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Symptoms are assessed on a case by case basis. Some cysts in the CNS can be asymptomatic (producing or showing no symptoms), depending on their location in the brain or spinal cord. If the cysts develop in critical areas of the central nervous system, they can present one or more of the following symptoms:
- Pressure in the spinal cord or brain
- Rupture of nerves around the cyst
- Weakness in specific parts of the body controlled by the cyst-infected brain region
- Inflammation
- Hydrocephalus
- Brainstem hemorrhage
- Seizures
- Visual disturbances and hearing Loss
- Headache
- Difficulty with balance or walking
In general, symptoms vary depending on the type of cyst and its location within the CNS.
This category of cysts takes over areas of necrotic tissue in the brain from injuries, diseases, or abnormalities, which occur due to the central nervous system's nonregenerative nature. These cysts can affect all germ layers of the CNS, but are most common in the arachnoid mater, and the ventricular space, which may block CSF pathways. These cysts can be static (stationary) or progressive. Some examples of cysts originating from the CNS tissue include:
- Arachnoid cysts (Leptomeningeal cysts)
- Ependymal cysts
- Cystic cerebellar astrocytomas
- Colloid cysts
Thyroglossal duct cysts most often present with a palpable asymptomatic midline neck mass above the level of the hyoid bone. The mass on the neck moves during swallowing or on protrusion of the tongue because of its attachment to the tongue via the tract of thyroid descent. Some patients will have neck or throat pain, or dysphagia.
The persistent duct or sinus can promote oral secretions, which may cause cysts to become infected. Up to half of thyroglossal cysts are not diagnosed until adult life. The tract can lie dormant for years or even decades, until some kind of stimulus leads to cystic dilation. Infection can sometimes cause the transient appearance of a mass or enlargement of the cyst, at times with periodic recurrences. Spontaneous drainage may also occur. Differential diagnosis are ectopic thyroid, enlarged lymph nodes, dermoid cysts and goiter.
Thyroglossal cyst usually presents as a midline neck lump (in the region of the hyoid bone) that is usually painless, smooth and cystic, though if infected, pain can occur. There may be difficulty breathing, dysphagia (difficulty swallowing), or dyspepsia (discomfort in the upper abdomen), especially if the cyst becomes large.
The most common location for a thyroglossal cyst is midline or slightly off midline, between the isthmus of the thyroid and the hyoid bone or just above the hyoid bone. A thyroglossal cyst can develop anywhere along a thyroglossal duct, though cysts within the tongue or in the floor of the mouth are rare.A thyroglossal cyst will move upwards with protrusion of the tongue.Thyroglossal cysts are associated with an increased incidence of ectopic thyroid tissue. Occasionally, a lingual thyroid can be seen as a flattened strawberry-like lump at the base of the tongue.
These cysts are found most often in young adults and are rare in infancy. The usual symptoms are the result of compression by the cyst, e.g., difficulty breathing or swallowing, cough, and chest pain. Malignant degeneration has been reported in these cysts on rare occasions. Chest x-rays show a smooth density just in front of the trachea or main stem bronchi at the carinal level. When the cyst communicates with the tracheobronchial tree, the air-fluid level may be seen within the cyst.
CT scanning is useful in localizing these cysts.
An infected thyroglossal duct cyst can occur when it is left untreated for a certain amount of time or simply when a thyroglossal duct cyst hasn't been suspected. The degree of infection can be examined as major rim enhancement has occurred, located inferior to the hyoid bone. Soft tissue swelling occurs, along with airway obstruction and trouble swallowing, due to the rapid enlargement of the cyst.
With infections, there can be rare cases where an expression of fluid is projected into the pharynx causing other problems within the neck.
Cysts rarely cause any symptoms, unless they become secondarily infected. The signs depend mostly upon the size and location of the cyst. If the cyst has not expanded beyond the normal anatomical boundaries of the bone, then there will be no palpable lump outside or inside the mouth. The vast majority of cysts expand slowly, and the surrounding bone has time to increase its density around the lesion, which is the body's attempt to isolate the lesion. Cysts that have expanded beyond the normal anatomic boundaries of a bone are still often covered with a thin layer of new bone. At this stage, there may be a sign termed "eggshell cracking", where the thinned cortical plate cracks when pressure is applied. A lump may be felt, which may feel hard if there is still bone covering the cyst, or fluctuant if the cyst has eroded through the bone surrounding it. A cyst may become acutely infected, and discharge into the oral cavity via a sinus. Adjacent teeth may be loosened, tilted or even moved bodily. Rarely, roots of teeth are resorbed, depending upon the type of cyst. The inferior alveolar nerve runs through the mandible and supplies sensation to the lower lip and chin. As most cysts expand slowly, there will be no altered sensation (anesthesia or paraesthesia), since the inferior alveolar canal is harmlessly enveloped or displaced over time. More aggressive cysts, or acute infection of any cyst may cause altered sensation.
Bronchogenic cysts are small, solitary cysts or sinuses, most typically located in the region of the suprasternal notch or over the manubrium.
There are several development cysts of the head and neck most of which form in the soft tissues rather than the bone. There are also several cysts, previously thought to arise from epithelial remanents trapped in embryonic lines of fusion, most of which are now believed to be odontogenic in origin or have an unknown cause. Their names are included for the sake of completeness.
The nasopalatine duct cyst (abbreviated NPDC) occurs in the median of the palate, usually anterior to first molars. It often appears between the roots of the maxillary central incisors. Radiographically, it may often appear as a heart-shaped radiolucency. It is usually asymptomatic, but may sometimes produce an elevation in the anterior portion of the palate. It was first described by Meyer in 1914.
The median palatal cyst has recently been identified as a possible posterior version of the nasopalatine duct cyst.
Most cysts in the body are benign (dysfunctional) tumors, the result of plugged ducts or other natural body outlets for secretions. However, sometimes these masses are considered neoplasm:
- Keratocyst
- Calcifying odotogenic cyst
- According to the current (2005) classification of the World Health Organization, both (parakeratizied) odontogenic keratocyst and calcifying odotogenic cyst have neoplastic characteristics, thus renamed as Keratocystic odontogenic tumor and Calcifying odotogenic tumor, respectively.
- Cystic ameloblastoma
- Long standing dentigerous cyst, odontogenic keratocyst, and residual cyst may have neoplastic potential converting into the locally aggressive ameloblastoma, or the malignant squamous cell carcinoma and mucoepidermoid carcinoma.
Odontogenic cyst are a group of jaw cysts that are formed from tissues involved in odontogenesis (tooth development). Odontogenic cysts are closed sacs, and have a distinct membrane derived from rests of odontogenic epithelium. It may contain air, fluids, or semi-solid material. Intra-bony cysts are most common in the jaws, because the mandible and maxilla are the only bones with epithelial components. That odontogenic epithelium is critical in normal tooth development. However, epithelial rests may be the origin for the cyst lining later.
Not all oral cysts are odontogenic cyst. For example, mucous cyst of the oral mucosa and nasolabial duct cyst are not of odontogenic origin.
In addition, there are several conditions with so-called (radiographic) 'pseudocystic appearance' in jaws; ranging from anatomic variants such as Stafne static bone cyst, to the aggressive aneurysmal bone cyst.
The nasopalatine cyst is the most common non-odontogenic cyst of the oral cavity, at an estimated occurrence rate of 73%.
A median mandibular cyst is a type of cyst that occurs in the midline of the mandible, thought to be created by proliferation and cystic degeneration of resting epithelial tissue that is left trapped within the substance of the bone during embryologic fusion of the two halves of the mandible, along the plane of fusion later termed the symphysis menti. A ture median mandibular cyst would therefore be classified as a non-odontogenic, fissural cyst. The existence of this lesion as a unique clinical entity is controversial, and some reported cases may have represented misdiagnosed odontogenic cysts, which are by far the most common type of intrabony cyst occurring in the jaws. It has also been suggested that the mandible develops as a bilobed proliferation of mesenchyme connected with a central isthmus. Therefore, it is unlikely that epithelial tissue would become trapped as there is no ectoderm separating the lobes in the first instance.
Neurocutaneous melanosis is associated with the presence of either giant congenital melanocytic nevi or non-giant nevi of the skin. It is estimated that neurocutaneous melanosis is present in 2% to 45% of patients with giant congenital melanocytic nevi. Patients with non-giant congenital melanocytic nevi seem to have a much lower, but undefined risk. Of these patients, only a small number are symptomatic, usually displaying symptoms before the age of 2.
These symptoms are the result of melanocytic lesions being present in the leptomeninges of the central nervous system.
Symptoms can include:
- Papilledema
- Cranial palsies
- Headache
- Vomiting
- Seizures
Others symptoms may also exist that are related to an increase in intracranial pressure. These symptoms seem to be present regardless of the malignancy of the melanin deposits within the central nervous system.
Approximately 10% of patient with neurocutaneous melanosis also present the Dandy–Walker syndrome and associated Dandy-Walker malformation. This malformation involves an enlargement of the posterior fossae and fourth ventricle along with agenesis of the cerebellar vermis. The abnormalities of the leptomeninges during fetal development due to neurocutaneous melanosis may be the cause of this increased incidence of the Dandy-Walker malformation. The development of hydrocephalus is the most common symptom associated with a combination of neurocutaneous melanosis and a Dandy-Walker malformation, occurring in about two out of three patients.
Choroid plexus cysts (CPCs) are cysts that occur within choroid plexus of the brain. The brain contains pockets or spaces called ventricles with a spongy layer of cells and blood vessels called the choroid plexus. This is in the middle of the fetal brain. The choroid plexus has the important function of producing cerebrospinal fluid. The fluid produced by the cells of the choroid plexus fills the ventricles and then flows around the brain and the spinal cord to provide a cushion of fluid around these structures.
CPCs can form within this structure and come from fluid trapped within this spongy layer of cells, much like a soap bubble or a blister. CPCs are often called "soft signs" or fetal ultrasound "markers" because some studies have found a weak association between CPCs and fetal chromosome abnormalities.
It is believed that many adults have one or more tiny CPCs. The fetal brain may create these cysts as a normal part of development. They are temporary and usually are gone by the 32nd week of pregnancy.
CPCs are a rare cause of intermittent hydrocephalus. This is caused by a blockage of foramina within the ventricular drainage system of the central nervous system (CNS), which can lead to expansion of the ventricles, compressing the brain (the cranial cavity cannot expand to accommodate the increase in fluid volume) and possibly causing damage.
Periapical cysts comprise approximately 75% of the types of cysts found in the oral region. The ratio of individuals diagnosed with periapical cysts is 3:2 male to female, as well as individuals between 20 and 60 years old. Periapical cysts occur worldwide.
Types of Periapical cysts:
Apical: 70%
Lateral: 20%
Residual: 10%
Periapical cysts begin as asymptomatic and progress slowly. Subsequent infection of the cyst causes swelling and pain. Initially, the cyst swells to a round hard protrusion, but later on the body resorbs some of the cyst wall, leaving a softer accumulation of fluid underneath the mucous membrane.
Larger cysts may cause bone expansion or displace roots. Discoloration of the affected tooth may also occur. Patient will present negative results to electric and ice test of the affected tooth but will be sensitive to percussion. Surrounding gingival tissue may experience lymphadenopathy. The alveolar plate may exhibit crepitus when palpated.
Periapical cysts exist in two structurally distinct classes:
Periapical true cysts - cysts containing cavities entirely surrounded in epithelial lining. Resolution of this type of cyst requires surgical treatment such as a cystectomy.
Periapical pocket cysts - epithelium lined cavities that have an opening to the root canal of the affected tooth. Resolution may occur after traditional root canal therapy.
A dentigerous cyst or follicular cyst is an odontogenic cyst - thought to be of developmental origin - associated with the crown of an unerupted (or partially erupted) tooth. The cyst cavity is lined by epithelial cells derived from the reduced enamel epithelium of the tooth forming organ. Regarding its pathogenesis, it has been suggested that the pressure exerted by an erupting tooth on the follicle may obstruct venous flow inducing accumulation of exudate between the reduced enamel epithelium and the tooth crown.
In addition to the developmental origin, some authors have suggested that periapical inflammation of non-vital deciduous teeth in proximity to the follicles of unerupted permanent successors may be a factor for triggering this type of cyst formation.
Histologically a normal dental follicle is lined by enamel epithelium, whereas a dentigerous cyst is lined by non-keratinized stratified squamous epithelium. Since the dentigerous cyst develops from follicular epithelium it has more potential for growth, differentiation and degeneration than a radicular cyst. Occasionally the wall of a dentigerous cyst may give rise to a more ominous mucoepidermoid carcinoma. Due to the tendency for dentigerous cysts to expand rapidly, they may cause pathological fractures of jaw bones.
On Fine needle aspiration, thin straw colored fluid is seen.
The usual radiographic appearance is that of a well-demarcated radiolucent lesion attached at an acute angle to the cervical area of an unerupted tooth. The border of the lesion may be radiopaque. The radiographic differentiation between a dentigerous cyst and a normal dental follicle is based merely on size. Radiographically, a dentigerous cyst should always be differentiated from a normal dental follicle. Dentigerous cysts are the most common cysts with this radiographic appearance. Radiographically the cyst appears unilocular with well defined margins and often sclerotic borders but sometimes it may be multilocular in appearance and may also have a continuous cystic membrane. Infected cysts show ill-defined margins. Follicular space more than 3mm is to be considered a dentigerous cyst.
Radiographically there are three types of dentigerous cyst, namely the Central type, Lateral type and the Circumferential type.
The most common location of dentigerous cysts are the Mandibular 3rd Molars and the Maxillary Canines, and they rarely involve deciduous teeth and are occasionally associated with odontomas.
A dentigerous cyst is often treated by excision of the cyst along with the extraction of the associated tooth.
In case of a large cyst marsupialization is done.
A renal cyst or kidney cyst, is a fluid collection in or on the kidney. There are several types based on the "Bosniak classification". The majority are benign, simple cysts that can be monitored and not intervened upon. However, some are cancerous or are suspicious for cancer and are commonly removed in a surgical procedure called nephrectomy.
Numerous renal cysts are seen in the cystic kidney diseases, which include polycystic kidney disease and medullary sponge kidney.
Most patients have symptoms in the first year of life. It is rare for symptoms to be undetected until adulthood, and usually adults have associated complications. The classic triad of intermittent abdominal pain, jaundice, and a right upper quadrant abdominal mass is found only in minority of patients.
In infants, choledochal cysts usually lead to obstruction of the bile ducts and retention of bile. This leads to jaundice and an enlarged liver. If the obstruction is not relieved, permanent damage may occur to the liver - scarring and cirrhosis - with the signs of portal hypertension (obstruction to the flow of blood through the liver) and ascites (fluid accumulation in the abdomen). There is an increased risk of cancer in the wall of the cyst.
In older individuals, choledochal cysts are more likely to cause abdominal pain and intermittent episodes of jaundice and occasionally cholangitis (inflammation within the bile ducts caused by the spread of bacteria from the intestine into the bile ducts). Pancreatitis also may occur. The cause of these complications may be related to either abnormal flow of bile within the ducts or the presence of gallstones
Renal cysts are classified by malignant risk using the Bosniak Classification System. The system was created by Dr. Morton Bosniak, a faculty member at the New York University Langone Medical Center in New York City.
The Bosniak classification categorizes renal cysts into five groups.
Category I
Category II
Category IIF
This category includes renal cysts with multiple thin septa, a septum thicker than hairline, slightly thick wall, or with calcification, which may be thick. It also includes intrarenal cysts larger than if there is no contrast enhancement.
Category IIF cysts have a 5-10% risk of being kidney cancer, and therefore follow-up is recommended. However, there is no consensus recommendation on the appropriate interval of follow up.
Category III
Category IV
Neurocutaneous melanosis is a congenital disorder characterized by the presence of congenital melanocytic nevi on the skin and melanocytic tumors in the leptomeninges of the central nervous system. These lesions may occur in the amygdala, cerebellum, cerebrum, pons, and spinal cord of patients. Although typically asymptomatic, malignancy occurs in the form of leptomeningeal melanoma in over half of patients. Regardless of the presence of malignancy, patients with symptomatic neurocutaneous melanosis generally have a poor prognosis with few treatment options. The pathogenesis of neurocutaneous melanosis is believed to be related to the abnormal postzygotic development of melanoblasts and mutations of the NRAS gene.
Choledochal cysts (a.k.a. bile duct cyst) are congenital conditions involving cystic dilatation of bile ducts. They are uncommon in western countries but not as rare in East Asian nations like Japan and China.
The median alveolar cyst is a rare cyst, occurring in the bony alveolus between the maxillary central incisors. It is distinguished from a periapical cyst by the fact that adjacent teeth are vital.
A nervous system neoplasm is a tumor affecting the nervous system. Types include:
- Nerve sheath tumor
- Brain tumor
- Arachnoid cyst
- Optic nerve glioma