Images of Musculoskeletal Oncology

Chondrosarcoma Secondary to Hereditary Multiple Osteocartilangious Exostoses

A 17 year old woman developed dyspareunia as a result of multiple hereditary osteocartilangious exostoses involving her pelvis and relieved by surgical excision of benign exostoses of one pubis.
These femoral exostoses were not treated.
We first became involved in her care when she developed a large, somewhat painful calcific mass in the same site three years later (arrow).
Biopsy revealed a low grade chondrosarcoma thought to be the result of malignant degeneration of one of the exostoses.

A wide excision was planned.
Note the widely resected specimen on the preop radiograph. The pelvis was resected from the symphysis to just below the acetabulum.
The resection defect was not reconstructed and the patient’s gait and function was amazingly close to normal.
This is a photograph of the resected specimen which demonstrates a mass of light blue chondrosarcoma in the soft tissues adjacent to the cartilage cap of the exostoses originating from the pubis. Note the similarity between the cartilage cap and the malignant chondrosarcoma.
The patient did quite well clinically, but developed a number of local recurrences that were surgically removed.

A recurrence developed in the floor of the pelvis and was surgically removed with a segment of the vagina and the adjacent urinary bladder.

The patient is alive and clinically doing well with disease at the age of 63, which is now 40 years after her initial chondrosarcoma resection. She has intermittent headaches due to two or three small brain metastases with which she is coping.
She is an intelligent woman who has founded a chondrosarcoma support group in the California area.
Learning Issues:

1. Malignant degeneration of a hereditary osteocartilaginous exostoses is a feared complication which occurs in about 10% of cases during the course of a patient’s lifetime.

2. The secondary chondrosarcomas may recur, but with proper treatment become a challenging chronic illness with long time survival, as in this patient's case.

3. Partial resection of the patient’s pelvis for neoplasm without reconstruction may preserve good function, depending on what was surgically removed.


Images of Musculoskeletal Oncology
University at Buffalo Department of Orthopaedics