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Lymphangiomyoma

MedGen UID:
44216
•Concept ID:
C0024223
•
Neoplastic Process
Synonyms: Lymphangioleiomyoma; Lymphangioleiomyomas; Lymphangiomyomas
SNOMED CT: Lymphangiomyoma (25239006)
 
Monarch Initiative: MONDO:0006842

Definition

A neoplasm with perivascular epithelioid cell differentiation, often associated with tuberous sclerosis. It is characterized by the presence of smooth muscle and epithelioid cells and by the proliferation of lymphatic vessels. Sites of involvement include the lymph nodes, lung, mediastinum, and retroperitoneum. [from NCI]

Professional guidelines

PubMed

Recommendations for the diagnosis and management of LAM: Looking towards the future.
Gupta N, McCarthy C
Respir Med Res 2023 Jun;83:101016. Epub 2023 Apr 21 doi: 10.1016/j.resmer.2023.101016. PMID: 37087907
French recommendations for the diagnosis and management of lymphangioleiomyomatosis.
Cottin V, Blanchard E, Kerjouan M, Lazor R, Reynaud-Gaubert M, Taille C, Uzunhan Y, Wemeau L, Andrejak C, Baud D, Bonniaud P, Brillet PY, Calender A, Chalabreysse L, Court-Fortune I, Desbaillets NP, Ferretti G, Guillemot A, Hardelin L, Kambouchner M, Leclerc V, Lederlin M, Malinge MC, Mancel A, Marchand-Adam S, Maury JM, Naccache JM, Nasser M, Nunes H, Pagnoux G, Prévot G, Rousset-Jablonski C, Rouviere O, Si-Mohamed S, Touraine R, Traclet J, Turquier S, Vagnarelli S, Ahmad K; OrphaLung network
Respir Med Res 2023 Jun;83:101010. Epub 2023 Mar 24 doi: 10.1016/j.resmer.2023.101010. PMID: 37087906
Lymphangioleiomyomatosis: pathogenesis, clinical features, diagnosis, and management.
McCarthy C, Gupta N, Johnson SR, Yu JJ, McCormack FX
Lancet Respir Med 2021 Nov;9(11):1313-1327. Epub 2021 Aug 27 doi: 10.1016/S2213-2600(21)00228-9. PMID: 34461049

Recent clinical studies

Therapy

Resection of Extrapulmonary Lymphangiomyoma and Post-Operative Management Considerations.
Abramov R, Rufizen L, Gilshtein H
Vasc Endovascular Surg 2023 Oct;57(7):756-759. Epub 2023 Mar 23 doi: 10.1177/15385744221144503. PMID: 36952511
Rapid Shrinkage of Retroperitoneal Lymphangioleiomyoma after Sirolimus Initiation.
Hirose M, Ishimaru Y, Sakata S, Sakagami T
Intern Med 2022 Jul 1;61(13):2081-2082. Epub 2021 Nov 27 doi: 10.2169/internalmedicine.8685-21. PMID: 34840234Free PMC Article
mTOR inhibitor therapy as a disease modifying therapy for tuberous sclerosis complex.
Franz DN, Krueger DA
Am J Med Genet C Semin Med Genet 2018 Sep;178(3):365-373. Epub 2018 Oct 11 doi: 10.1002/ajmg.c.31655. PMID: 30307123
Changes in lung function and chylous effusions in patients with lymphangioleiomyomatosis treated with sirolimus.
Taveira-DaSilva AM, Hathaway O, Stylianou M, Moss J
Ann Intern Med 2011 Jun 21;154(12):797-805, W-292-3. doi: 10.7326/0003-4819-154-12-201106210-00007. PMID: 21690594Free PMC Article
Pulmonary vascular disease--overview.
Cordasco EM
Angiology 1983 Oct;34(10):646-53. doi: 10.1177/000331978303401003. PMID: 6625221

Prognosis

Effect of fasting on the size of lymphangioleiomyomas in patients with lymphangioleiomyomatosis.
Taveira-DaSilva AM, Jones AM, Julien-Williams P, Shawker T, Glasgow CG, Stylianou M, Moss J
Chest 2015 Oct;148(4):1027-1033. doi: 10.1378/chest.15-0456. PMID: 26066372Free PMC Article
Prognostic significance of pulmonary lymphangioleiomyomatosis histologic score.
Matsui K, Beasley MB, Nelson WK, Barnes PM, Bechtle J, Falk R, Ferrans VJ, Moss J, Travis WD
Am J Surg Pathol 2001 Apr;25(4):479-84. doi: 10.1097/00000478-200104000-00007. PMID: 11257622
Angiomyolipoma of kidney.
Eble JN
Semin Diagn Pathol 1998 Feb;15(1):21-40. PMID: 9503504
Pulmonary lymphangioleiomyomatosis.
Viskum K
Monaldi Arch Chest Dis 1993;48(3):233-6. PMID: 8369788
Restrictive lung disease in pregnancy.
King TE Jr
Clin Chest Med 1992 Dec;13(4):607-22. PMID: 1478021

Clinical prediction guides

Effect of fasting on the size of lymphangioleiomyomas in patients with lymphangioleiomyomatosis.
Taveira-DaSilva AM, Jones AM, Julien-Williams P, Shawker T, Glasgow CG, Stylianou M, Moss J
Chest 2015 Oct;148(4):1027-1033. doi: 10.1378/chest.15-0456. PMID: 26066372Free PMC Article
Changes in lung function and chylous effusions in patients with lymphangioleiomyomatosis treated with sirolimus.
Taveira-DaSilva AM, Hathaway O, Stylianou M, Moss J
Ann Intern Med 2011 Jun 21;154(12):797-805, W-292-3. doi: 10.7326/0003-4819-154-12-201106210-00007. PMID: 21690594Free PMC Article
Prognostic significance of pulmonary lymphangioleiomyomatosis histologic score.
Matsui K, Beasley MB, Nelson WK, Barnes PM, Bechtle J, Falk R, Ferrans VJ, Moss J, Travis WD
Am J Surg Pathol 2001 Apr;25(4):479-84. doi: 10.1097/00000478-200104000-00007. PMID: 11257622
Lymphangioleiomyomatosis: abdominopelvic CT and US findings.
Avila NA, Kelly JA, Chu SC, Dwyer AJ, Moss J
Radiology 2000 Jul;216(1):147-53. doi: 10.1148/radiology.216.1.r00jl42147. PMID: 10887241
Pulmonary lymphangioleiomyomatosis.
Viskum K
Monaldi Arch Chest Dis 1993;48(3):233-6. PMID: 8369788

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