Summary
Description
Osteopoikilosis, multiple bone islands, is a condition is transmitted in an autosomal dominant manner. Lesions are usually located near the joint areas of the long bones, pelvis, scapula, and in the small bones of the hands and feet.
People and Age
The condition is hereditary, with equal susceptibility for males and females
Symptoms and Presentation
Osteopoikilosis presents with no symptoms. Though, those with a family history of the condition are more likely to inherit it.
Brief description of the xray
Radiographically, the bone appears to have a spotted appearance.
Complete Information on this Tumor
Introduction and Definition
Osteopoikilosis, multiple bone islands, is a condition of unknown origin which is transmitted in an autosomal dominant manner. The multiple, sclerotic, roundish lesions vary in size from a few millimeters to several centimeters. They are scattered within the cancellous bone and concentrated near the joint surfaces.
Incidence and Demographics
The lesions cluster at the end of long bones, around the acetabulum of the pelvis, and around the glenoid of the scapula, and in the small bones and cuboid bones of the hands and feet. Other than the pelvis, the axial skeleton is rarely involved. Lesions may increase or decrease in size as well as number.
high penetrance (meaning patients with the gene usually have the condition). Some cases are spontaneous and there is no family history. Males and females are equally affected.
Symptoms and Presentation
Patients are asymptomatic, and laboratory exams are normal. Osteosarcoma has been reported to occur in affected bone. Associated conditions include: dermatofibrosis lenticularis disseminata, scleroderma, syndactyly, dwarfism, endocrine abnormalities, melorrheostosis, and cleft palate. Most patients have no other findings.
X-Ray Appearance and Advanced Imaging Findings
Radiographically, lesions are seen in the metaphyseal and epiphyseal areas of affected bones. The bones show multiple discrete or clustered foci of radiopacity with uniform density, giving the bone a spotted appearance. Bone scan activity is normal.
Differential Diagnosis
Sclerotic metastatic cancer, mastocytosis, tuberous sclerosis
Histopathology findings
Pathologically, the lesions resemble a bone island.
Suggested Reading and Reference
Bullough and Invigorita, Atlas of Orthopaedic Pathology, 1st Ed. University Park Press
Greenfield and Arrington, Imaging of Bone tumors, Lippencott, 1995
Resnick, D., Bone and Joint Imaging, W.B. Saunders, Philadelphia, PA, 1989,pp. 1234-1239.
