Soft tissue
Vascular
Benign
Cystic / cavernous lymphangioma
Author: Vijay Shankar, M.D.
Topic Completed: 1 November 2012
Minor changes: 7 December 2020
Copyright: 2002-2021, PathologyOutlines.com, Inc.
PubMed Search: Cystic [title] OR cavernous [title] lymphangioma, "cystic hygroma"
Table of Contents
Definition / general | Clinical features | Case reports | Treatment | Clinical images | Gross description | Microscopic (histologic) description | Microscopic (histologic) images | Molecular / cytogenetics description | VideosCite this page: Shankar V. Cystic / cavernous lymphangioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissuelymphangiomacystic.html. Accessed October 16th, 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
AFIP images
Orbital tumor has large dilated lymphatics, some with blood
some with blood;
lymphocytes between
lymphatics
Images hosted on other servers:
Omentum
Pancreas
Mesenteric tumor
Broad ligament: H&E and D2-40
Breast tumor
Molecular / cytogenetics description
- Associated with Turner’s syndrome (Hum Pathol 1984;15:61) or other chromosomal abnormalities
Videos
Neck tumor
