OrthoNet

Tumour Syndromes
1) Jaffe-Campanacci - multifocal nonossifying fibroma. Pigmented skin lesions.

2) Albright’s - polyostotic fibrous dysplasia. Endocrine dysftn., precocious puberty, cutaneous pigmentation.

3) Ollier’s - dyschondroplasia with multiple enchondromas, typically affecting one-half the body. Significant bony deformity. Higher incidence of malignant degeneration.

4) Maffucci’s - multiple enchondromas with systemic hemangiomas. Bony deformity and malignant degeneration.

5) Enchondromatosis - multiple enchondromas. Bony deformity and malignant degeneration.

6) Multiple Hereditary Exostoses - autosomal dominant. Bony deformity and malignant degeneration.

7) Letterer-Siwe - disseminated LCH. Severe visceral involvement, esp. HSM, usually fatal in infancy.
Hand-Schuller-Christian - intermediate LCH. Endocrine abnormalities (DI), exophthalmos, & skeletal involvement.