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Introduction: Extraskeletal myxoid chondrosarcoma is a rare soft tissue tumor included by WHO in the group of tumors of uncertain differentiation because well formed cartilage is rarely seen in its setting; moreover some of the immunophenotype features of the tumor are still controversial. The reciprocal translocation t(9;22)(q22;q12) has recently been identified in many cases, and this chromosomal aberration is currently considered high specific of this tumor., Case Report: We report the case of a 75-year-old female submitted to radical nephrectomy for a voluminous kidney tumor with radiological evidence of extrarenal extension., Results: Grossly the lesion was set in the perirenal fat tissue. Histological examination revealed a malignant mesenchimal tumor with myxoid f eatures and areas of chondroid differentiation consistent with the diagnosis of extraskeletal myxoid chondrosarcoma. According with the diagnosis the tumor showed focal positivity to NSE by immunohistochemistry., Discussion: Differentiated cartilage is seen in extraskeletal myxoid chondrosarcoma in about 30% of the cases. Our case is then interesting not only for the rare site of tumor location but also for the extension of chondroid differentiation areas found on histologic examination in the tumor setting. Moreover immunohistochemistry showed focal positivity of the neoplastic cells for NSE, according with recent studies demonstrating expression of some neural/neuroendocrine markers in extrascheletal myxoid chondrosarcoma.

A type of malignant neoplasm of not frequent observation is reported and the difficulties concerning the distinction from the benign counterpart are underlined, for which a detailed instrumental study is made necessary, integrated with pre and, above all, postoperative histologic analysis. A white male patient of 83, affected by plurirecidivant chondromyxoma of the rib, was admitted for local recurrence. The lesion objectively appeared not dissimilar from the preceding ones, as an ovalar mass fixed on the underlying plains. Radiologic and ultrasonographic examinations seemed to confirm the admission diagnosis; at the end the patient underwent a new intervention. Histologic examination of the lesion yielded a diagnosis of chondromyxosarcoma. The clinical case is made even more interesting by the finding, through the TC technique, preoperatively performed, of a metastatic repetition on the opposite side. The authors examine the problem of the early recognition of a malignant neoplasm developing, in a patient with a clinical history of recidival chondromyxoma. Physical examination and laboratory analysis are not useful in the assessment of the occurring transformation. At molecular level chondromyxosarcoma is characterized by several genomic rearrangements and mutations. Though primitive chondromyxosarcoma often involves the ribs, chondromyxoma's transformation into its malignant counterpart is not a common event. The development of distant metastasis characterizes long term cases. Given the lack of encouraging results about chemo and radio-therapy efficacy in influencing the natural course of the disease, a systematic approach will be made necessary in patient management.

It is the purpose of this study to report our new surgical technique and the outcome of total en bloc spondylectomy (TES) to resect the primary spinal tumor as a whole in order to ensure oncological curability. TES consisting of en bloc laminectomy and en bloc corporectomy was performed on 7 patients with primary vertebral tumors between 1989 and 1993. There were 2 osteosarcomas, 1 malignant fibrous histiocytoma, 1 chondrosarcoma, 1 undifferentiated sarcoma, 1 solitary plasmocytoma, and 1 giant cell tumor. All of the patients had partial or complete pain relief after TES. None of the patients worsened neurologically after surgery and there was no local recurrence following TES, except for 1 patient who died 3 months postsurgery as a result of a tumor developing in the mediastinum. The advantage of TES is en bloc resection of the affected vertebrae by marginal or wide margin, not piecemeal pattern. This new surgical technique offers the most radical therapy of primary malignant tumors.

Laryngeal chondrosarcoma is an uncommon mesenchymal tumor and in the world literature only 220 cases have been reported. We report in this retrospective examination three cases which age ranged from 54 to 65 years; one was a man and two were women. Two of the tumors arose in the thyroid cartilage, one in the cricoid cartilage. All the cases corresponded to low-grade, well-differentiated chondrosarcomas of the hyaline type (grade I) and two of the patients were alive and free from recurrence or metastasis after 6 and 12 years. One had a recurrence 5 years later with a superior histologic grade (grade II) and died 6 months after laryngectomy from metastatic disease. The AA. discusses the importance of assessing location, size, state of the resection borders, histologic grade and recurrence for the correct evaluation of the neoplasia, for therapeutic and prognostic purposes.

Chondrosarcoma are malignant tumour originating in cartilaginous cells which tend to preserve their essentially cartilaginous nature throughout their evolution. They are rare at the level of the maxilla and mandibular. When localized in facial bone, chondrosarcoma follow a more aggressive pattern compared to those localized in long bones. They mainly affect individuals aged between 30 and 60 and show no sexual preference. In the majority of cases, chondrosarcoma have a relatively slow rate of growth. The only valid treatment is radical surgical removal. In overall terms, the prognosis in these patients is not good since the 5-year survival rate is less than 40%. During the period 1980-1992 only 5 cases of chondrosarcoma were diagnosed by the Division of Maxillo-Facial Surgery of the University of Turin. The authors considered it worthwhile to report the last case observed in a premaxillary localization given the advanced age of onset and the typical course of the pathology and treatment received. CASE REPORT. A male 83-year-old patient was referred to use due to a slow growing neoformation in the premaxillary region which had appeared approximately 5-6 months earlier. The patient had previously undergone the avulsion of residual upper teeth due to suspected "toothache". The neoformation filled the premaxilla, deforming the anterior middle portion of the palate and spreading towards the floor of the anterior nasal cavity. The gingiva of the upper alveolar crest presented ulceration with irregular edges and sanious bottom. On palpation the consistency was hard-ligneous and swelling, in continuity with the bone, was slightly painful. Incisional biopsy enabled the diagnosis of chondrosarcoma to be confirmed.(ABSTRACT TRUNCATED AT 250 WORDS)

The authors present a case of extraskeletal myxoid chondrosarcoma of the abdominal wall, an unusual localization and specific presentation. According to the literature, the clinical characters of this kind of tumor, the diagnostic iter and the therapeutic indications are being examined. The actual anatomopathological patterns used for the diagnosis of this neoplasm are being underlined.

The follow-up study of one chondrosarcoma of the hyoid bone is reported; the tumor was a well differentiated grade II chondrosarcoma that relapsed after 10 months and metastasized after 6 years. Fine needle aspiration cytologic method was utilized for the diagnosis of the lung metastases. Cytologic criteria in the differential diagnosis of the chondrosarcoma are discussed. Follow-up of the extra-skeleton chondrosarcoma has to be continued for a long time independently from the grade of differentiation.

The Authors analyze the main surgical techniques for the treatment of malignant sternal neoplasms. Their experience in two cases of sternal chondrosarcoma is reported. Techniques of reconstruction of the chest wall as well as long-term results are described.

Following a general introduction, the paper reports a case of rapidly developing chondrosarcoma of the upper jaw. The biological behaviour of this rare form of neoplasia at a maxillofacial level is briefly discussed together with the outcome.

The Authors report a rare case of mediastinal chondrosarcoma. Diagnosis, cytology and therapeutic problems are underlined.

Primary retroperitoneal tumors (PRT) constitute about 0.02 per cent of all tumors examined in the Department of Pathology of our hospital. We report 3 cases of malignant PRT - yolk-sac tumour, vascular leiomyosarcoma and extraskeletal chondrosarcoma - selected for their histological rarity and clinical features. Recent techniques available for the diagnosis of these neoplasms and surgical treatment are discussed. The role of radio-chemotherapy is also dealt with.

After a brief analysis of the anatomoclinical aspects of chondrosarcoma in general and of the characteristic and particular aspects of the forms with maxillofacial onset (lower age of onset, elective sites of onset anterior in the maxillary and posterior in the mandible, often insignificant standard X-ray pictures, high percentage of error in clinical and histological diagnosis) two cases of peripheral periodontal onset, with low degree of malignity (grade 1), with aspecific clinical and radiological aspects, both locally recurrent several times after nonradical surgery are reported.

The Authors report a new case of chondrosarcoma of the mandible. After stressing the rarity of the tumour in the facial bones, they discuss the treatment problem. They propose a surgical approach as long as metastases are absent and recommend radio and chemotherapy just for palliation of advanced cases.