Cystic / cavernous lymphangioma

Soft tissue

Vascular

Benign

Topic Completed: 1 November 2012

Minor changes: 7 December 2020

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PubMed Search: Cystic [title] OR cavernous [title] lymphangioma, "cystic hygroma"

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Table of Contents

Cite this page: Shankar V. Cystic / cavernous lymphangioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissuelymphangiomacystic.html. Accessed May 7th, 2021.

Definition / general

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

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 x-ray
Rarely occur in lymph nodes (Am J Surg Pathol 1992;16:335)

Case reports

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;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)
29 year old woman with longstanding neck swelling (Case of the Week #377)
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

Complete surgical excision is recommended to prevent recurrence
May cause fetal death due to hydrops
Intrauterine 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

Images hosted on other servers:

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

Thigh tumor

Mesenteric tumor: 12 year old girl

Mesenteric tumors

Gross description

May be massive
Gray white, edematous appearing
Variable sized cystic cavities with serous type fluid and smooth inner lining

Microscopic (histologic) description

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

Microscopic (histologic) images

Case #377