Extraskeletal chondroma | |
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Other names | Chondroma of soft parts |
Chondroma of the soft parts located in the little toe. | |
Specialty | Dermatology |
Extraskeletal chondroma is a cutaneous condition, a rare benign tumor of mature cartilage. [1]
An extraskeletal chondroma often manifests clinically as a nodular soft tissue mass that steadily enlarges without discomfort and may exist for varying lengths of time before diagnosis. [2] The fingers and toes are the most often affected areas. [3] The tumor is oval in shape and clearly defined; it rarely has a diameter more than 3 cm. [4]
Rather of coming from mature osseous or cartilaginous tissue, it is believed that this tumor originates from the fibrous stroma of soft tissues. [2] Micro trauma that occurs repeatedly could be the trigger. [5]
A positive diagnosis can only be made through a histological analysis. [5] An extraskeletal osteochondroma may show up on traditional radiography as a well-circumscribed, lobulated mass with intense core mineralization. [6] The extraskeletal position of the mass can be verified by CT, which can also reveal foci of ossification or calcification that help narrow the diagnosis to extraskeletal chondroma. [7] The most effective radiologic method is magnetic resonance imaging (MRI), which is able to characterize the tumor's extent, contour, shape, and intensity as well as its relationship to any calcifications or surrounding structures. [5]
According to histopathology, the tumor has a lot of lobular structures and certain sections that resemble mucus-containing hyaline cartilage. Eosinophilic cells with a spindle or circular form are sparsely distributed throughout the tumor matrix. Tumor cells enlarge and take on a rounded appearance, resembling hyaline cartilage. Incomplete lacuna-like voids can occasionally occur in the surrounding matrix. In few circumstances, multinuclear cells and nuclear atypia are present in benign chondroma as well. [4]
The only effective treatment is surgery, however recurrence is frequent. [8]
Chondrosarcoma is a bone sarcoma, a primary cancer composed of cells derived from transformed cells that produce cartilage. A chondrosarcoma is a member of a category of tumors of bone and soft tissue known as sarcomas. About 30% of bone sarcomas are chondrosarcomas. It is resistant to chemotherapy and radiotherapy. Unlike other primary bone sarcomas that mainly affect children and adolescents, a chondrosarcoma can present at any age. It more often affects the axial skeleton than the appendicular skeleton.
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.
Connective tissue disease, also known as connective tissue disorder, or collagen vascular diseases, refers to any disorder that affect the connective tissue. The body's structures are held together by connective tissues, consisting of two distinct proteins: elastin and collagen. Tendons, ligaments, skin, cartilage, bone, and blood vessels are all made of collagen. Skin and ligaments contain elastin. The proteins and the body's surrounding tissues may suffer damage when these connective tissues become inflamed.
A hamartoma is a mostly benign, local malformation of cells that resembles a neoplasm of local tissue but is usually due to an overgrowth of multiple aberrant cells, with a basis in a systemic genetic condition, rather than a growth descended from a single mutated cell (monoclonality), as would typically define a benign neoplasm/tumor. Despite this, many hamartomas are found to have clonal chromosomal aberrations that are acquired through somatic mutations, and on this basis the term hamartoma is sometimes considered synonymous with neoplasm. Hamartomas are by definition benign, slow-growing or self-limiting, though the underlying condition may still predispose the individual towards malignancies.
Osteochondromas are the most common benign tumors of the bones. The tumors take the form of cartilage-capped bony projections or outgrowth on the surface of bones (exostoses). It is characterized as a type of overgrowth that can occur in any bone where cartilage forms bone. Tumors most commonly affect long bones about the knee and in the forearm. Additionally, flat bones such as the pelvis and scapula may be affected.
Myositis ossificans comprises two syndromes characterized by heterotopic ossification (calcification) of muscle. The World Health Organization, 2020, has grouped myositis ossificans together with fibro-osseous pseudotumor of digits as a single specific entity in the category of fibroblastic and myofibroblastic tumors.
Subungual exostosis is a type of non-cancerous bone tumor of the chondrogenic type, and consists of bone and cartilage. It usually projects from the upper surface of the big toe underlying the nailbed, giving rise to a painful swelling that destroys the nail. Subsequent ulceration and infection may occur.
Chondroblastoma is a rare, benign, locally aggressive bone tumor that typically affects the epiphyses or apophyses of long bones. It is thought to arise from an outgrowth of immature cartilage cells (chondroblasts) from secondary ossification centers, originating from the epiphyseal plate or some remnant of it.
Keutel syndrome (KS) is a rare autosomal recessive genetic disorder characterized by abnormal diffuse cartilage calcification, hypoplasia of the mid-face, peripheral pulmonary stenosis, hearing loss, short distal phalanges (tips) of the fingers and mild mental retardation. Individuals with KS often present with peripheral pulmonary stenosis, brachytelephalangism, sloping forehead, midface hypoplasia, and receding chin. It is associated with abnormalities in the gene coding for matrix gla protein, MGP. Being an autosomal recessive disorder, it may be inherited from two unaffected, abnormal MGP-carrying parents. Thus, people who inherit two affected MGP alleles will likely inherit KS.
Trevor disease, also known as dysplasia epiphysealis hemimelica and Trevor's disease, is a congenital bone developmental disorder. There is 1 case per million population. The condition is three times more common in males than in females.
Rheumatoid vasculitis is a skin condition that is a typical feature of rheumatoid arthritis, presenting as peripheral vascular lesions that are localized purpura, cutaneous ulceration, and gangrene of the distal parts of the extremities.
Aponeurotic fibroma, also known as calcifying aponeurotic fibroma, and juvenile aponeurotic fibroma is characterized by a lesion that usually presents as a painless, solitary, deep fibrous nodule, often adherent to tendon, fascia, or periosteum, on the hands and feet. The World Health Organization in 2020 reclassified aponeurotic fibroma nodules as a specific benign type of the fibroblastic and myofibroblastic tumors. Aponeurotic fibromas are diagnosed based on histopathology and treated by surgical excision. They are more common in males than females.
Benign lipoblastomatosis is a tumor consisting of fetal-embryonal adipocytes, frequently confused with a liposarcoma, affecting exclusively infants and young children, with approximately 90% of cases occurring before 3 years of age. The term lipoblastomatosis was first used by Vellios et al. in 1958, at which point the tumor became generally accepted as a distinctive entity. Today Diffuse lipoblastoma is the preferred term for lipoblastomatosis. The tumor is rare, accounting for less than 1% of all childhood neoplasm, and it has been found to be more common in males than females. It often presents as an asymptomatic rapidly enlarging mass, occurring more often in the soft tissues of the extremities.
Collagenous fibroma, also known as desmoplastic fibroblastoma, is a slow-growing, deep-set, benign fibrous tumor, usually located in the deep subcutis, fascia, aponeurosis, or skeletal muscle of the extremities, limb girdles, or head and neck regions. The World Health Organization in 2020 reclassified desmoplastic fibroblastoma/collagenous fibroma as a specific benign tumor type within the broad category of fibroblastic and myofibroblastic tumors.
Cutaneous meningioma, also known as heterotopic meningeal tissue, and rudimentary meningocele is a developmental defect, and results from the presence of meningocytes outside the calvarium.
Chondroid lipomas are deep-seated, firm, yellow tumors that characteristically occur on the legs of women. They exhibit a characteristic genetic translocation t(11;16) with a resulting C11orf95-MKL2 fusion oncogene.
A cutaneous myxoma, or superficial angiomyxoma, consists of a multilobulated myxoid mass containing stellate or spindled fibroblasts with pools of mucin forming cleft-like spaces. There is often a proliferation of blood vessels and an inflammatory infiltrate. Staining is positive for vimentin, negative for cytokeratin and desmin, and variable for CD34, Factor VIIIa, SMA, MSA and S-100.
Pseudocyst of the auricle, also known as auricular pseudocyst, endochondral pseudocyst, cystic chondromalacia, intracartilaginous auricular seroma cyst, and benign idiopathic cystic chondromalacia, is a cutaneous condition characterized by a fluctuant, tense, noninflammatory swelling on the upper half of the ear, known as the auricle or pinna. Pseudocysts of the auricle are nontender, noninflammatory cystic lesions that progress over a 4- to 12-week period, ranging from 1 to 5 cm in diameter. They are usually unilateral, often on the right ear, but can also present bilaterally.
Fibrocartilaginous mesenchymoma of bone (FCMB) is an extremely rare tumor first described in 1984. About 26 cases have been reported in literature, 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.
Bizarre parosteal osteochondromatous proliferation (BPOP), also known as Nora's lesion, is a type of non-cancerous bone tumor belonging to the group of cartilage tumors. It is generally seen in the tubular bones of the hands and feet, where it presents with a rapidly enlarging painless lump in a finger or toe.