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Concept: Colloid cyst

171

Colloid cysts are rare congenital, intracranial neoplasms, commonly located in the third ventricle. Colloid cysts are endodermal congenital malformations. The cysts commonly range in size from 1–2 cm in diameter, although large cysts >3 cm in size have been reported. The components of the cyst include an outer fibrous capsule over an inner epithelium. The epithelium is usually a single layer of mucin-producing or ciliated cells. Such cysts contain mucoid and gelatinous material, which is positive for both Periodic acid Schiff (PAS) and mucicarmen staining. Although colloid cysts usually represent histopathologically benign neoplasms, they can result in sudden, unexpected and potentially lethal complications. The mechanism(s) of death is still a controversial subject and several mechanisms have been postulated to explain the sudden onset of severe symptoms and of fatal rapid deterioration in patients with colloid cysts. In this case, macroscopic and histological findings addressed the diagnosis of colloid cyst of the third ventricle with diffuse myocardial injury (coagulative myocytolysys or contraction band necrosis, CBN) and led us to conclude that acute cardiac arrest due to hypothalamus stimulation in the context of colloid cyst of the third ventricle was the cause of death. As the hypothalamic structures which are involved in neuroendocrine and autonomic regulation playing a key role in cardiovascular control are located close to the walls of the third ventricle which is the most frequent anatomical site of colloid cyst, this may suggest that reflex cardiac effects due to the compression of the hypothalamic cardiovascular regulatory centers by the cyst explain the sudden death in patients harboring a colloid cyst when signs of hydrocephalus or brain herniation are lacking.Virtual slidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/4915842848034158.

Concepts: Hypothalamus, Blood, Myocardial infarction, Heart, Intracranial pressure, Benign tumor, Congenital disorder, Colloid cyst

28

Colloid cysts of the third ventricle are rare benign lesions. We report here an exceptional familial case defined by the evidence of two colloid cysts in two relatives of the first degree, a mother and her daughter in our description. Only 15 cases are reported in the literature. The main differences compared with sporadic cases are an earlier age of discovery and a female predominance. In case of familial colloid cyst, we have to recover a brain MRI screening of all the relatives of the first degree.

Concepts: Family, Brain, Magnetic resonance imaging, Report, Case, Cyst, Ventricular system, Colloid cyst

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OBJECT Surgical indications for patients with pineal cysts are controversial. While the majority of patients harboring a pineal cyst require no treatment, surgery is a well-accepted option for a subset of those patients with secondary hydrocephalus or Parinaud’s syndrome. The majority of pineal cysts are identified incidentally during workup for other potential conditions, which may or may not be related to the presence of the cyst. In the absence of clear obstruction of CSF pathways, the treatment of presumed symptomatic pineal cysts is debatable. To clarify the role of surgery in these borderline cases, the authors reviewed their experience with resection of pineal cysts in the absence of ventriculomegaly or Parinaud’s syndrome. METHODS The authors retrospectively reviewed medical records and imaging of all patients surgically treated between 2001 and 2014 with a pineal cyst in the absence of ventriculomegaly and Parinaud’s syndrome. The presenting symptoms, preoperative cyst size, preoperative radiographic aqueductal compression, extent of resection, and radiographic and clinical follow-up were documented. RESULTS Eighteen patients (14 female and 4 male; mean age 24 years, range 4-47 years) underwent cyst resection in the absence of ventriculomegaly or Parinaud’s syndrome. Presenting symptoms included headache (17 patients), visual disturbances (10 patients), gait instability (5 patients), dizziness (5 patients), episodic loss of consciousness (2 patients), and hypersomnolence (1 patient). The mean preoperative cyst diameter was 1.5 cm (range 0.9-2.2 cm). All patients had a complete resection. At a mean clinical follow-up of 19.1 months (range postoperative to 71 months), 17 (94%) patients had resolution or improvement of their presenting symptoms. CONCLUSIONS The authors' results suggest that ventriculomegaly and Parinaud’s syndrome are not absolute requisites for a pineal cyst to be symptomatic. Analogous to colloid cysts of the third ventricle, intermittent occlusion of cerebrospinal fluid pathways may cause small pineal cysts to become intermittently symptomatic. A select cohort of patients with pineal cysts may benefit from surgery despite a lack of hydrocephalus or other obvious compressive pathology.

Concepts: Hospital, Surgery, Symptomatic treatment, Cerebrospinal fluid, Hydrocephalus, Ventricular system, Pineal gland cyst, Colloid cyst

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To analyze the safety and efficacy of neuroendoscopic colloid cyst resection and to examine patient satisfaction. This study presents retrospective analysis of a single surgeon’s experience with neuroendoscopic resection of colloid cysts.

Concepts: Cyst, Colloid cyst

3

OBJECTIVE Colloid cysts are rare, histologically benign lesions that may result in obstructive hydrocephalus and death. Understanding the natural history of colloid cysts has been challenging given their low incidence and the small number of cases in most reported series. This has complicated efforts to establish reliable prognostic factors and surgical indications, particularly for asymptomatic patients with incidental lesions. Risk factors for obstructive hydrocephalus in the setting of colloid cysts remain poorly defined, and there are no grading scales on which to develop standard management strategies. METHODS The authors performed a single-center retrospective review of all cases of colloid cysts of the third ventricle treated over nearly 2 decades at Washington University. Univariate analysis was used to identify clinical, imaging, and anatomical factors associated with 2 outcome variables: symptomatic clinical status and presentation with obstructive hydrocephalus. A risk-prediction model was defined using bootstrapped logistic regression. Predictive factors were then combined into a simple 5-point clinical scale referred to as the Colloid Cyst Risk Score (CCRS), and this was evaluated with receiver-operator characteristics. RESULTS The study included 163 colloid cysts, more than half of which were discovered incidentally. More than half of the incidental cysts (58%) were followed with surveillance neuroimaging (mean follow-up 5.1 years). Five patients with incidental cysts (8.8%) progressed and underwent resection. No patient with an incidental, asymptomatic colloid cyst experienced acute obstructive hydrocephalus or sudden neurological deterioration in the absence of antecedent trauma. Nearly half (46.2%) of symptomatic patients presented with hydrocephalus. Eight patients (12.3%) presented acutely, and there were 2 deaths due to obstructive hydrocephalus and herniation. The authors identified several factors that were strongly correlated with the 2 outcome variables and defined third ventricle risk zones where colloid cysts can cause obstructive hydrocephalus. No patient with a lesion outside these risk zones presented with obstructive hydrocephalus. The CCRS had significant predictive capacity for symptomatic clinical status (area under the curve [AUC] 0.917) and obstructive hydrocephalus (AUC 0.845). A CCRS ≥ 4 was significantly associated with obstructive hydrocephalus (p < 0.0001, RR 19.4). CONCLUSIONS Patients with incidentally discovered colloid cysts can experience both lesion enlargement and symptom progression or less commonly, contraction and symptom regression. Incidental lesions rarely cause acute obstructive hydrocephalus or sudden neurological deterioration in the absence of antecedent trauma. Nearly one-half of patients with symptomatic colloid cysts present with obstructive hydrocephalus, which has an associated 3.1% risk of death. The CCRS is a simple 5-point clinical tool that can be used to identify symptomatic lesions and stratify the risk of obstructive hydrocephalus. External validation of the CCRS will be necessary before objective surgical indications can be established. Surgical intervention should be considered for all patients with CCRS ≥ 4, as they represent the high-risk subgroup.

Concepts: Regression analysis, Hospital, Surgery, Hydrocephalus, Cyst, Lesion, Ventricular system, Colloid cyst

0

Acquired Chiari malformation and associated syringomyelia have been previously described following lumbar puncture/drainage, lumboperitoneal shunts, and spontaneous CSF leakage. In addition to these etiologies, space-occupying lesions such as meningiomas, epidermoid cysts, medulloblastomas, and arachnoid cysts are rare causes of acquired Chiari malformation and syringomyelia. We report a rare case of colloid cyst with hydrocephalus causing secondary Chiari malformation with syringomyelia.

Concepts: Causality, Philosophy of science, Cerebrospinal fluid, Cyst, Neurological disorders, Arnold-Chiari malformation, Colloid cyst, Arachnoid cyst

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To accurately characterize patient characteristics and the clinical presentation of fatal colloid cysts.

Concepts: Medicine, Mental status examination, Colloid cyst

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Colloid cysts are the most common pathologic lesions of the third ventricle. Although they are histologically benign, they may grow and can cause the hydrocephalus. A 5-year-old male patient underwent to surgery with the diagnosis of colloid cyst. In operation, after the anterior transcallosal approach, the cyst was punctured by syringe and obviously pus aspirated.

Concepts: Physician, Hydrocephalus, Cyst, Ventricular system, Colloid cyst

0

Endoscopic excision of colloid cysts is currently well established as a minimally invasive and highly effective technique that is associated with less morbidity in comparison to microsurgical resection.

Concepts: Surgery, Minimally invasive, Sebaceous cyst, Cyst, Ganglion cyst, Colloid cyst

0

Colloid cyst is a gelatin-containing cyst in the brain almost always found in the third ventricle. The specific shape and location of these cysts, a round well-delineated mass in the rostral part of the third ventricle adjacent to the foramen of Monro, on imaging are the main findings for diagnosis. Several masses of the third ventricle masquerading colloid cysts on images have been reported yet. Based on different surgical approach, pre-operative misdiagnosis of colloid cyst may have great impact on prognosis.

Concepts: Brain, Astrocytoma, Neurology, Medical diagnosis, Cyst, Ventricular system, Colloid cyst, Pilocytic astrocytoma