Dermatofibrosarcoma protuberans

Dermatofibrosarcoma protuberans
Updated: 07/28/2020
© Jun Wang, MD, PhD

General features
  • Low to intermediate grade malignancy
  • Rare, usually adults 20 - 40 years, more common in blacks
  • Locally aggressive, low rate of metastasis (only after repeated failures at local control)
  • May progress to fibrosarcoma or undifferentiated pleomorphic sarcoma
Clinical presentations
Pathogenesis
  • Fusion of PDGF-beta with collagen 1 alpha 1 gene causing overexpression of PDGFB
  • Abnormal activation of PDGF receptor by overexpressed PDGFB
Key morphological features
Genetic abnormalities
  • t(17;22)(q22;q13.1): COL1A1-PDGFB
Markers
Treatment
  • Excision with wide margin of subcutaneous fat
  • Tyrosine kinase inhibitor: Imatinib

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Contents

Pathology notes Jun Wang, MD, PhD 1. General information Frequently discussed syndromes Terms of dermatology Terms of hematology 2. Primary immunodeficiency disorders 3. Hemodynamic abnormalities Shock Thrombosis and embolism Bleeding disorders 4.  Benign blood cell disorders Anemia Benign white cell disorders 5. Hematopoietic neoplasms Lymphoid neoplasms Myeloid neoplasms Histiocytic neoplasms: Langerhans cell histiocytosis 6. Cardiovascular system Pulmonary hypertension Cardiovascular system tumors 7. Respiratory tract tumors 8. Gastrointestinal tract Congenital disorders Infectious gastroenteritis Oral cavity Esophagus Stomach Intestinal tumors Salivary glands Exocrine pancreas and gallbladder 9. Urinary system Kidney masses Lower urinary tract 10. Male reproductive system 11. Female genital tract 12. Breast 13. Endocrine organs Pituitary gland Adrenal gland Thyroid gland Parathyroid gland Endocrine pancreas M
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Anemia

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