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Lymphangiomatosis

MedGen UID:
83110
•Concept ID:
C0334102
•
Disease or Syndrome
SNOMED CT: Lymphangiomatosis (86970009); Lymphangiomatosis (403546006)

Definition

A lymphangioma affecting several anatomic sites. [from NCI]

Professional guidelines

PubMed

Differential Diagnosis of Generalized Cystic Lymphangiomatosis: A Literature Review.
Keven A, Gürbüz AF
Curr Med Imaging 2024;20:1-9. doi: 10.2174/0115734056264320231109071607. PMID: 38389351
Kaposiform lymphangiomatosis: Diagnosis, pathogenesis, and treatment.
McDaniel CG, Adams DM, Steele KE, Hammill AM, Merrow AC, Crane JL, Smith CL, Kozakewich HPW, Le Cras TD
Pediatr Blood Cancer 2023 Apr;70(4):e30219. Epub 2023 Jan 22 doi: 10.1002/pbc.30219. PMID: 36683202Free PMC Article
Treatment of thoracic lymphangiomatosis.
Rostom AY
Arch Dis Child 2000 Aug;83(2):138-9. doi: 10.1136/adc.83.2.138. PMID: 10906021Free PMC Article

Recent clinical studies

Etiology

Vascular Anomalies: Diagnosis of Complicated Anomalies and New Medical Treatment Options.
Adams DM, Ricci KW
Hematol Oncol Clin North Am 2019 Jun;33(3):455-470. doi: 10.1016/j.hoc.2019.01.011. PMID: 31030813
Nonmalignant Adult Thoracic Lymphatic Disorders.
Itkin M, McCormack FX
Clin Chest Med 2016 Sep;37(3):409-20. doi: 10.1016/j.ccm.2016.04.004. PMID: 27514588
Thoracic involvement in generalised lymphatic anomaly (or lymphangiomatosis).
Luisi F, Torre O, Harari S
Eur Respir Rev 2016 Jun;25(140):170-7. doi: 10.1183/16000617.0018-2016. PMID: 27246594Free PMC Article
The lung in multi-system disease.
Dinwiddie R
Paediatr Respir Rev 2000 Mar;1(1):58-63. doi: 10.1053/prrv.2000.0003. PMID: 16263447
Lymphangiomatosis with splenic involvement.
Avigad S, Jaffe R, Frand M, Izhak Y, Rotem Y
JAMA 1976 Nov 15;236(20):2315-7. PMID: 989835

Diagnosis

Renal Lymphangiomatosis.
Chawla S
Indian J Pediatr 2023 Apr;90(4):406-407. Epub 2023 Feb 15 doi: 10.1007/s12098-023-04486-z. PMID: 36790575
Multifocal vascular lesions.
Levin LE, Lauren CT
Semin Cutan Med Surg 2016 Sep;35(3):153-60. doi: 10.12788/j.sder.2016.054. PMID: 27607324
Splenic lymphangioma.
Ioannidis I, Kahn AG
Arch Pathol Lab Med 2015 Feb;139(2):278-82. doi: 10.5858/arpa.2013-0656-RS. PMID: 25611113
Lymphangiomatosis: clinical overview.
Blei F
Lymphat Res Biol 2011;9(4):185-90. doi: 10.1089/lrb.2011.0020. PMID: 22196283
Lymphangiomatosis and Gorham's Disease.
Rockson SG
Lymphat Res Biol 2011;9(4):181. doi: 10.1089/lrb.2011.9410. PMID: 22196281

Therapy

Sirolimus in the treatment of kaposiform lymphangiomatosis.
Zhou J, Yang K, Chen S, Ji Y
Orphanet J Rare Dis 2021 Jun 8;16(1):260. doi: 10.1186/s13023-021-01893-3. PMID: 34103076Free PMC Article
Vascular Anomalies: Diagnosis of Complicated Anomalies and New Medical Treatment Options.
Adams DM, Ricci KW
Hematol Oncol Clin North Am 2019 Jun;33(3):455-470. doi: 10.1016/j.hoc.2019.01.011. PMID: 31030813
Vascular anomaly cases for the pediatric hematologist oncologists-An interdisciplinary review.
Adams DM, Brandão LR, Peterman CM, Gupta A, Patel M, Fishman S, Trenor CC
Pediatr Blood Cancer 2018 Jan;65(1) Epub 2017 Jul 20 doi: 10.1002/pbc.26716. PMID: 28727248
A novel case of lymphangiomatosis in HIV.
Spier AB, Kurtz J, Sears D
Exp Mol Pathol 2017 Apr;102(2):251-254. Epub 2017 Feb 9 doi: 10.1016/j.yexmp.2017.02.003. PMID: 28189546
Interventional Treatment of Pulmonary Lymphatic Anomalies.
Itkin M
Tech Vasc Interv Radiol 2016 Dec;19(4):299-304. Epub 2016 Oct 8 doi: 10.1053/j.tvir.2016.10.005. PMID: 27993326

Prognosis

Updates to the Management of Gorham-Stout Disease and Osseous Vascular Lesions in the Head and Neck.
Burke AB, Dong C, Chandra SR
Oral Maxillofac Surg Clin North Am 2024 Feb;36(1):93-102. Epub 2023 Oct 29 doi: 10.1016/j.coms.2023.09.004. PMID: 37903662
Sirolimus in the treatment of kaposiform lymphangiomatosis.
Zhou J, Yang K, Chen S, Ji Y
Orphanet J Rare Dis 2021 Jun 8;16(1):260. doi: 10.1186/s13023-021-01893-3. PMID: 34103076Free PMC Article
Newcomers in Vascular Anomalies.
Al-Ibraheemi A
Surg Pathol Clin 2020 Dec;13(4):719-728. doi: 10.1016/j.path.2020.08.008. PMID: 33183729
Interventional Treatment of Pulmonary Lymphatic Anomalies.
Itkin M
Tech Vasc Interv Radiol 2016 Dec;19(4):299-304. Epub 2016 Oct 8 doi: 10.1053/j.tvir.2016.10.005. PMID: 27993326
Splenic lymphangioma.
Ioannidis I, Kahn AG
Arch Pathol Lab Med 2015 Feb;139(2):278-82. doi: 10.5858/arpa.2013-0656-RS. PMID: 25611113

Clinical prediction guides

Thoracic involvement of diffuse lymphangiomatosis successfully treated with sildenafil.
Maurac A, Debray MP, Crestani B, Taillé C
BMJ Case Rep 2019 Apr 23;12(4) doi: 10.1136/bcr-2018-228523. PMID: 31015245Free PMC Article
Thoracic involvement in generalised lymphatic anomaly (or lymphangiomatosis).
Luisi F, Torre O, Harari S
Eur Respir Rev 2016 Jun;25(140):170-7. doi: 10.1183/16000617.0018-2016. PMID: 27246594Free PMC Article
Complex lymphatic anomalies.
Trenor CC 3rd, Chaudry G
Semin Pediatr Surg 2014 Aug;23(4):186-90. Epub 2014 Jul 22 doi: 10.1053/j.sempedsurg.2014.07.006. PMID: 25241096
Improvement of disseminated lymphangiomatosis with recombinant interferon therapy.
Laverdière C, David M, Dubois J, Russo P, Hershon L, Lapierre JG
Pediatr Pulmonol 2000 Apr;29(4):321-4. doi: 10.1002/(sici)1099-0496(200004)29:4<321::aid-ppul13>3.0.co;2-c. PMID: 10738021
Splenic lymphangiomatosis: a rare cause of splenomegaly.
Marymont JV, Knight PJ
J Pediatr Surg 1987 May;22(5):461-2. doi: 10.1016/s0022-3468(87)80271-3. PMID: 3585671

Recent systematic reviews

Sirolimus treatment for paediatric head and neck lymphatic malformations: a systematic review.
Saibene AM, Rosso C, Felisati G, Pignataro L, Schindler A, Ghilardi G, Colletti G, Gaffuri M, Mozzanica F
Eur Arch Otorhinolaryngol 2023 Aug;280(8):3529-3540. Epub 2023 Apr 28 doi: 10.1007/s00405-023-07991-1. PMID: 37115326Free PMC Article
A Systematic Review of Lymphangioleiomyomatosis on Diagnosis and Molecular Mechanism.
Dong X, Jin L, Wang A, Wu L, Fan X, Hou Q, Li T, Zhao R, Zhang Y
Biomed Res Int 2021;2021:6612776. Epub 2021 Feb 10 doi: 10.1155/2021/6612776. PMID: 33628792Free PMC Article

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