PubMed Health⌕ Search

Biomedical subjects

F Ghavimi

Publications and source records attributed to F Ghavimi.

At least 37 records · Page 2Linked to original sources

Factors predictive of mortality in pediatric extremity rhabdomyosarcoma.

In order to examine factors predictive of fatal outcome in children presenting with histologically confirmed extremity rhabdomyosarcoma, we performed a retrospective analysis of our institutional experience from 1970 to 1985. Thirty-five patients were identified and staged according to international criteria (TNM). Variables evaluated for their predictive effect on fatal outcome included (1) tumor invasiveness, (2) tumor size, (3) anatomic location of the primary, (4) regional lymph node involvement, (5) distant metastases at presentation, (6) complete surgical resection, (7) use of amputation, and (8) alveolar histologic subtype. Significant predictors of mortality included (1) tumor invasiveness (P less than or equal to .0001), (2) regional node involvement (P less than or equal to .0002), (3) distant metastases at the time of presentation (P less than or equal to .001), (4) alveolar histology (P less than or equal to .001), (5) size of primary (P less than or equal to .007), and (6) completeness of surgical resection (P less than or equal to .05). In multivariate analysis, local tumor invasiveness was the most important predictor of fatal outcome with an associated relative risk of 18. We conclude that local tumor invasiveness is the most important determinant of clinical stage.

Adolescent↗

Radiocurability of microscopic disease in childhood rhabdomyosarcoma with radiation doses less than 4,000 cGy.

In an attempt to evaluate the radiocurability of microscopic disease in childhood rhabdomyosarcoma (RMS) with total tumor doses of less than 4,000 cGy, we performed a retrospective analysis of all patients with microscopic residual RMS who were treated at the Memorial Sloan-Kettering Cancer Center (MSKCC) during the years 1970 to 1987. There were 32 patients ranging in age from 3 months to 22 years (median, 6 years) with microscopic residual of either (1) a localized primary tumor (MSKCC, stage IB; Intergroup Rhabdomyosarcoma Study [IRS] group IIA), 19 patients; or (2) an involved lymph node region with the primary tumor completely resected (MSKCC stage III; IRS group IIC), 13 patients. Twenty-nine of the 32 patients presented with embryonal histology. All patients were treated with combination chemotherapy (CT) and megavoltage external beam radiotherapy (RT). The RT was delivered in either conventional fractionation of 180 to 200 cGy daily (30 patients) or hyperfractionation of 150 cGy twice daily (two patients). Fifteen patients received RT doses of less than 4,000 cGy with a range of 3,000 to 3,600 cGy and a median value of 3,100 cGy; 17 patients received 4,000 cGy or more with a range of 4,000 to 6,000 cGy and a median value of 4,600 cGy. With a median follow-up of 11 years, the relapse-free survival was 25 of 32 patients (less than 4,000 cGy, 12 of 15; greater than or equal to 4,000 cGy, 13 of 17). The RT local control rate was 30 of 32 (less than 4,000 cGy, 14 of 15; greater than or equal to 4,000 cGy, 16 of 17 [P = .94]). Our results suggest that radiation doses of below 4,000 cGy, when combined with effective multiagent CT, may be sufficient for local control of microscopic disease in childhood embryonal RMS.

Adolescent↗

Prognosis in childhood rhabdomyosarcoma of the extremity.

From 1970 to 1987, 34 patients younger than 22 years of age with extremity rhabdomyosarcoma were treated at the Memorial Sloan-Kettering Cancer Center (MSKCC). All patients were treated according to protocols consisting of surgery, radiotherapy, and multiple drug chemotherapy. Sixteen of 34 patients survived, and 14 continue to be disease-free; the 5-year survival rate was 44%. A retrospective univariate analysis of data according to the TNM staging system was undertaken. Tumor invasion, regional nodal involvement, distant metastases, and alveolar histologic condition each had a significant impact on survival. However, in multivariate analysis, the stage of disease at diagnosis was the most important predictor of survival outcome.

Adolescent↗

The influence of extensive bone erosion on local control in non-orbital rhabdomyosarcoma of the head and neck.

To investigate a recent report suggesting extensive bone erosion (EBE) as an important prognostic factor in head and neck rhabdomyosarcoma, a retrospective review was performed of 32 patients with gross residual and/or metastatic non-orbital head and neck rhabdomyosarcoma treated between the years 1971-1987. Treatment consisted of surgery, radiation therapy (median primary dose, 5000 cGy) and combination chemotherapy according to the Memorial Sloan-Kettering Cancer Center (MSKCC) T2 and T6 protocols. The MSKCC staging system was used: Stage II, 23 patients; Stage III, 6 patients; and Stage IV, 3 patients. With a median follow-up of 48 months (range 17 months to 13 years), the overall survival and local control rates were 72% (23/32) and 75% (24/32), respectively. Local control was achieved in 11/11 patients without extensive bone erosion (Stages II, 9/9; III, 1/1; IV, 1/1) as compared to 13/21 patients (Stages II, 10/14; III, 2/5; IV, 1/2) with extensive bone erosion (p = 0.02). Our data appear to support the recently reported finding that extensive bone erosion is an important predictor of local failure.

Adolescent↗

Mortality in pediatric paratesticular rhabdomyosarcoma: a multivariate analysis.

We performed a retrospective analysis of 28 children presenting with confirmed paratesticular embryonal rhabdomyosarcoma to examine factors predictive of fatal outcome. Complete surgery, defined as radical inguinal orchiectomy with clear microscopic margins plus radical, retroperitoneal lymph node dissection with extirpation of all gross disease, was performed in 21 patients (75 per cent). All patients received chemotherapy and 20 received radiation therapy according to protocol. Of the patients 16 have survived for more than 5 years with no evidence of disease. Univariate analysis revealed that only the presence of parenchymal metastases at diagnosis (p less than or equal to 0.040) and unresectability (p less than or equal to 0.003) were significant predictors of fatal outcome. Multivariate analysis showed that unresectability was the most important predictor of mortality with an estimated relative risk of 5.8.

Adolescent↗

A model for embryonal rhabdomyosarcoma tumorigenesis that involves genome imprinting.

Embryonal rhabdomyosarcomas (malignant pediatric tumors of striated muscle origin) have been shown to arise from cells that are clonally isodisomic for loci on chromosome 11p. We determined the parental origin of alleles in this genomic region in familial and sporadic cases of this disease and found that isodisomic chromosome 11p alleles in each tumor were of paternal origin. We have developed a modification of Knudson's two-hit model from these data that is capable of explaining the preferential allele retention and of resolving the apparent contradiction between such specific and early events in several embryonal tumors and discrepancies in the inheritance of predisposition in some of these diseases.

Alleles↗

Preliminary results of alternating combination chemotherapy (CT) and hyperfractionated radiotherapy (HART) in advanced rhabdomyosarcoma (RMS).

In an attempt to reduce treatment (Rx) related acute toxicity and improve protocol compliance without compromising local control nor overall survival, a Phase II single arm pilot employing alternating intensive multiagent CT and radiotherapy (RT) in children with gross residual or metastatic RMS was begun at Memorial Sloan-Kettering Cancer Center (MSKCC) in 1984. Hyperfractionated radiotherapy (HART) was adopted to allow for timely delivery of both the RT and CT. From July, 1984 through July, 1986 12 patients (pts), aged 2 to 23 yr (median 10 yr) were enrolled on study. CT treatment was delivered over approximately 14 months and included 2 induction and 5 maintenance cycles. The induction CT consisted of two repetitive cycles of Vincristine, Dactinomycin, Cyclophosphamide, Adriamycin, Bleomycin, and Methotrexate; the maintenance CT included the same agents as reduced drug doses. The HART was delivered to the primary site during cycle I of induction at fractions of 150 cGy BID to a total dose of 5400 cGy in 2 courses of 3000 cGy and 2400 cGy, respectively. One hundred percent of patients completed the recommended dose of HART; 0% required unplanned interruptions of HART due to treatment toxicity. With a median follow-up (f/u) from diagnosis of surviving patients of 25 months (range 20-30), the local control rate is 83% (10/12 pts) and the overall survival, 58% (7/12 pts). The median time (MT) to any failure, local or metastatic, is 9 months (mo); and to death, 14 mo. Comparison of results with 12 historical controls with concomitant split-course standard fractionation RT (180-200 cGy/per fraction) and T6 CT during a MSKCC trial from 1975-1984, matched by site and stage of primary, revealed that 9 pts (75%) completed the recommended dose of RT, and 7 pts (58%) required interruptions of RT. With a median f/u of 78 mo (range 34-109), the local control rate was 75% (9/12 pts) and the overall survival, 42% (5/12 pts). The MT to any failure was 14 mo; and to death, 18 mo. These results indicate that the mode of alternating CT and HART (HART T6) as employed in this pilot study is well tolerated. It appears to offer a significant improvement in protocol compliance over previous protocols using concomitant CT and RT without any apparent compromise in local primary control or survival rates with a median f/u suggestive of adequate elapsed time for the appearance of most relapses and deaths in advanced RMS.

Adolescent↗

Parameningeal rhabdomyosarcoma (including the orbit): results of orbital irradiation.

Twenty-three patients with parameningeal (including orbital rhabdomyosarcoma (RMS)) were treated at Memorial Sloan-Kettering Cancer Center (MSKCC) between July 1971 and January 1983. Twenty were children with a mean age of 6 and 3 were adults. In 6 patients, the primary tumor was from the orbit, whereas the remaining 17 had other parameningeal primary sites. The tumors were in a very progressive local stage, with extensive destruction of the facial bones in 19 patients. Eight patients were treated with T2 chemotherapy protocol and 15 received T6. Seven patients received 5,000 to 7,200 rad delivered to the primary tumor in 11-16 weeks, 15 patients received between 4,500 to 5,000 rad in 4-7 weeks, and 1 patient received 3,000 rad in 3 weeks for residual microscopic disease following surgery. Two patients were treated with radiation to the whole brain; no patients received radiation of the whole central nervous axis (CNA). Fifteen of the 23 patients (65%) are alive and well with a medical follow-up time of 5 years. Two patients died of therapeutic complications and six died of tumor spread. In five patients, involvement of the central nervous system (CNS) was the cause of death. The prognosis of orbital RMS with parameningeal involvement is no better than in other tumors of parameningeal sites. In those patients who had impaired vision because of optic nerve damage prior to treatment, the vision did not improve following treatment. There was no impaired vision seen due to radiation damage of eye structures except in the lens.

Adolescent↗

Treatment of genitourinary rhabdomyosarcoma in children.

We report on 27 children with rhabdomyosarcoma of the bladder, prostate, uterus, cervix and vagina treated with combined therapy of surgery, radiotherapy and multiple drug chemotherapy. Of the 27 patients 18 are alive for 13 to 147 months (median 86 months) and 9 have retained a functional bladder. Extirpative surgery followed by adjuvant chemotherapy and occasionally radiation therapy was most effective for local tumor control and 7 of 10 patients survived, including 5 with an intact functional bladder. Primary use of chemotherapy in 13 patients with extensive disease resulted in complete tumor control in only 3 but 7 survived, including 4 with an intact bladder. Radiation therapy used concomitantly with chemotherapy failed to control the local tumor but with subsequent extirpative surgery all 4 patients survived.

Adolescent↗

Recovery of adrenal function after treatment of adrenocortical carcinoma with o,p'-DDD.

The adrenolytic agent, 2,2-bis[2-chlorophenyl-4-chlorophenyl] 1,1 dichloroethane (o,p'-DDD), was used over a 20-month period following surgery in a 2 3/12-year-old girl for treatment of adrenocortical carcinoma. The child remained free of disease and was maintained on glucocorticoid and mineralo-corticoid supplements for 7 years. Hormonal evaluation was undertaken at 9 9/12 years of age to determine remaining adrenal steroidogenic capacity. Following discontinuation of both hydrocortisone and 9 alpha-fludrocortisone, she remained stable and asymptomatic. Immediately after discontinuing 9 alpha-fludrocortisone, the adrenal glomerulosa was able to respond to stimulation by the renin-angiotensin system as shown by the ability to achieve renal sodium conservation on a restricted sodium intake (less than 10 mEq/d for 5 d). The response of the adrenal fasciculata to ACTH stimulation showed a slower recovery. Baseline levels of cortisol were in the low normal range, but there was no increase in plasma cortisol or urinary 17-hydroxysteroids following stimulation with ACTH. The responses of cortisol, deoxycorticosterone, and corticosterone to ACTH stimulation gradually improved to achieve normal stimulated levels 18 months after stopping medications. Serum testosterone and delta 4-androstenedione were initially increased for level of puberty, while levels of dehydroepiandrosterone were prepubertal. Testosterone and delta 4-androstenedione did not suppress with dexamethasone (2 mg/d for 2 d; 4 mg/d for 2 d), and dehydroepiandrosterone decreased only slightly. However, administration of norethindrone (Norlutin) (10 mg orally, three times a day for 3 d) resulted in suppression while human chorionic gonadotrophin (hCG; 5000 U i.m. daily for 3 d) produced stimulation of testosterone, delta 4-androstenedione and dehydroepiandrosterone. Thus the androgens were felt be predominantly of ovarian origin. Dehydroepiandrosterone rose to low normal levels by 18 months after discontinuation of hydrocortisone. We thus demonstrate for the first time that both the adrenal glomerulosa and fasciculata have the capacity to recover normal function following treatment with o,p'-DDD. Further, we suggest that early exposure to excess adrenal androgens may result in mild alteration of gonadal function.

Adenocarcinoma↗

Rhabdomyosarcoma.

Explore the source record for details and available documents.

Adolescent↗

A prospective randomized clinical trial of total parenteral nutrition in children with cancer.

A prospective randomized clinical trial was undertaken to test the efficacy of total parenteral nutrition (TPN) among previously untreated children receiving abdominal/pelvic irradiation with or without adjuvant chemotherapy who were at risk for weight loss, malnutrition, and complications from treatment. Children were evaluated by weight/height determinations, anthropomorphic measurements, and laboratory studies. TPN was associated with an improved nutritional status during therapy as compared with control patients on ad libitum intake. However, when TPN was discontinued, weight declined and there were no differences among treated and control patients detected at three-month follow-up. Likewise there was no obvious effect from TPN on tolerance to therapy in the adequately nourished child. TPN as initial supportive therapy should be reserved for those children who are malnourished or marginally malnourished at the time of presentation. Close nutritional assessment during treatment is essential since approximately 25% of children undergoing abdominal/pelvic radiotherapy with chemotherapy can be expected to become malnourished during an initial course of therapy.

Adolescent↗

Immediate and long-term posttherapy neuropsychologic performance in children with acute lymphoblastic leukemia treated without central nervous system radiation.

Intellectual and neuropsychologic test performance was studied in 41 children with acute lymphoblastic leukemia within one year of completing multidrug therapy in which intrathecal methotrexate was the sole agent of central nervous system prophylaxis. To control for possible effects of intensive systemic chemotherapy and the disruptive psychosocial effects of treatment on psychometric test function, 33 children with embryonal rhabdomyosarcoma who had no CNS disease or treatment were similarly studied. All patients studied had been in continuous complete remission since diagnosis. Within each diagnostic group, patients were placed in a younger (less than 8 years) or an older (more than 8 years) group depending on age at the time of testing. No significant difference was found in intellectual function between the young ALL and ERMS groups or between the total ALL and ERMS groups. Further five-year follow-up of 12 of the youngest ALL patients showed no significant long-term change in intellectual or other specific neuropsychologic functions. Children with ALL who remained in continuous complete remission from diagnosis and who were treated with prophylactic intrathecal methotrexate and no CNS radiation did not have global or specific neuropsychologic impairment.

Adolescent↗

Comparison of morbidity in children requiring abdominal radiation and chemotherapy, with and without total parenteral nutrition.

We evaluated the effectiveness of total parenteral nutrition and placing the "bowel at rest," as compared to that of ad libitum food intake, on nutritional status and tolerance to combined chemotherapy and radiotherapy in a randomized, prospective trial in children with previously untreated malignancy requiring abdominal and pelvic irradiation and chemotherapy. Administration of TPN was found to be safe and efficacious in maintaining the children in good nutritional status during combined therapy; one-third of the control patients became malnourished and required TPN. There was no beneficial effect of "bowel at rest" and TPN on the ability of patients to tolerate combined therapies in terms of decreased toxicity; however, use of TPN was associated with improved adherence to chemotherapy schedules. Following termination of TPN or ad libitum food intake, and while receiving chemotherapy, the majority of the children who had previously received TPN lost significant weight. To date there has been no difference in mortality rate between the control and TPN groups. Although we conclude that TPN per se had little beneficial effect beyond that of maintaining good nutritional status, every child undergoing intensive combined therapy should have early and periodic assessments of nutritional status, so that the early signs of malnutrition can be detected, and the adverse effects of malnutrition can be prevented by nutritional replenishment, by TPN, or by other methods.

Abdominal Neoplasms↗

Dural sinus thrombosis in children with acute lymphoblastic leukemia.

Dural sinus thrombosis developed in two children with acute lymphoblastic leukemia during induction treatment with vincristine sulfate, prednisone, and asparaginase. Headache, nausea, emesis, and lethargy were the presenting signs. The diagnosis was confirmed by arteriography. The cause is presumed to be secondary to hypercoagulability due to asparaginase-induced antithrombin III deficiency. The patients received anticoagulation therapy and recovered completely. Only two of the six reported patients without heparinization survived.

Age Factors↗

Multidisciplinary treatment of advanced stages of embryonal rhabdomyosarcoma in children.

Twenty-eight previously untreated patients with embryonal rhabdomyosarcoma, stages II to IV, were treated with the T-6 induction protocol and maintained on the T-2 protocol. The T-6 protocol consisted of simultaneous administration of drugs and irradiation, given in 3 parts each separated by an obligatory period of rest. Twenty-four of the 28 patients (85.7%) survived 4 to 44 months, with a median follow-up of 24 months; 18 (64%) have been in continuous disease-free status. The immediate toxicity of the T-6 induction was severe and required intensive supportive care. To date, the results from the T-6 induction protocol were more effective than those achieved with the T-2 protocol.

Adolescent↗