Coexisting non-functioning pituitary macroadenoma and sellar-suprasellar lipoma: A case report and literature review

@#Intracranial lipomas are benign tumors that may occasionally be found in the suprasellar cistern while pituitary adenomas are far more common brain tumors. Pituitary adenomas may rarely coexist with other intracranial tumors in the sellar-suprasellar region. We share a unique case of a patient with coexisting non-functioning pituitary adenoma and sellar-suprasellar lipoma presenting with blurring of vision. We report a 55-year-old male presenting with a two-year history of blurring of vision with findings of a 2.7 x 3.0 x 3.2 cm homogeneously enhancing lobulated isointense mass on the sellar-suprasellar region. Hormonal workups revealed low cortisol and mildly elevated prolactin. He initially underwent endonasal transsphenoidal excision of the tumor which revealed to be a lipoma on histopathology. Due to minimal improvement of vision from the subtotal excision, he underwent repeat surgery through the transcranial approach which in turn showed a pituitary adenoma. The co-occurrence of two sellar-suprasellar tumors with different histology is rare, as most of the evidence is based on only a handful of case series. Intracranial lipomas result from persistence and abnormal differentiation of the meninx primitiva during the development of the subarachnoid cisterns. On the other hand, pituitary tumorigenesis is still largely unclear but appears to involve multiple tumor suppressor genes, oncogenes, cell cycle deregulation factors, and miRNAs. Given the differing pathogenesis of each tumor type, the coexistence may only be coincidental. The best surgical approach in this situation is unknown but the focus is on complete excision of the adenoma.

Craniopharyngioma and anterior central space of brain base / 中国医师进修杂志

Objective:To analyze the surgical (anatomical) approach of craniopharyngioma based on the concept of anterior central space of brain base.Methods:The clinical data of MRI images of 10 cases of craniopharyngioma who treated in Sanbo Brain Hospital, Capital Medical University from January 2021 to December 2021 were analyzed retrospectively. The brain cisterns involved in the growth of craniopharyngioma were analyzed from the perspective of anterior central space of brain base. Meanwhile, the surgical approach and the basis of selection were discussed.Results:Among all the surgical approaches that can reach the anterior central space of the brain base, the fronto-basal interhemispheric approach was the best.Conclusions:The concept of anterior central space of the brain base is a new observation and interpretation of the anatomy of the brain base from a new perspective, and then create a new concept of brain base surgery. As a new method to analyze the surgical anatomy of this area, its inclusion has important clinical significance.

Sellar/Suprasellar Germinoma Masquerading As Pituitary Adenoma

We report a case of sellar/suprasellar germinoma in a 17-year-old female which was misdiagnosed as pituitary adenoma both clinically and on imaging. Patient presented with severe headache of short duration and diminished visual acuity in left eye. Endocrinological work up revealed hyperprolactinemia and markedly reduced blood cortisol. MRI revealed homogenously enhanced intrasellar mass with suprasellar extension showing characteristic dumbbell conguration (snowman sign). Provisional diagnosis of pituitary adenoma was made and transsphenoidal resection of tumor was performed which on histology and immunohistochemistry proved to be germinoma. Sellar/suprasellar germinoma without diabetes insipidus is considered to be very difcult to diagnose preoperatively and the differentiation from pituitary adenoma based on MRI ndings was not possible in this case. However it is suggested that in a child with visual impairment, endocrine dysfunction and sellar/suprasellar mass a possibility of germ cell tumor should always be considered in differential diagnosis and a frozen section has to be performed to avoid excessive dissection.

A Case of Sporadic Suprasellar Hemangioblastoma Mimicking Meningioma

Hemangioblastoma (HBL) in the suprasellar region is very rare and a few cases have been reported. Suprasellar HBL without von Hippel-Lindau disease is much rarer. A 76-year old male patient presented progressively deteriorating visual disturbance. MRI demonstrated solid suprasellar mass of 20 mm in diameter, broadly based to planum sphenoidale and diaphragm sella and dural tail sign after the administration of gadolinium diethylene triamine penta-acetic acid (Gd-DTPA). Preoperative diagnosis was meningioma. Total resection of the tumor was not accomplished because of massive hemorrhage, and the histopathologic examination revealed the tumor to be HBL. The visual disturbance of the patient was not improved. The authors reviewed the literature and considered a differential diagnosis of suprasellar tumors and treatment of suprasellar HBL.

Emergency irradiation with 3.4 Gy/2f in sellar/suprasellar germinoma patients with rapid visual acuity decline / 中华医学杂志(英文版)

Background@#Rapid visual acuity (VA) decline was a common complaint in patients with sellar/suprasellar germinoma. In our hospital, 3.4 Gy/2f of emergency irradiation was applied to save patient VA and enable subsequent chemoradiotherapy. This study aimed to investigate the efficacy of emergency irradiation with 3.4 Gy/2f in patients with sellar/suprasellar germinoma who had rapid VA decline.@*Methods@#From January 2014 to December 2017, 33 patients with sellar/suprasellar germinoma who complained of VA decline within 3 months received 3.4 Gy/2f of emergency irradiation in Beijing Tiantan Hospital. The best-corrected VA (BCVA) and mean deviation (MD) were measured. Correlations between visual function change and clinical factors, including age at diagnosis, duration of VA decline, extent of tumor regression, serum level of tumor markers, were analyzed.@*Results@#Among 33 patients with sellar/suprasellar germinoma, the median diameter and volume of sellar/suprasellar lesions were 32 mm (range: 5–55 mm) and 12.9 cm3 (range 0.6–58.5 cm3), respectively. Data on pre- and post-emergency-irradiation BCVA were obtained in 32 patients. For the right eyes, BCVA was improved in 23 patients (71.9%), unchanged in 7 (21.9%), and worsened in 2 (6.2%); and for the left eyes, these numbers were 27 (84.4%), 4 (12.5%), and 1 (3.1%), respectively. In terms of the logarithm of the minimum angle of resolution (logarithm of the minimum angle of resolution = Log (1/BCVA) score, the improvement was significant in both eyes (P < 0.001). In terms of MD, six patients had paired data and the improvement was marginal in the right eyes (P = 0.068) and significant in the left eyes (P = 0.043). However, no clinical factor was found to have correlation with visual function improvement.@*Conclusion@#In sellar/suprasellar germinoma patients with VA decline, 3.4 Gy/2f of emergency irradiation was effective in improving visual function.

Pediatric sellar-suprasellar tubercular abscess:A case report and literature review

Abstract@#Sellar-suprasellar tuberculoma represents 1% of all intracranial tuberculomas, which can convert into a pituitary abscess. @*Objectives@#This paper aims to present a case of a common lesion in an uncommon site,discuss the challenges in diagnosis in terms of clinical manifestations, imaging and histologic findings, discuss aholistic approach to treatment,and enumerate identified clinical outcomes of reported cases in literature. @*Methodology@#This paper presents a case report of a sellar-suprasellar tubercular abscess,and reviews similar cases reported in literature. @*Results@#This is acase of a 16-year old female, Filipino, presenting with a chronic history of intermittent headache, fever, blurring of peripheral vision, polyuria, and increased sleeping time. On neurologic examination, the patient had bitemporal hemianopsia and decreased visual acuity on the right eye. Neuroimaging revealed a hypodense lesion at the sellar-suprasellar area with rim-enhancement on CT and MRI, and laboratory findings suggestive of panhypopituitarism. She underwent Right Pterional Craniotomy and intraoperatively there was note of a firm, yellowish capsule with intracapsular yellowish viscous fluid, which was positive for acid fast bacilli. Marsupialization of abscess was performed and hormonal replacement and anti-tubercular medications were given. @*Conclusion@#Tuberculoma in the sellar-suprasellar area, can impinge on the optic chiasm, producing bitemporal hemianopsia and pituitary dysfunction.It presents like other sellar-suprasellar masses with non-specific symptoms and these masses share similar features on cranial CT scan. Due to the complexity of the disease, treatment of sellar-suprasellar TB requires integrated management of an infectious disease expert, neurologist, neurosurgeon, endocrinologist, and adolescent medicine specialist. Outcomes of four other cases found in literature were generally good after aspiration or drainage of the abscess followed by TB treatment for 15 to 18 months with resulting improvement invision, marked reduction in the size or complete resolution of the mass, but with one case having loss of pituitary function.

Endoscopic Endonasal Approach for Suprasellar Lesions in Children: Complications and Prevention

The endoscopic endonasal approach (EEA) has been popularized in adults and has been applied to an expanding range of surgical modules and indications in this population. However, its clinical application in pediatric neurosurgery has been impeded by the differences in anatomical features and the relatively low incidence of diseases to which it is applicable. In this review article, we mainly discuss the surgical indications, feasibility, and complications of EEA for suprasellar lesions in children based on a review of the literature, focusing especially on the age-related anatomical features of the nasal cavity, various pathologic entities, and the impact of EEA on long-term craniofacial growth.

Sellar-Suprasellar Extraventricular Choroid Plexus Papilloma : A Case Report and Review of the Literature

Choroid plexus papillomas (CPPs) are relatively rare neuroectodermal tumors that develop from choroid plexus epithelial cells and are usually restricted to the ventricles. Extraventricular CPPs are very unusual and can be difficult to diagnose and treat. A 50-year-old male patient was admitted to our clinic complaining of headache and visual deterioration. Neurological examination found no abnormalities except decreased light perception and secondary optic atrophy in the left eye. Endocrine testing revealed normal levels of hormones produced by the pituitary and target glands. Magnetic resonance imaging of the brain revealed a huge regular-shaped lesion in the sellar-suprasellar region occupying the sella turcica and extending into the suprasellar cistern and planum sphenoidale. The lesion was completely excised by microsurgery via an ordinary left-sided pterional approach. Histopathology identified the lesion as a choroid plexus papilloma. Following the case report, literature on the origin, differential diagnosis, and treatment of this rare tumor is reviewed.

Expanded Endoscopic Transnasal Approach to the Chordoid Glioma of the Third Ventricle : The First Case Ever Reported

Chordoid glioma of the third ventricle is a rare and challenging tumor to surgery because of its unique anatomical location and its close juxtaposition to the neurovascular structures and hypothalamus. The authors report a case of chordoid glioma of the third ventricle in a 43-year-old woman, who presented with headache and somnolence. The tumor was approached by endoscopic transnasal technique with a favorable result. Histopathologic examination disclosed a neoplastic tissue composed of eosinophilic epithelioid cells, mucinous, periodic acid Schiff-diastase positive, extracellular matrix, and scattered lymphoplasmacytic infiltrates. The best treatment option remains controversial. Customarily, the surgical route to remove chordoid glioma is transcranial; however, the undersurface of the optic chiasm and optic nerves preclude an adequate surgical visualization. In contrast, an expanded endoscopic transnasal approach provides a direct midline corridor to this region without any brain retraction.

A Case of Meningioma Masquerading as Ischemic Optic Neuropathy

PURPOSE: We report a case of meningioma diagnosed as ischemic optic neuropathy. CASE SUMMARY: A 45-year-old women presented with decreased visual acuity in her left eye without eye movement pain. Her best corrected visual acuity (BCVA) in that eye was 0.3. She showed a relative afferent pupillary defect, abnormal color vision test, and inferior visual field defect in her left eye. The optic disc showed slight blurring superiorly and pallor temporally. Fluorescein angiography showed choroidal filling defect, and ischemic optic neuropathy was suspected. The carotid artery sonography showed normal results. The BCVA of the left eye was 0.5 after 7 months. The visual field test and color vision test were improved after 7 months. The optic disc was pale. The magnetic resonance imaging was performed because of persistent headache, and that showed a suprasellar mass that was removed by surgical resection and diagnosed as meningioma on biopsy. CONCLUSIONS: The possibility of compressive optic neuropathy should be considered in presumed ischemic optic neuropathy if the patient complains of persistent headache.

Sporadic Hemangioblastoma in the Pituitary Stalk: A Case Report and Review of the Literature

Hemangioblastomas in the pituitary stalk are especially rare. Most pituitary stalk hemangioblastomas reported in the literature were associated with von Hippel-Lindau (VHL) disease. Here, we report only the 3rd case of sporadic pituitary stalk hemangioblastoma diagnosed in a 60-year-old woman. Despite the danger of potential complications due to excessive vascularity or proximity to important neural structures, the tumor in our case was successfully removed while preserving pituitary function. In this case, complete surgical excision was shown to be an effective treatment option for symptomatic pituitary stalk hemangioblastoma, and we suggest careful evaluation of any highly enhancing mass with a signal void in the pituitary stalk preoperatively, even if no VHL disease is evident.

Transventricular Endoscopic Management of a Suprasellar Arachnoid Cyst Causing Precocious Puberty.

Suprasellar cysts arachnoid cysts (SSAC) are relatively uncommon making up less than 2% of all arachnoid cysts. Suprasellar arachnoid cyst causing precocious puberty is infrequent. The pathogenesis remains unknown in the majority of patients with precocious puberty. Endoscopic ventriculocystocisternostomy procedure is an effective and minimally invasive method in the treatment of these cysts. This report describes a 7-year old male who had a huge intracranial arachnoid cyst with initial symptoms and signs of sexual precocity. Magnetic resonance image (MRI) of the brain revealed the presence of large suprasellar cystic lesion.

Suprasellar salivary gland-like pleomorphic adenoma.

Suprasellar salivary gland-Like pleomorphic adenoma is not a common disease and seldom reported so far. We are reporting a case of a 23-year-old man with recurrent suprasellar salivary gland-like pleomorphic adenoma, who underwent an operation of subtotal, subfrontal resection under the wrong pathology diagnosis of benign teratoma in another hospital 4-year-ago. Four years later, he was admitted to our hospital for additional visual loss of the right eye (left, 1.0; right, 0.4) resulting from tumor regrowth. Magnetic resonance imaging revealed that suprasellar extension and compressed optic chiasm resulted in visual disturbance of the patient. The tumor was totally excised and histological examination evidenced the pathological features of intrasellar salivary gland-like pleomorphic adenoma. The patient did not receive any further treatment and he is free from tumor recurrence for 30 months after the operation. From this point of view, clinical prognosis of intrasellar salivary gland-like pleomorphic adenoma was good after total surgical resection.

Adult-Onset Sellar and Suprasellar Atypical Teratoid Rhabdoid Tumor Treated with a Multimodal Approach: A Case Report

We report a very rare case of sellar and suprasellar atypical teratoid rhabdoid tumor (ATRT) in a 42-year-old female patient. The tumor was removed subtotally with a transsphenoidal approach. Histopathologic study showed rhabdoid cells with prominent nucleoli and abundant cytoplasm. Immunohistochemistry for INI1 was completely negative in the tumor cells, consistent with ATRT. After surgery, she received radiotherapy including spinal irradiation with proton beam therapy and subsequent chemotherapy, with no evidence of recurrence for more than 2 years. Up to date, this is the 8th case of an adult-onset ATRT in the sellar or suprasellar region. Despite its rarity, ATRTs should be considered in the differential diagnosis of an unclear malignant sellar or suprasellar lesion in adult patients and the treatment strategies for adult ATRT patients could be differentiated from those of pediatric ATRT patients.

A Suprasellar Cystic Germ Cell Tumor Initially Diagnosed as an Arachnoid Cyst

We report here the case of a suprasellar cystic germ cell tumor (GCT) initially diagnosed as an arachnoid cyst. A 10-year-old boy experienced headache, dizziness, and diplopia, and was shown to have an approximately 2 cm suprasellar cyst. Two months after endoscopic third ventriculostomy was performed, a 5-6 cm cystic mass with an internal enhancing component was observed in the suprasellar cistern. Serum human chorionic gonadotropin levels were slightly increased in the serum and cerebrospinal fluid (55 and 162 IU/L, respectively) but were strikingly elevated in the cystic fluid (14,040 IU/L). The patient showed complete remission, with only a very small cystic lesion remaining after surgery, chemotherapy, and radiation treatment for a suprasellar mixed GCT. However, follow-up after treatment was complicated by moyamoya syndrome and cerebral infarction. GCT can be considered as a rare differential diagnosis in the case of a suprasellar cystic mass. Evaluation of tumor markers and close follow-up will be necessary.

Steroid-induced Spinal Epidural Lipomatosis after Suprasella Tumor Resection

Spinal epidural lipomatosis (SEL) is an abnormal localized accumulation of fat tissues in the epidural space. It is strongly related with steroid administration. The symptoms of SEL are various and range from back pain to paraplegia. In severe cases, decompressive laminectomy is the choice of treatment. A 32-year-old woman who had been under long-term steroid administration after suprasellar tumor resection was admitted for both leg radiating pain and weakness. She was diagnosed with SEL and had a decompressive laminectomy. During the operation, we found the nerve roots were compressed by epidural fat tissues and engorged vessels. After the operation, her radiating pain was relieved and motor weakness was improved.

Multiple Atraumatic Osteoporotic Vertebral Fractures in an Adolescent with Suprasellar Germinoma: A Case Report

We present the case of a patient with multiple atraumatic osteoporotic vertebral fractures in an adolescent with suprasellar germinoma and also review of relevant literature. The patient suffered from a rare adolescent brain tumour with common complications which are often overlooked and give rise to significant morbidity. Suprasellar germinoma is an intracranial neoplasm, that in addition to its rarity, has variable clinical presentation. Despite appropriate treatment and good outcome, tumour related morbidity is still of concern for these patients.

Bromocriptine for Control of Hyperthermia in a Patient with Mixed Autonomic Hyperactivity after Neurosurgery: A Case Report

Mixed autonomic hyperactivity disorder (MAHD) among patients with acquired brain injury can be rare. A delayed diagnosis of MAHD might exacerbate the clinical outcome and increase healthcare expenses with unnecessary testing. However, MAHD is still an underrecognized and evolving disease entity. A 25-yr-old woman was admitted the clinic due to craniopharyngioma. After an extensive tumor resection, she complained of sustained fever, papillary contraction, hiccup, lacrimation, and sighing. An extensive evaluation of the sustained fever was conducted. Finally, the cause for MAHD was suspected, and the patient was successfully treated with bromocriptine for a month.

A Case of Ectopic Rathke's Cleft Cyst in the Prepontine Cistern

A Rathke's cleft cyst (RCC) is a benign pituitary cyst derived from the remnant of Rathke's pouch, and usually presents as an intrasellar lesion with varying degrees of suprasellar extension. However, to date, a description of a primary prepontine RCC with no intrasellar component has not been reported. The author describes an exceptional case of a symptomatic RCC located behind the sella turcica in a 41-year-old woman who presented with severe headache. The author also provides an embryological hypothesis of the development of an ectopic RCC, with a special emphasis on radiologic characteristics.

Cupped disc with normal intraocular pressure: The long road to avoid misdiagnosis.

We present a series of six patients who had been receiving treatment for normal tension glaucoma (NTG; five patients) or primary open angle glaucoma (one patient). All of them were found to have optic neuropathy secondary to compression of the anterior visual pathway. Even though uncommon, compression of the anterior visual pathway is an important differential diagnosis of NTG. Diagnosis of NTG should be by exclusion. Here the possible causes of misdiagnosis are discussed. We present an approach to distinguish glaucomatous from nonglaucomatous optic neuropathy. The article also emphasizes how important it is for the clinicians to consider the total clinical picture, and not merely the optic disc morphology, to avoid the mismanagement of glaucoma, especially the NTG.