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Chordomas and chondrosarcomas involving the cavernous sinus: review of surgical treatment and outcome in 31 patients.

During the last 9 years, 31 patients with chordomas (20 cases) and chondrosarcomas (11 cases) involving the cavernous sinus have been treated using an aggressive surgical approach. On the basis of postoperative magnetic resonance imaging (MRI), 17 patients were considered to have undergone total removal, whereas in the remaining 14 cases the tumor was either subtotally or partially removed. Surgical complications were most commonly encountered among patients who had undergone previous operations. One patient died 3 months after the operation as a result of pulmonary embolism. Significant disability occurred in one patient because of thalamic perforator occlusion and hemorrhage. Recovery of extraocular muscle function was gratifying, and correlated to the preoperative functional level. After a median follow-up of 24 months, three recurrences (21%) occurred among the 14 patients who had undergone incomplete removal. No recurrence was observed among the 17 patients with total resection. This experience shows that gross radical removal of chordomas and chondrosarcomas involving the cavernous sinus can be accomplished with an acceptable surgical morbidity. However, much longer follow-up will be required to determine whether such aggressive surgical treatment results in cure or long-term control of these neoplasms.

Adolescent↗

Tracheal chondrosarcoma with an overview of other tumors of the trachea.

Chondrosarcomas of the trachea are extraordinarily rare tumors, with only four cases documented in the English literature. An additional case is reported with conventional and computed tomography correlation. Because primary tumors of the trachea are such rare neoplasms, a review of the subject is presented to put chondrosarcomas of the trachea into perspective. Malignant tumors are slightly more common than benign tumors of the trachea and occur more frequently in adults. The most common tumor involving the trachea is carcinoma, accounting for 80 to 90% of all cases. This, however, represents only 0.2% of all malignancies of the respiratory tract.

Aged↗

Myxoid chondrosarcoma with a translocation involving chromosomes 9 and 22.

Myxoid chondrosarcoma is an uncommon neoplasm thought to be derived from mesenchymal chondrocytic cells. Although cytogenetic abnormalities have been reported in sarcomas, too few cases have been studied to determine the frequency of nonrandom chromosomal changes in mesenchymal tumors. In this article, we describe a chondrosarcoma with a nonrandom reciprocal translocation t(9;22)(q22;q11). The cellular homologue to the retrovirus transforming gene of simian sarcoma virus is located on chromosome #22, and its possible significance in this case is discussed.

Chondrosarcoma↗

Recurrent breakpoints at 9q31 and 22q12.2 in extraskeletal myxoid chondrosarcoma.

A cytogenetic study of extraskeletal myxoid chondrosarcoma cells revealed a complex t(9;22;15)(q31;q12.2;q25) as a primary chromosome change. A reciprocal translocation involving identical breakpoints on chromosomes #9 and #22 in this tumor has been reported in the literature. We suggest that the breakpoints 9q31 and 22.q12.2 are associated with extraskeletal myxoid chondrosarcoma, a comparatively rare tumor of adulthood.

Aged↗

Chromosome rearrangements at 12q13 in two cases of chondrosarcomas.

We analyzed the karyotypes of two moderately differentiated (grade 2) chondrosarcomas. Case 1 had a reciprocal translocation between chromosomes 6 and 12, t(6;12)(q25;q13) in most of the cells analyzed, as well as trisomies of chromosomes 7, 8, 11, 17, 19, and 21 and tetrasomy of chromosome 19. A reciprocal translocation involving chromosomes 12 and 19, t(12;19)(q13;q13), was noted as a highly clonal abnormality in the other case. Some cells had t(12;19) as the sole chromosome abnormality. Thus, chromosome rearrangements involving the long arm of chromosome 12 at the same region (q13) were commonly identified in the two tumors. These findings suggest that the rearrangements at 12q13 are nonrandom acquired changes that characterize a subgroup of chondrosarcomas.

Adult↗

Cytogenetic study of extraskeletal mesenchymal chondrosarcoma. A case report.

Extraskeletal mesenchymal chondrosarcoma (EMC) is a rare and highly malignant type of chondrosarcoma of soft tissue origin. We performed a cytogenetic study on a patient with EMC. Cytogenetic analysis revealed the tumor karyotype: 48-49,XX, t(4;9)(q23;q22), add(10)(q?26), +16, ?del(19)(p13), +1-2mar[cp12] / 48-50,idem, t(1;20)(q21;q13), +mar[cp6] / 46,XX [7].

Adult↗

Mesenchymal chondrosarcoma: a case report.

Mesenchymal chondrosarcoma is a rare tumor distinctly different from the more common chondrosarcoma. It shows a predilection for the head and neck in both osseous and extraosseous forms. The prognosis for cure is poor, with a high incidence of local recurrence as well as regional and distant metastasis. Treatment is based on radical surgical excision, although combination chemo-therapy has recently shown promise. Additional experience with this tumor is required to define the most efficacious form of treatment.

Adult↗

Isolation and characterization of type IX collagen-proteoglycan from the Swarm rat chondrosarcoma.

Type IX collagen was partially purified from the Swarm rat chondrosarcoma by a series of a conventional salting-out procedures. The preparation was further separated by anion exchange chromatography into an unbound and a bound fraction in an A230 ratio of about 5:1. On sodium dodecyl sulfate-polyacrylamide gel electrophoresis, the bound fraction appeared as a broad band, whose molecular mass ranged from 250 to 270 kDa. Digestion with chondroitinase ABC reduced the apparent molecular mass of the bound fraction to about 250 kDa, a value comparable to the molecular mass of the unbound fraction. Tryptic peptide maps of the protein moieties of unbound and bound forms showed that their molecular structures were basically identical. A monoclonal antibody specific for LMW, one of the pepsin-resistant fragments of the rat sarcoma type IX, reacted with both the unbound and bound fractions. Together the results indicate that the unbound and bound fractions represent a type IX collagen devoid of the chondroitin sulfate chain and its proteoglycan form with covalently bound chondroitin sulfate, respectively. The extent of glycosaminoglycan attachment to type IX collagen molecules in rat chondrosarcoma (about 16%) is quite different from the extents described in chick embryo cartilage (about 80%), chick vitreous humour (100%) and bovine cartilage (less than 5%). Further studies on the neoplastic tissue will offer additional information regarding the biological basis and biological consequences of the glycosaminoglycan attachment to type IX collagen molecules.

Animals↗

Charged particle irradiation of chordoma and chondrosarcoma of the base of skull and cervical spine: the Lawrence Berkeley Laboratory experience.

Forty-five consecutive patients with chordoma or chondrosarcoma at the base of skull or cervical spine were treated at the University of California Lawrence Berkeley Laboratory (UCLBL) and University of California School of Medicine, San Francisco (UCSF) between November 1977 and October 1986. All patients had undergone a subtotal surgical resection. Twenty-three patients were treated definitively with charged particles, 13 patients were treated with photons and particles, and 9 patients were treated for recurrent disease. Total doses ranged from 36 to 80 Gray equivalent (GyE). Thirty-three patients are alive with a minimum followup of 1 year. The actuarial survival and local control for all patients at 5 years is 62% and 59%, respectively. Patients treated for primary disease had a 78% actuarial local control rate at 2 years, whereas the rate for patients with recurrent disease was 33%. Patients with smaller visible tumor volumes (less than 20 cc) had a significantly better local control rate than patients with larger tumor volumes (80% vs 33% actuarial rate at 5 years). Patients with chondrosarcoma had the highest local control rate, as did patients treated with particles alone. Complications included 3 patients with unilateral visual loss, two patients who became blind, and 4 patients with radiation injury to the brainstem.

Actuarial Analysis↗

Probable causes of recurrence in patients with chordoma and chondrosarcoma of the base of skull and cervical spine.

PURPOSE: 141 patients with chordoma and chondrosarcoma of the base of skull and cervical spine were treated with proton and photon irradiation between 1980 and 1989. The local disease was controlled in 111 of these patients. This study reviews the 26 patients who have had their disease recur, and who have evaluable diagnostic studies to examine for probable causes of recurrence. METHODS AND MATERIALS: The histologies of the recurrent tumors were 21 non-chondroid chordomas, two chondroid chordomas, and three chondrosarcomas. The prescribed doses ranged from 67 Cobalt-Gray-Equivalent (CGE) to 72 CGE (average of 69 CGE). Doses to small regions of the tumor were deliberately reduced where they abutted certain normal tissues (brain stem, spinal cord, optic chiasm, and optic nerves) in order to keep these structures at acceptance dose levels. The first study, CT or MR scan, on which there was evidence of increase in tumor was carefully evaluated and that volume transferred to the CT scan on which the treatment plan had been developed. The 3D dose distribution in the region of recurrence was carefully analyzed and a judgement made as to the most probable cause of recurrence. RESULTS: Approximately one quarter (6 of 26) of the cases failed in the prescribed dose region. More than half (15 of 26) failed in regions where tumor dose was limited by normal tissue constraints. Approximately 10% of the patients recurred in the surgical pathway and 10% were judged to be marginal misses. CONCLUSIONS: Overall, 75% of the patients failed in regions receiving less than the prescribed dose. All tumors which failed in the high dose region had volume greater than 75 cc. Patients with cervical spine disease had a higher rate of recurrence (10 or 26) and larger tumors (average volume of 102 cc) than those with base of skull disease (16 of 115) with an average volume of 63 cc.

Adolescent↗

Recurrent chondrosarcoma of the hyoid bone.

Chondrosarcoma is a malignant tumor of cartilaginous origin, and the second most common primary malignant bone tumor in the United States. Only approximately 10% of chondrosarcomas occur in the head and neck region, and of these the hyoid bone is a rare site of involvement. This report describes such a case, as well as the radiographic features as seen by CT scan.

Aged↗

Well-differentiated extraosseous chondrosarcoma of the foot with pulmonary metastases.

We report a case of a 38-year-old man with a well-differentiated extraosseous chondrosarcoma of the foot. This case is unusual because the tumor originated in the soft tissues of the foot and developed pulmonary metastases. It is also interesting to note that at the time of recurrence 8 years later, the lesion invaded the third metatarsophalangeal joint and adjacent bones. Well-differentiated chondrosarcoma resembling hyaline cartilage, primary in soft tissue, is a rare histological type, and only a few cases have been reported in the foot. The patient is alive and well 4.5 years after resection of the foot tumor and partial resection of the lung metastases.

Adult↗

[Costal chondroma and chondrosarcoma].

The aim of this study was to report two cases of chondrosarcoma located on the chest wall, in order to emphasize the difficulty encountered by the pathologist to differentiate a chondrosarcoma from a chondroma and the importance, in our opinion, of performing a large resection with wide margins in all cases.

Adolescent↗

Chondrosarcoma of the jaws in Ibadan, Nigeria--a report of four cases.

Four cases of chondrosarcoma of the jaws seen in our clinics within a period of 15 years are described. In all, the lesions were initially benign-looking making early diagnosis difficult. The histogenesis of chondrosarcoma of the jaws is briefly discussed. In our environment, the prognosis of this disease is very poor.

Adult↗