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[Multiple exostoses].

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Exostoses, Multiple Hereditary↗
PubMed1978

[Multiple cartilaginous exostoses and neoplastic degeneration: review of the literature (author's transl)].

In about 10% of patients suffering from multiple osteochondroma a malignant degeneration of one osteochondroma occurs. Data of 59 patients are collected from the literature. The malignant degeneration occurs at the age of 31 in average, mostly on the pelvic girdle, less frequently on the shoulder girdle and on the ribs. The development is slow in most cases, at times interrupted. The first clinical signs are an increase in swelling, rarely pain or neurological symptoms. Radiological findings and prognosis correspond well with those of a proliferative chondroma or a primary chondrosarcoma. Recurrences after local treatment are frequent, metastases are rarely found. Regular check-ups and good information of patients suffering from multiple osteochondroma are recommended.

Adult↗
PubMed1993

Multiple hereditary exostoses. An epidemiologic study of an isolated community in Manitoba.

From 1986 through 1988, 266 persons (149 adults and 117 children) were screened for multiple hereditary exostosis (MHE) in an isolated northern Ojibway community. Physical examination and confirmation by roentgenogram skeletal survey disclosed 21 children (19.4%) and 14 adults (9.5%) affected with MHE. Forty-one percent of children had lesions detectable before ten years of age, some as early as two years of age. Seventy-four percent of the lesions were characteristically sessile. Although lesions about the knee were most common, sites previously thought to be uncommon such as the metatarsals, hand, and spine were involved in 40% of the children. No cases of malignant degeneration have occurred in the adult population. Severity and multiplicity of lesions in successive generations point to an oncogenic gene origin. This study shows striking variance from current literature and provides a unique and valuable baseline assessment of research on the cause and natural history of MHE.

Adolescent↗