Tumour Syndromes

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.

8) Hand-Schuller-Christian – intermediate LCH. Endocrine abnormalities (DI), exophthalmos, & skeletal involvement.