Nasopharyngeal angiofibroma | |
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Other names | Juvenile nasopharyngeal angiofibroma [1] [2] |
Micrograph of a nasopharyngeal angiofibroma H&E stain. | |
Specialty | ENT surgery |
Nasopharyngeal angiofibroma is an angiofibroma also known as juvenile nasal angiofibroma, fibromatous hamartoma, and angiofibromatous hamartoma of the nasal cavity. [3] It is a histologically benign but locally aggressive vascular tumor of the nasopharynx that arises from the superior margin of the sphenopalatine foramen and grows in the back of the nasal cavity. It most commonly affects adolescent males (because it is a hormone-sensitive tumor). Though it is a benign tumor, it is locally invasive and can invade the nose, cheek, orbit (frog face deformity), or brain. [4] Patients with nasopharyngeal angiofibroma usually present with one-sided nasal obstruction with profuse epistaxis.
If nasopharyngeal angiofibroma is suspected based on physical examination (a smooth vascular submucosal mass in the posterior nasal cavity of an adolescent male), imaging studies such as CT or MRI should be performed. Biopsy should be avoided as to avoid extensive bleeding since the tumor is composed of blood vessels without a muscular coat.[ citation needed ]
Antral sign or Holman-Miller sign (forward bowing of posterior wall of maxilla) is pathognomic of angiofibroma.[ citation needed ]
DSA (digital subtraction angiography) of carotid artery to see the extension of tumors and feeding vessels[ citation needed ]
There are many different staging- systems published. [6] One of the most used is that of Radkowsky: [7]
Stage | Tumor Extension |
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IA | Limited to the nose or the nasopharynx |
IB | Extension into one or more paranasal sinus |
IIA | Minimal extension through sphenopalatine foramen into and including a minimal part of the medial-most part of pterygomaxillary fossa |
IIB | Full involvement of the pterygomaxillary fossa, displacing posterior wall of maxillary antrum forward. Lateral or anterior displacement of the branches of maxillary artery. Superior extension may occur, eroding the orbital bones |
IIC | Extension through the pterygomaxillary fossa into the cheek and temporal fossa or posterior to the pterygoid plates |
IIIA | Erosion of the skull base with minimal intracranial extension |
IIIB | Erosion of skull base with extensive intracranial involvement with or without cavernous sinus involvement |
Treatment for Nasopharyngeal angiofibroma (JNA) is primarily surgical. The tumor is primarily excised by external or endoscopic approach. Medical treatment and radiation therapy are only of historical interest.[ citation needed ]
External approaches:[ citation needed ]
Endoscopic approach is an excellent tool in primary and recurrent JNA, it allows visualisation and precise removal of the lesion. Preoperative embolisation of tumour may be of some use in reducing intraoperative bleeding.[ citation needed ]
Direct visualization is not common-[ citation needed ]
Prognosis for nasopharyngeal angiofibroma is favorable. Because these tumors are benign, metastasis to distal sites does not occur. However, these tumors are highly vascularized and grow rapidly. Removal is important in preventing nasal obstruction and recurrent epistaxis. Mortality is not associated with nasopharyngeal angiofibroma. [8]
Otorhinolaryngology ( oh-toh-RY-noh-LARR-in-GOL-ə-jee, abbreviated ORL and also known as otolaryngology, otolaryngology – head and neck surgery, or ear, nose, and throat is a surgical subspeciality within medicine that deals with the surgical and medical management of conditions of the head and neck. Doctors who specialize in this area are called otorhinolaryngologists, otolaryngologists, head and neck surgeons, or ENT surgeons or physicians. Patients seek treatment from an otorhinolaryngologist for diseases of the ear, nose, throat, base of the skull, head, and neck. These commonly include functional diseases that affect the senses and activities of eating, drinking, speaking, breathing, swallowing, and hearing. In addition, ENT surgery encompasses the surgical management of cancers and benign tumors and reconstruction of the head and neck as well as plastic surgery of the face and neck.
In anatomy, a polyp is an abnormal growth of tissue projecting from a mucous membrane. If it is attached to the surface by a narrow elongated stalk, it is said to be pedunculated; if it is attached without a stalk, it is said to be sessile. Polyps are commonly found in the colon, stomach, nose, ear, sinus(es), urinary bladder, and uterus. They may also occur elsewhere in the body where there are mucous membranes, including the cervix, vocal folds, and small intestine. Some polyps are tumors (neoplasms) and others are non-neoplastic, for example hyperplastic or dysplastic, which are benign. The neoplastic ones are usually benign, although some can be pre-malignant, or concurrent with a malignancy.
An endoscope is an inspection instrument composed of image sensor, optical lens, light source and mechanical device, which is used to look deep into the body by way of openings such as the mouth or anus. A typical endoscope applies several modern technologies including optics, ergonomics, precision mechanics, electronics, and software engineering. With an endoscope, it is possible to observe lesions that cannot be detected by X-ray, making it useful in medical diagnosis. Endoscopes use tubes which are only a few millimeters thick to transfer illumination in one direction and high-resolution images in real time in the other direction, resulting in minimally invasive surgeries. It is used to examine the internal organs like the throat or esophagus. Specialized instruments are named after their target organ. Examples include the cystoscope (bladder), nephroscope (kidney), bronchoscope (bronchus), arthroscope (joints) and colonoscope (colon), and laparoscope. They can be used to examine visually and diagnose, or assist in surgery such as an arthroscopy.
The nasal cavity is a large, air-filled space above and behind the nose in the middle of the face. The nasal septum divides the cavity into two cavities, also known as fossae. Each cavity is the continuation of one of the two nostrils. The nasal cavity is the uppermost part of the respiratory system and provides the nasal passage for inhaled air from the nostrils to the nasopharynx and rest of the respiratory tract.
A nosebleed, also known as epistaxis, is an instance of bleeding from the nose. Blood can flow down into the stomach, and cause nausea and vomiting. In more severe cases, blood may come out of both nostrils. Rarely, bleeding may be so significant that low blood pressure occurs. Blood may also come up the nasolacrimal duct and out from the eye.
Nasal polyps (NP) are noncancerous growths within the nose or sinuses. Symptoms include trouble breathing through the nose, loss of smell, decreased taste, post nasal drip, and a runny nose. The growths are sac-like, movable, and nontender, though face pain may occasionally occur. They typically occur in both nostrils in those who are affected. Complications may include sinusitis and broadening of the nose.
Hereditary hemorrhagic telangiectasia (HHT), also known as Osler–Weber–Rendu disease and Osler–Weber–Rendu syndrome, is a rare autosomal dominant genetic disorder that leads to abnormal blood vessel formation in the skin, mucous membranes, and often in organs such as the lungs, liver, and brain.
Septoplasty [ˈsɛp.toˌplæ.sti] (Etymology: L, saeptum, septum; Gk, πλάσσειν plassein – to shape), or alternatively submucous septal resection and septal reconstruction, is a corrective surgical procedure done to straighten a deviated nasal septum – the nasal septum being the partition between the two nasal cavities. Ideally, the septum should run down the center of the nose. When it deviates into one of the cavities, it narrows that cavity and impedes airflow. Deviated nasal septum or “crooked” internal nose can occur at childbirth or as the result of an injury or other trauma. If the wall that functions as a separator of both sides of the nose is tilted towards one side at a degree greater than 50%, it might cause difficulty breathing. Often the inferior turbinate on the opposite side enlarges, which is termed compensatory hypertrophy. Deviations of the septum can lead to nasal obstruction. Most surgeries are completed in 60 minutes or less, while the recovery time could be up to several weeks. Put simply, septoplasty is a surgery that helps repair the passageways in the nose making it easier to breathe. This surgery is usually performed on patients with a deviated septum, recurrent rhinitis, or sinus issues.
A benign tumor is a mass of cells (tumor) that does not invade neighboring tissue or metastasize. Compared to malignant (cancerous) tumors, benign tumors generally have a slower growth rate. Benign tumors have relatively well differentiated cells. They are often surrounded by an outer surface or stay contained within the epithelium. Common examples of benign tumors include moles and uterine fibroids.
Aerosinusitis, also called barosinusitis, sinus squeeze or sinus barotrauma is a painful inflammation and sometimes bleeding of the membrane of the paranasal sinus cavities, normally the frontal sinus. It is caused by a difference in air pressures inside and outside the cavities.
Chronic atrophic rhinitis, or simply atrophic rhinitis, is a chronic inflammation of the nose characterised by atrophy of nasal mucosa, including the glands, turbinate bones and the nerve elements supplying the nose. Chronic atrophic rhinitis may be primary and secondary. Special forms of chronic atrophic rhinitis are rhinitis sicca anterior and ozaena. It can also be described as the empty nose syndrome.
Functional endoscopic sinus surgery (FESS) is a procedure that is used to treat sinusitis and other conditions that affect the sinuses. Sinusitis is an inflammation of the sinuses that can cause symptoms such as congestion, headaches, and difficulty breathing through the nose.
Juvenile polyposis syndrome is an autosomal dominant genetic condition characterized by the appearance of multiple juvenile polyps in the gastrointestinal tract. Polyps are abnormal growths arising from a mucous membrane. These usually begin appearing before age 20, but the term juvenile refers to the type of polyp, not to the age of the affected person. While the majority of the polyps found in juvenile polyposis syndrome are non-neoplastic, hamartomatous, self-limiting and benign, there is an increased risk of adenocarcinoma.
Nasopharyngeal carcinoma (NPC), or nasopharynx cancer, is the most common cancer originating in the nasopharynx, most commonly in the postero-lateral nasopharynx or pharyngeal recess, accounting for 50% of cases. NPC occurs in children and adults. NPC differs significantly from other cancers of the head and neck in its occurrence, causes, clinical behavior, and treatment. It is vastly more common in certain regions of East Asia and Africa than elsewhere, with viral, dietary and genetic factors implicated in its causation. It is most common in males. It is a squamous cell carcinoma of an undifferentiated type. Squamous epithelial cells are a flat type of cell found in the skin and the membranes that line some body cavities. Undifferentiated cells are cells that do not have their mature features or functions.
A colorectal polyp is a polyp occurring on the lining of the colon or rectum. Untreated colorectal polyps can develop into colorectal cancer.
Angiofibroma (AGF) is a descriptive term for a wide range of benign skin or mucous membrane lesions in which individuals have: 1) benign papules, i.e. pinhead-sized elevations that lack visible evidence of containing fluid; 2) nodules, i.e. small firm lumps usually >0.1 cm in diameter; and/or 3) tumors, i.e. masses often regarded as ~0.8 cm or larger. AGF lesions share common macroscopic and microscopic appearances. Grossly, AGF lesions consist of multiple papules, one or more skin-colored to erythematous, dome-shaped nodules, or usually just a single tumor. Microscopically, they consist of spindle-shaped and stellate-shaped cells centered around dilated and thin-walled blood vessels in a background of coarse bundles of collagen. Angiofibromas have been divided into different types but commonly a specific type was given multiple and very different names in different studies. The following list gives a brief description of the AFG types and their alternate names.
Rhinosporidiosis is an infection caused by Rhinosporidium seeberi.
Endoscopic endonasal surgery is a minimally invasive technique used mainly in neurosurgery and otolaryngology. A neurosurgeon or an otolaryngologist, using an endoscope that is entered through the nose, fixes or removes brain defects or tumors in the anterior skull base. Normally an otolaryngologist performs the initial stage of surgery through the nasal cavity and sphenoid bone; a neurosurgeon performs the rest of the surgery involving drilling into any cavities containing a neural organ such as the pituitary gland. The use of endoscope was first introduced in Transsphenoidal Pituitary Surgery by R Jankowsky, J Auque, C Simon et al. in 1992 G.
Tornwaldt's disease is the inflammation or abscess of the embryonic cyst of pharyngeal bursa. It is located in the midline of the posterior wall of the nasopharynx. It is covered anteriorly by mucosa in the adenoid mass. It is bounded posteriorly by longus muscle.
A nasopharyngoscopy is a surgical procedure performed to examine the nose and throat. It is performed using a fiberoptic instrument called a flexible fiberoptic nasopharyngoscope, that is inserted through the nose in order to examine both it, and the back of the throat. It is typically used to identify a variety of conditions including nasal septal deviation, polyps, and edema, and the causes of conditions including nosebleeds and sore throat. This procedure can be combined with the intentional application of intrathoracic pressure to perform a procedure known as Müller's maneuver. It is often used to examine cases of obstructive sleep apnea, and is often favorable to other procedures as it does not require exposure to radiation, can be performed while the patient is sitting or in the supine position, can both be performed while the patient is asleep or awake, and is inexpensive. However, it is an invasive procedure, which may make it unfavorable to some. It is also often used to evaluate children with stridor. In other cases, it can be used to evaluate and diagnose cases of nasopharyngeal cancer, often in addition to a biopsy.
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