Fibromatosis Hyalinica Multiplex Juvenilis
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Fibromatosis Hyalinica Multiplex Juvenilis
The term fibromatosis refers to a group of soft tissue tumors which have certain characteristics in common, including absence of cytologic and clinical malignant features, a histology consistent with proliferation of well-differentiated fibroblasts, an infiltrative growth pattern, and aggressive clinical behavior with frequent local recurrence. It is classed by the World Health Organization as an intermediate soft tissue tumor related to the sarcoma family. Arthur Purdy Stout coined the term ''fibromatosis'' (in the name ''congenital generalized fibromatosis'', describing myofibromatosis), in 1954. Diagnosis Juvenile subtypes Subtypes of juvenile fibromatosis include: * Infantile myofibromatosis: solitary tumors commonly occurring in the head and neck regions; multiple tumors occurring in the skin, subcutaneous tissue, muscles, and/or less commonly bones; or, rarely, tumors occurring in an internal organ(s). * Aponeurotic fibroma * Infantile digital fibromatosis * Aggressive in ...
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Soft Tissue
Soft tissue connective tissue, connects and surrounds or supports internal organs and bones, and includes muscle, tendons, ligaments, Adipose tissue, fat, fibrous tissue, Lymphatic vessel, lymph and blood vessels, fasciae, and synovial membranes. Soft tissue is Tissue (biology), tissue in the body that is not hard tissue, hardened by the processes of ossification or calcification such as bones and teeth. It is sometimes defined by what it is not – such as "nonepithelial, extraskeletal mesenchyme exclusive of the reticuloendothelial system and glia". Composition The characteristic substances inside the extracellular matrix of soft tissue are the collagen, elastin and ground substance. Normally the soft tissue is very hydrated because of the ground substance. The fibroblasts are the most common cell responsible for the production of soft tissues' fibers and ground substance. Variations of fibroblasts, like chondroblasts, may also produce these substances. Mechanical character ...
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Fibromatosis Hyalinica Multiplex Juvenilis
The term fibromatosis refers to a group of soft tissue tumors which have certain characteristics in common, including absence of cytologic and clinical malignant features, a histology consistent with proliferation of well-differentiated fibroblasts, an infiltrative growth pattern, and aggressive clinical behavior with frequent local recurrence. It is classed by the World Health Organization as an intermediate soft tissue tumor related to the sarcoma family. Arthur Purdy Stout coined the term ''fibromatosis'' (in the name ''congenital generalized fibromatosis'', describing myofibromatosis), in 1954. Diagnosis Juvenile subtypes Subtypes of juvenile fibromatosis include: * Infantile myofibromatosis: solitary tumors commonly occurring in the head and neck regions; multiple tumors occurring in the skin, subcutaneous tissue, muscles, and/or less commonly bones; or, rarely, tumors occurring in an internal organ(s). * Aponeurotic fibroma * Infantile digital fibromatosis * Aggressive in ...
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Radiation Therapy
Radiation therapy or radiotherapy (RT, RTx, or XRT) is a therapy, treatment using ionizing radiation, generally provided as part of treatment of cancer, cancer therapy to either kill or control the growth of malignancy, malignant cell (biology), cells. It is normally delivered by a linear particle accelerator. Radiation therapy may be cure, curative in a number of types of cancer if they are localized to one area of the body, and have not metastasis, spread to other parts. It may also be used as part of adjuvant therapy, to prevent tumor recurrence after surgery to remove a primary malignant tumor (for example, early stages of breast cancer). Radiation therapy is synergistic with chemotherapy, and has been used before, during, and after chemotherapy in susceptible cancers. The subspecialty of oncology concerned with radiotherapy is called radiation oncology. A physician who practices in this subspecialty is a radiation oncologist. Radiation therapy is commonly applied to the canc ...
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Aggressive Fibromatosis
Aggressive fibromatosis or desmoid tumor is a rare condition. Desmoid tumors are a type of fibromatosis and related to sarcoma, though without the ability to spread throughout the body (metastasize). The tumors arise from cells called fibroblasts, which are found throughout the body and provide structural support, protection to the vital organs, and play a critical role in wound healing. These tumors tend to occur in women in their thirties, but can occur in anyone at any age. They can be either relatively slow-growing or malignant. However, aggressive fibromatosis is locally aggressive and invasive, with spindle-like growths. The tumors can lead to pain, life-threatening problems, or, rarely, death when they invade other soft tissue or compress vital organs such as intestines, kidneys, lungs, blood vessels, or nerves. Most cases are sporadic, but some are associated with familial adenomatous polyposis (FAP). Approximately 10% of individuals with Gardner's syndrome, a type of FA ...
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Fibrous Papule Of The Face
Fiber (spelled fibre in British English; from ) is a natural or artificial substance that is significantly longer than it is wide. Fibers are often used in the manufacture of other materials. The strongest engineering materials often incorporate fibers, for example carbon fiber and ultra-high-molecular-weight polyethylene. Synthetic fibers can often be produced very cheaply and in large amounts compared to natural fibers, but for clothing natural fibers have some benefits, such as comfort, over their synthetic counterparts. Natural fibers Natural fibers develop or occur in the fiber shape, and include those produced by plants, animals, and geological processes. They can be classified according to their origin: * Vegetable fibers are generally based on arrangements of cellulose, often with lignin: examples include cotton, hemp, jute, flax, abaca, piña, ramie, sisal, bagasse, and banana. Plant fibers are employed in the manufacture of paper and textile (cloth), and ...
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Elastofibroma Dorsi
Elastofibroma dorsi is an ill-defined fibroelastic tumor-like condition made up of enlarged and irregular elastic fibers. The World Health Organization, 2020, has classified elastofibroma tumors as one specific type of the fibroblastic and myofibroblastic tumors. Signs and symptoms Patient will present with a slow growing, deep-seated, firm mass, often presenting bilaterally. There may be pain or tenderness, but this is rare. When only present on one side, they are more often present on the right side. Cause There are several theories about origin: * There is support for a genetic predisposition, as there are alterations of short arm of chromosome 1; * Multifocality may suggest systemic enzymatic defect, resulting in abnormal elastogenesis; * Repeated trauma or friction seems unlikely, but is still a possibility. Diagnosis By computed tomography, there is a poorly circumscribed, heterogeneous soft tissue mass, with a signal intensity similar to skeletal muscle. The fact that ...
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Nodular Fasciitis
Nodular fasciitis (NF) is a benign, soft tissue tumor composed of myofibroblasts that typically occurs in subcutaneous tissue, fascia, and/or muscles. The literature sometimes titles rare NF variants according to their tissue locations. The most frequently used and important of these are cranial fasciitis and intravascular fasciitis. In 2020, the World Health Organization classified nodular fasciitis as in the category of benign fibroblastic/myofibroblastic tumors. NF is the most common of the benign fibroblastic proliferative tumors of soft tissue. Nodular fasciitis is a rapidly growing, usually self-limiting neoplasm that occurs primarily but not exclusively in adults. Due to its rapid growth, NF is often misdiagnosed as a malignant tumor, usually a sarcoma. Indeed, NF was originally termed subcutaneous pseudosarcomatous fibromatosis when first described in 1955 by Konwaler ''et al''. The correct diagnosis of a tumor as NF is pivotal to prevent its overtreatment as a more aggre ...
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Dermatofibroma
A dermatofibroma, or benign fibrous histiocytomas, is a benign nodule in the skin, typically on the legs, elbows or chest of an adult. It is usually painless. It usually ranges from 0.2 to 2 cm in size but larger examples have been reported. It typically results from mild trauma such as an insect bite. Risk factors for developing multiple dermatofibromas include lupus, HIV, blood cancer and some medicines that weaken immunity. It is usually diagnosed by its appearance, but a biopsy may be required. Other bumps such as granular cell tumor, melanoma, clear cell acanthoma and dermatofibrosis lenticularis disseminata may look similar. Usually no treatment is needed. It can remain unchanged for years but can resolve spontaneously. Signs and symptoms Dermatofibromas are hard solitary slow-growing papules (rounded bumps) that appear in a variety of colours, usually brownish to tan. They are often elevated or pedunculated. A dermatofibroma is associated with the dimple sign; by applyi ...
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Knuckle Pads
Knuckle pads, also known as heloderma, meaning similar to the skin of the Gila monster lizard for which it is named, are circumscribed, keratotic, fibrous growths over the dorsa of the interphalangeal joints. They are described as well-defined, round, plaque-like, fibrous thickening that may develop at any age, and grow to be 10 to 15mm in diameter in the course of a few weeks or months, then go away over time. Knuckle pads are sometimes associated with Dupuytren's contracture and camptodactyly, and histologically, the lesions are fibromas. Knuckle pads are generally non-responsive to treatment, including corticosteroids, and tend to recur after surgery; however, there has been some effectiveness with intralesional fluorouracil. Signs and symptoms Knuckle pads are benign subcutaneous fibrotic nodules that are seen in the finger joints and/or the extensor area of the foot. From a clinical perspective, these are well-defined, non-compressible, freely moveable lesions that res ...
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Pachydermodactyly
Pachydermodactyly is a superficial dermal fibromatosis that presents as a poorly circumscribed symmetric, infiltrative, asymptomatic soft-tissue hypertrophy of the proximal fingers, typically sparing the thumbs and fifth fingers and rarely extending proximally to the wrists or occurring distally.Freedberg, et al. (2003). ''Fitzpatrick's Dermatology in General Medicine''. (6th ed.). McGraw-Hill. . Signs and symptoms Pachydermodactyly is defined by gradual, asymptomatic thickening of the periarticular skin and soft tissue swelling, mainly affecting the lateral sides of the fingers' proximal interphalangeal (PIP) joints. The most commonly affected fingers are the second, third, and fourth; however, the dorsum of the hand and the fifth finger may also be affected. Although moderate erythema, fine desquamation, or lichenification may occasionally be seen, epidermal alterations are not typically present. There is no impact on hand movements. Causes Although the specific cause and ...
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Peyronie's Disease
Peyronie's disease (PD) is a benign, acquired penile connective tissue disease characterized by the occurrence of fibrotic plaques within the Tunica albuginea (penis), tunica albuginea — the dense elastic covering of the Corpora cavernosa of penis, corpora cavernosa. The plaques cause abnormal curvature, pain, penile deformities (e.g., narrowing or indentation), and usually erectile dysfunction, particularly during erection. The condition typically leads to significant sexual and psychological effects, including difficulty with penetration and lowered self-esteem or evasiveness. Peyronie's disease is most often seen in middle-aged and older men with a median age of onset between 55 and 60 years, although it has also been noted in younger individuals and adolescents. While the etiology of PD is still a mystery, the leading hypothesis is that it arises from dysregulated wound healing in response to Chronic condition, chronic microtrauma of the erect penis. This triggers a cascade ...
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