Chemotherapy in osteogenic sarcoma: the experience of the Pediatric Department of the Gustave Roussy Institute.
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Adolescent↗
Biomedical subjects
G Contesso
Publications and source records attributed to G Contesso.
Factor analysis as a means of determining response to chemotherapy in patients with osteogenic sarcoma.
The prognosis of localized osteogenic sarcoma (OS) has improved considerably since the introduction of neoadjuvant chemotherapy. However, there is a subset of patients who do not show full benefit from neoadjuvant chemotherapy because of chemoresistance. The early identification of poor responders to chemotherapy during neoadjuvant therapy remains difficult. In order to evaluate the role of bone scintigraphy we report our experience of dynamic technetium-99m hydroxymethylene diphosphonate bone scintigraphy in 19 cases of paediatric osteogenic sarcomas. Before the beginning of chemotherapy, a dynamic scan was recorded during 30 min followed by static images at 3 h. The procedure was repeated halfway through the course of chemotherapy (6th week). Histological grading of the response to chemotherapy was carried out in the 12th week, showing nine good responses and ten poor responses. Factor analysis of dynamic structures (FADS) applied to dynamic scans allowed us to identify three factors termed vascular, "soft tissue" and osseous factors. The effect of chemotherapy on each factor was evaluated. Using FADS we were able to detect all the poor histological responders with the combination of vascular and osseous factors. Six out of nine good histological responders were also classified as scintigraphic responders. FADS applied to dynamic bone scans allowed us to identify at an early stage all the poor histological responders to neoadjuvant chemotherapy. This method may have clinical relevance for the therapeutic strategy in patients with OS.
Adolescent↗
Bone metastases as the presenting manifestation of rhabdomyosarcoma in childhood.
Rarely, rhabdomyosarcoma can present with bone pain and bone lesions on radiographs without evidence of a primary tumor. Of 428 children with biopsy-proven rhabdomyosarcoma, four presented with radiographic evidence of bone metastases, but no primary tumor was found on subsequent evaluation. On radiographs, these metastases, located most commonly in the metaphyses of the extremities and in the spine, displayed a destructive or diffusely permeative pattern without sclerotic margins and mimicked the more common neuroblastoma. One patient also had diaphyseal cortical lytic metastases of the tibia. Radiographs defined metastases of the extremities better than the correlative bone scans. In the spine, on T2-weighted magnetic resonance (MR) images, metastases displayed high signal intensity which contrasted with the low-signal-intensity marrow in these pediatric patients. On histopathologic examination, metastatic rhabdomyosarcoma was composed of small cells of variable size, shape, and growth pattern similar to other round cell tumors. A positive desmin immunohistochemical test helped to establish the diagnosis. The radiologist, pathologist, and clinician should be aware of this unusual presentation of rhabdomyosarcoma so that suitable immunohistochemical tests are performed and appropriate chemotherapy given.
Adolescent↗
Oral contraceptive use and risk of benign breast disease in a French case-control study of young women.
We report the results of a French hospital-based case-control study designed to analyse the relation between the use of oral contraceptives (OC) and the risk of benign breast disease (BBD). The cases were 286 women, each less than 46 years old, with BBD histologically verified between 1982 and 1985. Controls were 382 patients, matched to cases on year of birth and month of interview, and who were hospitalized for a non-malignant disease other than BBD. Odds ratios were estimated by multivariate regression, taking into account level of education, place of residence, family history of breast cancer, age at menarche, number of children, age at first full-term pregnancy and Quetelet index. The risk of BBD was found to decrease significantly with a longer use of OC before the first full-term pregnancy (FFTP), but there was no association between the risk of BBD and the duration of OC use after FFTP. OC use before FFTP reduced the risk of non-proliferative disease, but did not significantly affect the risk of proliferative disease. These results did not depend on the amount of oestrogen (0.05 mg or more vs < 0.05 mg) contained in OC.
Adult↗
Polyhedral microcalcifications at mammography: histologic correlation with calcium oxalate.
Pathologists have associated calcium oxalate dihydrate (weddellite) in breast biopsy specimens with benign or borderline lesions and rarely with malignancy. These microcalcifications may have a polyhedral shape in histologic specimens owing to their crystalline structure. A retrospective radiologic-histologic correlation study was performed on 300 clusters of microcalcifications to determine if microcalcifications with a polyhedral shape could be found at mammography. In 19 cases (6.3%), polyhedral microcalcifications (PMs) were detected by two radiologists. In 12 of these 19 cases, weddellite crystals were found in the histologic specimen under polarized light; in seven cases, no calcification was found. Although rare, PMs can be found at mammography by radiologists aware of their existence. They are due to weddellite crystals and in this series were associated with benign disease in 89% of cases. Prospective studies are required to determine the frequency of PMs on screening mammograms and to evaluate their negative predictive value.
Breast↗
Extraskeletal mesenchymal chondrosarcoma.
Among seven patients with extraskeletal mesenchymal chondrosarcoma (EMC), three children (aged 3-6 years) developed EMC in a central location and four adults (aged 38-54 years) developed EMC in both central and peripheral sites. Conventional radiography and tomography and computed tomography (CT) depicted EMC as a soft-tissue mass with ring, arc, stippled, and highly opaque calcifications in four patients. Contrast-enhanced CT showed lobulation and peripheral tumoral enhancement, sometimes with central low-attenuation areas. On magnetic resonance (MR) images, EMC was a lobulated mass with high signal intensity on T2-weighted images and enhancement with low-signal-intensity focal areas on contrast-enhanced T1-weighted images. All adults developed recurrences and/or metastases and died. Of the three children, two were living and free of disease at the end of the study and the third child died of chemotherapeutic-induced leukemia. Although imaging features of EMC are nonspecific, its chondroid-type calcifications and foci of low signal intensity within enhancing lobules may reflect its dual histopathologic morphologic characteristics of differentiated cartilage islands interspersed within vascular undifferentiated mesenchyme.
Adult↗
Assessment of breast cancer recurrence with contrast-enhanced subtraction MR imaging: preliminary results in 26 patients.
Local relapse was suspected in 26 women treated conservatively for breast cancer. All women underwent routine magnetic resonance (MR) imaging and a dynamic MR subtraction study after injection of gadolinium-tetraazacyclododecanetetra-acetic acid. Twelve women had no local relapse. Surgical biopsy enabled confirmation of recurrence in 14 patients. Routine MR imaging did not allow differentiation of recurrence from glandular or scar tissue. Except for one case of fat necrosis, patients without local relapse showed no contrast enhancement 1 minute 34 seconds after injection. At dynamic MR imaging, all recurrences showed contrast enhancement 1 minute 34 seconds after injection. Nodular enhancement (n = 11) was found in invasive carcinoma, whereas linear enhancement was seen in intraductal carcinoma (n = 3). Subtraction of precontrast from postcontrast images always allowed better visualization of contrast enhancement. Contrast-enhanced subtraction dynamic MR imaging may prove to be accurate in diagnosis of local relapses of breast cancer.
Adult↗
[Intraductal carcinoma of the breast (DCIS). Should the treatment be conservative or not?].
Due to systematic mammography, DCIS is being seen with increased incidence than in the past. Asymptomatic women screened by mammography show a high incidence of microcalcifications. Lymph node involvement is seen in very few patients. Total mastectomy provides local control and long term survival approaching 100%. Immediate breast reconstruction allows better acceptance of mutilations. Conservative treatment has been advocated for localised DCIS. The classification system dividing DCIS into comedo- and non-comedo subtypes could be an oversimplification because of the frequent histological heterogeneity. Other criteria such as cytological features oncogene activity of C erb 2 and clinical and radiological features must be considered. It has now been demonstrated that DCIS does not have a multicentric distribution. Assessment of lesion size appears to be primordial for breast conservative treatment indications. A relationship has been demonstrated between lesion size and microinvasion. The aim is complete excision with free margins and a cosmetically acceptable postoperative result. Radiotherapy seems to lower the incidence of local recurrences in retrospectives studies. Prospective randomized trials are being conducted to compare lumpectomy with and without radiotherapy.
Breast Neoplasms↗
[Minimal breast cancer: definition].
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Breast Neoplasms↗
[Desmoid tumors or intra-abdominal fibromatoses].
Intraabdominal desmoid tumour or fibromatosis, recurrent but non-metastatic, invasive, fibroblastic proliferations, are rare tumours. From 1968 to 1989, 16 patients were treated at Gustave Roussy Institute. They were associated with familial adenomatous polyposis in 10% of cases. These tumours, observed mainly in young women (70 to 85% of cases), are aggravated by pregnancy, and spontaneous regression can occur at menopause, proving their hormonal dependence. Although histologically benign, they are serious lesions due to their invasive character; their excision is complete in only 50% of cases. They recur in 30% to 75% of cases and cause death of the patient in 30% of cases. Treatment is surgical but due to their often very slow course, and their spontaneous stabilisation in some cases, a mutilating surgical treatment (extensive small intestine resection) does not seem to be justified. Radiotherapy is effective only at doses incompatible with the site of these tumours (35 to 60 Gy). Chemotherapy has never been shown to be effective.
Adult↗
Risk factors for failure of immediate breast reconstruction with prosthesis after total mastectomy for breast cancer.
BACKGROUND: Immediate breast reconstruction (IBR) after mastectomy represents a clear improvement in the quality of life of patients with breast cancer, but prosthesis removal is relatively frequent. Therefore, it is important to identify risk factors related to this removal. METHODS: A series of 142 patients treated with mastectomy and IBR at the Institut Gustave-Roussy between January 1976 and December 1988 were studied. Forty-seven had an in situ carcinoma, 93 an infiltrating carcinoma, and 2 a fibrosarcoma. IBR failures were defined as removal of the prosthesis, and early failures as removal during the first 7 weeks after IBR. RESULTS: The early failure rate was significantly higher in patients with invasive carcinomas or fibrosarcomas than patients with in situ carcinomas (12% versus 0%, P = 0.04). The risk of late IBR failure was significantly higher in patients who had received postoperative radiation therapy than patients who did not (P = 0.0002). However, this increased risk applied only to women in whom the chest wall was exposed to radiation because of a high risk of recurrence. CONCLUSION: It might be preferable to postpone breast reconstruction for patients with infiltrating breast carcinoma because early complications are not infrequent and could delay adjuvant chemotherapy. Late complications associated with chest wall irradiation could be avoided with the use of alternative techniques such as the transverse rectus abdominis myocutaneous (TRAM) flap procedure.
Breast↗
Does malignant small round cell tumor of the thoracopulmonary region (Askin tumor) constitute a clinicopathologic entity? An analysis of 30 cases with immunohistochemical and electron-microscopic support treated at the Institute Gustave Roussy.
The morphology and clinical outcome of 30 patients with malignant small round cell tumors located in the thoracopulmonary region (Askin tumor) are reported. Histologically, all tumors had similar patterns, with small round-to-oval cells and a lobulated stroma. Immunohistochemical analysis always resulted in positive staining for one or several neural markers. No significant differences were found compared with the immunomarkers in 26 typical Ewing's sarcomas located outside the thoracic wall. In three specimens, electron microscopy confirmed the presence of membrane-bound neurosecretory granules. It was confirmed that there is a remarkable similarity among all malignant small round cell tumors, including Askin tumor and Ewing's sarcoma. Overall survival was poor with a 2-year rate of 38% and a 6-year rate of 14%.
Adolescent↗
Nucleoli and AgNOR proteins in 32 cases of primary breast carcinoma. Spatial pattern of interactions between 50 clinical and histometric criteria.
The purpose of this paper is to analyze a method allowing selection of the best morphometric criterion for quantifying AgNOR proteins under conventional observation conditions by light microscopy. We determined 50 parameters for 32 cases of primary breast carcinoma. For each case, three 100-nucleus samples (tumor center and periphery on a Giemsa-stained, 3-microns tumor section, periphery on a silver-stained section) were quantified. Distribution-free multidimensional data analysis was used to explore the geometric significance of the coefficient of correlation. This analysis revealed a clinical message linked with the largest "nucleolar structure" (nucleolus or AgNOR clump) per nucleus on the tumor periphery. Tumor recurrence or death correlated only with the coefficient of variation of the "nucleolar/nuclear" ratio of the greatest clump of AgNORs per nucleus periphery (CVRSa). The prognostic value of CVRSa is independent of that of conventional criteria, such as age, tumor size, Scarff-Bloom-Richardson grading and lymph node status. The largest AgNOR clump per nucleus may prove to be a further practical prognostic predictor.
Biometry↗
Imaging in the follow-up of soft tissue tumors.
Local recurrences of aggressive soft tissue tumors are frequent and very difficult to detect clinically after surgery and radiation therapy. Ultrasound is limited because the skin is thick. On contrast-enhanced CT, both scars and recurrences increase their signal. MRI is the best technique, and the only one we presently use in the detection of recurrent tumors. We are reporting 511 follow-up examinations in 182 patients after tumor removal. All patients had histologic confirmation or at least 6 months follow-up. There were 164 malignant tumors and 18 cases of aggressive fibromatosis.
Contrast Media↗
[Reflections on the classification validation of the most common forms of osteosarcomas by functional isotopic methods].
After noting the limits of a purely morphological approach, either the descriptive methods for classifying certain osteosarcomas, or the techniques connected with these limitations such as particularly limited or non significant sampling, the authors consider the possible contribution of new methods such as immunohistochemistry or in vivo radioisotope tests to improve osseous tumor classification. The latter methods may be particularly useful in clarifying a significant number of difficult diagnoses or in providing information on tumor location, to be combined with morphological observations regarding osteosarcomas.
Diagnosis, Differential↗
[Neurosarcoma associated with Von Recklinghausen disease: apropos of 25 cases observed at the Gustave Roussy Institute from 1967 to 1990].
In the absence of systematic immunohistochemistry investigations, only 25 cases (out of 69) clearly diagnosed as neurosarcoma due to the association with Von Recklinghausen disease (ie, neurofibromatosis type 1) and treated at the Institut Gustave Roussy were included in the present study. Neurosarcoma consists of a neurocristopathy whose cells migrate to several parts of the body in order to constitute neuroglia, Schwann cells, pigmented and endocrinal tissues. From 1967 to 1990, 25 cases of such neurosarcomas associated with a neurofibromatosis type 1 were seen at our institute. Three different histological terms exist for this tumour: malignant schwannoma, neurosarcoma, and more recently, malignant peripheral nerve sheath tumours (MPNST). The median age (23 years) of the patients with neurofibromatosis type 1 is lower than that of patient with isolated neurosarcoma. Their sex ratio is 2/1. Primary tumour surgical exeresis was performed in all cases, with poor results in 7. Post-operative radiation therapy was not systematically used in this series. It was administered only in cases with incomplete surgical exeresis or when a local recurrence occurred. Adjuvant CYVADIC (doxorubicin, procarbazine, cyclophosphamide, vincristine) chemotherapy was administered in 5 cases, of in cases of relapse (8). All cases but 2 (the most recent ones) relapsed within 1 to 226 months (median 7 months). In addition, 13 patients developed metastases. Overall, the 2-year and 4-year survival rates were 41% and 18%, respectively. Our observations confirm the very poor prognosis for the association of neurofibromatosis type 1 and neurosarcoma. This finding should lead to systematically associate radical surgical exeresis, post-operative irradiation and adjuvant chemotherapy in the treatment of these patients.
Adolescent↗
[Hyperplastic lesions of the high risk breast. Definitions, diagnostic and therapeutic problems].
It has become possible to select from the framework of those women who have epithelial proliferations a group that are at high risk. This has been achieved by bringing together methods employed in the fields of defining better what are lesions of the breast, in assessing what are the best ways early detection of cancer of the breast and in epidemiological researches. The precise significance of these lesions in the absence of sufficiently reliable biological parameters is still problematic and their future still uncertain. Their relationship to invasive cancer is far from being constant, but it is possible for them to regress spontaneously. There are still limits between the results obtained in experimental animals and in human beings but as yet there has been no medical treatment that can be claimed to be a certain way of avoiding proliferative activity in these mastopathies. Surgery is also often poorly adapted to this kind of situation. In view of this, the choice of therapy is often difficult and has to be tailored to each case.
Biopsy↗
[Elastofibroma dorsi. Apropos of 5 cases].
Elastofibroma is a rare tumor mainly found under the scapula. Based on a recent study of five cases, we would like to stress the importance of making the diagnosis of elastofibroma, as this lesion simply consists of dysplasia of the elastic fibres and is not malignant as could be suggested by clinical examination.
Aged↗