Spleen
Vascular tumors
Lymphangioma

Author: Vijay Shankar, M.D. (see Authors page)

Revised: 8 March 2018, last major update December 2013

Copyright: (c) 2003-2018, PathologyOutlines.com, Inc.

PubMed Search: Lymphangioma[TI] spleen

Cite this page: Shankar, V. Lymphangioma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/spleenlymphangioma.html. Accessed July 16th, 2018.
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
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:

Cystic spaces lined by attenuated endothelium

H&E staining

H&E and D2-40

Retroperitoneal lymphangioma

Positive stains
Differential diagnosis