Primary malignant myoepithelioma of the distal femur.

A 55-year-old male presented with a 1-month history of localized pain caused by an osteolytic and destructive lesion in the right distal femur. Histologically, the tumour consisted of spindle cells intermingled with epithelioid eosinophilic cells arranged in small cords embedded in a hyalinized-to-chondromyxoid stroma. Electron microscopy and immunohistochemistry showed features of myoepithelial differentiation. RT-PCR failed to demonstrate chimeric transcripts of extraskeletal myxoid chondrosarcoma. The final diagnosis was primary malignant myoepithelioma of bone. The patient is alive with lung metastases 13 months after surgery. Primary malignant myoepithelioma of bone is an exceptionally rare neoplasm that should be considered in the differential diagnosis with the more aggressive myxoid spindle cell sarcomas.

Pattern of relapse in 290 patients with nonmetastatic Ewing's sarcoma family tumors treated at a single institution with adjuvant and neoadjuvant chemotherapy between 1972 and 1999.

AIMS: Evaluation of pattern of recurrences of 290 patients with an Ewing's sarcoma family tumor (ESFT), who relapsed after adjuvant or neoadjuvant chemotherapy. METHODS: Retrospective analysis at a median follow-up of 16.6 years (range: 5-32) from the primary therapy. RESULTS: There were 378 recurrences, treated by surgery, and/or chemotherapy, radiotherapy, or only palliative treatments. At the last control 18 patients were alive and free of disease 2.5 to 20 years (median 12.1 year) from the last treatment, 4 were alive with uncontrolled disease, 2 died of second line chemotherapy-related toxicity, and 266 died of the tumor 4 months to 20.5 years from the first relapse (median 3.2 years). The 5-year event free survival after the last relapse and overall survival were 5.1 and 7.9%, respectively, and resulted significantly correlated with the time of first relapse, the site of first metastases, the treatment performed after relapse (all patients presently free of disease had been treated by surgery alone or combined with a second line chemotherapy) and for patients treated with neoadjuvant chemotherapy and locally by surgery, with the histologic response to preoperative chemotherapy. CONCLUSIONS: We confirm that the post-relapse outcome of patients with ESFT who relapse after conventional treatment is very poor. Nonetheless specific subgroups of patients may be cured even after 2 or 3 relapses: patients who relapse 2 or more years after primary treatment, patients who relapse with only lung metastases, and patients whose recurrences can be surgically treated.

Neoadjuvant chemotherapy for peripheral malignant neuroectodermal tumor of bone: recent experience at the istituto rizzoli.

PURPOSE: The results achieved in 44 patients with nonmetastatic peripheral neuroectodermal tumor (PNET) of bone treated with neoadjuvant chemotherapy are reported. PATIENTS AND METHODS: A six-drug regimen of chemotherapy (vincristine, doxorubicin, dactinomycin, cyclophosphamide, ifosfamide, and etoposide) was administered to all patients. Local treatment consisted of surgery in 20 patients, surgery followed by radiotherapy in 13, and radiotherapy only in 11. RESULTS: At a mean follow-up of 4.5 years (range, 2 to 7 years), 23 patients (52%) remain event-free, 20 have relapsed (45%), and one has died of chemotherapy-related toxicity. The 5-year event-free survival and overall survival were 54.2% and 62.7%, respectively. To assess the prognostic significance of neural differentiation in the family of Ewing's sarcoma, these results have been compared with the outcomes of 138 concomitant patients with typical Ewing's sarcoma (TES) who were treated according to the same protocol. Of these, 103 (75%) remained continuously event-free, 34 (24%) relapsed, and one died of chemotherapy-related toxicity. It follows that PNET patients treated with this chemotherapy regimen have a significantly worse prognosis than typical ES patients (5-year event-free survival, 54.2% v 70.6%, P <.012; 5-year overall survival, 62.7% v 78.3%, P <.002). CONCLUSION: The authors conclude that studies into new adjuvant therapy for Ewing's sarcoma modulated according to risk of relapse should also consider neural differentiation as a risk factor.

Prognostic factors in nonmetastatic Ewing's sarcoma of bone treated with adjuvant chemotherapy: analysis of 359 patients at the Istituto Ortopedico Rizzoli.

PURPOSE: The identification of prognostic factors in patients with nonmetastatic Ewing's sarcoma could allow the use of risk-adapted therapeutic strategies of treatment. PATIENTS AND METHODS: Data on 359 patients with nonmetastatic Ewing's sarcoma of bone treated at a single institution between January 1979 and April 1995 were retrospectively considered. The influence of clinical, hematologic, therapeutic, and histologic parameters on event-free survival was assessed. RESULTS: By univariate analysis, the following features were found to be associated with a poor prognosis: male sex (P <.02), age older than 12 years (P <.006), fever (P <.0001), anemia (P <.0025), high serum lactate dehydrogenase (LDH) level (P <.0001), axial location (P <.04), radiation therapy only for local control (P <.009), type of chemotherapy regimen (P <.0001), and poor chemotherapy-induced necrosis (P <.001). After multivariate analysis, the adverse independent prognostic factors were male sex (P <.04), age older than 12 years (P <.001), fever (P <.0002), anemia (P <.02), high serum LDH level (P <.0003), axial location (P <.02), and type of chemotherapy regimen (P <.0003). When the multivariate analysis was restricted to surgically treated patients, the adverse independent prognostic factors were poor chemotherapy-induced necrosis (P <.0001), fever (P <.015), anemia (P <.02), and high serum LDH level (P <.025). CONCLUSION: The prognosis in cases of nonmetastatic Ewing's sarcoma is influenced by many different clinical and hematologic variables, all of which are to be considered when patients are being stratified according to the risk of relapse. In surgically treated patients, the most important prognostic factor is chemotherapy-induced necrosis.

Predictive factors of histologic response to primary chemotherapy in osteosarcoma of the extremity: study of 272 patients preoperatively treated with high-dose methotrexate, doxorubicin, and cisplatin.

PURPOSE: In osteosarcoma of the extremity, a strong correlation between chemotherapy-induced necrosis and prognosis has been reported. The aim of this study was to investigate the possible factors that influence histologic response to primary chemotherapy. PATIENTS AND METHODS: In 272 patients with high-grade osteosarcoma of the extremity preoperatively treated with high-dose methotrexate (HDMTX), cisplatin (CDP), and doxorubicin (ADM), the histologic response to chemotherapy was evaluated and graded as complete (no viable tumor cells) or incomplete (persistence of viable tumor cells). Several factors, such as metastatic disease to the lung at diagnosis, sex, age, site and tumor volume, histologic subtype, serum alkaline phosphatase, lactate dehydrogenase (LDH), and methotrexate (MTX) pharmacokinetics were investigated to test their predictive significance on histologic response. RESULTS: Fifty-one patients with localized disease (20.6%) and none of the 25 patients with metastatic disease at presentation had a complete histologic response (P = .006). After multivariate analysis, performed on patients with localized disease only, MTX serum peak (> or = 700 micromol/L) and histologic subtype were proven to be significant predictive factors of histologic response. A complete response was seen in 28.8% of patients with 700 micromol/L or greater MTX serum levels and in 9.9% of those patients with lower levels (P = .001). The chondroblastic subtype was less responsive (6.1% of complete response), compared with the osteoblastic (16.3%), fibroblastic (33.3%), and telangiectatic (42.3%). CONCLUSION: Patients with metastatic osteosarcoma and localized chondroblastic osteosarcoma have a reduced chemosensitivity to primary chemotherapy with MTX, CDP, and ADM. MTX serum peak significantly influences tumor necrosis. A dose adaptation of MTX is recommended to obtain a serum peak of 700 micromol/L or greater when MTX is infused in 6 hours.

Chemotherapy-induced tumor necrosis as a prognostic factor in localized Ewing's sarcoma of the extremities.

PURPOSE: This study was performed to assess the prognostic value of the proposed histopathologic method to evaluate the response of the primary tumor to preoperative chemotherapy in Ewing's sarcoma. PATIENTS AND METHODS: The response to chemotherapy was evaluated from the specimens of 118 Ewing's sarcoma patients, who were preoperatively treated by chemotherapy alone. Responses were graded I to III (macroscopic viable tumor, microscopic viable tumor, and no viable tumor cells, respectively). Follow-up data were available for all patients, with a mean follow-up duration of 86 months (range, 30 to 158). RESULTS: A statistically highly significant difference was observed in outcome among the three groups of patients. For patients with total necrosis (grade III response), the estimated 5-year disease-free survival rate was 95%, in contrast to 68% for grade II responders and 34% for grade III responders (P < .0001). This difference was also confirmed when any single group was compared with the other groups. Among the parameters tested, patient age and the size of tumor had some prognostic value. CONCLUSION: The proposed histopathologic grading, to evaluate the effect of chemotherapy on the primary tumor, had the strongest correlation to clinical outcome. This method could therefore be used to identify patients with a high risk of recurrent disease. These patients could be randomized to receive alternative postoperative treatments to investigate whether more aggressive therapies will improve outcome.

Neoadjuvant chemotherapy for Ewing's tumour of bone: recent experience at the Rizzoli Orthopaedic Institute.

The results achieved in 157 patients with non-metastatic Ewing's sarcoma of the bone treated at a single institution between 1991 and 1997 according to a new protocol (REN-3) are reported. Induction chemotherapy consisted of two cycles of 'VAC': vincristine (V), doxorubicin (A), cyclophosphamide (C) alternated with one cycle of 'VIAc': V, ifosfamide (I), actinomycin (Ac). After local treatment, patients received three more cycles of VAC, two of VIAc, three cycles of I plus etoposide (E) and two cycles with V, C and Ac. Local treatment was surgery in 53% of patients, surgery+radiotherapy in 25% and radiotherapy only in 22%. With a follow-up ranging between 4 and 10 years (mean: 7 years), 110 patients (70%) remained continuously event-free, 2 patients died of toxicity and 45 patients relapsed: 33 due to metastases and 12 due to local recurrence always associated with metastases. The 5-year event-free survival (EFS) and overall survival (OS) were 71.0 and 76.5% respectively. These results are significantly better that the ones achieved in our previous three studies in which a three-drug VAC regimen (REA-1), and 4-drug VACAc regimen (REA-2 and REN-1) was used, and in our most recent study (REN-2) which was based on a six-drug regimen as in the present study, but where I and Ac were used only after the local treatment. However, since REN-3 surgery was used in a significantly larger number of patients, we cannot say whether the better outcome of this study was due to the use of a six-drug regimen with an early delivery of ifosfamide and actinomycin, or to the wider use of surgery as local treatment or both.

Malignant lymphoma involving the mandible. Clinical, morphologic, and immunohistochemical study of 17 cases.

We present 17 cases of non-Hodgkin's lymphoma of the mandible, collected over a span of 10 years. Clinically, the patients showed a male:female ratio of 1:2.4 and a mean age of 60.6 years. Swelling was the most common symptom. In 11 of 17 patients, the disease presented in stage I. Radiotherapy alone was employed in 11 patients, in conjunction with chemotherapy in 5 patients, and was preceded by surgery in the remaining case. At this writing ten of the subjects are still alive, five died following progression of the disease, and two were lost during follow-up while in complete remission. Histopathology revealed 15 B-cell lymphomas (1 centroblastic/centrocytic, nine centroblastic, one Burkitt's, two immunoblastic, and two lymphoblastic) and two peripheral T-cell lymphomas (pleomorphic). The above diagnoses were confirmed by immunohistochemical analysis with a wide panel of reagents against fixative-resistant molecules. Our data suggest that (a) lymphomas of the mandible are principally represented by high-grade B-cell varieties (88%); (b) primitive T-cell lymphomas (here documented for the first time) have the same incidence in the mandible as they have in lymph nodes; and (c) immunohistochemistry, besides confirming histogenetic interpretations based on morphologic findings, provides the key to the differential diagnosis from malignant large-cell nonlymphoid tumors.

Dedifferentiated clear cell chondrosarcoma.

Dedifferentiation, a change in the histologic character and clinical behavior of a tumor to a more immature and aggressive one, occurs in approximately 11% of all chondrosarcomas. The original lesion is usually a low-grade chondrosarcoma. Clear cell chondrosarcoma is a rare cartilaginous tumor of low-grade malignancy with a preference for the ends of long bones. It is usually curable by resection. Recurrence commonly follows inadequate surgery, and metastases to lung, brain, and bones can develop. However, dedifferentiation has not yet been described in association with clear cell chondrosarcoma. Three patients are described who were initially diagnosed as having clear cell chondrosarcoma of the femur. Two were treated with en bloc resection for a clear cell chondrosarcoma. One of these had an undifferentiated sarcoma in a local recurrence after 6 years. In the second, metastasis of the clear cell chondrosarcoma developed 5(1/2) years after surgery; autopsy revealed undifferentiated sarcoma in the lung, heart, and lumbar spine. The third patient had dedifferentiated clear cell chondrosarcoma at the time of resection following the biopsy diagnosis of clear cell chondrosarcoma. All three died with metastatic disease. These three patients represent three different manifestations of dedifferentiation-at initial diagnosis, at recurrence, and at metastasis. To our knowledge, this is the first description of dedifferentiation occurring in clear cell chondrosarcoma.

Osteosarcoma of the bones of the foot--an easily misdiagnosed malignant tumor.

OBJECTIVE: To evaluate the frequency of osteosarcoma involving the foot and determine the clinical outcome of affected patients. MATERIAL AND METHODS: We retrospectively reviewed the cases of osteosarcoma encountered between 1911 and 1992 at the Rizzoli Orthopedic Institute. In particular, we analyzed whether common clinical, radiographic, or histologic features could lead to a correct diagnosis of osteosarcoma of the foot. RESULTS: The bones of the foot were the primary site of osteosarcomas in 12 cases--0.6% of the entire series of such tumors at our institution during the study period. The mean age of the patients was 33 years. Initial symptoms were usually pain and swelling. Late diagnosis was common; the mean time interval between the first symptoms and diagnosis was 28 months. Misdiagnoses occurred in 6 of the 12 cases (50%): osteoblastoma, chondroblastoma, chondrosarcoma, osteoid osteoma, desmoid fibroma, and osteomyelitis were, respectively, the initial diagnoses. Histologically, 5 of the 12 tumors (42%) were low-grade lesions. Four of the seven patients with a high-grade tumor died of metastatic disease after a mean survival of 50 months. Eight patients are alive with no evidence of disease after a mean follow-up of 162 months. CONCLUSION: When a painful swelling in a bone of the foot is observed, even if numerous benign conditions (such as fractures, infections, or benign bone tumors) are far more likely to occur, osteosarcoma must be ruled out to avoid delays in the treatment. Osteosarcomas of the foot may easily be misdiagnosed, especially because they almost always occur in adults, in contrast to osteosarcomas in general. High-grade tumors of the foot are as aggressive as other osteosarcomas and should be managed accordingly--with use of a safe-margins surgical procedure and chemotherapy.

Prognostic significance of cyclin expression in human osteosarcoma.

To evaluate the distribution of cyclin protein expression, in relation to cell proliferation rate and clinical behavior, an immunohistochemical study was performed on 92 tumor samples of patients with high grade osteosarcoma (OS). A large cyclin A- and cyclin E-positive fraction was found respectively in 59% and 47% of the osteosarcomas, while immunostaining for cyclin D1 was weak or absent in most tumor samples. A positive, statistically significant correlation was found between A and E cyclins and Ki67 expression (p<0.001). Disease-free survival (DFS) analysis included 69 of the 92 patients. A significantly higher probability of metastasis was seen in patients lacking cyclin D1 compared to those in which cyclin D1 was positive (p<0.01). Conversely, patients with >40% of cyclin A-positive cells relapsed more frequently than those with <40% of cyclin A-positive cells (p<0.05). The multivariate analysis demonstrated that cyclin A had a lower predective risk in terms of disease-free survival as opposed to the loss of cyclin D1 that is considered a powerful prognostic factor.

Osteosarcoma of the pelvis.

AIMS: To describe the outcomes of a large number of patients with pelvic osteosarcoma, and to define the guidelines for appropriate treatment. METHODS: We reviewed 60 consecutive patients with primary pelvic high-grade osteosarcoma. The tumour involved the whole hemipelvis in 15 cases, while the most common location was the iliac wing in 29 cases (48.3%): 25 of these adjacent to or passing the sacroiliac joint. RESULTS: Thirty patients underwent surgery; there were 16 hindquarter amputations and 14 internal hemipelvectomies. All the patients who presented with metastasis died of their disease. In 18 cases wide margins were achieved, however, eight patients experienced local recurrence. Of the series, only eight patients are still alive. CONCLUSION: The use of intense chemotherapy and surgical wide margin, hardly seems to achieve local control, however, tumour necrosis was correlated with positive prognosis. When internal hemipelvectomy it is not safe enough, amputation must be considered, particularly for cases with sacrifice of the sciatic nerve roots or for older patients where a shorter surgical procedure can be less risky.

A comparison of methods of loco-regional chemotherapy combined with systemic chemotherapy as neo-adjuvant treatment of osteosarcoma of the extremity.

AIM: Our experience of pre-operative intraarterial (i.a.) vs intravenous (i.v.) infusion of cisplatinum (CDP) in a multiagent neo-adjuvant chemotherapy for osteosarcoma of the extremity is reported. METHODS: Two successive randomized studies were performed. In the first, pre-operatively, CDP i.a. vs CDP i.v. was applied in combination with high-dose methotrexate (HDMTX) and adriamycin (ADM) within a three-drug regimen. In the second, a combination of HDMTX, ADM and IFO, within a four-drug regimen was tested. RESULTS: The rate of responses to chemotherapy (tumour necrosis > or = 90%) was significantly higher (P<0.04) for the 142 patients treated with the four-drug regimen than in the 79 patients treated with a three-drug regimen (76%vs 62%). According to the route of CDP infusion, in the three-drug regimen the rate of responses was significantly higher (P=0.004) in patients treated with i.a. CDP (77%) than in patients treated i.v. (46%); with the four-drug regimen the rate of response was not significantly different in patients treated i.a. (81%) and in patients treated i.v. (71%). No significant differences in the rates of limb salvages, local recurrence and event-free survival (EFS) were seen between the i.a. and the i.v. groups. CONCLUSION: In the treatment of osteosarcoma of the extremity, the i.a. infusion of CDP does not offer any significant advantage when this drug is used within an aggressive, multiagent, pre-operative four-drug regimen.

Intra-articular hyaluronic acid in the treatment of osteoarthritis of the knee: clinical and morphological study.

OBJECTIVE: To evaluate, in a pilot, open clinical trial on 40 patients with knee osteoarthritis, the structural changes in the synovial membrane and cartilage following treatment with intra-articular hyaluronic acid (HA-Hyalgan). METHODS: The structural effects of HA given as 5 weekly injections (20 mg/2 ml once a week for 5 weeks), were evaluated by microarthroscopy and morphological analysis of biopsy samples taken at baseline and after 6 months, under blind conditions. Clinical efficacy was also evaluated using visual analogue scales for pain and functional parameters. RESULTS: At 6 months, the microarthroscopic evaluation indicated that the majority of the patients (60%) showed no changes compared to baseline, while 32.5% of the patients showed improvement in the grading and/or extension of cartilage lesions and 7.5% showed a worsened condition. These changes were accompanied by a statistically significant reduction in the synovial inflammation (p = 0.001). The results were confirmed by morphological examination of the cartilage and synovial membrane. At 6 months compared to baseline, a statistically significant reconstitution of the superficial amorphous layer of the cartilage (p = 0.0039), an improvement in the chondrocyte density (p = 0.0023) and vitality (p = 0.05), and a statistically significant reduction in synovial inflammation (p = 0.0001) accompanied by a significant increase in the synovial repair process (p = 0.0001) were observed. Significant and long lasting improvement in pain and joint mobility were also seen after HA treatment. Joint effusion, when present, was reduced. The treatment was well tolerated. CONCLUSION: Hyalgan represents a useful therapy for knee OA, with long-lasting symptomatic efficacy and potential positive effects on joint tissues. Other studies, in particular placebo-controlled studies, are warranted to confirm these promising results observed on joint tissues.

Periosteal chondroma. A review of twenty cases.

We are reporting on the cases of twenty patients with periosteal chondroma to stress the importance of a proper clinical and radiographic diagnosis of this lesion. Awareness of the features of the lesion helps to prevent overtreatment of this benign condition, because the cytological findings may be ominous. Even the radiographic pattern may be suggestive of malignant disease. Periosteal chondromas apparently arise from under the periosteum of the diaphysis or metaphysis in adolescents and young adults. Close cooperation between the surgeon, the radiologist, and the pathologist is necessary to achieve proper diagnosis and treatment. Marginal excision is usually effective treatment.

Dedifferentiated chondrosarcoma.

The cases of forty-six patients who had dedifferentiated chondrosarcoma were reviewed. Two groups were identified: one in which a low-grade malignant chondrosarcoma was the precursor lesion and one in which a moderate to high-grade malignant chondrosarcoma was the precursor lesion. The radiographic features of these lesions ranged from that of a cartilaginous lesion that appeared to be benign to that of a destructive osteolytic tumor in which the cartilaginous component was overshadowed by the dedifferentiated component. Only three of the forty-six patients survived for more than two years. Resection alone, even when it was wide or radical, was not successful in controlling this lethal sarcoma.

Benign fibrous histiocytoma of bone.

The cases of seven patients who had a lytic lesion that was histologically similar to a metaphyseal fibrous defect (non-ossifying fibroma) of bone were studied. The patients all were adults and had pain without a fracture. These features were considered distinctive for the lesion, which has the same histological appearance as benign fibrous histiocytoma of soft tissue. The lesion is a benign tumor with fibroblastic and histiocytic differentiation. This picture may be seen in foci in other lesions of bone (aneurysmal bone cyst, fibrous dysplasia, and giant-cell tumor). Ten cases of giant-cell tumor of bone that had a large component of the same foci were also reviewed. It should be emphasized that these areas are secondary reactive tissue rather than the true neoplastic tissue of benign fibrous histiocytoma.

Classification of bone tumors.

The classification of bone tumors relies on the cytologic features and products of tumor cells. This classification is reproducible and accepted by pathologists, oncologic surgeons and oncologists. Chondrogenic tumors are the second largest group of bone tumors. Their histologic pattern suggests a relationship to hyaline cartilage. Exostoses, or osteochondromas, represent about 1/3 of chondrogenic lesions. Chondromas are hyaline cartilage tumors which can be found centrally or subperiosteally; they may contain some calcifications and/or ossifications. Chondroblastomas are tumors whose cells produce, at least focally, a matrix similar to hyaline cartilage. Histology of chondromyxoid fibromas shows large or small areas where proliferating cells produce a matrix resembling the hyaline cartilage. Chondosarcomas are tumors whose malignant cells produce a cartilaginous matrix. Most of them occur in previously normal bones; they are classified as conventional or primary chondrosarcomas. Secondary chondrosarcomas result from the malignant transformation of a benign cartilaginous lesion less commonly enchondromas and most commonly osteocartilaginous exostoses, or osteochondromas. Less common variants include dedifferentiated, mesenchymal and clear cell chondrosarcomas. Osteogenic tumors are the third largest group of bone tumors, with osteosarcomas being the most frequent type. The most important criterion for a tumor to be considered an osteosarcoma is that the malignant tumor cells must produce a recognizable osteoid matrix, at least focally. Osteosarcomas are divided into three groups: osteoblastic, chondroblastic and fibroblastic, according to the dominant histologic feature. Osteosarcomas can be multifocal, synchronous or metachronous; they are also classified by the histologic grade of malignancy. Pathologically low grade lesions, which are clinically indolent, are generally known as low grade central and parosteal osteosarcomas.

Chondrosarcoma of the mobile spine: report on 22 cases.

STUDY DESIGN: A retrospective review of 22 cases of chondrosarcoma arising from the mobile spine. OBJECTIVE: To evaluate the role of oncologic and surgical staging in correlating management and outcome of chondrosarcoma involving the spine. SUMMARY OF BACKGROUND DATA: Approximately 10% of chondrosarcomas arise from the mobile spine, occurring mainly in adults, particularly elderly men. The course of the disease depends on the aggressiveness of the tumor, but also is influenced by the management. Intralesional surgery is followed almost constantly by local recurrence even with adjuvant therapy. METHODS: All charts, radiographs, and images were reviewed. The composite information provided by this review allowed for oncologic and surgical staging of the reviewed cases. According to Enneking criteria, the surgical procedures were defined as curettage (piecemeal excision) or en blocexcision. The margins were submitted to histologic study and reported as intralesional, marginal, or wide. - As primary management, 10 intralesional curettages (follow-up period, 2-119 months; average, 61 months) and 12 en bloc excisions (follow-up period, 39-207 months; average, 97 months) were performed. A total of 33 procedures were performed, including the management of the recurrences (18 curettages and 15 en bloc excisions: one for soft tissue recurrence). A clinical and radiographic follow-up period of of 2 to 236 months (average, 81 months; minimal follow-up period for survivors, 30 months; average follow-up period for survivors, 115 months) was available for all the patients. RESULTS: Three recurrences occurred in 14 patients treated by en bloc excision at onset or for recurrence, two in cases of histologically proven contaminated or intralesional margins. All but one patient were alive at final follow-up evaluation. Conversely, all the patients treated by one or more curettages (with or without adjuvant radiation therapy) had at least one recurrence, and 8 of 10 of these patients died of the disease. At final follow-up evaluation, nine patients had died of the disease; nine were continuously disease free (but one had died of another unrelated malignancy); and four were symptom free after management for recurrences (one was found alive 155 months after a soft-tissue metastasis en-bloc excision). CONCLUSIONS: En bloc excision, with wide or marginal histologic margins, is the suggested management for chondrosarcomas of the spine. Early diagnosis and careful surgical staging and planning are necessary for conducting adequate management. However, tumor contamination of the specimen margins, even in a small area, or spreading of the tumor myxoid content can worsen the prognosis.

Desmoplastic fibroma of bone. A report of six cases.

The clinical and pathological features of six cases of desmoplastic fibroma of bone are presented. Desmoplastic fibroma is rarely seen as a primary tumour of bone; when it does occur the sites of predilection are the long bones, but other sites such as the scapula and os calcis can be involved. Radiographically the lesion tends to expand the bone from within; it is well-demarcated and lytic, often with a trabeculated soap-bubble appearance. The cellular structure and the morphological arrangement are similar to those of aggressive fibromatosis of soft tissues. Differential diagnosis from malignant spindle-cell lesions of bone is important because the treatment of choice for desmoplastic fibroma of bone is simply excision with a thin layer of healthy tissue.