Surgical management of very large musculoskeletal sarcomas.

The management of almost all sarcomas in the musculoskeletal system includes surgery, although chemotherapy and radiotherapy also may play an important role in the treatment. The surgical treatment comprises complete resection of the tumor with a margin of normal tissue. This can be accomplished by local resection and then reconstruction of the resultant deficit, or amputation. Resection of the tumor without amputation, that is, limb-salvage surgery, can be accomplished in 80% of cases and is generally preferred by the patient. The very large, neglected, or aggressive tumor, however, can make resection with a wide margin impossible or very difficult. Many large tumors will require amputation in order to be sure that the entire tumor is removed, as the priority is saving the patient's life, with limb preservation secondary. Even an amputation may be very difficult when the pelvic or shoulder girdle is involved. Sometimes the surgeon may agree to do a marginal resection when the patient or family absolutely refuses amputation, but they must be made to understand that this may compromise survival. In cases where there is circumferential skin involvement or projected loss, some unusual techniques, such as amputation-replantation can be done, especially in the upper limb, where in spite of gross shortening, function can be much better than any prosthesis. Even if there are metastases present, sometimes amputation or even local resection is offered as palliation if the patient's life is made miserable by a large fungating tumor. Local resection of a huge tumor can be a formidable challenge to the surgeon, and the margin may consist of a few millimeters of compressed muscle. Good skin cover may be a major problem, and free tissue transfer may be necessary if local flaps are not possible. In all cases, one must ask if the resection-reconstruction with all its complications and risk to the patient of recurrence is really better than an amputation.

Medical management of osteoid osteoma.

OBJECTIVE: To see if the results of managing osteoid osteoma with medical treatment alone is comparable to those after surgery or other ablative therapy. DESIGN: A case series. SETTING: A tertiary care centre. PATIENTS: Eleven patients with osteoid osteoma treated over a 5-year period. The condition was diagnosed from a typical history, patient age, standard radiography, computed tomography, bone scanning, complete blood count and measurement of the erythrocyte sedimentation rate. INTERVENTIONS: Continued medical treatment with nonsteroidal anti-inflammatory drugs (NSAIDs) for 6 months after pain ceased. Surgery was done only in those who refused or could not tolerate medical treatment. MAIN RESULTS: Medical treatment successfully controlled the pain in all patients. Two patients decided to undergo surgery because of intolerance to the NSAIDS. In 7 patients the symptoms resolved after a mean time of 2.5 years. Two patients were still taking NSAIDS 5 years from the time of diagnosis. CONCLUSIONS: The natural history of osteoid osteoma is self-limited so patients should be offered nonoperative treatment, reserving ablative treatment for those who are unable or unwilling to take NSAIDs until their symptoms resolve.

Localised Ewing sarcoma/PNET of bone--prognostic factors and international data comparison.

BACKGROUND: To determine if the distribution of prognostic factors accounted for the differences when the outcome for localised Ewing Sarcoma/PNET bone in Saudi Arabia was compared with results from countries with well developed health care systems. PROCEDURE: Retrospective analysis was undertaken of 163 consecutive patients of all ages, treated with radical intent at KFSHRC from 1975 to 1998. Standard chemotherapy was commenced in all patients. The local treatment modality was resection +/- radiation in 30% and radiation treatment alone in 67%. Size data were available for 51 patients treated from 1994 to 1998, inclusive. One third of these patients had tumors with volume >500 ml. RESULTS: Three year survival significantly increased with the year of diagnosis, 1975-1988 45%; 1989-1993 55%; and 1993-1998 63% (P = 0.006). Favorable prognostic factors were age < or =14 (P = 0.07); site, distal extremity, and skull (P = 0.08); and volume < or = 200 ml (P = 0.06). Secondary prognostic factors were response to induction chemotherapy, both histological, 100% necrosis, (P = 0.04) and clinical CR+PR, (P = 0.02). From 1994 to 1998, 3 year survival for tumors in the distal extremity and skull was 80% and for small tumors, < 200 ml, at any site was 82%. In comparison, the 3 year survival for patients with tumors at any other sites was 60%, and for tumors >200 ml, 55%. CONCLUSIONS: Overall survival progressively improved. From 1994 to 1998 the survival of patients with small tumors and/or favorable sites was similar to the best reported results. It was not possible to compare results by tumor size for large tumors, > 500 ml, due to the absence of data from elsewhere. A better staging system is required for the international comparison of results.

Malignant fibrous histiocytoma: a retrospective study of 109 cases.

The purpose of this report is to assess the prognostic factors that could influence management and clinical outcome of malignant fibrous histiocytoma (MFH) of soft tissues. Between 1975 and 1998, 109 patients diagnosed with MFH of the soft tissues, seen at King Faisal Specialist Hospital and Research Center, have been reviewed. Of the 109 patients, 75 were men and 34 were women. The median age at presentation was 48 years (range: 3-94). Seven patients (6%) had regional nodal disease and 10 other patients (9%) with distant metastases were excluded from survival analysis. The remaining 92 patients had localized disease and had surgery as the primary treatment modality with or without radiotherapy and/or chemotherapy. Extremities were the most common location (58%). Tumors less than 5 cm represented 32%, whereas 68% had tumors 5 cm or more. Low-grade tumors constituted 46%, and the remaining 54% were high grade. Thirty-seven percent of patients had positive surgical margins histologically after complete gross resection. The 5- and 10-year relapse-free survival (RFS) rates were 39% and 36%, respectively. Isolated local recurrence occurred in 20 patients (22%), isolated metastatic disease without local recurrence in 9 patients (10%), and combined local and metastatic disease occurred in 20 patients (22%). The overall 5- and 10-year overall survival (OS) rates were 50% and 43%, respectively. On multivariate analysis, tumor size and radiation dose were significant factors for RFS (p = 0.04 and 0.0005, respectively). In terms of OS, size, histologic grade, and surgical margins were significant factors on multivariate analysis (p = 0.001. 0.006, and 0.0001, respectively). Complete surgical resection at the time of primary tumor presentation is likely to afford the best chance for RFS and OS. Radiation therapy plays an important role, in combination with surgery for better local control, particularly in high-grade lesions, and in cases with positive surgical margins after wide complete gross excision. The role of adjuvant chemotherapy remains investigational.