Benign metastasizing pleomorphic adenoma.
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The normal anatomy of the ascending lumbar veins and a catheterization technique used to demonstrate these veins at phlebography are reported. The use of this examination when dealing with malignant disease in the abdomen and retroperitoneal region is not warranted, on account of the wide range of anatomic and phlebographic variation.
Tissue sections and records of 56 rats with chordoma, identified in the National Toxicology Program's (NTP) data base of approximately 115,000 rats, were examined to determine morphological characteristics, incidence, and aspects of biological behavior. Chordomas occurred in aged rats, originated predominantly in lumbosacral vertebrae, were highly malignant, occurred three times more often in male versus female rats, and commonly produced bilateral posterior paresis, paralysis, and/or distention of the colon and rectum.
A chordoma was removed from the tail base of a 6.5-year-old ferret (Mustela putorius furo). A nodule was observed in the area of tumor development when the ferret was purchased at 3 months of age. Although the nodule did not enlarge for 2 years, slow, steady growth of the tumor was observed for 4 years before surgical removal. Eight months after removal of the chordoma, the ferret developed 2 cutaneous masses. One was adjacent to the vulva, close to where the chordoma had been removed from, whereas the other was in the nasofacial region. After 4 months of slow growth, both masses were removed and both were histologically and immunohistochemically consistent with chordoma. Over the next 8 weeks, additional masses developed in the facial, maxillary gingival, and scapular regions. Enlargement of the gingival mass caused dysphagia, and the ferret was euthanized. Although a necropsy was not performed, these additional masses had a clinical appearance and texture that was similar to the 2 previously removed cutaneous chordomas. To the authors' knowledge, this is the first report of a ferret coccygeal chordoma that developed close to the base of the tail. Ferret chordomas have been reported previously to metastasize to the subcutis overlying the tumor. However, this is the first report of a ferret chordoma that metastasized to a location distant to the primary site of neoplasm development. Cell proliferation indices did not predict this metastatic behavior. It is hypothesized that the long clinical period before removal may have predisposed this neoplasm to metastasis. Observations from this case suggest that chordomas in ferrets may have metastatic potential and so should be removed promptly.
BACKGROUND: For patients with sarcomas, radiotherapy can be used as neoadjuvant, adjuvant, or primary local therapy, depending on the site and type of sarcoma, the surgical approach, and the efficacy of chemotherapy. METHODS: The authors review the current status of advanced technology radiation therapy in the management of bone and soft tissue sarcoma. RESULTS: Advances in radiotherapy have resulted in improved treatment for bone and soft tissue sarcomas. Intensity-modulated radiation therapy (IMRT) uses modifications in the intensity of the photon-beam from a linear accelerator across the irradiated fields to enhance dose conformation in three dimensions. For proton-beam radiation therapy, the nuclei of hydrogen atoms are accelerated in cyclotrons or synchrotrons, extracted, and transported to treatment rooms where the proton beam undergoes a series of modifications that conform the dose in a particular patient to the tumor target. Brachytherapy and intraoperative radiation therapy have generally been used to treat microscopic residual disease in patients with sarcomas. These technologies deliver dose to tumor cells with irradiation of limited volumes of normal tissue. Patients who may benefit from technically advanced radiotherapy include those with skull base and spine/paraspinal sarcomas, Ewing's sarcoma, and retroperitoneal/extremity sarcomas. CONCLUSIONS: Advances in radiation therapy technology, particularly IMRT, proton-beam or other charged-particle radiation therapy, brachytherapy, and intraoperative radiation therapy, have led to improved treatment for patients with bone and soft tissue sarcomas.
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Thirteen patients with recurrent, previously irradiated tumors of the skull base or spine were reirradiated with 125I sources implanted interstitially using microsurgical or stereotactic techniques. Patients harbored difficult, end-stage recurrences of chordoma, meningioma, malignant meningioma, fibrosarcoma, invasive pituitary adenoma, and malignant schwannoma. In two other patients with malignant meningioma, the dose of external radiation was augmented by implanting 125I sources during the initial operation for excision of the lesion or at a separate surgical procedure after conventional teletherapy. Microsurgical implantation of 125I sources into basal tumors was limited by the difficulties inherent in operating in this region; it is not possible to visualize the entire tumor that requires implantation. Three of five chordomas stabilized or regressed; these patients probably benefited from the procedure. Two patients with recurrent malignant meningiomas had long term remission after interstitial brachytherapy. Interstitial 125I brachytherapy for recurrent tumors at the base of skull or adjacent to the spine can be more successful only if more aggressive surgical exposures of these regions are attempted. Implantation of sources for a "boost" dose, either microsurgically during the initial surgical resection of the lesion before conventional teletherapy or stereotactically after conventional teletherapy, may be a valuable adjunct to external irradiation for the control of potentially devastating tumors (such as chordomas and malignant meningiomas) before they recur with the severe consequences seen in the patients reported here.
Using new 125I brachytherapy techniques, we were able to deliver safely a tumor volume dose of 16,000 rads to a previous irradiated, large, recurrent sacral chordoma by means of the intraoperative interstitial implantation of 229 low activity 125I seeds and 40,000 rads to a previously irradiated, small, recurrent clival chordoma by means of the transnasal needle implantation of two high activity 125I seeds. Iodine-125 brachytherapy was followed by regression of tumor, lessening of symptoms, and bony recalcification in both cases.
In a consecutive series of 71 cases of extrinsic lesions involving the vertebral artery (VA), 51 patients presented with external compression of this vessel. The compressive agents included 34 tumors, 4 osteophytes, 5 fibrous bands, 4 traumatic lesions, 2 neural elements, and 2 infectious processes. The main site was the second portion of the VA (C2-C6) (30 of 51 patients). Compression always induced at least significant stenosis, and in 8 patients caused complete occlusion. The compression was either permanent (44 patients) or intermittent (7 patients). Symptoms were observed in 11 patients, including 2 with permanent deficits. Surgical release of compression was performed each time symptoms could be explained by a reduction in VA flow and also when the compressing agent needed to be removed, as in the cases involving tumors. VA decompression was achieved by direct approach in 37 patients, by reduction and fixation of a traumatic dislocation in 2 patients, and by distal revascularization in 4 patients. Medical treatment or roentgenotherapy was used in the other patients. Results were excellent in all but 2 patients, who died from traumatic and ischemic lesions, respectively. Therefore, it seems important to identify external causes of compression of the VA for two reasons: 1) to suppress symptoms of vertebrobasilar insufficiency when their relation to VA compression is clearly established, and 2) to remove compressive agents like tumors safely while preserving the VA.
Epidural cavernous angiomas are apparently rare lesions. We present an unusual case of chronic lumbar radiculopathy secondary to an epidural cavernous hemangioma extending into the intervertebral foramen. Few such reports have had the benefit of modern neuroimaging techniques to aid in preoperative diagnosis, and, therefore, magnetic resonance imaging of these lesions has not been extensively described. This article discusses the implications of the clinical presentation of the patient and reviews the characteristic magnetic resonance imaging appearance of this lesion garnered from this case and from a review of the literature.
OBJECTIVE: In this study, we evaluated patients' clinical outcome and recurrence rates at long-term follow-up after aggressive microsurgical resection of cranial base chordomas. METHODS: Seventy-four patients with chordomas underwent operations during a 16-year period from 1988 to 2004. The philosophy was to perform complete resection whenever possible and to provide adjuvant radiotherapy for remnants. Staged operations were performed for extensive tumors or if a sizable tumor remnant was noted after the first resection. Patients included primary (previously untreated) and previously operated or irradiated cases. Information was prospectively gathered concerning the patients' neurological condition, Karnofsky Performance Scale score, and tumor status on magnetic resonance imaging scans. RESULTS: There were 47 primarily operated patients (63.5%) and 27 patients (36.5%) who had previously undergone surgery or radiotherapy. A total of 121 procedures were performed in 74 patients. The mean follow-up period was 96 months, with a range of 1 to 198 months. A single stage removal was performed in 41 (55.4%) of the patients and multiple stage removal was performed in 33 (44.5%) of the patients. Gross total removal was accomplished in 53 (71.6%) of the patients, and subtotal resection was accomplished in 21 (28.4%) of the patients. During the follow-up period, 24 (32%) of the patients had no evidence of disease, 37 (50%) of the patients were alive with evidence of disease, 11 (14.8%) of the patients died of disease, and two (2.7%) of the patients died of complications. Recurrence-free survival at 10 years was 31% for the whole group, 42% for the primarily operated patients, and 26% for the reoperation cases (P = 0.0001). The average Karnofsky Performance Scale score was 80 +/- 11.7 preoperatively, 84 +/- 8.9 at the 1-year follow-up, and 86 +/- 12.8 at the last follow-up in surviving patients. No conclusion could be drawn regarding the value of radiotherapy because of the treatment philosophy and the small number of patients. CONCLUSION: Aggressive microsurgical resection of chordomas can be followed by long-term, tumor-free survival with good functional outcome. A more conservative strategy is recommended in reoperation cases, especially after previous radiotherapy, to reduce postoperative complications.
Metastases to the salivary glands from distant neoplasms are unusual, with most reported cases involving the parotid gland. Metastatic deposits in the submandibular gland are extremely rare with bilateral involvement not previously reported. We present the case of a patient with advanced breast carcinoma who has had metachronous involvement of both submandibular glands and review the literature on this subject.
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