Pulmonary Hamartoangiomyomatosis (lymphangiomyomatosis) in Tuberous Sclerosis

A Case in which Clinical Course had been Modified by Previous Surgical Intervention

Abstract

A 42-year-old woman was referred to this institution because of persistent air leak associated with left spontaneous pneumothorax. She had undergone a radical hysterectomy with bilateral oophorectomy and left nephrectomy one year previously. The renal tumor was identified as an angiomyolipoma. This, along with the characteristic dermal lesions led to the diagnosis of tuberous sclerosis.
Preoperative thin-slice chest CT showed multiple thin-walled, round and cystic air spaces in the pulmonary parenchyma bilaterally, as well as the left pneumothorax.
Through a left thoracotomy, suture ligation and laser ablation of the pulmonary bullae was done. Examination of the biopsy specimen of the lung showed hamartoangiomyomatosis with multiple bullae. The proliferating smooth muscle in the walls of the bullae was positively stained by HMB45 monoclonal antibody. The patient recovered uneventfully, and was doing well three years later without medications.
The prior surgical castration may have prevented progression of the disease.