Patrizia Bacchini

Chordoma of the Mobile Spine : Fifty Years of Experience

Study Design. A consecutive series of 52 chordomas of the mobile spine observed over a 50-year period includes a retrospective review of 15 cases treated prior to 1991 and a prospective group of 37 cases treated from 1991 to 2002. Objectives. This series reviews epidemiologic issues as well as clinical patterns of spinal chordomas. We attempt to correlate tumor extent, treatment, and outcomes over time. Summary of Background Data. Chordoma is the most frequent primary tumor of the mobile spine. Due to slow growth, both initial symptoms and recurrences after treatment arise later, making it difficult to evaluate the effectiveness of treatment protocols. Methods. A prospective series of 37 cases is compared with a retrospective group of 15 patients observed between 1954 and 1991. In the prospective study, all patients had imaging studies, and oncologic and surgical staging. When en bloc resection was not feasible, intralesional extracapsular excision was combined with radiation therapy. The prospective patients were clinically evaluated and imaged. Patients in the retrospective group were evaluated by chart and available images; of these, only one en bloc resection (intralesional margin) was performed. Survivors were all evaluated clinically and had radiographic studies. Results. Forty-eight patients were available for long-term follow-up. Four died due to post-operative complications, and six due to disease less than 2 years after treatment. Forty-two patients were followed over 2 years; 26 patients had over 5 years follow-up. All patients having radiation alone, intralesional excision, or a combination had recurrences in less than 2 years, and died in some cases after a long survival with symptomatic disease. Intralesional extracapsular excision with radiation had a high rate of recurrence (12 of 16 at average 30 months), but 3 patients are continuously disease-free (CDF) at mean 52 months and 5 are alive with disease at average 69 months (ranging 24 to 146). Twelve of 18 patients having en bloc resection are CDF at average 8 years (48 to 155 months). The remaining 6 recurred and of these 1 died. All of these (6) had been previously treated and/or had en bloc resections with contaminated margins. Conclusions. The only treatment protocol associated with CDF at follow-up longer than 5 years is margin-free en bloc resection.

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 Ewings sarcoma could allow the use of risk-adapted therapeutic strategies of treatment. PATIENTS AND METHODS Data on 359 patients with nonmetastatic Ewings 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 Ewings 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.

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 Ewings sarcoma. PATIENTS AND METHODS The response to chemotherapy was evaluated from the specimens of 118 Ewings 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.

Mesenchymal chondrosarcoma of bone and soft tissues

Mesenchymal chondrosarcoma of bone and soft tissues treated at the Istituto Ortopedico Rizzoli are reviewed. The skeletal locations were prominent in five cases. Only two cases were in the soft tissues. Radiographic picture in the bone shows an aggressive osteolysis, with soft tissues invasion. Histologic picture is the same in bone and soft tissues, and is highly distinctive: islands of well‐differentiated chondrosarcoma embedded in undifferentiated mesenchymal cells with high malignancy characteristics. The cases showed a poor prognosis, and no patient survived more than four years.

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.

Malignancy in giant cell tumor of bone

The term malignant giant cell tumor embraces multiple entities and therefore can be confusing. The goals of the current study were to define the clinicopathologic and histologic features of malignancy in giant cell tumors and to clarify the terminology.

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.

The Istituto Rizzoli-Beretta Experience With Osteosarcoma of the Jaw

Twenty‐eight osteosarcomas (OS) of the jaw were reported. There were 15 male and 13 female patients (age range, 9 to 68 years; mean, 36.9 years); 57.1% of the patients were older than 30 years of age. Swelling was the most frequent symptom; it was reported on an average of 6 months before diagnosis. Most of the tumors of the maxilla (eight patients) occurred in the alveolar ridge (six of eight). In the mandible (20 patients), the body was the preferred site (11 of 20). Radiographically most of the lesions were either lytic and sclerotic or only lytic. Histologically, 12 cases (42.9%) were osteoblastic osteosarcoma, ten (35.8%) were chondroblastic, four (14.3%) were fibroblastic, and two (7%) were round cell OS. Of the 28 cases, three (10.7%) were low grade, and 25 (89.3%) were high grade. Thirteen patients had intralesional surgery, and 13 had marginal surgery as their initial treatment. Recurrence was the rule in the first group, and it was 69% in the second group. Twenty patients (71%) died, and eight are alive. Of these, two are alive with disease. In the six patients who are alive and disease‐free, all but one had marginal to wide surgical margins at the time of the first procedure or wide margins at the time of the recurrences along with chemotherapy or radiation therapy. In the patient in which the surgical margins were marginal, the lesion was small 2.5 × 2 cm. This patient was alive without evidence of disease after 9 years of follow‐up.

Malignant fibrous histiocytoma of bone the experience at the Rizzoli Institute: Report of 90 cases

Ninety cases of malignant fibrous histiocytoma (MFH) of bone are reported. An analysis is presented with regard to sex, age, site, symptoms, radiography, macroscopic, and microscopic features. The staging was assessed according to the following classification: I B 3 cases; II A 11 cases; II B 66 cases; III: 10 cases. Among nonmetastatic patients, 68 had surgical treatment (adequate, 46; inadequate, 22); 8 had radiotherapy, and 3 refused any treatment. Among surgically treated patients, 20 had adjuvant chemotherapy, also. Follow‐up information was obtained in all cases. Overall survival rates were 34% at 5 years, and 28% at 10 years. Surgery alone did not appear to be successful (5‐year survival, 28%). Adjuvant chemotherapy improved the survival rate only in patients who underwent adequate surgery (5‐year survival, 57%). The recurrence rate after surgical treatment was high (31%) with significant differences after inadequate (64%), wide (19%), or radical (6.5%) surgical treatment. Adjuvant chemotherapy was ineffective in preventing local recurreace. Radiation therapy effected a clinical cure in three cases; the authors believe that radiation therapy has to be used only in inoperable tumors.

Solid variant of aneurysmal bone cyst

Background and Methods. Of the 200 cases of ABC in the Rizzoli Institute files, 15 had solid features on both gross and histologic examination. Inasmuch as fibrous proliferation with giant cell and bone production along with fibromyxoid areas and small aneurysmal spaces were found in the solid parts of the aneurysmal bone cyst, a grossly solid and radiographically osteolytic bone lesion with these microscopic features was called a solid aneurysmal bone cyst. Some authors call the same lesion extragnathic giant cell reparative granuloma.