Abstract
BACKGROUND: Luteinized thecoma with sclerosing peritonitis (LTSP) is a very rare condition, and its clinical management is not evidence-based. Here we describe a case of long-term disease control achieved with leuprorelin and tamoxifen therapy. CASE PRESENTATION: A 18-year-old woman with acute abdomen underwent surgical removal of an ovarian mass and received diagnosis of LTSP. Treatment plan consisted of leuprorelin and tamoxifen, followed by a good instrumental response. After 5 years, leuprorelin was stopped, and the patient continued tamoxifen alone. Ten years after diagnosis, she is still disease free. CONCLUSION: Even in the absence of solid evidence, the combination of leuprorelin and tamoxifen could be considered as a possible medical treatment of LTSP. Considering the limitations related to the rarity of disease, further studies are needed to improve its management.