Lymphangiolipomatosis: A New Pathological Entity

Abstract

To our knowledge lymphangiolipomatosis is a pathological entity that has not been previously described in any tissue. We report the initial case of this lesion noted at routine transurethral prostatectomy. CASE REPORT A 66-year-old white man with a history of a coronary artery bypass graft and mild cerebrovascular accident presented in acute urinary retention after ingesting a nonprescription cold remedy. An indwelling catheter was placed and he returned for urological evaluation 4 days later. Retrospectively the International Prostate Symptom Score was 20 before catheterization. The catheter was removed but the patient was unable to urinate and the catheter was reinserted. An escalating dose of doxazosin was instituted in anticipation of a repeat voiding trial. Several days later the patient elected transurethral resection of the prostate. Digital rectal examination revealed a moderately enlarged, clinically benign prostate. Prostate specific antigen (PSA) was normal at 3.4 ng./ml. Preoperative evaluation, including urinalysis, serological testing, electrocardiography, chest radiography and physical examination, was normal. Transurethral prostatectomy was performed primarily with an electrovaporization technique supplemented with standard cutting loop electrosurgical resection. The prostatic fossa evidenced typical trilobate prostatic enlargement and the bladder was mildly trabeculated. At resection the prostate had an unusual pale appearance but the operation proceeded routinely. Convalescence was uneventful. Followup demonstrated negligible post-void residual urine and a marked decrease in International Prostate Symptom Score of 5. Gross inspection of the prostatic specimen revealed gray pink tissue fragments floating at the surface of a 10% formalin solution. Microscopy showed diffuse infiltration of the prostatic stroma by clusters of mature adipocytes associated with dilated endothelial lined vessels, comprising approximately 50% of specimen volume (parts A and B of figure). The vessels lacked muscular walls and intraluminal erythrocytes. These lining cells failed to stain for PSA (part C of figure). Immunoperoxidase staining for factor VIII related antigen confirmed endothelial cells lining the vessels (part D of figure). Glandular and stromal hyperplasia represented 10% of specimen volume and there were scattered foci of periglandular chronic inflammation. No malignant elements were present.