Unusual extraskeletal myxoid chondrosarcoma in a child.
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Adolescent↗
Biomedical subjects
F Flamant
Publications and source records attributed to F Flamant.
Long-term sequelae of conservative treatment by surgery, brachytherapy, and chemotherapy for vulval and vaginal rhabdomyosarcoma in children.
Between 1970 and 1978, 17 girls with rhabdomyosarcoma (RMS) of the vulva or vagina were treated at the Institut Gustave-Roussy (IGR) by conservative treatment including surgery, brachytherapy, and chemotherapy. Twelve pubescent or postpubescent girls were studied for long-term sequelae. Eleven of 12 patients have had a normal puberty, two have a total of three healthy children, 11 have normal menses, and 10 normal menarche (one after hormonal replacement). Only one patient underwent hysterectomy following low-dose brachytherapy. Five girls have no vaginal complications, and three are sexually active. Three have had minimal vaginal sequelae, which required surgical correction to permit sexual intercourse. Four girls sustained serious sequelae (colorectal, vaginal, urethral, and ureteral stenosis). These sequelae, secondary to irradiation, are potentially avoidable in the future given the current advances in brachytherapy and improvements in dosimetry. This conservative treatment is useful and appropriate for girls with RMS of vulvar or vaginal origin when complete remission cannot be obtained with chemotherapy and partial colpectomy.
Adolescent↗
Yolk sac tumor in aberrant localization.
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Antineoplastic Combined Chemotherapy Protocols↗
[Neonatal Pepper's syndrome. Apropos of a series of 22 cases].
From 1960 to 1987, 22 neonates were treated at the Institut Gustave-Roussy for a Pepper' syndrome. During this period of time, 83 children were treated for the same syndrome, the neonatal type representing 26% of cases. Twelve of the 22 neonates presented with the rapidly evolving type, requiring an immediate aggressive treatment. In 4 of them death occurred early despite treatment. The 10 other neonates presented with a slowly progressive disease. In 5 of them treatment of the metastatic disease was not necessary to obtain remission. The survival rate of disease without relapses in 77%, with a median follow-up of 8 years. So, despite the high incidence of rapidly progressive forms, the prognosis of this disease remains favorable in neonates, provided an accurate treatment initiated early.
Adrenal Gland Neoplasms↗
An attempt to use a common staging system in rhabdomyosarcoma: a report of an international workshop initiated by the International Society of Pediatric Oncology (SIOP).
One hundred medical records of children with rhabdomyosarcoma were reviewed independently by ten European and American specialists primarily concerned with pediatric cancer patients. Each specialist assessed the stage of disease using a pre-treatment staging system (International Society of Pediatric Oncology-Union Internationale Contre le Cancer TNM [SIOP-UICC TNM] and a post-surgical staging system (Inter-group Rhabdomyosarcoma [IRS] grouping system). These two prognostic systems are based on different criteria, and there is no simple correspondence between them. The reliability of the two systems, based on patients' characteristics extracted from medical records, was similar and satisfactory (Kappa index: 0.78 and 0.82 with the TNM system and with the IRS system, respectively). Nevertheless, the IRS grouping system, which considers a primary method of treatment: Surgery, is dependent upon the surgeon, the institution, and the current concept of treatment. We believe that the TNM system provides the best description of extent of disease and should be used for determining therapeutic options and comparing therapeutic results.
Child↗
Histopathological classification of childhood rhabdomyosarcoma: a report from the International Society of Pediatric Oncology pathology panel.
Five hundred thirteen soft tissue tumours of childhood referred to the International Society of Pediatric Oncology (SIOP) Rhabdomyosarcoma study have been reviewed. The period covered was from January 1975 to December 1983. Three hundred thirty-nine neoplasms were regarded as embryonal rhabdomyosarcoma. The histological diagnosis of all referred tumours is given, and a classification of rhabdomyosarcomas developed during the course of the study is described. The relationship between histological subtypes and aspects of clinical behaviour is presented. The classification is thought to be helpful in diagnosis and also in assessment of the likelihood of recurrence and/or metastasis of childhood rhabdomyosarcoma.
Adolescent↗
Rhabdomyosarcoma in infants under one year of age: experience of the Institut Gustave-Roussy.
Forty-three infants under 1 year of age (median 4 months) with rhabdomyosarcoma (RMS), representing 11% of all childhood RMS seen over the same period, were treated and followed between 1955 and 1984 at our institute. Compared to the 340 older children (1-16 years of age), there were no significant differences in male/female ratio, primary site, or clinical stage, but there was a higher frequency of alveolar and poorly differentiated histologic subtypes in infants aged under 1 year (P = 0.05 and P less than 0.001, respectively) and increased frequency of botryoid RMS in older children (P less than 0.02). Although children older than 1 year of age had a significantly higher complete remission rate (83% vs. 51%, P less than 0.001), their 5 year overall survival (43%) was no different from that of infants under 1 year of age (35%). In the older group there was no survival difference between alveolar (38%) and nonalveolar (44%) histologic subtypes, but in infants younger than 1 year, alveolar subtype had a significantly poorer prognosis (13% vs. 47%, P = 0.01). Management of patients at this age group is difficult and major advances are needed to increase survival rates with a minimum of morbidity.
Adolescent↗
Treatment of rhabdomyosarcoma and other malignant mesenchymal tumours of childhood with ifosfamide + vincristine + dactinomycin (IVA) as front-line therapy (a SIOP study).
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Antineoplastic Combined Chemotherapy Protocols↗
Conservative treatment for lower gynecological tract malignancies in children and adolescents: the Institut Gustave-Roussy experience.
Between 1972 and 1986, 37 patients with lower genital tract malignancies were treated with intracavitary or interstitial brachytherapy. Thirteen patients presented with clear cell adenocarcinoma, 14 patients with embryonal rhabdomyosarcoma, 6 patients with endodermal sinus tumor, 3 patients with sarcoma, and 1 patient with an undifferentiated tumor. FIGO classification was: Stage I, 16%; Stage II, 47%; and Stage III, 37%. Treatment policy included initial exploratory laparotomy with lymph node biopsy and ovarian transposition, chemotherapy (except in clear cell adenocarcinoma) and/or external radiotherapy prior to interstitial brachytherapy. Chemotherapy consisted of a combination of VAC-Ad (V = vincristine, A = D actinomycin, C = cyclophosphamide, Ad = adriamycin) in rhabdomyosarcoma and sarcomas, and MAC-Ad (M = methotrexate) in endodermal sinus tumor. External radiotherapy was used in seven patients: in one to reduce a bulky clear cell adenocarcinoma (20 Gy) and in six for pelvic nodal involvement (45 Gy). Brachytherapy techniques depended on tumor site and extent, and on the anatomy of the patients. Vulvar tumors were implanted with iridium-192 wires by an afterloading plastic tube technique. Cervical and vaginal tumors were treated with individually tailored moulded vaginal applicators loaded with either cesium-137 or iridium-192, with or without interstitial implants by plastic tube or guide gutter technique. Computerized dosimetry allowed calculation of treatment volumes and doses delivered on the tumor and adjacent critical organs. The prescribed dose (including external radiotherapy) was 60-75 Gy with 1-3 brachytherapy applications of a low dose rate (0.2 Gy/hr). Six patients are dead: one from chemotherapy complication, three of metastases (two sarcomas, one endodermal sinus tumor) and two of pelvic failures and metastases (two clear cell adenocarcinoma). The overall disease free 5-year survival is 72%. Actuarial 5-year local control is 84%, but including salvage is 94%: three (two rhabdomyosarcoma, one clear cell adenocarcinoma) of the five local failures were salvaged by surgery, chemotherapy and/or brachytherapy. Metastases occurred in six patients, one (sarcoma) salvaged by chemotherapy and external radiotherapy. Complications requiring surgery occurred in five patients: two hydronephroses, one urethral stricture, one ileo-cecal obstruction, and one vesicovaginal fistula. Twelve of the 17 patients (71%) over 12 years of age are normally menstruating. Two patients have produced three normal children. This multidisciplinary management of lower gynecological tract tumors including brachytherapy is both conservative and effective.
Adenocarcinoma↗
Hemorrhagic cystitis following high-dose chemotherapy and bone marrow transplantation in children with malignancies: incidence, clinical course, and outcome.
Two hundred ninety-one courses of high-dose chemotherapy (HDC) with bone marrow transplantation (BMT) in children with malignancies were reviewed in order to assess the incidence, clinical course, outcome, and predisposing factors of hemorrhagic cystitis. Hemorrhagic cystitis occurred in 19 HDC courses (6.5%). Three patients had grade I hematuria linked to thrombopenia, nine had grade II hematuria despite platelet levels greater than 50 x 10(9)/L, and seven had grade III hematuria with clots and bladder obstruction. Severe complications occurred in grade III patients, but no deaths were directly linked to the cystitis. Fourteen patients recovered within two to 120 days of onset. The other patients died before the cystitis resolved, either of a relapse of the malignancy or of infection. Predisposing factors were age (increased incidence in older children), conditioning regimen containing cyclophosphamide, previous vesical irradiation, association with prolonged aplasia, and hepatic complications. The role of busulfan was also probable. No viral agent was found.
Adolescent↗
Male gonadal function after chemotherapy for solid tumors in childhood.
The testicular function of 30 adolescent or adult males having undergone polychemotherapy in childhood was assessed by means of a spermogram or testicular biopsy. At the time of examination, the patients were pubertal and had completed chemotherapy between 1 and 20 years previously (mean, 9 years). All patients who were prepubertal or intrapubertal at the time of treatment achieved normal puberty with normal growth. Twenty patients presented with azoospermia and/or severe disturbances in the germinal line on biopsy. This series confirms the toxicity of alkylating agents, in particular that of the mechlorethamine, vincristine, procarbazine, and prednisone combination (MOPP) and that of cyclophosphamide (CPM). However, dactinomycin, vinblastine, and vincristine did not appear to have a toxic effect on spermatogenesis. The prepubertal state did not protect the gonads of 19 patients who were prepubertal at diagnosis: 12 are now sterile as a result of the treatment. An increase in basal follicle-stimulating hormone (FSH) levels gives a good indication of testicular damage, although normal levels do not rule out the possibility of azoospermia.
Adolescent↗
[Immunohistochemical study of 20 synovial sarcomas in children].
Histopathologic study of 20 cases of synovial sarcoma (S.S.) in children has been conducted to evaluate the usefulness of immunohistochemistry in the diagnostic assessment of this tumor. Two of the 6 antibodies studied have been found to be the most informative: staining for keratin and vimentin proteins has been observed in most cases, outlining the epithelial and sarcomatous components of this tumor. The results are in accordance with those already described for S.S. in adults. The presence of cytokeratins in S.S. is discordant with classical histogenetic conceptions.
Child↗
[Clinical, anatomo-pathologic aspects and therapeutic results in 63 malignant ORL non-Hodgkin's lymphomas in children].
Sixty-three children with non Hodgkin's lymphoma involving the ENT zone (Waldeyer's ring) were treated between 1975 and 1985 at the Institut Gustave-Roussy. The tumors were localized in the cavum (32 cases), tonsils (16 cases), mandibles (9 cases) whereas 6 patients had more than one of these sites involved. At the time of diagnosis, 16 (25%) had neuro-meningeal involvement. Distribution, according to stages and histology was as follows: 4 stages I, 27 stages II, 12 stages III, 17 stages IV and 3 were unclassifiable. A Burkitt type of lymphoma was diagnosed in 37 (58%), a convoluted lymphoblastic type in 6 (10%) and 20 (32%) fell in a broad category of "other lymphoblastic variety" (32%). With regard to therapy, two consecutive time periods could be identified: from 1975 to 1980 all patients were given the same treatment and neuro-meningeal prophylaxis consisted of irradiation of the skull and intrathecal injections of methotrexate. Since 1981, the therapeutic regimen is designed according to the histo-immunological classification of the tumors and neuro-meningeal prophylaxis is carried out with the use of high dose methotrexate. With this new approach the survival rate has increased from 45 to 80% and the incidence of neuromeningeal relapses has decreased.
Adolescent↗
Role of parenteral antibiotherapy in gastrointestinal tract flora suppression. A study in children treated with high-dose chemotherapy and autologous bone marrow transplantation.
In order to determine the effect of parenteral antibiotherapy on the fecal flora in patients with profound and prolonged granulocytopenia, we initiated a prospective study of 62 cases of autologous bone marrow transplantation following high-dose chemotherapy. All patients were children from 2 to 18 years old, isolated in a protective environment, receiving a diet low in viable microbial content but no oral non-absorbable prophylactic antibiotics to decontaminate the gastrointestinal tract. Bacteriological analysis of fecal flora was conducted at least once a week before and during parenteral antibiotherapy, administered at the first greater than 38 degrees C febrile episode in these granulocytopenic patients (granulocyte count less than 0.5 X 10(9)/l). The 58 evaluable patients fell into three groups with regard to the systemic antibiotherapy: group A (n = 16): moxalactam + mezlocillin; group B (n = 15): moxalactam + tobramycin; and group C (n = 27): cefotaxime plus gentamicin. Fecal flora suppression was observed in 51/58 cases (88%) (group A: 15/16, group B: 13/15, group C: 23/27). It always occurred within 5 days of initiating parenteral antibiotherapy and persisted in 88% of the 51 patients over the whole period of systemic antibiotherapy. During the latter, fecal recolonization was observed in seven cases (12%), always by Enterobacteriaceae sensitive to the prescribed systemic antibiotherapy, never responsible for septicemia. Since parenteral antibiotherapy alone was able to suppress the gastrointestinal tract flora, the effects of this treatment should be considered in all trials of digestive tract decontamination.
Adolescent↗
[ORL cancer in the child. Histologic and topographic distribution. Therapeutic indications (apropos of 380 IGR cases 1975-1987)].
Three-hundred and eighty cases of head and neck cancers in children, observed in the Pediatric Department of the Institut Gustave Roussy over a 12-year period are reported. The authors study the clinical presentation of these cancers (primary site and histological distribution) and consider the major prognostic factors. A brief reminder is then given of the therapeutic indications for the main tumors observed.
Child↗
[Basal-cell nevomatosis associated with multifocal fetal rhabdomyoma. A case].
Nevoid basal-cell carcinoma is a hereditary syndrome. Its major features are a multiple basal-cell carcinoma which appears early in childhood, skeletal and genital abnormalities and ectopic calcifications. It may be associated with malignant schwannoma, medulloblastoma and lymphoma. We report one case of nevoid basal-cell carcinoma syndrome associated with foetal rhabdomyoma, thyroid gland polyadenoma and benign schwannoma. The first case of foetal rhabdomyoma associated with this syndrome was described in 1976.
Adult↗
[Treatment of malignant intracranial germ-cell tumors in children. 7 cases].
Seven children, aged from 9 to 16 years, with intracranial germ cell tumour received post-operative chemotherapy prior to radiotherapy. Treatment consisted of several courses of vinblastine, bleomycin and cisplatin alternating or not with courses of cyclophosphamide and actinomycin D. In the 6 children whose tumours secreted alphafoetoprotein or chorionic gonadotrophin, chemotherapy brought these markers down to normal. Tumoral regression at radiology exceeded 75 per cent in 6 patients. These results demonstrate the effectiveness of chemotherapy against intracranial germ cell tumours such as pure germinomas or secreting tumours. They encourage the systematic use of pre-irradiation chemotherapy in order to reduce the radiation doses delivered and to improve the prognosis.
Adolescent↗
Prognostic factors in 281 children with nonmetastatic rhabdomyosarcoma (RMS) at diagnosis.
Pretreatment characteristics of 281 children with nonmetastatic rhabdomyosarcoma, included in the registry of the International Society of Pediatric Oncology (SIOP) between January 1975 and December 1983, were examined to study the children's prognosis. The multivariate statistical method (Cox regression model) was used for each of two endpoints: survival time and disease-free time. The three most important predictors for survival time were primary site (p less than .001), clinical stage (p = .009), and sex (p = .020). The best results involved paratesticular and orbital primary sites, regardless of the clinical stage; males fared slightly better than females. These same three factors were also significant predictors for disease-free time.
Adolescent↗