Spleen
Vascular tumors
Angiosarcoma

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

Revised: 8 March 2018, last major update August 2013

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

PubMed Search: Angiosarcoma[TI] spleen

Cite this page: Shankar, V. Angiosarcoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/spleenangiosarcoma.html. Accessed May 21st, 2018.
Definition / general
  • Rare but most common malignant nonlymphoid tumor of spleen
Terminology
  • Term also encompasses lymphangiosarcomas
Epidemiology
  • Mean age 59 years, range 29 - 85 years, slight male preponderance
  • Associated with microangiopathic anemia, thrombocytopenia, consumptive coagulopathy
  • May develop years after insertion of foreign body, such as a gauze sponge
Pathophysiology
  • Uncertain in many cases but can develop from preexisting hemangioma or hemangioendothelioma
  • Therapeutic irradiation, thorium dioxide administration, pyothorax and polyvinyl chloride exposure are predisposing factors
Clinical features
  • Nonspecific symptoms and findings, such as upper abdominal pain and fullness, fatigue, weight loss, splenomegaly and anemia
  • Leukopoenia, elevated LDH levels and thrombocytopenia are also frequently reported; thrombocytopenia may be the first manifestation
  • Up to 30% present with splenic rupture and acute hemoperitoneum
  • Aggressive (median survival 6 months), almost uniformly fatal with widespread metastases to liver, bone or bone marrow; occasionally lymph nodes or brain
Case reports
Treatment
  • Splenectomy; often chemotherapy and radiotherapy to downstage advanced disease and improve resectability
Gross description
  • Marked splenomegaly, well defined hemorrhagic nodule or diffuse involvement of spleen, often with necrosis
Gross images

Images hosted on other servers:

Extensive necrosis

Hemorrhagic and nodular lesions

Hemorrhagic lesion in the spleen

Microscopic (histologic) description
  • Solid, papillary or freely anastomosing vascular channels (variable even within same case), lined by atypical, hyperchromatic cells with intracytoplasmic hyaline globules
  • Solid, sarcomatous, papillary and epithelial growth patterns can be seen
  • Cells may be epithelioid
  • Frequent hemorrhage, necrosis, hemosiderin, extramedullary hematopoiesis
  • Kaposi-like variant: Kaposi sarcoma-like spindle cell proliferation with slit formation and markedly dilated, sponge-like vascular channels filled with red blood cells
Microscopic (histologic) images

Images hosted on other servers:

Sinusoidal and cavernous growth patterns

Anastomosing vascular channels

Closely packed
vascular channels
lined by atypical
endothelial cells

Proliferating nodules in spleen

Cellular pleomorphism and mitoses in nodules

Diffuse staining with CD31


Kaposi-like variant

Positive stains
Negative stains
  • Keratin (may be focally positive)
Electron microscopy description
  • Shows endothelial cells with cytoplasmic inclusions
Differential diagnosis