Lymphangioma overview
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Haytham Allaham, M.D. [2] Badria Munir M.B.B.S.[3]
Overview
Lymphangioma arises from lymph vessels, which are normally involved in the re-circulation of excess body fluid back into the blood stream. The exact pathogenesis of lymphangioma is not fully understood. It is thought that lymphangioma is caused by either sequestration of lymph tissue, abnormal budding of lymph vessels, lack of fusion with the venous system, or obstruction of lymph vessels. Lymphangiomas most commonly develop at the head and neck regions. Lymphangioma is associated with a number of conditions that include Turner syndrome and Down syndrome. On gross pathology, characteristic findings of lymphangioma include a grey-white, well circumscribed, edematous mass with a variable size and consistency. On microscopic histopathological analysis, characteristic findings of lymphangioma include thin walled endothelial lining, intraluminal accumulation of eosinophilic deposits, and clusters of intraluminal lymphocytes. There are no known direct causes for lymphangioma. Lymphangioma must be differentiated from arteriovenous malformation, branchial cleft cyst, and dermoid cyst. Lymphangiomas are rare, accounting for 4% of all vascular tumors among children. The incidence of lymphangioma is estimated to be less than 2 cases per 100,000 individuals in the United States. Lymphangioma commonly affects individuals younger than 5 years of age. Lymphangioma affects men and women equally. There is no racial predilection to the lymphangioma. CT scan may be diagnostic of lymphangioma. Findings on CT scan suggestive of lymphangioma include a homogeneous, cystic, mass that may demonstrate intrinsic septations and compression of adjacent organs. The mainstay of therapy for lymphangioma is surgery. Treatment for lymphangioma involves the removal of the abnormal tissue; however complete removal may be impossible without removing other normal areas. Most patients need at least two procedures done for the removal process to be achieved.[1][2]
Pathophysiology
- Lymphangioma arises from lymph vessels, which are normally involved in the re-circulation of excess body fluid back into the blood stream.[3]
- The exact mechanism of development of abnormal malformations is not known.
- It is suggested that lymphangioma is formed as a result of one of four mechanisms:[4]
- Whimster describes pathogenesis of lymphangioma as the collection of lymphatic cisterns in the deep subcutaneous plane which get detached from normal network, however they keep connected to superficial lymph vessels through short and dilated vessels.[5]
- The cisterns originally derive from primitive lymph sac, which fail to connect to normal circulation.
- These are covered with thick muscular coat, which on contraction increase intramural pressure.
- Ultimately leads to dilated sacs which are visible on skin as out-pouchings.
- Whimster's description is proved with radio-graphic evidence as well.[6]
- Which showed large multilobulated cisterns extend deep in the dermis and laterally beyond the clinical lesions.
- These deep lymphangiomas had no connections with the adjacent normal lymphatics.
- Some lymphangiomas may represent vascular malformations during embryonic development rather than as true neoplasms.
- For more information on Vascular anamolies. Click here.
- Vascular endothelial growth factor (VEGF)–C and VEGF receptor-3 play a key role in development of lymphangiomas.[7]
Causes
- There are no known direct causes for lymphangioma.
- Cystic hygroma usually presents in syndromes such as Turner syndrome and Down syndrome. Therefore it is thought that the lymphatic malformations can arise from gene mutations, occuring as missing or deleted part of chromosomes.[10]
- Acquired lymphangioma circumscriptum occur as a sequele of chronic lymphatic obstruction such as after surgery, infections or radiotherapy.
Differentiating Lymphangioma from other Diseases
Lymphangioma must be differentiated from arteriovenous malformation, branchial cleft cyst, and dermoid cyst.
Epidemiology and Demographics
- Lymphangiomas are rare, accounting for 4% of all vascular tumors among children.[5]
- The incidence of lymphangioma is estimated to be less than 2 cases per 100,000 individuals in the United States.
- Lymphangioma commonly affects individuals younger than 5 years of age.
- Lymphangioma affects men and women equally.
- There is no racial predilection to the lymphangioma.
Risk Factors
The most potent risk factor in the development of lymphangioma is the presence of genetic disorders such as Turner syndrome, Down syndrome, and Noonan syndrome.[10]
Natural History, Complications and Prognosis
- Most of the patients with lymphangioma will develop a cervico-facial mass that often grows in proportion to the patient’s body growth rate.
- The mass usually recurs after surgical interventions.
- Common complications of lymphangioma include esophageal obstruction, upper respiratory tract obstruction, infections, and compression of adjacent tissues such as nerves and blood vessels. Prognosis of lymphangioma is generally excellent.
Diagnosis
History and Symptoms
The majority of patients with lymphangioma are asymptomatic.
Physical Exam
Lymphangioma patients often appear healthy. On physical exam a painless , compressible, soft neck mass that often transilluminates is a diagnostic finding on physical exam.
Laboratory Findings
There are no diagnostic lab findings associated with lymphangioma.
CT Scan
CT scan may be diagnostic of lymphangioma. Findings on CT scan suggestive of lymphangioma include a homogeneous, cystic, mass that may demonstrate intrinsic septations and compression of adjacent organs.
MRI
MRI may be diagnostic of lymphangioma. Findings on MRI suggestive of lymphangioma include hyperintense signal enhancement due to cyst formation.
Other Diagnostic Studies
The definitive diagnosis of lymphangioma is confirmed by a biopsy. Characteristic findings for lymphangioma on microscopic histopathological analysis can be found here.
Treatment
Medical Therapy
There is no medical treatment for lymphangioma; the mainstay of therapy is surgery.
Surgery
The mainstay of therapy for lymphangioma is surgery. Treatment for lymphangioma involves the removal of the abnormal tissue; however complete removal may be impossible without removing other normal areas. Most patients need at least two procedures done for the removal process to be achieved.
References
- ↑ Lymphangioma. Wikipedia (2016) https://en.wikipedia.org/wiki/Lymphangioma Accessed on March 3, 2015
- ↑ Lymphangioma. PathologyOutlines (2016) http://www.pathologyoutlines.com/topic/softtissuelymphangiomacystic.html Accessed on March 5, 2016
- ↑ Wiegand S, Eivazi B, Barth PJ, von Rautenfeld DB, Folz BJ, Mandic R, Werner JA (July 2008). "Pathogenesis of lymphangiomas". Virchows Arch. 453 (1): 1–8. doi:10.1007/s00428-008-0611-z. PMID 18500536.
- ↑ Chang MB, Newman CC, Davis MD, Lehman JS (September 2016). "Acquired lymphangiectasia (lymphangioma circumscriptum) of the vulva: Clinicopathologic study of 11 patients from a single institution and 67 from the literature". Int. J. Dermatol. 55 (9): e482–7. doi:10.1111/ijd.13264. PMID 26967121.
- ↑ 5.0 5.1 Ersoy AO, Oztas E, Saridogan E, Ozler S, Danisman N (March 2016). "An Unusual Origin of Fetal Lymphangioma Filling Right Axilla". J Clin Diagn Res. 10 (3): QD09–11. doi:10.7860/JCDR/2016/18516.7513. PMC 4843338. PMID 27134953.
- ↑ Levy AD, Cantisani V, Miettinen M (June 2004). "Abdominal lymphangiomas: imaging features with pathologic correlation". AJR Am J Roentgenol. 182 (6): 1485–91. doi:10.2214/ajr.182.6.1821485. PMID 15149994.
- ↑ Ferrara N, Kerbel RS (December 2005). "Angiogenesis as a therapeutic target". Nature. 438 (7070): 967–74. doi:10.1038/nature04483. PMID 16355214.
- ↑ 8.0 8.1 Oliveti A, Biasi TB, Funchal G (2017). "Lymphangioma secondary to irradiation after mastectomy". An Bras Dermatol. 92 (3): 395–397. doi:10.1590/abd1806-4841.20173952. PMC 5514585. PMID 29186257. Vancouver style error: initials (help)
- ↑ Hwang J, Lee YK, Burm JS (March 2017). "Treatment of Tongue Lymphangioma with Intralesional Combination Injection of Steroid, Bleomycin and Bevacizumab". Arch Craniofac Surg. 18 (1): 54–58. doi:10.7181/acfs.2017.18.1.54. PMC 5556746. PMID 28913305.
- ↑ 10.0 10.1 Pootrakul L, Nazareth MR, Cheney RT, Grassi MA (June 2014). "Lymphangioma circumscriptum of the vulva in a patient with Noonan syndrome". Cutis. 93 (6): 297–300. PMID 24999641.