Biomedical subjects

M Tefft

Publications and source records attributed to M Tefft.

At least 37 records · Page 2Linked to original sources

PubMed1990

Partial cystectomy in the management of rhabdomyosarcoma of the bladder: a report from the Intergroup Rhabdomyosarcoma Study.

Among 154 children with primary vesical rhabdomyosarcoma entered in the IRS (1972 to 1986), tumor excision constituting a partial cystectomy was performed in 33. These procedures were performed in the following situations (1) as an initial operation for localized disease (22); (2) as a secondary procedure for localized disease following chemotherapy (CT) or CT/irradiation response (6); and (3) as an initial operation in the presence of disseminated abdominal disease (5). Partial cystectomy included full-thickness resection of 15% to 80% of the bladder wall. Ureters were reimplanted or revised in two patients. The 33 patients undergoing partial cystectomy were in the following clinical groups: I, total excision (10); II, gross excision with "microscopic residual" or positive nodes (8); III, subtotal excision (10); and IV, tumor dissemination (5). Patients in clinical groups I and II received vincristine and actinomycin-D (VA) or VA + cyclophosphamide (VAC) +/- adriamycin (ADR). Patients in groups III and IV received the same agents +/- cisplatin. Irradiation (2,000 to 5,000 cGy) was administered to 18 patients (55%) in groups II to IV. Six relapses occurred among 28 patients with localized disease, and one among five patients with dissemination; all resulting in progressive disease and/or death. Estimated 3-year survival following partial cystectomy (79%) is similar to that for all patients with primary bladder tumors (78%). In contrast with the total group, in which the majority of survivors have eventually required total cystectomy; 25 of 26 survivors of partial cystectomy have functional bladders from 146 to 686 weeks (median, 356 weeks) from study entry.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

PubMed1990

Multimodal therapy for the management of primary, nonmetastatic Ewing's sarcoma of bone: a long-term follow-up of the First Intergroup study.

A total of 342 previously untreated eligible children were entered into the first Intergroup Ewing's Sarcoma Study (IESS) between May 1973 and November 1978. In group I institutions, patients were randomized between treatment 1 (radiotherapy to primary lesion plus cyclophosphamide, vincristine, dactinomycin, and Adriamycin [doxorubicin; Adria Laboratories, Columbus, OH] [VAC plus ADR]) or treatment 2 (same as treatment 1 without ADR), and group II institutions randomized patients between treatment 2 or treatment 3 (same as treatment 2 plus bilateral pulmonary radiotherapy [VAC plus BPR]). The percentages of patients relapse-free and surviving (RFS) at 5 years for treatments 1, 2, and 3 were 60%, 24%, and 44%, respectively. There was strong statistical evidence of a significant advantage in RFS for treatment 1 (VAC plus ADR) versus 2 (VAC alone) (P less than .001) and 3 (P less than .05) and also of treatment 3 versus 2 (P less than .001). Similar significant results were observed with respect to overall survival. Patients with disease at pelvic sites have significantly poorer survival at 5 years than those with disease at nonpelvic sites (34% v 57%; P less than .001). Among pelvic cases, there was no evidence of differing survival by treatment (P = .81), but among nonpelvic cases, there was strong evidence of differing survival by treatment (P less than .001). The overall percentage of patients developing metastatic disease was 44%; the percentages by treatments 1, 2, and 3 were 30%, 72%, and 42%, respectively. The overall incidence of local recurrence was 15%, and there was no evidence that local recurrence rate differed by treatment. Patient characteristics related to prognosis, both with respect to RFS and overall survival experience, were primary site (nonpelvic patients were most favorable) and patient age (younger patients were more favorable).

Adolescent↗

PubMed1990

Multimodal therapy for the management of nonpelvic, localized Ewing's sarcoma of bone: intergroup study IESS-II.

Two hundred fourteen eligible patients with previously untreated, localized Ewing's sarcoma of bone were randomized on IESS-II to receive Adriamycin (ADR; doxorubicin; Adria Laboratories, Columbus, OH), cyclophosphamide, vincristine, and dactinomycin by either a high-dose intermittent method (treatment [trt] 1) or a moderate-dose continuous method (trt 2) similar to the four-drug arm of IESS-I. Patient characteristics (sex, primary site, type of surgery) were stratified at the time of registration; these and other patient characteristics (age, time from symptoms to diagnosis, race) were distributed similarly between treatments. Surgical resection was encouraged, but not mandatory. Local radiation therapy was the same as for IESS-I. The median follow-up time is 5.6 years. The overall outcome was significantly better on trt 1 than on trt 2. At 5 years, the estimated percentages of patients who were disease-free, relapse-free, and surviving were 68%, 73%, and 77% for trt 1 and 48%, 56%, and 63% for trt 2 (P = .02, .03, and .05, respectively). The major reason for treatment failure for both treatment groups was the development of metastatic disease. The lung was the most common site of metastases followed by bone sites. The combined incidence of severe or worse toxicity (67%) was comparable between the treatments; however, severe or worse cardiovascular toxicity was significantly greater on trt 1. Tne only treatment-associated deaths (N = 3) were on trt 1 and were cardiac-related.

Adolescent↗

PubMed1989

Results of intensive therapy in children with localized alveolar extremity rhabdomyosarcoma: a report from the Intergroup Rhabdomyosarcoma Study.

Preliminary analyses of treatment results and prognostic factors from the first Intergroup Rhabdomyosarcoma Study (IRS I) suggested that alveolar histology and extremity site were among factors associated with an adverse outcome in clinical group I patients whose tumors had been completely resected. A high incidence of alveolar histology among the extremity tumors compounded the problem. These findings prompted an amendment to IRS II shortly after the study opened. Therapy for patients with alveolar extremity tumors in groups I and II was changed to more intensive repetitive pulse vincristine, dactinomycin, and cyclophosphamide (VAC) for 2 years. The outcome of 44 patients who received intensive therapy on IRS II is compared with a control series of 30 patients who were treated on IRS I. The control patients received standard VAC therapy for 2 years or VA for 1 year. Half of group I control patients received local radiotherapy, whereas none was given to group I intensive therapy patients. In both studies group II patients received radiotherapy to the primary tumor site and to identified positive regional lymph nodes. Compared with the control group, patients given more intensive therapy had a longer disease-free survival (DFS) (69% v 43% at 3 years), but this was of marginal significance (P = .06). The overall survival difference (77% v 57% at 3 years) was not significant (P = .23). Despite the limited statistical evidence, there is some indication that intensive therapy improved the prognosis of children with localized alveolar rhabdomyosarcoma (RMS).

Antineoplastic Combined Chemotherapy Protocols↗

PubMed1988

Disease patterns and survival rate in children with metastatic soft-tissue sarcoma. A report from the Intergroup Rhabdomyosarcoma Study (IRS)-I.

The patterns of disease extent, response to treatment, and survival rates were examined in 129 children registered as metastatic (Group IV) soft-tissue sarcoma patients on the Intergroup Rhabdomyosarcoma Study (IRS)-I protocol from November 1972 through October 1978. The patients' ages at diagnosis ranged from 3 months to 21 years (median, 10.5 years); 72 were male patients and 57 were female patients (male-to-female ratio, 1.3:1). The tumor arose in an extremity in 34 patients, the head and neck (exclusive of the orbit) in 27, the genitourinary tract in 25, the retroperitoneum or pelvis in 14, the trunk in 12, the intrathoracic region in nine, the hepatobiliary system in four, the perineum in two, and the orbit in two patients. The histologic types of soft-tissue sarcoma confirmed by the pathology review committee were embryonal rhabdomyosarcoma in 68 patients, alveolar rhabdomyosarcoma in 41, undifferentiated sarcoma in 14, embryonal-botryoid in two, pleomorphic in one patient, and other sarcoma in three patients. Of 105 patients who were confirmed in Group IV and had complete information concerning sites of metastatic deposits, 53 patients presented with metastases restricted to only one anatomic region or tissue; the other 52 had diffuse metastases. The most common sites of distant tumor deposits were the lungs, bone marrow, and other soft tissues. After treatment with vincristine, dactinomycin, and cyclophosphamide with or without Adriamycin (Adria Laboratories, Inc., Columbus, OH) and radiation therapy, 65 (50%) of the 129 eligible patients achieved complete disappearance of all detectable tumor. The likelihood of achieving and maintaining a complete response for 2 years was highest among patients whose tumors originated in the genitourinary tract, retroperitoneum-pelvis, or the hepatobiliary or perineum regions. Thirteen of these 45 children (29%) are alive and disease-free at a minimum of 2 years after initiation of treatment. Only 10 of the other 84 (8%) are surviving disease-free at 2 years (P = 0.02). The presence of restricted rather than diffuse metastases at diagnosis (P = 0.02) was the only other characteristic significantly related to long-term complete remissions. One hundred and two patients (79%) died at a median of 47 weeks after diagnosis, 92 from sarcoma and ten from other causes.(ABSTRACT TRUNCATED AT 400 WORDS)

Adolescent↗

PubMed1988

Clinical staging and treatment results in rhabdomyosarcoma of the female genital tract among children and adolescents.

From 1972 to 1984, 47 children and adolescents with primary tumors of the female genital tract were treated with eight different Intergroup Rhabdomyosarcoma Study (IRS I-II) protocols. These included patients with vaginal (28), uterine (ten), or vulval (nine) rhabdomyosarcoma or undifferentiated sarcomas. The mean age of patients with primary vaginal tumors was younger than 2 years. All were of the embryonal histologic subtype. The majority of these patients were treated with initial chemotherapy (vincristine, dactinomycin, +/- cyclophosphamide, +/- Adriamycin [(ADR) doxorubicin]) followed by delayed hysterectomy and/or partial vaginectomy. Among 26 patients with localized vaginal tumors, there have been six relapses resulting in one tumor-related death and one therapy-related death. The five patients with nonfatal relapse have been disease-free for from 2.5 to 6.5 years (mean, 4.4 years) since salvage therapy was commenced. The 19 patients without relapse have been continuously disease-free for 1.5 to 12 years (mean, 5.34 years: median, 6 years). No patient that received ADR initially relapsed. Ten patients, with a mean age greater than 13 years, had primary uterine sarcomas and were treated by the same chemotherapy regimens. Six with polypoid localized lesions, removed before chemotherapy, have remained disease-free for 2.5 to 6.5 years. Four, with more extensive local lesions or disseminated disease, treated with chemotherapy only, died 2 to 11 months from diagnosis. Nine patients with rhabdomyosarcomas of the vulva (age, 1-19 years) were managed by the same chemotherapy regimens +/- radiotherapy. Resection was carried out initially or after chemotherapy. Eight of these have been disease-free from 4 to 10 years (mean, 6.4 years); and one is alive with probable disease at 2.5 years.

Adolescent↗

PubMed1988

Melphalan and prednisone plus total bone marrow irradiation as initial treatment for multiple myeloma.

Patients who have received radiation to localized areas of marrow eventually regenerate marrow in the irradiated area, if the dose is 2,400 centigrays (cGy) or less. This trial was designed to deliver a radiation dose of 1500 cGy to all marrow containing sites in patients with multiple myeloma, a technique we refer to as total bone marrow irradiation, or TBMI. Patients with previously untreated myeloma received 12 weeks of melphalan (L-PAM) and prednisone (pred) therapy. Four weeks later, sequential irradiation was administered using the 3-2 technique with rest periods to permit recovery from radiation-induced cytopenia. This was followed by electron beam irradiation of the rib and skull fields. Following completion of TBMI, patients were untreated until relapse. Twenty patients were entered. At entry 5, 8, and 7 patients had low, intermediate and high tumor cell loads, respectively. Two patients had a serum Ca in excess of 12 mg/dl; 3 had an increased creatinine. The median performance (ECOG) was 1. At week 16, immediately prior to TBMI, 5 of the 20 patients fulfilled the Myeloma Task Force criteria for response and 5 others had improved. Six patients did not begin the radiation therapy portion of the protocol. Three had rapidly progressive disease, one persistent leukopenia, one refused radiation therapy and one was withdrawn by his physician. Only 6 of the fourteen patients receiving the radiation treatment phase of the protocol were able to tolerate the intended course of 1500 cGy to all areas. Eight other patients received lower doses. Patients completing the radiation phase of the protocol failed to have further reductions in M-protein or improvement in other parameters beyond those obtained on the chemotherapy phase of the protocol. The median duration of response and survival was 12.0 and 42 months, respectively. We suggest possible reasons for the disappointing results of this trial and conclude that this approach to the primary treatment of myeloma holds little promise.

Adult↗

PubMed1988

Advanced head and neck cancer: response to and toxicity of multimodality therapy.

This pilot study for resectable stage III and stage IV squamous cell carcinoma of the head and neck used a cytoreduction phase of preoperative radiation with cisplatin, followed by an eradicative treatment phase with radical surgery (group 1) or radical dose radiation and cisplatin (group 2), followed by adjuvant chemotherapy with 5-fluorouracil infusion and cisplatin delivered at 4-week intervals for six cycles following initial radiation therapy to the primary site. A total of 43 patients were treated between January 1984 and January 1987; 14 were classified with stage III carcinoma, 28 with stage IV, and one patient was not staged. Out of 43 patients, two did not complete therapy. Forty-one patients completed the eradicative phase of treatment. Complete tumor clearance at the end of the eradicative treatment phase was 88% (36 of 41 patients), 95% (18 of 19) in group 1 and 82% (18 of 22) in group 2. Actuarial recurrence-free survival was 61% at 3 years. Among 36 patients with complete tumor clearance after the eradicative treatment phase, there was no statistically significant difference for overall and recurrence-free survival between group 1 and group 2. In general, toxicity was not excessive, although mucositis, weight loss, and hematologic and neurologic toxicity were observed in varying degrees in these patients.

Adult↗

PubMed1988

Acute toxicities associated with radiation in the second National Wilms' Tumor Study.

Twenty-three of 303 patients (7.6%) entered into National Wilms' Tumor Study Number 2 (NWTS-2) with groups II, III, and IV disease experienced 26 toxic events thought to be related to radiotherapy (RT). The randomization between vincristine (VCR) plus dactinomycin (AMD) and the same two drugs plus doxorubicin (ADR) had a minimal effect on RT toxicity. Five (1.6%) fatalities were recorded and the surviving 18 patients recovered without discernible residua. Sixteen patients had hepatic, four had pulmonary, and three had cardiac toxicity. Hepatic toxicity was significantly more common when the right side of the abdomen or the whole abdomen was irradiated than when the left side was treated (P = .01). We conclude that acute RT-related toxicity in NWTS-2 was very similar to that in NWTS-1, in which 26 (7.2%) of 359 randomized patients developed RT-related toxicity and three died. This acceptable rate was maintained despite more intensive chemotherapy (CT) in NWTS-2.

Antineoplastic Combined Chemotherapy Protocols↗

PubMed1987

Paratesticular sarcoma in childhood and adolescence. A report from the Intergroup Rhabdomyosarcoma Studies I and II, 1973-1983.

This article reports on the diagnostic features, radiographic findings, staging, operative management, treatments, and treatment results for 95 children (mean age, 10 years) with paratesticular sarcoma; they were treated on the Intergroup Rhabdomyosarcoma Study (IRS) I and II protocols. Embryonal rhabdomyosarcoma (RMS) was the most common histologic subtype (97% of cases). Patients were randomly assigned to receive various therapeutic regimens according to IRS protocols. Among 81 patients (85%) with localized disease, most (57) were in Clinical Group I (localized, completely excised tumors), and 20 were in Group II (gross excision with tumor-involved, regional retroperitoneal lymph nodes or microscopic residual). Only four were in Group III (gross residual disease in the primary site or retroperitoneal lymph nodes). Three of them achieved a complete response (CR) after induction therapy and two then relapsed. The relapse-free survival (RFS) estimates at 3 years from diagnosis were 93% in Group I and 90% in Group II. Distant metastases were present at diagnosis in only 14 patients. Twelve of them achieved CR, but four subsequently relapsed; their 3-year RFS estimate was 67%. Overall, survival rates among the 95 children were excellent (89% at 3 years) compared to the entire IRS series (63%). Treatment with radical orchidectomy and chemotherapy was sufficient for Group I patients. Orchidectomy, chemotherapy, and radiation therapy were highly effective in Group II patients and in a large proportion of those with more advanced tumors. Recommendations for the diagnostic examinations and management of future patients with paratesticular sarcoma are given.

Adolescent↗

PubMed1987

Lymphatic metastases with childhood rhabdomyosarcoma. A report from the Intergroup Rhabdomyosarcoma Study.

A review of 1415 patients without distant metastasis from the Intergroup Rhabdomyosarcoma Study (IRS) I and II revealed an overall 10% incidence of identified lymphatic spread at diagnosis, whereas 81 of 592 children with localized rhabdomyosarcoma who had grossly complete resection (and therefore with more complete pathologic data) had histologically proven lymphatic spread (14%). The percentage of patients in this latter group with nodal metastases was highest for the prostate (41%), paratesticular sites (26%), and genitourinary sites overall (24%). Sites with a small percentage of proven lymphatic involvement were the orbit (0%), nonorbital head and neck sites (7%), and truncal sites (3%), whereas the percentage of patients with nodal metastases from extremity lesions was 12%. The primary tumor mean diameter was significantly larger in the group with nodal metastases, but there was no evidence of a relationship between lymphatic spread and age, sex, or histologic subtype. Patients with lymph node metastases who had resection had a poorer survival rate (logrank P value = 0.001), with a 3-year survival estimate of 54%, compared with 78% for patients without lymphatic metastases. Patients with extremity lesions and positive lymph nodes also did poorly when compared with patients with normal nodes (P = 0.006), and a similar observation was made for patients with paratesticular sarcoma (P = 0.06).

Actuarial Analysis↗

PubMed1987

Improved prognosis with intensive treatment of children with cranial soft tissue sarcomas arising in nonorbital parameningeal sites. A report from the Intergroup Rhabdomyosarcoma Study.

In the first Intergroup Rhabdomyosarcoma Study (IRS-I), it was found that, of 57 patients with nonorbital cranial parameningeal sarcoma treated from 1972 to 1976, 20 patients (35%) developed meningeal sarcoma and 90% (18/20) died of this complication. Since about half of these 20 patients had received inadequate radiation therapy and all had radiation therapy delayed until week 6, the treatment program was modified in December 1977 to begin radiation therapy on day 0 to the entire neuraxis and the primary tumor for all patients presenting with one or more risk factors indicating a high risk of meningeal involvement, and to administer periodic intrathecal medications to the same patients. The risk factors were intracranial extension of tumor, bony erosion at the cranial base, and cranial nerve palsy. In this report, the prognoses of two groups of patients with nonorbital cranial parameningeal sarcoma are compared: 95 patients treated before modification of the protocol in December 1977 with chemotherapy and nonintensive meningeal radiotherapy (preintensive group) and 68 patients treated subsequently with intensive meningeal radiotherapy and chemotherapy (intensive group). All patients received vincristine and actinomycin D (dactinomycin) with or without cyclophosphamide and Adriamycin (doxorubicin), according to the protocol. Radiation therapy consisted of 4000 to 5500 rad to the primary tumor in 5 to 6 weeks for most patients. The intensively treated patients also received intrathecal drugs via lumbar puncture and whole cranial or craniospinal radiation when one or more meningeal risk factors were present. In the preintensive group, the complete remission rate was 68% (65/95); the percentages of patients tumor free or alive at 3 years were 33% and 41%, respectively. In intensively treated patients, the complete remission rate was 76% (52/68); the percentages of patients tumor free or alive at 3 years were 57% and 68%, respectively. The improvements in the percentages of intensively treated patients tumor free or surviving were highly statistically significant (P less than 0.01 for both comparisons). Within the intensive treatment group, meningeal involvement at diagnosis was an unfavorable prognostic sign, since patients with no meningeal involvement had much better tumor-free survival at 3 years (81% vs. 51%) and survival (90% vs. 57%) than patients with evidence of meningeal involvement; the differences were highly statistically significant (P = 0.01).

Antineoplastic Combined Chemotherapy Protocols↗

PubMed1987

Pediatric orofacial and laryngopharyngeal rhabdomyosarcoma. An Intergroup Rhabdomyosarcoma Study report.

Eighty-nine children with localized rhabdomyosarcoma of orofacial and laryngopharyngeal sites were treated in accordance with the first and second Intergroup Rhabdomyosarcoma Study (IRS) protocols (IRS-I and IRS-II) between 1972 and 1984. Treatment included surgery (or biopsy) and chemotherapy for all patients and radiotherapy in the majority. The actuarial estimate of the three-year survival rate for all patients was 83% and did not differ significantly by primary site, histologic findings, or presence of adenopathy. A trend for a worse survival rate was seen in clinical group III patients and in those less than 5 years of age at diagnosis. Factors associated with an increased risk of local/regional relapse included omission of radiotherapy and a radiation dose of less than 40 Gy (4000 rad). We conclude that treatment of these patients as recommended in the IRS-I and IRS-II protocols results in very good local and regional tumor control and survival rates. Salvage therapy for local/regional recurrence may yield long-term remission and possibly cure.

Adolescent↗

PubMed1987

Localized orbital rhabdomyosarcoma. An interim report of the Intergroup Rhabdomyosarcoma Study Committee.

Children with rhabdomyosarcoma (RMS) (1461 total cases) were included in two prospective, randomized protocols conducted by the Intergroup Rhabdomyosarcoma Study (IRS) Committee from November 1972 to June 1983. Treatment assignment was determined by extent of tumor at diagnosis (clinicopathologic group) and not by primary site. Accordingly, 127 patients with RMS confined to the region of the eyelid and orbit were treated by a variety of regimens characterized by initial partial tumor resection followed by concurrent multiagent chemotherapy and orbital radiation therapy. Recurrent tumor developed in ten patients. Seven relapsed initially in the orbit (of whom 4 were salvaged) and three in regional nodes. In addition to the six patients who died of tumor, three died of other causes for a 93% 3-year actuarial survival rate. In 89 children who had subtotal resection or biopsy only, the local control rate with radiotherapy and chemotherapy was 94%.

Adolescent↗

PubMed1986

Infants younger than 1 year of age with rhabdomyosarcoma.

Of a total of 1561 patients registered in the Intergroup Rhabdomyosarcoma Study (IRS) as of May 1983, 78 patients (5%) were younger than 1 year of age. These infants did not differ from the older children (1-20 years of age) in male/female ratio, clinical grouping, or survival rates. Infants younger than 1 year of age had a significantly greater frequency of undifferentiated sarcoma, 18% versus 7% in older children (P less than 0.005). Infants also had a significantly greater proportion of cancers with botryoid pathology, 10% versus 4% in older children (P less than 0.005). When reviewed by the newly proposed IRS cytopathologic classification, there was no difference in pathologic types between the two age groups. Infants younger than 1 year of age had a higher rate of bladder-prostate-vagina primary tumor sites than older children, 24% versus 10% (P less than 0.05). In addition, infants tended to receive less of the prescribed doses of chemotherapy and radiation therapy than older children, and to develop more toxicity to treatment than older children. Despite these differences, the overall survival curve for the two age groups appears to be similar. In contrast to Wilms' tumor and neuroblastoma, in which age (less than 1 year) is a favorable prognostic factor, age does not appear to be an important prognostic factor in rhabdomyosarcoma.

Antineoplastic Agents↗

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↗