Soft tissue tumors
High grade vascular lesions
Intimal sarcoma

Author: David L. Stockman, M.D. (see Authors page)

Revised: 13 April 2016, last major update April 2016

Copyright: (c) 2003-2016, PathologyOutlines.com, Inc.

PubMed Search: Intimal sarcoma
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
Case Reports
Treatment
  • Excision where feasible as palliative measure
  • Chemotherapy for inoperable or disseminated disease
Gross Images

Images hosted on Pathout server:

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
Micro Images

Images hosted on Pathout server:

80 year old man with multiple splenic infarcts



Images hosted on other servers:

Causing aortic rupture

Micro 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
Immunohistochemistry
  • MDM2 is positive in most cases
Differential Diagnosis