Rhabdomyosarcoma of the bladder and prostate in children.
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Biomedical subjects
Publications and source records attributed to W Lawrence.
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Two hundred forty-eight patients with primary epidermoid carcinoma of the oral cavity, oropharynx, and hypopharynx, stages II, III, and IV were entered into a prospective randomized clinical trial of preoperative irradiation therapy (700 rads X 2) and surgery versus surgery alone. At 5 years both groups had a similar survival when analyzed according to stage of disease, primary site, or lymph node status. However, the group of patients receiving preoperative irradiation showed a lower incidence of local recurrence (22% versus 36%) preoperative irradiation showed a lower incidence of local recurrence (22% versus 36%) (P = 0.02). From this study we conclude that preoperative irradiation in this dose schedule has little influence on the ultimate outcome after surgical treatment of head and neck cancer.
The Intergroup Rhabdomyosarcoma Study, initiated in 1972, has admitted more than 700 patients with childhood rhabdomyosarcoma in a 6-year period. Although it was designed primarily to answer specific questions about various aspects of radiotherapy and chemotherapy, some lessons have been learned concerning the surgical approach to this disease. Operative resection, which is not always feasible, is determined chiefly by the clinical stage and anatomic site. Thus far, data for some anatomic sites indicated that total gross resection at some point in the treatment schedule achieved better results than did incomplete resection. A possible exception to this occurred when the orbit is the primary site. The incidence of lymphatic metastases from lesions in some anatomic sites (e.g., paratesticular, pelvic genito-urinary, and extremity) was higher than was previously appreciated. This finding will influence decisions concerning surgical biopsy or dissection of regional lymph nodes for many of these lesions. Differences in histology, particularly those relating to the alveolar type, indicated the need for a more aggressive approach to selected lesions, but we need more data to confirm this determination. Lastly, patients who initially received radiotherapy or chemotherapy to pelvic sites have had striking benefits, and this finding should encourage the development of schedules that specify operative resection of these lesions later in the program rather than as the initial treatment.
The characteristics of 554 evaluated patients entered into a clinical trial conducted by the Pediatric Intergroup Rhabdomyosarcoma Committee between November 1972 and September 1978 were examined for their relationship to prognosis. Prognosis was defined as disease-free time and overall survival time in clinical groups I and II and time on study and survival time in clinical groups III and IV; all times were measured from the start of treatment. The percentage of patients surviving 2 years differed significantly among the clinical groups: I, 92; II, 78; III, 64; and IV, 35. The percentage of patients free of disease at 2 years was significantly higher in group I than in group II (83 vs. 72%, respectively); P = 0.02. The patient characteristics of group I most related to disease-free and overall survival were histologic cell type (alveolar, unfavorable), lymphocyte count (low count, unfavorable), and primary site (disease in extremities, unfavorable). In group II, sex (male, favorable) and lymphocyte count (low count, unfavorable) were significantly related to disease-free and overall survival times. Patients in the clinical subgroup with both microscopic residual disease and lymph node metastasis had poorer survival than patients in other subgroups. Primary site of disease was the only characteristic of group III related to length of time on study and to survival. Orbit and the genitourinary system were favorable primary sites, whereas the retroperitoneal area and extremities were unfavorable. In group IV, primary site (genitourinary, favorable) was related to length of time on study and survival. Sex (male, favorable) was related to survival experience.
Fifty-eight children with genitourinary rhabdomyosarcoma are reported. Lesions involved the bladder (22), prostate (14), vagina/uterus (6), and paratesticular tissues (16). Fifteen of 58 had positive sampling of regional lymph nodes. Eleven of 15 received radiation to no more than 4500 rad in most cases, and 9 of 11 are diseases free. Two of 15 had no radiation and are disease free also. Twenty-three of 58 had negative nodes. Six of 23 had radiation to these nodal areas and 4 of 6 are disease free. Fifteen of 17 patients had no radiation and are disease free also. Sixteen of 20 patients with no node sampling are disease free; 9 of 16 had radiation but 7 of 16 did not. All patients in the Study had intensive systemic maintenance chemotherapy. One 1 patient failed in regional nodes despite 4500 rad to these node echelons. We suggest chemotherapy and radiotherapy, not to exceed 3500 rad in four weeks, to known residual disease including regions from which positive nodes have been obtained.
A combined intracranial facial approach adopted for head and neck neoplasms extending to or invading the base of the skull is capable of achieving long-term control of disease that has not been effectively treated by conventional resection. This is partially true for advanced lesions originating in the skin and the soft tissues of these sites. With the help of effective maxillofacial prostheses, the functional results of these procedures are quite acceptable.
Twenty-eight patients with T3 and T4 carcinomas of the paranasal sinuses underwent orbital exenteration and combined craniofacial resection of the anterior and middle cranial fossa and the soft tissues of the face. Three patients died from the procedure and nine had nonfatal complications. Seventy-two percent of the patients with carcinoma of the maxillary antrum are free of disease at 3 years and 50 percent are free of disease at 5 years.
A 3-mo-old female presented with growth retardation, vomiting, reflux esophagitis, recurrent aspiration pneumonias, and was found to have megaesophagus and microgastria. After the failure of conservative therapy a double-lumen jejunal (Hunt-Lawrence) pouch with distal Roux-en-Y anastomosis was anastomosed to the stomach to increase the gastric reservoir. One year later, there has been progressive weight gain, the megaesophagus and gastroesophageal reflux have lessened significantly, pneumonia has not recurred, and the tracheobronchitis and esophagitis have resolved. This suggests that the gastroesophageal reflux and megaesophagus were due to an inadequate reservoir with a secondary gastric overflow as the esophagus dilated to enlarge the reservoir capacity of the upper gastrointestinal tract. Utilization of a jejunal pouch increased the size of the gastric reservoir, allowed resolution of the secondary esophageal changes, and permitted normal growth to proceed.
Five hundred and sixty-two patients who had attended the Glasgow Blood Pressure Clinic regularly for 3 years between 1969 and 1978 were studied. The mean BPs for the group were 187/115 mmHg initially, 157/100 mmHg after 6 months, and 153/98 mmHg after 3 years. Twenty-eight per cent had 'normal' systolic pressure and 22% 'normal' diastolic pressure at 3 years. Thirty-seven per cent with mild, 83% with moderate, and 89% with severe systolic pressure elevation had moved into less severe categories by 3 years, as had 30% with mild, 60% with moderate and 84% with severe diastolic evaluation. Those patients with severe hypertension, who did not attain a less grade, had a statistically significant drop in pressure. Four per cent of all patients, however, had moved into a more severe severe grade of systolic pressure evaluation and 6% into a more more severe diastolic pressure grade at 3 years. These results suggest that the hospital Hypertension Clinic can play a useful part in the lowering of BP in out-patients. It is clear, however, that 'normal' BP levels are not achieved in a significant proportion of patients.
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One hundred and fifteen patients undergoing palliative bypass for unresectable periampullary carcinoma were reviewed. Postoperative mortality and subsequent length of survival correlated with both presenting symptoms and operative findings. Among 93 patients presenting with biliary obstruction alone, 42 underwent a biliary bypass only and 51, a biliary bypass with a prophylactic gastrojejunostomy. Operative mortality and the postoperative hospital stay were similar for these two groups; postoperative complications tended to be more common in patients undergoing the double bypass. Patients undergoing a biliary bypass alone required significantly more subsequent operations, usually for gastroduodenal obstruction. Thus, it appears that a prophylactic gastrojejunostomy should be performed upon most patients with unresectable periampullary carcinoma presenting with biliary obstruction alone to decrease the number of subsequent palliative operative procedures required.
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Extended resection of the trachea with construction of a mediastinal tracheostomy was performed upon 21 patients with primary or recurrent carcinoma involving the cervical part of the trachea. In 12 patients, major complications developed, and eight died of these complications. Necrosis of skin flaps and tracheal wall, leading to sepsis of the mediastinum and rupture of major vessels, were the two most common complications and causes of death. Three patients remained free of disease for 143, 77 and 48 months. A review of this experience suggests that this procedure should be limited to patients witha tumor localized to the cervical portion of the trachea and that well vascularized skin flaps--myocutaneous flaps--should be used to resurface the mediastinum and base of the neck as a means of preventing most of the complications reported in this investigation.
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Plasma carcinoembryonic antigen (CEA) in nanograms per milliliter was assayed in 149 patients with benign and 567 patients with malignant disease. Elevated CEA level (greater than 5.0) was a good indicator of malignant disease but a poor screening test for cancer because of the high false-negative rate. Degree of elevation of plasma CEA level correlated with incidence of metastatic disease in patients with colorectal, gastric, and breast carcinomas, but no correlation was seen between CEA levels and status of lymph nodes in patients with localized disease. Patients with localized colorectal cancer, but elevated CEA levels before resection, had a 2.1-fold increase in the incidence of recurrence; however, this added to the prognostic value of Dukes' staging only when the CEA level remained elevated postoperatively. In 87% of patients with colorectal cancer, the CEA level was elevated at the time of recurrence, but a therapeutic value of reexploration for unexplained CEA level elevation was not confirmed.
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The specific organ system effects of cancer and its treatment have been reviewed from the standpoint of nutritional impact. Nutritional impairment may occur as the result of specific organ system malfunction, and malnutrition itself may impair organ system functions in such a way as to compound these deficits. Nonmalignant, acute, and chronic illnesses resemble cancer in many ways, particularly in their ability to produce malnutrition and these organ system malfunctions. It is apparent that there is nothing particularly unique about many of these cancer states, from the standpoint of nutritional deficiency, that is not found in nonneoplastic conditions.