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Extramammary Paget's disease (EMPD) is a rare and slow-growing cancer, which occurs within the epithelial tissues [1] and accounts for 6.5% of all Paget's disease. [2] This disease presents similarly to the more conventional form of mammary Paget's disease (MPD). [ 3 ]
Vulvar cancer accounts for about 5% of all gynecological cancers and typically affects women in later life. Five-year survival rates in the United States are around 70%. [1] Symptoms of vulvar cancer include itching, a lump or sore on the vulva which does not heal and/or grows larger, and sometimes discomfort/pain/swelling in the vulval area.
Extramammary Paget's disease; Tumors of melanocytes, noninvasive; Invasive disease (vulvar carcinoma) The ISSVD further revised this classification in 2004, replacing the three-grade system with a single-grade system in which only the high-grade disease is classified as VIN. VIN is subdivided into: (Robbins Pathological Basis of Disease, 9th Ed)
[30] [85] Based on the cellular origin and histology, vulvar cancers are classified into squamous cell carcinomas, melanomas, basal cell carcinomas, adenocarcinomas, sarcomas and invasive extramammary Paget's disease. [85] Squamous cell carcinomas represent the most common variant of vulvar cancers and account for approximately 75%. [85]
Paget's disease may refer to several conditions described by Sir James Paget, surgeon and pathologist: Paget's disease of bone (most common use of the term "Paget's disease") Paget's disease of the breast; Paget–Schroetter disease; Paget's abscess; Extramammary Paget's disease (EMPD)
Vulvar cancers are those malignant neoplasms that originate from vulvar epithelium, while vulvar sarcomas develop from non-epithelial cells such as bone, cartilage, fat, muscle, blood vessels, or other connective or supportive tissue. [7] Epithelial and mesenchymal tissue are the origin of vulvar tumors. [1]
The nodules were solid or cystic; [10] 0.4 to 2.5 cm. in widest dimension; [9] [10] and usually located in the grooves (termed interlabial sulci or interlabial folds) between the labia majora and labia minora, [9] the clitoris, or the region between the vulva and anus (i.e. Frenulum of labia minora). [4]
Dermetological diseases: lichen sclerosus, lichen planus; Neoplasm: Paget's disease, vulvar carcinoma; Neurologic disorder: neuralgia secondary to herpes virus, spinal nerve injury, [23] pudendal nerve entrapment; In recent years, diagnostic algorithms for the diagnosis of the various sub-types of and causes of vulvar pain have been developed ...