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Osteopoikilosis

Summary

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.

The condition is hereditary, with equal susceptibility for males and females
Osteopoikilosis presents with no symptoms. Though, those with a family history of the condition are more likely to inherit it.
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.
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