Craniopharyngioma: A comprehensive review of the clinical presentation, radiological findings, management, and future Perspective.

Craniopharyngioma (CP) is a rare intracranial tumor arising from the epithelial remnants of Rathke's pouch, most frequently originating in the sellar/parasellar region. Histologically, CP is a benign low-grade tumor (WHO grade 1) with two distinct phenotypes: adamantinomatous CP (ACP) and papillary CP (PCP). Craniopharyngioma constitutes 1-3% of all primary intracranial tumors in adults and 5-10 % of intracranial tumors in children. The annual incidence ranges from 0.13 to 2 per 100,000 population per year with no gender predilection. Due to its unique anatomical locations, the most frequently reported clinical manifestations are headache, visual impairment, nausea/vomiting, and endocrine deficiencies resulting in sexual dysfunction in adults and growth failure in children. Growth hormone deficiency is the most predominant endocrinological disturbance associated with craniopharyngioma. Computed tomography (CT) is gold standard to detect calcifications in CP tissue (found in 90 % of these tumors). Magnetic Resonance Imaging (MRI) further characterizes craniopharyngiomas and helps to narrow down the differential diagnoses. In almost all craniopharyngioma cases, surgery is indicated to: establish the diagnosis, relieve mass-related symptoms, and remove as much tumor as is safely possible. Recent neurosurgical technical advances, including innovative surgical approaches, detailed radiotherapy protocols, targeted therapy, replacement of lost hormonal functions and quality of life all have the potential to improve the outcome of patients with craniopharyngioma. In this article, we present extensive literature on craniopharyngioma clinical presentation, radiological findings, management, and future prospective. The present article helps to identify further research areas that set the basis for the management of such a complex tumor.

Giant Ganglioneuroma of the Lumbar Spine: A Rare Cause of Radiculopathy.

Background: Ganglioneuroma (GN) is a rare, benign tumor that originates from neural crest cells and can potentially affect any anatomical site within the sympathetic nervous system. Typically, GNs are more frequently reported in children and young adults, with a slightly higher prevalence in females. We are reporting a rare case of a giant lumbar spine ganglioneuroma by outlining the clinical presentation, radiological finding, management, and outcome. Case Description. A 37-year-old female presented with low back pain radiating to the right lower limb for few years. Neurological examination revealed bilateral lower limb hyperreflexia (+3). Lumbar spine CT and MRI revealed a right paravertebral soft tissue lesion with heterogeneous signal intensity and enhancement at L1 to L3. The patient underwent complete resection of the lesion via a retroperitoneal approach. The surgery was uneventful. The histopathological sections were suggestive of mature ganglioneuroma. She was discharged in stable condition with follow-up at neurosurgery clinic. Conclusion: Giant ganglioneuromas are rare, benign tumors of sympathetic neurons. Complete surgical resection is the most effective therapeutic option for ganglioneuroma to avoid recurrence. Given the benign nature of ganglioneuroma, chemotherapy and radiotherapy tend to have a limited role following surgical resection.

Calvarial multiple myeloma: Raindrop skull.

Background: The "Raindrop skull" appearance represents the multiple punched-out and lytic lesions hitting a surface and creating a scattered splash pattern. Case Description: A 73-year-old female presented with multiple painless lumps over the forehead and head. The patient reported unintentional weight loss, fatigability, loss of appetite, fever, night sweats, and back pain for seven months (B symptoms). The examination revealed multiple, nonmobile, calvarial lesions with defined borders, measuring approximately 1 × 1 cm. Laboratory investigations of serum-free light chains showed a free kappa level of 12.91 mg/L, a lambda level of 4549.28 mg/L, and a free kappa/lambda ratio of 0.00. Radiological imaging of the skull and brain showed a "raindrop skull" appearance and multiple calvarial osteolytic lesions. The patient underwent a right superior iliac crest bone marrow aspirate and trephine biopsy. The laboratory and histopathological sections were compatible with multiple myeloma. A diagnosis of multiple myeloma (free light chain lambda) was rendered. Conclusion: Calvarial multiple myeloma is rare and requires a high index of suspicion to diagnose. "Raindrop skull" appearance is pathognomonic of calvarial multiple myeloma.

Spinal dural arteriovenous fistula: a comprehensive review of the history, classification systems, management, and prognosis.

Spinal dural arteriovenous fistulas account for the majority of spinal vascular malformations. They are typically located in the thoracolumbar region and are diagnosed in the middle-aged and elderly populations. Although spinal dural arteriovenous fistulas have been postulated to be acquired, their exact development remains uncertain. Typically, the arteriovenous shunt is situated close to the spinal nerve root, inside the dura mater, where the blood from the radiculomeningeal artery and radicular vein intermix. Throughout history, there have been multiple classification systems of spinal arteriovenous shunts since 1967. Those were mainly based on the evolution of diagnostic studies as well as the treatment of these lesions. Such classification systems have undergone significant changes over the years. Unlike intracranial dural arteriovenous fistula, spinal dural arteriovenous fistula is progressive in nature. The neurological manifestations, due to venous congestion, tend to be insidious as well as non-specific. These include sensory deficits, such as paresthesia, bilateral and/or unilateral radicular pain affecting the lower limbs, and gait disturbances. Spinal dural arteriovenous fistulas can be suspected on magnetic resonance imaging/magnetic resonance angiography and confirmed by digital subtraction angiography (DSA). The management includes surgery, endovascular therapy, and in selected cases, radiotherapy. The treatment goal of spinal dural arteriovenous fistula is to halt the progression of the disease. The prognosis depends on both the duration of symptoms as well as the clinical condition prior to therapy. The present article comprehensively reviews the pathophysiology, changes in classification systems, natural history, clinical manifestations, radiological features, management, and prognosis.

Invasive Giant Prolactinoma.

A 64-year-old man presented with an 8-year history of decreased libido, impotence, and blurred vision. The neurologic examination revealed a mild left facial weakness, visual acuity of (20/60), and right homonymous hemianopia. Laboratory investigation of serum prolactin was 7896 ng/mL. Testosterone, free T4, and adrenocorticotropic hormone were all decreased. Brain magnetic resonance imaging showed a giant sellar/suprasellar lesion, compressing on the brainstem and extending to the cerebellopontine angle. A diagnosis of panhypopituitarism secondary to a giant macroprolactinoma was made. The patient was started on hormonal replacement therapy and underwent craniotomy and tumor resection. The histopathologic findings were compatible with prolactinoma. Invasive giant prolactinomas are defined as 1) tumor size of >4 cm in diameter; 2) serum prolactin of >1000 ng/mL; and 3) mass effect or hyperprolactinemia-induced symptomatology. The management of invasive giant prolactinoma commonly comprises a multimodal approach of both medical treatment and surgical intervention.

Concurrent medulloblastoma and cardiac fibroma: a rare presentation of Gorlin-Goltz syndrome.

BACKGROUND: Gorlin-Goltz syndrome is a rare autosomal dominant disorder resulting from PTCH1 gene mutation and presents with variable clinical manifestations. The co-occurrence of medulloblastoma and cardiac fibroma in Gorlin-Goltz syndrome is extremely rare. The present article discusses a patient diagnosed with Gorlin-Goltz syndrome and concurrent medulloblastoma and cardiac fibroma. CASE PRESENTATION: A 19-month-old boy transferred to our hospital after a radiological finding of posterior fossa lesion and hydrocephalus. A pericardial mass was noted after persistent arrhythmias. Both tumors were excised for definitive management. The histopathological sections were diagnostic of desmoplastic nodular medulloblastoma, WHO grade 4 and cardiac fibroma. Molecular and genetic investigations confirmed a pathogenic variant of PTCH1 gene, suggestive of autosomal dominant Gorlin-Goltz syndrome. CONCLUSION: Co-occurrence of medulloblastoma and cardiac fibroma is extremely rare and poses a management dilemma. Genetic counseling and antenatal screening are of utmost importance to early detect and manage patients with Gorlin-Goltz syndrome.

Diffuse Calvarial Hyperstosis.

Calvarial hyperstosis can be an idiopathic benign finding or secondary to a metabolic pathology. We herein describe a case of diffuse calvarial hyperstosis. A 26-year-old man known to have end-stage renal disease on regular hemodialysis, tertiary hyperparathyroidism, extensive brown tumors, and severe developmental impairment with skeletal deformities was referred to us for macrocephaly. On examination, the patient was chairbound, with speech and motor developmental delay, and frontal bossing. Brain computed tomography revealed diffuse hyperstosis of the calvarium and facial bones expansion with multiple sclerotic and lytic areas, causing subsequent narrowing of the basilar skull foramina. Brain magnetic resonance imaging demonstrated an extensive expansile bone marrow abnormality in the calvarium and skull base. There was mild generalized prominence of cortical sulci and ventricular system. The findings were in keeping with his known hypermetabolic state and tertiary hyperparathyroidism. The patient was managed conservatively with regular follow-up in the clinic.

Giant Craniopharyngioma.

A 6-year-old girl presented with moderate-intensity headache, frequent vomiting, visual disturbance, and left-sided decreased hearing for 7 months. The neurologic examination revealed a right upper motor neuron facial nerve palsy, left-sided 4-mm sluggish pupil (right: 3-mm reactive), and unsteady gait. Fundoscopy was notable for bilateral papilledema. Brain magnetic resonance imaging with contrast demonstrated a giant multiloculated suprasellar cystic lesion (9.7 × 10.5 × 7.6 cm). It extended to the left anterior cranial fossa, both middle cranial fossae, and posterior fossa prepontine region with consequent effect on the brainstem and moderate hydrocephalus. The patient underwent a right frontal external ventricular drain insertion and left frontotemporal craniotomy and tumor resection. Histopathologic sections were compatible with adamantinomatous craniopharyngioma. Giant craniopharyngiomas have rarely been reported. This article presents the clinical and radiologic outcomes of a patient with a giant craniopharyngioma.

Intracranial dural arteriovenous fistula: a comprehensive review of the history, management, and future prospective.

Dural arteriovenous fistulas (DAVF) are abnormal acquired intracranial vascular malformations consisting of pathological connections located within the dura between the pial arteries and the veno vasora, comprising the walls of the dural sinuses, bridging veins, or transosseous emissary veins. Dural arteriovenous fistulas are distinguished from arteriovenous malformations by their arterial supply from the vessels that perfuse the dura mater and lack of a parenchymal nidus. They are most commonly situated at the transverse and cavernous sinuses. The mechanism of development behind dural arteriovenous fistula can be explained by the molecular and anatomical factors. Multiple classification systems have been proposed throughout history including; Djindjian and Merland, Cognard, and Borden classification systems. The aggressiveness of the clinical course in intracranial dural arteriovenous fistula can be predicted through the angiographic patterns of venous drainage, more specifically, the presence of cortical venous drainage, the presence of venous ectasia, and the aggressiveness of clinical presentation. Intracranial dural arteriovenous fistulas might be discovered incidentally. However, if symptomatic, the clinical presentation ranges from mild neurological deficits to severe, lethal intracranial hemorrhage. Angiography is the imaging of choice to investigate, diagnose, and plan treatment for intracranial dural arteriovenous fistula. The management algorithm of intracranial dural arteriovenous fistula can be broadly divided into conservative, surgical, endovascular, and/or radiosurgical options. With the advent of endovascular therapies, surgery has fallen out of favor for managing intracranial dural arteriovenous fistulas. In the present article, the pathophysiology, classifications, natural history, clinical manifestations, radiological features, management, and complications are comprehensively reviewed.

Rhino-Orbital-Cerebral Mucormycosis: A Rare Complication of Uncontrolled Diabetes.

Introduction: Fungal infection of the central nervous system has become more common over the past two decades. It is frequently diagnosed in patients with underlying pathological conditions. We herein report a case of rhino-orbital-cerebral mucormycosis by outlining the clinical presentation, radiological images, histopathological findings, management plan, and its clinical outcome. Case Description. A 47-year-old man, known to have type 2 diabetes mellitus, presented with severe headache involving the left side of the face, numbness along the left V2 trigeminal nerve, ptosis and dryness of the left eye, short-term memory loss, and right hand numbness. He had a social history of being a bee farmer for which he was exposed to bee stings several times in the past. Neuroradiological imaging showed a left temporal ring-enhancing lesion, suggestive of abscess. The patient underwent craniotomy and resection of the lesion. The histopathological evaluation was suggestive of cerebral mucormycosis, fungal sinusitis, and invasive skull base mucormycosis. Conclusion: Rhino-orbital-cerebral mucormycosis is a fulminant fungal infection commonly diagnosed in patients with uncontrolled diabetes. Early diagnosis with radiological and histopathological evaluation is required to identify patients at risk of rhino-orbital-cerebral mucormycosis.

Giant Colloid Cyst.

Giant colloid cysts are defined as cysts of more than 3 cm in maximal diameter. Few cases of giant colloid cysts have been reported in the literature. We herein describe a giant colloid cyst. A 15-year-old female presented with headache, blurred vision, and episodic behavioral changes for 3 months. Neurological examination was limited due to agitation and confusion. Fundoscopy was notable for bilateral papilledema. Brain computed tomography revealed a giant third-ventricular lesion, causing obstructive hydrocephalus. The patient underwent urgent ventriculoperitoneal shunt insertion initially and then cyst excision. The histopathological sections of the lesion were compatible with a colloid cyst. Five years following surgical resection, a brain magnetic resonance imaging did not demonstrate any evidence of residual or cyst recurrence. To our knowledge, giant colloid cysts have been rarely reported and pose a management dilemma in the literature. The present article highlights the symptomatology, radiological findings, and outcome of a giant colloid cyst.

Primary spinal cord glioblastoma: A rare cause of paraplegia.

Background: Primary spinal glioblastomas are extremely rare neoplasms and account for only 0.2% of glioblastoma cases. Due to the rare incidence of spinal cord glioblastoma in the literature, its natural history/ outcome remains undetermined. The present article describes the clinical presentation, radiological/pathological characteristics, and outcome of the primary spinal cord glioblastoma. Case Description: Two young patients initially presented with paresis that rapidly progressed to paraplegia. Nondermatomal sensory deficits were also noted, mainly affecting the lower limbs. Neuroradiological imaging revealed an extensive intramedullary spinal cord lesion, with no evidence of concurrent intracranial space-occupying lesions. Thoracic laminectomy, followed by tumor debulking and/or biopsy, was performed. The histomorphology was suggestive of glioblastoma, the World Health Organization grade 4 (Isocitrate Dehydrogenase-wild type). They were discharged in stable condition and were started on chemoradiotherapy, with clinicoradiological follow-up. One patient passed away after 9 months of initial presentation. The other patient was alive at 6-month follow-up. Conclusion: Primary spinal glioblastoma is a rare and challenging tumor. Patients commonly present with a progressive paresis, resulting in paraplegia, regardless of the surgical resection extent, and received adjuvant chemotherapy. Therefore, primary spinal cord glioblastoma should be considered in patients reporting a rapid lower limb weakness with neuroradiological evidence of extensive, exophytic intramedullary lesion of the spine. A biopsy-proven histopathological diagnosis is of indisputable importance to establish the final diagnosis and plan treatment options.

Pediatric craniocervical fusion: predictors of surgical outcomes, risk of recurrence, and re-operation.

PURPOSE: Craniocervical junction abnormalities include a wide variety of disorders and can be classified into congenital or acquired. This study aimed to review the surgical outcome of pediatric patients who underwent craniocervical and/or atlantoaxial fusion. METHODS: This is a retrospective cohort study including all pediatric patients (≤ 18 years) who underwent craniocervical and/or atlantoaxial fusion between 2009 and 2019 at quaternary medical city. RESULTS: A total of 25 patients met our criteria and were included in the study. The mean age was 9 years (range: 1-17 years). There was a slight female preponderance (N = 13; 52%). Most patients (N = 16; 64%) had non-trauamatic/chronic causes of craniocervical instability. Most patients presented with neck pain and/or stiffness (N = 14; 56%). Successful fusion of the craniocervical junction was achieved in most patients (N = 21; 84%). Intraoperative complications were encountered in 12% (N = 3) of the patients. Early postoperative complications were observed in five patients (20%). Five patients (20%) experienced long-term complications. Revision was needed in two patients (8%). Older age was significantly associated with higher fusion success rates (p = 0.003). The need for revision surgery rates was significantly higher among younger age group (3.75 ± 2, p = 0.01). CONCLUSIONS: The study demonstrates the surgical outcome of craniocervical and/or atlantoaxial fusion in pediatric patients. Successful fusion of the craniocervical junction was achieved in most patients. Significant association was found between older age and successful fusion, and between younger age and need for revision surgery.

Metastatic brain lesion as the initial presentation of follicular thyroid carcinoma.

Background: Metastatic brain lesions, of thyroid origin, are rare manifestations of differentiated thyroid cancer, with papillary thyroid carcinoma being the most common subtype. Considering the rarity of metastatic follicular thyroid carcinoma to the brain, the present article outlines its clinical presentation, neuroradiological findings, pathological features, and outcome. Case Description: A 52-year-old female presented with a 6-month history of progressive and holocephalic headache. Examination revealed a tracheal deviation to the left side due to an enlarged goiter. Brain CT scan showed a right occipital, slightly hyperdense lesion associated with a 0.4 cm midline shift to the left side. Brain MRI demonstrated a right occipital, avidly-enhancing, extra-axial lesion with disproportionate and extensive vasogenic edema. As the lesion was solitary, the patient underwent craniotomy and tumor resection. Histopathological examination revealed a tumor consistent of small follicles, composed of uniform round nuclei without papillary thyroid carcinoma nuclear features, suggestive of metastatic follicular thyroid carcinoma to the brain. Postoperatively, the patient was neurologically intact. She was discharged in a stable condition with laboratory/ radiological investigations and follow-up at neurosurgery, endocrine, radiotherapy, and thyroid surgery clinics. Conclusion: Follicular thyroid carcinoma may rarely metastasize to the central nervous system. A high index of suspicion is required to identify patients with thyroid cancer who initially present with neurological manifestations. Complete surgical resection of the metastatic brain lesion is safe, feasible and is associated with a prolonged overall survival.

Sellar xanthogranuloma: A diagnostic challenge.

Background: Sellar xanthogranulomas are rare intracranial lesions comprising <1% of all sellar lesions. They were described as a separate entity by the World Health Organization in 2000. Because of the paucity of sellar xanthogranuloma cases reported in the literature, they remain a diagnostic challenge with indefinite origin, clinical course, and outcome. The present study reports a case of sellar xanthogranuloma describing the clinical presentation, radiological/pathological characteristics, and outcome. Case Description: A 43-year-old female, known to have diabetes, hypothyroidism, and polycystic ovarian syndrome, presented with a 2-week history of sudden right-sided facial deviation, periorbital pain, and moderate-intensity headache. The patient also reported amenorrhea not improving with polycystic ovarian syndrome treatment. Neurologic examination showed bilateral visual field defects and impaired visual acuity. Computed tomography scan, without contrast, revealed a hypodense sellar lesion with areas of hyperdensity. Magnetic resonance imaging showed a well-defined sellar lesion, exhibiting high signals on T1-weighted and T2-weighted images. The patient underwent microscopic trans-nasal trans-sphenoidal excision of the lesion. Histological sections of the sellar lesion revealed fibrous connective tissue with chronic inflammatory cells and cholesterol clefts, suggestive of xanthogranuloma. The patient is currently followed up at neurosurgery, endocrinology, and ophthalmology clinics with periodic laboratory/radiological investigations. Conclusion: Sellar xanthogranulomas remain rare intracranial lesions with few cases reported in the literature. Patients mostly present with severe hypopituitarism and visual dysfunction. They show no characteristic radiological features. The diagnosis is confirmed histopathologically, and the prognosis is generally favorable.

Hypothalamic Lipoma: Outcome of an Intracranial Developmental Lesion.

BACKGROUND: Hypothalamic lipomas are benign developmental lesions that tend to be discovered incidentally. This article describes the radiological features, outcome, and the postulated theories behind hypothalamic lipomas development. METHODS: The electronic archive of neurosurgery was retrospectively reviewed. All patients with a neuroradiological diagnosis of hypothalamic lipoma, between 2005 and 2020, were included. RESULTS: Out of 246 patients with intracranial lipomas, a total of six patients with hypothalamic lipomas have been identified. On computed tomography images, one of the hypothalamic lipomas demonstrated calcification. On magnetic resonance imaging, peripheral enhancement after contrast administration was noted in one of the lesions. Considering the benign nature of the lesions, neurosurgical intervention was not indicated. CONCLUSION: The majority of patients with hypothalamic lipomas are asymptomatic and undergo brain imaging for other indications. Although uncommon, such developmental lesions can be identified in the general population, especially with the advancement of neuroimaging techniques.

Traumatic superficial temporal artery pseudoaneurysm: Successful management using endovascular embolization.

Superficial temporal artery pseudoaneurysms are uncommon but can be potentially life-threatening. Considering their rarity, the present article outlines the clinical presentation, radiological findings, intervention, and outcome of traumatic pseudoaneurysm of the superficial temporal artery. An 83-year-old female sustained a traumatic injury to the temple, resulting in right-sided swelling of the forehead. Brain computed tomography and cerebral angiogram revealed a right-sided homogenously-enhancing pseudoaneurysm in the frontal region. Successful occlusion of the lesion was achieved utilizing endovascular embolization. Three months after discharge, the patient reported no complaints or recurrence. Subsequent management included reassurance and observation with periodic clinical assessments. The unusual presentation of superficial temporal artery pseudoaneurysms requires clinicians to have thorough knowledge on the clinical presentation, proper steps in diagnosis, and the approach of choice in management. Endovascular embolization of superficial temporal artery pseudoaneurysms remains a valid approach to achieve successful occlusion of the lesion.