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Soft Tissue Tumors Part 3
Muscle, Vascular, Nerve, Other
Cystic / cavernous lymphangioma (cystic hygroma)
Author: Nat Pernick, M.D, PathologyOutlines.com, Inc.
Revised: 13 July 2010, last major update – October 2009
Copyright: (c) 2002-2010, PathologyOutlines.com, Inc.
Definition
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● Large, irregular vascular spaces (similar to cavernous hemangioma, may resemble cysts) lined by flattened, bland epithelial cells
● Cystic and cavernous hemangiomas are usually considered the same entity (eMedicine)
Clinical
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● In children, usually in neck or axilla; may extend into mediastinum
● Large mediastinal lesions may compress lungs, heart and nerves, but most lesions are asymptomatic and found on Xray
● Rarely occur in lymph nodes (Am J Surg Pathol 1992;16:335)
Case reports
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● 1 year old boy with giant mesenteric tumor (Can J Surg 2009;52:E42)
● 3 year old boy with scrotal tumor with hemorrhage (Afr J Paediatr Surg 2009;6:110)
● 4 year old girl with 30 cm omental tumor (BMC Surg 2006 Dec 27;6:18)
● 38 year old woman with breast tumor (World J Surg Oncol 2007;5:69)
● 46 year old man with 44 cm abdominal mass (Arch Pathol Lab Med 2000;124:1723)
● 50 year old man with mediastinal tumor (J Bras Pneumol 2008;34:982)
● 70 year old woman with 19 kg tumor of broad ligament (J Med Case Reports 2008 Sep 23;2:310)
Treatment and prognosis
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● Complete surgical excision is recommended to prevent recurrence
● May cause fetal death due to hydrops
● Intra-uterine sclerosing agents may reduce respiratory and feeding problems associated with neck tumors (Fetal Diagn Ther 2009;26:102)
Clinical images
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Scrotal tumor with Giant mesenteric tumor 47 cm tumor of 25 cm abdominal
hemorrhage compressing small bowel broad ligament cavity cyst
30 cm abdominal mass arising from omentum
Gross description
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● May be massive
● Gray-white, edematous appearing
● Variable sized cystic cavities with serous type fluid and smooth inner lining
Gross images
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Neck tumor in infant
Micro description
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● Large, irregular vascular spaces lined by flattened, bland epithelial cells with fibroblastic or collagenous stroma
● Stroma may contain lymphocytes
● Abdominal tumors may have marked inflammatory changes that obscure their lymphatic origin (Hum Pathol 2005;36:426)
● Rarely has papillary endothelial proliferation (Arch Pathol Lab Med 1979;103:306)
● No specialized epithelium, no cholesterol granulomas
● Not encapsulated, so removal can be difficult
Micro images
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AFIP Third Series
Orbital tumor has large dilated Numerous lymphatics, some with blood;
lymphatics, some with blood lymphocytes between lymphatics
Other images
Distended lymphatic channels Lymphatic channel has attenuated lining
interspersed with breast lobules
Dilated lymphatics lined by flattened endothelium
Mediastinal tumor Mesenteric tumor 44 cm abdominal mass:
H&E
and Factor VIII
Adrenal gland: Broad ligament: Factor VIII
H&E and CD31 H&E and D2-40
Videos
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Molecular / cytogenetics
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● Associated with Turner’s syndrome (Hum Pathol 1984;15:61) or other chromosomal abnormalities
End of Soft Tissue Tumors Part 3 - Muscle, Vascular, Nerve, Other > Lymphangioma > Cystic / cavernous (cystic hygroma)
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