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      Successful treatment of a patient with chyluria due to lymphangioleiomyomatosis using sirolimus

      case-report

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          Abstract

          Lymphangioleiomyomatosis (LAM) is a rare and progressive neoplastic disease of young woman, characterized by the proliferation of abnormal smooth muscle-like cells (LAM cells) in the lungs and axial lymphatics. A 44-year-old woman was referred to our hospital because pleural effusion was detected during a health checkup. She had chylothorax, chylous ascites, and chyluria, and her computed tomography scan showed a solid tumor in the pelvis. Surgical biopsy was performed; she was diagnosed as having LAM. We could not control the fluid collection and chyluria using standard medical treatments. Therefore, we chose to administer sirolimus, and her symptoms dramatically improved. The mechanism of chyluria presumably involved LAM cell infiltrates in the ureter via the lymphatic vessel flow, which causes LAM to develop because of ureter wall exposure.

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          Sirolimus for angiomyolipoma in tuberous sclerosis complex or lymphangioleiomyomatosis.

          John J. Bissler, Francis McCormack, Lisa Young … (2008)
          Angiomyolipomas in patients with the tuberous sclerosis complex or sporadic lymphangioleiomyomatosis are associated with mutations in tuberous sclerosis genes resulting in constitutive activation of the mammalian target of rapamycin (mTOR). The drug sirolimus suppresses mTOR signaling. We conducted a 24-month, nonrandomized, open-label trial to determine whether sirolimus reduces the angiomyolipoma volume in patients with the tuberous sclerosis complex or sporadic lymphangioleiomyomatosis. Sirolimus was administered for the first 12 months only. Serial magnetic resonance imaging of angiomyolipomas and brain lesions, computed tomography of lung cysts, and pulmonary-function tests were performed. Of the 25 patients enrolled, 20 completed the 12-month evaluation, and 18 completed the 24-month evaluation. The mean (+/-SD) angiomyolipoma volume at 12 months was 53.2+/-26.6% of the baseline value (P<0.001) and at 24 months was 85.9+/-28.5% of the baseline value (P=0.005). At 24 months, five patients had a persistent reduction in the angiomyolipoma volume of 30% or more. During the period of sirolimus therapy, among patients with lymphangioleiomyomatosis, the mean forced expiratory volume in 1 second (FEV1) increased by 118+/-330 ml (P=0.06), the forced vital capacity (FVC) increased by 390+/-570 ml (P<0.001), and the residual volume decreased by 439+/-493 ml (P=0.02), as compared with baseline values. One year after sirolimus was discontinued, the FEV1 was 62+/-411 ml above the baseline value, the FVC was 346+/-712 ml above the baseline value, and the residual volume was 333+/-570 ml below the baseline value; cerebral lesions were unchanged. Five patients had six serious adverse events while receiving sirolimus, including diarrhea, pyelonephritis, stomatitis, and respiratory infections. Angiomyolipomas regressed somewhat during sirolimus therapy but tended to increase in volume after the therapy was stopped. Some patients with lymphangioleiomyomatosis had improvement in spirometric measurements and gas trapping that persisted after treatment. Suppression of mTOR signaling might constitute an ameliorative treatment in patients with the tuberous sclerosis complex or sporadic lymphangioleiomyomatosis. (ClinicalTrials.gov number, NCT00457808.) 2008 Massachusetts Medical Society
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            Lymphangioleiomyomatosis - a wolf in sheep's clothing.

            Elizabeth P. Henske, Francis McCormack (2012)
            Lymphangioleiomyomatosis (LAM) is a rare progressive lung disease of women. LAM is caused by mutations in the tuberous sclerosis genes, resulting in activation of the mTOR complex 1 signaling network. Over the past 11 years, there has been remarkable progress in the understanding of LAM and rapid translation of this knowledge to an effective therapy. LAM pathogenic mechanisms mirror those of many forms of human cancer, including mutation, metabolic reprogramming, inappropriate growth and survival, metastasis via blood and lymphatic circulation, infiltration/invasion, sex steroid sensitivity, and local and remote tissue destruction. However, the smooth muscle cell that metastasizes, infiltrates, and destroys the lung in LAM arises from an unknown source and has an innocent histological appearance, with little evidence of proliferation. Thus, LAM is as an elegant, monogenic model of neoplasia, defying categorization as either benign or malignant.
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              The diagnosis and management of postoperative chylous ascites.

              Ilan Leibovitch, Yoram Mor, Jacob Golomb … (2002)
              Postoperative chylous ascites is a rare complication of retroperitoneal and mediastinal surgery that represents a difficult management problem due to the serious mechanical, nutritional and immunological consequences of the constant loss of protein and lymphocytes. We reviewed the topic of postoperative chylous ascites with special emphasis on the relevant diagnostic and imaging modalities. We propose a novel management algorithm. We performed a MEDLINE search of the literature on chylous ascites using chyloperitoneum as the subject heading and chylous ascites as an additional key word. The search yielded 651 articles. We focused on 102 series, collective reviews and mainly case reports related to the issue of postoperative chylous ascites. We propose a novel algorithm based on a step-up approach aimed at promoting decreased lymph production and flow as well as maintaining nutritional balance. The management algorithm integrates repeat palliative paracentesis, dietary measures, total parenteral nutrition therapy, peritoneovenous shunting and surgical closure of the lymphoperitoneal fistula. Due to the remarkable effectiveness of somatostatin therapy for the closure of lymphatic fistula somatostatin therapy should be attempted with or without total parenteral nutrition early in the course of treatment of chylous ascites before any invasive steps are taken. Various management modalities may be used successfully to treat chylous ascites. Therefore, treatment should be individualized and adjusted to the severity of lymphatic leakage and its consequences. The outcome mostly depends on the underlying pathological condition. Thus, in the absence of malignant or congenital underlying pathology the prognosis in cases of postoperative chylous ascites is good with the majority responding to conservative measures.
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                Author and article information

                Contributors
                Masaki Tominaga
                Journal
                Journal ID (nlm-ta): Respir Med Case Rep
                Journal ID (iso-abbrev): Respir Med Case Rep
                Title: Respiratory Medicine Case Reports
                Publisher: Elsevier
                ISSN (Electronic): 2213-0071
                Publication date PMC-release: 05 January 2018
                Publication date Collection: 2018
                Publication date (Electronic): 05 January 2018
                Volume: 23
                Pages: 86-89
                Affiliations
                [a ]Division of Gastroenterology, Department of Medicine, Kurume University, School of Medicine, Japan
                [b ]Center for Multidisciplinary Treatment of Cancer, Kurume University Hospital, Japan
                [c ]Division of Respirology, Neurology, and Rheumatology, Department of Medicine, Kurume University, School of Medicine, Japan
                [d ]Department of Obstetrics and Gynecology, Kurume University, School of Medicine, Japan
                [e ]Department of Radiology, Department of Medicine, Kurume University, School of Medicine, Japan
                Author notes
                [∗ ]Corresponding author. Division of Respirology, Neurology, and Rheumatology, Department of Medicine, Kurume University, School of Medicine, 67 Asahi-machi, Kurume, Fukuoka 830-0011, Japan.Division of Respirology, Neurology, and RheumatologyDepartment of MedicineKurume UniversitySchool of Medicine67 Asahi-machiKurumeFukuoka830-0011Japan tominaga_masaki@ 123456med.kurume-u.ac.jp
                Article
                Publisher ID: S2213-0071(17)30345-3
                DOI: 10.1016/j.rmcr.2018.01.002
                PMC ID: 5767566
                SO-VID: 23c23c16-09dd-4459-aedd-75298ef99493
                Copyright © © 2018 The Authors
                License:

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                Date received : 27 October 2017
                Date accepted : 4 January 2018
                Categories
                Subject: Case Report

                Keywords: lymphangioleiomyomatosis,chylothorax,chylous ascites,chyluria,sirolimus,ezetimibe
                Data availability:
                Keywords: lymphangioleiomyomatosis, chylothorax, chylous ascites, chyluria, sirolimus, ezetimibe

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