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Biomedical subjects

R B Raney

Publications and source records attributed to R B Raney.

At least 73 records · Page 4Linked to original sources
PubMed1986

Malignant fibrous histiocytoma of soft tissue in childhood.

Seven children aged 6 months to 11 years with malignant fibrous histiocytoma, a type of sarcoma of soft tissues, have been treated at the Children's Hospital of Philadelphia from January 1975 through July 1983. The primary tumor arose in the head and neck region in three patients, the chest wall in two patients and the pelvis or buttock in one patient each. Operative management consisted of complete tumor removal in the two patients with chest wall tumors, and biopsy only in the remaining five children. Afterward, all seven patients were treated with a multiple-agent chemotherapy program consisting of vincristine, dactinomycin, and cyclophosphamide for two years, with or without Adriamycin (doxorubicin). The five patients with residual tumor also received radiation therapy (RT) in doses of 1500 to 5500 rad. The two children with localized, completely excised sarcoma are continuously free of tumor at 1.4 and 9 years after initiation of treatment. Of the five with residual sarcoma, three had a complete response to radiation and chemotherapy, and two of them are free of recurrence at 4 and 5 years, respectively. In the three remaining children, the tumor spread regionally into the central nervous system or distantly into the lungs, subcutaneous tissues, and liver. Childhood malignant fibrous histiocytoma of soft tissue appears to be similar to childhood rhabdomyosarcoma in its modes of spread and response to management. Operative removal is the key to successful therapy. The roles of multiple-agent chemotherapy and RT remain to be defined. Adriamycin appears to be the most promising single agent. In the absence of concrete data, it seems prudent to follow the same guidelines for irradiation as those used for other soft tissue sarcomas of childhood.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1986

Late effects after treatment of twenty children with soft tissue sarcomas of the head and neck. Experience at a single institution with a review of the literature.

Twenty children with soft tissue sarcomas of the head and neck, treated at the Children's Hospital of Philadelphia and the Hospital of the University of Pennsylvania from 1972 to 1981, were evaluated for the late deleterious effects of treatment. All patients received radiation therapy and combination chemotherapy with vincristine, dactinomycin, and cyclophosphamide; certain patients also received Adriamycin (doxorubicin). All had ophthalmologic, otologic, growth, and cosmetic evaluations; 15 also had dental and maxillofacial examinations. The median age at diagnosis was 6 years (range, 7 months-13 years). Median follow-up from time of diagnosis was 5.5 years with a minimum of 3 years in all but four patients. The major problems encountered were related to the eyes (xerophthalmia and cataracts), ears (hearing loss), teeth (maleruption and caries), glandular structures (xerostomia, hypopituitarism), and development (craniofacial deformity). It is concluded that children treated for soft tissue sarcomas of the head and neck with combined modality therapy, including radiation enhancers, may show a variety of late treatment-related adversities. These children require close multidisciplinary follow-up for detection of late effects in order that appropriate prophylactic or symptomatic treatment can be instituted to minimize their consequences.

Adolescent↗
PubMed1986

Late effects of therapy in orbital rhabdomyosarcoma in children. A report from the Intergroup Rhabdomyosarcoma Study.

Late effects of therapy were evaluated in 50 children surviving orbital rhabdomyosarcoma following treatment on Intergroup Rhabdomyosarcoma Study I. All patients had microscopic or gross tumor present following surgery and subsequently received radiotherapy and various combinations of chemotherapeutic agents. Problems in the eye included infections and functional and structural changes. Decreased vision in the treated eye was the most common functional problem and in most patients related to cataract formation, which occurred in 90% of eyes. Changes in the cornea and retina were also seen. Bony hypoplasia of the orbit and facial asymmetry were present in one half of the children. Gonadal development was normal. Statural growth was retarded in 61% of the patients. All children were in school, with five having learning or behavioral problems. One child developed acute myeloblastic leukemia. The excellent survival in patients with orbital rhabdomyosarcoma provides support for treatment programs that use multiple-agent chemotherapy and radiotherapy and do not include orbital exenteration initially.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1985

Soft tissue sarcomas arising in the retroperitoneal space in children. A report from the Intergroup Rhabdomyosarcoma Study (IRS) Committee.

One hundred one eligible children with soft-tissue sarcomas arising within the retroperitoneal space have been registered on Intergroup Rhabdomyosarcoma Study Committee (IRS) studies I and II and followed for at least 2 years or until death. The most common presenting symptoms and signs were pain in the abdomen or lower extremities, and/or an abdominal mass, usually noted by a parent or a physician seeing the child for other complaints. The median age at diagnosis was 6.5 years and the sex ratio (M/F) 1.7:1. Histologic types were embryonal or botryoid rhabdomyosarcoma (RMS) in 58 patients, alveolar RMS in 8, pleomorphic RMS in 2, undifferentiated sarcoma in 20, extraosseous Ewing's sarcoma in 4 and unspecified sarcoma in 9. Median tumor size was 10 cm, significantly larger than the 7.5 cm noted in the IRS studies as a whole (P less than 0.05). One patient had complete tumor removal (Group I); 12 had grossly complete removal with microscopic residual tumor (Group II). Fifty-one patients had residual local tumor after biopsy or partial resection (Group III) and 37 patients had distant metastases at diagnosis (Group IV). Treatment included surgery, radiation therapy (RT) and combination chemotherapy with vincristine and actinomycin D with or without cyclophosphamide and Adriamycin (doxorubicin) according to protocol. Thirty-nine of 99 patients (39%) had major difficulties in the delivery of specified RT. Seventy patients received sufficient therapy to be evaluable for treatment response. Forty-one (58%) achieved a complete remission and 16 (23%) achieved partial remission. Twenty-four of 41 children (59%) achieving a CR have relapsed. The proportion of children who remained relapse-free at 2 and 3 years of follow-up was 44% or 42%, respectively. Overall, 40 children have developed recurrent sarcoma and the median disease-free interval and overall survival times were 54 and 88 weeks, respectively. Most children experienced severe myelosuppression; there were three early deaths from infection which occurred during granulocytopenic periods. One third of the patients experienced cystitis. Children with soft-tissue sarcomas arising in the retroperitoneal space have a poor prognosis associated with large tumors which, because of location and/or extent (Groups III or IV), are unresectable and difficult to treat.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1985

Rhabdomyosarcoma of the biliary tree in childhood. A report from the Intergroup Rhabdomyosarcoma Study.

Ten cases of rhabdomyosarcoma of the biliary tree (RMS/BT) in children are reported in this review of the Intergroup Rhabdomyosarcoma Study (IRS) I and II. RMS/BT constitutes 0.8% of evaluable tumors on IRS I and II. Intermittent obstructive jaundice with or without abdominal distention, fever, and loss of appetite is the typical presentation. Attribution of these symptoms to hepatitis commonly delayed definitive treatment. Demonstration of a mass in the porta hepatitis by ultrasound led quickly to surgical consultation and an exploratory laparotomy. Intraoperative cholangiography was a valuable technique in establishing the level of biliary tree obstruction and verifying a functioning drainage procedure. Resection of the mass with only microscopic or minimal gross residual disease was possible at the initial surgery in six of the ten patients. Included in this group are all four of the surviving patients. The continuity of bile flow was usually maintained by variations of a Roux-en-Y jejunostomy. Histology was embryonal RMS with some botryoid elements. Consultation with a surgical pathologist during the course of the surgery was of invaluable assistance. Multi-drug chemotherapy and radiotherapy consistent with the IRS protocols were given postoperatively. A second-look surgery was useful in evaluating residual or recurrent disease. Four patients are surviving free of disease at 6 months, 3, 6 1/4, and 6 1/2 years from diagnosis, respectively. This review demonstrates that a multidisciplinary approach in RMS/BT can lead to long-term survival.

Bile Duct Neoplasms↗
PubMed1985

Sarcomas of the vagina and uterus: the Intergroup Rhabdomyosarcoma Study.

During a 12-year period (1972-1984), 43 patients with sarcomas of the female genital tract were admitted to the Intergroup Rhabdomyosarcoma Study (IRS), including 31 with primary tumors of the vagina; and 12 with tumors of the uterus, including the cervix. Thirty-four of these can be evaluated on the basis of periods of observation from 18 months to 12 years. Primary tumors of the uterus were a distinct group, distinguished from those arising in the vagina by patient age-range and probably by prognosis, as well as site. Patients with vaginal tumors, with a mean age of 1.8 years, responded to a multimodality approach employing combinations of chemotherapy (vincristine sulfate and actinomycin D, or the aforementioned two drugs with cyclophosphamide +/- doxorubicin hydrochloride), irradiation, and/or surgery, with only one tumor-related death, among 24 evaluable patients. In contrast, among the patients with primary uterine tumors, in which the mean age was greater than 14 years, four of ten evaluable patients died secondary to tumor relapse or progression.

Adolescent↗
PubMed1985

Healing rates of treated and untreated bone lesions in histiocytosis X.

Evaluation of the response of histiocytosis X to various forms of treatment is difficult, because presentation patterns are protean and the disease can be self-limited. A retrospective evaluation was made of the healing of 42 histiocytosis X bone lesions, in 21 patients treated in variety of ways, using serial radiographs and a semiquantitative scoring system. Treatments included various combinations of surgery (simple biopsy or curettage), radiotherapy (200 to 1,200 centi-Gray [cGy]), chemotherapy (according to various protocols), and local steroid injection. Median times to a given degree of healing were similar across treatment groups and in untreated lesions. It was concluded that mode of treatment does not exert a strong influence on the rate of healing of histiocytosis X bone lesions. Some healing should be apparent 4 months after diagnosis, but complete healing may take many months. Treatment of histiocytosis X bone lesions is indicated only if intense pain or risk of fracture or deformity are present.

Adolescent↗
PubMed1984

Soft tissue sarcoma of the head and neck in childhood: nonorbital and nonparameningeal sites. A report of the Intergroup Rhabdomyosarcoma Study (IRS)-I.

Seventy-two patients with soft tissue sarcoma arising in the oral cavity, oropharynx, larynx, parotid region, cheek, scalp, and neck, were entered on the Intergroup Rhabdomyosarcoma Study (IRS)-I, and could be analyzed for survival. Sixty-three (88%) attained complete remission (CR), of whom 13 subsequently relapsed (78% actuarial 5-year retained CR rate). The patients with primary tumor arising in sites other than the neck had a retained CR rate exceeding 90%. In contrast, 11 relapses occurred in the 26 patients with neck primaries (54% retained CR rate). Females, and infants younger than age 24 months were more likely to relapse. Prognostic factors with little or no influence on relapse included tumor size, histology, regional lymph node status, clinical group, and treatment arm. Five of the 6 patients with failure at the primary site had either no radiotherapy (2 patients) or an insufficient dose (less than 3000 rad). No patient required major organ sacrifice such as laryngectomy or pharyngectomy. Isolated failure in regional nodes did not occur. Children with nonorbital, nonparameningeal head and neck soft tissue sarcoma treated in accordance with the IRS protocol have an excellent rate of local control and survival. Primary tumors arising in the neck are more likely to relapse locally or distantly.

Adolescent↗
PubMed1984

Electron microscopy of glandular Schwannoma.

This report is the first description of the ultrastructure of the glandular elements in a so-called glandular Schwannoma (malignant peripheral nerve sheath tumor with glandular elements). The appearance of this uncommon tumor was typical by light microscopy. Electron microscopy of the sarcomatous component revealed features consistent with Schwann cell origin. Ultrastructure of the glands revealed a well-differentiated epithelium with basement membrane, desmosomes and the presence of microvilli with core rootlets, glycocalyx, and intracytoplasmic R-bodies. No cilia or blepharoplasts were present, arguing against ependymal origin of these glands. The microvilli with core rootlets, the glycocalyx and the R-bodies indicate the glandular epithelium was of the "intestinal type." The basis for the presence of such epithelium in Schwann cell neoplasms is uncertain, but pathologic induction-interaction between neural crest and mesenchyme may be the most plausible explanation.

Adolescent↗
PubMed1984

Localized sarcoma of the chest wall.

This 9-year-old white male suffered mild trauma to the left side of the chest on two occasions in April 1981, and then developed a low-grade fever. A chest x-ray film showed pneumonia. He was then given oral ampicillin for 2 weeks, and the fever and left chest pain resolved. Repeat chest radiograph one month later showed a persistent density, and a bone scan revealed increased uptake in the left fifth rib. Osteomyelitis was diagnosed, and intravenous antibiotics were administered. In June 1981, the density had become larger on radiographic examinations, and a left chest wall mass was noted for the first time. He was referred to the Children's Hospital of Philadelphia, with the suspected diagnosis of Ewing's sarcoma of the left fifth rib. On arrival at the Children's Hospital of Philadelphia on June 13, 1981, the physical examination was unremarkable except for a 1 X 2-cm mass overlying the fifth rib anterolaterally. Dr Hugh Watts of the Division of Orthopedic Surgery reviewed the radiographs and bone scan and felt that the appearance was not consistent with primary Ewing's sarcoma of the rib. The boy was then taken to surgery by Dr. Moritz Ziegler on June 23, 1981, at which time biopsy of the lesion was accomplished. The tumor had by then grown to a size of 3 X 5 cm.

Child↗
PubMed1983

Prognosis of children with soft tissue sarcoma who relapse after achieving a complete response. A report from the Intergroup Rhabdomyosarcoma Study I.

Four hundred and twenty-three children with newly-diagnosed soft-tissue sarcoma were treated on the Intergroup Rhabdomyosarcoma Study I from November 1972 through December 1976. After institution of therapy, 341/423 (80.6%) achieved a complete response (no detectable tumor), but 115/341 (33.7%) subsequently relapsed. The types and efficacy of management after relapse were ascertained by questionnaire. Information sufficient for critical analysis was obtained for 98/115 patients (85%). Thirteen of the 98 patients had an isolated local recurrence; the other 85 developed metastases in regional, distant, or multiple sites simultaneously. Patients with distant metastases at diagnosis were most likely to develop recurrence, as were those with sarcoma arising in the perineum-anus, retroperitoneum-pelvis, gastrointestinal tract, or extremity. Those with tumor primary in the orbit or genitourinary tract had the lowest recurrence rate, as did those whose tumor histology was nonalveolar. Individualized treatment was given to 90 of the 98 patients after relapse, and a second complete response was achieved in 33 of them (37%). However, only five of the 90 patients (5.5%) are currently tumor-free. The outlook is poor when recurrence develops in patients still receiving chemotherapy; only two of 74 such patients survive, compared to three of 16 who have relapsed after cessation of treatment. Relapse after initial complete response to therapy has a poor prognosis, regardless of site of recurrence. Although effective retrieval therapy can prolong survival, only 2 of 12 patients (17%) with local recurrence and three of 78 patients (4%) with metastatic recurrence remain free of disease.

Adolescent↗
PubMed1983

Rhabdomyosarcoma of the ear in childhood. A report from the Intergroup Rhabdomyosarcoma Study-I.

Twenty-four children with rhabdomyosarcoma of the middle (22 patients) or external ear (two patients) were entered on the Intergroup Rhabdomyosarcoma Study-I protocol from 1972 to 1978. After surgery, treatment consisted of radiotherapy plus vincristine, dactinomycin, and cyclophosphamide, with or without Adriamycin. Nine of 19 patients (47%) who presented with localized sarcoma are free of disease at 2.2 to 6.5 years after diagnosis (median, 3.6 years). One is alive with regional recurrence at 6.7 years; another developed a contralateral cerebellar astrocytoma 4.4 years from diagnosis and died without evidence of rhabdomyosarcoma 2 months later. The other 13 children died of recurrent rhabdomyosarcoma at 5 to 25 months after diagnosis (median duration of survival, 10 months). Outcome was influenced by the presence of diagnosis of signs indicating meningeal extension; death rates were 5 of 5 patients with intracranial tumor, 3 of 4 with petrous bone erosion, 2 of 6 with facial nerve palsy, and 3 of 9 with no initial evidence of meningeal extension.

Child↗
PubMed1983

Renal cell carcinoma in children.

Data from four pediatric hospitals concerning 20 children treated for renal cell carcinoma (RCC) from 1964-1978 were reviewed. Median age of the patients (pts) was 11.8 years (range, 14 months-19 years). Twelve were male and eight female; 17 were white and three black. Most patients presented with pain and hematuria with or without a palpable mass. An intrarenal tumor was detected at IV urography (17 pts), arteriography (2 pts), or at surgery (1 pt). Treatment consisted of nephrectomy in 15 pts, renal biopsy (4 pts), or no surgery (1 pt), followed by chemotherapy (5 pts), radiation therapy (1 pt), or both (7 pts). Ten pts died of distant metastases at a median of one year (range, 0.2 to two years) after diagnosis. The other 10 pts (50%) survive free of relapse at a median of 4 years (range, two to ten years) from diagnosis. Proportions surviving free of recurrent disease two or more years by National Wilms' Tumor Study (NWTS) Group were 5/5 in Group I, 3/7 in Group II, 1/3 in Group III, and 1/5 in Group IV; by age at diagnosis, 6/6 in those under 11 years old and 4/14 in those 11 or older; and by type of surgery, 10/15 who had nephrectomy and 0/5 with limited or no surgery. The data indicate that radiation and chemotherapy had only minor if any influences on relapse-free survival. We conclude that (1) RCC in children is similar to its counterpart in adults; (2) RCC has a worse prognosis than Wilms' tumor except for the earliest stage; (3) nephrectomy alone is adequate treatment for Group I RCC, and (4) young age (less than 11 years old) may be prognostically favorable.

Adenocarcinoma↗
PubMed1983

Histiocytosis X in children: patterns of disease and results of treatment.

The pathologic materials and clinical courses of 36 children aged 1 month-22 years, with histiocytosis X (H-X) seen at the Philadelphia Children's Cancer Research Center from 1970 to 1979 were reviewed. The pathologic subtype of H-X was favorable (type II) in 31 patients, unfavorable (type I) in one patient, and unclassified in four patients whose specimens were limited to a skin biopsy. Sixteen patients had localized H-X involving bone (14 patients), soft tissue (1 patient), or skin only (1 patient); all are alive and well after treatment with surgery alone (12 patients), radiation therapy (RT) (3 patients), or observation (1 patient); only 1 of the 16 developed recurrent H-X. The other 20 patients presented with multifocal H-X involving the skeleton alone (3 patients); the skeleton and soft tissues other than liver (7 patients); soft tissue exclusive of the liver (3 patients); soft tissue including the liver (4 patients); or soft tissues, skeleton, and liver (3 patients). These 20 patients were treated with surgery alone (1 patient), RT (4 patients), or multiple drugs +/- RT (15 patients). Seven of the 20 patients are alive and well without recurrence at a median of 4 years after diagnosis. Nine of the 20 patients, including 3 with liver dysfunction, responded completely to initial therapy but developed recurrence; each was retreated with drugs and is alive and well at a median of 4 years. The remaining 4 patients had widespread disease with dysfunction of the liver and/or hematopoietic system at diagnosis, failed to respond, and died. We conclude that (1) patients with multiple bony lesions with or without associated soft tissue disease or skin involvement have a favorable outlook and do not require systemic chemotherapy; (2) systemic treatment also is unnecessary for patients with localized H-X since recurrence is rare; (3) drugs can benefit patients with multifocal H-X, although the optimal duration of therapy is unclear; and (4) favorable response to treatment indicates high probability of disease-free survival. However, organ dysfunction at diagnosis is ominous: four of seven patients with liver dysfunction are dead, as are all three patients who presented with peripheral blood count depression.

Adolescent↗
PubMed1983

Radiation-induced pneumothorax.

Pneumothorax is an uncommon complication of radiation therapy to the chest. The proposed pathogenesis is radiation-induced fibrosis promoting subpleural bleb formation that ruptures resulting in pneumothorax. We report on two young patients with primary sarcomas without pulmonary metastases who developed spontaneous pneumothorax after irradiation. Neither patient had antecedent radiographic evidence of pulmonary fibrosis.

Adolescent↗
PubMed1983

Mortality among children with rhabdomyosarcomas of the alveolar histologic subtype.

Of 116 patients with rhabdomyosarcoma of the alveolar histologic subtype who entered the Intergroup Rhabdomyosarcoma Study (IRS) from 1972 to 1978, there were 72 deaths (63%), compared with a mortality of 39% among patients with all nonalveolar types combined. The subgroup with alveolar histology contributed greater than 29% of the total mortality, although it represented 20% of the total patients (p less than .001). This recurrence rate reflects increased local, regional, and distant relapse. Differences were most marked in patients with grossly excised tumors (clinical groups I and II) in which the mortality was 44% in patients with alveolar tumors (48 patients) versus 13.5% in those with embryonal histology (111 patients), and 16% in all patients with nonalveolar tumor types. When survival was influenced by primary tumor site this reflected, in most body areas, an increase in the proportion of patients with the alveolar histology in that site. Patients with unresected or disseminated tumors (clinical groups III and IV) of the alveolar subtype have initial response rates to VAC and radiotherapy which are similar to those of patients with tumors of other cell subtypes (70% for group III and 50% for group IV). In these clinical groups, the differences in survival related to histologic subtype are not significant. The presence of the alveolar histologic subtype represents a rational basis for employing more intensive therapy in the management of patients with rhabdomyosarcomas.

Adolescent↗
PubMed1982

Bladder and prostatic tumors in the intergroup Rhabdomyosarcoma study (IRS-I): results of therapy.

Sixty-four children with primary rhabdomyosarcoma of the bladder or prostate were enrolled in the IRS during the initial five years (October 1972-November 1977). Observation of these patients for from 2 1/2-8 years forms the basis of this report. The chemotherapy-radiotherapy regimens employed (IRS) for each Clinical Group were: (1) Group I (completely excised tumor), sequential actinomycin-D (ACD), vincristine (VCN), and cyclophosphamide (CYP), i.e., standard (VAC), with or without radiotherapy (RT); (2) Group II (resected local disease with node involvement, "microscopic" residual, or local extension), RT plus sequential ACD and VCN, or RT plus standard VAC; (3) Group III, (gross residual disease); and (4) Group IV (dissemination), RT and either pulse VAC or pulse VAC plus Adriamycin (ADR). Pulse VAC consisted of VCN (day 1), plus daily intravenous ACD and CYP (days 1-5). Relapse rates were: Group I, 0/8; Group II, 6/23; and the mortality in Group III, 6/23; and in Group IV, 7/10. Among patients with bladder tumors, the rate of relapse was 2/11 following pelvic exenteration (anterior, 10; total 1); 5/12 following partial cystectomy or gross tumor excision; and 3/5 following a primary chemotherapy-radiotherapy (PCR) regimen. In patients with prostatic tumors (Groups I-III), relapse occurred in 0/14 patients treated initially by pelvic exenteration (anterior, 12; total, 2); and in 2/11 patients treated by a PCR regimen.

Adolescent↗