๐ ็ธฝ็ฎ้ ๏ฝ ๐ ่ฑๆๅๆ๏ผๆฌ็ฏ๏ผ ๏ฝ ๐ ๅฎๆด็ฟป่ญฏ ๏ฝ โญ ็ฒพ่ฏ็ญ่จ
Vulval leiomyomatosis
Vulval leiomyomatosis
Clinical features This rare condition is characterized by multiple leiomyomas in the vulva associated with esophageal leiomyomas.1โ8 The vulval tumors may appear before, concomitantly, or after the development of esophageal lesions. Involvement of the clitoris is sometimes noted. Patients can also present with Alport syndrome, characterized by inherited glomerulonephritis, ocular abnormalities, and deafness.5
Pathogenesis and histologic features The pathogenesis of the disease is unknown, but deletions and mutations in the COL4A6 and COL4A5 genes have been described.5,7 These
The spindled cells are positive for smooth-muscle actin, muscle-specific actin, and calponin but negative for desmin.
Differential diagnosis This tumor must be distinguished from myofibroma, intravascular nodular fasciitis, and vascular leiomyoma.