Soft tissue
Uncertain differentiation
Intimal sarcoma
Author: Vijay Shankar, M.D.
Last author update: 1 April 2016
Last staff update: 21 April 2021
Copyright: 2002-2023, PathologyOutlines.com, Inc.
PubMed Search: Intimal sarcoma
Table of Contents
Definition / general | Treatment | Gross description | Gross images | Microscopic (histologic) description | Microscopic (histologic) images | Immunohistochemistry & special stains | Differential diagnosisCite this page: Shankar V. Intimal sarcoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/softtissueintimalsarcoma.html. Accessed June 7th, 2023.
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
Treatment
- Excision where feasible as palliative measure
- Chemotherapy for inoperable or disseminated disease
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
Gross images
Case #206
80 year old man with multiple splenic infarcts. Left two images are from abdominal aorta
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 #206
80 year old man with multiple splenic infarcts
Immunohistochemistry & special stains
- MDM2 is positive in most cases
Differential diagnosis
- Angiosarcoma: CD31 is positive
- Alveolar rhabdomyosarcoma: desmin and myogenin and/or MyoD1 positivity
- Synovial sarcoma: look for translocation partners
- Metastatic melanoma: S100 positive
- Cardiac (atrial) myxoma: will appear as low grade lesion
- Leiomyosarcoma: desmin and SMA positivity
