2026-07-02 | Robot-Assisted Lobectomy for Lung Cancer Complicated by Lymphangioleiomyomatosis.
Lymphangioleiomyomatosis (LAM) is a rare systemic disease characterized by progressive cystic destruction of the lung parenchyma, resulting in extreme parenchymal fragility. Surgical treatment of lung cancer in patients with LAM is technically challenging due to the high risk of intractable air leaks. We report a case of robot-assisted thoracoscopic surgery (RATS) for lung cancer in a patient with LAM, highlighting a strategic hybrid approach to managing the exceptionally fragile lung tissue. A 56-year-old woman with tuberous sclerosis complex-associated LAM was diagnosed with Stage IA1 adenocarcinoma in the left upper lobe. We performed a RATS left upper lobectomy using the da Vinci Xi system (Intuitive Surgical, Sunnyvale, CA, USA). To protect the exceptionally fragile lung, a "no-touch" retraction technique was employed using rolled gauze and blunt-tipped robotic instruments. A fissureless technique was applied to minimize parenchymal injury. Crucially, for the division of the incomplete interlobar fissure and the bronchus, we utilized manual staplers equipped with bioabsorbable reinforcement material (buttressed staplers) deployed by a bedside assistant, which provided superior sealing for the fragile lung compared to the robotic staplers available at that time. Despite these precautions, a pinhole air leak occurred in the S8 segment, likely due to unintentional contact with a robotic joint, illustrating the extreme sensitivity of the LAM lung. This was identified via a meticulous sealing test and repaired with polyglycolic acid sheets and fibrin glue. The patient was discharged on POD 5 without persistent air leaks. At 1 year post-surgery, her respiratory function was well-preserved, exceeding predicted values. RATS offers superior visualization for lung cancer surgery in patients with LAM. However, given the extreme fragility of the lung, the selective use of manual buttressed staplers is a vital adjunct to prevent postoperative air leaks. Optimal outcomes depend on a constant awareness of lung fragility, a hybrid technical strategy, and a rigorous intraoperative sealing test to identify and repair even minor pleural injuries.
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2026-06-30 | Exploring glucocorticoid receptor signalling in lymphangioleiomyomatosis.
Lymphangioleiomyomatosis (LAM) is a rare, low-grade neoplasm that causes progressive cystic lung destruction and is often associated with renal angiomyolipomas (AMLs). Given evidence of pleiotropy linking LAM risk to pulmonary traits, we investigated whether glucocorticoid receptor (GR) signalling might influence LAM biology and clinical features. We combined cell-based studies, GR inhibition/activation assays, gene expression and single-cell RNA sequencing analyses, and hormone profiling in retrospective and prospective LAM cohorts. Cellular experiments employed murine Tsc2 -/- embryonic fibroblasts and human TSC2 -/- AML cells. Circulating steroid levels were measured in women with LAM and healthy controls, and associations with clinical variables were evaluated. In LAM/AML models, GR activation by glucocorticoids elicited transcriptional responses, whereas GR inhibition reduced clonogenic potential. GR stimulation was associated with CDKN1C upregulation through enhancer binding, and single-cell profiling suggested a shift towards slower proliferation and differentiation-prone states enriched for a LAM cell signature. Clinically, our analyses suggest that women with LAM may show altered circulating hormone profiles, including elevated adrenocorticotropic hormone (ACTH) and cortisol levels, together with reduced 17-hydroxyprogesterone, compared with controls. In a prospective cohort, ACTH levels were suggestively associated with advanced radiological disease stage. AML cells showed elevated expression of POMC, which encodes the precursor of ACTH, and POMC peptide was detected in LAM lung tissue. Our findings suggest that GR signalling may contribute to aspects of LAM cell behaviour and disease status. Further investigation of this pathway could clarify its role as a disease modifier and potential therapeutic target.
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