Soft tissue
Uncertain differentiation
Intimal sarcoma

Topic Completed: 1 April 2016

Revised: 5 September 2019

Copyright: 2002-2019,, Inc.

PubMed Search: Intimal sarcoma

Vijay Shankar, M.D.
Page views in 2018: 1,837
Page views in 2019 to date: 2,035
Cite this page: Shankar V. Intimal sarcoma. website. Accessed October 18th, 2019.
Definition / general
  • Undifferentiated pleomorphic sarcoma arising from intima or associated with a great vessel
  • Most commonly arises from pulmonary artery or thoracic aorta
  • Mean age 66 years, no gender preference (Am J Surg Pathol 2005;29:1184)
  • Patients present with symptoms from obstruction and compression
  • Most patients have metastatic disease; 80% die within 1 year
  • Excision where feasible as palliative measure
  • Chemotherapy for inoperable or disseminated disease
Gross images

Case of the Week #206

80 year old man with multiple splenic infarcts. Left two images are from abdominal aorta

Gross description
  • Multifocal, forming nodules and plaques on intimal surface that often obstruct vessels or embolize to distal peripheral sites
  • Tumors typically present with thromboemboli (J Bras Pneumol 2009;35:814)
  • Post-mortem diagnosis is common (Hum Pathol 1997;28:1306)
  • Intravascular, polypoid, adherent to vessel wall, can extend along intimal aspect
Microscopic (histologic) description
  • Tumors are typically largely necrotic, with poorly differentiated epithelioid and pleomorphic cells associated with tunica intima
  • Considered to be an undifferentiated pleomorphic sarcoma of the large vessels
Microscopic (histologic) images

Case of the Week #206

80 year old man with multiple splenic infarcts

  • MDM2 is positive in most cases
Differential diagnosis
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