Lymph nodes & spleen, nonlymphoma
Spleen-vascular tumors
Lymphangioma
Author: Vijay Shankar, M.D.
Topic Completed: 1 December 2013
Minor changes: 21 October 2020
Copyright: 2003-2021, PathologyOutlines.com, Inc.
PubMed Search: Lymphangioma[TI] spleen
Table of Contents
Definition / general | Epidemiology | Pathophysiology | Clinical features | Radiology description | Case reports | Treatment | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Positive stains | Differential diagnosisCite this page: Shankar V. Lymphangioma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/spleenlymphangioma.html. Accessed October 25th, 2021.
Definition / general
- Malformed development of lymphatic channels, usually subcapsular
- May involve entire organ
Epidemiology
- Extremely rare, usually an incidental finding
- Any age, often children and women
Pathophysiology
- Abnormal congenital development, bleeding or inflammation of the lymphatic system may cause progressive dilatation of the lymphatic channels but pathogenesis not well understood
Clinical features
- May be asymptomatic
- If symptomatic, upper left quadrant pain is most common symptom, frequently followed by fever, nausea, vomiting, weight loss
- Bleeding, consumptive coagulopathy, hypersplenism, portal hypertension may be seen in extensive and larger lesions
Radiology description
- Ultrasound usually shows hypoechoic spaces that contain internal echoes
- CT may show low density, well delimited subscapular cysts with thin walls with mural calcifications
Case reports
- 30 year old woman with solid cystic mass (World J Gastroenterol 2013;19:781)
- 46 year old woman (Br J Radiol 2007;80:e4)
- 84 year old man (Haematologica 2000;85:314)
Treatment
- Splenectomy is treatment of choice
Gross description
- Solitary or multilocular cystic masses
- Usually subcapsular if solitary
- Have thick fibrous wall with internal morphology characterized by fibrous trabeculae
- May have hyalinization and calcification of fibrous connective tissue
- Rarely solid and cystic (World J Gastroenterol 2013;19:781)
Gross images
Images hosted on other servers:
Multiple, thin walled cystic spaces
4.5 cm x 3.5 cm bulging cystic mass
4.5 cm x 4.5 cm retroperitoneal mass
Microscopic (histologic) description
- Classified as capillary, cavernous or cystic
- Subcapsular, multicystic
- Single layer of flattened endothelium lined spaces, filled with eosinophilic proteinaceous material, not red blood cells
- Endothelium may form small papillary projections
Microscopic (histologic) images
Images hosted on other servers:
H&E staining
H&E and D2-40
Retroperitoneal lymphangioma
Positive stains
- Factor VIII related antigen and CD31 are most reliable markers for endothelial cells lining lymphatic spaces
Differential diagnosis
