Enchondromatosis is a non-hereditary disease, characterised by the presence of multiple enchondromas. While Ollier Disease is typified by multiple enchondromas, in Maffucci Syndrome they are combined with haemangioma.

Due to the rarity of these diseases, systematic studies on clinical behaviour providing information how to treat patients are lacking.

This study intends to answer the following questions: What are predictive factors for developing chondrosarcoma? When is extensive surgery necessary? How often patients die due to dedifferentiation or metastasis?

Twelve institutes in eight countries participated in this descriptive retrospective EMSOS-study. 118 Patients with Ollier Disease and 15 patients with Maffucci Syndrome were included. Unilateral localization of disease was found in 60% of Ollier patients and 40% of patients with Maffucci Syndrome.

One of the predictive factors for developing chondrosarcoma is the location of the enchondromas; the risk increases especially when enchondromas are located in the scapula (33%), humerus (18%), pelvis (26%) or femur (15%). For the phalanges, this risk is 14% in the hand and 16% in the feet. The decision whether or not to perform extensive surgery is difficult, especially in patients who suffer multiple chondrosarcomas.

Malignant transformation was found in fourty-four patients with Ollier Disease (37%) and eight patients with Maffucci Syndrome (53%). Multiple synchronous or metachronous chondrosarcomas were found in 15 patients.

Nine patients died (range 21–54 yrs). Seven of them died disease related due to pulmonary metastasis (2 humerus, 2 pelvis, 3 femur). Two patients died from glioma of the brain.

In conclusion, one important predictive factor for developing chondrosarcoma is the location of the enchondromas; interestingly, only patients with chondrosarcoma outside the small bones died of their disease. In this series, no dedifferentiation of chondrosarcoma was seen. A first design flow-chart how to approach chondrosarcoma in patients with Ollier Disease and Maffucci Syndrome is in preparation.

Nowadays more attention is paid to the quality of life during and after cancer treatment, and fatigue is an important factor influencing this. Still little is known about the development of fatigue before, during and after cancer treatment and its contributing factors. We analyzed the level of fatigue, pain, anxiety and activity before and after the treatment of benign or low-grade malignant bone and soft tissue tumours in 43 patients. All patients were treated with surgery only. The mean age of the patients was 40 years (range 20 to 67 years). Fatigue severity was measured with the CIS-fatigue questionnaire, where a score of 35 or higher reflects severe fatigue. The VAS score was used to measure pain (0=no pain, 10=severe pain), and the Dutch version of the Spielberger State-Trait Anxiety Inventory to measure state anxiety. Physical activity was measured with an actometer, worn at the ankle for two weeks. All measurements were done before the tumor surgery and twelve months later.

Severe fatigue was seen in 35% of the patients before they had tumor surgery. After 12 months 32% of the patients still was severely fatigued. The mean VAS pain score was 2,3 before treatment and 2,2 after 12 months. The anxiety score lowered from 38,1 before treatment to 33,2 one year later. Actometer scores increased from 57,7 before treatment to 69,9 after 12 months. Fatigue severity correlated with pain and anxiety both before and 12 months after treatment, but not with actometer scores.

In this study we see that severe fatigue is present in 35% of tumor patients before they are treated, and this percentage remains high (32%) until one year after surgery. Since severe fatigue correlated with more anxiety and pain, these symptoms can help us understand and treat severe fatigue in tumor patients better.

Introduction: Both enchondromas and chondrosarcomas are mesenchymal neoplasms which originate from cartilage cells, and they occur mainly in the extremities. Both these tumours are resistant to chemotherapy and radiotherapy, and surgery is the only treatment option. In the last few years limb saving procedures have become the treatment of choice. Intra-operative cryosurgery has been introduced as a local adjuvant therapy for skeletal benign and low-grade malignant tumours. It is applied after curettage of the lesion to destroy any remaining tumour cells, and to enlarge the oncological margin of resection. Since the introduction of cryosurgery as an adjuvans, oncological and functional results of this extremity sparing surgery are significantly enhanced.

Patients and Methods: A retrospective study was conducted to evaluate the oncological and functional results, and the complications of cryosurgical treatment.

Data were prospectively collected from the tumour register and patient records. Functional scores of the affected limbs were assessed according to the Musculo-Skeletal Tumour Society scoring system.

Results: Between 1994 and 2003 123 patients (47 men, 76 women, average age 49 years; range 13–83 yrs) were treated with curettage and cryosurgery for an Enneking stage 3 enchondroma (75 patients) or a low-grade chondrosarcoma (55 patients).

The minimal follow up was two years, and the average follow up 50 months (range 24–119 months).

At follow up three recurrences had occurred in patients treated for enchondroma. One residual tumour was diagnosed in a patient with chondrosarcoma grade Ib. All patients were treated again with curettage and cryosurgery and disease free at the latest follow-up.

Of the 37 complications the most common were a fracture at the surgical site (18), fracture of osteosynthesis (6), 3 wound infection (3), delayed soft tissue healing (3), and transient nerve palsy (3).

Functional MSTS scores increased in time to an average of 28 points (94%) at two year follow up. No significant difference in scores were found regarding to localisation of the lesion, age or gender. A significant discrepancy in functional scores was observed between patients who did suffer from one or more complications and patients who did not.

Conclusion: We believe that the use of cryosurgery is an excellent adjuvant therapy after curettage to achieve local control of aggressive enchondromas and low grade chondrosarcomas. It avoids the need for segmental resection, making reconstruction of the bony defect easier and therefore results in excellent functional outcome. Due to the initial high fracture rate osteosynthesis at the surgical site is used more often, and weight baring mobilisation is postponed until full consolidation is reached.