Giant vertebral notochordal rest: a new entity distinct from chordoma.
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Chordoma↗
Biomedical subjects
J Rivel
Publications and source records attributed to J Rivel.
[Monostotic fibrous dysplasia of the calcaneus: two case reports].
The authors report two unusual cases of aggressive monostotic fibrous dysplasia involving the calcaneus. This lesion occurs on the 2th decade of life. The main symptom is talalgia. On plain films, differential diagnosis includes aggressive cystic or pseudocystic lesions of the calcaneus. On MR imaging, the tumor matrix is more suggestive of the diagnosis. Treatment of this aggressive form of fibrous dysplasia consists of a surgical curettage completed by cryotherapy and graft because of a high level of recurrence.
Adult↗
[Is the rings and arcs pattern of mineralization specific for chondroid matrix tumors?].
The rings and arcs pattern of mineralization is considered pathognomonic for cartilaginous tumors. The authors report four cases of osteoblastomas in which the presence of such a pattern of mineralization mistakenly lead to a diagnosis of cartilaginous tumor. These densities are not explained by the histologic findings.
Adolescent↗
[Primary intraosseous carcinoma of the jaws: results of treatment of 9 cases and proposed classification].
INTRODUCTION: Primary intraosseous carcinoma of the jaws is a rare squamous cell carcinoma arising in the jaw, having no initial connection with the oral mucosa and presumably developing from residues of the odontogenic epithelium. OBJECTIVES: To present 9 cases and to propose a staging system of primary intraosseous carcinoma of the jaws. CASES AND METHODS: Careful assessment of the clinical, radiological and histological examination of the specimens identified 9 patients with primary intraosseous carcinoma of the jaws. RESULTS: The male/female ratio was 8/1 and the mean age of patients at the time of diagnosis was 61.7 years. All lesions were located in the mandible. Usually, clinical symptoms had benign aspect and delay the diagnosis. Radiographic features were unilocular osteolytic lesion and ill-defined margins were usually noted with a mean tumor size of 3.4 centimeters. The mean delay of the diagnosis was 4.9 months. Surgery at the tumor site consisted of hemi-mandibulectomy in all cases. All patients but one had neck dissection. The postoperative radiotherapy mean dose was 62.6 Gy. Histologically, most squamous cell carcinomas were well differentiated with keratinization. All lymph nodes metastasis had capsular involved. Complications occurred in 2 cases. Four patients had recurrences in the mean delay of 13.8 months. The estimated overall 2-and 5-years survival was 65% and 46.5% respectively. DISCUSSION: Primary intra-osseous carcinoma of the jaws is a rare tumor none classified by UICC with poor prognosis. We recommend an aggressive treatment with postoperative radiotherapy. We propose a computer tomographic classification: T1 (tumor strictly intra-osseous), T2 (tumor with cortical destruction without involvement of adjacent soft-tissues) and T3 (tumor involvement of adjacent soft-tissues).
Adult↗
Galanin and galanin receptor expression in neuroblastic tumours: correlation with their differentiation status.
Neuroblastoma and its benign differentiated counterpart, ganglioneuroma, are paediatric neuroblastic tumours arising in the sympathetic nervous system. Their broad spectrum of clinical virulence is mainly related to heterogeneous biologic background and tumour differentiation. Neuroblastic tumours synthesize various neuropeptides acting as neuromodulators. Previous studies suggested that galanin plays a role in sympathetic tissue where it could be involved in differentiation and development. We investigated the expression and distribution of galanin and its three known receptors (Gal-R1, Gal-R2, Gal-R3) in 19 samples of neuroblastic tumours tissue by immunohistochemistry, in situ hybridization and fluorescent-ligand binding. This study provides clear evidence for galanin and galanin receptor expression in human neuroblastic tumours. The messengers coding for galanin, Gal-R1 and -R3 were highly expressed in neuroblastoma and their amount dramatically decreased in ganglioneuroma. In contrast, Gal-R2 levels remained unchanged. Double labelling studies showed that galanin was mainly co-expressed with its receptors whatever the differentiation stage. In neuroblastic tumours, galanin might promote cell-survival or counteract neuronal differentiation through the different signalling pathways mediated by galanin receptors. Finally, our results suggest that galanin influences neuroblastoma growth and development as an autocrine/paracrine modulator. These findings suggest potential critical implications for galanin in neuroblastic tumours development.
Cell Differentiation↗
[Endometrial hyperplasias resistant to progestins: alternatives to traditional treatments].
Endometrial hyperplasias are mainly regarded as a response to unopposed endogenous estrogenic stimulation and concern 12% of perimenopausal women. They are usually diagnosed because of irregular bleeding. They are divided into two categories based on the presence or absence of cytological atypia and further classified as simple or complex according to the extent of architectural abnormalities. Endometrial hyperplasias with cytological atypia are classically treated by hysterectomy. Endometrial hyperplasias without cytological atypia are classically treated by progestins. The bad observance (25% spontaneously stopping), the 30% recurrence rate after stopping progestin and the 12-53% resistance rate to treatment lead to propose a second-line therapy after endocrinological check-up, exploration of haemostasis, pelvic ultrasonography, hysteroscopy and endometrial biopsies. Standard treatments include uterine curettage which is not very effective and hysterectomy. Medical alternatives (gonadotropin-releasing hormone agonists, levonorgestrel-releasing intrauterine device) and surgical alternatives (endometrial resection, thermal balloon endometrial ablation) were developed to avoid treating functional pathology radically. These conservative procedures correct 80% of endometrial hyperplasia symptoms with a low rate of lateral effects. However, these results need to be confirmed by long-term studies. Some economical, legal or material factors can also limit carrying out the procedures. Clinical trials need to be performed to better define the place of medical and surgical alternatives to hysterectomy in the treatment of endometrial hyperplasias resistant to progestins.
Drug Resistance↗
The detection of Tel-TrkC chimeric transcripts is more specific than TrkC immunoreactivity for the diagnosis of congenital fibrosarcoma.
The t(12;15)(p13;q25) translocation, a recurrent chromosomal abnormality of congenital fibrosarcoma, leads to the expression of a Tel-TrkC fusion transcript. To determine whether detection of the chimeric protein may be helpful for the diagnosis of congenital fibrosarcoma, immunohistochemistry was performed with an anti-TrkC antibody on 26 spindle cell tumours of newborn or young children (n=19) or adults (n=7). Four out of five congenital fibrosarcomas showed TrkC immunoreactivity with cytoplasmic paranuclear staining. However, TrkC immunoreactivity was not restricted to congenital fibrosarcoma and was observed in infantile myofibromatosis, congenital haemangiopericytoma, desmoid tumour, nodular fasciitis, fibrous hamartoma, inflammatory myofibroblastic tumour, and adult fibrosarcoma. RT-PCR analysis was performed on nine cases, including four congenital fibrosarcomas, for which frozen material was available. Tel-TrkC transcripts were detected by RT-PCR in the four congenital fibrosarcomas analysed, but not in the five other spindle cell tumours. Furthermore, several Tel-TrkC transcripts encoding for kinase isoforms of the Tel-TrkC protein were detected in congenital fibrosarcoma and may be involved in oncogenesis. The reciprocal TrkC-Tel transcript was detected in only one congenital fibrosarcoma. While the detection of a Tel-TrkC fusion transcript is a recurrent feature of congenital fibrosarcoma, TrkC immunoreactivity does not appear specific for the diagnosis of fibromatous paediatric tumours.
Adult↗
[Lactating adenoma: radiologic aspects. A case report].
We report the US and MRI appearance in a patient with a giant lactating adenoma detected during pregnancy and followed-up for 12 months postpartum. The different imaging features of lactating adenomas are discussed, especially at US and MRI. We discuss also the role of core biopsy in diagnosing pregnancy associated breast masses.
Adenoma↗
Primary intraosseous carcinoma of the jaws.
OBJECTIVES: To present 8 new cases of primary intraosseous carcinoma of the jaws and to review the literature for an analysis of treatment modalities and patient outcomes. DATA SOURCES: A MEDLINE search from 1970 to 1999. The articles chosen and the study of the references of every one that produced additional articles provided database information for 28 patients. Eight new patients from our institutions were added. STUDY SELECTION: Our criteria of inclusion included the absence of ulceration of the oral mucosa, a negative result in the search for a distant primary tumor, and convincing histological documentation. DATA EXTRACTION: The variables of the analysis included age, sex, site of the tumor, condition of the oral mucosa, tumor size, neck status, treatment modalities, recurrences, and survival. DATA SYNTHESIS: Twenty-eight patients were identified in the literature, for a total of 36 patients. There were 28 males (78%) and 8 females (22%) ranging in age from 4 to 76 years (mean, 54 years). The tumor site was the mandible in 33 patients (92%) and the maxilla in 3 (8%). Of the 34 patients treated, 19 (56%) had recurrences. Overall 2- and 4-year survival was 60.5% and 39.9%, respectively. Patients who underwent radical surgery and postoperative radiotherapy (n = 11) had a 2- and 3-year survival probability of 61.3% and 40.9%, respectively, whereas in the remaining patients (n = 25), the rates were 59.7% and 31.3%, respectively (P =.60). CONCLUSIONS: Strict diagnostic criteria must be applied. The prognosis associated with primary intraosseous carcinoma of the jaws is poor and suggests the need for aggressive treatment.
Adolescent↗
Unusual manifestations of type II cryoglobulinaemia associated with Waldenström's macroglobulinaemia.
Cryoglobulinaemia in association with Waldenström's macroglobulinaemia is relatively common, ranging from 8% to 18% of cases; however, < 5% have symptoms or complications. We describe a patient with a history of cutaneous, peritoneal, and fallopian tube vasculitis related to type II cryoglobulinaemia associated with Waldenström's macroglobulinaemia. Cytotoxic treatment was initiated (cyclophosphamide, vincristine, and prednisone) and had a good initial response. However, after the third course of chemotherapy, the patient presented with septic shock and died. Even though cryoglobulinaemia is a model of systemic vasculitis, peritoneal and fallopian tube vasculitis associated with type II cryoglobulinemia has not been described previously.
Cryoglobulinemia↗
[Hepatobiliary cystadenoma with mesenchymal stroma: a hormone dependent tumor. Report of five cases with immunohistochemical study of hormone receptors].
Hepatobiliary cystadenomas with mesenchymal stroma are rare neoplasms, which occur only in females; they have a strong tendency to recur and a potential for malignant transformation. Microscopic features are characteristic but clinical findings are highly variable and laboratory data non specific. We report 5 cases with immunohistochemical study, showing the characteristics of mesenchymal stromal cells: myofibroblastic phenotype and expression of progesterone receptors in all cases and estrogen receptors in 3 out of 5 cases. These results point out the possible hormonal dependance of this tumor.
Adult↗
Brain stem infiltration by mixed Langerhans cell histiocytosis and Chester-Erdheim disease: more than just an isolated case?
Langerhans cell histiocytosis is classically considered as totally different from Chester-Erdheim's disease which consists in the infiltration of various parenchymas by macrophagic CD68-positive histiocytes. We report the case of a 46-year-old woman with a long history of diabetes insipidus who presented typical lesions of Langerhans cell histiocytosis on vulvar and skin biopsies as well as bony cellular infiltrates characteristic of Chester-Erdheim's disease. A few months later she presented cerebellar disorders and died after an 18-month course. At autopsy the pons was enlarged, due to numerous cellular infiltrates which were also scattered in the middle cerebellar pedoncles, dentate nuclei, midbrain and hypothalamus. There were S100-protein positive Langerhans cells intermingled with numerous ovoid CD68-positive histiocytes. There are a few reported cases of Chester-Erdheim's disease presenting foci of Langerhans cells histiocytosis in other parenchymas. In addition, there are 10 reported cases with diabetes insipidus and bilateral infiltration of the brain stem and cerebellum, considered as presenting either one type of histiocytosis or the other. Our case demonstrates that both histiocytoses may coexist in the brain and thus correspond in fact to the same pathology in certain particular cases.
Biopsy↗
Quantification of the inflammatory response in exudates to three polymers implanted in vivo.
Flow cytometry was used to quantify an inflammatory reaction in vivo as a new approach to evaluating the biocompatibility of biomaterials. The exudate formed inside cylindrical tubes composed of polyvinyl chloride (PVC), silicone elastomer (SIL), or polyurethane (PU) implanted subcutaneously in the dorsal region of rats was collected over a 3-week period. The volume, number of cells, and concentration of fibrinogen were determined in the exudate for the three biomaterials. The exudate was analyzed using a flow cytometry technique after labeling of the leukocytes with a monoclonal anti-CD45 antibody. Fibrinogen rose progressively over the 3-week period for the three polymers. After the different leukocyte lines were identified in rat blood samples, their determination in the exudate revealed differences among the three biomaterials. At day 2, PVC induced a predominantly neutrophilic inflammatory reaction whereas PU and SIL gave a mixture of monocytes and neutrophils. At day 9, the aspect of the cytograms was different, but the identification of the subpopulations was still possible. At day 23, the number of cell events became too low to distinguish the subpopulations. An even more detailed approach might be possible using specific labeling for each leukocyte line to establish a comparison among the three biomaterials. Flow cytometry associated with histomorphometric assessment might provide a precise quantitative in vivo test for determining the biocompatibility of materials.
Animals↗
Morphologic and histologic study of the ligamentum flavum in the thoraco-lumbar region.
The ligamentum flavum, of which there are only a few studies in the literature, has several features discussed in this work. On the macroscopic level, it has a metameric arrangement: it has two layers, superficial and deep, whose fibers are opposite, and it has close relations with the tendons of attachment of some spinal erector muscles. On the microscopic level, its structure is unique for a ligament, because of a predominance of elastic fibers, because of its intrinsic innervation at each level of the spine, and because this innervation grows poorer with increasing degeneration. The ligamentum flavum constitutes an active ligament, with an essential biomechanical role. Its injury is probably not without consequences, and therefore there are many technical problems about the surgical interlaminar approach.
Aged↗
Cervical dysplasia and human immunodeficiency virus infection in women: prevalence and associated factors. Groupe d'Epidémiologie Clinique du SIDA en Aquitaine (GESCA).
OBJECTIVE: To study the prevalence of Cervical Intraepithelial Neoplasia (CIN) and its association with HIV-I infection, controlling for other risk factors of CIN. DESIGN: Cross-sectional survey. SUBJECTS AND METHODS: HIV-1 seropositive (HIV+) and seronegative (HIV-) women were enrolled at the Obstetrics-Gynecologic Departments of the Bordeaux University Hospital from April 1993 to June 1995. A gynecologic check-up was performed with a clinical examination, a colposcopy and a Papanicolaou smear. Sexually Transmitted Diseases (STDs) were screened. Colposcopy was interpreted as: normal, low-grade or high-grade lesions. Interpretation of Papanicolaou smears was based on the 1988 Bethesda system using three descriptive diagnoses: normal, low-grade and high-grade Squamous Intraepithelial lesions (SILs). If colposcopy showed a high grade lesion or Papanicolaou smear a high-grade SIL, a cervical biopsy was performed. Absence of CIN was defined by normal Papanicolaou smear and colposcopy. High grade CIN was defined by either identification of high grade SIL on Papanicolaou smear or high grade lesion on colposcopy confirmed by CIN2-3 lesion on biopsy. Other cases were classified as low-grade CIN. HPV infection was diagnosed on presence of koilocytosis on cytological or histological specimens. RESULTS: Prevalence of CIN was significantly higher in the 128 HIV+ women than in the 102 HIV- women: 34.4% vs. 13.7% (O.R.=3.30). Among HIV+ women, 25.8% had low-grade CIN and 8.6% high-grade CIN versus 10.8% and 2.9%, respectively among the HIV- women. Prevalence of HPV infection was 50.0% among women with CIN vs. 5.8% in women without CIN (P= 10(-6)). In the multivariate analysis of the determinants of CIN, smoking more than ten pack-years and HPV infection were the only two variables associated with CIN while association with HIV infection disappeared. Among HIV+ women, the variables associated with CIN were clinical AIDS and HPV infection.
Acquired Immunodeficiency Syndrome↗
p53 protein expression in grade II astrocytomas: immunohistochemical study of 100 cases with long-term follow-up.
p53 protein expression was evaluated by immunohistochemistry in a homogeneous series of 100 supratentorial grade II astrocytomas with long-term follow-up. The staining was positive in 72 cases. The proportion of p53 positive tumors was slightly higher in younger patients. The tumor regrowths which derived from p53 positive tumors were themselves p53 positive, and this p53 immunopositivity was often stronger than in the initial tumors. All of the 10 gemistocytic astrocytomas included in our series were p53 positive, and age more than histological type appeared decisive in prognosis. p53 protein expression did not quite reach statistical significance as an independent predictive variable in multivariate analysis, whereas survival was related with age, mass effect, surgery and tumor location. Only a tendency to a longer survival was observed on the curves in younger patients with mildly positive tumors.
Adolescent↗
Primary angiosarcoma of bone in Paget's disease.
We report here a case of a primitive angiosarcoma of the bone occurring in Paget's disease. Only one case has been previously described in the literature. We review other types of tumours occurring in Pagetic bone and discuss medical and surgical management.
Bone Neoplasms↗
Astrocytomas and choroid plexus tumors in two families with identical p53 germline mutations.
Germline p53 mutations carry an increased risk of development of breast cancer, soft tissue and osteosarcomas, brain tumors, leukemia and adrenocortical carcinomas. Cerebral neoplasms are usually of astrocytic lineage and occur in 40% of affected families. This report presents clinical, neuropathological and molecular genetic data from 2 families in France with an identical p53 germline mutation in codon 248 (CGG->TGG; Arg->Trp) and a clustering of CNS tumors. The youngest patient in each family developed a malignant choroid plexus tumor while several young adults of both kindred succumbed to low-grade astrocytoma, anaplastic astrocytoma or glioblastoma. The only non-neural neoplasm was an adrenocortical carcinoma in a boy aged 4 years who developed an anaplastic choroid plexus papilloma 2 years later. Of 2 previously reported inherited choroid plexus tumors, 1 occurred in a family which also carried a germline mutation in codon 248. It remains to be shown whether this unusual pattern of CNS tumors is due to an organ-specific effect of this particular p53 mutation or whether it reflects the genetic background of the affected families.
Adult↗