Long-Term Remission of Peritoneal Lymphangiomatosis with Sirolimus Therapy: A Case Report with 8-Year Follow-Up

Our plain-English summary of Long-Term Remission of Peritoneal Lymphangiomatosis with Sirolimus Therapy: A Case Report with 8-Year Follow-Up, by P. Jovanovic, E. Zerem, M. Basic Denjagic, et al (Case Reports in Gastroenterology, 2026).

The study

• Authors: P. Jovanovic, E. Zerem, M. Basic Denjagic, Z. Rifatbegovic, E. Iljazovic, J. Mustedanagic-Mujanovic, L. Tupkovic Rakovac, I. Cerkez
• Journal: Case Reports in Gastroenterology
• Published: 2026;20(1):8-15
• Type of article: Case report
• Read it: PubMed · Publisher · Free full text

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, this is one of the longest documented remissions of peritoneal lymphangiomatosis under mechanistic target of rapamycin (mTOR) inhibition.

© 2025 The Author(s). Published by S. Karger AG, Basel.

In plain English

This case report described one patient, a 45-year-old woman treated in Tuzla, Bosnia and Herzegovina, with peritoneal lymphangiomatosis, a rare benign disorder of lymphatic vessels in the abdominal lining. It is not an asbestos study. Peritoneal mesothelioma appears only as a disease doctors considered before diagnosis, and the patient worked in a nonindustrial job with no relevant environmental exposures. Symptoms began in May 2014, and scans showed ascites (abdominal fluid), a thickened abdominal lining and cysts. Tissue taken at surgery in May 2015 confirmed the diagnosis, and staining ruled out mesothelial and lymphoid cancers. After conservative treatment failed, the drug sirolimus, 2 mg daily, was started in August 2016. Full functional status (Karnofsky score 100%) returned in under 2 months. As of February 2025 the authors reported complete clinical and radiologic remission for 8 years and 7 months. Type 2 diabetes developed during treatment and was controlled with insulin. The authors concluded that sirolimus, though off-label here, can be highly effective in diffuse unresectable disease.

We wrote this plain-English summary from the full article with the help of AI, and a second AI model checked each sentence against the article. It is not the authors' wording; the abstract and the article are their own account.

What the study did and found

• Design: Case report (single patient)
• Who was studied: One 45-year-old woman with a history of surgically repaired atrial septal defect, seen at University Clinical Center Tuzla, Bosnia and Herzegovina. Symptoms began in May 2014 and follow-up ran to February 2025.
• Exposure: No asbestos exposure is reported. The article says the patient had a nonindustrial occupation with no relevant environmental exposures. The intervention studied was sirolimus, an mTOR inhibitor, at 2 mg daily from August 2016.
• Main results:
  • Tissue from the May 2015 exploratory laparotomy showed thin-walled vascular spaces consistent with lymphangiomatosis. Immunohistochemistry ruled out mesothelial and lymphoid malignancies; peritoneal mesothelioma had been among the differential diagnoses.
  • Sirolimus was started in August 2016 at 2 mg daily. Trough levels were kept in a target range of 5 to 15 ng/mL, and the most recent value was 15.88 ng/mL in February 2025.
  • The patient's functional status fully recovered (Karnofsky score 100%) in under 2 months of sirolimus therapy.
  • As of February 2025, the patient had remained in complete clinical and radiologic remission for 8 years and 7 months, with no recurrent symptoms and no hospitalizations.
  • Abdominal MRI in August 2023 showed no residual or recurrent peritoneal lymphangiomatosis.
  • Type 2 diabetes mellitus was diagnosed during therapy. The authors considered it likely multifactorial (genetic predisposition and potential sirolimus-related metabolic effects). It was managed with diet and insulin, with HbA1c at 6.7%.
• The authors' conclusion: The authors concluded that peritoneal lymphangioma is diagnostically challenging and often mimics malignancy. In their view, sirolimus, though off-label in this context, can be highly effective in diffuse unresectable disease, and with proper monitoring may induce complete and durable remission.
• Limitations the authors state:
  • Molecular and genetic testing was not performed.
  • Because the surgical specimen was fragmented, margin status could not be assessed.
  • Follow-up was individualized rather than based on a formal international guideline.

Topics

Medical terms in this study, explained in our Lexicon: Mesothelioma, Ultrasound, Ascites, Laparotomy, Histopathology.

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