The cryosurgical treatment of chondroblastoma of bone: long-term oncologic and functional results.

BACKGROUND: Chondroblastoma of bone is a rare tumor that occurs most often in the epiphysis or apophysis of long bones. This benign tumor sometimes shows aggressive or malignant behaviour, and rarely metastases occur. Since wide resection often leads to growth impairment, intra-lesional curettage followed by an adjuvant therapy is developed to decrease the negative effects on growth and function without increased recurrence rates. PATIENTS AND METHODS: Between 1980 and 2001, 18 patients were treated for chondroblastoma of bone. Since 1989 cryosurgery has been used as an adjuvant treatment. The functional and oncologic results at follow-up were studied. RESULTS: Four patients were treated with curettage, and four with wide resection without adjuvant therapy. One patient developed a local recurrence and distant metastases. Ten patients were treated with curettage and cryosurgery. One patient developed a local recurrence 6 years later that was treated with curettage and cryosurgery again. No serious complications from cryosurgery occurred and the functional results at follow-up were excellent [mean MSTS score at follow-up 29]. CONCLUSIONS: Chondroblastoma of bone is a benign tumor with a relatively high recurrence rate. The local recurrence rates can be decreased with cryosurgery as a local adjuvant therapy with excellent functional results.

Cryosurgery in aggressive, benign, and low-grade malignant bone tumours.

Cryosurgery is a method of treatment for various tumours that induces tissue necrosis with ablative intent. It is used in benign, aggressive, and low-grade malignant bone tumours such as chondrosarcoma grade 1. We describe the history of the technique and the issues associated with cryobiology, as well as the indications, technique, complications, and results of cryosurgery. At the University Medical Centre Nijmegen, Netherlands, 302 tumours have been treated by use of cryosurgery with at least 2 years' follow-up. 96-100% of patients were cured-the response depended on tumour type. Comparison of functional results with data from studies shows that these results concur with other studies on cryosurgery, and are at least equal to results of marginal excision and mostly better than those of wide excisions of grade 1 chondrosarcomas. Thus from an oncological point of view, cryosurgery combined with intralesional excision is equal to marginal excision.

Angiosarcoma with malignant peripheral nerve sheath tumour developing in a patient with klippel-trénaunay-weber syndrome.

PURPOSE: We discuss the coexistence of Klippel-Trénaunay-Weber syndrome with various malignancies, the possible histogenetic pathways and therapeutic implications. PATIENT: We report on a 46-year-old man presenting with increasing pain and swelling of his right lower leg after fracturing his fibula. Since birth he was known as having the uncommon syndrome of Klippel-Trénaunay-Weber of his right lower leg. METHODS: Our patient underwent an above-knee amputation for biopsy-proven malignant vascular tumour, first thought to be a composite hemangio-endothelioma and/or angiosarcoma with lung metastases. RESULTS: In the amputated extremity, a vascular malformation was found with tumour showing various components with foci of angiosarcoma adjacent to diffuse neurofibroma and areas with high-grade malignant peripheral nerve sheath tumour. Amputation and palliative chemotherapy were indicated, but he died of pulmonary and cerebral metastases 2 months postoperatively. DISCUSSION: This case describes an angiosarcoma with malignant peripheral nerve sheath tumour developing in a patient with Klippel-Trénaunay-Weber syndrome. A case never described before in literature and probably, as in our case, difficult to diagnose at first biopsy.

Limb salvage in musculoskeletal oncology.

Since the early 1970s, substantial progress in dealing with musculoskeletal tumours has been made, with improvements in surgical skills, radiology, chemotherapy, pathology, and radiotherapy. Nowadays, 70-85% of all malignant tumours are treated by limb salvage, without compromising the oncological result. After many years, the functional result that may be achieved with a limb-saving procedure is becoming clear. Limb salvage has cosmetic advantages, but whether the quality of life of these patients is superior to that of those who undergo amputation is unclear. The non-oncological complication rate is much higher after limb salvage than after amputation, and consequently additional surgery is necessary. In the future, the co-operating disciplines should strive for better survival of these patients, for which the development of new chemotherapeutic drugs is especially needed.

Oncologic and functional results after treatment of giant cell tumors of bone.

Surgical treatment of giant cell tumor of bone has always been a difficult problem because of its local aggressive behavior. Oncologic results and functional outcome are reported here in a retrospective study of 36 patients, treated by various surgical procedures. The average age at the time of diagnosis was 34 years, and the median follow-up period was 7 years. Twenty-three patients were treated by intralesional excision with local adjuvant therapy, and 11 patients by extralesional excision. Two patients received radiotherapy only. Seven local tumor recurrences (30%) were encountered after intralesional procedures, while local tumor control was the rule after extralesional excision. Intralesional excision with local adjuvant therapy resulted in significantly better functional results compared with extralesional excision. Wide excision was associated with a poor functional outcome and marginal excision with a good functional outcome. For the treatment of giant cell tumor of bone, intralesional excision with local adjuvant therapy is recommended because of a good functional outcome. When applying cryosurgery as the local adjuvant, more vigorous freezing may be necessary to improve local tumor control.