Oncological and functional results of cryosurgical therapy of enchondromas and chondrosarcomas grade 1.

BACKGROUND AND OBJECTIVES: Cryosurgery using liquid nitrogen is used as adjuvant treatment after intralesional resection of bone tumours to induce cell death. It is applied to enlarge the oncological margins of resection and to reduce the local recurrence rate. The objective of this study is to analyze the oncological and functional results. METHODS: We studied the oncological and functional results of curettage and cryosurgery in 123 patients with 130 tumors. There were 75 enchondromas and 55 chondrosarcomas grade 1. The minimal follow-up period for all patients was 2 years (range 24-119 months). RESULTS: During follow-up one local recurrence of an active enchondroma and one local recurrence of an aggressive enchondroma occurred. They were treated with curettage and cryosurgery again. Both patients were disease-free at minimum of 3 years follow-up. No local recurrences after treatment of chondrosarcoma grade 1 were seen. Functional scores, according to the MSTS scoring system, showed an average score of 28 points (94%) at 2 years follow-up. Post-operative fractures were seen in 18 patients (14%). CONCLUSIONS: Curettage and cryosurgery for enchondroma and chondrosarcoma grade 1 has excellent oncological and functional results. The post-operative management has been adjusted to reduce the number of fractures.

Was isolation of Veillonella from spinal osteomyelitis possible due to poor tissue perfusion?

From a needle biopsy of the body of the 12th thoracic vertebra in a 74-year-old man with spinal osteomyelitis a Veillonella parvula was isolated. The significance of this bacterium as lactic acid indicator has been considered and discussed. Since this strictly anaerobic bacterium mainly uses lactic acid for energy generation, lactic acid must continuously and sufficiently have been produced in or near to the vertebrate body to permit outgrowth of this bacterium. Since neither microbial infections nor tumours could be demonstrated, we finally hypothesised that in this patient poor tissue perfusion has been the primary cause of lactic acid production.

Chondrosarcoma of bone: functional outcome and quality of life.

BACKGROUND AND OBJECTIVES: Outcome studies of chondrosarcoma have so far only reported oncological and functional results. Quality-of-life assessment becomes more important because more long-term survival after treatment of malignant diseases occur. The objective of this study is to analyze functional outcome and quality of life. METHODS: Functional evaluation of the patients was carried out according to the MSTS functional scoring system. For assessment of quality of life, questionnaires were used. RESULTS: Response of the questionnaires sent to 45 disease-free patients was 84%. The mean functional score of these 38 patients was 74% (20-100%). Best functional results were seen after bone graft reconstruction and curettage and cryosurgery with reconstruction. Quality-of-life analysis revealed a mean global health status of 75 (66-84). Furthermore, 24% of the patients experienced severe fatigue. Patients reported more problems with regard to physical functioning and sleep in comparison with healthy controls. No correlation was found between global health status scores and functional scores. CONCLUSIONS: Functional scores depend on the type of treatment with best results after curettage and cryosurgery. Quality-of-life analysis reveals problems on several domains, including fatigue, physical functioning and sleep.

Fibrous dysplasia of bone: management and outcome of 20 cases.

BACKGROUND AND OBJECTIVES: Fibrous dysplasia of bone is difficult to manage because of its variable clinical course with many different methods of treatment reported. Therefore we report on our experience. METHODS: We reviewed a series of 20 patients with 32 lesions included. The average age at the time of diagnosis was 32 years for monostotic disease, 26 years for polyostotic disease, and 3 years for McCune-Albright syndrome. The median follow-up period was 6 years. Functional and radiographic outcomes were scored. RESULTS AND CONCLUSIONS: Monostotic disease mostly presented with a circumscribed lesion and monitoring was often sufficient. Symptomatic circumscribed lesions showed satisfactory outcome when treated with curettage, cryosurgery and bone grafting. Lesions of the extended type were most of all seen in polyostotic disease and eventually needed operative treatment. In case of bony deformity, corrective osteotomies and rigid internal fixation were performed in addition to curettage, cryosurgery, and bone grafting. In polyostotic disease, expected outcomes were good, but in McCune-Albright syndrome, results were uniformly poor. J. Surg. Oncol. 2001;76:157-166.

Intraosseous schwannoma (neurilemmoma) of the cervical spine.

PURPOSE: To report on an extremely rare tumour located in the cervical spine, its treatment and result. Review of the literature. PATIENT: Case report of a 38-year-old woman with an intraosseous schwannoma of the cervical spine. RESULTS: After local curettage no evidence for local recurrence at long-term follow-up.

The saddle prosthesis in pelvic primary and secondary musculoskeletal tumors: functional results at several postoperative intervals.

The first purpose of this study was to evaluate the saddle prosthesis in patients with periacetabular tumors in terms of the functional results obtained after several postoperative intervals. The second purpose was to evaluate the complications and how they might be prevented in the future. Functional results according to the MSTS functional rating system were evaluated at several postoperative intervals in 15 patients treated with internal hemipelvectomy and reconstruction with the saddle prosthesis because of periacetabular primary (n = 9) or secondary (n = 6) malignancies. All complications were evaluated. Three months postoperatively, 7/9 patients with a primary tumor and 2/4 patients with a secondary tumor were able to walk outside without pain. Median functional results 3 and 6 months postoperatively were 40% and 50%, respectively. Deep infection occurred in 4 patients and fracture of the iliac remnant in 2. Heterotopic ossifications along the interpositional component were seen in 5 patients, but they did not negatively influence the functional outcome. Three (relative) contraindications to reconstruction with the saddle prosthesis could be ascertained: osteoporosis, extended involvement of the iliac wing by tumor, and insufficient soft-tissue quality after previous procedures. (Short-term) functional results after reconstruction with the saddle prosthesis are satisfactory if the above-mentioned contraindications are taken into consideration.

Diffuse and localized pigmented villonodular synovitis: evaluation of treatment of 38 patients.

We performed a retrospective study of 38 patients with pigmented villonodular synovitis (PVNS) to evaluate the treatment and functional results. The mean age of the patients was 32 (range 12-72) years at the time of treatment. Three types of PVNS have been identified: localized nodules in 9 patients, diffuse PVNS of the entire synovial membrane in 26, a combination of a diffuse involvement of the synovial membrane and an extra-articular presentation in 1, and extra-articular lesions in 2. The location of the lesions was knee (n = 31), hip (n = 3), ankle (n = 2), femoral triangle (n = 1), and gluteal region (n = 1). The procedures performed were surgery alone, surgery combined with radiosynovectomy and radiosynovectomy only. A follow-up was done after a mean of 4 (range 1-19) years in 34 patients. A functional evaluation according to the Musculoskeletal Tumor Society was performed in 31 patients. The mean functional evaluation score of 34 patients was 24 (range 15-30). Most of the ratings were excellent or good, in 6 cases fair, and in 2 cases poor. This study demonstrates that the functional results are good despite residual or recurrent disease; in addition, we showed that functional evaluation after treatment gives an optimal view of the impact and results of the operation.

Osteosarcoma: oncologic and functional results. A single institutional report covering 22 years.

BACKGROUND AND OBJECTIVES: The oncologic and functional results in patients treated because of osteosarcoma (OS) were evaluated. METHODS: Fifty-one patients with high-grade OS were treated between 1974 and 1996 at our hospital. All patient records were studied, and the surviving patients were evaluated according to the American Musculoskeletal Tumor Society functional rating system. The majority of patients received adjuvant chemotherapy (prior to 1983) or neoadjuvant chemotherapy (from 1983). Until 1987, all patients with extremity OS had ablative surgery; from 1987, the majority had limb-saving surgery. Lung metastases were resected in most cases. RESULTS: Overall 2-year and 5-year disease-free survival (DFS) rates were 27 of 51 and 16 of 42, respectively. Patients with vertebral or pelvic OS or contaminated margins after resection had a very bad outcome. In all other subgroups, including patients with various types of chemotherapy, response to chemotherapy, diameter of tumor, presence or absence of metastatic spread, and location of tumor, a 5-year DFS of about 50% was found. Recurrent disease in patients who had achieved a 2-year disease-free interval was relatively low (4/23 patients). CONCLUSIONS: Survival in our series was worse than in most other studies. A very bad outcome was found in patients with vertebral or pelvic OS or with contaminated margins after resection.

Intraoperative lymphatic mapping and the sentinel node concept in colorectal carcinoma.

BACKGROUND: Orderly progression of nodal metastases has been described for melanoma and breast cancer. The first draining lymph node, the sentinel node, is also the first to contain metastases and accurately predicts nodal status. The aim of this study was to assess the feasibility of lymphatic mapping and sentinel node biopsy in colorectal cancer. METHODS: In 50 patients with colorectal cancer patent blue dye was injected around the tumour. After resection of the tumour the specimen was examined to identify blue-stained lymph nodes. Routine histopathological examination was performed on all nodes and the blue, haematoxylin and eosin-stained tumour-negative nodes were tested immunohistochemically. RESULTS: Lymphatic mapping was possible in 35 of 50 patients (70 per cent). Pathological examination with haematoxylin and eosin staining showed lymph node metastases in 20 of 35 patients. In eight of these 20 patients the blue nodes showed tumour, while in 12 the blue nodes were not involved. This represents a false-negative rate of 60 per cent. CONCLUSION: Lymphatic mapping using patent blue dye is feasible in colorectal cancer. The blue-stained nodes do not predict nodal status of the remaining lymph nodes in the resected specimen. The concept of lymphatic mapping and sentinel node identification is not valid for colorectal cancer.

Ring chromosomes in a malignant mesenchymoma.

We report, for the first time, the cytogenetic and molecular genetic constitution of a human mesenchymoma. As in several other soft tissue sarcomas, supernumerary ring and rod-shaped marker chromosomes were observed next to an otherwise normal diploid karyotype. Comparative genomic in situ hybridization and whole chromosome painting experiments revealed that chromosome 1q21-q25 and 12q14-q15 sequences were amplified, and that these sequences resided on the supernumerary marker chromosomes. We assume that, in this malignant mesenchymoma, the observed chromosomal anomalies may be associated with its well differentiated liposarcomatous component.

Eosinophilic granuloma of bone: results of treatment with curettage, cryosurgery, and bone grafting.

Intralesional instillation of steroids is currently the first choice of treatment for eosinophilic granuloma of bone. However, some lesions fail to respond or are unsuitable for injection therapy due location, pending pathologic fracture, and soft tissue intrusion. The authors treated six patients with these lesion characteristics using curettage, cryosurgery, and bone grafting. After a mean follow-up of 34.3 months, all lesions healed completely, although one femoral fracture occurred 8 months after the operation. In selected cases of eosinophilic granuloma of bone, a primary surgical treatment seems feasible. The use of cryosurgery as adjuvant treatment extends the surgical margin and is of value in averting local recurrence.

Malignant fibrous histiocytoma of the stomach during pregnancy: a case report.

In this case report we describe a patient with a primary malignant fibrous histiocytoma of the stomach, diagnosed and operated upon during the sixth month of pregnancy. We stress the importance of a thorough diagnostic examination in cases of severe anaemia during pregnancy. Although very rare, malignancy of the gastrointestinal tract should be taken in consideration. No holding back is justified in diagnostic and therapeutic measurements because of pregnancy. Radiologic examination of the gastrointestinal tract should be replaced by endoscopy.

Synovial sarcoma: oncological and functional results.

AIM: To evaluate the oncological and functional result of the treatment of patients with a synovial sarcoma. This paper gives a retrospective review of 20 patients (15 male and five female) treated for synovial sarcoma at the Nijmegen University Hospital, The Netherlands. METHODS: The median age of the patients was 30 years (range: 14-71, mean 37 years). RESULTS: The tumour locations were: lower extremity in 12 patients; upper extremity in three; pelvic and groin region in four; and the retroperitoneal space in one. Surgical stages according to Enneking (Clin Orthop 1986; 204: 9-24) were IIA in five cases; IIB in seven; and IIIB in eight. The surgical margin was intralesional in three cases; marginal in three; wide in six; and radical in six. In one case the surgical margin could not be assessed and one patient was not operated. One patient developed a recurrent tumour and one developed nodal metastases. Eight patients who did not have metastases at the time of diagnosis developed metastase during follow-up. Fourteen patients died of metastatic disease; one patient died of diabetes; one is alive with disease; and four presently do not have evidence of disease. CONCLUSIONS: The outcome was poor, especially due to the occurrence of pulmonary metastases. The functional result according to the MSTS was 100% in four patients; in one the result was 93% because of the scar and venous insufficiency.

Treatment of benign and low-grade malignant intramedullary chondroid tumours with curettage and cryosurgery.

AIMS: To shed light on the controversy surrounding the methods of evaluating, staging and final treatment of intramedullary chondroid lesions. Controversy particularly exists for enchondroma and low-grade chondrosarcoma located in the extremities, because their accurate distinction is hampered by their radiographical and histological similarity. METHODS: Since 1991 we have treated 22 patients (mean age: 39.6 years) with 26 lesions (three chondroblastomas, 14 enchondromas and nine grade 1 chondrosarcomas) with curettage, cryosurgery and bone grafting. RESULTS: After a mean follow-up of 26 months no recurrences were observed. Complications consisted of two post-operative fractures, one wound infection and one intraoperative venous gas embolism. All bone grafts incorporated, resulting in full weight-bearing capacity and excellent functional results. CONCLUSION: The usefulness of a combination of curettage and cryosurgery as adjuvant therapy is considered to be equal to marginal resection according to orthopaedic oncological principles. The pre-operative assessment of these lesions and cryosurgical technique is described in detail.

Isolation of osteosarcoma-associated amplified DNA sequences using representational difference analysis.

Comparative genomic hybridization analysis of a primary osteosarcoma and its metastasis revealed two regions of DNA amplification, one at 17p11.2-12 and one at 19q12-13. Subsequent representational difference analysis of the primary tumor resulted in the isolation of two distinct tumor-amplified DNA fragments originating from chromosome 19. A YAC clone corresponding to one of the two isolated DNA fragments was used for fluorescence in situ hybridization on normal human lymphocyte metaphases and tumor-derived nuclei. This resulted in the localization of this YAC to 19q12-13.1 and confirmed the amplification status of the isolated fragment in the tumors. The availability of such RDA-isolated sequences may be instrumental in the search for genes relevant for tumor development.

Lipomatous hypertrophy of the interatrial septum: indication for surgery?

A fortuitous finding during open heart surgery of lipomatous hypertrophy of the interatrial septum is described in a 65-year old man with ischaemic heart complaints due to coronary artery disease and with premature ventricular contractions. An incision biopsy confirmed the diagnosis. The choice of treatment of lipomatous hypertrophy of the interatrial septum is controversial. Indications for surgery and surgical techniques are discussed.

Aneurysmal bone cysts treated by curettage, cryotherapy and bone grafting.

We treated 26 patients with 27 aneurysmal bone cysts by curettage and cryotherapy and evaluated local tumour control, complications and functional outcome. The mean follow-up time was 47 months (19 to 154). There was local recurrence in one patient. Two patients developed deep wound infections and one had a postoperative fracture. We compared our results with previous reports in which several different methods of treatment had been used and concluded that curettage with adjuvant cryotherapy had similar results to those of marginal resection, and that no major bony reconstruction was required. We recommend the use of cryotherapy as an adjuvant to the surgical treatment of aneurysmal bone cysts. It provides local tumour control. Combination with bone grafting achieved consolidation of the lesion in all our patients.

Unilateral hearing loss due to a rhabdomyoma in a six-year-old child.

A case report of a six-year-old child is presented, who had had a unilateral sensorineural hearing loss for several years. Because of impairment in the ABR as well as in the caloric testing a MRI and CT scan were performed. A 17 mm tumour in the cerebellopontine angle (CPA) was detected, which after suboccipital surgery proved to be a rhabdomyoma. This tumour has not been described before in the CPA. Unilateral sensorineural hearing loss should, at all ages, be an indication for further (radiodiagnostic) investigations.