Fibrous dysplasia

Fibrous dysplasia
Updated: 07/31/2018
© Jun Wang, MD, PhD

General features
  • Developmental, non-neoplastic disorder of bone-forming mesenchyme
  • Usually begins prior to puberty and grows slowly
  • Slightly more common in men
  • Malignant transformation: Rare, may represent dedifferentiation of low-grade osteosarcoma; may occur after radiation therapy
Clinical presentations
  • Deformities
  • May have pain or swelling
Pathogenesis
  • Bone maturation arrest at the woven bone stage
Most common sites
  • Medulla of diaphysis or metaphysis of craniofacial bones, femur, ribs
Key radiologic findings
  • Lytic lesion in the metaphysis or diaphysis 
  • "Ground glass" appearance
Key morphological features
  • Irregular trabeculae of metaplastic woven bone (never matures)
  • Hypocellular, fibroblastic stroma
Genetic abnormality
  • Probably activating point mutation (possibly a submit of GNAS1), resulting elevated intracellular cyclic adenosine monophosphates (cAMP)
Treatment
  • Follow up
  • Curettage, bone grafting, and stabilization for patients with symptoms (pain, deformity) or fracture


Back to bone tumors
Back to contents

Comments

Post a Comment

Popular posts from this blog

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 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 Multiple endocrine neoplasms 14. B
Read more

Female genital tract

Pathology of female genital tract   Updated: 01/23/2024 © Jun Wang, MD, PhD Key anatomic features Opened channel, pathological basis for pelvic inflammatory disease Cervix: exocervix, endocervix, transformation zone Uterus: fundus, body Fallopian tube: intramural, isthmus, ampulla, infundibulum, fimbriae Placenta: umbilical cord, fetal membrane, placenta Key histologic features Stratified squamous epithelium from vulva to exocervix Simple columnar epithelium from endocervix to fimbriae Uterine corpus Endometrium: Glands, stroma Myometrium Perimetrium: Mesothelium/peritoneum Ovaries: three components Epithelium (mesothelium) Stroma Germ cells (follicles) Placenta: Cytotrophoblast, syncytiotrophoblast, intermediate trophoblast Infectious/inflammatory diseases Diseases of vagina and vulva Molluscum contagiosum Lichen sclerosus Paget disease Sarcoma botryoides Squamous cell carcinoma Diseases of cervix Cervical intraepithelial neopla
Read more

Neoplasms of respiratory tract

Neoplasms of respiratory tract   Updated: 11/14/2022 © Jun Wang, MD, PhD Anatomy Nasal cavity Nasopharynx Oropharynx Laryngopharynx Larynx Trachea and bronchi Lungs Histology Squamous mucosa Respiratory mucosa May have abundant lymphoid tissue in pharynx Type I and Type II pneumocytes Key risk factors Human papillomavirus: Squamous cell carcinoma, squamous cell papilloma Epstein Barr virus: Nasopharyngeal carcinoma Smoking Tumors of nasal cavity and nasopharynx Olfactory neuroblastoma Nasopharyngeal angiofibroma Sinonasal papilloma NUT midline carcinoma Nasopharyngeal carcinoma Tumors or tumor like lesions of larynx Vocal cord polyp Squamous papilloma Squamous cell carcinoma Branchial pouch/cleft anomalies Branchial cyst Thyroglossal duct cyst Neoplasms of lung Squamous cell carcinoma Adenocarcinoma Large cell carcinoma Small cell carcinoma Carcinoid Lymphangioleiomyomatosis Neoplasms of pleura Mesothelioma
Read more