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Biomedical subjects

R B Raney

Publications and source records attributed to R B Raney.

At least 91 records · Page 5Linked to original sources
PubMed1982

Soft-tissue sarcoma of the trunk in childhood. Results of the intergroup rhabdomyosarcoma study.

Disease-free survival data were obtained on 30 children with soft-tissue sarcoma of the trunk. The children were clinically grouped and treated in accord with the IRS protocol (Cancer 1977; 40:2015) from November 1972 through December 1976. Histologically, 40% of the tumors were alveolar rhabdomyosarcoma (RMS), 20% were embryonal RMS, 20% were extraosseous Ewing's sarcoma, 17% were undifferentiated sarcoma, and 3% were pleomorphic RMS. In 13 patients with primary tumors of the chest wall, the male-to-female (M:F) ratio was 3:11, and the median age was 12.5 yr. Five of the ten patients (50%) with localized tumor (Groups I-III) were disease-free at a median of 4.7 yrs. after diagnosis; two died of locally recurrent tumor, two died of metastases to lungs or bones, and 1 died of intracranial hemorrhage. None of the four with chest wall tumors and distant metastases (Group IV) survived. In ten patients with localized paraspinal primary tumors, the M:F ratio was 8:2, and the median age was 3.5 yrs. None had metastases. Seven of ten (70%) were disease-free at a median of 4.7 yrs. after diagnosis. Two died after regional recurrence developed (one retroperitoneal, one meningeal), and one died of lung metastases. In six patients with primary tumors of the abdominal wall, the M:F ratio was 3:3 and the median age was 9.5 yrs. Three of five with localized tumor (Groups I-III) were disease-free at a median of 5 yr. after diagnosis; one died after regional recurrence, and one died from accidental trauma. The one Group IV patient died of tumor. Overall, 15 of 30 patients (50%) are alive and free of recurrent disease at a median of 5+ yr. after initiation of treatment. The authors conclude that prognosis is most favorable for patients with paraspinal tumors, least favorable for those with chest wall tumors, and intermediate in patients with abdominal wall tumors. Prognostic differences appeared to be influenced by extent of disease at diagnosis and histologic subtype of the sarcoma.

Abdominal Neoplasms↗
PubMed1982

Three-year relapse-free survival rates in childhood rhabdomyosarcoma of the head and neck: report from the Intergroup Rhabdomyosarcoma Study.

In 202 patients with rhabdomyosarcoma of the head and neck who registered in the first Intergroup Rhabdomyosarcoma Study, the primary lesions arose about the eye and orbit in 26%, in parameningeal sites in 46%, and in other head and neck areas in 28%. Histopathologically, 78% were embryonal-botryoid, 9% alveolar, 10% undifferentiated, and 3% extraosseous Ewing's types. Actual three-year relapse-free survival rates were calculated from data on 103 of these patients who were free of distant metastases at diagnosis and in whom follow-up had been completed for a three-year period. The actual relapse-free survival rates were 91% (21/23) for those with eye/orbit primaries, 46% (20/44) for those with parameningeal primaries, and 75% (27/36) for those with other head and neck sites affected. Among those with no clinical evidence of tumor activity at two years, 8% (6/75) had subsequent relapses.

Child↗
PubMed1982

Extremity lesions in the Intergroup Rhabdomyosarcoma Study (IRS-I): a preliminary report.

The sites in which rhabdomyosarcoma occurs most frequently in children and young adults are the head and neck, genitourinary tract, and the extremities. Among these three sites, tumors of the extremities have been associated with the highest relapse rate and lowest survival rate in patients treated by protocols of the Intergroup Rhabdomyosarcoma Study (IRS). In five of six patients treated by primary extremity amputation, tumor recurred. Seven of 21 patients in Clinical Group I (completely resected tumors), and 9 of 27 patients in Clinical Group II (patients with gross tumor excision but positive lymph nodes, "microscopic residual" disease, or extension beyond the muscle of origin) had relapse. Thirteen of 18 patients (72%) in Clinical Group III (gross residual disease) responded to a chemotherapy-radiotherapy regimen, but ten have subsequently had relapses; and 13 of 18 are dead. Of 30 patients in Clinical Group IV (disseminated disease on entry), 15 initially responded to chemotherapy, but 25 of 30 are dead. The period of surveillance is 2 1/2 to 7 1/2 years, with a mean of 46.3 months at least examination. Increased rates of relapse were seen in patients with extremity tumors, as opposed to tumors in other sites, irrespective of the specific IRS chemotherapy-radiotherapy regimen employed. In Clinical Group I (nonamputation), relapse rates by histologic subtype of rhabdomyosarcoma were alveolar subtype, 5/8; embryonal, 1/7; and all other subtypes, 1/6. Clinical Group II, relapse rates were alveolar, 6/12; embryonal, 5/11; all other subtypes, 3/10.

Amputation, Surgical↗
PubMed1982

Primary chemotherapy in the treatment of children with bladder--prostate tumors in the Intergroup Rhabdomyosarcoma Study (IRS-II).

Twenty-nine children (24, male; 5, female) with non-disseminated rhabdomyosarcomas of the bladder or prostate were treated (1978-1980) by a primary chemotherapy regimen consisting of vincristine, actinomycin D, and cyclophosphamide ("Pulse" VAC), with or without local radiotherapy. During the initial 20 wk of chemotherapy, nine children achieved a Clinical Complete Response (CCR). Three of these are without evidence of disease (NED) and have functional bladders, two following partial cystectomy. Four who achieved a CCR subsequently relapsed or remained biopsy positive, but are at present NED following radiotherapy and anterior exenteration. Two patients who achieved CCR status relapsed and have died of disease. Twelve patients had a Clinical Partial Response (CPR) in less than 20 wk and two others in less than 40 wk. Seven of these are NED with intact bladders following chemotherapy-radiotherapy; and an additional patient is NED following partial cystectomy. Four patients in the CPR group have been treated by exenteration following failure to achieve complete response, and are NED. One patient has died, and one has progressive disease. Six patients had an inadequate response to chemotherapy (NR). Anterior exenteration was carried out in three, and two of these have survived. The overall results in these 29 patients are: (A) alive and disease-free with functional bladders, 11; (B) alive and disease-free following anterior exenteration, 10; and (C) dead or death from tumor anticipated, 8. The function of retained bladders (11) has been satisfactory.

Adolescent↗
PubMed1981

Transient cortical blindness secondary to vincristine therapy in childhood malignancies.

Three children with malignancies developed severe neurotoxicity, including transient cortical blindness, following chemotherapy regimens. The only drug in common was vincristine sulfate. The clinical courses of these patient are described. All have completely recovered, although one child who was rechallenged with vincristine had a cardiorespiratory arrest, increasing neurotoxicity, and recurrence of blindness. Transient cortical blindness has not previously been attributed to vincristine sulfate therapy, but must be considered as a possible complication of treatment with this agent.

Blindness↗
PubMed1981

Treatment strategies for infants with malignant sacrococcygeal teratoma.

Twelve children with malignant sacrococcygeal teratoma containing embryonal carcinoma, were treated at the Children's Hospital of Philadelphia between 1971 and 1980. Their ages at diagnosis ranged from 2 days to 23 mo; 8 of the 12 (67%) were girls. Five of the 12 patients presented with localized tumors which were grossly completely excised. Four received no further therapy, and all 4 recurred with histologically documented embryonal carcinoma. Despite subsequent treatment with radiation therapy (RT) and chemotherapy (vincristine, actinomycin D, and cyclophosphamide, collectively called VAC, in all 4 plus adriamycin in 3, only 1 has survived free of disease, 8 yr from diagnosis. The other 3 died of tumor (2) or pneumonia after pulmonary RT (1). The fifth patient in this group received VAC with adriamycin (total dose, 350 mg/sq m) and died of autopsy-proven cardiotoxicity without tumor. The remaining 7 patients presented with either unresectable local tumor (3) or distant metastases (4). One of these 7 died 6 days after biopsy with massive liver metastases. The other 6 children were treated after operation with VAC (5) or vincristine and actinomycin D (1) chemotherapy, and 5 also received RT to the pelvis. One has survived free of disease at 6 yr after treatment with VAC and adriamycin and pelvic RT, but the other 5 died of tumor (2) or of pneumonia after pulmonary RT (3). Surgery alone is inadequate for successful management of children with malignant sacrococcygeal teratoma. Chemotherapy with VAC, with or without adriamycin, can be effective when combined with radiation, but fatal pulmonary toxicity can result from such combined therapy. The optimal therapeutic program for children with malignant sacrococcygeal teratoma is still evolving.

Coccyx↗
PubMed1981

Rhabdomyosarcoma of the female urogenital tract.

During the initial 6 yr (1972-1978) of the Intergroup Rhabdomyosarcoma Study (IRS), 27 patients with rhabdomyosarcoma of the female urogenital tract were treated, and the results of therapy were evaluated on the basis of 3-7 yr of observation. By primary site these tumors were: bladder, 14; vagina, 9; vulva, 2; and cervix uterus, 2. Twenty-three presented with localized tumors; the four remaining patients (1 bladder, 1 vagina, and 2 cervix) had distant metastases at diagnosis. Two patients with bladder tumors underwent pelvic exenteration, local irradiation, and chemotherapy (2 yr), and were without relapse. Ten patients were treated by partial cystectomy and postoperative chemotherapy (with or without local irradiation) and fatal recurrence occurred in three. The remaining seven patients are disease-free for 36-74 mo (mean, 51.8 mo). Six of the eight patients with nondisseminated vaginal tumors were treated by initial pelvic exenteration (2), hysterectomy-vaginectomy (2), or hemivaginectomy (2). All received postoperative chemotherapy and three received local irradiation. Delayed hysterectomy-vaginectomy after primary chemotherapy was performed in the two additional patients with vaginal tumors. Relapse has occurred in one of these eight patients with localized vaginal disease. The remaining seven have been observed for 3-5.5 yr (mean, less than 4 yr). Two patients with vulval lesions treated by a combined therapy regimen are without recurrence. Our conclusion is that chemotherapy has significantly increased survival in females with urogenital tract rhabdomyosarcomas.

Adolescent↗
PubMed1981

Special considerations related to primary site in rhabdomyosarcoma: experience of the Intergroup Rhabdomyosarcoma Study, 1972--76.

Rhabdomyosarcomas (RMS) arising in the head and neck region, retroperitoneum, and perineum were considered together here because the usual surgical approach is incisional biopsy. Thus successful treatment of these neoplasms depends on effective radiation therapy and chemotherapy. From November 1972 through December 1976 the Intergroup Rhabdomyosarcoma Study accrued 127 patients with primary tumors in the head and neck, 34 with orbital tumors, 24 with sarcomas arising in the retroperitoneum-pelvis (no genitourinary), and 11 with perineal lesions. Results of treatment varied among these primary sites. Patients with orbital RMS had the best prognoses; 77% of them were free of disease, compared with a 51% disease-free rate in patients with nonorbital head and neck RMS. In this latter group, disease recurrence was evenly divided among local failure, distant metastases, and direct meningeal extension. Prognoses were similar for retroperitoneal tumors; 46% of such patients are currently free of disease. That the perineum was a rare site for RMS was fortunate because only 3 of 11 such patients (27%) are free of detectable disease now (January 1979). We concluded that the site of the primary tumor is an important prognostic variable in children with RMS.

Adolescent↗
PubMed1980

Radiation to regional nodes for rhabdomyosarcoma of the genitourinary tract in children: is it necessary? A report from the Intergroup Rhabdomyosarcoma Study No. 1 (IRS-1).

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.

Child↗