[Three patients with hereditary multiple exostoses and malignant degeneration of an osteochondroma located in the pelvis].
In three male patients with hereditary multiple exostoses (HME), aged 50, 29 and 31 years, peripheral low-grade chondrosarcoma in the pelvic region led to swelling or pain. In the first patient, curative resection was not feasible because of the size and extension of the tumour. However, rapid tumour growth and unbearable pain necessitated a debulking procedure 16 months later. Histopathologic examination revealed a highly malignant dedifferentiated chondrosarcoma. The patient died two years after initial presentation as a result of local tumour growth. In the second patient, treatment consisted of wide resection of the tumour. Five years after the surgery the patient was free of disease. The third patient was initially treated by intralesional resection, followed by partial hemipelvectomy because of residual tumour. Thirteen months later, a local recurrence occurred that was treated by wide excision. Four years after the partial hemipelvectomy the patient was both pain-free and disease-free. Patients with HME are at increased risk for malignant degeneration of pelvic osteochondroma to chondrosarcoma. Periodic control of patients with pelvic osteochondromas is advised, preferably once every two years.
Onze Lieve Vrouwe Gasthuis, afd. Orthopedie, Postbus 95.500, 1090 HM Amsterdam. sj.ham@olvg.nl
S J
SJ
Ham
M
M
Heeg
J R
JR
van Horn
D000328:Adult; D001859:Bone Neoplasms / Q000209:etiology* / Q000235:genetics / Q000601:surgery; D002813:Chondrosarcoma / Q000209:etiology* / Q000235:genetics / Q000601:surgery; D005097:Exostoses, Multiple Hereditary / Q000150:complications* / Q000235:genetics / Q000601:surgery; D017809:Fatal Outcome; D006428:Hemipelvectomy; D006801:Humans; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D010146:Pain / Q000209:etiology; D010384:Pelvic Bones / Q000000981:diagnostic imaging / Q000473:pathology / Q000601:surgery; D011859:Radiography; D016896:Treatment Outcome