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Lymphangioma & lymphangiomatosis

Last author update: 1 November 2012

Last staff update: 27 January 2023 (update in progress)

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PubMed Search: Lymphangioma soft tissue

See Also: Cystic, Lymphangiomatosis, Lymphangiomatous papules / plaques postradiation

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Table of Contents

Cite this page: Shankar V. Lymphangioma & lymphangiomatosis. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissuelymphangioma.html. Accessed February 4th, 2023.

Definition / general

May represent malformations due to failure of lymphatics to communicate with venous system
Most described cases are cystic / cavernous
Skin tumors are called lymphangioma circumscriptum
Diffuse / multicentric lymphangiomas are often in thoracic cavity; also bone, extremities, soft tissue, viscera

Clinical features

Benign
See also discussion in these chapters: Lymph nodes, Mediastinum, Skin - nonmelanocytic tumors, Spleen
Lymphangiomatosis:
- Rare, idiopathic, occurs mostly in children
- Cases with visceral involvement are often fatal but cases involving limbs have good prognosis (Am J Surg Pathol 1993;17:329, Am J Surg Pathol 1995;19:125)
- Associated with:
  - Kaposiform hemangioendothelioma (Am J Surg Pathol 1993;17:321)
  - Bony lytic lesions and chylothorax (J Pediatr Hematol Oncol 2008;30:66)
  - Total cavopulmonary connection for complex congenital heart disease (Pediatr Cardiol 2008;29:771)
- Diffuse retroperitoneal cystic abdominal lymphangiomatosis is attributed to congenital malformation of lymphatic ducts; distribution often corresponds to primitive fetal lymphatic sacs; either capillary, cavernous or cystic; may have multisystem involvement involving bone, brain, extremities, liver, pancreas, soft tissue, spleen (Acta Cytol 2009;53:191)
- Gorham disease: diffuse lymphangiomatosis also involving bony tissue

Case reports

13 year old girl with isolated laryngeal lymphangioma (Indian J Otolaryngol Head Neck Surg 2011;63:90)
15 year old girl with perinephric lymphangiomatosis (Indian J Radiol Imaging 2010;20:224)
25 year old woman, with acute presentation of colonic lymphangiomatosis (Int J Surg Case Rep 2012;3:158)
25 year old woman with colonic lymphangiomatosis mimicking acute appendicitis (Indian J Cancer 2011;48:139)
33 year old woman with fatal case (Am J Med Sci 2008;336:445)
35 year old pregnant woman with large cystic tumor in fetal chest (Ultrasound Obstet Gynecol 2008;31:697)
39 year old healthy man with recurrent, complicated scrotal lymphangiomatosis (Can Urol Assoc J 2012;6:E11)
58 year old man with conjunctival lymphangioma (Case Rep Ophthalmol Med 2012;2012:836573)
71 year old man with mesenteric lymphangiomatosis (World J Gastroenterol 2009;15:3947)
Secondary lymphangiomas of vulva (Indian J Dermatol 2012;57:149)

Treatment

Excision if possible; also sclerotherapy or observation (Lymphology 2006;39:147)
Recurrence is minimized by complete excision (Mayo Clin Proc 2006;81:1197)
Intralesional bleomycin therapy may be an effective alternative to surgery (J Maxillofac Oral Surg 2011;10:152)

Clinical images

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Conjunctiva

Larynx

Microscopic (histologic) description

Large lymphatic channels in loose connective tissue stroma
Focally disorganized smooth muscle in wall of larger channels
Peripheral lymphoid aggregates
Often increased mast cells (Mod Pathol 1999;12:569)
Lymphangiomatosis: increase in size and number of thin walled channels; lymphatic spaces are interconnecting and dilated, lined by a single attenuated layer of endothelial cells involving the dermis, subcutis and occasionally underlying fascia and skeletal muscle

Microscopic (histologic) images

Contributed by Mark R. Wick, M.D.