Skin nonmelanocytic tumor
Fibrous, fibrohistiocytic and myofibroblastic neoplasms
Pleomorphic dermal sarcoma

Editor-in-Chief: Debra L. Zynger, M.D.
Anthony Martinez, M.D.

Topic Completed: 7 November 2019

Minor changes: 5 November 2020

Copyright: 2019-2021, PathologyOutlines.com, Inc.

PubMed Search: Pleomorphic dermal sarcoma

Anthony Martinez, M.D.
Page views in 2020: 6,115
Page views in 2021 to date: 2,909
Cite this page: Martinez A. Pleomorphic dermal sarcoma. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skintumornonmelanocyticpleomorphicdermalsarcoma.html. Accessed May 11th, 2021.
Definition / general
  • An undifferentiated pleomorphic tumor with overlapping features of atypical fibroxanthoma but a higher rate of local recurrence and metastasis
Essential features
  • Undifferentiated pleomorphic tumor involving the dermis that histologically looks like an atypical fibroxanthoma and has any of the following:
    • Size > 2 cm
    • Shows extensive involvement of deeper tissue (subcutis, skeletal muscle, fascia)
    • Necrosis
    • Perineural
    • Lymphovascular invasion
Terminology
  • Pleomorphic dermal sarcoma (PDS)
  • Undifferentiated pleomorphic sarcoma of the skin
  • Superficial malignant fibrous histiocytoma (terminology no longer used)
ICD coding
  • ICD-10: C49.9 - malignant neoplasm of connective and soft tissue, unspecified
Epidemiology
Sites
  • Head and neck, predilection for scalp
Etiology
  • Ultraviolet radiation induced damage and immunosuppression
Clinical features
Diagnosis
  • Dependent on clinical and tissue pathologic correlation
Prognostic factors
Case reports
Treatment
Clinical images

Images hosted on other servers:

Rapidly growing scalp mass

Well circumscribed, erythematous papule

Solitary protuberant mass on upper eyelid

Microscopic (histologic) description
  • Dermal based lesion composed of pleomorphic cells with vesicular nuclei and prominent nucleoli (Cancer 1973;31:1541)
    • Cells can be spindled or epithelioid, often with admixed multinucleated giant cells
    • Cells can be arranged in sheets and fascicles
  • Necrosis often present
  • Perineural and lymphovascular invasion can be seen
  • Infiltration into subcutis, skeletal muscle or fascia
  • Additional findings include myxoid change, pseudoangiomatous growth and storiform growth (Am J Surg Pathol 2012;36:1317)
Microscopic (histologic) images

Contributed by Anthony Martinez, M.D.

Dermal based neoplasm

Cytologic atypia

Necrosis

Extensive subcutis involvement

Sheet-like growth

HMWK

Positive stains
Negative stains
Molecular / cytogenetics description
Sample pathology report
  • Skin, scalp, excision:
    • Pleomorphic dermal sarcoma
    • Tumor measures 3.5 cm in greatest dimension and extensively involves the subcutaneous tissue
    • Necrosis is present: 30%
    • Lymphovascular invasion is not identified
    • Perineural invasion is not identified
    • Margins are negative
Differential diagnosis
Board review style question #1
The following image is from a scalp lesion in an 85 year old man. Immunostains for AE1 / AE3, 34betaE12 / HMWCK / high molecular weight, S100, actin - alpha smooth muscle, desmin and ERG are negative. Which is the best diagnosis?



  1. Atypical fibroxanthoma
  2. Leiomyosarcoma
  3. Pleomorphic dermal sarcoma
  4. Sarcomatoid squamous cell carcinoma
Board review style answer #1
C. Pleomorphic dermal sarcoma

Comment Here

Reference: Pleomorphic dermal sarcoma
Board review style question #2
An 80 year old man has a dermal based scalp lesion characterized by pleomorphic cells growing in fascicles. The lesion is < 2 cm, well circumscribed and completely confined to the dermis. Immunostains are negative for high and low molecular weight keratins, S100, actin - alpha smooth muscle and desmin. What is the best diagnosis?

  1. Atypical fibroxanthoma
  2. Leiomyosarcoma
  3. Pleomorphic dermal sarcoma
  4. Sarcomatoid squamous cell carcinoma
Board review style answer #2
A. Atypical fibroxanthoma

Comment Here

Reference: Pleomorphic dermal sarcoma
Back to top
Image 01 Image 02