Undifferentiated (embryonal) sarcoma of the liver: pathological findings and long-term survival after complete surgical resection

Article Abstract:

Most liver cancers are carcinomas, cancers which develop from cells descended from the outermost or innermost layers of the embryo. Liver sarcomas, which arise from embryonic mesenchyme, a far less common. In some cases, the liver sarcoma retains the undifferentiated characteristics of embryonic mesodermal cells. Such cancers, embryonal sarcomas of the liver, occur most commonly in children, but may affect adults as well. Undifferentiated liver sarcomas are often thought to have an especially poor prognosis. The authors present four cases, however, which illustrate that a favorable outcome is possible. Indeed, these few cases suggest that if surgical removal of the entire tumor is possible, the chances for survival may be good. The four patients ranged in age from seven to 29 years old. In the case a 29-year-old man, it was found at the time of surgery that the cancer had extended along the common bile duct and had invaded the duodenum. Complete resection of the tumor was not possible; this patient died 13 months after the diagnosis of the sarcoma. In contrast, complete removal of the liver sarcoma was achieved in the other three cases. In only one of those patients was chemotherapy given as an adjuvant to the surgical treatment. Radiation therapy was not given in any of the three cases. All three patients remain alive without evidence of disease at 18, 30, and 144 months after surgery. Microscopic examination of tumor specimens from these patients has provided a clue to a puzzling feature of the cancerous tissue. Undifferentiated liver sarcomas have been found to contain globules, both within cells and without, which stain strongly with the dye eosin. Observations of the present specimens indicated the presence of condensed chromatin in some of these globules. Condensed chromatin is an aggregation of the DNA and proteins which make up the chromosomes; it is an indication of so-called "programmed cell death", the process of apoptosis. In apoptosis, the cells die not because of some outside influence but because they are executing a genetic program to do so. (Consumer Summary produced by Reliance Medical Information, Inc.)

Liver cancer, Tumors, Embryonal, Embryonal tumors

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Ewing's sarcoma: radiographic pattern of healing and bony complications in patients with long-term survival

Article Abstract:

Ewing's sarcoma is a cancer affecting bones, which most commonly occurs in adolescents; it is more common among boys than girls. The disease is treated with both radiation and chemotherapy, and more than half the patients may be expected to live more than five years. Many of these long-term survivors will continue to remain disease-free. A study of long-term survivors of Ewing's sarcoma was conducted to determine the chronic effects that the cancer and its treatment had on the patient's bone. Of a total of 48 patients with Ewing's sarcoma, 22 survived five years or longer. In all cases, healing of the affected bone in response to the tumor and its subsequent destruction occurred slowly. The remodeling of the bone, which occurs in response to injury, took up to two years after treatment and was often incomplete. Bone fractures occurred in 11 cases; in one case, a fracture resulting from the pathological changes in the bone occurred over nine years after initial presentation. In most cases, the fractures occurred within the region of the bone that was exposed to radiation therapy. In four patients with fractures occurring after treatment, symptoms of distant metastatic spread of the cancer appeared. In one case, symptoms of metastatic disease appeared 12 years after initial treatment, and in another case, cancer recurred at the original site after 10 years. Two patients developed osteosarcoma at the site of the original tumor five and six years after their remission. Osteosarcoma is a form of bone cancer and, in these cases, is believed to have resulted from the radiation therapy for Ewing's sarcoma. Neither patient received a radiation dose greater than customary. These observations indicate that progression of disease may occur after long periods of remission in cases of Ewing's sarcoma. Furthermore, physicians should be alert, since a fracture or signs of bone weakness visible on X-rays may indicate the recurrence of cancer. (Consumer Summary produced by Reliance Medical Information, Inc.)

Care and treatment, Prognosis, Ewing's sarcoma

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Neurologic complications in children with soft tissue and osseous sarcoma

Article Abstract:

Tumors may adversely affect the function of the nervous system in a number of different ways. This review of 162 children with sarcomas illustrates the variety of ways and relative frequency in which these tumors cause neurological symptoms. The most common neurological complication, occurring in 11 percent of the patients, was the compression of the spinal cord due to the metastatic spread of cancer. Such metastatic tumors are most often treated with radiation, but 28 percent of the children with spinal cord compression required laminectomy, or the excision of the posterior arch of a vertebra. Metastases to the brain occurred in 7.5 percent of the cases a median of two years after the initial diagnosis. In no case was the brain metastasis the first sign of relapse. The metastases were most often intraparenchymal, that is, within the brain substance itself, but one-third affected the dura mater covering of the brain. Peripheral neuropathy was a common complication, occurring in 10 percent of the patients; in most cases the neuropathy was found to result from tumor growth pressing on a nerve or on a nerve plexus. Unfortunately, some neurological complications can be attributed to the chemotherapeutic treatment of the cancer. About 6.2 percent of the children experienced seizures which could be attributed to electrolyte disturbances following treatment. In addition, 2.5 percent developed symptoms of encephalopathy, including memory deficits, hemiparesis, and in one case, psychosis; these symptoms could be attributed to the neurotoxic effects of high-dose methotrexate. In half the cases, symptoms resolved spontaneously. The clinician should remain aware of the possibility for neurological complications so that they may be prevented or rapidly resolved. (Consumer Summary produced by Reliance Medical Information, Inc.)

Research, Complications and side effects, Cancer in children, Childhood cancer, Neurologic manifestations of general diseases, Neurologic manifestations

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