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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

 

 

                 Object name is kjr-10-319-g001.jpg               

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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Neck tumor

 

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)

 

 

 

This information is intended for physicians and related personnel, who understand that medical information is often imperfect, and must also be interpreted in the context of a patient's clinical data using reasonable medical judgment.  This website should not be used as a substitute for the advice of a licensed physician.

 

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