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Soft tissue tumors

Lymphangioma

Cystic / cavernous lymphangioma (cystic hygroma)


Reviewer: Vijay Shankar, M.D. (see Reviewers page)
Revised: 26 February 2013, last major update November 2012
Copyright: (c) 2003-2013, 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 lymphangiomas are usually considered the same entity (eMedicine)

Clinical features
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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)
● 7 year old boy with thigh tumor (Indian J Surg 2010;72:417)
● 12 year old girl with giant cystic lymphangioma of mesentery (Pan Afr Med J 2012;12:7)
● 12 year old girl with giant cystic lymphangioma of small bowel mesentery (Pan Afr Med J 2011;9:46)
● 25 year old woman with huge cystic lymphangioma mimicking ovarian malignancy (Turk J Gastroenterol 2011;22:344)
● 34 year old woman with cystic pancreatic lymphangioma (Rare Tumors 2012;4:e27)
● 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 Rep 2008;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 fetal neck tumors (Fetal Diagn Ther 2009;26:102)
● Rarely may spontaneously regress

Clinical images
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Omentum: 30 cm tumor in 4 year old girl


Scrotal tumor with hemorrhage in 3 year old boy


Abdominal cyst: 25 cm

Broad ligament: 19 kg tumor in 70 year old woman

Mesentery: 1 year old boy


Mesenteric tumors


Thigh tumor

Mesenteric tumor: 12 year old girl

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

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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Omentum

Pancreas

Abdominal mass

Mediastinal tumor

Mesenteric tumor

Adrenal gland: H&E and CD31

Broad ligament: H&E and D2-40

Breast tumor

Factor VIII

AFIP Third Series


Orbital tumor has large dilated lymphatics, some with blood

Numerous lymphatics, some with blood; lymphocytes between lymphatics

Videos
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Neck tumor

Molecular / cytogenetics description
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● Associated with Turnerís syndrome (Hum Pathol 1984;15:61) or other chromosomal abnormalities

End of Soft tissue tumors > Lymphangioma > Cystic / cavernous lymphangioma (cystic hygroma)


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