Diagnosis: Chondrosarcoma with emboli metastatic to lung
Following the open lung biopsy, a radiologic survey of the lower extremities revealed a small lesion in the right hip which showed the calcification pattern typical of chondrosarcoma. In this patient, invasion of local venous structures, intravascular growth of tumor, and subsequent embolization led to obstruction of a large part of the pulmonary arterial vascular bed by tumor.
Tumor embolism to the lung was first described by Schmidt in 1897 in a 37-year-old man with gastric carcinoma. More recently, in an attempt to define the incidence of tumor embolus to the pulmonary arterial vasculature in cancer patients, Winterbauer and colleagues reviewed the autopsies of366 patients with either carcinoma of the breast, stomach, liver, hypernephroma or choriocarcinoma. Ninety-five (26 percent) of the 366 patients had evidence of tumor emboli in the pulmonary arterial vasculature on microscopic sections of lung tissue. Tumor emboli were significant factors in the deaths of 30 patients (8.4 percent) and in ten cases were felt to be the primary cause of death. Despite the frequency of this clinical entity, the authors point out that it is rarely identified prior to death. cialis soft tablets
Chondrosarcoma is a tumor of cartilage which occurs most frequently in the proximal long bones; it has been reported to embolize to the pulmonary arteries. Marcove et al reviewed the experience at Memorial Sloan-Kettering Cancer Center over 40 years and found that of 121 patients with chondrosarcoma of the pelvis or upper femur, 39 had evidence of pulmonary metastases. No mention was made of the frequency of embolization to pulmonary arteries. As noted by Lichtenstein, the spread of aggressive chondrosarcoma may occur by invasion of regional venous channels with intravascular growth, extension of tumor locally, and subsequent embolization. Though this process usually occurs over an extended period of time, it may occasionally occur early and follow a rapid course, as was seen in our patient. Tumor embolus is not the only mechanism of pulmonary involvement in patients with chondrosarcoma. Pulmonary artery invasion may result from direct extension from adjacent bone structures.
Patients with tumor emboli may present with pulmonary hypertension and, if the clinical course is more prolonged, cor pulmonale. Increased pulmonary artery pressures may be caused by either direct vascular occlusion or spread of tumor cells into surrounding lymphatics with compression of alveoli and vessels. Cor pulmonale in patients with tumor emboli has been well documented, although it is infrequently detected pre-mortem. The syndrome is characterized by progressive dyspnea followed by right ventricular failure developing over a two- to eight-week period. The electrocardiogram does not always reveal evidence of right ventricular hypertrophy and patients are generally refractory to routine therapy for congestive heart failure.
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Unfortunately, the available treatments of metastatic chondrosarcoma are ineffective. Trials of radiation therapy and chemotherapy, as well as the therapeutic application of intravenous radioactive isotopes have resulted in uniformly poor outcomes. This was true for our patient who died with progressive respiratory failure one month after diagnosis of her tumor.