, panhypopituitarism and a sellar mass lesion. Granulomatous hypophysitis is characterized by granulomas with epithelioid histiocytes and multinucleated giant cells but also shows lymphocyte collections. With respect to immunohistochemistry our results show histiocytes (CD68+) and an heterogeneous inflammatory infiltrate (CD45RO+ y CD20+). We analyze the differential diagnosis with another granulomatous processes, infectious or not infectious, and with the histiocytosis. We examine the possible relation with the lymphocytic hypophysitis.

Current management of prolactinomas.

Prolactinomas constitute the largest group of pituitary adenomas in autopsy series. However, their relative incidence in recent surgical series is much less impressive since medical treatment with dopamine agonists is routinely employed, which in many cases leads to tumor shrinkage and normalization of prolactin levels. The clinical symptoms of hyperprolactinemia are menstrual dysfunction and galactorrhea in women and loss of libido and potency in men. Prolactinomas may present also as space occupying sellar mass lesions impinging on the adjacent structures like the pituitary gland, cavernous sinus and optic nerves. The standard primary treatment is medical by dopamine agonists. Prolactinomas are the prototype of tumors, the growth of which can be reliably and safely inhibited by specific drugs other than cytostatic chemotherapy. These unfortunately have side effects, like orthostatic hypotension, nausea and vomiting. The effects induced by dopamine agonists are suppressive but not tumoricidal. Thus, the therapeutic effect is only maintained as long as the drug is administered. Consequently. in most cases, treatment has to be continued life-long with a few exceptions, in whom normoprolactinemia persists even after discontinuation of dopamine agonists. Main indications of surgery in prolactinomas are intolerance of the medication, and tumors not responding to dopamine agonists. Occasionally, these may ultimately require radiation therapy. Remission rates in large series of surgically treated prolactinomas vary between 54% and 86%. In our consecutive series of 540 surgically treated prolactinomas, the normalization rate after transsphenoidal surgery basically depended on the preoperative prolactin levels, tumor size and extension. The remission rate of 82% in microprolactinomas with initial prolactin levels <200 ng/ml would even in small adenomas make one consider surgical treatment as an interesting alternative to long-term medical treatment.

Endocrine manifestations of Erdheim–Chester disease (a distinct form of histiocytosis)

Erdheim-Chester disease (ECD) is a disorder of unclear aetiology, characterized by exuberant histiocyte proliferation and a variable clinical course. We report the case of a woman with multi-organ involvement secondary to ECD. Central diabetes insipidus (CDI), hyperprolactinaemia, gonadotropin insufficiency and decreased insulin-like growth factor I levels were present, suggesting hypothalamic-pituitary dysfunction. The high-intensity signal of the posterior pituitary on T1-weighted images was absent on magnetic resonance imaging, but no sellar mass lesions or stalk thickening were apparent. Additionally, our patient had bilateral adrenal enlargement. Even though ECD is a rare cause of neuroendocrine dysfunction or adrenal enlargement, it should be considered in patients with these disorders in the setting of multiorgan disease.

Massive rupture of suprasellar dermoid cyst into ventricles

This 27-year-old man with a 2-year history of progressive visual loss in his left eye was admitted for an episode of sudden decreased level of consciousness and partial recovery with persistent confusion. Contrast-enhanced computerized tomography (CT) scanning revealed a sellar mass lesion with hypodense material floating on ventricular cerebrospinal fluid (CSF) (Fig. 1 left). Magnetic resonance (MR) images showed a tumor in the suprasellar region with the fat/fluid level spilling into the dilated ventricles (Fig. 1 center and right). The patient underwent a pterionofrontal craniotomy for resection of the tumor and a transient ventriculostomy. Postoperatively he made an excellent recovery. Intracranial dermoid cysts are rare congenital tumors that are usually benign and slow growing. Most arise in the midline basal cisterns and produce symptoms by interfering with spinal fluid pathways or vascular structures or by cyst rupture into the subarachnoid space or ventricular system. 1 The latter event releases keratin and cholesterol breakdown products that create irritation and may cause a chemical meningitis or ventriculitis that can be severe and long lasting. However, asymptomatic cases are not uncommon. 2,3 The imaging characteristics of dermoid cysts depend on the contents of the lesion. On CT scanning these lesions are demonstrated to be homogeneous, with an attenuation value similar to that of CSF. The hair and sebaceous content give the cyst a heterogeneous MR image signal, but the abundant lipid produces a high signal intensity on T 1-weighted images. Massive spilling of the sebaceous contents into the ventricles is shown as a fat/fluid level, with the fatty component floating on the fluid component.

Intrasellar Vascular Malformation Mimicking a Pituitary Macroadenoma

We report the case of a 61-year-old man with an unusual intrasellar vascular malformation, who presented with symptoms of hypopituitarism and whose neuroradiologic evaluation mimicked a pituitary macroadenoma. The histopathologic and radiologic findings are discussed. This rare lesion should be considered in the diagnosis of a sellar mass lesion.