Table of Contents
Definition / general | Essential features | Terminology | ICD coding | Epidemiology | Sites | Etiology | Diagrams / tables | Clinical features | Diagnosis | Radiology description | Radiology images | Prognostic factors | Case reports | Treatment | Clinical images | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Cytology description | Cytology images | Positive stains | Negative stains | Electron microscopy description | Electron microscopy images | Videos | Sample pathology report | Differential diagnosis | Additional references | Board review style question #1 | Board review style answer #1 | Board review style question #2 | Board review style answer #2Cite this page: Abdelzaher E. Colloid cyst. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/cnstumorcolloidcyst.html. Accessed December 22nd, 2024.
Definition / general
- Benign, unilocular, epithelium lined, mucin filled cyst of third ventricle (eMedicine: Colloid Cysts [Accessed 31 October 2023])
- Uncertain histogenesis, mostly of endodermal derivation (Acta Neuropathol 1992;83:605)
- Site, radiologic and pathologic features are distinctive so differential diagnosis may be limited
Essential features
- Benign, unilocular, epithelium lined, mucin filled cyst of third ventricle (eMedicine: Colloid Cysts [Accessed 31 October 2023])
- Usually adults (20 - 50 years); rare in children
- Located at anterosuperior third ventricle near foramen of Monro
- Excellent prognosis
Terminology
- Colloid cyst of the third ventricle
ICD coding
- ICD-10: G93.0 - cerebral cysts
Epidemiology
- Rare, 0.5 - 2.0% of all intracranial lesions and 10 - 20% of all intraventricular lesions (World Neurosurg 2019:123:351)
- Usually adults (20 - 50 years), rare in children, no sex predilection (World Neurosurg 2017:107:409)
Sites
- Anterosuperior third ventricle near foramen of Monro, attached to the third ventricular roof and choroid plexus
- Unusual sites: septum pellucidum, fourth ventricle (Acta Neurochir (Wien) 2004;146:397, Clin Neurol Neurosurg 2012;114:1095)
Etiology
- Usually sporadic
- Genetic predisposition is suggested with limited reports of familial clusters (World Neurosurg 2017:107:409, Asian J Neurosurg 2020;15:414)
Diagrams / tables
Clinical features
- Due to its position, causes intermittent obstruction of cerebrospinal fluid (CSF) flow and obstructive hydrocephalus with manifestations of increased intracranial pressure
- Headache is the most common symptom
- May also cause nausea, vomiting, blurred vision, gait disturbance, urinary incontinence and personality changes (BMC Neurol 2022;22:397)
- Sudden impaction (ball valve effect on the foramen of Monro) causes abrupt, transient lower limb paralysis (drop attacks) and rarely, sudden death (Emerg (Tehran) 2015;3:162)
- May be asymptomatic
Diagnosis
- Neuroimaging: computed tomography (CT) and magnetic resonance imaging (MRI)
- Biopsy
Radiology description
- Typical intraventricular location allows confident radiological diagnosis
- CT: unilocular hyperdense mass at or near the foramen of Monro (AJNR Am J Neuroradiol 2000;21:1470)
- MRI: spherical, usually nonenhancing, discrete cystic lesion at anterior third ventricle; most are intrinsically bright in precontrast T1 weighted MRI images (AJNR Am J Neuroradiol 2020;41:1833)
Radiology images
Prognostic factors
- Excellent prognosis
- Rare cases associated with sudden death (World Neurosurg 2019:123:351)
- Spontaneous regression is very rare (BMC Neurol 2022;22:397)
- Malignant transformation has not been reported
Case reports
- 10 year old girl with brainstem death due to a third ventricular colloid cyst (BJR Case Rep 2022;8:20220007)
- 31 year old woman with colloid cyst causing acute hydrocephalus during early pregnancy (Surg Neurol Int 2021:12:54)
- 49 year old man with spontaneous regression of colloid cyst (BMC Neurol 2022;22:397)
- 57 year old man with large colloid cyst obstructing the posterior third ventricle (J Neurosurg Case Lessons 2021;1:CASE2121)
- 77 year old woman with xanthogranulomatous colloid cyst (Surg Neurol Int 2019:10:169)
Treatment
- Excision (microsurgical or endoscopic) is curative (World Neurosurg 2021:149:e298)
- Stereotactic aspiration (potential for cyst recurrence)
- Observation may be reasonable in some stable, asymptomatic cases
Gross description
- 1 - 2 cm; larger cysts have been reported
- Round, unilocular, translucent with thin, glistening wall
- Cyst filled with clear or turbid viscid mucin that solidifies after fixation (AJNR Am J Neuroradiol 2000;21:1470)
- Specimens received are often only a wrinkled membrane
Microscopic (histologic) description
- Hypocellular, fibrous wall lined by simple to pseudostratified columnar epithelium with variable cilia or goblet cells (resembles bronchial epithelium) (Acta Neuropathol 1997;93:271, Diagn Cytopathol 2002;27:27)
- Cyst lining may be modified by pressure atrophy (become low cuboidal or flattened) or degenerative changes
- Unlike Rathke cleft and endodermal (enterogenous) cysts, lining epithelium is not prone to squamous metaplasia
- Fragments of normal choroid plexus are frequently attached to cyst
- Cyst contents are amorphous and proteinaceous, may show ghosts of desquamated lining cells and eosinophilic filamentous material (degenerated nucleoprotein and phospholipid) resembling infectious organisms (Actinomyces)
- In chronic lesions, a xanthogranulomatous reaction may occur (Surg Neurol Int 2019:10:169)
Microscopic (histologic) images
Cytology description
- Epithelial cells, cohesive sheets and individual ciliated cells and goblet cells (Diagn Cytopathol 2002;27:27)
- Abundant amorphous proteinaceous material with or without Actinomyces-like nucleoprotein arrays
- Presence of macrophages
Positive stains
- Epithelium: keratin (low and high molecular weight) (Hum Pathol 1992;23:811)
- Epithelium: EMA
- Mucin: PAS, mucicarmine (Diagn Pathol 2012:7:144)
Negative stains
Electron microscopy description
- Epithelial nature of lining cells is evident by cytoplasmic tonofilaments and desmosomes
- 6 cell types: ciliated cells, nonciliated cells with surface microvilli, goblet cells, basal cells, nonspecific small cells and occasional neuroendocrine cells with neurosecretory granules (Acta Neuropathol 1992;83:605)
- Well formed basal lamina
Videos
Colloid cyst
Sample pathology report
- Third ventricular cyst, endoscopic excision biopsy:
- Colloid cyst
Differential diagnosis
- Rathke cleft cyst:
- Intrasellar or suprasellar location
- Prone to squamous metaplasia
- Normal choroid plexus:
- Sometimes dominant or only epithelial tissue
- Papillary with cobblestone lining epithelium
- No ciliated or goblet cells
- EMA generally negative
- Choroid plexus papilloma:
- Papillary with pseudostratified lining epithelium
- No ciliated or goblet cells
- Papillary craniopharyngioma with xanthogranulomatous change:
- Squamous lining
Additional references
Board review style question #1
Board review style answer #1
B. Colloid cyst. Colloid cysts have a stereotypic location near the foramina of Monro, which causes intermittent obstruction of cerebrospinal fluid (CSF) flow and obstructive hydrocephalus. Answer D is incorrect because Rathke cleft cyst is sellar or suprasellar in location. Answer C is incorrect because endodermal (enterogenous) cysts mostly arise in the intraspinal compartment or in the posterior fossa. Answer A is incorrect because an arachnoid cyst is often located in the Sylvian fissure.
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Reference: Colloid cyst
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Board review style question #2
Which of the following brain cysts has a columnar cyst lining and is not prone to squamous metaplasia?
- Colloid cyst
- Endodermal (enterogenous) cyst
- Epidermoid cyst
- Rathke cleft cyst
Board review style answer #2
A. Colloid cyst. The lining epithelium of colloid cysts is not prone to squamous metaplasia. Answers B and D are incorrect because the lining epithelium of Rathke cleft cysts and endodermal (enterogenous) cysts is prone to squamous metaplasia. Answer C is incorrect because epidermoid cysts are lined by keratinized stratified squamous epithelium.
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Reference: Colloid cyst
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