II. Definitions

  1. Dermatofibrosarcoma Protuberans
    1. Low grade fibroblastic tumor presents as a firm, nodular, Subcutaneous Mass of the trunk or proximal extremities
    2. Locally destructive but rarely metastasizes

III. Epidemiology

  1. Onset ages 20 to 59 years old
  2. No gender predominance
  3. Rare (accounts for 1% of Soft Tissue Sarcomas)

IV. Findings

  1. Slow growing Subcutaneous Nodule with overlying pink Plaque
  2. Advanced lesions may ulcerate and bleed
  3. Distribution
    1. Trunk: 50 to 60%
    2. Proximal arms: 25%
    3. Head and Neck: 10 to 15%

V. Imaging

  1. Ultrasound
    1. Hypervascularity
    2. Tentacle-like projections

VI. Management

  1. Refer to orthopedic oncology for core needle biopsy and wide surgical resection

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Related Studies

Ontology: Dermatofibrosarcoma Protuberans (C0392784)

Definition (NCI) An intermediate cutaneous fibrohistiocytic neoplasm, presenting as a nodular cutaneous mass, most often on the trunk and the proximal extremities. The tumor diffusely infiltrates the dermis and the subcutaneous tissues. It is considered a locally aggressive neoplasm, which often recurs but rarely metastasizes.
Definition (NCI_NCI-GLOSS) A type of tumor that begins as a hard nodule and grows slowly. These tumors are usually found in the dermis (the inner layer of the two main layers of tissue that make up the skin) of the limbs or trunk of the body. They can grow into surrounding tissue but do not spread to other parts of the body. These tumors are related to giant cell fibroblastomas.
Concepts Neoplastic Process (T191)
MSH D018223
ICD10 M8832/1
SnomedCT 188140006, 76594008, 276799004
English DFSP - Dermatofibrosarc protru, Dermatofibrosarcom protuberans, Dermatofibroma protuberans, dermatofibrosarcoma protuberans, DFSP, DERMATOFIBROSARCOMA PROTUBERANS, [M]Dermatofibroma protuberans, dermatofibrosarcomas protuberans, Dermatofibrosarcoma protuberans (diagnosis), soft tissue malignant neoplasm dermatofibrosarcoma protuberans, Protuberans, Dermatofibrosarcoma, Protuberan, Dermatofibrosarcoma, Dermatofibrosarcoma Protuberan, Dermatofibrosarcoma protuberans, DFSP - Dermatofibrosarcoma protruberans, Dermatofibrosarcoma protuberans (disorder), dermatofibrosarcoma; protuberans, protuberans; dermatofibrosarcoma, Dermatofibrosarcoma protuberans, NOS, Dermatofibrosarcoma, Dermatofibrosarcoma Protuberans
Czech Dermatofibrosarkoma protuberans
Dutch dermatofibrosarcoma protuberans, dermatofibrosarcoom; protuberans, protuberans; dermatofibrosarcoom
French Dermatofibrosarcome protubérant
German Dermatofibrosarcoma protuberans
Hungarian Dermatofibrosarcoma protuberans
Italian Dermatofibrosarcoma protuberante
Japanese 隆起性皮膚線維肉腫, リュウキセイヒフセンイニクシュ
Portuguese Dermatofibrossarcoma protuberante
Spanish Dermatofibrosarcoma protuberante, dermatofibrosarcoma protuberante (trastorno), dermatofibrosarcoma protuberante, dermatofibrosarcoma protuberans