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Soft tissue tumors
Lymphangioma - general
Reviewer: Vijay Shankar, M.D. (see Reviewers page)
Revised: 26 February 2013, last major update November 2012
Copyright: (c) 2003-2013, PathologyOutlines.com, Inc.
See also subtypes:
cystic,
lymphangiomatosis,
lymphangiomatous papules/plaques post-radiation
Definition
=========================================================================
● 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
=========================================================================
● Benign
● See also discussion in these chapters:
bone,
breast,
kidney tumor,
lymph nodes,
mediastinum,
skin-nonmelanocytic tumors,
skin-nontumor,
spleen
Case reports
=========================================================================
● 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
=========================================================================
● 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
=========================================================================
Conjunctiva |
Larynx |
Micro 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)
Micro images
=========================================================================
Conjunctiva |
Foot |
Larynx |
Pleura |
Retroperitoneum |
Vulva |
Positive stains
=========================================================================
● 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
=========================================================================
● Ki-67 (or low,
Arch Pathol Lab Med 1997;121:1055)
Electron microscopy description
=========================================================================
● Poorly developed and discontinuous basal lamina; presence of anchoring fibrils
(Arch Pathol Lab Med 1980;104:316)
Additional references
=========================================================================
End of Soft tissue tumors > Lymphangioma - general
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