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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

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 advances in the pathobiology and clinical management of lymphangioleiomyomatosis.
Harari S, Spagnolo P, Cocconcelli E, Luisi F, Cottin V
Curr Opin Pulm Med 2018 Sep;24(5):469-476. doi: 10.1097/MCP.0000000000000502. PMID: 29927757

Recent clinical studies

Etiology

Primary retroperitoneal PEComa: an incidental finding.
Marinho BM, Canha AG, Silva DS, Silva JDP
BMJ Case Rep 2022 Nov 11;15(11) doi: 10.1136/bcr-2022-250466. PMID: 36368730Free PMC Article
A histopathological diagnosis of mesenteric cystic lymphangioma, clinically misdiagnosed as simple mesenteric cyst - case report.
Hanganu E, Gavrilescu SL, Trandafirescu MF, Chiforeanu AM, Mihăilă D, Florea ID, Anton-Păduraru DT, Burlea M
Rom J Morphol Embryol 2017;58(4):1525-1530. PMID: 29556652
Pericyte antigens in angiomyolipoma and PEComa family tumors.
Shen J, Shrestha S, Yen YH, Scott MA, Asatrian G, Barnhill R, Lugassy C, Soo C, Ting K, Peault B, Dry SM, James AW
Med Oncol 2015 Aug;32(8):210. Epub 2015 Jun 30 doi: 10.1007/s12032-015-0659-y. PMID: 26123600
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
Restrictive lung disease in pregnancy.
King TE Jr
Clin Chest Med 1992 Dec;13(4):607-22. PMID: 1478021

Diagnosis

Pleural Disease in Women.
Argento AC, Gillespie CT
Semin Respir Crit Care Med 2019 Jun;40(3):402-409. Epub 2019 Sep 16 doi: 10.1055/s-0039-1695050. PMID: 31525815
Summary for Clinicians: Lymphangioleiomyomatosis Diagnosis and Management Clinical Practice Guideline.
Feemster LC, Lyons PG, Chatterjee RS, Kidambi P, McCormack FX, Moss J, Wilson KC, Thomson CC
Ann Am Thorac Soc 2017 Jul;14(7):1073-1075. doi: 10.1513/AnnalsATS.201609-685CME. PMID: 28665705Free PMC Article
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
Lymphangioleiomyomatosis.
Johnson SF, Davey DD, Cibull ML, Schwartz RW, Strodel WE
Am Surg 1993 Jul;59(7):395-9. PMID: 8323071

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 lymphangiomyomatosis.
Eisera NB, Venegoni E, Colombi R
J R Soc Med 1988 Oct;81(10):607-9. doi: 10.1177/014107688808101021. PMID: 3184096Free PMC Article

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
Angiomyolipoma of kidney.
Eble JN
Semin Diagn Pathol 1998 Feb;15(1):21-40. PMID: 9503504
Native pulmonary muscular proliferation.
Wöckel W, Meister P, Karg O, Morresi-Hauf A
Pathol Res Pract 1997;193(9):599-605. doi: 10.1016/S0344-0338(97)80036-2. PMID: 9521017
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
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
Pulmonary lymphangiomyomatosis.
El Allaf D, Borlee G, Hadjoudj H, Henrard L, Marcelle R, van Cauwenberge H
Eur J Respir Dis 1984 Feb;65(2):147-52. PMID: 6698139

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