Department of Pathology, Habib Bourguiba University Hospital. SFAX- Tunisia (LA, KA, SM, RK, TSB); Department of nephrology, Hedi Chaker University Hospital. SFAX- Tunisia (MK, MBH, JH); Department of surgery, Habib Bourguiba University Hospital. SFAX- Tunisia (NA, MIB)
The leiomyomatosis is a benign lesion, which consists of a tumor-like hypertrophy of the smooth muscle. Leiomyomatosis tends to affect young boys (mean age : 6 years) and women (mean age : 40 years). In contrast to the renal lesions which are less severe among women, the leiomyomatosis is as severe in women as in men. This disease affects preferentially the lower third of oesophagus, and may extend down into the cardia. The oesophageal lesions can be diffuse, extensive or multinodular. Clinical symptoms are attributed to signs of compression of the oesophagus or neighbouring organs. They commonly include progressive dysphagia, vomiting or dyspepsia; less frequently, retrosternal pains, dyspnea, cough or weight loss. The Barium contrast study is the best diagnosis tool revealing a widened upper oesophagus and a smooth tapered narrowing of the distal oesophagus with reduced oesophageal motility; such findings often lead to the misdiagnosis of achalasia in up to 50% of patients. The leiomyomatosis is histologically defined by a proliferation of interlacing whorls of smooth muscle cells without evidence of mitotic figures or atypia, and staining with smooth muscle actin (SMA).