Nasopharyngeal angiofibroma | |
---|---|
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 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. Though it is a benign tumor, it is locally invasive and can invade the nose, cheek, orbit (frog face deformity), or brain. [4]
The tumor is highly vascular, meaning that is has a rich blood supply. Clinically, an individual with one may present with nosebleeds, followed by nasal obstruction and then mucus discharge from the nose.
Grossly, it is a firm mass that may be yellow, dark red, or even black. Histologically, it presents with several vascular spaces of varying sizes and wall thicknesses as well as fibrous or collagenous stroma with fibroblasts. Mast cells are common. Mitotic figures are usually not present. [5]
The cause is not well understood and remains under debate.
It is hypothesized by some that it may arise from a vascular source such as an AVM or as a remnant of the first branchial arch (an embryological feature).
Others have suggested that hormonal influences are the key component of the tumor's origins. Studies have shown the presence of androgen, progesterone, and estrogen receptors within the tumor. Some researchers have postulated that the tumor, with its strong predominance in adolescent males, is stimulated by androgen increases during puberty. This has been further supported by reports of increased tumor growth after administration of testosterone as well as reports of older women with decreased estrogen - and therefore decreased ability to counteract androgen hormone effects - presenting with the tumors. However the tumor has also been found in pregnant women. Such women have increased levels of "protective" estrogen, which would suggest androgen effects are not a significant factor.
Genetic abnormalities and HPV may also play roles in the tumor's etiology. [6]
The tumors are highly vascular, and as such, angiography - an imaging technique that uses x-rays and contrast dyes - is usually used in diagnosis by visualizing blood vessel locations and structures. Also due to the lesions' vascular nature, biopsy and fine needle aspiration are typically avoided as part of a diagnostic workup as these could cause significant bleeding. [5]
The preferred treatment is surgical resection. There are a variety of surgical techniques that can be used. Prior to surgery, an embolization procedure may be used to reduce the tumor's blood supply and thereby decrease excessive bleeding. Androgen receptor blocker medications may help reduce the tumor's size prior to surgery or if it reoccurs, but they do not eliminate the tumor. [6]
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 nosebleeds. Mortality is not associated with nasopharyngeal angiofibroma. [7]
Otorhinolaryngology is a surgical subspecialty 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, scalp, and neck.
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 be forced to flow up and through the nasolacrimal duct and out of the eye, producing bloody tears.
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, 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.
The theca folliculi comprise a layer of the ovarian follicles. They appear as the follicles become secondary follicles.
Adenoid hypertrophy, also known as enlarged adenoids refers to an enlargement of the adenoid that is linked to nasopharyngeal mechanical blockage and/or chronic inflammation. Adenoid hypertrophy is a characterized by hearing loss, recurrent otitis media, mucopurulent rhinorrhea, chronic mouth breathing, nasal airway obstruction, increased infection susceptibility, and dental malposition.
Masculinizing hormone therapy, also known as transmasculine hormone therapy or female-to-male hormone therapy, is a form of hormone therapy and gender affirming therapy which is used to change the secondary sexual characteristics of transgender people from feminine or androgynous to masculine. It is a common type of transgender hormone therapy, and is predominantly used to treat transgender men and other transmasculine individuals who were assigned female at birth. Some intersex people also receive this form of therapy, either starting in childhood to confirm the assigned sex or later if the assignment proves to be incorrect.
Feminizing hormone therapy, also known as transfeminine hormone therapy, is hormone therapy and sex reassignment therapy to change the secondary sex characteristics of transgender people from masculine or androgynous to feminine. It is a common type of transgender hormone therapy and is used to treat transgender women and non-binary transfeminine individuals. Some, in particular intersex people, but also some non-transgender people, take this form of therapy according to their personal needs and preferences.
Hypomenorrhea or hypomenorrhoea, also known as short or scanty periods, is extremely light menstrual blood flow. It is the opposite of heavy periods or hypermenorrhea which is more properly called menorrhagia.
Angiofibroma (AGF) is a descriptive term for a wide range of benign skin or mucous membrane lesions in which individuals have:
Genital leiomyomas are leiomyomas that originate in the dartos muscles, or smooth muscles, of the genitalia, areola, and nipple. They are a subtype of cutaneous leiomyomas that affect smooth muscle found in the scrotum, labia, or nipple. They are benign tumors, but may cause pain and discomfort to patients. Genital leiomyoma can be symptomatic or asymptomatic and is dependent on the type of leiomyoma. In most cases, pain in the affected area or region is most common. For vaginal leiomyoma, vaginal bleeding and pain may occur. Uterine leiomyoma may exhibit pain in the area as well as painful bowel movement and/or sexual intercourse. Nipple pain, enlargement, and tenderness can be a symptom of nipple-areolar leiomyomas. Genital leiomyomas can be caused by multiple factors, one can be genetic mutations that affect hormones such as estrogen and progesterone. Moreover, risk factors to the development of genital leiomyomas include age, race, and gender. Ultrasound and imaging procedures are used to diagnose genital leiomyomas, while surgically removing the tumor is the most common treatment of these diseases. Case studies for nipple areolar, scrotal, and uterine leiomyoma were used, since there were not enough secondary resources to provide more evidence.
An androgen-dependent condition, disease, disorder, or syndrome, is a medical condition that is, in part or full, dependent on, or is sensitive to, the presence of androgenic activity in the body.
Orchiectomy is a surgical procedure in which one or both testicles are removed. The surgery can be performed for various reasons:
Mammary-type myofibroblastoma (MFB), also named mammary and extramammary myofibroblastoma, was first termed myofibrolastoma of the breast, or, more simply, either mammary myofibroblastoma (MMFB) or just myofibroblastoma. The change in this terminology occurred because the initial 1987 study and many subsequent studies found this tumor only in breast tissue. However, a 2001 study followed by numerous reports found tumors with the microscopic histopathology and other key features of mammary MFB in a wide range of organs and tissues. Further complicating the issue, early studies on MFB classified it as one of various types of spindle cell tumors that, except for MFB, were ill-defined. These other tumors, which have often been named interchangeably in different reports, are: myelofibroblastoma, benign spindle cell tumor, fibroma, spindle cell lipoma, myogenic stromal tumor, and solitary stromal tumor. Finally, studies suggest that spindle cell lipoma and cellular angiofibroma are variants of MFB. Here, the latter two tumors are tentatively classified as MFB variants but otherwise MFB is described as it is more strictly defined in most recent publications. The World Health Organization in 2020 classified mammary type myofibroblastoma tumors and myofibroblastoma tumors as separate tumor forms within the category of fibroblastic and myofibroblastic tumors.
Gynecomastia is the abnormal non-cancerous enlargement of one or both breasts in males due to the growth of breast tissue as a result of a hormone imbalance between estrogens and androgens. Gynecomastia can cause significant psychological distress or unease.
A hormone-sensitive cancer, or hormone-dependent cancer, is a type of cancer that is dependent on a hormone for growth and/or survival. Examples include breast cancer, which is dependent on estrogens like estradiol, and prostate cancer, which is dependent on androgens like testosterone.
Cellular angiofibroma (CAF) is a rare, benign tumor of superficial soft tissues that was first described by M. R. Nucci et al. in 1997. These tumors occur predominantly in the distal parts of the female and male reproductive systems, i.e. in the vulva-vaginal and inguinal-scrotal areas, respectively, or, less commonly, in various other superficial soft tissue areas throughout the body. CAF tumors develop exclusively in adults who typically are more than 30 years old.
Angiofibroma of soft tissue (AFST), also termed angiofibroma, not otherwise specified, is a recently recognized and rare disorder that was classified in the category of benign fibroblastic and myofibroblastic tumors by the World Health Organization in 2020. An AFST tumor is a neoplasm that was first described by A. Mariño-Enríquez and C.D. Fletcher in 2012.
Gardner fibroma (GF) is a benign fibroblastic tumor. GF tumors typically develop in the dermis and adjacent subcutaneous tissue lying just below the dermis. These tumors typically occur on the back, abdomen, and other superficial sites but in rare cases have been diagnoses in internal sites such as the retroperitoneum and around the large blood vessels in the upper thoracic cavity. The World Health Organization, 2020, classified Gardner fibroma as a benign tumor in the category of fibroblastic and myofibroblastic tumors.