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Angiofibroma
Angiofibroma (AGF) is a descriptive term for a wide range of benign skin or mucous membrane (i.e. the outer membrane lining body cavities such as the mouth and nose) 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 (i.e. gross) 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 (i.e. the main fibrous component of connective tissue). Angiofibromas have been divided into different types but commonly a specific type was given multiple ...
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Nasopharyngeal Angiofibroma
Nasopharyngeal angiofibroma is an angiofibroma also known as juvenile nasal angiofibroma, fibromatous hamartoma, and angiofibromatous hamartoma of the nasal cavity. 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. Patients with nasopharyngeal angiofibroma usually present with one-sided nasal obstruction with profuse epistaxis. Signs and symptoms * Frequent chronic epistaxis or blood-tinged nasal discharge * Nasal obstruction and rhinorrhea * Facial dysmorphism (when locally invasive) * Conductive hearing loss from eustachian-tube obstruction * Diplopia, which occurs secondary to erosion into superior orbital fissure and due to th ...
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Koenen's Tumor
Koenen's tumor (KT), also commonly termed periungual angiofibroma,Freedberg, et al. (2003). ''Fitzpatrick's Dermatology in General Medicine''. (6th ed.). McGraw-Hill. . is a subtype of the angiofibromas. Angiofibromas are benign papule, nodule, and/or tumor lesions that are separated into various subtypes based primarily on the characteristic locations of their lesions. KTs are angiofibromas that develop in and under the toenails and/or fingernails. KTs were once considered as the same as another subtype of the angiofibromas viz., acral angiofibromas. While the literature may still sometimes regard KTs as acral angiofibromas, acral angiofibromas are characteristically located in areas close to but not in the toenails and fingernails as well as in the soles of the feet and palms of the hands. KTs are here regarded as distinct from acral angiofibromas. KTs most commonly develop in individuals who have the rare genetic disease, tuberous sclerosis (a heritable neurocutaneous disorder ...
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Tuberous Sclerosis
Tuberous sclerosis complex (TSC) is a rare multisystem autosomal dominant genetic disease that causes non-cancerous tumours to grow in the brain and on other vital organs such as the kidneys, heart, liver, eyes, lungs and skin. A combination of symptoms may include seizures, intellectual disability, developmental delay, behavioral problems, skin abnormalities, lung disease, and kidney disease. TSC is caused by a mutation of either of two genes, '' TSC1'' and '' TSC2'', which code for the proteins hamartin and tuberin, respectively, with ''TSC2'' mutations accounting for the majority and tending to cause more severe symptoms. These proteins act as tumor growth suppressors, agents that regulate cell proliferation and differentiation. Prognosis is highly variable and depends on the symptoms, but life expectancy is normal for many. The prevalence of the disease is estimated to be 7 to 12 in 100,000. The disease is often abbreviated to tuberous sclerosis, which refers to ...
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Fibroblastic And Myofibroblastic Tumors
Fibroblastic and myofibroblastic tumors (FMTs) develop from the mesenchymal stem cells which differentiate into fibroblasts (the most common cell type in connective tissue) and/or the myocytes/ myoblasts that differentiate into muscle cells. FMTs are a heterogeneous group of soft tissue neoplasms (i.e. abnormal and excessive tissue growths). The World Health Organization (2020) defined tumors as being FMTs based on their morphology and, more importantly, newly discovered abnormalities in the expression levels of key gene products made by these tumors' neoplastic cells. Histopathologically, FMTs consist of neoplastic connective tissue cells which have differented into cells that have microscopic appearances resembling fibroblasts and/or myofibroblasts. The fibroblastic cells are characterized as spindle-shaped cells with inconspicuous nucleoli that express vimentin, an intracellular protein typically found in mesenchymal cells, and CD34, a cell surface membrane glycopr ...
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Angiofibroma Of Soft Tissue
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 (i.e. growth of tissue that is not coordinated with the normal surrounding tissue and persists in growing even if the original trigger for growth is removed) that was first described by A. Mariño-Enríquez and C.D. Fletcher in 2012. AFST tumors typically occur in a leg but can occur in other locations; they develop in older children and adults including elderly individuals. AFSTs are slow-growing, often painless tumors composed primarily of spindle-shaped cells and a prominent vascular network. The spindle-shaped cells are benign tumor cells that in almost all cases have chromosome abnormalities that are thought to contribute to their abnormal development and/or growth. AFST tumors are commonly trea ...
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Papules
A papule is a small, well-defined bump in the skin. It may have a rounded, pointed or flat top, and may have a dip. It can appear with a stalk, be thread-like or look warty. It can be soft or firm and its surface may be rough or smooth. Some have crusts or scales. A papule can be flesh colored, yellow, white, brown, red, blue or purplish. There may be just one or many, and they may occur irregularly in different parts of the body or appear in clusters. It does not contain fluid but may progress to a pustule or vesicle. A papule is smaller than a nodule; it can be as tiny as a pinhead and is typically less than 1 cm in width, according to some sources, and 0.5 cm according to others. When merged together, it appears as a plaque. Its color might indicate its cause, such as white in milia, red in eczema, yellowish in xanthoma and black in melanoma. They may open when scratched and become infected and crusty. Definition A papule is a small, well-defined bump in th ...
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Multiple Endocrine Neoplasia Type 1
Multiple endocrine neoplasia type 1 (MEN-1) is one of a group of disorders, the multiple endocrine neoplasias, that affect the endocrine system through development of neoplastic lesions in pituitary, parathyroid gland and pancreas. It was first described by Paul Wermer in 1954. Signs and symptoms Parathyroid Hyperparathyroidism is present in ≥ 90% of patients. Asymptomatic hypercalcemia is the most common manifestation: about 25% of patients have evidence of nephrolithiasis or nephrocalcinosis. In contrast to sporadic cases of hyperparathyroidism, diffuse hyperplasia or multiple adenomas are more common than solitary adenomas. Pancreas Pancreatic islet cell tumors are today the major cause of death in persons with MEN-1. Tumors occur in 60-80% of persons with MEN-1 and they are usually multicentric. Multiple adenomas or diffuse islet cell hyperplasia commonly occurs. About 30% of tumors are malignant and have local or distant metastases. About 10-15% of islet cell tumors orig ...
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Adenoma Sebaceum
Adenoma sebaceum is a misnamed cutaneous disorder consisting of angiofibromas that begin in childhood (generally present between 2–5 years of age) and appear clinically as red papules on the face especially on the nasolabial folds, cheek and chinMarks, James G; Miller, Jeffery (2006). ''Lookingbill and Marks' Principles of Dermatology'' (4th ed.). Elsevier Inc. . mostly thought to be acne not responding to treatment. Adenoma sebaceum may at times be associated with tuberous sclerosis. Gradually the papules become more prominent with time and persist throughout life. Cosmetic removal by argon or pulse dye laser or scalpel is indicated. See also * List of cutaneous conditions * Skin lesion * Tuberous sclerosis Tuberous sclerosis complex (TSC) is a rare multisystem autosomal dominant genetic disease that causes non-cancerous tumours to grow in the brain and on other vital organs such as the kidneys, heart, liver, eyes, lungs and skin. A combinatio ... References De ...
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Hirsuties Coronae Glandis
Pearly penile papules (PPP) are benign small bumps on the human penis. They vary in size from 1–4 mm, are pearly or flesh-colored, smooth and dome-topped or filiform, and appear in one or several rows around the corona, the ridge of the head of the penis and sometimes on the penile shaft. They are painless, non-cancerous and not harmful. Symptoms and signs PPPs are small bumps on the human penis. They vary in size from 1–4 mm, are pearly or flesh-colored, smooth and dome-topped or filiform, and appear in one or several rows around the corona, the ridge of the glans and sometimes on the penile shaft. They are painless, non-cancerous and not harmful. Cause and mechanism PPPs are a type of angiofibroma. Their function is not well-understood. They are sometimes described as vestigial remnants of penile spines, sensitive features found in the same location in other primates. PPPs secrete oil that moistens the glans of the penis. They do not spread and ofte ...
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Cranial Cavity
The cranial cavity, also known as intracranial space, is the space within the skull that accommodates the brain. The skull minus the mandible is called the ''cranium''. The cavity is formed by eight cranial bones known as the neurocranium that in humans includes the skull cap and forms the protective case around the brain. The remainder of the skull is called the facial skeleton. Meninges are protective membranes that surround the brain to minimize damage of the brain when there is head trauma. Meningitis is the inflammation of meninges caused by bacterial or viral infections. Structure The capacity of an adult human cranial cavity is 1,200–1,700 cm3. The spaces between meninges and the brain are filled with a clear cerebrospinal fluid, increasing the protection of the brain. Facial bones of the skull are not included in the cranial cavity. There are only eight cranial bones: The occipital, sphenoid, frontal, ethmoid, two parietal, and two temporal bones are fused to ...
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Clivus (anatomy)
The clivus (, Latin for "slope"), or Blumenbach clivus, is a bony part of the cranium at the base of the skull. It is a shallow depression behind the dorsum sellae of the sphenoid bone. It slopes gradually to the anterior part of the basilar occipital bone at its junction with the sphenoid bone. It extends to the foramen magnum. It is related to the pons and the abducens nerve (CN VI). Structure The clivus is a shallow depression behind the dorsum sellae of the sphenoid bone. It slopes gradually to the anterior part of the basilar occipital bone at its junction with the sphenoid bone. Synchondrosis of these two bones forms the clivus. The clivus extends inferiorly to the foramen magnum. On axial planes, it sits just posterior to the sphenoid sinuses. It is medial to the foramen lacerum (the internal carotid artery reaches the middle cranial fossa above the foramen lacerum), proximal to its anastomosis with the Circle of Willis. It is anterior to the basilar artery. On sagi ...
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Pterygoid Processes Of The Sphenoid
The pterygoid processes of the sphenoid (from Greek ''pteryx'', ''pterygos'', "wing"), one on either side, descend perpendicularly from the regions where the body and the greater wings of the sphenoid bone unite. Each process consists of a medial pterygoid plate and a lateral pterygoid plate, the latter of which serve as the origins of the medial and lateral pterygoid muscles. The medial pterygoid, along with the masseter allows the jaw to move in a vertical direction as it contracts and relaxes. The lateral pterygoid allows the jaw to move in a horizontal direction during mastication (chewing). Fracture of either plate are used in clinical medicine to distinguish the Le Fort fracture classification for high impact injuries to the sphenoid and maxillary bones. The superior portion of the pterygoid processes are fused anteriorly; a vertical groove, the pterygopalatine fossa, descends on the front of the line of fusion. The plates are separated below by an angular cleft, t ...
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