{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,11]],"date-time":"2026-02-11T15:38:29Z","timestamp":1770824309215,"version":"3.50.1"},"reference-count":24,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"11","content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2023,11]]},"abstract":"<jats:p>Lymphangioleiomyomatosis (LAM) is a tuberous sclerosis complex (TSC)-associated tumor, characterized by the expression of neural crest lineages including neuronal markers. Neural crest cells can differentiate into multiple cell types that contribute to tissues associated with TSC-related tumors, and TSC-related tumors could be specifically associated with distinct neural crest subtypes. This study aimed to clarify the clinicopathological effects of expression of neuronal markers in LAM. Lung tissues from 40 patients with LAM (of whom 13, 1, and 26 had undergone lung transplantation, lobectomy, and partial lung resection, respectively) were immunohistochemically analyzed. All patients were women, and their median age was 36 years (range: 24\u201362\u00a0y). All patients who underwent lung transplantation or lobectomy were classified as LAM histologic score (LHS)-3, whereas those who underwent partial lung resection were classified as LHS-1. LAM cells expressed peripherin (65%), and neuron-specific \u03b2III-tubulin (43%). A comparison of the early (LHS-1) and advanced (LHS-3) stages of LAM revealed that neuron-specific \u03b2III-tubulin was significantly expressed in the early stage of LAM (<jats:italic toggle=\"yes\">P<\/jats:italic> = 0.0009). Neuron-specific \u03b2III-tubulin-positive LAM was associated with younger age (<jats:italic toggle=\"yes\">P<\/jats:italic> &lt; 0.0001), the coexistence of renal angiomyolipoma (<jats:italic toggle=\"yes\">P<\/jats:italic> = 0.027), and the absence of retroperitoneal LAM (<jats:italic toggle=\"yes\">P<\/jats:italic> = 0.045). Furthermore, based on the expression levels of immunohistochemical markers in LAM, 2 distinct clusters with different expression levels of neuronal markers were observed. Approximately 40% to 60% of patients with LAM expressed neuron-specific \u03b2III-tubulin and peripherin. Neuronal expression may be associated with disease severity.<\/jats:p>","DOI":"10.1097\/pas.0000000000002113","type":"journal-article","created":{"date-parts":[[2023,8,21]],"date-time":"2023-08-21T08:12:48Z","timestamp":1692605568000},"page":"1252-1260","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":0,"title":["Clinicopathological Impacts of Expression of Neuronal Markers in Lymphangioleiomyomatosis"],"prefix":"10.1097","volume":"47","author":[{"given":"Monami","family":"Kishi","sequence":"first","affiliation":[{"name":"Department of Human Pathology, Juntendo University Graduate School of Medicine, Bunkyo-ku, Tokyo, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takuo","family":"Hayashi","sequence":"additional","affiliation":[{"name":"Department of Human Pathology, Juntendo University Graduate School of Medicine, Bunkyo-ku, Tokyo, Japan"},{"name":"The Study Group for Pneumothorax and Cystic Lung 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of Thoracic Surgery, Nissan Tamagawa Hospital, Tokyo, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masaki","family":"Hosoya","sequence":"additional","affiliation":[{"name":"Department of Medical Oncology, Juntendo University Graduate School of Medicine, Bunkyo-ku, Tokyo, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yasuhito","family":"Sekimoto","sequence":"additional","affiliation":[{"name":"The Study Group for Pneumothorax and Cystic Lung Diseases, Setagaya-Ku, Tokyo, Japan"},{"name":"Department of Respiratory Medicine, Juntendo University Graduate School of Medicine, Bunkyo-ku, Tokyo, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Makiko K.","family":"Okura","sequence":"additional","affiliation":[{"name":"The Study Group for Pneumothorax and Cystic Lung Diseases, Setagaya-Ku, Tokyo, Japan"},{"name":"Department of Respiratory Medicine, Juntendo University Graduate School of Medicine, Bunkyo-ku, Tokyo, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoichiro","family":"Mitsuishi","sequence":"additional","affiliation":[{"name":"The Study Group for Pneumothorax and Cystic Lung Diseases, Setagaya-Ku, Tokyo, Japan"},{"name":"Department of Respiratory Medicine, Juntendo University Graduate School of Medicine, Bunkyo-ku, Tokyo, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoshinori","family":"Okada","sequence":"additional","affiliation":[{"name":"Department of Thoracic Surgery, Institute of Development, Aging and Cancer, Tohoku University, Sendai, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jun","family":"Kanno","sequence":"additional","affiliation":[{"name":"Department of Pathology, Nissan Tamagawa Hospital, Tokyo, Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takashi","family":"Yao","sequence":"additional","affiliation":[{"name":"Department of Human Pathology, Juntendo University Graduate 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