Periosteal chondroma at birth.

Periosteal chondroma is a slow-growing, cartilaginous, surface tumor that usually occurs in the second and third decades of life. The youngest reported age at diagnosis is 5 years. Marginal excision is the treatment of choice. We report a case of a periosteal chondroma noted at birth and treated conservatively. This report expands the age range of periosteal chondroma to include neonates and suggests a role for observation in its management.

Pathologic fractures after surgery and radiation for soft tissue tumors.

Twenty pathologic fractures occurred in 285 patients with soft tissue tumors that were treated with radiation therapy and surgery. Twelve of these fractures occurred in 11 patients from a subpopulation of 163 patients with 168 soft tissue tumors of the thigh (155 soft tissue sarcomas and 13 aggressive fibromatosis). The fractures occurred at a mean of 40.5 months after treatment and were not associated with significant trauma. Risk factors associated with the development of fracture included tumor location within the anterior compartment of the thigh, extensive surgical periosteal stripping, and a marginal or intralesional margin of resection. The dose, timing, and fractionation of radiation therapy were not related to the risk of fracture. A high rate of complications was seen with this series, including fracture nonunion (45%) and deep infection (20%). Prophylactic intramedullary fixation of the femur should be considered for patients undergoing resection of large tumors in the anterior compartment of the thigh requiring extensive periosteal stripping and adjuvant radiation therapy.

Limb salvage surgery with vascular reconstruction.

Vascular reconstruction and limb salvage surgery has been the authors' preferred approach when malignancy involves major vessels of the extremities. Treatment of 16 patients involved resection, with vascular grafting in 14 patients and rotationplasty in two patients. The major vessels were surrounded by tumor in six patients, nearly encased in three patients, invaded by tumor in four patients, and widely contaminated by intralesional surgery in three patients. The tumor stage included one Stage IB, 12 Stage IIB, two Stage IIIB sarcomas, and one multiply recurrent carcinoma. The largest average tumor dimension was 9.5 cm, and the length of grafting was 14 cm. Major nerves were sacrificed in eight (50%) patients, flaps or muscle transfers were done in seven (44%), chemotherapy was administered in nine (56%), radiation therapy was used in four (25%), and pulmonary metastasectomy was done in two (12%). At a mean followup of 56 months, 50% (eight of 16) of patients were alive without disease. Local recurrence was 12% (two of 16 patients), and infection was 12% (two of 16 patients). Limb salvage was achieved in 88% (14 of 16 patients), and functional status was judged good or excellent in 81% (13 of 16 patients). The complication rate observed in this subset of patients is significant, yet local control and the incidence of major complications was acceptable. Results observed from this series and data gathered from the literature clearly indicate that patients can avoid amputation, despite malignant involvement of major vessels to their extremities.

The role of radiotherapy and limb-conserving surgery in the management of soft-tissue sarcomas in adults.

The treatment of soft-tissue sarcomas has undergone significant changes over the past several decades. Previously, patients were often treated with surgery alone, which frequently necessitated amputation of the affected extremity. Less extensive, limb-sparing operations combined with adjuvant irradiation are now feasible for most patients without compromising the likelihood of cure.

Megavoltage radiotherapy for aneurysmal bone cysts.

PURPOSE: An aneurysmal bone cyst (ABC) is a rapidly expansile and destructive benign tumor of bone that is usually treated by curettage and bone graft, with or without adjuvant treatment. For recurrent tumors, or tumors for which surgery would result in significant functional morbidity, does radiotherapy (RT) provide a safe and effective alternative for local control? PATIENTS AND METHODS: Nine patients with histologically diagnosed aneurysmal bone cysts without other associated benign or malignant tumors were treated at the University of Florida with megavoltage RT between February 1964 and June 1992. The patients received local radiotherapy doses between 20 and 60 Gy, with 6 patients receiving 26--30 Gy. In 6 patients the diagnosis was made by biopsy alone; 3 underwent intralesional curettage before RT. Minimum follow-up was 20 months; 7 of 9 patients (77%) had follow-up greater than 11 years. RESULTS: No patient experienced a local recurrence (median follow-up, 17 years). One patient required stabilization of the cervical spine 10 months after RT because of dorsal kyphosis from vertebral body collapse. No other significant side effects were experienced, and no patients developed secondary malignancies. Four patients were lost to follow-up: at 20 months, 11.5 years, 17 years, and 20 years after the initiation of treatment, none with any evidence of local recurrence. All of the patients who had significant pain before RT had relief of their symptoms within 2 weeks of completion of therapy. CONCLUSIONS: Using modern-day RT, patients with recurrent or inoperable aneurysmal bone cysts can be treated effectively (with minimal toxicity) using a prescribed tumor dose of 26--30 Gy.

Extra copies of chromosomes 7, 8, 12, 19, and 21 are recurrent in adamantinoma.

Adamantinoma of long bones is a rare neoplasm predominantly involving the tibia. Cytogenetic studies of adamantinoma are few. Cytogenetic or molecular cytogenetic analysis of four adamantinomas, and a review of eleven cases in the literature reveals extra copies of chromosomes 7, 8, 12, 19, and 21 as recurrent in this neoplasm. Adamantinoma may be confused with a variety of primary and metastatic epithelial and mesenchymal neoplasms. Observation of these aneuploidies may be useful in establishing the diagnosis of adamantinoma.

Allograft-allograft healing? Salvage of massive allografts after fracture.

Two cases are presented in which an allograft was inserted to salvage a previous allograft bone reconstruction that had followed resection for osteosarcoma. In each instance an allograft-allograft junction was created and, with time, healed. In the first case, a fracture of a distal femoral osteoarticular allograft was salvaged by adding a second allograft and a total knee arthroplasty. This construct preserved the majority of the allograft and allowed rapid rehabilitation. In the second case, an allograft arthrodesis of the knee was salvaged after fracture by replacing the distal portion of the fractured allograft with a new allograft. Again, an allograft-allograft junction was created and healed within 6 months. Allograft fractures pose challenging reconstructive problems. In these two cases, the addition of more allograft facilitated continued limb salvage and function.

Percutaneous radiofrequency ablation of osteoid osteoma.

Osteoid osteoma is a benign bone tumor. Patients usually require surgical treatment for reliable pain relief. Difficulties with intraoperative localization of the tumor and anatomic locations that carry a high morbidity with en bloc resection complicate open surgery. Various methods have been developed to lessen the invasiveness of surgery including computed tomography-guided percutaneous radiofrequency thermal ablation. Eleven patients in three different centers were evaluated and diagnosed with osteoid osteoma based on typical histories, physical examinations, and imaging studies. All patients were treated with computed tomography-guided percutaneous radiofrequency thermal ablation after medical treatment failed. Excellent pain relief was reported in 10 patients. One patient suffered recurrence of a femoral neck lesion despite an initial 7-month period without pain. Patients were given a questionnaire to quantify the effectiveness of percutaneous radiofrequency ablation in terms of pain relief and return to function. The current study shows that percutaneous radiofrequency thermal ablation provides reliable, excellent pain relief and early return to function with minimal morbidity as compared with traditional open techniques. The authors suggest that this technique be used for all patients with extraspinal osteoid osteomas that are not immediately adjacent to neurovascular structures.

Revision surgery in dermatofibrosarcoma protuberans of the trunk and extremities.

AIMS: Dermatofibrosarcoma protuberans is a rare condition which is frequently misdiagnosed at presentation, resulting in a high incidence of local recurrence due to inadequate resection. The archives of the Department of Orthopaedics at the University of Florida were analysed to investigate the natural history and results of treatment for this tumour. METHODS: Between 1975 and 1996, 35 cases of DFSP were treated at the University of Florida. Of these, one was treated primarily, five were treated for local recurrence, 17 had tumour bed excisions following inadequate primary excisions elsewhere and 12 had tumour bed excisions following inadequate resection of local recurrences elsewhere. The data were analysed to assess the impact of age, gender, duration of symptoms, tumour site and size, surgical margin, number of operations and adjuvant treatments on survival and local recurrence outcomes. RESULTS: Complete follow-up was available for 34 patients. Mean follow-up was 58 months (range 12-144 months). Thirty-three patients remain alive and disease-free. One patient died of unrelated causes. The margins obtained were wide in 28 patients, marginal in six and intralesional in one. Of the seven patients with inadequate surgical margins, four received adjuvant radiation therapy and remain disease-free. No patient with an adequate margin developed a local recurrence, but there were three local recurrences in the patients with an inadequate margin who did not receive adjuvant radiation therapy (local recurrence rate: 8%). No patient developed lymphatic or distant metastasis. Local recurrences were more likely to be classified Stage IB (17/17) than primary tumours (1/18) (P<0.001). Local recurrence was more likely where the surgical margin was less than 2.5 cm from the lesion. CONCLUSIONS: Dermatofibrosarcoma protuberans is a low-grade tumour that has a high potential for local recurrence unless it can be completely excised. The overall rate of local recurrence in referred patients in this series was 20/35 cases (57%). All occurred after inadequate margins at previous surgery in other institutions. Revision surgery in these patients showed a local recurrence rate of 8%. To avoid extensive surgery for recurrences, initial treatment should be by wide excision incorporating the underlying deep fascia and a cuff of 2.5-3 cm of normal skin tissue. Radiation therapy provides a useful adjunct where adequate margins cannot be obtained.

Evaluating marrow margins for resection of osteosarcoma. A modern approach.

Intraoperative evaluation of bone marrow margins by frozen section analysis is a common practice in the surgical treatment of osteogenic sarcoma. The purpose of this study was to assess the clinical use of intraoperative marrow margin evaluation to rule out occult intramedullary tumor extension in osteosarcoma surgery. One hundred twenty-eight consecutive patients with high grade osteosarcoma diagnosed between 1988 and 1996 (Group 1) were reviewed retrospectively and compared with 92 consecutive patients treated from 1979 to 1984 (Group 2). Eighty-five patients in Group 1 met the inclusion criteria of having high grade intramedullary lesions of the long bones observed on preoperative magnetic resonance imaging evaluation of the lesion and intraoperative frozen section analysis of the bone marrow margin. Thirty-three patients in Group 2 met the same inclusion criteria with the exception of having preoperative magnetic resonance imaging. Ninety-two marrow margins in Group 1 and 33 marrow margins in Group 2 were evaluated by frozen section. All 92 marrow margins in patients in Group 1 were negative by frozen section analysis and permanent histologic analysis. Of the 33 marrow margins in patients in Group 2, three (9.1%) were reported positive for tumor. Of these, one was found to be a false positive result on permanent pathologic examination. In addition, one false negative frozen section result was found, which was positive for tumor on permanent pathologic examination. The difference in true positive results of marrow margins between Group 1 and Group 2 was statistically significant. Intraoperative marrow margin evaluation by frozen section is not mandatory with modern imaging techniques. Preoperative evaluation of tumor extent using magnetic resonance imaging and intraoperative evaluation of the specimen by the pathologist (done by bivalving the specimen) are reliable methods to ensure adequate surgical margins in most cases of conventional osteosarcoma of the long bones.

Long-term followup of patients with autogenous resection arthrodesis of the knee.

Arthrodesis of the knee may be indicated for the reconstruction after resection of tumor around the knee. Since the introduction of this technique, resection arthrodesis using segmental autogenous grafts has been the principal method of reconstruction. From August 1967 to February 1985, 73 patients underwent resection arthrodesis using autogenous grafts. All procedures were performed for malignant or potentially malignant lesions. Ten-year followup was available on 40 patients. The reconstructive procedure was performed using an intramedullary rod and hemicortical femoral or tibial grafts with a single autogenous nonvascularized fibula or with dual nonvascularized fibulae. Despite a high surgical complication rate, the majority of patients achieved successful limb salvage. Independent ambulation was achieved by 86% of the patients. A Musculoskeletal Tumor Society functional evaluation in 32 available patients at a mean of 17 years showed the majority of patients functioning satisfactorily. Long-term followup of these patients shows continued durability of the reconstruction and a persistent high level of function and patient satisfaction. Resection arthrodesis using massive autogenous grafts should continue to be in the armamentarium of the orthopaedic oncologist.

Local host response in osteosarcoma after chemotherapy referred to radiographs, CT, tumour necrosis and patient survival.

PURPOSE: The necrotic effect of chemotherapy on primary osteosarcoma has been shown to be predictive of the final outcome. Little attention has been paid to the local response of the host (LHR), which reflects the tumour-host relationship. DESIGN: A four-step grading system was developed based on distinct histological patterns of the LHR around the lesion. These responses were correlated with the chemotherapy-induced necrosis or chemosensitivity and analysed in an attempt to ascertain their influence on the patient prognosis. The ability of conventional radiographs and computed tomography to measure LHR was studied. METHODS: The grading system was applied to macroslides of specimens obtained from 72 patients with stage II B primary osteosarcoma in various limbs after wide resection and complete courses of pre- and postoperative chemotherapy who were treated between 1985 and 1991 with a median follow-up of 5 years and 9 months. The histological specimens were blindly reviewed by two pathologists and two experienced musculoskeletal oncologists to assign a grade of response. The results were correlated with tumour necrosis, patient survival and response features on conventional radiographs and CT images. RESULTS: Significant correlation was found between LHR and tumour necrosis or chemosensitivity (r=0.55) and between LHR and CT response (r=0.56). There was no correlation between LHR and the findings on conventional radiographs. A grade 4 LHR was predictive of long-term survival. CONCLUSIONS: The LHR to preoperative chemotherapy has a prognostic influence on patient survival and can be predicted by CT.

The usefulness of bone scintigraphy with SPECT images for detection of pulmonary metastases from osteosarcoma.

OBJECTIVE: We prospectively compared the ability of two techniques--bone scintigraphy with single-photon emission computed tomography (SPECT) of the chest and CT of the chest--to reveal potential osteosarcoma metastases of the lung. SUBJECTS AND METHODS: Our study included 27 patients with osteosarcoma who prospectively underwent both bone scintigraphy with SPECT of the chest and CT of the chest. The imaging results were compared with outcome or pathologic analysis of any lung lesions found. RESULTS: Eight (30%) of the 27 patients had pulmonary metastases. Four of these eight patients had positive results on both CT studies and bone SPECT studies, with additional lesions detected with bone SPECT in two of these four patients. The other four patients with pulmonary metastases had positive results on CT studies, whereas the results of bone SPECT studies remained negative. The results of bone SPECT studies were negative in the 19 patients without pulmonary metastases. CT, however, showed abnormalities in seven (37%) of the 19 patients, which were eventually attributed to benign conditions. CONCLUSION: Negative results on a bone SPECT study of the chest should not be used to exclude the possibility of lung metastases. However, if the results are positive, a bone SPECT study can be used to confirm abnormalities seen on CT scans and may also reveal subtle lesions missed on CT scans.

Local anesthetic infusion through nerve sheath catheters for analgesia following upper extremity amputation. Clinical report.

BACKGROUND AND OBJECTIVES: Reports about the efficacy of local anesthetic perfusion of nerve stumps following lower extremity amputation are conflicting. We report our experience with this technique following amputation of the upper extremity. METHODS: Six consecutive patients undergoing proximal upper extremity amputations (four forequarter amputations and two shoulder disarticulations) for malignancy were prospectively observed. In all patients, catheters were placed within the amputated nerve sheaths at the conclusion of the procedure. Bupivacaine. 0.25%, was administered through each catheter as a bolus and then as a continuous infusion for at least 72 hours after surgery. Narcotic usage, level of pain as reported verbally, and presence of phantom limb pain during the infusion were recorded. For at least 1 year after operation, data were gathered on the presence of phantom limb pain and its intensity during each follow-up visit. RESULTS: Complete analgesia was achieved in all patients by postoperative day 2. Narcotic usage was low. Three of the six patients reported phantom limb pain during follow-up evaluation. CONCLUSIONS: Continuous local anesthetic perfusion of amputated nerves via a catheter placed under direct vision provided excellent postoperative analgesia. The incidence of phantom limb pain for cancer patients did not differ from that previously reported but was easily managed pharmacologically. The technique may also be efficacious for traumatic amputations.

Arthrodesis after resection of bone tumors.

Advances in chemotherapy and radiographic imaging have allowed resection and limb-salvage surgery to be performed on the majority of patients with bone tumors. Extensive soft tissue resection, extra-articular resection, and social factors often contraindicate a mobile reconstruction of the involved or adjacent joint. In these cases, an arthrodesis often can maintain a functional extremity. Current soft tissue techniques and advances in orthopedic hardware have minimized complications and allowed successful outcomes for the majority of patients. This article reviews resection arthrodeses about the knee, shoulder, wrist, and ankle. The surgical technique, complications, and functional outcomes of these procedures are presented.

Radiotherapy for local control of aggressive fibromatosis.

PURPOSE: To evaluate local control rates in patients treated with radiotherapy for aggressive fibromatosis. METHODS AND MATERIALS: Fifty-three patients with histologically confirmed aggressive fibromatosis were treated with radiotherapy at the University of Florida between march 1975 and June 1992. The minimum length of follow-up was 2 years; 81% of the patients had follow-up for at least 5 years. The lesions arose in an extremity or limb girdle (39 patients), the trunk (10 patients), or the head and neck area (4 patients). Twenty-four patients were treated for gross disease and 29 for presumed microscopic residual disease after one or more operations. Patients were treated with total doses between 35 and 70 Gy; 83% of patients received 50 to 60 Gy. RESULTS: Local control was achieved in 23 of 29 patients (79%) treated postoperatively for microscopic residual disease and in 21 of 24 patients (88%) treated for gross disease; gross disease was controlled in all 8 patients with previously untreated lesions and in 13 of 16 patients treated postoperatively for gross residual or recurrent disease. Overall, aggressive fibromatosis was locally controlled in 83% of treated patients. All nine treatment failures occurred in patients with extremity lesions 4 to 68 months after initiation of treatment. Three recurrences were in the irradiated field, two were out of the field, and four were at the field margin. Eight patients were salvaged with surgery alone or combined with postoperative radiotherapy. A functional limb was maintained in 38 of 39 patients with extremity or limb girdle lesions. Pathologic fracture occurred in three patients; two patients required rod fixation for treatment. CONCLUSIONS: Radiotherapy is a valuable adjunct to surgery in the management of aggressive fibromatosis and can be used alone in patients with unresectable or inoperable disease.

Local control and functional results after twice-daily radiotherapy for Ewing's sarcoma of the extremities.

PURPOSE: Radiotherapy (RT) has been the predominant local treatment for Ewing's sarcoma of bone at the University of Florida. Twice-daily hyperfractionated RT was initiated in 1982 to improve local control and functional outcome. This retrospective review compares the results of once-daily vs. twice-daily RT in patients with primary Ewing's sarcoma of an extremity, with emphasis on functional outcome. METHODS AND MATERIALS: Between June 1971 and January 1990, 37 patients were treated at the University of Florida for nonmetastatic Ewing's sarcoma of bone with a primary lesion in an extremity. Three patients underwent amputation. Of 34 patients treated with RT, 31 had RT alone and 3 had a combination of RT and local excision. Before 1982, 14 patients received once-daily RT; since 1982, 17 patients have received twice-daily RT. Doses of once-daily RT varied from 47 to 61 Gy at 1.8-2 Gy per fraction. Doses of twice-daily RT varied, depending on the response of the soft-tissue component of the tumor to chemotherapy, and ranged from 50.4 to 60 Gy at 1.2 Gy per fraction. Some patients in the twice-daily RT group also received total body irradiation 1-3 months after local RT as part of a conditioning regimen before marrow-ablative therapy with stem cell rescue. They received either 8 Gy in two once-daily fractions or 12 Gy in six twice-daily fractions. The six patients who received surgery were excluded from local control analysis. Local control rates were calculated using the Kaplan-Meier (actuarial) method. Fifteen patients had a formal functional evaluation. RESULTS: In the 31 patients treated with RT alone, the actuarial local control rate at 5 years was 81% for patients treated twice daily and 77% for those treated once daily (p = NS). No posttreatment pathologic fractures occurred in patients treated twice daily, whereas five fractures occurred in those treated once daily (p = 0.01). On functional evaluation, less loss in range of motion (15 degrees vs. 28 degrees of loss, p = 0.02) and a lesser degree of muscle atrophy (8% vs. 21% loss in muscle circumference, p = 0.0004) occurred with twice-daily than with once-daily RT. A trend toward less fibrosis and less local alopecia was seen in patients treated twice daily. Patients treated twice daily received a higher Musculoskeletal Tumor Society functional rating (determined by the Department of Orthopaedics) than those treated once daily (29.4 vs. 26.0, p = 0.15). CONCLUSIONS: Local control rates were similar in the two groups (77% vs. 81%), but functional results were superior in the group treated twice daily.