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Biomedical subjects

M Gruner

Publications and source records attributed to M Gruner.

At least 19 recordsLinked to original sources

Is there a place for radiation therapy in the management of hepatoblastomas and hepatocellular carcinomas in children?

From May 1978 to August 1988, 15 children with a primary malignant liver tumor received radiation therapy as part of their management at the Institut Gustave-Roussy. Age ranged from 4 months to 13 years. The male to female ratio was 1.5. Eleven patients had a histologically proven hepatoblastoma, two a hepatocellular carcinoma, and histology was not documented in two. Resection of the primary liver tumor was performed in nine cases, and all patients also received sequential chemotherapy, generally preoperative and alternating vincristine, doxorubicin, cyclophosphamide with vincristine, cyclophosphamide, and cis-platinum. Radiotherapy was performed postoperatively in eight incompletely resected patients. Six of eight are alive and free of disease 4-83 months following treatment (median 39 months) and 11-98 months since diagnosis (median 45 months). All but one were treated to limited fields to a total dose of 25-45 Gy (median 40 Gy). One patient became resectable by a combination of 24 Gy to the whole liver and concomitant 5FU and Cis-Platinum and remains with no evidence of disease 68 months following radiation therapy. Of four unresectable primaries, only one was controlled by radiotherapy. Neither of two children with pulmonary metastases were controlled by whole lung irradiation to a dose of 18 and 20 Gy, respectively, and one still remains stable 41 months after resection of a residual metastatic nodule. Neither of two hepatocellular carcinomas were controlled by doses up to 40 Gy. This small series suggests that in hepatoblastoma, radiotherapy to a total of 25-45 Gy fractionated doses, combined with chemotherapy, can play a role in selected inoperable children and also in those with minimal postoperative residues below 2 cm. It also indicates that in hepatocellular carcinoma, radiotherapy is ineffective in this dose-range.

Adolescent

[Current features of malignant renal tumors in children].

The diagnosis of nephroblastoma is based on a postulate: any solid intrarenal tumour in a child between the ages of six months and six years is a nephroblastoma and therefore requires preoperative chemotherapy. Fine needle biopsy to obtain histological confirmation is only justified in doubtful cases. The prognosis is dominated by unfavourable, sarcomatous and anaplastic histological forms, which represent less than 10% of all nephroblastomas and are responsible for one half of deaths. Due to the better understanding of the therapeutic sequelae, particularly following radiotherapy, this modality is avoided whenever possible in young children. Radiotherapy is only indicated in stage II, with lymph node invasion, and stage III forms with favourable histology. It is indicated in all forms with unfavourable histology except for stage I anaplastic tumours. Surgery is indicated for metastases, especially lung metastases, once again to avoid irradiation. Lastly, bilateral partial nephrectomy is the best treatment for bilateral nephroblastoma.

Child

[Neurectodermal malignant tumor of the soft tissues after treatment with immunosuppressive agents of lipoid nephrosis].

Immunosuppressive drugs are known to increase the risk of inducing neoplasia, especially acute leukaemia when high doses are used. A case of nephrosis in a 10 year-old boy treated with chlormethine (cumulative dose: 0.8 mg/kg) and chlorambucil (cumulative dose: 10 mg/kg) is reported. Four years after the beginning of the treatment an extraskeletal Ewing's sarcoma occurred. Since the review of literature failed to find any malignancy induced by such an immunosuppressive treatment for nephrosis, the question whether or not this extraskeletal Ewing's sarcoma was attributable to this treatment remains unanswered.

Adrenal Cortex Hormones

[Bronchogenic cysts in the carina].

Between 1977 and 1990, 11 children with carinal bronchogenic cysts were operated in our institution: 8 girls and 3 boys, ranging in age from 1 month to 5 years. All were symptomatic (acute respiratory distress and recurrent bronchiolitis). Chest X-ray showed an unilateral over distension in 10/11 cases. Barium oesophagogram showed a compression in 6/10 cases. Bronchoscopy noticed an extrinsic compression in 10/11 cases and a tracheal and/or bronchial diskinesia in 5/11 cases. The computed tomography showed a low density mass in 4/4 cases. 9 cysts were left-sided and 2 right-sided. Both children underwent a second surgery for a second cyst. 2 pneumonectomies for complete parenchyma destruction were realised. 1 left pulmonary hypoplasia was noticed. A tracheal and/or bronchial diskinesia in post-operative was noticed in 5/6 cases. The clinical and functional respiratory following was good in 10/11 cases. An early surgery treatment is necessary before definitive sequelae.

Barium Sulfate

[Renal transplantation in children. Surgical aspects].

The renal transplantation in children has some specificities: urologic anomalies (vesico ureteral reflux, posterior urethral valves) are frequently the cause of the renal failure, and necessitate a thorough surgical preparation before transplantation (nephrectomy, reconstitution of urinary tract). The child must have a sterile, compliant and continent urinary tract on the day of the operation. In small children (< 15 kg), it is often necessary to operate through a transperitoneal incision, especially if the donor is an adult: the anastomoses will then concern the aorta and vena cava. The results are good, even better than in adults, except for very young children (under six years of age). Transplantation with living related donor (LRD) give the best results. Currently, the graft survival is 87% to 90% after three years with LRD, versus 65 to 77% with cadaveric donors.

Age Factors

Pancreatoblastoma: response to chemotherapy.

Two cases of pancreatoblastoma in children are reported here. Only biopsies were made at laparotomy as surgical resection by duodeno-pancreatectomy was not possible. In both children a dramatic response was observed with chemotherapy: doxorubicin plus cisplatin for one, cyclophosphamide, actinomycin D, bleomycin, vinblastine sulfate, and cisplatin for the other. After completion of the chemotherapy the first patient had a local resection; then he had radiotherapy. He is alive in first remission 40 months after the end of the treatment. In the second patient, regional recurrence occurred 8 months after chemotherapy was ended. A transient second remission was obtained with ifosfamide plus etoposide alternating with epirubicin plus vincristine. The patient died 36 months after the diagnosis. Therefore, these two cases suggest that chemotherapy may be proposed before any attempt at surgical excision. Nevertheless, early consolidation by radical resection or irradiation must be considered.

Adenocarcinoma

Nonmetastatic thoracic neuroblastomas: a review of 40 cases.

From 1982 to 1987, 40 children with non-metastatic thoracic neuroblastoma were treated with the same therapeutic regimen. According to TNM staging, there were 11 CS I, 19 CS II, and 10 CS III. All patients underwent surgery; 30 had primary surgical excision; in 10 whose tumors were deemed unresectable, surgery was delayed until after a trial of chemotherapy. Operation was completed by several courses of chemotherapy in case of microscopic residual disease or regional lymph node involvement; radiotherapy was delivered in case of gross residual disease. Using this therapeutic approach, EFS is 92% with a median follow-up of 40 months. Severe complications were rare and sequellae appear to be related to the disease, i.e., neurologic consequences of cord compression.

Antineoplastic Combined Chemotherapy Protocols

[Surgery for lung metastases in children].

The authors report a series of 76 children with lung metastases followed from 1974 to 1989. Forty-four children were operated on with 64 thoracotomies (50 wedge resections, 5 segmentectomies, 2 pneumonectomies, 6 biopsies, 1 excision of an inflammatory granuloma): 23 of these children are alive, and 17 of them without any adjuvant radiotherapy. Thirty-two were not operated on; of the 13 that are alive, 7 had no adjuvant radiotherapy. The indications for surgery were: to treat a complication of the metastasis, to verify persistent images on CT-scan, but essentially to avoid more aggressive treatment (chemotherapy, radiotherapy) with its risk of sequelae in children.

Adolescent

[Genital prognosis of boys with bladder exstrophy or epispadias with incontinence. Apropos of 14 cases].

Bladder exstrophy and epispadias with incontinence are associated with urinary tract and genital anomalies. The genital and sexual aspects were studied in 14 adolescent or adult males (12 exstrophies and 2 epispadias). The appearance of the penis was satisfactory in fifty percent of cases. Erections were always preserved but normal ejaculations were present in only one half of cases. Fertility potential was reduced. The improvement of surgical technique, and especially penile lengthening, has greatly improved sexual intercourse for these patients.

Adolescent

[Genital prognosis of girls with bladder exstrophy or epispadias].

The authors report ten cases of women with previous exstrophy of the bladder or epispadias with incontinence followed up to child-bearing age. Six of the ten females had urinary diversion and four retained their bladder and were continent. Three key aspects are considered: physical appearance of the external genitalia which was satisfactory in all cases; preservation of sexual function, known in only four of the ten cases and which was reported by these patients to be satisfactory, and finally, the ability to bear children. Three patients became pregnant resulting in four normal births (one patient had twins). Cesarean sections were recommended in patients with pregnancies at term. Cesarean should be performed systematically in patients with bladder reconstruction.

Adolescent

A case of rectal bleeding in infancy.

A case of colonic haemangioma in a 3 month old girl presenting with rectal bleeding and anaemia is presented. A colonic mass lesion was suspected on barium follow through and confirmed by enema and surgical excision.

Colonic Neoplasms

[Treatment of the loss of major wall substance in children using synthetic resorbable mesh. Apropos of 10 cases].

Ten children with a major parietal defect, abdominal or thoracic, were operated by using a resorbable polyglactine network, during these three last years. On six children, the parietal defect was only deep, according to the surgical ablation of a parietal malignant tumor; on four other children, the defect was total, interesting the muscular and aponeurotic plan, but also the skin (two gastroschisis, and two septic eviscerations). Neither reject nor suppuration were observed. In case of total defect, a parietal reflection may be necessary in the long term.

Abdominal Muscles

[Obstructive anuria in children. Apropos of 22 cases].

The authors report twenty two cases of obstructive anuria observed in children. Causes are diverse: 6 cases were observed during the course of tumors, 4 cases were secondary to bilateral renal stones (or unilateral in a single kidney), 3 cases were observed before surgical correction of latent or well tolerated congenital uropathy, and 9 cases in the immediate postoperative period (including 8 after antireflux surgery). In the emergency situation, treatment of obstructive anuria is based on urinary diversion ideally by percutaneous nephrostomy under ultrasonic control. But prevention is the best treatment of anuria: treatment of urinary tract infections resulting in renal stones, in case of tumor, ultrasonographic survey of chronic upper tract dilatation: rigorous atraumatic operative technique avoiding any oedema.

Adolescent

[Morphology of the laterotrusive paths in dentitions with normal function].

The occlusal surface of the posterior teeth of 20 subjects with class-I-occlusion were analysed by means of an electronic profilograph in laterotrusion. Each of the curvatures recorded was defined by an approximated radius as well as its angle to the occlusal plane. The software used was specifically developed to suit this particular purpose. The data showed characteristic regularities. The results indicate that the average radius of the canine is longer than that of the molars. An alternating increasing and decreasing tendency of the angles to the occlusal plane was observed. The results suggest that the concept of canine-protected occlusion may be strengthened by the fact that there is a genetic determination of the canine dominance.

Bicuspid

[Relationship between transversal joint guidance and buccal curve morphology].

The relationship between the morphology of the curvature of laterotrusion facets and tooth-guided tracings of the mediotrusive condyle path was studied in 20 probands with normal function. The varying inclinations of the dominant laterotrusion facets in cuspid-protected or group-guided occlusion has no bearing on the angle of the mediotrusive paths relative to the horizontal plane. The morphology of the buccal curve allows no predictions as to the direction of the mediotrusive path curvature. Only the movements of the idling condyle were influenced by the type of occlusal guidance. In lateral excursive movements of the mandible with the teeth in contact group-guidance frequently resulted in an upward movement of the laterotrusive condyle and thus to a compression of the intraarticular soft tissues. Although valid conclusions regarding any type of interdependence are impossible unless we are able to measure real tooth and joint movements simultaneously, the results of our study contradict other hypotheses at any rate.

Dental Occlusion, Balanced