Abstract

Introduction: Peritoneal lymphangiomatosis is an exceptionally rare lymphatic malformation in adults that may closely mimic malignant peritoneal conditions, including peritoneal carcinomatosis and pseudomyxoma peritonei. According to the International Society for the Study of Vascular Anomalies (ISSVA), peritoneal lymphangiomatosis is classified as a complex lymphatic anomaly within the spectrum of Generalized Lymphatic Anomaly (GLA). Materials and methods: We present the case of a female patient who was evaluated for large adnexal cystic masses and uterine fibroids at the Instituto de Cancerología y Dr. Bernardo del Valle S Hospital (INCAN), Guatemala City, Guatemala. Results: A 43-year-old woman presented with a two-year history of continuous abdominal pain and progressive abdominal enlargement. Physical examination revealed a welldefined, non-tender abdominopelvic mass extending from the pelvis to the mesogastrium. Computed tomography demonstrated a giant multilobulated abdominopelvic tumor with cystic components, internal calcifications, and necrotic areas, without ascites or distant metastasis. Tumor markers were negative. Exploratory laparotomy was performed, including total hysterectomy with bilateral salpingo-oophorectomy, omentectomy, appendectomy, and peritoneal lavage. Intraoperative findings revealed a giant uterine leiomyoma and multiple cystic peritoneal implants involving the root of mesentry, omentum, appendix, and rectosigmoid region. Definitive histopathological and immunohistochemical analysis confirmed the diagnosis of peritoneal lymphangiomatosis. Conclusion: This case highlights the importance of recognizing peritoneal lymphangiomatosis as a benign but deceptive entity that can simulate advanced peritoneal malignancy

Keywords

peritoneal lymphangiomatosis, giant uterine leiomyoma, complex lymphatic anomaly