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Soft Tissue Tumors Part 3
Muscle, Vascular, Nerve, Other
Lymphangiomatosis
Author: Nat Pernick, M.D, PathologyOutlines.com, Inc.
Revised: 17 October 2009, last major update – October 2009
Copyright: (c) 2002-2009, PathologyOutlines.com, Inc.
Definition
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● Diffuse / multicentric lymphangiomas, often in thoracic cavity, also extremities, bone, soft tissue, viscera
● See also lung-tumor chapter
Clinical
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● Rare, idiopathic, occurs mostly in children
● Cases with visceral involvement are often fatal (Am J Surg Pathol 1993;17:329)
● Cases involving limbs have good prognosis (Am J Surg Pathol 1995;19:125)
● Associated with kaposiform hemangioendothelioma (Am J Surg Pathol 1993;17:321)
● Associated with bony lytic lesions and chylothorax (J Pediatr Hematol Oncol 2008;30:66)
● Diffuse retroperitoneal cystic abdominal lymphangiomatosis: 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 spleen, liver, bone, pancreas, soft tissue, limbs or brain (Acta Cytol 2009;53:191)
● Gorham’s disease: diffuse lymphangiomatosis also involving bony tissue.
Case reports
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● 33 year old woman with fatal case (Am J Med Sci 2008;336:445)
● 71 year old man with mesenteric lymphangiomatosis (World J Gastroenterol 2009;15:3947)
● Three cases associated with surgery for complex congenital heart disease (Pediatr Cardiol 2008;29:771)
Treatment
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● Excision if symptomatic
Micro description
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● 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
Micro images
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Jejunal tumor (fig E-G) Colonic tumor Splenic tumor
Lung: H&E and CD31
Positive stains
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● CD31, Factor VIII
End of Soft Tissue Tumors Part 3 - Muscle, Vascular, Nerve, Other > Lymphangioma > Lymphangiomatosis
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