[Ewing's sarcoma].
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Antineoplastic Combined Chemotherapy Protocols↗
Biomedical subjects
N Delépine
Publications and source records attributed to N Delépine.
[Diagnosis and treatment of malignant bone fibrohistiocytoma].
Malignant fibrous histiocytoma (MFH) and giant cells sarcoma arise from fibrous tissue and histiocytic cells evenly distributed in all age group, except children much less common than osteosarcoma and chondrosarcoma. They most commonly localize in the major long bones (femur, tibia, humerus) and in pelvis. Pain and sometimes swelling are the most frequent symptoms but pathologic fractures are rather common. Medical imaging shows a purely osteolytic tumor centrally or eccentrically located, ill defined with a permeative or even moth eaten patterns. Periosteal reaction is scarce or absent. Osteogenic reaction or tumor calcifications are absent. Outcome was historically better than those of osteosarcoma but hangs on the grading of tumors; low grade sarcoma have a regional evolution while high grade malignant histiocytofibroma are threatened with metastases. Therapy must be adapted to the histological grading and the extent of the illness. Low grade localized MFH are best treated by wide resection. High grade MFH are preferentially treated by a comprehensive multidisciplinary approach with preoperative chemotherapy including high dose methotrexate, wide resection and postoperative chemotherapy. With such a treatment, nearly 80% of patients seen with localized tumor of the limb can be cured and keep a functional limb.
Adolescent↗
Results of multidisciplinary limb salvage in 240 consecutive bone sarcomas.
From 1979 to 1989, 240 bone sarcomas of limbs were treated by a multidisciplinary limb salvage protocol. The tumors included 112 osteosarcomas, 71 chondrosarcomas, 3 fibrosarcomas, 10 malignant histiocytofibromas, 40 Ewing's sarcomas and 4 other rarer sarcomas. Patients mean age was 28.6 yr (range 4-91 yr). The average tumor size was 13.5 cm (3-43 cm). There were 2 grade IA, 21 IB, 1 IIA, 188 IIB, and 28 III B according to Enneking's classification. Extratumoral en bloc resection was performed in all cases (large in 113, marginal in 121, intrafocal in 6) by the same surgeon. In poor responders to preoperative chemotherapy with Ewing's sarcomas or osteosarcomas, 35 Grays postoperative radiotherapy was administered. Ewing's sarcomas and osteosarcomas received short preoperative and long postoperative chemotherapy. At last follow-up (median 60 months, range 6-120 months), 150 patients were disease-free; 4 were under treatment and had visible disease; 86 had died. Actuarial disease-free survival rate for patients seen with localized previously untreated disease was 83% at 2 yr and 77% at 3 yr. Fifteen local recurrences were observed (6%). Statistical analysis confirmed the poor prognosis factors of visible metastasis, proximal location and large tumor size, and pointed out the major prognostic value of therapeutic factors: the effectiveness of chemotherapy in Ewing's sarcoma and osteosarcomas, and the adequacy of surgery in all cases. Limb salvage can be performed by a well experienced multidisciplinary team in 96% of limb sarcomas without major risk of local recurrence. However, amputation is safer if the surgeon lacks experience.
Adolescent↗
Osteogenic osteosarcoma: a model of curable disease by multidisciplinary approach of treatment.
The authors review the mean steps for the treatment of osteogenic osteosarcoma from the 1970's: 1), demonstration of the effectiveness of HDMTX and possibility of weekly administration, dose-response effect, interest of other drugs (BCD, ADR, CDDP, IFX); 2), use of the primary as chemosensibility witness; 3), extent of conservative surgery. In order to optimize the good results obtained by Rosen (more than 80% DFS at 5y) the authors studied the HDMTX pharmacokinetics, the value of the seric peak at the end of infusion as an effective test and individualized the HDMTX treatment in each patient following his own pharmacokinetics. This individual approach allows us to obtain more than 90% actuarial event-free survival at 4 years in patients treated by conservative surgery.
Bone Neoplasms↗
Present trends in treatment of Ewing's sarcoma.
The prognosis of Ewing's sarcoma, the first malignant tumor in children under 10, has improved dramatically in the last 10 yrs. The authors review the signs and symptoms that permit diagnosis and analyse the improvement in treatment, based on multidrug's combination, neoadjuvant chemotherapy and surgical treatment of the primary. They point out the necessity of closed multidisciplinary co-operation to treat these patients. Under these conditions, disease free survival now reaches more than 90% at 3 years 1/2.
Bone Neoplasms↗
[Preliminary results of 79 massive bone allografts in the conservative treatment of malignant tumors in adults and children].
We have used massive allografts, preserved by freezing to -30 degrees C and sterilised by irradiation, to reconstruct large defects in bone after resection of high grade neoplasms. The average length of the defect was 20.5 cm. Complications included 3 deep infections, 8 pseudoarthroses, of which 4 joined after further operation and 3 are still under treatment, and 4 late fractures of the graft. Seventy two cases have been followed up, and the functional result was excellent in 30, very good in 22, good in 13, poor in 3 and bad in 4. In 3 cases H.L.A. antibodies against the graft have been detected, but no clinical or radiological abnormality was noted. Bone scintigraphy showed progressive revascularisation of the graft from surrounding muscle. Reattachment of muscles to the allografts was successful, and contributed to the good functional results. Reconstruction with sterilised allografts, in association with long stemmed titanium prostheses or a cemented nail, gave a better functional results than using massive stainless steel prostheses in patients with a reasonable life expectancy.
Adolescent↗
[Osteogenic osteosarcoma, a model of a disease that is curable using a multidisciplinary treatment approach].
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Adolescent↗
Radioiodinated meta-iodobenzylguanidine uptake in medullary thyroid cancer. A French cooperative study.
Fifty meta-iodobenzylguanidine (MIBG) scintiscans were performed in three groups of medullary thyroid cancer (MTC) patients. Group 1 (n = 11) included treated patients with normal calcitonin levels; Group 2 (n = 24) included patients with elevated calcitonin levels due to sporadic and isolated MTC; Group 3 (n = 15) included patients with elevated calcitonin levels due to familial MTC or multiple endocrine neoplasia Type IIA syndrome (MEN). In Group 1 three pheochromocytoma were depicted by MIBG scintiscan. In Group 2 MTC was seen in a small number of patients (3 of 24). In Group 3, besides adrenal hyperplasia and pheochromocytoma four patients, MIBG scintigraphy showed where MTC had localized and spread in almost half of patients (7 of 15). MIBG uptake occurred in patients with relatively high calcitonin level (greater than 0.6 nmol/l). These data indicate that in patients with familial MTC or MEN syndrome, MIBG scintiscan can be useful not only in detecting associated pheochromocytoma, but also in showing MTC.
3-Iodobenzylguanidine↗
[Current clinical profile of Streptococcus B infections in a Parisian neonatal intensive care unit (apropos 41 cases)].
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Female↗
[Objective response of desmoid fibroma to chemotherapy].
Six patients with inoperable desmoid tumors have been treated by preoperative chemotherapy. In all six cases objective response occurred and allowed conservative surgery. Objective responses included tumor softening (6/6), releasing of articular motion previously limited (3/3), and tumor size' decreasing (2/6). Few histologic modifications were seen. Such data advocates for preoperative chemotherapy in desmoid tumor when large and threatening the limb or the life of the patient.
Adolescent↗
Hypomelanosis of Ito (incontinentia pigmenti achromians) and mosaicism for a microdeletion of 15q1.
Hypomelanosis of Ito (incontinentia pigmenti achromians), a sacrococcygeal complex dysembryoma, seizures, severe cerebral lesions, mental retardation, chorioretinal atrophy, hemihypotrophy of the body, and skeletal anomalies are reported in a female infant of North African origin. Karyotype analysis revealed mosaicism for a microdeletion of the proximal region of 15q similar to that observed in Willi-Prader syndrome. The possibility of gene assignment of Ito's disease or that it may represent a nonspecific marker for mosaicism are discussed.
Chromosome Banding↗
CNS-side effects induced by Ifosfamide-Mesna in children with osteosarcomas.
The authors have had the opportunity to see two cases of CNS side effects of Ifosfamide-Mesna association in children. Data in the medical literature about this subject remains poor. Only a few observations are available. In the referred cases, CNS side effects (lethargy, apathy and mutism) appeared a few hours after the second day of treatment and were spontaneously reversible in a few days. After reviewing possible mechanisms of this toxicity the authors pointed out the necessity to keep this type of side effects of Ifosfamide-Mesna association in mind, and to avoid it in susceptible patients.
Adolescent↗
[Experiences and perspectives on adjuvant chemotherapy in osteogenic osteosarcoma].
This article analyses the relevance of the published results of adjuvant chemotherapy in osteogenic osteosarcomas. It discusses the causation and prospects for treatment particularly the details of the chemotherapy, with reference to mono- or poly-chemotherapy, the influence of the dose and of the interval of administration, and also the value of pre- and post-operative chemotherapy. It is apparent that intensive pre-operative chemotherapy limited to 4 weeks is a reasonable mean of distinguishing the good responders. The problem of the choice of therapy for bad responders is also discussed.
Antineoplastic Combined Chemotherapy Protocols↗
[Argument for the multidisciplinary treatment of Ewing sarcoma with surgery].
The authors summarize the literature on the local treatment of Ewing's sarcoma. They remind us that including surgery in local treatment increases disease free survival rate by 20-25% while it reduces functional sequelae and radioinduced sarcoma risk. They underline the major interest of histologic examination of in bloc resected tumor to guide postoperative chemotherapy. They advocate that local treatment of Ewing's sarcoma should always be surgical and conservative with the help when necessary of massive homograft and lengthening prosthesis.
Bone Neoplasms↗
[Digital angiography of bone sarcoma in the child].
The authors analyze the results of 50 computed angiographies made for osteosarcoma and Ewing's sarcoma. Better tolerated than conventional angiography computed angiography is also more sensitive in preoperative evaluation of effectiveness of neoadjuvant chemotherapy. Automatic subtraction, direct measuring of tumor size and vascularization density give to angiographic changes after chemotherapy the most predictive value. After conservative surgery or radiotherapy digital angiography provides most reliable local follow up.
Angiography↗
[Inorganic pyrophosphates and parathormone in hypophosphatasia. Study of a family].
The serum concentration of parathormone is usually normal in hypophosphatasia, a rare disease with a defect of bone mineralisation and low serum alkaline phosphatase activity. Nevertheless there are three cases in the literature presenting a hyperparathyroidism with or without hypercalcemia. No anomaly of parathyroid was found at autopsy. The authors describe the first cases of hypophosphatasia with low serum concentration of parathormone and raise the possibility of a trouble in the calcium-parathormone feed-back. They also emphasize the interest of the urinary pyrophosphate excretion. Its increase seem to be the most constant and the most specific biological disorder.
Adult↗
[Problems posed by the detection of familial medullary cancer of the thyroid in children].
The authors give the approach managing for medullary thyroid carcinoma in families afflicted by the multiple endocrine tumor syndromes, as defined by "G. E. T. C." (Groupe d'Etude des Tumeurs à Calcitonine), French Group based in Paris, 1983. Diagnosis of MEN II a usually follows investigations in a family of an adult patient found, at thyroid surgery, to have a MTC. Age related probability of development of hereditary MCT is stated. Investigations of the family must be most careful if the index patient is found to have medullary carcinoma on the both sides of the thyroid gland, and/or pheochromocytoma. Sequential monitoring (by means of blood calcitonin measurements following IV infusion of pentagastrin) of family members at risk between 5-35, allows diagnosis of C-cell hyperplasia and treatment of tumor in his earliest stages--MEN II b is usually recognizable in infancy or early childhood by clinical markers (mucosal neuromata, Marfanlike habitus and abnormalities of myenteric plexus). Report of patients in whom MTC was manifest as early as 18 months of age and metastasized at 2 years, require subjects at risk should be screened by CT immunoassay as soon as feasible and repeated annually until 35 years, and every 5 years after 35. The diagnosis of subjects at risk within a family is made easy by recognition of aforesaid clinical features and some more precocious abnormalities = disturbed intradermal histamine reaction and thickened corneal nerve fibers.
Adolescent↗
[Comparison of different methods of medical images in the assessment of local extension of osteogenic sarcomas].
The authors compared different methods of medical visualisation (standard radiography, arteriography, quantitative isotope bone scan, telethermography, CT scan, visualisation by nuclear magnetic resonance) in 52 osteogenic sarcomas. In the majority of cases the diagnosis was suggested by standard X-rays at the first visit. CT scan and visualisation by nuclear magnetic resonance are the two basic examinations both in terms of assessing the future single block excision as well as deciding the approach to be used for biopsy. Telethermography and quantitative isotope bone scan usefully complement clinical examination and standard X-rays in following the course during chemotherapy. Arteriography is indicated only in the presence of large tumors threatening the vessels directly.
Adolescent↗