Long-Term Remission of Peritoneal Lymphangiomatosis with Sirolimus Therapy: A Case Report with 8-Year Follow-Up
Abstract
Abstract Introduction Peritoneal lymphangiomatosis is a rare benign lymphatic disorder with diagnostic and therapeutic challenges. It may mimic peritoneal carcinomatosis or mesothelioma, often requiring histological confirmation. Management options are limited, and long-term data on targeted therapies, including sirolimus, remain scarce. Case Presentation We present the case of a 45-year-old woman with a history of surgically corrected atrial septal defect, who developed progressive abdominal distension and dysuria in May 2014. Abdominal ultrasound and contrast-enhanced abdominal computed tomography revealed diffuse ascites, peritoneal thickening, and cystic changes. Diagnostic uncertainty led to an exploratory laparotomy in May 2015, where histopathology confirmed peritoneal lymphangiomatosis. After failed conservative management including dietary modification, diuretic therapy, and repeated paracenteses, she was started on sirolimus 2 mg/day in August 2016. Her serum trough levels were maintained between 5 and 15 ng/mL. She achieved a Karnofsky Performance Score of 100% (indicating full functional capacity) within 2 months and has remained in complete clinical and radiologic remission for over 8 years. Diabetes mellitus type 2 was diagnosed during follow-up and is well controlled with insulin therapy. Conclusion This case underscores the difficulty of diagnosing peritoneal lymphangiomatosis and highlights the transformative potential of sirolimus therapy. To our knowledge,…
How strong is the evidence?
Evidence Hub reads this as a case report, done in people. Its evidence level is 5 of 5 (Very low · case / narrative / animal / in vitro / in silico). Case reports, narrative reviews, and animal, cell or computer-model studies give the weakest evidence for human health.
Read the paper
Related studies
- Drug repositioning: a brief overview
- Cyclic nucleotide phosphodiesterases in Drosophila melanogaster
- Time-resolved in silico modeling of fine-tuned cAMP signaling in platelets: feedback loops, titrated phosphorylations and pharmacological modulation
- Current View on Green Tea Catechins Formulations, Their Interactions with Selected Drugs, and Prospective Applications for Various Health Conditions
- In silico Structure-based Identification of Novel Acetylcholinesterase Inhibitors Against Alzheimer’s Disease
- Development and In Vitro Evaluation of Controlled Release Viagra® Containing Poloxamer-188 Using Gastroplus™ PBPK Modeling Software for In Vivo Predictions and Pharmacokinetic Assessments
Go deeper in Evidence Hub
Evidence Hub adds a study data sheet (design, sample size, evidence level, key numbers), an AI summary and a chat grounded in the paper's text.