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Soft Tissue Tumors Part 3
Muscle, Vascular, Nerve, Other
Lymphangioma - general
Author: Nat Pernick, M.D, PathologyOutlines.com, Inc.
Revised: 22 October 2009, last major update – October 2009
Copyright: (c) 2002-2009, 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
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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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● Mediastinal tumor in pregnant woman with mixed capillary and cystic histology (Ultrasound Obstet Gynecol 2008;31:697)
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)
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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Retroperitoneal tumor Tumor of foot Dilated lymphatics lined by
flattened endothelium
H&E, CD31, elastin
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
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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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End of Soft Tissue Tumors Part 3 - Muscle, Vascular, Nerve, Other > Lymphangioma > General
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