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Typically patients present with progressive pain, often long standing and/or bony swelling and restricted range of movement in affected limb 3,12. The latter is most often the case in bones with little overlying soft tissues (e.g. short tubular bones of the hands and feet).
Most chondromyxoid fibromas are located in the metaphyseal region of long bones (60%), and may extend to the epiphyseal line and even rarely abut the articular surface 3,12. They are almost never just epiphyseal 3. The classical site is the upper 1/3rd of tibia ( which accounts for 25% of all cases) with the small tubular bones of the foot, the distal femur and pelvis being other relatively common locations 12.
Rarely occur in the skull or skull base.
Fibroma of tendon sheath is a benign tumor that presents as a small subcutaneous nodule that slowly increases in size. The tumors often have a multinodular growth pattern, with individual nodules being composed of bland, slender, spindle-shaped cells (myofibroblasts) in a dense, fibrous matrix.” A common microscopic finding is the presence of elongated, slit-like blood vessels. The lesions nearly always arise in the distal portions of the extremities. They often occur on the fingers, hands, toes, or feet. Although they are benign, they may recur in up to 40% of cases.
Although they may be regarded as a tumor of the skin, the lesions arise from tendons and aponeuroses in superficial sites, and are therefore properly classified as in the category "soft tissue tumor."
The biological nature of Fibroma of tendon sheath is not known, but the category appears to comprise a number of different pathologic processes. It is considered that about one-third of the lesions in this category may be acral variants of the entity, nodular fasciitis.
Giant-cell tumor of the tendon sheath, also known as giant-cell synovioma and localized nodular tenosynovitis, is a firm lesion, measuring 1 to 3 cm in diameter, and is most commonly attached to the tendons of the fingers, hands, and wrists, with a predilection for the flexor surfaces. Giant-cell tumor of tendon sheaths most often affect the wrist and fingers of males and females from the ages of 20-50 . These tumors are typically painless and can cause cortical erosion. Surgery to remove the tumor is a common treatment, though the tumors tend to recur.
Neurothekeoma is a benign cutaneous tumor first described by Gallager and Helwig, who proposed the term in order to reflect the presumed origin of the lesion from nerve sheath. Microscopically, the lesions described closely resembled the tumor, "nerve sheath myxoma", an entity first described by Harkin and Reed. The latter had, through the years, been variously described as "Bizarre cutaneous neurofibroma", "Myxoma of nerve sheath", and "Pacinian neurofibroma".
Clinically, neurothekeomas present as a solitary nodule of the skin. The most common sites of occurrence are the head and neck and the extremities. The lesions range in size from about 0.5 cm. to more than 3 cm. The average patient age is about 25 years, but neurothkeomas may occur at any age. Women are affected about more often; the male to female ratio is approximately 1:2.
Microscopically, neurothekeoma consists of closely aggregated bundles or fascicles of spindle-shaped cells. The fascicles may or may not have a myxoid background.
Since the time of their first description, it has been reported that neurothekeomas are likely not of nerve sheath origin, as implied by the term. Consequently, neurothekeoma and nerve sheath myxoma are likely not related histogenetically, although they are similar in appearance and in behavior.
The majority of cases occur in the second and third decades, with approximately 75% of cases occurring before the age of 30 years 1,12-15. There is no recognised gender predilection. Examples have however been seen in patients up to the age of 75 years. In some series there is a male predilection 12 whilst in others no such distribution is found 2
Plantar fibromatosis is most frequently present on the medial border of the sole, near the highest point of the arch. The lump is usually painless and the only pain experienced is when the nodule rubs on the shoe or floor. The overlying skin is freely movable, and contracture of the toes does not occur in the initial stages.
The typical appearance of plantar fibromatosis on magnetic resonance imaging (MRI) is a poorly defined, infiltrative mass in the aponeurosis next to the plantar muscles.
Only 25% of patients show symptoms on both feet (bilateral involvement). The disease may also infiltrate the dermis or, very rarely, the flexor tendon sheath
The soft fibroma (fibroma molle) or fibroma with a shaft (acrochordon, skin tag, fibroma pendulans) consist of many loosely connected cells and less fibroid tissue. It mostly appears at the neck, armpits or groin. The photo shows a soft fibroma of the eyelid.
CGCG lesions are found more commonly in the anterior of the maxilla and the mandible in younger people (before age 20). They are characterized by large lesions that expand the cortical plate and can resorb roots and move teeth. They are composed of multi-nucleated giant cells. CGCG has a slight predilection for females.
Radiographically :
It appears as multilocular radiolucencies of bone.The margin of the lesion has scalloped appearance and is well demarcated. Resorption and divergence of roots is also seen.
There are two types of CGCG's, non-aggressive and aggressive. The former has a slow rate of growth and thus less likely to resorb roots and perforate the cortical plate. The aggressive form has rapid growth and thus is much more likely to resorb roots and perforate the cortical plate. It also has a high rate for recurrence and can be painful and cause paresthesia.
Differential diagnosis to include: odontogenic keratocyst (OKC), ameloblastoma, odontogenic myxoma, hemangioma, central odontogenic fibroma, hyperparathyroid tumor, and cherubism.
Giant-cell fibroma is a type of fibroma not associated with trauma or irritation. It can occur at any age and on a mucous membrane surface. The most common oral locations are on the gingiva of the mandible, tongue, and palate. It is a localized reactive proliferation of fibrous connective tissue.
Giant-cell fibroma (GCF) is a benign non-neoplastic lesion first described by Weathers and Callihan (1974). It occurs in the first three decades of life and predominates in females (Houston, 1982; Bakos, 1992). Clinically, the GCF presents as an asymptomatic, papillary and pedunculated lesion. The most predominant location is the mandibular gingiva (Houston, 1982; Bakos, 1992). Histologically, the GCF is distinctive, consisting of fibrous connective tissue without inflammation and covered with stratified squamous hyperplastic epithelium. The most characteristic histological feature is the presence of large spindle-shaped and stellate-shaped mononuclear cells and multinucleated cells. These cells occur in a variety of lesions, such as the fibrous papule of the nose, ungual fibroma, acral fibrokeratoma, acral angiofibroma and desmoplastic fibroblastoma (Swan, 1988; Pitt et al., 1993; Karabela-Bouropoulou et al., 1999; Jang et al., 1999).
Despite many studies, the nature of the stellated multinucleate and mononuclear cell is not clear (Weathers and Campbell, 1974; Regezi et al., 1987; Odell et al., 1994; Magnusson and Rasmusson, 1995).
The hard fibroma (fibroma durum) consists of many fibres and few cells, e.g. in skin it is called dermatofibroma (fibroma simplex or nodulus cutaneous). A special form is the keloid, which derives from hyperplastic growth of scars.
Central giant-cell granuloma (CGCG) is a benign condition of the jaws. It is twice as likely to affect women and is more likely to occur in 20- to 40-year-old people. Central giant-cell granulomas are more common in the mandible and often cross the midline.
Plantar fascial fibromatosis, also known as Ledderhose's disease, Morbus Ledderhose, and plantar fibromatosis, is a relatively uncommon non-malignant thickening of the feet's deep connective tissue, or fascia. In the beginning, where nodules start growing in the fascia of the foot the disease is minor . Over time walking becomes painful. The disease is named after Dr. Georg Ledderhose, a German surgeon who described the condition for the first time in 1894. A similar disease is Dupuytren's disease, which affects the hand and causes bent hand or fingers.
As in most forms of fibromatosis, it is usually benign and its onset varies with each patient. The nodules are typically slow growing and most often found in the central and medial portions of the plantar fascia. Occasionally, the nodules may lie dormant for months to years only to begin rapid and unexpected growth. Options for intervention include radiation therapy, cryosurgery, treatment with collagenase clostridium histolyticum, or surgical removal only if discomfort hinders walking.
PCNA and Ki67 immunoreactivity happens in case of fibroma and peripheral granuloma.
A nonossifying fibroma (also called fibroxanthoma) is a common benign bone tumor in children and adolescents. However, it is controversial whether it represents a true neoplasm or rather a developmental disorder of growing bone. Radiographically, the tumor presents as a well marginated radiolucent lesion, with a distinct multilocular appearance. These foci consist of collagen rich connective tissue, fibroblasts, histiocytes and osteoclasts. They originate from the growth plate, and are located in adjacent parts of the metaphysis and diaphysis of long bones, most often of the legs. No treatment is needed in asymptomatic patients and spontaneous remission with replacement by bone tissue is to be expected.
Multiple nonossifying fibromas occur in Jaffe-Campanacci syndrome in combination with cafe-au-lait spots, mental retardation, hypogonadism, ocular and cardiovascular abnormalities.
The tibia is the most commonly involved bone, accounting for 85% of cases. It is usually painless, although there may be localized pain or fracture, and presents as a localized firm swelling of the tibia in children less than two decades old (median age for males 10, females 13). Several authors have related this non-neoplastic lesion to adamantinoma - a tumor involving subcutaneous long bones - stating the common cause to be fibrovascular defect. However, the latter is distinguished from an osteofibrous dysplasia by the presence of soft tissue extension, intramedullary extension, periosteal reaction and presence of hyperchromic epithelial cells under the microscope.
Osteofibrous dysplasia may also be mistaken for fibrous dysplasia of bone, although osteofibrous dysplasia is more likely to show an immunohistochemical reaction to osteonectin, neurofibromin, and S-100 protein.
frequency:- rare type of cyst
It can occur at any age, mostly between 2nd and 3rd decade of life.
Diameter is 2 to 4 cm
swelling pain maybe present.
intra bony expansions may produce hard bony expansion.
may perforate cortical bones
also it extends to soft tissue
maybe asymptomatic
The most common locations are the shaft and epyphises of long bones (fibula and humerus) but the spine, metatarsal bones, and ilium have been involved as well. Radiologic examination evidences osteolytic areas with a lobulated framework comprising radiolucent and radiodense foci admixed to speckled calcification. Cortical destruction is a common finding with no soft tissue expansion in many cases. Histopathology of the lesion shows large areas of mature fibrous stroma undergoing hyaline cartilage metaplasia resulting in conspicuous lobules or gradual transformation into chondroid foci. Both hyaline cartilage and chondroid in turn undergo calcification and endochondral cancellous bone formation mimicking epiphyseal plate-like cartilage.
Differential diagnosis is concerned with fibrocartilaginous dysplasia of bone, desmoplastic fibroma, low-grade fibrosarcoma, chondromyxoid fibroma and low-grade chondrosarcoma.
A full account of imaging findings on radiography, bone scan, CT and magnetic resonance has been provided by Sumner et al.
The ovarian fibroma, also fibroma, is a benign sex cord-stromal tumour.
Ovarian fibromas represent 4% of all ovarian neoplasms. They tend to occur mostly during perimenopause and postmenopause, the median age having been reported to be about 52 years, and they are rare in children. Lesions tend to be asymptomatic. If symptoms are present, the most common one is abdominal pain.
On gross pathology, they are firm and white or tan. On microscopic examination, there are intersecting bundles of spindle cells producing collagen.
There may be thecomatous areas (fibrothecoma). The presence of an ovarian fibroma can cause ovarian torsion in some cases.
The central odontogenic fibroma is a rare benign odontogenic tumor. It is more common in adults, with the average age being 40. It is twice as likely to affect women than men. It is usually found either in the anterior maxilla or the posterior mandible. Radiographically it presents with either radiolucency or mixed radiolucency/opaque. The simple type is characterized by delicate fibrillar stroma of collagen containing fibroblasts; the WHO type is characterized by more mature fibrillar stroma of collagen. Treatment is by surgical removal
Osteofibrous dysplasia (also known as ossifying fibroma) is a rare, benign non-neoplastic condition with no known cause. It is considered a fibrovascular defect. Campanacci described this condition in two leg bones, the tibia and fibula, and coined the term. This condition should be differentiated from Nonossifying fibroma and fibrous dysplasia of bone.
Fibrocartilaginous mesenchymoma of bone is (FCMB) is an extremely rare tumor first described in 1984. Fewer than 20 cases have been reported, with patient ages spanning from 9 to 25 years, though a case in a male infant aged 1 year and 7 months has been reported. Quick growth and bulky size are remarkable features of this tumor.
A schwannoma is a usually-benign nerve sheath tumor composed of Schwann cells, which normally produce the insulating myelin sheath covering peripheral nerves.
Symptoms may include:
- Swelling in the extremities (arms or legs), also called peripheral edema; the swelling often is painless.
- Difficulty in moving the extremity that has the tumor, including a limp.
- Soreness localized to the area of the tumor or in the extremity.
- Neurological symptoms.
- Pain or discomfort: numbness, burning, or "pins and needles."
- Dizzyness and/or loss of balance.
Dermatofibromas are hard solitary slow-growing papules (rounded bumps) that may 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 applying lateral pressure, there is a central depression of the dermatofibroma. Although typical dermatofibromas cause little or no discomfort, itching and tenderness can occur. Dermatofibromas can be found anywhere on the body, but most often they are found on the legs and arms. They occur most often in women; the male to female ratio is about 1:4. The age group in which they most commonly occur is 20 to 45 years.
Some physicians and researchers believe dermatofibromas form as a reaction to previous injuries such as insect bites or thorn pricks. They are composed of disordered collagen laid down by fibroblasts. Dermatofibromas are classed as benign skin lesions, meaning they are completely harmless, though they may be confused with a variety of subcutaneous tumours. Deep penetrating dermatofibromas may be difficult to distinguish, even histologically, from rare malignant fibrohistocytic tumours like dermatofibrosarcoma protuberans.
Dermatofibromas typically have a positive "buttonhole sign", or central dimpling in the center.
The calcifying odotogenic cyst or the Gorlin cyst, now known in the WHO Classification of Tumours as the calcifying cystic odontogenic tumor, is a benign odontogenic tumor of cystic type most likely to affect the anterior areas of the jaws. It is most common in people in their second to third decades but can be seen at almost any age. On radiographs, the calcifying odontogenic cyst appears as a unilocular radiolucency (dark area). In one-third of cases, an impacted tooth is involved. Microscopically, there are many cells that are described as "ghost cells", enlarged eosinophilic epithelial cells without nuclei.