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Biomedical subjects

M Tefft

Publications and source records attributed to M Tefft.

At least 55 records · Page 3Linked to original sources
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↗
PubMed1986

Postsplenectomy sepsis and other complications following staging laparotomy for Hodgkin's disease in childhood.

This is a survey of 234 pediatric patients in whom staging laparotomy/splenectomy was carried out (1975 to 1981) in the course of the Intergroup Hodgkin's Disease in Childhood Study (IHDCS). Relapse has occurred in 44 of these patients, and 12 have died, 7 secondary to extension of lymphoma, 2 with herpes or pneumocystis infections, 2 with leukemia, and 1 from an unrelated accident. During the period of surveillance (mean 5.5 yr), five episodes of bacterial sepsis (positive blood cultures) have occurred, including two due to Streptococcus pneumoniae; and three, to Hemophilus influenzae. The former occurred in the small group of patients in this series who had not received the prescribed pneumococcal vaccination. No fatalities were associated with these septic episodes. Intestinal obstruction secondary to adhesions (benign) occurred in eight patients and was managed without intestinal resection or mortality. One patient required operative release of an obstructed ureter following laparotomy, and one, oophorectomy for an infarcted (transposed) ovary.

Adolescent↗
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

The value of surveillance testing in childhood brain tumors.

Follow-up procedures in children with brain tumors are designed to confirm tumor response to initial therapy, identify recurrence or progression at the time of potential secondary treatment, and establish the patterns of failure. Serial use of neurologic examinations and computerized tomography scans are outlined for all tumor types. Tumors capable of subarachnoid metastasis (medulloblastoma, ependymomas, pineal/suprasellar germ cell tumors, and pineoblastoma) require periodic assessment of the neuraxis by cerebrospinal fluid (CSF) cytology and myelography to establish the frequency of subclinical meningeal seeding in study settings. Serial analysis of specific biochemical markers for medulloblastoma (CSF polyamines) and germ cell tumors (serum and CSF alpha-fetoprotein and beta-human chorionic gonadotropin) are suggested. Serial evoked potentials in brain stem gliomas are recommended.

Astrocytoma↗
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

Prognosis for Wilms' tumor patients with nonmetastatic disease at diagnosis--results of the second National Wilms' Tumor Study.

Multivariate statistical methods were used to study prognosis for 632 patients entered on the second National Wilms' Tumor Study who had nonmetastatic, unilateral disease at diagnosis. Separate analyses were conducted for each of four endpoints: abdominal recurrence, distant metastasis, relapse without regard to site, and death. The two most important predictors for metastasis and general relapse were an unfavorable (anaplastic or sarcomatous) histology and the presence of microscopically confirmed disease in the regional lymph nodes. Operative spillage of tumor increased the rates of abdominal recurrence and death, even after accounting for histology and lymph node effects. The presence of a tumor thrombus in the renal vein or IVC increased the risk of metastasis, and intrarenal vascular invasion was associated with general relapse after accounting for histology, lymph nodes, and spillage. However, these latter associations were weaker, and some uncertainty remains regarding the true prognostic import of such findings due to a high degree of collinearity among variables. By contrast to the results of a similar data analysis for the first National Wilms' Tumor Study, specimen weight had no bearing on outcome, and the effects of age at diagnosis were entirely explained by the association of age with other more critical factors.

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

Abdominal relapses in irradiated second National Wilms' Tumor Study patients.

Ten of 259 (3.8%) irradiated patients with group 2 and 3 tumors in the second National Wilms' Tumor Study experienced initial clinical relapse either in the operative site or elsewhere in the abdomen, excluding the liver and opposite kidney. Analysis of factors associated with abdominal recurrences has shown the independent significance of unfavorable histology, field size of the radiotherapy portals, and a postoperative delay of ten or more days before starting irradiation.

Abdominal Neoplasms↗
PubMed1984

Recurrent and metastatic mesoblastic nephroma in infancy.

Local recurrence or metastasis of mesoblastic nephroma is extremely uncommon. The first report of a child with mesoblastic nephroma to have both of these problems is presented. The usual course of mesoblastic nephroma is benign, with nephrectomy being curative. Adjuvant treatment is not necessary and may be lethal. However, a review of the five patients who developed locally recurrent and/or metastatic tumor indicates that chemotherapy and radiotherapy are of benefit and can produce long-term disease-free survival.

Antineoplastic Agents↗
PubMed1984

Complications related to 234 staging laparotomies performed in the Intergroup Hodgkin's Disease in Childhood study.

This is a study of 234 children and young adult patients entered in the Intergroup Hodgkin's Disease in Childhood (stage I-II) Study from November 1975 to June 1981 and followed for a mean of 3.8 years after laparotomy. All patients had a staging laparotomy with total splenectomy, liver biopsy, and sampling of abdominal lymph node groups. Four patients (1.7%) have had documented sepsis, and three have had possible sepsis. There has been no sepsis-related death. Intestinal obstruction requiring operation was noted in four patients (no intestinal resection required). Urgent operation was necessary in two patients, one with ureteral obstruction and one with ovarian torsion, following a repositioning procedure, neither of these patients died. Organisms in the four patients with positive blood cultures were Streptococcus pneumoniae (two) and Haemophilus influenzae (two). Of the 234 patients in the study, 194 (83%) had received polyvalent pneumococcal vaccine, and 174 (74%) were taking prophylactic antibiotics. One of the two patients with pneumococcal sepsis had not been vaccinated, and the second was vaccinated only during radiotherapy. Only one of the four patients with positive blood cultures was on a prophylactic antibiotic treatment regimen at the time of the septic episode. The liabilities in employing laparotomy-splenectomy for the evaluation of pediatric patients with Hodgkin's disease include both general surgical complications and an increase in the risk of hyperacute infection, specifically related to encapsulated species. The latter appears to be modified to a major degree by current prophylactic measures or therapy.

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

Primary Ewing's sarcoma of the ribs. A report from the intergroup Ewing's sarcoma study.

Thirty-six patients with primary Ewing's sarcoma of the ribs have been reviewed. Of these, 21 had clinically localized disease at diagnosis and were entered on protocol IESS 7299, eight had regional and seven metastatic disease at diagnosis and were entered on protocol 7450. The 21 with localized disease were treated with surgical excision or biopsy, followed by local radiotherapy (in all but one patient) and randomization to one of three chemotherapy regimens. Eleven patients (52%) remain disease-free for periods ranging from 18 to 64 months, respectively. Seven of eight patients who underwent complete surgical excision of the primary lesion remain disease-free, compared with four of 12 (excluding one patient who died disease-free) who remain disease-free after partial excision or biopsy. However, analysis of size of tumor at diagnosis reveals that smaller primary tumors have a better prognosis irrespective of extent of surgery. Protocol IESS 7450, consisted of radiotherapy to all areas of known disease and four drug chemotherapy. Four (50%) with regional disease but none with metastatic disease have remained alive and continuously disease-free. It is concluded that an aggressive approach to Ewing's sarcoma of the ribs is justified by the results, as even regional disease may be curable. The apparent prognostic advantage for those patients undergoing surgical excision may be explained by patient selection.

Adolescent↗
PubMed1983

Treatment of spinal cord compression: a retrospective analysis.

Epidural spinal cord compression from metastatic disease is a common neurological complication of cancer. The incidence is probably increasing owing to continual advancements in the treatment of cancer that have led to prolongation of life and a greater probability of secondary involvement of the spinal cord. A problem often encountered by the oncologist treating patients with epidural spinal cord compression is the recurrence of compression by metastatic tumor both in and out of the original treatment field. Radiotherapists are often presented with the difficult task of trying to determine the optimal dose/time fractionation with the hope of improving the therapeutic ratio. We have examined the charts of 80 patients treated at the Rhode Island Hospital during the last five years (1975-1980) with myelographic evidence of cord compression in order to determine 1) the recurrence rate of cord compression by metastatic tumor after radiotherapy treatment both in and out of the original treatment field; 2) the influence of various dose/fractionation schedules on the disease-free interval; 3) the percentage of recurrence out of the treatment field that might represent "skipped lesions" at the original time of diagnosis. Our results show 1) that 9 patients (11.3%) experienced recurrence within the original treatment field; 2) that 21 (26.3%) experienced recurrence within the spinal canal, but out of the original treatment field; 3) that 9 of the 21 (42%) cases of recurrence out of the original treatment field occurred within 1 week and thus were determined to be "skipped lesions" at the time of diagnosis; and 4) that there appears to be a dose-response relationship for those patients treated successfully without recurrences who did not have presenting symptoms of complete paraplegia.

Combined Modality Therapy↗
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↗
PubMed1982

Combination chemotherapy (vincristine, adriamycin, cyclophosphamide, and 5-fluorouracil) in the treatment of children with malignant hepatoma.

Members of Childrens Cancer Study Group and the Pediatric Division of the Southwest Oncology Group conducted a study of chemotherapy for children with malignant liver tumors. All patients received vincristine, cyclophosphamide, Adriamycin and 5-fluorouracil in 6 weekly cycles for one year. Surgical resection and irradiation were employed when indicated. Between January 1976 and August 1978, 62 patients were entered on study; one was rejected for a protocol error, and ten had inadequate trials of chemotherapy, dying within one month of entry. The median time on study for all patients was 12 months. Twenty-four patients had no measurable disease following surgical treatment and chemotherapy was employed as adjuvant treatment; 20/24 (83%) remain relapse-free from 8-42+ months, (median, 30 months). In 27 patients, residual measurable disease was available to determine the response to chemotherapy. The response rate was 12/27 (44%), lasting 3-45 months (median, 18 months). The median follow-up of all survivors is 30 months. Hematologic toxicity was significant, particularly during the initial courses of chemotherapy; 28/57 patients developed severe toxicity which was fatal in three. The results from the current study were compared to those from a previous one initiated in 1972, in which actinomycin D, vincristine, and cyclophosphamide were given in sequence, one during each month for one year. Although the population of the two studies was not identical, there was a difference in the response rates (P = 0.02), relapse-free interval (P = 0.008), and survival (P = 0.003). The most striking improvement was seen in the patients with Group I disease, there were 7/11 relapses in the first study and 1/16 in the current one.

Antineoplastic Agents↗
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↗