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Soft tissue tumors

Lymphangioma - general


Reviewer: Vijay Shankar, M.D. (see Reviewers page)
Revised: 17 November 2014, last major update November 2012
Copyright: (c) 2003-2014, PathologyOutlines.com, Inc.

See also subtypes: cystic, lymphangiomatosis, lymphangiomatous papules/plaques post-radiation

Definition
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● 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

Clinical features
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● Benign
● See also discussion in these chapters: bone, breast, kidney tumor, lymph nodes, mediastinum, skin-nonmelanocytic tumors, skin-nontumor, spleen

Case reports
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● 13 year old girl with isolated laryngeal lymphangioma (Indian J Otolaryngol Head Neck Surg 2011;63:90)
● 35 year old pregnant woman with large cystic tumor in fetal chest (Ultrasound Obstet Gynecol 2008;31:697)
● 58 year old man with conjunctival lymphangioma (Case Rep Ophthalmol Med 2012;2012:836573)
● Secondary lymphangiomas of vulva (Indian J Dermatol 2012;57:149)

Treatment
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● Excision if possible (Lymphology 2006;39:147); also sclerotherapy or observation
● 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

Micro description
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● 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)

Micro images
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Conjunctiva

Foot

Larynx

Pleura

Retroperitoneum

Vulva

Positive stains
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● CD31, CD34, D2-40 (Pediatr Dev Pathol 2005;8:181); patchy Factor VIII (Am J Clin Pathol 1981;75:167)
● Also CK7 (Hum Pathol 2000;31:1062)

Negative stains
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● Ki-67 (or low, Arch Pathol Lab Med 1997;121:1055)

Electron microscopy description
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● Poorly developed and discontinuous basal lamina; presence of anchoring fibrils (Arch Pathol Lab Med 1980;104:316)

Additional references
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Wikipedia, eMedicine

End of Soft tissue tumors > Lymphangioma - general


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