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

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

Topic Completed: 7 November 2019

Revised: 7 November 2019

Copyright: 2019, PathologyOutlines.com, Inc.

PubMed Search: Pleomorphic dermal sarcoma

Anthony Martinez, M.D.
Page views in 2019: 1,248
Page views in 2020 to date: 702
Cite this page: Martinez A. Pleomorphic dermal sarcoma. PathologyOutlines.com website. http://www.pathologyoutlines.com/topic/skintumornonmelanocyticpleomorphicdermalsarcoma.html. Accessed February 21st, 2020.
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 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 answer #2
A. Atypical fibroxanthoma

Comment Here

Reference: Pleomorphic dermal sarcoma
Back to top