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Pattern of Relapse in 290 patients with non metastatic Ewing’s sarcoma family tumors treated at a single institution with adjuvant and neoadjuvant chemotherapy between 1972 and 1999
Sarcomatoid carcinoma of the kidney
Among 315 cases of parenchymal renal carcinoma resected between 1975 and 1985, 19 cases (6 per cent) of sarcomatoid carcinoma were identified. All patients were symptomatic or had a palpable mass in the flank at hospitalization. Tumor stage generally was advanced at operation and metastases were detected in 8 patients (Robson stage IV), all of whom died after an average postoperative survival of 8 months. In 3 patients there was tumor invasion of the renal vein (Robson stage IIIA), and they died after an average postoperative period of 11 months. Of 4 patients with perinephric fat invasion (Robson stage II) 2 died of cancer after an average survival of 15 months and 2 are alive with no evidence of disease for an average of 73 months postoperatively. In these later 2 cases the sarcomatoid areas constituted less than 5 per cent of the entire tumor and the remaining tumor was low grade carcinoma. In 3 patients adequate followup is not available and 1 was lost to followup. This histological variant of parenchymal cell carcinoma is a high grade malignancy with a poor prognosis. Operative treatment appears to be ineffective in modifying the behavior of the tumor
Aggressive clear cell chondrosarcomas: do distinctive characteristics exist?: a report of 4 cases.
Dedifferentiated parosteal osteosarcoma: the experience of the Rizzoli Institute
BACKGROUND. Dedifferentiated parosteal osteosarcoma (DPOS) is a variant of osteosarcoma in which a high-grade sarcoma coexists with a conventional parosteal osteosarcoma (c-POS), either at presentation (synchronous type) or at the time of recurrence (metachronous type). Only approximately 60 patients have been reported in the literature. The objective of this study was to analyze the clinicopathologic and radiographic features of a relatively large number of patients with DPOS in an attempt to define further the histologic and biologic behavior of this rare entity. METHODS. In a series of 120 patients with parosteal osteosarcoma who were seen at the Rizzoli Institute from 1958 to 2000, the authors identified 29 patients who were diagnosed with DPOS. The authors reviewed the clinical and radiologic features, histologic sections, treatments, and outcomes in this group of patients with DPOS. RESULTS. Twelve patients were male, and 17 patients were female. The patients ranged in age from 15 years to 65 yrs (average, 36 years; median, 32 years). One tumor involved the scapula, one involved the ilium, and another involved the skull. All 26 of the other tumors were located in the long bones (14 in the femur, 5 in the humerus, 3 in the tibia, 3 in the fibula, and 1 in the ulna). In 18 patients, radiographic areas of lucency were seen within an otherwise sclerotic lesion. Histologically, the dedifferentiated component was high-grade osteoblastic osteosarcoma in 14 patients, fibroblastic osteosarcoma in 10 patients, giant cell-rich osteosarcoma in 3 patients, and chondroblastic osteosarcoma in 2 patients. All tumors were Stage IIB, and invasion of the medullary canal was detected in 19 patients (65%). Twenty-eight patients underwent surgery, and 18 of those patients received chemotherapy (5 patients received neoadjuvant chemotherapy, and 13 patients received adjuvant). Nine patients were dead and 20 patients were alive (average follow-up, 107 months; range, from 3 months to 36 years) at the last follow-up. Of the nine patients who died, one patient received no treatment, five patients underwent surgery (with three patients achieving adequate margins) in combination with chemotherapy, and three patients underwent surgery only (with adequate margins achieved). Of the 20 patients who remained alive, 13 patients underwent surgery (with 10 patients achieving adequate margins) in combination with chemotherapy, whereas 7 patients underwent surgery only (all with adequate margins). Seven of the nine patients who died had widespread metastases. One patient died of causes unrelated to the tumor, and another patient died shortly after undergoing resection of a lesion in the skull. CONCLUSIONS. Dedifferentiation occurred in approximately 24% of patients with c-POS. The prognosis for patients with DPOS was better than the prognosis for patients with dedifferentiated central and dedifferentiated peripheral chondrosarcoma. © 2005 American Cancer Society
Dedifferentiated chondrosarcomas arising in preexisting osteochondromas
Background: Dedifferentiated chondrosarcomas that arise in osteochondromas are extremely rare lesions for which very little information on treatment and outcome is available in the literature. The purpose of the present study was to describe the specific clinical, radiographic, and histologic features of this lesion and to evaluate the oncologic outcome after different treatment strategies. Methods: We reviewed the files of the Rizzoli Institute between 1970 and 2002 and identified eighteen patients for whom adequate records and histologic images were available and in whom a high-grade sarcoma had been diagnosed at the same location as a preexisting osteochondroma. Radiographic studies, histologic slides, and clinical records were reviewed, the features of those studies were tabulated, and prognostic features and the results of treatment were identified. Results: The patients included twelve men and six women with an average age of forty-six years (range, twenty-two to seventy-four years). Eight lesions occurred in patients with multiple osteochondromas, and ten occurred in patients with solitary lesions. The most common locations were the pelvis (six lesions) and the femur (five lesions). Symptoms included pain, swelling, and a growing mass; the average duration of symptoms was eighteen months. Radiographically, ten lesions appeared as a conventional secondary chondrosarcoma arising in an exostosis, whereas eight showed typical signs of dedifferentiation. Histologic evaluation of the cartilage component demonstrated thirteen grade-1 and two grade-2 chondrosarcomas. In three cases, no cartilage component was recognized. The dedifferentiated component was considered to be an osteosarcoma in nine cases (including six cases in which it was osteoblastic and three in which it was fibroblastic), a malignant fibrous histiocytoma in eight, and a fibrosarcoma in one. The dedifferentiated component represented an average of 59% (range, 20% to 100%) of the lesion. For the fifteen patients who were managed at the authors' institution, the two and five-year survival rates were 47% and 29%, respectively; the median survival time was fourteen months. Patients who were managed with a combination of surgery and chemotherapy had a better overall survival rate than did those who were managed with surgery alone (p = 0.03). Conclusions: Dedifferentiated chondrosarcoma arising in a preexisting osteochondroma is an extremely rare lesion with a poor prognosis. In the present small series, overall survival was better when wide surgical resection was combined with adjuvant chemotherapy. Level of Evidence: Therapeutic Level IV. See Instructions to Authors for a complete description of levels of evidence. Copyright © 2007 by the Journal of Bone and Joint Surgery, Incorporated
The temporalis osteomuscular flap versus the free calvarial bone graft. An experimental study in the growing rabbit
The authors have conducted an experimental study on the evolution of temporalis osteomuscular flaps compared with calvarial free bone grafts in young rabbits. The evaluation of the results has been performed with macroscopic, radiological and histological investigations in an early and late postoperative phase. This study has confirmed that the bone flaps remain viable and are characterized by a normal evolution. There is no evidence of anomaly of craniofacial growth nor asymmetry when compared with the contralateral side. On the other hand, the free bone grafts show the typical signs of necrosis and resorption. Some asymmetries are produced in comparison with the contralateral side, these being particularly evident in the two-months samples, while the overall craniofacial growth is not seriously impaired. This experimental study confirms the reliability of the temporalis osteomuscular flap in facial reconstruction. © 1987 Georg Thieme Verlag Stuttgart New York
ANEURYSMAL BONE CYST: A RADIOLUCENT LESION OF THE MANDIBLE. REPORT OF 3 CASES.
Aneurysmal bone cysts (ABCs) are benign bone lesions that may arise de novo in bone or rarely in soft tissue. Typically, there is no definite pre-existing lesion (primary ABC)
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