Angioleiomyoma | |
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Other names | Angiomyoma, vascular leiomyoma |
Angioleiomyoma, smooth muscles encircling dilatated blood vessels | |
Specialty | Oncology, rheumatology |
Symptoms | Pain (with or without tenderness), slow-growing benign mass in the subcutaneous region of the extremities. [1] |
Usual onset | Can occur at any age, with a peak in middle age. [2] |
Causes | Unknown. |
Differential diagnosis | Many, including ganglion cyst, traumatic neuroma, schwannoma, eccrine spiradenoma, angiolipoma, fibroma and synovial sarcoma. |
Treatment | Surgical excision. |
Frequency | Rare, more common in women. |
Angioleiomyoma (vascular leiomyoma, angiomyoma) of the skin is thought to arise from vascular smooth muscle, and is generally acquired. [3] Angioleiomyomas appear as small (<2 cm), firm, movable, slow growing subcutaneous nodules. Pain is a common symptom. They are most commonly seen on the extremities. The cause of angioleiomyoma is unknown.
Angioleiomyomas present as solitary, small, slow-growing, firm, movable, subcutaneous nodules that typically measure less than 2 cm in size. Pain is the most obvious symptom of angioleiomyomas. Wind and cold exposure can set off paroxysmal episodes of pain. It is believed to be caused by smooth muscle contractions that are active and cause localized ischemia. It seems that rest is a relieving aspect. [4] Although this tumor can develop anywhere in the body, it usually first appears in the extremities, then the head and trunk. The lesion is usually located in the subcutis, however it can also appear in the dermis. [5]
The exact etiology of angioleiomyomas is yet unknown. [6] Aetiological aspects that have been suggested include minor trauma, venous stasis, and hormonal alterations, particularly those related to estrogen. The venous stasis idea is supported by the observation of chronic inflammatory cell infiltrates in certain lesions. [4] Angioleiomyomas have been linked to Epstein-Barr virus (EBV) infection in immunocompromised patients. [5]
Histologically, angioleiomyomas have a unique pattern with several tiny vascular gaps surrounded by bundles of spindle-shaped smooth muscle cells. [7]
Angioleiomyoma appears to be benign on ultrasonography, with well-defined boundaries and a homogenous shape. High resistance in intratumour arteries is revealed by a color Doppler examination, indicating the existence of muscle arteries. [4]
Angioleiomyoma are treated by surgical excisision. [5]
Angioleiomyomas make around 5% of all benign soft tissue neoplasms. [4] They primarily afflict people in their third and fifth decades of life, and they are more common in women than in men. [8]
Veins are blood vessels in the circulatory system of humans and most other animals that carry blood towards the heart. Most veins carry deoxygenated blood from the tissues back to the heart; exceptions are those of the pulmonary and fetal circulations which carry oxygenated blood to the heart. In the systemic circulation, arteries carry oxygenated blood away from the heart, and veins return deoxygenated blood to the heart, in the deep veins.
Calcinosis cutis is an uncommon condition marked by calcium buildup in the skin and subcutaneous tissues. Calcinosis cutis can range in intensity from little nodules in one area of the body to huge, crippling lesions affecting a vast portion of the body. Five kinds of the condition are typically distinguished: calciphylaxis, idiopathic calcification, iatrogenic calcification, dystrophic calcification, and metastatic calcification.
A leiomyoma, also known as a fibroid, is a benign smooth muscle tumor that very rarely becomes cancer (0.1%). They can occur in any organ, but the most common forms occur in the uterus, small bowel, and the esophagus. Polycythemia may occur due to increased erythropoietin production as part of a paraneoplastic syndrome.
Hemangioendotheliomas are a family of vascular neoplasms of intermediate malignancy.
Lymphangiomas are malformations of the lymphatic system characterized by lesions that are thin-walled cysts; these cysts can be macroscopic, as in a cystic hygroma, or microscopic. The lymphatic system is the network of vessels responsible for returning to the venous system excess fluid from tissues as well as the lymph nodes that filter this fluid for signs of pathogens. These malformations can occur at any age and may involve any part of the body, but 90% occur in children less than 2 years of age and involve the head and neck. These malformations are either congenital or acquired. Congenital lymphangiomas are often associated with chromosomal abnormalities such as Turner syndrome, although they can also exist in isolation. Lymphangiomas are commonly diagnosed before birth using fetal ultrasonography. Acquired lymphangiomas may result from trauma, inflammation, or lymphatic obstruction.
May–Thurner syndrome (MTS), also known as the iliac vein compression syndrome, is a condition in which compression of the common venous outflow tract of the left lower extremity may cause discomfort, swelling, pain or iliofemoral deep vein thrombosis.
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.
Glomangiosarcoma is a low grade tumor of the soft tissue. They rarely metastasize, but metastases are possible. It is also known as malignant glomus tumor. Positive staining for vimentin has been reported.
Eccrine angiomatous hamartoma (EAH), first described by Lotzbeck in 1859, is a rare benign vascular hamartoma characterized histologically by a proliferation of eccrine and vascular components. EAH exists on a spectrum of cutaneous tumors that include eccrine nevus, mucinous eccrine nevus and EAH. Each diagnostic subtype is characterized by an increase in the number as well as size of mature eccrine glands or ducts, with EAH being distinguished by the added vascular component.
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.
Infantile digital fibromatosis (IDF), also termed inclusion body fibromatosis, Reye tumor, or Reye's tumor, usually occurs as a single, small, asymptomatic, nodule in the dermis on a finger or toe of infants and young children. IMF is a rare disorder with approximately 200 cases reported in the medical literature as of 2021. The World Health Organization in 2020 classified these nodules as a specific benign tumor type in the category of fibroblastic and myofibroblastic tumors. IDF was first described by the Australian pathologist, Douglas Reye, in 1965.
A tufted angioma, also known as an acquired tufted angioma, angioblastoma, angioblastoma of Nakagawa, hypertrophic hemangioma, progressive capillary hemangioma, and tufted hemangioma usually develops in infancy or early childhood on the neck and upper trunk, and is an ill-defined, dull red macule with a mottled appearance, varying from 2 to 5 cm in diameter.
Angiolipoleiomyoma is an acquired, solitary, asymptomatic acral nodule, characterized histologically by well-circumscribed subcutaneous tumors composed of smooth muscle cells, blood vessels, connective tissue, and fat.
Intravascular papillary endothelial hyperplasia (IPEH), also known as Masson's hemangio-endotheliome vegetant intravasculaire, Masson's lesion, Masson's pseudoangiosarcoma, Masson's tumor, and papillary endothelial hyperplasia, is a rare, benign tumor. It may mimic an angiosarcoma, with lesions that are red or purplish 5-mm to 5-cm papules and deep nodules on the head, neck, or upper 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.
Spiradenomas (SA) are rare, benign cutaneous adnexal tumors that may progress to become their malignant counterparts, i.e. spiradenocarcinomas (SAC). Cutaneous adnexal tumors are a group of skin tumors consisting of tissues that have differentiated towards one of the four primary adnexal structures found in normal skin: hair follicles, sebaceous sweat glands, apocrine sweat glands, and eccrine sweat glands. SA and SAC tumors were regarded as eccrine gland tumors and termed eccrine spiradenomas and eccrine spiradenocarcinomas, respectively. However, more recent studies have found them to be hair follicle tumors and commonly term them spiradenomas and spiradenocarcinomas, respectively. Further confusing the situation, SA-like and SAC-like tumors are also 1) manifestations of the inherited disorder, CYLD cutaneous syndrome (CCS), and 2) have repeatedly been confused with an entirely different tumor, adenoid cystic carcinomas of the salivary gland. Here, SA and SAC are strictly defined as sporadic hair follicle tumors that do not include the hereditary CCS spiradenomas and heridtary spiradenocarcinoms of CCS or the adenoid cystic carcinomas.
Hereditary leiomyomatosis and renal cell carcinoma (HLRCC) or Reed's syndrome is rare autosomal dominant disorder associated with benign smooth muscle tumors and an increased risk of renal cell carcinoma. It is characterised by multiple cutaneous leiomyomas and, in women, uterine leiomyomas. It predisposes individuals to renal cell cancer, an association denominated hereditary leiomyomatosis and renal cell cancer. It is also associated with increased risk of uterine leiomyosarcoma. The syndrome is caused by a mutation in the fumarate hydratase gene, which leads to an accumulation of fumarate. The inheritance pattern is autosomal dominant and screening can typically begin in childhood.
Stasis papillomatosis is a disease characterized by chronic congestion of the extremities, with blood circulation interrupted in a specific area of the body. A consequence of this congestion and inflammation is long-term lymphatic obstruction. It is also typically characterized by the appearance of numerous papules. Injuries can range from small to large plates composed of brown or pink, smooth or hyperkeratotic papules. The most typical areas where injuries occur are the back of the feet, the toes, the legs, and the area around a venous ulcer formed in the extremities, although the latter is the rarest of all. These injuries include pachydermia, lymphedema, lymphomastic verrucosis and elephantosis verrucosa. The disease can be either localized or generalized; the localized form makes up 78% of cases. Treatment includes surgical and pharmaceutical intervention; indications for partial removal include advanced fibrotic lymphedema and elephantiasis. Despite the existence of these treatments, chronic venous edema, which is a derivation of stasis papillomatosis, is only partially reversible. The skin is also affected and its partial removal may mean that the skin and the subcutaneous tissue are excised. A side effect of the procedure is the destruction of existing cutaneous lymphatic vessels. It also risks papillomatosis, skin necrosis and edema exacerbation.
Fibro-adipose vascular anomaly, also known as FAVA, is a type of vascular anomaly that is both rare and painful. FAVA is characterized by tough fibrofatty tissue taking over portions of muscle, most often contained within a single limb. FAVA also causes venous and/or lymphatic abnormalities.
Cutaneous leiomyoma, also known as leiomyoma cutis, or cutaneous leiomyomata, is a benign skin tumor made of smooth muscle cells. There are three different types of cutaneous leiomyomas, genital leiomyomas, angioleiomyomas, and piloleiomyomas. Cutaneous leiomyomas can occur sporadically or as a part of a genetic condition. Cutaneous leiomyomas are diagnosed by histopathology and treated by surgical excision.