Skin nonmelanocytic tumor

Fibrous, fibrohistiocytic and myofibroblastic neoplasms

Pleomorphic dermal sarcoma

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

Last author update: 7 November 2019
Last staff update: 31 March 2023

Copyright: 2019-2024,, Inc.

PubMed Search: Pleomorphic dermal sarcoma

Anthony Martinez, M.D.
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Cite this page: Martinez A. Pleomorphic dermal sarcoma. website. Accessed May 19th, 2024.
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
  • 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
  • Head and neck, predilection for scalp
  • Ultraviolet radiation induced damage and immunosuppression
Clinical features
  • Dependent on clinical and tissue pathologic correlation
Prognostic factors
Case reports
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


Extensive subcutis involvement

Sheet-like growth


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

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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
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